Spinal deformity caused by hyperimmunoglobulin E syndrome: clinical article.
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.
Department of Orthopaedics, Tokyo Medical and Dental University, Tokyo, Japan.
Naoko
N
Araya
Hiroyuki
H
Inose
Tsuyoshi
T
Kato
Masanori
M
Saito
Satoshi
S
Sumiya
Tsuyoshi
T
Yamada
Toshitaka
T
Yoshii
Shigenori
S
Kawabata
Atsushi
A
Okawa
D000293:Adolescent; D019072:Antibiotic Prophylaxis; D003952:Diagnostic Imaging; D006801:Humans; D007589:Job Syndrome / Q000150:complications* / Q000175:diagnosis*; D007738:Kyphosis / Q000175:diagnosis* / Q000209:etiology* / Q000601:surgery; D008297:Male; D016103:Spinal Fractures / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D013123:Spinal Fusion / Q000379:methods*
BMD = bone mineral density; HIES = hyperimmunoglobulin E syndrome; Hyper IgE syndrome; Job's syndrome; immunodeficiency; infection; osteoporosis; spinal deformity