Intrathecal aspergillosis and fusiform arterial aneurysms in an immunocompromised child: a clinico-pathological case report

We describe a 12-year-old boy with subarachnoid hemorrhage. Cerebral angiography revealed multiple fusiform intracranial arterial aneurysms. Surgical therapy other than ventricular drainage was not feasible due to the extent and pattern of pathological changes. Aspergillus antigen was found in the cerebrospinal fluid (CSF). However, cultures of urine, CSF, and samples from the upper airways were negative for Aspergillus and other fungi. The boy was immunocompromised due to prophylactic antibiotic therapy for recurrent pneumonia and continuous steroid therapy for his hemolytic anemia. There were Aspergillus antigens but no evidence of ongoing infection with the fungus was found. The patient suffered recurrent intraventricular hemorrhage and died on the 31st day after admission. Pathological examination showed multiple fusiform aneurysms, hypertrophy of the intima, and destruction of the internal elastic membrane of the cerebral arteries. No fungus infiltration was detected. We speculate that the patient had previously had an Aspergillus infection, causing segmental destruction of the internal elastic membrane. However, we cannot rule out noninfectious vasculitis as possible etiology. In the immunocompromised child, fusiform segmental intracranial arterial aneurysms may be secondary to the destruction of the internal elastic membrane by fungus infection. The condition may present with aneurysm rupture and subarachnoid hemorrhage. In this patient group, surgical therapy is rarely an option and the prognosis is poor.