Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency syndrome characterized by recurrent staphylococcal infections in the skin and lungs, with an incidence of less than one case per million persons. Skeletal and connective tissue abnormalities, such as scoliosis, osteoporosis, pathological fractures, and hyperextensive joints, are other manifestations of HIES. However, only one report documents the use of implants to treat spinal deformity caused by HIES, which was discovered following corrective surgery resulting in postoperative infection. In this case report, the authors describe a 16-year-old male with low-back pain and infections of the soft tissue. Radiological findings showed deteriorated kyphotic deformity due to the pathological compression fracture of T-11 with intensive conservative treatment. Anterior and posterior fixation surgery was performed. Thereafter, the patient showed no signs of infection. An investigation was conducted to avoid any postoperative infection.
Keywords: BMD = bone mineral density; HIES = hyperimmunoglobulin E syndrome; Hyper IgE syndrome; Job's syndrome; immunodeficiency; infection; osteoporosis; spinal deformity.