Hirschsprung's disease: functional and psychological follow up comparing total colonic and rectosigmoid aganglionosis.

AIMS: To compare the long term functional and psychosocial outcomes following surgical treatment for total colonic aganglionosis (TCA) with those in an age and gender matched group of patients with rectosigmoid aganglionosis (RSA). METHODS: Fifteen patients with TCA matched for age and gender with 15 patients with RSA were studied 7-17 years after the definitive operation. The internal and external sphincters were examined using anal endosonography. Functional outcome (faecal continence) was assessed by a surgeon not involved in the patients' care, and by a research psychologist in separate assessment sessions. The behavioural and emotional status of the patients was also assessed. RESULTS: Based on the surgeon's assessment, 6/15 TCA and 7/15 RSA patients were continent. In comparison, based on the psychological interview, 2/15 TCA and 6/15 RSA patients were continent. The TCA patients reported significantly more behavioural/emotional problems and lower levels of self esteem than the RSA patients. The parent and teacher assessments of psychosocial status revealed no differences between the groups. There was no association between incontinence and psychosocial adjustment in either group. There was no association between the assessments of functional outcome and the endosonographic appearance of the anal sphincters. CONCLUSIONS: The proportion of patients with faecal incontinence 7-17 years after definitive surgery was high in both groups, but no association was found between incontinence and the psychosocial outcome measures. TCA patients perceived themselves as less well adjusted than their matched pairs. Differences between the groups in length of hospitalisation and severity of illness, especially in infancy and early childhood, may account for these differences.

Educational attainments in early adolescence of infants who required major neonatal surgery.

PURPOSE: The aim of this study was to assess long-term educational attainment in adolescence of former infants who required major neonatal surgery. METHODS: The study is an extension of a prospective longitudinal study begun in 1983 on 30 full-term newborns requiring major neonatal surgery, a matched healthy control group, and full-term infants requiring neonatal intensive care for medical reasons. Educational attainment levels were obtained from the results of compulsory national curriculum examinations taken at age 11 years in 3 core academic subjects, English, Mathematics, and Science and teachers' assessments of current academic performance. Additionally, follow-up data on health were obtained from family general practitioners and parents. RESULTS: Seventy percent of the original surgical group, 48% of the original control group, and 77% of the original medical group underwent follow-up. The participants were aged between 11 and 13 years. After adjustment for social factors (gender, social group, and mother's age), the surgical group was significantly behind on all measures of educational attainment (English, P < .0005; Mathematics, P < .02; Science, P < .0005; academic performance, P = .004), compared with the control group and medical group who did not differ from each other. Independent predictors of outcome were mechanical ventilation for >or=4 days in the neonatal period and behavior problems at 3 years. At 12 months of age, independent predictors of cognitive functioning were length of hospitalization and, at 3 years of age, the number of operative procedures. Mechanical ventilation was not significantly associated with cognitive functioning at previous stages of the study. CONCLUSIONS: These results show that, in early adolescence, children who required major neonatal surgery were performing less well academically compared with their peers. This information can be used to increase awareness of the difficulties these children may experience during childhood so that intervention can be directed appropriately, thereby lessening the risk of later educational problems. J Pediatr Surg 36:858-862.

Coping strategies of children with faecal incontinence.

As part of a study concerning the psychosocial adjustment of 160 children treated for anorectal anomalies, the authors investigated the ways in which the children coped with faecal incontinence (FIC). At the time of assessment, the children were 6 to 18 years of age. Portions of the in-depth interviews with the children and their parents covered questions about methods of managing and coping with FIC at home, socially, and at school. In addition, information was obtained about child and family characteristics that have been shown to contribute to the ability to adapt to chronic health problems. The ways in which the children dealt with their problems could be grouped into three distinct phases and were different for boys and girls. In phase 1, around 6 to 7 years of age, boys were largely unaware of the unsocial nature of their condition; the girls were sensitive and withdrawn. In phase 2, between 8 and 11 years of age, boys used overt denial, girls used secretiveness. Phase 3, from around 12 years into adolescence, for both sexes was marked by continued covert denial and eventual acceptance of their disability. The coping strategies reflected a complex interrelationship between characteristics of the child, the family, the social environment, and the unsocial and embarrassing nature of FIC. The findings showed that coping with FIC has potentially severely disruptive implications for the overall development of the child.

Psychosocial adjustment of children treated for anorectal anomalies.

The psychosocial adjustment of 160 children with anorectal malformations was assessed at 6 to 17 years of age in relation to levels of continence (Kelly score). Five measures of emotional and behavioural adjustment were used to assess a number of domains of child/adolescent functioning and to include measures from multiple perspectives. The psychiatric assessment of the child identified a disorder in 29% of the group overall. Based on parental assessments, behavioural maladjustment was shown in 27% of the children, and on the basis of a self-report questionnaire 24% of the children were depressed. Behavioural adjustment as rated by teachers was similar to the norms. The level of continence achieved (total Kelly score) did not appear to influence psychological adjustment, with the exception of the incontinent young girls (6 to 11 years) who were shown to be less well adjusted than the continent young girls. Differences between children showing positive versus negative adjustment were dependent on the perspective of the respondent and were also related to the child's age and gender, age of achieving continence, frequency of accidents, and parental factors.

Social and emotional impact of faecal incontinence after surgery for anorectal abnormalities.

A significant proportion of children with anorectal malformations have long term problems with faecal continence. The psychological consequences of this chronic disability was assessed in 160 children and adolescents. The prevalence of clinically significant emotional problems among the sample overall, as assessed by a diagnostic psychiatric interview (19%), parental assessment (27%), and child self report depressive scale (24%) was higher than expected relative to normative populations. With the exception of the young girls (6-11 years), the incontinent children and adolescents were not judged to be less well adjusted than those with good bowel control. Treatment for anorectal malformations appears to be associated with an increased risk for behavioural and social problems, but this was not related to the level of continence. Parental factors and gender were significantly associated with outcome. These children and families would benefit from psychological evaluation and support especially during early childhood.

Intellectual development at 3 years of age of children who underwent major neonatal surgery.

Thirty full-term infants born with surgically correctable life-threatening abnormalities who underwent emergency neonatal surgery were followed up from birth to 3 years of age. Comparable data were obtained on a matched group of healthy newborn babies. At 3 years the cognitive functioning of children whose condition had been resolved in the early months of life was similar to the controls. Those children who required further medical or surgical treatment were functioning at lower levels than the controls, with language development being most affected. Number of operations, defined as all procedures under general anesthetic, was the medical factor most strongly associated with poorer outcome at 3 years. Family factors--higher maternal IQ and nonmanual social class--were also positively correlated with language development.

Developmental progress of newborns undergoing neonatal surgery.

The development of 30 full-term infants undergoing surgery soon after birth was compared with a matched group of 29 healthy newborn infants in a prospective longitudinal study. At 1 year of age the infants who underwent surgery were performing within the normal range, but significantly less well than the controls in almost all areas of development. Of the various neonatal and perinatal factors studied, the length of hospital admission was the one most strongly associated with developmental progress at 1 year of age.

Factors associated with developmental progress of full term neonates who required intensive care.

The development of 43 infants born at full term, who were admitted to neonatal intensive care units shortly after birth, was compared in a prospective longitudinal study with that of a group of 29 healthy newborn babies. Thirty infants required emergency operations during the neonatal period, and 13 were admitted for medical reasons. The effects of being in hospital and being separated from their mothers were also studied. At 1 year the 'sick' babies were performing significantly less well in almost all areas of development. In the group of sick infants, the mothers' mental health explained 25% of the variance in developmental outcome at 6 months. At 1 year the most important predictor was length of stay in hospital, which explained 35% of the variance.