Sigmoid Volvulus in a Neonate with Anorectal Malformation: A Case Report

Background: Sigmoid volvulus in the neonate is an extremely rare condition needing emergency treatment. Sporadic cases of neonatal sigmoid volvulus and some in association with Hirschsprung’s disease have been documented. Sigmoid volvulus has been described only twice in the literature in association with anorectal malformation. However, newborns with anorectal malformations might be especially at risk for sigmoid volvulus due to increased intraluminal intestinal weight caused by bowel obstruction and its existence might be underreported. Case presentation: This is the case report of a full-term neonate with trisomy 21 who was diagnosed with anorectal malformation upon birth. An abdominal X-ray on his second day of life showed typical radiological findings of sigmoid volvulus as described by the radiologist. However, the findings were interpreted as a dilated rectosigmoid sling typical for anorectal malformation by the surgeon and a transverse loop colostomy was placed. On the fourth postoperative day sigmoid perforation occurred and led to meconium peritonitis and septic shock. An emergency laparotomy was performed and a perforation at the distal sigmoid colon was found. Conclusion: There is an urgent need to raise awareness as to the existence of sigmoid volvulus in the neonate with anorectal malformations. The distinct radiological findings of the “coffee bean sign”, the “northern exposure sign” and an empty rectum allow to distinguish the extremely rare sigmoid volvulus from the common finding of a dilated rectosigmoid in patients with anorectal malformations. Detorsion of sigmoid volvulus is vital to preventing sigmoid perforation.