{"title":"Biopsy Diagnosis of Hirschsprung's Disease Using a Single Excisional Biopsy Based on the Anorectal Line.","authors":"Masahiro Takeda, Takanori Ochi, Hiroyuki Koga, Koichi Tsuboi, Atsushi Arakawa, Geoffrey J Lane, Atsuyuki Yamataka","doi":"10.1055/a-2065-9071","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong> A biopsy protocol for diagnosing Hirschsprung's disease (HD) in children using the anorectal line (ARL).</p><p><strong>Materials and methods: </strong> The ARL was adopted for diagnosing HD in 2016 using two excisional submucosal rectal biopsies performed at different levels, sequentially; the first just above the ARL and the second, further proximal (2-ARL). Currently, only the first-level biopsy is performed (1-ARL) and examined intraoperatively. Management was observation if normoganglionic, pull-through if aganglionic, and a second-level biopsy if hypoganglionic. Hypoganglionosis was considered physiologic if the second-level biopsy was normoganglionic and pathologic if hypoganglionic. Colon caliber change and bowel obstructive symptoms reflect the severity of hypoganglionosis.</p><p><strong>Results: </strong> For 2-ARL (<i>n</i> = 54), results were: normoganglionosis (<i>n</i> = 31/54; 57.4%), aganglionosis (<i>n</i> = 19/54; 35.2%), and hypoganglionosis (<i>n</i> = 4/54; 7.4%); physiologic (<i>n</i> = 3/54; 5.6%) and pathologic (<i>n</i> = 1/54; 1.9%). Normoganglionosis and aganglionosis were always duplicated in 2-ARL (kappa = 1.0). For 1-ARL (<i>n</i> = 36), results were: normoganglionosis (<i>n</i> = 17/36; 47.2%), aganglionosis (<i>n</i> = 17/36; 47.2%), and hypoganglionosis (<i>n</i> = 2/36; 5.6%). Second-level biopsies were normoganglionic (physiologic: <i>n</i> = 1) and hypoganglionic (pathologic: <i>n</i> = 1). All normoganglionic cases, except one, resolved conservatively. All aganglionic cases had pull-through with HD confirmed on histopathology. Both pathologic hypoganglionic cases had caliber change and severe obstructive symptoms as definitive indications for pull-through with hypoganglionosis of the entire rectum confirmed on histopathology. Physiologic hypoganglionic cases were observed and currently have regular defecation.</p><p><strong>Conclusion: </strong> Because the ARL is an objective functional, neurologic, and anatomic demarcation, normoganglionosis and aganglionosis can be diagnosed accurately with a single excisional biopsy. Only hypoganglionosis requires a second-level biopsy.</p>","PeriodicalId":56316,"journal":{"name":"European Journal of Pediatric Surgery","volume":null,"pages":null},"PeriodicalIF":1.5000,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Journal of Pediatric Surgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1055/a-2065-9071","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2023/3/30 0:00:00","PubModel":"Epub","JCR":"Q2","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0
Abstract
Introduction: A biopsy protocol for diagnosing Hirschsprung's disease (HD) in children using the anorectal line (ARL).
Materials and methods: The ARL was adopted for diagnosing HD in 2016 using two excisional submucosal rectal biopsies performed at different levels, sequentially; the first just above the ARL and the second, further proximal (2-ARL). Currently, only the first-level biopsy is performed (1-ARL) and examined intraoperatively. Management was observation if normoganglionic, pull-through if aganglionic, and a second-level biopsy if hypoganglionic. Hypoganglionosis was considered physiologic if the second-level biopsy was normoganglionic and pathologic if hypoganglionic. Colon caliber change and bowel obstructive symptoms reflect the severity of hypoganglionosis.
Results: For 2-ARL (n = 54), results were: normoganglionosis (n = 31/54; 57.4%), aganglionosis (n = 19/54; 35.2%), and hypoganglionosis (n = 4/54; 7.4%); physiologic (n = 3/54; 5.6%) and pathologic (n = 1/54; 1.9%). Normoganglionosis and aganglionosis were always duplicated in 2-ARL (kappa = 1.0). For 1-ARL (n = 36), results were: normoganglionosis (n = 17/36; 47.2%), aganglionosis (n = 17/36; 47.2%), and hypoganglionosis (n = 2/36; 5.6%). Second-level biopsies were normoganglionic (physiologic: n = 1) and hypoganglionic (pathologic: n = 1). All normoganglionic cases, except one, resolved conservatively. All aganglionic cases had pull-through with HD confirmed on histopathology. Both pathologic hypoganglionic cases had caliber change and severe obstructive symptoms as definitive indications for pull-through with hypoganglionosis of the entire rectum confirmed on histopathology. Physiologic hypoganglionic cases were observed and currently have regular defecation.
Conclusion: Because the ARL is an objective functional, neurologic, and anatomic demarcation, normoganglionosis and aganglionosis can be diagnosed accurately with a single excisional biopsy. Only hypoganglionosis requires a second-level biopsy.
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