Frontoethmoidal meningoencephalocele: appraisal of 200 operated cases.

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.

Seasonal variations in frontoethmoidal meningoencephalocele births in Cambodia.

OBJECT: Frontoethmoidal meningoencephalocele (fMEC) is relatively common in many Southeast Asian countries, with devastating aesthetic and social consequences for affected children. No cause has been detected to date. Among other factors, the authors of this paper attempt to identify a statistically significant difference in the spread of fMEC births throughout the year compared with other births. This seasonal variation in the incidence of fMEC births may provide clues to the causes of this condition. METHODS: From a group of 175 children with fMEC who underwent surgery at the authors' humanitarian institution (Children's Surgical Centre) in Phnom Penh between 2004 and 2008, 86 children were studied. These children were born at full term and had an accurately recorded date of birth. The birth dates of this fMEC group were compared with a group of > 15,000 other live births at one of the main maternity units in Phnom Penh in 2005 and 2006. RESULTS: Seasonal variation in incidence of fMEC by month of birth was highly statistically significant (p < 0.001), with the peak of births occurring in the dry season (between March and May). This is in contrast to the control group, in which there was an equal distribution of births throughout the year. More than 85% of the parents of children with fMEC who the authors treated were farmers, but this figure reflects the composition of the Cambodian population. CONCLUSIONS: Uneven spread in the incidence of fMEC births throughout the year suggests that a seasonal factor during the wet season may be suspected in the pathogenesis of fMEC in Cambodia.

Treatment of frontoethmoidal meningoencephalocele in Cambodia: a low-cost procedure for developing countries.

OBJECT: Frontoethmoidal meningoencephaloceles (MECs) are a relatively common abnormality in southeast Asia, with disastrous consequences for the sufferer. In Cambodia, a lack of skilled neuro- and craniofacial surgeons, and the cost of surgery limit the possibilities for appropriate treatment of patients with these lesions. The authors developed a low-cost humanitarian program with the goals of treating frontoethmoidal MECs, ensuring careful postoperative follow-up, and teaching Khmer surgeons how to treat these malformations. METHODS: This program was facilitated by two nongovernmental organizations: Rose Charities Cambodia provided the facilities, patients, and local staff, and "Médecins du Monde" provided visiting surgeons and anesthesiologists. All operations were free of charge for all patients. A strict follow-up program was organized to evaluate the surgical results, the social impact of the surgery, and the satisfaction levels of the children and their parents. RESULTS: Forty-five children and seven young adults with MECs were treated using a rather simple surgical technique. Of the three types of MECs encountered, the most frequent was the nasoethmoidal type (43 cases). The most common postoperative issue was a temporary CSF leak (in 16 patients). Cosmetic results were considered excellent or good in 40 patients, average in nine, and poor in one; two patients were lost to follow-up. The overall cost of each operation was estimated to be $380 (US dollars), far less than a standard MEC operation would cost in a more developed country. At the end of this humanitarian program, Khmer surgeons were able to treat standard cases of frontoethmoidal MECs without the help of foreigners. CONCLUSIONS: Patients in developing nations who have limited access to standard neurosurgical care can be treated for frontoethmoidal MECs with few complications and a satisfactory cost-to-benefit ratio.