Best practices in peri‐operative management of patients with skeletal dysplasias. Issue 10 (1st August 2017)

• Record Type: Journal Article
• Authors: 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.
• Is Part Of: American journal of medical genetics. Volume 173:Issue 10(2017)
• Page Start: 2584
• Page End: 2595
• Publication Date: 2017-08-01
• Subjects: perioperative management -- skeletal dysplasias; Medical genetics -- Periodicals; 616.14205
• DOI: 10.1002/ajmg.a.38357
• Languages: English
• ISSNs: 1552-4825

Abstract

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.