Extracorporeal Membrane Oxygenation in Premature Infants With Congenital Diaphragmatic Hernia.

Science.gov (United States)

Cuevas Guamán, Milenka; Akinkuotu, Adesola C; Cruz, Stephanie M; Griffiths, Pamela A; Welty, Stephen E; Lee, Timothy C; Olutoye, Oluyinka O

2017-11-14

Prematurity and low birth weight have been exclusion criteria for extracorporeal membrane oxygenation (ECMO); however, these criteria are not evidence based. With advances in anticoagulation, improved technology, and surgical expertise, it is difficult to deny a potential therapy based on these criteria alone. We report the outcome of three neonates who were ineligible based on traditional criteria but were offered ECMO as a life-saving measure. We highlight the interdisciplinary nature of modern decision-making. All three neonates had severe congenital diaphragmatic hernia diagnosed prenatally, had normal fetal karyotypes, were born prematurely, and weighed less than 2 kg. All three neonates underwent cervical venoarterial cannulation, stabilization on ECMO, and repair of their congenital diaphragmatic hernia early in their ECMO courses. All three infants had long courses of respiratory support attributable to lung hypoplasia, but there were no short- or long-term complications attributable to ECMO support directly. All three are alive at 2 years of age and were making progress developmentally. In conclusion, with interdisciplinary collaboration and clinical guidelines uniformly implemented, low birth weight infants may benefit from ECMO and should not be denied the therapy arbitrarily based on gestational age or size alone. Further research is essential to determine appropriate patient selection in premature infants.

Genetics Home Reference: congenital diaphragmatic hernia

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... of cells in the embryo. Environmental factors that influence development before birth may also increase the risk of ... occur? How can gene mutations affect health and development? More about Mutations and ... is only one affected individual in a family. When congenital diaphragmatic hernia occurs as a feature ...

Sphingolipids in Congenital Diaphragmatic Hernia; Results from an International Multicenter Study.

Directory of Open Access Journals (Sweden)

Kitty G Snoek

Full Text Available Congenital diaphragmatic hernia is a severe congenital anomaly with significant mortality and morbidity, for instance chronic lung disease. Sphingolipids have shown to be involved in lung injury, but their role in the pathophysiology of chronic lung disease has not been explored. We hypothesized that sphingolipid profiles in tracheal aspirates could play a role in predicting the mortality/ development of chronic lung disease in congenital diaphragmatic hernia patients. Furthermore, we hypothesized that sphingolipid profiles differ between ventilation modes; conventional mechanical ventilation versus high-frequency oscillation.Sphingolipid levels in tracheal aspirates were determined at days 1, 3, 7 and 14 in 72 neonates with congenital diaphragmatic hernia, born after > 34 weeks gestation at four high-volume congenital diaphragmatic hernia centers. Data were collected within a multicenter trial of initial ventilation strategy (NTR 1310.36 patients (50.0% died or developed chronic lung disease, 34 patients (47.2% by stratification were initially ventilated by conventional mechanical ventilation and 38 patients (52.8% by high-frequency oscillation. Multivariable logistic regression analysis with correction for side of the defect, liver position and observed-to-expected lung-to-head ratio, showed that none of the changes in sphingolipid levels were significantly associated with mortality /development of chronic lung disease. At day 14, long-chain ceramides 18:1 and 24:0 were significantly elevated in patients initially ventilated by conventional mechanical ventilation compared to high-frequency oscillation.We could not detect significant differences in temporal sphingolipid levels in congenital diaphragmatic hernia infants with mortality/development of chronic lung disease versus survivors without development of CLD. Elevated levels of ceramides 18:1 and 24:0 in the conventional mechanical ventilation group when compared to high

Congenital diaphragmatic hernia candidate genes derived from embryonic transcriptomes

DEFF Research Database (Denmark)

Russell, Meaghan K; Longoni, Mauro; Wells, Julie

2012-01-01

Congenital diaphragmatic hernia (CDH) is a common (1 in 3,000 live births) major congenital malformation that results in significant morbidity and mortality. The discovery of CDH loci using standard genetic approaches has been hindered by its genetic heterogeneity. We hypothesized that gene...

A fatal case of complicated congenital peritoneopericardial diaphragmatic hernia in a Holstein calf

OpenAIRE

Hicks, Keltie A.; Britton, Ann P.

2013-01-01

Congenital peritoneopericardial diaphragmatic hernia is a rare condition most commonly reported in cats and dogs. A 6-week-old Holstein heifer calf with a congenital peritoneopericardial diaphragmatic hernia complicated by a perforated abomasal ulcer is described. The clinical signs and pathological findings are compared with those reported in other species.

Epidemiology of congenital diaphragmatic hernia in Europe

DEFF Research Database (Denmark)

McGivern, Mark R.; Best, Kate E.; Rankin, Judith

2015-01-01

INTRODUCTION: Published prevalence rates of congenital diaphragmatic hernia (CDH) vary. This study aims to describe the epidemiology of CDH using data from high-quality, population-based registers belonging to the European Surveillance of Congenital Anomalies (EUROCAT). METHODS: Cases of CDH...... for isolated cases (ie, CDH cases that did not occur with any other congenital anomaly). There was significant variation in total and isolated CDH prevalence between registers. The proportion of cases that survived to 1 week was 69.3% (1392 cases) for total CDH cases and 72.7% (1107) for isolated cases...

A fatal case of complicated congenital peritoneopericardial diaphragmatic hernia in a Holstein calf

Science.gov (United States)

Hicks, Keltie A.; Britton, Ann P.

2013-01-01

Congenital peritoneopericardial diaphragmatic hernia is a rare condition most commonly reported in cats and dogs. A 6-week-old Holstein heifer calf with a congenital peritoneopericardial diaphragmatic hernia complicated by a perforated abomasal ulcer is described. The clinical signs and pathological findings are compared with those reported in other species. PMID:24155464

Congenital diaphragmatic hernia-associated pulmonary hypertension.

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Harting, Matthew T

2017-06-01

Congenital diaphragmatic hernia (CDH) is a complex entity wherein a diaphragmatic defect allows intrathoracic herniation of intra-abdominal contents and both pulmonary parenchymal and vascular development are stifled. Pulmonary pathology and pathophysiology, including pulmonary hypoplasia and pulmonary hypertension, are hallmarks of CDH and are associated with disease severity. Pulmonary hypertension (PH) is sustained, supranormal pulmonary arterial pressure, and among patients with CDH (CDH-PH), is driven by hypoplastic pulmonary vasculature, including alterations at the molecular, cellular, and tissue levels, along with pathophysiologic pulmonary vasoreactivity. This review addresses the basic mechanisms, altered anatomy, definition, diagnosis, and management of CDH-PH. Further, emerging therapies targeting CDH-PH and PH are explored. Published by Elsevier Inc.

Radionuclide scintigraphy in detection of gastroesophageal reflux in survivors of congenital diaphragmatic hernia

International Nuclear Information System (INIS)

Thomas, E.J.; Bharathi Dasan, J.; Patel, C.D.; Bal, C.S.; Malhotra, A.; Kumar, R.; Mitra, D.K.; Agarwala, S.; Padhy, A.K.

2003-01-01

Gastro-esophageal Reflux is a common cause of long-term morbidity in survivors of congenital diaphragmatic hernia. The present retrospective analysis was undertaken to find out the incidence of GER in survivors of congenital diaphragmatic hernia (CDH). A total of 26 survivors of congenital diaphragmatic hernia with a mean age of 19 months were studied. Of these, only 7 had clinical symptoms suggestive of GER such as recurrent respiratory tract infection, vomiting, regurgitation, and epigastric pain. The remaining 19 were asymptomatic and had radionuclide scintigraphy for detection of GER as a part of routine follow-up. All the 26 underwent radionuclide scintigraphy with 100-200 μCi (3.7 -7.4MBq) of Tc99m-Sulphur Colloid. GER was detected in 11 out of 26 patients (4 out of 7 symptomatic patients and 7 out of 19 asymptomatic patients). We concluded that there is high incidence of GER in survivors of congenital diaphragmatic hernia and these patients should be followed up for GER regularly. Scintiscanning being a simple, noninvasive test, may be used for initial evaluation and follow-up of survivors of CDH for GER. (author)

Bilious emesis as presenting symptom of congenital diaphragmatic hernia in an 8-month-old

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Daphney Clermont

2017-12-01

Full Text Available Although congenital diaphragmatic hernia (CDH primarily presents in either the prenatal or neonatal period, late presentation has a reported incidence of 2.5–25%. This case report describes an 8-month-old-male who presented to the emergency department with dehydration and a 24-h history of bilious emesis. Work-up led to the diagnosis of a left-sided Bochdalek CDH. He subsequently underwent laparoscopic diaphragmatic hernia repair with reduction of near-total length of incarcerated bowel. The patient had an uneventful recovery. Babies born with CDH are typically unstable at birth, and even with repair, the mortality rate remains high. However, in the case of delayed CDH diagnosis where there are no perinatal issues, surgical repair has very high success rates, provided that there are no complications regarding the delay. If there is not a high index of suspicion based on the patient's history and exam, delayed diagnosis and intervention with late CDH presentations can lead to complications which could otherwise be minimized. The purpose of this case report is to raise awareness and contribute to the growing knowledge about delayed CDH presentation among physicians and surgeons to facilitate early diagnosis and care of a morbid yet manageable condition.

Surgical management of the newborn with congenital diaphragmatic hernia.

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Tsao, Kuojen; Lally, Kevin P

2011-01-01

Despite advances in the surgical treatment and medical management over the last 20 years, neonates with congenital diaphragmatic hernia (CDH) remain one of the most challenging patient groups for all clinicians. Treatment strategies have shifted from emergent surgical repair and maximum ventilatory support to delayed repair and preoperative hemodynamic stabilization with lung-sparing ventilation strategies and extracorporeal membrane oxygenation. Subsequently, overall survival has improved to as high as 80% in some centers. However, specific treatment criteria are vague and highly variable amongst centers. This lack of consensus of these treatment modalities remains elusive due to the heterogeneity in disease severity as well as heterogeneity in patient care amongst centers. As a result of the rare incidence of disease and limited experience of individual centers, the evidence for CDH is typically reported as a homogenous disease largely supported by case series and networked-based studies. To better evaluate the data and compare treatment strategies, a classification and stratification of disease and centers is needed. Copyright © 2010 S. Karger AG, Basel.

A systematic review with meta-analysis of the prevalence of gastroesophageal reflux in congenital diaphragmatic hernia pediatric survivors.

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Arcos-Machancoses, J V; Ruiz Hernández, C; Martin de Carpi, J; Pinillos Pisón, S

2018-02-09

Congenital diaphragmatic hernia survivors are a well-known group at risk for developing gastroesophageal reflux disease that may be particularly long-term severe. The aim of this study is to provide a systematic review of the prevalence of gastroesophageal reflux in infant and children survivors treated for congenital diaphragmatic hernia.Electronic and manual searches were performed with keywords related to congenital diaphragmatic hernia, gastroesophageal reflux disease, and epidemiology terms. Summary estimates of the prevalence were calculated. Effect model was chosen depending on heterogeneity (I2). Factors potentially related with the prevalence, including study quality or the diagnostic strategy followed, were assessed by subgroup and meta-regression analyses. Risk of publication bias was studied by funnel plot analysis and the Egger test.The search yielded 140 articles, 26 of which were included in the analyses and provided 34 estimates of prevalence: 21 in patients aged 12 months or younger, and 13 in older children. The overall prevalence of gastroesophageal reflux disease in infants was 52.7% (95% confidence interval [CI]: 43.2% to 62.1%, I2 = 88.7%) and, in children over 1 year old, 35.1% (95% CI: 25.4% to 45.3%, I2 = 73.5%). Significant clinical and statistical heterogeneity was found. The strategy chosen for gastroesophageal reflux diagnosis influenced the reported prevalence. The only estimate obtained with a systematic use of multichannel intraluminal impedance provided a higher prevalence in both age groups: 83.3% (95% CI: 67.2% to 93.6%) and 61.1% (95% CI: 43.5% to 76.9%) respectively. This last prevalence did not significantly differ from that obtained using only low risk of bias estimates.As a conclusion, gastroesophageal reflux disease is commonly observed after congenital diaphragmatic hernia repair and is almost constantly present in the first months of life. It may be underdiagnosed if systematically esophageal monitoring is not

Imaging of congenital diaphragmatic hernias

International Nuclear Information System (INIS)

Taylor, George A.; Estroff, Judy A.; Atalabi, Omolola M.

2009-01-01

Congenital diaphragmatic hernias are complex and life-threatening lesions that are not just anatomic defects of the diaphragm, but represent a complex set of physiologic derangements of the lung, the pulmonary vasculature, and related structures. Imaging plays an increasingly important role in the care of these infants. Prenatal sonography and MRI have allowed early and accurate identification of the defect and associated anomalies. These tools have also been the key to defining the degree of pulmonary hypoplasia and to predicting neonatal survival and need for aggressive respiratory rescue strategies. In the postnatal period, conventional radiography supplemented by cross-sectional imaging in selected cases can be very useful in sorting out the differential diagnosis of intrathoracic masses, in the detection of associated anomalies, and in the management of complications. Understanding the pathogenesis of diaphragmatic defects, the underlying physiologic disturbances, and the strengths and limitations of current imaging protocols is essential to the effective and accurate management of these complex patients. (orig.)

Imaging of congenital diaphragmatic hernias

Energy Technology Data Exchange (ETDEWEB)

Taylor, George A.; Estroff, Judy A. [Harvard Medical School, Department of Radiology, Children' s Hospital Boston, Boston, MA (United States); Atalabi, Omolola M. [Harvard Medical School, Department of Radiology, Children' s Hospital Boston, Boston, MA (United States); The College of Medicine/University College Hospital, Ibadan (Nigeria)

2009-01-15

Congenital diaphragmatic hernias are complex and life-threatening lesions that are not just anatomic defects of the diaphragm, but represent a complex set of physiologic derangements of the lung, the pulmonary vasculature, and related structures. Imaging plays an increasingly important role in the care of these infants. Prenatal sonography and MRI have allowed early and accurate identification of the defect and associated anomalies. These tools have also been the key to defining the degree of pulmonary hypoplasia and to predicting neonatal survival and need for aggressive respiratory rescue strategies. In the postnatal period, conventional radiography supplemented by cross-sectional imaging in selected cases can be very useful in sorting out the differential diagnosis of intrathoracic masses, in the detection of associated anomalies, and in the management of complications. Understanding the pathogenesis of diaphragmatic defects, the underlying physiologic disturbances, and the strengths and limitations of current imaging protocols is essential to the effective and accurate management of these complex patients. (orig.)

A late presenting congenital diaphragmatic hernia misdiagnosed as spontaneous pneumothorax

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Chitra Sanjeev Juwarkar

2010-01-01

Full Text Available Congenital diaphragmatic hernia (CDH is described as (1 failure of diaphragmatic closure at development, (2 presence of herniated abdominal contents into chest and (3 pulmonary hypoplasia. Usually, pleural space is drained urgently when there is respiratory distress and radiological appearance of mediastinal shift. We present a case of a 5-month-old baby, diagnosed as tension pneumothorax and treated with chest drain insertion. CDH was the intraoperative diagnosis.

Laparoscopic repair of Morgagni diaphragmatic hernia in children ...

African Journals Online (AJOL)

Minimal invasive surgery allows for excellent visualisation of the diaphragm, and is increasingly used for the repair of diaphragmatic hernias in children. This report describes laparoscopic repairs between 2001 and 2007 of four Morgagni hernias in children. All defects were treated successfully using the laparoscopic ...

Recurrent Congenital Diaphragmatic Hernia in Ehlers-Danlos Syndrome

International Nuclear Information System (INIS)

Lin, I.C.; Ko, S.F.; Shieh, C.S.; Huang, C.F.; Chien, S.J.; Liang, C.D.

2006-01-01

Ehlers-Danlos syndrome (EDS) includes a group of connective tissue disorders with abnormal collagen metabolism and a diverse clinical spectrum. We report two siblings with EDS who both presented with congenital diaphragmatic hernia (CDH). The elder sister suffered from recurrent diaphragmatic hernia twice and EDS was overlooked initially. Echocardiography as well as contrast-enhanced magnetic resonance angiography (MRA) showed dilatation of the pulmonary artery, and marked elongation and tortuosity of the aorta and its branches. A diagnosis of EDS was eventually established when these findings were coupled with the clinical features of hyperelastic skin. Her younger brother also had similar features. This report emphasizes that EDS may present as CDH in a small child which could easily be overlooked. Without appropriate surgery, diaphragmatic hernia might occur. Echocardiographic screening is recommended in patients with CDH. Contrast-enhanced MRA can be helpful in delineation of abnormally tortuous aortic great vessels that are an important clue to the early diagnosis of EDS

Congenital diaphragmatic hernia with gastrointestinal symptomatology

International Nuclear Information System (INIS)

Siroka, M.; Bilicky, J.; Hernesniemi, B.

2014-01-01

The authors report a case of 6-week-old baby boy with congenital diaphragmatic hernia(CDH). He presented with nonspecific gastrointestinal symptoms. CDH is commonly manifested by nonspecific respiratory problems. The symptoms of CDH in older infants are atypical and misleading. Children may have only gastrointestinal problems even the acute abdomen. In our case, the ultrasound examination of abdomen did not detect the exact cause of vomiting and intolerance of oral intake, but the explanation has brought classic chest X-ray. (author)

Diaphragmatic hernia repair using a rectus abdominis muscle pedicle flap in three dogs.

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Chantawong, P; Komin, K; Banlunara, W; Kalpravidh, M

2013-01-01

To report the clinical use of a pedicle flap from the rectus abdominis muscle to repair extensive diaphragmatic tears in dogs with diaphragmatic hernia. Three dogs with a combination of radial and circumferential diaphragmatic tears were studied. The circumferential tear was repaired by suturing the wound edge with the edge at the abdominal wall. A pedicle flap of the rectus abdominis muscle was used for repairing the radial tear. The dogs were examined radiographically for lung and diaphragm appearance and evidence of reherniation at 10 days, and at one, two, and four months after surgery, and fluoroscopically for paradoxical motion of the diaphragm at one and four months. The rectus abdominis muscle pedicle flap was successfully used in all three dogs. The animals recovered uneventfully without evidence of reherniation during the four follow-up months. Fluoroscopic examination revealed no paradoxical motion of the diaphragm. A rectus abdominis muscle pedicle flap can be used for repairing large diaphragmatic defects in dogs.

Predictive capabilities of preoperative and postoperative pulmonary function tests in delayed repair of congenital diaphragmatic hernia.

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Tracy, T F; Bailey, P V; Sadiq, F; Noguchi, A; Silen, M L; Weber, T R

1994-02-01

To improve the survival of newborns with congenital diaphragmatic hernia (CHD), preoperative stabilization with conventional ventilatory therapy and extracorporeal membrane oxygenation (ECMO) have been used. Measurements that quantify pulmonary function may allow an accurate assessment of lethal pulmonary hypoplasia and predict outcome. Pulmonary function tests (PFTs) were obtained in 20 infants preoperatively and postoperatively; these included measurements of compliance, dynamic compliance, and tidal volume. Overall survival was 75%. Six surviving infants were initially managed with ventilator therapy alone, followed by repair (group 1). The remaining 14 patients, who were moribund at presentation or whose initial ventilator therapy failed, were placed on ECMO and received repair during bypass; nine survived (group 2), and five died (group 3). Compliance, dynamic compliance, and tidal volume obtained at initial presentation and immediately preoperatively were significantly higher for group 1 as compared with groups 2 and 3. Infants whose initial compliance was greater than 0.25 mL/cm H2O/kg and initial tidal volume was greater than 3.5 mL/kg did not require ECMO. Ultimate improvement in compliance was noted in 5 of 6 patients in group 1, 8 of 8 patients in group 2, and 5 of 5 in group 3. This improvement followed an initial decline in compliance in 9 of 14 survivors, from 15% to 76%. All six patients in group 1 had tidal volumes of more than 4 mL/kg, as did 7 of 9 patients in group 2. Only one patient among the ECMO nonsurvivors (group 3) had a postoperative tidal volume of this magnitude. These data suggest that initial PFTs may predict which infants will require ECMO.(ABSTRACT TRUNCATED AT 250 WORDS)

Laparoscopic repair of congenital pleuroperitoneal hernia using a polypropylene mesh in a dog

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H.F. Hartmann

2015-12-01

Full Text Available ABSTRACT Pleuroperitoneal hernias are the most uncommon type of diaphragmatic hernias in dogs and cats. The treatment of choice is surgery and may involve the use of prosthetic implant through celiotomy. In the current report, laparoscopic repair of a congenital pleuroperitoneal hernia using polypropylene mesh in a dog is described. The surgery was feasible. Appropriate reduction of the hernia was carried out and no complications were noted.

Congenital Diaphragmatic Hernia: Long-term Risk of Gastroesophageal Reflux Disease

NARCIS (Netherlands)

Peetsold, Marieke G.; Kneepkens, C. M. F. Frank; Heij, Hugo A.; Ijsselstijn, Hanneke; Tibboel, Dick; Gemke, Reinoud J. B. J.

2010-01-01

Objectives: Gastroesophageal reflux disease (GERD) is a well-recognized consequence of congenital diaphragmatic hernia (CDH). Results of studies examining predictive factors for early and late GERD are inconclusive. The aim of this study was to assess the incidence of early ( <2 years) and late GERD

Congenital Diaphragmatic Hernia: Long-term Risk of Gastroesophageal Reflux Disease

NARCIS (Netherlands)

Peetsold, M.G.; Kneepkens, C.M.F.; Heij, H.A.; IJsselstijn, H.; Tibboel, D.; Gemke, R.J.B.J.

2010-01-01

Objectives: Gastroesophageal reflux disease (GERD) is a well-recognized consequence of congenital diaphragmatic hernia (CDH). Results of studies examining predictive factors for early and lateGERDare inconclusive. The aim of this study was to assess the incidence of early (<2 years) and late GERD

Congenital Diaphragmatic Hernia and Occupational Therapy: A Case Report

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Bates, Angela C.

2011-01-01

This case report describes occupational therapy (OT) intervention in an outpatient setting and outcomes for a child diagnosed with congenital diaphragmatic hernia (CDH) from 4 to 28 months of age. There is little information on therapy intervention and outcomes of children who have survived. The patient is a white male, born at 35 weeks gestation…

Delayed presentation of congenital diaphragmatic hernia manifesting as combined-type acute gastric volvulus: a case report and review of the literature.

Science.gov (United States)

Anaya-Ayala, Javier E; Naik-Mathuria, Bindi; Olutoye, Oluyinka O

2008-03-01

Acute gastric volvulus associated with congenital diaphragmatic hernia is an unusual surgical emergency. We describe a case of an 11-year-old girl who presented with a 4-day history of abdominal pain, nonproductive retching, cough, and shortness of breath. A chest radiograph revealed a large air-fluid level in left hemithorax and the presence of intestinal loops with marked mediastinal deviation. Nasogastric decompression was unsuccessful. Via a thoracoscopic approach, the large fluid-filled stomach was percutaneously decompressed but could not be reduced. Through a left subcostal incision, a left-sided diaphragmatic defect about 4 x 5 cm was encountered. A large portion of small intestines, ascending and transverse colon, strangulated but viable stomach, and a large spleen herniated through the defect. The contents were reduced, revealing a combined gastric volvulus. Once the diaphragmatic defect was repaired primarily, there was insufficient space in the abdominal cavity to contain all the viscera reduced form the chest. Therefore, we placed an AlloDerm patch on the fascia and closed with a wound V.A.C (Kinetic Concepts Inc, San Antonio, TX). Two weeks later, the wound was definitively closed; she recovered uneventfully and was discharged home 3 days later. To our knowledge, only 26 previous cases of acute gastric volvulus complicating a congenital diaphragmatic hernia in children have been reported in the literature. Our patient represents the 27th case and the first combined type acute gastric volvulus case.

Congenital diaphragmatic hernia: A 4-year experience in a single ...

African Journals Online (AJOL)

Abstract. Background: This study aimed to evaluate congenital diaphragmatic hernia (CDH) patients in our department during a 4-year period. Patients and Methods: A retrospective study of 10 cases of CDH patients managed in the Neonatology and Pediatric Surgery Units of Goztepe Teaching Hospital from 2000 to 2004.

Prenatal imaging of a fetus with the rare combination of a right congenital diaphragmatic hernia and a giant omphalocele.

Science.gov (United States)

Nonaka, Ayasa; Hidaka, Nobuhiro; Kido, Saki; Fukushima, Kotaro; Kato, Kiyoko

2014-11-01

A co-existing right congenital diaphragmatic hernia and omphalocele is rare. We present images of a fetus diagnosed with this rare combination of anomalies. Early neonatal death occurred immediately after full-term birth due to severe respiratory insufficiency. In this case, disturbance of chest wall development due to the omphalocele rather than the diaphragmatic hernia was considered as the main cause of lung hypoplasia. Our experience suggests that caution should be exercised for severe respiratory insufficiency in a neonate with an omphalocele and diaphragmatic hernia, even in the absence of an intra-thoracic liver, one of the indicators of poor outcome for congenital diaphragmatic hernia. © 2014 Japanese Teratology Society.

Iatrogenic gastric perforation in a misdiagnosed case of late presenting congenital diaphragmatic hernia: Report of an avoidable complication

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Pradeep Kajal

Full Text Available Introduction: Congenital diaphragmatic hernia (CDH is a defect in diaphragm which usually presents with severe respiratory distress in neonatal period. Presentation of case: We present a case of congenital diaphragmatic hernia presenting at an age of 2.5 years in a male child. It was misdiagnosed as a case of pyothorax for which chest tube was attempted on left side resulting in iatrogenic gastric perforation. The patient was managed by early and prompt surgery. Discussion: Late presentation is usually rare with vast array of respiratory and gastrointestinal symptoms. It often leads to clinical and radiological misdiagnosis. Conclusion: Surgical intervention in misdiagnosed cases can lead to catastrophic iatrogenic complications. Keywords: Case report, Congenital diaphragmatic hernia, Pyothorax, Chest tube, Iatrogenic gastric perforation

Malformations associated with congenital diaphragmatic hernia: Impact on survival.

Science.gov (United States)

Bojanić, Katarina; Pritišanac, Ena; Luetić, Tomislav; Vuković, Jurica; Sprung, Juraj; Weingarten, Toby N; Schroeder, Darrell R; Grizelj, Ruža

2015-11-01

Congenital diaphragmatic hernia (CDH) is associated with high mortality. Survival is influenced by the extent of pulmonary hypoplasia and additional congenital defects. The purpose of this study was to assess the association of congenital anomalies and admission capillary carbon dioxide levels (PcCO2), as a measure of extent of pulmonary hypoplasia, on survival in neonates with CDH. This is a retrospective review of neonates with CDH admitted to a tertiary neonatal intensive care unit between 1990 and 2014. Logistic regression was used to assess whether hospital survival was associated with admission PcCO2 or associated anomalies (isolated CDH, CDH with cardiovascular anomalies, and CDH with noncardiac anomalies). The probabilities of survival (POS) score, based on birth weight and 5-min Apgar as defined by the Congenital Diaphragmatic Hernia Study Group were included as a covariate. Of 97 patients, 55 had additional malformations (cardiovascular n=12, noncardiac anomalies n=43). POS was lower in CDH with other anomalies compared to isolated CDH. Survival rate was 61.9%, 53.5% and 41.7% in isolated CDH, CDH with noncardiac anomalies and CDH with cardiovascular anomalies, respectively. After adjusting for POS score the likelihood of survival in CDH groups with additional anomalies was similar to isolated CDH (OR 0.95, 95% CI 0.22-4.15, and 1.10, 0.39-3.08, for CDH with and without cardiovascular anomalies, respectively). After adjusting for POS score, lower PcCO2 levels (OR=1.25 per 5mmHg decrease, P=0.003) were associated with better survival. Neonates with CDH have a high prevalence of congenital malformations. However, after adjusting for POS score the presence of additional anomalies was not associated with survival. The POS score and admission PcCO2 were important prognosticating factors for survival. Copyright © 2015 Elsevier Inc. All rights reserved.

Aggressive Surgical Management of Congenital Diaphragmatic Hernia: Worth the Effort?: A Multicenter, Prospective, Cohort Study.

Science.gov (United States)

Harting, Matthew T; Hollinger, Laura; Tsao, Kuojen; Putnam, Luke R; Wilson, Jay M; Hirschl, Ronald B; Skarsgard, Erik D; Tibboel, Dick; Brindle, Mary E; Lally, Pamela A; Miller, Charles C; Lally, Kevin P

2018-05-01

The objectives of this study were (i) to evaluate infants with congenital diaphragmatic hernia (CDH) that do not undergo repair, (ii) to identify nonrepair rate by institution, and (iii) to compare institutional outcomes based on nonrepair rate. Approximately 20% of infants with CDH go unrepaired and the threshold to offer surgical repair is variable. Data were abstracted from a multicenter, prospectively collected database. Standard clinical variables, including repair (or nonrepair), and outcome were analyzed. Institutions were grouped based on volume and rate of nonrepair. Preoperative mortality predictors were identified using logistic regression, expected mortality for each center was calculated, and observed /expected (O/E) ratios were computed for center groups and compared by Kruskal-Wallis ANOVA. A total of 3965 infants with CDH were identified and 691 infants (17.5%) were not repaired. Nonrepaired patients had lower Apgar scores (P HiLo = 5.1-16.7% and HiHi = 17.6-38.5%), leaving 3 groups: HiLo, HiHi, and Lo. Predictors of mortality were lower birth weight, lower Apgar scores, prenatal diagnosis, and presence of congenital anomalies. O/E ratios for mortality in the HiLo, HiHi, and Lo groups were 0.81, 0.94, and 1.21, respectively (P HiLo centers have 2.73 (2.4-3.1, 95% confidence interval) survivors beyond expectation. There are significant differences between repaired and nonrepaired CDH infants and significant center variation in rate of nonrepair exists. Aggressive surgical management, leading to a low rate of nonrepair, is associated with improved risk-adjusted mortality.

Laparoscopic diaphragmatic hernia repair using expanded polytetrafluoroethylene (ePTFE) for delayed traumatic diaphragmatic hernia.

Science.gov (United States)

Jee, Yeseob

2017-06-01

Traumatic diaphragmatic hernia (TDH) is an uncommon surgical problem, and diagnosis is often delayed. However, the mortality from bowel necrosis can reach 80%. Therefore, suspicion is needed and surgery is required to prevent complications. A 50-year-old man was transferred due to abdominal pain and vomiting. Chest X-ray and computed tomography (CT) scan showed herniation of the stomach through the left diaphragm. The patient had fallen down 15 months ago and CT scan at that time revealed a small defect of the diaphragm without herniation. We diagnosed delayed herniation of TDH and the patient underwent laparoscopic repair using an expanded polytetrafluoroethylene (ePTFE) mesh. Recovery was uneventful and the CT scan at 3 months after the operation showed no recurrence. We reported a delayed presenting TDH and considered a laparoscopic approach to be safe and feasible during elective surgery. Moreover, use of an ePTFE mesh for repair of large diaphragmatic hernia was also feasible.

Characteristics of Infants With Congenital Diaphragmatic Hernia Who Need Follow-Up of Pulmonary Hypertension.

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Kraemer, Ulrike S; Leeuwen, Lisette; Krasemann, Thomas B; Wijnen, René M H; Tibboel, Dick; IJsselstijn, Hanneke

2018-02-06

Pulmonary hypertension is one of the main causes of mortality and morbidity in patients with congenital diaphragmatic hernia. Currently, it is unknown whether pulmonary hypertension persists or recurs during the first year of life. Prospective longitudinal follow-up study. Tertiary university hospital. Fifty-two congenital diaphragmatic hernia patients admitted between 2010 and 2014. None. Pulmonary hypertension was measured using echocardiography and electrocardiography at 6 and 12 months old. Characteristics of patients with persistent pulmonary hypertension were compared with those of patients without persistent pulmonary hypertension. At follow-up, pulmonary hypertension persisted in four patients: at 6 months old, in three patients (patients A-C), and at 12 months old, in two patients (patients C and D). Patients with persistent pulmonary hypertension had a longer duration of mechanical ventilation (median 77 d [interquartile range, 49-181 d] vs median 8 d [interquartile range, 5-15 d]; p = 0.002) and hospital stay (median 331 d [interquartile range, 198-407 d) vs median 33 d (interquartile range, 16-59 d]; p = 0.003) than patients without persistent pulmonary hypertension. The proportion of patients with persistent pulmonary hypertension (n = 4) treated with inhaled nitric oxide (100% vs 31%; p = 0.01), sildenafil (100% vs 15%; p = 0.001), and bosentan (100% vs 6%; p pulmonary hypertension (n = 48). At 6 months, all patients with persistent pulmonary hypertension were tube-fed and treated with supplemental oxygen and sildenafil. Less than 10% of congenital diaphragmatic hernia patients had persistent pulmonary hypertension at ages 6 and/or 12 months. Follow-up for pulmonary hypertension should be reserved for congenital diaphragmatic hernia patients with echocardiographic signs of persistent pulmonary hypertension at hospital discharge and/or those treated with medication for pulmonary hypertension at hospital discharge.

Assessment and reduction of diaphragmatic tension during hiatal hernia repair.

Science.gov (United States)

Bradley, Daniel Davila; Louie, Brian E; Farivar, Alexander S; Wilshire, Candice L; Baik, Peter U; Aye, Ralph W

2015-04-01

During hiatal hernia repair there are two vectors of tension: axial and radial. An optimal repair minimizes the tension along these vectors. Radial tension is not easily recognized. There are no simple maneuvers like measuring length that facilitate assessment of radial tension. The aims of this project were to: (1) establish a simple intraoperative method to evaluate baseline tension of the diaphragmatic hiatal muscle closure; and, (2) assess if tension is reduced by relaxing maneuvers and if so, to what degree. Diaphragmatic characteristics and tension were assessed during hiatal hernia repair with a tension gage. We compared tension measured after hiatal dissection and after relaxing maneuvers were performed. Sixty-four patients (29 M:35F) underwent laparoscopic hiatal hernia repair. Baseline hiatal width was 2.84 cm and tension 13.6 dag. There was a positive correlation between hiatal width and tension (r = 0.55) but the strength of association was low (r (2) = 0.31). Four different hiatal shapes (slit, teardrop, "D", and oval) were identified and appear to influence tension and the need for relaxing incision. Tension was reduced by 35.8 % after a left pleurotomy (12 patients); by 46.2 % after a right crural relaxing incision (15 patients); and by 56.1 % if both maneuvers were performed (6 patients). Tension on the diaphragmatic hiatus can be measured with a novel device. There was a limited correlation with width of the hiatal opening. Relaxing maneuvers such as a left pleurotomy or a right crural relaxing incision reduced tension. Longer term follow-up will determine whether outcomes are improved by quantifying and reducing radial tension.

Early hemi-diaphragmatic plication following intraoperative phrenic nerve transection during complete AV canal repair

Directory of Open Access Journals (Sweden)

Hamad Alowayshiq

2018-04-01

Full Text Available Unilateral diaphragmatic palsy reduces pulmonary function by about 25% in older children and usually it is well tolerated; however, it causes severe respiratory distress in infants and young children. Diaphragmatic plication performed later than 10 days after cardiac surgery for patients under 1 year of age was associated with higher incidence of pneumonia and mortality. The management of the diaphragmatic paralysis due to phrenic nerve injury aiming mainly to preserve the respiratory function. Until now, the optimal management of diaphragmatic palsy in children who have undergone cardiac surgery remains controversial and consists of prolonged ventilation or diaphragmatic plication. In our case, many factors supported early diaphragmatic plication, the age of the patient, post-operative AV canal repair with severe pulmonary hypertension, and clear transection of the left phrenic nerve diagnosed intraoperatively.

Detection of gastroesophageal reflux in survivors of congenital diaphragmatic hernia: A radionuclide scintigraphic study in 26 children

International Nuclear Information System (INIS)

Thomas, E.J.; Bharathi Dasan, J.; Chandrasekhar, N.; Tripathi, M.; Kumar, R.; Kumar, A.; Malhotra, A.; Gupta, D.K.; Mitra, D.K.

2002-01-01

Introduction: Anatomical and functional esophageal abnormalities in survivors with CDH are well known. Gastro-esophageal Reflux (GER) is a common cause of long-term morbidity in survivors of congenital diaphragmatic hernia. The incidence of GER in these patients varies from 20-70% using various modalities. Aims: The present retrospective analysis was undertaken to find out the incidence of GER detected by radionuclide scintigraphy in survivors of congenital diaphragmatic hernia. Materials and Methods: Radionuclide scintigraphy for GER detection were performed in 26 survivors of congenital diaphragmatic hernia (17 male and 9 female) with a mean age of 19 months; age range 10 days to 56 months. Seven of the children had symptoms suggestive of GER. Of these 7, 3 had recurrent respiratory tract infection, 2 had regurgitation, 1 had vomiting and 1 had epigastric pain. The remaining 19 children were referred as part of routine follow up. All the children underwent radionuclide scintigraphy with 100-200 micro curie (3.7 -7.4MBq) of Tc99m-Sulphur Colloid. Results: The radionuclide scintigraphy detected GER in 11 out of 26(42.3%) children. Among the 7 symptomatic children, 4 (57%) had positive scintigraphic studies for reflux. Of these 4, 3(75%) had proximal reflux and 1 had distal reflux. Of the 19 asymptomatic patients, 7 (37%) were positive for GER on scintigraphy. Of these 7, 4 (57%) had proximal reflux and 3 (43%) had distal reflux. Conclusions: There is high incidence of GER in survivors of congenital diaphragmatic hernia irrespective of the presence or absence of symptoms suggestive of GER. Scintiscanning being a simple, noninvasive test can be used for initial evaluation of survivors of CDH for GER

[A case of strangulated congenital diaphragmatic hernia with necrosis and rupture of the colon and herniation into a left hemithorax in an adult (author's transl)].

Science.gov (United States)

Sarris, M; Georgoulis, J; Gatos, M; Dariotis, A

This is a case report of a successful repair of congenital diaphragmatic hernia in a 48 years old male that contained the transverse colon which was strangulated and ruptured in the left thoracic cavity. It was approached in two stages. First through a laparotomy the proximal part of the transverse colon was divided. The side going to the hernial sac was sutured and the proximal stump was anastomosed to the descending colon. In a second stage, two days later, through a felt thoracotomy the strangulated and ruptured colon was resected and the distal stump of the transverse colon was sutured and the hernia repaired.

Fetoscopic tracheal occlusion for severe congenital diaphragmatic hernia: retrospective study

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Angélica de Fátima de Assunção Braga

Full Text Available Abstract Background and objectives: The temporary fetal tracheal occlusion performed by fetoscopy accelerates lung development and reduces neonatal mortality. The aim of this paper is to present an anesthetic experience in pregnant women, whose fetuses have diaphragmatic hernia, undergoing fetoscopic tracheal occlusion (FETO. Method: Retrospective, descriptive study, approved by the Institutional Ethics Committee. Data were obtained from medical and anesthetic records. Results: FETO was performed in 28 pregnant women. Demographic characteristics: age 29.8 ± 6.5; weight 68.64 ± 12.26; ASA I and II. Obstetric: IG 26.1 ± 1.10 weeks (in FETO; 32.86 ± 1.58 (reversal of occlusion; 34.96 ± 2.78 (delivery. Delivery: cesarean section, vaginal delivery. Fetal data: Weight (g in the occlusion and delivery times, respectively (1045.82 ± 222.2 and 2294 ± 553; RPC in FETO and reversal of occlusion: 0.7 ± 0.15 and 1.32 ± 0.34, respectively. Preoperative maternal anesthesia included ranitidine and metoclopramide, nifedipine (VO and indomethacin (rectal. Preanesthetic medication with midazolam IV. Anesthetic techniques: combination of 0.5% hyperbaric bupivacaine (5-10 mg and sufentanil; continuous epidural predominantly with 0.5% bupivacaine associated with sufentanil, fentanyl, or morphine; general. In 8 cases, there was need to complement via catheter, with 5 submitted to PC and 3 to BC. Thirteen patients required intraoperative sedation; ephedrine was used in 15 patients. Fetal anesthesia: fentanyl 10-20 mg.kg-1 and pancuronium 0.1-0.2 mg.kg-1 (IM. Neonatal survival rate was 60.7%. Conclusion: FETO is a minimally invasive technique for severe congenital diaphragmatic hernia repair. Combined blockade associated with sedation and fetal anesthesia proved safe and effective for tracheal occlusion.

[Postnatal diagnosis of gastric volvulus revealing congenital diaphragmatic hernia].

Science.gov (United States)

Aprahamian, A; Nouyrigat, V; Grévent, D; Hervieux, E; Chéron, G

2017-05-01

Postnatally diagnosed congenital diaphragmatic hernias (CDH) are rare and have a better prognosis than those diagnosed prenatally. Postnatal symptoms can be respiratory, digestive, or mixed. Gastric volvulus can reveal CDH. Symptoms are pain, abdominal distension, and/or vomiting. Upper gastrointestinal barium X-ray radiography provides the diagnosis. Prognosis is related to early surgical management in complicated forms with intestinal occlusion or sub-occlusion. We report on an infant who presented with vomiting, which revealed gastric volvulus associated with a CDH. Progression was favorable after surgical treatment. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Inhalation of nitric oxide as a treatment of pulmonary hypertension in congenital diaphragmatic hernia

DEFF Research Database (Denmark)

Henneberg, Steen Winther; Jepsen, S; Andersen, P K

1995-01-01

Congenital diaphragmatic hernia (CDH) still has a mortality risk of around 40%. The concomitant pulmonary hypoplasia and the persistent pulmonary hypertension are of major prognostic importance. The use of a selective pulmonary vasodilator may revert this vicious circle that is fatal to many...

Pulmonary surfactant protein A, B, and C mRNA and protein expression in the nitrofen-induced congenital diaphragmatic hernia rat model

NARCIS (Netherlands)

van Tuyl, Minke; Blommaart, Piet jan E.; Keijzer, Richard; Wert, Susan E.; Ruijter, Jan M.; Lamers, Wouter H.; Tibboel, Dick

2003-01-01

Neonates with congenital diaphragmatic hernia (CDH) suffer from a diaphragmatic defect, lung hypoplasia, and pulmonary hypertension, with poor lung function forming the major clinical challenge. Despite prenatal diagnosis and advanced postnatal treatment strategies, the mortality rate of CDH is

Congenital diaphragmatic hernia interval on chromosome 8p23.1 characterized by genetics and protein interaction networks

DEFF Research Database (Denmark)

Longoni, Mauro; Hansen, Kasper Lage; Russell, Meaghan K.

2012-01-01

Chromosome 8p23.1 is a common hotspot associated with major congenital malformations, including congenital diaphragmatic hernia (CDH) and cardiac defects. We present findings from high‐resolution arrays in patients who carry a loss (n = 18) or a gain (n = 1) of sub‐band 8p23.1. We confirm a region...

Acute gastric volvulus and congenital diaphragmatic hernia, case report and review

Directory of Open Access Journals (Sweden)

Laura Pérez-Egido

2015-01-01

Full Text Available Congenital diaphragmatic hernia (CDH is the result of the incomplete fusion and closure of the pleuroperitoneal canal during the fetal development. CDH is usually diagnosed prenatally but, if undiagnosed, the clinical presentation ranges from asymptomatic children to serious respiratory or gastrointestinal symptoms. Acute gastric volvulus associated with CDH is a rare surgical emergency in children. We report two cases of acute gastric volvulus associated with CDH and review the literature.

Management of Postoperative Pulmonary Hypertension by Inhaled Nitric Oxide in a Newborn with Congenital Diaphragmatic Hernia

International Nuclear Information System (INIS)

Fettah, N. D.; Dilli, D.; Beken, S.; Zenciroglu, A.; Okumus, N.; Cavusoglu, H.; Ozgur, S.

2014-01-01

Postero-lateral congenital diaphragmatic hernia (CDH) is a life threatening anomaly characterized by diaphragmatic defect and intrathoracic herniation of abdominal viscera. In patients with CDH, the lungs are hypoplastic and persistent pulmonary hypertension develops in most cases. Although, inhaled nitric oxide (iNO) results in a reduction in pulmonary hypertension with improvement in oxygenation, its benefit in the patients with CDH remains controversial. In this report, the authors present successful management of postoperative pulmonary hypertension by iNO in a newborn with CDH. (author)

Imaging diagnosis--positive contrast peritoneographic features of true diaphragmatic hernia.

Science.gov (United States)

Choi, Jihye; Kim, Hyunwook; Kim, Mieun; Yoon, Junghee

2009-01-01

A true diaphragmatic hernia is a congenital diaphragmatic malformation that can appear identical to a peritoneopericardial diaphragmatic hernia (PPDH). True diaphragmatic hernias are rare in dogs. Herein we describe the use of positive contrast peritoneography for diagnosis of a true diaphragmatic hernia in two dogs.

Transient megaoesophagus and oesophagitis following diaphragmatic rupture repair in a cat.

Science.gov (United States)

Joseph, Rotem; Kuzi, Sharon; Lavy, Eran; Aroch, Itamar

2008-07-01

A 6-month-old domestic shorthair female cat was presented with suspected diaphragmatic hernia (DH) that was later confirmed by thoracic radiography. The cat underwent exploratory celiotomy with a diaphragmatic rupture (DR) repair and recovered. Six days later, it was represented with vomiting and anorexia. Megaoesophagus (MO) and gastric dilatation were diagnosed by contrast radiography. A second celiotomy revealed no abnormalities and gastropexy was performed. Endoscopy demonstrated MO, oesophagitis and gastro-oesophageal reflux. MO persisted for several weeks and was an unexpected complication as no association between DR (or DH) and MO has never been described in the veterinary literature. The cat was treated medically with aggressive prokinetic and antacid therapy along with prolonged temporary oesophageal diversion (percutaneous endoscopic gastrostomy tube) with an excellent outcome.

Imaging findings in fetal diaphragmatic abnormalities

Energy Technology Data Exchange (ETDEWEB)

Alamo, Leonor; Gudinchet, Francois [University Hospital Center of Lausanne, Unit of Radiopediatrics, Department of Radiology, Lausanne (Switzerland); Meuli, Reto [University Hospital Center of Lausanne, Department of Radiology, Lausanne (Switzerland)

2015-12-15

Imaging plays a key role in the detection of a diaphragmatic pathology in utero. US is the screening method, but MRI is increasingly performed. Congenital diaphragmatic hernia is by far the most often diagnosed diaphragmatic pathology, but unilateral or bilateral eventration or paralysis can also be identified. Extralobar pulmonary sequestration can be located in the diaphragm and, exceptionally, diaphragmatic tumors or secondary infiltration of the diaphragm from tumors originating from an adjacent organ have been observed in utero. Congenital abnormalities of the diaphragm impair normal lung development. Prenatal imaging provides a detailed anatomical evaluation of the fetus and allows volumetric lung measurements. The comparison of these data with those from normal fetuses at the same gestational age provides information about the severity of pulmonary hypoplasia and improves predictions about the fetus's outcome. This information can help doctors and families to make decisions about management during pregnancy and after birth. We describe a wide spectrum of congenital pathologies of the diaphragm and analyze their embryological basis. Moreover, we describe their prenatal imaging findings with emphasis on MR studies, discuss their differential diagnosis and evaluate the limits of imaging methods in predicting postnatal outcome. (orig.)

Associated morbidities to congenital diaphragmatic hernia and a relationship to human milk.

Science.gov (United States)

Froh, Elizabeth B; Spatz, Diane L

2012-08-01

The majority of what is known in the recent literature regarding human milk studies in the neonatal intensive care setting is specific to term and/or preterm infants (including very-low-birth-weight preterm infants). However, there is a lack of human milk and breastfeeding literature concerning infants with congenital anomalies, specifically infants diagnosed with congenital diaphragmatic hernia (CDH). By applying human milk research conducted among other populations of infants, this article highlights how human milk may have a significant impact on infants with CDH. Recent human milk studies are reviewed and then applied to the CDH population in regard to respiratory and gastrointestinal morbidities, as well as infection and length of stay. In addition, clinical implications of these relationships are discussed and suggestions for future research are presented.

Experience with the Management of Congenital Diaphragmatic ...

African Journals Online (AJOL)

Those who did not undergo surgery had lower Apgar scores and higher incidence of associated congenital malformations than the patients who underwent surgical repairs. The differences in the mean of 5 minute Apgar scores among the three groups were not statistically significant (p> 0.05). Age at surgery was higher in ...

The value of fetal ultrasonography and magnetic resonance imaging in the assessment of diaphragmatic hernias

International Nuclear Information System (INIS)

Amim, Bruno; Guerra, Fernando; Marchiori, Edson; Gasparetto, Emerson Leandro

2008-01-01

To demonstrate the relevance of ultrasonography and magnetic resonance imaging findings in the prenatal characterization and prognostic evaluation in cases of congenital diaphragmatic hernia. Materials And Methods: Fourteen pregnant women (mean gestational age = 28.7 weeks) who had undergone ultrasonography for suspicion of fetuses with congenital diaphragmatic hernia were assessed by means of magnetic resonance imaging on a 1.5 tesla equipment, following the standard protocol. Two radiologists evaluated the images and the findings were defined by consensus. Results: Twelve fetuses had left diaphragmatic hernia and two, right diaphragmatic hernia. Ultrasonography showed the fetal liver inside the thorax of five fetuses (three with left diaphragmatic hernia, and two with right diaphragmatic hernia) and magnetic resonance imaging in eight fetuses (six with left diaphragmatic hernia, and two with right diaphragmatic hernia). Stomach and small bowel loop herniation was observed in all of the fetuses with left diaphragmatic hernia (n = 12) at both magnetic resonance imaging and ultrasonography. Eight fetuses (seven with left diaphragmatic hernia and one with right diaphragmatic hernia) survived after surgical treatment. Conclusion: Ultrasonography and magnetic resonance imaging are complementary imaging methods in the evaluation of congenital diaphragmatic hernia. Magnetic resonance imaging is a helpful diagnostic method complementary to ultrasonography for evaluation of the fetal liver positioning, considering its relevance as a prognostic factor in cases of congenital diaphragmatic hernia. (author)

Neonatal diabetes mellitus and congenital diaphragmatic hernia: coincidence or concurrent etiology?

Directory of Open Access Journals (Sweden)

Topiol Emmanuelle S

2012-07-01

Full Text Available Abstract Neonatal diabetes mellitus (NDM is a rare metabolic disorder, affecting approximately 1 in 500,000 live births. The management of NDM is challenging, as the benefits of controlling hyperglycemia must be balanced with the risks of iatrogenic hypoglycemia. NDM occurs in both permanent and transient forms, which have been genetically and phenotypically well characterized. Herein, we present the previously unreported combination of transient NDM (TNDM and congenital diaphragmatic hernia (CDH. In addition to reviewing the management and genetics of NDM we discuss the potential for overlapping genetic or embryologic abnormalities to explain the concurrence of CDH and NDM.

Congenital diaphragmatic hernia with gastric volvulus

OpenAIRE

Jain, Prashant; Sanghavi, Beejal; Sanghani, Hemanshi; Parelkar, S. V.; Borwankar, S. S.

2007-01-01

Gastric volvulus is a surgical emergency presenting in various forms. Association with diaphragmatic defect is well known. Here we describe three cases of gastric volvulus associated with diaphragmatic defect having varied presentations and their management. A rare case of gastric volvulus with complete gangrene of the stomach is also reported. Three types of gastric volvulus have been described depending on the rotation axis: organoaxial, mesentericoaxial and combination of both types. Opera...

Congenital diaphragmatic hernia: neonatal outcomes following referral to a paediatric surgical centre.

LENUS (Irish Health Repository)

Chukwu, J

2012-02-01

Congenital diaphragmatic hernia (CDH) is a rare malformation observed in approximately 1 in 3000 live births. Estimates of postnatal survival range from 50 to 70% despite advances in neonatal care. Antenatal diagnosis is associated with termination of pregnancy in 25-50% pregnancy internationally which may not be reflective of the Irish population. We aimed to evaluate the mortality of infants with CDH who survived to admission in a tertiary referral paediatric hospital between 1996 and 2007. The Hospital In-Patient Enquiry system was used to determine the number of neonatal referrals for CDH to OLHSC between 1996 and 2007. Mortality, sex distribution, length of patient stay and the number of cases per year were examined. 141 neonates with CDH were over 12 years with approximately 12 referrals per annum of which 82 (58%) were male and 59 (42%) female. The average length of stay in the hospital was 33 (range 0-364) days. Overall 71% of the patients survived to discharge. In the first epoch (1996-2001) survival was 63% compared with 78% in the later epoch (2002-7). The overall survival for neonates with CDH presenting to OLCHC during the 12 year-period was 71% although this improved to 78% in recent epoch. Further study of associated congenital anomalies, number of terminations of pregnancy, complexity of the diaphragmatic defect and degree of pulmonary hypertension are required to compare this population with other international centres.

Pulmonary Vascular Defects in Congenital Diaphragmatic Hernia : the quest for early factors and intervention : Pulmonale vasculaire defecten in congenitale hernia diafragmatica : de zoektocht naar vroege factoren en interventie

NARCIS (Netherlands)

D.S. Mous (Daphne)

2017-01-01

markdownabstractCongenital diaphragmatic hernia (CDH) is a severe anomaly characterized by a diaphragmatic defect, lung hypoplasia and pulmonary hypertension. The associated pulmonary abnormalities are responsible for the high morbidity and mortality among patients with this disease. Vasodilator

Congenital peritoneopericardial diaphragmatic hernia in a terrier dog

Directory of Open Access Journals (Sweden)

Reza Kheirandish

2014-06-01

Full Text Available A one-month-old male terrier dog was referred in shock status with a history of anorexia, tachypnea, abdominal distention and progressive weight loss. Auscultation of right side of the lungs found enhanced respiratory noises. The thorough auscultation of the opposite side of the chest revealed the presence of typical intestinal sounds. Cardiac auscultation revealed muffled heart sounds and a diminished palpable precordial cardiac impulse was evident. The radiograph showed the presence of gas within the bowel in abrupt contrast to the adjacent structures of soft tissue opacity. Conservative treatment was failed and the animal died. At necropsy, cranial displacement of abdominal viscera into the pericardial sac was seen. A definitive diagnosis of peritoneopericardial diaphragmatic hernia was made. Although congenital pericardial diseases are rare in dogs, awareness of the clinical manifestation of these kinds of defects combined with early use of available imaging modalities can yield a preoperative diagnosis.

Hernia diagfragmática congénita derecha en el Hospital Universitario de Santander Right congenital diaphragmatic hernia at the Hospital Universitario de Santander

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Julio César Mantilla

2010-08-01

Full Text Available Introducción: La hernia diafragmática del lado derecho es una variable poco frecuente de los defectos congénitos diafragmáticos que permiten el paso del contenido abdominal a la cavidad torácica, causando graves trastornos en el desarrollo pulmonar fetal. Objetivo: Describir las características patológicas encontradas en la autopsia perinatal de un paciente con Hernia diafragmática congénita derecha en el Hospital Universitario de Santander. Caso clínico: Neonato de 35 semanas de gestación con diagnóstico prenatal de Hernia Diafragmática Congénita, quien fallece minutos después de su nacimiento debido a insuficiencia respiratoria aguda. En los hallazgos de autopsia se encuentra ausencia de la mayor parte del hemidiafragma derecho, herniación del contenido abdominal al tórax y una severa hipoplasia pulmonar. Conclusion: La Hernia diafragmática congénita del lado derecho se asocia con alta mortalidad neonatal y los hallazgos encontrados en el presente caso se correlacionan con los graves defectos estructurales pulmonares que se describen en otros casos reportados en la literatura. Salud UIS 2010; 42: 133-138Introduction: The congenital diaphragmatic hernia of the right side is the least common type of the congenital diaphragmatic defects which allows the passage of abdominal contents to the thoracic cavity, causing serious disorders on lung development. Objective: To describe the pathological features found in perinatal autopsy of a patient with Congenital Diaphragmatic Hernia of the right side at the Hospital Universitario de Santander. Case report: 35 weeks gestation neonate with prenatal diagnosis of congenital diaphragmatic hernia, who died due to acute respiratory failure. In the autopsy be found a severe pulmonary hypoplasia and in the microscopic examination, the pulmonary alveoli collapsed. Conclusion: The congenital diaphragmatic hernia of the right side is associated with high neonatal mortality and the findings in

High temporal versus high spatial resolution in MR quantitative pulmonary perfusion imaging of two-year old children after congenital diaphragmatic hernia repair

Energy Technology Data Exchange (ETDEWEB)

Weidner, M.; Hagelstein, C.; Schoenberg, S.O.; Neff, K.W. [University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Institute of Clinical Radiology and Nuclear Medicine, Mannheim (Germany); Zoellner, F.G.; Schad, L.R. [Heidelberg University, Computer Assisted Clinical Medicine, Medical Faculty Mannheim, Mannheim (Germany); Zahn, K. [University Medical Center Mannheim, Medical Faculty Mannheim, University of Heidelberg, Department of Pediatric Surgery, Mannheim (Germany); Schaible, T. [University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Department of Pediatrics, Mannheim (Germany)

2014-10-15

Congenital diaphragmatic hernia (CDH) leads to lung hypoplasia. Using dynamic contrast-enhanced (DCE) MR imaging, lung perfusion can be quantified. As MR perfusion values depend on temporal resolution, we compared two protocols to investigate whether ipsilateral lung perfusion is impaired after CDH, whether there are protocol-dependent differences, and which protocol is preferred. DCE-MRI was performed in 36 2-year old children after CDH on a 3 T MRI system; protocol A (n = 18) based on a high spatial (3.0 s; voxel: 1.25 mm{sup 3}) and protocol B (n = 18) on a high temporal resolution (1.5 s; voxel: 2 mm{sup 3}). Pulmonary blood flow (PBF), pulmonary blood volume (PBV), mean transit time (MTT), and peak-contrast-to-noise-ratio (PCNR) were quantified. PBF was reduced ipsilaterally, with ipsilateral PBF of 45 ± 26 ml/100 ml/min to contralateral PBF of 63 ± 28 ml/100 ml/min (p = 0.0016) for protocol A; and for protocol B, side differences were equivalent (ipsilateral PBF = 62 ± 24 vs. contralateral PBF = 85 ± 30 ml/100 ml/min; p = 0.0034). PCNR was higher for protocol B (30 ± 18 vs. 20 ± 9; p = 0.0294). Protocol B showed higher values of PBF in comparison to protocol A (p always <0.05). Ipsilateral lung perfusion is reduced in 2-year old children following CDH repair. Higher temporal resolution and increased voxel size show a gain in PCNR and lead to higher perfusion values. Protocol B is therefore preferred. (orig.)

Evaluation of lung function changes before and after surfactant application during artificial ventilation in newborn rats with congenital diaphragmatic hernia

NARCIS (Netherlands)

E.C. Scheffers; H. IJsselstijn (Hanneke); R. Tenbrinck (Robert); B.F. Lachmann (Burkhard); J.C. de Jongste (Johan); J.C. Molenaar; D. Tibboel (Dick)

1994-01-01

textabstractPatients with congenital diaphragmatic hernia (CDH) have unilateral or bilateral hypoplasia of the lungs including delayed maturation of the terminal air sacs. Because these lungs are highly susceptible to barotrauma and oxygen toxicity, even in full-term newborns, continued research

Spatial and temporal expression of glucocorticoid, retinoid, and thyroid hormone receptors is not altered in lungs of congenital diaphragmatic hernia

NARCIS (Netherlands)

Rajatapiti, Prapapan; Keijzer, Richard; Blommaart, Pietjan E.; Lamers, Wouter H.; de Krijger, Ronald R.; Visser, Theo J.; Tibboel, Dick; Rottier, Robbert

2006-01-01

The degree of associated pulmonary hypoplasia and persistent pulmonary hypertension are major determination factors for survival in congenital diaphragmatic hernia (CDH) patients. Glucocorticoids, thyroid hormone, and vitamin A have been shown to be involved in human lung development. To determine

[Prenatal diagnosis of a right thoracic congenital ectopic kidney with a diaphragmatic hernia: a combination with a good prognosis].

Science.gov (United States)

Cessans, C; Pharamin, J; Crouzet, K; Kessler, S; Puget, C; Bouali, O; Galinier, P; Marcoux, M-O

2015-11-01

Ectopic intrathoracic kidney is a rare congenital anomaly, usually asymptomatic. This anomaly is sometimes associated with a diaphragmatic hernia. Few cases of this combination have been described, often in the absence of a prenatal diagnosis. We report on the case of a female newborn infant who was diagnosed with an ectopic intrathoracic right kidney and a diaphragmatic hernia upon 33 weeks of gestation. The patient underwent surgery on the first day of life and the respiratory and renal outcomes were simple. We review the literature and discuss the seemingly good prognosis of this combination. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

Region of interest-based versus whole-lung segmentation-based approach for MR lung perfusion quantification in 2-year-old children after congenital diaphragmatic hernia repair

International Nuclear Information System (INIS)

Weis, M.; Sommer, V.; Hagelstein, C.; Schoenberg, S.O.; Neff, K.W.; Zoellner, F.G.; Zahn, K.; Schaible, T.

2016-01-01

With a region of interest (ROI)-based approach 2-year-old children after congenital diaphragmatic hernia (CDH) show reduced MR lung perfusion values on the ipsilateral side compared to the contralateral. This study evaluates whether results can be reproduced by segmentation of whole-lung and whether there are differences between the ROI-based and whole-lung measurements. Using dynamic contrast-enhanced (DCE) MRI, pulmonary blood flow (PBF), pulmonary blood volume (PBV) and mean transit time (MTT) were quantified in 30 children after CDH repair. Quantification results of an ROI-based (six cylindrical ROIs generated of five adjacent slices per lung-side) and a whole-lung segmentation approach were compared. In both approaches PBF and PBV were significantly reduced on the ipsilateral side (p always <0.0001). In ipsilateral lungs, PBF of the ROI-based and the whole-lung segmentation-based approach was equal (p=0.50). In contralateral lungs, the ROI-based approach significantly overestimated PBF in comparison to the whole-lung segmentation approach by approximately 9.5 % (p=0.0013). MR lung perfusion in 2-year-old children after CDH is significantly reduced ipsilaterally. In the contralateral lung, the ROI-based approach significantly overestimates perfusion, which can be explained by exclusion of the most ventral parts of the lung. Therefore whole-lung segmentation should be preferred. (orig.)

Region of interest-based versus whole-lung segmentation-based approach for MR lung perfusion quantification in 2-year-old children after congenital diaphragmatic hernia repair

Energy Technology Data Exchange (ETDEWEB)

Weis, M.; Sommer, V.; Hagelstein, C.; Schoenberg, S.O.; Neff, K.W. [Heidelberg University, Institute of Clinical Radiology and Nuclear Medicine, University Medical Center Mannheim, Medical Faculty Mannheim, Mannheim (Germany); Zoellner, F.G. [Heidelberg University, Computer Assisted Clinical Medicine, Medical Faculty Mannheim, Mannheim (Germany); Zahn, K. [University of Heidelberg, Department of Paediatric Surgery, University Medical Center Mannheim, Medical Faculty Mannheim, Mannheim (Germany); Schaible, T. [Heidelberg University, Department of Paediatrics, University Medical Center Mannheim, Medical Faculty Mannheim, Mannheim (Germany)

2016-12-15

With a region of interest (ROI)-based approach 2-year-old children after congenital diaphragmatic hernia (CDH) show reduced MR lung perfusion values on the ipsilateral side compared to the contralateral. This study evaluates whether results can be reproduced by segmentation of whole-lung and whether there are differences between the ROI-based and whole-lung measurements. Using dynamic contrast-enhanced (DCE) MRI, pulmonary blood flow (PBF), pulmonary blood volume (PBV) and mean transit time (MTT) were quantified in 30 children after CDH repair. Quantification results of an ROI-based (six cylindrical ROIs generated of five adjacent slices per lung-side) and a whole-lung segmentation approach were compared. In both approaches PBF and PBV were significantly reduced on the ipsilateral side (p always <0.0001). In ipsilateral lungs, PBF of the ROI-based and the whole-lung segmentation-based approach was equal (p=0.50). In contralateral lungs, the ROI-based approach significantly overestimated PBF in comparison to the whole-lung segmentation approach by approximately 9.5 % (p=0.0013). MR lung perfusion in 2-year-old children after CDH is significantly reduced ipsilaterally. In the contralateral lung, the ROI-based approach significantly overestimates perfusion, which can be explained by exclusion of the most ventral parts of the lung. Therefore whole-lung segmentation should be preferred. (orig.)

Prematurity and fetal lung response after tracheal occlusion in fetuses with severe congenital diaphragmatic hernia.

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Sananes, Nicolas; Rodo, Carlota; Peiro, Jose Luis; Britto, Ingrid Schwach Werneck; Sangi-Haghpeykar, Haleh; Favre, Romain; Joal, Arnaud; Gaudineau, Adrien; Silva, Marcos Marques da; Tannuri, Uenis; Zugaib, Marcelo; Carreras, Elena; Ruano, Rodrigo

2016-09-01

To evaluate the independent association of fetal pulmonary response and prematurity to postnatal outcomes after fetal tracheal occlusion for congenital diaphragmatic hernia. Fetal pulmonary response, prematurity (prematurity (prematurity was not statistically associated with mortality after controlling for fetal pulmonary response (aOR 0.52, 95% CI 0.12-2.30, p=0.367). Fetal pulmonary response after FETO is the most important factor associated with survival, independently from the gestational age at delivery.

Diaphragmatic hernia in the Coffin-Siris syndrome.

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Delvaux, V; Moerman, P; Fryns, J P

1998-01-01

At 32 weeks of gestation, delivery of a female fetus was induced because of severe malformations seen on ultrasonogram: congenital diaphragmatic hernia and cerebellar hypoplasia. The diagnosis of Coffin-Siris syndrome was based on the physical examination: coarse face with low-set ears, low nuchal hairline, scalp hypotrichosis and hypoplasia of the nails of fingers and toes with absence of the right fifth fingernail. Autopsy confirmed the prenatally diagnosed major associated abnormalities: hypoplasia of the cerebellum and congenital diaphragmatic hernia. Various clinical entities are included in the differential diagnosis.

Posterolateral diaphragmatic hernia with small-bowel incarceration ...

African Journals Online (AJOL)

Bochdalek hernia (BH), a closing defect of the peripheral posterior aspect of the diaphragm, is the most common of the congenital diaphragmatic hernias and is usually diagnosed in neonates. Symptomatic presentation of a right-sided diaphragmatic hernia in an adult is unusual. Owing to their rarity and varied presentation, ...

Radiological diagnosis of congenital diaphragmatic hernia in 17th century Korean mummy.

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Yi-Suk Kim

Full Text Available Congenital diaphragmatic hernia (CDH is a birth defect of the diaphragm resulting in pulmonary sequelae that threaten the lives of infants. In computed tomography (CT images of a 17th century middle-aged male mummy (the Andong mummy, we observed that the abdominal contents had protruded into the right thoracic cavity through the diaphragmatic defect, accompanied by a mediastinal shift to the left. On autopsy, the defect in the right posterolateral aspect of the diaphragm was reconfirmed, as was the herniation of the abdominal organs. The herniated contents included the right lobe of the liver, the pyloric part of the stomach, a part of the greater omentum, and the right colic flexure connecting the superior part of the ascending colon and the right part of the transverse colon. Taking our CT and autopsy results together, this case was diagnosed as the Bochdalek-type CDH. Herein we make the first ever report of a CT-assisted diagnosis of a pre-modern historical case of CDH. Our results show the promising utility of this modality in investigations of mummified human remains archaeologically obtained.

[Congenital diaphragmatic hernia: respiratory and vascular outcomes].

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Pennaforte, T; Rakza, T; Sfeir, R; Aubry, E; Bonnevalle, M; Fayoux, P; Deschildre, A; Thumerelle, C; de Lagausie, P; Benachi, A; Storme, L

2012-02-01

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care, and new postnatal treatment strategies, the rates of mortality and morbidity in the newborn with CDH remain high as the result of severe respiratory failure secondary to PH and PPH. Later, lung function assessments show obstructive and restrictive impairments due to altered lung structure and lung damage due to prolonged ventilatory support. The long-term consequences of pulmonary hypertension are unknown. Other problems include chronic pulmonary aspiration caused by gastro-oesophageal reflux and respiratory manifestations of allergy such as asthma or rhinitis. Finally, failure to thrive may be caused by increased caloric requirements due to pulmonary morbidity. Follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols. Copyright © 2012. Published by Elsevier Masson SAS.

Distal 4p microdeletion in a case of Wolf-Hirschhorn syndrome with congenital diaphragmatic hernia.

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Casaccia, Germana; Mobili, Luisa; Braguglia, Annabella; Santoro, Francesco; Bagolan, Pietro

2006-03-01

Wolf-Hirschhorn syndrome (WHS) is a well-known genetic condition characterized by typical facial anomalies, midline defects, skeletal anomalies, prenatal and postnatal growth retardation, hypotonia, mental retardation, and seizures. Affected patients with a microdeletion on distal 4p present a milder phenotype that lacks congenital malformations. WHS is rarely associated with congenital diaphragmatic hernia (CDH), and only 8 cases are reported in the literature. In almost all cases of CDH and WHS a large deletion of the short arm of chromosome 4 is present. A microdeletion of 2.6 Mb on distal 4p associated with CDH and multiple congenital malformations (i.e., cleft palate) is reported for the first time. Such a microdeletion should prompt a molecular study for WHS when in a fetus/newborn with CDH the association with cleft lip/palate and typical facial appearance (flat facial profile, hypertelorism) is found. Copyright 2006 Wiley-Liss, Inc.

Chylothorax associated with a congenital peritoneopericardial diaphragmatic hernia in a dog.

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Schmiedt, Chad Weber; Washabaugh, Kate F; Rao, Deepa B; Stepien, Rebecca L

2009-01-01

A 2-year-old dog was presented with a 3-month history of increasing respiratory effort and rate, inappetence, and lethargy. Chest radiographs demonstrated significant pleural effusion, which was consistent with chyle on biochemical and cytological evaluations. Further diagnostic evaluation, including a thoracic computed tomographic scan, revealed a peritoneopericardial diaphragmatic hernia (PPDH) resulting in a large, fat-attenuating mass within the pericardium. The dog was taken to surgery for repair of the PPDH, pericardectomy, and cisterna chyli ablation. Rapid and permanent resolution of the chylothorax occurred postoperatively. This is the first reported case of chylothorax secondary to PPDH.

Preoperative cardiorespiratory trends in infants with congenital diaphragmatic hernia.

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Moffitt, S T; Schulze, K F; Sahni, R; Wung, J T; Myers, M M; Stolar, C J

1995-04-01

The objective of this study was to determine the cardiovascular and pulmonary adaptations of infants with congenital diaphragmatic hernia (CDH) from birth until delayed surgery through the use of continuous monitoring. Continuous cardiovascular (HR, heart rate variability [HR-SD], BP, blood pressure variability [BP-SD], and oxygen saturation) and ventilatory (minute volume, airway pressure, and effective compliance) measurements were made on-line, using a computerized whole-body plethysmograph-incubator (Vital-trends, VT1000), in nine ventilated infants with CDH. Data collection commenced at birth and continued until surgery. Minute mean values for each variable were recorded. Hourly means were computed from the minute means, averaged across infants each hour over the first 50 hours of life, and regressed against postnatal age. Results showed a significant increase in BP (P newborn infants. Deviation from these idealized patterns may identify an infant who is not responding satisfactorily to the given therapy and who may require alternative treatment modalities.

Rare combination of left-sided congenital diaphragmatic hernia and omphalocele.

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Chee, Yuet Yee; Wong, Siu Chun Mabel; Wong, Ming Sum Rosanna

2017-08-07

We reported a rare case of left-sided posterolateral congenital diaphragmatic hernia (CDH) and omphalocele, which is not associated with chromosomal abnormalities or other syndromes. Omphalocele was detected antenatally (CDH was not detected in antenatal ultrasound). The patient suffered from respiratory failure secondary to severe pulmonary hypertension. As the combination of CDH and omphalocele is rare and with the abdominal content herniating into the omphalocele instead of the thorax, antenatal diagnosis of such condition can be difficult. Unlike other reported cases in the literature, our patient's respiratory condition has been improving with time and is surviving beyond the infancy period. We believe this to be the first such survival case reported in the literature. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Bochdalek Hernia with Adult Diaphragmatic Agenesis

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Erkan Akar

2013-10-01

Full Text Available    Diaphragmatic hernia arises from pleuro-peritoneal membranes inability to close pericardioperitoneal membranes. Diaphragmatic defect may be located in esophageal hiatus (hiatal hernia, nearby the hiatus (paraesophageal, retrosternal (Morgagni or posterolateral (Bochdalek. Congenital diaphragmatic hernias (CDH diagnosed after neonatal period are defined as late presenting CDH. This group of patients consist 5-31% of CDHs and lead to diagnostic difficulties. A case of adult type Bochdalek hernia who was admitted to our clinic with respiratory problems and recognized late with the absence of left diaphragm was discussed in the light of clinical and surgical methods.

Atypical right diaphragmatic hernia (hernia of Morgagni, spigelian hernia and epigastric hernia in a patient with Williams syndrome: a case report

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Rashid Farhan

2009-01-01

Full Text Available Abstract Introduction Williams syndrome is rare genetic disorder resulting in neurodevelopmental problems. Hernias of the foramen of Morgagni are rare diaphragmatic hernias and they mostly present on the right side, in the anterior mediastinum. They are usually asymptomatic and are difficult to diagnose, especially in patients with learning disabilities. Case presentation This 49-year-old woman with Williams syndrome, cognitive impairment and aortic stenosis presented to physicians with right-sided chest pain. She had previously undergone repair of her right spigelian and epigastric hernia. Her abdominal examination was unremarkable. Chest X-ray suggested right-sided diaphragmatic hernia and pleural effusion for which she received treatment. The computed tomography scan showed a diaphragmatic hernia with some collapse/consolidation of the adjacent lung. Furthermore, the patient had aortic stenosis and was high risk for anaesthesia (ASA grade 3. She underwent successful laparoscopic repair of her congenital diaphragmatic hernia leading to a quick and uneventful postoperative recovery. Conclusion These multiple hernias suggest that patients with Williams syndrome may have some connective tissue disorder which makes them prone to develop hernias especially associated with those parts of the body which may have intracavity pressure variations like the abdomen. Diaphragmatic hernia may be the cause of chest pain in these patients. A computed tomography scan helps in early diagnosis, and laparoscopic repair helps in prevention of further complications, and leads to quick recovery especially in patients with learning disabilities. In the presence of significant comorbidities, a less invasive operative procedure with quick recovery becomes advisable.

Predictors of prognosis in neonates with congenital diaphragmatic hernia: experience of 12 years

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Catarina Granjo Morais

2017-01-01

Full Text Available Introduction: Congenital diaphragmatic hernia (CDH is a severe malformation, displaying relevant mortality and morbidity rates in newborns.Aim: To characterize clinically and demographically all neonatal cases of CDH admitted to a level III Neonatal Intensive Care Unit during a 12-year period and to evaluate the predictive value of baseline characteristics on mortality and morbidity at discharge.Methods: Maternal/infant clinical and electronic records were ret-rospectively reviewed. All neonates with posterolateral CDH admitted between January 2003 and December 2014 were included.Results: Fifty-three newborns were included. Overall mortality during hospitalization was 22/53 (41.5%. Clinical characteristics associated with mortality were the presence of intrathoracic liver (p = 0.005, intrathoracic stomach (p = 0.015, elevated arterial pCO2 or lower pH values at admission (respectively, p = 0.001 and p < 0.001, pre-ductal oxygen saturation < 85% at admission (p = 0.012 and surgical repair with prosthetic patch (p = 0.041. Morbidity at discharge was reported in 7 (22.6% survivors. Stomach herniation and sepsis were associated with higher morbidity (respectively, p = 0.012 and p = 0.029. In a logistic regression, patch repair was the only variable with predictive value for death during hospitalization, with an odds ratio (OR of 15 (95% CI 0.98-228.9, and intrathoracic stomach was a predictor of morbidity at discharge (OR = 15.7, 95% CI 1.4-174.2.Conclusion: Structural characteristics, namely defect size and presence of intrathoracic stomach, remain the primary determinants of neonatal prognosis in CDH. Although post-natal approaches have progressively proven their value in increasing survival and improving management of high-risk cases, future researches should continue focusing on the definition of foetal anatomical markers of severity and prenatal treatment of CDH.

Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia.

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Stege, Gerben; Fenton, Alan; Jaffray, Bruce

2003-09-01

Reported survival in congenital diaphragmatic hernia (CDH) fails to allow for case selection bias. This study reports the incidence of CDH in a geographically defined population over 11 years and assesses the effect of new therapies (high-frequency oscillatory ventilation, extracorporeal membrane oxygenation, inhaled nitric oxide, and delayed surgery) on survival when case selection is avoided. A retrospective review of cases from a regional case registry, the Northern Region Congenital Anomaly Survey, was conducted. A total of 185 cases were identified. Mortality was 62% and did not vary significantly during the study period. Mortality was unaffected by the introduction of new therapies. There was a significant inverse correlation between the rate of elective termination and survival of live borns. The presence of an additional anomaly increased mortality to 79%. The mortality of CDH when complete case ascertainment is achieved is unaffected by new therapies. The survival rate is principally determined by the rate of antenatal termination and the incidence of associated anomalies. Reports of improved survival of CDH should be interpreted with caution, as variations in outcome are more likely to be explained by case selection artifact.

Sepsis risk factors in infants with congenital diaphragmatic hernia.

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Levy, Michaël; Le Sache, Nolwenn; Mokhtari, Mostafa; Fagherazzi, Guy; Cuzon, Gaelle; Bueno, Benjamin; Fouquet, Virginie; Benachi, Alexandra; Eleni Dit Trolli, Sergio; Tissieres, Pierre

2017-12-01

Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly and remains among the most challenging ICU-managed disease. Beside severe pulmonary hypertension, lung hypoplasia and major abdominal surgery, infective complications remain major determinants of outcome. However, the specific incidence of sepsis as well as associated risk factors is unknown. This prospective, 4-year observational study took place in the pediatric intensive care and neonatal medicine department of the Paris South University Hospitals (Le Kremlin-Bicêtre, France), CDH national referral center and involved 62 neonates with CDH. During their ICU stay, 28 patients (45%) developed 38 sepsis episodes. Ventilator-associated pneumonia (VAP: 23/38; 31.9 VAP per 1000 days of mechanical ventilation) and central line-associated blood stream infections (CLABSI: 5/38; 5.5 per 1000 line days) were the most frequently encountered infections. Multivariate analysis showed that gestational age at birth and intra-thoracic position of liver were significantly associated with the occurrence of sepsis. Infected patients had longer duration of mechanical and noninvasive ventilation (16.2 and 5.8 days, respectively), longer delay to first feeding (1.2 days) and a longer length of stay in ICU (23 days), but there was no difference in mortality. Healthcare-associated infections, and more specifically VAP, are the main infective threat in children with CDH. Sepsis has a significant impact on the duration of ventilator support and ICU length of stay but does not impact mortality. Low gestational age and intra-thoracic localization of the liver are two independent risk factors associated with sepsis.

Formed Due to Traumatic Diaphragmatic Injury, A Case of Gastric Perforation

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Ceren Sen Tanrikulu

2014-03-01

Full Text Available Diaphragmatic hernia may be congenital or traumatic in origin. Diaphragmatic hernias are more common in adult and occur as a result of penetrating injuries are more common than from blunt injuries. Traumatic diaphragmatic hernia may manifest immediately or without any findings that several months/years after the incident. Early diagnosis is difficult, and it is usually made intraoperatively. Frequently during the early phase of traumatic diaphragmatic hernias, abdominal organ injury can be seen, such as stomach, colon and liver. In this study, we presented a case with acute gastric strangulation and perforation in the diaphragmatic injury occurred as a result of penetrating thoracic trauma.

Mutations in STRA6 cause a broad spectrum of malformations including anophthalmia, congenital heart defects, diaphragmatic hernia, alveolar capillary dysplasia, lung hypoplasia, and mental retardation

NARCIS (Netherlands)

Pasutto, Francesca; Sticht, Heinrich; Hammersen, Gerhard; Gillessen-Kaesbach, Gabriele; Fitzpatrick, David R.; Nuernberg, Gudrun; Brasch, Frank; Schirmer-Zimmermann, Heidemarie; Tolmie, John L.; Chitayat, David; Houge, Gunnar; Fernandez-Martinez, Lorena; Keating, Sarah; Mortier, Geert; Hennekam, Raoul C. M.; von der Wense, Axel; Slavotinek, Anne; Meinecke, Peter; Bitoun, Pierre; Becker, Christian; Nuernberg, Peter; Reis, Andre; Rauch, Anita

2007-01-01

We observed two unrelated consanguineous families with malformation syndromes sharing anophthalmia and distinct eyebrows as common signs, but differing for alveolar capillary dysplasia or complex congenital heart defect in one and diaphragmatic hernia in the other family. Homozygosity mapping

CONGENITAL DIAPHRAGMATIC HERNIA IN A TWO-DAY-OLD NEONATE: ANAESTHETIC MANAGEMENT AND CHALLENGES

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Tridip Jyoti

2016-04-01

Full Text Available INTRODUCTION Congenital diaphragmatic hernia in a neonate is a challenging task to any anaesthesiologist. CDH occurs due to an early developmental defect that results in the extrusion of intra-abdominal organs (i.e. stomach, small intestines, spleen, liver into the thoracic cavity. In 85% of the cases it is left sided. This leads to lung hypoplasia, pulmonary hypertension, and pulmonary arteriolar dysregulation/reactivity. Historically, CDH was considered to be a surgical emergency and aggressive hyperventilatory strategies with high peak inspiratory pressures were employed to improve survival. But recent multicentre studies have shown the beneficial effect of conservative low volume ventilation with low inflation pressures and permissive hypercapnia. We present to you the successful management of a two day old neonate with this ventilation strategy.

Congenital diaphragmatic hernia with concurrent aplasia of the pericardium in a foal.

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Tăbăran, Alexandru-Flaviu; Nagy, Andras Laszlo; Cătoi, Cornel; Morar, Iancu; Tăbăran, Alexandra; Mihaiu, Marian; Bolfa, Pompei

2015-12-30

In veterinary medicine congenital abnormalities of the diaphragm and pericardium are rare, idiopathic malformations, being reported mainly in dogs. This report documents an unusual case of developmental defects in a foal consisting of diaphragmatic hernia concurrent with pericardial aplasia. Following a normal delivery, a full term, female Friesian stillborn foal with the placenta was presented for necropsy. External morphological examination indicated a normally developed foal. At necropsy, a large oval defect (approximately 20 × 15 cm in size) was observed in the left-dorsal side of the diaphragm (left lumbocostal triangle). This defect allowed the intestinal loops, spleen and partially the liver to translocate into the thorax. The loops of the left ascending colon, including the pelvic flexure and partially the small intestine covered the cranial and dorsal posterior parts of the heart due to the complete absence of the left pericardium. The remaining pericardium presented as a white, semi-transparent strip, partially covering the right side of the heart. The left lung and the main bronchus were severely hypoplastic to approximately one-fifth the size of their right homologue. The intermediate part of the liver, containing mainly the enlarged quadrate lobe was translocated in the thorax, severely enlarged and showed marked fibrosis. Histologically in the herniated lobes we diagnosed hepatic chronic passive congestion, telangiectasia and medial hypertrophy of blood vessels. Concomitant malformation involving diaphragmatic hernia and pericardial aplasia in horses have not been previously reported. Moreover, this is the first case describing pericardial aplasia in horse.

Diaphragmatic rupture with right colon and small intestine herniation after blunt trauma: a case report

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Muroni Mirko

2010-08-01

Full Text Available Abstract Introduction Traumatic diaphragmatic hernias are an unusual presentation of trauma, and are observed in about 10% of diaphragmatic injuries. The diagnosis is often missed because of non-specific clinical signs, and the absence of additional intra-abdominal and thoracic injuries. Case presentation We report a case of a 59-year-old Italian man hospitalized for abdominal pain and vomiting. His medical history included a blunt trauma seven years previously. A chest X-ray showed right diaphragm elevation, and computed tomography revealed that the greater omentum, a portion of the colon and the small intestine had been transposed in the hemithorax through a diaphragm rupture. The patient underwent laparotomy, at which time the colon and small intestine were reduced back into the abdomen and the diaphragm was repaired. Conclusions This was a unusual case of traumatic right-sided diaphragmatic hernia. Diaphragmatic ruptures may be revealed many years after the initial trauma. The suspicion of diaphragmatic rupture in a patient with multiple traumas contributes to early diagnosis. Surgical repair remains the only curative treatment for diaphragmatic hernias. Prosthetic patches may be a good solution when the diaphragmatic defect is severe and too large for primary closure, whereas primary repair remains the gold standard for the closure of small to moderate sized diaphragmatic defects.

Piston-pump-type high frequency oscillatory ventilation for neonates with congenital diaphragmatic hernia: a new protocol.

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Tamura, M; Tsuchida, Y; Kawano, T; Honna, T; Ishibashi, R; Iwanaka, T; Morita, Y; Hashimoto, H; Tada, H; Miyasaka, K

1988-05-01

High frequency ventilation and extracorporeal membrane oxygenation (ECMO) are devices that are expected to save the lives of newborn infants whose pulmonary conditions have deteriorated. A piston-pump-type high-frequency oscillator (HFO), developed by Bryan and Miyasaka called "Hummingbird," is considered to be superior to high frequency "jet" ventilators or those of the flow-interrupter type, and was used successfully in two neonates with congenital diaphragmatic hernia (CDH) in a high-risk group. The first baby was on a conventional ventilator with pharmacologic support for the first 54 hours and then operated on. Postoperative deterioration necessitated the use of HFO for the next eight days. The infant then recovered uneventfully. For the second baby, HFO was necessary both preoperatively and postoperatively. This baby had a major diaphragmatic defect and her case was complicated with pneumothorax. There was a long stormy course on HFO (total, 70 days), but the patient was successfully extubated on the 75th day postoperatively and is now doing well. We believe active long preoperative stabilization with pharmacologic support and preoperative and postoperative hyperventilation with a piston-pump-type HFO may be a new innovative strategy for the management of severe CDH patients.

Congenital diaphragmatic hernia may be associated with 17q12 microdeletion syndrome.

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Goumy, Carole; Laffargue, Fanny; Eymard-Pierre, Eléonore; Kemeny, Stéphen; Gay-Bellile, Mathilde; Gouas, Laetiti; Gallot, Denis; Francannet, Christine; Tchirkov, Andrei; Pebrel-Richard, Céline; Vago, Philippe

2015-01-01

Microdeletions of 17q12 encompassing TCF2 are associated with maturity-onset of diabetes of the young type 5, cystic renal disease, pancreatic atrophy, Mullerian aplasia in females and variable cognitive impairment. We report on a patient with a de novo 17q12 microdeletion, 1.8 Mb in size, associated with congenital diaphragmatic hernia (CDH). The 5-year-old male patient presented multicystic renal dysplasia kidneys, minor facial dysmorphic features and skeletal anomalies, but neither developmental delay nor behavioral abnormalities. CDH has been previously associated with the 17q12 microdeletion syndrome only in one prenatal case. The present study reinforces the hypothesis that CDH is part of the phenotype for 17q12 microdeletion and that 17q12 encompasses candidate(s) gene(s) involved in diaphragm development. We suggest that PIGW, a gene involved in an early step of GPI biosynthesis, could be a strong candidate gene for CDH. © 2014 Wiley Periodicals, Inc.

Delayed Presentation of Traumatic Diaphragmatic Hernia: The Evaluation of Surgical Treatment Results

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Ali Sadrizadeh

2015-06-01

Full Text Available Introduction: Diaphragmatic hernia could be caused by congenital disorders, blunt trauma or penetrating injuries. The diagnosis of traumatic diaphragmatic hernia is normally neglected during the first presentation leading to late complications and considerably increased mortality and morbidity among the patients. Materials and Methods: In this retrospective, descriptive study, we reviewed the medical records of patients presented with traumatic diaphragmatic hernia who had undergone surgical operations between 1982-2015 in Ghaem Hospital and Omid Hospital affiliated to Mashhad University of Medical Sciences, Iran. The studied variables included age, gender, clinical symptoms, location of hernia, involved organs, type of imaging modalities, surgical techniques, length of hospital stay, mortality rate and surgical complications. Results: In this study, 38 patients were diagnosed with traumatic diaphragmatic hernia consisting of 28 men and 10 women. In total, 79% and 21% of the patients suffered from penetrating trauma and blunt trauma, respectively. In addition, left-sided, right-sided and bilateral hernias were present in 33%, 4% and 1% of the patients, respectively. The most frequently herniated organ was the stomach, and the most common clinical symptoms were abdominal pain (84% and dyspnea (53%. Initially, chest radiographs were performed on all the patients, and thoracotomy was performed to repair diaphragmatic tears in all the cases (100%. In this study, 3 patients had previously undergone Hartmann’s operation for gangrenous herniated colon, and devolvulation of gastric volvulus had also been performed on 3 patients. The main post-operative complications were reported to be pneumonia and respiratory insufficiency (2 cases, and the mean length of hospital stay was 6 days (5-8 days which was longer (1-2 months in patients with gangrenous bowel (3 patients. Furthermore, no mortality was reported during the course of hospitalization in these

Elevation of the diaphragmatic cupola

International Nuclear Information System (INIS)

Semenov, V.M.; Talesnik, M.R.

1988-01-01

Altogether 45 patients with elevation of the diaphragmatic cupola were examined. A high frequency of erroneous initial interpretation of examination results was noted in inflammatory and tumorous lesions and congenital conditions. Routine and contrast methods (pneumoperitoneum, bronchography, pleurography and fistulography) were used. Disease-related methods of X-ray investigation were proposed. A variety of causes of diaphragm elevation was indicated

Challenges in the management of early versus late presenting congenital diaphragmatic hernia in a poor resource setting

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Abubakar Auwal

2011-01-01

Full Text Available Background: Despite the advances in management, congenital diaphragmatic hernia (CDH has continued to pose a significant challenge to paediatric surgeons. This is amplified in a setting like ours where there is a dearth of facilities to cope with the problem of CDH. This study was undertaken to highlight the peculiarities of the management of CDH in a poor resource setting. Methods: All confirmed cases of CDH were prospectively documented from 2003 till date. Results: Seven children were treated from 2003 till date. The diaphragmatic defect was on the left side in six (83.8% and on the right side in one (17.7%. All the patients had primary closure of the defect without patch via an abdominal approach. The three patients presenting at birth died while the remaining four patients survived. Conclusion: With inadequate neonatal intensive care facilities, the severe early presenting CDH has a dismal prognosis. In contrast, the late presenting CDH poses more diagnostic challenges; but once identified and appropriate treatment instituted, it has an excellent prognosis. We recommend that physicians should include CDH in the differential diagnosis of patients with birth asphyxia and in patients with chronic respiratory symptoms with failure to thrive.

Vasopressin in perioperative management of congenital ...

African Journals Online (AJOL)

Perioperative care of infants with diaphragmatic hernias can be a challenge because of pulmonary hypertension and systemic hypotension. The objective of this study was to report the usefulness of vasopressin infusion in improving pulmonary and systemic haemodynamics in an infant with congenital diaphragmatic hernia.

Modified transanal repair of congenital H-type rectovestibular fistula ...

African Journals Online (AJOL)

Congenital H-type rectovestibular fistulas are rare in the spectrum of anorectal malformations. Repair is associated with recurrence rates of up to 30%, using perineal repair, vestibuloanal pull-through or anterior anorectoplasty. The rarity of the malformation has limited experience with the surgical approach; hence, the rate ...

Congenital diaphragmatic hernia: a modern day approach.

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Waag, Karl-Ludwig; Loff, Steffan; Zahn, Katrin; Ali, Mansour; Hien, Steffen; Kratz, Markus; Neff, Wolfgang; Schaffelder, Regine; Schaible, Thomas

2008-11-01

Centralization of all complicated congenital diaphragmatic hernias (CDH) was organized in Germany from 1998, collecting 325 consecutive patients with striking increasing survival rates. This series report 244 patients from 2002 to 2007. Today, large defects are detected early in pregnancy by ultrasound and magnetic resonance imaging (MRI). In extracorporeal membrane oxygenation (ECMO) patients, prenatal lung head ratio (LHR) was 1.2 (median) at the 34th week of gestation or less than 25 ml lung tissue in MRI. This means that all patients below LHR of 1.4 should be transferred prenatally in a tertiary center. High risk group for survival was defined as LHR below 0.9, ie, 10 ml in MRI planimetry. Inborn patients show better results than outborns. In algorithm therapy, gentle ventilation plays an important role in preventing damage to the lung tissue and avoiding long term ventilation. When PaCO(2) was more than 75 mmHg, ventilation was changed to high frequency oscillatory ventilation (HFOV). Indication for ECMO was seen in preductal PaO(2) less than 50 mmHg over 2-4 h or less than 40 mmHg over 2 h. ECMO related risks included intracerebral bleeding (9%), intrapulmonary bleeding (14%), and convulsions (16%). Surgically, a longitudinal midline incision for exposure of the defect, the duodenal kinking, and probably for abdominal patching was perfect. A cone formed goretex patch provided more abdominal space and reduced abundant intrathoracical cavity. No drain was used. Postoperative complications were described. Overall survival in 244 consecutive patients was 86.5% for all patients born alive. All those who needed ECMO survived in 71%, underlining ECMO as a treatment of last choice. Follow-up for quality of life after CDH is described.

Traumatic diaphragmatic ruptures: clinical presentation, diagnosis and surgical approach in adults

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Hofmann, Sabine

2012-01-01

Full Text Available Objective: Traumatic diaphragmatic injuries are rare, but potentially life-threatening due to herniation of abdominal organs into the pleural cavities. They can be easily overlooked on initial diagnostics and a high index of suspicion is required. The aim of this retrospective study was to analyze the clinical presentation, diagnostic methods and surgical management of patients with diaphragmatic rupture at our institution. Methods: A retrospective study was performed to analyze our experience with patients suffering from traumatic diaphragmatic rupture. Charts were reviewed for sex, age, side-location, concomitant injuries, time-to-diagnosis, diagnostic methods, surgical approach and outcome. Results: Fourteen patients (median age: 46 yrs, range 18–71, 9 male, 5 female with diaphragmatic injuries (left side: 10, right side: 4 were treated between July 2003 and September 2011. Mechanism of injury was a penetrating trauma (14%, blunt trauma (50% and others (36%. Associated abdominal injuries included spleen rupture (n=3, liver laceration (n=2, abdominal wall laceration (n=2 and gastric perforation (n=1. Computed tomography was the most sensitive diagnostic method. All patients underwent trans-abdominal repair of the diaphragmatic defect (direct suture: 10, prosthetic mesh insertion: 4. Associated abdominal procedures included splenectomy (n=3, liver packing (n=2, abdominal wall reconstruction (n=2 and partial gastric resection (n=1. Morbidity and hospital mortality rate were 36% and 0%, respectively. Median postoperative hospital stay was 17 days (range: 7–40 days. Conclusion: Morbidity and mortality of diaphragmatic ruptures are mainly determined by associated injuries or complications of diaphragmatic herniation like incarceration of viscera or lung failure. Early diagnosis helps to prevent severe complications. Spiral CT-scan is the most reliable tool for acute diagnosis of diaphragmatic rupture and associated visceral lacerations

Radiographic findings in late-presenting congenital diaphragmatic hernia: helpful imaging findings

International Nuclear Information System (INIS)

Muzzafar, Sofia; Swischuk, Leonard E.; Jadhav, Siddharth P.

2012-01-01

Imaging findings in delayed presentation of congenital diaphragmatic hernia can be confusing and misleading, resulting in a delay in diagnosis. To evaluate the often puzzling plain film findings of late-presenting CDH in an effort to determine whether any of the findings could be helpful in arriving at an early diagnosis. We reviewed and documented the plain film findings and clinical data in eight patients seen during the last 20 years with late-presenting CDH. IRB exempt status was obtained in this study. There were five boys and three girls. The age range was 4 months to 12 years with a mean of 2.4 years. Five children presented with acute respiratory problems while three presented with acute abdominal pain. Two children presented with both respiratory and abdominal findings and one also presented with hematemesis. Two children had radiographic findings that were not difficult to analyze while the remaining six had findings that posed initial diagnostic problems. Although not common, late-presenting CDH can result in confusing plain film radiographic findings and a delay in diagnosis. We found that the most important finding in analyzing these radiographs is in evaluating the location and position of the gastric bubble with the more common left-side hernias. (orig.)

Radiographic findings in late-presenting congenital diaphragmatic hernia: helpful imaging findings

Energy Technology Data Exchange (ETDEWEB)

Muzzafar, Sofia; Swischuk, Leonard E.; Jadhav, Siddharth P. [University of Texas Medical Branch, Department of Pediatric Radiology, Galveston, TX (United States)

2012-03-15

Imaging findings in delayed presentation of congenital diaphragmatic hernia can be confusing and misleading, resulting in a delay in diagnosis. To evaluate the often puzzling plain film findings of late-presenting CDH in an effort to determine whether any of the findings could be helpful in arriving at an early diagnosis. We reviewed and documented the plain film findings and clinical data in eight patients seen during the last 20 years with late-presenting CDH. IRB exempt status was obtained in this study. There were five boys and three girls. The age range was 4 months to 12 years with a mean of 2.4 years. Five children presented with acute respiratory problems while three presented with acute abdominal pain. Two children presented with both respiratory and abdominal findings and one also presented with hematemesis. Two children had radiographic findings that were not difficult to analyze while the remaining six had findings that posed initial diagnostic problems. Although not common, late-presenting CDH can result in confusing plain film radiographic findings and a delay in diagnosis. We found that the most important finding in analyzing these radiographs is in evaluating the location and position of the gastric bubble with the more common left-side hernias. (orig.)

Radiographic findings in late-presenting congenital diaphragmatic hernia: helpful imaging findings.

Science.gov (United States)

Muzzafar, Sofia; Swischuk, Leonard E; Jadhav, Siddharth P

2012-03-01

Imaging findings in delayed presentation of congenital diaphragmatic hernia can be confusing and misleading, resulting in a delay in diagnosis. To evaluate the often puzzling plain film findings of late-presenting CDH in an effort to determine whether any of the findings could be helpful in arriving at an early diagnosis. We reviewed and documented the plain film findings and clinical data in eight patients seen during the last 20 years with late-presenting CDH. IRB exempt status was obtained in this study. There were five boys and three girls. The age range was 4 months to 12 years with a mean of 2.4 years. Five children presented with acute respiratory problems while three presented with acute abdominal pain. Two children presented with both respiratory and abdominal findings and one also presented with hematemesis. Two children had radiographic findings that were not difficult to analyze while the remaining six had findings that posed initial diagnostic problems. Although not common, late-presenting CDH can result in confusing plain film radiographic findings and a delay in diagnosis. We found that the most important finding in analyzing these radiographs is in evaluating the location and position of the gastric bubble with the more common left-side hernias.

Making meaning of pumping for mothers of infants with congenital diaphragmatic hernia.

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Froh, Elizabeth B; Deatrick, Janet A; Curley, Martha A Q; Spatz, Diane L

2015-01-01

To describe the process of initiation and maintenance of milk supply and potential transition to direct breastfeeding among mother/infant dyads with infants with congenital diaphragmatic hernia (CDH). A Level-III neonatal intensive care unit. Eleven mother/infant dyads with infants with CDH. Prospective, longitudinal qualitative descriptive design. Semistructured interviews were conducted over the course of the NICU stay. Conventional content analysis was used. Human milk oral care emerged from the interview data as a strong facilitating factor to encouraging mothers to continue pumping during hospitalization. Four main themes emerged regarding the importance and value of human milk oral care for the mothers in relation to pumping and maintenance of milk supply: (a) It motivates me; (b) I'm a part of my baby getting better; (c) We do it together, and (d) We're getting somewhere. The findings of this study reflect the importance and value of human milk oral care as a driving factor to motivate mothers to maintain milk supply during the critical time when the infant with CDH is not able to take in enteral nutrition and throughout the hospital stay. © 2015 AWHONN, the Association of Women's Health, Obstetric and Neonatal Nurses.

Occult diaphragmatic injuries caused by stab wounds.

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Leppäniemi, Ari; Haapiainen, Reijo

2003-10-01

Missed diaphragmatic perforation caused by penetrating trauma can lead to subsequent strangulation of a hollow viscus, which has prompted the use of invasive diagnostic procedures to exclude occult diaphragmatic injuries in asymptomatic, high-risk patients. The objective of this study was to determine the incidence of occult diaphragmatic injuries caused by stab wounds of the lower chest and upper abdomen, and to examine the natural history and consequences of missed diaphragmatic injuries. On the basis of patient data from two previous randomized studies from our institution, a retrospective analysis was performed on 97 patients treated for anterior stab wounds located between the nipple line, the umbilical level, and the posterior axillary lines not having indications for immediate surgical exploration. The patients were divided into two groups on the basis of their initial randomized management (open or laparoscopic exploration vs. expectant observation). In the exploration group (n = 47), four diaphragmatic injuries (9%) were detected (three left-sided and one right-sided). Excluding patients with associated injuries requiring surgical repair, the incidence of occult diaphragmatic injuries was 3 of 43 (7%). In the observation group (n = 50), there were two patients (4%) with delayed presentation of missed left-sided diaphragmatic injury 2 and 23 months later, respectively. Both injuries resulted from stab wounds of the left flank and presented with herniation of the stomach or small bowel and colon. The overall incidence of occult diaphragmatic injuries in left-sided thoracoabdominal stab wounds was 4 of 24 (17%), and was much lower after stab wounds of left epigastrium (0%), right lower chest (0%), and right epigastrium (4%). In asymptomatic patients with anterior or flank stab wounds of the lower chest or upper abdominal area, the risk of an occult diaphragmatic injury is approximately 7% which, if undetected, is associated with a high risk of subsequent

Spontaneous prematurity in fetuses with congenital diaphragmatic hernia: a retrospective cohort study about prenatal predictive factors.

Science.gov (United States)

Barbosa, Bruna Maria Lopes; Rodrigues, Agatha S; Carvalho, Mario Henrique Burlacchini; Bittar, Roberto Eduardo; Francisco, Rossana Pulcineli Vieira; Bernardes, Lisandra Stein

2018-01-12

To evaluate possible predictive factors of spontaneous prematurity in fetuses with congenital diaphragmatic hernia (CDH). A retrospective cohort study was performed. Inclusion criteria were presence of CDH; absence of fetoscopy; absence of karyotype abnormality; maximum of one major malformation associated with diaphragmatic hernia; ultrasound monitoring at the Obstetrics Clinic of Clinicas Hospital at the University of São Paulo School of Medicine, from January 2001 to October 2014. The data were obtained through the electronic records and ultrasound system of our fetal medicine service. The following variables were analyzed: maternal age, primiparity, associated maternal diseases, smoking, previous spontaneous preterm birth, fetal malformation associated with hernia, polyhydramnios, fetal growth restriction, presence of intrathoracic liver, invasive procedures performed, side of hernia and observed-to- expected lung to head ratio (o/e LHR). On individual analysis, variables were assessed using the Chi-square test and the Mann-Whitney test. A multiple logistic regression model was applied to select variables independently influencing the prediction of preterm delivery. A ROC curve was constructed with the significant variable, identifying the values with best sensitivity and specificity to be suggested for use in clinical practice. Eighty fetuses were evaluated, of which, 21 (26.25%) were premature. O/e LHR was the only factor associated with prematurity (p = 0.020). The ROC curve showed 93% sensitivity with 48.4% specificity for the cutoff of 40%. O/e LHR was the only predictor of prematurity in this sample.

Are all pulmonary hypoplasias the same? A comparison of pulmonary outcomes in neonates with congenital diaphragmatic hernia, omphalocele and congenital lung malformation.

Science.gov (United States)

Akinkuotu, Adesola C; Sheikh, Fariha; Cass, Darrell L; Zamora, Irving J; Lee, Timothy C; Cassady, Christopher I; Mehollin-Ray, Amy R; Williams, Jennifer L; Ruano, Rodrigo; Welty, Stephen E; Olutoye, Oluyinka O

2015-01-01

Patients with congenital diaphragmatic hernias (CDH), omphaloceles, and congenital lung malformations (CLM) may have pulmonary hypoplasia and experience respiratory insufficiency. We hypothesize that given equivalent lung volumes, the degree of respiratory insufficiency will be comparable regardless of the etiology. Records of all fetuses with CDH, omphalocele, and CLM between January 2000 and June 2013 were reviewed. MRI-based observed-to-expected total fetal lung volumes (O/E-TFLV) were calculated. An analysis of outcomes in patients with O/E-TFLV between 40% and 60%, the most inclusive range, was performed. 285 patients were evaluated (161, CDH; 24, omphalocele; 100, CLM). Fetuses with CDH had the smallest mean O/E-TFLV. CDH patients were intubated for longer and had a higher incidence of pulmonary hypertension. Fifty-six patients with the three diagnoses had an O/E-TFLV of 40%-60%. The need for ECMO, supplemental oxygen at 30days of life, and 6-month mortality were similar among groups. CDH patients had a significantly longer duration of intubation and higher incidence of pulmonary hypertension than the other two diagnoses. Given equivalent lung volumes (40%-60% of expected), CDH patients require more pulmonary support initially than omphalocele and CLM patients. In addition to lung volumes, disease-specific factors, such as pulmonary hypertension in CDH, also contribute to pulmonary morbidity and overall outcome. Copyright © 2015 Elsevier Inc. All rights reserved.

Junctional ectopic tachycardia following repair of congenital heart ...

African Journals Online (AJOL)

Background: Postoperative junctional ectopic tachycardia (JET) is a rare and transient phenomenon occurring after repair of congenital heart defects. Report on this arrhythmia in the subregion is rare. We set out to determine the incidence of this arrhythmia and review the treatment and outcomes of treatment in our centre.

Congenital pleuroperitoneal hernia presenting as gastrothorax in five cavalier King Charles spaniel dogs.

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Rossanese, M; Pivetta, M; Pereira, N; Burrow, R

2018-04-30

Five cavalier King Charles spaniels were examined for acute onset of respiratory distress. Thoracic radiographs demonstrated diaphragmatic hernia and tension gastrothorax, visible as a distended stomach occupying the left caudal thoracic cavity. Exploratory midline coeliotomy confirmed congenital pleuroperitoneal diaphragmatic hernia with herniation and dilatation of the stomach. The hernia configuration was consistent in all cases, with a defect affecting the left diaphragmatic crus. Congenital pleuroperitoneal diaphragmatic hernia is a rare condition caused by a defect in the dorsolateral diaphragm. Defects of the left crus of the diaphragm could result in the herniation of the stomach into the thoracic cavity with possible subsequent tension gastrothorax. Cavalier King Charles spaniels may have a predisposition to this condition. Tension gastrothorax is an acute life-threatening consequence of gastric herniation through a diaphragmatic defect that must be promptly recognised and surgically treated. © 2018 British Small Animal Veterinary Association.

Acute Intrathoracic Gastric Volvulus due to Diaphragmatic Hernia: A Rare Emergency Easily Overlooked

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Hyung Hun Kim

2011-05-01

Full Text Available Acute intrathoracic gastric volvulus occurs when the stomach undergoes organoaxial torsion in the chest due to either concomitant enlargement of the hiatus or a diaphragmatic hernia. Iatrogenic diaphragmatic hernia can occur after hiatal hernia repair and other surgical procedures, such as nephrectomy, esophagogastrectomy and splenopancreatectomy. We describe a 49-year-old woman who presented to our emergency department with acute moderate epigastric soreness and vomiting. She had undergone extensive gynecologic surgery including splenectomy 1 year before. The chest radiograph obtained in the emergency department demonstrated an elevated gastric air-fluid level in the left lower lung field. An urgent gastroscopy showed twisted structural abnormality of the stomach body. A computed tomography scan demonstrated the distended stomach, located in the left lower hemithorax through a left diaphragmatic defect. Emergent transthoracic repair was performed. Postoperative recovery was uneventful, and the patient did not experience any pain or difficulty with eating.

Extracorporeal Membrane Oxygenation Cannula Malposition in the Azygos Vein in a Neonate with Right-Sided Congenital Diaphragmatic Hernia

Directory of Open Access Journals (Sweden)

Seung Jun Choi

2016-05-01

Full Text Available Malposition of the extracorporeal membrane oxygenation (ECMO venous cannula in the azygos vein is not frequently reported. We hereby present such a case, which occurred in a neonate with right-sided congenital diaphragmatic hernia. Despite ECMO application, neither adequate flow nor sufficient oxygenation was achieved. On the cross-table lateral chest radiograph, the cannula tip was identified posterior to the heart silhouette, which implied malposition of the cannula in the azygos vein. After repositioning the cannula, the target flow and oxygenation were successfully achieved. When sufficient venous flow is not achieved, as in our case, clinicians should be alerted so they can identify the cannula tip location on lateral chest radiograph and confirm whether malposition in the azygos vein is the cause of the ineffective ECMO.

Management of tricuspid regurgitation in congenital heart disease: is survival better with valve repair?

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Said, Sameh M; Dearani, Joseph A; Burkhart, Harold M; Connolly, Heidi M; Eidem, Ben; Stensrud, Paul E; Schaff, Hartzell V

2014-01-01

Tricuspid valve (TV) regurgitation in congenital heart disease includes a heterogeneous group of lesions, and few series have documented the outcomes. We reviewed the records of 553 patients with congenital heart disease who had undergone TV surgery for tricuspid regurgitation from January 1993 to December 2010. Patients with Ebstein malformation were excluded. Their mean age was 32 ± 21 years, and 300 were female (54%). The most common diagnoses were conotruncal anomaly in 216 patients (39%), previous ventricular septal defect closure in 83 (15%), atrioventricular septal defect in 77 (14%), and pulmonary atresia with an intact ventricular septum in 11 (2%). Preoperative right-sided heart failure was present in 124 patients (22%), and 55 patients (10%) had pulmonary hypertension. TV repair was performed in 442 (80%) and TV replacement in 111 (20%) patients. Repeat sternotomy was performed in 415 patients (75%). Previous TV repair was present in 44 patients (8%); of these, 17 (38.6%) underwent repeat TV repair. The overall early mortality was 3.1% (17 patients) and was 2.5% for TV repair and 5.4% for TV replacement (P = .001). The mean follow-up period was 4.5 ± 4.1 years (maximum, 18). The overall survival at 1, 5, and 10 years was 97%, 93%, and 85%, respectively. Survival was better for patients with repair than with replacement. TV repair was an independent predictor of better survival (P = .001). Important tricuspid regurgitation can occur with a variety of congenital diagnoses. Early mortality is low and late survival is superior with tricuspid repair than with valve replacement. Surgical treatment of tricuspid regurgitation in congenital heart disease should be performed before the onset of heart failure. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

Chronic diaphragmatic hernia in 34 dogs and 16 cats.

Science.gov (United States)

Minihan, Anne C; Berg, John; Evans, Krista L

2004-01-01

Medical records of 34 dogs and 16 cats undergoing surgical repair of diaphragmatic hernia of >2 weeks' duration were reviewed, and long-term follow-up information was obtained. The most common clinical signs were dyspnea and vomiting; however, many of the animals were presented for nonspecific signs such as anorexia, lethargy, and weight loss. Thoracic radiographs revealed evidence of diaphragmatic hernia in only 66% of the animals, and additional imaging tests were often needed to confirm the diagnosis. Thirty-six hernias were repaired through a midline laparotomy; 14 required a median sternotomy combined with a laparotomy. In 14 animals, division of mature adhesions of the lungs or diaphragm to the herniated organs was necessary to permit reduction of the hernia. Fourteen animals required resection of portions of the lungs, liver, or intestine. All hernias were sutured primarily without the use of tissue flaps or mesh implants. Twenty-one of the animals developed transient complications in the postoperative period; the most common of these was pneumothorax. The mortality rate was 14%. Thirty-four (79%) of the animals that were discharged from the hospital had complete resolution of clinical signs, and none developed evidence of recurrent diaphragmatic hernia during the follow-up period. Nine were lost to follow-up.

Congenital diaphramatic hernia

Energy Technology Data Exchange (ETDEWEB)

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

2012-01-15

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

Congenital diaphramatic hernia

International Nuclear Information System (INIS)

Kline-Fath, Beth M.

2012-01-01

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

Congenital retrosternal hernias of Morgagni: Manifestation and treatment in children

Directory of Open Access Journals (Sweden)

Oleksii Slepov

2016-01-01

Full Text Available Background: Due to scarcity of congenital diaphragmatic hearnias of Morgagni (CDHM, non-specific clinical presentation in the pediatric age group, we aimed to investigate the incidence, clinical manifestations, anatomical characteristics, and develop diagnostic algorithm and treatment of CDHM in children. Materials and Methods: The patients′ records of children with CDHM treated in our hospital during past 20 years were retrospectively reviewed for the age at diagnosis, gender, clinical findings, anatomical features, operative details and outcome. Results: Since 1995 to 2014 we observed 6 (3 boys, 3 girls patients with CDHM, that comprise 3.2% of all congenital diaphragmatic hernia cases (n = 185. Age at diagnosis varied from 3 mo. to 10y.o. Failure to thrive was main symptom in 4 patients, followed by recurrent respiratory infections (n = 3, dyspnea (n = 3, and gastrointestinal manifestations: constipation (n = 2, abdominal pain (n = 1. Work-up consisted of plain X-ray for all (n = 6, upper GI (n = 3, barium enema (n = 2, sonography (n = 6 and CT (n = 2. Abdominal approach used in 5 patients, and thoracotomy in one. Herniated contents were: liver lobes (n = 4, transverse colon (n = 3 and greater omentum (n = 1. 5 had right-sided lesion, 1- left-sided. Defect repaired using local tissues. Post-operative course was uneventful; all patients appeared well during follow-up. Conclusion: CDHM is very uncommon anomaly, very occasionally diagnosed at the early age. Failure to thrive and recurrent respiratory infections are most frequent clinical manifestations. In suspected CDHM we advocate the following work-up: plain chest and abdominal X-ray, contrast study (upper GI series or barium enema, ultrasonographic screen and CT scan. Surgical repair via abdominal approach, using local tissues and hernia sac removal is preferred.

Comparison between late-presenting and isolated neonatal congenital diaphragmatic hernias

Directory of Open Access Journals (Sweden)

Christos Plataras

2011-01-01

Full Text Available Purpose: Late-presenting posterolateral congenital diaphragmatic hernias (CDH are anatomically similar to isolated neonatal CDH but are diagnosed and treated after the first month of life. We aim to characterise the clinical manifestations and short-term postoperative course of this entity and compare it with isolated CDH of the neonatal period. Materials and Methods: In the 30-year period from 1980 to 2010, 116 children with CDH were treated at the Aghia Sophia Children′s Hospital, Athens, Greece. Twenty-three (19% of these children were late-presenting cases, being diagnosed between the ages of 1 month and 4 years. Ninety-three were neonatal cases, of whom 22 (24% were excluded due to severe associated anomalies, leaving 71 cases of isolated neonatal CDH. We compared these two groups of patients with regard to preoperative symptoms, postoperative hospital stay, time to complete feeding, overall complication rate, and reoperation rate. Results: Isolated neonatal cases presented more often with acute respiratory symptoms (n=25; P= 0.016 and failure to thrive (n= 38; P= 0.03. Late-presenting cases presented more often with chronic respiratory symptoms (n=14;P= 0.0044 or gastrointestinal symptoms (n=12; P= 0.006. Thirty-five cases with minor or serious complications were reported in the neonatal group, whereas only five complications were observed in the late-presenting group (P= 0.028. We did not record any recurrences or reoperations in the late-presenting group, but we had two recurrences and three reoperations in the neonatal group. Time to full feeds and postoperative hospital stay was shorter in the late-presenting group. Conclusions: Our data demonstrate differences between the two groups in preoperative symptoms and short-term postoperative complications and short-term outcome. Late-presenting cases of CDH had a greater number of chronic symptoms preoperatively, more favorable postoperative outcomes, and less recurrences and reoperations.

A Case of Fatal Pulmonary Hypoplasia with Congenital Diaphragmatic Hernia, Thoracic Myelomeningocele, and Thoracic Dysplasia.

Science.gov (United States)

Ito, Ai; Fujinaga, Hideshi; Matsui, Sachiko; Tago, Kumiko; Iwasaki, Yuka; Fujino, Shuhei; Nagasawa, Junko; Amari, Shoichiro; Kaneshige, Masao; Wada, Yuka; Takahashi, Shigehiro; Tsukamoto, Keiko; Miyazaki, Osamu; Yoshioka, Takako; Ishiguro, Akira; Ito, Yushi

2017-10-01

Background  Congenital diaphragmatic hernia (CDH) is fatal in severe cases of pulmonary hypoplasia. We experienced a fatal case of pulmonary hypoplasia due to CDH, thoracic myelomeningocele (MMC), and thoracic dysplasia. This constellation of anomalies has not been previously reported. Case Report  A male infant with a prenatal diagnosis of thoracic MMC with severe hydrocephalus and scoliosis was born at 36 weeks of gestation. CDH was found after birth and the patient died of respiratory failure due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn at 30 hours of age despite neonatal intensive care. An autopsy revealed a left CDH without herniation of the liver or stomach into the thoracic cavity, severe hydrocephalus, Chiari malformation type II, MMC with spina bifida from Th4 to Th12, hemivertebrae, fused ribs, deformities of the thoracic cage and legs, short trunk, and agenesis of the left kidney. Conclusion  We speculate that two factors may be associated with the severe pulmonary hypoplasia: decreased thoracic space due to the herniation of visceral organs caused by CDH and thoracic dysplasia due to skeletal deformity and severe scoliosis.

Tracheomegaly: a complication of fetal endoscopic tracheal occlusion in the treatment of congenital diaphragmatic hernia

Energy Technology Data Exchange (ETDEWEB)

McHugh, Kieran; Afaq, Asim; Roebuck, Derek J. [Great Ormond Street Hospital for Children, Radiology Department, London (United Kingdom); Broderick, Nigel [Nottingham University Hospitals, Radiology Department, Nottingham (United Kingdom); Gabra, Hany O.; Elliott, Martin J. [Great Ormond Street Hospital for Children, Department of Cardiothoracic Surgery, London (United Kingdom)

2010-05-15

Fetal endoscopic tracheal occlusion (FETO) is a promising treatment for severe congenital diaphragmatic hernia, a condition that carries significant morbidity and mortality. It is hypothesised that balloon occlusion of the fetal trachea leads to an improvement in lung growth and development. The major documented complications of FETO to date are related to preterm delivery. To report a series of five infants who developed tracheomegaly following FETO. Review of all children referred with tracheomegaly to the paediatric intensive care and tracheal service at two referral centres. Five neonates presented with features of respiratory distress shortly after birth and were subsequently found to have marked tracheomegaly. Two neonates had tracheomalacia in addition. There are no previous reports in the literature describing tracheomalacia, or more specifically, tracheomegaly, as a consequence of FETO. We propose that the particularly compliant fetal airway is at risk of mechanical damage from in utero balloon occlusion. This observation of a new problem in this cohort suggests a thorough evaluation of the trachea should be performed in children who have had FETO in utero. It may be that balloon occlusion of the trachea earlier in utero (before 26 weeks' gestation) predisposes to this condition. (orig.)

Abnormal platelet-derived growth factor signaling accounting for lung hypoplasia in experimental congenital diaphragmatic hernia.

Science.gov (United States)

Dingemann, Jens; Doi, Takashi; Ruttenstock, Elke; Puri, Prem

2010-10-01

The pathogenesis of pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) is not fully understood. Platelet-derived growth factor A (PDGFA) and platelet-derived growth factor receptor α (PDGFRα) play a crucial role in lung development. It has been reported that PDGF induces H(2)O(2)-production and that oxidative stress may be an important mechanism for the impaired lung development in the nitrofen rat model. We hypothesized that pulmonary expression of PDGFA and PDGFRα is altered in the nitrofen induced CDH model. Pregnant rats received 100 mg nitrofen or vehicle on gestational day 9 (D9) and were sacrificed on D15, D18 or D21. RNA was extracted from fetal left lungs and mRNA levels of PDGFA and PDGFRα were determined using real-time polymerase chain reaction. Immunohistochemistry for protein expression of PDGFA and PDGFRα was performed. Pulmonary H(2)O(2) was measured colorimetrically. mRNA levels of PDGFRα at D15 (4.50 ± 0.87) and PDGFA at D18 (2.90 ± 1.38) were increased in the nitrofen group (P stress during lung development. Copyright © 2010 Elsevier Inc. All rights reserved.

Tracheomegaly: a complication of fetal endoscopic tracheal occlusion in the treatment of congenital diaphragmatic hernia

International Nuclear Information System (INIS)

McHugh, Kieran; Afaq, Asim; Roebuck, Derek J.; Broderick, Nigel; Gabra, Hany O.; Elliott, Martin J.

2010-01-01

Fetal endoscopic tracheal occlusion (FETO) is a promising treatment for severe congenital diaphragmatic hernia, a condition that carries significant morbidity and mortality. It is hypothesised that balloon occlusion of the fetal trachea leads to an improvement in lung growth and development. The major documented complications of FETO to date are related to preterm delivery. To report a series of five infants who developed tracheomegaly following FETO. Review of all children referred with tracheomegaly to the paediatric intensive care and tracheal service at two referral centres. Five neonates presented with features of respiratory distress shortly after birth and were subsequently found to have marked tracheomegaly. Two neonates had tracheomalacia in addition. There are no previous reports in the literature describing tracheomalacia, or more specifically, tracheomegaly, as a consequence of FETO. We propose that the particularly compliant fetal airway is at risk of mechanical damage from in utero balloon occlusion. This observation of a new problem in this cohort suggests a thorough evaluation of the trachea should be performed in children who have had FETO in utero. It may be that balloon occlusion of the trachea earlier in utero (before 26 weeks' gestation) predisposes to this condition. (orig.)

Tracheomegaly: a complication of fetal endoscopic tracheal occlusion in the treatment of congenital diaphragmatic hernia

Energy Technology Data Exchange (ETDEWEB)

McHugh, Kieran; Afaq, Asim; Roebuck, Derek J [Great Ormond Street Hospital for Children, Radiology Department, London (United Kingdom); Broderick, Nigel [Nottingham University Hospitals, Radiology Department, Nottingham (United Kingdom); Gabra, Hany O; Elliott, Martin J [Great Ormond Street Hospital for Children, Department of Cardiothoracic Surgery, London (United Kingdom)

2010-05-15

Fetal endoscopic tracheal occlusion (FETO) is a promising treatment for severe congenital diaphragmatic hernia, a condition that carries significant morbidity and mortality. It is hypothesised that balloon occlusion of the fetal trachea leads to an improvement in lung growth and development. The major documented complications of FETO to date are related to preterm delivery. To report a series of five infants who developed tracheomegaly following FETO. Review of all children referred with tracheomegaly to the paediatric intensive care and tracheal service at two referral centres. Five neonates presented with features of respiratory distress shortly after birth and were subsequently found to have marked tracheomegaly. Two neonates had tracheomalacia in addition. There are no previous reports in the literature describing tracheomalacia, or more specifically, tracheomegaly, as a consequence of FETO. We propose that the particularly compliant fetal airway is at risk of mechanical damage from in utero balloon occlusion. This observation of a new problem in this cohort suggests a thorough evaluation of the trachea should be performed in children who have had FETO in utero. It may be that balloon occlusion of the trachea earlier in utero (before 26 weeks' gestation) predisposes to this condition. (orig.)

Long-term neurodevelopmental outcomes of congenital diaphragmatic hernia survivors not treated with extracorporeal membrane oxygenation.

Science.gov (United States)

Frisk, Virginia; Jakobson, Lorna S; Unger, Sharon; Trachsel, Daniel; O'Brien, Karel

2011-07-01

Although there has been a marked improvement in the survival of children with congenital diaphragmatic hernia (CDH) in the past 2 decades, there are few reports of long-term neurodevelopmental outcome in this population. The present study examined neurodevelopmental outcomes in 10- to 16-year-old CDH survivors not treated with extracorporeal membrane oxygenation (ECMO). Parents of 27 CDH survivors completed questionnaires assessing medical problems, daily living skills, educational outcomes, behavioral problems, and executive functioning. Fifteen CDH survivors and matched full-term controls completed standardized intelligence, academic achievement, phonological processing, and working memory tests. Non-ECMO-treated CDH survivors demonstrated high rates of clinically significant difficulties on standardized academic achievement measures, and 14 of the 27 survivors had a formal diagnosis of specific learning disability, attention deficit hyperactivity disorder, or developmental disability. Specific problems with executive function, cognitive and attentional weaknesses, and social difficulties were more common in CDH patients than controls. Perioperative hypocapnia was linked to executive dysfunction, behavioral problems, lowered intelligence, and poor achievement in mathematics. Non-ECMO-treated CDH survivors are at substantial risk for neurodevelopmental problems in late childhood and adolescence. Copyright © 2011 Elsevier Inc. All rights reserved.

Management of diaphragmatic rupture from blunt trauma.

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Tan, K K; Yan, Z Y; Vijayan, A; Chiu, M T

2009-12-01

Diagnosis of diaphragmatic rupture is difficult, and delays could result in a catastrophic outcome. We reviewed our institution's management of patients with diaphragmatic rupture after blunt trauma. All patients in this study were treated at Tan Tock Seng Hospital, Singapore, from March 2002 to October 2008. Patients with penetrating injuries were excluded. The parameters included age, mechanism of injury, haemodynamic status at admission, Glasgow coma scale (GCS) score, injury severity score (ISS), imaging studies, location of diaphragmatic injuries, associated injuries and outcome. 14 patients with a median age of 38 years formed the study group. Vehicular-related incidents accounted for 71.4 percent of the injuries. The median GCS score on admission was 14 (range 3-15), while the median systolic blood pressure and heart rate were 94 (range 50-164) mmHg and 110 (range 76-140) beats per minute, respectively. The median ISS was 41 (range 14-66). All had chest radiographs performed in the emergency department, six (42.9 percent) had computed tomography performed before surgery, while the remaining eight (57.1 percent) were sent straight to the operating theatre from the emergency department. There were five (35.7 percent) right-sided and nine (64.3 percent) left-sided diaphragmatic ruptures. The mortality rate was 35.7 percent. Some of the associated injuries included eight (57.1 percent) splenic lacerations, five (35.7 percent) haemothorax and lung injuries, four (28.6 percent) bone fractures and three (21.4 percent) liver lacerations. 12 (85.7 percent) patients underwent repair of the diaphragmatic rupture using interrupted polypropylene suture, while the remaining two (14.3 percent) were too haemodynamically unstable to undergo definitive treatment. Advanced age, haemodynamic instability and raised ISS were associated with mortality. An accurate diagnosis of diaphragmatic rupture in trauma patients is difficult, and a thorough examination of both the

[Ladder step strategy for surgical repair of congenital concealed penis in children].

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Wang, Fu-Ran; Zhong, Hong-Ji; Chen, Yi; Zhao, Jun-Feng; Li, Yan

2016-11-01

To assess the feasibility of the ladder step strategy in surgical repair of congenital concealed penis in children. This study included 52 children with congenital concealed penis treated in the past two years by surgical repair using the ladder step strategy, which consists of five main steps: cutting the narrow ring of the foreskin, degloving the penile skin, fixing the penile skin at the base, covering the penile shaft, and reshaping the prepuce. The perioperative data of the patients were prospectively collected and statistically described. Of the 52 patients, 20 needed remodeling of the frenulum and 27 received longitudinal incision in the penoscrotal junction to expose and deglove the penile shaft. The advanced scrotal flap technique was applied in 8 children to cover the penile shaft without tension, the pedicled foreskin flap technique employed in 11 to repair the penile skin defect, and excision of the webbed skin of the ventral penis performed in another 44 to remodel the penoscrotal angle. The operation time, blood loss, and postoperative hospital stay were 40－100 minutes, 5－30 ml, and 3－6 days, respectively. Wound bleeding and infection occurred in 1 and 5 cases, respectively. Follow-up examinations at 3 and 6 months after surgery showed that all the children had a satisfactory penile appearance except for some minor complications (2 cases of penile retraction, 2 cases of redundant ventral skin, and 1 case of iatrogenic penile curvature). The ladder step strategy for surgical repair of congenital concealed penis in children is a simple procedure with minor injury and satisfactory appearance of the penis.

Thoracoscopic repair of congenital tracheo-oesophageal fistula manifesting in an adult

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Hrishikesh P Salgaonkar

2014-01-01

Full Text Available Congenital trcheo-oesophageal fistula (TOF without oesophageal atresia is usually diagnosed and managed in the neonatal period. Its presentation in adulthood is a rarity. Traditional treatment of a TOF in adults involves its repair via a thoracotomy. We report the case of a 23-year-old man diagnosed with an H-type TOF during workup undertaken for his symptoms of gastro-oesophageal reflux. This fistula located at the level of third thoracic vertebra was repaired successfully using a thoracoscopic approach.

Bilateral diaphragmatic paralysis after cardiac surgery: ventilatory assistance by nasal mask continuous positive airway pressure.

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Hoch, B; Zschocke, A; Barth, H; Leonhardt, A

2001-01-01

The case of an 8-month-old boy with bilateral diaphragmatic paralysis after surgical reoperation for congenital heart disease is presented. In order to avoid repeated intubation and long-term mechanical ventilation or tracheotomy, we used nasal mask continuous positive airway pressure (CPAP) as an alternative method for assisted ventilation. Within 24 hours the boy accepted the nasal mask and symptoms such as dyspnea and sweating disappeared. Respiratory movements became regular and oxygen saturation increased. Nasal mask CPAP may serve as an alternative treatment of bilateral diaphragmatic paralysis in infants, thereby avoiding tracheotomy or long-term mechanical ventilation.

The Canadian Pediatric Surgery Network (CAPSNet): Lessons Learned from a National Registry Devoted to the Study of Congenital Diaphragmatic Hernia and Gastroschisis.

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Butler, Alison E; Puligandla, Pramod S; Skarsgard, Erik D

2015-12-01

The Canadian Pediatric Surgery Network (CAPSNet) was created in 2005 by a geographically representative, multidisciplinary group of clinicians and researchers with the intent of establishing a national research registry for gastroschisis (GS) and congenital diaphragmatic hernia (CDH). Since then, CAPSNet has used this registry and its 16-center network to make contributions to the knowledge base informing best practices for GS and CDH care. More recently, CAPSNet has expanded its focus to include quality assurance and improvement at each of its sites, by issuing a benchmarked outcomes "report card" with its annual report. Finally, a major objective of CAPSNet has been to establish and adopt standardized, evidence-based practice guidelines for GS and CDH across all Canadian perinatal centers. Georg Thieme Verlag KG Stuttgart · New York.

Congenital absence of anterior papillary muscle of the tricuspid valve and surgical repair with artificial chordae.

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Tian, Chuan; Pan, Shiwei

2017-02-01

We report the case of a 26-year old woman who underwent successful tricuspid valve repair for the absence of the anterior papillary of the tricuspid valve. Preoperative echocardiography revealed grade IV tricuspid valve regurgitation, caused by congenital absence of the anterior papillary muscle and prolapse of the anterior leaflet. Tricuspid valve repair was performed using artificial chords consisting of two polytetrafluoroethylene sutures and a concomitant ring annuloplasty. Postoperative echocardiography revealed mild tricuspid valve regurgitation. This approach represented a safe and effective technique for tricuspid valve repair in congenital absence of papillary muscle. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Combined laparoscopic and open technique for repair of congenital abdominal hernia

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Ye, Qinghuang; Chen, Yan; Zhu, Jinhui; Wang, Yuedong

2017-01-01

Abstract Background: Prune belly syndrome (PBS) is a rare congenital disorder among adults, and the way for repairing abdominal wall musculature has no unified standard. Materials and methods: We described combining laparoscopic and open technique in an adult male who presented with PBS. Physical examination and radiological imaging verified the case of PBS. The deficiency of abdominal wall musculature was repaired by combining laparoscopic and open technique using a double-deck complex patch. Results: The patient successfully underwent abdominal wall repair by combining laparoscopic and open technique. Postoperative recovery was uneventful, and improvement in symptom was significant in follow-up after 3, 6, 12, and 24 months. Conclusions: Combining laparoscopic and open technique for repair of deficiency of abdominal wall musculature in PBS was an exploratory way to improve life quality. PMID:29049186

Evaluation of diaphragmatic motion in normal and diaphragmatic paralyzed dogs using M-mode ultrasonography.

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Choi, Mihyun; Lee, Namsoon; Kim, Ahyoung; Keh, Seoyeon; Lee, Jinsoo; Kim, Hyunwook; Choi, Mincheol

2014-01-01

Diagnosis of unilateral diaphragmatic paralysis in dogs is currently based on fluoroscopic detection of unequal movement between the crura. Bilateral paralysis may be more difficult to confirm with fluoroscopy because diaphragmatic movement is sometimes produced by compensatory abdominal muscle contractions. The purpose of this study was to develop a new method to evaluate diaphragmatic movement using M-mode ultrasonography and to describe findings for normal and diaphragmatic paralyzed dogs. Fifty-five clinically normal dogs and two dogs with diaphragmatic paralysis were recruited. Thoracic radiographs were acquired for all dogs and fluoroscopy studies were also acquired for clinically affected dogs. Two observers independently measured diaphragmatic direction of motion and amplitude of excursion using M-mode ultrasonography for dogs meeting study inclusion criteria. Eight of the clinically normal dogs were excluded due to abnormal thoracic radiographic findings. For the remaining normal dogs, the lower limit values of diaphragmatic excursion were 2.85-2.98 mm during normal breathing. One dog with bilateral diaphragmatic paralysis showed paradoxical movement of both crura at the end of inspiration. One dog with unilateral diaphragmatic paralysis had diaphragmatic excursion values of 2.00 ± 0.42 mm on the left side and 4.05 ± 1.48 mm on the right side. The difference between left and right diaphragmatic excursion values was 55%. Findings indicated that M-mode ultrasonography is a relatively simple and objective method for measuring diaphragmatic movement in dogs. Future studies are needed in a larger number of dogs with diaphragmatic paralysis to determine the diagnostic sensitivity of this promising new technique. © 2013 American College of Veterinary Radiology.

Diaphragmatic hernia: diagnostic approaches with review of the literature

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Eren, Suat [Department of Radiology, Faculty of Medicine, Atatuerk University, 25240 Erzurum (Turkey)]. E-mail: suateren@atauni.edu.tr; Ciris, Fahri [Department of Radiology, Faculty of Medicine, Atatuerk University, 25240 Erzurum (Turkey)

2005-06-01

Because surgical repair is indicated for the treatment of diaphragmatic hernia (DH), preoperative imaging of the diaphragmatic defect, hernia content, and associated complications with other organ's pathologies is important. While various techniques can be used on imaging of DHs, selection of the most effective but the least invasive technique will present the most accurate findings about DH, and will facilitate the management of DH. We reviewed the diaphragmatic hernia types associated with our cases, and we discussed the preferred imaging modalities for different DHs with review of the literature. We evaluated the imaging findings of 21 DH cases. They were Morgagni's hernia (n = 4), Bochdalek hernia (n = 2), iatrogenic DH (n = 4), traumatic DH (n = 6), and hiatal hernia (n = 5). Although its limited findings on DH and indirect findings about the diaphragmatic rupture, plain radiography is firstly preferred technique on DH. We found that ultrasound (US) is a useful tool on DH, on traumatic DH cases especially. Not only it shows diaphragmatic continuity and herniated organs, but also it reveals associated abdominal organ's pathologies. Computed tomography (CT) scan is most effective in many DH cases. It shows the herniated abdominal organs together with complications, such as intestinal strangulation, haemothorax, and rib fractures. We stressed that Multislice CT scan with coronal and sagittal reformatted images is the most effective and useful imaging technique on DH. With high sensitivity for soft tissue, MR imaging may be performed in the selected patients, on the late presenting DH cases or on the cases of the diagnosis still in doubt especially.

The Upturned Superior Mesenteric Artery Sign for First-Trimester Detection of Congenital Diaphragmatic Hernia and Omphalocele.

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Lakshmy, Ravi Selvaraj; Agnees, Joy; Rose, Nity

2017-03-01

The aim of this study was to follow the course of the superior mesenteric artery (SMA) in first-trimester fetuses to predict the location of the small bowel. Its abnormal course aids in early detection of congenital diaphragmatic hernia (CDH) and assessment of the contents of omphalocele. The SMA can be easily identified in a sagittal section of the fetus by using color Doppler sonography at the 11- to 14-week scan, and normally, it has a downward course caudally to supply the intestines. The course of the SMA points to the location of the bowel. We report a series of 7 cases detected in first trimester with an abnormal course of the SMA, 3 of which had CDH and 4 of which had omphalocele. In CDH, the intestines herniate into the thoracic cavity; hence, the SMA tends to have an upward course toward the thorax. In 4 cases of omphalocele, the SMA follows the exteriorized bowel into the base of the umbilical cord. Second-trimester sonography for detection of congenital malformations is a standardized protocol, but a careful anatomic survey at the 11- to 14-week scan is often rewarding. When there is a suspicion of an intrathoracic mass or a mediastinal shift, the upturned course of SMA serves as a valuable sign in confirmation of CDH. Chromosomal abnormalities are often reported in cases of omphalocele containing small bowel only, and the upward course of the SMA toward the base of the cord helps in its early prenatal diagnosis, which facilitates early genetic assessment in these fetuses. © 2017 by the American Institute of Ultrasound in Medicine.

Prenatally diagnosed 17q12 microdeletion syndrome with a novel association with congenital diaphragmatic hernia.

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Hendrix, Nancy W; Clemens, Michele; Canavan, Timothy P; Surti, Urvashi; Rajkovic, Aleksandar

2012-01-01

We describe the first reported case of a prenatally diagnosed and recently described 17q12 microdeletion syndrome. The fetus was noted to have a congenital diaphragmatic hernia (CDH), echogenic kidneys and cystic left lung on prenatal ultrasound. The patient underwent amniocentesis which resulted in a normal fluorescence in-situ hybridization and karyotype. An oligonucleotide microarray was then performed which demonstrated a 1.4-Mb deletion within the 17q12 region. The deletion caused haploinsufficiency for 17 genes, including AATF, ACACA, DDX52, DUSP14, GGNBP2, HNF-1B, LHX1, PIGW, SYNRG, TADA2A, and ZNHIT3. The deleted region on 17q12 is similar in size and gene content to previously reported 17q12 microdeletion syndromes, which have a minimal critical region of 1.52 Mb. The newly described 17q12 microdeletion syndrome has been associated with MODY5 (maturity-onset of diabetes of the young type 5), cystic renal disease, pancreatic atrophy, liver abnormalities, cognitive impairment and structural brain abnormalities. CDH has not been previously described with the 17q12 microdeletion syndrome. We hypothesize that CDH is part of the spectrum of this syndrome and likely not detected postnatally due to high prenatal mortality. Copyright © 2011 S. Karger AG, Basel.

Extra Lobes of Liver and Congenital Anomalies of Diaphragm in Children

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I.P. Zhurylo

2014-09-01

Full Text Available The literature data on hepatic congenital anomalies in children are summarized in this article. Three clinical observations of transposition of extra lobe of the liver (ELL into the thoracic cavity in children were analyzed. The true diaphragmatic hernia was found in all cases. Clinical manifestations of this disease depend on the severity of the compression of mediastinum organs. Indications for surgical correction were determined individually. ELL move into the abdominal cavity, excision of the hernia sac and alloplasty of the diaphragmatic defect were performed for one patient. Possible mechanisms for the formation of these complex congenital malformations were discussed based on the comparison of clinical data with the stages of embryogenesis liver, diaphragm and heart.

Diagnosis of congenital cystic adenomatoid malformation of the lung in newborn infants and children

NARCIS (Netherlands)

Heij, H. A.; Ekkelkamp, S.; Vos, A.

1990-01-01

Seventeen patients were diagnosed as having congenital cystic adenomatoid malformation of the lung during 1970-88. One case was associated with congenital diaphragmatic hernia and the child died before operation. The other 16 children underwent successful surgery. The patients presented in one of

Long-term nutritional morbidity for congenital diaphragmatic hernia survivors: Failure to thrive extends well into childhood and adolescence.

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Haliburton, Beth; Mouzaki, Marialena; Chiang, Monping; Scaini, Vikki; Marcon, Margaret; Moraes, Theo J; Chiu, Priscilla P

2015-05-01

Failure to thrive (FTT) is well documented among congenital diaphragmatic hernia (CDH) survivors ≤3years of age, but its etiology, severity, and persistence beyond this age require further elucidation. We conducted a single-center, retrospective study assessing anthropometrics, measured energy expenditure, and feeding tube (FT) use of 5-17 year olds in our multidisciplinary CDH clinic since January 2001. We stratified clinic visits based on age A: 5.0-6.9, B: 7.0-9.9, C: 10.0-14.9, and D: 15-17.9years. One hundred sixteen patients with 376 outpatient visits were reviewed. Anthropometric z-scores were below zero and did not vary across age cohorts. FTT and growth stunting each occurred in 14% of clinic visits. FTs inserted during infancy occurred in 25% of patients, and 60% remained by age 7years. In cohort A, those with FTs were lighter and shorter than those without (pFailure to thrive continues in long-term CDH survivors, FTs may not improve incidence of FTT. Increased energy expenditure may play a role. Copyright © 2015 Elsevier Inc. All rights reserved.

The case for early use of rapid whole genome sequencing in management of critically ill infants: Late diagnosis of Coffin-Siris syndrome in an infant with left congenital diaphragmatic hernia, congenital heart disease and recurrent infections.

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Sweeney, Nathaly M; Nahas, Shareef A; Chowdhury, Shimul; Del Campo, Miguel; Jones, Marilyn C; Dimmock, David P; Kingsmore, Stephen F; Investigators, Rcigm

2018-03-16

Congenital diaphragmatic hernia (CDH) results from incomplete formation of the diaphragm leading to herniation of abdominal organs into the thoracic cavity. CDH is associated with pulmonary hypoplasia, congenital heart disease and pulmonary hypertension. Genetically, it is associated with aneuploidies, chromosomal copy number variants, and single gene mutations. CDH is the most expensive non-cardiac congenital defect: Management frequently requires implementation of Extracorporeal Membrane Oxygenation (ECMO), which increases management expenditures 2.4 - 3.5-fold. The cost of management of CDH has been estimated to exceed $250 million per year. Despite in hospital survival of 80-90%, current management is imperfect, as a great proportion of surviving children have long-term functional deficits. We report the case of a premature infant prenatally diagnosed with CDH and congenital heart disease, who had a protracted and complicated course in the intensive care unit with multiple surgical interventions, including post-cardiac surgery ECMO, gastrostomy tube placement with Nissen fundoplication, tracheostomy for respiratory failure, recurrent infections and developmental delay. Rapid whole genome sequencing (rWGS) identified a de novo, likely pathogenic, c.3096_3100delCAAAG (p.Lys1033Argfs*32) variant in ARID1B, providing a diagnosis of Coffin-Siris syndrome. Her parents elected palliative care and she died later that day. Had rWGS been performed as a neonate, eight months of suffering and futile healthcare utilization may have been avoided. Cold Spring Harbor Laboratory Press.

Hepatic fibrosarcoma incarcerated in a peritoneopericardial diaphragmatic hernia in a cat

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Michael Linton

2016-03-01

Full Text Available Case summary A 14-year-old, female neutered domestic shorthair presented for dyspnoea. Thoracic ultrasonography and radiography showed that a heterogeneous mass was present within the pericardial sac, and the mass continued caudally with the mesenteric fat. On CT, the outline of the diaphragm was not continuous and there was an obvious defect with diaphragmatic thickening present at the mid-level of the liver. A pleural effusion and a small-volume pericardial effusion were also present. A ventral midline coeliotomy and median sternotomy revealed a 5 × 6 × 7 cm firm, irregular, tan-coloured soft tissue mass within the pericardial sac attached to both the diaphragmatic defect and liver. The mass was carefully dissected away from the heart and the diaphragmatic defect was repaired with primary closure. Postoperatively, the cat had a persistent pneumothorax that required continuous pleural suction for 41 h. The cat died 44 h postoperatively. Histopathology and immunohistochemistry confirmed the mass to be a hepatic fibrosarcoma incarcerated in a peritoneopericardial diaphragmatic hernia (PPDH. Relevance and novel information This is the first reported case of metaplastic transformation of liver into a sarcoma in a cat with PPDH. In addition, hepatic fibrosarcoma is a rarely reported location for fibrosarcoma in this species.

Standardization of pulmonary ventilation technique using volume-controlled ventilators in rats with congenital diaphragmatic hernia

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Rodrigo Melo Gallindo

Full Text Available OBJECTIVE: To standardize a technique for ventilating rat fetuses with Congenital Diaphragmatic Hernia (CDH using a volume-controlled ventilator. METHODS: Pregnant rats were divided into the following groups: a control (C; b exposed to nitrofen with CDH (CDH; and c exposed to nitrofen without CDH (N-. Fetuses of the three groups were randomly divided into the subgroups ventilated (V and non-ventilated (N-V. Fetuses were collected on day 21.5 of gestation, weighed and ventilated for 30 minutes using a volume-controlled ventilator. Then the lungs were collected for histological study. We evaluated: body weight (BW, total lung weight (TLW, left lung weight (LLW, ratios TLW / BW and LLW / BW, morphological histology of the airways and causes of failures of ventilation. RESULTS: BW, TLW, LLW, TLW / BW and LLW / BW were higher in C compared with N- (p 0.05. The morphology of the pulmonary airways showed hypoplasia in groups N- and CDH, with no difference between V and N-V (p <0.05. The C and N- groups could be successfully ventilated using a tidal volume of 75 ìl, but the failure of ventilation in the CDH group decreased only when ventilated with 50 ìl. CONCLUSION: Volume ventilation is possible in rats with CDH for a short period and does not alter fetal or lung morphology.

Prenatal diagnosis and perinatal outcome of 38 cases with congenital diaphragmatic hernia: 8-year experience of a tertiary Brazilian center Diagnóstico pré-natal e evolução perinatal de 38 casos de hérnia diafragmática congênita: 8 anos de experiência de um serviço terciário brasileiro

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Rodrigo Ruano

2006-06-01

Full Text Available PURPOSE: To evaluate the perinatal results for neonates with congenital diaphragmatic hernia diagnosed prenatally. METHOD: We reviewed data from 38 cases of congenital diaphragmatic hernia diagnosed prenatally from January 1995 to December 2003 in the Fetal Medicine Unit of the Department of Obstetrics and Gynecology, São Paulo University Medical School. The main data analyzed were gestational age at diagnosis, fetal karyotyping, side of diaphragmatic defect, presence of associated structural malformations, hepatic herniation, and severe mediastinal shift. Perinatal outcomes were obtained by reviewing hospital documents or by directly calling the patients' immediate relatives. RESULTS: Mean gestational age at diagnosis was 29 weeks (range, 16-37 weeks.Thirty (79% cases had a left diaphragmatic defect and 8 (21% had a right lesion. Associated structural malformations were observed in 21 (55% cases, in which 12 fetuses had a normal karyotype and 9 had chromosomal abnormalities. Isolated congenital diaphragmatic hernia was confirmed in 17 (45% cases. The overall perinatal mortality rate was 92%. Rates of fetal deaths, early neonatal deaths, late neonatal deaths, and survival were 42%, 50%, 0%, and 8%, respectively, in cases with associated structural malformations but normal karyotyping; 56%, 44%, 0%, and 0% for cases with chromosomal abnormalities; and, 0%, 76%, 12%, and 12% in cases with isolated congenital diaphragmatic hernia. The neonatal mortality rate was 89% in cases with isolated congenital diaphragmatic hernia. CONCLUSION: Perinatal mortality was very high in prenatally diagnosed cases of congenital diaphragmatic hernia. Earlier perinatal deaths are associated with the presence of other structural defects or chromosomal abnormalities. In cases of isolated congenital diaphragmatic hernia, mortality is related to the presence of herniated liver, right-sided lesion, and major mediastinal shift.OBJETIVO: Avaliar os resultados neonatais dos

The VICI-trial: high frequency oscillation versus conventional mechanical ventilation in newborns with congenital diaphragmatic hernia: an international multicentre randomized controlled trial.

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van den Hout, Lieke; Tibboel, Dick; Vijfhuize, Sanne; te Beest, Harma; Hop, Wim; Reiss, Irwin

2011-11-02

Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly of the diaphragm resulting in pulmonary hypoplasia and pulmonary hypertension. It is associated with a high risk of mortality and pulmonary morbidity. Previous retrospective studies have reported high frequency oscillatory ventilation (HFO) to reduce pulmonary morbidity in infants with CDH, while others indicated HFO to be associated with worse outcome. We therefore aimed to develop a randomized controlled trial to compare initial ventilatory treatment with high-frequency oscillation and conventional ventilation in infants with CDH. This trial is designed as a multicentre trial in which 400 infants (200 in each arm) will be included. Primary outcome measures are BPD, described as oxygen dependency by day 28 according to the definition of Jobe and Bancalari, and/or mortality by day 28. All liveborn infants with CDH born at a gestational age of over 34 weeks and no other severe congenital anomalies are eligible for inclusion. Parental informed consent is asked antenatally and the allocated ventilation mode starts within two hours after birth. Laboratory samples of blood, urine and tracheal aspirate are taken at the first day of life, day 3, day 7, day 14 and day 28 to evaluate laboratory markers for ventilator-induced lung injury and pulmonary hypertension. To date, randomized clinical trials are lacking in the field of CDH. The VICI-trial, as the first randomized clinical trial in the field of CDH, may provide further insight in ventilation strategies in CDH patient. This may hopefully prevent mortality and morbidity. Netherlands Trial Register (NTR): NTR1310.

Postoperative Feeding Difficulties after Repair of Congenital ...

African Journals Online (AJOL)

Of these, 24 (37.5%) developed feeding difficulties in the immediate post operative period. The causes of the feeding difficulties were Gastro-oesophageal reflux (GOR) 9, Recurrent diaphragmatic hernia 8, Adhesive intestinal obstruction 4, Poor intestinal motility 2, Campylobacter enteritis, 1, Hypertrophic pyloric stenosis, 1.

Diaphragmatic crural eventration

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Sivakumar K

2008-01-01

Full Text Available Aim: We evaluated patients with gastric volvulus secondary to diaphragmatic pathology. Materials and Methods: Eight patients (5 males and 3 females presented to the author in a tertiary care center during 1997-2006 were analyzed in terms of age, sex, symptomatology, diagnosis and predisposing factors. Observations: Six had an acute presentation and rest had chronic symptomatology. The two patients who had total gangrene stomach died postoperatively and one patient died preoperatively due to aspiration. All the cases presented with acute symptoms had diaphragmatic pathology, and out of these, three cases had the specific entity, which is named as diaphragmatic crural eventration. Conclusions: Diaphragmatic crural eventration is characterized by the defective development of the right crus of diaphragm, and this is embryologically significant as the right crus and ligaments of the stomach develop from dorsal mesoesophagus and mesogastrium. The author recommends a closer look for this defect of diaphragm while operating a case of gastric volvulus.

Unusual cause of brachial palsy with diaphragmatic palsy.

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Gupta, Vishal; Pandita, Aakash; Panghal, Astha; Hassan, Neha

2018-05-12

We report a preterm neonate born with respiratory distress. The neonate was found to have diaphragmatic palsy and brachial palsy. The neonate was born by caesarean section and there was no history of birth trauma. On examination, there was bilateral congenital talipes equinovarus and a scar was present on the forearm. The mother had a history of chickenpox during the 16 weeks of pregnancy for which no treatment was sought. On investigation, PCR for varicella was found to be positive in the neonate. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Assessment of lung development in isolated congenital diaphragmatic hernia using signal intensity ratios on fetal MR imaging

International Nuclear Information System (INIS)

Balassy, Csilla; Kasprian, Gregor; Weber, Michael; Herold, Christian; Prayer, Daniela; Brugger, Peter C.; Csapo, Bence

2010-01-01

To investigate developmental changes in the apparently unaffected contralateral lung by using signal intensity ratios (SIR) and lung volumes (LV), and to search for correlation with clinical outcome. Twenty-five fetuses (22-37 weeks' gestation) were examined. Lung/liver signal intensity ratios (LLSIR) were assessed on T1-weighted and T2-weighted sequences for both lungs, then together with LV compared with age-matched controls of 91 fetuses by using the U test. Differences in LLSIRs and lung volumes were correlated with neonatal outcomes. LLSIRs in fetuses with congenital diaphragmatic hernia (CDH) were significantly higher in both lungs on T1-weighted images and significantly lower on T2-weighted images, compared with normals (p < 0.05), increasing on T2-weighted imaging and decreasing on T1-weighted imaging during gestation. Total LV were significantly smaller in the CDH group than in controls (p < 0.05). No significant differences in LLSIR of the two lungs were found. Outcomes correlated significantly with total LV, but not with LLSIR. Changes in LLSIR seem to reflect developmental impairment in CDH; however, they provide no additional information in predicting outcome. LV remains the best indicator on fetal MR imaging of neonatal survival in isolated, left-sided CDH. (orig.)

Assessment of lung development in isolated congenital diaphragmatic hernia using signal intensity ratios on fetal MR imaging

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Balassy, Csilla; Kasprian, Gregor; Weber, Michael; Herold, Christian; Prayer, Daniela [Medical University of Vienna, Department of Radiology, Vienna (Austria); Brugger, Peter C. [Medical University of Vienna, Centre of Anatomy and Cell Biology, Vienna (Austria); Csapo, Bence [Medical University of Vienna, Department of Obstetrics and Gyneocology, Vienna (Austria)

2010-04-15

To investigate developmental changes in the apparently unaffected contralateral lung by using signal intensity ratios (SIR) and lung volumes (LV), and to search for correlation with clinical outcome. Twenty-five fetuses (22-37 weeks' gestation) were examined. Lung/liver signal intensity ratios (LLSIR) were assessed on T1-weighted and T2-weighted sequences for both lungs, then together with LV compared with age-matched controls of 91 fetuses by using the U test. Differences in LLSIRs and lung volumes were correlated with neonatal outcomes. LLSIRs in fetuses with congenital diaphragmatic hernia (CDH) were significantly higher in both lungs on T1-weighted images and significantly lower on T2-weighted images, compared with normals (p < 0.05), increasing on T2-weighted imaging and decreasing on T1-weighted imaging during gestation. Total LV were significantly smaller in the CDH group than in controls (p < 0.05). No significant differences in LLSIR of the two lungs were found. Outcomes correlated significantly with total LV, but not with LLSIR. Changes in LLSIR seem to reflect developmental impairment in CDH; however, they provide no additional information in predicting outcome. LV remains the best indicator on fetal MR imaging of neonatal survival in isolated, left-sided CDH. (orig.)

Body composition variation following diaphragmatic breathing ...

African Journals Online (AJOL)

Body composition variation following diaphragmatic breathing. ... effect of commonly prescribed diaphragmatic breathing training on the body composition ... a non-exercising control (NE) group (n = 22) or diaphragmatic breathing (DB) group.

The VICI-trial: high frequency oscillation versus conventional mechanical ventilation in newborns with congenital diaphragmatic hernia: an international multicentre randomized controlled trial

Directory of Open Access Journals (Sweden)

van den Hout Lieke

2011-11-01

Full Text Available Abstract Background Congenital diaphragmatic hernia (CDH is a severe congenital anomaly of the diaphragm resulting in pulmonary hypoplasia and pulmonary hypertension. It is associated with a high risk of mortality and pulmonary morbidity. Previous retrospective studies have reported high frequency oscillatory ventilation (HFO to reduce pulmonary morbidity in infants with CDH, while others indicated HFO to be associated with worse outcome. We therefore aimed to develop a randomized controlled trial to compare initial ventilatory treatment with high-frequency oscillation and conventional ventilation in infants with CDH. Methods/design This trial is designed as a multicentre trial in which 400 infants (200 in each arm will be included. Primary outcome measures are BPD, described as oxygen dependency by day 28 according to the definition of Jobe and Bancalari, and/or mortality by day 28. All liveborn infants with CDH born at a gestational age of over 34 weeks and no other severe congenital anomalies are eligible for inclusion. Parental informed consent is asked antenatally and the allocated ventilation mode starts within two hours after birth. Laboratory samples of blood, urine and tracheal aspirate are taken at the first day of life, day 3, day 7, day 14 and day 28 to evaluate laboratory markers for ventilator-induced lung injury and pulmonary hypertension. Discussion To date, randomized clinical trials are lacking in the field of CDH. The VICI-trial, as the first randomized clinical trial in the field of CDH, may provide further insight in ventilation strategies in CDH patient. This may hopefully prevent mortality and morbidity. Trial registration Netherlands Trial Register (NTR: NTR1310

MR diagnosis of diaphragmatic endometriosis

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Rousset, Pascal; Gregory, Jules; Coste, Joel; Rousset-Jablonski, Christine; Hugon-Rodin, Justine; Regnard, Jean-Francois; Chapron, Charles; Golfier, Francois; Revel, Marie-Pierre

2016-01-01

To evaluate magnetic resonance imaging (MRI) for diaphragmatic endometriosis diagnosis. Over a 2-year period, all diaphragmatic MRI performed in the context of diaphragmatic endometriosis were reviewed. Axial and coronal fat-suppressed T1- and T2-weighted sequences were analyzed by two independent readers for the presence of nodules, plaque lesions, micronodule clustering, or focal liver herniation. MR abnormalities were correlated to surgical findings in women surgically treated. Interobserver agreement was assessed by κ statistics. Twenty-three women with diaphragmatic endometriosis criteria comprised the population; 14 had surgical confirmation and nine had symptoms relief with hormonal treatment. MRI sensitivity was 83 % (19/23; 95 % confidence interval [CI]: 68, 98) for reader 1 and 78 % (18/23; 95 % CI: 61, 95) for reader 2. Kappa value was 0.86 (95 % CI: 0.47, 1.00). Readers 1 and 2 detected 35 and 36 lesions, respectively, all right-sided and agreed for 32 lesions on the type, location, and signal. Lesions were mostly nodules (23/32, 72 %), predominantly posterior (28/32, 87.5 %) and hyperintense on T1 (20/32, 63 %). MRI was negative for both readers in 2 surgically treated patients with small nodules or isolated diaphragmatic holes. MRI allows diaphragmatic endometriosis diagnosis with 78 to 83 % sensitivity and excellent interobserver agreement. (orig.)

MR diagnosis of diaphragmatic endometriosis

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Rousset, Pascal [Lyon 1 Claude Bernard University, Villeurbanne (France); Centre Hospitalier Lyon Sud, Radiology Department, Pierre Benite (France); Gregory, Jules; Coste, Joel [Paris Descartes University, Sorbonne Paris Cite, Paris (France); Groupe Hospitalier Cochin Hotel-Dieu, Biostatistics and Epidemiology department, Paris (France); Rousset-Jablonski, Christine [Centre Hospitalier Lyon Sud, Obstetric and Gynecologic Department, Pierre Benite (France); Hugon-Rodin, Justine [Paris Descartes University, Sorbonne Paris Cite, Paris (France); Groupe Hospitalier Cochin Hotel-Dieu, Gynecology Endocrinology Department, Paris (France); Regnard, Jean-Francois [Paris Descartes University, Sorbonne Paris Cite, Paris (France); Groupe Hospitalier Cochin Hotel-Dieu, Thoracic Surgery Department, Paris (France); Chapron, Charles [Paris Descartes University, Sorbonne Paris Cite, Paris (France); Groupe Hospitalier Cochin Hotel-Dieu, Obstetric and Gynecologic Department, Paris (France); Golfier, Francois [Lyon 1 Claude Bernard University, Villeurbanne (France); Centre Hospitalier Lyon Sud, Obstetric and Gynecologic Department, Pierre Benite (France); Revel, Marie-Pierre [Paris Descartes University, Sorbonne Paris Cite, Paris (France); Groupe Hospitalier Cochin Hotel-Dieu, Radiology Department, Paris (France)

2016-11-15

To evaluate magnetic resonance imaging (MRI) for diaphragmatic endometriosis diagnosis. Over a 2-year period, all diaphragmatic MRI performed in the context of diaphragmatic endometriosis were reviewed. Axial and coronal fat-suppressed T1- and T2-weighted sequences were analyzed by two independent readers for the presence of nodules, plaque lesions, micronodule clustering, or focal liver herniation. MR abnormalities were correlated to surgical findings in women surgically treated. Interobserver agreement was assessed by κ statistics. Twenty-three women with diaphragmatic endometriosis criteria comprised the population; 14 had surgical confirmation and nine had symptoms relief with hormonal treatment. MRI sensitivity was 83 % (19/23; 95 % confidence interval [CI]: 68, 98) for reader 1 and 78 % (18/23; 95 % CI: 61, 95) for reader 2. Kappa value was 0.86 (95 % CI: 0.47, 1.00). Readers 1 and 2 detected 35 and 36 lesions, respectively, all right-sided and agreed for 32 lesions on the type, location, and signal. Lesions were mostly nodules (23/32, 72 %), predominantly posterior (28/32, 87.5 %) and hyperintense on T1 (20/32, 63 %). MRI was negative for both readers in 2 surgically treated patients with small nodules or isolated diaphragmatic holes. MRI allows diaphragmatic endometriosis diagnosis with 78 to 83 % sensitivity and excellent interobserver agreement. (orig.)

Incidence and factors associated with sensorineural and conductive hearing loss among survivors of congenital diaphragmatic hernia.

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Partridge, Emily A; Bridge, Christina; Donaher, Joseph G; Herkert, Lisa M; Grill, Elena; Danzer, Enrico; Gerdes, Marsha; Hoffman, Casey H; D'Agostino, Jo Ann; Bernbaum, Judy C; Rintoul, Natalie E; Peranteau, William H; Flake, Alan W; Adzick, N Scott; Hedrick, Holly L

2014-06-01

The reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution. We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2004 through December 2012. Categorical variables were analyzed by Fisher's exact test and continuous variables by Mann-Whitney t-test (p≤0.05). A total of 112 patients met study inclusion criteria, with 3 (2.7%) patients diagnosed with SNHL and 38 (34.0%) diagnosed with CHL. SNHL was significantly associated with requirement for ECMO (p=0.0130), prolonged course of hospitalization (p=0.0011), duration of mechanical ventilation (p=0.0046), requirement for tracheostomy (p=0.0013), and duration of loop diuretic (p=0.0005) and aminoglycoside therapy (p=0.0003). We have identified hearing anomalies in over 30% of long-term CDH survivors. These findings illustrate the need for routine serial audiologic evaluations throughout childhood for all survivors of CDH and stress the importance of targeted interventions to optimize long-term developmental outcomes pertaining to speech and language. Copyright © 2014 Elsevier Inc. All rights reserved.

Causes of congenital unilateral pulmonary hypoplasia

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Currarino, G.; Williams, B.; Children's Medical Center, Dallas, TX

1985-01-01

A review of the roentgenograms and clinical records of 33 children with primary congenital underdevelopment of one lung showed that 9 patients had simple pulmonary hypoplasia, 8 had anomalous venous return to the right atrium or the inferior vena cava (scimitar syndrome), 7 had an absence of ipsilateral pulmonary artery, 7 had an accessory diaphragm, and 2 had a pulmonary sequestration adjacent to a small diaphragmatic hernia. (orig.)

VEGF receptor expression decreases during lung development in congenital diaphragmatic hernia induced by nitrofen

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Sbragia, L. [Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil, Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP (Brazil); Nassr, A.C.C. [Departamento de Hidrobiologia do Centro de Ciências Biológicas e da Saúde, Universidade Federal de São Carlos, São Carlos, SP, Brasil, Departamento de Hidrobiologia do Centro de Ciências Biológicas e da Saúde, Universidade Federal de São Carlos, São Carlos, SP (Brazil); Gonçalves, F.L.L. [Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil, Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP (Brazil); Schmidt, A.F. [Pediatrics House Office, Cincinnati Children' s Hospital Medical Center, Cincinnati, OH, USA, Pediatrics House Office, Cincinnati Children' s Hospital Medical Center, Cincinnati, OH (United States); Zuliani, C.C. [Departamento de Clínica Médica, Faculdade de Ciências Médicas, Universidade Estadual de Campinas, Campinas, SP, Brasil, Departamento de Clínica Médica, Faculdade de Ciências Médicas, Universidade Estadual de Campinas, Campinas, SP (Brazil); Garcia, P.V. [Departamento de Histologia e Embriologia, Instituto de Biologia, Universidade Estadual de Campinas, UNICAMP, Campinas, SP, Brasil, Departamento de Histologia e Embriologia, Instituto de Biologia, Universidade Estadual de Campinas, UNICAMP, Campinas, SP (Brazil); Gallindo, R.M. [Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil, Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP (Brazil); Pereira, L.A.V. [Departamento de Histologia e Embriologia, Instituto de Biologia, Universidade Estadual de Campinas, UNICAMP, Campinas, SP, Brasil, Departamento de Histologia e Embriologia, Instituto de Biologia, Universidade Estadual de Campinas, UNICAMP, Campinas, SP (Brazil)

2014-02-17

Changes in vascular endothelial growth factor (VEGF) in pulmonary vessels have been described in congenital diaphragmatic hernia (CDH) and may contribute to the development of pulmonary hypoplasia and hypertension; however, how the expression of VEGF receptors changes during fetal lung development in CDH is not understood. The aim of this study was to compare morphological evolution with expression of VEGF receptors, VEGFR1 (Flt-1) and VEGFR2 (Flk-1), in pseudoglandular, canalicular, and saccular stages of lung development in normal rat fetuses and in fetuses with CDH. Pregnant rats were divided into four groups (n=20 fetuses each) of four different gestational days (GD) 18.5, 19.5, 20.5, 21.5: external control (EC), exposed to olive oil (OO), exposed to 100 mg nitrofen, by gavage, without CDH (N-), and exposed to nitrofen with CDH (CDH) on GD 9.5 (term=22 days). The morphological variables studied were: body weight (BW), total lung weight (TLW), left lung weight, TLW/BW ratio, total lung volume, and left lung volume. The histometric variables studied were: left lung parenchymal area density and left lung parenchymal volume. VEGFR1 and VEGFR2 expression were determined by Western blotting. The data were analyzed using analysis of variance with the Tukey-Kramer post hoc test. CDH frequency was 37% (80/216). All the morphological and histometric variables were reduced in the N- and CDH groups compared with the controls, and reductions were more pronounced in the CDH group (P<0.05) and more evident on GD 20.5 and GD 21.5. Similar results were observed for VEGFR1 and VEGFR2 expression. We conclude that N- and CDH fetuses showed primary pulmonary hypoplasia, with a decrease in VEGFR1 and VEGFR2 expression.

VEGF receptor expression decreases during lung development in congenital diaphragmatic hernia induced by nitrofen

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L. Sbragia

2014-02-01

Full Text Available Changes in vascular endothelial growth factor (VEGF in pulmonary vessels have been described in congenital diaphragmatic hernia (CDH and may contribute to the development of pulmonary hypoplasia and hypertension; however, how the expression of VEGF receptors changes during fetal lung development in CDH is not understood. The aim of this study was to compare morphological evolution with expression of VEGF receptors, VEGFR1 (Flt-1 and VEGFR2 (Flk-1, in pseudoglandular, canalicular, and saccular stages of lung development in normal rat fetuses and in fetuses with CDH. Pregnant rats were divided into four groups (n=20 fetuses each of four different gestational days (GD 18.5, 19.5, 20.5, 21.5: external control (EC, exposed to olive oil (OO, exposed to 100 mg nitrofen, by gavage, without CDH (N-, and exposed to nitrofen with CDH (CDH on GD 9.5 (term=22 days. The morphological variables studied were: body weight (BW, total lung weight (TLW, left lung weight, TLW/BW ratio, total lung volume, and left lung volume. The histometric variables studied were: left lung parenchymal area density and left lung parenchymal volume. VEGFR1 and VEGFR2 expression were determined by Western blotting. The data were analyzed using analysis of variance with the Tukey-Kramer post hoc test. CDH frequency was 37% (80/216. All the morphological and histometric variables were reduced in the N- and CDH groups compared with the controls, and reductions were more pronounced in the CDH group (P<0.05 and more evident on GD 20.5 and GD 21.5. Similar results were observed for VEGFR1 and VEGFR2 expression. We conclude that N- and CDH fetuses showed primary pulmonary hypoplasia, with a decrease in VEGFR1 and VEGFR2 expression.

VEGF receptor expression decreases during lung development in congenital diaphragmatic hernia induced by nitrofen

International Nuclear Information System (INIS)

Sbragia, L.; Nassr, A.C.C.; Gonçalves, F.L.L.; Schmidt, A.F.; Zuliani, C.C.; Garcia, P.V.; Gallindo, R.M.; Pereira, L.A.V.

2014-01-01

Changes in vascular endothelial growth factor (VEGF) in pulmonary vessels have been described in congenital diaphragmatic hernia (CDH) and may contribute to the development of pulmonary hypoplasia and hypertension; however, how the expression of VEGF receptors changes during fetal lung development in CDH is not understood. The aim of this study was to compare morphological evolution with expression of VEGF receptors, VEGFR1 (Flt-1) and VEGFR2 (Flk-1), in pseudoglandular, canalicular, and saccular stages of lung development in normal rat fetuses and in fetuses with CDH. Pregnant rats were divided into four groups (n=20 fetuses each) of four different gestational days (GD) 18.5, 19.5, 20.5, 21.5: external control (EC), exposed to olive oil (OO), exposed to 100 mg nitrofen, by gavage, without CDH (N-), and exposed to nitrofen with CDH (CDH) on GD 9.5 (term=22 days). The morphological variables studied were: body weight (BW), total lung weight (TLW), left lung weight, TLW/BW ratio, total lung volume, and left lung volume. The histometric variables studied were: left lung parenchymal area density and left lung parenchymal volume. VEGFR1 and VEGFR2 expression were determined by Western blotting. The data were analyzed using analysis of variance with the Tukey-Kramer post hoc test. CDH frequency was 37% (80/216). All the morphological and histometric variables were reduced in the N- and CDH groups compared with the controls, and reductions were more pronounced in the CDH group (P<0.05) and more evident on GD 20.5 and GD 21.5. Similar results were observed for VEGFR1 and VEGFR2 expression. We conclude that N- and CDH fetuses showed primary pulmonary hypoplasia, with a decrease in VEGFR1 and VEGFR2 expression

Abordagem ventilatória protetora no tratamento da hérnia diafragmática congênita Gentle ventilatory approach for the treatment of congenital diaphragmatic hernia

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Felipe de Souza Rossi

2008-12-01

Full Text Available OBJETIVO: Descrever a evolução de recém-nascidos com diagnóstico de hérnia diafragmática congênita admitidos na Unidade de Terapia Intensiva Neonatal de um hospital privado de nível terciário, no qual aplicou-se uma estratégia ventilatória protetora. MÉTODOS: Coorte histórica com análise de prontuários de pacientes portadores de hérnia diafragmática congênita, admitidos de junho de 2001 a julho de 2006. Avaliaram-se dados referentes ao recém-nascido (índices prognósticos antenatais, peso ao nascimento, idade gestacional, sexo, dados da reanimação e estabililização pré-operatória, cuidados pós-operatórios e taxa de sobrevida. RESULTADOS: Oito neonatos tiveram diagnóstico de hérnia diafragmática congênita. O peso variou entre 2,38 e 3,45kg e a idade gestacional, entre 36 e 39 semanas; cinco deles eram do sexo masculino. Todos foram intubados em sala de parto até o final do primeiro minuto de vida. A correção cirúrgica ocorreu entre o segundo e o sexto dias de vida e, em quatro pacientes, houve necessidade do uso de patch. Uma estratégia ventilatória protetora foi utilizada em seis neonatos, com dados gasométricos visando PaO2 pré-ductal normal e tolerando-se hipercapnia (PaCO2 50 a 60mmHg. A extubação ocorreu entre o primeiro e o 12ºdias do pós-operatório, com exceção de um paciente. Seis recém-nascidos receberam alta, em média, com 30 dias de vida (19 a 55 dias. A sobrevida foi de 75%. CONCLUSÕES: A sistematização do cuidado de pacientes com hérnia diafragmática congênita pode garantir, em nosso meio, uma sobrevida comparável aos principais centros mundiais que lidam com a doença.OBJECTIVE: To describe the clinical evolution of newborns with congenital diaphragmatic hernia admitted to neoretal Intensive Care Unit of a tertiary private hospital and treated with a gentle ventilatory approach. METHODS: Analysis of charts of patients born between June 2001 and July 2006. The following data

Growth Patterns of Fetal Lung Volumes in Healthy Fetuses and Fetuses With Isolated Left-Sided Congenital Diaphragmatic Hernia.

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Ruano, Rodrigo; Britto, Ingrid Schwach Werneck; Sananes, Nicolas; Lee, Wesley; Sangi-Haghpeykar, Haleh; Deter, Russell L

2016-06-01

To evaluate fetal lung growth using 3-dimensional sonography in healthy fetuses and those with congenital diaphragmatic hernia (CDH). Right and total lung volumes were serially evaluated by 3-dimensional sonography in 66 healthy fetuses and 52 fetuses with left-sided CDH between 20 and 37 weeks' menstrual age. Functions fitted to these parameters were compared for 2 groups: (1) healthy versus those with CDH; and (2) fetuses with CHD who survived versus those who died. Fetal right and total lung volumes as well as fetal observed-to-expected right and total lung volume ratios were significantly lower in fetuses with CDH than healthy fetuses (Pvolume ratios did not vary with menstrual age in healthy fetuses or in those with CDH (independent of outcome). Lung volume rates were lower in fetuses with left-sided CDH compared to healthy fetuses, as well as in fetuses with CDH who died compared to those who survived. The observed-to-expected right and total lung volume ratios were relatively constant throughout menstrual age in fetuses with left-sided CDH, suggesting that the origin of their lung growth abnormalities occurred before 20 weeks and did not progress. The observed-to-expected ratios may be useful in predicting the outcome in fetuses with CDH independent of menstrual age. © 2016 by the American Institute of Ultrasound in Medicine.

Neurotrophins expression is decreased in lungs of human infants with congenital diaphragmatic hernia

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O'Hanlon LD

2014-02-01

Full Text Available Lynn D O'Hanlon, Sherry M Mabry, Ikechukwu I EkekezieChildren's Mercy Hospitals/University of Missouri-Kansas City School of Medicine, Department of Pediatrics, Section of Neonatal-Perinatal Medicine, Kansas City, MO, USAObjectives: To evaluate neurotrophin (NT (nerve growth factor [NGF], NT-3, and brain-derived neurotrophic factor [BDNF] expression in autopsy lung tissues of human congenital diaphragmatic hernia (CDH infants versus that of infants that expired with: 1 "normal" lungs (controls; 2 chronic lung disease (CLD; and 3 pulmonary hypertension (PPHN.Hypothesis: NT expression will be significantly altered in CDH lung tissue compared with normal lung tissue and other neonatal lung diseases.Study design: Immunohistochemical studies for NT proteins NGF, BDNF, and NT-3 were applied to human autopsy neonatal lung tissue samples.Subject selection: The samples included a control group of 18 samples ranging from 23-week gestational age to term, a CDH group of 15 samples, a PPHN group of six samples, and a CLD group of 12 samples.Methodology: The tissue samples were studied, and four representative slide fields of alveoli/saccules and four of bronchioles were recorded from each sample. These slide fields were then graded (from 0 to 3 by three blinded observers for intensity of staining.Results: BDNF, NGF, and NT-3 immunostaining intensity scores were significantly decreased in the CDH lung tissue (n=15 compared with normal neonatal lung tissue (n=18 (P<0.001. The other neonatal pulmonary diseases that were studied, CLD and PPHN, were much less likely to be affected and were much more variable in their neurotrophin expression.Conclusion: NT expression is decreased in CDH lungs. The decreased expression of NT in CDH lung tissue may suggest they contribute to the abnormality in this condition.Keywords: nerve growth factor, NGF, brain-derived neurotrophic factor, BDNF, neurotrophin-3, NT-3, chronic lung disease, persistent pulmonary hypertension, lung

A rare case report of Morgagni Hernia with Organo-Axial Gastric Volvulus and concomitant Para-esophageal hernia, repaired laparoscopically in a Septuagenarian

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Amol Mittal

Full Text Available Introduction: Simultaneous occurrence of Morgagni and the Para-esophageal hernia is a rare clinical condition with eight case reports in the English-language literature and only four managed laparoscopically. We describe a case of a Septuagenarian patient with Morgagni and concomitant Para-esophageal hernia treated laparoscopically. Presentation of a case: A 71-year-old male patient, presented with a one-month history of regurgitation of acid, retrosternal burning and vomiting after eating. Computed tomography (CT imaging demonstrated a large anterior diaphragmatic hernia, with herniation of bowel loops and anterosuperior displacement of the gastric antrum along with a grade III Para-esophageal hernia. The patient underwent simultaneous laparoscopic repair of Morgagni and Para-esophageal hernia with mesh reinforcement with Nissen’s total anti-reflux fundoplication. The patient’s postoperative recovery was uneventful. Discussion: A Morgagni Hernia is a rare congenital condition consisting of a Subcosto-sternal defect in the diaphragm. A Para-esophageal hernia is a rare variant of a hiatus hernia. Morgagni and Para-esophageal hernia may present with gastric volvulus or incarceration, requiring emergency treatment. Minimally invasive surgery is the preferred treatment, particularly for elderly patients and patients with comorbidities. The laparoscopic operation can provide excellent exposure and repair the hernia defect easily with minimal invasiveness and fewer complications. Conclusion: This case report highlights the co-existence of Morgagni and Para-esophageal hernias and validates the feasibility of laparoscopic repair of both hernias simultaneously. Keywords: Diaphragmatic hernia, Morgagni, Para-esophageal hernia, Minimally invasive surgery, Fundoplication, Case report

Congenital superior sternal cleft repair using our modified Sally technique: A case report

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Tomoyuki Kuwata

2014-12-01

Full Text Available We report the case of an 8-month-old male neonate who underwent reconstructive surgery for a congenital superior sternal cleft to decrease the risk of commotio cordis. With regard to use of the Sally technique for the repair of a relatively wide cleft (4 × 4 cm, we were concerned about respiratory problems caused by compression following closure of the sternal halves. By closing the sternal halves imperfectly, elevation of intrapleural pressure can be avoided to some extent. By bridging the surplus resected cartilage from the lower sternum over the gap of the upper sternum, the heart is protected by more rigid material. We recommend our modified Sally technique because it is both flexible and effective for sternal cleft repair.

Prophylactic use of the Arabin cervical pessary in fetuses with severe congenital diaphragmatic hernia treated by fetoscopic endoluminal tracheal occlusion (FETO): preliminary experience.

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Dobrescu, Oana; Cannie, Mieke M; Cordier, Anne-Gael; Rodó, Carlota; Fabietti, Isabella; Benachi, Alexandra; Carreras, Elena; Persico, Nicola; Hurtado, Ivan; Gucciardo, Léonardo; Jani, Jacques C

2016-01-01

The aim of this study was to describe whether the prophylactic use of a cervical pessary decreases the rate of premature birth in congenital diaphragmatic hernia (CDH) fetuses treated with fetoscopic tracheal occlusion (FETO). The study concerns a consecutive series of cases with CDH and FETO and a group of CDH without FETO. In a subgroup of the FETO group, a prophylactic cervical pessary was inserted the day following the procedure. Gestational age (GA) at birth was the primary outcome. Fifty-nine fetuses with FETO and 47 expectantly managed were included. The last 15 FETO had a cervical pessary inserted. The median GA at delivery in the FETO group with pessary was 35.1 weeks and was not different from that in the FETO group without a pessary (34.3 weeks; p = 0.28) but was below that in the expectantly managed group (38.3 weeks; p cervical pessary does not prolong gestation of CDH fetuses treated with FETO. © 2015 John Wiley & Sons, Ltd. © 2015 John Wiley & Sons, Ltd.

Wandering Spleen and Organoaxial Gastric Volvulus after Morgagni Hernia Repair: A Case Report and Review of the Literature

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Noemi Cantone

2016-01-01

Full Text Available Wandering spleen and gastric volvulus are two rare entities that have been described in association with congenital diaphragmatic hernia. The diagnosis is difficult and any delay can result in ischemia and necrosis of both organs. We present a case of a 13-year-old girl, previously operated on for anterior diaphragmatic hernia and intrathoracic gastric volvulus, that presented to our service for a subdiaphragmatic gastric volvulus recurrence associated with a wandering spleen. In this report we reviewed the literature, analyzing the clinical presentation, diagnostic assessment, and treatment options of both conditions, in particular in the case associated with diaphragmatic hernia.

Wandering Spleen and Organoaxial Gastric Volvulus after Morgagni Hernia Repair: A Case Report and Review of the Literature

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Gulia, Caterina; Miele, Vittorio; Trinci, Margherita; Briganti, Vito

2016-01-01

Wandering spleen and gastric volvulus are two rare entities that have been described in association with congenital diaphragmatic hernia. The diagnosis is difficult and any delay can result in ischemia and necrosis of both organs. We present a case of a 13-year-old girl, previously operated on for anterior diaphragmatic hernia and intrathoracic gastric volvulus, that presented to our service for a subdiaphragmatic gastric volvulus recurrence associated with a wandering spleen. In this report we reviewed the literature, analyzing the clinical presentation, diagnostic assessment, and treatment options of both conditions, in particular in the case associated with diaphragmatic hernia. PMID:27703832

Health-related quality of life and its determinants in children with a congenital diaphragmatic hernia

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2013-01-01

Background The development of new therapeutics has led to progress in the early management of congenital diaphragmatic hernia (CDH) in pediatric intensive care units (PICU). Little is known about the impact on the quality of life (QoL) of children and their family. The aim of this study was to assess the impact of CDH treated according to the most recent concepts and methods outlined above on child survivors’ QoL and their parents’ QoL. Patients and methods This study incorporated a cross-sectional design performed in two PICU (Marseille, France). Families of CDH survivors born between 1999 and 2008 were eligible. The following data were recorded: socio-demographics, antenatal history and delivery, initial hospitalization history. Self-reported data were collected by mail, including current clinical problems of the children (13-symptom list), children’s QoL (Kidscreen-27 questionnaire), and parents’ QoL (Short-Form 36 questionnaire). Children’s QoL score was compared with controls and QoL of survivors of childhood leukemia. Parent’s QoL was compared with controls. Non-parametric statistics were employed. Results Forty-two families agreed to participate and questionnaires were completed by 32 of them. Twenty-one children had a current clinical problems related to CDH. All the QoL scores of CHD survivors were significantly lower compared with controls. The physical well-being dimension was significantly higher for CHD survivors compared with survivors of childhood leukemia. Gastro-esophageal reflux at discharge, antenatal diagnosis, length of stay in the PICU, and neuropsychological and respiratory issues significantly impacted QoL scores of children. The parents of CHD survivors had significantly poorer score in emotional role dimension compared with controls. Conclusion The impact of CDH on QoL seems to be important and must be understood by clinicians who treat these children and their parents. PMID:23786966

BMP4 and LGL1 are Down Regulated in an Ovine Model of Congenital Diaphragmatic Hernia

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Heather eEmmerton-Coughlin

2014-11-01

Full Text Available Background/Purpose: The molecular pathophysiology of lung hypoplasia in congenital diaphragmatic hernia (CDH remains poorly understood. The Wnt signaling pathway and downstream targets, such as bone morphogenetic proteins (BMP 4 and other factors such as late gestation lung protein 1 (LGL1, are essential to normal lung development. Nitrofen-induced hypoplastic CDH rodent lungs demonstrate down regulation of the Wnt pathway including BMP4 and reduced LGL1 expression. The aim of the current study was to examine the molecular pathophysiology associated with a surgically induced CDH in an ovine model. Methods: Left thoracotomy was performed at 80 days in 14 fetal sheep; CDH was created in 7 experimental animals. Lungs were harvested at 136 days (term=145d. Lung weight and mean terminal bronchiole density (MTBD were measured to determine the degree of pulmonary hypoplasia. Quantitative real time PCR was undertaken to analyze Wnt2, Wnt7b, BMP4 and LGL1 mRNA expression. Results: Total lung weight was decreased while MTBD was increased in the CDH group (p<0.05, confirming pulmonary hypoplasia. BMP4 and LGL1 mRNA was significantly reduced in CDH lungs (p<0.05. Wnt2 mRNA was decreased, although not significantly (p<0.06. Conclusions: For the first time, down regulation of BMP4 and Lgl1 are reported in an ovine CDH model. In contrast to other animal models, these changes are persistent to near term. These findings suggest that mechanical compression from herniated viscera may play a more important role in causing pulmonary hypoplasia in CDH, rather than a primary defect in lung organogenesis.

Lung hypoplasia and its associated major congenital abnormalities in perinatal death: an autopsy study of 850 cases.

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Aghabiklooei, A; Goodarzi, P; Kariminejad, Mohammad H

2009-11-01

To determine the relative frequency of causes of lung hypoplasia (LH) and its associated congenital malformations among perinatal deaths. 850 medical reports of perinatal autopsies, in a 25-year period, assessed for LH as a cause of death. LH found in 96 (11.3%) cases, 89 (92.7%) were associated with major congenital malformation (secondary type) and primary type was seen in 7 cases (7.3%). Fourteen cases were associated with multiple congenital anomalies. 32 cases (33.3%) with Genito-urinary anomalies were the most common associated major malformations, followed by 19 cases (19.8%) of diaphragmatic impairment, 15 cases (15.6%) of musculoskeletal abnormalities and 11 cases (11.4%) of kidney agenesis. The most common musculoskletal abnormality was thanatophoric dwarfism in 10 cases (10.4%). Meckle-Gruber syndrome with 7 affected fetuses (7.3%) was the most common malformation syndrome associated with LH. More than ninety percent of LH was secondary to pathology outside the respiratory tract. Renal agenesis is the most common association observed in LH, followed by diaphragmatic hernia and thanatophoric dysplasia.

Blunt traumatic diaphragmatic rupture

Directory of Open Access Journals (Sweden)

Antonio Carlos Nogueira

2011-09-01

Full Text Available Traumatic injury of the diaphragm ranges from 0.6 to 1.2% and rise up to 5%among patients who were victims of blunt trauma and underwent laparotomy.Clinical suspicion associated with radiological assessment contributes to earlydiagnosis. Isolated diaphragmatic injury has a good prognosis. Generallyworse outcomes are associated with other trauma injuries. Bilateral andright diaphragmatic lesions have worse prognosis. Multi detector computed tomography (MDCT scan of the chest and abdomen provides better diagnosticaccuracy using the possibility of image multiplanar reconstruction. Surgicalrepair via laparotomy and/ or thoracotomy in the acute phase of the injury hasa better outcome and avoids chronic complications of diaphragmatic hernia.The authors present the case of a young male patient, victim of blunt abdominaltrauma due to motor vehicle accident with rupture of the diaphragm, spleenand kidney injuries. The diagnosis was made by computed tomography of thethorax and abdomen and was confirmed during laparotomy.

Difficult weaning in delayed onset diaphragmatic hernia

Directory of Open Access Journals (Sweden)

Ahmed Syed

2009-01-01

Full Text Available Diaphragmatic injuries are relatively rare and result from either blunt or penetrating trauma. Regardless of the mechanism, diagnosis is often missed and high index of suspicion is vital. The clinical signs associated with a diaphragmatic hernia can range from no outward signs to immediately life-threatening respiratory compromise. Establishing the clinical diagnosis of diaphragmatic injuries (DI can be challenging as it is often clinically occult. Accurate diagnosis is critical since missed DI may result in grave sequelae due to herniation and strangulation of displaced intra-abdominal organs. We present a case of polytrauma with rib fracture and delayed appearance of diaphragmatic hernia manifesting as difficult weaning from ventilatory support.

Hiatal hernia and diaphragmatic eventration in a leopard (Panthera pardus).

Science.gov (United States)

Kearns, K S; Jones, M P; Bright, R M; Toal, R; DeNovo, R; Orosz, S

2000-09-01

A 1-yr-old male leopard (Panthera pardus) presented for intermittent anorexia, emaciation, and generalized muscle wasting. Plain radiographs, ultrasonography, and esophageal endoscopy led to a diagnosis of diaphragmatic eventration with probable concurrent hiatal hernia. An exploratory laparotomy confirmed both diagnoses, and surgical repair and stabilization were performed. After surgery, the leopard was maintained on small liquid meals for 4 days, with a gradual return to normal diet over 2 wk. By 4 wk after surgery, the leopard was eating well and gaining weight, and it showed no recurrence of clinical signs for 2 yr subsequently, becoming mildly obese.

Combined laparoscopic and open technique for repair of congenital abdominal hernia: A case report of prune belly syndrome.

Science.gov (United States)

Ye, Qinghuang; Chen, Yan; Zhu, Jinhui; Wang, Yuedong

2017-10-01

Prune belly syndrome (PBS) is a rare congenital disorder among adults, and the way for repairing abdominal wall musculature has no unified standard. We described combining laparoscopic and open technique in an adult male who presented with PBS. Physical examination and radiological imaging verified the case of PBS. The deficiency of abdominal wall musculature was repaired by combining laparoscopic and open technique using a double-deck complex patch. The patient successfully underwent abdominal wall repair by combining laparoscopic and open technique. Postoperative recovery was uneventful, and improvement in symptom was significant in follow-up after 3, 6, 12, and 24 months. Combining laparoscopic and open technique for repair of deficiency of abdominal wall musculature in PBS was an exploratory way to improve life quality.

Lung volumes, ventricular function and pulmonary arterial flow in children operated on for left-sided congenital diaphragmatic hernia: long-term results

International Nuclear Information System (INIS)

Abolmaali, Nasreddin; Koch, Arne; Goetzelt, Knut; Vogelberg, Christian; Hahn, Gabriele; Fitze, Guido

2010-01-01

To compare MRI-based functional pulmonary and cardiac measurements in the long-term follow-up of children operated on for left-sided congenital diaphragmatic hernia (CDH) with age- and body size-matched healthy controls. Twelve children who received immediate postnatal surgery for closure of isolated left-sided CDH were included and received basic medical examinations, pulmonary function testing and echocardiography. MRI included measurement of lung volume, ventricular function assessment and velocity-encoded imaging of the pulmonary arteries and was compared with the data for 12 healthy children matched for age and body size. While patients' clinical test results were not suspicious, comparison between the MRI data for patients and those for healthy controls revealed significant differences. In patients, the volumes of the left lungs were increased and the tidal volume was larger on the right side. While the stroke volumes of both ventricles were reduced, heart rate and ejection fraction were increased. Flow, acceleration time and cross-sectional area of the left pulmonary artery were reduced. Functional MRI detected pulmonary and cardiac findings in the late follow-up of CDH children which may be missed by standard clinical methods and might be relevant for decisions regarding late outcome and treatment. (orig.)

Lung volumes, ventricular function and pulmonary arterial flow in children operated on for left-sided congenital diaphragmatic hernia: long-term results

Energy Technology Data Exchange (ETDEWEB)

Abolmaali, Nasreddin; Koch, Arne [Dresden University of Technology, OncoRay - Molecular and Biological Imaging, Medical Faculty Carl Gustav Carus, Dresden (Germany); Goetzelt, Knut; Vogelberg, Christian [University Clinics Carl Gustav Carus, Dresden University of Technology, Clinic and Policlinic for Pediatrics - Pediatric Pulmonology, Dresden (Germany); Hahn, Gabriele [University Clinics Carl Gustav Carus, Dresden University of Technology, Institute and Policlinic for Radiology - Pediatric Radiology, Dresden (Germany); Fitze, Guido [University Clinics Carl Gustav Carus, Dresden University of Technology, Clinic and Policlinic for Pediatric Surgery, Dresden (Germany)

2010-07-15

To compare MRI-based functional pulmonary and cardiac measurements in the long-term follow-up of children operated on for left-sided congenital diaphragmatic hernia (CDH) with age- and body size-matched healthy controls. Twelve children who received immediate postnatal surgery for closure of isolated left-sided CDH were included and received basic medical examinations, pulmonary function testing and echocardiography. MRI included measurement of lung volume, ventricular function assessment and velocity-encoded imaging of the pulmonary arteries and was compared with the data for 12 healthy children matched for age and body size. While patients' clinical test results were not suspicious, comparison between the MRI data for patients and those for healthy controls revealed significant differences. In patients, the volumes of the left lungs were increased and the tidal volume was larger on the right side. While the stroke volumes of both ventricles were reduced, heart rate and ejection fraction were increased. Flow, acceleration time and cross-sectional area of the left pulmonary artery were reduced. Functional MRI detected pulmonary and cardiac findings in the late follow-up of CDH children which may be missed by standard clinical methods and might be relevant for decisions regarding late outcome and treatment. (orig.)

Lung volumes, ventricular function and pulmonary arterial flow in children operated on for left-sided congenital diaphragmatic hernia: long-term results.

Science.gov (United States)

Abolmaali, Nasreddin; Koch, Arne; Götzelt, Knut; Hahn, Gabriele; Fitze, Guido; Vogelberg, Christian

2010-07-01

To compare MRI-based functional pulmonary and cardiac measurements in the long-term follow-up of children operated on for left-sided congenital diaphragmatic hernia (CDH) with age- and body size-matched healthy controls. Twelve children who received immediate postnatal surgery for closure of isolated left-sided CDH were included and received basic medical examinations, pulmonary function testing and echocardiography. MRI included measurement of lung volume, ventricular function assessment and velocity-encoded imaging of the pulmonary arteries and was compared with the data for 12 healthy children matched for age and body size. While patients' clinical test results were not suspicious, comparison between the MRI data for patients and those for healthy controls revealed significant differences. In patients, the volumes of the left lungs were increased and the tidal volume was larger on the right side. While the stroke volumes of both ventricles were reduced, heart rate and ejection fraction were increased. Flow, acceleration time and cross-sectional area of the left pulmonary artery were reduced. Functional MRI detected pulmonary and cardiac findings in the late follow-up of CDH children which may be missed by standard clinical methods and might be relevant for decisions regarding late outcome and treatment.

Author Details

African Journals Online (AJOL)

Kekre, GA. Vol 21, No 4 (2015) - Articles Case report: Thoracoscopic repair of renal ectopia associated with congenital diaphragmatic hernia: Report of two cases. Abstract PDF. ISSN: 1110-5704. AJOL African Journals Online. HOW TO USE AJOL... for Researchers · for Librarians · for Authors · FAQ's · More about AJOL ...

21 CFR 882.5830 - Implanted diaphragmatic/phrenic nerve stimulator.

Science.gov (United States)

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Implanted diaphragmatic/phrenic nerve stimulator... Implanted diaphragmatic/phrenic nerve stimulator. (a) Identification. An implanted diaphragmatic/phrenic nerve stimulator is a device that provides electrical stimulation of a patient's phrenic nerve to...

Evaluation of Neonatal Lung Volume Growth by Pulmonary Magnetic Resonance Imaging in Patients with Congenital Diaphragmatic Hernia.

Science.gov (United States)

Schopper, Melissa A; Walkup, Laura L; Tkach, Jean A; Higano, Nara S; Lim, Foong Yen; Haberman, Beth; Woods, Jason C; Kingma, Paul S

2017-09-01

To evaluate postnatal lung volume in infants with congenital diaphragmatic hernia (CDH) and determine if a compensatory increase in lung volume occurs during the postnatal period. Using a novel pulmonary magnetic resonance imaging method for imaging neonatal lungs, the postnatal lung volumes in infants with CDH were determined and compared with prenatal lung volumes obtained via late gestation magnetic resonance imaging. Infants with left-sided CDH (2 mild, 9 moderate, and 1 severe) were evaluated. The total lung volume increased in all infants, with the contralateral lung increasing faster than the ipsilateral lung (mean ± SD: 4.9 ± 3.0 mL/week vs 3.4 ± 2.1 mL/week, P = .005). In contrast to prenatal studies, the volume of lungs of infants with more severe CDH grew faster than the lungs of infants with more mild CDH (Spearman's ρ=-0.086, P = .01). Although the contralateral lung volume grew faster in both mild and moderate groups, the majority of total lung volume growth in moderate CDH came from increased volume of the ipsilateral lung (42% of total lung volume increase in the moderate group vs 32% of total lung volume increase in the mild group, P = .09). Analysis of multiple clinical variables suggests that increased weight gain was associated with increased compensatory ipsilateral lung volume growth (ρ = 0.57, P = .05). These results suggest a potential for postnatal catch-up growth in infants with pulmonary hypoplasia and suggest that weight gain may increase the volume growth of the more severely affected lung. Copyright © 2017 Elsevier Inc. All rights reserved.

Diaphragmatic Hernia Masquerading as Pleural Effusion

African Journals Online (AJOL)

As many as 30% of diaphragmatic hernias present late.[1] We present an unusual case report of a late presentation of isolated diaphragmatic injury, after apparently minor blunt trauma, which was initially misdiagnosed as pleural effusion. CASE REPORT. A 40-year-old man slipped and fell against the edge of his bath tub.

Use of milrinone to treat cardiac dysfunction in infants with pulmonary hypertension secondary to congenital diaphragmatic hernia: a review of six patients.

Science.gov (United States)

Patel, Neil

2012-01-01

Pulmonary hypertension and secondary cardiac dysfunction are important contributors of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). Milrinone, a phosphodiesterase-3 inhibitor, may be useful in this setting for its combined actions as a pulmonary vasodilator and to improve systolic and diastolic function. This study aimed to assess the effects of milrinone on cardiac function and pulmonary artery pressure in infants with CDH. A retrospective review of echocardiograms performed on infants with CDH who received milrinone was performed. Tissue Doppler imaging velocities were used to assess systolic and diastolic function. Pulmonary artery pressure was assessed from the pattern and velocity of ductal shunting. Six infants with CDH and severe pulmonary hypertension were identified. Systolic and diastolic myocardial velocities were reduced in the right ventricle (RV) and interventricular septum (IVS) at baseline. In the 72 h after commencement of milrinone, there was a significant increase in early diastolic myocardial velocities in the RV, accompanied by increasing systolic velocities in the RV and IVS. Oxygenation index was significantly reduced, blood pressure unchanged, and ductal shunt velocity minimally altered over the same time period. Milrinone use was associated with an improvement in systolic and diastolic function in the RV, corresponding to an improvement in clinical status. Copyright © 2012 S. Karger AG, Basel.

Lung MRI and impairment of diaphragmatic function in Pompe disease

DEFF Research Database (Denmark)

Wens, Stephan C A; Ciet, Pierluigi; Perez-Rovira, Adria

2015-01-01

Background: Pompe disease is a progressive metabolic myopathy. Involvement of respiratory muscles leads to progressive pulmonary dysfunction, particularly in supine position. Diaphragmatic weakness is considered to be the most important component. Standard spirometry is to some extent indicative ...... tool to visualize diaphragmatic dynamics in Pompe patients and to study chest-walland diaphragmatic movements in more detail. Our data indicate that diaphragmatic displacement may be severely disturbed in patients with Pompe disease....

Endometriosis-related spontaneous diaphragmatic rupture.

Science.gov (United States)

Triponez, Frédéric; Alifano, Marco; Bobbio, Antonio; Regnard, Jean-François

2010-10-01

Non-traumatic, spontaneous diaphragmatic rupture is a rare event whose pathophysiology is not known. We report the case of endometriosis-related spontaneous rupture of the right diaphragm with intrathoracic herniation of the liver, gallbladder and colon. We hypothesize that the invasiveness of endometriotic tissue caused diaphragm fragility, which finally lead to its complete rupture without traumatic event. The treatment consisted of a classical management of diaphragmatic rupture, with excision of the endometriotic nodule followed by medical ovarian suppression for six months.

Identifying neonates at a very high risk for mortality among children with congenital diaphragmatic hernia managed with extracorporeal membrane oxygenation.

Science.gov (United States)

Haricharan, Ramanath N; Barnhart, Douglas C; Cheng, Hong; Delzell, Elizabeth

2009-01-01

The purpose of this study was to identify mortality risk factors in children with congenital diaphragmatic hernia (CDH) treated with extracorporeal membrane oxygenation (ECMO) and generate a prediction score for those at a very high risk for mortality. Data on first ECMO runs of all neonates with CDH, between January 1997 and June 2007, were obtained from the Extracorporeal Life Support Organization registry (N = 2678). The data were split into "training data (TD)" (n = 2006) and "validation data" (n = 672). The primary outcome analyzed was in-hospital mortality. Modified Poisson regression was used for analyses. Overall in-hospital mortality among 2678 neonates (males, 57%; median age at ECMO, 1 day) was 52%. The univariate and multivariable analyses were performed using TD. An empirically weighted mortality prediction score was generated with possible scores ranging from 0 to 35 points. Of 69 who scored 14 or higher in the TD, 62 died (positive predictive value [PPV], 90%), of 37 with 15 or higher, 35 died (PPV, 95%), of 23 with 16 or higher, 22 died (PPV, 96%). A cut-off point of 15 was chosen and was tested using the separate validation dataset. In validation data, the cut-off point 15 had a PPV of 96% (23 died of 24). Scoring 15 or higher on the prediction score identifies neonates with CDH at a very high risk for mortality among those managed with ECMO and could be used in surgical decision making and counseling.

Traumatic diaphragmatic injuries: epidemiological, diagnostic and therapeutic aspects.

Science.gov (United States)

Thiam, Ousmane; Konate, Ibrahima; Gueye, Mohamadou Lamine; Toure, Alpha Omar; Seck, Mamadou; Cisse, Mamadou; Diop, Balla; Dirie, Elias Said; Ka, Ousmane; Thiam, Mbaye; Dieng, Madieng; Dia, Abdarahmane; Toure, Cheikh Tidiane

2016-01-01

Diaphragmatic injuries include wounds and diaphragm ruptures, due to a thoracoabdominal blunt or penetrating traumas. Their incidence ranges between 0.8 and 15 %. The diagnosis is often delayed, despite several medical imaging techniques. The surgical management remains controversal, particularly for the choice of the surgical approach and technique. The mortality is mainly related to associated injuries. The aim of our study was to evaluate the incidence of diaphragmatic injuries occuring in thoraco-abdominal traumas, and to discuss their epidemiology, diagnosis and treatment. We performed a retrospective study over a period of 21 years, between January 1994 and June 2015 at the Department of General Surgery of the Aristide Le Dantec hospital in Dakar, Senegal. All patients diagnosed with diaphragmatic injuries were included in the study. Over the study period, 1535 patients had a thoraco-abdominal trauma. There were 859 cases of blunt trauma, and 676 penetrating chest or abdominal trauma. Our study involved 20 cases of diaphragmatic injuries (1.3 %). The sex-ratio was 4. The mean age was 33 years. Brawls represented 83.3 % (17 cases). Stab attacks represented 60 % (12 cases). The incidence of diaphragmatic injury was 2.6 %. The wound was in the thorax in 60 % (seven cases). Chest radiography was contributory in 45 % (nine cases). The diagnosis of wounds or ruptures of the diaphragm was done preoperatively in 45 % (nine cases). The diaphragmatic wound was on the left side in 90 % (18 cases) and its mean size was 4.3 cm. The surgical procedure involved a reduction of herniated viscera and a suture of the diaphragm by "X" non absorbable points in 85 % (17 cases). A thoracic aspiration was performed in all patients. Morbidity rate was 10 % and mortality rate 5 %. The diagnosis of diaphragmatic rupture and wounds remains difficult and often delayed. They should be kept in mind in any blunt or penetrating thoraco-abdominal trauma. Diaphragmatic lesions

Imaging of diaphragmatic rupture after trauma

International Nuclear Information System (INIS)

Eren, S.; Kantarci, M.; Okur, A.

2006-01-01

Traumatic rupture of the diaphragm usually results from blunt or penetrating injuries, or iatrogenic causes. Most cases are initially overlooked in the acute phase because they present with variable clinical and radiological signs. An overlooked diaphragmatic injury presents as a hernia many years later with potentially serious complications, therefore selection of the most appropriate radiological technique and accurate diagnosis of traumatic diaphragmatic hernias (DH) on the first admission is important. Although the diagnosis of diaphragmatic injuries is problematic, various investigations may be used for diagnosis. We describe the imaging findings of 19 traumatic DH cases with various imaging techniques. The patients were acute trauma cases or cases with prior trauma or thoraco-abdominal surgery with clinical suspicion of DH. An evaluation of the imaging techniques used in the diagnosis of DH is presented

Assessment of Pulmonary Artery Stiffness of Repaired Congenital Heart Disease Patients

Science.gov (United States)

Lee, Namheon; Banerjee, Rajit; Taylor, Michael; Hor, Kan

2012-10-01

Surgical correction or palliation of congenital heart disease (CHD) often requires augmenting the main pulmonary artery (MPA) with non-native material or placing a cylindrical graft. The degree to which this intervention affects PA compliance is largely unknown. In this study, the MPA stiffness characteristics were assessed by its compliance, distensibility, and pressure-strain modulus. Coregistered velocity encoded phase-contrast MRI and cardiac catheterization data were available for a cohort of repaired CHD patients (n=8) and controls (n=3). All patients were repaired with either an RV-PA conduit or a RV outflow tract patch. We measured the MPA area change by MRI and MPA pressure during the cath. The measurements were taken through or just distal to the conduit. The MPA compliance and distensibility for the patients were significantly lower than the controls: compliance (9.8±10.8 vs 28.3±7.7mm^2/mmHg, p<0.05), distensibility (2.2±1.5 vs 6.6±2.1%Area change/mmHg, p=0.05). The patients had a significantly higher pressure-strain modulus (152.3±116.4mmHg, p<0.05) than the controls (35.8±10.6mmHg). The abnormally elevated PA stiffness due to the rigidity of the conduit or patch material may cause a compliance mismatch resulting in high stress levels contributing to the observed progressive PA dilatation. This may be a factor in the progressive RV dilatation seen in this cohort of repaired CHD patients.

Prenatal diagnosis of two fetuses with deletions of 8p23.1, critical region for congenital diaphragmatic hernia and heart defects.

Science.gov (United States)

Keitges, Elisabeth A; Pasion, Romela; Burnside, Rachel D; Mason, Carla; Gonzalez-Ruiz, Antonio; Dunn, Teresa; Masiello, Meredith; Gebbia, Joseph A; Fernandez, Carlos O; Risheg, Hiba

2013-07-01

Microdeletions of 8p23.1 are mediated by low copy repeats and can cause congenital diaphragmatic hernia (CDH) and cardiac defects. Within this region, point mutations of the GATA4 gene have been shown to cause cardiac defects. However, the cause of CDH in these deletions has been difficult to determine due to the paucity of mutations that result in CDH, the lack of smaller deletions to refine the region and the reduced penetrance of CDH in these large deletions. Mice deficient for one copy of the Gata4 gene have been described with CDH and heart defects suggesting mutations in Gata4 can cause the phenotype in mice. We report on the SNP microarray analysis on two fetuses with deletions of 8p23.1. The first had CDH and a ventricular septal defect (VSD) on ultrasonography and a family history of a maternal VSD. Microarray analysis detected a 127-kb deletion which included the GATA4 and NEIL2 genes which was inherited from the mother. The second fetus had an incomplete atrioventricular canal defect on ultrasonography. Microarray analysis showed a 315-kb deletion that included seven genes, GATA4, NEIL2, FDFT1, CTSB, DEFB136, DEFB135, and DEFB134. These results suggest that haploinsufficiency of the two genes in common within 8p23.1; GATA4 and NEIL2 can cause CDH and cardiac defects in humans. Copyright © 2013 Wiley Periodicals, Inc.

Budd-chiari syndrome caused by diaphragmatic hernia of the liver: a case report

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Song, Jae Min; Yoon, Jung Won; Kim, Jae Wook; Chung, Woo Kyoung; Chung, Hee Sun; Kim, Joo Hyung; Choi, Jun Ho; Kim, Seung Ho [Armed Forces Capital Hospital, Seongnam (Korea, Republic of)

2007-01-15

Budd-Chiari syndrome is an uncommon disorder, and it is caused by obstruction of the hepatic venous out-flow or inferior vena cava above the hepatic vein. It may result from a large number of conditions, including primary congenital obstructions of the hepatic veins or inferior vena cava by webs or bands. Secondary causes include trauma, polycythemia vera, chronic leukemia, pregnancy, tumors and use of oral contraceptives. No definitive etiologic factors have been identified in two thirds of all cases. We recently experienced a case of Budd-Chiari syndrome caused by diaphragmatic hernia in 21-year-old man. Postoperative follow up CT showed normal venous flow after reintroduction of the liver into the abdominal cavity and closure of the diaphragm defect.

Budd-chiari syndrome caused by diaphragmatic hernia of the liver: a case report

International Nuclear Information System (INIS)

Song, Jae Min; Yoon, Jung Won; Kim, Jae Wook; Chung, Woo Kyoung; Chung, Hee Sun; Kim, Joo Hyung; Choi, Jun Ho; Kim, Seung Ho

2007-01-01

Budd-Chiari syndrome is an uncommon disorder, and it is caused by obstruction of the hepatic venous out-flow or inferior vena cava above the hepatic vein. It may result from a large number of conditions, including primary congenital obstructions of the hepatic veins or inferior vena cava by webs or bands. Secondary causes include trauma, polycythemia vera, chronic leukemia, pregnancy, tumors and use of oral contraceptives. No definitive etiologic factors have been identified in two thirds of all cases. We recently experienced a case of Budd-Chiari syndrome caused by diaphragmatic hernia in 21-year-old man. Postoperative follow up CT showed normal venous flow after reintroduction of the liver into the abdominal cavity and closure of the diaphragm defect

Fetal lung volume in congenital diaphragmatic hernia: association of prenatal MR imaging findings with postnatal chronic lung disease.

Science.gov (United States)

Debus, Angelika; Hagelstein, Claudia; Kilian, A Kristina; Weiss, Christel; Schönberg, Stefan O; Schaible, Thomas; Neff, K Wolfgang; Büsing, Karen A

2013-03-01

To assess whether chronic lung disease (CLD) in surviving infants with congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia on the basis of the results of antenatal observed-to-expected fetal lung volume (FLV) ratio measurement at magnetic resonance (MR) imaging. The study received approval from the institutional review board, with waiver of informed consent for this retrospective review from patients who had previously given informed consent for prospective studies. The ratio of observed to expected FLV at MR imaging was calculated in 172 fetuses with CDH. At postpartum day 28, the need for supplemental oxygen implicated the diagnosis of CLD. At day 56, patients with CLD were assigned to one of three groups-those with mild, moderate, or severe CLD-according to their demand for oxygen. Logistic regression analysis was used to assess the prognostic value of the individual observed-to-expected FLV ratio for association with postnatal development of CLD. Children with CLD were found to have significantly smaller observed-to-expected FLV ratios at MR imaging than infants without CLD (P CLD revealed significant differences in observed-to-expected FLV ratio between patients with mild CLD and those with moderate (P = .012) or severe (P = .007) CLD. For an observed-to-expected FLV ratio of 5%, 99% of patients with CDH developed CLD, compared with less than 5% of fetuses with an observed-to-expected FLV ratio of 50%. Perinatally, development and grade of CLD were further influenced by the need for extracorporeal membrane oxygenation (ECMO) (P CLD in surviving infants with CDH is associated with the prenatally determined observed-to-expected FLV ratio. Early neonatal therapeutic decisions can additionally be based on this ratio. Perinatally, ECMO requirement and gestational age at delivery are useful in further improving the estimated probability of CLD.

Contemporary results of aortic valve repair for congenital disease: lessons for management and staged strategy.

Science.gov (United States)

Vergnat, Mathieu; Asfour, Boulos; Arenz, Claudia; Suchowerskyj, Philipp; Bierbach, Benjamin; Schindler, Ehrenfried; Schneider, Martin; Hraska, Victor

2017-09-01

Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease. From 2003 to 2015, 193 consecutive patients were included. The mean age was 9.2 ± 6.9 years (22% disease. The procedures performed were commissurotomy shaving (n = 74; 38%), leaflet replacement (n = 78; 40%), leaflet extension (n = 21; 11%) and neocommissure creation (n = 21; 11%). Post-repair geometry was tricuspid in 137 (71%) patients. The 10-year survival rate was 97.1%. Freedom from reoperation and replacement at 7 years was, respectively, 57% (95% confidence interval, 47-66) and 68% (95% confidence interval, 59-76). In multivariate analysis, balloon dilatation before 6 months, the absence of a developed commissure, a non-tricuspid post-repair geometry and cross-clamp duration were predictors for reoperation and replacement. After a mean follow-up period of 5.1 ± 3.0 years, 145 (75%) patients had a preserved native valve, with undisturbed valve function (peak gradient <40 mmHg, regurgitation ≤mild) in 113 (58%). Aortic valve repair in children is safe and effective in delaying the timing for more definitive solution. Surgical strategy should be individualized according to the age of the patient. Avoidance of early balloon dilatation and aiming for a tricuspid post-repair arrangement may improve outcomes. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Decreased Rac1 Cardiac Expression in Nitrofen-Induced Diaphragmatic Hernia.

Science.gov (United States)

Nakamura, Hiroki; Zimmer, Julia; Puri, Prem

2018-02-01

 The high incidence of cardiac malformations in humans and animal models with congenital diaphragmatic hernia (CDH) is well known. The hypoplasia of left heart is common among fetuses with CDH and has been identified as a poor prognostic factor. However, the precise mechanisms underlying cardiac maldevelopment in CDH are not fully understood. Ras-related C3 botulinum toxin substrate 1 (Rac1) plays a key role in cardiomyocyte polarity and embryonic heart development. Deficiency of Rac1 is reported to impair elongation and cytoskeletal organization of cardiomyocytes, resulting in congenital cardiac defects. We designed this study to test the hypothesis that Rac1 expression is downregulated in the developing hearts of rats with nitrofen-induced CDH.  Following ethical approval (REC1103), time-pregnant Sprague Dawley rats received nitrofen or vehicle on gestational day 9 (D9). Fetuses were sacrificed on D18 and D21 and divided into CDH and control (CTRL) ( n  = 6 for each group and time point). Quantitative real-time polymerase chain reaction (qRT-PCR), Western blotting, and confocal-immunofluorescence microscopy were performed to detect cardiac gene and protein expression of Rac1.  qRT-PCR and Western blot analysis revealed that Rac1 expression was significantly decreased in the CDH group compared with controls ( p  Rac1 cardiac expression was markedly decreased in the CDH group compared with controls.  Decreased cardiac Rac1 expression in the nitrofen-induced CDH suggests that Rac1 deficiency during morphogenesis may impair structural cardiac remodeling, resulting in congenital cardiac defects. Georg Thieme Verlag KG Stuttgart · New York.

Fractured Ribs and the CT Funky Fat Sign of Diaphragmatic Rupture

Directory of Open Access Journals (Sweden)

Iclal Ocak

2016-01-01

Full Text Available Traumatic diaphragmatic rupture remains a diagnostic challenge for both radiologists and surgeons. In recent years, multidetector CT has markedly improved the diagnosis of diaphragmatic injury in polytrauma patients. Herein, we describe two cases of subacute presentation of traumatic diaphragmatic rupture from a penetrating rib fracture and subsequent intrathoracic herniation of omental fat, representing the CT “funky fat” sign.

Diaphragm Repair with a Novel Cross-Linked Collagen Biomaterial in a Growing Rabbit Model.

Directory of Open Access Journals (Sweden)

Steffi Mayer

Full Text Available Neonates with congenital diaphragmatic hernia and large defects often require patch closure. Acellular collagen matrices (ACM have been suggested as an alternative to synthetic durable patches as they are remodeled by the host or could also be used for tissue engineering purposes.2.0x1.0 cm diaphragmatic defects were created in 6-weeks old New-Zealand white rabbits. We compared reconstruction with a purpose-designed cross-linked ACM (Matricel to 4-layer non-cross-linked small intestinal submucosa (SIS and a 1-layer synthetic Dual Mesh (Gore-Tex. Unoperated animals or animals undergoing primary closure (4/0 polyglecaprone served as age-matched controls. 60 (n = 25 resp. 90 (n = 17 days later, animals underwent chest x-ray and obduction for gross examination of explants, scoring of adhesion and inflammatory response. Also, uniaxial tensiometry was done, comparing explants to contralateral native diaphragmatic tissue.Overall weight nearly doubled from 1,554±242 g at surgery to 2,837±265 g at obduction (+84%. X-rays did show rare elevation of the left diaphragm (SIS = 1, Gore-Tex = 1, unoperated control = 1, but no herniation of abdominal organs. 56% of SIS and 10% of Matricel patches degraded with visceral bulging in four (SIS = 3, Matricel = 1. Adhesion scores were limited: 0.5 (Matricel to 1 (SIS, Gore-Tex to the left lung (p = 0.008 and 2.5 (Gore-Tex, 3 (SIS and 4 (Matricel to the liver (p<0.0001. Tensiometry revealed a reduced bursting strength but normal compliance for SIS. Compliance was reduced in Matricel and Gore-Tex (p<0.01. Inflammatory response was characterized by a more polymorphonuclear cell (SIS resp. macrophage (Matricel type of infiltrate (p<0.05. Fibrosis was similar for all groups, except there was less mature collagen deposited to Gore-Tex implants (p<0.05.Matricel induced a macrophage-dominated inflammatory response, more adhesions, had appropriate strength but a lesser compliance compared to native tissue. The herein

Mesentro-axial gastric volvulus in a morgagni diaphragmatic hernia in an old female

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Fansur, M.; Atiq, S.

2011-01-01

A Morgagni diaphragmatic hernia is a rare congenital anteromedial defect in adults (5%). Symptoms of visceral herniation are attributable to the organs involved. Imaging is the mainstay of diagnosis either in an asymptomatic person or in a person with respiratory and/or gastrointestinal symptoms, ultimately requiring surgical intervention because of the risk of incarceration. We present a rare case of 80 years old female with vague upper abdominal pain and recurrent vomiting. An anteromedial parasternal defect was established on conventional as well as on cross-sectional imaging in right hemi diaphragm through which the upper abdominal contents were protruding in the right hemi thorax, all enclosed in a peritoneal sac. The herniation resulted in mesentro-axial gastric volvulus. Due to age and anaesthesia risk, patient was conservatively managed. (author)

Drainage of pleural effusion improves diaphragmatic function in mechanically ventilated patients.

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Umbrello, Michele; Mistraletti, Giovanni; Galimberti, Andrea; Piva, Ilaria R; Cozzi, Ottavia; Formenti, Paolo

2017-03-01

Pleural effusion adversely affects the pressuregenerating capacity of the diaphragm. It uncouples the lung and chest wall, which may result in diaphragmatic dysfunction. Information on the effects of effusion drainage on diaphragmatic function is limited, but several studies report relief of dyspnoea after drainage, which was attributed to improved diaphragmatic mechanics, even if this issue was never formally addressed. To investigate the effect of drainage of unilateral pleural effusion on diaphragmatic function. In a prospective twostep protocol (at baseline and after drainage of effusion), we conducted a spontaneous breathing trial in fourteen critically ill, mechanically ventilated patients undergoing pressure support ventilation. We used ultrasonography of the ipsilateral hemidiaphragm to evaluate and record respiratory displacement and thickening during tidal and maximal breathing efforts. We recorded and analysed airway pressures, respiratory system compliance, vital capacity, indices of respiratory effort and arterial blood gases. After drainage of the effusion, the respiratory rate decreased and tidal volume increased, but haemodynamic parameters were unaffected and oxygenation levels showed a non-significant increase. Drainage was associated with significant decreases in indices of respiratory drive and the maximal pressure generated by the respiratory muscles, as well as an increased compliance of the respiratory system. Diaphragmatic displacement and thickening significantly increased after drainage. We found there was a significant correlation between the volume of the effusion drained and the increase in tidal diaphragmatic thickening. Drainage of a unilateral pleural effusion during weaning from mechanical ventilation improves diaphragmatic contractile activity and respiratory system performance.

The effect of anatomic differences on the relationship between renal artery and diaphragmatic crus.

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Esen, K; Tok, S; Balci, Yuksel; Apaydin, F D; Kara, E; Uzmansel, D

2018-01-01

The aim of this study is to investigate the effect of anatomic differences on the relationship between renal artery and diaphragmatic crus via the touch of two structures. The study included dynamic computed tomography (CT) scans of 308 patients performed mainly for characterisation of liver and renal masses. Anatomic differences including the thickness of the diaphragmatic crus, the localisation of renal artery ostium at the wall of aorta, the level of renal artery origin with respect to superior mesenteric artery were evaluated. Statistical relationships between renal artery-diaphragmatic crus contact and the anatomic differences were assessed. Thickness of the diaphragmatic crus at the level of renal artery origin exhibited a statistically significant relationship to renal artery-diaphragmatic crus contact at the left (p renal artery origin and renal artery- -diaphragmatic crus contact at the left (p renal artery ostium at the wall of aorta (right side, p = 0.436, left side, p = 0.681) did not demonstrate a relationship to renal artery-diaphragmatic crus contact. Thickness of the diaphragmatic crus and high renal artery origin with respect to superior mesenteric artery are crucial anatomic differences determining the relationship of renal artery and diaphragmatic crus. (Folia Morphol 2018; 77, 1: 22-28).

Health in adults with congenital heart disease

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Cuypers, Judith A. A. E.; Utens, Elisabeth M. W. J.; Roos-Hesselink, Jolien W.

2016-01-01

Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many

Paper-thinned diaphragm: CT sign of diaphragmatic eventration

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Im, Jung Gi; Han, Man Chung; Kim, Chu Wan; Shim, Young Soo

1990-01-01

CT findings of total left hemidiaphragmatic eventration in two adult patients are described. Chest radiograph showed elevated left hemidiaphragm and left hilum. Paper-thinned muscular portion of the diseased diaphragm was clearly demonstrated with CT scan. With demonstration of paper-thinned diaphragm by CT scan, differentiation of the diaphragmatic eventration from recent onset phrenic nerve paralysis by tumor invasion or from post-traumatic diaphragmatic hernia can be made

Paper-thinned diaphragm: CT sign of diaphragmatic eventration

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Im, Jung Gi; Han, Man Chung; Kim, Chu Wan; Shim, Young Soo [College of Medicine, Seoul National Univ., Seoul (Korea, Republic of)

1990-10-15

CT findings of total left hemidiaphragmatic eventration in two adult patients are described. Chest radiograph showed elevated left hemidiaphragm and left hilum. Paper-thinned muscular portion of the diseased diaphragm was clearly demonstrated with CT scan. With demonstration of paper-thinned diaphragm by CT scan, differentiation of the diaphragmatic eventration from recent onset phrenic nerve paralysis by tumor invasion or from post-traumatic diaphragmatic hernia can be made.

Warfarin-Associated Diaphragmatic Hernia: An Unusual Diagnosis

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Cristina Vilhena

2015-01-01

Full Text Available Fetal warfarin syndrome is a consequence of maternal intake of warfarin during pregnancy and comprises a wide range of manifestations, including some typical facial dysmorphologic features. The authors report a case of prenatal ultrasonographic diagnosis of warfarin embryopathy in an obese woman on unsupervised warfarin prophylaxis at the 16th week of gestation. The fetus presented with facial dysmorphism, pectus excavatum, diaphragmatic hernia, and pulmonary hypoplasia. To the best of our knowledge, this is the second reported case of warfarin-associated diaphragmatic hernia.

Comparison of Radiography and Ultrasonography for Diagnosis of Diaphragmatic Hernia in Bovines

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Hakim Athar

2010-01-01

Full Text Available The present study was conducted on 101 animals suffering from thoracoabdominal disorders; out of which twenty seven animals (twenty six buffaloes and one cow were diagnosed with diaphragmatic hernia based on clinical signs, radiography, ultrasonography, and left flank laparorumenotomy. Radiography alone confirmed diaphragmatic hernia in 18 cases (66.67% with a sac-like structure cranial to the diaphragm. In 15 animals the sac contained metallic densities while in three cases a sac-like structure with no metallic densities was present. Ultrasonography was helpful in confirming diaphragmatic hernia in 23 cases (85.18% and ultrasonographically reticular motility was evident at the level of 4th/5th intercostal space in all the animals. B+M mode ultrasonography was used for the first time for diagnosis of diaphragmatic hernia in bovines and the results suggested that ultrasonography was a reliable diagnostic modality for diaphragmatic hernia in bovines.

Straight line closure of congenital macrostomia

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Schwarz Richard

2004-01-01

Full Text Available The results of patients operated on by Nepal Cleft Lip and Palate Association (NECLAPA surgeons for congenital macrostomia were prospectively studied between January 2000 and December 2002. There were four males and three females with a median age of 10 years. Three had an associated branchial arch syndrome. In all patients an overlapping repair of orbicularis oris was done. Six patients had a straight line closure with excellent cosmetic results and one a Z-plasty with a more obvious scar. All had a normal appearing commissure. Overlapping orbicularis repair with straight line skin closure for this rare congenital anomaly is recommended.

Peritoneopericardial diaphragmatic hernia: a retrospective study of 31 cats and eight dogs.

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Banz, Angela C; Gottfried, Sharon D

2010-01-01

The records of 31 cats and eight dogs undergoing surgical correction of peritoneopericardial diaphragmatic hernia (PPDH) from 2000 through 2007 were reviewed. Weimaraners and long-haired cats of varying breeds, particularly Maine Coon cats, appear to be at higher risk of PPDH. Presenting complaints were most commonly related to the respiratory and gastrointestinal tracts in both dogs and cats, although respiratory signs were more prevalent in cats, and gastrointestinal signs were more common in dogs. The most common herniated organs were liver, gallbladder, and small intestine. Mortality associated with surgical repair of PPDH in cats and dogs was low in the first 2 weeks postoperatively, and prognosis for return to normal function was excellent. Peri-and postoperative complications were typically minor and self-limiting.

Functional restoration of diaphragmatic paralysis: an evaluation of phrenic nerve reconstruction.

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Kaufman, Matthew R; Elkwood, Andrew I; Colicchio, Alan R; CeCe, John; Jarrahy, Reza; Willekes, Lourens J; Rose, Michael I; Brown, David

2014-01-01

Unilateral diaphragmatic paralysis causes respiratory deficits and can occur after iatrogenic or traumatic phrenic nerve injury in the neck or chest. Patients are evaluated using spirometry and imaging studies; however, phrenic nerve conduction studies and electromyography are not widely available or considered; thus, the degree of dysfunction is often unknown. Treatment has been limited to diaphragmatic plication. Phrenic nerve operations to restore diaphragmatic function may broaden therapeutic options. An interventional study of 92 patients with symptomatic diaphragmatic paralysis assigned 68 (based on their clinical condition) to phrenic nerve surgical intervention (PS), 24 to nonsurgical (NS) care, and evaluated a third group of 68 patients (derived from literature review) treated with diaphragmatic plication (DP). Variables for assessment included spirometry, the Short-Form 36-Item survey, electrodiagnostics, and complications. In the PS group, there was an average 13% improvement in forced expiratory volume in 1 second (p Phrenic nerve operations for functional restoration of the paralyzed diaphragm should be part of the standard treatment algorithm in the management of symptomatic patients with this condition. Assessment of neuromuscular dysfunction can aid in determining the most effective therapy. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Traumatic diaphragmatic hernia in a 5-month-old boxer dog.

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Hoddinott, Katie

2013-05-01

A 5-month-old intact male boxer dog was presented to the Metro Animal Emergency Clinic, Dartmouth, Nova Scotia after being hit by a car. Radiography identified a diaphragmatic hernia with the stomach herniated into the thoracic cavity. Diaphragmatic herniorrhaphy and splenectomy were performed without complication. The patient returned to his regular active lifestyle.

Congenital spinal malformations

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

2001-01-01

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

Congenital Palatal Fistula Associated with Submucous Cleft Palate

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Eshete, Mekonen; Camison, Liliana; Abate, Fikre; Hailu, Taye; Demissie, Yohannes; Mohammed, Ibrahim; Butali, Azeez; Losken, H. Wolfgang

2016-01-01

Background: Although cleft lip and cleft palate are among the most common congenital malformations, the presence of an isolated congenital palatal fistula along with a submucous cleft is very rare. This appears as an oval-shaped, full-thickness fenestration in the palatal midline that does not fully extend anteriorly or posteriorly, accompanied by the findings of a submucous cleft. Because of the uncommon nature of this entity, there is controversy about its etiology, diagnosis, and management. Methods: Two cases of children with congenital palatal fistulae and a submucous cleft palate are presented who were treated in different settings by different surgeons. Cases are discussed along with a thorough review of the available literature. Results: Patient 1 presented at 4 years of age with “a hole in the palate” since birth and abnormal speech. His palatal fistula and submucous cleft were repaired with a modified von Langenbeck technique in Ethiopia. At a 2-year follow-up, the palate remained closed, but hypernasal speech persisted. Patient 2 was a 1-year-old presenting with failure to thrive and nasal regurgitation, who underwent a Furlow palatoplasty in the United States with good immediate results. She was unfortunately lost to follow-up. Conclusions: A congenital fenestration of the palate is rare. Reports reveal suboptimal speech at follow-up, despite various types of repair, especially when combined with a submucous cleft. Available literature suggests that repair should not focus on fistula closure only but instead on providing adequate palate length to provide good velopharyngeal function, as in any cleft palate repair. PMID:27014542

Health in adults with congenital heart disease.

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Cuypers, Judith A A E; Utens, Elisabeth M W J; Roos-Hesselink, Jolien W

2016-09-01

Since the introduction of cardiac surgery, the prospects for children born with a cardiac defect have improved spectacularly. Many reach adulthood and the population of adults with congenital heart disease is increasing and ageing. However, repair of congenital heart disease does not mean cure. Many adults with congenital heart disease encounter late complications. Late morbidity can be related to the congenital heart defect itself, but may also be the consequence of the surgical or medical treatment or longstanding alterations in hemodynamics, neurodevelopment and psychosocial development. This narrative review describes the cardiac and non-cardiac long-term morbidity in the adult population with congenital heart disease. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Repair of Postoperative Abdominal Hernia in a Child with Congenital Omphalocele Using Porcine Dermal Matrix

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V. Lambropoulos

2016-01-01

Full Text Available Introduction. Incisional hernias are a common complication appearing after abdominal wall defects reconstruction, with omphalocele and gastroschisis being the most common etiologies in children. Abdominal closure of these defects represents a real challenge for pediatric surgeons with many surgical techniques and various prosthetic materials being used for this purpose. Case Report. We present a case of repair of a postoperative ventral hernia occurring after congenital omphalocele reconstruction in a three-and-a-half-year-old child using an acellular, sterile, porcine dermal mesh. Conclusion. Non-cross-linked acellular porcine dermal matrix is an appropriate mesh used for the reconstruction of abdominal wall defects and their postoperative complications like large ventral hernias with success and preventing their recurrence.

Surgical repair of a congenital sternal cleft in a cat.

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Schwarzkopf, Ilona; Bavegems, Valerie C A; Vandekerckhove, Peter M F P; Melis, Sanne M; Cornillie, Pieter; de Rooster, Hilde

2014-07-01

To describe the clinical findings, diagnosis, and treatment of an incomplete cleft of the 5th-8th sternebra and a cranioventral abdominal wall hernia in a 2 month old Ragdoll kitten and to evaluate the short- and long-term outcome. Clinical report. Ragdoll cat (n = 1), 2 months old. Sternal cleft was confirmed by thoracic radiographs. Computed tomography (CT) was used to plan an optimal surgical approach. A ventral median incision was made, starting at the 3rd sternebra and extended into the abdomen. Ostectomy of the proximal part of the 5th left sternebra was performed. Lateral periosteal flaps were created, unfolded, and absorbable monofilament sutures preplaced to facilitate closure and the repair was reinforced by 2 peristernal sutures. A bone graft was applied, and the free margin of the omentum was sutured to the cranial aspect of the wound. No major complications occurred. At 3 weeks, CT scan confirmed approximation of the hemisternebrae and at 10 months, complete fusion of the hemisternebrae had not occurred, but a strong connection of the sternal bars was present. Sternal cleft is a rare congenital abnormality that can be corrected surgically with favorable outcome. © Copyright 2014 by The American College of Veterinary Surgeons.

Surgical and nonsurgical treatment of peritoneopericardial diaphragmatic hernia in dogs and cats: 58 cases (1999-2008).

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Burns, Colby G; Bergh, Mary Sarah; McLoughlin, Mary A

2013-03-01

To determine clinical findings and outcomes for cats and dogs with peritoneopericardial diaphragmatic hernia (PPDH) treated surgically or nonsurgically. Retrospective case series. 28 dogs and 30 cats. Medical records for cats and dogs evaluated at 1 of 2 veterinary teaching hospitals were reviewed, and data regarding clinical signs, diagnostic and surgical findings, and outcome were evaluated. Prevalence of PPDH in the 2 hospitals during the study period was 0.025% (0.062% and 0.015% for cats and dogs, respectively); PPDH was an incidental finding for 13 (46.4%) dogs and 15 (50.0%) cats. Other congenital abnormalities were identified in 16 (571%) dogs and 7 (23.3%) cats (most commonly umbilical hernias, abdominal wall hernias cranial to the umbilicus, or sternal anomalies). Thirty-four (58.6%) animals underwent surgical repair of PPDH; 27 (79.4%) of these animals had a primary diagnosis of PPDH. Detection of clinical signs of PPDH (primary diagnosis) and intestines in the pericardial sac were significantly associated with surgical treatment. Short-term mortality rate for surgically treated animals was 8.8% (3/34). Clinical signs associated with PPDH resolved in 29 (85.3%) of surgically treated animals. No significant differences were detected between dogs and cats or between surgically and nonsurgically treated animals regarding long-term survival rate. Results of this study indicated animals with clinical signs of PPDH were more likely to undergo surgery than were animals without such signs. Herniorrhaphy was typically effective for resolution of clinical signs. Long-term survival rates were similar regardless of treatment method. Surgical or nonsurgical treatment of PPDH may be appropriate for animals with or without clinical signs, respectively.

Diaphragmatic thickness ratio (inspiratory/expiratory) as a diagnostic method of diaphragmatic palsy associated with interescalene block.

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López Escárraga, V M; Dubos España, K; Castillo Bustos, R H; Peidró, L; Sastre, S; Sala-Blanch, X

2018-02-01

Diaphragmatic paralysis is a side-effect associated with interscalene block. Thickness index of the diaphragm muscle (inspiratory thickness/expiratory thickness) obtained by ultrasound has recently been introduced in clinical practice for diagnosis of diaphragm muscle atrophy. Our objective was to evaluate this index for the diagnosis of acute phrenic paresis associated with interscalene block. We designed an observational study in 22 patients scheduled for shoulder arthroscopy. Spirometry was performed (criteria of phrenic paresis was a decrease in FVC and FEV1 ≥20%). Ultrasound apposition zone was assessed in anterior axillary line and diaphragmatic displacement was evaluated on inspiration and expiration (number of intercostal spaces; phrenic paresis considered a reduction ≥25%) and thickness of the diaphragm muscle (a phrenic paresis was considered an index block at C5-C6 with 20ml of 0.5% ropivacaine. Twenty-one patients (95%) presented phrenic nerve block according to one or more of the methods used. One patient did not show any symptoms or signs suggestive of phrenic paralysis and was excluded. All the patients presented phrenic paresis based on the diaphragmatic thickness index, with the pre-block index being 1.8±0.5 and post-block of 1.05±0.06 (Pblock (from 1.9±0.5 intercostal spaces to 0.5±0.3; Pblock. This index does not require a baseline pre-assessment. Copyright © 2017 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

Isolated Crural Hematoma Mimicking Retroperitoneal Lymphadenopathy, A Unique Sign of Traumatic Diaphragmatic Rupture: A Case Report

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Abhishek Jha

2014-09-01

Full Text Available Diaphragmatic injury following blunt thoracoabdominal trauma is rare and is usually associated with key radiological features like dependent viscera sign, collar sign, diaphragmatic thickening and defects. It may also be associated with secondary signs like intrathoracic herniation of abdominal viscera. Diaphragmatic crura, which are attached to the upper lumbar vertebra represent prominently thickened folds along the posterior diaphragm, are usually inconspicuous on routine Computed Tomography (CT scans. We present a case of a young patient who sustained a motor vehicle accident and developed difficulty in breathing. CT scan of the patient revealed bilateral crural hematomas, with splenic and renal lacerations and no other sign of diaphragmatic injury. The patient was operated and blunt diaphragmatic rupture was confirmed at surgery.

Medical image of the week: Bochdalek hernia

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Omar M

2016-06-01

Full Text Available No abstract available. Article truncated at 150 words. A 61 year-old man presented for an evaluation of a nonproductive cough. He has a history of well-controlled asthma, allergic rhinitis and nasal polyposis, hypertension, gastro-esophageal reflux and obstructive sleep apnea. The ACE inhibitor used to treat hypertension was discontinued. The physical exam was unremarkable. Pulmonary function testing was normal. A PA and lateral chest radiograph was performed and revealed an abnormal contour of the left hemidiaphragm with a large lobulated opacity (Figure 1- blue arrows. Computed chest tomography revealed the lobulated opacity in the left lower lobe contained fat and was consistent with a Bochdalek hernia (Figure 2. Congenital diaphragmatic hernia is a major malformation in newborns and in the perinatal period. The diagnosis of congenital diaphragmatic hernia in adults is rare. There are three types of congenital diaphragmatic hernias: posterolateral (Bochdalek diaphragmatic hernia, subcostosternal (Morgagni hernia and esophageal hiatal hernia. The Bochdalek diaphragmatic hernia is the result of ...

Morgagni's Hernia

International Nuclear Information System (INIS)

Khalid, M.M.

2004-01-01

Congenital diaphragmatic hernias, including Morgagni's hernia, usually present in early childhood and are treated by surgical repair. This case report is about an unusual Morgagni's hernia, presenting with dyspepsia and chest pain, at the age 45 years. For many years the diagnosis remained a dilemma because patient's chest x-ray was not done and she was treated for 'ngina' and 'dyspepsia' Diagnosis was obvious once a chest x-ray was done, however, barium studies were performed for further confirmation. (author)

The Effect of Diaphragmatic Breathing on Attention, Negative Affect and Stress in Healthy Adults

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Xiao Ma

2017-06-01

Full Text Available A growing number of empirical studies have revealed that diaphragmatic breathing may trigger body relaxation responses and benefit both physical and mental health. However, the specific benefits of diaphragmatic breathing on mental health remain largely unknown. The present study aimed to investigate the effect of diaphragmatic breathing on cognition, affect, and cortisol responses to stress. Forty participants were randomly assigned to either a breathing intervention group (BIG or a control group (CG. The BIG received intensive training for 20 sessions, implemented over 8 weeks, employing a real-time feedback device, and an average respiratory rate of 4 breaths/min, while the CG did not receive this treatment. All participants completed pre- and post-tests of sustained attention and affect. Additionally, pre-test and post-test salivary cortisol concentrations were determined in both groups. The findings suggested that the BIG showed a significant decrease in negative affect after intervention, compared to baseline. In the diaphragmatic breathing condition, there was a significant interaction effect of group by time on sustained attention, whereby the BIG showed significantly increased sustained attention after training, compared to baseline. There was a significant interaction effect of group and time in the diaphragmatic breathing condition on cortisol levels, whereby the BIG had a significantly lower cortisol level after training, while the CG showed no significant change in cortisol levels. In conclusion, diaphragmatic breathing could improve sustained attention, affect, and cortisol levels. This study provided evidence demonstrating the effect of diaphragmatic breathing, a mind-body practice, on mental function, from a health psychology approach, which has important implications for health promotion in healthy individuals.

The Effect of Diaphragmatic Breathing on Attention, Negative Affect and Stress in Healthy Adults.

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Ma, Xiao; Yue, Zi-Qi; Gong, Zhu-Qing; Zhang, Hong; Duan, Nai-Yue; Shi, Yu-Tong; Wei, Gao-Xia; Li, You-Fa

2017-01-01

A growing number of empirical studies have revealed that diaphragmatic breathing may trigger body relaxation responses and benefit both physical and mental health. However, the specific benefits of diaphragmatic breathing on mental health remain largely unknown. The present study aimed to investigate the effect of diaphragmatic breathing on cognition, affect, and cortisol responses to stress. Forty participants were randomly assigned to either a breathing intervention group (BIG) or a control group (CG). The BIG received intensive training for 20 sessions, implemented over 8 weeks, employing a real-time feedback device, and an average respiratory rate of 4 breaths/min, while the CG did not receive this treatment. All participants completed pre- and post-tests of sustained attention and affect. Additionally, pre-test and post-test salivary cortisol concentrations were determined in both groups. The findings suggested that the BIG showed a significant decrease in negative affect after intervention, compared to baseline. In the diaphragmatic breathing condition, there was a significant interaction effect of group by time on sustained attention, whereby the BIG showed significantly increased sustained attention after training, compared to baseline. There was a significant interaction effect of group and time in the diaphragmatic breathing condition on cortisol levels, whereby the BIG had a significantly lower cortisol level after training, while the CG showed no significant change in cortisol levels. In conclusion, diaphragmatic breathing could improve sustained attention, affect, and cortisol levels. This study provided evidence demonstrating the effect of diaphragmatic breathing, a mind-body practice, on mental function, from a health psychology approach, which has important implications for health promotion in healthy individuals.

Identifying decreased diaphragmatic mobility and diaphragm thickening in interstitial lung disease: the utility of ultrasound imaging

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Santana, Pauliane Vieira; Prina, Elena; Albuquerque, André Luis Pereira; Carvalho, Carlos Roberto Ribeiro; Caruso, Pedro

2016-01-01

Objective: To investigate the applicability of ultrasound imaging of the diaphragm in interstitial lung disease (ILD). Methods: Using ultrasound, we compared ILD patients and healthy volunteers (controls) in terms of diaphragmatic mobility during quiet and deep breathing; diaphragm thickness at functional residual capacity (FRC) and at total lung capacity (TLC); and the thickening fraction (TF, proportional diaphragm thickening from FRC to TLC). We also evaluated correlations between diaphragmatic dysfunction and lung function variables. Results: Between the ILD patients (n = 40) and the controls (n = 16), mean diaphragmatic mobility was comparable during quiet breathing, although it was significantly lower in the patients during deep breathing (4.5 ± 1.7 cm vs. 7.6 ± 1.4 cm; p < 0.01). The patients showed greater diaphragm thickness at FRC (p = 0.05), although, due to lower diaphragm thickness at TLC, they also showed a lower TF (p < 0.01). The FVC as a percentage of the predicted value (FVC%) correlated with diaphragmatic mobility (r = 0.73; p < 0.01), and an FVC% cut-off value of < 60% presented high sensitivity (92%) and specificity (81%) for indentifying decreased diaphragmatic mobility. Conclusions: Using ultrasound, we were able to show that diaphragmatic mobility and the TF were lower in ILD patients than in healthy controls, despite the greater diaphragm thickness at FRC in the former. Diaphragmatic mobility correlated with ILD functional severity, and an FVC% cut-off value of < 60% was found to be highly accurate for indentifying diaphragmatic dysfunction on ultrasound. PMID:27167428

Diaphragmatic excursion: does it predict successful weaning from mechanical ventilation?

International Nuclear Information System (INIS)

Hayat, A.; Khalil, A.

2017-01-01

To measure the diaphragmatic excursion and its outcome on weani ng from mechanical ventilation. Study Design: Cross-sectional comparative study. Place and Duration of Study: Medical Intensive Care Unit (ICU), Military Hospital (MH), Rawalpindi, Pakistan, from January to December 2014. Methodology: Diaphragmatic excursion (DE) in cm was measured through ultrasound by marking liver and spleen displacement in patients who fulfilled the criteria of removal from ventilatory support. The patients were followed up for 48 hours and classified according to the outcome as successful weaning and weaning failure. Results: Out of 100 cases, 76 patients had a successful weaning while 24 had a failed weaning outcome. At a diaphragmatic excursion of 1.2 cm and more, out of 67 cases, 60 had a successful weaning (89.55%) while 7 cases (10.45%) had a weaning failure. At an excursion of less than 1. 2 cm, 17 out of 33 cases (51.5%) had successful weaning while 16 (48.48%) had weaning failure. At this cut off point (1.2 cm), the sensitivity and specificity for successful weaning were 78.95% and 70.83%, respectively. The positive and negative likelihood ratio (LR) for these values being 2.70 and 0.29, respectively. The positive predictive value was 82.35% and negative predictive value 60.00%. Conclusion: Ultrasonographic measurement of diaphragmatic excursion is a good method for predicting weaning outcome from mechanical ventilation. (author)

Retalho pediculado do músculo grande dorsal para reparo de defeito diafragmático experimental em cães Latissimus dorsi muscular flap for repair of experimental diaphragmatic defect in dogs

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Luciana Oliveira de Oliveira

2000-12-01

Full Text Available Neste experimento, foi utilizado um retalho do músculo grande dorsal, com pedículo dorso-caudal, para o reparo de defeitos diafragmáticos iatrogênicos em 18 cães adultos. O acesso ao diafragma foi através de toracotomia no décimo espaço intercostal, sendo criado um defeito diafragmático, onde o retalho foi suturado. O retalho muscular pediculado cobriu um terço do hemidiafragma direito. Os animais tiveram boa recuperação pós-cirúrgica, sem sinais de comprometimento respiratório. Foram feitas necropsias aos 15, 30 e 60 dias do pós -operatório. O local submetido ao implante apresentou algumas aderências com vísceras adjacentes e consolidação da sutura macro e microscopicamente. Conclui-se que o retalho pediculado do grande dorsal pode ser útil para o reparo de grandes defeitos diafragmáticos em cães.In this study, the Latissimus dorsi muscular flap was experimentally used for the repair of iatrogenic diaphragmatic defects in 18 adult dogs. The flap was elevated maintaining its dorso-caudal attachment. The diaphragm was accessed through a right thoracotomy in the tenth intercostal space, a defect in the diaphragm was created and the flap was sutured in the margins of the defect. The flap was capable of covering one third of the right hemidiaphragm. All the animals had good post-surgery recoveries. Respiratory function was not compromised. Necropsies were taken at 15, 30 and 60 days post-surgery. Macro and microscopically, the muscle showed few adhesions with adjacent viscera and excellent healing. The Latissimus dorsi muscular flap can be useful for reconstruction of large diaphragmatic defects in dogs.

Diaphragmatic lymphatic vessel behavior during local skeletal muscle contraction.

Science.gov (United States)

Moriondo, Andrea; Solari, Eleonora; Marcozzi, Cristiana; Negrini, Daniela

2015-02-01

The mechanism through which the stresses developed in the diaphragmatic tissue during skeletal muscle contraction sustain local lymphatic function was studied in 10 deeply anesthetized, tracheotomized adult Wistar rats whose diaphragm was exposed after thoracotomy. To evaluate the direct effect of skeletal muscle contraction on the hydraulic intraluminal lymphatic pressures (Plymph) and lymphatic vessel geometry, the maximal contraction of diaphragmatic fibers adjacent to a lymphatic vessel was elicited by injection of 9.2 nl of 1 M KCl solution among diaphragmatic fibers while Plymph was recorded through micropuncture and vessel geometry via stereomicroscopy video recording. In lymphatics oriented perpendicularly to the longitudinal axis of muscle fibers and located at skeletal muscle contraction (Dmc) decreased to 61.3 ± 1.4% of the precontraction value [resting diameter (Drest)]; however, if injection was at >900 μm from the vessel, Dmc enlarged to 131.1 ± 2.3% of Drest. In vessels parallel to muscle fibers, Dmc increased to 122.8 ± 2.9% of Drest. During contraction, Plymph decreased as much as 22.5 ± 2.6 cmH2O in all submesothelial superficial vessels, whereas it increased by 10.7 ± 5.1 cmH2O in deeper vessels running perpendicular to contracting muscle fibers. Hence, the three-dimensional arrangement of the diaphragmatic lymphatic network seems to be finalized to efficiently exploit the stresses exerted by muscle fibers during the contracting inspiratory phase to promote lymph formation in superficial submesothelial lymphatics and its further propulsion in deeper intramuscular vessels. Copyright © 2015 the American Physiological Society.

Neuralgic Amyotrophy: A Rare Cause of Bilateral Diaphragmatic Paralysis

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Neil Shinder

1998-01-01

Full Text Available Neuralgic amyotrophy, also known as brachial neuritis, is a well described clinical entity. Diaphragmatic dysfunction, as a result of phrenic nerve root involvement (cervical roots 3 to 5, is an uncommon, but increasingly recognized association. The case of a previously healthy 61-year-old woman who, after a prodrome of neck and shoulder discomfort, presented with severe orthopnea is described. Pulmonary function and electrophysiological studies led to a diagnosis of bilateral diaphragmatic paralysis. The patient's clinical course and the exclusion of other nerve entrapment syndromes and neurological disorders strongly favoured the diagnosis of neuralgic amyotrophy.

Video-assisted thoracoscopic implantation of a diaphragmatic pacemaker in a child with tetraplegia: indications, technique, and results

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Darcy Ribeiro Pinto Filho

2015-02-01

Full Text Available We report the case of a child with tetraplegia after cervical trauma, who subsequently underwent diaphragmatic pacemaker implantation. We reviewed the major indications for diaphragmatic pacing and the types of devices employed. We highlight the unequivocal benefit of diaphragmatic pacing in the social and educational reintegration of individuals with tetraplegia.

Diaphragmatic pacing stimulation in spinal cord injury: anesthetic and perioperative management

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Miguel L. Tedde

2012-11-01

Full Text Available OBJECTIVE: The standard therapy for patients with high-level spinal cord injury is long-term mechanical ventilation through a tracheostomy. However, in some cases, this approach results in death or disability. The aim of this study is to highlight the anesthetics and perioperative aspects of patients undergoing insertion of a diaphragmatic pacemaker. METHODS: Five patients with quadriplegia following high cervical traumatic spinal cord injury and ventilator-dependent chronic respiratory failure were implanted with a laparoscopic diaphragmatic pacemaker after preoperative assessments of their phrenic nerve function and diaphragm contractility through transcutaneous nerve stimulation. ClinicalTrials.gov: NCT01385384. RESULTS: The diaphragmatic pacemaker placement was successful in all of the patients. Two patients presented with capnothorax during the perioperative period, which resolved without consequences. After six months, three patients achieved continuous use of the diaphragm pacing system, and one patient could be removed from mechanical ventilation for more than 4 hours per day. CONCLUSIONS: The implantation of a diaphragmatic phrenic system is a new and safe technique with potential to improve the quality of life of patients who are dependent on mechanical ventilation because of spinal cord injuries. Appropriate indication and adequate perioperative care are fundamental to achieving better results.

Phrenic Nerve Conduction Abnormalities Correlate with Diaphragmatic Descent in Chronic Obstructive Pulmonary Disease.

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El-Tantawi, Gihan A Younis; Imam, Mohamed H; Morsi, Tamer S

2015-01-01

Diaphragmatic weakness in chronic obstructive pulmonary disease (COPD) is ascribed to hyperinflation-induced diaphragm shortening as well as impairment in cellular and subcellular structures. Although phrenic neuropathy is known to cause diaphragmatic weakness, phrenic neuropathy is rarely considered in COPD. This work aimed at assessing phrenic nerve conduction in COPD and its relation to radiographic hyperinflation and pulmonary function. Forty COPD patients were evaluated. Radiographic parameters of lung hyperinflation were measured on postero-anterior and lateral chest x-ray films. Flow volume loop parameters were obtained from all patients. Motor conduction study of the phrenic nerves was performed and potentials were recorded over the xiphoid process and the ipsilateral 7th intercostal space. Twenty-seven healthy subjects were enrolled as controls. Parameters of phrenic nerve conduction differed significantly in patients compared to controls. Phrenic nerve abnormalities were detected in 17 patients (42.5%). Electrophysiological measures correlated with diaphragmatic angle of depression on lateral view films and with lung height on postero-anterior films. They did not correlate with the flow volume loop data or disease severity score. Phrenic nerve conduction abnormality is an appreciated finding in COPD. Nerve stretching associated with diaphragmatic descent can be a suggested mechanism for nerve lesion. The presence of phrenic neuropathy may be an additional contributing factor to diaphragmatic dysfunction in COPD patients.

Features of Somatic and Reproductive Status of Women, who Gave Birth to a Child with Congenital Malformations, and Their Identifiсation Using «Case — Control» Method According to Data of Lviv Regional Clinical Perinatal Center in 2007–2009

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Zborovska N.V. Zborovska N.V.

2012-04-01

While comparing a range of reproductive anamnesis indices there was noticed a higher percentage of dead children, stillbirths, anemia in pregnancy, gestosis in the first half of pregnancy, gestosis in the second half of pregnancy, abnormal labors in mothers who gave birth to a child with congenital malformations than in women of control group. According to ultrasound diagnosis congenital abnormalities in pregnancy were detected in 31.6 % of cases. There were diagnosed 4 cases of Arnold — Chiari syndrome (spina bifida and internal hydrocephalus; and 3 cases of each: hydrocephalus, gastroschisis, small intestinal atresia, and 2 cases of each: congenital malformations, hypoplastic left heart syndrome, Down’s syndrome, congenital diaphragmatic hernia, omphalocele, congenital rectal atresia, and 1 case of each: spina bifida, congenital absence of forearm and hand, left ventricular rabdomioma, encephalocele.

Case Report - Diaphragmatic eventration complicated by gastric ...

African Journals Online (AJOL)

Eventration of the diaphragm with gastric volvulus is uncommon. Gastric perforation in these cases is rare and usually associated with acute gastric volvulus with strangulation. We describe a case of diaphragmatic eventration with chronic gastric volvulus with gastric perforation without strangulation in an elderly man.

Laparoscopic management of diaphragmatic endometriosis by three different approaches.

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Roman, Horace; Darwish, Basma; Provost, Delphine; Baste, Jean-Marc

2016-08-01

To report our three surgical approaches in the management of diaphragmatic endometriosis. Video article presenting laparoscopic surgical techniques, with and without robotic assistance. University hospital. Nulliparas with deep endometriosis associated with multiple endometriosis lesions of the diaphragm. Laparoscopic approach in women who present with small black-pigmented diaphragmatic lesions, with or without infiltration of the diaphragm, which are ablated using plasma energy. Robotic-assisted laparoscopic route in larger deep infiltrating implants, which are resected. To avoid phrenic nerve injury, robotic-assisted thoracoscopy is preferred in large lesions involving the central tendon of the diaphragm. The steps of each technique are emphasized. Surgical technique reports in anonymous patients are exempted from ethical approval by the Institutional Review Board. Seven patients have been managed by these procedures from July 2015 to March 2016. Patients' functional outcomes were uneventful, with no phrenic nerve palsy or residual chest and right shoulder pain. By combining resection and ablation techniques, the laparoscopy and thoracoscopy route, conventional and robotic-assisted minimally invasive approach, we offer a surgical strategy that is as conservative as possible, with an aim to limit postoperative adhesions between the liver and the diaphragm, and avoid diaphragmatic paralysis. Copyright © 2016 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

An unusual cause of orthopnoea-hashimoto's thyroiditis presenting as bilateral diaphragmatic palsy

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N.K. Thulaseedharan, MBBS, MD(General Medicine

2017-01-01

Full Text Available We report a case of 36 yr old male without any comorbidities, who presented with a history of gradually progressive dyspnoea and orthopnoea for 6 months. Physical examination revealed bradycardia, paradoxical respiration suggestive of bilateral diaphragmatic palsy. Fluoroscopy demonstrated the presence of bilateral diaphragmatic paralysis. Etiological work up showed evidence of autoimmune hypothyroidism due to hashimoto's thyroiditis. Other possibilities were ruled out with appropriate tests. He was started on thyroxine and showed symptomatic improvement.

Increasing mortality burden among adults with complex congenital heart disease.

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Greutmann, Matthias; Tobler, Daniel; Kovacs, Adrienne H; Greutmann-Yantiri, Mehtap; Haile, Sarah R; Held, Leonhard; Ivanov, Joan; Williams, William G; Oechslin, Erwin N; Silversides, Candice K; Colman, Jack M

2015-01-01

Progress in management of congenital heart disease has shifted mortality largely to adulthood. However, adult survivors with complex congenital heart disease are not cured and remain at risk of premature death as young adults. Thus, our aim was to describe the evolution and mortality risk of adult patient cohorts with complex congenital heart disease. Among 12,644 adults with congenital heart disease followed at a single center from 1980 to 2009, 176 had Eisenmenger syndrome, 76 had unrepaired cyanotic defects, 221 had atrial switch operations for transposition of the great arteries, 158 had congenitally corrected transposition of the great arteries, 227 had Fontan palliation, and 789 had repaired tetralogy of Fallot. We depict the 30-year evolution of these 6 patient cohorts, analyze survival probabilities in adulthood, and predict future number of deaths through 2029. Since 1980, there has been a steady increase in numbers of patients followed, except in cohorts with Eisenmenger syndrome and unrepaired cyanotic defects. Between 1980 and 2009, 308 patients in the study cohorts (19%) died. At the end of 2009, 85% of survivors were younger than 50 years. Survival estimates for all cohorts were markedly lower than for the general population, with important differences between cohorts. Over the upcoming two decades, we predict a substantial increase in numbers of deaths among young adults with subaortic right ventricles, Fontan palliation, and repaired tetralogy of Fallot. Anticipatory action is needed to prepare clinical services for increasing numbers of young adults at risk of dying from complex congenital heart disease. © 2014 The Authors. Congenital Heart Disease Published by Wiley Periodicals, Inc.

Combined mitral valve replacement associated with the Bentall procedure, diaphragmatic hernia repair and reconstruction of the pectus excavatum in a 26-year-old patient with Marfan syndrome.

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Stępiński, Piotr; Stankowski, Tomasz; Aboul-Hassan, Sleiman Sebastian; Szymańska, Anna; Marczak, Jakub; Cichoń, Romuald

2016-06-01

A 26-year-old man with Marfan syndrome was admitted as an emergency patient with ascending aorta aneurysm, severe mitral and aortic regurgitation, diaphragmatic hernia and pectus excavatum. After completion of diagnostics a combined surgical procedure was performed.

Combined mitral valve replacement associated with the Bentall procedure, diaphragmatic hernia repair and reconstruction of the pectus excavatum in a 26-year-old patient with Marfan syndrome

OpenAIRE

St?pi?ski, Piotr; Stankowski, Tomasz; Aboul-Hassan, Sleiman Sebastian; Szyma?ska, Anna; Marczak, Jakub; Cicho?, Romuald

2016-01-01

A 26-year-old man with Marfan syndrome was admitted as an emergency patient with ascending aorta aneurysm, severe mitral and aortic regurgitation, diaphragmatic hernia and pectus excavatum. After completion of diagnostics a combined surgical procedure was performed.

Perioperative survival rates after surgery for diaphragmatic hernia in dogs and cats: 92 cases (1990-2002).

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Gibson, Thomas W G; Brisson, Brigitte A; Sears, William

2005-07-01

To determine the survival rates of dogs and cats that underwent surgical treatment for traumatic diaphragmatic hernia within 24 hours of admission and determine whether timing of surgery affected perioperative survival rate. Retrospective study. 63 dogs and 29 cats treated surgically for traumatic diaphragmatic hernia. Medical records were reviewed to evaluate associations between perioperative survival rates and variables including timing of surgery in relation to admission and acute versus chronic diaphragmatic hernia. Among the 92 animals, 82 (89.1%) were discharged alive after surgery. Sixty-four (69.6%) patients received surgical intervention within 12 hours of admission, and 84 (91.3%) received surgical intervention within 24 hours of admission. Median time from admission to discharge was 4 days (2 to 33 days). Data for acute cases (68 dogs and cats) were analyzed separately. Sixty-three (92.6%) patients with acute diaphragmatic hernia received surgical intervention within 24 hours of admission to the hospital, and 59 (93.7%) of these patients were discharged alive. Twenty-nine (42.6%) patients with acute diaphragmatic hernia received surgical intervention within 24 hours of trauma, and 26 of 29 (89.7%) patients were discharged alive. An overall acute and chronic perioperative survival rate of 89.7% was observed in dogs and cats that received surgical intervention within 24 hours of admission. Results in 68 dogs and cats that underwent surgery within 24 hours of admission suggested that early surgical intervention for acute diaphragmatic hernia was associated with good perioperative survival rates.

Management of congenital esophageal stenosis associated with ...

African Journals Online (AJOL)

Aim The aim of this work was to study the incidence, management of congenital esophageal stenosis (CES) associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF), and its impact on esophageal stricture (ES) after primary repair. Patients and methods From January 2006 to December 2014, ...

Retrocrural Lymph Node Metastasis Disclosed by (18)F-FDG PET/CT: A Predictor of Supra-diaphragmatic Spread in Ovarian Cancer.

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Im, Hyung-Jun; Kim, Yong-Il; Paeng, Jin Chul; Chung, June-Key; Kang, Soon-Beom; Lee, Dong Soo

2012-03-01

Retrocrural lymph nodes (RCLNs) communicate with retroperitoneal and posterior mediastinal LNs. It is possible that, when RCLNs are involved, supra-diaphragmatic extension will occur in abdomino-pelvic cancers. The authors investigated performance of (18)F-FDG PET/CT to diagnose RCLN metastasis and whether RCLN metastases were associated with supra-diaphragmatic lymphatic metastases of ovarian cancer. Sixty-seven patients with stage IV ovarian cancer who had undergone (18)F-FDG PET/CT were included in this retrospective study. Diagnostic performance of (18)F-FDG PET/CT for RCLN metastasis was evaluated. Patients were divided into two groups by presence or absence of supra-diaphragmatic LN metastasis. The prevalences of RCLN metastasis between the two groups were compared and the odds ratio was calculated. Sensitivity and specificity of (18)F-FDG PET/CT for RCLN metastasis were 96.3 and 100%, respectively. Of the 67 study subjects, 27 patients had RCLN metastases (40.3%). Fifty patients had supra-diaphragmatic LN metastases. (18)F-FDG PET/CT showed 26 RCLN metastases in patients with supra-diaphragmatic LN metastases (54.5%), and only 1 in patients without supra-diaphragmatic LN metastasis (5.9%), and the difference between two groups was statistically significant (P supra-diaphragmatic LN metastasis was 17.3 (95% confidence interval = 2.1 to 140.9, P = 0.008). Performance of (18)F-FDG PET/CT to diagnose RCLN metastasis was excellent. RCLN metastasis revealed by (18)F-FDG PET/CT was strongly associated with supra-diaphragmatic LN spread of ovarian cancer. Thus, RCLN metastasis could be used as a predictor of supra-diaphragmatic lymphatic metastasis of ovarian cancer.

Thoracic and diaphragmatic endometriosis: Single-institution experience using a novel broadened diagnostic criteria

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Larraín, Demetrio; Suárez, Francisco; Braun, Hernán; Chapochnick, Javier; Diaz, Lidia; Rojas, Iván

2018-06-05

To describe our experience with the multidisciplinary management of both thoracic/diaphragmatic endometriosis (TED), applying a broadened definition of the “thoracic endometriosis syndrome (TES)” to define cases. We present a retrospective series of consecutive patients affected by pathology-proven TED, treated at our institution, during a period of 7 years. Five women were included. Two cases were referred due to catamenial chest/shoulder pain, one due to recurrent catamenial pneumothorax, one due to new-onset diaphragmatic hernia. One patient had not thoracic symptoms, and diaphragmatic endometriosis was found during gynecologic laparoscopy for pelvic endometriosis. Endometriosis was histologically confirmed in all cases. After follow-up all patients remain asymptomatic. Broadened TES criteria could increase the incidence of TED and determine better knowledge of this condition. Multidisciplinary, minimally invasive surgery is effective and safe, but should be reserved to tertiary referral centers.

Diaphragmatic Hernia Masquerading as Pleural Effusion | Nalladaru ...

African Journals Online (AJOL)

Rupture of the diaphragm is almost always due to major trauma. We present here an unusual and rare case of late presentation of diaphragmatic hernia after an innocuous injury. The patient was initially misdiagnosed as a left pleural effusion on the basis of chest X.ray and ultrasound findings. Finally, the diagnosis was ...

Diaphragmatic eventration complicated by gastric volvulus with perforation

OpenAIRE

Gupta, V; Chandra, A; Gupta, P

2012-01-01

Eventration of the diaphragm with gastric volvulus is uncommon. Gastric perforation in these cases is rare and usually associated with acute gastric volvulus with strangulation. We describe a case of diaphragmatic eventration with chronic gastric volvulus with gastric perforation without strangulation in an elderly man.

Crura sign: differentiation between traumatic rupture of the diaphragm and nontraumatic diaphragmatic elevation on CT

International Nuclear Information System (INIS)

Kim, Sung Jin; Han, Hae Ja; Kim, Wang Jung; Youk, Yong Soo; Han, Gi Seok; Cha, Sang Hoon; Park, Kil Sun; Kim, Dae Young

1997-01-01

To evaluate whether in patients with diffuse evaluation of a hemidiaphragm on chest radiographs, the apparence of the crura on CT might be helpful in differentiating between traumatic rupture of the diaphragm (TRD) and nontraumatic causes such as diaphragmatic palsy or diffuse diaphragmatic eventration. Among patients with diffuse elevations of a hemidiaphragm on chest radiograph, 27 who had patients undergone CT scans were retrospectively reviewed. Twelve patients had surgically proven TRD, and 15 had nontraumatic elevation of a hemidiaphragm such as diaphragmatic palsy or diffuse diaphragmatic eventration. Under the hypothesis that the affected crus is markedly thinner than the normal side in nontraumatic elevation but is normal in TRD ('crura sign', we optically assessed without measurement the thickness of both crura. In all patients with TRD, the thickness of the affected crus was similar to that of the contralateral side. In all patients with nontraumatic causes, however, the crus of the elevated hemidiaphragm was markedly thinner than that of the normal side. The 'crura sign' may be useful additional CT finding of traumatic rupture the diaphragm

Retrocrural Lymph Node Metastasis Disclosed by 18F-FDG PET/CT: A Predictor of Supra-diaphragmatic Spread in Ovarian Cancer

International Nuclear Information System (INIS)

Im, Hyung Jun; Kim, Yong il; Paeng, Jin Chul; Chung, June Key; Kang, Soon Beom; Lee, Dong Soo

2012-01-01

Retrocrual lymph nodes (RCLNs) communicate with retroperitoneal and posterior mediastinal LNs. It is possible that, when RCLNs are involved, supra diaphragmatic extension will occur in abdomino pelvic cancers. The authors investigated performance of 18F FDG PET/CT to diagnose RCLN metastasis and whether RCLN metastases were associated with supra diaphragmatic lymphatic metastases of ovarian cancer. Sixty seven patients with stage IV ovarian cancer who had undergone 18F FDG PET/CT were included in this retrospective study. Diagnostic performance of 18F FDG PET/CT for RCLN metastasis was evaluated. Patients were divided into two groups by presence or absence of supra diaphragmatic LN metastasis. The prevalences of RCLN metastasis between the two groups were compared and the odds ratio was calculated. Sensitivity and specificity of 18F FDG PET/CT for RCLN metastasis were 96.3 and 100%, respectively. Of the 67 study subjects, 27 patients had RCLN metastases (40.3%). Fifty patients had supra diaphragmatic LN metastases. 18F FDG PET/CT showed 26 RCLN metastases in patients with supra diaphragmatic LN metastases (54.5%), and only 1 in patients without supra diaphragmatic LN metastasis (5.9%), and the difference between two groups was statistically significant (P 18F FDG PET/CT to diagnose RCLN metastasis was excellent. RCLN metastasis revealed by 18F FDG PET/CT was strongly associated with supra diaphragmatic LN spread of ovarian cancer. Thus, RCLN metastasis could be used as a predictor of supra diaphragmatic lymphatic metastasis of ovarian cancer

New Technologies for Surgery of the Congenital Cardiac Defect

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David Kalfa

2013-07-01

Full Text Available The surgical repair of complex congenital heart defects frequently requires additional tissue in various forms, such as patches, conduits, and valves. These devices often require replacement over a patient’s lifetime because of degeneration, calcification, or lack of growth. The main new technologies in congenital cardiac surgery aim at, on the one hand, avoiding such reoperations and, on the other hand, improving long-term outcomes of devices used to repair or replace diseased structural malformations. These technologies are: 1 new patches: CorMatrix® patches made of decellularized porcine small intestinal submucosa extracellular matrix; 2 new devices: the Melody® valve (for percutaneous pulmonary valve implantation and tissue-engineered valved conduits (either decellularized scaffolds or polymeric scaffolds; and 3 new emerging fields, such as antenatal corrective cardiac surgery or robotically assisted congenital cardiac surgical procedures. These new technologies for structural malformation surgery are still in their infancy but certainly present great promise for the future. But the translation of these emerging technologies to routine health care and public health policy will also largely depend on economic considerations, value judgments, and political factors.

Current status of cardiovascular surgery in Japan 2013 and 2014: A report based on the Japan Cardiovascular Surgery Database. 2: Congenital heart surgery.

Science.gov (United States)

Hirata, Yasutaka; Hirahara, Norimichi; Murakami, Arata; Motomura, Noboru; Miyata, Hiroaki; Takamoto, Shinichi

2018-01-01

We analyzed the mortality and morbidity of congenital heart surgery in Japan using the Japan Cardiovascular Surgery Database (JCVSD). Data regarding congenital heart surgery performed between January 2013 and December 2014 were obtained from JCVSD. The 20 most frequent procedures were selected and the mortality rates and major morbidities were analyzed. The mortality rates of atrial septal defect repair and ventricular septal defect repair were less than 1%, and the mortality rates of tetralogy of Fallot repair, complete atrioventricular septal defect repair, bidirectional Glenn, and total cavopulmonary connection were less than 2%. The mortality rates of the Norwood procedure and total anomalous pulmonary venous connection repair were more than 10%. The rates of unplanned reoperation, pacemaker implantation, chylothorax, deep sternal infection, phrenic nerve injury, and neurological deficit were shown for each procedure. Using JCVSD, the national data for congenital heart surgery, including postoperative complications, were analyzed. Further improvements of the database and feedback for clinical practice are required.

Emergency traumatic Diaphragmatic injuries in Benin city | Iribhogbe ...

African Journals Online (AJOL)

Diaphragmatic injuries (DI) frequently accompany thoracoabdominal trauma. The diagnosis remains a challenge to surgeons and radiologists worldwide but missed injuries to the diaphragm is associated with great morbidity and mortality. We aimed to determine the prevalence of this injury in acute trauma and in general ...

[Congenital megacolon in neonates and infants: impact of early, one-stage repair on morbidity and surgical complications].

Science.gov (United States)

Calisti, A; Molle, P H; Vallasciani, S

2004-01-01

Hirschsprung's disease (HD) is a major cause of congenital bowel obstruction in children. Classical management includes stoma creation, a pull-through procedure, and restoration of bowel continuity. Colostomy may be also pulled through directly at second operation. Since the first report of a single stage (SS) primary pull-through without colostomy for HD, in 1980, several patients have been treated according to this procedure, even in neonatal period. All of them had a favourable outcome with better compliance of families and less hospital stay. The Authors reviewed the clinical records of 22 patients selected among 36 treated for HD between 1992 and 2000; in all, disease extended to rectum-sigma-descendent; all cases of "total aganglionosis" or "ultra short" cases were excluded. Fifteen patients underwent SS repair: 11 neonates were operated at mean age of 69 days (range 14-172). In four infants diagnosis was made later (mean age 15 +/- 9 months) and SS repair was performed 8 days after (range 4-14). Seven patients underwent multiple stage (MS) repairs for clinical reasons: pull-through of the previous colostomy was performed at mean age of 171 days (range 47-330). A Duhamel procedure with an Endo-GIA linear stapler was performed in all No differences in gestational age or birth weight were found in both groups. Complications were observed only in the MS group (2 related to colostomy, 1 to postoperative adhesions). The Authors conclude that primary SS treatment of HD is safe and effective even in newborn patient, reduce familiar stress and hospital stay. The lower morbidity and complication rates could be explained by the limited bowel manipulation. Accurate histological-hystochemical diagnosis and meticulous preoperative bowel management should be part of the management.

Repair of anomalous aortic origin of a coronary artery in 113 patients: a Congenital Heart Surgeons' Society report.

Science.gov (United States)

Poynter, Jeffrey A; Bondarenko, Igor; Austin, Erle H; DeCampli, William M; Jacobs, Jeffrey P; Ziemer, Gerhard; Kirshbom, Paul M; Tchervenkov, Christo I; Karamlou, Tara; Blackstone, Eugene H; Walters, Henry L; Gaynor, J William; Mery, Carlos M; Pearl, Jeffrey M; Brothers, Julie A; Caldarone, Christopher A; Williams, William G; Jacobs, Marshall L; Mavroudis, Constantine

2014-10-01

Anomalous aortic origin of a coronary artery (AAOCA) encompasses a wide morphologic spectrum, which has impeded consensus regarding indications for the diverse repair strategies. We constructed a profile of current surgical techniques and explore their application to morphologic variants. Patients<30 years old (n=113) with isolated AAOCA who underwent operations at 29 Congenital Heart Surgeons Society (CHSS) institutions from 1998 to 2012 were identified from the CHSS AAOCA Registry. Operative findings were related to surgical techniques at index repairs by cross-tabulation. Anomalous origin of the left main or left anterior descending coronary artery was present in 33 (29%) patients and of the right coronary artery in 78 (69%) patients; 2 arteries originated directly above the commissure between the left and right sinuses. There were 101 (89%) interarterial and intramural (IA/IM) arteries, 10 (9%) were interarterial but not intramural (IA/NIM) and 2 (2%) were neither interarterial nor intramural. Intramural arteries were unroofed in 100 (88%) operations, usually with intimal tacking after incision (n=47) or excision (n=25) of the common wall. Coronary reimplantation (n=11), pulmonary artery relocation (n=7; 5 for IA/NIM), simple ostioplasty (without unroofing; n=3), coronary artery bypass grafting (n=2), and ostial window (n=1) were less common. In 37 (33%) operations, a valvar commissure was taken down; 33 were resuspended. Current surgical repair of AAOCA is individualized to morphology, particularly the presence of intramural and/or interarterial segments. This report is foundational for future planned CHSS studies that will examine interventional and noninterventional outcomes and ultimately guide management of AAOCA. © The Author(s) 2014.

A tetrad of bicuspid aortic valve association: A single-stage repair

Science.gov (United States)

Barik, Ramachandra; Patnaik, A. N.; Mishra, Ramesh C.; Kumari, N. Rama; Gulati, A. S.

2012-01-01

We report a 27 years old male who presented with a combination of both congenital and acquired cardiac defects. This syndrome complex includes congenital bicuspid aortic valve, Seller's grade II aortic regurgitation, juxta- subclavian coarctation, stenosis of ostium of left subclavian artery and ruptured sinus of Valsalva aneurysm without any evidence of infective endocarditis. This type of constellation is extremely rare. Neither coarctation of aorta with left subclavian artery stenosis nor the rupture of sinus Valsalva had a favorable pathology for percutaneus intervention. Taking account into morbidity associated with repeated surgery and anesthesia patient underwent a single stage surgical repair of both the defects by two surgical incisions. The approaches include median sternotomy for rupture of sinus of Valsalva and lateral thoracotomy for coarctation with left subclavian artery stenosis. The surgery was uneventful. After three months follow up echocardiography showed mild residual gradient across the repaired coarctation segment, mild aortic regurgitation and no residual left to right shunt. This patient is under follow up. This is an extremely rare case of single stage successful repair of coarctation and rupture of sinus of Valsalva associated with congenital bicuspid aortic valve. PMID:22629035

Therapeutic effects of diaphragmatic plication for acquired unilateral non-malignant diaphragm paralysis in twenty patients

Directory of Open Access Journals (Sweden)

Reza Bagheri

2013-12-01

Full Text Available Background: Acquired paralysis of the diaphragm is a condition caused by trauma, surgical injuries, (lung cancer surgery, esophageal surgery, cardiac surgery, thoracic surgery, and is sometimes of an unknown etiology. It can lead to dyspnea and can affect ventilatory function and patients activity. Diaphragmatic plication is a treatment method which decreases inconsistent function of diaphragm. The aim of this study is to evaluate the outcome of diaphragmatic plication in patients with acquired unilateral non-malignant diaphragmatic paralysis. Methods: From 1991 to 2011, 20 patients with acquired unilateral diaphragmatic paralysis who underwent surgery enrolled in our study in Ghaem Hospital Mashhad University of Medical Science. Patients were evaluated in terms of age, sex, BMI, clinical symptoms, dyspnea score (DS, etiology of paralysis, diagnostic methods, respiratory function tests and complication of surgery. Some tests including dyspnea score were carried out again six months after surgery. We evaluated patients with SPSS version 11.5 and Paired t-test or nonparametric equivalent. Results: Twenty patients enrolled in our study. 14 were male and 6 were female. The mean age was 58 years and the average time interval between diagnosis to surgical treatment was 38.3 months. Acquired diaphragmatic paralysis was mostly caused by trauma (in 11 patients and almost occurred on the left side (in 15 patients. Diagnostic methods included chest x-ray, CT scan, ultrasonography and sniff. Test prior to surgery the average FVC was 41.4±7 percent and the average FEV1 was 52.4±6 percent and after surgery they were 80.1±8.6 percent and 74.4±1 percent respectively. The average increase in FEV1 and FVC 63.4±4, 61.1±7.8. Performing surgery also leads to a noticeable improvement in dyspnea score in our study. Conclusion: In patients with acquired unilateral non-malignant diaphragm paralysis diaphragmatic plication is highly recommended due to the

Why do we need more data on MR volumetric measurements of the fetal lung?

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Rubesova, Erika [Stanford University, Pediatric Radiology, Lucile Packard Children' s Hospital at Stanford, Stanford, CA (United States)

2016-02-15

Fetal lung hypoplasia is associated with a series of congenital anomalies, particularly the congenital diaphragmatic hernia. Evaluation of the severity of the lung hypoplasia is important for parental counseling, assessment of prognosis and planning of postnatal management. Although a large number of studies have been performed to measure fetal lung volumes in order to predict outcome, there are little data on fetuses younger than 24 weeks of gestation, the age when parental counseling is crucial if termination is considered. Few studies have evaluated prognosis of lung hypoplasia in fetuses with congenital chest anomalies other than congenital diaphragmatic hernia. We review the studies on measurements of the fetal lung volumes by MRI. (orig.)

Why do we need more data on MR volumetric measurements of the fetal lung?

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Rubesova, Erika

2016-01-01

Fetal lung hypoplasia is associated with a series of congenital anomalies, particularly the congenital diaphragmatic hernia. Evaluation of the severity of the lung hypoplasia is important for parental counseling, assessment of prognosis and planning of postnatal management. Although a large number of studies have been performed to measure fetal lung volumes in order to predict outcome, there are little data on fetuses younger than 24 weeks of gestation, the age when parental counseling is crucial if termination is considered. Few studies have evaluated prognosis of lung hypoplasia in fetuses with congenital chest anomalies other than congenital diaphragmatic hernia. We review the studies on measurements of the fetal lung volumes by MRI. (orig.)

The importance of social media for patients and families affected by congenital anomalies: A Facebook cross-sectional analysis and user survey.

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Jacobs, Robyn; Boyd, Leanne; Brennan, Kirsty; Sinha, C K; Giuliani, Stefano

2016-11-01

We aimed to define characteristics and needs of Facebook users in relation to congenital anomalies. Cross-sectional analysis of Facebook related to four congenital anomalies: anorectal malformation (ARM), congenital diaphragmatic hernia (CDH), congenital heart disease (CHD) and hypospadias/epispadias (HS/ES). A keyword search was performed to identify relevant Groups/Pages. An anonymous survey was posted to obtain quantitative/qualitative data on users and their healthcare needs. 54 Groups and 24 Pages were identified (ARM: 10 Groups; CDH: 9 Groups, 7 Pages; CHD: 32 Groups, 17 Pages; HS/ES: 3 Groups), with 16,191 Group members and 48,766 Page likes. 868/1103 (79%) of respondents were parents. Male:female ratio was 1:10.9. 65% of the users were 26-40years old. Common reasons for joining these Groups/Pages included: seeking support, education, making friends, and providing support to others. 932/1103 (84%) would like healthcare professionals (HCPs) to actively participate in their Group. 31% of the respondents felt that they did not receive enough support from their healthcare system. 97% of the respondents would like to join a Group linked to their primary hospital. Facebook Groups/Pages related to congenital anomalies are highly populated and active. There is a need for HCPs and policy makers to better understand and participate in social media to support families and improve patient care. Copyright © 2016 Elsevier Inc. All rights reserved.

Surgical Techniques for Diaphragmatic Resection During Cytoreduction in Advanced or Recurrent Ovarian Carcinoma: A Systematic Review and Meta-analysis.

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Bogani, Giorgio; Ditto, Antonino; Martinelli, Fabio; Lorusso, Domenica; Chiappa, Valentina; Donfrancesco, Cristina; Di Donato, Violante; Indini, Alice; Aletti, Giovanni; Raspagliesi, Francesco

2016-02-01

Optimal cytoreduction is one the main factors improving survival outcomes in patients affected by ovarian cancer (OC). It is estimated that approximately 40% of OC patients have gross disease located on the diaphragm. However, no mature data evaluating outcomes of surgical techniques for the management of diaphragmatic carcinosis exist. In the present study, we aimed to estimate surgery-related morbidity of different surgical techniques for diaphragmatic cytoreduction in advanced or recurrent OC. PubMed (MEDLINE), Web of Science, and Clincaltrials.gov databases were searched for records estimating outcomes of diaphragmatic peritoneal stripping (DPS) or diaphragmatic full-thickness resection (DFTR) for OC. The meta-analysis was performed using the Cochrane Review software. For the final analysis, 5 articles were available, including 272 patients. Diaphragmatic peritoneal stripping and DFTR were performed in 197 patients (72%) and 75 patients (28%), respectively. Pooled analysis suggested that the estimated pleural effusion rate was 43% and 51% after DPS and DFTR, respectively. The need for pleural punctures or chest tube placement was 4% and 9% after DPS and DFTR, respectively. The rate of postoperative pneumothorax (4% vs 9%; odds ratio, 0.31; 95% confidence interval, 0.05-2.08) and subdiaphragmatic abscess (3% vs 3%; odds ratio, 0.45; 95% confidence interval, 0.09-2.31) were similar after the execution of DPS and DFTR. Diaphragmatic surgery is a crucial step during cytoreduction for advanced or recurrent OC. Obviously, the choice to perform DPS or DFTR depends on the infiltration of the diaphragmatic muscle or not. Both the procedures are associated with a low pulmonary complication and chest tube placement rates.

Traumatic diaphragmatic injuries in infants and children: imaging findings

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Koplewitz, B.Z.; Manson, D.E.; Babyn, P.S.; Ramos, C.; Ein, S.H.

2000-01-01

Objectives. Traumatic diaphragmatic injuries (DI) in infants and children are uncommon and are often associated with multiple severe injuries. Delayed presentation can be life threatening due to organ herniation and strangulation. We present the imaging findings in a relatively large population of children who experienced this rare injury. Methods. Medical records of all patients admitted to our Trauma Service from 1977 to 1998 with DI were retrospectively reviewed recording imaging, clinical and surgical or autopsy findings. Results. Of sixteen patients with DI (7 females, 9 males; age 3 weeks to 15 years), 14 suffered from blunt trauma secondary to high-energy impact, and 2 from penetrating injuries. Unilateral DI occurred equally on each side, with one bilateral injury. Associated injuries, present in 81%, included severe head injuries, visceral, mesenteric and vascular injuries and multiple fractures. Six patients died from multiple organ failure (3), head injury (2), and shock (1). Findings in the initial chest X-ray suggested the diagnosis in 13 (81%) of 16 injuries, and CT demonstrated irregularity and thickening of the diaphragm in 4 out of 7. Conclusions. Plain film findings suggested the diagnosis in most; CT and MR were useful adjuncts. High index of suspicion and awareness of the mechanism of injury can lead to prompt diagnosis, early repair, and decreased morbidity and mortality. (orig.)

Diaphragmatic paralysis evaluated by phrenic nerve stimulation during fluoroscopy or real-time ultrasound

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McCauley, R.G.K.; Labib, K.B.

1984-10-01

Stimulation of the phrenic nerve by supplying an electrical impulse to the neck during fluoroscopy or real-time ultrasound (sonoscopy) of the diaphragm allows more precise functional evaluation than fluoroscopy and/or sonoscopy alone. This is especially true of patients who are unable to cooperate because the are on a ventilator, unconscious, or very young. The authors cite cases in which diaphragmatic paralysis was diagnosed by conventional methods but stimulation of the phrenic nerve demonstrated good diaphragmatic motion, leading to a change in prognosis in some cases and a change in therapy in others.

Diaphragmatic paralysis evaluated by phrenic nerve stimulation during fluoroscopy or real-time ultrasound

International Nuclear Information System (INIS)

McCauley, R.G.K.; Labib, K.B.

1984-01-01

Stimulation of the phrenic nerve by supplying an electrical impulse to the neck during fluoroscopy or real-time ultrasound (sonoscopy) of the diaphragm allows more precise functional evaluation than fluoroscopy and/or sonoscopy alone. This is especially true of patients who are unable to cooperate because the are on a ventilator, unconscious, or very young. The authors cite cases in which diaphragmatic paralysis was diagnosed by conventional methods but stimulation of the phrenic nerve demonstrated good diaphragmatic motion, leading to a change in prognosis in some cases and a change in therapy in others

Re-evaluation of lung to thorax transverse area ratio immediately before birth in predicting postnatal short-term outcomes of fetuses with isolated left-sided congenital diaphragmatic hernia: A single center analysis.

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Kido, Saki; Hidaka, Nobuhiro; Sato, Yuka; Fujita, Yasuyuki; Miyoshi, Kina; Nagata, Kouji; Taguchi, Tomoaki; Kato, Kiyoko

2018-05-01

We aimed to investigate whether the lung-to-thorax transverse area ratio (LTR) immediately before birth is of diagnostic value for the prediction of postnatal short-term outcomes in cases of isolated left-sided congenital diaphragmatic hernia (CDH). We retrospectively reviewed the cases of fetal isolated left-sided CDH managed at our institution between April 2008 and July 2016. We divided the patients into two groups based on LTR immediately before birth, using a cut-off value of 0.08. We compared the proportions of subjects within the two groups who survived until discharge using Fisher's exact test. Further, using Spearman's rank correlation, we assessed whether LTR was correlated with length of stay, duration of mechanical ventilation, and supplemental oxygen. Twenty-nine subjects were included (five with LTR < 0.08, and 24 with LTR ≥ 0.08). The proportion of subjects surviving until discharge was 40% (2/5) for patients with LTR < 0.08, as compared with 96% (23/24) for those with LTR ≥ 0.08. LTR measured immediately before birth was negatively correlated with the postnatal length of stay (Spearman's rank correlation coefficient, rs = -0.486), and the duration of supplemental oxygen (rs = -0.537). Further, the duration of mechanical ventilation was longer in patients with a lower LTR value. LTR immediately before birth is useful for the prediction of postnatal short-term outcomes in fetuses with isolated left-sided CDH. In particular, patients with prenatal LTR value less than 0.08 are at increased risk of postnatal death. © 2017 Japanese Teratology Society.

Tessier Number 30 Median Mandibular Cleft With Congenital Heart Anomalies in Qena, Egypt.

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Ali, Ahmed Ali Abdelrahim

2018-01-01

Median cleft deformities of the lower lip and mandible are very rare congenital anomalies. Our patient had median cleft of the lower lip, mandible, and the chin with tongue duplication, ankyloglossia, and cleft strap muscles with 2 neck contracture bands. This anomaly was associated with congenital heart disease transposition of great vessels, large ventricular septal defect, and severe pulmonary stenosis. Early repair was done at 6 months to improve feeding.

Congenital esophageal stenosis in 3 children: A case series

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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.

Natural history of extensive diaphragmatic injury on the right side: experimental study in rats

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Jorge Henrique Rivaben

2014-08-01

Full Text Available OBJECTIVE: To evaluate the natural healing of the rat diaphragm that suffered an extensive right penetrating injury.METHODS: Animals were submitted to an extensive penetrating injury in right diaphragm. The sample consisted of 40 animals. The variables studied were initial weight, weight 21 days after surgery; healing of the diaphragm, non-healing of the diaphragm, and herniated abdominal contents into the chest.RESULTS: Ten animals were used as controls for weight and 30 animals were operated. Two animals died during the experiment, so 28 animals formed the operated group; healing of the diaphragm occurred in 15 animals (54%, 11 other animals showed diaphragmatic hernia (39% and in two we observed only diaphragmatic injury without hernia (7%. Among the herniated organs, the liver was found in 100% of animals, followed by the omentum in 77%, small bowel in 62%, colon in 46%, stomach in 31% and spleen in 15%. The control group and the diaphragmatic healing subgroup showed increased weight since the beginning of the study and the 21 days after surgery (p <0.001. The unhealed group showed no change in weight (p = 0.228.CONCLUSION: there is a predominance of spontaneous healing in the right diaphragm; animals in which there was no healing of the diaphragm did not gain weight, and the liver was the organ present in 100% the diaphragmatic surface in all rats with healed diaphragm or not.

Potential implications of the helical heart in congenital heart defects.

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Corno, Antonio F; Kocica, Mladen J

2007-01-01

The anatomic and functional observations made by Francisco Torrent-Guasp, in particular his discovery of the helical ventricular myocardial band (HVMB), have challenged what has been taught to cardiologists and cardiac surgeons over centuries. A literature debate is ongoing, with interdependent articles and comments from supporters and critics. Adequate understanding of heart structure and function is obviously indispensable for the decision-making process in congenital heart defects. The HVMB described by Torrent-Guasp and the potential impact on the understanding and treatment of congenital heart defects has been analyzed in the following settings: embryology, ventriculo-arterial discordance (transposition of great arteries), Ebstein's anomaly, pulmonary valve regurgitation after repair of tetralogy of Fallot, Ross operation, and other congenital heart defects. The common structural spiral feature is only one of the elements responsible for the functional interaction of right and left ventricles, and understanding the form/function relationship in congenital heart defects is more difficult than for acquired heart disease because of the variety and complexity of congenital heart defects. Individuals involved in the care of patients with congenital heart defects have to be stimulated to consider further investigations and alternative surgical strategies.

Congenital Heart Disease: Causes, Diagnosis, Symptoms, and Treatments.

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Sun, RongRong; Liu, Min; Lu, Lei; Zheng, Yi; Zhang, Peiying

2015-07-01

The congenital heart disease includes abnormalities in heart structure that occur before birth. Such defects occur in the fetus while it is developing in the uterus during pregnancy. About 500,000 adults have congenital heart disease in USA (WebMD, Congenital heart defects medications, www.WebMD.com/heart-disease/tc/congenital-heart-defects-medications , 2014). 1 in every 100 children has defects in their heart due to genetic or chromosomal abnormalities, such as Down syndrome. The excessive alcohol consumption during pregnancy and use of medications, maternal viral infection, such as Rubella virus, measles (German), in the first trimester of pregnancy, all these are risk factors for congenital heart disease in children, and the risk increases if parent or sibling has a congenital heart defect. These are heart valves defects, atrial and ventricular septa defects, stenosis, the heart muscle abnormalities, and a hole inside wall of the heart which causes defect in blood circulation, heart failure, and eventual death. There are no particular symptoms of congenital heart disease, but shortness of breath and limited ability to do exercise, fatigue, abnormal sound of heart as heart murmur, which is diagnosed by a physician while listening to the heart beats. The echocardiogram or transesophageal echocardiogram, electrocardiogram, chest X-ray, cardiac catheterization, and MRI methods are used to detect congenital heart disease. Several medications are given depending on the severity of this disease, and catheter method and surgery are required for serious cases to repair heart valves or heart transplantation as in endocarditis. For genetic study, first DNA is extracted from blood followed by DNA sequence analysis and any defect in nucleotide sequence of DNA is determined. For congenital heart disease, genes in chromosome 1 show some defects in nucleotide sequence. In this review the causes, diagnosis, symptoms, and treatments of congenital heart disease are described.

Congenital scaphoid megalourethra associated with posterior urethral valve: A case report

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Apoorva Achyut Kulkarni

2018-01-01

Full Text Available Congenital scaphoid megalourethra is a very rare congenital anomaly of the anterior urethra in males. Here, a case of scaphoid megalourethra and posterior urethral valves is presented. A one and half year old male child came to us with complaints of ballooning at the tip of penis since birth. Micturating cystourethrogram showed a dilated glanular urethra. The patient underwent a Nesbitt’s longitudinal reduction urethroplasty with a single-staged, single layered repair. Post-operatively, the child passed a healthy stream of urine without straining

Epidemiology and Outcome of Major Congenital Malformations in a Large German County.

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Wittekindt, Boris; Schloesser, Rolf; Doberschuetz, Nora; Salzmann-Manrique, Emilia; Grossmann, Jasmin; Misselwitz, Bjoern; Rolle, Udo

2018-05-01

 Congenital malformations are associated with substantial neonatal morbidity and mortality. Furthermore, only sparse data are available on the modalities of care provided to and the associated clinical outcomes in affected neonates. In this study, we focused on five malformations that require surgery during the neonatal period: duodenal stenosis and atresia (DA), gastroschisis (GA), omphalocele (OM), congenital diaphragmatic herniation (CDH), and esophageal atresia (EA).  We reviewed the Hessian neonatal registry (2010-2015) to identify records including the ICD-10 (International Classification of Diseases, Tenth Edition) codes for the aforementioned diagnoses and identified 283 patients who were affected by at least one of these conditions. Multiple regression analyses were performed to further identify risk factors for mortality and extended length of hospital stay.  The incidence rates per 10,000 live births and inhospital mortality rates were as follows: DA: 1.79 and 3.6%; GA: 1.79 and 1.8%; OM: 1.60 and 24%; CDH: 1.32 and 27.5%; and EA: 2.67 and 11.1%, respectively. Thirty-three percent of the patients had not been born in a perinatal center in which corrective surgeries were performed. The following risk factors were significantly associated with early mortality: trisomy 13 and 18, congenital heart defects, prematurity, and high-risk malformations (OM and CDH). The predictors of length of stay were as follows: gestational age, number of additional malformations, and treatment in the center with the highest patient volume.  Epidemiology and outcome of major congenital malformations in Hesse, Germany, are comparable to previously published data. In addition, our data revealed a volume-outcome association with regard to the length of hospital stay. Georg Thieme Verlag KG Stuttgart · New York.

Diaphragmatic patch: A useful adjunct in surgical treatment of recurrent catamenial hemothorax

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J. Nwiloh

2011-11-01

Full Text Available Although catamenial hemothorax compared to pneumothorax is a rarer clinical presentation of thoracic endometriosis syndrome (TES, it is more commonly associated with diaphragmatic fenestrations. These openings may serve as entry portals for peritoneal fluid to access into the pleural space thereby perpetuating recurrent pleural effusion even after prior surgical pleurodesis. We report our experience with two patients with recurrent right catamenial hemothorax after previous interventions that were subsequently treated by talc pleurodesis and goretex diaphragmatic patch, and who have had no further recurrence at a mean follow up of 15 months.We therefore recommend that diaphragmatic patch should be considered as an adjunct to talc pleurodesis in patients with recurrent catamenial hemothorax when either multiple diaphragmatic fenestrations are seen at surgery or if there is concomitant bloody peritoneal fluid which could potentially lead to recurrence. The patch by sealing any occult pores and possible future fenestrations appear to decrease recurrent pleural effusion at an intermediate term follow up. Resumo: Embora o hemotórax catamenial comparado com o pneumotórax seja uma apresentação clínica mais rara de síndrome de endometriose torácica (TES, está mais associado a fenestrações diafragmáticas. Estas aberturas podem atuar como portais de entrada para o acesso ao fluido peritoneal na cavidade pleural, perpetuando assim o derrame pleural recorrente mesmo após uma pleurodese cirúrgica prévia. Registamos a nossa experiência em dois pacientes com hemotórax catamenial recorrente do lado direito após outras intervenções, que foram posteriormente tratados com pleurodese com talco e penso diafragmático em gore-tex, e que não apresentaram nenhuma outra recorrência durante um acompanhamento de 15 meses.Recomendamos, então, que o penso diafragmático seja considerado um auxiliar à pleurodese com talco em pacientes com hemot

Situs Inversus with Levocardia and Congenitally Corrected Transposition of Great Vessels in a 35 year old Male: A Case report.

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Ghorbnazadeh, Atefeh; Zirak, Nahid; Fazlinezhad, Afsoon; Moenipour, Aliasghar; Manshadi, Hamid Hoseinikhah; Teshnizi, Mohammad Abbasi

2017-01-01

Situs inversus with levocardia and congenitally corrected transposition of the great arteries represents a relatively very rare congenital condition and most patients are diagnosed in infancy or early age. This case report describes a 35-year old man with congenitally corrected transposition of the great arteries which presented with a five month history of exertional dyspnea. A diagnosis was confirmed by transesophageal echocardiogram, showing situs inversus, levocardia, atrioventricular and ventriculoarterial discordance. He underwent physiologic repair, and was discharged thirty five days after the operation, in a good general condition. Although management of the corrected transposition of the great arteries patients remains controversial, the recommendation is that physiologic repair may be the procedure of choice for some patients, particularly complicated cases.

Prenatal diagnosis of congenital paraesophageal hiatal hernia

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Min Jeng Cho

2018-05-01

Full Text Available Abstracts: Congenital paraesophageal hiatal hernia (CPEH is a rare condition. CPEH can cause important clinical problems such as gastric volvulus, hematemesis, vomiting, failure to thrive, and respiratory distress, it requires early diagnosis and prompt surgical treatment. In this paper, we describe a case of CPEH that was suspected in a prenatal ultrasound. Postnatal upper gastrointestinal contrast series confirmed a CPEH with intrathoracic gastric volvulus. An emergency operation was performed. The stomach was reduced, the hiatal defect was repaired by crural approximation, and a Nissen fundoplication was done. The prenatal diagnosis of CPEH is unusual, but prenatal detection is important because it allows planned neonatal surgery before the onset of complications and reduces long-term morbidity. Keywords: Congenital paraesophageal hiatal hernia, Antenatal diagnosis, Gastric volvulus

Radiographic diagnosis of diaphragmatic hernia: review of 60 cases in dogs and cats.

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Hyun, Changbaig

2004-06-01

Sixty cases of diaphragmatic hernia in dogs and cats were radiologically reviewed and categorized by their characteristic radiographic signs. Any particular predilection for age, sex, or breed was not observed. Liver, stomach and small intestine were more commonly herniated. At least two radiographs, at different angles, were required for a valid diagnosis, because some radiographic signs were not visible in a single radiographic view and more clearly detectable in two radiographic views. In addition to previously reported radiographic signs for diaphragmatic hernia, we found that the location of the stomach axis and the displacement of tracheal and bronchial segments were also useful radiographic signs.

The incidence of congenital malformations and variations in Göttingen minipigs.

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Ellemann-Laursen, S; Marsden, E; Peter, B; Downes, N; Coulby, D; Grossi, A B

2016-09-01

Knowledge of the incidence of spontaneous congenital abnormalities is critical for the accurate interpretation of findings in teratogenicity studies in any species. In this paper, results of the examination of 1739 neonatal Göttingen Minipigs are presented. Over the 2-year period under consideration, the incidence of external and visceral malformations was less than 0.2 and 0.1%, respectively. The most common external malformations were syndactyly, limb hyperflexion, domed head and scoliosis. The most common internal malformations were undescended testes, ventricular septal defect, diaphragmatic hernia and atrial septal defects. Pentadactyly and variation in the aortic arch's bifurcation (absent truncus bicaroticus) were the most common variations. These data will help support the use of the Göttingen Minipig as a non-rodent species in embryofetal development studies where concerns persist about the availability of background data. Copyright © 2016 Elsevier Inc. All rights reserved.

Delayed diaphragmatic herniation masquerading as a complicated parapneumonic effusion.

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Tsang, J; Ryan, F

1999-01-01

Injury to the diaphragm following blunt or penetrating thoracoabdominal trauma is not uncommon. Recognition of this important complication of trauma continues to be a challenge because of the lack of specific clinical and plain radiographic features, the frequent presence of other serious injuries and the potential for delayed presentation. Delayed diaphragmatic herniation often presents with catastrophic bowel obstruction or strangulation. Early recognition of diaphragmatic injury is required to avoid this potentially lethal complication. The case of a 35-year-old man with a history of a knife wound to the left flank 15 years previously, who presented with unexplained acute hypoxemic respiratory failure and a unilateral exudative pleural effusion that was refractory to tube thoracostomy drainage, is reported. After admission to hospital, he developed gross dilation of his colon; emergency laparotomy revealed an incarcerated colonic herniation into the left hemithorax. Interesting clinical features of this patient's case included the patient's hobby of weightlifting, a persistently deviated mediastinum despite drainage of the pleural effusion and deceptive pleural fluid biochemical indices.

Delayed Diaphragmatic Herniation Masquerading as a Complicated Parapneumonic Effusion

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John Tsang

1999-01-01

Full Text Available Injury to the diaphragm following blunt or penetrating thoraco-abdominal trauma is not uncommon. Recognition of this important complication of trauma continues to be a challenge because of the lack of specific clinical and plain radiographic features, the frequent presence of other serious injuries and the potential for delayed presentation. Delayed diaphragmatic herniation often presents with catastrophic bowel obstruction or strangulation. Early recognition of diaphragmatic injury is required to avoid this potentially lethal complication. The case of a 35-year-old man with a history of a knife wound to the left flank 15 years previously, who presented with unexplained acute hypoxemic respiratory failure and a unilateral exudative pleural effusion that was refractory to tube thoracostomy drainage, is reported. After admission to hospital, he developed gross dilation of his colon; emergency laparotomy revealed an incarcerated colonic herniation into the left hemithorax. Interesting clinical features of this patient's case included the patient's hobby of weightlifting, a persistently deviated mediastinum despite drainage of the pleural effusion and deceptive pleural fluid biochemical indices.

Sinus cut-off sign: A helpful sign in the CT diagnosis of diaphragmatic rupture associated with pleural effusion

International Nuclear Information System (INIS)

Kaya, Seyda Ors; Karabulut, Nevzat; Yuncu, Gokhan; Sevinc, Serpil; Kiroglu, Yilmaz

2006-01-01

The objective of our study was to describe the 'sinus cut-off' sign at CT in the diagnosis of diaphragmatic rupture in patients with blunt abdominal trauma complicated with pleural effusion, and evaluate its utility in an experimental model. Between January 2004 and March 2005, we observed an unusual interruption of costophrenic sinus at CT in three patients with blunt abdominal trauma accompanied with pleural effusion. This observation prompted us to evaluate the utility of this sign in an experimental model. Laparotomically, we created 2 cm diapragmatic lacerations at each hemidiaphragm in two rabbits and pushed up the abdominal viscera with omentum through the defect. To simulate hemothorax, we also injected 5-10 mL of diluted contrast material into the pleural space. Using a dual-slice helical CT scanner, limited thoracoabdominal CT examination was performed before and after injection of intrapleural contrast material. The images were analyzed for the presence of CT signs for diaphragmatic injury. The left posterior costophrenic sulcus was interrupted in all of the three patients with left pleural effusion. While it was associated with other findings of diaphragmatic injury, the 'sinus cut-off sign' was the sole finding in one patient. The sinus cut-off sign was observed on the CT scans of 100% of the rabbits with a left and right sided diaphragmatic rupture. The 'sinus cut-off sign' is useful and can increase the CT detection of acute diaphragmatic injury associated with pleural effusion

Posterior diaphragmatic defect detected on chest CT: the incidence according to age and the lateral chest radiographic appearances

International Nuclear Information System (INIS)

Lee, Son Youl; Choi, Yo Won; Jeon, Seok Chol; Heo, Jeong Nam; Park, Choong Ki

2007-01-01

We wanted to investigate the incidence of posterior diaphragmatic defect on chest CT in various age groups and its lateral chest radiographic appearances. The chest CT scans of 78 patients of various ages with posterior diaphragmatic defect were selected among 1,991 patients, and they were analyzed for the incidence of defect in various age groups, the defect location and the herniated contents. Their lateral chest radiographs were analyzed for the shape of the posterior diaphragm and the posterior costophrenic sulcus. The patients' ages ranged from 34 to 87 with the tendency of a higher incidence in the older patients. The defect most frequently involved the medial two thirds (n = 49, 50.4%) and middle one third (n = 36, 37%) of the posterior diaphragm. The retroperitoneal fat was herniated into the thorax through the defect in all patients, and sometimes with the kidney (n = 8). Lateral chest radiography showed a normal diaphragmatic contour (n = 51, 49.5%), blunting of the posterior costophrenic sulcus (n = 41, 39.8%), focal humping of the posterior diaphragm (n = 7, 6.8%), or upward convexity (n = 4, 3.9%) of the posterior costophrenic sulcus on the affected side. The posterior diaphragmatic defect discovered in asymptomatic patients who are without a history of peridiaphragmatic disease is most likely acquired, and this malady increases in incidence according to age. An abnormal contour of the posterior diaphragm or the costophrenic sulcus on a lateral chest radiograph may be a finding of posterior diaphragmatic defect

Postmortem magnetic resonance appearances of congenital high airway obstruction syndrome

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Arthurs, Owen J. [Great Ormond Street Hospital for Children NHS Foundation Trust, Department of Radiology, London (United Kingdom); UCL Institute of Child Health, London (United Kingdom); Chitty, Lyn S. [UCL Institute of Child Health, Genetics and Genomic Medicine, London (United Kingdom); Great Ormond Street and UCLH NHS Foundation Trusts, London (United Kingdom); Judge-Kronis, Lydia [Great Ormond Street Hospital for Children NHS Foundation Trust, Department of Histopathology, London (United Kingdom); Sebire, Neil J. [UCL Institute of Child Health, London (United Kingdom); Great Ormond Street Hospital for Children NHS Foundation Trust, Department of Histopathology, London (United Kingdom)

2015-04-01

Congenital high airway obstruction syndrome (CHAOS) is a rare life-threatening condition characterised by complete or near-complete developmental obstruction of the foetal airway. Although antenatal imaging findings have been described, the postmortem MRI findings have not been reported. To present postmortem MRI features of CHAOS. We retrospectively reviewed our hospital pathology and imaging databases for cases of CHAOS over a 2-year period. We identified two cases of CHAOS. In both cases, postmortem plain radiographs demonstrated gross abdominal distension with distortion and splaying of the rib cage. Both foetuses had characteristic postmortem MRI findings including large-volume fluid-filled lungs on T2-weighted imaging, diaphragmatic eversion, fluid-filled airway dilatation below the level of obstruction, centrally positioned and compressed heart, and massive ascites. One foetus had an associated limb abnormality. Postmortem MRI in foetuses suspected of having CHAOS allows confirmation of the diagnosis, determination of the anatomical level of the atresia or stenosis, and identification of associated abnormalities without the need for invasive autopsy. (orig.)

Catamenial pneumothorax revealing diaphragmatic endometriosis: a case report and revue of literature.

Science.gov (United States)

Aissa, Sana; Benzarti, Wafa; Alimi, Faouzi; Gargouri, Imen; Salem, Halima Ben; Aissa, Amène; Fathallah, Khadija; Abdelkade, Atef Ben; Alouini, Rafika; Garrouche, Abdelhamid; Hayoun, Abdelaziz; Abdelghani, Ahmed; Benzarti, Mohamed

2017-01-01

Catamenial pneumothorax (CP) is a rare entity of spontaneous, recurring pneumothorax in women. We aim to discuss the etiology, clinical course, and surgical treatment of a 42-year-old woman with CP. This patient had a right-sided spontaneous pneumothoraces occurred one week after menses. She had under-gone video-assisted thoracoscopic surgery (VATS) because of a persistent air leak under chest tube. VATS revealed multiple diaphragmatic fenestrations with an upper right nodule. Defects were removed and a large part of the diaphragm was resected. Pleural abrasion was then performed over the diaphragm. Diaphragmatic endometriosis was confirmed by microscopic examination. Medical treatment with GnRH agonists was prescribed, and after recovery, the patient has been symptoms free for 20 months.

Using Imitation with Congenitally Deafblind Adults: Establishing Meaningful Communication Partnerships

Science.gov (United States)

Hart, Paul

2006-01-01

All congenitally deafblind people are potential communication partners. The key question for practitioners is how to help them achieve that potential. Imitation offers a particularly powerful means of doing so because it allows both partners to occupy a joint dyadic space, where the process of repairing the damaged communication partnerships that…

Congenital cystic adenomatoid malformations (CCAM) - prenatal MRI diagnosis: a case report

International Nuclear Information System (INIS)

Gagov, E.; Iieva, E.; Gvanska, G.

2012-01-01

Full text: Congenital Cystic Adenomatoid Malformation/CCAM (recently termed Congenital Pulmonary Airway Malformation/CPAM) is a rare lung lesion that is believed to result from a cessation of bronchiolar maturation with overgrowth of mesenchymal elements. The differential diagnosis of a mass in the fetal thorax includes CCAM/CPAM, congenital diaphragmatic hernia, and pulmonary sequestration. We present a case of CCAM/CPAM detected on prenatal ultrasound examination with prenatal magnetic resonance imaging (MRI) correlation. A multicystic lesion in the right hemithorax of the fetus was identified on ultrasound at 26th weeks of gestation which was slightly increasing in size on follow-up examinations. MRI was performed for further evaluation and selecting the appropriate management. On MRI the lesion was confined to the right middle lobe, consisting of a single large 3 cm cyst surrounded by multiple smaller cysts and compressed normal parenchyma of the upper and lower lobes of the right lung (type I, Stocker and al. classification). No mediastinal shift or other abnormalities to the contralateral lung were detected. No complications, such as hydrothorax or polyhydramnios were identified. Based on the MR findings postnatal surgical removal of the lung lesion was planned. Continuous weekly ultrasound follow-up examination was recommended. Improvements in magnetic resonance imaging (MRI) now permit diagnostic images of the fetus to be obtained. Ultrasound (US) remains vital in all aspects of fetal imaging but MR serves as a useful second line imaging test. MR imaging can provide excellent tissue contrast with more accurate analysis of the fetal anatomy and superior differentiation between the abnormalities and adjacent structures, thereby allowing early planning of pre- and postnatal management

Diaphragmatic rupture causing repeated vomiting in a combined abdominal and head injury patient: a case report and review of the literature

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Symeonidis Dimitrios

2012-07-01

Full Text Available Abstract Background Diaphragmatic rupture after blunt abdominal injury is a rare trauma condition. Delayed diagnosis is not uncommon especially in the emergency room setting. Associated injuries often shift diagnosis and treatment priorities towards other more life-threatening conditions. Case presentation We present a challenging case of a young male with combined abdominal and head trauma. Repeated episodes of vomiting dominated on clinical presentation that in the presence of a deep scalp laceration and facial bruising shifted differential diagnosis towards a traumatic brain injury. However, a computed tomography scan of the brain ruled out any intracranial pathology. Finally, a more meticulous investigation with additional imaging studies confirmed the presence of diaphragmatic rupture that justified the clinical symptoms. Conclusions The combination of diaphragmatic rupture with head injury creates a challenging trauma scenario. Increased level of suspicion is essential in order to diagnose timely diaphragmatic rupture in multiple trauma patients.

Choosing Between MRI and CT Imaging in the Adult with Congenital Heart Disease.

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Bonnichsen, Crystal; Ammash, Naser

2016-05-01

Improvements in the outcomes of surgical and catheter-based interventions and medical therapy have led to a growing population of adult patients with congenital heart disease. Adult patients with previously undiagnosed congenital heart disease or those previously palliated or repaired may have challenging echocardiographic examinations. Understanding the distinct anatomic and hemodynamic features of the congenital anomaly and quantifying ventricular function and valvular dysfunction plays an important role in the management of these patients. Rapid advances in imaging technology with magnetic resonance imaging (MRI) and computed tomography angiography (CTA) allow for improved visualization of complex cardiac anatomy in the evaluation of this unique patient population. Although echocardiography remains the most widely used imaging tool to evaluate congenital heart disease, alternative and, at times, complimentary imaging modalities should be considered. When caring for adults with congenital heart disease, it is important to choose the proper imaging study that can answer the clinical question with the highest quality images, lowest risk to the patient, and in a cost-efficient manner.

Intrathoracic Kidney after Blunt Abdominal Trauma: A Case Report and Review of the Literature

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Fikret Halis

2015-01-01

Full Text Available Abdominal trauma is responsible for most genitourinary injuries. The incidence of renal artery injury and intrathoracic kidney is quite low in patients who present with blunt trauma experiencing damage. There are four defined etiologies for intrathoracic kidney, which include real intrathoracic ectopic kidney, eventration of the diaphragm, congenital diaphragmatic herniation, and traumatic diaphragmatic rupture. The traumatic intrathoracic kidney is an extremely rare case. We presented intrathoracic kidney case after traumatic posterior diaphragmatic rupture.

The Use of Extracorporeal Membrane Oxygenation in the Surgical Repair of Bronchial Rupture

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Ju-Hee Park

2016-02-01

Full Text Available Extracorporeal membrane oxygenation (ECMO has been used successfully in critically ill patients with traumatic lung injury and offers an additional treatment modality. ECMO is mainly used as a bridge treatment to delayed surgical management; however, only a few case reports have presented the successful application of ECMO as intraoperative support during the surgical repair of traumatic bronchial injury. A 38-year-old man visited our hospital after a blunt chest trauma. His chest imaging showed hemopneumothorax in the left hemithorax and a finding suspicious for left main bronchus rupture. Bronchoscopy was performed and confirmed a tear in the left main bronchus and a congenital tracheal bronchus. We decided to provide venovenous ECMO support during surgery for bronchial repair. We successfully performed main bronchial repair in this traumatic patient with a congenital tracheal bronchus. We suggest that venovenous ECMO offers a good option for the treatment of bronchial rupture when adequate ventilation is not possible.

[Congenital cystic lung lesions--review of the literature with three clinical cases].

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Slancheva, B; Hitrova, S; Markov, D; Vakrilova, L; Pramatarova, T; Yarukova, N; Brankov, O

2013-01-01

Congenital cystic lung lesions are rare. Mainly affects the lower respiratory patishta.i are congenital cystic malformation and adematozna bronchopulmonary sequestration (BPS). The pathogenesis of the occurrence of these malformations is not clear but they have a common clinical course. In most cases, the anomaly is asymptomatic and occurs with infections of the lung during the first year of life. Currently congenital lung lesions were classified into five types and is considered by most authors. The anomaly is due to the abnormal proliferation of terminal bronchioles accompanied by inhibition of alveolar development between 7-17 weeks, obstructed airway dysplasia and metaplasia of normal lung tissue. Early diagnosis is vital in making a medical decision on how to treat CCAM. Associated with abnormalities of the urinary tract, cardiovascular system, gastrointestinal atresia, diaphragmatic hernia skeletal abnormalities. In pregnancies in which prenatal lung lesions weighs registered necessary series of ultrasound examinations to track finding and using the Doppler to assess how the blood supply of the fault. The clinical presentation of malformations is respiratory distress, respiratory infection, and dyspnea. The use of CT and MRA allows better visualization of the pulmonary lesions. With its combination with arteriography and bronchoscopy are used to differentiate CCAM and pulmonary sequestration. We present three cases with lung lesions were born in Neonatologia clinic at the University Hospital of Obstetrics and Gynecology "Maternity" Sofia for the period 2010-2012 three cases CCAMs type 1, operated by 5 meters after birth with a good final outcome without complications in the postoperative period and lack of pulmonary symptoms up to 1 year after birth.

Measurement of Diaphragmatic Blood Flow and Oxygen Consumption in the Dog by the Kety-Schmidt Technique

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Rochester, Dudley F.

1974-01-01

To assess energy expenditure of the diaphragm directly, a method was devised for percutaneous catheterization of the left inferior phrenic vein in dogs. Necropsy studies, including retrograde injection of india ink and measurement of radioactivity in diaphragmatic muscle strips, suggested that the territory drained by the inferior phrenic vein was uniformly perfused, and that there were no major anastomoses between this bed and adjacent ones. Diaphragmatic blood flow (˙Q di) was calculated from the integrated diaphragmatic arteriovenous difference of 85Kr by the Kety-Schmidt technique. Diaphragmatic oxygen consumption (˙Vo2 di) was determined as the product of ˙Q di and the diaphragmatic arteriovenous oxygen content difference [(A-V)O2 di]. When lightly anesthetized dogs breathed quietly, ˙Q di was 22±SD 6 ml/min/100 g, (A-V)O2 di was 6.1±SD 2.5 ml/100 ml, and ˙VO2 di averaged 1.2±SD 0.3 ml/min/100 g. This represented 1.0±SD 0.2% of total body oxygen consumption. ˙VO2 di remained relatively constant during quiet breathing, whereas ˙Q di varied directly with cardiac output and reciprocally with (A-V)O2 di. The oxygen consumption of the noncontracting diaphragm was 60±SD 20% of the level measured during quiet breathing. The energy expended by the diaphragm to support simple hyperventilation was small. A 100% increase in minute ventilation, induced by inhalation of 5% CO2 in 21% or 14% O2, increased ˙Q di 13%, (A-V)O2 di 19%, and ˙VO2 di 40%. The diaphragm consumed 0.13±SD 0.09 ml O2 for each additional liter of ventilation. In four dogs, pneumonia appeared to increase ˙VO2 both by increasing minute ventilation and by increasing the energy cost per liter of ventilation. PMID:4825221

Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome

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Mong, Andrew; Johnson, Ann M.; Kramer, Sandra S.; Jaramillo, Diego [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Coleman, Beverly G. [Hospital of the University of Pennsylvania, Department of Radiology, Philadelphia, PA (United States); Hedrick, Holly L.; Flake, Alan; Johnson, Mark; Wilson, R.D.; Adzick, N.S. [Children' s Hospital of Philadelphia, The Center for Fetal Diagnosis and Treatment, Philadelphia, PA (United States); Kreiger, Portia [Children' s Hospital of Philadelphia, Department of Pathology and Laboratory Medicine, Philadelphia, PA (United States)

2008-11-15

Congenital high airway obstruction syndrome (CHAOS) is a rare disorder defined as any fetal abnormality that obstructs the larynx or trachea. Prompt airway intervention at delivery after accurate prenatal diagnosis may allow survival of this otherwise fatal condition. To identify prenatal MRI findings in CHAOS, to compare these findings with those of fetal US, to determine if imaging alters diagnosis and management decisions, and to correlate prenatal with postnatal imaging findings. Records and MRI scans of ten fetuses with CHAOS were reviewed, and the findings correlated with outside and same-day fetal US and postnatal imaging findings. Fetal lung volumes were measured on MRI scans. Large lung volumes were found in 90% of the fetuses. Increased lung signal intensity, inverted diaphragm, and a dilated, fluid-filled lower airway were identified in all. The obstruction level was identified in 90%. MRI changed screening US diagnosis in 70%, but was concordant with the tertiary care US imaging in 90%. Seven fetuses were terminated or died in utero, and three fetuses survived after ex utero intrapartum tracheostomy placement. Autopsy or bronchoscopy performed in 60% confirmed CHAOS. Postnatal chest radiographs and CT showed hyperinflation, while US and fluoroscopy showed diminished diaphragmatic motion. MRI demonstrates large lung volumes, increased lung signal intensity, inverted diaphragm, and dilated fluid-filled lower airway, and usually identifies the obstruction level. The degree of correlation between MRI and tertiary prenatal US is high, but CHAOS is frequently misdiagnosed on screening US. Correct diagnosis may enable planned airway management. Voluminous lungs and diaphragmatic abnormalities persist on postnatal imaging. (orig.)

Congenital high airway obstruction syndrome: MR/US findings, effect on management, and outcome

International Nuclear Information System (INIS)

Mong, Andrew; Johnson, Ann M.; Kramer, Sandra S.; Jaramillo, Diego; Coleman, Beverly G.; Hedrick, Holly L.; Flake, Alan; Johnson, Mark; Wilson, R.D.; Adzick, N.S.; Kreiger, Portia

2008-01-01

Congenital high airway obstruction syndrome (CHAOS) is a rare disorder defined as any fetal abnormality that obstructs the larynx or trachea. Prompt airway intervention at delivery after accurate prenatal diagnosis may allow survival of this otherwise fatal condition. To identify prenatal MRI findings in CHAOS, to compare these findings with those of fetal US, to determine if imaging alters diagnosis and management decisions, and to correlate prenatal with postnatal imaging findings. Records and MRI scans of ten fetuses with CHAOS were reviewed, and the findings correlated with outside and same-day fetal US and postnatal imaging findings. Fetal lung volumes were measured on MRI scans. Large lung volumes were found in 90% of the fetuses. Increased lung signal intensity, inverted diaphragm, and a dilated, fluid-filled lower airway were identified in all. The obstruction level was identified in 90%. MRI changed screening US diagnosis in 70%, but was concordant with the tertiary care US imaging in 90%. Seven fetuses were terminated or died in utero, and three fetuses survived after ex utero intrapartum tracheostomy placement. Autopsy or bronchoscopy performed in 60% confirmed CHAOS. Postnatal chest radiographs and CT showed hyperinflation, while US and fluoroscopy showed diminished diaphragmatic motion. MRI demonstrates large lung volumes, increased lung signal intensity, inverted diaphragm, and dilated fluid-filled lower airway, and usually identifies the obstruction level. The degree of correlation between MRI and tertiary prenatal US is high, but CHAOS is frequently misdiagnosed on screening US. Correct diagnosis may enable planned airway management. Voluminous lungs and diaphragmatic abnormalities persist on postnatal imaging. (orig.)

Vincent Alexander Bochdalek (1801-1883).

Science.gov (United States)

Loukas, Marios; El-Sedfy, Abraham; Tubbs, R Shane; Gribben, Walter B; Shoja, Mohammadali M; Cermakova, Andrea

2008-10-01

Vincent Alexander Bochdalek, Czech anatomist and professor, was one of the pioneers in describing congenital diaphragmatic hernias in newborns. Interestingly, there is very little in the literature and almost nothing in the English literature regarding this important medical figure. A dedicated individual, Bochdalek had great perseverance, which allowed him to surpass obstacles in both his personal life and career. He is recognized for his accurate description of posterior diaphragmatic herniation, for which during his lifetime, there was no treatment. In addition, Bochdalek has multiple eponyms in his honor: ganglion Bochdalecki, Bochdalek's basket, and the valves of Bochdalek. Always steadfast in his beliefs, Bochdalek fought for the creation of anatomy labs in Prague against society pressures. His devoted study of anatomy, especially of congenital diaphragmatic hernias, laid the foundation for the successful surgical correction of this once lethal anomaly.

Concurrent Mesh Repair of a Morgagni and Umbilical Hernia during a Laparoscopic Sleeve Gastrectomy in a Morbidly Obese Individual

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N.R Kosai

2016-10-01

Full Text Available Morgagni Hernia is a rare form of diaphragmatic hernia. It is mainly asymptomatic and often identified incidentally during surgery. Tension-free synthetic mesh repair is the preferred treatment modality. However, the use of synthetic mesh concurrently during a clean-contaminated surgery such as sleeve gastrectomy remains controversial due to the remote possibility of mesh infection. A middle-aged female 2 with BMI of 47 Kg/m was admitted electively for laparoscopic sleeve gastrectomy with concurrent umbilical hernia repair. Intra-operatively, a left Morgagni Hernia containing omentum and a segment of transverse colon was noted. She underwent a laparoscopic sleeve gastrectomy and simultaneous laparoscopic tension-free composite mesh repair of both Morgagni and umbilical hernia. Outpatient review three months later revealed excess weight loss of almost 30% with no recurrence of either hernia. In conclusion, the advantages of concurrent hernia repair during bariatric surgery outweigh the risk of mesh infection and should be performed to prevent future risk of visceral herniation and strangulation. Laparoscopic mesh repair of a Morgagni Hernia and umbilical hernia in the setting of an electively planned sleeve gastrectomy is feasible, effective and safe in the hands of a trained laparoscopic surgeon.

A rare case of diaphragmatic paralysis due to isolated phrenic nerve ...

African Journals Online (AJOL)

2008-11-04

Nov 4, 2008 ... Isolated phrenic nerve palsy is a rare condition resulting from birth injury, with many possible complications such as diaphragmatic paralysis, pulmonary infection, chronic lung disease, growth failure and even death.1-3 I report a case of neonatal isolated phrenic nerve palsy in an infant delivered.

New aspects of anesthetic management in congenital heart disease "common arterial trunk"

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Mohsen Ziyaeifard

2014-01-01

Full Text Available Now-a-days truncus arteriosus has been known as "common arterial trunk" (CAT and is an uncommon congenital cardiac defect presenting in about 1-3% congenital heart disease. Environmental and genetic factors effects on incidence of CAT and other conotruncal anomalies. The majority patients with CAT and 22q11 deletion have other anomalies such as hypoplasia or aplasia of the thymus or parathyroid glands and immune deficits (T-cell deficiency, calcium metabolism disorder (hypocalcemia, palatal defects, learning and speech disorder, craniofacial anomalies, and neuropsychological abnormalities. CAT without surgical treatment frequently involves early severe pulmonary arterial hypertension (PAH or early death from heart failure and associated conditions. Therefore, without corrective surgical repair, most CAT patients die in the initial years of life. In numerous centers early surgical repair associated with superior than 80% long-standing survival. Anesthesiologist must be performs comprehensive preoperative evaluation of infants or neonates with this disorder. In CAT patient exactly hemodynamic monitoring and suitable techniques to regulate pulmonary vascular resistance and systemic vascular resistance and cardiac function are more important than the select of a special anesthetic drug. Therefore, anesthetic drugs should be carefully administrated and titrate and under monitoring. Management of CAT after surgical repair depends on the adequacy of treatment, cardiac function, level of PAH, and degree of bleeding. Inotropic support is frequently necessary after the cardiac ischemia associated to the surgical repair. Pulmonary vasodilator drugs were used to PAH treatment.

Minimally Invasive Epicardial Pacemaker Implantation in Neonates with Congenital Heart Block.

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Costa, Roberto; Silva, Katia Regina da; Martinelli Filho, Martino; Carrillo, Roger

2017-10-01

Few studies have characterized the surgical outcomes following epicardial pacemaker implantation in neonates with congenital complete atrioventricular block (CCAVB). This study sought to assess the long-term outcomes of a minimally invasive epicardial approach using a subxiphoid access for pacemaker implantation in neonates. Between July 2002 and February 2015, 16 consecutive neonates underwent epicardial pacemaker implantation due to CCAVB. Among these, 12 (75.0%) had congenital heart defects associated with CCAVB. The patients had a mean age of 4.7 ± 5.3 days and nine (56.3%) were female. Bipolar steroid-eluting epicardial leads were implanted in all patients through a minimally invasive subxiphoid approach and fixed on the diaphragmatic ventricular surface. The pulse generator was placed in an epigastric submuscular position. All procedures were successful, with no perioperative complications or early deaths. Mean operating time was 90.2 ± 16.8 minutes. None of the patients displayed pacing or sensing dysfunction, and all parameters remained stable throughout the follow-up period of 4.1 ± 3.9 years. Three children underwent pulse generator replacement due to normal battery depletion at 4.0, 7.2, and 9.0 years of age without the need of ventricular lead replacement. There were two deaths at 12 and 325 days after pacemaker implantation due to bleeding from thrombolytic use and progressive refractory heart failure, respectively. Epicardial pacemaker implantation through a subxiphoid approach in neonates with CCAVB is technically feasible and associated with excellent surgical outcomes and pacing lead longevity.

Changing Landscape of Congenital Heart Disease.

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Bouma, Berto J; Mulder, Barbara J M

2017-03-17

Congenital heart disease is the most frequently occurring congenital disorder affecting ≈0.8% of live births. Thanks to great efforts and technical improvements, including the development of cardiopulmonary bypass in the 1950s, large-scale repair in these patients became possible, with subsequent dramatic reduction in morbidity and mortality. The ongoing search for progress and the growing understanding of the cardiovascular system and its pathophysiology refined all aspects of care for these patients. As a consequence, survival further increased over the past decades, and a new group of patients, those who survived congenital heart disease into adulthood, emerged. However, a large range of complications raised at the horizon as arrhythmias, endocarditis, pulmonary hypertension, and heart failure, and the need for additional treatment became clear. Technical solutions were sought in perfection and creation of new surgical techniques by developing catheter-based interventions, with elimination of open heart surgery and new electronic devices enabling, for example, multisite pacing and implantation of internal cardiac defibrillators to prevent sudden death. Over time, many pharmaceutical studies were conducted, changing clinical treatment slowly toward evidence-based care, although results were often limited by low numbers and clinical heterogeneity. More attention has been given to secondary issues like sports participation, pregnancy, work, and social-related difficulties. The relevance of these issues was already recognized in the 1970s when the need for specialized centers with multidisciplinary teams was proclaimed. Finally, research has become incorporated in care. Results of intervention studies and registries increased the knowledge on epidemiology of adults with congenital heart disease and their complications during life, and at the end, several guidelines became easily accessible, guiding physicians to deliver care appropriately. Over the past decades

Cleft Palate Repair Using a Double Opposing Z-Plasty.

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Moores, Craig; Shah, Ajul; Steinbacher, Derek M

2016-07-01

Cleft palate is a common congenital defect with several described surgical repairs. The most successful treatment modality remains a controversy. The goals of repair focus on achievement of normal speech and optimizing velopharyngeal function while minimizing both fistula formation and facial growth restriction. In this video, the authors demonstrate use of the double opposing Z-plasty technique in the repair of a Veau II type cleft palate. The video demonstrates the marking, incisions, dissection, and repair of the cleft. It also examines the use of von Langenbeck-type relaxing incisions and demonstrates a specific approach to the repair of this particular cleft. The authors believe that the Furlow double opposing Z-plasty with the von Langenbeck relaxing incision can provide the best postoperative outcome by combining the benefits of each individual operation. The Z-plasty technique works to correct the aberrant muscle of the soft palate while increasing the length of the palate. The authors believe that this results in better velopharyngeal function.

ISSN 2073 ISSN 2073-9990 East Cent. Afr. J. s 9990 East Cent. Afr.

African Journals Online (AJOL)

Hp 630 Dual Core

2016-08-01

Aug 1, 2016 ... Congenital Diaphragmatic Hernia Outcomes in East frica: The Ethiopian Experience. Miliard Derbew ... One child was diagnosed with congenital pulmonary airway malformation (CPAM). In total, 10 (83.3%) of our patients were initially misdiagnosed. Only one patient .... Superficial Surgical Site infection. 2.

Traumatic Pulmonary Herniation at the Diaphragmatic Junction in a Pediatric Patient: A Rare Complication of Blunt Chest Trauma.

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Orlik, Kseniya; Simon, Erin Leslie; Hemmer, Carrie; Ramundo, Maria

2016-07-01

We present a case of traumatic intercostal pulmonary herniation in an 11-year-old boy after blunt trauma to the chest, without associated chest wall disruption or pneumothorax. This condition is especially uncommon in children, with only 5 previously reported cases and most occurring after penetrating chest trauma. To date, there are no reports in literature describing traumatic intercostal lung herniation at the diaphragmatic junction with a closed chest cavity in a child. The number of traumatic lung herniation diagnoses may be expanded by a more liberal use of computed tomography when serious injury is suspected. Computed tomography and advanced imaging should be considered in pediatric trauma patients presenting with concern for intrathoracic injury that may not be seen on plain film. Traumatic blunt intrathoracic and intra-abdominal injuries in the pediatric population that are within proximity of diaphragmatic insertion should be thoroughly evaluated to rule out diaphragmatic injury. As in our case, invasive surgical intervention such as thoracoscopy may be necessary.

Comparison of 3D Echocardiogram-Derived 3D Printed Valve Models to Molded Models for Simulated Repair of Pediatric Atrioventricular Valves.

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Scanlan, Adam B; Nguyen, Alex V; Ilina, Anna; Lasso, Andras; Cripe, Linnea; Jegatheeswaran, Anusha; Silvestro, Elizabeth; McGowan, Francis X; Mascio, Christopher E; Fuller, Stephanie; Spray, Thomas L; Cohen, Meryl S; Fichtinger, Gabor; Jolley, Matthew A

2018-03-01

Mastering the technical skills required to perform pediatric cardiac valve surgery is challenging in part due to limited opportunity for practice. Transformation of 3D echocardiographic (echo) images of congenitally abnormal heart valves to realistic physical models could allow patient-specific simulation of surgical valve repair. We compared materials, processes, and costs for 3D printing and molding of patient-specific models for visualization and surgical simulation of congenitally abnormal heart valves. Pediatric atrioventricular valves (mitral, tricuspid, and common atrioventricular valve) were modeled from transthoracic 3D echo images using semi-automated methods implemented as custom modules in 3D Slicer. Valve models were then both 3D printed in soft materials and molded in silicone using 3D printed "negative" molds. Using pre-defined assessment criteria, valve models were evaluated by congenital cardiac surgeons to determine suitability for simulation. Surgeon assessment indicated that the molded valves had superior material properties for the purposes of simulation compared to directly printed valves (p 3D echo-derived molded valves are a step toward realistic simulation of complex valve repairs but require more time and labor to create than directly printed models. Patient-specific simulation of valve repair in children using such models may be useful for surgical training and simulation of complex congenital cases.

Case report: Non-invasive neurally adjusted ventilatory assist in a newborn with unilateral diaphragmatic paralysis.

Science.gov (United States)

Roosens, Sander; Derriks, Frank; Cools, Filip

2016-11-01

Diaphragmatic paralysis is a rare cause of respiratory distress in the newborn. In this paper, a patient with unilateral phrenic nerve injury after traumatic delivery is presented. The child inadequately responded to standard respiratory supportive measures. Non-invasive neurally adjusted ventilatory assist (NIV-NAVA®), providing an optimally synchronized respiratory support proportional to the effort of the patient, resulted in prompt clinical and biological improvement of the patient's respiratory condition. NAVA is a relatively new mode of ventilation in neonatal care. In this case of unilateral diaphragmatic paralysis, it provided an alternative strategy of non-invasive respiratory support avoiding prolonged mechanical ventilation. Pediatr Pulmonol. 2016;51:E37-E39. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Idiopathic diaphragmatic paralysis: Bell's palsy of the diaphragm?

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Crausman, Robert S; Summerhill, Eleanor M; McCool, F Dennis

2009-01-01

Idiopathic diaphragm paralysis is probably more common and responsible for more morbidity than generally appreciated. Bell's palsy, or idiopathic paralysis of the seventh cranial nerve, may be seen as an analogous condition. The roles of zoster sine herpete and herpes simplex have increasingly been recognized in Bell's palsy, and there are some data to suggest that antiviral therapy is a useful adjunct to steroid therapy. Thus, we postulated that antiviral therapy might have a positive impact on the course of acute idiopathic diaphragm paralysis which is likely related to viral infection. Three consecutive patients with subacute onset of symptomatic idiopathic hemidiaphragm paralysis were empirically treated with valacyclovir, 1,000 mg twice daily for 1 week. Prior to therapy, diaphragmatic function was assessed via pulmonary function testing and two-dimensional B-mode ultrasound, with testing repeated 1 month later. Diaphragmatic function pre- and post-treatment was compared to that of a historical control group of 16 untreated patients. All three subjects demonstrated ultrasound recovery of diaphragm function 4-6 weeks following treatment with valacyclovir. This recovery was accompanied by improvements in maximum inspiratory pressure (PI(max)) and vital capacity (VC). In contrast, in the untreated cohort, diaphragm recovery occurred in only 11 subjects, taking an average of 14.9 +/- 6.1 months (mean +/- SD). The results of this small, preliminary study suggest that antiviral therapy with valacyclovir may be helpful in the treatment of idiopathic diaphragm paralysis induced by a viral infection.

Congenital Upper Eyelid Coloboma: Clinical and Surgical Management.

Science.gov (United States)

Ortega Molina, José María; Mora Horna, Eduardo Ramón; Salgado Miranda, Andrés David; Rubio, Rosa; Solans Pérez de Larraya, Ana; Salcedo Casillas, Guillermo

2015-01-01

Purpose. The goal was to describe our experience in the surgical management and treatment of four patients with congenital upper eyelid colobomas. Methods. A descriptive, observational, retrospective study was performed including patients with congenital eyelid colobomas referred to Asociación para Evitar la Ceguera en México I.A.P. "Dr. Luis Sánchez Bulnes" between 2004 and 2014 and assessed by the Oculoplastics and Orbit Service. Results. The four cases required surgical treatment of the eyelid defects before one year of age and their evolution was monitored from the time of referral to the present day. One of the patients needed a second surgical procedure to repair the eyelid defect and correct the strabismus. Conclusions. Eyelid colobomas are a potential threat to vision at an early age, which requires close monitoring of the visual development of patients.

Syndromes and Disorders Associated with Omphalocele (III: Single Gene Disorders, Neural Tube Defects, Diaphragmatic Defects and Others

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Chih-Ping Chen

2007-06-01

Full Text Available Omphalocele can be associated with single gene disorders, neural tube defects, diaphragmatic defects, fetal valproate syndrome, and syndromes of unknown etiology. This article provides a comprehensive review of omphalocele-related disorders: otopalatodigital syndrome type II; Melnick–Needles syndrome; Rieger syndrome; neural tube defects; Meckel syndrome; Shprintzen–Goldberg omphalocele syndrome; lethal omphalocele-cleft palate syndrome; cerebro-costo-mandibular syndrome; fetal valproate syndrome; Marshall–Smith syndrome; fibrochondrogenesis; hydrolethalus syndrome; Fryns syndrome; omphalocele, diaphragmatic defects, radial anomalies and various internal malformations; diaphragmatic defects, limb deficiencies and ossification defects of skull; Donnai–Barrow syndrome; CHARGE syndrome; Goltz syndrome; Carpenter syndrome; Toriello–Carey syndrome; familial omphalocele; Cornelia de Lange syndrome; C syndrome; Elejalde syndrome; Malpuech syndrome; cervical ribs, Sprengel anomaly, anal atresia and urethral obstruction; hydrocephalus with associated malformations; Kennerknecht syndrome; lymphedema, atrial septal defect and facial changes; and craniosynostosis- mental retardation syndrome of Lin and Gettig. Perinatal identification of omphalocele should alert one to the possibility of omphalocele-related disorders and familial inheritance and prompt a thorough genetic counseling for these disorders.

Prenatal tracheal ligation or intra-amniotic administration of surfactant or dexamethasone prevents some structural changes in the pulmonary arteries of surgically created diaphragmatic hernia in rabbits Ligadura de traquéia no período pré-natal ou administração intra-amniótica de surfactante ou dexametasona evitam algumas alterações estruturais nas artérias pulmonares de fetos de coelho com hérnia diafragmática congênita produzida com cirurgia

Directory of Open Access Journals (Sweden)

Consuelo J. Rodrigues

2002-02-01

Full Text Available PURPOSE: Characterization of the structural changes occurring in the pulmonary arteries resulting from surgically produced congenital diaphragmatic hernia in rabbits, with particular emphasis on the preventive effects of prenatal tracheal ligation or administration of intra-amniotic dexamethasone or surfactant. METHODS: Twenty rabbit fetuses underwent surgical creation of a left-sided congenital diaphragmatic hernia on the 24th or 25th gestational day. They were divided according to the following procedures: congenital diaphragmatic hernia (n = 5, congenital diaphragmatic hernia plus tracheal ligation (n = 5, congenital diaphragmatic hernia plus intra-amniotic administration of dexamethasone 0.4 mg (n = 5 or surfactant (Curosurf 40 mg, n = 5. On gestational day 30, all the fetuses were delivered by caesarean section and killed. A control group consisted of five nonoperated fetuses. Histomorphometric analysis of medial thickness, cell nuclei density, and elastic fiber density of pulmonary arterial walls was performed. RESULTS: Arteries with an external diameter > 100 mum have a decreased medial thickness, lower cell nuclei density, and greater elastic fiber density when compared with arteries with external diameter 100 mum. Prenatal treatments with tracheal ligation or intra-amniotic administration of dexamethasone or surfactant prevented these changes. In arteries with external diameter OBJETIVO: Caracterização das alterações estruturais que ocorrem nas artérias pulmonares de fetos de coelho com hérnia diafragmática congênita produzida com cirurgia, com destaque especial aos efeitos preventivos da ligadura de traquéia ou administração intra-amniótica de dexametasona ou surfactante. MÉTODOS: Vinte fetos de coelho foram submetidos a cirurgia para produção de hérnia diafragmática no 24º ou 25º dia de gestação. Os animais foram divididos de acordo com os procedimentos: hérnia diafragmática congênita (n = 5, hérnia diafragm

A New Technique for Reconstruction of the Tricuspid Valve Ring in Congenital Tricuspid Insufficiency

OpenAIRE

Montero, C. G.; Tellez, G.; Simarro, E.; Juffé, A.; Amau, J. G.; Gurgos, R.; Figuera, D.; Cortina, A.

1983-01-01

A 50-year-old man was treated for a dysplasia-type congenital tricuspid insufficiency. The tricuspid valve ring was repaired with a perforated Teflon patch graft and the valve was replaced. The patient was discharged in good condition and is well after 18 months of follow-up.

Extended endoscopic endonasal surgery using three-dimensional endoscopy in the intra-operative MRI suite for supra-diaphragmatic ectopic pituitary adenoma.

Science.gov (United States)

Fuminari, Komatsu; Hideki, Atsumi; Manabu, Osakabe; Mitsunori, Matsumae

2015-01-01

We describe a supra-diaphragmatic ectopic pituitary adenoma that was safely removed using the extended endoscopic endonasal approach, and discuss the value of three-dimensional (3D) endoscopy and intra-operative magnetic resonance imaging (MRI) to this type of procedure. A 61-year-old-man with bitemporal hemianopsia was referred to our hospital, where MRI revealed an enhanced suprasellar tumor compressing the optic chiasma. The tumor extended on the planum sphenoidale and partially encased the right internal carotid artery. An endocrinological assessment indicated normal pituitary function. The extended endoscopic endonasal approach was taken using a 3D endoscope in the intraoperative MRI suite. The tumor was located above the diaphragma sellae and separated from the normal pituitary gland. The pathological findings indicated non-functioning pituitary adenoma and thus the tumor was diagnosed as a supra-diaphragmatic ectopic pituitary adenoma. Intra-operative MRI provided useful information to minimize dural opening and the supra-diaphragmatic ectopic pituitary adenoma was removed from the complex neurovascular structure via the extended endoscopic endonasal approach under 3D endoscopic guidance in the intra-operative suite. Safe and effective removal of a supra-diaphragmatic ectopic pituitary adenoma was accomplished via the extended endoscopic endonasal approach with visual information provided by 3D endoscopy and intra-operative MRI.

[Tissular expansion in giant congenital nevi treatment].

Science.gov (United States)

Nguyen Van Nuoi, V; Francois-Fiquet, C; Diner, P; Sergent, B; Zazurca, F; Franchi, G; Buis, J; Vazquez, M-P; Picard, A; Kadlub, N

2014-08-01

Surgical management of giant melanotic naevi remains a surgical challenge. Tissue expansion provides tissue of the same quality for the repair of defects. The aim of this study is to review tissular expansion for giant melanotic naevi. We conducted a retrospective study from 2000 to 2012. All children patients who underwent a tissular expansion for giant congenital naevi had been included. Epidemiological data, surgical procedure, complication rate and results had been analysed. Thirty-tree patients had been included; they underwent 61 procedures with 79 tissular-expansion prosthesis. Previous surgery, mostly simple excision had been performed before tissular expansion. Complete naevus excision had been performed in 63.3% of the cases. Complications occurred in 45% of the cases, however in 50% of them were minor. Iterative surgery increased the complication rate. Tissular expansion is a valuable option for giant congenital naevus. However, complication rate remained high, especially when iterative surgery is needed. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

CASE REPORT OF SEVERE PROLIFERATIVE RETINOPATHY IN A PATIENT WITH CONGENITAL LIPODYSTROPHY.

Science.gov (United States)

Rosin, Boris; Jaouni, Tareq

2017-08-21

A case report of a patient with severe proliferative retinopathy due to congenital lipodystrophy. We reviewed the medical history, imaging, and surgical procedures of a 25-year-old woman with a history of congenital lipodystrophy, presenting with bilateral combined tractional and exudative retinal detachment, poorly controlled diabetes mellitus, and extreme dislipidemia. The patient underwent retinal detachment repair surgery both eyes. On the last follow-up, both retinae were flat, and visual acuity had improved in the right eye to J3 for near and finger counting 3 m for distance. Surgery combining pars plana vitrectomy and scleral bucking successfully flattened both retinae and significantly improved visual acuity in one eye in this case of bilateral retinal detachment with combined tractional and exudative components in a patient with congenital lipodystrophy. Surgical control of retinal complications is thus possible, provided there is adequate control of the underlying risk factors.

Blunt bilateral diaphragmatic rupture—A right side can be easily missed

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Maria Michailidou

2015-12-01

Full Text Available Blunt diaphragmatic rupture (BDR is uncommon with a reported incidence range of 1%–2%. The true incidence is not known. Bilateral BDR is particularly rare. We presented a case of bilateral BDR and we think that the incidence is under-recognised thanks to an easily missed and difficult to diagnose right sided injury. Keywords: Blunt, Diaphragm, Bilateral, Injury

[Results of the treatment of congenital diaphagmatic hernia with conventional terapeutics modalities].

Science.gov (United States)

Luis, A L; Avila, L F; Encinas, J L; Andrés, A M; Suárez, O; Elorza, D; Rodríguez, I; Martínez, L; Murcia, J; Lassaletta, L; Tovar, J A

2006-07-01

In this study, we analyze our results in the treatment of congenital diaphragmatic hernia (CDH) using conventional ventilatory therapy without ECMO. fifty one CDH patients (27 males and 24 females) treated at our institution between 1997 and 2004 were reviewed. Data referred to obstetrics, prenatal diagnosis, newborn care, type of hernia and surgical treatment were analyzed. We recorded ventilatory treatment modalities and the outcome of the patients. We also compared the survival of our series with those expected using the formula proposed by the CDH study group in 2001. We finally analized separately those patients with early clinical presentation and who fulfilled ECMO criteria. Data from necropsies were also recovered when available. Prenatal diagnosis was made in 58% of the patients. Fifty nine percent were born by c-section. The diaphragmatic defect was left-sided in 42 patients, right in 8 and bilateral in 1. Ten patients needed a prosthetic patch to close the defect. Eighteen out of the 51 patients (35%) died, 11 of them without surgical treatment. Early presentation of clinical picture was evident in 44 patients; among them 46% required high frequency ventilation and 53% nitric oxide therapy. Medium age at operation was 56+/-49 hours. The 7 children with late clinical presentation are alive. Among the 44 remaining patients, 26 are also alive (59,09%), data similar to those expected by the formula (62.39%, p>0.05). Fifteen patients had oxigenation index (IO) over 40, with a stimated survival rate of 48%, a statistically significant lower rate when compared to patients with IO40 survived; in 4 out of the 7 available necropsy studies, a severe lung hypoplasia was found (index lung weight/body weight ventilatory measures in patients with IO40 are rarely candidates to ECMO therapy because of the associated severe lung hypoplasia confirmed by necropsy studies.

Management of patients with pulmonary arterial hypertension due to congenital heart disease: recent advances and future directions

NARCIS (Netherlands)

Blok, Ilja M.; van Riel, Annelieke C. M. J.; Mulder, Barbara J. M.; Bouma, Berto J.

2015-01-01

Pulmonary arterial hypertension is a serious complication of adult congenital heart disease associated with systemic-to-pulmonary shunts. Although early shunt closure restricts development of pulmonary arterial hypertension, patients remain at risk even after repair. The development of pulmonary

Vasopressin in perioperative management of congenital ...

African Journals Online (AJOL)

Annals of Pediatric Surgery ... Oxygenation failure, pulmonary hypertension and refractory systemic hypotension in infants with ... managing pulmonary and systemic perioperative haemodynamic instability in infants with diaphragmatic hernia.

Morgagni hernia presenting with lung consolidation unresponsive to ...

African Journals Online (AJOL)

Congenital diaphragmatic hernia (CDH) is a congenital malformation of the diaphragm that allows the abdominal organs to push into the chest cavity. We report the case of a 15-month-old patient who presented with a non-resolving opacity on a chest radiograph despite extensive antibiotic treatment. A large anterior ...

Variation in Perfusion Strategies for Neonatal and Infant Aortic Arch Repair: Contemporary Practice in the STS Congenital Heart Surgery Database.

Science.gov (United States)

Meyer, David B; Jacobs, Jeffrey P; Hill, Kevin; Wallace, Amelia S; Bateson, Brian; Jacobs, Marshall L

2016-09-01

Regional cerebral perfusion (RCP) is used as an adjunct or alternative to deep hypothermic circulatory arrest (DHCA) for neonates and infants undergoing aortic arch repair. Clinical studies have not demonstrated clear superiority of either strategy, and multicenter data regarding current use of these strategies are lacking. We sought to describe the variability in contemporary practice patterns for use of these techniques. The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2013) was queried to identify neonates and infants whose index operation involved aortic arch repair with cardiopulmonary bypass. Perfusion strategy was classified as isolated DHCA, RCP (with less than or equal to ten minutes of DHCA), or mixed (RCP with more than ten minutes of DHCA). Data were analyzed for the entire cohort and stratified by operation subgroups. Overall, 4,523 patients (105 centers) were identified; median age seven days (interquartile range: 5.0-13.0). The most prevalent perfusion strategy was RCP (43%). Deep hypothermic circulatory arrest and mixed perfusion accounted for 32% and 16% of cases, respectively. In all, 59% of operations involved some period of RCP. Regional cerebral perfusion was the most prevalent perfusion strategy for each operation subgroup. Neither age nor weight was associated with perfusion strategy, but reoperations were less likely to use RCP (31% vs 45%, P RCP and DHCA in the RCP group was longer than the DHCA time in the DHCA group (45 vs 36 minutes, P neonates and infants. In contemporary practice, RCP is the most prevalent perfusion strategy for these procedures. Use of DHCA is also common. Further investigation is warranted to ascertain possible relative merits of the various perfusion techniques. © The Author(s) 2016.

Video-assisted thoracoscopy for diaphragmatic plication: experimental study in a canine model.

Science.gov (United States)

Gonzalez-Zamora, Jose F; Perez-Guille, Beatriz; Soriano-Rosales, Rosa E; Jimenez-Bravo-Luna, Miguel A; Gutierrez-Castrellon, Pedro; Ridaura-Sanz, Cecilia; Alvarez, Fernando Villegas

2005-12-01

Plication of a nonfunctional hemidiaphragm usually restores altered ventilatory mechanics. This study compared two techniques in performing diaphragmatic plication: video-assisted thoracoscopy (group A) and thoracotomy (group B). Twenty dogs with induced paralysis of the right hemidiaphragm were randomly assigned to one of the two groups. Evaluations were performed before and after plication of the respiratory frequency (f) and lung area (LA) of the affected side. Operative time, time to resumption of walking, ingestion of fluids and solid food, pain intensity, and postoperative complications were measured. Group A had less pain after the surgery (P fluid ingestion (P < 0.05), and earlier resumption of walking (P < 0.019). Four weeks after the procedure, LA was similar in both groups, while a significant decrease in f was recorded in group A (P < 0.02). The remaining evaluated variables showed no differences. Both approaches were effective. Pain recorded in the postoperative period was less and recovery was faster in group A. Complications and surgical times were similar. The video-assisted thoracoscopy is a safe and efficient option for performing diaphragmatic plication in dogs.

Normal appearance and size of the diaphragmatic crura in children: CT evaluation

International Nuclear Information System (INIS)

Brengle, M.; Cohen, M.D.; Katz, B.

1996-01-01

Purpose. The objectives of the study were to document the normal CT appearance and size of the crura of the diaphragm in children. Materials and methods. The CT scans of 80 children (0-15 years) were reviewed. The children were divided into eight age groups. The maximal transverse diameters of the right and left crura were measured. They were normalized by comparison with the transverse and anterior-posterior diameters of the 12th thoracic vertebra (T12) and the transverse abdominal diameter at T12. The crura were also evaluated as to whether their contour was smooth or nodular. Results. The diaphragmatic crura of smaller children appear large, relative to body size and the diameters of the T12 vertebral body, compared with those in older children. Crural width does not increase significantly with age. Additionally, the crura were found to have a greater tendency to be nodular in appearance in children under the age of 5 years than in older children. Conclusion. Diaphragmatic crura are more nodular and larger relative to body size in younger children. (orig.). With 6 figs., 2 tabs

Management of the Sequelae of Severe Congenital Abdominal Wall Defects

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Sara Fuentes

2016-05-01

Full Text Available BackgroundThe survival rate of newborns with severe congenital abdominal wall defects has increased. After successfully addressing life-threatening complications, it is necessary to focus on the cosmetic and functional outcomes of the abdominal wall.MethodsWe performed a chart review of five cases treated in our institution.ResultsFive patients, ranging from seven to 18 years of age, underwent the following surgical approaches: simple approximation of the rectus abdominis fascia, the rectus abdominis sheath turnover flap, the placement of submuscular tissue expanders, mesh repair, or a combination of these techniques depending on the characteristics of each individual case.ConclusionsPatients with severe congenital abdominal wall defects require individualized surgical treatment to address both the aesthetic and functional issues related to the sequelae of their defects.

Laparoscopic repair of Morgagni hernia and cholecystectomy in a 40-year-old male with Down's sindrome. Report of a case.

Science.gov (United States)

De Paolis, P; Mazza, L; Maglione, V; Fronda, G R

2007-06-01

Morgagni-Larrey hernia (MH) is an unusual diaphragmatic hernia of the retrosternal region. Few cases of MH, treated laparoscopically, associated with Down's syndrome (DS) have been reported in literature. On October 2004, a DS 40-year-old male was admitted to our Department with mild abdominal pain and nausea. Hematochemical tests were within the normal range. Ultrasonography showed biliary sludge and multiple gallstones. Chest X-ray revealed a right-sided paracardiac mass that appeared as MH after a thoraco-abdominal computed tomography (CT). Four trocars were placed as a routinary cholecystectomy. Abdominal exploration confirmed the presence of a voluminous hernia through a wide diaphragmatic defect (12 cm) on the left side of the falciform ligament, containing the last 20 cm ileal loops and right colon with the third lateral of transverse. After retrograde cholecystectomy and reduction of the herniated ileo-colonic tract from multiple adherences, the defect was repaired with an interrupted 2/0 silk suture and then a running 2/0 polypropylene suture. Postoperative course was complicated by pulmonary edema but subsequently the patient was discharged without further complications and has no recurrence after 2 years. In conclusion, surgery is necessary for symptomatic MH and to prevent possible severe complications. We preferred laparoscopy for the reduced morbidity compared to laparotomy, even if in our case the postoperative course was not uneventful. There are still few comparative data about the modality of closure of the defect between primary repair with nonabsorbable suture material, in case of small defects, or continuous monofilament suture or prosthesis in case of large defects.

Congenital malformations of pediatric surgical interest: prevalence, risk factors, and prenatal diagnosis between 2005 and 2012 in the capital city of a developing country. Bogotá, Colombia.

Science.gov (United States)

Correa, Catalina; Mallarino, Christina; Peña, Rafael; Rincón, Luis Carlos; Gracia, Gloria; Zarante, Ignacio

2014-07-01

Congenital anomalies (CAs) cause nearly one third of infant deaths worldwide. Various surveillance systems have been established, such as the Bogota Congenital Malformations Surveillance Program (BCMSP). Some CAs are of special interest to pediatric surgeons: omphalocele, gastroschisis, intestinal and esophageal atresia, anorectal malformations, vascular anomalies, diaphragmatic hernias, hypospadias and cryptorchidism. The aim of this study is to determine the prevalence of such CAs, and identify possible risk factors. Data from the BCMSP were collected between January 2005 and April 2012. CAs were classified in accordance with the ICD-10 and grouped for analysis purposes. Data on CA frequencies were obtained from the BCMSP. Association analyses were performed using the case-control methodology. 282,523 births were registered. 4682 (1.66%) had one or more CAs at birth. The prevalence of CAs requiring pediatric surgery was 1 in 1000. The most frequent CAs were vascular anomalies, hypospadias, and anorectal malformations. Exposure to external factors was significantly associated with selected CAs. 51% of selected birth defects were not diagnosed in prenatal ultrasound. This study highlights the importance of evaluating the local prevalence of congenital malformations. We propose the creation of specialized centers in Bogota to manage patients with CAs. Copyright © 2014 Elsevier Inc. All rights reserved.

Repair of Double Orifice Left AV Valve (DOLAVV with Endocardial Cushion Defect in Adult

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Vivek Velayudhan Pillai

Full Text Available Abstract Double orifice left atrioventricular valve (DOLAVV or double orifice mitral valve (DOMV is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.

Contemporary management and outcomes in congenitally corrected transposition of the great arteries.

Science.gov (United States)

Kutty, Shelby; Danford, David A; Diller, Gerhard-Paul; Tutarel, Oktay

2018-01-11

Congenitally corrected transposition of the great arteries (ccTGA) can occur in isolation, or in combination with other structural cardiac anomalies, most commonly ventricular septal defect, pulmonary stenosis and tricuspid valve disease. Clinical recognition can be challenging, so echocardiography is often the means by which definitive diagnosis is made. The tricuspid valve and right ventricle are on the systemic arterial side of the ccTGA circulation, and are therefore subject to progressive functional deterioration. The natural history of ccTGA is also greatly influenced by the nature and severity of accompanying lesions, some of which require surgical repair. Some management strategies leave the right ventricle as the systemic arterial pump, but carry the risk of worsening heart failure. More complex 'double switch' repairs establish the left ventricle as the systemic pump, and include an atrial baffle to redirect venous return in combination with either arterial switch or Rastelli operation (if a suitable ventricular septal defect permits). Occasionally, the anatomic peculiarities of ccTGA do not allow straightforward biventricular repair, and Fontan palliation is a reasonable option. Regardless of the approach selected, late cardiovascular complications are relatively common, so ongoing outpatient surveillance should be established in an age-appropriate facility with expertise in congenital heart disease care. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

[Phrenic nerve stimulation protects against mechanical ventilation-induced diaphragmatic dysfunction through myogenic regulatory factors].

Science.gov (United States)

An, G H; Chen, M; Zhan, W F; Hu, B; Zhang, H X

2018-02-12

Objective: To explore the protective effect of electrical stimulation of phrenic nerve on diaphragmatic function during mechanical ventilation. Methods: Forty healthy adult SD rats were randomly divided into 5 groups: blank control group (BC), spontaneous breathing group (SB), electrical stimulation group (ES), mechanical ventilation group (MV), and electrical stimulation and mechanical ventilation group (MS). The rats in each group were treated for 18 h except for the BC group. After treatment, the diaphragm muscle tissue was obtained and the diaphragm contractility including peak-to-peak value(Vpp) and maximum rate of contraction(+ dT/dt max) were measured. Expression of MyoD and myogenin were detected. Results: Except for the ES and the MS groups, there was a significant difference for peak-to-peak value (Vpp) between each 2 groups ( P mechanical ventilation induced diaphragmatic function damage, and therefore plays a protective effect on the diaphragm.

Special Formula in the Pharmaceutical Market

Directory of Open Access Journals (Sweden)

B Imani

2014-04-01

Full Text Available Introduction: Infants born with congenital anomalies demand individualized nutritional evaluations and recommendations. The anatomical changes of neonatal surgical diseases create specific physiological constraints.Patients with different congenital anomalies have different nutritional support needs. It is essential to know the exact physiology of these anomalies in order to be able to manage and provide them with appropriate and suitable nutritional supports. This article reviews several nutrition-centered options to aid the medical provider caring for babies with common surgical diseases. Method and Materials: Medline searches were performed using the keywords congenital anomalies, gastroesophageal reflux, motility, nutrition and the text word nutrition in congenital GI anomalies . Bibliographies of recent review articles and relevant primary research reports, as well as Current Contents of ASPEN, ESPEGHAN guidelines were reviewed for additional relevant citations. This article describes the nutritional needs of infants with congenital anomalies that require surgical repair or palliation. It is limited to several commonly encountered surgical diseases of infants: • Foregut and midgut anomalies (esophageal atresia / tracheoesophageal fistula, intestinal atresia, malrotation • Pulmonary hypoplasia: congenital diaphragmatic hernia (CDH and lung malformations • Abdominal wall defects (giant omphalocele, gastroschisis • Diseases treated with enterostomy (eg, Hirschsprung’s disease [HD], imperforate anus   Discussion: Infants born with congenital anomalies demand individualized nutritional evaluations and recommendations. Just within general and thoracic pediatric surgery, diseases include congenital anomalies in the infant, common acquired conditions such as pyloric stenosis and necrotizing enterocolitis, trauma, feeding tube placement, and organ transplantation. With the exception of the surgical repair itself, few interventions hold

[A case of Crow-Fukase syndrome with respiratory failure due to bilateral diaphragmatic paralysis].

Science.gov (United States)

Namekawa, Michito; Muramatsu, Shin-ichi; Hashimoto, Ritsuo; Kawakami, Tadataka; Fujimoto, Ken-ichi; Nakano, Imaharu

2002-07-01

A 62-year-old man with well-controlled diabetes mellitus developed numbness of the bilateral feet and hands, followed by subacutely progressive weakness and amyotrophy of extremities. He became bed-ridden state, and dyspnea also appeared, so he was referred to our hospital. Physical examination revealed a lean man, with dark-reddish skin pigmentation, crabbed fingers, bilateral pretibial pitting edema, and bristles in extremities. Thoracoabdominal paradoxical respiration was observed and pulmonary vesicular sounds was decreased markedly in the both lungs. Laboratory data revealed hypoproteinemia, abnormalities of endocrine system, but M-protein was not detected. Serum vascular endothelial growth factor level was quite high. Chest radiography revealed elevation of the bilateral diaphragm, the % vital capacity (%VC) was 24%, and arterial blood gas analysis showed marked hypoxia with hypercapnia. These findings suggested that his respiratory failure was induced by bilateral diaphragmatic paralysis caused by bilateral phrenic nerve palsy due to Crow-Fukase syndrome. He became somnolent because of hypercapnic narcosis, so non-invasive positive pressure ventilation (NIPPV) was started. We treated him with intravenous immunoglobulin and oral corticosteroids therapies, and after these therapies, his symptoms were remarkably recovered and NIPPV became unnecessary soon. The most frequent causes of respiratory failure in Crow-Fukase syndrome are pleural effusion and pulmonary hypertension, and only two cases of this syndrome with respiratory failure caused by bilateral diaphragmatic paralysis were reported until now. When the patients with Crow-Fukase syndrome complain of dyspnea, we should take the diaphragmatic paralysis into consideration, which may be improved by appropriate therapies.

Diaphragmatic reinnervation in ventilator-dependent patients with cervical spinal cord injury and concomitant phrenic nerve lesions using simultaneous nerve transfers and implantable neurostimulators.

Science.gov (United States)

Kaufman, Matthew R; Elkwood, Andrew I; Aboharb, Farid; Cece, John; Brown, David; Rezzadeh, Kameron; Jarrahy, Reza

2015-06-01

Patients who are ventilator dependent as a result of combined cervical spinal cord injury and phrenic nerve lesions are generally considered to be unsuitable candidates for diaphragmatic pacing due to loss of phrenic nerve integrity and denervation of the diaphragm. There is limited data regarding efficacy of simultaneous nerve transfers and diaphragmatic pacemakers in the treatment of this patient population. A retrospective review was conducted of 14 consecutive patients with combined lesions of the cervical spinal cord and phrenic nerves, and with complete ventilator dependence, who were treated with simultaneous microsurgical nerve transfer and implantation of diaphragmatic pacemakers. Parameters of interest included time to recovery of diaphragm electromyographic activity, average time pacing without the ventilator, and percent reduction in ventilator dependence. Recovery of diaphragm electromyographic activity was demonstrated in 13 of 14 (93%) patients. Eight of these 13 (62%) patients achieved sustainable periods (> 1 h/d) of ventilator weaning (mean = 10 h/d [n = 8]). Two patients recovered voluntary control of diaphragmatic activity and regained the capacity for spontaneous respiration. The one patient who did not exhibit diaphragmatic reinnervation remains within 12 months of initial treatment. Surgical intervention resulted in a 25% reduction (p nerve transfers and pacemaker implantation can result in reinnervation of the diaphragm and lead to successful ventilator weaning. Our favorable outcomes support consideration of this surgical method for appropriate patients who would otherwise have no alternative therapy to achieve sustained periods of ventilator independence. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Fluorouracil implants caused a diaphragmatic tumor to be misdiagnosed as liver metastasis: a case report

International Nuclear Information System (INIS)

Shen, Yang-Yang; Qin, Hong-Wei; Zhang, Jian-Bo; Wang, Zhen-Dan; Li, Pang; Pang, Kai; Zhang, Bo; Li, Sheng; Cui, Kai

2016-01-01

Fluorouracil implants are widely used in peritoneal interstitial chemotherapy. Curative effects have been obtained, but implants have also caused some complications. We performed an analysis of a 66-year-old male patient’s case history, as well as conventional pathological analysis and Raman spectroscopic detection of the diaphragmatic tumor. We also analyzed the underlying causes of this condition to prevent complications and reduce misdiagnoses in future cases. The patient had a history of peritoneal fluorouracil implantation. Pathological analysis of the diaphragmatic mass revealed foreign particles, and Raman detection showed that the mass contained fluorouracil. Fluorouracil implants may persist due to the high concentrations of this drug used in peritoneal chemotherapy. This finding should provide guidance and improve the application of peritoneal implants. In clinical trials, and the diagnosis of liver metastasis should be based on pathological results

Pelvic ventral hernia repair in a pygopagus conjoint twin | Bhullar ...

African Journals Online (AJOL)

Pelvic ventral hernia repair in a surviving conjoint twin with multiple congenital anomalies that make surgery a challenge. Conjoint twins are a rare. The incidence is reported to be in the range of 1/50 000 to 1/100 000 live births. Of the conjoint twins, 40% are stillborn and an additional one-third die within 24 h of birth.

Radiological features of 80 cases of diaphragmatic rupture

International Nuclear Information System (INIS)

Sullivan, M.; Lee, R.

1989-01-01

The films from 80 cases of diaphragmatic rupture in the dog and cat over a 10 year period were examined. The most common findings were loss of the cupola and masking of the cardiac shadow. Identification of the stomach or intestine in the thorax made the diagnosis straightforward. However, in some animals these organs were not visible and there was considerable accumulation of free pleural fluid. The use of barium contrast studies and post drainage films were unable to confirm the presence of a rupture in all cases with inconclusive plain film findings. The identification of a rupture was only made by exploratory surgery in these animals

Hernioplastia diafragmática em cão com pericárdio bovino conservado em solução supersaturada de açúcar Diaphragmatic hernioplasty in dogs with bovine pericardium preserved in supersaturated sugar solution

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A. Mazzanti

2003-12-01

implantation site. Histologically, fine connective tissue occluded the diaphragmatic defect. Bovine pericardium preserved in 300% supersaturated sugar solution at room temperature is replaced by a fine layer of connective fibrous tissue and promote repair of large diaphragmatic defects in dogs.

FDG-PET identification of infected pulmonary artery conduit following tetralogy of fallot (TOF) repair

Energy Technology Data Exchange (ETDEWEB)

Zhang, Yu Yang; Williams, Hadyn; Pucar, Darko [Dept. of Radiology, Medical College of Georgia, Augusta (United States)

2017-03-15

Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease usually managed by serial surgical repairs. The repaired prosthetic valve or conduit is susceptible to life-threatening infection. FDG-PET is an effective alternative to evaluate the source of infection when other examinations are inconclusive. We report an unusual case of an infected pulmonary artery conduit after TOF repair although the echocardiogram was negative for vegetation, which was later confirmed by surgery and pathology. The case highlights the role of FDG-PET as a problem-solving tool for potential endocarditis and cardiac device infection cases after complex cardiac surgery.

FDG-PET identification of infected pulmonary artery conduit following tetralogy of fallot (TOF) repair

International Nuclear Information System (INIS)

Zhang, Yu Yang; Williams, Hadyn; Pucar, Darko

2017-01-01

Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease usually managed by serial surgical repairs. The repaired prosthetic valve or conduit is susceptible to life-threatening infection. FDG-PET is an effective alternative to evaluate the source of infection when other examinations are inconclusive. We report an unusual case of an infected pulmonary artery conduit after TOF repair although the echocardiogram was negative for vegetation, which was later confirmed by surgery and pathology. The case highlights the role of FDG-PET as a problem-solving tool for potential endocarditis and cardiac device infection cases after complex cardiac surgery

Traumatic hallux varus repair utilizing a soft-tissue anchor: a case report.

Science.gov (United States)

Labovitz, J M; Kaczander, B I

2000-01-01

Hallux varus is usually iatrogenic in nature; however, congenital and acquired etiologies have been described in the literature. The authors present a case of traumatic hallux varus secondary to rupture of the adductor tendon. Surgical correction was performed using a soft tissue anchor for maintenance of the soft tissues utilized for repair.

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with congenital heart disease

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Antonio Lopes

2014-01-01

Full Text Available Congenital heart disease (CHD with intracardiac/extracardiac shunts is an important etiology of pulmonary arterial hypertension (PAH. The majority of children with congenital cardiac shunts do not develop advanced pulmonary vasculopathy, as surgical repair of the anomalies is now performed early in life. However, if not repaired early, some defects will inevitably lead to pulmonary vascular disease (truncus arteriosus, transposition of the great arteries associated with a ventricular septal defect (VSD, atrioventricular septal defects remarkably in Down syndrome, large, nonrestrictive VSDs, patent ductus arteriosus and related anomalies. The majority of patients are now assigned to surgery based on noninvasive evaluation only. PAH becomes a concern (requiring advanced diagnostic procedures in about 2-10% of them. In adults with CHD, the prevalence of advanced pulmonary vasculopathy (Eisenmenger syndrome is around 4-12%. [1] This article will discuss the diagnostic and management approach for PAH associated with CHD (PAH-CHD.

A contemporary, single-institutional experience of surgical versus expectant management of congenital heart disease in trisomy 13 and 18 patients.

Science.gov (United States)

Costello, John P; Weiderhold, Allison; Louis, Clauden; Shaughnessy, Conner; Peer, Syed M; Zurakowski, David; Jonas, Richard A; Nath, Dilip S

2015-06-01

The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution's Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.

Long-term outcomes of a transmastoid lateral semicircular canal approach to congenital CSF otorrhea in children associated with recurrent meningitis and severe inner ear malformation.

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Wang, Jie; Li, Yongxin; Chen, Shubin; Hao, Xinping

2016-08-01

To investigate the long-term effectiveness of transmastoid lateral semicircular canal approach (TMLSCCA) to repair cerebrospinal fluid (CSF) leakage in children associated with recurrent meningitis and severe congenital inner malformation. A retrospective study was conducted in a university hospital, academic medical center. Fifteen children with recurrent meningitis, secondary to severe congenital inner ear malformation, were included in the study. All of them had CSF associated otorrhea and treated using TLSCCA to repair CSF otorrhea by packing the vestibule with muscle and fascia. Observation of the status of postoperative CSF leakage, recurrence of meningitis and complication were conducted. None of the cases had recurrent meningitis and CSF leakage after their TLSCCA procedure in the follow-up period of 1-8.5 years. One case presented with transient facial nerve paralysis and completely recovered 3 months later. TLSCCA for CSF otorrhea in children with recurrent meningitis secondary to congenital inner ear malformation is an alternative approach that offers some advantages. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

The helical ventricular myocardial band of Torrent-Guasp: potential implications in congenital heart defects.

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Corno, Antonio F; Kocica, Mladen J; Torrent-Guasp, Francisco

2006-04-01

The new concepts of cardiac anatomy and physiology, based on the observations made by Francisco Torrent-Guasp's discovery of the helical ventricular myocardial band, can be useful in the context of the surgical strategies currently used to manage patients with congenital heart defects. The potential impact of the Torrent-Guasp's Heart on congenital heart defects have been analyzed in the following settings: ventriculo-arterial discordance (transposition of the great arteries), double (atrio-ventricular and ventriculo-arterial) discordance (congenitally corrected transposition of the great arteries), Ebstein's anomaly, pulmonary valve regurgitation after repair of tetralogy of Fallot, Ross operation, and complex intra-ventricular malformations. The functional interaction of right and left ventricles occurs not only through their arrangements in series but also thanks to the structural spiral features. Changes in size and function of either ventricle may influence the performance of the other ventricle. The variety and complexity of congenital heart defects make the recognition of the relationship between form and function a vital component, especially when compared to acquired disease. The new concepts of cardiac anatomy and function proposed by Francisco Torrent-Guasp, based on his observations, should stimulate further investigations of alternative surgical strategies by individuals involved with the management of patients with congenital heart defects.

Elevated prothrombin time on routine preoperative laboratory results in a healthy infant undergoing craniosynostosis repair: Diagnosis and perioperative management of congenital factor VII deficiency.

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Jones, Kareen L; Greenberg, Robert S; Ahn, Edward S; Kudchadkar, Sapna R

2016-01-01

Congenital factor VII deficiency is a rare bleeding disorder with high phenotypic variability. It is critical that children with congenital Factor VII deficiency be identified early when high-risk surgery is planned. Cranial vault surgery is common for children with craniosynostosis, and these surgeries are associated with significant morbidity mostly secondary to the risk of massive blood loss. A two-month old infant who presented for elective craniosynostosis repair was noted to have an elevated prothrombin time (PT) with a normal activated partial thromboplastin time (aPTT) on preoperative labs. The infant had no clinical history or reported family history of bleeding disorders, therefore a multidisciplinary decision was made to repeat the labs under general anesthesia and await the results prior to incision. The results confirmed the abnormal PT and the case was canceled. Hematologic workup during admission revealed factor VII deficiency. The patient underwent an uneventful endoscopic strip craniectomy with perioperative administration of recombinant Factor VIIa. Important considerations for perioperative laboratory evaluation and management in children with factor VII deficiency are discussed. Anesthetic and surgical management of the child with factor VII deficiency necessitates meticulous planning to prevent life threatening bleeding during the perioperative period. A thorough history and physical examination with a high clinical suspicion are vital in preventing hemorrhage during surgeries in children with coagulopathies. Abnormal preoperative lab values should always be confirmed and addressed before proceeding with high-risk surgery. A multidisciplinary discussion is essential to optimize the risk-benefit ratio during the perioperative period. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

Electromyographic permutation entropy quantifies diaphragmatic denervation and reinnervation.

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Christopher Kramer

Full Text Available Spontaneous reinnervation after diaphragmatic paralysis due to trauma, surgery, tumors and spinal cord injuries is frequently observed. A possible explanation could be collateral reinnervation, since the diaphragm is commonly double-innervated by the (accessory phrenic nerve. Permutation entropy (PeEn, a complexity measure for time series, may reflect a functional state of neuromuscular transmission by quantifying the complexity of interactions across neural and muscular networks. In an established rat model, electromyographic signals of the diaphragm after phrenicotomy were analyzed using PeEn quantifying denervation and reinnervation. Thirty-three anesthetized rats were unilaterally phrenicotomized. After 1, 3, 9, 27 and 81 days, diaphragmatic electromyographic PeEn was analyzed in vivo from sternal, mid-costal and crural areas of both hemidiaphragms. After euthanasia of the animals, both hemidiaphragms were dissected for fiber type evaluation. The electromyographic incidence of an accessory phrenic nerve was 76%. At day 1 after phrenicotomy, PeEn (normalized values was significantly diminished in the sternal (median: 0.69; interquartile range: 0.66-0.75 and mid-costal area (0.68; 0.66-0.72 compared to the non-denervated side (0.84; 0.78-0.90 at threshold p<0.05. In the crural area, innervated by the accessory phrenic nerve, PeEn remained unchanged (0.79; 0.72-0.86. During reinnervation over 81 days, PeEn normalized in the mid-costal area (0.84; 0.77-0.86, whereas it remained reduced in the sternal area (0.77; 0.70-0.81. Fiber type grouping, a histological sign for reinnervation, was found in the mid-costal area in 20% after 27 days and in 80% after 81 days. Collateral reinnervation can restore diaphragm activity after phrenicotomy. Electromyographic PeEn represents a new, distinctive assessment characterizing intramuscular function following denervation and reinnervation.

[Adult congenital heart disease--between guidelines and clinical practice].

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Chessa, Massimo

2011-01-01

Advances in medical and surgical management of congenital heart disease have changed the prognosis of infants and children with cardiac defects, so that an increasing number of patients reach adolescence and adult life, even those with complex defects. Recent data suggest that the number of adults with congenital heart disease, either repaired or not, approaches the number of children with the disorder. A cure is rarely achieved and ongoing surveillance and management in conjunction with specialists in this highly specialized field is mandatory to provide optimal care for patients. The profile of this patient population is going to change over the next few decades. Ideally specialist units should be established in appropriate geographic locations; patients need to be concentrated for expertise, experience, and optimal management. Less specialized regional centers and outpatient clinics in districts in connection with grown-up congenital heart disease units should be created. Specialist units should accept responsibility for educating the professionals, training the specialists, and sharing particular skills between each other. Guidelines and recommendations should help physicians to make decision in their daily practice. However, the final judgment regarding the care of an individual patient must be made by his/her physician. This article will briefly discuss some aspects of these dedicated guidelines and how they influence the clinical daily practice.

Early hemi-diaphragmatic plication through a video assisted mini-thoracotomy in postcardiotomy phrenic nerve paresis

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Tsakiridis, Kosmas; Visouli, Aikaterini N.; Machairiotis, Nikolaos; Christofis, Christos; Stylianaki, Aikaterini; Katsikogiannis, Nikolaos; Mpakas, Andreas; Courcoutsakis, Nicolaos; Zarogoulidis, Konstantinos

2012-01-01

New symptom onset of respiratory distress without other cause, and new hemi-diaphragmatic elevation on chest radiography postcardiotomy, are usually adequate for the diagnosis of phrenic nerve paresis. The symptom severity varies (asymptomatic state to severe respiratory failure) depending on the degree of the lesion (paresis vs. paralysis), the laterality (unilateral or bilateral), the age, and the co-morbidity (respiratory, cardiac disease, morbid obesity, etc). Surgical treatment (hemi-diaphragmatic plication) is indicated only in the presence of symptoms. The established surgical treatment is plication of the affected hemidiaphragm which is generally considered safe and effective. Several techniques and approaches are employed for diaphragmatic plication (thoracotomy, video-assisted thoracoscopic surgery, video-assisted mini-thoracotomy, laparoscopic surgery). The timing of surgery depends on the severity and the progression of symptoms. In infants and young children with postcardiotomy phrenic nerve paresis the clinical status is usually severe (failure to wean from mechanical ventilation), and early plication is indicated. Adults with postcardiotomy phrenic nerve paresis usually suffer from chronic dyspnoea, and, in the absence of respiratory distress, conservative treatment is recommended for 6 months -2 years, since improvement is often observed. Nevertheless, earlier surgical treatment may be indicated in non-resolving respiratory failure. We present early (25th day postcardiotomy) right hemi-diaphragm plication, through a video assisted mini-thoracotomy in a high risk patient with postcardiotomy phrenic nerve paresis and respiratory distress. Early surgery with minimal surgical trauma, short operative time, minimal blood loss and postoperative pain, led to fast rehabilitation and avoidance of prolonged hospitalization complications. The relevant literature is discussed. PMID:23304442

Incarcerated Diaphragmatic Hernia with Bowel Perforation Presenting as a Tension Pneumothorax

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Ryan P. Offman

2014-03-01

Full Text Available We present an interesting case of a patient with a previously known diaphragmatic hernia in which the colon became incarcerated, ischemic and finally perforated. She had no prior history of abdominal pain or vomiting, yet she present with cardiovascular collapse. To our knowledge, this is the only case report of a tension pneumothorax associated with perforated bowel that was not in the setting of trauma or colonoscopy. [West J Emerg Med. 2014;15(2:142-144.

Laparoscopic Treatment of a Rare Right Diaphragmatic Rupture with Small Bowel Herniation after Blunt Thoracic Trauma

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H. Hoffmann

2010-01-01

Full Text Available Blunt traumatic diaphragmatic rupture (BTDR is a life-threatening condition with an incidence from 0,8%–1,6% in blunt trauma, mostly located on the left side. The main prognostic factors are severe side injuries and the delay of diagnosis. We present a rare case of a 68-year-old female, with an isolated right diaphragm rupture. The diagnosis was done with a delay of 4 days by thoracic radiographs, which showed a herniation of small bowel into the right thoracic cavity. A reposition of the small bowel and a closure of the diaphragmatic defect by running suture were carried out laparoscopicly. Although large prospective studies concerning the outcome of laparoscopic approach to right BTDR are still missing, we could show, that laparoscopy can be performed safely in right traumatic diaphragm rupture.

Quantification of renal and diaphragmatic interfractional motion in pediatric image-guided radiation therapy: A multicenter study

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Huijskens, S.C.; Dijk, I.W. van; Jong, R. de; Visser, J.; Fajardo, R.D.; Ronckers, C.M.; Janssens, G.O.R.J.; Maduro, J.H.; Rasch, C.R.; Alderliesten, T.; Bel, A.

2015-01-01

BACKGROUND AND PURPOSE: To quantify renal and diaphragmatic interfractional motion in order to estimate systematic and random errors, and to investigate the correlation between interfractional motion and patient-specific factors. MATERIAL AND METHODS: We used 527 retrospective abdominal-thoracic

Non-Invasive monitoring of diaphragmatic timing by means of surface contact sensors: An experimental study in dogs

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Galdiz Batxi

2004-09-01

Full Text Available Abstract Background Non-invasive monitoring of respiratory muscle function is an area of increasing research interest, resulting in the appearance of new monitoring devices, one of these being piezoelectric contact sensors. The present study was designed to test whether the use of piezoelectric contact (non-invasive sensors could be useful in respiratory monitoring, in particular in measuring the timing of diaphragmatic contraction. Methods Experiments were performed in an animal model: three pentobarbital anesthetized mongrel dogs. The motion of the thoracic cage was acquired by means of a piezoelectric contact sensor placed on the costal wall. This signal is compared with direct measurements of the diaphragmatic muscle length, made by sonomicrometry. Furthermore, to assess the diaphragmatic function other respiratory signals were acquired: respiratory airflow and transdiaphragmatic pressure. Diaphragm contraction time was estimated with these four signals. Using diaphragm length signal as reference, contraction times estimated with the other three signals were compared with the contraction time estimated with diaphragm length signal. Results The contraction time estimated with the TM signal tends to give a reading 0.06 seconds lower than the measure made with the DL signal (-0.21 and 0.00 for FL and DP signals, respectively, with a standard deviation of 0.05 seconds (0.08 and 0.06 for FL and DP signals, respectively. Correlation coefficients indicated a close link between time contraction estimated with TM signal and contraction time estimated with DL signal (a Pearson correlation coefficient of 0.98, a reliability coefficient of 0.95, a slope of 1.01 and a Spearman's rank-order coefficient of 0.98. In general, correlation coefficients and mean and standard deviation of the difference were better in the inspiratory load respiratory test than in spontaneous ventilation tests. Conclusion The technique presented in this work provides a non

Severe pregnancy-induced deterioration of truncal valve regurgitation in an adolescent patient with repaired truncus arteriosus

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Hoendermis, Elke S.; Drenthen, Willem; Sollie, Krystyna M.; Berger, Rolf M. F.

2008-01-01

Truncus arteriosus, a rare and complex congenital heart disease, is hallmarked by a single great vessel ( truncus) that arises over a large ventricular septal defect and provides both the pulmonary and systemic circulation. Pregnancy reports after repair for truncus arteriosus are scarce. Therefore,

Perioperative Challenges in Repeat Bladder Exstrophy Repair - Case Report

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Otu Enenyi Etta

2015-07-01

Full Text Available Bladder exstrophy is a rare congenital malformation. It presents as leakage of urine in the anterior abdominal wall following defects in midline anterior abdominal wall skin and bladder. We report the use of combined general anaesthesia and caudal epidural analgesia in a 4yr old boy for repeat bladder exstrophy repair. Problems of prolonged surgery and the challenges of pain and sedation management in the post operative period are discussed.

Correção simultânea de defeito congênito intracardíaco e pectus excavatum Simultaneous repair of congenital heart defect and pectus excavatum

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João Roberto Breda

2007-09-01

Full Text Available Relatamos tratamento simultâneo de pectus excavatum e defeito congênito intracardíaco representado por comunicação interatrial ostium secundum. Paciente do sexo masculino, 8 anos de idade, com diagnóstico clínico e ecocardiográfico de comunicação interatrial, associada à deformidade da parede torácica tipo pectus excavatum. Foi encaminhado para operação com correção simultânea do defeito congênito intracardíaco associado ao reparo do pectus. O tratamento operatório simultâneo do pectus excavatum e defeitos congênitos intracardíacos torna difícil o acesso ao coração. Foi feita a correção simultânea dessas alterações, com satisfatório resultado, sobretudo estético, para o paciente.The author describes the simultaneous treatment of pectus excavatum and congenital intracardiac defect (atrial septal defect represented by the interatrial foramen secundum. An 8-year-old boy, with clinical and echocardiography diagnosis of atrial septal defect associated with pectus excavatum was referred to a simultaneous surgical treatment of both abnormalities. The simultaneous surgical treatment of both pectus excavatum and congenital intracardiac defects make it difficult to access the heart. In this case, the simultaneous surgical treatment of atrial septal defect and pectus excavatum was a valuable alternative to surgical repair of both abnormalities, mainly due to its cosmetic outcome.

Quantification of renal and diaphragmatic interfractional motion in pediatric image-guided radiation therapy: A multicenter study

NARCIS (Netherlands)

Huijskens, Sophie C.; van Dijk, Irma W. E. M.; de Jong, Rianne; Visser, Jorrit; Fajardo, Raquel Dávila; Ronckers, Cécile M.; Janssens, Geert O. R. J.; Maduro, John H.; Rasch, Coen R. N.; Alderliesten, Tanja; Bel, Arjan

2015-01-01

To quantify renal and diaphragmatic interfractional motion in order to estimate systematic and random errors, and to investigate the correlation between interfractional motion and patient-specific factors. We used 527 retrospective abdominal-thoracic cone beam CT scans of 39 childhood cancer

Effect of upper costal and costo-diaphragmatic breathing types on electromyographic activity of respiratory muscles.

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Celhay, Isabel; Cordova, Rosa; Miralles, Rodolfo; Meza, Francisco; Erices, Pia; Barrientos, Camilo; Valenzuela, Saúl

2015-04-01

To compare electromyographic (EMG) activity in young-adult subjects with different breathing types. This study included 50 healthy male subjects with complete natural dentition, and no history of orofacial pain or craniomandibular-cervical-spinal disorders. Subjects were classified into two groups: upper costal breathing type, and costo-diaphragmatic breathing. Bipolar surface electrodes were located on sternocleidomastoid, diaphragm, external intercostal, and latissimus dorsi muscles. Electromyographic activity was recorded during the following tasks: (1) normal quiet breathing; (2) speaking the word 'Mississippi'; (3) swallowing saliva; and (4) forced deep breathing. Sternocleidomastoid and latissimus dorsi EMG activity was not significantly different between breathing types, whereas diaphragm and external intercostal EMG activity was significantly higher in the upper costal than costo-diaphragmatic breathing type in all tasks (P<0·05; Wilcoxon signed rank-sum test). Diaphragm and external intercostal EMG activity suggests that there could be differences in motor unit recruitment strategies depending on the breathing type.

Unusual delayed presentation of diaphragmatic hernia complicated by transverse colon and total small-bowel obstruction after postoperative chemotherapy of esophageal cancer

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Sun Y

2017-06-01

Full Text Available Yanlai Sun,1,2,* Lei Yin,2,* Hongfan Xue,2 Haipeng Wang,2 Zengjun Li,2 Jinming Yu3 1Post-doctoral Research Station, Tianjin Medical University, Tianjin, 2Department of Gastrointestinal Cancer Surgery, 3Department of Radiation Oncology, Shandong Cancer Hospital Affiliated to Shandong University, Shandong Academy of Medical Sciences, Jinan, People’s Republic of China *These authors equally contributed to this work Abstract: Diaphragmatic hernia (DH is defined as the passage of abdominal contents into the chest cavity through a defect in the diaphragm. DH occurs after chest or abdominal surgery, and is very rare and sporadically reported in the literature. However, the complications are significant and put the patient at great risk. The aim of the present report was to describe a special case with postesophagectomy diaphragmatic hernia (PDH because of its appearance during chemotherapy and confusion of the symptoms with the side effects of chemotherapy. A high index of suspicion needs to be maintained in clinical practice. Keywords: diaphragmatic hernia, postoperative chemotherapy, esophageal cancer, complications

Overview of Epidemiology, Genetics, Birth Defects, and Chromosome Abnormalities Associated With CDH

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Pober, Barbara R.

2010-01-01

Congenital diaphragmatic hernia (CDH) is a common and well-studied birth defect. The etiology of most cases remains unknown but increasing evidence points to genetic causation. The data supporting genetic etiologies which are detailed below include the association of CDH with recurring chromosome abnormalities, the existence of CDH-multiplex families, and the co-occurrence of CDH with additional congenital malformations. PMID:17436298

The association of congenital neuroblastoma and congenital heart disease

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

1989-01-01

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

One and a half ventricular repair as an alternative for hypoplastic right ventricle.

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Maluf, Miguel Angel; Carvalho, Antonio Carlos; Carvalho, Werther Brunow

2010-01-01

Patients with complex congenital heart disease, characterized by right ventricle hypoplasia, had a palliative surgical option with one and a half ventricular repair. From July 2001 to March 2009, nine patients (mean age 5.2 years, range 3 to 9 years) with hypoplastic right ventricle, underwent correction with one and a half ventricle technique. Preoperative diagnoses included: pulmonary atresia with intact ventricular septum, in six and Ebstein's anomaly, in three cases. Six patients had bidirectional cavo-pulmonary shunt (Glenn operation) previously. The surgical approach was performed with cardiopulmonary bypass to correct intracardiac defects: atrial septal defect closure (nine cases); right ventricle outlet tract reconstruction with porcine pulmonary prosthesis (seven cases); tricuspid valvuloplasty (three cases). There was one (11.1%) hospital death. All the patients left the hospital in good clinical conditions. One patient presented pulmonary stenosis at distal prosthesis anastomosis and needed surgical correction. There was one (12.5%) late deaths after reoperation. At mean follow-up of 39.8 months (range 16 months to 8.4 years) seven patients are alive in functional class I (NYHA). Surgical treatment of congenital cardiac anomalies in the presence of a hypoplastic right ventricle by means of one and a half ventricle repair has the advantages of reducing the surgical risk of biventricular repair compared to the Fontan circulation; it maintains a low right atrium pressure, a pulsatile pulmonary blood flow and improves the systemic oxygen saturation with short and medium-term promising results. Longer follow-up is needed to prove the efficacy of such a repair in the long term.

Magnetic resonance imaging of the fetus in congenital intrathoracic disorders: preliminary observations

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Liu Xiang; Ashtari, M.; Leonidas, J.C. [Dept. of Radiology, Schneider Children' s Hospital, Long Island Jewish Medical Center, NY (United States); Chan Ying [Fetal-Maternal Medicine, Schneider Children' s Hospital, Long Island Jewish Medical Center, and the Albert Einstein College of Medicine, NY (United States)

2001-06-01

Background and objective. Advances in magnetic resonance imaging (MRI) provide high-quality images of the intrathoracic organs. We studied the ability of MRI to define spatial relationships of the fetal lungs and measured lung volume in two cases of congenital diaphragmatic hernia (CDH), one of severe oligohydramnios secondary to bilateral cystic renal dysplasia and one case of prenatal chylothorax. Patients and methods. We performed pelvic MRI using single-shot fast spin echo (SSFSE) pulse sequence in four pregnant women referred because of abnormal prenatal ultrasound (US) findings associated with pulmonary hypoplasia. Results. The exact anatomic position of the contents of the hernia in CDH, including the position of the liver, was better defined with MRI. Pleural effusions were identified as well as the renal abnormality in the case of oligohydramnios. Lung volume was measured and the degree of pulmonary hypoplasia was quantified in every case. Lung-to-thorax ratio was calculated in the case of fetal chylothorax. Conclusion. Ongoing work suggests that MRI can provide additional detailed quantitative information in prenatal disorders associated with fetal lung compression and resulting hypoplasia. Correlation of fetal lung volume with postnatal management and outcome may affect prognosis in these cases. (orig.)

Magnetic resonance imaging of the fetus in congenital intrathoracic disorders: preliminary observations

International Nuclear Information System (INIS)

Liu Xiang; Ashtari, M.; Leonidas, J.C.; Chan Ying

2001-01-01

Background and objective. Advances in magnetic resonance imaging (MRI) provide high-quality images of the intrathoracic organs. We studied the ability of MRI to define spatial relationships of the fetal lungs and measured lung volume in two cases of congenital diaphragmatic hernia (CDH), one of severe oligohydramnios secondary to bilateral cystic renal dysplasia and one case of prenatal chylothorax. Patients and methods. We performed pelvic MRI using single-shot fast spin echo (SSFSE) pulse sequence in four pregnant women referred because of abnormal prenatal ultrasound (US) findings associated with pulmonary hypoplasia. Results. The exact anatomic position of the contents of the hernia in CDH, including the position of the liver, was better defined with MRI. Pleural effusions were identified as well as the renal abnormality in the case of oligohydramnios. Lung volume was measured and the degree of pulmonary hypoplasia was quantified in every case. Lung-to-thorax ratio was calculated in the case of fetal chylothorax. Conclusion. Ongoing work suggests that MRI can provide additional detailed quantitative information in prenatal disorders associated with fetal lung compression and resulting hypoplasia. Correlation of fetal lung volume with postnatal management and outcome may affect prognosis in these cases. (orig.)

Two Ports Laparoscopic Inguinal Hernia Repair in Children

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Medhat M. Ibrahim

2015-01-01

Full Text Available Introduction. Several laparoscopic treatment techniques were designed for improving the outcome over the last decade. The various techniques differ in their approach to the inguinal internal ring, suturing and knotting techniques, number of ports used in the procedures, and mode of dissection of the hernia sac. Patients and Surgical Technique. 90 children were subjected to surgery and they undergone two-port laparoscopic repair of inguinal hernia in children. Technique feasibility in relation to other modalities of repair was the aim of this work. 90 children including 75 males and 15 females underwent surgery. Hernia in 55 cases was right-sided and in 15 left-sided. Two patients had recurrent hernia following open hernia repair. 70 (77.7% cases were suffering unilateral hernia and 20 (22.2% patients had bilateral hernia. Out of the 20 cases 5 cases were diagnosed by laparoscope (25%. The patients’ median age was 18 months. The mean operative time for unilateral repairs was 15 to 20 minutes and bilateral was 21 to 30 minutes. There was no conversion. The complications were as follows: one case was recurrent right inguinal hernia and the second was stitch sinus. Discussion. The results confirm the safety and efficacy of two ports laparoscopic hernia repair in congenital inguinal hernia in relation to other modalities of treatment.

Prognostic indicators for perioperative survival after diaphragmatic herniorrhaphy in cats and dogs: 96 cases (2001-2013).

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Legallet, Claire; Thieman Mankin, Kelley; Selmic, Laura E

2017-01-07

To determine associations between perioperative mortality after surgery for traumatic diaphragmatic hernia, medical records of 17 cats and 79 dogs that underwent diaphragmatic herniorrhaphy were reviewed. The combined perioperative survival rate was 81.3% (88.2% in cats and 79.8% in dogs). Data from acute and chronic cases was assessed separately. Of the acute cases (12 cats and 48 dogs), 10 cats (83.3%) and 38 dogs (79.2%) survived to discharge. Of the chronic cases (5 cats and 31 dogs), 5 cats (100%) and 25 dogs (80.6%) survived to discharge. The time between trauma and surgery, trauma and admission, and admission and surgery were not associated with survival. For cats and dogs, increased duration of anesthesia and surgical procedure were associated with increased mortality (P = 0.0013 and 0.004, respectively). Animals with concurrent soft tissue injuries had a 4.3 times greater odds of mortality than those without soft tissue injury (P = 0.01). Animals with concurrent soft tissue and orthopedic injuries had a 7.3 times greater odds of mortality than those without soft tissue and orthopedic injuries (P = 0.004). Animals that were oxygen dependent had a 5.0 times greater odds of mortality than those that were not (P = 0.02). No other variables were significantly associated with survival. For cats and dogs that underwent surgery for traumatic diaphragmatic hernia, increased anesthetic duration, increased duration of surgical procedure, concurrent soft tissue injuries, concurrent soft tissue and orthopedic injuries, and perioperative oxygen dependence were associated with increased mortality.

Congenital spinal malformations; Kongenitale spinale Malformationen

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Ertl-Wagner, B.B.; Reiser, M.F. [Klinikum Grosshadern, Ludwig-Maximilians-Univ. Muenchen (Germany). Inst. fuer Klinische Radiologie

2001-12-01

Congenital spinal malformations form a complex and heterogeneous group of disorders whose pathogenesis is best explained embryologically. Radiologically, it is important to formulate a diagnosis when the disorder first becomes symptomatic. However, it is also crucial to detect complications of the disorder or of the respective therapeutic interventions in the further course of the disease such as hydromyelia or re-tethering after repair of a meningomyelocele. Moreover, once a congenital spinal malformation is diagnosed, associated malformations should be sought after. A possible syndromal classification such as in OEIS- or VACTERL-syndromes should also be considered. (orig.) [German] Kongenitale spinale Malformationen stellen eine komplexe Gruppe an Stoerungen dar, deren Genese sich am einfachsten aus der Embryologie heraus erklaeren laesst. Bei der klinisch-radiologischen Begutachtung ist zunaechst ihre korrekte Klassifikation im Rahmen der Erstdiagnose wichtig. Im weiteren Verlauf ist es jedoch zudem entscheidend, moegliche Komplikationen wie beispielsweise eine Hydromyelie oder ein Wiederanheften des Myelons nach Operation einer Spina bifida aperta zu erkennen. Zudem sollte bei der Diagnosestellung einer kongenitalen spinalen Malformation immer auch auf assoziierte Fehlbildungen, wie z.B. die Diastematomyelie oder das intraspinale Lipom bei der Spina bifida aperta, sowie auf eine moegliche syndromale Einordnung wie beispielsweise beim OEIS-oder VACTERL-Syndrom geachtet werden. (orig.)

MR imaging of congenital heart diseases in adolescents and adults

International Nuclear Information System (INIS)

Choe, Yeon Hyeon; Kang, I Seok; Park, Seung Woo; Lee, Heung Jae

2001-01-01

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of tetralogy of Fallot, and Senning's and Mustard's operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults

Comorbidity between Klinefelter syndrome and diaphragmatic hernia. A case report

Directory of Open Access Journals (Sweden)

Carolina Melendez Valdez

Full Text Available CONTEXT: Intrathoracic cystic lesions have been diagnosed in a wide variety of age groups, and the increasing use of prenatal imaging studies has allowed detection of these defects even in utero.CASE REPORT: A 17-year-old pregnant woman in her second gestation, at 23 weeks of pregnancy, presented an ultrasound with evidence of a cystic anechoic image in the fet al left hemithorax. A morphological ultrasound examination performed at the hospital found that this cystic image measured 3.7 cm x 2.1 cm x 1.6 cm. Polyhydramnios was also present. At this time, the hypothesis of cystic adenomatoid malformation was raised. Fet al echocardiography showed only a dextroposed heart. Fet al magnetic resonance imaging produced an image compatible with a left diaphragmatic hernia containing the stomach and at least the first and second portions of the duodenum, left lobe of the liver, spleen, small intestine segments and portions of the colon. The stomach was greatly distended and the heart was shifted to the right. There was severe volume reduction of the left lung. Fet al karyotyping showed the chromosomal constitution of 47,XXY, compatible with Klinefelter syndrome. In our review of the literature, we found only one case of association between Klinefelter syndrome and diaphragmatic hernia.CONCLUSIONS: We believe that the association observed in this case was merely coincidental, since both conditions are relatively common. The chance of both events occurring simultaneously is estimated to be 1 in 1.5 million births.

Umbilical cord sparing technique for repair of congenital hernia into the cord and small omphalocele.

Science.gov (United States)

Ceccanti, Silvia; Falconi, Ilaria; Frediani, Simone; Boscarelli, Alessandro; Musleh, Layla; Cozzi, Denis A

2017-01-01

Current repair of small omphaloceles and hernias into the umbilical cord is a straightforward procedure, whose repair may result in a suboptimal cosmetic outcome. We describe a novel repair technique retaining the umbilical cord elements in an attempt to improve the cosmetic appearance of the umbilicus. Eight neonates were consecutively treated more than a ten-year period. Size of the fascial defects ranged 1 to 3cm (median, 2). Present technique entails incision of the amniotic sac without its detachment from the skin, reduction of the extruded contents under direct vision, and closure of the abdominal wall defect by circumferential suturing of peritoneum and fascia around the base of the amniotic sac. The amniotic sac is then re-approximated and folded to create an umbilical stump, which is trimmed and left to shed naturally. All patients achieved a scarless abdomen with a normal appearing umbilicus in 6. The remaining 2 patients are awaiting surgery for persisting umbilical hernia repair and umbilicoplasty, respectively. Poor esthetic outcome was significantly associated with initial fascial defect ≥2.5cm in size (p=0.03). Present technique is a simple and cosmetically appealing repair for umbilical cord hernias and small omphaloceles, especially effective when the size of the fascial defect is less than 2.5cm. IV (Treatment Study). Copyright © 2017 Elsevier Inc. All rights reserved.

Successful three stage repair of a large congenital abdominal region defect

Directory of Open Access Journals (Sweden)

Vaidehi Agrawal

2015-06-01

Full Text Available We present two infants born with large, right upper quadrant defects which cannot be categorized as either a gastroschisis or omphalocele. We successfully managed one infant with a three stage repair using polytetrafluoroethylene (PTFE patch, porcine urinary bladder matrix (UBM and delayed surgical closure. The second infant passed away due to parental consent care withdrawal.

Lifetime Costs and Outcomes of Repair of Tetralogy of Fallot Compared to Natural Progression of the Disease: Great Ormond Street Hospital Cohort

Science.gov (United States)

Hunter, Rachael Maree; Isaac, Mark; Frigiola, Alessandra; Blundell, David; Brown, Kate; Bull, Kate

2013-01-01

Background Tetralogy of Fallot is a congenital heart disease that requires surgical repair without which survival through childhood is extremely rare. The aim of this paper is to use data from the mandatory follow-up of patients with Tetralogy of Fallot to model the health-related costs and outcomes over the first 55-years of life. Method A decision analytical model was developed to establish costs and outcomes for patients up to 55 years after diagnosis and first repair of Tetralogy of Fallot compared to natural progression. Data from Adult Congenital Heart Disease (ACHD) centres that follow up Tetralogy of Fallot patients and Great Ormond Street Hospital (GOSH), London, United Kingdom (UK) medical records was used to establish the cost and effectiveness of current interventions. Data from a Czech cohort was used for the natural, no intervention condition. Results The average cost per patient of a repair for Tetralogy of Fallot was £26,938 (SE = £4,140). The full life time cost per patient, with no discount rate, was £65,310 (95% CI £64,981–£65,729); £56,559 discounted (95% CI £56,159–£56,960). Patients with a repair had an average of 35 Quality Adjusted Life Years (QALYs) per patient over 55 years undiscounted and 20.16 QALYs discounted. If the disorder was left to take its natural course, patients on average had a total of 3 QALYs per patient with no discount rate and 2.30 QALYs discounted. Conclusion A model has been developed that provides an estimate of the value for money of an expensive repair of a congenital heart disease. The model could be used to test the cost-effectiveness of making amendments to the care pathway. PMID:23533645

Congenital high airway obstruction syndrome in the breech presentation managed by ex utero intrapartum treatment procedure after intraoperative external cephalic version.

Science.gov (United States)

Miwa, Ichiro; Sase, Masakatsu; Nakamura, Yasuhiko; Hasegawa, Keiko; Kawasaki, Masahiro; Ueda, Kazuyuki

2012-05-01

Congenital high airway obstruction syndrome (CHAOS) caused by laryngeal atresia was diagnosed by prenatal ultrasound in a male fetus at 26 weeks of gestation. Findings included massive ascites, subcutaneous edema, enlarged hyperechogenic lungs with diaphragmatic inversion, dilated trachea, polyhydramnios, and breech presentation. Those findings of CHAOS spontaneously returned to normal by 33 weeks of gestation. However, the placenta was localized to the anterior uterine wall. In addition, the fetal position had been breech until delivery. At 36 weeks of gestation, a planned ex utero intrapartum treatment (EXIT) procedure was performed following intraoperative external cephalic version (ECV) in which the fetus was approached from the posterior wall of the uterus. Laryngoscopy revealed the predicted laryngeal obstruction, and tracheostomy was placed. Intraoperative ECV may be a useful technique in breech presentation before EXIT procedure. © 2012 The Authors. Journal of Obstetrics and Gynaecology Research © 2012 Japan Society of Obstetrics and Gynecology.

Gastric volvulus in childhood.

Directory of Open Access Journals (Sweden)

Karande T

1997-04-01

Full Text Available Gastric volvulus is an uncommon condition more so in the paediatric age group. The cause of gastric volvulus may be idiopathic or secondary to various congenital or acquired conditions. In this short series of three patients, one had volvulus which was due to ligamentous laxity and mobile spleen, second had congenital postero-lateral diaphragmatic defect and the third had hiatus hernia.

Intrathoracic Gastric Volvulus presenting with GIT Bleed

OpenAIRE

Rahul Kadam; VSV Prasad

2017-01-01

Intrathoracic gastric volvulus in neonatal period is a life-threatening surgical emergency. We report a case of neonate with respiratory distress and GI bleeding who was diagnosed to have congenital diaphragmatic eventration with Intrathoracic gastric volvulus.

Vocal cord collapse during phrenic nerve-paced respiration in congenital central hypoventilation syndrome.

Science.gov (United States)

Domanski, Mark C; Preciado, Diego A

2012-01-01

Phrenic nerve pacing can be used to treat congenital central hypoventilation syndrome (CCHS). We report how the lack of normal vocal cord tone during phrenic paced respiration can result in passive vocal cord collapse and produce obstructive symptoms. We describe a case of passive vocal cord collapse during phrenic nerve paced respiration in a patient with CCHS. As far as we know, this is the first report of this etiology of airway obstruction. The patient, a 7-year-old with CCHS and normal waking vocal cord movement, continued to require nightly continuous positive airway pressure (CPAP) despite successful utilization of phrenic nerve pacers. On direct laryngoscopy, the patient's larynx was observed while the diaphragmatic pacers were sequentially engaged. No abnormal vocal cord stimulation was witnessed during engaging of either phrenic nerve stimulator. However, the lack of normal inspiratory vocal cord abduction during phrenic nerve-paced respiration resulted in vocal cord collapse and partial obstruction due to passive adduction of the vocal cords through the Bernoulli effect. Bilateral phrenic nerve stimulation resulted in more vocal cord collapse than unilateral stimulation. The lack of vocal cord abduction on inspiration presents a limit to phrenic nerve pacers.

Human Fanconi anemia monoubiquitination pathway promotes homologous DNA repair.

Science.gov (United States)

Nakanishi, Koji; Yang, Yun-Gui; Pierce, Andrew J; Taniguchi, Toshiyasu; Digweed, Martin; D'Andrea, Alan D; Wang, Zhao-Qi; Jasin, Maria

2005-01-25

Fanconi anemia (FA) is a recessive disorder characterized by congenital abnormalities, progressive bone-marrow failure, and cancer susceptibility. Cells from FA patients are hypersensitive to agents that produce DNA crosslinks and, after treatment with these agents, have pronounced chromosome breakage and other cytogenetic abnormalities. Eight FANC genes have been cloned, and the encoded proteins interact in a common cellular pathway. DNA-damaging agents activate the monoubiquitination of FANCD2, resulting in its targeting to nuclear foci that also contain BRCA1 and BRCA2/FANCD1, proteins involved in homology-directed DNA repair. Given the interaction of the FANC proteins with BRCA1 and BRCA2, we tested whether cells from FA patients (groups A, G, and D2) and mouse Fanca-/- cells with a targeted mutation are impaired for this repair pathway. We find that both the upstream (FANCA and FANCG) and downstream (FANCD2) FA pathway components promote homology-directed repair of chromosomal double-strand breaks (DSBs). The FANCD2 monoubiquitination site is critical for normal levels of repair, whereas the ATM phosphorylation site is not. The defect in these cells, however, is mild, differentiating them from BRCA1 and BRCA2 mutant cells. Surprisingly, we provide evidence that these proteins, like BRCA1 but unlike BRCA2, promote a second DSB repair pathway involving homology, i.e., single-strand annealing. These results suggest an early role for the FANC proteins in homologous DSB repair pathway choice.

Clinical value of Tei index in pediatric patients with repaired tetralogy of Fallot.

Science.gov (United States)

Song, Bing; Qi, Quan; Liu, Ruisheng; Xing, Wang; Tang, Hanbo; Li, Yuanmin

2015-01-01

Tetralogy of Fallot is a congenital heart disease characterized by underdevelopment of the right ventricular infundibulum. Present study aimed to explore the clinic value of Tei index in assessing right ventricular function of pediatric patients with repaired Tetralogy of Fallot. A total of 45 pediatric patients with repaired Tetralogy of Fallot were recruited and classified into: group A (Tei index 0.7; n=13, aging 4-14 years). The right ventricular Tei index value was related to the clinical characteristics of Tetralogy of Fallot repair patients. Right ventricular Tei index was positively correlated with ventilation time, drainage volumes, and negatively with drug assistance and Intensive Care Unit (ICU) stay, although time for drug assistance and ICU stay were not statically different between group B and group C. There was no significant difference in left ventricular ejection fraction. Tei index is a sensitive indicator of right ventricular dysfunction, and has important clinical value to better our understanding of right ventricular function after tetralogy of Fallot repair.

Cornelia de Lange syndrome

DEFF Research Database (Denmark)

Boyle, M I; Jespersgaard, C; Brøndum-Nielsen, K

2015-01-01

, and hirsutism. Congenital anomalies include malformations of the upper limbs, gastrointestinal malformation/rotation, pyloric stenosis, diaphragmatic hernia, heart defects and genitourinary malformations. Gastroesophageal reflux disease is present in almost all patients. In addition to classic forms, milder...

The effect of four anaesthetic protocols for maintenance of anaesthesia on trans-diaphragmatic pressure in dogs.

Directory of Open Access Journals (Sweden)

Kiriaki Pavlidou

Full Text Available The diaphragm is the main inspiratory muscle and the main indicator of diaphragmatic contractility is the trans-diaphragmatic pressure (Pdi. The aim of this clinical study was to determine the effect of four different anaesthetic protocols on Pdi in anaesthetized healthy dogs. Eighty client-owned dogs were recruited in this clinical study. All the animals received dexmedetomidine and morphine as premedication and propofol for induction. Anaesthesia was maintained with one of four protocols: isoflurane (I, isoflurane with CRI of propofol (IP, isoflurane with CRI of fentanyl (IF, and isoflurane with CRI of ketamine (IK. When the surgical plane of anaesthesia was achieved, two balloon catheters were inserted, one into the stomach and one into the mid-third of the oesophagus for Pdi measurement. Pdi value was the highest in groups I (14.9±4.7 mmHg and IK (15.2±3.5 mmHg and the lowest in groups IP (12.2±3.2 mmHg and IF (12.0±5.9 mmHg. There was a statistically significant difference (p = 0.029 between groups IK and IF. PE'CO2 was statistically significantly higher (p<0.0005 in group IF (7.7±0.8 kPa than in group IK (6.5±0.7 kPa. Isoflurane alone or isoflurane with ketamine for the maintenance of anaesthesia seem to better preserve the respiratory function and the diaphragmatic contractility than isoflurane with either propofol or fentanyl in dogs. Therefore, the use of isoflurane or isoflurane with ketamine may be of benefit when animals with respiratory problems have to be anaesthetized.

Long-Term Follow-Up after Phrenic Nerve Reconstruction for Diaphragmatic Paralysis: A Review of 180 Patients.

Science.gov (United States)

Kaufman, Matthew R; Elkwood, Andrew I; Brown, David; Cece, John; Martins, Catarina; Bauer, Thomas; Weissler, Jason; Rezzadeh, Kameron; Jarrahy, Reza

2017-01-01

Background  Phrenic nerve reconstruction has been evaluated as a method of restoring functional activity and may be an effective alternative to diaphragm plication. Longer follow-up and a larger cohort for analysis are necessary to confirm the efficacy of this procedure for diaphragmatic paralysis. Methods  A total of 180 patients treated with phrenic nerve reconstruction for chronic diaphragmatic paralysis were followed for a median 2.7 years. Assessment parameters included: 36-Item Short Form Health Survey (SF-36) physical functioning survey, spirometry, chest fluoroscopy, electrodiagnostic evaluation, a five-item questionnaire to assess specific functional issues, and overall patient-reported outcome. Results  Overall, 134 males and 46 females with an average age of 56 years (range: 10-79 years) were treated. Mean baseline percent predicted values for forced expiratory volume in 1 second, forced vital capacity, vital capacity, and total lung capacity, were 61, 63, 67, and 75%, respectively. The corresponding percent improvements in percent predicted values were: 11, 6, 9, and 13% ( p ≤ 0.01; ≤ 0.01; ≤ 0.05; ≤ 0.01). Mean preoperative SF-36 physical functioning survey scores were 39%, and an improvement to 65% was demonstrated following surgery ( p  ≤ 0.0001). Nerve conduction latency, improved by an average 23% ( p  ≤ 0.005), and there was a corresponding 125% increase in diaphragm motor amplitude ( p  ≤ 0.0001). A total of 89% of patients reported an overall improvement in breathing function. Conclusion  Long-term assessment of phrenic nerve reconstruction for diaphragmatic paralysis indicates functional correction and symptomatic relief. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Clinical, echocardiographic, and radiographic findings of peritoneopericardial diaphragmatic hernia in two dogs and a cat

International Nuclear Information System (INIS)

Hay, W.H.; Woodfield, J.A.; Moon, M.A.

1989-01-01

Peritoneopericardial diaphragmatic hernia was diagnosed in 2 dogs and a cat. One dog was referred because of clinical signs of cardiac tamponade and acute decompensation from liver entrapment within the hernia. Surgical correction of the hernia alleviated clinical signs in all 3 animals. Echocardiography was used in combination with radiography to provide a rapid and accurate diagnosis

Partial hammock valve: surgical repair in adulthood.

Science.gov (United States)

Aramendi, José I; Rodríguez, Miguel A; Voces, Roberto; Pérez, Pedro; Rodrigo, David

2006-09-01

We describe a forme frustrée of hammock valve involving only the posterior mitral leaflet. Three adult patients were referred to surgery with the diagnosis of severe mitral regurgitation due to fibrosis of the posterior mitral leaflet. The final diagnosis was done intraoperatively. In all of them the posterior leaflet was attached to some accessory papillary muscles arranged en palisade, with three to four fused muscle heads producing restrictive leaflet motion in systole. Repair consisted in division of the papillary muscles, patch augmentation, and ring annuloplasty. This previously unreported lesion is congenital but manifests itself in adulthood.

Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation

NARCIS (Netherlands)

A.J.J.C. Bogers (Ad); S.J. Head (Stuart); P.L. de Jong (Peter); M. Witsenburg (Maarten); A.P. Kappetein (Arie Pieter)

2010-01-01

textabstractAim of the study: To investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.Methods: A total of 32 patients with CCTGA were

Radiographic diagnosis of diaphragmatic hernia: review of 60 cases in dogs and cats

International Nuclear Information System (INIS)

Hyun, C.B.

2004-01-01

Sixty cases of diaphragmatic hernia in dogs and cats were radiologically reviewed and categorized by their characteristic radiographic signs. Any particular predilection for age, sex, or breed was not observed. Liver, stomach and small intestine were more commonly herniated. At least two radiographs, at different angles, were required for a valid diagnosis, because some radiographic signs were not visible in a single radiographic view and more clearly detectable in two radiographic views

Mitral valve repair in a rheumatic population: an experience at AFIC/NIHD

International Nuclear Information System (INIS)

Ahmad, M.; Janjuja, A.M.; Iqbal, M.A.

2017-01-01

Objective: To analyze the outcome of mitral valve repair in rheumatic heart disease. Study Design: Retrospective analytical. Place and Duration of Study: Armed forces institute of cardiology/National institute of heart diseases Rawalpindi from Jan 2011 to June 2013. Material and Methods: In this retrospective study of 28 patients (15 females). The mean age was 20.2 +- 11.2 years (range, 11 to 55 years). The cause of mitral regurgitation was rheumatic in all patients, with no congenital myxomatous, infective or ischemic cases. About 68 percent patients were in New York Heart Association (NYHA) functional class III or IV. In all the cases posterior ring annuloplasty was done with flexible C- Shape rings with size ranging from 20 to 26 mm. Repairs included anterior leaflet repair with cusp shortening and resuspension (n=17), posterior leaflet repair with quardranguloplasty and transpositioning (n=6), commissuroplasty (n=2) and mixed anterior posterior leaflet repair (n=3). Results: There was no operative mortality. Follow-up was carried out for 10 months. The trivial mitral regurgitation was found in 3 cases (10.7 percent). Four (14.2 percent) patients required mitral valve replacement due to valve dysfunction. Conclusion: Mitral valve repair in rheumatic patients, with current techniques, can effectively correct functional and hemodynamic abnormalities with satisfactory results. (author)

Midline cervical cleft: a rare congenital anomaly.

Science.gov (United States)

Renukaswamy, Gayathri Mandya; Soma, Marlene A; Hartley, Benjamin E J

2009-11-01

A midline cervical cleft (MCC) is a rare congenital anomaly due to failure of fusion of the first and second branchial arches during embryogenesis. It may present as a midline defect of the anterior neck skin with a skin projection or sinus, or as a subcutaneous fibrous cord. This report evaluates the clinical features and surgical management of an MCC. We analyzed a series of 4 patients with an MCC successfully treated at Great Ormond Street Hospital for Children in London. Three male patients and 1 female patient between 4 and 11 months of age were found to have an MCC. Each patient presented with an erythematous, fibrous band of tissue extending between the chin and the suprasternal notch. Treatment comprised surgical excision of the lesion and Z-plasty repair. We present the embryology, common clinical presentation, investigations, differential diagnosis, and histology, along with a literature review, of this uncommon malformation of the anterior neck. An MCC is a differential diagnosis to consider when assessing a child with a midline cervical lesion. Early surgical excision with Z-plasty repair of the soft tissue defect is the treatment of choice to prevent long-term complications.

The natural history of congenitally corrected transposition of the great arteries.

Science.gov (United States)

Huhta, James

2011-01-01

The natural history of congenitally corrected transposition of the great arteries is of clinical/surgical importance once the fetus is born without heart block or signs of heart failure. Without significant tricuspid valve malformation, associated defects such as ventricular septal defect and left ventricular outflow obstruction can be repaired surgically. The mortality and long-term outcome appear to be linked strongly with the severity of tricuspid valve regurgitation. Some patients with an intact ventricular septum and no right ventricular dysfunction will live long lives without detection, and some women will successfully complete pregnancy.

An unusual presentation of congenital bronchoesophageal fistula ...

African Journals Online (AJOL)

Autopsy revealed pus within the right lung, and a fistulous connection between the oesophagus and an intralobar sequestrated lung. No diaphragmatic hernia or intra-abdominal organ abnormality were seen, and an occipital meningomyelocoele was also confirmed. Key Words: Bronchoesophageal fistula, Hiatus hernia, ...

Surgical treatment of congenital mitral stenosis: medium-term results

Directory of Open Access Journals (Sweden)

Atik Fernando A.

2003-01-01

Full Text Available OBJECTIVE: For a cohort of patients with congenital mitral stenosis (CMS, to determine: patient outcomes, predictors of valve repairability and predictors of durability of valve repair. METHODS: From 1989 and 2002, 23 patients underwent surgical treatment of CMS, excluding those with common atrioventricular canal, and univentricular forms. The median age at operation was 15.5 months (range 2-204, and the median body weight was 11 Kg (range 4.5-51.6. Seventeen patients (73.9% had associated anomalies, including Shone's complex in nine (39.1% and pulmonary hypertension in 14 (60.9%. Mitral stenosis was severe in 14 patients (60.9% and moderate in the remaining (median trans-mitral gradient of 16 mmHg, range 8.5-32. Mitral valve repair was performed in 18 patients (78.3%, and valve replacement in five (21.7%. Repair techniques included papillary muscle splitting (n=10, excision of supravalvular ring (n=9 and commissurotomy (n=8. Twelve patients (52.2% required associated procedures. RESULTS: There were no early and late deaths at a mean follow-up of 58.5 ± 46.7 months (range 1-156. Mean hospital stay was 12.7 ± 8.2 days. There were no significant factors associated with unsuccessful valve repair. Actuarial freedom from reoperation at five years was 67.1% (CI 95%: 56.8% to 77.4%. The mitral valve repair group required reoperation in eight patients (44.4% (two early and six late, as opposed to one (20% in the replacement group. The presence of preoperative pulmonary hypertension was significantly related (p<0.005 to higher reoperation rates. All but two the followed patients are presently in functional class I and the echocardiography has shown less than 2+ mitral stenosis and/or regurgitation. CONCLUSION: Reoperations were the most important cause of morbidity at the medium-term follow-up of CMS. Preoperative pulmonary hypertension may predict the need for reoperation after mitral valve repair, which is the procedure of choice in CMS.

Cerebral oxygen delivery is reduced in newborns with congenital heart disease.

Science.gov (United States)

Lim, Jessie Mei; Kingdom, Theodore; Saini, Brahmdeep; Chau, Vann; Post, Martin; Blaser, Susan; Macgowan, Christopher; Miller, Steven P; Seed, Mike

2016-10-01

To investigate preoperative cerebral hemodynamics in newborns with congenital heart disease. We hypothesized that cerebral blood flow and oxygen delivery would be decreased in newborns with congenital heart disease compared with controls. Using a "feed-and-sleep" approach to performing neonatal magnetic resonance imaging, we measured cerebral blood flow by using a slice prescription perpendicular to the right and left internal carotid arteries and basilar artery at the level of the clivus. We calculated brain volume by segmenting a 3-dimensional steady-state free procession acquisition of the whole brain, allowing quantification of cerebral blood flow indexed to brain volume. Cerebral oxygen delivery was calculated as the product of cerebral blood flow and preductal systemic arterial oxygen content obtained via a combination of conventional pulse oximetry and laboratory analysis of venous blood samples for hemoglobin concentration. A complete set of measurements were obtained in 32 newborns with heart disease and 31 controls. There was no difference in gestational age between the heart disease and control groups. There was no difference in cerebral blood flow compared with controls (103.5 ± 34.0 vs 119.7 ± 40.4 mL/min), whereas cerebral oxygen delivery was significantly lower in the congenital heart disease subjects (1881 ± 625.7 vs 2712 ± 915.7 mLO2/min). Ten newborns with congenital heart disease had diffuse excessive high signal intensity in their white matter and 2 had white matter injury whereas another 5 had both. Newborns with unrepaired cyanotic congenital heart disease have decreased cerebral oxygen delivery due to arterial desaturation. If brain growth and development are adversely affected through oxygen conformance, our findings could have clinical implications in terms of timing of surgical repair. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

2013 update on congenital heart disease, clinical cardiology, heart failure, and heart transplant.

Science.gov (United States)

Subirana, M Teresa; Barón-Esquivias, Gonzalo; Manito, Nicolás; Oliver, José M; Ripoll, Tomás; Lambert, Jose Luis; Zunzunegui, José L; Bover, Ramon; García-Pinilla, José Manuel

2014-03-01

This article presents the most relevant developments in 2013 in 3 key areas of cardiology: congenital heart disease, clinical cardiology, and heart failure and transplant. Within the area of congenital heart disease, we reviewed contributions related to sudden death in adult congenital heart disease, the importance of specific echocardiographic parameters in assessing the systemic right ventricle, problems in patients with repaired tetralogy of Fallot and indication for pulmonary valve replacement, and confirmation of the role of specific factors in the selection of candidates for Fontan surgery. The most recent publications in clinical cardiology include a study by a European working group on correct diagnostic work-up in cardiomyopathies, studies on the cost-effectiveness of percutaneous aortic valve implantation, a consensus document on the management of type B aortic dissection, and guidelines on aortic valve and ascending aortic disease. The most noteworthy developments in heart failure and transplantation include new American guidelines on heart failure, therapeutic advances in acute heart failure (serelaxin), the management of comorbidities such as iron deficiency, risk assessment using new biomarkers, and advances in ventricular assist devices. Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

Congenital anomalies in Primorsky region.

Science.gov (United States)

Kiku, P; Voronin, S; Golokhvast, K

2015-01-01

has increased dramatically since 2000. This was due to the beginning of activities of medicogenetic service since 1998: the legal framework and information database were created, the flow of pregnant women was formed actively, and invasive prenatal diagnosis was introduced.Incidence of congenital anomalies has a reliable statistical association (chi-square) with bioclimatic zones and ecological situation. The high level of pathology is observed in both teenagers and children in the critical environmental situation areas, where there are enterprises of coal, mining and chemical industry, ship repair, construction, engineering sites, and areas with intensive chemical use and improvement of agriculture. For the most part these are cities and districts of the region where more than a half of the major manufacturing plants of the 1st and 2nd hazard classes are located. Exceeding the maximum allowable concentrations (MAC) of harmful substances in the air, soil, and water in these areas often reaches tenfold. It should be noted that in the territories with the critical environmental situation a relatively high level of malformations is observed in adolescents in the continental bioclimatic zone, and in children - on the coast, suggesting the influence of different climatic factors. Also a high level of the same congenital anomalies was revealed in children in bioclimatic zones of the coast and transition zones with the intense environmental situation.According to the results of the regression analysis, the varying degrees of influence of ecological and hygienic factors on the incidence of congenital anomalies were determined. In children, up to 77.3% of the spread of pathology depends on the complex of parameters of the environment; the proportion of the influence of sanitary and hygienic indicators is 63.1%, and natural and climatic indicators - 14.2%. Such factors as the characteristics of the soil condition, the level of air pollution, chemical pollution and adverse

Acute diaphragmatic paralysis caused by chest-tube trauma to phrenic nerve

Energy Technology Data Exchange (ETDEWEB)

Nahum, E.; Ben-Ari, J.; Schonfeld, T. [Pediatric Intensive Care Unit, Schneider Children' s Medical Center of Israel, Petah Tiqva (Israel); Horev, G. [Dept. of Diagnostic Radiology, Schneider Children' s Medical Center of Israel, Petah Tiqva (Israel); Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv (Israel)

2001-06-01

A 3{sup 1}/{sub 2}-year-old child developed unilateral diaphragmatic paralysis after chest drain insertion. Plain chest X-ray demonstrated paravertebral positioning of the chest-tube tip, and magnetic resonance imaging revealed hematomas in the region of the chest-tube tip and the phrenic nerve fibers. The trauma to the phrenic nerve was apparently secondary to malposition of the chest tube. This is a rare complication and has been reported mainly in neonates. Radiologists should notify the treating physicians that the correct position of a chest drain tip is at least 2 cm distant from the vertebrae. (orig.)

Acute diaphragmatic paralysis caused by chest-tube trauma to phrenic nerve

International Nuclear Information System (INIS)

Nahum, E.; Ben-Ari, J.; Schonfeld, T.; Horev, G.

2001-01-01

A 3 1 / 2 -year-old child developed unilateral diaphragmatic paralysis after chest drain insertion. Plain chest X-ray demonstrated paravertebral positioning of the chest-tube tip, and magnetic resonance imaging revealed hematomas in the region of the chest-tube tip and the phrenic nerve fibers. The trauma to the phrenic nerve was apparently secondary to malposition of the chest tube. This is a rare complication and has been reported mainly in neonates. Radiologists should notify the treating physicians that the correct position of a chest drain tip is at least 2 cm distant from the vertebrae. (orig.)

MR imaging of congenital heart diseases in adolescents and adults

Energy Technology Data Exchange (ETDEWEB)

Choe, Yeon Hyeon; Kang, I Seok; Park, Seung Woo; Lee, Heung Jae [Sungkwunkwan University School of Medicine, Seoul (Korea, Republic of)

2001-09-01

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of tetralogy of Fallot, and Senning's and Mustard's operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults.

Effects of eHealth physical activity encouragement in adolescents with complex congenital heart disease

DEFF Research Database (Denmark)

Klausen, Susanne Hwiid; Andersen, Lars L; Søndergaard, Lars

2016-01-01

OBJECTIVE: To assess benefit and harms of adding an eHealth intervention to health education and individual counseling in adolescents with congenital heart disease. DESIGN: Randomized clinical trial. SETTING: Denmark. PATIENTS: A total of 158 adolescents aged 13-16years with no physical activity...... restrictions after repaired complex congenital heart disease. INTERVENTIONS: PReVaiL consisted of individually tailored eHealth encouragement physical activity for 52weeks. All patients received 45min of group-based health education and 15min of individual counseling involving patients' parents. OUTCOMES......·kg(-1)·min(-1) (95% CI -2.66 to 1.36). Between-group differences at 1year in physical activity, generic health-related quality of life, and disease-specific quality of life were not statistically significant. CONCLUSIONS: Adding a tailored eHealth intervention to health education and individual...

Visceral subpleural hematoma of the left diaphragmatic surface following left upper division segmentectomy

Directory of Open Access Journals (Sweden)

Yasushi Mizukami

2017-10-01

Full Text Available Abstract Background Pulmonary visceral subpleural hematoma is rare. We report visceral subpleural hematoma of the left diaphragmatic surface following left upper division segmentectomy. This very rare case was difficult to distinguish from thoracic abscess. Case presentation A 68-year-old man with hypertension had undergone video-assisted thoracoscopic left upper division segmentectomy for suspected lung carcinoma. Deep vein thrombosis of the lower leg was identified and edoxaban, a so-called novel oral anticoagulant, was started on postoperative day 7. The chest drainage tube was removed on postoperative day 12 because of persistent air leakage, but fever appeared the same day. Computed tomography revealed a cavity with mixed air and fluid, so antibiotics were started on suspicion of abscess. Computed tomography-guided drainage was attempted, but proved unsuccessful. Fever continued and surgical investigation was therefore performed. Visceral subpleural hematoma was identified under the diaphragmatic surface of the left basal lung. We excised the pleura, then performed drainage and applied running sutures. The parenchyma and visceral pleura were covered with polyglycolic acid sheet and fibrin glue. Edoxaban was restarted on postoperative day 12 of video-assisted thoracoscopic surgery and no recurrence of hematoma has been revealed. Conclusions Visceral subpleural hematoma after thoracic surgery is extremely rare. Furthermore, correct diagnosis was difficult and surgery offered a good diagnostic and therapeutic procedure.

Surgical Outcomes of Pneumatic Compression Using Carbon Dioxide Gas in Thoracoscopic Diaphragmatic Plication.

Science.gov (United States)

Ahn, Hyo Yeong; Kim, Yeong Dae; Hoseok, I; Cho, Jeong Su; Lee, Jonggeun; Son, Joohyung

2016-12-01

Surgical correction needs to be considered when diaphragm eventration leads to impaired ventilation and respiratory muscle fatigue. Plication to sufficiently tense the diaphragm by VATS is not as easy to achieve as plication by open surgery. We used pneumatic compression with carbon dioxide (CO 2 ) gas in thoracoscopic diaphragmatic plication and evaluated feasibility and efficacy. Eleven patients underwent thoracoscopic diaphragmatic plication between January 2008 and December 2013 in Pusan National University Hospital. Medical records were retrospectively reviewed, and compared between the group using CO 2 gas and group without using CO 2 gas, for operative time, plication technique, duration of hospital stay, postoperative chest tube drainage, pulmonary spirometry, dyspnea score pre- and postoperation, and postoperative recurrence. The improvement of forced expiratory volume at 1 second in the group using CO 2 gas and the group not using CO 2 gas was 22.46±11.27 and 21.08±5.39 (p=0.84). The improvement of forced vital capacity 3 months after surgery was 16.74±10.18 (with CO 2 ) and 15.6±0.89 (without CO 2 ) (p=0.03). During follow-up (17±17 months), there was no dehiscence in plication site and relapse. No complications or hospital mortalities occurred. Thoracoscopic plication under single lung ventilation using CO 2 insufflation could be an effective, safe option to flatten the diaphragm.

Unusual presentation of adult Marfan syndrome as a complex diaphragmatic hiatus hernia.

Science.gov (United States)

Thakur, Shruti; Jhobta, Anupam; Sharma, Brij; Chauhan, Arun; Thakur, Charu S

2017-07-01

Marfan syndrome is multisystem connective tissue disorder that primarily involves the skeletal, cardiovascular, and ocular systems. The gastrointestinal complications in Marfan syndrome are rare, with only a few case reports described in the literature. We present a 25-year-old woman who presented with acute abdominal pain for 1 day. The imaging features revealed complex diaphragmatic hiatus hernia with organoaxial gastric volvulus. This is a unique case report about an adult patient with Marfan syndrome who presented with symptomatic paraesophageal hernia and organoaxial gastric volvulus. Copyright © 2014. Published by Elsevier Taiwan.

Unusual presentation of adult Marfan syndrome as a complex diaphragmatic hiatus hernia

Directory of Open Access Journals (Sweden)

Shruti Thakur

2017-07-01

Full Text Available Marfan syndrome is multisystem connective tissue disorder that primarily involves the skeletal, cardiovascular, and ocular systems. The gastrointestinal complications in Marfan syndrome are rare, with only a few case reports described in the literature. We present a 25-year-old woman who presented with acute abdominal pain for 1 day. The imaging features revealed complex diaphragmatic hiatus hernia with organoaxial gastric volvulus. This is a unique case report about an adult patient with Marfan syndrome who presented with symptomatic paraesophageal hernia and organoaxial gastric volvulus.

Results of aortic valve repair using decellularized bovine pericardium in congenital surgery.

Science.gov (United States)

Nordmeyer, Sarah; Murin, Peter; Schulz, Antonia; Danne, Friederike; Nordmeyer, Johannes; Kretzschmar, Johanna; Sumbadze, Daria; Schmitt, Katharina Rose Luise; Miera, Oliver; Cho, Mi-Young; Sinzobahamvya, Nicodeme; Berger, Felix; Ovroutski, Stanislav; Photiadis, Joachim

2018-04-30

The search for an optimal patch material for aortic valve reconstruction (AVR) is an ongoing challenge. In this study, we report our experience of AVR using decellularized bovine pericardial patch material in congenital heart surgery. Data of 40 consecutive patients who underwent AVR using the CardioCel® patch (Admedus Regen Pty Ltd, Perth, WA, Australia) between February 2014 and August 2016 were retrospectively reviewed. The median age of the patients at operation was 9 (2-34) years, and 18 patients were younger than 7 years. Twenty-six patients initially presented with aortic valve insufficiency (AI) and 14 with stenosis. Clinical and echocardiographic data were available until August 2017 for a median postoperative follow-up (FU) of 22 (6-42) months. Nine of 40 (23%) patients experienced an event during FU (death: n = 1, 2.5%; reoperation: n = 8, 20%). Overall, the probability of freedom from reoperation or death was 97 ± 3%, 76 ± 9% and 57 ± 12% at 12, 24 and 36 months of FU, respectively. Reason for reoperation was stenosis in 3 (37.5%) patients, insufficiency in 4 (50%) patients and 1 (12.5%) patient was diagnosed with aortic valve endocarditis. Of the remaining 31 patients, 2 patients are scheduled for reoperation (aortic valve stenosis: n = 1 and AI: n = 1) and 9 patients exhibit worsening of aortic valve function with moderate AI. Freedom from developing combined end point [death/reoperation/moderate degree of aortic valve dysfunction (aortic valve stenosis, AI)] after AVR was 92 ± 5%, 55 ± 9% and 28 ± 9% at 12, 24 and 36 months, respectively. AVR using decellularized bovine pericardial patch material in patients with congenital aortic valve disease show unsatisfactory results within the first 3 years of FU.

Congenital Hypothyroidism

Science.gov (United States)

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

Clinical and morphological characteristics of malformations in infants with congenital cytomegalovirus infection and congenital toxoplasmosis

Directory of Open Access Journals (Sweden)

L. Yu. Barycheva

2015-01-01

Full Text Available The results of following up infants with intrauterine infections and malformations were retrospectively analyzed. Infants with malformations were diagnosed as having congenital cytomegalovirus infection and congenital toxoplasmosis in 127 and 69 cases, respectively. The aim of the study was to characterize malformations in infants with congenital cytomegalovirus and congenital Toxoplasma infections. The infants with malformations in congenital cytomegalovirus infection were found to have higher mortality rates (61,4% than those with congenital toxoplasmosis (34,8%. Postmortem analysis indicated that there was a predominance of embryopathies in infants with congenital cytomegalovirus infection and that of fetopathies in those with congenital toxoplasmosis. The dead infants with congenital cytomegalovirus infection had more commonly developed visceral defects, including heart diseases, pneumopathies, gastrointestinal and genitourinary abnormalities; fetopathies of the central nervous system and eye were prevalent in congenital toxoplasmosis. The surviving children with congenital toxoplasmosis were more frequently observed to have disabling CNS and ocular sequels as obstructive hydrocephalus, infantile cerebral palsy, complete or partial blindness, and cerebrasthenic disorders than those with congenital cytomegalovirus infection. 

Mitochondrial disorders in congenital myopathies

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D. A. Kharlamov

2014-01-01

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

Successful endoscopic endonasal repair of nasal meningoencephalocele in a 21-day-old neonate

OpenAIRE

Mohindra, Satyawati; Mohindra, Sandeep; Patro, Sourabha Kumar

2015-01-01

Introduction: Indications of surgical intervention in congenital nasal meningoencephaloceles includes presence of cerebro spinal fluid rhinorrhea having a risk of causing meningitis, episodes of prior meningitis and bilateral nasal obstruction causing respiratory difficulty in these obligate nasal breathers. Many authors would like to wait till the patient attains the age of 2 to 3 years for repair of the defect due to surgical feasibility. However, early intervention prevents further episode...

Klinisch denken en beslissen in de praktijk. Een pasgeborene met dyspnoe

NARCIS (Netherlands)

Nusmeier, A.; de Jongste, J. C.; Bos, A. P.; Lam, J.; Hoekstra, M. O.

2003-01-01

A 2-day-old girl, born at term after an uneventful pregnancy and delivery, was admitted to the paediatric intensive care unit with dyspnoea and tachypnoea. Misleading interpretations of the radiological investigations suggested a congenital diaphragmatic hernia. The patient underwent laparotomy but

African Journal of Paediatric Surgery - Vol 8, No 3 (2011)

African Journals Online (AJOL)

Preemptive analgesia in paediatric surgery · EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT ... Comparison between late-presenting and isolated neonatal congenital diaphragmatic hernias · EMAIL FULL TEXT EMAIL FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL ...

Paediatric thoracoscopy: State of the art | Van Niekerk | South ...

African Journals Online (AJOL)

Many advanced procedures, including lobectomy, repair of tracheo-oesophageal fistula, excision of mediastinal tumours and diaphragmatic hernia repairs, are being done routinely in paediatric surgery centres around the world. This article reviews the state of the art of thoracoscopic surgery in children. The author selected ...

Cytomegalovirus Congenital Cataract

Directory of Open Access Journals (Sweden)

Ridha Wahyutomo

2011-06-01

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

Ultrasound assessment of diaphragmatic function in patients with amyotrophic lateral sclerosis.

Science.gov (United States)

Fantini, Riccardo; Mandrioli, Jessica; Zona, Stefano; Antenora, Federico; Iattoni, Andrea; Monelli, Marco; Fini, Nicola; Tonelli, Roberto; Clini, Enrico; Marchioni, Alessandro

2016-07-01

Evaluation of diaphragm function in Amyotrophic Lateral Sclerosis (ALS) is critical in determining when to commence non-invasive mechanical ventilation (NIV). Currently, forced vital capacity (FVC) and sniff nasal inspiratory pressure (SNIP) are volitional measures for this evaluation, but require collaboration and are poorly specific. The primary aim of this study was to assess whether diaphragmatic thickness measured by ultrasound (US) correlates with lung function impairment in ALS patients. The secondary aim was then to compare US diaphragm thickness index (ΔTdi) with a new parameter (ΔTmax index). 41 patients with ALS and 30 healthy subjects were enrolled in the study. All subjects underwent spirometry, SNIP and diaphragm US evaluation, while arterial blood gases were measured in some patients only. US assessed diaphragm thickness (Tdi) at tidal volume (Vt) or total lung capacity (TLC), and their ratio (ΔTmax) were recorded. Changes (Δ) in Tdi indices during tidal volume (ΔTdiVt) and maximal inspiration (ΔTdiTLC) were also assessed. ΔTdiTLC (p <0.001) and ΔTmax (p = 0.007), but not ΔTdiVt, differed between patients and controls. Significant correlation (p < 0.05) was found between ΔTdiTLC, ΔTmax and FVC. The ROC curve analysis for comparison of individual testing showed better accuracy with Δtmax than with ΔtdiTLC for FVC (AUC 0.76 and 0.27) and SNIP (AUC 0.71 and 0.25). Diaphragm thickness assessed by ultrasound significantly correlates with global respiratory alterations in patients with ALS. ΔTmax represents a new US index of early diaphragmatic dysfunction, better related with the routinely performed lung function tests. © 2016 Asian Pacific Society of Respirology.

75 FR 63498 - Eunice Kennedy Shriver National Institute of Child Health & Human Development; Notice of Closed...

Science.gov (United States)

2010-10-15

... National Institute of Child Health & Human Development; Notice of Closed Meeting Pursuant to section 10(d... Institute of Child Health and Human Development Special Emphasis Panel; Congenital Diaphragmatic Hernia..., Eunice Kennedy Shriver National Institute of Child Health and Human Development, NIH, 6100 Executive Blvd...

Diafragmahernie

DEFF Research Database (Denmark)

Hejmdal, Anders; Dakhil, Akram; Boel, Thomas

2009-01-01

-/pneumothorax. The patient's condition deteriorated, a life-threatening state of sepsis developed and she was transferred to a surgery facility where an incarcerated congenital diaphragmatic hernia was established. After surgery she was discharged with a temporary ostomy, but otherwise feeling well. Udgivelsesdato: 2009-Jan...

When referring physicians and researchers disagree on equipoise : the TOTAL trial experience

NARCIS (Netherlands)

Rodrigues, H. C. M. L.; Deprest, J.; v. d. Berg, P. P.

Objective In this article, we reflect on whether randomized controlled trials (RCTs) are adequate for the clinical evaluation of maternal-fetal surgery for congenital diaphragmatic hernia (CDH), focusing on the role of patients' preferences in the setting up of research protocols, on the requirement

Congenital Abnormalities

Science.gov (United States)

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

Interval between injection of contrast material and positive contrast cheliography affects accurate diagnosis of diaphragmatic hernia

Directory of Open Access Journals (Sweden)

Ismail A. Sen

2015-01-01

Full Text Available The aim of this study was to evaluate the clinical, surgical and diagnostic imaging findings in 11 cats and 3 dogs with suspected acute and chronic traumatic diaphragmatic hernia, and to compare the results of positive contrast cheliography (peritoneography taken immediately and 5 min after the injection of contrast material. Thoracic and abdominal radiography, ultrasonography, and positive contrast cheliography of all animals were performed. Eight cases were considered as acute and six cases were considered chronic. The contrast images taken immediately after the injection of contrast material revealed the contrast material in the thoracic cavity in 8/8 acute trauma patients, but in none of the chronic cases. In 5/6 of these cases contrast material was seen in the thoracal cavity only in additional images taken after 5 min. One patient was diagnosed with FIP and excluded from the study. Twelve cases had complete resolution and one animal died during the early postoperative period. Our results suggest that positive contrast cheliography performed immediately after the injection of contrast material may not reveal chronic cases of diaphragmatic hernia and a second imaging (or imaging after 5 min is indicated in order not to overlook chronic cases.

Surgical Outcomes of Pneumatic Compression Using Carbon Dioxide Gas in Thoracoscopic Diaphragmatic Plication

Directory of Open Access Journals (Sweden)

Hyo Yeong Ahn

2016-12-01

Full Text Available Background: Surgical correction needs to be considered when diaphragm eventration leads to impaired ventilation and respiratory muscle fatigue. Plication to sufficiently tense the diaphragm by VATS is not as easy to achieve as plication by open surgery. We used pneumatic compression with carbon dioxide (CO2 gas in thoracoscopic diaphragmatic plication and evaluated feasibility and efficacy. Methods: Eleven patients underwent thoracoscopic diaphragmatic plication between January 2008 and December 2013 in Pusan National University Hospital. Medical records were retrospectively reviewed, and compared between the group using CO2 gas and group without using CO2 gas, for operative time, plication technique, duration of hospital stay, postoperative chest tube drainage, pulmonary spirometry, dyspnea score pre- and postoperation, and postoperative recurrence. Results: The improvement of forced expiratory volume at 1 second in the group using CO2 gas and the group not using CO2 gas was 22.46±11.27 and 21.08±5.39 (p=0.84. The improvement of forced vital capacity 3 months after surgery was 16.74±10.18 (with CO2 and 15.6±0.89 (without CO2 (p=0.03. During follow-up (17±17 months, there was no dehiscence in plication site and relapse. No complications or hospital mortalities occurred. Conclusion: Thoracoscopic plication under single lung ventilation using CO2 insufflation could be an effective, safe option to flatten the diaphragm.

Turbine repair process, repaired coating, and repaired turbine component

Science.gov (United States)

Das, Rupak; Delvaux, John McConnell; Garcia-Crespo, Andres Jose

2015-11-03

A turbine repair process, a repaired coating, and a repaired turbine component are disclosed. The turbine repair process includes providing a turbine component having a higher-pressure region and a lower-pressure region, introducing particles into the higher-pressure region, and at least partially repairing an opening between the higher-pressure region and the lower-pressure region with at least one of the particles to form a repaired turbine component. The repaired coating includes a silicon material, a ceramic matrix composite material, and a repaired region having the silicon material deposited on and surrounded by the ceramic matrix composite material. The repaired turbine component a ceramic matrix composite layer and a repaired region having silicon material deposited on and surrounded by the ceramic matrix composite material.

Congenital tuberculosis

African Journals Online (AJOL)

Prof Ezechukwu

2012-06-20

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

Congenital megaprepuce: a 12-year experience (52 cases) of this specific form of buried penis.

Science.gov (United States)

Rod, Julien; Desmonts, Alexis; Petit, Thierry; Ravasse, Philippe

2013-12-01

Congenital megaprepuce is a specific form of buried penis. Children affected by this malformation usually suffer from a subjective difficulty in voiding which is the main indication for early surgical correction. The aim of this study was to report a single center's 12-year experience in the treatment of megaprepuce in children, describing the surgical procedure we used to treat it and the results. We retrospectively reviewed the charts of all children who underwent congenital megaprepuce repair between January 1999 and August 2011 in our institution. Fifty-two children were operated during the study period. Our single surgical technique, not an original one, consists of fixing the penile shaft at the base of the penis, and widely reducing the inner prepuce. Four children (8%) underwent revision surgery but according to our criteria a very good result was observed in only 23 patients, and a less good but still acceptable result in 25 patients. Our 12-year experience in the surgical treatment of congenital megaprepuce demonstrated satisfying results from a safe and simple surgical technique, but to achieve the optimum result remains difficult. Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Analysis of diaphragmatic movement before and after pulmonary rehabilitation using fluoroscopy imaging in patients with COPD

Directory of Open Access Journals (Sweden)

Chun EM

2015-01-01

Full Text Available Eun Mi Chun,1 Soo Jeong Han,2 Hitesh N Modi3 1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul, Republic of Korea; 2Rehabilitation Medicine, Ewha Womans University School of Medicine, Seoul, Republic of Korea; 3Scoliosis Research Institute, Department of Orthopedics, Korea University Guro Hospital, Seoul, Republic of Korea Background: The diaphragm is the principal inspiratory muscle. The purpose of this study was to assess improvements in diaphragmatic movement before and after pulmonary rehabilitation in patients with chronic obstructive pulmonary disease (COPD, using a fluoroscopy-guided chest X-ray.Patients and methods: Among 117 patients with COPD receiving pulmonary rehabilitation who underwent the initial fluoroscopy-guided chest X-ray and pulmonary function test, 37 of those patients who underwent both initial and follow-up fluoroscopy and pulmonary function tests were enrolled in this study. After hospital education, participants received pulmonary rehabilitation through regular home-based training for at least 3 months by the same physiatrist. We assessed the changes in diaphragm area with fluoroscopy-guided posteroanterior chest X-rays between pre- and postpulmonary rehabilitation. To minimize radiation hazards for subjects, the exposure time for fluoroscopy to take chest X-rays was limited to less than 5 seconds.Results: There were significant improvements (2,022.8±1,548.3 mm² to 3,010.7±1,495.6 mm² and 2,382.4±1,475.9 mm² to 3,315.9±1,883.5 mm²; right side P=0.001 and left side P=0.019, respectively in diaphragmatic motion area during full inspiration and expiration in both lungs after pulmonary rehabilitation. Pulmonary function tests showed no statistically significant difference between pre- and postpulmonary rehabilitation.Conclusion: The study suggests that the strategy to assess diaphragm movement using fluoroscopy is a relatively effective tool for the evaluation of pulmonary

Diaphragmatic mobility: relationship with lung function, respiratory muscle strength, dyspnea, and physical activity in daily life in patients with COPD.

Science.gov (United States)

Rocha, Flávia Roberta; Brüggemann, Ana Karla Vieira; Francisco, Davi de Souza; Medeiros, Caroline Semprebom de; Rosal, Danielle; Paulin, Elaine

2017-01-01

To evaluate diaphragmatic mobility in relation to lung function, respiratory muscle strength, dyspnea, and physical activity in daily life (PADL) in patients with COPD. We included 25 patients with COPD, classified according to the Global Initiative for Chronic Obstructive Lung Disease criteria, and 25 healthy individuals. For all of the participants, the following were evaluated: anthropometric variables, spirometric parameters, respiratory muscle strength, diaphragmatic mobility (by X-ray), PADL, and the perception of dyspnea. In the COPD group, diaphragmatic mobility was found to correlate with lung function variables, inspiratory muscle strength, and the perception of dyspnea, whereas it did not correlate with expiratory muscle strength or PADL. In patients with COPD, diaphragmatic mobility seems to be associated with airway obstruction and lung hyperinflation, as well as with ventilatory capacity and the perception of dyspnea, although not with PADL. Avaliar a relação da mobilidade diafragmática com a função pulmonar, força muscular respiratória, dispneia e atividade física de vida diária (AFVD) em pacientes com DPOC. Foram avaliados 25 pacientes com diagnóstico de DPOC, classificados de acordo com critérios da Global Initiative for Chronic Obstructive Lung Disease, e 25 indivíduos saudáveis. Todos foram submetidos às seguintes avaliações: mensuração antropométrica, espirometria, força muscular respiratória, mobilidade diafragmática (por radiografia), AFVD e percepção de dispneia. No grupo DPOC, houve correlações da mobilidade diafragmática com variáveis de função pulmonar, força muscular inspiratória e percepção de dispneia. Não houve correlações da mobilidade diafragmática com força muscular expiratória e AFVD. A mobilidade diafragmática parece estar associada tanto com a obstrução das vias aéreas quanto com a hiperinsuflação pulmonar em pacientes com DPOC, assim como com a capacidade ventilatória e percep

The problem of sterility in men and women after wide area sub-diaphragmatic irradiation

International Nuclear Information System (INIS)

Dana, M.; Weisgerber, C.; Teillet, F.; Desprez-Curely, J.P.; Goguel, A.; Chotin, G.; Bernard, J.

1976-01-01

Sub-diaphragmatic irradiation in an upside down Y pattern for Hodgkin's disease results in sterility in the woman. Protection consists of irradiating the lumbar chain only when possible or by displacement of the ovary before irradiation, and laterally for preference. Although subsequent pregnancy is then possible, the genetic risk remains. In the male, Y irradiation results in prolonged virtually complete azoospermia. Associated chemotherapy also causes definitive sterility in the male. Collection for a sperm bank before treatment is advised [fr

Diaphragmatic Breathing during Virtual Reality Exposure Therapy for Aviophobia: Functional Coping Strategy or Avoidance Behavior?

OpenAIRE

Mühlbauer, Andreas; Shiban, Youssef; Diemer, Julia Elisabeth; Müller, Jana; Brütting-Schick, Johanna; Pauli, Paul

2017-01-01

Background Although there is solid evidence for the efficacy of in vivo and virtual reality (VR) exposure therapy for a specific phobia, there is a significant debate over whether techniques promoting distraction or relaxation have impairing or enhancing effects on treatment outcome. In the present pilot study, we investigated the effect of diaphragmatic breathing (DB) as a relaxation technique during VR exposure treatment. Method Twenty-nine patients with aviophobia were randomly a...

Decreased intracellular [Ca2+ ] coincides with reduced expression of Dhprα1s, RyR1, and diaphragmatic dysfunction in a rat model of sepsis.

Science.gov (United States)

Wang, Meng-Meng; Hao, Li-Ying; Guo, Feng; Zhong, Bin; Zhong, Xiao-Mei; Yuan, Jing; Hao, Yi-Fei; Zhao, Shuang; Sun, Xue-Fei; Lei, Ming; Jiao, Guang-Yu

2017-12-01

Sepsis can cause decreased diaphragmatic contractility. Intracellular calcium as a second messenger is central to diaphragmatic contractility. However, changes in intracellular calcium concentration ([Ca 2+ ]) and the distribution and co-localization of relevant calcium channels [dihydropyridine receptors, (DHPRα1s) and ryanodine receptors (RyR1)] remain unclear during sepsis. In this study we investigated the effect of changed intracellular [Ca 2+ ] and expression and distribution of DHPRα1s and RyR1 on diaphragm function during sepsis. We measured diaphragm contractility and isolated diaphragm muscle cells in a rat model of sepsis. The distribution and co-localization of DHPRα1s and RyR1 were determined using immunohistochemistry and immunofluorescence, whereas intracellular [Ca 2+ ] was measured by confocal microscopy and fluorescence spectrophotometry. Septic rat diaphragm contractility, expression of DHPRα1s and RyR1, and intracellular [Ca 2+ ] were significantly decreased in the rat sepsis model compared with controls. Decreased intracellular [Ca 2+ ] coincides with diaphragmatic contractility and decreased expression of DHPRα1s and RyR1 in sepsis. Muscle Nerve 56: 1128-1136, 2017. © 2017 Wiley Periodicals, Inc.

The Perioperative Use of Dexmedetomidine in Pediatric Patients with Congenital Heart Disease: An Analysis from the Congenital Cardiac Anesthesia Society-Society of Thoracic Surgeons Congenital Heart Disease Database.

Science.gov (United States)

Schwartz, Lawrence I; Twite, Mark; Gulack, Brian; Hill, Kevin; Kim, Sunghee; Vener, David F

2016-09-01

Dexmedetomidine is a selective α-2 receptor agonist with a sedative and cardiopulmonary profile that makes it an attractive anesthetic for pediatric patients with congenital heart disease (CHD). Although several smaller, single-center studies suggest that dexmedetomidine use is gaining traction in the perioperative setting in children with CHD, there are limited multicenter data, with little understanding of the variation in use across age ranges, procedural complexity, and centers. The aim of this study was to use the Congenital Cardiac Anesthesia Society-Society of Thoracic Surgeons (CCAS-STS) registry to describe patient- and center-level variability in the use of dexmedetomidine in the perioperative setting in children with heart disease. To describe the use of dexmedetomidine in patients for CHD surgery, we analyzed all index cardiopulmonary bypass operations entered in the CCAS-STS database from 2010 to 2013. Patient and operative characteristics were compared between those who received intraoperative dexmedetomidine and those who did not. Selective outcomes associated with dexmedetomidine use were also described. Of the 12,142 operations studied, 3600 (29.6%) received perioperative dexmedetomidine (DEX) and 8542 did not receive the drug (NoDEX). Patient characteristics were different between the 2 groups with the DEX group generally exhibiting both lower patient and procedural risk factors. Patients who received dexmedetomidine were more likely to have a lower level of Society of Thoracic Surgeons mortality complexity than patient who did not receive it. Consistent with their overall lower risk profile, children in the DEX group also demonstrated improved outcomes compared with patients who did not receive dexmedetomidine. We described the growing use of dexmedetomidine in children anesthetized for surgical repair of CHD. Dexmedetomidine appears to be preferentially given to older and larger children who are undergoing less complex CHD surgery. We believe

Trisomy 18 Syndrome with Incomplete Cantrell Syndrome

Directory of Open Access Journals (Sweden)

Yi-Jen Hou

2008-06-01

Full Text Available The pentalogy of Cantrell was first described in 1958 by Cantrell and coworkers, who reported five cases in which they described a pentad of findings including a midline supraumbilical thoracoabdominal wall defect, a defect of the lower sternum, abnormalities of the diaphragmatic pericardium and the anterior diaphragm, and congenital cardiac anomalies. Trisomy 18 has an incidence of about 0.3 per 1000 newborns. We present a case of trisomy 18 with incomplete Cantrell syndrome. The patient presented with hypogenesis of the corpus callosum, vermian-cerebellar hypoplasia (Dandy-Walker variant, ventricular septal defect, dextrocardia, patent ductus arteriosus, a defect of the lower sternum, a midline supraumbilical abdominal wall defect with omphalocele, congenital left posterior diaphragmatic hernia (Bochdalek hernia, micrognathia, low-set and malformed ears, rocker-bottom feet, dorsiflexed hallux, hypoplastic nails, short neck, and wrist deformity. Trisomy 18 syndrome was unusually combined with the pentalogy of Cantrell. We present this case because of its rarity and high risk of mortality.

Congenital orbital teratoma

OpenAIRE

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

2013-01-01

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

Pulmonary arterial hypertension in adult congenital heart disease.

Science.gov (United States)

Brida, Margarita; Gatzoulis, Michael A

2018-05-02

Pulmonary arterial hypertension (PAH) is commonly associated with congenital heart disease (CHD) and relates to type of the underlying cardiac defects and repair history. Large systemic to pulmonary shunts may develop PAH if untreated or repaired late. PAH, when present, markedly increases morbidity and mortality in patients with CHD. Significant progress has been made for patients with Eisenmenger syndrome in pathophysiology, prognostication and disease-targeting therapy (DTT), which needs to be applied to routine patient care. Patients with PAH-CHD and systemic to pulmonary shunting may benefit from late defect closure if pulmonary vascular resistance (PVR) is still normal or near normal. Patients with PAH and coincidental defects, or previous repair of CHD should be managed as those with idiopathic PAH. Patients with a Fontan circulation, despite not strictly fulfilling criteria for PAH, may have elevated PVR; recent evidence suggests that they may also benefit from DTT, but more data are required before general recommendations can be made. CHD-PAH is a lifelong, progressive disease; patients should receive tertiary care and benefit from a proactive DTT approach. Novel biomarkers and genetic advances may identify patients with CHD who should be referred for late defect closure and/or patients at high risk of developing PAH despite early closure in childhood. Ongoing vigilance for PAH and further controlled studies are clearly warranted in CHD. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Repair of aorto-left ventricular tunnel arising from the left sinus of valsalva.

Science.gov (United States)

Nezafati, Mohammad Hassan; Maleki, Mahmood Hosseinzadeh; Javan, Hadi; Zirak, Nahid

2010-05-01

Aortico-left ventricular tunnel (ALVT) is a rare congenital cardiac defect that bypasses the aortic valve via a para-valvular connection from the left ventricle to the aorta. In most cases, the tunnel arises from the right aortic sinus. In this case report, we are presenting a case of ALVT, of which the aortic orifice arose from the left aortic sinus, requiring special attention to avoid the left coronary artery injury at the time of surgical repair.

Comparing Life Quality Strategies and Emotion Regulation in People with Congenital and Non-Congenital Motor Disability

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Seyedeh Zeynab Miraghaei

2017-10-01

Full Text Available Objective: The purpose of the present study was to compare emotion regulation strategies and life quality of people with congenital and non-congenital motor disabilities. Method: This study is a casual-comparative study and its population consisted of all people with congenital and non-congenital motor disability in Kahrizak Charity Foundation in Tehran in 2016. To conduct the study, available sampling method was used, and congenital and non-congenital disabled people were selected (200 people. To collect data, Cognitive Emotion Regulation Scale by Granovsky and life quality questionnaire were used. Research hypotheses were tested using multivariate analysis of variance. Results: The findings of this study showed that there is a significant difference between emotion regulation components in people with congenital and non-congenital disabilities (p<0.05. Also, according to the findings, a significant difference was observed between life quality dimensions (physical and mental health in people with congenital and non-congenital disabilities (p<0.05. Conclusion: According to the significant difference between two groups of subjects, necessary measures regarding consultation and psychotherapy should be taken into consideration to let people benefit from desirable mental health level.

The role of the Fanconi anemia pathway in DNA repair and maintenance of genome stability

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Aleksandra M. Koczorowska

2014-05-01

Full Text Available The Fanconi anemia (FA pathway is one of the DNA repair systems involved in removal of DNA crosslinks. Proteins which belong to this pathway are crucial to the protection of genetic information, whereas disturbances in their function have serious implications for the whole organism. Biallelic mutations in FA genes are the cause of Fanconi anemia – a genetic disease which manifests itself through numerous congenital abnormalities, chromosomal instability and increased predisposition to cancer. The FA pathway is composed of fifteen proteins. Eight of them, in the presence of DNA interstrand crosslinks (ICLs, form a nuclear core complex responsible for monoubiquitination of FANCD2 and FANCI, which is a key step of ICL repair. FA proteins which are not involved in the monoubiquitination step participate in repair of DNA double strand breaks via homologous recombination. Some of the FA proteins, besides having a direct role in the repair of DNA damage, are engaged in replication, cell cycle control and mitosis. The unperturbed course of those processes determines the maintenance of genome stability.

Hernia diafragmática paraesternal de Morgagni-Larrey en adulto Morgagni-Larrey parasternal diaphragmatic hernia in the adult

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L. A. Arráez-Aybar

2009-05-01

Full Text Available Con una prevalencia de 0,3-0,5/1.000 nacimientos, la hernia diafragmática congénita (HDC sigue siendo una anomalía grave, no bien entendida, alta mortalidad y tratamiento no siempre efectivo. En España se ha informado de una frecuencia del 0,69%oo con una tendencia decreciente en el periodo 1980-2006 del 0,10%oo por año. No obstante, hasta un 5% se diagnostican en adultos durante la realización de un reconocimiento por otra causa. Presentamos un cuadro de vómitos de tres meses de evolución en una mujer de 74 años por hernia diafragmática paraesternal de Morgagni-Larrey (retrocondroesternal, retrocostoxifoidea, retroesternal, subcostal, subesternal o subcostoesternal, que nos ha permitido realizar una actualización de esta patología en adultos y de la morfogénesis del diafragma toracoabdominal. Es en la embriología del diafragma donde encontramos explicación de algunas de sus alteraciones morfológicas y características clínicas, si bien persisten aspectos confusos de la misma. También analizamos el grado de controversia que persiste en algunos aspectos de su tratamiento quirúrgico (vías de acceso, uso o no de mallas y reducción o no del saco herniario. Por lo general priman las técnicas mínimamente invasivas. Consideramos el abordaje laparoscópico como de elección en pacientes adultos con hernia paraesternal candidatos a la cirugía.With a prevalence of 0.3-0.5/1000 births, congenital diaphragmatic hernia (CDH remains a serious, poorly understood abnormality with a high mortality rate that cannot always be effectively managed. Its reported frequency in Spain is 0.69%oo with a yearly decreasing trend of 0.10%oo during the period 1980-2006. Up to 5% of cases are incidentally identified in adults undergoing studies for other reasons. We report the case of a 74-year-old woman with vomiting for three months due to parasternal diaphragmatic hernia of Morgagni-Larrey (retrochondrosternal, retrocostoxyphoid, retrosternal

Paresia diafragmática bilateral idiopática Idiopathic bilateral diaphragmatic paresis

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Mônica Corso Pereira

2006-10-01

Full Text Available Relata-se o caso de um paciente com dispnéia intensa ao se deitar, em que foram excluídas doenças pulmonares, neuromusculares ou cardíacas, cuja investigação revelou paresia diafragmática bilateral. Um sinal chave para o diagnóstico foi a evidência de respiração paradoxal com o doente em decúbito supino. Havia piora da oxigenação e da capacidade vital forçada com a mudança da posição ortostática para supina. A fluoroscopia ortostática foi normal. A pressão inspiratória máxima estava muito reduzida. A estimulação elétrica transcutânea do diafragma foi normal, e a eletroestimulação do nervo frênico mostrou ausência de resposta, permitindo o diagnóstico de paresia bilateral do diafragma.We report the case of a patient with severe dyspnea upon reclining. Lung disease, neuromuscular disorders and heart disease were ruled out. However, during the course of the investigation, bilateral diaphragmatic paresis was discovered. A key sign leading to the diagnosis was evidence of paradoxical respiration in the dorsal decubitus position. When the patient was moved from the orthostatic position to the dorsal decubitus position, oxygenation and forced vital capacity worsened. The orthostatic fluoroscopy was normal. Maximal inspiratory pressure was severely reduced. The responses to transcutaneous electric stimulation of the diaphragm were normal. However, electric stimulation of the phrenic nerve produced no response, leading to the diagnosis of bilateral diaphragmatic paresis.

Key aspects congenital infection

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Yu. V. Lobzin

2014-01-01

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

Magnetic resonance imaging of complex congenital heart disease in aduits; Magnetresonanztomographie komplexer kongenitaler Herzerkrankungen beim Erwachsenen

Energy Technology Data Exchange (ETDEWEB)

Bremerich, J. [San Francisco Univ, CA (United States). Dept. of Radiology]|[Universitaetsklinik Basel (Switzerland). Abt. fuer Medizinische Radiologie; Wyttenbach, R.; Higgins, C.B. [San Francisco Univ, CA (United States). Dept. of Radiology; Buser, P. [Universitaetsklinik Basel (Switzerland). Abt. fuer Kardiologie; Steinbrich, W. [Universitaetsklinik Basel (Switzerland). Abt. fuer Medizinische Radiologie

1999-04-01

An increasing number of patients with complex congenital heart disease reaches adulthood, because treatment and patient outcome have improved considerably in recent years. Monitoring of these patients requires both definition of cardiac anatomy and assessment of function with good reproducibility. Complications after surgical repair such as restenoses of pulmonary arteries after surgical repair of Tetralogy of Fallot, ventricular hypertrophy, stenoses or leakage of baffles, or stenosis and aneurysms of anastomoses have to be detected at an early stage. Magnetic Resonance Imaging (MRI) is an excellent tool to monitor these patients because of its noninvasive nature, its good interstudy and interobserver reproducibility, and because it allows assessment of both cardiac anatomy and function. This paper reviews the current applications of MRI in complex congenital heart disease in adults. (orig.) [Deutsch] Durch Verbesserungen in der Diagnose und Therapie komplexer kongenitaler Herzerkrankungen kann eine zunehmende Anzahl von betroffenen Patienten das Erwachsenenalter erreichen. Diese Patienten benoetigen regelmaessige postoperative Verlaufskontrollen, um Komplikationen wie z.B. Restenosen der grossen Arterien, Stenosen, Lecks und Aneurysmata von Anastomosen, Ventrikelhypertrophie und -dilatation oder Klappendysfunktionen fruehzeitig zu erkennen und zu behandeln. Fuer nichtinvasive regelmaessige Verlaufskontrollen bietet sich die Magnetresonanztomographie (MRT) des Herzens an, da sie eine umfassende Untersuchung sowohl der Morphologie als auch der Funktion des Herzens mit guter Reproduzierbarkeit erlaubt. Die gegenwaertigen klinischen Anwendungen der MRT bei komplexen kongenitalen Herzerkrankungen beim Erwachsenen werden in dieser Uebersicht beschrieben. (orig.)

Right ventricular function late after total repair of tetralogy of Fallot

Energy Technology Data Exchange (ETDEWEB)

Straten, Alexander van; Roos, Albert de [Leiden University Medical Center, Department of Radiology, Leiden (Netherlands); Vliegen, Hubert W. [Leiden University Medical Center, Department of Cardiology, Leiden (Netherlands); Hazekamp, Mark G. [Leiden University Medical Center, Department of Cardiothoracic Surgery, Leiden (Netherlands)

2005-04-01

Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance. (orig.)

Right ventricular function late after total repair of tetralogy of Fallot

International Nuclear Information System (INIS)

Straten, Alexander van; Roos, Albert de; Vliegen, Hubert W.; Hazekamp, Mark G.

2005-01-01

Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance. (orig.)

Congenital symmastia revisited

DEFF Research Database (Denmark)

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

2012-01-01

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

Congenital Intrahepatic Portosystemic Shunts

Energy Technology Data Exchange (ETDEWEB)

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

2008-12-15

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

Diaphragmatic herniation following donor hepatectomy for living donor liver transplantation: a serious complication not given due recognition.

Science.gov (United States)

Lochan, Rajiv; Saif, Rehan; Ganjoo, Naveen; Sakpal, Mallikarjun; Panackal, Charles; Raja, Kaiser; Reddy, Jayanth; Asthana, Sonal; Jacob, Mathew

2017-11-01

A clear appreciation of benefits and risks associated with living donor hepatectomy is important to facilitate counselling for the donor, family, and recipient in preparation for living donor liver transplant (LDLT). We report a life-threatening complication occurring in one of our live liver donors at 12 weeks following hemi-liver donation. We experienced five donor complications among our first 50 LDLT: Clavien Grade 1, n=1; Clavien grade 2, n=3; and Clavien grade 3B, n=1. The one with Clavien grade 3B had a life-threatening diaphragmatic hernia occurring 12 weeks following hepatectomy. This was promptly recognized and emergency surgery was performed. The donor is well at 1-year follow-up. Here we provide a review of reported instances of diaphragmatic hernia following donor hepatectomy with an attempt to elucidate the pathophysiology behind such occurrence. Life-threatening donor risk needs to be balanced with recipient benefit and risk on a tripartite basis during the counselling process for LDLT. With increasing use of LDLT, we need to be aware of such life-threatening complication. Preventive measures in this regard and counselling for such complication should be incorporated into routine work-up for potential live liver donor.

Neonatal extracorporeal membrane oxygenation: Initial experience of Hospital de São João

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G. Rocha

2014-11-01

Full Text Available The purpose of this series is to report the initial ECMO experience of the Neonatal Intensive Care Unit of Hospital de São João. The first three clinical cases are reported. Case report 1: a 39 weeks gestational age girl with severe lung hypoplasia secondary to a bilateral congenital diaphragmatic hernia. Case report 2: a 39 weeks gestational age girl with a right congenital diaphragmatic hernia and a tracheal stenosis. Case report 3: a 34 weeks gestational age boy, with 61 days of life, with a Bordetella pertussis pneumonia, severe pulmonary hypertension, shock, hyperleukocytosis and seizures. Resumo: O objetivo desta série é apresentar a experiência inicial da Unidade de Cuidados Intensivos Neonatais do Hospital de São João com ECMO no recém-nascido. São apresentados os 3 primeiros casos. Caso 1: recém-nascido de 39 semanas de idade gestacional, com hipoplasia pulmonar severa secundária a hérnia diafragmática congénita bilateral. Caso 2: recém-nascido de 39 semanas de idade gestacional, com hérnia diafragmática congénita direita e estenose traqueal. Caso 3: pré-termo de 34 semanas de idade gestacional, sexo masculino, com 61 dias de vida, com pneumonia por Bordetella pertussis, hipertensão pulmonar severa, choque, hiperleucocitose e convulsões. Keywords: Extracorporeal membrane oxygenation, Newborn, Congenital diaphragmatic hernia, Tracheal stenosis, Bordetella pertussis infection, Palavras-chave: Oxigenação por membrana extracorporal, Recém-nascido, Hérnia diafragmática congénita, Estenose traqueal, Infeção por Bordetella pertussis

Congenital heart disease

Science.gov (United States)

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

Genetics Home Reference: congenital hypothyroidism

Science.gov (United States)

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

[Neonatal tumours and congenital malformations].

Science.gov (United States)

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

2008-06-01

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

Poorer right ventricular systolic function and exercise capacity in women after repair of tetralogy of fallot: a sex comparison of standard deviation scores based on sex-specific reference values in healthy control subjects.

Science.gov (United States)

Sarikouch, Samir; Boethig, Dietmar; Peters, Brigitte; Kropf, Siegfried; Dubowy, Karl-Otto; Lange, Peter; Kuehne, Titus; Haverich, Axel; Beerbaum, Philipp

2013-11-01

In repaired congenital heart disease, there is increasing evidence of sex differences in cardiac remodeling, but there is a lack of comparable data for specific congenital heart defects such as in repaired tetralogy of Fallot. In a prospective multicenter study, a cohort of 272 contemporary patients (158 men; mean age, 14.3±3.3 years [range, 8-20 years]) with repaired tetralogy of Fallot underwent cardiac magnetic resonance for ventricular function and metabolic exercise testing. All data were transformed to standard deviation scores according to the Lambda-Mu-Sigma method by relating individual values to their respective 50th percentile (standard deviation score, 0) in sex-specific healthy control subjects. No sex differences were observed in age at repair, type of repair conducted, or overall hemodynamic results. Relative to sex-specific controls, repaired tetralogy of Fallot in women had larger right ventricular end-systolic volumes (standard deviation scores: women, 4.35; men, 3.25; P=0.001), lower right ventricular ejection fraction (women, -2.83; men, -2.12; P=0.011), lower right ventricular muscle mass (women, 1.58; men 2.45; P=0.001), poorer peak oxygen uptake (women, -1.65; men, -1.14; Pstandard deviation scores in repaired tetralogy of Fallot suggest that women perform poorer than men in terms of right ventricular systolic function as tested by cardiac magnetic resonance and exercise capacity. This effect cannot be explained by selection bias. Further outcome data are required from longitudinal cohort studies.

Modified nuss procedure in concurrent repair of pectus excavatum and open heart surgery.

Science.gov (United States)

Sacco Casamassima, Maria Grazia; Wong, Ling Ling; Papandria, Dominic; Abdullah, Fizan; Vricella, Luca A; Cameron, Duke E; Colombani, Paul M

2013-03-01

Pectus excavatum (PE) can be associated with congenital and acquired cardiac disorders that also require surgical repair. The timing and specific surgical technique for repair of PE remains controversial. The present study reports the experience of combined repair of PE and open heart surgery at Johns Hopkins Hospital. A retrospective case review was conducted of all patients who presented for repair of PE deformity while undergoing concurrent open heart surgery from 1998 through 2011. A total of 9 patients met inclusion criteria. All patients had a connective tissue disorder. Repair of PE was performed by modified Nuss technique after completion of the cardiac procedure, performed through a median sternotomy. Open heart procedures were either aortic root replacement or mitral valvuloplasty. Eight patients had bar removal after an average period of 30.3 months. No PE recurrence, bar displacement, or upper sternal depression was reported in 7 patients. Postoperatively, 1 patient exhibited pectus carinatum after a separate spinal fusion surgery for scoliosis. One patient died of unrelated cardiac complications before bar removal. Simultaneous repair of PE and open heart surgery is safe and effective. We recommend that the decision to perform a single-stage versus a multistage procedure should be reserved until after the cardiac procedure has been completed. In such cases, the Nuss technique allows for correction of the pectus deformity with good long-term cosmetic and functional results. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Congenital heat disease

International Nuclear Information System (INIS)

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

1990-01-01

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

THREE PORTS LAPAROSCOPIC REPAIR OF ADULT MORGAGNI HERNIA AND RARE SIMULTANEOUS PRESENTATION OF PARA-ESOPHAGEAL HERNIA WITH MORGAGNI HERNIA

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Salman Assad

2018-05-01

Full Text Available We report two case reports of Morgagni hernia repair. Our first case was on 65 year old white male who presented with abdominal pain in right upper quadrant and right side of chest for last 3 days. He was having 3 episodes of dark appearing vomiting associated with pain. He also had two episodes of hematemesis. Patient had gastric outlet obstruction with severe distension of stomach because of incarcerated small bowel and colon in the right sided anterior diaphragmatic Morgagni hernia. Laparoscopic repair of incarcerated Morgagni hernia under general anesthesia was planned. We report our second case on rare simultaneous presentation of Morgagni Hernia with type 3 Para esophageal hernia. 60 years old female patient presented in clinic with a follow up of chest discomfort which was progressively increasing with shortness of breath and a chronic gastric reflux. Her vitals were within normal limits and had body mass index (BMI= 29.52kg/m2 (overweight category. Previous past medical history included multiple episodes of gastric regurgitation and cardiovascular intervention for coronary stenting. CT scan showed type 3 paraesophageal hernia (gastro esophageal junction with fundus of stomach displaced above diaphragm. The patient had more than 30% of her stomach incarcerated in the chest as a paraesophageal hernia. The gastro esophageal junction was intra-abdominal after lysis of adhesion. Mesh was placed after posterior crural repair, followed by Nissen fundoplication over a 54 French bougie patient also had an incidental finding of a reducible Morgagni hernia through an anterior defect, followed by a repair without mesh. Esophago-gastro-duodenoscopy showed there was no evidence of any air leak with good valve creation on retroflexion through a fundoplication.

Aid decision algorithms to estimate the risk in congenital heart surgery.

Science.gov (United States)

Ruiz-Fernández, Daniel; Monsalve Torra, Ana; Soriano-Payá, Antonio; Marín-Alonso, Oscar; Triana Palencia, Eddy

2016-04-01

In this paper, we have tested the suitability of using different artificial intelligence-based algorithms for decision support when classifying the risk of congenital heart surgery. In this sense, classification of those surgical risks provides enormous benefits as the a priori estimation of surgical outcomes depending on either the type of disease or the type of repair, and other elements that influence the final result. This preventive estimation may help to avoid future complications, or even death. We have evaluated four machine learning algorithms to achieve our objective: multilayer perceptron, self-organizing map, radial basis function networks and decision trees. The architectures implemented have the aim of classifying among three types of surgical risk: low complexity, medium complexity and high complexity. Accuracy outcomes achieved range between 80% and 99%, being the multilayer perceptron method the one that offered a higher hit ratio. According to the results, it is feasible to develop a clinical decision support system using the evaluated algorithms. Such system would help cardiology specialists, paediatricians and surgeons to forecast the level of risk related to a congenital heart disease surgery. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Inhaled Beta Agonist Bronchodilator Does Not Affect Trans-diaphragmatic Pressure Gradient but Decreases Lower Esophageal Sphincter Retention Pressure in Patients with Chronic Obstructive Pulmonary Disease (COPD) and Gastroesophageal Reflux Disease (GERD).

Science.gov (United States)

Del Grande, Leonardo M; Herbella, Fernando A M; Bigatao, Amilcar M; Jardim, Jose R; Patti, Marco G

2016-10-01

Chronic obstructive pulmonary disease (COPD) patients have a high incidence of gastroesophageal reflux disease (GERD) whose pathophysiology seems to be linked to an increased trans-diaphragmatic pressure gradient and not to a defective esophagogastric barrier. Inhaled beta agonist bronchodilators are a common therapy used by patients with COPD. This drug knowingly not only leads to a decrease in the lower esophageal sphincter (LES) resting pressure, favoring GERD, but also may improve ventilatory parameters, therefore preventing GERD. This study aims to evaluate the effect of inhaled beta agonist bronchodilators on the trans-diaphragmatic pressure gradient and the esophagogastric barrier. We studied 21 patients (mean age 67 years, 57 % males) with COPD and GERD. All patients underwent high-resolution manometry and esophageal pH monitoring. Abdominal and thoracic pressure, trans-diaphragmatic pressure gradient (abdominal-thoracic pressure), and the LES retention pressure (LES basal pressure-transdiaphragmatic gradient) were measured before and 5 min after inhaling beta agonist bronchodilators. The administration of inhaled beta agonist bronchodilators leads to the following: (a) a simultaneous increase in abdominal and thoracic pressure not affecting the trans-diaphragmatic pressure gradient and (b) a decrease in the LES resting pressure with a reduction of the LES retention pressure. In conclusion, inhaled beta agonist bronchodilators not only increase the thoracic pressure but also lead to an increased abdominal pressure favoring GERD by affecting the esophagogastric barrier.

Endovascular Treatment of Late Thoracic Aortic Aneurysms after Surgical Repair of Congenital Aortic Coarctation in Childhood

Science.gov (United States)

Juszkat, Robert; Perek, Bartlomiej; Zabicki, Bartosz; Trojnarska, Olga; Jemielity, Marek; Staniszewski, Ryszard; Smoczyk, Wiesław; Pukacki, Fryderyk

2013-01-01

Background In some patients, local surgery-related complications are diagnosed many years after surgery for aortic coarctation. The purposes of this study were: (1) to systematically evaluate asymptomatic adults after Dacron patch repair in childhood, (2) to estimate the formation rate of secondary thoracic aortic aneurysms (TAAs) and (3) to assess outcomes after intravascular treatment for TAAs. Methods This study involved 37 asymptomatic patients (26 female and 11 male) who underwent surgical repair of aortic coarctation in the childhood. After they had reached adolescence, patients with secondary TAAs were referred to endovascular repair. Results Follow-up studies revealed TAA in seven cases (19%) (including six with the gothic type of the aortic arch) and mild recoarctation in other six (16%). Six of the TAA patients were treated with stentgrafts, but one refused to undergo an endovascular procedure. In three cases, stengrafts covered the left subclavian artery (LSA), in another the graft was implanted distally to the LSA. In two individuals, elective hybrid procedures were performed with surgical bypass to the supraaortic arteries followed by stengraft implantation. All subjects survived the secondary procedures. One patient developed type Ia endoleak after stentgraft implantation that was eventually treated with a debranching procedure. Conclusions The long-term course of clinically asymptomatic patients after coarctation patch repair is not uncommonly complicated by formation of TAAs (particularly in individuals with the gothic pattern of the aortic arch) that can be treated effectively with stentgrafts. However, in some patients hybrid procedures may be necessary. PMID:24386233

Repair process and a repaired component

Energy Technology Data Exchange (ETDEWEB)

Roberts, III, Herbert Chidsey; Simpson, Stanley F.

2018-02-20

Matrix composite component repair processes are disclosed. The matrix composite repair process includes applying a repair material to a matrix composite component, securing the repair material to the matrix composite component with an external securing mechanism and curing the repair material to bond the repair material to the matrix composite component during the securing by the external securing mechanism. The matrix composite component is selected from the group consisting of a ceramic matrix composite, a polymer matrix composite, and a metal matrix composite. In another embodiment, the repair process includes applying a partially-cured repair material to a matrix composite component, and curing the repair material to bond the repair material to the matrix composite component, an external securing mechanism securing the repair material throughout a curing period, In another embodiment, the external securing mechanism is consumed or decomposed during the repair process.

Development of the Integrated Info Tech System

Science.gov (United States)

2007-01-01

all ECMO patients except for the following cases: 1. Patients who have Congenital Diaphragmatic Hernias and will require a prolonged ECMO run. 2...of the uterus. Other methods, such as the use of condoms, a diaphragm or cervical cap, Platelet Rich Plasma Gel Investigational Plan PRP Gel 17 1/06

Congenital orbital teratoma.

Science.gov (United States)

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

2013-12-01

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

Congenital orbital teratoma

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Shereen Aiyub

2013-01-01

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

Gerbode defect and multivalvular dysfunction: Complex complications in adult congenital heart disease.

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Ruivo, Catarina; Guardado, Joana; Montenegro Sá, Fernando; Saraiva, Fátima; Antunes, Alexandre; Correia, Joana; Morais, João

2017-07-01

We report a clinical case of a 40-year-old male with surgically corrected congenital heart disease (CHD) 10 years earlier: closure of ostium primum, mitral annuloplasty, and aortic valve and root surgery. The patient was admitted with acute heart failure. Transesophageal echocardiography (TEE) revealed a dysmorphic and severely incompetent aortic valve, a partial tear of the mitral valve cleft repair and annuloplasty ring dehiscence. A true left ventricular-to-right atrial shunt confirmed a direct Gerbode defect. The authors aim to discuss the diagnostic challenge of adult CHD, namely the key role of TEE on septal defects and valve regurgitations description. © 2017, Wiley Periodicals, Inc.

Burden and impact of congenital syndromes and comorbidities among adults with congenital heart disease.

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Bracher, Isabelle; Padrutt, Maria; Bonassin, Francesca; Santos Lopes, Bruno; Gruner, Christiane; Stämpfli, Simon F; Oxenius, Angela; De Pasquale, Gabriella; Seeliger, Theresa; Lüscher, Thomas F; Attenhofer Jost, Christine; Greutmann, Matthias

2017-08-01

Our aim was to assess the overall burden of congenital syndromes and non-cardiac comorbidities among adults with congenital heart disease and to assess their impact on circumstances of living and outcomes. Within a cohort of 1725 adults with congenital heart defects (65% defects of moderate or great complexity) followed at a single tertiary care center, congenital syndromes and comorbidities were identified by chart review. Their association with arrhythmias, circumstances of living and survival was analyzed. Within the study cohort, 232 patients (13%) had a genetic syndrome, 51% at least one comorbidity and 23% ≥2 comorbidities. Most prevalent comorbidities were systemic arterial hypertension (11%), thyroid dysfunction (9%), psychiatric disorders (9%), neurologic disorders (7%), chronic lung disease (7%), and previous stroke (6%). In contrast to higher congenital heart defect complexity, the presence of comorbidities had no impact on living circumstances but patients with comorbidities were less likely to work full-time. Atrial arrhythmias were more common among patients with moderate/great disease complexity and those with comorbidities but were less common among patients with congenital syndromes (pCongenital syndromes and comorbidities are highly prevalent in adults with congenital heart disease followed at specialist centers and add to the overall complexity of care. The presence of these additional factors has an impact on living circumstances, is associated with arrhythmias and needs to be further explored as prognostic markers. Copyright © 2017 Elsevier B.V. All rights reserved.

Congenital cytomegalovirus infection

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Katarina Rednak-Paradiž

2006-11-01

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

Reparação do diafragma de cães com segmento muscular homólogo ortotópico conservado em solução supersaturada de açúcar Diaphragm repair in dogs with muscular homologous ortothopic segment preserved in supersaturated sugar solution

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A. Mazzanti

2001-02-01

the 60-day group a total substitution of the diaphragmatic muscular portion by granulation tissue rich in collagen fibers had occurred, allowing the reconstruction of the diaphragm. The homologous diaphragmatic muscle segment preserved in 300%, satured sugar solution, at room temperature, was substituted by fibrous connective tissue. The implant did not present clinical and histological signs of rejection and therefore it can be used for the repair of diaphragmatic defects.

Prevalence of congenital amusia.

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Peretz, Isabelle; Vuvan, Dominique T

2017-05-01

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

Congenital Leukemia in Down's syndrome

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

2006-01-01

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

Prevalence of congenital anomalies in newborns with congenital heart disease diagnosis

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Egbe, Alexander; Lee, Simon; Ho, Deborah; Uppu, Santosh; Srivastava, Shubhika

2014-01-01

There is a known association between congenital heart disease (CHD) and other congenital anomalies (CA). These associations have been altered by changes in prenatal factors in recent time. We reviewed the largest database of inpatient hospitalization information and analyzed the current association between common CHD diagnoses and other congenital anomalies. Case-control study design. We reviewed the Nationwide Inpatient Sample (NIS) database from 1998 to 2008 and identified all live births with CHD diagnosis (case) and live births without CHD diagnosis (control). We compared prevalence of associated congenital anomalies between the case and control groups. Our cohort consisted of 97,154 and 12,078,482 subjects in the case and control groups, respectively. In the CHD population, prevalence of non-syndromic congenital anomaly (NSCA), genetic syndrome (GS), and overall extra-cardiac congenital anomaly (CA) were 11.4, 2.2, and 13.6%, respectively. In the control group, prevalence of NSCA, GS, and CA were 6.7, 0.3, and 7.0%, respectively. NSCA (odds ratio (OR): 1.88, confidence interval (CI): 1.73-1.94), GS (OR 2.52, CI 2.44-2.61), and overall CA (OR: 2.01, CI: 1.97-2.14) were strongly associated with CHD. Prevalence of GS and multiple organ-system CA decreased significantly over the study period. This is the largest and most comprehensive population-based study evaluating association between CHD and extra-cardiac malformation (ECM) in newborns. There was significant decrease in prevalence of GS and multiple CA over the study period

Congenital heart defects and extracardiac malformations.

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Rosa, Rosana Cardoso M; Rosa, Rafael Fabiano M; Zen, Paulo Ricardo G; Paskulin, Giorgio Adriano

2013-06-01

To review the association between congenital heart defects and extracardiac malformations. Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specifically explored the association between congenital heart defects and extracardiac malformations were included. Congenital heart diseases are responsible for about 40% of birth defects, being one of the most common and severe malformations. Extracardiac malformations are observed in 7 to 50% of the patients with congenital heart disease, bringing a greater risk of comorbidity and mortality and increasing the risks related to heart surgery. Different studies have attempted to assess the presence of extracardiac abnormalities in patients with congenital heart disease. Among the changes described, those of the urinary tract are more often reported. However, no study has evaluated all patients in the same way. Extracardiac abnormalities are frequent among patients with congenital heart disease, and patients with these alterations may present an increased risk of morbimortality. Therefore, some authors have been discussing the importance and cost-effectiveness of screening these children for other malformations by complementary exams.

Congenital Heart Defects and CCHD

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

Congenital amusias.

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Tillmann, B; Albouy, P; Caclin, A

2015-01-01

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

Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy

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Courtney A. Bishop

2018-01-01

Full Text Available Subjects with Duchenne Muscular Dystrophy (DMD suffer from progressive muscle damage leading to diaphragmatic weakness that ultimately requires ventilation. Emerging treatments have generated interest in better characterizing the natural history of respiratory impairment in DMD and responses to therapy. Dynamic (cine Magnetic Resonance Imaging (MRI may provide a more sensitive measure of diaphragm function in DMD than the commonly used spirometry. This study presents an analysis pipeline for measuring parameters of diaphragmatic motion from dynamic MRI and its application to investigate MRI measures of respiratory function in both healthy controls and non-ambulant DMD boys. We scanned 13 non-ambulant DMD boys and 10 age-matched healthy male volunteers at baseline, with a subset (n = 10, 10, 8 of the DMD subjects also assessed 3, 6, and 12 months later. Spirometry-derived metrics including forced vital capacity were recorded. The MRI-derived measures included the lung cross-sectional area (CSA, the anterior, central, and posterior lung lengths in the sagittal imaging plane, and the diaphragm length over the time-course of the dynamic MRI. Regression analyses demonstrated strong linear correlations between lung CSA and the length measures over the respiratory cycle, with a reduction of these correlations in DMD, and diaphragmatic motions that contribute less efficiently to changing lung capacity in DMD. MRI measures of pulmonary function were reduced in DMD, controlling for height differences between the groups: at maximal inhalation, the maximum CSA and the total distance of motion of the diaphragm were 45% and 37% smaller. MRI measures of pulmonary function were correlated with spirometry data and showed relationships with disease progression surrogates of age and months non-ambulatory, suggesting that they provide clinically meaningful information. Changes in the MRI measures over 12 months were consistent with weakening of

Virtual surgical modification for planning tetralogy of Fallot repair

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Plasencia, Jonathan; Babiker, Haithem; Richardson, Randy; Rhee, Edward; Willis, Brigham; Nigro, John; Cleveland, David; Frakes, David H.

2010-01-01

Goals for treating congenital heart defects are becoming increasingly focused on the long-term, targeting solutions that last into adulthood. Although this shift has motivated the modification of many current surgical procedures, there remains a great deal of room for improvement. We present a new methodological component for tetralogy of Fallot (TOF) repair that aims to improve long-term outcomes. The current gold standard for TOF repair involves the use of echocardiography (ECHO) for measuring the pulmonary valve (PV) diameter. This is then used, along with other factors, to formulate a Z-score that drives surgical preparation. Unfortunately this process can be inaccurate and requires a mid-operative confirmation that the pressure gradient across the PV is not excessive. Ideally, surgeons prefer not to manipulate the PV as this can lead to valve insufficiency. However, an excessive pressure gradient across the valve necessitates surgical action. We propose the use of computational fluid dynamics (CFD) to improve preparation for TOF repair. In our study, pre-operative CT data were segmented and reconstructed, and a virtual surgical operation was then performed to simulate post-operative conditions. The modified anatomy was used to drive CFD simulation. The pressure gradient across the pulmonary valve was calculated to be 9.24mmHg, which is within the normal range. This finding indicates that CFD may be a viable tool for predicting post-operative pressure gradients for TOF repair. Our proposed methodology would remove the need for mid-operative measurements that can be both unreliable and detrimental to the patient.

Heart Transplantation in Congenital Heart Disease: In Whom to Consider and When?

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Attenhofer Jost, Christine H.; Schmidt, Dörthe; Huebler, Michael; Balmer, Christian; Noll, Georg; Caduff, Rosmarie; Greutmann, Matthias

2013-01-01

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger's syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future. PMID:23577237

The impact of obesity on early postoperative outcomes in adults with congenital heart disease.

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Zaidi, Ali N; Bauer, John A; Michalsky, Marc P; Olshove, Vincent; Boettner, Bethany; Phillips, Alistair; Cook, Stephen C

2011-01-01

As the prevalence of obesity continues to increase, it now includes the growing number of patients with congenital heart disease (CHD). This particular obese patient population may pose additional intraoperative as well as postoperative challenges that may contribute to poor outcomes. Our aims were to determine the influence of obesity on morbidity and mortality in adults with CHD undergoing surgical repair at a free standing children's hospital. A retrospective analysis of adult (≥18 years) CHD surgery cases from 2002 to 2008 was performed. Congenital heart lesions were defined as mild, moderate, or complex. Patients were categorized by body mass index (BMI): underweight (BMI obese (BMI ≥ 30 kg/m(2)). Demographics, incidence of mortality, or specific morbidities were statistically compared using Fisher's exact test and analyses of variance (anovas). In this population (n = 165), overweight (29%) and obese (22%) patients were prevalent. Hypertension (HTN) and pre-HTN were more prevalent in obese and overweight patients. Postoperative renal dysfunction was observed in obese patients with complex CHD (P = .04). Mortality was not different among groups. Obesity is becoming increasingly common among adults with CHD. Despite marginal evidence of postoperative renal complications in obese patients with CHD of severe complexity, the overall presence of obesity did not influence mortality or short term postoperative morbidities. © 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.

US patterns of the diaphragmatic crura. Normal anatomy and its variants; Aspetti ecografici dei pilastri diaframmatici. Anatomia normale e sue varianti

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Crespi, G.; Martinoli, C.; Cicio, G.R. [Genua Univ., Genua (Italy). Cattedra R di Radiologia DICMI; Zappasodi, F. [Sobborgo Valzania, Cesena, FO (Italy); Valle, M. [Istituto Gaslini, Genua (Italy). Servizio di Radiologia

2000-06-01

Purpose of this work is to report the various US patterns of the diaphragmatic crura and the changes occurring during the different phases of respirations. The diaphragm has two US patterns: the central membranous part appears highly reflective while the posterior, upper and lateral muscular portions are hypoechoic and thick. The crura can sometimes appear quite bulky, which appearance is easy to misinterpret. It was carried out a three-stage work: first it was reviewed the US examinations of 23 subjects with a nodular appearance of the posteromedial bundles and studied the changes in thickness during respiration. Second it was studied the diaphragmatic crura in 30 subjects aged 18-71 years, 15 men and 15 women. It was used a commercially available unit with sector and convex 3.5 MHz probes at baseline and during breath hold and acquired multiple parasaggittal and transverse scans. The crura thickness was measured in all patients. Last, it was studied the diaphragmatic regions of 10 patients with right pleural effusion and of 8 patients with associated ascites and pleural effusion using 2.0-5.0 MHz convex phased-array transducers. It was found that focal thickening of the crura in 11 of 23 patients with US findings of diaphragmatic nodules, but only in deep inspiration. The thickening was 1.5-2.2 cm long and maximum thickness was 10 mm. In the other 12 subjects it was found 9 small lobules in the right and 3 in the left crus. In the anatomic study, it was observed a 3-band appearance of the diaphragmatic crura, probably referable to muscle bundles, in 30 subjects on sagittal images, in 12 on coronal images and in 28 on anterior transverse images. The diaphragmatic crura were identified in 26 subjects only. The left posterior crus was identified in 29 subjects on left coronal images and in 15 on anterior transverse images; it was demonstrated on anterior sagittal images in close proximity to the aorta in only 4 subjects. Right crus thickness, measured on sagittal

Congenital malaria in China.

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Zhi-Yong Tao

2014-03-01

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

[Identification of risk factors for congenital malformations].

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Canals C, Andrea; Cavada C, Gabriel; Nazer H, Julio

2014-11-01

The relative importance of congenital malformations as a cause of death in the first year of life is increasing along with the control of preventable causes of perinatal mortality. To identify risk factors for congenital malformations. Retrospective case-control study of births registered in the database of The Latin American Collaborative Study of Congenital Malformations (ECLAMC), in the period 2001-2010. Birth weight and gestational age were significantly lower in cases than controls, behaving as risk factors and associated with a greater severity of congenital malformations. The risk and severity of congenital malformations increased along with mother's age. Fetal growth retardation, a history of congenital malformations in the family, physical factors and acute illnesses of the mother in the first trimester of pregnancy were also significant risk factors for congenital malformations and their severity. The educational level of the mother was a protective factor for congenital malformations and their severity. Variables previously identified as risk factors for congenital malformations, were significantly related with the occurrence of congenital malformations and their severity.

Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features

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Yoo, Hye Jin; Kim, Woo Sun; Cheon, Jung-Eun; Shin, Su-Mi; Kim, In-One; Yeon, Kyung Mo [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, Seoul (Korea); Yoo, So-Young [Seoul National University College of Medicine and the Institute of Radiation Medicine, Department of Radiology, Seoul (Korea); Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea); Park, Kwi-Won; Jung, Sung-Eun [Seoul National University Children' s Hospital, Department of Pediatric Surgery, Seoul (Korea)

2010-08-15

Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF. To evaluate the clinical and radiologic features of congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula. We retrospectively reviewed postoperative esophagograms and medical records of 187 children (107 boys, 80 girls) who had primary repair of EA/TEF from 1992 to 2009 at our institution. We evaluated the incidence of CES, clinical findings, radiologic features and management of CES in these children. CES was diagnosed in 22 of 187 EA/TEF children (12%); one child had double CES lesions, for a total of 23 lesions. Ten of those 22 children (45%) had presented with significant symptoms of esophageal obstruction. The diagnosis of CES was delayed in 10 children (45%) until 1-10 years of age. On esophagogram, CES (n = 23) was located in the distal esophagus (n = 20, 87%) or mid-esophagus (n = 3, 13%). The degree of stenosis was severe (n = 6, 26%), moderate (n = 10, 43%), or mild (n = 7, 30%). Eight children, including two with unsuccessful esophageal balloon dilatation of CES, were treated surgically. Histologic examination revealed tracheobronchial remnant (n = 7) or fibromuscular hyperplasia (n = 1). One child with surgically treated CES developed achalasia at the age of 3 years 9 months. Esophagography after EA/TEF repair should be performed with a high index of suspicion for the presence of distal CES, because the diagnosis and adequate management of CES can often be delayed. (orig.)

Right sided traumatic diaphragmatic hernia repair with intrathoracic herniation of liver, stomach and transverse colon

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Surendra Patel

2015-07-01

Full Text Available Traumatic rupture of the right dome of the diaphragm is an uncommon clinical entity. The diagnosis is difficult due to lack of specificity in clinical signs and chest film findings. Clinical incidence of rupture of the right dome of the diaphragm due to blunt trauma is much less common (10% compared to the left (90% and usually associated with more grievous injuries with very high pre hospital mortality thus accounting for rare clinical diagnosis. We report a case of 26 year old male who had the blunt trauma chest referred to us after 7 days of injury with complaints of shortness of breath and vomiting. On investigations the patient was diagnosed as a case of the ruptured right dome of the diaphragm with intrathoracic herniation of the stomach. The patient also had fracture pelvis. Surgical exploration was done through right 6th intercostal space which revealed intrathoracic herniation of the stomach, liver and transverse colon, which were healthy. Contents reduced into the abdomen and diaphragm was repaired. Post operative chest X-ray suggested complete expansion of the right lung with no residual herniation of abdominal contents. Post operative recovery was uneventful with the patient discharged on 14th post operative day.

Congenital platelet function defects

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

Renal artery entrapment by the diaphragmatic crus

International Nuclear Information System (INIS)

Thony, F.; Baguet, J.-P.; Rodiere, M.; Sessa, C.; Janbon, B; Ferretti, G.

2005-01-01

The aim of this study is to describe renal artery entrapment (RAE) by the diaphragmatic crus and to elucidate the diagnostic and therapeutic approach to this entity. From 1995 to 2002, 15 patients (mean age 65) were found to have a RAE. They were investigated by CT scan (n=14) and/or MRA (n=2) for hypertension (n=7), chronic renal insufficiency (n=4) or aneurysms (n=4). The right (n=11) or the left (n=4) renal artery (RA) was involved. The compression was ostial (n=8) or truncal (n=7), and was ≥50% in eight cases. The course of the RA along the aorta on angiographic views (n=8) or a concentric ostial stenosis in a patient free of atheromatous lesions (n=7) were two findings suggestive of an RAE. Seven RAEs were indicated for treatment but only three were treated, by mean of stenting. Among the latter, two stents were patent at 6-month follow-up and one evolved to restenosis because of a stent fracture. RAEs may be suspected on angiographic views and proved by cross-sectional imaging because of specific imaging features. It is of importance to detect this etiology of RA stenosis because angioplasty with stenting is probably not always advisable. (orig.)

General Concepts in Adult Congenital Heart Disease.

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Mutluer, Ferit Onur; Çeliker, Alpay

2018-01-20

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

General Concepts in Adult Congenital Heart Disease

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Ferit Onur Mutluer

2018-02-01

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

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

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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.

The dysmorphic lung: imaging findings

International Nuclear Information System (INIS)

Mata, J.M.; Caceres, J.

1996-01-01

Congenital lung malformations are not infrequent and can be discovered in adults. It is, therefore, necessary to know their radiological manifestations in order to avoid diagnostic errors. We classify the congenital lung malformations in two main groups: dysmorphic lung and focal pulmonary malformations. We review the radiological spectrum of dysmorphic lung, based on a classification that emphasises the pulmonary abnormality, adding variants when diaphragmatic or venous abnormalities are present. In our opinion this approach allows for a rational use of advanced imaging techniques (CT, MRI). (orig.). With 13 figs

The dysmorphic lung: imaging findings

Energy Technology Data Exchange (ETDEWEB)

Mata, J.M. [SDI-UDIAT, Consorci Hospitalari del Parc Tauli, Parc Tauli s/n, E-08208 Barcelona (Spain)]|[Universitat Autonoma de Barcelona, Barcelona (Spain); Caceres, J. [Universitat Autonoma de Barcelona, Barcelona (Spain)]|[Hospital de la Santa Creu i Sant Pau, Barcelona (Spain)

1996-08-01

Congenital lung malformations are not infrequent and can be discovered in adults. It is, therefore, necessary to know their radiological manifestations in order to avoid diagnostic errors. We classify the congenital lung malformations in two main groups: dysmorphic lung and focal pulmonary malformations. We review the radiological spectrum of dysmorphic lung, based on a classification that emphasises the pulmonary abnormality, adding variants when diaphragmatic or venous abnormalities are present. In our opinion this approach allows for a rational use of advanced imaging techniques (CT, MRI). (orig.). With 13 figs.

Congenital Heart Information Network

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

Early detection of congenital syphilis

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Nagalakshmi Chowdhary

2014-01-01

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

Genetics Home Reference: critical congenital heart disease

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

[Tension gastrothorax as a cause of death by obstructive shock - case report].

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García-Regalado, Juan F; Navarro-Rojas, Mariana M

2014-07-01

Tension gastrothorax is caused by the herniation of the stomach into the thorax due to a congenital defect of the diaphragm; the Bochdaleck diaphragmatic hernia (HDB) is the most frequent type. Tension gastrothorax should be considered as a differential diagnosis in patients with obstructive shock and tension pneumothorax. A previously healthy 10 month-old male infant, who presented increased respiratory distress, increased volume of the left hemithorax, absence of breath sounds, ipsilateral hyper-resonance, 76% saturation, cold skin and capillary filling > 5 seconds, followed by a cardio-respiratory arrest. Due to clinical suspicion of pneumothorax, needle decompression was performed reversing cardiac arrest, but with persistent hemodynamic and respiratory instability; chest radiograph suggested diaphragmatic hernia. He underwent surgery confirming the presence of a diaphragmatic hernia of 5 cm. The evolution of this case shows the difficulty differentiating a tension gastrothorax from tension pneumothorax in patients admitted to the emergency room who are in serious condition; therefore, a high index of suspicion is needed for its identification.

Congenital Anorectal Malformation Severity Does Not Predict Severity of Congenital Heart Defects

NARCIS (Netherlands)

Jonker, Jara E.; Liem, Eryn T.; Elzenga, Nynke J.; Molenbuur, Bouwe; Trzpis, Monika; Broens, Paul M. A.

2016-01-01

Objective To determine the prevalence of congenital heart defects (CHDs) in patients with mild or severe congenital anorectal malformations (CARMs), and whether all patients with CARM need pediatric cardiology screening. Study design We included 129 patients with CARM born between 2004 and 2013, and

Adaptive growth factor delivery from a polyelectrolyte coating promotes synergistic bone tissue repair and reconstruction

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Shah, Nisarg J.; Hyder, Md. Nasim; Quadir, Mohiuddin A.; Dorval Courchesne, Noémie-Manuelle; Seeherman, Howard J.; Nevins, Myron; Spector, Myron; Hammond, Paula T.

2014-01-01

Traumatic wounds and congenital defects that require large-scale bone tissue repair have few successful clinical therapies, particularly for craniomaxillofacial defects. Although bioactive materials have demonstrated alternative approaches to tissue repair, an optimized materials system for reproducible, safe, and targeted repair remains elusive. We hypothesized that controlled, rapid bone formation in large, critical-size defects could be induced by simultaneously delivering multiple biological growth factors to the site of the wound. Here, we report an approach for bone repair using a polyelectrolye multilayer coating carrying as little as 200 ng of bone morphogenetic protein-2 and platelet-derived growth factor-BB that were eluted over readily adapted time scales to induce rapid bone repair. Based on electrostatic interactions between the polymer multilayers and growth factors alone, we sustained mitogenic and osteogenic signals with these growth factors in an easily tunable and controlled manner to direct endogenous cell function. To prove the role of this adaptive release system, we applied the polyelectrolyte coating on a well-studied biodegradable poly(lactic-co-glycolic acid) support membrane. The released growth factors directed cellular processes to induce bone repair in a critical-size rat calvaria model. The released growth factors promoted local bone formation that bridged a critical-size defect in the calvaria as early as 2 wk after implantation. Mature, mechanically competent bone regenerated the native calvaria form. Such an approach could be clinically useful and has significant benefits as a synthetic, off-the-shelf, cell-free option for bone tissue repair and restoration. PMID:25136093

The prevalence and risk factors for cholelithiasis and asymptomatic gallstones in adults with congenital heart disease.

Science.gov (United States)

Shiina, Yumi; Toyoda, Tomohiko; Kawasoe, Yasutaka; Tateno, Shigeru; Shirai, Takeaki; Matsuo, Kozo; Mizuno, Yoshiko; Ai, Toshiji; Niwa, Koichiro

2011-10-20

Cyanosis is considered to be a risk factor for cholelithiasis which is an important complication of cyanotic congenital heart disease (CCHD) in adults. In this study, the prevalence of cholelithiasis and asymptomatic calcium bilirubinate gallstones was evaluated in adults with congenital heart disease (CHD). Furthermore, risk factors for this potentially high risk complication were assessed. Subjects were derived from 114 consecutive congenital patients who visited our center from May 2008 to January 2009. For analyses of risk factors, we divided them into 4 groups: group A, 15 CCHD patients without reparative surgery (7 men, 31.8 ± 7.0 years old); group B, 41 CCHD patients rendered acyanotic by reparative surgery (21 men, 32.5 ± 11.8 years old); group C, 23 unoperated acyanotic CHD patients (11 men, 42.4 ± 16.4 years old); and group D, 35 patients who were acyanotic before and after operation (18 men, 36.3 ± 14.8 years old). Gallstones were identified by abdominal ultrasound and risk factors were analyzed by a multivariate logistic regression model. Cholecystectomy was performed in 5/114 (4.3%), asymptomatic gallstones were seen in 16/114 (14%), and symptomatic gallstones except for patients after cholecystectomy were seen in 7/114 (6.1%). In group A, 4 (27%) with gallstones underwent cholecystectomy (pnobody in group C, and 3 patients (8.6%) in group D. By a multivariate logistic regression model, CCHD by nature regardless of repair, prolonged cyanosis periods, higher frequency of cardiopulmonary bypass (CPB), and lower platelet counts were significant factors predicting gallstones (odds ratio 4.48, 1.08, 3.96, and 0.87, 95% CI, 1.14-17.5, 1.00-1.18, 1.65-9.54, and 0.75-0.99, respectively). The prevalence of cholelithiasis and asymptomatic gallstones is significantly high in CCHD patients regardless of cardiac repairs. CCHD by nature, prolonged cyanosis durations, high frequency of CPB and low platelet counts have influences on gallstone formation in adults

Prenatal Diagnosis of Bilateral Pulmonary Agenesis: a Case Report

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Lee, Kyung A; Cho, Jeong Yeon; Lee, Seung Mi; Jun, Jong Kwan; Kang, Ji Eun; Seo, Jeong Wook [Seoul National University College of Medicine, Seoul (Korea, Republic of)

2010-02-15

We report a case of bilateral pulmonary agenesis (BPA), which was suspected during a prenatal US examination and diagnosed by fetal magnetic resonance imaging (MRI). BPA is an extremely rare congenital anomaly and, although many fetal structural defects can be detected with a high degree of confidence after introducing high-resolution US, the prenatal diagnosis of BPA remains problematic. Other thoracic abnormalities, such as a congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, and pulmonary sequestration, should be excluded from the list of possible diagnoses before coming to the conclusion of BPA, because BPA is absolutely incompatible with extrauterine life, and an accurate internal diagnosis can prevent a futile intervention from being performed.

Minimally invasive repair of Morgagni hernia – A multicenter case series

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R. Lamas-Pinheiro

2016-09-01

Full Text Available Children may benefit from minimally invasive surgery (MIS in the correction of Morgagni hernia (MH. The present study aims to evaluate the outcome of MIS through a multicenter study.National institutions that use MIS in the treatment of MH were included. Demographic, clinical and operative data were analyzed.Thirteen patients with MH (6 males were operated using similar MIS technique (percutaneous stitches at a mean age of 22.2 ± 18.3 months. Six patients had chromosomopathies (46%, five with Down syndrome (39%. Respiratory complaints were the most common presentation (54%. Surgery lasted 95 ± 23 min. In none of the patients was the hernia sac removed; prosthesis was never used. In the immediate post-operative period, 4 patients (36% were admitted to intensive care unit (all with Down syndrome; all patients started enteral feeds within the first 24 h. With a mean follow-up of 56 ± 16.6 months, there were two recurrences (18% at the same institution, one of which was repaired with an absorbable suture; both with Down syndrome.The application of MIS in the MH repair is effective even in the presence of comorbidities such as Down syndrome; the latter influences the immediate postoperative recovery and possibly the recurrence rate. Removal of hernia sac does not seem necessary. Non-absorbable sutures may be more appropriate. Keywords: Laparoscopy, Minimally invasive surgery, Percutaneous, Morgagni hernia, Diaphragmatic hernia

Fetal chromosome abnormalities and congenital malformations: an ...

African Journals Online (AJOL)

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

Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia

NARCIS (Netherlands)

Bellini, Carlo; Mazzella, Massimo; Arioni, Cesare; Campisi, Corradino; Taddei, Gioconda; Tomà, Paolo; Boccardo, Francesco; Hennekam, Raoul C.; Serra, Giovanni

2003-01-01

We report a female infant with congenital lymphedema, facial anomalies, intestinal lymphangiectasia consistent with a diagnosis of Hennekam syndrome. At birth the patient presented with severe respiratory distress due to nonimmune hydrops fetalis, a congenital chylothorax (CC), and pulmonary

Laparoscopic repair of penetrating injury of the diaphragm: an experience from a district hospital

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Ali Yahya

2008-01-01

Full Text Available In this study we review our experience in using laparoscopy as a diagnostic and therapeutic tool in dealing with penetrating diaphragmatic injuries due to stab wounds and look at the feasibility of using this procedure in other similar institutions.Thirty patients, all of whom were males 20-30 years of age, presented to the surgical emergency unit of our hospital with upper abdominal and lower chest wall stab wounds between 01-05-1998 and 30-11-2006. Diagnosis of the diaphragm injury was either obvious with omentum herniating through the chest wall, or occult with confirmation of the injury at laparoscopy.All patients underwent diagnostic laparoscopy, which resulted in identification and efficient treatment of eight patients with diaphragmatic injury, and thereby laparotomy was avoided. The procedure converted to open surgery in one patient because of a small left-sided colonic tear. Laparoscopy is an efficient tool for the diagnosis and management of diaphragmatic injuries. It should be used routinely instead of exploratory laparotomy in haemodynamically stable patients with penetrating lower chest injuries.

Bilateral Diaphragmatic Paralysis in a Patient With Critical Illness Polyneuropathy

Science.gov (United States)

Chen, Hsuan-Yu; Chen, Hung-Chen; Lin, Meng-Chih; Liaw, Mei-Yun

2015-01-01

Abstract Bilateral diaphragmatic paralysis (BDP) manifests as respiratory muscle weakness, and its association with critical illness polyneuropathy (CIP) was rarely reported. Here, we present a patient with BDP related to CIP, who successfully avoided tracheostomy after diagnosis and management. A 71-year-old male presented with acute respiratory failure after sepsis adequately treated. Repeated intubation occurred because of carbon dioxide retention after each extubation. After eliminating possible factors, septic shock-induced respiratory muscle weakness was suspected. Physical examination, a nerve conduction study, and chest ultrasound confirmed our impression. Pulmonary rehabilitation and reconditioning exercises were arranged, and the patient was discharged with a diagnosis of BDP. The diagnosis of BDP is usually delayed, and there are only sporadic reports on its association with polyneuropathy, especially in patients with preserved limb muscle function. Therefore, when physicians encounter patients that are difficult to wean from mechanical ventilation, CIP associated with BDP should be considered in the differential diagnosis. PMID:26252301

Imaging modalities used to confirm diaphragmatic hernia in small animals

International Nuclear Information System (INIS)

Williams, J.; Leveille, R.; Myer, C.W.

1998-01-01

When a patient is presented for treatment following a traumatic accident such as being hit by a car, thoracic radiographs are usually an integral part of the overall diagnostic evaluation. Diagnosis at diaphragmatic hernia (DH) is often challenging in small animals. The thorax may contain substantial fluid, thereby masking the presence of cranially displaced abdominal soft tissues (e.g., liver or spleen). The most common cause of decreased radiographic visualization of the diaphragm on survey radiographs is pleural fluid; however, the second most common cause is DH. Obviously, if a gas-filledviscus is identified within the thoracic cavity on survey radiographs, the diagnosis of DH is straightforward and relatively routine. If, however, there is substantial pleural effusion and the herniated structure is a soft tissue parenchymal organ (e.g., liver or spleen), the diagnosis is less clearly defined on survey radiographs. This review discusses the various imaging modalities (survey, positional, and contrast-enhanced radiographs and ultrasonography) that can be used in the diagnosis or confirmation of DH

Congenital chylous ascites

International Nuclear Information System (INIS)

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

2011-01-01

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

Congenital nystagmus and negative electroretinography

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Roussi M

2011-04-01

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

Congenital and perinatal cytomegalovirus infection

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