This review will provide a comprehensive, up-to-date review of the current knowledge regarding the pathophysiology of muscle contractures in cerebral palsy. Although much has been known about the clinical manifestations of both dynamic and static muscle contractures, until recently, little was known about the underlying mechanisms for the development of such contractures. In particular, recent basic science and imaging studies have reported an upregulation of collagen content associated with muscle stiffness. Paradoxically, contractile elements such as myofibrils have been found to be highly elastic, possibly an adaptation to a muscle that is under significantin vivotension. Sarcomeres have also been reported to be excessively long, likely responsible for the poor force generating capacity and underlying weakness seen in children with cerebral palsy (CP). Overall muscle volume and length have been found to be decreased in CP, likely secondary to abnormalities in sarcomerogenesis. Recent animal and clinical work has suggested that the use of botulinum toxin for spasticity management has been shown to increase muscle atrophy and fibrofatty content in the CP muscle. Given that the CP muscle is short and small already, this calls into question the use of such agents for spasticity management given the functional and histological cost of such interventions. Recent theories involving muscle homeostasis, epigenetic mechanisms, and inflammatory mediators of regulation have added to our emerging understanding of this complicated area.
» Sarcopenia is common in both the elderly and children with cerebral palsy. » Children with cerebral palsy have muscles that are much smaller than muscles in typically developing peers. » Injections of botulinum toxin type A (BoNT-A) result in acute muscle atrophy in animal models and in human subjects. » It is not known when or if muscles recover fully after injection of BoNT-A. » These findings have implications for management protocols.
Hip displacement is the second most common musculoskeletal deformity in children with cerebral palsy. Hip surveillance programs have been implemented in many countries to detect hip displacement early when it is usually asymptomatic. The aim of hip surveillance is to monitor hip development to offer management options to slow or reverse hip displacement, and to provide the best opportunity for good hip health at skeletal maturity. The long-term goal is to avoid the sequelae of late hip dislocation which may include pain, fixed deformity, loss of function and impaired quality of life. The focus of this review is on areas of disagreement, areas where evidence is lacking, ethical dilemmas and areas for future research. There is already broad agreement on how to conduct hip surveillance, using a combination of standardised physical examination measures and radiographic examination of the hips. The frequency is dictated by the risk of hip displacement according to the child’s ambulatory status. Management of both early and late hip displacement is more controversial and the evidence base in key areas is relatively weak. In this review, we summarise the recent literature on hip surveillance and highlight the management dilemmas and controversies. Better understanding of the causes of hip displacement may lead to interventions which target the pathophysiology of hip displacement and the pathological anatomy of the hip in children with cerebral palsy. We have identified the need for more effective and integrated management from early childhood to skeletal maturity. Areas for future research are highlighted and a range of ethical and management dilemmas are discussed.
Background: A better understanding of early pain trajectories (patterns) following scoliosis surgery and how they relate to baseline patient characteristics and functional outcomes may allow for the development of mitigating strategies to improve patient outcomes. Methods: This was a prospective cohort study. Adolescents with idiopathic scoliosis were recruited across multiple centers. Latent growth mixture modeling techniques were used to determine pain trajectories over the first postoperative year. Results: The median numerical rating scale for pain in the hospital following surgery for adolescent idiopathic scoliosis was 5.0. It improved to 1.0 by 6 weeks, and was maintained at <1 by 3 to 12 months postoperatively. Three trajectories were identified, 2 of which involved moderate acute postoperative pain: 1 with good resolution and 1 with incomplete resolution by 1 year. The third trajectory involved mild acute postoperative pain with good resolution by 1 year. Membership in the “moderate pain with incomplete resolution” trajectory was predicted by higher baseline pain and anxiety, and patients in this trajectory reported worse quality of life than those in the trajectories with good resolution. Conclusions: Pain recovery following surgery for idiopathic scoliosis was found to be substantial during the first 6 weeks and continued up to 1 year. We identified 3 main trajectories, 2 with favorable outcomes and 1 with persistent pain and worse quality of life at 1 year postoperatively. The risk factors most associated with the latter trajectory included increased baseline pain and anxiety. Level of Evidence: Prognostic Level II. See Instructions for Authors for a complete description of levels of evidence.
The Ponseti method of congenital idiopathic clubfoot correction has traditionally specified plaster of Paris (POP) as the cast material of choice; however, there are negative aspects to using POP. We sought to determine the influence of cast material (POP v. semirigid fibreglass [SRF]) on clubfoot correction using the Ponseti method. Patients were randomized to POP or SRF before undergoing the Ponseti method. The primary outcome measure was the number of casts required for clubfoot correction. Secondary outcome measures included the number of casts by severity, ease of cast removal, need for Achilles tenotomy, brace compliance, deformity relapse, need for repeat casting and need for ancillary surgical procedures. We enrolled 30 patients: 12 randomized to POP and 18 to SRF. There was no difference in the number of casts required for clubfoot correction between the groups (p = 0.13). According to parents, removal of POP was more difficult (p < 0.001), more time consuming (p < 0.001) and required more than 1 method (p < 0.001). At a final follow-up of 30.8 months, the mean times to deformity relapse requiring repeat casting, surgery or both were 18.7 and 16.4 months for the SRF and POP groups, respectively. There was no significant difference in the number of casts required for correction of clubfoot between the 2 materials, but SRF resulted in a more favourable parental experience, which cannot be ignored as it may have a positive impact on psychological well-being despite the increased cost associated.
9 September 2021 in Developmental Medicine & Child Neurology