Normal Fetal Umbilical, Portal, and Hepatic Venous System: Four-dimensional STIC Rendering.

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Human liver segments: role of cryptic liver lobes and vascular physiology in the development of liver veins and left-right asymmetry.

Couinaud based his well-known subdivision of the liver into (surgical) segments on the branching order of portal veins and the location of hepatic veins. However, both segment boundaries and number remain controversial due to an incomplete understanding of the role of liver lobes and vascular physiology on hepatic venous development. Human embryonic livers (5-10 weeks of development) were visualized with Amira 3D-reconstruction and Cinema 4D-remodeling software. Starting at 5 weeks, the portal and umbilical veins sprouted portal-vein branches that, at 6.5 weeks, had been pruned to 3 main branches in the right hemi-liver, whereas all (>10) persisted in the left hemi-liver. The asymmetric branching pattern of the umbilical vein resembled that of a "distributing" vessel, whereas the more symmetric branching of the portal trunk resembled a "delivering" vessel. At 6 weeks, 3-4 main hepatic-vein outlets drained into the inferior caval vein, of which that draining the caudate lobe formed the intrahepatic portion of the caval vein. More peripherally, 5-6 major tributaries drained both dorsolateral regions and the left and right ventromedial regions, implying a "crypto-lobar" distribution. Lobar boundaries, even in non-lobated human livers, and functional vascular requirements account for the predictable topography and branching pattern of the liver veins, respectively.

Drainage of the Left Hepatic Vein into the Coronary Sinus, a Rare Intraoperative Finding.

In a 76-year-old female undergoing coronary artery bypass surgery, a persistent left hepatic vein was observed. Draining of this vein into the coronary sinus is an extremely rare embryological malformation and this is the first case to be reported as a solitary malformation in absence of other cardiac malformations.

Ultrasound of the fetal veins part 1: the intrahepatic venous system.

Advances in high-resolution ultrasound combined with color Doppler and three-dimensional (3 D) rendering have contributed to an increasing understanding of the fetal venous circulation in recent years. Still the sonographic evaluation of the venous system in the fetus remains difficult. This article reviews the normal and abnormal intrahepatic venous system. Normal anatomy and abnormal findings of the umbilical vein (UV), the ductus venosus (DV), portal veins, hepatic veins and the inferior vena cava are demonstrated by grayscale, color Doppler and 3D ultrasound and explained by numerous schemes. Typical variants and abnormalities such as agenesis of the DV and portal venous system, persistence of the right UV, UV varix are explained and the clinical value of normal and abnormal Doppler findings in the DV is discussed. Many of these abnormal findings can be detected by a targeted examination of the intrahepatic vasculature with means of color Doppler.

Evolution of congenital malformations of the umbilical-portal-hepatic venous system.

OBJECTIVE: The objective of this study is to describe the evolution of 8 cases of congenital malformations of the umbilical-portal-hepatic venous system diagnosed before the first month of life. MATERIALS AND METHODS: All cases of congenital malformation of the portal and hepatic venous system diagnosed prenatally or during the first month of life in our institution were systematically reviewed since November 2000. Clinical features, imaging, and anatomical findings were reviewed, focusing primarily on clinical and radiologic evolution. RESULTS: Eight cases of congenital malformation of the umbilical-portal-hepatic venous system were studied. Fifty percent of these malformations were diagnosed prenatally. We report 4 portosystemic shunts. Three involuted spontaneously, and the fourth one required surgical treatment. We report a variation of the usual anatomy of portal and hepatic veins that remained asymptomatic, an aneurysmal dilatation of a vitelline vein causing portal vein thrombosis that needed prompt surgical treatment with good result, a complex portal and hepatic venous malformation treated operatively, and a persistent right umbilical vein that remained asymptomatic. CONCLUSION: Prenatal diagnosis of malformations of the umbilical-portal-hepatic venous network is uncommon. Little is known about the postnatal prognosis. Clinical, biologic, and radiologic follow-up by ultrasonography is essential to distinguish pathologic situations from normal anatomical variants.

Congenital portosystemic vascular malformations.

Congenital portosystemic shunts are developmental abnormalities of the portal venous system resulting in the diversion of portal blood away from the liver to the systemic venous system. Such malformations are believed to come from an insult occurring between the fourth and eighth week of gestation during the development of hepatic and systemic venous systems, and could explain their frequent association with cardiac and other vascular anomalies. They are currently categorized into end-to-side shunts (type I) or side-to-side shunts (type II). This article aims to review the common symptoms and complications encountered in congenital portosystemic shunts, the surgical and endovascular treatment, and the role of liver transplantation in this disease. We will also focus on the current controversies and the areas where there is potential for future studies.

[Anatomy of the liver. What does the radiologist need to know?]. / Anatomie der Leber. Was muss der Radiologe wissen?

The liver is the largest unpaired parenchymatous organ in the human body and takes part in almost all important metabolic processes. Many patients show alterations of the whole organ due to vascular/cardiac disorders, metabolic or infectious diseases (congestion, fatty liver disease, fibrosis and cirrhosis). However the liver is also a common site for metastatic lesions of malignant tumors. Additionally, benign focal lesions, such as hemangioma or focal nodular hyperplasia (FNH) occur quite frequently. To describe and diagnose these lesions in terms of dignity and location, knowledge of the macroscopic structure and the relative position of the organ in relation to neighbouring organs are important as well as the histology. The microstructure of the liver and its vascular and biliary vessels are determined by the embryonic development and its function as a central metabolic organ.

Fetal development of the retrohepatic inferior vena cava and accessory hepatic veins: Re-evaluation of the Alexander Barry's hypothesis.

The retrohepatic inferior vena cava (IVC) is commonly considered to originate from the right vitelline or omphalomesenteric vein. In contrast, Alexander Barry hypothesized that one of the hepatic veins grows to merge with the subcardinal vein and develops into the retrohepatic IVC. We re-examined fetal development of the retrohepatic IVC and other related veins using serial histological sections of 20 human fetuses between 6 and 16 weeks of gestation. At 6-7 weeks, when a basic configuration of the portal-hepatic vein systems had just been established, one of hepatic veins (i.e., the posterocaudal vein in the present study) had grown caudally to reach the posterocaudal surface of the liver, and notably, extended into the primitive right adrenal gland (five of the eight early-staged fetuses). Because the inferior right hepatic vein (IRHV) and retrohepatic IVC appeared at the same developmental stage, it is likely that any peripheral remnants of the posterocaudal vein would continue to function as primary drainage territory for the IRHV. The caudate vein developed rapidly in accordance with marked caudal and leftward extension of Spiegel's lobe at 12-16 weeks. Thin accessory hepatic veins developed later than the caudate vein and IRHV. The present results supported Barry's hypothesis.

Regional histomorphometry of the hepatic inferior vena cava; a possible sphincteric mechanism / Histomorfometría regional de la vena cava inferior hepática; un posible mecanismo de esfínter

This study was aimed at identifying the changes in diameter and structural composition of the Hepatic Inferior Vena Cava in its infrahepatic, intrahepatic and suprahepatic portions. Eighty adult liver specimens from the Chiromo and Nairobi City mortuaries were used for morphometry, while twenty of them were processed for light microscopy. A constriction was noted in the mid-portion of the HIVC, while structurally; the intrahepatic portion had thicker fibromuscular adventitia. It is plausible that these are sphincteric apparatus to prevent backflow of blood in the Hepatic Inferior Vena Cava.

Este estudio tiene por objetivo identificar los cambios en el diámetro y la composición estructural de la vena cava inferior hepática en sus porciones infrahepática, intrahepática y suprahepática. Ochenta hígados de especímenes adultos de los depósitos de cadáveres de la ciudad de Nairobi y Chiromo fueron usadas para morfometría, mientras que veinte de ellos fueron procesados para microscopía de luz. Se observó una constricción en el medio de la HIVC, mientras que estructuralmente, la porción intrahepática había una gruesa adventicia fibromuscular. Es posible que este sea un aparato esfinteriano para evitar el reflujo de sangre en la vena cava inferior hepática.

The role of the dioxin-responsive element cluster between the Cyp1a1 and Cyp1a2 loci in aryl hydrocarbon receptor biology.

The aryl hydrocarbon receptor (AHR) plays a central role in 2,3,7,8-tetrachlorodibenzo-p-dioxin (dioxin) hepatotoxicity, regulation of xenobiotic metabolism, and hepatovascular development. Each of these processes appears to be dependent on binding of the AHR to dioxin- responsive elements (DREs) within the genome. The Cyp1a1 and Cyp1a2 loci represent linked genes thought to play important roles in AHR biology. In the mouse, 8 DREs are located in the 14-kb intergenic region between the Cyp1a1 and Cyp1a2 genes. Seven of these DREs, collectively known as the DRE cluster (DREC), are located 1.4 kb upstream of the Cyp1a1 transcriptional start site and 12.6 kb upstream of the Cyp1a2 start site. To investigate the role of the DREC in each aspect of AHR biology, we generated a DREC-deficient mouse model through homologous recombination. Using this mouse model, we demonstrate that the DREC controls the adaptive up-regulation of both Cyp1a1 and Cyp1a2 genes in vivo. Using selected aspects of acute hepatic injury as endpoints, we also demonstrate that DREC null mice are more sensitive to dioxin-induced hepatotoxicity than WT mice. The results of parallel toxicologic studies using individual Cyp1a1 and Cyp1a2 null mice support the observation that up-regulation of these P450s is not the cause of many aspects of dioxin hepatotoxicity. Finally, we observed normal closure of the ductus venosus (DV) in DREC null mice. Given the 100% penetrance of patent DV in Ahr null mice, these results indicate that Cyp1a1 and Cyp1a2 do not play a dominant role in AHR-mediated vascular development.

The ductus venosus and intrahepatic venous system in Callithrix jacchus jacchus and Macaca fascicularis fetuses.

BACKGROUND: The ductus venosus (DV) and the intrahepatic branches of the portal vein are arranged as parallel vessels. Blood shunting through the DV ensures fetal survival during periods of stress. The availability of a suitable animal model with similar structure and function to the human fetus would greatly improve the understanding of DV function. The anatomical and histological structure of the DV has not been thoroughly investigated in non-human primate species. METHODS: Morphological investigations were performed on eight marmoset (Callithrix jacchus jacchus) at 112.5 +/- 5.5 days gestational age (mean +/- SEM) and four near-term (165 days) cynomolgus (Macaca fascicularis) fetuses. RESULTS: The DV drains into the collectus venosus. An asymmetrical muscular lip forms a contractile element of the isthmic portion of the DV. A spherical 'dividing' eminence was found on the dorsal wall of the venous collector just above the outlet of the DV in marmoset fetuses. CONCLUSIONS: Our findings regarding the structure of the DV in cynomolgus and marmoset fetuses were generally in agreement with previous descriptions of the morpho-histological structure of the DV in human fetuses.

[Clinical significance of the doppler evaluation in ductus venosus, hepatic veins and pulmonary veins]. / Znaczenie kliniczne oceny przeplywów w przewodzie zylnym, zylach watrobowych i zylach plucnych.

Doppler evaluation of the fetal venous system at present improves assessment of fetal well-being in many conditions. Actually, analysis of ductus venosus waveforms seems to be the most important. It place role in detection of genetic abnormalities (in low pregnancies), prediction of outcomes growth-restricted fetuses, assessment of severity of heart failure due to congestive heart anomalies, fetal infections, hydrops fetalis. Hepatic veins are known rather as an additional parameter (for ductus venosus) in the severely compromised fetus. Doppler evaluation of pulmonary veins may be useful in prediction of fetal lung maturity and in detection some difficult circulatory anomalies.

Structural evidence for mechanisms to redistribute hepatic and ductus venosus blood flows in nonhuman primate fetuses.

OBJECTIVE: The ductus venosus (DV) and the intrahepatic branches of the portal vein (BPV) play an important role in umbilical blood distribution to the fetal liver and the rest of the fetal circulation. Increased DV shunting is a major fetal survival mechanism during stress situations. The availability of a nonpregnant primate animal model with similar structure and function would greatly improve our understanding of DV function. However, the anatomic and histologic structure of the DV and the BPV have not been thoroughly investigated in any nonhuman primate species. METHODS: Anatomic and immunohistochemical (Masson's and alpha-smooth actin stains) investigations were performed on 17 baboon fetuses at 173 +/- 5 days' gestation (mean +/- SEM, term = 180 days) (Papio sp. ) and 3 near term rhesus (Macaca mulatta) fetuses. RESULTS: In both species the branchless, funnel-shaped DV coursed cranially, posteriorly, and slightly oblique to the left side. The DV and the efferent hepatic veins drained into a dilated ampullary area (the collectus venosus) that joined directly with the inferior cava. The length of the DV in baboons increased with gestational age ( r = 0.86, n = 16). In 4 baboon fetuses, we observed an asymmetrical muscular lip at the isthmic portion of the DV. The media of intrahepatic BPV contained more smooth muscle cells than the media of the DV. CONCLUSION: In nonhuman primate fetuses, the DV drains into a dilated ampullary area. An asymmetrical muscular lip forms a contractile element of the isthmic portion of the DV. The increased thickness of smooth muscle tissue in the DV isthmus and intrahepatic BPV in nonhuman primate fetuses support the concept of a general organization of a contractile apparatus that performs a sphincter-like function in the central venous hepatic system and plays a key role in blood flow redistribution.

The aryl hydrocarbon receptor is required for developmental closure of the ductus venosus in the neonatal mouse.

A developmental role for the Ahr locus has been indicated by the observation that mice harboring a null allele display a portocaval vascular shunt throughout life. To define the ontogeny and determine the identity of this shunt, we developed a visualization approach in which three-dimensional (3D) images of the developing liver vasculature are generated from serial sections. Applying this 3D visualization approach at multiple developmental times allowed us to demonstrate that the portocaval shunt observed in Ahr-null mice is the remnant of an embryonic structure and is not acquired after birth. We observed that the shunt is found in late-stage wild-type embryos but closes during the first 48 h of postnatal life. In contrast, the same structure fails to close in Ahr-null mice and remains open throughout adulthood. The ontogeny of this shunt, along with its 3D position, allowed us to conclude that this shunt is a patent developmental structure known as the ductus venosus (DV). Upon searching for a physiological cause of the patent DV, we observed that during the first 48 h, most major hepatic veins, such as the portal and umbilical veins, normally decrease in diameter but do not change in Ahr-null mice. This observation suggests that failure of the DV to close may be the consequence of increased blood pressure or a failure in vasoconstriction in the developing liver.

Reference values of fetal ductus venosus, inferior vena cava and hepatic vein blood flow velocities and waveform indices during the second and third trimester of pregnancy.

OBJECTIVE: Our objective was to establish reference values for ductus venosus, inferior vena cava and hepatic vein flow velocities during ventricular systole (S-wave) and diastole (D-wave), the lowest forward velocity during atrial contraction (a-wave), the intensity-weighted mean flow velocity (Vmean) and different calculated indices. METHODS: Venous flow velocity waveforms were obtained from 329 singleton pregnancies at 20-42 weeks of gestation by pulsed-wave color Doppler. Reference values were constructed by means of a quadratic regression model after logarithmic transformation of original data. RESULTS: With advancing gestational age the peak velocity index for the vein (PVIV) and pulsatility index for the vein (PIV) decreased whereas blood flow velocities increased. Blood flow velocities were highest in the ductus venosus and lowest in the right hepatic vein. Values for PVIV and PIV were highest in the hepatic vein and lowest in the ductus venosus. During atrial contraction there was a blood flow towards the fetal heart in the ductus venosus, whereas in the inferior vena cava and in the hepatic vein blood flow was either in the opposite from the fetal heart (reverse flow), or there was absent flow (zero flow) or flow was towards the fetal heart (positive flow). CONCLUSIONS: The reference ranges and calculated velocities established in this study may be utilized in studies dealing with the role of ductus venosus and inferior vena cava blood flow in fetuses with chromosomal abnormalities or congenital heart disease as well as hypoxic conditions. We speculate, that the reduction in PVIV and PIV with advancing gestational age may reflect a decrease in cardiac afterload as a result of maturation of diastolic ventricular function.

Congenital hepatic shunts.

Abnormal vascular connections within the hepatic parenchyma are occasionally seen at ultrasonography (US) and require further evaluation. The radiologic findings in 42 children with infantile hepatic hemangioma (n = 28), vascular malformations (n = 10), or infradiaphragmatic total anomalous pulmonary venous return (TAPVR) (n = 4) associated with congenital vascular shunting were retrospectively reviewed. Arteriovenous connections are seen in infantile hepatic hemangiomas and arteriovenous malformations and manifest with aortic tapering at the level of the celiac trunk, hepatic artery enlargement with a low resistivity index (RI), and increased flow velocities in the hepatic veins that may assume an arterialized spectral pattern in late-stage disease. Congenital arterioportal shunts demonstrate a low RI in the hepatic artery, hepatofugal arterialized flow in the portal vein, and rapid development of signs of portal hypertension. Portosystemic shunting may be intra- or extrahepatic. A pulsatile triphasic spectral pattern is seen in the portomesenteric venous system in children with portosystemic shunting, and macroscopic connections between the portal system and the hepatic veins are evident. Infradiaphragmatic TAPVR is associated with a tortuous vessel that parallels the aorta, ends at the intrahepatic left portal vein or the ductus venosus, and has hepatopetal flow. Familiarity with the US features of various congenital abnormal hepatic vascular connections will aid in diagnosis and treatment.

Development of the ductus venosus in the SD rat.

We used scanning electron microscopy to observe the development of the ductus venosus in the fetal rat liver. At day 13 of gestation, the vascular system in the liver was already formed and the umbilical vein had branched many capillaries to the parenchyma of the liver and was connected to the posterior vena cava directly by one small ductus venosus. At day 14 of gestation, the umbilical vein bulged at its terminal part and bifurcated into the ductus venosus, which joined the posterior vena cava, and a branch that anastomosed with the vitelline vein. The ductus venosus had no branches and subsequently enlarged and then degenerated just before birth. The bulging part of the umbilical vein and its branches degenerated in the later stages of gestation. The vitelline vein developed to form the capillaries of the liver and the intestinal venous system. In the SD rat liver, the ductus venosus was therefore established by development of the terminal part of the umbilical vein, which anastomosed directly with the posterior vena cava.

The human fetal venous system: normal embryologic, anatomic, and physiologic characteristics and developmental abnormalities.

OBJECTIVE: The introduction of high-resolution ultrasonography combined with color-coded Doppler imaging offered a breakthrough in the evaluation of the human fetal venous system, considerably enhancing our understanding of fetal venous circulation in normal physiologic conditions, as well as providing us the ability to study circulatory changes in abnormal circumstances. The purpose of this study was to describe the normal anatomic development and complex of anomalies of the human fetal venous system and to review recently published series of these anomalies. METHODS: Normal embryologic and anatomic development is described. An English language literature search of recent MEDLINE listings was performed to glean data from recently published series reporting prenatal diagnosis of the various anomalies and their associated malformations. RESULTS: Anomalies of the human fetal venous system occur sporadically, often associated with cardiac or other malformations. The pathophysiologic mechanisms leading to abnormal in utero development of the human venous system remain largely undetermined. On the basis of the type of vein involved, embryologic precursor, and etiologic correlation (primary or secondary), classification into 4 major groups is described. CONCLUSIONS: Prenatal evaluation of fetuses found to have anomalies of the venous system should include a careful search for cardiac anomalies, including pulmonary venous drainage, and a detailed anatomic survey of the umbilical, portal, hepatic, and ductal systems to determine aberrant communication and, if possible, to discover clues to systemic diseases or thromboembolic phenomena.

Serial aggressive platelet transfusion for fetal alloimmune thrombocytopenia: platelet dynamics and perinatal outcome.

OBJECTIVES: Our purpose was to describe the fetal loss rate and platelet dynamics in fetal alloimmune thrombocytopenia managed by serial platelet transfusions. METHODS: Retrospective analysis over 10 years of consecutive pregnancies affected by fetal alloimmune thrombocytopenia requiring in utero platelet transfusions. RESULTS: There were 2 perinatal losses in 12 pregnancies managed by 84 platelet transfusions. One was obviously procedure related from exsanguination despite platelet transfusion. The attributable procedure related fetal loss rate was 1.2% per procedure but 8.3% per pregnancy. The median rate of fall in fetal platelet count per day after transfusion was lower at the placental cord insertion (n = 54) 40.5 x 10(9)/L (range, 5.4-96.1 x 10(9)/L) compared with that at the intrahepatic vein (n = 30) 50.9 x 10(9)/L,(range, 29.5-91 x 10(9)/L) (P = .0009). CONCLUSION: Pooling our results with those previously published yields a cumulative risk of serial weekly transfusions of approximately 6% per pregnancy, indicating the need for development of less invasive approaches.