Congenital Primary Essential Cutis Verticis Gyrata.

The patient present herein is unique because, unlike most cases of primary CVG, he presented with congenital asymmetric folds extending on to the forehead and has no associated medical problems. As discussed, most cases of primary CVG occur postpubertal, rarely occurring at birth. Only 10 cases of congenital primary CVG have been reported, of which 9 were reported in girls with Turner's syndrome and the other was of a female infant with a normal karyotype but severe developmental delay and hypotonia, making these all nonessential cases.3,8 This is the first documented case of congenital primary essential CVG in the literature. Because of the location and size of the folds, complete excision with primary closure was performed. To achieve an optimal closure, undermining in the subgaleal plane was done to maximize advancement of the adjacent soft tissue. Care was taken to reapproximate the hairline to avoid distortion, and multiple adjacent Z-plasties were created within the hair-bearing scalp to hide the future scar.