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CASE REPORT
Year : 2008  |  Volume : 26  |  Issue : 1  |  Page : 22-25
 

Witkop's tooth and nail syndrome: A multifaceted approach to dental management


Department of Pedodontics and Preventive Dentistry, The Oxford Dental College, Hospital and Research Center, Hosur Road, Bangalore, Karnataka, India

Correspondence Address:
R Neeraja
No-12/4, Yamunabhai Road, Maadhav Nagar, Bangalore - 560 001, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-4388.40317

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   Abstract 

Witkop's tooth and nail syndrome is a rare autosomal dominant disorder of ectodermal dysplasia characterized by hypodontia and nail dysplasia. Mutations in MSX-1 have been shown to be associated with this syndrome. There is failure of development and eruption of the dentition. Tooth shape may vary; the most common forms are conical and narrow crowns. The nails may be spoon shaped and slow growing and affect both finger and toe nails. The nail involvement is more severe in childhood. The present case describes a 14-year-old boy who showed the characteristic features of Witkop's syndrome. A multifaceted approach to the dental management of the patient is discussed.


Keywords: Dental management, tooth and nail dysplasia, Witkop′s syndrome


How to cite this article:
Subramaniam P, Neeraja R. Witkop's tooth and nail syndrome: A multifaceted approach to dental management. J Indian Soc Pedod Prev Dent 2008;26:22-5

How to cite this URL:
Subramaniam P, Neeraja R. Witkop's tooth and nail syndrome: A multifaceted approach to dental management. J Indian Soc Pedod Prev Dent [serial online] 2008 [cited 2020 Sep 24];26:22-5. Available from: http://www.jisppd.com/text.asp?2008/26/1/22/40317



   Introduction Top


Ectodermal dysplasia (ED) is the term used to describe a group of rare inherited disorders characterized by dysplasia of tissues of ectodermal origin. [1]

One among these is the 'tooth and nail syndrome' (TNS), which was first described by Witkop in 1965. [2] It is a rare autosomal dominant disorder characterized by hypodontia and morphological changes in teeth, along with dysgenesis of nails. The nail defects are however alleviated with age and may not be easily detectable during adulthood. [3] There is hypodontia and, infrequently, anodontia of the permanent teeth [4] ; seldom are more than 20 permanent teeth missing,

The congenital absence of teeth can seriously disable the young patient physically and emotionally, especially during adolescence. Retention of primary teeth in the absence of permanent successors is of significance since they help retain the height of the alveolar bone in an area which may be a site for further comprehensive therapy.

This case report describes TNS in a 14-year-old boy and the multifaceted treatment rendered.


   Case Report Top


A 14-year-old male patient reported to our institution with the chief complaints of having small teeth, excessive spacing between his upper front teeth, and difficulty in chewing.

The patient did not complain of intolerance to heat and had normal perspiration. However, he gave a history of slow growth of his toe nails, which also frequently tended to fracture.

The patient was moderately built and nourished. A general examination revealed normal hair (both in growth and texture) [Figure - 1] and skin. He had dysplastic toe nails; no abnormalities were detected in the finger nails [Figure - 2] a and b.

On intraoral examination, 16 retained deciduous and 4 permanent teeth (maxillary first permanent molars and two incisors) were present. All other permanent teeth were missing clinically.

The permanent maxillary molars were rudimentary and the maxillary incisors had long, narrow, tapering crowns, with excessive midline diastema. There was anterior deep bite and an open bite in the posterior region, with loss of the vertical dimension [Figure - 3].

Orthopantamogram revealed multiple missing permanent tooth buds, viz, 12, 13, 14, 15, 17, 18, 22, 23, 24, 25, 27, and 28 in the maxillary arch and total absence of all the permanent tooth buds in the mandibular arch. There was resorption of the roots to more than half their lengths with respect to 64, 71 74, 81, and 84 [Figure - 4].

Treatment

In order to provide esthetics and optimum function, a multifaceted approach was planned, keeping in mind the needs and limitations of the adolescent patient.

Extractions of 64, 71, 74, 81, and 84 were done in view of their short resorbed roots. Fixed partial dentures were planned for both arches to restore the remaining edentulous spaces. All four second primary molars were restored with stainless steel crowns to establish posterior occlusion [Figure - 5].

In order to give a fixed partial denture for the upper arch, the midline diastema was first closed with a simple fixed orthodontic appliance [Figure - 6],[Figure - 7].

In the maxillary arch, 53 and 63 were endodontically treated and restored after post and core build-up, so as to serve as abutments in addition to 11, 21, and 54. To facilitate transverse jaw growth, two independent fixed prosthetic units were given in the upper arch [Figure - 8],[Figure - 9],[Figure - 12].

Similarly, in the mandibular arch 72, 73, 82, and 83 were endodontically treated and restored following post and core build-up. These teeth were also used as abutments for a fixed partial denture extending from the premolar region on one side to the premolar region on the other side [Figure - 10],[Figure - 11],[Figure - 12].


   Discussion Top


The most common missing teeth in Witkop's syndrome are the maxillary incisors, canines, and the second molars. [2] Defects in primary teeth are so mild that no abnormality is noted until the permanent teeth fail to erupt. Such delayed eruption of permanent teeth has also been reported by Redpath and Winter [5] in cases of ectodermal dysplasia.

In the present case, the patient possessed conical incisors with narrow, tapering crowns. Although it is rare to find more than 20 teeth missing, this patient was unusual in that he had 28 congenitally missing permanent tooth buds.

There is no general consensus on the dental management of Witkop's TNS, nevertheless it is evident that any treatment given should keep in view the growth potential of the patient. [6]

Our approach to the management of this patient was largely governed by his age and the necessity to ensure conservation of his primary dentition. The focus of our treatment was to restore the functional needs of the patient. Improvement of the patient's facial esthetics and masticatory function was important in allowing him to integrate with others of his age, to prevent any psychological repercussions and to maintain his self-esteem.

The unesthetic excessive midline diastema was corrected by simple mechanics as reported in an earlier study. [7] Since there was absence of permanent successors, the retained primary teeth had to be salvaged for use as abutments in the fixed prosthesis. This was appropriate, considering the age of the patient, as it is preferable to wait for growth completion prior to placement of oral implants.

To facilitate transverse facial growth, the fixed prosthesis in the maxillary arch did not cross the midline. A similar case report was presented by Hogeboom who demonstrated the occurrence of jaw growth in an individual with ectodermal dysplasia; in this case, two segments of a detachable fixed prosthesis were separated at the midline to facilitate jaw growth. [8]

Treating a patient of tooth and nail dysplasia, requires a thorough knowledge of growth and development, and long-term follow-up is necessary for the modification and/or replacement of the prosthesis. Adjustments related to fit of prosthesis and occlusion must be monitored periodically at regular intervals due to jaw growth. When growth is stabilized, osseointegrated implants may be an alternative aid to support, stabilize, and retain the prosthesis. [9]

The patient was satisfied with his improved appearance and masticatory ability [Figure - 13],[Figure - 14]. Presently, he is on periodic evaluation and we plan to provide a more definitive treatment after growth completion.

 
   References Top

1.Dhanrajani P J, Jiffiy AO. Management of ectodermal dysplasia: A literature review. Dent Update 1998;25:73-5.  Back to cited text no. 1    
2.Witkop CJ Jr. Genetic diseases of the oral cavity. In: Tiecke RW, editor. Oral Pathology. New York: McGraw-Hill; 1965.  Back to cited text no. 2    
3.Jumlongras D, Bei M, Stimson JM, Wang W, Depalma SR, Seidman CE, et al . A nonsense mutation in MSX1 causes Witkop syndrome. Am J Hum Genet 2001;69:67-74.  Back to cited text no. 3    
4.Hodges SA, Harley KE. Witkop tooth and nail syndrome: Report of two cases in a family. Int J Pediatr Dent 1999;9:207-12.  Back to cited text no. 4    
5.Wicomb GM, Stephen LX, Beighton P. Dental implications of tooth-nail dysplasia (Witkop syndrome): A report of an affected family and an approach to dental management. J Clin Pediatr Dent 2004;28:107-12.  Back to cited text no. 5  [PUBMED]  
6.Redpath TH, Winter GB. Autosomal dominant ectodermal dysplasia with significant dental defects. Br Dent J 1969;126:123-8.  Back to cited text no. 6  [PUBMED]  
7.Chitty LS, Dennis N, Baraitser M. Hidrotic ectodermal dysplasia of hair, teeth and nails: Case reports and review. J Med Genet 1996;33:707-10.  Back to cited text no. 7  [PUBMED]  
8.Munshi A, Munshi AK. Midline space closure in the mixed dentition: A case report. J Indian Soc Pedo Prev Dent 2001;19:57-60.  Back to cited text no. 8    
9.Piegno MA, Blackman RB, Cronin RJ, Cavazos E. Prosthodontic management of Ectodermal dysplasia: A review of its literature. J Prosthet Dent 1996;76:541-5.  Back to cited text no. 9    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9], [Figure - 10], [Figure - 11], [Figure - 12], [Figure - 13], [Figure - 14]


This article has been cited by
1 Witkop Tooth and Nail Syndrome: A Report of Three Cases in a Family : Witkop Tooth and Nail Syndrome
Mahtab Memarpour, Fereshteh Shafiei
Pediatric Dermatology. 2010; : no
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