Extracranial correction of frontoethmoidal meningoencephaloceles: feasibility and outcome in 52 consecutive cases.

BACKGROUND

Although rare in the West, frontoethmoidal meningoencephaloceles are a common problem in Southeast Asia. The patients present with congenital herniation of brain tissue from the anterior cranial fossa through a defect in the facial skeleton. Associated craniofacial deformities include hypertelorism, orbital dystopia, and elongation of the face. The natural history of the disease is progressive, and the life expectancy of affected patients is drastically reduced. Conventional surgical therapy includes extracranial and intracranial approaches with frontal craniotomy or trepanation of the frontal bone.

METHODS

Given a lack of appropriate facilities in a developing country in the Third World, the authors operated on 52 Burmese patients with frontoethmoidal meningoceles using a purely extracranial approach.

RESULTS

Twenty nasofrontal, 16 nasoethmoidal, one nasoorbital, and 15 combined nasoethmoidal/nasoorbital meningoencephaloceles were surgically treated. Early cerebrospinal rhinorrhea was observed in 15 patients, and one of them required operative dural repair by means of an intracranial approach. In the other 14 patients, rhinorrhea resolved spontaneously. None of the patients developed meningitis or other infections of the central nervous system. There were no perioperative or postoperative deaths. Two patients developed secondary healing with exposure of wires or screws.

CONCLUSIONS

Frontal craniotomy is not mandatory in the treatment of frontoethmoidal meningoencephaloceles. Extracranial correction alone is capable of reliably closing the connection between the intracranial and extracranial spaces, and the incidence of early postoperative complications is low. The extracranial approach may be an attractive option in countries where craniofacial expertise is not available and where surgical facilities are limited.