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Surgical Management of Thoracolumbar Kyphosis in Patients With Mucopolysaccharidosis

A Systematic Review

Williams, Nicole, BMed, FRACS; Cundy, Peter J., MBBS, FRACS; Eastwod, Deborah M., MB, ChB, FRCS

doi: 10.1097/BRS.0000000000002242

Study Design. Systematic review.

Objective. To determine the indications and outcomes for surgical treatment of thoracolumbar kyphosis in patients with mucopolysaccharidoses (MPS) to define future studies.

Summary of Background Data. Improvements in the medical treatment of MPS have increased lifespan and improved quality of life for many patients, but with no effect on thoracolumbar kyphosis. A greater number of these challenging patients may now be considered spinal surgical candidates.

Methods. In accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, a PubMed database search was conducted using relevant keywords to identify articles describing MPS patients with thoracolumbar kyphosis treated surgically. Information was collected regarding demographics, indications for surgery, surgical details and outcomes.

Results. Sixteen articles describing 58 patients were included for review. The median age at surgery was 7 years (range 2.4–65 years) and median follow up was 3.7 years (range 7 weeks–10.3 years). The most commonly reported surgical indication was progression of deformity in 39/58 patients (67%). Preoperative neurological compromise was reported in 7/58 (12%) patients and resolved completely postoperatively in all pediatric patients. Three pediatric patients had died before final follow up. Postoperative paraplegia was reported in 2/51 patients who were neurologically intact preoperatively. Additional significant perioperative medical complications were reported in nine patients.

Conclusion. Thoracolumbar spinal surgery is most commonly performed in MPS I. Preoperative neurological compromise associated with thoracolumbar kyphosis was reported only in MPS IV and VI, where it was associated with factors other than the degree of kyphosis. Perioperative complications are common, may be catastrophic and relate to the complexity of the MPS condition. The indications for thoracolumbar kyphosis surgery in other conditions of short stature cannot be extrapolated to MPS patients.

Level of Evidence: 4

Department of Orthopedic Surgery, Women's and Children's Hospital, North Adelaide, South Australia, Australia

Department of Orthopedic Surgery, Great Ormond Street Hospital, London, United Kingdom.

Address correspondence and reprint requests to Nicole Williams, BMed FRACS, Department of Orthopedic Surgery, Women's and Children's Hospital, 72 King William Rd, North Adelaide, South Australia 5006, Australia; E-mail:

Received 14 February, 2017

Accepted 13 April, 2017

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

Relevant financial activities outside the submitted work: grants, payment for lecture, and travel, accommodations, and meeting expenses.

Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.