Exomphalos in the West of Scotland (1987–2002)

Abstract

Exomphalos is seen in approximately 3/10000 deliveries. Prognosis is related to the size of the defect and whether chromosomal or other structural abnormalities are present. Information regarding obstetric and paediatric outcomes for those pregnancies affected with exomphalos is essential in order that patients can receive accurate counselling. Ultrasound diaries, perinatal meeting lists, pathology reports, neonatal and paediatric admission records were used to identify cases of exomphalos from July 1987 to July 2002. The obstetric and paediatric case notes were then reviewed. Sixty per cent of cases of exomphalos were detected antenatally. Karyotype was obtained in 41%, and in 36% of these cases an abnormal karyotype was identified; 67% of cases were isolated lesions; 67% of women underwent termination of pregnancy; 33% women elected to continue with their pregnancy. Of these 65% had a live birth and 35% had an intrauterine death or miscarriage. In total 26% cases of exomphalos resulted in a live birth. Eighty-four per cent of these infants survived beyond 1 year.