Rare cause of upper bowel obstruction arising in a 17-year-old boy with cystic fibrosis: Superior mesenteric artery (Wilkie's) syndrome
Abstract
Superior mesenteric artery syndrome (SMAS) is extremely rare in childhood and adolescence, although uncommon, the complication of SMAS involving upper bowel obstruction is possible, even in cystic fibrosis (CF). To our knowledge, on other report of CF subjects developing SMAS has been mentioned in medical literature, advancing gastrointestinal complications for paediatric CF.
As delays in recognition may result in death or irreversible sequelae when signs and symptoms of upper gastrointestinal obstruction are present after rapid weight loss with no obvious etiology, clinicians should be aware of a high index of suspicion for SMAS in acutelly ill CF children, and consider the disease in the differential diagnosis of upper digestive obstruction in CF.