Predictors of developmental disabilities after open heart surgery in young children with congenital heart defects

OBJECTIVES: To determine the prevalence of persistent developmental impairments in children with congenital heart defects and to identify factors that enhance risk for an adverse outcome. STUDY DESIGN: Eligible infants (n = 131) <2 years of age requiring open heart surgery were recruited prospectively. Subjects were assessed during surgery and again 12 to 18 months later with standardized developmental assessments and formal neurologic examinations. RESULTS: Mean age at follow-up testing was 19.1 +/- 6.6 months. Assessments indicated that 41% had abnormal neurologic examinations. Gross and/or fine motor delays were documented in 42%, and 23% demonstrated global developmental delay. Univariate and multiple regression models identified the following factors increasing the risk for persistent developmental deficits: preoperative and acute postoperative neurodevelopmental status and microcephaly, type of heart lesion, length of deep hypothermic circulatory arrest, age at surgery, and days in the intensive care unit (P <.05). CONCLUSIONS: Children with congenital heart defects commonly have ongoing neurologic, motor, and developmental deficits well after surgical correction. The cause is multifactorial and includes brain injury before, during, and after heart surgery.     

Neurodevelopmental outcome at 2 years in 23 to 26 weeks old gestation infants

BACKGROUND: Assessment of neurologic and developmental outcome at 2 years age of infants with gestational age (GA)<27 weeks, born between 1996-2001. PATIENTS: A total of 110 live-born preterm infants with GA<27 weeks. METHODS: Main outcome criterions: Neurologic examination (according to Touwen) and classification of cerebral palsy by using the Gross Motor Function Classification System (GMFCS) at the corrected age of 1 and 2 years; assessment of mental and psychomotor development by using the Griffith Mental Development scales at the corrected age of 2 years; growth assessment at birth, 1 and 2 years. RESULTS: Mortality was 52%. Regular follow up was performed in 48 (91%) of the 53 surviving infants. Neurologic outcome: at 1 year age: 2% nonambulant cerebral palsy, 25% mild neurologic signs and 73% normal; at 2 years age: 4% nonambulant cerebral palsy, 2% ambulant cerebral palsy, 4% mild neurologic signs and 90% normal neurology. Developmental outcome at 2 years age: 40% DQ>-1 SD, 6% DQ between -1 SD and -2 SD (mild delay), 35% DQ between -2 SD and -3 SD (moderate delay) and 19% DQ<-3 SD (severe delay). Overall disability was found in 64%, severe disability in 27% of the infants. Profound growth failure in weight and head circumference<3rd centile at 2 years age was recorded in 39 and 19% of the infants, respectively. CONCLUSION: Developmental delay is very common in preterm infants<27 GA and exceeds the number of neurological disabilities (including cerebral palsy).     

Neonatal hyperviscosity: randomized study of effect of partial plasma exchange transfusion on long-term outcome

The use of partial plasma exchange transfusion in newborns with polycythemia and hyperviscosity was evaluated. Ninety-three infants with polycythemia and hyperviscosity were randomly assigned to receive either partial plasma exchange transfusion or symptomatic treatment; the infants were matched with control infants without polycythemia. Neonatal course and outcome at 1 and 2 years were evaluated for each of the three groups. Polycythemic infants had more fine motor and speech problems at 1 year of age than did control infants. At 2 years of age, polycythemic infants had more gross motor delays, neurologic diagnoses, fine motor abnormalities, and speech delays than did the control infants. There was no significant difference at 1 year between the polycythemic infants who had received partial plasma exchange transfusion and those given only symptomatic care. At 2 years, the group receiving partial plasma exchange transfusion had fewer neurologic diagnoses and fine motor abnormalities.     

早期干预对早产儿早期神经发育的影响

目的 分析早产儿和足月儿神经发育结局,探讨高危儿门诊对早产儿早期神经发育干预的作用,为临床早期干预提供一定依据.方法 选择2008-2011年在我院高危儿门诊规律随访的早产儿为观察组,随机抽取同期在本院儿童保健门诊保健的健康足月儿为对照组,在早产儿校正年龄/足月儿生后年龄3个月、6个月时分别进行Gesell测试和DDST筛查,评估并比较两组婴儿的神经发育.结果 校正年龄3个月时,早产儿大运动、语言、适应性与个人社交落后于足月儿[（62.2＆#177;22.7）分比（82.6＆#177;20.0）分,（91.8＆#177;49.3）分比（117.0 ＆#177;22.5）分,（63.1 ＆#177;29.5）分比（88.7＆#177;19.4）分,（66.5＆#177;35.7）分比（92.2＆#177;17.5）分,P均＜0.05],两组精细运动差异无统计学意义（P＞0.05）;校正年龄6个月时,早产儿大运动、精细运动、语言、适应性、个人社交等发育商与足月儿差异均无统计学意义（P＞0.05）.早产儿校正年龄6个月时大运动、适应性和个人社交发育商与校正年龄3个月时相比均提高,差异有统计学意义（P＜0.05）.结论 早产儿早期发育商水平低于足月儿,尤其是大运动、语言、适应性和个人社交落后于足月儿.通过早期干预,早产儿发育商后期能追赶上足月儿.应重视早产儿出院后干预,促进早产儿潜在能力得到最大程度的发挥.     

Longitudinal motor development of "apparently normal" high-risk infants at 18 months, 3 and 5 years

Background: Motor development appears to be more affected by premature birth than other developmental domains, however few studies have specifically investigated the development of gross and fine motor skills in this population. Aim: To examine longitudinal motor development in a group of “apparently normal” high-risk infants. Setting: Developmental follow-up clinic in a perinatal centre. Study design: Longitudinal observational cohort study. Subjects: Fifty-eight infants born less than 29 weeks gestation and/or 1000 g and without disabilities detected at 12 months. Outcome measures: Longitudinal gross and fine motor skills at 18 months, 3 and 5 years using the Peabody Developmental Motor Scales. The HOME scale provided information of the home environment as a stimulus for development. Results: A large proportion (54% at 18 months, 47% at 3 years and 64% at 5 years) of children continued to have fine motor deficits from 18 months to 5 years. The proportion of infants with gross motor deficits significantly increased over this period (14%, 33% and 81%, p<0.001), particularly for the ‘micropreemies’ (born <750 g). In multivariate analyses, gross motor development was positively influenced by the quality of the home environment. Conclusions: A large proportion of high-risk infants continued to have fine motor deficits, reflecting an underlying problem with fine motor skills. The proportion of infants with gross motor deficits significantly increased, as test demands became more challenging. In addition, the development of gross and fine motor skills appears to be influenced differently by the home environment.     

Neurodevelopmental status of newborns and infants with congenital heart defects before and after open heart surgery

BACKGROUND: Neurodevelopmental disabilities in children with congenital heart defects (CHDs) have been primarily attributed to intraoperative events without consideration of preoperative and postoperative factors. OBJECTIVE: To describe the preoperative and postoperative neurodevelopmental status of newborns and infants with CHDs. STUDY DESIGN: One hundred thirty-one children (56 newborns and 75 infants) were evaluated before and after surgery by using standardized neurobehavioral (newborn) and motor assessments (infant) and neurologic examinations. RESULTS: In newborns, neurobehavioral abnormalities were documented in >50% before surgery, with abnormalities persisting in most after surgery. In infants, neurodevelopmental abnormalities were observed in 38% before surgery. There was a significant association between preoperative and postoperative neurodevelopmental status, with status remaining unchanged in most. Newborns with acyanotic heart lesions were more likely to demonstrate neurologic compromise than those with cyanotic defects. For infants, arterial oxygen saturations <85% were significantly associated with an abnormality. There was a trend for a longer circulatory arrest time to be associated with greater risk for neurologic sequelae in newborns, whereas prolonged cardiopulmonary bypass was an important risk factor for infants. CONCLUSIONS: Neurodevelopmental abnormalities are common in young infants with CHDs and are often present before open heart surgery. These developmental concerns are clinically underappreciated. Early systematic developmental screening may be warranted in this population of interest.     

Motor function at school age in children with a preschool diagnosis of developmental language impairment

OBJECTIVES: To evaluate fine motor (FM) and gross motor (GM) function shortly after school entry in children with a preschool diagnosis of developmental language impairment (DLI). STUDY DESIGN: A cohort of children (n = 70) diagnosed at pre-school age with DLI was reevaluated in elementary school. Language, cognitive, and motor outcomes were assessed through the use of the Battelle Developmental Inventory (BDI). Language was further assessed through the use of the Vineland Adaptive Behavior Scale, Peabody Picture Vocabulary, and Expressive One Word Picture Vocabulary Tests. Performance below -1.5 SD of the normative mean on any test was considered to represent impairment. RESULTS: Forty-three children (mean age, 7.4 +/- 0.7 years) underwent reassessment at a mean of 3.8 +/- 0.7 years after initial preschool assessment. Mean scores for BDI motor domains (FM, 78.3 +/- 11.4; GM, 84.9 +/- 13.3) fell below normative values. Twenty-two children (52%) had motor impairment (FM, 17 of 42; GM, 15 of 42); 35 of 43 (81%) continued to have language impairment. BDI communication raw scores correlated most strongly with FM (rho = 0.73, P < .001) and GM (rho = 0.58, P = .003) raw scores but showed only moderate correlations with cognitive raw scores (rho = 0.41, P = .05). CONCLUSIONS: Impaired motor function is an important comorbidity in DLI. Factors critical to motor performance may also contribute to language deficits in DLI.     

Incidence and prevalence of HIV encephalopathy in children with HIV infection receiving highly active anti-retroviral therapy (HAART)

OBJECTIVE: To describe neurologic outcomes in children infected with HIV in the era of highly active anti-retroviral therapy (HAART), including rates of progressive HIV encephalopathy (PHE) and clinical sequelae among PHE survivors. STUDY DESIGN: Neurobehavior and school placement was assessed prospectively in the year 2000 in 126 children infected with HIV. PHE, developmental delay, and attention deficit disorder (ADHD) were the main outcome variables analyzed. Predictors of PHE were assessed in controlled analysis among age-matched controls. RESULTS: The rate of active PHE in 2000 was 1.6% (n = 2), and the prevalence of arrested PHE was 10% (n = 13). Residual motor and cognitive sequelae and need for special education was found in the majority of survivors. PHE relapse occurred in 3 (23%) children with previously arrested PHE. Viral load (VL) was the only significant factor associated with PHE. HIV or PHE was not associated with ADHD. Isolated developmental delay was not associated with HIV. CONCLUSIONS: PHE is an infrequent and reversible complication of HIV infection that responds to HAART and that may relapse if control of the virus is lost. Children with arrested PHE show higher rates of residual neurologic, cognitive, and scholastic impairments compared with children who never had PHE. Children with arrested PHE are the group of children with HIV infection most at risk for PHE, in the form of a relapse.     

Association between sleep position and early motor development

OBJECTIVE: To compare motor performance in infants sleeping in prone versus supine positions. STUDY DESIGN: Healthy 4-month-olds (supine: n = 71, prone: n = 12) and 6-month olds (supine: n = 50, prone: n = 22) were evaluated with the Alberta Infant Motor Scale (AIMS) and Peabody Developmental Motor Scale (PDMS), and parents completed a positioning diary. Infants were reassessed at 15 months. RESULTS: At 4 months, motor scores were lower in the supine group and were less likely to achieve prone extension (P < .05). At 6 months, there were wide discrepancies on the AIMS (supine: 44.5 +/- 21.6, prone: 60.0 +/- 18.8, P = .005) and the gross motor PDMS (supine: 85.7 +/- 7.6, prone: 90.2 +/- 9.5, P = .03). Motor delays were documented in 22% of babies sleeping supine. Prone sleep-positioned infants were more likely to sit and roll. Daily exposure to awake prone positioning was predictive of motor performance in infants sleeping supine. At 15 months, sleep position continued to predict motor performance. CONCLUSIONS: Infants sleeping supine may exhibit early motor lags, associated with less time in prone while awake. This has implications for accurate interpretation of assessment of infants at risk and prevention of inappropriate referrals. Rate of infant motor development appears influenced by extrinsic factors such as positioning practices.     

Validity of the fine motor area of the 12-month ages and stages questionnaire in infants following major surgery

The Ages and Stages Questionnaires (ASQ) are parent-report screening tools to identify infants at risk of developmental difficulties. The purpose of this study was to examine validity and internal reliability of the fine motor developmental area of the ASQ, 2nd edition (ASQ2-FM) for screening 12-month-old infants following major surgery. The ASQ2-FM was completed by caregivers of 74 infants who had cardiac surgery in the first 90 days of life, 104 infants who had noncardiac surgery in the first 90 days of life, and a control group of 154 infants. The Rasch item response analysis revealed that the ASQ2-FM had poor ability to discriminate among levels of fine motor ability. Sensitivity was poor (20%) and specificity was good (98%) when compared with the scores for the fine motor subscale of the Bayley Scales of Infant and Toddler Development. The ASQ2-FM under-identified infants at risk for fine motor delay; internal reliability and construct validity do not support use as a screening tool of fine motor development of infants aged 12 months who have undergone major surgery.     

Neurologic status of newborns with congenital heart defects before open heart surgery

Controversy exists regarding the integrity of the nervous system in the newborn with a congenital heart defect who must undergo corrective or palliative open heart surgery. Neurodevelopmental sequelae have been primarily attributed to surgical procedures without standardized evaluation of the preoperative neurologic status. OBJECTIVE: To determine whether newborns with congenital heart defects demonstrate abnormalities in neurobehavioral status before surgery. STUDY DESIGN: In this prospective study, a standardized neonatal neurobehavioral assessment and a neurologic examination were conducted independently in a consecutive series of 56 neonates referred to our hospital for investigation of open heart surgery. RESULTS: Neurobehavioral and neurologic abnormalities were documented in greater than half of the cohort and included hypotonia, hypertonia, jitteriness, motor asymmetries, and absent suck. Poor state regulation (62%) and feeding difficulties (34%) also were commonly observed. Furthermore, 3 subjects had seizures, 35.7% were microcephalic, and 12.5% were macrocephalic. The overall likelihood of neurobehavioral abnormalities was not enhanced by indicators of cardiorespiratory compromise. Interestingly, newborns with acyanotic congenital heart defects were more likely to demonstrate neurologic compromise than were those with cyanotic defects. CONCLUSIONS: Findings suggest that the prevalence of neurobehavioral abnormalities before surgery in newborns with congenital heart defects has been underappreciated and would indicate that factors other than intraoperative procedures should be considered in the genesis of brain injury in this population.congenital heart defects, neurologic examination, newborn.     

Validity of the Fine Motor Area of the 12-Month Ages and Stages Questionnaire in Infants Following Major Surgery

ABSTRACT

The Ages and Stages Questionnaires (ASQ) are parent-report screening tools to identify infants at risk of developmental difficulties. The purpose of this study was to examine validity and internal reliability of the fine motor developmental area of the ASQ, 2nd edition (ASQ2-FM) for screening 12-month-old infants following major surgery. The ASQ2-FM was completed by caregivers of 74 infants who had cardiac surgery in the first 90 days of life, 104 infants who had noncardiac surgery in the first 90 days of life, and a control group of 154 infants. The Rasch item response analysis revealed that the ASQ2-FM had poor ability to discriminate among levels of fine motor ability. Sensitivity was poor (20%) and specificity was good (98%) when compared with the scores for the fine motor subscale of the Bayley Scales of Infant and Toddler Development. The ASQ2-FM under-identified infants at risk for fine motor delay; internal reliability and construct validity do not support use as a screening tool of fine motor development of infants aged 12 months who have undergone major surgery.     

Cerebral palsy in Norway: prevalence, subtypes and severity.

BACKGROUND/AIM: To describe prevalence, subtypes and severity of cerebral palsy (CP) in Norway using criteria proposed by the Surveillance of Cerebral Palsy in Europe (SCPE) network. MATERIAL: All children in Norway with CP born in January 1996-December 1998 were registered in the Cerebral Palsy Registry of Norway. The Medical Birth Registry of Norway provided the perinatal data. RESULTS: A total of 374 children with CP were identified with a prevalence of 2.1 per 1000 live births. Detailed information was obtained from 294 (79%) children. Median age at clinical assessment was 6.9 years (range: 1.9-10.2 years). Thirty-three percent of the children had spastic unilateral CP, 49% spastic bilateral, 6% dyskinetic, 5% ataxic CP and 7% were not classified. Severely impaired vision and hearing were present in 5% and 4% of the children, respectively. Active epilepsy was present in 28%, mental retardation in 31% and severely impaired or no speech in 28% children. The most severe impairments in gross motor function were observed in children with low Apgar scores, and the most severe impairments in fine motor function in children born at term, with normal birth weight and low Apgar scores. CONCLUSION: Compared with other populations, the prevalence of CP as well as the proportions of subtypes and gross motor impairments were similar, whereas fine motor impairments and associated impairments were more common. The classification of children with mixed forms of CP is still a challenge. Children were more severely affected if Apgar scores were low, and if they were born at term.     

Neurologic disorders and cerebral lesion in children with TGA (transposition of the great arteries) (author's transl)

118 consecutive patients with TGA were examined for neurologic disorders (ND) and cerebral lesions (CL) between 1967--1979 of whom 43 are alive. 66 had simple TGA, 52 had VSD, PS and/or coarctation as additional lesion.--43 were corrected surgically of whom 32 are alive.--Neurologic examination was performed in all, EEG, CT, arteriography, scintigraphy and autopsy when necessary or possible. Patholog. findings (CL, ND) were detected in 66; 47 had definite ND or CL, while 19 showed gross motor development delay. 52 of 118 children with TGA were without neurolog. or cerebral pathology. --Several factors were investigated for their etiologic influence: 1. Additional cardiac lesions played no significant role. 2. The lower O2 saturation in the ascending aorta of the fetal circulation in TGA may be of importance. 3. Red blood count, hemoglobin, hematocrit were higher in the group with ND or CL. 4. Early appearance of cerebral lesion should suggest earlier corrective surgery.     

Early development of children with major birth defects requiring newborn surgery

Aim: To describe neurodevelopmental outcomes of neonates following cardiac or non‐cardiac surgery for major birth defects. Methods: From 1 June 2002 to 31 July 2004, infants born ≥33 weeks gestation who underwent major birth defect surgery were enrolled prospectively. Infants were assessed at a mean corrected age of 24 months (standard deviation (SD) = 8 months, range 18–36 months) using the Bayley Scales of Infant Development: Second Edition. Results: Of the 118 study infants, 79 (66%) were male, the mean gestation was 38.5 weeks (SD 1.9 weeks) and mean birthweight was 3194 g (SD 653 g). Forty‐five infants (47%) had undergone general surgery for non‐cardiac defects. The majority of infants (73%) performed below average in cognitive and language skills. Mental delay was found in 41% of infants; 16% were significantly delayed. Fine and gross motor skills were below average in 60% of infants. Twenty‐six percent of infants had motor delay; 9% were significantly delayed. Both the mean Mental Development Index (M = 88, SD = 19.8) and mean Psychomotor Development Index (M = 93, SD = 19.3) were significantly below the normative mean (d = 0.8, P < 0.001 and d = 0.5, P < 0.001, respectively). One in five children had global developmental delay. There was no significant difference in outcome between the cardiac and general surgery groups. Conclusions: The majority of infants performed below average on a standardised test of infant development. Our results show that infants requiring newborn surgery for major birth defects are at high risk of adverse neurodevelopmental outcomes. We recommend that follow‐up programmes include systematic multidisciplinary developmental monitoring and early intervention.     

Neurologic complications of HIV infection in children.

Neurologic abnormalities occur frequently in children with symptomatic HIV-1 infection (class P2) and include cognitive, language and motor deficits, as well as acquired microcephaly. Neurologic abnormalities can be seen as early as the first 3 months of age and can precede signs of immune deficiency and systemic illness. Hypotonia, delayed or poor head control and decreased vocalizations are some of the early neurologic manifestations of HIV-1 infection. In the majority of cases CNS impairment appears to be related to HIV-1 brain infection although at this time the exact timing of CNS invasion by the virus and the pathogenesis of CNS dysfunction are unknown. Treatment with antiretroviral agents can at least temporarily improve neurologic functioning in some children with HIV-1-related encephalopathy.     

Early gross motor development of preterm infants according to the Alberta Infant Motor Scale

OBJECTIVE: To systematically examine gross motor development in the first 18 months of life of preterm infants. STUDY DESIGN: A total of 800 preterm infants (356 boys), ages between 1 and 18 months and corrected for degree of prematurity, were assessed with the use of the Alberta Infant Motor Scale. RESULTS: Comparison of the mean Alberta Infant Motor Scale scores of the preterm infants with the norm-referenced values derived from term infants revealed that as a group, the preterm infants scored significantly lower at all age levels, even with full correction for degree of prematurity. CONCLUSIONS: In general, preterm infants exhibit different gross motor developmental trajectories compared with term infants in the first 18 months of life. The gross motor developmental profile of preterm infants may reflect a variant of typical gross motor development, which seems most likely to be specific for this population. As a consequence, adjusted norms should be used for proper evaluation and clinical decision-making in relation to preterm infants.     

Motor Developmental Status of Moderately Low Birth Weight Preterm Infants

Objective: Motor development is frequently reported to be impaired in very low birth weight (VLBW) infants, but little is known about the moderately low birth weight (MLBW) infants. The aim of this study was to investigate whether MLBW preterm infants present developmental delay. Methods: In a historical cohort study, 18±2 month-old infants with a history of low birth weight (LBW) were identified. All infants with complications of LBW with negative effects on development were excluded. Healthy infants with normal birth weight (2500–4000 g) were included as controls. All infants were evaluated by the Peabody Developmental Motor Scale II (PDMS-2) test and final scores compared between the two groups. Finding: 88 infants including 58 MLBW and 30 NBW with a mean birth weight of 1900±382.4 g and 3150±473.5 g respectively, were studied. In the MLBW group, gross and fine motor skill scores were below average in 6 (6.8%) and 10 (17%) infants, respectively. There were no significant differences between the two groups according to gross motor quotient (102.5±5.5 in NBW vs 100.1±7.2 in MLBW; P=0.1), but MLBW infants achieved significantly lower scores in fine motor (93.3±5.4 vs 99.6±5.0; P=0.001) and total motor quotient (97.0±5.9 vs 101.53±5.0; P=0.001). Conclusion: The finding of this study show developmental defects in fine motor skills in MLBW infants. Accurate monitoring of the developmental status of this population should be emphasized for an earlier recognition and intervention.Key Words: Low Birth Weight, Peabody Developmental Motor Scale II (PDMS-2), Motor Development, Infant     

Eye-hand co-ordination skills in very preterm infants <29 weeks gestation at 3 years: Effects of preterm birth and retinopathy of prematurity

INTRODUCTION: Preterm infants are known to have low gross motor and fine motor skills. We questioned whether poor eye-hand coordination skills are associated with moderate to severe stages of Retinopathy of Prematurity (ROP). AIMS: The aim of this study was to examine development, with specific reference to eye-hand coordination skills, among preterm infants <29 weeks gestation with different stages of ROP at 3 years of age. METHODS AND MATERIALS: Fifteen preterm infants (<29 weeks gestation) who developed Stage 3 ROP were matched for gestation, birthweight and gender with infants who developed Stage 2 and Stage 1/no ROP. Developmental (Griffiths Mental Development Scales and Peabody Developmental Motor Scales) and ophthalmic assessments in the 3 matched groups of 15 were performed at 3 years of age. RESULTS: 1) Whilst the eye-hand coordination scores and Peabody fine motor scores were lower in the Stage 3 ROP group, they were not significantly lower than the other ROP groups. 2) Locomotor, Peabody gross motor skills and hearing and speech were significantly lower in the infants with Stage 3 ROP. The other developmental domains were not significantly different to the severe ROP group. 3) All 3 groups (of preterm infants) had lower eye-hand coordination and Peabody fine motor scores compared to test norms. 4) There were 8 of 15 infants with Stage 3 ROP who developed moderate visual problems by 3 years of age. CONCLUSION: In preterm infants, low eye-hand coordination/fine motor scores are likely to be due to their extreme prematurity.     

Prognosis of motor development and joint hypermobility.

In a study of 59 infants aged 18 months there were 20 with joint hypermobility and delayed motor development, 19 with joint hypermobility and normal motor development, and 20 normal controls. They were reassessed for motor function 3.5 years later at the age of 5 years. Both gross and fine motor performance were significantly delayed in the group of children who exhibited joint hypermobility and motor delay in infancy. No significant delay was evident in those with joint hypermobility only. Joint hypermobility resolved more frequently in children who presented normal motor development at age 18 months. Infants with joint hypermobility and motor delay are a subgroup associated with a less favourable motor outcome and careful follow up is indicated.