Best practice guidelines for management of spinal disorders in skeletal dysplasia

Klane K. White; Klane K. White; Michael B. Bober; Tae Joon Cho; Michael J. Goldberg; Michael J. Goldberg; Julie Hoover-Fong; Melita Irving; Shawn E. Kamps; Shawn E. Kamps; William G. Mackenzie; Cathleen Raggio; Samantha A. Spencer; Viviana Bompadre; Ravi Savarirayan

doi:10.1186/s13023-020-01415-7

Best practice guidelines for management of spinal disorders in skeletal dysplasia

Klane K. White, Klane K. White, Michael B. Bober, Tae Joon Cho, Michael J. Goldberg, Michael J. Goldberg, Julie Hoover-Fong, Melita Irving, Shawn E. Kamps, Shawn E. Kamps, William G. Mackenzie, Cathleen Raggio, Samantha A. Spencer, Viviana Bompadre, Ravi Savarirayan

School of Medicine

Research output: Contribution to journal › Article › peer-review

1 Scopus citations

Abstract

Background: Disorders of the spine present a common and difficult management concern in patients with skeletal dysplasia. Due to the rarity of these conditions however, the literature, largely consisting of small, single institution case series, is sparse in regard to well-designed studies to support clinical decision making in these situations. Methods: Using the Delphi method, an international, multi-disciplinary group of individuals, with significant experience in the care of patients with skeletal dysplasia, convened to develop multi-disciplinary, "best practice"guidelines in the care of spinal disorders in patients with skeletal dysplasia. Results: Starting with 33 statements, the group a developed a list of 31 "best practice"guidelines. Conclusions: The guidelines are presented and discussed to provide context for clinicians in their decision making in this often-challenging realm of care.

Original language	English (US)
Article number	161
Journal	Orphanet journal of rare diseases
Volume	15
Issue number	1
DOIs	https://doi.org/10.1186/s13023-020-01415-7
State	Published - Jun 24 2020

Keywords

Cervical instability
Kyphosis
Myelopathy
Scoliosis
Skeletal dysplasia
Spinal cord compression
Spine

ASJC Scopus subject areas

Genetics(clinical)
Pharmacology (medical)

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10.1186/s13023-020-01415-7

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White, K. K., White, K. K., Bober, M. B., Cho, T. J., Goldberg, M. J., Goldberg, M. J., Hoover-Fong, J., Irving, M., Kamps, S. E., Kamps, S. E., Mackenzie, W. G., Raggio, C., Spencer, S. A., Bompadre, V., & Savarirayan, R. (2020). Best practice guidelines for management of spinal disorders in skeletal dysplasia. Orphanet journal of rare diseases, 15(1), Article 161. https://doi.org/10.1186/s13023-020-01415-7

White, KK, White, KK, Bober, MB, Cho, TJ, Goldberg, MJ, Goldberg, MJ, Hoover-Fong, J, Irving, M, Kamps, SE, Kamps, SE, Mackenzie, WG, Raggio, C, Spencer, SA, Bompadre, V & Savarirayan, R 2020, 'Best practice guidelines for management of spinal disorders in skeletal dysplasia', Orphanet journal of rare diseases, vol. 15, no. 1, 161. https://doi.org/10.1186/s13023-020-01415-7

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author = "White, {Klane K.} and White, {Klane K.} and Bober, {Michael B.} and Cho, {Tae Joon} and Goldberg, {Michael J.} and Goldberg, {Michael J.} and Julie Hoover-Fong and Melita Irving and Kamps, {Shawn E.} and Kamps, {Shawn E.} and Mackenzie, {William G.} and Cathleen Raggio and Spencer, {Samantha A.} and Viviana Bompadre and Ravi Savarirayan",

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doi = "10.1186/s13023-020-01415-7",

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AU - Bober, Michael B.

AU - Cho, Tae Joon

AU - Goldberg, Michael J.

AU - Hoover-Fong, Julie

AU - Irving, Melita

AU - Kamps, Shawn E.

AU - Mackenzie, William G.

AU - Raggio, Cathleen

AU - Spencer, Samantha A.

AU - Bompadre, Viviana

AU - Savarirayan, Ravi

PY - 2020/6/24

Y1 - 2020/6/24

N2 - Background: Disorders of the spine present a common and difficult management concern in patients with skeletal dysplasia. Due to the rarity of these conditions however, the literature, largely consisting of small, single institution case series, is sparse in regard to well-designed studies to support clinical decision making in these situations. Methods: Using the Delphi method, an international, multi-disciplinary group of individuals, with significant experience in the care of patients with skeletal dysplasia, convened to develop multi-disciplinary, "best practice"guidelines in the care of spinal disorders in patients with skeletal dysplasia. Results: Starting with 33 statements, the group a developed a list of 31 "best practice"guidelines. Conclusions: The guidelines are presented and discussed to provide context for clinicians in their decision making in this often-challenging realm of care.

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KW - Cervical instability

KW - Kyphosis

KW - Myelopathy

KW - Scoliosis

KW - Skeletal dysplasia

KW - Spinal cord compression

KW - Spine

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Best practice guidelines for management of spinal disorders in skeletal dysplasia

Abstract

Keywords

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