Hearing preservation and tumour control after radiosurgery for NF2-related vestibular schwannomas.

OBJECTIVES: We analyzed the effects of stereotactic radiosurgery on tumour control and cranial nerve function in patients with vestibular schwannomas (VS) secondary to neurofibromatosis type 2 (NF2). Irradiation was performed with a Gamma Knife, model C equipped with a high-precision, robotized positioning system (APS). METHODOLOGY: This study included 18 patients with 25 VSs secondary to NF2 that were treated from 2001 to 2010 with radiosurgery at our Gamma Knife Center. The radiosurgical procedure included high-resolution conformational dose-planning with multiple, small-diameter isocenters, a single-fraction, low-dose irradiation prescription, and highly accurate gamma rays delivery to the target with the APS. RESULTS: The median follow-up time was 4.4 y. For 16 tumours in 12 patients with available follow-up data, we observed an actuarial tumour control of 87.5% at 2 y and 80.2% at 5 y, based on the Kaplan-Meier method. No patient developed facial weakness. Serviceable hearing was preserved in 78% of cases. Patients treated for bilateral and unilateral tumours had similar outcomes. CONCLUSIONS: Radiosurgery could control tumour growth and preserve hearing function and facial weakness in patients with VS secondary to NF2. The enhanced techniques of radiosurgical irradiation provided with the Gamma Knife model C have improved the results of this treatment alternative to microsurgery.

Lobular capillary haemangioma of the nasal cavity during pregnancy.

OBJECTIVE: We report a pregnant patient with a rapidly growing mass within the nasal cavity, which required pre-operative super-selective embolisation and subsequent removal under general anaesthesia after childbirth. We also discuss the clinical, radiological and histological characteristics of lobular capillary haemangioma, and its treatment. METHOD: Case report and literature review. RESULTS: Lobular capillary haemangioma is a benign lesion originating in the vascular tissue of skin, mucosa, muscles, glands and bone. These lesions grow rapidly. Nasal localisation is rare. Microtrauma and pregnancy are the most commonly proposed aetiological factors. Reported incidence during pregnancy ranges from less than 2 per cent to approximately 5 per cent. The management of a pregnant woman with such a lesion may be complex, and depends on the severity of symptoms and the status of the pregnancy. Complete surgical excision, with or without pre-operative embolisation, is the treatment of choice. CONCLUSION: This uncommon lesion should be considered in any pregnant patient with a mass in the mouth or nasal cavity.

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.

Regulation of expression of galectin-7 in human nasal polyps by budesonide.

BACKGROUND: Nasal polyposis is a model for the study of inflammatory processes. We analyzed the expression of galectin-7, a growth regulator, in surface epithelium, glandular epithelium, and connective tissue in human nasal polyps, and examined the effect of the glucocorticoid budesonide on its expression in human nasal polyps ex vivo. METHODS: Using quantitative, computer-assisted microscopy and immunohistochemistry, we measured galectin-7 expression in nine nasal polyps obtained by surgical resection. Five polyps came from allergic patients and four came from non-allergic patients. RESULTS: Galectin-7 was expressed in all three polyp tissues analyzed. Treatment of polyps from allergic and non-allergic patients with 50 ng/ml budesonide increased the extent of galectin-7 expression in the connective tissue (p = 0.01). Conversely, budesonide at this concentration did not apparently affect galectin-7 expression in glandular epithelium; only a slight decrease in the percentage of the galectin-7-immunopositive cells was observed. In the surface epithelium of nasal polyps from non-allergic patients, the percentage of galectin-7-immunopositive cells was decreased (p = 0.03) by treatment with 250 ng/ml budesonide. In nasal polyps from allergic patients, this percentage was increased by treatment with 50 ng/ml budesonide (p = 0.0001). CONCLUSIONS: These data are consistent with a role for galectin-7 in the regulation of cell growth through a pro-apoptotic effect. Galectin-7 expression coincides with the degree of epithelial stratification, and is subject to upregulation in the connective tissue in response to treatment with 50 ng/ml budesonide. Budesonide modulates galectin-7 expression differently in the surface epithelia of polyps from allergic and non-allergic patients.

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.

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.

[Rehabilitation after shoulder rotator cuff surgery: in-patient or day-hospitalization (about 76 cases)]. / Rééducation des ruptures de coiffe de l'épaule opérées: prise en charge en hospitalisation en internat ou en hospitalisation de jour (à propos de 76 observations).

OBJECTIVE: To define criteria of orientation in day-hospitalized or in-patient after shoulder rotator cuff surgery. METHOD: Population of 71 patients (53.2 years +/-7) (76 shoulders) operated for rotator cuff tear by the same surgeon, then treated by physical therapy either in day-hospitalization or in-patient. Weekly, evaluation until the second month after surgery by setting as criteria: pain, passive and active range of motion and complications. RESULTS: Fifty-three shoulders benefited from rehabilitation therapy in hospitalization and 23 in day-hospitalization. There is no significant difference for criteria studied between the 2 populations except for pains at the fifteenth day after surgery. Pain and articular steepness seem more frequent if the surgery is complex. DISCUSSION: Day-hospitalization and in-patient give equivalent results, which explain themselves by the possibility of identical physical medicine and rehabilitation performances. The knowledge of the social context is important to choose the most adapted rehabilitation type because patients can benefit from assistance for the acts of the daily life and the domestic tasks. In-patient allows more comfort but implies a familial break.

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.

[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.

[Acute upper respiratory tract infections]. / Les infections aiguës des voies respiratoires supérieures.

Acute infections of the upper respiratory tract are reviewed from an ENT point of view. Only the most frequent pathologies are taken into account. Clinical presentation and an update of current treatment are presented.

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.

Glycohistochemical characteristics of nasal polyps from patients with and without cystic fibrosis.

OBJECTIVE: To investigate whether cystic fibrosis (CF)-related nasal polyps exhibit significantly distinct glycohistochemical characteristics when compared with single vs massive nasal polyps obtained from patients without CF. DESIGN: Glycohistochemical characteristics were identified by means of 8 histochemical probes, including 5 plant lectins (peanut, gorse seed, wheat germ, Maackia amurensis, and Sambucus nigra agglutinins), 2 animal lectins (14- and 16-kd galectins), and 1 neoglycoprotein (exposing the Thomsen-Friedenreich antigen). The binding of the 8 glycohistochemical markers was determined by means of computer-assisted microscopy. For each probe, 3 quantitative parameters were computed: the labeling index, which describes the percentage of tissue area specifically stained by a given marker; the mean optical density, which reflects the staining intensity; and the concentrational heterogeneity, which characterizes the level of heterogeneity of the staining intensity. SUBJECTS: A series of 61 nasal mucosa specimens was analyzed, including 6 normal cases, 23 single and 18 massive polyposis cases without CF, and 14 nasal polyps associated with CF. RESULTS: Normal and polyposal nasal mucosa differed in terms of the amounts and linkage types of sialic acids (revealed by the wheat germ, M amurensis, and S nigra agglutinins) rather than the characteristics of galactoside expression (monitored with the peanut agglutinin and 2 animal galectins). In contrast, nasal polyps markedly differed between patients with and without CF with respect to galactoside expression (revealed by the peanut agglutinin and the 14-kd galectin) and the display of binding site(s) for the neoglycoprotein. CONCLUSION: Normal and polyposal nasal mucosa differ essentially in sialic acid presentation, while nasal polyps from patients with CF have a higher level of various lectin-reactive galactoside residues than nasal polyps from those without CF.

Tonsillar lymphoma and AIDS.

A case of a patient presenting unilateral tonsillar enlargement, whereby non-Hodgkin lymphoma (NHL) and AIDS were diagnosed, is presented. The clinical and pathological aspects of this pathology, its pathogenicity and treatment are reviewed.

Laryngeal aspergillosis in an acquired immunodeficiency syndrome patient.

Aspergillosis is a rare infection. It varies considerably in its virulence depending on whether or not the patient affected is immunodepressed. The serious form is characterized by vascular invasion with haematogenic dissemination, tissue infarction and necrosis. The most often affected site is the lung. A laryngeal localization is exceptional and usually results from dissemination from a lower airways portal of entry. Diagnosis is difficult: cultures are only positive in 50 per cent of cases, while in 60 per cent of cases the characteristic histological features reminiscent of sprigs of mistletoe are only found on postmortem examination. In view of the high morbidity and mortality in acquired immunodeficiency syndrome (AIDS) sufferers, this condition must be treated early and aggressively. Treatment is based essentially on amphotericin B and itraconazole.

An unusual localisation of Kaposi's sarcoma: the external auditory canal.

The epidemic form of Kaposi's sarcoma is associated with human immunodeficiency virus infection. Cutaneous and mucosal manifestations are frequently reported in the ENT sphere, mostly involving the oral cavity. The external and middle ear are only rarely concerned with only one case of a mastoid lesion without extension to the external auditory canal (EAC) being reported to this day. The present article describes the first case of involvement of the EAC with extension to adjacent structures. This patient presented other Kaposi lesions and had been treated by systemic hormonal therapy. Thereafter local injection of a cytotoxic agent was given without effect. Finally, radiotherapy resulted in a 50% regression of the tumour mass. The epidemiologic factors and therapeutic modalities with their results are described.

Otogenic thrombosis of the lateral sinus: report of a case in a child.

A case of thrombosis of the lateral sinus complicating the mastoiditis in a five year old boy is presented. This intracranial complication of suppurative otitis media which has become rare since the use of antibiotics, still raises diagnostic and therapeutic problems. The various clinical and paraclinical elements of this rare pathology, its diagnosis and treatment are reviewed.