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046
PRIMARY vs STAGED CLOSURE OF GIANT EXOMPHALOS - CARDIAC ANOMALIES
DO NOT AFFECT OUTCOME
1
1
1 ,2
1
1
Clare Rees , Lucinda Tullie , Agostino Pierro , Edward Kiely , Ignatius Joseph Curry , Kate
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2
1 ,2
Cross , Simon Eaton , Paolo De Coppi
1
2
Great Ormond Street Hospital for Children, London, UK, Institute of Child Health, UCL,
London, UK
Aim: to describe management of giant exomphalos and investigate the effect of congenital
cardiac anomalies on outcomes
Methods: single centre retrospective case note review (with audit approval) was performed of
neonates presenting with giant exomphalos (fascial defect ≥5cm ± liver herniation) over 10
years (2004-2014).
Demographic and operative data were collected and outcomes compared between infants
who had primary or staged closure. Congenital cardiac anomalies were considered significant
if requiring surgery or long-term follow up. Data, median (range) were analysed with Mann
Whitney-U test, t test and Fishers exact test as appropriate.
Results: 22 patients were included, 20 with liver herniation and 1 Pentalogy of Cantrell.
Gestational age was 38 weeks (30-40), birth weight 2.7kg (1.4-4.6); 13 (60%) were male.
Two were managed conservatively due to severe co-morbidities, 5 underwent primary closure
TM
and 15 had application of prolene mesh silo and serial reduction. Five (23%) died, including
2 managed conservatively (1 adenovirus, 1 CDH), 1 renal failure, 2 pulmonary hypertension.
None died primarily because of exomphalos. Survivors were followed up for 38 months (271).
Of patients managed with a staged approach, 13 (87%) had fascial closure, 2 were closed
with a patch. Five had other operations before definitive closure (laparotomy [2], exploration
of bowel in silo [1], silo reapplication [2]). 1/5 primary and 11/15 staged closure patients
developed ventral herniae (p=1).
Cardiac anomalies were present in 19 (86%), 7 had minor and 12 major anomalies. Twelve
(55%) had other anomalies, including 1 chromosomal anomaly. Outcomes are shown in
Table.
Conclusions: Infants with giant exomphalos whose defect can be closed primarily without
tension have a shorter length of stay but other outcomes are similar. Infants with more
significant abdomino-visceral disproportion can be managed with a silo and staged closure the presence of significant cardiac anomalies does not affect surgical outcome.