Department of Pediatric Surgery, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Gastroenterology and Metabolism Research Institute and Amsterdam Reproduction and Development Research Institute, Meibergdreef 9, Amsterdam 1105 AZ, the Netherland; Department of Pediatrics, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Emma Children's Hospital Amsterdam UMC Follow-Me program and Emma Neuroscience Group, Amsterdam Reproduction and Development Research Institute, Amsterdam, the Netherland.
Department of Pediatric Surgery, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam and Vrije Universiteit Amsterdam, Amsterdam Gastroenterology and Metabolism Research Institute and Amsterdam Reproduction and Development Research Institute, Meibergdreef 9, Amsterdam 1105 AZ, the Netherland.
J Pediatr Surg. 2022 Nov;57(11):561-568. doi: 10.1016/j.jpedsurg.2022.02.022. Epub 2022 Mar 2.
Patients with Hirschsprung disease (HD) mostly undergo surgery around the age of three to six months. While awaiting surgery, therapy to treat the obstruction such as transanal irrigation (TAI) or laxatives is applied. The aim of this study was to gain insight in the prevalence and severity of complications occurring while awaiting surgery and to identify patient characteristics associated with the development of these complications.
This study retrospectively analyzed data of patients with HD operated in our center between 2000 and 2021. Complications emerging while awaiting surgery were graded using Clavien-Dindo (CD). Patient characteristics as predictor of a complication were tested using logistic regression analysis.
Twenty-two of 132 (17%) included patients (preoperative treatment: 94% TAI; 2% laxatives; 2% other therapy) developed 45 complications while awaiting surgery, including predominantly major complications (91%). Bowel perforation occurred most frequently (n = 9, 7%) wherefrom six caused by TAI (5%), including three patients with total colon aganglionosis (TCA) (2%) counting one life-threatening and one lethal perforation. The other perforations were caused by meconium ileus (n = 2) and Hirschsprung associated enterocolitis (HAEC) (n = 1). Other frequent complications were: sepsis (5%), ileus (4%) and persistent obstruction (4%). Predictive factor for developing complication was TCA (OR 9.905, CI 2.994-32.772, p < 0.001).
We found a complication rate of 17% in patients while awaiting surgery, reporting bowel perforation most frequently. We found this complication in patients with TCA being highly dangerous causing one life-threatening and one lethal perforation. Therefore, we advise in patients with (suspected) TCA to limit the time awaiting surgery.
level III.
大多数先天性巨结肠（HD）患儿在 3 至 6 个月大时接受手术治疗。在等待手术期间，会应用经肛门灌洗（TAI）或通便剂等治疗梗阻的方法。本研究旨在深入了解手术前发生的并发症的发生率和严重程度，并确定与这些并发症发生相关的患者特征。
本研究回顾性分析了 2000 年至 2021 年在我院接受手术的 HD 患儿的数据。使用 Clavien-Dindo（CD）分级评估手术前发生的并发症。使用逻辑回归分析测试患者特征与并发症发生的相关性。
在 132 例纳入患者中（术前治疗：94% TAI；2%通便剂；2%其他治疗），有 22 例（17%）在等待手术期间出现 45 种并发症，主要为严重并发症（91%）。肠穿孔最为常见（n=9，7%），其中 6 例由 TAI 引起（5%），包括 3 例全结肠无神经节细胞症（TCA）（2%），其中 1 例危及生命，1 例致命穿孔。其他穿孔由胎粪性肠梗阻（n=2）和巨结肠相关性结肠炎（HAEC）（n=1）引起。其他常见的并发症有：败血症（5%）、肠梗阻（4%）和持续性梗阻（4%）。发生并发症的预测因素是 TCA（OR 9.905，CI 2.994-32.772，p<0.001）。
我们发现手术前等待手术的患者中有 17%发生并发症，其中肠穿孔最为常见。我们发现 TCA 患者的这种并发症非常危险，导致 1 例危及生命，1 例致命穿孔。因此，我们建议在（疑似）TCA 患者中限制手术前等待的时间。
III 级
