Cavernomatous Transformation Of Portal Vein Research Articles

Spectrum of hematological abnormalities in patients with extrahepatic portal vein obstruction evaluated at a tertiary care center

Background and Introduction Extra-hepatic Portal Vein Obstruction (EHPVO) is a vascular disease characterized by post-thrombotic obstruction and cavernomatous transformation of portal vein leading to portal hypertension.Aims and objectives To analyze the spectrum of hematological abnormalities in all patients of ‘idiopathic EHPVO’ referred to Hematology department.Methods It is a retrospective study in which files of all patients of EHPVO presenting to hematology department over a period of 22 months were retrieved. All cases of chronic liver disease, malignancy and post-surgery were excluded. The laboratory evaluation included ELISA for anti-beta-2-glycoprotein1 antibodies, anti-cardiolipin antibodies, Protein C, Protein S, Antithrombin-III and plasma homocysteine, screening for Activated Protein C-resistance, mutation analysis for Factor V Leiden, MTHFR, JAK2 V617F, prothrombin G20210A mutation and flow cytometry for paroxysmal nocturnal hemoglobinuria (PNH) clone.Results Out of 122 patients, 52.4% (64) had underlying hematological cause for EHPVO. Hyperhomocysteinemia (15.5%) was the most common hematological abnormality followed by JAK2V617F allele positivity (10.6%) and antiphospholipid antibody (APLA) (10.6%). After excluding hyperhomocysteinemia, Protein C deficiency was the commonest cause in younger age group (<25yrs) while latent Myeloproliferative neoplasm (MPN) was commonest in the older subset. 4 patients in the older group had high PNH clone size on granulocytes and monocytes. In our study, the overall prevalence of acquired causes of EHPVO (hyperhomocysteinemia and positivity for JAK2V617F allele) was more common than inherited causes.Conclusion All patients with idiopathic EHPVO should undergo comprehensive evaluation for underlying hematological abnormalities especially for latent myeloproliferative neoplasm.

Combined Rex-bypass shunt with pericardial devascularization alleviated prehepatic portal hypertension caused by cavernomatous transformation of portal vein

ABSTRACTObjective: To evaluate the effects of combined Rex-bypass shunt and pericardial devascularization on prehepatic portal hypertension secondary to cavernomatous transformation of portal vein (CTPV).Methods: Forty-two patients aged from 3 years to 49 years (divided into 3 groups), 26 cases male and 16 female, with prehepatic vascular hepertention were treated with Rex-bypass shunt combined with pericardial devascularization. In each patient, preoperative assessment included ultrasound and computed tomographic angiography of the portal vein and blood analysis. The procedure was Rex-bypass shunt (with or without graft), and patients with moderate or severe gastroesophageal varices required additional paraesophagogastric devascularization. Splenectomy or subtotal splenectomy was performed if combined hypersplenism co-existed. All data were analyzed retrospectively.Results: No intraoperative death occurred, blood routine analysis improved (P < 0.05), the blood flow velocity (P < 0.05) and diameter (P < 0.05) of the left portal vein (LPV) significantly increased, the esophageal and gastric varices significantly relieved in 34 patients (P < 0.05), and better effects of earlier operations were demonstrated than the delayed ones (P < 0.05). During the period of follow-up from 6 to 64 months, the overall patency rate was 85.7% and the younger the age the better of the effect.Conclusion: Rex-bypass shunt combined with pericardial devascularization is a safe and effective procedure for prehepatic portal hypertension caused by CTPV.

Extrahepatic portal vein obstruction and portal vein thrombosis in special situations: Need for a new classification.

Extrahepatic portal vein obstruction is a vascular disorder of liver, which results in obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic portal vein, splenic vein, or superior mesenteric vein. Portal vein obstruction due to chronic liver disease, neoplasm, or postsurgery is a separate entity and is not the same as extrahepatic portal vein obstruction. Patients with extrahepatic portal vein obstruction are generally young and belong mostly to Asian countries. It is therefore very important to define portal vein thrombosis as acute or chronic from management point of view. Portal vein thrombosis in certain situations such as liver transplant and postsurgical/liver transplant period is an evolving area and needs extensive research. There is a need for a new classification, which includes all areas of the entity. In the current review, the most recent literature of extrahepatic portal vein obstruction is reviewed and summarized.

An Unusual Cause of Upper Gastrointestinal Bleeding: Duodenal Vascular Ectasia after Splenectomy

Cavernomatous transformation of portal vein (CTPV) is characterized by a collateral network of veins in the hepatic hilum that bypasses an obstructed or occluded extrahepatic portal vein. We report a case of CTPV, 25 years after splenectomy in a 39-year-old man with acute and chronic blood loss from duodenal vascular ectasia without esophageal or gastric varices or portal hypertensive gastropathy. This case demonstrates that distal part of duodenum assessment is important in gastrointestinal bleeding of patients with CTPV. Abbreviations: GI: Gastrointestinal; CTPV: Cavernomatous transformation of portal vein; CT: Computed tomography; PHD: Portal hypertensive duodenopathy.

Portal Revascularization in the Setting of Cavernous Transformation Through a Paracholedocal Vein: A Case Report

Diffuse thrombosis of the entire portal system (PVT) and cavernomatous transformation of the portal vein (CTPV) represents a demanding challenge in liver transplantation. We present the case of a patient with nodular regenerative hyperplasia and recurrent episodes of type B hepatic encephalopathy concomitant with PVT as well as CTPV, successfully treated with orthotopic liver transplantation. The portal inflow to the graft was carried out through the confluence of 2 thin paracholedochal varicose veins, obtaining good early graft function and recovery of the encephalopatic episodes. This alternative should be kept in mind as an option to assure hepatopetal splanchnic flow in those cases of diffuse thrombosis and cavernomatous transformation of portal vein.

Portal hypertension in infants and children.

The surgical care of infants and children with portal hypertension has been reported infrequently. It was our desire to add our experience to that already reported and to consolidate what appears to us to be the basic problems facing the surgeon in the diagnosis and care of these children. PATHOGENESIS The classification of Whipple 22 with slight modification serves equally well in children as in adults. I. Prehepatic A. Obstruction of inferior vena cava—congenital II. Hepatic A. Portal cirrhosis B. Thrombosis of hepatic veins (Chiari's syndrome) III. Posthepatic A. Congenital 1. Cavernomatous transformation of portal vein 2. Congenital stricture and obliteration of portal vein B. Acquired thrombosis of portal vein or its tributaries 1. Trauma 2. Infection 3. Idiopathic or spontaneous origin The posthepatic cases are the most distressing to the surgeon, and, although from a clinical standpoint they can be differentiated into congenital and acquired, they are too