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Proximal Junctional Kyphosis After Posterior Spinal Fusion for Severe Kyphoscoliosis in a Patient With PIEZO2-deficient Arthrogryposis Syndrome.

Authors
  • Uehara, Masashi1
  • Kosho, Tomoki2, 3
  • Takano, Kyoko2, 3
  • Inaba, Yuji4
  • Kuraishi, Shugo1
  • Ikegami, Shota1
  • Oba, Hiroki1
  • Takizawa, Takashi1
  • Munakata, Ryo1
  • Hatakenaka, Terue1
  • Takahashi, Jun1
  • 1 Department of Orthopaedic Surgery, Shinshu University School of Medicine, Matsumoto, Nagano, Japan. , (Japan)
  • 2 Department of Medical Genetics, Shinshu University Hospital, Matsumoto, Japan. , (Japan)
  • 3 Center for Medical Genetics, Shinshu University Hospital, Matsumoto, Japan. , (Japan)
  • 4 Division of Neurology, Nagano Children's Hospital, Azumino, Japan. , (Japan)
Type
Published Article
Journal
Spine
Publication Date
May 15, 2020
Volume
45
Issue
10
Identifiers
DOI: 10.1097/BRS.0000000000003347
PMID: 31770315
Source
Medline
Language
English
License
Unknown

Abstract

Case report. Describe the clinical and radiological outcomes of a patient with a piezo-type mechanosensitive ion channel component 2 (PIEZO2)-deficient arthrogryposis receiving surgery for severe kyphoscoliosis. Spinal deformity is a characteristic feature of arthrogryposis due to PIEZO2 gene deficiency, for which surgical correction is indicated when the deformity is progressive to avoid neurological deficits and respiratory impairment. However, there exist few reports on the surgical treatment of spinal deformity in PIEZO2-deficient arthrogryposis, and no therapeutic standards have been established. We retrospectively reviewed a case of proximal junctional kyphosis after posterior spinal fusion for severe kyphoscoliosis in PIEZO2-deficient arthrogryposis. The patient was a 13-year-old girl with PIEZO2-deficient arthrogryposis who underwent posterior spinal fusion with an all-pedicle screw construct from T2 to L2 for a preoperative main thoracic curve Cobb angle of 78° and thoracic kyphotic angle of 83°. Postoperative Cobb angle of the main thoracic curve and thoracic kyphotic angle were improved at 11° and 34°, respectively. Although revision surgery was required for neurological deficits from proximal junctional kyphosis, she could walk with a crutch and improvements in clinical questionnaire scores were noted at 2 years and 3 months after surgery. Based on the present case, posterior spinal fusion represents a good treatment option for severe spinal deformity in PIEZO2-deficient arthrogryposis. Careful consideration of fusion level is needed to prevent proximal junctional kyphosis. 5.

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