Original ArticleSpinal Coccidioidomycosis: A Current Review of Diagnosis and Management
Introduction
Coccidioidomycosis is an infectious disease caused by Coccidioides sp. (Coccidioides posadasii and Coccidioides immitis), an invasive, dimorphic fungus primarily found in the soil as mycelia-forming endospores, usually carried in the air.1, 2, 3, 4, 5, 6 In one-third of cases, coccidioidomycosis manifests as a self-limited community-acquired pneumonia; however, up to 60% of patients are asymptomatic, and extrathoracic dissemination may be the initial presentation, with patients experiencing fever, rash, or flulike symptoms.1, 2, 3, 4, 6 The fungus is endemic to the Sonoran desert region of southern Arizona, northern Mexico, and the San Joaquin Valley in California.1, 2, 4 The annual incidence of coccidioidomycosis in the United States ranges from 100,000 to 150,000 persons, and the infection rate is estimated at 3% per year in Arizona and dissemination occurs in 1% to <5% of those infected.1, 3, 4, 5, 7
Although dissemination is associated with immunocompromised patients,1, 2, 7, 8 disease of the vertebral column and adjacent structures is most frequently diagnosed in immunocompetent men and those of African origin,2 and it represents 19% of all extrathoracic presentations.1 Despite extensive case reports and series,1, 2, 3, 4, 5, 6, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21 the medical literature lacks studies focused specifically on spinal coccidioidomycosis. To our knowledge, this is the first review of the surgical and medical management of patients with spinal coccidioidomycosis, including the joint experience of the neurosurgical and infectious disease departments at our institution in an endemic area.
Section snippets
Methods
A review of the English-language medical literature was performed on PubMed using the keywords “coccidioidomycosis,” “cocci,” “spine,” “surgical,” “management,” and “vertebral.” Additional articles were located by cross-referencing publications encountered initially through PubMed searches. Inclusion criteria were articles (case reports, case series, literature reviews, and guidelines) originating from the peer-reviewed that described the risk factors, clinical presentation, diagnostic tests,
Demographics and Risk Factors
The clinical articles analyzed included 140 patients, 133 of whom were male (95%). Mean age was 36.2 years (range, 5–82 years). Ethnicities represented were African American (n = 73, 52%), white (n = 20, 14%), Hispanic (n = 17, 12%), Asian (n = 10, 7%), and Native American (n = 3, 2%); ethnicity was not specified for 17 patients (12%).
The most common demographic variables associated with disease included male sex, African American ethnicity, and inhabitance in an endemic area for more than 3
Management
Antifungal therapy consisted of amphotericin B and various azoles. The single most-used antifungal medication was amphotericin B in 61 patients (44%), followed by fluconazole in 21 patients (15%), voriconazole in 9 patients (6%), and itraconazole in 5 patients (4%). One patient (0.7%) each received posaconazole, ketoconazole, and miconazole. An unspecified azole drug was used in 47 patients (34%). In all, azoles were given to 84 patients (60%). Polytherapy (1 or more azoles with or without
Demographics and Risk Factors
Male sex, African American ethnicity, residing in an endemic area, steroid use, and being immunocompromised are the most commonly reported risk factors for extrathoracic coccidioidomycosis dissemination. In our analysis, patients were overwhelmingly male (95%) and more than half of the patients were African American, consistent with previous results.2, 3, 5, 6, 10, 11, 16 Human leukocyte antigen class II-DRB1*1301 allele is a possible immunologic factor associated with severe dissemination,
Limitations
This is a review of the literature published from 1977 to 2014. Results presented in this study may not be reproducible in different clinical or radiologic scenarios. Important metrics such as follow-up period length, antifungal use (type, dose, timing of dose, and duration of use), risk factors, and ethnicity were not detailed in all the studies analyzed. Data in retrospective studies require mining, and inconsistencies are present because all the information to be studied was not readily
Conclusions
African American ethnicity and male sex are significant risk factors for vertebral dissemination of coccidioidomycosis. Most affected patients are immunocompetent and do not have a history of steroid use. Pulmonary symptoms are generally not present and the association with central nervous system dissemination is uncommon. Aggressive medical therapy is required, including amphotericin B and/or azoles. In most cases, surgical intervention is needed in patients with neurologic compromise or
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.