Midgut volvulus in a child with left isomerism: A case report

Left isomerism, also known as polysplenia syndrome, is a rare condition characterized by abnormal lateralization of organs during embryonic development. It is associated with multiple anatomical abnormalities, including mirror image arrangement of thoracic and abdominal organs, disrupted vascular patterns, and increased susceptibility to cardiac and extra-cardiac anomalies. An 8-year-old previously healthy girl presented to the emergency department with recurrent vomiting and general fatigue. Two days prior, she fell while playing and sustained a direct abdominal impact upon hitting the ground. Following the injury, she developed diffuse abdominal pain and frequent episodes of non-bloody, non-bilious vomiting. Over the next 48 hours, her vomiting became progressively projectile and green. She visited a pediatric clinic twice, where she received intravenous fluids and antiemetics, with only minimal symptomatic improvement. On arrival at the ED, she appeared dehydrated, with borderline blood pressure and persistent diffuse abdominal pain. There were no known chronic medical conditions and no family history of gastrointestinal disorders, On physical exam, patient was ill-appearing, fatigued, and mildly lethargic, with dry mucous membranes, and delayed capillary refill. On abdominal examination, there was diffuse tenderness, predominantly in the epigastric and periumbilical regions along with mild abdominal distension with guarding but no rebound tenderness and decreased bowel sounds. An ultrasound was done and was inadequate. A CT abdomen with contrast revealed a left isomerism along with midgut volvulus. Finally, an urgent exploratory laparotomy was done with an uneventful recovery. Although rare, midgut volvulus should be ruled out in patients with left isomerism who develop abdominal pain.