Vascular anomalies associated with hepatic shunting

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

IVC: Inferior vena cava; SMV: Superior mesenteric veins.

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

IVC: Inferior vena cava.

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

HHT: Hereditary hemorrhagic telangiectasia.

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

HHT: Hereditary hemorrhagic telangiectasia.

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

HHT: Hereditary hemorrhagic telangiectasia.

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

IV: Intravenous.