Management of a painful or contracted hip dislocation in individuals with severe spastic quadriplegia is difficult. Clinical and radiographic results of 12 proximal femoral resection–interposition operations performed in seven non-ambulatory persons (five males, two females; mean age 14 years, 8 months; age range 6 years 11 months to 19 years 8 months) with severe spasticity were reviewed to determine if pain relief and restoration of motion were maintained. At a mean follow-up of 7 years 7 months (median 9 years 6 months) all participants maintained a good sitting position and a functional range of motion with improved hygiene. Hip pain was improved in all participants compared with their preoperative status. Proximal femur migration occurred causing slight pain in one person. Heterotopic ossification was observed but was not clinically significant. Complications included traction pin loosening and infection and a late supracondylar femur fracture 3 months after the operation. Proximal femoral resection effectively decreased pain and restored hip motion in those with severe spastic quadriplegia leading to improved sitting and perineal care.

Cervical accelerometry with digital signal processing (DSP) can identify signals that are consistently associated with swallowing during feeding of infants. It is shown that these signals, called initial discrete sounds (IDSs), become more uniform with advancing postmenstrual age (PMA) in healthy preterm infants. The objectives of this study were to determine if cervical accelerometry and DSP at a higher sample rate than previously used (22kHz versus 16kHz), in conjunction with a DSP software package that allows manipulation of the sound files, would improve the accuracy of the previously developed variance index (VI) method of analyzing accelerometric information. The modified VI method was then used to measure developmental differences in the IDS morphology of infants with and without bronchopulmonary dysplasia (BPD). VIs were calculated for 24 feeding studies of infants of between 32 and 39 weeks PMA: 12 studies on healthy preterm infants (n=10: three males, seven females; mean gestational age [GA] 28.6 weeks, SD 0.4; mean birthweight [BW] 1080g, SD 82; PMA mean 35.2 weeks, SD 0.6) and 12 studies on infants with BPD (n=7: five males, two females; GA 27.1 weeks, SD 0.4; BW 911g, SD 71; PMA 36.2 weeks, SD 0.6). There was a significant inverse correlation between VI and PMA for the healthy preterm group (r=0.66, m=–2.31/week, p=0.02). There was no significant correlation between VI and PMA for the BPD cohort. The VI of infants with BPD was significantly different from that of infants without BPD (p<0.007, multiple regression analysis, interaction PMA×Group). Additionally, using our modified VI technique, 100% of swallows were found to have the expected IDS signals.

This study documents the development of hand and upper-extremity function in young children who have cerebral palsy (CP) with upper-extremity involvement using longitudinal data. Assessments of hand function and the quality of upper-extremity movement were conducted on 29 males and 22 females (mean age 36.2 months, SD 10.6; age range 16 to 60 months at baseline) and on four other occasions over 10 months. Linear mixed effects modeling was used to estimate average developmental curves and the degree of individual differences in the patterns of development which were conditional on the body-site distribution of CP and severity of impairments. Results indicate that hand function in this clinical population develops differently from overall upper-extremity skills with declines in function in upper-extremity skills being more common and pronounced among older children. However, there is substantial interindividual variation. Distribution of CP and severity of impairments were significant predictors of development. Results are discussed in terms of their clinical implications.

Using a population-based register, this study sought to ascertain changes in the rate and severity of cerebral palsy (CP) in a geographically defined area of the UK among infants weighing less than 1500g and born between 1984 and 1995. There were 417414 live births in the area, which included Berkshire, Buckinghamshire, Northamptonshire, and Oxfordshire. Of the 898 children with CP (526 males, 372 females), 194 (21.6%) weighed less than 1500g at birth. The overall CP rate for neonatal survivors fell from 2.5 out of every 1000 in 1984 to 1986 to 1.7 in 1993 to 1995. The rate for those weighing less than 1000g rose to 90 out of every 1000 neonatal survivors in 1987 to 1989 and then fell to 57 in 1993 to 1995. A similar pattern is seen among infants weighing 1000 to 1499g at birth, the rate rising to 77 in 1987 to 1988 and then falling to 40 in 1993 to 1995. The rate of severe motor disability among infants weighing less than 1500g also decreased (24.6 in 1984–1986 to 12.5 in 1993–1995). The relation of these findings to changes in perinatal care in the early 1990s is not known.

This study examined the effect of low birthweight on school achievement and the mediating roles of cognitive and behavioural factors. The sample (115 females, 100 males) was selected from a longitudinal study of first-born singleton children, born between 1986 and 1988 of German-speaking parents, recruited from eight hospitals of the Rhine–Neckar region in Germany. Twenty-nine very-low-birthweight (VLBW; less than 1500g), 74 low-birthweight (LBW; 1500 to 2500g) and 112 normal-birthweight children (NBW; more than 2500g), all without severe neurological disability, were assessed at 11 years on cognitive, motor, scholastic, and behavioural measures. The scholastic measures included a school performance score and a teacher recommendation for type of secondary school the child should attend. LBW and VLBW children performed less well than the NBW group in all areas. LBW and VLBW groups remained at risk for school difficulties into late childhood, even when not neurologically impaired. Arithmetic, vocabulary, concentration, non-verbal intelligence, and attention problems were significant mediators of the effect of low birthweight on the school performance score. Arithmetic, vocabulary, motor skills, and attention problems were found to be mediating factors of birthweight on teacher's recommendations. The poor outcome of the low birthweight children could not be attributed to further obstetric risk factors. Parents and pediatricians should be made aware of specific long-term deficits of low birthweight children that may impair school performance, although they may be within a normal curriculum.

The purpose of this study was to explore O'Connor's four factors contributing to mothers' uncertainty concerning gastrostomy tube (G-tube) insertion in their children (lack of information; unclear value trade-offs; lack of support; social pressure) in a substitute decision-making context. Fifty mothers participated in one semi-structured interview at the time of their children's G-tube insertion. Children's ages ranged from 2 weeks to 17 years, slightly more than half were male, and most had a primary diagnosis related to a neurological (n=27) or cardiac (n=10) condition. Two-thirds of the mothers identified topics about which they wanted more information, the majority reported both gains and losses associated with their decision, three-quarters reported that they had received support during decision making, and half reported that they had felt pressure from family and health care professionals. Results indicate that mothers' decisions about G-tube insertion are complex and difficult. The existence and importance of O'Connor's factors in this context are confirmed by mothers' accounts. Because these factors are believed to be modifiable, health care professionals have the opportunity to potentially minimize the extent to which the factors contribute to decision uncertainly. It is recommended that health care professionals implement interventions focused on minimizing uncertainty.

Very little is known about childhood epilepsies in Bangladesh. This study was conducted within a national children's hospital in Dhaka city to provide baseline information on diagnosis and clinical outcomes of 151 children (98 males, 53 females, age range between 2 months to 15 years, median age of 3 years). Participants who presented with recurrent unprovoked seizures were followed up in an epilepsy clinic for at least 1 year. Of presenting families, 68.3% were from middle-income and lower-income groups. A history of perinatal asphyxia and neonatal seizures was present in 46.4% and 41.1% of participants respectively. Generalized, partial, and unclassifiable epilepsy were found in 63.6%, 25.2%, and 11.2% respectively. Severe outcome (malignant) epilepsy syndromes were diagnosed in 14.6%. Symptomatic epilepsy was found in 61%. Poor cognitive development was present in 72.8% and poor adaptive behaviour in 57%. Poor seizure remission occurred in 50.3%. Factors most predictive of poor seizure remission were: multiple types of seizures, poor cognition at presentation, high rates of seizures, associated motor disability, and EEG abnormalities. The study suggests that most children presenting at tertiary hospitals for seizure disorders come late and with associated neurodevelopmental morbidities. Specialized services are needed closer to their homes. The process for establishing early referral and comprehensive management of childhood epilepsies in Bangladesh requires further study.

CHARGE syndrome (coloboma, heart disease, atresia of the choanae, retarded growth and mental development, genital anomalies, and ear malformations and hearing loss) is a heterogeneous condition for which early prediction of intellectual outcome is important but difficult. The psychomotor milestones and intellectual outcome of a consecutive series of children with CHARGE syndrome who were observed by the same team from the neonatal period to the time of study were analyzed retrospectively. Twenty-one children (11 males and 10 females, aged from 5 to 12 years, mean 8 years 7 months, SD 2 years 5 months) were included. The influence of 19 early identifiable parameters that could be considered as deleterious for intellectual outcome was recorded. Generally, the main psychomotor milestones (0 to 4 years) were severely delayed, although intellectual outcome (at primary-school age) was satisfactory for half the children in this series. We show that extensive bilateral coloboma resulting in low vision, microcephaly, and brain malformation were the only three parameters that were predictive of poor intellectual outcome. Conversely, severe neonatal medical conditions, such as tracheotomy, conditions requiring long stays in hospital, or cardiac surgery were not predictive of poor intellectual outcome. Severe hearing loss was not found to be negatively correlated with intellectual outcome once coloboma had been taken into account.

This paper reports three females and two males with a distinctive congenital syndrome characterized by severe congenital hypotonia, facial diplegia, jaw ankylosis, velo-pharyngeal incoordination, pyramidal tract signs, and ocular motor apraxia. Patients were followed up at ages ranging from 20 months to 16 years. All cases of this syndrome are sporadic, without dysmorphological features, chromosomal, or MRI brain abnormalities. Electrophysiological studies indicate the brainstem as the site of the neurological dysfunction. Post-mortem CNS study of one of the patients demonstrated neuronal depletion of the IV, VII, VIII, and IX cranial nerve nuclei and intact morphology of the cerebral hemispheres. A vascular accident, early in foetal life, is the most likely cause of the clinical picture. The extent of brainstem involvement and its related clinical findings distinguishes these patients from those with Moebius, Pierre Robin, or Cogan syndromes. Outcome is better than what could be anticipated during the first few months of life given the severity of symptoms. Intelligence or developmental quotients are within the normal range for their age. Facial hypomimia, feeding, and speech articulatory performance difficulties are the main disabilities observed in these patients at follow-up.

Monozygous twin births have been associated with brain lesions such as hydranencephaly, multicystic encephalomalacia, and porencephaly. Prenatal circulatory injury has been considered to be the cause. Polymicrogyria is rare but has been reported in autopsied cases. The sibship in this case report, comprising monozygotic male twins and their elder sister from the same non-consanguineous parents, all had global developmental delay. Brain MRI showed polymicrogyria. We suggest that, apart from circulatory compromise, genetic etiology must be implicated as the cause of polymicrogyria.

Inflicted traumatic brain injury (ITBI) or shaken baby syndrome is recognized as a major cause of disability and death in the pediatric population. Although advances have been made in the recognition of the clinical, radiographic, and pathological findings of ITBI, less is known about the long-term outcomes of survivors. Health care providers recognize that these infants and children frequently have poor outcomes. Although an infant or child who sustains an ITBI may look well immediately after the trauma, that child may be left with serious and permanent disabilities.