SPR Unknown #36 -- FINAL

Nick Kennedy

Loyola University Medical Center

Martha Saker, MD

Children's Memorial Hospital

Abstract

11 year old male with incidental abdominal bruit on physical exam.

Keywords

1612 Middle aortic syndrome Abdominal aortic coarctation


Publication Date: 2010-04-16

History

11 year old male with incidental abdominal bruit on physical exam.

Findings

CT angiography demonstrates severe narrowing of the abdominal aorta beginning at the level of the renal arteries. In addition, there is severe proximal left renal artery stenosis. There is a prominent collateral vessel connecting the inferior mesenteric artery and the superior mesenteric artery (Arc of Riolan). The abdominal aorta returns to normal caliber just cephalad to its bifurcation. There was also moderate stenosis of the superior mesenteric artery.

DDx

Etiologies of Middle Aortic Syndrome:

1. Congenital

2. Acquired:

  • William’s Syndrome

Discussion

First described in 1963 by Dr. Sen, middle aortic syndrome may be congenital or secondary to other disease processes such as Neurofibromatosis or Takayasu’s Arteritis. There is a high incidence of concomitant renal or visceral arterial stenoses, thus complicating the surgical correction. Patients may present with renovascular hypertension, discrepant upper and lower extremity blood pressures, and lower extremity claudication. The complications of renovascular hypertension result in death by age 40 if left untreated.

CT or MR angiography are able to demonstrate the degree and length of the aortic narrowing. The greater spatial resolution of CT often better depicts associated visceral and renal arterial stenoses as well as clearly delineates the extent of collateral vessels.

The mainstay of treatment is open surgical aortic bypass graft. It is preferred to re-implant the renal arteries if there are significant stenoses identified on presurgical imaging as the renovascular hypertension is the real cause of morbidity and mortality in these patients. The younger the patient is, the more difficult the surgical planning becomes in order to attempt to place a graft long enough to allow for the child’s growth. Endovascular balloon angioplasty may be considered as a temporizing measure or in a minimally symptomatic older child or adult, but is not considered a durable therapy.

References

  1. Sen PK, Kinare SG, Engineer SD, et al. The middle aortic syndrome. British Heart Journal 1963; 25: 610-618
  2. Delis KT and Gloviczki P. Middle aortic syndrome: From presentation to contemporary open surgical and endovascular treatment. Perspectives in Vascular Surgery and Endovascular Therapy 2005; 17: 187-203.
  3. Sumboonnanonda A, Robinson BL, Gedroyc WM, et al. Middle aortic syndrome: Clinical and radiological findings. Arch Dis Child 1992; 67: 501-505.

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