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Surgical treatment options for spasticity in children and adolescents with hereditary spastic paraplegia
Laura A van de Pol1,2, Nina Burgert3, Petra E M van Schie3
1Department of Child Neurology, Amsterdam UMC, Vrije Universiteit, De Boelelaan 1117, Amsterdam, The Netherlands. l.vandepol@amsterdamumc.nl.
Insights
Selective dorsal rhizotomy (SDR) and intrathecal baclofen pump implantation (ITB) show positive outcomes for children with hereditary spastic paraplegia (HSP). SDR benefits walking patients, while ITB suits severe, progressive cases, with low complication risks.
Area of Science:
- Neurology
- Pediatric Orthopedics
- Neurosurgery
Background:
- Hereditary spastic paraplegia (HSP) is a group of inherited neurological disorders characterized by progressive lower-limb spasticity.
- Treatment options for spasticity in children with HSP aim to improve motor function and quality of life.
Purpose of the Study:
- To evaluate the outcomes and complications of selective dorsal rhizotomy (SDR) and intrathecal baclofen pump implantation (ITB) in pediatric patients with HSP.
- To assess the impact of these interventions on gross motor function and spasticity levels.
Main Methods:
- Retrospective study analyzing data from children diagnosed with HSP who underwent either SDR or ITB.
- Assessment of Gross Motor Function Measure (GMFM-66) scores and spasticity levels before and after surgical intervention.
- Analysis of patient demographics, genetic mutations, and surgical details.
Main Results:
- Ten pediatric patients with HSP were included, with mutations in ATL1 and SPAST genes being most common.
- Six patients underwent SDR, three ITB, and one had both procedures. Five SDR patients achieved independent walking or used walking aids.
- Postoperative spasticity reduction was observed in all patients, with a mean GMFM-66 score increase of +8.0. No surgical complications were reported.
Conclusions:
- Selective dorsal rhizotomy (SDR) is a viable and safe treatment for carefully selected pediatric patients with HSP, particularly those who are ambulatory.
- Intrathecal baclofen pump implantation (ITB) proved effective for managing spasticity in children with severe and progressive HSP.
- Both SDR and ITB demonstrated significant benefits in improving gross motor function with a low risk of complications.
Purpose:
To provide an overview of outcome and complications of selective dorsal rhizotomy (SDR) and intrathecal baclofen pump implantation (ITB) for spasticity treatment in children with hereditary spastic paraplegia (HSP).
Methods:
Retrospective study including children with HSP and SDR or ITB. Gross motor function measure (GMFM-66) scores and level of spasticity were assessed.
Results:
Ten patients were included (most had mutations in ATL1 (n = 4) or SPAST (n = 3) genes). Four walked without and two with walking aids, four were non-walking children. Six patients underwent SDR, three patients ITB, and one both. Mean age at surgery was 8.9 ± 4.5 years with a mean follow-up of 3.4 ± 2.2 years. Five of the SDR patients were walking. Postoperatively spasticity in the legs was reduced in all patients. The change in GMFM-66 score was + 8.0 (0-19.7 min-max). The three ITB patients treated (SPAST (n = 2) and PNPLA6 (n = 1) gene mutation) were children with a progressive disease course. No complications of surgery occurred.
Conclusions:
SDR is a feasible treatment option in carefully selected children with HSP, especially in walking patients. The majority of patients benefit with respect to gross motor function, complication risk is low. ITB was used in children with severe and progressive disease.
