OBJECTIVES: @entity299 is reported in 15-45% of omphalocele cases. Associated abnormalities of systemic veins have occasionally been reported in @entity1 and rarely documented in the fetus. We report a case series of interrupted @entity6323 (Int-IVC) in association with omphalocele and review the literature. METHODS: From our fetal database we identified all cases of omphalocele referred for fetal echocardiography (FE) between 1997 and 2012. We reviewed pre and postnatal medical records and performed a literature search from 1975 to present to identify previous relevant publications. RESULTS: Of 9627 fetuses referred for FE, 34 had an omphalocele. Gestational age at FE was 17(+6) to 26(+4)weeks. Seven of the 34 fetuses were shown to have an Int-IVC with azygos continuation to a right-sided superior vena cava ( @entity6480 ). The heart was structurally normal in all but one case. The abdominal wall defect was large and contained liver in all. There were three fetal demises and one @entity204 . Three cases were operated successfully. Since 1975, we identified 12 publications reporting omphalocele with @entity66 . @entity854 may lead to surgical complications. Failure of IVC formation is likely to be a developmental rather than a situs abnormality. Int-IVC with a dilated azygos influences venous access and may predispose to @entity49 . CONCLUSION: We have documented an association between large omphalocele and Int-IVC with azygos continuation to the @entity6480 . In this small series, this did not have surgical implications. It will however, influence route of any future cardiac catheterisation and may have long-term implications.

XXXX in fetuses with omphalocele. Case series of fetuses referred for fetal echocardiography and review of the literature.

@entity1 :: ('9606', 'Species') :: ['children'] @entity6480 :: ('696863', 'Species') :: ['SVC'] @entity66 :: ('MESH:D004194', 'Disease') :: ['systemic venous abnormalities'] @entity49 :: ('MESH:D019851', 'Disease') :: ['venous thrombosis'] @entity854 :: ('MESH:D018376', 'Disease') :: ['Abnormal IVC angulation'] @entity299 :: ('MESH:D006331', 'Disease') :: ['Congenital heart disease'] @entity6323 :: ('MESH:C563013', 'Disease') :: ['inferior vena cava'] @entity204 :: ('MESH:D003643', 'Disease') :: ['neonatal death']
@entity6323 :: (MESH:C563013,Disease) :: ['Interrupted inferior vena cava']
