MIGRATION AND ORGANIZATION NEURONAL DISORDERS ASSOCIATED WITH FACIAL HEMANGIOMA AND VASCULAR MALFORMATION: PRESENTATION OF 5 PATIENTS

Objectives: To present 5 patients who showed several types of neuronal migration or organization disorders associated with cutaneous facial hemangioma and vascular malformation.

Methods: We studied 40 patients, 31 females and 9 males, who showed cutaneous hemangioma or vascular malformation in face, neck and / or chest region. Cutaneous lesions consisted of hemangioma in 32 patients, and vascular malformations in 8 patients. Cerebral magnetic resonance (MR) and conventional cerebral arteriography and/or MR arteriography (MRA) were performed in all patients.

Results: Five patients, all females showed some type of neuronal migration or organization disorder. These alterations consisted of corticosubcortical heterotopía in 1 case, polymicrogyria with severe generalized hypoplasia of the cerebral hemisphere in 2 cases, and localized frontal polymicrogyria with subjacent lobular hypoplasia in 2 cases. Lesions were localized in the left cerebral hemisphere, and the frontal region was involved in every case. Five patients had cutaneous hemangioma and one vascular malformation. Cutaneous lesions were located in the left forehead in all patients. The affected cerebral hemisphere was hypoplastic or atrophic in all 5 patients. Some type of vascular malformation, such as absence or hypoplasia of internal carotid or anterior cerebral arteries was observed in all cases.

Conclusion: In our experience disorders of neuronal migration or cortical organization appear associated most often with cutaneous forehead ipsilateral hemangioma or vascular malformation, but not with cutaneous vascular lesions in other areas. Hemispheric malformations were always related with some cerebral vascular alteration such as ipsilateral internal carotid or anterior cerebral arteries absence or hypoplasia. All 5 patients in this series were females.