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Long‐term studies on congenital hepatic fibrosis in children

Congenital hepatic fibrosis (CHF) is an inherited malformation, defined pathologically by bands of fibrous tissue within the liver, linking portal tracts and containing multiple bile ductules (I , 2). It occurs in association with a range of inherited disorders involving the kidneys (3). Clinically, it is characterized by portal hypertension with well-preserved liver function. In addition, various segments of the intrahepatic bile ducts may be dilated producing bile stasis and cholangitis (4). This exposes CHF patients to two major risks: gastrointestinal haemorrhage caused by portal hypertension, and cholangitis due to bacterial infection of dilated intrahepatic bile ducts (4). We report our experience in 18 children with CHF. Between 1980 and 1989, we diagnosed CHF in 18 children. Histological diagnosis of CHF was made after either surgical (n = 2) or needle liver biopsy ( n = 16). Fibrous enlargement of portal tracts or portal to portal bands of fibrous tissue containing abnormal bile ducts together with the preserved basic lobular architecture of the liver parenchyma were identified in all liver tissue specimens. Liver function tests were performed in all children. Two children with cholangitis underwent percutaneous transhepatic cholangiography and one child endoscopic retrograde cholangiopancreatography (ERCP). Evaluation of possible portal hypertension consisted of oesophagoscopy (n = 16) and splenoportography (n = 8). Urinalysis, serum creatinine, creatinine clearance and renal ultrasound scans were carried out in all cases. Histological features typical of CHF were observed in 18 patients (lOM, 8F). Mean age at the time of diagnosis was 10 years (range 3 months to 17 years). Of these, 16 children have survived and have been followed-up for approximately 6 years. The patients presenting symptoms and signs are shown in Table 1. Liver function tests were normal in nine children. Three of seven children with cholestatic jaundice had cholangitis due to Caroli’s syndrome. Variceal bleeding was the presenting manifestation in four children. Varices were found in an additional seven cases of whom three subsequently bled. Splenoportography was performed in eight patients. Splenic pressure was greater than 20 mmH20 in all eight children on whom splenoportography was performed. Five children initially underwent endoscopic sclerotherapy. Obliteration was achieved in four. Splenectomy and distal splenorenal shunt were performed in six patients. Bleeding from oesophageal varices occurred in two patients following the shunt procedure, but was controlled by variceal sclerotherapy. One patient with a shunt and subsequent sclerotherapy failure underwent a successful devascularization procedure because of life-threatening recurrent haemorrhage. Of 16 patients, all had normal growth except 1 child with Caroli’s syndrome and recurrent biliary infections. Two girls with CHF associated with Caroli’s syndrome and biliary cirrhosis developed hepatic encephalopathy, which improved with a reduced protein index. Intrahepatic bile duct changes characteristic of Caroli’s syndrome were demonstrated in three of four children with cholangitis by means of pecutaneous cholangiography (n = 2) and ERCP (n = 1) (Fig. 1). Two girls developed renal failure also; one had biopsy-proven membranoproliferative glomerulonephritis and the other renal tubular ectasia. Two children eventually died; the first patient with numerous severe attacks of cholangitis died of liver failure and the second with renal tubular ectasia died of renal failure. Congenital hepatic fibrosis is a developmental disorder which is encountered mainly in children and young adults (1-5). Clinical expression of CHF is variable (2, 6). Four clinical forms have been identified: a form with portal hypertension, a cholangitic form, a portal hypertensive-cholangitic form and a latent form (2, 6). This study has demonstrated that the portal hypertensive form is the most common and