A case report of Hirschsprung's disease presenting as sigmoid volvulus and literature review, Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia

Abstract

Background: Sigmoid volvulus is an uncommon problem in children and adolescents, and is rarely considered a diagnosis in this group. A high index of suspicion is necessary to reach a diagnosis and avoid morbidity and mortality. Sigmoid volvulus is a rare complication of Hirschsprung's disease, which has been reported in neonates, children, and adults. Here we report a case of sigmoid volvulus accompanied by undiagnosed Hirschsprung's disease.

Case presentation: A 9 years old boy who presented with sudden onset of colicky abdominal pain of 4 h duration associated with gross abdominal distension and 2 episodes of non-bilious vomiting. A plain abdominal radiographs showed single hugely dilated bowel loops in the left lower quadrant with single air fluid level. Sigmoid volvulus was considered and rectal tube deflation was done and it was successful. Full thickness rectal biopsy was done and it was consistent with aganglionic megacolon. A primary trans-anal Soave endo-rectal pull through was done 3 weeks later, after biopsy result arrived, which yielded loss of symptoms and regular bowel movement.

Conclusions: Sigmoid volvulus should be considered in the differential for children presenting with acute onset of abdominal obstruction. It should be known that when its's diagnosed in children, it is often associated with Hirschsprung's disease. Therefore, a proper diagnostic and treatment algorithm should be followed in order not to miss associated HD and to give optimum care to such patients.

Keywords: Hirschsprung’s disease; Pediatrics; Sigmoid volvulus.

Fig. 1

PA plain abdominal x-ray showing single air fluid level with coffee bean appearance

Fig. 2

PA barium enema study showing dilated and redundant sigmoid colon without radiologic evidence of Hirschsprung’s disease

Fig. 3

a, b Pictures showing trans-anal Soave’s endo-rectal pull through

Fig. 4

Algorithm for the management of sigmoid volvulus in children and adolescents proposed by Salas et al. HD, Hirschsprung’s Disease

Fig. 5

Modified Salas’s et al. algorithm for the management of sigmoid volvulus in children and adolescents proposed by Abay et al. for low income countries. HD Hirschsprung’s disease. *Sigmoid resection and anastomosis should be done on semi-emergency basis as soon as rectal biopsy result arrives