Dental Findings on the Maple Syrup Urine Disease

In 1954, Menkes first described a metabilic obstruction of a herediatry nature caused by leucine, isoleucine and valine branching amino acid, which is now generally accepted as the Maple Syrup Urine Disease. There are no reports of oral involvement regarding this disease. The patient, a 5 year 4 month old girl, diagosed as having the Maple Syrup Urine Disease at the Department of Pediatrics Clinic, Kagoshima University School of Medicine, was referred to the Pedodontic Clinic, Kagoshima University School of Dentistry. The results were as follows: 1) In findings of the dental arch cast, the length and width of the dental arch were found to be small in size. 2) Susceptibility to dental arch caries was more serious than normal child due to her bad eating habits, poor dental health care and ebur dentis. 3) If their systemic condition is kept under control, we can consider patients with the Maple Syrup Urine Disease as a normal child.