Abstract

Objective: Renovascular hypertension is an important underdiagnosed secondary cause of resistant hypertension in young. Renal vascular atherosclerosis (90%) and renal fibromuscular dysplasia (FMD) (10%) are two main etiologies. FMD rarely cause clinically significant renal artery stenosis (RAS) and is typically truncal or distal. It's often curable with revascularization, percutaneous renal angioplasty being the treatment of choice. Design and Method: We hereby present a case of renovascular hypertension in young male which turned out to be unifocal FMD not amenable to percutaneous angioplasty. Results: We present a case of resistant hypertension in 34 years old male who was diagnosed with renovascular hypertension due to right RAS. Patient remained hypertensive (maximum BP 210/110 mmHg) despite being on nine antihypertensive medications including diuretics (Table). Computed tomographic angiogram revealed occlusion of right main renal artery (MRA) with reformation through lumbar collaterals. Digital substraction angiography revealed atretic right MRA at its origin while Diethylene Triamine Penta Acetic acid (DTPA) renogram showed a small poorly functional right kidney. As percutaneous interventional revascularization was not possible, autotransplantation of right kidney with in situ revacularization with reversed great saphenous vein graft was planned. Intraoperatively, small calibre right MRA was found which was not amenable to aortorenal bypass. Eventually, patient underwent right nephrectomy. Histopathological examination revealed ischemic nephropathy with fibromuscular dysplasia (Medial fibroplasia) (Figure). Patient recovered completely and doesn’t require antihypertensive drug treatment. Conclusions: FMD is rare cause of ostial unifocal RAS in males but must be ruled out in young hypertensives, even in absence of family history or the classic “string-of-beads” appearance in angiography. This case reiterates the need of multiple imaging modalities for early diagnosis and the usefulness of interventional procedures for prompt treatment of renovascular hypertension. In cases of FMD not amenable to percutaneous angioplasty, nephrectomy may be considered as curative option.

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