Andrea F. Duncan

Comparative Clinical Outcomes of Thymectomy for Myasthenia Gravis Performed by Extended Transsternal and Minimally Invasive Approaches

BACKGROUND Both transsternal and video-assisted thoracoscopic surgery (VATS) approaches are used for thymectomy in myasthenia gravis. We compared outcomes of simultaneous experiences in two institutions: one utilizing the transsternal approach exclusively, the other using VATS procedures for all patients. The Myasthenia Gravis Foundation of America guidelines were used to standardize reporting. METHODS Between March 1992 and September 2006, 95 thymectomies were performed for myasthenia gravis; 48 by VATS and 47 by transsternal approach. Preoperative classification and postoperative disease status were compared between the groups. RESULTS Mean age was 39.8 +/- 14.9 (VATS) versus 34.4 +/- 13.2 years (transsternal) (p = 0.07); the proportion of females was 52% versus 67% (p = 0.15); and preoperative duration of myasthenia gravis was 27 +/- 44 versus 20 +/- 45 months (p = 0.43), respectively. Clinical follow up was 89.5% complete at a mean of 6.0 +/- 4.0 years and 4.3 +/- 2.9 years (p = 0.03). The operative time was 128 +/- 34 minutes (VATS) versus 119 +/- 27 minutes (transsternal) (p = 0.22). The need for postoperative ventilation was 4.2% versus 16.2% (p = 0.07) and mean length of stay was 1.9 +/- 2.6 versus 4.6 +/- 4.2 days (p < 0.001). Thymomas were found in 8.3% of VATS versus 13.3% of transsternal patients (p = 0.44). No myasthenia gravis related deaths occurred and 95.8% of the VATS and 97.9% of the transsternal patients were in either complete stable remission, pharmacologic remission, or minimal manifestations status. In the VATS group, 13 of 17 (76.5%) patients stopped prednisone usage after surgery versus 5 of 14 (35.7%) in the transsternal group (p = 0.022). CONCLUSIONS Thymectomy is an effective treatment in patients with myasthenia gravis with equivalent clinical outcomes obtained by either approach.

Comparison of the Bayley II Mental Developmental Index and the Bayley III Cognitive Scale: Are we measuring the same thing?

Aim:  To better understand differences between Bayley Scales 3rd edition (Bayley III) Cognitive Scale and Bayley Scales 2nd edition (Bayley II) Mental Developmental Index (MDI) in 18–22‐month‐old children born term and preterm and to create a conversion algorithm using Bayley II MDI to calculate Bayley III Cognitive score.

Survival and neurodevelopmental outcomes among periviable infants

BACKGROUND Data reported during the past 5 years indicate that rates of survival have increased among infants born at the borderline of viability, but less is known about how increased rates of survival among these infants relate to early childhood neurodevelopmental outcomes. METHODS We compared survival and neurodevelopmental outcomes among infants born at 22 to 24 weeks of gestation, as assessed at 18 to 22 months of corrected age, across three consecutive birth‐year epochs (2000–2003 [epoch 1], 2004–2007 [epoch 2], and 2008–2011 [epoch 3]). The infants were born at 11 centers that participated in the National Institute of Child Health and Human Development Neonatal Research Network. The primary outcome measure was a three‐level outcome — survival without neurodevelopmental impairment, survival with neurodevelopmental impairment, or death. After accounting for differences in infant characteristics, including birth center, we used multinomial generalized logit models to compare the relative risk of survival without neurodevelopmental impairment, survival with neurodevelopmental impairment, and death. RESULTS Data on the primary outcome were available for 4274 of 4458 infants (96%) born at the 11 centers. The percentage of infants who survived increased from 30% (424 of 1391 infants) in epoch 1 to 36% (487 of 1348 infants) in epoch 3 (P<0.001). The percentage of infants who survived without neurodevelopmental impairment increased from 16% (217 of 1391) in epoch 1 to 20% (276 of 1348) in epoch 3 (P=0.001), whereas the percentage of infants who survived with neurodevelopmental impairment did not change significantly (15% [207 of 1391] in epoch 1 and 16% [211 of 1348] in epoch 3, P=0.29). After adjustment for changes in the baseline characteristics of the infants over time, both the rate of survival with neurodevelopmental impairment (as compared with death) and the rate of survival without neurodevelopmental impairment (as compared with death) increased over time (adjusted relative risks, 1.27 [95% confidence interval {CI}, 1.01 to 1.59] and 1.59 [95% CI, 1.28 to 1.99], respectively). CONCLUSIONS The rate of survival without neurodevelopmental impairment increased between 2000 and 2011 in this large cohort of periviable infants. (Funded by the National Institutes of Health and others; ClinicalTrials.gov numbers, NCT00063063 and NCT00009633.)

Effect of primary language on developmental testing in children born extremely preterm

The aim of this study was to better understand the impact of non‐English language spoken in the home on measures of cognition, language and behaviour in toddlers born extremely preterm.

Do currently recommended Bayley-III cutoffs overestimate motor impairment in infants born <27 weeks gestation?

Objective:To determine whether a Bayley-III motor composite score of 85 may overestimate moderate–severe motor impairment by analyzing Bayley-III motor components and developing cut-point scores for each.Study Design:Retrospective study of 1183 children born <27 weeks gestation at NICHD Neonatal Research Network centers and evaluated at 18–22 months corrected age. Gross Motor Function Classification System determined gross motor impairment. Statistical analyses included linear and logistic regression and sensitivity/specificity.Results:Bayley-III motor composite scores were strong indicators of gross/fine motor impairment. A motor composite cut-point of 73 markedly improved the specificity for identifying gross and/or fine motor impairment (94% compared with a specificity of 76% for the proposed new cut-point of 85). A Fine Motor Scaled Score <3 differentiated mild from moderate–severe fine motor impairment.Conclusions:This study indicates that a Bayley-III motor composite score of 85 may overestimate impairment. Further studies are needed employing term controls and longer follow-up.

Regional Cerebral Blood Flow in Children From 3 to 5 Months of Age

BACKGROUND AND PURPOSE: Understanding the relationship between brain and behavior in early childhood requires a probe of functional brain development. We report the first large study of regional CBF by use of arterial spin-labeling in young children. MATERIALS AND METHODS: Cerebral blood flow by use of arterial spin-labeling was measured in 61 healthy children between the ages of 3 and 5 months. Blood flow maps were parcellated into 8 broadly defined anatomic regions of each cerebral hemisphere. RESULTS: There was no sex effect; however, group analysis demonstrated significantly greater CBF in the sensorimotor and occipital regions compared with dorsolateral prefrontal, subgenual, and orbitofrontal areas (P < .0001). A significant age effect was also identified, with the largest increase in blood flow between 3 and 5 months occurring in the following regions: orbitofrontal (P < .009), subgenual (P < .002), and inferior occipital lobe (P = .001). CONCLUSIONS: These results are consistent with prior histologic studies demonstrating regional variation in brain maturation and suggest that arterial spin-labeling is sensitive to regional as well as age-related differences in CBF in young children.

Early working memory as a racially and ethnically neutral measure of outcome in extremely preterm children at 18–22 months

BACKGROUND Difficulties with executive function have been found in preterm children, resulting in difficulties with learning and school performance. AIM This study evaluated the relationship of early working memory as measured by object permanence items to the cognitive and language scores on the Bayley Scales-III in a cohort of children born extremely preterm. STUDY DESIGN Logistic regression models were conducted to compare object permanence scores derived from the Bayley Scales-III by race/ethnicity and maternal education, controlling for medical covariates. SUBJECTS Extremely preterm toddlers (526), who were part of a Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Networks multi-center study, were evaluated at 18-22 months corrected age. OUTCOME MEASURES Object permanence scores derived from the Bayley Developmental Scales were compared by race/ethnicity and maternal education, controlling for medical covariates. RESULTS There were no significant differences in object permanence mastery and scores among the treatment groups after controlling for medical and social variables, including maternal education and race/ethnicity. Males and children with intraventricular hemorrhage, retinopathy of prematurity, and bronchopulmonary dysplasia were less likely to demonstrate object permanence mastery and had lower object permanence scores. Children who attained object permanence mastery had significantly higher Bayley Scales-III cognitive and language scores after controlling for medical and socio-economic factors. CONCLUSIONS Our measure of object permanence is free of influence from race, ethnic and socio-economic factors. Adding this simple task to current clinical practice could help detect early executive function difficulties in young children.

Newborn intensive care survivors: a review and a plan for collaboration in Texas

BackgroundNeonatal intensive care is a remarkable success story with dramatic improvements in survival rates for preterm newborns. Significant efforts and resources are invested to improve mortality and morbidity but much remains to be learned about the short and long-term effects of neonatal intensive care unit (NICU) interventions. Published guidelines recommend that infants discharged from the NICU be in an organized follow-up program that tracks medical and neurodevelopmental outcomes. Yet, there are no standardized guidelines for provision of follow-up services for high-risk infants.The National Institute of Child Health and Human Development Neonatal Research Network and the Vermont Oxford Network have made strides toward standardizing practices and conducting outcomes research, but only include a subset of developmental follow-up programs with a focus on extremely preterm infants. Several studies have been conducted to gain a better understanding of current practices in developmental follow-up. Some of the major themes in these studies are the lack of personnel and funding to provide comprehensive follow-up care; feeding difficulties as a primary issue for NICU survivors, families, and programs; wide variability in referral and follow-up care practices; and calls for standardized, systematic developmental surveillance to improve outcomes.FindingsWe convened a one-day summit to discuss developmental follow-up practices in Texas involving four academic and three nonacademic centers. All seven centers described variable age and weight criteria for follow-up of NICU patients and a unique set of developmental practices, including duration of follow-up, types and timing of developmental assessments administered, education and communication with families and other health care providers, and referrals for services. Needs identified by the centers focused on two main themes: resources and comprehensive care. Participants identified key challenges for developmental follow-up, generated recommendations to address these challenges, and outlined components of a quality program.ConclusionsThe long-term goal is to ensure that all children maximize their potential; a goal supported through quality, comprehensive developmental follow-up care and outcomes research to continuously improve evidence-based practices. We aim to contribute to this goal through a statewide working group collaborating on research to standardize practices and inform policies that truly benefit children and their families.

Neurodevelopmental outcomes in postnatal growth-restricted preterm infants with postnatal head-sparing

Objective:To compare neurodevelopmental outcomes in postnatal growth-restricted infants born <29 weeks with and without postnatal head-sparing (PHS).Study Design:We analyzed developmental outcomes at 2 years of age among postnatally growth-restricted infants with and without head-sparing. The primary outcome was Bayley III cognitive composite score; secondary outcomes included Bayley III motor composite score, moderate/severe cerebral palsy, gross motor functional classification scale level⩾2, and presence or absence of neurodevelopmental impairment (NDI).Results:Of 1098 infants evaluated at 18 to 22 months, 658 were postnatally growth restricted, of whom 301 had head-sparing. In the multivariate model including independent risk factors for poor growth and poor developmental outcome, infants with head-sparing had higher adjusted motor composite scores (mean difference 4.65, P<0.01), but no differences in other neurodevelopmental outcomes.Conclusion:PHS is associated with improved neurodevelopmental outcome in extremely preterm infants, specifically Bayley III motor scores, but whether beneficial effects of PHS persist later in life is unknown.

Behavioral problems and socioemotional competence at 18 to 22 months of extremely premature children

Using a large sample of EP toddlers, this study reports increased rates of behavioral and socioemotional problems and associated risk factors. BACKGROUND: Behavior and socioemotional development are crucial aspects of child development . METHODS: A total of 2505 children born at <27 weeks’ gestation was evaluated at 18 to 22 months’ corrected age between January 1, 2008 and December 12, 2012 (86% follow-up). The Brief Infant and Toddler Social and Emotional Assessment was used to evaluate behavioral and socioemotional problems. Cognition and language were evaluated by using the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III). Logistic regression analysis was used to evaluate for perinatal and demographic factors associated with behavioral problems (≥75th percentile) and delayed socioemotional competence (≤15th percentile). Structural equation modeling with bootstrapping was used to identify possible associated risk factors and Bayley-III scores as mediators. RESULTS: Thirty-five percent (873) of children had behavioral problems, and 26% (637) displayed deficits in socioemotional competence. Male sex, public insurance, mothers with less than a high school education, and lower maternal age were associated with behavioral problems. Deficits in competence were associated with lower birth weight, public insurance, mothers with less than a high school education, and abnormal neuromotor exam. Bayley-III language and cognitive scores were significant mediators of the relationships between risk factors and both behavioral and competence scores (P < .05). CONCLUSIONS: Extremely premature children are at risk for behavioral problems and deficits in socioemotional competence. Sociodemographic factors were associated with both socioemotional competence and behavioral problems. Deficits in socioemotional competence were also associated with neuromotor abnormalities and cognitive and language function