Left-sided gallbladder with right-sided ligamentum teres hepatis: rare associated anomaly of exomphalos

PurposeThe aim of this investigation was to establish the association of left-sided gallbladder and right-sided ligamentum teres hepatis with exomphalos and to clarify the portal venous anomaly associated with right-sided ligamentum teres.MethodsThree male infants with exomphalos major associated with left-sided gallbladder and right-sided ligamentum teres hepatis were identified during a retrospective study of exomphalos cases (n = 35) in which exomphalos major comprised 18 cases over 19 years.ResultsThree infants with exomphalos major (8.6%) had a left-sided gallbladder and right-sided ligamentum teres hepatis. One male infant survived. Computed tomography showed that there is anomalous intrahepatic portal venous branching associated with right-sided ligamentum teres hepatis. The first branch of the portal vein ran to the right posterior segment, and then the portal vein formed a trunk of the left portal vein and right anterior portal veins. The latter vein formed the umbilical segment of the portal vein and finally joined the ligamentum teres hepatis. The inferior vena cava was on the left side below the kidney and crossed to the right side at the level of the kidney.ConclusionsLeft-sided gallbladder with right-sided ligamentum teres hepatis may not be such a rare associated anomaly in infants with exomphalos. However, there are no reports describing this anomaly with exomphalos in the English medical literature. We believe this is the first report. As long-term survival after repair of exomphalos is increasing, perhaps some of the survivors may develop liver disease which requires liver resection later in life. Recognition of this anomalous relationship with exomphalos is clinically important because it is associated with abnormal intrahepatic portal venous branching and intraabdominal vascular anomalies.