Short and long-term outcomes of children born with abdominal wall defects
Keywords: Neonatal Surgery
Exomphalos
Surgical outcomes
Abdominal wall defects
Gastroschisis
Paediatric Surgery
BAPS-CASS
Long-term outcomes

Background
Very occasionally, when a fetus is developing in the womb, problems occur with the normal processes controlling closure of the muscles of the abdominal wall and, as a result, some of the abdominal contents develop outside of the body. This is known as an abdominal wall defect. If the pregnancy continues to term, the newborn infant will need specialised surgical care. This situation occurs so infrequently that even a dedicated surgical centre will care for very few of these women and their babies in a year. Many centres have shared their experiences of managing these babies in the published literature but the majority of reports have included only a few infants. The focus of most previous studies has been to describe what happens to these newborn infants between birth and first discharge from hospital from a purely clinical perspective.
Aim
To explore methodologies to holistically understand the short and longer-term outcomes of children born with abdominal wall defects and to use the information to improve the care of future affected infants.
Methods
The quality of the published literature on short-term outcomes of children born with gastroschisis was scrutinised in a systematic review. The accompanying meta-analysis used published data as a means of identifying population outcome estimates.
Two national population-based cohort studies were undertaken, exploring the short-term outcomes of children born with exomphalos and the outcomes at seven to ten years of children born with gastroschisis. The latter study included an assessment of childhood outcomes from the point of view of the children themselves, along with their parents. Further parental perspectives on experiences of care were explored in a qualitative analysis of in-depth interviews with parents of children born with exomphalos.
Findings
Short-term outcomes of children born with gastroschisis have been published in a large number of small studies. Pooling the published data, where possible allowed the production of population estimates but heterogeneity between studies was marked. One in fourteen children born with gastroschisis died before their first birthday when managed in developed countries. Those who developed bowel complications in utero, had an increased risk of dying before one-year. The assessment of childhood outcomes for this latter group of children, who made up 11% of the population cohort, revealed a bleak outlook for many, of with one in three either dying or requiring complex surgery to gain allow them to be able to be fed via their gut, before their ninth birthday.
Due to methodological limitations, the extent of neurological and gastrointestinal morbidity among survivors in the cohort is unclear, but the findings of both the highly selected responses from the parent report and those of the clinical study provide enough concern to suggest that alternative methodologies need to be explored to identify the extent of ongoing sequelae as children grow older.
The live-born population of children with exomphalos is highly varied and a large burden of comorbidity was identified, however, two-thirds of infants were able to be have their abdominal wall defect surgically closed with a low-rate of early complications. A variety of techniques are employed by UK surgeons when the defect cannot be easily closed and evidence to guide management choice will be difficult to obtain using standard techniques due to the small number of these infants born annually in the UK. Parental experiences echoed the variability in management approach and in some cases highlighted a lack of respect for parental perspectives on management choice.
Conclusion
Children born with abdominal wall defects represent a spectrum from those with severe comorbidity who will need ongoing care, to those who have a straightforward course and a relatively short stay in hospital. Methods of risk-stratifying infants for the purposes of outcome assessment have been explored. This approach is crucial to contextualising the progress of an individual infant and counselling their parents about their likely prognosis.