Bridging Oral and Systemic Health in Children with Prader-Willi Syndrome: Case Reports and Dental Treatment Recommendations

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 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 pathophysiological impact of systemic problems on dental health and treatment. Results: The four cases along with cases from the 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.