Copyright
©The Author(s) 2020.
World J Gastroenterol. Nov 14, 2020; 26(42): 6582-6598
Published online Nov 14, 2020. doi: 10.3748/wjg.v26.i42.6582
Published online Nov 14, 2020. doi: 10.3748/wjg.v26.i42.6582
Table 1 Morgan and Superina extrahepatic shunts
Morgan and Superina extrahepatic shunts |
Type I: “Abernethy malformation” portal blood flow bypasses the liver entirely and empties into the IVC |
The splenic vein and SMV enter the IVC separately |
The splenic vein and SMV form common vessel before entering IVC |
Type II: Partial shunt where an “H type” connection between the portal system and the IVC. Some portal flow to the liver is still intact |
Table 2 Park’s classification of congenital intrahepatic portosystemic shunts
Park’s classification of congenital intrahepatic portosystemic shunts[5] |
Large connection of constant diameter from the right portal vein to the intrahepatic IVC |
Localized peripheral shunt from multiple or single communications between the peripheral branches of the portal and hepatic veins within one hepatic segment |
An aneurism between connecting peripheral portal and hepatic veins |
Multiple communications between peripheral and hepatic veins peripherally throughout the liver |
Patent ductus venosus |
Table 3 Hereditary hemorrhagic telangiectasia
Hereditary hemorrhagic telangiectasia (must have at least three of the following) |
Recurrent spontaneous epistaxis |
Mucocutaneous telangiectasia |
Family history of HHT |
Presence of visceral involvement |
Table 4 Vascular shunts associated with hereditary hemorrhagic telangiectasia
Vascular shunts associated with HHT | Associated systemic/Hepatic manifestations |
Arteriovenous | Hepatomegaly |
Pulmonary hypertension | |
High output cardiac failure | |
Biliary ischemia/biloma | |
Abdominal angina | |
Arterioportal | Focal nodular hyperplasia |
Non-cirrhotic portal hypertension | |
Hepatic encephalopathy | |
Portovenous | Hepatomegaly |
Hepatic encephalopathy | |
High output cardiac failure | |
Non-cirrhotic portal hypertension | |
Focal nodular hyperplasia |
Table 5 Hereditary hemorrhagic telangiectasia hepatic involvement treatment considerations
HHT hepatic involvement treatment considerations |
Symptom control: Iron deficiency, heart failure, esophageal varices |
Anti-VEGF antibodies (i.e., bevacizumab) |
Hepatic arterial embolization: Typically, an adult palliative option |
Liver transplant: In setting of extensive hepato-biliary necrosis or heart failure |
Table 6 Treatment considerations for hepatic hemangiomas
Treatment considerations for hepatic hemangiomas |
Monitoring for self-involution |
Propranolol (2-3 mg/kg/d) superior to corticosteroids or IV vincristine |
Surgical ligation or resection of internal or complex hemangiomas |
Enucleation for peripherally located hemangiomas |
Artery embolization or radiofrequency ablation for emergency bleeding or in preparation for surgical intervention |
Liver transplant for exceptionally large lesions or diffuse lesions, with severe complications such as heart failure and Kasabach-Merritt syndrome not amenable to medical management |
- Citation: Schmalz MJ, Radhakrishnan K. Vascular anomalies associated with hepatic shunting. World J Gastroenterol 2020; 26(42): 6582-6598
- URL: https://www.wjgnet.com/1007-9327/full/v26/i42/6582.htm
- DOI: https://dx.doi.org/10.3748/wjg.v26.i42.6582