Coccidioidomycosis is a common fungal infection in the southwestern US. Hydrocephalus is a serious complication of cranial coccidioidomycosis, and the surgical management of coccidioidomycosis-related hydrocephalus has unique challenges. The authors reviewed their institutional experience with hydrocephalus in the setting of coccidioidomycosis.
The authors retrospectively identified 44 patients diagnosed with coccidioidomycosis-related hydrocephalus at their institution since 1990, who underwent a total of 99 shunting procedures. The authors examined patient demographics, type of shunt and valve used, pressure settings, failure rates, medical treatment, ventricular response to shunting, and other variables.
The majority of patients were young (average age 37 years) men (male/female ratio 28:16) with a mean follow-up of 63 months. Patients of Asian and African descent were overrepresented in the cohort compared with regional demographic data. The overall shunt failure rate during follow-up was 50%, and the average number of revisions required if the shunt failed was 2.5 (range 1–8). Low to moderate draining pressures (mean 88 mm H2O) were used in this cohort. Fourteen patients received intrathecal antifungals, and a trend of initiating intrathecal therapy after need for a shunt revision was observed (p = 0.051). The majority of shunt failures (81%) were due to mechanical blockages in the drainage system. Most patients (59%) had at least partial persistent postoperative ventriculomegaly despite successful CSF diversion. Four patients (9%) died due to coccidioidomycosis during the follow-up period.
Coccidioidomycosis-related hydrocephalus more often affected young males in the study's cohort, especially those of African and Asian descent. Despite the best medical therapy, there was a high rate of shunt failure due to clogged catheters or valves due to the underlying disease process. Many patients continued to have ventriculomegaly even with adequate CSF diversion. The morbidity and mortality of this chronic disease process must be recognized by the treatment team, and patients should be appropriately counseled.