Idiopathic Thoracolumbar Syrinx with Cutaneous Marker

This 8-month-old girl presented with a 3-cm region of cutis aplasia and hypertrichosis of the lumbar region which had remained unchanged since her birth. She is the product of a normal pregnancy and delivery and has had no neurologic signs or symptoms. Specifically, her bowel and bladder functions are normal, as is the function of her lower extremities. Her head circumference is exactly at the 50th percentile and there are no other external signs of dysraphism or scoliosis. Review of her screening magnetic resonance (MR) imaging reveals a large syrinx cavity at the thoracolumbar junction (a, b) . There is no associated Chiari malformation and no gadolinium enhancement.