Gastro-intestinal function and quality of life are favorable in adolescent and adult gastroschisis patients.

BACKGROUND AND AIMS: Studies on long-term sequelae of gastroschisis are scarce. The limited data suggest increased abdominal complaints in young children. To provide proper counseling for both parents and patients, more information on long-term outcome is needed. This study aims to evaluate long-term outcome regarding GI function, gastroesophageal reflux (GER), health-related quality of life (HRQoL) and cosmetic satisfaction. METHODS: An observational longitudinal cohort study was performed. All patients (N = 43) born between 1982 and 2008 with gastroschisis that were admitted to the University Medical Centre Utrecht, Wilhelmina Children's Hospital were invited to fill in a survey. Data of included patients were compared to validated Dutch reference standards. RESULTS: Fourteen patients responded to the survey. The median follow-up was 18 years. Abdominal pain on weekly basis was present in two patients (14%) and feeding difficulties were present in one patient. Presence of a complication during gastroschisis treatment led to more GI symptoms (80% versus 22%). One patient experienced moderate complaints of regurgitation or dyspepsia. Although the overall HRQoL was lower in teenage gastroschisis patients as compared to healthy controls (73/100 versus 83/100, respectively), we found no relevant difference in overall HRQoL in the other age groups. Seven patients (50%) were satisfied with the cosmetic result of the scar. CONCLUSIONS: GI function and HRQoL in gastroschisis patients seem similar to healthy controls at adolescent and adult age. Complications during gastroschisis treatment led to an increase of abdominal complaints later in life. The surgical technique had no significant effect on the cosmetic results.

Factors Associated with Health-Related Quality of Life in Children with Intestinal Failure.

OBJECTIVE: To evaluate disease-specific and age-related factors contributing to health-related quality of life (HRQOL). in children with intestinal failure. STUDY DESIGN: A prospective study of HRQOL was performed in a regional intestinal rehabilitation program. Parent-proxy Pediatric Quality of Life Inventory surveys were administered annually to families of 91 children with intestinal failure over a 6-year period. Survey data was stratified by age and compared with pediatric HRQOL data in healthy and chronically ill populations. Linear mixed-effect models using multivariable regression were constructed to identify associations with HRQOL. RESULTS: A total of 180 surveys were completed by 91 children and their families. HRQOL scores were lowest for children ages 5-7 years (P < .001) and 8-12 years (P < .01), and these changes were primarily related to school dimension scores. In multivariable regression, age of 5 years and older and developmental delay were independently associated with lower HRQOL scores. The trend toward lower HRQOL scores parallels reference data from healthy and chronically ill children, although patients with intestinal failure scored lower than both populations at school age. CONCLUSIONS: Children with intestinal failure experience lower parent-proxy HRQOL scores in the 5-7 and 8-12 year age groups primarily related to school dimension scores. Multicenter data to validate these findings and identify interventions to improve QOL for children with intestinal failure are needed.

Gastroschisis at school age: what do parents report?

Children with gastroschisis are at high risk of morbidity in early life, which could affect long-term outcomes. We determined parent-reported outcomes in school-aged children born in 2000-2012, using paper questionnaires. Parent-perceived child vulnerability and motor function were compared with the Dutch reference data; parent-rated data on cognition, health status, quality of life, and behavior were compared with those of controls matched for age, gender, and maternal education level. Of 77 eligible participants, 31 (40%) returned the questionnaires. Parent-reported motor function was normal in 23 (74%) children. Total scores on health status, quality of life, and behavior did not differ significantly from those of matched controls. Children with gastroschisis had lower scores on cognition (median (interquartile range); 109 (87-127)) than their matched controls (124 (113-140); p = 0.04). Neonatal intestinal failure and increased parent-perceived vulnerability were associated with lower scores on cognition (ß - 25.66 (95% confidence interval - 49.41, - 1.91); - 2.76 (- 5.27, - 0.25), respectively).Conclusion: Parent-reported outcomes of school-aged children with gastroschisis were mainly reassuring. Clinicians and parents should be aware of the higher risk of cognitive problems, especially in those with neonatal intestinal failure or increased parent-perceived vulnerability. We recommend multidisciplinary follow-up at school age of children with gastroschisis and neonatal intestinal failure. What is Known: • Many infants with gastroschisis experience morbidity in early life. • Data on developmental outcomes and daily functioning in children with gastroschisis beyond the age of 5 years are scarce and conflicting. What is New: • Parents of school-aged children treated for gastroschisis report normal motor function, health status, quality of life, and behavior. • Children with gastroschisis, especially those with intestinal failure, may be at risk for cognitive problems at school age. Parents who reported their child as being more vulnerable also reported more cognitive problems at school age.

Short-term and family-reported long-term outcomes of simple versus complicated gastroschisis.

BACKGROUND: Our goal is to determine short- and long-term outcomes of simple gastroschisis (SG) and complicated gastroschisis (CG) patients including quality of life (QoL) measures, surgical reoperation rates, and residual gastrointestinal symptom burden. MATERIALS AND METHODS: Retrospective chart review of patients who underwent surgical repair of gastroschisis between January 1, 2009, and December 31, 2012, was performed at a quaternary children's hospital. Parent telephone surveys were conducted to collect information on subsequent operations and current health status as well as to assess QoL using two validated tools. RESULTS: Of 143 patients identified, 45 (31.5%) were reached and agreed to participate with a median follow-up age of 4.7 y. Although CG was associated with short-term outcomes such as longer length of stay, longer days to feeds, and higher complication rates, there were no major differences in long-term QoL outcomes when comparing SG and CG. Children with CG experienced abdominal pain/gas/diarrhea more often than those with SG and required more major abdominal procedures than those with SG (15% versus 0%, P = 0.009). CONCLUSIONS: Despite worse short-term outcomes, presence of certain gastrointestinal symptoms, and need for more surgical interventions for patients with CG, and overall QoL scores were reassuringly similar to those with SG.

Cognition and behaviour in children with congenital abdominal wall defects.

AIM: To characterise neurodevelopment at age two years and cognition and behaviour at age five years in children born with abdominal wall defects (gastroschisis or exomphalos). STUDY DESIGN: Participants were treated as neonates for gastroschisis or exomphalos and invited for routine clinical follow-up at ages two and five years. Thirty-nine two year-olds and 20 five year-olds with gastroschisis and 20 two year-olds and 10 five year-olds with exomphalos returned for age-appropriate assessments of development (two years) and intellectual functioning (IQ), executive function, and behavioural problems. Results were compared with normative data from the tests and published data from local term-born children. RESULTS: For both gastroschisis and exomphalos two year-olds, neurodevelopment was in line with the test normative data, but below the level of local normative data for all domains (effect sizes from -0.4 to -1.4 standard deviations). At five years, children with gastroschisis performed similarly to the normative mean for IQ but had high rates of various executive functioning problems on parent report (18-41% compared with 7% expected from norms). There was also a tendency for increased frequency of internalising problems (33% compared with normative expectation of 16%). Five year-olds with exomphalos also performed similarly to the normative mean for IQ and had low rates of executive and behavioural problems. CONCLUSIONS: Survivors of gastroschisis and exomphalos may be at risk of poor neurodevelopment in toddlerhood, depending on the reference group, and children with gastroschisis may be particularly at risk for executive functioning difficulties despite an IQ within normal limits.

Maternal Stressors and Social Support and Risks of Delivering Babies With Gastroschisis or Hypospadias.

We examined the association of maternal stressful life events and social support with risks of gastroschisis and hypospadias, using data from the National Birth Defects Prevention Study, a population-based case-control study of US births taking place in 2006-2011. We examined maternal self-reports of 7 life events and 3 sources of social support during the periconceptional period among mothers of 593 gastroschisis cases, 1,142 male hypospadias cases, and 4,399 nonmalformed controls. Responses to the questions on stressful life events were summed to form an index (higher is worse), as were responses to questions on social support (higher is better). We used logistic regression to estimate adjusted odds ratios and 95% confidence intervals. The adjusted odds ratios for gastroschisis for a 4-point increase in the stress index were 3.5 (95% confidence interval (CI): 2.6, 4.8) among nonteenage mothers (age ≥20 years) and 1.0 (95% CI: 0.5, 1.7) among teenage mothers (age <20 years). The odds ratio for hypospadias (among all mothers) was 0.8 (95% CI: 0.7, 1.1). Adjusted odds ratios for a social support score of 3 (versus 0) in the 3 respective groups were 0.6 (95% CI: 0.4, 1.0), 1.0 (95% CI: 0.5, 2.3), and 0.6 (95% CI: 0.4, 0.9). Given the lack of prior research on these outcomes and stress, results should be interpreted with caution.

Quality of life in patients with gastroschisis is comparable with the general population: A questionnaire survey.

AIM: To evaluate long-term quality of life and somatic growth of patients with gastroschisis and compare them with the general population. METHODS: We performed a questionnaire survey of the quality of life of our patients treated between 2004-2012. RESULTS: A questionnaire was sent to our 56 patients with gastroschisis, 38 mothers of patients (68%) responded to the questionnaire. 33 of 38 mothers claim that the quality of life of their child is very good, 4 of them responded that it is good. 1 mother confessed that the quality of life was very poor. Anthropometric data show comparable results with the standard population except for patients of 1 year of age who still have lower weight (P<0.001) and body height in the 5th percentile and patients of 3 years of age who are also significantly thinner. 13% of patients in our study group have gastrointestinal problems. 9 patients (24%) attend follow-up at the neurological center (Attention Deficit Hyperactivity Disorder n=6, mental retardation n=1, dysarthria n=2), however, overall intellectual abilities are within normal range. 7 patients underwent surgery for umbilical (n=3) or inguinal hernia (n=4), 2 patients were operated on for undescended testicles, 3 patients were operated on for an adhesive ileus. 92% of mothers are very satisfied with the cosmetic result of the scar. CONCLUSION: The study has shown that the majority of patients after operation of gastroschisis have a very good quality of life without limitation in comparison with the general population. The presented anthropometric data confirm that the development of patients with gastroschisis is favourable.

[Cognitive assessment in children who presented gastroschisis: a case-control study]. / Evaluacion cognitiva en niños que presentaron gastrosquisis: estudio de casos y controles.

INTRODUCTION: Gastroschisis is a malformation of the abdominal wall that is corrected by surgery and requires special hospital care, such as immobilisation, dietary restriction and separation from the mother for a variable period of time, among others. To analyse the possible cognitive repercussions, the Bayley III test was administered to 14 children. SUBJECTS AND METHODS: The study was conducted with seven cases (born with gastroschisis) and seven controls, in civil hospitals in Guadalajara, over the period January-April 2013. RESULTS: No evidence of retardation was found in the cases in the cognition, language and motor conduct assessments, whereas in those associated with socio-emotional and adaptive behaviour the scores favoured the control group. This shows that the latter have better adaptation and interaction strategies with respect to the cases group. CONCLUSIONS: Gastroschisis will not affect later neurodevelopment, at least in this group which presented only this abnormality and deficits that can be explained by their perinatal circumstances. Nevertheless, this prognosis cannot be generalised to other cases with a more severe clinical picture, a longer stay in hospital and associated comorbidity. In any case, it is important to inform the parents as of the prenatal stage about the expected consequences of this pathology.

TITLE: Evaluacion cognitiva en niños que presentaron gastrosquisis: estudio de casos y controles.Introduccion. La gastrosquisis es una malformacion de la pared abdominal que se corrige mediante cirugia y requiere cuidados hospitalarios especiales, como inmovilizacion, restriccion alimenticia y separacion de la madre durante tiempo variable, entre otros; para analizar las posibles repercusiones cognitivas se aplico la prueba de Bayley III a 14 niños. Sujetos y metodos. Siete casos (nacidos con gastrosquisis) y siete controles, en los hospitales civiles de Guadalajara, en el periodo enero-abril de 2013. Resultados. Los casos no evidenciaron retraso en las evaluaciones relacionadas con la cognicion, el lenguaje y la conducta motora, en tanto que en las asociadas a la conducta socioemocional y adaptativa las puntuaciones favorecieron al grupo control, lo que refleja que poseen mejores estrategias de adaptacion e interaccion respecto al grupo de casos. Conclusiones. La gastrosquisis no afectara el neurodesarrollo ulterior, por lo menos en este grupo que presento esta unica anormalidad y deficits explicables por sus circunstancias perinatales, sin que este pronostico pueda generalizarse a otros casos con un cuadro clinico mas grave, mayor duracion de la estancia hospitalaria y comorbilidad asociada. De cualquier forma, es importante informar a los padres desde la etapa prenatal respecto a las consecuencias esperadas de esta patologia.

Assessing quality of life in pediatric gastroschisis patients using the Pediatric Quality of Life Inventory survey: An institutional study.

BACKGROUND: The purpose of this study was to quantify quality of life (QOL) outcomes in gastroschisis children using a validated QOL inventory survey. METHODS: A chart review and prospective survey (2012 Pediatric Quality of Life Inventory™ (PedsQL™)) were performed for gastroschisis patients from 2005 to 2011. Demographics and scores were compared between patents with simple versus complicated gastroschisis and patients with and without bowel resection. RESULTS: One hundred nineteen patients (>2years of age) were identified. Twenty-eight families participated in the prospective survey with an average patient age of 5.8±2.3years. There were 11 complicated and 17 simple cases. Children with simple gastroschisis had lower rates of reoperation (0.06% versus 90%, p<0.001) and bowel resection (12% and 64%, p=0.004) than complicated patients. Average QOL scores for children with simple and complicated gastroschisis were 81.69/100±19.50 and 78.7/100±20.14 (p=0.70), respectively. Scores were also similar in children with and without bowel resection (74.72/100±19.94 and 83.29/100±19.10, p=0.171). Cronbach's alpha correlation was 0.912 for the overall survey. DISCUSSION: Despite increased need for reoperation and bowel resection, at >2years of age, PedsQL™ scores were similar between patients with simple and complicated gastroschisis. This study provides preliminary data on QOL outcomes for antenatal counseling of gastroschisis families.

The long-term neurodevelopmental and psychological outcomes of gastroschisis: A cohort study.

OBJECTIVES: Previous gastroschisis specific neurodevelopmental studies have focused on the first 3years of life. The aim of this study was to assess the intellectual, behavioral and neurological outcomes of older children and adolescents born with gastroschisis. STUDY DESIGN: Of 99 gastroschisis survivors born in Western Australia, 1992 to 2005, and who were at least 5years old, 42 agreed to take part in this study. The study assessed: intellectual ability, with age appropriate Wechsler intelligence scales; neurological status; hearing; vision; behavioral status with the Strengths and Difficulties Questionnaire (SDQ); and parenting style with the Parenting Relationship Questionnaire (PRQ). All results were compared to normative means. RESULTS: Median age at follow-up was 10years (range 5-17). No child had evidence of cerebral palsy or hearing loss; 1 child had amblyopia. Psychometric tests were completed in 39 children: mean full scale IQ was 98.2 (standard deviation [SD] 10.7); the working memory index was the only subscale to show a significant decrease from the normative mean (mean 95.5, SD 12.4, p=0.038). The mean SDQ behavioral scores were significantly lower for 3 of 5 domains and the Total Difficulties score. PRQ scores were significantly abnormal for 4 of 7 domains: Communication, Discipline, Satisfaction with School and Relational Frustration. CONCLUSIONS: Overall intellectual abilities were within a normal range. The decrease in working memory index and the behavioral and parenting relationship impairments could be an effect of perinatal factors, gastroschisis management and complications or the complexity of the socio-economic environment.

Growth and neurodevelopment at 16 to 24 months of age for infants born with gastroschisis.

OBJECTIVE: To examine growth, neurodevelopment and morbidity in infants with gastroschisis. STUDY DESIGN: We enrolled all infants with gastroschisis treated at the North Carolina Children's Hospital from March 2003 through June 2005. Neonatal data were collected. Medical history, growth and neurodevelopment were assessed at 16 to 24 months. RESULT: Of 24 infants, 17 completed follow-up. Weight and length were below the 10th percentile for five and six infants, respectively. Three infants scored less than 85 on the Bayley Scales of Infant Development, second edition. Small for gestational age (SGA) infants were smaller and had lower neurodevelopmental scores. Fourteen experienced continued bowel dysfunction; nine were rehospitalized. CONCLUSION: One-third of infants with gastroschisis experience growth delay. Infants who are SGA are at higher risk, suggesting that postnatal growth may be influenced by fetal phenomena, and may not be modifiable. Neurodevelopment is not delayed. Continued bowel dysfunction is common.