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Case Report
Pediatr Neurosurg 1998;28:31–34

Benjamin B. Fulmer a
Stephanie C. Dillard b
Elizabeth M. Musulman c
Cheryl A. Palmer b
Jerry Oakes a

Received: December 23, 1997
Accepted: February 26, 1998

Two Cases of Cerebral Aneurysms
in HIV+ Children

a Division of Neurosurgery, and
b Department of Pathology, University of

Alabama at Birmingham,
c Department of Pathology, The Children’s

Hospital of Alabama, Birmingham, Ala.,
USA

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Key Words
AIDS
Aneurysm, fusiform
HIV, pediatric

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Abstract
Two cases of fusiform cerebral aneurysms in human immunodeficiency virus
(HIV) positive children are presented. To our knowledge, only 9 patients with
this association have been reported. One of our patients represents the first
report of a patient with an aneurysm associated with varicella-zoster vasculitis. One patient presented with a subarachnoid hemorrhage, Hunt-Hess grade
IV, and posed difficult surgical management. The other patient suffered a
cerebral infarct with a resulting hemiparesis. The first patient had a ventriculostomy placed, initially improved, and subsequently died from rebleeding.
The second patient improved with medical management. AIDS arteriopathy,
and specifically fusiform aneurysms, are being increasingly reported. The various presentations of this surgically challenging entity in light of other AIDSrelated syndromes pose difficult management decisions. On occasion, the
intracranial aneurysm may be the initial form of presentation as was present
in our first patient.
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Introduction
Various types of cerebrovascular arteriopathy are increasingly being described in the acquired immunodeficiency syndrome (AIDS) patient population. Pathologic
findings include fibrosis, vasculitis, calcification and
aneurysms, all of which may lead to cerebral infarction
and/or hemorrhage. To this date, only 9 reported cases of
children infected with the HIV virus who have been
found to have intracerebral aneurysms were found in our
search [1–6]. This type of patient poses difficult management issues, from both a medical and a surgical standpoint. Because of their rarity, this association may elude
the diagnostician’s differential diagnosis.
We present 2 cases of intracerebral aneurysms in HIVpositive children. In the 1st case, the patient presented
with subarachnoid hemorrhage. In the 2nd, aneurysms

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were found during the workup of cerebral infarction. In
both cases, the circle of Willis was affected in multiple
areas by fusiform aneurysmal dilatation.

Case Reports
Case 1
A 6 year-old female who was previously considered to be healthy
presented with a 3-day history of cough, nausea, vomiting, and fever.
One day prior to admission she complained of headache and was
reported to have had a seizure. The morning of admission she
became increasingly lethargic.
On physical examination the patient weighed 19.5 kg. Her pulse
was 152/min, blood pressure was 99/57 mm Hg, and she was afebrile.
She had been intubated. Her pupils were 6 mm and unreactive on the
right and 5 mm and sluggishly reactive on the left. She had bilateral
third nerve palsies and a symmetric grimace. She localized to painful
stimuli briskly bilaterally. The remainder of the patient’s physical

W. Jerry Oakes, MD
The Children’s Hospital of Alabama, 1600 7th Avenue South
Suite 400, ACC Building
Birmingham, AL 35233 (USA)
Tel. +1 205 939 9653, Fax +1 205 939 9972, E-Mail Jerry.Oakes@ccc.uab.edu

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a

b

Fig. 1. A-P (a) and lateral (b) arteriogram
of the posterior circulation in patient 1 revealing a large basilar tip aneurysm with
fusiform dilatation of the trunk, bilateral superior cerebellar arteries and posterior cerebral arteries.
a

b

Fig. 2. AP arteriogram of the right (a)
and left anterior (b) circulation in patient 1
revealing fusiform dilatation of the internal
carotid, anterior cerebral arteries and middle
cerebral arteries.

examination was unremarkable except for one left posterior cervical
lymph node.
Computerized tomography (CT) of the head revealed bilateral
subarachnoid hemorrhage (SAH) with moderate panventricular
hemorrhage. She had acute hydrocephalus and a suspected basilar
artery aneurysm.
This patient was therefore admitted with a Hunt-Hess grade IV
SAH and a Fisher grade IV CT scan. The opening pressure from a
ventriculostomy was 25 mm Hg. The next hospital day she followed
commands but her third nerve persisted. An arteriogram was per-

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Pediatr Neurosurg 1998;28:31–34

formed which revealed a large basilar tip aneurysm with fusiform dilatation of the trunk, bilateral superior cerebellar arteries, and bilateral
posterior cerebral arteries (fig. 1). The anterior circulation injections
showed fusiform aneurysms of both internal carotid arteries including
the middle cerebral arteries and anterior cerebral arteries (fig. 2).
The patient was vigorously hydrated, with a central venous pressure of 10 mm Hg. Intracranial pressure (ICP) was maintained in the
normal range by ventriculostomy drainage, and the cerebral perfusion pressure was maintained at 70–80 mm Hg. Plans were being
made for surgical intervention when suddenly on the 4th hospital day

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Fig. 4. Contrast-enhanced CT in patient
2 revealing fusiform dilatation of much of
the circle of Willis.

Fig. 3. Gross dissection of the circle of Willis, revealing fusiform
dilatation in both the anterior and posterior circulation.

the ICP exceeded 100 mg Hg. The ventriculostomy expressed frank
blood. CT revealed increased SAH with a casted ventricular system.
Shortly thereafter, physical examination revealed brain death. Permission for an autopsy was obtained.
Just after this patient’s second event, an HIV test was reported as
positive. Subsequent testing of the mother found her also to be HIV+.
The patient’s mother denied any history of significant past illness.
Pathology. At autopsy, the brain was edematous, weighing
1,135 g. Diffuse subarachnoid hemorrhage was present bilaterally,
most notably over the occipital and parietal lobes, extending to the
undersurface of the brain encasing the circle of Willis. Coronal sectioning revealed extensive intraventricular hemorrhage with diffuse
distension of the ventricular system with clotted blood. There was a
2.0-cm hematoma present in the midportion of the midbrain extending to involve the cephalad portion of the pons. Upon dissection of
the circle of Willis, an aneurysm measuring 2.2 ! 2.1 ! 1.6 cm was
identified at the junction of the basilar arteries, and an area of rupture was present in the midline of the aneurysmic dome (fig. 3). In
addition, bilateral aneurysmal dilatations were present involving the
bifurcations of the internal carotid arteries with the anterior and
middle cerebral arteries, with each dilatation measuring at least
0.6 cm in diameter.

Cerebral Aneurysms in HIV+ Children

Histologically, the brain parenchyma showed diffuse anoxia and
multinucleated giant-cell HIV encephalitis. The dilated cerebral vessels
contained intimal thickening with fibroblast proliferation and a lymphocytic infiltrate, medial thinning and rarefaction of the elastic lamina, all features similar to previously described HIV-associated pediatric cerebral aneurysms [1, 6–8]. Immunohistochemistry for HIV antigens was indeterminate, most likely due to fixation artifact. Immunohistochemical stains directed against varicella-zoster virus (VZV)
showed staining of the dilated vessels as well as smaller vessels within
the brain parenchyma. VZV-associated vasculopathies have been described in AIDS patients, with some lesions of so-called ‘noninflammatory vasculopathy’ demonstrating marked intimal proliferation which
produces occlusion of the affected vessel and resulting infarction.
Occlusion was not present in the current case, and aneurysms associated with VZV infection of the brain have not been described [6].
Case 2
An 11-year-old girl who was known to be HIV-positive had already been treated for associated illness including pulmonary Mycobacterium avium intracellulare pneumonia and cytomegalovirus. She
presented to her primary physician with failure to thrive, fever and
left-sided weakness one year after being found to be HIV+.
Physical examination revealed her to be small for her age (height
and weight less than 5th percentile). Vital signs were normal. Neurologic examination revealed an awake, alert and fully oriented child
with a mild left hemiparesis (4/5). Her speech and the remainder of
her exam were grossly normal.
CT scan of the head with contrast revealed fusiform dilatation of
many areas of her circle of Willis (fig. 4). She had no evidence of
hemorrhage but did have a small basal ganglia/internal capsule
infarction on the right.

Pediatr Neurosurg 1998;28:31–34

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The patient’s hemiparesis nearly resolved over the next 2 months.
She lived 3 more years without further neurologic deficit. Eventually
she expired from other AIDS-related complications. An autopsy was
not performed.

Discussion
AIDS arteriopathy is being increasingly recognized,
and greater numbers of HIV-positive children are presenting with cerebrovascular accidents and/or hemorrhage. Fibrosis, calcification, aneurysms, emboli and vasculitis/vasculopathy have all been described in these patients [2, 3, 6, 9–11]. There is increasing evidence of direct
involvement of HIV virus particles within the vessel walls
[1, 2, 10–12]. One series of 380 pediatric patients who
were HIV+ found the incidence of radiographically proven stroke to be just under 1% [9]. While subacute AIDS
encephalitis is a well-recognized consequence of advanced disease [5], vascular involvement provides yet
more insight into the aggressive, widespread nature of the
virus.
As mentioned previously, we found only 9 reported
cases of intracranial aneurysms in HIV-positive patients,
all of whom were children [1–6]. There have been isolated
reports of aneurysms outside of the cranium such as in the
common carotid or the aorta [7].
Fusiform aneurysms are well known to pose difficult
surgical management decisions. In case 1, surgical options

were being considered before the patient sustained a terminal rehemorrhage. In case 2, the aneurysms were never
directly symptomatic, so neurosurgical consultation was
never sought. Fusiform aneurysms are defined as dilatation and elongation of a blood vessel. They can bleed,
cause cranial nerve deficits, or result in dissection and
infarction [6, 8]. On occasion, they can often be treated by
wrapping with various materials or reconstitution of the
lumen by fenestrated clips. If the involved vessels’ supply
is tolerant, they may be trapped. Overall, these options
are often unsatisfactory. In our 1st case, with extensive
disease of the circle of Willis, there was no perfect
answer.
Another remarkable feature of our 1st case is the VZV.
CNS VZV vasculopathy in AIDS has only been described
in one report of 4 patients [13] and two single patient
reports [14, 15], none of whom were children. In these
cases, marked intimal proliferation produced luminal occlusion, but an inflammatory vasculitis was not described.
Occlusion was not present in our 1st case, and aneurysms
associated with VZV infection have not been reported.
These 2 cases may prove to be illustrative of a clinical
picture that will become more easily recognized. In the
child that presents with fusiform aneurysmal involvement of the intracranial vessels, HIV-induced vasculopathy should be considered and may be the initial manifestation of clinical AIDS.

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