ECTRODACTYLY-ECTODERMAL DYSPLASIA-CLEFTING SYNDROME PDF

You can help by adding to it. March Research[ edit ] Current research regarding EEC syndrome is focused on the genetic components contributing to the presented traits found in patients with EEC. A normal human karyotype includes 22 pairs of autosomal or non-sex chromosomes and one pair of sex chromosomes, constituting a total of 46 chromosomes. During reproduction , each parent contributes 23 chromosomes; 22 autosomal chromosomes and one sex chromosome. Because the gene is dominant, only one parent must contribute the abnormal gene for the child to inherit the disease and the contributing parent will usually have the disease, due to the expression of the dominant gene in the parent. Though we can calculate the chance of inheritance of the gene, the degree of expression cannot be calculated.

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Figure 8 Photograph of the patient after 2 years follow-up. Discussion There are two clinical forms in which EEC syndrome may exist: one with cleft lip with or without cleft palate and the other with cleft palate alone [ 9 ]. In this case, the patient was born with both cleft lip and cleft palate. This disorder has been attributed to mutations in a gene encoding p63 [ 10 ].

From the aspect of oral health, patients with EEC syndrome are more susceptible to caries and gingivitis. They often face difficultly in maintaining oral hygiene due to ectrodactyly and limitations of using the hands [ 11 ].

Since the dental manifestations of this syndrome persist throughout life, dentists should work meticulously for the age appropriate dental rehabilitation of the patient. The preservation of deciduous and permanent teeth should be done whenever possible to conserve the investing bone for support and retention of removable prostheses.

Overdenture provides significant advantages biologically such as decreased bone resorption, increased masticatory performance, and directional sensitivity. Hence, in the present case report, we used tooth-supported overdenture as a viable option in contrast to other case reports [ 12 — 14 ].

Relining, rebasing, or remaking of the prosthesis is required to accommodate growth changes which we did at regular 6-month visits. A decrease in the secretory salivary levels increases the risk of caries after denture use. Therefore, fluoride application is very much important to protect the remaining teeth. The principal advantage of an overdenture is that the patient has the psychological benefit of having his own teeth which outweighs all the disadvantages stated.

In a similar case report, Gupta et al. Comprehensive treatment aiming consultation with a child psychologist, speech therapist, dermatologist, plastic surgeon, ophthalmologist, and renal specialist should be provided to the patient as and when required. In this case, the patient was referred to ophthalmologist as the patient complained of dry eyes, sticky discharge from the eyes, and eyelids completely glued upon while waking in the morning. The patient was diagnosed with nasolacrimal duct obstruction.

The patient was attended upon by a team of doctors which comprised a pedodontist, paediatrician, and plastic surgeon during his cleft lip and palate surgery at the age of 6 months and 3 years. Conclusion Early diagnosis and management of clinical manifestations associated with EEC syndrome requires a multipronged approach by a team consisting of physicians from several clinical modalities to provide comprehensive medical care.

This disorder is of concern to dental practitioners, and we need to be aware of dental conditions presenting with the syndrome and its management.

A sympathetic, rationale, and multidisciplinary approach is necessary to improve the physical, psychological, and social integration of such patients. Conflicts of Interest The authors declare that they have no conflicts of interest.

References R. Haase, and E. Andersson, P. Lindgren, and S. Durowaye, M. Adeboye, S. Yahaya-Kongoila et al. Gorlin, M. Cohen, and L. Bixler, J. Spivack, J. Bennett, and J. Dhar and A. Roelfsema and J. Marwaha and K. Pettit and P. Mattoo, A. Kapoor, and S. View at: Google Scholar R. Samra, S. Bhide, C. Goyal, and T. Elhamouly and K. Gupta, S. Mittal, S. Tewari, and S. This is an open access article distributed under the Creative Commons Attribution License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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