1/9. Omphalocele and umbilical cord cyst. prenatal diagnosis.We present a case of intrauterine fetal death at 32 week's gestation with omphalocele, umbilical cord allantoic cyst and polyhydramnios. Ultrasound diagnosis of anomalies was performed at 23 weeks of gestation. Fetal karyotype was normal: 46 XX. This association has been found to have a high rate of chromosomal abnormalities, especially trisomy 18. It's difficult to explain the reason why intrauterine fetal death has happened; one possible hypothesis is that the cord cyst, compressing umbilical vessels, have caused intrauterine vascular compromise of blood flow.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) in triplet pregnancy after IVF and CVS.BACKGROUND: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare sporadic condition. CASE: We identified an infant with major malformations resembling OEIS. He was the product of a 30-week triplet pregnancy conceived by in vitro fertilization (IVF) and evaluated by chorionic villi sampling (CVS). In this article, we review the possible pathogenetic mechanisms in this case, including IVF, multiple gestation, trauma to the uterus or uterine vessels following CVS, and placenta accreta. CONCLUSIONS: We conclude that the cumulative effects of all or some of these factors may have resulted in uteroplacental insufficiency adequate to produce this phenotype. This case provides additional evidence for the uterine vascular pathogenesis of OEIS complex in humans.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/9. Acardiac twin with externalized intestine adherent to placenta: unusual manifestation of omphalocele.TRAP (twin reversed arterial perfusion) syndrome produces an acardiac twin (acardiac monster, acardius, acardiacus, chorioangiopagus parasiticus, etc.). Acardiacs result from monozygotic multiple births in which three anatomic anomalies occur: (1) a fetus' cardiac development is disturbed; (2) artery-artery anastomosis carries blood from a normal ("pump") twin to the acardiac; (3) vein-vein anastomosis carries blood from the acardiac back to the normal twin. Whether reversal of blood flow in the acardiac results from or causes cardiac dysmorphogenesis has not been resolved. Acardiac twins demonstrate a complex constellation of malformations usually thought to result from reversed blood flow; omphalocele is particularly common. We report monochorionic monoamnionic male twins in which an acardiac twin demonstrated externalized intestines adherent to the placenta. The twins were delivered from a 30-year-old primigravida mother by cesarean section without maternal complications at 33 w. The mother has no significant past medical history. The macerated acardius had a 4-cm long attenuated umbilical cord with indeterminate number of vessels. Structures rostral to the thorax were absent save for one poorly developed hand and arm. The abdomen contained loose mesenchyme and no organs. The entire intestine (21 cm) along with two testes was located in a sac on the surface of the placenta. No histopathologic anomalies of formed structures were identified aside from spatial relationships and incomplete development. The normal twin required no intensive care and is doing well. To our knowledge, this is the first report of externalized intestine, which may represent an unusual consequence of omphalocele.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/9. Portal hypertensive hemorrhage from a left gastroepiploic vein caput medusa in an adhesed umbilical hernia.Caput medusa is a frequent incidental finding in patients with portal hypertension that usually represents paraumbilical vein portosystemic collateral vessels draining into body wall systemic veins. A symptomatic caput medusa was seen in a morbidly obese patient after an umbilical hernia repair, which was fed not by the left portal vein but by the left gastroepiploic vein, in a recurrent adhesed umbilical hernia that likely contained herniated omentum. Refractory hemorrhage from this caput medusa was successfully treated by transjugular intrahepatic portosystemic shunt creation and balloon-occluded variceal sclerosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/9. Giant omphaloceles with a small abdominal defect: prenatal diagnosis and neonatal management.A giant omphalocele is a liver-containing protrusion through an abdominal defect wider than 5 cm in diameter. The giant form with a small abdominal wall defect is a rare condition which, to our knowledge, has not been described previously. We describe three cases with the typical features of elongated vascular liver pedicle and angiomatosis of the hepatic portal system. The abnormal liver organogenesis, due to extra-abdominal development, represented a significant risk factor for hepatic thrombosis after visceral reduction and liver rotation. All the neonates underwent surgery on the first day of postnatal life. One died because of a postoperative liver infarction, and the survivors needed prolonged respiratory support. Prenatal sonographic features, timing, delivery, type of surgical repair, and postnatal outcome are reviewed. A prenatal sonographic diagnosis could be useful to evaluate the abdominal ring and serial ultrasound examinations are recommended to detect promptly ominous signs of hepatic and bowel damage. color Doppler may be useful to assess the anatomy of the abdominal vessels and their relationships with the herniated organs, although it was not used in any of the cases reported here. This congenital malformation might be considered as a pathological entity separate from giant omphalocele with large abdominal defect, with a severe prognosis due possibly to its different embryological development.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/9. Sonographic features of an umbilical cord abnormality combining a cord pseudocyst and a small omphalocele; a case report.The perinatal findings of a pregnancy complicated by an umbilical cord abnormality associated with a small omphalocele are presented. The lesion was first observed at 18 weeks of gestation and was associated with elevated maternal serum alpha-fetoprotein. Serial ultrasound examinations showed major changes in the cord appearances and an associated small omphalocele was identified during the third trimester. Colour flow imaging showed no blood flow within the lesion and no obstruction nor involvement of the main umbilical vessels. Postnatal investigations demonstrated focal myxoid degeneration of the cord, a small omphalocele and no other fetal abnormalities.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/9. Congenital diverticulum of the left ventricle extending into exomphalos.Ventricular diverticulum in association with exomphalos is rare. It is usually accompanied by split sternum, anterior pericardial and diaphragmatic defects and intracardiac abnormalities. This paper reports a neonate who presented with exomphalos and was noted to have a pulsating vessel, on the superior margin of the sac, which proved to be a left ventricular diverticulum. The successful correction is described.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/9. Anomalous inferior vena cava in association with omphalocele: a case report.We present the case of a 6-year-old boy who had an omphalocele repaired at day 1 of life. He had a secundum atrial septal defect and an anomalous inferior vena cava of a type which has not been previously reported. Cine-MRI was a useful noninvasive tool for diagnosing the anomalous subaortic innominate vein and four immature vessels which make up the venous drainage systems of the lumbar region. The recognition of this malformation is important in planning and executing surgical repair or cardiac catheterization for postoperative patients with omphaloceles.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/9. Embolization of mesothelial cells in lymphatics: the route to mesothelial inclusions in lymph nodes?AIMS: To provide evidence that lymphatic embolization is the mechanism for mesothelial inclusions in lymph nodes. methods AND RESULTS: A 60-year-old man with alcoholic cirrhosis and ascites had an umbilical hernia resected. The herniorrhaphy specimen contained numerous dermal and submesothelial lymphatic vessels filled by cells similar to the cells that lined the hernia sac. Most of the cells in lymphatics were submesothelial reactive cells, whose cytoplasm stained with antibodies against cytokeratins (AE1-AE3; 8, 18), smooth muscle actin, vimentin, desmin and tissue polypeptide antigen (TPA). Some cells seemed to be superficial mesothelial cells, being positive with high molecular weight anticytokeratin antibody 34 beta E12. On ultrastructural study submesothelial cells with intermediate cytoplasmic filaments, rough endoplasmic reticulum and primitive cell junctions, and scanty superficial mesothelial cells with microvilli, tonofilaments and desmosomes were found in the lymphatics. CONCLUSIONS: Lymphatic dissemination of mesothelial and submesothelial cells is an uncommon and not well known phenomenon. Lymphatic dissemination is probably the route by which the mesothelial cells reach the lymphatic nodes. These cells may be mistaken for malignant cells.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |