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Successful Management of a Patient With X-Linked Myotubular Myopathy for Scoliosis Surgery and Previous Cardiac Arrest After Prone Positioning: A Case Report

Flaherty, Devon, C., MD*; Lonner, Baron, MD; Gal, Jonathan, S., MD*

doi: 10.1213/XAA.0000000000000719
Case Reports

A 15-year-old boy with X-linked myotubular myopathy associated with severe hypotonia and pectus excavatum presented for posterior spinal fusion of T2-sacrum because of rest pain and severe progressive neuromuscular scoliosis. Previously, he experienced 2 separate instances of cardiac arrest after prone positioning under general anesthesia. A preoperative computed topography angiogram in the supine and prone positions revealed inferior vena cava and right ventricular outflow tract obstruction on prone positioning. Successful positioning and posterior spinal fusion occurred by staging the procedure, correction of volume status, early use of vasoactive and inotropic agents, and oblique prone positioning.

From the Departments of *Anesthesiology, Perioperative, and Pain Medicine

Orthopedics, Icahn School of Medicine at Mount Sinai Hospital, New York, New York.

Accepted for publication December 11, 2017.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Devon C. Flaherty, MD, Department of Anesthesiology, Perioperative, and Pain Medicine, Icahn School of Medicine at Mount Sinai Hospital, Box 1010, One Gustave L. Levy Pl, New York, NY 10029. Address e-mail to

© 2018 International Anesthesia Research Society
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