International Journal of Clinical Pediatric Dentistry

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VOLUME 12 , ISSUE 6 ( November-December, 2019 ) > List of Articles


Prosthodontic Management of a Pediatric Patient with Christ-Siemens-Touraine Syndrome: A Case Report

Anshad M Abdulla, Abdulrahman Y Almaliki, Nasim V Shakeela, Zuhair Alkahtani, Majed AS Alqahtani, Shan Sainudeen, Shaheen V Shamsuddin

Citation Information : Abdulla AM, Almaliki AY, Shakeela NV, Alkahtani Z, Alqahtani MA, Sainudeen S, Shamsuddin SV. Prosthodontic Management of a Pediatric Patient with Christ-Siemens-Touraine Syndrome: A Case Report. Int J Clin Pediatr Dent 2019; 12 (6):569-572.

DOI: 10.5005/jp-journals-10005-1697

License: CC BY-NC 4.0

Published Online: 25-11-2020

Copyright Statement:  Copyright © 2019; The Author(s).


Ectodermal dysplasias (ED) are a group of rare genetic disorders characterized by congenital defects involving two or more ectodermal structures. Christ-Siemens-Touraine syndrome or hypohidrotic ectodermal dysplasia is the commonest form of ED. Hypohidrotic ectodermal dysplasia (HED) is an X-linked disorder characterized by excessively dry skin due to the absence of sweat glands (anhidrosis), sparse body hair especially on the scalp and eyebrows (hypotrichosis), brittle nails, absence of sebaceous glands (asteatosis) and malformed or absent teeth. Oral manifestations include oligodontia or complete anodontia, conical teeth, underdeveloped alveolar ridges, generalized spacing and delayed eruption of permanent teeth. This case report discusses a classical case of HED and the options for rehabilitation in a growing patient. A thorough knowledge about the clinical manifestations of ED will lead to proper diagnosis and appropriate treatment plan thereby leading to significant improvements in esthetics, phonetics and masticatory function in ED patients, which in turn leads to improved quality of life in these individuals.

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  1. Kothiwala SK, Prajapat M, Kuldeep CM. Christ–Siemens–Touraine syndrome with palmoplantar keratoderma: a rare association. Indian Dermatol Online J 2016;7(5):393–395. DOI: 10.4103/2229-5178. 190506.
  2. Thurnam J. Two cases in which the skin, hair and teeth were very imperfectly developed. Proc R Med Chir Soc 1848;31:71–82. DOI: 10.1177/095952874803100108.
  3. Ladda R, Gangadhar S, Kasat V, et al. Prosthodontic management of hypohidrotic ectodermal dysplasia with anodontia: a case report in pediatric patient and review of literature. Ann Med Health Sci Res 2013;3(2):277–281. DOI: 10.4103/2141-9248.113679.
  4. Lesot H, Clauss F, Manière MC, et al. Consequences of X-linked hypohidrotic ectodermal dysplasia for the human jaw bone. Front Oral Biol 2009;13:93–99. DOI: 10.1159/000242398.
  5. Coelho LG Jr, Caldas AF Jr, Soriano EP, et al. Christ siemens touraine syndrome: a case report. Cases J 2009;2(1):38. DOI: 10.1186/1757-1626-2-38.
  6. Mokhtari S, Mokhtari S, Lotfi A. Christ-Siemens-Touraine syndrome: a case report and review of the literature. Case Rep Dent 2012; 586418. DOI: 10.1155/2012/586418.
  7. Retnakumari N, Varghese M, Kannan VP. Christ siemens touraine syndrome: a rare case report. J Indian Soc Pedod Prev Dent 2016;34(2):185–188. DOI: 10.4103/0970-4388.180451.
  8. Gupta S, Tyagi P. Prosthodontic management of anhidrotic ectodermal dysplasia. Indian J Dent Res 2011;22(2):348–351. DOI: 10.4103/0970-9290.84291.
  9. Shigli A, Airen SP. Prosthodontic management of patients with Christ–Siemens–Touraine syndrome. BMJ Case Rep 2012;2012:bcr1120115226. DOI: 10.1136/bcr.11.2011.5226.
  10. Bidra AS, Martin JW, Feldman E. Complete denture prosthodontics in children with ectodermal dysplasia: review of principles and techniques. Compend Contin Educ Dent 2010;31(6):426–433.
  11. Balci G, Baskan SZ, Akdenizi S. Ectodermal dysplasia: report of four cases and review of literature. Int Dent Med Disord 2008;1:56–59.
  12. Lexner MO, Bardow A, Hertz JM, et al. Anomalies of tooth formation in hypohidrotic ectodermal dysplasia. Int J Paediatr Dent 2007;17(1): 10–18. DOI: 10.1111/j.1365-263X.2006.00801.x.
  13. Jain N, Naitam D, Wadkar A, et al. Prosthodontic rehabilitation of hereditary ectodermal dysplasia in an 11-year-old patient with flexible denture: a case report. Case Rep Dent 2012;2012:489769. DOI: 10.1155/2012/489769.
  14. Hickey AJ, Vergo TJ. Prosthetic treatments for patients with ectodermal dysplasia. J Prosthet Dent 2001;86(4):364–368. DOI: 10.1067/mpr.2001.118876.
  15. Pigno MA, Blackman RB, Cronin RJ, et al. Prosthodontic management of ectodermal dysplasia: a review of the literature. J Prosthet Dent 1996;76:541–545. DOI: 10.1016/S0022-3913(96)90015-3.
  16. Yap AK, Klineberg I. Dental implants in patients with ectodermal dysplasia and tooth agenesis: a critical review of the literature. Int J Prosthodont 2009;22(3):268–276.
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