Portal cavernoma cholangiopathy in pediatric extrahepatic portal vein obstruction with or without shunt surgery.
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BACKGROUND: Portal cavernoma cholangiopathy (PCC) has recently been recognized as an abnormality of the intrahepatic and extrahepatic bile ducts caused by external compression by the portal cavernoma or ischemia in the biliary region. Although extrahepatic portal vein obstruction (EHPVO) is common in children with portal hypertension, the association between surgical treatment for EHPVO and the incidence of PCC in children is not well known. METHODS: We retrospectively reviewed the medical records of 14 cases of childhood-onset EHPVO in our department. PCC was defined by intra- and extrahepatic bile duct irregularities on imaging, both symptomatic and asymptomatic. RESULTS: There were six cases with PCC (Group A) and eight cases without PCC (Group B). EHPVO was diagnosed at 4.8 years and 4.1 years of age in Groups A and B, respectively. PCC was asymptomatic, except in one patient with repeated cholangitis. In Group A, 4/6 patients (66.7%) showed sufficient intrahepatic portal vein inflow via cavernoma at hilum, whereas in Group B, almost all cases (7/8 cases, 87.5%) showed poor intrahepatic portal vein delineation. PCC did not develop in 4/5 patients (80%) who underwent portosystemic shunt surgery, such as mesocaval shunting. CONCLUSIONS: PCC is a serious complication of EHPVO that affects its long-term prognosis. Although there are concerns about long-term problems such as hyperammonemia and pulmonary complications after portosystemic shunt surgery, it may also be beneficial as a prophylactic surgery for PCC.