摘要
目的 提出胆管囊状扩张症的临床分型及针对不同分型的治疗策略和手术方法。方法 回顾性分析1993年6月至2010年6月解放军总医院收治的434例胆管囊状扩张症患者的临床资料。收集和重新分析患者腹部CT、MRI、MRCP和胆道造影检查结果，根据胆管囊状扩张病变累及胆管树的部位及范围，结合其临床病理特征、发病因素及适用的手术方式提出一种新的胆管囊状扩张症的临床分型；分析新分型中不同胆管囊状扩张症的临床表现、手术方式、围手术期结果、随访结果等资料，针对不同分型胆管囊状扩张症制订治疗策略和手术方法。率的比较采用X2检验，理论频数〈5或总观测频数〈30时，采用Fisher确切概率法。结果 根据囊状扩张病变累及胆管树的部位及病理特征将其分为5种类型：（1）A型：周围肝管型肝内胆管囊状扩张。A1型：囊状扩张病变局限分布于部分肝段；A2型：囊状扩张病变弥漫分布于全肝。（2）B型：中央肝管型肝内胆管囊状扩张。B1型：单侧肝叶中央肝管囊状扩张；B2型：囊状扩张病变同时累及双侧肝叶主肝管及左、右肝管汇合部。（3）C型：肝外胆管型胆管囊状扩张。C1型：囊状扩张病变未累及胰腺段胆管；C2型：囊状扩张病变累及胰腺段胆管。（4）D型：肝内外胆管型胆管囊状扩张。D1型：囊状扩张病变累及单叶中央肝管和肝外胆管；D2型：囊状扩张病变累及双侧肝叶中央肝管和肝外胆管。（5）E型：壶腹胆管型胆管囊状扩张。本组434例胆管囊状扩张症患者中，A型24例（A1型17例、A2型7例），B型13例（B1型10例、B2型3例），C型300例（C1型56例、C2型244例），D型96例（D1型17例、D2型79例），E型1例。24例A型患者中，14例伴有先天性肝纤维化，16例合并多囊肾病，区别于其他各型患者。手术方式：24例A型患者中，17例A1型患者行部分肝切除术，3例A2型患者行肝移植，1例A2型患者行囊状扩张病变穿刺引流术，3例A2型患者采用非手术治疗；13例B型患者中，12例患者行肝切除术，1例患者合并胆管癌，采用非手术治疗；300例C型患者中，286例患者行肝外囊状扩张病变切除＋胆管空肠吻合术，14例患者因囊状扩张胆管恶变行胆管癌根治性切除术；96例D型患者中，35例患者行肝外胆管囊状扩张病变切除＋肝切除（肝内胆管囊状扩张病变累及的部分肝组织）＋胆管空肠吻合术，59例患者仅行肝外囊状扩张病变切除术，1例D1型和1例D2型患者伴有胆管癌，行根治性切除术；1例E型患者行EST治疗。399例患者获得随访，随访时间1～15年，平均随访57个月。33例患者出现胆管空肠吻合口狭窄和（或）结石。24例患者并发胆管癌，其中15例患者于随访期内死亡。46例患者因复发性胆管炎伴有吻合口狭窄或结石、肿瘤实施再次手术治疗（胆管空肠再吻合术、内镜下胆管取石术、胆管癌根治性切除术等）。其余患者无相关临床症状或偶发轻度的胆管炎，经对症治疗缓解。D型患者中联合肝切除者与仅行肝外胆管囊状扩张病变切除者的症状缓解率、狭窄和（或）结石复发率、再手术率分别为88.2% （30/34）、8.8%（93/34）、11.8%（4/34）和64.4%（38/59）、28.8%（17/59）、35.6%（21/59），两者比较，差异有统计学意义（P〈0.05）。结论 新的胆管囊状扩张症分型基于囊状扩张病变累及胆管树的部位及其病理特征，对于不同分型的胆管囊状扩张症选择不同的治疗策略和手术方法具有明确的指导作用。
Objective To suggest a new clinical classification of the cystic dilatation of bile duct （CDBD）, and to design the treatment modality according to different classifications.Methods The clinical data of 434 patients with CDBD who were admitted to the Chinese PLA General Hospital from June 1993 to June 2010 were retrospectively analyzed. The data of computed tomography （CT） and magnetic resonance cholangiopancreatography （MRCP） were collected. A new clinical classification of CDBD was proposed based on the site, pathological features, pathogenic factors of CDBD and relevant surgical procedures.The clinical condition, surgical procedure, treatment efficacy and results of follow up of CDBD patients based on the new classification were analyzed, and then proper surgical treatment method for patients with different types of CDBD was suggested. The data were analyzed using the chisquare test, and Fisher exact probability was applied when the theoretical frequency〈5 or the observed frequency〈30.Results CDBD was classified into 5 types according to the pathological features and the location of cystic dilation in the biliary tree： （1）Type A： cystic dilatation of the peripheral biliary tree was limited to the intrahepatic bile ducts. This type could be subdivided into type A1 （cystic dilation of the bile duct was limited to one hepatic lobe or several segments） and type A2 （cystic dilation of the bile duct was diffused to entire intrahepatic biliary tree）. （2）Type B： cystic dilation of central large intrahepatic bile duct above the hilar convergence. Commonly the disease presented a single localized form limited to one hepatic lobe （type B1）, and cystic dilation in hilar convergence or in bilobar central bile ducts （type B2） was rare. （3）Type C： cystic dilation of the extrahepatic bile duct. According to whether the intrapancreatic bile duct was involved, this type could be divided into type C1 （without intrapancreatic bile duct involvement） and C2 （with intrapancreatic bile duct involvement）.（4）Type D： cystic dilation involving both the intra and extrahepatic bile ducts. According to the range of involvement, this type could be divided into type D1 （cystic dilation limited to one lobe） and D2 （cystic dilation expanded to bilobar bile ducts）.（5）Type E： Cystic dilation of the distal common bile duct. Of the 434 patients, there were 24 patients with type A CDBD （type A1： 17 patients, type A2： 7 patients）, 13 patients with type B （type B1： 10 patients, type B2： 3 patients）, 300 patients with type C （type C1： 56 patients, type C2： 244 patients）, 96 patients with type D （type D1： 17 patients, type D2： 79 patients）, and 1 patient with type E. In 24 patients with type A CDBD, 14 were found with congenital hepatic fibrosis and 16 with polycystic kidney disease. Of the 24 patients with type A CDBD, 17 patients with type A1 received partial hepatectomy, 3 patients with type A2 received liver transplantation, 1 patient with type A2 received puncture and drainage of cystic lesions, and 3 patients with type A2 received non surgical treatment. Thirteen patients were with type B CDBD, 12 received hepatectomy and 1 with cholangiocarcinoma received nonsurgical treatment. Of the 300 patients with type C CDBD, 286 received cystectomy and Roux-en-Y cholangiojejunostomy, and 14 received radical resection of cholangiocarcinoma. Of the 96 patients with type D CDBD, 35 received extrahepatic cystectomy＋lobectomy＋Roux-en-Y cholangiojejunostomy, 59 received extrahepatic cystectomy without hepatectomy, 1 with type D1 and 1 with type D2 received radical resection of cholangiocarcinoma. One patient with type E CDBD received endoscopic papillectomy. A total of 399 patients followed up for 1-15 years, and the mean followup time was 57 months. Thirty three patients were complicated with anastomotic stricture and （or） biliary stone. There were 24 patients complicated with cholangiocarcinoma, and 15 of whom died during the follow up. Forty six patients received re operation （re anastomosis of bile duct and jejunum,choledochoscopic stone removal, radical resection of cholangiocarcinoma,and et al） because of recurrent cholangitis, anastomotic stricture, biliary stone or tumor. Other patients were free of biliary symptoms or with accidental mild cholangitis and received symptomatic treatment. The rates of symptom remission, structure and （or） biliary stone and reoperation were 88.2%（30/34）, 8.8%（3/34） and 11.8%（4/34） in patients who received extrahepatic cystectomy＋hepatectomy, and 64.4%（38/59）, 28.8%（17/59） and 35.6%（21/59） in patients who received extrahepatic cystectomy, and with significant difference （P〈0.05）.Conclusion The new clinical classification of CDBD based on the location of cystic dilation in the biliary tree and its pathological features could help to make a suitable surgical plan for clinical treatment.
出处
《中华消化外科杂志》
CAS
CSCD
北大核心
2013年第5期370-377,共8页
Chinese Journal of Digestive Surgery
基金
国家科技部科技支撑计划项目（2012BAI06801）
中国博士后科研基金（20110491868）
