Stephanie Culnan, Alyssa Carlson, Meredith L Farmer
{"title":"Bridging Knowledge and Practice: Insights Into Hirschsprung's Disease and Home Management.","authors":"Stephanie Culnan, Alyssa Carlson, Meredith L Farmer","doi":"10.1891/NN-2024-0067","DOIUrl":null,"url":null,"abstract":"<p><p>Hirschsprung's disease (HD) is a congenital intestinal motility disorder characterized by the absence of ganglionic cells in the distal intestine, leading to functional bowel obstruction and affecting 1 in every 5,000 neonates. A serious complication of HD is Hirschsprung-associated enterocolitis (HAEC), a life-threatening systemic illness responsible for a significant proportion of HD-related mortality. Proper management of HD, especially preventing HAEC, includes the use of rectal irrigations, which caregivers must master before their neonate's discharge. However, barriers such as short NICU stays and lack of standardized parental education hinder successful home care. This article provides an overview of HD pathophysiology, clinical manifestations, diagnostics, and management, alongside a practical parent education tool for rectal irrigation techniques to enhance at-home care and reduce morbidity.</p>","PeriodicalId":46706,"journal":{"name":"Neonatal Network","volume":"44 3","pages":"167-176"},"PeriodicalIF":0.6000,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Neonatal Network","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1891/NN-2024-0067","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"NURSING","Score":null,"Total":0}
引用次数: 0
Abstract
Hirschsprung's disease (HD) is a congenital intestinal motility disorder characterized by the absence of ganglionic cells in the distal intestine, leading to functional bowel obstruction and affecting 1 in every 5,000 neonates. A serious complication of HD is Hirschsprung-associated enterocolitis (HAEC), a life-threatening systemic illness responsible for a significant proportion of HD-related mortality. Proper management of HD, especially preventing HAEC, includes the use of rectal irrigations, which caregivers must master before their neonate's discharge. However, barriers such as short NICU stays and lack of standardized parental education hinder successful home care. This article provides an overview of HD pathophysiology, clinical manifestations, diagnostics, and management, alongside a practical parent education tool for rectal irrigation techniques to enhance at-home care and reduce morbidity.