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.