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

Abstract

Prader–Willi syndrome described in 1956 has a genetic origin, affecting both genders, varying in presence and intensity from individual to individual. A precocious diagnosis, before the manifestation of symptoms, has brought some improvement in the quality of life of the carriers in the last years. The objective of this case report was to describe the treatment realized in a 3-year-old boy who presented grade II obesity, difficulty of locomotion, hypotonia, and history of cardiopathy. A dental treatment under general anesthesia was defined, allowing an oral adequation in a single section, in which it was planned the extraction of the element 74 and atraumatic restorative treatment (ART) technique in the other teeth. The precocious intervention in this 3-year-old patient by the therapy realized with ART under general anesthesia was done with success, avoiding unnecessary extractions, preserving dental elements, and maintaining the oral cavity in adequate function.

Hygienic behaviors and use of dental care in patients with genetic syndromes

“…Angelman syndrome is characterized by developmental delay or mental retardation, severe speech abnormalities, gait ataxia and/or limb tremors, and exceptional seemingly joyful behavior (frequent laughter, smiling and excitability) [21]. These disorders make it di cult to perform hygienic procedures with the patient at home, and dental care is sometimes limited to emergency acute care and further treatment under general anesthesia [12,13,22]. Depending on the patient's cognitive ability and cooperation, professional preventive and even curative interventions are possible, and most importantly, parental education in oral hygiene care, proper nutrition and hydration [23,24].…”

“…Phenotypic features of syndromes involving the craniofacial region, e.g., symptoms of facial dysmorphia, hypo-or hypertonia of muscles, cleft palate, which require interdisciplinary specialized treatment, especially orthodontic, surgical, prosthetic and physiotherapy [6,8,9], are also a health problem. It is important to provide integrated dental care, in which maintaining dental health, especially the prevention and treatment of dental caries, is fundamental [12,13].…”

“…; SRS; Angelman syndrome (AS)), crowding of the teeth, enamel developmental disorders (SLOS; DS; SRS; AS), muscle hypotonia and mouth breathing (SLOS; PWS), tongue dysfunction (DS), bruxism (PWS), gastroesophageal re ux (SLOS; PWS), immune de ciency (DS) [6][7][8][9]. Many of the aforementioned factors can be controlled by paying attention to oral hygiene, proper nutrition and continuous dental care from birth to adulthood [10][11][12]. Phenotypic features of syndromes involving the craniofacial region, e.g., symptoms of facial dysmorphia, hypo-or hypertonia of muscles, cleft palate, which require interdisciplinary specialized treatment, especially orthodontic, surgical, prosthetic and physiotherapy [6,8,9], are also a health problem.…”

Prader‐Willi syndrome: Periodontal‐prosthodontic rehabilitation in an adult patient

“…This maximized efficiency for all involved, to include the patient. Roman-Torres et al recently described a pediatric PWS case successfully treated in a hospital environment with general anesthesia, 8 underscoring how sedation should be considered versus conventional treatment in this category of patients. Dougall and Fiske, in an article on special care dentistry services for patients with eating disorders, nicely outlined anesthesia and sedation concerns in individuals with PWS.…”

“…4 Once the PWS patient reaches adulthood, however, the focus usually becomes one of treating multiple chronic problems resultant from this condition. 3 Oral health conditions commonly seen in PWS children and adults include rampant caries, minimal thick salivary flow, enamel hypoplasia leading to severe dental erosion and attrition, candidiasis, delayed dental eruption with possible malocclusion [5][6][7][8][9][10][11] and periodontal disease. 12,13 High refined carbohydrate intake combined with inadequate oral hygiene can further advance the complications seen in this patient population, as well as higher rates of diabetes, respiratory problems, and cardiovascular issues.…”