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[Cervical lymph node metastasis as a primary manifestation of esthesioneuroblastoma].

A 51-year-old male patient presented with right-sided swelling of the neck and a 2-year history of decreasing olfaction that had culminated in complete anosmia. CT and MRI showed a calcified ethmoid tumor and destruction of the lamina cribrosa extending into the anterior fossa and frontal lobes. Contrast material demonstrated marked enhancement on the CT and MRI. Bilateral angiography of the external and internal carotid arteries failed to show tumor vascularity typical for an esthesioneuroblastoma but rendered an olfactory meningioma unlikely. Histological findings of cervical lymph nodes removed during neck dissection showed infiltration by an esthesioneuroblastoma (Kadish C classification). Tumor calcifications in these neoplasms are generally well seen on CT and--while not specific--may suggest the diagnosis. CT and MRI are the most sensitive methods of demonstrating local tumor growth, metastasis and bone destruction.

Combined Modality Therapy↗

[Kallmann's syndrome in families].

UNLABELLED: The authors present the incidence of Kallmann's syndrome in two families in four persons. In one family the syndrome was present in a boy and the brother of his mother. In the second family in siblings -- a boy and a girl. The two boys at preschool age underwent a surgery because of bilateral cryptorchism. In the first patient and his uncle additional hearing dysfunction and agenesis of the left kidney were diagnosed. In all the patients anosmia was diagnosed. All the patients receive a pharmacological treatment which improved significantly their clinical state, caused the development of tertiary sexual feature and an improvement of the psychological condition. CONCLUSIONS: 1. In patients with an abnormal development of the urethro-sexual organs a diagnosis for other disturbances or developmental defects is necessary. 2. In patients with an abnormal urethro-sexual development a permanent care of a psychologist is necessary.

Adolescent↗

Craniofacial fibrous dysplasia. conservative surgical management. Review of literature and report of a case.

The present study presents a case of craniofacial fibrous dysplasia attenuating the orbit and the nasal airway, which was treated by conservative surgical approaches. Nasal and orbital decompression, and facial recontouring were performed simultaneously. This was done via a transconjunctival and transcaruncular approaches with a lateral canthotomy, which provided access to the four walls of the orbit, midface and the lateral nasal wall. The reduction of the turbinates was done through a standard turbinectomy approach and the nasal decompression was done via intercartilaginous access and a full transfixion incision and nasal degloving. The bulk of the lesion was removed with chisels, gauges, and rotary instruments. The orbital debulking extended to the orbital apex, which was not involved by the fibrous dysplasia. Approximately 200 mg of dysplastic bone was removed. The functional and aesthetic results were good. The symptoms of chronic conjunctivitis and nasal obstruction have been alleviated and there is no deterioration of his condition. The symptoms of anosmia have also disappeared. Conservative surgical treatment of craniofacial dysplasia in adults with stable disease is recommended.

Adult↗

Consequences of olfactory loss and adopted coping strategies.

UNLABELLED: The aims of this study were to investigate the effects of loss of smell as regards the quality of life and the coping strategies used. METHODS: Seventy-two patients with anosmia (46%) or hyposmia (54%) filled in the validated Multi-Clinic Smell and Taste Questionnaire, the validated General Well-being Schedule (GWBS), and answered other questions shown to be of good validity. RESULTS: Several kinds of negative effects, risks associated with the loss, interference with daily routines and deteriorations in well-being were common. Physical health, financial security, profession, partnership, friendship, emotional stability and leisure were also deemed to be negatively affected and GWBS scores show compromised psychological well-being. The importance of olfaction seemed to be more noticeable after the loss of smell, and several kinds of problem- and emotion-focused coping strategies were adopted by these patients. CONCLUSIONS: We found that the loss of smell had substantial adverse effects on the quality of life and that high priority should be given to its diagnosis and treatment and to further research in this field. Furthermore, a combination of problem- and emotion-focused coping strategies may be suggested to patients who have recently lost the sense of smell.

Adaptation, Psychological↗

MRI findings in Kallmann syndrome.

Kallmann syndrome (KS) is a neuronal migration disorder characterised by hypogonadotrophic hypogonadism and anosmia or hyposmia. Five patients with clinical findings suggestive of KS were evaluated with MRI. All patients had abnormalities of olfactory system. Olfactory bulbs were absent in all patients. Olfactory sulci were absent in 3 patients and hypoplastic in 2 patients. Anterior pituitary was hypoplastic in two patients. The MRI findings in KS are characteristic and MRI is a useful adjunct to the diagnosis of KS.

Adult↗

Olfactory neuroblastoma associated with Kallman's syndrome.

Kallman's syndrome is an unusual clinical entity that includes anosmia and hypogonadotropic hypogonadism. We present a patient in whom an olfactory neuroblastoma was discovered during evaluation of his Kallman's syndrome. While other craniofacial abnormalities have been associated with this condition, to our knowledge this is the first report of its association with olfactory neuroblastoma.

Adolescent↗

Arteries to the proximal part of the olfactory tract.

Destruction of the vascularisation of the olfactory structures during fronto-orbital surgical approaches to the sellar region may result in anosmia as a complication. The goal of this study was to describe the sources of blood supply to the proximal olfactory tract and the macroscopic distribution of these vessels. 20 human brains fixed in formalin with arteries injected with ink-coloured gelatine were studied using a surgical microscope and the micro-dissection technique. The vessels running along the olfactory tract posteriorly and anteriorly on its inferior and superior surface were observed. These arteries and arterioles were most often branches of the constant artery supplying the posterior part of the straight gyrus and orbital gyri (38/40). Similarly, as branches of the medial orbitofrontal artery (7/40), they were found on the superior aspect of the tract. Branches of the distal medial striate artery directed to the olfactory structures were observed on the basal surface of the tract (20/40).

Anterior Cerebral Artery↗

Chondrosarcoma of the nasal septum: a case report.

The nasal septum is a particularly rare site of origin of a chondrosarcoma. A 55-year-old man presented with complaints of nasal obstruction and anosmia of one year duration. A fragile mass was detected in the left nasal cavity. Computed tomography showed a large hypodense mass with scattered calcifications, eroding both ethmoid sinuses and extending to the left orbit. Following a biopsy, the tumor was resected via a lateral rhinotomy approach. No evidence for recurrent disease was detected during a three-year follow-up period. Although unusual in the nasal septum, chondrosarcoma must be considered in the differential diagnosis of calcified intranasal masses.

Bone Neoplasms↗

Luffa operculata affects mucociliary function of the isolated frog palate.

BACKGROUND: Luffa operculata is a medicinal plant used in homeopathic and alternative medicine. In the United States, it is sold in a purified spray form, whereas a homemade L. operculata dry fruit infusion (DFI) is commonly used in Latin America. The L. operculata DFI is applied intranasally, inducing profuse mucous secretion and relieving nasal symptoms. Nevertheless, this medication may cause irritation of the nasal mucosa, as well as epistaxis or anosmia. Given the growing popularity of alternative medicine, a decision was made to evaluate the effects of this substance on mucous membranes. METHODS: The effects of L. operculata DFI on mucociliary transport velocity, ciliary beat frequency, and transepithelial potential difference (PD) were evaluated in an isolated frog palate preparation. We tested 46 palates immediately before immersion and again at 5 and 20 minutes after immersion. Four groups (n = 10) were tested in frog Ringer: control; L. operculata DFI, 60 mg/L; 600 mg/L; and 1200 mg/L. An additional group was tested using L. operculata DFI prepared with water (600 mg/L of H2O, n = 6). Epithelial samples were harvested for ultrastructural study. RESULTS: In treated palates, mucociliary transport velocity and ciliary beat frequency decreased significantly (p < 0.001 and p < 0.008, respectively). There was a dose-dependent decrease in PD modulus (p < .007). Our PD findings indicated ion-fluid transport abnormalities, which were confirmed by transmission electron microscopy that showed enlargement of interepithelial spaces. CONCLUSION: In this ex vivo model, the L. operculata DFI infusion promoted significant changes in the mucociliary function of the epithelium, suggesting that it is potentially noxious to human nasal mucosa.

Animals↗

[Olfactory acuity and improvement of olfaction after total laryngectomy].

OBJECTIVE: To test and evaluate the olfactory function of patient after total laryngectomy, seek to a practical method to ameliorate olfactory function and rise the qualitative character of these patients. METHODS: Using the T&T olfactory examination to evaluate the olfactory function of 60 cases. Four cases olfactory mucosae were observed by electron microscope. Observing relation among the remains olfaction, the time after operation and whether or not undergone the voice reconstruction. And analyse the reasons of the above hyposomnia. Using the closing-mouth and nasal out-word airflow maneuver (CNOAM) as the intervention in the patients of tracheoesophageal fistula voice reconstruction after total laryngectomy to observe the amelioration after intervention. RESULTS: It shows various degree of hyposmia and anosmia in the cases after total laryngectomy with or without tracheoesophageal fistula voice reconstruction, with significant deference (P < 0.01) compared to the normal person respectively. There are precisely correlation among the time after operation and whether or not undergone the voice reconstruction. The longer time leads to less remaining olfaction. The patients after total laryngectomy without tracheoesophageal fistula voice reconstruction have lost their olfaction thoroughly within 5 years. But for the patients after total laryngectomy with tracheoesophageal fistula voice reconstruction, they have a middle hyposmia within 5 years, with significant deference (P < 0.01) between the patients in 5 years and after 5 years. There were significant differences (P < 0.01) between the values of patients with and without tracheoesophageal fistula voice reconstruction. The ultrastructure of 4 cases of olfactory epithelium shows the apoptosis change. After the treatment of CNOAM, the remaining olfaction of most patients were improved, with significant deference (P < 0.01) compared to those before the treatment of CNOAM. CONCLUSIONS: The proceed hypofunction of olfaction may be influenced by the reform of respiratory air, the extinction of air velocity bypass the nasal cavity and the apoptosis of epithelial cells in the patients after total laryngectomy. But if we give an early intervention study such as tracheoesophageal fistula voice reconstruction and CNOAM, the olfactory function may be maintenance. During the intervention, the ending of olfactory nerves may be get uninterrupt stimulation. This may help the patients keep a better existing quality than those fail to accept the interventions.

Adult↗

[Olfactory neuroblastoma in an old patient. Report of a case].

Olfactory neuroblastoma or esthesioneuroblastoma is a rare embrionary tumor, much more in old patients, which uses to present as a pollipoid mass with nasal obstruction, epistaxis and anosmia of long evolution. We report the case of a 82-year-old male with such symptoms diagnosed by biopsy as neuroblastoma class III according the histologic Hyams grading. Due to the age of the patient and grade of the tumor we did not perform surgery and, although a chemotherapy-radiotherapy treatment was suggested, the patient was not agree with it and he has not been attended or followed in our consulting rooms.

Aged↗

[Progressive facial hemiatrophy].

The authors describe a case of progressive facial hemiatrophy in a woman aged 26 years, coming from the Isfarin region of Tadzhikistan. The patient views herself as being ill for 14 years, since the moment of an epileptic attack with tonic and clonic convulsions. Approximately at the same time she noted a small dry ulcer on the left on the vertex. The ulcer slowly increased, followed by skin atrophy. The disease progressed for 4 to 5 years. At present to the left there are folds in the form of scars on the face. The skin is thinned, united with the bones in the frontal and parietal areas, the subcutaneous fat is atrophic. The lips and nose at the left are subatrophic. Negligible enophthalmos, hemiatrophy of the tongue at the left. Alopecia. A certain deterioration of memory and reduction of the critical attitude are recorded. The patient is in a state of euphoria. Left-sided anosmia. The left auricular floor is subatrophic, hearing is almost lacking. Diffuse elevation of the tendinous reflexes of the limbs on the left side. X-ray signs of osteoporosis of the bones of the cranial vault on the left.

Adult↗

Epidemiological aspects of olfactory dysfunction.

PURPOSE: This study aimed at assessing the most common aetiological factors causing total or partial olfactory deficit and the statistical analysis of some clinical aspects. MATERIALS AND METHODS: 243 patients reporting alfactory dysfunction were enrolled in this STUDY: A case history was drawn up for each patient, and all of them underwent otolaryngology objective examination, including nasal endoscopy, paranasal sinuses CT-scan, and Utrecht method (GITU) based smell indentification test. RESULTS: Upper respiratory viral infections (40.2%), ceanial-facial traumas (39.3%), and rhinosinusal pathologies (6.3%) were the main aetiopathological factors indentified. A relevant number of case were recorded with unknown aetiology (14.2%). In 2 cases, olfactory dysfunction was due to neurological diseases, in 1 case it was due to intoxication and, in another case, it was congenital. The correlation between aetiology, gender, age, symptoms duration and deficit severity was studied. CONCLUSIONS: many different aetiological factors caused the loss or weakening of the sense of smell. They mainly affected olfactory neurosensorial structures and odorant conduction. However, there were many cases of unknown aetiology. Women, over 40 in particular, were the most affected. Anosmia and severe hyposmia were mainly correlated with traumas and viral pathologies.

Adolescent↗

[Advances in the studies of Kallmann syndrome].

Kallmann syndrome (KS) is a rare hereditary disease. It is characterized by hypogonadotrophic hypogonadism in association with anosmia or hyposmia. At present, three modes of inheritance and genes related to KS have been identified. This review focuses on the clinical diagnosis and advances in the studies of the pathogenesis gene for Kallmann syndrome.

Diagnosis, Differential↗

Immunohistochemical examination of olfactory mucosa in patients with olfactory disturbance.

The olfactory mucosa was examined by immunohistochemistry in patients with olfactory disturbance: anosmia due to choanal atresia and chronic sinusitis, early-stage common cold, late-stage common cold, and head trauma. The results indicate that the olfactory mucosa of patients with olfactory disturbance shows specific kinds of immunoreactive patterns and that immunohistochemistry is useful for examining the degree of degeneration of pathologic human olfactory mucosa and for clarification of prognosis.

Choanal Atresia↗

[A case of Kallmann syndrome: usefulness of the aging male's symptoms rating scale for assessment of subjective symptoms].

We report a case of Kallmann syndrome in which the aging male's symptoms rating scale (AMS) was useful for assessment of subjective symptoms. A 30-year-old male was admitted to Tsukuba University Hospital with a complaint of delayed puberty in May 2003. He presented with hypogonadism, gynecomastia and anosmia. The plasma levels of luteinizing hormone (LH), follicle stimulating hormone (FSH) and testosterone were very low. However, the LH-releasing hormone test and human chorionic gonadotropin (hCG) loading test identified normal function of pituitary and Leydig cell. After 12 months of treatment with hCG and human menopausal gonadotropin (hMG), the amount of pubic hair and the volume of testes had increased as well as the level of serum testosterone. The total score of AMS after treatment has been improved from 39 to 20 as compared with that before treatment.

Adult↗

Nonallergic rhinitis with eosinophilia syndrome and related disorders.

NARES is a clinical syndrome consisting of symptoms consistent with AR in which an absence of atopy has been demonstrated by allergen skin testing, with nasal cytology analysis showing greater than 20% eosinophils. Anosmia is a prominent feature not shared with AR. The pathophysiology of NARES is poorly understood, but a key component involves a self-perpetuating, chronic eosinophilic nasal inflammation with development of nasal micropolyposis and polyposis. Mast cells likely play an important role as well. NARES is a risk factor for the development of nasal polyposis and aspirin sensitivity, as well as obstructive sleep apnea. Treatment consists mainly of intranasal corticosteroids with or without the addition of second-generation antihistamines and/or LT receptor antagonists as an adjuvant.

Eosinophilia↗