S1-05 SESSION 1: NEUROSURGERY HYDROCEPHALUS AND ITS TREATMENT IN CROUZON’S SYNDROME

Abstract

Introduction: Hydrocephalus is frequently seen in Crouzon’s syndrome. The challenge is to treat the hydrocephalus while not impeding skull growth. We aim to show the hydrocephalus rate in our cohort of Crouzon’s patients as well as its treatment including endoscopic third ventriculostomy (ETV) and Ventriculo- peritoneal shunt (VPS). The need for more than one CSF diverting surgical procedure was documented. Methods: A departmental database search was performed to identify all Crouzon’s patients currently under our care. Records and op-notes were checked for initial craniofacial surgery, prevalence of hydrocephalus and its treatment. Results: We found 72 Crouzon patients that are currently under our care, 16 of which underwent surgery for hydrocephalus (22%). In 10 patients a VPS was inserted as initial hydrocephalus treatment, mean age 2y5m (2m-15y5m). In 6 patients firstly an ETV was performed, mean age 2y4m (1m-10y8m). In 3 out of these 6 ETV patients subsequently a VPS was needed due to unresolved hydrocephalus (50%). 12 out of the 13 patients (92%) who at some point underwent VPS insertion had to undergo at least on more shunt related procedure (1–4) Conclusions: Hydrocephalus is frequently seen in Crouzon’s syndrome, it was present in 22% of our 72 Crouzon’s patients. Most of these patients need more then one CSF-shunt related procedure (92% in our series). ETV was only sustainably effective in 50% of patients, however it delayed the insertion of a VPS shunt in all 6, thereby creating time for vault expansion surgeries to gain their maximal effect, since the presence of a shunt lowers the effect of vault expanding surgery in craniosynostosis.