Congenital Midgut Volvulus Associated with Fetal Anemia

Abstract

Congenital volvulus is a life-threatening condition, both for the fetus and for the newborn. A volvulus is a twist of small bowel loops or a proximal part of the colon around the mesenteric artery or its branches. The potential consequences of volvulus are ileus and necrosis of the intestinal wall. Prenatal diagnosis of midgut volvulus is difficult. It should be suspected antenatally when polyhydramnios, intestinal dilatation, ascites and/or signs of fetal anemia are present on ultrasound assessment. We report a case of a congenital midgut volvulus associated with fetal anemia. The fetal ultrasound performed at 32 weeks’ gestation showed a polyhydramnios, hydrothorax, thick ascites accumulation around the liver and the suspicion of a dilated bowel loop. Additionally, Doppler examination showed an increased value of peak systolic velocity in the middle cerebral artery. Cordocentesis confirmed significant fetal anemia. At 34 weeks, because of the suspicion of idiopathic meconium ileus and secondary anemia, a Cesarean section was performed after the administration of steroids. During the laparatomy, performed postnatally, a midgut volvulus was diagnosed. The affected portion of the ileum was resected and end-to-end anastomosis performed. An antenatal diagnosis of midgut volvulus should be considered when signs of fetal anemia, including an increased value of peak systolic velocity in the middle cerebral artery, are present with polyhydramnios, fetal ascites, dilated bowel loops on antenatal ultrasound. An assessment of the fetal hemodynamic status should be a part of the ultrasound assessment for patients with nonspecific fetal bowel pathologies, including congenital volvulus.

Ascites, anemia and (intestinal) atresia

“…Although our patient did not have direct evidence of midgut volvulus on either antenatal ultrasound such as the 'whirlpool' or 'coffee bean' sign 4,6 or at laparotomy, she showed indirect signs of congenital volvulus on ultrasound 6 including intestinal dilatation and ascites. Both the barium study and laparotomy findings of a low-lying duodenojejunal flexure as well as the finding of a torsed proximal ileal segment at laparotomy suggested the presence of malrotation, increasing the likelihood of intrauterine midgut volvulus with consequent intestinal atresia.…”

“…The other two cases required emergency preterm delivery and prolonged hospitalization. [4][5][6] Early treatment in our patient with intrauterine blood transfusion may have altered the natural history of her condition, allowing the pregnancy to progress to term. Given the increasing frequency with which fetal ascites and fetal anemia are being observed in association with intestinal obstruction and atresia, it is important for obstetricians and neonatologists to have an index of suspicion for this constellation of abnormalities.…”

“…Kornacki reported a case of midgut volvulus associated with fetal hydrops and anemia. 6 Ultrasound at 32 weeks of gestation showed fetal hydrops (ascites and hydrothorax) with polyhydramnios and a dilated bowel loop. There was Doppler evidence of increased MCA peak systolic velocity (1.73 times the median) with significant anemia confirmed on cordocentesis (hematocrit 24%).…”

Prenatal diagnosis of antenatal midgut volvulus: Specific ultrasound features

“…On the other hand, this sign is never present in intestinal atresia without volvulus. When midgut volvulus occurs in utero , it can be complicated by ischemic necrosis of the loops because of its associated vascular effects …”

What is your diagnosis?

“…When the middle cerebral artery Doppler indicates anemic fetus or in utero paracentesis shows hemorrhagic ascites emergency in utero transfusion may be required (15). In the overlooked cases, when fetus escapes the sudden insult and the condition becomes relatively chronic, hemorrhagic volvulus may result in fetal hydrops (15,18). For the previously mentioned benefits and indications of in utero intervention, paracentesis may also be performed safely in the immediate intrapartum period as an EXIT procedure as in the current case.…”