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[Selective dorsal rhizotomy in children with hereditary spastic paraplegia]
E I Smolyankina1, D Yu Zinenko1
1Veltischev Research Clinical Institute of Pediatrics and Pediatric Surgery, Moscow, Russia.
Insights
Selective Dorsal Rhizotomy (SDR) effectively reduced spasticity and improved lower limb movement in children with hereditary spastic paraplegia (HSP). This safe surgical option showed promising results, with sustained or increased mobility post-operation.
Area of Science:
- Neurosurgery
- Pediatric Neurology
- Genetics
Context:
- Hereditary spastic paraplegia (HSP), also known as Strumpell disease, is a group of inherited neurological disorders.
- HSP is characterized by progressive muscle stiffness and spasticity in the lower limbs, significantly impacting mobility and quality of life.
- Selective Dorsal Rhizotomy (SDR) is a surgical procedure aimed at reducing spasticity in the lower limbs.
Purpose:
- To evaluate the efficacy and safety of Selective Dorsal Rhizotomy (SDR) in pediatric patients diagnosed with hereditary spastic paraplegia (HSP).
- To analyze the impact of SDR on spasticity levels and lower limb motor function through pre-operative, immediate post-operative, and delayed post-operative assessments.
- To document any complications and observe the long-term functional outcomes following SDR in this patient cohort.
Summary:
- Eight pediatric patients with confirmed HSP underwent SDR between 2022 and 2024, with a mean age of 10.3 years.
- Assessments included spasticity testing and goniometry, revealing reduced spasticity and improved lower limb movements in most patients post-surgery.
- While three patients experienced movement limitations due to orthopedic deformities, overall functional levels were maintained or improved, with no reported complications during the mean 11-month follow-up.
Impact:
- SDR demonstrates significant potential as an effective and safe therapeutic intervention for managing spasticity in children with HSP.
- The findings support SDR as a viable treatment option, contributing to improved motor function and potentially enhancing the quality of life for affected children.
- Further long-term studies with larger patient cohorts are warranted to comprehensively understand the sustained benefits and optimal application of SDR in HSP management.
Objective:
To analyze the results of selective dorsal rhizotomy (SDR) in children with hereditary spastic paraplegia (Strumpell disease, HSP).
Material And Methods:
SDR was performed in 8 patients with genealogical or genetic verification of HSP between 2022 and 2024. Mean age of patients was 10.3±4.9 years. We analyzed the results via testing spasticity and goniometry before surgery, on the third postoperative day and in delayed postoperative period. Mean follow-up period was 11±7.5 months.
Results:
All children improved lower limb movements. There was regression of spasticity in most cases. Three children had significant impairment of movements in some joints due to orthopedic deformities. No regression of the post-surgery movements level and even increase later in some cases was observed. There were no complications.
Conclusion:
SDR is effective and safe in patients with HSP. Long-term follow-up of larger samples is required.
