[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.

A c.3216_3217delGA mutation in AGL gene in Tunisian patients with a glycogen storage disease type III: evidence of a founder effect.

Glycogen storage disease type III (GSD III) is an autosomal recessive disorder characterized by excessive accumulation of abnormal glycogen in the liver and muscles and caused by deficiency in the glycogen debranching enzyme, the amylo-1,6-glucosidase (AGL). In this study, we report the clinical, biochemical and genotyping features of five unrelated GSD III patients coming from the same region in Tunisia. The concentration of erythrocyte glycogen and AGL activity were measured by colorimetric and fluorimetric methods, respectively. Four CA/TG microsatellite markers flanking the AGL gene in chromosome 1 were amplified with fluoresceinated primers. The full coding exons and their relevant exon-intron boundaries of the AGL gene were directly sequenced for the patients and their parents. All patients showed a striking increase of erythrocytes glycogen content. No AGL activity was detected in peripheral leukocytes. Sequencing of the AGL gene identified a c.3216_3217delGA (p.Glu1072AspfsX36) mutation in the five patients which leads to a premature termination, abolishing the AGL activity. Haplotype analysis showed that the mutation was associated with a common homozygote haplotype. Our results suggested the existence of a founder effect responsible for GSD III in this region of Tunisia.

Addition of magnesium sulfate to bupivacaine improves analgesic efficacy after tonsillectomy: A randomized trial and a CONSORT analysis.

OBJECTIVES: The main objective of this study was to compare the wound infiltration (peritonsillar fossa) of magnesium sulphate combined with bupivacaine, bupivacaine alone and saline solution on post-tonsillectomy pain in children. The accessory objectives were to evaluate the effect of magnesium sulphate infiltration on prevention of laryngospasm and occurrence of nausea/vomiting. METHODS: This study is a prospective; double blinded and randomized clinical trial. Seventy-five children undergoing tonsillectomy were enrolled. Patients were randomized into three groups using closed envelop technique. Group 1 (N=24) received saline solution (NaCl), group 2 (N=25) received 0.25% bupivacaine (1mg/kg) and group 3 received magnesium sulphate (5mg/kg) and 0.25% bupivacaine (1mg/kg) after tonsillectomy using three-point technique. Pain was evaluated using mCHEOPS scale. The occurrence of laryngospasm, nausea and vomiting was monitored. RESULTS: The mCHEOPS scores of the group 3 were significantly lower than those of the group 2 and 1 (P<0.001). Time to first analgesic administration was longer for the group 3 than for the groups 2 and 1 (P<0.001). The mean consumption of additional analgesic drugs was lower for the group 3 than the other groups (P<0.001). There were no episodes of laryngospasm in the group 3 in comparison with the other groups. The difference of the incidence of nausea and vomiting was not statistically significant (P=0.628). CONCLUSION: The adjunction of magnesium sulphate to bupivacaine proved to provide more efficient pain control than bupivacaine alone. However, the small number of participants and the absence of sampling at the P level of 0.005 do not allow to conclude with absolute certainty.

[Pre- and post-chemotherapy tumor volumes: New prognostic factors in nasopharyngeal carcinoma]. / Volumes tumoraux avant et après chimiothérapie : de nouveaux facteurs pronostiques dans les carcinomes nasopharyngés.

PURPOSE: The pre- and post-induction chemotherapy tumor volumes of nasopharyngeal carcinomas may be prognostic indicators for adapting the therapeutic strategy. The objective of our study is to assess the prognostic impact of pre- and post-induction chemotherapy volumes in patients treated for locally advanced nasopharyngeal carcinomas. PATIENTS AND METHODS: This is a retrospective study including 52 patients with locally advanced nasopharyngeal carcinoma treated with 3 courses of induction chemotherapy (TPF) followed by intensity modulated radiotherapy associated with concomitant chemotherapy. RESULTS: The median initial and post induction chemotherapy total volumes were 92.3 and 41.5mL, respectively. At 3 years, the LRFS, DMFS, DFS and OS were respectively 85.9%, 63.5%, 56.8% and 67.8%. In multivariate study, the combination of a high initial volume (>100mL) and post-chemotherapy volume (>35mL) was an independent factor for LRFS, DMFS, DFS and OS. The total baseline volume had a better predictive prognostic value for DFS and OS than the TNM classification (8th edition 2017). CONCLUSION: The prognostic weight of tumor and nodal volumes was greater than the TNM classification (8th edition). The pre- and post-chemotherapy tumor volumes allow selecting a high-risk patients' subgroup "high initial and post chemotherapy volumes" in which it would be advisable to offer more intensive treatment regimens.

[Current management and perspectives for locally advanced nasopharyngeal carcinoma]. / État des lieux et perspectives thérapeutiques pour les carcinomes nasopharyngés localement évolués.

Nasopharyngeal carcinoma diagnosis is often made at a locally advanced stage (75 to 90% of cases) due to its deep localization. Concomitant radio-chemotherapy is the cornerstone of the treatment of locally advanced forms. The advent of intensity-modulated radiotherapy has improved oncological outcomes and reduced toxicity and is currently the gold standard for irradiation technique. For the locally advanced stage, the addition of induction chemotherapy has become the new standard care according to the latest international recommendations to reduce tumor volumes and act early on micro-metastases. Despite these therapeutic advances, the local and especially distant failure rate remains high. This article reviews current treatment strategies and discuss new approaches and perspectives of locoregional and systemic treatment to reduce treatment failures.

Therapeutic results of laryngeal preservation: a retrospective study.

AIM: The objective of this study is to report the therapeutic results of the preservation strategy in locally advanced laryngeal cancers. PATIENTS AND METHODS: Between January 2008 and December 2015, 24 patients with locoregional advanced non-metastatic laryngeal cancer (T2-4/N0-2) were collected retrospectively. Different therapeutic sequences were used: either induction chemotherapy followed by concurrent chemoradiotherapy or induction chemotherapy followed by radiotherapy or concurrent chemoradiotherapy or radiotherapy alone. RESULTS: The objective response rate was 85.7%. Overall survival rates at 1 year, 3 years and 5 years were 91.3%, 80.2% and 53.5%, respectively. Administration of induction chemotherapy did not improve overall survival. The 1-year overall survival was 83.3% in the induction chemotherapy group vs 94.1% for those who did not received induction chemotherapy (p = 0.7). CONCLUSION: Our study showed the feasibility of this preservation strategy in clinical practice, with acceptable term toxicity.

[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.

[A case of thyroid metastasis of an unusual cancer]. / Un cas de métastase intrathyroïdienne d'un primitif inhabituel.

Nasopharyngeal carcinoma affects preferentially young adults and is characterized by a high rate of node and visceral metastases that explains a part of therapeutic failure. We report a case of thyroid metastasis of a nasopharyngeal carcinoma. A 50-year-old patient had been treated of a nasopharyngeal carcinoma (T4 N2M0). Four years after a concomitant chemoradiotherapy, he presented with a compressive mass in the thyroid loge associated to cervical nodes. He underwent a total thyroidectomy and the definitive result concluded to an carcinome indifférencié de type nasopharyngé (UCNT) thyroid metastasis. A new concomitant chemoradiotherapy was delivered and consequently the tumor disappeared. Secondly, several node recurrences occurred. Metastasis of UCNT in thyroid gland is exceptional. Several problems are related to its aetiopathogenesis and to its treatment that is not systematized in the literature. The prognosis like for all metastatic forms of nasopharyngeal cancer is unfavorable.

[Cervical spondylodiskitis revealed by retropharyngeal abscess]. / Spondylodiscite cervicale révélée par un abcès rétropharyngé.

INTRODUCTION: Spondylodiskitis is rare in the cervical vertebrae. We report a case of cervical spondylodiskitis revealed by a retropharyngeal abscess caused by a pyogen agent. The aim of our work was to shed light on the diagnosis and therapeutic difficulties of spondylodiskitis associated with a retropharyngeal abscess. CASE REPORT: A 70-year-old patient was admitted to the emergency department for a progressive laryngeal dyspnoea of 3 months duration. Clinical examination showed severe dyspnoea. Oropharynx and pulmonary exams were unremarkable. Failure of medical treatment led to the performance of an emergency tracheotomy after which the patient developed a flaccid quadriparesis. Medullar MRI revealed a spondylodiskitis with medullary compression and a retropharyngeal abscess. Surgical biopsy concluded to a pyogen osteitis. Treatment in the form of antibiotics and cervical immobilisation was instituted. Evolution was marked with septicaemia, multiple organ failure and ionic disturbance leading to death. COMMENTS: Cervical spondylodiskitis is rare. Its revelation by retropharyngeal abscess is also rare. MRI is the tool of choice for diagnosing discovertebral infections and its prevertebral extensions. Treatment is medical and surgical.

[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.

[Frontal pneumosinus dilatans associated with multiple cerebral meningioma]. / Pneumosinus dilatans frontal associé à des méningiomes cérébraux multiples.

UNLABELLED: Pneumosinus dilatans is rare. It is defined as an expansion of one of paranasal sinuses. We discuss the etiopathogenic hypothesis of pneumosinus dilatans, its epidemiologic and clinical characteristics, and its therapeutic approaches. OBSERVATION: A man, 50 years old, presented frontal pneumosinus dilatans associated with multiple cerebral meningiomas, one of which was appended in the nasal cavity revealed by frontal deformation and nasal obstruction. CT and MRI confirmed the diagnosis. The patient was operated in the neurosurgery department, via the bicoronal approach, allowing the removal of meningioma and osteodural repair of the skull base. DISCUSSION: Pneumosinus dilatans is a rare pathology with an unknown etiology. It mainly affects the frontal sinus. Its association with cerebral meningioma has been described in the medical literature. Treatment is surgical and is recommended for cases with aesthetic problems.

[Head and neck sarcoma]. / Les sarcomes de la tête et du cou.

INTRODUCTION: Head and neck sarcomas comprise a heterogeneous and biologically diverse set of rare neoplasms. The difficulty treating the disease requires multidisciplinary consultation to improve outcome. In an effort to clarify the clinical behavior of head and neck sarcomas and evaluate treatment, we present our experience and review the relevant literature. PATIENTS AND METHODS: This is a retrospective analysis of 15 patients with histologically proven head and neck sarcoma treated in the ENT department between 1998 and 2007. All cases were confirmed by histologic exam with immunohistochemical analysis. RESULTS: Nine women and six men were included in the study. The mean age was 38.4 years (range: 6-73 years). Computed tomography and MRI were done in 66.7% and 60% of the patients, respectively, to evaluate tumor extension. Metastases were demonstrated in three cases. Curative treatment in eight cases was based on different combinations of chemotherapy, radiotherapy, and surgery. Complete remission was obtained in eight cases. Local recurrence was noted in two cases. Two patients died from tumor metastasis. Two patients were lost to follow-up. CONCLUSION: Head and neck sarcomas present diverse rare neoplasms. They make up 5%-20% of all soft tissue sarcomas. All age groups can be affected by this neoplasm, with no predominance in one sex or the other. The most common histologic type is rhabdomyosarcoma, especially in children. Because of the potential for systemic metastasis, extension must be assessed. Treatment is based essentially on the association of surgery, radiotherapy, and chemotherapy to optimize therapeutic results. Five-year survival varies between 44% and 80%.

[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.

[Surgery of pleomorphic adenoma of the parotid gland]. / Chirurgie de l'adénome pléomorphe de la parotide.

INTRODUCTION: Pleomorphic adenoma is the most common benign tumor of the parotid gland. It has a tendency of recurrence and malignant transformation. The surgical excision of this lesion continues to be the subject of a major debate. In this study, we discuss optimal surgical options for pleomorphic adenoma. MATERIALS AND METHODS: We reviewed clinical records of 82 patients who underwent surgery. RESULTS: The tumor was localized in the superficial lobe in 81.7% of cases. Tumors of the deep lobe were removed by total parotidectomy. Those of the superficial lobe underwent partial exofacial parotidectomy (7 cases), exofacial parotidectomy (44 cases), or total parotidectomy (16 cases). Transitory facial paralysis was higher after total parotidectomy. Only one patient developed a recurrence, his tumor showed capsule infiltration. DISCUSSION: Conventional or partial superficial parotidectomy may be a good compromise with fewer complications and low incidence of recurrence.

[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.

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.

Evaluation of Chitine synthase (CHS1) polymerase chain reaction assay in diagnosis of dermatophyte onychomycosis.

BACKGROUND: Onychomycosis is one of the most prevalent dermatophytic diseases. Mycological methods used in the conventional diagnosis may not be optimal. PCR was reported as a reliable alternative in the diagnosis of dermatophytosis. MATERIALS AND METHODS: A PCR method based on the amplification of the chitin synthase 1 gene was developed. The study included 119 strains of dermatophytes and non dermatophytic fungi, eight dermatophytic reference strains and 201 nail specimens from patients with dermatophytic onyxis. DNA extraction was carried out by using the QIAamp DNA extraction kit (Quiagen). RESULTS: PCR positivity was based on the production of a specific 432 bp fragment. None of the investigated non dermatophytic strains was positive. Sensitivity of PCR was higher as compared to mycological examination (90.5% vs. 81.1%). PCR was positive in 31 onyxis cases with positive direct examination but negative or contaminated culture. In contrast, PCR was negative in 10 cases where both direct examination and culture were found positive. CONCLUSION: PCR is an adequate tool for the diagnosis of dermatophytic onychomycosis. It is much adapted to cases where culture is negative or contaminated by overgrowing molds, which makes the identification of the causal agent problematic.

[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.