title: [Thirty-eight cases of dysplasia of the superior mesenteric artery].
abstract: Dysplasia of the renal and cervical arteries are well known, but dysplasia of the superior mesenteric artery (SMA) is less frequent and has specific presentation. There have been few reports on the different types of presentations. We report a series of 38 cases and present the characteristic features together with a comparison with data in the literature. These non-atheromatous non-inflammatory lesions of the arterial wall occurred either in a context of fibromuscular disease which cause is unknown (30/38 patients) or in patients with genetic disease such as neurofibromatosis (3/38) or Ehlers-Danlos disease (5/38). The fibromuscular disease presented three aspects with specific characteristics. a) stenosing lesions found predominantly in women with a different morphology than in the other localization (usually associated): irregular diffuse stenosis discovered in patients with hypertension, or ischemic digestive symptoms (6/14 patients). b) Aneurysms, also found predominantly in women, but less frequently associated with other dysplasias. These aneurysms were generally sacciform. Unlike data in the literature, our series only included rupture in 1/8 cases. The aneurysm was discovered during the exploration of abdominal pain or hypertension. c) The third type of fibromuscular disease concerned dissections which were observed in the male population, (except one case of segmental dysplasia), and presented with signs of digestive ischemia in the other seven patients, four in an emergency context requiring immediate treatment. Two genetic diseases were observed. a) Neurofibromatosis led to dysplasia analogous to fibromuscular disease, but with abnormal nerve formations in the wall. For the three cases observed, one involved only the visceral arteries and the two others thoraco-abdominal coarctation. b) We had five patients with Ehlers-Danlos syndrome, with dysplasia of the superior mesenteric artery. For three there was a fusiform dilatation and in one small aneurysms along the arterial trunk, which only required surveillance. There was only one aneurysm which required treatment by resection and venous graft. In these young patients, revascularization was generally achieved with autologous material and the prognosis was favorable. Embolization was successful in treating the sacciform aneurysm in one patient. In conclusion, dysplasia of the superior mesenteric artery has a specific presentation which must be recognized to enable diagnosis of this not uncommon condition in young subjects (even children) who often present with an acute abdomen requiring urgent treatment.
authors.affiliation: Clinique G. Bizet, 23 rue Georges Bizet, 75 116 Paris.
authors.forename: F
authors.initials: F
authors.lastname: Cormier
authors.forename: J-M
authors.initials: JM
authors.lastname: Cormier
mesh_terms: D000328:Adult; D000368:Aged; D000792:Angiography; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D017538:Mesenteric Artery, Superior / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery*; D008875:Middle Aged; D017253:Neurofibromatoses / Q000150:complications; D014652:Vascular Diseases / Q000000981:diagnostic imaging / Q000473:pathology*