Abstract
A 13-year-old boy presented with abdominal pain and vomiting. He had been followed up for Noonan syndrome and pulmonary valve stenosis. His abdomen was distended and bowel sounds were weak. Abdominal radiography and CT revealed an air-fluid level in the intestine. We diagnosed the patient with intestinal obstruction and inserted a long tube to treat him conservatively. His condition did not improve on observation for a few days, and emergency surgery was performed. A duplicate saccular intestine was found 60 cm proximal to the ileocecal valve, and it adhered to the small intestine and mesentery 210 cm distal from the Treitz ligament. This duplicate intestine was resected. Noonan syndrome is a congenital malformation disorder characterized by congestive heart disease and external anomalies. There is only 1 other report of a case of Noonan syndrome with digestive disorder. Therefore, we have reported this extremely rare case of Noonan syndrome with intestinal duplication.
