Congenital intrahepatic bile duct dilatation is a potentially curable disease: long-term results of a multi-institutional study.
OBJECTIVE
To report clinical presentation, perioperative outcome, and long-term results of surgical management of congenital intrahepatic bile duct (IHBD) dilatations (including Caroli disease) in a multi-institutional setting.


BACKGROUND
Congenital IHBD dilatations are a rare congenital disorder predisposing to intrahepatic stones, cholangitis, and cholangiocarcinoma. The management remains difficult and controversial for bilobar forms of the disease or when concurrent congenital hepatic fibrosis is associated.


METHODS
From 1976 to 2004, 33 patients (range 11 to 79 years) were retrospectively enrolled. Disease extent into the liver was unilobar in 26 patients and bilobar in 7 patients (21%). Cholangiocarcinoma, congenital hepatic fibrosis, and intrahepatic stones were present in 2, 10, and 20 patients, respectively. Transplantations or liver resections were performed in 5 and 27 patients, respectively, whereas 1 asymptomatic patient was managed conservatively.


RESULTS
Postoperative mortality was nil. Postoperative complications occurred in 16 of 32 operated patients (50%) and additional procedures for residual stones were required in 5 patients. During a median follow-up of 80 months (1 patient being lost for follow-up) no patient developed metachronous carcinoma. Six patients (30%) developed recurrent intrahepatic stones but satisfactory late outcome was achieved in 27 patients (87%).


CONCLUSIONS
Partial or total liver resection achieves satisfactory late outcome in congenital IHBD dilatations, when the affection is treated at an early stage and when the extent of liver resection is tailored to intrahepatic disease extent and takes into consideration the presence and severity of underlying chronic liver and renal diseases.
Mabrut
Jean-Yves
JY
Department of Abdominal Surgery and Transplantation, Saint-Luc University Hospital, Brussels, Belgium.
Partensky
Christian
C
Jaeck
Daniel
D
Oussoultzoglou
Elie
E
Baulieux
Jacques
J
Boillot
Olivier
O
Lerut
Jan
J
de Ville de Goyet
Jean
J
Hubert
Catherine
C
Otte
Jean-Bernard
JB
Audet
Maxime
M
Ducerf
Christian
C
Gigot
Jean-François
JF
D000328:Adult; D000368:Aged; D001649:Bile Duct Diseases / Q000151:congenital / Q000175:diagnosis / Q000601:surgery; D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities* / Q000601:surgery; D002648:Child; D002760:Cholangiopancreatography, Endoscopic Retrograde; D004108:Dilatation, Pathologic / Q000151:congenital / Q000175:diagnosis / Q000601:surgery; D005060:Europe / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy / Q000379:methods*; D006801:Humans; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D012720:Severity of Illness Index; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome