Ritwik Priyanshi, Vu Jaclyn
Department of Pediatric Dentistry, The University of Texas Health Science Center at Houston, School of Dentistry, 7500 Cambridge Street, Ste 5301,Canada.
Department of Pediatric, School of Dentistry, Dentistry, UT Health Science University, TX,United States.
Curr Pediatr Rev. 2021;17(4):336-344. doi: 10.2174/1573396317666210913101027.
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.
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.
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.
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.
普拉德-威利综合征（PWS）是一种复杂的神经发育障碍，由15号染色体q11-q13区域的基因改变引起，导致食欲亢进和神经内分泌缺陷。尽管有大量病例报告，但仍缺乏针对PWS牙科治疗的综合指南。本报告的目的是制定一份以问题为重点的清单，列出PWS口腔和全身参数之间的相互关系，使牙医能够预见PWS儿童和患者的独特治疗需求。
对四名到学术牙科诊所就诊的PWS儿科患者进行了评估。对过去二十年的文献进行了综述，以确定全身问题对牙齿健康和治疗的病理生理影响。
这四个病例以及文献中的病例被用来列举影响牙科治疗决策的显著口腔和牙齿及全身特征。它们构成了为PWS患者提供牙科治疗的集体建议和预防措施的基础。
由于肥胖（体重指数超过第95百分位）、肌张力减退、阻塞性睡眠呼吸暂停（OSA）和呼吸受限（因胸廓受压导致通气受限），牙科治疗禁忌使用镇静剂。全身麻醉后恢复时间延长、OSA和体温调节异常需要在牙科修复手术后延长监测时间。骨科问题和呼吸受限排除了保护性固定。口干和酸性唾液需要推荐口服补液产品。由于口腔卫生差和糖尿病，有必要进行牙周评估。尽早建立牙科之家并根据风险确定牙科护理频率应满足龋齿预防和修复需求。
