Login Register Cart 0
Generic placeholder image

Current Pediatric Reviews

Editor-in-Chief

ISSN (Print): 1573-3963
ISSN (Online): 1875-6336

Back
Journal Home About Journal Editorial Board Journal Insight Current Issue Volumes/Issues
Subscribe
Case Report

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

Author(s): Priyanshi Ritwik* and Jaclyn Vu

Volume 17, Issue 4, 2021

Published on: 12 September, 2021

Page: [336 - 344] Pages: 9

DOI: 10.2174/1573396317666210913101027

Price: $65

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.

Keywords: Prader-Willi syndrome, dental care, oral findings, obesity, dental treatment, sleep apnea.

« Previous Next »
Graphical Abstract

[1]
Butler MG, Manzardo AM, Forster JL. Prader-willi syndrome: Clinical genetics and diagnostic aspects with treatment approaches. Curr Pediatr Rev 2016; 12(2): 136-66.
[http://dx.doi.org/10.2174/1573396312666151123115250] [PMID: 26592417]
[2]
Munné-Miralvés C, Brunet-Llobet L, Cahuana-Cárdenas A, Torné-Durán S, Miranda-Rius J, Rivera-Baró A. Oral disorders in children with Prader-Willi syndrome: a case control study. Orphanet J Rare Dis 2020; 15(1): 43-8.
[http://dx.doi.org/10.1186/s13023-020-1326-8] [PMID: 32041633]
[3]
Prader-Willi syndrome 2014.ghr.nlm.nih.gov/condition/prader-willi-syndrome
[4]
Bailleul-Forestier I, Verhaeghe V, Fryns JP, Vinckier F, Declerck D, Vogels A. The oro-dental phenotype in Prader-Willi syndrome: a survey of 15 patients. Int J Paediatr Dent 2008; 18(1): 40-7.
[PMID: 18086025]
[5]
Bantim YCV, Kussaba ST, de Carvalho GP, Garcia-Junior IR, Roman-Torres CVG. Oral health in patients with Prader-Willi syndrome: current perspectives. Clin Cosmet Investig Dent 2019; 11: 163-70.
[http://dx.doi.org/10.2147/CCIDE.S183981] [PMID: 31308759]
[6]
Hurren BJ, Flack NA. Prader-Willi Syndrome: A spectrum of anatomical and clinical features. Clin Anat 2016; 29(5): 590-605.
[http://dx.doi.org/10.1002/ca.22686] [PMID: 26749552]
[7]
Saeves R, Reseland JE, Kvam BM, Sandvik L, Nordgarden H. Saliva in Prader-Willi syndrome: quantitative and qualitative characteristics. Arch Oral Biol 2012; 57(10): 1335-41.
[http://dx.doi.org/10.1016/j.archoralbio.2012.05.003] [PMID: 22673752]
[8]
Saeves R, Klinge RF, Risnes S. Microscopic structure of dental hard tissues in primary and permanent teeth from individuals with Prader-Willi syndrome. Arch Oral Biol 2016; 66: 55-60.
[http://dx.doi.org/10.1016/j.archoralbio.2016.02.008] [PMID: 26913968]
[9]
Saeves R, Strøm F, Sandvik L, Nordgarden H. Gastro-oesophageal reflux - an important causative factor of severe tooth wear in Prader-Willi syndrome? Orphanet J Rare Dis 2018; 13(1): 64-3.
[http://dx.doi.org/10.1186/s13023-018-0809-3] [PMID: 29685165]
[10]
Saeves R, Nordgarden H, Storhaug K, Sandvik L, Espelid I. Salivary flow rate and oral findings in Prader-Willi syndrome: a case-control study. Int J Paediatr Dent 2012; 22(1): 27-36.
[http://dx.doi.org/10.1111/j.1365-263X.2011.01153.x] [PMID: 21702855]
[11]
Saeves R, Espelid I, Storhaug K, Sandvik L, Nordgarden H. Severe tooth wear in Prader-Willi syndrome. A case-control study. BMC Oral Health 2012; 12: 12-2.
[http://dx.doi.org/10.1186/1472-6831-12-12] [PMID: 22639910]
[12]
Olczak-Kowalczyk D, Korporowicz E, Gozdowski D, Lecka-Ambroziak A, Szalecki M. Oral findings in children and adolescents with Prader-Willi syndrome. Clin Oral Investig 2019; 23(3): 1331-9.
[http://dx.doi.org/10.1007/s00784-018-2559-y] [PMID: 30006686]
[13]
Setti JS, Pinto SF, Gaetti-Jardim EC, Manrique GR, Mendonça JC. Multidisciplinary care in the intensive care unit for a patient with Prader-Willi syndrome: a dental approach. Rev Bras Ter Intensiva 2012; 24(1): 106-10.
[http://dx.doi.org/10.1590/S0103-507X2012000100016] [PMID: 23917721]
[14]
Scardina GA, Fucà G, Messina P. Oral diseases in a patient affected with Prader-Willi syndrome. Eur J Paediatr Dent 2007; 8(2): 96-9.
[PMID: 17571934]
[15]
Olczak-Kowalczyk D, Witt A, Gozdowski D, Ginalska-Malinowska M. Oral mucosa in children with Prader-Willi syndrome. J Oral Pathol Med 2011; 40(10): 778-84.
[http://dx.doi.org/10.1111/j.1600-0714.2011.01034.x] [PMID: 21457346]
[16]
Yanagita M, Hirano H, Kobashi M, et al. Periodontal disease in a patient with Prader-Willi syndrome: a case report. J Med Case Reports 2011; 5: 329-9.
[http://dx.doi.org/10.1186/1752-1947-5-329] [PMID: 21798057]
[17]
Xiao KK, Tomur S, Beckerman R, Cassidy K, Lypka M. Orthognathic correction in prader-willi syndrome: Occlusion and sleep restored. Cleft Palate Craniofac J 2019; 56(3): 415-8.
[http://dx.doi.org/10.1177/1055665618775724] [PMID: 29750570]
[18]
Ishihara Y, Sugawara Y, Ei Hsu Hlaing E, et al. Orthodontic correction of severe Class II malocclusion in a patient with Prader-Willi syndrome. Am J Orthod Dentofacial Orthop 2018; 154(5): 718-32.
[http://dx.doi.org/10.1016/j.ajodo.2017.05.040] [PMID: 30384943]
[19]
Driscoll DJ, Miller JL, Schwartz S, Cassidy SB. Prader-Willi Syndrome. Prader-Willi Syndrome. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Mirzaa G, Amemiya A, Eds. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2021.
[20]
Dental management of patients receiving oral bisphosphonate therapy: expert panel recommendations. J Am Dent Assoc 2006; 137(8): 1144-50.
[http://dx.doi.org/10.14219/jada.archive.2006.0355] [PMID: 16873332]
[21]
Ruggiero SL, Dodson TB, Fantasia J, et al. American Association of Oral and Maxillofacial Surgeons position paper on medication-related osteonecrosis of the jaw--2014 update. J Oral Maxillofac Surg 2014; 72(10): 1938-56.
[http://dx.doi.org/10.1016/j.joms.2014.04.031] [PMID: 25234529]
[22]
Famelart N, Diene G, Çabal-Berthoumieu S, et al. Equivocal expression of emotions in children with Prader-Willi syndrome: what are the consequences for emotional abilities and social adjustment? Orphanet J Rare Dis 2020; 15(1): 55-9.
[http://dx.doi.org/10.1186/s13023-020-1333-9] [PMID: 32085791]
[23]
Rice LJ, Einfeld SL. Cognitive and behavioural aspects of Prader-Willi syndrome. Curr Opin Psychiatry 2015; 28(2): 102-6.
[http://dx.doi.org/10.1097/YCO.0000000000000135] [PMID: 25599341]
[24]
Definition of dental home. Pediatr Dent 2018; 40(6): 12.
[PMID: 32074832]
[25]
Meyer SL, Splaingard M, Repaske DR, Zipf W, Atkins J, Jatana K. Outcomes of adenotonsillectomy in patients with Prader-Willi syndrome. Arch Otolaryngol Head Neck Surg 2012; 138(11): 1047-51.
[http://dx.doi.org/10.1001/2013.jamaoto.64] [PMID: 23165379]
[26]
Lewis BAFL, Heeger S, Cassidy SB. Speech and Language Skills of Individuals With Prader-Willi Syndrome. Am J Speech Lang Pathol 2002; 11: 285-94.
[http://dx.doi.org/10.1044/1058-0360(2002/033)]
[27]
Lalla E, Cheng B, Lal S, et al. Periodontal changes in children and adolescents with diabetes: a case-control study. Diabetes Care 2006; 29(2): 295-9.
[http://dx.doi.org/10.2337/diacare.29.02.06.dc05-1355] [PMID: 16443876]
[28]
Moore J, Csikar J, Kang J, Tugnait A, Campbell F, Clerehugh V. Awareness, practices, training, and confidence of Paediatric Diabetes Care Teams in relation to periodontitis. Pediatr Diabetes 2020; 21(2): 384-9.
[http://dx.doi.org/10.1111/pedi.12966] [PMID: 31854476]
[29]
Jain A, Bala I, Makkar JK. Anesthetic management of Prader-Willi syndrome: what if neuromuscular relaxants could not be avoided? J Anesth 2012; 26(2): 304-5.
[http://dx.doi.org/10.1007/s00540-011-1304-3] [PMID: 22198218]
[30]
Roman-Torres CVG, Kussaba ST, Bantim YCV, de Oliveira RBAA. Special care dentistry in a patient with Prader-Willi syndrome through the use of atraumatic restorative treatment under general anesthesia. Case Rep Dent 2017; 2017: 7075328.
[http://dx.doi.org/10.1155/2017/7075328] [PMID: 29318056]
[31]
Definition of dental home. Pediatric Dentistry 2018; 40(6): 12.

Rights & Permissions Print Cite
Article Metrics
13
1
© 2024 Bentham Science Publishers | Privacy Policy