Journal of Human Hypertension (2001) 15, 499–501
 2001 Nature Publishing Group All rights reserved 0950-9240/01 $15.00
www.nature.com/jhh

CASE REPORT

Moyamoya disease associated with
renovascular disease in a young AfricanBrazilian patient
FD Fuchs1, CR Francesconi2, PR Caramori1, R da Silva Júnior1, C Finger1 and
L Francisco Costa3
Divisions of 1Cardiology, 2Neurology and 3Vascular Surgery, Hospital de Clı́nicas de Porto Alegre, Porto
Alegre, RS, Brazil

Moyamoya is a rare disease characterized by fibrous
dysplasia of the internal carotid and proximal cerebral
arteries, which has been described mainly in young
Japanese. We present a case of Moyamoya disease with
renal artery involvement in a young male patient with
an African origin. A 15-year-old boy was referred to our
hospital due to uncontrolled blood pressure, headache,
somnolence, cognitive deficit and multiple lacunar
infarcts in the computed tomography. Cerebral arteriography showed the absence of the normal vascular
anatomy at the level of the circle of Willis. The intracraneal vessels presented severe stenosis or were
occluded and replaced by an extensive network of ectasic collateral vessels. Abdominal ultrasound examination identified asymmetric kidneys, and renal arteriog-

raphy showed a tight stenosis of the ostium and
proximal segment of right main artery, which was only
partially relieved by balloon angioplasty. A saphenous
bypass from aorta to the right renal artery re-established the renal blood flow. Blood pressure dropped
after surgery and was controlled with low doses of
diuretic and beta-blocker. After arteriography he
presented right haemiplegia, with partial recovering in
the following months. In conclusion, we described the
first case of Moyamoya disease with concomitant renovascular disease in a young adult of African origin, an
association that may be more frequent than usually suspected in clinical practice.
Journal of Human Hypertension (2001) 15, 499–501

Keywords: Moyamoya disease; renovascular hypertension

Introduction
Moyamoya is a rare disease of children and young
adults characterized by fibrous dysplasia of the
internal carotid and proximal cerebral arteries.1 A
fine collateral vascular network develops in the
ganglionic region of the brain, which gives an aspect
of a puff of smoke (moyamoya in Japanese) in the
cerebral arteriography.2 Most cases have been
reported in Japan, with a predominance of female
children,3 but an increasing number of cases have
been reported in non-Japanese adults.4 The coexistence of lesions in the cerebral and renal vascular
territories was scarcely reported.5–13 More recently,
Yamada and colleagues14 reported seven cases of
renovascular disease among 86 patients with angiographic documented Moyamoya disease. Most cases
occurred in patients with an Asian origin and a few
Correspondence: Dr Flávio Danni Fuchs, Serviço de Cardiologia,
Hospital de Clı́nicas de Porto Alegre, Ramiro Barcelos, 2350,
90.035–003, Porto Alegre, RS, Brazil
E-mail: ffuchs얀hcpa.ufrgs.br
Received 8 January 2001; revised and accepted 19 February 2001

were reported in Caucasian patients. We present a
case of Moyamoya disease with renal artery involvement in a young male adult, which may be the first
to be described in a patient with an African origin.

Case report
A 15-year-old boy was referred to our institution
from a provincial hospital with a history of uncontrolled blood pressure. According to the parents, he
had good health previously and was at the 6th grade
of the elementary school. They looked for medical
assistance due to the occurrence of headache and
somnolence, when high blood pressure levels were
detected. He was initially treated with large doses
of propranolol and captopril, and in the days preceding the referring he was hospitalised in an intensive care unit to receive a continuous infusion of
sodium nitroprussiate. The referring physician
reported some degree of cognitive impairment. At
inspection the boy and his parents were characterized as mulattos. Both the father and the mother
had one parent of African origin. On the initial
evaluation in our institution the patient presented

Moyamoya disease and renovascular hypertension
FD Fuchs et al

500

with aphasia, paresis of the right arm and Babinsky
sign at the left side. Blood pressure was around
160/100 mm Hg in several readings. Optic fundi
examination showed minimal arteriolar narrowing
and the remaining physical examination was unremarkable. No abdominal bruit was heard. Computed
tomography (CT scan) showed multiple small
lacunar infarcts, without any sign of cerebral haemorrhage. Abdominal ultrasound examination identified asymmetric kidneys, with the right measuring
8 cm and the left 13 cm. Cerebral arteriography
presented a picture characteristic of Moyamoya disease, with the disappearance of the normal vascular
anatomy at the level of the circle of Willis. The
intracraneal vessels presented severe stenosis or
were occluded and replaced by an extensive network of collateral vessels, which were sometimes
ectasic (Figure 1). Renal arteriography showed a
tight stenosis of the ostium and proximal segment
of right main artery (Figure 2a). No hormonal study
was done.
Blood pressure was partially controlled with a thiazide diuretic and high doses of propranolol and
minoxidil. The patient presented a deterioration of
neurological symptoms after the cerebral arteriography, becoming almost haemiplegic at the right side
for a few days. An attempt to dilate the renal artery
stenosis by balloon angioplasty produced suboptimal result (Figure 2b), despite high pressure

Figure 2 Right renal arteriography showing a tight stenosis of the
ostium and proximal segment of right main artery (a) and the
partial relief obtained by balloon angioplasty (b).

inflation (16 atm). A saphenous bypass from aorta to
the right renal artery re-established renal flow,
which was confirmed by a late post-operatory ultrasound and renal scintilography. The renal artery
lesion was not biopsed. After a period of worsening
of the motor deficit following the surgery, the
patient recovered partially, and started walking with
help a few days before being discharged. Blood
pressure dropped progressively after surgery and the
patient was discharged taking only 25 mg daily of
hydrochlorothiazide and 80 mg of propranolol
daily, with blood pressure around 140/80 mm Hg.
Three months after discharge he still had a motor
deficit, and was walking with help. Blood pressure
was controlled with the same doses of diuretic and
beta-blocker.

Discussion

Figure 1 Right (a) and left (b) carotid arteriography showing a
bilateral picture characteristic of Moyamoya disease, with the disappearance of the normal vascular anatomy at the level of the
circle of Willis.
Journal of Human Hypertension

Most cases of Moyamoya disease have been reported
in Japan, and even there the incidence is estimated
to be less than 1 in 100 000.3 In Brazil, the occurrence of Moyamoya disease has been scarcely
described,15–17 and in none of these cases the disease
presented with concomitant renovascular hypertension. In one study,17 two among three adults with
Moyamoya disease had hypertension, but none was
submitted to renovascular investigation.17 One of

Moyamoya disease and renovascular hypertension
FD Fuchs et al

these patients was a melanodermic man. To our
knowledge, our report is the first documented case
of Moyamoya disease with renovascular disease in
a patient with African ancestry.
The aetiology, natural history, pathogenesis and
ideal treatment of Moyamoya disease have been
poorly defined.18–20 Congenital and acquired forms
have been described, sometimes in association with
disorders of haemosthasy, sickle cell disease,
Down’s syndrome and infection. Hypertension is
uncommonly reported. A surgical procedure for
revascularisation of the ischaemic brain has been
proposed,21 but satisfactory follow-up after a conservative approach was described, mostly in adults.3
Our case had a cerebrovascular anatomy unsuitable
to surgical anastomosis.
The only estimate of the prevalence of renovascular disease in patients with Moyamoya disease was
given by Yamada and co-workers.14 In their series,
seven out of 86 patients with Moyamoya disease
(8%), prospectively examined with cerebral and
renal arteriography, had renal artery lesions. Two of
them presented with renovascular hypertension.
This prevalence, observed in a sample of young
patients, is certainly higher than in the general
population. The simultaneous intimal thickening in
intracranial and extracranial vessels was described
by Ikeda in patients with Moyamoya disease.22
These findings suggest that both focal and systemic
factors may be involved in the pathogenesis of the
vascular abnormalities, but they were not identified
to date.
In summary, we described the first case of Moyamoya disease with concomitant renovascular disease in a young adult of African origin. The medical
awareness about this association, and the consequent increasing in the level of suspicion, may lead
to the identification of new cases, to the better
understanding of its pathogenesis, and to the identification of an efficacious therapy or prevention.

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