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

Congenital Esophageal Stenosis: To dilate or To resect

Congenital esophageal stenosis [CES] is a rare condition in children. The definitive preoperative diagnosis often is difficult to make, and a standard therapeutic protocol remains controversial. This study was carried out to determine the proper management strategy in children suffering from CES. The medical records of 17 patients with CES treated during a period of10 years [from 1995 to 2005] were retrospectively reviewed. Each patient was evaluated as regard to the clinical presentation, pathology, management, and outcome. The ages of patients at time of diagnosis ranged from 3 months to 9 years. The sites of stenosis were located more frequently at the lower third of the esophagus [n=10] than the upper third [n-3] and middle third [n=4], The diagnosis was made by esophagogram, esophagoscopy and was confirmed by histopathologic examination. Fifteen patients were diagnosed primarily, while 2 patients were diagnosed after unsuccessful surgical treatment for an initial misdiagnosed achalasia of the cardia. Six patients had confirmed tracheobronchial remnants [TBR], five had fibromuscular stenosis [FMS] and 2 had membranous web stenosis [MS]. The histopathology was unknown in 4 patients due to inadequate biopsies taken during esophagoscopy. All patients were treated initially by repeated esophageal dilatations [2-8 times] over two to thirty month period. The dilatation alone was successful in 11 patients, but was complicated by esophageal perforation in one case. Six patients required surgery; five of them were treated by resection and anastomosis, and one required esophageal replacement The pathology of this later group was TBR in 5 patients and FMS in one. 1. this study emphasizes the diagnostic difficulties in some children with CES; 2. Esophageal dilatation may be tried initially 3. Resection should be reserved for cases not responding to repeated dilatation particularly those with proven TBR

Evaluation of TC sign by 3 dimensional sonography in extrahepatic biliary atresia: a prospective study

The tubular or triangular cord sign [TC sign] is very sensitive and specific in diagnosis of extrahepatic biliary atresia. It is a b and -like periportal echogenicity, which represents a cone shaped fibrotic mass cranial to the bifurcation of portal vein. TC sign anatomic characterization using 3 dimensional sonography in infants with biliary atresia. 20 infants having biliary atresia underwent both 2 dimensional and 3 dimensional ultrasonographic examinations using both 5 and 7 MHz convex linear transducers. Ultrasonographic findings were correlated to intraoperative details. 11 infants with neonatal hepatitis with absent TC sign were evaluated as a control group. The TC sign identified on 2 dimensional sonography was identified by 3 dimensional sonography. 3D ultra-sonography characterized the TC sign as the left hepatic bile duct, which was completely obliterated in 18 infants, [confirmed by intra-operative findings] and partially occluded in 2. It converged to meet the right hepatic duct which was also seen occluded [in all cases] as a fibrotic b and seen through a right modified oblique lateral scan [while using the liver as an acoustic window]. The common hepatic duct was seen occluded in all infants. The common bile duct was seen occluded in all except one which showed non-communicating cystic dilatation [confirmed by intra-operative cholangiogra-phy]. All infants with idiopathic neonatal hepatitis had patent intact biliary systems and recovered in the following 6-10 months. TC sign represents the obliterated left hepatic bile duct. 3Dimensional ultrasonography is superior to 2D sonography in providing better diagnostic imaging of the biliary tract in infants with cholestasis