UNASSIGNED: Congenital extra hepatic portosystemic shunt (CEPS) is a rare vascular malformation in which splanchnic and portal blood is shunted into the systemic circulation eluding the liver. Type 1 CEPS is sometimes difficult to differentiate from pathologies such as chronic portal veinthrombosis as the portal vein may not be visualized in either entities.
METHODS: A 3-year-old male child with a week of abdominal pain was diagnosed with chronic portal vein thrombosis in an out-of-hospital setting. Repeat abdominal ultrasound was done at our institution and we were able to visualize termination of the portal vein to the suprarenal infra-hepatic inferior vena cava with an end to side pattern and a focal hypoechoic hepatic lesion at segment eight of the liver. There was no evidence of cavernous transformation or sign of portal hypertension. Subsequently, tri-phasic computed tomography revealed similar findings, with the portal vein terminating at the suprarenal inferior vena cava. The focal hepatic lesion showed peripheral contrast enhancement in the arterial phase and appeared as a central non-enhancing area with evidence of homogeneous enhancement on the subsequent sequences.
UNASSIGNED: Type 1 CEPS can be easily confused with chronic portal vein thrombosis as the portal vein may not be visible and the hepatic artery shows compensatory enlargement in both entities. However, portal vein thrombosis is usually associated with underlying predisposing factors and can result in the development of secondary signs of portal hypertension and cavernous transformation which are critical to distinguish it from CEPS.
CONCLUSIONS: Chronic portal vein thrombosis is a great mimicker that should be distinguished from CEPS on ultrasound. A meticulous scan with color flow is helpful to scrutinize vascular anatomy, identify findings associated with CEPS such as hepatic lesions, and exclude signs of chronic portal vein thrombosis.

BACKGROUND: Porto-sinusoidal vascular disease (PSVD) and portal vein thrombosis (PVT) are causes of portal hypertension characterized respectively by an intrahepatic and a pre-hepatic obstacle to the flow in the portal system. As PVT may be a consequence of PSVD, in PVT patients at presentation, a pre-existing PSVD should be suspected. In these patients the identification of an underlying PSVD would have relevant implication regarding follow-up and therapeutic management, but it could be challenging. In this setting ultrasonography may be valuable in differential diagnosis. The aim of the study was to use ultrasonography to identify parameters to discriminate between PSVD and \"pure\" PVT and then to suspect PVT secondary to a pre-existing PSVD.
METHODS: Fifty-three patients with histologically proven PSVD and forty-eight patients affected by chronic PVT were enrolled and submitted to abdominal ultrasonography with elastography by acoustic radiation force impulse (ARFI).
RESULTS: ARFI was higher and superior mesenteric vein (SMV) diameter was wider in PSVD patients than in PVT patients. Thus, a prognostic score was obtained as linear combinations of the two parameters with a good discrimination capacity between PSVD and PVT (the area under the curve = 0.780; 95% confidence interval: 0.690-0.869).
CONCLUSIONS: A score based on ARFI and SMV diameter may be useful to suspect an underlying PSVD in patients with PVT and to identify a subgroup of patients to be submitted to liver biopsy.

OBJECTIVE: To evaluate liver function improvement and volume gain after percutaneous recanalization of chronic portal vein thrombosis (PVT) in non-cirrhotic patients.
METHODS: In this retrospective study, five non-cirrhotic participants between 21 and 67 years old with secondary chronic PVT (4-21 years from diagnose) were submitted to percutaneous portal vein recanalization, followed by varices and shunts embolization.
RESULTS: After a mean of 12.6 months, all portal veins remained patent and there was complete resolution of portal hypertension (PH) symptoms in all participants. There was a significant increase in liver volume of 39.8 ± 19.0% (p = 0.042), platelets count of 53120 ± 20188/μl (p = 0.042), and a significant decrease in total bilirubin levels from 1.04 ± 0.23 mg/dL to 0.51 ± 0.09 mg/dL (p = 0.043). We also found a non-significant increase in albumin levels from 3.88 ± 0.39 g/dL to 4.38 ± 0.27 g/dL (p = 0.078) and decrease in spleen diameter from 16.88 ± 4.03 cm to 14.15 ± 2.72 cm (p = 0.068).
CONCLUSIONS: In this retrospective study, even with a small number of participants, we were capable of showing a median of 39.8% increase in liver volume, laboratorial liver function improvement, platelets count and resolution of PH symptoms, including gastroesophageal varices disappearance after portal vein recanalization followed by shunt embolization.
CONCLUSIONS: In this small series of cases, recanalization of chronic PVT in non-cirrhotic participants was feasible, successful and safe despite the prolonged time of occlusion. This is a new and promising approaching to an old and still challenging disease.

Chronic portal vein thrombosis (PVT) is a major vascular complication of liver cirrhosis. Patients may be asymptomatic and chronic PVT might be detected incidentally on imaging. PVT is associated with worsening liver disease, poorer clinical outcomes, and might proceed to life-threatening intestinal ischemia. Management of chronic PVT with anticoagulation has been shown to be successful in promoting recanalization and reducing thrombus extension in patients with cirrhosis. However, optimal anticoagulation for PVT in cirrhosis has not yet been addressed in any large-scale trial, and the decision to anti-coagulate varies on a case by case presentation. We report the case of a 62-year-old male patient with a history of liver cirrhosis, pancytopenia, and grade II esophageal varices presenting with abdominal pain who was incidentally found to have chronic thrombosis of the portal vein on imaging and was managed appropriately with a good outcome.

BACKGROUND: Studies about treatment of patients with chronic portal vein thrombosis (CPVT) are still limited, especially in different types of CPVT. This study aimed to evaluate the effect of transjugular intrahepatic portosystemic shunt (TIPS) in all types of CPVT with variceal bleeding.
METHODS: Patients with CPVT who received TIPS treatment between January 2011 and June 2019 were divided into four types according to the extent of thrombosis. All patients had a history of variceal bleeding. The characteristics and clinical parameters were collected and recorded. Data on procedure success rate, variation in portal vein pressure, rebleeding, hepatic encephalopathy (HE), stent stenosis, and overall mortality were analyzed.
RESULTS: A total of 189 patients were included in this study (39 in type 1, 84 in type 2, 48 in type 3, 18 in type 4). The TIPS procedure success rate was 86.2%. The success rate was significantly different among the four types (89.7% vs. 88.1% vs. 83.3% vs. 77.8%, P = 0.001). In the TIPS success group, portal vein pressure was significantly reduced from 27.15 ± 6.59 to 19.74 ± 6.73 mmHg after the procedure (P < 0.001) and the rebleeding rate was significantly lower than that of the fail group (14.7% vs. 30.8%, P = 0.017). In addition, there were no significant differences in HE rate (30.7% vs. 26.9%, P = 0.912) or overall mortality (12.9% vs. 19.2%, P = 0.403) between the TIPS success group and the fail group. In the TIPS success group, we found that the occurrence of HE was significantly different (P = 0.020) among the four types, while there were no significant differences in rebleeding rate (P = 0.669), stent stenosis rate (P = 0.056), or overall mortality (P = 0.690).
CONCLUSIONS: TIPS was safe and effective in decreasing portal vein pressure and rebleeding rate in patients with CPVT.

BACKGROUND: The knowledge of natural history of patients with portal hypertension (PH) not due to cirrhosis is less well known than that of cirrhotic patients.
OBJECTIVE: To describe the clinical presentation and the outcomes of 89 patients with non-cirrhotic PH (25 with non-cirrhotic portal hypertension, INCPH, and 64 with chronic portal vein thrombosis, PVT) in comparison with 77 patients with Child A cirrhosis.
METHODS: The patients were submitted to a standardized clinical, laboratory, ultrasonographic and endoscopic follow-up. Variceal progression, incidence of variceal bleeding, portal vein thrombosis, ascites and survival were recorded.
RESULTS: At presentation, the prevalence of varices, variceal bleeding and ascites was similar in the 3 groups. During follow-up, the rate of progression to varices at risk of bleeding (p < 0.0001) and the incidence of first variceal bleeding (p = 0.02) were significantly higher in non-cirrhotic then in cirrhotic patients. A PVT developed in 32% of INCPH patients and in 18% of cirrhotics (p = 0.02).
CONCLUSIONS: In the patients with non-cirrhotic PH variceal progression is more rapid and bleeding more frequent than in cirrhotics. Patients with INCPH are particularly prompt to develop PVT. This observational study suggests that the management of patients with non-cirrhotic PH should take into consideration the natural history of portal hypertension in these patients and cannot be simply derived by the observation of cirrhotic patients.

Portal vein (PV) thrombosis (PVT) in the absence of liver disease or thrombophilia is rare. We report a 57-year-old male with a history of stage 3 chronic kidney disease who presented at the emergency department 18 months after abdominal surgery with progressive abdominal pain and distention. Computed tomography revealed PVT with multiple collaterals and moderate ascites. He had undergone partial gastrectomy and gastrojejunal anastomosis at an outside facility for gastrointestinal stromal tumors that caused an iatrogenic stenotic lesion in the PV. The patient underwent balloon angioplasty and endovascular deployment of an 8 mm × 100 mm Viabahn covered stent (W. L. Gore and Associates, Flagstaff, Arizona) in the extrahepatic PV via a transhepatic approach; the device allowed complete restoration of prograde portal flow with clinical improvement. At 6 months from the intervention, he remains symptom-free with normal liver function tests and patent endoprosthesis on antiplatelet therapy.

BACKGROUND: Portal vein thrombosis (PVT) is a rare but severe vascular disorder with an acute and a chronic course. Most patients have underlying liver cirrhosis; furthermore, thrombophilia is an important risk factor. However, idiopathic forms are also known.
METHODS: This review discusses nonsurgical treatment options in PVT.
CONCLUSIONS: Therapy of acute PVT is based on anticoagulation with heparin that is switched to oral anticoagulants, if applicable. Catheter-guided invasive therapy should be considered; however, patients with liver cirrhosis should be screened for portal hypertension before anticoagulation is mandatory. Therapy of chronic PVT is discussed controversially; therefore, a strict patient selection and an individual therapeutic decision are warranted depending on the etiology of PVT. Special forms of PVT including septic and malignant thrombosis as well as PVT in patients waiting for liver transplantation require particular therapy algorithms.
Die Pfortaderthrombose (PAT) ist eine seltene, aber gravierende Gefäßpathologie, die in eine akute und eine chronische Form unterschieden wird. Häufig tritt die PAT bei Patienten mit einer Leberzirrhose auf. Neben idiopathischen Formen sind insbesondere Patienten mit Gerinnungsstörungen betroffen.
Diese Übersicht beschreibt nichtchirurgische therapeutische Optionen der PAT.
Die Behandlung der akuten PAT basiert auf einer Antikoagulation mit Heparin, später gegebenenfalls auch mit oralen Antikoagulanzien. Kathetergeführte invasive Verfahren können zusätzlich erwogen werden. Insbesondere bei Leberzirrhose ist jedoch eine Vordiagnostik bezüglich einer portalen Hypertension unerlässlich. Die Behandlung der chronischen PAT mit Heparin ist umstritten, sodass hier eine strikte Patientenselektion und eine individuelle Therapieentscheidung notwendig sind. Im Rahmen des ätiologischen Kontexts sollten septische und maligne PATs sowie Patienten vor einer Lebertransplantation gesondert betrachtet werden.