The role of endoscopic third ventriculostomy in the treatment of hydrocephalus associated with faciocraniosynostosis.

OBJECT: The aim of this study was to assess the efficacy of endoscopic third ventriculostomy (ETV) in the treatment of hydrocephalus in children with complex craniosynostosis.

METHODS

Medical charts from 2194 children treated at the Craniofacial Unit of Necker Enfants Malades between 1995 and 2008 were reviewed. Among them, 293 were affected by a syndromic faciocraniosynostosis. The management of cases with hydrocephalus treated using ETV was examined and analyzed with regard to successes and failures.

RESULTS

Forty-six children with associated progressive hydrocephalus were identified, and 11 (8 boys, 3 girls) had undergone an ETV. All were affected by a faciocraniosynostosis (Crouzon syndrome in 5, Pfeiffer syndrome in 3, and unclassifiable in 3). A descent of the cerebellar tonsils was found in 5 children. In 7 children the hydrocephalus was controlled by an ETV. In the remaining 4 cases a ventriculoperitoneal shunt was also needed. The delay between the 2 surgeries ranged from 1 week to 6 months in these 4 patients. The follow-up period ranged from 11 months to 13 years.

CONCLUSIONS

Performing an ETV may facilitate control of hydrocephalus associated with faciocraniosynostosis in select cases. However, because of the relatively high failure rate of this procedure, a close clinical and radiological monitoring of these children is mandatory.