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.


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@article{Guidera1990OrthopaedicMI, title={Orthopaedic manifestations in congenitally insensate patients.}, author={Kenneth J. Guidera and H Multhopp and Timothy M. Ganey and John A. Ogden}, journal={Journal of pediatric orthopedics}, year={1990}, volume={10 4}, pages={514-21} }