Portal Steal Syndrome From a Large Linton’s Splenorenal Shunt after Liver Transplantation: Successful Endovascular Management Through Off-Label Application of a 30 mm Amplatzer Cardiac Plug

A 34-year-old patient underwent liver transplantation for progressive hepatic failure in the setting of congenital hepatic fibrosis. In past medical history, the patient had undergone splenectomy with proximal Linton’s splenorenal surgical shunt creation for symptomatic portal hypertension with hypersplenism. The patient developed an early allograft dysfunction, with radiologic evidence of a reduced portal flow associated to portal steal from the patent surgical shunt. The patient was successfully treated through endovascular placement of a 30 mm Amplatzer cardiac plug at the origin of the splenic vein.

Management of Pancreatico-duodenal arterio-venous malformation

Purpose To describe the interventional management and clinical outcome of pancreatico-duodenal arterio-venous malformations (PDAVMs). Material and Methods Seven patients presenting a PDAVM (6 women, 1 male; mean age: 61) were retrospectively reviewed. Technical, clinical success and complications of embolization and surgical management of symptomatic PDAVMs were assessed. Technical success was defined as a complete occlusion of the PDAVM and clinical success as no clinical symptom or recurrence during follow-up. Patients with asymptomatic PDAVMs were followed clinically, by Doppler ultrasound and CT-angiography. Results Mean follow-up time was 69 months (15-180). Five symptomatic patients presented with upper gastrointestinal bleeding (n=3), ascites (n=1), and abdominal pain (n=1). Two patients were asymptomatic. The PDAVMs were classified as follow: Yakes I (1), IIIa (2), IIIb (3) and IV (1). Five symptomatic patients were treated with 9 embolization sessions with arterial approach (onyx®, glue, coils) in 7 and venous approach in 2 (plugs, coils, covered stents, STS foam and onyx®). Technical success of embolization was 60% (3/5). Devascularization was incomplete for 2 Yakes IIIB patients. Clinical success of embolization was estimated at 80% (4/5) as one patient required additional surgery (Whipple) because of persistent bleeding. One splenic vein thrombosis was treated successfully by mechanical thrombectomy and heparin. No recurrence occurred during follow-up. No progression was documented in asymptomatic patients. Conclusion Embolization of symptomatic PDAVMs is effective and surgery should be performed in second intention. Complete devascularization is more difficult to obtain in Yakes III PDAVM.

USING NEURAL NETWORK MODELING TO PREDICT THE COURSE OF ACUTE PANCREATITIS

The database of studies of 82 patients with acute pancreatitis are presented. Using neural network analysis, the most indicative parameters for predicting acute pancreatitis were revealed: indexes of Kalf-Kalif intoxication modified by Kostyuchenko and Khomich, Reis, Garkavi, the ratio of leukocytes to ESR, leukocyte index, general intoxication index; sonographic parameters – the size of the head of the pancreas, the diameter of the splenic vein, the presence of free fluid in the abdominal cavity; biochemical parameters – blood amylase concentration, urine diastase. When conducting clustering in a multidimensional feature space, a Kohonen neural network was created. All analyzed objects were effectively divided into 3 clusters. The most severe and prognostically unfavorable is cluster 1, which included data from 30 patients, with the maximum mortality rate and maximum hospital stay.

P-P31 Splanchnic vein thrombosis in acute pancreatitis: Incidence, risk factors and long term outcomes

Background There is paucity of data on the incidence, risk factors and role of anticoagulation for splanchnic vein thrombosis (SVT) in acute pancreatitis (AP). Methods A retrospective review of AP admissions between 2018-2021 across North East England was undertaken. Data on demographics, etiology, severity of AP and SVT was collected. In addition, a selective anticoagulation policy for portal vein thrombosis (PVT) and progressive splenic vein thrombosis was explored. Results 401 patients were included with a mean age of 57.0 and M:F ratio of 1.6:1. 152 patients developed intestinal oedematous pancreatitis and 249 developed necrotising pancreatitis based on Revised Atlanta criteria (RAC). 109 patients (27.2%) developed SVT of which 27 developed a PVT and splenic vein thrombus, 36 PVT only and 46 splenic vein thrombus only. On univariate analysis, alcoholic aetiology, severe pancreatitis, necrotising pancreatitis with >50% necrosis and elevated CRP at 2 weeks were risk factors for developing SVT. On multivariable analysis, alcohol aetiology (OR 2.6, p = 0.002), and >50% pancreatic necrosis (OR 14.6,p = 0.048) increased the risk of developing SVT . 58 patients received anticoagulation for SVT, with a median duration of 90 days of anticoagulation. Recanalization rates were higher for PVT when compared to splenic vein thrombosis. 6 patients developing bleeding complications whilst on anticoagulation therapy. Conclusions A third of patients with AP develop SVT, particularly those with severe AP secondary to alcohol and with extensive pancreatic necrosis. A selective anticoagulation policy was associated with improved recanalization rates and fewer bleeding complications.

P-BN09 Management of isolated splenic vein thrombosis: Risks and benefits of Anticoagulation

Background Isolated splenic vein thrombosis (iSVT) is a common complication of pancreatic disease. Whilst patients remain asymptomatic, there is a risk of sinistral portal hypertension and subsequent bleeding from gastric varices if recanalization does not occur. There is a wide variation of iSVT treatment, even within single centres. We report outcomes of iSVT from tertiary referral hepatobiliary and pancreatic (HPB) units including the impact of anticoagulation on recanalization rates and subsequent variceal bleeding risk. Methods A retrospective cohort study including all patients diagnosed with iSVT on CT scan abdomen and pelvis between 2011 and 2019 from two institutions. Patients with both SVT and portal vein thrombosis at diagnosis, and isolated splenic vein thrombosis secondary to malignancy were excluded. The outcomes of anticoagulation, recanalization rates, risk of bleeding, and progression to portal vein thrombosis were examined. Results Ninety-eight patients with iSVT were included; of which thirty-nine patients received anticoagulation (40%). The most common cause of iSVT was acute pancreatitis n = 88 (90%). The recanalization rate in the anticoagulation group was 46% vs 15% in patients receiving no anticoagulation (p = 0.0008, OR = 4.7, 95% CI 1.775 to 11.72). Upper abdominal vascular collaterals (demonstrated on CT scan angiography) were significantly less among patients who received anticoagulation treatment (p = 0.03, OR = 0.4, 95% CI 0.1736 to 0.9288). The overall rate of upper GI variceal related bleeding was 3% (n = 3/98) and it was independent of anticoagulation treatment. Two of the patients received therapeutic anticoagulation. Conclusions The current data support that therapeutic anticoagulation is associated with a statistically significant increase in recanalization rates of the splenic vein; with a subsequent reduction in radiological left-sided portal hypertension. However, all patients had a very low risk of variceal bleeding regardless of anticoagulation. The findings from this retrospective study should merit further investigation in large-scale randomized clinical trials.

Acute Portal Vein, Superior Mesenteric Vein and Splenic Vein Thrombosis Secondary to JAK2 V617 Mutation- A Case Report

Portal vein thrombosis (PVT) is a rare finding which usually occurs in association with local factors such as cirrhosis, malignancy, pancreatitis, intraabdominal infections or systemic hypercoagulable states. It may present acutely as abdominal pain, ascites, fever or exist in a chronic state which is generally asymptomatic and an incidental finding. With advancement in Imaging and laboratory studies, PVT cases are diagnosed more frequently along with its predisposing factors. The invention of JAK2 mutation and it’s addition to the WHO criteria for Myeloproliferative neoplasm (MPN) diagnosis, has increased the number of MPN cases which were previously labelled idiopathic. We present a case of 54 year old female diagnosed with unprovoked PVT with bowel ischemia and JAK 2 mutation positive, managed surgically and with long term anticoagulation.

Spleen and Liver Fibrosis Is Associated to Treatment Response and Prognosis in Philadelphia-Negative Chronic Myeloproliferative Neoplasms

Introduction: Spleen and liver stiffness, investigated by transient elastography (TE), have been associated with marrow fibrosis in patients (pts) with Ph-negative myeloproliferative neoplasms (MPNs) (Iurlo et al, Br J Haematol. 2015; Webb et al, Ultrasound Q. 2015). Morover, spleen stiffness was found to be greater in Myelofibrosis (MF) and Polycythemia Vera (PV) compared to Essential Thrombocythemia (ET) (Benedetti et al, J Clin Med. 2020). Tissue stiffness can be assessed by ultrasound shear wave elastography (SWE), the two most common techniques being point SWE (pSWE) and bidimensional SWE (2D.SWE). Aims: The aims of this study are: 1) to identify TE differences between MPN pts and healthy volunteers (HV); 2) to evaluate specific TE features in pts with MF, PV and ET; 3) to assess whether spleen/liver stiffness may identify clinical-laboratory features associated with prognosis in MPNs Methods: In this monocentric study, MPN pts and HV received elastometric evaluation of spleen and liver stiffness by pSWE and 2D.SWE with an Esaote MyLab™9 ultrasound system. Spleen area, portal (PVD) and splenic vein diameter (SVD) were measured. Results: A total of 220 pts were included in this study: 142 (64.5%) MPN and 78 (35.5%) HV. MPN pts were affected by MF (63, 44.4%: 39 primary MF), PV (33, 23.2%) or ET (46, 32.4%). Compared to HV, MPN pts had greater median spleen maximal cross sectional area (79 vs 38 cm2, p<0.001), greater spleen stiffness (pSWE 31.3 vs 23.7 kPa, p<0.001; 2D.SWE 25.2 vs 18.7 kPa, p<0.001), and greater liver stiffness (pSWE 6.0 vs 4.9 kPa, p<0.001; 2D.SWE 5.4 vs 4.7 kPa, p<0.001). Additionally, PVD and SVD were significantly larger in MPNs than in HV (PVD 10.9 vs 9.2 mm, p<0.001; SVD 8 vs 6.3 mm, p<0.001). Comparing each MPN to HV, only MF retained all the significant differences; conversely, liver stiffness and PVD were comparable between ET/PV and HV. Clinical and laboratory features of MPN pts are shown in Tab 1. Compared to PV and ET pts, MF pts had higher spleen (p<0.001) and liver stiffness (p<0.001), larger PVD (p<0.001) and SVD (p<0.001). Conversely, ET and PV displayed comparable TE values. Notably, higher median spleen area (p<0.001), larger SVD (p=0.03) and PVD (p=0.02), higher liver (pSWE/2D.SWE, p<0.001/p=0.002) and spleen stiffness (pSWE/2D.SWE, p=0.01/p=0.001) were associated with increased marrow fibrosis grade. Grade 0-1 marrow fibrosis was present in 15 MF, 17 PV and 34 ET pts. Considering only these 66 MPN pts, spleen (40.8 vs 31.3/25.6 in PV/ET, p=0.006) and liver (6.5 vs 5.6/4.7 in PV/ET, p=0.01) stiffness was significantly higher in MF pts. Notably, increased spleen fibrosis was significantly associated with thrombotic history (32.2 vs 24.3 kPa in pts without previous thrombosis, p=0.02). Also, MPN pts with splanchnic vein thrombosis had higher spleen (pSWE: p<0.001; 2D.SWE: p<0.001) and liver stiffness (pSWE: p <0.001), and increased PVD (p=0.02) and spleen area (p=0003). In MF pts, TE data did not correlate with DIPSS risk category. However, a higher spleen stiffness (pSWE/2D.SWE, p=0.09/ p=0.03), liver stiffness (pSWE/2D.SWE, p=0.001/p=0.01), PVD (p=0.002), and SVD (p=0.01) were associated with larger spleen length by palpation. Also, a reduced SVD was associated with the presence of ≥1 high molecular risk mutation (HMR) (p=0.04). As expected, MF pts treated with JAK-inhibitors showed larger spleen area (143.8 vs 83.7 cm 2, p=0.01) and higher spleen stiffness (34.3 vs 24 kPa, p=0.01) compared to pts under cytoreductive therapy. However, pts in spleen response at the time of TE had lower median SVD/PVD (p=0.05/p=0.07) and reduced spleen stiffness (sSWE/2D.SWE: 31.5/25.9 vs 39.0/32.8 in non-responders, p=0.01/p=0.04) In ET/PV, TE data were comparable in pts with/without a complete hematological response. However, IFN was associated with enlarged spleen area and stiffness compared to cytoreduction. Conclusions: TE evaluation effectively distinguishes MF pts from HV and ET/PV, while ET/PV show relevant similarities to each other and to HV. TE data were significantly associated with prognostically relevant features including marrow fibrosis and history of thrombosis in all MPNs, and presence of large splenomegaly and HMR in MF. Finally, TE data were significantly associated with spleen response in MF. Overall, spleen/liver stiffness may help in correct MPN diagnosis, and may provide clinical guidance, being associated with known prognostic factors and treatment outcome. Figure 1 Figure 1. Disclosures Cavo: Bristol-Myers Squib: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Adaptive Biotechnologies: Consultancy, Honoraria; Takeda: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Amgen: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; GlaxoSmithKline: Consultancy, Honoraria; AbbVie: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Janssen: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: TRAVEL, ACCOMMODATIONS, EXPENSES, Speakers Bureau; Sanofi: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novartis: Honoraria; Celgene: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: Travel Accommodations, Speakers Bureau. Piscaglia: ESAOTE: Research Funding. Palandri: CTI: Consultancy; AOP: Membership on an entity's Board of Directors or advisory committees; Celgene: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees; Sierra Oncology: Membership on an entity's Board of Directors or advisory committees.

Sinistral Portal Hypertension after Pancreaticoduodenectomy with Splenic Vein Resection: Pathogenesis and Its Prevention

To achieve curative resection for pancreatic cancer during pancreaticoduodenectomy (PD), extensive portal vein (PV) resection, including porto-mesenterico-splenic confluence (PMSC), may sometimes be necessary if the tumor is close to the portal venous system. Recently, this extended resection has been widely accepted in high-volume centers for pancreatic resection due to its favorable outcomes compared with non-operative treatment. However, in patients with long-term survival, sinistral portal hypertension (SPH) occurs as a late-onset postoperative complication. These patients present gastrointestinal varices due to congested venous flow from the spleen, which may cause critical variceal bleeding. Since the prognosis of patients with pancreatic cancer has improved, owing to the development of chemotherapy and surgical techniques, SPH is no longer a negligible matter in the field of pancreatic cancer surgery. This review clarifies the pathogenesis and frequency of SPH after PD through PMSC resection and discusses its prediction and prevention.