A rare cutaneous sign of occult spinal dysraphism with tethered spinal cord.

©2008, World J Pediatr. All rights reserved. A female, born after normal pregnancy and labor, was referred for consultation for a 1-cm long fi brous appendage in the midline of her lumbosacral region one day after birth (Fig. 1). Although she was neurologically intact, computed tomography at 3 months revealed L4-L5 spina bifi da (Fig. 2A), and magnetic resonance imaging (MRI) showed a low conus medullaris level (L3), and a tethered dorsal spinal cord that seemed to connect with the site of the skin lesion (Fig. 2B). Spina bifi da occulta with tethered spinal cord due to a dermal sinus tract was diagnosed and the patient was treated surgically. At operation, the sinus tract extending from the skin to the spinal cord was observed, but the thickening of the fi lum terminale was not evident. Occult spinal dysraphism with tethered spinal cord, if not recognized, may result in permanent neurologic defi cits. Moreover, the neurological disturbances can be diffi cult to demonstrate in a young baby and clinical evaluation of bladder function is equally diffi cult at this age. To prevent serious sequelae, early screening of high-risk infants with cutaneous markers that present in 50%-90% of patients, radiologic diagnosis using X-rays, either MRI or sonography, and urodynamic testing, and the appropriate timing of surgical untethering should be performed. However, dorsal cutaneous lesions may be ignored unless they are common lesions such as midline hemangiomas, hairy patches, dimples or sinuses, or pigmented macules. In order to screen effectively, the physician must have a high degree of suspicion for such a rare skin abnormality as this case. References 1 Guggisberg D, Hadj-Rabia S, Viney C, Bodemer C, Brunelle F, Zerah M, et al. Skin markers of occult spinal dysraphism in children: a review of 54 cases. Arch Dermatol 2004;140:1109-1115. 2 Cornette L, Verpoorten C, Lagae L, Van Calenbergh F, Plets C, Vereecken R, et al. Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release. Neurology 1998;50:1761-1765. 3 Yamada S, Won DJ, Siddiqi J, Yamada SM. Tethered cord syndrome: overview of diagnosis and treatment. Neurol Res 2004;26:719-721. Received March 31, 2008 Accepted after revision May 8, 2008 A rare cutaneous sign of occult spinal dysraphism with tethered spinal cord