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
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
Pulmonary hypertension
High output cardiac failure
Biliary ischemia/biloma
Abdominal angina
ArterioportalFocal nodular hyperplasia
Non-cirrhotic portal hypertension
Hepatic encephalopathy
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