CEREBRAL CAVERNOMA IN CHILDHOOD : A CASE

REPORT

M. BOUSSALAH, N. TOUIL, O. KACIMI, N. CHIKHAOUIEmergency Radiology Department, Ibn Roch University Hospital,

Casablanca, Morroco

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INTRODUCTION :• Cerebral Cavernomas have recently received a

great deal of attention because of the improvement of neuroimaging and heightened clinical awareness.

• It accounts 5% to 10% of vascular malformations in the central nervous system [1].

• It is most commonly found in the cerebral hemispheres and rarely in the brainstem region with very low percentage in the mesencephalic region.

• They are low-flow vascular lesions affecting 0.4–0.5% of the population [2].

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INTRODUCTION :

• A congenital origin is generally assumed [3].• Single or multiple cerebral cavernomas may

be sporadic or familial, and may cause seizures and cerebral hemorrhages [3].

• We aim to establish the clinical and therapeutic characteristics of cerebral cavernomas in childhood and to purpose a literature review about its radiological particularities.

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MATERIEL AND METHODS :

•We report the case of a sporadic cerebral cavernomas in a 5-year-old boy, revealed by a severe head injury.

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A CASE REPORT :• S. M • Five years-old boy,• Without medical history of seizures, or familial

history of cavernomas,• Admitted in the Intensive Care Unit for Severe

head injury,• In the emergency setting, the computed-

tomography (CT-scan) showed deep hematomas above and below the tentorium [Figure. 1a],

• The control CT –scan showed radiological improvement [Figure. 1b].

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A CASE REPORT :

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Fig. 1 : Axial CT-scan images. A. left cerebellar hematoma few hours after the head injury. B. radiological improvement of the left cerebellar hematoma in the CT-scan control. Axial CT scan

A B

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A CASE REPORT :

• Magnetic Resonance Imaging (MRI) demonstrated popcorn-like rounded lesions on both T1- and T2-weighted images [Figure. 2].

• A rim of decreased signal intensity at the periphery of a heterogeneous central signal was characteristic on T2- weighted images [Figure. 3-4].

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A CASE REPORT :

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Fig. 2 : Contrast-enhanced T1-weighted axial (a), coronal (b) and sagittal ( c) images exhibit heterogeneous enhancement of left cerebellar , right frontal and parietal cerebral cavernomas.

A B C

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Fig. 3 : A and B. T2-weighted axial typical appearance of the left cerebellar “mulberry-like” lesion with peripheral ring giving decreased signal intensity.

ABA

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Fig. 4 : T2-weighted axial images : right frontal (A), left periventricular (B) and right occipital (C-D) lesions with peripheral ring giving decreased signal intensity.

A

B

C

D

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A CASE REPORT :

Figure. 5 : The angiography revealed no associated vascular abnormality.

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General data :

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• Cerebral cavernous angiomas (CCA) are vascular malformations characterized by abnormally enlarged capillaries without intervening brain parenchyma [3, 4].

• Macroscopic level : these lesions are variable in size, multiple or single, often encapsulated and multilobar [4].

• Localization : central nervous system, spinal cord, cranial nerves and ventricles [4].

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General data :

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• Familial CCA are characterized by familial occurrence of multiple lesions and appearance of new ones, related to genetic defects affecting angiogenesis [5].

• It accounts 5% to 10% of vascular malformations in the central nervous system [1].

• They are low-flow vascular lesions affecting 0.4 – 0.5% of the population [2].

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Age and gender :

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•May present at any age, a bimodal age distribution has often been reported in the literature [6].

•No significant sex difference, but there are reports that describe a female predominance in very young patients [6].

15Clinical presentation

:

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• Seizures are the most common manifestation, both in adults and children, followed by headache and focal symptoms [6].

• Cantu et al: two-fold increased rate of hemorrhage per patient and per year for CCA located in brainstem and cerebellum, and deep hemispheric CCA [7].

• Severe acute headache as a symptom of acute hemorrhage may be accompanied by neurological deficits and/or changes in the level of consciousness [4, 6].

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Familial forms :

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• Three genetic loci are known, and are inherited as autosomal dominants [6].

• The incidence of familial forms is 20% in Caucasians and 50% in Hispano - Americans.

• Characterized by their more aggressive behavior (hemorrhage, seizures, and neurological deficits) and show a high rate of multiple lesions [6].

• Familial forms have been found to present clinically at younger ages.

• No familial history was found in our case.

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Neuroimaging :

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Computed tomography :

•Can demonstrate lesions / hemorrhage in acute presentation [figure 1].

•Not appropriate for follow up.

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Neuroimaging :

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Magnetic Resonance Imaging : • Gold standard for its diagnosis.• Characteristic features : [6]

“mulberry-like” lesions with well-delineated nodules with a peripheral ring producing decreased signal intensity due to the deposition of hemosiderin on T2- weighting, central hyper-intense foci due to methemoglobin, and mixed signals on T1-weighting

• High-field MRI is the diagnostic tool of choice owing to its high sensitivity and specificity for small angiographically cryptic lesions [5].

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Neuroimaging :

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Magnetic Resonance Imaging : • The appearance of CCA on MRI allows grouping

into 4 broad categories : Table 1 [6]

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Neuroimaging :

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Conventional angiography :

•Not usually detectable because of there low flow character.

•Not suitable for interventional embolization.

• Invasive technique.•Our case : it shows no abnormalities

[figure. 5].

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Treatment :

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• It still represents a challenge.• Microsurgical resection : state of art treatment

in symptomatic, isolated and localized CCA, in non-eloquent regions of the brain [8].

• Controversial neurosurgical approaches in eloquent regions.

• Restricted to cases with life threating mass lesion or strong and therapy resistant epileptogeneity.

• Scott et al : benefit of neurosurgery in childhood [9].

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REFERENCES :1. Cristini A, Fischer C, Sindou M. Tectal plate cavernoma—a special entity of

brainstem cavernomas: case report. Surg Neurol 2004;61: 474- 8.2. Curling O Jr, Kelly DL, Jr, Elster AD, Craven TE. An analysis of the natural history

of cavernous angiomas. J Neurosurg 1991;75:702– 8.3. Robinson JR, Awald IA. Clinical spectrum and natural course. In Awald IA, Barrow

DI, eds. Cavernous malformations. Park Ridge, Ill: American Association of Neurological Surgeons, 1993:25–36.

4. Raychaudhuri R, Huntington Bajter H, Awad IA, Intracranial cavernous angioma: a practical review of clinical and biological aspects. Surgical Neurology 2005; 63: 319–328.

5. Kobayashi E, Bagshaw AP, Gotman J, Dubeau F, Metabolic correlates of epileptic spikes in cerebral cavernous angiomas. Epilepsy Research; 2007; 73 : 98—103.

6. Bigi S, Capone Mori A, Steinlin M, Remonda L, Landolt H, Boltshauser E, Cavernous malformations of the central nervous system in children: Presentation, treatment and outcome of 20 cases.. EJ of Pediatric Neurology 2011; 1 5 : 1 0 9 e1 1 6.

7. Cantu C, et al. Predictive factors for intracerebral hemorrhage in patients with cavernous angiomas. Neurol Res 2005;27(3): 314e8.

8. Amin-Hanjani S, et al. Risks of surgical management for cavernous malformations of the nervous system. Neurosurgery 1998;42(6):1220e7. discussion 1227e1228.

9. Maggi G, et al. Cerebral cavernous angiomas in critical areas. Reports of three cases in children. J Neurosurg Sci 1997;41(4): 353e7.

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CONCLUSION :• Cerebral cavernomas mostly present with

seizure, headache and focal deficits .• Children rarely suffer severe life threatening

hemorrhages, therefore emergency neurosurgical intervention is very rare.

• However, microsurgical resection appears to be a safe intervention that should be the first choice in cases of symptomatic cavernomas.

• With the MRI techniques, we are able to detect even small lesions and define their real extent.

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ABSTRACT :• Objectives : Cerebral cavernomas are vascular

malformations frequently localized in the central nervous system, and are very rare in childhood.

• We aim to establish the clinical and therapeutic characteristics of cerebral cavernomas in childhood and to purpose a literature review about its radiological particularities.

• Materials and methods : we report the case of a sporadic cerebral cavernomas in a 5-year-old boy, revealed by a severe head injury.

• Results : Our patient underwent a complete radiological exploration. In the emergency setting, the computed-tomography showed deep hematomas above and below the tentorium.

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ABSTRACT :• The control CT–scan showed radiological

improvement, an MRI demonstrated popcorn-like rounded lesions on both T1- and T2-weighted images. A rim of decreased signal intensity at the periphery of a heterogeneous central signal was characteristic on T2- weighted images. The angiography revealed no associated vascular abnormality.

• Conclusion : MRI is the best radiological technique to explore cavernomas. In the emergency setting, the first exam is often a CT-scan for patients presenting acute neurological sign(s) and/or with a clinical suspicion of hemorrhagic stroke. Angiography is generally not contributive; nevertheless, this exam is often necessary when an associated vascular abnormality is suspected. PEDIATRICS : PD 12