Nov 20 2010
Frontoethmoidal meningoencephalocele is a type of facial malformation that is rarely seen in developed countries, but is quite common in many Southeast Asian countries, occurring in 1 of every 5,000 live births. Most children affected by this condition do not have neurological complications or congenital brain anomalies, and are mentally normal. The malformation, however, often carries with it a social stigma, with many children experiencing ridicule by their peers. The etiology of this condition is not clearly defined and studies have not yielded a consensus, although most have suggested that it is linked to poverty. Past patient studies have suggested that genetic predisposition, higher paternal age, kindred relationship parentage, vitamin B deficiency (due to malnutrition), and drugs may be contributing factors. In Cambodia, more than 75 percent of people live on less than $2 a day and surgery is not affordable without the intervention of humanitarian efforts.
A compelling article published in the Journal of Neurosurgery: Pediatrics, entitled Frontoethmoidal meningoencephalocele: appraisal of 200 operated cases details the experiences of a cross-cultural surgical team from France or Germany and Cambodia who performed humanitarian work from 2004-2009 at Children's Surgical Centre at Kien Khleang, Phnom Penh, Cambodia. While there, they provided no-cost surgery to impoverished children with frontoethmoidal meningoencephalocele (fMEC). Authors are Ngiep Oucheng, MD, (Children's Surgical Centre), Frédéric Lauwers, MD, PhD (Médecins du Monde, Paris, and Maxillofacial Surgery, Hôpital Purpan, Toulouse, France), Jim Gollogly, MD (Children's Surgical Centre); Louisa Draper, MBBS, MA (Children's Surgical Centre); Bruno Joly, MD (Médecins du Monde, Paris); and Franck-Emmanuel Roux, MD, PhD (Médecins du Monde, Paris, and Neurosurgery, Hôpital Purpan, Toulouse, France). The article is published online at: http://thejns.org/doi/full/10.3171/2010.9.PEDS1043.
Children's Surgical Centre is an international non-governmental organization registered in the state of Alaska. In collaboration with the National Rehabilitation Centre at Kien Khleang Hospital, free ophthalmic and reconstructive surgical care is provided to disabled people who would otherwise not be able to get help. A program has been developed to encourage specialist surgeons from overseas to visit the center. Médecins du Monde provides two teams of visiting surgeons (from France and Germany), including a craniofacial surgeon, a neurosurgeon, an anesthetist, and periodically, nurses. The surgical teams visit Cambodia 2-4 times per year with stays of 1-2 weeks per visit to carry out clinical work. "The purpose of this program is twofold - promoting humanitarian efforts in which specialist surgeons perform much-needed surgeries at no cost to patients; and providing training to local medical providers so that they can help patients on a continual basis," said Pr. Roux.
While the authors have published previously on a smaller patient group, they believe that this fMEC case series is the largest published to date. This study analyzes the efficacy of a combined neurosurgical-craniofacial procedure performed in 200 of 257 patients seen over a 68-month period between 2004 and 2009. Of the 57 patients not operated on, intervention was refused or postponed in 51; one went to Singapore for treatment, and five were treated for various reasons via a small transfacial approach. Additional key patient demographics:
•Gender: 108 male, 92 female
•Age: 0-5 (48), 6-10 (44), 11-15 (63), 16-18 (23), older than 18 (22)
•Familial history: two patients
•Forty-six percent (92 patients) had at least one ophthalmological problem
Key operative details and outcome:
•Mean operative time: 2 hours, 35 minutes (range 45 minutes-5 hours)
•Mean hospitalization: 20 days (range 10-60 days)
•Mean follow-up: 11 months (range 2-29 months)
•Most common postoperative complications: CSF leaks and wound infection - 12 percent (24 patients)
•Mortality: three patients, ages 2, 6 and 11 died as a direct result of the surgical procedure; a 14-year-old boy died two days post operation due to malaria with major hyperthermia; and a 12-year-old girl died two months post operation due to acute meningitis.
•Cost: operations were free to patients, estimated overall cost per patient (funded by Médecins du Monde) was $380
•Cosmetic results: utilizing the subjective methods criteria, as judged by surgical team: 145 patients - good, 27 patients - average, 7 patients - poor, and 6 patients - worse. Of the 42 patients in whom no facial incision was required, 38 had good results. Patients and parents tended to be more satisfied with the surgical results than the surgeons.
•Social results: of 182 patients, 25 percent (45 patients) - improved social life, 23 percent (33 patients) - improved educational experience
In the study, the authors noted several points that warrant further discussion. Although there is no recent scientific evidence that shows increased mortality in children with untreated fMEC, most agree that it should be treated as soon as possible to avoid damaging effects on facial growth. Delaying treatment to 8-10 months of age may minimize complications, but in developing countries, since many children are malnourished, treatment should probably be postponed until at least age 1. It is very important to take into consideration local resources and experience with regard to anesthesia.
"Since this condition is generally not considered fatal, extra care must be taken when selecting patients for surgical treatment. Comprehensive information must be provided to patients and their parents about the condition, as well as the risks and benefits of treatment. One of the most rewarding aspects of our humanitarian work was being able to provide these children with an improved social life and educational experience, free from the derogatory name calling so many of them endured. In conclusion, our experience has demonstrated that fMEC surgery can be performed successfully by local surgeons after proper training, with limited surgical materials and a simple infrastructure. This should enable a larger number of patients in developing countries to undergo this surgery," said Pr. Roux.
Source: Children's Surgical Centre at Kien Khleang
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