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Spinal deformity caused by hyperimmunoglobulin E syndrome: clinical article.

Naoko Araya1, Hiroyuki Inose, Tsuyoshi Kato

  • 1Department of Orthopaedics, Tokyo Medical and Dental University, Tokyo, Japan.

Journal of Neurosurgery. Spine
|May 20, 2014
PubMed
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Hyperimmunoglobulin E syndrome (HIES) can cause spinal issues. This case report details successful surgical correction of spinal deformity in a HIES patient, preventing postoperative infection.

Area of Science:

  • Immunology
  • Orthopedic Surgery
  • Genetics

Background:

  • Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency.
  • HIES presents with recurrent infections and skeletal abnormalities like scoliosis and osteoporosis.
  • Spinal deformity management in HIES is rarely reported, with limited data on surgical interventions.

Observation:

  • A 16-year-old male with HIES presented with low-back pain and soft tissue infections.
  • Radiological evaluation revealed a deteriorated kyphotic deformity due to a pathological T-11 compression fracture.
  • Conservative treatment failed to correct the spinal deformity.

Findings:

  • The patient underwent combined anterior and posterior spinal fixation surgery.
  • Postoperatively, the patient exhibited no signs of infection.
Keywords:
BMD = bone mineral densityHIES = hyperimmunoglobulin E syndromeHyper IgE syndromeJob's syndromeimmunodeficiencyinfectionosteoporosisspinal deformity

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  • The surgical approach aimed to prevent complications, particularly postoperative infection.

    • Implications:

    • This case highlights the potential for successful surgical management of spinal deformities in HIES patients.
    • Careful surgical planning and execution may mitigate the risk of infection in this vulnerable population.
    • Further research is warranted to establish optimal treatment strategies for spinal manifestations of HIES.