Spinal Manifestations in a Patient with Congenital Insensitivity to Pain

Abstract

Spinal manifestations in congenital insensitivity to pain are relatively uncommon and easily misdiagnosed. We report on a patient with absent protective pain sensation, who developed spinal neuropathic arthropathy. At age 11 years, he presented with a destructive lesion at the L1-L2 level, causing him tingling sensation in both lower limbs. He was treated with combined anteroposterior spinal fusion from T12 to L3 and had full recovery. Five years later, he presented with a long history of clicking in his low back, muscle weakness and paresthesia in both lower extremities during walking, and evidence of Charcot arthropathy at the L4-L5 level, resulting in junctional kyphosis and canal narrowing. Posterior spinal arthrodesis from L3 to the sacrum was performed, due to lack of patient and parental consent for combined anterior decompression/posterior fusion. The patient resumed normal muscle function and his previous level of activities. Spinal complications should be anticipated in this condition and create diagnostic and therapeutic dilemmas. However, surgical management can produce favorable clinical results.