Surgical guidelines for the management of extra‐hepatic portal vein obstruction

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147

Portal vein thrombosis is an important cause of portal hypertension. PVT occurs in association with cirrhosis or as a result of malignant invasion by hepatocellular carcinoma or even in the absence of associated liver disease. With the current research into its genesis, majority now have an underlying prothrombotic state detectable. Endothelial activation and stagnant portal blood flow also contribute to formation of the thrombus. Acute non-cirrhotic PVT, chronic PVT (EHPVO), and portal vein thrombosis in cirrhosis are the three main variants of portal vein thrombosis with varying etiological factors and variability in presentation and management. Procoagulant state should be actively investigated. Anticoagulation is the mainstay of therapy for acute non-cirrhotic PVT, with supporting evidence for its use in cirrhotic population as well. Chronic PVT (EHPVO) on the other hand requires the management of portal hypertension as such and with role for anticoagulation in the setting of underlying prothrombotic state, however data is awaited in those with no underlying prothrombotic states. TIPS and liver transplant may be feasible even in the setting of PVT however proper selection of candidates and type of surgery is warranted. Thrombolysis and thrombectomy have some role. TARE is a new modality for management of HCC with portal vein invasion. ( J CLIN EXP HEPATOL 2015;5:22-40)

Section: Tipsmentioning

confidence: 99%

Portal Vein Thrombosis

Chawla

Bodh

2015

150

147

“…It is also important to note that previous work suggests that >90% patency can be achieved with meso-Rex shunt operations. 14 The patients from our institution did not fare this well, requiring numerous reoperations and long-term anti-coagulation for shunt thrombosis. These operations were not done at our institution, but were referred to a center that does a high volume of pediatric portal hypertension surgery.…”

Section: Conflicts Of Interestmentioning

confidence: 90%

“…With the development of novel surgical techniques, such as the meso-Rex bypass, some groups have suggested an aggressive surgical approach to patients with extrahepatic PVT. 11,14,17 Conversely, numerous series have suggested that the vast majority of these pediatric patients do well long-term without surgery, even when they develop complications including bleeding from both esophageal and gastric varices. [18][19][20] In all, our work supports the contention that a rather conservative approach to the management of noncirrhotic portal hypertension is warranted.…”

Section: Conflicts Of Interestmentioning

confidence: 99%

“…2 There are various surgical interventions available that attempt to improve the portal flow, including the meso-Rex bypass, 11 distal splenorenal shunt, 12,13 splenectomy followed by proximal splenorenal shunt, and the mesocaval shunt. 14 The aforementioned procedures are all significant operations and have a broad range of potential complications, including shunt thrombosis. Consideration of these risks is important, given that many patients have been reported to do well long-term without any surgical intervention.…”

mentioning

confidence: 99%

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Progression of Noncirrhotic Portal Hypertension in a Pediatric Population

Wojcik¹,

Zahid²,

Cai³

et al. 2011

P ortal hypertension is classically caused by cirrhotic liver disease. However, there are several etiologies of portal hypertension that do not coincide with liver failure. Noncirrhotic portal hypertension may manifest clinically by upper gastrointestinal (GI) bleeding from esophageal varices, leukopenia, and thrombocytopenia from splenomegaly. 1,2 While studies have shown that certain liver functions are affected as a result of reduced portal venous flow, ascites and clinical encephalopathy are not common given the lack of intrinsic liver dysfunction.1-8 Noncirrhotic etiologies of portal hypertension are classified as prehepatic, hepatic, and posthepatic. Prehepatic causes are associated with thrombosis within the portal venous system. Hepatic causes include noncirrhotic portal fibrosis and schistosomiasis. Finally, Budd-Chiari syndrome, inferior vena cava (IVC) thrombosis/occlusion, and constrictive pericarditis are posthepatic etiologies of portal hypertension.In pediatric patients, portal vein thrombosis (PVT), or extrahepatic portal vein obstruction (EHPVO), is the most common cause of noncirrhotic portal hypertension. The pathophysiology of PVT is poorly understood, although studies have identified several pre-disposing factors, including neonatal umbilical vein catheterization and hypercoagulable states.9,10 Indications for surgical intervention on these complex children are controversial, but include refractory variceal hemorrhage, severe thrombocytopenia, severe leukopenia, encephalopathy, and porto-pulmonary hypertension.2 There are various surgical interventions available that attempt to improve the portal flow, including the meso-Rex bypass, 11 distal splenorenal shunt, 12,13 splenectomy followed by proximal splenorenal shunt, and the mesocaval shunt. 14 The aforementioned procedures are all significant operations and have a broad range of potential complications, including shunt thrombosis. Consideration of these risks is important, given that many patients have been reported to do well long-term without any surgical intervention. 1,15While surgery is often recommended following a GI bleed requiring blood transfusion, specific indications for surgical intervention are unclear. Further, with the development of advanced endovascular techniques, clinical decision-making has become even more complex. We have recently established a pediatric portal hypertension program at our institution, which includes hepatologists, interventional radiologists, radiologists, and surgeons. As we develop our own protocols for the management of children with noncirrhotic portal hypertension, we have reviewed Background/Objectives: The optimal management of children with noncirrhotic portal hypertension is controversial. Some groups suggest early and aggressive surgical intervention, while others report long-term success with conservative management. Methods: We conducted a retrospective study of 26 patients with noncirrhotic portal hypertension treated at our institution. We compared platelet counts, white blood cell (WBC...

“…82 Rex shunt (mesenterico-left portal vein bypass) in children who have EHPVO is considered to be the treatment of choice because they restore normal portal flow to the liver. 83 Symptomatic portal hypertensive biliopathy is a definite indication for intervention and depends upon its presentation. Definitive treatment may be in the form of In childhood and early adult life, variceal bleeding, growth retardation, and hypersplenism are the main presenting clinical problems.…”

Section: Managementmentioning

confidence: 99%

Noncirrhotic Portal Hypertension

Rajekar¹,

Vasishta²,

Chawla³

et al. 2011

Portal hypertension is characterized by an increase in portal pressure (> 10 mmHg) and could be a result of cirrhosis of the liver or of noncirrhotic diseases. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension (NCPH) must be considered. The prognosis of this disease is much better than that of cirrhosis. Noncirrhotic diseases are the common cause of portal hypertension in developing countries, especially in Asia. NCPH is a heterogeneous group of diseases that is due to intrahepatic or extrahepatic etiologies. In general, the lesions in NCPH are vascular in nature and can be classified based on the site of resistance to blood flow. In most cases, these disorders can be explained