Extrahepatic bile duct atresia: how efficient is the hepatoporto-enterostomy? 1998

P Schweizer, and K Lünzmann
Department of Pediatric Surgery, University of Tübingen, Germany.

OBJECTIVE Prospectively achieved findings of 151 children with EHBA operated between 1972-1997, of 108 patients in particular who were operated five and more years ago, should give evidence of the efficiency of HPE. METHODS For five and more years the development of the disease was prospectively evaluated according to a cognitively conditioned program. The objective source parameter was the hepatic histology at the time of surgery. Departing from that parameter the structural hepatic changes were followed up for five years and more. They were related to the bile flow achieved and the age of the patient, at the time of surgery as well as to the present time of follow-up. RESULTS Of 108 patients who were operated five and more years ago, 62 are still alive (57%) 58 children are icterus-free. The following results can be stressed: 1) There is a close correlation between the age of the patient and the fibrosis level of the liver at the time of surgery. 2) There is a close correlation between the age and the fibrosis level at the time of surgery on the one hand and the long-term survival rate, the absence of icterus and the state of the hepatic function on the other. 3) There is a close correlation between the age and the fibrosis level at surgery on the one hand and the operatively feasible (continued) bile flow on the other. 4) With favorable conditions concerning age and fibrosis level at the time of surgery, the size of the bile flow achieved through surgery is influenced positively and negatively by the morphology and bio-physical parameters of the porta hepatis as well as by postoperative cholangitis episodes. CONCLUSIONS The efficiency of HPE depends on the age and (in correlation to that) on the level of hepatic fibrosis at the time of surgery, on the morphology and on bio-physical properties of the porta hepatis as well as on cholangitis episodes occurring after surgery. The fibrosis level of the Glisson's triads at the time of surgery, the morphology and bio-physical parameters of the porta hepatis are documented as causes for failing or discontinued bile flow after HPE and for the progression of fibrosis. In the absence of obvious causes it is postulated that the initially obliterating and continuing primary disease has progressed. The performance of the HPE is summarized in the results: of 108 children who were operated five and more years ago 62 are still alive, 58 are icterus-free, 25 show no progression of fibrosis and 46 of 82 patients who were initially cirrhosis-free have no cirrhosis at present.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008105 Liver Cirrhosis, Biliary FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes. Biliary Cirrhosis,Biliary Cirrhosis, Primary,Biliary Cirrhosis, Secondary,Cholangitis, Chronic Nonsuppurative Destructive,Liver Cirrhosis, Obstructive,Primary Biliary Cholangitis,Biliary Cirrhosis, Primary, 1,Primary Biliary Cirrhosis,Secondary Biliary Cholangitis,Secondary Biliary Cirrhosis,Biliary Cholangitides, Primary,Biliary Cholangitis, Primary,Biliary Cholangitis, Secondary,Cholangitides, Primary Biliary,Cholangitis, Primary Biliary,Cholangitis, Secondary Biliary,Cirrhosis, Biliary,Cirrhosis, Secondary Biliary,Liver Cirrhoses, Biliary,Obstructive Liver Cirrhosis,Primary Biliary Cholangitides,Secondary Biliary Cholangitides
D008297 Male Males
D011171 Portoenterostomy, Hepatic Operation for BILIARY ATRESIA by anastomosis of the BILE DUCTS into the JEJUNUM or DUODENUM. Hepatic Portoenterostomy,Hepatoportoenterostomy,Kasai Procedure,Hepatic Portoenterostomies,Hepatoportoenterostomies,Portoenterostomies, Hepatic
D011183 Postoperative Complications Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery. Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D011446 Prospective Studies Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group. Prospective Study,Studies, Prospective,Study, Prospective
D011788 Quality of Life A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease. HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children

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