HYDROCEPHALUS IN APERT SYNDROME - A RETROSPECTIVE REVIEW

A retrospective evaluation was carried out to define the incidence of hydrocephalus and associated factors in 44 patients with Apert syndrom e treated at The Hospital for Sick Children in Toronto over a 22-year period. Forty-three of these patients underwent cranioorbital decompre ssive procedures within 1 year of birth. Fifteen of 25 (60%) patients who had either a computed tomography scan or pneumoencephalogram had v entriculomegaly, and 3 of the 25 (12%) had associated brain anomalies. Ten of the 44 (23%) patients had cerebrospinal fluid (CSF) shunts pla ced, 7 lumboperitoneal and 3 ventriculoperitoneal. Six of the shunts w ere placed early after cranioorbital procedures (CSF leaks in 5 cases and a subgaleal fluid collection in 1 case). The average IQ of 15 pati ents evaluated by the Wechsler Intelligence Scale was 72.5, indicative of significant intellectual impairment. There was no correlation betw een IQ and ventricular size. Although hydrocephalus characterized by p rogressive ventricular dilatation is uncommon in Apert syndrome, posto perative problems related to impaired CSF circulation are common and m ay indicate an underlying CSF absorptive deficit.