Abstract
This is a retrospective radiographic review to assess post-operative sagittal plane deformities in patients with Spinal Muscular Atrophy type 2 that had been treated with posterior spinal instrumentation. Thirty-two patients with a history of either spinal fusion (N = 20) or growing rods (N = 12) were identified with an average of 7.6 (2.1–16.6) years post-operative follow-up. Forty percent (13/32) of the patients were identified as having obvious “tucked chin” (N = 4), “tipped trunk” (N = 9), or both (N = 3). Sacral incidence was the only parameter that was statistically significant change between pre-operative or immediate post-operative measurements (66.9° vs. 55.2° p = 0.03). However, at final follow-up, the post-operative thoracic kyphosis had decreased over time in those that developed a subsequent sagittal deformity (24.2°) whereas it increased in those that did not (44.7°, p = 0.008). This decrease in thoracic kyphosis throughout the instrumented levels, resulted in a greater lordotic imbalance (30.4° vs. 5.6°, p = 0.001) throughout the instrumented levels in the group that developed the subsequent cervical or pelvic sagittal deformities. In conclusion, sagittal plane deformities commonly develop outside the instrumented levels in children with SMA type 2 following posterior spinal instrumentation and may be the result of lordotic imbalance that occurs through continued anterior growth following posterior instrumentation.
Highlights
Spinal Muscular Atrophy (SMA) is classified into three types based on the onset of disease: type 1 has symptoms starting before 6 months of age, type 2 has onset between 6–18 months of age, and type 3 has onset after 18 months of age [1]
Scoliosis is common in all types of spinal muscular atrophy and spinal deformities occur earlier with increased disease severity (Type 1 < 2 years of age, Type 2: 1–7 years of age, Type 3: 4–14 years of age) [12,13]
Type 2 patients and provide radiographic parameters to assess for these deformities. These patients appear very sensitive to a lordotic imbalance that develops following posterior spinal instrumentation resulting in cervical kyphosis or anterior tipping of the trunk
Summary
Spinal Muscular Atrophy (SMA) is the most common fatal genetic disease affecting the pediatric population (1 in 6–10,000 live births). Before the widespread use of disease modifying agents, children with this disease experienced progressive weakness and early mortality. SMA is classified into three types based on the onset of disease: type 1 has symptoms starting before 6 months of age, type 2 has onset between 6–18 months of age, and type 3 has onset after 18 months of age [1]. Children with type 1 never sit and without intervention have a life expectancy
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