Authors: Davidovich, Esti; Eimerl, Dan; Aker, Memet; Shapira, Joseph; Peretz, Benjamin

Source: Pediatric Dentistry, Volume 27, Number 3, May/June 2005 , pp. 244-248(5)

Publisher: American Academy of Pediatric Dentistry

Abstract:

Dyskeratosis congenita (DKC) is a rare syndrome characterized by cutaneous hyperpigmentation, nail dystrophy, leukoplakia, and pancytopenia. The purpose of this case study was to describe the management of a 7-year-old girl diagnosed with DKC who urgently needed dental treatment under general anesthesia before bone marrow transplantation (BMT). The patient presented normal skin, nails, and hair, but oral examination revealed a number of ulcers, leukoplakia, gingival recessions, alveolar bone loss, and dental caries. Hematologic preparation included raising blood parameters, and the anesthesiologist to had consider pulmonary infection. The alveolar bone loss and the gingival recessions required the consultation of a periodontist. Avoiding stainless steel crowns was necessary due to potential plaque accumulation in the crown margins. The goal of this dental treatment was eliminating potential sources of infection before transplantation was conducted. It is important for the pediatric dentist to recognize the medical aspects associated with dental management prior to BMT, and to incorporate them into the treatment plan.

Keywords: DYSKERATOSIS CONGENITA; ORAL FINDINGS; DENTAL TREATMENT; ANESTHESIA; PERIOPERATIVE MEDICINE

Document Type: Research article

Publication date: 2005-05-01

More about this publication?

• Pediatric Dentistry is the official publication of the American Academy of Pediatric Dentistry, the American Board of Pediatric Dentistry and the College of Diplomates of the American Board of Pediatric Dentistry. It is published bi-monthly and is internationally recognized as the leading journal in the area of pediatric dentistry. The journal promotes the practice, education and research specifically related to the specialty of pediatric dentistry. This peer-reviewed journal features scientific articles, case reports and abstracts of current pediatric dental research.
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