This Article Full Text (PDF) Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal My Folders Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Bar-On, E. Articles by Steinberg, T. Search for Related Content PubMed PubMed Citation Articles by Bar-On, E. Articles by Steinberg, T.

Congenital insensitivity to pain

ORTHOPAEDIC MANIFESTATIONS

¹ Paediatric Orthopaedic Unit
² Department of Microbiology and Immunology, Faculty of Health Sciences, Ben Gurion University, Beer Sheva, Israel.
³ Child Neurology Unit, Schneider Children’s Medical Centre, 14 Kaplan Street, Peteh Tikva 49202, Israel.

Correspondence should be sent to Dr E. Bar-On.

We reviewed 13 patients with congenital insensitivity to pain. A quantitative sweat test was carried out in five and an intradermal histamine test in ten. DNA examination showed specific mutations in four patients. There were three clinical presentations: type A, in which multiple infections occurred (five patients); type B, with fractures, growth disturbances and avascular necrosis (three patients); and type C, with Charcot arthropathies and joint dislocations, as well as fractures and infections (five patients, four with mental retardation).

Patient education, shoeware and periods of non-weight-bearing are important in the prevention and early treatment of decubitus ulcers. The differentiation between fractures and infections should be based on aspiration and cultures to prevent unnecessary surgery. Established infections should be treated by wide surgical debridement. Deformities can be managed by corrective osteotomies, and shortening by shoe raises or epiphysiodesis. Joint dislocations are best treated conservatively.