Gastroschisis is a full thickness abdominal wall defect, usually to the right of the umbilicus, through which a variable amount of viscera herniates, without a covering membrane. Newborns with gastroschisis present challenging problems to paediatric surgeons. The incidence of gastroschisis is rising worldwide. In developed countries, advances in neonatal intensive care have improved survival of patients with gastroschisis. In the few reported studies from Africa, mortality rates of patients with gastroschisis are high. The aim of this study was to evaluate outcome of gastroschisis from a centre in Africa with modern neonatal intensive care facilities. Methods: A retrospective analysis of all neonates admitted with the diagnosis of gastroschisis at Inkosi Albert Luthuli Central Hospital (IALCH) over a 6-year period (2002-2007). Proportions in percentages were used for categorical variables. For continuous variables the mean with standard deviation (SD) were derived. Two sampled t-test was used to show the pvalue for the time to reduction between the non-survivors and survivors with a 95% confidence interval. Results: There was a significant increase in the prevalence of gastroschisis among neonatal surgical admissions from 6.2% in 2003 to 15.2% in 2007. There were more females 53.4%, the majority (71.7% had low birth weight and 64.2% were born prematurely. Although 75% (n=79) of the mothers attended antenatal clinic, antenatal diagnosis by ultrasound was made in only 13 (n=12%)). Most of the babies 90.6% were out-born, with 70.8% delivered by normal vaginal delivery (NVD), and 57.4% of the mothers were primiparous. Primary closure was achieved in 73.5% of the patients. The overall mean (SD) time from birth to primary surgical intervention was 16 (13.04) hours and was higher 17(9.1) hours in those who died compared to survivors 15 (16.0), but the difference was not statistically significant, p=0.4465 and mortality was 43% with sepsis as the leading cause. Staged closure with a plastic silo bag was associated with more than double the mortality as compared to primary closure. Conclusion: The prevalence of gastroschisis among neonatal surgical admissions has increased in accordance with international trends. Due to lack of antenatal diagnosis, most of the babies were out-born resulting into delay in offering surgical treatment. Mortality is still high despite the presence of modern intensive care. / Thesis (M.Med.)-University of KwaZulu-Natal, Durban, 2008.

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.