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Related Concept Videos

Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

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The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
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Modified Posterior Vertebral Column Resection for Patients with Thoracolumbar Kyphotic Deformity
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Spinal Deformity in Bethlem Myopathy.

Ozgur Dede1, Hoda Z Abdel-Hamid2, Vincent F Deeney1

  • 1Department of Orthopaedic Surgery, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.

Spine Deformity
|December 9, 2016
PubMed
Summary

Bethlem myopathy, a condition causing muscle weakness, can present with severe scoliosis. Surgical correction of spinal deformity in these patients showed positive outcomes without impacting pulmonary function.

Keywords:
Muscular dystrophyScoliosisSyndromic

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Area of Science:

  • Neuromuscular disorders
  • Orthopedic surgery
  • Spinal deformity

Background:

  • Bethlem myopathy is characterized by mild muscle weakness and a generally benign clinical course.
  • Severe scoliosis has not been previously documented in patients with Bethlem myopathy.

Purpose of the Study:

  • To describe the clinical and radiographic characteristics of spinal deformity in three patients diagnosed with Bethlem myopathy.
  • To highlight the potential association between Bethlem myopathy and severe scoliosis.

Main Methods:

  • A retrospective review of clinical and radiographic data was conducted for three brothers diagnosed with Bethlem myopathy.
  • Detailed analysis of spinal deformity features and outcomes following surgical intervention.

Main Results:

  • All three patients exhibited progressive scoliosis, presenting with both coronal and sagittal spinal imbalance.
  • Following posterior instrumented fusion, patients experienced no complications, maintained deformity correction for at least 26 months, and showed no negative impact on pulmonary function.

Conclusions:

  • Severe scoliosis, in conjunction with proximal muscle weakness, should be considered a potential manifestation of Bethlem myopathy.
  • Bethlem myopathy should be included in the differential diagnosis for adolescent patients presenting with progressive spinal deformity.