Intrauterine midgut volvulus without malrotation: diagnosis from the 'coffee bean sign'

Abstract

Fetal midgut volvulus is quite rare, and most cases are associated with abnormalities of intestinal rotation or fixation. We report a case of midgut volvulus without malrotation, associated with a meconium pellet, during the gestation period. This 2.79 kg, 33-wk infant was born via a spontaneous vaginal delivery caused by preterm labor. Prenatal ultrasound showed dilated bowel loops with the appearance of a 'coffee bean sign'. This patient had an unusual presentation with a distended abdomen showing skin discoloration. An emergency laparotomy revealed a midgut volvulus and a twisted small bowel, caused by complicated meconium ileus. Such nonspecific prenatal radiological signs and a low index of suspicion of a volvulus during gestation might delay appropriate surgical management and result in ischemic necrosis of the bowel. Preterm labor, specific prenatal sonographic findings (for example, the coffee bean sign) and bluish discoloration of the abdominal wall could suggest intrauterine midgut volvulus requiring prompt surgical intervention.

Figure 1

A fetal sonogram showing dilated bowel loops with the appearance of a ‘coffee bean sign’. No ascites was seen in the fetal abdomen.

Figure 2

A: Pre-operative infantogram showing a gas shadow only in the stomach, with an absence of any distal gas shadow; B: Unenhanced abdominal CT showed meconium (arrow) in the distal small bowel, with mild fluid distension of the proximal small bowel.

Figure 3

On laparotomy, the infant was found to have a midgut volvulus with necrosis and perforation of the small bowel. The small bowel was found to be twisted at the level of the narrow meconium-filled distal ileum (arrow).