FENG Bin, TIAN Ye, QIU Gui-xing, WENG Xi-sheng, JIANG Yu and ZHOU Xi

    Congenital insensitivity to pain with anhidrosis (CIPA) is an autosomal recessive form of sensory neuropathy manifesting with the lack of perception of pain.1 There have been few reports of Charcot spine in patients with CIPA,2 and surgery for such cases was accompanied by high revision proportion.3,4 The purpose of this report is to describe a patient with CIPA who developed a recurrent Charcot arthropathy in her lumbar spine and was treated by revision surgery, with the aim to discuss the strategy of surgical management for such cases.
    A 15-year-old girl from the eastern part of China was presented to our clinic and stated that she had progressive lumber hump ever since it was detected by her mother 2 years ago. This girl also felt weakness of right lower limb over the past 1 month.
    She had a history of anhidrosis and paroxysmal fever since she was born. She was consulted in children’s hospital for high fever when she was eleven months old. Neurological examinations showed that the girl was insensitive to superficial painful stimuli. Iodine-starch test revealed no sweating. She was diagnosed with CIPA and pneumonia which was treated with antibiotics. The patient’s parents were instructed to protect her from dangerous conditions ever since she was considered as insensitivity to pain. Nevertheless, she experienced right tibial, fibular, and left femoral shaft fracture. Thereafter, these fractures were treated by plaster immobilization or surgical treatment. Limb length discrepancy developed after the fractures of lower limbs.
    The patient presented lumbar hump when she visited our clinic. Physical examination showed increased joint laxity, dry skin, and obvious lumbar kyphosis deformity. She had Charcot arthropathy of both ankle joints. She also had 4 cm length discrepancy in right lower extremity shorter than the left and was being treated with orthosis. No obvious acro-osteolysis was presented.