Renovascular hypertension in children with moyamoya disease.

The Journal of Pediatrics
Y ChoiI O Kim


To examine the incidence, clinical and radiologic findings, and response to treatment of renovascular hypertension (RVHT) in moyamoya disease (MMD). A retrospective analysis of medical records in six RVHT cases (8.3%) among 72 MMD patients observed from November 1987 to December 1995. The age at onset of MMD ranged from 9 months to 7 years 1 month (mean, 3.3 years). The most common initial manifestation of MMD was transient ischemic attack. Hypertension was detected between 4 years 4 months and 12 years 3 months (mean, 7.87 years). Unstimulated plasma renin activity was elevated in all six cases. Renal ultrasonography and captopril technetium 99m-labeled dimercaptosuccinic acid scan showed abnormal findings in four of five and in three of four available studies, respectively. However, both imaging studies showed abnormal findings only in the most severely affected kidneys even with bilateral renal artery stenosis. Renal arteriography revealed bilateral lesions in three of the patients and unilateral lesions in the others. Renal angioplasty was performed in four cases but was successful in only one and partially successful in another. A renal artery specimen obtained during renal autotransplantation showed intimal fibroplasia. A...Continue Reading


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