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Case Reports
. 2023 Jul 5;17(1):308.
doi: 10.1186/s13256-023-03986-y.

Adult Hirschsprung's disease presenting as chronic constipation: a case report

Theresia Monica Rahardjo  1 , Yeppy Arief Nurzaman  2 , Janice Natalia  3 , Indra Hapdijaya  3 , Livia Devina  3 , Hendrik Andrianto  3 , Jeffrey Christian Mahardhika  2
Affiliations
Case Reports

Adult Hirschsprung's disease presenting as chronic constipation: a case report

Theresia Monica Rahardjo et al. J Med Case Rep. .

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.

Keywords: Adult; Case report; Congenital; Constipation; Hirschsprung’s disease.

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Conflict of interest statement

We declare that this case report has no conflicts of interests.

Figures

Fig. 1
Fig. 1
A Abdominal plain radiograph. This figure revealed that the air distribution in the colon and small intestine is increased with coiled spring appearance. There was neither stepladder appearance nor subdiaphragmatic free air. B–F Contrast enema study radiograph. The study showed that the sigmoid colon was dilated and the rectum was narrowed, with rectosigmoid index less than 1 and irregular rectosigmoid mucosa
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