Hydrocephalus in myelomeningocele: Shunts and problems with shunts

Abstract

Hydrocephalus (HC) is a major problem for the majority of patients with myelomeningocele (MM). Historically, the correlation of MM with HC was suspected as early as 1769 by Morgagni [1], who hypothesized that an excess of fluid caused both the spinal cyst and the hydrocephalus. He was, however, unable to explain the relationship in more detail, and his theory had to await a better understanding of the circulation of cerebrospinal fluid (CSF) in the twentieth century, with the works of Dandy and Blackfan, to gain approval [2]. Gardner imagined that overdistension of the fetal neural tube caused hydrocephalomyelia and explained all dysraphic states (which in his mind included the Chiari I malformation), but this interesting theory has not been upheld in face of the evidence gained from modern imaging and embryology [3]. The pathophysiology of MM-related HC (MMH) is still a matter of debate, with important therapeutic implications; in particular the role of prenatal meningeal irritation caused by the amniotic fluid, and the putative effect of in utero surgery [4]. © 2008 Springer-Verlag Italia.