Lee Hung-Chang, Yeung Chun-Yan, Fang Shiuh-Bin, Jiang Chuen-Bin, Sheu Jin-Cherng, Wang Nien-Lu
Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan, ROC.
J Formos Med Assoc. 2006 Feb;105(2):118-24. doi: 10.1016/S0929-6646(09)60332-6.
This study analyzed the clinical presentation, postoperative morbidity and mortality and incidence of associated extrahepatic biliary atresia in children with biliary cysts in Taiwan.
We retrospectively reviewed the records of 158 pediatric patients with biliary cysts seen between June 1981 and July 2004, with follow-up ranging from 12 months to 22 years (mean, 11.2 +/- 6.1 years). Patients were divided into three groups: biliary atresia-associated biliary cyst (BABC, 21 patients), non-biliary atresia-associated choledochal cyst (NBACC) in infancy (37 patients), and late NBACC (> 1 year of age, 100 patients).
BABC accounted for 36.2% of the infantile biliary cysts in this study. Extrahepatic cysts in late NBACC had a greater mean diameter than those in infantile NBACC and BABC (21.5 mm vs. 16.0 mm vs. 7.9 mm, p < 0.001). Cholangitis was the most serious complication within 3 months postoperatively in all three groups, resulting in four deaths (two in the infantile NBACC group and one each in the other two groups). Liver cirrhosis developed during long-term follow-up in nine of the 21 patients with BABC, four of whom died. Three of these nine patients underwent liver transplantation and remained well during follow-up. Chronic complications in NBACC occurred mainly in late IVa cases, with persistent intrahepatic dilatation developing in 12 of 35 patients and intrahepatic stones in five. Elevation of serum alanine aminotransferase (ALT) was found preoperatively in 85% of late NBACC and 35% of infantile NBACC cases. Postoperative normalization of ALT occurred after a mean of 152 +/- 23 days and 158 +/- 67 days in late NBACC and infantile NBACC, respectively. Higher ALT levels before operation were associated with a longer period until normalization.
The possibility of BABC must be included in the differential diagnosis when a small extrahepatic cyst (< 8 mm in diameter) with prolonged jaundice is found in infancy. Postoperative follow-up is essential for patients with NBACC due to their frequently prolonged elevation of serum ALT and possibility of residual intrahepatic dilatation. Cholangitis was the major cause of death within 3 months postoperatively in this study.
本研究分析了台湾地区胆管囊肿患儿的临床表现、术后发病率、死亡率及合并肝外胆管闭锁的发生率。
我们回顾性分析了1981年6月至2004年7月间收治的158例小儿胆管囊肿患者的病历，随访时间为12个月至22年（平均11.2±6.1年）。患者分为三组：胆管闭锁相关胆管囊肿（BABC，21例）、婴儿期非胆管闭锁相关胆总管囊肿（NBACC，37例）和大龄非胆管闭锁相关胆总管囊肿（>1岁，100例）。
本研究中，BABC占婴儿期胆管囊肿的36.2%。大龄非胆管闭锁相关胆总管囊肿的肝外囊肿平均直径大于婴儿期非胆管闭锁相关胆总管囊肿和BABC（21.5mm对16.0mm对7.9mm，p<0.001）。胆管炎是所有三组术后3个月内最严重的并发症，导致4例死亡（婴儿期非胆管闭锁相关胆总管囊肿组2例，其他两组各1例）。21例BABC患者中有9例在长期随访中发生肝硬化，其中4例死亡。这9例患者中有3例行肝移植，随访期间情况良好。非胆管闭锁相关胆总管囊肿的慢性并发症主要发生在IVa期晚期，35例患者中有12例出现持续性肝内扩张，5例出现肝内结石。大龄非胆管闭锁相关胆总管囊肿患者术前85%和婴儿期非胆管闭锁相关胆总管囊肿患者术前35%的血清丙氨酸氨基转移酶（ALT）升高。大龄非胆管闭锁相关胆总管囊肿和婴儿期非胆管闭锁相关胆总管囊肿术后ALT分别平均在152±23天和158±67天恢复正常。术前ALT水平较高与恢复正常所需时间较长有关。
婴儿期发现直径较小（<8mm）且黄疸持续时间较长的肝外囊肿时，鉴别诊断必须考虑BABC的可能性。非胆管闭锁相关胆总管囊肿患者术后随访至关重要，因为他们的血清ALT常长期升高且可能存在残留肝内扩张。胆管炎是本研究中术后3个月内死亡 的主要原因。
