Assistência multiprofissional em unidade de terapia intensiva ao paciente portador de síndrome de Prader-Willi: um enfoque odontológico

Abstract

Assistência multiprofissional em unidade de terapia intensiva ao paciente portador de síndrome de Prader-Willi: um enfoque odontológico INTRODUCTIONPrader-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)(2)(3)(4)(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

Special Care Dentistry in a Patient with Prader–Willi Syndrome through the Use of Atraumatic Restorative Treatment under General Anesthesia

“…The oral changes observed are compatible with those present in the scientific literature with multiple dental caries [ 3 , 10 , 11 ], tooth 74 with pulp necrosis and dentoalveolar abscess, generalized enamel demineralization [ 10 ], reasonable oral hygiene [ 9 , 10 , 14 ], presence of biofilm [ 12 ], atypical unobtrusive swallowing, hypotonic tongue, and predominantly oral breathing.…”

“…The presence of a precarious oral hygiene encounters PWS patient's systemic conditions due to the cognitive and self-care deficit associated with the patient's behavioral disturbance, evinced by a prior poor oral hygiene. Besides, it is suggested that the lack of precocious multidisciplinary support may contribute to the opportunistic disease advance [ 9 ].…”

<p>Oral health in patients with Prader-Willi syndrome: current perspectives</p>

“…Prader-Willi syndrome (PWS) presents odontological aspects of interest. Many deleterious effects on the oral cavity were associated with this behavioral and endocrine disorder that may cause surgery and/or worsening of the clinical condition 1…”

“…A variety of oral diseases are found in patients with PWS (Table 2) such as caries,1,17,21,25–31 hypoplastic enamel,1,17,25,28,31 viscous saliva,1,17,21,25,30–32 periodontal disease,25–28 tooth wear,1,26,27,31,32 dental erosion,33 rampant caries,1,29,31 oral microsomia,25,31 dental malocclusion,1,17,21,26–29 candidiasis,25,27 erythematous lesions of the oral mucosa,27 increased biofilm accumulation,1,21,25,31,32 gingivitis,1,25,27,31,32 angular cheilitis, dry mouth,28 demineralization,21,25,31 dentoalveolar abscess,29,31 hypotonic tongue,21,31 mouth breathing,21,31,32 root stumps30 and a delay in tooth eruption 28Table 2Oral manifestation, referenceOral manifestation Júnior et al, 2005Scardina; Fuca; Messina, 2007Bailleuil-Forestier et al, 2008Yanagita et al, 2011Witt et al, 2011Setti et al, 2012Gardens et al, 2014Song et al, 2015Hurren, Flack, 2016 (review)Tayal et al, 2016Roman-Torres et al, 2017Olczak-Kowalczyk et al, 2018*…”

“…Even though some centers are successful in maintaining satisfactory dental conditions,33,36 other studies reported episodes of caries,1,21,25–31 rampant caries, and gingivitis 1,29,31. There was also a report of a patient with PWS who needed hospitalization due to pneumonia and subsequent referral to ICU for mechanical ventilation with a diagnosis of septic shock secondary to hospital pneumonia 1. An integrated multidisciplinary approach starting from the neonatal period is mandatory20 to greatly improve the health and quality of life in children with PWS 40.…”