{Reference Type}: Systematic Review
{Title}: The oral phenotype and dental management in patients with maple syrup urine disease; case report and scoping review.
{Author}: Hassona Y;Alqaisi D;Flaifl Y;Alkilani A;
{Journal}: BMC Oral Health
{Volume}: 24
{Issue}: 1
{Year}: 2024 Mar 21
{Factor}: 3.747
{DOI}: 10.1186/s12903-024-04135-7
{Abstract}: <B>OBJECTIVE: </B>The literature about oral manifestations and dental management in maple syrup urine disease (MSUD) is sparse. The aim of this report is to present a new case of MSUD with special emphasis on oral findings and to review the relevant literature.<BR><B>METHODS: </B>A case report of a 4-year-old boy with MSUD was described according to the CARE guidelines for describing case reports. Scoping review of relevant literature was performed, according to the PRISMA-ScR guidelines, by searching PubMed, Medline, Embase, and the grey literature for articles describing dental management and/or oral manifestations in MSUD.<BR><B>RESULTS: </B>The initial search identified 219 articles, but only 4 met the inclusion criteria. Rampant caries and plaque induced gingivitis were the main oro-dental findings in MSUD. Other oral findings included enamel hypoplasia, skeletal abnormalities, and abnormal oral behaviors. Disease-related factors appeared to play a major role in the development of the observed oral phenotype.<BR><B>CONCLUSIONS: </B>Oral health in MSUD seems to be influenced by the reliance on semi-synthetic diet and associated neurocognitive complications. Tailored oral health promotional interventions should be included in the multidisciplinary management of patients with MSUD.