scholarly journals Congenital Extrahepatic Portosystemic Shunts: Spectrum of Findings on Ultrasound, Computed Tomography, and Magnetic Resonance Imaging

2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Pankaj Gupta ◽  
Anindita Sinha ◽  
Kushaljit Singh Sodhi ◽  
Anupam Lal ◽  
Uma Debi ◽  
...  

Congenital extrahepatic portosystemic shunt (CEPS) is a rare disorder characterised by partial or complete diversion of portomesenteric blood into systemic veins via congenital shunts. Type I is characterised by complete lack of intrahepatic portal venous blood flow due to an end to side fistula between main portal vein and the inferior vena cava. Type II on the other hand is characterised by partial preservation of portal blood supply to liver and side to side fistula between main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. The presentation of these patients is variable. Focal liver lesions, most commonly nodular regenerative hyperplasia, are an important clue to the underlying condition. This pictorial essay covers imaging characteristics in abdominopelvic region.

2020 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Ji-Chen Wang ◽  
Shi-Feng Cai ◽  
Chen Su ◽  
Hui-Li Fan ◽  
Yong-Hao Gai ◽  
...  

Background: Spontaneous portosystemic shunts (SPSS) are one of the hallmarks of Budd-Chiari syndrome (BCS). Ultrasound can accurately show the location and type of portosystemic collaterals. Objectives: To study the sonographic feature of SPSS in patients with BCS and to evaluate differences in the main portal vein diameter among multiple types of portosystemic shunts. Patients and Methods: Ultrasonographies of 44 patients with SPSS among 352 BCS patients between June 2000 and November 2015 were reviewed retrospectively. The SPSS in 44 BCS patients were first detected by ultrasound and then confirmed via digital subtraction angiography (DSA), computed tomography angiography (CTA) or magnetic resonance venography (MRV). The location, course, diameter and hemodynamics of the spontaneous portosystemic shunts were observed by ultrasound. In addition, one-way analysis of variance (ANOVA) was performed to evaluate the difference in the main portal vein diameter between the different shunt types. Results: The blood drainage patterns of SPSS in 44 of 352 patients with BCS were classified as the following five types: portal-umbilical shunts (15/44), portal-hepatic shunts (11/44), portal-accessory hepatic shunts (6/44) (the accessory hepatic veins included the inferior right hepatic vein and the caudate lobe vein), splenorenal shunts (8/44) and main portal vein-inferior vena cava shunts (4/44). The corresponding hemodynamics of the five types mentioned above were obtained. Main portal vein-inferior vena cava shunts had a significantly larger mean portal trunk diameter compared with all other types (P < 0.05 for all comparisons). In addition, the mean portal trunk diameters in portal-umbilical shunts and portal-hepatic shunts were obviously larger than that of splenorenal shunts (P < 0.05), while there were no statistically significant differences between the other types. Conclusion: Spontaneous portosystemic shunts are not rare in patients with BCS. Ultrasound is a reliable means for their diagnosis and it offers substantial information for use in clinical treatment.


2008 ◽  
Vol 295 (6) ◽  
pp. G1266-G1273 ◽  
Author(s):  
Nikolai Siebert ◽  
Daniel Cantré ◽  
Christian Eipel ◽  
Brigitte Vollmar

Hepatic blood supply is uniquely regulated by the hepatic arterial buffer response (HABR), counteracting alterations of portal venous blood flow by flow changes of the hepatic artery. Hydrogen sulfide (H2S) has been recognized as a novel signaling molecule with vasoactive properties. However, the contribution of H2S in mediating the HABR is not yet studied. In pentobarbital-anesthetized and laparotomized rats, flow probes around the portal vein and hepatic artery allowed for assessment of the portal venous (PVBF) and hepatic arterial blood flow (HABF) under baseline conditions and stepwise reduction of PVBF for induction of HABR. Animals received either the H2S donor Na2S, DL-propargylglycine as inhibitor of the H2S synthesizing enzyme cystathionine-γ-lyase (CSE), or saline alone. Additionally, animals were treated with Na2S and the ATP-sensitive potassium channel (KATP) inhibitor glibenclamide or with glibenclamide alone. Na2S markedly increased the buffer capacity to 27.4 ± 3.0% ( P < 0.05 vs. controls: 15.5 ± 1.7%), whereas blockade of H2S formation by DL-propargylglycine significantly reduced the buffer capacity (8.5 ± 1.4%). Glibenclamide completely reversed the H2S-induced increase of buffer capacity to the control level. By means of RT-PCR, Western blot analysis, and immunohistochemistry, we observed the expression of both H2S synthesizing enzymes (CSE and cystathionine-β-synthase) in aorta, vena cava, hepatic artery, and portal vein, as well as in hepatic parenchymal tissue. Terminal branches of the hepatic afferent vessels expressed only CSE. We show for the first time that CSE-derived H2S contributes to HABR and partly mediates vasorelaxation of the hepatic artery via activation of KATP channels.


2017 ◽  
Vol 20 (4) ◽  
pp. 354-362 ◽  
Author(s):  
Mark Benedict ◽  
Manuel Rodriguez-Davalos ◽  
Sukru Emre ◽  
Zenta Walther ◽  
Raffaella Morotti

Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein is the result of malformation of the splanchnic venous system. Congenital portosystemic shunts are divided into extra- and intrahepatic shunts. Two shunts have been defined: Type I is characterized by the complete diversion of portal blood into the vena cava with an associated congenital absence of the portal vein. Type II is defined by an intact but diverted portal vein through a side-to-side, extrahepatic connection to the vena cava. The clinical manifestations of Abernethy malformation are diverse with a typical presentation consisting of hypoxia and hepto-pulmonary syndrome. Histologically, focal nodular hyperplasia, nodular regenerative hyperplasia, liver adenoma, hepatoblastoma, and hepatocellular carcinoma have all been reported. Herein, we report a case of Abernethy malformation, type Ib, in a 12-month-old male who was found to have a small hepatocellular carcinoma at the time of explant. The immunohistochemical characteristics in relation to the genetic aspects are discussed. To our knowledge, this is the first reported case of hepatocellular carcinoma developing in a patient who is under the age of 5 years with Abernethy malformation.


2019 ◽  
Vol 141 (5-6) ◽  
pp. 134-137

Abernethy malformation or congenital agenesis of the portal vein (CAPV) is a rare malformation of the abdominal splanchnic venous system. This malformation is commonly found in children and is often associated with other malformations such as congenital cardiac anomalies and skeletal system disorders, as well as liver tumors. There are two types of Abernethy malformation. In type I, portal blood bypasses the liver completely, with the superior mesenteric vein and the splenic vein draining into the inferior vena cava separately (type Ia), or together (type Ib). There are no intrahepatic portal vein branches in the liver. Type II is a partial portocaval shunt in which portal blood partially supplies the liver. There is no unified therapeutic approach for all patients with Abernethy malformation, however, liver transplantation is recommended in patients with liver disease (encephalopathy, poor liver function) and those with liver tumors. In this case report we present a case of Abernethy type Ib malformation in a 17-year-old patient with chronic malaise and uper abdominal pain. During diagnostic work-up, an unresectable liver tumor was found and the patient was successfully treated with orthotopic liver transplantation.


2020 ◽  
Author(s):  
Nan Liu ◽  
Ying Xing ◽  
Chen Wang

AbstractPulmonary arterial hypertension (PAH) is a vascular disorder associated with significant morbidity and mortality. The pathophysiology of PAH remains controversial, but the only currently available therapies for PAH are pharmacological pulmonary artery vasodilation, decreasing right ventricular (RV) afterload, and relieving symptoms. By now, there is no therapy being able to minimize vascular remodeling processes and thus to reverse or delay the natural history of the disease. It has been generally thought that reduction of RV preload was detrimental, which deteriorated the systemic hemodynamics. In the present study, however, we repetitively and briefly occluded (RBO) both superior vena cava and inferior vena cava by ligation (occlusion for less than 5 seconds then re-open for 30 seconds and repeated 5 cycles as one sequence, 1 sequence every 6 hours) to intermittently restrict RV preload, for continuous 24 hours, total 5 sequences, in the Sugen 5416 (VEGF receptor blocker) and hypoxia induced PAH rat models and we found this strategy was beneficial for lowering pulmonary vascular resistance (PVR).


Author(s):  
Emma Brouwer ◽  
Arjan B te Pas ◽  
Graeme R Polglase ◽  
Erin V McGillick ◽  
Stefan Böhringer ◽  
...  

IntroductionDuring delayed umbilical cord clamping, the factors underpinning placental transfusion remain unknown. We hypothesised that reductions in thoracic pressure during inspiration would enhance placental transfusion in spontaneously breathing preterm lambs.ObjectiveInvestigate the effect of spontaneous breathing on umbilical venous flow and body weight in preterm lambs.MethodsPregnant sheep were instrumented at 132–133 days gestational age to measure fetal common umbilical venous, pulmonary and cerebral blood flows as well as arterial and intrapleural (IP) pressures. At delivery, doxapram and caffeine were administered to promote breathing. Lamb body weights were measured continuously and breathing was assessed by IP pressure changes.ResultsIn 6 lambs, 491 out of 1117 breaths were analysed for change in body weight. Weight increased in 46.6% and decreased in 47.5% of breaths. An overall mean increase of 0.02±2.5 g per breath was calculated, and no net placental transfusion was observed prior to cord clamping (median difference in body weight 52.3 [−54.9–166.1] g, p=0.418). Umbilical venous (UV) flow transiently decreased with each inspiration, and in some cases ceased, before UV flow normalised during expiration. The reduction in UV flow was positively correlated with the standardised reduction in (IP) pressure, increasing by 109 mL/min for every SD reduction in IP pressure. Thus, the reduction in UV flow was closely related to inspiratory depth.ConclusionsSpontaneous breathing had no net effect on body weight in preterm lambs at birth. UV blood flow decreased as inspiratory effort increased, possibly due to constriction of the inferior vena cava caused by diaphragmatic contraction, as previously observed in human fetuses.


1998 ◽  
Vol 39 (2) ◽  
pp. 152-156
Author(s):  
H. Dinç ◽  
A. Sari ◽  
H. Resit Gümele ◽  
N. Cihanyurdu ◽  
A. Baki

Purpose: to assess portal and splanchnic haemodynamics, and splanchnic vascular resistance in patients with advanced post-hepatitic cirrhosis and in healthy volunteers, by means of duplex Doppler ultrasound (US) Material and Methods: the duplex Doppler US examination was performed in 16 patients with cirrhosis and in 24 healthy volunteers. We investigated vessel diameters, mean flow velocities, and mean blood flows in the portal vein, the superior mesenteric artery (SMA), and the splenic artery (SA), and measured the resistive index values of SMA and SA Results: the mean portal venous blood flow in patients with cirrhosis (829 ± 264 ml/min) was not statistically different from those in the volunteers (734 ± 194 ml/min). the ratio of the SMA and SA blood flows (621 ml/min) to the portal venous blood flow (734 ml/min) was 0.85 in the control subjects. the mean portal venous blood flow (1261 ml/min) and the portal venous velocity (14.6 cm/s) were higher in the patients with recanalized para-umbilical veins than in the volunteers and in the patients without recanalized para-umbilical veins. the SMA and SA blood flows were significantly increased in patients with cirrhosis compared with volunteers. Splanchnic inflow (the sum of the SMA and SA blood flows) was higher than the portal blood flow in patients with cirrhosis except in the subjects with recanalized para-umbilical veins. SMA and SA resistive index values were significantly higher in these patients than in the volunteers Conclusion: Splanchnic blood flow and splanchnic vascular impedance increased significantly in patients with advanced post-hepatitic cirrhosis. Splanchnic inflow must not exceed portal venous blood flow in patients with recanalized para-umbilical veins. Portal vein velocity and portal venous blood flow measurements alone are not useful parameters for discriminating patients with cirrhosis from healthy subjects


2021 ◽  
pp. 17-22
Author(s):  
V. I. Rusin ◽  
S. O. Boyko ◽  
V. V. Rusin ◽  
S. Sh. S. Boyko

Summary. Purpose. Conduct an anatomical examination of the inferior vena cava (IVC) and its branches and determine the paths of collateral venous blood flow. Materials and methods. An anatomical examination of the IVC and its branches was performed on 27 corpses as a result of autopsy. The bodies of the corpses were hypostenic-normosthenic type. The organ complex was eviscerated by the Shore method. The degree of IVC coverage by the liver in relation to the circumference of the IVC was determined. Measurements of the total length of the IVC and for each of the individual 6 segments of the IVC were performed. The hepatic and lumbar veins were studied and the paths of collateral venous blood flow were analyzed. Results and discussion. The average length of IVC in the infrarenal segment was 107.6 mm, in the retrohepatic — 59.3 mm, in the suprarenal — 26.2 mm, in the interrenal — 23.4 mm, in the infradiaphragm — 15.2 mm, in the supradiaphragm — 12.0 mm, along the entire subdiaphragm segment — 197.8 mm. The coverage of IVC by the liver by 1/2 of its circumference was detected in 13 (48.1 %), by 2/3 — in 11 (40.7 %), by 1/3 — in 2 (7.4 %), by the whole length – in 1 (3.7 %) cases. Up to 23 venous trunks flow into the retrohepatic part of the IVC. The avascular area is located under the main hepatic veins with an average length of 13.1 mm and under the right renal vein with an average length of 17.8 mm. In 92.6 % of cases, the lumbar veins had an odd nature of confluence with the IVC – one common trunk. Conclusions: The anatomical study presented new knowledge of the clinical anatomy of IVС branches.


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