Transalar transsphenoidal meningoencephalocele presenting in the form of recurrent meningitis: Report of two cases and discussion of the diagnosis and treatment.

INTRODUCTION: Skull base meningoencephaloceles are rare congenital malformations. The two cases of transalar transsphenoidal malformation reported here differ from the classical transsphenoidal meningoencephalocele. CASE REPORTS: Case 1 was a three-and-a-half-year-old boy and case 2 was a 36-year-old man. Both cases presented with clinical features of recurrent meningitis. Surgical management of case 1 was performed via an intradural infratemporal fossa craniotomy with reoperation 2years later. In the second case, surgery was initially performed via an endonasal approach and then via pterional craniotomy. Reoperation via Sekhar's transpetrosal approach was also a failure. Only closure of the trigeminal-pontine angle via a suboccipital retrosigmoid approach allowed repair of the defect. DISCUSSION: Surgical access to the pterygopalatine fossa is complex due to its anatomical position and its anatomical relations with nerves and vessels. An endoscopic approach appears to be a valuable alternative to classical craniotomy. CONCLUSION: In the two cases reported here, neurosurgery allowed lasting closure of the defect.

Cervical lymph node metastases and T1 squamous cell carcinoma of the lips.

OBJECTIVE: The occurrence of cervical lymph node metastasis is a major prognostic factor for head and neck squamous cell carcinoma (SCC) of the lip. This study focuses on patients with T1 stage tumours,in order to describe the prevalence of metastatic lymphadenopathies. METHODS: A multicenter retrospective study was performed in three tertiary care referral centers and included 59 patients surgically treated for T1 stage SCC of the lips from January 1996 to December 2006. This surgical treatment concerned the tumour, with cervical neck dissection when lymph node metastasis was suspected. RESULTS: Cervical lymph node metastasis was found and histologically proven in 7 patients (11.9% of the cases, pN+ group; 95% confidence interval, 3.6 to 20.2%) during follow-up, never at the time of diagnosis. Metastasis appeared 13.3 ± 7.9 months (min. 5.3 months, max. 29.1 months) after the initial treatment and involved the IB level in all cases. Tumour progression was significantly longer in the pN+ group compared to patients with no lymph node metastases (sN-group). The pN+ group also had a significantly higher proportion of poorly or moderately differentiated tumours, and a significant decrease in overall survival, disease-free survival, and disease-specific survival. CONCLUSION: Lymph node metastasis occurs in roughly 12% of T1 stage SCC of the lips, and the management of neck lymph node areas is necessary. A randomized trial is needed to determine the proportion of occult lymph node metastases in T1N0 patients and the impact of this therapeutic procedure on survival.

Rapidly progressive bilateral postmeningitic deafness in children: Diagnosis and management.

OBJECTIVES: To determine the diagnostic approach to severe or profound bilateral postmeningitic deafness and to propose management guidelines. MATERIAL AND METHODS: A retrospective review of five patients (two adolescents and three infants) with rapidly progressive severe bilateral deafness following an episode of meningitis managed between 2004 and 2010. RESULTS: The two adolescents presented Neisseria meningitidis meningitis and the three infants presented Streptococcus pneumoniae meningitis. Acquired bilateral deafness was diagnosed by audiometry an average of 68.8 days (range: 9-210) after the episode of meningitis. Behavioural audiological testing, adapted to age and state of health, was performed in all patients. Deafness was confirmed by Auditory Brainstem Response tests. All five patients were assessed by computed tomography (CT) and magnetic resonance imaging (MRI) within ten days. T2-weighted MRI sequences showed endolymph changes in four patients. CT scan demonstrated ossification in only one patient. Bilateral cochlear implant was performed in all patients, with complete electrode array insertion for eight implants and partial insertion for two implants (20 and 21 out of 22 electrodes inserted). Good results were obtained with cochlear implants in four cases. CONCLUSIONS: Bilateral deafness can occur immediately or several months after bacterial meningitis, regardless of the micro-organism responsible, justifying screening by behavioural audiological testing adapted to age for two years following bacterial meningitis. Auditory Brainstem Response testing can confirm audiometric findings. When severe or profound bilateral deafness is observed, MRI must be performed urgently to detect endolymph inflammation or ossification. Early bilateral cochlear implantation is recommended in the presence of ossification.

Treatment of earlobe keloids by surgical excision and cryosurgery.

BACKGROUND: The long-term results of keloid treatments remain poor. OBJECTIVE: To assess the long-term efficacy of a technique combining surgical excision and immediate cryosurgery in patients with ear keloids. METHODS: A retrospective study was performed between January 2001 and June 2011. All patients referred for treatment of an ear keloid and undergoing subsequent surgical excision followed by immediate freezing of the postoperative wound using cryosurgery were included. The primary end-point was the number of patients who achieved major flattening of the keloid (defined as a reduction of more than 80% of the keloid) at the time of the study after a minimum follow-up of 24 months after treatment. RESULTS: Sixty-six patients with a total of 97 keloids were included. Median follow-up time after treatment was 43 months. 'Major' flattening was observed in 69 keloids (71%). Fifty-six of these lesions (81%) were treated with one session and 13 (19%) required a second session after keloid recurrence. Fifteen lesions (15%) were considered 'treatment failure', including 11 keloids in eight patients who were lost to follow-up and four keloids which achieved less than 30% reduction of their surface. Thirteen other keloids were evaluated as displaying significant or moderate improvement. Treatment side-effects were partial amputation of the ear lobe in three patients with a very large keloid of the ear lobe and hypopigmentation in five patients. LIMITATION: Retrospective study. CONCLUSION: The combination of surgical excision and cryosurgery can be considered for the treatment of large ear keloids.

BRAF mutation in papillary thyroid carcinoma: predictive value for long-term prognosis and radioiodine sensitivity.

OBJECTIVES: BRAF pV600E mutation is the most common oncogenic event and the most specific mutation for papillary thyroid carcinoma (PTC). Many studies over the last decade have shown a direct relationship between BRAF mutation and aggressive tumour characteristics, resulting in poor prognosis. However, several recent studies have suggested that BRAF mutation is not associated with poor prognosis of PTC. The present study was designed to evaluate the association between BRAF mutation with clinicopathological factors and tumour recurrence. MATERIAL AND METHODS: In this retrospective study, BRAF mutation status was examined by direct sequencing on paraffin-embedded tumour specimens from 46 patients undergoing surgery for PTC in our institution from 1985 to 2000. The relationship between BRAF mutation and gender, advanced age, extrathyroid extension, multifocal tumour, cervical lymph node metastasis, tumour size and advanced pT stage of PTC and its predictive role for the risk of tumour recurrence were investigated with a median follow-up of 10.1 (±6.5)years. RESULTS: BRAF mutation was detected in 20 of the 46 patients (43.5%) included in the study. No statistically significant correlation was demonstrated between the presence of BRAF mutation and the various clinicopathological factors studied. No significant difference in tumour recurrence rate or radioiodine sensitivity was observed between the two subgroups: mutant BRAF and wild-type BRAF. CONCLUSION: Although BRAF mutation appears to play a role in local tumour progression, it is not a risk factor for poor prognosis or tumour recurrence in PTC.

Sialendoscopy: a new diagnostic and therapeutic tool.

BACKGROUND: Sialendoscopy is a recently developed minimally invasive diagnostic and therapeutic procedure for the management of obstructive diseases of the salivary glands. This report describes our early experience with this new tool and compares our results with the literature data. MATERIAL AND METHODS: This was a retrospective analysis of the 33 first cases treated at a teaching hospital from October 2009 to June 2011. RESULTS: The success rate for diagnostic sialendoscopy was 94%. Sialolithiasis was found in 19 cases and salivary duct stenosis in 11; no canal anomaly was found in two cases. The success rate for stone removal was 79%, while treatment of strictures failed in four cases. Longer surgical experience led to shorter operating times and improved indications as well as better therapeutic outcomes. There were no complications. CONCLUSION: Sialendoscopy is a safe technique that can easily be learned by surgeons familiar with endoscopic surgery. However, practical experience is needed to reduce operating times, lower failure rates and determine its precise indications.

Management of voice prosthesis leakage with Blom-Singer large esophage and tracheal flange voice prostheses.

INTRODUCTION: Voice rehabilitation after total laryngectomy is a major socio-economic challenge. Voice rehabilitation can be achieved by voice prostheses, which provide rapid patient satisfaction. Enlarged tracheo-oesophageal puncture is a frequent complication and can be difficult to manage. MATERIAL AND METHOD: A prospective study was conducted from November 2010 to October 2011 on 28 Blom-Singer large oesophageal and tracheal flange voice prostheses placed in 18 patients with enlarged tracheo-oesophageal puncture causing leakage around the voice prosthesis. RESULT: Leakage around the voice prosthesis resolved in all patients with a mean prosthesis lifespan of 95 days. The patients considered voice quality to be similar to that obtained with the initial voice prosthesis. CONCLUSION: The Blom-Singer large oesophageal and tracheal flange voice prosthesis is a useful solution for the management of periprosthetic leakage ensuring similar voice quality and an identical lifespan to that of other voice prostheses.

Acquired cholesteatoma in children: strategies and medium-term results.

OBJECTIVES: To assess paediatric cholesteatoma surgical management strategies, residual disease and recurrence rates and especially the medium-term auditory impact. MATERIAL AND METHODS: Retrospective study of 22 cases of acquired middle ear cholesteatoma selected from a series of 77 children under the age of 16 operated for cholesteatoma between 1st January 2000 and 31st December 2003 on the basis of the following criteria: first-line surgical management with postoperative follow-up greater than four years. Surgical strategies, preoperative and postoperative (at 1 year and at the final visit) audiograms and residual disease and recurrence rates were analysed. RESULTS: A canal wall up tympanoplasty was performed in 82% of cases as first-line procedure and a canal wall down tympanoplasty was performed in 32% of cases. Residual cholesteatoma was observed in 9% of cases and recurrent disease was observed in 18% of cases. The mean preoperative hearing loss was 26dB with an air-bone gap of 23dB with values of 26dB and 20dB respectively at the end of follow-up. CONCLUSION: The majority of children were operated by two-stage canal wall up tympanoplasty. Long-term hearing results remained stable and close to preoperative values. The recurrence rate (residual disease and relapse) was low (27%), as reported in the literature.

Laryngeal abnormalities are frequent in the 22q11 deletion syndrome.

OBJECTIVES: The 22q11 microdeletion is a chromosomal disorder detected by fluorescence in situ hybridization (FISH). It has been known since the 80s, and is involved in many malformative syndromes (DiGeorge sequence, VCFS syndrome, etc.). Airway abnormalities are frequently localized in the larynx, as reported in the following series. METHODS: A retrospective chart review of laryngeal abnormalities and 22q11 deletion in a tertiary referral center. RESULTS: Five cases of laryngeal abnormalities associated to 22q11 deletion syndrome (DS) were found in a series of 35 cases. Abnormalities encountered were subglottic stenosis (3%), glottic web (9%), laryngeal paralysis (9%), vocal nodule (3%), laryngomalacia (3%) associated with bronchial malposition (3%). CONCLUSION: Laryngeal abnormalities are relatively common (14% in this series) and important to recognize with the 22q11 deletion syndrome, especially if cardiac surgery is planed. Conversely, in case of laryngeal abnormalities associated to other malformation (like facial dysmorphia or cardiac malformation), the 22q11 deletion must be searched.

Acute mastoiditis in children: 10 years experience in a French tertiary university referral center.

Acute mastoiditis have been increasingly reported in the last decade, with bacteriologic modifications and new therapeutic guidelines. This study is a retrospective review of 36 children hospitalized for acute mastoiditis in a French tertiary university referral center from 1999 to 2009, to assess incidence, bacteriology of pathogens and management. There were 10 periosteitis and 26 subperiosteal abscesses. There was a trend toward increased incidence over the past 10 years. The mean age of the patients was 31.8 months. A total of 63.2% children received 7.2 days antibiotic prior to hospitalization for acute otitis media, with sensitive pathogens in 80%. The pathogens were Streptococcus pneumoniae (36.1%), S. pyogenes A (13.9%), Staphylococcus coagulase-negative (13.9%), Pseudomonas aeruginosa (8.3%), Fusobacterium necroforum (8.3%) and Haemophiluss influenzae (2.8%). Cultures were negative in 16.7%. All patients received intravenous antibiotics. Eleven patients underwent bilateral myringotomy with or without tympanostomy tubes. Mastoidectomy was performed in 24 patients. Decrease in the length of hospitalization and delay from admission to surgery were significantly correlated. We observed a trend in the increase of acute mastoiditis at our center. The pathogens were dominated by S. pneumoniae. F. necroforum and P. aerguginosae were pathogens found in children over 2 years of age. Mastoidectomy was performed in 92.3% of subperiostal abscesses. However, there has been a trend toward conservative nonsurgical treatment in recent reported studies and further prospective studies are warranted to evaluate the long-term sequelae.

Basaloid squamous cell carcinoma of the head and neck: report of 18 cases.

OBJECTIVE: To evaluate the clinical course and pathological characteristics of basaloid head and neck squamous cell carcinoma. METHOD: Retrospective study of 18 cases of basaloid head and neck squamous cell carcinoma. Epidemiological, clinical and histological data were analysed and the Kaplan-Meier test used to estimate survival rates. RESULTS: The majority of lesions were at an advanced stage. These lesions were primarily localised in the larynx, hypopharynx and oropharynx. Routine pre-therapeutic assessment of squamous cell carcinoma was performed. Pathological diagnosis was difficult, although immunostaining was extremely useful. Positive staining for KL1, MNF 116 and 34ßE12 and negative immunostaining for chromogranin and synaptophysin were also important factors in obtaining a definitive diagnosis. In the majority of cases, treatment involved surgery and radiotherapy. The five-year survival rate was 5 per cent. CONCLUSION: Basaloid squamous cell carcinoma is an uncommon head and neck lesion, with a challenging histological diagnosis. These lesions must be carefully monitored due to their aggressive course, and require multimodality treatment.

[Role of hypnotherapy in the treatment of debilitating tinnitus]. / Intérêt de l'hypnose dans le traitement des acouphènes invalidants.

OBJECTIVE: Hypnotherapy is currently used for tinnitus therapy in our university hospital. The aim of this study was to evaluate its efficacy. MATERIAL AND METHODS: This study was performed on 110 patients suffering from distressing tinnitus. They were treated during five sessions with hypnotherapy, supplemented by instruction on self-hypnotherapy. A subjective evaluation was done by the practitioner at the end of the sessions of hypnotherapy. Then a questionnaire on psychologic distress (Wilson 1991) was sent retrospectively to the patients. RESULTS: We received 65 responses which were used for this study. Before treatment, the mean value of the Wilson score was 54 (28-104). After treatment, it was: 31 (0-86). 69% of the patients felt an improvement > or = 5 points Wilson score. These results were compared with the evaluation carried out by the practitioner at the end of the sessions of hypnosis. There was a "significant correlation" between the evaluation of the felt benefice, analyzed by the practitioner at the end of the sessions of hypnosis, and by the patient questioned long after the treatment. These results had significant correlation with the evaluation made by the therapist at the end of the five sessions of hypnotherapy. They show, how effective (68% improvement) this therapeutic approach can be. CONCLUSION: Hypnotherapy can be regarded as an effective treatment against distressing tinnitus.

[Thyroid differenciated carcinoma in children. Study from Normandy]. / Cancers thyroïdiens différenciés de l'enfant. l'expérience normande.

OBJECTIVE: Definition of a strategy for the management of thyroid differenciated carcinoma in children. DESIGN AND SETTING: Retrospective cohort study from the Normandy area in France. METHOD: Analysis of the medical records of 13 children and adolescents (age > 15 years), presenting with thyroid differenciated carcinoma in three Normandy French hospitals from 1994 to 2006, to determine the clinical features and treatment of the disease. RESULTS: X of the patients were male and y were female, with a mean age at presentation of 11 years. Most frequently symptom was solitary nodes in the thyroid gland (69%). Most frequent histological type was papillary cancer (92%). Size of tumor was > 4 cm in 23% of cases. Children had undergone surgery with total thyroidectomy, radio-iodine treatment and suppressive hormonotherapy. We observed 46% post surgery complications. All patients were alive and none developed a recurrence. CONCLUSION: Thyroid differenciated carcinoma in children and adolescents were more agressif with most frequently metastasis and recurrence than thyroid differenciated carcinoma of adults. Pronostic is good with 90% of survival at 20 years. We propose a coherent plan of treatment: 1. Thyroidectomy with cervical central lymph node dissection (group VI) completed bilateral selected head neck dissection compartments (groups IIa, III, IV) if macroscopic lymph node metastases in lateral cervical compartment. 2. Postoperative radioiodine is done in all tumor > T1N0 and completed with hormonotherapy.

[CT scan, MR imaging and anatomopathologic correlation in the glottic carcinoma T1-T2]. / Corrélation TDM, IRM et anatomopathologique dans les cancers glottiques T1-T2.

OBJECTIVES: Endoscopy and imaging are necessary to diagnose glottis carcinoma. Today, CT scan is the gold standard but MR imaging should be more sensitive for neoplastic invasion detection. The purpose of this study was to determine which exam to perform (CT scan or MRI) for neoplastic invasion. MATERIAL AND METHODS: This prospective study span a 18 months period. Seven patients with glottis carcinoma (TI-T2) underwent CT and MR imaging before surgery. Findings at imaging and pathologic examination were compared. RESULTS: Both CT and MR imaging were interesting, more specific (90%) than sensitive (74%). The anterior commissure, ventricles, subglottis, thyroid and arytenoid cartilages are the more difficult area analyzing. CONCLUSION: CT imaging stays the gold standard. MR imaging is more effective on second intention to refine the data. Indications are being improved, with complete cure and more preserved laryngealfunctions.

Endoscopic revision of external dacryocystorhinostomy failure.

OBJECTIVE: To evaluate the usefulness of endoscopic analysis and surgery of the lacrimal sac in cases of external dacryocystorhinostomy (DCR) failure. MATERIAL & METHOD: In a retrospective study, 17 endoscopic procedures in 17 patients during 11 years with recurrent lacrimal obstruction after external DCR were performed. Endoscopic analysis and procedures were carried out with a routine silicone tube catheterization for 3 to 6 months. RESULTS: In 13 patients, scar tissue was the cause of the obstacle, while in 3 patients an unsuitable location of the ostia and in 1 case an inflammatory polyp were found. After a long-term follow-up (56 months), the epiphora was controlled in 94% of the cases. The mean delay between the first DCR and the recurrence of epiphora was 22 months. CONCLUSION: A persistent or recurrent epiphora can be explored after an external procedure and treated by endoscopic procedure. The endonasal approach for DCR was considered safe, and effective particularly in patients with unsuccessful external DCR.

[Comparison of two antibioprophylaxis protocols in ear surgery]. / Comparaison de deux protocoles d'antibioprophylaxie dans la chirurgie de l'oreille.

INTRODUCTION: During surgical procedure, antibioprophylaxis is known to decrease bacterial proliferation and limit postoperative complications such as infections. In France, antibiotic prescription guidelines have been established for ear surgery, but applied with discrepancies. The purpose of the study was to evaluate the necessity of antibioprophylaxis in ear surgery. MATERIAL AND METHODS: Retrospective study of two consecutives series of ear surgery with two different antibioprophylaxis protocols. In the first series (n=100), antibioprophylaxis by amoxicillin and clavulanic acid was given only in cases of chronic otitis media with otorrhea and cholesteatoma (contaminated surgery). In the second series (n=107), no antibiotic was administered. The number of infected complications was evaluated by reviewing medical charts. RESULTS: The percentage of infected complications was 5% versus 6.5% in the second series (with no antibioprophylaxis), for all types of ear surgery, 9.4% versus 4.2% after ear contaminated surgery. No statistical difference was observed between the two series. CONCLUSION: In ear surgery, postoperative infected complications do not more frequently occur without antibioprophylaxis, either in otorrhea or in cholesteatoma surgery.

Quality of life in head and neck cancers patients: predictive factors, functional and psychosocial outcome.

The principal endpoints in head and neck cancer are survival with improvement of quality of life (QoL) in cancer patients. Patients treated for head and neck cancer suffer from a number of symptom domains: physical symptoms linked to diet and feeding, communication disorders, pain and their general state of health; psychological symptoms including depression, irritability, loss of self-esteem (occasionally feelings of shame), and social symptoms including relationship difficulties with partner (sexual disorders) or with other family members, loss of work, reduction in salary, and sense of uselessness, resulting in a negative impact on their daily life. At present, most tools only partially evaluate patient QoL, concentrating on the global impact of disease and its treatment on patients' physical and psychological condition. The "sociability" of individual patients is rarely evaluated, and the development of qualitative studies in this domain will enable improved understanding of the social factors involved in each patient's adaptability to disease, its treatment and after-effects.

[Laryngeal schwannomas]. / Schwannomes du larynx.

Laryngeal schwannoma is a rare benign tumour of the larynx. Schwannomas derive from the Schwann cells. The majority occurs in supraglottic area and may arise at any age. Magnetic resonance imaging (MRI) is the best imaging tool for suggesting the diagnosis in the appropriate clinical context. Conservative surgery is the treatment of choice. These tumors have to be distinguished from neurofibromas. We report 2 cases of laryngeal schwannoma. The first one concerned an 8-month-old baby with a laryngeal stridor history which seemed to be related to laryngomalacia. The second case was a 22-year-old man with a 5-year history of dysphonia. On nasofibroscopic examination, a sub mucosal mass enlarged the posterior part of the right false vocal cord in both cases. Both CT scan and MRI sequences showed a well margined lesion in the same place. Histological biopsies identified a schwannoma. External surgeries were subsequently and successfully performed. Diagnosis (imaging), specific treatment (endoscopic or external surgery), and possible association of laryngeal schwannomas are discussed.

[Primary malignant tumors of the lacrimal sac and nasolacrimal duct]. / Les tumeurs malignes primitives des voies lacrymales.

OBJECTIVES: To assess the clinical characteristics, the workup, therapeutic approach and pathological findings of primitive tumors of the lacrimal sac. MATERIAL AND METHODS: Three cases were retrospectively reviewed from the database of our ENT department. They were analyzed and compared to cases reported in the literature. RESULTS: One adenoid cystic carcinoma and two adenocarcinomas were observed, all were adults and the sex ratio was one man for 2 women. Clinically they presented neither significant lacrimal or rhinologic sign. A CT-scan and a MRI were performed along with a physical examination. An entire surgical resection was done with subsequent postoperative radiotherapy. Two patients died, and one is alive, free of disease. CONCLUSION: Lacrimal tumors should be considered in the differential diagnosis of a banal chronic epiphora. The ENT practitioner must associate this factor to any clinical evaluation. The management of these lesions requires a complete surgical resection, routinely associated with postoperative radiation.