Non-Union After Multiple Lumbar Fusion Surgeries in a Patient With Facioscapulohumeral Muscular Dystrophy: A Case Report and Review of the Literature

World Neurosurg. 2020 Jul:139:281-285. doi: 10.1016/j.wneu.2020.04.030. Epub 2020 Apr 18.

Abstract

Background: Facioscapulohumeral muscular dystrophy (FSHD) is a rare condition affecting 1/20,000 persons and the third most common muscular dystrophy condition, with an autosomal dominant pattern of inheritance characterized by progressive muscular weakness primarily involving the face, shoulder girdle, and upper arm. The condition is associated with atrophic musculature of the trunk and core leading to difficulties with gait, posture, and function. FSHD leaves as many as 20% of patients wheelchair-bound and most commonly presents with low back, neck, and shoulder pain.

Case description: We present the case of a patient with FSHD who underwent multiple spinal fusion surgeries without relief in her back pain. Imaging studies serve to highlight the extent of paraspinal muscle atrophy and provides the basis for a discussion on the preoperative factors that may predict patients most likely to benefit from surgery. We then provide a brief review of the literature on the role of paraspinal muscle atrophy in back pain.

Conclusions: This case adds to our understanding of the surgical management of patients with FSHD and patients with atrophic core musculature as a whole.

Keywords: Case report; FSHD; Facioscapulohumeral muscular dystrophy; Failed back surgery.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Back Pain / etiology
  • Back Pain / surgery
  • Failed Back Surgery Syndrome / pathology*
  • Female
  • Humans
  • Lumbar Vertebrae / surgery
  • Middle Aged
  • Muscular Atrophy / etiology
  • Muscular Atrophy / pathology
  • Muscular Dystrophy, Facioscapulohumeral / complications
  • Muscular Dystrophy, Facioscapulohumeral / pathology*
  • Paraspinal Muscles / pathology*
  • Spinal Fusion