Dental Treatment Approach to the Child With Chanarın-Dorfman Syndrome: A Case Report

Year 2016, Volume: 43 Issue: 2 - Volume: 43 Issue: 2, 113 - 118, 01.05.2016

İrem Can Olgen Erçin Şeberol Merve Kurun Aksoy Nurhan Uslu Özalp

Abstract

Chanarin-Dorfman Syndrome CDS is a rare autosomal recessive inherited neutral lipid metabolism disorder characterized with ichthyosis lesions and multi-system involvement. There are limited number of sources notification of this syndrome. The aim of this case report was to report difficulties in dental treatment process of an 8year-old patient diagnosed with CDS who was referred to Ankara University Faculty of Dentistry Department of Pediatric Dentistry due to dental pain. In the extra oral examination, erythema and desquamation of the skin on entire body surface, scalp scaling was detected and the patient had difficulty in opening her mouth because of anguler fissures. In the intraoral examination multiple carious teeth 16, 26, 36, 46, 54, 55, 64, 65, 75, 84 and 85 were determined, also molar incisor hypomineralization MIH was diagnosed. Conservative, surgical, prophylactic treatment and removable space maintainers were applied. There was no literature detected about the dental approach for this syndrome. It can be concluded that there is a major necessity of the children with CDS have to be redirected to the pediatric dentists in early stages of the childhood and the preventive programmes should be applied

Keywords

Chanarin-Dorfman syndrome, ichthyosis, autosomal recessive, dental treatment

CHANARIN-DORFMAN SENDROMLU ÇOCUK HASTAYA DENTAL TEDAVİ YAKLAŞIMI: OLGU SUNUMU

Abstract

Chanarin-Dorfman Sendromu CDS , nadir görülen, otozomal ressesif genetik geçişli, iktiyozis ve multi sistem tutulumu ile karakterize bir lipid metabolizması hastalığıdır. Bu sendroma ait sınırlı sayıda kaynak bildirimi bulunmaktadır. Bu olgu sunumunda, diş ağrısı nedeniyle Ankara Üniversitesi Diş Hekimliği Fakültesi Pedodonti Anabilim Dalı’na başvuran 8 yaşındaki CDS’li hastanın diş tedavisi sırasında sendroma bağlı karşılaşılan problemlerin sunulması amaçlanmıştır. Hastanın ağız dışı muayenesinde, kafa derisi ve tüm vücutta eritem, deskuamasyon ve pullanma ile birlikte hastanın dudak kenarlarındaki çatlaklar nedeniyle ağzını açmakta zorlandığı gözlenmiştir. Hastanın ağız içi muayenesinde ise çok sayıda çürük varlığı 16, 26, 36, 46, 54, 55, 64, 65, 75, 84 ve 85 no’lu dişler tespit edilirken hastaya büyük azı keser hipomineralizasyonu BAKH teşhisi konulmuştur. Hastaya gerekli konservatif, cerrahi ve profilaktik tedaviler uygulanmış olup hareketli yer tutucu yapılmıştır. Literatürde söz konusu sendromla ilgili dental yaklaşıma ait hiçbir kaynağa rastlanmamış olup, CDS’li çocuk hastaların erken dönemde bir çocuk diş hekimine yönlendirilmesi ve koruyucu uygulamaların yapılması gerekliliği bir kez daha gözler önüne serilmektedir

Keywords

Chanarin-Dorfman sendromu, iktiyozis, otozomal ressesif, diş tedavisi

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