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Sample records for congenital extrahepatic portosystemic

  1. Congenital extrahepatic portosystemic shunts

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    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S. [Department of Diagnostic Imaging, Hospital for Sick Children, 555 University Avenue, M5G 1X8, Toronto, Ontario (Canada)

    2003-09-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  2. Congenital extrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

    Murray, Conor P.; Yoo, Shi-Joon; Babyn, Paul S.

    2003-01-01

    A congenital extrahepatic portosystemic shunt (CEPS) is uncommon. A type 1 CEPS exists where there is absence of intrahepatic portal venous supply and a type 2 CEPS where this supply is preserved. The diagnosis of congenital portosystemic shunt is important because it may cause hepatic encephalopathy. To describe the clinical and imaging features of three children with CEPS and to review the cases in the published literature. The diagnostic imaging and medical records for three children with CEPS were retrieved and evaluated. An extensive literature search was performed. Including our cases, there are 61 reported cases of CEPS, 39 type 1 and 22 type 2. Type 1 occurs predominantly in females, while type 2 shows no significant sexual preponderance. The age at diagnosis ranges from 31 weeks of intrauterine life to 76 years. Both types of CEPS have a number of associations, the most common being nodular lesions of the liver (n=25), cardiac anomalies (n=19), portosystemic encephalopathy (n=10), polysplenia (n=9), biliary atresia (n=7), skeletal anomalies (n=5), and renal tract anomalies (n=4). MRI is recommended as an important means of diagnosing and classifying cases of CEPS and examining the associated cardiovascular and hepatic abnormalities. Screening for CEPS in patients born with polysplenia is suggested. (orig.)

  3. Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia

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    Newman, Beverley [Lucile Packard Children' s Hospital, Department of Radiology, Stanford University School of Medicine, Stanford, CA (United States); Feinstein, Jeffrey A. [Stanford University School of Medicine, Division of Pediatric Cardiology, Department of Pediatrics, Lucile Packard Children' s Hospital, Stanford (United States); Cohen, Ronald A.; Patel, Hitendra [Children' s Hospital and Research Center, Department of Diagnostic Radiology, Oakland, CA (United States); Feingold, Brian; Kreutzer, Jacqueline [Children' s Hospital of Pittsburgh, Department of Pediatrics, Division of Pediatric Cardiology, Pittsburgh, PA (United States); Chan, Fandics P. [Stanford University School of Medicine, Cardiovascular Imaging Section, Department of Radiology, Lucile Packard Children' s Hospital, Stanford, CA (United States)

    2010-07-15

    Heterotaxy with polysplenia is associated with many cardiovascular anomalies including the occasional occurrence of congenital extrahepatic portosystemic shunts (CEPS). Missing this anomaly can lead to inappropriate and ineffective therapy. To emphasize the importance and associated anatomy of CEPS in conjunction with heterotaxy with polysplenia. Review of three young children who presented with cyanosis and pulmonary hypertension without a cardiac etiology. They were known (1) or discovered (2) to have heterotaxy with polysplenia. There was absence of the intrahepatic inferior vena cava (IVC) with azygos or hemiazygos continuation in all three cases. In spite of normal liver function, they were discovered to have large portosystemic shunts, splenorenal in location, along with diffuse peripheral pulmonary arterial dilatation suggestive of CEPS (Abernethy malformation) with hepatopulmonary or, more accurately, portopulmonary syndrome. All CEPS were ipsilateral to the spleens. Patency of the portal veins in these cases allowed for percutaneous shunt closure with resolution of cyanosis. CEPS is associated with heterotaxy with polysplenia and can be symptomatic because of pulmonary arteriovenous (AV) shunting. Portal and hepatic vein patency are critical for determining feasibility of CEPS closure. (orig.)

  4. Ammonia concentrations in arterial blood, venous blood, and cerebrospinal fluid of dogs with and without congenital extrahepatic portosystemic shunts.

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    Or, Matan; Devriendt, Nausikaa; Kitshoff, Adriaan M; Peremans, Kathelijne; Vandermeulen, Eva; Paepe, Dominique; Polis, Ingeborgh; Martlé, Valentine; de Rooster, Hilde

    2017-11-01

    OBJECTIVE To compare ammonia concentrations in arterial blood, venous blood, and CSF samples of dogs with and without extrahepatic portosystemic shunts (EHPSS). ANIMALS 19 dogs with congenital EHPSS and 6 healthy control dogs. PROCEDURES All dogs underwent a physical examination and then were anesthetized for transsplenic portal scintigraphy to confirm the presence or absence of EHPSS. While dogs were anesthetized, arterial and venous blood samples and a CSF sample were simultaneously collected for determination of ammonia concentration, which was measured by use of a portable blood ammonia analyzer (device A) and a nonportable biochemical analyzer (device B). Results were compared between dogs with EHPSS and control dogs. RESULTS Arterial, venous, and CSF ammonia concentrations for dogs with EHPSS were significantly greater than those for control dogs. For dogs with EHPSS, ammonia concentrations in both arterial and venous blood samples were markedly increased from the reference range. There was a strong positive correlation between arterial and venous ammonia concentrations and between blood (arterial or venous) and CSF ammonia concentrations. CONCLUSIONS AND CLINICAL RELEVANCE Results indicated that blood and CSF ammonia concentrations in dogs with EHPSS were greater than those for healthy dogs and were strongly and positively correlated, albeit in a nonlinear manner. This suggested that the permeability of the blood-brain barrier to ammonia may be abnormally increased in dogs with EHPSS, but further investigation of the relationship between blood or CSF ammonia concentration and clinical signs of hepatic encephalopathy or the surgical outcome for dogs with EHPSS is warranted.

  5. Congenital Intrahepatic Portosystemic Shunts

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    Lee, Woong Hee; Kim, Young Tong; Jou, Sung Shick; Shin, Hyeong Cheol [Soonchunhyang University, Asan (Korea, Republic of)

    2008-12-15

    Intrahepatic portosystemic shunts are an anomalous connection between the portal vein and hepatic vein/IVC, which may be either congenital or acquired secondary to liver cirrhosis or portal hypertension. Cases of congenital intrahepatic shunts are usually encountered in children and may spontaneously resolve. We report 5 cases of congenital intrahepatic portosystemic shunts in neonates and an adult

  6. Congenital portosystemic shunts with and without gastrointestinal bleeding - case series

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    Gong, Ying; Chen, Jun; Chen, Qi; Ji, Min; Pa, Mier; Qiao, Zhongwei [Children' s Hospital of Fudan University, Department of Radiology, Shanghai (China); Zhu, Hui [Fudan University Shanghai Cancer Center, Department of Radiology, Shanghai (China); Zheng, Shan [Children' s Hospital of Fudan University, Department of Surgery, Shanghai (China)

    2015-12-15

    The clinical presentation of congenital portosystemic shunt is variable and gastrointestinal bleeding is an uncommon presentation. To describe the imaging features of congenital portosystemic shunt as it presented in 11 children with (n = 6) and without gastrointestinal bleeding (n = 5). We performed a retrospective study on a clinical and imaging dataset of 11 children diagnosed with congenital portosystemic shunt. A total of 11 children with congenital portosystemic shunt were included in this study, 7 with extrahepatic portosystemic shunts and 4 with intrahepatic portosystemic shunts. Six patients with gastrointestinal bleeding had an extrahepatic portosystemic shunt, and the imaging results showed that the shunts originated from the splenomesenteric junction (n = 5) or splenic vein (n = 1) and connected to the internal iliac vein. Among the five cases of congenital portosystemic shunt without gastrointestinal bleeding, one case was an extrahepatic portosystemic shunt and the other four were intrahepatic portosystemic shunts. Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high. (orig.)

  7. Cellophane banding for the gradual attenuation of single extrahepatic portosystemic shunts in eleven dogs.

    Science.gov (United States)

    Youmans, K R; Hunt, G B

    1998-08-01

    To evaluate the efficacy and short term effects of a cellophane banding technique for progressive attenuation of canine single extrahepatic portosystemic shunts. A prospective trial of 11 dogs with single congenital extrahepatic shunts. Rectal ammonia tolerance testing and routine biochemical tests were performed preoperatively on all dogs. In seven dogs, preoperative abdominal Doppler ultrasonography was also performed. Exploratory laparotomy revealed a single extrahepatic portocaval shunt in each animal, which was attenuated using a cellophane band with an internal diameter of 2 to 3 mm. The abdomen was closed routinely. Follow-up biochemical analysis and abdominal Doppler ultrasonography or splenoportography were performed postoperatively. The shunt was not amenable to total ligation in 11 dogs, based upon reported criteria. All dogs recovered uneventfully from surgery without evidence of portal hypertension, and showed clinical improvement thereafter. Shunt occlusion was deemed to have occurred in 10 dogs based on resolution of biochemical and/or sonographic abnormalities. One dog continued to have sonographic evidence of portosystemic shunting when evaluated 3 weeks after surgery, despite normal ammonia tolerance, but was lost to subsequent follow-up. Two dogs, in which 3 mm cellophane bands were placed, experienced delayed shunt occlusion. Cellophane banding is simple to perform, and causes progressive attenuation of single extrahepatic shunts in dogs. Further work is needed to determine the maximum diameter of a cellophane band which will produce total attenuation, and the long-term safety and reliability of the treatment.

  8. Genetic and Functional Analysis of Congenital Portosystemic Shunts in Dogs

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    van den Bossche, L.

    2017-01-01

    The general aim of this thesis was to gain further insight into the pathogenesis of congenital portosystemic shunts (CPSS), elucidate mechanisms involved in the pathophysiology of CPSS, and explore predictors of recovery after surgical ligation of the shunt. For intrahepatic portosystemic shunts

  9. Portosystemic shunts for extrahepatic portal hypertension in children.

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    Tocornal, J; Cruz, F

    1981-07-01

    Twenty-three children with prehepatic portal hypertension and hemorrhage due to ruptured esophagogastric varices had portosystemic shunts. Their ages ranged from two years and seven months to 15 years. Eleven were less than eight years of age. Twenty patients had portal vein cavernomatosis and three patients had double portal veins. In 21 patients, a mesocaval type of shunt was done. A splenorenal shunt was performed in two. There was no surgical mortality. Two shunts occluded, both in rather young infants--two years and seven months and three years of age. In all the others, there was no further bleeding, and the shunts remained patent, as shown by abdominal angiograms. Neuropsychiatric disorders, probably due to hepatic encephalopathy, occurred in only one patient. On the basis of this favorable experience, we believe that an elective portosystemic shunt should, in general, be performed upon children with prehepatic portal hypertension after one major variceal hemorrhage. We favor a mesocaval type of shunt in these children because of the larger diameter of the vessels involved in the anastomosis and because it preserves the spleen, maintaining defense against subsequent infection.

  10. Haematology and coagulation profiles in cats with congenital portosystemic shunts.

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    Tzounos, Caitlin E; Tivers, Michael S; Adamantos, Sophie E; English, Kate; Rees, Alan L; Lipscomb, Vicky J

    2017-12-01

    Objectives The objectives of this study were, first, to report the haematological parameters and coagulation times for cats with a congenital portosystemic shunt (CPSS) and the influence of surgical shunt attenuation on these parameters; and, second, to identify any association between prolongation in coagulation profiles and incidence of perioperative haemorrhage. Methods This was a retrospective clinical study using client-owned cats with a CPSS. Signalment, shunt type (extra- or intrahepatic), degree of shunt attenuation (complete or partial), haematological parameters, prothrombin time (PT) and activated partial thromboplastin time (aPTT) test results, and occurrence of any perioperative clinical bleeding complications were recorded for cats undergoing surgical treatment of a CPSS at the Royal Veterinary College, UK, between 1994 and 2011. Results Forty-two cats were included. Thirty-six (85.7%) had an extrahepatic CPSS and six (14.3%) had an intrahepatic CPSS. Preoperatively, mean cell volume (MCV) and mean cell haemoglobin (MCH) were below the reference interval (RI) in 32 (76.2%) and 31 (73.8%) cats, respectively. Red blood cell count and mean cell haemoglobin concentration (MCHC) were above the RI in 10 (23.8%) and eight (19.1%) cats, respectively. Postoperatively, there were significant increases in haematocrit ( P = 0.044), MCV ( P = 0.008) and MCH ( P = 0.002). Despite the significant increase in MCV postoperatively, the median MCV postoperatively was below the RI, indicating persistence of microcytosis. Preoperatively, PT was above the upper RI in 14 cats (87.5%), and aPTT was above the upper RI in 11 cats (68.8%). No cat demonstrated a perioperative clinical bleeding complication. Conclusions and relevance Cats with a CPSS are likely to present with a microcytosis, but rarely present with anaemia, leukocytosis or thrombocytopenia. Surgical attenuation of the CPSS results in a significant increase in the HCT and MCV. Coagulation profiles in cats with a

  11. Congenital hepatic arteriovenous fistula with intrahepatic portosystemic shunt and aortic stenosis in a dog

    International Nuclear Information System (INIS)

    Koide, K.; Koide, Y.; Wada, Y.; Nakaniwa, S.; Yamane, Y.

    2004-01-01

    Examination of a 2-month-old male golden retriever presented to the hospital revealed malnutrition, ascites, cardiac murmur and hyperammonemia. Identification of subaortic stenosis and hepatic arteriovenous fistula was made through ultrasonography and angiocardiography. In addition, intrasurgical mesenteric portography showed an intrahepatic portosystemic shunt. The dog did not show portal hypertension and secondary multiple extrahepatic portosystemic shunts. Surgical correction was attempted after medical treatment. The hepatic artery branch which was connected to the hepatic arteriovenous fistula was separated, and completely ligated using silk ligature. However, the separation of the intrahepatic shunt blood vessel was unsuccessful and the dog died 15 hr postoperatively

  12. Surgical attenuation of congenital portosystemic shunts in dogs. Techniques, complications and prognosis

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    Kummeling, A.|info:eu-repo/dai/nl/304828793

    2009-01-01

    The general aim of this thesis was to identify factors associated with outcome after surgical attenuation of congenital portosystemic shunts (CPSS) in dogs and to clarify underlying mechanisms of postoperative recovery in this disease. Two surgical techniques used for CPSS attenuation, ligation and

  13. Congenital intrahepatic arterioportal and portosystemic venous fistulae with jejunal arteriovenous malformation depicted on multislice spiral CT

    International Nuclear Information System (INIS)

    Chae, Eun Jin; Goo, Hyun Woo; Yoon, Chong Hyun; Kim, Seong-Chul

    2004-01-01

    We report a symptomatic infant with very rare congenital arterioportal and portosystemic venous fistulae in the liver. Multislice CT after partial transcatheter embolisation revealed not only the complicated vascular architecture of the lesion, but also an incidental jejunal arteriovenous malformation which explained the patient's melena. The patient underwent ligation of the hepatic artery and resection of the jejunal arteriovenous malformation. Postoperative multislice CT clearly demonstrated the success of the treatment. (orig.)

  14. Evaluation of hepatic steatosis in dogs with congenital portosystemic shunts using Oil-Red-O staining

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    Hunt, GB; Luff, J; Daniel, L; Van den Bergh, R.

    2015-01-01

    The aims of this prospective study were to quantify steatosis in dogs with congenital portosystemic shunts using a fat-specific stain, to compare the amount of steatosis in different lobes of the liver, and to evaluate intra- and inter-Observer variability in lipid point counting. Computer-assisted point counting of lipid droplets was undertaken following Oil-Red-O staining in 21 dogs with congenital portosystemic shunts and 9 control dogs. Dogs with congenital portosystemic shunts had significantly more small lipid droplets ( 9 μ) and lipogranulomas per tissue point (p = 0.023 and 0.01, respectively). In conclusion, computer-assisted counting of lipid droplets following Oil Red O staining of liver biopsy samples allows objective measurement and detection of significant differences between dogs with CPS and normal dogs. This method will allow future evaluation of the relationship between different presentations of CPS (anatomy, age, breed) and lipidosis, as well as the impact of hepatic lipidosis on outcomes following surgical shunt attenuation. PMID:23528942

  15. RE: Endovascular Treatment of Congenital Intrahepatic Portosystemic Shunts with Amplatzer Plugs

    International Nuclear Information System (INIS)

    Sierre, Sergio; Alonso, Jose; Lipsich, Jose

    2012-01-01

    In our paper entitled 'Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device' published in the Journal of Vascular and Interventional Radiology in 2004, we already reported the use of the AVP in the treatment of an intrahepatic portosystemic venous shunt. This situation does not undervalue the quality of the reported case, but for didactic purposes, we believe it is important to state that the work of Dr. Lee confirms, as was previously reported, that these devices are useful and safe for these rare situations.

  16. RE: Endovascular Treatment of Congenital Intrahepatic Portosystemic Shunts with Amplatzer Plugs

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    Sierre, Sergio; Alonso, Jose; Lipsich, Jose [Hospital Nacional de Pediatria ' JP Garrahan' , Combate de los Pozos, Buenos (Argentina)

    2012-01-15

    In our paper entitled 'Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device' published in the Journal of Vascular and Interventional Radiology in 2004, we already reported the use of the AVP in the treatment of an intrahepatic portosystemic venous shunt. This situation does not undervalue the quality of the reported case, but for didactic purposes, we believe it is important to state that the work of Dr. Lee confirms, as was previously reported, that these devices are useful and safe for these rare situations.

  17. Histologic examination of hepatic biopsy samples as a prognostic indicator in dogs undergoing surgical correction of congenital portosystemic shunts: 64 cases (1997-2005).

    Science.gov (United States)

    Parker, Jacquelyn S; Monnet, Eric; Powers, Barbara E; Twedt, David C

    2008-05-15

    To determine whether results of histologic examination of hepatic biopsy samples could be used as an indicator of survival time in dogs that underwent surgical correction of a congenital portosystemic shunt (PSS). Retrospective case series. 64 dogs that underwent exploratory laparotomy for an extrahepatic (n = 39) or intrahepatic (25) congenital PSS. All H&E-stained histologic slides of hepatic biopsy samples obtained at the time of surgery were reviewed by a single individual, and severity of histologic abnormalities (ie, arteriolar hyperplasia, biliary hyperplasia, fibrosis, cell swelling, lipidosis, lymphoplasmacytic cholangiohepatitis, suppurative cholangiohepatitis, lipid granulomas, and dilated sinusoids) was graded. A Cox proportional hazards regression model was used to determine whether each histologic feature was associated with survival time. Median follow-up time was 35.7 months, and median survival time was 50.6 months. Thirty-eight dogs were alive at the time of final follow-up; 15 had died of causes associated with the PSS, including 4 that died immediately after surgery; 3 had died of unrelated causes; and 8 were lost to follow-up. None of the histologic features examined were significantly associated with survival time. Findings suggested that results of histologic examination of hepatic biopsy samples obtained at the time of surgery cannot be used to predict long-term outcome in dogs undergoing surgical correction of a PSS.

  18. Hepatic gene expression and plasma albumin concentration related to outcome after attenuation of a congenital portosystemic shunt in dogs

    NARCIS (Netherlands)

    Kummeling, A.; Penning, L.C.; Rothuizen, J.; Brinkhof, B.; Weber, M.F.; van Sluijs, F.J.

    2012-01-01

    Abstract In dogs with a congenital portosystemic shunt (CPSS), the outcome after CPSS attenuation is difficult to predict but is most likely related to hepatic and vascular proliferation that follows the attenuation. The aim of this study was to evaluate the prognostic value of shunt localization

  19. Morphology of congenital portosystemic shunts involving the left colic vein in dogs and cats.

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    White, R N; Parry, A T

    2016-05-01

    To describe the anatomy of congenital portosystemic shunts involving the left colic vein in dogs and cats. Retrospective review of a consecutive series of dogs and cats managed for congenital portosystemic shunts. For inclusion a shunt involving the left colic vein with recorded intraoperative mesenteric portovenography or computed tomography angiography along with direct gross surgical observations at the time of surgery was required. Six dogs and three cats met the inclusion criteria. All cases had a shunt which involved a distended left colic vein. The final communication with a systemic vein was variable; in seven cases (five dogs, two cats) it was via the caudal vena cava, in one cat it was via the common iliac vein and in the remaining dog it was via the internal iliac vein. In addition, two cats showed caudal vena cava duplication. The morphology of this shunt type appeared to be a result of an abnormal communication between either the left colic vein or the cranial rectal vein and a pelvic systemic vein (caudal vena cava, common iliac vein or internal iliac vein). This information may help with surgical planning in cases undergoing shunt closure surgery. © 2016 British Small Animal Veterinary Association.

  20. Hepatic Encephalopathy due to Congenital Multiple Intrahepatic Portosystemic Venous Shunts Successfully Treated by Percutaneous Transhepatic Obliteration

    Directory of Open Access Journals (Sweden)

    Shinsuke Takenaga

    2016-11-01

    Full Text Available Hepatic encephalopathy due to intrahepatic portosystemic venous shunts (IPSVS in a non-cirrhotic condition is rare. Here we report a rare case of a patient with congenital multiple IPSVS successfully treated by percutaneous transhepatic obliteration. The patient was a 67-year-old woman who presented to our hospital with progressive episodes of consciousness disorder and vomiting. Laboratory tests revealed hyperammonemia (192.0 μg/dL, and computed tomography revealed multiple IPSVS in both lobes. There was no evidence of underlying liver disease or hepatic trauma. Transcatheter embolization for IPSVS was performed because conservative therapy was not sufficiently effective. After endovascular shunt closure, hepatic encephalopathy improved. The serum ammonia level normalized during the 5-year follow-up period. Thus, transcatheter embolization may be an effective therapy for patients with symptomatic and refractory IPSVS. Careful follow-up is necessary for portal hypertension-related complications after transcatheter embolization for IPSVS.

  1. Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review.

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    McElhinney, Doff B; Marx, Gerald R; Newburger, Jane W

    2011-01-01

    Published case reports suggest that congenital portosystemic venous connections (PSVC) and other abdominal venous anomalies may be relatively frequent and potentially important in patients with polysplenia syndrome. Our objective was to investigate the frequency and range of portal and other abdominal systemic venous anomalies in patients with polysplenia and inferior vena cava (IVC) interruption who underwent a cavopulmonary anastomosis procedure at our center, and to review the published literature on this topic and the potential clinical importance of such anomalies. Retrospective cohort study and literature review were used. Among 77 patients with heterotaxy, univentricular heart disease, and IVC interruption who underwent a bidirectional Glenn and/or modified Fontan procedure, pulmonary arteriovenous malformations were diagnosed in 33 (43%). Bilateral superior vena cavas were present in 42 patients (55%). Despite inadequate imaging in many patients, a partial PSVC, dual IVCs, and/or renal vein anomalies were detected in 15 patients (19%). A PSVC formed by a tortuous vessel running from the systemic venous system to the extrahepatic portal vein was found in six patients (8%). Abdominal venous anomalies other than PSVC were documented in 13 patients (16%), including nine (12%) with some form of duplicated IVC system, with a large azygous vein continuing to the superior vena cava and a parallel, contralateral IVC of similar or smaller size, and seven with renal vein anomalies. In patients with a partial PSVC or a duplicate IVC that connected to the atrium, the abnormal connection allowed right-to-left shunting. PSVC and other abdominal venous anomalies may be clinically important but under-recognized in patients with IVC interruption and univentricular heart disease. In such patients, preoperative evaluation of the abdominal systemic venous system may be valuable. More data are necessary to determine whether there is a pathophysiologic connection between the

  2. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

    International Nuclear Information System (INIS)

    Bruckheimer, Elchanan; Dagan, Tamir; Atar, Eli; Schwartz, Michael; Kachko, Ludmila; Superina, Riccardo; Amir, Gabriel; Shapiro, Rivka; Birk, Einat

    2013-01-01

    Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ∼50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5–13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2–8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia

  3. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

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    Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net; Dagan, Tamir [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel); Atar, Eli; Schwartz, Michael [Schneider Children' s Medical Center Israel, Section of Radiology (Israel); Kachko, Ludmila [Schneider Children' s Medical Center Israel, Section of Anesthesiology (Israel); Superina, Riccardo; Amir, Gabriel [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel); Shapiro, Rivka [Schneider Children' s Medical Center Israel, Section of Gastroenterology (Israel); Birk, Einat [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel)

    2013-12-15

    Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by {approx}50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 {+-} 11.3 to 10.8 {+-} 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 {+-} 0.5 to 4.0 {+-} 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 {+-} 53.6 to 65.7 {+-} 9.6 {mu}mol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

  4. Aberrant hepatic lipid storage and metabolism in canine portosystemic shunts.

    Directory of Open Access Journals (Sweden)

    Lindsay Van den Bossche

    Full Text Available Non-alcoholic fatty liver disease (NAFLD is a poorly understood multifactorial pandemic disorder. One of the hallmarks of NAFLD, hepatic steatosis, is a common feature in canine congenital portosystemic shunts. The aim of this study was to gain detailed insight into the pathogenesis of steatosis in this large animal model. Hepatic lipid accumulation, gene-expression analysis and HPLC-MS of neutral lipids and phospholipids in extrahepatic (EHPSS and intrahepatic portosystemic shunts (IHPSS was compared to healthy control dogs. Liver organoids of diseased dogs and healthy control dogs were incubated with palmitic- and oleic-acid, and lipid accumulation was quantified using LD540. In histological slides of shunt livers, a 12-fold increase of lipid content was detected compared to the control dogs (EHPSS P<0.01; IHPSS P = 0.042. Involvement of lipid-related genes to steatosis in portosystemic shunting was corroborated using gene-expression profiling. Lipid analysis demonstrated different triglyceride composition and a shift towards short chain and omega-3 fatty acids in shunt versus healthy dogs, with no difference in lipid species composition between shunt types. All organoids showed a similar increase in triacylglycerols after free fatty acids enrichment. This study demonstrates that steatosis is probably secondary to canine portosystemic shunts. Unravelling the pathogenesis of this hepatic steatosis might contribute to a better understanding of steatosis in NAFLD.

  5. Aberrant hepatic lipid storage and metabolism in canine portosystemic shunts.

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    Van den Bossche, Lindsay; Schoonenberg, Vivien A C; Burgener, Iwan A; Penning, Louis C; Schrall, Ingrid M; Kruitwagen, Hedwig S; van Wolferen, Monique E; Grinwis, Guy C M; Kummeling, Anne; Rothuizen, Jan; van Velzen, Jeroen F; Stathonikos, Nikolas; Molenaar, Martijn R; Helms, Bernd J; Brouwers, Jos F H M; Spee, Bart; van Steenbeek, Frank G

    2017-01-01

    Non-alcoholic fatty liver disease (NAFLD) is a poorly understood multifactorial pandemic disorder. One of the hallmarks of NAFLD, hepatic steatosis, is a common feature in canine congenital portosystemic shunts. The aim of this study was to gain detailed insight into the pathogenesis of steatosis in this large animal model. Hepatic lipid accumulation, gene-expression analysis and HPLC-MS of neutral lipids and phospholipids in extrahepatic (EHPSS) and intrahepatic portosystemic shunts (IHPSS) was compared to healthy control dogs. Liver organoids of diseased dogs and healthy control dogs were incubated with palmitic- and oleic-acid, and lipid accumulation was quantified using LD540. In histological slides of shunt livers, a 12-fold increase of lipid content was detected compared to the control dogs (EHPSS Plipid-related genes to steatosis in portosystemic shunting was corroborated using gene-expression profiling. Lipid analysis demonstrated different triglyceride composition and a shift towards short chain and omega-3 fatty acids in shunt versus healthy dogs, with no difference in lipid species composition between shunt types. All organoids showed a similar increase in triacylglycerols after free fatty acids enrichment. This study demonstrates that steatosis is probably secondary to canine portosystemic shunts. Unravelling the pathogenesis of this hepatic steatosis might contribute to a better understanding of steatosis in NAFLD.

  6. Hyperammonemia and systemic inflammatory response syndrome predicts presence of hepatic encephalopathy in dogs with congenital portosystemic shunts.

    Directory of Open Access Journals (Sweden)

    Mickey S Tivers

    Full Text Available Hepatic encephalopathy (HE is an important cause of morbidity and mortality in patients with liver disease. The pathogenesis of he is incompletely understood although ammonia and inflammatory cytokines have been implicated as key mediators. To facilitate further mechanistic understanding of the pathogenesis of HE, a large number of animal models have been developed which often involve the surgical creation of an anastomosis between the hepatic portal vein and the caudal vena cava. One of the most common congenital abnormalities in dogs is a congenital portosystemic shunt (cpss, which closely mimics these surgical experimental models of HE. Dogs with a cPSS often have clinical signs which mimic clinical signs observed in humans with HE. Our hypothesis is that the pathogenesis of HE in dogs with a cPSS is similar to humans with HE. The aim of the study was to measure a range of clinical, haematological and biochemical parameters, which have been linked to the development of HE in humans, in dogs with a cPSS and a known HE grade. One hundred and twenty dogs with a cPSS were included in the study and multiple regression analysis of clinical, haematological and biochemical variables revealed that plasma ammonia concentrations and systemic inflammatory response syndrome scores predicted the presence of HE. Our findings further support the notion that the pathogenesis of canine and human HE share many similarities and indicate that dogs with cPSS may be an informative spontaneous model of human HE. Further investigations on dogs with cPSS may allow studies on HE to be undertaken without creating surgical models of HE thereby allowing the number of large animals used in animal experimentation to be reduced.

  7. Contrast-enhanced computed tomography angiography and volume-rendered imaging for evaluation of cellophane banding in a dog with extrahepatic portosystemic shunt

    Directory of Open Access Journals (Sweden)

    H. Yoon

    2011-04-01

    Full Text Available A 4-year-old, 1.8 kg, male, castrated Maltese was presented for evaluation of urolithiasis. Urinary calculi were composed of ammonium biurate. Preprandial and postprandial bile acids were 44.2 and 187.3 μmol/ , respectively (reference ranges 0–10 and 0–20 μmol/ , respectively. Single-phase contrast-enhanced computed tomography angiography (CTA with volume-rendered imaging (VRI was obtained. VRI revealed a portocaval shunt originating just cranial to a tributary of the gastroduodenal vein and draining into the caudal vena cava at the level of the epiploic foramen. CTA revealed a 3.66 mm-diameter shunt measured at the level of the termination of the shunt and a 3.79 mm-diameter portal vein measured at the level between the origin of the shunt and the porta of the liver. Surgery was performed using cellophane banding without attenuation. Follow-up single-phase CTA with VRI was obtained 10 weeks after surgery. VRI revealed no evidence of portosystemic communication on the level of a cellophane band and caudal to the cellophane band. CTA demonstrated an increased portal vein diameter (3.79–5.27 mm measured at the level between the origin of the shunt and the porta of the liver. Preprandial and postprandial bile acids were 25 and 12.5 μmol/ , respectively (aforementioned respective reference ranges, 3 months post-surgery. No problems were evident at 6 months.

  8. Orthotopic liver transplantation for portosystemic encephalopathy in an adult with congenital absence of the portal vein

    NARCIS (Netherlands)

    Wojcicki, M; Haagsma, EB; Gouw, ASH; Slooff, MJH; Porte, RJ

    Congenital absence of the portal vein (CAPV) is a very rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. There is no portal perfusion of the liver and no portal hypertension. This abnormality is usually coincidentally discovered in children, the

  9. Acquired portosystemic collaterals: anatomy and imaging

    Energy Technology Data Exchange (ETDEWEB)

    Leite, Andrea Farias de Melo; Mota Junior, Americo, E-mail: andreafariasm@gmail.com [Instituto de Medicina Integral Professor Fernando Figueira de Pernambuco (IMIP), Recife, PE (Brazil); Chagas-Neto, Francisco Abaete [Universidade de Fortaleza (UNIFOR), Fortaleza, CE (Brazil); Teixeira, Sara Reis; Elias Junior, Jorge; Muglia, Valdair Francisco [Universidade de Sao Paulo (FMRP/USP), Ribeirao Preto, SP (Brazil). Faculdade de Medicina

    2016-07-15

    Portosystemic shunts are enlarged vessels that form collateral pathological pathways between the splanchnic circulation and the systemic circulation. Although their causes are multifactorial, portosystemic shunts all have one mechanism in common - increased portal venous pressure, which diverts the blood flow from the gastrointestinal tract to the systemic circulation. Congenital and acquired collateral pathways have both been described in the literature. The aim of this pictorial essay was to discuss the distinct anatomic and imaging features of portosystemic shunts, as well as to provide a robust method of differentiating between acquired portosystemic shunts and similar pathologies, through the use of illustrations and schematic drawings. Imaging of portosystemic shunts provides subclinical markers of increased portal venous pressure. Therefore, radiologists play a crucial role in the identification of portosystemic shunts. Early detection of portosystemic shunts can allow ample time to perform endovascular shunt operations, which can relieve portal hypertension and prevent acute or chronic complications in at-risk patient populations. (author)

  10. Acquired portosystemic collaterals: anatomy and imaging

    International Nuclear Information System (INIS)

    Leite, Andrea Farias de Melo; Mota Junior, Americo; Chagas-Neto, Francisco Abaete; Teixeira, Sara Reis; Elias Junior, Jorge; Muglia, Valdair Francisco

    2016-01-01

    Portosystemic shunts are enlarged vessels that form collateral pathological pathways between the splanchnic circulation and the systemic circulation. Although their causes are multifactorial, portosystemic shunts all have one mechanism in common - increased portal venous pressure, which diverts the blood flow from the gastrointestinal tract to the systemic circulation. Congenital and acquired collateral pathways have both been described in the literature. The aim of this pictorial essay was to discuss the distinct anatomic and imaging features of portosystemic shunts, as well as to provide a robust method of differentiating between acquired portosystemic shunts and similar pathologies, through the use of illustrations and schematic drawings. Imaging of portosystemic shunts provides subclinical markers of increased portal venous pressure. Therefore, radiologists play a crucial role in the identification of portosystemic shunts. Early detection of portosystemic shunts can allow ample time to perform endovascular shunt operations, which can relieve portal hypertension and prevent acute or chronic complications in at-risk patient populations. (author)

  11. Multiple congenital PSS in a dog: case report and literature review.

    Science.gov (United States)

    Leeman, Jessica J; Kim, Stanley E; Reese, David J; Risselada, Marije; Ellison, Gary W

    2013-01-01

    A 4 yr old spayed female mixed-breed dog presented with a 2 yr history of recurring increases in liver enzymes. Two congenital portosystemic shunts (PSSs) were identified using computed tomography (CT) angiography, which included a portoazygous and portorenal extrahepatic shunt. Double right renal veins were also identified. The shunts were successfully identified and attenuated with cellophane banding. Multiple congenital PSS is a rare phenomenon, but should be considered during exploratory laparotomy for PSS and in dogs with poor response to surgical attenuation of a single PSS. CT proved to be a crucial part of accurate diagnosis and surgical planning for this dog with multiple congenital PSS.

  12. Regional cerebral blood flow assessed by single photon emission computed tomography (SPECT) in dogs with congenital portosystemic shunt and hepatic encephalopathy.

    Science.gov (United States)

    Or, Matan; Peremans, Kathelijne; Martlé, Valentine; Vandermeulen, Eva; Bosmans, Tim; Devriendt, Nausikaa; de Rooster, Hilde

    2017-02-01

    Regional cerebral blood flow (rCBF) in eight dogs with congenital portosystemic shunt (PSS) and hepatic encephalopathy (HE) was compared with rCBF in eight healthy control dogs using single photon emission computed tomography (SPECT) with a 99m technetium-hexamethylpropylene amine oxime ( 99m Tc-HMPAO) tracer. SPECT scans were abnormal in all PSS dogs. Compared to the control group, rCBF in PSS dogs was significantly decreased in the temporal lobes and increased in the subcortical (thalamic and striatal) area. Brain perfusion imaging alterations observed in the dogs with PSS and HE are similar to those in human patients with HE. These findings suggest that dogs with HE and PSS have altered perfusion of mainly the subcortical and the temporal regions of the brain. Copyright © 2016 Elsevier Ltd. All rights reserved.

  13. Determining the optimal portal blood volume in a shunt before surgery in extrahepatic portal hypertension

    Directory of Open Access Journals (Sweden)

    Yurchuk Vladimir A

    2016-04-01

    Full Text Available The aim of the study: To determine the necessary shunt diameter and assess the optimal portal blood volume in a shunt in children with extrahepatic portal hypertension before the portosystemic shunt surgery. Changes in the liver hemodynamics were studied in 81 children aged from 4 to 7 years with extrahepatic portal hypertension. We established that it is necessary to calculate the shunt diameter and the blood volume in a shunt in patients with extrahepatic portal hypertension before the portosystemic shunt surgery. It allows us to preserve the hepatic portal blood flow and effectively decrease the pressure in the portal system. Portosystemic shunt surgery in patients with extrahepatic portal hypertension performed in accordance with the individualized shunt volume significantly decreases portal pressure, preserves stable hepatic hemodynamics and prevents gastro-esophageal hemorrhage.

  14. Splenophrenic portosystemic shunt in dogs with and without portal ...

    African Journals Online (AJOL)

    The possible existence of the same pattern of porto-caval connection in dogs having a single congenital portosystemic shunt (CPSS) and in dogs having multiple acquired portosystemic shunt (MAPSS) secondary to portal hypertension (PH) was evaluated. Retrospective evaluation of all CT examinations of patients having ...

  15. Extrahepatic manifestations of cholestasis

    NARCIS (Netherlands)

    Glasova, Helena; Beuers, Ulrich

    2002-01-01

    Pruritus, fatigue and metabolic bone disease represent three major extrahepatic manifestations of chronic cholestatic liver disease that considerably affect the patient's quality of life. The present article reviews pathogenetic aspects of and current therapeutic approaches to extrahepatic

  16. Post-operative abdominal CT scanning in extrahepatic biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Day, D L; Mulcahy, P F; Letourneau, J G; Dehner, L P

    1989-07-01

    A retrospective review of the abdominal CT scans of 26 children with extrahepatic biliary atresia was performed, and the results were correlated with available surgical and pathologic data. Associated congenital anomalies or acquired abnormalities were identified in these patients. Congenital anomalies included polysplenia, venous anomalies and bowel stenosis. Acquired abnormalities developed secondary to cirrhosis, portal hypertension, intrahepatic biliary duct dilatation, and hepatic ischemia. Despite frequent episodes of ascending cholangitis in these children, no hepatic abscesses were identified by CT or by pathologic examination. In conclusion, abdominal CT scanning of children with extrahepatic biliary atresia can define congenital and acquired abnormalities and provide important anatomic data for the surgeons before liver transplantation. (orig.).

  17. Treatment of hepatic encephalopathy by retrograde transcaval coil embolization of an ileal vein-to-right gonadal vein portosystemic shunt

    International Nuclear Information System (INIS)

    Nishie, Akihiro; Yoshimitsu, Kengo; Honda, Hiroshi; Kaneko, Kuniyuki; Kuroiwa, Toshiro; Fukuya, Tatsuro; Irie, Hiroyuki; Ninomiya, Toshiharu; Yoshimitsu, Takahiro; Hirakata, Hideki; Okuda, Seiya; Masuda, Kouji

    1997-01-01

    A 43-year-old non-cirrhotic woman suffered from encephalopathy caused by an extrahepatic portosystemic shunt between the ileal vein and inferior vena cava via the right gonadal vein. Percutaneous transcatheter embolization with stainless steel coils was performed by the retrograde systemic venous approach. Encephalopathy improved dramatically

  18. Extrahepatic biliary atresia in a border collie.

    Science.gov (United States)

    Schulze, C; Rothuizen, J; van Sluijs, F J; Hazewinkel, H A; van den Ingh, T S

    2000-01-01

    Progressive lameness and leg pain were the predominant clinical signs in a 17-week-old male border collie presented for examination. On clinical investigation, extrahepatic cholestasis in association with rickets due to inadequate vitamin D resorption was diagnosed. The dog was treated parenterally with vitamin D and a cholecystoduodenostomy was performed. At 25 days postsurgery the lameness had resolved and bone structure was radiographically normal. However, at six weeks postsurgery, the dog's condition deteriorated rapidly and euthanasia was finally performed at eight weeks postsurgery. At postmortem examination, Toxocara canis nematodes were found to have invaded the biliary system via the anastomosis between the gallbladder and duodenum, causing biliary and hepatic toxocariasis. The cause of the primary extrahepatic cholestasis was atresia of the common bile duct at the hepatic end. The liver tissue showed microscopic lesions of chronic extrahepatic cholestasis as well as acute inflammation associated with the nematode invasion. There was no postmortem evidence of bone lesions. Extrahepatic biliary atresia is extremely rare in animals and has not been described before in dogs. In contrast, it represents the most common cause of congenital cholestasis in children, occurring in approximately one per 10,000 to 15,000 live births.

  19. Aberrant hepatic lipid storage and metabolism in canine portosystemic shunts

    NARCIS (Netherlands)

    Van Den Bossche, Lindsay; Schoonenberg, Vivien A.C.; Burgener, Iwan A.; Penning, Louis C.; Schrall, Ingrid M.; Kruitwagen, Hedwig S.; Van Wolferen, Monique E.; Grinwis, Guy C.M.; Kummeling, Anne; Rothuizen, Jan; Van Velzen, Jeroen F.; Stathonikos, Nikolas; Molenaar, Martijn R.; Helms, Bernd J.; Brouwers, Jos F.H.M.; Spee, Bart; Van Steenbeek, Frank G.

    2017-01-01

    Non-alcoholic fatty liver disease (NAFLD) is a poorly understood multifactorial pandemic disorder. One of the hallmarks of NAFLD, hepatic steatosis, is a common feature in canine congenital portosystemic shunts. The aim of this study was to gain detailed insight into the pathogenesis of steatosis in

  20. Embolization of nonvariceal portosystemic collaterals in transjugular intrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

    Bilbao, Jose Ignacio; Arias, Mercedes; Longo, Jesus Maria; Alejandre, Pedro Luis; Betes, Maria Teresa; Elizalde, Arlette Maria

    1997-01-01

    Percutaneous embolization of large portosystemic collaterals was performed in three patients following placement of a transjugular intrahepatic portosystemic shunt in order to improve hepatopetal portal flow. Improved hepatic portal perfusion was achieved in these cases, thereby theoretically reducing the risk of chronic hepatic encephalopathy

  1. Transjugular intrahepatic portosystemic shunt

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Hyung; Han, Joon Koo; Chung, Jin Wook; Han, Man Chung [Seoul National University College of Medicidne, Seoul (Korea, Republic of)

    1992-05-15

    As a new interventional procedure for the control of variceal bleeding, a portosystemic shunt can be established with the installment of metallic stent through the transjugular approach. In order to evaluate the clinical usefulness of the procedure, transjugular intrahepatic portosystemic shunt procedure were performed in 5 patients with variceal bleeding due to liver cirrhosis. The metallic stents were mainly a self expandable Wallstent (Schneider, Switzerland), An 8 to 10 mm shunt was formed by the insertion of the stent and balloon dilatation after puncture of the proximal portal vein from the right or middle hepatic vein. The patency of the shunt was proven by portography after the procedure. The portal pressure measured in 3 patients before and after the procedure improved with decrease from 31 mmHg to 25 mmHg. The procedure failed in 1 patient due to pre-existing portal vein thrombosis. During the follow-up period from 1 month to 4 months, shunts were patent in all 4 patients. However, hepatic encephalopathy occurred in one patient one week following the procedure. Though the follow-up period was not long enough for full evaluation, we found the transjugular intrahepatic portosystemic shunt was a safe and effective procedure for the control of variceal bleeding by lowering the portal pressure. For the appropriate application for this procedure, the optimal size of the shunt and optical degree of the resultant decompression are yet to be determined in the future.

  2. Utilização de probiótico e de lactulose no controle de hiperamonemia causada por desvio vascular portossistêmico congênito em um cão Use of probiotic and lactulose to control hyperammonemia secundary to a congenital portosystemic shunt in a dog

    Directory of Open Access Journals (Sweden)

    Alexandre Martini de Brum

    2007-04-01

    Full Text Available O desvio vascular portossistêmico pode ser congênito ou adquirido nos cães. A enfermidade pode levar a alterações neurológicas decorrentes de encefalopatia hepática e a hiperamonenia é um dos mecanismos implicados na fisiopatologia deste quadro. O tratamento clínico visa a reduzir os níveis séricos de amônia com o uso de antibióticos e lactulose. Em humanos com hepatopatias, os probióticos podem ser utilizados para reduzir a hiperamonemia. A resposta clínica e laboratorial de um cão com desvio vascular portossistêmico foi demonstrada com a utilização de lactulose e de probiótico, isoladamente e associados, sendo que a melhor evolução foi obtida na terapia conjunta.The portosystemic shunt may be congenital or acquired in dogs. The disease can cause neurologic signs manifested by hepatic encephalopathy. The hyperammonemia is a mechanism involved on the physiopathology of this disorder. The objective of the clinical treatment is to decrease the levels of ammonia with antibiotics and lactulose. In human medicine, probiotics are used to reduce the hyperammonemia in patients with hepatic diseases. This report compares the use of lactulose and probiotic monotherapy and in association in a dog with congenital portosystemic shunt, showing the patient’s clinical and laboratorial evolutions. The better clinical and laboratorial reponse was obtained with the association of lactulose and probiotic.

  3. Annular pancreas causing extrahepatic biliary obstruction

    International Nuclear Information System (INIS)

    Ogulin, M.; Jamar, B.

    2004-01-01

    Background. Annular pancreas is an uncommon congenital abnormality, consisting of a flat band of pancreatic tissue, which encircles duodenum or extrahepatic biliary duct. We present a case of obstructive jaundice, caused by annular pancreas. Case report. A 46 years old female was admitted because of a sudden onset of abdominal pain, vomiting and jaundice. For the last six years she occasionally noticed her skin was light yellow, in the last year she felt distension in the upper abdomen, especially after fatty meals. Conclusions. Two US examinations, the first one six months before the admission, showed dilated hepatic ducts. The reason of dilatation was unclear, even after the endoscopic US examination. At operation an almost complete obstruction of the common hepatic duct was found, caused by a narrow band of pancreatic tissue. (author)

  4. Biliary fibrosis in microsurgical extrahepatic cholestasis in the rat.

    Science.gov (United States)

    Sánchez-Patán, Fernando; Anchuelo, Raquel; Corcuera, María-Teresa; Casado, Isabel; Gómez-Aguado, Fernando; Aller, María-Angeles; Cruz, Arturo; Alonso, María-José; Arias, Jaime

    2008-01-01

    A new model of extrahepatic cholestasis, using a microsurgical technique, is performed as an alternative to the traditional model of the bile duct ligated-rat, in order to study the stage of fibrosis in the long-term. Male Wistar rats were divided into two groups: I (Sham-operated, n = 9) and II [Microsurgical Cholestasis (MC), n = 10]. After 4 weeks, portal pressure, types of portosystemic collateral circulation, mesenteric venous vasculopathy, hepatic function test, and liver histopathology were studied by using the Knodell index and fibrosis was determined by reticulin and Sirius red stains. The animals with MC presented portal hypertension with extrahepatic portosistemic collateral circulation, associated with mesenteric venous vasculopathy and increased plasma levels of bilirubin (6.30 +/- 1.80 vs. 0.22 +/- 0.37 mg/dL; P = 0.0001), alkaline phosphatase (293.00 +/- 82.40 vs. 126.30 +/- 33.42 U/L; P = 0.001), AST (380.00 +/- 78.50 vs. 68.33 +/- 11.74 IU/L; P = 0.0001), ALT (87.60 +/- 22.32 vs. 42.22 +/- 7.89 IU/L; P = 0.0001), and LDH (697.76 +/- 75.13 vs. 384.80 +/- 100.03 IU/L; P = 0.0001). On the contrary, plasma levels of albumin decreased (2.72 +/- 0.12 mg/dl vs. 2.99 +/- 0.10; P = 0.001). The microsurgical resection of the extrahepatic biliary tract in the rat produces an experimental model of hepatic inflammation, characterized by a high Knodell hepatic activity index (4), bile proliferation, and fibrosis.

  5. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

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    Full Text Available ... bear denotes child-specific content. Related Articles and Media Radiation Dose in X-Ray and CT Exams Contrast Materials Venography Images related to Transjugular Intrahepatic Portosystemic ...

  6. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

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  7. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

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    Full Text Available ... story about radiology? Share your patient story here Images × Image Gallery Radiologist and patient consultation. View full size ... X-Ray and CT Exams Contrast Materials Venography Images related to Transjugular Intrahepatic Portosystemic Shunt (TIPS) Sponsored ...

  8. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

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    Full Text Available ... Intrahepatic Portosystemic Shunt (TIPS)? What are some common uses of the procedure? How should I prepare? What does the equipment look like? How does the procedure work? How is the procedure performed? What will I ...

  9. Extrahepatic portal hypertension in childhood

    International Nuclear Information System (INIS)

    Takehara, Hiroo; Komi, Nobuhiko; Goh, Masahiro; Yoshida, Sadahiro; Kameoka, Kazuhiro; Hino, Masao; Sui, Osamu

    1986-01-01

    Four pediatric patients with extrahepatic portal hypertension had undergone portography and computerized radionuclide angiography in order to examine shape of portal system and to evaluate hepatic blood flow before and aftersurgical treatment. In all patients, cavernous transformation of portal system was demonstrated by portography, and in one of them spontaneous splenorenal shunt occurred. In 3 of them, who underwent esophageal transsection combined with paraesophagogastric devascularization and splenectomy, so-called direct operation, increase of portal blood flow was revealed by computerized radionuclide angiography. It is suggested that direct operation increasing portal blood flow after surgery is effective in treating extrahepatic portal hypertension in childhood. (author)

  10. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

    Medline Plus

    Full Text Available Toggle navigation Test/Treatment Patient Type Screening/Wellness Disease/Condition Safety En Español More Info Images/Videos About Us News Physician Resources Professions Site Index A-Z Transjugular Intrahepatic Portosystemic Shunt ( ...

  11. Transjugular Intrahepatic Portosystemic Shunt (TIPS)

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  12. Congenital absence of the portal vein presenting as pulmonary hypertension

    International Nuclear Information System (INIS)

    Jun, Sur Young; Lee, Whal; Cheon, Jung Eun; Kim, Woo Sun; Kim, In One; Yeon, Kyung Mo

    2007-01-01

    Congenital absence of the portal vein (CAPV) is a rare malformation in which intestinal and splenic venous flow bypasses the liver and drains directly into the systemic circulation via a congenital portosystemic shunt. We describe two cases of CAPV presenting as pulmonary hypertension that were initially suspected as primary pulmonary hypertension. However, subsequent ultrasonography and CT detected the absence of a portal vein and the presence of a portosystemic shunt. Pulmonary hypertension is a recognized complication of liver disease and portal hypertension. However, these two cases illustrate that CAPV may result in pulmonary hypertension without liver disease or portal hypertension

  13. Congenital absence of the portal vein presenting as pulmonary hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Jun, Sur Young; Lee, Whal; Cheon, Jung Eun; Kim, Woo Sun; Kim, In One; Yeon, Kyung Mo [Seoul National University Hospital, Seoul (Korea, Republic of)

    2007-11-15

    Congenital absence of the portal vein (CAPV) is a rare malformation in which intestinal and splenic venous flow bypasses the liver and drains directly into the systemic circulation via a congenital portosystemic shunt. We describe two cases of CAPV presenting as pulmonary hypertension that were initially suspected as primary pulmonary hypertension. However, subsequent ultrasonography and CT detected the absence of a portal vein and the presence of a portosystemic shunt. Pulmonary hypertension is a recognized complication of liver disease and portal hypertension. However, these two cases illustrate that CAPV may result in pulmonary hypertension without liver disease or portal hypertension.

  14. [Extrahepatic biliary atresia: diagnostic methods].

    Science.gov (United States)

    Cauduro, Sydney M

    2003-01-01

    To emphasize the importance of precocious diagnosis of extrahepatic biliary atresia and its direct relationship with the surgical re-establishment of the biliary flow before the second month of life. To discuss several complementary methods with the aim of selecting the ones that present better evidence, and avoiding delays in diagnosis and worse prognostic. Bibliographical researching regarding the period of 1985-2001, in Medline and MdConsult, using the following key words: neo-natal cholestasis; extrahepatic biliary atresia; neo-natal hepatitis. National and foreign articles were also elected based on the bibliography of consulted publications, and when necessary, for better understanding of the theme, opinions emitted in theses and textbooks were referred. The revision of the consulted bibliography led to the assumption that early diagnosis of EHBA and surgical treatment to reestablish the biliary flow up to 60 days of life are fundamental in order to achieve good results. Among several complementary methods of diagnosis, cholangiography by MR, US and the hepatic biopsy are the ones that provide the largest success indexes. The referring of patients bearers of EHBA to centers of references in Brazil, is still made tardily, probably due to lack of enlightenment of the doctors of primary attention, allied to bureaucratic and technological difficulties. The experience in England in relation to the "Yellow Alert" program, allowed that the number of children referred to surgical treatment before the 60 days of life increased significantly. Among the complementary methods, the MR cholangiography, ultrasonography and hepatic biopsy should be used, depending on the technological resources of the diagnosis units.

  15. Extrahepatic spread of hepatocellular carcinoma: a pictorial review

    International Nuclear Information System (INIS)

    Hong, Seong Sook; Kim, Tae Kyoung; Sung, Kyu-Bo; Kim, Pyo Nyun; Ha, Hyun Kwon; Kim, Ah Young; Lee, Moon-Gyu

    2003-01-01

    Although extrahepatic spread of hepatocellular carcinoma (HCC) is uncommon, it can be found anywhere in the body. Most extrahepatic metastases of HCC occur in patients with advanced-stage intrahepatic tumor, but incidental extrahepatic lesions have also occasionally been found in patients with early-stage intrahepatic HCC. The detection of extrahepatic metastatic disease is crucial when planning therapy for patients with HCC and should be used to avoid unnecessary surgical intervention. In this study we illustrate the radiologic findings of extrahepatic metastases of HCC involving various sites. The presumed mechanism of extrahepatic extension of HCC is also discussed. (orig.)

  16. Treatment of hepatic neoplasm through extrahepatic collaterals

    Energy Technology Data Exchange (ETDEWEB)

    Soo, C.S.; Chuang, V.P.; Wallace, S.; Charnsangavej, C.; Carrasco, H.

    1983-04-01

    Twenty-nine patients with hepatic artery occlusion were treated with additional hepatic infusion or embolization through extrahepatic collaterals. Seventeen courses of hepatic infusion were performed in 13 patients through the inferior pancreaticoduodenal artery, left gastric artery, or right gastric artery. Twenty-five hepatic embolization procedures were performed in 16 patients through the right and left phrenic arteries, left and right gastric arteries, pancreaticoduodenal artery, gastroduodenal artery, or omentoepiploic artery. In one patient gastric ulcers developed following left gastric artery infusion. No complication related to the embolization procedure was observed in the embolization group. The extrahepatic collaterals are important alternative routes for continuous transcatheter management of hepatic neoplasms following hepatic artery occlusion.

  17. Treatment of hepatic neoplasm through extrahepatic collaterals

    International Nuclear Information System (INIS)

    Soo, C.S.; Chuang, V.P.; Wallace, S.; Charnsangavej, C.; Carrasco, H.

    1983-01-01

    Twenty-nine patients with hepatic artery occlusion were treated with additional hepatic infusion or embolization through extrahepatic collaterals. Seventeen courses of hepatic infusion were performed in 13 patients through the inferior pancreaticoduodenal artery, left gastric artery, or right gastric artery. Twenty-five hepatic embolization procedures were performed in 16 patients through the right and left phrenic arteries, left and right gastric arteries, pancreaticoduodenal artery, gastroduodenal artery, or omentoepiploic artery. In one patient gastric ulcers developed following left gastric artery infusion. No complication related to the embolization procedure was observed in the embolization group. The extrahepatic collaterals are important alternative routes for continuous transcatheter management of hepatic neoplasms following hepatic artery occlusion

  18. Extrahepatic complications to cirrhosis and portal hypertension

    DEFF Research Database (Denmark)

    Møller, Søren; Henriksen, Jens H; Bendtsen, Flemming

    2014-01-01

    In addition to complications relating to the liver, patients with cirrhosis and portal hypertension develop extrahepatic functional disturbances of multiple organ systems. This can be considered a multiple organ failure that involves the heart, lungs, kidneys, the immune systems, and other organ...

  19. The extrahepatic manifestations of hepatitis B virus.

    Science.gov (United States)

    Baig, Saeeda; Alamgir, Mohiuddin

    2008-07-01

    Hepatitis B Virus (HBV) leads to a number of hepatic complications, from acute to chronic hepatitis, cirrhosis and hepatocellular carcinoma, is a well-established fact. Upcoming clinical research, over the years, associates numerous extrahepatic manifestations during the acute and chronic episodes of hepatitis B with significant morbidity and mortality. A causal relationship between HBV and serious autoimmune disorders has also been observed among certain susceptible vaccine recipients in a defined temporal period following immunization. The cause of these extrahepatic manifestations is generally believed to be immune mediated. The most commonly described include skin rash, arthritis, arthralgia, glomerulonephritis, polyarteritis nodosa, and papular acrodermatitis etc. The serum-sickness like "arthritis-dermatitis" prodrome has also been observed in approximately one-third of patients acquiring HBV infections. Skin manifestations of HBV infection typically present as palpable purpura reported to be caused by chronic HBV, although this association remains controversial. To consider the relationship between HBV and other clinically significant disorders as well as serious autoimmune disorders among certain vaccine recipients is the topic of this review. Variable factors that influence extrahepatic manifestation are discussed, including possible synergy between hepatitis B virus and the immune system.

  20. A Case of Adenomyomatous Hyperplasia of the Extrahepatic Bile Duct

    Directory of Open Access Journals (Sweden)

    Masakatsu Numata

    2011-08-01

    Full Text Available Adenomyomatous hyperplasia is rarely found in the extrahepatic bile duct. A 54-year-old man was referred to our center with a diagnosis of extrahepatic bile duct stenosis which had been detected by endoscopic retrograde choloangiopancreatography. Abdominal computed tomography revealed thickening of the wall of the middle extrahepatic bile duct, however no malignant cells were detected by cytology. Since bile duct carcinoma could not be ruled out, we performed resection of the extrahepatic duct accompanied by lymph node dissection. Histopathologically, the lesion was diagnosed as adenomyomatous hyperplasia of the extrahepatic bile duct. Present and previously reported cases showed the difficulty of making a diagnosis of adenomyomatous hyperplasia of the extrahepatic bile duct preoperatively or intraoperatively. Therefore, when adenomyomatous hyperplasia is suspected, a radical surgical procedure according to malignant disease may be necessary for definitive diagnosis.

  1. The transjugular intrahepatic portosystemic shunt (TIPS)

    International Nuclear Information System (INIS)

    Owen, A.R.; Stanley, A.J.; Vijayananthan, A.; Moss, J.G.

    2009-01-01

    The creation of an intrahepatic portosystemic shunt via a transjugular approach (TIPS) is an interventional radiological procedure used to treat the complications of portal hypertension. TIPS insertion is principally indicated to prevent or arrest variceal bleeding when medical or endoscopic treatments fail, and in the management refractory ascites. This review discusses the development and execution of the technique, with focus on its clinical efficacy. Patient selection, imaging surveillance, revision techniques, and complications are also discussed.

  2. The transjugular intrahepatic portosystemic shunt (TIPS)

    Energy Technology Data Exchange (ETDEWEB)

    Owen, A.R. [Department of Radiology, Austin Health, Heidelberg, Melbourne (Australia)], E-mail: andrewowen@doctors.org.uk; Stanley, A.J. [Department of Gastroenterology, Glasgow Royal Infirmary, Glasgow (United Kingdom); Vijayananthan, A. [Department of Biomedical Imaging, University of Malaya, Kuala Lumpur (Malaysia); Moss, J.G. [Department of Radiology, Gartnavel General Hospital, Glasgow (United Kingdom)

    2009-07-15

    The creation of an intrahepatic portosystemic shunt via a transjugular approach (TIPS) is an interventional radiological procedure used to treat the complications of portal hypertension. TIPS insertion is principally indicated to prevent or arrest variceal bleeding when medical or endoscopic treatments fail, and in the management refractory ascites. This review discusses the development and execution of the technique, with focus on its clinical efficacy. Patient selection, imaging surveillance, revision techniques, and complications are also discussed.

  3. Use of transsplenic injection of agitated saline and heparinized blood for the ultrasonographic diagnosis of macroscopic portosystemic shunts in dogs.

    Science.gov (United States)

    Gómez-Ochoa, Pablo; Llabrés-Díaz, Francisco; Ruiz, Sergio; Corda, Andrea; Prieto, Saul; Sosa, Ivan; Gregori, Tommaso; Gascón, Manuel; Couto, Guillermo C

    2011-01-01

    We describe the use of ultrasonography-guided percutaneous splenic injection of agitated saline and heparinized blood for the diagnosis of portosystemic shunts (PSS) in 34 dogs. Agitated saline mixed with 1 ml of heparinized autologous blood was injected into the spleen of 34 sedated dogs under sonographic guidance. The transducer was then sequentially repositioned to visualize the portal vein, the caudal vena cava, and the right atrium through different acoustic windows. It was possible to differentiate between intrahepatic and extrahepatic shunts depending on the entry point of the microbubbles into the caudal vena cava. Portoazygos shunts and portocaval shunts could be differentiated based on the presence of microbubbles in the caudal vena cava and/or the right atrium. In one dog, collateral circulation due to portal hypertension was identified. In dogs with a single extrahepatic shunt, the microbubbles helped identify the shunting vessel. The technique was also used postoperatively to assess the efficacy of shunt closure. All abnormal vessels were confirmed by exploratory laparotomy or with ultrasonographic identification of the shunting vessel. Ultrasound-guided transsplenic injection of agitated saline with heparinized blood should be considered as a valuable technique for the diagnosis of PSS; it is easy to perform, safe, and the results are easily reproducible.

  4. Extrahepatic biliary obstruction; postoperative morbidity and mortality

    International Nuclear Information System (INIS)

    Hussain, Z.; Khan, K.I.; Vaseem, M.; Rana, S.H.

    2010-01-01

    The objectives of this study are to evaluate the surgical management, both definitive and palliative, in selected patients with biliary obstruction and to find out the postoperative morbidity and mortality in these patients. Duration of the study is two years conducted from June 2002 to May 2004. The study was carried out at. the surgical. unit 4 of the Combined Military Hospital and surgical department of the Military Hospital. Thirty eight cases of biliary obstruction were included. A convenient sampling technique was followed. Data analyzed by using SPSS version 10.0 for windows on computer. Descriptive statistics like frequency, percentage, average etc were computed for data presentation. Any inferential test-was not found to be applicable for this descriptive type case series. We selected 38 patients with features of extrahepatic biliary obstruction. Out of these (n 38) 15 patients (39.5%) suffered from benign diseases while those having malignant diseases were 23 (60.5%). 19 (50%) patients died within two years of follow up while 19 (50%) were the survivors. Mortality was maximum for the malignant cases. In benign cases only one patient died. Maximum deaths 6 (31.6%) occurred in the period of up to one month of operation. 20 patients had one or another complication of operation and hence the morbidity came out to be 52%. According to our results the mortality and morbidity related to extrahepatic biliary obstruction in our patients was higher compared to other studies which can only be reduced by early detection and treatment. (author)

  5. Novel extrahepatic cytochrome P450s

    International Nuclear Information System (INIS)

    Karlgren, Maria; Miura, Shin-ichi; Ingelman-Sundberg, Magnus

    2005-01-01

    The cytochrome P450 enzymes are highly expressed in the liver and are involved in the metabolism of xenobiotics. Because of the initiatives associated with the Human Genome Project, a great progress has recently been seen in the identification and characterization of novel extrahepatic P450s, including CYP2S1, CYP2R1, CYP2U1 and CYP2W1. Like the hepatic enzymes, these P450s may play a role in the tissue-specific metabolism of foreign compounds, but they may also have important endogenous functions. CYP2S1 has been shown to metabolize all-trans retinoic acid and CYP2R1 is a major vitamin D 25-hydroxylase. Regarding their metabolism of xenobiotics, much remains to be established, but CYP2S1 metabolizes naphthalene and it is likely that these P450s are responsible for metabolic activation of several different kinds of xenobiotic chemicals and contribute to extrahepatic toxicity and carcinogenesis

  6. Risk factors for extrahepatic biliary tract carcinoma in men

    DEFF Research Database (Denmark)

    Ahrens, Wolfgang; Timmer, Antje; Vyberg, Mogens

    2007-01-01

    OBJECTIVES: To identify risk factors of carcinoma of the extrahepatic biliary tract in men. METHODS: Newly diagnosed and histologically confirmed patients, 35-70 years old, were interviewed between 1995 and 1997 in Denmark, Sweden, France, Germany and Italy. Population controls were frequency...... for extrahepatic biliary tract carcinoma in men (odds ratio 2.49; 95% confidence interval 1.32-4.70), particularly for gall bladder tumors (odds ratio 4.68; 95% confidence interval 1.85-11.84). For a body mass index [height (m) divided by squared weight (kg)] >30 at age 35 years, an excess risk was observed (odds...... as a strong risk factor for extrahepatic biliary tract carcinoma, whereas we did not find any strong lifestyle-associated risk factors. Inconsistent results across studies concerning the association of extrahepatic biliary tract carcinoma with overweight and obesity may be explained by the different...

  7. Portosystemic pressure reduction achieved with TIPPS and impact of portosystemic collaterals for the prediction of the portosystemic-pressure gradient in cirrhotic patients

    Energy Technology Data Exchange (ETDEWEB)

    Grözinger, Gerd, E-mail: gerd.groezinger@med.uni-tuebingen.de [Department of Diagnostic Radiology, Department of Radiology, University of Tübingen (Germany); Wiesinger, Benjamin; Schmehl, Jörg; Kramer, Ulrich [Department of Diagnostic Radiology, Department of Radiology, University of Tübingen (Germany); Mehra, Tarun [Department of Dermatology, University of Tübingen (Germany); Grosse, Ulrich; König, Claudius [Department of Diagnostic Radiology, Department of Radiology, University of Tübingen (Germany)

    2013-12-01

    Purpose: The portosystemic pressure gradient is an important factor defining prognosis in hepatic disease. However, noninvasive prediction of the gradient and the possible reduction by establishment of a TIPSS is challenging. A cohort of patients receiving TIPSS was evaluated with regard to imaging features of collaterals in cross-sectional imaging and the achievable reduction of the pressure gradient by establishment of a TIPSS. Methods: In this study 70 consecutive patients with cirrhotic liver disease were retrospectively evaluated. Patients received either CT or MR imaging before invasive pressure measurement during TIPSS procedure. Images were evaluated with regard to esophageal and fundus varices, splenorenal collaterals, short gastric vein and paraumbilical vein. Results were correlated with Child stage, portosystemic pressure gradient and post-TIPSS reduction of the pressure gradient. Results: In 55 of the 70 patients TIPSS reduced the pressure gradient to less than 12 mmHg. The pre-interventional pressure and the pressure reduction were not significantly different between Child stages. Imaging features of varices and portosystemic collaterals did not show significant differences. The only parameter with a significant predictive value for the reduction of the pressure gradient was the pre-TIPSS pressure gradient (r = 0.8, p < 0.001). Conclusions: TIPSS allows a reliable reduction of the pressure gradient even at high pre-interventional pressure levels and a high collateral presence. In patients receiving TIPSS the presence and the characteristics of the collateral vessels seem to be too variable to draw reliable conclusions concerning the portosystemic pressure gradient.

  8. Portosystemic pressure reduction achieved with TIPPS and impact of portosystemic collaterals for the prediction of the portosystemic-pressure gradient in cirrhotic patients

    International Nuclear Information System (INIS)

    Grözinger, Gerd; Wiesinger, Benjamin; Schmehl, Jörg; Kramer, Ulrich; Mehra, Tarun; Grosse, Ulrich; König, Claudius

    2013-01-01

    Purpose: The portosystemic pressure gradient is an important factor defining prognosis in hepatic disease. However, noninvasive prediction of the gradient and the possible reduction by establishment of a TIPSS is challenging. A cohort of patients receiving TIPSS was evaluated with regard to imaging features of collaterals in cross-sectional imaging and the achievable reduction of the pressure gradient by establishment of a TIPSS. Methods: In this study 70 consecutive patients with cirrhotic liver disease were retrospectively evaluated. Patients received either CT or MR imaging before invasive pressure measurement during TIPSS procedure. Images were evaluated with regard to esophageal and fundus varices, splenorenal collaterals, short gastric vein and paraumbilical vein. Results were correlated with Child stage, portosystemic pressure gradient and post-TIPSS reduction of the pressure gradient. Results: In 55 of the 70 patients TIPSS reduced the pressure gradient to less than 12 mmHg. The pre-interventional pressure and the pressure reduction were not significantly different between Child stages. Imaging features of varices and portosystemic collaterals did not show significant differences. The only parameter with a significant predictive value for the reduction of the pressure gradient was the pre-TIPSS pressure gradient (r = 0.8, p < 0.001). Conclusions: TIPSS allows a reliable reduction of the pressure gradient even at high pre-interventional pressure levels and a high collateral presence. In patients receiving TIPSS the presence and the characteristics of the collateral vessels seem to be too variable to draw reliable conclusions concerning the portosystemic pressure gradient

  9. Preoperative intraluminal irradiation of the extrahepatic bile duct tumor

    International Nuclear Information System (INIS)

    Kamada, Tadashi; Tsujii, Hirohiko; Arimoto, Takuro; Irie, Goro.

    1991-01-01

    From 1984 through 1986, six patients with extrahepatic bile duct tumor were treated preoperatively with intraluminal irradiation of the bile duct. There were no unresectable cases and pathological examination of the surgical specimens showed moderate to remarkable tumor regression in all cases. Postoperative biliary tract hemorrhage occurred in 2 of 3 patients who received 60 Gy at a point 7.5 mm from the center of the source. With accurate preoperative diagnosis of the tumor extent and careful setting of the target area of intraluminal irradiation, improved local tumor control of extrahepatic bile duct tumor can be expected with this method. (author)

  10. The Effects of Travel Burden on Outcomes After Resection of Extrahepatic Biliary Malignancies: Results from the US Extrahepatic Biliary Consortium.

    Science.gov (United States)

    O'Connor, Sean C; Mogal, Harveshp; Russell, Gregory; Ethun, Cecilia; Fields, Ryan C; Jin, Linda; Hatzaras, Ioannis; Vitiello, Gerardo; Idrees, Kamran; Isom, Chelsea A; Martin, Robert; Scoggins, Charles; Pawlik, Timothy M; Schmidt, Carl; Poultsides, George; Tran, Thuy B; Weber, Sharon; Salem, Ahmed; Maithel, Shishir; Shen, Perry

    2017-12-01

    Surgical resection of extrahepatic biliary malignancies has been increasingly centralized at high-volume tertiary care centers. While this has improved outcomes overall, increased travel burden has been associated with worse survival for many other malignancies. We hypothesized that longer travel distances are associated with worse outcomes for these patients as well. Data was analyzed from the US Extrahepatic Biliary Consortium database, which retrospectively reviewed patients who received resection of extrahepatic biliary malignancies at 10 high-volume centers. Driving distance to the patient's treatment center was measured for 1025 patients. These were divided into four quartiles for analysis: travel distances were associated with decreased overall survival, especially in the 3rd quartile of our study. Patients traveling longer distances also had a lower household income, suggesting that these patients have significant barriers to care.

  11. Prevention and treatment of complications after transjugular intrahepatic portosystemic shunt

    Directory of Open Access Journals (Sweden)

    XUE Hui

    2016-02-01

    Full Text Available The application of transjugular intrahepatic portosystemic shunt (TIPS in the treatment of cirrhotic portal hypertension has been widely accepted both at home and abroad. This article focuses on the fatal complications of TIPS (including intraperitoneal bleeding and acute pulmonary embolism, shunt failure, and recurrent portosystemic hepatic encephalopathy, and elaborates on the reasons for such conditions and related preventive measures, in order to improve the accuracy and safety of intraoperative puncture, reduce common complications such as shunt failure and hepatic encephalopathy, and improve the clinical effect of TIPS in the treatment of cirrhotic portal hypertension.

  12. Radiation therapy in extrahepatic bile duct carcinoma

    International Nuclear Information System (INIS)

    Mahe, Marc; Romestaing, Pascale; Talon, Bernard; Ardiet, J.M.; Salerno, Nathalie; Sentenac, Irenee; Gerard, J.P.

    1991-01-01

    Fifty-one patients with carcinoma of the extrahepatic bile ducts (EHBD) received radiation therapy between Jan 1980-Dec 1988. The location of the tumors was: proximal third, 20 patients; middle third, 23; distal third, 3; diffuse, 5 patients. Thirty-six patients underwent surgery with complete gross resection in 14 (10/14 with positive margins), incomplete gross resection in 12 and only biopsy in 10. Fifteen patients had only biliary drainage without laparotomy after cytologic diagnosis of malignancy in 11/15. Radiation therapy was done with curative intent after complete or incomplete resection (n=26) and it was palliative in patients who had no resection or only biliary drainage (n=25). Twenty-five patients received external radiation-therapy (ERT) alone to the tumor and lymph nodes (mean dose 45 Gy/2Gy per fraction for cure, 35 Gy/10 fractions for palliation), 8 patients had only iridium-192 ( 192 Ir) implant (50-60 Gy at a 1 cm radius for cure, 30 Gy for palliation), 17 patients had both ERT + 192 Ir(ERT 42.5 Gy + 192 Ir 10-15 Gy for cure; ERT 20 Gy/5 fractions + 192 Ir 20-30 Gy for palliation) and one intra-operative irradiation + ERT. The overall survival rate for the entire group was 55, 28.5 and 15 percent at 12, 24, 36 months and median survival 12 months. Median survival was 22 months in patients treated with curative intent and only 10 months after palliative treatment (p0.03). Among patients who had curative treatment, median survival was 27.5 months after complete gross resection and 13 months after incomplete gross resection (p0.045). After complete gross resection 5/14 patients were alive without evolutive disease at 11, 19, 20, 23 and 41 months, 2 were alive with metastases at 25 and 27 months and 7/14 died of cancer from 7 to 59 months. The rate of complications was low: 3 cholangitis responsive to antibiotics, 1 hemobilia and 2 gastric ulcers. These results are encouraging especially for patients with complete gross resection but they must be

  13. Congenital Hypothyroidism

    Science.gov (United States)

    ... Disease Featured Resource Find an Endocrinologist Search Congenital Hypothyroidism March 2012 Download PDFs English Espanol Editors Rosalind S. ... Resources MedlinePlus (NIH) Mayo Clinic What is congenital hypothyroidism? Newborn babies who are unable to make enough ...

  14. Extrahepatic manifestations of cholestatic liver diseases: pathogenesis and therapy

    NARCIS (Netherlands)

    Pusl, Thomas; Beuers, Ulrich

    2005-01-01

    Pruritus, fatigue, and metabolic bone disease are frequent complications of cholestatic liver diseases, which can be quite distressing for the patient and can considerably reduce the quality of life. The molecular pathogenesis of these extrahepatic manifestations of cholestasis is poorly understood,

  15. Cholangiopathy in extrahepatic portal venous obstruction: radiological appearances

    International Nuclear Information System (INIS)

    Nagi, B.; Kochhar, R.; Bhasin, D.; Singh, K.

    2000-01-01

    To evaluate cholangiographic abnormalities resulting from extrahepatic portal venous obstruction (EHPVO) by sonography and endoscopic retrograde cholangiopancreaticography (ERCP). Material and Methods: Forty-three patients with an established diagnosis of EHPVO were subjected to duplex Doppler sonography and ERCP. Of these, 8 patients had obstructive jaundice. Results: Dilated common bile duct with pericholedochal varices showing a continuous type of flow pattern was seen in 5 EHPVO patients with obstructive jaundice. ERCP revealed cholangiographic abnormalities in 40 patients (93%). Extrahepatic bile ducts were involved in 100% of cases compared to intrahepatic bile ducts (57%). Abnormalities noted were contour irregularity with indentations, displacement and angulation, strictures and filling defects in the extrahepatic ductal system. Intrahepatic bile ducts showed dilatation with areas of narrowing and filling defects. Conclusion: Cholangiographic abnormalities are very common in patients with EHPVO, even without clinical manifestations of biliary disease. Extrahepatic bile ducts are far more often involved compared to intrahepatic bile ducts. Extrinsic compression with contour irregularity is the most common cholangiographic finding. Sonographic findings are not diagnostic. Dilated common bile duct with pericholedochal varices was seen in only 5 patients

  16. Diffusion-weighted MR imaging for detection of extrahepatic cholangiocarcinoma

    International Nuclear Information System (INIS)

    Cui, Xing-Yu; Chen, Hong-Wei; Cai, Song; Bao, Jian; Tang, Qun-Feng; Wu, Li-Yuan; Fang, Xiang-Ming

    2012-01-01

    Objectives: To measure the sensitivity of diffusion-weighted imaging (DWI) and determine the most appropriate b value for DWI; to explore the correlation between the apparent diffusion coefficient (ADC) value and the degree of extrahepatic cholangiocarcinoma differentiation. Methods: Preoperative diffusion-weighted imaging and magnetic resonance examinations were performed for 31 patients with extrahepatic cholangiocarcinoma. Tumor ADC values were measured, and the signal-to-noise ratio, contrast-to-noise ratio, and signal-intensity ratio between the diffusion-weighted images with various b values as well as the T2-weighted images were calculated. Pathologically confirmed patients were pathologically graded to compare the ADC value with different b values of tumor at different degrees of differentiation, and the results were statistically analyzed by using the Friedman test. Results: A total of 29 cases of extrahepatic cholangiocarcinoma were detected by DWI. As the b value increased, tumor signal-to-noise ratio and contrast-to-noise ratio between the tumor and normal liver gradually decreased, but the tumor signal-intensity ratio gradually increased. When b = 800 s/mm 2 , contrast-to-noise ratio between tumor and normal liver, tumor signal-intensity ratio, and tumor signal-to-noise ratio of diffusion-weighted images were all higher than those of T2-weighted images; the differences were statistically significant (P 2 was the best in DWI of extrahepatic cholangiocarcinoma; the lesion ADC value declined as the degree of cancerous tissue differentiation decreased.

  17. An unusual case of intrahepatic portosystemic venous shunt

    African Journals Online (AJOL)

    vena cava (IVC) (most common). Intrahepatic portosystemic venous shunts are rare vascular anomalies that may be detected in asymptomatic patients, given the recent advances in radiological imaging techniques. Accurate shunt evaluation and classification can be performed with ultrasound and multi-detector computed.

  18. Consequences of extrahepatic manifestations of hepatitis C viral infection (HCV

    Directory of Open Access Journals (Sweden)

    Agnieszka Pawełczyk

    2016-04-01

    Full Text Available The hepatitis C virus (HCV is a primarily hepatotropic virus. However, numerous extrahepatic symptoms are observed in patients chronically infected with HCV, e.g. cryoglobulinemia, lymphoproliferative disorders, kidney diseases, disturbances of the central and peripheral nervous system, thyroid gland, pancreas, lymph nodes and pituitary gland, that develop at various times after the infection. Complex mechanisms underlie these processes, both molecular, related to direct effects of the virus on cells or tissues and indirect mechanisms, resulting from the response of the immune system to infection (via cytokines or oxidative stress, and from the antiviral treatment used. Understanding these mechanisms may contribute to the definition of new prognostic factors, important for the early diagnosis of the infection, which in turn may improve treatment efficacy.This paper is a review of the incidence of selected extrahepatic manifestations of HCV infection and their underlying pathogenetic mechanisms and risk factors.

  19. [Surgical treatment of extrahepatic portal hypertension in children].

    Science.gov (United States)

    Sevriugov, B L; Ulŕikh, E V; Korolev, M P; Kupatadze, D D; Ivanov, A P; Nabokov, V V

    1994-08-01

    In the period from 1985 to 1992 sixty-three patients aged from 7 months to 15 years were treated for the portal hypertension syndrome, 60 had the extrahepatie form. Forty-five various operations for portosystemic shunting were carried out: formation of proximal splenorenal anastomosis in 31, distal splenorenal anastomosis in 4, mesentericocaval anastomosis in 6, gastrocaval anastomisis in 2, and an atypical vascular shunt in 2 cases. Nonshunting operations were performed on 8 patients. Since 1986 39 sessions of endoscopic sclerotherapy were conducted, 16 of them were carried out in cases with esophagogastric bleeding.

  20. Aneurysmal malformation of the extrahepatic portal vein: a case report

    International Nuclear Information System (INIS)

    Lee, Jeong Min; Kang, Sung Soo; Kim, Chong Soo; Han, Hyeun Young

    1999-01-01

    Aneurysmal malformation of the portal vein is a rare entity. To our knowledge, only scattered reports of portal vein aneurysms appear in the literature in English, and there is no previously published report in Korean. We describe a case exhibiting aneurysmal malformation of the extrahepatic portal vein at the hepatic hilum ; the findings demonstrated by ultrasound, CT and angiography are discussed, a review of previously described cases is included

  1. Successful surgical management of an extrahepatic biliary cystadenocarcinoma

    Directory of Open Access Journals (Sweden)

    Márcio Lobo Guimarães

    2011-10-01

    Full Text Available Extrahepatic bile duct cancer is an uncommon disease, and few cases are curable by surgery. We report a case of extrahepatic biliary cystadenocarcinoma (BCAC associated with atrophy of the left hepatic lobe. A 54-year old male was admitted with painless obstructive jaundice and a hepatic palpable mass noticed one month before presentation. Liver functions tests were consistent with cholestatic damage and serum carbohydrate antigen 19.9 (CA 19-9 was increased before treatment. Magnetic resonance imaging (MRI disclosed dilatation of the left hepatic bile duct with irregular wall thickening close to the hepatic confluence, and atrophy of left hepatic lobe. The patient was submitted to en bloc extended left hepatectomy with resection of caudate lobe, hilar lymphadenectomy, and suprapancreatic biliary tree resection. All surgical margins were grossly negative, and postoperative course was uneventful, except for a minor bile leak. The patient was discharged on the 15th postoperative day; he is alive without tumor recurrence one year after primary therapy. Although technically challenging, extended en bloc resection is feasible in adults with extrahepatic BCAC and can improve survival with acceptable and manageable morbidity.

  2. Congenital tuberculosis

    African Journals Online (AJOL)

    Prof Ezechukwu

    2012-06-20

    Jun 20, 2012 ... Key words: Congenital tuberculo- sis, case report, miliary tuberculosis. Introduction. Congenital tuberculosis defines tuberculosis in infants of .... tary TB and otitis media, resulting in seizures, deafness, and death. It is therefore not surprising that the index case who presented at twelve weeks of age, had ...

  3. Congenital Abnormalities

    Science.gov (United States)

    ... tube defects. However, there is also a genetic influence to this type of congenital anomaly. Unknown Causes The vast majority of congenital abnormalities have no known cause. This is particularly troubling for parents who plan to have more children, because there is no way to predict if ...

  4. Multidetector CT portal venography in evaluation of portosystemic collateral vessels

    International Nuclear Information System (INIS)

    Agarwal, A.; Jain, M.

    2008-01-01

    This essay shows the usefulness of multidetector CT angiography for evaluation of the splenoportal venous system, which is essential in the management of patients with portal hypertension and its complications, such as portal vein thrombosis. By providing scanning with reconstruction of thin axial source images and reformatting into thicker multiplanar reformats, multidetector CT can help to determine the extent and location of portosystemic collateral vessels in patients with portal hypertension and is probably the optimal imaging technique in this setting.

  5. Frequency of helicobacter pylori antibodies in porto-systemic encephalopathy,

    International Nuclear Information System (INIS)

    Sethar, G.H.; Ahmed, R.; Afsar, S.; Zuberi, B.F.

    2004-01-01

    Objective: To study the frequency of Helicobacter pylori antibodies in patients presenting with porto-systemic encephalopathy due to liver disease. Patients and Methods: During the study period, seventy-six patients of porto-systemic encephalopathy due to liver diseases was selected. These subjects were evaluated for hepatic encephalopathy grade, modified Child-Pugh classification and were managed according to the standard practices. These patients were evaluated for Helicobacter (H. pylori) antibody status by ELlSA (Abbott Laboratories) method. Results: Out of 76 patients studied and tested for H. pylori antibodies, 48(63.2%) were males and 28(36.8%) were females with age ranging between 17 and 85 years. Out of 76 patients who presented with porto-systemic encephalopathy, 59(77.6%) had a positive H. pylori antibody test. Thirty-five of these were males and 24 were females. A significant number of patients who presented with higher grade of encephalopathy were H. pylori antibody positive (p<0.001). Conclusion: In this study, frequency of H. pylori antibodies was significantly high in patients of porto-systematic encephalopathy. (author)

  6. Role of the transjugular intrahepatic portosystemic shunt in the management of severe complications of portal hypertension in idiopathic noncirrhotic portal hypertension.

    Science.gov (United States)

    Bissonnette, Julien; Garcia-Pagán, Juan Carlos; Albillos, Agustín; Turon, Fanny; Ferreira, Carlos; Tellez, Luis; Nault, Jean-Charles; Carbonell, Nicolas; Cervoni, Jean-Paul; Abdel Rehim, Mohamed; Sibert, Annie; Bouchard, Louis; Perreault, Pierre; Trebicka, Jonel; Trottier-Tellier, Félix; Rautou, Pierre-Emmanuel; Valla, Dominique-Charles; Plessier, Aurélie

    2016-07-01

    Idiopathic noncirrhotic portal hypertension is a heterogeneous group of diseases characterized by portal hypertension in the absence of cirrhosis. The efficacy and safety of transjugular intrahepatic portosystemic shunt (TIPS) in this population are unknown. The charts of patients with idiopathic noncirrhotic portal hypertension undergoing TIPS in seven centers between 2000 and 2014 were retrospectively reviewed. Forty-one patients were included. Indications for TIPS were recurrent variceal bleeding (n = 25) and refractory ascites (n = 16). Patients were categorized according to the presence (n = 27) or absence (n = 14) of significant extrahepatic comorbidities. Associated conditions were hematologic, prothrombotic, neoplastic, immune, and exposure to toxins. During follow-up (mean 27 ± 29 months), variceal rebleeding occurred in 7/25 (28%), including three with early thrombosis of the stent. Post-TIPS overt hepatic encephalopathy was present in 14 patients (34%). Eleven patients died, five due the liver disease or complications of the procedure and six because of the associated comorbidities. The procedure was complicated by hemoperitoneum in four patients (10%), which was fatal in one case. Serum creatinine (P = 0.005), ascites as indication for TIPS (P = 0.04), and the presence of significant comorbidities (P = 0.01) at the time of the procedure were associated with death. Mortality was higher in patients with significant comorbidities and creatinine ≥100 μmol/L (P portal hypertension who have normal kidney function or do not have severe extrahepatic conditions, TIPS is an excellent option to treat severe complications of portal hypertension. (Hepatology 2016;64:224-231). © 2016 by the American Association for the Study of Liver Diseases.

  7. Use of 99mTCO4(-) trans-splenic portal scintigraphy for diagnosis of portosystemic shunts in 28 dogs.

    Science.gov (United States)

    Morandi, Federica; Cole, Robert C; Tobias, Karen M; Berry, Clifford R; Avenell, James; Daniel, Gregory B

    2005-01-01

    Ultrasound-guided percutaneous trans-splenic portal scintigraphy (TSPS) using 99mTcO4(-) has been used to image the portal venous system in normal dogs. Compared with per-rectal portal scintigraphy, it provides higher count density, consistent nuclear venograms of the splenic and portal vein, and significantly decreased radiation exposures. This paper describes the use of TSPS for the diagnosis of portosystemic shunts in 28 dogs. TSPS was performed injecting 70 +/- 28 MBq of 99mTcO4(-) (mean +/- SD) into the splenic parenchyma with ultrasound guidance. A dynamic acquisition at a frame rate of four frames/s for 5 min was initiated after placement of the needle and approximately 2s prior to injection. All dogs had diagnoses confirmed via exploratory laparotomy or ultrasonographic identification of the shunting vessel(s). Three studies (10.7%) were nondiagnostic because of intraperitoneal rather than intrasplenic injection of the radionuclide. Three pathways were recognized on the scintigraphic images: (1) portoazygos shunts--the 99mTcO4(-) bolus traveled dorsally, running parallel to the spine and entering the heart craniodorsally; (2) single portocaval or splenocaval shunts--the 99mTcO4(-) bolus ran from the area of the portal vein/splenic vein junction in a linear fashion toward the caudal vena cava entering the heart caudally; (3) internal thoracic shunt-the 99mTcO4 bolus traveled ventrally along the thorax and abdomen entering the cranial aspect of the heart. Single and multiple shunts were easily distinguished. There were no distinguishing features between single intra and extrahepatic portocaval shunts.

  8. Expression of Her-2/neu in extrahepatic cholangiocarcinoma

    Directory of Open Access Journals (Sweden)

    Shamekh R

    2017-02-01

    Full Text Available Rania Shamekh,1,* Marilin Rosa,2,* Zena Sayegh,2 Masoumeh Ghayouri,2 Richard Kim,3 Mokenge P Malafa,3 Domenico Coppola2 1Department of Pathology, University of South Florida, 2Department of Anatomic Pathology, 3Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL, USA *These authors contributed equally to this work Background: Receptor tyrosine-protein kinase erbB-2, which is also frequently called human epidermal growth factor receptor-2 (Her-2 or Her-2/neu, has been found to be overexpressed in various human cancers.Hypothesis: The aim of this pilot study was to explore the frequency of Her-2/neu gene amplification and protein expression in extrahepatic cholangiocarcinoma (EHBC. We used the World Health Organization classification criteria for EHBC.Materials and methods: This was a retrospective study using 88 tissue samples, including 45 samples from non-neoplastic biliary tissue (NNB and 43 samples of extrahepatic cholangiocarcinoma (EHBC. A tissue microarray including NNB and EHBC was constructed and analyzed by immunohistochemistry (IHC and dual in situ hybridization for Her-2/neu protein expression and amplification, respectively. The Her-2/neu expression was scored following the guidelines used for the ToGA study.Results: All NNB samples and all but one EHBC samples showed no expression of Her-2/neu by IHC. The one EHBC case immunohistochemically positive for Her-2/neu had an IHC score of 3+. Her-2/neu gene amplification was present in two EHBC samples only and included the case found to be positive by IHC.Conclusion: Our findings are similar to those reported in the literature. Although Her-2/neu overexpression has been documented in many types of cancer, Her-2/neu protein overexpression tends to have no role in the development and/or progression of EHBC. Keywords: extrahepatic, cholangiocarcinoma, Her-2/neu, ToGA, immunohistochemistry

  9. External Beam Radiotherapy for Carcinoma of the Extrahepatic Biliary System

    International Nuclear Information System (INIS)

    Chun, Ha Chung; Lee, Myung Za

    1996-01-01

    Purpose : To evaluate the effectiveness and tolerance of patients of external beam radiotherapy of carcinoma of the extrahepatic biliary system (EHBS) including gall bladder (GB) and extrahepatic bile ducts (EHBD) and to define the role of radiotherapy for these tumors. Methods and Materials : We retrospectively analyzed the records of 43 patients with carcinoma of the EHBS treated with external beam radiotherapy at our institution between April, 1986 and July, 1994. Twenty three patients had GB cancers and remaining 20 patients did EHBD cancers. Of those 23 GB cancers, 2 had Stage II, 12 did Stage III and 9 did Stage IV disease, respectively. Male to female ratio was 11 to 12. Fifteen patients underwent radical surgery with curative intent and 8 patients did biopsy and bypass surgery alone. Postoperatively 16 patients were irradiated with 4500 cGy or higher doses and 4 patients with 3180 to 4140 cGy. Follow up periods ranged from 8 to 34 months. Results : overall median survival time of patients with GB cancer was 11 months. Median survival time for patients with Stage III and IV disease were 14 months and 5 months, respectively. Corresponding two year survival rates were 36%(4/11) and 13%(1/8), respectively. Those who underwent surgery with curative intent showed significantly better survival at 12 months than those who underwent bypass surgery alone(67% vs 13%). None of the patients died of treatment related complications. Median survival time for entire group of 20 EHBD patients was 10 months. Median survivals of 10 Stage III and 7 Stage IV disease were 10 and 8 months, respectively. Two patients who underwent Whipple's procedure had 11 and 14 month survival and those treated with resection and drainage showed median survival of 10 month. Conclusion : Postoperative external beam radiotherapy for carcinoma of the extrahepatic billary system is well tolerated and might improve survival of patients. especially those with respectable lesions with microscopic or

  10. Congenital rubella

    Science.gov (United States)

    ... that usually closes shortly after birth remains open ( patent ductus arteriosus ) Narrowing of the large artery that ... prior to pregnancy can prevent congenital rubella. Pregnant women who have not had the vaccine should avoid ...

  11. Unilocular extrahepatic biliary cystadenoma mimicking choledochal cyst: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ju Hyun; Lee, Dong Ho; Kim, Hyoung Jung; Ko, Young Tae; Lim, Joo Won; Yang, Moon Ho [Kyunghee University Hospital, Seoul (Korea, Republic of)

    2004-12-15

    We report here on a case of extrahepatic biliary cystadenoma arising from the common hepatic duct. A 42-year-old woman was evaluated by us to find the cause of her jaundice. Ultrasonography and CT showed a cystic dilatation of the common hepatic duct and also marked dilatation of the intrahepatic duct. Direct cholangiography demonstrated a large filling defect between the left hepatic duct and the common hepatic duct; dilatation of the intrahepatic duct was also demonstrated. Following excision of the cystic mass, it was pathologically confirmed as a unilocular biliary mucinous cystadenoma arising from the common hepatic duct.

  12. Abnormalities of intrahepatic bile ducts in extrahepatic biliary atresia.

    Science.gov (United States)

    Raweily, E A; Gibson, A A; Burt, A D

    1990-12-01

    The infantile cholangiopathies are a group of conditions associated with neonatal jaundice, which include extrahepatic biliary atresia, paucity of intra-hepatic bile ducts and disorders associated with persistence of fetal biliary structures, the so-called ductal plate malformations. Although previously regarded as distinct entities, it has recently been suggested that they may represent parts of a disease spectrum in which the principal process is one of bile duct destruction, the morphological manifestations in individual cases being influenced by the stage of intra-uterine development at which such injury occurs and by the site within the biliary system at which there is maximum damage. To further examine this concept, we have studied liver biopsy specimens from 37 neonates with extrahepatic biliary atresia, with particular reference to abnormalities of the intrahepatic bile ducts. Paucity of intrahepatic ducts, defined as a bile duct: portal tract ratio of less than 0.9, was identified in six cases (16.2%). In eight cases (21.6%) we found concentric tubular ductal structures similar to those observed in ductal plate malformations. In one case, both abnormalities could be demonstrated. Our findings support the concept that there is overlap between the various types of infantile cholangiopathy.

  13. Extrahepatic manifestations associated with Chronic Hepatitis C Virus Infection

    Directory of Open Access Journals (Sweden)

    A. Flores-Chávez

    Full Text Available Summary Chronic hepatitis C virus (HCV infection has been associated with both organ-specific and systemic autoimmune diseases, with cryoglobulinemia being the most frequent associated disease. Experimental, virologic, and clinical evidence have demon-strated a close association between HCV infection and some systemic autoimmune diseases, especially Sjögren’s syndrome, but also rheumatoid arthritis and lupus. A higher prevalence of hematological processes has also been described in patients with HCV infection, including cytopenias and lymphoproliferative disorders (B-cell lymphoma. In addition, patients with chronic HCV infection have a higher frequency of other extrahepatic manifestations including endocrine, metabolic and cardiovascular disorders that may worse the prognosis of patients, along with neuropsychiatric manifestations and general symptoms that have a significant influence on the quality of life of the patient. Direct-acting antiviral therapies (DAAs that have recently begun to be used are providing the opportunity to effectively cure chronic HCV infection and reduce the burden of both hepatic and extrahepatic complications.

  14. Microwell Scaffolds for the Extrahepatic Transplantation of Islets of Langerhans

    Science.gov (United States)

    Buitinga, Mijke; Truckenmüller, Roman; Engelse, Marten A.; Moroni, Lorenzo; Ten Hoopen, Hetty W. M.; van Blitterswijk, Clemens A.; de Koning, Eelco JP.; van Apeldoorn, Aart A.; Karperien, Marcel

    2013-01-01

    Allogeneic islet transplantation into the liver has the potential to restore normoglycemia in patients with type 1 diabetes. However, the suboptimal microenvironment for islets in the liver is likely to be involved in the progressive islet dysfunction that is often observed post-transplantation. This study validates a novel microwell scaffold platform to be used for the extrahepatic transplantation of islet of Langerhans. Scaffolds were fabricated from either a thin polymer film or an electrospun mesh of poly(ethylene oxide terephthalate)-poly(butylene terephthalate) (PEOT/PBT) block copolymer (composition: 4000PEOT30PBT70) and were imprinted with microwells, ∼400 µm in diameter and ∼350 µm in depth. The water contact angle and water uptake were 39±2° and 52.1±4.0 wt%, respectively. The glucose flux through electrospun scaffolds was three times higher than for thin film scaffolds, indicating enhanced nutrient diffusion. Human islets cultured in microwell scaffolds for seven days showed insulin release and insulin content comparable to those of free-floating control islets. Islet morphology and insulin and glucagon expression were maintained during culture in the microwell scaffolds. Our results indicate that the microwell scaffold platform prevents islet aggregation by confinement of individual islets in separate microwells, preserves the islet’s native rounded morphology, and provides a protective environment without impairing islet functionality, making it a promising platform for use in extrahepatic islet transplantation. PMID:23737999

  15. congenital Biliary atresia

    African Journals Online (AJOL)

    embryonic form), comprising 10-35% of cases2. The pathology of the extrahepatic biliary system widely varies in these ... hepatic duct, with cystic structures found in the porta hepatis. .... Nelson Textbook of. Paediatrics 15th edition : Chapter 302.

  16. Carcinosarcoma of the Extrahepatic Bile Duct Presenting with Stone-like Radiological Findings.

    Science.gov (United States)

    Kumei, Shinsuke; Onishi, Yutaka; Ogura, Takeshi; Kusumoto, Chosei; Matsuno, Yasuko; Nishigami, Takashi; Maeda, Mitsuo; Harada, Masaru

    2015-01-01

    A 73-year-old woman was referred to our hospital due to epigastralgia and jaundice. The radiological findings showed a stone-like tumor in the extrahepatic bile duct. The patient was initially thought to have adenocarcinoma of the bile duct based on the findings of a pathological examination of the bile duct biopsy specimen and underwent pancreaticoduodenectomy; the final diagnosis of the lesion was so-called carcinosarcoma of the extrahepatic bile duct. She died of liver metastasis six months after the surgery. This case suggests that surgical resection is not adequate for achieving a radical cure, and the optimal treatment for extrahepatic bile duct carcinosarcoma should be established immediately.

  17. Congenital amusias.

    Science.gov (United States)

    Tillmann, B; Albouy, P; Caclin, A

    2015-01-01

    In contrast to the sophisticated music processing reported in the general population, individuals with congenital amusia show deficits in music perception and production. Congenital amusia occurs without brain damage, sensory or cognitive deficits, and has been suggested as a lifelong deficit with genetic origin. Even though recognized for a long time, this disorder has been systematically studied only relatively recently for its behavioral and neural correlates. The currently most investigated hypothesis about the underlying deficits concerns the pitch dimension, notably with impaired pitch discrimination and memory. Anatomic and functional investigations of pitch processing revealed that the amusic brain presents abnormalities in the auditory and inferior frontal cortices, associated with decreased connectivity between these structures. The deficit also impairs processing of pitch in speech material and processing of the time dimension in music for some of the amusic individuals, but does not seem to affect spatial processing. Some studies suggest at least partial dissociation in the disorder between perception and production. Recent studies revealed spared implicit pitch perception in congenital amusia, supporting the power of implicit cognition in the music domain. Current challenges consist in defining different subtypes of congenital amusia as well as developing rehabilitation programs for this "musical handicap." © 2015 Elsevier B.V. All rights reserved.

  18. CT findings of extrahepatic alveolar echinococcus (report of 12 cases)

    International Nuclear Information System (INIS)

    Liu Wenya; Shang Ge; Dang Jun

    2000-01-01

    Objective: To analyze the CT findings of extrahepatic alveolar echinococcus (EAE), and assess the value of CT scanning for the diagnosis of such cases. Methods: 12 patients with hepatic alveolar echinococcus (HAE) verified by operation and histology were examined by CT because of new complains. It was found that multiple organs were involved by the same lesions. Results: Brain AE (7 cases) showed single or multiple cerebral nodules, characterized by honeycombed hypodense structures or target sign after enhancement. Lung AE (3 cases) appeared as irregular, peripherally scattered nodules, with small vacuoles or cavities inside. The only 1 case with heart AE demonstrated a multiple calcifications and vacuoles within the mass. Adrenal gland AE (2 cases) presented as plaques containing different sizes of hypodense areas and calcifications. Retroperitoneal AE (2 cases) exhibited mass with plentiful calcifications. Conclusion: CT can define the location and morphology of the lesion, providing a reliable method for the diagnosis and treatment of the disease

  19. Abnormal Gas Diffusing Capacity and Portosystemic Shunt in Patients With Chronic Liver Disease

    OpenAIRE

    Park, Moon-Seung; Lee, Min-Ho; Park, Yoo-Sin; Kim, Shin-Hee; Kwak, Min-Jung; Kang, Ju-Seop

    2012-01-01

    Background Pulmonary dysfunctions including the hepatopulmonary syndrome and portosystemic shunt are important complications of hepatic cirrhosis. To investigate the severity and nature of abnormal gas diffusing capacity and its correlation to portosystemic shunt in patients with chronic liver disease. Methods Forty-four patients with chronic liver disease (15 chronic active hepatitis (CAH), 16 Child-Pugh class A, and 13 Child-Pugh class B) without other diseases history were enrolled in the ...

  20. Extrahepatic biliary obstrution secondary to neuroendocrine tumor of the common hepatic duct

    Directory of Open Access Journals (Sweden)

    Faraz A. Khan

    2017-01-01

    Conclusion: NET of the extrahepatic biliary tree are a rare entity. Typical presentation is with painless jaundice and other symptoms related to obstruction of the biliary tree and the diagnosis is usually made post-operatively.

  1. Extrahepatic Manifestations and Autoantibodies in Patients with Hepatitis C Virus Infection

    Directory of Open Access Journals (Sweden)

    Takashi Himoto

    2012-01-01

    Full Text Available Patients with chronic hepatitis C virus (HCV infection frequently have many extrahepatic manifestations, as persistent HCV infection often triggers lymphoproliferative disorders and metabolic abnormalities. These manifestations primarily include autoimmune disorders such as cryoglobulinemia, Sjögren’s syndrome, and autoimmune thyroid disorders. It has been well established that chronic HCV infection plays important roles in the production of non-organ-specific autoantibodies, including antinuclear antibodies and smooth muscle antibodies, and organ-specific autoantibodies such as thyroid autoantibodies. However, the clinical significance of autoantibodies associated with the extrahepatic manifestations caused by HCV infection has not been fully recognized. In this paper, we mainly focus on the relationship between extrahepatic manifestations and the emergence of autoantibodies in patients with HCV infection and discuss the clinical relevance of the autoantibodies in the extrahepatic disorders.

  2. Congenital Hydrocephalus.

    Science.gov (United States)

    Estey, Chelsie M

    2016-03-01

    There are several types of hydrocephalus, which are characterized based on the location of the cerebrospinal fluid (CSF) accumulation. Physical features of animals with congenital hydrocephalus may include a dome-shaped skull, persistent fontanelle, and bilateral ventrolateral strabismus. Medical therapy involves decreasing the production of CSF. The most common surgical treatment is placement of a ventriculoperitoneal shunt. Postoperative complications may include infection, blockage, drainage abnormalities, and mechanical failure. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Congenital diplopodia

    International Nuclear Information System (INIS)

    Brower, Jason S.; Wootton-Gorges, Sandra L.; Costouros, John G.; Boakes, Jennette; Greenspan, Adam

    2003-01-01

    Diplopodia, or duplicated foot, is a rare congenital anomaly. It differs from polydactyly in that supernumerary metatarsal and tarsal bones are present as well as extra digits. Only a few cases of this anomaly have been reported in the literature to date. We present a newborn male without intrauterine teratogen exposure who was born with a duplicate foot of the left lower extremity and imperforate anus. (orig.)

  4. Congenital toxoplasmosis.

    Science.gov (United States)

    Kieffer, François; Wallon, Martine

    2013-01-01

    Congenital toxoplasmosis results from the transplacental transmission of the parasite Toxoplasma gondii after a maternal infection acquired in pregnancy. Prevalence of congenital infection ranges from 0.1 to 0.3 per 1000 live births. The maternal-fetal transmission rate increases with gestational age at maternal seroconversion, from less than 15% at 13 weeks of gestation to over 70% at 36 weeks. Conversely, the later the maternal infection, the lower the risk of symptomatic congenital infection (infections acquired during the third trimester are most often asymptomatic at birth). Prenatal diagnosis is currently performed by PCR analysis in amniotic fluid. Antenatal management and treatment vary considerably among countries. In some European countries, maternal infections are detected through serological screening allowing a prompt treatment with spiramycin, which is expected to reduce the risk of vertical transmission. If PCR analysis in amniotic fluid is positive or if maternal infection was acquired in the third trimester of pregnancy, a combination with pyrimethamine and sulphonamide is given until delivery. Benefits of antenatal treatments remain controversial. Infected newborns are prescribed pyrimethamine and sulphonamide for 12 months. Despite antenatal and postnatal treatment, chorioretinitis can occur at any age (prevalence>20% at 10 years of age): long-term ophthalmological follow-up remains necessary. Copyright © 2013 Elsevier B.V. All rights reserved.

  5. Congenital Cytomegalovirus Hepatitis in the XXI Century is not Uncommon!

    Directory of Open Access Journals (Sweden)

    N.P. Skorodumova

    2014-08-01

    Full Text Available The article presents features of early clinical and laboratory diagnosis of congenital cytomegalovirus (CMV hepatitis. The analysis of the causes of late diagnosis of chronic hepatitis is carried out. Some children have repeatedly appealed to the doctor and treated for gastroduodenitis, cholecystitis, biliary dyskinesia, ulcerative colitis. Chart review allowed to assume that the extrahepatic manifestations of chronic hepatitis and liver cirrhosis district pediatricians interpreted as allergy (recurrent urticaria, juvenile rheumatoid arthritis (arthritis, vegetovascular dystonia (ECG changes; endocrine violations (delayed menstruation, striae on the abdominal skin — as the feature of puberty; signs of hypo- or hyperthyroidism — as endemic pathology. A sequence of doctor’s actions in diagnosing congenital CMV infection, mainly affecting the liver, is provided.

  6. Colectomy for porto-systemic encephalopathy: is it still topical?

    Directory of Open Access Journals (Sweden)

    Rym Ennaifer

    2013-06-01

    Full Text Available Hepatic encephalopathy (HE is a common long term complication of porto-systemic shunt. We report herein the case of a 59-year-old man with Child-Pugh A cirrhosis treated successfully 9 years earlier with distal splenorenal shunt for uncontrolled variceal bleeding. In the last year, he developed a severe and persistent hepatic encephalopathy secondary to the shunt, which was resistant to medical therapy. As liver transplantation was not available and obliteration of the shunt was hazardous, we performed subtotal colectomy in order to reduce ammonia production. This therapeutic option proved successful, as the grade of encephalopathy decreased and the patient improved. Our experience indicates that colonic exclusion should be considered as an option in the management of HE refractory to medical treatment in highly selected patients when liver transplantation is not available or even as a bridge given the long waiting time on lists.

  7. Assessment of surgical portosystemic shunts and associated complications: The diagnostic and therapeutic role of radiologists

    Energy Technology Data Exchange (ETDEWEB)

    Taslakian, Bedros, E-mail: btaslakian@gmail.com [Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020—PO Box: 11-0236, Beirut (Lebanon); Faraj, Walid, E-mail: wf07@aub.edu.lb [Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020—PO Box: 11-0236, Beirut (Lebanon); Khalife, Mohammad, E-mail: mk12@aub.edu.lb [Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020—PO Box: 11-0236, Beirut (Lebanon); Al-Kutoubi, Aghiad, E-mail: mk00@aub.edu.lb [Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020—PO Box: 11-0236, Beirut (Lebanon); El-Merhi, Fadi, E-mail: fe19@aub.edu.lb [Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020—PO Box: 11-0236, Beirut (Lebanon); Saade, Charbel, E-mail: cs39@aub.edu.lb [Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020—PO Box: 11-0236, Beirut (Lebanon); Hallal, Ali, E-mail: ah05@aub.edu.lb [Department of General Surgery, American University of Beirut Medical Center, Riad El-Solh 1107 2020—PO Box: 11-0236, Beirut (Lebanon); Haydar, Ali, E-mail: ah24@aub.edu.lb [Department of Radiology, American University of Beirut Medical Center, Riad El-Solh 1107 2020—PO Box: 11-0236, Beirut (Lebanon)

    2015-08-15

    Highlights: • Portal hypertension is the pathological increase in portal venous pressure. • Surgical portosystemic shunting is an accepted methods to decrease portal venous pressure. • Surgical portosystemic shunts are divided into selective and nonselective. • Shunt thrombosis is a serious complication, resulting in shunt dysfunction. • Imaging is essential in the assessment of the shunt function and anatomy. - Abstract: Surgical portosystemic shunting, the formation of a vascular connection between the portal and systemic venous circulation, has been used as a treatment to reduce portal venous pressure. Although the use of portosystemic shunt surgery in the management of portal hypertension has declined during the past decade in favour of alternative therapies, and subsequently surgeons and radiologists became less familiar with the procedure, it remains a well-established treatment. Knowledge of different types of surgical portosystemic shunts, their pathophysiology and complications will help radiologists improve communication with surgeons and enhance their understanding of the diagnostic and therapeutic role of radiology in the assessment and management of these shunts. Optimal assessment of the shunt is essential to determine its patency and allow timely intervention. Both non-invasive and invasive imaging modalities complement each other in the evaluation of surgical portosystemic shunts. Interventional radiology plays an important role in the management of complications, such as shunt thrombosis and stenosis. This article describes the various types of surgical portosystemic shunts, explains the anatomy and pathophysiology of these shunts, illustrates the pearls and pitfalls of different imaging modalities in the assessment of these shunts and demonstrates the role of radiologists in the interventional management of complications.

  8. Assessment of surgical portosystemic shunts and associated complications: The diagnostic and therapeutic role of radiologists

    International Nuclear Information System (INIS)

    Taslakian, Bedros; Faraj, Walid; Khalife, Mohammad; Al-Kutoubi, Aghiad; El-Merhi, Fadi; Saade, Charbel; Hallal, Ali; Haydar, Ali

    2015-01-01

    Highlights: • Portal hypertension is the pathological increase in portal venous pressure. • Surgical portosystemic shunting is an accepted methods to decrease portal venous pressure. • Surgical portosystemic shunts are divided into selective and nonselective. • Shunt thrombosis is a serious complication, resulting in shunt dysfunction. • Imaging is essential in the assessment of the shunt function and anatomy. - Abstract: Surgical portosystemic shunting, the formation of a vascular connection between the portal and systemic venous circulation, has been used as a treatment to reduce portal venous pressure. Although the use of portosystemic shunt surgery in the management of portal hypertension has declined during the past decade in favour of alternative therapies, and subsequently surgeons and radiologists became less familiar with the procedure, it remains a well-established treatment. Knowledge of different types of surgical portosystemic shunts, their pathophysiology and complications will help radiologists improve communication with surgeons and enhance their understanding of the diagnostic and therapeutic role of radiology in the assessment and management of these shunts. Optimal assessment of the shunt is essential to determine its patency and allow timely intervention. Both non-invasive and invasive imaging modalities complement each other in the evaluation of surgical portosystemic shunts. Interventional radiology plays an important role in the management of complications, such as shunt thrombosis and stenosis. This article describes the various types of surgical portosystemic shunts, explains the anatomy and pathophysiology of these shunts, illustrates the pearls and pitfalls of different imaging modalities in the assessment of these shunts and demonstrates the role of radiologists in the interventional management of complications

  9. Clinical results of the transjugular intrahepatic portosystemic shunt

    Energy Technology Data Exchange (ETDEWEB)

    Park, Auh Whan; Sim, Jae In; Ryeom, Hun Kyu; Kim, Yong Joo [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    1994-04-15

    To evaluate the clinical results of transjugular intrahepatic portosystemic shunt(TIPS) for the control of variceal bleeding. TIPS creation was attempted in 23 patients with endoscopically confirmed variceal bleeding. Most patients had multiple episodes of bleeding in the past and have been treated with multiple endoscopic sclerotherapies. Pre-and post-procedural hepatic and portal vein pressures were measured. After creation of TIPS patients were followed up at regular intervals. TIPS has been successfully accomplished in 22 of 23 patients using Wallstent(n = 21) and Strecker stent(n = 1). Immediate bleeding control was achieved in all patients with shunt creation. No procedure-related complication was noted. Portal vein pressure was reduced from 30.7 {+-} 5.8 mmHg to 20.8 {+-} 4.7 mmHg. The mean pressure gradient of portosystemic shunt dropped from 22.8 {+-} 6.0 prior to TIPS to 12.2 {+-} 4.1 immediately after. During the follow-up period (6-556 days, mean; 10 months), seven patients died; progressive hepatic failure (n 4), variceal rebleeding (n = 2), and respiratory failure(n = 1). Hepatic encephalopathy after TIPS was noted in 7 patients(31.8%). Variceal rebleeding occurred in 3 patients(13.6%). The remaining 15 patients have survived an average of 11 months. This results suggest that TIPS is a safe and effective method for lowering portal pressure and controlling variceal bleeding. Furthermore if these initial results are encouraged by further long-term observation, TIPS could replace endoscopic and risky surgical intervention.

  10. Congenital syphilis

    International Nuclear Information System (INIS)

    Lee, Sang Wook; Kim, Kyung Soo; Hur, Don

    1983-01-01

    In recent years, marked increase in incidence of congenital syphilis has occurred throughout the world due to changes in social norms and development of penicillin-resistant strains. Early diagnosis plays an important role in congenital syphilis as the clinical manifestations may simulate many other conditions in the paediatric age group. The authors analyzed 52 cases of congenital syphilis admitted to the department of paediatrics, Chosun University Hospital, clinically and radiologically. Among them, 18 cases were born in this hospital and 34 cases were admitted from OPD, during the period of 8 years from January, 1975 to December, 1982. The results obtained were as follows; 1. In 28 of 34 cases (82%), the first clinical manifestations were below the age of 3 months. 2. Among the 52 cases, a male predominance was observed with a male to female ratio of 2 : 1. 3. The serologic test (VDRL) of the 52 studied cases showed reactive response in 49 cases (94%), and that of syphilitic mothers except 6 cases, reactive in all studied cases. 4. The major manifestations of the 52 cases were bone tenderness (12%) and swelling of the joints (7%) in skeletal system, hepatosplenomegaly (79%) and skin lesions (73%) in extraskeletal one. 5. The radiological skeletal changes were detected in 45 of 52 cases (87%), and the commonest findings were detected in 45 of 52 cases (87%), and the commonest findings were metaphysitis (83%) and periostitis (81%). The most characteristic type of metaphysitis were transverse trophic line (74%) and zone of rarefaction (65%). 6. The commonest bones to be affected were growing metaphyses of the long bones, particulary about the wrist and the knee. The order of frequency were radius (80%), uina (80%), tibia (77%), femur (69%) and humerus (40%)

  11. Arteriomesentericography in patients with extrahepatic forms of portal hypertension

    International Nuclear Information System (INIS)

    Semenov, V.S.; Gotman, L.N.

    1980-01-01

    The reverse contrast examination of the system, carried out by means of superior arteriomesentericography, is the optimal method for diagnosing pathological changes in the portal channel of splenectomized patients with the syndrome of the extrahepatic form of portal hypertension. The selective catheterization of the superior mesenteric artery was made in 36 patients with the prerenal block of the portal system. In all cases the venous phase of portography was obtained, which served as a basis for selecting the amount of surgical intervention in these patients. Depending on the level of the block of the portal channel, the patients fell into 3 groups. The characteristic X-ray appearance of portal hypertension, manifested mainly by the presence of portoportal and portocaval collaterals, is described for each group. The thrombosis of the main vessels of the loose type of their structure excludes the possibility of portocaval anastomosis, while in the presence of the pronounced surgical trunk of the superior mesenteric vein vascular shunting may be recommended. Portal hypertension, in contrast to the normal state, requires prolonged contrast examination of the venous phase due to a slower portorenal blood flow

  12. Results of radiation therapy in extrahepatic bile duct carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Hatano, Kazuo; Cho, Keiichi; Okamoto, Moriyo (Chiba Univ. (Japan). School of Medicine) (and others)

    1992-06-01

    From January 1975 to August 1988, 40 patients with extrahepatic bile duct carcinoma were treated by external irradiation at Chiba University Hospital and the National Medical Center Hospital. Thirty-four patients (male: 20, female: 14) were evaluable. Eighteen patients were postoperative cases because the surgical margin was positive for tumor cells in the postoperative pathological examination; the other 16 were inoperable cases. Survival in postoperative and inoperable cases was not significantly different, with median survival times of 13.8 and 8.1 months, respectively. Survival in the recanalization-positive and negative-groups was significantly different (p<0.05) after irradiation, with median survival times of 13.5 and 6.0 months, respectively. Complications of therapy were recognized in 68% of all cases. They were mainly gastrointestinal symptoms such as nausea, vomiting, erosive gastritis and loss of appetite, but they were not severe. Distant metastasis was recognized in only 4 patients (10%): three had bony metastasis and one had supraclavicular and pulmonary hilar lymph node metastasis. Ninety percent of all cases died from hepatic failure or peritonitis carcinomatosa due to failure to obtain local control by external irradiation. A more effective modality of treatment is necessary to cure these patients. (author).

  13. Results of radiation therapy in extrahepatic bile duct carcinoma

    International Nuclear Information System (INIS)

    Hatano, Kazuo; Cho, Keiichi; Okamoto, Moriyo

    1992-01-01

    From January 1975 to August 1988, 40 patients with extrahepatic bile duct carcinoma were treated by external irradiation at Chiba University Hospital and the National Medical Center Hospital. Thirty-four patients (male: 20, female: 14) were evaluable. Eighteen patients were postoperative cases because the surgical margin was positive for tumor cells in the postoperative pathological examination; the other 16 were inoperable cases. Survival in postoperative and inoperable cases was not significantly different, with median survival times of 13.8 and 8.1 months, respectively. Survival in the recanalization-positive and negative-groups was significantly different (p<0.05) after irradiation, with median survival times of 13.5 and 6.0 months, respectively. Complications of therapy were recognized in 68% of all cases. They were mainly gastrointestinal symptoms such as nausea, vomiting, erosive gastritis and loss of appetite, but they were not severe. Distant metastasis was recognized in only 4 patients (10%): three had bony metastasis and one had supraclavicular and pulmonary hilar lymph node metastasis. Ninety percent of all cases died from hepatic failure or peritonitis carcinomatosa due to failure to obtain local control by external irradiation. A more effective modality of treatment is necessary to cure these patients. (author)

  14. Pharmacological strategies for protection of extrahepatic islet transplantation.

    Science.gov (United States)

    Omori, K; Komatsu, H; Rawson, J; Mullen, Y

    2015-06-01

    The safety and effectiveness of islet transplantation has been proven through world-wide trials. However, acute and chronic islet loss has hindered the ultimate objective of becoming a widely used treatment option for type 1 diabetes. A large islet loss is attributed, in part, to the liver being a less-than-optimal site for transplantation. Over half of the transplanted islets are destroyed shortly after transplantation due to direct exposure to blood and non-specific inflammation. Successfully engrafted islets are continuously exposed to the liver micro-environment, a unique immune system, low oxygen tension, toxins and high glucose, which is toxic to islets, leading to premature islet dysfunction/death. Investigations have continued to search for alternate sites to transplant islets that provide a better environment for prolonged function and survival. This article gathers courses and conditions that lead to islet loss, from organ procurement through islet transplantation, with special emphasis on hypoxia, oxidative stress, and antigen non-specific inflammation, and reviews strategies using pharmacological agents that have shown effectiveness in protecting islets, including a new treatment approach utilizing siRNA. Pharmacological agents that support islet survival and promote β-cell proliferation are also included. Treatment of donor pancreata and/or islets with these agents should increase the effectiveness of islets transplanted into extrahepatic sites. Furthermore, the development of methods designed to release these agents over an extended period, will further increase their efficacy. This requires the combined efforts of both islet transplant biologists and bioengineers.

  15. An inguinal hernia sac tumor of extrahepatic cholangiocarcinoma origin

    Directory of Open Access Journals (Sweden)

    Yamazaki Hidehiro

    2006-03-01

    Full Text Available Abstract Background Metastatic hernia sac tumor from biliary malignancy is extremely rare with only one such case previously reported. We herein report an additional case of extrahepatic cholangiocarcinoma presenting as a hernia sac tumor. Case presentation A 78-year-old man presented with an irreducible right inguinal hernia associated with a firm tumor, 2.0 cm in diameter. A computed tomography scan demonstrated a soft tissue density mass with heterogeneous enhancement within the right inguinal canal. The patient underwent a hernia repair and the hernia sac tumor was resected. Histological examination of the tumor revealed a metastatic adenocarcinoma suggesting the tumor was of pancreato-biliary origin. Further investigation using imaging studies disclosed a primary tumor in the upper bile duct. The patient died of the disease nine months after the resection. Conclusion Hernia sac tumors should be considered when an irreducible, growing mass appears within an inguinal hernia. Computed tomography may be useful for the early detection of hernia sac tumors from undiagnosed intra-abdominal malignancies.

  16. Morphologic Features of Extrahepatic Manifestations of Hepatitis C Virus Infection

    Directory of Open Access Journals (Sweden)

    Huaibin M. Ko

    2012-01-01

    Full Text Available Cirrhosis and hepatocellular carcinoma are the prototypic complications of chronic hepatitis C virus infection in the liver. However, hepatitis C virus also affects a variety of other organs that may lead to significant morbidity and mortality. Extrahepatic manifestations of hepatitis C infection include a multitude of disease processes affecting the small vessels, skin, kidneys, salivary gland, eyes, thyroid, and immunologic system. The majority of these conditions are thought to be immune mediated. The most documented of these entities is mixed cryoglobulinemia. Morphologically, immune complex depositions can be identified in small vessels and glomerular capillary walls, leading to leukoclastic vasculitis in the skin and membranoproliferative glomerulonephritis in the kidney. Other HCV-associated entities include porphyria cutanea tarda, lichen planus, necrolytic acral erythema, membranous glomerulonephritis, diabetic nephropathy, B-cell non-Hodgkin lymphomas, insulin resistance, sialadenitis, sicca syndrome, and autoimmune thyroiditis. This paper highlights the histomorphologic features of these processes, which are typically characterized by chronic inflammation, immune complex deposition, and immunoproliferative disease in the affected organ.

  17. Angiography-based C-arm CT for the assessment of extrahepatic shunting before radioembolization

    International Nuclear Information System (INIS)

    Heusner, Till Alexander; Hahn, S.; Forsting, M.; Antoch, G.; Hamami, M.E.; Poeppel, T.; Bockisch, A.; Ertle, J.; Hilgard, P.

    2010-01-01

    Purpose: to retrospectively assess the accuracy of angiography-based C-arm CT for the detection of extrahepatic shunting before SIRT. Materials and methods: 30 patients (mean age: 64 ± 12 years) with hypervascularized hepatic tumors underwent hepatic angiography, coil embolization of gastrointestinal collaterals and 99mTc-macroaggregated albumin (MAA) SPECT/CT before SIRT. Before MAA injection via a microcatheter from the intended treatment position, an angiography and angiography-based C-arm CT (XperCT trademark, Philips Healthcare) were acquired. Angiographies and XperCT trademark were performed from 48 microcatheter positions followed by MAA injections and MAA-SPECT/CT. MAA-SPECT/CT served as the reference standard for determining the accuracy of hepatic arteriography and C-arm CT for the detection of extrahepatic shunting. Results: MAA-SPECT/CT revealed extrahepatic shunting in 5 patients (17%). Hepatic arteriography yielded a true negative in 22 (73%), a false negative in 5 (17%), and an unclear result in 3 patients (10%). C-arm CT yielded a true positive in 3 (10%), true negative in 24 (80%), false positive in 1 (3%), and false negative in 2 patients (7%). The specificity and the NPV of hepatic arteriography for the detection of extrahepatic shunting were 88% and 81%, respectively. For C-arm CT the sensitivity, specificity, PPV, NPV, and accuracy for the detection of extrahepatic shunting were 60%, 96%, 75%, 92%, and 90%, respectively. Conclusion: C-arm CT offers additional information to angiography when assessing SIRT patients for extrahepatic shunting. More accurate detection of extrahepatic shunting may optimize the workflow in SIRT preparations by avoiding unnecessary repeat angiographies. (orig.)

  18. Association between biliary complications and technique of hilar division (extrahepatic vs. intrahepatic in major liver resections

    Directory of Open Access Journals (Sweden)

    Gamaletsos Evangelos

    2006-08-01

    Full Text Available Abstract Background Division of major vascular and biliary structures during major hepatectomies can be carried out either extrahepatically at the porta hepatic or intrahepatically during the parenchymal transection. In this retrospective study we test the hypothesis that the intrahepatic technique is associated with less early biliary complications. Methods 150 patients who underwent major hepatectomies were retrospectively allocated into an intrahepatic group (n = 100 and an extrahepatic group (n = 50 based on the technique of hilar division. The two groups were operated by two different surgical teams, each one favoring one of the two approaches for hilar dissection. Operative data (warm ischemic time, operative time, blood loss, biliary complications, morbidity and mortality rates were analyzed. Results In extrahepatic patients, operative time was longer (245 ± 50 vs 214 ± 38 min, p Conclusion Intrahepatic hilar division is as safe as extrahepatic hilar division in terms of intraoperative blood requirements, morbidity and mortality. The extrahepatic technique is associated with more severe bile leaks and biliary injuries.

  19. An experimental study on microcholangiographic manifestation in extrahepatic bile duct obstruction

    International Nuclear Information System (INIS)

    Lee, Yul; Kang, Heung Sik; Park, Jae Hyung; Kim, Chu Wan

    1987-01-01

    Microcholangiographic using microbarium was done for radiological observation of the morphology of intrahepatic microbiliary system and its charge after extrahepatic bile duct obstruction. Regurgitation of microbarium into the systemic circulation was also observed. Extrahepatic bile ducts of 40 rabbits were ligated and microcholangiography was done just after ligation, after 1 day, 3 days and 5 days. Injection pressure of microbarium was 58 cm H 2 O in 20 rabbits and 93 cm H 2 O in another 20 rabbits. Histologic findings of the liver was compared with microcholangiographic findings. The results were as follows: 1. In microcholangiography, interlobular bile ducts and ductules were well noted, but bile canaliculi were not visible. 2. After extrahepatic bile duct ligation, ductules and small interlobular bile ducts were tortuously dilated and proliferated. These findings progressed according as the time after extrahepatic bile duct ligation especially between 1 and 2 days. 3. Microbarium was regurgitated into hepatic sinusoids from a portal tract due to rupture of interlobular bile ducts or ductules, and this was observed only in limited portions of sample tissue sections, but could be found in most of 40 rabbits. From the above results, the morphology of intrahepatic microbiliary system and its change after extrahepatic bile duct obstruction could be radiologically observed with microcholangiography, so this technique could be used for experimental study about the biliary system.

  20. Evaluation of the angiographic findings for extrahepatic arterial supply to primary hepatic cancer and interventional therapy

    International Nuclear Information System (INIS)

    Wang Weiyu; Lv Weifu; Hou Changlong; Zhang Xingming; Zhang Zhengfeng; Lu Dong; Gao Zonggen

    2007-01-01

    Objective: To study the angiographic characteristics of extrahepatic arterial supply for primary hepatic cancer (PHC)and the significance of interventional therapy. Methods: 32 cases of primary. hepatic cancer were undertaken routine celiac arterial angiography and explored the extrahepatic arterial supply for the tumor, then followed by superselective transcatheter arterial chemoembolization (TACE). Results: 37 extrahepatic feeding arteries to hepatic cancers in 32 cases were found including 12 from superior mesenteric arteries(SMA), 9 right inferior phrenic arteries (RIPA), 1 left inferior phrenic arteries (LIPA), 2 pancreatic arterial arch, 1 right internal thoracic artery(RITA), 1 right intercostal artery(RICA), 6 left gastric arteries (LGA), 1 splenic artery, 2 omental arteries (OTA), 2 gastroduodenal arteries. The most common extrahepatic feeding arteries were originated from SMA and RIPA. The rest 33 were performed with superselective transcatheter arterial chemoembolization and the other 4 with only transcatheter arterial chemotherapeutic perfusion due to failure of superselective catheterization. Conclusion: The extrahepatic feeding artery is commonly seen with various kinds and also necessary for interventional treatment same as the primary ones for hepatic cancers. (authors)

  1. Evaluation of computed tomography on diagnosis of portosystemic collaterals in portal hypertension

    International Nuclear Information System (INIS)

    Ohe, Takashi; Kuronuma, Yukio; Fujiwara, Hiromichi; Ibuki, Yoshikazu; Maehara, Misao; Sugaya, Hitoshi; Harada, Takashi; Iwasaki, Naoya; Hyodo, Haruo

    1987-01-01

    We analyzed the diagnostic capability of CT to demonstrate the eight types of portosystemic collaterals in patient with portal hypertension. A total of 62 patients with portal hypertension underwent both CT and conventional angiography. All of these eight types of collaterals, such as esophageal varices, paraesophageal varices, coronary and short gastric pathway, dilated vein in splenic hilus, splenorenal and splenoretroperitoneal pathway, paraumbilical pathway and small veins on liver surface, caput medusa, azygos system, were demonstrated on CT better than angiography, except coronary and short gastric pathyway. And we also made comparative study of CT with per-rectal portal scintigraphy in 9 patients who underwent both studies. In 7 of these 9 patients, portosystemic collaterals were recognized on scintigram less than CT. In conclusion, CT provides much qualified images than conventional angiography or per-rectal portal scintigraphy in evaluating portosystemic collaterals. (author)

  2. Evaluation of computed tomography on diagnosis of portosystemic collaterals in portal hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Ohe, T; Kuronuma, Y; Fujiwara, H; Ibuki, Y; Maehara, M; Sugaya, H; Harada, T; Iwasaki, N; Hyodo, H

    1987-04-01

    We analyzed the diagnostic capability of CT to demonstrate the eight types of portosystemic collaterals in patient with portal hypertension. A total of 62 patients with portal hypertension underwent both CT and conventional angiography. All of these eight types of collaterals, such as esophageal varices, paraesophageal varices, coronary and short gastric pathway, dilated vein in splenic hilus, splenorenal and splenoretroperitoneal pathway, paraumbilical pathway and small veins on liver surface, caput medusa, azygos system, were demonstrated on CT better than angiography, except coronary and short gastric pathyway. And we also made comparative study of CT with per-rectal portal scintigraphy in 9 patients who underwent both studies. In 7 of these 9 patients, portosystemic collaterals were recognized on scintigram less than CT. In conclusion, CT provides much qualified images than conventional angiography or per-rectal portal scintigraphy in evaluating portosystemic collaterals.

  3. Gallbladder varices in extrahepatic portal venous obstruction: demonstration by intravenous

    International Nuclear Information System (INIS)

    Gulati, M.

    2002-01-01

    Full text: We performed a prospective study to determine frequency of presence of gallbladder varices (GBV) by intravenous CT portography (CTP) in patients with extrahepatic portal venous obstruction (EHPVO). 90 patients (age range: 2-55 years) with EHPVO (initially diagnosed on abdominal sonography) underwent CTP using a subsecond helical CT scanner. Axial overlapping sections of 2mm were obtained with collimation 3mm and table speed 4.5mm/sec (pitch 1.5). Presence and patterns of GBV were studied. CTP demonstrated GBV in 54 (60%) of 90 patients.GBV were said to be present when one or more of the following findings were seen: diffuse wall enhancement (26/90), pinpoint areas of enhancement in GB wall (33/90), obvious large collaterals in GB wall (8/90) and pericholecystic collaterals (49/90). Presence of GBV did not correlate with the site and extent of EHPVO. Contiguous intrahepatic collaterals extending from GB bed to intrahepatic portal vein branches were seen in 41 of 54 (76%) of patients with GBV, suggesting the role of GBV serving as bridging portoportal collaterals. Hepatic perfusion defects were seen in 5/54 patients with GBV and were not seen in remaining 36/90 patients of EHPVO. GB calculi were seen in only 4/54 cases with GBV (as determined on sonography) suggesting no increase in risk for cholelithiasis. GBV commonly develop as bridging collaterals in patients with EHPVO. CTP is very useful in detecting these varices and planning biliary surgery, given the frequency of iatrogenic surgical bleeding in these patients. Copyright (2002) Blackwell Science Pty Ltd

  4. Congenital amusia.

    Science.gov (United States)

    Williamson, Victoria J; Stewart, Lauren

    2013-01-01

    For most people, music, like language, is acquired effortlessly in early life. But a few percent of the population have lifelong difficulties in the perception and production of music. In this chapter we discuss psycho-acoustic and behavioral studies that have attempted to delineate the nature of the auditory perceptual deficits in this group and consider whether these difficulties extend outside the musical domain. Finally, we review structural imaging studies in this group which point to subtle anomalies in temporal and frontal areas. We suggest that amusia can be considered a disorder of neural development, which has relatively specific consequences at the behavioral level. Studies of congenital amusia provide a unique window on the neurocognitive architecture of music processing. Copyright © 2013 Elsevier B.V. All rights reserved.

  5. MRCP and 3D LAVA imaging of extrahepatic cholangiocarcinoma at 3 T MRI

    Energy Technology Data Exchange (ETDEWEB)

    Li, N.; Liu, C.; Bi, W.; Lin, X.; Jiao, H. [Shandong Medical Imaging Research Institute, Shandong University, Jinan (China); Zhao, P., E-mail: Gavinsdu@163.com [Department of Radiology, Shandong Provincial Hospital, Shandong University, Jinan (China)

    2012-06-15

    Extrahepatic cholangiocarcinoma (CCA) is a primary bile duct malignant tumour with poor prognosis. Familiarity with their varied imaging characteristics can be helpful in developing a correct diagnosis and in optimal treatment planning, and thus contribute to a better prognosis. The purpose of this article is to illustrate the typical appearances of extrahepatic CCA on magnetic resonance cholangiopancreatography (MRCP) and three-dimensional (3D) LAVA (liver acquisition with volume acceleration) sequences at 3 T magnetic resonance imaging (MRI), and to discuss the superiority of the two techniques in the diagnosis of CCA.

  6. Transjugular Intrahepatic Portosystemic Shunts in Children with Biliary Atresia

    International Nuclear Information System (INIS)

    Huppert, Peter E.; Goffette, Pierre; Astfalk, Wolfgang; Sokal, Emil M.; Brambs, Hans-Juergen; Schott, Ullrich; Duda, Stephan H.; Schweizer, Paul; Claussen, Claus D.

    2002-01-01

    Purpose: We retrospectively evaluated the technical and long-term clinical results of transjugularintrahepatic portosystemic shunts (TIPS) in children with portal hypertension and biliary atresia (BA). Methods: Nine children with BA and recurrent bleeding from esophagogastric and/or intestinal varices were treated by TIPS at the age of 34-156 months and followed-up in two centers. Different types of stents were used. Results: Shunt insertion succeeded in all patients, but in two a second procedure was necessary. Seven procedures lasted more than 3 hr, mainly due to difficult portal vein puncture.Variceal bleeding ceased in all patients; however, 16 reinterventions were performed in eight patients for clinical reasons (n =11) and sonographically suspected restenosis (n =5). Four patients underwent successful liver transplantation 4-51 months after TIPS and five are in good clinical conditions 64-75 months after TIPS. Conclusions: TIPS in children with BA is technically difficult, mainly due to periportal fibrosis and small portal veins. Frequency of reinterventions seems to be higher compared with adults

  7. Transcaval transjugular intrahepatic portosystemic shunt: preliminary clinical results

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kwang Hun; Lee, Do Yun; Won, Jong Yoon [Yonsei University College of Medicine, Seoul (Korea, Republic of); Park, Sang Joon [Hallym University College of Medicine, Seoul (Korea, Republic of); Kim, Jae Kyu; Yoon, Woong [Chonnam National University Hospital, Gwangju (Korea, Republic of)

    2003-03-01

    To determine the feasibility of transcaval transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins. Transcaval TIPS, performed in six patients, was indicated by active variceal bleeding (n=2), recurrent variceal bleeding (n=2), intractable ascites (n=1), and as a bridge to liver transplantation (n=1). The main reasons for transcaval rather than classic TIPS were the presence of an unusually acute angle between the hepatic veins and the level of the portal bifurcation (n=3), hepatic venous occlusion (n=2), and inadequate small hepatic veins (n=1). Technical and functional success was achieved in all patients. The entry site into liver parenchyma from the inferior vena cava was within 2 cm of the atriocaval junction. Procedure-related complications included the death of one patient due to hemoperitoneum despite the absence of contrast media spillage at tractography, and another suffered reversible hepatic encephalopathy. In patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins, transcaval TIPS creation is feasible.

  8. Transjugular Intrahepatic Portosystemic Shunt (TIPS): Current Status and Future Possibilities

    International Nuclear Information System (INIS)

    Bilbao, Jose Ignacio; Quiroga, Jorge; Herrero, Jose Ignacio; Benito, Alberto

    2002-01-01

    Since the insertion of the first TIPS in 1989 much has been learned about this therapeutic procedure. It has an established role for the treatment of some complications of portal hypertension: prevention of recurrent variceal bleeding and rescue of patients with acute uncontrollable variceal bleeding. In addition TIPS is useful for Budd-Chiari syndrome, refractory ascites and hepatorenal syndrome, although its specific role in these indications remains to be definitively established. However, the decrease in sinusoidal blood flow induced by TIPS can lead to the patient developing hepatic encephalopathy and liver failure in some cases. Therefore, TIPS should be used with caution in patients with very poor liver function. From a technical point of view, successful placement of TIPS is achieved in more than 98% of cases by experienced groups. At present, evaluation of TIPS dysfunction based on morphology probably leads to an overdiagnosis of this complication since most of these cases are not associated with clinical manifestations (recurrent bleeding or refractory ascites). The major disadvantage of TIPS remains its poor long-term patency requiring a mandatory surveillance program. The indicator for shunt function/malfunction should be the portosystemic pressure gradient, which is best assessed by intravascular measurements. Shunt obstructions may be prevented or reduced by the use of stent-grafts in the future

  9. Transcaval transjugular intrahepatic portosystemic shunt: preliminary clinical results

    International Nuclear Information System (INIS)

    Lee, Kwang Hun; Lee, Do Yun; Won, Jong Yoon; Park, Sang Joon; Kim, Jae Kyu; Yoon, Woong

    2003-01-01

    To determine the feasibility of transcaval transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins. Transcaval TIPS, performed in six patients, was indicated by active variceal bleeding (n=2), recurrent variceal bleeding (n=2), intractable ascites (n=1), and as a bridge to liver transplantation (n=1). The main reasons for transcaval rather than classic TIPS were the presence of an unusually acute angle between the hepatic veins and the level of the portal bifurcation (n=3), hepatic venous occlusion (n=2), and inadequate small hepatic veins (n=1). Technical and functional success was achieved in all patients. The entry site into liver parenchyma from the inferior vena cava was within 2 cm of the atriocaval junction. Procedure-related complications included the death of one patient due to hemoperitoneum despite the absence of contrast media spillage at tractography, and another suffered reversible hepatic encephalopathy. In patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins, transcaval TIPS creation is feasible

  10. [Congenital hypothyroidism].

    Science.gov (United States)

    Castilla Peón, María Fernanda

    Congenital hypothyroidism (CH) is a cause of preventable mental retardation; therefore, timely diagnosis and treatment by the primary care physician is very important. CH screening must be performed between the second and fifth days of life with capillary blood done with a heel prick and must be confirmed by measurement of thyroid hormones in venous blood. The most common cause of CH is thyroid dysgenesis, which may be identified by a thyroid scan carried out before initiating treatment. Treatment should be with levothyroxine (10-15μg/kg/day) and should not be delayed or suspended during the first 3 years of life due to the deleterious effect on neurodevelopment in case of low thyroid hormones during this time. Preterm or sick infants or those with Down syndrome require special consideration. This article provides diagnostic and therapeutic algorithms for CH. Copyright © 2015 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  11. Hepaticocystic duct and a rare extra-hepatic "cruciate" arterial anastomosis: a case report

    Directory of Open Access Journals (Sweden)

    Abeysuriya Vasitha

    2008-02-01

    Full Text Available Abstract Introduction The variations in the morphological characteristics of the extra-hepatic biliary system are interesting. Case presentation During the dissection of cadavers to study the morphological characteristics of the extra-hepatic biliary system, a 46-year-old male cadaver was found to have drainage of the common hepatic duct drains directly into the gall bladder neck. The right and left hepatic ducts were not seen extra-hepatically. Further drainage of the bile away from the gallbladder and into the duodenum was provided by the cystic duct. Formation of the common bile duct by the union of the common hepatic duct and cystic duct was absent. Further more the right hepatic artery was found to be communicating with the left hepatic artery by a "bridging artery" after giving rise to the cystic artery. An accessory hepatic artery originated from the "bridging artery" forming a "cruciate" hepatic arterial anastomosis. Conclusion Combination of a Hepaticocystic duct and an aberrant variation in the extra-hepatic arterial system is extremely rare.

  12. Signet Ring Cell Carcinoma of the Extrahepatic Bile Duct Diagnosed by Preoperative Biopsy: A Case Report

    Directory of Open Access Journals (Sweden)

    Emiri Kita

    2014-11-01

    Full Text Available A 73-year-old woman was admitted because of obstructive jaundice. Computed tomography revealed a stricture in the lower bile duct with enhanced bile duct wall. Endoscopic retrograde cholangiopancreatography (ERCP revealed a tapering stenosis at the lower bile duct. Transpapillary histological biopsy using biopsy forceps through ERCP was performed; the diagnosis of signet ring cell carcinoma (SRCC of the bile duct was established. Regional lymph node enlargement and distant metastases were not detected on diagnostic imaging. Pancreaticoduodenectomy with pylorus preservation was performed. Histological examination of the resected specimen confirmed SRCC of the extrahepatic bile duct coexisting with adenocarcinoma (ADC of the extrahepatic bile duct with negative resection margins. However, tumor cells directly invaded the pancreatic parenchyma and the muscle layer of the duodenum, prompting us to administer adjuvant chemotherapy to the patient, with no sign of tumor recurrence at 1-year follow-up. Almost all tumors originating from the extrahepatic bile duct are ADC and other histological variants are rare. Of these, SRCC is extremely rare and only four cases have been reported. Furthermore, to the best of our knowledge, this is the first case report regarding the preoperative diagnosis of SRCC of the bile duct. Current reports indicate that younger age and Asian ethnicity are the clinical features of SRCC of the extrahepatic bile duct. Immunohistochemical staining of CK7, CK20 and MUC2 may be useful for predicting prognosis. Chemotherapy has not resulted in increased survival rates and only surgical resection currently serves as a curative treatment.

  13. Carcinoid tumors of the extrahepatic bile ducts: a study of seven cases

    NARCIS (Netherlands)

    Maitra, A.; Krueger, J. E.; Tascilar, M.; Offerhaus, G. J.; Angeles-Angeles, A.; Klimstra, D. S.; Hruban, R. H.; Albores-Saavedra, J.

    2000-01-01

    The authors report seven patients with carcinoid tumors of the extrahepatic bile ducts (EHBDs). All patients were women, with an average age at diagnosis of 49.8 years (range, 37-67 yrs). The most common presenting symptom was painless jaundice with or without pruritus. Although one patient had

  14. Congenital platelet function defects

    Science.gov (United States)

    ... pool disorder; Glanzmann's thrombasthenia; Bernard-Soulier syndrome; Platelet function defects - congenital ... Congenital platelet function defects are bleeding disorders that cause reduced platelet function. Most of the time, people with these disorders have ...

  15. Congenital Heart Information Network

    Science.gov (United States)

    ... heart defects. Important Notice The Congenital Heart Information Network website is temporarily out of service. Please join ... and Uwe Baemayr for The Congenital Heart Information Network Exempt organization under Section 501(c)3. Copyright © ...

  16. Congenital heart disease

    Science.gov (United States)

    Congenital heart disease (CHD) is a problem with the heart's structure and function that is present at birth. ... Fraser CD, Kane LC. Congenital heart disease. In: Townsend CM Jr, ... Sabiston Textbook of Surgery: The Biological Basis of Modern ...

  17. Evaluation of portosystemic collaterals by MDCT-MPR imaging for management of hemorrhagic esophageal varices

    International Nuclear Information System (INIS)

    Kodama, Hideaki; Aikata, Hiroshi; Takaki, Shintaro; Azakami, Takahiro; Katamura, Yoshio; Kawaoka, Tomokazu; Hiramatsu, Akira; Waki, Koji; Imamura, Michio; Kawakami, Yoshiiku; Takahashi, Shoichi; Toyota, Naoyuki; Ito, Katsuhide; Chayama, Kazuaki

    2010-01-01

    Objective: To study the correlation between changes in portosystemic collaterals, evaluated by multidetector-row computed tomography imaging using multiplanar reconstruction (MDCT-MPR), and prognosis in patients with hemorrhagic esophageal varices (EV) after endoscopic treatment. Methods: Forty-nine patients with primary hemostasis for variceal bleeding received radical endoscopic treatment: endoscopic injection sclerotherapy (EIS) or endoscopic variceal ligation (EVL). Patients were classified according to the rate of reduction in feeding vessel diameter on MDCT-MPR images, into the narrowing (n = 24) and no-change (n = 25) groups. We evaluated changes in portosystemic collaterals by MDCT-MPR before and after treatment, and determined rebleeding and survival rates. Results: The left gastric and paraesophageal (PEV) veins were recognized as portosystemic collaterals in 100 and 80%, respectively, of patients with EV on MDCT-MPR images. The rebleeding rates at 1, 2, 3, and 5 years after endoscopic treatment were 10, 15, 23, and 23%, respectively, for the narrowing group, and 17, 24, 35, and 67%, respectively, for the no-change group (P = 0.068). Among no-change group, the rebleeding rate in patients with large PEV was significantly lower than that with small PEV (P = 0.027). The rebleeding rate in patients with small PEV of the no-change group was significantly higher than that in the narrowing group (P = 0.018). There was no significant difference in rebleeding rates between the no-change group with a large PEV and narrowing group (P = 0.435). Conclusion: Changes in portosystemic collaterals evaluated by MDCT-MPR imaging correlate with rebleeding rate. Evaluation of portosystemic collaterals in this manner would provide useful information for the management of hemorrhagic EV.

  18. Preoperative MR angiography evaluation of transjugular intrahepatic portosystemic shunt

    International Nuclear Information System (INIS)

    Guo Li; Yang Dakuan; Yuan Shuguang; Yan Dong; Wang Jiaping; Li Yingchun

    2010-01-01

    Objective: To discuss the application of MR angiography (MRA) in deciding the puncture points of transjugular intrahepatic portosystemic shunt (TIPS). Methods: Preoperative MRA was performed in 59 patients with portal hypertension (study group) in order to search for the causes of portal hypertension, to observe the patterns and route of the hepatic and portal veins and to measure the vascular diameter at the scheduled puncture site. MRA was also performed in 50 healthy subjects, which was served as the control group. The results were compared between two groups. Results: The diseases in the study group included simple cirrhosis (n=49), cirrhosis accompanied with hepatocellular carcinoma (n=4), pure portal vein thrombosis (n=3), splenic vein stenosis (n=1) and Budd-Chiari syndrome (n=2). In study group the type I, II and III of the hepatic vein classification were seen in 14, 39 and 12 cases respectively,while in control group in 12, 34 and 14 cases respectively. In study group, the right, middle and left hepatic vein which had the diameter larger enough for puncturing existed in 52, 40 and 28 cases respectively, while in control group in 46, 34 and 23 cases respectively. The safe point for puncture via the right and the left branch of the portal vein was located beyond the distance of (16.2 ± 3.1) mm and (14.2 ± 3.8) mm respectively. Conclusion: MRA is a valuable non-invasive examination, which is of great value in determining the causes of portal hypertension and in planning the puncturing sites before performing TIPS. (authors)

  19. Immunohistochemical analysis of restenotic tissue after transjugular portosystemic shunt

    International Nuclear Information System (INIS)

    Lu Qin; An Yanli; Deng Gang; Fang Wen; Zhu Guangyu; Li Guozhao; Wei Xiaoying; Liu Yuanyuan; Teng Gaojun

    2005-01-01

    Objective: To investigate the changes of several restenotic tissue elements after transjugular portosystemic shunt, and to provide more informations for the mechanism of TIPS restenosis. Methods: TIPS was performed in 6 swine to set up TIPS animal models. 14-21 days after operation, the models were sacrificed to obtain the TIPS tissues for pathological examinations, including electric microscope, HE staining, and immunohistochemical staining of anti-SMC-actin-α, PCNA, Vementin, myoglobulin, eNOS and iNOS. Then , the results were comparatively analyzed between TIPS obstructed shunt tissues and non-obstructed shunt tissues. Results: Restenosis was occurred with different degrees in 4 swine of the 6 TIPS models. Electric microscopic results showed that the restenosis tissues were composed of over proliferated collagen, SMCs and fibroblasts. Anti-SMC-actin-α and PCNA were strongly positive expression in restenotic tissues, and also positive in patent tissues. Vimentin expressed strongly in unstenotic tissues, on the contrary, it expressed obviously weaker in restenotic tissues. Myoglobulin expressed more strongly in restenotic tissues and weakened in unstenotic tissues. eNOS expressed positive in normal liver tissues, and expressed weaker near TIPS restenotic tissues. iNOS showed stronger expression in restenotic tissues and could hardly expressed in normal liver tissues. Conclusions: Restenotic rate may be 67% in TIPS swine models. Restenotic tissues may be mainly composed of proliferated SMCs positively expressed anti-SMC-actin-α with strong ability of movement. eNOS may be expressed in normal liver tissues and instead iNOS be expressed in strongly injured liver tissues. (authors)

  20. Reversal of Transjugular Intrahepatic Portosystemic Shunt (TIPS)-Induced Hepatic Encephalopathy Using a Strictured Self-Expanding Covered Stent

    International Nuclear Information System (INIS)

    Cox, Mitchell W.; Soltes, George D.; Lin, Peter H.; Bush, Ruth L.; Lumsden, Alan B.

    2003-01-01

    Hepatic encephalopathy is a known complication following percutaneous transjugular intrahepatic portosystemic shunt (TIPS) placement. We describe herein a simple and effective strategy of TIPS revision by creating an intraluminal stricture within a self-expanding covered stent, which is deployed in the portosystemic shunt to reduce the TIPS blood flow. This technique was successful in reversing a TIPS-induced hepatic encephalopathy in our patient

  1. Identification and treatment of variation of extrahepatic bile duct in laparoscopic cholecystectomy

    Directory of Open Access Journals (Sweden)

    PENG Lei

    2015-10-01

    Full Text Available ObjectiveTo investigate the identification and treatment of variation of extrahepatic bile duct in laparoscopic cholecystectomy (LC, and to reduce the occurrence of bile duct injury. MethodsThis study included 60 patients who received LC in the People′s Hospital of Caidian District in Wuhan and had structural variation of extrahepatic bile duct found during the operation from January 2012 to January 2014. The clinical data were retrospectively analyzed, and the intraoperative and postoperative conditions were summarized. ResultsDuring operation, cystic duct variation was found in 32 cases, abnormal position of the point where the cystic duct joins the extrahepatic bile duct in 20 cases, the cystic duct and the common hepatic duct having the common wall before joining the common bile duct in 2 cases, aberrant bile duct in the gallbladder bed in 2 cases, and accessory hepatic duct in 4 cases. Fifty-one patients (85% successfully underwent LC; 9 patients (15% were converted to open surgery. All patients finished surgery successfully. There were 2 cases of postoperative complications; one patient developed residual stones in the bile duct, and bile leakage occurred in the other patient at one week after LC, who recovered after reoperation. All patients were cured and discharged, without severe complications such as intraperitoneal hemorrhage, infection, and intestinal injury. ConclusionIdentifying the structural variation of extrahepatic bile duct, dissecting the Calot′s triangle meticulously, and determining the type of variation of extrahepatic bile duct play important roles in LC and significantly reduce the incidence of bile duct injury.

  2. Radiation Exposure in Transjugular Intrahepatic Portosystemic Shunt Creation

    Energy Technology Data Exchange (ETDEWEB)

    Miraglia, Roberto, E-mail: rmiraglia@ismett.edu; Maruzzelli, Luigi, E-mail: lmaruzzelli@ismett.edu; Cortis, Kelvin, E-mail: kelvincortis@ismett.edu [Mediterranean Institute for Transplantation and Advanced Specialized Therapies (ISMETT), Radiology Service, Department of Diagnostic and Therapeutic Services (Italy); D’Amico, Mario, E-mail: mdamico@ismett.edu [University of Palermo, Department of Radiology (Italy); Floridia, Gaetano, E-mail: gfloridia@ismett.edu; Gallo, Giuseppe, E-mail: ggallo@ismett.edu; Tafaro, Corrado, E-mail: ctafaro@ismett.edu; Luca, Angelo, E-mail: aluca@ismett.edu [Mediterranean Institute for Transplantation and Advanced Specialized Therapies (ISMETT), Radiology Service, Department of Diagnostic and Therapeutic Services (Italy)

    2016-02-15

    PurposeTransjugular intrahepatic portosystemic shunt (TIPS) creation is considered as being one of the most complex procedures in abdominal interventional radiology. Our aim was twofold: quantification of TIPS-related patient radiation exposure in our center and identification of factors leading to reduced radiation exposure.Materials and methodsThree hundred and forty seven consecutive patients underwent TIPS in our center between 2007 and 2014. Three main procedure categories were identified: Group I (n = 88)—fluoroscopic-guided portal vein targeting, procedure done in an image intensifier-based angiographic system (IIDS); Group II (n = 48)—ultrasound-guided portal vein puncture, procedure done in an IIDS; and Group III (n = 211)—ultrasound-guided portal vein puncture, procedure done in a flat panel detector-based system (FPDS). Radiation exposure (dose-area product [DAP], in Gy cm{sup 2} and fluoroscopy time [FT] in minutes) was retrospectively analyzed.ResultsDAP was significantly higher in Group I (mean ± SD 360 ± 298; median 287; 75th percentile 389 Gy cm{sup 2}) as compared to Group II (217 ± 130; 178; 276 Gy cm{sup 2}; p = 0.002) and Group III (129 ± 117; 70; 150 Gy cm{sup 2}p < 0.001). The difference in DAP between Groups II and III was also significant (p < 0.001). Group I had significantly longer FT (25.78 ± 13.52 min) as compared to Group II (20.45 ± 10.87 min; p = 0.02) and Group III (19.76 ± 13.34; p < 0.001). FT was not significantly different between Groups II and III (p = 0.73).ConclusionsReal-time ultrasound-guided targeting of the portal venous system during TIPS creation results in a significantly lower radiation exposure and reduced FT. Further reduction in radiation exposure can be achieved through the use of modern angiographic units with FPDS.

  3. [Surgical treatment of intraoperative injuries and cicatricial strictures of extrahepatic bile ducts].

    Science.gov (United States)

    Tret'iakov, A A; Slepykh, N I; Kornilov, A K; Karimov, Z Kh

    1998-01-01

    The analysis of 70 cases of surgical treatment for intraoperative injuries and cicatricial strictures of extrahepatic bile ducts was carried out. In 25 patients surgical procedure was restorative and in 45--reconstructiver. Most common causes of corrective operations were: iatrogenic injuries of extrahepatic bile ducts (14) and cicatricial strictures of hepaticocholedochal duct due to intraoperative trauma (31). The problems of operative technique in performing biliobilio-, hepato-hepatico and hepatico-jejuno-anastomoses are considered. There were three deaths in the early postoperative period: 2 patients died of hepatic failure, pyogenic cholangiogenic intoxication caused by cholangioectasies and intrahepatic abscesses, and 1-due to generalyzed peritonitis caused by acute gastric ulcer perforation. Special attention is paid to the choice of the method of prolonged drainage used in reconstructive as well as in restorative operations.

  4. Cryoglobulinemia and its correlation with clinical extrahepatic manifestations in chronic hepatitis C

    Directory of Open Access Journals (Sweden)

    Joanna Jabłońska

    2017-12-01

    Results: Cryoglobulins were found in 93 persons (37.8%. Type II cryoglobulinemia was detected in 28 persons. Patients with cryoglobulinemia were elder (p<0.0004. 54 patients (21.95% manifested clinical extrahepatic symptoms. Arthralgia was found in 27 cases (10.98%. Skin changes were found in 22 patients (8.94%. 12 persons had glomerulonephritis (4.88%. 11 patients (4.47% had sicca syndrome. 6 patients developed peripheral polyneuropathy (2.4%. 4 persons developed B cell lymphoma (1.63%. There was no correlation between presence of symptoms and grading, staging, age, HCV genotype, and the presence of autoantibodies. Extrahepatic manifestations were present more frequently in women (p<0.0008.

  5. Non-Alcoholic Fatty Liver Disease and Extra-Hepatic Cancers

    Directory of Open Access Journals (Sweden)

    Claudia Sanna

    2016-05-01

    Full Text Available Non-alcoholic fatty liver disease (NAFLD is a leading cause of chronic liver disease but the second cause of death among NAFLD patients are attributed to malignancies at both gastrointestinal (liver, colon, esophagus, stomach, and pancreas and extra-intestinal sites (kidney in men, and breast in women. Obesity and related metabolic abnormalities are associated with increased incidence or mortality for a number of cancers. NAFLD has an intertwined relationship with metabolic syndrome and significantly contributes to the risk of hepatocellular carcinoma (HCC, but recent evidence have fuelled concerns that NAFLD may be a new, and added, risk factor for extra-hepatic cancers, particularly in the gastrointestinal tract. In this review we critically appraise key studies on NAFLD-associated extra-hepatic cancers and speculate on how NAFLD may influence carcinogenesis at these sites.

  6. Portal hypertension: a review of portosystemic collateral pathways and endovascular interventions

    International Nuclear Information System (INIS)

    Pillai, A.K.; Andring, B.; Patel, A.; Trimmer, C.; Kalva, S.P.

    2015-01-01

    The portal vein is formed at the confluence of the splenic and superior mesenteric vein behind the head of the pancreas. Normal blood pressure within the portal system varies between 5 and 10 mmHg. Portal hypertension is defined when the gradient between the portal and systemic venous blood pressure exceeds 5 mmHg. The most common cause of portal hypertension is cirrhosis. In cirrhosis, portal hypertension develops due to extensive fibrosis within the liver parenchyma causing increased vascular resistance. In addition, the inability of the liver to metabolise certain vasodilators leads to hyperdynamic splanchnic circulation resulting in increased portal blood flow. Decompression of the portal pressure is achieved by formation of portosystemic collaterals. In this review, we will discuss the pathophysiology, anatomy, and imaging findings of spontaneous portosystemic collaterals and clinical manifestations of portal hypertension with emphasis on the role of interventional radiology in the management of complications related to portal hypertension

  7. Percutaneous treatment of extrahepatic bile duct stones assisted by balloon sphincteroplasty and occlusion balloon

    Energy Technology Data Exchange (ETDEWEB)

    Park, Yong Sung; Kim, Ji Hyung; Choi, Young Woo; Lee, Tae Hee; Hwang, Cheol Mog; Cho, Young Jun; Kim, Keum Won [Konyang University Hospital, Daejeon (Korea, Republic of)

    2005-12-15

    To describe the technical feasibility and usefulness of extrahepatic biliary stone removal by balloon sphincteroplasty and occlusion balloon pushing. Fifteen patients with extrahepatic bile duct stones were included in this study. Endoscopic stone removal was not successful in 13 patients, and two patients refused the procedure due to endoscopy phobia. At first, all patients underwent percutaneous transhepatic biliary drainage (PTBD). A few days later, through the PTBD route, balloon assisted dilatation for common bile duct (CBD) sphincter was performed, and then the stones were pushed into the duodenum using an 11.5 mm occlusion balloon. Success rate, reason for failure, and complications associated with the procedure were evaluated. Eight patients had one stone, five patients had two stones, and two patients had more than five stones. The procedure was successful in 13 patients (13/15). In 12 of the patients, all stones were removed in the first trial. In one patients, residual stones were discovered on follow-up cholangiography, and were subsequently removed in the second trial. Technical failure occurred in two patients. Both of these patients had severely dilated CBD and multiple stones with various sizes. Ten patients complained of pain in the right upper quadrant and epigastrium of the abdomen immediately following the procedure, but there were no significant procedure-related complications such as bleeding or pancreatitis. Percutaneous extrahepatic biliary stone removal by balloon sphincteroplasty and subsequent stone pushing with occlusion balloon is an effective, safe, and technically feasible procedure which can be used as an alternative method in patients when endoscopic extrahepatic biliary stone removal was not successful.

  8. Extrahepatic Blood Supply to Hepatocellular Carcinoma: Angiographic Demonstration and Transcatheter Arterial Chemoembolization

    International Nuclear Information System (INIS)

    Miyayama, Shiro; Matsui, Osamu; Taki, Keiichi; Minami, Tetsuya; Ryu, Yasuji; Ito, Chiharu; Nakamura, Koichi; Inoue, Dai; Notsumata, Kazuo; Toya, Daisyu; Tanaka, Nobuyoshi; Mitsui, Takeshi

    2006-01-01

    Purpose. To evaluate the incidence of each extrahepatic collateral pathway to hepatocellular carcinoma (HCC) and to assess technical success rates and complications of transcatheter arterial chemoembolization (TACE) through each collateral. Methods. We retrospective evaluated extrahepatic collateral pathways to HCC on angiography in 386 procedures on 181 consecutive patients. One hundred and seventy patients had previously undergone TACE. TACE through extrahepatic collaterals using iodized oil and gelatin sponge particles was performed when a catheter was advanced into the tumor-feeding branch to avoid nontarget embolization. Results. A single collateral was revealed in 275 TACE procedures, two were revealed in 74, and three or more were revealed in 34. Incidences of collateral source to HCC were 83% from the right inferior phrenic artery (IPA), 24% from the cystic artery, 13% from the omental artery, 12% from the right renal capsular artery (RCA) and left IPA, 8% from the right internal mammary artery (IMA) and right intercostal artery (ICA), and 7% from the right inferior adrenal artery (IAA). Technical success rates of TACE were 53% in the right ICA, 70% in the cystic artery, 74% in the omental artery, 93% in the left IPA, 96% in the right IPA, and 100% in the right RCA, right IMA, and right IAA. Complications included skin necrosis after TACE through the right IMA (n = 1), cholecystitis after TACE through the cystic artery (n = 1), and ulcer formation after TACE through the right gastric artery (n = 1), in addition to pleural effusion and basal atelectasis after TACE through the IPA and IMA. Conclusion. Our study suggests that TACE through extrahepatic collaterals is possible with high success rates, and is also relatively safe

  9. Role of diffusion-weighted magnetic resonance imaging in the diagnosis of extrahepatic cholangiocarcinoma

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    AIM: To determine the clinical value of diffusion-weight- ed imaging (DWI) for the diagnosis of extrahepatic cholangiocarcinoma (EHCC) by comparing the diagnostic sensitivity of DWI and magnetic resonance cholan-giopancreatography (MRCP). METHODS: Magnetic resonance imaging examination was performed in 56 patients with suspected EHCC. T1- weighted imaging, T2-weighted imaging, MRCP and DWI sequence, DWI using single-shot spin-echo echoplanar imaging sequence with different b values (100, 300, 500, 800 and 1...

  10. A Technique to Define Extrahepatic Biliary Anatomy Using Robotic Near-Infrared Fluorescent Cholangiography.

    Science.gov (United States)

    Maker, Ajay V; Kunda, Nicholas

    2017-11-01

    Bile duct injury is a rare but serious complication of minimally invasive cholecystectomy. Traditionally, intraoperative cholangiogram has been used in difficult cases to help delineate anatomical structures, however, new imaging modalities are currently available to aid in the identification of extrahepatic biliary anatomy, including near-infrared fluorescent cholangiography (NIFC) using indocyanine green (ICG).1-5 The objective of the study was to evaluate if this technique may aid in safe dissection to obtain the critical view. Thirty-five consecutive multiport robotic cholecystectomies using NIFC with ICG were performed using the da Vinci Firefly Fluorescence Imaging System. All patients received 2.5 mg ICG intravenously at the time of intubation, followed by patient positioning, draping, and establishment of pneumoperitoneum. No structures were divided until the critical view of safety was achieved. Real-time toggling between NIFC and bright-light illumination was utilized throughout the case to define the extrahepatic biliary anatomy. ICG was successfully administered to all patients without complication, and in all cases the extrahepatic biliary anatomy was able to be identified in real-time 3D. All procedures were completed without biliary injury, conversion to an open procedure, or need for traditional cholangiography to obtain the critical view. Specific examples of cases where x-ray cholangiography or conversion to open was avoided and NIFC aided in safe dissection leading to the critical view are demonstrated, including (1) evaluation for aberrant biliary anatomy, (2) confirmation of non-biliary structures, and (3) use in cases where the infundibulum is fused to the common bile duct. NIFC using ICG is demonstrated as a useful technique to rapidly identify and aid in the visualization of extrahepatic biliary anatomy. Techniques that selectively utilize this technology specifically in difficult cases where the anatomy is unclear are demonstrated in order

  11. Resection of Concomitant Hepatic and Extrahepatic Metastases from Colorectal Cancer - A Worthwhile Operation?

    Science.gov (United States)

    Diaconescu, Andrei; Alexandrescu, Sorin; Ionel, Zenaida; Zlate, Cristian; Grigorie, Razvan; Brasoveanu, Vladislav; Hrehoret, Doina; Ciurea, Silviu; Botea, Florin; Tomescu, Dana; Droc, Gabriela; Croitoru, Adina; Herlea, Vlad; Boros, Mirela; Grasu, Mugur; Dumitru, Radu; Toma, Mihai; Ionescu, Mihnea; Vasilescu, Catalin; Popescu, Irinel

    2017-01-01

    Background: The benefit of hepatic resection in case of concomitant colorectal hepatic and extrahepatic metastases (CHEHMs) is still debatable. The purpose of this study is to assess the results of resection of hepatic and extrahepatic metastases in patients with CHEHMs in a high-volume center for both hepatobiliary and colorectal surgery and to identify prognostic factors that correlate with longer survival in these patients. It was performed a retrospective analysis of 678 consecutive patients with liver resection for colorectal cancer metastases operated in a single Centre between April 1996 and March 2016. Among these, 73 patients presented CHEHMs. Univariate analysis was performed to identify the risk factors for overall survival (OS) in these patients. Results: There were 20 CHMs located at the lymphatic node level, 20 at the peritoneal level, 12 at the ovary and lung level, 12 presenting as local relapses and 9 other sites. 53 curative resections (R0) were performed. The difference in overall survival between the CHEHMs group and the CHMs group is statistically significant for the entire groups (p 0.0001), as well as in patients who underwent R0 resection (p 0.0001). In CHEHMs group, the OS was statistically significant higher in patients who underwent R0 resection vs. those with R1/R2 resection (p=0.004). Three variables were identified as prognostic factors for poor OS following univariate analysis: 4 or more hepatic metastases, major hepatectomy and the performance of operation during first period of the study (1996 - 2004). There was a tendency toward better OS in patients with ovarian or pulmonary location of extrahepatic disease, although the difference was not statistically significant. In patients with concomitant hepatic and extrahepatic metastases, complete resection of metastatic burden significantly prolong survival. The patients with up to 4 liver metastases, resectable by minor hepatectomy benefit the most from this aggressive onco

  12. Percutaneous treatment of extrahepatic bile duct stones assisted by balloon sphincteroplasty and occlusion balloon

    International Nuclear Information System (INIS)

    Park, Yong Sung; Kim, Ji Hyung; Choi, Young Woo; Lee, Tae Hee; Hwang, Cheol Mog; Cho, Young Jun; Kim, Keum Won

    2005-01-01

    To describe the technical feasibility and usefulness of extrahepatic biliary stone removal by balloon sphincteroplasty and occlusion balloon pushing. Fifteen patients with extrahepatic bile duct stones were included in this study. Endoscopic stone removal was not successful in 13 patients, and two patients refused the procedure due to endoscopy phobia. At first, all patients underwent percutaneous transhepatic biliary drainage (PTBD). A few days later, through the PTBD route, balloon assisted dilatation for common bile duct (CBD) sphincter was performed, and then the stones were pushed into the duodenum using an 11.5 mm occlusion balloon. Success rate, reason for failure, and complications associated with the procedure were evaluated. Eight patients had one stone, five patients had two stones, and two patients had more than five stones. The procedure was successful in 13 patients (13/15). In 12 of the patients, all stones were removed in the first trial. In one patients, residual stones were discovered on follow-up cholangiography, and were subsequently removed in the second trial. Technical failure occurred in two patients. Both of these patients had severely dilated CBD and multiple stones with various sizes. Ten patients complained of pain in the right upper quadrant and epigastrium of the abdomen immediately following the procedure, but there were no significant procedure-related complications such as bleeding or pancreatitis. Percutaneous extrahepatic biliary stone removal by balloon sphincteroplasty and subsequent stone pushing with occlusion balloon is an effective, safe, and technically feasible procedure which can be used as an alternative method in patients when endoscopic extrahepatic biliary stone removal was not successful

  13. Expression of Bcl-2 and Bax in extrahepatic biliary tract carcinoma and dysplasia

    Science.gov (United States)

    Li, Sheng-Mian; Yao, Shu-Kun; Yamamura, Nobuyoshi; Nakamura, Toshitsugu

    2003-01-01

    AIM: To compare the difference of expression of Bcl-2 and Bax in extrahepatic biliary tract carcinoma and dysplasia, and to analyze the role of Bcl-2 and Bax proteins in the progression from dysplasia to carcinoma and to evaluate the correlation of Bcl-2/Bax protein expression with the biological behaviors. METHODS: Expressions of Bcl-2 and Bax were examined immunohistochemically in 27 cases of extrahepatic biliary tract carcinomas (bile duct carcinoma: n = 21, carcinoma of ampulla of Vater: n = 6), and 10 cases of atypical dysplasia. Five cases of normal biliary epithelial tissues were used as controls. A semiquantitative scoring system was used to assess the Bcl-2 and Bax reactivity. RESULTS: The expression of Bcl-2 was observed in 10 out of 27 (37.0%) invasive carcinomas, 1 out of 10 dysplasias, none out of 5 normal epithelial tissues. Bax expression rate was 74.1% (20/27) in invasive carcinoma, 30% (3/10) in dysplasia, and 40% (2/5) in normal biliary epithelium. Bcl-2 and Bax activities were more intense in carcinoma than in dysplasia, with no significant difference in Bcl-2 expression (P = 0.110), and significant difference in Bax expression (P = 0.038). Level of Bax expression was higher in invasive carcinoma than in dysplasia and normal tissue (P = 0.012). Bcl-2 expression was correlated to Bax expression (P = 0.0059). However, Bcl-2/Bax expression had no correlation with histological subtype, grade of differentiation, or level of invasion. CONCLUSION: Increased Bcl-2/Bax expression from dysplasia to invasive tumors supports the view that this is the usual route for the development of extrahepatic biliary tract carcinoma. Bcl-2/Bax may be involved, at least in part, in the apoptotic activity in extrahepatic biliary carcinoma. PMID:14606101

  14. Porto-systemic collaterals in cirrhosis of the liver. Selective percutaneous transhepatic catheterization of the portal venous system in portal hypertension

    Energy Technology Data Exchange (ETDEWEB)

    Hoevels, J; Lunderquist, A; Tylen, U; Simert, G [Lund Univ. (Sweden)

    1979-01-01

    In 93 patients with cirrhosis of the liver and portal venous hypertension the main tributaries of the portal vein were examined by percutaneous transhepatic catheterization. The appearance and degree of porto-systemic collaterals were analysed. Esophageal varices were demonstrated in 82 patients. No correlation was found between the portal venous pressure and the extent of porto-systemic communications.

  15. Reversibility of hyperintense globus pallidus on T 1-weighted MRI following surgery for a portosystemic shunt in an 8-year-old girl

    International Nuclear Information System (INIS)

    Ikeda, Shinji; Sera, Yoshihisa; Yoshida, Mituhiro; Ohshiro, Hajime; Uchino, Shinichiro; Seguchi, Sasa; Endo, Fumio

    1999-01-01

    An 8-year-old Japanese girl with a portosystemic shunt had shown hyperammonaemia since she was 3 years of age. MRI of her brain showed bilateral hyperintense globus pallidus. A portosystemic shunt was evident on US and angiography. She underwent surgical banding of the shunt, after which the lesion and clinical symptoms disappeared. (orig.)

  16. Congenital orbital teratoma

    OpenAIRE

    Aiyub, Shereen; Chan, Weng Onn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

    2013-01-01

    We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The p...

  17. Restoration of Liver Function and Portosystemic Pressure Gradient after TIPSS and Late TIPSS Occlusion

    International Nuclear Information System (INIS)

    Maedler, U.; Hansmann, J.; Duex, M.; Noeldge, G.; Sauer, P.; Richter, G.M.

    2002-01-01

    TIPSS (transjugular intrahepatic portosystemic shunt) may be indicated to control bleeding from esophageal and gastric varicose veins, to reduce ascites, and to treat patients with Budd-Chiari syndrome and veno-occlusive disease. Numerous measures to improve the safety and methodology of the procedure have helped to increase the technical and clinical success. Follow-up of TIPSS patients has revealed shunt stenosis to occur more often in patients with preserved liver function (Child A, Child B). In addition, the extent of liver cirrhosis is the main factor that determines prognosis in the long term. Little is known about the effects of TIPSS with respect to portosystemic hemodynamics. This report deals with a cirrhotic patient who stopped drinking 7 months prior to admission. He received TIPSS to control ascites and recurrent esophageal bleeding. Two years later remarkable hypertrophy of the left liver lobe and shunt occlusion was observed. The portosystemic pressure gradient dropped from 24 mmHg before TIPSS to 11 mmHg and remained stable after shunt occlusion. The Child's B cirrhosis prior to TIPSS turned into Child's A cirrhosis and remained stable during the follow-up period of 32 months. This indicates that liver function of TIPSS patients may recover due to hypertrophy of the remaining non-cirrhotic liver tissue. In addition the hepatic hemodynamics may return to normal. In conclusion, TIPSS cannot cure cirrhosis but its progress may be halted if the cause can be removed. This may result in a normal portosystemic gradient, leading consequently to shunt occlusion

  18. Treatment of Portosystemic Shunt Myelopathy with a Stent Graft Deployed through a Transjugular Intrahepatic Route

    International Nuclear Information System (INIS)

    Jain, Deepak; Arora, Ankur; Deka, Pranjal; Mukund, Amar; Bhatnagar, Shorav; Jindal, Deepti; Kumar, Niteen; Pamecha, Viniyendra

    2013-01-01

    A case of surgically created splenorenal shunt complicated with shunt myelopathy was successfully managed by placement of a stent graft within the splenic vein to close the portosystemic shunt and alleviate myelopathy. To our knowledge, this is the first report of a case of shunt myelopathy in a patient with noncirrhotic portal fibrosis without cirrhosis treated by a novel technique wherein a transjugular intrahepatic route was adopted to deploy the stent graft

  19. Effects of transjugular intrahepatic portosystemic shunt (TIPS) on blood volume distribution in patients with cirrhosis

    DEFF Research Database (Denmark)

    Busk, Troels M; Bendtsen, Flemming; Henriksen, Jens H

    2017-01-01

    increased (+22%, prestores central hypovolaemia......BACKGROUND: Cirrhosis is accompanied by portal hypertension with splanchnic and systemic arterial vasodilation, and central hypovolaemia. A transjugular intrahepatic portosystemic shunt (TIPS) alleviates portal hypertension, but also causes major haemodynamic changes. AIMS: To investigate effects...... catheterization. Central and arterial blood volume (CBV) and cardiac output (CO) were determined with indicator dilution technique. RESULTS: After TIPS, the thoracic blood volume increased (+10.4% of total blood volume (TBV), p

  20. Evaluation of late stage iatrogenic extrahepatic bile duct stricture by using magnetic resonance cholangiopancreagraphy

    International Nuclear Information System (INIS)

    Sun Changjin; Zhou Xiangping; Liu Rongbo; Song Bin; Yan Zhihan; Chen Xian; Wang Wentao; Xiong Yan; Xu Minsheng; Gu Jianping

    2003-01-01

    Objective: To assess the role of magnetic resonance cholangiopancreagraphy (MRCP) in the preoperative evaluation of late stage iatrogenic extrahepatic bile duct stricture. Methods: Eighteen cases, which were diagnosed as late stage iatrogenic extrahepatic bile duct strictures only by MRCP and proved by surgery and pathology, were classified according to Bismuth classification and the surgical operation strategy was planed on the basis of MR cholangiopancreagraphic findings before surgery. The results were compared with surgery. Results: Diagnostic MR Cholangiopancreagrams were acquired in 18 patients. Among all 18 patients, the level of stricture was classified as Bismuth I in 3 patients, Bismuth II in 7 patients, Bismuth III in 3 patients, and Bismuth IV in 3 patients. A Bismuth II stricture was incorrectly classified as a Bismuth III lesion. On the basis of MR cholangiopancreagraphic findings, a surgical operation strategy can be planed. The therapeutic plan anticipated with MRCP matched the actually used procedure in 16 of 18 patients. conclusion: MRCP plays an important role in the evaluation of late stage iatrogenic extrahepatic bile duct stricture

  1. Extrahepatic Portal Hypertension following Liver Transplantation: a Rare but Challenging Problem

    Directory of Open Access Journals (Sweden)

    B. Malassagne

    1998-01-01

    Full Text Available This study reports our experience of 8 cases of extrahepatic portal hypertension after 273 orthotopic liver transplantations in 244 adult patients over a 10- year period. The main clinical feature was ascites, and the life-threatening complication was variceal bleeding. Extrahepatic portal hypertension was caused by portal vein stenosis in 6 patients, and left-sided portal hypertension in 2 patients after inadventent ligation of portal venous tributaries or portasystemic shunts. All patients with portal vein stenosis had complete relief of portal hypertension after percutaneous transhepatic venoplasty (n=4 or surgical reconstruction (n=2, after a median follow-up of 33 (range: 6–62 months. Of the 2 patients with left-sided portal hypertension, one died after splenectomy and one rebled 6 months after left colectomy. This study suggests that extrahepatic portal hypertension is a series complication after liver transplantation that could be prevented by meticulous portal anastomosis and closure of portal tributaries or portasystemic shunts to improve the portal venous flow. However, any ligation has to be performed under ultrasound guidance to avoid inadventent venous ligations.

  2. Definition of ACLF and inclusion criteria for extra-hepatic organ failure.

    Science.gov (United States)

    Wang, Xiaojing; Sarin, Shiv Kumar; Ning, Qin

    2015-07-01

    A prominent characteristic of ACLF is rapid hepatic disease progression with subsequent extra-hepatic organ failure, manifesting as either hepatic coma or hepatorenal syndrome, which is associated with a high mortality rate in a short time. The APASL definition mainly emphasizes recognizing patients with hepatic failure. These patients may subsequently develop extra-hepatic multisystem organ failure leading to high mortality. It is therefore worthwhile to identify the short interim period between the development of liver failure and the onset of extra-hepatic organ failure, the potential therapeutic 'golden window.' Interventions during this period may prevent the development of complications and eventually change the course of the illness. Organ failure is suggested to be a central component of ACLF and may behave differently from chronic decompensated liver disease. Clear and practical criteria for the inclusion of organ failure are urgently needed so that patients with these life-threatening complications can be treated in a timely and appropriate manner. Recent studies suggested that the scoring systems evaluating organ failure [acute physiology, age and chronic health evaluation (APACHE) and sequential organ failure assessment (SOFA) scores] work better than those addressing the severity of liver disease [Child-Pugh and model of end-stage liver disease (MELD) scores] in ACLF. However, a key problem remains that the former scoring systems are reflective of organ failure and not predictive, thus limiting their value as an early indication for intervention.

  3. Cytomegalovirus Congenital Cataract

    Directory of Open Access Journals (Sweden)

    Ridha Wahyutomo

    2011-06-01

    Full Text Available Cytomegalovirus congenital infection is an infection caused by the the subfamily â Herpesviridae, during pregnancy. The incidence of infections among newborn infants is 1 %. One of the effects of congenitally acquired infection is the congenital cataract. A 6-year-old child complained to have a blurred vision diagnosed with cytomegalovirus congenital cataract. The diagnosis was confirmed by a positive serology testing for Ig M and Ig G CMV. The laboratory test using Giemsa staining to find inclusion bodies and a faster PCR could not be carried out (Sains Medika, 3(1:84-88.

  4. Genetics Home Reference: congenital hypothyroidism

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Congenital hypothyroidism Congenital hypothyroidism Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Congenital hypothyroidism is a partial or complete loss of function ...

  5. Portosystemic shunting in portal hypertension: evaluation with portal scintigraphy with transrectally administered I-123 IMP

    International Nuclear Information System (INIS)

    Kashiwagi, T.; Azuma, M.; Ikawa, T.; Takehara, T.; Matsuda, H.; Yoshioka, H.; Mitsutani, N.; Koizumi, T.; Kimura, K.

    1988-01-01

    Portosystemic shunting was evaluated with rectal administration of iodine-123 iodoamphetamine (IMP) in seven patients without liver disease and 53 patients with liver cirrhosis. IMP (2-3 mCi [74-111 MBq]) was administered to the rectum through a catheter. Images of the chest and abdomen were obtained for up to 60 minutes with a scintillation camera interfaced with a computer. In all patients, images of the liver and/or lungs were observed within 5-10 minutes and became clear with time. In patients without liver disease, only liver images could be obtained, whereas the lung was visualized with or without the liver in all patients with liver cirrhosis. The portosystemic shunt index was calculated by dividing counts of lungs by counts of liver and lung. These values were significantly higher in liver cirrhosis, especially in the decompensated stage. Transrectal portal scintigraphy with IMP appears to be a useful method for noninvasive and quantitative evaluation of portosystemic shunting in portal hypertension

  6. Risk factors for stent graft thrombosis after transjugular intrahepatic portosystemic shunt creation.

    Science.gov (United States)

    Jahangiri, Younes; Kerrigan, Timothy; Li, Lei; Prosser, Dominik; Brar, Anantnoor; Righetti, Johnathan; Schenning, Ryan C; Kaufman, John A; Farsad, Khashayar

    2017-12-01

    To identify risk factors of stent graft thrombosis after transjugular intrahepatic portosystemic shunt (TIPS) creation. Patients who underwent TIPS creation between June 2003 and January 2016 and with follow-up assessing stent graft patency were included (n=174). Baseline comorbidities, liver function, procedural details and follow-up liver function tests were analyzed in association with hazards of thrombosis on follow-up. Competing risk cox regression models were used considering liver transplant after TIPS creation as the competing risk variable. One-, 2- and 5-year primary patency rates were 94.1%, 91.7% and 78.2%, respectively. Patient age [sub-hazard ratio (sHR): 1.13; P=0.001], body mass index (BMI) value for trend=0.017). Older age, lower BMI and higher post-TIPS portosystemic gradients were associated with higher hazards of shunt thrombosis after TIPS creation using stent grafts. Higher rates of shunt thrombosis were seen in patients for whom TIPS creation was clinically unsuccessful. The association between TIPS thrombosis and higher post-TIPS portosystemic gradients may indicate impaired flow through the shunt, a finding which may be technical or anatomic in nature and should be assessed before procedure completion.

  7. Usefulness of color and pulsed Doppler's in the evaluation of surgical portosystemic shunts in pediatric patients

    International Nuclear Information System (INIS)

    Berrocal, T.; Prieto, C.; Cortes, P.; Rodriguez, R.; Pastor, I.

    2003-01-01

    Portosystemic shunts are performed to relieve symptomatic portal hypertension symptomatic or removal pressure in hepatic vascularisation in patients with Budd-Chiari's syndrome. Most surgical portosystemic shunts can be suitably studied by means of ultrasound scan complemented by color and pulsed Dopplers, proved one understands the hemodynamics of the surgical procedures involved. This article demonstrates the usefulness and limitations of the ultrasound scan Duplex Doppler in the evaluation of portosystemic shunts performed on pediatric patients. Pulsed Doppler provides information regarding the nature and direction of blood flow. Color doppler is capable of directly revealing the shunt and, in most cases, permits the anastomosis to be located. The types of shunts that appear include proximal and distal spleno-renal, portocaval and mesocaval. Types of vascular connections are illustrated,s well as expected post-surgical blood flow direction in affected vessels. The ultrasound scanning technique is discussed, as well as the criteria for determining vascular permeability. Also highlighted are the advantages, limitations and diagnostic difficulties associated with the different forms of Doppler. (Author) 17 refs

  8. Mitochondrial disorders in congenital myopathies

    Directory of Open Access Journals (Sweden)

    D. A. Kharlamov

    2014-01-01

    Full Text Available The literature review gives data on the role of mitochondrial disorders in the pathogenesis of congenital myopathies: congenital muscular dystrophies and congenital structural myopathies. It describes changes in congenital muscular dystrophies with type VI collagen, in myodystrophy with giant mitochondria, in congenital central core myopathies, myotubular myopathy, etc. Clinical and experimental findings are presented. Approaches to therapy for energy disorders in congenital myopathies are depicted.

  9. Congenital heat disease

    International Nuclear Information System (INIS)

    Higgins, C.B.; Silverman, N.H.; Kersting-Somerhoff, B.A.

    1990-01-01

    The book covers the tomographic anatomy of the normal and congenitally malformed heart and tomographic imaging of the normal heat. It then compares echocardiographic evaluation and the use of MR imaging in the diagnosis and evaluation of individual congenital cardiac malformations

  10. Key aspects congenital infection

    Directory of Open Access Journals (Sweden)

    Yu. V. Lobzin

    2014-01-01

    Full Text Available The key questions to solve the problem of congenital infection in the Russian Federation are: using in national practice over world accepted terminology adapted to the recommendations of the World Health Organization; representation of the modern concepts of an infectious process in the classification of congenital infections; scientific development and introducing in clinical practice the «standard case definitions», applied to different congenital infections; optimization of protocols and clinical guidelines for diagnosis, treatment and prevention of congenital infections; improvement a knowledge in the infectious disease for all  pecialists involved in the risk assessment of congenital infections, manage pregnancy and children. Based on our experience and analysis of publications, the authors suggest possible solutions.

  11. Prominent porto-systemic collateral pathways in patients with portal hypertension: demonstration by gadolinium-enhanced magnetic resonance angiography

    International Nuclear Information System (INIS)

    Caldana, Rogerio Pedreschi; Bezerra, Alexandre Araujo Sergio; Cecin, Alexnadre Oliveira; Souza, Luis Ronan Marques Ferreira de; Goldman, Susan Menasce; D'Ippolito, Giuseppe; Szejnfeld, Jacob

    2003-01-01

    To demonstrate the usefulness of gadolinium-enhanced magnetic resonance angiography in the evaluation of prominent porto-systemic collateral pathways. We reviewed the images from 40 patients with portal hypertension studied with gadolinium-enhanced magnetic resonance angiography and selected illustrative cases of prominent porto-systemic collateral pathways. The scans were performed using high field equipment (1.5 Tesla) and a 3 D volume technique. Image were obtained after intravenous injection of paramagnetic contrast media using a power injector. Magnetic resonance angiography demonstrated with precision the porto-systemic collateral pathways, particularly when investigating extensive territories or large vessels. The cases presented show the potential of this method in the investigation of patients with portal hypertension. Gadolinium-enhanced magnetic resonance angiography is a useful method for the evaluation of patients with portal hypertension and prominent collateral pathways. (author)

  12. Congenital symmastia revisited

    DEFF Research Database (Denmark)

    Sillesen, Nanna H; Hölmich, Lisbeth R; Siersen, Hans Erik

    2012-01-01

    Symmastia is defined as medial confluence of the breast. The term 'symmastia' is modified from Greek (syn meaning 'together', and mastos meaning 'breast') and was first presented by Spence et al. in 1983. Two forms of symmastia exist: an iatrogenic and a congenital version. Congenital symmastia...... is a rare condition in which web-like soft tissue traverses the sternum to connect the breasts medially. The literature on congenital symmastia is limited, few cases have been published, and knowledge about ideal treatment is still insufficient....

  13. The Role of Radiotherapy in the Treatment of Extrahepatic Bile Duct Carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Hyun Soo; Kim, Gwi Eon; Lee, Hyung Sik; Suh, Chang Ok; Loh, John Ku; Lee, Jong Tae [Yonsei National University College of Medicine, Seoul (Korea, Republic of)

    1991-12-15

    Twenty-seven patients with unresectable extrahepatic bile duct carcinoma (n=21) or with microscopic evidence of tumor rest after aggressive surgery for extrahepatic bile duct carcinoma(n=6) between 1985 and 1990 were given radiotherapy consisting intentionally external radiotherapy and /or intraluminal therapy using Gamma-Med 12i (192-lr) high dose rate (HDR) remote control afterloading system following bile drainage procedures and Gianturco stent insertion. The objectives of this study has been to assess the feasibility and effects on survival of a combination of external radiotherapy and brachytherapy with which we hope to achieve optimal loco-regional control for patients with unresectable extrahepatic bile duct tumors. Sixteen patients were men and eleven were women, and the mean age was 58 years (34-70 ). 10MV X-ray was used for radiation therapy, with the total dose ranging from 45 Gy to 55 Gy, and intraluminal brachytherapy performed after external radiotherapy, with the dose of total 15 Gy. The minimum follow up was 12 months. Failure were predominantly local-regional, without distant failure. Median survival was 10 months; 2-year actuarial survival rates was 21%. Median survival for common hepatic duct(CHD) cancer was 9 months; for common bile duct (CBD) cancer, was 16 months. And median survival for incomplete surgery/external radiotherapy group and external/intraluminal radiotherapy group was 10 months; for external radiotherapy alone group, was 6 months. Use of chemotherapy and/or hyperthermia were not affected in survival. Therefore, our result is that the survival rates in the group of external/intraluminal radiotherapy were comparable with ones in the group of incomplete resection/external radiotherapy, and so we believe that the aggressive local and regional radiotherapy can improve the quality of life and the survival length.

  14. Extrahepatic biliary atresia with choledochal cyst: Prenatal MRI predicted and post natally confirmed: A case report

    Directory of Open Access Journals (Sweden)

    Madhavi Nori

    2013-01-01

    Full Text Available Extrahepatic biliary atresia (EHBA is an uncommon cause of neonatal jaundice. Antenatal Magnetic Resonance Imaging (MRI diagnosis of EHBA has not been published to the best of our knowledge till date. EHBA with cystic component is likely to be mistaken for choledochal cyst. A case that was antenatally predicted and postnatally confirmed by surgery and histopathology is being reported. All imaging signs are analyzed herewith. Imaging helps in the prediction of EHBA and also helps in early postnatal surgical referral which in turn improves the results of Kasai′s portoenterostomy.

  15. Protein induced by vitamin K absence or antagonist-II production is a strong predictive marker for extrahepatic metastases in early hepatocellular carcinoma: a prospective evaluation

    International Nuclear Information System (INIS)

    Bae, Hyun-Mi; Lee, Jeong-Hoon; Yoon, Jung-Hwan; Kim, Yoon Jun; Heo, Dae Seog; Lee, Hyo-Suk

    2011-01-01

    Clinicians often experience extrahepatic metastases associated with hepatocellular carcinoma (HCC), even if no evidence of intrahepatic recurrence after treatment is observed. We investigated the pretreatment predictors of extrahepatic metastases in HCC patients. Patients diagnosed with HCC without evidence of extrahepatic metastases were prospectively enrolled. We evaluated the correlation between extrahepatic metastases and pretreatment clinical variables, including serum tumor markers. A total of 354 patients were included. Seventy-six patients (21%) had extrahepatic metastases during the observation period (median, 25.3 months; range, 0.6-51.3 months). Cox regression multivariate analysis showed that serum protein induced by vitamin K absence or antagonist-II (PIVKA-II) production levels, the intrahepatic tumor stage, platelet count, and portal vein thrombosis were independent risk factors for extrahepatic metastases. Patients with a PIVKA-II production ≥ 300 mAU/mL had a 2.7-fold (95% confidence interval; 1.5-4.8; P < 0.001) and 3.7-fold (95% confidence interval; 2.0-6.6; P < 0.001) increased risk for extrahepatic metastases after adjustment for stage, platelet count, alpha-fetoprotein ≥ 400 ng/mL, and portal vein thrombosis according to the AJCC and BCLC staging systems, respectively. PIVKA-II production levels might be a good candidate predictive marker for extrahepatic HCC metastases, especially in patients with smaller and/or fewer tumors in the liver with in stages regardless of serum alpha-fetoprotein

  16. Long-term follow-up of patients with a clinically benign extrahepatic biliary stenosis and K-ras mutation in endobiliary brush cytology

    NARCIS (Netherlands)

    van Heek, N. Tjarda; Rauws, Erik A. J.; Caspers, Eric; Drillenburg, Paul; Gouma, Dirk J.; Offerhaus, G. Johan A.

    2002-01-01

    Background. K-ras mutations in endobiliary brush cytology are an early event in carcinogenesis and justify a suspicion of malignancy in patients with extrahepatic biliary stenosis. However, K-ras mutations have been detected in specimens obtained by brushing of clinically benign extrahepatic biliary

  17. The diagnostic value of MRI and MRCP in congenital duct cysts and complications

    International Nuclear Information System (INIS)

    Xiao Fang; Huang Suiqiao; Hu Tao

    2009-01-01

    Objective: To investigate the value of MRI and MRCP in diagnosis of congenital duct cysts and their complications. Methods: The MRI and MRCP of 29 cases of congenital duct cyst verified by pathological findings were analyzed retrospectively. Results: In 29 cases of Congenital choledochocele, 10 of which are type lb and 7 were Ic, appearing as the focal choledochal ecstasis, which were connected with the bile duct tree and displayed by MRCP. 10 cases were type IV and 2 are type V, appearing as the multiple cholangiectasis extrahepaticly and/or intrahepaticly and displayed by MRCP as the multiple cystoid with different sizes. There were 7 cases with complication of calculus, 4 in type Ib and 3 in type IV, appearing as single or multiple non-enhanced lesion with clear border. There were 3 cases with complication of tumors, 1 cholangiocarcinoma in type IV and type V respectively, appearing as the enhanced nodus within the wall of dilatant bile duct, 1 gallbladder carcinoma in type lb with multiple liver metastasis. The interruption of distal choledochocele was demonstrated in the complication of biliary atresia in 1 of type V. There was pancreatitis in 1 of type IV. The diagnosis and typing of choledochocele can be accurately made by MRI combined with MRCP, and complications were easily and clearly showed. The accuracy rate for location of choledochocele was 100%. Conclusion: There is an important value for MRI and MRCP examination in diagnosis of congenital bile duct cysts and their complications. (authors)

  18. Congenital orbital teratoma.

    Science.gov (United States)

    Aiyub, Shereen; Chan, Wengonn; Szetu, John; Sullivan, Laurence J; Pater, John; Cooper, Peter; Selva, Dinesh

    2013-12-01

    We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  19. Congenital orbital teratoma

    Directory of Open Access Journals (Sweden)

    Shereen Aiyub

    2013-01-01

    Full Text Available We present a case of mature congenital orbital teratoma managed with lid-sparing exenteration and dermis fat graft. This is a case report on the management of congenital orbital teratoma. A full-term baby was born in Fiji with prolapsed right globe which was surrounded by a nonpulsatile, cystic mass. Clinical and imaging features were consistent with congenital orbital teratoma. Due to limited surgical expertise, the patient was transferred to Adelaide, Australia for further management. The patient underwent a lid-sparing exenteration with frozen section control of the apical margin. A dermis fat graft from the groin was placed beneath the lid skin to provide volume. Histopathology revealed mature tissues from each of the three germ cell layers which confirmed the diagnosis of mature teratoma. We describe the successful use of demis fat graft in socket reconstruction following lid-sparing exenteration for congenital orbital teratoma.

  20. Congenital Constriction Band Syndrome

    OpenAIRE

    Rajesh Gupta, Fareed Malik, Rishabh Gupta, M.A.Basit, Dara Singh

    2008-01-01

    Congenital constriction bands are anomalous bands that encircle a digit or an extremity. Congenitalconstriction band syndrome is rare condition and is mostly associated with other musculoskeletaldisorders.We report such a rare experience.

  1. Congenital anterior urethral diverticulum

    International Nuclear Information System (INIS)

    Jung, Hyun Sub; Chung, Young Sun; Suh, Chee Jang; Won, Jong Jin

    1985-01-01

    Two cases of congenital anterior urethral diverticular which have occurred in a 4 year old and one month old boy are presented. Etiology, diagnostic procedures, and its clinical results are briefly reviewed

  2. Multidetector Computed Tomography Assessment in Biliary Atresia for the Diagnosis of Portosystemic Collaterals before Liver Transplant

    International Nuclear Information System (INIS)

    Cadavid A, Lina; Barber, Ignasi; Bueno, Javier

    2011-01-01

    Introduction: Chronic liver disease increases portal vein pressure and modifies splanchnic circulation. This is particularly significant in infants with biliary atresia. Large collaterals steal portal flow and increase the risk of post transplant portal vein thrombosis. Objective: to describe different types of portosystemic collaterals prior to liver transplantation with low-dose multidetector CT (MDCT) in patients with biliary atresia. Material and methods: 13 patients with severe liver dysfunction due to biliary atresia underwent low-dose 64-MDCT before liver transplantation (effective tube current ranged from 20 to 120 mAs according to weight, with a kilo voltage of 80-120 for all CT). Hepatic arterial and portal venous phases were performed after IV contrast administration [1.5-2 ml/kg]. The mean age of the study group was1 year (range, 4 months to 3.6 years). Two radiologists reviewed the CT images to determine the grade and types of the portosystemic collaterals. Results: A total of 16 CT scans were obtained.the most common portosystemic collaterals found were esophageal (11), gastric submucosal (8), gastric adventitial (7, splenic (7), hemorrhoidal (10), mesenteric [dilated or tortuous branches of the inferior mesenteric vein (8)], retroperitoneal varices [gastro renal shunt (10), splenorenal shunt (4)] and dilated or tortuous left gastric vein (13). Conclusion: MDCT provides important information on venous system patency, presence of varices, and location of venous shunts in pediatric patients with biliary atresia going to liver transplant. in addition, it is critical to detect collaterals that are not evident on ultrasound in order to avoid the steal phenomenon that may lead to portal vein thrombosis and graft failure.

  3. Usefulness of a balloon-expandable, covered stent for the transjugular intrahepatic portosystemic shunt

    Directory of Open Access Journals (Sweden)

    Rössle M

    2018-01-01

    Full Text Available The availability of polytetrafluoroethylene (PTFE covered, self-expandable nitinol stents in 2001 considerably improved the patency, response rates and survival of the transjugular intrahepatic portosystemic shunt (TIPS. Side effects of portosystemic shunting such as hepatic encephalopathy (HE and worsening of hepatic function, however, remained a problem. To reduce HE, underdilatation of nitinol stents has been practiced for many years. However, as shown recently, underdilatation was a flop since, due to their intrinsic memory, nitinol stents always expanded to reach their nominal diameter of 8 or 10 mm. To overcome this problem and to be able to perform permanent shunts with a smaller diameter of < 8 mm, we studied the usefulness of a balloon-expandable, covered, metallic stent which allowed adjustment to any diameter between 5 and 12 mm. Methods: 30 patients with cirrhosis and symptomatic portal hypertension were included. The mean Child-Pugh score was 8 ± 2.17 patients had refractory ascites, 9 patients variceal bleeding and four patients other indications for the TIPS. Results: The TIPS was successfully implanted in all patients within 69.6 ± 21.8 min. The shunt reduced the portosystemic pressure gradient by 57.5 ± 14.2% with a mean stent diameter of 7.4 ± 1.0 mm (5 -10.3 mm. During a mean follow-up of 330 ± 249 days, shunt revision was necessary in 5 patients (17%, four of them had insufficient response and received stent dilatation and one patient had stent misplacement requiring a parallel shunt. Three patients (10% developed HE. Conclusions: The covered, balloon-expandable stent could be placed accurately and allowed creation of adapted shunts with smaller diameters as usual. This resulted in a comparatively low rate of HE.

  4. Extrahepatic portal hypertension in childhood. Portographic findings and evaluation of hepatic blood flow by computerized radionuclide angiography

    Energy Technology Data Exchange (ETDEWEB)

    Takehara, Hiroo; Komi, Nobuhiko; Goh, Masahiro; Yoshida, Sadahiro; Kameoka, Kazuhiro; Hino, Masao; Sui, Osamu

    1986-05-01

    Four pediatric patients with extrahepatic portal hypertension had undergone portography and computerized radionuclide angiography in order to examine shape of portal system and to evaluate hepatic blood flow before and aftersurgical treatment. In all patients, cavernous transformation of portal system was demonstrated by portography, and in one of them spontaneous splenorenal shunt occurred. In 3 of them, who underwent esophageal transsection combined with paraesophagogastric devascularization and splenectomy, so-called direct operation, increase of portal blood flow was revealed by computerized radionuclide angiography. It is suggested that direct operation increasing portal blood flow after surgery is effective in treating extrahepatic portal hypertension in childhood.

  5. Ischemic Cholangitis Caused by Transcatheter Hepatic Arterial Chemoembolization 10 Months After Resection of the Extrahepatic Bile Duct

    International Nuclear Information System (INIS)

    Hasegawa, Kiyoshi; Kubota, Keiichi; Aoki, Taku; Hirai, Ichiro; Miyazawa, Masashi; Ohtomo, Kuni; Makuuchi, Masatoshi

    2000-01-01

    We report a case of ischemic cholangitis that occurred after transcatheter hepatic arterial chemoembolization (TAE). Ten months prior to TAE the patient had undergone central bisegmentectomy for hepatocellular carcinoma with resection of the extrahepatic bile duct. Eleven days after TAE, he developed suppurative cholangitis and multiple organ failure. Prior surgical ligation of the peribiliary arteries around the extrahepatic bile duct followed by TAE was considered to have played a crucial role in the development of ischemic cholangitis. This case demonstrates the importance of blood flow from the peribiliary arteries for the survival of the biliary epithelium

  6. Congenital chylous ascites

    International Nuclear Information System (INIS)

    Romanska-Kita, J.; Borszewska-Kornacka, M. K.; Rudzinska, I.; Wawrzoniak, T.; Dobrzanska, A.; Czech-Kowalska, J.

    2011-01-01

    Congenital chylous ascites is a rare entity, conditioned by numerous factors and with changing dynamics of the disease. Because of the lack of therapeutic and diagnostic standards, this disease constitutes to be a medical challenge. This article presents current knowledge on pathogenesis, diagnostics and management of this disease, as well as a case of a newborn with primary congenital chylous ascites in the abdominal cavity. (authors)

  7. Multifocal Congenital Hemangiopericytoma.

    Science.gov (United States)

    Robl, Renata; Carvalho, Vânia Oliveira; Abagge, Kerstin Taniguchi; Uber, Marjorie; Lichtvan, Leniza Costa Lima; Werner, Betina; Mehrdad Nadji, Mehrdad

    2017-01-01

    Congenital hemangiopericytoma (HPC) is a rare mesenchymal tumor with less aggressive behavior and a more favorable prognosis than similar tumors in adults. Multifocal presentation is even less common than isolated HPC and hence its clinical and histologic recognition may be challenging. A newborn infant with multifocal congenital HPC causing severe deformity but with a favorable outcome after chemotherapy and surgical removal is reported. © 2016 Wiley Periodicals, Inc.

  8. The role of MR angiography before transjugular placement of a portosystemic stent shunt (TIPS)

    International Nuclear Information System (INIS)

    Mueller, M.F.; Siewert, B.; Kim, D.; Edelman, R.R.; Stokes, K.R.; Finn, J.P.

    1994-01-01

    The authors employed magnetic resonance angiography (MRA) to guide catheter placement for transjugular intrahepatic portosystemic stent shunt (TIPS) procedures in 14 of 24 patients, and compared the results to the 10 patients who did not have prior panning based on MRA. Two-dimensional time-of-flight venography was performed during breath holding, and projection venograms were formatted in sagittal, coronal and axial planes. MRA defined venous anatomy sufficiently well to shorten the procedure and helped to minimize invasiveness. With MRA guidance, intrahepatic needle punctures were significantly fewer (without MRA guidance: mean 12.1; with MRA guidance: mean 3.5, p [de

  9. Intrahepatic Portosystemic Venous Shunt: Successful Embolization Using the Amplatzer Vascular Plug II

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Young Ju; Shin, Byung Seok; Lee, In Ho; Ohm, Joon Young; Lee, Byung Seok; Ahn, Moon Sang [Chungnam National University Hospital, Chungnam National University College of Medicine, Daejeon (Korea, Republic of); Kim, Ho Jun [Dept. of Radiology, Konyang University Hospital, Konyang University College of Medicine, Daejeon (Korea, Republic of)

    2012-11-15

    A 67-year-old woman presented with memory impairment and behavioral changes. Brain MRI indicated hepatic encephalopathy. Abdominal CT scans revealed an intrahepatic portosystemic venous shunt that consisted of two shunt tracts to the aneurysmal sac that communicated directly with the right hepatic vein. The large tract was successfully occluded by embolization using the newly available AMPLATZERTM Vascular Plug II and the small tract was occluded by using coils. The patient's symptoms disappeared after shunt closure and she remained free of recurrence at the 3-month follow-up evaluation.

  10. Pancreaticoportal Fistula and Disseminated Fat Necrosis After Revision of a Transjugular Intrahepatic Portosystemic Shunt

    International Nuclear Information System (INIS)

    Klein, Seth J.; Saad, Nael; Korenblat, Kevin; Darcy, Michael D.

    2013-01-01

    A 59-year old man with alcohol related cirrhosis and portal hypertension was referred for transjugular intrahepatic portosystemic shunt (TIPS) to treat his refractory ascites. Ten years later, two sequential TIPS revisions were performed for shunt stenosis and recurrent ascites. After these revisions, he returned with increased serum pancreatic enzyme levels and disseminated superficial fat necrosis; an iatrogenic pancreaticoportal vein fistula caused by disruption of the pancreatic duct was suspected. The bare area of the TIPS was subsequently lined with a covered stent-graft, and serum enzyme levels returned to baseline. In the interval follow-up period, the patient has clinically improved.

  11. Minimal hepatic encephalopathy characterized by parallel use of the continuous reaction time and portosystemic encephalopathy tests

    DEFF Research Database (Denmark)

    Lauridsen, M M; Schaffalitzky de Muckadell, O B; Vilstrup, H

    2015-01-01

    Minimal hepatic encephalopathy (MHE) is a frequent complication to liver cirrhosis that causes poor quality of life, a great burden to caregivers, and can be treated. For diagnosis and grading the international guidelines recommend the use of psychometric tests of different modalities (computer...... based vs. paper and pencil). To compare results of the Continuous Reaction time (CRT) and the Portosystemic Encephalopathy (PSE) tests in a large unselected cohort of cirrhosis patients without clinically detectable brain impairment and to clinically characterize the patients according to their test...

  12. Cardiac and renal effects of a transjugular intrahepatic portosystemic shunt in cirrhosis

    DEFF Research Database (Denmark)

    Busk, Troels M; Bendtsen, Flemming; Møller, Søren

    2013-01-01

    Refractory ascites and recurrent variceal bleeding are among the serious complications of portal hypertension and cirrhosis for which a transjugular intrahepatic portosystemic shunt (TIPS) can be used. Cirrhotic patients have varying degrees of haemodynamic derangement, mainly characterized...... to improve in patients with the hepatorenal syndrome. The clinical and haemodynamic effects of TIPS have been studied intensively and will be reviewed in the present paper. Considerable knowledge on the effects of TIPS on the pathophysiology of cirrhosis has been gained, but studies on the central...

  13. Hepatic Veins and Inferior Vena Cava Thrombosis in a Child Treated by Transjugular Intrahepatic Portosystemic Shunt

    International Nuclear Information System (INIS)

    Carnevale, Francisco Cesar; Santos, Aline Cristine Barbosa; Tannuri, Uenis; Cerri, Giovanni Guido

    2010-01-01

    We report the case of a 9-year-old boy with portal hypertension, due to Budd-Chiari syndrome, and retrohepatic inferior vena cava thrombosis, submitted to a transjugular intrahepatic portosystemic shunt (TIPS) by connecting the suprahepatic segment of the inferior vena cava directly to the portal vein. After 3 months, the withdrawal of anticoagulants promoted the thrombosis of the TIPS. At TIPS revision, thrombosis of the TIPS and the main portal vein and clots at the splenic and the superior mesenteric veins were found. Successful angiography treatment was performed by thrombolysis and balloon angioplasty of a severe stenosis at the distal edge of the stent.

  14. Pancreaticoportal Fistula and Disseminated Fat Necrosis After Revision of a Transjugular Intrahepatic Portosystemic Shunt

    Energy Technology Data Exchange (ETDEWEB)

    Klein, Seth J., E-mail: kleins@mir.wustl.edu; Saad, Nael [Washington University School of Medicine, Interventional Radiology Section, Mallinckrodt Institute of Radiology (United States); Korenblat, Kevin [Washington University School of Medicine, Division of Gastroenterology, Department of Internal Medicine (United States); Darcy, Michael D. [Washington University School of Medicine, Interventional Radiology Section, Mallinckrodt Institute of Radiology (United States)

    2013-04-15

    A 59-year old man with alcohol related cirrhosis and portal hypertension was referred for transjugular intrahepatic portosystemic shunt (TIPS) to treat his refractory ascites. Ten years later, two sequential TIPS revisions were performed for shunt stenosis and recurrent ascites. After these revisions, he returned with increased serum pancreatic enzyme levels and disseminated superficial fat necrosis; an iatrogenic pancreaticoportal vein fistula caused by disruption of the pancreatic duct was suspected. The bare area of the TIPS was subsequently lined with a covered stent-graft, and serum enzyme levels returned to baseline. In the interval follow-up period, the patient has clinically improved.

  15. Recanalization of an Occluded Intrahepatic Portosystemic Covered Stent via the Percutaneous Transhepatic Approach

    Energy Technology Data Exchange (ETDEWEB)

    Chan, Chih Yang; Liang, Po Chin [National Taiwan University Hospital, Taipei (China)

    2010-08-15

    A 41-year-old woman with liver cirrhosis had recurrent portal hypertension and bleeding from esophageal varices due to complete occlusion of a previously inserted transjugular intrahepatic portosystemic shunt stent. Because recanalization of the stent by the transjugular approach was unsuccessful, ultrasound-guided entry to the splenic vein and portal vein was used. After catheter-directed intrathrombus thrombolysis, successful opening of the stent was achieved and a stent was placed. We herein report a rare case in which thrombolysis and recanalization of a TIPS stent were performed via a percutaneous transhepatic approach

  16. Extrahepatic portal vein occlusion without recurrence after pancreaticoduodenectomy and intraoperative radiation therapy

    International Nuclear Information System (INIS)

    Mitsunaga, Shuichi; Kinoshita, Taira; Kawashima, Mitsuhiko; Konishi, Masaru; Nakagohri, Toshio; Takahashi, Shinichiro; Gotohda, Naoto

    2006-01-01

    Purpose: Although there are no definitive studies that characterize the survival benefit of intraoperative radiation therapy (IORT), the therapy does not seem to produce significant complication. In our institution, pancreaticoduodenectomy (PD) and IORT are often complicated by the development of extrahepatic portal vein occlusion (EHPO). The aim of this study was to characterize the phenomenon of EHPO after PD and IORT. Methods and Materials: Between September 1992 and December 2001, 107 patients received macroscopic curative PD for periampullary disease in our institution. IORT (radiation dose: 20 Gy) was performed in 53 of these patients. Criteria for diagnosis of EHPO were as follows: (1) computerized tomography findings of occlusive extrahepatic portal vein (2) symptoms of portal hypertension, and (3) confirmation to exclude tumor recurrence from origin of EHPO, because this study examined whether EHPO was a complication of PD and IORT. Results: EHPO was diagnosed in 12 patients. Among patient and operative variables, IORT was the only statistically significant factor associated with a diagnosis of EHPO (p = 0.0052). The median developed time to EHPO and overall survival after surgery in EHPO patients were 358 days and 2,562 days, respectively. Eight patients (67%) with EHPO died during the follow-up period. At 5 years after therapy, EHPO was diagnosed in 67% of survivors who had received IORT. Conclusions: Patients undergoing IORT and PD have a relatively high incidence of EHPO, and patients who develop postoperative EHPO have poor prognoses

  17. Chronic Extrahepatic Bile Duct Dilatation: Sonographic Screening in the Patients with Opioid Addiction

    Energy Technology Data Exchange (ETDEWEB)

    Farahmand, H.; PourGholami, M.; Fathollah, Sheikh [Rafsanjan University of Medical Sciences, Rafsanjan (Iran, Islamic Republic of)

    2007-06-15

    One of the best known side effects of using opium is spasm of the sphincter of Oddi, which may increase the diameter of the extrahepatic bile ducts. Ultrasound is the first imaging modality used for evaluating the biliary system because it is commonly available and noninvasive. The principal objective of this study was to measure the common bile duct (CBD) diameter via ultrasonography in opium addicts and to evaluate the relation between the CBD diameter and the period of addiction. This research was an analytical-cross sectional study that was done on 110 opium addicts that were admitted to a drug treatment center. The diameter of the CBD in these cases was measured by ultrasonography and the results were analyzed with other factors like age, the period of addiction and the laboratory findings. According to the findings, there is a significant increase in the range of the CBD diameter in comparison with normal bile ducts. Also, the mean diameter of the CBD in the different age groups showed a significant difference (p < 0.0001) and there was a significant relation between the CBD diameter and the period of addiction (p < 0.001, r = 0.74); so, with the increased length of the addiction period, the mean CBD diameter increases. Opium addiction is one of the factors that causes extrahepatic bile duct dilatation, so in these cases, if no obstructing lesion was found on ultrasound examination and the serum bilirobine and alkaline phosphatase levels are normal, then further evaluation is not needed.

  18. Role of biliary tract cytology in the evaluation of extrahepatic cholestatic jaundice

    Science.gov (United States)

    Gupta, Mamta; Pai, Radha R.; Dileep, Devi; Gopal, Sandeep; Shenoy, Suresh

    2013-01-01

    Background: Endoscopic evaluation is critical in assessing the cause of obstructive jaundice. Cytological techniques including bile aspiration and biliary brushings have become the initial diagnostic modality. Aim: The aim of this study is to evaluate the role of endoscopic biliary tract cytology as a diagnostic tool in the evaluation of extrahepatic cholestatic jaundice. Materials and Methods: A total of 56 biliary tract specimens including 34 bile aspirations and 22 biliary brushings from 41 consecutive patients who had presented with obstructive jaundice and underwent endoscopic retrograde cholangiopancreatography (ERCP) were assessed by cytological examination. The smears prepared were analyzed for standard cytological features. Results: Cytologic diagnosis was adenocarcinoma in 13 (31.7%) cases, atypical in 2 (4.9%), reactive in 3 (7.3%) and benign changes in 19 (46.3%) cases. 4 (9.8%) cases were non-diagnostic. Serum bilirubin was significantly elevated in the malignant group. Biliary stricture was the most common finding on ERCP (68.3%). On cytological examination, presence of solitary, intact atypical cells, enlarged nuclei, irregular nuclear membrane, coarse chromatin and nucleoli were important cytologic criteria for differentiating malignant from benign biliary specimens. Conclusions: Regular use of bile cytology and brushings during ERCP evaluation of extrahepatic cholestatic jaundice is invaluable in obtaining a morphologic diagnosis. A systematic approach, use of strict cytomorphologic criteria and inclusion of significant atypia as malignant diagnosis may improve the sensitivity. PMID:24130407

  19. Influence of distal ileum exclusion on hepatic and renal functions in presence of extrahepatic cholestasis

    Directory of Open Access Journals (Sweden)

    Evandro Luis de Oliveira Costa

    Full Text Available OBJECTIVE: To verify whether the ileal exclusion interferes with liver and kidney functional changes secondary to extrahepatic cholestasis.METHODS: We studied 24 rats, divided into three groups with eight individuals each: Group 1 (control, Group 2 (ligation of the hepatic duct combined with internal biliary drainage, and Group 3 (bile duct ligation combined with internal biliary drainage and exclusion of the terminal ileum. Animals in Group 1 (control underwent sham laparotomy. The animals of groups 2 and 3 underwent ligation and section of the hepatic duct and were kept in cholestasis for four weeks. Next, they underwent an internal biliary bypass. In Group 3, besides the biliary-enteric bypass, we associated the exclusion of the last ten centimeters of the terminal ileum and carried out an ileocolic anastomosis. After four weeks of monitoring, blood was collected from all animals of the three groups for liver and kidney biochemical evaluation (albumin, ALT, AST, direct and indirect bilirubin, alkaline phosphatase, cGT, creatinine and urea.RESULTS: there were increased values of ALT, AST, direct bilirubin, cGT, creatinine and urea in rats from Group 3 (p < 0.05.CONCLUSION: ileal exclusion worsened liver and kidney functions in the murine model of extrahepatic cholestasis, being disadvantageous as therapeutic procedure for cholestatic disorders.

  20. Synchronous double primary cancers of the extrahepatic bile duct: A case report and literature review.

    Science.gov (United States)

    Nishi, Takeshi; Sato, Yoshitoshi; Hanaoka, Takuya; Takahashi, Takuya; Miura, Hiroshi; Takubo, Kenji

    2018-01-01

    Double cancers of the biliary tract system are rare. Most of these cancers are synchronous double cancers of the gall bladder and bile duct, associated with pancreaticobiliary maljunction (PBM). Synchronous double cancers of the extrahepatic bile duct without PBM are especially rare, and only 4 cases have been reported. A 78-year-old woman was admitted to our hospital for examination of hyperbilirubinemia and liver dysfunction. Contrast-enhanced abdominal computed tomography, Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography revealed 2 stenotic regions in the common bile duct: at its junction with the cystic duct and in the distal bile duct. No findings suggested PBM, such as a markedly long common channel. The diagnosis based on endoscopic brush cytology from both stricture portions was adenocarcinoma. The patient had a pylorus-preserving pancreaticoduodenectomy with regional lymph node resection. Macroscopically, there were 2 stenotic regions at the cystic duct junction and in the distal bile duct. Microscopically, the tumor at the junction of the cystic duct was a well-to-moderately differentiated adenocarcinoma. On the other hand, the tumor of the distal bile duct was a poorly differentiated adenocarcinoma. There was no evidence of communication between these 2 cancers. Double cancers of the extrahepatic bile duct without PBM are very rare. Therefore, an accurate diagnosis prior to surgery is necessary. Furthermore, this rare condition seems to be associated with a poor prognosis. Copyright © 2018 The Authors. Published by Elsevier Ltd.. All rights reserved.

  1. Chronic Extrahepatic Bile Duct Dilatation: Sonographic Screening in the Patients with Opioid Addiction

    International Nuclear Information System (INIS)

    Farahmand, H.; PourGholami, M.; Fathollah, Sheikh

    2007-01-01

    One of the best known side effects of using opium is spasm of the sphincter of Oddi, which may increase the diameter of the extrahepatic bile ducts. Ultrasound is the first imaging modality used for evaluating the biliary system because it is commonly available and noninvasive. The principal objective of this study was to measure the common bile duct (CBD) diameter via ultrasonography in opium addicts and to evaluate the relation between the CBD diameter and the period of addiction. This research was an analytical-cross sectional study that was done on 110 opium addicts that were admitted to a drug treatment center. The diameter of the CBD in these cases was measured by ultrasonography and the results were analyzed with other factors like age, the period of addiction and the laboratory findings. According to the findings, there is a significant increase in the range of the CBD diameter in comparison with normal bile ducts. Also, the mean diameter of the CBD in the different age groups showed a significant difference (p < 0.0001) and there was a significant relation between the CBD diameter and the period of addiction (p < 0.001, r = 0.74); so, with the increased length of the addiction period, the mean CBD diameter increases. Opium addiction is one of the factors that causes extrahepatic bile duct dilatation, so in these cases, if no obstructing lesion was found on ultrasound examination and the serum bilirobine and alkaline phosphatase levels are normal, then further evaluation is not needed

  2. Sonographic findings of hepatobiliary fascioliasis accompanied by extrahepatic expansion and ectopic lesions.

    Science.gov (United States)

    Teke, Memik; Önder, Hakan; Çiçek, Mutalip; Hamidi, Cihad; Göya, Cemil; Çetinçakmak, Mehmet Güli; Hattapoğlu, Salih; Ülger, Burak Veli

    2014-12-01

    The aim of the study was to describe the sonographic findings of hepatobiliary fascioliasis with extrahepatic expansion and ectopic lesions. The study included 45 patients with fascioliasis. All diagnoses were confirmed via serologic enzyme-linked immunosorbent assays. Sonographic findings in the hepatobiliary system, extrahepatic expansion, and ectopic lesions were defined. The most common hepatic lesions were subcapsular localized, small, confluent, multiple hypoechoic nodules with poorly defined borders. We also detected ectopic lesion in 5 patients (11.1%) and live parasites in the gallbladder and bile duct in 11 (24.4%). The large spectrum of entities in the differential diagnosis of hepatobiliary fascioliasis may lead to misdiagnosis and incorrect treatment. However, the diagnosis can be made when the characteristic sonographic features are seen, such as heterogeneity of the liver with multiple poorly defined hypoechoic-isoechoic lesions and multiple echogenic nonshadowing particles in the gallbladder or common bile ducts. Nonetheless, the differential diagnosis of fascioliasis versus other hepatic lesions may still be difficult. In these situations, pathologic confirmation should be performed to exclude the possibility of malignancy. © 2013 by the American Institute of Ultrasound in Medicine.

  3. Extrahepatic duct injury in blunt trauma: two case reports and a literature review.

    Science.gov (United States)

    Zago, Thiago Messias; Pereira, Bruno Monteiro Tavares; Calderan, Thiago Rodrigues Araujo; Hirano, Elcio Shiyoiti; Fraga, Gustavo Pereira

    2014-08-01

    Traumatic injuries of the extrahepatic biliary tract are rare. Associated injuries are usually responsible for immediate indication for surgical treatment, the time when an injury to the extrahepatic biliary ducts may be diagnosed. However, missed injuries are often common. The primary aim of this paper is to describe the clinical features, diagnosis, treatment, and outcome of two patients with left hepatic duct injury after blunt abdominal trauma. As a secondary objective, a literature review is presented. The two cases presented in this study are as follows: (1) A young male, involved in a motor vehicle crash, was admitted with blunt hepatic trauma in a general hospital. Endoscopic retrograde cholangiography was conducted 3 weeks later and revealed a large leakage at the left hepatic duct. Exploratory laparotomy was performed 26 days after the initial traumatic event and identified a complete section of the left hepatic duct, treated with anastomosis. (2) A male fell from a height. On exploratory laparotomy, a 30 % partial injury of the left hepatic duct was found in addition to hemoperitoneum, liver injury, gallbladder detachment together with cystic duct rupture, retroperitoneal hematoma to the right, and cecum hematoma. A high level of suspicion is necessary to identify injuries to the hepatic ducts. Early diagnosis that occurs during laparotomy due to associated injuries is important to reduce complications.

  4. Hepatic Encephalopathy Secondary to Intrahepatic Portosystemic Venous Shunt: Balloon-Occluded Retrograde Transvenous Embolization with n-Butyl Cyanoacrylate and Microcoils

    International Nuclear Information System (INIS)

    Yamagami, Takuji; Nakamura, Toshiyuki; Iida, Shigeharu; Kato, Takeharu; Tanaka, Osamu; Matsushima, Shigenori; Ito, Hirotoshi; Okuyama, Chio; Ushijima, Yo; Shiga, Kensuke; Nishimura, Tsunehiko

    2002-01-01

    We report a 70-year-old woman with hepatic encephalopathy due to an intrahepatic portosystemic venous shunt that was successfully occluded by percutaneous transcatheter embolization with n-butyl cyanoacrylate and microcoils

  5. Portal hypertension: a review of portosystemic collateral pathways and endovascular interventions.

    Science.gov (United States)

    Pillai, A K; Andring, B; Patel, A; Trimmer, C; Kalva, S P

    2015-10-01

    The portal vein is formed at the confluence of the splenic and superior mesenteric vein behind the head of the pancreas. Normal blood pressure within the portal system varies between 5 and 10 mmHg. Portal hypertension is defined when the gradient between the portal and systemic venous blood pressure exceeds 5 mmHg. The most common cause of portal hypertension is cirrhosis. In cirrhosis, portal hypertension develops due to extensive fibrosis within the liver parenchyma causing increased vascular resistance. In addition, the inability of the liver to metabolise certain vasodilators leads to hyperdynamic splanchnic circulation resulting in increased portal blood flow. Decompression of the portal pressure is achieved by formation of portosystemic collaterals. In this review, we will discuss the pathophysiology, anatomy, and imaging findings of spontaneous portosystemic collaterals and clinical manifestations of portal hypertension with emphasis on the role of interventional radiology in the management of complications related to portal hypertension. Copyright © 2015 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

  6. Gastric Varices Bleed at Lower Portosystemic Pressure Gradients than Esophageal Varices.

    Science.gov (United States)

    Morrison, Joseph D; Mendoza-Elias, Nasya; Lipnik, Andrew J; Lokken, R Peter; Bui, James T; Ray, Charles E; Gaba, Ron C

    2018-05-01

    To quantify and compare portosystemic pressure gradients (PSGs) between bleeding esophageal varices (EV) and gastric varices (GV). In a single-center, retrospective study, 149 patients with variceal bleeding (90 men, 59 women, mean age 52 y) with EV (n = 69; 46%) or GV (n = 80; 54%) were selected from 320 consecutive patients who underwent successful transjugular intrahepatic portosystemic shunt (TIPS) creation from 1998 to 2016. GV were subcategorized using the Sarin classification as gastroesophageal varices (GEV) (n = 57) or isolated gastric varices (IGV) (n = 23). PSG before TIPS was measured from the main portal vein to the right atrium. PSGs were compared across EV, GEV, and IGV groups using 1-way analysis of variance. Overall mean baseline PSG was 21 mm Hg ± 6. PSG was significantly higher in patients with EV versus GV (23 mm Hg vs 19 mm Hg; P IGV (16 mm Hg); this difference was statistically significant (P IGV 17 mm Hg; P IGV bled versus 9% (5/57) of GEV and 3% (2/69) of EVs (P = .169). Mean final PSG after TIPS was 8 mm Hg (IGV 6 mm Hg vs EV and GEV 8 mm Hg; P = .005). GV bleed at lower PSGs than EV. EV, GEV, and IGV bleeding is associated with successively lower PSGs. These findings highlight distinct physiology, anatomy, and behavior of GV compared with EV. Copyright © 2017 SIR. Published by Elsevier Inc. All rights reserved.

  7. The transjugular intrahepatic portosystemic stentshunt (TIPSS): A new nonoperative, transjugular percutaneous procedure. [Radiological studies

    Energy Technology Data Exchange (ETDEWEB)

    Richter, G M; Noeldge, G; Siegerstetter, V; Franke, M; Wenz, W; Palmaz, J C; Roessle, M

    1989-08-01

    In a 49-year-old male patient suffering from severe symptoms of end-stage portal hypertension and Child's stage C metabolic status, an intrahepatic stent-assisted portosystemic shunt was established for the first time exclusively by means of interventional radiology. Via transjugular access, a modified Brockenbrough needle was used to puncture the right branch of the portal vein via the right liver vein. As a target, a Dormia-basket was used that had previously been exposed in the right main portal branch. After successful puncture and balloon dilation of the artificial tract, two Palmaz stents were implanted to keep the tract permanently open. The portosystemic pressure gradient dropped from 38 to 18 mm Hg. The clinical status of the patient improved substantially during the following days. However, the patient died on day 11 after the procedure because of sudden onset of acute respiratory distress arising from acute nosocomial fungus and cytomegalovirus infection worsened by his primary immunoincompetence. Autopsy demonstrated a totally patent shunt without superficial thrombus. Microscopically, a thin endothelial layer on the inner shunt surface was found to be present. (orig.).

  8. Shear Wave Elastography of the Spleen for Monitoring Transjugular Intrahepatic Portosystemic Shunt Function: A Pilot Study.

    Science.gov (United States)

    Gao, Jing; Zheng, Xiao; Zheng, Yuan-Yi; Zuo, Guo-Qing; Ran, Hai-Tao; Auh, Yong Ho; Waldron, Levi; Chan, Tiffany; Wang, Zhi-Gang

    2016-05-01

    To assess the feasibility of splenic shear wave elastography in monitoring transjugular intrahepatic portosystemic shunt (TIPS) function. We measured splenic shear wave velocity (SWV), main portal vein velocity (PVV), and splenic vein velocity (SVV) in 33 patients 1 day before and 3 days to 12 months after TIPS placement. We also measured PVV, SVV, and SWV in 10 of 33 patients with TIPS dysfunction 1 day before and 3 to 6 days after TIPS revision. Analyses included differences in portosystemic pressure gradient (PPG), PVV, SVV, and mean SWV before and after TIPS procedures; comparison of median SWV before and after TIPS procedures; differences in PVV, SVV, and SWV before and at different times up to 12 months after TIPS placement; accuracy of PVV, SVV, and SWV in determining TIPS dysfunction; and correlation between PPG and SWV. During 12 months of follow-up, 23 of 33 patients had functioning TIPS, and 10 had TIPS dysfunction. The median SWV was significantly different before and after primary TIPS placement (3.60 versus 3.05 m/s; P = .005), as well as before and after revision (3.73 versus 3.06 m/s; P = .003). The PPG, PVV, and SVV were also significantly different before and after TIPS placement and revision (P function and determining TIPS dysfunction. © 2016 by the American Institute of Ultrasound in Medicine.

  9. The association of congenital neuroblastoma and congenital heart disease

    International Nuclear Information System (INIS)

    Bellah, R.; D'Andrea, A.; Children's Hospital, Boston, MA; Darillis, E.; Fellows, K.E.

    1989-01-01

    Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma. (orig.)

  10. Congenital malaria in China.

    Directory of Open Access Journals (Sweden)

    Zhi-Yong Tao

    2014-03-01

    Full Text Available BACKGROUND: Congenital malaria, in which infants are directly infected with malaria parasites from their mother prior to or during birth, is a potentially life-threatening condition that occurs at relatively low rates in malaria-endemic regions. It is recognized as a serious problem in Plasmodium falciparum-endemic sub-Saharan Africa, where recent data suggests that it is more common than previously believed. In such regions where malaria transmission is high, neonates may be protected from disease caused by congenital malaria through the transfer of maternal antibodies against the parasite. However, in low P. vivax-endemic regions, immunity to vivax malaria is low; thus, there is the likelihood that congenital vivax malaria poses a more significant threat to newborn health. Malaria had previously been a major parasitic disease in China, and congenital malaria case reports in Chinese offer valuable information for understanding the risks posed by congenital malaria to neonatal health. As most of the literature documenting congenital malaria cases in China are written in Chinese and therefore are not easily accessible to the global malaria research community, we have undertaken an extensive review of the Chinese literature on this subject. METHODS/PRINCIPAL FINDINGS: Here, we reviewed congenital malaria cases from three major searchable Chinese journal databases, concentrating on data from 1915 through 2011. Following extensive screening, a total of 104 cases of congenital malaria were identified. These cases were distributed mainly in the eastern, central, and southern regions of China, as well as in the low-lying region of southwest China. The dominant species was P. vivax (92.50%, reflecting the malaria parasite species distribution in China. The leading clinical presentation was fever, and other clinical presentations were anaemia, jaundice, paleness, diarrhoea, vomiting, and general weakness. With the exception of two cases, all patients

  11. Extrahepatic bile duct resection in combination with liver resection for hilar cholangiocarcinoma : A report of 42 cases

    NARCIS (Netherlands)

    IJitsma, AJC; Appeltans, BMG; de Jong, KP; Porte, RJ; Peeters, PMJG; Slooff, MJH

    2004-01-01

    From September 1986 until December 2001, 42 patients (20 males and 22 females) underwent a combined extrahepatic bile duct resection (EHBDR) and liver resection (LR) for hilar cholangiocarcinoma (HC). The aim of this study was to analyze patient survival, morbidity, and mortality as well as to seek

  12. Two Cases of Type Va Extrahepatic Bile Duct Duplication With Distal Klatskin Tumor Surgically Treated with Whipple Procedure and Hepaticojejunostomy.

    Science.gov (United States)

    Hammad, Tariq A; Alastal, Yaseen; Khan, Muhammad Ali; Hammad, Mohammad; Alaradi, Osama; Nigam, Ankesh; Sodeman, Thomas C; Nawras, Ali

    2015-10-01

    We describe the diagnostic and therapeutic challenges of a type Va extrahepatic bile duct duplication coexistent with distally located hilar cholangiocarcinoma (Klatskin tumor). We present 2 cases that were diagnosed preoperatively and treated with a modified surgical technique of a combined pylorus-preserving Whipple procedure and hepaticojejunostomy.

  13. Congenital cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Katarina Rednak-Paradiž

    2006-11-01

    Full Text Available Background: CMV is the most common agent that causes congenital virus infection. Only 10 % of infected children have symptomatic infection immediately after birth. Signs of central nervous system damage, neurosensory deafness and delayed psychomotor development may manifest as a result of asimptomatic congenital infection later in childhood. In the article we present basic properties of CMV; we describe clinical picture of the congenital infection and possibilities of diagnose and its treatment. We present five children with symptomatic congenital CMV infection that were hospitalized for the period 1992–2002 at the Neonatal department in the University Children’s Hospital in Ljubljana.Conclusions: Identification of infected neonates, especially those with asimptomatic congenital CMV infection, is difficult. Latest incidence of infection in Slovenia is unknown. With new investigations the efficiency of antiviral therapy was discovered but exact indications for therapy are not yet known. CMV vaccine, once available, may ultimately be the best control strategy for this important public health problem. Proper educating women in childbearing age about the risks of CMV and how to avoid disease transmission during pregnancy (hand washing, avoiding mouth-to-mouth contact with preschool children, usage of gloves especially when handling dipers or respiratory secretions are the only control strategies available.

  14. Prevalence of congenital amusia.

    Science.gov (United States)

    Peretz, Isabelle; Vuvan, Dominique T

    2017-05-01

    Congenital amusia (commonly known as tone deafness) is a lifelong musical disorder that affects 4% of the population according to a single estimate based on a single test from 1980. Here we present the first large-based measure of prevalence with a sample of 20 000 participants, which does not rely on self-referral. On the basis of three objective tests and a questionnaire, we show that (a) the prevalence of congenital amusia is only 1.5%, with slightly more females than males, unlike other developmental disorders where males often predominate; (b) self-disclosure is a reliable index of congenital amusia, which suggests that congenital amusia is hereditary, with 46% first-degree relatives similarly affected; (c) the deficit is not attenuated by musical training and (d) it emerges in relative isolation from other cognitive disorder, except for spatial orientation problems. Hence, we suggest that congenital amusia is likely to result from genetic variations that affect musical abilities specifically.

  15. Nitrofurantoin and congenital abnormalities

    DEFF Research Database (Denmark)

    Czeizel, A.E.; Rockenbauer, M.; Sørensen, Henrik Toft

    2001-01-01

    or fetuses with Down’s syndrome (patient controls), 23 (2.8%) pregnant women were treated with nitrofurantoin. The above differences between population controls and cases may be connected with recall bias, because the case-control pair analysis did not indicate a teratogenic potential of nitrofurantoin use......Objective: To study human teratogenic potential of oral nitrofurantoin treatment during pregnancy. Materials and Methods: Pair analysis of cases with congenital abnormalities and matched population controls in the population-based dataset of the Hungarian Case-Control Surveillance of Congenital...... during the second and the third months of gestation, i.e. in the critical period for major congenital abnormalities. Conclusion: Treatment with nitrofurantoin during pregnancy does not present detectable teratogenic risk to the fetus....

  16. Congenital syphilis: literature review

    Directory of Open Access Journals (Sweden)

    Eduardo Chaida Sonda

    2013-01-01

    Full Text Available Syphilis is an infectious disease caused by Treponema pallidum and has high rates of vertical transmission, which can reach 100% depending on the maternal disease and stage of pregnancy. The diagnosis of gestational syphilis is simple and its screening is required during the prenatal period. However, this disease still has a high prevalence, affecting two million pregnant women worldwide. The procedures performed in newborns with congenital syphilis represent costs that are three-fold higher than the ones spent with a baby without this infection. The treatment is generally carried out with penicillin and must be extended to sexual partners. Inadequate or lack of treatment of congenital syphilis can result in miscarriage, premature birth, acute complications and other fetal sequelae. KEYWORDS: Congenital syphilis. Treponema pallidum. Vertical transmission.

  17. Congenital cataract screening

    Directory of Open Access Journals (Sweden)

    Zhale Rajavi

    2016-01-01

    Full Text Available Congenital cataract is a leading cause of visual deprivation which can damage the developing visual system of a child; therefore early diagnosis, management and long-term follow-up are essential. It is recommended that all neonates be screened by red reflex examination at birth and suspected cases be referred to ophthalmic centers. Early surgery (1 year is highly recommended. After surgery, amblyopia treatment and periodic follow-up examinations should be started as soon as possible to achieve a satisfactory visual outcome. Practitioners should consider the possibility of posterior capsular opacity, elevated intraocular pressure and amblyopia during follow-up, especially in eyes with microphthalmia and/or associated congenital anomalies. All strabismic children should undergo slit lamp examination prior to strabismus surgery to rule out congenital lens opacities. From a social point of view, equal and fair medical care should be provided to all children regardless of gender.

  18. Extrahepatic Arteries Originating from Hepatic Arteries: Analysis Using CT During Hepatic Arteriography and Visualization on Digital Subtraction Angiography

    International Nuclear Information System (INIS)

    Ozaki, Kumi; Kobayashi, Satoshi; Matsui, Osamu; Minami, Tetsuya; Koda, Wataru; Gabata, Toshifumi

    2017-01-01

    PurposeTo investigate the prevalence and site of origin of extrahepatic arteries originating from hepatic arteries on early phase CT during hepatic arteriography (CTHA) was accessed. Visualization of these elements on digital subtraction hepatic angiography (DSHA) was assessed using CTHA images as a gold standard.Materials and MethodsA total of 943 patients (mean age 66.9 ± 10.3 years; male/female, 619/324) underwent CTHA and DSHA. The prevalence and site of origin of extrahepatic arteries were accessed using CTHA and visualized using DSHA.ResultsIn 924 (98.0%) patients, a total of 1555 extrahepatic branches, representing eight types, were found to originate from hepatic arteries on CTHA. CTHA indicated the following extrahepatic branch prevalence rates: right gastric artery, 890 (94.4%); falciform artery, 386 (40.9%); accessory left gastric artery, 161 (17.1%); left inferior phrenic artery (IPA), 43 (4.6%); posterior superior pancreaticoduodenal artery, 33 (3.5%); dorsal pancreatic artery, 26 (2.8%); duodenal artery, 12 (1.3%); and right IPA, 4 (0.4%). In addition, 383 patients (40.6%) had at least one undetectable branch on DSHA. The sensitivity, specificity, and accuracy of visualization on DSHA were as follows: RGA, 80.0, 86.8, and 80.4%; falciform artery, 53.9, 97.7, and 80.0%; accessory LGA, 64.6, 98.6, and 92.3%; left IPA, 76.7, 99.8, and 98.7%; PSPDA, 100, 99.7, and 99.9%; dorsal pancreatic artery, 57.7, 100, and 98.8%; duodenal artery, 8.3, 99.9, and 98.7%; and right IPA, 0, 100, and 99.6%, respectively.ConclusionExtrahepatic arteries originating from hepatic arteries were frequently identified on CTHA images. These arteries were frequently overlooked on DSHA.

  19. Extrahepatic Arteries Originating from Hepatic Arteries: Analysis Using CT During Hepatic Arteriography and Visualization on Digital Subtraction Angiography

    Energy Technology Data Exchange (ETDEWEB)

    Ozaki, Kumi, E-mail: ozakik-rad@umin.org [Kanazawa University Graduate School of Medical Science, Department of Radiology (Japan); Kobayashi, Satoshi [Kanazawa University Graduate School of Medical Science, Department of Quantum Medicine Technology (Japan); Matsui, Osamu; Minami, Tetsuya; Koda, Wataru; Gabata, Toshifumi [Kanazawa University Graduate School of Medical Science, Department of Radiology (Japan)

    2017-06-15

    PurposeTo investigate the prevalence and site of origin of extrahepatic arteries originating from hepatic arteries on early phase CT during hepatic arteriography (CTHA) was accessed. Visualization of these elements on digital subtraction hepatic angiography (DSHA) was assessed using CTHA images as a gold standard.Materials and MethodsA total of 943 patients (mean age 66.9 ± 10.3 years; male/female, 619/324) underwent CTHA and DSHA. The prevalence and site of origin of extrahepatic arteries were accessed using CTHA and visualized using DSHA.ResultsIn 924 (98.0%) patients, a total of 1555 extrahepatic branches, representing eight types, were found to originate from hepatic arteries on CTHA. CTHA indicated the following extrahepatic branch prevalence rates: right gastric artery, 890 (94.4%); falciform artery, 386 (40.9%); accessory left gastric artery, 161 (17.1%); left inferior phrenic artery (IPA), 43 (4.6%); posterior superior pancreaticoduodenal artery, 33 (3.5%); dorsal pancreatic artery, 26 (2.8%); duodenal artery, 12 (1.3%); and right IPA, 4 (0.4%). In addition, 383 patients (40.6%) had at least one undetectable branch on DSHA. The sensitivity, specificity, and accuracy of visualization on DSHA were as follows: RGA, 80.0, 86.8, and 80.4%; falciform artery, 53.9, 97.7, and 80.0%; accessory LGA, 64.6, 98.6, and 92.3%; left IPA, 76.7, 99.8, and 98.7%; PSPDA, 100, 99.7, and 99.9%; dorsal pancreatic artery, 57.7, 100, and 98.8%; duodenal artery, 8.3, 99.9, and 98.7%; and right IPA, 0, 100, and 99.6%, respectively.ConclusionExtrahepatic arteries originating from hepatic arteries were frequently identified on CTHA images. These arteries were frequently overlooked on DSHA.

  20. Congenital maxillary double lip

    Directory of Open Access Journals (Sweden)

    Dinesh Singh Chauhan

    2012-01-01

    Full Text Available Double lip, also referred to as "macrocheilia," is a rare anomaly which affects the upper lip more commonly than the lower lip. It consists of a fold of excess or redundant hypertrophic tissue on the mucosal side of the lip. The congenital double lip is believed to be present at birth and becomes more prominent after eruption of teeth. It affects esthetics and also interferes with speech and mastication. Simple surgical excision produces good functional and cosmetic results. We report a case of a non-syndromic congenital maxillary double lip in a 21-year-old male patient.

  1. Congenital hearing impairment

    Energy Technology Data Exchange (ETDEWEB)

    Robson, Caroline D. [Children' s Hospital and Harvard Medical School, Division of Neuroradiology, Department of Radiology, Boston, MA (United States)

    2006-04-15

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  2. Congenital spinal malformations

    International Nuclear Information System (INIS)

    Ertl-Wagner, B.B.; Reiser, M.F.

    2001-01-01

    Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [de

  3. Congenital hearing impairment

    International Nuclear Information System (INIS)

    Robson, Caroline D.

    2006-01-01

    Establishing the etiology of congenital hearing impairment can significantly improve treatment for certain causes of hearing loss and facilitates genetic counseling. High-resolution CT and MRI have contributed to the evaluation and management of hearing impairment. In addition, with the identification of innumerable genetic loci and genetic defects involved in hearing loss, genetic testing has emerged as an invaluable tool in the assessment of hearing impairment. Some of the common forms of congenital hearing loss are reviewed and their imaging features illustrated. (orig.)

  4. Congenital laryngeal anomalies,

    Directory of Open Access Journals (Sweden)

    Michael J. Rutter

    2014-12-01

    Full Text Available Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants.

  5. Congenital Heart Defects and CCHD

    Science.gov (United States)

    ... and more. Stony Point, NY 10980 Close X Home > Complications & Loss > Birth defects & other health conditions > Congenital heart defects and ... in congenital heart defects. You have a family history of congenital heart ... syndrome or VCF. After birth Your baby may be tested for CCHD as ...

  6. C-arm CT for planning and guidance of extrahepatic embolizations

    International Nuclear Information System (INIS)

    Wacker, F.K.; Meissner, O.A.; Meyer, B.C.

    2009-01-01

    Interventional radiological vascular embolizations are complex procedures that require exact imaging of the target region to facilitate safe and effective treatment. The purpose of this paper is to present the technique and feasibility of flat detector C-arm computed tomography (C-arm CT) for control and guidance of extrahepatic abdominal embolization procedures. C-arm CT images can provide important information on both vascular and cross-sectional anatomy of the target region, help in determining therapy endpoints and provide follow-up during and immediately after the abdominal interventions.The cases presented demonstrate that C-arm CT images are beneficial for abdominal embolization procedures and facilitate precise treatment. (orig.) [de

  7. Primary extrahepatic hydatid cyst of the soft tissue: a case report

    Directory of Open Access Journals (Sweden)

    Guraya Salman

    2012-11-01

    Full Text Available Abstract Introduction Hydatid disease of the soft tissue is an exceedingly uncommon site to be affected by the tapeworm Echinococcus. The presentation is often vague and misleading. The diagnostic armamentarium has to be supplemented by a meticulously taken history and clinical examination. Case presentation The present case report describes a 33-year-old Saudi male with a painless swelling in the right buttock which turned out to be a primary hydatid disease of the soft tissue. The lump was successfully excised surgically and the patient had an uneventful discharge. Conclusion Surgical excision of the extrahepatic hydatid disease remains the mainstay of treatment; although medical treatment is available for the recurrent and disseminated disease.

  8. Extrahepatic bile duct ligation in broiler chickens: ultrastructural study of Ito cell

    Directory of Open Access Journals (Sweden)

    Ekowati Handharyani

    2004-12-01

    Full Text Available The Ito cell (fat-storing cell is a cell lying in perisinusoidal space of liver. The function of Ito cell is expanding from a site of fat-storing site to a center of extracellular matrix metabolism and mediator production in the liver. This study was performed in order to evaluate the Ito cells in cholestatic condition. The artificial cholestatic was conducted by ligation of extrahepatic bile ducts (bile duct ligation = BDL in broilers. The results showed that BDL induced bile congestion, fibrosis, proliferation of Ito cells and intrahepatic bile ductules. Immunohistochemistry demonstrated that Ito cells were scattered throughout the fibrotic areas, and larger in size with more extensive immunoreactivity than those in normal livers. Ultrastructural study demonstrated that Ito cells were closely associated with the production of extracellular collagen fibers. Ito cells actively react against hepatocytic injuries, especially in fibrogenesis of cholestatic livers.

  9. A case of distal extrahepatic cholangiocarcinoma with two positive resection margins.

    Science.gov (United States)

    Warner, Wayne A; Ramcharan, Wesley; Harnanan, Dave; Umakanthan, Srikanth; Maharaj, Ravi

    2016-11-01

    Cholangiocarcinoma is an uncommon primary malignancy of the biliary tract that is challenging to diagnose and treat effectively due to its relatively silent and late clinical presentation. The present study reports a case of a 60-year-old male with distal extrahepatic cholangiocarcinoma with a 3-week history of painless obstructive jaundice symptoms and subjective weight loss. Imaging revealed an obstructing lesion in the common bile duct, just distal to the entrance of the cystic duct. Pathology revealed moderately differentiated cholangiocarcinoma with two positive proximal resection margins. The two positive resection margins presented a challenge during surgery and points to an urgent need for further studies to better illuminate diagnostic and therapeutic options for patients with similar clinicopathological presentation.

  10. Extrahepatic bile duct carcinoma treated by intraluminal irradiation with iridium-192 wire. Report of a case

    Energy Technology Data Exchange (ETDEWEB)

    Ikeda, H; Kuroda, T; Uchida, H [Osaka Univ. (Japan). Faculty of Medicine

    1980-08-01

    A 57-year-old male with obstructive jaundice was diagnosed extrahepatic bile duct carcinoma at bifurcation by percutaneous transhepatic cholangiography (PTC). He was treated 3,300 rad of external irradiation and then intraluminal irradiation using the Iridium-192 wire by two times with the aid of PTC internal drainage, each was given by the dose of 1,600 rad at 5 mm inside the tumor from the PTC-tube. He had been well for about 1 year and then died because of ascites and cachexia. Autopsy revealed only microscopic tumor cells remaining around the common duct below the cystic junction. It was confirmed that intraluminal irradiation using the Iridium-192 wire was potentially curable and easily applicable to the bile duct carcinoma.

  11. Extrahepatic bile duct carcinoma treated by intraluminal irradiation with iridium-192 wire

    International Nuclear Information System (INIS)

    Ikeda, Hiro; Kuroda, Tomosumi; Uchida, Hideo

    1980-01-01

    A 57-year-old male with obstructive jaundice was diagnosed extrahepatic bile duct carcinoma at bifurcation by percutaneous transhepatic cholangiography (PTC). He was treated 3,300 rad of external irradiation and then intraluminal irradiation using the Iridium-192 wire by two times with the aid of PTC internal drainage, each was given by the dose of 1,600 rad at 5 mm inside the tumor from the PTC-tube. He had been well for about 1 year and then died because of ascites and cachexia. Autopsy revealed only microscopic tumor cells remaining around the common duct below the cystic junction. It was confirmed that intraluminal irradiation using the Iridium-192 wire was potentially curable and easily applicable to the bile duct carcinoma. (author)

  12. Extrahepatic biliary cystadenoma with mesenchymal stroma: a true biliary cystadenoma? A case report.

    LENUS (Irish Health Repository)

    Hennessey, Derek B

    2012-02-01

    Biliary cystadenomas are benign but potentially malignant cystic neoplasms, which classically contain mesenchymal stroma similar to ovarian tissue. We report a case of an extra-hepatic biliary cystadenoma with mesenchymal stroma along with a discussion of current pathological opinion. CASE PRESENTATION: A 54-year-old female presented with abdominal pain, abnormal liver function tests and a mass on ultrasound. Computerized Tomography identified a complex multi-locular cyst in the common hepatic duct. Radical excision of the lesion and a Roux-en-Y loop bilio-enteric anastomosis was performed. Histology confirmed the presence of a benign biliary cystadenoma with ovarian type stroma. CONCLUSION: Biliary cystadenomas classically contain mesenchymal stroma similar to ovarian tissue. It now appears that cystadenomas without mesenchymal stroma appear to be more akin to similar cystic lesions of the pancreas, and may represent a dissimilar neoplasm. Therefore, malignant transformation can occur, so complete excision is recommended.

  13. Radiation therapy for patients with obstructive jaundice caused by carcinoma of the extrahepatic biliary system

    International Nuclear Information System (INIS)

    Kawamura, Masashi; Nakagawa, Hirofumi; Kataoka, Masaaki

    1992-01-01

    From February 1980 through September 1990, 92 patients with obstructive jaundice resulting from biliary tract cancer were registered at Shikoku Cancer Center Hospital or Ehime University Hospital. Radiation therapy (RT) was used to treat 38 of these patients (30 with carcinoma of the extrahepatic bile duct, excluding ampulla of Vater, and eight patients with carcinoma of the gallbladder). Of 38 patients, 11 underwent intraoperative radiation therapy (IORT), and 27 were treated by external radiation therapy (ERT) alone. In contrast, 54 patients (39 with carcinoma of the extrahepatic bile duct and eight with carcinoma of the gallbladder) were not treated by RT. All jaundiced patients received external and/or internal biliary drainage of some kind. Among patients undergoing biliary drainage with a catheter, 21 patients who underwent RT (four with IORT) survived significantly longer than 19 patients who did not (generalized Wilcoxon test: p<0.05). There were no significant differences in survival between 7 patients with recanalization and 11 patients with no recanalization. Concerning the survival of laparotomized patients, excluding those with complete resection or perioperative death, eight patients treated with postoperative ERT survived longer than 12 patients who did not have postoperative ERT (not significant). Eleven patients underwent IORT. A patient with unresectable carcinoma of the hilar bile duct survived 2 years and 3 months after a combination treatment of ERT and IOTR. In four of eight autopsied patients, radiation effects of Grade II were observed (Oboshi and Shimosato's evaluation system for the histological effects of radiation therapy). Our experience suggests that RT is effective in patients with obstructive jaundice caused by carcinoma of the biliary system. (author)

  14. Simultaneous Multitarget Irradiation Using Helical Tomotherapy for Advanced Hepatocellular Carcinoma With Multiple Extrahepatic Metastases

    International Nuclear Information System (INIS)

    Jang, Jeong Won; Kay, Chul Seung; You, Chan Ran; Kim, Chang Wook; Bae, Si Hyun.; Choi, Jong Young; Yoon, Seung Kew; Han, Chi Wha; Jung, Hyun Suk; Choi, Ihl Bong

    2009-01-01

    Purpose: The prognosis of hepatocellular carcinoma (HCC) patients with extrahepatic metastases is extremely poor. Helical tomotherapy, an image-guided, intensity-modulated radiotherapy system, can allow for simultaneous and precise targeting of multiple cancerous lesions, while sparing normal tissues. This study evaluated the feasibility and outcome of tomotherapy for advanced HCC with metastases. Patients and Methods: A total of 42 consecutive HCC patients with metastases were treated with tomotherapy using the Hi-Art system. A total of 152 intra- and extrahepatic lesions (3.5 lesions/patient) were treated simultaneously, with a dose of 51.03 Gy (range, 30-57.61) in 10 fractions. Transarterial chemolipiodolization using epirubicin (50 mg) and cisplatin (60 mg) was repeated in patients with intrahepatic HCC (mean size, 9.0 cm) after tomotherapy. Results: An objective response (complete response and partial response) was achieved in 45.2% of patients with intrahepatic tumors, 68.4% of patients with pulmonary lesions, 60.0% of patients with lymph node/adrenal lesions, and 66.7% of patients with soft-tissue metastases. The complete response rate for those with pulmonary and lymph node/adrenal metastases was 26.3% and 5.0%, respectively. The overall survival rate at 1 and 2 years was 50.1% and 14.9%, respectively, with a median survival of 12.3 months. The actuarial in-field tumor control rate for ≤1 year was 79.0%. No cases of Grade 4-5 acute toxicity occurred. Conclusion: The results of this study have shown that helical tomotherapy is safe and feasible without major toxicities for the treatment of advanced HCC and results in excellent tumor control and a potential survival benefit. This approach is expected to be a useful palliative option for selected HCC patients with metastases.

  15. NAFLD and NASH in HCV Infection: Prevalence and Significance in Hepatic and Extrahepatic Manifestations

    Directory of Open Access Journals (Sweden)

    Luigi Elio Adinolfi

    2016-05-01

    Full Text Available The aim of this paper is to review and up to date the prevalence of hepatitis C virus (HCV-associated non-alcoholic fatty liver disease (NAFLD and non-alcoholic steatohepatitis (NASH and their significance in both accelerating progression of HCV-related liver disease and development of HCV-associated extrahepatic diseases. The reported mean prevalence of HCV-related NAFLD was 55%, whereas NASH was reported in 4%–10% of cases. HCV genotype 3 directly induces fatty liver deposition, namely “viral steatosis” and it is associated with the highest prevalence and degree of severity, whereas, HCV non-3 genotype infection showed lower prevalence of steatosis, which is associated with metabolic factors and insulin resistance. The host’s genetic background predisposes him or her to the development of steatosis. HCV’s impairment of lipid and glucose metabolism causes fatty liver accumulation; this seems to be a viral strategy to optimize its life cycle. Irrespective of insulin resistance, HCV-associated NAFLD, in a degree-dependent manner, contributes towards accelerating the liver fibrosis progression and development of hepatocellular carcinoma by inducing liver inflammation and oxidative stress. Furthermore, NAFLD is associated with the presence of metabolic syndrome, type 2 diabetes, and atherosclerosis. In addition, HCV-related “metabolic steatosis” impairs the response rate to interferon-based treatment, whereas it seems that “viral steatosis” may harm the response rate to new oral direct antiviral agents. In conclusion, a high prevalence of NAFLD occurs in HCV infections, which is, at least in part, induced by the virus, and that NAFLD significantly impacts progression of the liver disease, therapeutic response, and some extrahepatic diseases.

  16. Annexin A2 is a natural extrahepatic inhibitor of the PCSK9-induced LDL receptor degradation.

    Directory of Open Access Journals (Sweden)

    Nabil G Seidah

    Full Text Available Proprotein convertase subtilisin/kexin-9 (PCSK9 enhances the degradation of hepatic low-density lipoprotein receptor (LDLR. Deletion of PCSK9, and loss-of-function mutants in humans result in lower levels of circulating LDL-cholesterol and a strong protection against coronary heart disease. Accordingly, the quest for PCSK9 inhibitors has major clinical implications. We have previously identified annexin A2 (AnxA2 as an endogenous binding partner and functional inhibitor of PCSK9. Herein, we studied the relevance of AnxA2 in PCSK9 inhibition and lipid metabolism in vivo. Plasma analyses of AnxA2(-/- mice revealed: i a ∼1.4-fold increase in LDL-cholesterol without significant changes in VLDLs or HDLs, and ii a ∼2-fold increase in circulating PCSK9 levels. Western blotting and immunohistochemistry of AnxA2(-/- tissues revealed that the LDLR was decreased by ∼50% in extrahepatic tissues, such as adrenals and colon. We also show that AnxA2-derived synthetic peptides block the PCSK9≡LDLR interaction in vitro, and adenoviral overexpression of AnxA2 in mouse liver increases LDLR protein levels in vivo. These results suggest that AnxA2 acts as an endogenous regulator of LDLR degradation, mostly in extrahepatic tissues. Finally, we identified an AnxA2 coding polymorphism, V98L, that correlates with lower circulating levels of PCSK9 thereby extending our results on the physiological role of AnxA2 in humans.

  17. Severe congenital neutropenia

    DEFF Research Database (Denmark)

    Borregaard, Niels

    2014-01-01

    In this issue of Blood, Tidwell et al1 demonstrate that mutations in the start codon (protein synthesis is initiated at the codon ATG) of neutrophil elastase (ELANE) result in the production of N-terminally truncated elastase, which mislocates to the nucleus and results in severe congenital neutr...... neutropenia (SCN)....

  18. Giant Congenital Melanocytic Nevus

    DEFF Research Database (Denmark)

    Rasmussen, Bo Sonnich; Henriksen, Trine Foged; Kølle, Stig-Frederik Trojahn

    2015-01-01

    Giant congenital melanocytic nevi (GCMN) occur in 1:20,000 livebirths and are associated with increased risk of malignant transformation. The treatment of GCMN from 1981 to 2010 in a tertiary referral center was reviewed evaluating the modalities used, cosmetic results, associated complications...

  19. Congenital neck masses.

    Science.gov (United States)

    Rosa, Peter A; Hirsch, David L; Dierks, Eric J

    2008-08-01

    Congenital neck lesions reflect abnormal embryogenesis in head and neck development. A thorough knowledge of embryology and anatomy is critical in the diagnosis and treatment of these lesions. The appropriate diagnosis of these lesions is necessary to provide appropriate treatment and long-term follow up, because some of these lesions may undergo malignant transformation or be harbingers of malignant disease.

  20. Congenital cystic eyeball

    Directory of Open Access Journals (Sweden)

    Gupta V

    1990-01-01

    Full Text Available A rare case of histopathologically proved case of congenital cystic eye in a one day old girl is described. It was an unusually large cystic mass bulging forwards stretching the upperlid. There was no rudimentary eyeball in the orbit. The cystic eye′s predilection for the left eye has been pointed out for the first time in this article.

  1. Congenital extracranial meningioma

    International Nuclear Information System (INIS)

    Wong, H.F.; Ng, S.H.; Wai, Y.Y.; Wan, Y.L.; Kong, M.S.

    1995-01-01

    The authors report a case of congenital meningioma in a newborn. This tumour is extremely rare and only six cases have been reported in the literature. Those reported cases were mainly intracranial. This is the first case of a neonatal extracranial meningioma that was evaluated preoperatively by computed tomography and magnetic resonance imaging. (orig.)

  2. Congenital cystic adenomatoid malformation

    International Nuclear Information System (INIS)

    Chaudhry, A.K.; Azam, M.; Maqsood, R.; Naz, B.; Salam, A.

    2003-01-01

    This case report presents the clinical picture, diagnostic methodology and surgical treatment of a female child who presented with chronic cough and dyspnoea due to congenital malformation of lung. A discussion of diagnosis and management is presented at the end. (author)

  3. Pseudoamblyopia in Congenital Cyclotropia

    Directory of Open Access Journals (Sweden)

    Antonio Frattolillo

    2017-01-01

    Full Text Available Purpose. To study the effect of surgery on amblyopia and suppression associated with congenital cyclovertical strabismus. Methods. The fixation pattern was investigated with microperimetry before and soon after surgery in ten consecutive children operated for congenital superior oblique palsy at the S. Martino Hospital, Belluno, Italy, between September 2014 and December 2015. Changes in visual performance in terms of best-corrected visual acuity (BCVA and stereopsis between the day before and one week after surgery were also evaluated. No other amblyopia treatment has been administered during the time study. Results. Surgical correction of the excyclodeviation in congenital SO palsy determined monocular and binocular sensory consequences: monocularly, in the cyclodeviated amblyopic eye, BCVA (0.46–0.03 LogMAR; p<0.0001 and the fixation pattern improved, as demonstrated by microperimetry examination. Binocularly, stereopsis improved or emerged while suppression at the Worth four-dot test disappeared. Conclusions. In the absence of further amblyopic factors such as coexisting constant vertical and/or horizontal deviation and anisometropia, the amblyopia encountered in congenital SO palsy may resolve soon after the surgical alignment. Therefore, it may be considered and defined “pseudoamblyopia.”

  4. Identification of congenital deafblindness

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2012-01-01

    . The study evaluated the assessment procedure of 190 children and adults found to be congenitally deafblind. Among the 190 individuals 76 percent were determined using functional assessment in addition to medical examination. A case example involving a 12-year-old child is also presented to illustrate...

  5. Abnormal Gas Diffusing Capacity and Portosystemic Shunt in Patients With Chronic Liver Disease

    Science.gov (United States)

    Park, Moon-Seung; Lee, Min-Ho; Park, Yoo-Sin; Kim, Shin-Hee; Kwak, Min-Jung; Kang, Ju-Seop

    2012-01-01

    Background Pulmonary dysfunctions including the hepatopulmonary syndrome and portosystemic shunt are important complications of hepatic cirrhosis. To investigate the severity and nature of abnormal gas diffusing capacity and its correlation to portosystemic shunt in patients with chronic liver disease. Methods Forty-four patients with chronic liver disease (15 chronic active hepatitis (CAH), 16 Child-Pugh class A, and 13 Child-Pugh class B) without other diseases history were enrolled in the study. Evaluation of liver function tests, arterial blood gases analysis, ultrasonography, pulmonary function test including lung diffusing capacity of carbon monoxide (DLco), forced vital capacity(FVC), forced expiratory volume 1 seconds(FEV1), total lung capacity(TLC), DLco/AV(alveolar volume) and thallium-201 per rectum scintigraphy were performed. We were analyzed correlations between pulmonary function abnormalities and heart/liver (H/L) ratio in patients with chronic liver diseases. Results In CAH, percentage of patients with DLco and DLco/VA (Child-Pugh class A and B patients. The means of DLco and DLco/VA were significantly (P Child-Pugh class. The mean H/L ratio in Child-Pugh class B increased markedly (P Child-Pugh class A. The frequency of specific pulmonary function abnormality in patients with Child-Pugh class B was significantly (P Child-Pugh class A and CAH. There was a inverse linear correlation between H/L ratio and DLco (r = -0.339, P < 0.05) and DLco/VA (r = -0.480, P < 0.01). Conclusion A total of 62% of patients with advanced liver disease have abnormal pulmonary diffusion capacity with a reduced DLco or DLco/VA and abnormal portosystemic shunt (increased H/L ratio) is common hemodynamic abnormality. Therefore, inverse linear correlation between DLco or DLco/VA and H/L ratio may be an important factor in predicting pulmonary complication and meaningful diagnostic and prognostic parameters in patients with advanced chronic liver disease. PMID:27785203

  6. Characterization of the coagulation profile in children with liver disease and extrahepatic portal vein obstruction or shunt.

    Science.gov (United States)

    Beattie, William; Magnusson, Maria; Hardikar, Winita; Monagle, Paul; Ignjatovic, Vera

    2017-03-01

    Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children. Children with severe liver disease had coagulopathic changes, including either decreased concentration or activity of factor II, factor V, and factor VII. Nineteen percent (8/42) of the cohort had significant bleeding. Thrombophilic changes were also observed, including decreased concentration or activity of protein C, protein S, and antithrombin and increased concentration and activity of factor VIII and Von Willebrand factor. Similar coagulation factor changes were observed in children with extrahepatic portal vein obstruction or shunt. There was a trend toward greater changes in coagulation factor activity compared to concentration. This study provides a detailed description of the changes in both the concentration and activity of coagulation factors in pediatric liver disease and extrahepatic portal vein obstruction or shunt. Interestingly, procoagulant and anticoagulant factors were deranged in portal vein obstruction or shunt to a similar degree as in liver disease. An improved understanding of the coagulation profile in the pediatric setting will contribute to the improved management of liver disease and extrahepatic portal obstruction or shunt. PELD: pediatric end-stage liver disease score; MELD: model for end-stage liver disease score; ELISA: enzyme-linked immunosorbent assay; MCRI: Murdoch Childrens

  7. Portal hypertension: Imaging of portosystemic collateral pathways and associated image-guided therapy.

    Science.gov (United States)

    Bandali, Murad Feroz; Mirakhur, Anirudh; Lee, Edward Wolfgang; Ferris, Mollie Clarke; Sadler, David James; Gray, Robin Ritchie; Wong, Jason Kam

    2017-03-14

    Portal hypertension is a common clinical syndrome, defined by a pathologic increase in the portal venous pressure. Increased resistance to portal blood flow, the primary factor in the pathophysiology of portal hypertension, is in part due to morphological changes occurring in chronic liver diseases. This results in rerouting of blood flow away from the liver through collateral pathways to low-pressure systemic veins. Through a variety of computed tomographic, sonographic, magnetic resonance imaging and angiographic examples, this article discusses the appearances and prevalence of both common and less common portosystemic collateral channels in the thorax and abdomen. A brief overview of established interventional radiologic techniques for treatment of portal hypertension will also be provided. Awareness of the various imaging manifestations of portal hypertension can be helpful for assessing overall prognosis and planning proper management.

  8. The transjugular portosystemic stent shunt (TIPSS) as an intervention in clinical complication of portal hypertension

    International Nuclear Information System (INIS)

    Thalhammer, A.; Jacobi, V.; Schwarz, W.; Balzer, J.; Abolmaali, N.; Vogl, T.J.

    2001-01-01

    Most frequent complications in patients with liver cirrhosis are due to portal hypertension. Beside ascites circumvent vessles formate with vasodilatation. Due to counterregulation a secondary hyperaldosteronism develops with release of vasocontrictive agents. If conservative and endoscopic methods fail, indication for building a portosystemic shunt is given. The TIPSS procedure is less invasive than the surgical method of Warren-Shunt, so the radiological intervention has replaced surgery. Reducing the portal pressure by the shunt, the clinical complications change for the better. Still problems are defined as hepatic encephalopathy and right ventricular heart failure. Regular follow up investigations have to be performed to detect complications in the shunt. Using regular clinical and radiological check up TIPSS is of clinical benefit with good long term results. (orig.) [de

  9. Usefulness of computed tomography (CT) in the diagnosis of portosystemic collaterals in liver cirrhosis

    International Nuclear Information System (INIS)

    Tsukune, Yoshihiko

    1984-01-01

    This study assesses the usefulness of computed tomography (CT) in the diagnosis of portosystemic collaterals in liver cirrhosis. Seventy-eight patients with liver cirrhosis underwent both CT and angiography. Comparison was made between CT and angiography on eleven types of collaterals, and many of them were demonstrated on CT scans better than angio. Especially, esophageal varices, paraesophageal varices, umbilical pathway and caput medusa were diagnostic on CT scans. Gastrorenal collaterals, splenorenal collaterals, retroperitoneal pathway are also well demonstrated. Dilatation of azygos systems and small veins in the liver surface are only observed on CT scans. However, coronary varices and short gastric varices are well diagnostic in angiography. But considering all types of collaterals, it was stressed that angiography can be eliminated by CT in evaluation of collaterals in liver cirrhosis. (author)

  10. Understanding the Pathophysiology of Portosystemic Shunt by Simulation Using an Electric Circuit.

    Science.gov (United States)

    Kim, Moonhwan; Lee, Keon-Young

    2016-01-01

    Portosystemic shunt (PSS) without a definable cause is a rare condition, and most of the studies on this topic are small series or based on case reports. Moreover, no firm agreement has been reached on the definition and classification of various forms of PSS, which makes it difficult to compare and analyze the management. The blood flow can be seen very similar to an electric current, governed by Ohm's law. The simulation of PSS using an electric circuit, combined with the interpretation of reported management results, can provide intuitive insights into the underlying mechanism of PSS development. In this article, we have built a model of PSS using electric circuit symbols and explained clinical manifestations as well as the possible mechanisms underlying a PSS formation.

  11. Transjugular intrahepatic portosystemic shunt by direct transcaval approach: indications and anatomic foundation

    International Nuclear Information System (INIS)

    Chu Jianguo; Sun Xiaoli; Huang He; Xu Xiaoming; Pu Longsong; Lv Chunyan; Sun Peng; Yang Shuhui; Liu Shuying

    2004-01-01

    Objective: To investigate into the indications and related anatomic foundation of transjugular intrahepatic portosystemic shunt (TIPS) creation by direct transcaval approach in patients with portal hypertension cirrhosis suffering unusual anatomy between the hepatic veins and portal bifurcation; and to evaluate the security, feasibility and clinical significance. Methods: Direct transcaval approach TIPS were performed in 65 patients including active variceal bleeding (n=52), intractable ascites (n=12), and as a bridge to liver transplantation (n=1). Results: Technical and functional success were achieved in all patients. The success rate was 100% without related complications including the technique and primary patency rate is obvious higher than classical TIPS. Conclusion: In patients with unusual anatomy between the hepatic veins and portal bifurcation, and inaccessible or inadequate hepatic veins, transcaval TIPS creation is secure and feasible. The results suggest that the direct transcaval approach offering favorable primary patency because the shunt has a straight line in construction

  12. Transjugular intrahepatic portosystemic shunt in a patient with cavernomatous portal vein occlusion

    International Nuclear Information System (INIS)

    Kawamata, Hiroshi; Kumazaki, Tatsuo; Kanazawa, Hidenori; Takahashi, Shuji; Tajima, Hiroyuki; Hayashi, Hiromitsu

    2000-01-01

    A 23-year-old woman with liver cirrhosis secondary to primary sclerosing cholangitis was referred to us for the treatment of recurrent bleeding from esophageal varices that had been refractory to endoscopic sclerotherapy. Her portal vein was occluded, associated with cavernous transformation. A transjugular intrahepatic portosystemic shunt (TIPS) was performed after a preprocedural three-dimensional computed tomographic angiography evaluation to determine feasibility. The portal vein system was recanalized and portal blood flow increased markedly after TIPS. Esophageal varices disappeared 3 weeks after TIPS. Re-bleeding and hepatic encephalopathy were absent for 3 years after the procedure. We conclude that with adequate preprocedural evaluation, TIPS can be performed safely even in patients with portal vein occlusion associated with cavernous transformation.

  13. Transjugular intrahepatic portosystemic shunt: results and prognostic factors in patients with post-necrotic liver cirrhosis

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jae Hyung; Chung, Jin Wook; Han, Joon Koo; Han, Man Chung [Seoul National University College of Medicine, Seoul (Korea, Republic of); Kim, Yong Joo [Kyungbook National University College of Medicine, Taegu (Korea, Republic of)

    1997-01-01

    To evaluate the effectiveness of transjugular intrahepatic portosystemic shunt(TIPS) in the management of gastroesophageal variceal bleeding and predictive factors for long-term survival in patients with post-necrotic liver cirrhosis. A total of 49 patients with post-necrotic liver cirrhosis underwent TIPS over a recent three-year period. Forty-five had a history of hepatitis B viral infection, and four, of hepatitis C viral infection. In all patients, the indication for the procedure was variceal bleeding. Child-Pugh class was A in seven patients, B in 16 and C in 26 patients at the time of the last bleeding. The effectiveness of portal decompression and bleeding control was evaluated. Long-term survival was calculated by the Kaplan-Meier method and predictive factors were analyzed using the Wilcoxon test. The procedure was technically successful in all cases. The portosystemic pressure gradient decreased significantly from 21.4 {+-} 6.4 mmHg to 12.0 {+-} 5.1 mmHg(N=45). Active variceal bleeding was controlled in 34 of the 37 emergency patients. The total length of follow-up was from one day to three and a half years(mean : 383 {+-} 357 days). Rebleeding developed in 17 patients (35%). Hepatic encephalopathy, either newly developed or aggravated, occurred in 16 (32.7%). The thirty-day mortality rate was 20.4%, and the one-year survival rate was 63.8%. The significant predictive factors for poor prognosis were Child-Pugh class C and post-TIPS hepatic encephalopathy. TIPS is effective in portal decompression in the patients with variceal bleeding due to post-necrotic liver cirrhosis. The Child-Pugh classification and hepatic encephalopathy after TIPS are considered to be significant predictive factors for long-term survival.

  14. Transjugular intrahepatic portosystemic shunt: results and prognostic factors in patients with post-necrotic liver cirrhosis

    International Nuclear Information System (INIS)

    Park, Jae Hyung; Chung, Jin Wook; Han, Joon Koo; Han, Man Chung; Kim, Yong Joo

    1997-01-01

    To evaluate the effectiveness of transjugular intrahepatic portosystemic shunt(TIPS) in the management of gastroesophageal variceal bleeding and predictive factors for long-term survival in patients with post-necrotic liver cirrhosis. A total of 49 patients with post-necrotic liver cirrhosis underwent TIPS over a recent three-year period. Forty-five had a history of hepatitis B viral infection, and four, of hepatitis C viral infection. In all patients, the indication for the procedure was variceal bleeding. Child-Pugh class was A in seven patients, B in 16 and C in 26 patients at the time of the last bleeding. The effectiveness of portal decompression and bleeding control was evaluated. Long-term survival was calculated by the Kaplan-Meier method and predictive factors were analyzed using the Wilcoxon test. The procedure was technically successful in all cases. The portosystemic pressure gradient decreased significantly from 21.4 ± 6.4 mmHg to 12.0 ± 5.1 mmHg(N=45). Active variceal bleeding was controlled in 34 of the 37 emergency patients. The total length of follow-up was from one day to three and a half years(mean : 383 ± 357 days). Rebleeding developed in 17 patients (35%). Hepatic encephalopathy, either newly developed or aggravated, occurred in 16 (32.7%). The thirty-day mortality rate was 20.4%, and the one-year survival rate was 63.8%. The significant predictive factors for poor prognosis were Child-Pugh class C and post-TIPS hepatic encephalopathy. TIPS is effective in portal decompression in the patients with variceal bleeding due to post-necrotic liver cirrhosis. The Child-Pugh classification and hepatic encephalopathy after TIPS are considered to be significant predictive factors for long-term survival

  15. Minilaparotomy-Assisted Transmesenteric-Transjugular Intrahepatic Portosystemic Shunt: Comparison with Conventional Transjugular Approach

    Energy Technology Data Exchange (ETDEWEB)

    Jalaeian, Hamed, E-mail: hjalaeia@umn.edu; Talaie, Reza; D’Souza, Donna; Taleb, Shayandokht [University of Minnesota, Division of Interventional Radiology, Department of Radiology (United States); Noorbaloochi, Siamak [University of Minnesota, School of Medicine (United States); Flanagan, Siobhan; Hunter, David; Golzarian, Jafar [University of Minnesota, Division of Interventional Radiology, Department of Radiology (United States)

    2016-10-15

    PurposeThis study was performed to compare the intrahepatic shunt function outcome and procedural complications of minilaparotomy-assisted transmesenteric (MAT)-transjugular intrahepatic portosystemic shunt (TIPS) placement with the conventional transjugular method.MethodsThis is a retrospective review of all patients who had a MAT or conventional TIPS procedure over a 6-year period at our institute. The primary patency rate, fluoroscopy time, technical success, major procedure-related complications, and mortality data were compared between two treatment groups.ResultsWe included 49 patients with MAT-TIPS, and 63 with conventional TIPS, with an average follow-up of 21.43 months. The primary patency rates at 6 and 12 months were 82.9 and 66.7 % in the conventional TIPS group, and 81.0 and 76.5 % in the MAT-TIPS group (p = 1.000, and 0.529), respectively. There was no significant difference in technical success rate, post-procedure portosystemic pressure gradient, fluoroscopy time, and peri-procedural mortality rate between treatment groups. Major procedural-related complications were seen more frequently among MAT-TIPS patients (p = 0.012). In the MAT-TIPS group, 5 (10.2 %) patients developed post-procedure minilaparotomy, wound-related complications, and 5 (10.2 %) developed bacterial peritonitis; whereas, none of patients with conventional TIPS had either of these complications (p = 0.014).ConclusionWhile both MAT-TIPS and conventional TIPS had similar shunt primary patency rate and technical success rate, the MAT approach was associated with a significantly higher rate of minilaparotomy-related wound complications or infectious complications. These complications maybe prevented by a change in post-procedure monitoring and therapy.

  16. Role of extrahepatic UDP-glucuronosyltransferase 1A1: Advances in understanding breast milk-induced neonatal hyperbilirubinemia.

    Science.gov (United States)

    Fujiwara, Ryoichi; Maruo, Yoshihiro; Chen, Shujuan; Tukey, Robert H

    2015-11-15

    Newborns commonly develop physiological hyperbilirubinemia (also known as jaundice). With increased bilirubin levels being observed in breast-fed infants, breast-feeding has been recognized as a contributing factor for the development of neonatal hyperbilirubinemia. Bilirubin undergoes selective metabolism by UDP-glucuronosyltransferase (UGT) 1A1 and becomes a water soluble glucuronide. Although several factors such as gestational age, dehydration and weight loss, and increased enterohepatic circulation have been associated with breast milk-induced jaundice (BMJ), deficiency in UGT1A1 expression is a known cause of BMJ. It is currently believed that unconjugated bilirubin is metabolized mainly in the liver. However, recent findings support the concept that extrahepatic tissues, such as small intestine and skin, contribute to bilirubin glucuronidation during the neonatal period. We will review the recent advances made towards understanding biological and molecular events impacting BMJ, especially regarding the role of extrahepatic UGT1A1 expression. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Congenital imprinting disorders

    DEFF Research Database (Denmark)

    Eggermann, Thomas; Netchine, Irène; Temple, I Karen

    2015-01-01

    Imprinting disorders (IDs) are a group of eight rare but probably underdiagnosed congenital diseases affecting growth, development and metabolism. They are caused by similar molecular changes affecting regulation, dosage or the genomic sequence of imprinted genes. Each ID is characterised...... by specific clinical features, and, as each appeared to be associated with specific imprinting defects, they have been widely regarded as separate entities. However, they share clinical characteristics and can show overlapping molecular alterations. Nevertheless, IDs are usually studied separately despite...... EUCID.net (European network of congenital imprinting disorders) now aims to promote better clinical care and scientific investigation of imprinting disorders by establishing a concerted multidisciplinary alliance of clinicians, researchers, patients and families. By encompassing all IDs and establishing...

  18. Congenital intestinal lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Popović Dušan Đ.

    2011-01-01

    Full Text Available Background. Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. Case report. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and suportive therapy. Conclusion. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  19. [Congenital intestinal lymphangiectasia].

    Science.gov (United States)

    Popović, Dugan D j; Spuran, Milan; Alempijević, Tamara; Krstić, Miodrag; Djuranović, Srdjan; Kovacević, Nada; Damnjanović, Svetozar; Micev, Marjan

    2011-03-01

    Congenital intestinal lymphangiectasia is a disease which leads to protein losing enteropathy. Tortuous, dilated lymphatic vessels in the intestinal wall and mesenterium are typical features of the disease. Clinical manifestations include malabsorption, diarrhea, steatorrhea, edema and effusions. Specific diet and medication are required for disease control. A 19-year old male patient was hospitalized due to diarrhea, abdominal swelling, weariness and fatigue. Physical examination revealed growth impairment, ascites, and lymphedema of the right hand and forearm. Laboratory assessment indicated iron deficiency anaemia, lymphopenia, malabsorption, inflammatory syndrome, and urinary infection. Enteroscopy and video capsule endoscopy demonstrated dilated lymphatic vessels in the small intestine. The diagnosis was confirmed by intestinal biopsy. The patient was put on high-protein diet containing medium-chain fatty acids, somatotropin and supportive therapy. Congenital intestinal lymphangiectasia is a rare disease, usually diagnosed in childhood. Early recognition of the disease and adequate treatment can prevent development of various complications.

  20. Congenital diaphramatic hernia

    Energy Technology Data Exchange (ETDEWEB)

    Kline-Fath, Beth M. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Fetal Care Center of Cincinnati, Cincinnati, OH (United States); Cincinnati Children' s Hospital Medical Center, Department of Radiology, MLC 5031, Cincinnati, OH (United States)

    2012-01-15

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  1. Congenital Hepatic Cyst

    Directory of Open Access Journals (Sweden)

    Aldo Recinos

    2017-04-01

    Full Text Available Congenital hepatic cyst is a rare and nonsymptomatic condition in infants and children. Its incidence is 2.5% in the postnatal life with a much lower incidence in the prenatal period. Incidental finding on antenatal imaging is the most common presentation. We present a case of a newborn in whom fetal ultrasound detected a cyst within the fetal liver. Postnatal imaging revealed a liver cyst in the right lobe of the liver, with no other intrahepatic structure affected. Liver function tests were abnormal, but the patient was asymptomatic. Posterior follow-up imaging showed a minor decrease in size. Management of congenital hepatic cyst is usually conservative, done with periodic ultrasound monitoring. However, surgical treatment is the mainstay of treatment when hydrops, progressive enlargement, hemorrhage, torsion, or compression of adjacent structures occurs. Malignant transformation can occur, but it is extremely rare. Partial or total removal of the cyst is the preferred treatment in neonates with a large lesion.

  2. Congenital diaphramatic hernia

    International Nuclear Information System (INIS)

    Kline-Fath, Beth M.

    2012-01-01

    Congenital diaphragmatic hernia, despite advances in therapy, remains a complex condition with significant morbidity and mortality. The etiology of the disorder is still incompletely understood, though the pulmonary hypoplasia and pulmonary hypertension that develop secondarily must be overcome to improve survival. Prenatal US and fetal MRI have helped in the development of a greater understanding of this disease. Also with these modalities, measurement techniques have been developed in an attempt to provide prognosticators for the development of pulmonary hypoplasia and pulmonary hypertension. There is a broad range of approaches for performing these measurements, and variability among imaging centers is noted. Despite inconsistent approaches, these techniques have become the foundation for counseling and prenatal and postnatal therapy. It is hoped that with further research with prenatal US and fetal MRI and the development of innovative medical and surgical therapies that the morbidity and mortality of children with congenital diaphragmatic hernias can be significantly reduced. (orig.)

  3. Characterizing Congenital Amusia

    OpenAIRE

    Stewart, Lauren

    2011-01-01

    The ability to make sense of the music in our environment involves sophisticated cognitive mechanisms that, for most people, are acquired effortlessly and in early life. A special population of individuals, with a disorder termed congenital amusia, report lifelong difficulties in this regard. Exploring the nature of this developmental disorder provides a window onto the cognitive architecture of typical musical processing, as well as allowing a study of the relationship between processing of ...

  4. The heart: Congenital disease

    International Nuclear Information System (INIS)

    Higgins, C.B.

    1987-01-01

    The most important diagnostic requirement in congenital heart disease (CHD) is definition of cardiovascular pathoanatomy. The considerable success in operative correction of even the most complex anomalies in recent years compels ever increasing precision in preoperative demonstration of these anomalies. Early experience with magnetic resonance imaging (MRI) at several institutions indicated that this modality is an effective noninvasive technique for evaluation of CHD. Indeed, MRI seems to have some advantage over other techniques, including angiography, for definitive diagnosis of congenital anomalies of the heart and great arteries and veins. The absence of ionizing radiation and contrast medium in MRI is an additional advantage; the former is particularly important for children, who, up to this time, have frequently been subjected to enormous radiation burdens from multiple cineangiograms during initial diagnosis and follow-up. This chapter describes the MRI appearance of cardiovascular anatomy im the segmental fashion proposed for analysis of complex CHD. Likewise, MRI demonstration of congenital cardiovascular lesions is organized into abnormalities situated at the four segmental cardiovascular levels: great vessels, atria, ventricles, and visceroatrial relationship. The role of MRI in evaluation of complex ventricular anomalies such as single ventricle and thoracic aortic abnormalities is specifically described

  5. Autoimmunity and Extrahepatic Manifestations in Treatment-Naïve Children with Chronic Hepatitis C Virus Infection

    Directory of Open Access Journals (Sweden)

    Giuseppe Indolfi

    2012-01-01

    Full Text Available Hepatitis C virus (HCV infection has been associated with autoimmunity and extrahepatic manifestations in adults. Few data are available on these topics in children. Nonorgan specific auto-antibodies development is part of the natural course of chronic hepatitis C in children. Smooth muscle autoantibody is the most common autoantibody found, while liver-kidney microsomal type-1 antibody positivity is the most peculiar autoimmune feature of children with HCV infection. The clinical significance of non-organ specific autoantibodies in the course of paediatric chronic hepatitis C is still debated. Autoantibody positivity can be considered neutral for most patients, while it can be associated with negative connotations for others, especially those positive for liver-kidney microsomal type-1 autoantibody. Subclinical hypothyroidism but not autoimmune thyroiditis has been demonstrated in HCV infection in children, while only few cases of HCV-associated membranoproliferative glomerulonephritis have been described. Single reports are available in the literature reporting the anecdotal association between chronic hepatitis C and other extrahepatic manifestations such as myopathy and opsoclonus-myoclonus syndrome. Despite the low incidence of extrahepatic manifestations of chronic hepatitis C in children, overall, available data suggest a careful monitoring.

  6. Inflammation-based prognostic score is a useful predictor of postoperative outcome in patients with extrahepatic cholangiocarcinoma.

    Science.gov (United States)

    Oshiro, Yukio; Sasaki, Ryoko; Fukunaga, Kiyoshi; Kondo, Tadashi; Oda, Tatsuya; Takahashi, Hideto; Ohkohchi, Nobuhiro

    2013-03-01

    Recent studies have revealed that the Glasgow prognostic score (GPS), an inflammation-based prognostic score, is useful for predicting outcome in a variety of cancers. This study sought to investigate the significance of GPS for prognostication of patients who underwent surgery with extrahepatic cholangiocarcinoma. We retrospectively analyzed a total of 62 patients who underwent resection for extrahepatic cholangiocarcinoma. We calculated the GPS as follows: patients with both an elevated C-reactive protein (>10 mg/L) and hypoalbuminemia (L) were allocated a score of 2; patients with one or none of these abnormalities were allocated a s ore of 1 or 0, respectively. Prognostic significance was analyzed by the log-rank test and a Cox proportional hazards model. Overall survival rate was 25.5 % at 5 years for all 62 patients. Venous invasion (p = 0.01), pathological primary tumor category (p = 0.013), lymph node metastasis category (p GPS (p = 0.008) were significantly associated with survival by univariate analysis. A Cox model demonstrated that increased GPS was an independent predictive factor with poor prognosis. The preoperative GPS is a useful predictor of postoperative outcome in patients with extrahepatic cholangiocarcinoma.

  7. Role of extrahepatic UDP-glucuronosyltransferase 1A1: Advances in understanding breast milk-induced neonatal hyperbilirubinemia

    International Nuclear Information System (INIS)

    Fujiwara, Ryoichi; Maruo, Yoshihiro; Chen, Shujuan; Tukey, Robert H.

    2015-01-01

    Newborns commonly develop physiological hyperbilirubinemia (also known as jaundice). With increased bilirubin levels being observed in breast-fed infants, breast-feeding has been recognized as a contributing factor for the development of neonatal hyperbilirubinemia. Bilirubin undergoes selective metabolism by UDP-glucuronosyltransferase (UGT) 1A1 and becomes a water soluble glucuronide. Although several factors such as gestational age, dehydration and weight loss, and increased enterohepatic circulation have been associated with breast milk-induced jaundice (BMJ), deficiency in UGT1A1 expression is a known cause of BMJ. It is currently believed that unconjugated bilirubin is metabolized mainly in the liver. However, recent findings support the concept that extrahepatic tissues, such as small intestine and skin, contribute to bilirubin glucuronidation during the neonatal period. We will review the recent advances made towards understanding biological and molecular events impacting BMJ, especially regarding the role of extrahepatic UGT1A1 expression. - Highlights: • Breast-feeding can be a factor for the development of neonatal hyperbilirubinemia. • UDP-glucuronosyltransferase (UGT) 1A1 is the sole bilirubin-metabolizing enzyme. • Extrahepatic UGT1A1 plays an important role in bilirubin metabolism. • We discuss the potential mechanism of breast milk-induced neonatal jaundice.

  8. Extrahepatic Pseudoaneurysms and Ruptures of the Hepatic Artery in Liver Transplant Recipients: Endovascular Management and a New Iatrogenic Etiology

    International Nuclear Information System (INIS)

    Saad, Wael E. A.; Dasgupta, Niloy; Lippert, Allison J.; Turba, Ulku C.; Davies, Mark G.; Kumer, Sean; Gardenier, Jason C.; Sabri, Saher S.; Park, Auh-Whan; Waldman, David L.; Schmitt, Timothy; Matsumoto, Alan H.; Angle, John F.

    2013-01-01

    To characterize extrahepatic pseudoaneurysm regarding incidence and etiology and determine the effectiveness of endovascular management. A retrospective audit of 1,857 liver transplants in two institutions was performed (1996–2009). Recipients’ demographics, clinical presentation, transplant type, biliary anastomosis, and presence of biliary endoprostheses were noted. Pseudoaneurysms were classified into iatrogenic (associated with biliary endoprosthesis or angioplasty) or spontaneous extrahepatic pseudoaneurysms. Spontaneous and iatrogenic pseudoaneurysms were compared for time from transplant, presenting symptoms, location in the arterial anatomy, and 3-month graft survival. Arterial patency and 6-month graft survival were calculated. Twenty pseudoaneurysms were found (1.1 %, 20/1,857): 9 (0.5 % of transplants, 9/1,857) were spontaneous and 11 (0.6 % of transplants, 11/1,857) were “iatrogenic” (due to minimally invasive procedures: 4 angioplasty and 7 biliary endoprostheses). Sixty percent (12/20) underwent endovascular management (4 coil embolization and 8 stent-grafts). Technical success was 83 % (10/12) with a mean arterial patency of 70 % (follow-up mean, 4.9; range, 0–18 months). The 1-, 3-, and 6-month graft survival was 70, 40, and 35 %, respectively. Due to minimally invasive procedures, posttransplant extrahepatic pseudoaneurysms are no longer an exclusive complication of the transplant surgery itself. Endovascular management is effective to stabilize patients but has not improved historic postsurgical graft survival.

  9. Extrahepatic Pseudoaneurysms and Ruptures of the Hepatic Artery in Liver Transplant Recipients: Endovascular Management and a New Iatrogenic Etiology

    Energy Technology Data Exchange (ETDEWEB)

    Saad, Wael E. A., E-mail: wspikes@yahoo.com; Dasgupta, Niloy; Lippert, Allison J.; Turba, Ulku C.; Davies, Mark G. [University of Virginia Health System, Division of Vascular Interventional Radiology, Department of Radiology (United States); Kumer, Sean [University of Virginia Health System, Division of Solid Organ Transplantation, Department of Surgery (United States); Gardenier, Jason C.; Sabri, Saher S.; Park, Auh-Whan [University of Virginia Health System, Division of Vascular Interventional Radiology, Department of Radiology (United States); Waldman, David L. [University of Rochester Medical Center, Department of Imaging Sciences (United States); Schmitt, Timothy [University of Virginia Health System, Division of Solid Organ Transplantation, Department of Surgery (United States); Matsumoto, Alan H.; Angle, John F. [University of Virginia Health System, Division of Vascular Interventional Radiology, Department of Radiology (United States)

    2013-02-15

    To characterize extrahepatic pseudoaneurysm regarding incidence and etiology and determine the effectiveness of endovascular management. A retrospective audit of 1,857 liver transplants in two institutions was performed (1996-2009). Recipients' demographics, clinical presentation, transplant type, biliary anastomosis, and presence of biliary endoprostheses were noted. Pseudoaneurysms were classified into iatrogenic (associated with biliary endoprosthesis or angioplasty) or spontaneous extrahepatic pseudoaneurysms. Spontaneous and iatrogenic pseudoaneurysms were compared for time from transplant, presenting symptoms, location in the arterial anatomy, and 3-month graft survival. Arterial patency and 6-month graft survival were calculated. Twenty pseudoaneurysms were found (1.1 %, 20/1,857): 9 (0.5 % of transplants, 9/1,857) were spontaneous and 11 (0.6 % of transplants, 11/1,857) were 'iatrogenic' (due to minimally invasive procedures: 4 angioplasty and 7 biliary endoprostheses). Sixty percent (12/20) underwent endovascular management (4 coil embolization and 8 stent-grafts). Technical success was 83 % (10/12) with a mean arterial patency of 70 % (follow-up mean, 4.9; range, 0-18 months). The 1-, 3-, and 6-month graft survival was 70, 40, and 35 %, respectively. Due to minimally invasive procedures, posttransplant extrahepatic pseudoaneurysms are no longer an exclusive complication of the transplant surgery itself. Endovascular management is effective to stabilize patients but has not improved historic postsurgical graft survival.

  10. Role of extrahepatic UDP-glucuronosyltransferase 1A1: Advances in understanding breast milk-induced neonatal hyperbilirubinemia

    Energy Technology Data Exchange (ETDEWEB)

    Fujiwara, Ryoichi, E-mail: fujiwarar@pharm.kitasato-u.ac.jp [Department of Pharmaceutics, School of Pharmacy, Kitasato University, Tokyo (Japan); Maruo, Yoshihiro [Department of Pediatrics, Shiga University of Medical Science, Shiga (Japan); Chen, Shujuan [Department of Pharmacology, Laboratory of Environmental Toxicology, University of California at San Diego, La Jolla, CA 92093 (United States); Tukey, Robert H., E-mail: rtukey@ucsd.edu [Department of Pharmacology, Laboratory of Environmental Toxicology, University of California at San Diego, La Jolla, CA 92093 (United States); Department of Chemistry & Biochemistry, Laboratory of Environmental Toxicology, University of California at San Diego, La Jolla, CA 92093 (United States)

    2015-11-15

    Newborns commonly develop physiological hyperbilirubinemia (also known as jaundice). With increased bilirubin levels being observed in breast-fed infants, breast-feeding has been recognized as a contributing factor for the development of neonatal hyperbilirubinemia. Bilirubin undergoes selective metabolism by UDP-glucuronosyltransferase (UGT) 1A1 and becomes a water soluble glucuronide. Although several factors such as gestational age, dehydration and weight loss, and increased enterohepatic circulation have been associated with breast milk-induced jaundice (BMJ), deficiency in UGT1A1 expression is a known cause of BMJ. It is currently believed that unconjugated bilirubin is metabolized mainly in the liver. However, recent findings support the concept that extrahepatic tissues, such as small intestine and skin, contribute to bilirubin glucuronidation during the neonatal period. We will review the recent advances made towards understanding biological and molecular events impacting BMJ, especially regarding the role of extrahepatic UGT1A1 expression. - Highlights: • Breast-feeding can be a factor for the development of neonatal hyperbilirubinemia. • UDP-glucuronosyltransferase (UGT) 1A1 is the sole bilirubin-metabolizing enzyme. • Extrahepatic UGT1A1 plays an important role in bilirubin metabolism. • We discuss the potential mechanism of breast milk-induced neonatal jaundice.

  11. Extrahepatic angiogenesis hinders recovery of portal hypertension and collaterals in rats with cirrhosis resolution.

    Science.gov (United States)

    Hsu, Shao-Jung; Tsai, Ming-Hung; Chang, Ching-Chih; Hsieh, Yu-Hsin; Huang, Hui-Chun; Lee, Fa-Yauh; Chuang, Chiao-Ling; Hou, Ming-Chih; Lee, Shou-Dong

    2018-03-30

    Liver cirrhosis is characterized by portal hypertension. However, the alteration of portal hypertension-related derangements during cirrhosis resolution is not well known. The present study aimed to establish animal models with cirrhosis resolution and to investigate the relevant changes during this process. Male Sprague-Dawley rats were applied. In reverse thioacetamide (rTAA) model, rats were randomly allocated into four groups with control, thioacetamide (TAA) cirrhosis and rTAA groups that discontinued TAA for 4 or 8 weeks after cirrhosis induction. In reverse bile duct ligation (rBDL) model, rats received choledochoduodenal shunt surgery upon the establishment of cirrhosis and 4, 8, or 16 weeks were allowed after the surgery. At the end, portal hypertension-related parameters were evaluated. Cirrhosis resolution was observed in rTAA groups. Portal pressure (PP) decreased after cirrhosis resolution but remained higher than control group (control, TAA, rTAA4, rTAA8 (mmHg): 5.4 ± 0.3, 12.9 ± 0.3, 8.6 ± 0.4, 7.6 ± 0.6). Further survey found the increased splanchnic blood flow did not reduce during cirrhosis resolution. The extrahepatic pathological angiogenesis was not ameliorated (% of mesenteric window area: 1.2 ± 0.3, 7.3 ± 1.1, 8.3 ± 1.0, 11.3 ± 2.7). In collateral system, the shunting degree reduced while the vessels structure remained. The vascular contractility of all systems and nitric oxide (NO) production were normalized. In rBDL series, PP decreased in rBDL16 groups but the extrahepatic angiogenesis persisted. In conclusion, cirrhosis resolution attenuates but not completely normalizes portal hypertension because of persistently high splanchnic inflow and angiogenesis. In clinical setting, vascular complications such as varices could persist after cirrhosis resolution and further investigation to define the follow-up and treatment strategies is anticipated. © 2018 The Author(s). Published by Portland Press Limited on behalf of the Biochemical

  12. Extrahepatic disposition of 3H-aflatoxin B1 in the rainbow trout (Oncorhynchus mykiss)

    International Nuclear Information System (INIS)

    Larsson, P.; Tjaelve, H.; Ngethe, S.; Ingebrigtsen, K.

    1992-01-01

    Whole-body autoradiography in rainbow trout (Oncorhynchus mykiss) after oral and intravenous administration of 3 H-labelled aflation B 1 showed labelling of several extrahepatic tissues, such as the uveal melanin and the vitreous humour of the eyes, the trunk and head kidney, the olfactory rosettes and the pyloric caecae. Liquid chromatography of extracts of the vitreous humour showed that unmetabolized 3 H-AFB 1 was the main labelled material present at this site. Liquid chromatography of extracts of the uveal melanin showed presence of aflatoxicol and aflatoxin B 1 in proportions of about 3:1. The binding to the pigment is probably due to a hydrophobic type of interaction with the melanin. Microautoradiography showed that melanin-containing cells in the trunk and head kidney and in the olfactory rosettes also accumulated high amounts of radioactivity. In the trunk kidney there was, in addition, a labelling of the second segment of the proximal tubules and of the distal tubules and the collecting ducts. Studies in vitro with microsomal and 12,000xg supernatant preparations of the trunk kidney showed formation of DNA- and protein-bound metabolites from the aflatoxin B 1 . It is probable that the bioactivation of the aflatoxin B 1 is confined to the second segment of the proximal tubules. The labelling of the distal tubules and the collecting ducts, which was confined to the cytoplasm of the cells, may be related to excretion and/or absorption processes. Microautoradiography of the olfactory rosettes, showed labelling of the sensory epithelium, but not the indifferent epithelium. A low formation of protein-bound aflatoxin B 1 -metabolites was found in incubations with microsomal preparations of this tissue. The same observation was made in incubations with microsomal preparations of the head kidney. In the pyloric caeca bound metabolites were observed in vivo at a level comparable to that found in the trunk kidney. Our results suggest that retention and metabolism

  13. Radiology of congenital heart disease

    International Nuclear Information System (INIS)

    Amplatz, K.

    1986-01-01

    This is a text on the radiologic diagnosis of congenital heart disease and its clinical manifestations. The main thrust of the book is the logical approach which allows an understanding of the complex theory of congenital heart disease. The atlas gives a concise overview of the entire field of congenital heart disease. Emphasis is placed on the understanding of the pathophysiology and its clinical and radiological consequences. Surgical treatment is included since it provides a different viewpoint of the anatomy

  14. Early detection of congenital syphilis

    Directory of Open Access Journals (Sweden)

    Nagalakshmi Chowdhary

    2014-01-01

    Full Text Available Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson′s teeth.

  15. Genetics Home Reference: congenital mirror movement disorder

    Science.gov (United States)

    ... Health Conditions Congenital mirror movement disorder Congenital mirror movement disorder Printable PDF Open All Close All Enable ... view the expand/collapse boxes. Description Congenital mirror movement disorder is a condition in which intentional movements ...

  16. The origin and reduction of spurious extrahepatic counts observed in 90Y non-TOF PET imaging post radioembolization

    Science.gov (United States)

    Walrand, Stephan; Hesse, Michel; Jamar, François; Lhommel, Renaud

    2018-04-01

    Our literature survey revealed a physical effect unknown to the nuclear medicine community, i.e. internal bremsstrahlung emission, and also the existence of long energy resolution tails in crystal scintillation. None of these effects has ever been modelled in PET Monte Carlo (MC) simulations. This study investigates whether these two effects could be at the origin of two unexplained observations in 90Y imaging by PET: the increasing tails in the radial profile of true coincidences, and the presence of spurious extrahepatic counts post radioembolization in non-TOF PET and their absence in TOF PET. These spurious extrahepatic counts hamper the microsphere delivery check in liver radioembolization. An acquisition of a 32P vial was performed on a GSO PET system. This is the ideal setup to study the impact of bremsstrahlung x-rays on the true coincidence rate when no positron emission and no crystal radioactivity are present. A MC simulation of the acquisition was performed using Gate-Geant4. MC simulations of non-TOF PET and TOF-PET imaging of a synthetic 90Y human liver radioembolization phantom were also performed. Internal bremsstrahlung and long energy resolution tails inclusion in MC simulations quantitatively predict the increasing tails in the radial profile. In addition, internal bremsstrahlung explains the discrepancy previously observed in bremsstrahlung SPECT between the measure of the 90Y bremsstrahlung spectrum and its simulation with Gate-Geant4. However the spurious extrahepatic counts in non-TOF PET mainly result from the failure of conventional random correction methods in such low count rate studies and poor robustness versus emission-transmission inconsistency. A novel proposed random correction method succeeds in cleaning the spurious extrahepatic counts in non-TOF PET. Two physical effects not considered up to now in nuclear medicine were identified to be at the origin of the unusual 90Y true coincidences radial profile. TOF reconstruction removing

  17. Invaded depth of extrahepatic bile duct cancer diagnosed on angiographic CT during late contrast enhancement

    International Nuclear Information System (INIS)

    Kikuyama, Masataka; Sasada, Yuzo; Koide, Shigeki; Hirai, Ritsuko; Oota, Yuji

    2006-01-01

    Thirteen patients with extrahepatic bile duct cancer were evaluated for enhancement pattern and tumor feature during late contrast enhancement on angiographic CT (AG-CT), performed before surgery. Eleven patients with late contrast enhancement had advanced cancers with invasion over the subserosal layer. Eight of these patients whose cancer had an irregular outer layer had invasion over the serosal layer (≥panc 1 b, General Rules for Surgical and Pathological Studies on Cancer of the Biliary Tract, Japanese Society of Biliary Surgery). Seven of the 13 patients had intra-pancreatic bile duct cancer. Among them, five had an irregular outer layer of the intra-pancreatic bile duct cancer; one of three with a wall thickness under 5 mm on AG-CT had panc 1 b invasion, and the other two had panc 2 (General Rules for Surgical and Pathological Studies on Cancer of the Biliary Tract, Japanese Society of Biliary Surgery) invasion. Two with a wall thickness over 5 mm on AG-CT had panc 2 invasion. These results suggest that late contrast enhancement on AG-CT is effective for diagnosing the depth of tumor invasion of the bile duct. (author)

  18. Therapeutic percutaneous ultrasound-guided cholecystocentesis in three dogs with extrahepatic biliary obstruction and pancreatitis.

    Science.gov (United States)

    Herman, Beth A; Brawer, Robert S; Murtaugh, Robert J; Hackner, Susan G

    2005-12-01

    Three dogs were examined because of acute pancreatitis. In all 3, distension of the gallbladder was seen ultrasonographically, and extrahepatic biliary tract obstruction (EHBO) was diagnosed on the basis of ultrasonographic findings and serum biochemical abnormalities (i.e., high serum bilirubin and cholesterol concentrations and increased hepatic enzyme activities). In all 3 dogs, percutaneous ultrasound-guided cholecystocentesis (PUCC) was used to decompress the gallbladder, with cholecystocentesis performed multiple times in 1 dog. Serum bilirubin concentration was substantially decreased following the procedure in all 3 dogs. Two of the 3 dogs did not require surgery to resolve the obstruction. In the third dog, an exploratory laparotomy was performed because of concerns about development of abdominal effusion following the procedure. Bile staining of the mesenteric fat was seen during the laparotomy, but no defect in the gallbladder wall could be identified. In most dogs with EHBO secondary to pancreatitis, the obstruction resolves spontaneously as the acute pancreatitis improves so that surgery is not required. In those few dogs in which EHBO does not resolve or in which EHBO results in complications, therapeutic PUCC may be useful in relieving gallbladder distension.

  19. Molecular biomarkers in extrahepatic bile duct cancer patients undergoing chemoradiotherapy for gross residual disease after surgery

    Energy Technology Data Exchange (ETDEWEB)

    Koh, Hyeon Kang; Kim, Kyu Bo; Chie, Eui Kyu; Ha, Sung W. [Seoul National University College of Medicine, Seoul (Korea, Republic of); Park, Hae Jin [Dept. of Radiation Oncology, Soonchunhyang University Hospital, Seoul (Korea, Republic of)

    2012-12-15

    To analyze the outcomes of chemoradiotherapy for extrahepatic bile duct (EHBD) cancer patients who underwent R2 resection or bypass surgery and to identify prognostic factors affecting clinical outcomes, especially in terms of molecular biomarkers. Medical records of 21 patients with EHBD cancer who underwent R2 resection or bypass surgery followed by chemoradiotherapy from May 2001 to June 2010 were retrospectively reviewed. All surgical specimens were re-evaluated by immunohistochemical staining using phosphorylated protein kinase B (pAKT), CD24, matrix metalloproteinase 9 (MMP9), survivin, and {beta}-catenin antibodies. The relationship between clinical outcomes and immunohistochemical results was investigated. At a median follow-up of 20 months, the actuarial 2-year locoregional progression-free, distant metastasis-free and overall survival were 37%, 56%, and 54%, respectively. On univariate analysis using clinicopathologic factors, there was no significant prognostic factor. In the immunohistochemical staining, cytoplasmic staining, and nuclear staining of pAKT was positive in 10 and 6 patients, respectively. There were positive CD24 in 7 patients, MMP9 in 16 patients, survivin in 8 patients, and {beta}-catenin in 3 patients. On univariate analysis, there was no significant value of immunohistochemical results for clinical outcomes. There was no significant association between clinical outcomes of patients with EHBD cancer who received chemoradiotherapy after R2 resection or bypass surgery and pAKT, CD24, MMP9, survivin, and {beta}-catenin. Future research is needed on a larger data set or with other molecular biomarkers.

  20. Plastic or metal stents for benign extrahepatic biliary strictures: a systematic review

    Directory of Open Access Journals (Sweden)

    Vleggaar Frank P

    2009-12-01

    Full Text Available Abstract Background Benign biliary strictures may be a consequence of surgical procedures, chronic pancreatitis or iatrogenic injuries to the ampulla. Stents are increasingly being used for this indication, however it is not completely clear which stent type should be preferred. Methods A systematic review on stent placement for benign extrahepatic biliary strictures was performed after searching PubMed and EMBASE databases. Data were pooled and evaluated for technical success, clinical success and complications. Results In total, 47 studies (1116 patients on outcome of stent placement were identified. No randomized controlled trials (RCTs, one non-randomized comparative studies and 46 case series were found. Technical success was 98,9% for uncovered self-expandable metal stents (uSEMS, 94,8% for single plastic stents and 94,0% for multiple plastic stents. Overall clinical success rate was highest for placement of multiple plastic stents (94,3% followed by uSEMS (79,5% and single plastic stents (59.6%. Complications occurred more frequently with uSEMS (39.5% compared with single plastic stents (36.0% and multiple plastic stents (20,3%. Conclusion Based on clinical success and risk of complications, placement of multiple plastic stents is currently the best choice. The evolving role of cSEMS placement as a more patient friendly and cost effective treatment for benign biliary strictures needs further elucidation. There is a need for RCTs comparing different stent types for this indication.

  1. Prognostic significance of contrast-enhanced CT attenuation value in extrahepatic cholangiocarcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Asayama, Yoshiki [Kyushu University, Department of Advanced Imaging and Interventional Radiology, Graduate School of Medical Sciences, Fukuoka (Japan); Nishie, Akihiro; Ishigami, Kousei; Ushijima, Yasuhiro; Takayama, Yukihisa; Okamoto, Daisuke; Fujita, Nobuhiro; Honda, Hiroshi [Kyushu University, Departments of Clinical Radiology, Fukuoka (Japan); Ohtsuka, Takao [Kyushu University, Departments of Surgery and Oncology, Fukuoka (Japan); Yoshizumi, Tomoharu [Kyushu University, Departments of Surgery and Sciences, Fukuoka (Japan); Aishima, Shinichi [Saga University, Pathology and Microbiology, Faculty of Medicine, Saga (Japan); Kyushu University, Departments of Anatomic Pathology, Graduate School of Medical Sciences, Fukuoka (Japan); Oda, Yoshinao [Kyushu University, Departments of Anatomic Pathology, Graduate School of Medical Sciences, Fukuoka (Japan)

    2017-06-15

    To determine whether washout characteristics of dynamic contrast-enhanced computed tomography (CT) could predict survival in patients with extrahepatic cholangiocarcinoma (EHC). This study collected 46 resected cases. All cases were examined by dynamic contrast study on multidetector-row CT. Region-of-interest measurements were obtained at the non-enhanced, portal venous phase and delayed phase in the tumour and were used to calculate the washout ratio as follows: [(attenuation value at portal venous phase CT - attenuation value at delayed enhanced CT)/(attenuation value at portal venous phase CT - attenuation value at unenhanced CT)] x 100. On the basis of the median washout ratio, we classified the cases into two groups, a high-washout group and low-washout group. Associations between overall survival and various factors including washout rates were analysed. The median washout ratio was 29.4 %. Univariate analysis revealed that a lower washout ratio, venous invasion, lymphatic permeation and lymph node metastasis were associated with shorter survival. Multivariate analysis identified the lower washout ratio as an independent prognostic factor (hazard ratio, 3.768; p value, 0.027). The washout ratio obtained from the contrast-enhanced CT may be a useful imaging biomarker for the prediction of survival of patients with EHC. (orig.)

  2. Molecular biomarkers in extrahepatic bile duct cancer patients undergoing chemoradiotherapy for gross residual disease after surgery

    International Nuclear Information System (INIS)

    Koh, Hyeon Kang; Kim, Kyu Bo; Chie, Eui Kyu; Ha, Sung W.; Park, Hae Jin

    2012-01-01

    To analyze the outcomes of chemoradiotherapy for extrahepatic bile duct (EHBD) cancer patients who underwent R2 resection or bypass surgery and to identify prognostic factors affecting clinical outcomes, especially in terms of molecular biomarkers. Medical records of 21 patients with EHBD cancer who underwent R2 resection or bypass surgery followed by chemoradiotherapy from May 2001 to June 2010 were retrospectively reviewed. All surgical specimens were re-evaluated by immunohistochemical staining using phosphorylated protein kinase B (pAKT), CD24, matrix metalloproteinase 9 (MMP9), survivin, and β-catenin antibodies. The relationship between clinical outcomes and immunohistochemical results was investigated. At a median follow-up of 20 months, the actuarial 2-year locoregional progression-free, distant metastasis-free and overall survival were 37%, 56%, and 54%, respectively. On univariate analysis using clinicopathologic factors, there was no significant prognostic factor. In the immunohistochemical staining, cytoplasmic staining, and nuclear staining of pAKT was positive in 10 and 6 patients, respectively. There were positive CD24 in 7 patients, MMP9 in 16 patients, survivin in 8 patients, and β-catenin in 3 patients. On univariate analysis, there was no significant value of immunohistochemical results for clinical outcomes. There was no significant association between clinical outcomes of patients with EHBD cancer who received chemoradiotherapy after R2 resection or bypass surgery and pAKT, CD24, MMP9, survivin, and β-catenin. Future research is needed on a larger data set or with other molecular biomarkers.

  3. [Prevalence of extra-hepatic manifestations in infection with hepatitis C virus: study of 140 cases].

    Science.gov (United States)

    Kahloun, A; Babba, T; Fathallah, B; Ghozzi, M; Ezzine, H; Said, Y; Azzouz, M M

    2011-06-01

    To determine the extrahepatic manifestations (EHM) in chronic hepatitis C and to correlate signs with age, sex, degree of fibrosis and genotype of hepatitis C virus. One hundred forty cases of chronic infection by hepatitis C virus were investigated in a period of 10 years. By interrogation, clinical examination and laboratory tests, the EHM were determined. Correlations with age, sex, viral genotype and degree of fibrosis were determined by the chi2 test. Mean age of our patients was 59 years (16-85 years). 74% were women. The genotype 1b was found in 75% of cases. The clinical EHM were found in 62% of cases: buccal dryness in 17.1% of cases, arthralgias in 33% of cases and fatigue in 65% of cases. 25% of patients had at least one biological EHM associated with chronic hepatitis C: proteinuria in 3 cases, cryoglobulinemia in 4 cases, dysthyroidism in 8 cases and more frequently a positive immunologie test. During the follow-up, we found one case of breast cancer, one case of rectal cancer, 2 cases of MALT lymphoma and one case pf splenic lymphoma. A positive correlation was found between the prevalence of EHM in chronic hepatitis C and the female sex. A degree of fibrosis ³ 2 in METAVIR classification was significantly associated with more important frequency of EHM. EHM should be screened systematically in chronic infection with HCV. Pathogenic mechanisms in a B lymph node proliferation or diabetes and outcome of these abnormalities under antiviral therapy should be further investigated.

  4. Colloid Carcinoma of the Extrahepatic Biliary Tract with Metastatic Lymphadenopathy Mimicking Cystic Neoplasm: A Case Report

    International Nuclear Information System (INIS)

    Han, Na Yeon; Park, Beom Jin; Sung, Deuk Jae; Kim, Min Ju; Cho, Sung Bum; Kim, Dong Sik; Lee, Jeong Hyeon

    2013-01-01

    The patient is a previously healthy 52-year-old woman who presented with dyspepsia for two months. Multiple imaging modalities including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) showed diffuse bile duct dilatation with an obstructive lesion of the distal extrahepatic biliary duct (EHD) as well as two masses in the peripancreatic area. The peripancreatic masses appeared cystic with posterior acoustic enhancement on ultrasound, low density on CT imaging, and high signal intensity on T2-weighted MRI. The lesion in the distal EHD exhibited similar characteristics on CT and MRI. A Whipple procedure was performed and histological specimens showed malignant cells with large mucin pools that was consistent with a diagnosis of colloid carcinoma of the EHD with metastatic lymphadenopathies. Colloid carcinoma, also called mucinous carcinoma, is classified as a histologic variant of adenocarcinoma. Because the colloid carcinoma of the biliary tree is exceedingly rare, the imaging characteristics and the clinical features of colloid carcinoma remain relatively unknown. We report a case of colloid carcinoma of the common bile duct and its accompanied metastatic lymphadenopathies with characteristic imaging findings reflecting abundant intratumoral mucin pools

  5. Molecular and Genetic Studies of Congenital Myopathies

    Science.gov (United States)

    2018-03-21

    Central Core Disease; Centronuclear Myopathy; Congenital Fiber Type Disproportion; Multiminicore Disease; Myotubular Myopathy; Nemaline Myopathy; Rigid Spine Muscular Dystrophy; Undefined Congenital Myopathy

  6. Genetics Home Reference: Fukuyama congenital muscular dystrophy

    Science.gov (United States)

    ... with mental retardation Muscular dystrophy, congenital, Fukuyama type Muscular dystrophy, congenital, with central nervous system involvement Polymicrogyria with muscular dystrophy Related Information How ...

  7. Are congenital malformations more frequent in fetuses with intrahepatic persistent right umbilical vein? A comparative study.

    Science.gov (United States)

    Adiego-Calvo, Ignacio; Saviron-Cornudella, Ricardo; Martinez-Payo, Cristina; Rubio-Aranda, Encarna; Sancho-Sauco, Javier; Cisneros-Gimeno, Ana Isabel; Perez-Perez, Pilar; Lerma-Puertas, Diego; Whyte-Orozco, Jaime

    2016-12-01

    Persistent right umbilical vein (PRUV) is a vascular anomaly where the right umbilical vein remains as the only conduit that returns oxygenated blood to the fetus. It has classically been described as associated with numerous defects. We distinguish the intrahepatic variant (better prognosis) and the extrahepatic variant (associated with worse prognosis). The objective of this study was to compare rates of congenital malformations in fetuses with intrahepatic PRUV (I-PRUV) versus singleton pregnancies without risk factors. A multicenter, crossover design, comparative study was performed between 2003 and 2013 on fetuses diagnosed with I-PRUV (n=56), and singleton pregnancies without congenital malformation risk factors (n=4050). Fifty-six cases of I-PRUV were diagnosed (incidence 1:770). A statistically significant association between I-PRUV and the presence of congenital malformations (odds ratio 4.321; 95% confidence interval 2.15-8.69) was found. This positive association was only observed with genitourinary malformations (odds ratio 3.038; 95% confidence interval 1.08-8.56). Our rate of malformations associated with I-PRUV (17.9%) is similar to previously published rates. I-PRUV has shown a significant increase in the rate of associated malformations, although this association has only been found to be statistically significant in the genitourinary system. Noteworthy is the fact that this comparative study has not pointed to a significant increase in the congenital heart malformation rate. Diagnosis of isolated I-PRUV does not carry a worse prognosis. Copyright © 2016. Published by Elsevier B.V.

  8. Prevalence of Congenital Malformations

    Directory of Open Access Journals (Sweden)

    Akhavan Karbasi Sedighah

    2009-05-01

    Full Text Available Congenital malformation (CM will begin to emerge as one of the major childhood health problems .Treatment and rehabilitation of children with congenital malformations are costly and complete recovery is usually impossible. The aim of this study was to determine frequency of CM in Yazd central city of the Islamic Republic of Iran to find out if there has been any difference in the rate and types of CM in this area. This descriptive-observational study carried on 4800 births delivered at all maternity hospitals in Yazd from October 2003 to June 2004. Prevalence of CM was 2.83% (2.86 % in male and 2.68 % in female out of the 136 cases 69(51.88% were males and 64 (48.12% were females and 3 with ambiguous genitalia. Positive family history of CM in sibling was in only 6 cases (4.41%.Overall, musculoskeletal (0.83%, central nerv-ous system (0.47% and genital system (0.37% were accounted as the most common. Frequency of CM was more seen in still birth (12.5% as in comparison to live birth (2.71%. There was not statistical difference be-tween prevalence of CM and neonatal's gender, gestational age, birth order and mother's age, drug ingestion, illness and parental consanguinity. In this study the overall prevalence of congenital malformation among the newborn was higher than those previous reported in Iran and determining the causes of this difference needs more extensive studies.

  9. Congenital Midureteric Stricture

    Directory of Open Access Journals (Sweden)

    Singh Shalinder

    2001-01-01

    Full Text Available Congenital midureteric obstruction is a rare entity which can be caused by either ureteric valves or strictures. We report our experience with four patients with midureteric obstruction due to stricture over a six-year period. The condition needs to be differentiated from obstruction of the pelviureteric and vesicoureteric junctions. Obstruction can be initially screened by ultrasound and confirmed by a radionuclide scan with furosemide challenge. Retrograde ureteropyelography and/or intravenous urography may be required to define the level of the stricture.

  10. Nonclassic Congenital Adrenal Hyperplasia

    Directory of Open Access Journals (Sweden)

    Selma Feldman Witchel

    2010-01-01

    Full Text Available Nonclassic congenital adrenal hyperplasia (NCAH due to P450c21 (21-hydroxylase deficiency is a common autosomal recessive disorder. This disorder is due to mutations in the CYP21A2 gene which is located at chromosome 6p21. The clinical features predominantly reflect androgen excess rather than adrenal insufficiency leading to an ascertainment bias favoring diagnosis in females. Treatment goals include normal linear growth velocity and “on-time” puberty in affected children. For adolescent and adult women, treatment goals include regularization of menses, prevention of progression of hirsutism, and fertility. This paper will review key aspects regarding pathophysiology, diagnosis, and treatment of NCAH.

  11. Congenital Syphilis Masquerading as Leukemia

    OpenAIRE

    Lee, Tiffany; Bell, Stephanie; Scimeme, Jason; Maraqa, Nizar

    2017-01-01

    As of late, the incidence of congenital syphilis in the United States is increasing. Each new case represents a failure of preventing, diagnosing, and treating syphilis in pregnant women. Pediatricians should confirm that all women have received adequate screening for and management of syphilis during pregnancy. Congenital syphilis is easily treatable but may be a diagnostic challenge with high morbidity and mortality.

  12. What Are Congenital Heart Defects?

    Science.gov (United States)

    ... a baby with a congenital heart defect. Family history and genetics Congenital heart disease is not usually passed along ... you or your child to a specialist in genetic testing. Cardiac MRI to diagnose a ... Factors to review family history, smoking, and medicines that increase your risk of ...

  13. [Neonatal tumours and congenital malformations].

    Science.gov (United States)

    Berbel Tornero, O; Ortega García, J A; Ferrís i Tortajada, J; García Castell, J; Donat i Colomer, J; Soldin, O P; Fuster Soler, J L

    2008-06-01

    The association between pediatric cancer and congenital abnormalities is well known but, there is no exclusive data on the neonatal period and the underlying etiopathogenic mechanisms are unknown. First, to analyze the frequency of neonatal tumours associated with congenital abnormalities; and second, to comment on the likely etiopathogenic hypotheses of a relationship between neonatal tumours and congenital abnormalities. Historical series of neonatal tumours from La Fe University Children's Hospital in Valencia (Spain), from January 1990 to December 1999. Histological varieties of neonatal tumours and associated congenital abnormalities were described. A systematic review of the last 25 years was carried out using Medline, Cancerlit, Index Citation Science and Embase. The search profile used was the combination of "neonatal/congenital-tumors/cancer/neoplasms" and "congenital malformations/birth defects". 72 neonatal tumours were identified (2.8% of all pediatric cancers diagnosed in our hospital) and in 15 cases (20.8%) there was some associated malformation, disease or syndrome. The association between congenital abnormalities and neonatal tumours were: a) angiomas in three patients: two patients with congenital heart disease with a choanal stenosis, laryngomalacia; b) neuroblastomas in two patients: horseshoe kidney with vertebral anomalies and other with congenital heart disease; c) teratomas in two patients: one with cleft palate with vertebral anomalies and other with metatarsal varus; d) one tumour of the central nervous system with Bochdaleck hernia; e) heart tumours in four patients with tuberous sclerosis; f) acute leukaemia in one patient with Down syndrome and congenital heart disease; g) kidney tumour in one case with triventricular hydrocephaly, and h) adrenocortical tumour: hemihypertrophy. The publications included the tumours diagnosed in different pediatric periods and without unified criteria to classify the congenital abnormalities. Little data

  14. Congenital nystagmus and negative electroretinography

    Directory of Open Access Journals (Sweden)

    Roussi M

    2011-04-01

    Full Text Available Mirella Roussi, Hélène Dalens, Jean Jacques Marcellier, Franck BacinDepartment of Ophthalmology, Clermont-Ferrand University, Clermont-Ferrand, FranceAbstract: Congenital nystagmus is a pathologic oculomotor state appearing at about three to four months of age. The precise diagnosis requires detailed clinical examination and electrophysiological findings. This case report presents two male patients with congenital nystagmus examined longitudinally from the age of six months until 17-18 years of age. Clinical and electrophysiological protocols were detailed. The first results showed electronegative electroretinography in the two cases and examination combined with electroretinographic findings helped us to make the diagnosis of Congenital Night Stationary Blindness (CSNB. This diagnosis was confirmed by genetic studies. CSNB is interesting to study because through electrophysiological findings, it enables a better understanding of the physiology of neural transmission in the outer part of the retina.Keywords: Congenital nystagmus, negative electroretinography, congenital night stationary blindness

  15. Congenital Leukemia in Down's syndrome

    International Nuclear Information System (INIS)

    Iqbal, W.; Khan, F.; Muzaffar, M.; Khan, U. A.; Rehman, M. U.; Khan, M. A.; Bari, A.

    2006-01-01

    Congenital Leukemia is a condition and often associated with fatal outcome/sup 1/. Most of the neonatal cases reported have acute non-lymphoblastic leukemia, in contrast to the predominance of acute lymphoblastic leukemia found in later childhood. congenital leukemia is occasionally associated with number of congenital anomalies and with chromosomal disorders such as Down's syndrome. Subtle cytogenetic abnormalities may occur more commonly in the affected infants and their parents, when studied with newer cytogenetic techniques/sup 2/. Inherent unstable hematopoieses resulting from chromosomal aberration in children with Downs's syndrome can present with transient myeloproliferative disorder, mimicking leukemia which undergoes spontaneous recovery/sup 3/. Only few cases of congenital leukemia with Downs syndrome, presented as congenital leukemia. (author)

  16. Congenital nephrotic syndrome

    Directory of Open Access Journals (Sweden)

    Claudia Fanni

    2014-06-01

    Full Text Available CNS (Congenital nephrotic syndrome is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary and secondary forms (acquired and associated with other syndromes. The most common form is the Finnish CNS (CNF, congenital nephrotic syndrome of the Finnish type, a hereditary form whose name derives from the fact that the highest incidence is described in that country (1.2:10,000. The pathogenesis, the clinical picture, the diagnostic criteria, the therapy and the outcome are described in details.  Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

  17. Comparison of transhepatic and extrahepatic routes for EUS-guided rendezvous procedure for distal CBD obstruction.

    Science.gov (United States)

    Dhir, Vinay; Bhandari, Suryaprakash; Bapat, Mukta; Joshi, Nitin; Vivekanandarajah, Suhirdan; Maydeo, Amit

    2013-04-01

    EUS-guided rendezvous procedure (EUS-RV) can be done by the transhepatic (TH) or the extrahepatic (EH) route. There is no data on the preferred access route when both routes are available. To compare the success, complications, and duration of hospitalization for patients undergoing EUS-RV by the TH or the EH route. Patients with distal common bile duct (CBD) obstruction, who failed selective cannulation, underwent EUS-RV by the TH route through the stomach or the EH route through the duodenum. A total of 35 patients were analysed (17 TH, 18 EH). The mean procedure time was significantly longer for the TH group (34.4 vs. 25.7 min; p = 0.0004). There was no difference in the technical success (94.1 vs. 100%). However, the TH group had a higher incidence of post-procedure pain (44.1 vs. 5.5%; p = 0.017), bile leak (11.7 vs. 0; p = 0.228), and air under diaphragm (11.7 vs. 0; p = 0.228). All bile leaks were small and managed conservatively. Duration of hospitalization was significantly higher for the TH group (2.52 vs. 0.17 days; p = 0.015). EUS-RV has similar success rate by the TH or the EH route. However, the TH route has higher post-procedure pain, longer procedure time, and longer duration of hospitalization. The EH route should be preferred for EUS-RV in patients with distal CBD obstruction when both access routes are technically feasible.

  18. Evaluation of the radiotherapy combined with surgery for extrahepatic bile duct cancer and pancreatoduodenal cancer

    International Nuclear Information System (INIS)

    Takezawa, Jiro

    1984-01-01

    Curative resection of extrahepatic bile duct cancer and pancreatoduodenal cancer is very difficult, and its long term result is pessimistic. Twenty patients with bile duct cancer (12 resected and 8 non-resected) and 23 patients with pancreatoduodenal cancer (10 resected and 13 non-resected) were treated with extracorporeal Cobalt-60 irradiation following the surgery. The just opposed anterior and posterior ports were used with the field size ranging from 6 x 6 cm to 13 x 14 cm. The delivered doses ranged from 46 to 60 Gy (70 - 108TDF) for bile duct cancer, and from 18 to 66 Gy (28 - 104TDF) for pancreatoduodenal cancer. On one of resected and 4 of non-resected patients with pancreatoduodenal cancer, the therapeutic schedule could not be pursued completely because of decreased leucocytes and emaciation. The postoperative survivals were compared between the cases with and without adjunctive radiotherapy. The longest and mean survivals of the patients with resected bile duct cancer were 41 mos. and 20 mos. respectively, in the irradiated v.s. 21 mos. and 14 mos. in the non-irradiated group. The mean survival of the patients without resection of bile duct cancer was 15 mos. in the irradiated v.s. 6 mos. in the non-irradiated group; one resected case has survived more than 61 mos. after radiotherapy and is still alive. The mean survival after resection of pancreatoduodenal cancer was 16 mos. in the irradiated v.s. 11 mos. in non-irradiated group, and 3 in the former are still alive, one having survived the longest 50 mos. The longest and mean survival of the patients without resection of pancreatoduodenal cancer were 22 mos. and 8 mos. respectively v.s. 7 mos. and 4 mos. of the non-irradiated patients. Advantageous effects of radiotherapy combined with surgery for bile duct cancer and pancreatoduodenal cancer were observed throughout this clinical study. (author)

  19. Radiotherapy Is Associated With Improved Survival in Adjuvant and Palliative Treatment of Extrahepatic Cholangiocarcinomas

    International Nuclear Information System (INIS)

    Shinohara, Eric T.; Mitra, Nandita; Guo Mengye; Metz, James M.

    2009-01-01

    Purpose: Extrahepatic cholangiocarcinomas (EHC) are rare tumors of the biliary tree because of their low incidence, large randomized studies examining radiotherapy (RT) for EHC have not been performed. The purpose of this study was to examine the role of adjuvant and palliative RT in the treatment of EHC in a large patient population. Methods and Materials: This was a retrospective analysis of 4,758 patients with EHC collected from the Surveillance, Epidemiology, and End Results database. The primary endpoint was overall survival. Results: Patients underwent surgery (28.8%), RT (10.0%), surgery and RT (14.7%), or no RT or surgery (46.4%). The median age of the patient population was 73 years (range, 23-104), 52.5% were men, and 80.7% were white. The median overall survival time was 16 months (95% confidence interval [CI] 15-17), 9 months (95% CI 9-11), 9 months (95% CI 9-10), and 4 months (95% CI 3-4) for surgery and RT, surgery, RT, and no RT or surgery, respectively. The overall survival was significantly different between the surgery and surgery and RT groups (p < .0001) and RT and no RT or surgery groups (p < .0001) on the log-rank test. The propensity score-adjusted analyses of surgery and RT vs. surgery (hazard ratio, 0.94; 95% CI, 0.84-1.05) were not significantly different, but that for RT vs. no RT or surgery (hazard ratio, 0.61; 95% CI, 0.54-0.70) was significantly different. Conclusion: These results suggest that palliative RT prolongs survival in patients with EHC. The benefit associated with surgery and RT was significant on univariate analysis but not after controlling for potential confounders using the propensity score. Future studies should evaluate the addition of chemotherapy and biologic agents for the treatment of EHC.

  20. Extrahepatic Bile Duct Cancers: Surgery Alone Versus Surgery Plus Postoperative Radiation Therapy

    International Nuclear Information System (INIS)

    Gwak, Hee Keun; Kim, Woo Chul; Kim, Hun Jung; Park, Jeong Hoon

    2010-01-01

    Purpose: The goal of this study was to determine the role of radiotherapy after curative-intent surgery in the management of extrahepatic bile duct (EHBD) cancers. Methods and Materials: From 1997 through 2005, 78 patients with EHBD cancer were surgically staged. These patients were stratified by the absence of adjuvant radiation (n = 47, group I) versus radiation (n = 31, group II) after resection. Pathology examination showed 27 cases in group I and 20 cases in group II had microscopically positive resection margins. The patients in group II received 45 to 54 Gy of external beam radiotherapy. The primary endpoints of this study were overall survival, disease-free survival, and prognostic factors. Results: There were no differences between the 5-year overall survival rates for the two groups (11.6% in group I vs. 21% in group II). However, the patients with microscopically positive resection margins who received adjuvant radiation therapy had higher median disease-free survival rates than those who underwent surgery alone (21 months vs. 10 months, respectively, p = 0.042). Decreasing local failure was found in patients who received postoperative radiotherapy (61.7% in group I and 35.6% in group II, p = 0.02). Outcomes of the patients with a positive resection margin and lymph node metastasis who received postoperative radiation therapy were doubled compared to those of patients without adjuvant radiotherapy. Resection margin status, lymph node metastasis, and pathology differentiation were significant prognostic factors in disease-free survival. Conclusions: Adjuvant radiotherapy might be useful in patients with EHBD cancer, especially for those patients with microscopic residual tumors and positive lymph nodes after resection for increasing local control.

  1. Fetal chromosome abnormalities and congenital malformations: an ...

    African Journals Online (AJOL)

    The results also showed that Multiple congenital anomalies (MCA) represented among 42.2%, congenital malformation of CNS represents 26.6%, congenital malformation of the skeletal system 20%, congenital polycystic kidney 8.8% and pyloric stenosis in 2.2%. Among the 21 women with abnormal karyotype of amniotic ...

  2. Genetics Home Reference: critical congenital heart disease

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Critical congenital heart disease Critical congenital heart disease Printable PDF Open All Close All ... for Disease Control and Prevention: Congenital Heart Defects Disease InfoSearch: Congenital Heart Defects KidsHealth from Nemours Lucile Packard Children's ...

  3. The Transjugular Intrahepatic Portosystemic Shunt in the Treatment of Portal Hypertension: Current Status

    Directory of Open Access Journals (Sweden)

    Gilles Pomier-Layrargues

    2012-01-01

    Full Text Available The transjugular intrahepatic portosystemic shunt (TIPS represents a major advance in the treatment of complications of portal hypertension. Technical improvements and increased experience over the past 24 years led to improved clinical results and a better definition of the indications for TIPS. Randomized clinical trials indicate that the TIPS procedure is not a first-line therapy for variceal bleeding, but can be used when medical treatment fails, both in the acute situation or to prevent variceal rebleeding. The role of TIPS to treat refractory ascites is probably more justified to improve the quality of life rather than to improve survival, except for patients with preserved liver function. It can be helpful for hepatic hydrothorax and can reverse hepatorenal syndrome in selected cases. It is a good treatment for Budd Chiari syndrome uncontrollable by medical treatment. Careful selection of patients is mandatory before TIPS, and clinical followup is essential to detect and treat complications that may result from TIPS stenosis (which can be prevented by using covered stents and chronic encephalopathy (which may in severe cases justify reduction or occlusion of the shunt. A multidisciplinary approach, including the resources for liver transplantation, is always required to treat these patients.

  4. Transjugular intrahepatic portosystemic shunt for severe jaundice in patients with acute Budd-Chiari syndrome.

    Science.gov (United States)

    He, Fu-Liang; Wang, Lei; Zhao, Hong-Wei; Fan, Zhen-Hua; Zhao, Meng-Fei; Dai, Shan; Yue, Zhen-Dong; Liu, Fu-Quan

    2015-02-28

    To evaluate the feasibility of transjugular intrahepatic portosystemic shunt (TIPS) for severe jaundice secondary to acute Budd-Chiari syndrome (BCS). From February 2009 to March 2013, 37 patients with severe jaundice secondary to acute BCS were treated. Sixteen patients without hepatic venule, hepatic veins (HV) obstruction underwent percutaneous angioplasty of the inferior vena cava (IVC) and/or HVs. Twenty-one patients with HV occlusion underwent TIPS. Serum bilirubin, liver function, demographic data and operative data of the two groups of patients were analyzed. Twenty-one patients underwent TIPS and the technical success rate was 100%, with no technical complications. Sixteen patients underwent recanalization of the IVC and/or HVs and the technical success rate was 100%. The mean procedure time for TIPS was 84.0±12.11 min and angioplasty was 44.11±5.12 min (Pjaundice in either group. Severe jaundice is not a contraindication for TIPS in patients with acute BCS and TIPS is appropriate for severe jaundice due to BCS.

  5. Basic anatomic study of transjugular intrahepatic portosystemic shunt by direct transcaval approach

    International Nuclear Information System (INIS)

    Wu Xia; Xu Ke

    2007-01-01

    Objective: To investigate the indications and related anatomic foundation of transjugular intrahepatic portosystemic shunt (TIPS) through direct transcaval approach, and to evaluate the safety, feasibility and clinical significance. Methods: Sixty four patients diagnosed as hepatocirrhosis clinically were involved, including the function of liver Child B (n=40), Child C (n=24). After 2 phrase of hepatic CT enhancement scanning and postprocessing through multiple planner reconstruction (MPR) and curve planner reconstruction (CPR), the data were conjugated statistically by ANOVA. Results: The length of the intrahepatic segment of the inferior cava in Child B is longer than that in Child C (P<0.05). Referring the points of hepatic vein entrance to vena cava as A1, 2 cm away from right hepatic vein as A2, the crotch of portal vein as B1, 2 cm away from right portal vein as B2. The length of A1B1 is shorter than that of A2B1(P<0.05). The angle between A1B2 and right portal vein is smaller than that of A2B2 and right portal vein (P<0.05). Conclusion: Transcaval TIPS creation is safe and feasible, providing the direct transcaval approach as a favorable fluent patency way and less influence on hemodynamics in comparison with traditional TIPS. (authors)

  6. Transjugular intrahepatic portosystemic shunt for the treatment of Budd-Chiari syndrome

    International Nuclear Information System (INIS)

    Han Guohong; He Chuangye; Yin Zhanxin; Meng Xiangjie; Wang Jianhong; Zhang Hongbo; Zhou Xinming; Wu Kaichun; Ding Jie; Fan Daiming

    2008-01-01

    Objective: To evaluate the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) for Budd-Chiari syndrome (BCS). Methods: 14 patients with clinical findings of progressive liver function abnormality and severe complication of portal hypertension (upper gastrointestinal bleeding and refractory ascites)were diagnosed as BCS. Based on imaging manifestations, these patients were classified as follows: obstruction of inferior vena cava (1 case); obstruction of hepatic veins (5 cases); combined obstruction (obstruction of hepatic veins and inferior vena cava)(8 cases). During the procedure, different puncture points were selected for fulfilling the real condition; including 7 cases from hepatic vein to portal vein; 4 cases from inferior vena cava to hepatic vein, 4 cases from the right hepatic vein to portal vein. After the operation, the follow-up evaluation of blood flow in the shunt was performed. Results: All of these patients were successfully performed with TIPS, with average pressure of portal vein decreasing from (4.9 ± 1.4) KPa to(3.2 ± 1.5) KPa. After 5 to 64 mouths follow-up, the recurrent upper gastrointestinal bleeding occurred in two patients due to stent stenosis and were well controlled through balloon angioplasty. Conclusions: TIPS is an effective treatment for BCS with progressive liver dysfunction or severe portal hypertension with upper gastrointestinal bleeding and refractory ascites. In addition, it also contributes to the treatment of the recurrent or severe portal hypertension after the inferior vena cava or hepatic vein angioplasty. (authors)

  7. Mid-term effect of direct intrahepatic portosystemic shunt for the treatment of portal hypertension

    International Nuclear Information System (INIS)

    Luo Jianjun; Yan Zhiping; Wang Jianhua; Liu Qingxin; Qu Xudong

    2009-01-01

    Objective: To retrospectively analyze the mid-term clinical results of direct intrahepatic portosystemic shunt (DIPS) in treating patients with portal hypertension. Methods: DIPS were created in 23 patients with portal hypertension. Both preoperative and postoperative portal systemic pressure gradient (PPG), liver function and clinical symptoms were recorded and compared. Shunt patency was checked by color Doppler ultrasonography and the data were statistically analyzed by Kaplan-Meier method. Results DIPS creation was successfully accomplished in all 23 patients. No serious complications occurred after DIPS except for hemorrhagic ascites (n = 1) and mild hepatic encephalopathy (n = 3). Mean PPG significantly decreased from preoperative (32.6 ± 5.3) mmHg with a range of (23 - 43) mmHg to postoperative (10.1 ± 2.7) mmHg with a range of (5-14) mmHg (P < 0.001). After the procedure, the albumin level also markedly decreased while the bilirubin level distinctly increased. Obvious improvement of the clinical symptoms was observed. The cumulated primary patency rate of the shunt one and two years after treatment was 77.4% and 50.2% respectively. Conclusion: The mid-term clinical results indicate that DIPS is an effective and safe procedure for treating patients with portal hypertension. (authors)

  8. Transjugular Intrahepatic Portosystemic Shunt Placement During Pregnancy: A Case Series of Five Patients

    International Nuclear Information System (INIS)

    Ingraham, Christopher R.; Padia, Siddharth A.; Johnson, Guy E.; Easterling, Thomas R.; Liou, Iris W.; Kanal, Kalpana M.; Valji, Karim

    2015-01-01

    Background and AimsComplications of portal hypertension, such as variceal hemorrhage and ascites, are associated with significant increases in both mortality and complications during pregnancy. Transjugular intrahepatic portosystemic shunt (TIPS) is a well-established procedure for treating portal hypertension, but the safety of TIPS during pregnancy is largely unknown. In this series, we review five patients who underwent TIPS placement while pregnant and describe their clinical outcomes.MethodsFive pregnant patients with cirrhosis and portal hypertension underwent elective TIPS for complications of portal hypertension (four for secondary prevention of variceal bleeding and one for refractory ascites). Outcomes measured were recurrent bleeding episodes or need for further paracenteses during pregnancy, estimated radiation dose to the fetus and gestational age at delivery. All patients were followed after delivery to evaluate technical and clinical success of the procedure.ResultsAll five patients survived pregnancy and went on to deliver successfully. When TIPS was performed for secondary prevention of variceal bleeding (n = 4), no patients demonstrated variceal bleeding after TIPS placement. When TIPS was performed for refractory ascites (n = 1), no further paracenteses were required. All patients delivered successfully, albeit prematurely. Average radiation dose estimated to the fetus was 16.3 mGy.ConclusionsThis series suggests that TIPS can be performed in selective pregnant patients with portal hypertension, with little added risk to the mother or fetus

  9. Evaluation of fluency stent-grafts in transjugular intrahepatic portosystemic shunts

    International Nuclear Information System (INIS)

    Zhao Jianbo; Li Yanhao; Chen Yong; He Xiaofeng; Zeng Qingle; Mei Quelin; Lu Wei

    2009-01-01

    Objective: To evaluate the efficacy of Fluency stent-graft (Bard Corp) in transjugular intrahepatic portosystemic shunt (TIPS). Methods: The clinical data of 21 consecutive patients treated by TIPS using Fluency stent-grafts were retrospectively reviewed. All of them were recurrent variceal bleeding secondary to portal vein hypertension, 1 was bleeding secondary to primary hepatic carcinoma with port vein thrombus, and 1 was Budd-Chiari syndrome. They were followed-up after (10.1±4.6) months (2.0 to 24.0 months). Stent-grafts patancy, portal vein pressure and liver function were recorded and compared. Results: Twenty-five stent-grafts were successfully implanted in 21 patients, 23 stent grafts were 8 mm 2 were 10 mm in diameter. The covered length of the stents varied from 6 to 8 cm. The bleeding was stopped and the portal vein pressure decreased significantly from (25.4±3.5) mm Hg to (15.4±2.8) mm Hg (t= 12.495, P 0.05). Conclusion: The Fluency stent-grafts could increase the patency of the TIPS, but its efficacy on the long-term effect and hepatic encephalopathy need further investigation. (authors)

  10. Transjugular Intrahepatic Portosystemic Shunt Placement in Patients with Cirrhosis and Concomitant Portal Vein Thrombosis

    International Nuclear Information System (INIS)

    Ha, Thuong G. Van; Hodge, Justin; Funaki, Brian; Lorenz, Jonathan; Rosenblum, Jordan; Straus, Christopher; Leef, Jeff

    2006-01-01

    Purpose. To determine the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) creation in patients with liver cirrhosis complicated by thrombosed portal vein. Methods. This study reviewed 15 cases of TIPS creation in 15 cirrhotic patients with portal vein thrombosis at our institution over an 8-year period. There were 2 women and 13 men with a mean age of 53 years. Indications were refractory ascites, variceal hemorrhage, and refractory pleural effusion. Clinical follow-up was performed in all patients. Results. The technical success rate was 75% (3/4) in patients with chronic portal vein thrombosis associated with cavernomatous transformation and 91% (10/11) in patients with acute thrombosis or partial thrombosis, giving an overall success rate of 87%. Complications included postprocedural encephalopathy and localized hematoma at the access site. In patients with successful shunt placement, the total follow-up time was 223 months. The 30-day mortality rate was 13%. Two patients underwent liver transplantation at 35 days and 7 months, respectively, after TIPS insertion. One patient had an occluded shunt at 4 months with an unsuccessful revision. The remaining patients had functioning shunts at follow-up. Conclusion. TIPS creation in thrombosed portal vein is possible and might be a treatment option in certain patients

  11. Transjugular Intrahepatic Portosystemic Shunt: Histologic and Immunohistochemical Study of Autopsy Cases

    International Nuclear Information System (INIS)

    Terayama, Noboru; Matsui, Osamu; Kadoya, Masumi; Yoshikawa, Jun; Gabata, Toshifumi; Miyayama, Shiro; Takashima, Tsutomu; Kobayashi, Kenichi; Nakanishi, Isao; Nakanuma, Yasuni

    1997-01-01

    Purpose: To assess the histologic findings associated with stenosed and occluded transjugular intrahepatic portosystemic shunt (TIPS) tracts. Methods: Four TIPS tracts within three autopsy livers were histologically studied for vascular components by routine staining and immunohistochemical staining. TIPS had been performed for bleeding from esophageal varices in patients with cirrhosis of the liver. Results: Two TIPS, examined on days 4 and 53, showed occlusion by fibrin thrombus. In the former, no endothelial cells were detected, but coagulative necrosis of hepatocytes was found in the surrounding liver. In the latter, bile pigments were seen on the luminal surface. In the two other TIPS without tract occlusion, examined on days 49 and 293, a layer of endothelial cells, proliferation of smooth muscle cells, and deposition of an extracellular matrix such as collagen were confirmed. In the tract examined on day 293, there was protrusion of hepatocytes into the lumen through the stent wires. Conclusion: Short- and midterm TIPS occlusions were caused by thrombus forming after necrosis of hepatocytes and bile leakage, respectively. Long-term TIPS stenosis was associated with a combination of pseudointimal hyperplasia and ingrowth of hepatocytes

  12. Transjugular Intrahepatic Portosystemic Shunt Placement During Pregnancy: A Case Series of Five Patients

    Energy Technology Data Exchange (ETDEWEB)

    Ingraham, Christopher R., E-mail: cringra@uw.edu; Padia, Siddharth A., E-mail: spadia@uw.edu; Johnson, Guy E., E-mail: gej@uw.edu [University of Washington, Department of Interventional Radiology (United States); Easterling, Thomas R., E-mail: easter@uw.edu [University of Washington, Department of Obstetrics and Gynecology (United States); Liou, Iris W., E-mail: irisl@medicine.washington.edu [University of Washington, Department of Medicine (United States); Kanal, Kalpana M., E-mail: kkanal@uw.edu [University of Washington, Physics Section, Department of Radiology (United States); Valji, Karim, E-mail: kvalji@uw.edu [University of Washington, Department of Interventional Radiology (United States)

    2015-10-15

    Background and AimsComplications of portal hypertension, such as variceal hemorrhage and ascites, are associated with significant increases in both mortality and complications during pregnancy. Transjugular intrahepatic portosystemic shunt (TIPS) is a well-established procedure for treating portal hypertension, but the safety of TIPS during pregnancy is largely unknown. In this series, we review five patients who underwent TIPS placement while pregnant and describe their clinical outcomes.MethodsFive pregnant patients with cirrhosis and portal hypertension underwent elective TIPS for complications of portal hypertension (four for secondary prevention of variceal bleeding and one for refractory ascites). Outcomes measured were recurrent bleeding episodes or need for further paracenteses during pregnancy, estimated radiation dose to the fetus and gestational age at delivery. All patients were followed after delivery to evaluate technical and clinical success of the procedure.ResultsAll five patients survived pregnancy and went on to deliver successfully. When TIPS was performed for secondary prevention of variceal bleeding (n = 4), no patients demonstrated variceal bleeding after TIPS placement. When TIPS was performed for refractory ascites (n = 1), no further paracenteses were required. All patients delivered successfully, albeit prematurely. Average radiation dose estimated to the fetus was 16.3 mGy.ConclusionsThis series suggests that TIPS can be performed in selective pregnant patients with portal hypertension, with little added risk to the mother or fetus.

  13. Adopting wedge hepatic venography with CO2 during transjugular intrahepatic portosystemic shunt procedures

    International Nuclear Information System (INIS)

    Zhang Linpeng; Chen Songtao; Shi Xiulan

    2012-01-01

    Objective: To renovate angiography in identifying portal vein anatomy during transjugular intrahepatic portosystemic shunt (TIPS) procedures, saving the time of TIPS procedures, decreasing the risk of the complications of the post-procedure. Methods: The difference between the Wedge hepatic venography with Carbon Dioxide in 6 cases and Inferior Mesenteric artery angiography in 7 cases during TIPS procedures were compared in the identification of portal vein anatomy. The quality of images, their effects on the procedures, the complications and the recovery post-procedure were evaluated. Results: Using CO 2 , the portal veins were opacified in all 6 cases. TIPS procedures succeeded in all cases except 1 case because of poor coagulation function. Using Inferior Mesenteric artery angiography, the portal veins were opacified in all 7 cases. TIPS procedure succeeded in all cases except 1 case because of chronic portal occlusion. Puncture-site hematoma occurred in 1 case after TIPS procedure. Conclusion: Wedge hepatic venography with Carbon Dioxide is superior, safer and more convenient than Inferior Mesenteric Artery angiography in identifying portal vein anatomy during TIPS. (authors)

  14. Pathophysiological appraisal of a rat model of total hepatic ischemia with an extracorporeal portosystemic shunt.

    Science.gov (United States)

    Suzuki, S; Nakamura, S; Sakaguchi, T; Mitsuoka, H; Tsuchiya, Y; Kojima, Y; Konno, H; Baba, S

    1998-11-01

    Animal models of total hepatic ischemia (THI) and reperfusion injury are restricted by concomitant splanchnic congestion. This study was performed to determine the requirement suitable for an extracorporeal portosystemic shunt (PSS) to maintain the intestinal integrity in a rat model of THI. Using a polyethylene tube (0.86 or 1 mm i.d.), PSS was placed between the mesenteric and jugular veins. Comparison was done between THI models with or without PSS and a partial ischemia model with hepatectomy of the nonischemic lobes. Well-tolerated hepatic ischemic period, portal pressure after 10 min of hepatic ischemia, portal endotoxin levels at 1 h after reperfusion, histological features of the small bowel just before reperfusion, and local jejunal and ileal blood hemoglobin oxygen saturation index (ISO2) were compared among the models. Animals without PSS poorly tolerated 30 min of THI. Animals receiving THI with PSS or partial hepatic ischemia tolerated a longer ischemic period (60 min) with a significantly higher small bowel ISO2, lower portal pressure and endotoxin levels (P tube as well as partial hepatic ischemia were significantly lower than those after THI with PSS using a 0.86-mm i.d. tube. THI with PSS using a 1-mm i.d. tube was strikingly similar to partial hepatic ischemia in the pathophysiological profile during hepatic ischemia. PSS with a tube 1 mm or more in inner diameter offers pathophysiological advantages in experiments on THI and reperfusion. Copyright 1998 Academic Press.

  15. Pictorial essay: trans-jugular intra-hepatic porto-systemic shunt (TIPS)

    International Nuclear Information System (INIS)

    Rao Anuradha, T.N.; Rastogi, H.; Pandey, U.C.

    2001-01-01

    TIPS is an interventional radiologic method of creating a portosystemic shunt by percutaneous means. It is a relatively new weapon in the armamentarium of the management of portal hypertension with variceal bleeding or intractable ascites. Using needles, angioplasty balloon catheters and expandable metallic stents, a shunt is established directly inside the liver parenchyma connecting a large hepatic vein with a main portal vein branch. Depending on the diameter of the expandable stent used, the created TIPS diverts various amounts of portal blood into the systemic circulation and results in significant portal decompression, cessation of hemorrhage from esophageal varices and resolution of ascites. TIPS is not only an effective and safe alternative to shunt surgery but is life saving in cases of variceal hemorrhage. The use of TIPS seems to be a valid alternative to surgery and has several advantages over either esophageal transection or surgical shunts. TIPS avoids a general anesthetic, laparotomy, and disruption of the hepatic venous system that may adversely affect subsequent hepatic transplantation. TIPS has been performed on patients without interfering with successful transplantation

  16. Usefulness and pitfalls of MAA SPECT/CT in identifying digestive extrahepatic uptake when planning liver radioembolization

    International Nuclear Information System (INIS)

    Lenoir, Laurence; Garin, Etienne; Edeline, Julien; Rolland, Yann; Pracht, Marc; Raoul, Jean-Luc; Ardisson, Valerie; Bourguet, Patrick; Clement, Bruno; Boucher, Eveline

    2012-01-01

    Identifying gastroduodenal uptake of 99m Tc-macroaggregated albumin (MAA), which is associated with an increased risk of ulcer disease, is a crucial part of the therapeutic management of patients undergoing radioembolization for liver tumours. Given this context, the use of MAA single photon emission computed tomography (SPECT)/CT may be essential, but the procedure has still not been thoroughly evaluated. The aim of this retrospective study was to determine the effectiveness of MAA SPECT/CT in identifying digestive extrahepatic uptake, while determining potential diagnostic pitfalls. Overall, 139 MAA SPECT/CT scans were performed on 103 patients with different hepatic tumour types. Patients were followed up for at least 6 months according to standard requirements. Digestive, or digestive-like, uptake other than free pertechnetate was identified in 5.7% of cases using planar imaging and in 36.6% of cases using SPECT/CT. Uptake sites identified by SPECT/CT included the gastroduodenal region (3.6%), gall bladder (12.2%), portal vein thrombosis (6.5%), hepatic artery (6.5%), coil embolization site (2.1%) as well as falciform artery (5.0%). For 2.1% of explorations, a coregistration error between SPECT and CT imaging could have led to a false diagnosis by erroneously attributing an uptake site to the stomach or gall bladder, when the uptake actually occurred in the liver. SPECT/CT is more efficacious than planar imaging in identifying digestive extrahepatic uptake sites, with extrahepatic uptake observed in one third of scans using the former procedure. However, more than half of the uptake sites in our study were vascular in nature, without therapeutic implications. The risk of coregistration errors must also be kept in mind. (orig.)

  17. HCV-RNA quantification in liver bioptic samples and extrahepatic compartments, using the abbott RealTime HCV assay.

    Science.gov (United States)

    Antonucci, FrancescoPaolo; Cento, Valeria; Sorbo, Maria Chiara; Manuelli, Matteo Ciancio; Lenci, Ilaria; Sforza, Daniele; Di Carlo, Domenico; Milana, Martina; Manzia, Tommaso Maria; Angelico, Mario; Tisone, Giuseppe; Perno, Carlo Federico; Ceccherini-Silberstein, Francesca

    2017-08-01

    We evaluated the performance of a rapid method to quantify HCV-RNA in the hepatic and extrahepatic compartments, by using for the first time the Abbott RealTime HCV-assay. Non-tumoral (NT), tumoral (TT) liver samples, lymph nodes and ascitic fluid from patients undergoing orthotopic-liver-transplantation (N=18) or liver resection (N=4) were used for the HCV-RNA quantification; 5/22 patients were tested after or during direct acting antivirals (DAA) treatment. Total RNA and DNA quantification from tissue-biopsies allowed normalization of HCV-RNA concentrations in IU/μg of total RNA and IU/10 6 liver-cells, respectively. HCV-RNA was successfully quantified with high reliability in liver biopsies, lymph nodes and ascitic fluid samples. Among the 17 untreated patients, a positive and significant HCV-RNA correlation between serum and NT liver-samples was observed (Pearson: rho=0.544, p=0.024). Three DAA-treated patients were HCV-RNA "undetectable" in serum, but still "detectable" in all tested liver-tissues. Differently, only one DAA-treated patient, tested after sustained-virological-response, showed HCV-RNA "undetectability" in liver-tissue. HCV-RNA was successfully quantified with high reliability in liver bioptic samples and extrahepatic compartments, even when HCV-RNA was "undetectable" in serum. Abbott RealTime HCV-assay is a good diagnostic tool for HCV quantification in intra- and extra-hepatic compartments, whenever a bioptic sample is available. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. Usefulness and pitfalls of MAA SPECT/CT in identifying digestive extrahepatic uptake when planning liver radioembolization

    Energy Technology Data Exchange (ETDEWEB)

    Lenoir, Laurence; Garin, Etienne [Comprehensive Cancer Institute Eugene Marquis, Department of Nuclear Medicine, CS 44229, Rennes (France); University of Rennes 1, Rennes (France); Liver Metabolisms and Cancer, INSERM, U-991, Rennes (France); Edeline, Julien [University of Rennes 1, Rennes (France); Comprehensive Cancer Institute Eugene Marquis, Department of Medical Oncology, CS 44229, Rennes (France); Rolland, Yann [Comprehensive Cancer Institute Eugene Marquis, Department of Medical Imaging, CS 44229, Rennes (France); Pracht, Marc [Comprehensive Cancer Institute Eugene Marquis, Department of Medical Oncology, CS 44229, Rennes (France); Raoul, Jean-Luc [Comprehensive Cancer Institute Paoli Calmette, Department of Medical Oncology, Marseille (France); Ardisson, Valerie [Comprehensive Cancer Institute Eugene Marquis, Department of Nuclear Medicine, CS 44229, Rennes (France); Bourguet, Patrick [Comprehensive Cancer Institute Eugene Marquis, Department of Nuclear Medicine, CS 44229, Rennes (France); University of Rennes 1, Rennes (France); Clement, Bruno [Liver Metabolisms and Cancer, INSERM, U-991, Rennes (France); Boucher, Eveline [Liver Metabolisms and Cancer, INSERM, U-991, Rennes (France); Comprehensive Cancer Institute Eugene Marquis, Department of Medical Oncology, CS 44229, Rennes (France)

    2012-05-15

    Identifying gastroduodenal uptake of {sup 99m}Tc-macroaggregated albumin (MAA), which is associated with an increased risk of ulcer disease, is a crucial part of the therapeutic management of patients undergoing radioembolization for liver tumours. Given this context, the use of MAA single photon emission computed tomography (SPECT)/CT may be essential, but the procedure has still not been thoroughly evaluated. The aim of this retrospective study was to determine the effectiveness of MAA SPECT/CT in identifying digestive extrahepatic uptake, while determining potential diagnostic pitfalls. Overall, 139 MAA SPECT/CT scans were performed on 103 patients with different hepatic tumour types. Patients were followed up for at least 6 months according to standard requirements. Digestive, or digestive-like, uptake other than free pertechnetate was identified in 5.7% of cases using planar imaging and in 36.6% of cases using SPECT/CT. Uptake sites identified by SPECT/CT included the gastroduodenal region (3.6%), gall bladder (12.2%), portal vein thrombosis (6.5%), hepatic artery (6.5%), coil embolization site (2.1%) as well as falciform artery (5.0%). For 2.1% of explorations, a coregistration error between SPECT and CT imaging could have led to a false diagnosis by erroneously attributing an uptake site to the stomach or gall bladder, when the uptake actually occurred in the liver. SPECT/CT is more efficacious than planar imaging in identifying digestive extrahepatic uptake sites, with extrahepatic uptake observed in one third of scans using the former procedure. However, more than half of the uptake sites in our study were vascular in nature, without therapeutic implications. The risk of coregistration errors must also be kept in mind. (orig.)

  19. Survival Outcomes in Resected Extrahepatic Cholangiocarcinoma: Effect of Adjuvant Radiotherapy in a Surveillance, Epidemiology, and End Results Analysis

    International Nuclear Information System (INIS)

    Vern-Gross, Tamara Z.; Shivnani, Anand T.; Chen, Ke; Lee, Christopher M.; Tward, Jonathan D.; MacDonald, O. Kenneth; Crane, Christopher H.; Talamonti, Mark S.; Munoz, Louis L.; Small, William

    2011-01-01

    Purpose: The benefit of adjuvant radiotherapy (RT) after surgical resection for extrahepatic cholangiocarcinoma has not been clearly established. We analyzed survival outcomes of patients with resected extrahepatic cholangiocarcinoma and examined the effect of adjuvant RT. Methods and Materials: Data were obtained from the Surveillance, Epidemiology, and End Results (SEER) program between 1973 and 2003. The primary endpoint was the overall survival time. Cox regression analysis was used to perform univariate and multivariate analyses of the following clinical variables: age, year of diagnosis, histologic grade, localized (Stage T1-T2) vs. regional (Stage T3 or greater and/or node positive) stage, gender, race, and the use of adjuvant RT after surgical resection. Results: The records for 2,332 patients were obtained. Patients with previous malignancy, distant disease, incomplete or conflicting records, atypical histologic features, and those treated with preoperative/intraoperative RT were excluded. Of the remaining 1,491 patients eligible for analysis, 473 (32%) had undergone adjuvant RT. After a median follow-up of 27 months (among surviving patients), the median overall survival time for the entire cohort was 20 months. Patients with localized and regional disease had a median survival time of 33 and 18 months, respectively (p < .001). The addition of adjuvant RT was not associated with an improvement in overall or cause-specific survival for patients with local or regional disease. Conclusion: Patients with localized disease had significantly better overall survival than those with regional disease. Adjuvant RT was not associated with an improvement in long-term overall survival in patients with resected extrahepatic bile duct cancer. Key data, including margin status and the use of combined chemotherapy, was not available through the SEER database.

  20. General Concepts in Adult Congenital Heart Disease.

    Science.gov (United States)

    Mutluer, Ferit Onur; Çeliker, Alpay

    2018-01-20

    Congenital heart disease in adults (adult congenital heart disease) is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face.

  1. General Concepts in Adult Congenital Heart Disease

    Directory of Open Access Journals (Sweden)

    Ferit Onur Mutluer

    2018-02-01

    Full Text Available Congenital heart disease in adults (adult congenital heart disease is a growing burden for healthcare systems. While infant mortality due to congenital heart disease in the last four decades decreased by almost 3-fold, adult congenital heart disease prevalence increased by more than 2-fold in United States. Adult congenital heart disease prevalence is expected to increase steadily until 2050 in projections. Adult congenital heart disease is a multifaceted problem with many dimensions. This manuscript aims to provide an overview of the common adult congenital heart diseases and summarize important points in management of these diseases with possible problems and complications that the patients and the physicians face

  2. Treatment of extrahepatic portal hypertension following a whipple procedure with a Rex shunt: report of a case.

    Science.gov (United States)

    Reichman, Trevor W; Anthony, Tiffany; Testa, Giuliano

    2011-02-01

    The Rex shunt is a mesenteric vein to left portal vein decompressive shunt used for the treatment of portal vein thrombosis and portal hypertension. Its use has been reported primarily in the pediatric population where portal vein thrombosis occurs with some frequency. The shunt is thought to represent a more physiologic shunt, since it restores hepatopedal blood flow through the liver. This report describes the use of this shunt in an adult who had frequent gastrointestinal bleeding secondary to extrahepatic portal vein thrombosis, which occurred as a complication after a pancreaticoduodenectomy.

  3. Endovascular treatment for extrahepatic portal vein bifurcation stenosis after a Whipple procedure using the kissing stents technique.

    Science.gov (United States)

    Zhang, Wen-guang; Liu, Dong-mei; Li, Zhen; Wang, Yan-Li; Ding, Peng-xu; Zhou, Peng-li; Wang, Zhong-gao; Han, Xin-wei

    2014-01-01

    A 57-year-old man presented with a rare extrahepatic portal vein bifurcation scar stenosis involving the proximal splenic vein and superior mesenteric vein after a Whipple procedure. He was treated with endovascular coil embolization for the gastroesophageal varices and kissing stents for the portal vein bifurcation stenosis. This case illustrates a rarely seen complication after the Whipple procedure and a novel management strategy that can be considered in the management of this complex disease. Copyright © 2014 Elsevier Inc. All rights reserved.

  4. Profiles in congenital heart disease

    International Nuclear Information System (INIS)

    Freed, M.D.; Keane, J.F.

    1986-01-01

    Pediatric cardiology has made great strides in the diagnosis, management, and correction of complex congenital malformations in the past two decades. The foundation of these advances is a more precise understanding of the physiology and anatomy of complex lesions that has been obtained from cardiac catheterization and angiography. The techniques for catheterization of infants and children have been discussed in another paper. This chapter focuses on brief profiles of some of the more important congenital abnormalities. The incidence cited in the discussion of each abnormality pertains to a population comprises of children and adults referred to The Children's Hospital Medical Center and Peter Bent Brigham Hospital, respectively, for evaluation of congenital heart disease

  5. Congenital pyriform aperture stenosis

    International Nuclear Information System (INIS)

    Osovsky, Micky; Aizer-Danon, Anat; Horev, Gadi; Sirota, Lea

    2007-01-01

    Nasal airway obstruction is a potentially life-threatening condition in the newborn. Neonates are obligatory nasal breathers. The pyriform aperture is the narrowest, most anterior bony portion of the nasal airway, and a decrease in its cross-sectional area will significantly increase nasal airway resistance. Congenital nasal pyriform aperture stenosis (CNPAS) is a rare, unusual form of nasal obstruction. It should be considered in the differential diagnosis of any neonate or infant with signs and symptoms of upper airway compromise. It is important to differentiate this level of obstruction from the more common posterior choanal stenosis or atresia. CNPAS presents with symptoms of nasal airway obstruction, which are often characterized by episodic apnea and cyclical cyanosis. (orig.)

  6. Characterizing congenital amusia.

    Science.gov (United States)

    Stewart, Lauren

    2011-04-01

    The ability to make sense of the music in our environment involves sophisticated cognitive mechanisms that, for most people, are acquired effortlessly and in early life. A special population of individuals, with a disorder termed congenital amusia, report lifelong difficulties in this regard. Exploring the nature of this developmental disorder provides a window onto the cognitive architecture of typical musical processing, as well as allowing a study of the relationship between processing of music and other domains, such as language. The present article considers findings concerning pitch discrimination, pitch memory, contour processing, experiential aspects of music listening in amusia, and emerging evidence concerning the neurobiology of the disorder. A simplified model of melodic processing is outlined, and possible loci of the cognitive deficit are discussed.

  7. Neurobiology of Congenital Amusia.

    Science.gov (United States)

    Peretz, Isabelle

    2016-11-01

    The past decade of research has provided compelling evidence that musical engagement is a fundamental human trait, and its biological basis is increasingly scrutinized. In this endeavor, the detailed study of individuals who have musical deficiencies is instructive because of likely neurogenetic underpinnings. Such individuals have 'congenital amusia', an umbrella term for lifelong musical disabilities that cannot be attributed to intellectual disability, lack of exposure, or brain damage after birth. Key points are reviewed here that have emerged during recent years regarding the neurobiology of the disorder, focusing on the importance of recurrent processing between the right inferior frontal cortex and the auditory cortex for conscious monitoring of musical pitch, and how this relates to developmental cognitive disorders in general. Copyright © 2016 Elsevier Ltd. All rights reserved.

  8. Congenital sensorineural hearing loss

    International Nuclear Information System (INIS)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-01-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems

  9. Congenital sensorineural hearing loss

    Energy Technology Data Exchange (ETDEWEB)

    Mafee, M.F.; Selis, J.E.; Yannias, D.A.; Valvassori, G.E.; Pruzansky, S.; Applebaum, E.L.; Capek, V.

    1984-02-01

    The ears of 47 selected patients with congenital sensorineural hearing loss were examined with complex-motion tomography. The patients were divided into 3 general categories: those with a recognized syndrome, those with sensorineural hearing loss unrelated to any known syndrome, and those with microtia. A great variety of inner ear anomalies was detected, but rarely were these characteristic of a particular clinical entity. The most common finding was the Mondini malformation or one of its variants. Isolated dysplasia of the internal auditory canal or the vestibular aqueduct may be responsible for sensorineural hearing loss in some patients. Patients with microtia may also have severe inner ear abnormalities despite the fact that the outer and inner ears develop embryologically from completely separate systems.

  10. A case of pancreatic arteriovenous malformation with portal hypertension: treatment with transjuguIar intrahepatic portosystemic shunt

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Seong Hoon; Kim, Young Whan; Kim, Yong Joo [School of Medicine, Kyungpook National Univ., Daegu (Korea, Republic of)

    2004-03-01

    Arteriovenous malformation of the pancreas is a rare disease, and it is manifested by gastrointestinal bleeding and/or portal hypertension. Surgery is definitely the treatment of choice at the early stage of the disease, and a transcatheter embolization is an alternative treatment for the control of bleeding and if the lesion is surgically inaccessible. We describe a 62-year-old man who had refractory ascites and esophageal variceal bleeding caused by a pancreatic arteriovenous malformation associated with portal hypertension; this was successfully treated by a transjugular intrahepatic portosystemic shunt.

  11. A case of pancreatic arteriovenous malformation with portal hypertension: treatment with transjuguIar intrahepatic portosystemic shunt

    International Nuclear Information System (INIS)

    Kim, Seong Hoon; Kim, Young Whan; Kim, Yong Joo

    2004-01-01

    Arteriovenous malformation of the pancreas is a rare disease, and it is manifested by gastrointestinal bleeding and/or portal hypertension. Surgery is definitely the treatment of choice at the early stage of the disease, and a transcatheter embolization is an alternative treatment for the control of bleeding and if the lesion is surgically inaccessible. We describe a 62-year-old man who had refractory ascites and esophageal variceal bleeding caused by a pancreatic arteriovenous malformation associated with portal hypertension; this was successfully treated by a transjugular intrahepatic portosystemic shunt

  12. [Clinical value of MRI united-sequences examination in diagnosis and differentiation of morphological sub-type of hilar and extrahepatic big bile duct cholangiocarcinoma].

    Science.gov (United States)

    Yin, Long-Lin; Song, Bin; Guan, Ying; Li, Ying-Chun; Chen, Guang-Wen; Zhao, Li-Ming; Lai, Li

    2014-09-01

    To investigate MRI features and associated histological and pathological changes of hilar and extrahepatic big bile duct cholangiocarcinoma with different morphological sub-types, and its value in differentiating between nodular cholangiocarcinoma (NCC) and intraductal growing cholangiocarcinoma (IDCC). Imaging data of 152 patients with pathologically confirmed hilar and extrahepatic big bile duct cholangiocarcinoma were reviewed, which included 86 periductal infiltrating cholangiocarcinoma (PDCC), 55 NCC, and 11 IDCC. Imaging features of the three morphological sub-types were compared. Each of the subtypes demonstrated its unique imaging features. Significant differences (P big bile duct cholangiocarcinoma. MRI united-sequences examination can accurately describe those imaging features for differentiation diagnosis.

  13. A Study of Technical Approach Methods to Transabdominal Ultrasonography of the Extrahepatic Bile Ducts and of Following Effects from the Scan Training

    International Nuclear Information System (INIS)

    Lee, In Ja; Kang, Dae Hyun; Kim, Bo Young

    2008-01-01

    The purposes of this study are to analyze abnormal dilatation of the extrahepatic bile ducts by using transabdominal ultrasound, to confirm the existence of bile ducts diseases and their interrelationship, and for it to give a new theoretical basis for the technical access to extrahepatic bile ducts, upon which to analyze the ripple effects of the scan training. After teaching technical access process based on the new theory about extrahepatic bile duct to the thirty students who are studying ultrasonography, we allocated three hours per one student (30 mins 6 times) to focus on the training of scanning skill. Training has been performed by one-to-one method. For evaluation, all the students have to perform the scans on (1) confluence of the right and left hepatic ducts (extrahepatic bile ducts and cystic duct), (2) the suprapancreatic bile duct, (3) the intrapancreatic bile duct, (4) intrapapilla Duct, based on the clearly divided concept. The existing training and methods have had low confidency about transabdominal ultrasonography of the extrahepatic bile duct and had limitation with which they could image only the suprapancreatic bile duct. The evaluation after finishing the train based on the new theory, however, all the students (30 students) can access to (1) confluence of the right and left hepatic ducts(extrahepatic bile ducts and cystic duct), (2) the suprapancreatic bile duct objectively. 24 students can access to (3) the intrapancreatic bile duct and only one student can even make an image for (4) the intrapapilla Duct Though the evaluation on extrahepatic bile duct has to be performed with multi-sided method considering intrahepatic cause, bile duct cause and pathophysiological cause, only if we can image the extrahepatic bile duct to ampular of Vater objectively and confidently, we can greatly reduce invasive procedure such as ERCP, which is for the purpose of simple differential diagnosis and painful to the patients. Therefore if we concentrate on

  14. Congenital broncho-oesophageal fistula

    African Journals Online (AJOL)

    1983-04-09

    Apr 9, 1983 ... Rigid bronchoscopy performed under general anaesthesia .... Blackburn WR, Armour)' RA. Congenital esophago-pulmonary fistulas without esophageal atresia: an analysis of 260 fistulas in infants, children and adults.

  15. Congenital heart defect corrective surgeries

    Science.gov (United States)

    ... make it bigger with a patch made of Gore-tex, a man-made (synthetic) material. Another way ... 434. Bhatt AB, Foster E, Kuehl K, et al; American Heart Association Council on Clinical Cardiology. Congenital ...

  16. Congenital Heart Defects (For Parents)

    Science.gov (United States)

    ... to be associated with genetic disorders, such as Down syndrome . But the cause of most congenital heart defects isn't known. While they can't be prevented, many treatments are available for the defects and related health ...

  17. Genetics Home Reference: congenital hyperinsulinism

    Science.gov (United States)

    ... Topic: Hypoglycemia Health Topic: Metabolic Disorders Genetic and Rare Diseases Information Center (1 link) Congenital hyperinsulinism Educational Resources (7 links) Boston Children's Hospital: Hypoglycemia and Low Blood Sugar in Children Cook Children's Hospital (PDF) Disease InfoSearch: ...

  18. CHRNE Mutation and Congenital Myasthenia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2009-01-01

    Full Text Available The CHRNE e1293insG mutation was identified in 14 (60% of 23 North African families with an early onset form of congenital myasthenic syndrome studied at centers in France, Tunisia, Algeria, and UK.

  19. Portal hypertensive enteropathy diagnosed by capsule endoscopy and demonstration of the ileal changes after transjugular intrahepatic portosystemic shunt placement: a case report

    Directory of Open Access Journals (Sweden)

    Carella Alessandra

    2011-03-01

    Full Text Available Abstract Introduction Recent data suggest that mucosal abnormalities can occur even in the duodenum, jejunum, and distal ileum of cirrhosis patients. We present a case of portal hypertensive enteropathy in a cirrhosis patient shown by capsule endoscopy and the effect of transjugular intrahepatic portosystemic shunt on the ileal pictures. Case presentation An 83-year-old Caucasian woman was admitted to our hospital for anemia and a positive fecal occult blood test. An upper gastrointestinal endoscopy revealed small varices without bleeding signs and hypertensive gastropathy. Colonoscopy was negative. To rule out any other cause of bleeding, capsule endoscopy was performed; capsule endoscopy revealed severe hyperemia of the jejunum-ileal mucosa with active bleeding. Because of the persistence of anemia and the frequent blood transfusions, not responding to β-blocker drugs or octreotide infusion, a transjugular intrahepatic portosystemic shunt was performed. Anemia improved quickly after the transjugular intrahepatic portosystemic shunt, and no further blood transfusion was necessary in the follow-up. The patient developed portal encephalopathy two months later and was readmitted to our department. We repeated the capsule endoscopy that showed a significant improvement of the gastric and ileal mucosa without any signs of bleeding. Conclusion Hypertensive enteropathy is a rare condition, but it seems more common with the introduction of capsule endoscopy in clinical practice. This case shows that the jejunum can be a source of bleeding in cirrhosis patients, and this is the first demonstration of its resolution after transjugular intrahepatic portosystemic shunt placement.

  20. The E-cadherin repressor slug and progression of human extrahepatic hilar cholangiocarcinoma

    Directory of Open Access Journals (Sweden)

    Wang Xin-sheng

    2010-07-01

    Full Text Available Abstract Objectives This study explored the expression and function of Slug in human extrahepatic hilar cholangiocarcinoma (EHC to identify its role in tumor progression. Methods The expression of Snail and Slug mRNA in 52 human tissue samples of EHC was investigated. The mRNA of Snail and Slug were quantified using reverse transcriptase-PCR, and correlations with E-cadherin expression and clinicopathological factors were investigated. We then investigated transfection of Slug cDNA in endogenous E-cadherin-positive human EHC FRH0201 cells, selectively induced the loss of E-cadherin protein expression, and then small interfering RNA (siRNA for inhibition of Slug expression in endogenous Slug-positive human EHC QBC939 cells, selectively induced the loss of Slug protein expression. A Boyden chamber transwell assay was used for invasion. Results Slug mRNA was overexpressed in 18 cases (34.6% of EHC compared with adjacent noncancerous tissue. E-Cadherin protein expression determined in the same 52 cases by immunohistochemistry was significantly down-regulated in those cases with Slug mRNA overexpression (P = 0.0001. The tumor and nontumor ratio of Slug mRNA was correlated with nodal metastasis(p = 0.0102, distant metastasis (p = 0.0001and Survival time(p = 0.0443. However, Snail mRNA correlated with neither E-cadherin expression nor tumor invasiveness. By inhibiting Slug expression by RNA interference, we found that reduced Slug levels upregulated E-cadherin and decreased invasion in QBC939 cell. When the QBC939 cells was infected with Slug cDNA,, significant E-cadherin was downregulated and increased invasion in QBC939 cell. Conclusions The results suggested that Slug expression plays an important role in both the regulation of E-cadherin expression and in the acquisition of invasive potential in human EHC. Slug is possibly a potential target for an antitumor therapy blocking the functions of invasion and metastasis in human EHCs.

  1. Congenital and perinatal cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Chun Soo Kim

    2010-01-01

    Full Text Available Cytomegalovirus (CMV is currently the most common agent of congenital infection and the leading infectious cause of brain damage and hearing loss in children. Symptomatic congenital CMV infections usually result from maternal primary infection during early pregnancy. One half of symptomatic infants have cytomegalic inclusion disease (CID, which is characterized by involvement of multiple organs, in particular, the reticuloendothelial and central nervous system (CNS. Moreover, such involvement may or may not include ocular and auditory damage. Approximately 90% of infants with congenital infection are asymptomatic at birth. Preterm infants with perinatal CMV infection can have symptomatic diseases such as pneumonia, hepatitis, and thrombocytopenia. Microcephaly and abnormal neuroradiologic imaging are associated with a poor prognosis. Hearing loss may occur in both symptomatic and asymptomatic infants with congenital infection and may progress through childhood. Congenital infection is defined by the isolation of CMV from infants within the first 3 weeks of life. Ganciclovir therapy can be considered for infants with symptomatic congenital CMV infection involving the CNS. Pregnant women of seronegative state should be counseled on the importance of good hand washing and other control measures to prevent CMV infection. Heat treatment of infected breast milk at 72?#608;for 5 seconds can eliminate CMV completely.

  2. The Features of Extrahepatic Collateral Arteries Related to Hepatic Artery Occlusion and Benefits in the Transarterial Management of Liver Tumors

    International Nuclear Information System (INIS)

    Yang, Lin; Zhang, Xiao Ming; Ren, Yong Jun; Miao, Nan Dong; Huang, Xiao Hua; Dong, Guo Li

    2013-01-01

    Purpose. To investigate the extrahepatic collateral arteries related to hepatic artery occlusion (HAO) and to determine its benefits in the transarterial management of liver tumors. Methods and Findings. Eleven patients (7 hepatocellular carcinomas, 3 liver metastases, and 1 with hemangioma) with HAO confirmed with digital subtraction angiography (DSA) were admitted to our hospital. Of the 11 patients, 7 were men and 4 were women, with an average age of 41.5 ± 15.5 years (range: 29 to 70 years). DSA was performed to evaluate the collateral routes to the liver. In the 11 patients with HAO, DSA showed complete occlusion of the common hepatic artery in 9 patients and the proper hepatic artery (PHA) in 2 patients. Extrahepatic collateral arteries supplying the liver were readily evident. The collateral arteries originated from the superior mesenteric artery (SMA) in 8 patients, from the gastroduodenal artery in 2 patients, and from the left gastric artery (LGA) in 1 patient. Transcatheter treatment was successfully performed via the collateral artery in all patients except the one who had hemangioma. Conclusions. DSA is an effective method for detecting collateral circulation related to HAO and may provide information to guide transcatheter management decisions

  3. The peculiarities of macroscopic status of the upper gastrointestinal tract mucosa in patients with hepatic and extrahepatic portal hypertension

    Directory of Open Access Journals (Sweden)

    Stepanov Yu.M.

    2018-03-01

    Full Text Available Identification of endoscopic signs of concomitant pathology of the upper gastrointestinal tract allows to determine the tactics of managing patients with different forms of portal hypertension (PG. The analysis of macroscopic picture of esophagus, stomach and duodenum mucosa was carried out in 104 patients with portal hypertension (88 patients with hepatic portal hypertension, 46 – with extrahepatic portal hypertension, 16 – without portal hypertension. The frequency of changes in the esophagus, specific for gastroesophageal reflux disease did not differ significantly in the researched groups. The frequency and severity of varicose veins of the esophagus were diagnosed more often in patients with hepatic form of portal hypertension (OR=10.0, p<0.05. The predominance of different pathological changes of the gastric mucosa in patients with extrahepatic form of portal hypertension was revealed. Portal gastropathy in this group was 5.3 times higher than in patients with hepatic hypertension and 21 times higher than in the III group (without portal hypertension (p<0.01. There were no significant differences of endoscopic picture of the duodenum mucosa between the researched groups.

  4. Globus pallidus MR signal abnormalities in children with chronic liver disease and/or porto-systemic shunting

    Energy Technology Data Exchange (ETDEWEB)

    Hanquinet, Sylviane; Anooshiravani, Mehrak; Merlini, Laura [University Hospital of Geneva, Department of Pediatric Radiology, Geneva (Switzerland); Morice, Claire; Cousin, Vladimir; McLin, Valerie A. [University Hospital of Geneva, Swiss Center for Liver Disease in Children, Geneva (Switzerland); Courvoisier, Delphine S. [University Hospital of Geneva, Division of Quality of Care, Geneva (Switzerland)

    2017-10-15

    Detection of subclinical hepatic encephalopathy in children is difficult. We aimed to assess the changes in imaging of the central nervous system in children with chronic liver disease using MR imaging, diffusion, and {sup 1}H -spectroscopy. Forty three children with chronic liver disease and/or porto-systemic shunting (111.4±56.9 months) and 24 controls (72.0±51.8 months) underwent brain MRI/spectroscopy on a 1.5T to examine T1, T2, ADC, Cho/Cr, ml/Cr, Glx/Cr ratio spectroscopy in the globus pallidus. Patients were divided into 3 groups according to the ratios of globus pallidus/putamen T1 signal: isointense (i), hyperintense (h), much more hyperintense (h+). The relationship with clinical and biological data was analyzed. T1 signal intensity and ml/Cr were significantly different between controls and group h+ (p=0.001). ADC did not differ significantly between groups. Age correlated strongly with the presence of a T1 signal ratio (p > 0.001). There was no correlation between imaging findings and biological parameters. In children with chronic liver disease and/or porto-systemic shunting, the presence of a hyperintense T1 signal in the globus pallidus correlated strongly with age. Biological and clinical parameters were not predictive of these changes. MRI may become a useful screening tool for hepatic encephalopathy in children. (orig.)

  5. Globus pallidus MR signal abnormalities in children with chronic liver disease and/or porto-systemic shunting

    International Nuclear Information System (INIS)

    Hanquinet, Sylviane; Anooshiravani, Mehrak; Merlini, Laura; Morice, Claire; Cousin, Vladimir; McLin, Valerie A.; Courvoisier, Delphine S.

    2017-01-01

    Detection of subclinical hepatic encephalopathy in children is difficult. We aimed to assess the changes in imaging of the central nervous system in children with chronic liver disease using MR imaging, diffusion, and "1H -spectroscopy. Forty three children with chronic liver disease and/or porto-systemic shunting (111.4±56.9 months) and 24 controls (72.0±51.8 months) underwent brain MRI/spectroscopy on a 1.5T to examine T1, T2, ADC, Cho/Cr, ml/Cr, Glx/Cr ratio spectroscopy in the globus pallidus. Patients were divided into 3 groups according to the ratios of globus pallidus/putamen T1 signal: isointense (i), hyperintense (h), much more hyperintense (h+). The relationship with clinical and biological data was analyzed. T1 signal intensity and ml/Cr were significantly different between controls and group h+ (p=0.001). ADC did not differ significantly between groups. Age correlated strongly with the presence of a T1 signal ratio (p > 0.001). There was no correlation between imaging findings and biological parameters. In children with chronic liver disease and/or porto-systemic shunting, the presence of a hyperintense T1 signal in the globus pallidus correlated strongly with age. Biological and clinical parameters were not predictive of these changes. MRI may become a useful screening tool for hepatic encephalopathy in children. (orig.)

  6. Transjugular Intrahepatic Portosystemic Shunt for Portal Hypertension in Hepatocellular Carcinoma with Portal Vein Tumor Thrombus.

    Science.gov (United States)

    Qiu, Bin; Li, Kai; Dong, Xiaoqun; Liu, Fu-Quan

    2017-09-01

    In patients with hepatocellular carcinoma (HCC), limited therapeutic options are available for portal hypertension resulted from portal vein tumor thrombus (PVTT). We aimed to determine safety and efficacy of TIPS for treatment of symptomatic portal hypertension in HCC with PVTT. We evaluated clinical characteristics of 95 patients with HCC and PVTT out of 992 patients who underwent TIPS. The primary endpoints included success rate, procedural mortality, serious complications, decrease in portosystemic pressure gradient, and symptom relief. The secondary endpoints included recurrence of portal hypertension, overall survival, adverse events related to treatments for HCC, and quality of life measured by Karnofsky Performance Status Scale (KPS). Success rate of TIPS was 95.8% (91/95), with procedural mortality of 1.1%. Serious complications related to TIPS procedure occurred in 2.1% (2/95) of patients. The symptoms of portal hypertension were well relieved. Variceal bleeding was successfully controlled and terminated in 100% of patients, with a recurrence rate of 39.2% in 12 months. Refractory ascites/hydrothorax was controlled partially or completely in 92.9% of patients during 1 month after TIPS, with a recurrence rate of 17.9% in 12 months. Survival rate at 6, 12, 24, and 36 months was 75.8, 52.7, 26.4, and 3.3%, respectively. No unexpected adverse event related to treatments for HCC was observed. The KPS score was 49 ± 4.5 and 63 ± 4.7 before and 1 month after TIPS, respectively (p portal hypertension in HCC with PVTT.

  7. Combined transjugular intrahepatic portosystemic shunt and other interventions for hepatocellular carcinoma with portal hypertension.

    Science.gov (United States)

    Qiu, Bin; Zhao, Meng-Fei; Yue, Zhen-Dong; Zhao, Hong-Wei; Wang, Lei; Fan, Zhen-Hua; He, Fu-Liang; Dai, Shan; Yao, Jian-Nan; Liu, Fu-Quan

    2015-11-21

    To evaluate combination transjugular intrahepatic portosystemic shunt (TIPS) and other interventions for hepatocellular carcinoma (HCC) and portal hypertension. Two hundred and sixty-one patients with HCC and portal hypertension underwent TIPS combined with other interventional treatments (transarterial chemoembolization/transarterial embolization, radiofrequency ablation, hepatic arterio-portal fistulas embolization, and splenic artery embolization) from January 1997 to January 2010 at Beijing Shijitan Hospital. Two hundred and nine patients (121 male and 88 female, aged 25-69 years, mean 48.3 ± 12.5 years) with complete clinical data were recruited. We evaluated the safety of the procedure (procedure-related death and serious complications), change of portal vein pressure before and after TIPS, symptom relief [e.g., ascites, hydrothorax, esophageal gastric-fundus variceal bleeding (EGVB)], cumulative rates of survival, and distributary channel restenosis. The characteristics of the patients surviving ≥ 5 and portal hypertension symptoms were ameliorated. During the 5 year follow-up, the total recurrence rate of resistant ascites or hydrothorax was 7.2% (15/209); 36.8% (77/209) for EGVB; and 39.2% (82/209) for hepatic encephalopathy. The cumulative rates of distributary channel restenosis at 1, 2, 3, 4, and 5 years were 17.2% (36/209), 29.7% (62/209), 36.8% (77/209), 45.5% (95/209) and 58.4% (122/209), respectively. No procedure-related deaths and serious complications (e.g., abdominal bleeding, hepatic failure, and distant metastasis) occurred. Moreover, Child-Pugh score, portal vein tumor thrombosis, lesion diameter, hepatic arterio-portal fistulas, HCC diagnosed before or after TIPS, stent type, hepatic encephalopathy, and type of other interventional treatments were related to 5 year survival after comparing patient characteristics. TIPS combined with other interventional treatments seems to be safe and efficacious in patients with HCC and portal

  8. Pathological Predictors of Shunt Stenosis and Hepatic Encephalopathy after Transjugular Intrahepatic Portosystemic Shunt

    Directory of Open Access Journals (Sweden)

    Fuliang He

    2016-01-01

    Full Text Available Background. Transjugular intrahepatic portosystemic shunt (TIPS is an artificial channel from the portal vein to the hepatic vein or vena cava for controlling portal vein hypertension. The major drawbacks of TIPS are shunt stenosis and hepatic encephalopathy (HE; previous studies showed that post-TIPS shunt stenosis and HE might be correlated with the pathological features of the liver tissues. Therefore, we analyzed the pathological predictors for clinical outcome, to determine the risk factors for shunt stenosis and HE after TIPS. Methods. We recruited 361 patients who suffered from portal hypertension symptoms and were treated with TIPS from January 2009 to December 2012. Results. Multivariate logistic regression analysis showed that the risk of shunt stenosis was increased with more severe inflammation in the liver tissue (OR, 2.864; 95% CI: 1.466–5.592; P=0.002, HE comorbidity (OR, 6.266; 95% CI, 3.141–12.501; P<0.001, or higher MELD score (95% CI, 1.298–1.731; P<0.001. Higher risk of HE was associated with shunt stenosis comorbidity (OR, 6.266; 95% CI, 3.141–12.501; P<0.001, higher stage of the liver fibrosis (OR, 2.431; 95% CI, 1.355–4.359; P=0.003, and higher MELD score (95% CI, 1.711–2.406; P<0.001. Conclusion. The pathological features can predict individual susceptibility to shunt stenosis and HE.

  9. Outcomes of Locoregional Tumor Therapy for Patients with Hepatocellular Carcinoma and Transjugular Intrahepatic Portosystemic Shunts

    Energy Technology Data Exchange (ETDEWEB)

    Padia, Siddharth A., E-mail: spadia@uw.edu; Chewning, Rush H., E-mail: rchewnin@uw.edu; Kogut, Matthew J., E-mail: kogutm@uw.edu; Ingraham, Christopher R., E-mail: cringa@uw.edu; Johnson, Guy E., E-mail: gej@uw.edu [University of Washington Medical Center, Section of Interventional Radiology, Department of Radiology (United States); Bhattacharya, Renuka, E-mail: renuka@uw.edu [University of Washington Medical Center, Division of Gastroenterology and Hepatology, Department of Medicine (United States); Kwan, Sharon W., E-mail: shakwan@uw.edu; Monsky, Wayne L., E-mail: wmonsky@uw.edu; Vaidya, Sandeep, E-mail: svaidya@uw.edu [University of Washington Medical Center, Section of Interventional Radiology, Department of Radiology (United States); Hippe, Daniel S., E-mail: dhippe@uw.edu [University of Washington Medical Center, Department of Radiology (United States); Valji, Karim, E-mail: kvalji@uw.edu [University of Washington Medical Center, Section of Interventional Radiology, Department of Radiology (United States)

    2015-08-15

    PurposeLocoregional therapy for hepatocellular carcinoma (HCC) can be challenging in patients with a transjugular intrahepatic portosystemic shunt (TIPS). This study compares safety and imaging response of ablation, chemoembolization, radioembolization, and supportive care in patients with both TIPS and HCC.MethodsThis retrospective study included 48 patients who had both a TIPS and a diagnosis of HCC. Twenty-nine of 48 (60 %) underwent treatment for HCC, and 19/48 (40 %) received best supportive care (i.e., symptomatic management only). While etiology of cirrhosis and indication for TIPS were similar between the two groups, treated patients had better baseline liver function (34 vs. 67 % Child-Pugh class C). Tumor characteristics were similar between the two groups. A total of 39 ablations, 17 chemoembolizations, and 10 yttrium-90 radioembolizations were performed on 29 patients.ResultsAblation procedures resulted in low rates of hepatotoxicity and clinical toxicity. Post-embolization/ablation syndrome occurred more frequently in patients undergoing chemoembolization than ablation (47 vs. 15 %). Significant hepatic dysfunction occurred more frequently in the chemoembolization group than the ablation group. Follow-up imaging response showed objective response in 100 % of ablation procedures, 67 % of radioembolization procedures, and 50 % of chemoembolization procedures (p = 0.001). When censored for OLT, patients undergoing treatment survived longer than patients receiving supportive care (2273 v. 439 days, p = 0.001).ConclusionsAblation appears to be safe and efficacious for HCC in patients with TIPS. Catheter-based approaches are associated with potential increased toxicity in this patient population. Chemoembolization appears to be associated with increased toxicity compared to radioembolization.

  10. Inpatient Cost Assessment of Transjugular Intrahepatic Portosystemic Shunt in the USA from 2001 to 2012.

    Science.gov (United States)

    Kuei, Andrew; Lee, Edward Wolfgang; Saab, Sammy; Busuttil, Ronald W; Durazo, Francisco; Han, Steven-Huy; ElKabany, Mohamed; McWilliams, Justin P; Kee, Stephen T

    2016-10-01

    Despite widespread use of transjugular intrahepatic portosystemic shunt (TIPS) for treatment of portal hypertension, a paucity of nationwide data exists on predictors of the economic impact related to TIPS. Using the National Inpatient Sample (NIS) database from 2001 to 2012, we aimed to evaluate factors contributing to hospital cost of patients admitted to US hospitals for TIPS. Using the NIS, we identified a discharge-weighted national estimate of 61,004 TIPS procedures from 2001 to 2012. Through independent sample analysis, we determined profile factors related to increases in hospital costs. Of all TIPS cases, the mean charge adjusted for inflation to the year 2012 is $125,044 ± $160,115. The mean hospital cost adjusted for inflation is $44,901 ± $54,565. Comparing pre- and post-2005, mean charges and cost have increased considerably ($98,154 vs. $142,652, p < 0.001 and $41,656 vs. $46,453, p < 0.001, respectively). Patients transferred from a different hospital, weekend admissions, Asian/Pacific Islander patients, and hospitals in the Northeastern and Western region had higher cost. Number of diagnoses and number of procedures show positive correlations with hospital cost, with number of procedures exhibiting stronger relationships (Pearson 0.613). Comorbidity measures with highest increases in cost were pulmonary circulation disorders ($32,157 increase, p < 0.001). The cost of the TIPS procedure is gradually rising for hospitals. Alongside recent healthcare reform through the Affordable Care Act, measures to reduce the economic burden of TIPS are of increasing importance. Data from this study are intended to aid physicians and hospitals in identifying improvements that could reduce hospital costs.

  11. Usefulness of Transjugular Intrahepatic Portosystemic Shunt in the Management of Bleeding Ectopic Varices in Cirrhotic Patients

    International Nuclear Information System (INIS)

    Vidal, V.; Joly, L.; Perreault, P.; Bouchard, L.; Lafortune, M.; Pomier-Layrargues, G.

    2006-01-01

    Purpose. To evaluate the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in the control of bleeding from ectopic varices. Methods. From 1995 to 2004, 24 cirrhotic patients, bleeding from ectopic varices, mean age 54.5 years (range 15-76 years), were treated by TIPS. The etiology of cirrhosis was alcoholic in 13 patients and nonalcoholic in 11 patients. The location of the varices was duodenal (n = 5), stomal (n = 8), ileocolic (n = 6), anorectal (n = 3), umbilical (n = 1), and peritoneal (n 1). Results. TIPS controlled the bleeding in all patients and induced a decrease in the portacaval gradient from 19.7 ± 5.4 to 6.4 ± 3.1 mmHg. Postoperative complications included self-limited intra-abdominal bleeding (n = 2), self-limited hemobilia (n = 1), acute thrombosis of the shunt (n = 1), and bile leak treated by a covered stent (n = 1). Median follow-up was 592 days (range 28-2482 days). Rebleeding occurred in 6 patients. In 2 cases rebleeding was observed despite a post-TIPS portacaval gradient lower than 12 mmHg and was controlled by variceal embolization; 1 patient underwent surgical portacaval shunt and never rebled; in 3 patients rebleeding was related to TIPS stenosis and treated with shunt dilatation with addition of a new stent. The cumulative rate of rebleeding was 23% and 31% at 1 and 2 years, respectively. One- and 2-year survival rates were 80% and 76%, respectively. Conclusion. The present series demonstrates that bleeding from ectopic varices, a challenging clinical problem, can be managed safely by TIPS placement with low rebleeding and good survival rates

  12. Experimental study of an endothelial progenitor cell coated stent in transjugular intrahepatic portosystemic shunt

    International Nuclear Information System (INIS)

    Shi Hongjian; Teng Gaojun; Cao Aihong; Chen Jun; Deng Gang

    2009-01-01

    Objective: To evaluate the efficacy of a self-expandable metal stent coated with autologous endothelial progenitor cells (EPCs) for prevention of restenosis in transjugular intrahepatic portosystemic shunt (TIPS) in a swine model. Methods: EPCs were coated on the metal stents using fibrin gel before TIPS procedure. TIPS was performed in 15 young adult pigs, using an autologous EPC-seeded stent (treatment group, n=9) or a conventional bare metal stent (control group, n=6). All pigs were sacrificed at 2 weeks after TIPS procedure. Portography was performed immediately before the euthanasia. Gross and microscopic pathological exams and immunohistochemical exams of the TIPS track specimens were performed. Fisher test and t test were used to analyse the data. Results: TIPS was performed successfully in all the 15 swine. On day 14 of follow-up, direct portography and necropsy demonstrated that 5 shunts remained patent, 2 shunts stenosed, and the remaining 2 shunts occluded in the treatment group (n=9); while 5 shunts were occluded and one shunt was stenotic in the control group (n=6). The patency rate was 56% vs 0 (P=0.03) between the two groups. Histological analyses showed a greater pseudo-intimal hyperplasia in the TIPS track of the control group than that of the treatment group (pseudointimal thickness at hepatic vein, hepatic parenchyma and portal vein site was (1.2±0.4), (1.3±0.5), (1.5±0.4) mm vs (1.0±0.6), (0.9±0.5), (1.0±0.4) mm respectively (P<0.05). Conclusion: The EPC-coated metal stent is feasibly constructed in vitro and improves the patency in TIPS in a porcine model. (authors)

  13. Transjugular Intrahepatic Portosystemic Shunt Insertion for the Management of Portal Hypertension in Children.

    Science.gov (United States)

    Johansen, Lauren; McKiernan, Patrick; Sharif, Khalid; McGuirk, Simon

    2018-04-17

    To describe our 20-year experience with transjugular intrahepatic portosystemic shunt (TIPSS) procedures for children with resistant portal hypertension (PHTN). Retrospective review of all children that had a TIPSS performed at Birmingham Children's Hospital from 1 January 1995 - 1 January 2015. 40 children underwent 42 attempted TIPSS for resistant PHTN with recurrent variceal bleeding (n = 35), refractory ascites (n = 4), and hypersplenism (n = 1). Median age at operation was 12 years (range, 7 months - 17 years). Thirty-four procedures were elective and 8 were emergency cases.TIPSS was established in 33 cases (79%). Median portal venous pressure reduction was 10mmHg. Variceal bleeding ceased in 27 (96%) and ascites improved in all. Clinical improvement following TIPSS enabled 7 children to be bridged to transplantation and 7 others to become suitable for transplantation. The 1-year and 5-year survival with TIPSS was 57% and 35%, respectively. Child-Pugh score C was an independent risk factor for adverse outcome (LR = 8.0; 95% CI 2.7 - 23.5; P = 0.001).There were 6 major complications: hepatic artery thrombosis and infarction (n = 1), hepatic artery pseudoaneurysm (n = 1), bile leak (n = 1), and hepatic encephalopathy (n = 3). Encephalopathy was resistant to medical treatment in 2 cases, necessitating staged closure in one.Ten patients (30%) required intervention to maintain TIPSS patency. The 1-year and 5-year freedom from reintervention was 71% and 55%, respectively. A TIPSS is highly successful in controlling symptoms in children with resistant PHTN and facilitating liver transplantation. However, it is technically demanding and not without risk. Patients must be appropriately selected and counselled.

  14. Plasma Glucose Level Is Predictive of Serum Ammonia Level After Retrograde Occlusion of Portosystemic Shunts.

    Science.gov (United States)

    Ishikawa, Tsuyoshi; Aibe, Yuki; Matsuda, Takashi; Iwamoto, Takuya; Takami, Taro; Sakaida, Isao

    2017-09-01

    The purpose of this study was to evaluate predictors of reduction in ammonia levels by occlusion of portosystemic shunts (PSS) in patients with cirrhosis. Forty-eight patients with cirrhosis (21 women, 27 men; mean age, 67.8 years) with PSS underwent balloon-occluded retrograde transvenous obliteration (BRTO) at one institution between February 2008 and June 2014. The causes of cirrhosis were hepatitis B in one case, hepatitis C in 20 cases, alcohol in 15 cases, nonalcoholic steatohepatitis in eight cases, and other conditions in four cases. The Child-Pugh classes were A in 24 cases, B in 23 cases, and C in one case. The indication for BRTO was gastric varices in 40 cases and hepatic encephalopathy in eight cases. Testing was conducted before and 1 month after the procedure. Statistical analyses were performed to identify predictors of a clinically significant decline in ammonia levels after BRTO. Occlusion of PSS resulted in a clinically significant decrease in ammonia levels accompanied by increased portal venous flow and improved Child-Pugh score. Univariate analyses showed that a reduction in ammonia levels due to BRTO was significantly related to lower plasma glucose levels, higher RBC counts, and higher hemoglobin concentration before the treatment. Furthermore, multivariate logistic regression identified preoperative plasma glucose level as the strongest independent predictor of a significant ammonia reduction in response to BRTO. In addition, although BRTO resulted in significantly declined ammonia levels in patients with normal glucose tolerance before the procedure, ammonia levels were not significantly decreased after shunt occlusion in patients with diabetes mellitus or impaired glucose tolerance before BRTO, according to 75-g oral glucose tolerance test results. Preoperative plasma glucose level is a useful predictor of clinically significant ammonia reduction resulting from occlusion of PSS in patients with cirrhosis. Even if PSS are present, control

  15. Radiation dose reduction during transjugular intrahepatic portosystemic shunt implantation using a new imaging technology

    Energy Technology Data Exchange (ETDEWEB)

    Spink, C., E-mail: c.spink@uke.de [Department of Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Avanesov, M. [Department of Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Schmidt, T. [Philips Healthcare, Hamburg (Germany); Grass, M. [Philips Research, Hamburg (Germany); Schoen, G. [Department of Medical Biometry and Epidemiology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Adam, G.; Bannas, P.; Koops, A. [Department of Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany)

    2017-01-15

    Highlights: • The new imaging technology halved the radiation exposure. • DSA image quality observed was not decreased after technology upgrade. • Radiation time and contrast consumption not significantly increased using the new technology. - Abstract: Objective: To compare patient radiation dose in patients undergoing transjugular intrahepatic portosystemic shunt (TIPS) implantation before and after an imaging-processing technology upgrade. Methods: In our retrospective single-center-study, cumulative air kerma (AK), cumulative dose area product (DAP), total fluoroscopy time and contrast agent were collected from an age- and BMI-matched collective of 108 patients undergoing TIPS implantation. 54 procedures were performed before and 54 after the technology upgrade. Mean values were calculated and compared using two-tailed t-tests. Two blinded, independent readers assessed DSA image quality using a four-rank likert scale and the Wilcoxcon test. Results: The new technology demonstrated a significant reduction of 57% of mean DAP (402.8 vs. 173.3 Gycm{sup 2}, p < 0.001) and a significant reduction of 58% of mean AK (1.7 vs. 0.7 Gy, p < 0.001) compared to the precursor technology. Time of fluoroscopy (26.4 vs. 27.8 min, p = 0.45) and amount of contrast agent (109.4 vs. 114.9 ml, p = 0.62) did not differ significantly between the two groups. The DSA image quality of the new technology was not inferior (2.66 vs. 2.77, p = 0.56). Conclusions: In our study the new imaging technology halved radiation dose in patients undergoing TIPS maintaining sufficient image quality without a significant increase in radiation time or contrast consumption.

  16. Transjugular intrahepatic porto-systemic shunt in the elderly: Palliation for complications of portal hypertension

    Science.gov (United States)

    Syed, Mubin I; Karsan, Hetal; Ferral, Hector; Shaikh, Azim; Waheed, Uzma; Akhter, Talal; Gabbard, Alan; Morar, Kamal; Tyrrell, Robert

    2012-01-01

    AIM: To present a dedicated series of transjugular intrahepatic porto-systemic shunts (TIPS) in the elderly since data is sparse on this population group. METHODS: A retrospective review was performed of patients at least 65 years of age who underwent TIPS at our institutions between 1997 and 2010. Twenty-five patients were referred for TIPS. We deemed that 2 patients were not considered appropriate candidates due to their markedly advanced liver disease. Of the 23 patients suitable for TIPS, the indications for TIPS placement was portal hypertension complicated by refractory ascites alone (n = 9), hepatic hydrothorax alone (n = 2), refractory ascites and hydrothorax (n = 1), gastrointestinal bleeding alone (n = 8), gastrointestinal bleeding and ascites (n = 3). RESULTS: Of these 23 attempted TIPS procedure patients, 21 patients had technically successful TIPS procedures. A total of 29 out of 32 TIPS procedures including revisions were successful in 21 patients with a mean age of 72.1 years (range 65-82 years). Three of the procedures were unsuccessful attempts at TIPS and 8 procedures were successful revisions of our existing TIPS. Sixteen of 21 patients who underwent successful TIPS (excluding 5 patients lost to follow-up) were followed for a mean of 14.7 mo. Ascites and/or hydrothorax was controlled following technically successful procedures in 12 of 13 patients. Bleeding was controlled following technically successful procedures in 10 out of 11 patients. CONCLUSION: We have demonstrated that TIPS is an effective procedure to control refractory complications of portal hypertension in elderly patients. PMID:22400084

  17. Sarcopenia Is Risk Factor for Development of Hepatic Encephalopathy After Transjugular Intrahepatic Portosystemic Shunt Placement.

    Science.gov (United States)

    Nardelli, Silvia; Lattanzi, Barbara; Torrisi, Sabrina; Greco, Francesca; Farcomeni, Alessio; Gioia, Stefania; Merli, Manuela; Riggio, Oliviero

    2017-06-01

    Hepatic encephalopathy (HE) is an important complication in patients with cirrhosis who received transjugular intrahepatic portosystemic shunts (TIPS). We investigated whether a decrease in muscle mass was associated independently with the occurrence of HE after TIPS. We performed a prospective study of 46 consecutive patients with cirrhosis (mean age, 58.6 ± 9.1 y; mean model for end-stage liver disease score, 11.3 ± 3.3; mean Child-Pugh score, 7.6 ± 1.5) who received TIPS from January 2013 through December 2014 at a tertiary center in Rome, Italy. All patients underwent computed tomography analysis at the level of the third lumbar vertebrae to determine the skeletal muscle index; sarcopenia was defined by sex-specific cut-off values. We estimated the incidence of the first episode of HE after TIPS, taking into account the competing risk nature of the data (death or liver transplantation). Twenty-six patients (57%) were found to have sarcopenia. Twenty-one patients (46%) developed overt HE in the 7 ± 9 months after TIPS placement; all of these patients were sarcopenic, according to the skeletal muscle index. Of the 25 patients without HE after TIPS, only 5 had sarcopenia. In multivariate analysis, model for end-stage liver disease score (subdistribution hazard ratio, 1.16; 95% confidence interval, 1.01-1.34; P = .043) and sarcopenia (subdistribution hazard ratio, 31.3; 95% confidence interval, 4.5-218.07; P Sarcopenia should be considered in selecting patients for TIPS therapy. Nutritional status should be evaluated in patients with sarcopenia before TIPS placement, which might reduce the incidence of HE. Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.

  18. Transjugular Intrahepatic Portosystemic Shunt Dysfunction: Concordance of Clinical Findings, Doppler Ultrasound Examination, and Shunt Venography.

    Science.gov (United States)

    Owen, Joshua M; Gaba, Ron Charles

    2016-01-01

    The objective of this study was to evaluate the concordance between clinical symptoms, Doppler ultrasound (US), and shunt venography for the detection of stent-graft transjugular intrahepatic portosystemic shunt (TIPS) dysfunction. Forty-one patients (M:F 30:11, median age 55 years) who underwent contemporaneous clinical exam, Doppler US, and TIPS venography between 2003 and 2014 were retrospectively studied. Clinical symptoms (recurrent ascites or variceal bleeding) were dichotomously classified as present/absent, and US and TIPS venograms were categorized in a binary fashion as normal/abnormal. US abnormalities included high/low (>190 or 50 cm/s), absent flow, and return of antegrade intra-hepatic portal flow. Venographic abnormalities included shunt stenosis/occlusion and/or pressure gradient elevation. Clinical and imaging concordance rates were calculated. Fifty-two corresponding US examinations and venograms were assessed. The median time between studies was 3 days. Forty of 52 (77%) patients were symptomatic, 33/52 (64%) US examinations were abnormal, and 20/52 (38%) TIPS venograms were abnormal. Concordance between clinical symptoms and TIPS venography was 48% (25/52), while the agreement between US and shunt venography was 65% (34/52). Clinical symptoms and the US concurred in 60% (31/52) of the patients. The sensitivity of clinical symptoms and US for the detection of venographically abnormal shunts was 80% (16/20) and 85% (17/20), respectively. Both clinical symptoms and the US had low specificity (25%, 8/32 and 50%, 16/32) for venographically abnormal shunts. Clinical findings and the US had low concordance rates with TIPS venography, with acceptable sensitivity but poor specificity. These findings suggest the need for improved noninvasive imaging methods for stent-graft TIPS surveillance.

  19. Radiofrequency Ablation for the Treatment of Hepatocellular Carcinoma in Patients with Transjugular Intrahepatic Portosystemic Shunts

    International Nuclear Information System (INIS)

    Park, Jonathan K.; Al-Tariq, Quazi Z.; Zaw, Taryar M.; Raman, Steven S.; Lu, David S.K.

    2015-01-01

    PurposeTo assess radiofrequency (RF) ablation efficacy, as well as the patency of transjugular intrahepatic portosystemic shunts (TIPSs), in patients with hepatocellular carcinoma (HCC).Materials and MethodsRetrospective database review of patients with pre-existing TIPS undergoing RF ablation of HCC was conducted over a 159-month period ending in November 2013. TIPS patency pre- and post-RF ablation was assessed by ultrasound, angiography, and/or contrast-enhanced CT or MRI. Patient demographics and immediate post-RF ablation outcomes and complications were also reviewed.Results19 patients with 21 lesions undergoing 25 RF ablation sessions were included. Child-Pugh class A, B, and C scores were seen in 1, 13, and 5 patients, respectively. Eleven patients (58 %) ultimately underwent liver transplantation. Immediate technical success was seen in all ablation sessions without residual tumor enhancement (100 %). No patients (0 %) suffered liver failure within 1 month of ablation. Pre-ablation TIPS patency was demonstrated in 22/25 sessions (88 %). Of 22 cases with patent TIPS prior to ablation, post-ablation patency was demonstrated in 22/22 (100 %) at immediate post-ablation imaging and in 21/22 (95 %) at last follow-up (1 patient was incidentally noted to have occlusion 31 months later). No immediate complications were observed.ConclusionAblation efficacy was similar to the cited literature values for patients without TIPS. Furthermore, TIPS patency was preserved in the majority of cases. Patients with both portal hypertension and HCC are not uncommonly encountered, and a pre-existing TIPS does not appear to be a definite contraindication for RF ablation

  20. Three-dimensional Image Fusion Guidance for Transjugular Intrahepatic Portosystemic Shunt Placement.

    Science.gov (United States)

    Tacher, Vania; Petit, Arthur; Derbel, Haytham; Novelli, Luigi; Vitellius, Manuel; Ridouani, Fourat; Luciani, Alain; Rahmouni, Alain; Duvoux, Christophe; Salloum, Chady; Chiaradia, Mélanie; Kobeiter, Hicham

    2017-11-01

    To assess the safety, feasibility and effectiveness of image fusion guidance with pre-procedural portal phase computed tomography with intraprocedural fluoroscopy for transjugular intrahepatic portosystemic shunt (TIPS) placement. All consecutive cirrhotic patients presenting at our interventional unit for TIPS creation from January 2015 to January 2016 were prospectively enrolled. Procedures were performed under general anesthesia in an interventional suite equipped with flat panel detector, cone-beam computed tomography (CBCT) and image fusion technique. All TIPSs were placed under image fusion guidance. After hepatic vein catheterization, an unenhanced CBCT acquisition was performed and co-registered with the pre-procedural portal phase CT images. A virtual path between hepatic vein and portal branch was made using the virtual needle path trajectory software. Subsequently, the 3D virtual path was overlaid on 2D fluoroscopy for guidance during portal branch cannulation. Safety, feasibility, effectiveness and per-procedural data were evaluated. Sixteen patients (12 males; median age 56 years) were included. Procedures were technically feasible in 15 of the 16 patients (94%). One procedure was aborted due to hepatic vein catheterization failure related to severe liver distortion. No periprocedural complications occurred within 48 h of the procedure. The median dose-area product was 91 Gy cm 2 , fluoroscopy time 15 min, procedure time 40 min and contrast media consumption 65 mL. Clinical benefit of the TIPS placement was observed in nine patients (56%). This study suggests that 3D image fusion guidance for TIPS is feasible, safe and effective. By identifying virtual needle path, CBCT enables real-time multiplanar guidance and may facilitate TIPS placement.

  1. Radiofrequency Ablation for the Treatment of Hepatocellular Carcinoma in Patients with Transjugular Intrahepatic Portosystemic Shunts

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jonathan K., E-mail: jonathan.park09@gmail.com [David Geffen School of Medicine at UCLA, Department of Radiology (United States); Al-Tariq, Quazi Z., E-mail: qat200@gmail.com [Stanford University School of Medicine, Department of Radiology (United States); Zaw, Taryar M., E-mail: taryar.zaw@gmail.com; Raman, Steven S., E-mail: sraman@mednet.ucla.edu; Lu, David S.K., E-mail: dlu@mednet.ucla.edu [David Geffen School of Medicine at UCLA, Department of Radiology (United States)

    2015-10-15

    PurposeTo assess radiofrequency (RF) ablation efficacy, as well as the patency of transjugular intrahepatic portosystemic shunts (TIPSs), in patients with hepatocellular carcinoma (HCC).Materials and MethodsRetrospective database review of patients with pre-existing TIPS undergoing RF ablation of HCC was conducted over a 159-month period ending in November 2013. TIPS patency pre- and post-RF ablation was assessed by ultrasound, angiography, and/or contrast-enhanced CT or MRI. Patient demographics and immediate post-RF ablation outcomes and complications were also reviewed.Results19 patients with 21 lesions undergoing 25 RF ablation sessions were included. Child-Pugh class A, B, and C scores were seen in 1, 13, and 5 patients, respectively. Eleven patients (58 %) ultimately underwent liver transplantation. Immediate technical success was seen in all ablation sessions without residual tumor enhancement (100 %). No patients (0 %) suffered liver failure within 1 month of ablation. Pre-ablation TIPS patency was demonstrated in 22/25 sessions (88 %). Of 22 cases with patent TIPS prior to ablation, post-ablation patency was demonstrated in 22/22 (100 %) at immediate post-ablation imaging and in 21/22 (95 %) at last follow-up (1 patient was incidentally noted to have occlusion 31 months later). No immediate complications were observed.ConclusionAblation efficacy was similar to the cited literature values for patients without TIPS. Furthermore, TIPS patency was preserved in the majority of cases. Patients with both portal hypertension and HCC are not uncommonly encountered, and a pre-existing TIPS does not appear to be a definite contraindication for RF ablation.

  2. Transjugular Intrahepatic Portosystemic Shunt Dysfunction: Concordance of Clinical Findings, Doppler Ultrasound Examination, and Shunt Venography

    Directory of Open Access Journals (Sweden)

    Joshua M Owen

    2016-01-01

    Full Text Available Objectives: The objective of this study was to evaluate the concordance between clinical symptoms, Doppler ultrasound (US, and shunt venography for the detection of stent-graft transjugular intrahepatic portosystemic shunt (TIPS dysfunction. Materials and Methods: Forty-one patients (M:F 30:11, median age 55 years who underwent contemporaneous clinical exam, Doppler US, and TIPS venography between 2003 and 2014 were retrospectively studied. Clinical symptoms (recurrent ascites or variceal bleeding were dichotomously classified as present/absent, and US and TIPS venograms were categorized in a binary fashion as normal/abnormal. US abnormalities included high/low (>190 or 50 cm/s, absent flow, and return of antegrade intra-hepatic portal flow. Venographic abnormalities included shunt stenosis/occlusion and/or pressure gradient elevation. Clinical and imaging concordance rates were calculated. Results: Fifty-two corresponding US examinations and venograms were assessed. The median time between studies was 3 days. Forty of 52 (77% patients were symptomatic, 33/52 (64% US examinations were abnormal, and 20/52 (38% TIPS venograms were abnormal. Concordance between clinical symptoms and TIPS venography was 48% (25/52, while the agreement between US and shunt venography was 65% (34/52. Clinical symptoms and the US concurred in 60% (31/52 of the patients. The sensitivity of clinical symptoms and US for the detection of venographically abnormal shunts was 80% (16/20 and 85% (17/20, respectively. Both clinical symptoms and the US had low specificity (25%, 8/32 and 50%, 16/32 for venographically abnormal shunts. Conclusion: Clinical findings and the US had low concordance rates with TIPS venography, with acceptable sensitivity but poor specificity. These findings suggest the need for improved noninvasive imaging methods for stent-graft TIPS surveillance.

  3. [Transjugular intrahepatic portosystemic shunting with covered stents in children: a preliminary study of safety and patency].

    Science.gov (United States)

    Zurera, L J; Espejo, J J; Canis, M; Bueno, A; Vicente, J; Gilbert, J J

    2014-01-01

    To retrospectively analyze the safety and efficacy of transjugular intrahepatic portosystemic shunting (TIPS) using covered stents in children. We present 6 children (mean age, 10.6 years; mean weight, 33.5kg) who underwent TIPS with 8mm diameter Viatorr(®) covered stents for acute (n=4) or recurrent (n=2) upper digestive bleeding that could not be controlled by endoscopic measures. Five of the children had cirrhosis and the other had portal vein thrombosis with cavernous transformation. We analyzed the relapse of upper digestive bleeding, the complications that appeared, and the patency of the TIPS shunt on sequential Doppler ultrasonography or until transplantation. A single stent was implanted in a single session in each child; none of the children died. The mean transhepatic gradient decreased from 16mmHg (range: 12-21mmHg) before the procedure to 9mmHg (range: 1-15mmHg) after TIPS. One patient developed mild encephalopathy, and the girl who had portal vein thrombosis with cavernous transformation developed an acute occlusion of the TIPS that resolved after the implantation of a coaxial stent. Three children received transplants (7, 9, and 10 months after the procedure, respectively), and the patency of the TIPS was confirmed at transplantation. In the three remaining children, patency was confirmed with Doppler ultrasonography 1, 3, and 5 months after implantation. None of the children had new episodes of upper digestive bleeding during follow-up after implantation (mean: 8.1 months). Our results indicate that TIPS with 8mm diameter Viatorr(®) covered stents can be safe and efficacious for the treatment of upper digestive bleeding due to gastroesophageal varices in cirrhotic children; our findings need to be corroborated in larger series. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

  4. Transjugular Intrahepatic Portosystemic Shunt before Abdominal Surgery in Cirrhotic Patients: A Retrospective, Comparative Study

    Directory of Open Access Journals (Sweden)

    Evelyne Vinet

    2006-01-01

    Full Text Available Surgery in cirrhotic patients is associated with high morbidity and mortality related to portal hypertension and liver insufficiency. Therefore, preoperative portal decompression is a logical approach to facilitate abdominal surgery and hopefully to improve postoperative survival. The present study evaluated the clinical outcomes of 18 patients (mean age 58 years with cirrhosis (seven alcoholics and 11 nonalcoholics who underwent transjugular intrahepatic portosystemic shunt (TIPS placement before antrectomy (n=5, colectomy (n=10, small-bowel resection (n=1, pancreatectomy (n=1 and nephrectomy (n=1. TIPS was performed a mean (± SD of 72±21 days before surgery and induced a marked mean decrease in portohepatic gradient from 21.4±3.9 mmHg to 8.4±3.4 mmHg. Cirrhotic patients (n=17 who underwent elective abdominal surgery without preoperative TIPS placement were used as the control group. Both groups were matched for age, etiology of cirrhosis, indications for surgery, type of surgery and coagulation parameters. The mean Pugh score was significantly higher in the TIPS group (7.7 versus 6.2. No significant differences were observed for operative blood loss, postoperative complications, duration of hospitalization and one-month (83% versus 88% or one-year (54% versus 63% cumulative survival rate. Analysis using the Cox proportional hazards model showed that neither TIPS placement nor preoperative Pugh score were independent predictors for survival. The present study suggests that preoperative TIPS placement does not improve postoperative evolution after abdominal surgery in cirrhotic patients with good or moderately impaired liver function.

  5. Three-Dimensional Path Planning Software-Assisted Transjugular Intrahepatic Portosystemic Shunt: A Technical Modification

    Energy Technology Data Exchange (ETDEWEB)

    Tsauo, Jiaywei, E-mail: 80732059@qq.com; Luo, Xuefeng, E-mail: luobo-913@126.com [West China Hospital of Sichuan University, Institute of Interventional Radiology (China); Ye, Linchao, E-mail: linchao.ye@siemens.com [Siemens Ltd, Healthcare Sector (China); Li, Xiao, E-mail: simonlixiao@gmail.com [West China Hospital of Sichuan University, Institute of Interventional Radiology (China)

    2015-06-15

    PurposeThis study was designed to report our results with a modified technique of three-dimensional (3D) path planning software assisted transjugular intrahepatic portosystemic shunt (TIPS).Methods3D path planning software was recently developed to facilitate TIPS creation by using two carbon dioxide portograms acquired at least 20° apart to generate a 3D path for overlay needle guidance. However, one shortcoming is that puncturing along the overlay would be technically impossible if the angle of the liver access set and the angle of the 3D path are not the same. To solve this problem, a prototype 3D path planning software was fitted with a utility to calculate the angle of the 3D path. Using this, we modified the angle of the liver access set accordingly during the procedure in ten patients.ResultsFailure for technical reasons occurred in three patients (unsuccessful wedged hepatic venography in two cases, software technical failure in one case). The procedure was successful in the remaining seven patients, and only one needle pass was required to obtain portal vein access in each case. The course of puncture was comparable to the 3D path in all patients. No procedure-related complication occurred following the procedures.ConclusionsAdjusting the angle of the liver access set to match the angle of the 3D path determined by the software appears to be a favorable modification to the technique of 3D path planning software assisted TIPS.

  6. Congenital Short QT Syndrome

    Directory of Open Access Journals (Sweden)

    Charles Antzelevitch

    2004-04-01

    Full Text Available Long QT intervals in the ECG have long been associated with sudden cardiac death. The congenital long QT syndrome was first described in individuals with structurally normal hearts in 1957.1 Little was known about the significance of a short QT interval. In 1993, after analyzing 6693 consecutive Holter recordings Algra et al concluded that an increased risk of sudden death was present not only in patients with long QT interval, but also in patients with short QT interval (<400 ms.2 Because this was a retrospective analysis, further evaluation of the data was not possible. It was not until 2000 that a short-QT syndrome (SQTS was proposed as a new inherited clinical syndrome by Gussak et al.3 The initial report was of two siblings and their mother all of whom displayed persistently short QT interval. The youngest was a 17 year old female presenting with several episodes of paroxysmal atrial fibrillation requiring electrical cardioversion.3 Her QT interval measured 280 msec at a heart rate of 69. Her 21 year old brother displayed a QT interval of 272 msec at a heart rate of 58, whereas the 51 year old mother showed a QT of 260 msec at a heart rate of 74. The authors also noted similar ECG findings in another unrelated 37 year old patient associated with sudden cardiac death.

  7. Congenital hypothyroidism in neonates

    Directory of Open Access Journals (Sweden)

    Aneela Anjum

    2014-01-01

    Full Text Available Context: Congenital hypothyroidism (CH is one of the most common preventable causes of mental retardation in children and it occurs in approximately 1:2,000-1:4,000 newborns. Aims and Objectives: The aim of this study is to determine the frequency of CH in neonates. Settings and Design: This cross-sectional study was conducted in neonatal units of the Department of Pediatrics Unit-I, King Edward Medical University/Mayo Hospital, Lahore and Lady Willington Hospital Lahore in 6 months (January-June 2011. Materials and Methods: Sample was collected by non-probability purposive sampling. After consent, 550 newborn were registered for the study. Demographic data and relevant history was recorded. After aseptic measures, 2-3 ml venous blood analyzed for thyroid-stimulating hormone (TSH level by immunoradiometric assay. Treatment was started according to the individual merit as per protocol. Statistical Analysis Used: Data was analyzed by SPSS 17 and Chi-square test was applied to find out the association of CH with different variables. Results: The study population consisted of 550 newborns. Among 550 newborns, 4 (0.8% newborns had elevated TSH level. CH had statistically significant association with mother′s hypothyroidism (P value 0.000 and mother′s drug intake during the pregnancy period (P value 0.013. Conclusion: CH is 0.8% in neonates. It has statistically significant association with mother′s hypothyroidism and mother′s drug intake during pregnancy.

  8. Congenital coronary artery fistula

    International Nuclear Information System (INIS)

    Oh, Yeon Hee; Kim, Hong; Zeon, Seoc Kil; Suh, Soo Jhi

    1986-01-01

    Congenital coronary artery fistula (CCAF) is communication of a coronary artery or its main branch with one of the atria or ventricles, the coronary sinus, the superior vena cava, or the pulmonary artery. In Korean peoples, only 4 cases of the CCAF were reported as rare as worldwide and authors want to report another case of CCAF, confirmed by operation. 10-year-old girl shows a fistula between sinus node artery of the right coronary artery and right atrium on root aortogram with left-to-right shunt and Qp/Qs=1.58, in which simple ligation of the sinus node artery from right coronary artery was performed. All of the 5 Korean CCAF (4 were previously reported and 1 of authors) were originated from right coronary artery, and of which 4 were opening into right ventricle and 1 of authors were into right atrium. Associated cardiac anomaly was noted in only 1 case as single coronary artery. Ages were from 9 months of age to 10 years old and no adult left case were found. 3 were female and 2 were male patients.

  9. Congenital nephrotic syndrome.

    Science.gov (United States)

    Hamed, Radi Ma

    2003-01-01

    The congenital nephrotic syndrome (CNS) is an uncommon disorder with onset of the nephrotic syndrome usually in the first three months of life. Several different diseases may cause the syndrome. These may be inherited, sporadic, acquired or part of a general malformation syndrome. The clinical course is marked by failure to thrive, recurrent life threatening bacterial infections, and early death from sepsis and/or uremia. A characteristic phenotype may be seen in children with CNS. The majority of reported cases of CNS are of the Finnish type (CNF). Although the role of the glomerular basement membrane has been emphasized as the barrier for retaining plasma proteins, recent studies have clearly shown that the slit diaphragm is the structure most likely to be the barrier in the glomerular capillary wall. The gene (NPHS1) was shown to encode a novel protein that was termed nephrin, due to its specific location in the kidney filter barrier, where it seems to form a highly organized filter structure. Nephrin is a transmembrane protein that probably forms the main building block of an isoporous zipper-like slit diaphragm filter structure. Defects in nephrin lead to the abnormal or absent slit diaphragm resulting in massive proteinuria and renal failure.

  10. [Penile congenital abnormalities].

    Science.gov (United States)

    Boillot, B; Teklali, Y; Moog, R; Droupy, S

    2013-07-01

    Congenital abnormalities of the penis are usually diagnosed at birth and pose aesthetic and functional problems sometimes requiring surgical management. A literature review was conducted on Medline considering the articles listed until January 2012. Hypospadias is the most common malformation (1 in 250 boys. Familial forms: 7%). The causes remain hypothetical but the doubling of the incidence in 30 years could be linked to fetal exposure to endocrine disruptors "estrogen-like" used in the food industry in particular. Surgical treatment is usually intended to improve the aesthetic appearance but sometimes, in case of significant curvature or posterior meatus, necessary for normal sexual life and fertility. Other malformations (epispades, buried penis, transpositions, twists and preputial abnormalities) as well as management for functional or aesthetic consequences of these malformations in adulthood require complex surgical care in a specialized environment. The improvement of surgical techniques and pediatric anesthesia allows an early and effective specialized surgical approach of penile malformations. Management of sequelae in adulthood must be discussed and requires experience of surgical techniques on pediatric and adult penis. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  11. Congenital cystic lung malformations

    International Nuclear Information System (INIS)

    Stoever, B.; Scheer, I.; Bassir, C.; Chaoui, R.; Henrich, W.; Schwabe, M.; Wauer, R.

    2006-01-01

    Purpose: The aim of the study concerning congenital cystic lung malformations was to evaluate prenatal diagnoses postnatally to determine prognostic factors as well as to define optimized perinatal management. Materials and Methods: The study is based on 45 prenatal ultrasound examinations depicting fetal cystic lung lesions. 32 of the mothers had follow-up examinations. 5 pregnancies were terminated due to CCAM and additional malformations. Complete regression of the lesions was seen prenatally in 8 cases and postnatally in 5 children. Results: Surgical intervention due to respiratory insufficiency was necessary in 4 neonates. According to the imaging results, CCAM was present in 4 cases and sequestration in 7 patients. No correlation between the imaging findings and the surgical results was found in 3 children: One child suffered from rhadomyoid dysplasia, and in the case of the second child, a left-sided hernia of the diaphragm and additional sequestration were detected. The third child showed AV malformation. The cystic lesions of the 14 children operated upon were proven histologically. The degree of accuracy in the present study was high. Conclusion: Precise perinatal management is warranted in order to determine according to the clinical relevance surgical intervention and to prevent complications after the first year of life. This is performed during the neonatal period for respiratory insufficient neonates and within the first year of life for clinically stable children. (orig.)

  12. Congenital pulmonary lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Campisi Corradino

    2006-10-01

    Full Text Available Abstract Congenital pulmonary lymphangiectasia (PL is a rare developmental disorder involving the lung, and characterized by pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth with severe respiratory distress, tachypnea and cyanosis, with a very high mortality rate at or within a few hours of birth. Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. Secondary PL may be caused by a cardiac lesion. The diagnostic approach includes complete family and obstetric history, conventional radiologic studies, ultrasound and magnetic resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy, and pleural effusion examination. During the prenatal period, all causes leading to hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation and pleural drainage are nearly always necessary to obtain a favorable outcome of respiratory distress. Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy, present medical problems which are characteristic of chronic lung disease.

  13. The impact of caudate lobe resection on margin status and outcomes in patients with hilar cholangiocarcinoma: a multi-institutional analysis from the US Extrahepatic Biliary Malignancy Consortium.

    Science.gov (United States)

    Bhutiani, Neal; Scoggins, Charles R; McMasters, Kelly M; Ethun, Cecilia G; Poultsides, George A; Pawlik, Timothy M; Weber, Sharon M; Schmidt, Carl R; Fields, Ryan C; Idrees, Kamran; Hatzaras, Ioannis; Shen, Perry; Maithel, Shishir K; Martin, Robert C G

    2018-04-01

    The objective of this study was to determine the impact of caudate resection on margin status and outcomes during resection of extrahepatic hilar cholangiocarcinoma. A database of 1,092 patients treated for biliary malignancies at institutions of the Extrahepatic Biliary Malignancy Consortium was queried for individuals undergoing curative-intent resection for extrahepatic hilar cholangiocarcinoma. Patients who did versus did not undergo concomitant caudate resection were compared with regard to demographic, baseline, and tumor characteristics as well as perioperative outcomes. A total of 241 patients underwent resection for a hilar cholangiocarcinoma, of whom 85 underwent caudate resection. Patients undergoing caudate resection were less likely to have a final positive margin (P = .01). Kaplan-Meier curve of overall survival for patients undergoing caudate resection indicated no improvement over patients not undergoing caudate resection (P = .16). On multivariable analysis, caudate resection was not associated with improved overall survival or recurrence-free survival, although lymph node positivity was associated with worse overall survival and recurrence-free survival, and adjuvant chemoradiotherapy was associated with improved overall survival and recurrence-free survival. Caudate resection is associated with a greater likelihood of margin-negative resection in patients with extrahepatic hilar cholangiocarcinoma. Precise preoperative imaging is critical to assess the extent of biliary involvement, so that all degrees of hepatic resections are possible at the time of the initial operation. Copyright © 2017 Elsevier Inc. All rights reserved.

  14. Clinical value of K-ras codon 12 analysis and endobiliary brush cytology for the diagnosis of malignant extrahepatic bile duct stenosis

    NARCIS (Netherlands)

    Sturm, P. D.; Rauws, E. A.; Hruban, R. H.; Caspers, E.; Ramsoekh, T. B.; Huibregtse, K.; Noorduyn, L. A.; Offerhaus, G. J.

    1999-01-01

    Extrahepatic biliary stenosis can be caused by benign and malignant disorders. In most cases, a tissue diagnosis is needed for optimal management of patients, but the sensitivity of biliary cytology for the diagnosis of a malignancy is relatively low. The additional diagnostic value of K-ras

  15. Combined liver and extrahepatic bile duct resection for biliary invasion of colorectal metastasis: a case-cohort analysis and systematic review

    NARCIS (Netherlands)

    Wiggers, Jimme K.; te Riele, Wouter W.; van Dongen, Tristan H.; Verheij, Joanne; Busch, Olivier R. C.; van Gulik, Thomas M.

    2016-01-01

    Background: Colorectal liver metastases (CRLMs) with biliary invasion can be treated with a combined liver and extrahepatic bile duct resection. The aim of this study was to analyze outcomes of this procedure in a case-cohort analysis and systematic review. Methods: Consecutive patients who

  16. [Congenital cardiopathy and cerebral abscess].

    Science.gov (United States)

    Paixão, A; de Andrade, F F; Sampayo, F

    1989-01-01

    During 1986 the authors came across two cases of brain abscess among children with congenital heart disease followed at the Pediatric Cardiology Service and decided to evaluate their global experience on the subject. In a retrospective study of 860 infants and children with cyanotic congenital heart disease and final diagnosis, there were four cases complicated with brain abscess. The following items were evaluated: prevalence of the complication, type of congenital heart disease, date and age at the diagnosis of brain abscess, diagnostic methods, neurosurgical treatment and results. The main findings were: all patients were above two years of age and had noncorrected cyanotic congenital heart disease belonging to the classic high risk group; the first two cases had been treated in other institutions and only scanty information was available; two recent cases had early diagnosis on CAT scan followed by neurosurgical treatment. All children survived. brain abscess is a rare but severe complication occurring in patients with noncorrected cyanotic congenital heart disease above two years of age; whenever prevention turns impossible, early diagnosis and treatment provide good short term and long term results. A multidisciplinar approach with full cooperation is advocated.

  17. Cyanotic congenital heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Yeon, Kyung Mo; Yoo, Shi Joon; Han, Man Chung; Hong, Chang Yee; Lee, Yung Kyoon [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1979-12-15

    Authors analyzed 265 cases of cyanotic congenital heart disease in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between April 1973 and August 1979. The results are as follows; 1. Among 265 cases, 178 patients were male and 87 patients were female. 240 patients were below the age of 20 and none was over 35 year. 2. The incidence of individual lesions are as follows: tetralogy of Fallot-176; double outlet right ventricle-20; pentalogy-12; trilogy-11; corrected transposition of great arteries-10; complete transposition of great arteries-8; pulmonary atresia-7; single ventricle-6; Ebstein's anomaly-5; total anomalous pulmonary venous return-4; tricuspid atresia-3; double outlet left ventricle-1; truncus arteriosus-1; hypoplastic left ventricle-1. 3. Fallot's teralogy, pentalogy and trilogy were characteristic in their simple chest and angiocardiographic manifestations, but in a few cases of tetralogy and pentalogy it was difficult to differentiate them from double outlet right ventricle or pulmonary atresia. 4. In double outlet right ventricle and transposition of great arteries which are the pathologic spectrum resulting from abnormal conal growth, differential points were ventricular and great arterial loop patterns and their connections but it was very difficult to differentiate them from each other by single injection into one ventricle alone. 5. Ebstein's anomaly and total anomalous pulmonary venous return were so characteristic in angiocardiography was done ventriculography alone. 6. In 7 cases with double outlet right ventricle and transposition of great arteries, selective biventriculography was done and more accurate diagnosis could be made, which was quite difficult with one ventriculography alone. In 31 cases, cineangiocardiography was done and it gave more accurate information about the type and degree of pulmonary stenosis and overriding of aorta, the origin

  18. Cyanotic congenital heart disease

    International Nuclear Information System (INIS)

    Yeon, Kyung Mo; Yoo, Shi Joon; Han, Man Chung; Hong, Chang Yee; Lee, Yung Kyoon

    1979-01-01

    Authors analyzed 265 cases of cyanotic congenital heart disease in which cardiac catheterization and angiocardiography were done at the Department of Radiology, Seoul National University Hospital between April 1973 and August 1979. The results are as follows; 1. Among 265 cases, 178 patients were male and 87 patients were female. 240 patients were below the age of 20 and none was over 35 year. 2. The incidence of individual lesions are as follows: tetralogy of Fallot-176; double outlet right ventricle-20; pentalogy-12; trilogy-11; corrected transposition of great arteries-10; complete transposition of great arteries-8; pulmonary atresia-7; single ventricle-6; Ebstein's anomaly-5; total anomalous pulmonary venous return-4; tricuspid atresia-3; double outlet left ventricle-1; truncus arteriosus-1; hypoplastic left ventricle-1. 3. Fallot's teralogy, pentalogy and trilogy were characteristic in their simple chest and angiocardiographic manifestations, but in a few cases of tetralogy and pentalogy it was difficult to differentiate them from double outlet right ventricle or pulmonary atresia. 4. In double outlet right ventricle and transposition of great arteries which are the pathologic spectrum resulting from abnormal conal growth, differential points were ventricular and great arterial loop patterns and their connections but it was very difficult to differentiate them from each other by single injection into one ventricle alone. 5. Ebstein's anomaly and total anomalous pulmonary venous return were so characteristic in angiocardiography was done ventriculography alone. 6. In 7 cases with double outlet right ventricle and transposition of great arteries, selective biventriculography was done and more accurate diagnosis could be made, which was quite difficult with one ventriculography alone. In 31 cases, cineangiocardiography was done and it gave more accurate information about the type and degree of pulmonary stenosis and overriding of aorta, the origin of great

  19. The role of B/D ratio and A/D ratio to defferentiate malignancy from benignancy in distal extrahepatic bile duct obstruction

    International Nuclear Information System (INIS)

    Rhim, Hyun Chul; Baek, JUng Hwan; Jeon, Eui Yong; Koh, Byung Hee; Cho, On Koo; Kim, Young Hwan

    1994-01-01

    To determine wheter bilirubin/extrahepatic bile duct diameter ratio(B/D ratio) or alkalinephosphatase/extrahepatic bile duct diameter ratio (A/D ratio) can be used to differentiate malignant from benigndisease in distal extrahepatic bile duct obstruction during ultrasonographic examination. We retrospectively reviewed the sonograms and laboratory data of 100 patients with obstructive jaundice (benign : n=50, malignant: : n=50). The diagnosis was confirmed either surgically (n=66) or clinically (n=34). The B/D ratio and A/D radio were calculated by means of dividing total bilirubin (mg/dl) and alkaline phosphatase (I.U.)respectively by maximum extrahepatic bile duct diameter(mm) on ultrasonogram. Significant difference in B/D ratio was found between the benignancy (0.28+0.25) and malignancy (0.98+0.84) groups (P<0.001). Significant difference in A/D ratio was also found between the benignancy (15.00+10.22) and malignancy (32.44+30.28) groups(P<0.001) Accuracies to predict malignancy according to criteria of B/D and A/D ratios were less than 75% and 65%respectively. On the other hand, the positive predictive value and specificity were relatively high. The B/D ratio and A/D ratio calculated from ultrasonograms can be used as a valuable screening index todifferentiate malignancy from benignacy in patients with distal extrahepatic bile duct obstruction, especially when the sonography is difficult because of the interposition of bowel gas or obesity

  20. Magnetic resonance imaging of congenital heart disease

    International Nuclear Information System (INIS)

    Fletcher, B.D.; Jacobstein, M.D.

    1988-01-01

    Focusing primarily on MR imaging of the heart, this book covers other diagnostic imaging modalities as well. The authors review new technologies and diagnostic procedures pertinent to congenital heat disease and present each congenital heat abnormality as a separate entity

  1. Genetic Counseling for Congenital Heart Defects

    Science.gov (United States)

    ... Artery Disease Venous Thromboembolism Aortic Aneurysm More Genetic Counseling for Congenital Heart Defects Updated:Jan 19,2018 ... with congenital heart disease considers having children. Genetic counseling can help answer these questions and address your ...

  2. CCT and sonographic findings in congenital craniopharyngioma

    Energy Technology Data Exchange (ETDEWEB)

    Helmke, K.; Hausdorf, G.; Moehrs, D.; Laas, R.

    1984-11-01

    In a case of congenital craniopharyngioma results of the sonographic and CCT examinations are reported and compared with postmortem macroscopic cuts. Comparison is made of clinical data from six cases with congenital craniopharyngioma as reported in the literature.

  3. CCT and sonographic findings in congenital craniopharyngioma

    International Nuclear Information System (INIS)

    Helmke, K.; Hausdorf, G.; Moehrs, D.; Laas, R.

    1984-01-01

    In a case of congenital craniopharyngioma results of the sonographic and CCT examinations are reported and compared with postmortem macroscopic cuts. Comparison is made of clinical data from six cases with congenital craniopharyngioma as reported in the literature. (orig.)

  4. Genetics Home Reference: severe congenital neutropenia

    Science.gov (United States)

    ... A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons. ... Genetic Testing Registry: Severe congenital neutropenia 2, autosomal dominant Genetic Testing Registry: Severe congenital neutropenia 3, autosomal ...

  5. Genetics Home Reference: congenital dyserythropoietic anemia

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions CDA Congenital dyserythropoietic anemia Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Congenital dyserythropoietic anemia ( CDA ) is an inherited blood disorder that affects ...

  6. Cerebral palsy and congenital malformations

    DEFF Research Database (Denmark)

    Garne, Ester; Dolk, Helen; Krägeloh-Mann, Inge

    2007-01-01

    AIM: To determine the proportion of children with cerebral palsy (CP) who have cerebral and non-cerebral congenital malformations. METHODS: Data from 11 CP registries contributing to the European Cerebral Palsy Database (SCPE), for children born in the period 1976-1996. The malformations were...... classified as recognized syndromes, chromosomal anomalies, cerebral malformations or non-cerebral malformations. Prevalence of malformations was compared to published data on livebirths from a European database of congenital malformations (EUROCAT). RESULTS: Overall 547 out of 4584 children (11.9%) with CP...... were reported to have a congenital malformation. The majority (8.6% of all children) were diagnosed with a cerebral malformation. The most frequent types of cerebral malformations were microcephaly and hydrocephaly. Non-cerebral malformations were present in 97 CP children and in further 14 CP children...

  7. Antenatal diagnosis of congenital deafness.

    Science.gov (United States)

    Isaacson, G

    1988-01-01

    Advances in the field of antenatal diagnosis have made possible the detection of profound sensorineural hearing loss prior to birth. Fetal motion in response to sound and auditory evoked potential testing can determine the presence of fetal hearing in the third trimester of pregnancy. Imaging modalities including ultrasound, computed tomography, and magnetic resonance imaging hold promise for the diagnosis of some forms of congenital deafness in the second trimester fetus. The methods by which congenital deafness soon may be diagnosed and the implications for the otologist are discussed.

  8. On diagnosis of congenital toxoplasmosis

    International Nuclear Information System (INIS)

    Denisova, L.B.; Vorontsova, S.V.; Shvedov, V.A.

    1999-01-01

    Chemical manifestations are considered and CT-semiotics of congenital toxoplasmosis, which can form intracranial calcinates, is described. Taking a certain case of congenital toxoplasmosis observation as an example the potentialities and significance of X-ray computerized tomography (CT) in the identification of brain pathological changes are demonstrated. It is shown that intracranial calcificates may be a sign of toxoplasmosis infection. In case of cytomegalovirus infection the calcificates on CT-scans have mostly periventricular localization. Equally with the revealing of brain status in case of chronic stage of neutrotoxoplasmosis the CT has also played a decisive role in diagnosis of pseudotumoroseus course of ischemic insult [ru

  9. MRI of congenital urethroperineal fistula

    Energy Technology Data Exchange (ETDEWEB)

    Ghadimi-Mahani, Maryam; Dillman, Jonathan R.; Pai, Deepa; DiPietro, Michael [C. S. Mott Children' s Hospital, Department of Radiology, Section of Pediatric Radiology, University of Michigan Health System, Ann Arbor, MI (United States); Park, John [C. S. Mott Children' s Hospital, Department of Pediatric Urology, University of Michigan Health System, Ann Arbor, MI (United States)

    2010-12-15

    We present the MRI features of a congenital urethroperineal fistula diagnosed in a 12-year-old boy being evaluated after a single urinary tract infection. This diagnosis was initially suggested by voiding cystourethrogram and confirmed by MRI. Imaging revealed an abnormal fluid-filled tract arising from the posterior urethra and tracking to the perineal skin surface that increased in size during micturition. Surgical resection and histopathological evaluation of the abnormal tract confirmed the diagnosis of congenital urethroperineal fistula. MRI played important roles in confirming the diagnosis and assisting surgical planning. (orig.)

  10. MRI of congenital urethroperineal fistula

    International Nuclear Information System (INIS)

    Ghadimi-Mahani, Maryam; Dillman, Jonathan R.; Pai, Deepa; DiPietro, Michael; Park, John

    2010-01-01

    We present the MRI features of a congenital urethroperineal fistula diagnosed in a 12-year-old boy being evaluated after a single urinary tract infection. This diagnosis was initially suggested by voiding cystourethrogram and confirmed by MRI. Imaging revealed an abnormal fluid-filled tract arising from the posterior urethra and tracking to the perineal skin surface that increased in size during micturition. Surgical resection and histopathological evaluation of the abnormal tract confirmed the diagnosis of congenital urethroperineal fistula. MRI played important roles in confirming the diagnosis and assisting surgical planning. (orig.)

  11. Congenital hypothyroidism: insights into pathogenesis and treatment

    OpenAIRE

    Cherella, Christine E.; Wassner, Ari J.

    2017-01-01

    Congenital hypothyroidism occurs in approximately 1 in 2000 newborns and can have devastating neurodevelopmental consequences if not detected and treated promptly. While newborn screening has virtually eradicated intellectual disability due to severe congenital hypothyroidism in the developed world, more stringent screening strategies have resulted in increased detection of mild congenital hypothyroidism. Recent studies provide conflicting evidence about the potential neurodevelopmental risks...

  12. The prevalence of congenital anomalies in Europe

    DEFF Research Database (Denmark)

    Dolk, Helen; Loane, Maria; Garne, Ester

    2010-01-01

    EUROCAT (European Surveillance of Congenital Anomalies) is the network of population-based registers of congenital anomaly in Europe, with a common protocol and data quality review, covering 1.5 million annual births in 22 countries. EUROCAT recorded a total prevalence of major congenital anomali...

  13. Standardization of MIP technique in three-dimensional CT portography: usefulness in evaluation of portosystemic collaterals in cirrhotic patients

    International Nuclear Information System (INIS)

    Kim, Jong Gi; Kim, Yong; Kim, Chang Won; Lee, Jun Woo; Lee, Suk Hong

    2003-01-01

    To assess the usefulness of three-dimensional CT portography using a standardized maximum intensity projection (MIP) technique for the evaluation of portosystemic collaterals in cirrhotic patients. In 25 cirrhotic patients with portosystemic collaterals, three-phase CT using a multide-tector-row helical CT scanner was performed to evaluate liver disease. Late arterial-phase images were transferred to an Advantage Windows 3.1 workstation (Gener Electric). Axial images were reconstructed by means of three-dimensional CT portography, using both a standardized and a non-standardized MIP technique, and the respective reconstruction times were determined. Three-dimensional CT portography with the standardized technique involved eight planes, namely the spleno-portal confluence axis (coronal, lordotic coronal, lordotic coronal RAO 30 .deg. C, and lordotic coronal LAO 30 .deg. C), the left renal vein axis (lordotic coronal), and axial MIP images (lower esophagus level, gastric fundus level and splenic hilum). The eight MIP images obtained in each case were interpreted by two radiologists, who reached a consensus in their evaluation. The portosystemic collaterals evaluated were as follows: left gastric vein dilatation; esophageal, paraesophageal, gastric, and splenic varix; paraumbilical vein dilatation; gastro-renal, spleno-renal, and gastro-spleno-renal shunt; mesenteric, retroperitoneal, and omental collaterals. The average reconstruction time using the non-standardized MIP technique was 11 minutes 23 seconds, and with the standardized technique, the time was 6 minutes 5 seconds. Three-dimensional CT portography with the standardized technique demonstrated left gastric vein dilatation (n=25), esophageal varix (n=18), paraesophageal varix (n=13), gastric varix (n=4), splenic varix (n=4), paraumbilical vein dilatation (n=4), gastro-renal shunt (n=3), spleno-renal shunt (n=3), and gastro-spleno-renal shunt (n=1). Using three-dimensional CT protography and the non

  14. Hemodynamic changes in liver measured by multi-imaging methods before and after transjugular intrahepatic portosystemic stent-shunt

    International Nuclear Information System (INIS)

    Huang Yonghui; Chen Wei; Li Jiaping; Zhuang Wenquan; Li Ziping; Yang Jianyong

    2007-01-01

    Objective: To evaluate hemodynamic changes in liver treated by transjugular intrahepatic portosystemic stent-shunt (TIPSS) with hepatic computed tomography (CT) perfusion, Doppler ultrasound and portal vein pressure measurement, as well as the correlation among these methods. Methods: Hepatic CT perfusion was performed in 9 cirrhotic patients one week before TIPSS and 72 hours after TIPSS. Intraoperative portal vein pressure was measured before and after portosystemic shunt establish. The follow- up hepatic CT perfusion were carried out in 3 cases at 3 months and 6 months postoperatively. The hemodynamic surveillance by Doppler ultrasound were performed in 48 hours and 3 months after TIPSS for 9 cases, and in 6 months after TIPSS for 6 cases. Two cases underwent venography and portal vein pressure measurement in 6 months after TIPSS treatment. Results: The mean of portal vein perfusion (PVP), total hepatic blood flow (THBF), hepatic perfusion index (HPI) and portal vein free pressure (PVFP) before TIPSS were (0.92 ± 0.18) ml·min·ml -1 , (1.28±0.17) ml·min -1 ·ml -1 , (28 ± 8)%, and (23.92±0.86) mmHg, respectively. In 72 hours after TIPSS, the mean of PVP, THBF, HPI and PVFP were (0.21 ± 0.15) ml·min -1 ·ml -1 , (0.74 ± 0.18) ml·min -1 ·ml -1 , (74±13)%, and (12.62±1.54) mm Hg, respectively. After treatment, the mean of PVP was (0.49±0.05) ml·min -1 ·ml -1 at 3 months and (0.57±0.03) ml·min -1 ·ml -1 at 6 months, respectively. There was negative correlation between PVP and PVFP before TIPSS (r= 0.678, P 0.05). Moreover, a significant correlation was found between the degree of portal vein pressure decrease and portal vein perfusion decrease (r=0.867, P 3 /s was not less than that of main portal vein (9.83±5.72) cm 3 /s until six months after treatment. Conclusion: The portal vein pressure obviously decreased after TIPSS, and meanwhile, most blood flow of portal vein passed through portosystemic stent shunt without liver parenchyma perfusion

  15. MELD Score as a Predictor of Early Death in Patients Undergoing Elective Transjugular Intrahepatic Portosystemic Shunt (TIPS) Procedures

    International Nuclear Information System (INIS)

    Montgomery, Aaron; Ferral, Hector; Vasan, Rajiv; Postoak, Darren W.

    2005-01-01

    Purpose. To Evaluate the MELD score as a predictor of 30-day mortality in patients undergoing elective TIPS procedures. Methods. This was a retrospective, IRB-approved study. The medical records of all patients who underwent a TIPS procedure between May 1, 1999 and June 1, 2003 in a single institution were reviewed. Patients who underwent elective TIPS were selected. Elective TIPS was performed in 119 patients with a mean age of 55.1 (± 9.6) years. The MELD and Child-Pugh scores before TIPS, etiology of cirrhosis, portosystemic gradients before and after TIPS, procedure time, and procedural complications were obtained from the medical records. The MELD and Child-Pugh scores before TIPS were compared between the survivor group (SG) and the early death (EDG) group. The early death rate was calculated for MELD score subgroups (1-10, 11-17, 18-24, and >24). Data were analyzed using the Fisher exact test, chi-square test and independent-sample t-test. A p value of less than 0.05 was considered significant. Results. Technical success rate was 100%. The early death rate was 10.9% (13/119). The mean MELD scores before TIPS were 19.4 (± 5.9) (EDG) and 14 (± 4.2) (SG) (p = 0.025). The early death rate was highest in the pre-TIPS MELD > 24 subgroup. The Child-Pugh scores were 9.0 (± 1.6) (SG) and 9.8 ± 1.06 (EDG) (p 0.08). The mean portosystemic gradients before TIPS were 20.5 (± 7.7) mmHg (EDG) and 22.7 (± 7.3) (SG) (p > 1) and the mean portosystemic gradients after TIPS were 6.5 (± 3.5) (EDG) and 6.9 (± 2.4) (SG) (p > 1). The mean procedural times were 95.6 (± 8.4) min (EDG) and 89.2 (± 7.5) min (SG) (p > 1). No early death was attributed to a fatal complication during TIPS. Conclusion. The MELD score is useful in identifying patients at a higher risk of early death after an elective TIPS. On the basis of our results, we do not endorse elective TIPS in patients with MELD scores > 24

  16. Diagnostic accuracy of (18) F-methylcholine positron emission tomography/computed tomography for intra- and extrahepatic hepatocellular carcinoma.

    Science.gov (United States)

    Bieze, Matthanja; Klümpen, Heinz-Josef; Verheij, Joanne; Beuers, Ulrich; Phoa, Saffire S K S; van Gulik, Thomas M; Bennink, Roelof J

    2014-03-01

    Diagnosis of hepatocellular carcinoma (HCC) primarily involves imaging. The aim of this study was to assess the accuracy of (18) F-fluorocholine ((18) F-FCH) positron emission tomography (PET) for detection of HCC and evaluation of extent of disease. Patients with HCC >1 cm were included between 2009 and July 2011, and follow-up closed in February 2013. Diagnosis was based on American Association for the Study of Liver Diseases criteria, and all patients underwent (18) F-FCH PET/computed tomography (CT) at baseline before treatment, 6 underwent a second PET/CT posttreatment, and 1 a third during follow-up. Whole-body PET and low-dose CT imaging were performed 15 minutes after (18) F-FCH injection. Evaluation of imaging was done with standardized uptake value (SUV) ratios: SUV maximum of the lesion divided by the SUV mean of surrounding tissue. Statistical analyses included descriptive analyses, receiver operating characteristic curve, McNemar's test, and Kaplan-Meier's test at 5% level of significance. Twenty-nine patients revealed 53 intrahepatic lesions. In 48 of 53 lesions, (18) F-FCH PET was positive (SUVratio , 1.95 ± 0.66; sensitivity, 88%; specificity, 100%). PET/CT showed uptake in 18 extrahepatic lesions and no uptake in 3 lesions affirmed non-HCC lesions; all lesions were confirmed with additional investigation (accuracy, 100%). In 17 of 29 patients, additional lesions were found on PET/CT imaging, with implications for treatment in 15 patients. Posttreatment PET/CT showed identical results, compared with standard treatment evaluation. This study shows additional value of (18) F-FCH PET/CT for patients with HCC. (18) F-FCH PET/CT has implications for staging, management, and treatment evaluation because of accurate assessment of extrahepatic disease. © 2014 by the American Association for the Study of Liver Diseases.

  17. Experimental comparison study of the tissue characteristics in transjugular intrahepatic portosystemic shunt and vascular stent

    International Nuclear Information System (INIS)

    Lu Qin; An Yanli; Deng Gang; Fang Wen; Zhu Guangyu; Niu Huanzhang; Yu Hui; Li Guozhao; Teng Gaojun; Wang Zhen; Wei Xiaoying

    2009-01-01

    Objective: To investigate the tissue characteristics within vascular stent and transjugular intrahepatic portosystemic shunt(TIPS) on swine and to provide more information for the understanding and prevention of vascular stent and TIPS restenosis. Methods: Animal models for TIPS were built in 6 swine and vascular stents were implanted in iliac veins simultaneously. 14-28 days after the operation, the 6 swine were killed to remove the TIPS and vascular stent and the pathological examinations were performed on the tissues within the shunt and stent. The similarities and differences of the tissues within the shunt and stent were analyzed with Krttskal Wallis test. Results: Restenosis of TIPS occurred in 4 models and complete occlusion were seen in 2, while all vascular stents were patent and coated with a thin layer of intimal tissue. Electron microscopic results showed that the tissues in restenotic TIPS were loose and with more extra matrix and fibers, and less smooth muscle, fibroblastic and myofibroblastic cells with different and irregular shape and rich secretory granules. The tissues in patent TIPS contained more extra fibers, smooth muscle and fibroblastic cells with normal organelle. The intimal tissues in vascular stent contained more fibers and fibroblasts cells, less smooth muscle cells. On immunohistochemical staining, the tissues in restenotic and patent TIPS as well as the intimal tissues in vascular stent had strong positive expression for anti-SMC- actin-α, the expression were gradually weakened for PCNA, the intimal tissues in vascular stent had a strong positive expression for vimentin, while the expression of the tissues in restenotic and patent TIPS were weakened gradually. For myoglobulin, the tissues in restenotic TIPS had weakly positive expression, the expression in patent TIPS and vascular stent were almost negative. Western blot results for TGF-β showed that the absorbance ratios of the intima tissues in vascular stent, normal vascular

  18. Combination therapy using PSE and TIO ameliorates hepatic encephalopathy due to intrahepatic portosystemic venous shunt in idiopathic portal hypertension

    International Nuclear Information System (INIS)

    Kojima, Seiichiro; Ito, Hiroyuki; Takashimizu, Shinji; Ichikawa, Hitoshi; Matsumoto, Tomohiro; Hasebe, Terumitsu; Watanabe, Norihito

    2016-01-01

    A 64-year-old woman treated for anemia and ascites exhibited hepatic encephalopathy. Abdominal ultrasonography and computed tomography (CT) showed communication between the portal vein and the middle hepatic vein, indicating an intrahepatic portosystemic venous shunt (PSS). Since hepatic encephalopathy of the patient was resistant to medical treatment, interventional radiology was performed for the treatment of shunt obliteration. Hepatic venography showed anastomosis between the hepatic vein branches, supporting the diagnosis of idiopathic portal hypertension (IPH). To minimize the increase in portal vein pressure after shunt obliteration, partial splenic artery embolization (PSE) was first performed to reduce portal vein blood flow. Transileocolic venous obliteration (TIO) was then performed, and intrahepatic PSS was successfully obliterated using coils with n-butyl-2-cyanoacrylate (NBCA). In the present case, hepatic encephalopathy due to intrahepatic PSS in the patient with IPH was successfully treated by combination therapy using PSE and TIO

  19. Liver perfusion scintigraphy prior to and after transjugular intrahepatic portosystemic shunts (TIPS) in patients with portal hypertension

    International Nuclear Information System (INIS)

    Willkomm, P.; Schomburg, A.; Reichmann, K.; Bangard, M.; Overbeck, B.; Biersack, H.J.; Brensing, K.A.; Sauerbruch, T.

    2000-01-01

    Purpose: This investigation was performed to compare the hemodynamic results of the transjugular intrahepatic portosystemic shunt, a new interventional treatment for portal hypertension, with those observed after the established surgical shunt interventions. Methods: We examined 22 patients with portal hypertension due to liver cirrhosis before and after elective TIPS by liver perfusion scintigraphy. The relative portal perfusion was determined before and after the shunt procedure. Additionally, we measured the portal pressure gradient (PPG: Portal-central venous pressure, mmHg). Results: Prior to TIPS, the relative portal perfusion was significantly reduced to 22±9.1%. After the intervention we calculated values of 23.1±10.7% in the TIPS-group (p=0.67; not significant). In spite of unchanged portal perfusion, the portal pressure was significantly (p [de

  20. Transjugular Intrahepatic Portosystemic Shunt for Treatment of Cirrhosis-related Chylothorax and Chylous Ascites: Single-institution Retrospective Experience

    Energy Technology Data Exchange (ETDEWEB)

    Kikolski, Steven G., E-mail: skikolski@ucsd.edu; Aryafar, Hamed, E-mail: haryafar@ucsd.edu; Rose, Steven C., E-mail: scrose@ucsd.edu [University of California San Diego Health Sciences, Department of Radiology (United States); Roberts, Anne C., E-mail: acroberts@ucsd.edu [University of California San Diego Health Sciences, Department of Vascular and Interventional Radiology (United States); Kinney, Thomas B., E-mail: tbkinney@ucsd.edu [University of California San Diego Health Sciences, Department of Radiology (United States)

    2013-08-01

    PurposeTo investigate the efficacy and safety of the use of transjugular intrahepatic portosystemic shunt (TIPS) creation to treat cirrhosis-related chylous collections (chylothorax and chylous ascites).MethodsWe retrospectively reviewed data from four patients treated for refractory cirrhosis-related chylous collections with TIPS at our institution over an 8 year period.ResultsOne patient had chylothorax, and three patients had concomitant chylothorax and chylous ascites. There were no major complications, and the only procedure-related complications occurred in two patients who had mild, treatable hepatic encephalopathy. All patients had improvement as defined by decreased need for thoracentesis or paracentesis, with postprocedure follow-up ranging from 19 to 491 days.ConclusionTIPS is a safe procedure that is effective in the treatment of cirrhosis-related chylous collections.

  1. Congenital Aortic Stenosis and Aneurysms

    NARCIS (Netherlands)

    D. van der Linde (Denise)

    2013-01-01

    textabstractDue to improvements in pediatric cardio-thoracic surgery, anesthesia and diagnostics over the past decades, the number of adult patients with congenital heart disease (CHD) is growing. This causes an increasing demand in clinical practice for insight in long term outcome in both

  2. Severe congenital neutropenia (Kostmann Syndrome)

    African Journals Online (AJOL)

    Severe congenital neutropenia (SCN), Kostmann syndrome is a heterogenous disorder of myelopoiesis characterized by severe chronic ... erogenous hematological disorders, characterized by extremely low circu- lating neutrophils and ..... tic activation of STAT5 and stimulate. G-CSF-induced cell proliferation.26, 27.

  3. Angiocardiography in congenital heart malformations

    International Nuclear Information System (INIS)

    Soto, B.; Pacifico, A.D.

    1990-01-01

    The contents of this book are well organized, it deals with the technique of angiocardiography, and describes the angiocardiographic anatomy of the normal heart and offers an excellent sequential analysis of cardiac morphology. The rest of the book describes the angiocardiographic findings observed in individual congenital cardiac disorders

  4. Congenital Pulmonary Malformation in Children

    Directory of Open Access Journals (Sweden)

    Montasser Nadeem

    2012-01-01

    Full Text Available Congenital Pulmonary Malformations (CPMs are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life.

  5. [Genetics of congenital heart diseases].

    Science.gov (United States)

    Bonnet, Damien

    2017-06-01

    Developmental genetics of congenital heart diseases has evolved from analysis of serial slices in embryos towards molecular genetics of cardiac morphogenesis with a dynamic view of cardiac development. Genetics of congenital heart diseases has also changed from formal genetic analysis of familial recurrences or population-based analysis to screening for mutations in candidates genes identified in animal models. Close cooperation between molecular embryologists, pathologists involved in heart development and pediatric cardiologists is crucial for further increase of knowledge in the field of cardiac morphogenesis and genetics of cardiac defects. The genetic model for congenital heart disease has to be revised to favor a polygenic origin rather than a monogenic one. The main mechanism is altered genic dosage that can account for heart diseases in chromosomal anomalies as well as in point mutations in syndromic and isolated congenital heart diseases. The use of big data grouping information from cardiac development, interactions between genes and proteins, epigenetic factors such as chromatin remodeling or DNA methylation is the current source for improving our knowledge in the field and to give clues for future therapies. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  6. Genetics of Primary Congenital Hypothyroidism

    NARCIS (Netherlands)

    Zwaveling-Soonawala, Nitash; van Trotsenburg, Paul

    2018-01-01

    Congenital hypothyroidism (CH) is one of the most common preventable forms of mental retardation and since the implementation of neonatal screening programs in the mid-1970s, early detection and treatment have proven to be very successful in preventing brain damage. CH may be of thyroidal (=

  7. Congenital Pulmonary Malformation in Children

    OpenAIRE

    Nadeem, Montasser; Elnazir, Basil; Greally, Peter

    2012-01-01

    Congenital Pulmonary Malformations (CPMs) are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life.

  8. Neural Correlates of Congenital Amusia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2006-10-01

    Full Text Available Magnetic resonance T1-weighted images were analyzed, using voxel-based morphometry (VBM, to detect brain anatomical differences in two independent groups of adults with congenital amusia (or tone-deafness compared to controls with normal pitch perception and no formal musical training.

  9. Congenital bronchobiliary fistula: MRI appearance

    International Nuclear Information System (INIS)

    Hourigan, Jon S.; Carr, Michael G.; Burton, Edward M.; Ledbetter, Joel C.

    2004-01-01

    Congenital bronchobiliary fistula (CBBF) is a rare anomaly. Twenty-three cases have been reported since the anomaly was first described in 1952. Most of these cases were diagnosed by bronchoscopy, cholangiography, or hepatobiliary nuclear imaging. Our case of a newborn with bilious emesis with CBBF was depicted by T1-weighted gradient-echo MRI sequences. (orig.)

  10. Congenital lymphoedema of the genitalia

    NARCIS (Netherlands)

    Bolt, R. J.; Peelen, W.; Nikkels, P. G.; de Jong, T. P.

    1998-01-01

    Isolated congenital lymphoedema of the external genitalia in boys is extremely rare. It can have major physical and emotional consequences for the children. Three male patients with primary lymphoedema of the penis and scrotum are described. The first case presented with lymphoedema of the prepuce

  11. What's New in Congenital Scoliosis?

    Science.gov (United States)

    Pahys, Joshua M; Guille, James T

    2018-03-01

    Congenital scoliosis is a failure of vertebral formation, segmentation, or a combination of the 2 arising from abnormal vertebral development during weeks 4 to 6 of gestation. The associated spinal deformity can be of varying severity and result in a stable or progressive deformity based on the type and location of the anomalous vertebra(e). Bracing for congenital scoliosis is rarely indicated, while recent reports have demonstrated the utility of serial derotational casting for longer curves with multiple anomalous vertebrae as an effective "time buying strategy" to delay the need for surgery. Earlier hemivertebra excision and short-segment posterior spinal fusion have been advocated to prevent future curve progression of the deformity and/or the development of large compensatory curves. It has been shown in recent long-term follow-up studies that growth rates of the vertebral body and spinal canal are not as dramatically affected by pedicle screw instrumentation at a young age as once thought. Growth friendly surgery with either spine-based or rib-based anchors has demonstrated good results with curve correction while maintaining spinal growth. Rib-based anchors are typically more commonly indicated in the setting of chest wall abnormalities and/or when spinal anatomy precludes placement of spinal instrumentation. Recently, magnetically controlled growing rods have shown promising results in several studies that include a small subset of congenital scoliosis cases. A literature search was performed to identify existing studies related to the treatment of congenital scoliosis published from January 1, 2005 to June 1, 2016. Databases included PubMed, Medline, and the Cochrane Library. The search was limited to English articles and yielded 36 papers. This project was initiated by the Pediatric Orthopaedic Society of North America Publications Committee and was reviewed and approved by the Pediatric Orthopaedic Society of North America Presidential Line. A total of

  12. Congenital anomalies in Primorsky region.

    Science.gov (United States)

    Kiku, P; Voronin, S; Golokhvast, K

    2015-01-01

    According to WHO hereditary diseases and congenital malformations contribute significantly to the health of population. Thus, the problems of epidemiology, clinical presentation, diagnosis and treatment of congenital abnormalities are of interest for many researchers [2]. In addition, the dynamic accounting for the incidence of congenital malformations and hereditary diseases allows the researchers to assess the ecological situation in the region [1]. The occurrence of congenital anomalies in the world varies; it depends heavily on how carefully the data is collected [4]. Multifactorial or polygenic diseases develop under the influence of environmental factors in the presence of defective genes. They can constitute up to 90% of all chronic pathology [2-5]. To determine the incidence of congenital anomalies under the influence of environmental factors. The study used the methodology of system evaluation of congenital anomalies incidence in Primorsky region, depending on bio-climatic and environmental conditions. The authors used health statistics for the period from 2000 to 2014, F.12 class for congenital abnormalities in adolescents and children that were compared in geographical and temporal aspects with environmental factors of 33 settlements in Primorsky region. The environment is represented by nature and climate (6 factor modules) and sanitation (7 factor modules) blocks of factors. When formalizing the information database of the environment a specially developed 10-point assessment scale was used. Statistical processing of the information was carried out using Pearson's chi-squared test and multiple regression method from SSPS application program package. The study found that over the 15-year period the level of congenital abnormalities in children increased by 27.5% and in adolescents - by 35.1%, and in 2014 it amounted to 1687.6 and 839.3 per 100 000 people, respectively. The predictive model shows a steady further growth of this pathology. The incidence

  13. Transjugular Intrahepatic Porto-Systemic Stent-Shunt for Therapy of Bleeding Esophageal Varices Due to Extramedullary Hematopoiesis in Primary Myelofibrosis: A Case Report

    OpenAIRE

    Phillip, Veit;Berger, Hermann;Straub, Melanie;Saugel, Bernd;Treiber, Matthias;Einwächter, Henrik;Schmid, Roland M.;Huber, Wolfgang

    2016-01-01

    Background: Primary myelofibrosis belongs to the group of myeloproliferative syndromes. Extramedullary hematopoiesis in the liver can lead to portal hypertension. Patient and Methods: We report a case of a patient with life-threatening, endoscopically not treatable bleeding from esophageal varices due to extramedullary hematopoiesis of the liver that was successfully treated with placement of a transjugular intrahepatic porto-systemic stent-shunt (TIPS). Results: Therapy of variceal bleeding ...

  14. Transjugular Intrahepatic Portosystemic Shunt, Mechanical Aspiration Thrombectomy, and Direct Thrombolysis in the Treatment of Acute Portal and Superior Mesenteric Vein Thrombosis

    International Nuclear Information System (INIS)

    Ferro, Carlo; Rossi, Umberto G.; Bovio, Giulio; Dahamane, M'Hamed; Centanaro, Monica

    2007-01-01

    A patient was admitted because of severe abdominal pain, anorexia, and intestinal bleeding. Contrast-enhanced multidetector computed tomography demonstrated acute portal and superior mesenteric vein thrombosis (PSMVT). The patient was treated percutaneously with transjugular intrahepatic portosystemic shunt (TIPS), mechanical aspiration thrombectomy, and direct thrombolysis, and 1 week after the procedure, complete patency of the portal and superior mesenteric veins was demonstrated. TIPS, mechanical aspiration thrombectomy, and direct thrombolysis together are promising endovascular techniques for the treatment of symptomatic acute PSMVT

  15. Long-Term Follow-Up After Successful Transjugular Intrahepatic Portosystemic Shunt Placement in a Pediatric Patient with Budd-Chiari Syndrome

    International Nuclear Information System (INIS)

    Carnevale, Francisco Cesar; Szejnfeld, Denis; Moreira, Airton Mota; Gibelli, Nelson; Gregorio, Miguel Angel De; Tannuri, Uenis; Cerri, Giovanni Guido

    2008-01-01

    Orthotopic liver transplantation is the standard of care in patients with Budd-Chiari syndrome (BCS), and transjugular intrahepatic portosystemic shunt (TIPS) has become an important adjunct procedure while the patient is waiting for a liver. No long-term follow up of TIPS in BCS patients has been published in children. We report successful 10-year follow-up of a child with BCS and iatrogenic TIPS dysfunction caused by oral contraceptive use.

  16. Epidemiology of cancers of the liver, gall bladder, extrahepatic bile duct and pancreas in Nagasaki city from 1973 to 1982 with reference to atomic bomb exposure

    International Nuclear Information System (INIS)

    Takeshima, Fuminao; Shimokawa, Isao; Takashima, Kazuhiko

    1990-01-01

    This epidemiology study included 808 cases of liver cancers, 378 cases of gall bladder and extrahepatic bile duct cancer, and 312 cases of pancreas cancer registered at the Nagasaki Tumor Registry from 1973 to 1982, with emphasis on the relationship of these cancers to radiation exposure. The incidence of liver, gall bladder and extrahepatic bile duct cancer in both sexes tended to be higher in A-bomb survivors than in the nonexposed population, but were particularly significantly higher in male A-bomb survivors in each age-adjusted relative risk group. The incidence of pancreas cancer in females tended to be higher among A-bomb survivors than in the nonexposed population, and age-adjusted relative risk was significantly higher for female A-bomb survivors. No significant differences in relative distribution of histological type between exposed and nonexposed groups was observed. (author)

  17. [Identification of risk factors for congenital malformations].

    Science.gov (United States)

    Canals C, Andrea; Cavada C, Gabriel; Nazer H, Julio

    2014-11-01

    The relative importance of congenital malformations as a cause of death in the first year of life is increasing along with the control of preventable causes of perinatal mortality. To identify risk factors for congenital malformations. Retrospective case-control study of births registered in the database of The Latin American Collaborative Study of Congenital Malformations (ECLAMC), in the period 2001-2010. Birth weight and gestational age were significantly lower in cases than controls, behaving as risk factors and associated with a greater severity of congenital malformations. The risk and severity of congenital malformations increased along with mother's age. Fetal growth retardation, a history of congenital malformations in the family, physical factors and acute illnesses of the mother in the first trimester of pregnancy were also significant risk factors for congenital malformations and their severity. The educational level of the mother was a protective factor for congenital malformations and their severity. Variables previously identified as risk factors for congenital malformations, were significantly related with the occurrence of congenital malformations and their severity.

  18. Association Between Portosystemic Shunts and Increased Complications and Mortality in Patients With Cirrhosis.

    Science.gov (United States)

    Simón-Talero, Macarena; Roccarina, Davide; Martínez, Javier; Lampichler, Katharina; Baiges, Anna; Low, Gavin; Llop, Elba; Praktiknjo, Michael; Maurer, Martin H; Zipprich, Alexander; Triolo, Michela; Vangrinsven, Guillaume; Garcia-Martinez, Rita; Dam, Annette; Majumdar, Avik; Picón, Carmen; Toth, Daniel; Darnell, Anna; Abraldes, Juan G; Lopez, Marta; Kukuk, Guido; Krag, Aleksander; Bañares, Rafael; Laleman, Wim; La Mura, Vincenzo; Ripoll, Cristina; Berzigotti, Annalisa; Trebicka, Jonel; Calleja, Jose Luis; Tandon, Puneeta; Hernandez-Gea, Virginia; Reiberger, Thomas; Albillos, Agustín; Tsochatzis, Emmanuel A; Augustin, Salvador; Genescà, Joan

    2018-05-01

    Spontaneous portosystemic shunts (SPSS) have been associated with hepatic encephalopathy (HE). Little is known about their prevalence among patients with cirrhosis or clinical effects. We investigated the prevalence and characteristics of SPSS in patients with cirrhosis and their outcomes. We performed a retrospective study of 1729 patients with cirrhosis who underwent abdominal computed tomography or magnetic resonance imaging analysis from 2010 through 2015 at 14 centers in Canada and Europe. We collected data on demographic features, etiology of liver disease, comorbidities, complications, treatments, laboratory and clinical parameters, Model for End-Stage Liver Disease (MELD) score, and endoscopy findings. Abdominal images were reviewed by a radiologist (or a hepatologist trained by a radiologist) and searched for the presence of SPSS, defined as spontaneous communications between the portal venous system or splanchnic veins and the systemic venous system, excluding gastroesophageal varices. Patients were assigned to groups with large SPSS (L-SPSS, ≥8 mm), small SPSS (S-SPSS, SPSS (W-SPSS). The main outcomes were the incidence of complications of cirrhosis and mortality according to the presence of SPSS. Secondary measurements were the prevalence of SPSS in patients with cirrhosis and their radiologic features. L-SPSS were identified in 488 (28%) patients, S-SPSS in 548 (32%) patients, and no shunt (W-SPSS) in 693 (40%) patients. The most common L-SPSS was splenorenal (46% of L-SPSS). The presence and size of SPSS increased with liver dysfunction: among patients with MELD scores of 6-9, 14% had L-SPSS and 28% had S-SPSS; among patients with MELD scores of 10-13, 30% had L-SPSS and 34% had S-SPSS; among patients with MELD scores of 14 or higher, 40% had L-SPSS and 32% had S-SPSS (P SPSS, 34% of patients with S-SPSS, and 20% of patients W-SPSS (P SPSS groups). Recurrent or persistent HE was reported in 52% of patients with L-SPSS, 44% of patients with S

  19. Magnetic resonance cholangiopancreatography (MRCP) using new negative per-oral contrast agent based on superparamagnetic iron oxide nanoparticles for extrahepatic biliary duct visualization in liver cirrhosis.

    Science.gov (United States)

    Polakova, Katerina; Mocikova, Ingrid; Purova, Dana; Tucek, Pavel; Novak, Pavel; Novotna, Katerina; Izak, Niko; Bielik, Radoslav; Zboril, Radek; Miroslav, Herman

    2016-12-01

    Magnetic resonance cholangiopancreatography (MRCP) is often used for imaging of the biliary tree and is required by surgeons before liver transplantation. Advanced liver cirrhosis and ascites in patients however present diagnostic problems for MRCP. The aim of this study was to find out if the use of our negative per-oral contrast agent containing superparamagnetic iron oxide nanoparticles (SPIO) in MRCP is helpful for imaging of hepatobiliary tree in patients with liver cirrhosis. Forty patients with liver cirrhosis were examined on a 1.5 T MR unit using standard MRCP protocol. Twenty patients (group A) underwent MRCP after administration of per-oral SPIO contrast agent 30 min before examination. In group B, twenty patients were examined without per-oral bowel preparation. Ascites was present in eleven patients from group A and in thirteen patients in group B. Four radiologists analyzed MR images for visibility and delineation of the biliary tree. χ 2 tests were used for comparison of the visibility of intrahepatic and extrahepatic biliary ducts in patients with and without ascites. Better extrahepatic biliary duct visualization and visibility of extraluminal pathologies in patients with ascites was proved after administration of SPIO contrast agent. No statistically significant difference between group A and B was found for visualization of extrahepatic biliary ducts in patients without ascites. Delineation of intrahepatic biliary ducts was independent on bowel preparation. Application of our negative per-oral SPIO contrast agent before MRCP improves the visualization of extrahepatic biliary ducts in patients with ascites which is helpful during the liver surgery, mainly in liver transplantation.

  20. Prenatal education for congenital toxoplasmosis.

    Science.gov (United States)

    Di Mario, Simona; Basevi, Vittorio; Gagliotti, Carlo; Spettoli, Daniela; Gori, Gianfranco; D'Amico, Roberto; Magrini, Nicola

    2015-10-23

    Congenital toxoplasmosis is considered a rare but potentially severe infection. Prenatal education about congenital toxoplasmosis could be the most efficient and least harmful intervention, yet its effectiveness is uncertain. To assess the effects of prenatal education for preventing congenital toxoplasmosis. We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (31 May 2015), and reference lists of relevant papers, reviews and websites. Randomized and quasi-randomized controlled trials of all types of prenatal education on toxoplasmosis infection during pregnancy. Cluster-randomized trials were eligible for inclusion. Two review authors independently assessed trials for inclusion and risk of bias, extracted data and checked them for accuracy. Two cluster-randomized controlled trials (RCTs) (involving a total of 5455 women) met the inclusion criteria. The two included trials measured the effectiveness of the intervention in different ways, which meant that meta-analysis of the results was not possible. The overall quality of the two studies, as assessed using the GRADE approach, was low, with high risk of detection and attrition bias in both included trials.One trial (432 women enrolled) conducted in Canada was judged of low methodological quality. This trial did not report on any of the review's pre-specified primary outcomes and the secondary outcomes reported results only as P values. Moreover, losses to follow-up were high (34%, 147 out of 432 women initially enrolled). The authors concluded that prenatal education can effectively change pregnant women's behavior as it increased pet, personal and food hygiene. The second trial conducted in France was also judged of low methodological quality. Losses to follow-up were also high (44.5%, 2233 out of 5023 women initially enrolled) and differential (40% in the intervention group and 52% in the control group). The authors concluded that prenatal education for congenital toxoplasmoses has a

  1. The Predictive Value of Indocyanine Green Clearance in Future Liver Remnant for Posthepatectomy Liver Failure Following Hepatectomy with Extrahepatic Bile Duct Resection.

    Science.gov (United States)

    Yokoyama, Yukihiro; Ebata, Tomoki; Igami, Tsuyoshi; Sugawara, Gen; Mizuno, Takashi; Yamaguchi, Junpei; Nagino, Masato

    2016-06-01

    Postoperative liver failure (PHLF) is one of the most common complications following major hepatectomy. The preoperative assessment of future liver remnant (FLR) function is critical to predict the incidence of PHLF. To determine the efficacy of the plasma clearance rate of indocyanine green clearance of FLR (ICGK-F) in predicting PHLF in cases of highly invasive hepatectomy with extrahepatic bile duct resection. Five hundred and eighty-five patients who underwent major hepatectomy with extrahepatic bile duct resection, from 2002 to 2014 in a single institution, were evaluated. Among them, 192 patients (33 %) had PHLF. The predictive value of ICGK-F for PHLF was determined and compared with other risk factors for PHLF. The incidence of PHLF was inversely proportional to the level of ICGK-F. With multivariate logistic regression analysis, ICGK-F, combined pancreatoduodenectomy, the operation time, and blood loss were identified as independent risk factors of PHLF. The risk of PHLF increased according to the decrement of ICGK-F (the odds ratio of ICGK-F for each decrement of 0.01 was 1.22; 95 % confidence interval 1.12-1.33; P bile duct resection. This criterion may be useful for highly invasive hepatectomy, such as that with extrahepatic bile duct resection.

  2. Differentiation grade for extrahepatic bile duct adenocarcinoma: Assessed by diffusion-weighted imaging at 3.0-T MR

    International Nuclear Information System (INIS)

    Huang, Xin-Qiao; Shu, Jian; Luo, Li; Jin, Ming-Li; Lu, Xiao-Fei; Yang, Shu-Gen

    2016-01-01

    Highlights: • DWI can assess the prognostic of extrahepatic bile duct adenocarcinomas (EBDA). • The ADC value of EBDA lesion descended as the differentiation grade decreased. • The cut off value from ROC Curve of ADC can predict EBDA differentiation grade. • To some extend, it is reliable as we analysed a relatively large number of EBDAs. - Abstract: Purpose—: To assess the pathological differentiation grade in the patients with extrahepatic bile duct adenocarcinoma (EBDA) using diffusion-weighted imaging (DWI) at 3.0-T MR. Methods—: Sixty-eight patients who were clinically and histologically diagnosed with EBDA underwent abdominal DWI within 2 weeks before surgery. The lesion signal intensity, signal intensity ratio of the lesion and hepar (SIR-LH) value, and apparent diffusion coefficient (ADC) value in patients with EBDA were retrospectively analysed. Results: —In the 68 patients, 22 well-differentiated, 36 moderately-differentiated, and 10 poorly-differentiated EBDAs were histopathological confirmed. These EBDAs exhibited hyper-intensity on DWI in 95.59% of patients. Hyper-intensity lesions were found in 90.91% of patients with good-differentiation, in 97.22% with moderate-differentiation and in 100% with poor-differentiation. There showed no statistical difference for the lesion signal intensity (P = 0.426) and SIR-LH value (P = 0.766) on DWI among three groups. The median ADC value of the well-differentiated, moderately-differentiated and poorly-differentiated EBDAs were 1.506 × 10 −3 mm 2 /s, 1.275 × 10 −3 mm 2 /s and 1.154 × 10 −3 mm 2 /s, respectively. As the pathological differentiation grade decreased, the lesion ADC value of EBDA gradually declined (x 2 = 51.220, P = 0.000). The ADC value <1.184 × 10 −3 mm 2 /s can predict the poorly-differentiated EBDA with a sensitivity of 100% and a specificity of 94.83%. The ADC value >1.316 × 10 −3 mm 2 / s can forecast the well-differentiated EBDA with a sensitivity of 100% and a

  3. Differentiation grade for extrahepatic bile duct adenocarcinoma: Assessed by diffusion-weighted imaging at 3.0-T MR

    Energy Technology Data Exchange (ETDEWEB)

    Huang, Xin-Qiao, E-mail: 1109489147@qq.com; Shu, Jian, E-mail: shujiannc@163.com; Luo, Li, E-mail: 23857275@qq.com; Jin, Ming-Li, E-mail: 1242037853@qq.com; Lu, Xiao-Fei, E-mail: 82901782@qq.com; Yang, Shu-Gen, E-mail: 82587849@qq.com

    2016-11-15

    Highlights: • DWI can assess the prognostic of extrahepatic bile duct adenocarcinomas (EBDA). • The ADC value of EBDA lesion descended as the differentiation grade decreased. • The cut off value from ROC Curve of ADC can predict EBDA differentiation grade. • To some extend, it is reliable as we analysed a relatively large number of EBDAs. - Abstract: Purpose—: To assess the pathological differentiation grade in the patients with extrahepatic bile duct adenocarcinoma (EBDA) using diffusion-weighted imaging (DWI) at 3.0-T MR. Methods—: Sixty-eight patients who were clinically and histologically diagnosed with EBDA underwent abdominal DWI within 2 weeks before surgery. The lesion signal intensity, signal intensity ratio of the lesion and hepar (SIR-LH) value, and apparent diffusion coefficient (ADC) value in patients with EBDA were retrospectively analysed. Results: —In the 68 patients, 22 well-differentiated, 36 moderately-differentiated, and 10 poorly-differentiated EBDAs were histopathological confirmed. These EBDAs exhibited hyper-intensity on DWI in 95.59% of patients. Hyper-intensity lesions were found in 90.91% of patients with good-differentiation, in 97.22% with moderate-differentiation and in 100% with poor-differentiation. There showed no statistical difference for the lesion signal intensity (P = 0.426) and SIR-LH value (P = 0.766) on DWI among three groups. The median ADC value of the well-differentiated, moderately-differentiated and poorly-differentiated EBDAs were 1.506 × 10{sup −3} mm{sup 2}/s, 1.275 × 10{sup −3} mm{sup 2}/s and 1.154 × 10{sup −3} mm{sup 2}/s, respectively. As the pathological differentiation grade decreased, the lesion ADC value of EBDA gradually declined (x{sup 2} = 51.220, P = 0.000). The ADC value <1.184 × 10{sup −3} mm{sup 2}/s can predict the poorly-differentiated EBDA with a sensitivity of 100% and a specificity of 94.83%. The ADC value >1.316 × 10{sup −3} mm{sup 2}/ s can forecast the well

  4. Congenital adrenal hyperplasia: Case report.

    OpenAIRE

    Jaime Avaria E.; María José Vargas F.; Loreto Triviño F.; Andrea Gleisner E.

    2013-01-01

    INTRODUCTION: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disease whose main cause is the deficiency of 21-hydroxylase, an enzyme involved in the synthesis of cortisol and aldosterone. There are two forms of CAH, a classical and nonclassical form, being the first objective of analysis in the clinical case. Its clinical manifestations vary in severity, depending on the level of hormone deficiency. Within the classic is described the salt-wasting form, whose consequences are ...

  5. Congenital hypopituitarism and renal failure

    Directory of Open Access Journals (Sweden)

    Gaurav Atreja

    2011-01-01

    Full Text Available Congenital hypopituitarism is potentially fatal in the newborn period but treatable if the diagnosis is made early. We report a neonate who presented with hypothermia and severe hypoglycemia. He also had undescended testis and micropenis. Initial screening revealed panhypopituitarism, which was corrected promptly. He developed renal failure due to initial cardiovascular compromise related to hypotension but recovered quickly with standard management. Magnetic resonance imaging revealed absent stalk of anterior pituitary.

  6. Congenital unilateral hydrocephalus - CT findings

    International Nuclear Information System (INIS)

    Schulman, H.; Landau, D.; Schulman, P.; Hertzanu, Y.

    2000-01-01

    Congenital unilateral hydrocephalus is extremely uncommon with 18 cases previously reported in the English literature. Two additional newborns with unilateral hydrocephalus are presented. The second baby also presented a mega cisterna magna. This unusual association between Dandy-Walker variant and unilateral hydrocephalus has not been previously reported. Following ventriculo-peritoneal shunt, the babies had a normal cognitive neurodevelopment. The role of cranial computed tomography (CT) in diagnosis and follow-up is emphasized

  7. TYPE IC CHOLEDOCHAL CYST PRESENTING AN EXTRAHEPATAL CHOLESTASIS IN A 3 YEAR OLD BOY

    Directory of Open Access Journals (Sweden)

    Muhammad Reza

    2015-10-01

    Full Text Available Choledochal cyst is a rare congenital dilatation of the bile ducts, mostly diagnosed in childhood. Whenappropriate resection is not performed, cholangiocarcinoma may occur in a high incidence within thesecond decade of life. This report aims to present a rare case in experience of diagnosis and managementtype IC choledochal cyst in children. We present case of a 3-year-old boy who came with jaundice anditchy skin, abdominal pain, brownish urine, pales colored of stool. Abdominal ultrasonography andcomputed tomography scan revealed type IC choledochal cyst. Patient underwent complete cyst removalsurgery and bilioenteric anastomosis through Roux-en-y hepaticojejunostomy. Excision biopsy confirmedthe diagnosis of type IC choledochal cyst. Post surgical follow up shown good physical and laboratorycondition  and  there was no  recurrence  of  symptoms. Early  surgical  procedure  through Roux-en-yhepatojejunostomy, has been performed. Long  term  follow up also  facilities good prognostic  to  thepatient. [MEDICINA 2015;46:56-60].Kista  koledokus  adalah merupakan  penyakit  saluran  empedu  bawaan  yang  jarang  dijumpai  danbanyak terdiagnosis pada saat usia anak-anak. Tindakan berupa reseksi kista adalah yang terpentingdilakukan,  jika  tidak  segera  dilakukan  maka  dapat  meningkatkan  resiko  terjadinyacholangiocarcinoma dalam usia dekade kedua penderita dalam kehidupan. Tujuan kasus ini dilaporkanuntuk menggambarkan pengalaman dalam mendiagnosis dan tata  laksana kista koledokal tipe ICyang jarang pada anak-anak. Laporan kasus ini pada anak laki-laki berumur 3 tahun dengan keluhankulit tampak kuning dan gatal, nyeri perut, urin berwarna kecoklatan, tinja yang pucat. Ultrasonografidan CT  scan abdomen memperlihatkan adanya kista koledokus. Tindakan bedah  eksisi kista dananastomosis bilioenterik dengan menggunakan tehnik hepatojejunostomi Roux-en-y. Diagnosa kistakoledokus  tipe  IC  terkonfirmasi  saat

  8. Congenital uronephropathy pattern in children

    Directory of Open Access Journals (Sweden)

    Husein Alatas

    2001-10-01

    Full Text Available To obtain the basic data of congenital uronephropathy pattern and the affecting factors in children, we conducted a cross-sectional study at the Department of Child Health Cipto Mangunkusumo (CM Hospital Jakarta from 1995 to 1999 and 9 teaching hospitals throughout Indonesia. During the study period 134 patients were obtained, 116 patients from the CM Hospital and 18 patients from other teaching hospitals. Most patients (48.8% were below 1 year of age; male were affected more than female (2.4:1. The disorder was classified into two groups, i.e., congenital nephropathy and uropathy. There were 10 children with nephropathy, i.e., 4 with unilateral renal hypoplasia, 3 with polycystic kidney, and 3 with renal agenesis. In the uropathy group, 43 were with hypospadia, 22 with primary reflux vesicoureter, 18 with neurogenic bladder, and 17 with ureteropelvic junction obstruction. The complications found were urinary tract infection (71.2%, chronic renal failure (15.7%, hypertension (3.7%, and acute renal failure (1.5%. Consanguinity, familial disorders, maternal diseases, x-ray exposure and abortion efforts were found in a small proportion of patients. History of drug or herbs use in the first trimester of pregnancy was found in a large proportion of patients, mostly took analgesics (especially acetaminophen. In conclusion, uropathy disorders were much more common than congenital nephropathy. The most common complication was urinary tract infection, followed by chronic renal failure, hypertension, and acute renal failure.

  9. Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia

    NARCIS (Netherlands)

    Bellini, Carlo; Mazzella, Massimo; Arioni, Cesare; Campisi, Corradino; Taddei, Gioconda; Tomà, Paolo; Boccardo, Francesco; Hennekam, Raoul C.; Serra, Giovanni

    2003-01-01

    We report a female infant with congenital lymphedema, facial anomalies, intestinal lymphangiectasia consistent with a diagnosis of Hennekam syndrome. At birth the patient presented with severe respiratory distress due to nonimmune hydrops fetalis, a congenital chylothorax (CC), and pulmonary

  10. RISK FACTORS IN CHILD CONGENITAL MALFORMATIONS

    OpenAIRE

    Alina-Costina LUCA; Mirabela SUBOTNICU

    2015-01-01

    Congenital heart malformations are among the most common congenital malformations. Congenital heart malformations occur due to genetic and environmental factors during embryonic morphogenesis period of the heart. About 25% of these malformations are severe, requiring intervention immediately after birth or in infancy. Abnormalities of structure and function of the heart and great vessels are the consequence of teratogenic factors occurring between day 19 and 45 of gestation. (Yagel et al...

  11. Hip dysplasia and congenital hip dislocation

    Energy Technology Data Exchange (ETDEWEB)

    Lingg, G.; Nebel, G.; von Torklus, D.

    1981-11-01

    In human genetics and orthopedics quite different answers have been given to the question of hereditary transmission and frequency of hip dysplasia in families of children with congenital hip dislocation. We therefore have made roentgenometric measurements of 110 parents of children with congenital hip dislocation. In 25% we found abnormal flat acetabulae, whereas 12% had pathologic deep hips. This may propose a new concept of morphology of congenital hip dysplasia.

  12. Congenital pseudoarthrosis associated with venous malformation

    International Nuclear Information System (INIS)

    Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N.; Hamamy, H.; Al-Hadidi, S.

    2007-01-01

    Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

  13. Congenital pseudoarthrosis associated with venous malformation

    Energy Technology Data Exchange (ETDEWEB)

    Al-Hadidy, A.; Haroun, A.; Al-Ryalat, N. [Jordan University Hospital, Radiology Department, P.O. Box 340621, Amman (Jordan); Hamamy, H. [Endocrinology and Genetics, National Center for Diabetes, Amman (Jordan); Al-Hadidi, S. [Jordan University Hospital, Departments of Orthopedics, Amman (Jordan)

    2007-06-15

    Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. (orig.)

  14. Clinical and morphological characteristics of malformations in infants with congenital cytomegalovirus infection and congenital toxoplasmosis

    Directory of Open Access Journals (Sweden)

    L. Yu. Barycheva

    2015-01-01

    Full Text Available The results of following up infants with intrauterine infections and malformations were retrospectively analyzed. Infants with malformations were diagnosed as having congenital cytomegalovirus infection and congenital toxoplasmosis in 127 and 69 cases, respectively. The aim of the study was to characterize malformations in infants with congenital cytomegalovirus and congenital Toxoplasma infections. The infants with malformations in congenital cytomegalovirus infection were found to have higher mortality rates (61,4% than those with congenital toxoplasmosis (34,8%. Postmortem analysis indicated that there was a predominance of embryopathies in infants with congenital cytomegalovirus infection and that of fetopathies in those with congenital toxoplasmosis. The dead infants with congenital cytomegalovirus infection had more commonly developed visceral defects, including heart diseases, pneumopathies, gastrointestinal and genitourinary abnormalities; fetopathies of the central nervous system and eye were prevalent in congenital toxoplasmosis. The surviving children with congenital toxoplasmosis were more frequently observed to have disabling CNS and ocular sequels as obstructive hydrocephalus, infantile cerebral palsy, complete or partial blindness, and cerebrasthenic disorders than those with congenital cytomegalovirus infection. 

  15. Congenital heart defects and extracardiac malformations.

    Science.gov (United States)

    Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

    2013-06-01

    To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

  16. Congenital hypothyroidism presenting with postpartum bradycardia

    International Nuclear Information System (INIS)

    Kara, S.; Tayman, C.; Tonbul, A.; Tatli, M.; Andiran, N.; Turkay, S.

    2013-01-01

    Congenital hypothyroidism is a clinical condition characterized by lack of thyroid hormone because of thyroid gland developmental and thyroid hormone biosynthesis disorders. The most common cause of permanent hypothyroidism is congenital factors. Prompt diagnosis is critical. However, overt signs of hypothyroidism are rarely present at birth, and 95% of affected babies are asymptomatic. Hypoxemia, apnea, acidosis, increased intracranial pressure, vagal stimulus and central nerve system abnormalities represent the most common causes of bradycardia in the neonate. Bradycardia associated with congenital hypothyroidism is very rare. In this paper, a case of severe congenital hypothyroidism, induced by maternal blocker antibodies, who presented with bradycardia, is reported. (author)

  17. Major congenital anomalies in a Danish region

    DEFF Research Database (Denmark)

    Garne, Ester; Hansen, Anne Vinkel; Birkelund, Anne Sofie

    2014-01-01

    INTRODUCTION: This study describes the prevalence of congenital anomalies and changes over time in birth outcome, mortality and chronic maternal diseases. MATERIAL AND METHODS: This study was based on population data from the EUROCAT registry covering the Funen County, Denmark, 1995...... mortality decreased significantly over time for cases with major congenital anomalies (p congenital anomaly cases, 8% had a registration of one of these chronic maternal diseases......: diabetes, epilepsy, mental disorder, thyroid disease, asthma, or inflammatory bowel disease. Medication for these conditions accounted for 46% of maternal drug use. CONCLUSION: Maternal morbidity and use of potentially teratogenic medication have increased among congenital anomaly cases. Foetal and infant...

  18. Congenital rubella syndrome and delayed manifestations

    DEFF Research Database (Denmark)

    Dammeyer, Jesper Herup

    2010-01-01

    Objective: Several hypotheses of different medical and psychological delayed manifestations among people who have congenital rubella syndrome (CRS) have been discussed. This study tests some of these hypotheses of delayed manifestations. Methods: Gathering information about 35 individuals who hav...... which people with CRS face must primarily be understood in relation to congenital deafblindness and dual sensory and communicative deprivation....... CRS and who are congenitally deafblind. Results: None of the hypotheses could be confirmed when individuals with CRS were compared to a control group of individuals who were congenital deafblind with different aetiology than CRS. Conclusions: This study concludes that those health related problems...

  19. The March of Extrahepatic Collaterals: Analysis of Blood Supply to Hepatocellular Carcinoma Located in the Bare Area of the Liver After Chemoembolization

    International Nuclear Information System (INIS)

    Miyayama, Shiro; Yamashiro, Masashi; Okuda, Miho; Yoshie, Yuichi; Nakashima, Yoshiko; Ikeno, Hiroshi; Orito, Nobuaki; Matsui, Osamu

    2010-01-01

    The purpose of this study was to evaluate changes in vascular supply to hepatocellular carcinoma (HCC) located in the bare area of the liver in patients who were mainly treated with chemoembolization. Twenty-six patients with HCC showing a mean diameter of 3.1 ± 1.4 cm (mean ± standard deviation) were mainly treated with chemoembolization. All patients underwent 2.7 ± 2.3 chemoembolization sessions over 40.1 ± 25.2 months. Tumor feeding branches demonstrated in each chemoembolization session were retrospectively evaluated. Initially, 18 tumors (59.2%) were supplied by the hepatic artery (H) and 8 (30.8%) by both the hepatic and the extrahepatic arteries (H + C). Fourteen tumors (53.8%) recurred at the posterior aspect of the tumor and were supplied by H (n = 4), H + C (n = 5), and extrahepatic collaterals (C) (n = 5). Several tumors recurred despite repeated chemoembolization, and these were supplied by H (n = 1), H + C (n = 7), and C (n = 2) at the second recurrence, by H (n = 1), H + C (n = 2), and C (n = 3) at the third, by H + C (n = 2) and C (n = 2) at the fourth, by H + C (n = 2) and C (n = 2) at the fifth, and by H (n = 1) and C (n = 1) at the sixth. One tumor was supplied by H at the seventh and by H + C at the eighth recurrence. As the number of local recurrences increased, the feeding vessel shifted from H to C. Especially, the right inferior phrenic artery (IPA) and renal capsular artery (RCA) supplied the tumor early, while the small right RCAs, adrenal arteries, and intercostal and lumbar artery supplied late recurrences in turns. In conclusion, HCCs located in the bare area are frequently supplied by extrahepatic vessels initially, while recurrence after chemoembolization is mainly due to extrahepatic blood supply. The right IPA and RCA are common feeding vessels demonstrated early, while other extrahepatic collateral supply from the retroperitoneal circulation occurs in turns during the later course.

  20. Prominent porto-systemic collateral pathways in patients with portal hypertension: demonstration by gadolinium-enhanced magnetic resonance angiography; Vias colaterais porto-sistemicas exuberantes em portadores de hipertensao portal: demonstracao pela angiografia por ressonancia magnetica com gadolinio

    Energy Technology Data Exchange (ETDEWEB)

    Caldana, Rogerio Pedreschi; Bezerra, Alexandre Araujo Sergio; Cecin, Alexnadre Oliveira; Souza, Luis Ronan Marques Ferreira de; Goldman, Susan Menasce; D' Ippolito, Giuseppe; Szejnfeld, Jacob [Universidade Federal de Sao Paulo (UNIFESP/EPM), Sao Paulo, SP (Brazil). Dept. de Diagnostico por Imagem]. E-mail: rogercal@uol.com.br

    2003-03-01

    To demonstrate the usefulness of gadolinium-enhanced magnetic resonance angiography in the evaluation of prominent porto-systemic collateral pathways. We reviewed the images from 40 patients with portal hypertension studied with gadolinium-enhanced magnetic resonance angiography and selected illustrative cases of prominent porto-systemic collateral pathways. The scans were performed using high field equipment (1.5 Tesla) and a 3 D volume technique. Image were obtained after intravenous injection of paramagnetic contrast media using a power injector. Magnetic resonance angiography demonstrated with precision the porto-systemic collateral pathways, particularly when investigating extensive territories or large vessels. The cases presented show the potential of this method in the investigation of patients with portal hypertension. Gadolinium-enhanced magnetic resonance angiography is a useful method for the evaluation of patients with portal hypertension and prominent collateral pathways. (author)

  1. A rare combination: congenital factor VII deficiency with Chiari malformation.

    Science.gov (United States)

    Bay, Ali; Aktekin, Elif; Erkutlu, Ibrahim

    2015-12-01

    Congenital factor (VII) deficiency is a rare bleeding disorder. We present a patient with congenital FVII deficiency and congenital hydrocephalus who underwent a ventriculoperitoneal shunt operation and needed no prophylaxis after the procedure.

  2. Congenital hydrocephalus in clinical practice : A genetic diagnostic approach

    NARCIS (Netherlands)

    Verhagen, J. M. A.; Schrander-Stumpel, C. T. R. M.; Krapels, P. C.; de Die-Smulders, C. E. M.; van Lint, F. H. M.; Willekes, C.; Weber, J. W.; Gavilanes, A. W. D.; Macville, M. V. E.; Stegmann, A. P. A.; Engelen, J. J. M.; Bakker, J.; Vos, Y. J.; Frints, S. G. M.

    2011-01-01

    Congenital hydrocephalus is a common and often disabling disorder. The etiology is very heterogeneous. Little is known about the genetic causes of congenital hydrocephalus. A retrospective survey was performed including patients with primary congenital hydrocephalus referred to the Department of

  3. Human extrahepatic portal vein obstruction correlates with decreased factor VII and protein C transcription but increased hepatocyte proliferation.

    Science.gov (United States)

    Chiu, Bill; Melin-Aldana, Hector; Superina, Riccardo A

    2007-10-01

    A 3-year-old girl developed extrahepatic portal vein obstruction (EHPVO) after a liver transplant. She had sequelae of portal hypertension that required another transplantation. The circumstances allowed for comparison of liver-dependent coagulation factor production between the second donor liver and the explanted liver with EHPVO. Liver samples from the explanted first graft and the second transplant were obtained. Fresh tissue was used to perform reverse transcription-polymerase chain reaction with primers against factors V, VII, as well as VIII, protein C, and paraffin-embedded sections for hepatocyte proliferation using Ki-67 antibody as well as for apoptosis using TUNEL assay. The transcription of factor VII and that of protein C were decreased in the explant as compared with the newly transplanted liver (factor VII, 77% of the donor; protein C, 88% of the donor). The transcription of factor V and that of factor VIII were unchanged. The explant had a greater percentage of proliferating hepatocytes than the new organ (0.85% +/- 0.75% vs 0.11% +/- 0.21%). The percentage of apoptotic cells was similar between the 2 livers (0.09% +/- 0.13% vs 0.09% +/- 0.13%). Idiopathic EHPVO is associated with a reduction in liver-dependent coagulation factor transcription and an increase in hepatocyte proliferation. Portal blood flow deprivation alters hepatic homeostasis and initiates mechanisms that attempt to restore liver-dependent coagulation factors.

  4. Mixed Large Cell Neuroendocrine Carcinoma and Adenocarcinoma with Spindle Cell and Clear Cell Features in the Extrahepatic Bile Duct

    Directory of Open Access Journals (Sweden)

    John Wysocki

    2014-01-01

    Full Text Available Mixed adenoneuroendocrine carcinomas, spindle cell carcinomas, and clear cell carcinomas are all rare tumors in the biliary tract. We present the first case, to our knowledge, of an extrahepatic bile duct carcinoma composed of all three types. A 65-year-old man with prior cholecystectomy presented with painless jaundice, vomiting, and weight loss. CA19-9 and alpha-fetoprotein (AFP were elevated. Cholangioscopy revealed a friable mass extending from the middle of the common bile duct to the common hepatic duct. A bile duct excision was performed. Gross examination revealed a 3.6 cm intraluminal polypoid tumor. Microscopically, the tumor had foci of conventional adenocarcinoma (CK7-positive and CA19-9-postive surrounded by malignant-appearing spindle cells that were positive for cytokeratins and vimentin. Additionally, there were separate areas of large cell neuroendocrine carcinoma (LCNEC. Foci of clear cell carcinoma merged into both the LCNEC and the adenocarcinoma. Tumor invaded through the bile duct wall with extensive perineural and vascular invasion. Circumferential margins were positive. The patient’s poor performance status precluded adjuvant therapy and he died with recurrent and metastatic disease 5 months after surgery. This is consistent with the reported poor survival rates of biliary mixed adenoneuroendocrine carcinomas.

  5. Detection of Epstein Barr Virus by Chromogenic In Situ Hybridization in cases of extra-hepatic biliary atresia

    Directory of Open Access Journals (Sweden)

    Farahmand Fatemeh

    2008-04-01

    Full Text Available Abstract Introduction Extra-hepatic biliary atresia (EHBA is an important cause of neonatal cholestasis. Several infectious agents have been proposed as etiologic factors such as Rotavirus and Reovirus. There is limited data on the role of Epstein Barr virus (EBV infection in EHBA, so we decided to study the presence of EBV virus in a series of 16 proven EHBA cases by Chromogenic in situ hybridization (CISH technique. Methods In the current study a total of 16 liver wedge biopsies of proven cases of EHBA were selected in a period of 4 years. CISH staining for EBV-encoded RNA (EBER transcript was performed. Results The review of H&E-stained slides of liver biopsies revealed fibrosis and marked ductular proliferation. In CISH-stained slides, EBV trace was observed in hepatocytes in two cases and in biliary epithelium in one case of EHBA. Discussion Considering the association of hepatitis with the Epstein-Barr virus in later life, it is likely that EBV hepatitis and its complications occur in the neonatal/perinatal period. Since EHBA is a relatively rare disease, a similar study on wedge biopsies of this number of proven cases of EHBA has not been performed to date. Current observation proposes the need for a study of larger series and employing other methods for confirming the etiologic role of EBV in EHBA cases.

  6. Isoferritins in rat Kupffer cells, hepatocytes, and extrahepatic macrophages. Biosynthesis in cell suspensions and cultures in response to iron

    International Nuclear Information System (INIS)

    Doolittle, R.L.; Richter, G.W.

    1981-01-01

    Cultures of Kupffer cells and of hepatocytes, prepared from single rat livers, synthesized ferritin protein equally efficiently. In culture but not in suspension, both sorts of cells responded significantly to stimulation with iron by increased ferritin synthesis. As determined by isoelectric focusing, the isoferritin profiles of newly synthesized 14 -labeled Kupffer cell and hepatocyte ferritin were identical, each having three bands. However, unlabeled ferritin, extracted from nonparenchymal liver cells (mainly Kupffer and endothelial cells) of iron-loaded rats, contained an acidic isoferritin that was not present in hepatocyte ferritin. Investigation of ferritin synthesis in cultured peritoneal and alveolar macrophages yielded similar results. The isofocusing profile of newly synthesized peritoneal macrophage ferritin was indistinguishable from the profile of fresh Kupffer cell or hepatocyte ferritin. Thus, the three isoferritins common to Kupffer cells, hepatocytes, and extrahepatic macrophages are neither cell- nor tissue-specific. However, modifications on intracellular storage may affect the isofocusing properties. The findings, although consistent with the LnH24-n subunit model of ferritin protein, indicate identical restrictive genomic control of the H:L ratios in these sorts of cells. Further, they make it probable that Kupffer cell ferritin iron, originating by endogenous synthesis, is the principal source of Kupffer cell hemosiderin iron

  7. Multidetector CT venography and contrast-enhanced MR venography of the inferior mesenteric vein in paediatric extrahepatic portal vein obstruction

    International Nuclear Information System (INIS)

    Chennur, Vikash SrinivasaiahSetty; Sharma, Raju; Gamanagatti, Shivanand; Gupta, Arun Kumar; Bhatnagar, Veereshwar; Vishnubhatla, Sreenivas

    2011-01-01

    Extrahepatic portal vein obstruction (EHPVO) is a common cause of paediatric portal hypertension and the only permanent treatment is shunt surgery. The inferior mesenteric vein (IMV) is a portal venous channel that can be used for the shunt when the splenic vein/superior mesenteric vein is thrombosed or when a lienorenal shunt is not possible. To compare MDCT venography (MDCTV) and contrast-enhanced MR venography (CEMRV) for visualisation of the IMV in children with EHPVO. This was a prospective study of 26 children (4-12 years, median 10 years) who underwent MDCTV and CEMRV. The IMV visualisation was graded using 4- and 2-point scales and the difference in visualisation was assessed by calculating the exact significance probability (P). The IMV was visualised in all children on MDCTV and 25/26 children on CEMRV (96%). The images were diagnostic in 23/26 children (88%) on MDCTV and in 18/26 (69%) children on CEMRV (P = 0.063). MDCTV and CEMRV are comparable for IMV visualisation with a tendency toward MDCTV being superior. (orig.)

  8. Multidetector CT venography and contrast-enhanced MR venography of the inferior mesenteric vein in paediatric extrahepatic portal vein obstruction

    Energy Technology Data Exchange (ETDEWEB)

    Chennur, Vikash SrinivasaiahSetty; Sharma, Raju; Gamanagatti, Shivanand; Gupta, Arun Kumar [All India Institute of Medical Sciences (AIIMS), Department of Radiology, New Delhi (India); Bhatnagar, Veereshwar [AIIMS, Department of Paediatric Surgery, New Delhi (India); Vishnubhatla, Sreenivas [AIIMS, Department of Biostatistics, New Delhi (India)

    2011-03-15

    Extrahepatic portal vein obstruction (EHPVO) is a common cause of paediatric portal hypertension and the only permanent treatment is shunt surgery. The inferior mesenteric vein (IMV) is a portal venous channel that can be used for the shunt when the splenic vein/superior mesenteric vein is thrombosed or when a lienorenal shunt is not possible. To compare MDCT venography (MDCTV) and contrast-enhanced MR venography (CEMRV) for visualisation of the IMV in children with EHPVO. This was a prospective study of 26 children (4-12 years, median 10 years) who underwent MDCTV and CEMRV. The IMV visualisation was graded using 4- and 2-point scales and the difference in visualisation was assessed by calculating the exact significance probability (P). The IMV was visualised in all children on MDCTV and 25/26 children on CEMRV (96%). The images were diagnostic in 23/26 children (88%) on MDCTV and in 18/26 (69%) children on CEMRV (P = 0.063). MDCTV and CEMRV are comparable for IMV visualisation with a tendency toward MDCTV being superior. (orig.)

  9. [Results of the mesoportal bypass (Rex shunt) in the treatment of idiopathic extrahepatic portal vein obstruction in children].

    Science.gov (United States)

    Domínguez Amillo, E; De la Torre Ramos, C; Andrés Moreno, A; Encinas Hernández, J L; Hernández Oliveros, F; López Santamaría, M

    2017-01-25

    Extrahepatic portal vein obstruction (EPVO) is the principal cause of portal hypertension in children. The objective of this study was to analyze the capacity of the surgical technique that creates a mesoportal shunt to treat changes caused by EPVO. Retrospective review of patients with idiopathic EPVO who underwent a mesoportal shunt and analysis of the changes in the number of leucocytes, platelets, prothrombin time and spleen size one year after the surgery. Twelve patients underwent surgery, out of which 10 had prior leukopenia, 11 thrombopenia, 9 longer prothrombin times and all had hypersplenism. One patient suffered a postoperative shunt thrombosis, was reoperated and underwent a change in the operative technique. The remaining patients (92%) have functioning shunts 4.3 ± 2.5 years after surgery, and none have suffered any episode of gastrointestinal bleeding. One year after surgery, there were significant changes in the number of platelets, prothrombin time and spleen size, with no significant changes in the number of leukocytes. However, the number of patients who went from a leukopenic to a normal state was significant, as happened with changes in prothrombin time. Mesoportal Rex shunt improves some of the disorders caused by portal hypertension in children suffering EPVO, with a high rate of surgical success. This technique should be of first choice in these patients.

  10. Outcome of self-expanding metal stenting followed by radiation therapy for unresectable extrahepatic bile duct cancer

    International Nuclear Information System (INIS)

    Jin Jing; Gao Li; Yu Zihao; Han Jianzhu; Zhai Renyou

    2007-01-01

    Objective: To retrospectively analyze the efficacy for self-expanding metal stenting followed by radiation therapy for unresectable extrahepatic bile duct cancer. Methods: From Jan 1996 to Sep 2002, 17 patients were treated with self-expanding metal stenting followed by Ir 192 high dose brachytherapy (brachytherapy group, n=5)and by external beam radiation therapy(external radiation therapy group, n=9). Brachytherapy was delivered on the same day or the following day after stenting with the total dose of DT 20- 30 Gy, twice daily, for 2-3 days. The median total dosage of external radiation therapy was 48 Gy (14-66 Gy), 2 Gy/fraction. Results: Jaundice was released after metal stenting from 71.4% to 14.3% (P=0.001), showing no significant difference between brachytherapy and external radiation therapy in terms of jaundice release(χ 2 =0.21, P=0.65). The median survival period(5-35 months)for whole groups was 12 months 1-, 2-year survival rates were 40.8% and 8.16%, respectively. No significant difference was shown for patients received either brachytherapy or external radiation therapy for 8 months, but with 1,2 year survival rate as 40.0%, 13.3%, respectively (χ 2 =1.10, P=0.29). Conclusions: Self-expanding metal stenting is able to release jaundice significantly and followed by high dose radiation therapy may further prolong the survival. (authors)

  11. Technical and clinical outcome of transjugular intrahepatic portosystemic stent shunt: Bare metal stents (BMS) versus viatorr stent-grafts (VSG)

    Energy Technology Data Exchange (ETDEWEB)

    Sommer, Christof M., E-mail: cmsommer@gmx.com [Department of Diagnostic and Interventional Radiology, University Hospital Heidelberg, INF 110, 69120 Heidelberg (Germany); Gockner, Theresa L.; Stampfl, Ulrike; Bellemann, Nadine [Department of Diagnostic and Interventional Radiology, University Hospital Heidelberg, INF 110, 69120 Heidelberg (Germany); Sauer, Peter; Ganten, Tom [Department of Gastroenterology, University Hospital Heidelberg, Heidelberg (Germany); Weitz, Juergen [Department of General, Abdominal and Transplantation Surgery, University Hospital Heidelberg, Heidelberg (Germany); Kauczor, Hans U.; Radeleff, Boris A. [Department of Diagnostic and Interventional Radiology, University Hospital Heidelberg, INF 110, 69120 Heidelberg (Germany)

    2012-09-15

    Highlights: ► Twelve month mean number of TIPS revisions per patient was significantly lower in VSG. ► First TIPS revision was performed significantly later in the VSG. ► There was no significant difference of hepatic encephalopathy in both study groups. -- Abstract: Purpose: To compare retrospectively angiographical and clinical results in patients undergoing transjugular intrahepatic portosystemic stent-shunt (TIPS) using BMS or VSG. Materials and methods: From February 2001 to January 2010, 245 patients underwent TIPS. From those, 174 patients matched the inclusion criteria with elective procedures and institutional follow-up. Group (I) consisted of 116 patients (mean age, 57.0 ± 11.1 years) with BMS. Group (II) consisted of 58 patients with VSG (mean age, 53.5 ± 16.1 years). Angiographic and clinical controls were scheduled at 3, 6 and 12 months, followed by clinical controls every 6 months. Primary study goals included hemodynamic success, shunt patency as well as time to and number of revisions. Secondary study goals included clinical success. Results: Hemodynamic success was 92.2% in I and 91.4% in II (n.s.). Primary patency was significantly higher in II compared to I (53.8% after 440.4 ± 474.5 days versus 45.8% after 340.1 ± 413.8 days; p < 0.05). The first TIPS revision was performed significantly later in II compared to I (288.3 ± 334.7 days versus 180.1 ± 307.0 days; p < 0.05). In the first angiographic control, a portosystemic pressure gradient ≥15 mmHg was present in 73.9% in I and in 39.4% in II (p < 0.05). Clinical success was 73.7–86.2% after 466.3 ± 670.1 days in I and 85.7–90.5% after 617.5 ± 642.7 days in II (n.s.). Hepatic encephalopathy was 37.5% in I and 36.5% in II (n.s.). Conclusion: VSG increased primary shunt patency as well as decreased time to and number of TIPS revisions. There was a trend of higher clinical success in VSG without increased hepatic encephalopathy.

  12. Technical and clinical outcome of transjugular intrahepatic portosystemic stent shunt: Bare metal stents (BMS) versus viatorr stent-grafts (VSG)

    International Nuclear Information System (INIS)

    Sommer, Christof M.; Gockner, Theresa L.; Stampfl, Ulrike; Bellemann, Nadine; Sauer, Peter; Ganten, Tom; Weitz, Juergen; Kauczor, Hans U.; Radeleff, Boris A.

    2012-01-01

    Highlights: ► Twelve month mean number of TIPS revisions per patient was significantly lower in VSG. ► First TIPS revision was performed significantly later in the VSG. ► There was no significant difference of hepatic encephalopathy in both study groups. -- Abstract: Purpose: To compare retrospectively angiographical and clinical results in patients undergoing transjugular intrahepatic portosystemic stent-shunt (TIPS) using BMS or VSG. Materials and methods: From February 2001 to January 2010, 245 patients underwent TIPS. From those, 174 patients matched the inclusion criteria with elective procedures and institutional follow-up. Group (I) consisted of 116 patients (mean age, 57.0 ± 11.1 years) with BMS. Group (II) consisted of 58 patients with VSG (mean age, 53.5 ± 16.1 years). Angiographic and clinical controls were scheduled at 3, 6 and 12 months, followed by clinical controls every 6 months. Primary study goals included hemodynamic success, shunt patency as well as time to and number of revisions. Secondary study goals included clinical success. Results: Hemodynamic success was 92.2% in I and 91.4% in II (n.s.). Primary patency was significantly higher in II compared to I (53.8% after 440.4 ± 474.5 days versus 45.8% after 340.1 ± 413.8 days; p < 0.05). The first TIPS revision was performed significantly later in II compared to I (288.3 ± 334.7 days versus 180.1 ± 307.0 days; p < 0.05). In the first angiographic control, a portosystemic pressure gradient ≥15 mmHg was present in 73.9% in I and in 39.4% in II (p < 0.05). Clinical success was 73.7–86.2% after 466.3 ± 670.1 days in I and 85.7–90.5% after 617.5 ± 642.7 days in II (n.s.). Hepatic encephalopathy was 37.5% in I and 36.5% in II (n.s.). Conclusion: VSG increased primary shunt patency as well as decreased time to and number of TIPS revisions. There was a trend of higher clinical success in VSG without increased hepatic encephalopathy

  13. Adrenomyeloneuropathy associated with congenital cataract

    International Nuclear Information System (INIS)

    Komori, Tetsuo; Nagashima, Toshiko; Hirose, Kazuhiko; Tanabe, Hitoshi; Tsubaki, Tadao

    1988-01-01

    Two cases of adrenomyeloneuropathy (AMN) in a family and the results of their MRI study are reported. Case 1, a 24-year-old male proband, was admitted to our hospital because of gait disturbance for three years. Bilateral cataracts were pointed out at birth, which required left side lenticotomy at age four. Neurological examinations on admission revealed a marked spastic paraparesis with pathological reflexes and a mild hypesthesia in the distal part of the left leg. No abnormal findings were detected in X-ray studies on the spine and spinal cord, electromyography and nerve conduction test. Serum very long chain fatty acids (VLCFAs) levels were apparently elevated, and the serum cortisol response to ACTH was low. Case 2, a 53-year-old woman, is the mother of Case 1 having a complaint of gait disturbance since age 51. She presented a mild spastic paraparesis with localized hypesthesia in the distal part of the both legs. She also had bilateral congenital cataracts. Her serum VLCFAs analysis indicated the intermediate levels between that of AMN and the normal control. Adrenal functions were normal. Cranial MRI (TR 2,000 msec/TE 80 msec) study disclosed high signal intensity areas in bilateral internal capsules in Case 1. These findings, suggesting the pathological change of dysmyelination, seemed to be well compatible with the clinical pictures. With these clinical findings and the laboratory data, these two cases were diagnosed as AMN. In addition, association of congenital cataract with AMN in both cases was characteristic in this family, which hasn't been reported in the literature. On regarding the genetic background of these two disorders, AMN and congenital cataract, it was speculated that each gene could be closely located on the same or very adjacent locus, possibly on Xq. (author)

  14. Association between portal vein pressure drop gradient after transjugular intrahepatic portosystemic shunt and clinical prognosis

    Directory of Open Access Journals (Sweden)

    XU Zhengguo

    2016-12-01

    Full Text Available ObjectiveTo investigate the association between portal vein pressure drop gradient in patients with cirrhotic portal hypertension treated by transjugular intrahepatic portosystemic shunt (TIPS and clinical prognosis, as well as the ideal range of portal vein pressure drop. MethodsA total of 58 patients who underwent TIPS in Xinqiao Hospital of Third Military Medical University from November 2013 to December 2015 were enrolled. All the patients underwent TIPS and embolization of the gastric coronary vein and the short gastric veins, and the change intervals of portal vein pressure gradient were monitored. The follow-up time ranged from 3 days to 2 years, and the association of portal vein pressure drop gradient with postoperative liver function, splenic function, rebleeding rate, hepatic encephalopathy, and portal hypertensive gastrointestinal diseases was analyzed. The paired t-test was used for comparison of parameters before and after treatment. ResultsThe patients had a significant reduction in liver function on day 3 after surgery. At 2 month after surgery, the levels of TBil was rised and had significant changes[(49.81±27.82μmol/L vs (31.64±17.67 μmol/L,t=5.372,P<0.001]. At 6 months after surgery, red blood cell count and platelet count had no significant changes,but,white blood cell count was reduced[(3.79±1.37)×109/L vs (4.57±2.24×109/L,t=2.835,P=0.006]. There was a 23% reduction in portal vein pressure after surgery (from 30.62±3.56 mmHg before surgery to 21.21±2.90 mmHg after surgery, t=23.318,P<0.001. All the patients had varying degrees of relief of gastrointestinal symptoms associated with portal vein hypertension, such as abdominal distension, poor appetite, and diarrhea. Of all patients, none experienced in-stent restenosis or occlusion and 13 experienced hepatic encephalopathy after surgery, which tended to occur at the time when postoperative portal vein pressure was reduced to 14.7-25.7 mmHg, i

  15. Surgery for congenital choanal atresia.

    Science.gov (United States)

    Cedin, Antonio C; Atallah, Alvaro N; Andriolo, Régis B; Cruz, Oswaldo L; Pignatari, Shirley N

    2012-02-15

    Congenital choanal atresia is a rare abnormality characterized by unilateral or bilateral lack of patency of the posterior end of the nasal cavity. With an incidence of 1:5000 to 1:8000 births, it is twice as prevalent in females as it is in males. Surgical procedures aim to provide adequate functional choanal patency and a low rate of restenosis, avoid harm to any structure in development, enable shorter surgery and hospitalization times, and minimize morbidity and mortality. To evaluate the effectiveness and safety of the available surgical techniques for the treatment of congenital choanal atresia in patients with unilateral and bilateral atresia. We searched the Cochrane Ear, Nose and Throat Disorders Group Trials Register; the Cochrane Central Register of Controlled Trials (CENTRAL); PubMed; EMBASE; CINAHL; Web of Science; BIOSIS Previews; Cambridge Scientific Abstracts; ISRCTN and additional sources for published and unpublished trials. The date of the search was 31 January 2011. We planned to include parallel randomized or quasi-randomized controlled trials testing surgical approaches for the treatment of congenital atresia (irrespective of gender and age) that evaluated normal/adequate respiratory function (self reported or preserved nasal airway) and restenosis as the main primary outcomes. We did not consider reoperation and non-congenital atresia (e.g. traumatic, iatrogenic atresias) for inclusion. Three review authors independently assessed the titles and abstracts of the identified articles to determine potential relevance. For dichotomous and continuous variables, we planned to calculate risk ratios (relative risks; RR) and mean differences (MD) with 95% confidence intervals (CI), respectively. We planned to use the random-effects model since we were expecting substantial clinical and methodological heterogeneity. No randomized controlled trials were identified. From the 120 reports retrieved using our search strategy, 46 primary studies had the

  16. Laboratory Diagnosis of Congenital Toxoplasmosis

    Science.gov (United States)

    Pomares, Christelle

    2016-01-01

    Recent studies have demonstrated that screening and treatment for toxoplasmosis during gestation result in a decrease of vertical transmission and clinical sequelae. Early treatment was associated with improved outcomes. Thus, laboratory methods should aim for early identification of infants with congenital toxoplasmosis (CT). Diagnostic approaches should include, at least, detection of Toxoplasma IgG, IgM, and IgA and a comprehensive review of maternal history, including the gestational age at which the mother was infected and treatment. Here, we review laboratory methods for the diagnosis of CT, with emphasis on serological tools. A diagnostic algorithm that takes into account maternal history is presented. PMID:27147724

  17. Schizencephaly/congenital cerebral clefts

    International Nuclear Information System (INIS)

    Friedman, H.; Naidich, T.P.

    1987-01-01

    Schizencephaly (from the Greek meaning ''split brain''), is a term developed in the 1940s to explain symmetric clefts in the brain seen at autopsy in children with histories of severe neurologic defects. Use of the term has been expanded to include a variety of cerebral clefts. A review of the experience at Children's Memorial Hospital as well as case materials made available to the authors are presented, including CT, MR imaging, and US findings. Theories of etiology and pathogenesis of these congenital clefts, associated anomalies, and the spectrum of appearance of these clefts are discussed

  18. Genetically caused congenital anomalies of reproductive system

    Directory of Open Access Journals (Sweden)

    L. F. Kurilo

    2013-01-01

    Full Text Available Classification of congenital disorders, their frequency of occurrence in populations, and some terminology questions discussed in the review. Genetically caused congenital anomalies of reproductive system are outlined. Full information about genetic syndromes is stated in the book: Kozlova S.I., Demikova N.S. Hereditary syndromes and genetic counseling. M., 2007.

  19. Congenital segmental dilatation of the colon

    African Journals Online (AJOL)

    Congenital segmental dilatation of the colon is a rare cause of intestinal obstruction in neonates. We report a case of congenital segmental dilatation of the colon and highlight the clinical, radiological, and histopathological features of this entity. Proper surgical treatment was initiated on the basis of preoperative radiological ...

  20. Congenital Heart Diseases associated with Identified Syndromes ...

    African Journals Online (AJOL)

    Recognised syndromes were seen in 69(68%) cases. Down syndrome with 54 children contributed 78.3% of those with known syndromes. Other identified syndromes and associations were Marfan's, Noonan's, Edwards, Prune Belly, Apert, Ellis-van creveld syndrome and congenital rubella syndrome. Congenital heart ...

  1. The genetic landscape of familial congenital hydrocephalus.

    Science.gov (United States)

    Shaheen, Ranad; Sebai, Mohammed Adeeb; Patel, Nisha; Ewida, Nour; Kurdi, Wesam; Altweijri, Ikhlass; Sogaty, Sameera; Almardawi, Elham; Seidahmed, Mohammed Zain; Alnemri, Abdulrahman; Madirevula, Sateesh; Ibrahim, Niema; Abdulwahab, Firdous; Hashem, Mais; Al-Sheddi, Tarfa; Alomar, Rana; Alobeid, Eman; Sallout, Bahauddin; AlBaqawi, Badi; AlAali, Wajeih; Ajaji, Nouf; Lesmana, Harry; Hopkin, Robert J; Dupuis, Lucie; Mendoza-Londono, Roberto; Al Rukban, Hadeel; Yoon, Grace; Faqeih, Eissa; Alkuraya, Fowzan S

    2017-06-01

    Congenital hydrocephalus is an important birth defect, the genetics of which remains incompletely understood. To date, only 4 genes are known to cause Mendelian diseases in which congenital hydrocephalus is the main or sole clinical feature, 2 X-linked (L1CAM and AP1S2) and 2 autosomal recessive (CCDC88C and MPDZ). In this study, we aimed to determine the genetic etiology of familial congenital hydrocephalus with the assumption that these cases represent Mendelian forms of the disease. Exome sequencing combined, where applicable, with positional mapping. We identified a likely causal mutation in the majority of these families (21 of 27, 78%), spanning 16 genes, none of which is X-linked. Ciliopathies and dystroglycanopathies were the most common etiologies of congenital hydrocephalus in our cohort (19% and 26%, respectively). In 1 family with 4 affected members, we identified a homozygous truncating variant in EML1, which we propose as a novel cause of congenital hydrocephalus in addition to its suggested role in cortical malformation. Similarly, we show that recessive mutations in WDR81, previously linked to cerebellar ataxia, mental retardation, and disequilibrium syndrome 2, cause severe congenital hydrocephalus. Furthermore, we confirm the previously reported candidacy of MPDZ by presenting a phenotypic spectrum of congenital hydrocephalus associated with 5 recessive alleles. Our study highlights the importance of recessive mutations in familial congenital hydrocephalus and expands the locus heterogeneity of this condition. Ann Neurol 2017;81:890-897. © 2017 American Neurological Association.

  2. Congenital lobar emphysema: Is surgery routinely necessary ...

    African Journals Online (AJOL)

    Congenital lobar emphysema (CLE) is a rare congenital abnormality characterised by overinflation of a pulmonary lobe. Its aetiology is unknown. The management of CLE has traditionally been surgical. A newborn boy with a birthweight of 2.5 kg was delivered at full-term by caesarian section due to food delivery.

  3. Congenital Diverticular Disease of the Entire Colon

    Directory of Open Access Journals (Sweden)

    A. Patel

    2013-01-01

    Full Text Available Congenital or true colonic diverticulosis is a rare condition typified by the preservation of the colonic wall architecture within the diverticular outpouching. Cases of multiple jejunal diverticula have been reported as well as cases of solitary giant diverticula of the colon. There have been no reports in the literature of pancolonic congenital diverticulosis.

  4. Congenital malformations in paediatric and neurosurgical practices ...

    African Journals Online (AJOL)

    Congenital malformations in paediatric and neurosurgical practices: problems and pattern (A preliminary report) ... Open Access DOWNLOAD FULL TEXT ... over a 5-year period (1998 to 2002) with congenital anomalies to the Paediatric Surgery and Neurosurgery units of the University Teaching Hospital, Ilorin, Nigeria.

  5. Cyanotic congenital heart disease and atherosclerosis

    DEFF Research Database (Denmark)

    Tarp, Julie Bjerre; Jensen, Annette Schophuus; Engstrøm, Thomas

    2017-01-01

    Improved treatment options in paediatric cardiology and congenital heart surgery have resulted in an ageing population of patients with cyanotic congenital heart disease (CCHD). The risk of acquired heart disease such as atherosclerosis increases with age.Previous studies have speculated whether...

  6. Genetics Home Reference: congenital central hypoventilation syndrome

    Science.gov (United States)

    ... Kravis EM, Zhou L, Rand CM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B mutations and phenotype. Am J Respir Crit ... BA, Leurgans SE, Berry-Kravis EM, Weese-Mayer DE. Congenital central hypoventilation syndrome: PHOX2B genotype determines risk for sudden death. Pediatr ...

  7. The changing epidemiology of congenital heart disease

    NARCIS (Netherlands)

    van der Bom, Teun; Zomer, A. Carla; Zwinderman, Aeilko H.; Meijboom, Folkert J.; Bouma, Berto J.; Mulder, Barbara J. M.

    2011-01-01

    Congenital heart disease is the most common congenital disorder in newborns. Advances in cardiovascular medicine and surgery have enabled most patients to reach adulthood. Unfortunately, prolonged survival has been achieved at a cost, as many patients suffer late complications, of which heart

  8. Health in adults with congenital heart disease

    NARCIS (Netherlands)

    Cuypers, Judith A. A. E.; Utens, Elisabeth M. W. J.; Roos-Hesselink, Jolien W.

    2016-01-01

    Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many

  9. Health in adults with congenital heart disease.

    Science.gov (United States)

    Cuypers, Judith A A E; Utens, Elisabeth M W J; Roos-Hesselink, Jolien W

    2016-09-01

    Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many adults with congenital heart disease encounter late complications. Late morbidity can be related to the congenital heart defect itself, but may also be the consequence of the surgical or medical treatment or longstanding alterations in hemodynamics, neurodevelopment and psychosocial development. This narrative review describes the cardiac and non-cardiac long-term morbidity in the adult population with congenital heart disease. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  10. Trans-jugular intrahepatic porto-systemic shunt placement for refractory ascites: a 'real-world' UK health economic evaluation.

    Science.gov (United States)

    Parker, Matthew J; Guha, Neil; Stedman, Brian; Hacking, Nigel; Wright, Mark

    2013-07-01

    To assess the benefit of trans-jugular intrahepatic porto-systemic shunt (TIPS) placement for refractory ascites. A retrospective observational study of all patients undergoing TIPS for refractory ascites in our hospital between 2003 and 2012. Secondary care. Cirrhotic patients with refractory ascites. We examined direct real-world (National Health Service) health related costs in the year before and after the TIPS procedure took place. Data were collected relating to the need for reintervention and hepatic encephalopathy. Data were available for 24 patients who underwent TIPS for refractory ascites (86% of eligible patients). TIPS was technically successful in all cases. Mean number of bed days in the year prior to TIPS was 30.3 and 14.3 in the year following (p=0.005). No patient had ascites at the end of the year after the TIPS with less requirement for paracentesis over the course of the year (p<0.001). Mean reduction in cost was £2759 per patient. TIPS was especially cost-effective in patients requiring between 6 and 12 drains per year with a mean saving of £9204 per patient. TIPS is both a clinically effective and economically advantageous therapeutic option for selected patients with refractory ascites.

  11. Long-term outcome following trans-jugular intrahepatic portosystemic shunt for variceal bleeding due to portal hypertension

    International Nuclear Information System (INIS)

    Cui Jinguo; Zhang Shutian; Feng Zitan; Zhou Guifen; Pan Xinyuan; Liang Zhihui

    2002-01-01

    Objective: To study the 6-year outcome following trans-jugular intrahepatic portosystemic shunt (TIPSS) for variceal bleeding due to portal hypertension. Methods: 65 patients, 51 males, 14 females, aged 35-72 years old with averaged 4.5 years, have been undergone TIPSS because of portal hypertension due to cirrhosis or Budd-Chiari syndrome. The portal pressures were measured before and after TIPSS. Follow-up study was done by color Doppler sonography or Barium esophageal radiography for 3 months to 6 years (averaged 18 months). Repeated interventional treatments were done in cases of restenosis of the shunts. Results: There were 0, 2, 10, 5, 0 cases of recurrent bleeding after 3 months, 6 months, 1 year, 2 year and 3-6 year following TIPSS respectively. Stenosis occurred in shunt paths due to thrombosis or smooth muscle cell proliferation or neo-intimal hyperplasia were relieved after thrombolytic therapy and repeated balloon angioplasty or stent plant among most of them. 2 were failed due to serious stenosis. 7 cases died, 2 of massive bleeding, 1 of the other cause and 4 of hepatic cancer. The other patients are getting well. Conclusions: Although there were very high rates of restenosis (34%), but most of them could be treated again with interventional therapy, and in kept patency effectively. TIPSS is a still practical valuable management for massive gastric bleeding

  12. Transjugular intrahepatic portosystemic shunt in patients with active variceal bleeding due to portal hypertension and portal vein thrombosis

    International Nuclear Information System (INIS)

    Shin, Hyun Woong; Ryeom, Hun Kyu; Lee, Sang Kwon; Lee, Jong Min; Kim, Young Sun; Suh, Kyung Jin; Kim, Tae Hun; Kim, Yong Joo

    1997-01-01

    To evaluate the feasibility and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in patients with active variceal bleeding due to liver cirrhosis and pre-existing portal vein thrombosis. Of a total of 123 patients who underwent TIPS, 14 patients with intractable variceal bleeding due to portal hypertension and portal vein thrombosis were included in this study. Noncavernomatous portal vein occlusion was seen in eight patients, and complete portal vein occlusion with cavernomatous trans-formation in six. For all patients, the methods used for TIPS placement were the same as those used in patients with patents portal veins. In seven of eight patients with noncavernomatous occlusion, right hepatic vein-right portal vein shunting was performed; in one with knoncavernomatous occlusion, a shunt was created between the right hepatic and left portal vein. In five of six patients with cavernomatous occlusion, the right hepatic and main portal vein were connected via a collateral vein. The procedures were technically successful in all except one patient. Immediate hemostatis was achieved after all technically successful procedures, and no significant complications were encountered. Minor complications were noted in six patients (three biliary tree punctures, one transperitoneal puncture, one splenic vein perforation, one hepatic subcapsular hematoma). TIPS is a technically feasible and hemodynamically effective procedure, even in patients with active variceal bleeding due to cirrhosis and complete portal vein occlusion

  13. Bleeding ectopic duodenal varix: use of a new microvascular plug (MVP) device along with transjugular intrahepatic portosystemic shunt (TIPSS).

    Science.gov (United States)

    Bhardwaj, Richa; Bhardwaj, Gaurav; Bee, Erik; Karagozian, Raffi

    2017-08-16

    Ectopic varices (ECV) occur along the gastrointestinal (GI) tract outside the common variceal sites and represent 2%-5% of all GI variceal bleeds with mortality rates up to 40%. Management is challenging because of inaccessibility and increased risk of rebleeding. We report what is to our knowledge the first clinical use of a new microvascular plug (MVP) with transjugular intrahepatic portosystemic shunt (TIPSS) for a bleeding duodenal varix (DV). A 68-year-old man presented with melena. Endoscopy demonstrated a grade II varix in the second part of the duodenum with red wale sign. TIPSS was performed and portogram revealed a single DV. Poststent placement venogram revealed a persistent varix and hence a 5-7 mm MVP was deployed. Subsequent imaging showed cessation of blood through the DV. The patient had no further bleeding. TIPSS with embolisation is an effective treatment for ECV. This MVP offers advantages due to its size and compatibility and can be redeployed in case of suboptimal placement. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  14. Endovascular Management of Refractory Hepatic Encephalopathy Complication of Transjugular Intrahepatic Portosystemic Shunt (TIPS): Comprehensive Review and Clinical Practice Algorithm

    International Nuclear Information System (INIS)

    Pereira, Keith; Carrion, Andres F.; Salsamendi, Jason; Doshi, Mehul; Baker, Reginald; Kably, Issam

    2016-01-01

    Transjugular intrahepatic portosystemic shunt (TIPS) has evolved as an effective intervention for treatment of complications of portal hypertension. The use of polytetrafluoroethylene-covered stents have improved the patency of the shunts and diminished the incidence of TIPS dysfunction. However, TIPS-related refractory hepatic encephalopathy (rHE) poses a significant challenge. Approximately 3–7 % of patients with TIPS develop rHE. Refractory hepatic encephalopathy is defined as a recurrent or persistent encephalopathy despite appropriate medical treatment. Hepatic encephalopathy can be an extremely debilitating complication that profoundly affects quality of life. The approach to management of patients with rHE is complex and typically requires collaboration between different specialties. Liver transplantation is the ultimate treatment for rHE; however, the ongoing shortage of organ donation markedly limits this treatment option. Alternative therapies such as shunt occlusion or reduction can control symptoms and serve as a ‘bridge’ therapy to liver transplantation. Therefore, interventional radiologists play a key role in the management of these patients by offering a variety of endovascular techniques. The purpose of this review is to highlight some of these endovascular techniques and to develop a therapeutic algorithm that can be applied in clinical practice for the management of rHE

  15. Endovascular Management of Refractory Hepatic Encephalopathy Complication of Transjugular Intrahepatic Portosystemic Shunt (TIPS): Comprehensive Review and Clinical Practice Algorithm

    Energy Technology Data Exchange (ETDEWEB)

    Pereira, Keith, E-mail: keithjppereira@gmail.com [Jackson Memorial Hospital/University of Miami Hospital, Department of Interventional Radiology (United States); Carrion, Andres F., E-mail: andres.carrionmonsa@jhsmiami.org [Jackson Memorial Hospital/University of Miami Hospital, Department of Hepatology (United States); Salsamendi, Jason, E-mail: JSalsamendi@med.miami.edu; Doshi, Mehul, E-mail: MDoshi@med.miami.edu; Baker, Reginald, E-mail: RBaker@med.miami.edu; Kably, Issam, E-mail: ikably@med.miami.edu [Jackson Memorial Hospital/University of Miami Hospital, Department of Interventional Radiology (United States)

    2016-02-15

    Transjugular intrahepatic portosystemic shunt (TIPS) has evolved as an effective intervention for treatment of complications of portal hypertension. The use of polytetrafluoroethylene-covered stents have improved the patency of the shunts and diminished the incidence of TIPS dysfunction. However, TIPS-related refractory hepatic encephalopathy (rHE) poses a significant challenge. Approximately 3–7 % of patients with TIPS develop rHE. Refractory hepatic encephalopathy is defined as a recurrent or persistent encephalopathy despite appropriate medical treatment. Hepatic encephalopathy can be an extremely debilitating complication that profoundly affects quality of life. The approach to management of patients with rHE is complex and typically requires collaboration between different specialties. Liver transplantation is the ultimate treatment for rHE; however, the ongoing shortage of organ donation markedly limits this treatment option. Alternative therapies such as shunt occlusion or reduction can control symptoms and serve as a ‘bridge’ therapy to liver transplantation. Therefore, interventional radiologists play a key role in the management of these patients by offering a variety of endovascular techniques. The purpose of this review is to highlight some of these endovascular techniques and to develop a therapeutic algorithm that can be applied in clinical practice for the management of rHE.

  16. Three-dimensional C-arm CT-guided transjugular intrahepatic portosystemic shunt placement: Feasibility, technical success and procedural time

    Energy Technology Data Exchange (ETDEWEB)

    Ketelsen, Dominik; Groezinger, Gerd; Maurer, Michael; Grosse, Ulrich; Horger, Marius; Nikolaou, Konstantin; Syha, Roland [University of Tuebingen, Department of Diagnostic and Interventional Radiology, Tuebingen (Germany); Lauer, Ulrich M. [University of Tuebingen, Internal Medicine I, Department of Gastroenterology, Hepatology and Infectious disease, Tuebingen (Germany)

    2016-12-15

    Establishment of transjugular intrahepatic portosystemic shunts (TIPS) constitutes a standard procedure in patients suffering from portal hypertension. The most difficult step in TIPS placement is blind puncture of the portal vein. This study aimed to evaluate three-dimensional mapping of portal vein branches and targeted puncture of the portal vein. Twelve consecutive patients suffering from refractory ascites by liver cirrhosis were included in this retrospective study to evaluate feasibility, technical success and procedural time of C-arm CT-targeted puncture of the portal vein. As a control, 22 patients receiving TIPS placement with fluoroscopy-guided blind puncture were included to compare procedural time. Technical success could be obtained in 100 % of the study group (targeted puncture) and in 95.5 % of the control group (blind puncture). Appropriate, three-dimensional C-arm CT-guided mapping of the portal vein branches could be achieved in all patients. The median number of punctures in the C-arm CT-guided study group was 2 ± 1.3 punctures. Procedural time was significantly lower in the study group (14.8 ± 8.2 min) compared to the control group (32.6 ± 22.7 min) (p = 0.02). C-arm CT-guided portal vein mapping is technically feasible and a promising tool for TIPS placement resulting in a significant reduction of procedural time. (orig.)

  17. Long Term Follow-up of a Transjugular Intrahepatic Portosystemic Shunt: A Comparison of Covered and Uncovered Stents

    Energy Technology Data Exchange (ETDEWEB)

    Joo, Seung Moon; Park, Jae Hyung; Kim, Hyo Cheol; Jae, Hwan Jun; Chung, Jin Wook [Seoul National University Hospital, Seoul (Korea, Republic of)

    2009-01-15

    To evaluate the long term patency of transjugular intrahepatic portosystemic shunts (TIPS) and to compare the patency rate of covered and uncovered stents in TIPS. The study population included 78 patients with portal hypertension that underwent TIPS between January 1999 and July 2007 at our institution using uncovered stents in 53 patients and covered stents in 25 patients. The primary and secondary patency rates of TIPS were estimated to compare the uncovered and covered stent groups. The primary and secondary patency rates of the TIPS patients were found to be 83.9% and 93.9% at the 6 month follow-up and 73.5% and 88.5% at the12 month follow-up for uncovered and covered stents, respectively. A breakdown patency rates for the 12 month follow-up revealed that the primary patency rates were 76.6% and 66.3% for uncovered and covered stents, respectively; whereas, the secondary patency rates were 94.3% and 73.8% for the uncovered and covered stents, respectively. A comparative analysis did not provide evidence to suggest that a difference exists between the patency rates of the uncovered and covered stent groups (p>0.05). No significant difference was found between the patency rates of the uncovered and covered stent groups. A follow-up to this study would be a more thorough randomized evaluation of the different types of covered stents to compare long-term patency rates.

  18. MRI of congenital pituitary insufficiency

    International Nuclear Information System (INIS)

    Almeida Magalhaes, Alvaro C. de; Uehara, Karla C.; Iezzi, Denise

    1995-01-01

    We compare 1,5 T magnetic resonance (MR) image findings in 193 patients with congenital pituitary congenital insufficiency. One hundred and thirty nine of the MR studies were obtained in patients who had isolated growth hormone deficiency. Other fifth - four patients had multiple pituitary hormone deficiency. On MR images, normal anterior and posterior lobes of the pituitary glands can be clearly differentiated because the posterior lobe has a characteristic high intensity on TI-weighted images. In fifty-four patients, the high- intensity of the posterior lobe was not seen, but a similar high signal intensity was observed at the proximal stump in fifty-one patients. this high- intensity area is the newly formed ectopic posterior lobe, which also secrets anti-diuretic hormone just as the posterior lobe would. MR imaging can demonstrate the transection of the pituitary stalk and the formation of the ectopic lobe, revealing to be a useful diagnostic tool in the definition of the type of alteration in growth defects of endocrine origin. (author)

  19. Corpuscular radiation and congenital anomalies

    International Nuclear Information System (INIS)

    Sato, Shinji; Yajima, Akira

    1983-01-01

    Many explorations have been done by our antecessors to find out about the congenital anomalies which might be caused by X-ray or γ-ray and experimental teratological researches have also been done, with some results. However, there have been less systematic studies on corpuscular radiation. Neutron ray is a radioactive ray no electrically-charged equally as photon (X-ray, γ-ray). With an equal dosage of it as photon's, its localized energy is high so that it is different in RBE, OER, etc. In heavy charged particle (proton ray, He ion, Ne ion, π-meson ray), there is the characteristic that the energy increases at around the deepest spot within the range rather than at the point of injection into a system, which is called Bragg peak. The type and energy of this radiation reflected in the uniqueness in energy distribution, in ionization density and in LET makes it the most important radioactive biological parameter. At this paper, we shall review the types of radioactive rays and discuss the congenital anomalies (teratogenecity) including the experimental results obtained by application of our proton ray. (author)

  20. Chemoembolization of Extrahepatic Collateral Arteries for Treatment of Hepatocellular Carcinoma in the Caudate Lobe of the Liver

    International Nuclear Information System (INIS)

    Woo, Sungmin; Kim, Hyo-Cheol; Chung, Jin Wook; Jung, Hyun-Seok; Hur, Saebeom; Lee, Myungsu; Jae, Hwan Jun

    2015-01-01

    PurposeThis study was designed to evaluate the efficacy and safety in performing chemoembolization of extrahepatic collateral arteries (EHC) for hepatocellular carcinoma (HCC) located in the caudate lobe.MethodsBetween January 2006 and November 2013, chemoembolization via EHC was performed in 35 patients with 35 caudate HCCs. Preprocedural and follow-up CT or MR scans, angiographic images, and medical records were reviewed retrospectively in consensus. Chi-square analysis was used to evaluate the relationship between tumor characteristics and type of EHC and that between tumor response and the characteristics of the tumor and chemoembolization.ResultsIn 31 (88.6 %) patients, EHCs supplying the caudate HCC originated from the right inferior phrenic artery (RIPA). The remaining four HCCs were supplied by the gastroduodenal artery, dorsal pancreatic artery, and right and left gastric arteries. Superselective catheterization of tumor-feeding vessels from the EHC was achieved in 27 patients (77.1 %). There were no major complications. Individual tumor response supplied by the EHC at follow-up contrast-enhanced CT were as follows: complete response (n = 18), partial response (n = 9), stable disease (n = 3), and progressive disease (n = 3). Non-RIPA EHCs were significantly more common in patients who had previously received chemoembolization via the RIPA (50 %) than those who had not (6.5 %; P = 0.01). There was no significant predictive factor associated with tumor response.ConclusionsHCC in the caudate lobe can be supplied by several EHCs. Chemoembolization via these arteries can be performed safely and effectively

  1. Distant Metastasis Risk Stratification for Patients Undergoing Curative Resection Followed by Adjuvant Chemoradiation for Extrahepatic Bile Duct Cancer

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Kyubo [Department of Radiation Oncology, Seoul National University College of Medicine, Seoul (Korea, Republic of); Chie, Eui Kyu, E-mail: ekchie93@snu.ac.kr [Department of Radiation Oncology, Seoul National University College of Medicine, Seoul (Korea, Republic of); Jang, Jin-Young; Kim, Sun Whe [Department of Surgery, Seoul National University College of Medicine, Seoul (Korea, Republic of); Han, Sae-Won; Oh, Do-Youn; Im, Seock-Ah; Kim, Tae-You; Bang, Yung-Jue [Department of Internal Medicine, Seoul National University College of Medicine, Seoul (Korea, Republic of); Ha, Sung W. [Department of Radiation Oncology, Seoul National University College of Medicine, Seoul (Korea, Republic of); Institute of Radiation Medicine, Medical Research Center, Seoul National University (Korea, Republic of)

    2012-09-01

    Purpose: To analyze the prognostic factors predicting distant metastasis in patients undergoing adjuvant chemoradiation for extrahepatic bile duct (EHBD) cancer. Methods and Materials: Between January 1995 and August 2006, 166 patients with EHBD cancer underwent resection with curative intent, followed by adjuvant chemoradiation. There were 120 males and 46 females, and median age was 61 years (range, 34-86). Postoperative radiotherapy was delivered to tumor bed and regional lymph nodes (median dose, 40 Gy; range, 34-56 Gy). A total of 157 patients also received fluoropyrimidine chemotherapy as a radiosensitizer, and fluoropyrimidine-based maintenance chemotherapy was administered to 127 patients. Median follow-up duration was 29 months. Results: The treatment failed for 97 patients, and the major pattern of failure was distant metastasis (76 patients, 78.4%). The 5-year distant metastasis-free survival rate was 49.4%. The most common site of distant failure was the liver (n = 36). On multivariate analysis, hilar tumor, tumor size {>=}2 cm, involved lymph node, and poorly differentiated tumor were associated with inferior distant metastasis-free survival (p = 0.0348, 0.0754, 0.0009, and 0.0078, respectively), whereas T stage was not (p = 0.8081). When patients were divided into four groups based on these risk factors, the 5-year distant metastasis-free survival rates for patients with 0, 1, 2, and 3 risk factors were 86.4%, 59.9%, 32.5%, and 0%, respectively (p < 0.0001). Conclusion: Despite maintenance chemotherapy, distant metastasis was the major pattern of failure in patients undergoing adjuvant chemoradiation for EHBD cancer after resection with curative intent. Intensified chemotherapy is warranted to improve the treatment outcome, especially in those with multiple risk factors.

  2. The role of extrahepatic metabolism in the pharmacokinetics of the targeted covalent inhibitors afatinib, ibrutinib, and neratinib.

    Science.gov (United States)

    Shibata, Yoshihiro; Chiba, Masato

    2015-03-01

    Despite the fact that much progress has been made recently in the development of targeted covalent inhibitors (TCIs), their pharmacokinetics (PK) have not been well characterized in the light of extrahepatic clearance (CLextH) by glutathione (GSH)/glutathione S-transferase (GST)-dependent conjugation attributable to the unique electrophilic structure (e.g., acrylamide moiety) of TCI compounds. In the present study, CLextH values were examined in rat, dog, and monkey to predict the contribution of CLextH to the PK of the TCIs afatinib, ibrutinib, and neratinib in humans. Afatinib and neratinib both underwent extensive conjugation with GSH in buffer and cytosol fractions of liver and kidney, whereas ibrutinib showed much lower reactivity/susceptibility to GSH/GST-dependent conjugation. The CLextH in each species was calculated from the difference between observed total body clearance and predicted hepatic clearance (CLH) in cryopreserved hepatocytes suspended in 100% serum of the corresponding species. The power-based simple allometry relating the CLextH for the unbound compound to animal body weight was applicable across species for afatinib and neratinib (R(2) ≥ 0.9) but not for ibrutinib (R(2) = 0.04). The predicted AUC after oral administration of afatinib and neratinib agreed reasonably closely with reported values in phase I dose-escalation studies. Comparisons of CLextH and CLH predicted that CLextH largely determined the PK of afatinib (>90% as a proportion of total body clearance) and neratinib (∼34%) in humans. The present method can serve as one of the tools for the optimization of PK in humans at the discovery stage for the development of TCI candidates. Copyright © 2015 by The American Society for Pharmacology and Experimental Therapeutics.

  3. Percutaneous placement of self-expandable metallic biliary stents in malignant extrahepatic strictures: indications of transpapillary and suprapapillary methods

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Deok Hee; Yu, Jeong Sik; Kim, Ki Hwang [Yonsei University College of Medicine, Seoul (Korea, Republic of); Hwang, Jae Cheol [Ulsan University Hospital, Seoul (Korea, Republic of)

    2000-06-01

    To compare the efficacy of suprapapillary and transpapillary methods of transhepatic biliary metallic stent placement in malignant biliary strictures and to specify the indications of each method applied. Stents were placed in 59 patients. Strictures were categorized as type A (within 3 cm of the ampulla, n = 27), type B (over 3 cm from ampulla, n = 7), type C (within 3 cm of the bending portion, n = 9), or type D (over 3 cm above the bending portion, n=16). The stenting method was suprapapillary in 34 cases and transpapillary in 25. The rates of initial and long-term patency and of early recurrence were compared. Initial patency rates for the suprapapillary and transpapillary methods were 1/7 (14.3%) and 20/20 (100%) respectively for type A (p < 0.0001), 4/5 (80.0%) and 2/2 for type B, 3/7 (42.9%) and 2/2 for type C, and 15/16 (93.8%) and 0/0 for type D. Early recurrence rates were 7/30 (23.3%) using the suprapapillary method and 4/29 (13.8%) using the transpapillary method (p = 0.51). The long-term patency rate did not differ significantly according to either type (p = 0.37) or method (p = 0 . 6 2 ). For good initial patency, the transpapillary method is recommended for strictures of the distal extrahepatic duct near the ampulla and just above the bending portion. Long-term patency is not influenced by the stenting method employed.

  4. Extrahepatic portal venous system thrombosis in recurrent acute and chronic alcoholic pancreatitis is caused by local inflammation and not thrombophilia.

    Science.gov (United States)

    Rebours, Vinciane; Boudaoud, Larbi; Vullierme, Marie-Pierre; Vidaud, Dominique; Condat, Bertrand; Hentic, Olivia; Maire, Frédérique; Hammel, Pascal; Ruszniewski, Philippe; Lévy, Philippe

    2012-10-01

    Extrahepatic portal venous system thrombosis (EPVST) occurs in 13% of patients with either recurrent acute (AP) or chronic (CP) alcoholic pancreatitis. The role of thrombophilia has never been assessed in this entity. All consecutive patients with alcoholic AP or CP were included in a prospective study. All patients underwent a computerized tomography (CT) scan of the pancreas to evaluate EPVST as well as thorough testing for thrombophilia (protein C, S, and antithrombin deficiency, factor II, factor V, and JAK2 gene mutations, homocystein, biological antiphospholipid syndrome). A total of 119 patients (male, n=100 (84%); smokers, n=110 (92%)) were included. EPVST was found in 41 patients (35%). The portal, superior mesenteric, or splenic veins were involved in 34%, 24%, and 93% of patients, respectively. Thrombophilia was identified in 18% (n=22), including the biological antiphospholipid syndrome, factor V Leiden mutation, and factor II G20210A gene mutation in 21 (17.6%), 2 (1.6%), and 1 patient (0.8%), respectively. On univariate analysis, the factors associated with EPVST were smoking (RR=1.6 (1.38-1.85), P=0.03), pseudocysts (RR=2.91 (1.29-6.56), P=0.008), a pseudocyst in the pancreatic tail (P=0.03), a high CT severity index for AP (P=0.007), and pancreatic parenchymal necrosis (P=0.02). The presence of hemostatic risk factors was not associated with an increased risk of EPVST. On multivariate analysis, only pseudocysts were associated with EPVST (hazard ratio: 6.402; 95% confidence interval (1.59-26.54), P=0.009). EPVST is found in 35% of patients with acute/chronic alcoholic pancreatitis. Local inflammation appears to be the major predisposing condition. The presence of some form of thrombophilia does not increase the risk of EPVST and should not be systematically searched for in case of EPVST.

  5. Warren shunt combined with partial splenectomy in children with extra-hepatic portal hypertension, massive splenomegaly and severe hypersplenism

    Directory of Open Access Journals (Sweden)

    Sretenović Aleksandar

    2014-01-01

    Full Text Available Introduction. Extra-hepatic portal vein obstruction (EHPVO is one of the most often causes of portal hypertension in children. Objective. Establishing the importance of shunt surgery in combination with partial spleen resection in selected pediatric patients with EHPVO, enormous splenomegaly and severe hypersplenism. Methods. Distal splenorenal shunt (DSRS with partial spleen resection was performed in 22 children age from 2 to 17 years with EHPVO. Indications for surgery were pain and abdominal discomfort caused by spleen enlargement, as well as symptomatic hypersplenism with leucopenia, thrombocytopenia and anemia. The partial spleen resection was performed by ligation of blood vessels to caudal two thirds of the spleen. After ischemic parenchymal demarcation transection with electrocautery LigaSure was performed with preservation of 20-30% of spleen tissue, and then Warren DSRS was created. Platelet and leucocytes counts and liver function tests were obtained before, one month and one year after surgery. Growth was assessed with SD scores (Z scores for height, weight and body mass index at the time of surgery and one year later. Results. In all patients postoperative period was without significant complications. Platelets and leucocytes counts were normalized. Patency rate of shunts was 100%. Two significant shunts stenosis were observed and successfully treated with percutaneous angioplasty. During the follow-up period (1 to 9 years all patients were asymptomatic, with improved quality of life and growth. Conclusion. Results of our study indicate that shunt surgery with a partial spleen resection is an effective and safe procedure for patients with enormous splenomegaly and severe hypersplenism caused by EHPVO.

  6. Chemoembolization of Extrahepatic Collateral Arteries for Treatment of Hepatocellular Carcinoma in the Caudate Lobe of the Liver

    Energy Technology Data Exchange (ETDEWEB)

    Woo, Sungmin; Kim, Hyo-Cheol, E-mail: angiointervention@gmail.com; Chung, Jin Wook; Jung, Hyun-Seok; Hur, Saebeom; Lee, Myungsu; Jae, Hwan Jun [Seoul National University Hospital, Department of Radiology, Seoul National University College of Medicine, Institute of Radiation Medicine, Seoul National University Medical Research Center, and Clinical Research Institute (Korea, Republic of)

    2015-04-15

    PurposeThis study was designed to evaluate the efficacy and safety in performing chemoembolization of extrahepatic collateral arteries (EHC) for hepatocellular carcinoma (HCC) located in the caudate lobe.MethodsBetween January 2006 and November 2013, chemoembolization via EHC was performed in 35 patients with 35 caudate HCCs. Preprocedural and follow-up CT or MR scans, angiographic images, and medical records were reviewed retrospectively in consensus. Chi-square analysis was used to evaluate the relationship between tumor characteristics and type of EHC and that between tumor response and the characteristics of the tumor and chemoembolization.ResultsIn 31 (88.6 %) patients, EHCs supplying the caudate HCC originated from the right inferior phrenic artery (RIPA). The remaining four HCCs were supplied by the gastroduodenal artery, dorsal pancreatic artery, and right and left gastric arteries. Superselective catheterization of tumor-feeding vessels from the EHC was achieved in 27 patients (77.1 %). There were no major complications. Individual tumor response supplied by the EHC at follow-up contrast-enhanced CT were as follows: complete response (n = 18), partial response (n = 9), stable disease (n = 3), and progressive disease (n = 3). Non-RIPA EHCs were significantly more common in patients who had previously received chemoembolization via the RIPA (50 %) than those who had not (6.5 %; P = 0.01). There was no significant predictive factor associated with tumor response.ConclusionsHCC in the caudate lobe can be supplied by several EHCs. Chemoembolization via these arteries can be performed safely and effectively.

  7. Portosystemic collateral circulation in the falciform ligament: evaluation with three dimensional dynamic contrast enhanced MR angiography in patients with portal hypertension

    International Nuclear Information System (INIS)

    Wu Zhuo; Liang Biling; Li Yong; Zhong Jinglian; Ye Ruixin; Zhang Rong

    2009-01-01

    Objective: The purpose of our study was to investigate three dimensional dynamic contrast enhanced MR angiography(3D DCE MRA) in the detection of portosystemic collateral circulation in the falciform ligament in patients with portal hypertension. Methods: From April 2003 to July 2008, 53 portal hypertension patients with varices in the falciform ligament were evaluated with 3D DCE MRA. Two radiologists independently assessed the number, diameter, location and drainages of the portosystemic collateral circulation in the falciform ligament according to the information on the 3D DCE MRA. Results: The veins in the falciform ligament were classified into the superior and inferior groups, and both groups arise from the left trunk of the portal vein. In our study, the number of varices detected on 3D DCE MRA images varied from 1 to 3, and the diameters of these vessels varied from 0.4 to 2.6 cm. The inferior group consisted of paraumbilical/umbilical veins (47 cases), which flowed toward umbilicus and then drained upwards (n=16) including deep superior epigastric veins (n=7), superficial superior epigastric veins (n=9), downwards (n=40) including deep inferior epigastric veins (n=7), superficial inferior epigastric veins (n=33), or upwards and downwards at the same time (n=9). The superior group of vessels in the falciform ligament were directly anastomosed with the internal thoracic vessels (n=6). Conclusion: In patients with portal hypertension, 3D DCE MRA can optimally demonstrate the portosystemic collateral circulation in the falciform ligament, which includes the superior and inferior drainage groups. (authors)

  8. Comparison of Technical and Clinical Outcome of Transjugular Portosystemic Shunt Placement Between a Bare Metal Stent and a PTFE-Stentgraft Device.

    Science.gov (United States)

    Lauermann, J; Potthoff, A; Mc Cavert, M; Marquardt, S; Vaske, B; Rosenthal, H; von Hahn, T; Wacker, F; Meyer, B C; Rodt, Thomas

    2016-04-01

    To analyse technical and clinical success of transjugular intrahepatic portosystemic shunt (TIPS) in patients with portal hypertension and compare a stent and a stentgraft with regard to clinical and technical outcome and associated costs. 170 patients (56 ± 12 years, 32.9% females) treated with TIPS due to portal hypertension were reviewed. 80 patients received a stent (group 1) and 83 a stentgraft (group 2), and seven interventions were unsuccessful. Technical data, periprocedural imaging, follow-up ultrasound and clinical data were analysed with focus on technical success, patency, clinical outcome and group differences. Cost analysis was performed. Portal hypertension was mainly caused by ethyltoxic liver cirrhosis with ascites as dominant symptom (80%). Technical success was 93.5% with mean portosystemic gradient decrease from 16.1 ± 4.8 to 5.1 ± 2.1 mmHg. No significant differences in technical success and portosystemic gradient decrease between the groups were observed. Kaplan-Meier analysis yielded significant differences in primary patency after 14 days, 6 months and 2 years in favour of the stentgraft. Both groups showed good clinical results without significant difference in 1-year survival and hepatic encephalopathy rate. Costs to establish TIPS and to manage 2-year follow-up with constant patency and clinical success were 8876 € (group 1) and 9394 € (group 2). TIPS is a safe and effective procedure to manage portal hypertension. Stent and stentgraft enabled good technical and clinical results with a low complication rate. Primary patency rates are clearly in favour of the stentgraft, whereas the stent was more cost effective with similar clinical results in both groups.

  9. Comparison of Technical and Clinical Outcome of Transjugular Portosystemic Shunt Placement Between a Bare Metal Stent and a PTFE-Stentgraft Device

    Energy Technology Data Exchange (ETDEWEB)

    Lauermann, J., E-mail: jostlauermann@gmail.com [Hannover Medical School, Department of Diagnostic and Interventional Radiology (Germany); Potthoff, A. [Hannover Medical School, Department of Gastroenterology, Hepatology and Endocrinology (Germany); Mc Cavert, M. [Beaumont Hospital, Department of Diagnostic and Interventional Radiology (Ireland); Marquardt, S. [Hannover Medical School, Department of Diagnostic and Interventional Radiology (Germany); Vaske, B. [Hannover Medical School, Institute of Biometry (Germany); Rosenthal, H. [Hannover Medical School, Department of Diagnostic and Interventional Radiology (Germany); Hahn, T. von [Hannover Medical School, Department of Gastroenterology, Hepatology and Endocrinology (Germany); Wacker, F.; Meyer, B. C.; Rodt, Thomas, E-mail: rodt.thomas@mh-hannover.de [Hannover Medical School, Department of Diagnostic and Interventional Radiology (Germany)

    2016-04-15

    PurposeTo analyse technical and clinical success of transjugular intrahepatic portosystemic shunt (TIPS) in patients with portal hypertension and compare a stent and a stentgraft with regard to clinical and technical outcome and associated costs.Materials and Methods170 patients (56 ± 12 years, 32.9 % females) treated with TIPS due to portal hypertension were reviewed. 80 patients received a stent (group 1) and 83 a stentgraft (group 2), and seven interventions were unsuccessful. Technical data, periprocedural imaging, follow-up ultrasound and clinical data were analysed with focus on technical success, patency, clinical outcome and group differences. Cost analysis was performed.ResultsPortal hypertension was mainly caused by ethyltoxic liver cirrhosis with ascites as dominant symptom (80 %). Technical success was 93.5 % with mean portosystemic gradient decrease from 16.1 ± 4.8 to 5.1 ± 2.1 mmHg. No significant differences in technical success and portosystemic gradient decrease between the groups were observed. Kaplan–Meier analysis yielded significant differences in primary patency after 14 days, 6 months and 2 years in favour of the stentgraft. Both groups showed good clinical results without significant difference in 1-year survival and hepatic encephalopathy rate. Costs to establish TIPS and to manage 2-year follow-up with constant patency and clinical success were 8876 € (group 1) and 9394 € (group 2).ConclusionTIPS is a safe and effective procedure to manage portal hypertension. Stent and stentgraft enabled good technical and clinical results with a low complication rate. Primary patency rates are clearly in favour of the stentgraft, whereas the stent was more cost effective with similar clinical results in both groups.

  10. Management of Transjugular Intrahepatic Portosystemic Shunt (TIPS)-associated Refractory Hepatic Encephalopathy by Shunt Reduction Using the Parallel Technique: Outcomes of a Retrospective Case Series

    International Nuclear Information System (INIS)

    Cookson, Daniel T.; Zaman, Zubayr; Gordon-Smith, James; Ireland, Hamish M.; Hayes, Peter C.

    2011-01-01

    Purpose: To investigate the reproducibility and technical and clinical success of the parallel technique of transjugular intrahepatic portosystemic shunt (TIPS) reduction in the management of refractory hepatic encephalopathy (HE). Materials and Methods: A 10-mm-diameter self-expanding stent graft and a 5–6-mm-diameter balloon-expandable stent were placed in parallel inside the existing TIPS in 8 patients via a dual unilateral transjugular approach. Changes in portosystemic pressure gradient and HE grade were used as primary end points. Results: TIPS reduction was technically successful in all patients. Mean ± standard deviation portosystemic pressure gradient before and after shunt reduction was 4.9 ± 3.6 mmHg (range, 0–12 mmHg) and 10.5 ± 3.9 mmHg (range, 6–18 mmHg). Duration of follow-up was 137 ± 117.8 days (range, 18–326 days). Clinical improvement of HE occurred in 5 patients (62.5%) with resolution of HE in 4 patients (50%). Single episodes of recurrent gastrointestinal hemorrhage occurred in 3 patients (37.5%). These were self-limiting in 2 cases and successfully managed in 1 case by correction of coagulopathy and blood transfusion. Two of these patients (25%) died, one each of renal failure and hepatorenal failure. Conclusion: The parallel technique of TIPS reduction is reproducible and has a high technical success rate. A dual unilateral transjugular approach is advantageous when performing this procedure. The parallel technique allows repeat bidirectional TIPS adjustment and may be of significant clinical benefit in the management of refractory HE.

  11. Transjugular intrahepatic portosystemic stent shunt (TIPSS) with use of Roesch-Uchida transjugular liver access set; Evaluation by CT and its clinical application

    Energy Technology Data Exchange (ETDEWEB)

    Takahashi, Motoichiro; Okawada, Taketoshi; Kato, Ryoichi; Mochizuki, Takao; Ohkawa, Yoshihisa; Kaneko, Masao [Hamamatsu University School of Medicine, Shizuoka (Japan); Ke, Xu; Hanguo, Zhang; Fangxian, He

    1992-12-01

    Appropriateness of the Roesch-Uchida transjugular liver access set designed for transjugular intrahepatic portosystemic stent shunt (TIPSS) procedure was confirmed, especially about the catheter angle and effective length of the 20 G puncture needle, by CT analysis on three dimensional vascular anatomy of the liver. Clinically, TIPSS using the set was successfully made for two patients, connecting superior right hepatic vein with right portal vein in one patient and middle hepatic vein with left portal vein in another patient with hypoplastic right portal vein. Prior to TIPSS procedure, verification of vascular anatomy on CT images is the key to success of TIPSS in safe. (author).

  12. [Gender-specific influencing factors on incidence, risk factors and outcome of carcinoma of the liver, gallbladder, extrahepatic bile duct and pancreas].

    Science.gov (United States)

    Grundmann, R T; Meyer, F

    2014-04-01

    This overview comments on gender-specific differences in incidence, risk factors and prognosis in patients with carcinoma of the liver, gallbladder, extrahepatic bile duct and pancreas. For the literature review, the MEDLINE database (PubMed) was searched under the key words "liver cancer", "gallbladder cancer", "extrahepatic bile duct carcinoma", "pancreatic cancer" AND "gender". There were significant gender differences in the epidemiology of the analysed carcinomas. The incidence of hepatocellular carcinoma (HCC) is much higher in men than in women, one of 86 men, but only 1 out of 200 women develop a malignant primary liver tumour in Germany in the course of their life. The lifetime risk for carcinomas of the gallbladder and extrahepatic bile ducts in Germany amounts to about 0.6 % for women and 0.5 % for men, specifically gallbladder carcinomas are observed more frequently in women than in men. For pancreatic cancer, no clear gender preference exists in Germany, although the mortality risk for men is higher than that for women (age-adjusted standardised death rate in men 12.8/100, 000 persons, in women 9.5). Remarkable is furthermore the shift of the tumour incidence in the last decades. Liver cancer has increased among men in Germany by about 50 % in the last 30 years, the incidence of gallbladder carcinoma has inversely dropped. The prognosis of these cancers across all tumour stages is uniformly bad in an unselected patient population. This is probably the main reason why only little - if any - gender differences in survival are described. In addition to avoiding the known risk factors such as hepatitis B and C virus infection, alcohol abuse, and smoking, the avoidance of overweight and obesity plays an increasingly important role in the prevention of these cancers. Georg Thieme Verlag KG Stuttgart · New York.

  13. The usefulness of high-resolution three-dimensional dynamic MR imaging with sensitivity encoding for evaluating extrahepatic bile duct cancer

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Kon; Ko, Seog Wan [Chonbuk National University Hospital and Medical School, Jeonju (Korea, Republic of)

    2006-07-15

    We assessed the usefulness of high-resolution 3D dynamic MR imaging with sensitivity encoding (mSENSE) for evaluating bile duct cancer. Twenty-three patients with extrahepatic bile duct cancer underwent multiphasic 3D GRE MRI, including two delayed phases without and with mSENSE. The first delayed phases were obtained with volumetric interpolated breath-hold imaging (VIBE) and then the higher in-place resolution images (320 X 168) were obtained using mSENSE. The two delayed phase images were compared quantitatively by measuring the signal-to-noise ratio (SNR) of liver and tumor, the liver-visceral fat contrast and the tumor-visceral fat contrast-to-noise ratio (CNR); the two delayed phase images were compared qualitatively by evaluating the sharpness of the hepatic vessels and bile duct, the artifacts and the conspicuity of bile duct cancer. The quantitative results with mSENSE image were significantly better than those with conventional VIBE. Though the clarity of the intrahepatic vessels and the intrahepatic bile duct, and the artifacts did not differ significantly between the two images ( {rho} > 0.05), the clarity of the extrahepatic vessels, the extrahepatic bile duct and the bile duct cancer were better on the mSENSE image than on the VIBE ( {rho} < 0.05). The higher in-plane resolution 3D GRE image obtained with mSENSE was of a better image quality than the conventional VIBE images. This technique shows promise for use as a comprehensive exam for assessing bile duct cancer.

  14. The usefulness of high-resolution three-dimensional dynamic MR imaging with sensitivity encoding for evaluating extrahepatic bile duct cancer

    International Nuclear Information System (INIS)

    Kim, Young Kon; Ko, Seog Wan

    2006-01-01

    We assessed the usefulness of high-resolution 3D dynamic MR imaging with sensitivity encoding (mSENSE) for evaluating bile duct cancer. Twenty-three patients with extrahepatic bile duct cancer underwent multiphasic 3D GRE MRI, including two delayed phases without and with mSENSE. The first delayed phases were obtained with volumetric interpolated breath-hold imaging (VIBE) and then the higher in-place resolution images (320 X 168) were obtained using mSENSE. The two delayed phase images were compared quantitatively by measuring the signal-to-noise ratio (SNR) of liver and tumor, the liver-visceral fat contrast and the tumor-visceral fat contrast-to-noise ratio (CNR); the two delayed phase images were compared qualitatively by evaluating the sharpness of the hepatic vessels and bile duct, the artifacts and the conspicuity of bile duct cancer. The quantitative results with mSENSE image were significantly better than those with conventional VIBE. Though the clarity of the intrahepatic vessels and the intrahepatic bile duct, and the artifacts did not differ significantly between the two images ( ρ > 0.05), the clarity of the extrahepatic vessels, the extrahepatic bile duct and the bile duct cancer were better on the mSENSE image than on the VIBE ( ρ < 0.05). The higher in-plane resolution 3D GRE image obtained with mSENSE was of a better image quality than the conventional VIBE images. This technique shows promise for use as a comprehensive exam for assessing bile duct cancer

  15. Application of PET-CT in monitoring residual and extrahepatic metastatic lesions for hepatocellular carcinoma with positive alpha fetoproteins after interventional therapy

    International Nuclear Information System (INIS)

    Zhu Guangyu; Teng Gaojun; Guo Jinhe; Deng Gang; He Shicheng; Fang Wen; Li Guozhao; Chen Xiaohui; Wei Xiaoying

    2010-01-01

    Objective: To investigate the value of positron emission tomography-computed tomography (PET-CT) in monitoring the residual lesions in lipiodol sedimentary region and extrahepatic metastastic lesions of hepatocellular carcinoma (HCC) with alpha fetoproteins (AFP) positive after interventional therapy. Methods: The data of 20 cases with primary HCC confirmed by histopathology were retrospectively analyzed. Their AFP levels decreased to normal range after interventional treatments, but rose to abnormal high level during following-up. After the abdominal routine imaging examinations, the definite diagnosis of the residual lesions in lipiodol sedimentary region or extrahepatic lesions can't be made confidently. All cases were scanned by PET-CT, and according to their PET-CT results, the further treatments were given and the therapeutic results were monitored with radiology and AFP tests. Results: In all 20 cases, 10 of them were detected to have the extrahepatic metastastic lesions by PET-CT, including 4 with abdominal wall metastasis upon the liver, 3 with solitary pulmonary metastasis with diameter less than 1 cm, 2 with mesenteric metastasis, 1 with metastasis of operative incisions, but these lesions were neglected by abdominal routine imaging examinations. Eight cases showed the uneven lipiodol sedimentary region in the primary lesion by CT or MRI examination, but can't be diagnosed whether it was residual lesion with other examinations including DSA. A definite diagnosis was obtained by PET-CT. In 2 cases, lymph nodes less than 1.5 cm were found in the hepatic portal area (PHA) and retroperitoneum on CT images, which was determined to be metastatic by PET-CT. All the detected lesions were given further treatments of surgery or interventional therapy. Most patients showed decreased AFP levels except the 2 patients with lymph node metastasis. The imaging examinations also indicated that the treatments had a good effect on lesions. Conclusion: In the patients with

  16. [Sex differences in congenital heart disease].

    Science.gov (United States)

    Aubry, P; Demian, H

    2016-12-01

    Gender influences the clinical presentation and the management of some acquired cardiovascular diseases, such as coronary artery disease, resulting in different outcomes. Differences between women and men are also noticed in congenital heart disease. They are mainly related to the prevalence and severity of some congenital heart defects at birth, and in adulthood to the prognosis, incidence of Eisenmenger syndrome and risks of pregnancy. The role of gender on the risk of operative mortality of congenital heart surgery remains debated. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  17. Cardiovascular magnetic resonance in congenital heart disease

    International Nuclear Information System (INIS)

    Cazacu, A.; Ciubotaru, A.

    2010-01-01

    The increasing prevalence of congenital heart disease can be attributed to major improvements in diagnosis and treatment. Cardiovascular magnetic resonance imaging plays an important role in the clinical management strategy of patients with congenital heart disease. The development of new cardiovascular magnetic resonance (CMR) techniques allows comprehensive assessment of complex cardiac anatomy and function and provides information about the long-term residual post-operative lesions and complications of surgery. It overcomes many of the limitations of echocardiography and cardiac catheterization. This review evaluates the role of cardiovascular magnetic resonance imaging modality in the management of subject with congenital heart disease (CHD). (authors)

  18. Congenital anomalies of the neonatal head

    International Nuclear Information System (INIS)

    Benson, C.B.; Teele, R.L.; Dobkin, G.R.; Fine, C.; Bundy, A.L.; Doubilet, P.M.

    1987-01-01

    US is a noninvasive modality that has proved highly valuable in the assessment of congenital intracranial anomalies in the neonate. The patterns of malformation must be familiar to those who obtain and interpret neonatal cranial sonograms. The authors present a variety of cases of congenital anomalies studied with US, including agenesis of the corpus callosum, Dandy-Walker malformation, occipital enecphalocele, congenital hydrocephalus, vein of Galen aneurysm, hydranencephaly, holoprosencephaly, absence of the septum pellucidum, schizencephaly, and hypoplastic cerebellum. Correlation with other radiographic modalities and pathologic follow-up, available in a majority of cases, is included

  19. Etiological evaluation of primary congenital hypothyroidism cases.

    Science.gov (United States)

    Bezen, Diğdem; Dilek, Emine; Torun, Neşe; Tütüncüler, Filiz

    2017-06-01

    Primary congenital hypothyroidism is frequently seen endocrine disorder and one of the preventable cause of mental retardation. Aim of study was to evaluate the frequency of permanent/transient hypothyrodism, and to detect underlying reason to identfy any marker which carries potential to discriminate permanent/transient form. Forty eight cases older than 3 years of age, diagnosed as primary congenital hypothyroidism and started thyroxin therapy in newborn-period, and followed up between January 2007-June 2013 were included in the study. Thyroid hormon levels were evaluated and thyroid ultrasonography was performed in cases who are at the end of their 3 years of age, after 6 weeks of thyroxine free period. Thyroid sintigraphy was performed if serum thyroid-stimulating hormone was high (≥ 5 mIU/mL) and perchlorate discharge test was performed if uptake was normal or increased on sintigraphy. Cases with thyroid-stimulating hormone levels ≥ 5 mIU/mL were defined as permanent primary congenital hypothyroidism group and as transient primary congenital hypothyroidism group with normal thyroid hormones during 6 months. The mean age was 3.8±0.7 years. Mean diagnosis age was 16.6±6.5 days and 14 cases (29.2%) were diagnosed by screening program of Ministry of Health. There were 23 cases (14F, 9M) in permanent primary congenital hypothyroidism group and 12 (52.2%) of them were dysgenesis (8 hypoplasia, 4 ectopia), and 11 (47.8%) dyshormonogenesis. In transient primary congenital hypothyroidism group, there were 25 cases (17M, 8F). The mean thyroid-stimulating hormone levels at diagnosis were similar in two groups. The mean thyroxin dose in permanent primary congenital hypothyroidism group was significantly higher than transient group at the time of thyroxin cessation (2.1±0.7, 1.5±0.5 mg/kg/d, respectively, p=0.004). Thyroxin dose ≥1.6 mcg/kg/d was 72% sensitive and 69.6% specific for predicting permenant primary congenital hypothyroidism. Transient primary

  20. Epidemiology of congenital diaphragmatic hernia in Europe

    DEFF Research Database (Denmark)

    McGivern, Mark R.; Best, Kate E.; Rankin, Judith

    2015-01-01

    INTRODUCTION: Published prevalence rates of congenital diaphragmatic hernia (CDH) vary. This study aims to describe the epidemiology of CDH using data from high-quality, population-based registers belonging to the European Surveillance of Congenital Anomalies (EUROCAT). METHODS: Cases of CDH...... for isolated cases (ie, CDH cases that did not occur with any other congenital anomaly). There was significant variation in total and isolated CDH prevalence between registers. The proportion of cases that survived to 1 week was 69.3% (1392 cases) for total CDH cases and 72.7% (1107) for isolated cases...