Focal selective dorsal rhizotomy and concurrent deformity correction: a combined approach.

OBJECTIVE: Selective dorsal rhizotomy (SDR) is a neurosurgical procedure to reduce spasticity in children with cerebral palsy and spastic diplegia. The authors developed a procedure called focal SDR for children with spasticity predominantly in the L5 or S1 motor distribution, which can be combined with orthopedic correction of fixed soft-tissue or bony deformity. The authors describe in detail the technique of minimally invasive focal SDR and propose selection criteria. METHODS: The authors conducted a retrospective study of patients who underwent focal SDR at their institution and underwent baseline and 1-year postoperative 3D gait analysis. Modified Ashworth scale (MAS) and Gait Deviation Index (GDI) scores were the primary outcome measures. RESULTS: Ten patients met the study criteria, all with an underlying diagnosis of cerebral palsy. All underwent focal SDR at the unilateral or bilateral S1 level, and 4 additionally underwent focal SDR at the L5 level unilaterally or bilaterally. All but 1 patient underwent concurrent orthopedic surgery. The improvement in spasticity of the plantar flexors, as measured by the MAS score, was 2.2 (p < 0.001). In the patients who underwent L5 focal SDR, there was an improvement in the hamstring MAS score of 1.4 (p = 0.004). The mean improvement in the GDI score following focal SDR was 11 (range -6 to 29, p < 0.001). CONCLUSIONS: Focally impairing spasticity in the gastrocsoleus complex and/or hamstrings muscle group in the setting of less functionally impactful proximal tone is extremely common in cerebral palsy. The novel technique of focal SDR, combined with orthopedic intervention, improves spasticity scores and overall gait mechanics. Further investigation is warranted to define the ideal candidacy and outcomes.

Comparison of outcomes following surgical intervention and inpatient rehabilitation stays in children with cerebral palsy.

INTRODUCTION: Literature is limited on functional outcomes in children with cerebral palsy (CP) following surgical procedures and a subsequent inpatient rehabilitation unit (IRU) stay. OBJECTIVE: To compare functional outcomes and length of stay (LOS) in children with CP following a surgical procedure and IRU stay based on the surgical procedure performed, pattern of involvement, etiology, and Gross Motor Function Classification System (GMFCS) level. DESIGN: Retrospective cohort study. SETTING: Tertiary care pediatrics. PARTICIPANTS: Pediatric patients with CP who underwent one of three surgical procedures followed by an IRU stay. INTERVENTIONS: Selective dorsal rhizotomy (SDR), single-event multilevel orthopedic surgery (SEMLS), or intrathecal baclofen (ITB) pump implantation and subsequent IRU stay. MAIN OUTCOME MEASURES: IRU LOS, Functional Independence Measure for Children (WeeFIM) total score, sub-scores, and efficiency. RESULTS: Children undergoing SDR had a longer LOS (p ≤ .015). Children with spastic diplegia, GMFCS level II, and prematurity-based CP had higher WeeFIM efficiency scores (p ≤ .046, ≤.021, and .034 respectively). Greater changes in WeeFIM™ scores were associated with spastic diplegia, SDR, GMFCS level II, longer LOS, and higher admission scores (p ≤ .045). CONCLUSIONS: Although statistically and functionally significant improvements in children with CP following surgical interventions and an IRU stay were seen, those with higher WeeFIM change scores tended to have spastic diplegia, to have undergone SDR, GMFCS level II, longer LOS, and higher admission scores.

Magnetic Resonance-Guided Laser Interstitial Thermal Therapy for Palliative Rhizotomy: A Novel Technical Application.

BACKGROUND: Spastic cerebral palsy is caused by an insult to the developing brain. Various medical and surgical procedures are used to reduce tone. OBJECTIVE: To describe a novel method of magnetic resonance-guided laser interstitial thermal ablation for palliative rhizotomy. METHODS: Patients treated at a single institution with percutaneous rhizotomy using magnetic resonance-guided laser interstitial thermal therapy were identified. Preoperative and postoperative Modified Ashworth Scale scores were collected as well as procedural information. RESULTS: Two male children (7.8 and 19 yr, respectively) with spastic quadriparesis were treated using this technique. Neither patient experienced surgical or perioperative complications, and both were discharged from the hospital within 48 h. Each of them demonstrated improvement in his Modified Ashworth Scale score with no need for retreatment for spasticity at last follow-up. CONCLUSION: These 2 cases illustrate a novel technique for treating spasticity in the setting of cerebral palsy. Further study of this technique in additional patients, and comparison with traditional methods of surgical tone reduction, are warranted.

Kinematics associated with treadmill walking in Rett syndrome.

BACKGROUND AND PURPOSE: Individuals with Rett syndrome suffer from severely impaired cognitive and motor performance. Current movement-related therapeutic programs often include traditional physical therapy activities and assisted treadmill walking routines for those individuals who are ambulatory. However, there are no quantitative reports of kinematic gait parameters obtained during treadmill walking. The purpose of this research was to characterize the kinematic patterns of the lower limbs during treadmill walking as speed was slowly increased. METHODS: Seventeen independently ambulatory females diagnosed with a methyl-CpG-binding protein 2 gene mutation walked on a motorized treadmill while joint kinematics were obtained by a camera-based motion capture system and analysis software. RESULTS: Stride times progressively decreased as treadmill speeds increased. There were significant main effects of speed on sagittal knee and hip ranges of motion and hip velocity. There were large joint asymmetries and variance values relative to other ambulatory patient populations, although variance values decreased as walking speed increased. CONCLUSIONS: The results indicate that individuals with Rett syndrome can adapt their kinematic gait patterns in response to increasing treadmill speed, but only within a narrow range of speeds. We suggest that treadmill training for ambulatory individuals with Rett syndrome may promote improved walking kinematics and possibly provide overall health benefits.Implications for rehabilitationWalking is an activity that can counter the negative impacts of the sedentary lifestyle of many individuals with disabilities, including those individuals with Rett syndrome.Documentation of the lower limb kinematic patterns displayed during walking by ambulatory females with Rett syndrome can be used by clinicians to evaluate their patients' gait performance in response to therapeutic and pharmacological interventions designed to promote walking.The ability to adapt to increases in treadmill speed suggests that a training program of treadmill walking may be effective in promoting improved gait performance in individuals with Rett syndrome.

Predictors of voice outcome in pediatric non-selective laryngeal reinnervation.

OBJECTIVES: Non-selective laryngeal reinnervation (NSLR) using the ansa cervicalis to the recurrent laryngeal nerve (RLN) is a promising treatment option for pediatric unilateral neuronal vocal fold movement impairment (VFMI). The aim is to describe our clinical outcomes with this technique and to identify preoperative characteristics that may predict postoperative voice outcomes. METHODS: This is a cohort study of pediatric patients with unilateral neuronal VFMI, who underwent NSLR from March 2012 to July 2018. Pre- and postoperative Pediatric Voice Related Quality of Life (PVRQOL) questionnaires, Consensus Auditory Perceptual Evaluation of Voice (CAPE-V) ratings, and objective voice measures were obtained. In addition, patients underwent preoperative laryngeal electromyography (LEMG). RESULTS: Thirty-two patients were identified. Twenty-one had complete data sets for analysis. The mean duration of VFMI was 9.02 years (range 1.1-26.1 years). There were significant improvements in PVRQOL (P = .0005), in all CAPE-V subsets (P ≤ .0001 to .0195), mean and maximum intensities (P = .0342 and 0.0110, respectively), cepstral peak prominence (P = .0001), and cepstral spectral index of dysphonia (P ≤ .0001). A worse preoperative LEMG correlated with a greater change in maximum phonation time (P = .0162) and maximum intensity (P = .0346). Age at injury and duration of injury had no significant impact on voice outcomes; however, patients with concurrent posterior glottic insufficiency did have smaller changes in PVRQOL (P = .012). CONCLUSION: NSLR is an effective treatment for pediatric unilateral neuronal VFMI even many years after initial RLN injury. LEMG may help predict voice outcomes of reinnervation in pediatric patients, but further data is still needed. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1525-1531, 2020.

Comprehensive Care in Cerebral Palsy.

Children with cerebral palsy (CP) will be cared for at some point by all pediatricians and many pediatric subspecialists due to this condition being the most common motor disability of childhood. Comprehensive care of the child with CP requires individuals with specialized training, and these children benefit from an interdisciplinary team approach to care. CP is heterogeneous due to varied causes, which necessitates individualized treatment plans. The CP specialist must be prepared to support the needs of the child with CP holistically and must dialogue regularly with members of the team and involve the family in decision-making.

Temporal Gait Measures Associated With Overground and Treadmill Walking in Rett Syndrome.

Rett syndrome is a severe neurodevelopmental disorder leading to intellectual impairment and global developmental delays, including difficulty or inability to walk. Assessing differences in temporal parameters and associated variability between overground and treadmill walking is important if gait training is to be incorporated into intervention protocols. Fourteen female patients with Rett syndrome (mean age 10.4 years ± SD 5.1) were evaluated during overground and treadmill walking. Stride, stance, swing, and double support times, and the variance of these measures, were obtained. Wilcoxon signed-rank tests were used to assess for potential differences between overground and treadmill measures. Treadmill gait resulted in decreases in swing and double support times. When normalized to stride time, treadmill gait displayed an increase in stance time with decreases in swing and double support times. Excepting stance time, treadmill gait resulted in decreased variability, indicating a more regularized gait while walking on the treadmill. These results suggest that treadmill walking can be beneficial for ambulatory patients with Rett syndrome and could be incorporated into a therapeutic protocol designed to maintain the maximum degree of mobility and overall general health as part of a comprehensive health management approach.

Botulinum Toxin in the Treatment of Pediatric Upper Limb Spasticity.

Botulinum neurotoxin (BoNT) is one of the mainstays in the treatment of pediatric spasticity and dystonia. When considering initiation of BoNT treatment for spasticity, treatment goals and responses to prior conservative measures such as passive range of motion exercises, splinting, and other medication trials should be reviewed. As a general rule, children should be engaged in therapy services around the time of the injections and have a robust home program in place. When managing spasticity in children with BoNT injections, the practitioner should be well versed in functional anatomy with specialized training in injection techniques. Localization techniques in addition to anatomical landmarks are recommended for improved efficacy and include limited electromyography, electrical stimulation, and/or ultrasound guidance. A follow-up visit for the purpose of reassessment during the peak effect of the drug is advised. It is known that BoNT is effective at reducing spasticity and improving range of motion, but it remains to be determined to what degree this translates into improved function, activity, and participation.

Population pharmacokinetics of oral baclofen in pediatric patients with cerebral palsy.

OBJECTIVE: To characterize the population pharmacokinetics (PK) of oral baclofen and assess impact of patient-specific covariates in children with cerebral palsy (CP) in order to support its clinical use. SUBJECTS DESIGN: Children (2-17 years of age) with CP received a dose of titrated oral baclofen from 2.5 mg 3 times a day to a maximum tolerated dose of up to 20 mg 4 times a day. PK sampling followed titration of 10-12 weeks. Serial R- and S-baclofen plasma concentrations were measured for up to 16 hours in 49 subjects. Population PK modeling was performed using NONMEM 7.1 (ICON PLC; Ellicott City, Maryland). RESULTS: R- and S-baclofen showed identical concentration-time profiles. Both baclofen enantiomers exhibited linear and dose/kg-proportional PK, and no sex differences were observed. Average baclofen terminal half-life was 4.5 hours. A 2-compartment PK model with linear elimination and transit absorption steps adequately described concentration-time profiles of both baclofen enantiomers. The mean population estimate of apparent clearance/F was 0.273 L/h/kg with 33.4% inter-individual variability (IIV), and the apparent volume of distribution (Vss/F) was 1.16 L/kg with 43.9% IIV. Delayed absorption was expressed by a mean transit time of 0.389 hours with 83.7% IIV. Body weight, a possible genetic factor, and age were determinants of apparent clearance in these children. CONCLUSION: The PK of oral baclofen exhibited dose-proportionality and were adequately described by a 2-compartment model. Our population PK findings suggest that baclofen dosage can be based on body weight (2 mg/kg per day) and the current baclofen dose escalation strategy is appropriate in the treatment of children with CP older than 2 years of age.

Neuromuscular scoliosis.

The purpose of this focused review is to provide an overview of neuromuscular scoliosis from the perspective of the rehabilitation physician. Scoliosis is a common consequence of neuromuscular diseases, including central nervous system disorders such as cerebral palsy and spinal cord injury; motor neuron disorders, for example, spinal muscular atrophy; muscle fiber disorders, for example, Duchenne muscular dystrophy; multifactorial disorders, for example, spina bifida; and many other neuropathic and myopathic conditions. Unlike adolescent idiopathic scoliosis, which is the most common form of spinal deformity, neuromuscular scoliosis is more severe and more progressive, and is associated with more morbidity. Factors that contribute to this spinal deformity include asymmetric paraplegia, imbalance of mechanical forces, intraspinal and congenital anomalies of the spine, altered sensory feedback, and abnormal posture via central pathways. Spinal deformity combined with limitations due to an underlying neuromuscular condition lead to significant physiologic impairments that affect limb movement, cardiopulmonary function, gait, standing, sitting, balance, trunk stability, bimanual activities, activities of daily living, and pain, as well as concerns with self-image and social interactions. Evaluation and management of this population requires understanding of disease progression, pulmonary status, functional limitations, indications for conservative and surgical interventions, and social considerations.