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Spinal Muscular Atrophy G12.9 335.10


synonyms: Spinal Muscular Atrophy, SMA

Spinal Muscular Atrophy ICD-10

Spinal Muscular Atrophy ICD-9

  • 335.10 Spinal muscular atrophy, unspecified

Spinal Muscular Atrophy ICD-10

  • G12.9  Spinal muscular atrophy, unspecifi

Spinal Muscular Atrophy Etiology / Epidemiology / Natural History

  • Caused by deletion in the Survival motor neuron I (SMN-I) gene in 95%-98% of patients

Spinal Muscular Atrophy Anatomy

Spinal Muscular Atrophy Clinical Evaluation

Spinal Muscular Atrophy Xray / Diagnositc Tests

  • diagnosed with a genetic testing for SMN-I defects

Spinal Muscular Atrophy Classification / Treatment

  • Genetic testing generally indicated
  • Hip subluxation / dislocation: associated with Spinal Muscular Atrophy TYpe II. Patients are generally non-ambulatory and have little pain or functional limitations. Hip subluxation.dislocation is best treated with observation. (Sporer SM, JPO 2003;23:10).
  • Scoliosis surgery:  recommended when curves progress beyond 50 to 60 degrees and are associated with impaired sitting balance and/or worsening pulmonary function. Generally posterior spinal fusion with instrumentation.  Combined anterior and posterior fusion for curves more than 100 degrees and very young patients (to prevent crankshaft phenomenon). Pulmonary morbidity is associated with an anterior procedures. 

Spinal Muscular Atrophy Associated Injuries / Differential Diagnosis

Spinal Muscular Atrophy Complications

Spinal Muscular Atrophy Follow-up Care

Spinal Muscular Atrophy Review References

  • Sucato DJ. Spine deformity in spinal muscular atrophy. J Bone Joint Surg Am. 2007 Feb;89 Suppl 1:148-54. Review. Erratum in: J Bone Joint Surg Am. 2007 May;89(5):1090-1.
  • Aprin H, Bowen JR, MacEwen GD, Hall JE. Spine fusion in patients with spinal muscular atrophy. J Bone Joint Surg Am. 1982 Oct;64(8):1179-87.
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