Lipsett P A, Segev D L, Colombani P M
Johns Hopkins Hospital, Baltimore, MD 21287, USA.
Baillieres Clin Gastroenterol. 1997 Dec;11(4):619-41. doi: 10.1016/s0950-3528(97)90013-0.
The authors present a review of the classification, aetiology, presentation, treatment and long-term outcome of children and adults with biliary atresia and choledochal cyst disease. Biliary atresia should be suspected in any infant with jaundice beyond the second week of life. Although the aetiology and pathogenesis remain unclear, early management with portoenterostomy has significantly improved the course of this disease. Recent advances in immunosuppression have made liver transplantation a valuable and necessary adjunct to biliary bypass. With choledochal cyst disease, adults, unlike children, often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.
作者对患有胆道闭锁和胆总管囊肿疾病的儿童及成人的分类、病因、临床表现、治疗及长期预后进行了综述。对于任何出生后第二周仍有黄疸的婴儿，都应怀疑患有胆道闭锁。尽管病因和发病机制尚不清楚，但早期进行肝门空肠吻合术已显著改善了该病的病程。免疫抑制方面的最新进展使肝移植成为胆道分流术有价值且必要的辅助手段。对于胆总管囊肿疾病，与儿童不同，成人常表现为急性胆道症状或胰腺炎。治疗的首选方法仍然是肝外囊肿切除和胆道分流术。这种治疗方法具有出色的长期效果，可将恶性肿瘤的发生风险降至最低。
