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Selective Dorsal Rhizotomy in Children with Hereditary Spastic Paraplegia
Amanda N Stanton1,2, Nathan S Fredricks3, Anthony M Price4
1Department of Neurosurgery, University of Florida Health Shands, Gainesville, Florida, USA.
Insights
Selective Dorsal Rhizotomy (SDR) significantly reduced spasticity in children with hereditary spastic paraplegia (HSP). Patients showed improved mobility, quality of life, and reduced need for orthotics after the procedure.
Area of Science:
- Neurosurgery
- Pediatric Orthopedics
- Genetics
Background:
- Selective Dorsal Rhizotomy (SDR) is known to improve spasticity, but its efficacy in hereditary spastic paraplegia (HSP) is not well-established.
- This study investigates the outcomes of SDR in pediatric patients diagnosed with HSP.
Purpose of the Study:
- To evaluate the effectiveness of SDR in reducing spasticity and improving functional outcomes in children with HSP.
- To assess the safety and tolerability of SDR in this specific patient population.
Main Methods:
- A retrospective chart review was conducted on pediatric patients (≤18 years) with HSP who underwent SDR.
- Standardized assessments including the Modified Ashworth Scale (MAS), Gross Motor Function Measure-66 (GMFM-66), and Pediatric Quality of Life (PedsQL) module were used.
- Genetic mutations (ATL1, SPAST) and HSP complexity were noted.
Main Results:
- Six pediatric patients with HSP (4-14 years) underwent SDR, with a median follow-up of 17.65 months.
- Post-SDR, median MAS decreased from 16 to 0, GMFM-66 improved from 74.8 to 79.1, and 10-Meter Walk Test (10MWT) times improved.
- Five patients reported improved PedsQL scores, with notable reductions in pain and fatigue; orthotic dependence decreased significantly.
Conclusions:
- SDR appears to be a safe and effective procedure for reducing spasticity in pediatric HSP patients.
- The intervention is associated with improvements in ambulation, functional mobility, quality of life, and decreased reliance on orthotic devices.
- Larger studies are warranted to confirm statistical significance and optimize patient selection for SDR in HSP.
Introduction:
While selective dorsal rhizotomy (SDR) improves spasticity, the benefit in hereditary spastic paraplegia (HSP) remains unclear. This study describes a single institution's outcomes and experience with SDR in children with HSP.
Methods:
We conducted a retrospective chart review of pediatric patients (≤18 years) with HSP who underwent SDR at Monroe Carell Jr. Children's Hospital between July 2013 and January 2024. Patients were evaluated with standardized assessments, including the Modified Ashworth Scale (MAS), Gross Motor Function Classification System (GMFCS), Gross Motor Function Measure-66 (GMFM-66), Manual Ability Classification System (MACS), Functional Mobility Scale (FMS), 10-Meter Walk Test (10MWT), Pediatric Balance Scale (PBS), and Pediatric Quality of Life (PedsQL) Cerebral Palsy Module.
Results:
Six patients (2 males, 4 females) aged 4-14 years underwent SDR, with a median follow-up of 17.65 months (range 11.8-38.9). Our cohort had four ATL1 and two SPAST mutations. Five patients had uncomplicated HSP, while 1 had complicated HSP. A median of 60% of rootlets were cut bilaterally (range 56-63). No major surgical complications occurred. Median MAS decreased from 16 preoperatively to 0 postoperatively. GMFM-66 improved from 74.8 to 79.1. In FMS testing, three patients achieved independent ambulation across all surfaces. 10MWT times improved from a median of 5.6 to 4.7 s. MACS scores improved in one patient and were otherwise stable. Balance scores remained stable or improved. Absolute PedsQL scores improved in 5 patients, with pain and fatigue showing greatest reduction. At follow-up, only 1 patient required orthotic support, compared to four preoperatively.
Conclusion:
SDR may offer marked reductions in spasticity, with associated improvements in ambulation, functional mobility, quality of life, and orthotic dependence. SDR was safe and well tolerated. Larger studies are needed to confirm statistical significance, define long-term efficacy, and optimize patient selection.
