Orthopaedic manifestations of congenital insensitivity to pain

Abstract

Congenital insensitivity to pain is a rare inherited disorder that requires the combined skills of paediatricians, neurologists and orthopaedic surgeons.

Congenital Insensitivity to Pain with Recurrent Septic Arthritis of the Left Knee

“…2 The principal orthopaedic manifestations are neuropathic joints, osteomyelitis and recurrent fractures of the limbs. 3 Patients are at high risk of injury due to loss of pain sensation which is a protective mechanism of the body. 4 The importance of orthopaedic management lies in the prevention of musculoskeletal complications by educating the patients and family members regarding the risks in relation to the environment in the absence of pain constraints and the high incidence of self-abusive behavior.…”

Spinal Manifestations in a Patient with Congenital Insensitivity to Pain

“…7,12 Other principal orthopaedic manifestations of the disease include recurrent fractures, autoamputation, bone and joint infections, neurotrophic joint disease, mainly evident in the weight-bearing joints, joint dislocations, acro-osteolysis, limb length discrepancy, as the result of growth arrest, and scoliosis. 1,[7][8][9]11,12,[15][16][17] The benchmark of orthopaedic management in congenital insensitivity to pain focuses on preventing occurrence of musculoskeletal complications in patients who are otherwise physically fully active, through proper education aiming to increase awareness in the patients and their family environment of the possible risks related to the absence of pain constraints and the increased incidence of self-abusive behavior. Fortunately, with further maturation, the children learn to self-protect and develop a better understanding of their limitations.…”

Skeletal complications in congenital insensitivity to pain with anhidrosis: a case series of 14 patients and review of articles published in Japanese

“…Without it, there is a lack of protective reactions to acute pain. Therefore, individuals with CIPA may sustain repeated bone fractures and multiple joint dislocations [6][7][8][9]. In addition, patients often do not realize that they have injuries, which leads to delays in diagnosis and difficulties with treatment.…”