Cryptococcus gattii infection complicated by immune reconstitution inflammatory syndrome in three apparently immunocompetent children

Background Paediatric Cryptococcus gattii disease is rare, with only two previous cases recorded in the Northern Territory (NT) over the last 54 years. Immune reconstitution inflammatory syndrome (IRIS) is a recognised complication of C. gattii infection, even in the absence of an identified immunodeficiency syndrome; however, limited paediatric data exist. We present a series of three paediatric patients treated for C. gattii infection in the NT during 2016/2017. Case discussions All three cases were males aged 8-13 years at the time of presentation. Two were Aboriginal Australians from remote NT communities, and the third was a Timorese child from a remote district in Timor-Leste. All cases had evidence of brain cryptococcomas, and two had associated pulmonary lesions. Each child was treated with a 6-week induction phase of intravenous liposomal amphotericin and flucytosine and then continued on a 2-year course of eradication oral fluconazole. Persistent high intracranial pressure (ICP) complicated each case, requiring serial lumbar punctures and, in two cases, insertion of ventriculoperitoneal shunts. All three cases were diagnosed with IRIS between 5 and 10 weeks after commencement of antifungal treatment and were managed with high-dose corticosteroids, which were weaned slowly (6-20 months post-commencement). Conclusions Paediatric C. gattii disease is rare, although three recent cases in the NT highlight some of the challenges involved in managing the infection, including persistent raised ICP and complications such as IRIS. There is a need for further collaborative research into paediatric C. gattii disease.