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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. 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 Médecins 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. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: Reconstruction of large craniofacial defects has largely improved since custom-made implants have been developed in the past decade. For large lesions in fronto-orbital region (such as osteomeningioma), we applied a simple and reliable protocol to perform optimal primary reconstruction with PEEK (polyetheretherketone) specific implant at the same time of the resection. Material and methods: Our protocol is based on virtual preoperative surgery with a planned bone resection that allows engineering of a specific implant to accurately fit to the defect during the surgery. Thus tumour removal and optimal immediate reconstruction are performed easily in a single-step procedure. The use of navigation is required to perform accurate resection according to the planning. We report our experience in five patients requiring complex orbito-frontal reconstruction. Results: Planned resection was always achieved with accurate placement of the implant. Optimal orbital reconstruction is allowed and permits exophthalmos correction and orbital contour symmetry. No major complication was observed. Conclusion: We provide a simple one-step technique to reconstruct the orbit while achieving symmetric cosmetic and functional results, reducing operative time and avoiding donor site morbidity. [Copyright &y& Elsevier]

The aim of our study was to assess the utility and reliability of magnetic resonance imaging (MRI) in antenatal diagnosis of craniosynostosis. We retrospectively reviewed the MRI examinations of the head of 15 fetuses requested over a period of 11 years on the basis of sonographic suspicion of craniosynostosis. The postnatal diagnosis was available for 14 neonates. No termination of pregnancy was performed. There were four neonates with sporadic multisuture craniosynostoses, three of which were syndromic, including one Crouzon and one Pfeiffer syndrome. Eight neonates were normal, two showed cranial vault deformities without synostosis, and one was lost to follow-up. MRI showed a high predictive value for craniosynostosis, as there were no false-negative or false-positive diagnoses. However, the severity of the abnormalities were underestimated in two neonates. We suggest that prenatal MRI has diagnostic value when synostosis is suspected on ultrasonography. Moreover, MRI is accurate in the detection of associated brain abnormalities, which is an important prognostic issue in this diagnosis. Prenatal diagnosis of craniosynostosis is difficult and could benefit from three-dimensional ultrasonography and three-dimensional CT. [ABSTRACT FROM AUTHOR]

Discusses the abstract of the study 'International Multicenter Study of Head Injury in Children,' by K.M. Leung, D. Fong et al, presented at the 6th Annual Scientific Meeting of the Hong Kong Neurosurgical Society held on December 4, 1999.

Craniosynostosis in newborns is caused by the premature closure of the cranial sutures leading to cranial vault deformity. It results in aesthetic imbalance and developmental disabilities and surgery is frequent during the first months of growth. Our study focused on scaphocephaly defined as the premature closure of the sagittal suture. We hypothesised that the effective mechanical properties of sutures were altered as compared to those of the parietal adjacent tissue considered as control. The population consisted of seven males and four females (mean age 4.9 months). Sixteen suture samples and thirty-four parietal tissue samples were harvested during corrective surgery and investigated by using three-point bending tests to obtain the structure-stiffness of specimens. An energy model was used to derive the effective Young's modulus. A histological study complemented the experimental protocol. Fused sutures were thicker than adjacent bone and the natural curvature of sutures did not influence the static mechanical response. The stiffness of stenotic sutures was significantly higher than that of the parietal bone. The effective Young's modulus of stenotic sutures was significantly lower than that of the parietal adjacent tissue. The parietal tissue showed a parallel bone architecture whereas the central stenotic tissue was disorganised with more vascularisation. The stenotic suture differed in structural and mechanical terms from the adjacent bone during calvarial growth in the first year of life. Our study emphasised the alteration of effective tissue properties in craniosynostosis. • Premature closure of sutures causes craniosynostosis and skull deformity. • Fused sutures were stiffer and Young's modules were lower than normality. • Exploration of suture mechanical response may contribute to craniostenosis aetiology. [ABSTRACT FROM AUTHOR]