title: Successful endovascular treatment of a ruptured superior mesenteric artery in a patient with Ehlers‒Danlos syndrome.
abstract: The purpose of this study was to describe covered-stent treatment of a ruptured dissection of the superior mesenteric artery (SMA) in a patient with Ehlers‒Danlos syndrome. The patient was a 13-year-old girl initially presenting with abdominal pain. Dissection and rupture of the SMA were diagnosed on detailed examination. Conservative treatment was performed initially because open surgery was considered high risk. However, the abdominal pain recurred, and we decided to perform endovascular therapy. A coronary artery covered stent was placed in the true lumen to close the entry site of the dissection. The false lumen was obliterated using a post-dilation technique, completing treatment of the rupture. The patient recovered uneventfully after surgery. Classic-type Ehlers‒Danlos syndrome was diagnosed on the basis of physical findings and genetic analysis. The stent has remained adequately patent as of 2 years after surgery. This case report shows that dissection and rupture of the SMA can be treated successfully using a covered coronary artery stent in a patient with Ehlers‒Danlos syndrome.
authors.affiliation: Cardiovascular Center, Yokohama City University Medical Center, Yokohama, Japan. shorty@mtj.biglobe.ne.jp
authors.forename: Shota
authors.initials: S
authors.lastname: Yasuda
authors.forename: Kiyotaka
authors.initials: K
authors.lastname: Imoto
authors.forename: Keiji
authors.initials: K
authors.lastname: Uchida
authors.forename: Daisuke
authors.initials: D
authors.lastname: Machida
authors.forename: Hiromasa
authors.initials: H
authors.lastname: Yanagi
authors.forename: Tadahisa
authors.initials: T
authors.lastname: Sugiura
authors.forename: Kenji
authors.initials: K
authors.lastname: Kurosawa
authors.forename: Munetaka
authors.initials: M
authors.lastname: Masuda
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000293:Adolescent; D000784:Aortic Dissection / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D017542:Aneurysm, Ruptured / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000295:instrumentation; D024062:Collagen Type V / Q000235:genetics; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D057510:Endovascular Procedures / Q000295:instrumentation; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D017538:Mesenteric Artery, Superior / Q000000981:diagnostic imaging / Q000601:surgery*; D009154:Mutation; D010641:Phenotype; D011474:Prosthesis Design; D015607:Stents; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: C530725:COL5A1 protein, human; D024062:Collagen Type V