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INTRODUCTION: Rhinoscleroma (RS) is a chronic, progressive, granulomatous infectious disease endemic in Egypt and the Middle East, affecting the nose in 95-100% of cases, and resulting in functional and esthetic sequelae. The aim of the study was to investigate the feasibility of performing reconstructive procedures to improve external deformities in RS patients, with long-term follow up. PATIENTS & METHODS: This prospective, controlled cohort study included 25 patients who were seeking rhinoplasty to improve their esthetic appearance, with evident clinical and histopathological history of RS. Another 25 patients seeking esthetic augmentation rhinoplasty in the same period formed the control group. All the participants were evaluated objectively by two independent rhinoplasty surgeons, and subjectively with ROE 2 years postoperatively. RESULTS: All the participants showed significant improvement in esthetic facial evaluation postoperatively, with a correlative rise in ROE score. There was an insignificant difference between the studied groups. Inconsequential complications were encountered throughout the follow-up period, but with insignificant incidence in both groups. CONCLUSION: Rhinoplasty for correction of RS external deformities is both safe and beneficial, with no risks of flaring up or increased complications. LEVEL OF EVIDENCE: Level 2b.

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CONTEXT.­: Rhinoscleroma is a rare, chronic, infectious granulomatous process involving the upper respiratory tract caused by gram-negative bacilli, Klebsiella rhinoscleromatis. The site most commonly affected is the nasopharynx; however, lesions in various other locations have been described. OBJECTIVE.­: To review the literature for all the reported cases of rhinoscleroma in the past 5 years. DATA SOURCES.­: Published cases of rhinoscleroma from a PubMed (National Center for Biotechnology Information, Bethesda, Maryland) search were reviewed. CONCLUSIONS.­: Rhinoscleroma in nonendemic regions is extremely rare; however, with increased travel, immigration, and globalization, it is imperative to recognize this entity because the symptoms can be devastating and in some cases fatal. Although nasopharynx is the common site of involvement, unusual sites such as the trachea can be involved in rare cases. Rhinoscleroma can be managed effectively with a combination of antibiotics and surgical debridement and repair; however, recurrence rates do remain high.

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BACKGROUND: Scleroma is a rare, chronic, granulomatous infectious disease of the respiratory tract mucosa which begins in the nose and spreads to the respiratory tract and adjoining structures. We report on the extensions and the management of 134 cases of scleroma in the Highlands region of Papua New Guinea. METHODS: The charts and treatment records of 134 scleroma cases were retrospectively reviewed from 1995 to 2013. The staging, extensions, treatment and results of treatment were reviewed and analysed. RESULTS: Of the 134 cases, 72 (53.7%) were females and the age ranged from 6 to 65 years. The disease was confined to the nose and nasopharynx in 71 (53.0%) cases. Extension of the disease from the nose to the Eustachian tube occurred in nine (6.7%) cases and into the middle ear in four (3.0%) cases. Further extensions to the larynx and trachea were seen in 17 (12.7%) cases each and the bronchus in three (2.2%) cases. Primary laryngoscleroma without involvement of the nose and nasopharyngeal stenosis without laryngeal involvement were managed in 11 (8.2%) and nine (6.7%) cases respectively. Others cases treated were ethmoid scleroma with proptosis and scleroma involving the upper lip in one (0.75%) case each. Ninety-nine (74.9%) patients were treated medically while 35 (26.1%) patients required surgery. Cure were achieved in 83 (61.9%) cases treated medically and 26 (19.4%) had successful surgical outcome. CONCLUSION: Scleroma begins in the nose and when not arrested, extends causing obstruction to the airway and cosmetic deformity which requires surgical intervention.

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Rhinoscleroma (RS) is a rare chronic granulomatous disease of the upper airways affecting nasal cavity, nasopharynx, and paranasal sinuses. Klebsiella rhinoscleromatis is the causative agent of this infection and Mikulicz cells are specific to this lesion. RS is commonly seen in poorer regions such as Central Africa, South America, Middle East, India and Indonesia. It is predominantly found in rural areas and people with poor socio-economic conditions. Most patients present with chronic rhinitis, sneezing, headache and deviated nasal septum similar to current case. An association with oral cavity has not been reported previously, as per authors' knowledge. This report describes a rare case of RS of nasal cavity extending into the oral cavity.

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A 39 year-old man came to our institution because of a five-year history of a progressive painful growing mass on his left nostril, which cause airway obstruction with ulceration. Because of a suspicion of malignancy, surgery (mass resection and subtotal nasal reconstruction) was performed. Histologic samples ruled out malignancy, and tissue cultures for fungus and mycobacteria were negative, but regular aerobic cultures were positive for Klebsiella rhinoscleromatis. The patient was given a six-month regimen of ciprofloxacin, and a dramatic improvement was observed.

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OBJECTIVE: Scleroma is a chronic specific granulomatous disease of the upper respiratory tract caused by Klebsiella Rhinoscleromatis. It usually affects the subglottic region and upper trachea resulting in various degrees of stenosis. Patients with laryngotracheal stenosis may present with stridor, shortness of breath or exercise intolerance and may be tracheostomy dependent. In this work, we presented the experience of our Institute in the management of patients with scleromatous laryngotracheal stenosis using the already designed procedures for traumatic laryngotracheal stenosis. PATIENTS AND METHODS: The study was a non controlled prospective study. It was conducted in Oto-Rhino-Laryngology and Head and Neck Surgery Department of Zagazig University Hospitals, Egypt. It included 38 patients with scleromatous subglottic stenosis and/or upper tracheal stenosis. The patients were classified into four grades according to Myer-Cotton's scale. The surgical treatment modalities included endoscopic CO2 laser surgery with dilatation, laryngotracheal reconstruction, and partial cricotracheal resection with thyrotracheal anastomosis. RESULTS: The average follow-up period was 32.1 months. Twenty four patients (63%) had an excellent outcome. Nine patients (24%) had a good outcome. Five patients (13%) were still tracheostomy dependent. Eleven patients (29%) developed postoperative granulation tissue. The overall success rate was 87%. CONCLUSION: Scleromatous laryngotracheal stenosis is considered a challenging surgical problem. It requires a multidisciplinary approach by well-trained personnel. The surgical techniques designed for cases of laryngotracheal stenosis of a traumatic etiology can be applied for cases of scleroma with approximately the same success rates.

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OBJECTIVE: We report an extremely rare case of primary otoscleroma. METHOD: We present a case report and a review of the world literature concerning otoscleroma. RESULTS: An adult woman presented with chronic suppurative otitis media with tubotympanic disease and conductive hearing loss. On mastoid exploration, dark granulations were seen, which were identified as otoscleroma on histopathological examination. The patient responded well to streptomycin. CONCLUSION: To the best of our knowledge, this is the first report of primary otoscleroma in the world literature. This case indicates that Frisch's bacillus can also spread to the middle ear.

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INTRODUCTION: Rhinoscleroma and Rosai-Dorfman disease have been reported to coexist in the same patient at different sites. Rosai-Dorfman disease may have an aetiological relationship with rhinoscleroma, although this has not yet been proved. CASE REPORT: A case of a 42-year-old woman with recurrent nasal masses is presented, with histopathological proof of both conditions coexisting in the same nasal lesion. DISCUSSION: The aetiopathology, clinical features and treatment of both diseases are discussed and a literature survey is reported. Histologically, the presence of Mikulicz cells with entrapped, rod-like, Gram-negative bacilli and Russell bodies suggests rhinoscleroma. Emperipolesis and S-100-positive histiocytes confirm the diagnosis of Rosai-Dorfman disease. The presence of both in the same slides from affected tissues has never been demonstrated before. In the light of this evidence, the author believes that rhinoscleroma must be considered in the aetiology of Rosai-Dorfman disease.

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Rhinoscleroma is a chronic, granulomatous infection that most frequently affects the respiratory mucosa, especially the nasal cavity and eventually extending through the lower respiratory tract. The disease is endemic in some countries of Central America (El Salvador and Guatemala), Indonesia, India, Poland, Hungary, Russia and some African countries as well. It is a rare disease in South America. We report a 51-year-old male resident of a psychiatric institution in São Paulo presenting with progressive nasal obstruction, frontal headache, yellowish nasal discharge and a mass extruding through the right nasal vestibule. The present case report describes a Rhino-Sinus scleroma where histopathology was vital in the diagnosis. The patient was treated by surgical excision of the nasal mass followed by a course of ciprofloxacin. He has remained asymptomatic up to the last visit six months following treatment and has shown no evidence of recurrence.

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Rhinoscleroma is a chronic granulomatous condition of the respiratory tract, and is not uncommon in tropical regions; particularly, Mexico, Central America and the Middle East. A few cases have been reported in North America, primarily involving immigrants from endemic countries. The causative organism is Klebsiella rhinoscleromatis, a Gram-negative coccobacillus. Diagnosis is made on the basis of culture of the organism and the characteristic pathology of Mikulicz cells on light microscopy. The condition primarily affects the upper airway, and frequently presents with nasal discharge, nasal obstruction or frontal facial pain. Despite the term 'rhinoscleroma', there may be involvement of the entire respiratory tract. Although the condition is slowly progressive, its natural course portends extensive destruction. Laryngotracheal involvement occurs in approximately 15% to 80% of cases, but patients rarely present with isolated laryngotracheal disease. In the present paper, a case of rhinoscleroma presenting with symptoms of upper airway obstruction is described.

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Se presenta el primer caso publicado de rinoescleroma en un paciente chileno, con historia de tres años de evolución de obstrucción nasal izquierda que luego se hace bilateral. Es estudiado en el Policlínico de Otorrinolaringología del Hospital Barros Luco Trudeau, pesquisándose una masa en fosas nasales de aspecto polipoideo atípico. Mediante estudio histopatológico e infectológico se confirma el diagnóstico de rinoescleroma, con cultivo positivo para Klebsiella rhinoscleromatis. El paciente es tratado con moxifloxacino durante seis semanas previo a la cirugía endoscópica, efectuándose resección de la patología en ambas fosas nasales. Se trata con antibiótico durante seis semanas más, quedando, a los tres meses postcirugía, con leve obstrucción nasal a izquierda secundaria a una estenosis fibrosa de coana izquierda, residual a su patología.

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Rhinoscleroma is an uncommon chronic, destructive infection of the respiratory mucosa caused by Klebsiella rhinoscleromatis. This coccobacillus can be found in the typical histiocytes, the Mikulicz cells. Extranasal and nodal involvement in this disease is rare, but documented. Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is also a rare, non-hereditary disorder. Bilateral cervical lymphadenopathy with emperipolesis, as the main histological characteristic, is the most common presentation. It can also occur extranodally. We report a case of rhinoscleroma occurring in a 62-year-old woman since 1984, who developed parotid gland and lymph node involvement. The changes in the nasal mucosa and the parotid gland showed chronic inflammation with Mikulicz cells. In the lymph nodes, features characteristic of Rosai-Dorfman disease were seen. Taking into consideration the literature dealing with both of these diseases, we discuss that Rosai-Dorfman disease could be a special type of lymph node reaction and is not necessarily an entity of its own. Therefore, it should be known as Rosai-Dorfman lymph node reaction. Furthermore, there seems to be an interconnection between Rosai-Dorfman disease and rhinoscleroma.

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A case with palatal presentation of scleroma is presented due to its rarity and unusual manifestations. The patient presented with only palatal symptoms. Nasal endoscopy revealed signs of all three stages of scleroma: catarrhal, granulomatous and sclerotic stages. Histopathology was vital in the diagnosis. The patient was treated by medical therapy, and palatal symptoms were relieved by uvulopalatopharyngoplasty.

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Rhinoscleroma is a chronic, granulomatous infection most frequently affecting the respiratory tract mucosa. The disease is endemic in some geographic areas and is sporadic in Italy, where medio-facial granulomatoses must be differentiated from malignant naso-sinus neoplasms. The present work describes a case of rhino-sinus scleroma with extramaxillary extension, defining the most important clinical characteristics and the treatment performed.

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BACKGROUND: Rhinoscleroma is a rare infectious disease of the upper respiratory tract caused by Klebsiella rhinoscleromatis. The nasal mucosa represents the primary region of occurrence which is most likely a result of respiratory transmission. Without adequate++ treatment, the disease can potentially spread to the rest of the upper and middle respiratory tract within a period of several years. Healing often occurs with extensive scarring and adhesions of the nose, palate and larynx. A life threatening late stage manifestation includes subglottic stenosis requiring immediate surgical intervention. Medical treatment primarily consists of a long-term course of antibiotics. Ciprofloxacin (Fluoroquinolon) has proved to be one of the most effective drugs. CASE REPORT: We report about a 33 year-old Egyptian male, who presented in our department with a 10 year history of a previously, only temporarily successfully treated rhinoscleroma. His main symptoms were nasal obstruction, epistaxis and inspiratory stridor. FINDINGS: We began treatment with Ciprofloxacin over a four week period, which lead to an improvement of his symptoms. The treatment was considered complete after several biopsies and smears were negative for live specimens. We then chose to reduce the scar tissue that was causing obstruction in the larynx and the nose. CONCLUSION: The rhinoscleroma is a rare disease in geographic areas with poor hygiene and can mimic several other infectious and malignant diseases. Treatment should include a long-term antimicrobial therapy and surgical intervention in cases with symptomatic obstruction.

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Rhinoscleroma caused by the bacillus Klebsiella rhinoscleromatis and rhinosporidiosis caused by the fungus Rhinosporidium seebri are rare, specific nasal infections, both of which have a certain geographical distribution. To the best of our knowledge no association between them has been reported in the international literature. We have documented such an association in two male Indian patients aged-32 and 27 years, respectively-both presenting with unilateral blood-stained discharge and nasal blockage. They showed strawberry-like polypoidal masses, and histological examination confirmed the diagnosis. Klebsiella rhinoscleromatis was cultured twice in the first case. The patients were treated with complete excision and a long course of septrin, for which Klebsiella rhinoscleromatis is sensitive. The purpose of this paper is to report the first association of these two granulomatous infections, to show the impact of immigration on the differential diagnosis, and to review the relevant literature.

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Nineteen patients with scleroma respiratorium treated surgically were studied retrospectively. The results suggested that the surgical operation combined with antibiotic therapy were beneficial to the curtive effect of the patients in the granulomatous stage with nasal or pharyngeal obstruction or nasal sinuses involvement due to the serious proliferation of the lesions; tracheotomy should be considered in the patients with laryngeal obstruction up to the second degree or above; plastic operations were necessary for the patients with cicatrical stenosis or imperforation remained of the nasal cavity, pharynx, larynx or trachea.

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