Vol. 17, No. 5
THE JOHNS HOPKINS MICROBIOLOGY NEWSLETTER
Monday, February 2, 1998
A. Provided by Carmela Groves, R.N., M.S., Chief, Division of Outbreak Investigation, Maryland Department of Health and Mental Hygiene
No information provided this week.
B. The Johns Hopkins Hospital: Information provided by Dr. Blaire Baisden, Department of Pathology
Case Presentation
A forty-six year old female complained of persistent headache during a clinic visit with her oncologist. She had completed chemotherapy for acute myelogenous leukemia four weeks prior to presentation and had experienced slow marrow recovery. Computed tomography of the head showed complete opacification of the left maxillary and ethmoid sinuses. On examination by an otolarnygologist, the patient was noted to have dark discoloration of the nasal mucosa. Frozen section examination showed broad, nonseptate hypae with necrosis and prominent angioinvasion. Fungal culture subsequently grew Cunninghamella sp. Antifungal therapy was initiated and the patient underwent extensive surgical debridement.
Fungal sinusitis
The incidence of fungal sinusitis has shown a precipitous rise since the 1970s due to an increase in immunosuppressive therapies as well as a heightened awareness of the entity. The specific type of fungal disease is dependent upon environmental, host immune status and local tissue factors. Four major types of fungal sinusitis are recognized.
Allergic fungal sinusitis is a non-invasive form of fungal sinusitis that primarily occurs in young adults with long histories of asthma and nasal polyposis. Most patients have elevated IgE and absolute eosinophil counts. The diagnostic feature of allergic fungal sinusitis is "allergic mucin" an eosinophilic or basophilic mucoid material with embedded eosinophils, sloughed epithelial cells, cellular debris and Charcot-Leyden crystals imparting a distinctive laminated "tidewater" appearance. Fungal hyphae may be sparse or inapparent even with special stains. The mainstays of therapy for allergic fungal sinusitis are systemic steroids and removal of the tenacious mucus to restore mucociliary function. Antifungal therapy has no role in the treatment of allergic fungal sinusitis.
Mycetoma fungal sinusitis is an extramucosal "fungal ball" within a sinus cavity, most frequently the maxillary sinus. The host is typically immunocompetent but may have a history of trauma or insult to the affected sinus. Histologically, the fungus elicits little or no inflammatory response. Because the fungus is noninvasive, simple curettage is usually curative and antifungal therapy is not indicated.
Chronic indolent sinusitis is an invasive form of fungal sinusitis in patients without identifiable immune deficiency. In contrast to fulminant forms in immunocompromised patients, the disease is indolent progressing over months to years. It is characterized clinically by chronic headache and progressive facial swelling and may lead to visual impairment. Microscopically, chronic indolent sinusitis is characterized by a granulomatous inflammatory infiltrate. Depending on the immune status of the host, these patients may be at risk for fulminant invasive disease.
Fulminant sinusitis is usually seen in the imunocompromised host and is characterized by progressive destruction of the sinuses and extension into the orbit and brain. Microscopically, there is a destructive pattern of invasive growth with prominent tissue necrosis and frequent angioinvasion. In contrast to chronic indolent sinusitis, there is scant inflammatory host response. Aspergillus, Mucormycosis, Pseudoallescheria boydii and Fusarium sp. are the most common pathogens. The mainstays of therapy for both chronic, indolent and fulminant sinusitis are aggressive surgical debridement and intravenous antifungal therapy.
References:
1. Westra W. Pathology of nasal cavity and paranasal inuses. Critical Issues of Surgical Pathology, 1997.
2. Corey JP, Romberger CF, Shaw GY. Fungal diseases of the sinuses. Otolarngol Head Neck Surg 1009; 103: 1012-15.
3. Katzenstein LA, Sale AR, Greenberger PA. Pathologic findings in allergic aspergillus sinusitis. Am J Surg Pathol 1983; 7: 439-43.