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Portal vein aneurysm (PVA) is rarely encountered, and published papers describing this etiology in adults and children typically include only case reports or small case series. We present a clinical case of PVA in a child associated with severe complications, including diffuse thrombosis of the portal venous system. A 10-year-old boy presented with abdominal pain and vomiting, resulting in an initial diagnosis of pancreatic head tumor based on suspicious images on abdominal grayscale ultrasound. Contrast-enhanced computed tomography confirmed a diagnosis of occlusive PVA thrombosis (36 × 37 × 95 mm). Lacking drastic symptoms, the patient was treated with conservative anticoagulant therapy. On follow-up, the thrombosis appeared to shrink gradually and disappeared at 6 months based on Doppler ultrasound imaging. The PVA was reduced in size, and hepatopetal flow was restored. Surgeons and radiologists should be aware of this rare entity to ensure that a precise diagnosis can be established and to provide suitable treatment.

Portal vein thrombosis (PVT) is a rare but severe entity that can cause clinically significant sequela such as worsening portal hypertension or mesenteric ischemia. Those cases refractory to medical management may be referred for endovascular intervention. Several technical considerations have been described in the literature, but a cohesive comparison of these multiple techniques is lacking.The purpose of this article is to review the diagnosis and endovascular management of PVT, including areas in which further research is warranted.Cases of PVT can be readily diagnosed using ultrasound, computed tomography, or magnetic resonance imaging. Treatment often begins with systemic anticoagulation and endovascular interventions may be used in selected cases. Determining the optimal approach to accessing the portal venous system depends on the underlying disease and chronicity of the thrombus and the degree of occlusion. Once access to the portal venous system is established, catheter-directed therapy may be performed to achieve recanalization.Despite the heterogeneity in patient presentation, cases of PVT can be readily diagnosed across several imaging modalities. Strategizing interventional approaches involves evaluation of the underlying disease and the chronicity of the thrombus.· This review will enable interventionalists to establish a framework for treating portal vein thrombosis by identifying patient risk factors and thrombus characteristics that determine patient management.. · The unique risks and benefits for transhepatic, transsplenic, and transmesenteric approaches for establishing portal venous access will be discussed.. · Advantages and complications of thrombolysis, thrombectomy, and transjugular intrahepatic portosystemic shunt creation for treating portal vein thrombosis will be reviewed in detail based on our extensive institutional experience..· Ju C, Li X, Gadani S et al. Portal Vein Thrombosis: Diagnosis and Endovascular Management. Fortschr Röntgenstr 2022; 194: 169 - 180.Die Pfortaderthrombose (PVT) ist eine seltene, aber schwere Erkrankung, die klinisch signifikante Folgeerscheinungen wie eine Verschlechterung der portalen Hypertonie oder eine mesenteriale Ischämie verursachen kann. Fälle, die auf eine medizinische Behandlung nicht ansprechen, können zur endovaskulären Intervention überwiesen werden. In der Literatur sind verschiedene technische Gesichtspunkte beschrieben worden, aber ein umfassender Vergleich dieser verschiedensten Methoden fehlt.In diesem Artikel werden die Diagnose und die endovaskuläre Behandlung der PVT besprochen, einschließlich der Bereiche, in denen weitere Forschung erforderlich ist.PVT-Fälle lassen sich mit Ultraschall, Computertomografie oder Magnetresonanztomografie leicht diagnostizieren. Die Behandlung beginnt häufig mit einer systemischen Antikoagulation, und in ausgewählten Fällen können endovaskuläre Interventionen eingesetzt werden. Die Bestimmung des optimalen Zugangs zum Pfortadersystem hängt von der Grunderkrankung und der Chronizität des Thrombus sowie vom Grad der Okklusion ab. Sobald der Zugang zum Pfortadersystem hergestellt ist, kann eine Katheter-gesteuerte Therapie durchgeführt werden, um eine Rekanalisierung zu erreichen.Trotz der Heterogenität der Patienten bei Vorstellung können Fälle von PVT mit verschiedenen bildgebenden Verfahren leicht diagnostiziert werden. Bei der Planung von Interventionen müssen die Grunderkrankung und die Chronizität des Thrombus berücksichtigt werden.· Diese Übersicht ermöglicht es den interventionell tätigen Ärzten, Rahmenbedingungen für die Behandlung von Pfortaderthrombosen zu schaffen, indem Risikofaktoren für Patienten und Thrombusmerkmale identifiziert werden, die das Patientenmanagement bestimmen.. · Die besonderen Risiken und Vorteile transhepatischer, transsplenischer und transmesenterialer Ansätze zur Etablierung eines portalvenösen Zugangs werden diskutiert.. · Vorteile und Komplikationen der Thrombolyse, der Thrombektomie und der Anlage eines transjugulären intrahepatischen portosystemischen Shunts zur Behandlung von Pfortaderthrombosen werden basierend auf der umfangreichen Erfahrung in unserem Institut im Detail besprochen..

Pylephlebitis is defined as the development of portal venous thrombosis secondary to intra–abdominal infection or inflammation. We present the case of a 69-year-old male with pylephlebitis who attended the Emergency Department with nonspecific abdominal pain, rigors and deranged liver function tests. After an initial negative ultrasound study, computed tomography (CT) scanning demonstrated perforated diverticulitis with an associated thrombus within the portal venous system. Prompt imaging coupled with a high index of clinical suspicion helps in identifying this condition early, significantly reducing morbidity and mortality rates. This case emphasizes the importance of careful evaluation of the portal venous system in cases of intra–abdominal sepsis to exclude this rare, and sometimes fatal, condition.

Background and aim Hemostasis profile is often complicated in liver cirrhosis. Thromboelastography is a global viscoelastic test recommended by the current practice guideline and consensus. This cross-sectional study aimed to evaluate the association of thromboelastography profile with severity of liver cirrhosis and presence of portal venous system thrombosis (PVST). Methods Overall, 116 and 50 cirrhotic patients were included in the Shenyang and Xi’an cohorts, respectively. Thromboelastography parameters were compared between cirrhotic patients with Child–Pugh class A and B/C, those with and without decompensated events, and those with and without PVST. Hypercoagulability would be considered if at least two of the following thromboelastography parameters were met: shortened reactive time (R), shortened coagulation time (K), increased angle, and increased maximum amplitude (MA). Results In the Shenyang cohort, 16 patients had shortened R, of whom seven (43.75%) had prolonged K and 11 (68.75%) decreased MA. In the Xi’an cohort, 24 patients had shortened R, of whom seven (29.17%) had prolonged K and 15 (62.50%) decreased MA. In the Shenyang cohort, the prevalence of hypercoagulability was not significantly different between cirrhotic patients with Child–Pugh class A and B/C (3.85% vs. 6.25%, P = 0.873), those with and without decompensated events (5.49% vs. 4.00%, P = 1.000), and those with and without PVST (4.17% vs. 5.88%, P = 1.000), which were similar to the results obtained in the Xi’an cohort. Conclusion There is a high rate of discordance between R and other thromboelastography parameters. In addition, hypercoagulability may not be related to more advanced stage of liver cirrhosis or presence of PVST.

Background Anomalies of the portal venous system can be congenital or acquired, the latter being related to spontaneous thrombosis or iatrogenic alterations such as complications of perinatal catheterization of the umbilical vein. These conditions can be clinically silent for years and then manifest abruptly causing severe clinical emergencies. Case presentation This case report describes the diagnosis and interventional management of a singular abnormality in the portal venous system of an 8-year-old female that led to severe portal hypertension and acute variceal bleeding. Peculiar imaging findings were not pathognomonic for any of the known congenital and acquired portal vein anomalies: absence of a normal extrahepatic portal vein; splenic and mesenteric veins merging into a dilated left gastric vein; presence of an aberrant mesenteric venous collateral with a stenotic connection with the intrahepatic right portal branch; and absence of porto-systemic shunt. The case was successfully managed with percutaneous transhepatic portography and angioplasty. Conclusions Prompt non-invasive imaging characterization allowed to understand the singular vascular abnormality and mini-invasive interventional radiology management resolved portal hypertension and variceal bleeding.

Hemodynamic change after total paracentesis was investigated because it might lead to various complications. Although cell-free and concentrated ascites reinfusion therapy (CART) is safer and more effective than total paracentesis in theory, hemodynamic change after CART has been never reported. And previous studies did not mention hemodynamics of the venous system. We investigated the hemodynamic change, including that of the venous system, before and after CART using color Doppler ultrasonography and fast Fourier transform analysis. Twenty-eight patients with tensive cirrhotic ascites underwent ultrasonography the day before and after total volume CART. The diameter and velocity of the main, right, and left portal vein; inferior vena cava (IVC); and right renal vein were measured using ultrasonography. A total of 11.8 ± 4.4 L of ascites (range 3.6–20.9 L) was filtered and concentrated to 0.85 ± 0.40 L (range 0.36–1.50 L). The diameter of the IVC increased from median 13.5 ± 5.4 mm (range 4–25 mm) to 18.5 ± 4.1 mm (range 7–29 mm) (p = 0.007). The diameter of the right segmental renal vein significantly increased after KM-CART [from 5.0 ± 1.0 (4–8) mm to 7.0 ± 2.0 (3–10) mm] (p = 0.011). Hemodynamic change of the portal venous system was not significant. The time to the next CART in patients with an IVC diameter ≥ 20 mm and

The role of ligation of the portal venous branches to the caudate lobe (cPVL) as preparation for planned major hepatectomy is unclear. The aim of this study was to evaluate the efficacy of laparoscopic cPVL (Lap-cPVL) concomitant with transileocolic portal vein embolization of the right portal venous system (rTIPE), namely, Lap-cPVL/rTIPE, for planned right hemihepatectomy (rHx) in advanced hepatobiliary cancer patients. Thirty-one patients who underwent rHx after rTIPE with/without Lap-cPVL between March 2013 and March 2020 were enrolled in this study. The Lap-cPVL was performed for the portal branches of the right caudate lobe. Eight of the 31 patients underwent Lap-cPVL/rTIPE. The degree of hypertrophy was significantly increased in Lap-cPVL/rTIPE (19.3%, range 6.5–25.6%) as compared to rTIPE (7.2%, range - 1.1 to 21.2%) (p=0.027). The functional kinetic growth rate was also significantly increased in Lap-cPVL/rTIPE (5.40%, range 2.17–5.97) than that in rTIPE (1.85%, range - 0.22 to 6.45%) (p=0.046). Postoperative liver failure ≧ grade B occurred in 21.7% of patients in rTIPE, while there was no postoperative liver failure ≧ grade B in Lap-cPVL/rTIPE. Mortality rates were zero after rHx in this study. Lap-cPVL/rTIPE is safe and provides an additional effect on liver hypertrophy in advanced hepatobiliary cancers.

Diagnosis and severity assessments of portosystemic shunts (PSSs) are important because the pathology sometimes results in severe hepatic encephalopathy, which can be treated almost completely by shunt embolization. At present, morphological assessment of PSS is performed mainly by computed tomography, and ultrasound is used for blood flow assessment. In two cases of PSS-related hepatic encephalopathy, we used time-resolved 3D cine phase-contrast (4D-flow) magnetic resonance imaging (MRI) to assess blood flow before and after shunt embolization. Before the intervention, blood flow in the main trunk of the superior mesenteric vein was mostly hepatofugal. However, post-interventional 4D-flow MRI revealed hepatopetal superior mesenteric vein flow with significantly increased portal vein blood flow. 4D-flow MRI is an ideal adjunct to Doppler ultrasonography, allowing for objective and visual assessment of morphology and blood flow of the portal venous system, including PSSs, and is useful in determining the indications for, and outcome of, PSS embolization.

Purpose Portal venous system thrombosis is a complication of partial splenic artery embolization, and pre-treatment risk assessment is thus important. The purpose of this study was to identify the risk factors for portal venous system thrombosis after partial splenic artery embolization. Materials and methods We retrospectively analyzed 67 consecutive patients who underwent contrast-enhanced computed tomography before and after first partial splenic artery embolization between July 2007 and October 2018. As risk factors, we investigated age, sex, hematological data, liver function, steroid use, heparin use, and findings from pre- and post-treatment computed tomography. Uni- and multivariate analyses were performed to evaluate the relationship between thrombus appearance or growth and these factors. Values of p Results Partial splenic artery embolization was technically successful in all 67 patients. Nine patients showed appearance or growth of thrombus. Univariate analysis showed maximum diameter of the splenic vein before treatment (p = 0.0076), percentage of infarcted spleen (p = 0.017), and volume of infarcted spleen (p = 0.022) as significant risk factors. Multivariate analysis showed significant differences in maximum diameter of the splenic vein before treatment (p = 0.041) and percentage of infarcted spleen (p = 0.023). According to receiver operating characteristic analysis, cutoffs for maximum diameter of the splenic vein and percentage of infarcted spleen for distinguishing the appearance or growth of thrombus were 17 mm and 58.2%. Conclusion Large maximum diameter of the splenic vein before partial splenic artery embolization and high percentage of infarcted spleen after partial splenic artery embolization were identified as risk factors for portal venous system thrombosis. Level of Evidence Level 4, Case Series

A 56-year-old female with thrombocythemia complicated by portal venous system thrombosis presented with recurrent left pleural effusions after failed recanalization via mechanical thrombectomy and stenting at an outside center. With no other cause, splenic vein thrombosis and left-sided portal hypertension was suggested as a possible etiology. Partial splenic embolization was performed with immediate decrease in effusions and resolution by 8 weeks. Portal and splenic venous system thrombosis may cause recurrent pleural effusions from left-sided portal hypertension and fluid leakage across diaphragmatic defects. Upper pole partial splenic embolization may treat recurrent left pleural effusions and offer an alternative to splenectomy.

Portal vein thrombosis refers to complete or partial obstruction of the portal venous system, in the intrahepatic or extrahepatic venous tract or even in the splenic or superior mesenteric veins. This common and potentially fatal condition can develop in various clinical contexts, especially those of liver cirrhosis, hepatocellular carcinoma, and other solid tumors. Certain characteristics, such as the time since the onset of the thrombus (acute or chronic), its biology (hematic or tumoral), the presence of collateral vessels, and the magnetic resonance imaging aspects, are important components of a thorough, careful analysis, as well as informing decisions regarding the appropriate therapeutic strategy. Here, we present a brief review of the anatomy of the portal venous system and a systematic approach to analyzing the condition, using a mnemonic (ABCD, for age, biology, collaterals, and diffusion). We discuss the various imaging methods and illustrate our discussion with images selected from the case files archived at our facility. Resumo Trombose da veia porta refere-se à obstrução completa ou parcial do sistema venoso portal, localizada nos tratos venosos intra-hepáticos ou extra-hepáticos e até mesmo nas veias esplênica ou mesentérica superior. Vários contextos clínicos podem ser responsáveis pelo desenvolvimento desta condição frequente e potencialmente fatal, especialmente a cirrose hepática, o carcinoma hepatocelular e outros tumores sólidos. Algumas características como o tempo de aparecimento do trombo (agudo ou crônico), sua biologia (hemático ou tumoral), a presença de vasos colaterais e o seu comportamento na ressonância magnética são importantes para uma análise completa e criteriosa, assim como para o gerenciamento adequado da estratégia terapêutica. No presente artigo apresentamos breve revisão da anatomia do trato venoso portal, seguida de uma abordagem sistemática usando um mnemônico (ABCD) para análise da trombose da veia porta por diferentes métodos de imagem, utilizando imagens de casos selecionados do arquivo de ensino do nosso serviço.

Background Portal venous gas (PVG) is a rarely observed clinical finding generally associated with intestinal ischemia. The proper clinical response to the finding of PVG depends somewhat on the setting in which it is observed. Here we describe a case in which extensive arterial gas emboli (AGE) were encountered during point-of-care ultrasound (POCUS) and subsequent computed tomography (CT) identified PVG secondary to gastric wall ischemia as the likely source. Case Report A 69-year-old woman with history of metastatic colon cancer presented to the emergency department (ED) with altered mental status. On arrival, she was hypotensive, hypothermic, cachectic, and with abdominal distension. POCUS was performed to evaluate the source of the patient's hypotension, revealing the presence of PVG, as well as gas bubbles in all four chambers of the heart and the aorta. CT scan revealed gastric wall ischemia and confirmed the presence of significant air emboli throughout the portal venous system. Given the overall poor prognosis, the decision was made to forego further chemotherapy or surgery and the patient died later that week while under hospice care. Why Should an Emergency Physician Be Aware of This? AGE can occur in the setting of PVG. This may cause multi-organ failure by disrupting blood flow to organs, especially in patients with circulatory dysfunction, such as shock. Depending on the setting in which it is diagnosed, early detection of PVG may expedite earlier assessments of a patient's negative prognosis or initiation of attempted life-saving treatment. In this case report, we show that POCUS can be used to obtain an expedited diagnosis in a critically ill patient.

Venous thrombosis within the hepatic vasculature is associated with a distinct array of risk factors, characteristics, and potential complication. As such, it entails unique management considerations and strategies relative to the more common categories of venous thromboembolic disease. Although broadly divided into thrombosis of the afferent vasculature (the portal venous system) and efferent vasculature (the hepatic venous system), presentations and management strategies within these groupings are heterogeneous. Management decisions are influenced by a variety of factors including the chronicity, extent, and etiology of thrombosis. In this review we examine both portal vein thrombosis and hepatic vein thrombosis (and the associated Budd-Chiari Syndrome). We consider those factors which most impact presentation and most influence treatment. In so doing, we see how the particulars of specific cases introduce nuance into clinical decisions. At the same time we attempt to organize our understanding of such cases to help facilitate a more systematic approach. Critically, we must recognize that although increasing evidence is emerging to help guide our management strategies, the available data remain limited and largely retrospective. Indeed, current paradigms are based largely on observational experiences and expert consensus. As new and more rigorous studies emerge, treatment strategies are likely to be continually refined, and paradigm shifts are sure to occur.

Resumen En este artículo se revisan los aspectos más importantes de la epidemiología, fisiopatología, abordaje diagnóstico y terapéutico de la trombosis del sistema venoso portal y mesentérico, para finalizar discutiendo la experiencia clínica de un caso a la luz de la literatura relevante. Esta complicación es usualmente relacionada con afecciones sistémicas y hepáticas, pero rara vez se presenta aislada y relacionada con el trauma. En ausencia de tratamiento adecuado durante la etapa aguda, la trombosis puede evolucionar hacia el infarto intestinal y la muerte del paciente. En casos crónicos, se ha asociado con la degeneración del sistema venoso portal hacia cambios por hipertensión portal crónica. Abstract This article reviews the epidemiology, pathophysiology, diagnostic and therapeutic approach of the portal and mesenteric venous system thrombosis, with a clinical case discussion considering the relevant literature. This complication is usually related to systemic and hepatic conditions, but rarely occurs isolated and related to trauma. In the absence of adequate treatment during the acute stage, thrombosis can progress to intestinal infarction and death. In chronic cases, it has been associated with degeneration of the portal venous system into changes due to chronic portal hypertension.

Malformations of the portal venous system consist of congenital and acquired anomalies. Congenital portosystemic shunts represent rare vascular developmental anomalies that allow partial or complete diversion of blood flow from the portal venous system to the systemic venous system, bypassing the liver. Congenital portosystemic shunts may be associated with malformations or congenital absence of the portal vein, and it was first described by John Abernethy in 1793. Most cases are diagnosed in early childhood, but some congenital shunts may remain asymptomatic and are encountered incidentally because of the widespread use of computed tomography and magnetic resonance imaging. In this report, we discuss the case of a 40-year-old female who presented to the Emergency Department with right upper quadrant pain, nausea, and vomiting. Clinical presentation and abdominal computed tomography angiography were consistent with the diagnosis of calculous cholecystitis and congenital absence of portal vein with intrahepatic portosystemic shunts. We discuss the importance of radiology in diagnosing such incidental malformations, coupled with a review of the current literature on this topic.

A 43-year-old male patient, who received a deceased donor liver transplant for background ethanol-related decompensated cirrhosis, presented 7 months after transplant with mild abdominal distension and pain. On evaluation, the patient had thrombocytopenia, high serum-ascites albumin gradient ascites, and deranged liver functions. The Doppler study of the splenoportal axis showed hepatofugal flow in the recipient's portal vein, normal hepatic veins, a normal liver, splenomegaly, mild ascites, and multiple periportal collaterals. A transjugular liver biopsy and a hepatic venous pressure gradient measurement were done, which suggested mild portal tract inflammation with portal tract fibrosis with prominent portal venous thickening and normal hepatic venous pressure gradient (4 mm). However, the patient had a progressive increase in ascites and a dramatic increase in serum bilirubin level. A triple-phase computed tomography was done that showed rapid contrast flow in both the portal and hepatic arterial phase, suggesting arterialization of the portal flow with possible suspicion of a communicating arterioportal fistula. The patient underwent digital subtraction angiography, which was followed by an embolization of the arterioportal fistula. After embolization, serum bilirubin gradually decreased and ascites resolved. A repeat Doppler of the portal venous system showed established hepatopetal flow with progressively rising portal flow velocities.

Background Hepatic portal venous gas in infants is frequently due to late presentation of necrotizing enterocolitis which is considered a relative indicator for surgical intervention. Case summary A preterm baby underwent an umbilical catheter placement and discovered in abdominal radiograph to have air in the portal venous system due to malpositioning of the umbilical catheter. Conclusion Hepatic portal venous gas in infants without signs of necrotizing enterocolitis could result from malposition of umbilical venous catheter, and in that case, should be managed medically, with no need for surgical intervention.

This study investigated the morphologic changes of portal cavernoma in children with extrahepatic portal venous obstruction and explored the relationship with prognosis.From February 2008 to October 2017, there were 107 patients with cavernous transformation of the portal vein admitted to our hospital. Rex shunts were performed in 99 cases, Warren shunts in 7 cases, and laparoscopic splenic vessel ligation in 1 case. Intraoperative superior mesenteric venography was used to determine the structure of the portal venous system. According to the morphologic features of the portal vein shown by portal venography, groups were assigned as follows: patients with the cotton form of portal cavernoma; patients with visible collateral veins of portal cavernoma; patients with and without a visible left gastric vein; and patients with and without a clearly visible intrahepatic portal vein. The preoperative and postoperative portal pressure, preoperative incidence of esophageal varices, time at onset, incidence of postoperative rebleeding, preoperative and postoperative size of the spleen, and age at time of operation were compared between these groups.The preoperative incidence of esophageal varices, time at onset, postoperative size of spleen, and age at time of operation were significantly lower in the group with the cotton form than in those with visible collateral veins (P .05). There was a significant correlation between the visible left gastric vein and esophageal varices (P = .002). The time at onset, preoperative and postoperative size of the spleen, and age at time of operation were markedly lower in the group with a good visible intrahepatic portal vein than in those without a clearly visible intrahepatic portal vein (P .05). The visible left gastric vein was notably associated with the performance of a gastroportal shunt (P = .000), and the group with a visible left gastric vein had a higher ratio of children undergoing a gastroportal shunt.The cotton form, an early-stage manifestation of cavernous transformation of the portal vein, typically occurs in younger children with a shorter time to onset. Children with the cotton form of portal cavernoma typically have a better prognosis after Rex shunt.

Portal hypertension represents a dysfunctional hemodynamic alteration within the portal venous system, usually due to cirrhosis. These altered hemodynamics play a direct role in the development of acute, life-threatening variceal hemorrhage and mark an important pathophysiologic step in the development of other sudden and life-threatening complications such as type 1 hepatorenal syndrome and spontaneous bacterial peritonitis. Many life-saving interventions in these patients are aimed at reducing portal venous pressure. The contribution of portal hypertension to the development of variceal hemorrhage, type 1 hepatorenal syndrome (known also by the name HRS-AKI), and spontaneous bacterial peritonitis in cirrhotic patients is discussed. This article reviews the pathophysiology and incidence of life-threatening complications of cirrhotic portal hypertension, with a discussion of management options and treatment costs.

AIM Lenvatinib is an oral, multitargeted, tyrosine kinase inhibitor, which suppress tumor angiogenesis and tumor progression. It was non-inferior to sorafenib in overall survival in untreated advanced hepatocellular carcinoma (HCC). Sorafenib had a beneficial effect on portocollateral circulation with portal hypertension in translating and clinical studies. However, the hemodynamic effects of lenvatinib appear to be different from those of sorafenib because the efficacy of lenvatinib for vascular endothelial growth factor receptors and fibroblast growth factor receptors is different from that of sorafenib. This study was prospectively performed to evaluate the portal hemodynamic effect of lenvatinib in patients with advanced HCC using duplex Doppler ultrasonography. METHODS In total, 28 Child-Pugh class A or B patients with advanced HCC received lenvatinib depending on body weight daily for 2 weeks. Primary outcomes were changes in the hemodynamics of the portal venous system using duplex Doppler ultrasonography before and after the 2-week administration of lenvatinib. RESULTS The portal venous flow velocity (cm/s) significantly reduced (27 ± 12.1 vs. 22.6 ± 8.0, P = 0.019), while portal venous area (cm2 ) did not change after the 2-week administration (0.80 ± 0.36 vs. 0.82 ± 0.27, P = 0.665). Therefore, the congestion index (portal venous area/portal venous flow velocity), which reflects the pathophysiological hemodynamics of the portal venous system significantly worsened (0.037 ± 0.025 vs. 0.043 ± 0.024, P = 0.045). CONCLUSIONS Considering that this was a short-term study, because lenvatinib could be an agent that aggravates portal hypertension, it will be necessary to verify its clinical effects for portal hypertension in future studies.

Ductus venosus agenesis is a rare anomaly which is significantly associated with cardiac, extracardiac and chromosomal anomalies. The prognosis depends on its association with other congenital abnormalities independent of the type of shunt. The aim of this study was to evaluate the ductus venosus in 2nd trimester scans and to evaluate associated congenital malformations associated with agenesis of the ductus venosus and its feto-neonatal outcome. We performed a retrospective study for a 5 year period from November 2015 to November 2019. Of the 8 cases seen, 2 (25%) had an intrahepatic shunt and 6 (75%) presented with extrahepatic umbilical venous drainage. Agenesis of ductus venosus was associated in 5 cases (62%) with cardiac abnormalities, 6 cases (75%) with extracardiac anomalies and 3 (37%) cases with chromosomal abnormality. Our study showed poorer outcome in fetuses associated with both cardiac and extracardiac anomalies accounting for 75% of all cases whereas intrahepatic type with mild cardiomegaly had favourable pregnancy outcome. Agenesis of ductus venosus is significantly associated with fetal cardiac and extracardiac abnormalities independent of the type of umbilical venous drainage. Extrahepatic umbilical venous drainage even if isolated has the significant risk of congestive heart failure, hydrops and/or intrahepatic portal venous system anomalies. The intrahepatic type has better prognosis if associated with no or minor abnormality.

To outline the use of radiological investigations in patients with congenital portosystemic shunts (CPS) and to assess the degree of portal venous deprivation in relation to the development of liver tumors.Single center retrospective cohort study of all patients with CPS referred from 1990 to 2016. Radiological investigations were reviewed for the presence of intrahepatic portal veins. Two groups were defined: Group 1 - without evidence of preservation of an intrahepatic portal venous system and, Group 2 - with evidence of intrahepatic portal venous flow. These groups were compared for the development of liver tumors, serum ammonia, and nature of subsequent surgical intervention. The ratio of infraceliac to supraceliac aortic diameter and hepatic enhancement in biphasic CT scans were also used to infer hepatic arterial in-flow and compared to age matched controls. Nonparametric tests were used throughout. A P value of 0.05 was considered significant. Data are quoted as median (IQR).45 patients (Group 1, n = 12: Group 2, n = 33) were investigated for CPS at a median age of 8 months (1 month-14 years). Liver tumors were more common in Group 1 than Group 2 [11/12 (92%) versus 10/33 (29%); P 0.001]. Aortic ratio was significantly lower in patients with CPS compared to control (0.82 versus 0.96; P 0.001), but there was no difference between patients with or without tumors (0.82 versus 0.82; P = 0.52). Enhancement of the liver parenchyma was greater in portal venous rather than arterial phases by a median difference of 28 (15-50) Hounsfield units in controls, compared to 15 (8.5-23.5) in CPS patients (P = 0.04). A single stage closure was possible in 2/6 (33%) operated patients in Group 1 and 14/20 (70%) in Group 2 (P = 0.32).Patients without radiological evidence of intrahepatic portal venous flow were significantly more likely to have associated hepatic tumors with a relative risk 3.1.This manuscript includes data that are III and IV Levels-of-Evidence.