Keywords acute intracerebral hemorrhage - cortical cerebral aneurysm - pediatric
Case Presentation
An 8-year-old boy was found collapsed in the bathroom at home. On arrival to a regional
hospital, he had a Glasgow Coma Score of 3 and was intubated.
His computed tomography (CT) brain and angiogram ([Figs. 1 ] and [2 ]) showed large frontoparietal intraparenchymal hemorrhage measuring 61 x 39 x 48
mm, with a 14 mm spherical hyperdense lesion within the hematoma. Upon contrast administration,
this lesion avidly enhanced, suggesting an arterial filling body—likely an aneurysm.
There was significant mass effect with 4 mm subfalcine herniation to the right. He
was transferred to a tertiary care center via air ambulance approximately within 2
hours, during which he developed a unilateral fixed dilated left pupil.
Fig. 1 Cranial computed tomography (CT) scan. (A ) Sagittal and (B ) axial showing large frontoparietal intraparenchymal hemorrhage with uniformly enhancing
lesion. (C ) Coronal and (D ) sagittal images with three-dimensional reconstruction of CT scan and CT angiogram
demonstrating cortical aneurysm.
Fig. 2 (A ) Hematoxylin and eosin stain of the aneurysm. (B ) Immunohistochemical stain.
Background Medical History
Background Medical History
Severe autism, attention deficit hyperactive disorder, and behavioral exacerbations
with explosive violent outbursts are the background medical history.
Operation
Upon arrival at the tertiary center, he immediately underwent emergency craniotomy.
Intraoperatively, the hemorrhage was evacuated. An aneurysmal mass was noted to arise
from the cortical artery over the parietal lobe with clear evidence of recent rupture.
Due to the relative size of the aneurysm (~15 mm) and the parent vessel (approximately
<2 mm), the aneurysm had a fusiform configuration despite being spherical in shape.
The distal branch territory was damaged by the hematoma. Hence, the aneurysm was obliterated
by clipping the proximal artery. The aneurysm was resected for histopathological analysis.
An ipsilateral external ventricular drain (EVD) was placed for monitoring and cerebrospinal
fluid (CSF) drainage.
During the following 6 hours, the patient developed raised intracranial pressures
(ICP). Repeat CT showed trapping of the contralateral ventricle due to persistent
subfalcine herniation. A contralateral EVD was inserted for CSF diversion. Over the
next 48 hours, he had a persistently raised ICP despite maximal medical management
and aggressive CSF drainage. A further CT scan demonstrated hypodensity in the basal
ganglia; retrospectively, this was evolving on previous scans. Although hypoxic injury
was suspected, given ongoing intracranial hypertension, decompressive craniectomy
was performed, whereupon ICP stabilized. All the investigations were negative for
infective etiology.
Magnetic resonance imaging scan of the brain, performed on the seventh postoperative
day, demonstrated extensive hypoxic brain injury. It was thought that this was most
likely sustained during initial collapse. The patient developed generalized tonic–clonic
seizures and showed minimal evidence of neurological improvement. He was palliated
and subsequently died.
Histopathology Report
Histopathology specimen was suggestive of a ruptured vessel with an attenuated wall
in keeping with an aneurysm ([Fig. 2A ]). The immunohistochemical stain positive for CD31 (brown staining) confirmed that
the structure is a vessel ([Fig. 2 ]).
Discussion and Review of Literature
Discussion and Review of Literature
Pediatric intracranial aneurysmal hemorrhages are rare and the incidence in children
of less than 15 years is 0.05 to 0.09 per 100,000 person-years.[1 ] Interestingly, in the pediatric population, the overall incidence of aneurysm is
noted to be higher in males compared with females (3:2) as opposed to adults (1:2).[1 ] In the case discussed here, the parent artery was occluded due to the distal location,
size, and configuration of the aneurysm. This patient represents the first report
of a noninfective, large peripheral artery aneurysm in a child in the published literature
to our knowledge.
Distal segment middle cerebral artery (MCA) aneurysms account for only 1.1 to 1.7%
of aneurysms.[2 ] A literature review yielded nine cases of ruptured cortical MCA (M4 region) aneurysms,
all in adult patients.[2 ]
The variation in the developmental anatomy of cerebral arterial vessels during embryogenesis
can result in the formation of aneurysms, the first stage of which happens during
angiogenesis.[3 ] The intradural segments of the intracranial arteries have a decreased amount of
elastic lamina and thinner adventitia, which increases the risk for aneurysm formation.[4 ]
There are multiple intrinsic and environmental factors, including hemodynamic stress,
congenital, infectious (15%), and traumatic (5–10%), which may affect structural changes
of cerebral vessels resulting in the development of aneurysm in children.[1 ]
[5 ]
Surgery is considered the best option for the treatment of ruptured cortical aneurysms
with intracerebral hemorrhage, since it allows both management of mass effect and
aneurysmal exclusion.[2 ]
Conclusion
Ruptured cortical aneurysm should be considered as a rare etiology along with other
common differentials such as arteriovenous malformation or tumors in pediatric patients
with spontaneous intracerebral bleed.
