Multilevel surgery (MLS), which is also referred to as single-event multilevel surgery (SEMLS), is when 4 or more orthopedic surgical procedures are performed in 1 session in an operating room. There is then one extended period of rehabilitation time after this, rather than the surgeries being performed individually with their own rehabilitation/recovery time after each procedure. MLS has become the standard approach to orthopedic surgery in children with bilateral spastic cerebral palsy. The objective of this study was to examine the effects of MLS on long-term gait outcomes at skeletal maturity in ambulatory individuals with bilateral spastic cerebral palsy. Secondarily, this study also sought to examine the safety of MLS. This was a retrospective study of individuals from 3 centers. Clinical evaluations and follow-ups and standardized gait data were evaluated. Results demonstrated that 94% of children did not have any change in their Gross Motor Function Classification System (GMFCS) score. The authors reported that among the 231 children in this study, 77% showed a clinically-significant improvement in long term (with a mean of approximately 9 years after MLS) improvement in Gait Profile Score (GPS). They note a limitation that there was no non-MLS group in the study to compare outcomes in a group without surgery. Surgical adverse events were recorded in 47% of the individuals in this study. 7/231 individuals had a grade III adverse event and 1/231 had a permanent grade IV adverse event, without any grade V complications recorded. This study is noted to be the largest outcome study with the longest follow-up after MLS. The authors conclude that MLS is safe and effective for ambulatory children with bilateral spastic cerebral palsy. This study can be a helpful reference when talking to families about the risks and benefits of MLS.
While consensus papers on intrathecal baclofen (ITB) have addressed treatment of both spasticity and dystonia, most research on the topic focuses on spasticity rather than dystonia. The aim of this study was to examine the effect of intrathecal baclofen on function and activity of individuals with dyskinetic cerebral palsy. This was a retrospective chart review of individuals with dyskinetic cerebral palsy who were treated with intrathecal baclofen at a single site between 2004 and 2013. Parental grading of activities of daily living and pain and provider grading of dystonia, spasticity, and passive range of motion before and after intrathecal baclofen were collected. Data from 25 individuals was included in the study. Functional follow-up occurred at a mean of 1.5 years after intrathecal baclofen implantation. 20/25 individuals were reported to have improved activities of daily living (with 16/22 having improved sitting and 17/25 having improved communication) and 13/25 having improved pain scores. Using the Barry-Albright Dystonia Scale, dystonia improved from a mean of 7.1 (SD 5.2) to 1.5 (SD 1.8) at rest and improved from a mean of 19.2 (SD 3.4) to 9.9 (SD 3.7) with activity. Spasticity (using the Modified Ashworth Scale) improved in 19/25 individuals. Only small changes were seen in passive range of motion after intrathecal baclofen (increased in some individuals and decreased in others). 5/25 participants required catheter revisions during the study period. One individual required intrathecal baclofen pump explanation due to an infection, with a new pump implanted at a later time. There was subcutaneous leakage of cerebrospinal fluid post-operatively, which was treated with compression only, in 10/25 individuals. This study demonstrated that intrathecal baclofen can have a positive impact on activities of daily living and dystonia in individuals with dyskinetic cerebral palsy. The authors acknowledged that a randomized control trial would be valuable for studying this topic further and noted that one is currently underway. This study can be a helpful reference when discussing the risks and benefits of intrathecal baclofen for individuals with dyskinetic cerebral palsy.
Early botulinum toxin injections may be considered in cases of cerebral palsy, obstetric brachial plexus palsy, clubfoot, or congenital torticollis. Botulinum toxin has a grade A level of evidence for reduction of focal spasticity in CP, and is recommended for treatment of lower limb muscles in CP. It is considered to be safe for children with CP >2 years of age, but there are limited recommendations for children < 2 years of age. This study reviews the current literature regarding the safety of intramuscular botulinum toxin injections in children with orthopedic and neurologic-related musculoskeletal disorders who are under 2 years of age. The authors also reviewed the evidence on the effectiveness of early Botox injections in different pathologies and different protocols used. Inclusion criteria for articles included at least 1 child under 2 years of age, safety-related or effectiveness outcome of botulinum toxin injections (BTI), BTI in the striated muscles of the neck, trunk, or limb, and articles must have been in English.
Results/Conclusions: 104 articles were selected for review, 17 of which had individual data on children under 2 years of age that could be extracted. 11 articles exclusively included children under 2 years of age. 3 included children with CP, 6 included children with OBPP, 4 included children with clubfoot, and 2 children with congenital torticollis. One study described a child with myelomeningocele with lower limb deformities, and 1 a child with zone II finger flexor tendon repair. A total of 473 children under 2 years of age received BTI in the striated muscles of the limbs and neck; 55 children with CP, 112 with obstetric brachial plexus palsy, 257 with clubfoot, and 44 with congenital torticollis. Two studies were RCTs, 1 with level 1 evidence and 1 with level 2. Four studies were prospective with level 3 and 4 evidence, 8 studies were retrospective with level 4 evidence, and 3 studies were case reports with level 5 evidence according to the modified Sackett scale. Three studies including 328 of the 473 infants included a systematic evaluation of adverse effects. One severe adverse effect of transverse myelitis after BTI for clubfoot was reported, but a link between the myelitis and BTI was excluded. The most frequently-reported adverse events were all mild, including pain at the point of injection, weakness in the injected muscle, asthenia, hematoma at injection point, and dysesthesia at the injection point. These were reported at rates varying between 3.7% and 25% in the various studies. In 1 study, 1 moderate adverse event of mild dysphagia after injection was reported out of 27 infants (3.7%). No relations were reported between age to time of injection, gender, protocol used, and frequency of events in any study.
One RCT with level 2 evidence including 15 children with CP, GMFCS I-II, with repeat BTI in the hamstrings at a mean age of 16 months described a significant increase in popliteal angle and decrease in spasticity 1 and 3.5 years later compared to control receiving daily stretching. No significant differences were seen in passive ankle dorsiflexion or spasticity of the ankle plantar flexors. Another study with level 4 evidence found no significant effect on the prevention of hip subluxation after BTI in a sample of 7 children ages 13-45 months. However, if adjusted for age and severity, the subgroup <2 years of age with a Reimers index <30% there was a statistically-significant mean improvement of 3% in the Reimers index after injection. Two studies evaluating motor function found no statistically-significant improvement in motor function on the PEDI or GMFM-66 at 1 and 3.5-year follow-up for children with CP.
In obstetric brachial plexus palsy, 1 study with level 4 evidence found BTI improved passive external rotation by a mean 26 degrees in 8 children, another showed improvement by 10.1 points of active ER on the active movement scale in 8 children, and another study with level 5 evidence found an increase in antagonist muscle strength after BTI in 4 infants on the Medical Research Council scale. A study with level 4 evidence showed 58.35 of infants with surgical indications had surgeries cancelled, modified or postponed after BTI. Another study with level 4 evidence had 24/35 infants injected for glen humeral subluxation had no recurrence at 1 year.
One RCT with level 1 evidence found no change in time in plaster or number or percutaneous tenotomies after BTI for children with clubfoot. One study with level 4 evidence and 2 with level 5 evidence found a significant increase of 30 to 50 degrees in ankle dorsiflexion in infants with combined treatment with casting and physiotherapy. Two studies with level 4 evidence found an increase in active and passive cervical motion in 75% of infants with congenital torticollis, and mean improvement of 11.9 degrees lateral rotation.
Botox was used more frequently than disport, with total doses ranging from 6 to 12 U/kg Botox or 30U/kg for Dysport for CP. For brachial plexus, the total maximum dose used was 10U/kg. In children with clubfoot, doses ranged from 5 to 11.6U/kg Botox. Pain management varied between topicals, general anesthesia, no anesthesia, or unspecified. Guidance varied between palpation, e-stim or EMG, or unspecified.
Based on these studies, the authors conclude that the safety of BTI in children under 2 appears similar to older children, though rates may vary based on pathology and injection site. There is not yet a high enough level of evidence to conclude effectiveness of BTI in these populations. More studies are needed.
Relevance: As providers managing these conditions and considering these interventions, it is important to be aware of the current state of the literature, and to consider how we can perform better studies to determine whether these interventions are both safe and effective for our patients.
The purpose of this study is to examine the prevalence of development of secondary attention deficit/hyperactivity disorder (SADHD) within 5-10 years after TBI, and associated risk factors. The authors conducted a prospective cohort study of 187 children (81 with TBI, 106 controls) ages 3-7 years who were hospitalized for either TBI, or orthopedic injuries for a control group. Study sites included 3 tertiary children’s hospitals and 1 general hospital in Ohio, and patients were enrolled between January 2003 to June 2008. Inclusion criteria included an accidental cause of injury, no preinjury neurologic problems or delays, and English as the primary language. Outcome assessments were the DSM-Oriented Attention-Deficit/Hyperactivity Problems Scale on a parent-reported Child Behavior Checklist, report of an ADHD diagnosis, and/or treatment eith stimulant medication not present at baseline assessment. Surveys were completed at baseline, 6 months, 12 months, 18 months, 3.4 years and 6.8 years after injury. The final assessment was completed after middle school transition with the expectation that deficits in executive function and attention would emerge at that time. ADHD was defined as Tscores greater than 65 on the DSM-Oriented Attention Deficit/Hyperactivity Problems Scale, parent-reported history of ADHD diagnosis or prescribed stimulant medication.
Of the original cohort of 221, 15 were excluded with uncomplicated mild TBI, 16 children with primary ADHD were excluded, and 3 children were missing parent rating scales at the baseline assessment, resulting in the cohort of 187 children. Mean age of injury was 5.1 years, 57.8% were male, and 26.7% were nonwhite race/ethnicity. Retention rates didn’t differ across injury groups. 48 children without preinjury ADHD met the definition of SADHD. 61.5% of those with severe TBI developed SADHD within the first year after injury, while 50.0% of those with moderate TBI and 53.8% with complicated mild TBI who developed SADHD developed it later than the first year after injury. Children with severe TBI had increased risk for development of SADHD compared to control, while mild and moderate TBI groups did not differ significantly. Although the risk of SADHD developing after complicated mild TBI and moderate TBI did not meet the threshold for statistical significance, all TBI severity groups demonstrated almost twice the risk for SADHD compared with controls. Lower maternal educational level and greater family dysfunction were significantly associated with risk for SADHD. The interaction of male sex and lower levels of maternal education were also associated with increased risk of SADHD.
Relevance: As providers managing children with TBI and the subsequent cognitive effects of TBI, this study provides important information on risk factors for SADHD that may help guide monitoring and expectations for symptoms, as well as for counseling of families. The study supports post-injury clinical monitoring.
The authors of this study sought to assess self-determination of adolescents with neonatal brachial plexus palsy (NBPP) compared with their typically-developing peers via self-reported measures of function due to the importance of self-determination on enhancing learning and improving postschool outcomes. Via a case-control design, they studied 20 adolescents with NBPP along with 20 age/gender-matched typically developing adolescents by measuring the American Institutes for Research (AIR) Self-Determination Scale and a physical assessment (AROM, Grip/pinch strength, MRC muscle strength, 9-Hole Peg Test, and Mallet scale). Parents of each participant completed a demographic survey. The authors found similar self-determination levels in adolescents with NBPP to those of typically-developing adolescents; however, all adolescents rated their opportunities to engage in self-determined behaviors at school significantly lower than at home. Given the results of this study, one would require further assessment of opportunities at school to gauge if self-determination is a factor that is limiting for adolescents with neonatal brachial plexus palsy in a school setting.
This cross-sectional designed study compared femoral cartilage thickness via ultrasound measurement in 40 patients with diplegic cerebral palsy to 51 healthy controls. They found a significant difference in the mean cartilage thickness measurements of the medial condyle and intercondylar area of knees in the cerebral palsy group with those patients classified as GMFCS V having the thinnest cartilage in all measured areas. They also found a moderate negative correlation between age and thickness measurements that did not exist in the healthy group. These findings support routine surveillance, as well as consideration of joint health problems in the assessment of pain of unknown etiology in children with cerebral palsy.