Congenital insensitivity-to-pain with anhidrosis (CIPA): a case report with 4-year follow-up

Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006 Jun;101(6):769-73. doi: 10.1016/j.tripleo.2005.07.030. Epub 2006 Apr 21.

Abstract

Congenital Insensitivity-to-pain with anhidrosis (CIPA) is a rare disorder in which pain perception is absent from birth, despite the fact that all other sensory modalities remain intact or minimally impaired and tendon reflexes are present. The challenge in dentistry is to manage the self-mutilation behavior avoiding serious damages especially to oral structures, hands and fingers. A Brazilian case of CIPA is presented and discussed with clinical documentation of the oral-related problems over a 4-year follow-up. A conservative treatment (mouthguard-like appliance) was proposed with the objective to avoid full mouth extraction.

Publication types

  • Case Reports

MeSH terms

  • Consanguinity
  • Female
  • Follow-Up Studies
  • Humans
  • Hypohidrosis / complications*
  • Infant
  • Mouth Protectors
  • Oral Ulcer / etiology*
  • Pain Insensitivity, Congenital / complications*
  • Self Mutilation / etiology*
  • Self Mutilation / prevention & control
  • Tooth Avulsion / etiology*