title: Dyskeratosis congenita: dental management of a medically complex child.
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.
authors.affiliation: Department of Pediatric Dentistry, The Hebrew University-Hadassah School of Dental Medicine, Jerusalem, Israel.
authors.forename: Esti
authors.initials: E
authors.lastname: Davidovich
authors.forename: Dan
authors.initials: D
authors.lastname: Eimerl
authors.forename: Memet
authors.initials: M
authors.lastname: Aker
authors.forename: Joseph
authors.initials: J
authors.lastname: Shapira
authors.forename: Benjamin
authors.initials: B
authors.lastname: Peretz
mesh_terms: D000361:Agammaglobulinemia / Q000209:etiology / Q000628:therapy; D016301:Alveolar Bone Loss / Q000150:complications; D016026:Bone Marrow Transplantation; D002648:Child; D017742:Dental Care for Chronically Ill; D003731:Dental Caries / Q000150:complications / Q000628:therapy; D003773:Dental Plaque / Q000150:complications; D003793:Dental Restoration, Permanent; D019871:Dyskeratosis Congenita / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D005889:Gingival Recession / Q000150:complications; D006801:Humans; D007972:Leukoplakia, Oral / Q000150:complications; D019226:Oral Ulcer / Q000150:complications; D014081:Tooth Extraction