Macro- and microcirculation patterns of intrahepatic blood flow changes in patients with hereditary hemorrhagic telangiectasia

Table 1 Basic characteristics of 18 patients with hereditary hemorrhagic telangiectasia

Case No.	Gender (M/F)	Age (yr)	HVaMs (yes/no)	Coexisting lesions	Malignant lesions (yes/no)	Location HVaMs
1	F	83	Yes	-	No	Diffuse
2	F	60	Yes	-	No	hilum;
						left hepatic lobe;
						segment 8
3	F	75	Yes	Aneurysm of the mesenteric vein	No	Hilum
4	F	50	Yes	-	No	Hilum;
						left hepatic lobe
5	F	36	Yes	FNH; cholecystolithiasis	No	Hilum;
						left hepatic lobe;
						segment 8
6	F	75	Yes	Sludge in the gall bladder;	No	Hilum
				Ectatic V. cava; ascites
7	F	58	Yes	Cholecystolithiasis	No	Hilum
8	F	65	Yes	-	No	Hilum;
						left hepatic lobe
9	F	66	Yes	Splenomegaly	No	Left hepatic lobe;
						segment 8
10	F	52	Yes	Steatosis hepatis	No	Segment 8
11	F	76	Yes	Cholecystolithiasis;	No	Hilum
				Hepatic cysts
12	F	49	Yes	Steatosis hepatis	No	Hilum;
						segment 8
13	F	55	Yes	Steatosis hepatis;	No	Segment 8
				Hepatic cysts
14	M	72	Yes	-	No	Hilum
15	F	21	Yes	High-flow angiomas	No	Hilum
16	M	69	Yes	High-flow angiomas	No	Segment 8
17	F	62	Yes	Ectatic Aorta abd.; hepatic cysts;	No	Hilum;
				Hepatomegaly;		segment 8
				Steatosis hepatis
18	F	46	Yes	Thrombosis of V. porta (cavernous transformation);	No	Left hepatic lobe;
				hepatic cysts;		segment 8
				bilioma

Table 2 Findings on B-Mode, color coded Doppler sonography and Power Doppler

Case No.	Shunt type (a-v/A-p/V-p)	Portal hyper-tension (yes/no)	Tortuousity extra-hepatic A. Hepatica (yes/no)	Vmax portal vein(cm/s)	Ri hepatic artery	Vmax hepatic artery(cm/s)	Intrahepatic arterial hyper-vascularization (yes/no)	Diameter common hepatic artery (mm)
1	a-v + a-p	No	No	45.0	0.66	105.5	Yes	7.4
2	a-v + a-p + v-p	Yes	Yes	32.3	0.60	111.7	Yes	19.5
3	a-v + a-p	No	Yes	20.0	0.56	148.1	No	15.5
4	a-v + a-p	Yes	Yes	20.0	0.55	96.0	Yes	9.5
5	a-v	No	No	44.0	0.59	81.0	No	9.7
6	a-v + a-p	No	Yes	36.0	0.56	145.5	Yes	13.8
7	a-v + a-p	No	Yes	40.0	0.52	60.2	Yes	14.2
8	a-v + a-p + v-p	Yes	Yes	18.0	0.51	109.2	Yes	13.7
9	a-v + a-p	No	Yes	22.8	0.55	86.7	Yes	13.2
10	a-v	No	Yes	27.0	0.62	92.5	No	13.3
11	a-v	No	Yes	20.0	0.56	87.9	No	8.7
12	a-v	No	Yes	26.6	0.64	245.7	Yes	8.2
13	a-v + a-p	No	Yes	26.9	0.62	218.3	Yes	9.9
14	a-v	No	Yes	25.4	0.65	40.5	No	7.8
15	a-v	No	Yes	27.6	0.64	46.0	No	12.2
16	a-v	No	No	25.2	0.60	86.7	No	10.0
17	a-v + a-p	Yes	Yes	30.0	0.50	123.8	Yes	7.8
18	a-v + a-p	Yes	Yes	29.6	0.35	112.1	Yes	12.0

RI: Resistive index; Vmax: Peak flow velocity.

Table 3 Considered sonographic criteria in 18 patients with hereditary hemorrhagic telangiectasia

Case No.	Caselitz criteria (2003)	Buscarini grading (2008)	“Color spots” Buonamico (2008) (Yes/No)
1	2 Major + 2 Minor	3	Yes
2	2 Major + 3 Minor	4	Yes
3	1 Major + 2 Minor	1	Yes
4	2 Major + 2 Minor	3	Yes
5	1 Major + 2 Minor	1	No
6	2 Major + 3 Minor	4	Yes
7	2 Major + 3 Minor	3	Yes
8	2 Major + 2 Minor	4	Yes
9	2 Major + 1 Minor	3	Yes
10	1 Major + 2 Minor	2	No
11	1 Major + 1 Minor	2	Yes
12	2 Major + 3 Minor	2	Yes
13	1 Major + 3 Minor	3	Yes
14	1 Major + 1 Minor	1	No
15	1 Major + 2 Minor	1	No
16	1 Major + 0 Minor	3	No
17	2 Major + 4 Minor	3	Yes
18	2 Major + 4 Minor	3	Yes