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Am J Med Genet A. 2017 Oct;173(10):2584-2595. doi: 10.1002/ajmg.a.38357. Epub 2017 Aug 1.

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

Author information

1
Department of Orthopedics and Sports Medicine, Seattle Children's Hospital, Seattle, Washington.
2
Division of Genetics, Nemour's Alfred I du Pont Hospital for Children, Wilmington, Delaware.
3
Division of Pediatric Orthopaedics, Seoul National University Children's Hospital, Seoul, South Korea.
4
McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University, Baltimore, Maryland.
5
Department of Clinical Genetics, Guy's and St Thomas NHS, London, United Kingdom of Great Britain and Northern Ireland.
6
Department of Orthopedic Surgery, Nemour's Alfred I du Pont Hospital for Children, Wilmington, Delaware.
7
Department of Radiology, Seattle Children's Hospital, Seattle, Washington.
8
Department of Orthopedic Surgery, Hospital for Special Surgery, New York, New York.
9
Division of Pulmonary Medicine, Seattle Children's Hospital, Seattle, Washington.
10
Department of Orthopedic Surgery, Boston Children's Hospital, Boston, Massachusetts.
11
Victorian Clinical Genetics Services, Murdoch Children's Research Institute, University of Melbourne, Parkville, Victoria, Australia.
12
Department of Anesthesia, Nemour's Alfred I du Pont Hospital for Children, Wilmington, Delaware.

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.

KEYWORDS:

perioperative management; skeletal dysplasias

PMID:
28763154
DOI:
10.1002/ajmg.a.38357
[Indexed for MEDLINE]

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