Abo-Namous Reem, Kuebler Joachim F, Potthoff Andrej, Madadi-Sanjani Omid, Uecker Marie, Dingemann Jens, Petersen Claus, Ure Benno, Schukfeh Nagoud
Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany.
Department of Gastroenterology, Hannover Medical School, Hannover, Germany.
Innov Surg Sci. 2024 Jun 14;9(2):93-98. doi: 10.1515/iss-2023-0061. eCollection 2024 Jun.
Choledochal malformation (CM) is a rare disease that can lead to malignancy and potential long-term sequelae despite surgical resection. There is no long-term follow-up data on patients after CM resection in Germany. We aimed to determine the long-term outcome of our patients with a duration of follow-up >10 years and focused on long-term sequelae and health-related quality of life (HRQOL).
All patients who had undergone CM-resection in our department from 01/1978 to 06/2009 were contacted. Patients were interviewed about postoperative complications and their present medical attendance. HRQOL was determined using Pediatric Quality of Life Inventory 4.0 (PedsQL), version for adults. The PedsQL scales the HRQOL from 0 to 100, with higher scores indicating a better HRQOL. Scores were compared to those published for a healthy population.
Out of 56 patients who were contacted, 23 (41 %) participated. The median age at time of surgery was 3.1 years (6 days-16.1 years) and at time of the survey 24.3 years (11.1-53.8 years). Eighteen patients (78 %) had ceased their gastroenterologic follow-up at a median time of 4.3 years after surgery. Five (22 %) were still in gastroenterologic follow-up, two of these had an uneventful clinical course, and three (13 %) had ongoing complications attributed to the CM. One of these had undergone hemihepatectomy 34 years postoperatively due to bile duct stenosis, one had undergone removal of bile duct stones 14 years postoperatively, and one suffered from portal vein thrombosis with esophageal and jejunal varices. There was no mortality in our series. Median total HRQOL score was 89. There was no significant difference in the median total health, physical health, and psychosocial health scores of our patients in comparison to the healthy population.
We confirmed that the majority of patients after CM resection are lost to follow-up. Those who answered our questionnaire showed a good HRQOL. Given the high rate of severe long-term complications and the life-long risk of malignancy, we recommend a transition program for all patients.
胆总管畸形（CM）是一种罕见疾病，即便进行手术切除仍可能导致恶性肿瘤及潜在的长期后遗症。德国尚无CM切除术后患者的长期随访数据。我们旨在确定随访时间超过10年的患者的长期预后，并关注长期后遗症及健康相关生活质量（HRQOL）。
联系了1978年1月至2009年6月期间在我科接受CM切除术的所有患者。就术后并发症及目前的就医情况对患者进行了访谈。使用成人版的儿童生活质量量表4.0（PedsQL）来确定HRQOL。PedsQL将HRQOL按0至100进行评分，分数越高表明HRQOL越好。将分数与针对健康人群公布的分数进行比较。
在联系的56名患者中，23名（41%）参与了研究。手术时的中位年龄为3.1岁（6天至16.1岁），调查时的中位年龄为24.3岁（11.1至53.8岁）。18名患者（78%）在术后中位时间4.3年时停止了胃肠病学随访。5名（22%）仍在接受胃肠病学随访，其中2名临床过程顺利，3名（13%）有与CM相关的持续并发症。其中1名因胆管狭窄在术后34年接受了半肝切除术，1名在术后14年接受了胆管结石清除术，1名患有门静脉血栓形成伴食管和空肠静脉曲张。我们的研究系列中无死亡病例。HRQOL总评分中位数为89。与健康人群相比，我们患者的总体健康、身体健康和心理社会健康评分中位数无显著差异。
我们证实，CM切除术后的大多数患者失去了随访。回答我们问卷的患者显示出良好的HRQOL。鉴于严重长期并发症的高发生率及恶性肿瘤的终身风险，我们建议为所有患者制定一个过渡计划。
