SPR unknown # 57-- FINAL

Cynthia Tan M.D.

Loyola Univerisity Medical Center

ctan@lumc.edu

Cynthia Rigsby MD

Children's

Keywords

Renal artery aneurysm neurofibromatosis 1692 SPR unknown 57


Publication Date: 2011-03-04

History

6 year old with hypertension

Findings

Renal angiogram shows two saccular and one fusiform aneurysms with mild narrowing between the main renal artery and the first aneurysm. Saccular aneurysms have narrow necks. A prominent capsular artery courses over the outer aspect of the kidney and communicates with a capsular artery in the midpole.

Doppler US shows dampened renal arterial flow with delayed systolic upstroke at the hilum and intrarenal foci on each side.

Diagnosis

Renal artery aneurysms with proximal stenosis related to Neurofibromatosis

DDx

Polyarteritis Nodosa, Fibromuscular Dysplasia, Neurofibromatosis-1 (arterial pathology most common in renal artery, presents with HTN), Kawasaki, Ehler-Danlos syndrome, Atherosclerosis (often fusiform), Trauma, Renal Angiomyolipoma, Dilated renal vein

Discussion

Aneurysms are caused by fibromuscular dysplasia, collagen disorders, atherosclerosis or sepsis. RAAs have been associated with NF1 and Kawasaki disease. Majority are saccular, while 20% are fusiform. Majority of patients are asymptomatic, however hypertension may be associated. Pheochromocytomas are also associated with NF1 and may also a source of hypertension. Higher risk of rupture in pregnant females or if aneurysm is >2 cm. Aneurysms commonly occur at bifurcation of main renal artery.

If diameter >1.5 cm, repair by endovascular coil or stent grafts is considered for narrow neck aneurysms not located at branch points. Repair cures hypertension in 20-50% of cases.

References

  1. Caffey's Pediatric Diagnostic Imaging. Tenth Edition, Vol 2, page 1828.
  2. Renal artery stenosis and aneurysms associated wtih neurofibromatosis. Myo H. Journal of Vascular Surgery. Vol 41 issue 3.
  3. Renal artery aneurysm, hypertension and neurofibromatosis. Sharma AK. The Royal society of medicine. Vol 84. June 1991.

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