Successful Management of a Complicated Forearm Fracture in a Patient with Congenital Insensitivity to Pain: A Case...

Congenital insensitivity to pain (CIPA) is an extremely rare syndrome that is caused by a mutation in the NTRK gene. CIPA is characterized by recurrent episodes of unexplained fever, infections, skeletal complications, anhidrosis, mental retardation, and ocular injuries due to the absence of reaction to painful stimuli. Several musculoskeletal complications have been reported and included fractures, avascular necrosis, joint dislocations, soft tissue, and bone infections. The severity of these complications presented complex diagnostic and therapeutic dilemmas. The collaboration of medical professionals from different specialties to provide appropriate medical care is needed.

We present a case of a 1-year-old girl, diagnosed with CIPA, admitted to our center complaining of wound discharge and fever from a previously repaired left forearm fracture with nail insertion, which was found to be infected after a failed trial of nail removal. A multidisciplinary- management from anesthesia, orthopedics, ophthalmology, and pediatrics was conducted. The patient underwent successful nail removal, wound debridement, and radius bone reduction with the appropriate anesthetic protocol that did not include analgesic medications. The follow-up course was uneventful with proper alignment and virtually stable health status, with no apparent skeletal or ocular complications.

This report shows a case of CIPA with complicated forearm fracture. Patients with CIPA should be afforded a multidisciplinary healthcare approach in order to improve their quality of life and decrease the rate of complications. Parents and patients should be educated promptly. Orthopedics complications imply significant morbidity and require prompt surgical intervention with intense follow-up.