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Encephaloceles of the anterior cranial fossa
R Macfarlane1, J T Rutka, D Armstrong
1Division of Neurosurgery, University of Toronto, Ont., Canada.
Insights
Surgical repair of encephaloceles, particularly before age two, can allow facial remodeling and improve outcomes. Early treatment of sincipital or basal defects with hypertelorism is recommended for optimal developmental results.
Area of Science:
- Neurosurgery
- Pediatric Surgery
- Craniofacial Surgery
Background:
- Encephaloceles are congenital herniations of brain tissue through skull defects.
- Sincipital and basal encephaloceles present unique surgical challenges.
- Hypertelorism is a common associated anomaly, affecting facial aesthetics and function.
Purpose of the Study:
- To review surgical outcomes for sincipital and basal encephaloceles.
- To evaluate the impact of early surgical intervention on hypertelorism and developmental outcomes.
- To assess the safety and efficacy of transcranial repair and orbital translocation.
Main Methods:
- Retrospective review of 114 encephalocele cases treated between 1979 and 1994.
- Analysis of surgical techniques, including transcranial repair and orbital translocation.
- Assessment of patient demographics, presenting symptoms, surgical timing, and developmental outcomes.
Main Results:
- Encephaloceles were evident at birth in 64% of patients; others presented with CSF rhinorrhea, nasal obstruction, or feeding difficulties.
- Hypertelorism affected 73% of patients; early repair (before age 2) led to regression in most cases.
- Transcranial repair with pericranial grafts was effective; 5 patients with gross hypertelorism underwent successful orbital translocation.
Conclusions:
- Early surgical management of encephaloceles, especially those with hypertelorism, is crucial for optimal facial growth and development.
- Transcranial repair and orbital translocation are safe and effective procedures with low morbidity.
- Prompt treatment allows for facial skeleton remodeling, improving functional and aesthetic outcomes in children with sincipital and basal encephaloceles.
Abstract:
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 patients with sincipital and 5 patients with basal defects were reviewed retrospectively. The condition was evident at birth in 64% of patients, while the remainder presented with either cerebrospinal fluid (CSF) rhinorrhea, nasal obstruction, or feeding difficulty. Hypertelorism affected 73% of patients. All encephaloceles were repaired transcranially, at a mean age of 2 years, usually by means of an intradural pericranial graft. Five children with gross hypertelorism underwent orbital translocation at the time of encephalocele repair. Of those not corrected, primary and secondary hypertelorism regressed in most instances where 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 recurred in 1 patient after orbital translocation, requiring recorrection 2 years later. One patient with untreated secondary hypertelorism failed to regress after the encephalocele was excised at the age of 4 months. Developmental outcome was normal in 59% of children, whilst 18% have mild mental or physical disability, and 23% are severely impaired. A child with a sincipital or basal defect and mild hypertelorism should have the encephalocele treated in early childhood to allow the facial skeleton to remodel with growth. When an encephalocele is accompanied by gross hypertelorism or a facial cleft, one-stage correction can be undertaken safely in early childhood with minimal mortality and acceptable morbidity.