Abstract
Introduction: Self-injurious behavior is one of the characteristic symptoms of Lesch-Nyhan syndrome. In severe cases, the patient's lips or tongue are often injured, but there are no reports of cases leading to infective endocarditis via bacteremia. There are non-invasive treatments for self-injurious behavior, but surgery is occasionally needed. Herein, we report a case of Lesch-Nyhan syndrome that required surgery to control self-injurious behavior.
Case presentation: A 21-year-old boy with Lesch-Nyhan syndrome exhibited uncontrollable self-injurious behavior. He visited our hospital with a high fever. Physical examination revealed ulcerations on both his lips and tongue, and there was also a defect on his tongue. All blood cultures that were taken were positive. Vegetations were found on the heart valves through echocardiography, revealing that he has infective endocarditis. To prevent recurrence of ulceration, we performed total tooth extraction and glossectomy. After four months, there is no recurrence of ulcerations or infective endocarditis.
Discussion: Although total tooth extraction is irreversible, invasive, and has a significant impact on the patient's appearance, it is a definite way not only to prevent ulcerations caused by self-injurious behavior but also to protect his life.
