[Congenital insensitivity to pain: difficulty of management]. / L'insensibilité congénitale à la douleur : difficultés de prise en charge.

INTRODUCTION: Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare disorder, most often of genetic origin. CASE REPORT: The authors present the case of two siblings, 10 and 13 years old, both followed-up since the age of 2 for CIPA diagnosed after discovering insensitivity to pain during iterative falls, burns, and of severe oro-digital self-mutilating behavior. Sural nerve biopsy and an electromyogram confirmed the diagnosis. DISCUSSION: CIPA with anhidrosis is a very rare disease. It is characterized by unexplained fever episodes, anhidrosis, pain insensitivity, self-mutilating behavior, and sometimes mental retardation. Complications of this insensitivity (non-treated fractures, burns, and oro-digital mutilation) may be lethal. Treatment remains preventive. The patient must observe a very strict hygiene. Prevention for maxillofacial involvement consists in breaking the cycle of oral self-mutilation.

[Giant pleomorphic adenoma of the parotid gland]. / Adénome pléomorphe géant de la parotide.

INTRODUCTION: Slow evolution pleomorphic parotid adenoma may be diagnosed late. These giant tumors have a functional, esthetic, and social impact. They raise prognostic issues because of the risk of degeneration. We present a case of a giant pleomorphic adenoma involving the parotid glands and try to explain the reasons for the diagnostic delay and describe therapeutic specificities. CASE REPORT: A 54 year-old male patient consulted for swelling of the parotid region slowly evolving over the last 10 years. This lesion had been unsuccessfully treated by conventional methods. A painless 20cm long plurinodular mass was located in the right lateral cervical region. Its consistence was heterogeneous. The CT scan revealed a heterogeneous tumor with hyperdense and hypodense areas without any associate lesions. A total conservative parotidectomy was performed; the anatomopathological examination confirmed the diagnosis of a pleomorphic adenoma, weighing 1.2kg for a diameter of 19cm, without malignant degeneration. The postoperative evolution was uneventful. DISCUSSION: The recommended treatment for giant parotid adenoma is total conservative parotidectomy. Besides its functional and esthetic impact, the giant parotid adenoma is at high risk of malignant degeneration. Documentation on the nature of the lesion, its evolutive potential, and an early surgical management could limit its incidence.

Plemorphic adenoma of the infratemporal space: a new case report.

Plemorphic adenoma is a frequent benign tumor of the major salivary glands. It could also develop from accessory salivary glands. We are reporting an extremely rare case of pleomorphic adenoma developing from the infratemporal space. The final diagnosis was based on histological confirmation. The treatment was mainly a complete resection via an anterior transmaxillary approach. Diagnosis, clinical behaviour, and treatment of pleomorphic adenoma of the infra-temporal space are reviewed from the literature.

[Compressive retropharyngeal hematoma in a hemophiliac patient]. / Hématome compressif rétropharyngé chez un patient hémophile.

INTRODUCTION: The retropharyngeal hematoma is rare and usually due to trauma, anticoagulation therapy complication or ascending aortic dissection. This presentation is extremely rare in hemophiliac patients. OBSERVATION: A 23-year-old hemophiliac patient presented with a spontaneous onset large retropharyngeal hematoma extended to the floor of the mouth associated with dyspnea, dysphagia and dysphonia. The patient underwent adequate and successful medical treatment. DISCUSSION: Hemophilia A is characterized by a deficit in factor VIII. Clinical symptoms are not specific and vary with the level of the intrinsic factor. Hematoma of the cervical region is a rare but potentially life-threatening event. The treatment requires transfusion of the specific factor and education of the patient and his relatives.

[Cervical Pott's disease revealed by retropharyngeal abscesses]. / Abcès rétropharyngés révélant un mal de Pott cervical.

INTRODUCTION: Retropharyngeal abscesses are exceptional in adults. The etiologies are numerous, cervical spine tuberculosis is one of them. OBSERVATION: We report two cases of cervical Pott's disease revealed by a retropharyngeal abscess. The clinical presentation was non-specific, dominated by oropharyngeal obstruction. Radiological findings suggested the diagnosis, showing a retropharyngeal collection with vertebral osteolysis. The bacteriological and histological assessment confirmed the diagnosis. The evolution was favorable after treatment by antituberculosis drugs. DISCUSSION: Vertebral tuberculosis is rare. Cervical involvement is exceptional, and retropharyngeal abscesses can be the revealing feature of this condition. Symptoms are not specific. The diagnosis is based on radiological and bacteriological assessment. Treatment with antituberculosis drugs leads to a good outcome.

[Primary tuberculosis of the parotid gland]. / Tuberculose primaire de la glande parotide.

OBJECTIVES: To report on a case of primary tuberculosis of the parotid gland in a 13-year-old child, and to stress the corresponding diagnostic pitfalls. CASE REPORT: A 13-year-old child presented with an isolated mass of the right parotid area. Radiological findings were in favour of a cystic lesion. Skin tuberculin test was positive. Bacteriological and histopathological examinations were realized after spontaneous fistulization of the mass, and allowed the diagnosis of tuberculosis of the parotid gland. Evolution was favourable under antituberculous chemotherapy. DISCUSSION: Localization of tuberculosis in salivary glands is rare. Diagnosis is difficult because there are no specific clinical, radiological or biological signs of the disease. Only bacteriological and histopathological findings can confirm the diagnosis. Fine needle aspiration cytology is very helpful, and permits to avoid abusive surgery. Treatment is based on antituberculous drugs and allows usually a favourable evolution.

[Cranio-cervical junction malformations: a rare cause of bilateral vocal cord paralysis]. / Les malformations de la jonction craniocervicale: une cause rare de diplégie laryngée.

OBJECTIVES: To report a rare and reversible cause of bilateral vocal cord paralysis in an adult. MATERIAL AND METHODS: A 44 years old woman has consulted for a laryngeal stridor with hoarseness. This symptomatology was preceded by chronic headache and dizziness. The examination noted bilateral vocal cord paralysis in paramedian position, and a cerebellum syndrome. The patient underwent urgent tracheotomy. RESULTS: A magnetic resonance imaging was performed for the etiologic research, and revealed a Chiari I malformation with a compression of the bulb. Neurosurgical decompression permitted resolution of the symptoms, and selvage of the tracheotomy was possible 2 months after surgery. CONCLUSIONS: Bilateral vocal cord paralysis is rare on Chiari I malformation. It's secondary to a direct compression or an ischemia of the brain stem. The oto-rhino-laryngologists have to know this etiology to make the diagnosis early and permit a neurosurgical treatment.

[Cervicofacial cellulitis revealing cutaneous lymphomas]. / Dermohypodermites cervicofaciales révélatrices de lymphomes cutanés.

INTRODUCTION: The cervicofacial localization of cutaneous lymphomas is rare. These lymphomas usually present as a long-lasting and treatment-refractory papule or nodule. Lymphomas can also be revealed by cervicofacial cellulitis. CASES: We report 2 cases of cervicofacial cellulitis revealing a cutaneous lymphoma. The diagnosis was proved by multiple biopsies, performed because there was no clinical improvement in spite of an aggressive and adequate antibiotherapy. Our 2 patients were treated by radio and chemotherapy. DISCUSSION: Cutaneous lymphomas are lymphocytic proliferations stemming from cutaneous lymphoid tissue, without nodal, medullary, or visceral localization. Their clinical presentation is quite polymorphic, and cellulitis is one of the modes of revelation, especially forehead and neck localization. They have no portal of entry and are resistant to treatment. The diagnosis relies on histology, and biopsies must be performed if there is a suspicion of lymphoma. The treatment is radio and chemotherapy, and the evolution depends on the tumoral stage.

[Solitary bony plasmocytoma of the mandible]. / Plasmocytome solitaire osseux de la mandibule.

INTRODUCTION: Solitary plasmocytoma is a rare malignant tumor, belonging to the larger family of plasma cell proliferative diseases observed in bones. The mandible localization reported here is extremely rare. CASE REPORT: A 57-year-old man consulted for a tumefaction of the right mandible. Physical examination revealed a mandibular tumor with an osseous consistency covered by a healthy mucous. Radiologically, the tissue processed from the mandible, which presented a defect and an eroded cortical. Hemimandibulectomy was performed. Pathologic examination of the operative specimen demonstrated plasmocytoma. Complementary radiotherapy was performed. Outcome at 13 months was favorable, without recurrence of multiple myeloma. DISCUSSION: Plasma cell tumors are rare, about 10% of them are solitary plasmocytomas observed in intra- or extramedullar sites. Pathology is required for confirmation. An exhaustive assessment is needed to rule out multiple myeloma. Most solitary forms are observed in the spine. Appropriate treatment combines surgery and radiotherapy. Careful surveillance is required because of the frequent progression to multiple myeloma.

[Mesenchymal chondrosarcoma of the mandible]. / Chondrosarcome mésenchymateux de la mandibule.

INTRODUCTION: Mesenchymal chondrosarcoma is a rare form of chondrosarcoma and mandibular localization is rare. OBSERVATION: We report a case of mesenchymal chondrosarcoma of the mandible in a 23-year-old patient who consulted for a voluminous tumor of the right mandible. Radiological findings suggested a malignant tumor. A hemimandibulectomy and a pelvectomy were performed. The histological assessment showed zones of neoplastic cartilage and small ovoid cells, typical of mesenchymal chondrosarcoma. DISCUSSION: Mesenchymal chondrosarcoma is a rare malignant tumor of skeletal and extra skeletal origin. The most frequent localizations are the femur, the ribs, and facial bones. Histologically, these tumors have a typical biphasic pattern consisting of both small cells and islands of atypical cartilage. Because of its rich vascular component, this lesion has often been confused with hemangiopericytoma. Surgery is the usual treatment. The prognosis is very poor with a high risk of relapse and metastasis.

Hydatid cyst of the neck.

Hydatid disease located in the head and neck is uncommon, and hydatid cysts rarely present as a cervical mass. We report an unusual case of primary hydatid cyst arising in the soft tissues of the neck. The clinical presentation was non-specific. The diagnosis was suspected by the radiological findings, and confirmed by histopathology. Characteristics of this rare disease, its diagnostic difficulties, and treatment are discussed.

Ectopic lingual thyroid.

Ectopic lingual thyroid is an uncommon embryological aberration characterised by the presence of thyroid tissue located in a site other than the normal, pretracheal region. The tongue is the most frequent ectopic location of the thyroid gland; the clinical incidence of lingual thyroid varies between 1:3000 and 1:10,000. We present a new case of lingual thyroid in a 10-year-old child who presented dysphagia. The radiological findings and the biopsy confirmed the diagnosis. As the mass was well tolerated, surgery was not indicated. At this time, there are no signs of complications or malignant transformation.

[Association of Bezold's abscess and sigmoid sinus thrombosis. A case report]. / Association d'un abcès de Bezold et d'une thrombophlébite du sinus sigmoïde. A propos d'un cas.

Bezold's abscess and sigmoid sinus thrombosis are both classic complications of otitis media. However, since the introduction of antibiotics both have become very rare. Very few cases of these complications occurring concomitantly have been reported. We describe the clinical case of a 45 year old male with a 10 year history of neglected right ear cholesteatoma. The patient presented to our department with a 10 day history of purulent right otorrhea, otalgia, increasing headach, pyrexia and suppurating right parapharyngeal mass. CT scan confirmed right middle ear cholesteatoma associated with ipsilateral Bezold abscess and and sigmoid sinus thrombosis. Treatment was by mastoidectomy, broad spectrum intravenous antibiotics and anticoagulation. The patient made a full uncomplicated recovery. The association between chronic middle ear disease, sigmoid sinus thrombosis and Bezold abscess are discussed.

[Giant form of infantile myofibromatosis located on the jaw]. / Forme géante de myofibromatose infantile localisée à la joue.

INTRODUCTION: Infantile myofibromatosis is a rare fibrovascular-like tumour, characterized by the development of single or multiple nodular lesions arising from cutaneous, subcutaneous, muscular bone or visceral structures, diagnosed before 2 years. OBSERVATION: We report a case of infantile myofibromatosis located on the jaw, which is unique because of its large size (12 cm), its location and its neonatal presentation. It was a voluminous proliferate tumour with an ulcerated centre, located on the left jaw. Surgical excision was complete and the diagnosis was maded on histological examination. Recovery was uncomplicated with no recurrence on follow up. DISCUSSION: Diagnosis of infantile myofibromatosis is difficult because of the clinical heterogeneity and the histopathological appearance. The histological diagnosis relies on identification of two separate components, fascicular myofibroblastic at the periphery and hemangiopericytome in the centre. The most freqaent treatment is conservative surgical excision, because recurrence rates are low and there is a possibility of spontaneous regression. Some authors recommend conservative management of very large or multiple lesions particularly if excision will result in significant functional or cosmetic morbidity.

[Primary sub-mandibular gland tumours: experience based on 68 cases]. / Les tumeurs primitives de la glande sous-maxillaire: à propos de 68 cas.

UNLABELLED: Sub-mandibulary gland tumours are less common than tumours of the parotid and pose many clinical and therapeutic challengers. OBJECTIVE: We report our experience of sub-mandibular tumours and review the literature. PATIENTS AND METHODS: Retrospective studies of sub-mandibular gland tumours presenting to our department between 1986 and 2000. RESULTS: 68 cases were reviewed comprising 37 benign and 31 malignant tumours (15 females and 33 males). Average age of patient was 46 years and all presented with a sub-mandibular swelling. Clinical suspicion of malignancy was associated with symptom of pain, cervical adenopathy, nerve palsy, skin and/or bone invasion. CAT Scans were performedd to assess tumour extent / invasion. Definitive diagnosis was by complete excision and pathological examination. Pleomorphic adenoma (n= 32) were the most frequent benign tumours. For malignant lesions (n= 31) the most frequent were: Adenoid cystic carcinoma (n= 10), epidermoid carcinoma (n= 5), adenocarcinoma (n= 5), mucoepidermoid carcinoma (n= 3), malignant non Hodgkinien lymphoma (n= 5). Treatment was by total surgical excision of the submandibular gland for the begnin tumours. For the malignant lesions it was associated acording to their extension with other anatomical region or in case of adenopathy with neck dissection. Radiotherapy was performed in 24 cases and chemotherapy in 10 cases. CONCLUSION: Malignity in sub-mandibular gland tumours is more frequent than in the parotid gland. Any delay in diagnosis or inappropriate management may result in a poor prognosis for the patient.

[Bronchogenic cyst of the tongue]. / Kyste bronchogénique de la langue.

OBJECTIVES: Bronchogemc cyst is a rare aberration secondary to abnormal detachment of accessory lung buds from the primitive foregut. Through one case of bronchogenic cyst of the tongue, the authors made a review of this exceptional localisation. MATERIAL AND METHODS: We report one case of bronchogenic cyst of the tongue treated in the Ibn Sina Hospital. RESULTS: Our patient was a new born admitted in our formation for a voluminous cystic mass ot the tongue, responsible of difficulty for mouth occlusion and feeding The radiology I confirms the liquid nature of this mass. The treatment was surgical, and the diagnosis of bronchogenic cyst was histological. CONCLUSION: Bronchogenic cysts are generally intrathoracic. The localization in the cervico-facial area is rare, and only 4 cases of bronchogenic cyst of the tongue were described in the literature. The clinical presentation is non specific, depending on the volume of the cyst. Radiology permits to confirm the nature of the mass. The certitude diagnosis is histological: bronchogenic cysts are lined by pseudostratified ciliated columnar epithelium with accessory tissues in the cyst wall, smooth muscle, seromucinous glands and cartilage, simulating a bronchus. Complete surgical excision is the base of the treatment.

[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.

[Mastoid eosinophilic granuloma: a case report]. / Le granulome éosinophile de la mastoïde: à propos d'un cas.

OBJECTIVES: Langerhans cell histiocytosis is a proliferating cell disease that may take various forms characterised by bone, skin lymph nodes and visceral lesions. Eosinophilic granuloma is a localised form of histiocytosis X, or Langerhans' cells histiocytosis, a benign form with unknown aetiology. At the head and neck level, it is usually located on the temporal, and usually occur in association with multifocal disease; however, isolated lesions may occur in the mastoid bone alone. METHODS: The author's present a case of a patient with unifocal eosinophilic granuloma limited to the mastoid treated in ENT and radiotherapy departments. RESULTS: CT scan demonstrated an osteolytic lesion of the mastoid. The biopsy and immunohistochemical study confirmed the diagnosis. A surgery followed by a radiotherapy (20 Gy) have stabilised the situation. There is no recurrency after one year. CONCLUSION: Unifocal eosinophilic granuloma of the temporal bone is a benign lesion of langerhans' cell histiocytosis. Its diagnosis is difficult when the disease remains isolated. Its treatment depend on its extension and its risks. The excellent prognosis does not dispense on regulary follow up.