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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.
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.
