Gross Motor Development of Children with Congenital Heart Disease Receiving Early Systematic Surveillance and Individualized Intervention: Brief Report.

Purpose: This retrospective study aims to describe the gross motor development of children aged 4 to 24 months with congenital heart disease (CHD) enrolled in a systematic developmental follow-up program and to describe the frequency of physical therapy sessions they received between 4 and 8 months of age. Methods: Twenty-nine infants with CHD underwent motor evaluations using the AIMS at 4 months, and the Bayley-III at 12 and 24 months. Results: Based on AIMS, 79% of 4-month-old infants had a gross motor delay and required physical therapy. Among these, 56.5% received one to two physical therapy sessions, and 43.5% received three to six sessions. Infants who benefited from regular interventions tended to show a better improvement in motor scores from 12 to 24 months. Conclusion: This study highlights the importance of early motor screening in infants with CHD and suggests a potential benefit of early physical therapy in at-risk children. Abbreviations: CHD: Congenital heart disease; AIMS: Alberta Infant Motor Scales; Bayley-III: Bayley Scales of Infant and Toddler Development, Third edition; Bayley-III/GM: Gross Motor section of the Bayley Scales of Infant and Toddler Development, Third edition.

Impacts of an Interdisciplinary Developmental Follow-Up Program on Neurodevelopment in Congenital Heart Disease: The CINC Study.

Objectives: This study investigates the impact of an early systematic interdisciplinary developmental follow-up and individualized intervention program on the neurodevelopment of children with complex congenital heart disease (CHD) who required cardiac surgery. Study Design: We prospectively enrolled 80 children with CHD: 41 were already followed at our neurocardiac developmental follow-up clinic from the age of 4 months, while 39 were born before the establishment of the program and therefore received standard health care. We conducted cognitive, motor, and behavioral assessments at 3 years of age. We used one-way multivariate analyses of variance to compare the neurodevelopmental outcome of both groups. Results: Between-group analyses revealed a distinct neurodevelopmental profile with clinically significant effect size (P < 0.001, partial η2 = 0.366). Children followed at our clinic demonstrated better receptive language performances (P = 0.048) and tended to show higher scores on visuo-constructive tasks (P = 0.080). Children who received standard health care exhibited greater performances in working memory tasks (P = 0.032). We found no group differences on global intellectual functioning, gross and fine motor skills, and behaviors. Referral rates for specific remedial services were higher in patients followed at our neurocardiac clinic compared to the historical cohort (P < 0.005). Conclusions: Overall, the impact of the developmental follow-up and individualized intervention program on neurodevelopmental outcomes remains subtle. Nevertheless, results, although limited by several factors, point toward an advantage for the children who took part in the program regarding receptive language skills over children who received standard health care. We hypothesize that group differences may be greater with growing age. Further research involving larger cohorts is needed to clearly assess the effectiveness of neurocardiac developmental follow-up programs at school age.

Superior Performance in Prone in Infants With Congenital Heart Disease Predicts an Earlier Onset of Walking.

Infants with congenital heart disease are at risk of impaired neurodevelopment, which frequently manifests as motor delay during their first years of life. This delay is multifactorial in origin and environmental factors, such as a limited experience in prone, may play a role. In this study, we evaluated the motor development of a prospective cohort of 71 infants (37 males) with congenital heart disease at 4 months of age using the Alberta Infant Motor Scales (AIMS). We used regression analyses to determine whether the 4-month AIMS scores predict the ability to walk by 18 months. The influence of demographic and clinical variables was also assessed. Fifty-one infants (71.8%) were able to maintain the prone prop position (AIMS score of ≥3 in prone) at 4 months. Of those, 47 (92.2%) were able to walk by 18 months compared to only 12/20 (60%) of those who did not maintain the position. Higher AIMS scores were predictive of a greater likelihood of walking by 18 months ( P < .001), with the scores in prone having a higher predictive ability compared to those in other positions (Exp(B) 15.2 vs 4.0). Shorter hospital stays and female gender were also associated with an earlier onset of walking. In conclusion, our study demonstrates that early ventral performance in infants with congenital heart disease impacts the age of acquisition of walking and could be used to guide referral to rehabilitation.

Significant motor improvement in an infant with congenital heart disease and a rolandic stroke: The impact of early intervention.

OBJECTIVE: To report the impact of early motor intervention in an infant with congenital heart disease (CHD) and a stroke. METHODS AND RESULTS: A 35-week newborn with a complex CHD and a normal MRI presented with early motor developmental delay at 2 months. She began an intervention program, which included biweekly motor developmental therapy with a physiotherapist, parental education, and daily home exercises. At 4 months, she underwent cardiac surgery. Following surgery, she was diagnosed with a stroke involving the right sylvian artery territory. She continued her intensive intervention program. The 12-month assessment revealed an evident gain of motor function. Despite MRI evidence of a chronic infarct involving the primary motor cortex, the child had normal motor functions. CONCLUSION: This case report supports the positive impact of early intervention in infants with CHD and its potential effect on enhancing neuroplasticity, even in children with cerebro-vascular accidents involving areas of motor function.

Epidemiology of severe hearing impairment in a population-based cerebral palsy cohort.

BACKGROUND: Comorbidities including hearing impairment occur commonly in individuals with cerebral palsy (CP). METHODS: Hearing impairment was assessed in a registry-derived population-based sample of children with CP. RESULTS: Hearing impairment was documented in 12.7% (27 of 212) with less than a quarter of these (or 2.7% overall [6 of 212]) having a severe hearing loss of greater than 70 dB bi-aurally. Those with severe hearing impairment were more likely to be nonambulatory (Gross Motor Function Classification System IV/V; 100% versus 34.0% in the registry; P < 0.001) and have a spastic quadriplegic or dyskinetic CP variant (100% versus 42%, P = 0.001). Severe neonatal hyperbilirubinemia necessitating exchange transfusion demonstrated a striking association with hearing impairment in the context of CP in spite of small observed numbers; three of three had severe hearing loss, accounting for 50% of cases of severe hearing loss. CONCLUSIONS: These results suggest a specific CP context and antecedent conditions that necessitate a heightened vigilance for the detection and remediation of hearing impairment.

Spectrum of visual disorders in a population-based cerebral palsy cohort.

BACKGROUND: Children with cerebral palsy are known to be at increased risk for visual impairment. METHODS: In a population-based sample drawn from a geographically defined registry, the profile of visual impairment in children with cerebral palsy was investigated. RESULTS: Close to half (49.8%; 106/213) had a visual impairment. The majority of these individuals had strabismus (55.7%; 59/106) and a slightly lesser fraction had refractive errors (20.7%; 22/106) or severe visual loss (18.9%; 20/106). The vast majority of children with severe visual loss had spastic quadriplegia (83%; 17/20) or were nonambulatory (i.e., Gross Motor Function Classification Scale IV/V, 80%; 16/20). CONCLUSIONS: Knowledge of this profile will assist practitioners to heighten their appreciation of potential visual disturbances in certain subsets of children with cerebral palsy.

Normal imaging in patients with cerebral palsy: what does it tell us?

OBJECTIVE: To identify distinctive clinical features characterizing children with cerebral palsy (CP) and normal-appearing magnetic resonance imaging (MRI) findings. STUDY DESIGN: Using a population-based CP registry, the Registre de la Paralysie Cérébrale au Québec (Quebec Cerebral Palsy Registry), various antenatal, perinatal, and postnatal predictor variables, as well as current phenotype, were compared in patients with normal-appearing MRI findings and those with abnormal MRI findings. RESULTS: Of the 213 patients evaluated, 126 (60%) had MRI imaging results available and were included in our analysis. Of these 126 patients, 90 (71%; 51 males, 39 females) had abnormal findings and 36 (29%; 17 males and 19 females) had normal-appearing findings. Compared with other CP variants, normal-appearing MRI was more prevalent (P = .001) in dyskinetic CP (72.7%; 8 of 11) and less prevalent (P = .002) in spastic hemiplegic CP (10%; 4 of 40). There were no significant differences between the 2 groups (P > .05) in terms of the prevalence of perinatal or postnatal clinical features or clinical outcomes. Furthermore, 42% (15 of 36) of the children with normal-appearing MRI exhibited a high degree of functional disability (Gross Motor Functional Classification System IV-V), compared with 33% (30 of 90) with abnormal MRI. CONCLUSION: No clinical features, except a higher prevalence of dyskinetic CP, was identified in the children with normal-appearing MRI. More refined imaging techniques may be needed to evaluate patients with normal-appearing MRI findings. Furthermore, genetic or functional, rather than gross structural lesions, may underlie the pathophysiology of CP in this cohort. Finally, the high proportion of substantial functional disability underscores the importance of continuous follow-up even in the absence of early structural abnormalities on imaging.

The relationship between gross motor function and manual ability in cerebral palsy.

A retrospective cohort study was conducted to describe the relationship between gross motor function and manual ability in children with cerebral palsy and explore differences between cerebral palsy subtypes and associated comorbidities. Children with cerebral palsy born between 1999 and 2008 were included from the Registre de la Paralyse Cérébrale de Québec identifying 332 children. The overall agreement between Gross Motor Function Classification System and Manual Ability Classification Scale Levels was moderate (kappa 0.457, standard error 0.034) with a strong positive correlation (Spearman rho of 0.820, standard error 0.023). This agreement was moderate among children with spastic quadriparesis and dysketic cerebral palsy, fair in children with spastic diplegia, and poor in children with spastic hemiplegia. Children with cognitive impairment showed a higher correlation than those without cognitive impairment. The correlation between gross motor function and manual ability in children with CP varies based on neurologic subtype and cognitive level.

Term neonatal encephalopathy antecedent cerebral palsy: a retrospective population-based study.

OBJECTIVE: To compare the clinical profile of term-born cerebral palsy children with or without antecedent moderate to severe neonatal encephalopathy. We hypothesized that antecedent neonatal encephalopathy is associated with a spastic quadriparesis cerebral palsy clinical profile, a higher severity of functional motor impairment, and a greater number of associated comorbidities. METHODS: Using the Quebec Cerebral Palsy Registry, neurologic subtype, Gross Motor Function Classification System stratification, and comorbidities were compared in children with cerebral palsy with and without antecedent neonatal encephalopathy. Differences between groups were evaluated using chi square analysis for categorical variables and student t test for continuous variables. RESULTS: We identified 132 children with cerebral palsy born full term over a 4 year-interval (1999-2002 inclusive) within the Quebec Cerebral Palsy Registry, of which 44 (33%) had an antecedent neonatal encephalopathy. Spastic quadriplegia subtype of cerebral palsy and Gross Motor Function Classification System Level III-V (non-independent ambulation) were significantly associated with antecedent neonatal encephalopathy. The mean number of comorbidities experienced was not different in the two groups. Of five documented comorbidities, only severe communication difficulties were found to be associated (p < 0.05) with antecedent neonatal encephalopathy. CONCLUSION: A pattern of increased neuromotor impairment, functional gross motor severity and possible communication difficulties was found in the 33% of children with cerebral palsy born at term and with a history of neonatal encephalopathy.

Does antenatal tobacco or alcohol exposure influence a child's cerebral palsy? A population-based study.

Antenatal tobacco and alcohol exposure are established risk factors for premature birth and an independent risk factor for cerebral palsy. Both exert adverse effects on fetal development. In children with cerebral palsy, whether antenatal exposure to tobacco or alcohol is associated with a difference in clinical profile remains unknown. The Quebec Cerebral Palsy Registry was used to compare neurologic subtypes, gross motor functional impairment, and comorbidities in children with cerebral palsy who were or were not prenatally exposed to alcohol or tobacco. Information on in utero exposure was available in 249 children with cerebral palsy born from 1999-2002, of whom 77 were exposed to alcohol and 62 to tobacco in utero. No association was evident between exposure to tobacco or alcohol during pregnancy and neurologic subtype, Gross Motor Function Classification System score, mean number of comorbidities experienced, or each of eight comorbidities explored. Adjusting for prematurity or low birth weight exerted no effect on these results. In utero exposure to tobacco or alcohol does not assist in predicting clinical profiles of cerebral palsy.

Congenital non-central nervous system malformations in cerebral palsy: a distinct subset?

AIM: The aim of this article was to identify and contrast the subset of children with cerebral palsy (CP) and non-central nervous system (CNS) congenital malformations with children with CP but no coexisting non-CNS congenital malformations. METHOD: A population-based regional comprehensive CP registry was used to identify children with CP who had non-CNS congenital malformations (n = 34; 19 males, 15 females; 22 classified as Gross Motor Function Classification System [GMFCS] levels I-III, 12 as GMFCS level IV or V). Their clinical features were then compared with other children with CP without non-CNS congenital malformations (n = 207; 115 males, 92 females; 138 classified as GMFCS levels I-III, 69 as GMFCS level IV or V). RESULTS: Children with CP and non-CNS congenital malformations did not differ from those without in terms of neurological subtype distribution or functional severity, as measured by the GMFCS. Also, there was no association with previous maternal infections (i.e. toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus 2 [TORCH]), maternal fever, use of illicit substances, asphyxia, neonatal encephalopathy, intraventricular haemorrhage, or septicaemia. The incidence of comorbidities such as convulsions, communication difficulties, gavage feeding, cortical blindness, and auditory impairment was not higher in this subgroup. INTERPRETATION: The incidence of congenital non-CNS malformations among children with CP is appreciable. Children with these non-CNS malformations do not appear to differ from other children with CP regarding neurological subtype, functional severity, and comorbidities, or maternal or obstetrical factors. Thus, the specific presence of a non-CNS congenital malformation does not appear to assist the practitioner in the management or understanding of a child's CP.

Predicting comorbidities with neuroimaging in children with cerebral palsy.

A population-based registry was used to ascertain whether neuroimaging findings of children with cerebral palsy could predict the occurrence of certain comorbidities. Neuroimaging findings and comorbidities data were extracted from the Quebec Cerebral Palsy Registry for children born in a 4-year birth interval (1999-2002) covering half of the province's population. Neuroimaging studies were classified into 10 mutually exclusive categories (periventricular white matter injury/leukomalacia, cerebral malformation, cerebral vascular accident, deep gray matter injury, superficial gray matter injury, diffuse gray matter injury, intracranial hemorrhage, infection, nonspecific findings, and normal). Comorbidities studied included cortical blindness, severe auditory impairment, inability to communicate verbally, assisted feeding, and the presence of afebrile seizures in the prior 12 months. Neuroimaging results were available for a total of 213 children. Only deep gray matter injury (defined as signal abnormality or volume loss in subcortical gray matter, n = 9) was significantly (P < 0.05) linked with the occurrence of both the inability to communicate verbally (n = 5, 55.6% vs n = 46, 22.5%, P = 0.04) and with a higher mean number of comorbidities (1.67 vs 0.70, P < 0.01), and therefore with increased burden of comorbidities. These findings may improve our ability to prognosticate the outcome of children with cerebral palsy, enabling targeted early direct interventions.

Population-based study of neuroimaging findings in children with cerebral palsy.

BACKGROUND: Neuroimaging is currently recommended as a standard evaluation in children with cerebral palsy (CP). AIMS: Utilizing imaging findings from a population-based registry (REPACQ), the frequency and proportion of cerebral radiologic abnormalities in children CP over a four year birth cohort was investigated. METHODS: Descriptions of CT and MRI studies were extracted from the Registry dataset and classified into 10 distinct categories. RESULTS: Two hundred and thirteen children had imaging available (119 males, 94 females, mean age of 44 months [SD. ± 14 months] at Registry inscription). Eighty seven percent of participants had documented cerebral abnormalities, the most common of which were periventricular white matter injury (PVWMI) (19.2%), diffuse gray matter injury (14.6%), cerebral vascular accident (CVA) (11.7%), and cerebral malformation (11.3%). Also, 18.8% of participants had non-specific radiologic findings and 13.1% of participants had normal imaging results. Severe CP (i.e. GMFCS Level IV-V) and spastic quadriplegic CP were significantly associated with the neuroimaging findings of gray matter injury, while spastic hemiplegic CP was association with CVA, and dyskinetic and spastic diplegic CP were both associated with normal and non-specific neuroimaging findings. CONCLUSIONS: Specific patterns of neuroimaging findings in children with CP were found to be associated with neurological subtype, CP severity (i.e. GMFCS Level) and other categorical variables.

Cross-sectional comparison of periventricular leukomalacia in preterm and term children.

OBJECTIVE: To document and contrast the characteristics of preterm and term-born children with cerebral palsy attributed to underlying radiologic periventricular white matter injury (leukomalacia) (PVWMI/PVL). METHODS: A comprehensive cerebral palsy population-based registry (REPACQ) for a 4-year inclusive (1999-2002) birth cohort was systematically searched for all children with radiologic evidence for PVWMI/PVL. Clinical features, neurologic subtype, gross motor functional impairment, and comorbidities were compared in those children born preterm (<37 weeks) and those born at term (> or = 37 weeks). RESULTS: Of 242 children with cerebral palsy in the registry, 213 had available neuroimaging, in which 41 had PVWMI/PVL: 26 preterm born and 15 term born. Neurologic subtype differed significantly between preterm and term-born children with respect to the frequency of spastic hemiplegia (5/26 vs 8/15; p < 0.05) and spastic diplegia (9/26 vs 2/15; p < 0.05). The groups also differed significantly from a functional perspective (Gross Motor Function Classification System for Cerebral Palsy level I-II; 12/26 vs 12/15; p < 0.05). The comorbidity spectrum was similar between the 2 groups except for the occurrence of cortical blindness in the term-born children (3/15 vs 0/26; p < 0.05). CONCLUSION: Differences between preterm and term-born children with cerebral palsy with periventricular white matter injury (leukomalacia) suggest that despite a common radiologic pattern, these are different clinicopathologic entities with perhaps a different gestational timing of acquired injury.

Determinants of ambulation in children with spastic quadriplegic cerebral palsy: a population-based study.

The objective of this study was to identify factors that predict ambulation in spastic quadriplegic cerebral palsy. A 4-year registry-based birth cohort was searched for patients with a diagnosis of spastic quadriplegic cerebral palsy. All patients were then divided in 2 groups: (a) Gross Motor Function Classification System level < or = III (ambulant group) and (b) Gross Motor Function Classification System level > or = IV (nonambulant group). Clinical features were then compared between the 2 groups. A total of 85 children with a diagnosis of spastic quadriplegic cerebral palsy were identified. Of these, 65 and 20 were classified in the ''nonambulant'' and ''ambulant'' groups, respectively. The presence of seizures in the first 24 or 72 hours of life and the administration of antibiotics during pregnancy/delivery were all associated with an eventual inability to achieve ambulation. A gestational age < or = 27 weeks, birth weight <1000 g, Caucasian mother, and the presence of hyperbilirubinemia were significantly linked with independent ambulation.

Comorbidities in cerebral palsy and their relationship to neurologic subtype and GMFCS level.

OBJECTIVE: Utilizing a population-based registry, the burden of comorbidity was ascertained in a sample of children with cerebral palsy and stratified according to both neurologic subtype and functional capability with respect to gross motor skills. METHODS: The Quebec Cerebral Palsy Registry was utilized to identify children over a 4-year birth interval (1999-2002 inclusive) with cerebral palsy. Information on neurologic subtype classified according to the qualitative nature and topographic distribution of the motor impairment on neurologic examination, Gross Motor Function Classification System (GMFCS) categorization of motor skills, and the presence of certain comorbidities (cortical blindness, auditory limitations, nonverbal communication skills, gavage feeding status, and coexisting afebrile seizures in the prior 12 months) was obtained. RESULTS: The frequency of individual comorbidities, their proportional distribution, and mean number of occurrences basically falls into a significant dichotomous distribution. Across the spectrum of comorbidities considered, these comorbidities are relatively infrequently encountered in those with spastic hemiplegic or spastic diplegic variants or ambulatory GMFCS status (levels I-III), while these entities occur at a frequent level for those with spastic quadriplegic, dyskinetic, or ataxic-hypotonic variants or nonambulatory GMFCS status (levels IV and V). CONCLUSION: The enhanced burdens of comorbidity are unevenly distributed in children with cerebral palsy in a manner that can be associated with either a specific neurologic subtype (spastic quadriplegic, dyskinetic, ataxic-hypotonic) or nonambulatory motor status (Gross Motor Function Classification System levels IV and V). This provides enhanced value to the utilization of these classification approaches.

The relationship of cerebral palsy subtype and functional motor impairment: a population-based study.

AIM: Traditionally, cerebral palsy (CP) had been classified according to the distribution and quality of motor impairment. A standardized functional classification of gross motor skills has recently been validated - the Gross Motor Function Classification System (GMFCS). The relationship between the neurological subtype of CP and GMFCS level remains undefined in CP. METHOD: The Quebec Cerebral Palsy Registry (Registre de la paralysie cérébrale au Québec [REPACQ]) over a 4-year birth interval (1999-2002 inclusive) identified 301 children with CP. Information on both CP subtype and GMFCS level was available for 243 children (138 males, 105 females) with final data extraction at a mean age of 44 months (SD 14mo, range 24-79mo). Proportions of children with a particular CP subtype at GMFCS levels I to III versus levels IV to V were determined and compared. RESULTS: CP subtype versus GMFCS levels I to III or IV to V was distributed proportionally as follows: spastic diplegic, 51/52 (98%) versus 1/52 (2%); spastic quadriparetic, 20/85 (24%) versus 65/85 (76%); spastic hemiplegic, 76/77 (99%) versus 1/77 (1%); dyskinetic, 4/16 (25%) versus 12/16 (75%); other (triplegic or ataxic-hypotonic), 10/13 (77%) versus 3/13 (23%). These distributions (proportions) all yielded significant (p<0.001) Pearson chi(2) values. INTERPRETATION: Neurological subtype is a powerful predictor of functional status related to ambulation. This has implications for counseling families.