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Essentials of Diagnosis
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- High index of suspicion in individuals from endemic regions.
- Extensive nasal polyposis adherent to nasal septum, with minimal sinus involvement.
- Cultures with Klebsiella pneumoniae rhinoscleromatis (typically not normal nasal flora).
- Nonenhancing, well-defined borders on CT scans; bone and cartilage rarely involved.
- Biopsies of actively involved areas (septum or inferior turbinates) diagnostic.
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General Considerations
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Rhinoscleroma is a rare, chronic progressive granulomatous disease of the upper respiratory tract caused by Klebsiella rhinoscleromatis. Nasal disease presents with three typical stages: (1) catarrhal (atrophic), with nonspecific rhinitis; (2) proliferative (granulomatous), characterized by granulomatous reaction and the presence of Mikulicz cells; and (3) cicatricial (sclerotic), in which mucosal fibrosis is seen. The rise in the incidence of rhinoscleroma in the United States may be due to the increased number of immigrants from endemic regions such as Eastern and Central Europe, Central and South America, East Africa, and the Indian subcontinent. Rhinoscleroma may be found in all age groups, but most frequently affects adolescents and young adults. Poor hygiene, crowded living conditions, and poor nutrition contribute to its spread via airborne transmission.
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The chronicity of this disease is believed to be a result of the ability of the bacteria to evade the host defenses during the proliferative stage. During the catarrhal phase, the organism gains access to the subepithelial layer via ulcerations that allow deep colonization. The bacteria then spread to other areas through the subepithelium and are phagocytosed by histiocytes, forming Mikulicz cells. The organism continues to multiply intracellularly until the Mikulicz cells rupture and deliver viable bacteria interstitially. This cycle continues and eventually leads to clinically evident granuloma formation and pseudoepitheliomatous hyperplasia.
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Rhinoscleroma manifests primarily in the nose, but it can affect any part of the upper respiratory tract, including the eustachian tube, maxillary antrum, oral cavity, larynx, orbit, trachea, and bronchi. In advanced disease, nasal obstruction (94%), nasal deformity (32%), epistaxis (11%), and crusting (94%) are the main symptoms. Laryngeal involvement may present as hoarseness with associated findings of interarytenoid hyperemia, exudates, and vocal cord edema. Late laryngeal fibrosis typically involves the glottis and subglottis, with subsequent stridor and potential airway obstruction.
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A combination of conservative surgical debridement and long-term antibiotic coverage is the mainstay of therapy for rhinoscleroma. Tetracycline has been shown to be effective and inexpensive for patients unless contraindicated. Fluoroquinolones may be used as an alternative, given their excellent gram-negative activity, intracellular efficacy, and low toxicity. The organism is often difficult to eradicate, and relapse can occur even with aggressive therapy due to the organism's ability to remain dormant in its spore form.
Ammar ME, Rosen A. Rhinoscleroma mimicking nasal polyposis.
Ann Otol Rhinol Laryngol 2001;110(3):290–292
[PubMed: 11269777]
. (Clinical findings and management of rhinoscleroma.)
Canalis RF, Zamboni L. An interpretation of the structural changes responsible for the chronicity of rhinoscleroma.
Laryngoscope 2001;111(6):1020–1026
[PubMed: 11404614]
. (Review of the pathophysiology of rhinoscleroma.)
de Pontual L, Ovetchkine P, Rodriguez D, et al. Rhinoscleroma: a French national retrospective study of epidemiological and clinical features.
Clin Infect Dis 2008;47(11):1396–1402
[PubMed: 18947330]
. (Review of the clinical course of a series of patients with rhinoscleroma.)
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Essentials of Diagnosis
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- High index of suspicion in individuals from endemic regions.
- Nasal obstruction and epistaxis, frequent ocular involvement
- Friable, polypoid, vascular nasal mass with red “strawberry” appearance.
- Histopathology showing thick-walled sporangium filled with endospores, pseudoepitheliomatous hyperplasia.
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General Considerations
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Rhinosporidiosis is a chronic granulomatous inflammatory disease caused by Rhinosporidium seebri. It is endemic in India and Sri Lanka and has been reported sporadically in multiple other locations, including South America and Africa. The organism is found in stagnant water and soil and is thought to be spread by contaminated water and inoculation of spores into traumatized epithelium.
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The disease follows an indolent course and typically involves the nose and nasopharynx (70–85%) and the eye, particularly the conjunctiva or lacrimal sac (15%), but it can also involve other sites, including the skin, paranasal sinuses, palate, tonsil, larynx, tracheobronchial tree, parotid gland, and genitalia. The rare disseminated form is fatal and involves skin, bone, and brain.
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Rhinosporidium seebri is difficult to isolate in cultures and some aspects of its epidemiology and life cycle remain controversial. After a period of growth, the early spore begins a series of mitotic divisions. With each division the number of nuclei and spores increases, and the spore wall thickens. The mature sporangium discharges its contents, releasing copious spores into the nasal secretions. Staining with hematoxylin–eosin stain reveals pseudoepitheliomatous hyperplasia, thick-walled fungal sporangia containing numerous endospores, and fibrous stroma with chronic inflammatory cells. The organism is present in all stages of development.
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Patients typically present with symptoms of progressive nasal obstruction, chronic epistaxis, and watery rhinorrhea that becomes purulent during infection. Clinical exam reveals a polypoid, friable nasal mass with a red surface that bleeds easily due to its underlying vascularity. The mass grows slowly and is usually painless. The surface of the lesions contains pin-sized yellow spots bulging through attenuated epithelium that represent the mature sporangia and give the mass its classic “strawberry” appearance.
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Treatment is surgical excision with cauterization of the base. Antifungals, steroids, dapsone, and radiotherapy have been used with limited efficacy.
Capoor MR, Khanna G, Rajni, et al. Rhinosporidiosis in Delhi, north India: case series from a non-endemic area and mini-review.
Mycopathologia. 2009;168(2):89–94
[PubMed: 19347603]
. (Clinical course of patients with rhinosporidiosis in a non-endemic area and brief review of disease forms.)
van der Coer JM, Marres HA, Wielinga EW, Wong-Alcalá LS. Rhinosporidiosis in Europe.
J Laryngol Otol 1992;106(5): 440–443
[PubMed: 1613374]
. (Review of epidemiologic features of rhinosporidiosis and summary of reported cases in Europe.)
Loh KS, Chong SM, Pang YT, Soh K. Rhinosporidiosis: differential diagnosis of a large nasal mass.
Otolaryngol Head Neck Surg 2001;124(1):121–122
[PubMed: 11228469]
. (Case presentation and review of clinical features of rhinosporidiosis.)
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Human Immundeficiency Virus (HIV)
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Essentials of Diagnosis
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- HIV serology is diagnostic.
- CD4 cell counts and viral titers are indicative; lower CD4 counts and higher viral titers correlate with symptomatic immunodeficiency.
- Opportunistic pathogens can be seen with CD4 <50 cells/mm3. Empirically treat for Pseudomonas aeruginosa in HIV patients with sinusitis when CD4 <200 cells/mm3.
- Endoscopic cultures should be used to guide antibiotic coverage.
- Biopsy nasal masses and suspicious skin lesions to rule out malignant neoplasms.
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General Considerations
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Rhinosinusitis may affect up to 68% of patients infected with the human immunodeficiency virus (HIV), with an incidence and severity that correlate with the stage of HIV infection. As immune function deteriorates, the incidence of opportunistic infections increases, especially with CD4 counts below 50 cells/mm3.
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HIV infection results in a gradual depression of humoral and cellular immunity, primarily due to the depletion of helper T lymphocytes. The result is an increased susceptibility to infection. With respect to sinonasal disease, HIV-infected patients have been found to have increased mucociliary transport time, resulting in stasis and thick, tenacious nasal secretions that increase the risk of sinonasal infection. Some studies have suggested that polyclonal B-cell activation with increased immunoglobulin production may result in increased atopy in HIV-positive patients and thus a higher incidence of allergic symptoms. However, the relationship between HIV infection and increased atopy is unclear at this time.
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The typical presentation of rhinosinusitis in these patients is no different from that in seronegative patients; common findings consist of fever, facial pain or pressure, headache, postnasal drip, purulent nasal discharge, periorbital swelling, and nasal congestion. As the HIV infection progresses, the inflammatory response is reduced, resulting in less mucosal edema and rhinorrhea. The microbiology is usually the same as in seronegative patients when the CD4 count is > 50 cells/mm3, with Streptococcus pneumoniae, Haemophilusinfluenzae, and Moraxella catarrhalis being common for acute infection and Staphylococcus aureus,Pseudomonas aeruginosa, and anaerobes being common for chronic infection. When the CD4 count falls below 50 cells/mm3, the risk of infection by opportunistic bacterial, fungal, protozoal, and viral organisms increases. Although extrasinus complications of sinusitis are not known to have a greater incidence in these patients, a high index of suspicion is required with progressive immunodeficiency. These patients are also at risk for life-threatening invasive fungal sinusitis, particularly if the absolute neutrophil count is less than 600/mm3. Skin lesions such as Kaposi's sarcoma, herpetic ulcerations, and seborrhea-like dermatitis are common cutaneous processes that affect the nose and surrounding facial skin. These lesions may herald progression from asymptomatic HIV infection to AIDS. Nasopharyngeal lymphoid hypertrophy affects 56–88% of patients early in the disease course, causing nasal obstruction and serous otitis media, and may warrant biopsy to rule out lymphoma.
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The level of immunodeficiency should guide initial antibiotic therapy, and endoscopically obtained cultures should be performed to further tailor therapy. When the CD4 count is greater than 200 cells/mm3, therapy should include coverage for Streptococcus, Staphylococcus, and Haemophilus influenzae. First-line choices include amoxicillin, amoxicillin/clavulanate, cefuroxime, trimethoprim/sulfamethoxazole, or a macrolide. With incomplete response to initial antibiotic therapy, the development of chronicity, or a CD4 count below 200 cells/mm3, coverage should expand to include Pseudomonas aeruginosa and anaerobes. Clindamycin and metronidazole may improve response in these cases. Antibiotic therapy should be continued for a minimum of 3 weeks, along with systemic decongestants, mucolytics, and nasal saline irrigation. In chronic disease, topical nasal steroids may reduce inflammation and rhinorrhea. Prophylactic treatment with trimethoprim/sulfamethoxazole has been shown to decrease the risk of sinusitis and otitis media. When medical measures fail or in the case of extrasinus complications, functional endoscopic sinus surgery has been shown to be safe and effective. A low CD4 count is not a contraindication for surgical management.
De Vincentiis GC, Sitzia E, Bottero S, Giuzio L, Simonetti A, Rossi P. Otolaryngologic manifestations of pediatric immunodeficiency.
Int J Pediatr Otorhinolaryngol 2009;73 Suppl 1:S42–S48
[PubMed: 20114155]
. (Review of head and neck manifestations of immunodeficiency, including HIV, in the pediatric population.)
Friedman M, Landberg R, Tanyeri H, et al. Endoscopic sinus surgery in patients infected with HIV.
Laryngoscope 2000;110(10 Pt 1):1613–1616
[PubMed: 11037812]
. (Indications and outcomes of functional endonasal sinus surgery in patients with HIV.)
Gurney TA, Murr AH. Otolaryngologic manifestations of human immunodeficiency virus infection.
Otolaryngol Clin North Am 2003;36(4):607–624
[PubMed: 14567056]
. (Comprehensive review of the head and neck manifestations of HIV.)
Prasad HK, Bhojwani KM, Shenoy V, Prasad SC. HIV manifestations in otolaryngology.
Am J Otolaryngol 2006;27(3):179–185
[PubMed: 16647982]
. (Description of otolaryngologic findings in a large series of patients with HIV.)
Shah AR, Hairston JA, Tami TA. Sinusitis in HIV: microbiology and therapy.
Curr Infect Dis Rep. 2005;7(3):165–169
[PubMed: 15847717]
. (Review of findings and medical and surgical treatment recommendations.)
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We would like to acknowledge Ashish R. Shah, MD, John M. Ryzenman, MD, and Thomas A. Tami, MD for their contribution to this chapter in the previous editions of CDT.