A J Holland1, W D Ford, R J Linke, M E Furness, C Hayward. 1. Department of Paediatric Surgery, Division of Surgery, Division of Medical Imaging, Women's and Children's Hospital, Adelaide, S.A., Australia.
Abstract
OBJECTIVE: To determine the influence of antenatal ultrasound on the management of exomphalos. METHODS: Retrospective case note review of 23 fetuses and infants referred to our institution with either a pre- or postnatal diagnosis of exomphalos over a 7-year period. RESULTS: There were 21 cases of exomphalos of which 18 were correctly diagnosed on antenatal ultrasound by 18 weeks' gestation. There were 2 false-positives and 3 false-negatives, including 1 case of amniotic band syndrome with an abdominal wall defect and 1 morphologically normal fetus. Associated anomalies were correctly identified in 12 but incorrectly reported in 8. Maternal serum alpha-fetoprotein levels were abnormal in 61% of cases of abdominal wall defects in this series. Amniocentesis was performed in 12 and cordocentesis in 1. There were 13 terminations, including 2 trisomy 18s and 1 trisomy 13. Two fetal deaths followed amniocentesis. Of the 10 live births, 9 had their exomphalos repaired with a 1-year survival rate of 89%. Prenatal diagnosis did not appear to influence outcome. CONCLUSIONS: Antenatal ultrasound diagnosed 86% of cases of exomphalos and correctly reported 67% of associated anomalies. Amniocentesis may have led to the death of 1 morphologically normal fetus.
OBJECTIVE: To determine the influence of antenatal ultrasound on the management of exomphalos. METHODS: Retrospective case note review of 23 fetuses and infants referred to our institution with either a pre- or postnatal diagnosis of exomphalos over a 7-year period. RESULTS: There were 21 cases of exomphalos of which 18 were correctly diagnosed on antenatal ultrasound by 18 weeks' gestation. There were 2 false-positives and 3 false-negatives, including 1 case of amniotic band syndrome with an abdominal wall defect and 1 morphologically normal fetus. Associated anomalies were correctly identified in 12 but incorrectly reported in 8. Maternal serum alpha-fetoprotein levels were abnormal in 61% of cases of abdominal wall defects in this series. Amniocentesis was performed in 12 and cordocentesis in 1. There were 13 terminations, including 2 trisomy 18s and 1 trisomy 13. Two fetal deaths followed amniocentesis. Of the 10 live births, 9 had their exomphalos repaired with a 1-year survival rate of 89%. Prenatal diagnosis did not appear to influence outcome. CONCLUSIONS: Antenatal ultrasound diagnosed 86% of cases of exomphalos and correctly reported 67% of associated anomalies. Amniocentesis may have led to the death of 1 morphologically normal fetus.