[Möbius syndrome]. / Le syndrome de Möbius.

Möbius syndrome is a rare congenital disease characterized by facial and abducens nerve palsy. Children are unable to smile, frown, suck, grimace, blink their eyes, and to move their eyes laterally. The aim of this study was to analyze the diagnosis and treatment of this disease. Maxillofacial examination reveals: facial diplegia, retrognathism, palatine and dental malformations. Möbius syndrome is usually associated with multiple cranial nerve involvement, limb or orofacial malformation, and Poland syndrome. Although the pathogenesis of the syndrome is unclear, a number of mechanisms have been suggested including vascular and genetic hypotheses. Symptomatic treatment is used to manage this syndrome. The diagnosis of Möbius syndrome may be difficult in some patients with atypical signs of facial diplegia and other cranial nerve palsies, it requires a multidisciplinary approach.

[An unusual foreign body in the orbit]. / Un corps étranger orbitaire insolite.

INTRODUCTION: Foreign bodies in the orbit are rare. They can generate more or less serious complications depending on their nature and size. We report an exceptional case of a bulky foreign body in the orbit (the tip of a pen), which did not lead to any complication. OBSERVATION: A 13-year-old child presented with a right orbital trauma caused by a pen. He consulted 3 months later when a small palpebral swelling appeared. The CT scan showed the presence of a foreign body on the orbital floor. Wound debridement allowed extracting the tip of a pen measuring 3.5cm without any complication. There were no postoperative complications. DISCUSSION: The originality of this observation is two-fold; the singularity of the foreign body and its total harmlessness in spite of its large size. However, orbital trauma and a secondary orbital syndrome must lead to emergency imaging.

[Maxillary cementoblastoma. A case report]. / Cémentoblastome maxillaire. A propos d'un cas.

Cementoblastoma is a rare benign odontogenic neoplasm. Its cause is unknown. It represents less 6 % of all odontogenic tumors. The aim of our work is to present a rare case of maxillary cementoblastoma involving an included central incisor. A 32-year old man consulted for a left maxillary swelling to projection under nostril. A panoramic radiographic examination revealed an included tooth 21 with a radiopaque lesion around its root. The computed tomography revealed the included tooth 21 with a process around its root. This process is well-defined, high-dense and is surrounded by a radiolucent halo. The treatment should consist of complete removal of the lesion with the tooth 21. Histological examination concluded to the diagnosis of a cementoblastoma. The cementoblastoma occurs most frequently in young persons and predominantly among men. The mandibular premolar and molar are the more frequently reached. The maxillary localization remains rare. This tumor occurs around the roots of the posterior and lower teeth. Radiographically, the lesion is well-defined and attached to the root of the tooth, radiopaque dense and surrounded by a radiolucent halo. The cementoblastoma evolves slowly and has the tendency to blow the cortical. The prognosis is good.

[A maxillary lip bumper used to manage labial self-mutilation]. / Utilisation du lip bumper ou pare-choc labial dans la prise en charge d'une autophagie labiale.

INTRODUCTION: The repair of facial self-mutilation often fails if the reconstruction is not protected. The authors report a case of a lip self-mutilation treated by an orthodontic device: the "lip bumper". OBSERVATION: A four-year-old patient presented with self-mutilation of the lower lip in a context of multiple malformations. The defect concerned two third of the lower lip. The treatment combined antibiotics, local care, psychiatric management and a lip bumper. The evolution was favorable without need for secondary reconstruction. DISCUSSION: The lip bumper is a simple orthodontic device. It pushes the lower lip away from the dental arch. It is used to stop the pattern "agitation-mutilation-infection".

[Cheek paraganglioma: a rare location]. / Paragangliome jugal: une localisation rare.

OBSERVATION: A 29 year-old-woman presented with a massive painless slow-growing tumor of the right cheek, with a benign aspect on clinical examination. The CT scan with injection, showed a solid-cystic mass, well defined, and partially enhanced. The mass was easily removed with a complete endobuccal excision. The histological examination and immunohistochemical study revealed a paraganglioma. There was no postoperative complication. No recurrence was noted after six months of follow-up. DISCUSSION: The paraganglioma is a rare neuroendocrine tumor and its location in the cheek has never been reported.

[Bilateral congenital macrostomia]. / Macrostomie congénitale bilatérale.

INTRODUCTION: Congenital macrostomia, a transverse facial cleft, is a rare deformity of the mouth, which can occur alone or in association with other deformities. We report a case of congenital bilateral macrostomia. We discuss the difficulties of plastic surgery in this pathology. OBSERVATION: Congenital macrostomia in a one-year-old girl compromised feeding. The patient presented an isolated bilateral transverse facial cleft. Surgery associated a suture of the orbicularis oris muscle and a cutaneous W plasty. At twelve months, follow-up has been uneventful. DISCUSSION: Congenital macrostomia is most commonly associated with others anomalies. Many procedures are described regarding surgical correction of macrostomia. Reconstruction of the integrity of the oral sphincter associated with W plasty usually gives the best results.

Osteomyelite mandibulaire chronique primitive de l'enfant a propos d'un cas et de revue de litterature

L'osteomyelite mandibulaire chronique primitive (OCP) non suppurative est rare et de cause inconnue. Les auteurs decrivent un cas rare d'osteomyelite mandibulaire chronique primitive chez une fille de 6 ans; l'examen histologique a permis de confirmer le diagnostic. Jusqu'a present; seuls quelques cas de cette affection; dont le debut se situe dans l'enfance ou l'adolescence; ont ete decrits. Le traitement doit etre conservateur surtout chez l'enfant. L'association chirurgie; antibiotherapie reste le traitement de choix. L'oxygenotherapie hyperbare est indiquee dans les formes refractaires. Les anti-inflammatoires non steroidiens donnent de bons resultats surtout dans les formes primitives. Le traitement passe d'abord par la prevention et la bonne hygiene bucco-dentaire

Myxome du maxillaire

Le myxome est une variete rare des tumeurs benignes des maxillaires; dont l'origine serait le mesenchyme embryonnaire du follicule dentaire. Les manifestations cliniques et radiologiques sont variables et non specifiques et peuvent preter confusion avec d'autres lesions. Nous decrivons un cas de myxome odontogene du maxillaire tres envahissant; observe chez une jeune fille de 13 ans. Cliniquement; l'enfant presentait une tumefaction maxillaire gauche; indolore et ferme a la palpation. Une TDM du massif facial montrait un processus expansif osteolytique soufflant le maxillaire. Deux biopsies tumorales ont ete en faveur d'un myxome odontogene. Le traitement a consiste en une exerese large de la tumeur par voie endobuccale; la tumeur s'individualise bien du plan osseux. L'examen anatomopathologique de la piece d'exerese a pose le diagnostic de myxome. L'evolution a court terme n'a pas montre de recidive locale clinique et radiologique. Le diagnostic s'est base sur des arguments cliniques; radiologiques et histologiques. Cette tumeur est localement agressive; pouvant entrainer d'importantes deformations faciales et des perturbations dentaires. Le caractere recidivant du myxome impose un traitement radical large depassant les limites de la lesion. La perte de substance importante impose une reparation chirurgicale ou prothetique

[Apropos of an uncommon disease of the larynx: pyolaryngocele]. / A propos d'une pathologie rare du larynx: la pyolaryngocèle.

The laryngopyocele is a rare complication of laryngocele. About one case of laryngopyocele and review of literature, the authors recall the main characteristics of this affection. Its clinical picture is often alarming. The CT scan, not often made emmergenceley, give a characteristic image. The treatment is mainly surgical but also endoscopic, then facilitated by the use of laser. The fact of thinking to this diagnosis toward a laryngeal dyspnea with fever permit sometimes to avoid tracheotomy then usually made emmergencely.

[Ossifying fibroma of the maxilla (apropos of 2 cases)]. / Les fibromes ossifiants des maxillaires (à propos de 2 cas).

The ossifiant fibroma is an authentic scarce benign tumoral processus. According to a literature review and two recent cases taken in charge in our service, having stand diagnostical and therapeutical problems. They seem interesting to accost the subject of the ossifiant fibroma in maxillo-facial surgery and put the accent on the differential diagnosis in histological plane between ossifiant fibroma and fibrous dysplasia having given different therapeuticals indicents. The two reported cases interest the masculine young subjects in one case with mandibular localisation and in the other one maxillary.

[True osteoma of the maxilla. Apropos of 3 cases]. / Les ostéomes vrais des maxillaires: à propos de 3 cas.

The authors report 3 cases of authentic maxillary osteoma. Two cases concerns an exceptional osteoma with multiple localisation and the third case a giant maxilla osteoma. Topographic, diagnostic and therapeutic notions of these tumors are related.

[Ethmoid-frontal mucocele. Apropos of 15 cases from 1985 to 1990]. / Les mucocèles ethmoïdo-frontales (à propos de 15 cas, 1985-1990).

Between 1985 and 1990, 15 ethmoid frontal mucoceles were collated. All our patients consulted us at the exteriorization stage. A CT-scan is the ideal examination, making it possible to assert the diagnosis and to individualize the bone destruction and the neighbouring tissue repression. Trans-ethmoid or trans-nasal marsupialization by an external or endonasal approach provided excellent results after a 1 to 5 year follow-up period.