Challenging problems of shunt management in spina bifida

Abstract

Hydrocephalus in the context of spina bifida may represent the most complicated and confusing form of congenital hydrocephalus. Essentially all patients with spina bifida aperta have an associated Chiari II malformation. In some, especially those with very low lesion levels, this malformation may be very mild. Experimental evidence suggests that the Chiari II malformation results from in utero loss of cerebrospinal fluid (CSF) into the amniotic fluid and subsequent failure of distension of the fetal head leading to a very small posterior fossa and severe herniation of the hindbrain [1]. One of the major indications for prenatal repair of the myelomeningocele is to minimize this descent of the cerebellum and brainstem and therefore maximize the chance that the patient may be able to thrive without a shunt [2-4]. © 2008 Springer-Verlag Italia.