Secondary craniofacial reconstruction of huge frontoethmoidal encephalomeningocele after primary neurosurgical repair.

Frontoethmoidal encephalomeningocele is a congenital herniation of intracranial contents, including meninges, brain and part of the ventricle, through a bony defect in the skull at the junction of the frontal and ethmoid bones. Management involves meticulous preoperative assessment using computed tomography scans and magnetic resonance imaging, and surgical repair of the central nervous system, skeletal deformities of the orbit, downward displacement of the medial canthi, upward displacement of the eyebrows, and nasal deformities. Frontoethmoidal encephaloceles are best operated on via a craniofacial approach which enables repair of the central nervous system and skeletal deformities in one stage. However, a two-stage reconstruction must be considered when a prolonged operative time is expected or the patient's general condition increases the risks. There have only been a few reports of two-stage reconstructions. We performed a two-stage reconstruction of a huge frontoethmoidal encephalomeningocele, with neurosurgical repair during the first procedure and craniofacial reconstruction during the second procedure. We report on the surgical procedures and the problems encountered.