Congenital duodenal obstruction

Background/ Purpose: Congenital duodenal obstruction is a frequent cause of intestinal obstruction in the newborn. This study aimed to analyze various factors affecting the outcome of these cases at our institution

Materials and Methods: Seventy one cases of congenital duodenal obstruction were included in this retrospective review. Each case was studied as regard to: age at presentation, gestaional age, clinical data, other associated congenital anomalies, cause of obstruction, management, and outcome. Patients with abdominal wall defects [omphalocoele, gastroschisis] and diaphragmatic hernias were excluded from the study

Results: The causes of duodenal obstruction were: duodenal atresia [n= 37], duodenal diaphragm [n= 12], malrotation [n= 14], and annular pancreas [n= 8]. Age ranged from 2 days to 24 months. Bilious vomiting was the main presenting symptom. Plain radiography was the most valuable diagnostic tool in all cases except malrotation and partial obstruction. Gastrointestinal [GIT] contrast study was very valuable in that later group. Overall mortality was 15 cases [21.1 %]. The causes of deaths were: prolonged gastric stasis and neonatal sepsis[n= 7], other associated cardiac anomalies [n=5], and extensive bowel gangrene due to neglected volvulus neonatorum[n= 3]

Conclusion: This study showed that [1] the diagnosis of congenital duodenal obstruction is still delayed in many patients referred to our institutions.[2] early postoperative survival is still far from ideal ; [3] the mortality is related to delayed presentation, associated cardiac defects, and prolonged gastric stasis; and [4] late complications are more common than previously expected

Progression of disease in extra hepatic biliary atresia is associated with reduced total hepatic blood flow: descriptive results of a prospective pilot study

Hepatic fibrosis and cirrhosis develop progressively in extrahepatic biliary atresia despite timely surgical intervention. We aimed to study total hepatic blood flow [hepatic artery and portal vein flow] as a possible predictive factor of outcome of infants having biliary atresia who had underwent Kasai portoenterostomy. Twenty Infants having biliary atresia underwent colored and pulsed Doppler ultrasound studies. They were done before and 2-3 months after portoenterostomy. Hepatic artery, portal vein and single hepatic vein mean flow, mean diameter, mean velocity, hepatic arterial to portal venous flow ratio and total hepatic flow/kg were calculated and correlated to final outcome. The detected mean total hepatic flow and total hepatic flow/kg preoperatively was 685.5 +/- 296 ml/min and 147.1 +/- 51.4 ml/min/kg and post-operatively in those who became anicteric was 854.4 +/- 107 ml/min and 149.5 +/- 37.2 ml/min/kg, 539.2 +/- 337.7 ml/min and 112.1 +/- 78.6 ml/min/kg in those who developed chronic disease and in those who died was 157.6 +/- 79.6 and 30.9 +/- 16.1 ml/min/kg respectively. Unresolving cholestasis in infants having biliary atresia with poor outcome following portoenterostomy is associated with decreased post-operative total hepatic flow. Preoperative total hepatic flow did not correlate with postoperative total hepatic flow

importance of pathological findings in identification of patients at risk of developing Hirschsprung's disease associated enterocolitis

Enterocolitis remains the most serious complication of Hirschsprung's disease, and has been reported in 15% to 50% of cases. Patients are prone to develop relapses despite a defunctioning colostomy and after definitive pull-through procedures. There are several risk factors as delayed diagnosis of Hirschsprung's disease, long spastic segment, trisomy 21 and .family history of Hirschsprung's disease. Currently no clinical factor or test is available to predict patients prone to develop postoperative enterocolitis. Thirty-two Hirschsprung's disease patients were studied in an attempt to correlate the histologic changes of the colonic mucosa with the clinical and post-operative course to outline a histological guide line of value in prediction of patients prone to develop clinical enterocolitis