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CEREBRAL CAVERNOMA IN CHILDHOOD : A CASE REPORT

CEREBRAL CAVERNOMA IN CHILDHOOD : A CASE REPORT. M . BOUSSALAH , N. TOUIL , O. KACIMI , N. CHIKHAOUI Emergency Radiology Department, Ibn Roch University Hospital, Casablanca, Morroco PEDIATRICS PD 12. INTRODUCTION :.

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CEREBRAL CAVERNOMA IN CHILDHOOD : A CASE REPORT

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  1. CEREBRAL CAVERNOMA IN CHILDHOOD : A CASE REPORT M. BOUSSALAH, N. TOUIL, O. KACIMI, N. CHIKHAOUI Emergency Radiology Department, IbnRoch University Hospital, Casablanca, Morroco PEDIATRICS PD 12

  2. PEDIATRICS : PD 12 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].

  3. PEDIATRICS : PD 12 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.

  4. PEDIATRICS : PD 12 MATERIEL AND METHODS : • We report the case of a sporadic cerebral cavernomas in a 5-year-old boy, revealed by a severe head injury.

  5. PEDIATRICS : PD 12 A CASE REPORT : • S. M • Fiveyears-old boy, • Withoutmedicalhistory 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].

  6. PEDIATRICS : PD 12 A CASE REPORT : A B 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

  7. PEDIATRICS : PD 12 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].

  8. PEDIATRICS : PD 12 A CASE REPORT : A B C 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.

  9. PEDIATRICS : PD 12 A CASE REPORT : B A A Fig. 3 : A and B. T2-weighted axial typical appearance of the left cerebellar “mulberry-like” lesion with peripheral ring giving decreased signal intensity.

  10. PEDIATRICS : PD 12 A CASE REPORT : A C B D 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.

  11. PEDIATRICS : PD 12 A CASE REPORT : Figure. 5 : The angiography revealed no associated vascular abnormality.

  12. PEDIATRICS : PD 12 General data : • 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].

  13. PEDIATRICS : PD 12 General data : • Familial CCAare 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].

  14. PEDIATRICS : PD 12 Age and gender : • 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].

  15. PEDIATRICS : PD 12 Clinical presentation : • 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].

  16. PEDIATRICS : PD 12 Familial forms : • 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.

  17. PEDIATRICS : PD 12 Neuroimaging : • Computed tomography : • Can demonstrate lesions / hemorrhage in acute presentation [figure 1]. • Not appropriate for follow up.

  18. PEDIATRICS : PD 12 Neuroimaging : • 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].

  19. PEDIATRICS : PD 12 Neuroimaging : • Magnetic Resonance Imaging : • The appearance of CCAon MRI allows grouping into 4 broad categories : Table 1 [6]

  20. PEDIATRICS : PD 12 Neuroimaging : • 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].

  21. PEDIATRICS : PD 12 Treatment : • 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].

  22. PEDIATRICS : PD 12 REFERENCES : • Cristini A, Fischer C, Sindou M. Tectal plate cavernoma—a special entity of brainstem cavernomas: case report. SurgNeurol2004;61: 474- 8. • 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. • 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. • Raychaudhuri R, Huntington Bajter H, Awad IA, Intracranial cavernous angioma: a practical review of clinical and biological aspects. Surgical Neurology 2005; 63: 319–328. • Kobayashi E, Bagshaw AP, Gotman J, Dubeau F, Metabolic correlates of epileptic spikes in cerebral cavernous angiomas. Epilepsy Research; 2007; 73 : 98—103. • 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. • Cantu C, et al. Predictive factors for intracerebral hemorrhage in patients with cavernous angiomas. Neurol Res 2005;27(3): 314e8. • Amin-Hanjani S, et al. Risks of surgical management for cavernous malformations of the nervous system. Neurosurgery 1998;42(6):1220e7. discussion 1227e1228. • Maggi G, et al. Cerebral cavernous angiomas in critical areas. Reports of three cases in children. J NeurosurgSci 1997;41(4): 353e7.

  23. PEDIATRICS : PD 12 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.

  24. PEDIATRICS : PD 12 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 deephematomasabove and below the tentorium.

  25. PEDIATRICS : PD 12 ABSTRACT : • The control CT–scan showedradiologicalimprovement, an MRIdemonstratedpopcorn-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.

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