Management of the Frontoethmoidal Encephalomeningocele

INTRODUCTION: The Frontoethmoidal encephalomeningocele (FEEM) is a congenital neural tube anomaly, with herniation of intracranial material such as the brain and leptomeninges through a defect of the dura and anterior skull base at the junction of the frontal and ethmoidal bones. It may result not only in neural defects, sensorimotor deficits, neurological morbidities, visual impairment, impaired nasal function, and a potential risk of intracranial infection, but also in significant craniofacial disfigurement with complex deformities in the frontal, orbital, and nasal regions. MATERIALS AND METHOD: The standard two-staged surgical protocol comprises of the first stage performed by a neurosurgeon, which aims at correcting the neural defect by a formal craniotomy; then the second stage performed by a craniomaxillofacial or plastic and reconstructive surgeon, to correct craniofacial hard and soft tissue deformities. The case discussed was managed using a modified intracranial-transcranial single stage approach, achieving both the desired objectives. RESULTS AND CONCLUSION: This protocol elucidates the importance and value of teamwork between the Neurosurgeon and Craniomaxillofacial surgeon, in comprehensively and efficiently managing small to moderately sized FEEMs, assuring their complete elimination, satisfactory defect closure, effective functional treatment as well as esthetic correction and reconstruction of the attendant craniofacial deformities by means of a single-stage definitive surgical approach.