Dental considerations and management in Noonan Syndrome: A case report with review of literature

Nipun Jain1Orcid, Priyanka Venugopal2Orcid, Arun Mamachan Xavier3Orcid, Parvathy Kumaran4Orcid, Balagopal R Varma5Orcid, Suresh Kumar J6Orcid

Highlights

Noonan syndrome is a relatively common genetic disorder seen in dental practise.
Not all dental characteristic features are seen at a given time.
Children with Noonan syndrome are generally uncooperative in dental chair and owing to their systemic complications; dental management is mostly carried out under general anaesthesia.

Abstract

Noonan syndrome is a genetic disorder of autosomal dominance with an estimated prevalence of 1:1000 – 1:2500 live birth. The typical features include short stature, cardiovascular abnormalities and characteristics facial deformity. Dental features reported so far include malocclusion, dental caries, giant cell and cystic lesion. Multidisciplinary treatment plays a key role in the overall quality of life of the patient. This case report describes a 6-year-old boy with Noonan syndrome.

Keywords: Cardiovascular Abnormalities; Comprehensive Dental Care; Noonan Syndrome

Author Affiliations

  1. Post Graduate Student, Department of Pediatric and Preventive Dentistry, Amrita School of Dentistry, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India
  2. Post Graduate Student, Department of Pediatric and Preventive Dentistry, Amrita School of Dentistry, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India
  3. Reader, Department of Pediatric and Preventive Dentistry, Amrita School of Dentistry, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India (✉ Correspondence: arunmamachan@yahoo.co.in)
  4. Professor, Department of Pediatric and Preventive Dentistry, Amrita School of Dentistry, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India
  5. Professor, Department of Pediatric and Preventive Dentistry, Amrita School of Dentistry, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India
  6. Professor, Department of Pediatric and Preventive Dentistry, Amrita School of Dentistry, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India
  1. Noonan JA. Noonan Syndrome: An Update and Review for the Primary Pediatrician. Clin Pediatr (Phila) 1994;33:548–555
  2. Alhassoun R. Clinical manifestation and dental management of Noonan Syndrome. Int Med 2020;2:260
  3. Mallineni SK, Yiu CKY, King NM. Oral manifestations of Noonan syndrome: Review of the literature and a report of four cases. Rom J Morphol Embryol 2014;55:1503–1509
  4. Lutz JC, Nicot R, Schlund M, Schaefer E, Bornert F, Fioretti F, et al. Dental and maxillofacial features of Noonan Syndrome: Case series of ten patients. J Cranio-Maxillofacial Surg 2020;48:242–250
  5. Tartaglia M, Gel ZM. Noonan syndrome and clinically related disorders. Best Pract Res Clin Endocrinol Metab 2011;25:161–179
  6. Hwang I, Lee Y, Sim D, Mah Y. Oral Features in a Child with Noonan Syndrome : A Case Report. J Korean Acad Pedtatric Dent 2018;45:115–122
  7. Anthonappa RP, King NM. Oral and dental manifestations in noonan syndrome. In: Amrit Bhangoo, editors. Noonan Syndrome: Characteristics and Interventions. Academic Press; 2019. p.135–158
  8. WHO Child Growth Standards. Dev Med Child Neurol 2009;51:1002–1002
  9. Malaquias, A. C., & Jorge, AAL. Growth Failure and Experience With Growth Hormone Therapy in Noonan Syndrome. In: Amrit Bhangoo, editor. Noonan Syndrome: Characteristics and Interventions. Academic Press; 2019. p.15–29
  10. Van der Burgt I, Berends E, Lommen E, Van Beersum S, Hamel B, Mariman E. Clinical and molecular studies in a large Dutch family with Noonan syndrome. Am J Med Genet 1994;53:187–191
  11. Athota, JP, Bhat, M, Nampoothiri, S, Gowrishankar K, Narayanachar SG, Puttamallesh V, et al. Molecular and clinical studies in 107 Noonan syndrome affected individuals with PTPN11 mutations. BMC Med Genet 2020;21:50
  12. Kouz K, Lissewski C, Spranger S, Mitter D, Riess A, Lopez-Gonzalez V et al. Genotype and phenotype in patients with Noonan syndrome and a RIT1 mutation. Genet Med 2016;18:1226-1234
  13. Romano AA, Allanson JE, Dahlgren J, Gelb BD, Hall B, Pierpont ME, et al. Noonan syndrome: Clinical features, diagnosis, and management guidelines. Pediatrics 2010;126:746–59
  14. Cao H, Alrejaye N, Klein OD, Goodwin AF, Oberoi S. A review of craniofacial and dental findings of the RASopathies. Orthod Craniofacial Res 2017;20:32–8
  15. Baird PA, De Jong BP. Noonan’s syndrome (XX and XY Turner phenotype) in three generations of a family. J Pediatr 1972;80:110-114
  16. Barberia Leache E, Saavedra Ontiveros D, Maroto Edo M. Etiopathogenic analysis of the caries on three patients with Noonan Syndrome. Med Oral 2003;8:136–142
  17. Sharma PR, MacFadyen UM, Fung DE. Dental management of a child patient with Noonan’s syndrome. Dent Update 2007;34(2):117–118, 120
  18. Ortega Ade O, Guaré Rde O, Kawaji NS, Ciamponi AL. Orofacial aspects in Noonan syndrome: 2 case report. J Dent Child (Chic) 2008;75:85–90
  19. Sahebjamee M, Ameri NG, Farhud DD. First report of new oral findings in a case with Noonan syndrome. Iranian J Publ Health 2008;37:131–137
  20. Emral ME, Akcam MO. Noonan syndrome: a case report. J Oral Sci 2009;51:301–306.
  21. Ierardo G, Luzzi V, Panetta F, Sfasciotti GL, Polimeni A. Noonan syndrome: a case report. Eur J Paediatr Dent 2010;11:97–100
  22. Bufalino A, Carrera M, Carlos R, Coletta RD. Giant cell lesions in Noonan syndrome: case report and review of the literature. Head Neck Pathol 2010;4:174–177
  23. Sugar AW, Ezsias A, Bloom AL, Morcos WE. Orthognathic surgery in a patient with Noonan’s syndrome. J Oral Maxillofac Surg 1994;52:421-425
  24. Nirmal T, Muthu MS, Arranganal P. Noonan syndrome: a case report. J Indian Soc Pedod Prev Dent 2001;19:77–79
  25. Okada M, Sasaki N, Kaihara Y, Okada R, Amano H, Miura K, Kozai K. Oral findings in Noonan syndrome: report of a case. J Oral Sci 2003;45:117–121
  26. Asokan S, Muthu MS, Rathna Prabhu V. Noonan syndrome: a case report. J Indian Soc Pedod Prev Dent 2007;25:144–147
  27. Morice A, Harroche A, Cairet P, Khonsari RH: Preoperative detailed coagulation test are required in patients with Noonan syndrome. J Oral Maxillofac Surg 2017;76: 1553-1558.
  28. Cancino CMH, Gaião L, Sant’Ana Filho M, Oliveira FAM, Giant cell lesions with a Noonan like phenotype: a case report, J Contemp Dent Pract, 2007, 8(4):67–73.
  29. Aggarwal V, Malik V, Kapoor PM, Kiran U. Noonan syndrome: An anesthesiologist’s perspective. Ann Card Anaesth 2011;14:214-217
  30. Shah, S. Analgesia, Anesthesia, and Perioperative Considerations in Noonan Syndrome. Noonan Syndrome. In: Amrit Bhangoo, editors. Noonan Syndrome: Characteristics and Interventions. Academic Press; 2019. p.123–134
  31. Bajwa SJS, Gupta S, Kaur J, Panda A, Bajwa SK, Singh A, et al. Anesthetic considerations and difficult airway management in a case of Noonan syndrome. Saudi J Anaesth 2011;5:345–347

Article Info

Contemp Pediatr Dent 2021:2(2):126-133

Received: 13 May 2021

Accepted: 15 June 2021

Online First: 23 June 2021

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					Nipun Jain, Priyanka Venugopal, Arun Mamachan Xavier, Parvathy Kumaran, Balagopal R Varma, Suresh Kumar J. Dental considerations and management in Noonan Syndrome: A case report with review of literature. Contemp Pediatr Dent 2021:2(2):126-133.
				
			

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