Hirschsprung disease presenting as sigmoid volvulus: a case report and review of the literature

Sigmoid Volvulus in Children: Diagnosis and Therapeutic Challenge

“…In pediatrics, it is most often associated with congenital anomalies of the fixation of the colon (a redundant sigmoid colon with a short mesenteric axis), a longstanding history of constipation, or Hirschsprung disease [1,[3][4][5][6]. Different studies found an incidence of Hirschsprung disease in up to 18% of patients with sigmoid volvulus [4,6]. There are also reports that identified roundworm infestation [6,7], malrotation [1], and neurologic disorders [2,6] as predisposing factors.…”

“…Nevertheless, this diagnosis can be challenging, especially in children. In adults, abdominal radiographs identify the omega or coffee bean sign in more than 60% of cases, but in children this has only a 17-30% success rate [1,2,4]. The most common findings in radiographs of children are colonic distention or an air-fluid level in sigmoid loops [1,8].…”

“…Proponents of enema reduction advocate that it is a diagnostic (identification of the "bird's beak" sign) and therapeutic procedure that simultaneously facilitates bowel preparation for surgery, being successful in up to 77% of cases [1-3, 6, 7]. Nevertheless, it is believed by some to have a higher complication rate (perforation due to bowel ischemia), and sometimes more than one attempt is necessary [1,4]. As for endoscopic reduction, previous reports have stated a lower success rate in children [1,2,7], but a recent review of the literature report- ed a success rate of 69% and no associated complications, also having the advantage that it allows for inspection of the bowel mucosa and avoiding exposure to radiation [6].…”

“…For stable patients submitted to successful nonsurgical reduction, posterior elective sigmoidectomy is the gold standard, due to the high risk of recurrence (more than 65%). This is even more important in this age group, because of the patients' longer average life expectancy [1,[4][5][6]. It should be done as a semi-elective procedure during the same period of hospital stay, normally after at least 24-48 h, due to the high early recurrence rates [1,6].…”

Sigmoid volvulus in a neonate: Case report and review of literature

“…[6] The aetiology of SV is different in children than in adults with constipation being the most common cause in the elderly. [7] Although the aetiology in children is not completely understood, the hypothesis is the presence of a congenital elongation of the sigmoid colon with a pathological long colonic mesentery in association with a narrow base or lack of fixation of a part of the colon. [8] Constipation can be considered as a cause with progressive colonic redundancy or a result.…”