Absence of the inferior vena cava in a patient with omphalocele: Two-dimensional echocardiographic and cineangiographic findings

We report an unusual form of absence of the inferior vena cava (IVC) in a patient with a repaired omphalocele. Two sets of bilateral paravertebral veins served as the channels of systemic venous return from the lower half of the body. These veins were narrower than typical azygos or hemiazygos continuation in the absence of the IVC, with the result that a catheter from the femoral vein could not reach the right atrium (RA). Other associated venous-side anomalies were present, including a chamber between the hepatic vein (HV) and RA, narrowing and angulation at the junction between the HV and the chamber, and a subaortic innominate vein. All these anomalies were demonstrated by two-dimensional and color Doppler echocardiography, The recognition of these venous anomalies is important for cardiac catheterization or IVC cannulation for cardiopulmonary bypass in patients with omphalocele.