This 2018 expert consensus updates the 2007 standard-of-care guidelines for spinal muscular atrophy using international Delphi working groups. Part 1 covers diagnosis, rehabilitation, orthopedic management, and nutrition. It directly addresses the impact of nusinersen approval on surgical planning.
SMA patients who once had limited life expectancy are now surviving and improving on nusinersen, approved in December 2016. This means orthopedic surgeons are operating on a population whose long-term drug delivery needs must be built into the surgical plan from day one.
When you plan posterior spinal fusion for any SMA patient, confirm that one to two mid-lumbar levels will remain unexposed in the midline. This is a consensus requirement, not optional, and failing to do so may permanently eliminate intrathecal access.
For scoliosis surveillance, start spine radiographs when curves exceed 20 degrees and follow every 6 months. Move to surgical discussion at 50 degrees or 10 degrees of progression per year. In children under 8-10 years, choose growth-friendly instrumentation. In patients 12 or older, proceed to definitive posterior fusion.
For fracture management, remember that bone fragility in SMA is not purely from disuse. SMN protein has a direct role in bone remodeling via osteoclast stimulatory factor. Annual DEXA and Vitamin D monitoring are standard, and cast immobilization beyond 4 weeks in non-ambulatory patients actively worsens their osteoporosis.
This 2018 expert consensus updates the 2007 standard-of-care guidelines for spinal muscular atrophy using international Delphi working groups. Part 1 covers diagnosis, rehabilitation, orthopedic management, and nutrition. It directly addresses the impact of nusinersen approval on surgical planning.
SMA patients who once had limited life expectancy are now surviving and improving on nusinersen, approved in December 2016. This means orthopedic surgeons are operating on a population whose long-term drug delivery needs must be built into the surgical plan from day one.
When you plan posterior spinal fusion for any SMA patient, confirm that one to two mid-lumbar levels will remain unexposed in the midline. This is a consensus requirement, not optional, and failing to do so may permanently eliminate intrathecal access.
For scoliosis surveillance, start spine radiographs when curves exceed 20 degrees and follow every 6 months. Move to surgical discussion at 50 degrees or 10 degrees of progression per year. In children under 8-10 years, choose growth-friendly instrumentation. In patients 12 or older, proceed to definitive posterior fusion.
For fracture management, remember that bone fragility in SMA is not purely from disuse. SMN protein has a direct role in bone remodeling via osteoclast stimulatory factor. Annual DEXA and Vitamin D monitoring are standard, and cast immobilization beyond 4 weeks in non-ambulatory patients actively worsens their osteoporosis.