Colorectal Center, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH, USA.
Department of Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, USA.
J Pediatr Surg. 2024 Jul;59(7):1250-1255. doi: 10.1016/j.jpedsurg.2024.03.034. Epub 2024 Mar 18.
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 <29 days; 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.
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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，并在诊断后不久接受经肛门拖出术（PT）。延迟就诊的患儿的最佳治疗和术后结局尚不清楚。
对参与小儿结直肠和骨盆学习联盟（Pediatric Colorectal and Pelvic Learning Consortium）的各中心的 HD 患儿进行了多中心回顾性研究。根据诊断时的年龄（新生儿<29 天；婴儿 29 天-12 个月；幼儿 1 岁-5 岁；儿童>5 岁）对患儿进行分层。
共有来自 14 个中心的 679 例 HD 患儿纳入研究，其中大多数（69%）在新生儿期被诊断。诊断时的年龄与 30 天并发症发生率或 PT 修正的需求无关。诊断年龄越大，PT 后行粪便转流的可能性越大（新生儿 10%，婴儿 12%，幼儿 26%，儿童 28%，P<0.001），术后因便秘或尿失禁需要干预的可能性也越大（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 天的结局或修正手术的需求，但延迟诊断与 PT 后粪便转流的需求增加有关。
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