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Mid-aortic syndrome presenting in childhood

Y P Panayiotopoulos1, M R Tyrrell, G Koffman

  • 1Department of Surgery, Guy's Hospital, London, UK.

The British Journal of Surgery
|February 1, 1996
PubMed
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Mid-aortic syndrome (MAS) involves abdominal aorta narrowing, often causing hypertension. Treatment varies from medication to surgery, with varying success rates in pediatric cases.

Area of Science:

  • Vascular Surgery
  • Pediatric Cardiology
  • Nephrology

Background:

  • Mid-aortic syndrome (MAS) is a rare condition causing segmental narrowing of the abdominal aorta and its major branches.
  • While typically diagnosed in young adults, MAS can present as a complex pediatric challenge.

Purpose of the Study:

  • To review the clinical presentation, management, and outcomes of pediatric patients with mid-aortic syndrome.
  • To evaluate the effectiveness of different treatment modalities for MAS in children.

Main Methods:

  • Retrospective review of 13 pediatric patients diagnosed with MAS over 20 years.
  • Analysis of clinical data, including associated conditions (neurofibromatosis, eosinophilia, Williams syndrome), hypertension, and renal function.
  • Review of diagnostic arteriography findings and treatment strategies (antihypertensive medication, percutaneous transluminal angioplasty, surgical revascularization).

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Main Results:

  • All 13 patients presented with hypertension.
  • Six children were successfully managed with antihypertensive medication alone.
  • Seven children required surgical intervention for refractory hypertension and progressive renal impairment, utilizing various revascularization techniques.

Conclusions:

  • Mid-aortic syndrome in children presents with significant hypertension and potential renal compromise.
  • While medical management is effective in some cases, surgical revascularization is crucial for refractory disease.
  • A range of surgical techniques can be employed for successful renal revascularization in pediatric MAS.