Abstract
Opsoclonus myoclonus syndrome (OMS), also known as Kinsbourne Syndrome, is a rare disorder affecting the central nervous system. It presents clinically with ataxia, diffuse or focal muscle spasms, and rapid, irregular eye movements. Hyper-IgM (HIGM) syndrome is a rare primary immunologic disorder which causes susceptibility to repeated opportunistic infections due to lack of immunoglobulin class switching. We present the case of a 5-year-old male with both OMS and HIGM syndrome who underwent general anesthesia for an emergency appendectomy. The goals and challenges of anesthetic management that attenuates immunosuppression for these two infrequent conditions in a single pediatric patient are discussed.