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Adult Hirschsprung’s disease presenting as chronic constipation: a case report
BACKGROUND: Hirschsprung’s disease is a congenital disorder identified by the absence of ganglion cells at the Meissner’s plexus of the submucosa and Auerbach’s plexus of the muscularis. This disease can be found in approximately 1 in 5000 live births. It is a congenital disorder that is rarely diag...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10320872/ https://www.ncbi.nlm.nih.gov/pubmed/37403154 http://dx.doi.org/10.1186/s13256-023-03986-y |
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author | Rahardjo, Theresia Monica Nurzaman, Yeppy Arief Natalia, Janice Hapdijaya, Indra Devina, Livia Andrianto, Hendrik Mahardhika, Jeffrey Christian |
author_facet | Rahardjo, Theresia Monica Nurzaman, Yeppy Arief Natalia, Janice Hapdijaya, Indra Devina, Livia Andrianto, Hendrik Mahardhika, Jeffrey Christian |
author_sort | Rahardjo, Theresia Monica |
collection | PubMed |
description | BACKGROUND: Hirschsprung’s disease is a congenital disorder identified by the absence of ganglion cells at the Meissner’s plexus of the submucosa and Auerbach’s plexus of the muscularis. This disease can be found in approximately 1 in 5000 live births. It is a congenital disorder that is rarely diagnosed in adults, where 95% of cases are diagnosed in infants aged under 1 year old. Here we present a rare case of adult Hirschsprung’s disease to enrich the body of knowledge in diagnosing adult patients with chronic refractory constipation symptoms. CASE REPORT: An 18-year-old Indonesian woman came to the general surgery department of Unggul Karsa Medika Teaching Hospital with a defecating problem (constipation) since childhood. There was no history of her passage of meconium. A contrast enema study showed that the sigmoid colon was dilated and the rectum was narrowed, with rectosigmoid index < 1. With these findings, it was suspected that the patient may have ultra-short segment Hirschsprung’s disease. The patient was then referred to the digestive surgery department of referral hospital for surgical treatment. CONCLUSION: In adult patients presenting with history of constipation since childhood, it is necessary to consider the possibility of Hirschsprung’s disease that was not diagnosed in early childhood. Hirschsprung’s disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. Surgical removal of the aganglionic segment of the gut is the definitive treatment for Hirschsprung’s disease. |
format | Online Article Text |
id | pubmed-10320872 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-103208722023-07-06 Adult Hirschsprung’s disease presenting as chronic constipation: a case report Rahardjo, Theresia Monica Nurzaman, Yeppy Arief Natalia, Janice Hapdijaya, Indra Devina, Livia Andrianto, Hendrik Mahardhika, Jeffrey Christian J Med Case Rep Case Report BACKGROUND: Hirschsprung’s disease is a congenital disorder identified by the absence of ganglion cells at the Meissner’s plexus of the submucosa and Auerbach’s plexus of the muscularis. This disease can be found in approximately 1 in 5000 live births. It is a congenital disorder that is rarely diagnosed in adults, where 95% of cases are diagnosed in infants aged under 1 year old. Here we present a rare case of adult Hirschsprung’s disease to enrich the body of knowledge in diagnosing adult patients with chronic refractory constipation symptoms. CASE REPORT: An 18-year-old Indonesian woman came to the general surgery department of Unggul Karsa Medika Teaching Hospital with a defecating problem (constipation) since childhood. There was no history of her passage of meconium. A contrast enema study showed that the sigmoid colon was dilated and the rectum was narrowed, with rectosigmoid index < 1. With these findings, it was suspected that the patient may have ultra-short segment Hirschsprung’s disease. The patient was then referred to the digestive surgery department of referral hospital for surgical treatment. CONCLUSION: In adult patients presenting with history of constipation since childhood, it is necessary to consider the possibility of Hirschsprung’s disease that was not diagnosed in early childhood. Hirschsprung’s disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. Surgical removal of the aganglionic segment of the gut is the definitive treatment for Hirschsprung’s disease. BioMed Central 2023-07-05 /pmc/articles/PMC10320872/ /pubmed/37403154 http://dx.doi.org/10.1186/s13256-023-03986-y Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Rahardjo, Theresia Monica Nurzaman, Yeppy Arief Natalia, Janice Hapdijaya, Indra Devina, Livia Andrianto, Hendrik Mahardhika, Jeffrey Christian Adult Hirschsprung’s disease presenting as chronic constipation: a case report |
title | Adult Hirschsprung’s disease presenting as chronic constipation: a case report |
title_full | Adult Hirschsprung’s disease presenting as chronic constipation: a case report |
title_fullStr | Adult Hirschsprung’s disease presenting as chronic constipation: a case report |
title_full_unstemmed | Adult Hirschsprung’s disease presenting as chronic constipation: a case report |
title_short | Adult Hirschsprung’s disease presenting as chronic constipation: a case report |
title_sort | adult hirschsprung’s disease presenting as chronic constipation: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10320872/ https://www.ncbi.nlm.nih.gov/pubmed/37403154 http://dx.doi.org/10.1186/s13256-023-03986-y |
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