Bayley Scales Of Infant Development, Second Edition Research Articles

Early cognitive development in children with infantile Pompe disease

This report describes the cognitive development of 17 children with infantile Pompe disease who participated in a 52-week clinical trial of enzyme replacement therapy (ERT) via biweekly infusion of Myozyme® (alglucosidase alfa). Subjects were six months of age or younger (adjusted for gestational age) upon initiation of ERT. The Mental Scale of the Bayley Scales of Infant Development-Second Edition (BSID-II) was administered to obtain a Mental Development Index (MDI) at baseline and weeks 12, 26, 38, and 52 of ERT to assess cognitive development in this treated cohort. Data regarding motor development were also obtained at the same visits and these were used to determine correlations between cognitive and motor development. Over the course of the study, two subgroups of subjects emerged: high responders who were sitting independently and/or ambulating by week 52 (n=13) and limited responders who showed minimal motor gains throughout the first year of ERT (n=4). In the high responder group, MDI scores on the BSID-II remained stable throughout the study and were within normal limits. Positive correlations between cognitive and motor development were also present. These data suggest that the cognitive function of infants up to 18months of age with Pompe disease is unaffected by the possible presence of glycogen in the central nervous system. Continued investigation of the cognitive development of older survivors is warranted.

Adaptive assessment of young children with visual impairment

The aim of this study was to assess the effect of adaptations for children with low vision of the Bayley Scales, a standardized developmental instrument widely used to assess development in young children. Low vision adaptations were made to the procedures, item instructions and play material of the Dutch version of the Bayley Scales of Infant Development — Second Edition (BSID-II), and the Original and Low Vision versions were administered to children with visual impairment over an interval of two weeks. Although quantitative analysis revealed no significant differences between the Original and Low Vision versions of the test in children’s scores, feedback from test administrators indicates that the Low Vision materials make the test easier to administer and more engaging for children.

Differentiating Developmental Outcome between Infants with Severe Disability in Research Studies: The Role of Bayley Developmental Quotients

To investigate whether infants with a score <50 on the Bayley Scales of Infant Development, Second Edition (BSID-II) demonstrated differences in functional ability, and to assess whether the Bayley Developmental Quotients (DQs) indicated such differences. Preterm infants (n = 67; 47 boys) with posthemorrhagic ventricular dilatation were evaluated at 25 months past term age using the BSID-II and grading of functional ability. Mental and Motor Bayley DQs were derived and compared with functional ability. Among the 34 subjects (51%) with a BSID-II score <50, there were clinically significant differences in the ability to walk, sit, eat, speak, and see. In all subjects, there were significant differences in Mental and Motor Bayley DQs based on grade of disability in each domain except hearing. Bayley DQ quantified the spread of functional ability in all children, provided a continuous parameter to compare ability in severely delayed children, and should be considered in future therapeutic trials of infants with brain injury.

The Modified Checklist for Autism in Toddlers in extremely low gestational age newborns: individual items associated with motor, cognitive, vision and hearing limitations

The Modified Checklist for Autism in Toddlers (M-CHAT) has yielded elevated rates of screening failure for children born preterm or with low birthweight. We extended these findings with a detailed examination of M-CHAT items in a large sample of children born at extremely low gestational age. The sample was grouped according to children's current limitations and degree of impairment. The aim was to better understand how disabilities might influence M-CHAT scores. Fourteen participating institutions of the Extremely Low Gestational Age Newborns (ELGAN) Study prospectively collected information about 1086 infants who were born before the 28th week of gestation and had an assessment at age 24-months. The 24-month visit included a neurological assessment, the Bayley Scales of Infant Development, Second edition (BSID-II), M-CHAT and a medical history form. Outcome measures included the distribution of failed M-CHAT items among groups classified according to cerebral palsy diagnosis, gross motor function, BSID-II scores and vision or hearing impairments. M-CHAT items were failed more frequently by children with concurrently identified impairments (motor, cognitive, vision and hearing). In addition, the frequency of item failure increased with the severity of impairment. The failed M-CHAT items were often, but not consistently, related to children's specific impairments. Importantly, four of the six M-CHAT 'critical items' were commonly affected by presence and severity of concurrent impairments. The strong association between impaired sensory or motor function and M-CHAT results among extremely low gestational age children suggests that such impairments might give rise to false positive M-CHAT screening.

Executive function deficit in preschool children born very low birth weight with normal early development

Background Many studies showed that children born very low birth weight (VLBW) are at high risk of executive function (EF) deficit, including impulse control, working memory and cognitive flexibility. However, they did not exclude the influence of abnormal early development on EF deficit. Aims The aim was to investigate if six-year-old VLBW children with normal early development still have EF deficit. Methods The research was conducted in two groups. The VLBW group included 37 children at aged 6, with more than 70 of Bayley Scales of Infant Development-Second Edition (BSIDII) before aged 2. The normal group included 22 term children aged 6 who were born healthy and developed normally, with comparable IQ and social economic status. Five instruments, including Comprehensive Nonverbal Attention Test Battery (CNAT), Tower of London (ToL), Wisconsin Card Sorting Test (WCST), Knox's Cube Test and Digit Span Subtest of WISC-IV, were analyzed to evaluate four kinds of EF, including impulse control, planning, cognitive flexibility and working memory. Results The EF of VLBW group was significantly lower in independent t-test on the scores of planning in ToL, cognitive flexibility in WCST and nonverbal working memory in Knox's Cube Test. Yet, the inferiority in EF of VLBW group became less significant when ANCOVA analysis was used to adjust gestation age and birth weight. Conclusions Six-year-old VLBW children even with normal early development are still at risk of deficits in “planning”, “cognitive flexibility” and “nonverbal working memory” while the preterm factors, both gestation age and birth weight, were important covariant factors.

Effects of Traditional Swaddling on Development: A Randomized Controlled Trial

Evidence of the effects of tight, prolonged binding of infants on development is inconclusive and based on small ethnographic studies. The null hypothesis was that Mongolian infants not swaddled or swaddled tightly in a traditional setting (to >7 months of age) do not have significantly different scores for the Bayley Scales of Infant Development, Second Edition (BSID-II). In a randomized controlled trial, 1279 healthy newborns in Ulaanbaatar, Mongolia, were allocated at birth to traditional swaddling or nonswaddling. The families received 7 months of home visits to collect data and monitor compliance. At 11 to 17 months of age, the BSID-II was administered to 1100 children. No significant between-group differences were found in mean scaled mental and psychomotor developmental scores. The unadjusted mean difference between the groups was -0.69 (95% confidence interval [CI]: -2.59 to 1.19) for psychomotor and -0.42 (95% CI: -1.68 to 0.84) for mental scores in favor of the swaddling group. A subgroup analysis of the compliant sample produced similar results. BSID-II-scaled psychomotor and mental scores were 99.98 (95% CI: 99.03-100.92) and 105.52 (95% CI: 104.89-106.14), respectively. Background characteristics were balanced across the groups. In the Mongolian context, prolonged swaddling in the first year of life did not have any significant impact on children's early mental or psychomotor development. Additional studies in other settings need to confirm this finding. The Mongolian infants in this trial had scaled BSID-II mental and psychomotor scores comparable to United States norms.

Prenatal PBDEs and Neurodevelopment: Herbstman et al. Respond to Goodman et al. and to Banasik and Strosznajder

Prenatal PBDEs and Neurodevelopment: Herbstman et al. Respond to Goodman et al. and to Banasik and Strosznajder

Prenatal PBDEs and Neurodevelopment: Accuracy of Assessment

Herbstman et al. (2010) measured eight polybrominated diphenyl ethers (PBDEs) in cord blood and reported that children of mothers with higher cord blood concentrations of PBDEs 47, 99, and 100 scored lower on mental and physical development tests at 12, 24, 36, and 72 months of age. Here, we raise several issues that limit the conclusions that may be drawn from their study. In the study by Herbstman et al. (2010), only 210 cord blood specimens from 329 mothers were available, and assessments were conducted for only 96–118 children at each age. Several congeners were measured in the study; overall, the percentage of individual congeners below the limit of detection (LOD) ranged from 18.6% to 96.1%. For congeners on which major assessments were conducted, the range of values < LOD was 18.6–50.2%. Herbstman et al. (2010) did not state how many samples were < LOD for each assessment, so it is possible that the percentage was even higher and may have led to a large impact on the results, particularly given the small sample size for each assessment. Herbstman et al. (2010) measured PBDEs in cord blood and maternal blood only once, but individual levels most likely changed over the course of the pregnancy and over the period when developmental assessments were conducted. The median values were relatively low, and there was no reliable indication of interindividual variability, so even small changes over time could have altered any observed exposure–response associations. In addition, the authors did not indicate how assessments of individual children changed over time. If different children contributed to associations at different ages, the reported findings may not be indicative of a causal role for PBDEs. Notably, most associations were found at earlier ages, suggesting that even if PBDEs were causal, effects were reversible. Herbstman et al. (2010) reported that all PBDEs were correlated with one another, as were repeated developmental scores. Yet associations varied both among PBDEs for the same tests and among repeated tests for the same PBDEs. If PBDEs are truly causal and acting via similar mechanisms of action, results should be repeatable across PBDEs and repeated tests. This is not the case, thus suggesting that chance or some other factor (e.g., alcohol, caffeine, poor diet, methylmercury, polychlorinated biphenyls) is a more likely explanation. In addition, all of the mothers in the study were pregnant and lived near the World Trade Center (WTC) on 11 September 2001. Given this proximity, there is no way of knowing whether other unmeasured exposures or other factors (e.g., psychological, behavioral) contributed to neurological effects. Herbstman et al. (2010) used the Bayley Scales of Infant Development, Second Edition (BSID-II) to measure developmental impairment, which is based on a mean ± SD of 100 ± 15 to define normal development (Bayley 1993). This means that in 68% of the standard population, scores ranged from 85 to 115. The authors reported that through univariate analysis, the change in the BSID-II score from the 25th percentile of PBDE level to the 75th percentile was −5.57. This degree of change is well within the SD of the test, which makes it impossible to determine whether the relationship observed was due to PBDEs or simply the interindividual variability inherent to the test. Because the authors did not assess the association between PBDE levels and those scoring outside the SD of the test (compared with those scoring within), it is impossible to determine whether a clinically significant association between PBDE cord blood levels and developmental impairment exists. Changes in IQ scores are not very meaningful unless they are put directly into context with the scoring ranges in the test design; Herbstman et al. did not provide much information as to the scores that were actually produced, though they implied that those from mothers with higher PBDE levels were somehow impaired when they may well have been normal. Taken together, these factors prevent an accurate assessment of whether prenatal exposure to PBDEs is associated with adverse neurodevelopmental effects.

Adjunctive levetiracetam in patients aged 1 month to

Background: In a recent double-blind, placebo-controlled study, adjunctive levetiracetam (LEV) was reported to be effective and well tolerated during 5-day treatment in patients aged 1 month to <4 years with partial-onset seizures. A study was planned to fulfill the regulatory requirement to evaluate the long-term safety of LEV as adjunctive therapy for partial-onset seizures in pediatric patients. Objective: This study evaluated the long-term effectiveness and tolerability of adjunctive LEV in infants and young children with partial-onset seizures. Methods: This was a prospective, open-label, outpatient, multicenter study (N01148; ClinicalTrials.gov identifier NCT00152516) conducted as an extension of a previously published study (N01009; NCT00175890). Patients were enrolled from 3 sources, as follows: (1) patients who had completed study N01009; (2) patients who had failed screening for entry into study N01009 but fulfilled the eligibility criteria for entry into this study; and (3) patients who were directly enrolled. The study consisted of a 2- to 4-week retrospective baseline period (and a 3- to 10-day prospective baseline period for directly enrolled patients), a 2- to 8-week uptitration/conversion period, and a maintenance period. Eligible patients were required to have epilepsy with partial-onset seizures, treated with a stable regimen of 1 or 2 antiepileptic drugs. Patients received adjunctive LEV, 20 to 80 mg/kg/d, for up to 48 weeks (total study duration). The primary variable for effectiveness was the percentage reduction from baseline in the weekly frequency of partial-onset seizures, as recorded in patients' diaries. Data for effectiveness were also analyzed by age strata (1 month to <1 year, 1 to <2 years, and 2 to <4 years). Neuropsychological assessment was conducted with the Bayley Scales of Infant Development, Second Edition (BSID-II). All analyses were performed on observed data, and the last-observation-carried-forward approach was not used. The intent-to-treat (ITT) population was defined as all patients who took at least one dose of LEV during the study. Treatment-emergent adverse events (TEAEs) were assessed by observation, spontaneous reporting, standard questions, review of diary cards, and neuropsychologists' clinical reports. Additional measures included physical and neurologic examinations, vital signs, ECGs, routine blood chemistry, and routine hematology assessments. Results: The study included 152 patients in the ITT population. In total, 51.3% (78/152) of the patients were male, and mean (SD) age was 23.5 (12.4) months. The mean LEV maintenance dose was 56.1 (16.2) mg/kg/d, and the median (Q1–Q3) treatment duration was 287.8 (209.0–295.5) days. Ninety-seven patients (63.8%) completed the study. The BSID-II subpopulation included 51 patients. During maintenance, the overall median (Q1–Q3) percentage reduction from baseline in the weekly frequency of partial-onset seizures was 56.0% (−10.9% to 92.8%), which was sustained over time and appeared comparable across the age strata (1 month to <1 year, n = 25, 50.9%; 1 to <2 years, n = 48, 58.0%; and 2 to <4 years, n = 59, 55.0%). The overall responder rate (ie, ≥50% reduction from baseline in weekly partial-onset seizures) was 53.8% (71/132), was maintained over time, and was consistent across the age strata (1 month to <1 year, 52.0%; 1 to <2 years, 56.3%; and 2 to <4 years, 52.5%). Mean BSID-II raw scores for psychomotor development and behavioral functioning remained static, whereas mental development appeared to improve over time, although this was not tested statistically. At least one TEAE was reported in 143 patients (94.1%). The most frequently reported TEAEs were pyrexia (60/152; 39.5%), upper respiratory tract infection (42/152; 27.6%), and vomiting (28/152; 18.4%). The most common TEAEs affecting the central nervous system were convulsion (25/152; 16.4%), irritability (19/152; 12.5%), and somnolence (16/152; 10.5%). Most TEAEs (77.0%) were mild or moderate in intensity. Conclusion: Adjunctive LEV treatment for up to 48 weeks was associated with effective and sustained seizure control and had an acceptable tolerability profile in this small, selected population of infants and young children aged 1 month to <4 years with partial-onset seizures.

Neurodevelopment in 1‐year‐old Japanese infants after congenital heart surgery

The purpose of this study was to determine the prevalence of persistent neurodevelopmental sequelae in 1-year-old infants after open heart surgery for congenital heart disease, using the Bayley Scales of Infant Development second edition (BSID-II). A secondary objective was to confirm the applicability and usefulness of the BSID-II in Japanese infants. Thirty-nine infants who underwent repair of a ventricular septal defect before 6 months of age and 108 normal Japanese infants at 1 year of age were assessed using the BSID-II. In normal infants, scores on the Mental Development Index and the Psychomotor Development Index components of the BSID-II ranged from borderline retardation to very superior following a normal distribution similar to those obtained for US controls. No problems were encountered, either in translation or in following the instructions when the BSID-II was used to evaluate the 1-year-old Japanese infants. On the other hand, the mean scores on the Mental Development Index and the Psychomotor Development Index were significantly lower in Japanese patients than in normal Japanese infants, particularly for gross motor development (P < 0.001). Linear regression analysis showed that a longer intensive care unit stay was associated with impaired cognitive development at 1 year of age (P= 0.03). Neurodevelopmental functions in 1-year-old infants with congenital heart disease were lower than those in normal infants, especially for gross motor function using the BSID-II. One risk factor that correlated with neurodevelopmental sequelae was the length of intensive care unit stay.

Flash visual evoked potentials at 2-year-old infants with different birth weights

Increased prevalence of visual impairments has been reported in preterm populations. However, it remains unclear about the long-term visual electrophysiological outcomes and their association with visual cognitive functions in premature infants. We investigated visual electrophysiological outcome of 2-year-old infants of different birth weights by flash visual evoked potentials (FVEPs) in order to explore the correlation between visual cognitive functions and FVEPs and to assess the application of FVEPs in evaluating the visual capability of an infant. The FVEPs of 77 infants, including 25 very low birth weight (VLBW) premature infants, 16 low birth weight (LBW) premature infants and 36 full-term infants, were tested with a visual electrophysiological testing device. Neuromotor development was assessed with the Bayley Scales of Infant Development, Second Edition (BSID-II). The visual cognitive functions were evaluated by scoring the proportion passed of 12 items chosen from the BSID-II for infants at 23 to 25 months of age. The second prominent positive wave (P2) was the major component presented in all three groups. The mean latency of P2 in the VLBW, LBW and full-term groups was 149.65+/-23.79 ms, 129.39+/-8.70 ms, and 126.14+/-7.73 ms respectively. There was no significant difference in mean latency of P2 wave between the LBW and full-term groups; the mean latency of the P2 wave in the VLBW group was delayed more significantly than those of the other two groups. The difference in amplitude of the P2 peak to the preceding N2 peak (N2P2) between the three groups was not statistically significant. The latency of the P2 main wave was negatively correlated with mental developmental index (MDI) (r'(MDI) = -0.35) and visual cognitive capability (r'(visual capability) = -0.21). The latency of the P2 main wave on FVEPs was delayed more significantly in premature infants than in full-term infants at 2 years of corrected age. The visual functional development was delayed in premature infants, especially in VLBW infants (gestational age <32 weeks). The FVEPs were reported low but there were statistically significant correlations between measures of visual cognition and P2 peak latency. As a noninvasive and convenient method, FVEPs are useful in assessing certain aspects of an infant's visual development and visual function.

POST-OPERATIVE NEURODEVELOPMENTAL FINDINGS IN SYNDROMIC AND NON-SYNDROMIC CRANIOSYNOSTOSIS

Objective To evaluate the developmental situation of children that undergo operation because of syndromic and non-syndromic craniosynostosis. Materials & Methods In this prospective study, 24 children (4 to 16 months of age) who underwent neurosurgeryical intervention because of non-syndromic (79%) and syndromic (21%) craniosynostosis were recruited. For psychological evaluation, the Bayley Scales of Infant Development-Second Edition (BSID-II) was applied one month before and one year after surgical correction. The scale consists of three parts, Mental Developmental Index (MDI), Psychomotor Developmental Index (PDI), and Behavior Rating Scale. The MDI and PDI yield age-standard scores (100 ± SD). Results Mean baseline BSID-II scores revealed a mild delay in mental and motor scores (MDI: 84.3±2.1; PDI: 80.5±4.2) in non-syndromic craniosynostosis and a moderate delay in mental and motor scores (MDI: 70.3±3.1; PDI: 64.3±1.7) in syndromic craniosynostosis. Mean postoperative BSID-II score revealed improved motor scores (MDI: 91.3±0.1; PDI: 91.3±0.2) in non-syndromic craniosynostosis and mean postoperative BSID-II score revealed a mild delay in mental scores and no change in PDI. Among children with syndromic craniosynostosis, mean BSID-II score indicated mild baseline deficits in both mental and motor scores post operatively. Conclusion Mental development and prognosis was better in non-syndromic craniosynostosis comparing syndromic craniosynostosis. Surgery is effective in neurodevelopmental growth in non syndromic types of craniosynostosis but in syndromic type, remodeling surgery does not significantly affect neurodevelopmental outcome. Keywords : Craniosynostosis, BSID-II, Neurodevelopmental

Validation of the Functional Status II questionnaire in the assessment of extremely‐low‐birthweight infants

The increased survival of infants born at extremely low birthweight (ELBW) has been associated with significant morbidity, including higher rates of neurodevelopmental disability. However, formalized testing to evaluate these problems is both time-consuming and costly. The revised Functional Status questionnaire (FS-II) was designed to assess caregivers' perceptions of the functional status of children with chronic diseases. We evaluated the reliability and validity of the FS-II for ELBWinfants at 18 to 22 months corrected age using data from the US Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) Neonatal Research Network (NRN). Exploratory factor analyses were conducted using data from the network's first follow-up study of 1080 children born in 1993 to 1994 (508 males, 572 females [53%]), and results were confirmed using data from the next network follow-up of 4022 children born in 1995 to 2000 (1864 males, 2158 females [54%]). Results suggest that a two-factor solution comprising measures of general health and independence is most appropriate for ELBW infants. These factors differed from those found among chronically ill children, and new, more appropriate scales are presented for screening ELBWsurvivors. Both scales demonstrated good internal consistency: Cronbach's a=0.87 for general health and a=0.75 for independence. Construct validity of the scales was assessed by comparing mean scores on the scales according to scores on the Bayley Scales of Infant Development, second edition (BSID-II), and medical conditions. As hypothesized, infants with greater functional impairments according to their BSID-II scores or medical conditions had lower scores on the general health and independence scales, supporting the validity of the scales.

Impact of Neonatal Thyroid Hormone Insufficiency and Medical Morbidity on Infant Neurodevelopment and Attention Following Preterm Birth

Infants born preterm are at risk of both transiently reduced thyroid hormone levels and impaired neurocognitive development, including attention deficits. The objective of this study was to examine the effects of reduced thyroid hormone levels on general neurodevelopment and attention at 3 months corrected age. Sixty-four infants born 24 to 35 weeks gestation were stratified into four gestational age groups: Group A, 23-26 weeks (n = 10); Group B, 27-29 weeks (n = 23); Group C, 30-32 weeks (n = 20); Group D, 33-35 weeks (n = 11). Controls were 33 healthy infants born full-term (Group E). In preterm only, free thyroxine (FT(4)), triiodothyronine (T(3)), and thyrotropin (TSH) were measured at 2 and 4 weeks of life and at 40 weeks postconceptional age. At 3 months corrected age, all infants were assessed with the Bayley Scales of Infant Development-Second Edition (BSID-II), from which both mental development index (MDI) and psychomotor development index (PDI) scores and four indices of attention were derived: sustained attention, selective attention, attention shift, and total attention. Gestational age-stratified preterm groups differed significantly in T(3) and FT(4) levels at 2 and 4 weeks of life in infants born less than 27 weeks gestation. Preterm infants overall scored significantly below full-term on BSID-II MDI and PDI, selective, sustained, and total attention scales. In the preterm group, FT(4) levels were positively associated with PDI and selective, sustained, and total attention. Reduced levels of thyroid hormone in the neonatal period in preterm infants are associated with a reduced neurocognitive outcome in the attention domain at 3 months corrected age.

Association between motor and mental functioning in toddlers with cerebral palsy

Purpose: This study aimed to examine the association between motor and mental functioning in toddlers with cerebral palsy (CP).Methods: The Mental and Motor Scales of the Bayley Scales of Infant Development–Second Edition (BSID-II) were administered.Subjects: Seventy-eight toddlers with CP (mean age 2 years 7 months, SD 1 month; 43 boys, Gross Motor Function Classification System (GMFCS) levels I–V).Results: Spearman correlation coefficient between mental and motor functioning was 0.67 (p < 0.01). Sixty-five percent (51/78) of the children performed at the same level in mental and motor functioning, of whom 36 performed significantly delayed and 15 within normal limits. Thirty-five percent of the children were more delayed in motor functioning than in mental functioning.Conclusion: In toddlers with CP, an association was found between mental and motor functioning. When motor and mental functioning did not develop synchronously, it was always in favour of mental functioning. This study emphasizes the importance of examining both mental functioning and motor functioning separately of pre-school children with CP.

Brief Report: Taiwanese Infants' Mental and Motor Development 6 24 Months

To establish the normative data of the Bayley Scales of Infant Development-Second Edition (BSID-II) on Taiwanese infants from age 6 to 24 months and to explore the factors that relate to their mental and motor development. Five hundred and seven Taiwanese full-term infants were prospectively examined with the BSID-II at 6, 12, 18, and 24 months of age. Taiwanese infants' Bayley mental and motor raw scores were lower than the United States norms from age 6 to 24 months, however, the discrepancy gradually declined with increasing age. Gender, intrauterine growth status, birth order, region of residence, maternal education, and paternal occupation were shown to have longitudinal associations with their mental and/or motor scores. Differences existed in the mental and motor development among Taiwanese and American infants. Our preliminary norms of the BSID-II may be more appropriate than the United States norms for Taiwanese children.

Presurgical and postsurgical assessment of the neurodevelopment of infants with single-suture craniosynostosis: comparison with controls

Although most infants with single-suture craniosynostosis (SSC) appear to have neurodevelopmental test scores in the average range, SSC has been associated with cognitive and motor delays during infancy. Whether and when surgery improves such deficits are not yet known. The authors aimed to compare the pre- and postsurgical neurodevelopmental status of patients with SSC with those of control infants without craniosynostosis. The authors conducted a large, multicenter, longitudinal study of 168 infants with craniosynostosis and 115 controls without synostosis who were of similar age, race, sex, and socioeconomic status. The authors assessed participants by using the Bayley Scales of Infant Development, Second Edition (BSID-II) and the Preschool Language Scale, Third Edition (PLS-3) at enrollment, before patients' intracranial surgery, and when participants were 18 months of age (after surgery for patients). After adjusting for potential confounding factors in linear regression analyses, the authors found a tendency for patients to perform similarly to or slightly worse than controls on neurodevelopmental examinations at both visits. After surgery, the patients' mean scores were 0.6 to three points lower than those of controls on the five BSID-II and PLS-3 scales (p = 0.02-0.07). Compared with controls, patients had 2.3 and 1.9 times the adjusted odds of scoring in the delayed range on either BSID-II scale (Mental Development Index and Psychomotor Development Index) for the first and second visits, respectively (p = 0.001 and p = 0.015, respectively). The patients' mean adjusted test scores were nearly unrelated to the timing of their surgery. These findings support recommendations for neurodevelopmental screening in infants with SSC. Longer follow-up, as is being conducted with the patients in the present study, will be critical for identifying the potential longer-term correlates of SSC and its surgical correction.

Predictors of motor development in children prenatally exposed to cocaine

The current study examined the pattern of motor development across the first 18 months of life in infants with in utero exposure to cocaine to determine how prenatal drug effects and level of exposure relates to motor development. Motor development was examined at 1, 4, 12, and 18 months of age (corrected for prematurity). Infants were divided into cocaine exposed ( n=392) and comparison ( n=776) groups. Exposure status was determined by meconium assay and maternal self-report with alcohol, marijuana, tobacco, and opiates present in both groups. Motor skills were assessed at 1 month using the NICU Network Neurobehavioral Scale (NNNS), at 4 months using the posture and fine motor assessment of infants (PFMAI), at 12 months using the Bayley Scales of Infant Development-Second Edition (BSID-II), and at 18 months using the Peabody Developmental Motor Scales (PDMS). Examiners masked to exposure status performed all assessments. Motor scores were converted to standard ( z) scores, and hierarchical linear modeling (HLM) was used to examine the change in motor skills from 1 to 18 months of age. Infants with exposure to cocaine showed low motor skills at their initial status of 1 month but displayed significant increases over time. Both higher and lower levels of tobacco use related to poorer motor performance on average. Heavy cocaine use related to poorer motor performance as compared to no use, but there were no effects of level of cocaine use on change in motor skills.

American society of maxillofacial surgeons outcome study: preoperative and postoperative neurodevelopmental findings in single-suture craniosynostosis.

The purpose of this study was to prospectively determine the neurodevelopmental effects associated with single-suture, nonsyndromic craniosynostosis before and after surgery. Children diagnosed with single-suture craniosynostosis were evaluated by a psychologist using the Bayley Scales of Infant Development-Second Edition (BSID-II) within 2 months before and again 1 year after surgical correction. The BSID-II is a widely used measure of infant cognitive and motor development. The scale consists of three parts, the Mental Developmental Index (MDI), the Psychomotor Developmental Index (PDI), and the Behavior Rating Scale. The MDI and PDI yield age-standard scores (mean, 100; SD, 16). The children ranged in age from 2.5 to 10 months at the time of the craniofacial reconstruction (average age, 5.9 months). Metopic synostosis was diagnosed in 23 percent, sagittal synostosis in 45 percent, and unilateral coronal synostosis in 32 percent of patients. Twenty-two patients were evaluated preoperatively, of whom 15 patients were evaluated postoperatively. Mean baseline BSID-II scores revealed a mild delay in mental and motor scores (MDI, 82.3; PDI, 79.5). Mean postoperative BSID-II scores still revealed a mild delay in mental scores but significantly improved motor scores (MDI, 79.3; PDI, 89.3). Of the 15 children, four (27 percent) had BSID-II evaluations that were in the average range for all scales and nine infants (60 percent) had at least one MDI or PDI score in the significantly delayed range (<70). Among children with single-suture nonsyndromic craniosynostosis, mean Bayley scores indicated mild baseline deficits in both mental and motor scores. After surgical treatment, improvement was seen in the motor scale. It appears from this sample that neurodevelopmental abnormalities may be present in children with single-suture synostosis, and some may persist at 1 year of follow-up.

P0256 DEVELOPMENTAL DELAY IN INFANTS WITH CHRONIC INTESTINAL FAILURE (CIFX) AND LIVER FAILURE.

Introduction: Developmental delay is known to occur in infants with liver failure. Not previously studied is the development of children with liver failure secondary to CIFx and the extent delay is due to inter current liver disease. Aim: To report the cognitive and motor development of children with CIFx and ascertain whether delay relates to severity of liver failure or hospitalisation Methods: 15 patients median age (months) 10, (3.6 – 14.5) with CIFx, and 27 patients median age (months) 6.13 (3.7 – 15.1) with EHBA were assessed using the Bayley Scales of Infant Development, Second Edition (BSID-II). Developmental age scores were calculated using the Mental Developmental Index (MDI) and Psychomotor Developmental Index (PDI) scores, providing an estimate of the patients’ cognitive and motor skills at assessment. 8 of the CIFx patients and none of the EHBA were born prematurely Results: There was significant delay of cognitive and motor development in both groups however the CIFx patients showed a much greater delay. These results cannot be explained by degree of liver failure, since bilirubin and albumin levels were similar in both groups. However, the CIFx patients did have a significantly longer period of admission than the EHBA group. A Jonckheere-Terpstra test proved significant suggesting that length of hospitalisation may account for some of the level of developmental delay (MDI: p=0.001; PDI: p=0.011, Jonckheere-Terpstra test).Table 1Conclusion: Patients who experience prolonged hospitalisation are at risk of severe developmental delay especially if they have CIFx and liver disease. This group of patients should receive intensive physiotherapy and play therapy.