Department of Pediatric Surgery, Pediatric Liver and Gut Research Group, Pediatric Research Center.
HUS Medical Imaging Center, Children's Hospital.
J Pediatr Gastroenterol Nutr. 2021 Jun 1;72(6):820-825. doi: 10.1097/MPG.0000000000003054.
The aim of the study was to assess long-term morbidity in children operated for choledochal malformation (CM) by relating clinical complications to liver histopathology, follow-up imaging, liver stiffness, and biochemistry.
A single-center retrospective follow-up study including all CM patients (n = 55, 71% girls) treated during 1976 to 2018 was performed. Mann-Whitney U test and Spearman rank correlation were used for statistical analyses.
During median follow-up of 5.8 (interquartile range, 2.5-12) years, 1 patient was lost to follow-up whereas all survived. Intraoperative liver biopsies showed fibrosis in 32%, and patients with Metavir stage ≥2 were younger at surgery (0.36 [0.11-1.9] vs 3.8 [0.72-10.5] years, P = 0.024) than those without fibrosis. Overall, 21% had long-term complications including cholangitis in 9 (>2 episodes in 5) patients, anastomotic stricture in 2 referred patients and adhesive volvulus or hepatocellular carcinoma in 1 each. Anastomotic strictures were successfully managed nonoperatively and hepatocellular carcinoma with thermoablation. In postoperative magnetic resonance cholangiography (MRCP) performed 6.4 (3.6-16) years after hepaticojejunostomy, diameters of both main intrahepatic ducts had decreased significantly to 3.0 (2.5-3.5) mm (P = 0.0001) but a distal cyst stump was remaining in 30% with a length of 6.0 (4.0-20) mm that associated with operation age (r = 0.71, P = 0.015) and fusiform CM type. Follow-up ultrasound revealed mild dilation of intrahepatic bile ducts in 6.3% and mildly to moderately elevated liver biochemistry in 23%, and liver stiffness (>7 kPa) in 22%.
Whilst cholangitis was the most common postoperative problem, individual patients experienced other more significant complications and one quarter of patients showed evidence of underlying liver dysfunction.
本研究旨在通过将临床并发症与肝组织病理学、随访影像学、肝硬度和生物化学相关联，评估胆总管畸形（CM）患儿的长期发病率。
进行了一项单中心回顾性随访研究，纳入了 1976 年至 2018 年期间接受治疗的所有 CM 患者（n=55，71%为女性）。采用 Mann-Whitney U 检验和 Spearman 秩相关分析进行统计学分析。
在中位数为 5.8 年（四分位距，2.5-12）的随访期间，1 例患者失访，所有患者均存活。术中肝活检显示 32%存在纤维化，纤维化患者的手术年龄更小（0.36 [0.11-1.9]岁 vs 3.8 [0.72-10.5]岁，P=0.024）。总体而言，21%的患者出现长期并发症，包括 9 例（5 例有超过 2 次发作）患者的胆管炎、2 例转诊患者的吻合口狭窄以及各 1 例粘连性扭转或肝细胞癌。吻合口狭窄经非手术治疗和热消融治疗成功管理，肝细胞癌经热消融治疗。在胆肠吻合术后 6.4 年（3.6-16 年）进行的术后磁共振胰胆管造影（MRCP）中，肝内主要胆管的直径均显著下降至 3.0（2.5-3.5）mm（P=0.0001），但 30%的患者仍存在远端囊肿残端，长度为 6.0（4.0-20）mm，与手术年龄相关（r=0.71，P=0.015），且为梭形 CM 型。超声随访显示 6.3%的患者肝内胆管轻度扩张，23%的患者肝功能生化指标轻度至中度升高，22%的患者肝硬度（>7kPa）升高。
尽管胆管炎是最常见的术后问题，但个别患者还经历了其他更严重的并发症，四分之一的患者存在潜在的肝功能障碍证据。
