Single-Stage Combined Craniofacial Repair for Frontoethmoidal Meningoencephalocele

Frontoethmoidal meningoencephalocele (FMEC) is a rare congenital anomaly characterized by herniation of brain tissue and meninges through a defect in the cranium and associated with facial dysmorphism. Treatment modalities include extra cranial, transcranial, or combined craniofacial approaches. The combined approach is considered the best treatment choice.

Twelve patients with FMEC aging from 6 months to 4 years were treated by single-stage combined craniofacial approach between July 2011 and July 2015. They were followed up for evaluation of outcome and detection of complications.

Seven patients (58.3%) were males and 5 patients (41.7%) were females. Eight patients (66.7%) were less than 2 years while 4 patients (33.3%) were between 2 and 4 years. The main presentations were external mass, telecanthus and hypertelorbitism, radiologically, frontobasal bone defect and herniated dural sac with brain tissue were detected in all patients. Excision of the mass with dural repair, craniofacial reconstruction, and medial canthopexy were done for all patients. Orbital translocation was done for 8 patients (75%), nasal reconstruction for 7 patients (58.3%), while dacryocystorhinostomy in 3 patients (25%). Venticuloperitoneal shunt was done before correction of FMEC in one patient (8.3%). The follow-up period ranged from 6 to 48 months with mean 29.2 months. The esthetic results were satisfactory in 9 patients (75%). Ugly facial scars were recorded in 3 patients (25%).

Early surgical management for FMEC is advisable to avoid deleterious effects on facial growth. Meticulous perioperative care is important for successful surgery. The authors recommend combined craniofacial approach to achieve good outcome and decrease the incidence of complications.