Left isomerism with normal heart and gut malrotation

Sameh Latif Abdel Latif Salam, M.Sc.1, Islam Badr, M.Sc.2.

1. Radiology department, Kasr Alainy teaching hospitals, Cairo University, Egypt.
2.Fetal medicine unit, Cairo university, Egypt.

A female patient, 27 years old (G2P1) with unremarkable medical history was referred to us for routine ultrasound. Our examinations at 20 and 27 weeks of gestation revealed the following findings:

  • Situs ambiguous
    • Interrupted hepatic and supra hepatic segments of IVC with azygos vein continuation.
    • Ambiguous distribtion of umbilical vein into portal sinus.
    • Direct drainage of hepatic veins into right atrium.
    • Right sided stomach with equal sized liver lobes.
  • Normal cardiac morphology with concordant atrio-ventricular and ventriculo-arterial connections with normal SVC and pulmonary venous connections into their respective atria.
  • Normal fetal heart rate and rhythm.
  • The renal segment of IVC is seen on the right side at mid abdomen level receiving both renal veins with left one coursing from left to right between aorta and mesenteric vessels.

Our prenatal diagnosis was left isomerism with normal heart and suspected gut malrotation based upon the appearance of reversed relationship between superior mesenteric artery being on the right to the left sided superior mesenteric vein, with most of bowel loops seen in the left lumbar region of the abdomen.

Postnatal upper GI series examination confirmed the prenatal diagnosis of gut malrotation and the fetus was refered to a pediatric surgeon for surgical correction.

Images 1-4: 27 weeks gestation study shows axial and sagittal views of normal heart with azygos continuation, interrupted IVC and direct insertion of hepatic veins into right atrium.

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Images 5-8: 27 weeks gestation study shows situs ambiguous with most of bowel loops in the left lumbar region and reversed SMA/SMV relationship suggesting gut malrotation.

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Videos 1 and 2 (20 weeks of gestation) and videos 3-8 (27 weeks of gestation): 2D images in different planes show normal heart with azygos continuation, interrupted IVC and direct insertion of hepatic veins into right atrium, situs ambiguous with most of bowel loops in the left lumbar region and reversed SMA/SMV relationship suggesting gut malrotation.

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