MULTI-DISCIPLINARY APPROACH IN THE MANAGEMENT OF CHRIST-SIEMENS-TOURAINE SYNDROME WITH ANODONTIA: A CASE REPORT OF TWO SIBLINGS

Authors

  • Manesh Lahori Principal, Dean, Dr., Director and Head Of the Department, Department of Prosthodontics & Crown and Bridge, Kanti Devi Dental College and Hospital, Uttar Pradesh, India
  • Sonal Gupta Head of the Department, Dr., Department of Pediatrics and Preventive Dentistry, Kanti Devi Dental College and Hospital, Uttar Pradesh, India
  • Menia Gumro Post Graduate, Dr., Department of Pediatrics and Preventive Dentistry, Kanti Devi Dental College and Hospital, Uttar Pradesh, India
  • Utkarsh Singh Post Graduate Student, Dr., Department of Pediatrics and Preventive Dentistry, Kanti Devi Dental College and Hospital, Uttar Pradesh, India
  • Nivea Verma Post Graduate, Dr., Department of Prosthodontics and Crown & Bridge, Kanti Devi Dental College and Hospital, Uttar Pradesh, India
  • Mehr Yazdani Post Graduate Student, Dr., Department of Pediatrics and Preventive Dentistry, Kanti Devi Dental College and Hospital, Uttar Pradesh, India

DOI:

https://doi.org/10.46827/ejse.v10i4.5454

Keywords:

Christ-Siemens-Touraine syndrome, anodontia, Hereditary Ectodermal Dysplasia (HED), multidisciplinary approach

Abstract

Aim: The purpose of the case report was to highlight the clinical features and multidisciplinary approach to managing children suffering from Christ-Siemens-Touraine syndrome with complete anodontia and its prosthetic rehabilitation. Background: Christ-Siemens-Touraine syndrome, which is a recessive autosomal disorder, is the most common form of Heredity Ectodermal dysplasia in which two or more ectodermally derived anatomic structures fail to develop, thus leading to hypoplasia or aplasia of structures such as skin, hair, nails, teeth, nerve cells, sweat glands, parts of the eye and ear and other organs. Case report: A 7-year-old boy and his 6-year-old younger sibling were referred to the Department of Pediatric & Preventive Dentistry with the chief complaint of missing teeth in the whole mouth and difficulty in speaking and chewing. After thorough examination and evaluation, in order to improve appearance, mastication, and speech, removable complete maxillary and mandibular dentures were planned to be the best treatment choice.

 

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Author Biographies

  • Sonal Gupta, Head of the Department, Dr., Department of Pediatrics and Preventive Dentistry, Kanti Devi Dental College and Hospital, Uttar Pradesh, India

    Dr. Sonal Gupta is a professor and Head of the Department at KD Dental College, Mathura, India. She has published more than 100 articles in national and international journal and she’s a national level speaker as well.

  • Utkarsh Singh, Post Graduate Student, Dr., Department of Pediatrics and Preventive Dentistry, Kanti Devi Dental College and Hospital, Uttar Pradesh, India
    Dr. Utkarsh Singh is a post graduate student at KD Dental College, Mathura, India

References

Ville B, Damm D, Allen C , Bouquot J. Oral and Maxillo-facial Pathology, 3rd ed. Philadelphia: W.B. Saunders; 2008.

Pinheiro M, Freire-Maia N. Ectodermal dysplasias: A clinical classification and casual review. Am J Med Genet 1994;53: 153-162.

Gardel P, Mercier C, Molhant G. Christ-Siemens-Touraine syndrome. A new case. Rev Stomatol Chir Maxillofac 1984;85: 115-8.

Daniel E, McCurdy EA, Shashi V, et al. Ectodermal dysplasia: otolaryngologic manifestations and management. Laryngoscope 2002;112: 962-7.

Merkx MA, Arnold WP. Ectodermal dysplasia: a heterogenic deviation. Ned Tijdschr Tandheelkd. 1995;102: 334-6.

Imirzalioglu P, Uckan S, Haydar SG. Surgical and prosthodontic treatment alternatives for children and adolescents with ectodermal dysplasia: a clinical report. J Prosthet Dent 2002;88: 569-572.

Bergendal B. Prosthetic habilitation of a young patient with hypohydrotic ectodermal dysplasia and oligodontia: a case report of 20 years treatment. Int J Prosthodont 2001;14: 471-479

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

Balci G, Baskan SZ, Akdenizi S. Ectodermal dysplasia: report of four cases and review of literature. Int Dent Med Disord 2008;1:56–59.

Adıgüzel O, Kaya S, Yavuz İ, Atakul F. Oral findings of ectodermal dysplasia and literature review. Int Dent Med Disorders 2008;1(1):43-49.

Tarjan I, Gabris K, Rozsa N. Early prosthetic treatment of patients with ectodermal dysplasia: a clinical report. J Pros-thet Dent 2005;93:419-24.

Imirzalioglu P, Uckan S, Haydar SG. Surgical and prostho-dontic treatment alternatives for children and adolescents with ectodermal dysplasia: a clinical report. J Prosthet Dent 2002;88:569-72.

Till MJ, Marques AP. Ectodermal dysplasia: treatment considerations and case reports. Northwest Dent 1992;71(3):25-28.

Huang SX, Liang JL, Sui WG, et al. EDA mutation as a cause of hypohidrotic ectodermal dysplasia: a case report and review of the literature. Genet Mol Res 2015;14(4):44–51.

Kumar P R, Srivatsa. G, Kashinath K R. prosthodontic management of ectodermald dysplasia –A case report. Indian J compre dent care. 2011;1(1):86-94.

Vergo TJ Jr. Prosthodontics for pediatric patients with congenital/developmental orofacial anomalies: a long-term follow-up. J Prosthet Dent. 2001;86(4):342–349.

Manuja N1, Passi S, Pandit IK, Singh N. Management of a case of ectodermal dysplasia: a multidisciplinary approach. J Dent Child (Chic). 2011;78(2):107-117.

Shaw RM. Prosthetic management of hypohidrotic ectoder-mal dysplasia with anodontia. Case report. Aust Dent J 1990;35:113-6.

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Published

2024-06-28

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