WorldWideScience

Sample records for long-gap esophageal atresia

  1. Long-gap esophageal atresia.

    Science.gov (United States)

    Shieh, Hester F; Jennings, Russell W

    2017-04-01

    The management of long-gap esophageal atresia remains challenging with limited consensus on the definition, evaluation, and surgical approach to treatment. Efforts to preserve the native esophagus have been successful with delayed primary anastomosis and tension-based esophageal growth induction processes. Esophageal replacement is necessary in a minority of cases, with the conduit of choice and patient outcomes largely dependent on institutional expertise. Given the complexity of this patient population with significant morbidity, treatment and long-term follow-up are best done in multidisciplinary esophageal and airway treatment centers. Copyright © 2017 Elsevier Inc. All rights reserved.

  2. Jejunum for bridging long-gap esophageal atresia

    NARCIS (Netherlands)

    Bax, Klaas (N) M. A.

    OBJECTIVE: Exploring pros and cons of bridging long-gap esophageal atresia with an orthotopic jejunal pedicle graft. Retrospective series of 19 patients. METHODS: From 1988 through 2005, 19 patients with long-gap esophageal atresia received a jejunal graft. Median age at reconstruction was 76 days.

  3. Thoracoscopic traction technique in long gap esophageal atresia : entering a new era

    NARCIS (Netherlands)

    van der Zee, David C.; Gallo, Gabriele; Tytgat, Stefaan H A

    2015-01-01

    Objective: To describe the evolution from delayed management of long gap esophageal atresia to thoracoscopic treatment directly after birth without the placement of a gastrostomy. Background: Long gap esophageal atresia remains a challenge for pediatric surgeons. Over the years, several techniques h

  4. Thoracoscopic traction technique in long gap esophageal atresia : entering a new era

    NARCIS (Netherlands)

    van der Zee, David C.; Gallo, Gabriele; Tytgat, Stefaan H. A.

    2015-01-01

    To describe the evolution from delayed management of long gap esophageal atresia to thoracoscopic treatment directly after birth without the placement of a gastrostomy. Long gap esophageal atresia remains a challenge for pediatric surgeons. Over the years, several techniques have been described to d

  5. Long-Gap Esophageal Atresia : a Meta-Analysis of Jejunal Interposition, Colon Interposition, and Gastric Pull-Up

    NARCIS (Netherlands)

    Gallo, G.; Zwaveling, S.; Groen, Henk; Van der Zee, D.; Hulscher, Jan

    2012-01-01

    Aim There is still no consensus about the optimal surgical approach for esophageal replacement in the case of long-gap esophageal atresia (LGEA) or extensive corrosive strictures. The aim of this article was to perform a meta-analysis comparing the most widely used techniques for esophageal replacem

  6. Achievement of feeding milestones after primary repair of long-gap esophageal atresia.

    Science.gov (United States)

    Khan, Khalid M; Krosch, Tara C; Eickhoff, Jeffrey C; Sabati, Arash A; Brudney, James; Rivard, Andrew L; Foker, John E

    2009-06-01

    To determine the pattern of feeding milestones following primary repair of long-gap esophageal atresia (EA). A questionnaire based upon well established feeding milestones was used. Children after long-gap EA repair, n=40, were compared from after primary repair to healthy children from birth, n=102. The age when surveyed of the EA group and controls was different: 6.2+/-4.7 (mean+/-standard deviation) years, range 1.1-20.9, versus 2.5+/-2.4 years, range 0.0-12.1, p=0.00. The esophageal gap length in the EA group was 5.1+/-1.2 cm and age at repair was 5.5+/-5.0 months. There was no statistically significant difference between the atresia group and controls for feeding milestones; Self feeding finger foods approached significance. There was, however, greater variability in the timing of milestones in the atresia group compared to controls. Feeding milestones were negatively correlated with age at primary repair: drinking with a covered sippy cup, rho=-0.51, p=0.01 and self feeding finger foods, rho=-0.36, p=0.04 were statistically significant. Drinking from a cup correlated with gestational age, rho=0.38, p=0.04, and negatively correlated to esophageal gap length, rho=-0.45, p=0.01. Despite delayed onset of feeding, major milestones after EA repair occurred in similar pattern to normal infants. An early referral for primary repair is beneficial for earlier acquisition of milestones for infants with long-gap EA.

  7. Two-Stage Thoracoscopic Repair of Long-Gap Esophageal Atresia Using Internal Traction Is Safe and Feasible.

    Science.gov (United States)

    Tainaka, Takahisa; Uchida, Hiroo; Tanano, Akihide; Shirota, Chiyoe; Hinoki, Akinari; Murase, Naruhiko; Yokota, Kazuki; Oshima, Kazuo; Shirotsuki, Ryo; Chiba, Kosuke; Amano, Hizuru; Kawashima, Hiroshi; Tanaka, Yujiro

    2017-01-01

    The treatment of long-gap esophageal atresia remains an issue for pediatric surgeons. Many techniques for treating long-gap esophageal atresia have been proposed, but the optimal method has not been established. The thoracoscopic esophageal elongation technique has recently been developed. We previously reported a case in which two-stage thoracoscopic repair was performed using internal esophageal traction without esophageal tearing, and we retrospectively reviewed the outcomes of this procedure in this study. Five patients underwent thoracoscopic treatment involving internal esophageal traction for esophageal atresia involving a long gap or vascular ring over a 5-year period. Between November 2010 and November 2015, 5 patients were treated with thoracoscopic traction. All of these patients successfully underwent thoracoscopic-delayed primary anastomosis. Conversion to open thoracotomy was not required in any case. The postoperative complications experienced by the patients included minor anastomotic leakage in 2 cases, anastomotic stenosis in 1 case, gastroesophageal reflux (GER) in 4 cases, and a hiatal hernia in 1 case. None of the patients died. Two-stage thoracoscopic repair for esophageal atresia involving a long gap or vascular ring is a safe and feasible procedure; however, we must develop methods for treating minor anastomotic complications and GER due to esophageal traction in future.

  8. 13 ribs as a predictor of long gap esophageal atresia: myth or reality? Analysis of associated findings of esophageal atresia and abnormal rib count.

    Science.gov (United States)

    Durell, Jonathan; Dagash, Haitham; Eradi, Bala; Rajimwale, Ashok; Nour, Shawqui; Patwardhan, Nitin

    2017-08-01

    The presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. Babies with EA from January 2005 - December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. Seventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs. Paradoxically, 10 patients with esophageal atresia +/- trachea-esophageal atresia (EA +/- TEF) and supernumerary ribs underwent primary repair. Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap. Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587. VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. We found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/- TEF. Prognosis study - level IV. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. [Esophageal atresia type I. Is impossible possible?].

    Science.gov (United States)

    Ruiz de Temiño, M; Esteban, J A; Elías, J; González, N; Gracia, J; Romeo, M; Escartín, R; Burgués, P; Sainz, A; Pueyo, C

    2006-01-01

    Treatment of esophageal atresia with "long gap" remains difficult and controversial. According to the idea that esophageal anastomosis is imposible in most cases, several esophageal substitution methods have been proposed, as esophagocoloplasty, gastric transposition or reversed gastric tube. Nevertheless reconstruction of native esophagus is accepted as the best option if posible. "Long gap" definition is imprecise, expressed by variability in percent of these cases in total esophageal atresias reported in different series in literature. We report our experience in seven cases type I esophageal atresia with long gap and the different therapeutic options used, with attention to delayed or early esophageal anastomosis feasibility and outcome. We have treated 121 patients with esophageal atresia from whom we analized 7 cases with pure esophageal atresia with "long gap" (5.8%). Six patients underwent gastrostomy and two gastrostomy and esophagostomy. Five patient underwent primary repair with esophageal anastomosis, delayed between 14 days and 4 months in 4 cases. One patient underwent esophageal anastomosis in the first day without gastrostomy. Retroesternal esophagocoloplasty was performed in 2 patients about their first year of life. Esophagogram was done in first month after surgery and pH monitoring of gastroesophageal reflux. Follow-up ranged from 6 months to 28 years. Esophageal anastomosis was feasible in all 5 patients in whom it was tried. Stricture occurred in two patients, one patient underwent anastomotic resection and new esophageal anastomosis. Esophageal reflux was present in two patients, one of them required funduplication. One patient was dead by complications of cardiac malformation. Remaining patients have normal swallowing and are in normal growth curves. Patients with esophagocoloplasty had not relevant early or late complications. In most pure esophageal atresia, delayed or even early esophageal anastomosis is feasible, making use of surgical

  10. Esophageal atresia and tracheo-esophageal fistula.

    Science.gov (United States)

    van der Zee, David C; Tytgat, Stefaan H A; van Herwaarden, Maud Y A

    2017-04-01

    Management of esophageal atresia has merged from correction of the anomaly to the complete spectrum of management of esophageal atresia and all its sequelae. It is the purpose of this article to give an overview of all aspects involved in taking care of patients with esophageal atresia between January 2011 and June 2016, as well as the patients who were referred from other centers. Esophageal atresia is a complex anomaly that has many aspects that have to be dealt with and complications to be solved. By centralizing these patients in centers of expertise it is believed that the best care can be given. Copyright © 2017 Elsevier Inc. All rights reserved.

  11. Current knowledge on esophageal atresia

    Institute of Scientific and Technical Information of China (English)

    Paulo Fernando Martins Pinheiro; Ana Cristina Sim(o)es e Silva; Regina Maria Pereira

    2012-01-01

    Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus.The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care,neonatal anesthesia,ventilatory and nutritional support,antibiotics,early surgical intervention,surgical materials and techniques.Indeed,mortality is currently limited to those cases with coexisting severe life-threatening anomalies.The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed.The primary surgical correction for EA and TEF is the best option in the absence of severe malformations.There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial.The primary complications during the postoperative period are leak and stenosis of the anastomosis,gastro-esophageal reflux,esophageal dysmotility,fistula recurrence,respiratory disorders and deformities of the thoracic wall.Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair.The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses.Much remains to be studied regarding this condition.This manuscript provides a literature review of the current knowledge regarding EA.

  12. Morbidity and mortality in esophageal atresia and ...

    African Journals Online (AJOL)

    Background The outcome of newborns with esophageal atresia (EA) ... Their mean birth weight was 2.4 kg. (700–3800 ... weight, gestational age, associated anomalies, method of diagnosis .... the risk classification of patients with EA/TEF and.

  13. Genetics Home Reference: esophageal atresia/tracheoesophageal fistula

    Science.gov (United States)

    ... Home Health Conditions EA/TEF esophageal atresia/tracheoesophageal fistula Printable PDF Open All Close All Enable Javascript ... the expand/collapse boxes. Description Esophageal atresia/tracheoesophageal fistula ( EA/TEF ) is a condition resulting from abnormal ...

  14. Etiological study on isolated esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Szendrey, T.; Danyi, G.; Czeizel, A.

    1985-01-01

    A study group of 160 index patients with isolated esophageal atresia, a control group of 160 matched healthy controls, and the first-degree relatives of patients and controls were examined; epidemiological, family planning, teratological, and genetic data were obtained by personal interview in the study and control groups. One half of the index patients were male. Intrauterine growth retardation, a higher proportion of mothers under 19 or over 30 years of age, and less skilled professions of the parents were found in the study group. There were more extramarital conceptions, more pregnancies in spite of the use of contraceptive pills, and more delayed conceptions in index patients mothers. The teratogens studied did not have an obvious pathological effect here. The sib occurrence of isolated esophageal atresia was 0.43%, which did not correspond well to the expected figure of 1.34% based on the polygenic model.

  15. Congenital esophageal atresia with tracheo-esophageal fistula

    Energy Technology Data Exchange (ETDEWEB)

    Rhee, Chung Sik [Ewha Womans University College of Medicine, Seoul (Korea, Republic of)

    1970-10-15

    Three cases of esophageal atresia with tracheo-esophageal fistula. 1). Case 1: A female infant birth Wt. 1.95 kg , Apgar Score 10, Skeletal anomalies, was delivered after a pregnancy compeicated by hydroamnious on Aug. 17, 1970. The family history was not contributory. 2) Case 2: A male infant birth Wt. 2.8 kg , Apgar Score 8, was forcep delivered after a pregnancy on Feb. 8, 1970. This infant is twin. The family history was not contributory. 3) Case 3: A female infant birth Wt. 2.22 kg , Apgar Score 10, was C-section after a pregnancy on May, 16. 1970. The family history was not contributory. All cases: After 24 hours 5% glucose solution was given and immediately vomited and some of it regurgitating through the nose and mouth with associated cyanosis and dyspnea. A catheter was inserted through the nose into the esophagus under diagnosis of the esophageal atresia.

  16. Embryogenesis of esophageal atresia: Is localized vascular accident a factor?

    Directory of Open Access Journals (Sweden)

    Dutta Hemonta

    2009-01-01

    Full Text Available Several theories on embryogenesis of esophageal atresia have been proposed, none could explain the whole spectrum of this anomaly. We report a new variant of esophageal atresia in which the two blind pouches were joined by an atretic band. Histology of the atretic part showed groups of striated muscle arranged haphazardly without any lumen. The existing theories on etiology of esophageal atresia cannot explain this variant. However, localized vascular accident during intrauterine life resulting in disturbances in regional microcirculation could be a possible factor as demonstrated by Louw and Barnard in relation to jejunoileal atresia. This is contrary to the current understanding that disproportionate growth of the horizontal esophageal folds results in esophageal atresia.

  17. Dumping syndrome after esophageal atresia repair without antireflux surgery.

    Science.gov (United States)

    Michaud, Laurent; Sfeir, Rony; Couttenier, Frédéric; Turck, Dominique; Gottrand, Frédéric

    2010-04-01

    In childhood, the surgical treatment of gastroesophageal reflux is the main cause of dumping syndrome. We report the cases of 2 children with esophageal atresia who presented with dumping syndrome without any precipitating known factors, such as gastroesophageal reflux surgery or associated microgastria. Our data suggest (1) that dumping syndrome can occur after primary anastomosis of esophageal atresia without antireflux surgery and (2) that dumping syndrome should be considered in every child treated surgically for esophageal atresia presenting with digestive symptoms, malaise, failure to thrive, or refusal to eat.

  18. Magnetic compression anastomosis as a nonsurgical treatment for esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Zaritzky, Mario [Hospital de Ninos de La Plata, Department of Radiology, Buenos Aires (Argentina); University of Chicago Medical Center, Department of Radiology, Chicago, IL (United States); Ben, Ricardo [Hospital de Ninos de La Plata, Department of Gastroenterology, Buenos Aires (Argentina); Zylberg, Gaston I.; Yampolsky, Brian [Hospital de Ninos de La Plata, Department of Radiology, Buenos Aires (Argentina)

    2009-09-15

    We describe a unique technique to promote a nonsurgical esophageal anastomosis with magnets in children with esophageal atresia. To evaluate the efficacy of magnetic lengthening of atretic esophageal ends to produce an anastomosis and to communicate our results after more than 2 years of follow-up. Between September 2001 and March 2004, five children were selected for treatment. Two of the children had esophageal atresia without fistula (type A) and three had atresia with fistula converted to type A surgically; however, surgeons failed to achieve an anastomosis because of the width of the gap. Neodymium-iron-boron magnets were used. Daily chest radiographs were taken until union of the magnets was observed. They were then replaced with an orogastric tube. Anastomosis was achieved in all patients in an average of 4.8 days. One patient, with signs of early sepsis, was successfully treated with antibiotics. In four of the five patients, esophageal stenosis developed. At the time of this report, two patients were free of treatment and on an oral diet (after 26 months), two patients required periodic balloon dilatation, and one patient had recently undergone surgery due to recurrent esophageal stenosis not amenable to balloon dilatation. Magnetic esophageal anastomosis is a feasible method in selected patients with esophageal atresia. Esophageal anastomosis was achieved in all patients. The only observed complication of significance was esophageal stenosis. One patient needed surgery because of stenosis. (orig.)

  19. Asymmetric sweating and flushing in infants with esophageal atresia.

    Science.gov (United States)

    Cozzi, Denis A; Mele, Ermelinda; Totonelli, Giorgia; Ceccanti, Silvia; Frediani, Simone; Cozzi, Francesco

    2009-06-01

    Of 136 infants with repaired esophageal atresia, one presented an unilateral facial flushing and 2 presented a flushing and sweating of one half of the body. The topography of these disorders and/or the associated clinical manifestations suggest that the asymmetry may be related to an instability of unilateral autonomic centers more than to a surgical injury of upper thoracic sympathetic chain during esophageal repair.

  20. Esophageal Atresia and Tracheoesophageal Fistula with Unilateral Pulmonary Agenesis - Hypoplasia

    Directory of Open Access Journals (Sweden)

    Shraddha Verma

    2013-03-01

    Full Text Available Association of unilateral severe pulmonary hypoplasia or agenesis and esophageal atresia (EA with or without tracheoesophageal fistula (TEF is an exceedingly rare and highly lethal combination. We report a case of full term male baby who had EA with TEF and right lung hypoplasia, managed at our centre. He is alive and doing well at 10 years of age.

  1. Esophageal Atresia and Tracheoesophageal Fistula with Unilateral Pulmonary Agenesis - Hypoplasia

    Directory of Open Access Journals (Sweden)

    Katragadda Laxmi Narsimha Rao

    2013-04-01

    Full Text Available Association of unilateral severe pulmonary hypoplasia or agenesis and esophageal atresia (EA with or without tracheoesophageal fistula (TEF is an exceedingly rare and highly lethal combination. We report a case of full term male baby who had EA with TEF and right lung hypoplasia, managed at our centre. He is alive and doing well at 10 years of age.

  2. Preoperative management of children with esophageal atresia: current perspectives

    Directory of Open Access Journals (Sweden)

    Parolini F

    2017-01-01

    Full Text Available Filippo Parolini,1 Anna Lavinia Bulotta,1 Sonia Battaglia,1 Daniele Alberti1,2 1Department of Pediatric Surgery, “Spedali Civili” Children’s Hospital, 2Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy Abstract: Esophageal atresia remains one of the most challenging congenital anomalies of the newborn. In recent years, because of the advances in prenatal diagnosis, neonatal critical care, and surgical procedures, overall outcomes have improved substantially, including for premature children. Nowadays, most of the research is focused on medium- and long-term morbidity, with particular reference to respiratory and gastroesophageal problems; the high frequency of late sequelae in esophageal atresia warrants regular and multidisciplinary checkups throughout adulthood. Surprisingly, there are few studies on the impact of prenatal diagnosis and there is continuing debate over the prenatal and preoperative management of these complex patients. In this review, we analyze the literature surrounding current knowledge on the management of newborns affected by esophageal atresia, focusing on prenatal management and preoperative assessment. Keywords: prenatal diagnosis, esophageal atresia, tracheoesophageal fistula, ultrasound scan, tracheobronchoscopy

  3. Esophageal Anastomosis Medial to Preserved Azygos Vein in Esophageal Atresia with Tracheoesophageal Fistula: Restoration of Normal Mediastinal Anatomy

    Directory of Open Access Journals (Sweden)

    Kumar Abdul Rashid

    2012-09-01

    Full Text Available Objective: We intended to prospectively study the technical feasibility and advantages of esophageal anastomosis medial to the preserved azygos vein in neonates diagnosed with esophageal atresia with tracheoesophageal fistula (EA/TEF. The results were compared to the cases where azygos vein was either not preserved, or the anastomosis was done lateral to the arch of preserved azygos vein. Material and methods: A total of 134 patients with EA/TEF were admitted between January 2007 and July 2008 of which 116 underwent primary repair. Eleven patients with long gap esophageal atresia with or without tracheoesophageal fistula and 7 patients who expired before surgery were excluded. Patients were randomly divided in three groups comparable with respect to the gestational age, age at presentation, sex, birth weight, associated anomalies and the gap between the pouches after mobilization: Group A (azygos vein ligated and divided, Group B (azygos vein preserved with esophageal anastomosis lateral to the vein, and Group C azygos vein preserved with esophageal anastomosis medial to the vein. All the patients were operated by extra-pleural approach. The three groups were compared with respect to operative time and early postoperative complications like pneumonitis, anastomotic leaks and mortality. Odds ratio and Chi square test were used for the statistical analysis. Results: Group A, B and C had 35, 43 and 38 patients respectively. No significant difference was observed in average operative time in the 3 groups. Though incidence of postoperative pneumonitis was higher in group A (28% as compared to group B (13.95% and group C (11.62%, it was not statistically significant (p > 0.005. Anastomotic leak occurred in 7 patients in group A (20%, 6 patients in group B (13.95% and 4 patients (10.52% in group C (p > 0.005. Group A had 3 major and 4 minor anastomotic leaks; group B had 2 major and 4 minor leaks and group C had 1 major and 3 minor leaks. There were10

  4. Esophageal atresia: Factors influencing survival - Experience at an Indian tertiary centre

    Directory of Open Access Journals (Sweden)

    Tandon R

    2008-01-01

    Full Text Available Objective: To study the clinical profile of the cases of esophageal atresia (EA and/or tracheoesophageal fistula (TEF and various factors affecting the surgical and early postoperative management and their outcome. Materials and Methods: A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading. Results: EA with TEF was the commonest type in 117 cases (92%. Associated congenital anomalies were present in 52 (41% patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5% cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival ( P = < 0.001. Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%. Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases. Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C. Conclusion Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.

  5. Congenital aplasia of the optic chiasm and esophageal atresia: a case report

    Directory of Open Access Journals (Sweden)

    Madonia Maurizio

    2011-08-01

    Full Text Available Abstract Introduction The complete absence of the chiasm (chiasmal aplasia is a rare clinical condition. Hypoplasia of the optic nerve and congenital nystagmus are almost invariably associated characteristics. Microphthalmos or anophthalmos are common features in chiasmal aplasia, while central nervous system abnormalities are less frequent. Esophageal atresia can be isolated or syndromic. In syndromic cases, it is frequently associated with cardiac, limb, renal or vertebral malformations and anal atresia. More rarely, esophageal atresia can be part of anophthalmia-esophageal-genital syndrome, which comprises anophthalmia or microphthalmia, genital abnormalities, vertebral defects and cerebral malformations. Here, a previously unreported case of chiasmal aplasia presenting without microphthalmos and associated with esophageal atresia is described. Case presentation Aplasia of the optic chiasm was identified in a Caucasian Italian 8-month-old boy with esophageal atresia. An ultrasound examination carried out at 21 weeks' gestation revealed polyhydramnios. Intrauterine growth retardation, esophageal atresia and a small atrial-septal defect were subsequently detected at 28 weeks' gestation. Repair of the esophageal atresia was carried out shortly after birth. A jejunostomy was carried out at four months to facilitate enteral feeding. The child was subsequently noted to be visually inattentive and to be neurodevelopmentally delayed. Magnetic resonance imaging revealed chiasmal aplasia. No other midline brain defects were found. His karyotype was normal. Conclusion If achiasmia is a spectrum, our patient seems to depict the most severe form, since he appears to have an extremely severe visual impairment. This is in contrast to most of the cases described in the literature, where patients maintain good--or at least useful-- visual function. To the best of our knowledge, the association of optic nerve hypoplasia, complete chiasmal aplasia, esophageal

  6. THORACOSCOPIC REPAIR OF ESOPHAGEAL ATRESIA WITH DISTAL FISTULA IN A NEW BORN: A TECHNICAL CASE REPORT

    Directory of Open Access Journals (Sweden)

    E. Brandigi

    2013-12-01

    Full Text Available Introduction. Esophageal atresia encompasses a group of congenital anomalies comprising of an interruption of the esophageal continuity with or without a persistent communication with the trachea. Esophageal atresia with tracheoesophageal fistula (type C accounts for 85% of all esophageal atresia. Minimally invasive approach to correct esophageal atresia with distal fistula is becoming more generally accepted. The outcome of these technique are critically analyzed and compared with results from open repair. We present one case of type IIIB esophageal atresia treated by a thoracoscopic approach. Case Report. The patient was a 2-days-old infant male, weight 3 kg with esophageal atresia and distal tracheoesophageal fistula without other associated disease. A polidramanios was detected in prenatal age by a prenatal ultrasound evaluation. He underwent to a thoracoscopic repair of the defect. The operation was approached through the right chest using a three-trocar technique (three 5-mm with the patient placed in a three-quarter prone position. The azygos vein was ligated by Ligature device. The fistula was ligated by two resorbable stiches suture and dissected, the proximal esophagus was opened and an anastomosis was made over a 6 Ch nasogastric tube with interrupted and resorbable stiches suture. On the postoperative day 7, gastrografin swallow was performed and oral feeding was started. The patient’s six month upper Gastrointestinal barium studies was normal. Discussion and conclusion. Thoracoscopic repair of esophageal atresia is considered to be one of the more advanced and most difficult pediatric surgical procedures and it undoubtely has necessary an elevated learning curve. The minimally invasive approach was larged accepted in the last ten years also for the well documented sequelae of traditional open repair of esophageal atresia. More experience is needed to determine the exact place of this approach.

  7. Less-invasive MR indices of clinically evident esophageal variceal bleeding in biliary atresia patients

    Directory of Open Access Journals (Sweden)

    Yuan Heng Mo

    2012-09-01

    Conclusion: Less-invasive indices, including the corrected splenic length platelet ratio and the splenic volume index-to-platelet count ratio, may be valuable predictors of esophageal variceal bleeding in patients with biliary atresia.

  8. Dysphagia among Adult Patients who Underwent Surgery for Esophageal Atresia at Birth

    Directory of Open Access Journals (Sweden)

    Valérie Huynh-Trudeau

    2015-01-01

    Full Text Available BACKGROUND: Clinical experiences of adults who underwent surgery for esophageal atresia at birth is limited. There is some evidence that suggests considerable long-term morbidity, partly because of dysphagia, which has been reported in up to 85% of adult patients who undergo surgery for esophageal atresia. The authors hypothesized that dysphagia in this population is caused by dysmotility and/or anatomical anomalies.

  9. Surgical intervention for esophageal atresia in patients with trisomy 18.

    Science.gov (United States)

    Nishi, Eriko; Takamizawa, Shigeru; Iio, Kenji; Yamada, Yasumasa; Yoshizawa, Katsumi; Hatata, Tomoko; Hiroma, Takehiko; Mizuno, Seiji; Kawame, Hiroshi; Fukushima, Yoshimitsu; Nakamura, Tomohiko; Kosho, Tomoki

    2014-02-01

    Trisomy 18 is a common chromosomal aberration syndrome involving growth impairment, various malformations, poor prognosis, and severe developmental delay in survivors. Although esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a potentially fatal complication that can only be rescued through surgical correction, no reports have addressed the efficacy of surgical intervention for EA in patients with trisomy 18. We reviewed detailed clinical information of 24 patients with trisomy 18 and EA who were admitted to two neonatal intensive care units in Japan and underwent intensive treatment including surgical interventions from 1982 to 2009. Nine patients underwent only palliative surgery, including six who underwent only gastrostomy or both gastrostomy and jejunostomy (Group 1) and three who underwent gastrostomy and TEF division (Group 2). The other 15 patients underwent radical surgery, including 10 who underwent single-stage esophago-esophagostomy with TEF division (Group 3) and five who underwent two-stage operation (gastrostomy followed by esophago-esophagostomy with TEF division) (Group 4). No intraoperative death or anesthetic complications were noted. Enteral feeding was accomplished in 17 patients, three of whom were fed orally. Three patients could be discharged home. The 1-year survival rate was 17%: 27% in those receiving radical surgery (Groups 3 and 4); 0% in those receiving palliative surgery (Groups 1 and 2). Most causes of death were related to cardiac complications. EA is not an absolute poor prognostic factor in patients with trisomy 18 undergoing radical surgery for EA and intensive cardiac management.

  10. Weekday vs. weekend repair of esophageal atresia and tracheoesophageal fistula.

    Science.gov (United States)

    Sayari, Arash J; Tashiro, Jun; Wang, Bo; Perez, Eduardo A; Lasko, David S; Sola, Juan E

    2016-05-01

    We hypothesize that weekend esophageal atresia and tracheoesophageal fistula (EA/TEF) repair has worse outcomes compared to procedures performed on weekdays. Kids' Inpatient Database (1997-2009) was searched for EA/TEF in infants admitted at analysis (MVA) compared complications, mortality, and resource utilization (length of stay [LOS] total charges [TC]) between weekday and weekend procedures. Overall, 861 EA/TEF cases with known day of repair were identified. Cohort survival was 96%. On risk-adjusted MVA, complication rates were higher with EA/TEF repair on a weekend (OR: 2.2) compared to a weekday. Additionally, complications (OR: 6.5) and LOS (OR: 9.3) were found to be higher among African American children compared to Caucasians. LOS was higher in patients with Medicaid (OR: 2.4) and repairs performed at non-teaching hospitals (OR: 3.2). Weekend vs. weekday procedure had no significant effect on mortality or resource utilization. By risk-adjusted MVA, increased complication rates for EA/TEF are seen in patients undergoing repair on weekends compared to weekdays. Additionally, African American children experienced higher complication rates compared to Caucasians. LOS after repair varies according to race, payer status, and hospital characteristics. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Non-VACTERL-type anomalies are frequent in patients with esophageal atresia/tracheo-esophageal fistula and full or partial VACTERL association.

    NARCIS (Netherlands)

    Jong, E.M. de; Felix, J.F.; Deurloo, J.A.; Dooren, M.F. van; Aronson, D.C.; Torfs, C.P.; Heij, H.A.; Tibboel, D.

    2008-01-01

    BACKGROUND: The VACTERL association is the nonrandom co-occurrence of Vertebral anomalies, Anal atresia, Cardiovascular malformations, Tracheo-esophageal fistula (TEF) and/or Esophageal atresia (EA), Renal anomalies, and/or Limb-anomalies. The full phenotype of patients with EA/TEF and other anomali

  12. Non-VACTERL-type anomalies are frequent in patients with esophageal atresia/tracheo-esophageal fistula and full or partial VACTERL association.

    NARCIS (Netherlands)

    Jong, E.M. de; Felix, J.F.; Deurloo, J.A.; Dooren, M.F. van; Aronson, D.C.; Torfs, C.P.; Heij, H.A.; Tibboel, D.

    2008-01-01

    BACKGROUND: The VACTERL association is the nonrandom co-occurrence of Vertebral anomalies, Anal atresia, Cardiovascular malformations, Tracheo-esophageal fistula (TEF) and/or Esophageal atresia (EA), Renal anomalies, and/or Limb-anomalies. The full phenotype of patients with EA/TEF and other anomali

  13. An Unusual Case of Foreign Body Lodged in the Laryngopharynx of Neonate with Esophageal Atresia

    Directory of Open Access Journals (Sweden)

    Rahul Gupta

    2016-01-01

    Full Text Available A blunt‑tipped red rubber catheter is used to confirm the presence of esophageal atresia in any newborn with drooling of saliva and frothing from the mouth. Failure to pass it beyond 10cms into the esophagus is considered diagnostic. We here in report an extremely rare case of broken tip of red rubber catheter lodged in the laryngopharynx of 2-day-old neonate of esophageal atresia with distal tracheoesophageal fistula. During endotracheal intubation foreign body was accidentally removed.

  14. Oesophageal elongation with traction sutures (FOKER procedure in a newborn baby with long-gap oesophageal atresia (LGEA: Maybe too early, maybe too dangerous?

    Directory of Open Access Journals (Sweden)

    Holger Till

    2013-01-01

    Full Text Available In children with long gap oesophageal atresia (LGEA, the FOKER technique (oesophageal elongation with traction sutures has been criticized for its high complication rate. We advocate analysing such problems to increase the safety in the future. The present case report will focus on timing. A female newborn (3000 g with LGEA (gap of 5 cm was delivered in an outward hospital. On day two of life, she received traction sutures on both pouches. By day five, all sutures had torn out, and a primary anastomosis was attempted. However, it leaked severely. Thus, on day ten, the oesophagus was approached from the neck converting the proximal end into a spit fistula and closing the distal end blindly. Furthermore, the gastro-oesophageal (GE- junction was wrapped with a Teflon sling. When the baby arrived in our institution, she suffered from cavernous oesophageal masses extending from the thoracic inlet down to the diaphragm and fistulas draining them into the neck as well as into the right lung. Moreover, the Teflon sling had dislodged allowing for GE-reflux. In several stages, the oesophageal remnants were resected without any complications. Finally, Prof. Alaa Hamza performed a colonic interposition, which is working well today. In conclusion, the present case aims to caution paediatric surgeons to apply traction sutures for oesophageal elongation in newborns with LGEA.

  15. [Intraoperative management of esophageal atresia: small steps that cannot be ignored in Madagascar].

    Science.gov (United States)

    Randriamizao, Harifetra Mamy Richard; Rakotondrainibe, Aurélia; Rahanitriniaina, Nadia Marie Philibertine; Rajaonera, Andriambelo Tovohery; Andriamanarivo, Mamy Lalatiana

    2017-01-01

    The management of esophageal atresia is still limited due to the precariousness of technical equipments in Madagascar. Our case study aims to highlight possible therapeutic options and to describe the progresses to be made so as to optimize treatment of this congenital pathology. We collected the medical records of all patients hospitalized for esophageal atresia in the Department of Surgical Reanimation at the University Hospital-JRA Antananarivo. The first patients admitted who survived for a period of 42 months (between January 2011 and June 2014) were included in the study. Among the 17 patients admitted with esophageal atresia, three full-term newborns with type III esophageal atresia, successively admitted in Surgical Reanimation, were the first patients in Madagascar to survive after surgery. Only one patient had associated malformations. These three babies were extubated early in the operating room; they underwent oxygen therapy until normal spontaneous breathing. New-born babies underwent post-operatory physical therapy allowing to improve their respiratory status. Overall mortality rate from this pathology was 76.5% in 42 months. Despite these initial success, progresses still remain to be done in the treatment of this congenital anomaly, from diagnosis up to postoperative period. The improvement of technical equipments, especially ventilation and nutritional support equipments would allow more meaningful results, like in the countries where progresses has been made in the field of reanimation.

  16. Diagnosis and treatment of gastroesophageal reflux in patients with esophageal atresia

    NARCIS (Netherlands)

    J.H.L.J. Bergmeijer (Jan Hein)

    2002-01-01

    textabstractIn the last two decades, surgical treatment of children born with esophageal atresia has become a standard procedure. Postoperative mortality- now negligible in those born at term without other severe anomalies- mainly relates to patients with associated severe congenital cardiac

  17. Unilateral pulmonary agenesis and esophageal atresia with a tracheoesphageal fistula-23 year followup

    Directory of Open Access Journals (Sweden)

    Michael Curci

    2015-04-01

    Full Text Available Pulmonary agenesis, esophageal atresia and a tracheoesophageal fistula (EA + TEF are a rare combined congenital anomalies associated with a high morbidity and mortality. For those patients that have survived these malformation, there has been limited long-term follow up. This case report describes a 23-year followup with evaluation of the patient's pulmonary, cardiac and gastrointestinal function.

  18. Esophageal atresia with tracheoesophageal fistula in a patient with 7q35-36.3 deletion including SHH gene.

    Science.gov (United States)

    Busa, Tiffany; Panait, Nicoleta; Chaumoitre, Kathia; Philip, Nicole; Missirian, Chantal

    2016-10-01

    Terminal 7q deletion is rarely reported in the literature. Holoprosencephaly and sacral dysgenesis are found in association with this deletion, due to haploinsufficiency of SHH and HLBX9 genes respectively. We report on a 2-year-old boy with 7q35-36.3 deletion encompassing SHH identified by oligonucleotide array comparative genomic hybridization. In addition to other frequent features, the patient presented with esophageal atresia and tracheoeosophageal fistula diagnosed at birth. This case, together with two others previously described, one presenting with esophageal atresia, the other with congenital esophageal stenosis, confirms the possible association between congenital esophageal malformations and 7q terminal deletion including SHH.

  19. First Report of A Unique Presentation of Hypertrophic Pyloric Stenosis Following Type I Esophageal Atresia; A Case Report

    Directory of Open Access Journals (Sweden)

    Amrollah Salimi

    2017-06-01

    Full Text Available Combination of congenital esophageal atresia and subsequent hypertrophic pyloric stenosis is a rare condition which occurs in early infancy. The underlying etiology and pathophysiology of this association still remains unclear. In this paper we report a unique case of hypertrophic pyloric stenosis, for the first time, which occurred in an infant who underwent surgery for type I esophageal atresia. Therefore, we intend to highlight the role of poor nutrition intake in the incidence of this condition.

  20. Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence

    DEFF Research Database (Denmark)

    Bjørsum-Meyer, Thomas; Herlin, Morten; Qvist, Niels

    2016-01-01

    Background: The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac...... defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases. Furthermore, we aimed to collect existing knowledge...... in the embryopathogenesis and genetics in order to discuss a possible link between the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome. Case presentation: Our first case was a white girl...

  1. Esophageal Atresia: Migration of the gastrostomy tube into the bronchus

    Directory of Open Access Journals (Sweden)

    Hosseini Seyed Mohammad

    2008-01-01

    Full Text Available A 2-day-old baby boy, 38 weeks gestation, weight 2000 g was brought due to hypersalivation and imperforate anus with gasless abdomen on plain X-ray. He underwent a gastrostomy tube insertion and colostomy. In contrast study of the stomach, on the 5th postoperative day, the dye spilled into the tracheo bronchial tree and the catheter was seen,entering the right main bronchus. The patient underwent right thoracotomy and the presence of fistula and catheter were confirmed. The fistula and distal esophagus were closed and fixed to the prevertebral fascia because of a long gap. He is under follow-up and recieving home care for a later delayed primary anastomosis.

  2. Primary Repair of Esophageal Atresia with Distal Tracheoesophageal Fistula in a Low and ndash;Birth-Weight Neonate

    Directory of Open Access Journals (Sweden)

    Deniz Hanta

    2015-06-01

    Full Text Available Esophageal atresia is complex congenital anomaly of respiratory tract occuring often associated with anomalies of other systems and chromosomal abnormalities. This congenital anomaly is a surgical emergency. The management mode of Esophageal atresia/ Tracheoesophageal Fistula in the premature, low birth weight neonate remains controversial. Traditionally, treatment has been based on a staged approach. The primary repair of Esophageal atresia and fistula closure is achievable in Low Birth Weight infants and offer a good treatment in the absence of additional severe malformations. Treatment of these neonates should not be limited to tertiary pediatric surgical centers, contrarily it is also achievable in secondary heath care centers. Near cooperation with neonatal intensivist and the pediatric surgeon is essential for good survival. [Cukurova Med J 2015; 40(2.000: 340-344

  3. A Novel Technique for Risk Calculation of Anastomotic Leakage after Thoracoscopic Repair for Esophageal Atresia with Distal Fistula

    NARCIS (Netherlands)

    Zee, D.C. van der; Vieira Travassos, D.; Jong, J.R. de; Tytgat, S.H.A.J.

    2008-01-01

    Purpose: This study was designed to determine the risk of anastomotic leakage after thoracoscopic repair for esophageal atresia by digitally measuring the length of the proximal esophagus and distance of carina to proximal esophagus. Methods: With the use of Picture Archiving and Communication Syste

  4. A pilot investigation of feeding problems in children with esophageal atresia.

    Science.gov (United States)

    Baird, R; Levesque, D; Birnbaum, R; Ramsay, M

    2015-04-01

    While many long-term complications of esophageal atresia (EA) have been well investigated, little is known about feeding difficulties in children after surgical correction of EA and its impact on caregivers. This study investigates the feeding behaviors of children with EA through a validated feeding questionnaire. The Montreal Children's Hospital Feeding Scale (MCH-FS) was filled out by the primary caregiver during patient follow-up visits in the multidisciplinary EA clinic. Demographic information, EA subtype, associated anomalies and outcomes were recorded. Results were compared between groups and to a normative sample. Thirty caregivers have completed the MCH-FS; 26 patients had type C atresia (86.7%). In comparison to controls, 17.5% of EA cases are one standard deviation above the mean feeding difficulty score, while 6.7% (n = 2) cases are greater than two standard deviations above normative values. Typical EA patients (type C who were not born Feeding difficulties of patients with typical EA appear mild. Likely explanations include the use of early protocolized care and intensive multidisciplinary care in follow up. Nonetheless, patients with complicated EA (non-type C) and their caregivers tend to experience significant feeding difficulties. Early targeted care may be required for this patient subset, and additional cases will be investigated to confirm these preliminary findings and explore further risk factors of feeding problem in this cohort.

  5. Surgical Outcomes in Esophageal Atresia and Tracheoesophageal Fistula: A Comparison between Primary and Delayed Repair

    Directory of Open Access Journals (Sweden)

    H Davari

    2006-01-01

    Full Text Available Background: The purpose of this study was to investigate outcomes of surgical repair of esophageal atresia (EA or tracheoesophageal fistula (TEF in newborns, with respect to incidence of death and other complications in early or late operations. Methods: Charts of all 80 infants with EA/TEF, operated in Alzahra hospital (A tertiary hospital of Isfahan University of Medical Sciences from 2002 to 2004 were reviewed. Patients were designed in two groups as, primary and delayed repair groups. Patients demographics, frequency of associated anomalies, and details of management and outcomes were studied. Results: There were 48 male and 32 female patients with a frequency of 28(35% preterm infant and mean birth weight of 2473±595 g. Overall survival rate was 71.2%. Mortality rate in delayed repair group was significantly higher than the other one (22.5% vs. 6.3% but with matching, according to full term/preterm proportion, the significant differences were failed. Female sex and being preterm were the most powerful predictors of death (nearly odds ratio=7 for both. Conclusion: in this study mortality and complications rates are higher in delayed repair than early one, although our data proposed that in absence of sever life threatening anomalies the most important factor for death is gestational age and female sex, and primary repair is opposed to it. Although mortality rate and complications are equal in two strategies, with matching cases for being preterm, but primary repair stays the better choice due to economic considerations. Keywords: tracheoesophageal fistula, esophageal atresia, delayed repair, primary repair, outcome

  6. Transthoracic single port with peroral assistance: an animal experiment to assess a less invasive technique for human esophageal atresia repair.

    Science.gov (United States)

    Henriques-Coelho, Tiago; Soares, Tony R; Miranda, Alice; Moreira-Pinto, João; Correia-Pinto, Jorge

    2012-12-01

    Thoracoscopic repair of esophageal atresia has becoming the gold standard in many centers because it allows a better cosmetic result and avoids the musculoskeletal sequelae of a thoracotomy. Natural orifice translumenal endocopic surgery (NOTES) is a new surgical paradigm, and its human application has already been started in some procedures. In the present study, we explore the feasibility of performing an esophagoesophageal anastomosis using a single transthoracic single port combined with a peroral access in a rabbit model to simulate repair of esophageal atresia by hybrid NOTES in a human newborn. Adult male rabbits (Oryctolagus cuniculus, n=28) were used to perform the surgical protocol. We used a transthoracic telescope with a 3-mm working channel and a flexible endoscope with a 2.2-mm working channel by peroral access. We performed total esophagotomy with peroral scissors followed by an esophagoesophageal anastomosis achieved with a rigid transthoracic scope helped by the peroral operator. Extracorporeal transthoracic knots were performed to complete the anastomosis. The anastomoses were examined in loco and ex loco, after animal sacrifice. We successfully accomplished a complete esophageal anastomosis in all rabbits using a combination of transthoracic and peroral 3-mm instruments. This study provides important insights for a possible translation of hybrid NOTES to human newborns with esophageal atresia. Forward studies to accomplish their feasibility in human newborns will still be necessary.

  7. Role of preoperative tracheobronchoscopy in newborns with esophageal atresia:A review

    Institute of Scientific and Technical Information of China (English)

    Filippo; Parolini; Giovanni; Boroni; Stefania; Stefini; Cristina; Agapiti; Tullia; Bazzana; Daniele; Alberti

    2014-01-01

    Preoperative tracheobronchoscopy(TBS) in the diag-nostic assessment of newborns affected by esophageal atresia(EA) was described in 1981. Nevertheless, the value of the procedure is actually much debated; only a few studies have clearly explored the advantages of TBS and this procedure is not yet routinely included inthe diagnostic and therapeutic assessment in many in-ternational pediatric surgery settings. Routine preoper-ative TBS is a safe procedure that enables the accurate examination of the tracheobronchial tree, the visualiza-tion of tracheoesophageal fistula and the diagnosis of tracheomalacia or associated respiratory anomalies. When a distal fistula is found, its occlusion with a Fog-arty balloon catheter improves mechanical ventilation and facilitates surgical repair. This review provides a detailed overview on the use of TBS in newborns with EA, focusing on technical aspects, anesthesiological management, indications and limits. The benefits and risks of the procedure are also compared with alterna-tive diagnostic tools, such as an esophageal contrast study, computed tomography scan and ultrasound.

  8. [Nutrition in infants with esophagocolonoplasty for esophageal atresia and tracheoesophageal fistula].

    Science.gov (United States)

    Hagău, N; Cocu, S; Geczi-Toth, I; Studnicska, D; Ciuce, C

    2008-01-01

    The aim of this clinical prospective study was the follow up of a nutritional management protocol for children with esophageal atresia and tracheoesophageal fistula for whom the esophageal substitution was performed with left vascularized colon. In this study entered infants aged 3 months to 18 months old with a major nutritional deficit, due to respiratory infections complications, parastomal leaks and accelerated gastrointestinal transit. All infants were underweight, with a single exception, who had no preoperative complications. The patient was enterally fed postoperatively, all the other patients receiving combined enteral and parenteral nutrition for 5-6 days. The enteral nutrition was delivered early through a trans-anastomotic feeding tube. In the 5th-7th day, complete enteral nutrition was obtained. The parenteral nutrition followed our own recipe: a 10% amino-acid mixture, 50% glucose and Ringer plus electrolytes and vitamins. There was a critical transitional stage between the gastric tube feeding and the oral nutrition. These infants have the suction and the deglutition reflexes modified, followed by oral sensory and motor deficits. After the release from the hospital the patients have been surveyed, the oldest reaching now the age of 7. The earlier the reconstruction was performed, the less problems in oral nutrition were encountered.

  9. Gaseous distention of the hypopharynx and cervical esophagus with nasal CPAP: a mimicker of pharyngeal perforation and esophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Walor, David; Berdon, Walter; Holt, Peter D.; Fox, Matthew [Columbia University Medical Center, Department of Radiology, New York, NY (United States); Children' s Hospital of New York, New York, NY (United States); Anderson, Nicole [Columbia University Medical Center, Department of Neonatology, New York, NY (United States); Children' s Hospital of New York, New York, NY (United States)

    2005-12-01

    Nasal continuous positive airway pressure (CPAP) has been used since 1975 as the initial treatment for respiratory distress syndrome (RDS) in very premature infants. Gaseous distention of the abdomen (CPAP belly) is a common secondary effect of CPAP. Gaseous distention of the hypopharynx is also common. To determine the incidence of hypopharyngeal distention in infants on CPAP. We performed a retrospective review of the chest radiographs of 57 premature infants treated with CPAP during a 4-week period to find the presence and degree of hypopharyngeal distention. Of the 57 radiographs, 14 (25%) revealed gaseous distention of the hypopharynx and/or cervical esophagus. On occasion, this raised concern for pharyngeal perforation or esophageal atresia. Awareness that CPAP-related hypopharyngeal distention is common should help radiologists avoid erroneous consideration of esophageal atresia or hypopharyngeal perforation. (orig.)

  10. Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association with Mayer-Rokitansky-Küster-Hauser syndrome in co-occurrence: two case reports and a review of the literature.

    Science.gov (United States)

    Bjørsum-Meyer, Thomas; Herlin, Morten; Qvist, Niels; Petersen, Michael B

    2016-12-21

    The vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome are rare conditions. We aimed to present two cases with the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser co-occurrence from our local surgical center and through a systematic literature search detect published cases. Furthermore, we aimed to collect existing knowledge in the embryopathogenesis and genetics in order to discuss a possible link between the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome. Our first case was a white girl delivered by caesarean section at 37 weeks of gestation; our second case was a white girl born at a gestational age of 40 weeks. A co-occurrence of vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect association and Mayer-Rokitansky-Küster-Hauser syndrome was diagnosed in both cases. We performed a systematic literature search in PubMed ((VACTERL) OR (VATER)) AND ((MRKH) OR (Mayer-Rokitansky-Küster-Hauser) OR (mullerian agenesis) OR (mullerian aplasia) OR (MURCS)) without limitations. A similar search was performed in Embase and the Cochrane library. We added two cases from our local center. All cases (n = 9) presented with anal atresia and renal defect. Vertebral defects were present in eight patients. Rectovestibular fistula was confirmed in seven patients. Along with the uterovaginal agenesis, fallopian tube aplasia appeared in five of nine cases and in two cases ovarian involvement also existed. The co-occurrence of the vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal

  11. Distribution of Interstitial Cells of Cajal in the Esophagus of Fetal Rats with Esophageal Atresia

    Directory of Open Access Journals (Sweden)

    Caner Isbir

    2016-04-01

    Full Text Available Aim: Scarcity of the interstitial cells of Cajal (ICC is related to motility disorders. In the study, we aimed to evaluate the number and density of ICCs in the fetal rat esophagus in the adriamycin - esophageal atresia (EA model. Material and Method: Rat fetuses were divided into three groups as a control, adriamycin group without EA and adriamycin group with EA. Four doses of adriamycin, 2 mg/kg each, were injected intraperitoneally to the adriamycin group rats between on 6 and 9 days of gestation. The presence of ICCs in the esophagus of the rat fetuses was determined by using an immunohistochemistry technique (c-kit, CD117. The average numbers of ICCs were calculated with microscopic evaluation by using a visual scoring system (range1 to 3. Results: Seven fetuses were included in each group. The ICCs score 3 distributions of fetuses were 5 (72% fetuses in the control group, 3 (43% fetuses in the adriamycin group without EA, 1 (14% fetus in the adriamycin group with EA. It have been found that there was a marked reduction of ICCs distribution in the adriamycin group with EA compared to control group (p 0.05. Discussion: ICCs density was significantly decreased in the rat fetuses with EA compared to the fetuses without EA. These findings support the idea that ICCs density may be congenitally abnormal in EA. This may be led to dismotility seen in the operated esophagus due to EA.

  12. Impaired VEGF Signaling in Lungs with Hypoplastic Esophageal Atresia and Effects on Branching Morphogenesis

    Directory of Open Access Journals (Sweden)

    Xiaomei Liu

    2016-07-01

    Full Text Available Background/Aims: Patients with esophageal atresia (EA and tracheoesophageal fistula (TEF often suffer chronic respiratory tract disease. We previously reported that primary lung maldevelopment caused by deficient branching of embryonal airways in experimental EA-TEF was induced by Adriamycin. In this study, we investigated the Vascular endothelial growth factor (VEGF pathway in the developing lung in an EA-TEF rat model. We further analyzed the effect of recombinant VEGF treatment in vitro on branching morphogenesis of embryo lungs in experimental EA-TEF. Methods: Pregnant rats received either Adriamycin or vehicle on E7, E8 and E9. Lungs were recovered at E15, E18 and E21. Expression of VEGF and receptors (Flk-1 and Flt-1 were assessed by quantitative PCR, immunohistochemistry and immunoblotting. E13 lungs were cultured for 72 hours with 50 ng/mL of recombinant rat VEGF in serum-free medium. The rates of increase in bud count and airway contour were evaluated. Results: Our results showed a significant downregulation of VEGF during pseudoglandular and canalicular stages. In contrast, there were significantly higher levels of the Flt-1 receptor in the canalicular stage, which may represent a compensatory response to decreased VEGF. However, both variables returned to normal levels at the saccular stage. Exogenous VEGF treatment enhanced hypoplastic lung growth, evidenced by the increase in bud count and airway contour. Conclusions: A VEGF signaling defect possibly plays an important role in defective embryonic airway branching. Additionally, VEGF treatment may accelerate lung growth in EA-TEF lungs.

  13. An assessment of quality of life of operated cases of esophageal atresia in the community

    Directory of Open Access Journals (Sweden)

    Harshjeet Singh Bal

    2016-01-01

    Full Text Available Aims: To evaluate the outcome of the operated children of esophageal atresia (EA focusing on their early and late morbidity and mortality and quality of life (QoL of survivors. Settings and Design: A cross-sectional follow-up with retrospective analysis of available medical and surgical records of children who underwent repair for EA. Materials and Methods: The medical records of the children who underwent repair for EA during the period from 2000 to 2011 at the Christian Medical College Hospital, Vellore, were collected retrospectively. Patients with parents were invited to visit the hospital for follow-up and nutritional status, digestive and respiratory symptoms, status of associated anomalies and QoL assessment of children done. QoL assessment was done using the PedsQL™ 4.0 generic core scales questionnaire comprising 4 scale scores: physical, emotional, social functioning, and school functioning. Mean scores are calculated based on a 5-point response scale for each item and transformed to a 0-100 scale with a higher score representing better QoL. Statistical Analysis Used: Statistical Package for Social Sciences (SPSS version 16 using Chi-square or Fisher′s exact test. Results: Of 79 patients operated during the said period, there were 10 deaths and a total of 69 (87% children survived. Of the 66 patients available for follow-up, we interviewed 30 parents and children while for the remaining 36 children, out-patients charts were reviewed retrospectively. Mean follow-up duration was 3.56 years. The height and weight for age measurement showed 47% and 56% of children respectively as below the 5 th percentile. Main problems faced by operated EA children were of the respiratory (26% and gastroesophageal (36% tracts. In spite of the mentioned problems faced, the overall QoL of this group appeared good. In 23 of 30 patients, who answered PedsQL™ , more than 70% had scores >85 out of 100 in QoL scoring. Conclusions: While survivals of the

  14. Comparison of neonatal tolerance to thoracoscopic and open repair of esophageal atresia with tracheoesophageal fistula

    Institute of Scientific and Technical Information of China (English)

    MA Li; LIU Yong-zhe; MA Ya-qun; ZHANG Sheng-suo; PAN Ning-ling

    2012-01-01

    Background Advances in minimally invasive surgical techniques and neonatal intensive care for neonates have allowed for repair of the neonatal esophageal atresia with tracheoesophageal fistula (EA/TEF) to be approached endoscopically.However,thoracoscopic surgery in children is still performed in only a few centers throughout the world.The aim of this study was to compare the neonatal tolerance to the thoracoscopic repair (TR) and the open repair (OR)and also to discuss anesthetic management in thoracoscopic procedure.Methods We performed a prospective study enrolling newborns diagnosed with EA with distal TEF (type C) receiving the repair surgery between June 2009 and January 2012 in our institution.Data collected included the newborns' gestational age and weight at the time of the operation,operative time,parameters of intraoperative mechanical ventilation,oxygenation,end-tidal carbon dioxide (ETCO2),and analysis of blood gases.Time to extubation and length of stay were also recorded.Results Intravenous induction with muscle paralysis followed by pressure-control ventilation and tracheal intubation regardless of the position of the fistula can be performed uneventfully in EA/TEF newborns with no additional airway anomalies and large,pericarinal fistulas in our experiences.The thoracoscopic approach appeared to take longer than the open approach.During the procedure of repair,hypercarbia and acidosis developed immediately 1 hour after pneumothorax in both groups.CO2 insufflation did have additional influence on the respiratory function of the newborns in the TR group; values of PaCO2 and ETCO2 were higher in the TR group but the difference did not reach statistical significance.By the end of the procedure,values of PaCO2 and ETCO2 returned to the baseline levels while pH did not,but all parameters made no difference in the two groups.Besides,time to extubation was shorter in the TR group.Conclusions Thoracoscopic repair of EA/TEF is comparable to the open repair

  15. Endoscopic Stenting and Clipping for Anastomotic Stricture and Persistent Tracheoesophageal Fistula after Surgical Repair of Esophageal Atresia in an Infant

    Directory of Open Access Journals (Sweden)

    Mohammed Amine Benatta

    2014-01-01

    Full Text Available Anastomotic stricture (AS and recurrent tracheoesophageal fistula (TEF are two complications of surgical repair of esophageal atresia (EA. Therapeutic endoscopic modalities include stenting, tissue glue, and clipping for TEF and endoscopic balloon dilation bougienage and stenting for esophageal strictures. We report herein a two-month infant with both EA and TEF who benefited from a surgical repair for EA, at the third day of life. Two months later he experienced deglutition disorders and recurrent chest infections. The esophagogram showed an AS and a TEF confirmed with blue methylene test at bronchoscopy. A partially covered self-expanding metal type biliary was endoscopically placed. Ten weeks later the stent was removed. This allows for easy passage of the endoscope in the gastric cavity but a persistent recurrent fistula was noted. Instillation of contrast demonstrated a fully dilated stricture but with a persistent TEF. Then we proceeded to placement of several endoclips at the fistula site. The esophagogram confirmed the TEF was obliterated. At 12 months of follow-up, he was asymptomatic. Stenting was effective to alleviate the stricture but failed to treat the TEF. At our knowledge this is the second case of successful use of endoclips placement to obliterate recurrent TEF after surgical repair of EA in children.

  16. Alternations in genes expression of pathway signaling in esophageal tissue with atresia: results of expression microarray profiling.

    Science.gov (United States)

    Smigiel, R; Lebioda, A; Blaszczyński, M; Korecka, K; Czauderna, P; Korlacki, W; Jakubiak, A; Bednarczyk, D; Maciejewski, H; Wizinska, P; Sasiadek, M M; Patkowski, D

    2015-04-01

    Esophageal atresia (EA) is a congenital defect of the esophagus involving the interruption of the esophagus with or without connection to the trachea (tracheoesophageal fistula [TEF]). EA/TEF may occur as an isolated anomaly, may be part of a complex of congenital defects (syndromic), or may develop within the context of a known syndrome or association. The molecular mechanisms underlying the development of EA are poorly understood. It is supposed that a combination of multigenic factors and epigenetic modification of genes play a role in its etiology. The aim of our work was to assess the human gene expression microarray study in esophageal tissue samples. Total RNA was extracted from 26 lower pouches of esophageal tissue collected during thoracoscopic EA repair in neonates with the isolated (IEA) and the syndromic form (SEA). We identified 787 downregulated and 841 upregulated transcripts between SEA and controls, and about 817 downregulated and 765 upregulated probes between IEA and controls. Fifty percent of these genes showed differential expression specific for either IEA or SEA. Functional pathway analysis revealed substantial enrichment for Wnt and Sonic hedgehog, as well as cytokine and chemokine signaling pathways. Moreover, we performed reverse transcription polymerase chain reaction study in a group of SHH and Wnt pathways genes with differential expression in microarray profiling to confirm the microarray expression results. We verified the altered expression in SFRP2 gene from the Wnt pathway as well as SHH, GLI1, GLI2, and GLI3 from the Sonic hedgehog pathway. The results suggest an important role of these pathways and genes for EA/TEF etiology. © 2014 International Society for Diseases of the Esophagus.

  17. High incidence of fracture events in patients with Long-Gap Esophageal Atresia (LGEA: A retrospective review prompting implementation of standardized protocol

    Directory of Open Access Journals (Sweden)

    Sigrid Bairdain

    2015-12-01

    Conclusion: Episodes of paralysis appeared to be the most significant risk factor for fractures in patients with LGEA who underwent the Foker procedure. The incidence of symptomatic fractures decreased significantly following implementation of a standardized protocol in this series of LGEA patients with continued prospective evaluation.

  18. Evaluación de una serie de pacientes con atresia del esófago, durante un período de 5 años Evaluation of a series of patients with esophageal atresia, during a 5-year period

    Directory of Open Access Journals (Sweden)

    Francisco Javier Mejía Sarasti

    2003-03-01

    Full Text Available En un período de 5 años comprendido entre el 1 de enero de 1997 y el 31 de diciembre de 2001 se presentaron 73 pacientes con atresia del esófago los cuales fueron intervenidos quirúrgicamente. La edad gestacional promedio fue 37 semanas y el peso promedio al nacer, 2.458 gramos; el 88% de estos pacientes tuvieron atresia esofágica con fístula traqueoesofágica distal y el 11% no presentaron fístula. El tratamiento quirúrgico en la mayoría de los pacientes fue la resección de la fístula con anastomosis primaria del esófago y se realizaron ascensos gástricos en 3 neonatos con atresia del esófago sin fístula con cabos muy separados. Durante el período de estudio no se presentaron complicaciones intraoperatorias. La complicación postquirúrgica más frecuente fue la estenosis que se evidenció en 27 casos. La sobrevida global al período neonatal fue del 83%. La frecuencia de neumonía antes de llevar los pacientes a cirugía fue del 63% y tuvo relación con la edad en días al momento de la cirugía. La mortalidad de nuestros pacientes se encontró asociada con el peso al nacer y la presencia de otras malformaciones congénitas. Se concluyó que esta serie de pacientes tiene las mismas características descritas en la literatura mundial y que el tratamiento quirúrgico que les hemos dado ha tenido un éxito semejante al obtenido en otros lugares del mundo. Between january 1ST 1997 and December 31st 2001 we attended 73 patients with the diagnosis of esophageal atresia at San Vicente de Paúl Hospital, in Medellín, Colombia. Average gestational age was 37 weeks and average weight, 2458 grams; 88% of these patients had also distal tracheo-esophageal fistula while in 11% there was no fistula. The most common treatment was the resection of the fistula plus primary correction through termino-terminal anastomosis. We performed 3 gastric ascents in cases without fistula with a long gap atresia. There were no intraoperative complications

  19. Esophageal atresia associated with anorectal malformation: Is the outcome better after surgery in two stages in a limited resources scenario?

    Directory of Open Access Journals (Sweden)

    Sunita Singh

    2012-01-01

    Full Text Available Aims: To analyze whether outcome of neonates having esophageal atresia with or without tracheoesophageal fistula (EA±TEF associated with anorectal malformation (ARM can be improved by doing surgery in 2 stages. Materials and Methods : A prospective study of neonates having both EA±TEF and ARM from 2004 to 2011. The patients with favorable parameters were operated in a single stage, whereas others underwent first-stage decompression surgery for ARM. Thereafter, once septicemia was under control and ventilator care available, second-stage surgery for EA±TEF was performed. Results: Total 70 neonates (single stage = 20, 2 stages = 30, expired after colostomy = 9, only EA±TEF repair needed = 11 were enrolled. The admission rate for this association was 1 per 290. Forty-one percent (24/70 neonates had VACTERL association and 8.6% (6/70 neonates had multiple gastrointestinal atresias. Sepsis screen was positive in 71.4% (50/70. The survival was 45% (9/20 in neonates operated in a single stage and 53.3% (16/30 when operated in 2 stages (P = 0.04. Data analysis of 50 patients revealed that the survived neonates had significantly better birth weight, better gestational age, negative sepsis screen, no cardiac diseases, no pneumonia, and 2-stage surgery (P value 0.002, 0.003, 0.02, 0.02, 0.04, and 0.04, respectively. The day of presentation and abdominal distension had no significant effect (P value 0.06 and 0.06, respectively. This was further supported by stepwise logistic regression analysis. Conclusions: In a limited resources scenario, the survival rate of babies with this association can be improved by treating ARM first and then for EA±TEF in second stage, once mechanical ventilator care became available and sepsis was under control.

  20. [Medium-term outcome, follow-up, and quality of life in children treated for type III esophageal atresia].

    Science.gov (United States)

    Lepeytre, C; De Lagausie, P; Merrot, T; Baumstarck, K; Oudyi, M; Dubus, J-C

    2013-10-01

    The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65%), asthma before the age of 3 years (66%), hospitalization for a respiratory event (45%), fundoplication (20%), and esophageal dilatation (45%). We noted current chronic cough (16%), asthma (30%), dysphagia (39%), and symptomatic gastroesophageal reflux (9%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good.

  1. Comparison of environmental risk factors for esophageal atresia, anorectal malformations, and the combined phenotype in 263 German families.

    Science.gov (United States)

    Zwink, N; Choinitzki, V; Baudisch, F; Hölscher, A; Boemers, T M; Turial, S; Kurz, R; Heydweiller, A; Keppler, K; Müller, A; Bagci, S; Pauly, M; Brokmeier, U; Leutner, A; Degenhardt, P; Schmiedeke, E; Märzheuser, S; Grasshoff-Derr, S; Holland-Cunz, S; Palta, M; Schäfer, M; Ure, B M; Lacher, M; Nöthen, M M; Schumacher, J; Jenetzky, E; Reutter, H

    2016-11-01

    Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) and anorectal malformations (ARM) represent the severe ends of the fore- and hindgut malformation spectra. Previous research suggests that environmental factors are implicated in their etiology. These risk factors might indicate the influence of specific etiological mechanisms on distinct developmental processes (e.g. fore- vs. hindgut malformation). The present study compared environmental factors in patients with isolated EA/TEF, isolated ARM, and the combined phenotype during the periconceptional period and the first trimester of pregnancy in order to investigate the hypothesis that fore- and hindgut malformations involve differing environmental factors. Patients with isolated EA/TEF (n = 98), isolated ARM (n = 123), and the combined phenotype (n = 42) were included. Families were recruited within the context of two German multicenter studies of the genetic and environmental causes of EA/TEF (great consortium) and ARM (CURE-Net). Exposures of interest were ascertained using an epidemiological questionnaire. Chi-square, Fisher's exact, and Mann-Whitney U-tests were used to assess differences between the three phenotypes. Newborns with isolated EA/TEF and the combined phenotype had significantly lower birth weights than newborns with isolated ARM (P = 0.001 and P expectation, and warrants further analysis in large prospective epidemiological studies.

  2. Determinants of gap length in esophageal atresia with tracheoesophageal fistula and the impact of gap length on outcome

    Directory of Open Access Journals (Sweden)

    Muffazzal Rassiwala

    2016-01-01

    Full Text Available Aim: This study was aimed at identifying factors which may affect the gap length in cases of esophageal atresia with tracheoesophageal fistula (EA-TEF and whether gap length plays any role in determining the outcome. Materials and Methods: All consecutive cases of EA-TEF were included and different patient parameters were recorded. Plain radiographs with a nasogastric tube in the upper esophagus were taken. Patients were grouped into T1-T2; T2-T3; T3-T4; and T4 depending on the thoracic vertebral level of the arrest of the tube. During surgery, the gap length between the pouches was measured using a Vernier caliper and the patients were grouped into A, B, and C (gap length >2.1 cm; >1-≤2 cm and ≤1 cm. The operative gap groups were compared with the radiography groups and the other recorded parameters. Results: Total numbers of cases were 69. Birth weight was found to be significantly lower in Group A (mean = 2.14 kg as compared to Group B (mean = 2.38 kg and Group C patients (mean = 2.49 kg (P = 0.016. The radiographic groups compared favorably with the intraoperative gap length groups (P < 0.001. The need for postoperative ventilation (70.83% in Group A vs. 36.84% in Group C, P = 0.032 and mortality (62.5%, 26.9% and 15.8% in Group A, B, and C, respectively, P = 0.003 co-related significantly with the gap length. Conclusion: Birth weight had a direct reciprocal relationship with the gap length. Radiographic assessment correlated with intraoperative gap length. Higher gap length was associated with increased need for postoperative ventilation and poor outcome.

  3. Esophageal Strictures in Children

    Directory of Open Access Journals (Sweden)

    Ahmad Bazrafshan

    2014-07-01

    Conclusion: Anastomotic stricture after surgical repair of esophageal atresia comprised the most common cause of esophageal stricture. Proximal esophagus was the most common site of stricture. Most of the patients recovered with dilatation, surgery, or a combination of the two.

  4. The Evaluation of Standardized Nursing Model in Congenital Esophageal Atresia Postoperative Care%标准化护理模式在先天性食道闭锁患儿术后护理的效果评价

    Institute of Scientific and Technical Information of China (English)

    殷茵

    2015-01-01

    目的:总结标准化护理模式在32例新生儿先天性食道闭锁术后的护理经验。具体包括综合情况,管道护理,呼吸道护理和并发症观察。方法分析自2007年2月~2014年6月我科收治的先天性食道闭锁患儿,对是否使用标准化护理模式分组使用χ2检验和 FISH 确切改良法评估。结果标准化护理模式的不良事件发生率明显低于对照组。结论标准化护理模式可明显提高先天性食道闭锁术后患儿护理质量。%Objective To standardize models of care in 32 cases of congenital esophageal atresia of nursing experience. Including the consolidation of pipes care, respiratory care and complications encountered. Methods Since February 2007 to June 2014 congenital esophageal admitted to our department atresia, whether using a standardized model of care group using chi-square test and FISH exact assessment of the improved method. Results Standardized care model incidence of adverse events was significantly lower than the control group. Conclusion The standardized model of care can significantly improve the care quality of children with congenital esophageal atresia.

  5. Biliary Atresia

    Science.gov (United States)

    ... Liver Disease & NASH Definition & Facts Symptoms & Causes Diagnosis Treatment Eating, Diet, & Nutrition Clinical Trials Biliary Atresia Cirrhosis Hemochromatosis Hepatitis A through E (Viral Hepatitis) Hepatitis ...

  6. Validation of an inanimate low cost model for training minimal invasive surgery (MIS) of esophageal atresia with tracheoesophageal fistula (AE/TEF) repair.

    Science.gov (United States)

    Maricic, Maximiliano Alejo; Bailez, María Marcela; Rodriguez, Susana P

    2016-09-01

    We present the results of the validation of an inanimate model created for training thoracoscopic treatment of esophageal atresia with lower tracheoesophageal fistula (EA/TEF). We used different domestic materials such as a piece of wood (support), corrugated plastic tubes (PVC) of different sizes to simulate ribs, intercostal spaces, trachea and spine and tubular latex balloons to simulate the esophagus and lungs to make the basic model. This device was inserted into the thoracic cavity of a rubber dummy simulating a 3kg newborn with a work area volume of 50ml. The model was designed taking into account the experience of doing this procedure in neonates. The cost of the materials used was 50 US$. Regular video endoscopic equipment and 3mm instruments were used. Thirty-nine international faculty or pediatric surgeons attending hands on courses with different levels of training in minimal invasive surgery (MIS) repair of EA/TEF performed the procedure in the model. We compared the performance of the practitioners with their experience in thoracoscopic repair of EA. A Likert-type scale was used to evaluate results. Previous experience in MIS, anatomical appearance of the model, surgical anatomy compared to a real patient, and utility as a training method were analyzed. We also used a checklist to assess performance. We evaluated: number of errors and types of injuries, quality of the anastomosis, and duration of procedure. To analyze the results we used a T-test, chi-square test and Excel® database to match up some results. Thirty-nine questionnaires were completed. Seven surgeons were experts (≥30 TEF/EA repairs as surgeon), 10 had intermediate level of experience (5 to 29 repairs as surgeon) and 22 were beginners (less than 5 repairs). To simplify the analysis we divided the respondents into low experience LE (MIS operative room setting and team work. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Surgical treatment of congenital esophageal atresia in neonates%新生儿食管闭锁的外科治疗

    Institute of Scientific and Technical Information of China (English)

    潘征夏; 吴春; 李洪波; 王刚; 李勇刚; 代江涛; 安永; 杨杰先

    2011-01-01

    目的 总结新生儿食管闭锁的诊断和治疗经验.方法 回顾性分析我院2002年6月至2010年6月收治的新生儿食管闭锁61例,男34例,女27例.手术年龄18 h~7 d,平均(2.5±0.6)d,体重1 500~4 000 g,其中低体重儿(<2 500g)16例.61例中按Gross病理解剖分类:Ⅰ型2例,Ⅲa型32例,Ⅲb型27例,Ⅲa型中有1例远端食管有局限狭窄,开口约0.2 cm;合并畸形:先天性心脏病15例,肠道畸形4例,泌尿系畸形3例.结果 61例中2例Ⅰ型食管闭锁先行近端食管引流、胃造瘘,2周后行结肠代食管手术,59例Ⅲ型均Ⅰ期食管气管瘘切断缝扎、食管端端吻合术,Ⅲa型中远端食管有1例局限性狭窄,行纵切横缝解除狭窄.术后并发单侧或双侧严重肺炎42例,硬肿症3例,近期吻合口狭窄24例,吻合口瘘3例.除1例术后2d合并肠穿孔死亡,3例放弃治疗(均为早期病例:1例为术后1周出现核黄疸,2例术后合并严重肺部感染不能脱离呼吸机),余57例均痊愈出院.术后随访3个月~8年,轻度胃食管反流3例,余均进食良好,生长发育正常.结论 尽早诊断、及时手术,积极预防和治疗并发症,新生儿食管闭锁可取得良好的效果.%Objective To summarize the diagnosis and treatment for congenital esophageal atresia (CEA) in neonates. Methods From June 2002 to June 2010, 61 neonates with congenital esophageal atresia underwent surgery at this center. Of these patients, 34 were boys and 27 were girls. Their age ranged from 18 hours to 7 days (mean, 2. 5 ±0. 6 days). Their weight ranged from 1500 grams to 4000 grams. Sixteen patients were very low-birth-weight infants (<1500 g). According to the anomalies of CEA, 2 were diagnosed with type Ⅰ CEA, and the other 59 were type Ⅲ CEA (32 type Ⅲa and 27 type Ⅲb). The most common associated anomalies were cardiac anomalies (16, 26%), followed by intestinal anomalies (5, 8%) and renal anomalies (3, 5%). Results The 2 cases with type Ⅰ CEA underwent

  8. 食管闭锁术后食管再手术的临床探讨%Re-operation after the primary repair for patients with esophageal atresia

    Institute of Scientific and Technical Information of China (English)

    沈淳; 郑珊; 肖现民

    2012-01-01

    Objective The aim of the study is to summarize the clinical experience of re-operation after esophageal atresia (EA) primary repair.Methods From 2005.6 ~ 2011.7,8 cases of reoperation were performed in our hospital after primary esophageal atresia repair.There were 6 males to 2 females.Patients' ages ranged from 6m to 52 m 7 cases were Gross type Ⅲ and 1was Gross type Ⅰ.Thepresenting complaints were coughing after feeding or feeding difficulty.6 cases had TEF recurrence and 2 cases had severe anastomotic stricturerefractory to dilatation.All cases had pre-operative esophagogram and esophagoscopic examinations.4 also had bronchoscopy.Single lung ventilation was required in 6 patients during procedure.All cases had access via right-sided thoracotomy.Results The average re-operation time was 3.5 h,and the average stay in hospital was 25 d.All patients stayed in PICU for several days.Follow-up period ranged from 1-72 m.No anastomotic leakage or recurrence of fistula occurred.1patient needed anastomotic dilatation once.Conclusions Factors such as not cutting the fistula in primary repair,high tension,leakage and inflammation couldall cause TEFR and AS.TEFR and/or serious AS are the indications for re-operation after EA primary repair.Esophagoscopy and bronchoscopy before re-operation are very important for TEFR diagnosis and treatment.Unilateral lung ventilation during anaesthesia procedure can provide more security for patients.In general,prognosis was good after re-operation.%目的 分析总结食管闭锁术后食管再手术的临床经验.方法 2005年6月至2011年7月,8例接受食管闭锁术后食管再手术患儿,男6例,女2例.再手术时年龄6~52个月.7例GrossⅢ型,1例Gross Ⅰ型.再手术前表现为进食后呛咳及进食困难.6例食管气管瘘复发,2例严重狭窄反复扩张无效.8例行食管造影和食管镜检查瘘管及狭窄情况;4例行支气管镜检查.6例术中单肺通气.8例经右胸再手术,切断、缝扎

  9. Esophageal Atresia and Tracheoesophageal Fistula

    Science.gov (United States)

    ... nav nav, .header-9#header-section #main-nav, #overlay-menu nav, #mobile-menu, #one-page-nav li . ... How to Care for Your Child’s TeethRead Article >>Dental Hygiene: How to Care for Your Child’s TeethSeptember ...

  10. [Biliary atresia in children].

    Science.gov (United States)

    Famulski, W; Sobaniec-Lotowska, M; Sulkowski, S; Ostapiuk, H; Kemona, A

    1989-05-01

    Congenital biliary tract atresia was found in 0.58% of children dying in the first year of life. Most frequently atresia included the extrahepatic bile ducts, among them the common bile duct. Five cases (35.7%) had atresia associated with other developmental anomalies, which may suggest a genetic determination of the atresia. The most frequent cause of death of the newborns and infants with congenital biliary tract atresia was bronchopneumonia with associated biliary cirrhosis of the liver.

  11. Jejunum ileal intestinal atresia.

    Directory of Open Access Journals (Sweden)

    Claudio J. Puente Fonseca

    2005-12-01

    Full Text Available The intestinal atresia is one of the most important causes of intestinal obstruction in newborn. They constitute aorund 95% of total intestinal obstructions in this age group. Most of intestinal atresias are jejunoieal atresia. Although it is not frequent their relationship with other congenital anomalies, has been described the association in some cases with defects of intestine rotation, meconium peritonitis, with meconium ileus and rarely with the Hirschsprung diseases. The hereditary character has also been described in certain multiple intestinal atresias. We presented the Good Clinical Practices Guideline for Jejunoileal atresia, approved by consensus in the 1st National Good Clinical Practices Workshop in Pediatric Surgery (Cienfuegos, Cuba, March 7 – 9, 2002.

  12. Tracheoesophageal fistula and esophageal atresia repair

    Science.gov (United States)

    ... breathing machine (ventilator). The care team may use suction to keep fluids from going into the lungs. ... through the skin into the chest wall) to drain fluids from the space between the outside of ...

  13. 食管闭锁术后食管气管瘘复发再手术的临床分析%Clinical analysis of reoperation for esophageal atresia with recurrent tracheoesophageal fistulas

    Institute of Scientific and Technical Information of China (English)

    朱海涛; 沈淳; 肖现民; 董岿然; 郑珊

    2014-01-01

    目的:分析食管闭锁(EA)术后食管气管瘘复发(RTEF)再手术的临床资料,总结RTEF的原因、诊断方法及再手术的经验。方法回顾性分析2005年1月至2013年12月我们收治的10例EA术后RTEF患儿的临床资料,其中男7例,女3例,再手术平均年龄为19个月,平均体重为8.3 kg,所有病例均为GrossⅢ型;10例中有9例曾接受经右胸食管气管瘘结扎+食管端端吻合术。结果在初次手术中有4例(4/10)瘘管并未切断。9例(9/10)复发瘘管的食管端开口于原食管吻合口狭窄处或近端食管。8例(8/10)术后存在不同程度吻合口狭窄,1例合并吻合口瘘。再手术前所有患儿行食管欧乃派克造影检查,确诊和疑似RTEF分别为5例(5/10,50%)、4例(4/10,40%)。所有RTEF通过胃镜及支气管镜确诊(10/10,100%)。患儿均接受再次进胸手术,平均手术时间3.2 h,平均术后住院时间为15.8 d。术后均获随访3~96个月,食管造影提示所有病例均治愈,未再发生RTEF。结论 EA术后RTEF与初次手术瘘管处理不当、食管吻合口局部炎症反应等因素有关,需再次手术治疗,再手术的疗效及患儿预后良好。%Objetive To summarize the reoperative experience for esophageal atresia (EA)with recur-rent tracheoesophageal fistulas (RTEF). Methods A retropective analysis was given to the 1 0 RTEF patients underwent reoperation in our center from Jan.2005 and Dec.2013.Male to female ratio was 4:1.The median age and average weight at reoperation were 19 months and 8.3 kilograms respectively.All cases were Gross type Ⅲ.9 cases underwent the primary repair of EA. Results During the primary repair,TEF were not cut in 4/1 0 cases.Esophageal orifices of the fistula in 9 of 1 0 cases were located around the anastomotic site or the proximal esophagus.8 cases had anastomotic stricture and 1 case had anastomotic leakage after the primary re

  14. Passage of nasogastric tube through tracheo-esophageal fistula into stomach: A rare event.

    Science.gov (United States)

    Kamble, Ravikiran Shankar; Gupta, Rahulkumar; Gupta, Abhaya; Kothari, Paras; Dikshit, K Vishesh; Kesan, Krishnakumar; Mudkhedkar, Kedar

    2014-07-16

    Esophageal atresia with tracheo-oesophageal fistula (TEF) occurs in 1 in 3500 live births. Anorectal malformation is found to be associated with 14% of TEF. Esophageal atresia with TEF is a congenital anomaly which classically presents as excessive frothing from the mouth and respiratory distress. Rarely gastric position of the feeding tube in a case of TEF can be obtained delaying the diagnosis of TEF. We had an uncommon situation where a nasogastric tube reached the stomach through the trachea and tracheo-esophageal fistula, leading to misdiagnosis in a case of esophageal atresia with tracheoesophageal fistula. By using a stiff rubber catheter instead of a soft feeding tube for the diagnosis of esophageal atresia and TEF, such situation can be avoided.

  15. 不同卧位结合呼吸机辅助呼吸支持管理在预防先天性食道闭锁术后并发症的临床效果分析%The analysis of clinical results of different supine combination of mechanical ventilation ;supporting the management for preventing postoperative complications of esophageal ;atresia

    Institute of Scientific and Technical Information of China (English)

    夏回容

    2016-01-01

    Objective:To study the prophylactic effect of combined supine mechanical ventilation supporting the management of esophageal atresia in postoperative complications. Methods:80 patients were selected from January 2015 to June 2016 in the hospital for treatment of congenital esophageal atresia surgery, and divided into control group and observation group with 40 cases each according to the postoperative management method. The control group was treated by holding pillow supine posture and implement routine care, and the observation group was maintained 30º supine mechanical ventilation support management, in order to compare incidence of postoperative complications between them. Results:The incidence of postoperative complications in the observation group was lower than that in the control group (x2=6.03, P0.05). The children SaO2 levels in the observation group were significantly higher than that in the control group (t=19.24, P<0.05). The patient care satisfaction rate of observation group was 95.0%, significantly higher than 77.5%in the control group (x2=6.38, P<0.05). Conclusion:It works well for patients with esophageal atresia surgery after using 30º supine intervention and mechanical ventilation support management. It can reduce the incidence of postoperative complications, and should be widely applied.%目的:探讨不同卧位结合呼吸机辅助呼吸支持管理在先天性食道闭锁术后并发症中的预防效果。方法:选取80例行先天性食道闭锁术的患儿,根据术后管理方法不同将其分为对照组与观察组,每组40例。对照组患儿术后保持去枕平卧位姿势并实施常规护理;观察组患儿术后保持30 o卧位干预及呼吸机辅助呼吸支持管理。比较两组呼吸机辅助呼吸时间、心率(HR)、平均动脉压(MAP)及动脉血氧饱和度(SaO2)指标以及术后并发症的发生率。结果:观察组术后并发症发生率低于对照组(x2=6.03,P<0.05);两组患儿呼吸机

  16. An analysis of risk factors in anastomotic stricture after thoracospic repair of congenital esophageal atresia%胸腔镜技术治疗先天性食管闭锁术后食管狭窄的相关因素分析

    Institute of Scientific and Technical Information of China (English)

    陶俊峰; 黄金狮; 陶强; 陈快; 樊玮; 刘智文; 徐美汉

    2014-01-01

    目的:探讨胸腔镜技术治疗先天性食管闭锁术后食管狭窄的可能影响因素,以规避部分有害因素,降低食管狭窄的发生率。方法回顾性分析2008年10月至2013年4月本院经胸腔镜治疗的46例先天性食管闭锁患儿临床资料,其中18例术后诊断为食管狭窄;拟定可能影响因素包括:手术时体重、手术日龄、缝合方式、胸腔镜手术学习曲线、食管盲端距离、呼吸机使用时间、胸腔引流管的使用、术后GER、吻合口漏及术后进食时间;按各因素条件使用Excel表将46例患儿相关资料建立数据库,先行单因素检验,再将可能的危险因素纳入多因素非条件Logistic模型,筛选出影响术后食管狭窄的危险因素。结果手术日龄、手术体重及胸腔镜手术学习曲线在单因素分析中存在差异,而食管盲端距离、吻合口漏及术后GER在单、多因素分析中均有不同,差异有统计学意义(P<0.05)。结论经胸腔镜治疗先天性食管闭锁术后食管狭窄主要与食管盲端距离、吻合口漏及GE R有关,预防措施主要是降低食管吻合口的紧张度,减少食管吻合口漏的发生和术后积极治疗GE R。%Objetive To explore the possible influential factors of anastomotic stricture in order to avoid the harmful factors,and to reduce the rate of anastomotic stricture after thoracospic repair of congenital esophage-al atresia. Methods Retrospective analysis was given to 46 cases of congenital esophageal atresia underwent thoracoscopic procedures in our hospital from October,2008 to April,2013.Among the 46 cases,18 were diag-nosed as esophageal stricture,which may be owing to the weight during the operation,the age,methods of anasto-mosis,thoracoscopic operation learning curve,gap length,ventilation time,drainage tube,GER,anastomotic leak-age,eating time,etc.A clinical database of 46 cases was set up with Excellaccording to all of those factors

  17. Anesthetic management for surgery of esophagus atresia in a newborn with Goldenhar's syndrome

    Directory of Open Access Journals (Sweden)

    Rosana Guerrero-Domínguez

    2015-08-01

    Full Text Available BACKGROUND AND OBJECTIVES: Goldenhar's syndrome is a polymalformative condition consisting of a craniofacial dysostosis that determines difficult airway in up to 40% of cases. We described a case of a newborn with Goldenhar's syndrome with esophageal atresia and tracheoesophageal fistula who underwent repair surgery.CASE REPORT: We report the case of a 24-h-old newborn with Goldenhar's syndrome. He had esophageal atresia with distal tracheoesophageal fistula. It was decided that an emergency surgery would be performed for repairing it. It was carried out under sedation, intubation with fibrobronchoscope distal to the fistula, to limit the air flow into the esophagus, and possible abdominal distension. Following complete repair of the esophageal atresia and fistula ligation, the patient was transferred to the intensive care unit and intubated under sedation and analgesia.CONCLUSIONS: The finding of a patient with Goldenhar's syndrome and esophageal atresia assumes an exceptional situation and a challenge for anesthesiologists, since the anesthetic management depends on the patient comorbidity, the type of tracheoesophageal fistula, the usual hospital practice and the skills of the anesthesiologist in charge, with the main peculiarity being maintenance of adequate pulmonary ventilation in the presence of a communication between the airway and the esophagus. Intubation with fibrobronchoscope distal to the fistula deals with the management of a probably difficult airway and limits the passage of air to the esophagus through the fistula.

  18. Computed tomography and upper gastrointestinal series findings of esophageal bronchi in infants

    Energy Technology Data Exchange (ETDEWEB)

    Colleran, Gabrielle C. [Boston Children' s Hospital and Harvard Medical School, Department of Radiology, Boston, MA (United States); Our Lady' s Children' s Hospital Crumlin, Department of Radiology, Dublin (Ireland); Ryan, Ciara E. [Our Lady' s Children' s Hospital Crumlin, Department of Pathology, Dublin (Ireland); Lee, Edward Y. [Boston Children' s Hospital and Harvard Medical School, Department of Radiology, Boston, MA (United States); Sweeney, Brian [Our Lady' s Children' s Hospital Crumlin, Department of Surgery, Dublin (Ireland); Rea, David; Brenner, Clare [Our Lady' s Children' s Hospital Crumlin, Department of Radiology, Dublin (Ireland)

    2017-02-15

    Esophageal bronchus is a rare form of communicating bronchopulmonary foregut malformation and a rare but important cause of an opaque hemithorax on chest radiography. A higher incidence of esophageal bronchus is associated with esophageal atresia, tracheo-esophageal fistula (TEF) and VACTERL (vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities) association. In the presence of these conditions, the pediatric radiologist may be the first to consider the diagnosis of esophageal bronchus or esophageal lung. To describe the imaging features in five children with esophageal bronchus. We reviewed hospital records and teaching files at two large pediatric tertiary referral centers over the 24-year period from January 1992 to January 2016. We reviewed all imaging studies and tabulated findings on radiography, fluoroscopic upper gastrointestinal (GI) series and CT. We then described the imaging features of esophageal bronchi with emphasis on CT and upper GI findings in four infants and one toddler. Three cases were identified from one institution (cases 2, 3, 4) and two from another (cases 1, 5). All five cases occurred in association with other midline malformations: four of the five had VACTERL association and three of the five had esophageal atresia and TEF. Lung opacification, ipsilateral mediastinal shift, and an abnormal carina and anomalous vascular anatomy suggest an esophageal bronchus or an esophageal lung on CT. While esophageal bronchus is a rare cause of an opaque hemithorax, CT and upper GI imaging play key roles in its diagnosis. Associations with esophageal atresia with tracheo-esophageal fistula and VACTERL association are particularly pertinent. Early diagnosis of esophageal bronchus might prevent complications such as aspiration and infection, which can allow for parenchymal sparing surgery as opposed to pneumonectomy. (orig.)

  19. PYLORIC ATRESIA IN ASSOCIATION WITH MULTIPLE COLONIC ATRESIAS IN A NEONATE: AN UNREPORTED ASSOCIATION

    Directory of Open Access Journals (Sweden)

    Vijay C Pujar

    2012-01-01

    Full Text Available Pyloric atresia is rare cause of gastrointestinal obstruction in neonates and usually occurs as an isolated anomaly. They have been associated with multiple small bowel and colonic atresias but not reported in association with isolated multiple colonic atresias. A case of pyloric atresia oc-curring in association with multiple colonic atresias is being reported here.

  20. Association of Biliary Atresia with Jejuno-Ileal Atresia

    Directory of Open Access Journals (Sweden)

    Alireza Alam Sahebpour

    2009-09-01

    Full Text Available Background:Jejuno-ileal atresia (JIA is a congenital anomaly characterized clinically by bilious vomiting and abdominal distension. The incidence of JIA is between 1: 330 to 1: 3000 live births in different parts of the world. It has been associated with various congenital anomalies but the association of JIA with biliary atresia is extremely rare (0-3.2 %. Case Presentation:We herein present a case of jejunal atresia with meconium peritonitis associated with biliary atresia. The patient was a boy who was born at 39 weeks of gestation with polyhydramnious detected on Prenatal Ultra Sonography done at 8th month of gestation. Conclusion:It is important to explore gallbladder in cases of JIA especially when associated with meconium peritonitis.

  1. Sonic hedgehog and Glis famliy expression in the human neonatal of congenital esophageal atresia and tracheoesophageal fistula%Sonic hedgehog基因及Gli家族在人类食管闭锁并气管食管瘘中的表达

    Institute of Scientific and Technical Information of China (English)

    陈璐璐; 陈永卫

    2012-01-01

    目的 研究Sonic hedgehog基因及Gli家族在人类先天性食管闭锁并气管食管瘘(esophageal atresia and tracheoesophageal fistula,EA-TEF)的表达特点,探讨EA-TEF病因及发病机制的可能影响因素.方法 食管吻合术中留取22例EA-TEF患儿近端食管盲端及远端气管食管瘘管组织,7例行HE染色,10例行real-time RT-PCR处理,5例行免疫荧光染色处理.观察食管盲端及气管食管瘘管形态上的变化及各指标的差异.结果 ①形态学:瘘管组织内皮下可见粘液腺体,肌层稀疏且肌肉组织结构紊乱;②Shh表达:食管盲端组织中可见表达,瘘管组织中未有表达;③Glis表达:Gli-1、Gli-3mRNA表达无差异,Gli-2mRNA表达差异有统计学意义(P<0.05),瘘管中表达低于食管盲袋.结论 气管食管瘘组织具有气管源性特征.EA-TEF的发生可能与Shh信号通路表达下调有关.Gli-2的功能缺失在EA-TEF的发生中可能发挥重要作用.%Objective To describe the differential expression of Shh and Gli family members between normal proximal esophageal pouch and distal tracheoesophageal fistula (EA-TEF).Methods Twenty twohuman proximal esophageal pouch and distal fistula samples were obtained at the time of standard repair of EA-TEF.Seven samples were processed for HE,ten for real-time reverse-transcriptase polymerase chain reaction (RT-PCR),and five for immunohistochemistry.Results Shh was confirmed to be present by immunohistochemistry in the proximal esophageal pouch,but was specifically absent in the distal fistula tract.Gli1and-3 mRNA expression showed no difference in the proximal pouch and distal esophagus using real-time RT-PCR.Gli-2 mRNA levels were much lower in the fistula tract than in the adjacent esophagus.Conclusions Abnomal expression of Shh may contribute to the malformation of EA-TEF.Gli2down-regulation may also play an important role in the mechanisms of EA-TEF malformation.These results support the conclusion that the fistula tract

  2. Embryology of oesophageal atresia

    Science.gov (United States)

    Ioannides, Adonis S.; Copp, Andrew J.

    2013-01-01

    Oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) are important human birth defects of unknown aetiology. The embryogenesis of OA/TOF remains poorly understood mirroring the lack of clarity of the mechanisms of normal tracheo-oesophageal development. The development of rat and mouse models of OA/TOF has allowed the parallel study of both normal and abnormal embryogenesis. Although controversies persist, the fundamental morphogenetic process appears to be a rearrangement of the proximal foregut into separate respiratory (ventral) and gastrointestinal (dorsal) tubes. This process depends on the precise temporal and spatial pattern of expression of a number of foregut patterning genes. Disturbance of this pattern disrupts foregut separation and underlies the development of tracheo-oesophageal malformations. PMID:19103415

  3. Biliary atresia: pathogenesis and treatment.

    Science.gov (United States)

    Bates, M D; Bucuvalas, J C; Alonso, M H; Ryckman, F C

    1998-01-01

    Biliary atresia is a disorder of infants in which there is obliteration or discontinuity of the extrahepatic biliary system, resulting in obstruction of bile flow. Untreated, the resulting cholestasis leads to progressive conjugated hyperbilirubinemia, cirrhosis, and hepatic failure. Biliary atresia has an incidence of approximately one in 10,000 live births worldwide. Evidence to date supports a number of pathogenic mechanisms for the development of biliary atresia. An infectious cause, such as by a virus, would seem most pausible in many cases. The clinical observation that biliary atresia is rarely encountered in premature infants would support an agent acting late in gestation. However, no infectious or toxic agent has been conclusively implicated in biliary atresia. Genetic mechanisms likely play important roles, even regarding susceptibility to other specific causes, but no gene whose altered function would result in obstruction or atresia of the biliary tree has been identified. The variety of clinical presentations support the notion that the proposed mechanisms are not mutually exclusive but may play roles individually or in combination in certain patients. Biliary atresia, when untreated, is fatal within 2 years, with a median survival of 8 months. The natural history of biliary atresia has been favorably altered by the Kasai portoenterostomy. Approximately 25 to 35% of patients who undergo a Kasai portoenterostomy will survive more than 10 years without liver transplantation. One third of the patients drain bile but develop complications of cirrhosis and require liver transplantation before age 10. For the remaining one third of patients, bile flow is inadequate following portoenterostomy and the children develop progressive fibrosis and cirrhosis. The portoenterostomy should be done before there is irreversible sclerosis of the intrahepatic bile ducts. Consequently, a prompt evaluation is indicated for any infant older than 14 days with jaundice to

  4. Biliary atresia associated with meconium peritonitis.

    Science.gov (United States)

    Chung, Mei-Yung; Ko, Tan-Yung; Huang, Chung-Bin; Lee, Chiang-Hsuan; Hsieh, Chih-Sung

    2006-01-01

    Biliary atresia, malrotation, meconium peritonitis and transient hypothyroidism are occasionally seen in neonatal infants. Biliary atresia associated with malrotation has been reported in some patients with polysplenia syndrome, but biliary atresia associated with meconium peritonitis has only been described by a few investigators. Here we present a case of meconium peritonitis due to malrotation with volvulus, followed by biliary atresia and transient hypothyroidism during early infancy.

  5. Biliary atresia Atresia de las vías biliares.

    Directory of Open Access Journals (Sweden)

    Pastor Thomas Olivares

    2005-12-01

    Full Text Available Biliary atresia is an obstructive and progressive process of unknown etiology that affects intra and/or extrahepatics biliary tracts and cause neonatal jaundice by serious obstruction of the biliary flow. It is presented with a frequency of 1 by each 10 000 – 15000 born alive. Is not hereditary and the most probable etiology is the progressive inflammatory illness of the biliary tracts. We presented the Good Clinical Practices Guideline for Biliary atresia, approved by consensus in the 4th National Good Clinical Practices Workshop in Pediatric Surgery (Las Tunas, Cuba, March, 2005.
    La atresia de vías biliares es un proceso obstructivo y progresivo de etiología desconocida que afecta las vías biliares intra y/o extrahepáticas y causa ictericia neonatal por obstrucción grave del flujo biliar. Se presenta con una frecuencia de 1 por cada 10 000 – 15 000 nacidos vivos. No es hereditaria y la etiología más probable es la enfermedad inflamatoria progresiva de las vías biliares. Se presenta la Guía de Buenas Prácticas Clínicas para Atresia intestinal yeyunoileal, aprobada por consenso en el 4º Taller Nacional de Buenas Prácticas Clínicas en Cirugía Pediátrica (Las Tunas, marzo 2005.

  6. Detergent-free Decellularized Nerve Grafts for Long-gap Peripheral Nerve Reconstruction

    Directory of Open Access Journals (Sweden)

    Srikanth Vasudevan, PhD

    2014-08-01

    Conclusions: This study describes a detergent-free nerve decellularization technique for reconstruction of long-gap nerve injuries. We compared DFD grafts with an established detergent processing technique and found that DFD nerve grafts are successful in promoting regeneration across long-gap peripheral nerve defects as an alternative to existing strategies.

  7. Esophageal Cancer

    Science.gov (United States)

    ... from your throat to your stomach. Early esophageal cancer usually does not cause symptoms. Later, you may ... You're at greater risk for getting esophageal cancer if you smoke, drink heavily, or have acid ...

  8. The Combination of Gastroschisis, Jejunal Atresia, and Colonic Atresia in a Newborn

    Directory of Open Access Journals (Sweden)

    Zachary Bauman

    2015-01-01

    Full Text Available We encountered a rare case of gastroschisis associated with jejunal atresia and colonic atresia. In our case, the jejunal atresia was not discovered for 27 days after the initial abdominal wall closure. The colonic atresia was not discovered for 48 days after initial repair of the gastroschisis secondary to the rarity of the disorder. Both types of atresia were repaired with primary hand-sewn anastomoses. Other than the prolonged parenteral nutrition and hyperbilirubinemia, our patient did very well throughout his hospital course. Based on our case presentation, small bowel atresia and colonic atresia must be considered in patients who undergo abdominal wall closure for gastroschisis with prolonged symptoms suggestive of bowel obstruction. Our case report also demonstrates primary enteric anastomosis as a safe, well-tolerated surgical option for patients with types of intestinal atresia.

  9. A unique case of segmental vasal atresia

    Science.gov (United States)

    Alalayet, Yasen Fayez; Alkasim, F; Shiba, N; Aldhuayan, I; Alhamaidi, S; Alghamdi, G; Aljobair, F; Shoura, J; Alkhlaif, R

    2014-01-01

    We report a case of a 2-year-old boy who presented with an empty left scrotum. Clinical examination revealed a left palpable undescended testis. During orchidopexy, segmental atresia of the vas deferens was found, and microsurgical repair was carried out. Segmental vasal atresia is an extremely rare condition and is infrequently diagnosed, especially in the pediatric age group. This is the first reported case of segmental vasal atresia without an association with cystic fibrosis. PMID:25419520

  10. A study of associated congenital anomalies with biliary atresia

    Directory of Open Access Journals (Sweden)

    Lucky Gupta

    2016-01-01

    Full Text Available Background/Purpose: This study aims to analyze the incidence and type of various associated anomalies among infants with extrahepatic biliary atresia (EHBA, compare their frequency with those quoted in the existing literature and assess their role in the overall management. Materials and Methods: A retrospective study was performed on 137 infants who underwent the Kasai procedure for EHBA during the past 12 years. The medical records were reviewed for the incidence and type of associated anomalies in addition to the details of the management of the EHBA. Results: Of the137 infants, 40 (29.2% were diagnosed as having 58 anomalies. The majority of patients had presented in the 3 rd month of life; mean age was 81 ± 33 days (range = 20-150 days. There were 32 males and 8 females; boys with EHBA had a higher incidence of associated anomalies. Of these 40 patients, 22 (37.9% had vascular anomalies, 13 patients (22.4% had hernias (umbilical-10, inguinal-3, 7 patients (12.1% had intestinal malrotation, 4 patients (6.8% had choledochal cyst, 1 patient (1.7% had Meckel′s diverticulum, 3 patients (5% had undergone prior treatment for jejunoileal atresias (jejunal-2, ileal-1, 2 patients (3.4% had undergone prior treatment for esophageal atresia and tracheoesophageal fistula, 2 patients (3.4% had spleniculi, and 2 patients (3.4% were diagnosed as having situs inversus. Conclusions: The most common associated anomalies in our study were related to the vascular variation at the porta hepatis and the digestive system. The existence of anomalies in distantly developing anatomic regions in patients with EHBA supports the possibility of a "generalized" insult during embryogenesis rather than a "localized" defect. In addition, male infants were observed to have significantly more associated anomalies as compared with the female infants in contrast to earlier reports.

  11. Pyloric atresia associated with epidermolysis bullosa

    Energy Technology Data Exchange (ETDEWEB)

    Garcia Hernandez, J.B.; Orense, M.; Celorio, C.; Canga, C.

    1987-07-01

    Since the first reported case of pyloric atresia and epidermolysis bullosa in 1977 by Korber and Glasson, this association has been firmly established. We present a new case giving particular emphasis to the theory that pyloric atresia is secondary to the primary disease epidermolysis bullosa.

  12. Esophageal cancer.

    Science.gov (United States)

    Vakil, Nimish; Affi, Aboud

    2002-07-01

    Despite advances in our knowledge of esophageal cancer, 50% of patients present with incurable disease, and the overall survival after diagnosis is poor. The incidence of esophageal adenocarcinoma of the distal esophagus is rising at a rapid rate in developed countries. Recent advances in the epidemiology of esophageal cancer offer insights into preventive strategies in patients who are at risk. New developments in diagnosis may help detect the disease at an early stage. New diagnostic modalities permit more accurate staging procedures and allow appropriate selection of therapy. New studies provide more information on multimodality therapy for esophageal cancer, and new endoscopic techniques allow resection of small lesions without surgery. New stent designs provide better palliation by providing tumor ingrowth. These developments in the treatment of esophageal cancer are the focus of this review.

  13. Evolución a largo plazo de 55 pacientes operados de atresia esofágica

    Directory of Open Access Journals (Sweden)

    Ruperto Llanes Céspedes

    2003-06-01

    Full Text Available Los pacientes operados de atresia esofágica constituyen un reto para los médicos involucrados en su seguimiento. Se realizó un estudio retrospectivo de las historias clínicas de 55 pacientes vivos después de operarse de atresia esofágica, en un período de 15 años, donde se evaluaron las complicaciones encontradas a largo plazo, con el objetivo de identificarlas y conocer su tratamiento. La presencia de reflujo gastroesofágico en 30 niños (54,54 %, la dismotilidad esofágica en 28 (50,90 % y las manifestaciones respiratorias recurrentes en 26 pacientes (47,27 % fueron las más frecuentes, seguidas de las estenosis de la sutura en 11 (20 %, retardo del crecimiento y desarrollo en 10 (18,18 % y la recurrencia de la fístula en 2 niños (3,63 %. Se concluye que en el seguimiento a largo plazo de los pacientes operados de atresia esofágica se deben realizar el diagnóstico precoz y el tratamiento de múltiples complicaciones que definen su calidad de vida.The patients operated for esophageal atresia become a challenge for physicians involved in their follow-up. A retrospective study of the medical histories of 55 live patients after they had been operated for esophageal atresia in a period of 15 years was undertaken where long-term complications were evaluated to identify them and find out their treatment. Gastroesophageal reflux in 30 children (54,54 %, esophageal dismotility in 28 children (50,90 % and recurrent respiratory manifestations in 26 patients (47,27 % were the most frequent complications followed by suture stenosis in 11 (20 %, retarded growth and development in 10 (18,18 % and recurrent tracheoesophageal fistula in 2 children. It is concluded that early diagnosis and treatment of multiple complications, which define the quality of life of patients, should be made in the long-term follow-up of patients operated for esophageal atresia.

  14. Assessing portal hypertension in post-operative biliary atresia patients using 2D magnetic resonance angiography

    Energy Technology Data Exchange (ETDEWEB)

    Hiki, Saori; Horikoshi, Kentarou; Kobayashi, Hiroyuki; Yamataka, Atsuyuki; Miyano, Takeshi; Kuwatsuru, Ryouhei; Katayama, Hitoshi [Juntendo Univ., Tokyo (Japan). School of Medicine

    2000-01-01

    2D magnetic resonance angiography (MRA) was performed in 38 post-operative biliary atresia (BA) patients. Collateral circulation other than esophageal varices that could not be observed with endoscopy was detected. By using contrast, the portal vasculature could be clearly delineated. MRA can be performed without using general anesthesia obviating the nead for hospitalization. At present, MRA alone is not sufficient for the complete assessment of varices in post-operative BA patients, and mucosal changes cannot be detected without endoscopy. However, combined with endoscopy, it provides valuable additional follow-up information without the need for general anesthesia or hospitalization. (author)

  15. Congenital Pyloric Atresia with Distal Duodenal Atresia- Role of CT Scan

    Directory of Open Access Journals (Sweden)

    Yogender Singh Kadian

    2014-07-01

    Full Text Available The mainstay of diagnosis of congenital pyloric atresia is by plain X-ray of the abdomen showing a large gas bubble with no gas distally. But very rarely it can be associated with distal duodenal atresia when the baby may present as lump abdomen. In such a situation apart from the X-ray, another radiological investigation is needed to delineate the exact nature of the lump. Since the role of ultrasonography is limited in intestinal pathologies and contrast studies are not informative in atresias, the CT scan is the ideal choice. We had managed a case of pyloric atresia with similar presentation with preoperative CT scan.

  16. Esophageal Cancer Prevention

    Science.gov (United States)

    ... the lower part of the esophagus, near the stomach. See the following PDQ summaries for more information about esophageal cancer: Esophageal Cancer Screening Esophageal Cancer Treatment Esophageal cancer is found more ...

  17. Esophageal Cancer Screening

    Science.gov (United States)

    ... Esophageal Cancer Prevention Esophageal Cancer Screening Research Esophageal Cancer Screening (PDQ®)–Patient Version What is screening? Go to ... the esophagus and the stomach). Being overweight . Esophageal Cancer Screening Key Points Tests are used to screen for ...

  18. Outcomes of surgical management of intestinal atresias

    African Journals Online (AJOL)

    2013-11-22

    Nov 22, 2013 ... shock (1), anesthesia‑related (1), undetermined (1). Two of the mortalities (40%) had ... Key words: Intestinal atresia, outcomes, surgical management ... with information on prenatal ultrasonography (18/23), only 50% (9/18) ...

  19. [Unilateral choanal atresia: a missed diagnosis].

    Science.gov (United States)

    Joshua, Ben-Zion; Gluck, Ofer; Puterman, Mark

    2012-11-01

    Bilateral choanal atresia is a congenital anomaly usually diagnosed at birth. In contrast, unilateral atresia causes variable degrees of nasal stuffiness and discharge that might mask the diagnosis and delay the proper treatment. We present five cases of unilateral atresia in which the correct diagnosis was delayed and erroneous treatments were instituted--two adults who had undergone unnecessary septal and turbinate surgery, two older children who were treated medically and one patient who was treated for epiphora. Insufficient awareness of this entity occurring in adults and older children and other possible causes of diagnostic error are discussed. A unilateral mucoid or watery rhinorrhea and obstruction, that lasts from early childhood without evidence of sinusitis and not responding to any medical treatment, should alert the physician to consider unilateral atresia and to perform endoscopic and computed tomography examinations at an early age. Careful interpretation of the computed tomography scan including the axial planes is required.

  20. Esophageal replacement.

    Science.gov (United States)

    Kunisaki, Shaun M; Coran, Arnold G

    2017-04-01

    This article focuses on esophageal replacement as a surgical option for pediatric patients with end-stage esophageal disease. While it is obvious that the patient׳s own esophagus is the best esophagus, persisting with attempts to retain a native esophagus with no function and at all costs are futile and usually detrimental to the overall well-being of the child. In such cases, the esophagus should be abandoned, and the appropriate esophageal replacement is chosen for definitive reconstruction. We review the various types of conduits used for esophageal replacement and discuss the unique advantages and disadvantages that are relevant for clinical decision-making. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Epidemiology of small intestinal atresia in Europe

    DEFF Research Database (Denmark)

    Best, Kate E; Tennant, Peter W G; Addor, Marie-Claude;

    2012-01-01

    The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe.......The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe....

  2. [Tricuspid atresia: A therapeutic problem].

    Science.gov (United States)

    Mata, L A; Espino Vela, J; Castro, A; Cárcamo Tercero, J; Martínez Ríos, M A; Pliego, J; Attié, F

    1975-01-01

    1. The authors present 80 cases of tricuspid atresia of the Children's Cardiology Service of the Instituto Nacional de Cardiología de México. They propose their own classification based on the position of the visceral suits and of the great arteries and the possible existance of a persistent truncus arteriosus. 2. The requirements that should be fulfiled to establish an anatomic and hemodynamic diagnosis are determined; and the need to know in detail the anatomy of the right ventricle; of its exit chamber, of the pulmonary valve and of the pulmonary truncus is emphasized; as well as the measures in the auricles and the left ventricle. 3. They insist upon the need, when the pulmonar arterial pressure cannot be measured directly, to introduce a catheter in a pulmonar vein, because the pressures obtained are quite similar to the pressure in the pulmonar arterial territory. 4. The fundamental data of the anatomic and hemodynamic study are discussed and illustrated. They confirm the close relation that exists between the diameter of the exit chamber of the right ventricle and of the valvular ring, the pulmonary truncus and its branches. 5. In all cases of tricuspid atresia with AQRS deviated to the right and downwards in the frontal plane; transposition of the great arteries should be thought of when it deviates to the left and upwards, in patients with crossed great arteries. 6. In the cases operated with sistemic-pulmonary fistula, there was an intrahospital mortality range of 25%, similar to that obtained by most authors. 7. The paliative surgical procedures are analyzed, and those that at present could be considered as "corrective" such as Fontan and Kreutzer's operations. The need, to gather several diagnostic parameters. To establish the surgical indication in the sistemicpulmonary and venus shunts, is emphasized.

  3. Esophageal anastomosis.

    Science.gov (United States)

    Yuan, Y; Wang, K-N; Chen, L-Q

    2015-01-01

    This review gives an overview of the esophageal anastomosis. The history, various techniques and substitution organs, their advantages and disadvantages, healing mechanism, complications, and actual trend of this essential part of esophageal surgery are described. The history of the esophageal anastomosis extending from the first anastomosis in 1901 to today has undergone more than one century. In the early days, the success rate of the anastomosis was extremely low. As the technology progressed, the anastomosis got significant achievement. Various anastomotic techniques are currently being used. However, controversies exist on the choice of anastomotic method concerning the success rate, postoperative complication and quality of life. How to choose the method, no one can give the best answer. We searched the manuscripts about the esophageal anastomoses in recent years and studied the controversy questions about the anastomosis. Performing an esophageal anastomosis is a technical matter, and suture healing is independent of the patient's biologic situation. Every anastomosis technique has its own merit, but the outcomes were different if it was performed by different surgeons, and we also found that the complication rate of the anastomosis was mainly associated with the surgeons. So the surgeons should learn from their previous experience and others to avoid technical errors.

  4. Eosinophilic esophagitis

    Institute of Scientific and Technical Information of China (English)

    Anand R Gupte; Peter V Draganov

    2009-01-01

    Eosinophilic esophagitis is increasingly recognized in adults. The diagnosis is based on the presence of both typical symptoms and pathologic findings on esophageal biopsy. Patients usually present with dysphagia, food impaction and/or reflux-like symptoms, and biopsy of the esophagus shows more than 15 eosinophils per high-power field. In addition,it is essential to exclude the presence of known causes of tissue eosinophilia such as gastroesophageal reflux disease, infections, malignancy, collagen vascular diseases, hypersensitivity, and inflammatory bowel disease. There are no standardized protocols for the therapy of eosinophilic esophagitis. A variety of therapeutic approaches including acid suppression, dietary modi f icat ions, topical cor t icosteroids and endoscopic dilation can be used alone or in combination.

  5. Oesophageal atresia: a 10-year experience of a Paediatric Intensive Care Unit

    Directory of Open Access Journals (Sweden)

    Liliana Pinho

    2016-03-01

    Full Text Available Background/Purpose: Oesophageal atresia (OA is a congenital malformation with a variable prognosis. The aims were to establish OA’s incidence in the central region, to characterize infants with OA admitted and to compare its clinical outcome after surgical repair, according to OA classifi cation. Methods: A retrospective review of infants with OA admitted to a PICU, after surgical repair, between 2002 and 2011. Patient characteristics, OA’s classifi cation, surgery, morbidity and mortality were analyzed. Two groups were compared according to OA classifi cation. Results: Thirty-four infants were admitted, out of which 65% were male, with a median gestational age of 36 weeks and birth weight of 2310g. Nineteen of them presented other malformations, mainly cardiac. Nine cases were classifi ed as long-gap OA. Fistula ligation and primary oesophageal anastomosis was the most common surgical option (n=27. Early complications occurred in 13 infants (38%, mostly anastomotic leak, and were similar according to gap length (p=0.704. PICU stay and mechanical ventilation were longer in long-gap OA patients (p=0.009 and p<0.001 respectively and in infants with other malformations (p=0.027 and p=0.003 respectively. There was no mortality. Conclusions: The frequency of OA associated malformations implies a systematic screening of these patients. Gap length and presence of associated malformations were the major determinants of length of intensive care stay and ventilation days in OA patients.

  6. Electrophysiologic studies in tricuspid atresia.

    Science.gov (United States)

    Serratto, M; Pahlajani, D B

    1978-12-01

    Five patients with tricuspid atresia underwent His bundle studies with the recording catheter placed close to the mitral valve ring. Right atrial pacing and measurement of the refractory periods were performed in three. The prolonged intraatrial conduction time found in all patients is thought to be caused by a hypertrophied and dilated right atrium with increased internodal distance due to stretching of internodal pathways. The A-H interval was normal in all; the H-V interval was short in three and normal in two. The pattern of left axis deviation in the group with a short H-V interval is thought to be due to early origin of the posterior branches of the left bundle branch from the bundle of His and early activation of the posteroinferior parts of the left ventricle. The pattern of left axis deviation in the group with a normal H-V interval may be related to the previously reported anomalous course of the left bundle. Atrial pacing produced a normal response. The refractory periods were within normal range, suggesting functional integrity of conduction through the atrioventricular node and bundle branches.

  7. Microtia and congenital aural atresia.

    Science.gov (United States)

    Genc, Selahattin; Kahraman, Erkan; Ozel, Halil Erdem; Arslan, Ilker Burak; Demir, Ahmet; Selcuk, Adin

    2012-11-01

    The purpose of this study was to show the clinical characteristics of microtia and congenital aural atresia cases in Turkey and to make the classification. For this purpose, records of 28 patients with microtia who were admitted to the ENT Clinic of Eskisehir Military Hospital, Turkey, between 1995 and 2011 and 3 patients admitted to the ENT outpatient clinic of Kocaeli Derince Education and Research Hospital, Turkey, were analyzed retrospectively. Of the total 31 patients with microtia (35 microtic ears), involvement of the right ear of 20 patients (64.5%), the left ear of 7 patients (22.5%), and bilateral involvement in 4 patients (12.9%) were observed. There was a unilateral involvement in 27 patients (87.1%). According to the Marx grading, 2 patients (5.7%) had grade 1 malformation, 3 (8.6%) had grade 2 malformation, 29 (82.9%) had grade 3 malformation, and 1 (2.9%) had grade 4 malformation (anotia). Although the characteristics of microtia vary in different population, the results in Turkey are consistent with those in the literature.

  8. Primary Transanal Management of Rectal Atresia in a Neonate.

    Science.gov (United States)

    M, Braiek; A, Ksia; I, Krichen; S, Belhassen; K, Maazoun; S, Ben Youssef; N, Kechiche; M, Mekki; A, Nouri

    2016-01-01

    Rectal atresia (RA) with a normal anus is a rare anomaly. We describe a case of rectal atresia in a newborn male presenting with an abdominal distension and failure of passing meconium. The rectal atresia was primarily operated by transanal route.

  9. Wind sock deformity in rectal atresia

    Directory of Open Access Journals (Sweden)

    Hosseini Seyed

    2009-01-01

    Full Text Available Rectal atresia is a rare anorectal deformity. It usually presents with neonatal obstruction and it is often a complete membrane or severe stenosis. Windsock deformity has not been reported in rectal atresia especially, having been missed for 2 years. A 2-year-old girl reported only a severe constipation despite having a 1.5-cm anal canal in rectal examination with scanty discharge. She underwent loop colostomy and loopogram, which showed a wind sock deformity of rectum with mega colon. The patient underwent abdominoperineal pull-through with good result and follow-up. This is the first case of the wind sock deformity in rectal atresia being reported after 2 years of age.

  10. Esophageal cancer

    DEFF Research Database (Denmark)

    Mortensen, M. B.

    2007-01-01

    The distribution of adenocarcinomas and squamous cell carcinomas in esophageal cancer (EC) has changed, and focus directed towards tumors of the distal esophagus and the esophagogastric junction. The genetic events leading to EC are not fully clarified, but important risk factors have been...

  11. Atresia of the gastrointestinal tract: imaging evaluation; Atresia do trato gastrintestinal: avaliacao por metodos de imagem

    Energy Technology Data Exchange (ETDEWEB)

    Figueiredo, Sizenildo da Silva; Nobrega, Bruno Barcelos da; Ribeiro, Luiza Helena Vilela; Costa, Marlos Augusto Bittencourt; Monteiro, Soraya Silveira; Lederman, Henrique Manoel [Universidade Federal de Sao Paulo (UNIFESP/EPM), SP (Brazil). Dept. de Diagnostico por Imagem]. E-mail: brunoradiol@hotmail.com; Oliveira, Galba Leite [Irmandade Santa Casa de Misericordia de Sao Paulo, SP (Brazil). Dept. de Diagnostico por Imagem; Esteves, Edward [Goias Univ., Goiania, GO (Brazil). Faculdade de Medicina. Dept. de Cirurgia e Urologia Pediatrica

    2005-04-01

    A wide spectrum of congenital anomalies may affect the gastrointestinal tract at any level from the esophagus to the anus. Atresia is an important cause of gastrointestinal obstruction with high morbidity rate in neonates. Different pathogenetic mechanisms could cause this malformation and the two classical explanations are: a defect of recanalization of the intestinal tube or an interruption of blood supply during intrauterine life. The authors present a literature review with an iconographic essay of imaging findings in children with gastrointestinal atresia. (author)

  12. Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome

    Directory of Open Access Journals (Sweden)

    Rohit S Loomba

    2015-01-01

    Full Text Available Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right ventricle in the setting of Rubenstein-Taybi syndrome.

  13. ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA

    Directory of Open Access Journals (Sweden)

    O. E. Iryshkin

    2012-01-01

    Full Text Available Biliary atresia is an inflammatory fibrosing process, which invades both extrahepatic and intrahepatic bile ducts. There are two forms of biliary atresia: embryonic form, associated with another visceral malformations, and perinatal form, which is an isolated disease. Biliary atresia is a heterogeinc disease which may appear as a result of different etiological factors, the most important of them are genetics, viral infection, vascular factors, toxins and disregulation of immune system. The characteristic morphological features of biliaryatresia are: intracellular and ductular cholestasis, ductular proliferation, giant cell transformation, portal and perilobular oedema and/or fibrosis. As the patalogical process progresses, ductopenia appears, and fibrosis turns to cirrhosis. Embryonic form mophologically manifests with ductal plate malformation is, what is an important diagnostic criterion. Pro- gnosis of biliary atresia depends on form of disease, age of patient and type of surgical operation. Today surgical treatment includes two steps: palliative portoenterostomia, performed in neonatal period and further liver trans- plantation. However liver transplantation is more preferable as first and single-step treatment. 

  14. Dysphagia in children with repaired oesophageal atresia

    NARCIS (Netherlands)

    Coppens, C.H.; Engel-Hoek, L. van den; Scharbatke, H.E.; Groot, S.A. de; Draaisma, J.M.T.

    2016-01-01

    Dysphagia is a common problem in children with repaired oesophageal atresia (OA). Abnormalities in the oropharyngeal and oesophageal phase have hardly been studied. The aims of this study were to assess the prevalence of dysphagia in children with repaired OA and to identify and differentiate oral

  15. Multiple gastrointestinal atresias in two consecutive siblings.

    Science.gov (United States)

    Gahukamble, D B; Gahukamble, L D

    2002-03-01

    Two consecutive female siblings with multiple gastrointestinal atresias are described. The history of consanguinity in the parents and the presence of extensive typical pathological lesions suggest a genetically-induced developmental fault in the alimentary tract during the early embryonic period.

  16. Bridging long gap peripheral nerve injury using skeletal muscle-derived multipotent stem cells

    Institute of Scientific and Technical Information of China (English)

    Tetsuro Tamaki

    2014-01-01

    Long gap peripheral nerve injuries usually reulting in life-changing problems for patients. Skeletal muscle derived-multipotent stem cells (Sk-MSCs) can differentiate into Schwann and perineurial/endoneurial cells, vascular relating pericytes, and endothelial and smooth muscle cells in the damaged peripheral nerve niche. Application of the Sk-MSCs in the bridging conduit for repairing long nerve gap injury resulted favorable axonal regeneration, which showing supe-rior effects than gold standard therapy--healthy nerve autograft. This means that it does not need to sacriifce of healthy nerves or loss of related functions for repairing peripheral nerve injury.

  17. Esophageal Mucormycosis

    Directory of Open Access Journals (Sweden)

    Benjamin Boatright

    2014-01-01

    Full Text Available Mucormycosis is a rare invasive fungal infection with high mortality. It usually affects patients with poorly controlled diabetes, immunosuppression, or hematological malignancies. Gastroenterologists need to be aware of this rare infection because endoscopy can facilitate early diagnosis and prompt appropriate therapy. Here we describe a case of invasive esophageal mucormycosis that developed in a 63-year-old man with diabetes, acute promyelocytic leukemia, and prolonged leukopenia after chemotherapy. Upper endoscopy showed distal circumferential esophageal wall thickening with devitalization. The mucosa did not bleed after endoscopic biopsy. Histopathology confirmed mucormycosis. He was treated with various antifungal agents including echinocandins, fluconazole, and liposomal amphotericin B. Despite aggressive antifungal therapy and supportive care, the patient died 24 days later.

  18. Urogenital abnormalities and atresia of the gastrointestinal tract

    OpenAIRE

    2005-01-01

    Introduction. The goal of the study was to investigate the frequency of urogenital congenital abnormalities among atresias of the digestive system and analyze fetal maldevelopment. The study also deals with gastrointestinal and urogenital embryology. Material and methods. This retrospektive study analyzed the clinical status of 55 new-borns admitted to the Pediatric Surgery Clinic in Novi Sad due to atresia of the gastrointestinal tract during 1995-2003. All atresias were classified at primor...

  19. Chronic Pulmonary Aspergillosis Complicating Bronchial Atresia

    Directory of Open Access Journals (Sweden)

    Mazen O. Al-Qadi

    2014-01-01

    Full Text Available Bronchial atresia is a rare pulmonary developmental anomaly characterized by the presence of a focal obliteration of a segmental or lobar bronchial lumen. The lung distal to the atretic bronchus is typically emphysematous along with the presence of mucus filled ectatic bronchi (mucoceles. BA is usually asymptomatic but pulmonary infections can rarely develop in the emphysematous lung distal to the atretic bronchus. We present a unique case of chronic pulmonary aspergillosis (CPA in a patient with BA with no evidence of immune dysfunction. The patient was treated initially with voriconazole and subsequently underwent surgical excision of the involved area. On follow-up, she has done extremely well with no evidence for recurrence. In summary, we describe the first case of chronic pulmonary aspergillosis in an immunocompetent patient with bronchial atresia.

  20. A Rare Case of Jejunal Atresia Due to Intrauterine Intussusception.

    Science.gov (United States)

    Joshi, Sanjeev B; Kinhal, Vidyadhar; Desai, Mahesh; Tilak; Choudhari, Fazal Ur Rehman

    2015-09-01

    Intestinal atresia is generally caused by intrauterine vascular obstructions involving mesenteric vessels. Intrauterine intussusceptions (IUI) are one of these disruptive events. Intestinal intussusceptions affects children commonly between 3 months and 3 years of age, but it rarely affects in intrauterine life. The relationship between intrauterine intussusception and intestinal atresia has been demonstrated by few cases in literature, suggesting intrauterine intussusception as a rare cause of intestinal atresia. We report a 7-day-old full term neonate presenting with intrauterine intussusceptions (jejuno-jejunal) resulting in jejunal atresia.

  1. Risks of Esophageal Cancer Screening

    Science.gov (United States)

    ... Esophageal Cancer Prevention Esophageal Cancer Screening Research Esophageal Cancer Screening (PDQ®)–Patient Version What is screening? Go to ... the esophagus and the stomach). Being overweight . Esophageal Cancer Screening Key Points Tests are used to screen for ...

  2. Lower esophageal sphincter pressure in histologic esophagitis.

    Science.gov (United States)

    Welch, R W; Luckmann, K; Ricks, P; Drake, S T; Bannayan, G; Owensby, L

    1980-06-01

    The fasting lower esophageal sphincter pressure of 18 normal volunteers was compared to 22 patients with symptoms and objective evidence of gastroesophageal reflux. Lower esophageal sphincter pressure was measured by rapid pull-through using an 8-lumen radially perfused catheter that sampled pressure every45 degrees around the circumference of the sphincter. The 22 reflux patients were subdivided for analysis into two groups, those with an acute inflammatory infiltrate on biopsy and those without inflammation. Those patients without inflammatory esophagitis had normal sphincter pressures. Those with a definite inflammatory infiltrate had pressures significantly less than normal. The least reliable separation between normals and those with inflammatory esophagitis occurred in the anterior orientations. We conclude that while basal lower esophageal sphincter pressure measurement may identify patients with reflux and inflammatory esophagitis, it is of no help in identifying those patients with reflux unassociated with inflammation. Decreased basal fasting LESP does not appear to be the most important primary determinant of gastroesophageal reflux.

  3. A Rare Case of Jejunal Atresia Due to Intrauterine Intussusception

    OpenAIRE

    Joshi, Sanjeev B; Kinhal, Vidyadhar; Desai, Mahesh; Tilak,; Choudhari, Fazal UR Rehman

    2015-01-01

    Intestinal atresia is generally caused by intrauterine vascular obstructions involving mesenteric vessels. Intrauterine intussusceptions (IUI) are one of these disruptive events. Intestinal intussusceptions affects children commonly between 3 months and 3 years of age, but it rarely affects in intrauterine life. The relationship between intrauterine intussusception and intestinal atresia has been demonstrated by few cases in literature, suggesting intrauterine intussusception as a rare cause ...

  4. Radiological features of atresia of the aortic isthmus

    Energy Technology Data Exchange (ETDEWEB)

    Khalil, M.; Taenzer, V.; Trempenau, B.

    1986-10-01

    The most important angiographic features of atresia of the aortic isthmus are illustrated by three patients. The first patient to have reached an advanced age despite atresia of the aortic isthmus is reported and the diagnosis was confirmed at autopsy. The haemodynamic effects, the age at operation and the incidence of other associated cardiovascular malformations are discussed with reference to the literature.

  5. Promoting nerve regeneration through long gaps using a small nerve tissue graft.

    Science.gov (United States)

    Saito, Ikuo; Oka, Yoshinori; Odaka, Mitsuaki

    2003-03-01

    If nerve tissue is capable of inducing regeneration, as suggested by the neurotropism theory, then even small pieces of nerve tissue should have the potential to induce nerve regeneration. Therefore, long gaps might presumably be bridged via the neurotrophic potential of small pieces of nerve tissue grafted into the middle of the nerve gap. It is necessary to confirm the validity of the neurotropism theory and to also explore the potential usefulness of small nerve grafting through long gaps. A small piece of nerve tissue was grafted into a silicone tube bridging a relatively long nerve gap in an attempt to promote nerve regeneration. A 15-mm gap was created in the left sciatic nerve of 31 Wistar rats (8 weeks of age). The experimental groups included one with nonvascularized nerve tissue grafted into a silicone tube with no distal nerve suturing (NV-A), another with vascularized nerve tissue grafted into a silicone tube with no distal nerve suturing (V-A), a third group with nonvascularized nerve tissue grafted into a silicone tube with distal nerve suturing (NV-P), a fourth group with vascularized nerve tissue grafted into a silicone tube with distal nerve suturing (V-P), and a group with no nerve segment grafted into the silicone tube (control). Electrophysiologic and histologic examinations were performed 10 weeks after the operation. No regeneration was obtained in the control group. Nerve regeneration was evident at the proximal end of the tube in the NV-A, V-A, NV-P, and V-P groups, and at the distal end in the NV-P and V-P groups. The degree of distal regeneration was extremely slight in the NV-A and V-A groups. An electrophysiologic examination performed in the NV-P and V-P groups revealed better results in the latter group. Small nerve grafts are capable of inducing nerve regeneration even over a long nerve gap, by grafting nerve tissue into the middle of the lesion using a silicone tube.

  6. One-step management of apple-peel atresia.

    Science.gov (United States)

    Harper, L; Michel, J-L; de Napoli-Cocci, S; Aulagne, M-B; Maurel, A; Mazouzi, S; Ramful, D

    2009-01-01

    Apple-peel atresia is a rare form of bowel atresia associating proximal jejunal atresia with a typical coil-shaped distal small bowel. Many of the children suffering from this condition are still managed with initial enterostomy followed by delayed anastomosis. Between 2000 and 2007, we managed four patients with apple-peel atresia. Three by primary anastomosis and one by initial enterostomy with delayed anastomosis. Total duration of total parenteral nutrition (TPN) was an average 109 days. Survival rate was 100% with an average follow-up of 4.3 years. Nowadays, the prognosis of apple-peel atresia depends mainly on prolonged TPN-related morbidity rather than immediate postoperative complications. We therefore recommend that those infants affected be managed by primary anastomosis in order to reduce the duration of parenteral nutrition, followed by careful nutrition by teams experienced in neonatal TPN and short bowel syndrome.

  7. Effects of circular myotomy on the healing of esophageal suture anastomosis: an experimental study

    Directory of Open Access Journals (Sweden)

    Tannuri Uenis

    1999-01-01

    Full Text Available For esophageal reconstruction in newborns with esophageal atresia, esophageal reunion with an end-to-end anastomosis is the ideal procedure, although it may result in leaks and strictures due to tension on the suture line, mainly in cases with a wide gap between the ends. Circular myotomy (Livaditis' procedure is the best method to elongate the proximal esophageal pouch and reduce anastomotic tension. This experimental investigation in dogs was undertaken to attempt to verify that circular myotomy decreases the anastomotic leak rate in newborns with wide gap esophageal atresia, and to analyze whether the technique promotes morphologic changes in the anastomotic scar. A pilot study demonstrated that it is necessary to resect more than 8 cm (40% of the total esophageal length in order to obtain high leak rates. In the experimental project, such resection was performed in dogs divided into two groups (control group, anastomosis only, and experimental group, anastomosis plus circular myotomy in the proximal esophageal segment. The animals were killed in the 14th postoperative day, submitted to autopsy, and were evaluated as to the presence of leaks and strictures, as well as to the features (macroscopic and microscopic aspects of the anastomosis. Leak rates were the same in both groups. Morphometric analysis revealed that in animals in the experimental group, the anastomotic scar was thinner than the control animals, and the isolated muscular manchette distal to the site of myotomy was replaced by fibrous tissue. Correspondingly, a decreased number of newly formed small vessels were noted in the experimental animals, compared to control animals. We concluded that circular myotomy does not decrease the incidence of anastomotic leaks, and it also promotes deleterious changes in anastomotic healing.

  8. The Discharge Development and Arc Modes in Vacuum at A Long Gap Distance in Vacuum Interrupters

    Institute of Scientific and Technical Information of China (English)

    CHENG Shaoyong; XIU Shixin; WANG Jimei; SHEN Zhengchao

    2007-01-01

    The influence of an arc current on the discharge development and the arc modes of a single coil type axial magnetic field (AMF) electrode were investigated by a high-speed charge couple device (CCD) video camera in a long gap distance of 40 mm. The distribution of the axial magnetic field of the single coil type AMF electrode was computed. By computational results, the single coil type AMF electrode could generate higher axial magnetic flux density than the slot type AMF electrode. It was found that the single coil type AMF electrode could perform better than the slot type AMF electrode with the same designing parameters. And the development of the arc modes experienced diffuse mode, constricted but unstable mode, and constricted and stable mode with the amplitude of the arc current increasing. The correlation between the vacuum arc and the noise components of arc voltage was investigated too. The interruption capability could be known in a practical commercial vacuum interrupter by the test results in a demountable vacuum interrupter (DVC) with a electrode diameter of 50 mm and a gap distance of 40mm.The test results could provide reference to design the high voltage vacuum interrupter adopting the single coil type AMF electrode.

  9. Characteristics of long-gap AC streamer discharges under low pressure conditions

    Science.gov (United States)

    Yang, Yaqi; Li, Weiguo; Xia, Yu; Yuan, Chuangye

    2017-10-01

    The generation and propagation of a streamer is a significant physical process of air gap discharge. Research on the mechanism of streamers under low-pressure conditions is helpful for understanding the process of long-gap discharge in a high-altitude area. This paper describes laboratory investigations of streamer discharge under alternating current (AC) voltage in a low pressure test platform for a 60 cm rod-plane gap at 30 kPa, and analyzes the characteristics of streamer generation and propagation. The results show that the partial streamer and breakdown streamer all occur in the positive half-cycle of AC voltage near the peak voltage at 30 kPa. The partial streamer could cause the distortion of current and voltage waveform, and it appears as the branching characteristic at the initial stage. With the extension of the streamer, the branching and tortuosity phenomena become gradually obvious, but the branching is suppressed when the streamer crosses the gap. The low-pressure condition has little influence on the tortuosity length and the tortuosity number of the streamer, but affect the diameter of streamer obviously.

  10. Current options for management of biliary atresia.

    Science.gov (United States)

    Gallo, Amy; Esquivel, Carlos O

    2013-03-01

    It is encouraging that we are improving the technical aspects of treatment modalities for biliary atresia. However, it is clear that more needs to be done to best develop new treatment plans while applying the modalities we have (porto-enterostomy or liver transplantation or both) in a way that will afford the best survival and quality-of-life. This review article will discuss a number of points that are vital to improving care and illustrates the need to further scrutinize treatment decisions.

  11. Esophageal duplication and congenital esophageal stenosis.

    Science.gov (United States)

    Trappey, A Francois; Hirose, Shinjiro

    2017-04-01

    Esophageal duplication and congenital esophageal stenosis (CES) may represent diseases with common embryologic etiologies, namely, faulty tracheoesophageal separation and differentiation. Here, we will re-enforce definitions for these diseases as well as review their embryology, diagnosis, and treatment. Copyright © 2017. Published by Elsevier Inc.

  12. Intramural injection with botulinum toxin significantly elongates the pig esophagus

    DEFF Research Database (Denmark)

    Larsen, Heidi Fhær; Jensen, Thorbjørn Søren Rønn; Rasmussen, Lars

    2013-01-01

    Surgical treatment of long-gap esophageal atresia (LGEA) is challenging. Methods which facilitate stretching of the esophageal pouches may allow primary anastomosis. Botulinum toxin type A (BTX-A) blocks acetylcholine release in neuromuscular junctions, thereby causing muscle relaxation. We...

  13. Progress on materials and scaffold fabrications applied to esophageal tissue engineering

    Energy Technology Data Exchange (ETDEWEB)

    Shen, Qiuxiang; Shi, Peina; Gao, Mongna; Yu, Xuechan; Liu, Yuxin; Luo, Ling; Zhu, Yabin, E-mail: zhuyabin@nbu.edu.cn

    2013-05-01

    The mortality rate from esophageal disease like atresia, carcinoma, tracheoesophageal fistula, etc. is increasing rapidly all over the world. Traditional therapies such as surgery, radiotherapy or chemotherapy have been met with very limited success resulting in reduced survival rate and quality of patients' life. Tissue-engineered esophagus, a novel substitute possessing structure and function similar to native tissue, is believed to be an effective therapy and a promising replacement in the future. However, research on esophageal tissue engineering is still at an early stage. Considerable research has been focused on developing ideal scaffolds with optimal materials and methods of fabrication. This article gives a review of materials and scaffold fabrications currently applied in esophageal tissue engineering research. - Highlights: ► Natural and synthesized materials are being developed as scaffold matrices. ► Several technologies have been applied to reconstruct esophagus tissue scaffold. ► Tissue-engineered esophagus is a promising artificial replacement.

  14. Follicular atresia in the prepubertal spiny mouse (Acomys cahirinus) ovary.

    Science.gov (United States)

    Hułas-Stasiak, Monika; Gawron, Antoni

    2011-10-01

    This study was designed to determine follicular atresia in the newborn and the prepubertal spiny mouse. We analyzed the processes of follicle loss using classical markers of apoptosis (TUNEL reaction, active caspase-3) and autophagy (Lamp1). Numerous small clear vacuoles and autophagosomes as well as strong Lamp1 staining were observed in dying oocytes of all follicle types, especially of the primordial and primary ones. Active caspase 3 and the TUNEL reaction were detected only in the granulosa cells of large secondary and antral follicles. The expression of apoptosis and autophagy markers was also changing during the prepubertal period. Western blot analysis indicated that at the moment of birth, females undergo an increased rate of follicular atresia mediated by autophagy, while apoptosis is the dominant form of ovarian atresia in consecutive postnatal days. On the basis of these observations, we concluded that apoptosis and autophagy are involved in follicular atresia and these processes are cell and developmental stage-specific.

  15. Biliary Atresia: Cellular Dynamics and Immune Dysregulation

    Science.gov (United States)

    Feldman, Amy; Mack, Cara L.

    2012-01-01

    The cause of biliary atresia (BA) is unknown and in the past few decades the majority of investigations related to pathogenesis have centered on virus infections and immunity. The acquired or perinatal form of BA entails a progressive, inflammatory injury of bile ducts, leading to fibrosis and obliteration of both the extrahepatic and intrahepatic bile ducts. Theories of pathogenesis include viral infection, chronic inflammatory or autoimmune-mediated bile duct injury and abnormalities in bile duct development. This review will focus solely on human studies pertaining to a potential viral trigger of bile duct injury at diagnosis and provide insight into the interplay of the innate and adaptive immune responses in the pathogenesis of disease. PMID:22800972

  16. SMALL BOWEL DIVERTICULOSIS WITH JEJUNAL ATRESIA

    Directory of Open Access Journals (Sweden)

    Srinivas

    2015-07-01

    Full Text Available A 24 year s old primigravida had presented to us in third trimester with an antenatal ultrasound diagnosis of intestinal obstruction in the fetus and polyhydramnios. The parents were counseled and the obstetrician was advised to conduct the delivery as per obstetric indication, the patient delivered at 36 wks by an emergency LSCS. The baby was 1.8Kgs in weight and had an APGAR score of 9 and 10 at 1 and 5 minutes of life. Baby had significant bilious aspirate and did not pass meconium, it did not have dysmorphic featu res or any other associated anomalies, abdomen was soft and no masses were palpable. X ray abdomen revealed three gas bubbles with distal paucity of gas (Triple bubble sign. With the above findings a diagnosis of jejunal atresia was made and a laparotomy was planned on day 2 of life.

  17. Changes of smooth muscle contractile filaments in small bowel atresia

    OpenAIRE

    Gfrörer, Stefan; Fiegel, Henning; Ramachandran, Priya; Rolle, Udo; Metzger, Roman

    2012-01-01

    AIM: To investigate morphological changes of intestinal smooth muscle contractile fibres in small bowel atresia patients. METHODS: Resected small bowel specimens from small bowel atresia patients (n = 12) were divided into three sections (proximal, atretic and distal). Standard histology hematoxylin-eosin staining and enzyme immunohistochemistry was performed to visualize smooth muscle contractile markers α-smooth muscle actin (SMA) and desmin using conventional paraffin sections of the proxi...

  18. Esophageal lichen planus

    OpenAIRE

    Oliveira,Janine Pichler de; Uribe, Natalia Caballero; Abulafia,Luna Azulay; Quintella, Leonardo Pereira

    2015-01-01

    Abstract Lichen planus is a chronic inflammatory disease that affects the skin, mucous membranes, nails and scalp. Esophageal lichen planus is a rarely reported manifestation of lichen planus, presenting itself commonly in middle-aged women, with symptoms such as dysphagia. We report a case of esophageal lichen planus in a 54-year-old woman associated with oral, cutaneous and ungual lichen planus. Although lichen planus is a disorder well known by dermatologists, reports of esophageal lichen ...

  19. Endoscopic surgery in a child with unilateral choanal atresia

    Directory of Open Access Journals (Sweden)

    Diego HELLÍN-MESEGUER

    2016-03-01

    Full Text Available Introduction and objective: Choanal atresia is rare, being twice as common in women than in men, and in 50% of cases approximately it is associated with other congenital anomalies. Although there has been some controvesia about the treatment of choice of this malformation, in the last decade, the introduction of endoscopes in sinus surgery has improved the outcome. Case description: We report the case of a child who has left chronic rhinorrhea with persistent nasal obstruction. By endoscopy and CT diagnosis, left choanal atresia is confirmed. Unilateral choanal atresia endoscopic surgery is performed by creating flaps with intranasal mucosa, preventing nasal packing. Discussion: Different techniques have been considered for the treatment of choanal atresia, which include traditional and endoscopic approach. The results of endoscopic surgery in the choanal atresia vary according to different authors. The two most important aspects of these results are the creation of nasal mucosa flaps and avoiding nasal packing. Conclusions: Surgical treatment of unilateral choanal atresia by endoscopic surgery can reduce the risk of restenosis.

  20. Esophageal lichen planus.

    Science.gov (United States)

    Oliveira, Janine Pichler de; Uribe, Natalia Caballero; Abulafia, Luna Azulay; Quintella, Leonardo Pereira

    2015-01-01

    Lichen planus is a chronic inflammatory disease that affects the skin, mucous membranes, nails and scalp. Esophageal lichen planus is a rarely reported manifestation of lichen planus, presenting itself commonly in middle-aged women, with symptoms such as dysphagia. We report a case of esophageal lichen planus in a 54-year-old woman associated with oral, cutaneous and ungual lichen planus. Although lichen planus is a disorder well known by dermatologists, reports of esophageal lichen planus are rare in dermatologic literature. The esophageal lichen planus is little known and underdiagnosed, with a significant delay between the onset of symptoms and diagnosis.

  1. Esophageal capsule endoscopy

    Institute of Scientific and Technical Information of China (English)

    Ignacio Fernandez-Urien; Cristina Carretero; Raul Armendariz; Miguel Mu(n)oz-Navas

    2008-01-01

    Capsule endoscopy is now considered as the first imaging tool for small bowel examination.Recently,new capsule endoscopy applications have been developed,such as esophageal capsule endoscopy and colon capsule endoscopy.Esophageal capsule endoscopy in patients with suspected esophageal disorders is feasible and safe,and could be also an alternative procedure in those patients refusing upper endoscopy.Although large-scale studies are needed to confirm its utility in GERD and cirrhotic patients,current results are encouraging and open a new era in esophageal examination.

  2. Brain metastasis from esophageal carcinoma

    Directory of Open Access Journals (Sweden)

    Almasi Saeid

    2004-10-01

    Full Text Available Brain metastasis from esophageal carcinoma is rare. In our center, among 301 cases of esophageal cancer referred for radiotherapy during a 14-year period, brain metastasis from esophageal carcinoma was detected in one case. An unusual case of esophageal carcinoma that presented with brain metastasis is reported.

  3. Time-intensity trading in bilateral congenital aural atresia patients.

    Science.gov (United States)

    Schmerber, Sébastien; Sheykholeslami, Kianoush; Kermany, Mohammad Habiby; Hotta, Shoko; Kaga, Kimitaka

    2005-04-01

    In an effort to examine the rules by which information of bilaterally applied bone-conducted signals arising from interaural time differences (ITD) and interaural intensity differences (IID) is combined, data were measured for continuous 500 Hz narrow band noise at 65-70 dB HL in 11 patients with bilateral congenital aural atresia. Time-intensity trading functions were obtained by shifting the sound image towards one side using ITD, and shifting back to a centered sound image by varying the IID in the same ear (auditory midline task). ITD values were varied from -600 to +600 micros at 200 micros steps, where negative values indicate delays to the right ear. The results indicate that time-intensity trading is present in patients with bilateral aural atresia. The gross response properties of time-intensity trading in response to bone-conducted signals were comparable in patients with bilateral aural atresia and normal-hearing subjects, though there was a larger inter-subject variability and higher discrimination thresholds across IIDs in the atresia group. These results suggest that the mature auditory brainstem has a potential to employ binaural cues later in life, although to a restricted degree. A binaural fitting of a bone-conducted hearing aid might optimize binaural hearing and improve sound lateralization, and we recommend now systematically bilateral fitting in aural atresia patients.

  4. Transnasal endoscopic repair of acquired posterior choanal stenosis and atresia

    Institute of Scientific and Technical Information of China (English)

    WANG Qin-ying; WANG Shen-qing; LIN Shan; CHEN Hai-hong; LU Yu-yu

    2008-01-01

    Background There are conqenital and acquired choanal atresias and many approaches have been used for their repair.We assessed the clinical effect of power instrument.endoscopic repair of acquired choanaI stenosis and atresia.Methods Nineteen patients,aged from 32 to 61 years,with acquired choanal stenosis and atresia (from trauma in 5 cases and from radiotherapy after nasopharyngeal carcinoma in 14:6 bilateral and 13 unilateral cases),underwent transnasal endoscopic repair of choanal stenosis and atresia.No patient had stenting.Antibiotic and local glucocorticoid were administered postoperatively.Results Eiqhteen patients remained free of symptoms for 12-40 months after the surgery,and the diameter of the neochoana was more than 1 cm after the procedure.One patient required revision surgery and recovered completely with no restenosis at 12 months after the second surgery.There were no postoperative complications.Histology of the resected tissue revealed respiratory epithelial-lined stromal tissue with chronic inflammation,edema and fibrosis,but no tumor cells.Conclusions Transnasal endoscopic approach is a useful procedure for the repair of acquired choanal stenosis and atresia:it is highly successful,safe and effective with swift recovery and short time of hospitalizalion.It is very important in postoperative care to remove any granulation or polyps at the site of the neochoana at that time.

  5. Changes of smooth muscle contractile filaments in small bowel atresia

    Institute of Scientific and Technical Information of China (English)

    Stefan Gfroerer; Henning Fiegel; Priya Ramachandran; Udo Rolle; Roman Metzger

    2012-01-01

    AIM:To investigate morphological changes of intestinal smooth muscle contractile fibres in small bowel atresia patients.METHODS:Resected small bowel specimens from small bowel atresia patients (n =12) were divided into three sections (proximal,atretic and distal).Standard histology hematoxylin-eosin staining and enzyme immunohistochemistry was performed to visualize smooth muscle contractile markers α-smooth muscle actin (SMA) and desmin using conventional paraffin sections of the proximal and distal bowel.Small bowel from agematched patients (n =2) undergoing Meckel's diverticulum resection served as controls.RESULTS:The smooth muscle coat in the proximal bowel of small bowel atresia patients was thickened compared with control tissue,but the distal bowel was unchanged.Expression of smooth muscle contractile fibres SMA and desmin within the proximal bowel was slightly reduced compared with the distal bowel and control tissue.There were no major differences in the architecture of the smooth muscle within the proximal bowel and the distal bowel.The proximal and distal bowel in small bowel atresia patients revealed only minimal differences regarding smooth muscle morphology and the presence of smooth muscle contractile filament markers.CONCLUSION:Changes in smooth muscle contractile filaments do not appear to play a major role in postoperative motility disorders in small bowel atresia.

  6. Excellent long-term outcome for survivors of apple peel atresia

    NARCIS (Netherlands)

    Festen, S; Brevoord, JCD; Goldhoorn, GA; Festen, C; Hazebroek, FWJ; van Heurn, LWE; de Langen, ZJ; van der Zee, DC; Aronson, DC

    2002-01-01

    Background: Apple peel atresia is the rarest type of small bowel atresia. Because of its rare occurrence and high mortality rate, little is known about the long-term outcome of these children. Methods: The patient charts, operative reports, and office notes of 15 children with apple peel atresia fro

  7. Excellent long-term outcome for survivors of apple peel atresia

    NARCIS (Netherlands)

    Festen, S; Brevoord, JCD; Goldhoorn, GA; Festen, C; Hazebroek, FWJ; van Heurn, LWE; de Langen, ZJ; van der Zee, DC; Aronson, DC

    Background: Apple peel atresia is the rarest type of small bowel atresia. Because of its rare occurrence and high mortality rate, little is known about the long-term outcome of these children. Methods: The patient charts, operative reports, and office notes of 15 children with apple peel atresia

  8. Recurrent pneumothorax associated with bronchial atresia: report of a case.

    Science.gov (United States)

    Tanaka, Kazuhisa; Suzuki, Hidemi; Nakajima, Takahiro; Tagawa, Tetsuzo; Iwata, Takekazu; Mizobuchi, Teruaki; Yoshida, Shigetoshi; Yoshino, Ichiro

    2015-10-01

    We herein report a case of recurrent pneumothorax associated with congenital bronchial atresia. A 26-year-old male presented with chest pain. Chest roentgenograms showed left pneumothorax, a left apical bulla and an area of hyperlucency in the left upper lung field, and chest computed tomography revealed a discontinuation of the left superior bronchus. Additionally, both ventilation and perfusion scintigraphy showed a defect in the left superior segment. A thoracoscopy-assisted left superior segmentectomy was performed, and a pathological examination indicated left superior segmental bronchial atresia, which might have predisposed the peripheral lung to emphysematous conditions. No relapse was observed 6 months after the operation. Although this entity is rare, congenital bronchial atresia should be considered in the differential diagnosis when a patient has suffered from a recurrent spontaneous pneumothorax.

  9. Imaging findings of bronchial atresia in fetuses, neonates and infants

    Energy Technology Data Exchange (ETDEWEB)

    Alamo, Leonor; Meuli, Reto [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Diagnostic and Interventional Radiology, Lausanne (Switzerland); Vial, Yvan [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Obstetrics and Gynecology, Lausanne (Switzerland); Gengler, Carole [University Hospital of Lausanne (CHUV) and University of Lausanne (UNIL), Department of Pathology, Lausanne (Switzerland)

    2016-03-15

    Congenital lung malformations are increasingly detected before birth. However, bronchial atresia is rarely identified in utero and not always recognized in neonates. There are two types of atresia: (1) proximal, located at the level of the mainstem or the proximal lobar bronchi, which is extremely rare and usually lethal during pregnancy, causing a tremendous volume increase of the distal involved lung with secondary hypoplasia of the normal lung, and (2) peripheral, located at the segmental/subsegmental bronchial level, which may present as an isolated lesion or as part of a complex congenital malformation. Prenatal findings are mostly nonspecific. Postnatal exams show overinflated lung areas and focal bronchial dilations. The typical fluid-filled bronchoceles are not always observed in neonates but develop progressively in the first months of life. This pictorial essay describes the spectrum of imaging findings of bronchial atresia in fetuses, neonates and infants. (orig.)

  10. Radiation Therapy, Paclitaxel, and Carboplatin With or Without Trastuzumab in Treating Patients With Esophageal Cancer

    Science.gov (United States)

    2016-10-14

    Adenocarcinoma of the Gastroesophageal Junction; Esophageal Adenocarcinoma; Stage IB Esophageal Cancer; Stage IIA Esophageal Cancer; Stage IIB Esophageal Cancer; Stage IIIA Esophageal Cancer; Stage IIIB Esophageal Cancer

  11. Retrospective Study of a Series of Choanal Atresia Patients

    Science.gov (United States)

    Manica, Denise; Schweiger, Cláudia; Netto, Cátia C Saleh; Kuhl, Gabriel

    2013-01-01

    Introduction Although it has been more than 250 years since the first description of choanal atresia (CA), there are still doubts about this abnormality. The differences between unilateral and bilateral forms are seldom discussed. Objectives Aggregate data from patients diagnosed with CA, grouping patients with unilateral and bilateral forms. Methods Retrospective study. Results Eighteen patients were included: 12 (66.6%) presented bilateral atresia, of which 77.8% were mixed bony-membranous type and 22.2% were pure bony type. From the 12 patients with bilateral atresia, 10 presented related malformations, 3 of whom had CHARGE syndrome (coloboma, heart defects, choanal atresia, retardation of growth and development, genitourinary problems, ear abnormalities). From the remaining 6 patients with unilateral atresia, only 2 showed malformations, 1 renal and 1 cardiac. All patients with unilateral atresia needed only 1 surgical procedure, and patients with the bilateral form needed a median of 2.85 interventions (p = 0.003). The median age of surgical procedure in the unilateral group was 6 years, ranging from 6 months to 18 years, and in the bilateral group was 25 days, ranging from 6 days to 6 years (p = 0.003). The median interval between diagnosis and surgery was 9 months in the unilateral group, ranging from 1 month to 18 years, and in the bilateral group was 1 day, ranging from 1 day to 2 months (p = 0.001). Discussion and Conclusions Success rates with the endoscopic approach vary from 62 to 100%. Nonetheless, most of these reports present results without considering the number of compromised sides. In our opinion, unilateral and bilateral cases involve distinct patients (taking into account the related malformations), have diverging clinical presentations, and show discrepant restenosis rates and therefore could be considered in different groups of analysis. PMID:25992054

  12. Anesthesia for Congenital Esoshageal Atresia%先天性食管闭锁的麻醉

    Institute of Scientific and Technical Information of China (English)

    王大柱; 郭奉玲; 孙乃珠; 李凤荣; 王秀珍

    1988-01-01

    The artical reports the experience of anesthesia for 40 cases with congenital esophageal atresia.Most of the infants had a low birth weight and had complication of pneumonia or other malformations,so the anesthesia was very difficult. The patients were intubated after acetylpromazine and promethazine injected intramuseulary.Anesthesia was maintained by ketamine,diazepam or dolantine.the respiration was assisted by the modifications of the"T" piece system. An appropriate anesthesia was directly related to the postoperative survival rate.No death and no su ch side effects as laryngospasm or asphyxia resulted from ketamine or diazepare.The function of cardiovascular system was stzble.The infants were conscious and could be extu bated soon after operation.%本文报道40例先天性食管闭锁手术的麻醉,其中早产儿占50%.全部患儿合并肺炎,50%以上患儿还合并有多种畸形.采用清醒插管,氯胺酮,安定麻醉、应用根据T管原理改良的小儿呼吸囊管理呼吸,取得了良好效果,麻醉、手术过程平稳,循环稳定,术终苏醒迅速.

  13. Management of congenital choanal atresia: A pedodontist′s role

    Directory of Open Access Journals (Sweden)

    Fathima Niloofar

    2015-01-01

    Full Text Available Choanal atresia is a rare cryptic congenital anomaly with developmental failure of the nasal cavity to communicate with the nasophaynx, resulting in a significant physiological and anatomical alteration of the dento-facial complex. It is considered as a life-threatening or lethal congenital malformation in an obligatory nasal breathing neonate. Elective transnasal endoscopic repair is the most preferred and minimally invasive procedure to correct this anomaly. This case report describes the multi-disciplinary team approach that included pedodontists to manage the case of a 6-month-old infant with choanal atresia suffering from breathlessness, feeding difficulty, and disturbed sleep.

  14. Methylation in esophageal carcinogenesis

    Institute of Scientific and Technical Information of China (English)

    Da-Long Wu; Feng-Ying Sui; Xiao-Ming Jiang; Xiao-Hong Jiang

    2006-01-01

    Genetic abnormalities of proto-oncogenes and tumor suppressor genes have been demonstrated to be changes that are frequently involved in esophageal cancer pathogenesis. However, hypermethylation of CpG islands, an epigenetic event, is coming more and more into focus in carcinogenesis of the esophagus. Recent studies have proved that promoter hypermethylation of tumor suppressor genes is frequently observed in esophageal carcinomas and seems to play an important role in the pathogenesis of this tumor type. In this review, we will discuss current research on genes that are hypermethylated in human esophageal cancer and precancerous lesions of the esophagus. We will also discuss the potential use of hypermethylated genes as targets for detection, prognosis and treatment of esophageal cancer.

  15. Bleeding esophageal varices

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000268.htm Bleeding esophageal varices To use the sharing features on ... veins in the esophagus to balloon outward. Heavy bleeding can occur if the veins break open. Any ...

  16. Oral and esophageal disorders.

    Science.gov (United States)

    Noyer, C M; Simon, D

    1997-06-01

    This article focused on the approach to oral and esophageal disorders in patients with AIDS. Most of these disorders respond to various therapeutic regimens. Some of the oral complications can be prevented with dental prophylaxis, whereas recurrent esophageal disease in some patients may require long-term suppressive therapy. As patients with AIDS live longer with lower CD4 counts, gastroenterologists need to become familiar with the approach to and management of the more common lesions of the mouth and esophagus.

  17. Tracheo-esophageal fistula in children: a diagnosis to keep in mind. Two case reports and review of the literature = Fístula traqueoesofágica en niños: un diagnóstico para tener en cuenta. Reporte de dos casos y revisión de la literatura

    Directory of Open Access Journals (Sweden)

    Olga Lucía Morales Múnera

    2013-07-01

    Full Text Available The tracheo-esophageal fistula without esophageal atresia is a rare type esophageal malformation. It has a multifactorial etiology including environmental and genetic factors. Common clinical manifestations are coughing and choking after meals, cyanosis and/or recurrent pneumonia. Diagnosis requires a high clinical suspicion index. Fistula confirmation is done with imaging studies including upper digestive series, video-fluoroscopy or with the use of bronchoscopy wich allows direct visualization of the fistula or methylene blue passage through the abnormal communication. Fistula closure can be done endoscopically or surgically, in both cases with good results.

  18. Long-term pulmonary function in esophageal atresia-A case-control study

    DEFF Research Database (Denmark)

    Pedersen, Rikke N; Markøv, Simone; Kruse-Andersen, Søren

    2017-01-01

    expiratory volume 1 sec (FEV1) was 78.2% ± 12.4% of predicted, and forced expiratory fraction 25-75% (FEF25-75%) was 71.5 ± 17.8% of predicted in EA patients, lower than disease controls (P TLC) was lower in patients with EA than in the controls (P 

  19. Endoscopic management of esophageal stenosis in children:New and traditional treatments

    Institute of Scientific and Technical Information of China (English)

    2016-01-01

    Post-esophageal atresia anastomotic strictures and postcorrosiveesophagitis are the most frequent types ofcicatricial esophageal stricture. Congenital esophagealstenosis has been reported to be a rare but typicaldisease in children; other pediatric conditions are peptic,eosinophilic esophagitis and dystrophic recessiveepidermolysis bullosa strictures. The conservative treatmentof esophageal stenosis and strictures (ES) ratherthan surgery is a well-known strategy for children.Before planning esophageal dilation, the esophagealmorphology should be assessed in detail for its length,aspect, number and level, and different conservativestrategies should be chosen accordingly. Endoscopicdilators and techniques that involve different adjuvanttreatment strategies have been reported and dependon the stricture's etiology, the availability of differenttools and the operator's experience and preferences.Balloon and semirigid dilators are the most frequentlyused tools. No high-quality studies have reported on thedifferences in the efficacies and rates of complicationsassociated with these two types of dilators. There is noconsensus in the literature regarding the frequency ofdilations or the diameter that should be achieved. Theuse of adjuvant treatments has been reported in casesof recalcitrant stenosis or strictures with evidence ofdysphagic symptoms. Corticosteroids (either systemicallyor locally injected), the local application of mitomycin C,diathermy and laser ES sectioning have been reported.Some authors have suggested that stenting can reduceboth the number of dilations and the treatment length. Inmany cases, this strategy is effective when either metallicor plastic stents are utilized. Treatment complications,such esophageal perforations, can be conservativelymanaged, considering surgery only in cases with severepleural cavity involvement. In cases of stricture relapse,even if such relapses occur following the execution ofwell-conducted conservative

  20. Thirty-four years' experience with biliary atresia in Denmark

    DEFF Research Database (Denmark)

    Kvist, N; Davenport, M

    2011-01-01

    Biliary atresia (BA) is a rare disease in Denmark (population ∼ 5.5 million) and there has been some controversy on how smaller countries should manage such conditions to obtain the best possible outcomes. The aim of this study was to evaluate the efficacy of primary surgery (i. e., Kasai...

  1. Thirty-four years' experience with biliary atresia in Denmark

    DEFF Research Database (Denmark)

    Kvist, N; Davenport, M

    2011-01-01

    Biliary atresia (BA) is a rare disease in Denmark (population ~¿5.5 million) and there has been some controversy on how smaller countries should manage such conditions to obtain the best possible outcomes. The aim of this study was to evaluate the efficacy of primary surgery (i.¿e., Kasai...

  2. Duodenal atresia in association with situs inversus abdominus

    Directory of Open Access Journals (Sweden)

    Raghu Shankar

    2012-01-01

    Full Text Available We report a rare association of duodenal atresia with situs inversus abdominus in a newborn. The infantogram revealed "reverse double-bubble sign" without dextrocardia. The sonography and echocardiography confirmed the diagnosis of situs inversus abdominus with multiple cardiac anomalies. Laparotomy and a duodenoduodenostomy were carried out.

  3. Colonic atresia in cattle: A prospective study of 43 cases

    Science.gov (United States)

    Ducharme, Norm G.; Arighi, Mimi; Horney, F. Don; Barker, Ian K.; Livesey, Michael A.; Hurtig, Mark H.; Johnson, Roger P.

    1988-01-01

    This prospective study was initiated to document the success rate obtained in the treatment of colonic atresia in calves, identify factors that influence survival rate, and to report the histopathological appearance of the proximal blind end of the ascending colon. Forty-three calves with intestinal obstruction due to colonic atresia were admitted to the Ontario Veterinary College between September 1982 and May 1986. Parameters recorded prospectively in this study included age, breed, sex, history, vital signs, acid-base and electrolyte status, location of intestinal atresia, medical and surgical management, and outcome. The typical history and clinical signs included failure to pass meconium or feces, decreased appetite, and progressive depression and abdominal distension. The most common site of colonic atresia was the midportion of the spiral loop of the ascending colon (n = 25). Of the 43 calves, three (7%) were euthanized at surgery, 21 (49%) died in the hospital, and 19 (44%) survived and were discharged from the hospital. Four of the surviving calves died subsequent to discharge giving an overall long-term (mean 15.9 months) survival rate of 35%. No significant risk factors were identified, although experienced surgeons showed a trend towards increased survival rate. ImagesFigure 2.Figure 3.Figure 4.Figure 5. PMID:17423141

  4. Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case ...

    African Journals Online (AJOL)

    Pyloric atresia epidermolysis bullosa aplasia cutis syndrome: a case report and literature ... skin over the right leg from the knee joint up to the middle of the right foot. ... Examination indicated no signs of child abuse; the parents refused an ...

  5. Spontaneous oesophageal rupture with duodenal atresia in a newborn infant.

    OpenAIRE

    1981-01-01

    A newborn infant with spontaneous oesophageal rupture associated with duodenal atresia is described. The diagnosis was established by the pattern of hydropneumothorax on the chest x-ray film and by the aspiration of gastric juice by thoracocentesis. Necropsy showed a tear, which extended from the oesophagogastric junction to the upper portion of the stomach.

  6. Oesophageal atresia with tracheoesophageal fistula and anal atresia in a patient with a de novo microduplication in 17q12

    NARCIS (Netherlands)

    Smigiel, R.; Marcelis, C.L.M.; Patkowski, D.; Leeuw, N. de; Bednarczyk, D.; Barg, E.; Mascianica, K.; Maria Sasiadek, M.; Brunner, H.G.

    2014-01-01

    Oesophageal atresia (OA) and tracheoesophageal fistula (TOF) are foregut malformations with a heterogeneous etiology. OA/TOF may occur as an isolated anomaly or as part of a syndrome. Chromosomal anomalies have been reported in 6-10% of OA/TOF. Several genes have been implicated in cases of syndromi

  7. Unilateral pulmonary veins atresia: evaluation by computed tomography; Atresia unilateral das veias pulmonares: avaliacao por tomografia computadorizada

    Energy Technology Data Exchange (ETDEWEB)

    Eifer, Diego Andre; Arsego, Felipe Veras, E-mail: felipesoarestorres@gmail.com [Hospital de Clinicas de Porto Alegre (HCPA), RS (Brazil). Serv. de Radiologia; Torres, Felipe Soares [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Escola de Medicina

    2013-11-15

    Unilateral pulmonary vein atresia is a rare congenital condition. In addition to cardiac malformations or pulmonary hypertension, patients may present with recurrent pulmonary infections or hemoptysis in childhood or adolescence. The authors report a case where the typical findings of such condition were observed at computed tomography in an adult patient. (author)

  8. Oesophageal atresia with tracheoesophageal fistula and anal atresia in a patient with a de novo microduplication in 17q12

    NARCIS (Netherlands)

    Smigiel, R.; Marcelis, C.L.M.; Patkowski, D.; Leeuw, N. de; Bednarczyk, D.; Barg, E.; Mascianica, K.; Maria Sasiadek, M.; Brunner, H.G.

    2014-01-01

    Oesophageal atresia (OA) and tracheoesophageal fistula (TOF) are foregut malformations with a heterogeneous etiology. OA/TOF may occur as an isolated anomaly or as part of a syndrome. Chromosomal anomalies have been reported in 6-10% of OA/TOF. Several genes have been implicated in cases of syndromi

  9. Assessing esophageal dysphagia.

    Science.gov (United States)

    Kruger, Danielle

    2014-05-01

    Dysphagia, or difficulty swallowing, is a common problem. Although most cases are attributable to benign disease processes, dysphagia is also a key symptom in several malignancies, making it an important symptom to evaluate. The differential diagnosis of dysphagia requires an understanding of deglutition, in particular the oropharyngeal versus esophageal stages. Stroke is the leading cause of oropharyngeal dysphagia, which is common in older adults and frequently presents as part of a broader complex of clinical manifestations. In esophageal dysphagia, difficulty swallowing is often the main complaint and is caused by localized neuromuscular disorders or obstructive lesions.

  10. Association of Duodenal Atresia, Malrotation, and Atrial Septal Defect in a Down-Syndrome Patient

    Directory of Open Access Journals (Sweden)

    R Angotti

    2016-04-01

    Full Text Available Duodenal atresia is the frequent cause of neonatal intestinal obstruction. The association between duodenal atresia, intestinal malrotation, cardiac anomalies and Down syndrome is infrequently reported. We present a prenatally suspected case of duodenal atresia which was associated with malrotation and atrial septal defect in a patient of Down syndrome. Duodenotomy and resection of web was performed in addition to Ladd’s procedure. Postoperative course remained uneventful.

  11. Appearances are Deceptive - Passing a Nasogastric Tube does Not Always Rule Out Oesophageal Atresia.

    Science.gov (United States)

    Kumar, Manish; Thomas, Niranjan

    2016-04-01

    Oesophageal atresia/trachea-Oesophageal fistula is commonly diagnosed in the newborn period by inability to pass a nasogastric tube (NGT). We present the instance of a newborn baby where the diagnosis of oesophageal atresia was delayed because of an apparent successful passage of nasogastric tube to the stomach. Failure to reinsert the NGT raised the suspicion of oesophageal atresia which was confirmed by contrast study showing blind upper oesophageal pouch.

  12. Avaliação da atresia maxilar associada ao tipo facial Evaluation of maxillary atresia associated with facial type

    Directory of Open Access Journals (Sweden)

    Marina Gomes Pedreira

    2010-06-01

    Full Text Available OBJETIVOS: associar a atresia maxilar aos tipos faciais, verificar o dimorfismo entre os gêneros masculino e feminino, bem como a correlação entre os gêneros e os tipos faciais. MÉTODOS: inicialmente, a amostra constou de 258 telerradiografias da cabeça, em norma lateral. Após aplicada a análise do Vert de Ricketts, foram excluídas 108 telerradiografias, por não satisfazerem o critério de seleção. Portanto, a amostra final foi de 150 telerradiografias e 150 modelos pertencentes a 150 indivíduos brancos na faixa etária de 14 anos a 18 anos e 11 meses, independentemente do tipo de má oclusão. A mesma foi dividida em: 50 mesofaciais; 50 braquifaciais e 50 dolicofaciais. Para os 150 modelos, aplicou-se a análise de Schwarz. RESULTADOS: a presença da atresia maxilar na amostra selecionada correspondeu a 64% nos dolicofaciais, 58% nos braquifaciais e 52% nos mesofaciais. CONCLUSÕES: não houve evidência de associação da atresia com o tipo facial. Quanto ao dimorfismo de gênero, foi proporcionalmente maior para o dolicofacial masculino enquanto o feminino não apresentou proporções diferentes.OBJECTIVES: To associate maxillary atresia with facial types, investigating whether dimorphism occurs between males and females and evaluating the percentage of such dimorphism according to gender and facial type. METHODS: Initially, the sample consisted of 258 lateral cephalometric radiographs. After analyzing Ricketts' VERT index, 108 radiographs were excluded for not meeting the selection criteria. Therefore, the sample consisted of 150 lateral cephalometric radiographs and 150 models of 150 Caucasian individuals aged 14 years to 18 years and 11 months, regardless of malocclusion type. The sample was divided into 50 mesofacials, 50 brachyfacials and 50 dolichofacials. The Schwarz's analysis was applied to all 150 models. RESULTS: The presence of maxillary atresia in the sample consisted of 64% in dolichofacials, 58% in brachyfacials

  13. Unilateral pulmonary vein atresia: A rare case of hemoptysis

    Directory of Open Access Journals (Sweden)

    Cindy S. DeMastes-Crabtree

    2015-01-01

    Full Text Available We present a rare case of hemoptysis secondary to isolated unilateral pulmonary vein atresia. Isolated pulmonary vein atresia is a rare condition in which patients typically acquire a diagnosis in infancy and early childhood [Mataciunas et al.; Pourmoghadam et al.]. Our patient presented during puberty with several previous episodes of hemoptysis prior to her admission and diagnosis. The initial diagnosis was suspected in our patient from chest computerized tomography (CT, and confirmed with cardiac catheterization and pulmonary angiography. Treatment aim is to preserve lung function and minimize irreversible pulmonary remodeling [Pourmoghadam et al.; Harrison et al.]. Conservative monitoring can be considered with milder or asymptomatic cases, while others may require preoperative collateral artery banding, surgical anastomosis between the pulmonary vein (PV & left atrium (LA and even pneumonectomy [Pourmoghadam et al.].

  14. Anaesthesia for biliary atresia and hepatectomy in paediatrics

    Directory of Open Access Journals (Sweden)

    Rebecca Jacob

    2012-01-01

    Full Text Available The scope of this article precludes an ′in depth′ description of all liver problems and I will limit this review to anaesthesia for biliary atresia - a common hepatic problem in the very young - and partial hepatectomy in older children. I will not be discussing the problems of anaesthetising children with hepatitis, cirrhosis, congenital storage diseases or liver failure. Extrahepatic biliary obstruction is an obliterative cholangiopathy of infancy which is fatal if untreated. Diagnosis involves exclusion of other causes of neonatal jaundice and treatment involves a hepatico portoenterostomy carried out at the earliest. This is a review of current concepts in anaesthesia and postoperative management of neonates with extrahepatic biliary atresia. Anaesthesia for hepatic resection has seen great changes in recent times with the improvement in surgical techniques, technology and a better understanding of the underlying physiology. These are reviewed along with the problems of postoperative pain management.

  15. A newborn with duodenal atresia and a gastric perforation

    Directory of Open Access Journals (Sweden)

    Akcora Bulent

    2010-01-01

    Full Text Available Congenital duodenal atresia complicated by gastric perforation (GP is a very rare and a very mortal condition. Only three newborns could be cured in the reported 13 cases. We report a successfully treated newborn with this complicated disease. A 2-day-old male was hospitalized with prediagnosis of duodenal obstruction. Twelve hours later, significant abdominal distention occurred promptly. At laparotomy, GP and preampullary duodenal atresia were detected. Gastrorrhaphy and duodenoduodenostomy were performed in the same operation. The patient was discharged on the 15th postoperative day. This complicated disease can be treated by early diagnosis and surgical intervention. We choose one-stage operation because of the clean peritoneal cavity. However, generalized peritonitis may require two-stage operation in delayed cases.

  16. Congenital biliary atresia: liver injury begins at birth

    DEFF Research Database (Denmark)

    Makin, Erica; Quaglia, Alberto; Kvist, Nina

    2009-01-01

    -note review for infants with definite BA who underwent laparotomy within first week of life. RESULTS: Three infants were identified who had occlusive BA evident on the first day of life. In all cases, their liver was grossly normal, and histologic changes were trivial. CONCLUSION: This suggests......BACKGROUND: The timing of onset of liver injury in biliary atresia (BA) is not known, although in approximately 10% of cases, biliary pathologic condition associated with the biliary atresia splenic malformation syndrome must begin well before birth. METHODS: The study involved retrospective case...... that the detrimental cholestatic liver injury, later characteristic of BA, only begins from the time of birth despite a prenatal occlusive biliary pathology. It may be that tissue injury only occurs with the onset of the perinatal bile surge initiating periductal bile leakage and the triggering of an inflammatory...

  17. BIRTH INJURY RELATED UNILATERAL ANTERIOR NARES PARTIAL FIBROUS ATRESIA: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Jayagar

    2016-02-01

    Full Text Available Congenital atresia of anterior nares has been rarely reported and it may co-exist along with posterior choanal atresia. (1,2 In our case, birth injury caused by forceps delivery has caused unilateral anterior nares partial atresia. Twenty eight years old male patient came with the complaints of left nostril blocked sensation since birth, aggravated with attacks of upper respiratory tract infections. On examination he had left anterior nares partial atresia caused by fibrous bands as a result of birth injury due to instrumental delivery. Rhinoplasty performed to open-up left nostril and patient relieved of his symptoms and also on cosmetic appearance

  18. Contemporary Hearing Rehabilitation Options in Patients with Aural Atresia

    Directory of Open Access Journals (Sweden)

    Jacky F. W. Lo

    2014-01-01

    Full Text Available Congenital aural atresia is the failure of development of the external auditory canal. It usually occurs in conjunction with microtia, which is the malformation of the auricle due to a failure of development of the external ear. Aural atresia, with or without microtia, may significantly affect the hearing and social life of the patients. It is important for every medical practitioner to be aware of the possible treatment options for hearing rehabilitation in this group of patients. In the era of modern technology, new choices, including Bone-Anchored Hearing Aid (BAHA (Cochlear Ltd. and Oticon Medical, Vibrant Soundbridge (VSB (MED-EL, Innsbruck, Austria, and Bonebridge system (BB (MED-EL, Innsbruck, Austria, provide high-end alternatives to traditional Bone Conduction Hearing Aid and Auditory Canal Reconstruction. All these options have advantages and disadvantages, and they are appropriate for different patients and/or at different ages. This paper aims to provide an overview of the management of hearing rehabilitation in congenital aural atresia patients and a discussion of each treatment option.

  19. Treatment Options by Stage (Esophageal Cancer)

    Science.gov (United States)

    ... Cancer Prevention Esophageal Cancer Screening Research Esophageal Cancer Treatment (PDQ®)–Patient Version General Information About Esophageal Cancer ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  20. Treatment strategies in the management of jejunoileal and colonic atresia

    Directory of Open Access Journals (Sweden)

    Chadha Rajiv

    2006-01-01

    Full Text Available BACKGROUND/PURPOSE: The purpose of this prospective study was to review the operative findings, treatment strategies, as well as the results of management of 46 consecutive cases of jejunoileal and colonic atresia, managed over a 2-year period. MATERIALS AND METHODS: There were 42 patients with jejunoileal atresia (JIA and 4 with colonic atresia (CA. The 4 group types were: type I-membranous (n=20, type II- blind ends separated by a fibrous cord (n=6, type IIIa- blind ends with a V-shaped mesenteric defect (n=10, type IIIb- apple-peel atresia (n=4 and type IV- multiple atresias (n=6. Primary surgery for JIA consisted of resection with a single anastomosis (n=37, anastomosis after tapering jejunoplasty (n=3, multiple anastomosis (n=1 and a Bishop-Koop ileostomy (n=1. For CA, resection with primary anastomosis was performed. A single end-to-oblique anastomosis after adequate resection of dilated proximal bowel, was the preferred surgical procedure. In the absence of facilities for administering TPN, early oral/nasogastric (NG tube feeding was encouraged. In patients with anastomotic dysfunction, conservative treatment of the obstruction followed after its resolution by gradually increased NG feeds, was the preferred treatment protocol. RESULTS: Late presentation or diagnosis with hypovolemia, electrolyte imbalance, unconjugated hyperbilirubinemia (n=25 and sepsis (n=6, were significant preoperative findings. After resection and anastomosis, significant shortening of bowel length was seen in 16 patients (34.7%. Postoperative complications included an anastomotic leak (n=3, a perforation proximal to the anastomosis in 1 and anastomotic dysfunction in 5 patients. Full oral or NG tube feeding was possible only by the 13th to 31st postoperative day (POD, after the primary surgery in patients with anastomotic dysfunction and those undergoing reoperation. Overall, 38 patients survived (82.6%. Mortality was highest in patients with type IIIb or type IV

  1. Esophageal epiphrenic diverticulum associated with diffuse esophageal spasm.

    Science.gov (United States)

    Matsumoto, Hideo; Kubota, Hisako; Higashida, Masaharu; Manabe, Noriaki; Haruma, Ken; Hirai, Toshihiro

    2015-01-01

    Esophageal diverticulum, a relatively rare condition, has been considered to be associated with motor abnormalities such as conditions that cause a lack of coordination between the distal esophagus and lower esophageal sphincter. We herein report a case of esophageal epiphrenic diverticulum associated with diffuse esophageal spasm. A 73-year-old woman presented with dysphagia and regurgitation. Imaging examinations revealed a right-sided esophageal diverticulum located about 10cm above the esophagogastric junction. High-resolution manometry revealed normal esophageal motility. However, 24-h pH monitoring revealed continuous acidity due to pooling of residue in the diverticulum. An esophageal epiphrenic diverticulum was diagnosed and resected thoracoscopically. Her dysphagia recurred 2 years later. High-resolution manometry revealed diffuse esophageal spasm. The diverticulum in the present case was considered to have been associated with diffuse esophageal spasm. The motility disorder was likely not identified at the first evaluation. In this case, the patient's symptoms spontaneously resolved without any treatment; however, longer-term follow-up is needed. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

  2. Esophageal epiphrenic diverticulum associated with diffuse esophageal spasm

    Science.gov (United States)

    Matsumoto, Hideo; Kubota, Hisako; Higashida, Masaharu; Manabe, Noriaki; Haruma, Ken; Hirai, Toshihiro

    2015-01-01

    Introduction Esophageal diverticulum, a relatively rare condition, has been considered to be associated with motor abnormalities such as conditions that cause a lack of coordination between the distal esophagus and lower esophageal sphincter. Presentation of case We herein report a case of esophageal epiphrenic diverticulum associated with diffuse esophageal spasm. A 73-year-old woman presented with dysphagia and regurgitation. Imaging examinations revealed a right-sided esophageal diverticulum located about 10 cm above the esophagogastric junction. High-resolution manometry revealed normal esophageal motility. However, 24-h pH monitoring revealed continuous acidity due to pooling of residue in the diverticulum. An esophageal epiphrenic diverticulum was diagnosed and resected thoracoscopically. Her dysphagia recurred 2 years later. High-resolution manometry revealed diffuse esophageal spasm. Discussion The diverticulum in the present case was considered to have been associated with diffuse esophageal spasm. The motility disorder was likely not identified at the first evaluation. Conclusion In this case, the patient’s symptoms spontaneously resolved without any treatment; however, longer-term follow-up is needed. PMID:26143577

  3. Atrio-esophageal fistula complicating esophageal achalasia.

    Science.gov (United States)

    Achouh, Paul; Pouly, Julia; Azarine, Arshid; Fabiani, Jean-Noël

    2011-08-01

    A 75-year-old male, known to have achalasia, was admitted to the intensive care unit with massive upper gastrointestinal bleeding and sepsis. He had a history of purulent pericarditis 18 months earlier. He also presented with atrial fibrillation associated with a cerebral transient ischemic accident two months earlier. A contrast computed tomography scan showed an atrio-esophageal fistula with active extravasation of contrast. He was operated on via a median sternotomy, and the defects in the atrial wall, inferior vena cava and diaphragm were closed using pericardial patches. An esophagectomy was to be performed 24 hours later, but the patient died from septic shock and multiple organ failure before his second procedure.

  4. Esophagitis Induced by Doxycycline Treatment

    Directory of Open Access Journals (Sweden)

    Engin Şenel

    2016-09-01

    Full Text Available Esophagitis is the most common disease of the esophagus. Drug use is one of the reasons of chemical esophagitis. Herein, we report two male patients of 17 and 18 years of age who developed esophagitis in acne treatment with doxycycline. Both patients took doxycycline capsules for approximately one week. Ulcerations were detected at upper and mid-esophagus of the 17-year-old patient. Circular ulcerations were found at mid-esophagus of the 18-year-old case. Doxycycline induced esophagitis is a preventable disease with the physician giving appropriate medication ingestion advice to the patient.

  5. Epidemiological investigation of esophageal carcinoma

    Institute of Scientific and Technical Information of China (English)

    Hong Zhang; Shao-Hua Chen; You-Ming Li

    2004-01-01

    AIM: To review the characteristics of esophageal carcinoma in recent 30 years in the epidemiological investigation.METHODS: A total of 1 520 cases of esophageal carcinoma in the First Affiliated Hospital of Zhejiang University Medical College admitted from 1970 until now were reviewed. Their age, gender, position of carcinoma and histological type were analyzed.RESULTS: The morbidity of esophageal carcinoma was increasing during the observation period. Compared with the 1970s (9.5%), the ratio of adenocarcinoma significantly increased after the 1980s (19.1%). The difference was significant (P≤0.05).CONCLUSION: The morbidity of esophageal adenocarcinoma was increasing and advanced clinical study should be strengthened.

  6. MULTIPLE ASSOCIATED ANOMALIES IN PATIENTS OF DUODENAL ATRESIA: A CASE SERIES

    Directory of Open Access Journals (Sweden)

    Bilal Mirza

    2012-04-01

    Full Text Available Duodenal atresia has been reported in association with various malformations and syndromes common being Down syndrome, malrotation, and annular pancreas. Its association with multiple anomalies is rare and scarcely reported in literature. Herein 3 cases of duodenal atresia associated with multiple congenital anomalies are being reported.

  7. Forkhead box A3 attenuated the progression of fibrosis in a rat model of biliary atresia.

    Science.gov (United States)

    Dong, Rui; Yang, Yifan; Shen, Zhen; Zheng, Chao; Jin, Zhu; Huang, Yanlei; Zhang, Zhien; Zheng, Shan; Chen, Gong

    2017-03-30

    Biliary atresia is a rare, devastating disease of infants where a fibroinflammatory process destroys the bile ducts, leading to fibrosis and biliary cirrhosis, and death if untreated. The cause and pathogenesis remain largely unknown. We tried to investigate factors involved in biliary atresia, especially forkhead box A3 (Foxa3), which might exert a role in the treatment of liver disease. We used RNA sequencing to sequence the whole transcriptomes of livers from six biliary atresia and six choledochal cysts patients. Then, we employed a rat disease model by bile duct ligation (BDL) and adenovirus transduction to address the function of Foxa3 in biliary atresia. We found that tight junction, adherence junction, cell cycle, apoptosis, chemokine singling, VEGF and MAPK signaling pathways were enriched in biliary atresia livers. We showed that Foxa3 expression was notably decreased in liver samples from biliary atresia patients. More importantly, we found that its lower expression predicted a poorer overall survival of biliary atresia patients. Rats that received BDL surgery and Foxa3 expression adenovirus resulted in a significant decrease in the deposition of collagen, and expression of profibrotic cytokines (transforming growth factor-β and connective tissue growth factor) and fibrosis markers (α-smooth muscle actin, collagen I and collagen III), as compared with rats that received BDL surgery and control adenovirus. Our data suggested a protection role for Foxa3 during the progression of liver fibrosis in biliary atresia, and thereby supported increasing Foxa3 as a targeted treatment strategy.

  8. Seasonal prevalence and intensity of follicular atresia in Baltic cod Gadus morhua callarias L

    DEFF Research Database (Denmark)

    Kraus, Gerd; Tomkiewicz, Jonna; Diekmann, R.;

    2008-01-01

    of atresia, i.e. number of atretic oocytes in relation to normally developed vitellogenic oocytes, was low amounting to 1.4% on average. Similar to prevalence, relative intensity of atresia differed significantly between maturity stages and increased with decreasing female condition. The population egg loss...

  9. Apoptotic and proliferative changes during induced atresia of pre-ovulatory follicles in the rat

    NARCIS (Netherlands)

    A.L.L. Durlinger (Alexandra); P. Kramer; B. Karels (Bas); J.A. Grootegoed (Anton); J.Th.J. Uilenbroek (Jan); A.P.N. Themmen (Axel)

    2000-01-01

    textabstractAtresia, a degenerative process through which many follicles are removed from the growing pool, involves apoptotic changes in the follicular granulosa cells. To identify histochemical markers of early stages of atresia, an in-vivo rat model was used which al

  10. Fertility, pregnancy, and delivery after biventricular repair for pulmonary atresia with an intact ventricular septum.

    NARCIS (Netherlands)

    Drenthen, W.; Pieper, P.G.; Roos-Hesselink, J.W.; Zoon, N.; Voors, A.A.; Mulder, B.J.M.; Dijk, A.P.J. van; Vliegen, H.W.; Sollie, K.M.; Ebels, T.; Veldhuisen, D.J. van

    2006-01-01

    The objective of the present study to investigate fertility, pregnancy, and delivery in women with biventricular repair for pulmonary atresia with an intact ventricular septum (PAIVS). Using a nationwide registry (CONCOR), 37 patients with pulmonary atresia were identified, 6 of whom (aged 21 to 34

  11. [Giant esophageal fibrovascular polyp].

    Science.gov (United States)

    Palacios, Fernando; Contardo, Carlos; Guevara, Jorge; Vera, Augusto; Aguilar, Luis; Huamán, Manuel; Palomino, Américo; Yabar, Alejandro

    2003-01-01

    Fibrovascular polyps are extremely rare benign neoplasias of the esophagus, which usually originate in the lower cricoid area. They do not produce any discomfort in the patient for a long time, however it may make itself evident by the patient's regurgitation of the polyp, producing asphyxia or, more frequently, dysphagia. The case of a 58 year old male patient is presented herein, with a 9 month record of dysphagia, weight loss and intermittent melena. The barium x-ray showed a distended esophagus, with a tumor running from the upper esophageal sphincter to the cardia. The endoscopy confirmed the presence of a pediculated tumor, implanted in the cervical esophagus. Surgeons suspected the potential malignancy of the tumor and performed a transhiatal esophagectomy. The final pathologic diagnosis was giant fibrovascular esophageal polyp.

  12. Atresia of the bilateral pulmonary veins: a rare and dismal anomaly identified on cardiac CT

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo; Park, Sang-Hyub; Koo, Hyun Jung; Cho, Young Hoon; Lee, Eunsol [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea, Republic of)

    2014-08-15

    Imaging findings of bilateral pulmonary vein atresia have not been described. To describe cardiac CT findings and clinical outcomes of bilateral pulmonary vein atresia. Three newborns with bilateral pulmonary vein atresia were encountered at our institution during a period of 8 years. We evaluated prenatal echocardiographic findings, clinical presentations, postnatal echocardiographic findings, chest radiographic findings, cardiac CT findings and clinical outcomes. All newborns presented immediately after birth with severe cyanosis, respiratory distress and acidosis that were unresponsive to medical management. Prenatal and postnatal echocardiographic studies and chest radiography were misleading, inconclusive or nonspecific in making the diagnosis in these children; however cardiac CT clearly demonstrated atresia of the bilateral pulmonary veins with multiple small mediastinal collateral veins and pulmonary edema. Surgical treatments were not feasible for this anomaly. Their clinical outcomes were universally dismal and all infants died within 3 days. Cardiac CT provides an accurate diagnosis of bilateral pulmonary vein atresia and leads to prompt treatment decision in these children. (orig.)

  13. Esophageal Inlet Patch

    Directory of Open Access Journals (Sweden)

    C. Behrens

    2011-01-01

    Full Text Available An inlet patch is a congenital anomaly consisting of ectopic gastric mucosa at or just distal to the upper esophageal sphincter. Most inlet patches are largely asymptomatic, but in problematic cases complications related to acid secretion such as esophagitis, ulcer, web and stricture may occur. The diagnosis of inlet patch is strongly suggested on barium swallow where the most common pattern consists of two small indentations on the wall of the esophagus. The diagnosis of inlet patch is confirmed via endoscopy with biopsy. At endoscopy, the lesion appears salmon-coloured and velvety and is easily distinguished from the normal grey-white squamous epithelium of the esophagus. The prominent margins correlate with the radiological findings of indentations and rim-like shadows on barium swallow. Histopathology provides the definitive diagnosis by demonstrating gastric mucosa adjacent to normal esophageal mucosa. No treatment is required for asymptomatic inlet patches. Symptomatic cases are treated with proton pump inhibitors to relieve symptoms related to acid secretion. Strictures and webs are treated with serial dilatation and should be biopsied to rule out malignancy.

  14. Hypnotherapy for Esophageal Disorders

    Science.gov (United States)

    Riehl, Megan E.; Keefer, Laurie

    2015-01-01

    Hypnotherapy is an evidence based intervention for the treatment of functional bowel disorders, particularly irritable bowel syndrome. While similar in pathophysiology, less is known about the utility of hypnotherapy in the upper gastrointestinal tract. Esophageal disorders, most of which are functional in nature, cause painful and uncomfortable symptoms that impact patient quality of life and are difficult to treat from a medical perspective. After a thorough medical workup and a failed trial of proton pump inhibitor therapy, options for treatment are significantly limited. While the pathophysiology is likely multifactorial, two critical factors are believed to drive esophageal symptoms—visceral hypersensitivity and symptom hypervigilance. The goal of esophageal directed hypnotherapy is to promote a deep state of relaxation with focused attention allowing the patient to learn to modulate physiological sensations and symptoms that are not easily addressed with conventional medical intervention. Currently, the use of hypnosis is suitable for dysphagia, globus, functional chest pain/non-cardiac chest pain, dyspepsia, and functional heartburn. In this article the authors will provide a rationale for the use of hypnosis in these disorders, presenting the science whenever available, describing their approach with these patients, and sharing a case study representing a successful outcome. PMID:26046715

  15. esophageal cancer: preliminary results

    Directory of Open Access Journals (Sweden)

    Afsaneh Maddah Safaei

    2017-01-01

    Full Text Available Purpose: Dysphagia is a common initial presentation in locally advanced esophageal cancer and negatively impacts patient quality of life and treatment compliance. To induce fast relief of dysphagia in patients with potentially operable esophageal cancer high-dose-rate (HDR brachytherapy was applied prior to definitive radiochemotherapy. Material and methods : In this single arm phase II clinical trial between 2013 to 2014 twenty patients with locally advanced esophageal cancer (17 squamous cell and 3 adenocarcinoma were treated with upfront 10 Gy HDR brachytherapy, followed by 50.4 Gy external beam radiotherapy (EBRT and concurrent chemotherapy with cisplatin/5-fluorouracil. Results : Tumor response, as measured by endoscopy and/or computed tomography scan, revealed complete remission in 16 and partial response in 4 patients (overall response rate 100%. Improvement of dysphagia was induced by brachytherapy within a few days and maintained up to the end of treatment in 80% of patients. No differences in either response rate or dysphagia resolution were found between squamous cell and adenocarcinoma histology. The grade 2 and 3 acute pancytopenia or bicytopenia reported in 4 patients, while sub-acute adverse effects with painful ulceration was seen in five patients, occurring after a median of 2 months. A perforation developed in one patient during the procedure of brachytherapy that resolved successfully with immediate surgery. Conclusions : Brachytherapy before EBRT was a safe and effective procedure to induce rapid and durable relief from dysphagia, especially when combined with EBRT.

  16. European biliary atresia registries: summary of a symposium

    DEFF Research Database (Denmark)

    Petersen, C.; Harder, D.; Abola, Z.

    2008-01-01

    with variable follow-up revealed an overall survival of 78% (range from 41% to 92%), of whom 342 patients (37%) have had liver transplants. Survival with native liver ranged from 14% to 75%. There was a marked variance in reported management and outcome by country (e.g., referral patterns, timing of surgery......, centralization of surgery). In conclusion, EBAR represents the first attempt at an overall evaluation of the outcome of BA from a pan-European perspective. The natural history and outcome of biliary atresia is of considerable relevance to a European population. It is essential that there is further support...

  17. [Amniotic band sequence and bilateral choanal atresia: a case report].

    Science.gov (United States)

    Del Toro-Valero, Azucena; Estrada-De la Fuente, Alejandro; Velázquez Santana, Héctor; Glicerio González, Jorge; Navarro Meza, María Cristina; Ortega-Hinojosa, Lilia; López-Cardona, María Guadalupe

    2011-08-01

    Amniotic band sequence (ABS) is a group malformation that mainly affects limbs; clinically, constriction rings and lymphedema of the fingers, arms and legs, acrosyndactyly and pseudosyndactyly are observed; also there is congenital amputation of limbs due to distal swelling. Less frequently, craniofacial and trunk involvement are reported in some patients. Etiology is still unknown and most cases are isolated. In this report we present the case of a 45-day-old male with diagnosis of SBA and bilateral choanal atresia as attached finding, and review possible causes of SBA and associated alterations.

  18. Hepatobiliary scintigraphy for early diagnosis of biliary atresia

    DEFF Research Database (Denmark)

    Brittain, Jane Maestri; Kvist, Nina; Johansen, Lars Søndergaard;

    2016-01-01

    INTRODUCTION: The aim of this study was to evaluate the validity of (99m)Technetium-trimethylbromo-iminodiacetic acid hepatobiliary scintigraphy (HS) for the diagnosis of biliary atresia (BA). METHODS: From January 2005 to December 2009, a total of 47 infants with conjugated hyperbilirubinaemia...... sensitivity and NPV prevent un-necessary surgery. Because of the low specificity of HS in diagnosing BA, it should be part of a multimodality imaging strategy when the result supports a clinical suspicion of BA. In cases with non-draining HS and normal GGTP blood levels, supplemental imaging modalities...

  19. Bronchial atresia in a neonate with congenital cytomegalovirus infection

    Directory of Open Access Journals (Sweden)

    Abdullah A Yousef

    2013-01-01

    Full Text Available Bronchial atresia (BA is characterized by a mucus-filled bronchocele in a blind-ending segmental or lobar bronchus with hyperinflation of the obstructed segment of the lung. We describe a neonate who presented on his 9 th day of life with respiratory distress. Chest computed tomography showed a soft tissue density involving the right middle lobe (RML. RML lobectomy confirmed the diagnosis of BA. Cytomegalovirus was detected by polymerase chain reaction in blood, urine, and tracheal aspirates which may provide further insight into the pathogenesis of BA.

  20. Dietary habits and esophageal cancer.

    Science.gov (United States)

    Palladino-Davis, A G; Mendez, B M; Fisichella, P M; Davis, C S

    2015-01-01

    Cancer of the esophagus is an underestimated, poorly understood, and changing disease. Its overall 5-year survival is less than 20%, even in the United States, which is largely a function of a delay in diagnosis until its more advanced stages. Additionally, the epidemiologic complexities of esophageal cancer are vast, rendering screening and prevention limited at best. First, the prevalence of esophageal cancer is unevenly distributed throughout the world. Second, the two histological forms (squamous cell and adenocarcinoma) vary in terms of their geographic prevalence and associated risk factors. Third, some populations appear at particular risk for esophageal cancer. And fourth, the incidence of esophageal cancer is in continuous flux among groups. Despite the varied prevalence and risks among populations, some factors have emerged as consistent associations while others are only now becoming more fully recognized. The most prominent, scientifically supported, and long-regarded risk factors for esophageal cancer are tobacco, alcohol, and reflux esophagitis. Inasmuch as the above are regarded as important risk factors for esophageal cancer, they are not the sole contributors. Dietary habits, nutrition, local customs, and the environment may be contributory. Along these lines, vitamins, minerals, fruits, vegetables, meats, fats, salted foods, nitrogen compounds, carcinogens, mycotoxins, and even the temperature of what we consume are increasingly regarded as potential etiologies for this deadly although potentially preventable disease. The goal of this review is to shed light on the less known role of nutrition and dietary habits in esophageal cancer.

  1. Gender effects on esophageal motility

    Directory of Open Access Journals (Sweden)

    Dantas R.O.

    1998-01-01

    Full Text Available It has been suggested that there are no gender effects on esophageal motility. However, in previous studies the subjects did not perform multiple swallows and the quantitative features of esophageal contractions were not evaluated. In order to investigate the gender effects on esophageal motility we studied 40 healthy normal volunteers, 20 men aged 37 ± 15 years (mean ± SD, and 20 women aged 38 ± 14 years. We used the manometric method with an eight-lumen polyvinyl catheter and continuous perfusion. The upper and lower esophageal sphincter pressures were measured by the rapid pull-through method. With the catheter positioned with one lumen opening in the lower esophageal sphincter, and the others at 5, 10 and 15 cm above the sphincter, ten swallows of a 5-ml water bolus alternated with ten dry swallows were performed. Statistical analysis was done by the Student t-test and Mann-Whitney test. Gender differences (P<0.05 were observed for wet swallows in the duration of contractions 5 cm above the lower esophageal sphincter (men: 3.7 ± 0.2 s, women: 4.5 ± 0.3 s, mean ± SEM, and in the velocity of contractions from 15 to 10 cm above the lower esophageal sphincter (men: 4.7 ± 0.3 cm/s, women: 3.5 ± 0.2 cm/s. There was no difference (P>0.05 in sphincter pressure, duration and percentage of complete lower esophageal sphincter relaxation, amplitude of contractions, or in the number of failed, multipeaked and synchronous contractions. We conclude that gender may cause some differences in esophageal motility which, though of no clinical significance, should be taken into consideration when interpreting esophageal motility tests.

  2. Seasonal variation in detection of esophageal eosinophilia and eosinophilic esophagitis

    Science.gov (United States)

    Jensen, Elizabeth T.; Shah, Neil D.; Hoffman, Kate; Sonnenberg, Amnon; Genta, Robert M.; Dellon, Evan S.

    2015-01-01

    Summary Background Seasonal variation has been reported in diagnosis of eosinophilic esophagitis (EoE), but results are not consistent across studies and there are no national-level data in the United States. Aim To determine if there is seasonal variation in diagnosis of esophageal eosinophilia and EoE in the U.S., while accounting for factors such as climate zone and geographic variation. Methods This was a cross-sectional study using a U.S. national pathology database. Patients with esophageal eosinophilia (≥15 eosinophils per high-power field) comprised the primary case definition and were compared to those with normal esophageal biopsies. We calculated the crude and adjusted odds of esophageal eosinophilia by season, as well as by day of the year. Sensitivity analyses were performed using more restrictive case definitions of EoE, and after stratification by climate zone. Results 14,524 cases with esophageal eosinophilia and 90,459 normal controls were analyzed. The adjusted odds of esophageal eosinophilia were higher in the late spring and summer months, with the highest odds in July (aOR 1.13; 95%CI: 1.03–1.24). These findings persisted with increasing levels of esophageal eosinophilia, as well as across EoE case definitions. Seasonal variation was strongest in temperate and cold climates, and peak diagnosis varied by climate zone. Conclusions There is a mild but consistent seasonal variation in the diagnosis of esophageal eosinophilia and EoE, with cases more frequently diagnosed during summer months. These findings take into account climate and geographic differences, suggesting that aeroallergens may contribute to disease development or flare. PMID:26059636

  3. Vibrant soundbridge in aural atresia: does severity matter?

    Science.gov (United States)

    McKinnon, B J; Dumon, T; Hagen, R; Lesinskas, E; Mlynski, R; Profant, M; Spindel, J; Van Beek-King, J; Zernotti, M

    2014-07-01

    Congenital aural atresia (CAA) poses significant challenges to surgical remediation. Both bone anchored hearing aids (BAHA) and the Vibrant Soundbridge (VSB) have been considered as alternatives or adjuncts to conventional atresiaplasty. A consensus statement on VSB implantation in children and adolescents recommended against implantation when the Jahrsdoerfer score was less than 8. More recent publications suggest that patients with Jahrsdoerfer scores between three and seven may benefit from VSB implantation. The purpose of this study was to further investigate the outcomes of VSB implantation in CAA. The study was a multi-center, retrospective review. A retrospective review of data (patient's demographic, clinical, implant and audiological information) from four collaborating centers that have performed VSB implantation in CAA was performed. Outcomes based on severity of the atresia using the Jahrsdoerfer and Yellon-Branstetter scoring systems were also evaluated. Data from 28 patients from the four centers revealed no iatrogenic facial nerve injuries or change in bone thresholds. Post-operative speech threshold and speech recognition was, respectively, 39 dB and 94%. Jahrsdoerfer and Yellon scores ranged from 4 to 9 and 4 to 12, respectively. The scores did not correlate to or predict outcomes. Three individual elements of the scores did correlate to initial, but not long-term outcomes. Atresiaplasty and BAHA in the management of CAA are not complete solutions. VSB may offer an alternative in these surgically complex patients for achieving amplification, though better metrics for patient selection need to be developed. LEVEL OF EVIDENCE : IV.

  4. [Prevalence of erosive esophagitis and peptic esophageal strictures].

    Science.gov (United States)

    Vasilevskiĭ, D I; Skurikhin, S S; Luft, A V; Mednikov, S N; Silant'ev, D S; Kulagin, V I; Dvoretskiĭ, S Iu; Bagnenko, S F

    2015-01-01

    Gastroesophageal reflux disease is a widespread among population in economically developed countries including Russia. It was analyzed the results of 34 903 endoscopic examinations of upper gastrointestinal tract in ethnically and socially homogeneous population of Leningrad region with symptoms of gastric dispepsia. Procedures were performed for the period 2007-2013. Prevalence of erosive esophagitis was 4.9%. Peptic esophageal strictures due to chronic reflux-associated inflammation were revealed in 0.2% of examined patients (3.7% of patients with erosive esophagitis). Obtained data allow to considergastroesophageal reflux disease as a socially significant problem in Russia requiring close attention and further study.

  5. Delayed Recognition of Type 1 Sigmoid-Colon Atresia: The Perforated Web Variety

    Directory of Open Access Journals (Sweden)

    Ghulam Mustafa

    2010-08-01

    Full Text Available Colonic atresias are the rare malformations of the colon and constitute about 1.7 to 15% of all gastrointestinal (GI atresias. A 6-month old infant presented with recurrent episodes of sub-acute intestinal obstruction since birth. During the index admission, patient had clinical signs of complete intestinal obstruction. The patient was operated and type I sigmoid-colon atresia found which on further exploration tuned out to be of perforated mucosal web variety. The resection of the involved part of colon and a primary end to oblique colo-colic anastomosis was performed.

  6. Endoscopic appearance of esophageal hematomas

    Institute of Scientific and Technical Information of China (English)

    Rodica Ouatu-Lascar; Gayatri Bharadhwaj; George Triadafilopoulos

    2000-01-01

    @@INTRODUCTION Esophageal hematomas develop from the dissection of the mucosa from the muscular layers of the esophageal wall and represent an uncommon condition affecting all ages[t-3]. Although the most common cause of esophageal hematomas is iatrogenic mechanical injury-induced by prolonged nasogastric intubation, difficult or forceful endoscopic intubation, or the result of variceal injection sclerotherapy- some may be spontaneous,particularly in patients receiving anticoagulants[3-6]. Presenting symptoms most commonly include dysphagia, hematemesis, and sub-sternal or epigastric pain[5,9].

  7. Absent upper blind Pouch in a case of tracheo-esophageal fistula

    Directory of Open Access Journals (Sweden)

    Man Mohan Harjai

    2015-01-01

    Full Text Available A common upper airway and digestive tract is a rare congenital anomaly that is usually fatal and its exact incidence is not known. It is a diagnostic challenge as it requires high index of suspicion. It should be considered in a neonate with respiratory distress in a non-vigorous baby requiring endotracheal intubation, which is difficult even in expert hand. We present a newborn with suspected tracheo-esophageal fistula that was diagnosed intraoperatively to have absent upper blind pouch of the esophagus and on autopsy found to have laryngeal atresia with absent vocal cords and a common aerodigestive tract continuing distally with trachea. The neonate was ventilated with endotracheal tube (ETT placement which in retrospect we came to know that it was in the esophagus. The neonate also had associated multiple congenital anomalies of VACTERL association. The importance of teamwork between neonatologist, pediatric surgeon, anesthesiologist, and radiologist is highlighted for diagnosis and management of such rare cases.

  8. Prevention of Esophageal Variceal Rebleeding

    Directory of Open Access Journals (Sweden)

    Gin-Ho Lo

    2006-12-01

    Full Text Available The rate of rebleeding of esophageal varices remains high after cessation of acute esophageal variceal hemorrhage. Many measures have been developed to prevent the occurrence of rebleeding. When considering their effectiveness in reduction of rebleeding, the associated complications cannot be neglected. Due to unavoidable high incidence of complications, shunt surgery and endoscopic injection sclerotherapy are now rarely used. Transjugular intrahepatic portosystemic stent shunt was developed to replace shunt operation but is now reserved for rescue therapy. Nonselective beta-blockers alone or in combination with isosorbide mononitrate and endoscopic variceal ligation are currently the first choices in the prevention of variceal rebleeding. The combination of nonselective beta-blockers and endoscopic variceal ligation appear to enhance the efficacy. With the advent of newly developed measures, esophageal variceal rebleeding could be greatly reduced and the survival of cirrhotics with bleeding esophageal varices could thereby be prolonged.

  9. Drugs Approved for Esophageal Cancer

    Science.gov (United States)

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for esophageal cancer. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  10. Esophageal ulceration complicating doxycycline therapy

    Institute of Scientific and Technical Information of China (English)

    Mohammad A. Al-Mofarreh; Ibrahim A. Al Mofleh

    2003-01-01

    AIM: To report present state of iatrogenic drug-induced esophageal injury (DIEI) induced by medications in a private clinic.METHODS: Iatrogenic drug-induced esophageal injury (DIEI) induced by medications has been more frequently reported. In a private clinic we encountered 36 cases of esophageal ulcerations complicating doxycycline therapy in a mainly younger Saudi population (median age 29 years).RESULTS: The most frequent presenting symptoms were oclynophagia, retrostemal burning pain and dysphagia (94 %,75 % and 56 %, respectively). The diagnosis was according to medical history and confirmed by endoscopy in all patients.Beside withdrawal of doxycycline, when feasible, all patients were treated with a proton-pump inhibitor (PPI) and a prokinetic. Thirty patients who reported to the clinic after treatment were improved within 1-7 (median 1.7) days.CONCLUSION: Esophageal ulceration has to be suspected in younger patients with odynophagia, retrosternal burning pain and/or clysphagia during the treatment with doxycycline.

  11. Caustic ingestion and esophageal function

    Energy Technology Data Exchange (ETDEWEB)

    Cadranel, S.; Di Lorenzo, C.; Rodesch, P.; Piepsz, A.; Ham, H.R. (Children University Hospital, Brussels (Belgium))

    1990-02-01

    The aim of the present study was to investigate esophageal motor function by means of krypton-81m esophageal transit scintigraphy and to compare the results with the functional and morphological data obtained by means of triple lumen manometry and endoscopy. In acute and subacute stages of the disease, all clinical, anatomical, and functional parameters were in good agreement, revealing significant impairment. In chronic stages, the severity of the dysphagia was not correlated to the importance of the residual stenosis. Conversely, 81mKr esophageal transit and manometric's findings were in good agreement with the clinical symptoms, during the entire follow-up period ranging between 3 months to 7 years. The 81mKr test is undoubtedly the easiest and probably the most physiological technique currently available for long-term functional evaluation of caustic esophagitis.

  12. Late presentation of gastric tube ulcer perforation after oesophageal atresia repair.

    Science.gov (United States)

    Hazebroek, Eric J; Hazebroek, Frans W J; Leibman, Steven; Smith, Garett S

    2008-07-01

    Ulcer formation in intrathoracic grafts after oesophageal replacement is considered an infrequent complication of the procedure. We present a rare case of a gastric tube ulcer with perforation, more than 30 years after gastric tube interposition for oesophageal atresia.

  13. Epidemiology of small intestinal atresia in Europe: a register-based study.

    LENUS (Irish Health Repository)

    Best, Kate E

    2012-09-01

    The epidemiology of congenital small intestinal atresia (SIA) has not been well studied. This study describes the presence of additional anomalies, pregnancy outcomes, total prevalence and association with maternal age in SIA cases in Europe.

  14. Assessment of liver fibrosis by Fibroscan as compared to liver biopsy in biliary atresia

    OpenAIRE

    Shen, Qiu-Long; Chen, Ya-Jun; Wang, Zeng-Meng; Zhang, Ting-Chong; Pang, Wen-Bo; Shu, Jun; Peng, Chun-Hui

    2015-01-01

    AIM: To evaluate liver stiffness measurement (LSM) using non-invasive transient elastography (Fibroscan) in comparison with liver biopsy for assessment of liver fibrosis in children with biliary atresia (BA).

  15. Fecundity regulation by atresia in turbot Scophthalmus maximus in the Baltic Sea

    DEFF Research Database (Denmark)

    Nissling, A.; Thorsen, A.; da Silva, Filipa

    2016-01-01

    spawned) v. potential fecundity (number of developing oocytes), suggesting significant variability in reproductive potential. The extent of fecundity regulation in relation to fish condition (Fulton’s condition factor) is discussed, suggesting an association between levels of atresia and fish condition......Down-regulation of fecundity through oocyte resorption was assessed in Baltic Sea turbot Scophthalmus maximus at three locations in the period from late vitellogenesis in April to spawning during June to July. The mean±s.d. total length of the sampled fish was 32⋅7±3⋅1 cm and mean±s.d. age was 6......⋅2±1⋅5 years. Measurements of atresia were performed using the ‘profile method’ with the intensity of atresia adjusted according to the ‘dissectormethod’ (10⋅6% adjustment; coefficient of determination was 0⋅675 between methods). Both prevalence (portion of fish with atresia) and intensity (calculated...

  16. Pregnancy after biventricular repair for pulmonary atresia with ventricular septal defect.

    NARCIS (Netherlands)

    Drenthen, W.; Pieper, P.G.; Zoon, N.; Roos-Hesselink, J.W.; Voors, A.A.; Mulder, B.J.M.; Dijk, A.P.J. van; Sollie, K.M.; Vliegen, H.W.; Ebels, T.; Veldhuisen, D.J. van

    2006-01-01

    Information on pregnancy and delivery in women with biventricular repair for isolated noncomplex pulmonary atresia with a ventricular septal defect (PAVSD) is limited. Using a nationwide congenital heart disease registry (CONgenital CORvitia [CONCOR]), 9 women with biventricular repair for PAVSD

  17. Ovarian Follicular Atresia of Ewes during Spring Puerperium

    Directory of Open Access Journals (Sweden)

    Radoslava Vlčková

    2012-01-01

    Full Text Available The distribution of healthy and atretic follicles on the ovarian surface of improved Valachian ewes 17, 24, and 32 days postpartum is reported in this study. The number of healthy follicles was higher on day 24 postpartum and their mean diameter tended to increase to day 32 (P<0.05 with the greatest diameter of 5 mm. 78–81% of atretic follicles ≥3 mm in diameter was observed where apoptosis began in the follicular cells situated at the follicular cavity. The early atretic follicles are characterized by the presence of mitotic pictures. In one ewe 24 days postpartum, small regressive follicular cysts were observed. Contracting atresia is characterized by thickening of the theca interna even to 190 μm. Progesterone and oestradiol-17β concentrations were maintained at relatively low levels, but with no significant difference between the days postpartum.

  18. Atresia pulmonar con comunicación interventricular

    Directory of Open Access Journals (Sweden)

    Tomasa Centella Hernández

    2014-04-01

    Full Text Available La atresia pulmonar con comunicación interventricular es una cardiopatía congénita cianótica, severa y rara, de alta complejidad, que se caracteriza por la ausencia de conexión entre el ventrículo derecho y las arterias pulmonares. Coexiste con una comunicación interventricular. El flujo hacia el territorio pulmonar puede realizarse a través del ductus arterioso o de colaterales sistémico-pulmonares. La dificultad de esta cardiopatía viene determinada por los diferentes niveles de interrupción desde el ventrículo derecho hasta el territorio pulmonar, y por la diferencia anatómica de las fuentes del flujo hacia dicho territorio, lo que determina diferentes tipos de abordaje quirúrgico.

  19. Gastric Duplication Cyst in Association with Duodenal Atresia in a Neonate

    Science.gov (United States)

    Mirshemirani, Alireza; Roshanzamir, Fatollah; Razavi, Sajad; Sarafi, Mehdi

    2016-01-01

    Concurrence of duodenal atresia and gastric duplication cyst is extremely rare entity. We report a 6-day-old female neonate who presented with neonatal intestinal obstruction. X-ray abdomen showed double bubble sign. At laparotomy, a huge cystic structure attached to greater curvature of the stomach along with duodenal atresia of second part of duodenum was found. The cystic structure was excised and duodeno-duodenostomy performed. Histopathology report confirmed it gastric duplication cyst. PMID:26816679

  20. Glycomic analyses of ovarian follicles during development and atresia

    Science.gov (United States)

    Hatzirodos, Nicholas; Nigro, Julie; Irving-Rodgers, Helen F.; Vashi, Aditya V.; Hummitzsch, Katja; Caterson, Bruce; Sullivan, Thomas R.; Rodgers, Raymond J.

    2012-01-01

    To examine the detailed composition of glycosaminoglycans during bovine ovarian follicular development and atresia, the specialized stromal theca layers were separated from the stratified epithelial granulosa cells of healthy (n = 6) and atretic (n = 6) follicles in each of three size ranges: small (3–5 mm), medium (6-9 mm) and large (10 mm or more) (n = 29 animals). Fluorophore-assisted carbohydrate electrophoresis analyses (on a per cell basis) and immunohistochemistry (n = 14) were undertaken. We identified the major disaccharides in thecal layers and the membrana granulosa as chondroitin sulfate-derived ∆uronic acid with 4-sulfated N-acetylgalactosamine and ∆uronic acid with 6-sulfated N-acetylgalactosamine and the heparan sulfate-derived Δuronic acid with N-acetlyglucosamine, with elevated levels in the thecal layers. Increasing follicle size and atresia was associated with increased levels of some disaccharides. We concluded that versican contains 4-sulfated N-acetylgalactosamine and it is the predominant 4-sulfated N-acetylgalactosamine proteoglycan in antral follicles. At least one other non- or 6-sulfated N-acetylgalactosamine proteoglycan(s), which is not decorin or an inter-α-trypsin inhibitor family member, is present in bovine antral follicles and associated with hitherto unknown groups of cells around some larger blood vessels. These areas stained positively for chondroitin/dermatan sulfate epitopes [antibodies 7D4, 3C5, and 4C3], similar to stem cell niches observed in other tissues. The sulfation pattern of heparan sulfate glycosaminoglycans appears uniform across follicles of different sizes and in healthy and atretic follicles. The heparan sulfate products detected in the follicles are likely to be associated with perlecan, collagen XVIII or betaglycan. PMID:22057033

  1. Atresia of large ovarian follicles of the rat

    Directory of Open Access Journals (Sweden)

    Maria Słomczyn´ ska

    2011-08-01

    Full Text Available In the rat, at the beginning of pregnancy a cohort of antral follicles develops until the preovulatory stage. However, these follicles, differentiating in the hyperprolactinemic milieu, produce only small amount of estradiol, do not ovulate and undergo rapid degeneration. They constitute an interesting physiological model of atresia. In the present study, we analysed the development and subsequent degeneration of such follicles. The study was performed on Wistar female rats killed in succession between days 1-9 of pregnancy. Excised ovaries were submitted to a routine histological procedure. Paraffin sections were subjected to hematoxylin and eosin staining or in situ DNA labelling. Histological and TUNEL staining revealed that the investigated group of follicles grew slower than that on the corresponding days of the estrous cycle and reached a preovulatory size and morphological appearance on day 5 of pregnancy. They did not ovulate and between days 6 and 9 of pregnancy an increasing number of apoptotic cells appeared within these follicles. They were localized predominantly in the antral granulosa layer, especially near the cumulus oophorus complex (COC and in the region linking the COC with the follicular wall. The COC and the theca layer were much less affected. In late stages of atresia, also cumulus cells became apoptotic but degenerating oocytes did not exhibit positive TUNEL staining. Only limited number of the theca cells have undergone apoptosis and generally they were not hypertrophied. Our findings indicate that much smaller than normal amount of intrafollicular estradiol was sufficient to support a normal, according to the morphological criteria, although slower development of antral follicles to the late preovulatory stage.

  2. The extended Kasai portoenterostomy for biliary atresia: A preliminary report

    Directory of Open Access Journals (Sweden)

    Priya Ramachandran

    2016-01-01

    Full Text Available Aims: In 1955, Professor Morio Kasai first performed a hepatic portoenterostomy. Since then, the procedure has changed the lives of children with biliary atresia (BA. We report our initial experience in performing "extended" Kasai portoenterostomy (KPE, a modification of the original procedure. Materials and Methods: Since 2013, we have used the technique of "extended KPE" and prospectively recorded data on all children undergoing this operation. Data on demographics, clinical features, liver function tests, and perioperative cholangiogram findings were collected. Outcome of KPE was measured by Jaundice Disappearance Rate (JDR and Native Liver Survival Rate (NLSR. We present our preliminary results from a 30-month period (February 2013 to May 2015. Results: Thirty-one children underwent KPE during this period (19 males and only 1 child had biliary atresia splenic malformation (BASM. The mean age at KPE was 73 ΁ 24 days. Five (16.1% children were more than 90 days old at the time of KPE. Fourteen children cleared jaundice (JDR 45.2%. Eleven (35.5% children developed episodes of cholangitis, of whom 8 had early cholangitis (within 3 months of the operation. The proportion of children who survived with their own liver 6 months after KPE (NLSR was 84.2%. Of those children older than 90 days, 2 cleared jaundice and have survived with their native livers for more than 16 months. Conclusion: In our preliminary report of 31 children, we conclude that the extended KPE leads to increased jaundice clearance and improved NLSR in children with BA.

  3. Esophageal malignancy: A growing concern

    Institute of Scientific and Technical Information of China (English)

    Jianyuan Chai; M Mazen Jamal

    2012-01-01

    Esophageal cancer is mainly found in Asia and east Africa and is one of the deadliest cancers in the world.However,it has not garnered much attention in the Western world due to its low incidence rate.An increasing amount of data indicate that esophageal cancer,particularly esophageal adenocarcinoma,has been rising by 6-fold annually and is now becoming the fastest growing cancer in the United States.This rise has been associated with the increase of the obese population,as abdominal fat puts extra pressure on the stomach and causes gastroesophageal reflux disease (GERD).Long standing GERD can induce esophagitis and metaplasia and,ultimately,leads to adenocarcinoma.Acid suppression has been the main strategy to treat GERD; however,it has not been proven to control esophageal malignancy effectively.In fact,its side effects have triggered multiple warnings from regulatory agencies.The high mortality and fast growth of esophageal cancer demand more vigorous efforts to look into its deeper mechanisms and come up with better therapeutic options.

  4. Cutaneous Metastases From Esophageal Adenocarcinoma

    Science.gov (United States)

    Triantafyllou, Stamatina; Georgia, Doulami; Gavriella-Zoi, Vrakopoulou; Dimitrios, Mpistarakis; Stulianos, Katsaragakis; Theodoros, Liakakos; Georgios, Zografos; Dimitrios, Theodorou

    2015-01-01

    The aim of this study is to present 2 rare cases of cutaneous metastases originated from adenocarcinoma of the gastro-esophageal junction, thus, underline the need for early diagnosis and possible treatment of suspicious skin lesions among patients with esophageal malignancy. Metastatic cancer to the skin originated from internal malignancies, mostly lung cancer, breast cancer, and colorectal cancer, constitute 0.5 to 9% of all metastatic cancers.5,8,15 Skin metastases, mainly from squamous cell carcinomas of the esophagus, are rarely reported. Cutaneous metastasis is a finding indicating progressiveness of the disease.17 More precisely, median survival is estimated approximately 4.7 months.2,14 This study is a retrospective review of 2 cases of patients with adenocarcinoma of the esophagus and a review of the literature. Two patients aged 60 and 32 years old, respectively, underwent esophagectomy. Both pathologic reports disclosed adenocarcinoma of the gastro-esophageal junction staged T3 N2 M0 (stage IIIB). During follow-up time, the 2 patients were diagnosed with cutaneous metastases originated from the primary esophageal tumor 11 and 4 months after surgery, respectively. The first patient is alive 37 months after diagnosis, while the second one died 16 months after surgery. Cutaneous metastasis caused by esophageal adenocarcinoma is possible. Therefore, follow-up of patients who were diagnosed with esophageal malignancy and underwent esophagectomy is mandatory in order to reveal early surgical stages. PMID:25785344

  5. Ultrasonographic findings of esophageal varices

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Hyo Seouk; Seol, Byeong Ryong; Rha, Seung Woon [Shilla General Hospital, Seoul (Korea, Republic of)

    1999-05-01

    To demonstrate the clinical usefulness of ultrasonography for detecting esophageal varices. In 20 cases of esophaged varix, the authors analysed the transabdominal ultrasonographic findings of the esophagogastric junction and compared mural thickness, the anteroposterior diameter of the esophagus, and the echogenic nature of the esophageal mucosal layer with those of 78 normal patients. The anterior and posterior mural thickeness of normal esophagus was 2.2{+-}0.7 and 2.4{+-}0.8mm respectively, but for variceal esophagus, the corresponding readings were 5.9{+-}1.3 and 5.2{+-}1.3mm respectively. The anteroposterior diameter of normal esophagus was 7.9{+-}2.1mm and that of variceal esophagus was 14.0{+-}1.8mm. There was a stastically significant difference (p<0.01) in mural thickness and anteroposterior diameter of the esophagus between a normal and variceal patient with regard to change of echogenic nature at the esphagogastric junction. Normal esophageal mucosa showed a thin and uniform echogenic line, but for variceal mucosa, the echogenic pattern was irregular, tortuous and thick. The athors believe that transabdominal US is helpful for detecting esophageal varices in patients with liver cirrhosis and UGI bleeding. Important clinically useful sonographic findings in diagnosing esophageal varix are as follows:1) mural thickness more than 6mm; 2) anteroposterior diameter of the esophagus of more than 15mm;3) irregular, tortuous and thickened echogenic mucosa.

  6. Methoxychlor and its metabolites inhibit growth and induce atresia of baboon antral follicles.

    Science.gov (United States)

    Gupta, Rupesh K; Aberdeen, Graham; Babus, Janice K; Albrecht, Eugene D; Flaws, Jodi A

    2007-08-01

    Methoxychlor (MXC), an organochlorine pesticide, inhibits growth and induces atresia of antral follicles in rodents. MXC metabolites, mono-OH MXC (mono-OH) and bis-OH MXC (HPTE), are thought to be more toxic than the parent compound. Although studies have examined effects of MXC in rodents, few studies have evaluated the effects of MXC in primates. Therefore, the present study tested the hypothesis that MXC, mono-OH, and HPTE inhibit growth and induce atresia of baboon antral follicles. To test this hypothesis, antral follicles were isolated from adult baboon ovaries and cultured with vehicle (dimethylsulfoxide; DMSO), MXC (1-100 micro g/ml), mono-OH (0.1-10 micro g/ml), or HPTE (0.1-10 micro g/ml) for 96 hr. Growth was monitored at 24 hr intervals. After culture, follicles were processed for histological evaluation of atresia. MXC, mono-OH, and HPTE significantly inhibited follicular growth and increased atresia compared to DMSO. Moreover, the adverse effects of MXC and its metabolites on growth and atresia in baboon antral follicles were observed at lower (100-fold) doses than those causing similar effects in rodents. These data suggest that MXC and its metabolites inhibit growth and induce atresia of baboon antral follicles, and that primate follicles are more sensitive to MXC than rodent follicles.

  7. Bilateral congenital choanal atresia and osteoma of ethmoid sinus with supernumerary nostril: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Li Xue-zhong

    2011-12-01

    Full Text Available Abstract Introduction Congenital choanal atresia is a relatively rare deformity, especially bilateral congenital choanal atresia. We report a case of bilateral congenital choanal atresia in a 22-year-old Chinese man, who was also diagnosed with congenital right accessory nasal deformity, osteoma of his left ethmoid sinus and congenital keratoleukoma of his right eye. Case presentation A 22-year-old Chinese man presented with mouth breathing, sleep snoring and difficult feeding after birth, with no olfactory sensation. Three-dimensional computed tomography revealed bilateral choanal atresia and a high density bony shadow in his left ethmoid sinus that extended to his left frontal sinus. Conclusions Choanal atresia is often accompanied by other congenital abnormalities. To the best of our knowledge, this is the first report of choanal atresia accompanied by congenital accessory nasal deformity and congenital keratoleukoma.

  8. Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators

    Directory of Open Access Journals (Sweden)

    Gürkan Altun

    2013-01-01

    Full Text Available Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein′s anomaly, Uhl′s anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.

  9. Supra-transumbilical laparotomy (STL approach for small bowel atresia repair: Our experience and review of the literature

    Directory of Open Access Journals (Sweden)

    Ernesto Leva

    2013-01-01

    Full Text Available Background: Supra-Transumbilical Laparotomy (STL has been used in paediatric surgery for a broad spectrum of abdominal procedures. We report our experience with STL approach for small bowel atresia repair in newborns and review previous published series on the topic. Patients and Methods: Fourteen patients with small bowel atresia were treated via STL approach at our Institution over a 5-year period and their charts were retrospectively reviewed. Results: STL procedure was performed at mean age of 3.1 day. No malrotation disorders were detected with pre-operative contrast enema. Eight patients (54.1% presented jejunal atresia, five (35.7% ileal atresia, and one (7.1% multiple ileal and jejunal atresias. Standard repair with primary end-to-back anastomosis was performed in all but one patient. In the newborn with multiple atresia, STL incision was converted in supra-umbilical transverse incision due to difficulty of exposition. After surgery, one patient developed anastomotic stricture, and another developed occlusion due to adhesions: Both infants required second laparotomy. No infections of the umbilical site were recorded, and cosmetic results were excellent in all patients. Conclusions: Increasing evidence suggests that STL approach for small bowel atresia is feasible, safe and provides adequate exposure for small bowel atresia surgery. When malrotation and colonic/multiple atresia are pre-operatively ruled out, STL procedure can be choosen as first approach.

  10. Prevention and Treatment of Esophageal Stenosis after Endoscopic Submucosal Dissection for Early Esophageal Cancer

    Directory of Open Access Journals (Sweden)

    Jing Wen

    2014-01-01

    Full Text Available Endoscopic submucosal dissection (ESD for the treatment of esophageal mucosal lesions is associated with a risk of esophageal stenosis, especially for near-circumferential or circumferential esophageal mucosal defects. Here, we review historic and modern studies on the prevention and treatment of esophageal stenosis after ESD. These methods include prevention via pharmacological treatment, endoscopic autologous cell transplantation, endoscopic esophageal dilatation, and stent placement. This short review will focus on direct prevention and treatment, which may help guide the way forward.

  11. Atresia das vias biliares extra-hepáticas: conhecimentos atuais e perspectivas futuras Extrahepatic biliary atresia: current concepts and future directions

    Directory of Open Access Journals (Sweden)

    Elisa de Carvalho

    2007-04-01

    Full Text Available OBJETIVOS: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. FONTES DOS DADOS: Foram selecionadas pelos sites de busca médica (MEDLINE e PubMed pesquisas relacionadas à atresia biliar, utilizando as seguintes palavras-chave: biliary atresia,etiopathogenesis, diagnosis, treatment, prognosis, children. SÍNTESE DOS DADOS A atresia das vias biliares extra-hepáticas é a principal indicação de transplante hepático na faixa etária pediátrica. Quanto à etiologia, o citomegalovírus, o reovírus e o rotavírus têm sido os agentes mais estudados como possíveis desencadeadores da obstrução imunomediada da árvore biliar. A resposta imune, especialmente o predomínio da resposta TH1 e do interferon gama, a susceptibilidade genética e as alterações do desenvolvimento embrionário da árvore biliar são aspectos que podem participar na etiopatogênese da obliteração das vias biliares extra-hepáticas. Ainda hoje, o único tratamento disponível é a portoenterostomia, cujos resultados são melhores quando realizada nos primeiros 2 meses de vida. Em relação ao prognóstico, as crianças não tratadas vão a óbito na totalidade, por complicações relacionadas à hipertensão portal e à cirrose hepática, e mesmo os casos tratados necessitam, em sua maioria, do transplante hepático. CONCLUSÃO: A atresia das vias biliares extra-hepáticas continua sendo a principal indicação de transplante hepático em crianças, e a mudança deste panorama depende de um melhor entendimento da etiopatogenia da obstrução biliar nos diferentes fenótipos da doença. Investigações futuras a respeito do papel do interferon gama e de outras citocinas são necessárias para avaliar se esses aspectos seriam potenciais alvos de intervenção terapêutica.OBJECTIVE: To provide an updated review on extrahepatic biliary atresia, focusing

  12. 21 CFR 878.3610 - Esophageal prosthesis.

    Science.gov (United States)

    2010-04-01

    ... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Esophageal prosthesis. 878.3610 Section 878.3610...) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices § 878.3610 Esophageal prosthesis. (a) Identification. An esophageal prosthesis is a rigid, flexible, or expandable tubular device...

  13. Gallium-67 imaging in candidal esophagitis

    Energy Technology Data Exchange (ETDEWEB)

    Rundback, J.H.; Goldfarb, C.R.; Ongseng, F. (Beth Israel Medical Center, New York, NY (USA))

    1990-01-01

    Ga-67 scanning has been used to evaluate esophageal carcinoma. It has demonstrated candidal infection in other body sites and, in one previous case, in the esophagus. The authors present a case of diffuse esophageal uptake of Ga-67 in esophageal candidiasis.

  14. Long -term outcomes of oesophageal atresia without or with proximal tracheooesophageal fistula - Gross types A and B.

    Science.gov (United States)

    Koivusalo, A I; Sistonen, S J; Lindahl, H G; Rintala, R J; Pakarinen, M P

    2017-05-01

    Because of an extended gap between esophageal pouches a variety of methods are employed to treat oesophageal atresia (OA) without (type A) or with (type B) proximal tracheooesophageal fistula. This retrospective observational study describes their single centre long-term outcomes from 1947 to 2014. Of 693 patients treated for OA 68 (9.7%) had type A (n=58, 8.3%) or B (n=10, 1.4%). Hospital records were reviewed. Main outcome measures were survival and oral intake. Nine (13%) patients had early and 10 (15%) delayed primary anastomosis, 30 (44%) underwent reconstruction including colonic interposition (n=13), reversed gastric tube (n=11) and jejunum interposition (n=6), whereas19 (28%) had died without a definite repair. Median follow up was 35 (interquartile range, 7.4-40) years. Thirty-one (63%) of 49 patients with definitive repair survived long term. Survival was 22% for early and 80% for delayed primary anastomosis, 57% for colon interposition, 82% for gastric tube and 84% for jejunum interposition. Gastrooesophageal reflux was most common after gastric tube (80%), dysphagia after colon interposition (50%), and 3 (60%) of 5 survivors with jejunum interposition had permanent feeding ostomy because of neurological disorder. Endoscopic follow-up disclosed no oesophageal cancer or dysplasia. Repair in the most recent patients from 1985 to 2014 (n=14) included delayed primary anastomosis (n=7), jejunum interposition (n=6) and gastric tube (n=1) with 93% long-term survival. Morbidity among long-term survivors of type A or B OA is high. With modern management survival is, however, excellent and patients without neurological disorder achieve full oral intake either after primary anastomosis or reconstruction. IV. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. [Esophageal diseases: GERD, Barrett, achalasia and eosinophilic esophagitis].

    Science.gov (United States)

    Calvet, Xavier; Villoria, Albert

    2014-09-01

    At Digestive Disease Week (DDW) 2014, developments in esophageal disease were presented. Highlights include: the usefulness of impedancemetry to diagnose reflux disease, or the effectiveness of PPIs for treating non-cardiac chest pain. Concerning Barrett's esophagus, its prevalence is identical in patients with and without reflux symptoms, Barrett segments less than 1cm probably do not require follow-up, and in older patients with long-segment Barrett, initial endoscopies overlooked up to 2% of significant lesions. Regarding achalasia, surgical myotomy is no more effective than endoscopic dilation and may even be less effective than peroral endoscopic myotomy (POEM). In terms of eosinophilic esophagitis, it is important to systematically take biopsies in patients with dysphagia so that cases of eosinophilic esophagitis are not overlooked. In addition, for this condition, routine endoscopic dilations not only do not seem useful in improving the course of the disease, but could also worsen the response to medical treatment.

  16. Esophageal tissue engineering: A new approach for esophageal replacement

    Institute of Scientific and Technical Information of China (English)

    Giorgia Totonelli; Panagiotis Maghsoudlou; Jonathan M Fishman; Giuseppe Orlando; Tahera Ansari; Paul Sibbons; Martin A Birchall

    2012-01-01

    A number of congenital and acquired disorders require esophageal tissue replacement.Various surgical techniques,such as gastric and colonic interposition,are standards of treatment,but frequently complicated by stenosis and other problems.Regenerative medicine approaches facilitate the use of biological constructs to replace or regenerate normal tissue function.We review the literature of esophageal tissue engineering,discuss its implications,compare the methodologies that have been employed and suggest possible directions for the future.Medline,Embase,the Cochrane Library,National Research Register and ClinicalTrials.gov databases were searched with the following search terms:stem cell and esophagus,esophageal replacement,esophageal tissue engineering,esophageal substitution.Reference lists of papers identified were also examined and experts in this field contacted for further information.All full-text articles in English of all potentially relevant abstracts were reviewed.Tissue engineering has involved acellular scaffolds that were either transplanted with the aim of being repopulated by host cells or seeded prior to transplantation.When acellular scaffolds were used to replace patch and short tubular defects they allowed epithelial and partial muscular migration whereas when employed for long tubular defects the results were poor leading to an increased rate of stenosis and mortality.Stenting has been shown as an effective means to reduce stenotic changes and promote cell migration,whilst omental wrapping to induce vascularization of the construct has an uncertain benefit.Decellularized matrices have been recently suggested as the optimal choice for scaffolds,but smart polymers that will incorporate signalling to promote cell-scaffold interaction may provide a more reproducible and available solution.Results in animal models that have used seeded scaffolds strongly suggest that seeding of both muscle and epithelial cells on scaffolds prior to implantation is a

  17. Reflux esophagitis revisited: Prospective analysis of radiologic accuracy

    Energy Technology Data Exchange (ETDEWEB)

    Ott, D.J.; Gelfand, D.W.; Wu, W.C.

    1981-01-15

    A prospective radiologic-endoscopic study of the esophagogastric region in 266 patients, including 206 normals and 60 with esophagitis, is reported. The endoscopic classification grading severity of esophagitis was grade 1-normal; grades 2. 3. and 4-mild, moderate, and severe esophagitis, respectively. Radiology detected 22% of patient with mild esophagitis, 83% with moderate esophagitis, and 95% with severe esophagitis. Although hiatal hernia was present in 40% of normals and 89% with esophagitis, absence of radiographic hiatal hernia excluded esophagitis with 95% accuracy. The implications of this study regarding the role of radiology in evaluating patient with suspected reflux esophagitis are discussed.

  18. Ultrasound-guided percutaneous cholecysto-cholangiography for the exclusion of biliary atresia in infants

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Kyung Min; Ryeom, Hun Kyu; Choe, Byung Ho; Kim, Kap Cheol; Kim, Jong Yeol; Lee, Jong Min; Kim, Hye Jeong; Lee, Hee Jung [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    2006-08-15

    The aim of this study is to determine the feasibility and effectiveness of performing an ultrasound-guided percutaneous cholecysto-cholangiogram (PCC) for excluding biliary atresia as the cause of neonatal jaundice. Between Oct. 2003 and Feb. 2005, six ultrasound-guided PCC procedures were performed to five jaundiced infants (4 females and 1 male; mean age: 60 days old) for whom possibility of biliary atresia could not be ruled out by the DISIDA scan as the cause of their neonatal jaundice. Gallbladder puncture was performed under ultrasound guidance with a 23-gauge needle. Contrast material injection during fluoroscopic examination was performed after dilatation of the gallbladder lumen with normal saline under ultrasound guidance. The criteria used for excluding biliary atresia were complete visualization of the extrahepatic biliary trees and/or contrast excretion into the duodenum. The complications and final diagnosis was assessed according to the clinical and laboratory findings. The procedures were successful in all the patients without any complication. Biliary atresia could be ruled out in all the patients. The final diagnosis was neonatal cytomegalovirus hepatitis in two patients, total parenteral nutrition-associated cholestasis in two patients, and combined cytomegalovirus hepatitis and total parenteral nutrition-associated cholestasis in one patient. Ultrasound-guided PCC is a feasible and effective method for the early definitive exclusion of biliary atresia as the cause of neonatal jaundice. By the technique of injecting normal saline before contrast injection, PCC can be done even in a totally collapsed or very small gallbladder.

  19. Association of Nasal Nostril Stenosis with Bilateral Choanal Atresia: A Case Report

    Directory of Open Access Journals (Sweden)

    Shahin Abdollahifakhim

    2014-01-01

    Full Text Available Introduction: Neonatal nasal airway obstruction induces various degrees of respiratory distress. The management of this disease, including surgical repair, will depend on the severity and location of the obstruction. We describe here a case of congenital nasal nostril stenosis that required surgical repair for stenting of both nares after coanal atresia repair.   Case Report: A 2 days old female newborn referred to neonatal department of Tabriz Children’s Hospital affiliated to the University of Medical Sciences of Tabriz, Iran on the 3rd of December, 2011 immediately after birth with respiratory distress due to bilateral coanal atresia and nasal hypoplasia with very small nostrils. CT scan showed normal brain and bilateral choanal atresia with normal size Pyriform apertures.   Conclusion: Nasal obstruction can lead to airway compromise and respiratory distress. Congenital bony nasal deformities are being recognized as an important cause of newborn airway obstruction. Nasal hypoplasia is seen in many craniofacial syndromes. Although our patient had hypoplastic nostrils with respiratory distress due to bilateral coanal atresia, correction of hypoplastic nostrils was necessary for completing the operation of choanal atresia.

  20. Immediate primary anastomosis for isolated oesophageal atresia: A single-centre experience

    Directory of Open Access Journals (Sweden)

    Ibrahim Uygun

    2015-01-01

    Full Text Available Background: Isolated oesophageal atresia without tracheo-oesophageal fistula represents a major challenge for most paediatric surgeons. Here, we present our experience with six neonates with isolated oesophageal atresia who successfully underwent immediate primary anastomosis using multiple Livaditis circular myotomy. Materials and Methods: All six neonates were gross type A isolated oesophageal atresia (6%, from among 102 neonates with oesophageal atresia, treated between January 2009 and December 2013. Five neonates were female; one was male. The mean birth weight was 2300 (range 1700-3100 g. Results: All six neonates successfully underwent immediate primary anastomosis using multiple myotomies (mean 3; range 2-4 within 10 (median 3 days after birth. The gap under traction ranged from 6 to 7 cm. One neonate died of a major cardiac anomaly. Another neonate was lost to follow-up after being well for 3 months. Three anastomotic strictures were treated with balloon dilatation, and four anastomotic leaks were treated conservatively. The mean duration of follow-up was 33 months. Conclusions: To treat isolated oesophageal atresia, an immediate primary anastomosis can be achieved using multiple myotomies. Although, this approach is associated with high complication rates, as are other similar approaches, these complications can be overcome.

  1. The Sea Lamprey as an Etiological Model for Biliary Atresia

    Directory of Open Access Journals (Sweden)

    Yu-Wen Chung-Davidson

    2015-01-01

    Full Text Available Biliary atresia (BA is a progressive, inflammatory, and fibrosclerosing cholangiopathy in infants that results in obstruction of both extrahepatic and intrahepatic bile ducts. It is the most common cause for pediatric liver transplantation. In contrast, the sea lamprey undergoes developmental BA with transient cholestasis and fibrosis during metamorphosis, but emerges as a fecund adult with steatohepatitis and fibrosis in the liver. In this paper, we present new histological evidence and compare the sea lamprey to existing animal models to highlight the advantages and possible limitations of using the sea lamprey to study the etiology and compensatory mechanisms of BA and other liver diseases. Understanding the signaling factors and genetic networks underlying lamprey BA can provide insights into BA etiology and possible targets to prevent biliary degeneration and to clear fibrosis. In addition, information from lamprey BA can be used to develop adjunct treatments for patients awaiting or receiving surgical treatments. Furthermore, the cholestatic adult lamprey has unique adaptive mechanisms that can be used to explore potential treatments for cholestasis and nonalcoholic steatohepatitis (NASH.

  2. Measurement of Gastric Circumference in Foetuses with Oesophageal Atresia.

    Science.gov (United States)

    Hoopmann, M; Kagan, K O; Borgmeier, F; Seitz, G; Arand, J; Wagner, P

    2015-11-01

    Background: The specific recognition of oesophageal atresia (OA) with or without a tracheal fistula in a foetus is a diagnostic challenge for prenatal medicine. The aim of the present work is to analyse the value of the measurement of gastric size in the diagnosis of this significant malformation. Materials and Methods: Altogether, the examinations of 433 pregnancies between the 18.4 and 39.1 weeks of gestation were retrospectively analysed. 59 of these foetuses exhibited an OA. By means of a linear regression analysis with normal foetuses, significant parameters influencing gastric size were examined. Subsequently the gastric sizes were transformed into z values and a comparison was made between OA with and without fistulae with the help of t tests. Results: In the normal foetuses there was a significant association between the gastric circumference and the abdominal circumference (circumference = 6.809 + 0.179 × abdominal circumference, r = 0.686, p values in the normal group and in the groups of OA with fistula and without fistula amounted to 0.0 (SD 1.0), -1.3 (SD 2.2) and -4.5 (SD 1.0). Conclusion: Measurements of the gastric circumference below the 5th percentile should lead to further diagnostic measures, especially when associated with polyhydramnios. Although OA without a fistula is always conspicuous, only about one in three OAs with fistula are associated with a significantly smaller stomach.

  3. Maternal microchimerism in the livers of patients with Biliary atresia

    Directory of Open Access Journals (Sweden)

    Magrane Greg

    2004-07-01

    Full Text Available Abstract Background Biliary atresia (BA is a neonatal cholestatic disease of unknown etiology. It is the leading cause of liver transplantation in children. Many similarities exist between BA and graft versus host disease suggesting engraftment of maternal cells during gestation could result in immune responses that lead to BA. The aim of this study was to determine the presence and extent of maternal microchimerism (MM in the livers of infants with BA. Methods Using fluorescent in situ hybridization (FISH, 11 male BA & 4 male neonatal hepatitis (NH livers, which served as controls, were analyzed for X and Y-chromosomes. To further investigate MM in BA, 3 patients with BA, and their mothers, were HLA typed. Using immunohistochemical stains, the BA livers were examined for MM. Four additional BA livers underwent analysis by polymerase chain reaction (PCR for evidence of MM. Results By FISH, 8 BA and 2 NH livers were interpretable. Seven of eight BA specimens showed evidence of MM. The number of maternal cells ranged from 2–4 maternal cells per biopsy slide. Neither NH specimen showed evidence of MM. In addition, immunohistochemical stains confirmed evidence of MM. Using PCR, a range of 1–142 copies of maternal DNA per 25,000 copies of patients DNA was found. Conclusions Maternal microchimerism is present in the livers of patients with BA and may contribute to the pathogenesis of BA.

  4. Decreased mortality but increased morbidity in neonates with jejunoileal atresia; a study of 114 cases over a 34-year period.

    NARCIS (Netherlands)

    Stollman, T.H.; Blaauw, I. de; Wijnen, M.H.W.A.; Staak, F.H.J.M. van der; Rieu, P.N.M.A.; Draaisma, J.M.T.; Wijnen, R.M.H.

    2009-01-01

    PURPOSE: The aim of the study was to evaluate patient demographics, classification and location of the atresia, operative management, postoperative care, and outcome in 114 infants with jejunoileal atresia (JIA) over a period of more than 3 decades. METHODS: This was a retrospective case series in a

  5. Prevention of vitamin K deficiency bleeding in breastfed infants: lessons from the Dutch and Danish biliary atresia registries

    DEFF Research Database (Denmark)

    Hasselt, P.M. van; Koning, T.J. de; Vries, E. de;

    2008-01-01

    in infants with biliary atresia. PATIENTS AND METHODS: From Dutch and Danish national biliary atresia registries, we retrieved infants who were either breastfed and received 1 mg of oral vitamin K at birth followed by 25 microg of daily oral vitamin K prophylaxis (Netherlands, 1991-2003), 2 mg of oral...

  6. Efficacy of Intensity Modulated Radiation Therapy After Surgery in Early Stage of Esophageal Carcinoma;

    Science.gov (United States)

    2016-07-30

    Esophageal Neoplasm; Esophageal Cancer TNM Staging Primary Tumor (T) T2; Esophageal Cancer TNM Staging Primary Tumor (T) T3; Esophageal Cancer TNM Staging Regional Lymph Nodes (N) N0; Esophageal Cancer TNM Staging Distal Metastasis (M) M0

  7. Sudden fetal death associated with both duodenal atresia and umbilical cord ulcer: a case report and review.

    Science.gov (United States)

    Anami, Ai; Morokuma, Seiichi; Tsukimori, Kiyomi; Kondo, Haruhiko; Nozaki, Masahiro; Sueishi, Katsuo; Nakano, Hitoo

    2006-04-01

    We encountered one case of duodenal atresia complicated by massive intrauterine hemorrhage due to the perforation of an umbilical cord ulceration (UCU). UCU is an extremely rare complication in the perinatal period. Although the prenatal diagnosis of upper intestinal atresia has been established, little is known about the association between UCU and upper intestinal atresia. In this article, we report our case, review past articles, and discuss the underlying pathophysiological mechanisms of the cause of an UCU. Given the characteristic sites of upper intestinal atresia, we speculate that regurgitation of gastric or intestinal juice into the amniotic fluid could be responsible for the development of UCU. We also believe that close observation is required for patients who have upper intestinal atresia.

  8. Methoxychlor induces atresia by altering Bcl2 factors and inducing caspase activity in mouse ovarian antral follicles in vitro.

    Science.gov (United States)

    Basavarajappa, Mallikarjuna S; Karman, Bethany N; Wang, Wei; Gupta, Rupesh K; Flaws, Jodi A

    2012-12-01

    Methoxychlor (MXC) is an organochlorine pesticide widely used in many countries against various species of insects that attack crops and domestic animals. MXC reduces fertility by increasing atresia (death) of antral follicles in vivo. MXC also induces atresia of antral follicles after 96 h in vitro. The current work tested the hypothesis that MXC induces morphological atresia at early time points (24 and 48 h) by altering pro-apoptotic (Bax, Bok, Casp3, and caspase activity) and anti-apoptotic (Bcl2 and Bcl-xL) factors in the follicles. The results indicate that at 24 h, MXC increased Bcl-xL and Bax mRNA levels and increased the ratio of Bax/Bcl2. At 48-96 h, MXC induced morphological atresia. At 24-96 h, MXC increased caspase activities. These data suggest that MXC may induce atresia by altering Bcl2 factors and inducing caspase activities in antral follicles.

  9. Simultaneous vibrant soundbridge implantation and 2nd stage auricular reconstruction for microtia with aural atresia

    Directory of Open Access Journals (Sweden)

    Lynne Hsueh Yee Lim

    2011-09-01

    Full Text Available Aural atresia and severe microtia are associated malformations that result in problems with hearing and cosmesis, associated speech and language difficulties and diminished self-esteem. In cases where middle ear ossiculoplasty and aural atresia canalplasty are expected to give poor hearing outcomes that would eventually require the use of hearing aids, bone anchored hearing aids or active middle ear implants may be better options. This case report describes a simultaneous Vibrant Soundbridge implantation and 2nd stage auricular reconstruction with rib graft cartilage for an 11-year-old boy with grade III microtia and aural atresia 8 months after the 1st stage reconstruction. Audiometric results of the Vibrant Soundbridge aided ear were comparable to that of the contralateral hearing aid aided ear.

  10. Simultaneous Vibrant Soundbridge Implantation and 2nd Stage Auricular Reconstruction for Microtia with Aural Atresia.

    Science.gov (United States)

    Lim, Lynne Hsueh Yee; Xiang, Ling; Del Prado, Jocelynne; Ee Chin, Ling; Beltrame, Millo Achille

    2011-07-01

    Aural atresia and severe microtia are associated malformations that result in problems with hearing and cosmesis, associated speech and language difficulties and diminished self-esteem. In cases where middle ear ossiculoplasty and aural atresia canalplasty are expected to give poor hearing outcomes that would eventually require the use of hearing aids, bone anchored hearing aids or active middle ear implants may be better options. This case report describes a simultaneous Vibrant Soundbridge implantation and 2(nd) stage auricular reconstruction with rib graft cartilage for an 11-year-old boy with grade III microtia and aural atresia 8 months after the 1(st) stage reconstruction. Audiometric results of the Vibrant Soundbridge aided ear were comparable to that of the contralateral hearing aid aided ear.

  11. Anaesthetic Management of a Patient with Synchronous Kartagener Syndrome and Biliary Atresia.

    Science.gov (United States)

    Kendigelen, Pınar; Tütüncü, Ayşe Çiğdem; Erbabacan, Şafak Emre; Kaya, Güner; Altındaş, Fatiş

    2015-06-01

    Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated.

  12. Diagnosis of osteopetrosis in bilateral congenital aural atresia: Turning point in treatment strategy

    Science.gov (United States)

    Verma, Ritu; Jana, Manisha; Bhalla, Ashu Seith; Kumar, Arvind; Kumar, Rakesh

    2016-01-01

    Aural atresia is a rare congenital malformation of the external and middle ear. There are several syndromic associations of this anomaly with those involving the first and second branchial arches. Occurrence of aural atresia with sclerosing skeletal dysplasia is unknown and has never been reported. The co-existence of a sclerosing dysplasia can make the surgical treatment in aural atresia difficult and risky; and the auditory improvement may not be as expected. Moreover, internal auditory canal narrowing and hence sensorineural hearing loss in sclerosing dysplasia might add to the already existing conductive hearing loss in such patients. In this case report we have described an unknown association of bilateral microtia with sclerosing skeletal dysplasia (autosomal dominant osteopetrosis) and clinical implications of these two conditions occurring together leading to a change in the management plan. PMID:27170934

  13. Distal esophageal spasm: an update.

    Science.gov (United States)

    Achem, Sami R; Gerson, Lauren B

    2013-09-01

    Distal esophageal spasm (DES) is an esophageal motility disorder that presents clinically with chest pain and/or dysphagia and is defined manometrically as simultaneous contractions in the distal (smooth muscle) esophagus in ≥20% of wet swallows (and amplitude contraction of ≥30 mmHg) alternating with normal peristalsis. With the introduction of high resolution esophageal pressure topography (EPT) in 2000, the definition of DES was modified. The Chicago classification proposed that the defining criteria for DES using EPT should be the presence of at least two premature contractions (distal latencylong acting), calcium-channel blockers, anticholinergic agents, 5-phosphodiesterase inhibitors, visceral analgesics (tricyclic agents or SSRI), and esophageal dilation. Acid suppression therapy is frequently used, but clinical outcome trials to support this approach are not available. Injection of botulinum toxin in the distal esophagus may be effective, but further data regarding the development of post-injection gastroesophageal reflux need to be assessed. Heller myotomy combined with fundoplication remains an alternative for the rare refractory patient. Preliminary studies suggest that the newly developed endoscopic technique of per oral endoscopic myotomy (POEM) may also be an alternative treatment modality.

  14. Esophageal Cancer Risk Prediction Models

    Science.gov (United States)

    Developing statistical models that estimate the probability of developing esophageal cancer over a defined period of time will help clinicians identify individuals at higher risk of specific cancers, allowing for earlier or more frequent screening and counseling of behavioral changes to decrease risk.

  15. Brain Abscess after Esophageal Dilatation

    DEFF Research Database (Denmark)

    Gaïni, S; Grand, M; Michelsen, J

    2007-01-01

    with malaise, progressive lethargy, fever, aphasia and hemiparesis. Six days before she had been treated with esophageal dilatation for a stricture caused by accidental ingestion of caustic soda. The brain abscess was treated with surgery and antibiotics. She recovered completely. This clinical case...

  16. Molecular Biology of Esophageal Cancer

    Institute of Scientific and Technical Information of China (English)

    HuanXi; JanBrabender; RalfMetzger; PaulM.Schneider

    2004-01-01

    There have been many new developments in our understanding of esophageal carcinoma biology over the past several years. Information regarding both of the major forms of this disease, adenocarcinoma and squamous cell carcinoma, has accumulated in conjunction with data on precursor conditions such as Barrett's esophagus. Interesting and promising findings have included overexpression of proto-oncogenes,loss of heterozygosity at multiple chromosomal loci, tumor suppressor gene inactivation, epigenetic silencing by DNA methylation, and mutations and deletions involving the tumor suppressor gene p53. Important cancer pathways, the cyclin kinase inhibitor cascade and the DNA mismatch repair process, implicated in the genesis of multiple tumor types have also been inculpated in esophageal carcinogenesis. Alterations in the p16 and p15 cyclin kinase inhibitors including point mutations and homozygous deletions have been reported in primary esophageal tumors. Further developments in the field of molecular carcinogenesis of esophageal malignancies promise to yield improvements in prevention, early detection, prognostic categorization, and perhaps gene-based therapy of this deadly disease.

  17. microRNA-222 modulates liver fibrosis in a murine model of biliary atresia

    Energy Technology Data Exchange (ETDEWEB)

    Shen, Wen-jun; Dong, Rui; Chen, Gong, E-mail: chengongzlp@hotmail.com; Zheng, Shan

    2014-03-28

    Highlights: • The RRV infected group showed cholestasis, retardation and extrahepatic biliary atresia. • miR-222 was highly expressed, and PPP2R2A was inhibited in the murine biliary atresia model. • miR-222 profoundly modulated the process of fibrosis in the murine biliary atresia model. • miR-222 might represent a potential target for improving biliary atresia prognosis. - Abstract: microRNA-222 (miR-222) has been shown to initiate the activation of hepatic stellate cells, which plays an important role in the pathogenesis of liver fibrosis. The aim of our study was to evaluate the role of miR-22 in a mouse model of biliary atresia (BA) induced by Rhesus Rotavirus (RRV) infection. New-born Balb/c mice were randomized into control and RRV infected groups. The extrahepatic bile ducts were evaluated. The experimental group was divided into BA group and negative group based on histology. The expression of miR-222, protein phosphatase 2 regulatory subunit B alpha (PPP2R2A), proliferating cell nuclear antigen (PCNA) and phospho-Akt were detected. We found that the experimental group showed signs of cholestasis, retardation and extrahepatic biliary atresia. No abnormalities were found in the control group. In the BA group, miR-222, PCNA and Akt were highly expressed, and PPP2R2A expression was significantly inhibited. Our findings suggest that miR-222 profoundly modulated the process of fibrosis in the murine BA model, which might represent a potential target for improving BA prognosis.

  18. Methoxychlor inhibits growth and induces atresia of antral follicles through an oxidative stress pathway.

    Science.gov (United States)

    Gupta, Rupesh K; Miller, Kimberly P; Babus, Janice K; Flaws, Jodi A

    2006-10-01

    The mammalian ovary contains antral follicles, which are responsible for the synthesis and secretion of hormones that regulate estrous cyclicity and fertility. The organochlorine pesticide methoxychlor (MXC) causes atresia (follicle death via apoptosis) of antral follicles, but little is known about the mechanisms by which MXC does so. Oxidative stress is known to cause apoptosis in nonreproductive and reproductive tissues. Thus, we tested the hypothesis that MXC inhibits growth and induces atresia of antral follicles through an oxidative stress pathway. To test this hypothesis, antral follicles isolated from 39-day-old CD-1 mice were cultured with vehicle control (dimethylsulfoxide [DMSO]), MXC (1-100 microg/ml), or MXC + the antioxidant N-acetyl cysteine (NAC) (0.1-10 mM). During culture, growth was monitored daily. At the end of culture, follicles were processed for quantitative real-time polymerase chain reaction of Cu/Zn superoxide dismutase (SOD1), glutathione peroxidase (GPX), and catalase (CAT) mRNA expression or for histological evaluation of atresia. The results indicate that exposure to MXC (1-100 microg/ml) inhibited growth of follicles compared to DMSO controls and that NAC (1-10 mM) blocked the ability of MXC to inhibit growth. MXC induced follicular atresia, whereas NAC (1-10 mM) blocked the ability of MXC to induce atresia. In addition, MXC reduced the expression of SOD1, GPX, and CAT, whereas NAC reduced the effects of MXC on their expression. Collectively, these data indicate MXC causes slow growth and increased atresia by inducing oxidative stress.

  19. Congenital external auditory canal atresia and stenosis: temporal bone CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Hoon; Kim, Bum Soo; Jung, So Lyung; Kim, Young Joo; Chun, Ho Jong; Choi, Kyu Ho; Park, Shi Nae [College of Medicine, Catholic Univ. of Korea, Seoul (Korea, Republic of)

    2002-04-01

    To determine the computed tomographic (CT) findings of atresia and stenosis of the external auditory canal (EAC), and to describe associated abnormalities in surrounding structures. We retrospectively reviewed the axial and coronal CT images of the temporal bone in 15 patients (M:F=8:7;mean age, 15.8 years) with 16 cases of EAC atresia (unilateral n=11, bilateral n=1) and EAC stenosis (unilateral n=3). Associated abnormalities of the EAC, tympanic cavity, ossicles, mastoid air cells, eustachian tube, facial nerve course, mandibular condyle and condylar fossa, sigmoid sinus and jugular bulb, and the base of the middle cranial fossa were evaluated. Thirteen cases of bony EAC atresia (one bilateral), with an atretic bony plate, were noted, and one case of unilateral membranous atresia, in which a soft tissue the EAC. A unilateral lesion occurred more frequently on the right temporal bone (n=8, 73%). Associated abnormalities included a small tympanic cavity (n=8, 62%), decreased mastoid pneumatization (n=8, 62%), displacement of the mandibular condyle and the posterior wall of the condylar fossa (n=7, 54%), dilatation of the Eustachian tube (n=7, 54%), and inferior displacement of the temporal fossa base (n=8, 62%). Abnormalities of ossicles were noted in the malleolus (n=12, 92%), incus (n=10, 77%) and stapes (n=6, 46%). The course of the facial nerve was abnormal in four cases, and abnormality of the auditory canal was noted in one. Among three cases of EAC stenosis, ossicular aplasia was observed in one, and in another the location of the mandibular condyle and condylar fossa was abnormal. In the remaining case there was no associated abnormality. Atresia of the EAC is frequently accompanied by abnormalities of the middle ear cavity, ossicles, and adjacent structures other than the inner ear. For patients with atresia and stenosis of this canal, CT of the temporal bone is essentially helpful in evaluating these associated abnormalities.

  20. VACTERL (vertebral anomalies, anal atresia or imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal and limb defect spectrum presenting with portal hypertension: a case report

    Directory of Open Access Journals (Sweden)

    Losa Ignatius

    2010-05-01

    Full Text Available Abstract Introduction We report for the first time a unique case of VACTERL (vertebral anomalies, anal atresia or imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal and limb defect spectrum associated with portal hypertension. The occurrence of both VACTERL spectrum and extrahepatic portal hypertension in a patient has not been reported in the literature. We examined whether or not there was any association between extrahepatic portal hypertension and VACTERL spectrum. Case Presentation A two-and-half-year-old Caucasian girl with VACTERL spectrum presented with hematemesis and abdominal distension. She had caput medusae, ascites, splenomegaly, gastric and esophageal varices. Her liver function tests were within normal limits. Magnetic resonance imaging of the liver with contrast showed a thready portal vein with collateral vessels involving both right and left portal veins without intrahepatic duct dilation. Conclusion A thready portal vein, with features of extrahepatic portal hypertension, is a rare non- VACTERL-type defect in patients with VACTERL spectrum. Understandably, clinicians should give low priority to looking for portal hypertension in VACTERL spectrum patients presenting with gastrointestinal bleeding. However before routinely looking for a thready portal vein and/or extrahepatic portal hypertension in asymptomatic VACTERL spectrum patients, we need further evidence to support this rare association.

  1. Radionuclide Esophageal Transit Scintigraphy in Primary Hypothyroidism

    Science.gov (United States)

    Khan, Shoukat H; Madhu, Vijay P; Rather, Tanveer A; Laway, Bashir A

    2017-01-01

    Background/Aims Esophageal dysmotility is associated with gastrointestinal dysmotility in various systemic and neuroregulatory disorders. Hypothyroidism has been reported to be associated with impaired motor function in esophagus due to accumulation of glycosaminoglycan hyaluronic acid in its soft tissues, leading to changes in various contraction and relaxation parameters of esophagus, particularly in the lower esophageal sphincter. In this study we evaluated esophageal transit times in patients of primary hypothyroidism using the technique of radionuclide esophageal transit scintigraphy. Methods Thirty-one patients of primary hypothyroidism and 15 euthyroid healthy controls were evaluated for esophageal transit time using 15–20 MBq of Technetium-99m sulfur colloid diluted in 10–15 mL of drinking water. Time activity curve was generated for each study and esophageal transit time was calculated as time taken for clearance of 90% radioactive bolus from the region of interest encompassing the esophagus. Esophageal transit time of more than 10 seconds was considered as prolonged. Results Patients of primary hypothyroidism had a significantly increased mean esophageal transit time of 19.35 ± 20.02 seconds in comparison to the mean time of 8.25 ± 1.71 seconds in healthy controls (P < 0.05). Esophageal transit time improved and in some patients even normalized after treatment with thyroxine. A positive correlation (r = 0.39, P < 0.05) albeit weak existed between the serum thyroid stimulating hormone and the observed esophageal transit time. Conclusions A significant number of patients with primary hypothyroidism may have subclinical esophageal dysmotility with prolonged esophageal transit time which can be reversible by thyroxine treatment. Prolonged esophageal transit time in primary hypothyroidism may correlate with serum thyroid stimulating hormone levels. PMID:27444283

  2. Clinical Study of Time Optimizing of Endoscopic Photodynamic Therapy on Esophageal and/or Gastric Cardiac Cancer

    Science.gov (United States)

    2015-12-10

    Stage I Esophageal Adenocarcinoma; Stage II Esophageal Adenocarcinoma; Stage III Esophageal Adenocarcinoma; Stage I Esophageal Squamous Cell Carcinoma; Stage II Esophageal Squamous Cell Carcinoma; Stage III Esophageal Squamous Cell Carcinoma

  3. Atresia de coanas, revisión y una mirada desde la evidencia

    OpenAIRE

    2014-01-01

    La atresia de coanas es la anomalía congénita nasal más común. Cuando es bilateral, se presenta con dificultad respiratoria desde el nacimiento. La atresia unilateral se manifiesta con insuficiencia ventilatoria y rinorrea unilateral, pudiendo pasar inadvertida. El diagnóstico se sospecha ante la ausencia de paso de aire en las fosas nasales y la imposibilidad de hacer progresar una sonda nasogástrica. Se confirma mediante examen endoscópico y tomografía computarizada. El tratamiento definiti...

  4. Reverse gastric tube oesophageal substitution for staged repair of oesophageal atresia and tracheo-oesophageal fistula

    Directory of Open Access Journals (Sweden)

    Christopher Olusanjo Bode

    2014-01-01

    Full Text Available The management of oesophageal atresia and tracheo-oesophageal atresia (OATOF is very challenging. While in developed countries survival of patients with this condition has improved, the outcome in many developing countries has been poor. Primary repair through a thoracotomy (or video-assisted thoracoscopic surgery where available is the gold standard treatment of OATOF. However, in our setting where patients typically present late and with minimum support resources such as Neonatal Intensive Care Unit and total parenteral nutrition; staged repair may be the only hope of survival of these patients and this communication highlights the essential steps of this mode of treatment.

  5. BICORNUATE UTERUS WITH CERVICAL ATRESIA AND VAGINAL AGENESIS ASSOCIATED WITH OVARIAN ENDOMETRIOSIS - A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Alpana

    2013-06-01

    Full Text Available ABSTRACT: Vaginal agenesis combined with a functional uterus is a rare type of Mullerian duct an omaly. Only 7 – 8% of patients with vaginal agenesis have a functional uterus. Women born with vaginal agenesis, cervical atresia combined with a functioning endometrium typically present with hematometra , disabling pelvic pain and progressively worsening en dometriosis. Almost all need an abdominal hysterectomy for relief. We report a case of severe endometriosis in a 29 years old woman having a bicornuate uterus with cervical atresia and vaginal agenesis.

  6. Prenatal Diagnosis and Pathology of Laryngeal Atresia in Congenital High Airway Obstruction Syndrome

    Directory of Open Access Journals (Sweden)

    Piya Chaemsaithong

    2012-01-01

    Full Text Available Congenital high airway obstruction syndrome is a rare but life-threatening condition. Therefore, prenatal diagnosis is important. The obstruction can be due to laryngeal/tracheal atresia or external compression. While a differential diagnosis with congenital cystic adenomatoid malformation (CCAM type III may be difficult, it is still possible with ultrasonography. In this study, we report a case of bilateral echogenic lungs with hydrops fetalis. After the prenatal diagnosis of laryngeal atresia, the couple opted to have an elective termination of pregnancy performed at 20 weeks of gestation. The diagnosis was confirmed by a complete pathological examination.

  7. Newborn with congenital facial palsy and bilateral anotia/atresia of external auditory canal: Rare occurrence

    Science.gov (United States)

    Mahale, Rohan R.; Mehta, Anish; John, Aju Abraham; Buddaraju, Kiran; Shankar, Abhinandan K.; Rangasetty, Srinivasa

    2016-01-01

    Congenital facial palsy (CFP) is clinically defined as facial palsy of the seventh cranial nerve which is present at birth or shortly thereafter. It is generally considered to be either developmental or acquired in origin. Facial palsy of developmental origin is associated with other anomalies including those of pinna and external auditory canal, which range from mild defects to severe microtia and atresia. We report a 2-day-old male newborn that had right CFP with bilateral anotia and atresia of external auditory canals which is rare. PMID:27857806

  8. Successful pregnancy by IVF in a patient with congenital cervical atresia

    Institute of Scientific and Technical Information of China (English)

    Achour Radhouane; Basly Mohamed; Ben Aissa Imen; Ferjaoui Aymen; NEJI Khaled

    2015-01-01

    Congenital cervical atresia and hypoplasia are rare abnormalities that generally require reconstructive or extirpative procedures to relieve outflow tract obstruction. Infertility is a common sequel, and only four previous pregnancies have been reported. We report a case of successful pregnancy afterin-vitro fertilization in a 32-year-old patient with congenital cervical atresia diagnosed at the age of 28 years. She was referred to our unit and had a succeful pregnancy afterin-vitro fertilization. Caesarean section was perfomed at 38 weeks gestation. A healthy male baby weighing 3 650 g was safely delivered.

  9. Atresia de la vena porta en un canino : Reporte de un caso

    OpenAIRE

    Allende, Miriam G.; Acosta, Walter; Baschar, H. A.; Massone, Adriana R.; Diez, M. L.; Amo, Alicia N. del

    2006-01-01

    La atresia vascular portal es una anomalía congénita poco frecuente en los caninos. Los shunts porto sistémicos (SPS) son comunicaciones entre la vena porta y el sistema venoso, de manera que la sangre portal pasa a la circulación general sin ingresar al hígado. Los mismos pueden ser congénitos o adquiridos y presentar una ubicación intra o extra hepática. En asociación a la atresia portal se han hallado shunts simples congénitos y múltiples adquiridos. El presente trabajo describe la present...

  10. [Nasal breath recovery and rhinoplasty in cleft lip and palate patient with unilateral choanal atresia].

    Science.gov (United States)

    Chkadua, T Z; Ivanova, M D; Daminov, R O; Brusova, L A; Savvateeva, D M

    2016-01-01

    The paper presents the analysis of clinical case of endoscopic nasal breath restoration and elimination of the secondary cleft lip nasal deformity in 27 years old patient with unilateral choanal atresia and secondary nasal deformity after rhinocheiloplasty. Preoperative examination revealed the absence of nasal breathing on collateral side due to complete bone choanal atresia. Surgical treatment included endoscopic choanal repair, elimination of the secondary nasal deformity, septoplasty, conchotomy and lateroposition of the inferior conchae. The treatment resulted in nasal breath restoration and elimination of nasal deformity. Long-term follow-up at 1 and 12 months post-operatively proved stable positive aesthetic and functional results.

  11. Laparoscopic duodenoduodenostomy with parallel anastomosis for duodenal atresia.

    Science.gov (United States)

    Oh, Chaeyoun; Lee, Sanghoon; Lee, Suk-Koo; Seo, Jeong-Meen

    2017-06-01

    Currently, a diamond-shaped anastomosis is preferred for the surgical repair of duodenal atresia (DA) in both open and laparoscopic surgery. We report the results of laparoscopic duodenoduodenostomy with parallel anastomosis (LDPA) in DA. We retrospectively reviewed 22 patients who underwent laparoscopic duodenoduodenostomy from February 2005 to May 2015 in Samsung Medical Center. All patients underwent operation within the first month after birth. Patients who were transversely anastomosed after duodenotomy and patients who underwent simultaneous operation on combined anomalies were excluded. Parallel anastomosis was used in all surgeries. Four trocars were used in laparoscopic repair. After mobilization of both proximal and distal ends, the proximal end was incised transversely and the distal end was incised longitudinally. Duodenoduodenostomy with parallel anastomosis using a 5-0 glyconate monofilament was performed with interrupted sutures. Eleven patients (50 %) were male. Median gestational age was 36 + 6 weeks (32 + 7-40 + 6). Median age at the time of operation and median body weight were 3 days (1-12) and 2.53 kg (1.63-3.18), respectively. All patients were diagnosed prenatally and 16 patients (72.7 %) had associated anomalies. Median operation time was 142 min (96-290) and median postoperative day to start oral feeding was 5 days (3-9) and median postoperative day of reaching full feeding was 11 days (6-19). Median postoperative day was 13 days (10-60). There was no anastomotic leakage or stenosis. Median follow up was 3.5 months (1-21). Currently, there is no late complication. LDPA can be performed easily to patients who have DA in neonatal period. It is anatomically natural and the risk of leakage or stenosis does not seem significant. Therefore, parallel anastomosis should be considered as a safe procedural option for laparoscopic duodenoduodenostomy in DA.

  12. Endotoxin and CD14 in the progression of biliary atresia

    Directory of Open Access Journals (Sweden)

    Chen Ching-Mei

    2010-12-01

    Full Text Available Abstract Background Biliary atresia (BA is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/or extra-hepatic bile ducts. However, the mechanisms contributing to the pathogenesis of BA remain uncertain. Because of decreased bile flow, infectious complications and damaging endotoxemia occur frequently in patients with BA. The aim of this study was to investigate endotoxin levels in patients with BA and the relation of these levels with the expression of the endotoxin receptor, CD14. Methods The plasma levels of endotoxin and soluble CD14 were measured with a pyrochrome Limulus amebocyte lysate assay and enzyme-linked immunosorbent assay in patients with early-stage BA when they received the Kasai procedure (KP, in patients who were jaundice-free post-KP and followed-up at the outpatient department, in patients with late-stage BA when they received liver transplantation, and in patients with choledochal cysts. The correlation of CD14 expression with endotoxin levels in rats following common bile duct ligation was investigated. Results The results demonstrated a significantly higher hepatic CD14 mRNA and soluble CD14 plasma levels in patients with early-stage BA relative to those with late-stage BA. However, plasma endotoxin levels were significantly higher in both the early and late stages of BA relative to controls. In rat model, the results demonstrated that both endotoxin and CD14 levels were significantly increased in liver tissues of rats following bile duct ligation. Conclusions The significant increase in plasma endotoxin and soluble CD14 levels during BA implies a possible involvement of endotoxin stimulated CD14 production by hepatocytes in the early stage of BA for removal of endotoxin; whereas, endotoxin signaling likely induced liver injury and impaired soluble CD14 synthesis in the late stages of BA.

  13. Outcomes in children with biliary atresia following liver transplantation

    Institute of Scientific and Technical Information of China (English)

    Li-Ying Sun; Chong Dong; Jin-Peng Tu; Jian Wang; Yi-He Liu; Yuan Liu; Li-Xin Yu; Yu Wang; Jing Li and Zhong-Yang Shen; Yun-Sheng Yang; Zhi-Jun Zhu; Wei Gao; Lin Wei; Xiao-Ye Sun; Wei Qu; Wei Rao; Zhi-Gui Zeng

    2013-01-01

    BACKGROUND: Congenital biliary atresia is a rare condition characterized  by  idiopathic  dysgenesis  of  the  bile  ducts.  If untreated,  congenital  biliary  atresia  leads  to  liver  cirrhosis, liver failure and premature death. The present study aimed to evaluate  the  outcomes  of  orthotopic  liver  transplantation  in children with biliary atresia. METHOD: We retrospectively analyzed 45 patients with biliary atresia  who  had  undergone  orthotopic  liver  transplantation from September 2006 to August 2012. RESULTS: The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION: With  advances  in  surgical  techniques and  management,  children  with  biliary  atresia  after  liver transplantation  can  achieve  satisfactory  survival  in  China, although there remains a high risk of complications in the early postoperative period.

  14. The Changing Face of Esophageal Cancer

    Directory of Open Access Journals (Sweden)

    Rachel E. Melhado

    2010-06-01

    Full Text Available The two main histological esophageal cancer types, adenocarcinoma and squamous cell carcinoma, differ in incidence, geographic distribution, ethnic pattern and etiology. This article focuses on epidemiology with particular reference to geographic and temporal variations in incidence, along with a review of the evidence supporting environmental and genetic factors involved in esophageal carcinogenesis. Squamous cell carcinoma of the esophagus remains predominantly a disease of the developing world. In contrast, esophageal adenocarcinoma is mainly a disease of western developed societies, associated with obesity and gastro-esophageal reflux disease. There has been a dramatic increase in the incidence of adenocarcinoma in developed countries in parallel with migration of both esophageal and gastric adenocarcinomas towards the gastro-esophageal junction.

  15. The Changing Face of Esophageal Cancer

    Energy Technology Data Exchange (ETDEWEB)

    Melhado, Rachel E., E-mail: raye732001@yahoo.co.uk; Alderson, Derek; Tucker, Olga [Academic Department of Surgery, Queen Elizabeth Hospital, University Hospitals Birmingham, Birmingham (United Kingdom)

    2010-06-28

    The two main histological esophageal cancer types, adenocarcinoma and squamous cell carcinoma, differ in incidence, geographic distribution, ethnic pattern and etiology. This article focuses on epidemiology with particular reference to geographic and temporal variations in incidence, along with a review of the evidence supporting environmental and genetic factors involved in esophageal carcinogenesis. Squamous cell carcinoma of the esophagus remains predominantly a disease of the developing world. In contrast, esophageal adenocarcinoma is mainly a disease of western developed societies, associated with obesity and gastro-esophageal reflux disease. There has been a dramatic increase in the incidence of adenocarcinoma in developed countries in parallel with migration of both esophageal and gastric adenocarcinomas towards the gastro-esophageal junction.

  16. Radiation-induced esophagitis in lung cancer

    Directory of Open Access Journals (Sweden)

    Baker S

    2016-10-01

    Full Text Available Sarah Baker, Alysa Fairchild Department of Radiation Oncology, Cross Cancer Institute, University of Alberta, Edmonton, AB, Canada Abstract: Radiation-induced esophagitis is the most common local acute toxicity of radiotherapy (RT delivered for the curative or palliative intent treatment of lung cancer. Although concurrent chemotherapy and higher RT dose are associated with increased esophagitis risk, advancements in RT techniques as well as adherence to esophageal dosimetric constraints may reduce the incidence and severity. Mild acute esophagitis symptoms are generally self-limited, and supportive management options include analgesics, acid suppression, diet modification, treatment for candidiasis, and maintenance of adequate nutrition. Esophageal stricture is the most common late sequela from esophageal irradiation and can be addressed with endoscopic dilatation. Approaches to prevent or mitigate these toxicities are also discussed. Keywords: non–small cell lung cancer, acute, late, toxicity, stricture

  17. Unexpected esophageal diseases appeared in thyroid resections

    OpenAIRE

    Ye-huan, Liu; Shi-xu, Lyu; Yi-li, Zhou; Ou-chen, Wang; Xiao-hua, Zhang

    2015-01-01

    Objective In order to avoid the misdiagnosis of thyroid diseases, we need to discuss the clinical features and diagnostic methods of cervical esophageal cancer and Zenker’s diverticulum. Methods The clinical and laboratory data of seven cases were reviewed retrospectively, and in all cases, esophageal-related diseases were misdiagnosed as thyroid diseases preoperatively. Among them, two cases were cervical esophageal cancer metastasized to thyroids but initially, they were misdiagnosed as thy...

  18. Oral blastomycosis, laryngeal papillomatosis and esophageal tuberculosis

    OpenAIRE

    Montoya, Manuel; Hospital Regional del Cusco. Cusco, Perú. Universidad Nacional de San Antonio Abad del Cusco. Cusco, Perú. Médico infectólogo.; Chumbiraico, Robert; Hospital Regional del Cusco. Cusco, Perú. Universidad Nacional de San Antonio Abad del Cusco. Cusco, Perú. médico internista.; Ricalde, Melvin; Hospital Regional del Cusco. Cusco, Perú. Universidad Nacional de San Antonio Abad del Cusco. Cusco, Perú. médico neumólogo.; Cazorla, Ernesto; Hospital Regional del Cusco. Cusco, Perú. Universidad Nacional de San Antonio Abad del Cusco. Cusco, Perú. médico gastroenterólogo.; Hernández-Córdova, Gustavo; Universidad Nacional de San Antonio Abad del Cusco. Cusco, Perú. Estudiante de medicina.

    2014-01-01

    Esophageal involvement is an extremely rare complication of tuberculosis even in countries with high prevalence of infection. We report the case of a 57 year-old hiv-seronegative patient with simultaneous diagnoses of oral blastomycosis and laryngeal papillomatosis. Both were confirmed by anatomopathological analysis. The esophageal biopsy revealed granulomatous esophagitis with necrosis and ziehl–neelsen stain showed acid-fast alcohol resistant bacilli suggestive of tuberculosis. The pat...

  19. A Long-Gap Peripheral Nerve Injury Therapy Using Human Skeletal Muscle-Derived Stem Cells (Sk-SCs): An Achievement of Significant Morphological, Numerical and Functional Recovery.

    Science.gov (United States)

    Tamaki, Tetsuro; Hirata, Maki; Nakajima, Nobuyuki; Saito, Kosuke; Hashimoto, Hiroyuki; Soeda, Shuichi; Uchiyama, Yoshiyasu; Watanabe, Masahiko

    2016-01-01

    Losses in vital functions of the somatic motor and sensory nervous system are induced by severe long-gap peripheral nerve transection injury. In such cases, autologous nerve grafts are the gold standard treatment, despite the unavoidable sacrifice of other healthy functions, whereas the prognosis is not always favorable. Here, we use human skeletal muscle-derived stem cells (Sk-SCs) to reconstitute the function after long nerve-gap injury. Muscles samples were obtained from the amputated legs from 9 patients following unforeseen accidents. The Sk-SCs were isolated using conditioned collagenase solution, and sorted as CD34+/45- (Sk-34) and CD34-/45-/29+ (Sk-DN/29+) cells. Cells were separately cultured/expanded under optimal conditions for 2 weeks, then injected into the athymic nude mice sciatic nerve long-gap model (7-mm) bridging an acellular conduit. After 8-12 weeks, active cell engraftment was observed only in the Sk-34 cell transplanted group, showing preferential differentiation into Schwann cells and perineurial/endoneurial cells, as well as formation of the myelin sheath and perineurium/endoneurium surrounding regenerated axons, resulted in 87% of numerical recovery. Differentiation into vascular cell lineage (pericyte and endothelial cells) were also observed. A significant tetanic tension recovery (over 90%) of downstream muscles following electrical stimulation of the sciatic nerve (at upper portion of the gap) was also achieved. In contrast, Sk-DN/29+ cells were completely eliminated during the first 4 weeks, but relatively higher numerical (83% vs. 41% in axon) and functional (80% vs. 60% in tetanus) recovery than control were observed. Noteworthy, significant increase in the formation of vascular networks in the conduit during the early stage (first 2 weeks) of recovery was observed in both groups with the expression of key factors (mRNA and protein levels), suggesting the paracrine effects to angiogenesis. These results suggested that the human Sk

  20. Acute Necrotizing Esophagitis Followed by Duodenal Necrosis.

    Science.gov (United States)

    Del Hierro, Piedad Magdalena

    2011-12-01

    Acute Necrotizing Esophagitis is an uncommon pathology, characterized by endoscopic finding of diffuse black coloration in esophageal mucosa and histological presence of necrosis in patients with upper gastrointestinal bleeding. The first case of acute necrotizing esophagitis followed by duodenal necrosis, in 81 years old woman with a positive history of Type 2 Diabetes Mellitus, Hypertension, and usual intake of Nonsteroidal Anti-inflammatory drugs, is reported. Although its etiology remains unknown, the duodenal necrosis suggests that ischemia could be the main cause given that the branches off the celiac axis provide common blood supply to the distal esophageal and duodenal tissue. The massive gastroesophagic reflux and NSAID intake could be involved.

  1. Doxycycline-induced ulceration mimicking esophageal cancer

    Science.gov (United States)

    Tahan, Veysel; Sayrak, Hakan; Bayar, Nevzat; Erer, Burak; Tahan, Gulgun; Dane, Faysal

    2008-01-01

    Introduction Doxycycline-induced esophageal ulcer patients are mostly young persons with no history of esophageal dysfunction. Heartburn, midsternal pain and dysphagia are the most common symptoms. It has generally a benign course. The present case is the first report of doxycycline-induced extensive ulcerations, mimicking esophageal cancer in two esophageal segments alongside, in the literature. Case presentation This report describes a 16-year-old Caucasian girl who, while taking doxycycline capsules100 mg twice a day for acne vulgaris for 3 months, developed these symptoms. An upper endoscopy revealed multiple circumferential deep ulcerations surrounding fragile, irregular, hyperemic and hypertrophic mucosa at the level of the mid-esophagus and concomitantly in the lower esophageal sphincter. The lesions were biopsied to exclude esophageal carcinoma because of the suspicious appearance in the endoscopic examination. The histopathological examination, haematoxylin and eosin stained sections showed ulceration with a mixed inflammatory infiltrate. Doxycycline was discontinued and she was given sucralfate 1 g qid and omeprazole 20 mg bid orally. All symptoms of the patient were resolved on the third day of the treatment. After 4 weeks of the therapy, an upper endoscopic control examination demonstrated normal findings. Conclusion The present case has been an uncommon presentation of doxycycline-induced extensive ulcerations, mimicking esophageal cancer in two esophageal segments, concomitantly. Even the lesions were biopsied to exclude esophageal carcinoma. A modification on the behavior of taking drugs can prevent these unpleasant complications. PMID:18778470

  2. Minimally invasive surgery for esophageal achalasia

    Institute of Scientific and Technical Information of China (English)

    Luigi Bonavina

    2006-01-01

    Esophageal achalasia is the most commonly diagnosed primary esophageal motor disorder and the second most common functional esophageal disorder. Current therapy of achalasia is directed toward elimination of the outflow resistance caused by failure of the lower esophageal sphincter to relax completely upon swallowing. The advent of minimally invasive surgery has nearly replaced endoscopic pneumatic dilation as the first-line therapeutic approach. In this editorial, the rationale and the evidence supporting the use of laparoscopic Heller myotomy combined with fundoplication as a primary treatment of achalasia are reviewed.

  3. Esophageal motility abnormalities in gastroesophageal reflux disease.

    Science.gov (United States)

    Martinucci, Irene; de Bortoli, Nicola; Giacchino, Maria; Bodini, Giorgia; Marabotto, Elisa; Marchi, Santino; Savarino, Vincenzo; Savarino, Edoardo

    2014-05-06

    Esophageal motility abnormalities are among the main factors implicated in the pathogenesis of gastroesophageal reflux disease. The recent introduction in clinical and research practice of novel esophageal testing has markedly improved our understanding of the mechanisms contributing to the development of gastroesophageal reflux disease, allowing a better management of patients with this disorder. In this context, the present article intends to provide an overview of the current literature about esophageal motility dysfunctions in patients with gastroesophageal reflux disease. Esophageal manometry, by recording intraluminal pressure, represents the gold standard to diagnose esophageal motility abnormalities. In particular, using novel techniques, such as high resolution manometry with or without concurrent intraluminal impedance monitoring, transient lower esophageal sphincter (LES) relaxations, hypotensive LES, ineffective esophageal peristalsis and bolus transit abnormalities have been better defined and strongly implicated in gastroesophageal reflux disease development. Overall, recent findings suggest that esophageal motility abnormalities are increasingly prevalent with increasing severity of reflux disease, from non-erosive reflux disease to erosive reflux disease and Barrett's esophagus. Characterizing esophageal dysmotility among different subgroups of patients with reflux disease may represent a fundamental approach to properly diagnose these patients and, thus, to set up the best therapeutic management. Currently, surgery represents the only reliable way to restore the esophagogastric junction integrity and to reduce transient LES relaxations that are considered to be the predominant mechanism by which gastric contents can enter the esophagus. On that ground, more in depth future studies assessing the pathogenetic role of dysmotility in patients with reflux disease are warranted.

  4. Laboratory animal models for esophageal cancer

    Directory of Open Access Journals (Sweden)

    Dhanya Venugopalan Nair

    2016-11-01

    Full Text Available The incidence of esophageal cancer is rapidly increasing especially in developing countries. The major risk factors include unhealthy lifestyle practices such as alcohol consumption, smoking, and chewing tobacco to name a few. Diagnosis at an advanced stage and poor prognosis make esophageal cancer one of the most lethal diseases. These factors have urged further research in understanding the pathophysiology of the disease. Animal models not only aid in understanding the molecular pathogenesis of esophageal cancer but also help in developing therapeutic interventions for the disease. This review throws light on the various recent laboratory animal models for esophageal cancer.

  5. Pradaxa-induced esophageal ulcer.

    Science.gov (United States)

    Wood, Michele; Shaw, Paul

    2015-10-09

    Pradaxa (dabigatran) is a direct thrombin inhibitor approved for prevention of stroke and systemic embolism in patients with non-valvular atrial fibrillation. We describe a case of esophageal ulceration associated with Pradaxa administration in a 75-year-old man. The patient reported difficulty swallowing and a burning sensation after taking his first dose of Pradaxa. An esophagogastroduodenoscopy (EGD) revealed linear ulcerations in the mid-esophagus. Pradaxa was held beginning the day before the EGD. The patient reported that his pain and difficulty swallowing resolved on stopping Pradaxa. Pradaxa is formulated with a tartaric acid excipient to reduce variability in absorption. We hypothesise that the capsule lodged in the patient's esophagus and the tartaric acid may have caused local damage resulting in an esophageal ulcer. It is important to educate patients on proper administration of Pradaxa, to decrease the risk of this rare, but potentially serious adverse event.

  6. Missed diagnosis of atresia of the right pulmonary artery in woman with left-sided pneumothorax

    DEFF Research Database (Denmark)

    Dagnegård, Hanna; Ryom, Philip

    2016-01-01

    woman with an unknown atresia of the right pulmonary artery received a left-sided pneumothorax. The diagnosis was initially missed in spite of adequate imaging and the condition progressed to respiratory stop. We describe the course of diagnostics and the chosen strategy of treatment....

  7. Growth and development after oesophageal atresia surgery: Need for long-term multidisciplinary follow-up

    NARCIS (Netherlands)

    H. IJsselstijn (Hanneke); S.J. Gischler (Saskia); L.C.C. Toussaint (Leontien); M. Spoel (Marjolein); M.H.M. van der Cammen-van Zijp (Monique); D. Tibboel (Dick)

    2015-01-01

    markdownabstractAbstract Survival rates in oesophageal atresia patients have reached over 90%. In long-term follow-up studies the focus has shifted from purely surgical or gastrointestinal evaluation to a multidisciplinary approach. We reviewed the literature on the long-term morbidity of these

  8. MicroRNAs: New Insight in Modulating Follicular Atresia: A Review

    Science.gov (United States)

    Worku, Tesfaye; Rehman, Zia Ur; Talpur, Hira Sajjad; Bhattarai, Dinesh; Ullah, Farman; Malobi, Ngabu; Kebede, Tesfaye; Yang, Liguo

    2017-01-01

    Our understanding of the post-transcriptional mechanisms involved in follicular atresia is limited; however, an important development has been made in understanding the biological regulatory networks responsible for mediating follicular atresia. MicroRNAs have come to be seen as a key regulatory actor in determining cell fate in a wide range of tissues in normal and pathological processes. Profiling studies of miRNAs during follicular atresia and development have identified several putative miRNAs enriched in apoptosis signaling pathways. Subsequent in vitro and/or in vivo studies of granulosa cells have elucidated the functional role of some miRNAs along with their molecular pathways. In particular, the regulatory roles of some miRNAs have been consistently observed during studies of follicular cellular apoptosis. Continued work should gradually lead to better understanding of the role of miRNAs in this field. Ultimately, we expect this understanding will have substantial benefits for fertility management at both the in vivo or/and in vitro levels. The stable nature of miRNA holds remarkable promise in clinical use as a diagnostic tool and in reproductive medicine to solve the ever-increasing fertility problem. In this review, we summarize current knowledge of the involvement of miRNAs in follicular atresia, discuss the challenges for further work and pinpoint areas for future research. PMID:28208755

  9. MicroRNAs: New Insight in Modulating Follicular Atresia: A Review

    Directory of Open Access Journals (Sweden)

    Tesfaye Worku

    2017-02-01

    Full Text Available Our understanding of the post-transcriptional mechanisms involved in follicular atresia is limited; however, an important development has been made in understanding the biological regulatory networks responsible for mediating follicular atresia. MicroRNAs have come to be seen as a key regulatory actor in determining cell fate in a wide range of tissues in normal and pathological processes. Profiling studies of miRNAs during follicular atresia and development have identified several putative miRNAs enriched in apoptosis signaling pathways. Subsequent in vitro and/or in vivo studies of granulosa cells have elucidated the functional role of some miRNAs along with their molecular pathways. In particular, the regulatory roles of some miRNAs have been consistently observed during studies of follicular cellular apoptosis. Continued work should gradually lead to better understanding of the role of miRNAs in this field. Ultimately, we expect this understanding will have substantial benefits for fertility management at both the in vivo or/and in vitro levels. The stable nature of miRNA holds remarkable promise in clinical use as a diagnostic tool and in reproductive medicine to solve the ever-increasing fertility problem. In this review, we summarize current knowledge of the involvement of miRNAs in follicular atresia, discuss the challenges for further work and pinpoint areas for future research.

  10. The BAHA Softband. A new treatment for young children with bilateral congenital aural atresia.

    NARCIS (Netherlands)

    Hol, M.K.S.; Cremers, C.W.R.J.; Coppens-Schellekens, W.; Snik, A.F.M.

    2005-01-01

    OBJECTIVE: To evaluate the validity of a bone-anchored hearing aid (BAHA) Softband (fitted unilaterally and bilaterally) in young children with bilateral congenital aural atresia. SUBJECTS: Two children with severe bilateral congenital conductive hearing loss, who had been fitted with a transcutaneo

  11. Neonatal and pediatric esophageal perforation.

    Science.gov (United States)

    Rentea, Rebecca M; St Peter, Shawn D

    2017-04-01

    Esophageal perforation (EP) is a rare complication that is often iatrogenic in origin. In contrast with adult patients in whom surgical closure of the defect is preferred, nonoperative treatment has become a common therapeutic approach for EP in neonates and children. Principles of management pediatric EP includes rapid diagnosis, appropriate hemodynamic monitoring and support, antibiotic therapy, total parenteral nutrition, control of extraluminal contamination, and restoration of luminal integrity either through time or operative approaches. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Vitiligo associated with esophageal adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Ali Asilian

    2013-01-01

    Full Text Available Vitiligo is a disease that results in depigmented areas in the skin. It may develop at any age but the average age at onset is 20 years. Association of vitiligo and melanoma has been commonly reported, but malignancies other than melanoma have been rarely associated with vitiligo. We report a 73-year-old patient with new onset vitiligo who developed esophageal adenocarcinoma in the following years.

  13. Optimal lymphadenectomy for esophageal adenocarcinoma.

    Science.gov (United States)

    Oezcelik, A

    2013-08-01

    Recently published data have shown that an extended lymphadenectomy during the en bloc esophagectomy leads to a significant increased long-term survival for esophageal adenocarcinoma. On the other hand some studies indicate that the increased survival is based on stage migration and that the surgical complication rate is increased after extended lymphadenectomy. The aim of this review was to give an overview about all aspects of an extended lymphadenectomy in patients with esophageal adenocarcinoma. The review of the literature shows clearly that the number of involved lymph nodes is an independent prognostic factor in patients with esophageal adenocarcinoma. Furthermore, an extended lymphadenectomy leads to an increased long-term survival. Some studies describe that 23 lymph nodes should be removed to predict survival; other studies 18 lymph nodes or 15 lymph nodes. Opponents indicate that the survival benefit is based on stage migration. The studies with a large study population have performed a Cox regression analyzes and identified the number of lymph nodes removed as an independent factor for improved survival, which means it is significant independently from other parameters. Under these circumstances is stage migration not an option to explain the survival benefit. An important difficulty is, that there is no standardized definition of an extended lymphadenectomy, which means the localization and number of removed lymph nodes differ depending from the performing centre. The controversies regarding the survival benefit of the lymphadenectomy is based on the lack of standardisation of the lymphadenectomy. The main goal of further studies should be to generate a clear definition of an extended lymphadenectomy in patients with esophageal adenocarcinoma.

  14. The Role of ARF6 in Biliary Atresia.

    Directory of Open Access Journals (Sweden)

    Mylarappa Ningappa

    Full Text Available Altered extrahepatic bile ducts, gut, and cardiovascular anomalies constitute the variable phenotype of biliary atresia (BA.To identify potential susceptibility loci, Caucasian children, normal (controls and with BA (cases at two US centers were compared at >550000 SNP loci. Systems biology analysis was carried out on the data. In order to validate a key gene identified in the analysis, biliary morphogenesis was evaluated in 2-5-day post-fertilization zebrafish embryos after morpholino-antisense oligonucleotide knockdown of the candidate gene ADP ribosylation factor-6 (ARF6, Mo-arf6.Among 39 and 24 cases at centers 1 and 2, respectively, and 1907 controls, which clustered together on principal component analysis, the SNPs rs3126184 and rs10140366 in a 3' flanking enhancer region for ARF6 demonstrated higher minor allele frequencies (MAF in each cohort, and 63 combined cases, compared with controls (0.286 vs. 0.131, P = 5.94x10-7, OR 2.66; 0.286 vs. 0.13, P = 5.57x10-7, OR 2.66. Significance was enhanced in 77 total cases, which included 14 additional BA genotyped at rs3126184 only (p = 1.58x10-2, OR = 2.66. Pathway analysis of the 1000 top-ranked SNPs in CHP cases revealed enrichment of genes for EGF regulators (p<1 x10-7, ERK/MAPK and CREB canonical pathways (p<1 x10-34, and functional networks for cellular development and proliferation (p<1 x10-45, further supporting the role of EGFR-ARF6 signaling in BA. In zebrafish embryos, Mo-arf6 injection resulted in a sparse intrahepatic biliary network, several biliary epithelial cell defects, and poor bile excretion to the gall bladder compared with uninjected embryos. Biliary defects were reproduced with the EGFR-blocker AG1478 alone or with Mo-arf6 at lower doses of each agent and rescued with arf6 mRNA.The BA-associated SNPs identify a chromosome 14q21.3 susceptibility locus encompassing the ARF6 gene. arf6 knockdown in zebrafish implicates early biliary dysgenesis as a basis for BA, and also

  15. Physical development and cognitive performance in a monozygotic twins for biliary atresia: Report of a case and literature reviewing

    Directory of Open Access Journals (Sweden)

    Yang Chen

    2016-08-01

    Full Text Available A case of monozygotic twins, one of whom suffered from biliary atresia and the other one was apparently healthy, is described herein. Children with biliary atresia appear to be vulnerable to developmental lags in the physical and cognitive performance, due to malnutrition and deteriorated liver function. In our case where the child with biliary atresia, who underwent a successful kasai operation, has achieved comparable growth development and cognitive performance in comparision with her sister, which suggest that the early diagnosis and timly kasai operation could allow children with biliary artesia to grow well in the short-term following time.

  16. Hepatocellular carcinoma in the native liver of a 38-year-old female patient with biliary atresia

    Directory of Open Access Journals (Sweden)

    Yutaka Kanamori

    2015-11-01

    Full Text Available We report a rare case of hepatocellular carcinoma in native liver in a case of biliary atresia. The patient was a 38-year-old female with three children who had an aggressive tumor, resulting in her subsequent death. We also review 14 reports, published previously in the English language medical literature, concerning hepatocellular carcinoma originating from native liver in biliary atresia cases and discuss the possible etiology, and propose more careful follow up for the patients with biliary atresia who suffer from repetitive cholangitis and/or experience the child delivery.

  17. Proteomic profiling of fetal esophageal epithelium, esophageal cancer, and tumor-adjacent esophageal epithelium and immunohistochemical characterization of a representative differential protein, PRX6

    Science.gov (United States)

    Guo, Jun-Hui; Xing, Guo-Lan; Fang, Xin-Hui; Wu, Hui-Fang; Zhang, Bo; Yu, Jin-Zhong; Fan, Zong-Min; Wang, Li-Dong

    2017-01-01

    AIM To understand the molecular mechanism of esophageal cancer development and provide molecular markers for screening high-risk populations and early diagnosis. METHODS Two-dimensional electrophoresis combined with mass spectrometry were adopted to screen differentially expressed proteins in nine cases of fetal esophageal epithelium, eight cases of esophageal cancer, and eight cases of tumor-adjacent normal esophageal epithelium collected from fetuses of different gestational age, or esophageal cancer patients from a high-risk area of esophageal cancer in China. Immunohistochemistry (avidin-biotin-horseradish peroxidase complex method) was used to detect the expression of peroxiredoxin (PRX)6 in 91 cases of esophageal cancer, tumor-adjacent normal esophageal tissue, basal cell hyperplasia, dysplasia, and carcinoma in situ, as well as 65 cases of esophageal epithelium from fetuses at a gestational age of 3-9 mo. RESULTS After peptide mass fingerprint analysis and search of protein databases, 21 differential proteins were identified; some of which represent a protein isoform. Varying degrees of expression of PRX6 protein, which was localized mainly in the cytoplasm, were detected in adult and fetal normal esophageal tissues, precancerous lesions, and esophageal cancer. With the progression of esophageal lesions, PRX6 protein expression showed a declining trend (P PRX6 protein expression showed a declining trend with age (P PRX6 protein expression was significantly higher in well-differentiated esophageal cancer tissues than in poorly differentiated esophageal cancer tissues (P PRX6 protein is associated with fetal esophageal development and cancer differentiation. PMID:28293090

  18. Novel device to sample the esophageal microbiome--the esophageal string test.

    Directory of Open Access Journals (Sweden)

    Sophie A Fillon

    Full Text Available A growing number of studies implicate the microbiome in the pathogenesis of intestinal inflammation. Previous work has shown that adults with esophagitis related to gastroesophageal reflux disease have altered esophageal microbiota compared to those who do not have esophagitis. In these studies, sampling of the esophageal microbiome was accomplished by isolating DNA from esophageal biopsies obtained at the time of upper endoscopy. The aim of the current study was to identify the esophageal microbiome in pediatric individuals with normal esophageal mucosa using a minimally invasive, capsule-based string technology, the Enterotest™. We used the proximal segment of the Enterotest string to sample the esophagus, and term this the "Esophageal String Test" (EST. We hypothesized that the less invasive EST would capture mucosal adherent bacteria present in the esophagus in a similar fashion as mucosal biopsy. EST samples and mucosal biopsies were collected from children with no esophageal inflammation (n = 15 and their microbiome composition determined by 16S rRNA gene sequencing. Microbiota from esophageal biopsies and ESTs produced nearly identical profiles of bacterial genera and were different from the bacterial contents of samples collected from the nasal and oral cavity. We conclude that the minimally invasive EST can serve as a useful device for study of the esophageal microbiome.

  19. DUCTAL STENTING IN PULMONARY ATRESIA NEONATES WITH MULTIPLE CONGENITAL ANOMALIES AND SEPTIC CONDITION

    Directory of Open Access Journals (Sweden)

    Laurentia Utari Wibisono

    2015-10-01

    Full Text Available Neonates with  pulmonary  atresia  usually  appear  normal  at  birth with  pulmonary  circulationmaintained by the presence of a patent ductus arteriosus (PDA. Rapid deterioration will suddenlyocccur  if the duct close. Surgical shunt  is still be used as a standard protocol  in many centers as apalliative procedure. We report a 2 days-old, low birth weight, and mild cyanotic neonate with pulmonaryatresia and PDA accompanied by atresia ani, bladder and cloaca extropy, ambiguous genitalia andsepsis. We decided to perform PDA stenting because our patient have a high surgical shunt risk. Thisprocedure was very  important  to keep  the duct remains open until patient ready  for  total surgicalcorrection. [MEDICINA 2015;46:42-45].Neonatus dengan atresia pulmonal biasanya tampak normal saat lahir dengan adanya patent ductusarteriosus (PDA yang memelihara aliran darah paru. Kondisi neonatus akan segera memburuk jikaduktus menutup. Pembuatan shunt dengan pembedahan merupakan protokol standar yang masihdikerjakan di banyak pusat kesehatan. Kami melaporkan neonatus berusia 2 hari dengan berat badanlahir rendah dan sianosis ringan dengan diagnosis atresia pulmonal, PDA, atresia ani, ekstropi buli-buli dan kloaka, jenis kelamin ambigu, dan sepsis. Kami memutuskan untuk melakukan pemasanganstent pada PDA karena pasien kami memiliki risiko yang tinggi untuk pembedahan (pembuatan shunt.Tindakan ini sangat penting untuk menjaga duktus tetap terbuka sampai pasien siap untuk dilakukanoperasi koreksi. [MEDICINA 2015;46:42-45].

  20. An imaging study of the facial nerve canal in congenital aural atresia.

    Science.gov (United States)

    Zhao, Shouqin; Han, Demin; Wang, Zhenchang; Li, Jie; Qian, Yanni; Ren, Yuanyuan; Dong, Jiyong

    2015-01-01

    We conducted a prospective study to investigate the abnormalities of the facial nerve canal in patients with congenital aural atresia by computed tomography (CT). Our study population was made up of 99 patients--68 males and 31 females, aged 6 to 22 years (mean: 13.5)--who had unilateral congenital aural atresia without any inner ear malformations. We compared our findings in these patients with those in 50 controls-33 males and 17 females, aged 5 to 22 years (mean: 15.0)-who had normal ears. We classified the congenital aural atresia patients into three groups (A, B, and C) according to their Jahrsdoerfer grading scale score (≥8; 6 or 7; and ≤5, respectively). The course of the facial nerve canal in both the controls and the study patients was determined by temporal bone CT with multiplanar reconstruction. The distances from different parts of the facial nerve canal to surrounding structures were also measured. The course of the facial nerve canal in the normal ears did not vary much, and there were no statistically significant differences according to head side and sex. In groups B and C, the tympanic segment, mastoid segment, and angle of the second genu of the facial nerve canal were all significantly smaller than those of the controls (p < 0.01 in all cases). Statistically, the tympanic segment of the facial nerve canal in patients with congenital aural atresia was downwardly displaced. The mastoid segment of the facial nerve canal in these patients was more anterior than that of the controls. We conclude that congenital aural atresia is often accompanied by abnormalities of the facial nerve canal, especially in the tympanic segment, the mastoid segment, and the second genu. We found that the lower the Jahrsdoerfer score was, the shorter the tympanic segment was and the more forward the mastoid segment was.

  1. Management of oesophageal atresia in a developing country: Is primary repair forbidden?

    Directory of Open Access Journals (Sweden)

    Samuel Osei-Nketiah

    2016-01-01

    Full Text Available Background: The aim of the study was to evaluate the outcomes of initial surgical procedures for oesophageal atresia at our institution. Primary repair of oesophageal atresia at our centre was perceived to be associated with a high mortality rate. In view of this, almost all patients seen since January 2014 were offered initial surgery for staged repair. Materials and Methods: A retrospective review of records of infants with oesophageal atresia seen at the centre from January 2007 to December 2014 was used in this study. Results: Eighty-five cases of oesophageal atresia were seen over the study period. Of these, 74 (87.1 had surgery performed. Surgical outcome was, however, indicated in 67 of the 74, with overall survival rate of 40.3%. Among the 67 patients, early primary repair was performed in 24 (35.8% with a survival rate of 45.8% (11 patients. Totally, 12 (17.9% of the 67 had initial procedure for delayed primary repair, with a survival rate of 16.7% (2 patients. The remaining 31 (46.3% patients had initial surgery for staged repair, with survival rate of 45.2% (14 patients. There was no association between the type of surgery and the surgical outcome (χ2 = 3.396, df = 2, P = 0.183. Conclusion: The overall surgical survival rate of 40.3% for oesophageal atresia at our institution is low. This study did not show any difference in the survival rate of 45.8% and 45.2% associated with primary repair and staged repair respectively.

  2. Management guidelines of eosinophilic esophagitis in childhood

    DEFF Research Database (Denmark)

    Papadopoulou, A; Koletzko, S; Heuschkel, R

    2014-01-01

    (peak value) in ≥1 biopsy specimens are considered a minimum threshold for a diagnosis of EoE. The disease is restricted to the esophagus, and other causes of esophageal eosinophilia should be excluded, specifically proton pump inhibitor-responsive esophageal eosinophilia. This position paper aims...

  3. Iatrogenic esophageal perforation in a newborn

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jeong Mi; Yeon, Kyung Mo; Park, Won Soon; Choi, Jung Hwan [College of Medicine, Seoul National Univ., Seoul (Korea, Republic of)

    1990-07-15

    Esophagus and pharyngeal structures of newborn are very week and so vulnerable. When a parallel longitudinal tubular structure around (especially behind) the esophagus is seen, traumatic esophageal perforation must be differentiated. We report a case of esophageal perforation in a premature twin baby by nasogastric tube insertion.

  4. PET-CT manifestation of Candida esophagitis

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Yong Whee [Sung-Ae Hospital, Seoul (Korea, Republic of); O, Joo Hyun [Kangnam St. Mary' s Hospital, Catholic University Medical School, Seoul (Korea, Republic of)

    2007-04-15

    Candida esophagitis (moniliasis) is the most common infection of the gullet and has generally been attributed to as a complication of immune suppressed state. However, as the current case. Holt found the disease to occur in 3 of his 13 patients without predisposing condition. Predisposing factors other than immune deficient conditions include aplastic anemia, alcoholism and Parkinson's disease and age, diabetes mellitus, and disruption of mucosal integrity. Growing prevalence of Candida esophagitis in recent years is accounted for by an increase in the number of patients with organ transplantation, malignancy and AIDS as well as populrization of endoscopy. Microorganisms that reached the esophagus in oral secretions are rarely cultured from the esophageal surface. Of many species C. albicans is the most common offender although C. tropicalis has also been isolated with high prevalence, particularly in the patients with cancer and disseminated candidiasis. Clinically, the patients with Candida esophagitis seek medical care for esophageal or retrosternal pain, dysphagia or distress. Candida esophagitis may be the extension from oropharyngeal infection but in the majority the esophagus is the sole site of infection. The middle and lower thirds of the esophagus are more typically affected than the upper third. Diagnosis can be indicated by double contrast esophagography or endoscopy and confirmed by potassium hydroxide (KOH) stain or biopsy. It is to be noted that the more presence of Candida in smear or cultured specimen cannot indict Candida as definitive offender. Differential diagnosis includes herpes simplex infection, cytomegalovirus infection, reflux esophagitis or radiation esophagitis.

  5. Palliation of Dysphagia from Esophageal Cancer

    NARCIS (Netherlands)

    M.Y.V. Homs (Marjolein)

    2004-01-01

    textabstractThe prognosis of esophageal cancer is poor with a 5-year survival of 10-15%. In addition, over 50% of patients with esophageal cancer already have an inoperable disease at presentation. The majority of these patients require palliative treatment to relieve progressive dysphagia. Metal

  6. Palliative Endoscopic Therapy of Esophageal Cancer

    Institute of Scientific and Technical Information of China (English)

    H.Schaefer; A.H.Hoelscher

    2004-01-01

    Patients with locally unresectable esophageal cancer or distant metastasis are usually treated with definite radiotherapy or radiochemotherapy. Dysphagia of these patients should further be treated by endoscopic therapy in order to maintain swallowing and oral food intake as long as possible. The same situation is present in patients with local recurrence of esophageal cancer after surgery or radiochemotherapy.

  7. Esophageal motility abnormalities in gastroesophageal reflux disease

    Institute of Scientific and Technical Information of China (English)

    Irene; Martinucci; Nicola; de; Bortoli; Maria; Giacchino; Giorgia; Bodini; Elisa; Marabotto; Santino; Marchi; Vincenzo; Savarino; Edoardo; Savarino

    2014-01-01

    Esophageal motility abnormalities are among the main factors implicated in the pathogenesis of gastroesophageal reflux disease. The recent introduction in clinical and research practice of novel esophageal testing has markedly improved our understanding of the mechanisms contributing to the development of gastroesophageal reflux disease, allowing a better management of patients with this disorder. In this context, the present article intends to provide an overview of the current literature about esophageal motility dysfunctions in patients with gastroesophageal reflux disease. Esophageal manometry, by recording intraluminal pressure, represents the gold standard to diagnose esophagealmotility abnormalities. In particular, using novel techniques, such as high resolution manometry with or without concurrent intraluminal impedance monitoring, transient lower esophageal sphincter (LES) relaxations, hypotensive LES, ineffective esophageal peristalsis and bolus transit abnormalities have been better defined and strongly implicated in gastroesophageal reflux disease development. Overall, recent findings suggest that esophageal motility abnormalities are increasingly prevalent with increasing severity of reflux disease, from nonerosive reflux disease to erosive reflux disease and Barrett’s esophagus. Characterizing esophageal dysmotility among different subgroups of patients with reflux disease may represent a fundamental approach to properly diagnose these patients and, thus, to set up the best therapeutic management. Currently, surgery represents the only reliable way to restore the esophagogastric junction integrity and to reduce transient LES relaxations that are considered to be the predominant mechanism by which gastric contents can enter the esophagus. On that ground, more in depth future studies assessing the pathogenetic role of dysmotility in patients with reflux disease are warranted.

  8. Comparative genomic analysis of esophageal cancers.

    Science.gov (United States)

    Caygill, Christine P J; Gatenby, Piers A C; Herceg, Zdenko; Lima, Sheila C S; Pinto, Luis F R; Watson, Anthony; Wu, Ming-Shiang

    2014-09-01

    The following, from the 12th OESO World Conference: Cancers of the Esophagus, includes commentaries on comparative genomic analysis of esophageal cancers: genomic polymorphisms, the genetic and epigenetic drivers in esophageal cancers, and the collection of data in the UK Barrett's Oesophagus Registry.

  9. Esophageal diverticula in Parma wallabies (Macropus parma).

    Science.gov (United States)

    Okeson, Danelle M; Esterline, Meredith L; Coke, Rob L

    2009-03-01

    Four adult, wild caught Parma wallabies (Macropus parma) presented with intermittent, postprandial, midcervical swellings. Esophageal diverticula were discovered in the four animals. One of two wallabies was managed successfully with surgery. A third animal died of other causes. The fourth animal died with possible complications from the diverticulum. This is the first published report of esophageal diverticula in macropods.

  10. Systemic complications of esophageal lichen planus.

    Science.gov (United States)

    Manna, R; Garcovich, S; Giovinale, M; Marinaro, A; Manganelli, C; Zampetti, A; Feliciani, C

    2013-01-01

    Lichen planus is an uncommon inflammatory mucocutaneous disorder affecting the skin and its appendages, as well as oral and genital mucosa. Involvement of the esophageal mucosa is rare and causes significant morbidity, with dysphagia and risk of long-term complications, such as esophageal strictures and stenosis. Esophageal lichen planus is an underreported condition in the spectrum of lichenoid tissue reactions, presenting the risk of systemic manifestations. We describe a patient with severe, long-standing esophageal lichen planus, which had led to marked weight-loss, malnutrition syndrome and chronic respiratory distress due to recurrent aspiration pneumonia. Diagnosis was confirmed by the presence of concomitant muco-cutaneous lesions and characteristic endoscopic and histological findings. Systemic therapy with cyclosporine A and micronutrient supplementation led to rapid clinical improvement. Early diagnosis of esophageal lichen planus as well as effective systemic immunosuppressive treatment is crucial in order to prevent short- and long-term complications.

  11. [Endoscopic surgery for benign esophageal diseases].

    Science.gov (United States)

    Ozawa, Soji

    2006-07-01

    Gastroesophageal reflux disease (GERD) and esophageal achalasia are common benign esophageal diseases. Today minimally invasive surgery is recommended to treat these diseases. Surgical indications for GERD are failure of medical management, medical complications attributable to a large hiatal hernia, 'atypical' symptoms (asthma, hoarseness, cough, chest pain, aspiration), etc. according to the Society of American Gastrointestinal Endoscopic Surgeons (SAGES) guidelines. Laparoscopic Nissen fundoplication has emerged as the most widely accepted procedure for GERD patients with normal esophageal motility. Partial fundoplication (e.g., Toupet fundoplication) is also considered to decrease the possibility of postoperative dysphagia. Although pneumatic dilatation has been the first line treatment for esophageal achalasia, laparoscopic Heller myotomy and partial fundoplication (e.g., Dor fundoplication) to prevent reflux is preferred by most gastroenterologists and surgeons as the primary treatment modality. Laparoscopic surgery for GERD and esophageal achalasia are effective in most patients and safe in all patients. Finally, laparoscopic surgery should be performed only by skilled surgeons.

  12. Lichenoid esophagitis: clinicopathologic overlap with established esophageal lichen planus.

    Science.gov (United States)

    Salaria, Safia N; Abu Alfa, Amer K; Cruise, Michael W; Wood, Laura D; Montgomery, Elizabeth A

    2013-12-01

    Lichen planus (LP) affects mucocutaneous surfaces and is characterized by intraepithelial and lamina propria lymphocytosis and squamous cell apoptosis (Civatte bodies). Lichen planus esophagitis (LPE) is underrecognized; concurrent cutaneous disease is present in some patients, but LPE alone is more common. We diagnose patients with characteristic pathologic findings of LPE and known correlation with skin disease or immunofluorescence (IF) results as LPE but use descriptive terminology ("lichenoid esophagitis pattern" [LEP]) when confirmation is unavailable. We reviewed clinicopathologic features of patients diagnosed at our institution with LPE or LEP. There were 88 specimens with LPE or LEP from 65 patients. Most patients were female. Seventeen patients had LPE confirmed by IF. Five patients had both esophageal (1 with IF) and skin LP. Strictures were a prominent presenting feature in LPE patients, with disease distribution more frequent in the upper and lower esophagus. Dysphagia was a common reason for endoscopy in LEP patients. Rheumatologic diseases are more common in patients with LPE compared with LEP. Viral hepatitides and human immunodeficiency virus (HIV) infections are associated with LEP. We defined polypharmacy as patients taking >3 medications; this finding was present in both LPE and LEP cohorts; however, this is a prominent feature in those with established LPE. Progression to dysplasia was noted in both cohorts. About 5% of LPE patients have tandem skin manifestations. LPE is more likely than LEP to arise in women, result in stricture formation, and be associated with rheumatologic disorders and polypharmacy, whereas LEP is associated with viral hepatitis and HIV. Both can progress to neoplasia. As the risk of stricture formation is high in patients with LPE, it is worth performing pertinent IF studies to confirm LPE, although knowledge of the clinical association of LEP with viral hepatitis, HIV, and use of multiple medications is of value in

  13. Esophageal strictures during treatment for acute lymphoblastic leukemia.

    LENUS (Irish Health Repository)

    Kelly, Kevin

    2012-02-01

    Esophageal stricture is a rare complication of paediatric cancer treatment that usually occurs after esophageal exposure to radiotherapy. We describe 4 cases of esophageal stricture during chemotherapy for acute lymphoblastic leukemia. All patients presented with refractory vomiting and were diagnosed with radiologic contrast studies. None of the patients had received radiotherapy. Esophageal candidiasis was seen in 2 patients but the remaining 2 patients had earlier systemic candidiasis. High-dose dexamethasone may predispose these children to both esophageal candidiasis and peptic esophagitis. The etiology of esophageal strictures during treatment for acute leukemia is likely to be multifactorial but systemic candidiasis may play a significant role.

  14. A comparative analysis by SAGE of gene expression profiles of esophageal adenocarcinoma and esophageal squamous cell carcinoma

    NARCIS (Netherlands)

    van Baal, Jantine W. P. M.; Milana, Francesco; Rygiel, Agnieszka M.; Sondermeijer, Carine M. T.; Spek, C. Arnold; Bergman, Jacques J. G. H. M.; Peppelenbosch, Maikel P.; Krishnadath, Kausilia K.

    2008-01-01

    Esophageal adenocarcinoma (EA) and esophageal squamous cell carcinoma (ESCC) are the two main types of esophageal cancer. Despite extensive research the exact molecular basis of these cancers is unclear. Therefore we evaluated the transcriptome of EA in comparison to non-dysplastic Barrett's esophag

  15. Giant mid-esophageal diverticulum. Conservative treatment of postoperative leakage.

    Science.gov (United States)

    Dallatomasina, S; Casaccia, M; Chessa, M; Serrano, J; Nardi, I; Troilo, B; Miggino, M; Valente, U

    2009-01-01

    Mid-esophageal diverticula are rare entities. Only symptomatic patients usually receive surgical treatment. Esophageal leakage is one of the most common complications after these procedures. Though in literature, operative management is the preferred treatment for esophageal fistula, conservative approach is described in case of small leaks. We report a case of an operated giant mid-esophageal diverticulum complicated with an esophageal fistula. The patient underwent a surgical treatment and recovered completely.

  16. Methoxychlor inhibits growth and induces atresia through the aryl hydrocarbon receptor pathway in mouse ovarian antral follicles.

    Science.gov (United States)

    Basavarajappa, Mallikarjuna S; Hernández-Ochoa, Isabel; Wang, Wei; Flaws, Jodi A

    2012-08-01

    Methoxychlor (MXC) is an organochlorine pesticide used against pests that attack crops, vegetables, and livestock. MXC inhibits growth and induces atresia (death) of mouse ovarian antral follicles in vitro. Since several studies indicate that many chemicals act through the aryl hydrocarbon receptor (AHR) pathway, the current study tested the hypothesis that MXC binds to the AHR to inhibit growth and induce atresia of antral follicles. The data indicate that MXC binds to AHR. Further, a relatively high dose of MXC (100μg/ml) inhibits growth and induces atresia in both wild-type (WT) and AHR null (AHRKO) follicles, whereas a lower dose of MXC (10μg/ml) inhibits growth and induces atresia in WT, but not in AHRKO follicles. These data indicate that AHR deletion partially protects antral follicles from MXC induced slow growth and atresia. Collectively, these data show that MXC may act through the AHR pathway to inhibit follicle growth and induce atresia in antral follicles of the ovary.

  17. Activity assessment of eosinophilic esophagitis.

    Science.gov (United States)

    Schoepfer, Alain; Safroneeva, Ekaterina

    2014-01-01

    The activity of eosinophilic esophagitis (EoE) can be assessed with patient-reported outcomes and biologic measures. Patient-reported outcomes include symptoms and quality of life, whereas biologic measures refer to endoscopic, histologic, and biochemical activity (e.g. blood biomarkers). So far, a validated tool to assess EoE activity in the above-mentioned dimensions is lacking. Given the lack of a standardized way to assess EoE activity in the various dimensions, the results of different clinical trials may be difficult to compare. For symptom assessment in adult patients, the symptom 'dysphagia' should be evaluated according to different standardized food consistencies. Furthermore, symptom assessment should take into account the following items: avoidance of specific food categories, food modification, and time to eat a regular meal. A distinct symptom recall period (e.g. 2 weeks) has to be defined for symptom assessment. Performing an 'esophageal stress test' with ingestion of a standardized meal to measure symptom severity bears the potential risk of acute food bolus impaction and should therefore be avoided. The description of endoscopic findings in EoE has meanwhile been standardized. Histologic evaluation of EoE activity should report either the size of the high-power field used or count the eosinophils per mm(2). There is a current lack of blood biomarkers demonstrating a good correlation with histologic activity in esophageal biopsies. The development and validation of an adult and pediatric EoE activity index is urgently needed not only for clinical trials and observational studies, but also for daily practice.

  18. Combined duodenal and jejunal atresia forming a bile filled cyst, presenting as an abdominal mass: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Ahmad Zaghal

    2015-11-01

    Full Text Available The simultaneous occurrence of duodenal and jejunoileal atresia in the newborn is very rare. This is not surprising considering the unrelated embryogenesis behind the development of the two congenital anomalies. We present a case of duodenal atresia associated with an apple peel jejunoileal atresia in a newborn presenting with a prenatal diagnosis of an abdominal cystic mass and a palpable abdominal mass at birth. Exploration revealed a type 3 duodenal atresia followed by a type 3b proximal jejunal atresia (apple peel configuration, with a dilated short segment of bowel between the two. This segment of bowel was the cystic structure detected prenatally and preoperatively on exam. Double foregut atresias should be considered in the differential diagnosis of right upper quadrant cystic masses in the newborn period.

  19. Successful treatment of corrosive esophageal strictures after failed esophageal reconstructions with colon and jejunum

    Institute of Scientific and Technical Information of China (English)

    ZHOU Jing-hai; JIANG Yao-guang; WANG Ru-wen; ZHAO Yun-ping; GONG Tai-qian; TAN Qun-you; MA Zheng; LIN Yi-dan; DENG Bo

    2006-01-01

    Dense and extensive esophageal strictures after caustic agent ingestion require surgical treatment. Colon, stomach and jejunum can be used to reconstruct esophagus. Here, we report an unusual patient with corrosive esophageal stricture who had received unsuccessful esophageal replacements twice at other hospitals. Colon interposition had been first performed 6 months after corrosive esophageal burn,but the colon graft necrosis occurred. Esophageal reconstruction had been carried out 10 years later in another hospital. However, the graft necrosis developed again 5 months later. A salvage operation was performed to remove the necrotic transplant in our hospital. Then as much food as possible had been given to expand the stomach through the gastrostomy since the procedure. The patient underwent esophagectomy and concomitant gastroesophagostomy in the neck 1.5 years later. Esophageal dilations had been performed to prevent recurrent anastomotic stricture for 1 year. He has eaten a normal diet since being discharged.

  20. Growth and development after oesophageal atresia surgery: Need for long-term multidisciplinary follow-up.

    Science.gov (United States)

    IJsselstijn, Hanneke; Gischler, Saskia J; Toussaint, Leontien; Spoel, Marjolein; Zijp, Monique H M van der Cammen-van; Tibboel, Dick

    2016-06-01

    Survival rates in oesophageal atresia patients have reached over 90%. In long-term follow-up studies the focus has shifted from purely surgical or gastrointestinal evaluation to a multidisciplinary approach. We reviewed the literature on the long-term morbidity of these patients and discuss mainly issues of physical growth and neurodevelopment. We conclude that growth problems - both stunting and wasting - are frequently seen, but that sufficient longitudinal data are lacking. Therefore, it is unclear whether catch-up growth into adolescence and adulthood occurs. Data on determinants of growth retardation are also lacking in current literature. Studies on neurodevelopment beyond preschool age are scarce but oesophageal atresia patients seem at risk for academic problems and motor function delay. Many factors contribute to the susceptibility to growth and development problems and we propose a multidisciplinary follow-up schedule into adulthood future care which may help improve quality of life.

  1. Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia

    DEFF Research Database (Denmark)

    Madsen, Stine Skipper; Kvist, Nina; Thorup, Jørgen

    2015-01-01

    cirrhosis. The Danish Health and Medicines Authority (DHMA) demands diagnostic evaluation of children with elevated level of serum bilirubin after two weeks of age. Biliary atresia has to be excluded if conjugated bilirubin level is above than 20 μmol/l, and/or more than 20% of total bilirubin......: During the period, 73 patients where operated with a portoenterostomy ad modum Kasai. Patients older than 84 days at the time of operation were excluded, 54 patients were available for analysis. Conjugated bilirubin in μmol/l and the percentage value were significantly above the DHMA threshold limit......: mean 129.7 μmol/l (42-334 μmol/l) and 73% (28-97%), respectively. CONCLUSION: The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion. FUNDING: none. TRIAL REGISTRATION...

  2. Increased conjugated bilirubin is sufficient to initiate screening for biliary atresia

    DEFF Research Database (Denmark)

    Madsen, Stine Skipper; Kvist, Nina; Thorup, Jørgen

    2015-01-01

    . This percentage value has caused diagnostic trouble over the years. The objective of the present study was to investigate the possibility of changing the recommendations. METHODS: This was a retrospective analysis of the medical records of children operated for biliary atresia in the 1993-2012 period. RESULTS......: During the period, 73 patients where operated with a portoenterostomy ad modum Kasai. Patients older than 84 days at the time of operation were excluded, 54 patients were available for analysis. Conjugated bilirubin in μmol/l and the percentage value were significantly above the DHMA threshold limit......: mean 129.7 μmol/l (42-334 μmol/l) and 73% (28-97%), respectively. CONCLUSION: The total amount of conjugated bilirubin above 20 μmol/l is sufficient to require further evaluation for biliary atresia. The percentage value is unnecessary and may cause confusion. FUNDING: none. TRIAL REGISTRATION...

  3. Ileal atresia and multiple jejunal perforations in a premature neonate with gestational alloimmune liver disease

    Directory of Open Access Journals (Sweden)

    Ryan M. McAdams, MD

    2017-04-01

    Full Text Available Recovery after surgical repair of an ileal atresia with or without intestinal perforation requires prolonged exposure to parenteral nutrition (PN that may lead to PN-associated liver disease. Early liver failure and cholestasis out of proportion for PN exposure may be a harbinger for gestational alloimmune liver disease (GALD, a potentially life-threatening condition that often requires liver transplant if not treated in a timely manner. This case report presents a premature neonate with ileal atresia and multiple jejunal perforations who developed liver failure and was later determined to have GALD. Recognition of clinical and laboratory findings consistent with GALD is essential to promote early treatment, which can enhance neonatal outcomes and impact future pregnancies.

  4. Gastric serosal tear due to congenital pyloric atresia: A rare anomaly, a rare complication

    Directory of Open Access Journals (Sweden)

    Mithat Gunaydin

    2011-01-01

    Full Text Available Congenital pyloric atresia (CPA is a very rare malformation with unknown aetiology. It has has numerous complications including gastric perforation, aspiration pneumonia. Gastric perforations in newborns occur by three mechanisms: trauma, ischaemia, or spontaneous. Here, we report a newborn with CPA presenting with gastric serosal tearing without full-cut gastric perforation. The diagnosis was confirmed with the help of plain abdominal radiograph, ultrasound, contrast-study, and at operation. Treatment of CPA is surgery irrespective of the type of atresia. We performed serosa repair and then the solid, cord-like atretic pylorus was excised with accompanying gastroduodenostomy. Our patient had an uneventful course and was discharged at the end of the second postoperative week.

  5. Cystic lung changes in a thin section CT in an asymptomatic young adult with unilateral pulmonary vein atresia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seung Choul; Yi, Jeong Geun; Park, Jeong Hee [Konkuk Univ. Medical Center, Seoul (Korea, Republic of)

    2012-07-15

    Unilateral pulmonary vein atresia is a rare anomaly, usually associated with symptoms of recurrent hemoptysis and pneumonia in early childhood. Only one report of an asymptomatic adult patient can be found in the literature. We present the case of an asymptomatic patient with unilateral right pulmonary vein atresia in a 20 year old man. Chest radiograph and multidetector computed tomography showed not only pulmonary vein atresia, pulmonary artery hypoplasia, but also cystic lung changes on thin section CT, along with septal and bronchovascular bundle thickening, and ground-glass opacity. Unilateral pulmonary vein atresia could be another disease which can show cystic lung changes on thin section chest CT.

  6. [Laparoscopic surgery for esophageal achalasia].

    Science.gov (United States)

    Ozawa, S; Ando, N; Ohgami, M; Kitagawa, Y; Kitajima, M

    2000-04-01

    Laparoscopic surgery for esophageal achalasia was first reported by Shimi et al. in 1991. Subsequently the procedure has been performed all over the world and laparoscopic Heller myotomy and Dor fundoplication (Heller and Dor operation) is now thought to be the operation of first choice. It is indicated for patients who are resistant to medical therapy (calcium blocker etc.) or have pneumatic dilatation and those with frequent aspiration at night. As Csendes et al. reported that surgical treatment was better than pneumatic dilatation and as laparoscopic surgery is less invasive, the indications for the laparoscopic Heller and Dor operation can include all achalasia patients except those who respond to medical therapy, do not accept surgery, or cannot tolerate surgery. We successfully performed the laparoscopic Heller and Dor operation on 22 patients, all of whom had an uneventful postoperative course. Manometric evaluation, endoscopic examination, and 24-hour pH monitoring showed good results. There are six important technical points: 1) flexible laparoscopy; 2) pneumoperitoneum; 3) gauze in the abdominal cavity to absorb blood; 4) laparosonic coagulating shears; 5) extracorporeal knot-tying technique; and 6) intracorporeal knot-tying technique. If an experienced surgeon is in charge, the laparoscopic Heller and Dor operation is an ideal, minimally invasive treatment for esophageal achalasia.

  7. Updates on esophageal and gastric cancers

    Institute of Scientific and Technical Information of China (English)

    Amy Gallo; Charles Cha

    2006-01-01

    Esophageal and gastric cancers are both common and deadly. Patients present most often after disease progression and survival is therefore poor. Due to demographic variability and recent changes in disease incidence, much emphasis has been placed on studying risk factors for both esophageal and gastric cancers.However, with increasing understanding of these diseases, low survival rates persist and continued intensive studies are necessary to optimize treatment plans. This review article discusses updates in the evolving epidemiology, clinical presentation, risk factors,and diagnostic and treatment modalities of esophageal and gastric cancers.

  8. Esophageal scintigraphy: A comparison with esophagoscopy

    Energy Technology Data Exchange (ETDEWEB)

    Kjellen, G.; Andersson, P.; Sandstroem, S.

    1987-01-01

    Fifty consecutive patients with different esophageal symtoms were investigated with esophageal endoscopy, transit scintigraphy, and gastroesophageal (GE) scintigraphy with extra-abdominal compression. Scintigraphic findings were abnormal in 27 of those 31 patients (87%) who were classified as abnormal at endoscopy. A prolonged transit time was the commonest finding, but hiatal hernia and GE reflux were also found. However, the scintigraphic procedure showed abnormalities in 6 of 19 (31%) patients who were classified as normal at endoscopy. Esophageal scintigraphy is recommended as a screening test before endoscopy is decided on. 20 refs.

  9. A functional study on small intestinal smooth muscles in jejunal atresia

    Directory of Open Access Journals (Sweden)

    Preeti Tyagi

    2016-01-01

    Full Text Available Aim: The present study was aimed to assess the contractile status of neonatal small intestinal smooth muscle of dilated pre-atretic part of intestinal atresia to resolve debatable issues related to mechanisms of persistent dysmotility after surgical repair. Materials and Methods: A total of 34 longitudinally sectioned strips were prepared from pre-atretic dilated part of freshly excised 8 jejunal atresia type III a cases. Spontaneous as well as acetylcholine- and histamine-induced contractions were recorded in vitro by using organ bath preparations. Chemically evoked contractions were further evaluated after application of atropine (muscarinic blocker, pheniramine (H1 blocker, and lignocaine (neuronal blocker to ascertain receptors and neuronal involvement. Histological examinations of strips were made by using Masson trichrome stain to assess the fibrotic changes. Results: All 34 strips, except four showed spontaneous contractions with mean frequency and amplitude of 5.49 ± 0.26/min and 24.41 ± 5.26 g/g wet tissue respectively. The response to ACh was nearly twice as compared to histamine for equimolar concentrations (100 μM. ACh (100 μM induced contractions were attenuated (by 60% by atropine. Histamine (100 μM-induced contractions was blocked by pheniramine (0.32 μM and lignocaine (4 μM by 74% and 78%, respectively. Histopathological examination showed varying degree of fibrotic changes in muscle layers. Conclusions: Pre-atretic dilated part of jejunal atresia retains functional activity but with definitive histopathologic abnormalities. It is suggested that excision of a length of pre-atretic part and early stimulation of peristalsis by locally acting cholinomimetic or H1 agonist may help in reducing postoperative motility problems in atresia patients.

  10. Specific echocardiographic findings useful for the diagnosis of common pulmonary vein atresia

    Directory of Open Access Journals (Sweden)

    Hiroyuki Nagasawa

    2015-12-01

    Full Text Available In this paper, we report a case of common pulmonary vein atresia, which is a very rare disease characterized by cyanosis, heart failure and pulmonary hypertension. Reverse flow in the pulmonary artery at end-diastole as well as in the isthmus of the aorta from early systole to end-diastole detected by echocardiography were found to be specific features useful in diagnosing the disease.

  11. Radiological evaluation of pulmonary atresia: An analysis of cineangiography in 32 cases

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Ho; Yeon, Kyung Mo; Park, Jae Hyung; Han, Man Chung; Yoon, Yong Soo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1983-06-15

    Total 32 cases of pulmonary atresia were diagnosed radiographically at Seoul National University Hospital from March, 1979 to August 1982. Some characteristic radiological findings were analyzed in chest PA and cineangiographies. The results were as follows: 1. In the evaluation of chest PA, cardiomegaly was noticed in 16 cases, dextrocardia in 8 cases, elevated cardiac apex in 7 cases and right-sided aortic arch in 6 cases. The pulmonary vascularties were mildly decreased in 20 cases, markedly decreased in 9 cases and decreased with reticular pattern in 3 cases. 2. As final diagnoses after cineangiography, pulmonary atresia was associated with Tetralogy of Fallot variant in 17 cases, transposition of great vessels in 7 cases, single ventricle in 5 cases, tricuspid atresia in 2 cases and intact ventricular septum in 1 case. 3. The classification according to the pattern of pulmonary artery is main pulmonary trunk with PDA(Type Ia) in 10 cases, pulmonary arterial confluence with PDA (Type Ib) in 10 cases, no pulmonary arterial confluence with PDA (Type Ic) in 5 cases, main pulmonary trunk without PDA (Type IIa) in 0 case, pulmonary arterial confluence without PDA (Type IIb) in 5 cases, and no pulmonary arterial confluence without PDA ( Type IIc) in 2 cases. 4. Pulmonary wedge venography was done and successful in 8 cases. Among them, confluence between right and left pulmonary arteries was noticed in 5 cases. 5. Biventricular cineangiograpy and/or pulmonary wedge venography, if necessary, is essential for the accurate diagnosis of pulmonary atresia to demonstrate detailed anatomy of pulmonary artery.

  12. Large Right Ventricular Clot in Pulmonary Atresia With Intact Ventricular Septum: In Defense of Biventricular Approach.

    Science.gov (United States)

    Dutta, Nilanjan; Ghosh, Rajarshi; Awasthy, Neeraj; Iyer, Parvathi U; Girotra, Sumir; Iyer, Krishna S

    2016-09-01

    Thrombus formation within the right ventricle (RV) in the setting of pulmonary atresia with intact ventricular septum (PAIVS) is not a very common occurrence and can be catastrophic. We present the case of a seven-month-old child with PAIVS and RV clot who successfully underwent biventricular repair. We discuss the interesting case and the rationale for management by means of biventricular repair over single ventricle repair when feasible in such a setting.

  13. Sirenomelia and ndash; Mermaid Syndrome with Oesophageal Atresia: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Veena Raja

    2015-09-01

    Full Text Available Sirenomelia or Mermaid syndrome is a very rare congenital deformity in which legs are fused together and commonly associated with abnormal kidney development, genital, and rectal abnormalities. In this present case, sirenomelia was associated with oesophageal atresia, which is a rare association and occurs in about 20-35% of cases. [J Interdiscipl Histopathol 2015; 3(3.000: 113-116

  14. Destruction of the germinal disc region of an immature preovulatory chicken follicle induces atresia and apoptosis.

    Science.gov (United States)

    Yao, H H; Volentine, K K; Bahr, J M

    1998-09-01

    The germinal disc region (GDR), which contains the germinal disc and overlying granulosa cells, is essential for completion of maturation of the preovulatory chicken follicle. The current study was conducted to test the hypothesis that destruction of the GDR (GDRX) of an immature preovulatory chicken follicle blocks ovulation, induces apoptosis, and causes atresia. The GDR of immature preovulatory follicles (F2) were destroyed by freezing with dry ice (3 mm in diameter) 48-50 h before ovulation. As a control for the effect of freezing, a nonGDR portion (a portion of the follicular wall opposite to the GDR relative to the follicular stalk) of other F2 follicles were destroyed (nonGDRX). Treatment of F2 follicles by GDRX caused atresia and blocked ovulation of all treated follicles (6 of 6), whereas none of the nonGDRX follicles (0 of 5) underwent atresia. Treatment of follicles by GDRX induced apoptotic DNA fragmentation (laddering) in theca and granulosa layers obtained from the frozen area and in the theca layer obtained from the follicular wall distal to the frozen area. In contrast, apoptosis was only present in theca and granulosa layers in the frozen area of the nonGDRX follicle. Furthermore, the in situ DNA end-labeling technique demonstrated that in the GDRX follicle 24 h after treatment, cells in the theca interna, endothelial cells in blood vessels of the theca externa, and a few granulosa cells underwent apoptosis. These results indicate that destruction of the GDR of an immature preovulatory follicle causes atresia and apoptosis and blocks ovulation. These novel findings suggest that the GDR maintains development of the chicken preovulatory follicle by producing one or more survival factors. Without the GDR, chicken follicles cannot develop further and they eventually die.

  15. Equol inhibits growth, induces atresia, and inhibits steroidogenesis of mouse antral follicles in vitro.

    Science.gov (United States)

    Mahalingam, Sharada; Gao, Liying; Gonnering, Marni; Helferich, William; Flaws, Jodi A

    2016-03-15

    Equol is a non-steroidal estrogen metabolite produced by microbial conversion of daidzein, a major soy isoflavone, in the gut of some humans and many animal species. Isoflavones and their metabolites can affect endogenous estradiol production, action, and metabolism, potentially influencing ovarian follicle function. However, no studies have examined the effects of equol on intact ovarian antral follicles, which are responsible for sex steroid synthesis and further development into ovulatory follicles. Thus, the present study tested the hypothesis that equol inhibits antral follicle growth, increases follicle atresia, and inhibits steroidogenesis in the adult mouse ovary. To test this hypothesis, antral follicles isolated from adult CD-1 mice were cultured with vehicle control (dimethyl sulfoxide; DMSO) or equol (600 nM, 6 μM, 36 μM, and 100 μM) for 48 and 96 h. Every 24h, follicle diameters were measured to monitor growth. At 48 and 96 h, the culture medium was subjected to measurement of hormone levels, and the cultured follicles were subjected to gene expression analysis. Additionally, follicles were histologically evaluated for signs of atresia after 96 h of culture. The results indicate that equol (100 μM) inhibited follicle growth, altered the mRNA levels of bcl2-associated X protein and B cell leukemia/lymphoma 2, and induced follicle atresia. Further, equol decreased the levels of estradiol, testosterone, androstenedione, and progesterone, and it decreased mRNA levels of cholesterol side-chain cleavage, steroid 17-α-hydroxalase, and aromatase. Collectively, these data indicate that equol inhibits growth, increases atresia, and inhibits steroidogenesis of cultured mouse antral follicles.

  16. Non-invasive assessment of pulmonary blood supply after staged repair of pulmonary atresia.

    OpenAIRE

    Del Torso, S.; Kelly, M J; Kalff, V; Stellin, G; Mee, R B; Venables, A W

    1985-01-01

    Radionuclide studies were performed to determine pulmonary blood flow in six children who had undergone surgery for pulmonary atresia, ventricular septal defect, and hypoplastic pulmonary arteries with or without major aortopulmonary collateral arteries. Lung blood flow was assessed from both particle perfusion lung scans and the pulmonary and systemic phase of a radionuclide dynamic flow study. Five patients had perfusion defects identified on the particle perfusion lung scan. In three of th...

  17. Complete occipitalization of the atlas with bilateral external auditory canal atresia.

    Science.gov (United States)

    Dolenšek, Janez; Cvetko, Erika; Snoj, Žiga; Meznaric, Marija

    2017-02-18

    Fusion of the atlas with the occipital bone is a rare congenital dysplasia known as occipitalization of the atlas, occipitocervical synostosis, assimilation of the atlas, or atlanto-occipital fusion. It is a component of the paraxial mesodermal maldevelopment and commonly associated with other dysplasias of the craniovertebral junction. External auditory canal atresia or external aural atresia is a rare congenital absence of the external auditory canal. It occurs as the consequence of the maldevelopment of the first pharyngeal cleft due to defects of cranial neural crest cells migration and/or differentiation. It is commonly associated with the dysplasias of the structures derived from the first and second pharyngeal arches including microtia. We present the coexistence of the occipitalization of the atlas and congenital aural atresia, an uncommon combination of the paraxial mesodermal maldevelopment, and defects of cranial neural crest cells. The association is most probably syndromic as minimal diagnostic criteria for the oculoariculovertebral spectrum are fulfilled. From the clinical point of view, it is important to be aware that patients with microtia must obtain also appropriate diagnostic imaging studies of the craniovetebral junction due to eventual concomitant occipitalization of the atlas and frequently associated C1-C2 instability.

  18. A Tutorial on Implantable Hearing Amplification Options for Adults with Unilateral Microtia and Atresia

    Directory of Open Access Journals (Sweden)

    Joannie Ka Yin Yu

    2014-01-01

    Full Text Available Background. Patients with unilateral atresia and microtia encounter problems in sound localization and speech understanding in noise. Although there are four implantable hearing devices available, there is little discussion and evidence on the application of these devices on patients with unilateral atresia and microtia problems. Objective. This paper will review the details of these four implantable hearing devices for the treatment of unilateral atresia. They are percuteaneous osseointegrated bone anchored hearing aid, Vibrant Soundbridge middle ear implant, Bonebridge bone conduction system, and Carina fully implantable hearing device. Methods. Four implantable hearing devices were reviewed and compared. The clinical decision process that led to the recommendation of a device was illustrated by using a case study. Conclusions. The selection of appropriate implantable hearing devices should be based on various factors, including radiological findings and patient preferences, possible surgical complications, whether the device is Food and Drug Administration- (FDA-/CE-approved, and the finances. To ensure the accurate evaluation of candidacy and outcomes, the evaluation methods should be adapted to suite the type of hearing device.

  19. Vibrant Soundbridge(®) in preschool children with unilateral aural atresia: acceptance and benefit.

    Science.gov (United States)

    Leinung, M; Zaretsky, E; Lange, B P; Hoffmann, V; Stöver, T; Hey, C

    2017-01-01

    The aim of this pilot study was to determine the acceptance and benefit from the middle ear implant system Vibrant Soundbridge(®) (VSB, MED-EL Corporation, Austria) by means of a questionnaire, compared to a previously used conventional bone conducting hearing device, in preschool children with unilateral congenital aural atresia. Prospective cohort study. All nine children with unilateral congenital aural atresia used the VSB and had previous experience with a bone conducting hearing device. The benefit from the VSB was evaluated by questionnaires concerning acceptance of hearing aids, handling, listening effort, behavior, quality of life, and the duration of daily use and compared to the experience with the bone conducting hearing device. In addition, to quantify the benefit from the VSB use, audiological assessment (pure-tone audiometry via free field testing, speech audiometry, and localization test) was performed with and without VSB. The questionnaires and audiological test results were compared pairwise. According to all questionnaire areas, children benefited significantly more from the VSB compared to bone conducting hearing device (ps VSB. Children performed significantly better with the VSB than without it in the audiological assessment. Children with unilateral aural atresia benefited significantly more from the VSB compared to a conventional bone conducting hearing device according to the parents' questionnaires and yielded better results in the audiometry and localization test with the VSB than without it.

  20. Congenital cholesteatoma of the infratemporal fossa with congenital aural atresia and mastoiditis: a case report

    Directory of Open Access Journals (Sweden)

    Abdel-Aziz Mosaad

    2012-06-01

    Full Text Available Abstract Background Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis. Case presentation A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma. Conclusions Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology.

  1. Report of congenital colonobladder fitula with atresia ani in a lamb and treatment by surgery

    Directory of Open Access Journals (Sweden)

    Mohsen Vahar

    2015-06-01

    Full Text Available Colonobladder fistula and anal atresia, including congenital malformations in animals, occur due to genetics, environmental factors and vascular insufficiency. A male lamb was brought to a private veterinary clinic in Amol city, Mazandaran province, Iran. The lamb had clinical symptoms such as lethargy, dehydration, closed anus and watery feces in the genital organ. After taking radiograph and clinical examinations, it was found that the lamb was suffering from both colonobladder fistula and anal atresia; and emergency surgery was done. First, aseptic conditions and analgesia at the lumbosacral region of the trunk were put into practice and after cutting the abdominal muscles, accumulated feces in intestine were removed via an intestinal incision. The intestine was separated from the bladder via an incision and the bladder was sutured. Then, after creating an artificial hole in the base of the tail as the animal's new anus, the intestine was sutured to the original anus. At the end of surgery, serum therapy with sugar-salt dextrose 5% NaCl 0.9% solution and antibiotic therapy with penicillin and streptomycin were performed. This report is the first report of an unusual lamb with anal atresia and colonobladder fistula in the north of Iran. This surgery was achievable and is an obligatory surgery in affected animal rather than alternative euthanasia.

  2. Percutaneous recanalization and balloon angioplasty of congenital isolated local atresia of the aortic isthmus in adults.

    Science.gov (United States)

    Joseph, G; Mandalay, A; Rajendiran, G

    2001-08-01

    Congenital isolated local atresia of the aortic isthmus is anatomically similar and morphogenetically related to congenital coarctation of the aorta and was encountered in 4 out of 26 consecutive adult patients selected for coarctation angioplasty at our center. Anterograde recanalization of the atresia was safely and successfully accomplished in all four patients, using a brachial approach. Balloon dilation in the four patients, with ancillary stent implantation in one patient, resulted in reduction of translesion gradient from 84 +/- 11 mm Hg to 9 +/- 7 mm Hg without complications. Angiography in the three non-stented patients after a mean follow-up of 13 months showed no evidence of restenosis, dissection or aneurysm formation, though one patient had mild dilatation of the posterior aspect of the aortic isthmus. The clinical presentation of patients with isolated local aortic atresia, and their short- to mid-term response to percutaneous treatment, is similar to that of patients with isolated severe coarctation of aorta. Copyright 2001 Wiley-Liss, Inc.

  3. New successful one-step surgical repair for apple peel atresia

    Directory of Open Access Journals (Sweden)

    Machmouchi M

    2011-11-01

    Full Text Available Mahmoud MachmouchiDepartment of Pediatrics, Royal Commission Hospital, Jubail, Saudi ArabiaAbstract: A new successful surgical approach in two identical twins delivered with intestinal atresia, “apple peel” type is reported. This technique consists of: (1 an end-to-end oblique primary anastomosis with single layer inverted 5/0 Vicryl® sutures (Ethicon, Inc, Somerville, NJ; (2 proximal tube jejunostomy using Foley catheter, size 10 French, inserted through a stab wound in the left upper quadrant and entering the proximal dilated loop at about 10 cm proximal from its blind end (site of anastomosis; and (3 transanastomotic stenting using feeding tube, size 6 French, exteriorized in conjunction with the Foley catheter and reaching the lumen of the distal loop for more than 20 cm. The postoperative course was uneventful and progressive oral feed became ad libitum around day 40 postoperative. This procedure is simple, performed in one stage, and responds to the most obligatory requirements of this congenital malformation.Keywords: proximal jejunal atresia, intestinal atresia, surgical repair, anastomosis

  4. A STUDY ON CYCLOOXYGENASE -2 PROTEIN EXPRESSION IN ESOPHAGEAL CAICONOGENESIS

    Institute of Scientific and Technical Information of China (English)

    王立峰; 张伟; 王吾如; 王洪平; 韩双廷; 曲平; 刘义; 李茉; 刘伯齐; 林培中

    2001-01-01

    To investigate cyclooxygenase- 2(Cox-2) protein expression in esophageal cancer and precancerous lesions. Methods: One hundred twenty biopsy specimens from esophageal carcinoma and 113 from patients with esophageal premalingnant lesions, 27 from individuals with normal esophageal mucosa and 3 from Barrett's esophagus were examined for Cox-2 protein expression by immunohistochemistry. Results: Cox-2 protein was not observed in normal esophageal squamous and glandular epithelium, hyperplasia from mild to severe dysplasia lesions and carcinoma in situ. Positive Cox-2 protein expression was found in 4 of 60 specimens of invasive squamous-cell carcinomas, 21 of 30 specimens of esophageal adenocarcinomas and in 3 of 3 Barret's esophageal tissues. Conclusion: The Cox-2 protein expression may be associated with the development of the esophageal adenocarcinomas but not esophageal squamous-cell carcinomas.

  5. PET for Staging of Esophageal Cancer

    Institute of Scientific and Technical Information of China (English)

    A.H.Hoelscher

    2004-01-01

    FDG-PET is of clinical value especially for detection of distant metastases or recurrent esophageal cancer. For the staging of primary tumor or locoregional lymph node metastasis PET is currently not suitable.

  6. Prevalence of reflux esophagitis among patients undergoing ...

    African Journals Online (AJOL)

    Ahmed Gado

    2013-10-25

    Oct 25, 2013 ... Age, male sex, obesity, and hiatus hernia (HH) were the purported risk factors for ... esophagus observed to rise proximally from the gastric plica- tions and intestinal .... No case of esophageal cancer was reported in this study.

  7. Esophageal transit scintigraphy in systemic sclerosis.

    Science.gov (United States)

    Chojnowski, Marek; Kobylecka, Małgorzata; Olesińska, Marzena

    2016-01-01

    Systemic sclerosis is a rare connective tissue disease, distinctive features of which are fibrosis and microangiopathy. The esophagus is one of the most commonly involved internal organs. Most patients experience dysphagia, difficulties in swallowing and gastro-esophageal reflux. However, in up to one third of cases, the initial onset of esophageal disease may be clinically silent. There are several diagnostic modalities available for assessing both morphological and functional abnormalities of the esophagus. If structural abnormalities are suspected, endoscopy is the method of choice. Functional evaluation is best achieved with manometry. Both endoscopy and manometry are invasive techniques, with low patient acceptance. Barium-contrast study is well tolerated, but qualitative assessment of functional abnormalities is imprecise. Esophageal scintigraphy is an easy, non-invasive, sensitive and specific diagnostic modality. It can detect esophageal dysfunction even in asymptomatic patients. In patients already diagnosed with systemic sclerosis, scintigraphy is useful in evaluating severity and progression of the disease.

  8. Qualitative and Quantitative Studies of Polygene Proteins Expression in Esophageal Precancerous Lesions and Esophageal Carcinoma

    Institute of Scientific and Technical Information of China (English)

    LI Chao-xia; WU Ming-yao; KUANG Li-ping

    2007-01-01

    Objective: To examine the expressions of MDM2, P53 and P27 proteins in chronic esophagitis, para-cancer mucosa and esophageal carcinoma. Methods: Immunohistochemistry was used to detect the expressions of MDM2, P53 and P27 proteins in forty-seven patients suffering from chronic esophagitis and eighty-five cases of esophageal carcinoma and corresponding para-cancer mucosa. Flow cytometry((FCM) was applied to detect the quantities of these proteins expressed in fresh tissues of 48 cases of esophageal cancer and their para-cancer tissues and 24 cases of relative normal mucosa at the surface of cutting edge. Results: Immunohistochemistry results showed that the expressions of the three studied proteins were very similar in the epithelia of chronic esophagitis and para-cancer mucosa (P>0.05). Both the qualitative and quantitative studies displayed that the P53 protein had no expression and its accumulations would appear only in the early stages of esophagus canceration while the MDM2 and P27 proteins had different degrees of expressions in cases of normal esophageal mucosa. MDM2 protein markedly increased in the advanced stages of esophageal canceration. A quantitative study showed that the expression of P27 protein had a linearity of decreasing tendency (F=9.132,P=0.002) in the course of esophageal canceration. Conclusion: Chronic esophagitis may be a precancerous lesion. Owing to the changes of the P53 and P27 proteins, we can also conclude that these occur in the early stages of esophagus oncogenesis, however the changes of MDM2 expression may occur in the advanced stage of esophageal canceration.

  9. CT findings of esophageal schwannoma: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Man Ho; Ryu, Dae Shick; Eom, Dae Woon; Shin, Dong Rock; Choi, Soo Jung; Ahn, Jae Hong; Park, Man Soo; Yoo, Dong Kon [Gangneung Asan Hospital, College of Medicine, University of Ulsan, Gangneung (Korea, Republic of)

    2015-03-15

    Esophageal schwannomas are a relative rare benign neoplasm that usually occurs in the upper esophagus, in the middle aged women. We report a case of a 67-year-old man with a lower esophageal schwannoma. This lesion was composed of homogenous density, iso-attenuating with the chest wall muscle on pre- and post-contrast chest computed tomography (CT). The CT findings of the esophageal schwannoma are similar to those of esophageal leiomyoma. Hense, esophageal schwannoma may be a differential diagnosis with esophageal leiomyoma.

  10. Dysphagia due to Multiple Esophageal Rings

    Directory of Open Access Journals (Sweden)

    Stephen N Sullivan

    1995-01-01

    Full Text Available A 27-year-old Saudi man with dysphagia due to multiple esophageal rings is reported and the literature reviewed. Dysphagia due to multiple esophageal rings is very rare. Only 15 cases have been reported. The patient is usually male and has had dysphagia for many years when presenting. The cause of the rings is unknown. Theories to explain dysphagia are that the rings are either congenital or an unusual manifestation of gastroesophageal reflux.

  11. Successful tubes treatment of esophageal fistula

    OpenAIRE

    Zhou, Ning; Chen, Wei-Xing; Li, You-ming; Xiang, Zhun; Gao, Ping; Fang, Ying

    2007-01-01

    Aim: To discuss the merits of “tubes treatment” for esophageal fistula (EF). Methods: A 66-year-old female who suffered from a bronchoesophageal and esophagothoratic fistula underwent a successful “three tubes treatment” (close chest drainage, negative pressure suction at the leak, and nasojejunal feeding tube), combination of antibiotics, antacid drugs and nutritional support. Another 55-year-old male patient developed an esophagopleural fistula (EPF) after esophageal carcinoma operation. He...

  12. Herpetic Esophagitis in Immunocompetent Medical Student

    OpenAIRE

    Andréia Vidica Marinho; Vinícius Mendes Bonfim; Luciana Rodrigues De Alencar; Sebastião Alves Pinto; João Alves de Araújo Filho

    2014-01-01

    Esophagitis caused by herpes simplex virus (HSV) is often documented during periods of immunosuppression in patients infected with human immunodeficiency virus (HIV); it is rare in immunocompetent diagnosed patients. Case reports of herpetic esophagitis in students of health sciences are extremely rare. The disease presents with a clinical picture characterized by acute odynophagia and retrosternal pain without obvious causes and ulcers, evidenced endoscopically in the middistal esophagus. Di...

  13. Laryngopharyngeal reflux in patients with reflux esophagitis

    Institute of Scientific and Technical Information of China (English)

    Yung-Chih Lai; Pa-Chun Wang; Jun-Chen Lin

    2008-01-01

    AIM: To assess the prevalence of laryngopharyngeal reflux (LPR) in patients with reflux esophagitis and dis-close factors contributing to the development of LPR.METHODS: A total of 167 patients who proved to have reflux esophagitis by endoscopy were enrolled.ings for the diagnosis of LPR. We used validated ques-tionnaires to identify the presence of laryngopharyn-geal symptoms, and stringent criteria of inclusion to increase the specificity of laryngoscopic findings. The data of patients were analyzed statistically to find out factors related to LPR.RESULTS: The prevalence rate of LPR in studied sub-jects with reflux esophagitis was 23.9%. Age, hoarse-ness and hiatus hernia were factors significantly as-sociated with LPR. In 23 patients with a hiatus hernia,the group with LPR was found to have a lower trend of esophagitis grading.CONCLUSION: Laryngopharyngeal reflux is present in patients with reflux esophagitis, and three predicting factors were identified. However, the development of LPR might be different from that of reflux esophagi-tis. The importance of hiatus hernia deserves further study.

  14. Metabolic syndrome is associated with erosive esophagitis

    Institute of Scientific and Technical Information of China (English)

    Jung Ho Park; Dong IL Park; Hong Joo Kim; Yong Kyun Cho; Chong IL Sohn; Woo Kyu Jeon; Byung Ik Kim

    2008-01-01

    AIM: To clarify whether insulin resistance and metabolic syndrome are risk factors for erosive esophagitis.METHODS: A case-control study was performed using the database of the Kangbuk Semsung Hospital Medical Screening Center.RESULTS: A total of 1679 cases of erosive esophagitis and 3358 randomly selected controls were included.Metabolic syndrome was diagnosed in 21% of the cases and 12% of the controls (P<0.001).Multiple logistic regressions confirmed the association between erosive esophagitis and metabolic syndrome (Odds ratio,1.25; 95% CI,1.04-1.49).Among the components of metabolic syndrome,increased waist circumference,elevated serum triglyceride levels and hypertension were significant risk factors for erosive esophagitis (allP<0.01).Furthermore,increased insulin resistance (Odds ratio,0.91; 95% CI,0.85-0.98)and fatty liver,as diagnosed by ultrasonography (odds ratio,1.39; 95% CI,1.20-1.60),were also related to erosive esophagitis even after adjustment for a series of confounding factors.CONCLUSION: Metabolic syndrome and increased insulin resistance are associated with an increased risk of developing erosive esophagitis.

  15. Congenital ileal atresia presenting as a single cyst-like dilated bowel on prenatal sonography at late third trimester: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Ji, Eun Kyung; Cho, Young A; Kwon, Tae Hee [CHA General Hospital, Pochon CHA University College of Medicine, Pochon (Korea, Republic of)

    2003-12-15

    Small bowel obstruction is suspected when distension of bowel loops are seen on prenatal sonography. However, ileal atresia could show atypical feature that is absent of bowel dilatation. We present a case of ileal atresia that appeared as a single cyst-like dilatation of small bowel on late third trimester prenatal sonography.

  16. Acute iliofemoral venous thrombosis in patients with atresia of the inferior vena cava can be treated successfully with catheter-directed thrombolysis

    DEFF Research Database (Denmark)

    Broholm, Rikke; Jørgensen, Maja; Just, Sven;

    2011-01-01

    To assess the effectiveness and clinical outcomes of catheter-directed thrombolysis in patients with atresia of the inferior vena cava (IVC) and acute iliofemoral deep vein thrombosis (DVT).......To assess the effectiveness and clinical outcomes of catheter-directed thrombolysis in patients with atresia of the inferior vena cava (IVC) and acute iliofemoral deep vein thrombosis (DVT)....

  17. Atresia brônquica congênita: relato de dois casos. Contribuição da tomografia computadorizada ao diagnóstico Congenital bronchial atresia: report of two cases. Contribution of CT scan to diagnosis

    Directory of Open Access Journals (Sweden)

    ALECSANDRA CALIL MOYSES FAURE

    2000-06-01

    Full Text Available Atresia brônquica congênita é uma anomalia rara, caracterizada pela presença de broncocele com hiperinsuflação distal. Relatam-se dois casos dessa anomalia e descrevem-se os achados clínicos e radiológicos que suportam o seu diagnóstico sem a necessidade de uso de métodos invasivos ou cirúrgicos.Bronchial atresia is a rare, congenital anomaly characterized by the presence of bronchocele with distal hyperinflation. The authors report two cases of segmental bronchial atresia and describe the clinical and roentgenographic findings supporting the diagnosis in the absence of other invasive diagnostic modalities or surgical exploration.

  18. Esophageal and Small Intestinal Mucosal Integrity in Eosinophilic Esophagitis and Response to an Elemental Diet.

    Science.gov (United States)

    Warners, Marijn J; Vlieg-Boerstra, Berber J; Verheij, Joanne; van Hamersveld, Patricia H P; van Rhijn, Bram D; Van Ampting, Marleen T J; Harthoorn, Lucien F; de Jonge, Wouter J; Smout, Andreas J P M; Bredenoord, Albert J

    2017-07-01

    The esophageal mucosal integrity is impaired in eosinophilic esophagitis (EoE) and it has been suggested that the duodenal permeability is increased. The absence of food allergens may restore the integrity. The aims of this study were to assess duodenal permeability in EoE and to evaluate the effect of an elemental diet on the esophageal and duodenal integrity. In this prospective study 17 adult EoE patients and 8 healthy controls (HC) were included. Esophageal biopsy specimens were sampled before and after 4 weeks of elemental diet to measure eosinophil counts and gene expression of tight junction and barrier integrity proteins. Esophageal and duodenal impedance were measured by electrical tissue impedance spectroscopy and Ussing chambers were used to measure transepithelial resistance (TER) and transepithelial molecule flux. Small intestinal permeability was measured using a test, measuring lactulose/mannitol (L/M) ratios. In EoE patients, the esophageal but not the duodenal integrity was impaired, compared with HC. We observed no significant difference between L/M ratios of HC and EoE patients. After diet, eosinophil counts decreased significantly, which was paralleled by normalization of esophageal impedance and transepithelial molecule flux. The esophageal TER improved significantly, but did not reach values seen in HC. Esophageal expression of genes encoding for barrier integrity proteins filaggrin and desmoglein-1 was impaired at baseline and restored after diet. An elemental diet restores esophageal integrity, suggesting that it is at least partly secondary to allergen exposure. Duodenal integrity seems not to be affected in EoE, and possibly plays a minor role in its pathophysiology.

  19. Clinical and dosimetric factors of radiation-induced esophageal injury: Radiation-induced esophageal toxicity

    Institute of Scientific and Technical Information of China (English)

    Wen-Bo Qiao; Yan-Hui Zhao; Yan-Bin Zhao; Rui-Zhi Wang

    2005-01-01

    AIM: To analyze the clinical and dosimetric predictive factors for radiation-induced esophageal injury in patients with non-small-cell lung cancer (NSCLC) during threedimensional conformal radiotherapy (3D-CRT). METHODS: We retrospectively analyzed 208 consecutive patients (146 men and 62 women) with NSCLC treated with 3D-CRT. The median age of the patients was 64 years (range 35-87 years). The clinical and treatment parameters including gender, age, performance status, sequential chemotherapy, concurrent chemotherapy, presence of carinal or subcarinal lymph nodes, pretreatment weight loss, mean dose to the entire esophagus, maximal point dose to the esophagus, and percentage of volume of esophagus receiving >55 Gy were studied. Clinical and dosimetric factors for radiation-induced acute and late grade 3-5 esophageal injury were analyzed according to Radiation Therapy Oncology Group (RTOG) criteria.RESULTS: Twenty-five (12%) of the two hundred and eight patients developed acute or late grade 3-5 esophageal injury. Among them, nine patients had both acute and late grade 3-5 esophageal injury, two died of late esophageal perforation. Concurrent chemotherapy and maximal point dose to the esophagus ≥60 Gy were significantly associated with the risk of grade 3-5 esophageal injury. Fifty-four (26%) of the two hundred and eight patients received concurrent chemotherapy. Among them, 25 (46%) developed grade 3-5 esophageal injury (P = 0.0001<0.01). However, nograde 3-5 esophageal injury occurred in patients who received a maximal point dose to the esophagus <60 Gy (P= 0.0001<0.01).CONCLUSION: Concurrent chemotherapy and the maximal esophageal point dose ≥60 Gy are significantly associated with the risk of grade 3-5 esophageal injury in patients with NSCLC treated with 3D-CRT.

  20. Personal Experience in TCM Treatment of Reflux Esophagitis

    Institute of Scientific and Technical Information of China (English)

    洪流

    2003-01-01

    @@ Reflux esophagitis falls into the category of reflux gastroesophagopathy. It is characterized by inflammation, erosion, ulcer formation and fibrosis of the esophageal mucous membrane due to reflux of the content of the stomach and duodenum into the esophagus.

  1. Nutrition in peri-operative esophageal cancer management

    NARCIS (Netherlands)

    Steenhagen, E.; Vulpen, J.K. van; Hillegersberg, R. van; May, A.M.; Siersema, P.D.

    2017-01-01

    INTRODUCTION: Nutritional status and dietary intake are increasingly recognized as essential areas in esophageal cancer management. Nutritional management of esophageal cancer is a continuously evolving field and comprises an interesting area for scientific research. Areas covered: This review

  2. [Current status and perspectives of radiotherapy for esophageal cancer].

    Science.gov (United States)

    Wu, S X; Wang, L H

    2016-09-23

    Esophageal cancer is one of the most common cancers in China. More than 80% of esophageal cancer patients are diagnosed at a late stage and are not eligible for surgery. Radiotherapy is one of the most important modalities in esophageal cancer treatment. Here we reviewed the advances in esophageal cancer radiotherapy and radiotherapy-based combined-modality therapy, such as optimization of radiation dose and target volume, application of precise radiotherapy technique and the integration of radiotherapy with chemotherapy and targeted therapy.

  3. Migrated esophageal stent posing a challenge for ventilation

    Directory of Open Access Journals (Sweden)

    Nita D'souza

    2017-01-01

    Full Text Available Self-expandable esophageal stents are being commonly used for palliative treatment in advanced esophageal cancer patients to relieve dysphagia, prevent tracheoesophageal fistula, and facilitate symptomatic betterment. The modern covered stents reduce the ingrowth of the tumor but have seen an increase in the incidence of stent migrations. We report a rather complicated presentation of an esophageal stent for esophageal dilatation and a challenging management of a difficult tracheostomy.

  4. Effects of anti-hypertensive drugs on esophageal body contraction

    Institute of Scientific and Technical Information of China (English)

    Koichi; Yoshida; Kenji; Furuta; Kyoichi; Adachi; Shunji; Ohara; Terumi; Morita; Takashi; Tanimura; Shuji; Nakata; Masaharu; Miki; Kenji; Koshino; Yoshikazu; Kinoshita

    2010-01-01

    AIM:To clarify the effects of anti-hypertensive drugs on esophageal contraction and determine their possi-ble relationship with gastro-esophageal reflux disease.METHODS:Thirteen healthy male volunteers were enrolled. Esophageal body peristaltic contractions and lower esophageal sphincter (LES) pressure were measured using high resolution manometry. All subjects were randomly examined on four separate occasions following administrations of nifedipine,losartan,and atenolol,as well as without any drug administ...

  5. Spectrum of esophageal abnormality seen on thoracic CT

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kyung Hee; Cho, Soon Gu; Jeon, Yong Sun; Jeong, Seok [Inha university Hospital, Incheon (Korea, Republic of); Kim, Hyung Jin [Samsung Medical Center, Seoul (Korea, Republic of)

    2006-04-15

    A variety of diseases involve the esophagus including esophagitis, benign or malignant tumors, varices, and esophageal perforation. We reviewed the thoracic CT of these various esophageal diseases, and classified them by similar CT findings. The CT findings were circumferential wall thickening, nodular wall thickening, abnormal luminal dilatation, fistula formation, and mass or mass like lesion. Although CT alone has limited diagnostic ability in esophageal disease, it may have an important role in diagnosing submucosal dissection, fistula, perforation, and intramural abscess.

  6. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar Angiographic study of pulmonary circulation in tetralogy of Fallot with pulmonary atresia

    OpenAIRE

    2005-01-01

    OBJETIVO: Identificar os tipos de suprimento sangüíneo vascular pulmonar na tetralogia de Fallot com atresia pulmonar por meio de estudo hemodinâmico. MÉTODOS: Foram submetidos a estudo cineangiocardiográfico 56 pacientes portadores de tetralogia de Fallot com atresia pulmonar com idade de 20 dias a 4 anos e efetuadas injeções de contraste nas seguintes estruturas vasculares: 1) veia pulmonar encunhada, 2) colaterais aortopulmonares, 3) aorta torácica e 4) ductus arteriosus e/ou shunt sistêmi...

  7. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar Angiographic study of pulmonary circulation in tetralogy of Fallot with pulmonary atresia

    Directory of Open Access Journals (Sweden)

    Marco Aurélio Santos

    2005-02-01

    Full Text Available OBJETIVO: Identificar os tipos de suprimento sangüíneo vascular pulmonar na tetralogia de Fallot com atresia pulmonar por meio de estudo hemodinâmico. MÉTODOS: Foram submetidos a estudo cineangiocardiográfico 56 pacientes portadores de tetralogia de Fallot com atresia pulmonar com idade de 20 dias a 4 anos e efetuadas injeções de contraste nas seguintes estruturas vasculares: 1 veia pulmonar encunhada, 2 colaterais aortopulmonares, 3 aorta torácica e 4 ductus arteriosus e/ou shunt sistêmico pulmonar. RESULTADOS: Dos 56 pacientes, 15 tinham o suprimento sangüíneo pulmonar através de colaterais aortopulmonares, em 36 o suprimento sangüíneo pulmonar era feito isoladamente pelo ductus arteriosus e em 5 pelo ductus arteriosus e colaterais aortopulmonares. Conforme a presença ou ausência de estruturas vasculares que compõem a circulação pulmonar na tetralogia de Fallot com atresia pulmonar e do tipo de perfusão vascular pulmonar, os doentes foram classificados em 6 tipos. CONCLUSÃO: Em função da grande complexidade e extrema variabilidade do suprimento sangüíneo pulmonar na tetralogia de Fallot com atresia pulmonar torna-se possível, com este tipo de abordagem, a obtenção de informações, suficientemente necessárias, para o correto manuseio clínico-cirúrgico.OBJECTIVE: To identify the types of pulmonary vascular blood supply in tetralogy of Fallot with pulmonary atresia by use of hemodynamic study. METHODS: Fifty-six patients with tetralogy of Fallot and pulmonary atresia, and ages ranging from 20 days to 4 years, underwent cineangiocardiographic study with contrast medium injections in the following vascular structures: 1 wedged pulmonary vein; 2 aortopulmonary collaterals; 3 thoracic aorta; and 4 ductus arteriosus or systemic-pulmonary shunt. RESULTS: In the 56 patients studied, pulmonary blood was supplied as follows: in 15, by aortopulmonary collaterals; in 36, only by the ductus arteriosus; and in 5, by the ductus

  8. Meconial peritonitis in a rare association of partial ileal apple-peel atresia with small abdominal wall defect

    Directory of Open Access Journals (Sweden)

    V. Insinga

    2014-06-01

    Full Text Available Intestinal atresia type III B (apple peel and gastroschisis are both congenital malformations who require early surgical correction in neonatal age. Their association is very rare. We present the case of a full term infant with partial apple peel ileal atresia and a small defect of the anterior abdominal wall, complicated by in utero intestinal perforation and subsequent meconial peritonitis. We observed a partial atresia of small intestine, with involvement of terminal ileus savings of jejunum and a large part of the proximal ileum, small anterior abdominal wall defect with herniation of few bowel loops, intestinal malrotation. Paralytic ileus and infections are the main causes of morbidity and mortality at neonatal age. In our case, in spite of the mild phenotype, prognosis has been complicated by the onset of functional bowel obstruction, caused by chemical peritonitis resulting from contact with either amniotic fluid and meconium.

  9. Black esophagus (acute esophageal necrosis) after spinal anesthesia.

    Science.gov (United States)

    Román Fernández, A; López Álvarez, A; Fossati Puertas, S; Areán González, I; Varela García, O; Viaño López, P M

    2014-01-01

    Acute esophagic necrosis or black esophagus is an uncommon clinical entity that owes its name to the endoscopic view of the necrotic esophageal mucosa. It is always related with a critical medical condition and usually has an ischemic etiology. We report the first case of acute esophageal necrosis after a spinal anesthetic for partial hip joint arthroplasty. We discuss the underlying pathophysiological mechanisms.

  10. Eosinophilic esophagitis: A newly established cause of dysphagia

    OpenAIRE

    Yan, Brian M; Shaffer, Eldon A.

    2006-01-01

    Eosinophilic esophagitis has rapidly become a recognized entity causing dysphagia in young adults. This review summarizes the current knowledge of eosinophilic esophagitis including the epidemiology, clinical presentation, diagnostic criteria, pathophysiology, treatment, and prognosis. An extensive search of PubMed/Medline (1966-December 2005) for available English literature in humans for eosinophilic esophagitis was completed. Appropriate articles listed in the bibliographies were also atta...

  11. Strategies to improve the outcome of esophagectomy for esophageal cancer

    NARCIS (Netherlands)

    Boone, J.

    2009-01-01

    Esophageal cancer is the 8th most common type of malignancy in the world. For the past decades the incidence of esophageal cancer has rapidly increased, particularly due to a rise in adenocarcinoma of the esophagus. Yet, worldwide the incidence of esophageal squamous cell carcinoma (ESCC) is highest

  12. Translational Research on Esophageal Cancer: From Cell Line to Clinic

    NARCIS (Netherlands)

    J.J. Boonstra (Jurjen)

    2011-01-01

    textabstractWorldwide esophageal cancer is a signifi cant and an increasing health problem. In 2005, there were 497,700 new cases, and the prevalence is expected to increase by approximately 140% by 2025. Esophageal squamous cell carcinoma (ESCC) accounts for most of the cases of esophageal cancer w

  13. Tratamento cirúrgico da atresia de vias biliares: a experiência do hospital municipal jesus, 1997-2000 Surgical treatment of biliary atresia: the hospital municipal jesus experience 1997-2000

    Directory of Open Access Journals (Sweden)

    Lisieux Eyer de Jesus

    2001-04-01

    Full Text Available OBJETIVO: Analisar a indicação, resultados e limitações da portoenterostomia para o tratamento da atresia de vias biliares em hospital infantil terciário no Rio de Janeiro-RJ/Brasil. MÉTODO: Foram estudados prospectivamente oito pacientes submetidos à portoenterostomia do tipo Kasai 1, num período de três anos (1997-2000. Todos foram submetidos à antibioticoterapia profilática, uso de colerético, reavaliação médica mensal nos primeiros seis meses de seguimento e reavaliação laboratorial, ultra-sonográfica e através de endoscopia digestiva alta em prazos programados. RESULTADOS: Em cinco pacientes foi possível obter drenagem biliar estável e satisfatória por > 6 meses pós-operatórios. Quatro faleceram no período de observação (um por insuficiência hepática, um por colangite, dois por hemorragias digestivas, inclusive os três pacientes em que não foi obtida drenagem biliar satisfatória. Quatro persistem em bom estado geral, anictéricos, um deles com fibrose hepática residual grave e provável indicação futura de transplante hepático. Detectamos problemas graves com relação ao encaminhamento tardio de doentes para tratamento e para disponibilizar transplante hepático quando necessário. CONCLUSÕES: Os resultados da portoenterostomia são compensadores quando é possível obter bom débito biliar no pós-operatório. Em pacientes em que não é obtida boa drenagem biliar o pós-operatório é tormentoso e o óbito é esperado até o segundo ano de vida sem o uso de transplante hepático. É necessário disponibilizar transplante hepático pediátrico em nosso meio e conscientizar a comunidade médica para o encaminhamento precoce de bebês ictéricos além dos primeiros 15 dias de vida para investigação e tratamento em unidades especializadas.BACKGROUND: Indications, results and limitations of portoenterostomy to treat biliary atresia in a referral pediatric hospital in Rio de Janeiro, Brazil, are discussed

  14. Woman with surgical reconstruction of anal atresia who realized pregnancy with in vitro fertilisation

    Directory of Open Access Journals (Sweden)

    Garalejić Eliana

    2010-01-01

    Full Text Available Introduction. Anal atresia is a congenital anomaly, very life threatening and urgent. Surgical treatment of this anomaly consists of colostomy first, and then of anal reconstruction. Case report. We presented a 31-year old female with the surgery treatment of anal atresia in the early childhood. In the reproductive period, due to tubal infertily, the patient was included in the program of in vitro fertilization (IVF, in the Clinic for Gynecology and Obstetrics 'Narodni front', Belgrade. Within this program a long protocol of ovarian stimulation was performed. Ultrasonographic and color Doppler monitoring of the patient was applied by the use of an ultrasonographic apparatus type Siemens Acuson X 150, while any hormonal examinations were performed by an Architect Abbott unit. During the IVF program, the growth of follicules was controlled by the use of ultrasonography, microcirculation of the ovaries and the uterus was marked by a power-pulsating color Doppler, and hormonal examination was performed starting from the day of stimulation up to the day of injecting Pregnyl®. The patient was administered Suprefact® (buserelin sc from the 21st day of the menstrual cycle, as well as from the 3rd day of the cycle, for totally 11 days. The patient was given 29 ampoules of Gonal F® (recombinant human FSH 75 IJ im and 15 ampoules of Menopur® (menotrophin im. Due to a modified pelvic anatomy, the left ovary aspiration was disabled, while the right ovary aspired seven oocytes successfully. Three embryos were inserted in the uterus. The delivery was performed by cesarean section. Conclusion. In the reported patient with a modified pelvic anatomy due to four corrective surgeries of anal atresia, and tubal infertility in the reproductive period, the method of choice for the realization of pregnancy was the IVF procedure. The realized pregnancy and the delivery could be considered highly successful in regard to possible risks.

  15. Anatomic Variants on Computed Tomography in Congenital Aural Atresia and Stenosis

    Science.gov (United States)

    Qin, Feng-hua; Dai, Peidong; Yang, Lin

    2015-01-01

    Objectives To quantitatively analyzing the anatomic variants on temporal computed tomography (CT) in congenital external auditory canal stenosis (EACS), congenital aural atresia (CAA), and normal ear structure. Methods Through a retrospective study, we analyzed 142 temporal high-resolution CT studies performed in 71 microtia patients. The following 6 parameters were compared among the three groups: Marx classification, medial canal diameter, vertical facial nerve (VFN) anterior displacement, tegmen mastoideum position, tympanic cavity volume, and malleus-incus joint or malleus-incus complex (MIC) area. Results The results showed that the microtia distributions in the Marx classification in these three groups were significantly different, as 86% (31 of 35) of ears with major microtia (third-degree dysplasia) had an atresia, and in 54.8% (23 of 42) of the minor microtic (first-degree or second-degree) ears, the bony or cartilaginous part of the external auditory canal was stenotic. Measurement data also showed that the potential medial canal diameter of the atresia group was obviously shorter than that of the stenosis group. The VFN anterior displacement and temporomandibular joint backward-shift together lead to medial canal diameters in ears with atresic canals that is smaller than those with stenotic canals. The tegmen mastoideum position was not significantly different between the three groups. Conclusion The mal-development of the external auditory canal is significantly associated with auricle and middle ear developmental anomalies. Compared with CAA ears, EACS have better development of the auricle, canal, tympanic cavity and MIC and relatively safer surgical operation except for the position of the tegmen mastoideum and the VFN. PMID:26622948

  16. Denture mis-swallowing in the sliding esophageal hiatal hernia mimics esophageal perforation.

    Science.gov (United States)

    Chen, Chao-Yang; Lee, Shih-Chun; Chen, Chun-Wen; Chen, Jen-Chih

    2008-08-01

    Mis-swallowing of a foreign body in the esophagus coexisting with sliding hernia might be misdiagnosed as esophageal perforation with mediastinal abscess. We report an 89-year-old woman, bedridden for a long period in a nursing home after a previous cerebrovascular accident, who was sent to our emergency department in a state of sepsis because she had swallowed a radio-opaque partial denture. The retention of the denture as an esophageal foreign body was complicated with mediastinitis and bilateral pleural effusion. The inability of the patient to give a reliable clinical history delayed the diagnosis. This report highlights the difficulty in precisely locating a partial denture because of conflicting radiologic findings and the coexistence of esophageal sliding hernia, all of which led to a misdiagnosis of possible esophageal perforation. A right posterolateral thoracotomy with gastrostomy was performed to remove the lower esophageal foreign body after esophagoscopy failed. The surgical finding of a coincidental sliding esophageal hiatal hernia correlated well with the clinical presentation. Managing such a complicated esophageal foreign body in this elderly patient was challenging.

  17. Esophageal surgery in minimally invasive era

    Institute of Scientific and Technical Information of China (English)

    2016-01-01

    The widespread popularity of new surgical technologiessuch as laparoscopy, thoracoscopy and robotics has ledmany surgeons to treat esophageal diseases with thesemethods. The expected benefits of minimally invasive surgery (MIS) mainly include reductions of postoperativecomplications, length of hospital stay, and pain andbetter cosmetic results. All of these benefits couldpotentially be of great interest when dealing with theesophagus due to the potentially severe complicationsthat can occur after conventional surgery. Moreover,robotic platforms are expected to reduce many of thedifficulties encountered during advanced laparoscopicand thoracoscopic procedures such as anastomoticreconstructions,accurate lymphadenectomies, andvascular sutures. Almost all esophageal diseases areapproachable in a minimally invasive way, includingdiverticula, gastro-esophageal reflux disease, achalasia,perforations and cancer. Nevertheless, while the limitsof MIS for benign esophageal diseases are mainlytechnical issues and costs, oncologic outcomes remainthe cornerstone of any procedure to cure malignancies,for which the long-term results are critical. Furthermore,many of the minimally invasive esophageal operationsshould be compared to pharmacologic interventionsand advanced pure endoscopic procedures; such acomparison requires a difficult literature analysis andleads to some confounding results of clinical trials. Thisreview aims to examine the evidence for the use of MISin both malignancies and more common benign diseaseof the esophagus, with a particular emphasis on futuredevelopments and ongoing areas of research.

  18. The esophageal microbiota in health and disease.

    Science.gov (United States)

    Di Pilato, Vincenzo; Freschi, Giancarlo; Ringressi, Maria Novella; Pallecchi, Lucia; Rossolini, Gian Maria; Bechi, Paolo

    2016-10-01

    The esophageal mucosa is among the sites colonized by human microbiota, the complex microbial ecosystem that colonizes various body surfaces and is increasingly recognized to play roles in several physiological and pathological processes. Our understanding of the composition of the esophageal microbiota in health and disease is challenged by the need for invasive sampling procedures and by the dynamic nature of the esophageal environment and remains limited in comparison with the information available for other body sites. Members of the genus Streptococcus appear to be the major components of the microbiota of the healthy esophagus, although the presence of several other taxa has also been reported. Dysbiosis, consisting of enrichment in some Gram-negative taxa (including Veillonella, Prevotella, Haemophilus, Neisseria, Campylobacter, and Fusobacterium), has been reported in association with gastroesophageal reflux disease and is hypothesized to contribute to the evolution of this condition toward Barrett's esophagus (which is the most common esophageal precancerous lesion) and, eventually, adenocarcinoma. Some Campylobacter species (mostly C. concisus) are also putatively involved in the progression of disease toward adenocarcinoma. However, variable findings have recently been reported in additional studies. Causative relationships between dysbiosis or specific bacterial species and esophageal diseases remain controversial and warrant further investigations. © 2016 The Authors. Annals of the New York Academy of Sciences published by Wiley Periodicals, Inc. on behalf of New York Academy of Sciences.

  19. Multidisciplinary approach for patients with esophageal cancer

    Institute of Scientific and Technical Information of China (English)

    Victoria M Villaflor; Marco E Allaix; Bruce Minsky; Fernando A Herbella; Marco G Patti

    2012-01-01

    Patients with esophageal cancer have a poor prognosis because they often have no symptoms until their disease is advanced.There are no screening recommendations for patients unless they have Barrett's esophagitis or a significant family history of this disease.Often,esophageal cancer is not diagnosed until patients present with dysphagia,odynophagia,anemia or weight loss.When symptoms occur,the stage is often stage Ⅲ or greater.Treatment of patients with very early stage disease is fairly straight forward using only local treatment with surgical resection or endoscopic mucosal resection.The treatment of patients who have locally advanced esophageal cancer is more complex and controversial.Despite multiple trials,treatment recommendations are still unclear due to conflicting data.Sadly,much of our data is difficult to interpret due to many of the trials done have included very heterogeneous groups of patients both histologically as well as anatomically.Additionally,studies have been underpowered or stopped early due to poor accrual.In the United States,concurrent chemoradiotherapy prior to surgical resection has been accepted by many as standard of care in the locally advanced patient.Patients who have metastatic disease are treated palliatively.The aim of this article is to describe the multidisciplinary approach used by an established team at a single high volume center for esophageal cancer,and to review the literature which guides our treatment recommendations.

  20. Estudo angiográfico da circulação pulmonar na tetralogia de Fallot com atresia pulmonar

    OpenAIRE

    2005-01-01

    OBJETIVO: Identificar os tipos de suprimento sangüíneo vascular pulmonar na tetralogia de Fallot com atresia pulmonar por meio de estudo hemodinâmico. MÉTODOS: Foram submetidos a estudo cineangiocardiográfico 56 pacientes portadores de tetralogia de Fallot com atresia pulmonar com idade de 20 dias a 4 anos e efetuadas injeções de contraste nas seguintes estruturas vasculares: 1) veia pulmonar encunhada, 2) colaterais aortopulmonares, 3) aorta torácica e 4) ductus arteriosus e/ou shunt sistêmi...

  1. Congenital pouch colon in a girl associated with bilateral atresia of cervix uteri and uterus didelphys

    Directory of Open Access Journals (Sweden)

    Rajiv Chadha

    2013-01-01

    Full Text Available This report describes a girl with congenital pouch colon (CPC, uterus didelphys with septate vagina, and a cloacal anomaly. The girl underwent cloacal reconstruction at the age of 15 months. Subsequently, at puberty, the child had primary amenorrhea with severe cyclic abdominal pain due to endometriosis of both the uteruses and adnexal cysts with hematometra and hematosalpinx. Laparotomy with removal of both uteri and the left fallopian tube was performed. Both uteri had atresia of the cervix uteri. This report emphasizes the need for comprehensive evaluation and a long-term management strategy for associated gynecologic anomalies in girls with CPC, especially with regard to patency of the outflow tract.

  2. ATRESIA CONGÉNITA DEL OÍDO Y SU MANEJO

    Directory of Open Access Journals (Sweden)

    Dr. Daniel Orfila

    2016-11-01

    Los implantes cocleares siguen siendo los más usados y en las que se tiene mayor experiencia. Las prótesis de conducción ósea implantables o semi implantables cambiaron el manejo de las atresias y malformaciones de oído externo y medio. Pese a lo prometedor que se visualiza el presente y futuro con el uso de estos dispositivos, siempre se debe tener presente que requieren de un acto quirúrgico para su implantación y que no están exentas de complicaciones, por lo cual se debe elegir juiciosamente la prótesis a usar.

  3. 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.

  4. Respiratory Care of Infants and Children with Congenital Tracheo-Oesophageal Fistula and Oesophageal Atresia.

    Science.gov (United States)

    Sadreameli, Sara C; McGrath-Morrow, Sharon A

    2016-01-01

    Despite acute respiratory and chronic respiratory and gastro-intestinal complications, most infants and children with a history of oesophageal atresia / trachea-oesophageal fistula [OA/TOF] can expect to live a fairly normal life. Close multidisciplinary medical and surgical follow-up can identify important co-morbidities whose treatment can improve symptoms and optimize pulmonary and nutritional outcomes. This article will discuss the aetiology, classification, diagnosis and treatment of congenital TOF, with an emphasis on post-surgical respiratory management, recognition of early and late onset complications, and long-term clinical outcomes.

  5. Tratamiento quirúrgico de la atresia de coanas: Caso clínico

    OpenAIRE

    Ágreda M,Beatriz; Urpegui G,Ángel; Vallés V,Héctor

    2013-01-01

    La atresia de coanas es la alteración congénita más frecuente del desarrollo nasal. Puede ser unilateral o bilateral, y según eso, la clínica puede aparecer desde el momento del nacimiento, o bien permanecerá asintomática hasta la edad adulta. En el neonato, si es bilateral, se manifiesta por una disnea inspiratoria y una cianosis cíclica al lactar, que se alivian con el llanto; y en el adulto, la clínica es de insuficiencia respiratoria nasal unilateral, con rinorrea. El tratamiento es quirú...

  6. Biliary Atresia Associated with Polysplenia Syndrome, Dextrocardia, Situs Inversus Totalis and Malrotation of Intestines

    Directory of Open Access Journals (Sweden)

    Praveen Mathur

    2014-01-01

    Full Text Available Biliary atresia (BA is a rare disease and the end result of a destructive, inflammatory cholangiopathy, leading to fibrosis and biliary cirrhosis. It is classified into syndromic variety with various congenital anomalies and non-syndromic (isolated anomaly. We present here a 1-month-old female child with the syndromic variety of BA associated with polysplenia syndrome, dextrocardia, situs inversus totalis and malrotation of intestines. She developed jaundice in the first week of life. Kasai operation was performed but she developed cholangitis and septicemia 2.5 months after surgery and succumbed later.

  7. Airway and esophageal stenting in patients with advanced esophageal cancer and pulmonary involvement.

    Directory of Open Access Journals (Sweden)

    Fabrice Paganin

    Full Text Available BACKGROUND: Most inoperable patients with esophageal-advanced cancer (EGC have a poor prognosis. Esophageal stenting, as part of a palliative therapy management has dramatically improved the quality of live of EGC patients. Airway stenting is generally proposed in case of esophageal stent complication, with a high failure rate. The study was conducted to assess the efficacy and safety of scheduled and non-scheduled airway stenting in case of indicated esophageal stenting for EGC. METHODS AND FINDINGS: The study is an observational study conducted in pulmonary and gastroenterology endoscopy units. Consecutive patients with EGC were referred to endoscopy units. We analyzed the outcome of airway stenting in patients with esophageal stent indication admitted in emergency or with a scheduled intervention. Forty-four patients (58+/-\\-8 years of age with esophageal stenting indication were investigated. Seven patients (group 1 were admitted in emergency due to esophageal stent complication in the airway (4 fistulas, 3 cases with malignant infiltration and compression. Airway stenting failed for 5 patients. Thirty-seven remaining patients had a scheduled stenting procedure (group 2: stent was inserted for 13 patients with tracheal or bronchial malignant infiltration, 12 patients with fistulas, and 12 patients with airway extrinsic compression (preventive indication. Stenting the airway was well tolerated. Life-threatening complications were related to group 1. Overall mean survival was 26+/-10 weeks and was significantly shorter in group 1 (6+/-7.6 weeks than in group 2 (28+/-11 weeks, p<0.001. Scheduled double stenting significantly improved symptoms (95% at day 7 with a low complication rate (13%, and achieved a specific cancer treatment (84% in most cases. CONCLUSION: Stenting the airway should always be considered in case of esophageal stent indication. A multidisciplinary approach with initial airway evaluation improved prognosis and decreased

  8. 2011 update on esophageal achalasia

    Institute of Scientific and Technical Information of China (English)

    Seng-Kee Chuah; Pin-I Hsu; Keng-Liang Wu; Deng-Chyang Wu; Wei-Chen Tai; Chi-Sin Changchien

    2012-01-01

    There have been some breakthroughs in the diagnosis and treatment of esophageal achalasia in the past few years.First,the introduction of high-resolution manometry with pressure topography plotting as a new diagnostic tool has made it possible to classify achalasia into three subtyPes.The most favorable outcome is predicted for patients receiving treatment for type Ⅱ achalasia (achalasia with compression).Patients with type I (classic achalasia) and type Ⅲ achalasia (spastic achalasia) experience a less favorable outcome.Second,the first multicenter randomized controlled trial published by the European Achalasia Trial group reported 2-year follow-up results indicating that laparoscopic Heller myotomy was not superior to endoscopic pneumatic dilation (PD).Although the follow-up period was not long enough to reach a convincing conclusion,it merits the continued use of PD as a generally available technique in gastroenterology.Third,the novel endoscopic technique peroral endoscopic myotomy is a promising option for treating achalasia,but it requires increased experience and cautious evaluation.Despite all this good news,the bottom line is a real breakthrough from the basic studies to identify the actual cause of achalasia that may impede treatment success is still anticipated.

  9. Neoadjuvant Treatment for Esophageal Cancer

    Institute of Scientific and Technical Information of China (English)

    PaulM.Schneider; HuanXi; StephanE.Baldus; JanBrabender; RalfMetzger

    2004-01-01

    Because the conflicting data currently available from the performed randomized trials it is very difficult to provide strict guidelines for the treatment of patients with locoregional advanced esophageal cancers. Surgery however, remains the standard of care for potentially resectable disease. Preoperative chemotherapy is still controversial with two large randomized trials resulting in two different conclusions regarding the survival benefit. Preoperative chemoradiation is also controversial since only one randomized trial showed a clear survival benefit however, the patients treated with surgery alone in this trial had an unusually poor outcome. And the study by Urba et al was not powered enough to show a clear survival benefit for patients treated with neoadjuvant chemoradiation. The results of three metaanalysis of these randomized studies show lower rate of resection, higher rate of R0-resection, more often postoperative mortality and better prognosis for patients with neoadjuvant radiochemotherapy. As a consequence one may consider offering neoadjuvant chemotherapy or neoadjuvant radiochemotherapy to patients with locallyadvanced disease under the premise that patients have a good performance status and understand the controversies about this therapeutic option. Larger trials with sufficient power to clearly detect survival benefits for patients treated with neoadjuvant chemotherapy or radiochemotherapy are necessary before this therapeutic option will be the standard of care.

  10. Reproducibility of esophageal scintigraphy using semi-solid yoghurt

    Energy Technology Data Exchange (ETDEWEB)

    Imai, Yukinori; Kinoshita, Manabu; Asakura, Yasushi; Kakinuma, Tohru; Shimoji, Katsunori; Fujiwara, Kenji; Suzuki, Kenji; Miyamae, Tatsuya [Saitama Medical School, Moroyama (Japan)

    1999-10-01

    Esophageal scintigraphy is a non-invasive method which evaluate esophageal function quantitatively. We applied new technique using semi-solid yoghurt, which can evaluate esophageal function in a sitting position. To evaluate the reproducibility of this method, scintigraphy were performed in 16 healthy volunteers. From the result of four swallows except the first one, the mean coefficients of variation in esophageal transit time and esophageal emptying time were 12.8% and 13.4% respectively (interday variation). As regards the interday variation, this method had also good reproducibility from the result on the 2 separate days. (author)

  11. Minimally invasive surgery for esophageal cancer.

    Science.gov (United States)

    Santillan, Alfredo A; Farma, Jeffrey M; Meredith, Kenneth L; Shah, Nilay R; Kelley, Scott T

    2008-10-01

    Esophageal cancer represents a major public health problem worldwide. Several minimally invasive esophagectomy (MIE) techniques have been described and represent a safe alternative for the surgical management of esophageal cancer in selected centers with high volume and expertise in them. This article reviews the most recent and largest series evaluating MIE techniques. Recent larger series have shown MIE to be equivalent in postoperative morbidity and mortality rates to conventional surgery. MIE has been associated with less blood loss, less postoperative pain, and decreased intensive care unit and hospital length of stay compared with conventional surgery. Despite limited data, conventional surgery and MIE have shown no significant difference in survival, stage for stage. The myriad of MIE techniques complicates the debate of defining the optimal surgical approach for treating esophageal cancer. Randomized controlled trials comparing MIE with conventional open esophagectomy are needed to clarify the ideal procedure with the lowest postoperative morbidity, best quality of life after surgery, and long-term survival.

  12. Herpetic esophagitis: a diagnosis to remember

    Directory of Open Access Journals (Sweden)

    Marina Pinheiro

    2016-02-01

    Full Text Available Introduction: Herpetic esophagitis is a well-recognized infection in immunocompromised hosts, having been rarely described in immunocompetent individuals. Case report: The authors describe a case of a 16-year-old female adolescent admitted to the emergency room with a threeday history of fever, odynophagia, dysphagia for liquid and solid food and retrosternal pain. The upper endoscopy revealed linear and round erosions in the distal esophagus and the histologic findings were compatible with herpetic esophagitis. Discussion/conclusion: Herpetic esophagitis is an underdiagnosed condition in immunocompetent children and adolescents, but it should not be overlooked. An esophagoscopy is required to make a definitive diagnosis. It is usually a selflimited infection and the mainstay of treatment is supportive care. The use of acyclovir is still controversial but its early initiation may shorten the clinical course of the disease.

  13. Esophageal stent migration leads to intestinal obstruction

    Directory of Open Access Journals (Sweden)

    Oguzhan Karatepe

    2009-07-01

    Full Text Available Background: Self-expanding metallic stents are the devices of choice in the treatment of malign or benign strictures of esophagus. Stent migration is a well-known complication of this procedure. Aims: We report a case of intestinal obstruction caused by esophageal stent migration, in which surgical intervention was used. Methods: A 65-year-old woman, who had a medical history of gastric cancer operation and esophageal stent application admitted to our emergeny department with a 48-hour history of abdominal pain, nausea and vomiting. An emergeny laparotomy was performed and the migrated stent leading to intestinal obstruction was removed. Results: The patient recovered without incident and was discharged on postoperative day 3. Conclusion: This case illustrates that esophageal stent migration has to be considered as a potential life-threatening complication.

  14. Herpetic Esophagitis in Immunocompetent Medical Student

    Directory of Open Access Journals (Sweden)

    Andréia Vidica Marinho

    2014-01-01

    Full Text Available Esophagitis caused by herpes simplex virus (HSV is often documented during periods of immunosuppression in patients infected with human immunodeficiency virus (HIV; it is rare in immunocompetent diagnosed patients. Case reports of herpetic esophagitis in students of health sciences are extremely rare. The disease presents with a clinical picture characterized by acute odynophagia and retrosternal pain without obvious causes and ulcers, evidenced endoscopically in the middistal esophagus. Diagnosis depends on endoscopy, biopsies for pathology studies, and immunohistochemistry techniques. The disease course is often benign; however, treatment with acyclovir speeds the disappearance of symptoms and limits the severity of infection. In this report, we present a case of herpetic esophagitis in an immunocompetent medical student, with reference to its clinical features, diagnosis, and treatment. The disease may have manifested as a result of emotional stress experienced by the patient.

  15. Acute esophageal necrosis: a rare syndrome.

    Science.gov (United States)

    Gurvits, Grigoriy E; Shapsis, Alexander; Lau, Nancy; Gualtieri, Nicholas; Robilotti, James G

    2007-01-01

    Acute esophageal necrosis, which presents as a black esophagus on endoscopy, is a rare disorder that is poorly described in the medical literature. In this study, we analyze all cases reported to date to define risk factors, clinical presentation, endoscopic features, histologic appearance, treatment, complications, outcome and etiopathogenesis of the disease and to describe a distinct medical syndrome and propose a staging system. We searched Medline and PubMed from January 1965 to February 2006 for English-language articles using the key words "acute esophageal necrosis," "necrotizing esophagitis," and "black esophagus." A total of 88 patients were reported in the literature during the 40 years, 70 men and 16 women with an average age of 67 years. Patients were generally admitted for gastrointestinal bleeding and cardiovascular event/shock. Patients presented with hematemesis and melena in more than 70% of the cases. Upper endoscopy showed black, diffusely necrotic esophageal mucosa predominantly affecting the distal third of the organ. Necrosis was confirmed histologically in most cases. Complications included strictures or stenoses, mediastinitis/abscesses, and perforations. Overall mortality was 31.8%. This study provides a structured approach to identifying risk factors, diagnosis, and pathogenesis of the acute esophageal necrosis. Risk factors include age, male sex, cardiovascular disease, hemodynamic compromise, gastric outlet obstruction, alcohol ingestion, malnutrition, diabetes, renal insufficiency, hypoxemia, hypercoagulable state, and trauma. Mechanism of damage is usually multifactorial secondary to ischemic compromise, acute gastric outlet obstruction, and malnutrition. Overall, acute esophageal necrosis should be viewed as a poor prognostic factor, associated with high mortality from the underlying clinical disease.

  16. Avoiding complications in esophageal cancer surgery

    DEFF Research Database (Denmark)

    Mortensen, Michael Bau

    2013-01-01

    Modern handling of esophageal cancer patients is based on a multidisciplinary concept, but surgery remains the primary curative treatment modality. Improvements in the perioperative care have reduced the overall morbidity and mortality, but 2-7% of the patients may still die within 30 days...... as a direct consequence of complications related to the esophagectomy procedure. Primarily based on results from randomized studies published after 2000 this review describes some of the factors that may contribute to the development of postoperative complications following esophageal cancer surgery as well...

  17. Esophageal web in Plummer-Vinson syndrome.

    Science.gov (United States)

    Okamura, H; Tsutsumi, S; Inaki, S; Mori, T

    1988-09-01

    In Plummer-Vinson syndrome, esophagography often reveals a web at the anterior wall of the cervical esophagus. The pathogenesis of the esophageal web and the cause of dysphagia in this syndrome were investigated radiographically, endoscopically, manometrically, and histologically. It was considered that the web seen in the esophagogram may have been formed due to the restriction of dilation of the esophageal wall, which results from repetitive inflammation and the subsequent healing process. Dysphagia in this syndrome may be explained by a decrease in swallowing power. Iron deficiency anemia may play the main role in the above histological changes and the resulting decrease in swallowing power.

  18. Esophageal papilloma: Flexible endoscopic ablation byradiofrequency

    Institute of Scientific and Technical Information of China (English)

    Gianmattia del Genio; Federica del Genio; Pietro Schettino; Paolo Limongelli; Salvatore Tolone; Luigi Brusciano; Manuela Avellino; Chiara Vitiello; Giovanni Docimo; Angelo Pezzullo; Ludovico Docimo

    2015-01-01

    Squamous papilloma of the esophagus is a rare benignlesion of the esophagus. Radiofrequency ablation is anestablished endoscopic technique for the eradication ofBarrett esophagus. No cases of endoscopic ablation ofesophageal papilloma by radiofrequency ablation (RFA)have been reported. We report a case of esophagealpapilloma successfully treated with a single sessionof radiofrequency ablation. Endoscopic ablation ofthe lesion was achieved by radiofrequency using anew catheter inserted through the working channelof endoscope. The esophageal ablated tissue wasremoved by a specifically designed cup. Completeablation was confirmed at 3 mo by endoscopy withbiopsies. This case supports feasibility and safety of asa new potential indication for BarrxTM RFA in patientswith esophageal papilloma.

  19. Eosinophilic esophagitis in adults: An update

    Institute of Scientific and Technical Information of China (English)

    Monjur Ahmed

    2016-01-01

    Eosinophilic esophagitis is a worldwide chronic allergic disease of the esophagus.In the last decade,there is an epidemic of this entity in the western world.Mostly seen in children and young adults,patients present with dysphagia or food impaction in the emergency room.Characteristic endoscopic findings,esophageal eosinophilia and non-responsiveness to proton pump inhibitors help make the diagnosis.Avoidance of foodallergens,administration of steroidal anti-inflammatory medications and dilation of the esophagus are the mainstays of treatment.Investigations are ongoing for mucosal healing and optimum maintenance treatment.

  20. Asymptomatic Esophageal Varices Should Be Endoscopically Treated

    Directory of Open Access Journals (Sweden)

    Nib Soehendra

    1998-01-01

    Full Text Available Endoscopic treatment has generally been accepted in the management of bleeding esophageal varices. Both the control of acute variceal bleeding and elective variceal eradication to prevent recurrent bleeding can be achieved via endoscopic methods. In contrast to acute and elective treatment, the role of endoscopic therapy in asymptomatic patients who have never had variceal bleeding remains controversial because of the rather disappointing results obtained from prophylactic sclerotherapy. Most published randomized controlled trials showed that prophylactic sclerotherapy had no effect on survival. In some studies, neither survival rate nor bleeding risk was improved. In this article, the author champions the view that asymptomatic esophageal varices should be endoscopically treated.

  1. Atresia anal, fístula uretrorretal congênita, bolsa escrotal acessória e pseudo-hermafroditismo em bezerro mestiço Anal atresia, congenital urethrorectal fistula, accessory scrotum and pseudohermafroditism in a crossbred calf

    Directory of Open Access Journals (Sweden)

    Thaís Gomes Rocha

    2010-05-01

    Full Text Available Neste trabalho, é descrito o caso de um bezerro mestiço recém-nascido que apresentava atresia anal tipo 2, fístula uretrorretal congênita, bolsa escrotal bífida e pseudo-hermafroditismo masculino. O principal sinal clínico era a eliminação de fezes por meio do óstio prepucial, uma apresentação incomum em casos de fístula uretrorretal em animais machos. Apesar de o quadro de atresia anal ser relativamente comum nessa espécie, os outros defeitos congênitos encontrados são pouco frequentes.In this study, the case of a newborn calf, which presented type 2 anal atresia, congenital urethrorectal fistula, bifid scrotum and male pseudohermafroditism is described. The main clinical sign was the elimination of feces by the prepucial ostium, an unusual finding in cases of urethrorectal fistula in male animals. Although anal atresia is relatively common in bovines, the other congenital defects found in this case are uncommon.

  2. Associação de agenesia sacrococcígea e atresia anal em gato sem raça definida Sacrococcygeal agenesis association and anal atresia in mixed breed cats

    Directory of Open Access Journals (Sweden)

    Felipe Purcell de Araújo

    2009-09-01

    Full Text Available O presente trabalho teve como objetivo descrever o caso de um felino, que desde o nascimento apresentou atresia anal, ausência de cauda e malformação dos membros pélvicos. Ao exame radiográfico, pôde-se observar presença de agenesia da sétima vértebra lombar, sacro e vértebras coccígeas, espinha bífida, meningocele, hiperflexão dos joelhos e desvio valgo dos tarsos, diagnosticando-se agenesia sacrococcígea associada à atresia anal.This paper has the objective to report a case of a cat that since birth had anal atresia, absence of tail and malformation of the pelvic member. The radiographic examination revealed agenesis of the seventh lumbar vertebra, sacral and coccygeal vertebrae, spina bifida, meningocele, hyperflexion of the knees and tarsal valgus deviation, diagnosing sacrococcygeal agenesis associated with anal atresia.

  3. Diagnosis of biliary atresia can not be excluded by declining trend of serum direct bilirubin

    Directory of Open Access Journals (Sweden)

    Keita Terui

    2013-11-01

    Full Text Available The aim of this paper is to investigate the clinical courses of patients with biliary atresia (BA during neonatal period. We examined 19 patients with BA, who underwent blood tests including direct bilirubin (D-Bil within 20 days of age, in 3 tertiary hospitals in Japan. The first blood sample was collected at 8.4±6.5 days of age. The acholic stool was observed within 2 weeks of age in 16 cases (84.2%. Decrease of T-Bil was observed in all the subjects, with a range of reduction of 6.5±3.3 mg/dL, from 10.4±7.5 to 29.8±9.1 days of age. Decrease of D-Bil was also observed in 17 out of 19 cases (89%, with a range of reduction of 1.1±1.0 mg/dL, from 15.5±8.0 to 24.9±9.6 days of age. A significant decrease of D-Bil was observed in 2 cases of biliary atresia splenic malformation syndrome. We therefore conclude that clinicians treating icteric infants should not exclude a diagnosis of BA even if the level of D-Bil has a declining tendency.

  4. Subdural hemorrhage: A unique case involving secondary vitamin K deficiency bleeding due to biliary atresia.

    Science.gov (United States)

    Miyao, Masashi; Abiru, Hitoshi; Ozeki, Munetaka; Kotani, Hirokazu; Tsuruyama, Tatsuaki; Kobayashi, Naho; Omae, Tadaki; Osamura, Toshio; Tamaki, Keiji

    2012-09-10

    Extrahepatic biliary atresia (EHBA) is a rare disease characterized by progressive and obliterative cholangiopathy in infants and is one of the major causes of secondary vitamin K deficiency bleeding (VKDB) due to cholestasis-induced fat malabsorption. Breast feeding increases the tendency of bleeding in EHBA patients because breast milk contains low amounts of vitamin K. A 2-month-old female infant unexpectedly died, with symptoms of vomiting and jaundice prior to death. She had been born by uncomplicated vaginal delivery and exhibited normal growth and development with breastfeeding. There was no history of trauma. She received vitamin K prophylaxis orally. In an emergency hospital, a CT scan showed a right intracranial hematoma and mass effect with midline shift to the left. In the postmortem examination, severe atresia was observed in the whole extrahepatic bile duct. Histologically, cholestasis, periductal fibrosis, and distorted bile ductules were noted. The gallbladder was not identified. A subdural hematoma and cerebellar tonsillar herniation were found; however, no traumatic injury in any part of the body was observed. Together, these findings suggest that the subdural hemorrhage was caused by secondary vitamin K deficiency resulting from a combination of cholestasis-induced fat malabsorption and breastfeeding. Subdural hemorrhage by secondary VKDB sometimes occurs even when vitamin K prophylaxis is continued. This case demonstrated that intrinsic factors, such as secondary VKDB (e.g., EHBA, neonatal hepatitis, chronic diarrhea), should also be considered in infant autopsy cases presenting with subdural hemorrhage.

  5. The contribution of fetal MR imaging to the assessment of oesophageal atresia

    Energy Technology Data Exchange (ETDEWEB)

    Hochart, V.; Verpillat, P.; Bigot, J.; Avni, F.E. [CHRU Lille, Jeanne de Flandre Hospital, Department of Pediatric Radiology, Lille (France); Langlois, C. [EA2694 USDL CHRU Lille, Department of Biostatistics, Lille (France); Garabedian, C.; Debarge, V.H. [CHRU Lille, Jeanne de Flandre Hospital, Department of Obstetrics and Gynecology, Lille (France); Sfeir, R. [CHRU Lille, Jeanne de Flandre Hospital, Department of Pediatric Surgery, Reference Center for Congenital Anomalies of the Esophagus, Lille (France)

    2014-10-11

    Present challenges are to improve the diagnosis rate of oesophageal atresia (OA) and evaluate as completely as possible a fetus affected by OA, specifically the type of OA and the length of the gap. Our aim was to evaluate the accuracy of fetal MR imaging (fMRI) for diagnosis of OA. We reviewed fMRI performed because of sonographic suspicion of an OA. The signs reviewed included stomach size, ''pouch sign'', bowing of the trachea and visualization of the lower oesophageal lumen. The fetuses were assigned by consensus as having or not having EA, as well as having a tracheaoesophageal fistula (TOF). All findings were correlated with postnatal data. Sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were calculated. Se, Sp, PPV and NPV of the technique were respectively 91 %, 100 %, 100 % and 88 %. The presence of the pouch sign yielded corresponding values of 82 %, 100 %, 100 % and 78 %. Mid-tracheal bowing was correlated positively with EA. The type of atresia was correctly evaluated in 90 % of patients. fMRI is useful for the diagnosis of EA through the visualization of the oesophageal pouch or through associated signs such as tracheal bowing. Visualization of the lower oesophageal lumen seems to be a good sign of TEF. (orig.)

  6. Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis.

    Science.gov (United States)

    Miyano, Go; Morita, Keiichi; Kaneshiro, Masakatsu; Miyake, Hiromu; Koyama, Mariko; Nouso, Hiroshi; Yamoto, Masaya; Nakano, Reiji; Tanaka, Yasuhiko; Nishiguchi, Tomizo; Kawamura, Takakazu; Fukumoto, Koji; Urushihara, Naoto

    2015-01-01

    We describe herein a case of unilateral pulmonary agenesis (PA) with oesophageal atresia (EA)/tracheoesophageal fistula (TEF) that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

  7. A novel association of alveolar capillary dysplasia, atypical duodenal atresia, and subglottic stenosis.

    Science.gov (United States)

    Shimizu, Takeru; Fukuda, Taeko; Inomata, Shinichi; Satsumae, Tsuyoshi; Tanaka, Makoto

    2011-04-01

    Alveolar capillary dysplasia (ACD), which is a rare and lethal congenital pulmonary anomaly found in newborns, begins its onset or causes deterioration of the infant's condition some time after birth. Various congenital anomalies in combination with ACD have been reported, except for subglottic stenosis. Therefore, we aim to report a novel association in a case of ACD with the combination of atypical duodenal atresia and subglottic stenosis. The male infant was scheduled for duodeno-duodenostomy because a double-bubble sign was observed on a chest radiograph. He arrived at the operating theater without any symptoms. After induction of general anesthesia, although mask ventilation was performed without difficulties throughout the entire procedure, oxygen saturation values of the upper and lower extremities dissociated after several attempts of intubation. Surgery was canceled because of instability of the respiratory condition. Respiratory insufficiency worsened progressively, and the infant died at 5 days of age. An autopsy confirmed ACD and revealed cartilaginous subglottic stenosis, which had made intubation difficult. This report highlights the hazards of the onset and worsening of ACD, and the importance of thorough echocardiography before surgery when atypical duodenal atresia is suspected. Anesthesiologists should also be prepared for the difficulty of intubation.

  8. Respiratory Distress Secondary to Rhabdomyosarcoma of the Tongue and Co-existent Choanal Atresia.

    Science.gov (United States)

    Chatopadhayay, Rahul; Tiwari, Preeti; Gangopadhyay, A N; Pandey, Vaibhav

    2016-07-01

    Whilst rhabdomyosarcoma (RMS) is the third most common solid tumour in children, congenital RMS of the tongue is extremely rare and usually present as painless progressive mass since birth (Dagher and Helman in Oncologist 4:34-44, 1999; Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126-128, 2010). In neonates, presentation with respiratory distress is unexpected as neonates are preferential nasal breathers and restricted oral breathing due to tumour usually poses no problem. We herein report a case of rhabdomyosarcoma of the tongue with co-existent unilateral choanal atresia, presenting with respiratory distress. The baby developed upper respiratory tract infection following which developed severe respiratory distress. Airway symptoms were precipitated as there was combined obstruction of both the nostrils due to infection or adenoid enlargement and unilateral chonal atresia. Treatment of respiratory distress in the presence of RMS and bilateral nasal pathology must first prioritise the security of the airway, before taking a multi-factorial approach to the therapy of the lingual mass (Childs and Goudy in Int J Pediatr Otorhinolaryngol 5:126-128, 2010). This case illustrates the importance of vigilance with respect to co-existent nasal pathology, in order to avoid the occurrence of complete airway obstruction. We therefore feel that any diagnosis of lingual RMS should warrant a formal examination of both nasal cavities.

  9. Congenital Left Circumflex Coronary Artery Atresia Detected by 64-Slice Computed Tomography: A Case Report

    Directory of Open Access Journals (Sweden)

    Chen-Yuan Liu

    2007-06-01

    Full Text Available A variety of coronary artery disorders, including intramyocardial coronary segments and coronary artery anomalies, can result in sudden cardiac death, especially in young adults. The detection of structural coronary artery abnormalities is important in the management of patients at risk of sudden cardiac death. Coronary artery anomalies occur in about 1% of the population. Congenital absence of left circumflex coronary artery (LCX is a very rare vascular anomaly, and few cases have been reported in the literature, with a frequency of only 0.003% in all patients who underwent coronary angiography. Although coronary catheterization is the gold standard for the evaluation of coronary arterial patency disease, noninvasive computed tomography (CT is considered the diagnostic method of choice for the detection and evaluation of coronary artery anomaly. Herein, we report the case of a 17-year-old girl who presented with exertional dyspnea and chest pain and who was studied at our emergency department with the final diagnosis of LCX atresia detected by 64-slice CT. She may be the first case of congenital LCX atresia proved by multislice CT.

  10. Unilateral pulmonary agenesis associated with oesophageal atresia and tracheoesophageal fistula: A case report with prenatal diagnosis

    Directory of Open Access Journals (Sweden)

    Go Miyano

    2015-01-01

    Full Text Available We describe herein a case of unilateral pulmonary agenesis (PA with oesophageal atresia (EA/tracheoesophageal fistula (TEF that was diagnosed prenatally and repaired by esophagoesophagostomy with stable postoperative course. The patient was born at 34 weeks gestation, after ultrasonography at 22 weeks gestation showed possible right-sided diaphragmatic eventration or PA and EA was subsequently suspected due to hydramnios. The initial X-ray showed mediastinal shift to the right, and coil up sign of the nasogastric tube, without intracardiac anomaly. Immediately after the diagnosis of EA/TEF and unilateral PA on day 0, the patient was intubated in the operating room, and a gastrostomy tube was placed. After pulmonary status stabilized, at 4 days old, EA/TEF was repaired through a thoracotomy in the right 4 th intercostal space. The right main bronchus was noted to continue into the distal oesophagus; this fistula was ligated and divided, and a single-layer esophagoesophagostomy was performed under mild tension with one vertebral gap. The neonate was maintained on mechanical ventilation and gradually weaned to extubation at 7 days old. The postoperative course was uneventful, with the exception of prolonged jaundice that emerged at 3 months old. Laparoscopic cholangiography at that time excluded biliary atresia, and jaundice resolved spontaneously. The patient has not shown any respiratory symptoms or feeding difficulties as of the 12-month follow-up.

  11. Compound heterozygous mutations in TTC7A cause familial multiple intestinal atresias and severe combined immunodeficiency.

    Science.gov (United States)

    Yang, W; Lee, P P W; Thong, M-K; Ramanujam, T M; Shanmugam, A; Koh, M-T; Chan, K-W; Ying, D; Wang, Y; Shen, J J; Yang, J; Lau, Y L

    2015-12-01

    Familial multiple intestinal atresias is an autosomal recessive disease with or without combined immunodeficiency. In the last year, several reports have described mutations in the gene TTC7A as causal to the disease in different populations. However, exact correlation between different genotypes and various phenotypes are not clear. In this study, we report identification of novel compound heterozygous mutations in TTC7A gene in a Malay girl with familial multiple intestinal atresias and severe combined immunodeficiency (MIA-SCID) by whole exome sequencing. We found two mutations in TTC7A: one that destroyed a putative splicing acceptor at the junction of intron 17/exon 18 and one that introduced a stop codon that would truncate the last two amino acids of the encoded protein. Reviewing the recent reports on TTC7A mutations reveals correlation between the position and nature of the mutations with patient survival and clinical manifestations. Examination of public databases also suggests carrier status for healthy individuals, making a case for population screening on this gene, especially in populations with suspected frequent founder mutations.

  12. Karyotype analysis of a male exhibiting Meckel's diverticulum and aural atresia

    Energy Technology Data Exchange (ETDEWEB)

    Frizzell, B.; Hicks, M.F. (David Lipscomb Univ., Nashville, TN (United States))

    Patau's Syndrome is caused by inheritance of an extra chromosome 13. It is characterized primarily by severe mental retardation, cleft palate, and retarded growth. Most fetuses expressing Patau's Syndrome spontaneously abort, and those that are born usually die before one year. Both Meckel's diverticulum and aural atresia are defects found in patients with Patau's at levels higher than those in the general population. An otherwise asymptomatic male expressing only Meckel's diverticulum and aural atresia has a female sibling whose son expressed Patau's syndrome. Twenty percent of patients with Patau's show a translocation of part of chromosome 13 to another D chromosome. If a translocation were the cause of the expression of Patau's in this family, it is possible that the normal male inherited a balanced translocation and the Patau's male received an unbalanced translocation. A karyotype analysis of the non-Patau's male was done to determine if such a translocation were present.

  13. Atresia anal en perros y gatos: conceptos actuales a partir de tres casos clínicos Anal atresia in dogs and cats: the scope from three clinical cases

    Directory of Open Access Journals (Sweden)

    EM García-González

    2012-01-01

    Full Text Available La atresia anal se define como la falta de comunicación del recto y el perineo a través del ano, siendo esta la malformación anorrectal reportada con mayor frecuencia en el perro y el gato. Está asociada a alteraciones en la diferenciación de la cloaca en el embrión en desarrollo; sin embargo los mecanismos fisiopatológicos involucrados hasta el momento no han sido completamente esclarecidos. En el presente documento se expone la experiencia en el diagnóstico y manejo de tres pacientes (dos perros y un gato con atresia anal, presentamos los resultados del análisis de la frecuencia de esta patología en nuestro centro hospitalario, realizamos una revisión detallada de las teorías de los mecanismos fisiopatológicos involucrados en el desarrollo embrionario y con base en estos criterios, sugerimos la clasificación del tipo de atresia anal más apropiada partiendo del análisis de las propuestas existentes y su relación con los conceptos actuales de la anatomía embriológica.Anal atresia is defined as the lack of a complete communication between rectum and the anus, it is the most common anorectal malformation and has been observed mainly in dogs. It has been associated to alterations of anogenital differentiation of the cloaca in the embryo, however, the exact mechanisms remain unclear. Different criteria have been proposed in the literature to classify anal atresia. This study shows the diagnosis, treatment and outcome of three cases (two dogs and one cat with anal atresia. The frequency of this malformation in our hospital is reported as well as a discussion of the theories of the processes involved in the development of anal atresia, and a revised classification for this pathology according to embryo anatomy and development is proposed.

  14. Pseudoepitheliomatous hyperplasia mimicking esophageal squamous cell carcinoma in a patient with lye-induced esophageal stricture.

    Science.gov (United States)

    Han, Jang Soo; Lee, Sang Woo; Suh, Kang Heum; Kim, Seung Young; Hyun, Jong Jin; Jung, Sung Woo; Koo, Ja Seol; Yim, Hyung Joon

    2014-06-01

    Pseudoepitheliomatous hyperplasia is a benign condition that may be caused by prolonged inflammation, chronic infection, and/or neoplastic conditions of the mucous membranes or skin. Due to its histological resemblance to well-differentiated squamous cell carcinoma, pseudoepitheliomatous hyperplasia may occasionally be misdiagnosed as squamous cell carcinoma. The importance of pseudoepitheliomatous hyperplasia is that it is a self-limited condition that must be distinguished from squamous cell carcinoma before invasive treatment. We report here on a rare case of esophageal pseudoepitheliomatous hyperplasia in a 67-year-old Korean woman with a lye-induced esophageal stricture. Although esophageal pseudoepitheliomatous hyperplasia is infrequently encountered, pseudoepitheliomatous hyperplasia should be considered in the differential diagnosis of esophageal lesions.

  15. Expression of heat shock protein 27 in the esophageal tissue of rats with reflux esophagitis

    Institute of Scientific and Technical Information of China (English)

    ZHENG Chao-xu; WANG Zhuo-qing; LIN Wei-bin; CHU Zhong-hua; CHEN Liu-hua; JI Zhuang-qi

    2011-01-01

    Background Little attention has been paid to the expression of heat shock protein 27 (HSP27) in patients with reflux esophagitis (RE), and few studies of the importance of HSP27 in esophagitis have been carried out in animal models.This study aimed to explore the expression of HSP27 in the esophageal tissue of rats with RE. Methods Eighty female Wistar rats were randomly divided into experimental groups A and B and control groups C and D (n=20 in each group). To establish RE, rats in the two experimental groups received pylorus and forestomach ligations,while rats in the control group received gastrostomy and gastric perforation repair. The rats in groups A and C were sacrificed 7 days after surgery, and the rats in groups B and D were sacrificed 14 days after surgery. In groups A and B,10 and 8 rats were diagnosed with RE by pathological examination, respectively (they were included in groups A' and B',respectively). The histopathological diagnosis of all the lower esophageal tissues in groups C and D was normal and 20normal specimens were randomly selected for groups C and D' with 10 specimens in each group. Macroscopic and microscopic esophagitis scores were assessed for the specimens in groups A' and B'. Lower esophageal tissues were collected from groups A', B', C, and D', and paraffin-embedded slices were made using part of the tissues. The expression of HSP27 in the tissues was detected using the two-step streptavidin-peroxidase immunohistochemical method. Some collected tissues were frozen, and expressions of HSP27 mRNA were detected using fluorescence quantitative polymerase chain reaction (FQ-PCR). Results Median macroscopic and microscopic esophagitis scores in groups A' (n=10) and B' (n=8) were 1.0 and 1.5,and 2.0 and 2.5, respectively. There were no significant differences in the macroscopic or microscopic esophagitis scores between the two groups (Z=-0.330, P=0.741; Z=-0.142, P=0.887, respectively). Immunohistochemical staining showed that HSP27 was

  16. Diagnosis of esophageal motility disorders: esophageal pressure topography vs. conventional line tracing

    OpenAIRE

    2015-01-01

    OBJECTIVES: Enhanced characterization of esophageal peristaltic and sphincter function provided by esophageal pressure topography (EPT) offers a potential diagnostic advantage over conventional line tracings (CLT). However, high-resolution manometry (HRM) and EPT require increased equipment costs over conventional systems and evidence demonstrating a significant diagnostic advantage of EPT over CLT is limited. Our aim was to investigate whether the inter-rater agreement and/or accuracy of eso...

  17. Estrogen receptor alpha overexpressing mouse antral follicles are sensitive to atresia induced by methoxychlor and its metabolites.

    Science.gov (United States)

    Paulose, Tessie; Hannon, Patrick R; Peretz, Jackye; Craig, Zelieann R; Flaws, Jodi A

    2012-06-01

    Methoxychlor (MXC) and its metabolites bind to estrogen receptors (ESRs) and increase ovarian atresia. To test whether ESR alpha (ESR1) overexpressing (ESR1 OE) antral follicles are more sensitive to atresia compared to controls, we cultured antral follicles with vehicle, MXC (1-100 μg/ml) or metabolites (0.1-10 μg/ml). Results indicate that MXC and its metabolites significantly increase atresia in ESR1 OE antral follicles at lower doses compared to controls. Activity of pro-apoptotic factor caspase-3/7 was significantly higher in ESR1 OE treated antral follicles compared to controls. ESR1 OE mice dosed with MXC 64 mg/kg/day had an increased percentage of atretic antral follicles compared to controls. Furthermore, pro-caspase-3 levels were found to be significantly lower in ESR1 OE ovaries than controls dosed with MXC 64 mg/kg/day. These data suggest that ESR1 OE ovaries are more sensitive to atresia induced by MXC and its metabolites in vitro and in vivo compared to controls.

  18. Esophageal Granular Cell Tumor and Eosinophilic Esophagitis: Two Interesting Entities Identified in the Same Patient

    Directory of Open Access Journals (Sweden)

    Alfredo J. Lucendo

    2008-02-01

    Full Text Available We illustrate the case of a 41-year-old male with allergic manifestations since childhood. He sought medical attention for intermittent, progressive dysphagia from which he had been suffering for a number of years, having felt the sensation of a retrosternal lump and a self-limited obstruction to the passage of food. Endoscopy detected a submucosal tumor in the upper third of the esophagus, which was typified, via biopsy, as a granular cell tumor with benign characteristics and probably responsible for the symptoms. Two years later, the patient sought medical attention once again as these symptoms had not abated, hence digestive endoscopy was repeated. This revealed stenosis of the junction between the middle and lower thirds of the organ which had not been detected previously but was passable under gentle pressure. Eosinophilic esophagitis was detected after biopsies were taken. Esophageal manometry identified a motor disorder affecting the esophageal body. Following three months of treatment using fluticasone propionate applied topically, the symptoms went into remission, esophageal stenosis disappeared and the esophageal biopsies returned to normal. This is the first documented case of the link between granular cell tumors and Eosinophilic esophagitis, two different disorders which could cause dysphagia in young patients.

  19. Spatial distribution patterns of anorectal atresia/stenosis in China: Use of two-dimensional graph-theoretical clustering

    Institute of Scientific and Technical Information of China (English)

    Ping Yuan; Liang Qiao; Li Dai; Yan-Ping Wang; Guang-Xuan Zhou; Ying Han; Xiao-Xia Liu; Xun Zhang; Yi Cao; Juan Liang; Jun Zhu

    2009-01-01

    AIM:To investigate the spatial distribution patterns of anorectal atresia/stenosis in China.METHODS:Data were collected from the Chinese Birth Defects Monitoring Network (CBDMN),a hospitalbased congenital malformations registry system.All fetuses more than 28 wk of gestation and neonates up to 7 d of age in hospitals within the monitoring sites of the CBDMN were monitored from 2001 to 2005.Two-dimensional graph-theoretical clustering was used to divide monitoring sites of the CBDMN into different clusters according to the average incidences of anorectal atresia/stenosis in the different monitoring sites.RESULTS:The overall average incidence of anorectal atresia/stenosis in China was 3.17 per 10 000 from 2001 to 2005.The areas with the highest average incidences of anorectal atresia/stenosis were almost always focused in Eastern China.The monitoring sites were grouped into 6 clusters of areas.Cluster 1 comprised the monitoring sites in Heilongjiang Province,Jilin Province,and Liaoning Province;Cluster 2 was composed of those in Fujian Province,Guangdong Province,Hainan Province,Guangxi Zhuang Autonomous Region,south Hunan Province,and south Jiangxi Province;Cluster 3 consisted of those in Beijing Municipal City,Tianjin Municipal City,Hebei Province,Shandong Province,north Jiangsu Province,and north Anhui Province;Cluster 4 was made up of those in Zhejiang Province,Shanghai Municipal City,south Anhui Province,south Jiangsu Province,north Hunan Province,north Jiangxi Province,Hubei Province,Henan Province,Shanxi Province and Inner Mongolia Autonomous Region;Cluster 5 consisted of those in Ningxia Hui Autonomous Region,Gansu Province and Qinghai Province;and Cluster 6 included those in Shaanxi Province,Sichuan Province,Chongqing Municipal City,Yunnan Province,Guizhou Province,Xinjiang Uygur Autonomous Province and Tibet Autonomous Region.CONCLUSION:The findings in this research allow the display of the spatial distribution patterns of anorectal atresia/stenosis in

  20. Stent placement for esophageal strictures : an update

    NARCIS (Netherlands)

    Hirdes, Meike Madeleine Catharine; Vleggaar, Frank Paul; Siersema, Peter Derk

    2011-01-01

    The use of stents for esophageal strictures has evolved rapidly over the past 10 years, from rigid plastic tubes to flexible self-expanding metal (SEMS), plastic (SEPS) and biodegradable stents. For the palliative treatment of malignant dysphagia both SEMS and SEPS effectively provide a rapid relief

  1. Acute esophageal necrosis caused by alcohol abuse

    Institute of Scientific and Technical Information of China (English)

    Tetsu Endo; Juichi Sakamoto; Ken Sato; Miyako Takimoto; Koji Shimaya; Tatsuya Mikami; Akihiro Munakata; Tadashi Shimoyama; Shinsaku Fukuda

    2005-01-01

    Acute esophageal necrosis (AEN) is extremely rare and the pathogenesis of this is still unknown. We report a case of AEN caused by alcohol abuse. In our case, the main pathogenesis could be accounted for low systemic perfusion caused by severe alcoholic lactic acidosis. After the healing of AEN, balloon dilatation was effective to manage the stricture.

  2. Esophageal achalasia in adolescence - two case reports

    Directory of Open Access Journals (Sweden)

    Inês Vaz Silva

    2016-02-01

    Full Text Available Introduction: Achalasia is a rare disorder, particularly in pediatrics, characterized by esophageal aperistalsis and inadequate relaxation of the lower esophageal sphincter. Its etiology remains unclear. Cases: We describe two adolescents with dysphagia for solids and liquids, vomiting, weight loss and nocturnal cough for a few months. Initially it was considered to be an eating disorder, and the diagnosis of achalasia was reached later by esophageal manometry in one case and by intraoperative biopsy in another. The patients were submitted to Heller myotomy with an antireflux procedure, laparoscopically in one case, and by laparotomy in another, both with a favorable outcome. Discussion/Conclusions: We emphasize the rarity and diagnostic challenge of these cases. The nonspecific symptoms often lead to the diagnosis of an eating disorder, delaying the correct treatment. If symptoms persist achalasia must be considered and the esophageal manometry is the diagnostic test of choice. The gold standard in treatment is surgical, and we highlight the effectiveness of the techniques applied.

  3. Esophageal cancer awareness in Bomet district, Kenya

    African Journals Online (AJOL)

    EB

    Objective: To determine baseline level of knowledge of esophageal cancer in Bomet District in order to develop targeted ... malignancy in men and the third most common in .... Breast. 8. 10. Colon. 5. 6. “Leg” (as proposed. 4. 5 by interviewee).

  4. Axial force measurement for esophageal function testing

    Institute of Scientific and Technical Information of China (English)

    Flemming H Gravesen; Peter Funch-Jensen; Hans Gregersen; Asbjφrn Mohr Drewes

    2009-01-01

    The esophagus serves to transport food and fluid from the pharynx to the stomach. Manometry has been the "golden standard" for the diagnosis of esophageal motility diseases for many decades. Hence, esophageal function is normally evaluated by means of manometry even though it reflects the squeeze force (force in radial direction) whereas the bolus moves along the length of esophagus in a distal direction. Force measurements in the longitudinal (axial) direction provide a more direct measure of esophageal transport function. The technique used to record axial force has developed from external force transducers over in-vivo strain gauges of various sizes to electrical impedance based measurements. The amplitude and duration of the axial force has been shown to be as reliable as manometry. Normal, as well as abnormal, manometric recordings occur with normal bolus transit, which have been documented using imaging modalities such as radiography and scintigraphy. This inconsistency using manometry has also been documented by axial force recordings. This underlines the lack of information when diagnostics are based on manometry alone. Increasing the volume of a bag mounted on a probe with combined axial force and manometry recordings showed that axial force amplitude increased by 130% in contrast to an increase of 30% using manometry. Using axial force in combination with manometry provides a more complete picture of esophageal motility, and the current paper outlines the advantages of using this method.

  5. Axial force measurement for esophageal function testing.

    Science.gov (United States)

    Gravesen, Flemming H; Funch-Jensen, Peter; Gregersen, Hans; Drewes, Asbjørn Mohr

    2009-01-14

    The esophagus serves to transport food and fluid from the pharynx to the stomach. Manometry has been the "golden standard" for the diagnosis of esophageal motility diseases for many decades. Hence, esophageal function is normally evaluated by means of manometry even though it reflects the squeeze force (force in radial direction) whereas the bolus moves along the length of esophagus in a distal direction. Force measurements in the longitudinal (axial) direction provide a more direct measure of esophageal transport function. The technique used to record axial force has developed from external force transducers over in-vivo strain gauges of various sizes to electrical impedance based measurements. The amplitude and duration of the axial force has been shown to be as reliable as manometry. Normal, as well as abnormal, manometric recordings occur with normal bolus transit, which have been documented using imaging modalities such as radiography and scintigraphy. This inconsistency using manometry has also been documented by axial force recordings. This underlines the lack of information when diagnostics are based on manometry alone. Increasing the volume of a bag mounted on a probe with combined axial force and manometry recordings showed that axial force amplitude increased by 130% in contrast to an increase of 30% using manometry. Using axial force in combination with manometry provides a more complete picture of esophageal motility, and the current paper outlines the advantages of using this method.

  6. [R1 resection of esophageal carcinoma].

    Science.gov (United States)

    Gockel, I; Wittekind, C

    2017-08-02

    The microscopic identification of residual tumor tissue in the oral or aboral resection margins (R1 resection) of esophageal specimens following oncologic esophageal resection, increases the risk of tumor recurrence and disease-related morbidity. Esophageal resection with its associated risks is only meaningful, if an R0 situation can be safely achieved. The relevance of microscopic involvement of the circumferential resection margin (CRM) in esophageal carcinoma in its different definitions by the British and the American Societies of Pathology has up to now never been investigated in a prospective study. According to the German S3 guideline, radiochemotherapy should be performed in a postoperatively proven R1 situation, which cannot be converted by a curative extended re-resection into an R0 situation or in unfavorable conditions for an extended re-resection, independent of neoadjuvant therapy. In the case of an R1 situation in the region of the CRM, an extended re-resection is not simply possible on account of the anatomical conditions with corresponding limitations by the aorta and the spinal column, in contrast to extensions of the re-resection orally or aborally.

  7. Esophageal testing: What we have so far

    Institute of Scientific and Technical Information of China (English)

    2016-01-01

    Gastroesophageal reflux disease (GERD) is a commondisorder of the gastrointestinal tract. In the last fewdecades, new technologies have evolved and havebeen applied to the functional study of the esophagus,allowing for the improvement of our knowledge of thepathophysiology of GERD. High-resolution manometry(HRM) permits greater understanding of the function ofthe esophagogastric junction and the risks associatedwith hiatal hernia. Moreover, HRM has been found tobe more reproducible and sensitive than conventionalwater-perfused manometry to detect the presenceof transient lower esophageal sphincter relaxation.Esophageal 24-h pH-metry with or without combinedimpedance is usually performed in patients withnegative endoscopy and reflux symptoms who havea poor response to anti-reflux medical therapy toassess esophageal acid exposure and symptom-refluxcorrelations. In particular, esophageal 24-h impedanceand pH monitoring can detect acid and non-acid refluxevents. EndoFLIP is a recent technique poorly appliedin clinical practice, although it provides a large amountof information about the esophagogastric junction.In the coming years, laryngopharyngeal symptomscould be evaluated with up and coming non-invasive orminimally invasive techniques, such as pepsin detectionin saliva or pharyngeal pH-metry. Future studies arerequired of these techniques to evaluate their diagnostic accuracy and usefulness, although the available dataare promising.

  8. Esophageal testing: What we have so far.

    Science.gov (United States)

    de Bortoli, Nicola; Martinucci, Irene; Bertani, Lorenzo; Russo, Salvatore; Franchi, Riccardo; Furnari, Manuele; Tolone, Salvatore; Bodini, Giorgia; Bolognesi, Valeria; Bellini, Massimo; Savarino, Vincenzo; Marchi, Santino; Savarino, Edoardo Vincenzo

    2016-02-15

    Gastroesophageal reflux disease (GERD) is a common disorder of the gastrointestinal tract. In the last few decades, new technologies have evolved and have been applied to the functional study of the esophagus, allowing for the improvement of our knowledge of the pathophysiology of GERD. High-resolution manometry (HRM) permits greater understanding of the function of the esophagogastric junction and the risks associated with hiatal hernia. Moreover, HRM has been found to be more reproducible and sensitive than conventional water-perfused manometry to detect the presence of transient lower esophageal sphincter relaxation. Esophageal 24-h pH-metry with or without combined impedance is usually performed in patients with negative endoscopy and reflux symptoms who have a poor response to anti-reflux medical therapy to assess esophageal acid exposure and symptom-reflux correlations. In particular, esophageal 24-h impedance and pH monitoring can detect acid and non-acid reflux events. EndoFLIP is a recent technique poorly applied in clinical practice, although it provides a large amount of information about the esophagogastric junction. In the coming years, laryngopharyngeal symptoms could be evaluated with up and coming non-invasive or minimally invasive techniques, such as pepsin detection in saliva or pharyngeal pH-metry. Future studies are required of these techniques to evaluate their diagnostic accuracy and usefulness, although the available data are promising.

  9. Early detection and determinants of esophageal cancer

    NARCIS (Netherlands)

    Bus, P.

    2014-01-01

    Barrett’s esophagus (BE) is a premalignant condition of the esophagus and characterized by the metaplastic replacement of esophageal squamous epithelium by specialized intestinal-type columnar epithelium. A BE diagnosis is based on endoscopy and histological examination of biopsies taken during endo

  10. The role of HRCT and three-dimensional VR CT findings in patients of congenital atresia combined with microtia.

    Science.gov (United States)

    Gao, Ruzhen; Wang, Yun; Fan, Yue; Ai, Xing; Zhang, Xiaona; Xue, Huadan; Chen, Xiaowei; Jin, Zhengyu

    2012-12-01

    To determine the anatomic differences in patients of atresia by using high-resolution computed tomography (HRCT) and 3D volume rendered (VR) CT. High-resolution computed tomography (HRCT) was performed in 43 atresia patients including 34 unilateral atresia patients (n=34, 26 males, 8 females, mean age 13.82 years, range 8-19 years) and 9 bilateral atresia patients (6 males, 3 females, mean age 13.2 years, range 9-19 years). HRCT and 3D VR findings were compared with those in 43 normal ears of the unilateral atresia patients with normal PTA results (n=34, 26 males, 8 females, mean age 13.82 years, range 8-19 years) and 11 patients with sensorineural hearing loss but with no associated aplasia of the middle and inner ear (n=22, 7 males and 4 females, range 8-20.8 years, median age of 13.4 years) by using the independent one sample T test. On the HRCT images, the angle between the basic line and the tympanic segment of the facial nerve is more acute. And the area of the malleus-incus-joint or the malleus-incus-complex in the diseased ears is smaller than that in the control subjects (PVR CT image. The facial nerve demonstrates abnormal lateral and anterior displacement in the CAA patients and the area of the Malleus-incus-joint and the tympanic cavity are significantly smaller, and the oval window is much narrower in the control group. HRCT and 3D VR CT provide valuable information about preoperative planning of patients with CAA. Measurements of all the angles and length serve as useful adjunct measurements in determining surgical candidacy. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  11. Histomorphological and Immunophenotypic Features of Pill-Induced Esophagitis.

    Directory of Open Access Journals (Sweden)

    Ji Won Kim

    Full Text Available The aim of this study was to investigate histomorphological and immunophenotypic features in pill-induced esophagitis. We comparatively evaluated the histomorphological, immunophenotypic features of pill-induced esophagitis vs. reflux esophagitis, as well as clinical information and endoscopic findings. Fifty-two tissue pieces from 22 cases of pill-induced esophagitis, 46 pieces from 20 reflux esophagitis, and 16 pieces from 14 control samples were subjected to immunohistochemistry for inflammatory infiltrates (CD3 for T lymphocyte, CD20 for B lymphocyte, CD56 for NK cell, CD68 for macrophage, CD117 for mast cell and eosinophil chemotaxis-associated proteins (Erk, leptin, leptin receptor, pSTAT3, phospho-mTOR. As a result, Histomorphology showed that a diffuse pattern of dilated intercellular spaces was more frequently observed in pill-induced esophagitis, while reactive atypia and subepithelial papillary elongation were more often found in reflux esophagitis (P < 0.05, respectively. Interestingly, intraepithelial eosinophilic microabscess, intraepithelial pustule and diffuse pattern of dilated intercellular spaces were observed in 14% (3 cases, 9% (2 cases and 32% (7 cases of pill-induced esophagitis, respectively, but in no cases of reflux esophagitis. Regarding intraepithelial inflammatory infiltrates in pill-induced esophagitis, T lymphocytes were the most common cells, followed by eosinophil; 11 and 7 in one x400 power field, respectively. Intraepithelial pSTAT3-positive pattern was more frequently observed in pill-induced esophagitis than in reflux esophagitis, at 45% (10 cases versus 10% (2 cases, respectively (P < 0.05. Considering the distal esophageal lesion only, intraepithelial pustule, diffuse dilated intercellular spaces and stromal macrophages were more frequently found in distal pill-induced esophagitis, whereas reactive atypia and intraepithelial mast cells in reflux esophagitis (P < 0.05, respectively. In conclusion, diffuse

  12. Restenosis following balloon dilation of benign esophageal stenosis

    Institute of Scientific and Technical Information of China (English)

    Ying-Sheng Cheng; Ming-Hua Li; Ren-Jie Yang; Hui-Zhen Zhang; Zai-Xian Ding; Qi-Xin Zhuang; Zhi-Ming Jiang; Ke-Zhong Shang

    2003-01-01

    AIM: To elucidate the mechanism of restenosis following balloon dilation of benign esophageal stenosis.METHODS: A total of 49 rats with esophageal stenosis were induced in 70 rats using 5 ml of 50 % sodium hydroxide solution and the double-balloon method, and an esophageal restenosis (RS) model was developed by esophageal stenosis using dilation of a percutaneous transluminal coronary angioplasty (PTCA) balloon catheter. These 49 rats were divided into two groups: rats with benign esophageal stricture caused by chemical burn only (control group, n=21) and rats with their esophageal stricture treated with balloon catheter dilation (experimental group, n=28). Imaging analysis and immunohistochemistry were used for both quantitative and qualitative analyses of esophageal stenosis and RS formation in the rats, respectively.RESULTS: Cross-sectional areas and perimeters of the esophageal mucosa layer, muscle layer, and the entire esophageal layers increased significantly in the experimental group compared with the control group. Proliferating cell nuclear antigen (PCNA) was expressed on the 5th day after dilation, and was still present at 1 month. Fibronectin (FN)was expressed on the 1st day after dilation, and was still present at 1 month.CONCLUSION: Expression of PCNA and FN plays an important role in RS after balloon dilation of benign esophageal stenosis.

  13. DIAGNOSTIC VALUE OF MINIPROBE ULTRASONOGRAPHY IN ESOPHAGEAL PROTRUDING LESIONS

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objective To investigate the diagnostic value of miniprobe ultrasonography (MPS) in the protruding lesions of esophagus. Methods Sixty-two patients with protruding lesions of esophagus were examined with MPS. Results The MPS examination showed diseases of esophageal polyp, inflammatory protruding, esophageal leiomyoma, esophageal leiomyosarcoma, esophageal venous aneurysm, esophageal lipoma, esophageal cyst, esophageal carcinoma, and extra esophageal compression including aorta compression, lung tumor compression and spina compression. Fourteen patients were verified by surgical operations and pathological examinations, resulting in 92.86% (13/14) diagnosis accuracy rate. Forty-eight cases had results of gastroscope examination consistent with that of MPS. Twelve cases had results of CT and MRI examination consistent with that of MPS. Follow-up was completed on 22 patients. Conclusion Besides imaging the esophageal layer, MPS can precisely locate the histological layer of the esophageal protruding lesions and predict their characters. This indicates its value in the diagnosis and differential diagnosis of the protruding lesions of esophagus. Furthermore, the miniature probe used in MPS examination can pass the structured esophagus, enabling wider application of the examination.

  14. Effect of age on proximal esophageal response to swallowing

    Directory of Open Access Journals (Sweden)

    Roberto Oliveira Dantas

    2010-12-01

    Full Text Available CONTEXT: It has been demonstrated that the ageing process affects esophageal motility. OBJECTIVES: To evaluate the effect of the age on the proximal esophageal response to wet swallows. METHOD: We measured the proximal esophageal response to swallows of a 5 mL bolus of water in 69 healthy volunteers, 20 of them aged 18-30 years (group I, 27 aged 31-50 years (group II, and 22 aged 51-74 years (group III. We used the manometric method with continuous perfusion. The proximal esophageal contractions were recorded 5 cm from a pharyngeal recording site located 1 cm above the upper esophageal sphincter. The time between the onset of the pharyngeal and of the proximal esophageal recording (pharyngeal-esophageal time and the amplitude, duration and area under the curve of the proximal esophageal contraction were measured. RESULTS: The pharyngeal-esophageal time was shorter in group I subjects than in group II and III subjects (P<0.05. The duration of proximal esophageal contractions was longer in group I than in groups II and III (P<0.001. There was no differences between groups in the amplitude or area under the curve of contractions. There were no differences between groups II and III for any of the measurements. CONCLUSION: We conclude that the age may affects the response of the proximal esophagus to wet swallows.

  15. Esophageal Cancer in Iran: A Review

    Directory of Open Access Journals (Sweden)

    Siavosh Nasseri-Moghaddam

    2010-01-01

    Full Text Available Esophageal cancer is the second and third most common malignancy in Iranian malesand females, respectively, claiming lives of approximately 5800 Iranians each year.Squamous cell carcinoma (SCC is presently the most common type accounting forabout 90% of all esophageal cancers in Iran. Recent studies have shown that there isa gradual increase in the incidence of adenocarcinoma of the distal esophagus alongwith gastric cardia adenocarcinoma. Thirty-five years ago, the age standardizied rate (ASR of esophageal SCC in thecity of Gonbad (Golestan Province, northeast of Iran was found to be one of the highestrates for any single cancer that had been reported worldwide (ASR >100/105/year.Recent studies have shown that the incidence of SCC in Gonbad has declined to lessthan half of what it was in the past. This decline in the incidence of esophageal SCCparallels an improvement in the socioeconomic situation of people living in thisregion. According to recent cancer registry data in Iran there is still an obviousintracountry variability between the incidence of esophageal cancer in the south withan ASR of 3 for males and 2 for females in Kerman and 43 and 36 in the northeasternprovince of Golestan. The reasons for this very high rate of SCC in northeastern Iranhave been the subject of several studies during the past 35 years. According to resultsof these studies the suspected risk factors are: low intake of fruits and vegetables, drinkinghot tea, consumption of opium products and tobacco, H.pyloriinfection in the stomach,using unhealthy drinking water from cisterns and genetic susceptibility. The mainsuspected mutagens are polycyclic aromatic hydrocarbons (PAH and N-nitrosocompounds. In order to embark primary and secondary prevention of this fatal cancer,further prospective studies are presently underway in the region. The Golestanesophageal cancer cohort study which follows of 50,000 subjects is on going. We expectsimple and feasible evidence based

  16. with esophageal squamous cell cancer

    Directory of Open Access Journals (Sweden)

    Tao Li

    2017-02-01

    Full Text Available Purpose: The aim of this study was to retrospectively observe and analyze the long-term treatment outcomes of 191 elderly patients with esophageal squamous cell cancer (ESCC who were treated with californium-252 (252Cf neutron brachytherapy (NBT in combination with external beam radiotherapy (EBRT. Material and methods : From January 2002 to November 2012, 191 patients with ESCC underwent NBT in combination with EBRT. The total radiation dose to the reference point via NBT was 8-25 Gy-eq in two to five fractions with one fraction per week. The total dose via EBRT was 50-60 Gy, which was delivered over a period of 5 to 6 weeks with normal fractionation. Results : The median survival time for the 191 patients was 23.6 months, and the 5-year rates for overall survival (OS and local-regional control (LRC were 28.7% and 54.2%, respectively. The patients’ age was a factor that was significantly associated with OS (p = 0.010, according to univariate analysis. The 5-year OS (LRC was 37.3% (58.6% for patients aged 70-74 years and 14.5% (47.9% for patients aged > 74 years (p = 0.010 and p = 0.038. In multivariate analysis, age and clinical N stage were associated with OS and LRC (p = 0.011 [0.041] and p = 0.005 [0.005]. From the time of treatment completion to the development of local-regional recurrence or death, 5 (2.6% patients experienced fistula and 15 (7.9% experienced massive bleeding. The incidence of severe late complications was related to older age (p = 0.027, higher NBT dose/fraction (20-25 Gy/5 fractions, and higher total dose (> 66 Gy. Conclusions : The clinical data indicated that NBT in combination with EBRT produced favorable local control and long-term survival rates for elderly patients with ESCC, and that the side effects were tolerable. Patient’s age, clinical stage N status, and radiation dose could be used to select the appropriate treatment for elderly patients.

  17. Sphingosine-1-phosphate and ceramide are associated with health and atresia of bovine ovarian antral follicles.

    Science.gov (United States)

    Hernández-Coronado, C G; Guzmán, A; Espinosa-Cervantes, R; Romano, M C; Verde-Calvo, J R; Rosales-Torres, A M

    2015-02-01

    The follicle destiny towards ovulation or atresia is multi-factorial in nature and involves outcries, paracrine and endocrine factors that promote cell proliferation and survival (development) or unchain apoptosis as part of the atresia process. In several types of cells, sphingosine-1-phospate (S1P) promotes cellular proliferation and survival, whereas ceramide (CER) triggers cell death, and the S1P/CER ratio may determine the fate of the cell. The aim of present study was to quantify S1P and CER concentrations and their ratio in bovine antral follicles of 8 to 17 mm classified as healthy and atretic antral follicles. Follicles were dissected from cow ovaries collected from a local abattoir. The theca cell layer, the granulosa cells and follicular fluid were separated, and 17β-estradiol (E2) and progesterone (P4) concentrations were measured in the follicular fluid by radioimmunoassay. Based on the E2/P4 ratio, the follicles were classified as healthy (2.2±0.3) or atretic (0.2±0.3). In both follicular compartments (granulosa and theca cell layer), sphingolipids were extracted and S1P and CER concentrations were quantified by HPLC (XTerra RP18; 5 µm, 3.0×150 mm column). Results showed that in both follicular compartments, S1P concentrations were higher in healthy antral follicles than in atretic antral follicles (P<0.05). The concentration of CER in the granulosa cells was higher in atretic antral follicles than in healthy antral follicles, but no differences were observed in the theca cell layer. The S1P/CER ratio in both follicular compartments was also higher in healthy antral follicles. Interestingly, in these follicles, there was a 45-fold greater concentration of S1P than CER in the granulosa cells (P<0.05), whereas in the theca cell layer, S1P had only a 14-fold greater concentration than CER when compared with atretic antral follicles. These results suggest that S1P plays a role in follicle health, increasing cellular proliferation and survival. In

  18. Esophageal Manometry in Patients with Chest Pain and Normal Coronary Arteriogram.

    Science.gov (United States)

    Ferguson, S C; Hodges, K; Hersh, T; Jinich, H

    1981-02-01

    Evaluation of the esophagus is helpful in determining the source of chest pain. Eighteen per cent of 72 patients with a normal coronary angiogram had esophageal disease as a source of chest pain. Eight had diffuse esophageal spasm, four had reflux esophagitis and one had an esophageal ulcer. Five of eight patients with diffuse esophageal spasm had relief of symptoms with nitroglycerin. Despite normal coronary arteriogram and normal esophageal manometry 42 of 49 other patients had relief of chest pain with nitroglycerin.

  19. First successful repair of an aortico-to-right ventricular tunnel (ARVT) in d-transposition of the great arteries with aortic valve atresia and ventricular septal defect.

    Science.gov (United States)

    Wagner, Robert; Vollroth, Marcel; Daehnert, Ingo; Kostelka, Martin

    2015-04-01

    The presented case reports on the first successful complex biventricular repair in a neonate with an aortico-to-right ventricular tunnel and dextrotransposition of the great arteries complicated by aortic atresia.

  20. Epidemiologic differences in esophageal cancer between Asian and Western populations

    Institute of Scientific and Technical Information of China (English)

    Han-Ze Zhang; Guang-Fu Jin; Hong-Bing Shen

    2012-01-01

    Esophageal cancer is a common cancer worldwide and has a poor prognosis.The incidence of esophageal squamous cell cancer has been decreasing,whereas the incidence of esophageal adenocarcinoma has been increasing rapidly,particularly in Western men.Squamous cell cancer continues to be the major type of esophageal cancer in Asia,and the main risk factors include tobacco smoking,alcohol consumption,hot beverage drinking,and poor nutrition.In contrast,esophageal adenocarcinoma predominately affects the whites,and the risk factors include smoking,obesity,and gastroesophageal reflux disease.In addition,Asians and Caucasians may have different susceptibilities to esophageal cancer due to different heritage backgrounds.However,comparison studies between these two populations are limited and need to be addressed in the near future.Ethnic differences should he taken into account in preventive and clinical practices.

  1. Detection of esophageal ulcerations with technetium-99m albumin sucralfate

    Energy Technology Data Exchange (ETDEWEB)

    Goff, J.S.; Adcock, K.A.; Schmelter, R.

    1986-07-01

    Technetium-99m albumin-sucralfate ((/sup 99m/Tc)Su) can be used to demonstrate peptic ulcer disease in man and animals. We evaluated the usefulness of (/sup 99m/Tc)Su for detecting various grades of esophagitis. (/sup 99m/Tc)Su adhered to the distal esophagus for up to 3 hr in five of six patients with esophageal ulcers but adhered to only two of nine with lesser degrees of esophagitis. No adherence was seen in five patients without esophagitis. Thus, (/sup 99m/Tc)Su may not be useful for detecting any but the most severe grade of esophagitis. Based on these results, we speculate that the previously documented beneficial effects of sucralfate on mild to moderate esophagitis may be due to other mechanisms besides adherence to the ulcerated mucosa.

  2. Esophageal clearance scintigraphy in, diabetic patients; A preliminary study

    Energy Technology Data Exchange (ETDEWEB)

    Karayalcin, B.; Karayalcin, U.; Aburano, Tamio; Nakajima, Kenichi; Hisada, Kinichi; Morise, Toshio; Okada, Toshihide; Takeda, Ryoyu (Kanazawa Univ. (Japan). School of Medicine)

    1992-05-01

    The aim of this preliminary study was to evaluate the predictive value of esophageal clearance scintigraphy (ECS) in the diagnosis of esophageal autonomic neuropathy in diabetic patients without any esophageal symptoms. A single swallon ECS was performed in 12 diabetic patients and 15 normal volunteers, and esophageal transit time (ETT) and esophageal (Es) T 1/2 values were calculated. ETT and Es 1/2 were found to be significantly prolonged in the diabetic group (p<0.01 and p<0.05, respectively). In this preliminary study, our results strongly suggest that ECS may be an important noninvasive diagnostic tool in the evaluation of diabetic patients with asymptomatic esophageal autonomic neuropathy. (author).

  3. Horizontal ventricular septum with dextroversion: hearts with and without aortic atresia.

    Science.gov (United States)

    Thilenius, O G; Bharati, S; Lev, M; Karp, R B; Arcilla, R A

    1987-01-01

    Two hearts with horizontal ventricular septum, dextroversion (situs solitus), ventricular septal defects, and malaligned great vessels are reported. One of the hearts had aortic atresia and the infant died; the other patient had a Fontan-type physiologic correction. Reviewing the literature, the following conclusions are drawn: (a) Hearts with horizontal ventricular septum and those with criss-cross atrioventricular connections may be the result of different degrees of rotation of the ventricular muscle mass. This rotation is not likely to be postseptational but preseptational. (b) Only those hearts with a complete 180 degrees rotation should be called criss-cross hearts. (c) Partial rotation results in a horizontal septum such that the right ventricle is invariably superior, regardless of atrioventricular concordance or discordance, situs solitus or inversus, or dextroversion. (d) Physiologic surgical correction is often possible but has to be tailored to the details of each heart.

  4. External auditory canal atresia of probable congenital origin in a dog.

    Science.gov (United States)

    Schmidt, K; Piaia, T; Bertolini, G; De Lorenzi, D

    2007-04-01

    A nine-month-old Labrador retriever was referred to the Clinica Veterinaria Privata San Marco because of frequent headshaking and downward turning of the right ear. Clinical examination revealed that there was no external acoustic meatus in the right ear. Computed tomography confirmed that the vertical part of the right auditory canal ended blindly, providing a diagnosis of external auditory canal atresia. Cytological examination and culture of fluid from the canal and the bulla revealed only aseptic cerumen; for this reason, it was assumed that the dog was probably affected by a congenital developmental deformity of the external auditory canal. Reconstructive surgery was performed using a "pull-through" technique. Four months after surgery the cosmetic and functional results were satisfactory.

  5. Pulmonary Atresia with Intact Ventricular Septum and Absence of Tricuspid Valve. A Report of Four Cases

    Directory of Open Access Journals (Sweden)

    Adel E. González Morejón

    2013-12-01

    Full Text Available Pulmonary atresia with intact ventricular septum suggests a simple congenital heart malformation. However, nothing could be further from the truth. Among the morphological peculiarities of this condition, the presence of congenitally unguarded tricuspid valve orifice is rarely observed. The present paper aims at describing a series of four patients with both malformations detected in the William Soler Children’s Heart Center from 1986 through 2012. Echocardiographic diagnosis could be accurately performed in the last two patients; identification of the initial cases was established by the findings at necropsy. All patients underwent Blalock – Taussig shunt as initial palliative treatment, which was associated with balloon atrioseptostomy in two cases. Only one of the last children mentioned survived. Vital prognosis of patients suffering from this association of malformations lies in early echocardiographic detection.

  6. Incidental Finding of Inferior Vena Cava Atresia Presenting with Deep Venous Thrombosis following Physical Exertion

    Directory of Open Access Journals (Sweden)

    Shalini Koppisetty

    2015-01-01

    Full Text Available Inferior vena cava atresia (IVCA is a rare but well described vascular anomaly. It is a rare risk factor for deep venous thrombosis (DVT, found in approximately 5% of cases of unprovoked lower extremity (LE DVT in patients <30 years of age. Affected population is in the early thirties, predominantly male, often with a history of major physical exertion and presents with extensive or bilateral DVTs. Patients with IVC anomalies usually develop compensatory circulation through the collateral veins with enlarged azygous/hemizygous veins. Despite the compensatory circulation, the venous drainage of the lower limbs is often insufficient leading to venous stasis and thrombosis. We describe a case of extensive and bilateral deep venous thrombosis following physical exertion in a thirty-six-year-old male patient with incidental finding of IVCA on imaging.

  7. Methoxychlor induces atresia of antral follicles in ERalpha-overexpressing mice.

    Science.gov (United States)

    Tomic, Dragana; Frech, Maria Silvina; Babus, Janice K; Gupta, Rupesh K; Furth, Priscilla A; Koos, Robert D; Flaws, Jodi A

    2006-09-01

    Methoxychlor (MXC) is a pesticide that is known to bind to estrogen receptor alpha (ERalpha) and to induce atresia of antral ovarian follicles. Although studies have shown that MXC is toxic to the ovary, we hypothesize that perturbation to the estrogen-signaling system (i.e., increase or decrease in estrogen sensitivity) might alter ovarian responsiveness to MXC. Thus, we examined whether ERalpha overexpression alters the ability of MXC to increase follicle atresia. To do so, we employed a transgenic mouse model in which ERalpha can be inducibly overexpressed in animal tissues (ERalpha overexpressors). We dosed female controls and ERalpha overexpressors with sesame oil (vehicle control) or MXC (32 and 64 mg/kg/day) for 20 days. After dosing, the ovaries were collected for histological evaluation of follicle numbers and follicle atresia, while blood was collected for measurements of hormones. Estrous cycles were determined in all animals to ensure that all were terminated during estrus. Although there were no significant effects of MXC on the numbers of primordial, primary, and preantral follicles in both controls and ERalpha overexpressors, there was an effect on antral follicles. Specifically, our data indicate that 32 and 64 mg/kg MXC increased the percentage of atretic follicles compared to vehicle in both control and ERalpha overexpressor groups. Moreover, there was a clear trend toward greater sensitivity to 64 mg/kg MXC in ERalpha-overexpressing mice compared to control animals. Specifically, at the 64-mg/kg MXC dose, ERalpha-overexpressing mice had a significantly higher percentage of atretic follicles compared to control animals (controls = 21.5 +/- 3%, n = 5; ERalpha overexpressors = 37 +/- 23%, n = 9, p < or = 0.05 vs. controls). After 20 days of dosing, there were no differences in estradiol levels between controls and ERalpha-overexpressing mice in all treatment groups. Follicle-stimulating hormone (FSH) levels were similar in sesame oil-treated control

  8. Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia

    Directory of Open Access Journals (Sweden)

    Muhammad SAEED*

    2014-12-01

    Full Text Available How to Cite This Article: Saeed M, Haq A, Qadir Kh.Bart’s Syndrome Associated Corpus Callosum Agenesis and Choanal Atresia. Iran J Child Neurol. 2014 Autumn;8(4: 76-79.AbstractObjectiveBart’s syndrome is defined as congenital localized absence of skin, and associated with epidermolysis bullosa. A newborn with Bart’s syndrome is reported because it is a very rare condition, especially when associated with corpus callosum agenesis and concomitant choanal atresia. Clinically it is characterized by raw beefy areas of denuded skin mainly on hands and feet.We report a rare case of a term female newborn born to non-consanguineous parents who presented with congenital absence of skin in, face, trunk and extremities. To the best of our knowledge, this is the first report presenting a case of Bart’s syndrome associated with corpus callosum agenesis.ReferencesBart BJ, Garlin RJ, Anderson VE, Lynch FW. Congenital localized absence of skin and associated abnormalities resembling epidermolysis bullosa. A new syndrome. Arch Dermatol 1966; 93: 296-304.Bart BJ. Epidermolysis bullosa and congenital localized absence of skin. Arch Dermatol 1970; 101: 78-81.Skoven I, Drzewiecki KT. Congenital localized skin defect and epidermolysis bullosa hereditaria letalis. Acta Derm Venereol 1979; 59: 533-537.Wojnarowska FT, Eady RA, Wells RS. Dystrophic epidermolysis bullosa presenting with congenital localized absence of skin: report of four cases. Br J Dermatol 1983; 108: 477-483.Kanzler MH, Smoller B, Woodley DT. Congenital localized absence of the skin as a manifestation of epidermolysis bullosa. Arch Dermatol 1992; 128:1087-90.Maman E, Maor E, Kachko L, Carmi R. Epidermolysis bullosa, pyloric atresia, aplasia cutis congenita: histopathological delineation of an autosomal recessive disease. Am J Med Genet 1998; 78: 127-133.McCarthy MA, Clarke T, Powell FC. Epidermolysis bullosa and aplasia cutis. Int J Derm 1991; 30: 481-484.Puvabanditsin S, Garrow E, Daeun K

  9. Transcatheter pulmonary valve perforation using chronic total occlusion wire in pulmonary atresia with intact ventricular septum

    Science.gov (United States)

    Bakhru, Shweta; Marathe, Shilpa; Saxena, Manish; Verma, Sudeep; Saileela, Rajan; Dash, Tapan K; Koneti, Nageswara Rao

    2017-01-01

    Background: Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies. Objective: To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation. Methods: This is a single-center, nonrandomized, retrospective study conducted during June 2008 to September 2015. Twenty-four patients with PA IVS were selected for the procedure during the study period. The median age and weight of the study population were 8. days and 2.65 kg, respectively. Four patients were excluded after right ventricular angiogram as they showed right ventricular-dependent coronary circulation. The pulmonary valve perforation was attempted using various types of CTO wires based on the tip load with variable penetrating characteristics. Results: The procedure was successful in 16 of twenty patients using CTO wires: Shinobi in nine, Miracle in four, CROSS-IT in two, and Conquest Pro in one. Two patients had perforation of right ventricular outflow tract (RVOT). Pericardiocentesis was required in one patient to relieve cardiac tamponade. Later, the same patient underwent successful hybrid pulmonary valvotomy. The other patient underwent ductus arteriosus (DA) stenting. Balloon atrial septostomy was needed in three cases with systemic venous congestion. Desaturation was persistent in five cases necessitating DA or RVOT stenting to augment pulmonary blood flow. There were two early and two late deaths. The mean follow-up was 22.66 ± 16 months. Three patients underwent one and half ventricle repair and one Blalock–Taussig shunt during follow-up. Conclusion: Perforation of the pulmonary valve can be done successfully using CTO wires in selected cases of pulmonary atresia with intact

  10. Silencing of the rotavirus NSP4 protein decreases the incidence of biliary atresia in murine model.

    Directory of Open Access Journals (Sweden)

    Jiexiong Feng

    Full Text Available Biliary atresia is a common disease in neonates which causes obstructive jaundice and progressive hepatic fibrosis. Our previous studies indicate that rotavirus infection is an initiator in the pathogenesis of experimental biliary atresia (BA through the induction of increased nuclear factor-kappaB and abnormal activation of the osteopontin inflammation pathway. In the setting of rotavirus infection, rotavirus nonstructural protein 4 (NSP4 serves as an important immunogen, viral protein 7 (VP7 is necessary in rotavirus maturity and viral protein 4 (VP4 is a virulence determiner. The purpose of the current study is to clarify the roles of NSP4, VP7 and VP4 in the pathogenesis of experimental BA. Primary cultured extrahepatic biliary epithelia were infected with Rotavirus (mmu18006. Small interfering RNA targeting NSP4, VP7 or VP4 was transfected before rotavirus infection both in vitro and in vivo. We analyzed the incidence of BA, morphological change, morphogenesis of viral particles and viral mRNA and protein expression. The in vitro experiments showed NSP4 silencing decreased the levels of VP7 and VP4, reduced viral particles and decreased cytopathic effect. NSP4-positive cells had strongly positive expression of integrin subunit α2. Silencing of VP7 or VP4 partially decreased epithelial injury. Animal experiments indicated after NSP4 silencing, mouse pups had lower incidence of BA than after VP7 or VP4 silencing. However, 33.3% of VP4-silenced pups (N = 6 suffered BA and 50% of pups (N = 6 suffered biliary injury after VP7 silencing. Hepatic injury was decreased after NSP4 or VP4 silencing. Neither VP4 nor VP7 were detected in the biliary ducts after NSP4. All together, NSP4 silencing down-regulates VP7 and VP4, resulting in decreased incidence of BA.

  11. [Prognostic factors related to mortality of children with atresia of bile ducts].

    Science.gov (United States)

    Monroy-Teniza, Zuhy Arlette; Flores-Calderón, Judith; Villasís-Keever, Miguel Ángel

    2015-01-01

    Introducción: la atresia de vías biliares (AVB) es el resultado final de un proceso destructivo, idiopático e inflamatorio que afecta los conductos biliares intra y extrahepáticos, dando lugar a fibrosis y progresivamente a cirrosis biliar. El objetivo fue identificar los factores relacionados con la mortalidad en niños con AVB. Métodos: estudio observacional, longitudinal, analítico y retrospectivo aplicado a pacientes con diagnóstico de atresia de vías biliares atendidos entre los años 2008 y 2012 en hospital de tercer nivel. Resultados: se incluyeron un total de 66 pacientes con AVB; 49 (74.2 %) fueron niñas. Solo se realizó operación de Kasai a 47, la edad al momento del envío fue de 4.5 meses. Al comparar los grupos con y sin Kasai, resultaron estadísticamente significativas la edad menor al momento del envío y la edad del diagnóstico, así como un menor puntaje en el PELD score y en el CHILD-PUGH para el grupo con Kasai. Los pacientes que fallecieron tuvieron una calificación de PELD estadísticamente mayor (mediana 20) que los que vivieron (mediana 13), p = 0.004. El factor relacionado directamente con la mortalidad, fue el antecedente de cirugía de Kasai con una RM de 0.17 (IC 95 %: 0.04-0.71; p = 0.016). Conclusiones: el pronóstico de los niños con AVB continúa siendo sombrío, dado que se diagnostican en etapas tardías. El factor más importante relacionado con la mortalidad en estos pacientes es la realización de cirugía de Kasai.

  12. Extrahepatic bile duct atresia from the pathologist’s perspective: pathological features and differential diagnosis

    Directory of Open Access Journals (Sweden)

    Peter Van Eyken

    2014-06-01

    Full Text Available Extrahepatic biliary atresia (EHBA refers to stenosis or atresia of the extrahepatic biliary tree. It accounts for 25-30% of cases of neonatal cholestasis. If left untreated, EHBA progresses to biliary cirrhosis and is universally fatal within the first 2 years of life. Early diagnosis is crucial since surgical treatment (Kasai procedure is the only treatment option. Histopathologic examination of liver biopsy specimens is a key element in the diagnostic work-up of infants with suspected EHBA. Pathologic diagnosis aims at excluding non-surgically correctable causes of neonatal cholestasis thereby leading to surgical exploration for confirmation of the diagnosis. All published data indicate that pathologists can diagnose EHBA with high sensitivity, high specificity and reasonable interobserver agreement. The most useful histologic features in the diagnosis of EHBA are portal tract changes including ductular proliferation and bile plugs in ducts and ductules. These lesions are not pathognomonic but can be seen in extrahepatic obstruction of any cause. Total parenteral nutrition (TPN-associated cholestasis and alpha1-antitrypsin (A1AT deficiency cannot be differentiated from EHBA without access to clinical data and may lead to false-positive diagnosis. False-negative interpretation may be caused by early age at diagnosis or by small/indequate specimens. The pathologist also plays a role in the examination of the resected fibrotic segment and of explant specimens. Histopathology can yield prognostic information, being also an indispensable tool in research for the possible pathogenesis of this disease. A well-coordinated, multidisciplinary approach is required in the assessment of suspected cases of EHBA.  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

  13. Simultaneous Esophageal Squamous Cell Carcinoma and Adenocarcinoma: A Case Report

    OpenAIRE

    Maleki, Iradj; Shekarriz, Ramin; Nosrati, Anahita; Orang, Elahe

    2015-01-01

    Esophageal squamous cell carcinoma is a rather common cancer in northern Iran. Incidence of adenocarcinoma of esophagus has an increasing trend in Iran. Co-existence of both cancers in one patient is very rare. We report a middle age woman from northern Iran with a typical presentation of esophageal cancer, who was found to have a dual esophageal cancer. The disease was found in the advanced stage with pulmonary metastasis at the presentation. Palliative chemo-radiotherapy induced partial cli...

  14. Esophageal space-occupying lesion caused by Ascaris lumbricoides

    OpenAIRE

    Zheng,Ping-Ping; Wang, Bing-Yuan; Wang, Fei(Physics Department, Zhengzhou University, Henan, 450001, China); Ao, Ran; Wang, Ying

    2012-01-01

    Ascaris lumbricoides is the largest intestinal nematode parasite of man, which can lead to various complications because of its mobility. As the esophagus is not normal habitat of Ascaris, the report of esophageal ascariasis is rare. An old female presented with dysphagia after an intake of several red bean buns and haw jellies. The barium meal examination revealed a spherical defect in the lower esophagus. Esophageal bezoar or esophageal carcinoma was considered at the beginning. The patient...

  15. Percutaneous endoscopic gastrostomy for nutritional palliation of upper esophageal cancer unsuitable for esophageal stenting

    Directory of Open Access Journals (Sweden)

    Ana Grilo

    2012-09-01

    Full Text Available CONTEXT: Esophageal cancer is often diagnosed at an advanced stage and has a poor prognosis. Most patients with advanced esophageal cancer have significant dysphagia that contributes to weight loss and malnutrition. Esophageal stenting is a widespread palliation approach, but unsuitable for cancers near the upper esophageal sphincter, were stents are poorly tolerated. Generally, guidelines do not support endoscopic gastrostomy in this clinical setting, but it may be the best option for nutritional support. OBJECTIVE: Retrospective evaluation of patients with dysphagia caused advanced esophageal cancer, no expectation of resuming oral intake and with percutaneous endoscopic gastrostomy for comfort palliative nutrition. METHOD: We selected adult patients with unresecable esophageal cancer histological confirmed, in whom stenting was impossible due to proximal location, and chemotherapy or radiotherapy were palliative, using gastrostomy for enteral nutrition. Clinical and nutritional data were evaluated, including success of gastrostomy, procedure complications and survival after percutaneous endoscopic gastrostomy, and evolution of body mass index, albumin, transferrin and cholesterol. RESULTS: Seventeen males with stage III or IV squamous cell carcinoma fulfilled the inclusion criteria. Mean age was 60.9 years. Most of the patients had toxic habits. All underwent palliative chemotherapy or radiotherapy. Gastrostomy was successfully performed in all, but nine required prior dilatation. Most had the gastrostomy within 2 months after diagnosis. There was a buried bumper syndrome treated with tube replacement and four minor complications. There were no cases of implantation metastases or procedure related mortality. Two patients were lost and 12 died. Mean survival of deceased patients was 5.9 months. Three patients are alive 6, 14 and 17 months after the gastrostomy procedure, still increasing the mean survival. Mean body mass index and laboratory

  16. Biliary atresia

    Science.gov (United States)

    ... Elsevier; 2016:chap 356. Suchy FJ. Anatomy, histology, embryology, developmental anomalies, and pediatric disorders of the biliary ... M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health ...

  17. Pulmonary Atresia

    Science.gov (United States)

    ... Kawasaki Disease Long Q-T Syndrome Marfan Syndrome Metabolic Syndrome Mitral Valve Prolapse Myocardial Bridge Myocarditis Obstructive Sleep Apnea Pericarditis Peripheral Vascular Disease Rheumatic Fever Sick Sinus Syndrome Silent Ischemia Stroke Sudden ...

  18. Tricuspid Atresia

    Science.gov (United States)

    ... congenital heart defect, including: A mother who had German measles (rubella) or another viral illness during early ... a family member or friend along, if possible. Learning that your baby has a serious heart condition ...

  19. Tricuspid atresia

    Science.gov (United States)

    ... This procedure connects half of the veins carrying blue blood from the upper half of the body directly ... Fontan procedure. The rest of the veins carrying blue blood from the body are connected directly to the ...

  20. Esophageal motility disorders; Motilitaetsstoerungen des Oesophagus

    Energy Technology Data Exchange (ETDEWEB)

    Hannig, C.; Rummeny, E. [Klinikum rechts der Isar der Technischen Universitaet Muenchen, Institut fuer Roentgendiagnostik, Muenchen (Germany); Wuttge-Hannig, A. [Gemeinschaftspraxis fuer Radiologie, Nuklearmedizin und Strahlentherapie, Muenchen (Germany)

    2007-02-15

    For the better understanding of esophageal motility, the muscle texture and the distribution of skeletal and smooth muscle fibers in the esophagus are of crucial importance. Esophageal physiology will be shortly mentioned as far as necessary for a comprehensive understanding of peristaltic disturbances. Besides the pure depiction of morphologic criteria, a complete esophageal study has to include an analysis of the motility. New diagnostic tools with reduced radiation for dynamic imaging (digital fluoroscopy, videofluoroscopy) at 4-30 frames/s are available. Radiomanometry is a combination of a functional pressure measurement and a simultaneous dynamic morphologic analysis. Esophageal motility disorders are subdivided by radiologic and manometric criteria into primary, secondary, and nonclassifiable forms. Primary motility disorders of the esophagus are achalasia, diffuse esophageal spasm, nutcracker esophagus, and the hypertonic lower esophageal sphincter. The secondary motility disorders include pseudoachalasia, reflux-associated motility disorders, functionally caused impactions, Boerhaave's syndrome, Chagas' disease, scleroderma, and presbyesophagus. The nonclassificable motility disorders (NEMD) are a very heterogeneous collective. (orig.) [German] Zum Verstaendnis der Motilitaet des Oesophagus sind muskulaere Architektur und Verteilung der quergestreiften und glatten Muskelfasern von Bedeutung. Die Physiologie des Oesophagus wird in soweit kurz dargestellt, als sie fuer das Verstaendnis von peristaltischen Stoerungen notwendig ist. Neben der Erfassung rein morphologischer Kriterien ist bei der Untersuchung der Speiseroehre eine diagnostische Bewertung der Motilitaet erforderlich. Es stehen uns heute strahlungsarme dynamische Aufzeichnungsverfahren (digitale dynamische Aufzeichnung, Videofluoroskopie) mit Bildsequenzen von 4-30 Bildern/s zur Verfuegung. Die Kombination einer funktionellen Methode zur Darstellung der Morphologie und der

  1. Esophageal Motor Disorders in Terms of High-Resolution Esophageal Pressure Topography: What Has Changed?

    Science.gov (United States)

    Kahrilas, Peter J.

    2010-01-01

    The concept of high-resolution manometry (HRM) is to use sufficient pressure sensors such that intraluminal pressure can be monitored as a continuum along luminal length much as time is viewed as a continuum in conventional manometry. When HRM is coupled with pressure topography plots, pressure amplitude is transformed into spectral colors with isobaric conditions indicated by same-colored regions on the display. Together, these technologies are called high-resolution esophageal pressure topography (HREPT). HREPT has several advantages compared with conventional manometry, the technology that it was designed to replace. (i) The contractility of the entire esophagus can be viewed simultaneously in a uniform format, (ii) standardized objective metrics can be systematically applied for interpretation, and (iii) topographic patterns of contractility are more easily recognized and have greater reproducibility than with conventional manometry. Compared with conventional manometry, HREPT has improved sensitivity for detecting achalasia, largely due to the objectivity and accuracy with which it identifies impaired esophagogastric junction (EGJ) relaxation. In addition, it has led to the subcategorization of achalasia into three clinically relevant subtypes based on the contractile function of the esophageal body: classic achalasia, achalasia with esophageal compression, and spastic achalasia. Headway has also been made in understanding hypercontractile conditions, including diffuse esophageal spasm and a newly described entity, spastic nutcracker. Ultimately, clinical experience will be the judge, but it seems likely that HREPT data, along with its well-defined functional implications, will improve the clinical management of esophageal motility disorders. PMID:20179690

  2. Whole greater than the parts: integrated esophageal centers (IEC) and advanced training in esophageal diseases.

    Science.gov (United States)

    Triadafilopoulos, G; Clarke, J; Hawn, M

    2017-10-01

    An integrated esophageal center (IEC) is a multidisciplinary team with expertise, skill, range, and facilities necessary to achieve optimal outcomes in patients with esophageal diseases efficiently and expeditiously. Within IEC, patients presenting with esophageal symptoms undergo a detailed clinical, functional and structural evaluation of their esophagus prior to implementation of tailored medical, endoscopic or surgical therapy. Serving as a core, the IEC clinical practice also supports research and innovation in esophageal diseases as well as public and physician education. Referrals to the unit may be primary, either from primary care or self-initiated, or secondary from other specialty practices, to reassess patients who have previously failed therapies and to manage complex or complicated cases. The fundamental goals of the IEC are to provide value for patients with esophageal diseases, streamlining complex diagnostic investigations and expediting therapies aiming at reducing costs while improving clinical outcomes, and to accelerate knowledge generation through robust interaction and cross-training across disciplines. The organization of the IEC goes beyond traditional academic and clinical silos and involves a director and administrative team coordinating faculty and fellows from both medical and surgical disciplines and supported by other clinical lines, such as radiology, pathology, etc., while it interfaces with physicians, the public, basic, translational and clinical research groups, and related industry partners. © The Authors 2017. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  3. Two-Stage Explantation of a Magnetic Lower Esophageal Sphincter Augmentation Device Due to Esophageal Erosion.

    Science.gov (United States)

    Parmar, Abhishek D; Tessler, Robert A; Chang, Howard Y; Svahn, Jonathan D

    2017-08-01

    Implanting a magnetic lower esophageal sphincter augmentation device (LINX, Torax Medical) has become an increasingly common option in the surgical management of gastroesophageal reflux disease. As the enthusiasm for placing this device increases, experience in the management of device-related complications-including erosion-is necessary. We report a staged approach to LINX removal in a 64-year-old female with symptoms of odynophagia secondary to partial erosion of a LINX device into the esophagus. The patient had a 12-bead LINX device placed in 2011 at an outside, international facility. In late 2013, she began experiencing symptoms of odynophagia. An esophagogastroduodenoscopy at our institution in October 2015 demonstrated two metallic beads eroding through the distal esophageal lumen. An elective endoscopic removal of the two visible beads was performed. A postoperative esophagram confirmed that there was no resulting esophageal perforation. The patient noted mild improvement in her symptoms. After a 12-week period to allow for complete healing, the remaining 10 beads of the LINX device were explanted laparoscopically without complication. No further procedures were undertaken. At 2 months' follow-up, the patient noted complete resolution of her symptoms. Transmural erosion of the LINX device into the esophageal lumen is a rare occurrence, with only five such complications reported in the published literature. We present the first account of LINX explantation for esophageal erosion in the United States. We demonstrated that a staged laparoendoscopic approach to LINX removal in these cases is feasible with minimal morbidity.

  4. Operação de Fontan-Kreutzer em anomalias cardíacas complexas outras que não atresia tricúspide lb, ventrículo único e atresia pulmonar com septo ventricular íntegro The operation of Fontan-Kreutzer in complex congenital anomalies other than lb isolated tricuspid atresia, single ventricle and pulmonary atresia with intact septum

    Directory of Open Access Journals (Sweden)

    Miguel Barbero-Marcial

    1988-08-01

    Full Text Available Oitenta e quatro pacientes foram submetidos a operação tipo Fontan, no Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, entre 1979 e 1989; as idades variaram entre 5 meses e 31 anos. Estes pacientes foram colocados em 5 grupos anatômicos: 1 atresia tricúspide la, 3 pacientes (1 óbito, 33,3%; 2 atresia tricúspide lb, 48 pacientes (5 óbitos, 10,4%; 3 ventrículo único: 18 pacientes (2 óbitos, 11,1%; 4 atresia pulmonar com septo interventricular íntegro, 5 pacientes (1 óbito, 20,0%; 5 anomalias complexas, 10 pacientes (zero óbitos. O grupo das anomalias complexas foi dividido em 2 subgrupos: pacientes com situs solitus e pacientes com situs inversus. A maioria destes pacientes foi submetida a procedimentos adicionais: em 5 pacientes, uma das valvas A-V foi fechada; em 1 paciente, foram realizadas valvoplastia mitral e septação do átrio único; em 1 paciente, foi realizada a secção de feixe anômalo (feixe de Kent. A evolução tardia (6 meses a 8 anos revelou que 9 pacientes encontram-se em classe funcional I e 1 na classe funcional II (NYHA. Baseados nos resultados obtidos, concluímos que a operação de Fontan modificada é uma alternativa válida em anomalias cianóticas complexas.Between 1979 and 1989, 84 patients underwent a Fontan operation or one of its modifications; the ages ranged from 5 months to 31 years. Patients were placed into five anatomic groups: 1 tricuspid atresia la (3 cases, 1 death, 33.3%; 2 tricuspid atresia lb (48 cases, 5 deaths, 10.4%; 3 single ventricle (18 cases, 2 deaths, 11.1%; 4 pulmonary atresia, intact ventricular septum (5 cases, 1 death, 20.0%; 5 complex anomalies (10 cases, no deaths. The subgroup of complex anomalies was divided in: with situs inversus and with situs solitus. In the majority of these patients, additional procedures were performed; in 5, one A-V valve was closed; in 2, complex atrial septation was done; in 1, a mitral

  5. The image variations in mastoid segment of facial nerve and sinus tympani in congenital aural atresia by HRCT and 3D VR CT.

    Science.gov (United States)

    Wang, Zhen; Hou, Qian; Wang, Pu; Sun, Zhaoyong; Fan, Yue; Wang, Yun; Xue, Huadan; Jin, Zhengyu; Chen, Xiaowei

    2015-09-01

    To find the variations of middle ear structures including the spatial pattern of mastoid segment of facial nerve and the shapes of the sinus tympani in patients with congenital aural atresia (CAA) by using the high-resolution (HR) CT and 3D volume rendered (VR) CT images. HRCT was performed in 25 patients with congenital aural atresia including six bilateral atresia patients (n=25, 21 males, 4 females, mean age 13.8 years, range 6-19). Along the long axis of the posterior semicircular canal ampulla, the oblique axial multiplanar reconstruction (MPR) was set to view the depiction of the round window and the mastoid segment of facial nerve. Volumetric rending technique was used to demonstrate the morphologic features. HRCT and 3D VR findings in atresia ears were compared with those in 19 normal ears of the unilateral ears of atresia patients. On the basic plane, the horizontal line distances between the mastoid segment of the facial nerve and the round window (h-RF) in atresia ears significantly decreased compared to the control ears (PVR CT images. HRCT and 3D VR CT could help a better understanding of different kinds of variations in mastoid segment of facial nerve and sinus tympani in CAA ears. And it may further help surgeons to make the correct decision for hearing rehabilitation. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  6. Sarcopenia and Visceral Obesity in Esophageal and Gastric Cancer

    Science.gov (United States)

    2017-02-17

    Esophageal Cancer; Gastric Cancer; Sarcopenia; Sarcopenic Obesity; Obesity; Visceral Obesity; Quality of Life; Surgery; Complication of Treatment; Chemotherapeutic Toxicity; Physical Activity; Oncology

  7. Congenital esophageal stenosis in 3 children: A case series

    Directory of Open Access Journals (Sweden)

    Mackenzie C. Lees

    2017-08-01

    Full Text Available Congenital esophageal stenosis (CES is rare condition found in 1 per 25,000 to 50,000 live births. It is characterized by intrinsic narrowing of the esophagus secondary to congenital malformation of the esophageal wall architecture. Diagnosis is often difficult to definitively establish as the symptoms are often initially attributed to esophageal strictures secondary to reflux, or occur within the context of a tracheo-esophageal fistula (TEF in the newborn. Endoscopic dilation and surgical repair are the mainstays of treatment. We report a series of three cases seen recently at our institution, the University of Alberta/Stollery Children's Hospital.

  8. [A case of esophageal achalasia followed by Parkinson's disease].

    Science.gov (United States)

    Mitani, Maki; Kawamoto, Kunihiko; Funakawa, Itaru; Jinnai, Kenji

    2005-08-01

    In 1992, a 63 year-old woman complained of dysphagia and chest pain, and was diagnosed with esophageal achalasia. Three years later, she developed resting tremor, cog-wheel rigidity, and retro-pulsion, and was diagnosed with Parkinson's disease and given appropriate medication. Several years later, intractable vomitting and aspiration pneumonia developed, and the lower esophageal sphincter was dilated using a pneumatic balloon dilator under gastroscopic guidance in 2004. That procedure improved her symptoms and the esophageal dilation was visualized on chest CT images. Herein, we report this rare case of esophageal achalasia followed by Parkinson's disease and discuss the relationship between the two diseases.

  9. Antipsychotic-induced life-threatening 'esophageal dyskinesia'.

    Science.gov (United States)

    Horiguchi, J; Shingu, T; Hayashi, T; Kagaya, A; Yamawaki, S; Horikawa, Y; Kitadai, Y; Inoue, M; Nishikawa, T

    1999-03-01

    We report two patients with lingual dyskinesia and complaints of food regurgitation following long-term antipsychotic therapy. Esophageal contrast radiography revealed dyskinetic movements extending from the pharynx to the upper portion of the esophagus. The elevation of intraesophageal pressure was confirmed by esophageal manometry. The dyskinetic movements almost disappeared along with improvement of lingual dyskinesia following the administration of sulpiride in one patient. Another patient suddenly died due to asphyxiation of foods before the beginning of treatment. We termed this life-threatening movement, 'esophageal dyskinesia'. It should be emphasized that 'esophageal dyskinesia' associated with lingual dyskinesia is a potentially fatal adverse reaction to antipsychotic therapy.

  10. Aortic Pseudoaneurysm Secondary to Mediastinitis due to Esophageal Perforation

    Directory of Open Access Journals (Sweden)

    Claudia Patricia Zuluaga

    2016-01-01

    Full Text Available Esophageal perforation is a condition associated with high morbidity and mortality rates; it requires early diagnosis and treatment. The most common complication of esophageal rupture is mediastinitis. There are several case reports in the literature of mediastinitis secondary to esophageal perforation and development of aortic pseudoaneurysm as a complication. We report the case of a patient with an 8-day history of esophageal perforation due to foreign body (fishbone with mediastinitis and aortic pseudoaneurysm. The diagnosis was made using Computed Tomography (CT with intravenous and oral water-soluble contrast material. An esophagogastroduodenoscopy did not detect the perforation.

  11. Dangers to Children at Home: Corrosive esophageal burn

    Directory of Open Access Journals (Sweden)

    Ahmet Guven

    2008-12-01

    Full Text Available Inappropriate storage of cleaners which excessively used in daily is hazardous to children. Ingestion of potent corrosive agents, especially alkaline solutions may lead to acute esophageal burns, esophageal or stomach perforation, and even to death. Early of these injuries may be associated with esophageal strictures in later and the treatment of strictures might be and take long follow-up. This article reviews related issues on the diagnosis and management of esophageal burns, strictures and preventive medicine in children. [TAF Prev Med Bull 2008; 7(6.000: 535-540

  12. Esophageal intramural pseudodiverticulosis characterized by barium esophagography: a case report

    LENUS (Irish Health Repository)

    O'Connor, Owen J

    2010-05-21

    Abstract Introduction Esophageal intramural pseudodiverticulosis is a rare condition characterized by the dilatation of the submucosal glands. Case presentation We present a case of esophageal intramural pseudodiverticulosis in a 72-year-old Caucasian man who presented with dysphagia and with a background history of alcohol abuse. An upper gastrointestinal endoscopy of our patient showed an esophageal stricture with abnormal mucosal appearances, but no malignant cells were seen at biopsy. Appearances on a barium esophagram were pathognomonic for esophageal intramural pseudodiverticulosis. Conclusion We demonstrate the enduring usefulness of barium esophagography in the characterization of abnormal mucosal appearances at endoscopy.

  13. Investigation of intra-esophageal air kinetics and esophageal sphincters in patients with total laryngectomy during esophageal speech.

    Science.gov (United States)

    Bozan, Aykut; Vardar, Rukiye; Akyildiz, Serdar; Kirazli, Tayfun; Ogut, Fatih; Yildirim, Esra; Bor, Serhat

    2015-08-01

    The purpose of this study was to evaluate the air kinetics of well- and poor-speaking patients and their upper (UES) and lower (LES) esophageal sphincter pressures . The esophageal speech capability of 23 total laryngectomy patients was assessed with the Wepman scale. LES and UES points and pressures were measured, and air kinetics were compared. All patients were male, with an average age of 58 years. Both the LES and UES pressures were not statistically different between good-speaking and poor-speaking patients (p > 0.05). The ability to speak was estimated only by looking at tracings. Good speakers are able to retain air successfully and on a long-term basis between the upper and lower esophageal sphincters. During short and/or rapid speech, these patients are able to rapidly suck and then expel the air from their upper esophagus. During long speeches, after sucking the air into their distal esophagus, they used the air in the upper part of the esophagus during the speech, only later seeming to fill the lower esophagus with the air as a possible reserve in the stomach. It has been shown that the basic requirement for speaking is the capacity to suck and store the air within the esophagus. For successful speech, the air should be stored inside the esophagus. MII technology contributes to our understanding of speech kinetics and occupies an important place in patient training as a biofeedback technique.

  14. Eosinophilic esophagitis-endoscopic distinguishing findings

    Institute of Scientific and Technical Information of China (English)

    Ana Célia Caetano; Raquel Gon(c)alves; Carla Rolanda

    2012-01-01

    Eosinophilic esophagitis (EE) is the most frequent condition found in a group of gastrointestinal disorders called eosinophilic gastrointestinal diseases.The hypothetical pathophysiological mechanism is related to a hypersensitivity reaction.Gastroesophageal reflux disease-like complaints not ameliorated by acid blockade or occasional symptoms of dysphagia or food impaction are likely presentations of EE.Due to its unclear pathogenesis and unspecific symptoms,it is difficult to diagnose EE without a strong suspicion.Although histological criteria are necessary to diagnosis EE,there are some characteristic endoscopic features.We present the case of a healthy 55-year-old woman with dysphagia and several episodes of esophageal food impaction over the last six months.This case report stresses the most distinguishing endoscopic findings-mucosa rings,white exudative plaques and linear furrows-that can help in the prompt recognition of this condition.

  15. Thoracoscopic esophagectomy for intrathoracic esophageal cancer.

    Science.gov (United States)

    Osugi, Harushi; Takemura, Masashi; Lee, Sigeru; Nishikawa, Takayuki; Fukuhara, Kennichirou; Iwasaki, Hiroshi; Higashino, Masayuki

    2005-08-01

    Thoracoscopic approaches for esophageal cancer are still disparate. Complete scopic technique is feasible for esophagectomy. Mini-thoracotomy is effective for excellent exposure of the mediastinum for lymph node dissection. The magnifying effect of a video, by keeping the camera in close proximity to the dissection is essential to perform the same quality of dissection as open surgery. The benefit, for respiratory morbidity, remains to be studied in a large number of patients. Minimizing the chest wall injury contributed, to the reduction of constrictive pulmonary damage. Survival after the thoracoscopic approach was favorably compared with open surgery, when extensive lymphadenectomy was performed. Because the efficacy improves with the surgeon's experience, satisfactory outcome will only be obtained in a center performing a sufficient volume of esophageal surgery to provide the surgeon with opportunities to refine his necessary skills. Improvements in technique and instrumentation should make the procedure more accessible and steepen the learning curve.

  16. [Surgery in esophageal carcinoma: risks and results].

    Science.gov (United States)

    von Flüe, M; Ackermann, C; Tondelli, P

    1990-04-07

    Surgical treatment of esophageal cancer is largely palliative. To clarify the indication it is necessary to assess the effectiveness of the palliation in relief of dysphagia and the operative risks. In a retrospective study we analyzed the perioperative morbidity and follow-up in 25 patients with carcinoma of the esophagus treated between 1984 and 1988 (5 years). With combined anesthesia, early extubation and intensive pulmonary therapy, no perioperative respiratory insufficiency was observed. Perioperative mortality was 0%. An anastomotic leak in 2 patients with a cervical anastomosis was healed in both cases by conservative management. On hospital discharge all patients were able to eat normally. 13 patients died after 1 year on average (4 months to 3 years). 12 patients are alive 6 months to 4 years after operation, 10 of them without symptoms. Our results show that with optimal perioperative management of esophageal carcinoma low morbidity is possible and good palliation of dysphagia is feasible.

  17. Successful tubes treatment of esophageal fistula.

    Science.gov (United States)

    Zhou, Ning; Chen, Wei-xing; Li, You-ming; Xiang, Zhun; Gao, Ping; Fang, Ying

    2007-10-01

    To discuss the merits of "tubes treatment" for esophageal fistula (EF). A 66-year-old female who suffered from a bronchoesophageal and esophagothoratic fistula underwent a successful "three tubes treatment" (close chest drainage, negative pressure suction at the leak, and nasojejunal feeding tube), combination of antibiotics, antacid drugs and nutritional support. Another 55-year-old male patient developed an esophagopleural fistula (EPF) after esophageal carcinoma operation. He too was treated conservatively with the three tubes strategy as mentioned above towards a favorable outcome. The two patients recovered with the tubes treatment, felt well and became able to eat and drink, presenting no complaint. Tubes treatment is an effective basic way for EF. It may be an alternative treatment option.

  18. Radiation-Induced Esophagitis Exacerbated by Everolimus

    Directory of Open Access Journals (Sweden)

    Yuji Miura

    2013-06-01

    Full Text Available Background: Everolimus, a potent mammalian target of rapamycin (mTOR inhibitor, has shown anticancer activity against various types of cancer, including renal cell carcinoma (RCC; however, little information is available on the efficacy and safety of the combination of everolimus and radiotherapy. We report a case of radiation-induced esophagitis that might have been exacerbated by the sequential administration of everolimus. Case Presentation: A 63-year-old Japanese man with RCC complained of back pain, and magnetic resonance imaging revealed vertebral metastases. He received radiotherapy (30 Gy/10 fractions to the T6-10 vertebrae. Everolimus was administered immediately after the completion of radiotherapy. One week later, he complained of dysphagia, nausea and vomiting. An endoscopic examination of the esophagus showed erosive esophagitis in the middle to lower portions of his thoracic esophagus, corresponding to the irradiation field. Conclusion: Clinicians should be aware that everolimus might lead to the unexpected exacerbation of radiation toxicities.

  19. Esophageal Foreign Body Causing Direct Aortic Injury

    Directory of Open Access Journals (Sweden)

    ECS Lam

    2003-01-01

    Full Text Available Foreign bodies in the esophagus are uncommon causes of esophageal perforation. Many nonperforating cases are successfully managed by flexible gastroscopy. However, complicated foreign bodies such as those that result in esophageal perforation and vascular injury are best managed surgically. Gastroscopy remains the primary method of diagnosis. A case of a 59-year-old woman who developed retrosternal and intrascapular pain, odynophagia and hematemesis after eating fish is reported. Flexible gastroscopy showed arterial bleeding from the midthoracic esophagus. Computed tomography scan localized a 3 cm fish bone perforating the esophagus with surrounding hematoma. An aortogram did not reveal an actively bleeding aortoesophageal fistula. The fish bone was surgically removed and the patient recovered with no postoperative complications. This case illustrates the importance of early consideration for surgical intervention when confronted with a brisk arterial bleed from the esophagus with suggestive history of foreign body ingestion.

  20. Successful tubes treatment of esophageal fistula

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Aim: To discuss the merits of "tubes treatment" for esophageal fistula (EF). Methods: A 66-year-old female who suffered from a bronchoesophageal and esophagothoratic fistula underwent a successful "three tubes treatment" (close chest drainage, negative pressure suction at the leak, and nasojejunal feeding tube), combination of antibiotics, antacid drugs and nutritional support. Another 55-year-old male patient developed an esophagopleural fistula (EPF) after esophageal carcinoma operation. He too was treated conservatively with the three tubes strategy as mentioned above towards a favorable outcome. Results:The two patients recovered with the tubes treatment, felt well and became able to eat and drink, presenting no complaint. Conclusion: Tubes treatment is an effective basic way for EF. It may be an alternative treatment option.

  1. Eosinophilic Esophagitis in Brazilian Pediatric Patients

    Science.gov (United States)

    Pinheiro, Mayra Isabel Correia; de Góes Cavalcanti, Luciano Pamplona; Honório, Rodrigo Schuler; de Alencar Moreno, Luís Hélder; Fortes, Mayara Carvalho; da Silva, Carlos Antônio Bruno

    2013-01-01

    We examined 11 pediatric patients with eosinophilic esophagitis with a tardy diagnosis. The symptoms were initially thought to be related to other diseases, leading to the use of inadequate therapeutic approaches. The patients were between 3 and 17 years old (mean 7.8 ± 3.8 years), and 8 of the patients were male. Common symptoms included abdominal pain, regurgitation, difficulty in gaining weight, vomiting, dysphagia, and coughing. The mean age for the onset of symptoms was 4.3 ± 2.9 years. Endoscopic findings included normal mucosa in five (45%) patients, thickening of the mucosa with longitudinal grooves in three (27%), erosive esophagitis in two (18%), and a whitish stippling in one (9%) patient. Treatment included the use of a topical corticosteroid for 10 patients. In eight (73%) cases, the treatment made the symptoms disappear. Ten patients underwent histopathological management after treatment, with a decrease in the number of eosinophils. PMID:24106430

  2. Giant esophageal epiphrenic diverticulum: presentation and treatment.

    Science.gov (United States)

    Santos, Marcelo Protásio Dos; Akerman, Denise; Santos, Caio Pasquali Dias Dos; Santos, Paulo Vicente Dos; Radtke, Marcos Claudio; Beraldo, Fernando Bray; Gonçalves, José Eduardo

    2017-09-21

    Epiphrenic diverticulum is a rare disease associated with esophageal motor disorders that is usually asymptomatic and has a well-established surgical indication. We report a case of giant epiphrenic diverticulum in a 59-year-old symptomatic woman who was diagnosed after underwent complementary exams. Because of her symptoms, the surgical treatment was chosen, and esophageal diverticulectomy was performed along with laparoscopic cardiomyotomy and anterior partial fundoplication. RESUMO O divertículo epifrênico é uma patologia rara associada a distúrbios motores esofágicos e, frequentemente, assintomática, tendo indicações cirúrgicas bem estabelecidas. Relatamos um caso de divertículo epifrênico gigante em paciente de 59 anos, sexo feminino, sintomática, diagnosticada por exames complementares. Devido à sintomatologia, optou-se por tratamento cirúrgico, sendo realizada a diverticulectomia esofágica com cardiomiotomia e fundoplicatura parcial anterior laparoscópica.

  3. Fatal hemorrhage in irradiated esophageal cancer patients

    Energy Technology Data Exchange (ETDEWEB)

    Nemoto, Kenji; Takai, Yoshihiro; Ogawa, Yoshihiro; Kakuto, Yoshihisa; Ariga, Hisanori; Matsushita, Haruo; Wada, Hitoshi; Yamada, Shogo [Tohoku Univ., Sendai (Japan). Dept. of Radiology

    1998-09-01

    Between 1980 and 1994, 423 patients with esophageal cancer were given curative radiation therapy. Of these patients, 31 died of massive hemorrhage and were used as the subjects of analysis in this study. The incidence of massive hemorrhage in all patients was 7% (31/423). In the 31 patients who died of massive hemorrhage, 27 had local tumors and two had no tumors at hemorrhage (two unknown cases). The mean time interval from the start of radiation to hemorrhage was 9.2 months. In 9 autopsy cases the origin of hemorrhage was a tear of the aorta in 5 cases, necrotic local tumor in 3 cases and esophageal ulcer in 1 case. The positive risk factors for this complication seemed to be excess total dose, infection, metallic stent, and tracheoesophageal fistula. Chest pain or sentinel hemorrhage proceeding to massive hemorrhage was observed in about half of the patients. (orig.)

  4. Management of proton pump inhibitor responsive-esophageal eosinophilia and eosinophilic esophagitis: controversies in treatment approaches.

    Science.gov (United States)

    Kochar, Bharati; Dellon, Evan S

    2015-01-01

    Eosinophilic esophagitis (EoE) is a chronic immune-mediated clinicopathologic disease. The prevalence of EoE is approximately 1/2000 persons, EoE is now the most common cause of food impactions, with healthcare expenditures approaching US$ 1 billion annually. This article will discuss challenges related to proton pump inhibitor responsive esophageal eosinophilia, including distinguishing this condition from EoE and understanding the mechanisms behind the PPI response. For EoE, we will review multiple ongoing debates about treatment and monitoring strategies, including selecting treatment outcomes, optimizing medication formulations, approaching the steroid-refractory patient, conducting dietary elimination, prescribing long-term maintenance therapy and performing esophageal dilation.

  5. Síndrome de Treacher Collins com atresia coanal: relato de caso e revisão de suas características Treacher Collins syndrome with choanal atresia: a case report and review of disease features

    Directory of Open Access Journals (Sweden)

    Eduardo C. Andrade

    2005-02-01

    Full Text Available A Síndrome de Treacher Collins ou disostose mandibulofacial apresenta-se com deformidades crânio-faciais, tendo expressão e severidade variável. É uma malformação congênita que envolve o primeiro e segundo arcos branquiais. A Síndrome de Treacher Collins é rara e sua incidência está estimada em uma faixa de 1:40000 a 1:70000 nascidos vivos. Esta síndrome é caracterizada por anormalidades dos pavilhões auriculares, hipoplasia dos ossos da face, obliqüidade antimongolóide das fendas palpebrais com coloboma palpebral inferior e fissura palatina. A Síndrome de Treacher Collins raramente está associada com atresia coanal. Estes pacientes são apropriadamente acompanhados por uma equipe multidisciplinar que inclui cirurgiões crânio-faciais, oftalmologistas, fonoaudiologistas, cirurgiões dentistas e otorrinolaringologistas. Relatamos neste artigo um caso raro de Síndrome de Treacher Collins com atresia coanal, uma revisão da patologia e intervenção multidisciplinar.Treacher Collins Syndrome - or mandibulofacial dysostosis - is a rare condition that presents several craniofacial deformities of different levels. This is a congenital malformation involving the first and second branchial arches. Incidence is estimated to range between 1-40,000 to 1-70,000 of live births. The disorder is characterized by abnormalities of the auricular pinna, hypoplasia of facial bones, antimongoloid slanting palpebral fissures with coloboma of the lower eyelids and cleft palate. Treacher Collins Syndrome is rarely associated with choanal atresia. A multidisciplinary team, including craniofacial surgeon, ophthalmologist, speech therapist, dental surgeon and otorhinolaryngologist, is the most appropriate setting to manage these patients. This study reports a rare case of Treacher Collins Syndrome with choanal atresia, presenting literature review and multidisciplinary intervention.

  6. “Layer-to-Layer” Esophagogastric Anastomosis Combined with Intrathoracic Esophageal Mucosal Extention in Patients with Esophageal and Cardia Cancer

    Institute of Scientific and Technical Information of China (English)

    SHIZhongqi; CAIPing; YANYu; CHENYinchun; LIUJunhua; YOUQinshen; CAIJixiang; XIZhongxia

    2005-01-01

    Objective: To evaluate the clinical value of intrathoracic esophagogastric “layer-to-layer” anastomosis with esophageal mucosa extended varied in plane and to study the effective methods to prevent anastomotic leaking or stricture postoperatively. Methods: From May 1985 to December 2002, 2 240 esophageal and stomach cardia cancer patients treated by intrathoracic esophagogastric “layer-to-layer”anastomosis with esophageal mucosa extended varied in plane were retrospectively analyzed. Results:There was no anastomotic leaking and severe stricture in all above cases. Conclusion: Intrathoracic esophagogastric “layer-to-layer” anastomosis with esophageal mucosal extended varied in plane is an effective method to prevent anastomotic leaking and stricture postoperatively.

  7. Non-surgical treatment of esophageal achalasia

    Institute of Scientific and Technical Information of China (English)

    Vito Annese; Gabrio Bassotti

    2006-01-01

    Esophageal achalasia is an infrequent motility disorder characterized by a progressive stasis and dilation of the oesophagus; with subsequent risk of aspiration,weight loss, and malnutrition. Although the treatment of achalasia has been traditionally based on a surgical approach, especially with the introduction of laparoscopic techniques, there is still some space for a medical approach. The present article reviews the non-surgical therapeutic options for achalasia.

  8. Black esophagus: Acute esophageal necrosis syndrome

    Institute of Scientific and Technical Information of China (English)

    Grigoriy; E; Gurvits

    2010-01-01

    Acute esophageal necrosis (AEN), commonly referred to as "black esophagus", is a rare clinical entity arising from a combination of ischemic insult seen in hemodynamic compromise and low-flow states, corrosive injury from gastric contents in the setting of esophago-gastroparesis and gastric outlet obstruction, and decreased function of mucosal barrier systems and reparative mechanisms present in malnourished and debilitated physical states. AEN may arise in the setting of multiorgan dysfunction, hypoperfusi...

  9. Radiotherapy of double primary esophageal carcinoma

    Institute of Scientific and Technical Information of China (English)

    Ze Fen Xiao; Zong Yi Yang; Zong Mei Zhou; Wei Bo Yin; Xian Zhi Gu

    2000-01-01

    @@ INTRODUCTION Double primary esophageal carcinoma is defined as having two foci of squamous cell cancer simultaneously or consecutively developing in different sites of esophagus. This rare disease appears mostly in the literature as case reports[1-4],reports about its treatment are even more infrequent. Here we present our experiences of radiation therapy in 37 patients with this disease and focus the discussion on the optimum method of treatment and complications.

  10. Medicolegal aspects of esophageal cancer surgery.

    Science.gov (United States)

    De Giorgio, Fabio

    2005-01-01

    Forensic implications of esophageal cancer surgery are varied and complex depending on the field of specialization involved i.e. civil law, criminal law, insurance or social security and for the distinct probative requirements related to each field. The aim of this article is to reconstruct the logical procedure of a forensic doctor who actually examines a practical case to establish the profiles of professional responsibility in particular in civil or criminal law.

  11. Technological advances in radiotherapy for esophageal cancer

    Institute of Scientific and Technical Information of China (English)

    Milan; Vosmik; Jiri; Petera; Igor; Sirak; Miroslav; Hodek; Petr; Paluska; Jiri; Dolezal; Marcela; Kopacova

    2010-01-01

    Radiotherapy with concurrent chemotherapy and surgery represent the main treatment modalities in esophageal cancer.The goal of modern radiotherapy approaches,based on recent technological advances,is to minimize post-treatment complications by improving the gross tumor volume definition (positron emission tomography-based planning),reducing interfraction motion (image-guided radiotherapy) and intrafraction motion (respiratory-gated radiotherapy),and by better dose delivery to the precisely defined planning ...

  12. Multidisciplinary management for esophageal and gastric cancer

    Directory of Open Access Journals (Sweden)

    Boniface MM

    2016-04-01

    Full Text Available Megan M Boniface,1 Sachin B Wani,2 Tracey E Schefter,3 Phillip J Koo,4 Cheryl Meguid,1 Stephen Leong,5 Jeffrey B Kaplan,6 Lisa J Wingrove,7 Martin D McCarter1 1Section of Surgical Oncology, Division of GI, Tumor and Endocrine Surgery, Department of Surgery, 2Division of Gastroenterology and Hepatology, Department of Therapeutic and Interventional Endoscopy, 3Department of Radiation Oncology, 4Division of Radiology-Nuclear Medicine, Department of Radiology, 5Division of Medical Oncology, 6Department of Pathology, University of Colorado Denver, 7Department of Food and Nutrition Services, University of Colorado Hospital Cancer Center, Aurora, CO, USA Abstract: The management of esophageal and gastric cancer is complex and involves multiple specialists in an effort to optimize patient outcomes. Utilizing a multidisciplinary team approach starting from the initial staging evaluation ensures that all members are in agreement with the plan of care. Treatment selection for esophageal and gastric cancer often involves a combination of chemotherapy, radiation, surgery, and palliative interventions (endoscopic and surgical, and direct communication between specialists in these fields is needed to ensure appropriate clinical decision making. At the University of Colorado, the Esophageal and Gastric Multidisciplinary Clinic was created to bring together all experts involved in treating these diseases at a weekly conference in order to provide patients with coordinated, individualized, and patient-centered care. This review details the essential elements and benefits of building a multidisciplinary program focused on treating esophageal and gastric cancer patients. Keywords: tumor board, upper gastrointestinal malignancies, patient centered

  13. Surgical treatment of giant esophageal leiomyoma

    Institute of Scientific and Technical Information of China (English)

    Bang-Chang Cheng; Sheng Chang; Zhi-Fu Mao; Mao-Jin Li; Jie Huang; Zhi-Wei Wang; Tu-Sheng Wang

    2005-01-01

    AIM: To summarize the operative experiences for giant leiomyoma of esophagus.METHODS: Eight cases of giant esophageal leiomyoma (GEL) whose tumors were bigger than 10 cm were treated surgically in our department from June 1980 to March 2004.and esophagoscopy. Leiomyoma located in upper thirds of the esophagus in one case, middle thirds of the esophagus in five cases, lower thirds of the esophagus in two cases. Resection of tumors was performed successfully in all of these cases. Operative methods included transthoracic extramucosal enucleation and buttressing the muscular defect with pedicled great omental flap (one case), esophagectomy and esophagogastrostomy above the arch of aorta (three cases), total esophagectomy and esophageal replacement with colon (four cases). Histological examination confirmed that all of these cases were leiomyoma.RESULTS: All of the eight patients recovered approvingly with no mortality and resumed normal diet after operation. Vomiting during meals occurred in one patient with esophagogastrostomy, and remained 1 mo. Reflux esophagitis occurred in one patient with esophagogastrostomy and was alleviated with medication. Thoracic colon syndrome (TCS) occurred in one patient with colon replacement at 15 mo postoperatively. No recurrence occurred in follow-up from 6 mo to 8 years.CONCLUSION: Surgical treatment for GEL is both safe and effective. The choices of operative methods mainly depend on the location and range of lesions. We prefer to treat GEL via esophagectomy combined with esophagogastrostomy or esophagus replacement with colon. The long-time quality of life is better in the latter.

  14. [Minimally Invasive Treatment of Esophageal Benign Diseases].

    Science.gov (United States)

    Inoue, Haruhiro

    2016-07-01

    As a minimally invasive treatment of esophageal achalasia per-oral endoscopic myotomy( POEM) was developed in 2008. More than 1,100 cases of achalasia-related diseases received POEM. Success rate of the procedure was more than 95%(Eckerdt score improvement 3 points and more). No serious( Clavian-Dindo classification III b and more) complication was experienced. These results suggest that POEM becomes a standard minimally invasive treatment for achalasia-related diseases. As an off-shoot of POEM submucosal tumor removal through submucosal tunnel (per-oral endoscopic tumor resection:POET) was developed and safely performed. Best indication of POET is less than 5 cm esophageal leiomyoma. A novel endoscopic treatment of gastroesophageal reflux disease (GERD) was developed. Anti-reflux mucosectomy( ARMS) is nearly circumferential mucosal reduction of gastric cardia mucosa. ARMS is performed in 56 consecutive cases of refractory GERD. No major complications were encountered and excellent clinical results. Best indication of ARMS is a refractory GERD without long sliding hernia. Longest follow-up case is more than 10 years. Minimally invasive treatments for esophageal benign diseases are currently performed by therapeutic endoscopy.

  15. Herpes simplex ulcerative esophagitis in healthy children

    Directory of Open Access Journals (Sweden)

    Abdulrahman A Al-Hussaini

    2011-01-01

    Full Text Available Herpes simplex virus is a common cause of ulcerative esophagitis in the immunocompromised or debilitated host. Despite a high prevalence of primary and recurrent Herpes simplex virus infection in the general population, Herpes simplex virus esophagitis (HSVE appears to be rare in the immunocompetent host. We report three cases of endoscopically-diagnosed HSVE in apparently immunocompetent children; the presentation was characterized by acute onset of fever, odynophagia, and dysphagia. In two cases, the diagnosis was confirmed histologically by identification of herpes viral inclusions and culture of the virus in the presence of inflammation. The third case was considered to have probable HSVE based on the presence of typical cold sore on his lip, typical endoscopic finding, histopathological evidence of inflammation in esophageal biopsies and positive serologic evidence of acute Herpes simplex virus infection. Two cases received an intravenous course of acyclovir and one had self-limited recovery. All three cases had normal immunological workup and excellent health on long-term follow-up.

  16. Eosinophilic esophagitis: From pathophysiology to treatment.

    Science.gov (United States)

    D'Alessandro, Alessandra; Esposito, Dario; Pesce, Marcella; Cuomo, Rosario; De Palma, Giovanni Domenico; Sarnelli, Giovanni

    2015-11-15

    Eosinophilic esophagitis (EoE) is a chronic immune disease, characterized by a dense eosinophilic infiltrate in the esophagus, leading to bolus impaction and reflux-like symptoms. Traditionally considered a pediatric disease, the number of adult patients with EoE is continuously increasing, with a relatively higher incidence in western countries. Dysphagia and food impaction represent the main symptoms complained by patients, but gastroesophageal reflux-like symptoms may also be present. Esophageal biopsies are mandatory for the diagnosis of EoE, though clinical manifestations and proton pump inhibitors responsiveness must be taken into consideration. The higher prevalence of EoE in patients suffering from atopic diseases suggests a common background with allergy, however both the etiology and pathophysiology are not completely understood. Elimination diets are considered the first-line therapy in children, but this approach appears less effective in adults patients, who often require steroids; despite medical treatments, EoE is complicated in some cases by esophageal stricture and stenosis, that require additional endoscopic treatments. This review summarizes the evidence on EoE pathophysiology and illustrates the safety and efficacy of the most recent medical and endoscopic treatments.

  17. Surgical Treatment of Perforation Esophageal Carcinoma

    Institute of Scientific and Technical Information of China (English)

    Depu Duan; Jihua Zou; Zhigang Cai; Shengyong Wu; Haibo Xiao; Yiyong Zhou; Xiang Liang; Dekui Sun; Songchang Wu

    2006-01-01

    OBJECTIVE To determine the ideal method of surgical preoperative treatment for perforation with esophageal carcinoma.METHODS 36 cases of perforation with esophageal carcinoma were treated surgically in this series.Perforations occurred into the right lung in14 cases ,the mediastinum in 17 cases and trachea in 5 cases.Open thoracic surgery was performed in 34 cases,in which the right thoracic approach using a 3-incision method was applied in 16 cases,and operation by stages in 15 cases.Of the 34 cases,retrosternal substitution of the esophagus with stomach or colon was performed in 26 cases.RESULTS Surgery was successful in 31 cases and operative death occurred in 3 cases.The postoperative follow up study was from 3~72months.Of these cases 15 wree alive at 7~12 months, 2 at 24 months,and 1 at 72 months. The results can be considered satisfactory.CONCLUSION The therapeutic results of surgical treatment of perforation with esophageal carcinoma were markedly superior to that of conventional conservative treatment. The authors suggest that surgical intervention without delay should be undertaken for patients having a perforation with carcinoma of the esophagus. A right thoracic approach with a 3-incision method (retrosternal replacement of esophagus with stomach or colon) or operation by stages is preferable.

  18. Esophageal button battery ingestion in children.

    Science.gov (United States)

    Şencan, Arzu; Genişol, İncinur; Hoşgör, Münevver

    2017-07-01

    Button battery lodged in the esophagus carries a high risk of morbidity and mortality. The purpose of this study was to present cases of patients with esophageal button battery ingestion treated at our clinic and to emphasize the importance of early diagnosis and treatment. Records of patients admitted to our hospital for foreign body ingestion between January 2010 and May 2015 were retrospectively reviewed. Cases with button battery lodged in the esophagus were included in the study. Patient data regarding age, sex, length of time after ingestion until admission, presenting clinical symptoms, type and localization of the battery, management, and prognosis were analyzed. Among 1891 foreign body ingestions, 71 were localized in the esophagus, and 8 of those (11.2%) were cases of button battery ingestion. Mean age was 1.7 years. Admission was within 6 hours of ingestion in 5 cases, after 24 hours had elapsed in 2, and 1 month after ingestion in 1 case. All patients but 1 knew the history of ingestion. Prompt endoscopic removal was performed for all patients. Three patients developed esophageal stricture, which responded to dilatation. Early recognition and timely endoscopic removal is mandatory in esophageal button battery ingestion. It should be suspected in the differential diagnosis of patients with persistent respiratory and gastrointestinal symptoms.

  19. γδ T cells and Foxp3(+) Treg cells infiltration in children with biliary atresia and its significance.

    Science.gov (United States)

    Li, Kang; Zhang, Xi; Tang, Shao-Tao; Yang, Li; Cao, Guo-Qing; Li, Shuai; Yang, De-Hua

    2015-01-01

    To investigate the changes in the proportion of γδ T cells and Foxp3(+) Treg cells in children with BA (biliary atresia). The distribution of γδ T cells in the liver tissues and the proportion of γδ T cells and Foxp3(+) Treg cells were observed and detected in BA Group (32 cases) and control group (CG) (12 cases) by using immunohistochemical methods and flow cytometry. The periportal bile duct of liver in BA Group was surrounded by a large number of γδ T cells and a certain degree of Foxp3(+) Treg cells infiltration. Additionally, the proportion of γδ T cells and Foxp3(+) Treg cells was significantly higher than that in CG (PTreg cells (PTreg cell proliferation in liver tissues of patients with biliary atresia exacerbated the progressive inflammatory injury of bile ducts.

  20. Partial trisomy 4q and partial monosomy 9p in a girl with choanal atresia and various dysmorphic findings.

    Science.gov (United States)

    Cakmak-Genc, Gunes; Karakas-Celik, Sevim; Dursun, Ahmet; Piskin, İbrahim Etem

    2015-09-01

    We report a new-born girl with partial trisomy of 4q28-qter and partial monosomy of 9p24-9ter. Our patient has choanal atresia, hypertelorism, wide nasal bridge, high arched palate, discrete nipples, heart defects, myoclonic seizures and various dysmorphic findings. Standard chromosomal analysis with G-banding with Trypsin-Giemsa revealed 46,XX,der(9)t(4;9)(q28;p24) resulting from the mother's t(4,9) (q28;p24) karyotype. Deletions of the terminal part of 9p and partial trisomy of chromosome 4q are rare chromosomal alterations. To our knowledge, this is the first report of choanal atresia in a patient with a partial trisomy of 4q28-qter and partial monosomy 9p24-9ter combination, which were detected by integrated cytogenetic and genomic analysis.