Renal Artery Stenosis
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Narrowing of 1 or both renal arteries and/or their more distal branches, resulting in decreased perfusion, increased renin release, increased vascular resistance, and systemic hypertension
- Hypertension in infants and young children is often secondary to some identifiable cause. Of those with secondary hypertension, most have intrinsic renal disease (e.g., renal scarring, dysplasia, chronic nephritis).
- Up to 5% of adults with hypertension have renal artery stenosis (RAS).
- RAS accounts for ~10% of secondary hypertension in children. Its importance clinically is not its frequency but its potential curability.
- Any condition associated with thromboembolic events (such as a complication of an umbilical artery catheter in newborns)
- Renal trauma including renal artery surgery (e.g., transplantation)
- Extrinsic compression of the renal artery (e.g., Wilms tumor, neuroblastoma, or pheochromocytoma).
Reduce risk factors, such as thromboembolic events, which can lead to renal artery narrowing.
Arterial narrowing leads to diminished perfusion of the affected kidney, leading to signals in the juxtaglomerular apparatus, which lead to renin release and results in increased vascular resistance and blood pressure (BP).
- Majority are caused by fibromuscular dysplasia (FMD), a noninflammatory vascular disease of unknown etiology.
- Primarily affects females and affects up to 4 in 100 adults
- The renal vasculature is the most common arterial bed affected.
- FMD can concurrently affect other vascular beds including carotid, vertebral, and intracranial vascular beds.
- Arterial narrowing by atheroma is common in adults but rare in children.
Commonly Associated Conditions
- RAS may occur in many other conditions, including congenital anomalies (e.g., renal artery hypoplasia), neurocutaneous disorders (neurofibromatosis [type 1], tuberous sclerosis), vasculitis (Wegener, polyarteritis nodosa, Kawasaki disease, Takayasu arteritis, moyamoya disease), syndromes (Williams, Marfan, Alagille), and infections (e.g., congenital rubella and fungal infection [immunocompromised hosts]).
- Nephrotic syndrome may accompany renal artery stenosis and is probably secondary to it.
- RAS has been associated with multicystic dysplasia in the contralateral kidney.