Abnormal direct entry of the umbilical vein into the portal vein: report of a case

Abnormalities of the intra-abdominal umbilical venous connections are rare congenital vascular anomalies. We report a case of an aneurismal dilatation of an umbilical vein directly connected to the portal vein, vital for the delivery of placental blood in the fetus. A term female newborn presented with a dilated umbilical vein detected by prenatal ultrasound. CT angiography revealed an aneurismal dilatation of a vascular structure, from the umbilical ring to the main portal vein. Follow-up CT angiography showed thrombus progression up to the level of SMV inlet. A laparotomy revealed that the normal course of the umbilical vein was absent. The aneurysm was resected and no thrombus was noted in the aneurysm. Post-operative ultrasound revealed an isolated thrombus at the right main portal vein that resolved spontaneously on the follow-up ultrasound. At the 6-month post-operative follow-up, the baby was well and without complications. Although not evaluated for the presence of a ductus venosus, this anomalous umbilical-portal venous connection might have been associated with an absent ductus venosus.     

An extremely uncommon complication of ITP: spontaneous rupture of an ovarian follicle cyst and massive intra-abdominal bleeding

Idiopathic thrombocytopenic purpura usually presents with minor bleeding such as petechia and purpura. Rarely, life-threatening events as intracranial and intra-abdominal bleedings can be seen. We would like to present a rare case diagnosed as idiopathic thrombocytopenic purpura, presenting with abdominal pain and paleness. In this 17-year-old female patient, extensive abdominal sensitivity was revealed on physical examination and massive intra-abdominal hemorrhage secondary to distended ovarian follicle rupture was seen on ultrasonography and abdominal computed tomography. The case was treated successfully with intravenous immunoglobin, thrombocyte suspension, and pulse methylprednisolone.     

Fetal schistocytic hemolytic anemia and umbilical vein varix

A rare case of schistocytic hemolytic anemia presenting in a fetus secondary to a varix of the intra-abdominal umbilical vein is reported. A patient was referred to our hospital at 32 weeks of gestation because of an abnormal hypoechoic finding in the fetal liver. Prenatal ultrasound showed turbulent flow through a 12-mm diameter dilatation of the fetal intra-abdominal umbilical vein consistent with a varix. Cardiomegaly also was noted. At birth, the 1098-g, growth-retarded, male neonate was in severe congestive heart failure secondary to anemia as the initial hemoglobin was 5 g/dL. Additional evaluation found the anemia to be secondary to schistocytic hemolysis. After the neonate received a transfusion of packed erythrocytes and supportive care, the anemia quickly resolved, and he was discharged to home doing well after a 6-week stay in the neonatal intensive care unit. Prompt recognition of the varix prenatally and thorough evaluation of the newborn postnatally led to appropriate diagnosis and treatment.     

Two types of umbilical venous pulsations and outcome of high-risk pregnancy

Normally, blood flows evenly in the umbilical vein, without fluctuation. A pulsating pattern has been reported during fetal heart failure and asphyxia. Recently we have noticed two types of pulsating pattern; its relationship to adverse outcome is unclear.In a prospective multicenter study, recording of umbilical cord venous blood flow was conducted in high-risk pregnancies admitted for routine artery Doppler. In cases of pulsating flow or signs of vascular resistance in the umbilical artery, the examination was extended to the intra-abdominal part of the umbilical vein. Venous pulsation, single or double, were noted and correlated to perinatal outcome. Venous flow pulsatility was noted in 83 fetuses during 2 years, 26 had a double pulsating pattern, which was closely related to increased vascular resistance in the umbilical artery and perinatal mortality. A single pulsating venous pattern in one location had a good prognosis. In conclusion a double pulsating venous pattern, especially if extending to the cord, is an ominous finding in high-risk pregnancy associated with poor perinatal outcome. A single pulsating pattern predicted a much better outcome and might be an indication for delivery in the high-risk case.     

Large supraumbilical pseudocystic tumour due to ectopic pancreatic tissue located in a rest of the omphaloenteric duct.

Ectopic umbilical pancreatic tissue is extremely rare. We report on a case of a two-year-old boy who suffered from a large recurrent supraumbilical tumour with central cystic degeneration. Ectopic pancreatic tissue was located within the submucosal layer of an umbilical rest of the omphaloenteric duct. Peptic erosion and inflammatory alteration of tissue surrounding the umbilical vein caused recurrent bleeding and formation of a pseudocyst as well as chronical inflammatory granulations within the abdominal wall.     

Limitations and dangers of the Sengstaken-Blakemore tube in the treatment of hemorrhage from gastric varices

Gastric varices are frequent in children with portal hypertension, particularly in patients with portal vein thrombosis. The relation between the presence of gastric varices and the risk of bleeding has not yet been investigated; these are not taken into account when calculating the risk of bleeding according to Beppu's criteria. Gastric varices may be the site of a sudden hemorrhage, either primarily or after sclerotherapy of esophageal varices. Mechanical compression of bleeding esophageal varices by means of a Sengstaken-Blakemore tube is known to be effective in the few patients who fail to respond to intensive medical management. When the source of the bleeding is located on the fundal wall, far from the cardia, the inferior balloon of the Sengstaken-Blakemore tube has no chance of being effective. Furthermore, it may worsen the hemorrhage by a direct stripping effect on fundal varices following efforts to vomit or by enhancing the venous pressure gradient. The inappropriate employment of this tube may ultimately result in fatalities. For bleeding gastric varices, the Linton-Nachlas tube is more indicated. It is very important to understand its correct use as well its limitations. Offprint requests to: C. Previtera     

Factor XIII deficiency in a newborn]

Factor XIII deficiency is an uncommon inherited disorder which is characterized by umbilical cord bleeding and an unusually high incidence of intracranial hemorrhage. We report here a case of Factor XIII deficiency in a child that presented a caput. succedaneum as the first manifestation of the disease and then an umbilical cord bleeding. The importance of performing a quantitative FXIII assay in the presence of strong clinical suspicion is strengthened because of the normality of the standard screening tests and the important therapeutic consequences.     

Okkulte abdominale Blutung nach stumpfem Bauchtrauma

A relevant intra-abdominal hemorrhage was diagnosed in a 12-year-old boy after an abdominal trauma of unknown origin. In the initial ultrasound examination and in a laparatomy the bleeding source could not be identified. With contrast-enhanced ultrasound (CEUS) a splenic lesion could be shown. After multiple blood and plasma transfusions stabilization of the patient was possible without the need for surgical intervention. In selected cases the use of intravenous contrast agents in children after traumatic intra-abdominal bleeding can be useful even though pediatric approval is still currently lacking.     

Newborn with congenital arteriovenous fistulas of the abdominal wall complicated by a ductus arteriosus aneurysm

A case of congestive heart failure in a neonate resulting from a congenital abdominal arteriovenous fistula is described; an aneurysm of the ductus arteriosus was also found in the patient. Abdominal aortography was performed and demonstrated dilated feeding arteries, including the hypogastric arteries, which communicated with a dilated umbilical vein. Ligation of the feeding arteries and the umbilical vein led to resolution of the heart failure. Transient liver dysfunction occurred, however. Symptoms improved dramatically upon removal of the ligature from the umbilical vein. Although there is one previous report of an arteriovenous fistula involving the umbilical vein, we know of no prior report of a congenital arteriovenous fistula in association with a ductus arteriosus aneurysm.     

Peritoneal hemorrhage due to a ruptured ovarian cyst in ITP

Immune thrombocytopenic purpura in children rarely causes severe bleeding. The incidence of intracranial hemorrhage is approximately 0.2% to 1.0%, and severe bleeding (defined as persistent epistaxis, melena, menorrhagia, gastrointestinal bleeding, etc, requiring hospitalization or transfusion) occurs in only 5% of patients. Epstein-Barr virus (EBV) associated idiopathic thrombocytopenic purpura (ITP) tends to behave similarly to non-EBV - associated ITP with no increase in hemorrhagic complications and only a small increase in time to remission. Immune thrombocytopenic purpura diagnosed in adolescence is more likely to be chronic then in childhood ITP, but has a higher rate of spontaneous resolution than in adults. However, females in this age group are in their early childbearing years and present a unique set of possible hemorrhagic complications not seen in younger patients. We present the case of an 18-year-old female with EBV-associated ITP, who developed a severe intra-abdominal bleed secondary to a hemorrhagic ovarian cyst.     

Management of acute intra-abdominal hemorrhage in ruptured hepatoblastoma with transarterial embolization using calibrated gelfoam particles

This brief report aims to evaluate the treatment outcome of transarterial embolization in ruptured hepatoblastoma complicated with acute intra-abdominal hemorrhage. Three children (mean age 6 years) with high-risk hepatoblastoma presented with rupture and acute intra-abdominal hemorrhage. In addition to aggressive fluid resuscitation and blood product support, super-selective embolization of the arteries with active bleeding or pseudoaneurysm was performed using calibrated gelfoam particles, with a technical success rate of 100%. Hemodynamic status and hemoglobin level were normalized in all patients within 2 days postembolization. The 30-day survival rate was 100%. No major complication was detected apart from mild elevation of alanine transaminase.     

Oxidative stress and antioxidant status in fetal circulation in preeclampsia

Preeclampsia is associated with oxidative stress in maternal circulation. The purpose of this study was to explore oxidative stress and antioxidants in the fetal circulation in preeclampsia. Women with preeclampsia (n = 19) or uncomplicated pregnancies (n = 33) delivered by cesarean section were included. Blood was sampled separately from the umbilical vein and artery. 8-Iso-prostaglandin F(2alpha) (8-isoprostane), a stable product of lipid peroxidation, is a reliable marker of oxidative stress. Concentration of total 8-isoprostane in cord plasma was analyzed by gas chromatography-mass spectrometry. Antioxidant status was evaluated measuring ferric reducing ability of plasma and vitamin E. There was no difference between preeclampsia and control groups regarding median plasma concentration of 8-isoprostane in umbilical vein (955 versus 780 pg/mL, p = 0.41) or in umbilical artery (233 versus 276 pg/mL, p = 0.65). Concentration of 8-isoprostane was much higher in plasma from the umbilical vein than artery, suggesting placenta as the source of 8-isoprostane. Median ferric reducing ability of plasma concentration was higher in preeclampsia than in controls, both in the umbilical vein and artery. Median vitamin E concentration in the umbilical vein was higher in preeclampsia, but no difference was found in the umbilical artery. In conclusion, no evidence of increased oxidative stress, evaluated by 8-isoprostane concentration, was found in fetal circulation in preeclampsia.     

Gestational diabetes and its effect on the umbilical cord

The structural changes in the umbilical cord of full term fetuses from 18 cases of gestational diabetes were studied. All these were insulin-dependent and their blood glucose levels were well controlled. Light microscopy showed rupture and erosion of the endothelial lining of umbilical arteries resulting in increased permeability and haemorrhages. The umbilical vein was unduly dilated. The smooth muscle in the walls of these vessels showed disruption and degeneration of its fibres. 'Wharton's jelly' showed alteration in the pattern of distribution of its fibres with large empty spaces amongst them. It is suggested that gestational diabetes has a deleterious effect on umbilical vessels and the connective tissue component of 'Wharton's jelly'.     

Extrahepatic stenosis of the portal vein after infection or catheterization of the umbilical vein during the neonatal period (author's transl)]

147 children with high risk of developing a prehepatic portal vein obstruction were reexamined. The children had survived during their neonatal period:--Serious umbilical infection and sepsis (n=33)--Multiple exchange transfusion (n=55)--Long-term umbilical vein catheter treatment for at least 24 hrs (n=59). The reexamination at the age of 2--4 years revealed one case of total occlusion of the vena lienalis with partial obstruction of the vena portae in a child with former umbilical sepsis. Even though the etiologic relation of neonatal umbilical disorders and prehepatic portal obstruction is evident, the risk for the individual case seems to be low.     

Severe fetal anemia as a consequence of extra‐abdominal umbilical vein varix: A case report and review of the literature

Umbilical vein varix is associated with a high incidence of fetal anomalies and perinatal complications. There are two types of umbilical vein varix: fetal intra‐abdominal and extra‐abdominal. Herein, a case is reported of severe fetal anemia with extra‐abdominal umbilical vein varix. A 33‐year‐old primigravida was referred to our hospital for fetal growth restriction, fetal cardiomegaly, and decreased fetal movements at 26 weeks' gestation. A Doppler assessment showed an elevated middle cerebral artery peak systolic velocity at 2.2 MoM, suggesting fetal anemia. Umbilical vein varix had caused intermittent turbulent flow, provoking hemolytic anemia. Intrauterine transfusion improved fetal circulatory failure and anemia and prolonged gestational period. At 33 weeks' gestation, the patient underwent cesarean delivery due to nonreassuring fetal status. Pathological analysis revealed focal loss of vascular smooth muscle of the umbilical vein. Extra‐abdominal umbilical vein varix has been reported in 14 cases including this case. The antenatal diagnosis rate is reported to be 79%; fetal heartbeat abnormalities and fetal deaths were reported as 50% and 14%, respectively. Eighty‐six percent of patients had intra‐umbilical cord thrombosis, but currently this is the only case of hemolytic anemia. Furthermore, extra‐abdominal umbilical vein varix may present as fetal hydrops with anemia. During ultrasound examination of fetal anemia, umbilical cord screening should be performed with caution.     

小儿门静脉海绵样变治疗的思路

目的 探讨小儿门静脉海绵样变治疗的最佳术式。方法 对1990年～2002年收治的16例门静脉海绵样变的诊治过程进行回顾性分析。本组16例中13例因消化道出血入院，另3例因脾肿大、脾功能亢进人院。治疗方法有药物治疗，胃镜下注射硬化剂或套扎术，12例手术治疗。手术方式多种。7例行1次手术，3例行2次手术，1例行3次手术，1例行4次手术。结果 术后随访1～11年，3例仍有出血，非手术疗法可控制；4例经钡餐胃镜检查食道静脉曲张情况明显好转；4例B超下可测及吻合口的直径及血流速度；本组死亡3例，2例死于大出血、休克，1例死于术后败血症。结论 (1)小儿门静脉海绵样变所致的门脉高压症发病年龄早，消化道大出血为其主要症状，可危及生命。(2)治疗上应酌情选择降压效果明显、止血确切的手术方法。如未做过手术，脾大和脾亢不明显者，可首选肠腔分流；脾大和脾亢明显者在切脾同时作断流， 脾肾分流术；若已行脾肾分流术后仍出血，可选择肠腔分流。胃右静脉或胃冠状静脉与肝左静脉属支吻合法是一种新的门体分流术式，在条件允许时可试用。(3)对合并胆道疾病者，应先降低门脉压力、后行胆道手术。     

Ulcerative esophagitis. A rare source of upper gastrointestinal bleeding in a neonate. Use of fiberoptic endoscopy for diagnosis

A case of upper gastrointestinal hemorrhage in a newborn infant due to ulcerative esophagitis is described. This is a previously undescribed cause of upper gastrointestinal neonatal bleeding. This case further illustrates the safety and efficacy of fiberoptic endoscopy in elucidating the source of upper gastrointestinal bleeding in the immediate newborn period.     

The metabolic and endocrine milieu of the human fetus at 18-21 weeks of gestation. II. Blood glucose, lactate, pyruvate and ketone body concentrations

Blood levels of glucose, lactate, pyruvate and total ketone bodies were measured in 16 conscious mothers and their minimally stressed fetuses at 18-21 weeks gestation. Blood samples were taken from the maternal antecubital vein and from the fetal umbilical vein and artery during fetoscopy prior to termination of pregnancy. The mean concentrations of blood glucose, lactate and pyruvate were similar in maternal and fetal venous blood; no significant difference was found between the fetal umbilical artery and vein concentrations. Maternal total blood ketone body levels were significantly higher than fetal levels with no detectable umbilical venous arterial concentration difference. There was a significant inverse relationship between the umbilical artery levels and the umbilical venous-arterial concentration differences for blood glucose and lactate.     

Fetal central blood circulation in the third trimester of normal pregnancy--a longitudinal study. I. Aortic and umbilical blood flow

Fetal central blood circulation was evaluated in 21 uncomplicated pregnancies every other week from the 27th gestational week till term. Blood flow in the fetal descending thoracic and abdominal aorta and in the intra-abdominal umbilical vein was measured with a combined ultrasound real-time and 2 MHz pulsed Doppler technique. The mean fetal blood velocities were fairly constant at the three measuring sites during the last trimester: 34.6 cm X s-1 (S.D. 5.5), 32.7 cm X s-1 (S.D. 5.5) and 12.6 cm X s-1 (S.D. 3.1), respectively. The aortic diameter increased with gestational age, whereas the umbilical vein diameter increased until the 34th gestational week followed by a stagnation. The mean weight-related blood flow in the fetal thoracic descending aorta decreased slightly towards term (from 240.8 ml X min-1 X kg-1 (S.D. 53.6) in the 28th week to 212.6 ml X min-1 X kg-1 (S.D. 37.3). In the umbilical vein, the corresponding blood flow decrease was linear and more pronounced: from 138.7 ml X min-1 X kg-1 (S.D. 76.0) to 65.2 ml X min-1 X kg-1 (S.D. 14.2). The results indicate that the placental proportion of fetal blood flow decreases with gestational age.     

Aneurysmal dilatation of persistent vitelline vein with thrombus in a neonate

The paired vitelline veins selectively involute and form a part of the portal vein during embryonic development. The presence of a persistent vitelline vein segment after birth is very rare and can be confused with anomalies of the portal and umbilical veins. We present sonographic, CT and MRI findings of aneurysmal dilatation of a persistent vitelline vein with thrombus in a neonate; this case was first misdiagnosed as an umbilical vein varix by prenatal US. MRI was used to identify the persistent vitelline vein segment and the remnant umbilical vein segment.