Spinal Coccidioidomycosis: A Current Review of Diagnosis and Management

Objective

Coccidioidomycosis is an invasive fungal disease that may present with extrathoracic dissemination. Patients with spinal coccidioidomycosis require unique medical and surgical management. We review the risk factors and clinical presentations, discuss the indications for surgical intervention, and evaluate outcomes and complications after medical and surgical management.

Methods

A review of the English-language literature was performed. Eighteen articles included the management of 140 patients with spinal coccidioidomycosis.

Results

For the 140 patients, risk factors included male sex (95%), African American ethnicity (52%), and a recent visit to endemic areas (16%). The most frequent clinical presentation was pain (n = 80, 57%), followed by neurologic compression (52%). One-third of patients had concurrent pulmonary disease. The sensitivity of culture and histology for coccidioidomycosis was 80% and 90%, respectively. Complement fixation titers >1:128 suggest extensive or refractory vertebral infection. The most commonly affected spinal segments were the thoracic and lumbar spine (69%); an additional 40 patients (29%) had epidural and paravertebral abscesses. All patients received therapy with azoles (60%) and/or amphotericin B (43%). Surgical and medical management were used conjunctively to treat 110 patients (79%), with debridement (95% [105/110]) and fusion (64% [70/110]) being the most common surgical procedures. Clinical outcome improved/remained unchanged in 83 patients (59%) and worsened in 4 patients (3%). The mortality was 7%. Infection recurrence and disease progression were the most frequent complications.

Conclusions

Emphasis should be placed on continuous and lifelong appropriate azole therapy. Spinal instability and neurologic compromise are surgical indications for decompression and fusion.

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.