W. Daniel Williamson

Cytomegalovirus urinary excretion and long term outcome in children with congenital cytomegalovirus infection.

Background. Cytomegalovirus (CMV) is the most frequent cause of congenital infection, and both symptomatic and asymptomatic infants may have long term sequelae. Children with congenital CMV infection are chronically infected and excrete CMV in the urine for prolonged periods. However, the effect of prolonged viral replication on the long term outcome of these children is unknown. Objective. To determine whether duration of CMV excretion is associated with outcome at 6 years of life in symptomatic and asymptomatic congenitally infected children. Methods. Longitudinal cohort study. Children congenitally infected with CMV were identified at birth and followed prospectively in a study of long term effects of congenital CMV infection. The relationship between duration of CMV urinary excretion and growth, neurodevelopment and presence and progression of sensorineural hearing loss (SNHL) at 6 years of age was determined. Results. There was no significant difference in the duration of viral urinary excretion between children born with asymptomatic (median, 4.55 years) and symptomatic (median, 2.97 years) congenital CMV infection (P = 0.11). Furthermore there was no association between long term growth or cognitive outcome and duration of viral excretion. However, a significantly greater proportion of children who excreted CMV for <4 years had SNHL and progressive SNHL compared with children with CMV excretion >4 years (P = 0.019, P = 0.009, respectively). Conclusions. Children congenitally infected with CMV are chronically infected for years, but the duration of CMV urinary excretion is not associated with abnormalities of growth, or neurodevelopmental deficits. However, SNHL and progressive SNHL were associated with a shorter duration of CMV excretion.

Children of Battered Women: Developmental and Learning Profiles

Children in battered womens shelters have been shown to have more behavior problems than their peers but limited information is available about their development. A pilot survey was undertaken to determine the prevalence of developmental or academic problems in children of residents of a battered womens shelter. Demographic data, medical and school histories and responses to standardized developmental or behavioral surveys were obtained from 39 mothers of 76 children. Two-thirds of the children were victims of abuse. On the Minnesota Child Development Inventory, the mean General Developmental Quotient (DQ) of 28 preschool children was 98; however, 39% had developmental delays by test criteria. Of 46 school-aged children, 21 (46%) had evidence of academic problems, including grade repetition, failing grades and need for special educational services. On the Louisville Behavior Checklist, 75% of 48 children had behavior problems. Children in a battered womens shelter are likely to experience academic and behavioral problems; however, further study is needed to elucidate etiological factors.

Early neurodevelopmental outcome of low birth weight infants surviving neonatal intraventricular hemorrhage

Neonatal intraventricular hemorrhage has emerged äs one of the most important and frequently encountered lesions in the central nervous System of the prematurely born infant [1]. Intraventricular hemorrhage is known to contribute significantly to neonatal mortality and morbidity. However, information regarding its effects on the long-term outcome of survivors is limited. This report describes the early neurodevelopmental Status of twenty-eight low birth weight infants surviving symptomatic neonatal intraventricular hemorrhage documented by computerized tomography and relates outcome to gestational age and grade of hemorrhage based on severity and location [2].

Cerebellar hemorrhage in the term neonate: Developmental and neurologic outcome

To elucidate the effects of cerebellar hemorrhage on the term neonate, neurodevelopmental assessments were conducted at a mean age of 32 months on six children. In addition to cerebellar hemorrhage, ventriculomegaly was present on each subjects initial computed tomographic scan. All were managed without surgical evacuation. Two patients required shunts for progressive ventriculomegaly. Five patients had follow-up computed tomography indicating mild atrophy of the superior anterior vermis of the cerebellum; however, none had abnormal ventricular size or abnormalities of the cerebrum. On detailed examination conducted between the ages of 18 and 48 months, five had hypotonia, truncal ataxia, and intention tremor; two had nystagmus. Only one patient walked independently. Intellectual performance of four patients was within the retarded range and two had mildly delayed development. Two patients had markedly disordered expressive language. These data suggest that term neonates surviving cerebellar hemorrhage have neurologic deficits related to the site of hemorrhage, and cognitive deficits related to more generalized cerebral insult.

Visually Impaired Infants in the 1980s A Survey of Etiologic Factors and Additional Handicapping Conditions in a School Population

To identify etiologic factors and ophthalmologic diagnoses related to visual impairment in young infants, a survey was carried out within the 22 school districts providing educational services to all visually impaired infants, birth to 3 years of age, in Harris County, Texas. Review of 102 records revealed that 51 percent had impaired vision associated with prenatal factors, and 31 percent were associated with perinatal events. The most frequent ophthalmologic diagnoses were lesions of the optic nerve, optic pathway, and visual centers of the brain (cortical blindness). A majority of the infants had multiple handicaps: 17 percent had hearing loss, 48 percent had cerebral palsy, 46 percent had seizures, and 78 percent had severe developmental delay. The high incidence of multiple handicaps and significant medical problems indicates the necessity for interface between physicians and educators who serve the visually impaired infant.

Congenital infections: Clinical outcome and educational implications

More than 40,000 neonates with congenital infections are born each year in the United States. The most frequently occurring congenital infections include toxoplasmosis, rubella (German measles), cytomegalovirus, herpes, and syphilis. Each of these five prenatal infections may leave significant developmental, neurologic, and sensory deficits, causing infected infants to need early intervention services. This review provides a clinical summary and a description of methods of transmission and prevention for each of these congenital infections.

66 THE DEVELOPMENTAL PROFILE OF THE NON-HANDICAPPED VERY LOW BIRTH WEIGHT (VLBW) INFANT AT 1 YEAR OF AGE

Information about general outcome of VLBW infants (i.e. those with retardation, C.P., blindness or deafness vs. those without major handicaps) is readily available. Yet, descriptions of the developmental profile of the non-handicapped VLBW infant are limited. Do VLBW infants without major handicaps exhibit uneven development at any early age? This question was addressed by studying 61 VLBW infants (followed prospectively to a mean age of 34 mo.) who met the following criteria: 1) normal cognitive functioning (group mean 92±19) 2) absence of major motor or sensory impairment 3) assessment on the Revised Gesell Developmental Schedule (GDS) at 1 year of age (mean chronologic age of 53±5 wks, mean corrected age 42±5 wks). Mean birth weight was 1082±221 gms; mean gestational age was 28.9±2 wks. The following is the developmental profile on the GDS (quotient of performance age ÷ corrected age):Both fine motor and language quotients were significantly lower than adaptive quotient (Students t test for matched pairs; t=8.35, 9.21; p<.001). 36 infants had ≥4 wk difference between fine motor and adaptive performance; for language performance, 32 had ≥4 wk difference. This study reveals that a majority of non-handicapped VLBW infants have significant deficits in fine motor and/or language skills at an early age. Longitudinal follow-up will reveal whether these weaknesses persist to school age, when such skills become major determinants of academic success.

ETIOLOGIC FACTORS OF VISUAL IMPAIRMENT IN INFANTS, 0–3 YEARS

To identify etiologic factors related to major visual impairment in young children, a survey was done of 22 school districts providing educational services to visually impaired infants, birth to 3 years, in Harris County. Review of 102 records yielded the following:17% had hearing loss, 49% cerebral palsy, 47% seizures (½ with infantile spasms). D.Q. was <50 in 78% of those tested. These data indicate that perinatal or postnatal CNS insult are major causes of visual impairment at 0-3 years. The high incidence of multiple handicap and significant medical problems indicate a necessity for interface between pediatrics and education in management of the young, visually impaired child.

CEREBELLAR HEMORRHAGE: DEVELOPMENTAL & NEUROLOGIC OUTCOME

In an effort to elucidate the long-term effects of neonatal cerebellar hemorrhage, 6 children with this lesion documented on CT scan were assessed neurodevelopmentally. All were managed conservatively without surgical evacuation of the hematoma. Perinatal data and developmental performance (on the Gesell, Bayley or McCarthy) are shown below:Neurologic findings included mild to moderate hypotonia (5), ataxia (4), titubation (2), dysmetria and/or tremor (4). Of the 5 children above 1 year of age, only 1 ambulated without aids. In general, motor skills were more severely impaired than cognitive functioning. These data suggest that children surviving cerebellar hemorrhage not only have neurologic deficits related to the site of hemorrhage, but cognitive deficits, related to a more generalized cerebral insult. Follow-up is indicated to clarify ultimate intellectual and neurologic functioning.