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.

Single-event multilevel surgery in cerebral palsy: Value added by a co-surgeon.

ABSTRACT: The aim of this study was to compare outcomes for single-event multilevel surgery (SEMLS) in cerebral palsy (CP) performed by 1 or 2 attending surgeons.A retrospective review of patients with CP undergoing SEMLS was performed. Patients undergoing SEMLS performed by a single senior surgeon were compared with patients undergoing SEMLS by the same senior surgeon and a consistent second attending surgeon. Due to heterogeneity of the type and quantity of SEMLS procedures included in this study, a scoring system was utilized to stratify patients to low and high surgical burden. The SEMLS events scoring less than 18 points were categorized as low burden surgery and SEMLS scoring 18 or more points were categorized as high burden surgery. Operative time, estimated blood loss, hospital length of stay, and operating room (OR) utilization costs were compared.In low burden SEMLS, 10 patients had SEMLS performed by a single surgeon and 8 patients had SEMLS performed by 2 surgeons. In high burden SEMLS, 10 patients had SEMLS performed by a single surgeon and 12 patients had SEMLS performed by 2 surgeons. For high burden SEMLS, operative time was decreased by a mean of 69 minutes in cases performed by 2 co-surgeons (P = 0.03). Decreased operative time was associated with an estimated savings of $2484 per SEMLS case. In low burden SEMLS, a trend toward decreased operative time was associated for cases performed by 2 co-surgeons (182 vs 221 minutes, P = 0.11). Decreased operative time was associated with an estimated savings of $1404 per low burden SEMLS case. No difference was found for estimated blood loss or hospital length of stay between groups in high and low burden SEMLS.Employing 2 attending surgeons in SEMLS decreased operative time and OR utilization cost, particularly in patients with a high surgical burden. These findings support the practice of utilizing 2 attending surgeons for SEMLS in patients with CP.Level of Evidence: Level III.

Black-White Risk Differentials in COVID-19 (SARS-COV2) Transmission, Mortality and Case Fatality in the United States: Translational Epidemiologic Perspective and Challenges.

BACKGROUND: Social and health inequities predispose vulnerable populations to adverse morbidity and mortality outcomes of epidemics and pandemics. While racial disparities in cumulative incidence (CmI) and mortality from the influenza pandemics of 1918 and 2009 implicated Blacks with survival disadvantage relative to Whites in the United States, COVID-19 currently indicates comparable disparities. We aimed to: (a) assess COVID-19 CmI by race, (b) determine the Black-White case fatality (CF) and risk differentials, and (c) apply explanatory model for mortality risk differentials. METHODS: COVID-19 data on confirmed cases and deaths by selective states health departments were assessed using a cross-sectional ecologic design. Chi-square was used for CF independence, while binomial regression model for the Black-White risk differentials. RESULTS: The COVID-19 mortality CmI indicated Blacks/AA with 34% of the total mortality in the United States, albeit their 13% population size. The COVID-19 CF was higher among Blacks/AA relative to Whites; Maryland, (2.7% vs. 2.5%), Wisconsin (7.4% vs. 4.8%), Illinois (4.8% vs. 4.2%), Chicago (5.9% vs. 3.2%), Detroit (Michigan), 7.2% and St. John the Baptist Parish (Louisiana), 7.9%. Blacks/AA compared to Whites in Michigan were 15% more likely to die, CmI risk ratio (CmIRR) = 1.15, 95% CI, 1.01-1.32. Blacks/AA relative to Whites in Illinois were 13% more likely to die, CmIRR = 1.13, 95% CI, 0.93-1.39, while Blacks/AA compared to Whites in Wisconsin were 51% more likely to die, CmIRR = 1.51, 95% CI, 1.10-2.10. In Chicago, Blacks/AA were more than twice as likely to die, CmIRR = 2.24, 95% CI, 1.36-3.88. CONCLUSION: Substantial racial/ethnic disparities are observed in COVID-19 CF and mortality with Blacks/AA disproportionately affected across the United States.

Implication of Vaginal and Cesarean Section Delivery Method in Black-White Differentials in Infant Mortality in the United States: Linked Birth/Infant Death Records, 2007-2016.

Racial/ethnic disparities in infant mortality (IM) continue to persist in the United States, with Black/African Americans (AA) being disproportionally affected with a three-fold increase in mortality compared to Whites. Epidemiological data have identified maternal characteristics in IM risk such as preeclampsia, eclampsia, maternal education, smoking, maternal weight, maternal socioeconomic status (SES), and family structure. Understanding the social gradient in health including implicit bias, as inherent in the method of labor and delivery and the racial heterogeneity, may facilitate intervention mapping in narrowing the Black-White IM risk differences. We aimed to assess the temporal/racial trends and the methods of delivery, mainly vaginal vs. cesarean section (C-section) as an exposure function of IM. The United States linked birth/infant death records (2007-2016) were used with a cross-sectional ecological design. The analysis involved chi squared statistic, incidence rate estimation by binomial regression model, and period percent change. Of the 40,445,070 births between 2007 and 2016, cumulative mortality incidence was 249,135 (1.16 per 1000). The IM rate was highest among Black/AA (11.41 per 1000), intermediate among Whites (5.19 per 1000), and lowest among Asian /Pacific Islanders (4.24 per 1000). The cumulative incidence rate difference, comparing vaginal to cesarean procedure was 1.73 per 1000 infants, implying excess IM with C-section. Compared to C-section, there was a 31% decreased risk of IM among mothers with vaginal delivery, rate ratio (RR) = 0.69, 95% confidence interval (CI): 0.64-0.74. Racial disparities were observed in the method of delivery associated with IM. Black/AA mothers with vaginal delivery had a 6% decreased risk of IM compared to C-section, RR = 0.94, 95% CI: 0.92-0.95, while Whites with vaginal delivery had a 38% decrease risk of IM relative to C-section, RR= 0.68, 95% CI: 0.67-0.69, p < 0.001. Infant mortality varied by race, with Black/AA disproportionally affected, which is explained in part by labor and delivery procedures, suggestive of reliable and equitable intrapartum assessment of Black/AA mothers during labor, as well as implicit bias marginalization in the healthcare system.

DNA Methylation of Candidate Genes (ACE II, IFN-γ, AGTR 1, CKG, ADD1, SCNN1B and TLR2) in Essential Hypertension: A Systematic Review and Quantitative Evidence Synthesis.

Physical, chemical, and social environments adversely affect the molecular process and results in cell signal transduction and the subsequent transcription factor dysregulation, leading to impaired gene expression and abnormal protein synthesis. Stressful environments such as social adversity, isolation, sustained social threats, physical inactivity, and highly methylated diets predispose individuals to molecular level alterations such as aberrant epigenomic modulations that affect homeostasis and hemodynamics. With cardiovascular disease as the leading cause of mortality in the US and blacks/African Americans being disproportionately affected by hypertension (HTN) which contributes substantially to these deaths, reflecting the excess mortality and survival disadvantage of this sub-population relative to whites, understanding the molecular events, including epigenomic and socio-epigenomic modulations, is relevant to narrowing the black-white mortality risk differences. We aimed to synthesize epigenomic findings in HTN namely (a) angiotensin-converting enzyme 2 (ACE II) gene, (b) Toll-like receptor 2 (TLR2) gene, (c) interferon γ (IFN-γ) gene, and (d) Capping Actin Protein, Gelosin-Like (CAPG) gene, adducin 1(ADD1) gene, (e) Tissue inhibitor of metalloproteinase 3 (TIMP3), (f) mesoderm specific transcript (MEST) loci, (g) sodium channel epithelial 1 alpha subunit 2 (SCNN1B), (h) glucokinase (CKG) gene (i) angiotensin II receptor, type1 (AGTR1), and DNA methylation (mDNA). A systematic review and quantitative evidence synthesis (QES) was conducted using Google Scholar and PubMed with relevant search terms. Data were extracted from studies on: (a) Epigenomic modulations in HTN based on ACE II (b) TLR2, (c) IFN-γ gene, (d) CAPG, ADD1, TIMP3, MEST loci, and mDNA. The random-effect meta-analysis method was used for a pooled estimate of the common effect size, while z statistic and I^2 were used for the homogeneity of the common effect size and between studies on heterogeneity respectively. Of the 642 studies identified, five examined hypermethylation while seven studies assessed hypomethylation in association with HTN. The hypermethylation of ACE II, SCNN1B, CKG, IFN-γ gene, and miR-510 promoter were associated with hypertension, the common effect size (CES) = 6.0%, 95% CI, -0.002-11.26. In addition, the hypomethylation of TLR2, IFN-γ gene, ADD1, AGTR1, and GCK correlated with hypertension, the CES = 2.3%, 95% CI, -2.51-7.07. The aberrant epigenomic modulation of ACE II, TLR2, IFN-γ, AGTR1, and GCK correlated with essential HTN. Transforming the environments resulting from these epigenomic lesions will facilitate early intervention mapping in reducing HTN in the US population, especially among socially disadvantaged individuals, particularly racial/ethnic minorities.

Implication of Spiritual Network Support System in Epigenomic Modulation and Health Trajectory.

With challenges in understanding the multifactorial etiologies of disease and individual treatment effect heterogeneities over the past four decades, much has been acquired on how physical, chemical and social environments affect human health, predisposing certain subpopulations to adverse health outcomes, especially the socio-environmentally disadvantaged (SED). Current translational data on gene and adverse environment interaction have revealed how adverse gene-environment interaction, termed aberrant epigenomic modulation, translates into impaired gene expression via messenger ribonucleic acid (mRNA) dysregulation, reflecting abnormal protein synthesis and hence dysfunctional cellular differentiation and maturation. The environmental influence on gene expression observed in most literature includes physical, chemical, physicochemical and recently social environment. However, data are limited on spiritual or religious environment network support systems, which reflect human psychosocial conditions and gene interaction. With this limited information, we aimed to examine the available data on spiritual activities characterized by prayers and meditation for a possible explanation of the nexus between the spiritual network support system (SNSS) as a component of psychosocial conditions, implicated in social signal transduction, and the gene expression correlate. With the intent to incorporate SNSS in human psychosocial conditions, we assessed the available data on bereavement, loss of spouse, loneliness, social isolation, low socio-economic status (SES), chronic stress, low social status, social adversity (SA) and early life stress (ELS), as surrogates for spiritual support network connectome. Adverse human psychosocial conditions have the tendency for impaired gene expression through an up-regulated conserved transcriptional response to adversity (CTRA) gene expression via social signal transduction, involving the sympathetic nervous system (SNS), beta-adrenergic receptors, the hypothalamus-pituitary-adrenal (HPA) axis and the glucocorticoid response. This review specifically explored CTRA gene expression and the nuclear receptor subfamily 3 group C member 1 (NR3C1) gene, a glucocorticoid receptor gene, in response to stress and the impaired negative feedback, given allostatic overload as a result of prolonged and sustained stress and social isolation as well as the implied social interaction associated with religiosity. While more remains to be investigated on psychosocial and immune cell response and gene expression, current data on human models do implicate appropriate gene expression via the CTRA and NR3C1 gene in the SNSS as observed in meditation, yoga and thai-chi, implicated in malignant neoplasm remission. However, prospective epigenomic studies in this context are required in the disease causal pathway, prognosis and survival, as well as cautious optimism in the application of these findings in clinical and public health settings, due to unmeasured and potential confoundings implicated in these correlations.

Aberrant Epigenomic Modulation of Glucocorticoid Receptor Gene (NR3C1) in Early Life Stress and Major Depressive Disorder Correlation: Systematic Review and Quantitative Evidence Synthesis.

Early life stress (ELS) induced by psychological trauma, child maltreatment, maternal separation, and domestic violence predisposes to psycho-behavioral pathologies during adulthood, namely major depressive disorder (MDD), anxiety, and bipolar affective disorder. While environmental data are available in illustrating this association, data remain to be established on the epigenomic underpinning of the nexus between ELS and MDD predisposition. Specifically, despite the observed aberrant epigenomic modulation of the NR3C1, a glucocorticoid receptor gene, in early social adversity and social threats in animal and human models, reliable scientific data for intervention mapping in reducing social adversity and improving human health is required. We sought to synthesize the findings of studies evaluating (a) epigenomic modulations, mainly DNA methylation resulting in MDD following ELS, (b) epigenomic modifications associated with ELS, and (c) epigenomic alterations associated with MDD. A systematic review and quantitative evidence synthesis (QES) were utilized with the random effect meta-analytic procedure. The search strategy involved both the PubMed and hand search of relevant references. Of the 1534 studies identified through electronic search, 592 studies were screened, 11 met the eligibility criteria for inclusion in the QES, and 5 examined ELS and MDD; 4 studies assessed epigenomic modulation and ELS, while 2 studies examined epigenomic modulations and MDD. The dense DNA methylation of the 1F exon of the NR3C1, implying the hypermethylated region of the glucocorticoid receptor gene, was observed in the nexus between ELS and MDD, common effect size (CES) = 14.96, 95%CI, 10.06-19.85. With respect to epigenomic modulation associated with child ELS, hypermethylation was observed, CES = 23.2%, 95%CI, 8.00-38.48. In addition, marginal epigenomic alteration was indicated in MDD, where hypermethylation was associated with increased risk of MDD, CES = 2.12%, 95%CI, -0.63-4.86. Substantial evidence supports the implication of NR3C1 and environmental interaction, mainly DNA methylation, in the predisposition to MDD following ELS. This QES further supports aberrant epigenomic modulation identified in ELS as well as major depressive episodes involving dysfunctional glucocorticoid-mediated negative feedback as a result of allostatic overload. These findings recommend prospective investigation of social adversity and its predisposition to the MDD epidemic via aberrant epigenomic modulation. Such data will facilitate early intervention mapping in reducing MDD in the United States population.

Risk factors for pancreatitis after posterior spinal fusion in children with cerebral palsy.

This study reports on the prevalence and risk factors of acute pancreatitis after posterior spinal fusion for cerebral palsy scoliosis. Pancreatitis diagnosis was based on elevated amylase or lipase above three times the upper normal limit. Perioperative data were compared between patients with and without pancreatitis. We included 300 patients; 55% developed acute pancreatitis. Gastrostomy dependence was more common in the pancreatitis group (P=0.048). Perioperative data were similar between groups. Patients with pancreatitis had longer duration of hospitalization (19 vs. 13 days, P<0.001). Acute pancreatitis is common after cerebral palsy scoliosis surgery. Gastrostomy dependence increases its risk. Although no mortality was reported, hospital stay was longer.

Hip Reconstruction in Children With Unilateral Cerebral Palsy and Hip Dysplasia.

BACKGROUND: Highly functioning children with unilateral cerebral palsy (CP) who have hip involvement (type IV hemiplegia) may present with hip dysplasia during their adolescence. The aim of this report is to assess the outcomes of combined femoral and acetabular reconstruction in this population. METHODS: This study is a retrospective review of all patients with unilateral CP, Gross Motor Function Classification System types I and II, who had hip reconstruction for unilateral dysplasia between 1989 and 2013. Clinical variables (pain and hip passive range of motion) were reviewed. Hip morphology was assessed radiographically according to Melbourne Cerebral Palsy Hip Classification System. Three-dimensional gait analyses were also reviewed to evaluate the effect of surgery on these patients' gaits. RESULTS: Twelve patients were included with a mean age at surgery of 14 years (range, 7 to 19 y) and follow-up mean of 4 years (range, 1 to 8 y). Nine hips were improved according to Melbourne Cerebral Palsy Hip Classification System. Migration percentage decreased significantly (P<0.001) from 45% (30% to 86%) to 15% (0% to 28%). Neck shaft angle decreased (P<0.001) from 144 degrees (range, 129 to 156 degrees) to 125 degrees (range, 114 to 139 degrees). Tonnis angle and Sharp angle also decreased significantly. All patients were pain free at the last visit. Overall level of gait function as measured by Gait Deviation Index and Gait Profile Score [78 (61 to 89) and 12 (8 to 16), respectively] for all patients was maintained without significant changes. CONCLUSIONS: In hemiplegic type IV CP, with high functional level (Gross Motor Function Classification System I and II), hip dysplasia is a rare occurrence during adolescent years. Combined hip reconstruction improves hip morphology, relieves pain, and maintains a high level of function. LEVEL OF EVIDENCE: Level IV-therapeutic study.

Factors predicting postoperative complications following spinal fusions in children with cerebral palsy scoliosis.

PURPOSE: The purpose of this study was to review the postoperative complications after posterior spinal fusion (PSF) in cerebral palsy (CP) scoliosis and identify the predictive preoperative risk factors. METHODS: All PSFs consecutively performed for CP scoliosis between 2004 and 2013 were reviewed. Preoperative risk score (ORS) and postoperative complications score (POCS) were used as measures of all recorded preoperative risk factors and postoperative complications, respectively. RESULTS: The review included 303 children with a mean age of 14.6 ± 3.0 years. Mean hospitalization was 16 days. Dependence on G-tube feeding was associated with higher POCS (P = 0.027). Postoperative fever, seizures, and septicemia were associated with higher ORS (P < 0.01). Specifically, postoperative pancreatitis and deep wound infections were more common in children with G-tube. CONCLUSION: This study suggests that G-tube dependence is a predictive risk factor of complications after PSF in CP scoliosis. Children with G-tube need special perioperative care. No other specific preoperative risk factor predicted postoperative complications.

Prophylactic methylprednisolone to reduce inflammation and improve outcomes from one lung ventilation in children: a randomized clinical trial.

BACKGROUND: One lung ventilation (OLV) results in inflammatory and mechanical injury, leading to intraoperative and postoperative complications in children. No interventions have been studied in children to minimize such injury. OBJECTIVE: We hypothesized that a single 2-mg·kg(-1) dose of methylprednisolone given 45-60 min prior to lung collapse would minimize injury from OLV and improve physiological stability. METHODS: Twenty-eight children scheduled to undergo OLV were randomly assigned to receive 2 mg·kg(-1) methylprednisolone (MP) or normal saline (placebo group) prior to OLV. Anesthetic management was standardized, and data were collected for physiological stability (bronchospasm, respiratory resistance, and compliance). Plasma was assayed for inflammatory markers related to lung injury at timed intervals related to administration of methylprednisolone. RESULTS: Three children in the placebo group experienced clinically significant intraoperative and postoperative respiratory complications. Respiratory resistance was lower (P = 0.04) in the methylprednisolone group. Pro-inflammatory cytokine IL-6 was lower (P = 0.01), and anti-inflammatory cytokine IL-10 was higher (P = 0.001) in the methylprednisolone group. Tryptase, measured before and after OLV, was lower (P = 0.03) in the methylprednisolone group while increased levels of tryptase were seen in placebo group after OLV (did not achieve significance). There were no side effects observed that could be attributed to methylprednisolone in this study. CONCLUSIONS: Methylprednisolone at 2 mg·kg(-1) given as a single dose prior to OLV provides physiological stability to children undergoing OLV. In addition, methylprednisolone results in lower pro-inflammatory markers and higher anti-inflammatory markers in the children's plasma.

Disruption of basal lamina components in neuromotor synapses of children with spastic quadriplegic cerebral palsy.

Cerebral palsy (CP) is a static encephalopathy occurring when a lesion to the developing brain results in disordered movement and posture. Patients present with sometimes overlapping spastic, athetoid/dyskinetic, and ataxic symptoms. Spastic CP, which is characterized by stiff muscles, weakness, and poor motor control, accounts for ∼80% of cases. The detailed mechanisms leading to disordered movement in spastic CP are not completely understood, but clinical experience and recent studies suggest involvement of peripheral motor synapses. For example, it is recognized that CP patients have altered sensitivities to drugs that target neuromuscular junctions (NMJs), and protein localization studies suggest that NMJ microanatomy is disrupted in CP. Since CP originates during maturation, we hypothesized that NMJ disruption in spastic CP is associated with retention of an immature neuromotor phenotype later in life. Scoliosis patients with spastic CP or idiopathic disease were enrolled in a prospective, partially-blinded study to evaluate NMJ organization and neuromotor maturation. The localization of synaptic acetylcholine esterase (AChE) relative to postsynaptic acetylcholine receptor (AChR), synaptic laminin ß2, and presynaptic vesicle protein 2 (SV2) appeared mismatched in the CP samples; whereas, no significant disruption was found between AChR and SV2. These data suggest that pre- and postsynaptic NMJ components in CP children were appropriately distributed even though AChE and laminin ß2 within the synaptic basal lamina appeared disrupted. Follow up electron microscopy indicated that NMJs from CP patients appeared generally mature and similar to controls with some differences present, including deeper postsynaptic folds and reduced presynaptic mitochondria. Analysis of maturational markers, including myosin, syntrophin, myogenin, and AChR subunit expression, and telomere lengths, all indicated similar levels of motor maturation in the two groups. Thus, NMJ disruption in CP was found to principally involve components of the synaptic basal lamina and subtle ultra-structural modifications but appeared unrelated to neuromotor maturational status.

Cerebral palsy patients discovered dead during sleep: experience from a comprehensive tertiary pediatric center.

OBJECTIVES: It is not uncommon for children with cerebral palsy (CP) to be discovered dead during sleep (DDDS); however, the factors associated with this pattern of mortality remain unknown. The current study aims to describe the mortality associated with children with CP from a single, tertiary care center who were DDDS. METHODS: A retrospective (case-only) design to examine proportionate mortality and patient characteristics and co-morbidities that may be related to children DDDS between 1993 and 2011. RESULTS: There were 177 patients with CP whose deaths were reported to our institution during the study period, of which 19 were DDDS at home. The period proportionate mortality (PPM) was 114.5 per 1000. The average age at time of death was 17 years and 6 months (minimum, 6 years; maximum, 25 years). All but one of the DDDS patients had gastrointestinal feeding tubes, seizure disorders, respiratory disorders, and were non-ambulatory. Very importantly, our DDDS patients manifested clusters of respiratory disorders, namely recurrent aspiration pneumonia (10/19), asthma pneumonitis (4/19), food/vomitius inhalation (6/19), reactive airway disease (16/19), respiratory failure (14/19), chronic bronchitis (7/19), chronic obstructive lung disease (9/19), and nocturnal respiratory insufficiency (16/19). CONCLUSIONS: Respiratory disorders, severe motor disability, seizures, and intellectual status are possible co-morbidities that may be associated with DDDS. There is a need for further study in order to understand what type of monitoring and care (if any) may help prevent DDDS related to these co-morbidities and sleep disorders/abnormalities.

Surgical Treatment of Scoliosis in Non-Ambulatory Spastic Quadriplegic Cerebral Palsy Patients: A Matched Cohort Comparison of Unit Rod Technique and All-Pedicle Screw Constructs.

STUDY DESIGN: Matched cohort. OBJECTIVE: To compare the unit rod instrumentation (UR) technique with all-pedicle screw (PS) constructs in the surgical care of scoliosis in Gross Motor Function Classification System IV/V non-ambulatory spastic quadriplegic cerebral palsy patients. SUMMARY OF BACKGROUND DATA: Over the past 20 years, there has been a transition from the UR technique to the use of pedicle screws and iliac screws in neuromuscular scoliosis. To date, no head-to-head comparative analysis has been reported between the UR technique and PS constructs for posterior segmental spinal instrumentation and fusion in cerebral palsy patients. METHODS: A matched cohort study was performed between 2 tertiary-care pediatric centers: 1 using UR technique and the other PS constructs. Minimum follow-up was 2 years postoperatively (PS 2.5 years, UR 4.6 years, not significant). Fourteen patients were matched from each center based on age (mean age: PS 15.4 years, UR 15.5 years), preoperative pelvic obliquity (mean: PS 33.8°, UR 29.1°) and major coronal Cobb angle (mean: PS 100.9°, UR 100.1°). RESULTS: There was posterior-only surgery in 14 of 14 PS and 11 of 14 UR surgeries. The final follow-up Cobb angle was lower in the PS group (13.5° vs. 34.3°, p < .05), with 86.5% correction in the PS group and 65.7% in the UR group. Final follow-up pelvic obliquity was similar (PS 8.5° vs. UR 3.3°; not significant). There were no major complications in the PS group. In the UR group, there was 1 deep infection and 1 reoperation for removal of a prominent sublaminar wire. CONCLUSIONS: This is the first study to directly compare UR with PS constructs using matched patient cohorts in this patient population. All-pedicle screw constructs had better correction of coronal Cobb angle, lower blood loss, and shorter hospital stays. There was no difference in the correction of pelvic obliquity, complications, or reoperations.

Wrist fusion in patients with severe quadriplegic cerebral palsy.

We report clinical and radiographic outcomes of wrist fusion achieved with pin or plate fixation in 14 patients with severe quadriplegic cerebral palsy (CP) (19 wrists). Average patient age at the time of surgery was 16.8 ± 1.7 years (14-20 years). Mean follow-up time for the 14 patients was 5.9 ± 3.1 years (range, 1-11 years). Indication for surgery was severe wrist deformity that interfered with hygienic care. Few complications occurred, and outcomes were satisfactory. Statistically significant mean difference was shown between the pre- and postoperative radiographic angles (37°, P = 0.001, and 24°, P = 0.04, for lateral and anteroposterior views, respectively). Caregivers reported that appearance was the most perceived rationale for surgery (63 %). Improved hygienic care was the primary perceived benefit. The majority (88 %) were satisfied with the results. We recommend wrist fusion to improve hygienic care, positioning, and appearance of the wrist, hand, and fingers in patients with severe quadriplegic CP.

Osteochondritis dissecans of the talar head: natural history and review of literature.

Osteochondritis dissecans (OCD) of the talar dome is relatively common, however, OCD of the talar head is extremely rare. We present two cases of OCDs of the talar head. This report describes the natural history of this rare condition and its evolution until skeletal maturity. The literature of OCD in this unusual location is reviewed.

Rectus femoris transfer improves stiff knee gait in children with spastic cerebral palsy.

BACKGROUND: Stiff knee gait is common among children with ambulatory cerebral palsy (CP). When surgery is indicated, rectus femoris transfer as a primary treatment enhances knee range of motion, reduces time to peak knee flexion, increases peak knee flexion, and reduces toe drag. QUESTIONS/PURPOSES: We determined whether (1) distal rectus femoris transfer improved knee range of motion, time to peak knee flexion, peak knee flexion, and toe drag in children with CP diagnosed with stiff knee gait; and (2) patients in some subgroups (eg, those with relatively high knee range of motion compared with those with low knee range of motion before rectus femoris transfer) had greater improvement in these parameters. METHODS: We retrospectively reviewed gait data from 56 patients (99 limbs) preoperatively, short-term, and long-term. Subgroup analyses were performed to determine whether patients with high knee range of motion relative to those with low or moderate knee range of motion improved differentially after rectus femoris transfer. The minimum followup was 7 years (mean ± SD, 10 ± 2 years; range, 7-13 years). RESULTS: The mean peak knee flexion increased from baseline to short-term and to long-term followup. Patients with low peak knee flexion had the greatest improvement of peak knee flexion after rectus femoris transfer relative to the moderate and high peak knee flexion subgroups. Similarly, the greatest improvement after rectus femoris transfer for knee range of motion occurred in the low knee range of motion subgroup relative to moderate and high subgroups. Rectus femoris transfer improved mean time to peak knee flexion at short-term and long-term followup compared with baseline. Likewise, there was a decrease in toe drag at short- and long-term after rectus femoris transfer. CONCLUSION: Distal rectus femoris transfer selectively improved peak knee flexion, toe drag, and reduced time to peak knee flexion in ambulatory children with CP with stiff knee gait. LEVEL OF EVIDENCE: Level IV, therapeutic study. See guidelines for authors for a complete description of levels of evidence.