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

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 pr...

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Bibliographic Details
Published inA&A practice Vol. 10; no. 12; p. 340
Main Authors Flaherty, Devon C, Lonner, Baron, Gal, Jonathan S
Format Journal Article
LanguageEnglish
Published United States 15.06.2018
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Summary: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.
ISSN:2575-3126
DOI:10.1213/XAA.0000000000000719