ABSTRACT
Cantrell syndrome is a rare condition of congenital defects of 5 developmentally associated structures: the abdominal wall, sternum, diaphragm, pericardium, and heart. Few patients survive, and even fewer survive with good outcomes of quality of life. We present a case with the pentalogy and profound cardiac malformations. Despite repeated life-threatening conditions, the boy survived to have his first operation at 5 months old. The operation reduced the heart within the thoracoabdominal area, repaired the abdominal wall defect, and released multiple stenoses of the heart and great vessels. The patient recovered uneventfully. At 4.5 years old, he is healthy and developed normally.
Subject(s)
Heart Defects, Congenital/pathology , Pentalogy of Cantrell/pathology , Abdominal Wall/surgery , Cardiac Surgical Procedures , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/surgery , Hernia, Umbilical/surgery , Humans , Infant, Newborn , Infant, Small for Gestational Age , Male , Pentalogy of Cantrell/surgery , Quality of Life , Radiography , Reoperation , UltrasonographyABSTRACT
Objective To study the value of DSA for hepatic vascular anatomy,and to evaluate the efficacy of portal vein occlusion in rabbits with hepatic VX2 tumor.Methods Twenty New Zealand white rabbits were randomly divided into two groups with 10 in each group,including test group A and positive control group B of ham operation.For the test group A,portal branch ligation(PBL)was performed for the left external branch after 3 weeks of the tumor implantation to the left external lobe.Two weeks later,the DSA of hepatic artery and portal vein were performed in all of the rabbits.Results The total displaying effectiveness of the branches of hepatic artery by DSA was better than that by vascular perfusion.There was hypovascular blood supply to hepatic artery implantation of the tumor in the test group A,comparing with that of the group B.Conclusion DSA can clearly display spacial details of the hepatic vascular anatomy in rabbits,and play an important role in post-procedual evaluation of the portal vein occlusion in rabbits.