[Widespread of Scleroma. A rare clinical case]. / Skleroma, rasprostranennaya forma. Redkoe klinicheskoe nablyudenie.

Scleroma is a chronic infectious disease characterized by developing inflammation nye granulomas, mainly of the upper respiratory tract, with subsequent scarring. The reasons for the development of scleroma remain unclear, but it is generally accepted that the gram-negative coccal bacterium Klebsiella rhinoscleromatis (pas-Volkovich's loch - Frisch) is the causative agent of this disease. Endemic in the spread of scleroma is considered There are tropical and temperate zones such as Africa, Asia, Eastern Europe, South America and Central America. Features of development scleroma in the present case and the complexity of the diagnosis caused by atypical, the rapid development of the disease, the greater prevalence of the process (including the orbit), with the transition to the soft tissues of the cheek bone and upper jaw, which is not typical for scleroma. The process was primarily localized in the left maxillary sinus and mimicked sinusitis. The presence of radicular cysts and darkening of the left maxillary sinus on radiographs, taking into account the clinical picture of the disease, were interpreted incorrectly, which led to the establishment of an incorrect diagnosis and subsequent surgical interventions. More targeted and consistent examination of histo-the logical material obtained after three surgical interventions made it possible to identify the characteristic signs scleroma, establish the correct diagnosis and prescribe effective treatment. In our case, we applied Ciprofloxacin course for 3 months at a maximum daily dose of 2000 mg in combination with probiotics. During treatment, it was noted a significant improvement in the patient's well-being, facial pains became less intense, a decrease in the volume of ma tissues of the left cheek and improved nasal breathing.

Patología infecciosa del vestíbulo nasal / Infectious diseases of the nasal vestibule

El vestíbulo nasal corresponde a la primera porción de la fosa nasal, éste se encuentra delimitado lateralmente por los cartílagos alares y medialmente por el borde caudal del septum nasal y la columela. Las enfermedades infecciosas del vestíbulo nasal son patologías frecuentes en la práctica clínica; su diagnóstico se realiza en base a sospecha clínica y examen físico, requiriendo habitualmente solo manejo médico ambulatorio. Desde el punto de vista etiológico pueden ser virales, bacterianas y fúngicas. Las principales especies bacterianas involucradas corresponden a Staphylococcus coagulasa negativa, S. epidermidis, S. hominis y S. haemolyticus, difteroides spp y S. aureus. Su manejo es esencialmente médico con casos excepcionales requiriendo manejo quirúrgico. En la actualidad existe escasa información epidemiológica al respecto, lo que dificultad la clasificación de los dichos cuadros clínicos. Se realizó una revisión de la literatura sobre cuadros infecciosos que afectan el vestíbulo nasal para lograr sistematizar y clarificar las distintas patologías y sus tratamientos.

The nasal vestibule corresponds to the first portion of the nasal passage, limited laterally by the lateral crus and medially by the caudal edge of the nasal septum and columella. Infectious diseases of the nasal vestibule are frequent in clinical practice, diagnosis is made based on clinical suspicion and physical examination, usually requiring only ambulatory medical management. In terms of etiology, they can be viral, bacterial and fungal. The main bacterial species involved correspond: Coagulase-negative Staphylococcus, S. epidermidis, S. hominis and S. haemolyticus, difteroides spp and S. aureus. Management is essentially medical and only exceptionally requires surgery. Currently, there is a lack of epidemiological information in this regard, which makes it difficult to classify these clinical conditions. A review of the literature on infectious conditions that affect the nasal vestibule was performed, to systematize and clarify the different pathologies and their management.

Airway scleromas and their extensions.

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.

[Rhinoscleroma disease accompanied with laryngotracheal stenosis: a case report].

Rhinoscleroma disease accompanied with laryngotracheal stenosis as a main clinical manifestation is seldom. Here one case is reported. At the beginning, the patient had pharyngalgia and his swallowing was not smooth. After that, he had difficulty in breathing. Surgical therapy, antibiotics treatment and nutritional support were performed. The patient was cured.

Rhinoscleroma: a case series report and review of the literature.

Rhinoscleroma is a rare chronic granulomatous infectious disease and important differential diagnosis in developing countries and emigrants from these regions. In this survey we present seven cases from University Hospital Kigali, Rwanda, and one more from Germany. All cases are discussed on the background of the current literature.

Surgical management of scleromatous laryngotracheal stenosis.

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.

Role of MR imaging in laryngoscleroma.

To evaluate the role of MR imaging in patients with laryngoscleroma. We retrospectively reviewed the MR imaging of 14 patients (11 female, 3 male with mean age of 31 years) with pathologically proven laryngoscleroma. They presented with dysphonia (n = 12), stridor (n = 8) and airway obstruction (n = 4). They underwent T1- and T2-weighted MR images and post contrast study after injection of 0.1 mmol Gd/DTPA. Laryngoscleroma was seen in the subglottic (n = 13) and supraglottic (n = 1) regions. Laryngoscleroma at granulomatous stage (n = 6) appeared as diffuse circumferential soft tissue mass with high (n = 4) or mixed (n = 2) signal intensity on T2-weighted images with homogenous (n = 4) and inhomogeneous (n = 2) pattern of contrast enhancement. At fibrotic stage (n = 8), laryngoscleroma was seen as diffuse asymmetrical circumferential thickening of the subglottic region with low signal intensity on T2-weighted images and mild contrast enhancement. Subglottic lesions encircled the subglottic region with marked (n = 5) and mild (n = 9) narrowing of the airway with variable degree of extension into the trachea in three patients. There was diffuse thickening of the epiglottis, aryepiglottic folds in one patient with supraglottic scleroma. MR imaging is a non-invasive imaging modality for accurate localization, extension and staging of laryngoscleroma. These data is important for treatment planning.

Coexistence of rhinoscleroma with Rosai-Dorfman disease: is rhinoscleroma a cause of this disease?

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.

Rhinoscleroma in an immigrant from Egypt: a case report.

Rhinoscleroma is a chronic indolent granulomatous infection of the nose and the upper respiratory tract caused by Klebsiella rhinoscleromatis; this condition is endemic to many regions of the world including North Africa. We present a case of rhinoscleroma in a 51-year-old Egyptian immigrant with 1-month history of epistaxis. We would postulate that with increased travel from areas where rhinoscleroma is endemic to other non-endemic areas, diagnosis of this condition will become more common.

Rhinoscleroma causing severe bilateral nasal obstruction.

Rhinoscleroma is a chronic, infectious and granulomatous disease of the respiratory tract. There is often a delay in diagnosis due to unfamiliarity with the disease and also because culture is not always positive. We report a case in a 26-year-old woman with granular mass obstructing bilateral nasal cavities and causing breathing difficulty. Histopathological examination showed characteristic Mikulicz histiocytes containing numerous Gram-negative intracellular rod-shaped bacilli consistent with the diagnosis of rhinoscleroma. The patient was treated with gemifloxacin and tetracycline and remains asymptomatic over a year follow-up period. It is important to consider rhinoscleroma in cases of chronic nasal obstruction. As culture is not always positive, histopathological examination may be crucial to the diagnosis.

Estenosis subglótica como manifestación tardía de un rinoescleroma. Presentación de un caso / Laryngeal stenosis as late manifestation of rhinoscleroma. Case report

El rinoescleroma es una entidad crónica, progresiva y de aspecto granulomatoso, resultado de la infección por Klebsiella rinoescleromatis. Es en general infrecuente, pero endémica en ciertas regiones del mundo. Afecta típicamente a la cavidad nasal, pero también a otras partes del sistema respiratorio, como la región laringotraqueal, cuyo compromiso puede representar un riesgo para la vida. Presentamos el caso de un paciente de sexo masculino de 46 años de edad, con el antecedente de rinoescleroma diagnosticado y tratado hace 15 años, que presenta estenosis subglótica como secuela tardía de esta enfermedad (AU)

Rhinoscleroma is a chronic, progressive, granulomatous disease resulting from infection with Klebsiella rhinoscleromatis. It is generally uncommon, but endemic in certain regions of the world. It typically affects the nasal cavity, but also other parts of the respiratory system, such as the laryngotracheal region, which may be life-threatening if compromised. We present a 46-year-old male patient with a history of rhinoscleroma diagnosed and treated 15 years ago, with subglottal stenosis as a late sequela of this disease (AU)

Rhinoscleroma causing severe bilateral nasal obstruction

Rhinoscleroma is a chronic, infectious and granulomatous disease of the respiratory tract. There is often a delay in diagnosis due to unfamiliarity with the disease and also because culture is not always positive. We report a case in a 26-year-old woman with granular mass obstructing bilateral nasal cavities and causing breathing difficulty. Histopathological examination showed characteristic Mikulicz histiocytes containing numerous Gram-negative intracellular rod-shaped bacilli consistent with the diagnosis of rhinoscleroma. The patient was treated with gemifloxacin and tetracycline and remains asymptomatic over a year follow-up period. It is important to consider rhinoscleroma in cases of chronic nasal obstruction. As culture is not always positive, histopathological examination may be crucial to the diagnosis.

[Laryngeal stenosis as late manifestation of rhinoscleroma. Case report]. / Estenosis subglótica como manifestación tardía de un rinoescleroma. Presentación de un caso.

Rhinoscleroma is a chronic, progressive, granulomatous disease resulting from infection with Klebsiella rhinoscleromatis. It is generally uncommon, but endemic in certain regions of the world. It typically affects the nasal cavity, but also other parts of the respiratory system, such as the laryngotracheal region, which may be life-threatening if compromised. We present a 46-year-old male patient with a history of rhinoscleroma diagnosed and treated 15 years ago, with subglottal stenosis as a late sequela of this disease.

[Endonasal surgery of choanal atresia].

This paper was designed to study clinical manifestations of choanal atresia (CA) in 11 patients with a view to optimizing methods for medical and diagnostic management of this severe pathology using endonasal surgical techniques. Detailed analysis of possible causes of congenital CA and mechanisms of acquired CA was performed using computed tomography (CT) and retrograde endoscopy with the help of rigid optics. Potential of certain instruments designed and manufactured in Russia for the management of CA is demonstrated, such as retrograde 110o Sekunda endoscope and silicone horseshoe-shaped protectors. An original diagnostic algorithm is proposed allowing for the development of indications for surgical intervention, determination of its extent, and the choice of the post-operative treatment. Improved surgical instruments have been designed for the purpose. A case of hereditary CA in a child and his mother are presented along with results of treatment of nasopharyngeal scleroma in two patients. Clinical manifestations of CA concomitant with scleroma, diphtheria, and chemical chaonal burns are considered.

Grave afectación laringotraqueal secundaria a rinoescleroma por Klebsiella pneumoniae ssp. ozaenae / No disponible

No disponible

[Rare concurrence of airway scleroma and nonspecific aortoarteritis (Takayasu's disease)].

A case of a rare concomitance of airway scleroma and nonspecific aortoarteritis was studied in a 14-year-old girl, a native from the scleroma endemic Rovno Region. She had been treated for 4 years for this disease verified by a histological study of a laryngeal biopsy specimen. The fact that scleroma was a predictor of Takayasu's disease in this case cannot be ruled out; suggesting a pathogenetic association between both diseases. Concentric narrowing of the lumen of the aorta and its branching vessels contributed to the development of hypoxia and CNS ischemia, which worsened the severity of a pathological process in the airway and caused a fatal outcome during an episode of asphyxia.