Учебники / Rhinosinusitis - A Guide for Diagnosis and Management 2008

Fig. 11.5 Culture results in a patient later diagnosed with immunodeficiency, showing the growth of atypical Mycobacterium in a sinusitis otherwise refractory to traditional antibiotics

these microbes requires that treatment be driven by culture results to avoid development of resistant strains of Mycobacterium[1,32,33] (Fig. 11.5). An infectious disease specialist may be consulted in this scenario, especially in the rare occurrence of tuberculin infection.

Rhinoscleroma

Caused by Klebsiella rhinoscleromatis, rhinoscleroma is a rare chronic infection characterized by the formation of granulomatous nasal masses [34]. Rhinoscleroma patients present in one of three classic stages. The first stage, or the atrophic stage, is evidenced by symptoms and endoscopy findings mimicking rhinosinusitis, such as nasal obstruction, rhinorrhea, purulent discharge, and crusting. This is followed by the hypertrophic stage, where nasal nodules begin to form, eventually leading to the destruction of nasal cartilage. Finally, the sclerotic stage is marked by extensive fibrosis and scarring [1]. Although this condition primarily affects the nose, with nasal symptoms present in 95% to 100% of rhinoscleroma patients, other sites of involvement have been reported including the sinuses, eustachian tubes, mouth, larynx, trachea, bronchi, nearby skin, and even the brain, causing destruction and stenosis in these sites [34]. As a result, voice hoarseness, as well as hearing and vision loss, may result if this condition is not effectively managed [35].

Rhinoscleroma is minimally contagious, transmitted through prolonged exposure, and is more likely to be found in the rural areas of Central Europe, the Middle East, Central and South America, Asia, and tropical Africa [34,35]. It is therefore prudent to ask suspected patients about any recent extended travel in these areas.

Diagnosis is made through a combination of a positive culture as well as histopathological examination of a biopsy of the affected nasal tissue [34]. The classic biopsy findings compatible with rhinoscleroma include Mikulicz cells, macrophages containing large amounts of bacteria-filled vacuoles, and Russell bodies, spherical structures found in the cytoplasm of plasma cells. The culture with sensitivity serves to further support the diagnosis as well as to later direct therapy, although less than 70% of cultures come back positive for the Klebsiella rhinoscleromatis bacterium [1,34].

Treatment includes the use of extended courses of culture-directed antibiotics lasting between 1 and 3 months. Some antibiotics commonly used for rhinoscleroma include flouroquinolones such as ciprofloxacin and levofloxacin, clindamycin, rifampin, and tetracycline [34]. Surgical debridement may also be needed in cases of significant nasal stenosis [35].

Hematologic-Oncologic Illnesses

Neoplasms

Distinguishing neoplasms from other infectious and inflammatory processes causing persistent symptoms of rhinosinusitis is an important role for rhinologists [36]. While the source may be one of the atypical infectious and autoimmune disorders outlined in earlier sections of this chapter—such as syphilis, fungus, and Wegener’s granulomatosis—neoplasms should also be part of the differential diagnosis. Neoplasms are either benign, as is usually the case in inverted papillomas, or malignant. Malignant neoplasms of the nasal cavity and paranasal sinuses can be broadly categorized as epithelial, including squamous cell, transitional cell, adenoid cystic and adenocarcinomas; nonepithelial, including lymphoid tumors, reticular tumors, and sarcomas; or metastatic cancer [37].

Neoplasms of the nasal cavity or paranasal sinuses should be ruled out in a patient with prolonged symptoms of rhinosinusitis recalcitrant to conventional therapy or with persistent unilateral symptoms. The clinical picture of most sinonasal tumors lacks specificity, with symptoms resembling more benign processes, including nasal obstruction, nasal discharge, epistaxis, rhinitis, anosmia, facial pain, and/or neuropathies [36,38]. Physical examination of the nasal cavity may reveal an intranasal mass, septal destruction, mucosal ulceration, necrosis, destruction of sinus walls, or the presence of an oronasal fistula [38] (Fig. 11.6).

As with most malignant processes, early diagnosis is crucial for a favorable prognosis. A biopsy is required for determination of histopathology. An adequate specimen is imperative, which is sometimes difficult to obtain for reasons of nasal anatomy or the possible presence of necrosis. Multiple biopsies may be needed for

mucosal sites, symptoms of congestive heart failure with liver involvement, and seizures with cerebral vascular malformations [40].

Nosebleeds are the most common complaint of these patients and severely affect their quality of life [42]. On nasal endoscopy, multiple telangiectases can be observed (Fig. 11.9). They have been described as superficial dilated vascular structures found along the nasal mucosa [42]. Crusting from dried blood may also be seen. On general physical examination, telangiectases can be appreciated on the face, especially on the lips, oral cavity, and fingers [1]. A thorough history should include a family history significant for nosebleeds; the severity and frequency of the patient’s nosebleeds; and the need for intervention for previous nosebleeds, such as nasal packing, cauterization, iron supplementation, or blood transfusions.

Diagnosis of HHT is made based on the presence of at least two of the following criteria: recurrent nosebleeds; multiple telangiectases, especially at the lips, in the nose, and in the oral cavity; the presence of visceral arteriovenous malformations; or a positive first-degree family history of HHT [43]. If there is a suspicion of HHT, the patient should be referred to an HHT center where a multidisciplinary approach can be made for further testing and screening. These centers include geneticists, radiologists, hematologists, psychiatrists, otolaryngologists, dermatologists, cardiologists, and pulmonologists, touching on each aspect of HHT [40].

Although there is currently no cure for this condition, interventions should be performed to control the patient’s symptoms and prevent the complications that are associated with the arteriovenous malformations and telangiectases [40]. In terms of the symptom of recurrent epistaxis, several treatments may be selected based on the severity of the nosebleeds: these include (in order of treatment for mild recurrent nosebleeds to severe nosebleeds) the use of water-based nasal lubricants and environmental humidity, administration of topical estrogen/progesterone preparations,

Fig. 11.9 Endoscopic picture of the right nasal cavity of a patient with hereditary hemorrhagic telangiectasia. There are multiple lesions along the anterior septum (arrows) and middle turbinate

chemical cauterization, bipolar electrocoagulation, laser therapy, embolization of nasal arteries, and septodermoplasty. As a last resort, the Young’s procedure can be performed, which involves the closure of the nostrils [40,41].

Human Immunodeficiency Virus (HIV)

HIV is an immune-altering infection that causes increased vulnerability to potentially fatal illnesses, including opportunistic infections and malignancies. Specific to otolaryngology, approximately 80% of HIV-infected patients present with head and neck symptoms [44]. These patients have an increased risk of rhinosinusitis, both acute and recurrent. Studies suggest this increased incidence of rhinosinusitis is related to decreased CD4 counts, increased IgE production, and prolonged mucociliary transport time inherent to HIV-infected patients. Specifically, CD4 counts less than 200/mm [3] have been associated with resistant and chronic rhinosinusitis, whereas excessive IgE leads to IgE-mediated allergic rhinitis. In addition, delayed mucociliary clearance, which is usually chronic and thought to be irreversible, has been identified as a major factor of recurrent rhinosinusitis in the HIV-infected population [44–46].

Head and neck symptoms of patients with HIV infection, in order of prevalence, include oropharyngeal complaints, neck edema, and rhinologic and otologic complaints [44]. The rhinologic complaints are consistent with symptoms of rhinosinusitis: anosmia, nasal congestion, purulent discharge, fever, and facial pain and pressure. Physical examination findings on nasal exam are also consistent with those found with rhinosinusitis but are also more likely to include epistaxis and associated cervical lymphadenopathy [44,45].

Because of the head and neck manifestations, HIV infections are often diagnosed by otolaryngologists [44]. Centers for Disease Control (CDC) guidelines indicate that all patients with an opportunistic illness consistent with HIV infection should be HIV tested and counseled. Enzyme immunoassay (EIA) is the most widely used serologic test for detecting HIV-1 antibody. If this test is found reactive for HIV-1 antibody, a supplemental test is done for increased specificity, the most common of which is the Western blot [47]. If rhinosinusitis is present, a nasal culture should be sent for bacterial, fungal, and AFB (acid-fast bacilli) pathogens to aid in the selection of medical therapy. As with other disease processes, radiographic imaging is useful in determining the extent of involvement, with MRI or CT being the most sensitive. The majority of HIV-infected patients with rhinosinusitis have posterior sinus involvement; the severity of disease found on imaging is inversely related to CD4 counts [46].

Patients diagnosed with HIV are typically managed by a health care provider specializing in HIV treatment. Treatment usually includes highly active antiretroviral therapy (HAART), often as a combination of antiretroviral drugs to increase chances of successfully altering viral replication [48]. Along with antiretroviral treatment, patients may need medications for prophylaxis and/or comorbidities related to their immunocompromised state. Regarding otolaryngologic

manifestations, treatment varies depending on the pathogen isolated and may include antifungal therapy, antituberculous treatment, culture-directed antibiotics, and even chemotherapy and/or radiation therapy if applicable for malignancy [44]. Rhinosinusitis in patients with HIV infection can be difficult to eradicate, requiring the use of long-term oral or intravenous antibiotics. Surgery is indicated if medical therapy fails; in particular, functional endoscopic sinus surgery is useful in this scenario [44,46]. Additionally, preventative measures—such as use of guaifenesin, nasal irrigations, and the pneumococcal vaccine—have all been shown to have some benefit with HIV-related rhinosinusitis [49–51].

Substance Abuse: Cocaine

Although substance abuse is not generally thought of as a systemic disease contributing to rhinosinusitis, the symptoms and manifestations of substance abuse can mimic those of granulomatous sinus disease and those of rhinosinusitis. Specifically, nasal inhalation of cocaine is a form of substance abuse that directly affects the nasal and sinus cavities. Localized destruction of the nasal and sinus cavities is a direct result of the vasoconstrictive effects of inhaled cocaine. Vasoconstriction is directly followed by vasodilation of the mucosa and chronic tissue ischemia, resulting in atrophy of the surrounding tissue and increased susceptibility for anaerobic infection. Chronic vasoconstriction and tissue ischemia from intranasal cocaine abuse leads to tissue necrosis, resulting in extensive erosion of the nasal and sinus cavities [52].

Patients often present with complaints of nasal and facial pain, nasal congestion, epistaxis, and discolored or malodorous nasal discharge. Endoscopic exam may reveal septal perforation, hard palate perforation, necrosis of the nasal septum, and nasal crusting [52]. Necrosis and destruction of the nasal septum may extend to total loss of the nasal septum, saddle nose formation, and atrophy of the nasal turbinates. Not as commonly, patients may also complain of visual disturbances, including diplopia or blurred vision, accompanied by severe headaches and eye swelling (Fig. 11.10); this complaint may be indicative of sinuorbital involvement and should be further evaluated using nuclear magnetic resonance imaging [53].

To more fully evaluate nasal cavity destruction, a CT scan should be performed. Tissue destruction can mimic that of granulomatous disease. Therefore, workup should include serologic testing to determine if the destruction of the nasal mucosa results from a granulomatous disease process or from localized tissue necrosis secondary to substance abuse. Intranasal substance abuse is often not reported by patients; therefore, serology testing should include autoimmune screening as well as serum drug screening. Tissue biopsy of the nasal mucosa or nasal septum may reveal granulation tissue with inflammatory cells and may show extensive bacterial overgrowth. However, pathology will show no evidence of neoplasm or granuloma formation in patients where nasal and sinus cavity destruction is caused by intranasal substance abuse [52,54]. Subsequent to mucosal damage, secondary bacterial sinusitis is quite common [52]. Typically, the offending organism is found to be staphylococcus or streptococcus and may be identified through bacterial culture of nasal secretions [55,56].

Fig. 11.10 Endoscopic picture of a patient complaining of left eye pain who had a history of previous sinus surgery. The white powdered substance located within the left maxillary sinus is crushed narcotics, which the patient was routinely snorting

Patients found to have a history of cocaine use should be referred to drug counseling. In patients in whom extensive erosion and necrosis of the nasal septum and nasal mucosa is identified, surgical intervention may be indicated [52]. Treatment for the rhinosinusitis symptoms typically includes the use of steroids and culturedirected antibiotic therapy [53,55], but many of these treatment paradigms have suboptimal results unless the patient ceases use of intranasal cocaine.

Conclusion

Historically, the terms lethal midline granuloma or idiopathic midline destructive disease have been used to describe the process of progressive inflammation and necrosis of the nasal cavity and paranasal sinuses. Although still useful for establishing a broad differential, these all-inclusive terms have been replaced by the specific pathogens involved [36]. Current research and improved technology have given us the knowledge and tools needed to better diagnose occult otorhinolaryngologic processes. Therefore, the differential for persistent rhinosinusitis needs to include, but not be limited to, the autoimmune, infectious, and hematologiconcologic systemic diseases described in this chapter.

References

1.Tami TA. Granulomatous diseases and chronic rhinosinusitis. Otolaryngol Clin N Am 2005;38:1267–1278.

2.Lloyd G, Lund VJ, Beale T, et al. Radiology in focus: rhinologic changes in Wegener’s granulomatosis. J Laryngol Otol 2002;116: 565–569.

3.Woywodt A, Haubitz M, Haller H, et al. Wegener’s granulomatosis. Lancet 2006;367: 1362–1366.

4.Lagnese ME, Dhaliwal G. Case report: an initial diagnosis of Wegener’s granulomatosis in an 82-year-old woman. Hosp Phys 2007;52:33–36.

5.Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 1990;33:1101–1107.

6.Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med 1997;337:1124–1234.

7.Cox CE, Davis-Allen A, Judson MA. Sarcoidosis. Med Clin N Am 2005;89:817–828.

8.Wang X, Kim J, McWilliams R, Cutting G. Increased prevalence of chronic rhinosinusitis in carriers of a cystic fibrosis mutation. Arch Otolaryngol Head Neck Surg 2005;131:237–240.

9.Gilljam M, Ellis L, Corey M, Zielenski J, Durie P, Tullis E. Clinical manifestations of cystic fibrosis among patients with diagnosis in adulthood. Chest 2004;126:1215–1224.

10.Jorissen M, De Boek K, Cuppens H. Genotype-phenotype correlations for the paranasal sinuses in cystic fibrosis. Am J Respir Crit Care Med 1999;159:1412–1416.

11.Young M, Gould J, Upton, G. Nasal polyposis in children with cystic fibrosis: a long-term follow-up study. Ann Otol Rhinol Laryngol 2002;111:1081–1086.

12.Rosenstein B, Cutting G. The diagnosis of cystic fibrosis: a consensus statement. J Pediatr 1998;132:589–595.

13.Ratgen F, Doring G. Seminar: cystic fibrosis. Lancet 2003;361:681–689.

14.Flume P, O’Sullivan B, Robinson K, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007;176:957–969.

15.Abril A, Calamia KT, Cohen MD. The Churg Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum 2003;33:106–114.

16.Pagnoux C, Gulpain P, Guillevin L. Churg-Strauss syndrome. Curr Opin Rheumatol 2007;19:25–32.

17.Keogh KA, Specks U. Churg-Strauss syndrome. Semin Respir Crit Care Med 2006;27: 148–157.

18.Rapini RP, Warner NB. Relapsing polychondritis. Clinics Dermatol 2006;24:482–485.

19.Liu CM, Hata TR, Swinyer L, et al. Morphology: relapsing polychondritis. Int J Dermatol 2003;42:707–708.

20.Kent PD, Michet CJ, Luthra HS, et al. Relapsing polychondritis. Curr Opin Rheumatol 2004;16:56–61.

21.D’Cruz DP, Khamashta MA, Hughes GRV. Systemic lupus erythematosis. Lancet 2007;369:587–596.

22.Rothfield N, Sontheimer R, Bernstein M. Lupus erythematosis: systemic and cutaneous manifestations. Clinics Dermatol 2006;24:348–362.

23.Gungor A, Adusumilli V, Corey J. Fungal sinusitis: progression of disease in immunosuppression. Ear Nose Throat J 1998;77:207–212.

24.Mandava P, Chaljub G, Patterson K, et al. MR imaging of cavernous sinus invasion by mucormycosis: a case study. Clin Neurol Neurosurg 2001;103:101–104.

25.Mylona S, Tzavara V, Ntai S, et al. Chronic invasive sinus aspergillosis in an immunocompetent patient: a case report. Dentomaxillofacial Radiol 2007;36:102–104.

26.Kauffman CA. Fungal infections. Proc Am Thorac Soc (PATS) 2006;3:35–40.

27.Iwen PC, Rupp ME, Hinrichs SH. Invasive mold sinusitis: 17 cases in immunocompromised patients and review of the literature. Clin Infect Dis 1997;24:1178–1184.

28.Goh BT. Syphilis in adults. Sex Transm Dis 2005;81:448–452.

29.Baughn RE, Musher DM. Secondary syphilitic lesions. Clin Microbiol Rev 2005;18: 205–216.

30.Hamlyn E, Marriott D, Gallagher RM. Secondary syphilis presenting as tonsillitis in three patients. J Laryngol Otol 2006;120:602–604.

31.Wallace RJ, Brown-Elliott BA, Hall L, et al. Clinical and laboratory features of Mycobacterium mageritense. J Clin Microbiol 2002;40:2930–2935.

32.Cincik H, Ferguson BJ. The impact of endoscopic cultures on care in rhinosinusitis. Layngoscope 2006;116:1562–1568.

33.Griffith DE. Therapy of nontuberculous mycobacterial disease. Curr Opin Infect Dis 2007;20:198–203.

34.Hart CA, Rao SK. Rhinoscleroma. J Med Microbiol 2000;49:395–396.

35.Yilmaz M, Aydil U, Vural C, et al. Simultaneous occurrence of nasal and cervical rhinoscleroma. J Otolaryngol 2006;35:206–208.

36.Chain JR, Kingdom TT. Non-Hodgkin’s lymphoma of the frontal sinus presenting as osteomyelitis. Am J Otolaryngol 2006;28:42–45.

37.Thompson LD. Mini-symposium: head and neck pathology—sinonasal carcinomas. Curr Diagn Pathol 2006;12:40–53.

38.Al-Hakeem DA, Fedele S, Carlos R, et al. Extranodal NK/T-cell lymphoma, nasal type. Oral Oncol 2007;43:4–14.

39.Chen S, Wu CS, Chan K, et al. Primary sinonasal non-Hodgkin’s lymphoma masquerading as chronic rhinosinusitis: an issue of routine histopathological examination. J Laryngol Otol 2003;117:404–407.

40.Sabba` C. A rare and misdiagnosed bleeding disorder: hereditary hemorrhagic telangiectasia. J Thromb Haemost 2005;3:2201–2210.

41.Kuhnel¨ TS, Wagner BH, Schurr CP, et al. Clinical strategy in hereditary hemorrhagic telangiectasia. Am J Rhinol 2005;19:508–513.

42.Geisthoff UW, Sittel C, Plinkert PK. Contact endoscopic findings in hereditary hemorrhagic telangiectasia. Head Neck 2005;28:56–63.

43.HHT Foundation International. Hereditary Hemorrhagic Telangiectasia page. Available at: http://www.hht.org/index.php. Accessed July 12, 2007.

44.Prasad HK, Bhojwani KM, Shnoy V. HIV manifestations in otolaryngology. Am J Otolaryngol 2006;27:179–185.

45.Milgrim LM, Rubin JS, Small CB. Mucociliary clearance abnormalities in the HIV-infected patient: a precursor to acute sinusitis. Larnygoscope 1995;105:1202–1208.

46.Godofsky EW, Zinreich J, Armstrong M, et al. Sinusitis in HIV-infected patients: a clinical and radiographic review. Am J Med 1992;93:163–170.

47.Centers for Disease Control. Revised Recommendations for HIV testing of Adults, Adolescents, and Pregnant Women in Health-Care Settings page. Available at: http://www.cdc.gov/ mmwr/preview/mmwrhtml/rr5514a1.htm. Accessed July 12, 2007.

48.Yeni PG, Hammer SM, Hirsch MS, et al. Treatment for adult HIV infection: 2004 recommendations of the International AIDS Society-USA panel. JAMA 2004;292:251–265.

49.Wawrose SF, Tami TA, Amoits CP. The role of guaifenesin in the treatment of sinonasal disease in patients infected with the human immunodeficiency virus. Laryngoscope 1992;102:1225–1228.

50.Tomooka LT, Murphy C, Davidson TM. Clinical study and literature review of nasal irrigation. Laryngoscope 2000;110:1189–1193.

51.Shafinoori S, Ginocchio CC, Greenberg AJ, et al. Impact of pneumococcal conjugate vaccine and the severity of winter influenza-like illnesses on invasive pneumococcal infections in children and adults. Pediatr Infect Dis J 2005;24:10–16.

52.Talbott JF, Gorti GK, Koch RJ. Midfacial osteomyelitis in a chronic abuser: a case report. Ear Nose Throat J 2001; 80:738–743.

53.Neugebauer P, Fricke J, Neugebauer A, et al. Sinuorbital complications after intranasal cocaine abuse. Strabismus 2004;12:205–209.

54.Leibovitch I, Khoramian D, Goldberg RA. Severe destructive sinusitis and orbital apex syndrome as a complication of intranasal cocaine abuse. Am J Emerg Med 2006;24:499–501.

55.Gordon RJ, Lowy FD. Bacterial infection in drug users. N Engl J Med 2005;353:1945–1954.

56.Klutmans J, van Belkum A, Verburgh H. Nasal carriage of Staphylococcus aureus: epidemiology, underlying mechanisms, and associated risks. Clin Microbiol Rev 1997;10:505–520.