Adult Hirschsprung’s disease presenting as chronic constipation: a case report
Abstract 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 rar...
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| Format: | Article |
| Language: | English |
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BMC
2023-07-01
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| Series: | Journal of Medical Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13256-023-03986-y |
| _version_ | 1797784607802785792 |
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| author | Theresia Monica Rahardjo Yeppy Arief Nurzaman Janice Natalia Indra Hapdijaya Livia Devina Hendrik Andrianto Jeffrey Christian Mahardhika |
| author_facet | Theresia Monica Rahardjo Yeppy Arief Nurzaman Janice Natalia Indra Hapdijaya Livia Devina Hendrik Andrianto Jeffrey Christian Mahardhika |
| author_sort | Theresia Monica Rahardjo |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-03-13T00:42:19Z |
| format | Article |
| id | doaj.art-1feebbcaadfc4586abd5df365e218998 |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-03-13T00:42:19Z |
| publishDate | 2023-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-1feebbcaadfc4586abd5df365e2189982023-07-09T11:14:52ZengBMCJournal of Medical Case Reports1752-19472023-07-011711510.1186/s13256-023-03986-yAdult Hirschsprung’s disease presenting as chronic constipation: a case reportTheresia Monica Rahardjo0Yeppy Arief Nurzaman1Janice Natalia2Indra Hapdijaya3Livia Devina4Hendrik Andrianto5Jeffrey Christian Mahardhika6Faculty of Medicine, Maranatha Christian UniversityUnggul Karsa Medika Teaching HospitalFaculty of Medicine, Maranatha Christian UniversityFaculty of Medicine, Maranatha Christian UniversityFaculty of Medicine, Maranatha Christian UniversityFaculty of Medicine, Maranatha Christian UniversityUnggul Karsa Medika Teaching HospitalAbstract 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.https://doi.org/10.1186/s13256-023-03986-yHirschsprung’s diseaseConstipationCongenitalAdultCase report |
| spellingShingle | Theresia Monica Rahardjo Yeppy Arief Nurzaman Janice Natalia Indra Hapdijaya Livia Devina Hendrik Andrianto Jeffrey Christian Mahardhika Adult Hirschsprung’s disease presenting as chronic constipation: a case report Journal of Medical Case Reports Hirschsprung’s disease Constipation Congenital Adult 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 | Hirschsprung’s disease Constipation Congenital Adult Case report |
| url | https://doi.org/10.1186/s13256-023-03986-y |
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