6 Iconography
Access to the text (HTML) Access to the text (HTML)
PDF Access to the PDF text

Access to the full text of this article requires a subscription.
  • If you are a subscriber, please sign in 'My Account' at the top right of the screen.

  • If you want to subscribe to this journal, see our rates

  • You can purchase this item in Pay Per ViewPay per View - FAQ : 30,00 € Taxes included to order
    Pages Iconography Videos Other
    3 6 0 0

Joint Bone Spine
Volume 80, n° 6
pages 650-652 (décembre 2013)
Doi : 10.1016/j.jbspin.2013.03.006
accepted : 7 Mars 2013
Diagnosis and surgical treatment of progressive pseudorheumatoid dysplasia in an adult with severe spinal disorders and polyarthropathy

Xi Yang, Yueming Song, Qingquan Kong
 Department of Orthopedic Surgery, West China Hospital, Sichuan University, No. 37, GuoXue Road, Chengdu, Sichuan, China 

Corresponding author. Tel.: +86 28 85 42 24 70; fax: +86 28 85 42 34 38.

Progressive pseudorheumatoid dysplasia (PPD) is a rare autosomal-recessive disorder. The polyarthritis of PPD has been detailed before. However, the spinal disorder and surgical treatment been rarely mentioned. A 44-year-old patient who has been misdiagnosed as juvenile rheumatoid arthritis (JRA) and given unilateral total hip replacement yet, suffers mainly from severe spinal disorder this time. The platyspondyly, Scheuermann-like lesions of the spine and JRA-like features of the peripheral joints were found on radiographic films, combining negative inflammatory and rheumatoid factors, which most suggested the diagnosis of PPD. As the homozygous nucleotide deletion was found in WISP3 gene, diagnosis of PPD was definite. Neurological examination and further imaging examination indicated severe compression of thoracic and lumbar spinal cord which might lead to his conspicuous spinal disorder. Decompressive laminectomy, posterior fusion and fixation were performed. And an excellent clinical outcome was achieved 1year after the decompression and fusion: leg pain and hypoesthesia resolved and osseous fusion performed. This is the first reported decompression in the adult spine of PPD. Surgical treatment could receive satisfactory result in PPD, however, it is a palliative therapy which has less help to prevent the development of this disease. Early diagnosis and rehabilitation interventions remain the most important. Clinical, radiographic and genetic features in PPD are crucial in the differential diagnosis.

The full text of this article is available in PDF format.

Keywords : Platyspondyly, Polyarthrosis, Spinal disorder, Progressive pseudorheumatoid dysplasia

© 2013  Société française de rhumatologie@@#104156@@
EM-CONSULTE.COM is registrered at the CNIL, déclaration n° 1286925.
As per the Law relating to information storage and personal integrity, you have the right to oppose (art 26 of that law), access (art 34 of that law) and rectify (art 36 of that law) your personal data. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
Personal information regarding our website's visitors, including their identity, is confidential.
The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Article Outline
You can move this window by clicking on the headline