Case Report of Patient with Hunter Syndrome (Mucopolysaccharidosis II)

Abstract

Hunter syndrome (mucopolysaccharidosis type II), an X-linked recessive inherited congenital heredity disease, is quite rate. The syndrome develops due to a deficiency of glycosaminoglycan degrading enzymes, which causes multi-organ disease from accumulation of metabolites in tissues throughout the whole body. We report here treatment of an 8-year-8-month-old boy with Hunter syndrome for dental caries. Blood oxygen saturation in the patient was remarkably decreased during dental treatment performed using regular behavior management, with that intraoperative hypoventilation condition determined to be caused by general anesthesia. Thus, we performed the necessary dental treatment using a minimally invasive method while monitoring changes in oxygen saturation. Our experience with this case highlighted the importance of recognizing disease symptoms for best treatment planning.