Maxillary bone myxoma.

INTRODUCTION: Maxillary bone myxoma is a rare benign mesenchymal tumor, slow-growing but locally aggressive. Pathogenesis remains disputed. OBJECTIVE: To study the clinical, radiological and histological features and treatment of maxillary myxoma, based on a pediatric case report. CASE REPORT: An infant of two and a half months presented with endonasal tumor extending to ethmoid. Surgical excision was performed on an endonasal approach. Myxoma was diagnosed by histologic examination of the surgical specimen, whereas initial biopsy had suggested fibrous dysplasia. No recurrence was observed after two and a half years' surveillance. CONCLUSION: Positive diagnosis of maxillary myxoma is histological. Treatment is primarily surgical. Strict long-term surveillance is required because of the high risk of recurrence.

Les kystes amygdaloides : a propos de 31 cas

Introduction: Le kyste amygdaloide est une tumeur kystique latero-cervicale haute rare; issue de la 2eme fente branchiale. Il represente 2 des tumeurs latero-cervicales du cou; et 6;1a 85;2 des anomalies de la deuxieme fente. La forme oropharyngee est tres rare. But : Analyser les caracteristiques anatomo-cliniques et discuter les modalites de prise en charge et les indications therapeutiques de cette affection. Methode : etude retrospective menee sur une periode de 10 ans (2000- 2009) a propos de 31 cas de kystes amygdaloides colliges au service d'ORL et de chirurgie cervico-faciale de La Rabta. Resultat : L'age moyen de nos patients etait de 28 ans et 7 mois avec un sex-ratio de 0;47. La duree d'evolution etait de 14 mois. L'examen physique a trouve une masse submandibulaire dans 2 cas; retro-angulo-mandibulaire dans 2 cas et jugulo-carotidienne dans 27 cas. Vingt neuf patients ont eu une echographie cervicale ayant montre une masse kystique dans 23 cas. La TDM cervicale a ete pratiquee dans 13 cas; faisant evoquer le diagnostic dans 7 cas. Une masse liquidienne parapharyngee a ete retrouvee dans un cas motivant la pratique d'un examen radiologique complementaire par une IRM cervicale. Une ponction cytologique a ete pratiquee dans 16 cas; ayant montre un materiel kystique dans 12 cas. Trente patients ont eu un traitement chirurgical avec exerese complete du kyste. Une mise a plat de la collection paraphryngee a ete pratiquee dans un cas. L'evolution a ete favorable dans 29 cas avec un recul moyen de 2ans et 9 mois. Deux cas de recidive ont ete notes. Conclusion : Les kystes amygdaloides sont des malformations relativement rares; leur diagnostic est suspecte a l'examen physique oriente par les donnees de l'imagerie et confirmee par l'examen anatomopathologique. Le traitement est chirurgical; seule l'exerese complete du kyste permet de prevenir les recidives ulterieures

Rhinorrhée cérébrospinale: difficultés thérapeutiques et facteurs d'échec

Introduction : La rhinorrhee cerebrospinale (rCS) resulte d'une breche osteomeningee faisant communiquer les cavites naso-sinusiennes avec les espaces sous-arachnoidiens. Sa gravite est liee au risque de complications infectieuses endocraniennes. Objectifs : discuter les modalites de prise en charge de la rCS et en analyser les facteurs d'echec. Patients et methode : Il s'agit d'une etude retrospective a propos de 15 patients presentant une rhinorrhee cerebrospinale en rapport avec une breche meningee traitee dans notre service. Resultats : L'age moyen de nos patients etait de 44;9 ans. Le motif de consultation etait une rhinorrhee claire intermittente. la notion de meningite etait rapportee dans 2cas. Un scanner du massif facial etait pratique dans tous les cas. Une cisterno IRM etait pratiquee dans 7 cas. La voie endoscopique etait adoptee dans14 cas et la voie combinee dans 1 cas. L'interposition de la greffe etait faite selon la procedure overlay dans tous les cas. Deux recidives ont ete notees et ont ete reprises chirurgicalement avec succes. Conclusion : La voie endonasale est une approche efficace et non invasive des breches osteomeningees. elle trouve son indication meme en cas d'echec de la voie transcranienne

[Pediatric mucoceles: clinical aspects and therapeutic approaches]. / Mucocèles de l'enfant : présentations cliniques et modalités thérapeutiques.

INTRODUCTION: Paranasal sinuses mucoceles are extremely rare in children and adolescents. The objective of this study was to assess their clinical presentations and the contribution of endoscopic surgery in their management. MATERIAL AND METHODS: we retrospectively reviewed 11 children with paranasal sinus mucoceles who were managed during a period of 15 years (1995-2009). RESULTS: The mean age was 11.8 years with a sex ratio of 2.66. One patient had a history of nasal polyposis surgery. The symptomatology was dominated by ophthalmological complains. The ethmoïd sinus was the main localization. Orbital extension was found in eight cases, pyomucocele in one case. The surgical management was endoscopic (nine cases), and by an external approach (two cases). Two cases of recurrence were assessed. The mean follow-up was 22.3 months. DISCUSSION: Paranasal sinuses mucocele is exceptional in children. Cystic fibrosis is the main etiologic factor. The symptoms depend on the location and size of the mucocele. The treatment is surgical and most authors prefer the endoscopic approach.

Ramsay Hunt syndrome.

INTRODUCTION: Ramsay Hunt syndrome is a viral infection that combines facial palsy and pinnal vesicular rash. OBJECTIVES: To detail diagnostic criteria, treatment and prognosis in Ramsay Hunt syndrome. PATIENTS AND METHODS: A retrospective study of 15 patients with Ramsay Hunt syndrome admitted to the ENT department of La Rabta Hospital (Tunis) from 2003 to 2009. RESULTS: The main presenting symptom was facial palsy associated with conchal vesicular rash. House-Brackmann classification of facial nerve function ranged from III to V. All patients were treated with acyclovir and corticosteroids. Mean follow-up was 8 months. Ten patients showed improvement in facial palsy, four had permanent palsy and one moved from grade IV to grade III. CONCLUSION: Ramsay Hunt syndrome involves severe dysfunction, with poorer facial nerve prognosis than in Bell's palsy.

Extranodal NK/T-cell lymphoma, nasal type: report of 15 cases.

OBJECTIVES: To define the epidemiological and clinical features and complementary investigation findings of extranodal NK/T-cell lymphoma, nasal type and to discuss the diagnostic difficulties and the various treatment options. PATIENTS AND METHODS: This retrospective study was based on 15 patients with extranodal NK/T-cell lymphoma, nasal type, managed between 1990 and 2009. RESULTS: This series comprised 13 men and two women (sex ratio=6.5) with a mean age of 52 years (range: 35-81 years). The mean time to first consultation was 6 months. The most common symptoms were nasal obstruction (87%) and purulent nasal discharge (73%), followed by epistaxis (60%). Physical examination demonstrated the presence of a tumour of the nasal cavity in 11 patients. The diagnosis was confirmed by histological examination of a biopsy completed by immunohistochemistry. CT scan of the facial bones was performed in all patients of this series. The site of extranodal NK/T-cell lymphoma was essentially nasal (12 cases). Orbital extension was observed in four cases, associated with intracranial extension in two cases and osteolysis was observed in 11 patients. Lymphomas were classified as stage IE in 74% of cases and stage IIE in 26% of cases. Only one patient was lost to follow-up during treatment. Three patients died before any treatment. Treatment therefore concerned 12 patients. Stage IE lymphomas were treated by radiotherapy and/or chemotherapy. All stage IIE lymphomas were treated by chemotherapy alone. Stage IE patients had a better prognosis. CONCLUSION: Extranodal NK/T-cell lymphoma, nasal type, is an aggressive form of non-Hodgkin's lymphoma comprising specific clinicopathological characteristics. The addition of chemotherapy for advanced stages does not appear to improve survival compared radiotherapy alone, which remains the treatment of choice especially for localized stages.

[Rosai-Dorfman disease: therapeutic issues in 2 cases]. / La maladie de Rosai - Dorfman : enjeux thérapeutiques à propos de 2 observations.

Rosai-Dorfman disease (RDD) is a benign lymphoproliferative disorder characterized by cervical lymph node enlargement with a consistent risk of airway compression and esthetic damage. Extranodal localizations are also described. There is no therapeutic consensus for pediatric forms of RDD. Through 2 pediatric cases with nodal involvement in 1 patient and a sinonasal and soft tissue localization in the other, we focus on the management problems of both nodal and extranodal RDD.

[Fifty-four cases of nasolabial cysts]. / Le kyste nasolabial: 54 cas.

INTRODUCTION: Nasolabial cysts (NLC) are a rare condition presenting as cystic epithelial lesion. We report the clinical and radiological (CT scan) aspects of this rare clinical entity and we assess the various available surgical treatment. PATIENTS AND METHODS: Fifty-four patients presenting with NLC underwent surgery between 2000 and 2009. The diagnosis was made on clinical and radiological arguments and confirmed by histological examination after surgical excision. The studied parameters were: gender, functional signs having led to consultation, localization, results of radiological exploration, treatment modalities, anatomopathology and postoperative evolution. RESULTS: The average age of the 30 men and 24 women was 38 years with extremes ranging between 24 and 53 years. The reason for consultation was swelling of the anterior nasal floor in every case and a nasal obstruction for 33 patients. The average time between initial swelling and consultation was 18 months. Swelling was unilateral for 52 patients. CT scan was prescribed for 20 patients and revealed a cystic mass with an average diameter of 23 mm. Cyst excision was made under general anesthesia in every case. Most of the patients (52) were operated via a vestibular approach. Histological examination confirmed the diagnosis of nasolabial cyst in every case. DISCUSSION: NLC is a rare condition which must be suggested when a cystic mass is found in the anterior nasal floor. CT scan confirms the diagnosis and cyst extension. Cyst excision is performed by vestibular approach. The endoscopic marsupialization is an interesting new therapeutic alternative.

Interest of fine-needle aspiration cytology in thyroid nodule.

OBJECTIVES: The aim of the present study was to evaluate our technique of thyroid fine-needle aspiration cytology (FNAC), its limitations and means of improvement by comparing our results with those of literature. MATERIAL AND METHODS: Thyroid FNAC results from 117 consecutive patients (May 2006 to July 2007) were categorized into four groups: benign, with suspected malignancy, malignant and unrepresentative. The FNAC results were compared with histopathologic analysis after thyroidectomy. RESULTS: All "benign" and "malignant" FNAC findings were confirmed on final histology. All "suspect" FNAC findings were benign on histology. Cytological diagnosis of malignancy was consistent with histological examination in all cases. Sensitivity was 100% (no false negatives), specificity 67% (28% false positives), positive predictive value 72% and negative predictive value 100%. There was a significant difference in the proportion of "unrepresentative" results between two of the operators performing aspiration (51% versus 29.2%). CONCLUSIONS: The main pitfall of FNAC in thyroid pathology is the "suspect" category, for which positive diagnosis is founded on histological criteria alone. The second limitation identified in this study was the high number of "unrepresentative" aspirates. However, the technique remains useful.

Effect of pantoprazole in patients with chronic laryngitis and pharyngitis related to gastroesophageal reflux disease: clinical, proximal, and distal pH monitoring results.

Few studies had evaluated the results of proton pump inhibitors on distal and proximal pH recording using a dual-channel probe. The aim of this study was to determine the clinical and pH-metric effect of treatment with pantoprazole 80 mg for 8 weeks in patients with ear, nose, and throat (ENT) manifestations of gastroesophageal reflux disease associated with pathological proximal acid exposure. We conducted a prospective open study. Patients included had to have chronic pharyngitis or laryngitis, and a pathological gastroesophagopharyngeal reflux. All patients received treatment with pantoprazole 80 mg daily for 8 weeks. One week after the end treatment, patients had a second ENT examination and a 24-hour pH monitoring using dual-channel probe. We included 33 patients (11 men, 22 women). A pathological distal acid reflux was found in 30 patients (91%). After treatment, the improvement of ENT symptoms was found in 51.5% of patients. Normalization of 24-hour proximal esophageal pH monitoring was observed in 22 patients (66%). After treatment, the overall distal acid exposure, the number of distal reflux events, and the number of reflux during more than 5 minutes were significantly decreased (respectively: 19.4% vs 7.2% [P < 0.0001], 62.7 vs 28.4 [P < 0.0001], and 10.4 vs 3.9 [P < 0.0001] ). Similarly, in proximal level, the same parameters were significantly decreased after treatment (respectively: 6.8% vs 1.6% [P < 0.0001], 32.6 vs 8.1 [P < 0.0001], and 3.4 vs 0.6 [P= 0.005] ). Treatment with pantoprazole reduced the frequency and severity of gastroesophagopharyngeal acid reflux in patients with chronic pharyngitis and laryngitis.

[Intraorbital cavernous hemangioma]. / Les hémangiomes caverneux intra-orbitaires.

Cavernous hemangioma is the most frequent primary vascular orbital tumor in adults. This slowly evolving lesion is usually located behind the ocular globe, between the extrinsic muscles. It leads to axial exophthalmia. Surgical treatment is indicated when there is a risk of visual impairment. The prognosis is related to the size of the lesion and its extension. CT scan is rather unspecific, but MRI is highly sensitive and specific in case of progressive painless exophthalmia.

[Nasopharyngeal angiofibroma: report of 15 cases treated by embolization]. / Le fibrome nasopharyngien: à propos de 15 cas traités par embolisation.

PURPOSE: To report a series of 15 new cases of nasopharyngeal angiofibroma treated with presurgical embolization along with a review of the literature. MATERIALS AND METHOD: Retrospective review of 15 cases of nasopharyngeal angiofibroma treated with presurgical embolization. The clinical, CT and MR features as well as postsurgical follow-up are reviewed. RESULTS: Presurgical embolization reduced surgical difficulties, especially hemorrhage, allowing complete tumor resection in most cases. Two cases of recurrent disease and 1 case of residual tumor were noted and treated by repeat embolization. CONCLUSION: Presurgical embolization reduces intraoperative hemorrhage and allows complete resection of these highly vascularized tumors.

Les traumatismes externes du larynx

Objectif : Les traumatismes externes du larynx sont rares. Ces traumatismes; ouverts ou fermes; peuvent etre isoles; ou s'integrer dans la cadre d'un traumatisme cervical complexe ou d'un polytraumatisme. Leurs aspects etiologiques; cliniques; diagnostiques et therapeutiques sont tres varies. Les principes generaux de leur prise en charge et de leur traitement chirurgical dependent de la gravite des lesions Materiel et methodes : Nous rapportons une etude retrospective portant sur 30 patients ayant ete suivis et traites pour un traumatisme externe du larynx dans le service d'ORL de la Rabta. La serie a ete colligee sur une periode de 25 ans allant de 1983 a 2008. Resultats : L'age moyen de nos patients est de 30 ans. Les etiologies sont dominees par les accidents de la circulation (53de nos patients). Tous nos malades ont eu un examen endolarynge par le nasofibroscope ou le miroir larynge. La laryngoscopie sous AG a ete realisee chez 25 patients. La tomodensitometrie cervicale a ete pratiquee chez 16 patients. Au decours de ces examens; tous nos patients ont ete classes selon la classification de Schaeffer modifiee par Fuhrman. Plus que la moitie des patients (54) sont classes en stade I et II. L'exploration chirurgicale a ete indiquee pour 16 patients. Le traitement a ete conservateur dans tous les cas. Le recul moyen est de 2 ans. Trois de nos patients ont developpe une stenose sous glottique. Deux d'entre eux ont eu une laryngoplastie d'agrandissement type Rethi. Conclusion : Tout traumatisme larynge decouvert; doit etre pris en charge correctement afin d'eviter des complications pouvant mettre en jeu aussi bien le pronostic vital que le pronostic fonctionnel

Implantation cochleaire et malformation de l'oreille interne

Les indications de l'implant cochleaire se sont elargies depuis 1995. La plupart des candidats a l'implant sont des enfants sourds profonds congenitaux. L'implant peut actuellement se discuter dans certains cas particuliers notamment chez des enfants presentant une malformation de l'oreille interne (vesicule unique; malformation de type Mondini; Mondini-like; dilatation de l'aqueduc du vestibule). Les risques sont chirurgicaux et infectieux : geyser a la cochleostomie; paralysie faciale; otoliquorrhee secondaire et meningite. Nous rapportons le cas d'une fille agee de 4 ans et 8 mois qui a presente suite a un traumatisme minime une surdite brutale totale bilaterale qui n'a pas recupere sous traitement medical. L'audiometrie avait confirme la surdite bilaterale et l'examen orthophonique a retrouve un langage oral bien conserve. Une imagerie a ete pratiquee mettant en evidence une dilatation bilaterale des aqueducs du vestibule et des sacs endolymphatiques avec un aspect normal de la cochlee et des canaux semi-circulaires.La patiente a beneficiee d'une implantation cochleaire gauche avec des suites simples et un excellent resultat a 2 ans

Les carcinomes adenoides kystiques de la parotide a propos de 3 cas

Les carcinomes adenoides kystiques (CAK) representent 2 a 4des tumeurs de la parotide et 12des tumeurs malignes. Ce sont des tumeurs a croissance lente qui sont caracterisees par leur capacite a infiltrer les gaines nerveuses. Les auteurs rapportent trois cas de CAK de la glande parotide diagnostiques et traites entre l'annee 2000 et 2007. Il s'agissait de 2 hommes et une femme qui ont consulte devant l'apparition d'une tumefaction de la loge parotidienne associee a une paralysie faciale dans un cas. Les aires ganglionnaires etaient libres. Tous les patients ont beneficie d'une exploration comportant une echographie; une TDM dans un cas et une IRM chez un patient. Le traitement a consiste en une parotidectomie totale associee a un curage ganglionnaire dans tous les cas et a une radiotherapie complementaire. L'evolution a ete favorable dans deux cas. Le carcinome adenoide kystique de la parotide est une tumeur rare; caracterisee par une evolution tres lente; une agressivite locale; un caractere fortement recidivant localement et un haut pouvoir metastatique a distance

[Mandibular aneurysmal bone cyst mimicking a malignant tumor]. / Kyste osseux anévrismal mandibulaire mimant une tumeur maligne.

CASE: We report the case of a 24-year-old woman presenting with a rapidly growing mass in the right masseteric region. MRI suggested a diagnosis of a rhabdomyosarcoma but CT-scan evoked a benign lesion such as chondroblastoma or aneurismal bone cyst. The biopsy specimen proved the diagnosis of aneurismal bone cyst and the patient was treated by surgical curettage. There was no recurrence after 4 years of follow-up. DISCUSSION: The aneurismal bone cyst of the jaw is a rare pseudocystic lesion, characterized by replacement of the normal bone with fibro-osseous tissue containing blood-filled sinusoidal or cavernous spaces. The lesion generally affects young persons under 20 years of age. The molar region, the angle, and the ascending ramus of the mandible are the predominantly affected sites. The lesion does not have any clinical or radiological specificity and surgical curettage is the standard treatment. Recurrence is rare and occurs within 30 months after surgery.

Le melanome malin des cavites nasosinusiennes : a propos d'un cas

Introduction : Le melanome malin primitif naso-sinusien est une affection tres rare et de mauvais pronostic. Elle compte pour environ 90des tumeurs cutaneo-muqueuses et seulement 4des cancers naso-sinusiens. Cas clinique : Les auteurs rapportent un cas de melanome malin des fosses nasales chez une patiente de 53 ans. Le tableau clinique etait domine par une obstruction nasale gauche associee a une epistaxis intermittente. Le diagnostic a ete confirme par l'examen histologique et le traitement a ete base uniquement sur la chimiotherapie. L'evolution etait defavorable avec deces de la patiente a un an de suivi. Discussion : Les melanomes malins des fosses nasales representent 8des melanomes cervico-faciaux. Cette tumeur se voit surtout chez l'adulte a partir de la 6eme decade sans predilection de sexe. Les symptomes les plus communement observes sont l'obstruction nasale et les epistaxis. L'aspect endoscopique est generalement celui d'un bourgeon charnu polypoide tres pigmente brun noir ou achromique rose unilateral. Le traitement reste essentiellement chirurgical consistant une exerese large par voie externe. La radiotherapie et la chimiotherapie sont parfois proposees pour des cas non chirurgicaux ou evolues

Plasmocytome ganglionnaire : a propos d'un cas

Les plasmocytomes ganglionnaires sont tres rares. Ils peuvent etre en rapport avec des metastases a partir d'un myelome multiple ou d'un plasmocytome extramedullaire. Les localisations primaires ganglionnaires restent exceptionnelles. Nous rapportons un cas collige et opere au service d'ORL et CMF de l'hopital la Rabta. Il s'agit d'une femme de 74 ans operee d'une tumefaction submandibulaire gauche. Le diagnostic est fait a l'examen histologique qui a revele un plasmocytomeganglionnaire primaire. Le traitement est radiochirurgical. Nous presentons les aspects histopatologiques; cliniques; biologiques et therapeuthiques de cette affection rare