What Is Rhinoscleroma?
Rhinoscleroma is a chronic granulomatous disease caused by a bacteria known as Klebsiella rhinoscleromatis. It is a gram-negative rod-like shaped bacteria. This disease usually appears in the nasal mucosa but can progress to any part of the upper respiratory tract.
What Is the Historical Background of Rhinoscleroma?
Hebra first described rhinoscleroma in 1870. Polish surgeon Johann von Mikulich described the histologic features of rhinoscleroma in 1877. In 1882 von Frisch identified the causative organism. In 1961, Steffen and Smith proposed that Klebsiella rhinoscleromatis is the main etiologic factor responsible for rhinoscleroma.
What Is the Epidemiology of Rhinoscleroma?
This disease is more prevalent in regions of Africa, Southeast Asia, Mexico, Central and South America, and Eastern Europe countries. However, sporadic cases of rhinoscleroma have also been reported worldwide. Five percent of all cases are found in Africa. In recent years, this disease has been rising in America, usually in the immigrant population.
This disease can affect a person of any race and is found in more females than males. Generally affecting people of age 10 to 30 years.
What Are the Clinical Features of Rhinoscleroma?
The nasal cavity is the most commonly affected area in 95 to 100 percent of the cases, followed by the nasopharynx (18 to 43 percent), larynx (15 to 40 percent), trachea (12 percent) and bronchi (two to seven percent).
The disease affects mostly middle-aged women living in poor hygienic conditions. Human beings are the only host for Klebsiella rhinoscleromatis. The bacteria travel via nasal droplets or contaminated material.
There are three stages of rhinoscleroma -
1. Catarrhal Phase - This is the initial phase in which the patient has nonspecific rhinitis (irritation and swelling of the mucus membrane of the nose) with foul-smelling discharge, epistaxis, and nasal obstruction. This stage can last for weeks or months.
2. Granulomatous Phase (Proliferative or Nodular) - The clinical features are more prominent in the granulomatous phase, which involves a granulomatous nodular mass that causes nasal obstruction. The mucosal tissue inside the nose becomes bluish-red, and the symptoms include bleeding noses, nasal obstruction, a hoarse voice, and thickening or numbing of the soft palate.
3. Sclerotic Phase (Cicatricial or Fibrotic) - If this condition is left untreated, it leads to a sclerotic phase. In this phase, permanent deformities can be seen, which include nasal deformities, anosmia (loss of smell), dysphonia (a disorder of voice), dysphasia (language disorder), oral anesthesia, and stridor (high-pitched whistling sound while breathing).
The initial nodule is small in size and intra-nasal, and it can also grow into an exophytic giant tumor, which can even obstruct the entire respiratory tract. In rare cases, it may also extrude into the oral cavity, the skull base, the orbit, and the brain.
How to Diagnose Rhinoscleroma?
I. Cytologic Methods -
The diagnosis of rhinoscleroma can be made by cytologic methods as they are easy to perform and do not cause any pain. In these tests, small amounts of body fluids can be taken and evaluated in the lab to find the causative agent. For this condition, the specimen or sample is directly taken from the lesion and assessed under the microscope.
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Chronic upper respiratory tract infections can be diagnosed by tissue biopsy of the lesion.
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Nasal endoscopy can be done, which helps identify all three stages of rhinoscleroma.
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Bronchoscopy (a procedure to look at lungs or air passages) can be done in the initial stage for early diagnosis of rhinoscleroma.
The positive finding of this disease can be made by culturing the sample with MacConkey agar and evaluating it under the microscope. However, it is only helpful in 50 to 60 percent of the cases.
II. Radiographic Features -
1. Computed Tomography -
The characteristic radiological feature of rhinoscleroma can be easily seen in computed tomography (CT) scans, which is the presence of nasal mass.
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Computed tomography for primary nasal and nasopharyngeal rhinoscleroma soft-tissue masses of variable sizes can be seen.
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Lesions are non-enhancing and homogeneous and have distinct borders.
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The lesions do not invade the surrounding tissues.
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The lesions may be seen, causing irregular narrowing of the airway.
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In the trachea, crypt-like irregularities can be seen.
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Thickening of walls and nodules can also be seen.
2. MRI (Magnetic Resonance Imaging) - In MRI, nasal masses can be visualized, obstructing the nasal cavity, and secretions may get retained in the various sinuses.
What Is the Treatment of Rhinoscleroma?
The treatment for rhinoscleroma includes long-term antibiotic therapy and surgical intervention in patients with obstruction in the nasal cavity.
Long-term Antibiotic Therapy -
The choice of long-term antibiotic therapy should be planned based on the age and sex of the patient. Antibiotics such as Cephalosporins and Clindamycin can be given in cases with bacterial infections, and Ciprofloxacin can be prescribed for sclerotic lesions.
Surgical Procedures -
Surgery combined with antibiotic therapy is the best treatment in cases with granulomatous disease, nasal or pharyngeal obstruction, or nasal sinus involvement.
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Tracheotomyis a surgical airway management procedure in which the surgeon makes a hole in the front of the neck to the trachea. This is beneficial in cases with laryngeal obstruction in the granulomatous and sclerotic stages.
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Plastic surgery can be done in patients with perforations in the nasal cavity, pharynx, larynx, or trachea.
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Granulomatous lesions can be effectively treated by open excision surgery.
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Laser therapy or surgery can treat airway obstruction and tissue deformities.
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Rhinoplasty (surgery to change the shape of the nose) can be done to treat external nasal deformities.
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Palatal symptoms can be corrected by uvulopalatopharyngoplasty (a surgical procedure to open the upper airways).
What Is the Differential Diagnosis of Rhinoscleroma?
The differential diagnosis of other forms of nasal masses includes granulomatous and neoplastic disease that may resemble rhinoscleroma.
Granulomatous diseases include -
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Bacterial infections such as tuberculosis, leprosy, and syphilis.
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Fungal infections such as histoplasmosis and sporotrichosis.
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Parasitic infections such as mucocutaneous leishmaniasis
Neoplasms such as lymphoma.
The various clinical findings that help differentiate rhinoscleroma from other nasal malignancies include-
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Expansion of the nasal cavities.
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Scalloping of the sinus wall.
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Lack of bone destruction.
Conclusion
Rhinoscleroma is an uncommon condition that usually depends upon the hygiene of the patient. Moreover, this is rarely lethal unless it causes any airway obstruction. It can be treated easily with antibiotic therapy but can go for long-term, and surgery can also be performed in case of any airway compromise. The recurrence rate of this condition is also high if precautions are not taken after the treatment. Patients should immediately consult a doctor if symptoms are found, as, with delay, the rate of severity can also increase.
