Best practices in peri-operative management of patients with skeletal dysplasias

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

Research output: Contribution to journalArticle

Abstract

Patients with skeletal dysplasia frequently require surgery. This patient population has an increased risk for peri-operative complications related to the anatomy of their upper airway, abnormalities of tracheal-bronchial morphology and function; deformity of their chest wall; abnormal mobility of their upper cervical spine; and associated issues with general health and body habitus. Utilizing evidence analysis and expert opinion, this study aims to describe best practices regarding the peri-operative management of patients with skeletal dysplasia. A panel of 13 multidisciplinary international experts participated in a Delphi process that included a thorough literature review; a list of 22 possible care recommendations; two rounds of anonymous voting; and a face to face meeting. Those recommendations with more than 80% agreement were considered as consensual. Consensus was reached to support 19 recommendations for best pre-operative management of patients with skeletal dysplasia. These recommendations include pre-operative pulmonary, polysomnography; cardiac, and neurological evaluations; imaging of the cervical spine; and anesthetic management of patients with a difficult airway for intubation and extubation. The goals of this consensus based best practice guideline are to provide a minimum of standardized care, reduce perioperative complications, and improve clinical outcomes for patients with skeletal dysplasia.

Original languageEnglish (US)
JournalAmerican Journal of Medical Genetics, Part A
DOIs
StateAccepted/In press - 2017

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Practice Guidelines
Spine
Airway Extubation
Perioperative Care
Polysomnography
Expert Testimony
Thoracic Wall
Politics
Intubation
Anesthetics
Anatomy
Lung
Health
Population

Keywords

  • Perioperative management
  • Skeletal dysplasias

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

Cite this

Best practices in peri-operative management of patients with skeletal dysplasias. / White, Klane K.; Bompadre, Viviana; Goldberg, Michael J.; Bober, Michael B.; Cho, Tae Joon; Hoover Fong, Julie E; Irving, Melita; Mackenzie, William G.; Kamps, Shawn E.; Raggio, Cathleen; Redding, Gregory J.; Spencer, Samantha S.; Savarirayan, Ravi; Theroux, Mary C.

In: American Journal of Medical Genetics, Part A, 2017.

Research output: Contribution to journalArticle

White, KK, Bompadre, V, Goldberg, MJ, Bober, MB, Cho, TJ, Hoover Fong, JE, Irving, M, Mackenzie, WG, Kamps, SE, Raggio, C, Redding, GJ, Spencer, SS, Savarirayan, R & Theroux, MC 2017, 'Best practices in peri-operative management of patients with skeletal dysplasias', American Journal of Medical Genetics, Part A. https://doi.org/10.1002/ajmg.a.38357
White, Klane K. ; Bompadre, Viviana ; Goldberg, Michael J. ; Bober, Michael B. ; Cho, Tae Joon ; Hoover Fong, Julie E ; Irving, Melita ; Mackenzie, William G. ; Kamps, Shawn E. ; Raggio, Cathleen ; Redding, Gregory J. ; Spencer, Samantha S. ; Savarirayan, Ravi ; Theroux, Mary C. / Best practices in peri-operative management of patients with skeletal dysplasias. In: American Journal of Medical Genetics, Part A. 2017.
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