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

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.

Best practice guidelines regarding prenatal evaluation and delivery of patients with skeletal dysplasia

“…6e8 This group has employed this methodology previously with the aim of improving standards of management, and stimulating further research to optimize health outcomes for individuals with skeletal dysplasia. 9,10 The lead author (R.S.) created a list of statements based on a systematic literature review.…”

Best Practice Guidelines in Managing the Craniofacial Aspects of Skeletal Dysplasia

“…Many patients have abnormalities in the development and growth of the craniofacial skeleton leading to restricted opening of the jaw and di culties in intubation. These risks are compounded by di cult venous access due to joint contractures and/or excess subcutaneous fat, a small thorax, obesity, and associated cardiac and other major organ disease 4 Polysomnography is a non-invasive test typically including an electroencephalogram, electro-oculogram, chin and leg electromyogram, electrocardiogram, and pulse oximetry, with assessments of oro-nasal air ow, abdominal and chest wall movements, and video recording. Measurement of partial pressure of carbon dioxide (PCO2) is recommended during standard pediatric polysomnography and is required to diagnose hypoventilation in adults and children.…”

Best practice guidelines regarding diagnosis and management of patients with type II collagen disorders

“…The Delphi methodology has been widely used in medical research to determine appropriate treatment and best practice guidelines, [4][5][6] and has been successfully used by this group previously. [7][8][9] The primary author (R.S.) created statements based on systematic literature review that were evaluated by the authors who edited the list.…”

“…The comorbidities, anatomic constraints, and perioperative issues seen in these patients need to be thoroughly considered prior to proceeding. 8 20. In type II collagen disorders with disproportionate short stature, coxa vara and genu valgum are frequently present Hip involvement in type II collagen disorders is very common with the typical deformity being coxa vara.…”