A two-year-old patient was hospitalized for hypertension associated with loss of strength and sensitivity in the lower limbs.
Arteriography showed decreased aortic caliber, 20 % stenosis of the right renal ostium, critical stenosis of the left renal artery and absence of flow in the left lower renal pole.
It was decided to perform left renal autotransplantation with anastomosis to the iliac artery and biopsy of the renal artery that reported findings consistent with FD.
She was discharged with minoxidil and prolonel.
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One year later, the patient was hospitalized for hypertensive crisis; renal scintigraphy showed a gammagraphic exclusion of the self-transplanted left kidney and impaired perfusion of the right kidney.
Due to suspicion of large vessel vasculitis type AT, a pediatric rheumatology evaluation was requested.
Although the patient met the criteria for classifying TA (this entity of the abdominal aorta and renal arteries associated with hypertension), previous biopsy showed no vasculitis findings.
Another differential diagnosis pointed to SCD, but this was ruled out because renal lesions in SCD have a characteristic pearl collar image and rarely affect the ostium or proximal segments.
In this patient the image of a pearl collar was not observed and the renal artery affection was in the proximal portion of the artery; for these imaging findings, SAoM was finally diagnosed.
A new arteriography was performed, which reported irregular abdominal aorta with progressive distal thinning, occlusion of the arterial anastomosis of the autotransplantation and progression of the right renal artery stenosis. It was decided to perform a successful primary renal angioplasty, which was not
Due to the difficult control of blood pressure figures, selective venous sampling of the renal veins is performed to measure renina, a difference of 10:1 in concentrations of the kidney autotransplanted against the right kidney.
This confirmed the suspicion of renovascular hypertension originating in the autotransplanted kidney.
It was not possible to perform embolization of the renal artery autotransplanted due to the risk of extensive necrosis, since parasytic branches were found that provided flow to the kidney autotransplanted and pelvis intrinsic muscles.
It was decided to perform left autotransplant nephrectomy.
The evolution was satisfactory with better control of blood pressure figures, so the patient was discharged.
