Idiopathic mid-aortic syndrome in children

Christine B. Sethna, Bernard S. Kaplan, Anne Marie Cahill, Omaida C. Velazquez, Kevin E.C. Meyers

Research output: Contribution to journalArticle

81 Scopus citations

Abstract

Mid-aortic syndrome (MAS) is an uncommon condition characterized by narrowing of the abdominal aorta and stenosis of its major branches. Our goal was to illustrate the presentation, diagnosis and management of six new cases of idiopathic MAS together with 96 cases of idiopathic MAS from the literature. The mean age of the 102 cases was 14.3 years (19 days to 49 years). Our patient who presented at 19 days of age is the youngest reported to date. Clinical presentations included hypertension (94%), claudication (17%), renal failure (4%) and intestinal ischemia (1%). Angiography was the diagnostic imaging study of choice. Renal arteries were involved in 91% of patients, while the superior mesenteric artery and celiac artery were involved in 35%. Thirteen percent of cases were managed medically, and the remainder was treated surgically. Our experience shows that initial conservative blood pressure management of idiopathic MAS is feasible unless medical control of hypertension is unsatisfactory, renal function is at risk or there are symptoms of claudication or intestinal ischemia. Careful timing and planning of a surgical intervention is possible for most cases and may, in select cases, be considered after completion of puberty to allow growth to be completed.

Original languageEnglish (US)
Pages (from-to)1135-1142
Number of pages8
JournalPediatric Nephrology
Volume23
Issue number7
DOIs
StatePublished - Jul 1 2008
Externally publishedYes

Keywords

  • Aortic diseases
  • Claudication
  • Hypertension
  • Pediatrics
  • Renovascular disease

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

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  • Cite this

    Sethna, C. B., Kaplan, B. S., Cahill, A. M., Velazquez, O. C., & Meyers, K. E. C. (2008). Idiopathic mid-aortic syndrome in children. Pediatric Nephrology, 23(7), 1135-1142. https://doi.org/10.1007/s00467-008-0767-4