Diagnosis and treatment of biliary hypoplasia: a multicenter survey

Objective To enhance the awareness of biliary hypoplasia (BH) through exploring its diagnosis, treatment and prognosis. Methods For this retrospective study, the clinical data of BH were collected at five domestic hospitals from January 2009 to January 2017. There were 18 boys and 12 girls with an operative age of (83±36) days, a birth weight of (2.75±1.0) kg and a postnatal time of jaundice at (30±6) days. Their birth was term (n=21) and premature (n=9). And the stool was pale yellow stool (n=28) and white (n=2); dark urine (n=19); hepatomegaly & splenomegaly (n=18); mild ascites (n=12). Results All of them were operated. The procedures included cholecystostomy (n=20), cholangiography & biliary flushing (n=8) and non-treatment after cholangiography (n=2). And postoperative medications included antibiotics (n=15), hormone (n=22), hepatic protector & cholagogue (n=30) and gamma globulin (n=9). The follow-up period was 40 to 288 weeks. Jaundice subsided (n=21) and persisted (n=9) within 6 months. And 4/9 had the complications of itchiness (n=3) (1/3 with growth retardation) and anemia (n=1). They were divided into two groups of good prognosis (n=21) and poor prognosis (n=9) according to whether or not jaundice clearing at 6 months post-operation (icteric regression, total bilirubin ≤20 μmol/L), the growth up to standard and presence/absence of complications. Hepatocyte degeneration (n=6 vs. 8) and spot-like necrosis (n=3 vs. 7) were found in good and poor prognosis group. And the difference was statistically significant (P=0.004 & 0.002). Conclusions As a rare disease, BH may be diagnosed by cholangiography and liver biopsy and treated by surgery and postoperative medications. Surgical options include cholecystostomy and flushing. The overall prognosis is excellent. A poor prognosis may be associated with hepatic injury. Key words: Biliary Tract; Dysplasia; Retrospective studies; Multicenter study