Transiliac-Shortening Osteotomy to Treat Ischial Pressure Injury due to Fixed Pelvic Obliquity: A Case Report.

CASE: A 17-year-old adolescent boy with Gross Motor Function Classification System 5 cerebral palsy and neuromuscular scoliosis underwent posterior spinal fusion and segmental spinal instrumentation from T3 to the pelvis. He developed a right ischial pressure injury a few months postoperatively, which persisted despite nonoperative measures. He subsequently underwent an ipsilateral transiliac-shortening osteotomy 16 months after spinal surgery to treat his residual pelvic obliquity and the ischial pressure injury, which healed completely. At the 1-year follow-up visit, there were no further signs of pressure injury. CONCLUSION: This case report describes transiliac-shortening osteotomy as a viable treatment option for non-healing ischial pressure injuries secondary to fixed pelvic obliquity.

Simultaneous subtalar arthroeresis, midfoot soft tissue release, and talo-navicular arthrodesis in children with planovalgus neurologic foot.

PURPOSE: Planovalgus foot deformity (PVFD) is common in children with neuromuscular conditions and severe deformity may require surgical correction. This study aims to assess clinical and radiological outcomes of PVFD secondary to neuromuscular disease managed by subtalar arthroeresis (SuAE), midfoot soft tissue release and talo-navicular arthrodesis (TNA). METHODS: A retrospective analysis of children with neuromuscular disease and nonreducible PVFD who underwent SuAE, midfoot soft tissue release, and TNA and with a minimum follow-up of 5 years was performed. A total of 60 patients with neuromuscular disease (108 feet) including cerebral palsy were reviewed. Mean age at surgery was 12.7 ± 4.6 years (6-17). Mean follow-up was 7 ± 2.9 years (5-10). Clinical outcomes and radiologic correction at final follow-up were compared with preoperative values. Statistical analysis was performed and significance was set at P < 0.01. RESULTS: Statistically significant radiological improvements between pre- and postoperative values were found for all angle values. At final follow-up, there was a significant improvement in VAS score (4.8 vs. 2; P < 0.01). There was also a positive trend in the improvement of walking ability. No cases of pseudoarthrosis were reported at final follow-up. Screw removal was required in 5 out of 108 feet (4.6%) and 2 feet (3.3%) had delayed medial wound healing. CONCLUSIONS: SuAE combined with TNA and midfoot soft tissue is a safe and feasible procedure that can provide good clinical and radiologic results in patients with neuromuscular disease and nonreducible PVFD; the procedure can improve foot stability, and has a limited number of complications. LEVEL OF EVIDENCE: IV.

Prevalence and Risk Factors for Cerebral Palsy in Children With Congenital Heart Disease Based on Risk of Surgical Mortality.

BACKGROUND: Children with congenital heart disease (CHD) have a higher prevalence of motor impairment secondary to brain injury, resulting in cerebral palsy (CP). The purpose of this study is to determine the prevalence of CP in CHD in a single-center cohort, stratify risk based on surgical mortality using Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) categories and identify risk factors. METHODS: Retrospective cohort study of pediatric patients registered in the University of Florida (UF) Society of Thoracic Surgeons Congenital Heart Surgery database from 2006 to 2017 with a diagnosis of CHD who continued follow-up for more than two years at UF. RESULTS: A total of 701 children with CHD met inclusion criteria. Children identified to have CP were 54 (7.7%). Most common presentation was spastic hemiplegic CP with a Gross Motor Function Classification System of level 2. Analysis of surgical and intensive care factors between the two groups showed that children with CHD and CP had longer time from admission to surgery (P = 0.003), higher STAT categories 4 and 5 (P = 0.038), and higher frequency of brain injury and seizures (P < 0.001). Developmental disabilities and rehabilitation needs were significantly greater for children with CHD and CP when compared with those with CHD alone (P < 0.001). CONCLUSIONS: In our cohort, 7.7% children with CHD develop CP; this is significantly higher than the 2010 US population estimate of 0.3%. Our study suggests higher STAT categories, brain injury, and seizures are associated with developing CP in children with CHD.

Quality of life and clinical outcomes in severely involved cerebral palsy patients and spastic hips undergoing Castle surgery: a cross-sectional study.

PURPOSE: As progressive hip dislocation causes pain in children with spastic cerebral palsy (CP) and spasticity needs surgical correction, we aimed to describe clinical and radiographic outcomes in CP patients with painful hip deformity treated with the Castle salvage procedure. METHODS: We included all patients operated in the same hospital between 1989 and 2017 with painful spastic hips and femoral head deformity making joint reconstruction unfeasible. We collected clinical and functional data from medical records and evaluated radiographies to classify cases for femoral head shape and migration, type of deformity, spinal deformity, and heterotopic ossification. We investigated quality of life one year after surgery. RESULTS: We analyzed 41 patients (70 hips) with complete medical records. All had severe function compromise GMFCS V (Gross Motor Function Classification System) and heterotopic ossifications, all but one had scoliosis, and most had undergone other surgeries before Castle procedure. Patients were followed up for 77.1 months (mean) after surgery. The mean initial migration index was 73%. Seven patients had complications, being three patients minor (two femur and one tibial fracture) and four majors (patients requiring surgical revision). Quality of life was considered improved by most of the carers (35 children; 85.3%) as level 4/5 according to CPCHILD instrument. No child was able to stand or walk, but moving in and out of bed, of vehicles, and to a chair, remaining seated, or visiting public places was "very easy." CONCLUSION: We considered most patients (37 patients-90%, 66 hips-94%) as having satisfactory outcomes because they had no or minor complications, absence of pain, free mobility of the lower limbs and were able to sit in a wheelchair.

Distal femoral osteotomy and patellar tendon advancement for the treatment of crouch gait in patients with bilateral spastic cerebral palsy.

BACKGROUND: Crouch gait, or flexed knee gait, represents a common gait pattern in patients with spastic bilateral cerebral palsy (CP). Distal femoral extension and/or shortening osteotomy (DFEO/DFSO) and patellar tendon advancement (PTA) can be considered as viable options when knee flexion contractures are involved. Better outcomes have been reported after a combination of both, independently of the presence of knee extensor lag. In this study, we evaluated the clinical and kinematic outcomes of these procedures. PATIENTS AND METHODS: We reviewed a cohort of 52 limbs (28 patients) who were treated for crouch gait by DFEO/DFSO alone (group 1, n = 15) or DFEO/DFSO + PTA (group 2, n = 37) as a part of single event multilevel surgery (SEMLS). The mean age at surgery was 14 years, and the mean follow-up time was 18 months. The physical examination data and three-dimensional standardized gait analysis were collected and analyzed before the surgery and postoperatively. RESULTS: Overall knee range of motion improved in all limbs. The knee flexion decreased significantly in both groups at initial, mid, and terminal stance. Hip flexion significantly decreased in mid-stance for limbs in group 2. Both clinical and gait parameters were most improved in limbs who underwent DFEO/DFSO + PTA. Increased pelvic tilt was observed in both groups after surgery. CONCLUSION: Although DFEO/DFSO alone was successful in correcting knee flexion contractures, PTA has helped to improve knee extensor lag and knee extension during gait. LEVEL OF EVIDENCE: Therapeutic level IV.

Does Size Really Matter? Incidence of Periprosthetic Fractures After Femoral Osteotomy in Patients With Cerebral Palsy.

OBJECTIVE: Of children, 30% to 35% with cerebral palsy (CP) develop hip subluxation or dislocation and often require reconstructive hip surgery, including varus derotation osteotomy (VDRO). A recent literature review identified postoperative fractures as the most common complication (9.4%) of VDROs. This study aimed to assess risk factors for periprosthetic fracture after VDRO in children with CP. METHODS: A total of 347 patients (644 hips, 526 bilateral hips) with CP and hip subluxation or dislocation (129 females; mean age at index VDRO: 8.6 y, SD 3.4, range: 1.5 to 17.7; 2 Gross Motor Function Classification System (GMFCS) I, 35 GMFCS II, 39 GMFCS III, 119 GMFCS IV, 133 GMFCS V, 21 unavailable) were included in this retrospective, single-group intervention (VDRO) study at a tertiary referral center. Imaging and clinical documentation for patients age 18 years or younger at index surgery, treated with VDRO were reviewed to determine demographic data, GMFCS level, surgeon, type of hardware implanted, use of anticonvulsants and steroids, type of postoperative immobilization, presence of periprosthetic fractures, fracture location and mechanism, and time from surgery to fracture. Potential determinants of periprosthetic fractures were assessed using mixed effects logistic regression. RESULTS: Of 644 hips, 14 (2.2%, 95% CI: 1.3%, 3.6%) sustained a periprosthetic fracture, at a median of 2.1 years postoperatively (interquartile range: 4.6 y, range: 1.2 mo to 7.8 y). Patients with a fracture had a median age at index surgery of 7.3 years (interquartile range: 4.3, range: 2.8 to 17.8; 1 GMFCS II, 6 GMFCS IV, 7 GMFCS V). Periprosthetic fractures were not significantly related to age at index surgery ( P = 0.18), sex ( P = 0.30), body mass index percentile ( P = 0.87), surgery side ( P = 0.16), anticonvulsant use ( P = 0.35), type of postoperative immobilization ( P = 0.40), GMFCS level ( P = 0.31), or blade plate size ( P = 0.17). Only surgeon volume significantly related to periprosthetic fracture (odds ratio = 5.03, 95% CI: 1.53, 16.56, P = 0.008), with the highest-volume surgeon also using smaller blade plates ( P < 0.01). CONCLUSIONS: Periprosthetic fractures after VDRO surgery in children with CP are uncommon, and routine hardware removal appears unnecessary. The data suggest that the common dogma of putting in the largest blade plate possible to maximize fixation may increase the risk of periprosthetic fracture. Due to the overall low fracture rate, especially when contextualized relative to the risk of hardware removal, a reactive approach to hardware removal appears warranted. LEVEL OF EVIDENCE: Level III-retrospective study (targeting varus derotational osteotomies in children with cerebral palsy).

Incidence and sequence of scoliosis and windswept hip deformity: which comes first in 4148 children with cerebral palsy? A longitudinal cohort study.

BACKGROUND: The aim was to analyse whether scoliosis or windswept hip deformity (WSH) occurs first for children with cerebral palsy (CP). METHODS: This longitudinal cohort study using data from 1994 - 2020 (26 years) involved 41,600 measurements of 4148 children (2419 [58.3%] boys) with CP born 1990 - 2018 and registered into the Swedish CP follow-up program. Children were followed from a mean age of 2.8 [SD 1.4] years, until they developed either scoliosis or WSH or were removed at surgery. RESULTS: WSH developed first in 16.6% of the children (mean age 8.1 [SD 5.0] years), and scoliosis in 8.1% (mean age 8.1 [SD 4.9] years). The incidence of WSH was higher than scoliosis across all levels I-V of the Gross Motor Function Classification System (GMFCS), both sexes, and for those with dyskinetic (20.0%) or spastic (17.0%) CP. The incidence of scoliosis was highest (19.8%) and developed earliest in children with GMFCS level V (mean age 5.5 [SD 3.5] years), and in children with dyskinetic (17.9%) CP (mean age 7.0 [SD 4.7] years). CONCLUSIONS: WSH presents earlier than scoliosis in most children with CP. Children with higher GMFCS level or dyskinetic CP are more likely to develop these deformities at a younger age.

Functional outcomes following surgery for spastic hip adductor muscles in ambulatory and non-ambulatory adults.

OBJECTIVE: To evaluate functional outcomes of surgery of spastic hip adductor muscles (obturator neurotomy with or without adductor longus tenotomy) in ambulatory and non-ambulatory patients, using preoperatively defined personalized goals. DESIGN: Retrospective observational descriptive study. PATIENTS: Twenty-three patients with adductor spasticity who underwent obturator neurotomy between May 2016 and May 2021 at the Clinique des Cèdres, Cornebarrieu, France, were included. METHODS: Postoperative functional results were evaluated in accordance with the Goal Attainment Scaling method. Patients were considered "responders" if their score was ≥ 0. Secondary outcomes included spasticity, strength, hip range of motion and change in ambulatory capacity. When data were available, a comparison of pre- and postoperative 3-dimensional instrumented gait analysis was also performed. RESULTS: Among the 23 patients only 3 were non-walkers. Seventeen/22 patients achieved their main goal and 14/23 patients achieved all their goals. Results were broadly similar for both walking goals (inter-knee contact, inter-feet contact, fluidity, walking perimeter, toe drag) and non-walking goals (intimacy, transfer, pain, posture, dressing). CONCLUSION: Surgery of spastic hip adductor muscles results in functional improvement in ambulation, hygiene, dressing and posture and can be offered to patients with troublesome adductor overactivity. The use of a motor nerve block is recommended to define relevant goals before the surgery.

External Urethral Sphincter Botulinum Toxin Injection to Treat Pseudodysynergia in Patients With Cerebral Palsy.

OBJECTIVE: To evaluate urinary outcomes following cystoscopic external urinary sphincter onabotulinumtoxinA (BTX) injections in patients with cerebral palsy (CP). Adults with CP can suffer from bladder outlet obstruction and urinary retention due to a spastic external urethral sphincter ("pseudodysynergia"). We have used BTX injections into the sphincter to relieve the obstruction and allow patients to maintain spontaneous voiding rather than intermittent catheterization. METHODS: Patients were included in this retrospective cohort study if they had a diagnosis of CP, were at least 18 years of age, and underwent a urethral external sphincter BTX injection between 2016-2023. The procedure included 100 u or 200 u of BTX mixed in 4cc of saline. Primary outcomes were subjective, patient or caregiver reported changes in retention, lower urinary tract symptoms (LUTS), frequency of recurrent urinary tract infections (UTIs), and hydronephrosis or bladder stones/debris on ultrasound. RESULTS: Fifty patients were included; the majority were male (60%), lived at home with assistance (58%), and had a Gross Motor Function Classification System level of V (50%; ie, severe CP). The most common indications for BTX were retention (96%), LUTS (48%), hydronephrosis (18%), and recurrent UTIs (22%). Post-BTX improvement was seen in 67% of those with LUTS, 65% with retention, 67% with hydronephrosis, and 73% with recurrent UTIs. Most patients underwent repeat injections (60%). There were no significant complications associated with injections. CONCLUSION: External urethral sphincter BTX is a safe, effective option for treating pseudodysynergia in adults with 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.

The Etiologic Risk Factors for Cerebral Palsy at an Orthopedic Surgery Clinic in South Africa.

BACKGROUND: Cerebral palsy (CP) is a group of disorders that affect movement and posture caused by injury to the developing brain. Although prematurity and low birth weight are common causes in developed countries, birth asphyxia, kernicterus, and infections have been identified as predominant etiologies in Africa. There is, however, very little information on the etiology of CP in South Africa. We aimed to determine the etiology, severity, and topographic distribution of CP in children undergoing orthopedic surgery at our tertiary pediatric unit. METHOD: A retrospective folder review was performed for patients with CP who underwent orthopedic surgery from July 2018 to June 2022. Data were collected on perinatal circumstances, etiologic risk factors for developing CP, severity of disability as classified by the Gross Motor Function Classification Scale (GMFCS), and topographic distribution. Descriptive analysis was performed. RESULTS: A total of 202 patients were included in the analysis. Prematurity (gestational age less than 37 weeks) was noted in 41.6% of the cohort and was the most common risk factor. Hypoxic-ischemic encephalopathy (30.7%), postnatal infections (13.4%), congenital brain malformations (10.4%), and cerebral infections were the next most common etiologic risk factors. Forty-eight percent of patients were classified as GMFCS IV or V. There was a predominance of bilateral (69.5%) compared with unilateral (21.3%) subtypes. CONCLUSION: Most patients undergoing orthopedic surgery for musculoskeletal sequelae of CP had GMFCS levels of IV or V and were bilateral subtypes, emphasizing the need for intervention at a primary care level to decrease the incidence of this frequently preventable condition.

What Do We Really Know About the Natural History of Spastic Hip Dysplasia and Pain in Total-involvement Cerebral Palsy? A Systematic Review.

BACKGROUND: Hip surveillance protocols and surgery for spastic hip dysplasia have become standard of care for children with cerebral palsy (CP) out of concern for long-term sequelae, including pain. It is unclear if available data support that spastic hip dysplasia/dislocation independently correlates with pain in total-involvement CP. A better understanding of this correlation may help guide decision-making for these medically complex patients. METHODS: We undertook a systematic literature review to assess published data on the association of spastic hip dysplasia and pain in total-involvement CP using PubMed (which includes the MedLine databases) and EMBASE databases. A total of 114 English-language articles were identified. Fifteen articles met the inclusion criteria and were evaluated using the PRISMA guidelines for systematic reviews. RESULTS: Of 15 articles that specifically assessed the association of spastic hip dysplasia and pain, 5 articles provided strong evidence per our criteria regarding the correlation of pain and spastic hip dysplasia. All 5 articles described the severity of CP in their studied population, radiographically defined hip displacement, included a control group, and described how pain was assessed. Nevertheless, there was no standard classification of dysplasia between studies and the ages of patients and methods of pain determination varied. Four of the articles provided level III evidence and one provided level II evidence. Of these 5 articles with the strongest available evidence, data from 2 did not support a correlation between hip dysplasia and hip pain, 2 supported a correlation, and 1 was equivocal. CONCLUSIONS: Even the best available evidence on spastic hip dysplasia and pain reveals no consensus or conclusion on whether spastic hip dysplasia and dislocation in total-involvement CP is independently correlated with pain. LEVEL OF EVIDENCE: Level III-Prognostic study.

Dorsal Rhizotomy in the Pediatric Patient.

SUMMARY: The majority of cases of dorsal rhizotomy surgeries in children are done to improve the spasticity associated with cerebral palsy, and more recent techniques are selective in nature and referred to as selective dorsal rhizotomy (SDR). The techniques applied to selective dorsal rhizotomy surgery has changed since it was first described and continues to undergo modifications. Approaches to surgery and monitoring vary slightly among centers. This article provides a review of the rationale, variety of surgical approaches, and intraoperative neurophysiologic monitoring methods used along with discussion of the risks, complications and outcomes in these surgeries.

Histological chorioamnionitis and pathological stages on very preterm infant outcomes.

AIMS: Histological chorioamnionitis (HCA) is a condition linked to preterm birth and neonatal infection and its relationship with various pathological stages in extremely preterm neonates, and with their associated short- and long-term consequences, remains a subject of research. This study investigated the connection between different pathological stages of HCA and both short-term complications and long-term outcomes in preterm infants born at or before 32 weeks of gestational age. METHODS: Preterm infants born at ≤ 32 weeks of gestation who underwent placental pathology evaluation and were followed-up at 18-24 months of corrected age were included. Neonates were classified based on their exposure to HCA and were further subdivided into different groups according to maternal inflammatory responses (MIR) and fetal inflammatory responses (FIR) stages. We compared short-term complications during their hospital stay between the HCA-exposed and -unexposed groups and examined the influence of HCA stages on long-term outcomes. RESULTS: The HCA group exhibited distinct characteristics such as higher rates of premature rupture of membranes > 18 h, reduced amniotic fluid, early-onset sepsis, bronchopulmonary dysplasia and intraventricular haemorrhage (IVH) grades III-IV (P < 0.05). The moderate-severe HCA group displayed lower gestational age, lower birth weight and higher incidence of IVH (grades III-IV) and preterm sepsis compared with the mild HCA group (P < 0.05). After adjusting for confounders, the MIR stages 2-3 group showed associations with cognitive impairment and cerebral palsy (P < 0.05), and the FIR stages 2-3 group also showed poor long-term outcomes and cognitive impairment (P < 0.05). CONCLUSIONS: Moderate-severe HCA was associated with increased early-onset sepsis, severe IVH and poor long-term outcomes, including cognitive impairment and cerebral palsy. Vigilant prevention strategies are warranted for severe HCA cases in order to mitigate poorer clinical outcomes.

Therapeutic effects of extracorporeal shock wave therapy on patients with spastic cerebral palsy and Rett syndrome: clinical and ultrasonographic findings.

BACKGROUND: Extracorporeal shock wave therapy (ESWT) is reportedly effective for improving spasticity and motor function in children with cerebral palsy (CP). Because late-stage Rett syndrome has a similar presentation, this study aimed to investigate the effects of ESWT on these two diseases. MATERIAL AND METHODS: Patients diagnosed with spastic CP and Rett syndrome received 1500 impulses of ESWT at 4 Hz and 0.1 mJ/mm2, on their spastic legsonce weekly for a total of 12 weeks. Outcomes were assessed before and 4 and 12 weeks after ESWT. Clinical assessments included the Modified Ashworth Scale (MAS), passive range of motion (PROM), and Gross Motor Function Measure 88 (GMFM-88). Ultrasonographic assessments included muscle thickness, acoustic radiation force impulse (ARFI), and strain elastography. RESULTS: Fifteen patients with CP and six with Rett syndrome were enrolled in this study. After ESWT, patients with CP showed significant clinical improvement in the MAS (P = 0.011), ankle PROM (P = 0.002), walking/running/jumping function (P = 0.003), and total function (P < 0.001) of the GMFM-88. The patients with Rett syndrome showed improved MAS scores (P = 0.061) and significantly improved total gross motor function (P = 0.030). Under ARFI, patients with CP demonstrated decreased shear wave speed in the gastrocnemius medial head (P = 0.038). Conversely, patients with Rett syndrome show increased shear-wave speeds after ESWT. CONCLUSION: Our study provides evidence that a weekly course of low-dose ESWT for 12 weeks is beneficial for children with both CP and Rett syndrome, with the clinical effects of reducing spasticity and improving the gross motor function of the lower limbs. The ARFI sonoelastography reveals improvement of muscle stiffness in patients with CP after ESWT, but deteriorated in patients with Rett syndrome. The diverse therapeutic response to ESWT may be caused by the MECP2 mutation in Rett syndrome, having a continuous impact and driving the pathophysiology differently as compared to CP, which is secondary to a static insult. Trial registration IRB 201700462A3. Registered 22March 2017, https://cghhrpms.cgmh.org.tw/HRPMS/Default.aspx .

The relationship between hip displacement, scoliosis, and pelvic obliquity in 106 nonambulatory children with cerebral palsy: a longitudinal retrospective population-based study.

BACKGROUND AND PURPOSE: The relationship between hip displacement (HD), pelvic obliquity (PO), and scoliosis in nonambulatory children with cerebral palsy (CP) has not been clearly elucidated. The aims of this population-based study were to examine the prevalence and temporal sequence of these deformities in nonambulatory children with CP and to evaluate how probable it is that severe unilateral HD contributes to development of scoliosis. PATIENTS AND METHODS: This longitudinal study comprised 106 nonambulatory children, enrolled in a surveillance program. Pelvic radiographs for measurements of migration percentage (MP) and PO were taken once a year from the diagnosis of HD. Spine radiographs were taken in patients with clinically detected scoliosis. Radiographic follow-up continued until skeletal maturity. RESULTS: Clinically significant scoliosis (Cobb angle ≥ 40°) occurred in 60 patients at a mean age of 11.8 years. 65 patients developed clinically significant HD (MP ≥ 40%) at a mean age of 4.8 years. 24 patients had no significant hip or spine deformities, 22 had HD only, 17 had scoliosis only, and 43 had both deformities. HD was diagnosed before scoliosis in all except 1 of the patients with both deformities. 14 of 19 patients with severe unilateral HD (MP ≥ 60%) had scoliosis convexity to the opposite side of the displaced hip. CONCLUSION: The combination of scoliosis and HD was frequent, and HD was diagnosed first in almost all the patients. HD might be a contributory cause of scoliosis in patients with severe, unilateral HD, PO, and later scoliosis with convexity to the opposite side.

The upper limb in children with cerebral palsy. Evaluation and treatment.

Management of the upper limb in children with cerebral palsy is often complex and must be carried out by a team experienced in this field. Several clinical parameters must be taken into consideration, such as higher functions, visual problems, overall upper limb function, motor control, sensitivity, presence of hemineglect or synkinesis, limb position at rest and during walking. And last but not least, a complete analysis of the upper limb is required. It is only after this exhaustive assessment - which often includes occupational therapy, physiotherapy and in some cases, video and electromyography evaluations - that a treatment indication can be discussed with the patient's family. Other than baseline treatment consisting of rehabilitation, occupational therapy and bracing, botulinum toxin injections could be an option, targeting specific muscle groups. Surgical treatments, which are often indicated in severe forms with contractures, are proposed after the patient's case is presented at a multidisciplinary meeting. These include selective neurotomy, muscle-tendon release, transfer or lengthening, and procedures on bone and joints (osteotomy, arthrodesis). LEVEL OF EVIDENCE: Expert opinion.

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.