Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
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Advances in Clinical and Experimental Medicine

2014, vol. 23, nr 4, July-August, p. 605–610

Publication type: original article

Language: English

Ophthalmic Manifestations in Patients with Ectodermal Dysplasia Syndromes

Ugur Keklikci1,A,B,C,D,E,F, Izzet Yavuz2,A,B,C,E,F, Selcuk Tunik3,B,C,E, Zelal Baskan Ulku4,A,B,C, Sedat Akdeniz5,A,B,C,D

1 Department of Ophthalmology, Dicle University, Faculty of Medicine, Diyarbakir, Turkey

2 Department of Pediatric Dentistry, Dicle University, Faculty of Dentistry, Diyarbakir, Turkey

3 Department of Histology and Embryology, Dicle University, Faculty of Medicine, Diyarbakir, Turkey

4 Department of Prosthetic Dentistry, Dicle University, Faculty of Dentistry, Diyarbakir, Turkey

5 Department of Dermatology, Dicle University, Faculty of Medicine, Diyarbakir, Turkey

Abstract

Background. Ectodermal dysplasia (ED) is a disorder that results from abnormal formation of at least two of the four major ectodermal derivatives in the developing embryo. The ectoderm of the embryo forms the skin, teeth, hair and nails, sweat glands and part of the eyes.
Objectives. The aim of this article is to reveal ophthalmologic symptoms and signs as multidisciplinary, reliable criteria for ectodermal dysplasia.
Material and Methods. In this retrospective study, 24 patients with ED were analyzed from the recorded data. Ophthalmological examination of the patients, who had previously received the diagnosis of ED in the dental department, was done. During the examination, ocular symptoms related to tear film, corneal changes, lacrimal duct, periorbital hyperpigmentation, alteration lashes and eyebrows were evaluated.
Results. The age ranged between 3–45, and the mean and standard deviation (Mean ± SD) was 15.8 ± 7.4 years. The number of males was 13 (54.2%) and females, 11 (45.8%). Eighteen patients (75.0%) suffered from ocular complaints related to the ocular surface. In 11 of the patients with ED, there were dry eye symptoms. While the mean age of cases with eye involvement was 17.5, it was 23.1 in cases with dry eye symptoms.
Conclusion. In the study, it was observed that, in patients with ED, ocular complaints, particularly dry eye symptoms, may increase as age advances.

Key words

dry eye, ectodermal dysplasia, ocular involvement.

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