MULTIPLE POSTERIOR FOSA ASPERGILOMAS IN A PATIENT WITH CHRONIC GRANULOMATOSIS

Abstract

The invasive infection with Aspergillus fumigatus usually occurs in immunocompromised patients. CNS involvement is extremely rare in children. Usually, the diagnosis is hard, often made after the death of the patient. We describe the first report of a pediatric case of multiple CNS aspergillomas confirmed by a biopsy. We present the case of 3 years old boy who was hospitalized for evolving intracranial hypertension with a cerebellar syndrome. A brain CT showed a left cerebellar mass and early tonsillar commitment and a supra-tentorial lesion in the right occipital lobe. Therefore, a metastatic tumor was suspected and an emergency surgical intervention with cerebral biopsy and ventriculocisternostomy was performed. The cerebral biopsy revealed an Aspergillus fumigatus granuloma and further investigations showed that the patient has chronic granulomatosis due to a homozygous mutation of CYBA gene encoding p22phox. After a favorable evolution in reanimation, he continued the treatment with Voriconazole. At last follow-up, the neurological examination finds no sign of motor focus, no cerebellar syndrome or nystagmus. Walking is difficult with the enlargement of the support base. CNS invasive infection with Aspergillus fumigatus in a child is extremely rare and the presentation might mimic that of a tumor especially with a location of aspergilloma as in our case both supratentorially and infratentorially.

Keywords

• cerebellar aspergilloma
• cyba gene
• intracranial pressure