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Respiratory Complications After Posterior Spinal Fusion for Neuromuscular Scoliosis

Children With Rett Syndrome at Greater Risk Than Those With Cerebral Palsy

Cohen, Jacob L. BS; Klyce, Walter BA; Kudchadkar, Sapna R. MD, PhD; Kotian, Ronak N. MBBS; Sponseller, Paul D. MD, MBA

doi: 10.1097/BRS.0000000000003075

Study Design. Retrospective cohort.

Objective. To determine how respiratory failure rates and duration of intensive care unit (ICU) stay after posterior spinal fusion (PSF) for neuromuscular scoliosis compare between children with Rett syndrome (RS) versus cerebral palsy (CP).

Summary of Background Data. Rett syndrome and CP are associated with high incidence of neuromuscular scoliosis and respiratory dysfunction.

Methods. We included 21 patients with RS (mean age, 13 ± 3.1 yrs) and 124 with CP (mean age, 14 ± 3.2 yrs) who underwent PSF by one surgeon from 2004 to 2017. Preoperative motor function was assessed using the Gross Motor Function Classification System (GMFCS). Primary outcomes were respiratory failure and duration of ICU stay. Secondary outcomes were pneumonia and prolonged use of positive pressure ventilation (PPV). Using multivariate regression, we identified associations of age, intraoperative vital signs, duration of hospital stay, number of vertebral levels fused, anesthesia and surgery durations, and estimated blood loss with longer ICU stay and respiratory failure.

Results. A greater proportion of CP patients (96%) than RS patients (66%) were in GMFCS IV or V (P < 0.01). Respiratory failure was more common in RS patients (43% vs. 19%; P = 0.02), as was PPV (67% vs. 31%; P < 0.01). RS patients had shorter median durations of anesthesia and surgery (P < 0.01). RS patients had a longer median (interquartile range) ICU stay (4 days [1–5] vs. 2 days [2–19]; P = 0.01). Incidence of pneumonia did not differ between groups (P = 0.69). Only RS diagnosis (P = 0.02) and prolonged PPV (P < 0.01) were associated with longer ICU stay.

Conclusion. Despite better preoperative motor function and shorter anesthesia and surgery durations, patients with RS experienced more respiratory failure, prolonged PPV use, and longer ICU stays after PSF than did children with CP.

Level of Evidence: 4

We investigated immediate postoperative outcomes of children with Rett syndrome versus cerebral palsy after undergoing posterior spinal fusion. Children with Rett syndrome had more respiratory failure, more need for positive pressure ventilation, and longer intensive care unit stays, despite better preoperative motor function and shorter surgeries.

Division of Pediatric Orthopaedics, Department of Orthopaedic Surgery, Bloomberg Children's Center, Baltimore, Maryland

Departments of Anesthesiology and Critical Care Medicine, Pediatrics and Physical Medicine and Rehabilitation, The Johns Hopkins University School of Medicine, Baltimore, Maryland.

Address correspondence and reprint requests to Paul D. Sponseller, MD, MBA, Bloomberg Children's Center, 1800 Orleans Street, 7359A, Baltimore, MD 21287; E-mail:

Received 1 February, 2019

Accepted 25 March, 2019

The manuscript submitted does not contain information about medical device(s)/drug(s).

No funds were received in support of this work.

No relevant financial activities outside the submitted work.

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.