Journal of South Asian Association of Pediatric Dentistry

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VOLUME 5 , ISSUE 2 ( May-August, 2022 ) > List of Articles

CASE REPORT

Dental Management of Prader-Willi Syndrome in a 7-year-old Girl: A Rare Case Report

Mallayya C Hiremath, Srinath Krishnappa, Sahana Srinath, Raja Jayadeva Nayak, Aarcha S Kumar

Keywords : Dental management, Genetic, Pyogenic granuloma, Syndrome

Citation Information : Hiremath MC, Krishnappa S, Srinath S, Nayak RJ, Kumar AS. Dental Management of Prader-Willi Syndrome in a 7-year-old Girl: A Rare Case Report. J South Asian Assoc Pediatr Dent 2022; 5 (2):96-100.

DOI: 10.5005/jp-journals-10077-3231

License: CC BY-NC 4.0

Published Online: 23-08-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Abstract

Prader-Willi syndrome (PWS) is a rare genetic disorder characterized by a lack of expression of paternal genes located on chromosome 15q11-q13. Prader-Willi syndrome is characterized by hypothalamic dysfunction. This anomaly causes cognitive, neurological, and endocrine abnormalities, among which hyperphagia is the important feature. Prader-Willi syndrome is of interest to the dentist because PWS is associated with atrophy of salivary glands leading to decreased salivary secretion and increased risk for dental caries and gingivitis. The present case report describes a clinical case of a 7-year-old girl with PWS. The case presented with multiple carious teeth, partial ankyloglossia, and pyogenic granuloma of gingiva. Restorations were done for carious teeth and 64, 83 were extracted under local anesthesia, and an excisional biopsy of the gingival lesion was done. Periodic dental visits and early interventions are needed to improve the oral health of children with PWS.


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  1. Elena G, Bruna C, Benedetta M, et al. Prader-Willi syndrome: clinical aspects. J Obes 2012; 473941. DOI: 10.1155/2012/473941
  2. Gajic M, Stevanovic R. Monograph: Handicapped Child in a Dental Office. Faculty of Dentistry, University of Belgrade; 2002.
  3. Tayal E, Indushekar KR, Saraf BG, et al. Prader-Willi syndrome: a case report. Public Health Open J 2016;1(1):12–15. DOI: 10.17140/PHOJ-1-104
  4. Trizno AA, Jones AS, Carry PM, et al. The prevalence and treatment of hip dysplasia in Prader-Willi syndrome (PWS). J Pediatr Orthop 2018;38(3):e151–e156. DOI: 10.1097/BPO.0000000000001118
  5. Umapathy T, Premkishore K, Kathariya MD, et al. Oral and general findings: management of Prader-Willi syndrome. J Indian Acad Oral Med Radiol 2013;25(1):43–47. DOI: 10.5005/jp-journals-10011-1335
  6. Bantim YCV, Kussaba ST, de Carvalho GP, et al. Oral health in patients with Prader-Willi syndrome: current perspectives. Clin Cosmet Investig Dent 2019;11:163–170. DOI: 10.2147/CCIDE.S183981
  7. Bailleul-Forestier I, Verhaeghe V, Fryns JP, et al. The oro-dental phenotype in Prader-Willi syndrome: a survey of 15 patients. Int J Paediatr Dent 2008;18(1):40–47. DOI: 10.1111/j.1365-263X.2007.00857.x
  8. Salako NO, Ghafouri HM. Oral findings in a child with Prader-Labhart-Willi syndrome. Quintessence Int 1995;26(5):339–341.
  9. Bazopoulou-Kyrkanidou E, Papagiannoulis L. Prader-Willi syndrome: report of a case with special emphasis on oral problems. J Clin Pediatr Dent 1992;17(1):37–40.
  10. Munne-Miralves C, Brunet-Llobet L, Cahuana-Cárdenas A., et al. Oral disorders in children with Prader-Willi syndrome: a case-control study. Orphanet J Rare Dis 2020;15(1):43–50. DOI: 10.1186/s13023-020-1326-8
  11. Goldstone AP, Holland AJ, Hauffa BP, et al. Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab 2008;93(11):4183–4197. DOI: 10.1210/jc.2008-0649
  12. Kamal R, Dahiya P, Puri A. Oral pyogenic granuloma: various concepts of etiopathogenesis. J Oral Maxillofac Pathol 2012;16(1):79–82. DOI: 10.4103/0973-029X.92978
  13. Jafarzadeh H, Sanatkhani M, Mohtasham N. Oral pyogenic granuloma: a review. J Oral Sci 2006;48(4):167–175. DOI: 10.2334/josnusd.48.167
  14. Yangita M, Hirano H, Kobashi M, et al. Periodontal disease in a patient with Prader-Willi syndrome: a case report. J Med Case Rep 2011;5(1):329–334. DOI: 10.1186/1752-1947-5-329
  15. El-Bassyouni HT, Hassan N, Mahfouz I, et al. Early detection and management of Prader-Willi syndrome in Egyptian patients. J Pediatr Genet 2019;8(4):179–186. DOI: 10.1055/s-0039-1695042
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