Bjørnland Kristin, Pakarinen Mikko P, Stenstrøm Pernilla, Stensrud Kjetil J, Neuvonen Malla, Granström Anna L, Graneli Christina, Pripp Are H, Arnbjörnsson Einar, Emblem Ragnhild, Wester Tomas, Rintala Risto J
Department of Paediatric Surgery, Oslo University Hospital-Rikshospitalet and University of Oslo, Oslo, Norway.
Department of Paediatric Surgery, Children's Hospital, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
J Pediatr Surg. 2017 Sep;52(9):1458-1464. doi: 10.1016/j.jpedsurg.2017.01.001. Epub 2017 Jan 5.
Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome.
Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records.
73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs.
A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II.
经肛门直肠内拖出术（ERPT）是治疗先天性巨结肠（HD）最常用的技术。然而，关于长期肠道功能的了解仍然有限。这项横断面多中心研究评估了大量HD患者的长期肠道功能，并探讨了预后不良的预测因素。
4岁以上患者或其父母填写了一份经过验证的肠道功能问卷。临床细节从病历中进行回顾性记录。
73/200（37%）患者报告肠道功能完全没有受损，即没有便秘、大便失禁、造口、阑尾造口术或灌肠需求。7例（4%）有造口，33例（17%）使用顺行或直肠结肠灌肠。大多数排便控制紊乱和便秘在年龄较大的组中明显较少见，但异常排便频率和社会问题保持不变。综合征患者（n = 31）比非综合征患者（14%，P<0.001）更频繁地出现大便失禁（46%）。患有综合征（校正比值比5.6，95%可信区间2.1 - 15，P = 0.001）或完全经肛门ERPT（校正比值比2.4，95%可信区间1.1 - 5.7，P = 0.038）与预后不良显著相关，预后不良定义为有造口、阑尾造口术、每日大便失禁或需要定期直肠冲洗。
相当数量的HD患者在确定性手术后多年仍存在肠道问题。HD患者中年龄较大者的排便控制明显优于年龄较小者，但一些成年患者仍有相当严重的肠道问题。完全经肛门ERPT与较差的预后相关。HD患者需要进行长期随访。预后研究：二级。
