Surgical Management of Congenital Intrahepatic Bile Duct Dilatation, Caroli's Disease and Syndrome

Abstract

LR for unilobar and LT for diffuse bilobar congenital IHBDD complicated with cholangitis and/or portal hypertension achieved excellent long-term patient outcomes and survival. Because of the bad prognosis of cholangiocarcinoma and the sizeable morbidity-mortality after LT, timely indication for surgical treatment is of major importance.

Cystic Bile Duct Dilatations and Caroli’s Disease

“…The reported average prevalence is close to 7 %, but higher values of up to 25-30 % have been reported [ 10 ]. The prevalence of cholangiocarcinoma in a recent French multicenter study [ 10 ] was 5.2 %, but the same authors stated that this prevalence may be underestimated because the series is a retrospective and multicentric study and therefore patients with malignancy who did not require surgery were not included in the study.…”

“…Liver resection (LR) is considered as the treatment of choice for patients with localized forms without underlying chronic liver disease, when the impaired intrahepatic bile ducts can be completely resected [ 10 ]. In contrast, a diffuse bilobar disease is more challenging to cure and patients in whom radical resection cannot be performed are candidates for OLT [ 10 ] (Fig. 9.15 ).…”

“…The extent of the disease (localized or diffuse) into the liver and the presence of concurrent underlying chronic liver (congenital hepatic fi brosis, secondary biliary cirrhosis, CC) or kidney disease are decision-making factors infl uencing the surgical management. Liver resection (LR) is considered as the treatment of choice for patients with localized forms without underlying chronic liver disease, when the impaired intrahepatic bile ducts can be completely resected [ 10 ]. In contrast, a diffuse bilobar disease is more challenging to cure and patients in whom radical resection cannot be performed are candidates for OLT [ 10 ] (Fig.…”

“…9.7 ) [ 8 ], and was later classifi ed by Guntz et al [ 9 ] according to shape and extent of intrahepatic bile duct dilatations (type 1 grape-bunch-like saccular of peripheral IHBDD, type 2 fusiform dilatation of large IHBDD, and type 3 saccular dilatation of large IHBDD). Unilobar extension of Caroli's disease is more frequently encountered (80 %) in surgical series [ 10 ], especially on the left side of the liver ( Fig. 9.8 ), but the disease may diffusely involve the entire intrahepatic biliary tree.…”

Hepatolithiasis

“…This entity was fi rst described by Caroli et al [ 5 ] in 1958 and later classifi ed by Guntz et al [ 6 ] according to the morphology and the extent of intrahepatic bile duct dilatations. A recent multicenter study conducted by the French Association of Surgery [ 7 ] has shown that prevalence of intrahepatic lithiasis in patients with Caroli's disease is above 50 %, whereas in Eastern patients, it seems mainly related to biliary infestations and infections [ 8 ].…”