Pediatric Nephrology

, Volume 24, Issue 11, pp 2225–2232

Mid-aortic syndrome: long-term outcome of 36 children

  • Albina Tummolo
  • Stephen D. Marks
  • Marike Stadermann
  • Derek J. Roebuck
  • Clare A. McLaren
  • George Hamilton
  • Michael J. Dillon
  • Kjell Tullus
Original Article

DOI: 10.1007/s00467-009-1242-6

Cite this article as:
Tummolo, A., Marks, S.D., Stadermann, M. et al. Pediatr Nephrol (2009) 24: 2225. doi:10.1007/s00467-009-1242-6

Abstract

The clinical characteristics and outcomes of children with mid-aortic syndrome (MAS) and the effectiveness of different therapeutic approaches in reducing hypertension are still debated. We conducted a single-centre retrospective review of the records of children with MAS over 30 years. Children with angiographic evidence of a narrowed abdominal aorta were included. Therapeutic approaches included medical management, percutaneous transluminal angioplasty and/or surgical intervention. Thirty-six children had presented at a median age of 2.7 years (10 days–10 years). Thirteen (36%) patients had associated syndromes, and 44% had been diagnosed with cerebrovascular disease. All patients had involvement of multiple arteries. The mortality rate was 8% after a median follow-up period of 4.5 (range 1.1–19.7) years. Among the children who survived, 90% had obtained a reduction in their blood pressure (BP). Of the patients, 76% had had a normal estimated glomerular filtration rate (eGFR) at the last follow-up examination. Seventeen percent (six of 36) had renal dysfunction at presentation. Although MAS is a severe and widespread disease, in most cases it can be effectively treated with a combination of medical, angioplasty and surgical interventions.

Keywords

Renovascular hypertensionRenal artery stenosisCerebrovascular diseasePercutaneous transluminal angioplastySurgical intervention

Abbreviations

MAS

mid-aortic syndrome

BP

blood pressure

eGFR

estimated glomerular filtration rate

HTN

hypertension

NF-1

neurofibromatosis type 1

PTA

percutaneous transluminal angioplasty

DMSA

dimercaptosuccinic acid

RVH

renovascular hypertension

CVA

cerebrovascular accident

CKD

chronic kidney disease

Copyright information

© IPNA 2009

Authors and Affiliations

  • Albina Tummolo
    • 1
  • Stephen D. Marks
    • 2
  • Marike Stadermann
    • 2
  • Derek J. Roebuck
    • 3
  • Clare A. McLaren
    • 3
  • George Hamilton
    • 4
  • Michael J. Dillon
    • 2
  • Kjell Tullus
    • 2
  1. 1.Department of Paediatric NephrologyUniversity of BariBariItaly
  2. 2.Department of Paediatric NephrologyGreat Ormond Street Hospital for Children NHS TrustLondonUK
  3. 3.Department of Paediatric RadiologyGreat Ormond Street Hospital for ChildrenLondonUK
  4. 4.Department of SurgeryRoyal Free HospitalLondonUK