Renovascular hypertension in children with moyamoya disease.
OBJECTIVE
To examine the incidence, clinical and radiologic findings, and response to treatment of renovascular hypertension (RVHT) in moyamoya disease (MMD).


METHODS
A retrospective analysis of medical records in six RVHT cases (8.3%) among 72 MMD patients observed from November 1987 to December 1995.


RESULTS
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. At the last follow-up, one patient had normal blood pressure without the use of antihypertensive agents, but the other five patients needed this medication to control blood pressure.


CONCLUSIONS
Because RVHT may be more commonly associated with MMD than has hitherto been appreciated, it is recommended that blood pressure be carefully followed and that diagnostic procedures for RVHT be carried out in hypertensive patients with MMD.
Choi
Y
Y
Department of Pediatrics, College of Medicine, Seoul National University, Korea.
Kang
B C
BC
Kim
K J
KJ
Cheong
H I
HI
Hwang
Y S
YS
Wang
K C
KC
Kim
I O
IO
D000293:Adolescent; D000792:Angiography; D000806:Angiotensin-Converting Enzyme Inhibitors; D000959:Antihypertensive Agents; D001706:Biopsy; D001794:Blood Pressure; D002216:Captopril; D002648:Child; D002675:Child, Preschool; D005260:Female; D005352:Fibromuscular Dysplasia / Q000473:pathology; D005500:Follow-Up Studies; D006801:Humans; D006978:Hypertension, Renovascular / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D015994:Incidence; D007223:Infant; D002546:Ischemic Attack, Transient / Q000209:etiology; D007668:Kidney / Q000000981:diagnostic imaging; D016030:Kidney Transplantation; D008297:Male; D009072:Moyamoya Disease / Q000150:complications*; D015609:Organotechnetium Compounds; D011877:Radionuclide Imaging; D019275:Radiopharmaceuticals; D012078:Renal Artery Obstruction / Q000000981:diagnostic imaging; D012083:Renin / Q000097:blood; D012189:Retrospective Studies; D012640:Seizures / Q000209:etiology; D004113:Succimer; D019783:Technetium Tc 99m Dimercaptosuccinic Acid; D017539:Tunica Intima / Q000473:pathology; D014463:Ultrasonography
D000806:Angiotensin-Converting Enzyme Inhibitors; D000959:Antihypertensive Agents; D015609:Organotechnetium Compounds; D019275:Radiopharmaceuticals; D019783:Technetium Tc 99m Dimercaptosuccinic Acid; D002216:Captopril; D004113:Succimer; D012083:Renin