Selective Spinal Fusion for Neuromuscular Scoliosis in a Patient with Pompe Disease: A Case Report and Review of the Literature

JBJS Case Connect. 2017 Jan-Mar;7(1):e15. doi: 10.2106/JBJS.CC.16.00137.

Abstract

Case: A 16-year-old girl with Pompe disease underwent surgery for scoliosis. She had been able to walk without any assistance, and kept her balance by swinging her waist. Therefore, we performed posterior selective spinal correction and fusion to avoid any adverse effects on walking ability that could occur with immobilization of the lumbosacral spine. After surgery, she was highly satisfied with her ability to perform the activities of daily living.

Conclusion: For nonambulatory patients with scoliosis and Pompe disease, long fusion from the upper thoracic spine to the pelvis is generally required. However, in ambulatory patients, in order to maintain the ability to walk, selective spinal fusion is an alternative.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Female
  • Glycogen Storage Disease Type II / complications*
  • Humans
  • Neuromuscular Diseases / etiology
  • Neuromuscular Diseases / surgery*
  • Scoliosis / etiology
  • Scoliosis / surgery*
  • Spinal Fusion / methods*
  • Treatment Outcome