SACRAL MEDIAL TELANGIECTATIC VASCULAR NEVUS - A STUDY OF 43 CHILDREN

Background: Medial telangiectatic vascular nevi are capillary vascular malformations frequently observed at birth occurring mostly on the fa ce or on the nape as a single lesion or as multiple macules affecting more than one site simultaneously, In 1990, Metzker and Shamir reporte d a medial telangiectatic vascular nevus (MTVN) in the sacral region a long the midline and called this particular variety of MTVN 'butterfly -shaped mark'. Objective: Our study was performed to investigate the m orphology and localization of an MTVN in the sacral region (sMTVN) in a group of Caucasian children. Methods: We observed 43 children with s MTVN, ranging in age from 1 month to 12 years (mean 8 years), for a pe riod of 6 years. Results: sMTVN was found as a red-violet macular lesi on of rhomboid or triangular shape in 16 patients and as a group of li ttle red-violet macules in 10 patients. In 16 patients moreover the wh ole back was involved with many small red-violet spots on and around t he spinal column, and in 1 patient with classic sMTVN satellite macule s were also present over both buttocks, Four patients suffered from ep ilepsy with mental deficiency. In 6 patients, the family history showe d sMTVN in one or more members of the family, No case presented an ass ociation with spina bifida. Conclusion: In our study, sMTVN shows a mo rphological polymorphism while Metzker and Shamir reported the same cl inical aspect in all 25 of their patients. In accordance with these au thors, we noted that sMTVN persist into childhood and adult life in th e same way as occipital MTVN.