Enterocolitis and bowel function in children with Hirschsprung disease and trisomy 21

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• 作者：Natasha Kwendakwema^a ; Ragheed Al-Dulaimi^c ; Angela P. Presson^c ; Sarah Zobell^b ; Austin M. Stevens^a ; Brian T. Bucher^a ; ^b ; Douglas C. Barnhart^a ; ^b ; Michael D. Rollins^a ; ^b ; ^{Michael.Rollins@imail2.org}
• 关键词：Hirschsprung disease ; Trisomy 21 ; Enterocolitis ; HAEC
• 刊名：Journal of Pediatric Surgery
• 出版年：2016
• 出版时间：December 2016
• 年：2016
• 卷：51
• 期：12
• 页码：2001-2004
• 全文大小：257 K
• 卷排序：51

文摘

The purpose of this study was to study the effect of trisomy 21 (T21) on enterocolitis rates and bowel function among children with Hirschsprung disease (HD).MethodsA retrospective cohort study of patients with HD treated at our tertiary children's hospital (2000–2015) and a cohort of patients with HD treated in our pediatric colorectal center (CRC) (2011–2015) were performed.Results26/207 (13%) patients with HD had T21. 70 (41%) with HD alone were diagnosed with enterocolitis episodes compared to 9 (38%) with HD + T21 (p = 0.71). 55/207 patients were managed in the CRC. 11/55 patients (20%) had HD + T21. 25 (58%) with HD had one or more enterocolitis episodes compared to 4 (36%) with HD + T21 (p = 0.20). Number of hospitalizations for enterocolitis was similar between all groups. Toilet training was assessed in 32 CRC patients (25 HD, 7 HD + T21). One child with HD + T21 was toilet trained by age 4 years versus 12 with HD (p = 0.20). Laxative or enema therapy was required for constipation management in 57% HD versus 64% HD + T21.ConclusionEnterocolitis rates in children with HD + T21 did not differ from rates in children with HD alone. The majority of patients with CRC follow-up had constipation requiring laxative or enema therapy, which demonstrates the need for consistent postoperative follow-up.Level of Evidence: Retrospective Study – Level II.