Diagnostic challenges in non-cirrhotic portal hypertension - porto sinusoidal vascular disease

Table 1 Histological differences between porto-sinusoidal vascular disease, extrahepatic portal vein obstruction and cirrhosis

	PSVD	EHPVO	Cirrhosis
Portal and periportal zone
Small portal vein obliteration (“hepato-portal sclerosis", phlebosclerosis)	++	+/-	-
Periportal shunt vessel	++	+	-
Fibrous septa	+/-; without bridging1	-	++; with bridging delimitating nodules
Inflammation	-	-	++
Portal biliopathy	-	+/-	-
Liver lobule (parenchyma)
Perisinusoidal fibrosis	+	-	+/-
Sinusoidal dilatation	++	++	-
Nodular regenerative hyperplasia	+	+	-
Hepatocyte atrophy	+	+	-
Centrilobular zone
Central vein dilatation	++	+		-
Perivenular fibrosis	++	+		-

¹Incomplete septal fibrosis. ++: Almost always present; +: Usually present; +/–: Occasionally present; –: Usually absent; PSVD: Porto-sinusoidal vascular disease; EHPVO: Extrahepatic portal vein obstruction.

Table 2 Main invasive/non invasive characteristics found in porto-sinusoidal vascular disease, extrahepatic portal vein obstruction and cirrhosis

Diagnostic tools	PSVD	EHPVO	Cirrhosis
Invasive tools
Liver biopsy	Predominant vascular anomalies	Predominant vascular anomalies	Predominant architectural changes and fibrosis
Hepatic hemodinamics	FHVP N	FHVP N	FHVP N
	WHVP ↓	WHVP ↓	WHVP ↑
	HVPG N or slightly ↑	HVPG N	HVPG ↑
	Frequent vein-to-vein communications	Infrequent vein-to-vein communications	Infrequent vein-to-vein communications
	Hyperdynamic circulatory state	Hyperdynamic circulatory state	Hyperdynamic circulatory state
Endoscopic findings	Esophageal varices	Esophageal varices	Esophageal varices
	Gastric varices (GOV1, GOV2) more common	Gastric varices more common (especially IGV1, IGV2)	Gastric varices less common
	Portal hypertensive gastropathy less common	Portal hypertensive gastropathy less common	Portal hypertensive gastropathy more common
Non invasive tools
Ultrasound examination
Liver	Normal/irregular surface	Normal/irregular surface	Irregular surface
	Homogeneous/innhomogenous parenchima	Homogeneous/innhomogenous parenchima	Innhomogenous
			Focal liver lesions
Portal veins	Dilatated, hyperechoic with thickened walls	Chronic thrombosis	Dilatated
	+/- spontaneous shunts	+/- cavernomatous transformation	+/- thrombosis
	+/- thrombosis	+/- portal biliopathy
Spleen	Often giant splenomegaly	Often giant splenomegaly	Splenomegaly
Porto systemic collaterals, +/- ascites
CEUS examination	Delayed periportal enhancement		Homogenous/heterogenous/delayed periportal enhancement
Cross-sectional imaging	Heterogeneous hepatic enhancement or paucity of the medium size portal branches	(1) Better characterization of the level and extension of thrombus; and (2) Better characterization of the portal biliopathy	Better characterization of the focal liver lesions
Elastography
Liver stiffenss	N or slightly ↑	N or slightly ↑ (less then in the PSVD)	↑ (has prognostic value)
Spleen stiffness	↑	↑	↑ (less then in PSVD or EHPVO)

PSVD: Porto-sinusoidal vascular disease; EHPVO: Extrahepatic portal vein obstruction; CEUS: Contrast-enhanced ultrasound; HVPG: Hepatic venous pressure gradient; WHVP: Wedged hepatic venous pressure; FHVP: Free hepatic vein press.