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Cardiology175 papers

Lesion of thoracic spine

Last edited: 4/14/2026

Overview

Diffuse idiopathic skeletal hyperostosis (DISH), also known as Forestier's disease, is a chronic disorder characterized by abnormal bone formation in the ligaments and entheses, predominantly affecting the spine and causing stiffness and pain. Pycnodysostosis, a rare skeletal dysplasia, can also involve spinal abnormalities leading to back pain and fractures due to high bone density and fragility.

Diagnosis

  • Key Criteria for DISH: Presence of flowing osteophyte formation along the anterolateral spinal dura, absence of apophyseal or sacroiliac joint involvement, and at least three contiguous vertebrae with modified Stoke's criteria 6.
  • Recommended Tests: Radiography (X-ray) is primary; CT and MRI may be used for further evaluation of spinal involvement and complications 3.
  • Pycnodysostosis Diagnosis: Clinical features include short stature, characteristic facial appearance, and acroosteolysis; genetic testing confirms cathepsin K gene mutations 411.
  • Management

  • DISH: Symptomatic treatment with NSAIDs for pain and stiffness; physical therapy to maintain mobility 5.
  • Pycnodysostosis: Supportive care focusing on fracture prevention, nutritional support, and management of associated conditions like anemia 1012.
  • Special Populations

  • Pediatrics: Early diagnosis crucial; overlap with conditions like osteopetrosis and thalassemia can complicate management 912.
  • Elderly: DISH more prevalent; careful monitoring for complications such as dysphagia and respiratory issues 2.
  • Comorbidities: Prolonged retinoid use may be associated with early onset DISH; monitor patients with cystic acne treated long-term with retinoids 5.
  • Key Recommendations

  • Investigate young patients with a history of prolonged retinoid exposure for DISH when presenting with back stiffness 5 (Evidence: Moderate).
  • Consider radiographic evaluation with CT or MRI for detailed assessment of spinal involvement in DISH patients 3 (Evidence: Moderate).
  • Manage pycnodysostosis with multidisciplinary care addressing bone fragility, nutritional deficiencies, and associated systemic manifestations 10 (Evidence: Weak).
  • References

    1 Ashraf O, Channapatna Suresh S, Raju B, Jumah F, Sun H, Gupta G et al.. Jacques Forestier: Forgotten Contributions of a Rheumatologist to Spine Surgery. World neurosurgery 2021. link 2 Cherfane P, Smaily H, Khalaf MG, Ghaoui N, Melkane AE. Otolaryngologic manifestations of diffuse idiopathic skeletal hyperostosis (Forestier's disease): A systematic review of the literature. Joint bone spine 2021. link 3 Kuperus JS, Westerveld LA, Rutges JP, Alblas J, van Rijen MH, Bleys RL et al.. Histological characteristics of diffuse idiopathic skeletal hyperostosis. Journal of orthopaedic research : official publication of the Orthopaedic Research Society 2017. link 4 Baglam T, Binnetoglu A, Fatih Topuz M, Baş Ikizoglu N, Ersu R, Turan S et al.. Pycnodysostosis at otorhinolaryngology. International journal of pediatric otorhinolaryngology 2017. link 5 Zhao S, Goodson NJ. Diffuse idiopathic skeletal hyperostosis and isotretinoin in cystic acne. BMJ case reports 2015. link 6 Mader R, Buskila D, Verlaan JJ, Atzeni F, Olivieri I, Pappone N et al.. Developing new classification criteria for diffuse idiopathic skeletal hyperostosis: back to square one. Rheumatology (Oxford, England) 2013. link 7 Ornetti P, Prati C, Fery-Blanco C, Streit G, Toussirot E, Wendling D. Pedicle stress fracture: an unusual complication of pycnodysostosis. Clinical rheumatology 2008. link 8 Mills KL, Johnston AW. Pycnodysostosis. Journal of medical genetics 1988. link 9 Kumar R, Misra PK, Singhal R. An unusual case of pycnodysostosis. Archives of disease in childhood 1988. link 10 Santhanakrishnan BR, Panneerselvam S, Ramesh S, Panchatcharam M. Pycnodysostosis with visceral manifestation and rickets. Clinical pediatrics 1986. link 11 Meneses de Almeida L. A genetic study of pycnodysostosis. Progress in clinical and biological research 1982. link 12 Benz G, Schmid-Rüter E. Pycnodysostosis with Heterozygous beta-thalassemia. Pediatric radiology 1977. link

    Original source

    1. [1]
      Jacques Forestier: Forgotten Contributions of a Rheumatologist to Spine Surgery.Ashraf O, Channapatna Suresh S, Raju B, Jumah F, Sun H, Gupta G et al. World neurosurgery (2021)
    2. [2]
    3. [3]
      Histological characteristics of diffuse idiopathic skeletal hyperostosis.Kuperus JS, Westerveld LA, Rutges JP, Alblas J, van Rijen MH, Bleys RL et al. Journal of orthopaedic research : official publication of the Orthopaedic Research Society (2017)
    4. [4]
      Pycnodysostosis at otorhinolaryngology.Baglam T, Binnetoglu A, Fatih Topuz M, Baş Ikizoglu N, Ersu R, Turan S et al. International journal of pediatric otorhinolaryngology (2017)
    5. [5]
    6. [6]
      Developing new classification criteria for diffuse idiopathic skeletal hyperostosis: back to square one.Mader R, Buskila D, Verlaan JJ, Atzeni F, Olivieri I, Pappone N et al. Rheumatology (Oxford, England) (2013)
    7. [7]
      Pedicle stress fracture: an unusual complication of pycnodysostosis.Ornetti P, Prati C, Fery-Blanco C, Streit G, Toussirot E, Wendling D Clinical rheumatology (2008)
    8. [8]
      Pycnodysostosis.Mills KL, Johnston AW Journal of medical genetics (1988)
    9. [9]
      An unusual case of pycnodysostosis.Kumar R, Misra PK, Singhal R Archives of disease in childhood (1988)
    10. [10]
      Pycnodysostosis with visceral manifestation and rickets.Santhanakrishnan BR, Panneerselvam S, Ramesh S, Panchatcharam M Clinical pediatrics (1986)
    11. [11]
      A genetic study of pycnodysostosis.Meneses de Almeida L Progress in clinical and biological research (1982)
    12. [12]
      Pycnodysostosis with Heterozygous beta-thalassemia.Benz G, Schmid-Rüter E Pediatric radiology (1977)

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