Sincipital meningoencephaloceles (MECs) are rare congenital malformations characterized by the herniation of brain or meningeal tissue through an opening in the anterior floor of the skull base. These malformations always affect the frontal bone, specifically the glabellar region and the naso-frontal angle. A collaboration between M & eacute;decins du Monde and the Children's Surgical Center in Phnom Penh has enabled the treatment of over four hundred cases over twenty years. Patients typically have not undergone radiological examinations, with surgical strategies based on clinical and intraoperative observations. There are several clinical forms of MECs: naso-frontal, naso-ethmoidal, and naso-orbital. The 1972 classification by Suwanwela remains the most relevant. Naso-frontal MECs are often associated with significant skin expansion without major bone deformity, unlike naso-ethmoidal MECs, which lead to significant deformities and are the most common. Naso-orbital MECs, being rarer, most frequently result in an increased orbital volume. The nature of the herniated tissue and the volume of the hernia are critical in determining the surgical strategy. Canthopexy, a key element of reconstruction, must be precise and adhere to strict rules to ensure an aesthetic result. The fronto-nasal remodeling completes the repair. The surgery for MECs follows well-codified principles. The humanitarian context influences management, with an emphasis on surgical safety and skill transmission. Treatment relies more on clinical assessment than radiological, although imaging advancements are now available in Cambodia. Long-term follow-up, especially in children, remains a challenge to document in order to evaluate the impacts on growth and the stability of surgical outcomes. (c) 2024 Published by Elsevier Masson SAS.
Les méningo-encéphalocèles (MEC) sincipitaux sont des malformations congénitales rares qui se manifestent par une hernie de tissu cérébral ou méningé à travers une ouverture dans l’étage antérieur de la base du crâne. Ces malformations touchent toujours l’os frontal, plus précisément la région glabellaire et l’angle naso-frontal. Une collaboration entre Médecins du Monde et le Children's Surgical Center de Phnom Penh a permis de prendre en charge plus de quatre cents cas sur vingt ans. Les patients n’ont généralement pas eu d’examens radiologiques, les stratégies chirurgicales étant basées sur les observations cliniques et peropératoires. Il existe plusieurs formes cliniques de MEC : naso-frontales, naso-ethmoïdales, et naso-orbitaires. La classification de Suwanwela de 1972 reste la plus pertinente. Les MEC naso-frontales sont souvent associés à une importante expansion cutanée sans déformation osseuse majeure, contrairement aux MEC naso-ethmoïdales, qui entraînent des déformations significatives et sont les plus fréquentes. Les MEC naso-orbitaires, plus rares, provoquent le plus souvent une augmentation du volume orbitaire. La nature du tissu hernié et le volume de la hernie sont déterminants pour la stratégie chirurgicale. La canthopexie, un élément clé de la reconstruction, doit être précise et répond à des règles strictes pour assurer un résultat esthétique. Le remodelage fronto-nasal finalise la réparation. La chirurgie des MEC suit des principes bien codifiés. Le contexte humanitaire influence la prise en charge, avec un accent sur la sécurité chirurgicale et la transmission des compétences. Le traitement repose sur une vision clinique plus que radiologique, même si les avancées en imagerie sont aujourd’hui disponibles au Cambodge. Le suivi à long terme, surtout chez les enfants, reste un défi à documenter pour évaluer les impacts sur la croissance et la stabilité des résultats chirurgicaux.
Objective. We developed since 2004 a humanitarian teaching program based on a low cost treatment of frontoethmoidal meningoencephalocele and including an evaluation of our results. This craniofacial malformation is frequent in south East Asia with devastating aesthetic and social consequences for affected children. No cause has been detected to date.Methods. This program was facilitated by two nongovernmental organizations: "Children SurgicalCenter" at Phnom Penh provided the facilities, patients, and local staff and "Medecins du Monde-Operation sourire" provided visiting surgeons twice a year. All operations were free of charge for all patients.Results. Overall, 289 patients were operated (9 years) and 93% of them were seen during follow-up. This follow up remained a challenge because of the local conditions but its strict organization allowed us to show that 77% of the patients were satisfied by the aesthetic results. The most common postoperative issue was a temporary CSF leak. At the end of this program Khmer surgeons were able to treat standard cases without the help of foreigners.Conclusions. This program was selected and totally funded by the "L'Oreal Foundation" and four international papers associating Khmer and French surgeons were published. The development of this humanitarian program allowed several Khmer surgeons to come in France for a complementary teaching program in the fields of Neurosurgery and Facial surgery by a partnership between the Calmette Hospital in Phnom Penh and the CHU of Toulouse.
In Cambodia, spina bifida is rare, but frontoethmoidal meningoencephalocoeles (MECs) are common. Mean life expectancy for patients with congenital MECs may be <20 years, but the complex treatment required has not been available in the country until recently. During visits by combined neurosurgical/craniofacial teams from both Germany and France, a method of repair has been developed that is suitable for the local conditions, affordable and has allowed Cambodian surgeons to learn how to successfully treat MECs. The surgical technique and initial results with 30 patients have been described in a previous publication. This paper presents the outcomes of 128 cases and illustrates that it is cost-effective for these patients to be treated in Cambodia.