Prenatal diagnosis of fetal midgut volvulus: a case description

Intestinal malrotation has an incidence of 1 per 6000 live births. The most serious consequence of malrotation is volvulus. Midgut volvulus is a rare condition in which the small bowel and proximal colon twist around the superior mesenteric artery, leading to a high-grade proximal bowel obstruction and vascular compromise of the intestine, thereby leading to infarction of the involved intestine. Midgut volvulus rarely occurs antenatally and is usually not lethal in utero. There are only 7 cases of intrauterine fetal demise caused by midgut volvulus reported in the literature. We report a case of intrauterine fetal demise at 38 weeks of gestation, due to cardiovascular failure and shock from midgut volvulus. Non-specific abnormalities, including ascites and dilated bowel, had been seen on the antenatal ultrasound from the 15th week of gestation. In addition to the volvulus, the fetus had intestinal atresia and arthrogryposis.

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.

This report describes a rare case of intrauterine midgut volvulus that presented at term. The pregnancy was uncomplicated. There were no signs of fetal distress or polyhydramnios, and the child was delivered vaginally. This patient had the unusual presentation in that at the time of delivery, the patient was distended and acidotic. She immediately required an extensive resection of gangrenous small bowel. Comparing this case to the 10 other cases of fetal intestinal volvulus that have been reported, it seems this child was particularly fortunate that the volvulus occurred at a point in gestation when she was mature enough to tolerate birth and surgery. This case is also the first demonstration that volvulus can present with abdominal distension in the immediate newborn period.