胆道发育不良患儿的临床病理特点回顾

Retrospective analyses of clinicopathological features of biliary dysplasia

目的 探讨胆道发育不良患儿的临床表现、病理特点、诊断、治疗及预后,提高对该病的认识.方法 回顾性分析天津市儿童医院外科2010年6月至2016年11月收治的5例胆道发育不良患儿的发病情况、治疗过程,并通过复习国内外文献对胆道发育不良的疾病特点进行总结.结果 5例胆道发育不良患儿均因生后不久出现皮肤、巩膜黄染,内科治疗病情无改善入外科治疗;肝功能检查见胆红素水平升高,以直接胆红素升高为主,伴肝酶不同程度增高;超声检查示胆囊干瘪未充盈或胆囊腔狭小,胆总管显示不清.5例患儿行手术探查,术中造影显示肝内、外胆道通畅,管腔纤细;病理检查见部分汇管区中小叶间胆管缺失,小叶间胆管/汇管区＜0.5,诊断为胆道发育不良,予留置胆囊引流管,术后抗炎、补液、保肝及对症治疗.经2个月～6年门诊随访,5例患儿均存活至今,其中3例黄疸清除,1例皮肤仍黄染并伴有瘙痒,1例未退黄.结论 术前检查提示梗阻性黄疸患儿,术中造影提示胆道纤细,结合病理检查证实小叶间胆管/汇管区比例＜0.5,可以明确胆道发育不良的诊断.

Objective To explore the clinical manifestations,pathological characteristics,diagnosis,treatment and prognosis of biliary dysplasia.Methods From June 2010 to November 2016,five patients of biliary dysplasia were examined along with a literature review.Results With a postnatal history of jaundice of skin and sclera,they were not cured medically.Preoperative tests showed elevated levels of direct bilirubin and transaminase.Ultrasound indicated small or poor filing gallbladder.Intraoperative cholangiography showed intra and extra-hepatic bile duct patency with delicate luminal;pathological examination of liver biospy indicated an absence of interlobular bile duct in portal area and interlobular bile duct/portal ratio was 〈0.5.Then a drainage tube was inserted into gallbladder and symptomatic measures of anti-inflammatory,intravenous fluid therapy and hepatic protection were offered.During a follow-up period of 2-6 years,all patients survived.Three of them had jaundice clearance.Jaundice persisted in one case while another one had jaundice and skin itching.Conclusions Children with obstructive jaundice on intraoperative cholangiography having delicate bile duct and interlobular bile duct or portal area ratio 〈0.5 may establish a definite diagnosis of biliary dysplasia.