The most commonly associated anomalies in patients with extrahepatic biliary atresia are cardiovascular, digestive and splenic defects. Of the cardiovascular anomalies, there are very few reports of biliary atresia with cardiomyopathy. We report the first case of a child with extrahepatic biliary atresia and restrictive cardiomyopathy. The patient was a 13-month-old boy diagnosed with extrahepa...

Infants with Down syndrome are known to have a high frequency of associated birth defects and some authors have suggested an association between Down syndrome and oesophageal atresia. We evaluated data from the Sicilian Registry of Congenital Malformations. Our finding of an incidence of 0.9% of oesophageal atresia in children with Down syndrome is more than 30 times higher than expected and mo...

rectovaginal fistula with atresia ani is a congenital condition that affects the anal opening and rectum by the formation of an abnormal connection between the rectum and vagina. this was diagnosed in a domestic short hair kitten. the presenting physical abnormalities included depression, dehydration, partial tail agenesis, anal atresia and a discharge of watery feces from the vaginal opening. ...

Isolated atresia of the aortic arch is rare. In aortic arch atresia the arch is continuous, yet a segment of it is atretic so that apart from a collateral circulation, communication with the lower aortic segment is absent. Haemodynamically, the condition is similar to complete interruption of the aortic arch. To our knowledge only 10 cases of isolated aortic arch atresia or interruption have be...

The electrocardiograms from 28 unselected autopsied cases of tricuspid atresia, and eight autopsied cases of single ventricle were analysed: only those cases of single ventricle which showed left axis deviation were included. Electrocardiographic evidence of left ventricular preponderance in the precordial and unipolar limb leads was found in 27 of the 28 cases of tricuspid atresia, and three o...

OBJECTIVE To examine the prevalence of congenital heart disease in babies with oesophageal atresia and its influence on outcome. DESIGN Retrospective analysis. SETTING The resident population of one health region. RESULTS A total of 153 babies with oesophageal atresia were identified from 509 975 live births (0.30 per 1000); 26 (17%) had cardiac defects. Survival of babies with normal hea...

INTRODUCTION Laparoscopic duodenal atresia repair is an advanced procedure performed in select pediatric surgical centers. Recently, sub-6mm endosurgical staplers have been introduced, facilitating and accelerating the creation of intracorporeal intestinal anastomoses. PRESENTATION OF CASE We performed a laparoscopic duodenojejunostomy in a one-day-old child with duodenal atresia due to annul...

We are reporting two neonates with isolated pyloric atresia, with an objective to emphasize the importance of considering this rare condition in the differential diagnosis of upper intestinal atresias. Both had non-bilious vomiting and epigastric fullness. X-ray showed a dilated gastric shadow with no evidence of gas in the rest of the abdomen. On exploration they had pyloric atresia which was ...

In a cell culture model of bile duct epithelial cells, the effect of prostaglandin E2, lithocholic acid and deoxycholic acid was studied. Bile acids and prostaglandin are administered postoperatively in biliary atresia empirically as choleretics. Prostaglandin E2 and the bile acids all had inhibitory effects on bile duct epithelial cells in culture. There is no clinical study proving the effica...

Long gap esophageal atresia with or without tracheo-esophageal fistula may require feeding gastrostomy as a part of staged reconstruction.[1] We have devised a technique to secure/anchor the feeding gastrostomy (Stamm gastrostomy) more easily and safely. Five neonates underwent feeding gastrostomy by this technique. Out of five patients, four patients had tracheo-esophageal fistula with long ga...