关键词: Caroli disease HPB choledochal cyst liver surgery

来  源:   DOI:10.1089/lap.2021.0068

Abstract:
Background: Biliary tree cysts (BTCs) represent an either localized or multifocal abnormal dilatation of the biliary tree, which entails an increased risk of acute cholangitis and cholangiocarcinoma (2.5%-16%). Its incidence in Western countries is ∼1/100.000-1/150.000, being more frequent in Asia (1/1.000). These cysts are usually classified according to Todani classification, which is based on site and morphology of cysts. Patients and Methods: This is a retrospective multicentric descriptive study of patients surgically treated for BTCs. From 2005 to 2018, 25 cases were collected between Hospital de la Santa Creu i Sant Pau (Barcelona, Spain) and Ospedale San Paolo (Savona, Italy). Clinical presentation was characterized by abdominal pain, jaundice, fever, and sometimes weight loss. Eight patients presented Todani type I, 1 patient Todani type II, 3 patients Todani III, 1 patient Todani type IV, and 12 patients Todani type V. Results: Among the 25 surgically treated patients, 12 patients underwent liver resection, 7 patients underwent resection of the extrahepatic biliary tree, 3 patients underwent BTC removal through a duodenotomy, 1 patient underwent resection of the extrahepatic biliary tree and liver resection, and 2 patients underwent pancreatoduodenectomy. Overall 30-day morbidity rate was 20%, and 90-day mortality was 0%. Pathologic examination confirmed diagnosis of cholangiocarcinoma in 5 patients (20%). After a median follow-up of 59 months, 20 patients are alive and in good conditions, whereas 50% of patients with cholangiocarcinoma died for disease progression. Conclusions: Surgical treatment for BTCs is associated with acceptable postoperative outcomes, with moderate morbidity and null mortality rates. Moreover, the risk of developing cholangiocarcinoma is still high that prompts surgical treatment once diagnosis is made.
摘要:
背景:胆道囊肿(BTC)代表胆道树的局部或多灶性异常扩张,这意味着急性胆管炎和胆管癌的风险增加(2.5%-16%)。其在西方国家的发病率为〜1/100.000-1/150.000,在亚洲更为常见(1/1.000)。这些囊肿通常根据Todani分类法进行分类,这是基于囊肿的部位和形态。患者和方法:这是一项针对接受BTC手术治疗的患者的回顾性多中心描述性研究。从2005年到2018年,在圣塔克里乌医院(巴塞罗那,西班牙)和OspedaleSanPaolo(萨沃纳,意大利)。临床表现以腹痛为特征,黄疸,发烧,有时会减肥。八名患者表现为托达尼I型,1名患者TodaniII型,3名患者TodaniIII,1名患者TodaniIV型,结果:在25例经手术治疗的患者中,12例患者接受了肝切除术,7例患者接受肝外胆管树切除术,3例患者通过十二指肠切开术进行了BTC切除,1例患者接受肝外胆管树切除术和肝切除术,2例患者行胰十二指肠切除术。总体30天发病率为20%,90天死亡率为0%。病理检查证实胆管癌5例(20%)。在中位随访59个月后,20名病人还活着,身体状况良好,而50%的胆管癌患者因疾病进展而死亡。结论:BTC的手术治疗与可接受的术后结局相关,具有中等发病率和零死亡率。此外,发生胆管癌的风险仍然很高,一旦确诊,就需要进行手术治疗。
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