Arthritis in a child secondary to congenital insensitivity to pain and self-aggression. Why and when pain is good?
Author: Emad Y, El Yasaki A, Ragab Y, Khalifa M, Moawayh O, Salama M.
Source:
Clinical rheumatology, 26(7), 1164-1166.
A 9 year-old female child presented with recurrent arthritis of ankles, left knee
and unequal leg length. Clinical examination revealed mild valgus deformity in
her left knee with grade 2 effusion, arthritis of both ankles and deformity in
her left wrist. Examination of the affected joints showed no evidence of
tenderness upon active or passive movements and the patient did not show any
limping upon gait analysis. Past history of the patient revealed evidence of
previous dislocation of her left hip and previous fibular fracture. Revision of
her previous x-rays showed left hip dislocation, fracture left fibula and
fracture of right metatarsal bone after repetitive trauma which pass unnoticed.
Recent x-ray of her left knee showed osteochondral injury. Laboratory
investigations were done to rule out common causes of childhood arthritis and
revealed: ESR 12 1st hours, CRP negative, negative rheumatoid factor, and
negative ANNeurological evaluation of the patient documented congenital
insensitivity to pain and EMG studies confirmed evidence of sensory neuropathy.
Traumatic arthritis resulting from congenital insensitivity to pain with
self-aggression is rarely encountered in children but should be considered in the
differential diagnosis specially if radiological features point to repetitive
trauma with attempts of healing.