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Bridging oral and systemic health in children with Prader-Willi syndrome: Case reports and dental treatment recommendations

Author(s):

Jaclyn Vu and Priyanshi Ritwik*  

Abstract:


Background: Prader-Willi Syndrome (PWS) is a complex neurodevelopmental disorder caused by gene alterations on chromosome 15q11-q13 resulting in hyperphagia and neuroendocrine deficits. A comprehensive guide for dental treatment for PWS is lacking despite numerous case reports. The objective of this report and review was to develop a problem-focused list of the interrelationship between oral and systemic parameters of PWS and enable dentists in anticipating the unique treatment needs of children and individuals with PWS.

Methods: Four pediatric patients with PWS presenting to an academic dental clinic were evaluated. A literature review spanning the last twenty years was performed to identify the pathophsyiological impact of systemic problems on dental health and treatment.

Results: The four cases along with cases from literature were used to enumerate salient oro-dental and systemic features influencing treatment decisions in dentistry. They formed the basis for collective recommendations and precautions for rendering dental treatment in patients with PWS.

Conclusion: Sedation for dental treatment is contraindicated due to obesity (BMI over 95th percentile), hypotonia, obstructive sleep apnea (OSA) and respiratory limitations (restricted ventilation due to weight on thoracic cage). Prolonged recovery from general anesthesia, OSA and temperature dysregulation necessitate extended monitoring after dental rehabilitation under general anesthesia. Orthopedic problems and respiratory limitations exclude protective stabilization. Xerostomia and acidic saliva necessitate recommendations for oral rehydrating products. Periodontal assessment is necessary due to poor oral hygiene and diabetes mellitus. Early establishment of a dental home and risk-based frequency of dental care should address caries prevention and restorative needs.

Keywords:

Prader-Willi syndrome, dental care, oral findings

Affiliation:

UT Health Science University School of Dentistry Department of Pediatric Dentistry, Department of Pediatric Dentistry The University of Texas Health Science Center at Houston, School of Dentistry 7500 Cambridge Street, Ste 5301



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