Frontoethmoidal meningoencephalocele: appraisal of 200 operated cases

Abstract

Object Frontoethmoidal mengingoencephaloceles (fMECs) are frequently observed in Cambodia, especially in poor families. The authors describe issues related to the surgical treatment of fMECs in Cambodia at the end of a humanitarian program that provided surgery free of charge to patients and their families. Methods The authors reviewed 257 cases of fMEC involving patients who presented to their institution, the Children's Surgical Center in Phnom Penh, between 2004 and 2009. They treated 200 of these patients surgically (108 males, 92 females; 89% younger than 18 years) using a “low-cost” management plan with no routine pre- or postoperative investigations. Initially, surgery was performed by visiting foreign surgeons who taught the procedures to resident surgeons. Patients were not charged for consultations or treatment and received at least 1 follow-up examination 6 months postoperatively. Results The nasoethmoidal type was the most frequent fMEC encountered (69%). Many patients had associated ophthalmological issues (46% of cases). Only 1 familial case was detected. Combined neurosurgical and facial procedures were successfully standardized and learned by surgeons initially unfamiliar with fMEC management. A neurosurgical approach avoided the need for a facial incision in 42 cases, improving cosmetic results. The most common postoperative issues were a temporary CSF leak (24 cases [12%]) and/or infection (28 cases [14%]). There were 3 deaths directly related to the operations. Cosmetic results were good in 145 cases, average in 27, poor in 7, and worse than preoperative appearance in 6 patients. Fifteen patients were lost to follow-up. The parents of 87% of the children were rice farmers. Questionnaire results confirmed that fMEC has important social and educational consequences for the affected children and that these consequences can be partially improved by fMEC correction. Conclusions This experience in fMEC management demonstrates that local surgeons can treat these malformations with limited surgical materials and in a nonspecialized infrastructure after principles of treatment have been learned and if they are carefully respected. Surgery for fMEC can thus be more accessible to a larger number of patients in developing countries. Moreover, local treatment facilitates better postoperative and follow-up care.