Orthopaedic manifestations in congenitally insensate patients.
The spectrum of orthopaedic problems in eight congenitally insensate patients was reviewed. The conditions included congenital insensitivity to pain, Riley-Day syndrome, and Lesch-Nyhan syndrome. In each of these conditions, the patient has an abnormality of interpretation of painful stimuli or lacks normal pain avoidance, leading to self-inflicted damage. The orthopaedic problems and complications included fracture, self-mutilation, autoamputation, osteomyelitis, septic arthritis, Charcot joints, scoliosis, and dislocation. Effective management consists of early diagnosis and patient/parent education to prevent as many complications as possible. Fractures may be treated conservatively, while progressive scoliosis requires operative intervention. Osteomyelitis, septic arthritis, and Charcot joints require appropriate operative treatment.
Shriners Hospital for Crippled Children, Tampa Unit, FL 33612-9499.
K J
KJ
Guidera
H
H
Multhopp
T
T
Ganey
J A
JA
Ogden
D000293:Adolescent; D000328:Adult; D001847:Bone Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004402:Dysautonomia, Familial / Q000150:complications* / Q000503:physiopathology / Q000523:psychology; D005260:Female; D006801:Humans; D007223:Infant; D007926:Lesch-Nyhan Syndrome / Q000150:complications* / Q000503:physiopathology / Q000523:psychology; D008297:Male; D000699:Pain Insensitivity, Congenital / Q000150:complications* / Q000503:physiopathology / Q000523:psychology; D010353:Patient Education as Topic; D011859:Radiography