Spinal Dysraphism and Myelomeningocele

Excerpt

Spinal dysraphism encompasses congenital problems that result in an abnormal bony formation of the spine and/or the spinal cord. It is caused by the maldevelopment of the ectodermal, mesodermal, and neuroectodermal tissues. The two major types of spinal dysraphism are based on the appearance, i.e., aperta (open) if the lesion is visible and occulta (closed) if the lesion is not visible on the surface. Common manifestations are meningocele, myelomeningocele, lipomeningocele, lipomyelomenigocele, myeloschisis, and rachischisis.

This condition also correlates with cutaneous conditions including port-wine stain, hemangioma, hypertrichosis, fibroma pendulum, pigmentary nevus, lipoma, dermal sinus, and deviation of the gluteal furrow. Myelomeningocele is a spinal dysraphism in which the spinal cord and its contents herniate through a congenital bony defect at the posterior elements of the spine, in most cases, the spinous process. It falls under the aperta category and is also known as an open neural tube defect. This is a serious malformation and is associated with a high mortality rate. Myelomeningocele is the most common presentation of spinal dysraphism and constitutes about 80% of the cases.