Cauterization for epistaxis in hereditary hemorrhagic telangiectasia.

UNLABELLED: PROBLEM/OBJECTIVES: Epistaxis is the most common manifestation of hereditary hemorrhagic telangiectasia (HHT); it is present in >90% of HHT patients > 45 years old. Depending on severity, treatment consists of managing bleeding via medical and surgical methods. The aim of our study was to evaluate the efficacy and safety of endonasal surgical arterial cauterization to treat chronic epistaxis in patients with HHT who are no longer responding to first-line therapies. METHODOLOGY: Five patients were included in our study. The day before surgery, all patients underwent devascularization embolization of the external carotid arterial branches involved in nasal bleeding. The primary efficacy endpoint of treatment was assessed by the Epistaxis Severity Score (ESS), which was systematically evaluated preoperatively in our department, as well as every 3 months postoperatively. Data were collected retrospectively from the medical records of patients. RESULTS: Endonasal surgical arterial cauterization was associated with a ≥ 50% reduction in the ESS up to 9 months postoperatively. In one of our patients, cerebrospinal fluid (CSF) leakage occurred intraoperatively during cauterization of the posterior ethmoid artery. Closure of the dural skull base defect was successfully performed intraoperatively. DISCUSSION: Although our results are preliminary and included few patients, they support a role for endonasal surgical arterial cauterization as a second-line treatment method for chronic epistaxis in patients with HHT.

[Tinnitus management in 2013]. / Les acouphènes: quelle prise en charge en 2013?

Tinnitus is a common symptom, affecting nearly 10% of the adult population. Even if most of the patients support it, it can be particularly disabling in some cases. Recent advances in neuroscience have greatly improved the understanding of its pathophysiology, resulting in development of new therapeutic strategies. The management of tinnitus requires a systematic and rational approach to avoid incomplete diagnosis procedures and unnecessary exams. The diagnostic approach will be held to identify the causal pathology. Etiological treatment, if it is possible, will be the first therapeutic step. Various symptomatic treatments, such as tinnitus retraining therapy, cognitive behavioral therapy, or neuromodulation will promote habituation to the tinnitus, to ensure that the latter won't cause discomfort anymore.

Minimally invasive endoscopic unilateral transsphenoidal surgery for pituitary adenomas.

OBJECTIVE: The aim of this study was to analyse the results of minimally invasive endoscopic unilateral transsphenoidal surgery for pituitary adenomas. METHODS: A series of 83 patients presenting with a pituitary lesion was reviewed retrospectively. Surgical procedures were performed between February 2007 and December 2010. The extent of resection was evaluated on post-operative contrast-enhanced MRI. We also reviewed our complications and compared them with the literature. RESULTS: Our series included 10 micro- and 73 macro-adenomas, with cavernous sinus invasion in 60.3%. Fifty-three per cent were non-secreting. Complete resection of the macro-adenomas was achieved in 22.5% of the cases. Complications were as follows: 2 deaths (2.5%), 2 sinusitis (2.5%), 5 CSF leaks (6.2%), 1 meningitis (1.2%), 16 post-operative anterior lobe insufficiency (19.8%) and 4 persistent diabetes insipidus (4.9%). Thirty-seven patients (44.6%) presented with a compression of optical pathways with an improvement of visual acuity in 94.3%. CONCLUSIONS: Purely endoscopic unilateral endonasal transsphenoidal adenoma resection is minimally invasive surgery leading to a good rate of gross total tumour resection and it is associated with a low complication rate.

Hearing outcome after gamma knife radiosurgery for vestibular schwannoma: a prospective Belgian clinical study.

INTRODUCTION: Leksel Gamma Knife (LGK) radiosurgery is a safe and efficient therapeutic approach for vestibular schwannoma (VS) with low side effects. The goal of radiosurgery is not necessarily to cause significant tumour necrosis or to obtain a complete radiographic response, but to halt the tumour's growth permanently through its biological elimination. The 2 major aims of radiosurgery for VS are long-term tumour control and functional hearing preservation. The purpose of this study is to report our experience with LGK radiosurgery in the management of VS and to evaluate the hearing preservation rate after a minimum one-year follow-up. MATERIAL AND METHODS: Between January 2000 and January 2011, 415 patients with unilateral VS underwent LGK radiosurgery at the University Erasmus Hospital of Brussels. There were 349 patients with previously untreated VS (86 grade I, 96 grade II, 141 grade III, 9 grade IVa, 17 unknown grades, according to Koos) and 66 patients with post-operative residual tumour. All patients in our series underwent evaluation with high resolution neurodiagnostic imaging including computed tomography and magnetic resonance imaging, and clinical evaluation as well as audiological tests that included tonal and speech audiometries. The Gardner Robertson (GR) classification is used to report the results of this study. We identified 276 patients treated for VS with LGK, tested and retested with speech and tonal audiometries by the same team, and followed for a minimum of one year. RESULTS: Before LGK, 144 patients had serviceable (85 GR class I and 59 GR class II) hearing; 95 (65.97%) of these patients had preservation of serviceable hearing (Pure tone average < or = 50 db and Speech discrimination > or = 50%) at minimum one-year audiological follow-up. It was observed that 44 of the 85 GR class I patients (51.76%) maintained their level of audition and 66 of these (74.64%) preserved serviceable hearing. In the 34 patients with preradiosurgery non-serviceable hearing (GR class III-IV) 25 of these patients (73.52%) maintained their hearing. The tumour was stable or declining in size in 90.44% of cases. CONCLUSION: LGK radiosurgery provides excellent tumour control in vestibular schwannomas and has low toxicity even after long-term follow-up.

Unusual presentation of adenoid cystic carcinoma of the maxillary antrum.

Unusual presentation of adenoid cystic carcinoma of the maxillary antrum. Adenoid cystic carcinoma (ACC) is a rare tumour which affects mainly the major and accessory salivary glands. It is an aggressive neoplasm characterized by early neural invasion and a high incidence of local recurrence and distant metastases, which may develop years after the initial resection. Surgery followed by radiotherapy seems to be the best treatment. Based on a well-illustrated case of extensive maxillary ACC involving the palate, orbit floor, and pterygo-palatine fossa, we discuss the prognosis and the importance of an early diagnosis of ACC.

[Combined middle and inner ear abnormality: report of a case]. / Anomalie combinée de I'oreille moyenne et de l'oreille interne: a propos d'un cas.

Congenital anomalies of the middle ear associated to anomalies of the inner ear are rarely described. Our case displays a malformation due to an association of congenital absence of the round window and aplasia of the semi-circular canals, causing conductive hearing loss. First diagnosis was a congenital atresia isolated of the round window, related to a previous surgical exploration. We tried a novel technique designed to restore the function of the round window. Labyrinthine anomalies prevented a good outcome. This kind of malformative association could be due to the complexity of early genetic control of ear development. Attentive analysis of imaging should be performed before middle ear surgery to determine the presence of inner ear anomalies which are quite frequent and which would limit its usefulness.

Carotid artery occlusion due to fulminant rhinocerebral mucormycosis.

Mucormycosis is a rare disorder with a potentially lethal course caused by an opportunistic fungus of the Phycomycetes family. Rhinocerebral mucormycosis (RCM) is an acute fulminant form of invasive fungal sinusitis occurring principally in individuals who are immunologically or metabolically compromised. This form originates from one of the paranasal sinuses and may spread to the orbit or the intracranial space either by direct extension or using the unique capability of spreading along vascular walls, leaving the bony structure intact. Carotid artery occlusion is a complication of RCM. Because of the rapidity with which this disease progresses, prompt and aggressive therapy is essential. The morbidity and mortality of this disease are directly related to the length of time before diagnosis and treatment. The diagnosis is difficult because invasive mucormycosis may be present with no mucosal changes and a normal sinus CT scan. It is a histological diagnosis. Treatment of RCM includes early diagnosis, correction of underlying conditions, early and radical surgical debridement, and lipid formulation of amphotericin B. Multiple surgeries are often necessary for adequate debridement. We report a case of carotid artery occlusion due to RCM and discuss the literature on its early signs and symptoms, pathophysiology, and treatment options.

[External auditory meatus adenocarcinoma]. / Un adénocarcinome du méat acoustique externe.

We report the case of a man presenting a deafness and a hemorrhagic ear discharge since one year. CT scanner and MRI reveal an invasive tumoral lesion of the external auditory meatus (EAM) expending into the posterior fossa. After surgery the diagnosis of high grade ceruminal gland adenocarcinoma is established whereas the malignancy was not obvious on earlier biopsy. Cancers arising in the EAM are uncommon and are essentially representating by squamous cell cancers and basal cell cancers. The precise diagnosis of a glandular tumor is a challenge for the pathologist because the limits between benign and malignant tumors are not obvious. Integration of clinical and radiological behavior and the histology of the tumor is necessary for a early diagnosis and a complete surgery.

Preservation of hearing in vestibular schwannomas treated by radiosurgery using Leksell Gamma Knife: preliminary report of a prospective Belgian clinical study.

INTRODUCTION: Radiosurgery is an alternative to the microsurgical resection of vestibular schwannoma (VS). Since its introduction, radiosurgery has been used in more than 8000 patients with VS worldwide and the long term tumor control rates are reported to be 86 to 100%. The aim of this study is to report our experience with Leksell Gamma Knife (LGK) radiosurgery in the management of VS and to evaluate the serviceable hearing preservation rate after one-year follow-up. MATERIAL AND METHODS: Between January 2000 and October 2002, 95 patients with unilateral VS underwent LGK radiosurgery at the University Erasmus Hospital of Brussels with the first worldwide installed LGK C. All patients in our series underwent evaluation with high resolution neurodiagnostic imaging including computed tomography and MRI, and clinical evaluation as well as audiological tests that included tonal and vocal audiometries. The Gardner Robertson (GR) classification is used to report the results of this study. We identified 48 patients treated for VS with LGK, tested and retested with vocal and tonal audiometries by the same team, and followed for a minimum of one year. There were 38 patients with previously untreated VS (9 grade I, 9 grade II, 20 grade III according to Koos) and 10 patients with postoperative evolutive residual tumor. RESULTS: Before LGK, 24 patients had serviceable (17 GR class I and 7 GR class II) hearing; 16 (67%) of these patients had preservation of serviceable hearing (Pure tone average < 50 db and Speech discrimination > 50%) at the one-year audiological follow-up. It was observed that 9 of the 17 GR class I patients (52.9%) maintained their level of audition and 14 of these (82.3%) preserved serviceable hearing. No deterioration of hearing occurred in the 7 patients with preradiosurgery radiosurgery nonserviceable hearing (GR class III) at the one-year follow-up. One patient improved from GR class V to III after LGK. No patient developed trigeminal neuropathy and only one patient who had preradiosurgery facial nerve dysfunction experienced deterioration at one-year follow-up. CONCLUSIONS: In view of the high tumor control rate and excellent long-term cranial nerve preservation rates, LGK radiosurgery should now be considered as an excellent alternative strategy to microsurgery for the management of VS grade I to III as well as in cases of residual tumor after microsurgery. Compared to results obtained in centers with long-term experience, our data suggest that LGK radiosurgery is an efficient reproducible therapeutic approach that offers high rate of hearing preservation. This justifies the choice of radiosurgery as the first treatment option in VS.

Bilateral vocal fold palsy caused by chronic motor axonal neuropathy.

The authors report a first case of chronic motor axonal neuropathy involving ENT manifestations, in a 64-year-old male presenting with gait difficulties, effort dyspnoea and dysphonia. Eleven months after the first symptoms, he developed severe hypoventilation, limb weakness and bilateral vocal fold palsy and had to be intubated for respiratory failure. The diagnosis of chronic motor axonal neuropathy was suspected on clinical and electrophysiological grounds. The patient improved dramatically after a five-day course of 0.4 g/kg intravenous immunoglobulin. He is still being treated with methylprednisolone 0.5 mg/kg every other day and remains stable. We conclude the bilateral vocal fold palsy may be associated with chronic motor axonal neuropathy and that the immunosuppressive treatment may be effective in such cases.

General Shrapnel and the "unfortunate" Dr. H. J. Shrapnell.

Several aspects of Shrapnell's career are still little known or erroneously typed. 1. Some historians of Medicine profess that Dr. Shrapnell was the inventor of a shell which bears his name. 2. Others neglect the negative points of Shrapnell's paper published in 1832 on the pars flaccida. To be able to comment on these different aspects of Shrapnell's life and work we compared the biographies of the anatomist and of the artillery officer. We noticed the positive and also the negative points of his paper. We conclude that: 1. Dr. Shrapnell was not an artillery officer, but the two men were present in the British Army; their surnames, their names and the period when they lived were about the same. 2. Errors in Shrapnell's paper were due to the philosophy and lack of anatomical and pathological knowledge of the time. 3. Otologists' reception was better than that of the Anatomists because for the Otologists, Shrapnell's membrane was recognized as an important original area of cholesteatoma, whereas for the Anatomists it was just a detail.

Systemic leishmaniasis involving the nose.

A case of a patient, presenting with a granulomatous lesion of the anterior nasal septum mucosa spreading to the columella and the nasal floor, whereby leishmaniasis was diagnosed, is presented. The clinical and pathological aspects of this pathology, its diagnosis and treatment are reviewed.

Cervical thymic cyst: a case report.

This paper describes the case of a young man who presented with a mass in the left side of his neck. Surgical excision revealed an ectopic thymic cyst. Thymic cyst should be included in the differential diagnosis of neck masses, especially in children. The authors review the embryology, diagnosis, and management of this pathology.

UPPP for snoring: long-term results and patient satisfaction.

We retrospectively survey 57 patients who underwent uvulopalatopharyngoplasty (UPPP) because of habitual snoring over a five-year period. A total of 100 patients were sent questionnaires concerning persistent snoring, excessive daytime sleepiness (EDS), body mass index (BMI) and postoperative complications. After 5 years the success rate was 53%. The mean snoring score was 16.7 preoperatively and decreased to 10.6 postoperatively. There was a relationship between the body mass index (BMI) preoperatively and the efficiency of the surgery. The mean EDS score was 11.1 before and 9.4 after surgery. UPPP in patients complaining of snoring is quite successful but the results decline significantly with time and patients should be warned of the possibility of snoring remaining or returning.

Unknown primary detected by FDG-PET. A review of the present indications of FDG-PET in head and neck cancers.

To investigate the indications of 18F-2-fluoro-2-Deoxy-D-glucose (FDG) Positron Emission Tomography (PET) in head and neck cancer, the present study focuses on a case of cervical metastatic lymph node, which is not detected by the routine approach. It discusses the potential role of FDG-PET in the detection of unknown primary tumors, lymph node metastasis and post radiation follow-up, and demonstrates the implications of its findings through a few examples. Based on the literature in the field of head and neck oncology, the paper recommends the following uses for FDG-PET: 1. To guide biopsy or even local resection at the initial stage of examining the unknown primary lesions in case of high clinical suspicion 2. A whole body PET in high-risk patients may prevent unnecessary treatment and reduce the number of examinations 3. To monitor tumor response before full-dose irradiation so as not to delay the salvage surgery when applicable 4. To detect residual, recurrent or secondary neoplasm after definitive radiotherapy at least 4 months post-treatment 5. To revise the necessity of neck treatment in case of a negative PET, in the NO necks; and 6. In cases of clinical suspicion for laryngeal cancer recurrence and absence of objective findings before obtaining biopsy.

[The oto-rhino-laryngology and cervico-facial surgery department]. / Le service d'Oto-Rhino-Laryngologie et de Chirurgie Cervico-Faciale.

ENT department was present at the opening of the hospital but its activity began to increase in 1989. Now all the fields of modern ENT are present due to the collaboration of physicians very specialized in otology, rhinology, laryngology and head and neck surgery. Drilled in all surgical techniques using the best technologies available, the members of our team are also working in collaboration with the Laboratory of Histopathology in the field of nasal inflammation, cholesteatoma and malignant tumors of head and neck area.

Detection of macrophage migration inhibitory factor (MIF) in human cholesteatomas and functional implications of correlations to recurrence status and to expression of matrix metalloproteinases-3/9, retinoic acid receptor-beta, and anti-apoptotic galectin-3.

OBJECTIVES: To investigate whether the expression of the macrophage migration inhibitory factor (MIF) 1) is detectable, 2) changes in relation to recurrence and infection status, and 3) relates to the levels of expression of growth regulators/differentiation markers, including galectin-1, -3, and -8, retinoid acid receptors (RAR)]-alpha, -beta, and -gamma, binding sites for sarcolectin, and invasion markers (cathepsins -B and -D, and matrix metalloproteinases [MMP]-2, -3, and -9) in human cholesteatomas. STUDY DESIGN: An analysis of 56 cholesteatomas resected by the same surgeon using canal wall up and canal wall down surgical procedures. METHODS: The immunohistochemical levels of expression of MIF and the proteases were quantitatively determined (using computer-assisted microscopy) on routine histologic slides by specific antibodies, and statistically correlated to parameters of the other markers determined previously in conjunction with data on apoptosis/proliferation. RESULTS: MIF expression was detected. It was significantly higher in the epithelium (P =.002) and vessels (P =.04) of the connective tissues (but not in the connective tissue itself) of recurrent as opposed to non-recurrent cholesteatomas. The MIF expression is significantly correlated (P =.006) to the RAR beta expression in non-infected cholesteatomas, and to MMP-3 (P <.01) and anti-apoptotic galectin-3 (P =.01) in infected cholesteatomas. The level of MIF expression was also correlated significantly to MMP-9 (P = 0.003), RAR beta (P <.001), and galectin-8 (P =.003) expression in the cholesteatomas regardless of their infection status. CONCLUSIONS: MIF expression in human cholesteatomas is related to the levels of biologic aggressiveness reflected in their recurrence status and MMP expression, and to the differentiation status reflected in their galactin and RAR beta expressions. Together with galectin-3, it could cooperate to form an anti-apoptotic feedback loop.

The levels of expression of galectin-3, but not of galectin-1 and galectin-8, correlate with apoptosis in human cholesteatomas.

OBJECTIVES: To investigate whether galectins 1, 3, and 8 are expressed in human cholesteatomas and whether any such expression does correlate with the level of apoptosis, which is, as we have previously shown, predictive of recurrence.7 STUDY DESIGN: The analysis of 52 cholesteatomas resected by the same surgeon by means of canal wall up and canal wall down procedures. METHODS: The immunohistochemical levels of expression of galectins 1, 3, and 8 were quantitatively determined (using computer-assisted microscopy) on conventional histological slides by means of specific anti-galectin-1, anti-galectin-3, and anti-galectin-8 antibodies. The level of apoptosis in each cholesteatoma under study had already been determined 7 by means of the in situ labeling of nuclear DNA fragmentation (Tolt-mediated dUTP nick end labeling [TUNEL] staining). RESULTS: Galectin-1 was expressed markedly in both the epithelial and the connective tissue areas of all the cholesteatomas under study. The levels of expression of galectin-3 and galectin-8 were considerably lower than that of galectin-1. The level of expression of galectin-3 correlated both highly and positively with the level of apoptosis. CONCLUSIONS: An upregulation of galectin-3 (known to have an antiapoptotic and antianoikis effect in certain model systems) expression, which is associated with pronounced apoptotic activity, could have a physiologically protective effect against the characteristically substantial apoptotic features occurring in recurrent cholesteatomas.

The levels of retinoid RARbeta receptors correlate with galectin-1, -3 and -8 expression in human cholesteatomas.

Cholesteatoma is a benign disease characterized by the presence of an unrestrained growth and the accumulation of keratin debris in the middle ear cavity. This often recurs, even when surgical resection is thought to be complete. In a previous study we showed that cholesteatomas with the highest apoptotic indices recurred more rapidly and also exhibited a high level of p53 immunopositive cells. In view of their relevance to the characterization of the cell differentiation status, the present study focuses on the expression of retinoid acid receptors (RARs) and galectins in human cholesteatomas. Retinoids control the differentiation processes in keratinocytes while galectins play strikingly modulatory roles at apoptosis and cell adhesion levels in a wide variety of tissue (embryonic, normal and neoplastic). To clarify the expression of these two protein families in human cholesteatomas we examined and quantified the levels of immunohistochemical expression of RARalpha, beta and gamma, and also galectin-1, -3 and -8 in a series of 70 human cholesteatomas. Our data show clearly that predominantly RARbeta and galectin-1 were expressed. The RARgamma concentration was significantly lower than that of the RARalpha; this was also observed for the galectin-8 concentration in comparison with the galectin-3 one. Furthermore, the level of RARbeta expression correlated highly (P=0.00001) with the level of galectin-8 expression, which also correlated significantly with the level of RARalpha and RARgamma expression. In addition, this parameter also correlated with the level of galectin-1 and galectin-3 expression. These data suggest that cholesteatomas may originate in an undifferentiated population of keratinocytes, and that a relation may exist between retinoid activity and galectins.

Mucins and secreted factors.

Mucin is a large glycoprotein (M up to 4-6.10(6)) with a high content of serine, threonine, and proline residues and numerous O-linked saccharides, often occurring in clusters on the polypeptide. Nine mucin genes exist in humans that encode an apomucin highly modified by O-glycans in forming epithelial mucins. These are variably expressed by epithelial cells of the gastrointestinal, tracheobronchial and reproductive tracts. They may be found either membrane-associated or secreted. As might be imagined from their ubiquitous and complex nature, the biological roles of glycans are quite varied. Protection against infection is an important biological role. Alterations in their carbohydrate moiety have been reported in airway and salivary mucins secreted by patients suffering of cystic fibrosis (CF). Moreover, changes in expression of glycans are also often reported in the setting of transformation and progression to malignancy. Due to the key roles played by glycans of glycoconjugates in both physiological and pathological events, glycobiology and carbohydrate chemistry have become of increasing importance in modern biotechnology.