Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown. A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonates <29days; infants 29 days–12 months; toddler 1 year–5 years and child >5 years). 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P<0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P<0.001). Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through.

中文翻译：

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先天性巨结肠的延迟诊断是否会影响术后和功能结果？儿科结直肠和盆腔学习联盟的多中心审查

先天性巨结肠 (HD) 是儿童功能性肠梗阻的罕见原因。患者通常在新生儿期被诊断出来，并在诊断后不久接受拉通（PT）治疗。延迟就诊的儿童的最佳治疗和术后结果尚不清楚。在参与的儿科结直肠和骨盆学习联盟站点对 HD 儿童进行了多中心回顾性审查。儿童按诊断时的年龄进行分层（新生儿 <29 天；婴儿 29 天至 12 个月；幼儿 1 岁至 5 岁；儿童 > 5 岁）。纳入来自 14 个地点的 679 名 HD 患者；大多数（69%）在新生儿期被诊断出来。诊断时的年龄与 30 天并发症发生率或 PT 修改需要的差异无关。诊断时年龄越大，PT 后接受粪便改道的可能性越大（新生儿 10%，婴儿 12%，幼儿 26%，儿童 28%，P<0.001），并且术后更需要对便秘或失禁进行干预（新生儿56%，婴儿 62%，幼儿 78%，儿童 69%，P<0.001）。 HD 的延迟诊断不会影响术后 30 天的结果或修复手术的需要，但延迟诊断与拉出后粪便改道的需求增加相关。