Multidisciplinary care in the intensive care unit for a patient with Prader-Willi syndrome: a dental approach Assistência multiprofissional em unidade de terapia intensiva ao paciente portador de síndrome de Prader-Willi: um enfoque odontológico

Prader-Labhart-Willi or Prader-Willi syndrome (PWS) was first reported in 1956, and additional syndrome details have been elucidated since then. This syndrome is primarily characterized by severe neonatal hypotonia, dysmorphic changes, retarded physical and cognitive development, early hyperphagia, obesity, sleep disorders, psychiatric and behavioral disorders, growth deficit and hypogonadism. Its population incidence ranges from 1:15,000 to 1:50,000 live births, independent of gender, race or social status.(1-5) This syndrome is considered to be a complex neurobehavioral genetic disorder caused by 15q11.2-q13 chromosome changes.(2,3) Among oral features, prominent examples include hypoplasia of the enamel of the teeth, rampant cavities, taurodontism, delayed dental eruption, excessively worn teeth, facial hypotonia resulting in severe poor occlusion, biofilm accumulation, gingivitis, Candida albicans infections, geographic tongue and skin lesions.(4,5) PWS newborns typically have difficulty in sucking due to facial Juliana Santiago Setti1, Sérgio Felix Pinto2, Ellen Cristina GaettiJardim3, Gustavo Rodrigues Manrique3, José Carlos Garcia de Mendonça3