The role of fetal neurosurgery in spina bifida

Abstract

Myelomeningocele (MMC) is a nonlethal form of neural tube defect (NTD) that results from failure of the neural tube to fuse during early embryogenesis. The lesion is characterized by protrusion of the meninges through a midline bony defect of the spine, a sac containing cerebrospinal fluid and dysplastic neural tissue not covered by skin. MMC represents an important congenital defect of the brain and spinal cord that affects approximately one in 2,000 live births and about 23% of pregnancies that end in elective abortion [1, 2]. MMC leads to lifelong and significant physical disabilities including paraplegia, hydrocephalus, bladder and fecal incontinence, sexual dysfunction, skeletal deformation and mental impairment [3]. The mortality rate, which can be as high as 47% on long-term followup, is principally attributable to the hindbrain herniation observed in Chiari malformation and to the renal failure observed in neurogenic bladder dysfunction [4]. © 2008 Springer-Verlag Italia.