title: Disease Beyond the Arch: A Systematic Review of Middle Aortic Syndrome in Childhood.
abstract: BACKGROUND
  Middle aortic syndrome (MAS) is a rare clinical entity in childhood, characterized by a severe narrowing of the distal thoracic and/or abdominal aorta, and associated with significant morbidity and mortality. MAS remains a relatively poorly defined disease. This paper systematically reviews the current knowledge on MAS with respect to etiology, clinical impact, and therapeutic options.
  
  
  METHODS
  A systematic search of 3 databases (Embase, MEDLINE, and Cochrane Central Register of Controlled Trials) yielded 1,252 abstracts that were screened based on eligibility criteria resulting in 184 full-text articles with 630 reported cases of childhood MAS. Data extracted included patient characteristics, clinical presentation, vascular phenotype, management, and outcomes.
  
  
  RESULTS
  Most cases of MAS are idiopathic (64%), 15% are associated with Mendelian disorders, and 17% are related to inflammatory diseases. Extra-aortic involvement including renal (70%), superior mesenteric (30%), and celiac (22%) arteries is common, especially among those with associated Mendelian disorders. Inferior mesenteric artery involvement is almost never reported. The majority of cases (72%) undergo endovascular or surgical management with residual hypertension reported in 34% of cases, requiring medication or reintervention. Clinical manifestations and extent of extra-aortic involvement are lacking.
  
  
  CONCLUSIONS
  MAS presents with significant involvement of visceral arteries with over two thirds of cases having renal artery stenosis, and one third with superior mesenteric artery stenosis. The extent of disease is worse among those with genetic and inflammatory conditions. Further studies are needed to better understand etiology, long-term effectiveness of treatment, and to determine the optimal management of this potentially devastating condition.
authors: Institute of Medical Science and The Cardiovascular Sciences Collaborative Program, University of Toronto, Toronto, Ontario, Canada; Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada;
authors: Rawan K
authors: RK
authors: Rumman
authors: Hospital Library and Archives, The Hospital for Sick Children, Toronto, Ontario, Canada;
authors: Cheri
authors: C
authors: Nickel
authors: Department of Pediatrics, Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada; Faculty of Medicine, University of Toronto, Ontario, Canada;
authors: Mina
authors: M
authors: Matsuda-Abedini
authors: Faculty of Medicine, University of Toronto, Ontario, Canada; Department of Surgery, Division of Urology, The Hospital for Sick Children, Toronto, Ontario, Canada;
authors: Armando J
authors: AJ
authors: Lorenzo
authors: Department of Pediatrics, Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada; Faculty of Medicine, University of Toronto, Ontario, Canada;
authors: Valerie
authors: V
authors: Langlois
authors: Department of Pediatrics, Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada; Faculty of Medicine, University of Toronto, Ontario, Canada;
authors: Seetha
authors: S
authors: Radhakrishnan
authors: Faculty of Medicine, University of Toronto, Ontario, Canada; Division of Image Guided Therapy, Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, Ontario, Canada;
authors: Joao
authors: J
authors: Amaral
authors: Faculty of Medicine, University of Toronto, Ontario, Canada; Division of Cardiology, Labatt Family Heart Center, The Hospital for Sick Children, Toronto, Ontario, Canada;
authors: Luc
authors: L
authors: Mertens
authors: Department of Pediatrics, Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada; Faculty of Medicine, University of Toronto, Ontario, Canada; Department of Medicine, University Health Network, Toronto, Ontario, Canada. rulan.parekh@sickkids.ca.
authors: Rulan S
authors: RS
authors: Parekh
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001012:Aorta, Abdominal / Q000473:pathology / Q000503:physiopathology; D001013:Aorta, Thoracic / Q000473:pathology / Q000503:physiopathology; D001018:Aortic Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D062186:Arterial Pressure; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D018450:Disease Progression; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D006973:Hypertension / Q000209:etiology / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012307:Risk Factors; D013577:Syndrome
keywords: abdominal aorta; blood pressure; bypass; coarctation; endovascular; grafting; hypertension; middle aortic syndrome; renal artery stenosis.