[Extracranial and intracranial vascular abnormalities and nevus of Ota in the same family].
We present a 17 year old patient suffering from hemangioma on the left side of his face and neck. The evolution of his disease has been followed-up since he was 8 1/2 months old. His mother has nevus of Ota in the sclera and adjacent zones in both sides. At the age of 8 1/2 months, the patient had an ischemic stroke in the territory of the left middle cerebral artery during the surgical correction of the aortic arch coarctation. The conventional arteriogram performed at 10 months of life showed several malformations of the intracranial and extracranial arteries. At the age of 16 years the cutaneous hemangioma was atrophic and a new angiographic study by magnetic resonance arteriography (MRA) showed all the vascular anomalies with higher objectivity than the conventional arteriography, especially the absence of some arteries and the abnormal position of some others. Moreover it could detect the decrease of the caliber of the intracranial arteries and the disappearance of hemangiomatous malformations even though the patient had not received any treatment for the cutaneous hemangioma. We think that this syndrome, which still has no definite name, has a parallel spontaneous biological process with progressing vascular size during the first months or years of life and, later, the vessels regress slowly but progressively, leading to a small caliber or even the occlusion of the vessels of the cutaneous hemangioma and of the abnormal extracranial and intracranial arteries. At the same time, there is a slow but progressive presence of transdural collateral vascularization of the brain. We do not find any relationship between the hemangiomatous syndrome of the patient and the nevus of Ota of his mother.
Pascual-Castroviejo
I
I
Servicio de Neurologia Pediátrica, Hospital Universitario de la Paz, Madrid, España. ipascual@hulp.insalud.es
Pascual-Pascual
S I
SI
Moreno
F
F
Viaño
J
J
Martinez
V
V
D000293:Adolescent; D000328:Adult; D001165:Arteriovenous Malformations / Q000473:pathology*; D005153:Facial Neoplasms / Q000473:pathology; D005260:Female; D006391:Hemangioma / Q000473:pathology; D006801:Humans; D007223:Infant; D018810:Magnetic Resonance Angiography; D008297:Male; D009507:Nevus of Ota / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology