小儿先天性胆管扩张症的诊治:附44例报告

Diagnosis and treatment of congenital biliary dilatation in children:a report of 44 cases

背景与目的:先天性胆管扩张症(CBD)是小儿较常见的胆道畸形,可发生于肝内和肝外胆管的任何部位。随着时间的推移,患者易并发胆道结石、胰腺炎、胆管癌、复发性胆管炎、门静脉高压症、自发性囊肿破裂等严重并发症。CBD常伴有胰胆管合流异常(PBM),且临床症状不典型,部分急性发作患者腹腔粘连严重,手术难度大且术后并发症多,因此其诊断与治疗对小儿外科医生带来了巨大挑战。本研究总结分析小儿CBD的诊治经验,以期为临床工作提供参考。方法:回顾性分析中南大学湘雅医院2010年6月-2017年8月间收治的44例CBD患儿的临床资料。结果:44例患儿中女38例,男6例,男女比1:6.3;发病年龄为2~161个月,中位发病年龄为63个月。主要临床症状为腹痛30例(68.1%)、皮肤巩膜黄染20例(45.5%)、恶心呕吐7例(15.9%)。37例行彩超检查,30例(81.1%)考虑为CBD;32例行CT检查,29例(90.6%)考虑诊断为CBD;20例行磁共振胰胆管成像(MRCP)检查,20例(100.0%)均考虑诊断为CBD,其中18例(90.0%)伴有PBM。按Todani分型:I型34例(77.3%),IVA型10例(32.7%);按董氏分型:C1型26例(59.1%),C2型8例(18.2%),D1型8例(18.2%),D2型2例(4.5%)。一期行胆囊切除+胆总管囊肿切除+肝总管空肠Roux-en-Y吻合术的29例患儿术中出血(80.0±25.0)mL,无并发症发生,术后住院(8.0±1.6)d;行胆囊切除+胆总管囊肿切除+左半肝切除+右肝管空肠Roux-en-Y吻合术的1例术中出血150.0 mL,术后住院10 d;一期行胆总管囊肿切开T管引流术,3个月后行二期胆总管囊肿切除+肝总管空肠Roux-en-Y吻合术的6例患儿术中出血(500.0±125.0)mL,术后住院(11.0±4.2)d;1例行胆总管空肠端侧吻合术患儿术中出血200.0 mL,术后出现吻合口瘘,术后住院24 d,6个月后行二期手术,术中失血200.0 mL,术后住院7 d;7例未行手术治疗,其中4例为IVA型。40例获随访20~110个月(中位随访时间60个月),其中35例行手术治疗患儿均恢复良好,5例未行手术治疗患儿中3例(60.0%)症状反复发作,1例因反复发作胆管炎死亡。结论:MRCP诊断CBD的准确率高,且能显示有无PBM及PBM类型,术中可根据MRCP显示的胰胆管汇合部位而避免损伤胰管并完全切除病变胆管,可作为诊断CBD的首选方法;董氏分型有助于手术方式的选择,为部分IVA型患者提供合理的手术方式。

Background and Aims:Congenital biliary dilatation (CBD) is a relatively common biliary malformation in children,which can occur in any part of both intrahepatic and extrahepatic bile ducts.The patients are prone to develop serious complications such as biliary stones,pancreatitis and cholangiocarcinoma,recurrent cholangitis,portal hypertension,spontaneous cyst rupture with the passage of time.CBD is often associated with pancreaticobiliary maljunction (PBM),and lacks of typical clinical symptoms.Severe abdominal adhesions are found in some patients with acute attack,for whom,the operation is difficult to perform and many postoperative complications may develop.Thus,the diagnosis and treatment of this condition pose a great challenge to pediatric surgeons.This study was to summarize the experiences in diagnosis and treatment of pediatric CBD,so as to provide relevant strategies for clinical work.Methods:The clinical data of 44 children with CBD are admitted in Xiangya Hospital of Central South University from June 2010 to August 2017 were retrospectively analyzed.Results:Among the 44 cases,there were 38 females and 6 males,with a male-female ratio of 1:6.3.The onset age ranged from 2 to 161 months,and the median onset age was 63 months.The main clinical symptoms were abdominal pain in 30 cases (68.1%),skin scleral jaundice in 20 cases (45.5%) and nausea and vomiting in 7 cases (15.9%).Ultrasound examination was performed in 37 patients,of whom,30 cases (81.1%) were considered as CBD;32 patients underwent CT scan,and 29 cases (90.6%) of them were considered as CBD;20 patients were subjected to MRCP examination,and all of them (100.0%) were considered as CBD,among whom,18 cases (90.0%) had concomitant PBM.According to Todani classification,34 patients (77.3%) were classified as type I and 10 cases (32.7%) were classified as type IVA;according to Dong’s classification,there were26 cases (59.1%) with type C1,8 cases (59.1%) with type C2,8 cases (59.1%) with type D1,and 2 cases (4.5%) with type D2 diseases.In 29 pediatric patients who underwent primary cholecystectomy and choledochal cyst resection plus Roux-en-Y choledochojejunostomy,the intraoperative bleeding was (80.0±25.0) mL,and no complications occurred,and the length of postoperative hospital stay was (8.0±1.6) d;in one child who received cholecystectomy,choledochal cyst resection and left hemihepatic resection plus right hepaticojejunal Roux-en-Y anastomosis,the intraoperative blood loss was 150.0 mL,and the postoperative hospitalization was 10 d;in 6 cases undergoing primary choledochal cyst incision plus T-tube drainage,and secondary choledochal cyst resection plus Roux-en-Y choledochojejunostomy 3 months later,the intraoperative bleeding was (500.0±125.0) mL and the length of postoperative hospitalization was (11.0±4.2) d;in one case underwent choledochojejunostomy,the amount of intraoperative bleeding was 200.0 mL,anastomotic fistula occurred after operation,hospitalized for24 d postoperatively,and the second operation was performed 6 months later,with intraoperative blood loss of 200.0 mL and postoperative hospitalization of 7 d;7 children (15.9%) did not receive surgical treatment,of whom,4 cases had type IVA disease.Forty children were followed up for 20 to 110 months (median follow-up time was 60 months),35 cases receiving surgical treatment were all recovered well,and 3 cases develop repeated recurrence of symptoms and one case died due to recurrent cholangitis in the 5 cases who did not receive surgical treatment.Conclusion:MRCP has a high accuracy for diagnosis of CBD.It can distinguish the presence or absence of the PBM and the PBM type,and the damage of pancreatic duct can be avoided and diseased bile duct can be completely removed the intraoperative according to the display of the confluence of pancreaticobiliary ducts by MRCP during operation,so MRCP can be used as the first choice for CBD diagnosis.The Dong’s classification is helpful for procedure selection,and provides a proper surgical method for some type IVA patients.