Hirschsprung disease in an adult with intestinal malrotation and volvulus: an exceptional association
| Autor: | Sixte Sondji, Emile Nini, Elise Lupon, François Labbe |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
Adult
Chronic occlusion medicine.medical_specialty Constipation medicine.medical_treatment Malrotation intestinal lcsh:Medicine Context (language use) Case Report 030204 cardiovascular system & hematology 03 medical and health sciences Ileostomy 0302 clinical medicine Multiple occlusive syndrome Intestine Small medicine Humans Adult Hirschsprung disease Hirschsprung Disease Mesentery Small bowel volvulus Colectomy business.industry lcsh:R Anastomosis Surgical General Medicine medicine.disease digestive system diseases Volvulus Surgery Bowel obstruction medicine.anatomical_structure Intestinal malrotation 030220 oncology & carcinogenesis Female medicine.symptom business Intestinal Obstruction Intestinal Volvulus |
| Zdroj: | Journal of Medical Case Reports Journal of Medical Case Reports, Vol 13, Iss 1, Pp 1-5 (2019) |
| ISSN: | 1752-1947 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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