Surgical treatment options for spasticity in children and adolescents with hereditary spastic paraplegia.

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.

Impact of selective dorsal rhizotomy to cerebral palsy children caregivers' burden.

PURPOSE: It is known that cerebral palsy (CP) children's caregivers suffer from burden, depression, and stress, impairing their quality of life (QoL). The more severe the CP, the more burden the caregiver has. Psychosocial support, education, therapies, and financial support are inversely related to the level of stress of the caregiver. Most parents of CP patients submitted to selective dorsal rhizotomy (SDR) report improvement not just on spasticity, but also in the functional role of the children, what can impact on caregiver's QoL. Our objective was to evaluate the burden of CP children's caregivers with and without previous SDR. METHODS: Spastic CP children caregivers were divided into two groups: those who take care of children without previous SDR (control group) and those that children were previously submitted to SDR (surgical group). The burden index was compared between groups using Burden Interview Questionnaire (BIQ). For statistical analysis, we used SPSS. RESULTS: The control group had enrolled 31 participants and the surgical group 36. The mean GMFCS level on the control and surgical groups was 3.94 ± 1.26 and 3.74 ± 1.12 (p = 0.61), respectively. The surgical group caregivers presented less burden related to the feeling that they should be doing more to their child (p = 0.003) and if they could do a better job in caring (p = 0.032), compared to controls. The total BIQ index was not significantly different between groups (surgical 32.14 ± 12.34 vs. control 36.77 ± 12.77; p = 0.87). Low economic status had a weak correlation to a higher BIQ index (R2 = 0.24). After age-matching, there was a significative higher BIQ index in the control group (p = 0.008). CONCLUSION: Caregivers of spastic CP children who were previously submitted to SDR presented less burden related to feeling of the amount of given care than those without previous surgery. The impression that they could do a better job with their kids was higher in the control group. The severity of CP and low economic status were related to more burden in both groups. After pairing groups by age, the control group had a significative higher BIQ index compared to the SDR group. CLINICAL TRIAL REGISTRATION: Trial registration number: CAAE 73407317.6.0000.0068 (Ethical and Research Committee of University of Sao Paulo, Sao Paulo, Brazil, approved on 08/06/2021). All the subjects were freely given an informed consent to participate in the study that was obtained from all participants. Non-consented ones were excluded from the study.

Transcutaneous ultrasound level check for selective dorsal rhizotomy.

AIM: Single-level selective dorsal rhizotomy (SDR) surgery requires an intra-operative level check to identify the L1 vertebral level or the conus medullaris. Typically, this requires a pre-operative or intra-operative x-ray. We present our experience using initial transcutaneous ultrasound as an alternative to x-ray level check. METHODS: A prospective SDR database was used to identify patients. The operation notes were reviewed to identify the level check method and any complications or wrong-level surgery. RESULTS: Data are reported for the first 160 SDR surgeries performed within our centre, mean age 6.47 years (range 2.5-19 years). The first 11 patients had combined x-ray and transcutaneous ultrasound for pre-incision level check. This allowed the neurosurgeon to assess the accuracy and feasibility of using transcutaneous ultrasound instead of x-ray. The subsequent 149 patients had ultrasound alone for transcutaneous pre-incision level check. An intra-operative ultrasound level check was performed for all patients following skin incision and dissection down to the spinal lamina. In this way, the conus level was confirmed before dural opening. For all patients at all ages, the combination of initial transcutaneous ultrasound followed by intra-operative ultrasound allowed accurate identification of the conus. There were no instances of wrong-level surgery. Learning points are presented within this paper. CONCLUSION: Combined use of transcutaneous ultrasound followed by intra-operative ultrasound can allow accurate identification of the conus, saving radiation exposure and potentially improving theatre efficiency. Appropriate training and experience are required for any neurosurgeon using these techniques.

Gabapentin as part of a multimodal pain protocol for selective dorsal rhizotomy does not impact percentage of rootlets transected.

PURPOSE: We aim to determine whether preoperatively initiated gabapentin for pain control impacts the percentage of rootlets cut during monitored, limited laminectomy selective dorsal rhizotomy (SDR) procedure. METHODS: This retrospective cohort study includes participants with cerebral palsy who had SDR for treatment of spasticity between 2010 and 2019 at a single-institution tertiary care center. One-level laminectomy SDR aimed to evaluate the cauda equina roots from levels L2-S1 with EMG monitoring. Gabapentin titration began 3 weeks prior to SDR. Data was analyzed using simple linear regression. Thirty-one individuals met inclusion criteria. Mean age was 7 years, 4 months. Eighteen participants (58%) identified as male, 12 (39%) female, and one (3%) non-binary. Thirty (97%) had bilateral CP. Sixteen (52%) were GMFCS II, four (13%) GMFCS III, five (16%) GMFCS IV, and six (19%) GMFCS V. RESULTS: Mean percentage of rootlets transected was 50.75% (SD 6.00, range 36.36-60.87). There was no relationship between the dose of gabapentin at time of SDR and percentage of rootlets cut with a linear regression slope of - 0.090 and an R2 of 0.012 (P = 0.56). CONCLUSION: Results indicate that preoperative initiation of gabapentin did not impact the percentage of rootlets transected. Thus, gabapentin can be initiated prior to SDR at moderate dosages without impacting SDR surgical outcomes.

The effect of selective dorsal rhizotomy on the improvement of the quality of life of children with stage GMFCS IV and V cerebral palsy: Pain, nursing, positioning, and dressing.

AIM: The objective of this study is to evaluate the benefit of selective dorsal rhizotomy on the quality of life of patients with severe spasticity with significant impairment of gross motor functions (GMFCS stages IV and V) according to 4 items: pain, nursing care, positioning, and dressing. MATERIALS AND METHODS: We conducted a monocentric retrospective cohort study including patients who underwent selective dorsal rhizotomy between March 2008 and May 2022 at the University Hospital of Marseille. RESULTS: Seventy percent of patients showed an improvement in quality of life criteria: dressing, nursing, positioning, and pain at the last follow-up. A small proportion of patients still showed a worsening between the first 2 follow-ups and the last follow-up. Postoperatively, 27.3% of patients were free of joint spasticity treatment, and we have shown that there was a significant decrease in the number of children who received botulinum toxin postoperatively. However, there was no significant reduction in the number of drug treatments or orthopaedic procedures following RDS. For the CPCHILD© scores, an overall gain is reported for GMFCS IV and V patients in postoperative care. The gain of points is more important for GMFCS IV patients. Improvement was mainly observed in 2 domains, "comfort and emotions" and "hygiene and dressing". For the "quality of life" item, only 3 parents out of the 8 noted a positive change. CONCLUSION: Our study shows an improvement in nursing care, positioning, and dressing which are associated with a reduction in pain in children with a major polyhandicap GMFCS IV and V who have benefited from a selective dorsal rhizotomy.

Single-level laminoplasty approach to selective dorsal rhizotomy with conus localization by rapid spine MRI.

INTRODUCTION: While selective dorsal rhizotomy (SDR) was originally described as a multilevel approach, single-level approaches are now popularized. Conus localization is beneficial for operative planning in single-level selective dorsal rhizotomy. Our approach to SDR involves minimal exposure for a single-level laminoplasty, preserving one attached interspinous ligament. Pre-operative conus localization is required for this tailored approach to determine the laminoplasty level and dictate rostral or caudal division of the superior spinus ligament. While rapid MRI sequences have been popularized for pediatric cranial imaging, its utility for spinal imagining is less well-described, and specific application for conus localization has not been reported. OBJECTIVE: Illustrate that rapid MRI without sedation is sufficient to identify conus level for tailored single-level laminoplasty SDR. MATERIAL AND METHODS: Patients undergoing SDR from 2014 to 2022 at one institution were reviewed for type of pre-operative MRI (rapid vs full), conus level, procedural time for MRI, and radiology report. The typical rapid MRI has four sequences utilizing single-shot technique (scout, sagittal T2, axial T2, and axial T1) that typically take less than 1 min each of acquisition time, with non-single-shot sequences added periodically in cooperative patients. To include time for patient positioning, pre-scan shimming, procedural incidentals, and other patient-specific variations, MRI procedure length was recorded as documented in the electronic medical record. RESULTS: N = 100 patients had documentation of an MRI for pre-operative imaging. Seventy-nine of these had a rapid MRI, and 21 required a full MRI with anesthesia for their treatment plan. Mean total procedure time for rapid MRI was 21.5 min (median 17). Mean procedure time for MRI under general anesthesia was 91.2 min (median 94). Of patients with rapid MRI imaging, 2/79 had an ambiguous conus level (1 from motion artifact, 1 from spinal hardware) vs 1/21 with a full MRI under anesthesia (due to spinal hardware). CONCLUSION: Rapid spinal MRI without sedation can be used for conus localization in a pediatric population. This may be routinely used as pre-operative imaging for a single-level approach to selective dorsal rhizotomy, without sedation or intubation procedures.

History and evolution of surgical treatment for spasticity: a journey from neurotomy to selective dorsal rhizotomy.

The evolution of neurosurgical approaches to spasticity spans centuries, marked by key milestones and innovative practitioners. Probable ancient descriptions of spasmodic conditions were first classified as spasticity in the 19th century through the interventions of Dr. William John Little on patients with cerebral palsy. The late 19th century witnessed pioneering efforts by surgeons such as Dr. Charles Loomis Dana, who explored neurotomies, and Dr. Charles Sherrington, who proposed dorsal rhizotomy to address spasticity. Dorsal rhizotomy rose to prominence under the expertise of Dr. Otfrid Foerster but saw a decline in the 1920s due to emerging alternative procedures and associated complications. The mid-20th century saw a shift toward myelotomy but the revival of dorsal rhizotomy under Dr. Claude Gros' selective approach and Dr. Marc Sindou's dorsal root entry zone (DREZ) lesioning. In the late 1970s, Dr. Victor Fasano introduced functional dorsal rhizotomy, incorporating electrophysiological evaluations. Dr. Warwick Peacock and Dr. Leila Arens further modified selective dorsal rhizotomy, focusing on approaches at the cauda equina level. Later, baclofen delivered intrathecally via an implanted programmable pump emerged as a promising alternative around the late 1980s, pioneered by Richard Penn and Jeffrey Kroin and then led by A. Leland Albright. Moreover, intraventricular baclofen has also been tried in this matter. The evolution of these neurosurgical interventions highlights the dynamic nature of medical progress, with each era building upon and refining the work of significant individuals, ultimately contributing to successful outcomes in the management of spasticity.

Palliative single-level selective dorsal rhizotomy for children with spastic cerebral palsy Gross Motor Function Classification System level IV and V: a case series and systematic review of the literature.

OBJECTIVE: Single-level selective dorsal rhizotomy (SDR), typically indicated for ambulatory patients, is a controversial topic for severe spastic cerebral palsy (CP) with Gross Motor Function Classification System (GMFCS) level IV or V. The objective of this case series and systematic literature review was to outline the indication and outcome of palliative SDR for nonambulatory patients with CP and GMFCS level IV and V, focusing on improvement of spasticity and of patient and caregiver reported quality of life assessment. METHODS: A retrospective case series of patients with CP and GMFCS level IV or V who underwent single-level SDR at the authors' institution is presented. Furthermore, two databases (PubMed and Embase) were searched and a systematic review with a search string based on the terms "selective dorsal rhizotomy," "cerebral palsy," and "outcome" was conducted. The primary outcome was the reduction of spasticity based on the modified Ashworth scale (MAS). Secondary outcomes were change on the Gross Motor Function Measure-66 (GMFM-66), evaluation of patient-reported outcome measures (PROMs), surgical morbidity, and mortality. RESULTS: Eleven consecutive children under the age of 25 years undergoing palliative single-level SDR were included. All patients showed a reduction in MAS score (mean 1.09 ± 0.66 points) and no surgical morbidity and mortality occurred. For the systematic review results from our case series, in addition to 4 reports, 274 total patients were included. Reduction of spasticity based on MAS score was noted in all studies (mean range 1.09-3.2 points). Furthermore, in 2 studies spasticity of the upper extremities showed a MAS score reduction as well (range 1.7-2.8 points). The GMFM-66 score improved in 72% of the patients, while bladder function improved in 78% of the patients. Based on the PROMs, 92% of the patients/caregivers were satisfied with the outcome and their quality of life after the procedure. Two wound infections (2.7%) and one CSF leak (1.3%) occurred, while no surgery-related deaths were described. CONCLUSIONS: This analysis showed an improvement in spasticity, daily care, and comfort for patients with CP and GMFCS levels IV and V. Larger cohorts analyzing the outcome of palliative single-level SDR, based on the MAS, GMFM-66, and PROMs, are still needed and should be the focus of future studies. Systematic review registration no.: CRD42024495762 (https://www.crd.york.ac.uk/prospero/).

Postoperative outcomes and stimulation responses for sectioned nerve roots during selective dorsal rhizotomy in cerebral palsy.

BACKGROUND: Cerebral palsy (CP) is the most cause of motor dysfunction in children. Selective dorsal rhizotomy (SDR) plays a major role in long term spasticity control. However, limited data exists on the effect of SDR on postoperative spasticity treatment requirements and supraspinal effects, and the stimulation responses of dorsal nerve roots in those with CP. METHODS: The current study included the outcome for 35 individuals undergoing SDR for motor functional outcome, spasticity, baclofen dose changes, botulinum toxin injection frequency, and spasticity related orthopedic procedures. We also report on the stimulation responses in 112 individuals who underwent SDR at our institution. RESULTS: There was a significant difference in gross motor function measures (GMFM)-66 scores at last follow up that remained present when considering only ambulatory children but not with non-ambulatory children. Ashworth scores were significantly decreased for both upper and lower extremities after SDR at all follow up points. There was a significant decrease in Baclofen dose and botulinum toxin injections requirements after SDR, but no significant difference in the need for orthopedic intervention. A total of 5502 dorsal nerve roots were tested showing a decrease in stimulation intensity and increase in grade on the right side and for descending lumbosacral levels. CONCLUSIONS: SDR improves gross motor scores during short term follow up but has additional benefits in decreasing baclofen dosing and botulinum toxin injections requirements after surgery. They stimulation responses of sectioned dorsal nerve roots adds to the limited available data and our understanding of the pathological changes that occur in CP.

A preliminary study on the spasticity reduction of quadriceps after selective dorsal rhizotomy in pediatric cases of spastic cerebral palsy.

OBJECTIVE: This study aimed to evaluate the potential alleviation of quadriceps spasticity in children diagnosed with spastic cerebral palsy (CP) following selective dorsal rhizotomy (SDR). METHODS: A retrospective study was conducted on children suffering from spastic CP who underwent SDR at the Department of Neurosurgery, Shanghai Children's Hospital, from July 2018 to September 2020. Inclusion criteria comprised children exhibiting quadriceps spasticity exceeding modified Ashworth Scale grade 2. Muscle tone and motor function were assessed before the operation, at short-term follow-up and at the last follow-up after SDR. Additionally, intraoperative neurophysiological monitoring data were reviewed. RESULTS: The study comprised 20 eligible cases, where, prior to surgery, 35 quadriceps muscles exhibited spasticity exceeding modified Ashworth Scale grade 2. Following short-term and mid-term follow-up, specifically an average duration of 11 ± 2 days and 1511 ± 210 days after SDR, it was observed that muscle tension in adductors, hamstrings, gastrocnemius, and soleus decreased significantly. This reduction was accompanied by a decrease in quadriceps muscle tone in 24 out of 35 muscles (68.6%). Furthermore, the study found that intraoperative electrophysiological parameters can predict postoperative spasticity relief in the quadriceps. The triggered electromyographic (EMG) output of the transected sensory root/rootlets after single-pulse stimulation revealed that the higher the EMG amplitudes in quadriceps, the greater the likelihood of postoperative decrease in quadriceps muscle tension. CONCLUSIONS: SDR demonstrates the potential to reduce muscle spasticity in lower extremities in children diagnosed with CP, including a notable impact on quadriceps spasticity even they are not targeted in SDR. The utilization of intraoperative neurophysiological monitoring data enhances the predictability of quadriceps spasticity reduction following SDR.

Treatment of ZC4H2 Variant-Associated Spastic Paraplegia with Selective Dorsal Rhizotomy and Intensive Postoperative Rehabilitation: A Case Report.

Selective dorsal rhizotomy (SDR) has been used to treat children with spastic cerebral palsy (CP), and its beneficial effect on quality of life and ambulation has been confirmed in long-term follow-up studies. However, the role of SDR in the treatment of spasticity in patients with hereditary spastic paraplegia (HSP) and related disorders is not well-established. Here, we report the first patient with the ZC4H2 variant who underwent SDR to treat spastic paraplegia. Abnormal gait was discovered during a regular checkup at the age of 3 years and 9 months, and she was diagnosed with spastic paraplegia. She was heterozygous for the ZC4H2 variant and underwent SDR at the age of 5 years and 11 months, which alleviated the spasticity. The patient underwent inpatient postoperative rehabilitation for 4 months and continued outpatient physiotherapy after discharge. The Gross Motor Function Measure-88 score and maximum walking speed decreased transiently 1 month postoperatively, but gradually recovered, and continuously improved 6 months postoperatively. SDR and postoperative intensive rehabilitation were effective in improving motor and walking functions up to 6 months after surgery, although long-term follow-up is needed to draw conclusions.

'I Didn't Know What Was Going to Happen': Children's and Young People's Experiences and Their Involvement Before and After Selective Dorsal Rhizotomy.

AIMS: To explore experiences, expectations, and involvement of children and young people (CYP) in decision-making for selective dorsal rhizotomy (SDR) surgery, post-operative physiotherapy treatment and outcomes. METHODS: A qualitative study design using one to one interviews. Five CYP (2 girls and 3 boys) participated, and interviews lasted between 45 min and 2 h. Data were analyzed using thematic analysis. RESULTS: Children and young people are reliant on their parents to make decisions and inform them of the SDR process. Experiences of living with cerebral palsy and its management are centered on their routine social, psychological, and physiological challenges. Individual characteristics and attributes of CYP have an impact on how they cope with the rehabilitation burden and adjust to their changing levels of function and participation. CONCLUSIONS: Although CYP reported that SDR offers them a greater 'freedom to choose' in how they participate in daily life, further consideration is required to meet their psychosocial needs, particularly in preparing for SDR and adjusting afterwards.

Patient- and Caregiver-Reported Outcome Measures after Single-Level Selective Dorsal Rhizotomy in Pediatric and Young Adult Patients with Spastic Cerebral Palsy.

INTRODUCTION: The aim of this cohort study was to assess the outcome of single-level selective dorsal rhizotomy (SDR) in children and young adults with spastic cerebral palsy (CP) treated at our institution, focusing on patient-reported outcome measures (PROMs) and quality of life (QoL) of patients and their caregivers. METHODS: We included consecutive patients undergoing SDR from 2018 to 2020 at our institution. Subjective outcome was measured through PROMs, while functional outcome was measured through baseline characteristics, operative outcome, as well as short- and long-term follow-up. Furthermore, the effect of age at the time of surgery on patient/caregiver satisfaction was analyzed. RESULTS: Seven patients (3 female, 43%) with a median age at surgery of 11.9 years (IQR 8.7-15.5) were included. All patients had a Gross Motor Function Classification (GMFCS) score of at least IV before surgery. Five surgeries were palliative and two non-palliative. Based on PROMs, SDR showed very good QoL and health-related outcome measures for both palliative and non-palliative patients. Patient/caregiver satisfaction was higher for the early subgroup (age ≤11) than the late subgroup (age >11). Functional outcome showed reduced spasticity in both groups. Blood transfusions were never needed, while no cerebrospinal fluid leak, infection, or permanent morbidity was seen. CONCLUSION: Based on PROMs, SDR leads to high satisfaction and improved QoL, especially if done at an early age. Further studies with larger cohorts are necessary to underline and confirm our observations.

Goal setting, goal attainment and quality of life of children following selective dorsal rhizotomy.

AIM: The aim of this study is to describe the individualized occupational performance issues identified by parents/carers and children prior to selective dorsal rhizotomy (SDR) surgery and analyse change up to 2 years post surgery in goal attainment and quality of life (QoL). METHOD: The Australian SDR Research Registry (trial registration: ACTRN12618000985280) was used to extract data for individualized goals, goal attainment and QoL based on the Canadian Occupational Performance Measure (COPM) and the Cerebral Palsy Quality of Life Questionnaire for Children (CP QOL-Child parent-proxy) at baseline and 1 and 2 years following SDR. Change in mean scores was analysed using linear mixed models. RESULTS: Fifty-two children had COPM scores at baseline and 1 and/or 2 years post, of which 28 had two QoL scores. COPM problem areas included leisure (n = 39), productivity (n = 37) and self-care (n = 173). The most common goals were walking (26.1%), participation in physical activities (17.7%) and transitions (14.1%). Mean COPM scores improved significantly between baseline to 1 year and baseline to 2 years (P < 0.001). Mean QoL scores improved between baseline to 1 year for functional QoL domains: participation and physical health (P = 0.003) and pain and impact of disability (P = 0.011). CONCLUSIONS: Collaborative goal setting is an integral part of family-centred rehabilitation practice. The COPM was an appropriate individualized outcome measure in identifying meaningful goals for our SDR cohort. Results demonstrate improved scores in goal attainment and improvement in functional QoL domains. This paper highlights the need to include outcomes that measure daily life experiences.

'Wanting no regrets': Parental decision making around selective dorsal rhizotomy.

BACKGROUND: Selective dorsal rhizotomy (SDR) is an irreversible neurosurgical procedure used to reduce spasticity while aiming to improve gross motor function for children with cerebral palsy (CP). Little research has explored the complexity of parental decision-making experiences surrounding SDR surgery and required rehabilitation. The aim was to explore parental experiences and expectation in the decision-making process around the SDR operation, physiotherapy treatment and outcomes. METHOD: Qualitative methods using in-depth, semi-structured interviews were used. Eighteen parents (11 mothers and 7 fathers) participated whose child had SDR and completed 2-year post-operative rehabilitation. Data were analysed using thematic analysis. RESULTS: Decision making involved an interacting process between the 'parental information seeking experience', 'influence of professional encounters and relationships' and 'emotional and social aspects'. Despite underlying uncertainties about outcomes, parental drivers of expectations and aspirations for their child preceded and sustained this decision-making process. A foundational narrative of 'wanting no regrets' resolved their decision to undertake SDR. Outcomes did not always match expectations, and parents moved away from a position of idealism, which was driven in part by parental information seeking including overly optimistic media representation. CONCLUSION: Universally, parents expressed they had 'no regrets' on their decision, yet many felt a mismatch between expectation and outcomes of SDR surgery. A greater understanding of the complex nature of SDR decision making is required to help improve family preparedness and provide support from clinicians to enable balanced discussions in parental decision-making.

Efficacy of Selective Dorsal Rhizotomy and Intrathecal Baclofen Pump in the Management of Spasticity.

BACKGROUND: Neurosurgical indications and interventions provided in the management of spasticity have evolved significantly over time. Selective dorsal rhizotomy (SDR) and intrathecal baclofen (ITB) pumps have been used to improve mobility, reduce lower extremity spasticity, and increase quality of life in patients with various diagnoses. METHODS: Studies describing ITB and SDR outcomes in adult and pediatric patients were identified from Medline and Embase databases. Only publications between January 1990 to January 2021 were included. Combinations of search terms 'Selective Dorsal Rhizotomy', 'Selective Posterior Rhizotomy', 'functional posterior rhizotomy', 'intrathecal baclofen pump', and 'spasticity' were used. Only studies in English language and those that included parameters for lower extremity outcome (i.e., spasticity, ambulation) were included. Only studies describing follow-up 12 months or greater were included. Case reports, reviews without primary data, or inaccessible publications were excluded. RESULTS: Two hundred and ninety publications between January 1990 to January 2021 were identified. Of these, 62 fit inclusion and exclusion criteria for a total of 1291 adult and 2263 patients. Etiologies in adult and pediatric populations varied substantially with multiple sclerosis, cerebral palsy, and trauma comprising the majority of causes for spasticity in adult patients. In pediatric patients, cerebral palsy was the predominant etiology of spasticity. While outcomes after SDR and ITB varied, both are effective for long-term tone reduction. SDR appeared to have a greater effect on function compared to baseline when comparing relatively similar subgroups. The complication rates for either intervention were significant; ITB had a much greater incidence of wound and hardware adverse events, whereas SDR was associated with a not insignificant incidence of new bladder or sensory deficit. CONCLUSION: ITB and SDR have demonstrated efficacy and utility for tone reduction in a variety of conditions. The selection of a specific intervention may have a variety of determining features including the etiology of spasticity, age of patient, as well as balancing benefit and complication profiles of each technique. Appropriate patient selection is essential for providing optimal patient outcomes.

Spinal cord tethering after selective dorsal rhizotomy below the conus medullaris.

BACKGROUND: Two techniques for selective dorsal rhizotomy (SDR) involve stimulating and sectioning nerve rootlets either below the conus medullaris or above the entrances to their respective dural root sleeves. In general, both techniques lead to sustained improvements in lower extremity spasticity with low complication rates. To our knowledge, spinal cord tethering has not been previously reported as a complication of SDR using either technique. METHODS: We review the presentation, treatment, and outcome of two patients who developed symptomatic spinal cord tethering after SDR below the conus. RESULTS: A 4-year-old male and a 6-year-old female each with a history of spastic diparetic cerebral palsy underwent L1-L2 osteoplastic laminectomy and SDR below the conus. Both surgeries went well with no known complications. Both patients initially did well, with marked improvement in their tone and gait. There were no significant immediate postoperative bowel or bladder problems. The male patient presented 10 months after surgery with new-onset urinary incontinence. A urological evaluation was performed but no imaging was performed. He re-presented 8 months later with worsened urinary incontinence, new fecal incontinence, and worsened gait. An MRI showed signs of tethering, including a holocord syrinx. He underwent two untethering surgeries as well syrinx fenestration, and although his gait improved, it remained worse than it had been several months after surgery. The incontinence did not improve. The second patient also developed urinary incontinence a year after SDR. Due to our experience with the first patient, we obtained an MRI immediately and found evidence of tethering. She underwent untethering and her incontinence improved. CONCLUSION: We report two cases of spinal cord tethering after SDR below the conus, a previously unreported complication. It is important to realize that this complication can occur after SDR.

Selective dorsal rhizotomy: short-term results and early experiences with a newly established surgical treatment in Slovenia.

BACKGROUND: Spasticity is characterised by an atypical increase of muscle tone, affecting normal movements and interfering with the patient quality of life. The medicines may limit the effects of the disease and selective dorsal rhizotomy (SDR) can be used for selected cases or cases refractory to medicine. We present the surgical technique and the short-term results of this newly established surgical treatment in Slovenia. METHODS: A retrospective analysis was performed of all patients that underwent the SDR from 2017 to 2019. The median follow-up was of 10 months. The following data have been collected: aetiology of spasticity, age at SDR, number of sectioned lumbar rootlets L1-S2, intraoperative disappearance of the H-reflex and intraoperative preservation of the bulbocavernosus reflex. The motor functions of all children have been classified by the Gross Motor Function Classification System (GMFCS) and Gross Motor Function Measure (GMFM-88). Twelve children underwent SDR, the median age at surgery was 9.6 years (min 3.9-max 16 years). RESULTS: A mean of 57.8% of dorsal rootlets L2-S1 have been cut, while at level L1 50% of the dorsal roots have been routinely sectioned. The median amount of S2 rootlets cut was 14.3%. Postoperatively, we observed a sudden decrease in muscle tone. In all patients, there was an improvement of the muscle tone and of the gait pattern. The GMFM improved from 187.8 to 208.3 after a follow-up of 6 months. CONCLUSIONS: There was no complication in terms of wound healing, cerebrospinal fluid fistula of neurological dysfunctions. Despite the relatively short follow-up, our early results confirm the efficacy of the SDR.

Treatment of spasticity in children and adolescents with cerebral palsy in Northern Europe: a CP-North registry study.

BACKGROUND: Spasticity is present in more than 80% of the population with cerebral palsy (CP). The aim of this study was to describe and compare the use of three spasticity reducing methods; Botulinum toxin-A therapy (BTX-A), Selective dorsal rhizotomy (SDR) and Intrathecal baclofen therapy (ITB) among children and adolescents with CP in six northern European countries. METHODS: This registry-based study included population-based data in children and adolescents with CP born 2002 to 2017 and recorded in the follow-up programs for CP in Sweden, Norway, Denmark, Iceland and Scotland, and a defined cohort in Finland. RESULTS: A total of 8,817 individuals were included. The proportion of individuals treated with SDR and ITB was significantly different between the countries. SDR treatment ranged from 0% ( Finland and Iceland) to 3.4% (Scotland) and ITB treatment from 2.2% (Sweden) to 3.7% (Denmark and Scotland). BTX-A treatment in the lower extremities reported 2017-2018 ranged from 8.6% in Denmark to 20% in Norway (p < 0.01). Mean age for undergoing SDR ranged from 4.5 years in Norway to 7.3 years in Denmark (p < 0.01). Mean age at ITB surgery ranged from 6.3 years in Norway to 10.1 years in Finland (p < 0.01). Mean age for BTX-A treatment ranged from 7.1 years in Denmark to 10.3 years in Iceland (p < 0.01). Treatment with SDR was most common in Gross Motor Function Classification System (GMFCS) level III, ITB in level V, and BTX-A in level I. The most common muscle treated with BTX-A was the calf muscle, with the highest proportion in GMFCS level I. BTX-A treatment of hamstring and hip muscles was most common in GMFCS levels IV-V in all countries. CONCLUSION: There were statistically significant differences between countries regarding the proportion of children and adolescents with CP treated with the three spasticity reducing methods, mean age for treatment and treatment related to GMFCS level. This is likely due to differences in the availability of these treatment methods and/or differences in preferences of treatment methods among professionals and possibly patients across countries.

Systematic review on use and efficacy of selective dorsal rhizotomy (SDR) for the management of spasticity in non-pediatric patients.

PURPOSE: Selective dorsal rhizotomy (SDR) has been used to improve mobility and reduce lower extremity spasticity in patients with a various CNS conditions. Incidentally, literature on SDR has been performed in the pediatric population as such there is a paucity of research on the use in adult patients. METHODS: Studies describing SDR in adults were identified from Medline and Embase databases. Combinations of search terms "Selective Dorsal Rhizotomy," "Selective Posterior Rhizotomy," and "Adult" were used. Only literature in English language on patients over the age of 18 years and that included measures for lower extremity outcome (i.e., spasticity, mobility) were included. Case reports, reviews without primary data, or inaccessible publications were excluded. RESULTS: One hundred twenty-nine publications between 1970 and 2019 were identified. Twelve of these publications fit the inclusion criteria (n = 141 patients). In series where it was reported, SDR resulted in ambulatory improvement (54%, n = 44 out of 81), reduced spasticity (75.2%, n = 106 out of 141), and minimized muscle and joint pain (74.5%, n = 64 out of 86). SDR also showed improvement in parameters of the activities of daily life. 92.3% (n = 48 out of 52) of patients post-SDR developed new lower limb paresthesia. CONCLUSION: The success and efficacy appear durable in the short-term, but further follow-up is necessary to validate these findings. The goal of the intervention dictates the ideal adult patient for SDR. Patients seeking ambulatory improvement, any etiology of spasticity besides MS, seem favorable. Positive locomotive predictors include the ability to isolate lower extremity function, lack of contractures, lower limb strength, and post-SDR physiotherapy.