Superior Mesenteric Artery Syndrome in Down Syndrome: A Case Report

Down syndrome is a common chromosomal abnormality, which is associated with a wide spectrum of multiorgan complications, including gastrointestinal disorders. Superior mesenteric artery syndrome is a rare type of small intestinal obstruction caused by compression of the duodenum between the superior mesenteric artery anteriorly and the abdominal aorta posteriorly. A 12-year-old boy with Down syndrome came to the emergency department with a first seizure secondary to electrolyte disturbances. He had been having recurrent episodes of bilious vomiting. Computed tomography of the abdomen revealed a markedly distended stomach and proximal duodenum with a narrow aortomesenteric angle consistent with superior mesenteric artery syndrome. The patient received successful conservative treatment with aggressive fluid resuscitation, bowel decompression, and electrolyte correction. Child protection services found that the patient had rapid weight loss recently after parents were confronted with the confirmed diagnosis of Down syndrome in their child. Physicians need to consider superior mesenteric artery syndrome in any child with an intellectual disability presenting with a clinical picture of intestinal obstruction.