title: Familial superior mesenteric artery syndrome.
abstract: Superior mesenteric artery syndrome (SMAS) has an incidence of 0.1-0.3%. It is caused by a narrow angle between the aorta and the superior mesenteric artery, resulting in the clamping of the third portion of the duodenum. It is more common in females (2:1), with no racial differences. It is also more frequent in patients under 30 years and may have a congenital or acquired aetiology. There is only one case of the familial form described in the medical literature. We present a case of a 20-year-old woman, with recurrent episodes of epigastric discomfort and vomiting since she was 10 years old. After clinical suspicion, diagnosis was achieved after digestive endoscopy, upper gastrointestinal transit and abdominal MR. The patient was kept under parenteral nutrition for 17 days before surgery. She was discharged 8 days after surgery and the postoperative course was uneventful.
authors.affiliation: Department of Surgery, Hospital do Espirito Santo de Evora EPE, Evora, Portugal.
authors.forename: Ana Raquel
authors.initials: AR
authors.lastname: Martins
authors.affiliation: Fundacao Champalimaud, Lisboa, Portugal.
authors.forename: José Filipe
authors.initials: JF
authors.lastname: Cunha
authors.affiliation: Hospital do Espirito Santo de Evora EPE, Evora, Portugal.
authors.forename: Joana
authors.initials: J
authors.lastname: Patrício
authors.affiliation: Hospital do Espirito Santo de Evora EPE, Evora, Portugal.
authors.forename: Jorge
authors.initials: J
authors.lastname: Caravana
mesh_terms: D000713:Anastomosis, Roux-en-Y; D003937:Diagnosis, Differential; D016099:Endoscopy, Gastrointestinal / Q000379:methods*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D064847:Multimodal Imaging; D013478:Superior Mesenteric Artery Syndrome / Q000175:diagnosis* / Q000601:surgery; D016896:Treatment Outcome; D055815:Young Adult