Maple Syrup Distribution Disease (MSUD) in a Pediatric Patient: Clinical Course of Conservative Management in Two Cases

Maple syrup urine disease (MSUD) is a rare, autosomal recessive metabolic disorder caused by a genetic defect in the metabolism of branched-chain amino acids. This metabolic disorder in children can affect tooth development and increase the prevalence of caries. During dental treatment, the systemic conditions of these patients should be considered, procedures that may create metabolic stress should be avoided, and treatment planning should be undertaken with a multidisciplinary approach. This case report examines conservative dentistry approaches in two pediatric patients diagnosed with MSUD. The study aimed to address the pain, functional, and aesthetic needs of two siblings diagnosed with MSUD, alleviate the adverse effects on gingival health, and treat tooth sensitivity. In both cases, invasive procedures were avoided, and treatments were performed using noninvasive methods whenever possible. Behavior management techniques increased patient compliance, and local anesthesia administration and procedure durations were planned with consideration of metabolic stress factors. Preventive treatments were implemented to prevent premature and unnecessary tooth loss, and patients were invited for follow-up appointments at six-month intervals. The purpose of this case report is to highlight the points to consider during dental treatment in individuals diagnosed with MSUD and to demonstrate the effectiveness of the conservative approach.