Lal Richa, Agarwal Shaleen, Shivhare Rakesh, Kumar Ashok, Sikora Sadiq S, Saxena Rajan, Kapoor Vinay K
Department of Surgical Gastroenterology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences (SGPGIMS), Lucknow, 226014, India.
J Hepatobiliary Pancreat Surg. 2005;12(2):129-34. doi: 10.1007/s00534-004-0960-1.
BACKGROUND/PURPOSE: Type IV-A choledochal cysts are characterized by congenital cystic dilatation of the biliary tree extending to involve the intrahepatic biliary channels also. A single-center experience of the management of type IV-A choledochal cysts is presented.
Thirty-five out of 105 (33%) patients with choledochal cysts, who underwent surgery at a tertiary care center in northern India from January 1989 to December 2002, were found to have a type IV-A (Todani's classification) cyst. The mean age of the patients was 24 years (range, 3 months to 60 years); 17 patients in the group were adults and 22 were females. Presenting features were abdominal pain, jaundice, cholangitis, and abdominal lump, in various combinations.
Excision of the extrahepatic part of the cyst and a wide bilio-enteric anastomosis was achieved in 32 (91%) patients, while internal drainage of the cyst was necessitated in 3 patients, for technical reasons: collaterals due to portal hypertension (1 patient) and dense adhesions (2 patients). Six (17%) patients developed postoperative complications: 3 had bilio-enteric anastomosis leaks, with 2 requiring a percutaneous proximal biliary diversion; 2 had intraabdominal bleeds requiring re-exploration, and 1 had external pancreatic fistula that closed spontaneously. Follow-up information was available for 28 (80%) patients. Mean duration of follow up was 25 months (median, 12 months; range, 6 months to 9 years). Three patients required re-operation, for anastomotic stricture (n = 2) and hepatolithiasis and recurrent cholangitis (n = 1) during follow up.
Excision of the extrahepatic part of the cyst and drainage of the intrahepatic part by a wide hilar or subhilar anastomosis gave satisfactory results in the majority of patients with type IV-A choledochal cysts. Close long-term follow up of these patients is essential, because they are likely to present with complications related to the residual intrahepatic part of the disease.
背景/目的：IV-A型胆总管囊肿的特征是胆管树先天性囊性扩张，且累及肝内胆管。本文介绍了单中心对IV-A型胆总管囊肿的治疗经验。
1989年1月至2002年12月在印度北部一家三级医疗中心接受手术的105例胆总管囊肿患者中，35例（33%）被诊断为IV-A型（Todani分类）囊肿。患者的平均年龄为24岁（范围3个月至60岁）；该组中有17例成年患者，22例为女性。主要症状为腹痛、黄疸、胆管炎和腹部肿块，各种症状组合出现。
32例（91%）患者成功切除囊肿肝外部分并进行了广泛的胆肠吻合，3例因技术原因进行了囊肿内引流：门静脉高压导致的侧支循环（1例）和致密粘连（2例）。6例（17%）患者出现术后并发症：3例发生胆肠吻合口漏，2例需要经皮近端胆管引流；2例腹腔内出血需要再次手术探查，1例胰外瘘自行闭合。28例（80%）患者有随访信息。平均随访时间为25个月（中位数12个月；范围6个月至9年）。随访期间，3例患者因吻合口狭窄（2例）和肝内胆管结石及复发性胆管炎（1例）需要再次手术。
对于大多数IV-A型胆总管囊肿患者，切除囊肿肝外部分并通过广泛的肝门或肝门下方吻合引流肝内部分可取得满意效果。对这些患者进行密切的长期随访至关重要，因为他们可能会出现与疾病残留肝内部分相关的并发症。
