Nevoid Hypertrichosis Associated with Spina Bifida

A 14 year old female presented with a patch of hair on her lower back since infancy (Figure 1). There was history of prolonged low backache. The antenatal and birth history was uneventful. Developmental milestones achieved by her were normal for age. There was no history of urinary or fecal incontinence or lower limb weakness. On examination, a well-defined patch of terminal, dark hair was noted on the lumbosacral region in the midline, though a tuft of hair was not present. Underlying skin was normal. No other cutaneous or mucosal abnormality was noted in the patient. No sensory or motor deficit was elicited. Pelvic radiograph (anteroposterior view) revealed spina bifida at L5 level and scoliosis (Figure 2). No other bony or articular abnormality was found. Vertebral height and intervertebral disc spaces were maintained. Soft tissue appeared to be normal. Based on history and examination, a diagnosis of nevoid hypertrichosis with spina bifida was made.