title: Is congenital hepatic fibrosis a pure liver disease?
abstract: OBJECTIVE
  An association between congenital hepatic fibrosis (CHF) and several different conditions is being increasingly recognized. We aimed to investigate, prospectively, these associated disorders and the clinical consequences for patients with CHF.
  
  
  METHODS
  CHF was diagnosed using liver biopsy, abdominal ultrasound (US), Doppler US, upper endoscopy, and abdominal computed tomography (CT) in 19 patients (13 women, 6 men). CT portography and splenoportography with digital subtraction angiography were performed if indicated. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to investigate the extent of portal vein involvement of the common bile duct if it existed, to remove a stone located in the common bile duct when documented, and to confirm the diagnosis of Caroli's syndrome. Cranial MRI was done when clinical findings suggested brain involvement.
  
  
  RESULTS
  The mean age of the patients was 29.47+/-12.06, ranging from 13 to 57. CHF-associated diseases were Caroli's syndrome, polycystic kidney disease, cavernous transformation of the portal vein, Joubert's syndrome, von Meyenburg complex, polydactyly, medullary sponge kidney, and pancreatic duct atrophy. In two cases, cholangiocarcinoma had developed. There was only one case with pure CHF. Portosystemic shunt, TIPS, or splenectomy were performed in some cases to control bleeding from esophageal varices. Papillotomy and stone extraction from the common bile duct were performed in four patients with Caroli's syndrome complicated by cholangitis. Three patients died of complications of CHF. Two patients with Caroli's syndrome underwent liver transplantation.
  
  
  CONCLUSIONS
  In this prospective study, it seems that CHF is not a pure liver disease but rather a multiorgan disorder involving the brain, portal vein, kidneys, and bile ducts. In most cases, the clinical picture includes other organ involvement, rather than purely the liver parenchyma.
authors.affiliation: Department of Gastroenterology, Hacettepe University, Ankara, Turkey.
authors.forename: Ozlem
authors.initials: O
authors.lastname: Yönem
authors.forename: Nihal
authors.initials: N
authors.lastname: Ozkayar
authors.forename: Ferhun
authors.initials: F
authors.lastname: Balkanci
authors.forename: Ozgür
authors.initials: O
authors.lastname: Harmanci
authors.forename: Cenk
authors.initials: C
authors.lastname: Sökmensüer
authors.forename: Osman
authors.initials: O
authors.lastname: Ersoy
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Bayraktar
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D003952:Diagnostic Imaging; D005260:Female; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications / Q000151:congenital* / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D016019:Survival Analysis