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

on behalf of the Skeletal Dysplasia Management Consortium

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)
Pages (from-to)2584-2595
Number of pages12
JournalAmerican Journal of Medical Genetics, Part A
Volume173
Issue number10
DOIs
StatePublished - Oct 2017

Keywords

  • perioperative management
  • skeletal dysplasias

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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