Various surgical procedures in biliary duct malformations in childhood

Abstract

From the congenital biliary malformations the atresia and hypoplasia only are discussed with reference' to their operative therapy. At first a survey is given of the most important anatomical variations. The atresias are separated in operable (3) and non operable types (3). As far as concerning the four different types of hypoplasia only the intrahepatie variation does not need exploration. It seems that a great deal of the extrahepatie hypoplasias is masked under the inspissated bile syndrom. Among the direct bilio-digestive anastomosis in question of the operable atresias the ¥-en-Roux enterostemy ist pointed out. Numerous indirect bilio-digestive anastomosis have been tried in case of non operable atresias, mostly invain (hepato-enterostemy, cholangiojejunostomy, bile duct prosthesis after Sterling). Only the quadrate lobe resection (Hasse) and the hepato-porte-jejunostomy (Kasai-Kimura) are reported to be of some value. Procedures using an external or internal lymph-fistula represent the last group of possible methods to relieve cholestasis, for instance the lymphatico-esophageal anastomosis (Surnga), the lymphatieo-jejunal anastomosis (Fonkalsrnd and Longmire) a. o. The results of operation and in consequence the prognosis of atresia are so far bad. The literature refers 10--20°/0 as theoretically correctable and about 3% only as definitely curable. These figures are of course based on a rather in_homogeneons contingent of patients and should be read with a certain reservation in mind. Though operation must be tried in every case indicated. Today liver transplantation seems to be more hopeful.--From 1963--1971 we observed 31 cases of biliary atresia, survivors 1 (6 months postep.) and 22 real hypoplasias with 8 survivors.-In eases with correctable extrahepatic atresia we prefer an enterostemy of Y-en-Roux-type. in the rest the hepatoporto-jejunostomy after Kasai-Kimura seems to us to be the most hopeful method nowaday.Zusammen]assun9. Von den angeborenen GallenwegsmiBbildungen werden nur die Atresien und Hypoplasien hinsichtlich der operativen Therapie besprochen. Zun~chst wird eine ~bersicht ihrer wichtigsten anatomischen Formen gegeben, bei den Atresien getrennt naeh operablen (3) und inoperablen Typen (3). Von den 4 unterschiedenen Hypoplasieformen ist nur die intrahepatisehe nicht revisionsbediirftig. Ein gut Teil der extrahepatisehen Hypoplasien wird vermutlich durch das Syndrom der eingedickten Galle maskiert. Unter den fiir die operablen Atresien infragekommenden direkten bilio-digestiven Anastomosen wird die Enterestomie in Form der Roux-Anastomose wegen ihrer geringen Cholangitisgefahr herausgestellt. Bei den inoperablen Atresien wurden zahlreiche indirekte bilio-digestive Anastomosen(Hepato-Enterostomien, Cholangio-Jejunostomie, kiinstliche Gallengange nach Sterling) versucht. Lediglich die Lobns quadratus-Resektion (Hasse) und die Hepato-Porte-Jejunostomie (Kasai-Kimura) besitzen begrenzte Bedeutung. Die 45*