ENCEPHALOCELES OF THE ANTERIOR CRANIAL FOSSA - MANAGEMENT AND OUTCOME

From a total of 114 encephaloceles treated surgically at The Hospital for Sick Children in the 15 years to 1994, the case records of 17 pati ents with sincipital and 5 patients with basal defects were reviewed r etrospectively. The condition was evident at birth in 64% of patients, while the remainder presented with either cerebrospinal fluid (CSF) r hinorrhea, nasal obstruction, or feeding difficulty. Hypertelorism aff ected 73% of patients. All encephaloceles were repaired transcranially , at a mean age of 2 years, usually by means of an intradural pericran ial graft. Five children with gross hypertelorism underwent orbital tr anslocation at the time of encephalocele repair. Of those not correcte d, primary and secondary hypertelorism regressed in most instances whe re the encephalocele was treated before the age of 2 years. There were no deaths. The only case of CSF rhinorrhea occurred in a patient with a basal defect, in whom intradural repair was not possible because of adherence of diencephalic structures to the sac wall. Hypertelorism r ecurred in 1 patient after orbital translocation, requiring recorrecti on 2 years later. One patient with untreated secondary hypertelorism f ailed to regress after the encephalocele was excised at the age of 4 m onths. Developmental outcome was normal in 59% of children, whilst 18% have mild mental or physical disability, and 23% are severely impaire d. A child with a sincipital or basal defect and mild hypertelorism sh ould have the encephalocele treated in early childhood to allow the fa cial skeleton to remodel with growth. When an encephalocele is accompa nied by gross hypertelorism or a facial cleft, one-stage correction ca n be undertaken safely in early childhood with minimal mortality and a cceptable morbidity.