Congenital Insensitivity to Pain and Anhidrosis: A painful experience by a painless child

Abstract

A three-year-old boy with congenital insensitivity to pain and anhidrosis (CIPA) was first presented with transphyseal separation of distal left humerus. Surgical treatment with closed reduction and k-wiring of transphyseal separation of distal humerus resulted in devastating surgical site infection and osteomyelitis. Multiple surgical debridement and application of external fixator to eliminate infection were futile. At the age of four years, he refused to bear weight as a result of left hip dislocation. Closed reduction and spica cast was unsuccessful, resulting in recurrent left hip dislocation. He has since not been able to walk. At the age of five years, the right hip was dislocated followed by ipsilateral subtrochanteric femur fracture. Appropriate treatments such as hip spica and titanium elastic nail were not possible due to extensive bruising and skin ulceration over the right thigh. This child ended up with a functionless left upper limb, bilateral hip dislocation and malunited right femur fracture. Conservative treatment and watchful neglect might be the ideal treatment for patients with CIPA.