Down syndrome and neurofibromatosis: a case report

Spec Care Dentist. 2014 Nov-Dec;34(6):313-8. doi: 10.1111/scd.12062. Epub 2013 Dec 10.

Abstract

Background: The dental management of a patient presenting with both Down syndrome and neurofibromatosis type 1 (NF1) has not previously been described well in the dental literature.

Case description: A 20-year-old male with both of these genetic anomalies sought comprehensive treatment at the Special Needs Dental clinic at the Arizona School of Dentistry and Oral Health. He presented with multiple decayed surfaces, retained primary teeth, and intra/extra oral soft tissue tumors. Dental extractions and tumor reduction surgery took place at a private dental office due to the need for intravenous sedation for patient management. At the conclusion of the patient's -treatment, while his oral health was improved, there was little improvement in the facial aesthetics of his case.

Clinical implications: Coordinating care among health care providers in a patient with Trisomy 21 and NF1 is essential for a reliable and predictable outcome. However, as neurofibromas are often known to recur, the treatment risks and advantages should be reviewed prior to surgical intervention.

Keywords: Down syndrome; access to care; dental treatment; neurofibromatosis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Down Syndrome / complications*
  • Humans
  • Male
  • Neurofibromatoses / complications*
  • Neurofibromatoses / surgery
  • Young Adult