Department of Pediatric Surgery, Kyushu University, Higashi-ku, Fukuoka 812-8582, Japan.
J Pediatr Surg. 2009 Dec;44(12):2312-5. doi: 10.1016/j.jpedsurg.2009.07.051.
Biliary atresia (BA) with hilar cyst is an uncommon variant, which constitutes less than 10% of all types of BA, and the operative procedure for this type of BA remains controversial.
We have had 200 cases with BA from 1963 to 2008 in our institute and our branch hospitals, in which 12 cases (6%) were BA with a hilar cyst. The clinical records of all cases were evaluated retrospectively.
Twelve BA patients with a hilar cyst included 2 boys and 10 girls. The diagnosis of BA was confirmed by intraoperative cholangiography (cloudy or treelike pattern). In all 12 cases, a hepaticojejunostomy was performed at a median age of 71.6 days (range, 24-136 days). The follow-up periods were 1.2 to 23.2 years. The current mean total bilirubin level was 0.8 mg/dL (range, 0.2-3.5 mg/dL), and the mean direct bilirubin level was 0.2 mg/dL (range, 0.0 to 0.8 mg/dL). Methylprednisolone or oral prednisolone was administered in 8 cases after operation, and 10 of 12 cases achieved a jaundice free state. The postoperative complications were cholangitis (n = 10), gastroesophageal varices (n = 7), splenomegaly (n = 3), ileus (n = 1), and pulmonary hypertension (n = 1). The overall survival rate with a native liver was 10 (83.3%) of 12 cases, of which 9 cases were jaundice-free and only 1 case showed recurrent jaundice. The other 2 cases underwent living-donor liver transplantation at age of 2 and 20 years, respectively, and they are currently doing well.
Most cases of BA with a hilar cyst achieved excellent clinical outcome after a hepaticojejunostomy.
胆管闭锁（BA）合并肝门部囊肿是一种罕见的变异，不到所有 BA 类型的 10%，这种类型的 BA 的手术方法仍存在争议。
我们的研究所及其分院自 1963 年至 2008 年共收治了 200 例 BA 患者，其中 12 例（6%）为 BA 合并肝门部囊肿。回顾性评估了所有病例的临床记录。
12 例 BA 合并肝门部囊肿患者中包括 2 名男性和 10 名女性。BA 的诊断通过术中胆管造影（云雾状或树状图案）得到证实。在所有 12 例患者中，中位年龄为 71.6 天（范围，24-136 天）时进行了肝肠吻合术。随访时间为 1.2 至 23.2 年。目前的总胆红素平均水平为 0.8mg/dL（范围，0.2-3.5mg/dL），直接胆红素平均水平为 0.2mg/dL（范围，0.0-0.8mg/dL）。术后 8 例患者给予甲基强的松龙或口服泼尼松龙治疗，12 例中有 10 例达到无黄疸状态。术后并发症包括胆管炎（n=10）、胃食管静脉曲张（n=7）、脾肿大（n=3）、肠梗阻（n=1）和肺动脉高压（n=1）。12 例患者中，10 例（83.3%）具有原生肝的总生存率，其中 9 例无黄疸，仅 1 例出现复发性黄疸。另外 2 例分别在 2 岁和 20 岁时接受了活体供肝移植，目前情况良好。
大多数 BA 合并肝门部囊肿患者在进行肝肠吻合术后取得了良好的临床结果。
