A prospective, national observational study to assess the incidence, comorbidities, management and outcomes of infants born with exomphalos in the UK and Ireland.
- The UK population of live-born infants with exomphalos has undergone significant selection from the point of prenatal diagnosis associated with the high frequency of concomitant chromosomal abnormalities, malformation syndromes and structural defects.
- Current information about the incidence of live-born infants with exomphalos and burden of comorbidity in this population is limited.
- Variation in practice exists where primary fascial closure is not possible.
- Population-based information is essential to provide a true picture of the prognosis of infants with exomphalos and the outcomes of varying management techniques.
- This study will provide a national picture of the incidence of live-born exomphalos, its associated abnormalities, management and outcomes.
1st March 2014 – 29th February 2016
Exomphalos is congenital abdominal wall defect characterised by herniation of the abdominal content through the umbilical ring. The exomphalos defect and the contents therein have a variety of structural variations, with a spectrum associated of clinical sequelae including pulmonary hypoplasia, distortion of hepatic venous drainage, intestinal non-rotation and bowel dilatation. Management of larger defects containing liver and those with substantial viscero-abdominal disproportion represent a significant challenge to paediatric surgeons.
Varying management strategies have evolved to deal with these so-called 'major' defects and such infants represent the most complex and challenging babies born with exomphalos from a surgical point of view. There remains a lack of consensus about the optimal management approach in such cases.
A prospective observational study of a contemporary cohort will provide information on the impact of prenatal selection on the population of live-born infants with exomphalos, their associated comorbidities and outcome. It will also generate key data on a large number of infants who have undergone differing management strategies and allow comparison of the outcomes between these methods. Such data could inform the development of future interventional studies.
To determine the number of infants live-born with exomphalos in the UK and Ireland, assess their burden of co-morbidities and describe their management and outcomes.
- What is the current incidence of live-born exomphalos in the UK and Ireland?
- How are these infants managed?
- What is the incidence of associated abnormalities in live-born infants?
- What factors are important in prognostication?
- What are the outcomes following different techniques of staged closure?
Any live-born infant with herniation of the abdominal content through the umbilical ring, the contents being covered by a membrane. This membrane may have been ruptured at the time of delivery.
Excluded: All infants with gastroschisis.
The study has been approved by the NRES Committee South Central-Oxford A (Ref:12/SC/0416)
Ms Anna-May Long, ST8 Paediatric Surgery; Professor Mark Davenport, Kings College Hospital; Professor Marian Knight, NPEU
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