Occult Spinal Dysraphism
A 3-year-old girl was referred for the removal of excess hair on the back, which had been present since birth, for cosmetic reasons. The physical examination revealed a tuft of coarse, dark hair in the midline of the back just above the natal cleft (Panel A). Her history revealed leakage of clear fluid from the hypertrichotic region since birth. At that time, magnetic resonance imaging had shown syringomyelia at L1 (Panel B, arrowhead), diastematomyelia with bony spurs at L3 and L4 (Panel C, arrow), and tethered cord and low-lying conus medullaris at L5 (Panel B, arrow). At the age of 4 days, the patient had undergone repair of leakage of cerebrospinal fluid, and at 7 months of age, she had undergone drainage of the syrinx, release of tethered cord, and removal of bony spurs to repair the diastematomyelia. In the 3-year follow-up after surgery, the patient had had no leakage of cerebrospinal fluid, no urinary or stool incontinence, no difficulty in walking, and no developmental delay. The histopathological examination of a specimen of cerebrospinal fluid that had been obtained during the first surgery was compatible with meningocele. A patch of hair in the lower back may be a cutaneous marker of spinal dysraphism and should prompt consideration of underlying spinal abnormalities to prevent neurologic sequelae. The parents were informed about the laser technique for removing excess hair when the child is old enough to cooperate with the procedure.