Extrahepatic biliary atresia (EHBA) is an infrequent disease confined to early infancy. Its aetiology is not clear, but an infectious agent has been implicated. Demonstration of a time-space clustering would support this hypothesis. Therefore, we investigated the time-space distribution of all 89 cases of EHBA born in a 10-year period in the Netherlands. We carried out a similar study in West Germany and analysed 130 cases of EHBA, born between 1969 and 1986, from 4 paediatric surgical centres. Analysis of these cases did not reveal any evidence for clustering in specific years or in a specific period of the year. The places of birth of the patients also were randomly distributed over rural areas, villages and towns. Neither did a method to reveal time-space interaction give any support for clustering. This random distribution of patients with EHBA suggests that EHBA could be pathogenetically a heterogeneous disease.

Portal vein arterialization (PVA) is an acquired concept in shunt surgery for portal hypertension. This technique, recently described as both a temporary and permanent procedure in adult liver transplantation, is reported by the authors in two cases of pediatric transplantation. The indication was low portal blood flow after reperfusion with poor graft function due to persistence of spontaneous retroperitoneal venous shunts. In both cases described, PVA allowed for satisfactory macroscopic liver reperfusion. The increase in portal blood flow from 150 to 500 ml/min in the second patient enabled the liver to be reperfused correctly and led to successful transplantation. The graft function in both cases improved in the 1st postoperative week, but thrombosis of the PVA occurred in the 1st patient 2 months after transplantation. Signs of hepatic hyperarterialization occurred in the second patient and this necessitated a dearterialization of the portal vein 2 weeks later. Although the benefit of this procedure appears to be beyond doubt in the immediate postoperative period, we have no data on long-term arterialization. We do think that PVA can be performed in pediatric liver transplantation, but it may need to be done only in special, individual situations when no valid alternative can be proposed, such as in the absence of a mesenteric vein and/or the presence of spontaneous retroperitoneal venous shunts.

Background: Bile excretion is obstructed in children with extrahepatic bile duct atresia (EHBA) resulting in fat malabsorption and disturbed lipid metabolism. Aim: Investigate if the bile duct ligated rat exhibits similar deviations as patients with EHBA under different feeding conditions. Methods: 6 bile duct ligated Wistar rats and 12 matched paired controls were randomised over 3 feeding groups. Rats were killed 16 or 30 days postsurgery. Faeces, blood and livers were collected. Fat absorption was evaluated, markers for cholestasis and the fatty acid composition of serum phospholipids (PL) and cholesterol esters (CE) were determined. Fatty acid desaturation activities in liver microsomes were measured. Results: Cholestatic bile duct ligated rats have a lower fat absorption coefficient and a lower fraction of 18:2n–6 and 18:3n–3 in serum triglycerides than their controls. This demonstrates that bile duct ligated rats suffer from fat malabsorption. In contrast to the observations in serum triglycerides, 18:2n–6 and 18:3n–3 were not reduced in serum PL and CE of cholestatic rats. Overflow of 18:2n–6 rich biliary PL in the general circulation could contribute to this observation. In agreement with what was found in man, serum PL of cholestatic rats have a higher 16:0/18:0 ratio, increased monoenes and reduced unsaturated fatty acids. However, no differences were observed in microsomal desaturation activities. Conclusion: Cholestatic bile duct ligated rats exhibit similar deviations in serum fatty acid composition as found in patients with EHBA, therefore they can be used as a model for this human disease.

In the differential diagnosis of unclear neonatal and infant cholestasis the formerly applied 131-I-Rose Bengal scintigraphy has been widely abandoned in favour of hepatobiliary scintigraphy with 99mTc-labelled IDA derivatives excreted with the bile. Our results in 32 jaundiced children ranging from ages 2 weeks to 32 weeks show a high sensitivity of scintigraphy in proving an occlusion in 92% with a clearly lower specificity of 79%, if based on the demonstration of marked bile in the intestine as the only criterion. Diagnostic accuracy, however, can be further increased in additional criteria such as hepatocellular clearance and patient age are considered. The examination is of equally high importance for post-operative follow-up. Here, scintigraphy is superior to all other imaging procedures in the evaluation of bile flow situations. In 52 examinations of portoenterostomies, the prognostic value can be demonstrated not only with a good coincidence of the scintigraphic data with the bilirubin level, but also with the histological degree of liver fibrosis at the time of operation. Early and late complications of hepatoportojejunostomy can usually be recognised and localised. Since the prognosis of extrahepatic biliary atresia depends on an early operation-presently by means of reconstruction of biliary flow-indications for hepatobiliary scintigraphy must be given more widely and earlier (at the latest in the fourth week of life) in all unclear cases of cholestatic syndrome.