Sacral Medial Telangiectatic Vascular Nevus: A Study of 43 Children

Abstract

Background: Medial telangiectatic vascular nevi are capillary vascular malformations frequently observed at birth occurring mostly on the face or on the nape as a single lesion or as multiple macules affecting more than one site simultaneously. In 1990, Metzker and Shamir reported a medial telangiectatic vascular nevus (MTVN) in the sacral region along the midline and called this particular variety of MTVN ‘butterfly-shaped mark’. Objective: Our study was performed to investigate the morphology and localization of an MTVN in the sacral region (sMTVN) in a group of Caucasian children. Methods: We observed 43 children with sMTVN, ranging in age from 1 month to 12 years (mean 8 years), for a period of 6 years. Results: sMTVN was found as a red-violet macular lesion of rhomboid or triangular shape in 16 patients and as a group of little red-violet macules in 10 patients. In 16 patients moreover the whole back was involved with many small red-violet spots on and around the spinal column, and in 1 patient with classic sMTVN satellite macules were also present over both buttocks. Four patients suffered from epilepsy with mental deficiency. In 6 patients, the family history showed sMTVN in one or more members of the family. No case presented an association with spina bifida. Conclusion: In our study, sMTVN shows a morphological polymorphism while Metzker and Shamir reported the same clinical aspect in all 25 of their patients. In accordance with these authors, we noted that sMTVN persist into childhood and adult life in the same way as occipital MTVN.

Skin Markers of Occult Spinal Dysraphism in Children

“…[6][7][8] The screening value of isolated lumbosacral vascular lesions remains difficult to determine because, despite the International Society for the Study of Vascular Anomalies (ISSVA) classification, ambiguity persists in the terminology used in the literature. [9][10][11][12][13][14] At present, the importance of dimples is still under discussion. [15][16][17] Furthermore, no one has established whether isolated deviation of the gluteal furrow (DGF) represents a cutaneous marker of OSD.…”

Nevus simplex: A reconsideration of nomenclature, sites of involvement, and disease associations

“…Our case series emphasizes that NS can involve multiple anatomic sites beyond the ''classic'' locations of the nape, glabella, and eyelids, an observation noted in several previous studies. 1,2,4,8,10,15,17,[19][20][21][22][23][24][25][26] Atypical locations included the left and right side of the forehead, nasal bridge, nose, columella, philtrum, upper cutaneous lip, vermillion, lower cutaneous lip, as well as upper, middle, and lower back (Figs 3 and 4). The differentiation of NS from port-wine stains in these areas is much easier when the typical sites are also affected, as was the case in all of our patients.…”

“…Our findings as well as those in previous reports do serve, however, to emphasize that these sites are part of the spectrum of NS. 1,8,17,21,22,24,25 The natural history of NS is of spontaneous fading in the majority of patients, although NS involving the nape, and occasionally other sites, may persist. 1,3,4,13,17,20 Pasyk et al 17 reported 6 families with persistence of NS beyond infancy and autosomal dominant inheritance.…”

Acquired Nevoid Telangiectasia

“…In congenital cases, medial telangiectatic nevi are not uncommon [1]. Recently, their possible association with the autosomal dominant Williams syndrome has been discussed [2].…”