• Mary C Theroux, Martha Lopez, Patricia J Olszewsky, Sabina DiCindio, Lynda Arai, Colleen Ditro, Michael B Bober, Olubukola Opeyemi Olla, Tetsu Uejima, David W West, and William G Mackenzie.
• Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA.
• Paediatr Anaesth. 2017 Jun 1; 27 (6): 596-603.

BackgroundMetatropic dysplasia is a rare form of skeletal dysplasia requiring multiple anesthetics for surgical and imaging procedures, most of which are orthopedic procedures. We provide centralized care to patients with skeletal dysplasia at our tertiary care pediatric hospital, and we were able to collect the largest number of metatropic dysplasia patients reported to date.AimThe aim of this retrospective study was to describe and characterize the anesthetic difficulties in this high-risk population.MethodsMedical charts of all patients with metatropic dysplasia were reviewed to collect data, including anesthetics performed, difficulties, and complications related to the anesthetic care, co-morbid conditions, and related events.ResultsTwenty-three patients with metatropic dysplasia underwent 188 anesthetics with 61% of the anesthetics having been administered for orthopedic procedures. Fourteen of 23 (60.8%) progressively became difficult to intubate over the course of their care, with 12 out of 14 having undergone cervical spine fusion. These 14 patients had a total of 133 procedures. Sixty procedures (45.1%) had an airway described as difficult. Glidescope was the difficult airway tool most commonly used (68%) with flexible fiberoptic scope used 12% and Miller or Macintosh blade used 18% of the time. In addition to the airway difficulties, spinal canal narrowing or stenosis was widely prevalent, and no neuraxial anesthetic was performed in any of our patients.ConclusionDifficult airway is the most common co-morbid condition present in patients with metatropic dysplasia, especially if their cervical spine has been fused. Familiarity with the difficulties involving the airway and its management is critical in safe and successful management of anesthesia in this high-risk population.© 2017 John Wiley & Sons Ltd.

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