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Case Reports
. 2019 Apr 29;13(1):124.
doi: 10.1186/s13256-019-2020-0.

Hirschsprung disease in an adult with intestinal malrotation and volvulus: an exceptional association

Elise Lupon  1 , François Labbe  2 , Emile Nini  2 , Sixte Sondji  2
Affiliations
Case Reports

Hirschsprung disease in an adult with intestinal malrotation and volvulus: an exceptional association

Elise Lupon et al. J Med Case Rep. .

Abstract

Background: Hirschsprung disease is a neonatal discovery in almost all cases, and the association of Hirschsprung disease in adults with symptomatic intestinal malrotation is unusual. This combination delays diagnosis and can lead to mistake in surgical strategy.

Case presentation: A 43-year-old patient with a history of colectomy for colonic inertia and megadolichocolon was admitted to the Carcassonne Hospital emergency room for a volvulus of small bowel obstruction in a chronic intestinal obstruction context with episodes of acute, variable-looking occlusive syndromes. Intestinal malrotation was discovered during surgical small bowel detorsion. The acute occlusion syndrome recurred after the procedure. In view of the unfavorable evolution, an emptying of the dilated small bowel and a discharge ileostomy upstream of the rectum were performed. In the face of postoperative improvement, rectal manometry and deep full parietal rectal biopsies made it possible to highlight the diagnosis of Hirschsprung disease. The patient thus had functional acute occlusive syndromes and chronic occlusion due to Hirschsprung disease of attenuated form and acute organic occlusive syndromes related to her incomplete common mesentery.

Conclusions: This rare association, which may be responsible for delayed diagnostic and therapeutic wandering, highlights the importance of performing manometry and deep full parietal biopsies before a colectomy for colonic inertia, as well as the possibility of suggesting a common Hirschsprung disease and/or mesentery in an adult with multiple occlusive syndromes of variable appearance.

Keywords: Adult Hirschsprung disease; Chronic occlusion; Constipation; Malrotation intestinal; Multiple occlusive syndrome; Small bowel volvulus.

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Conflict of interest statement

Ethics approval and consent to participate

The patient gave us her ethical approval.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
Abdominal axial cross-sectional computed tomography showing hydroaeric small intestine distention with pneumatosis of the small intestine loops
Fig. 2
Fig. 2
Volvulus of the small intestine with torsion of the mesentery and major dilation of the small intestine
Fig. 3
Fig. 3
Highlighting of a mesentery detached from the posterior wall and very elongated after manual detorsion
See this image and copyright information in PMC

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