[Massive lytic osteodystrophy or Gorham-Stout disease of the craniomaxillofacial area]. / L'ostéodystrophie lytique massive ou maladie de Gorham-Stout à localisation craniomaxillofaciale.

Gorham's disease also called idiopathic massive osteolysis represents a rare affection with unknown aetiology. This disease was described in different bones of the body but its location on maxillo-facial skeleton is particular by the number of cases in the world (only 32), by morphological, functional consequences and the prognosis. The authors describe a new case of male teenager (17 years old) who had a massive osteolysis of mandibular ramus, upper maxillary, malaire, sphenoid, temporal and occipital left bone. The authors give precision about epidemiological data, methods of diagnosis and therapeutic procedures.

Massive osteolysis of the maxillo-facial bones: case report and review of the literature.

An unusual case of massive osteolysis destroying the left side of the mandible, the maxilla, the orbit, the cranium bones and the upper cervical spine is reported. The evolution of this disease was observed over a period of 4 years. The literature was reviewed, only 39 cases have been found involving the maxillo-facial bones. In our case, attempts at surgery was without success. However, further radiotherapy with 35 Gy controlled the progression of this osteolysis.

[An ophthalmological complication of cherubism]. / Une complication ophtalmologique du chérubisme.

INTRODUCTION: Cherubism is a rare familial fibro-osseous lesion usually observed in children. The diagnosis is clinical and radiological. Outcome is generally favorable, but with a risk of ophthalmologic complications, especially lower eyelid retraction, proptosis, diplopia, displacement of eyeball and loss of visual acuity. Clinicians must be aware of these complications for proper detection and specific treatment. OBSERVATION: We report a case of cherubism observed in a 10-year-old boy who presented exophthalmia with reduced visual acuity on the right due to reduction of the volume of the orbital cavity and compression of the optic nerve. Partial maxillectomy was required for decompression. DISCUSSION: Cherubism can lead to various types of ophthalmologic complications. Exophthalmos and loss of visual acuity due to compression of the optic nerve are the most common. Surgery is generally not required, but can become crucial in selected patients.

[Temporomandibular osteomyelitis secondary to progressive necrotizing otitis externa]. / L'ostéomyélite temporomandibulaire secondaire à une otite externe nécrosante progressive.

INTRODUCTION: Osteomyelitis of the temporomandibular joint is a rare complication of invasive external otitis, often occurring after inappropriate initial treatment of the causal affection. OBSERVATION: A 65-year-old diabetic man was treated for invasive otitis externa complicated by destructive osteomyelitis of the ipsilateral temporomandibular joint. The diagnosis was suspected clinically and confirmed by imaging. The course was favorable with medical treatment. DISCUSSION: Osteomyelitis is an extremely unusual complication of invasive otitis externa. Diffusion of the infection toward the temporomandibular joint can be secondary to an osteitis of the bony canal or spread via congenital dehiscences of the external auditory canal. Pain, limitation of mouth opening and auricular swelling are the main clinical signs. Treatment is essentially medical, based on adapted antibiotic therapy.

[Myxoma of the upper jaw. Apropos of a case]. / Myxome du maxillaire supérieur. A propos d'un cas.

We report a patient with odontogenic myxoma of the upper maxillary. The authors underline for this particular case, that there are no clinical features specific of the tumor; the diagnosis can be only histological. The evolution of this tumor is always benign.

[Chondrosarcoma of the jaw. A case localized to the mandible]. / Chondrosarcome des os maxillaires. Une localisation mandibulaire.

Chondrosarcoma is a malignant cartilaginous tumor rarely found in the jaw bone. Tumor progression is slow with strong propensity to local recurrence after surgical removal. The prognosis is poor and depends on the degree of differentiation and the quality of the resection. We report a case of mandibular chondrosarcoma that exhibited particularly massive extension to soft tissue. We discuss the epidemiology, diagnosis, treatment and follow-up of these tumors.

[Osteoradionecrosis of the jaws. Apropos of 7 cases]. / L'ostéoradionécrose des maxillaires. A propos de 7 cas.

Jaw osteoradionecrosis (ORN) is one of the most serious complications of cervico-facial cancer irradiation. Severity is related to major bone lysis and the difficult medical and surgical treatment. We report our experience in seven cases over a ten-year period. Mandibular localization was predominant (85%). The mean age of the patients was 36 years. The average delay to development of ORN was 5.5 years. The treatment was medico-surgical, based on wide surgical exeresis (6/7), frequently via endobuccal surgical approach (4/7). Prevention remains the best treatment of ORN associated with an accurate management of all patients who need radiotherapy.

[Central giant cell reparative granuloma of the jaw]. / Le granulome réparateur central à cellules géantes des maxillaires.

The authors report a series of 22 cases of central giant cells Granuloma of the jaws. There were 12 located in the maxilla, and 10 in the mandible. The majority of lesions were observed in patients under the age of 30 years, and occurs more often in females than in males, particularly in mandibular lesions. This lesion, which the pathogenesis still remains obscure, appears as radiolucency, well or ill-defined, uni or multilocular with trabeculations coursing through the lesion. Histologic study shows giant cells, fibroblastic cells, and foci of hemorrhage and osteoid tissue. The authors insist on the problem of differential diagnosis which can make a radiolucency with giant cells. It must be differentiated especially from giant cells tumor, which treatment and course are different. Finally, the authors underline a good prognosis of this lesion, which simple curettage is generally sufficient, and which recurrences remain rare.

[Osteosarcoma of the jaws. Apropos of 12 cases]. / Les ostéosarcomes des maxillaires. A propos de 12 cas.

We report 12 cases of osteosarcoma (10 located in the mandible and 2 in the maxilla). There were 8 men and 4 women who were older than usually observed in osteosarcoma at other sites (8 of 121 patients were over 30 and the average age was 34 years). Clinical signs were variable. Bone tumefaction was the most frequent sign, with a diameter greater than 5 cm in most cases (10 out of 12). Surgery alone was used for 8 patients and radiosurgery for 3. Follow-up is available for 8 patients. All presented local recurrence with extensive tumors progression. All died within an interval of 3 to 30 months despite palliative surgery and/or radiotherapy or chemotherapy.