Dyskeratosis congenita: Dental management of a medically complex child

Esti Davidovich, Dan Eimerl, Memet Aker, Joseph Shapira, Benjamin Peretz

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)244-248
Number of pages5
JournalPediatric Dentistry
Volume27
Issue number3
StatePublished - 2005
Externally publishedYes

Keywords

  • Anesthesia
  • Dental Treatment
  • Dyskeratosis Congenita
  • Oral Findings
  • Perioperative Medicine

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