%0 Journal Article
%T Dental Treatments under the General Anesthesia in a Child with Keratitis, Ichthyosis, and Deafness Syndrome
%A Sera S£¿msek Derelioglu
%A Y¨¹cel Y£¿lmaz
%A Sultan Keles
%J Case Reports in Dentistry
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/618468
%X KID syndrome is a rare genodermatosis characterized by keratitis, ichthyosis, and sensorineural deafness. Although the dermatological, ophthalmologic, and sensorineural defects are emphasized in the literature, oral and dental evaluations are so superficial. In this case report, dental and oral symptoms of a three year and five months old boy with KID syndrome, suffering severe Early Childhood Caries (s-ECC) and dental treatments done under General Anesthesia (GA) were reported. 1. Introduction Keratitis-ichthyosis-deafness (KID) syndrome is congenital ectodermal disorder without a clear mode of inheritance and is characterized by erythrokeratoderma, sensorineural hearing loss, and vascularizing keratitis [1¨C5]. KID syndrome is usually associated with less severe keratoderma and a milder hearing problem, but the eye involvement (keratitis) may eventually lead to impaired vision [3]. Keratitis is rather a late finding and may not sometimes be seen [6]. The cause of KID syndrome was identified as a germline missense mutation in the GJB2 (gap junction¦Â-2) gene encoding for connexin-26, which is essential for gap function formation in various tissues [7, 8]. Mutations in the GJB2 gene encoding connexin 26 are detrimental to function of cochlea, palmoplantar epidermis, hair follicles, corneal epithelium, and sweat glands and ducts, causing nonsyndromic sensorineural deafness, palmoplantar keratoderma and hearing impairment, Vohwinkel syndrome, and KID syndrome [8¨C11]. The first component required for diagnosis is characteristic skin findings which are usually present at birth or in early infancy. The well-demarcated, erythematous, hyperkeratotic plaques with verrucous surface are distributed over face and extremities alternating with smoother areas [3]. Hyperkeratotic plaques over the face give patients an appearance of premature aging [12]. The second one is presence of sensorineural hearing loss. The third component is ophthalmologic defects, which can progress total blindness [13]. Most patients have sparse or absent scalp hair, eyebrows, and eyelashes [3]. Nails may be thickened, deformed, brittle, white, hypoplastic, or normal. In some patients teeth are normally developed, but in others they may be defective and they are likely to develop caries [3, 14¨C17]. However, oral mucosa, status of the primary teeth and development of permanent teeth of the children with KID syndrome, and treatment approaches for those children have not been investigated so far. Here, we report the dental restorative treatments provided for a three-year and five-month-old
%U http://www.hindawi.com/journals/crid/2013/618468/