[Ductal carcinomas of the parotid gland]. / Les carcinomes canalaires de la parotide.

PURPOSE: To describe the clinical, therapeutic and prognostic features of ductal carcinomas of the parotid gland. MATERIAL AND METHODS: Five patients with ductal carcinoma of the parotid gland (primary and secondary carcinoma) treated, between 2007 and 2019, in our ENT department, were reviewed. RESULTS: Four men and one woman were included. The mean age was 61,4 years. One patient had a history of an invasive ductal carcinoma of the breast. Four patients consulted for swelling in the parotid region. One patient referred to our department for dysfunction of facial nerve. Skin invasion was found in one case. Four patients underwent total parotidectomy with sacrifice of the facial nerve (three cases). One patient underwent extended parotidectomy involving the skin. An ipsilateral selective neck dissection was performed in four cases. One patient had a parotid gland biopsy. Ductal carcinoma was primary in four cases and metastatic from breast origin in one case. Four patients were treated with postoperative radiotherapy. Remission was obtained in three cases. One patient had a local and meningeal recurrence. The patient with metastatic carcinoma had pulmonary, bone, hepatic and brain progression. CONCLUSION: Ductal carcinoma is a rare and aggressive tumor of the parotid gland. It can be primary or secondary. The treatment is based on surgery and radiotherapy. The prognosis is poor.

[Ectopic thymus: A rare cause of neck mass in children]. / Thymus ectopique : une cause rare de tuméfaction cervicale chez le nourrisson.

INTRODUCTION: Ectopic cervical thymus (ECT) is a rare embryological abnormality in children. It can be revealed by a compressive neck mass mistaken for a malignant tumor. Through a new case of ECT, we review the embryopathogenesis, diagnostic difficulties and therapeutic features. CLINICAL OBSERVATION: A 19-month-old girl presented a right cervical mass that quickly increased in size, causing intermittent dyspnea. The physical examination objectified a 6-cm, soft and compressible, painless right cervical tumefaction, extending from the mastoid area to the ipsilateral supraclavicular fossa. The diagnosis suggested based on CT was a cervicomediastinal cystic lymphangioma. The diagnoses discussed based on MRI were a collection of necrotic lymphadenopathy, rhabdomyosarcoma or neurofibroma debris. The mass was surgically excised through a laterocervical incision. A whitish multilobular tissular mass was found, adherent to the neurovascular axis of the neck. Pathological examination concluded in normal ectopic thymus tissue. The postoperative course was uneventful. CONCLUSION: Although ECT is a rare benign anomaly, it should be considered as a possible cause of a neck mass in children. Surgery is the curative treatment. Before surgery, the presence of a mediastinal thymus must be confirmed to avoid the risk of a total thymectomy in children. MRI is helpful in delineating thymic ectopia compared to the mediastinal thymus.

[Idiopathic facial paralysis in children]. / Paralysie faciale idiopathique de l'enfant.

BACKGROUND: Idiopathic facial palsy is the most common cause of facial nerve palsy in children. Controversy exists regarding treatment options. The objectives of this study were to review the epidemiological and clinical characteristics as well as the outcome of idiopathic facial palsy in children to suggest appropriate treatment. PATIENTS AND METHODS: A retrospective study was conducted on children with a diagnosis of idiopathic facial palsy from 2007 to 2012. RESULTS: A total of 37 cases (13 males, 24 females) with a mean age of 13.9 years were included in this analysis. The mean duration between onset of Bell's palsy and consultation was 3 days. Of these patients, 78.3% had moderately severe (grade IV) or severe paralysis (grade V on the House and Brackmann grading). Twenty-seven patients were treated in an outpatient context, three patients were hospitalized, and seven patients were treated as outpatients and subsequently hospitalized. All patients received corticosteroids. Eight of them also received antiviral treatment. The complete recovery rate was 94.6% (35/37). The duration of complete recovery was 7.4 weeks. DISCUSSION: Children with idiopathic facial palsy have a very good prognosis. The complete recovery rate exceeds 90%. However, controversy exists regarding treatment options. High-quality studies have been conducted on adult populations. Medical treatment based on corticosteroids alone or combined with antiviral treatment is certainly effective in improving facial function outcomes in adults. In children, the recommendation for prescription of steroids and antiviral drugs based on adult treatment appears to be justified. CONCLUSION: Randomized controlled trials in the pediatric population are recommended to define a strategy for management of idiopathic facial paralysis.

[Predictive factors for recurrence after surgery of nasal polyposis].

Introduction: Endoscopic sinus surgery has become the treatment of choice in the surgical management of patients with nasal polyposis. The aim of our study is to identify the role of some epidemiological, clinical and therapeutic factors in recurrence after surgery of nasal polyposis. Materials and methods: We conducted a retrospective study over a period of 11 years (between 2000 and 2010) including 184 patients operated for nasal polyposis after failure of prolonged medical treatment. We evaluated the impact of epidemiological and clinical factors (age, sex, asthma, Widal disease, allergy and stage of nasal polyposis at the time of surgery) and treatment (surgical technique, observance of postoperative topical steroids ) on postoperative recurrence. Results: Nasal poly­posis recurred in 26.6% of patients after an average period of 23 months. Widal disease, asthma and bad observance of the intranasal steroid therapy were significantly associated with postoperative recurrence in the univariate analysis. In multi­variate analysis the bad observance of the intranasal steroid therapy was the only factor significantly associated with recurren­ce. Conclusion: Postoperative steroids prescribed routi­nely in our practice can effectively prevent recurrence after endonasal surgery and this result was found in both uni­variate and multivariate analysis.

[Bronchoscopy for foreign body inhalation in the pediatric population: lessons learned from 223 cases]. / Bronchoscopie pour inhalation de corps étrangers chez l'enfant : à propos de 223cas.

INTRODUCTION: Inhalation of foreign body in children is a serious accident that may compromise the vital prognosis of the child. The diagnostic was difficult in the absence of a recognizable penetration syndrome. Bronchoscopy is still recommended as the appropriate diagnostic and treatment of foreign bodies. The purpose of this study was to analyze the diagnostic and the treatment result of bronchoscopy and discuss its indications. MATERIAL AND METHODS: [corrected] A retrospective study analyzing data related to 223 children undergoing bronchoscopy due to suspicion of foreign body aspiration over a period of 10 years (2000-2009). The average age of the children was 29 months (range: one month-13 years). Approximately, two thirds of these patients were boys. The penetration syndrome was reported in 79.8% of cases. RESULTS: During bronchoscopy, the foreign body was confirmed only in 57.4%. Foreign bodies were found in the bronchus in 79.7% of cases. Among the foreign bodies, 78.1% were of vegetal origin. The average time of stay of the foreign body was of 16.1 days. Penetration syndrome and abnormal physical exam were the most sensitive parameters (79.7% and 82.8%, respectively) but with low specificity (24.2% and 35.8%, respectively). The combination of clinical and radiological signs suggestive of foreign body was the most specific sign (74.7%). Similarly, we found a statistically significant correlation between positive bronchoscopy and simultaneous suggestive clinical and radiological signs (P=0.03). The multivariate study showed that predictors factors of positivity of the bronchoscopy were: abnormal physical exam (P=0.016), abnormal radiological exam (P=0.003) and type of indication (P=0.005). DISCUSSION: The diagnosis of laryngotracheobronchial foreign body recures an array of arguments. It is suspected on the clinical interview specially penetration syndrome and on the clinical and radiological presentation. Any suspicion should lead to a bronchoscopy.

[Maxillary sinusitis of odontogenic origin: surgical treatment]. / Sinusites maxillaires d'origine dentaire : traitement chirurgical.

PURPOSE: The frequency of maxillary sinusitis of dental origin (MSDO) is under estimated. The medical and surgical treatment has improved thanks to endoscopic guidance. We report our experience and strategy in the treatment of MSDO. PATIENTS AND METHODS: Twenty-two patients presenting with MSDO were treated between 1998 and 2008. The results were evaluated on clinical, functional, sinusal, and odontologic signs. RESULTS: MSDO accounted for 16% of surgically managed sinusitis. CT was performed in 95% of cases. The etiologies were apical leakage in seven patients, migration of a tooth or root during extraction, or presence of ectopic tooth in the sinus in nine patients, a cyst in three patients, and oroantral communication in three patients. Surgery was performed after antibiotic and NSAID treatment. The first surgical step was the treatment of the odontogenic source. The second step was sinus drainage by endoscopic treatment in 64%, Caldwell-Luc in 23%, and drainage by oroantral communication enlarged then closed in the same operative time in 13%. The follow up ranged from 3 months to 10 years. Early postoperative superinfection was observed in two patients. Two patients presented with recurrent sinusitis. The postoperative sequels were hyposmia in three patients, dental pulpotomy, and trigeminal neuralgia in five patients treated by Caldwell-Luc surgery. DISCUSSION: Nasal endoscopy has improved the surgical management of MSDO. It makes curettage and exclusion of sinus cavities obsolete. It is reliable and has a low rate of complications. The best treatment remains prevention.

Primary sinonasal meningioma.

INTRODUCTION: Primary meningiomas of the sinonasal tract are rare tumors. Their positive diagnosis is difficult to establish. From one case observation, we report the clinical features, the diagnosis difficulties and the therapeutic modalities of primary meningioma of the sinonasal tract. CASE REPORT: A seventeen-year-old girl consulted for a left unilateral nasal obstruction with progressive evolution without episodes of epistaxis, smell disorder or headaches over a year. Physical examination revealed a grayish polypoid tumor in the left nasal fossa. CT scan evidenced an isodense lesion of the left nasal fossa slightly enhanced pushing back the lateral nasal wall without invasion or intracranial connection. Biopsy was in favour of an inverted papilloma. The tumor was resected via endoscopic approach. Pathological examination established the diagnosis of meningothelial menigioma. The prognosis was favourable without recurrence after a six-month follow-up. CONCLUSION: The positive diagnosis of primary sinonasal meningioma is difficult to establish because of their infrequent occurrence in this ectopic site and of their non-specific clinical appearance. The final diagnosis rests on the histological examination. Immunohistochemical studies are helpful to establish the accurate diagnosis. Imaging confirms the primitive nature of these tumors. Prognosis is excellent after complete surgical extirpation without the necessity of adjuvant therapy.

[Para-pharyngeal tumors, contribution of imaging]. / Tumeurs parapharyngées, apport de l'imagerie.

OBJECTIVES: Para-pharyngeal tumors are located deeply. Imaging is mandatory for their management. We conducted a retrospective study to determine the contribution of imaging for their diagnosis and treatment. PATIENTS AND METHODS: Imaging was performed for 20 cases of primary para-pharyngeal tumors between 1986 and 2008. We compared the imaging to the anatomic and histological features of these tumors. RESULTS: Computed tomography and MRI confirmed the para-pharyngeal location of tumors. Tumors were located in the prestyloid compartment in eight cases, in the retrostyloid compartment in five cases, and in the retropharyngeal compartment in one case. Six tumors had filled all the para-pharyngeal space. Salivary gland tumors had filled the prestyloid space in two cases, and in two other malignant cases all para-pharyngeal space were invaded. MRI failed to differentiate the nature of tumor and its malignancy except when there was obvious bone erosion. The treatment was mainly surgical. The mean follow-up was 5 years 6 months. DISCUSSION: Imaging contributes to the etiological diagnosis and assesses tumor extension, thus helping to choose the surgical approach. MRI is the most contributive examination; its resolution is more adapted to the diagnosis of deep tumors. CT scan is contributive when studying the bone structure.

Internal jugular vein thrombosis by protein C activated resistance.

INTRODUCTION: Jugular vein thrombosis is mainly due to infectious, neoplastic and iatrogenic causes. Activated protein C (APC) resistance is an exceptional cause of jugular vein thrombosis. CASE REPORT: A 40-year-old woman consulted for left lateral neck swelling present for two weeks. Neck ultrasound revealed left internal jugular vein thrombosis, which was confirmed by contrast CT. The rest of the examination, including routine clotting assessment and inflammatory work-up, was normal. Further investigations demonstrated APC resistance with Factor V Leiden mutation. Treatment consisted of oral anticoagulants with a good outcome. DISCUSSION: APC resistance is a recently identified and relatively frequent cause of thrombophilia, mostly due to Factor V Leiden mutation. APC resistance is responsible for 20% to 50% of all thrombotic events. The laboratory diagnosis is based on two tests: a phenotypic test based on APTT with and without APC and a genotypic test based on detection of a Factor V Leiden mutation. CONCLUSION: Screening for APC resistance and Factor V Leiden mutation is now part of the aetiological work-up of thromboses, particularly in subjects younger than 50. Treatment is based on oral anticoagulants.

[Post-radiation mucocele in two patients treated for nasopharyngeal cancer]. / Mucocèle après radiothérapie chez deux patients traités pour cancer du nasopharynx.

A 30-year-old woman, with a history of nasopharyngeal carcinoma, which was treated by radiotherapy nine years previously, presented with occasional diplopia and recent headaches. A nasopharyngeal biopsy showed no recurrence. The imaging revealed a sphenoidal sinus mucocele. Endoscopic marsupialization of the mucocele allowed clinical improvement. A 56-year-old woman presented, five years after radiotherapy for nasopharyngeal carcinoma, with a fronto-orbital mass. CT-scan revealed a fronto-ethmoidal mucocele. Nasopharyngeal biopsy showed tumour recurrence. Marsupialization of mucocele was performed. Recurrence of the carcinoma was treated by radiotherapy and chemotherapy. Sphenoidal sinus mucocele developing after radiotherapy for nasopharyngeal carcinoma has rarely been reported. CT scan and MRI are useful tools in making the diagnosis. Biopsy is required to diagnose recurrence or associated radio-induced tumor. Endoscopic approach gives good results.

Unusual form of cutaneous leishmaniasis: erysipeloid form.

UNLABELLED: We report the epidemiological and clinical characteristics of the erysipeloid form of cutaneous leishmaniasis as well as its diagnostic and therapeutic challenges. CASE REPORT: A 63-year-old woman, with no medical history, presented with a one-month history of erythematous nasal swelling. The lesion appeared after an accidental trauma. Erythematous infiltrative plaque was noted on the center of the face. There were also crust formations on the traumatic region. Despite local treatment and oral antibiotherapy, there was no improvement. The diagnosis of cutaneous leishmaniasis was confirmed by positive skin smears. Histopathological examinations of a skin biopsy showed no malignancy. The patient was treated intramuscularly with 10mg/kg per day systemic meglumine antimoniate with partial regression of symptoms. CONCLUSION: The erysipeloid type is a rare and unusual presentation of cutaneous leishmaniasis that often causes late diagnosis. Diagnosis is confirmed by the demonstration of the parasite by skin smear, histopathological examination and polymerase chain reaction. There are various therapeutic options. The evolution is generally favourable.

Occult lymph node metastasis in laryngeal squamous cell carcinoma: therapeutic and prognostic impact.

OBJECTIVES: To assess the incidence and impact on treatment and prognosis of occult lymph node metastasis in laryngeal cancer. PATIENTS AND METHODS: A retrospective study was performed on 164 patients treated for laryngeal cancer, initially classified as N0, with cervical lymph node dissection. RESULTS: Occult metastases were found in 41 neck specimens (12.5%) from 32 patients (19.5%). Involvement per neck level was: 7% level IIa, 2.4% IIb, 4.2% III and 2.7% IV. Lymph node involvement was significantly increased in case of T3T4 tumor or invasion of the pre-epiglottic space or cartilage. Survival was significantly influenced by pN status (pN- = 12 years, vs pN+ = 9 years; P = 0.006). CONCLUSION: Level IIb or IV involvement is rare. Superselective neck dissection (IIa, III) seems to be indicated in T1T2 N0 tumor. In case of advanced tumor or pre-epiglottic space or cartilage invasion, functional neck dissection is mandatory.

Cholesteatoma of the frontal sinus.

PURPOSE OF STUDY: Cholesteatoma of the paranasal sinuses is a rare pathology. A review of the literature reported less than 30 cases. These lesions mainly involve the frontal sinus. CASE REPORT: A 25-year-old man presented with a right painful fronto-orbital mass associated with an ipsilateral eyelid oedema and a fever as high as 40°C. He experienced a general epileptic seizure requiring his admission in an intensive care unit. CT-Scan with iodine injection evidenced the opacification of the right frontal sinus with bone lysis of the posterior wall. Complete surgical resection of a cystic structure containing keratin material was performed via eyebrow incision. The pathological examination confirmed the diagnosis of sinus cholesteatoma. Neurological signs entirely disappeared after surgery. Craniofacial MRI realized 2 months later showed no sign of recurrence. Obliteration of the right frontal sinus was performed 4 months later. CONCLUSION: Although benign, cholesteatoma can spread to the surrounding structures leading to several complications including infections that can be life-threatening for the patient. CT-scan and MRI are useful examinations for diagnosis and follow-up. Complete surgical resection is required in order to avoid recurrence.

B-cell lymphoma of the mandible.

INTRODUCTION: Primary malignant lymphoma of the mandible is rare. It is frequently mistaken for a dental lesion, delaying diagnosis. We here report a case of mandibular lymphoma and present the clinical and radiological characteristics and means of treatment of this pathology. CASE REPORT: A 17-year-old woman consulted for right facial tumefaction with 1 year's evolution. Clinical examination found a mass facing the horizontal branch of the mandible, extending to the right parotid region without cutaneous involvement. CT and MRI showed a large expansive process of mandibular origin with parotid, temporal and intracranial extension. Biopsy indicated large B-cell lymphoma. The patient showed good evolution 2 years after chemotherapy. CONCLUSION: Lymphoma is the second most frequent form of head and neck tumor, after epidermoid carcinoma. Only 0.6% of locations are mandibular, almost always consisting of B-cell lymphoma. They are often initially misdiagnosed as a dental pathology. Complete remission after chemotherapy ranges from 60 to 80% at 1 year.

Association of squamous cell carcinoma of the larynx and chronic lymphoid leukemia.

INTRODUCTION: The association of squamous cell carcinoma of the larynx and chronic lymphocytic leukemia (CLL) is exceptional. We report an observation of this association and present the therapeutic problems as well as the effects on prognosis. OBSERVATION: Direct laryngoscopy showed a tumor of the right hemilarynx, with the biopsy concluding in moderately differentiated keratinizing squamous cell carcinoma. The patient had a total laryngectomy, with bilateral lymph node evidement. The anatomopathological examination of the operative specimen demonstrated infiltration of the larynx and squamous cell carcinoma adenopathies and CLL. It was decided to monitor the chronic lymphoid leukemia, classified as Binet stage B. The synchronous or metachronous onset of a second cancer in a patient with CLL is more frequent than in the general population. The synchronous association of squamous cell carcinoma of the larynx and CLL has been described only rarely. The therapeutic strategy should focus first on the cancer with the shortest survival rate. The prognosis is more negative in an association of cervicofacial squamous cell carcinoma and leukemia than in a single cervicofacial cancer.

Pendred syndrome in Tunisia.

OBJECTIVES: We report a clinical and genetic study of three consanguineous Tunisian families affected by Pendred syndrome. PATIENTS AND METHODS: Three families from the south of Tunisia were identified as affected by Pendred syndrome. The patients and their families underwent ENT and general examination and audiovestibular and radiological tests. Molecular DNA analysis was performed by the Sfax Human Molecular Genetics Department. RESULTS: Forty-three patients (mean age: 21 years [2-60 years]) were affected. Tonal audiometry showed bilateral sensorineural hearing loss in 87.5% of cases, and mixed hearing loss in 12.5% with bilateral high frequency sensorineural hearing loss and conductive hearing loss at lower frequencies. Deafness was severe in 21% and profound in 79% of cases. Thyroid goiter was found in 46.5% of cases. Inner ear CT scan found enlarged bilateral vestibular aqueducts in all cases. Hormone analysis was normal and perchlorate test negative in all cases. A single Pendred syndrome (PDS) gene mutation, L445W, was found. DISCUSSION: Pendred syndrome is the most frequent congenital deafness syndrome. It is characterized by great intrafamilial phenotype variability.

Hypopharyngeal amyloidosis: A case report.

Hypopharyngeal amyloidosis is rare. Management depends on etiology. We report a case of hypopharyngeal amyloidosis and review the characteristics of this exceptional pathology. A 60-year-old woman with a history of diabetes and chronic cervicalgia consulted for dysphagia and deteriorated general health status, which had been evolving for 2 months. Clinical examination found two ulcerations of the lateral edge of the tongue and right pyriform sinus salivary stasis. Panendoscopy found regular swelling of the posterior wall of the hypopharynx and cervical esophagus. The pyriform sinuses and larynx were normal. Cervical CT and MRI showed thickening of the posterior wall of the hypopharynx. Biopsy found amorphous acellular eosinophil interstitial deposits, shown to be amyloid on Congo red staining, leading to a diagnosis of amyloidosis. Etiological assessment pointed to myeloma. The patient was managed by chemotherapy associating melphalan and prednisone. Evolution at 12 months' follow-up was good. Localized amyloidosis is a rare lesion of the superior aerodigestive tract, predominating in the larynx. Hypopharyngeal involvement is exceptional. Diagnosis is histological. Management depends on etiology. Local treatment is exceptional other than in case of complication. Systemic forms with associated myeloma are of poor prognosis.

[Cutaneous basosquamous carcinoma]. / Carcinome basocellulaire métatypique.

INTRODUCTION: Basosquamous carcinoma is a rare entity that essentially affects the head and neck region in male patients. The authors present the clinical signs and progression as well as the therapeutic consequences of this disease through two observations. CASE REPORT 1: A 41-year-old man presented with basosquamous carcinoma of the right temporoparietal region treated initially with surgery alone. Five years later, he was operated on for a local and lymph node recurrence followed by radiation therapy, stabilizing the disease for 4 years; subsequently a second recurrence with metastasis to the chest area occurred. The patient died 10 years after the onset of his disease of diffuse pneumopathy with severe septicemia. CASE REPORT 2: A 71-year-old man presented retroauricular basosquamous carcinoma at first treated with wide resection, but the surgical limits were invaded. He developed local recurrences treated with surgical resection until total petrosectomy, but the surgical limits were always invaded. Radiotherapy was delivered. Seven years after the end of treatment, he developed a local recurrence invading the brain, which was deemed untreatable. DISCUSSION: Basosquamous carcinoma is characterized by its severe aggression and its tendency to recur. Treatment is essentially surgical. Radiotherapy is an adjuvant for the cases with high risk of recurrence. The role of chemotherapy is not yet proved.

[Neurofibromatosis type 1 revealed by malignant peripheral nerve sheath tumor]. / Tumeur maligne des gaines nerveuses périphériques révélant une neurofibromatose type 1.

UNLABELLED: Neurofibromatosis type 1 or Von Recklinghausen's disease is an affection with variable clinical expression. Malignant transformation is rare and dangerous. OBJECTIVE: The aim of this work is to study diagnostic criterias as well as treatment of this malignant transformation. CASE REPORT: Sixteen years old patient presented with gradually progressive basicervical mass without compression nor dysthyroidy signs. Physical examination revealed a painless, hard and well limited mass measuring 4 cm. It was located in front of sternal manuhrium and associated to multiples "café au lait" spots, iris Lisch nodules and generalized neurofibromas. This mass was excised by cervical surgery. The histological exam concluded to a low grade malignant peripheral nerve sheath tumour Unfortunately despite a larger reoperation including resection of the manubrium, the limit of this excision were not safe. Early recurrence was observed, although treated by surgery, chemotherapy and radiotherapy, the patient died by mediastinal invasion.