[成人先天性胆道系统囊性疾病]

Târcoveanu E, Niculescu D, Georgescu St, Cotea Elena, Vintilă D, Dimofte G, Neacşu C N, Lupaşcu C, Crumpei Felicia, Cîrdei C

Clinica I Chirurgie, Facultatea de Medicină, Universitatea de Medicină şi Farmacie Gr.T. Popa Iaşi.

Rev Med Chir Soc Med Nat Iasi. 2003 Oct-Dec;107(4):817-21.

Congenital cystic disease of the biliary system is a complex syndrome of ectasies of the intra-, extra- or both situation of biliary tree. This disease has an unsure etiopathogeny. It is uncommon through the third age, with a greater incidence in child, teen-ager and young adult. The goal of our study is to evaluate the symptoms, diagnosis, treatment and histological aspects of the congenital biliary cysts. We performed a retrospective study from March 1988 to July 2003 about 11 patients with this disease treated in our surgical clinic. Clinical features, methods of diagnosis and surgical treatment were assessed. All patients were females with mean age 51 years (extreme 26-77 years). The symptoms were: right upper quadrant pain--11 cases (100%), jaundice--6 cases (54.5%), fever--3 cases (27%), palpable abdominal mass--2 cases (18%), weight loss--1 case (9%). The imaging diagnosis was helpful (ultrasonography, CT, ERCP, percutaneous cholangiography and preoperative cholangiography). In concordance with Todani classification the patients were included in the following types: Ia--3 cases (27%), I b--1 case (9%), I c--3 cases (27%), IV a--2 cases (18%), IV b--1 case (9%), V--1 case (9%). All patients were operated on: after cholecystectomy and transcystic cholangiography (11 cases--100%) we performed the total excision of the cyst--9 cases (82%) with choledochal jejunostomy (Roux-en-Y)--6 cases (54.5%), choledochal jejunostomy (omega)--1 case (9%) and choledochal duodenostomy--2 cases (18%). In one case of neoplasic cyst with portal invasion we performed a cyst-jejunostomy (omega) and in one case of Caroli disease with total obstruction of the distal choledoc, the solution of choice was choledocal-duodenostomy. The microscopic pathology of the cyst wall showed: chronic intramural inflammation--9 cases (82%) and the absence of the nervous intramural terminations--1 case (9%). The additional lesions was: hepatic cirrhosis--1 case (9%) and hepatic fibrosis--3 cases (27%). We found three cases with neoplasia: malign cyst with advanced local invasion--1 case (9%), pancreatic carcinoma--1 case (9%) and gallbladder carcinoma (microscopic finding)--1 case (9%). The postoperative morbidity includes biliary fistula--2 cases (18%) and wound infection--2 cases (18%). Long-term follow-up revealed cholangitis in one case--9%. The cystic dilatations of the common bile duct is an exclusive indication for surgery as soon as it was discovered.

先天性胆管系统囊性疾病是一种胆管树内、外或二者均出现扩张的复杂综合征。该疾病的病因尚不明确。在老年人中并不常见，在儿童、青少年和年轻成年人中发病率较高。我们研究的目的是评估先天性胆管囊肿的症状、诊断、治疗及组织学特征。我们对1988年3月至2003年7月在我们外科诊所接受治疗的11例该疾病患者进行了回顾性研究。评估了临床特征、诊断方法及手术治疗情况。所有患者均为女性，平均年龄51岁（范围26 - 77岁）。症状包括：右上腹疼痛——11例（100%），黄疸——6例（54.5%），发热——3例（27%），可触及腹部肿块——2例（18%），体重减轻——1例（9%）。影像学诊断有帮助（超声、CT、ERCP、经皮胆管造影及术前胆管造影）。根据Todani分类，患者被归入以下类型：Ia——3例（27%），Ib——1例（9%），Ic——3例（27%），IVa——2例（18%），IVb——1例（9%），V——1例（9%）。所有患者均接受了手术：在胆囊切除术和经胆囊胆管造影术后（11例——100%），我们进行了囊肿全切术——9例（82%），其中行胆总管空肠吻合术（Roux - en - Y）——6例（54.5%），胆总管空肠吻合术（omega）——1例（9%），胆总管十二指肠吻合术——2例（18%）。1例肿瘤性囊肿伴门静脉侵犯患者行囊肿空肠吻合术（omega），1例卡洛里病伴胆总管远端完全梗阻患者，首选的解决方法是胆总管十二指肠吻合术。囊肿壁的微观病理显示：慢性壁内炎症——9例（82%），壁内神经末梢缺失——1例（9%）。其他病变包括：肝硬化——1例（9%），肝纤维化——3例（27%）。我们发现3例肿瘤：恶性囊肿伴局部晚期侵犯——1例（9%），胰腺癌——1例（9%），胆囊癌（显微镜下发现）——1例（9%）。术后并发症包括胆瘘——2例（18%）和伤口感染——2例（18%）。长期随访发现1例胆管炎——9%。一旦发现胆总管囊性扩张，手术是唯一的治疗指征。