The outcomes of pregnancies following a prenatal diagnosis of fetal exomphalos in Western Australia.
OBJECTIVE
To review the perinatal outcomes for prenatally diagnosed exomphalos from a single geographical region.


METHODS
Retrospective review of cases of prenatally identified exomphalos in the state of Western Australia in the ten-year period 1998-2007 using the medical databases of the sole tertiary obstetric and paediatric hospitals.


RESULTS
Ninety-four cases of prenatally identified exomphalos comprise this consecutive case series. Culture-proven karyotypic abnormalities occurred in 40 (42.6%) fetuses. No karyotypically abnormal fetus survived the neonatal period, with 33 of 40 (82.5%) pregnancies interrupted, five of 40 (12.5%) resulting in fetal demise and two (5%) neonatal deaths. For the 49 (52.1%) fetuses with a normal karyotype, 26 (53.1%) had associated abnormalities with termination occurring in 22 (84.6%). Prenatally isolated exomphalos was present in 23 cases (24.5%), with live birth in 15 cases (30.6% of euploid fetuses). Fourteen (93.3%) of the liveborn prenatally isolated exomphalos cases survived with no postoperative deaths, although four (28.5%) had significant abnormalities detected postdelivery and most have experienced childhood morbidity.


CONCLUSIONS
In the the majority of cases of prenatally detected exomphalos the pregnancy was interrupted secondary to chromosomal or structural abnormalities. In only 10.6% of prenatally recognised fetuses with exomphalos was the disorder truly isolated with neonatal survival occurring.
Calvert
Nick
N
School of Women's and Infants' Health, Faculty of Medicine, Dentistry and Health Sciences, The University of Western Australia, Western Australia, Australia.
Damiani
Sara
S
Sunario
Julian
J
Bower
Caroline
C
Dickinson
Jan E
JE
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000235:genetics; D002869:Chromosome Aberrations; D005260:Female; D006554:Hernia, Umbilical / Q000000981:diagnostic imaging* / Q000453:epidemiology / Q000235:genetics; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D011247:Pregnancy; D011256:Pregnancy Outcome; D011296:Prenatal Diagnosis / Q000379:methods*; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016216:Ultrasonography, Prenatal; D014904:Western Australia / Q000453:epidemiology