Mortality after spinal fusion in children with cerebral palsy and cerebral-palsy-like conditions: A 30-year follow-up study.

AIM: To report survival probability of a large cohort of children with cerebral palsy (CP) after spinal fusion. METHOD: All children with CP who had spinal fusion between 1988 and 2018 at the reporting facility were reviewed for survival. Death records of the institutional CP database, institutional electronic medical records, publicly available obituaries, and the National Death Index through the US Centers for Disease Control were searched. Survival probabilities with different surgical eras, comorbidities, ages, and curve severities were compared using Kaplan-Meier curves. RESULTS: A total of 787 children (402 females, 385 males) had spinal fusion at a mean age of 14 years 1 month (standard deviation 3 years 2 months). The 30-year estimated survival was approximately 30%. Survival decreased for children who had spinal fusion at younger ages, longer postoperative hospital stays, longer postoperative intensive care unit stays, gastrostomy tubes, and pulmonary comorbidities. INTERPRETATION: Children with CP who required spinal fusions had reduced long-term survival compared with an age-matched typically developing cohort; however, a substantial number survived 20 to 30 years after the surgery. This study had no comparison group of children with CP scoliosis; therefore, we do not know whether correction of scoliosis affected their survival.

Walking activity after multilevel orthopedic surgery in children with cerebral palsy.

AIM: To determine how surgical burden and preoperative factors affect the recovery of walking activity after multilevel orthopedic surgery (MLS). METHOD: In this retrospective study, inclusion criteria were a diagnosis of cerebral palsy, MLS, and walking activity monitoring using a StepWatch device within 12 months pre-MLS and 24 months post-MLS. The outcome measure was total mean strides per day normalized to age and Gross Motor Function Classification System level. Pre- and postoperative walking activity were compared using unpaired t-tests; the effects of preoperative predictors and surgical burden on the recovery of walking activity were evaluated using regression analysis. RESULTS: Participants included 178 children (mean age 12 years 10 months [SD 8 years 7 months; range 4-20 years]; 91 males, 87 females). On average, children returned to baseline walking activity 3 months after low-burden surgery and 1 year 2 months after high-burden surgery. Postoperative walking activity was higher for children who had surgery at a younger age and those with a higher preoperative mobility function. INTERPRETATION: The burden of MLS was found to be inversely related to the time to recovery of postoperative walking activity. These findings provide evidence to help clinicians set expectations for return to function post-MLS. Further study is necessary to investigate the impact of postoperative factors on walking activity recovery. WHAT THIS PAPER ADDS: High-burden surgeries lead to longer recovery than low-burden surgeries. Younger children recover walking activity faster after multilevel orthopedic surgery. Children with high preoperative mobility function recover walking activity faster after surgery.

OBJETIVO: Determinar como a carga cirúrgica e os fatores pré-operatórios afetam a recuperação da atividade de caminhada após cirurgia ortopédica multinível (MLS). MÉTODOS: Neste estudo retrospectivo, os critérios de inclusão foram um diagnóstico de paralisia cerebral, MLS e monitoramento da atividade de caminhada usando um dispositivo Step Watch dentro de 12 meses pré-MLS e 24 meses pós-MLS. A medida de resultado foi o total de passos médios por dia normalizados para idade e nível do Sistema de Classificação da Função Motora Grossa. A atividade de caminhada pré e pós-operatória foi comparada usando testes t não pareados; os efeitos dos preditores pré-operatórios e da carga cirúrgica na recuperação da atividade de caminhada foram avaliados por meio de análise de regressão. RESULTADOS: Os participantes incluíram 178 crianças (idade média de 12 anos e 10 meses [DP 8 anos e 7 meses; intervalo de 4 a 20 anos]; 91 meninos, 87 meninas). Em média, as crianças retornaram à atividade de caminhada inicial 3 meses após a cirurgia de baixa carga e 1 ano e 2 meses após a cirurgia de alta carga. A atividade de caminhada pós-operatória foi maior para crianças que foram operadas em idade mais jovem e aquelas com maior função de mobilidade pré-operatória. INTERPRETAÇÃO: A carga de MLS foi inversamente relacionada ao tempo de recuperação da atividade de caminhada pós-operatória. Esses achados fornecem evidências objetivas para ajudar os médicos a definir as expectativas de retorno à função pós-MLS. Mais estudos são necessários para investigar o impacto dos fatores pós-operatórios na recuperação da atividade de caminhada.

Prevalence and Risk Factors Associated With Pelvic Rod/Screw Radiographic Lucency Following Scoliosis Surgery in Spastic Cerebral Palsy: A Longitudinal Study.

BACKGROUND: Radiographic lucency around a smooth pelvic rod (Galveston/unit rod technique) or sacroiliac/iliac screw following spinal fusion in children with nonambulatory spastic cerebral palsy (CP) has been described as a "windshield wiper" phenomenon. We evaluated demographics, radiographs, and complications in 101 cases from a single center to determine prevalence, risk factors, and complications associated with persistent radiographic lucency from 1 to 5 years following spinal fusion. METHODS: Inclusion criteria were diagnosis of nonambulatory spastic quadriplegic CP [Gross Motor Function Classification System (GMFCS) IV-V], under 18 years of age, scoliosis treated by posterior fusion from upper thoracic to sacrum with pelvic fixation (Galveston rod, iliac screw, or sacroiliac screw), adequate radiographs (preoperative, immediate postoperative, first-year, and second-year), and minimum 5-year follow-up. We evaluated demographics, radiographic parameters, comorbidities, scoliosis curve type, type of pelvic screw/rod, use of off-set connector, screw width, associated with posterior column osteotomy and/or additional anterior spinal release concurrent with posterior spine fusion, and infection over the follow-up period. Specific attention was given to the area and shape of the radiographic lucency. The logistic regression analysis was performed for continuous and categorical variables to define risk factors ( P =0.05). RESULTS: In 101 patients, data were collected at mean intervals of 1-year, 2-year, and >5-year follow-up and were 12.9±1.5, 25.8±2.5, and 81.5±23.0 months, respectively. Prevalence of pelvic rod/screw radiographic lucency was unchanged at 33%, 35%, and 24% at 1-year, 2-year, and >5-year follow-up, respectively, and radiographic parameters did not change ( P >0.05). Furthermore, no risk factors or complications were associated with radiographic lucency around pelvic rods/screws ( P >0.05). CONCLUSION: In patients with spastic nonambulatory CP who had scoliosis treated with posterior spinal fusion from upper thorax to pelvis, the prevalence of pelvic rod/screw lucency is high. Persistent lucency >2 mm around pelvic implants is not clinically significant, does not warrant advanced imaging, or indicate a complication if stable over time and wider distally than proximally. LEVEL OF EVIDENCE: Level III.

Hip Displacement in MECP2 Disorders: Prevalence and Risk Factors.

BACKGROUND: Methyl-CpG binding protein 2 (MECP2) disorders, including Rett syndrome and MECP2 duplication syndrome, are typified by profound intellectual disability, spasticity, and decline in gross motor function. Unlike scoliosis, linked to disease severity, little has been reported regarding the hip. The aim of this study was to report the prevalence and risk factors of hip displacement (HD) in MECP2 disorders. METHODS: This was a retrospective, comparative study. Children with a genetically confirmed MECP2 disorder were included. The primary outcome measure was the prevalence of HD (migration percentage>30%). Secondary outcomes included age at HD onset, ambulatory status, presence of clinically relevant scoliosis, genetic severity, presence of seizures, and associated comorbidities. Analysis of proportions of categorical variables was performed using χ2 testing (P=0.05). RESULTS: Fifty-six patients (54 Rett syndrome and 2 MECP2 duplication syndrome), diagnosed at 6.6 (SD: 4.7) years, met the inclusion criteria. The prevalence of HD was 36% [onset, 7.7 (SD: 3.8) y]. Risk factors for HD were nonwalker status (P=0.04), scoliosis (P=0.001), and refractory epilepsy (P=0.04). CONCLUSIONS: The prevalence of HD in MECP2 disorders is comparable to cerebral palsy, associated with proxy measures of disease severity. These results can be used to develop hip surveillance programs for MECP2 disorders, allowing for timely management. LEVEL OF EVIDENCE: Level III.

Identifying Opportunities for Early Detection of Cerebral Palsy.

This study aimed to evaluate assessment and referral practices for the early detection and diagnosis of children at risk for or with cerebral palsy (CP) by health care and education providers in Maryland and Delaware. A secondary aim was to identify barriers for using early detection tools and identify opportunities for change to support early diagnosis and improve care. Seventy-two participants answered ≥ 50% of the survey questions. Most were occupational or physical therapists (86%) working in early intervention (61%). Eighty-eight percent indicated awareness that CP can be diagnosed by 12 months. Though 86% stated they typically suspect a diagnosis of CP between 0 and 12 months, only 19% reported that their patients received a CP diagnosis < 12 months. The Developmental Assessment of Young Children (73%) and the Peabody Developmental Motor Scales-2 (59%) were used most. Many respondents indicated never using magnetic resonance imaging (70%), the General Movements Assessment (87%), or the Hammersmith Infant Neurological Exam (69%). Participants identified clinical signs and symptoms prompting a referral for the diagnostic assessment of CP, most commonly stiffness in legs (95%), excessive head lag (93%), and persistent fisting (92%). Policy and organizational change, clinician education, and training are needed to support the implementation of CP early detection guidelines.

Closed displaced femur fractures in children with nonambulatory cerebral palsy.

Femoral fractures in children withcerebral palsy (CP) represent a frequent medical problem, and treatment represents a challenge. The purpose of this study was to review the closed displaced femoral fractures in our population of nonambulatory children with CP to compare the results of nonoperative and operative treatment modalities to improve the care of these children. From 2006 to 2020, children with nonambulatory CP were selected with inclusion criteria of displaced femoral fracture and were divided into nonoperative and operative groups. Forty-four children met the inclusion criteria. The nonoperative group included 23 children and the operative group included 21 children. Mechanism of injury was unknown in 48% of the fractures. Fourteen (25%) fractures occurred after a femoral plate fixation during a reconstructive hip surgery, and 38 (86%) children had osteopenia. Our results reveal a high prevalence of osteopenia, low-energy trauma, malunion in nonoperative treatment, and peri-implant fractures. Suspicion of child abuse should be considered when the fracture has an unclear mechanism of the injury. Removal of proximal femoral implants may be considered to prevent peri-implant fractures. Femoral fractures should preferably be treated nonoperatively. Operative treatment should be considered for diaphyseal fractures in children capable of standing transfers, larger children, children with more severe spasticity or movement disorder or those who have suffered a high-energy fracture. Due to the high prevalence of proximal fractures in the presence of hardware, operative treatment is usually required for these fractures. In contrast, distal fractures are adequately managed nonoperatively.

Does the Addition of Intrathecal Baclofen Along with or After Soft-Tissue Hip Adductor Surgery Decrease the Need for Hip Reconstruction Compared with Soft-Tissue Surgery Alone for Children with Nonambulatory Cerebral Palsy?

Purpose: Intrathecal baclofen (ITB) is a well-known treatment option for cerebral palsy (CP) spasticity. The combination of soft-tissue release and ITB for spasticity is common. This study compared patients who had soft-tissue release before ITB (PRE-ITB), soft-tissue release at the same time as ITB (ST-ITB), and no ITB (NON-ITB) but had soft-tissue release at a similar age as PRE-ITB. Methods: Inclusion criteria were a spastic or mixed nonambulatory CP diagnosis, prior hip adductor surgery, no prior reconstructive surgery, and at least a five-year post-operative follow-up. Thirty hips were identified as PRE-ITB, 20 hips as ST-ITB, and 43 hips as NON-ITB. The primary outcome variables were the subsequent hip surgery during the study period and/or a migration percentage ≥ 50% at the final follow-up defined as "unsuccessful hip." Results: The mean follow-up duration was 9.0 years (SD 2.4) for PRE-ITB, 9.4 (SD 3.6) for ST-ITB, and 9.3 (SD 3) for NON-ITB. The odds of unsuccessful outcomes were not different between NON-ITB and PRE-ITB but were lower for the ST-ITB group. The need for subsequent osteotomies or revision adductor surgery was significantly higher in ST-ITB compared with PRE-ITB (p = 0.02) or NON-ITB (p = 0.015). The incidence of surgical site infection over the whole follow-up period was higher in ST-ITB (40%) compared with PRE-ITB (13.3%, p = 0.035) and NON-ITB (0, p < 0.001). Conclusion: The addition of tone management with ITB did not reduce the need for later hip surgery but did increase the risk for surgical site infection.

The influence of tone on proximal femoral and acetabular geometry in neuromuscular hip displacement: A comparison of cerebral palsy and spinal muscular atrophy.

Purpose: The aim of this article was to compare longitudinal changes in hip morphology in cerebral palsy (hypertonic) and spinal muscular atrophy (hypotonic) to examine the influence of muscle tone on development of hip displacement. Methods: Children with spinal muscular atrophy (Types I and II) and cerebral palsy (Gross Motor Function Classification System IV and V) with hip displacement (migration percentage >30%) were included. Head shaft angle, migration percentage, and acetabular index were measured at T1 (1-2.5 years), T2 (3-5 years), and T3 (6-8 years). Analysis of variance testing and linear regression were utilized. Results: Sixty patients (cerebral palsy, N = 41; spinal muscular atrophy, N = 19) were included. Hip displacement occurred earlier in spinal muscular atrophy (34 months) than cerebral palsy (49 months) (p = 0.003). Head shaft angle was high and did not change between T1, T2, and T3, but significant changes in migration percentage were found (cerebral palsy: 23%, 36%, 45% (p < 0.01) and spinal muscular atrophy: 37%, 57%, 61% (p = 0.02)). Migration percentage increased by age in cerebral palsy (r = 0.41, p < 0.001), but not in spinal muscular atrophy (r = 0.18, p = 0.09). Acetabular index increased with migration percentage (cerebral palsy: r = 0.41, p < 0.001; spinal muscular atrophy: r = 0.48, p < 0.001). Conclusion: Persistent lateral physeal tilt by head shaft angle was found for both spinal muscular atrophy and cerebral palsy. Abnormal physeal alignment may be causally related to weakness of the hip abductor muscles rather than spasticity or muscle imbalance, resulting in coxa valga and secondary acetabular dysplasia. Level of evidence: III (case-control study).

No difference in postoperative complication rates or cardiopulmonary function for early versus late scoliosis correction in Duchenne muscular dystrophy.

BACKGROUND: Given reduced rates of both pulmonary function decline and scoliosis progression with steroid treatment in Duchenne muscular dystrophy (DMD), the role of early scoliosis surgery has been questioned. The purpose of this study was to compare the postoperative complication rates of early versus late scoliosis correction in DMD. METHODS: This study was a retrospective cohort, conducted at an academic tertiary level children's hospital. Patients with DMD who underwent posterior scoliosis correction, with preoperative pulmonary function testing [forced vital capacity (FVC)] were included and divided into two groups by preoperative curve angles: ≤ 45° and > 45°. The primary outcome variable was postoperative complications by Clavien-Dindo classification grading. Secondary outcome variables included postoperative complications occurring after the first 90 days, age at surgery, duration of wheelchair dependency preoperatively, pulmonary function, steroid utilization, shortening fraction by echocardiogram, surgery duration, intensive care unit/hospital length of stay, days intubated, infection, and percent curve correction. Two-tailed t-test and Chi-square testing were used for analysis of patient factors and Clavien-Dindo complication grade, respectively. RESULTS: Thirty-one patients were included with a total follow-up of 8.3 ± 3.2 years, 4.8 ± 2.2 years post-spinal fusion. Steroid treatment (prednisone, deflazacort) was utilized for 21 (67.7%) patients. Primary curve correction was not different between groups (65.0% vs 71.4% [p = 0.37]). There were no significant differences in Clavien-Dindo classification grades between groups (p > 0.05). For the entire cohort, the overall complication rate was higher for patients with steroid treatment (61.9% vs 10.0% [p = 0.008]). Neither forced vital capacity nor fractional shortening on echocardiogram was different between groups at final follow-up (p = 0.6 and p = 0.4, respectively). CONCLUSION: The comparable risk of perioperative complications for early and late scoliosis correction supports a "watchful waiting" approach, whereby curves less than 45° can be carefully followed while cardiopulmonary function is maintained. Patients undergoing steroid treatment should be counseled regarding the higher risk of postoperative blood transfusion and deep wound infection. LEVEL OF EVIDENCE: III Retrospective cohort.

Neuromuscular Adaptations in Gluteus Medius Activity Following Resurfacing and Total Hip Arthroplasty.

In middle-aged patients, the choice between resurfacing and total hip arthroplasty may be difficult given recent studies showing differences in functional outcomes. Success of clinical outcomes, defined by a greater range of motion and reduced pain following surgery, are dependent on a return in function of incised muscle groups. To identify neuromuscular recovery following hip arthroplasty, hip abductor activity was assessed throughout the first year of recovery. Analysis focused on characterization of the temporal activity of the gluteus medius during activities of daily living. Adaptation in muscle firing and biomechanical outcomes was revealed in both groups, though more pronounced in the THA cohort with increasingly difficult activities. Differences between groups should be considered by clinicians when considering the best treatment options for their patients.

Gait, hip strength and functional outcomes after a Ganz periacetabular osteotomy for adolescent hip dysplasia.

BACKGROUND: The Ganz (Bernese) periacetabular osteotomy was first described for the treatment of adult patients with hip dysplasia; however, it has also been used for adolescent patients. Its effectiveness in improving patients in the short term has not been fully established and no studies, to our knowledge, have analyzed gait and functional outcome in patients with adolescent hip dysplasia. METHODS: An Institutional Review Board-approved prospective study of a consecutive series of patients undergoing a Ganz (Bernese) periacetabular osteotomy for adolescent hip dysplasia was carried out. Standard radiographic parameters were analyzed. Harris hip scores, gait analysis, hip abductor, and flexion strength testing preoperatively and at 6 months and 1 year postoperatively were performed. Statistical analysis compared the 6-month and 1-year data to the preoperative values and a second analysis was performed comparing the patients with hip dysplasia to normative data of similarly aged patients. RESULTS: Twenty-one patients (24 hips) underwent a Ganz periacetabular osteotomy at an average age of 16.1 years for a primary diagnosis of hip dysplasia and pain. There was a significant improvement in all radiographic parameters from preoperation to 1 year in lateral center edge angle (5.5 to 33.0 degrees) (P<0.05), acetabular index of the weight-bearing zone (29.0 to 10.5 degrees) (P<0.05), ventral center edge angle (-1.1 to 32.5 degrees) (P<0.05), and medialization of the joint center relative to the contralateral side (1.12 to 1.05) (P<0.05). Isokinetic abductor strength decreased initially from 62 Nm/kg preoperatively to 58 Nm/kg at 6 months but was improved to 64 Nm/kg at 1 year. Flexion strength decreased from 92 Nm/kg preoperatively to 58 Nm/kg at 6 months but improved to 69 Nm/kg at 1 year. Abductor impulse decreased from 0.26 Nm/kg-s preoperatively to 0.23 Nm/kg-s at 6 months but improved to 0.29 Nm/kg-s at 1 year. Flexion pull-off power decreased from 1.35 Watts/kg to 1.27 Watts/kg and then improved to 1.29 Watts/kg. The Harris hip scores improved from 64.6 at the preoperative evaluation to 74.5 at the 1-year evaluation (Max 89). CONCLUSIONS: The Bernese periacetabular osteotomy is effective in significantly improving radiographic parameters for adolescent hip dysplasia with improvement in functional outcome. Because the osteotomy allows for medialization of the hip joint center and limited abductor muscle dissection the abductor strength improved at follow-up, however, the hip flexion pull-off was decreased. Strategies to improve hip flexion power preoperatively and postoperatively in the future are necessary. LEVEL OF EVIDENCE: Level IV.

Who Needs a Pediatric Intensive Care Unit After Posterior Spinal Fusion for Adolescent Idiopathic Scoliosis?

BACKGROUND: Hypotensive events (HEs) following posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS) can lead to delayed neurologic postoperative deficits (DNPD). This study aimed to determine the incidence of HEs (mean arterial pressure [MAP] <60 mmHg) after PSF for AIS and identify predictors for HEs. METHODS: Medical records of 99 consecutive patients who had PSF for AIS were retrospectively reviewed (2011-2013). Perioperative data were collected. Patients were divided into two groups based on MAP readings by an arterial line in the pediatric intensive care unit immediately postoperatively into postoperative day one: Group 1 (MAP ≥60 mmHg) and Group 2 (multiple occurrences of MAPs <60 mmHg). Mean values were compared using the independent t test. Multiple logistic regression was used to estimate the association of preoperative and intraoperative parameters with multiple HEs. RESULTS: Group 1 had 68 patients (68.7%) and Group 2 had 31 patients (31.3%). None of the compared parameters were associated significantly with multiple HE. However, patients who did not exhibit HEs within the first four hours postoperatively remained stable throughout the rest of the postoperative period. Only those with HEs in the first four hours experienced subsequent HEs in the first 24 hours. There were no DNPD or other major complications. CONCLUSION: Results showed that the incidence of HEs after PSF in AIS can be as high as 31.3%. We did not find any significant risk factors. Although DNPD after PSF is a rare complication and we had none in this series, we suggest that these patients with multiple HEs may be at risk for DNPD as a result of hypotension and potential for cord ischemia. Therefore, all patients after PSF should be monitored in a pediatric intensive care unit-type environment or postanesthesia recovery room initially. If stable for, at least, the initial four hours, then patients should be good candidates for a less intensive environment. LEVEL OF EVIDENCE: Level 3.