摘要目的:通过分析胆道发育不良(biliary hypoplasia，BH)诊断、治疗及预后，提高广大儿科医务工作者对BH的认识。方法:回顾性分析国内5家医院2009年1月至2017年1月间收治的30例BH患儿的临床随访资料，讨论BH的诊断、治疗及预后。其中，男18例，女12例；足月儿21例，早产儿9例；手术日龄为(83±36)d；出生体重为(2.75±1.00)kg；黄疸出现时间为生后(30±6)d。大便颜色浅黄28例，白色2例；尿色深19例；肝脾肿大18例，轻度腹水12例。结果:本组30例均行手术治疗，其中20例仅行胆囊造瘘，8例行胆囊造瘘及胆道冲洗，2例行胆道造影后未予其他治疗。术后使用抗生素15例，激素22例，保肝及利胆药30例，丙种球蛋白9例。本组获随访40～288周。术后6个月内黄疸消退21例；未消退9例，其中4例出现并发症，包括术后皮肤瘙痒3例(其中1例伴生长迟缓)，贫血1例。根据术后6个月内是否退黄(黄疸消退，总胆红素≤20 μmol/L)，生长发育是否达标，是否伴有并发症，将患儿分为预后良好组(21例)和预后不良组(9例)。胆道造影检查示预后良好组出现肝细胞变性6例，点状坏死3例；预后不良组出现肝细胞变性8例，点状坏死7例，两组上述指标间比较，差异均有统计学意义( P＝0.004和0.002)。 结论:BH为罕见疾病，通过胆道造影及肝活检诊断，主要治疗方法为外科手术及术后辅助用药，手术包括胆囊造瘘及胆道冲洗，总体预后良好，预后不良与肝损伤程度有关。

abstractsObjective: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.