Kasai Yöntemiyle Portoenterostomi Yapılan Biliyer Atrezi Olgularında Sağkalımı Etkileyen Faktörlerin İncelenmesi

Abstract

Biliary atresia is a disease of intra and extra hepatic biliary ducts with inflammation and progressive destruction leading to death in two years if not treated. Surgical operation for biliary drainage is regarded as first-line treatment. High rates of event-free survival are accepted as a treatment success. Survival could be predicted when the prognostic factors are determined in biliary atresia cases. Sixty three biliary atresia cases underwent Kasai hepatoportoenterostomy between 2000 and 2012. Medical records of thirty five, of which up to date survival information obtained were reviewed retrospectively. Sex, birth date, age at hospitalisation and operation; laboratory test results; ALT, AST, GGT, ALP, TBL, DBL, APTT, INR, viral serology, ultrasonography, liver scintigraphy scan reports, operation notes and other medical records were noted and analyzed retrospectively. Cases needed liver transplantation or died defined as poor prognosis group; and the cases with event-free survival defined as good prognosis group. In good and poor prognosis groups, age at hospitalization was 52.43 ± 34.59 and 70.52 ± 42.95, age at operation was 69.21 ± 41.59 and 86.05 ± 37.17 days respectively and there was no significance (p=0.145, p=0.145). When defined according to age at the time of operation (younger and older than 60 days), there was 7 (70%) and 7 (28%) cases from the good prognosis group respectively and showed significance (p= 0.029). vi Serum levels of TBL and DBL after Kasai operation and just before discharging were 12.52 ± 4.79 mg/dL and 9.31 ± 3.44 mg/dL for poor prognosis group, and 8.16 ± 2.81 ve 5.81 ± 2.09 mg/dL for good prognosis group respectively. The good prognosis group had significantly lower levels of TBL and DBL (p=0.004, p=0.003). When a cut of value of 8.84 mg/dl for TBL was selected, the sensitivity and specificity rates were 75% and 62%; if the cut of value of 7.21 mg/dL for DBL was selected, sensitivity and specificity were 78.6% and 65% respectively. Ductal plate malformation was detected histopathologically in 4 (33%) cases in good prognosis group which was found significant higher since none of the cases in poor prognosis group showed this finding (p=0.047). There were no significant difference between the good and poor prognosis groups for incidences of cholangitis (14% and 33%, p>0.05). When evaluating BA, US, HIDA scintigraphy and operative examination should be performed as soon as possible. Preoperative liver needle biopsy should be used in suspicious and challenging cases for differential diagnosis. Early age at operation, decrease in DBL and TBL in the postoperative period and the presence of ductal plate malformation in histopathological examination are good prognostic factors for long term outcome.