å¡ CASE REPORT å¡ Portal Hepatic Venous Shunt via an Intrahepatic Portal Vein Aneurysm Takenori Sakai, Toshikazu Katoh, Kouzou Asada, Kan Yagi and Ryouichi Yoshida A rare case of portal hepatic venous shunt (PHVS) via an intrahepatic portal vein aneurysm (PVA) is presented. A 66-year-old man was admitted for examination of a mass in the liver. Ultrasonography demonstrated a cystic lesion (15 mm in diameter) at the posterior superior segment of the right hepatic lobe which communicated with the right portal vein (RPV) and right hepatic vein (RHV). Superior mesenteric portography showed a biloculate aneurysm in RPV and PHVS. Color doppler ultrasonography indicated that flow in the tracts entered the aneurysmal cavity from RPV and drained into RHV. (Internal Medicine 31: 899-903, 1992) Key words: color doppler ultrasonography, hyperammoniemia, hepatic fibrosis
Introduction Portal hepatic venous shunts (PHVS) within hepatic parenchyma are rare, and PHVS via a portal vein aneurysm (PVA) are extremely rare: only ten cases have been reported to our knowledge. We present a case of PHVS via an intrahepatic PVA. The etiology, clinical features, and treatment are discussed and previously published cases are reviewed. Case Report
cavity from the right portal vein and draining into the right hepatic vein (Fig. 2). Plain computed tomography (CT) indicated a 20 x 30mm well circumscribed, bi loculate, low density area in the right lobe (Fig. 3-a). By enhanced CT, the mass was homogenously enhanced and its density was the same as that of the inferior vena cava (Fig. 3-b). Magnetic resonant imaging (MRI) showed an area with no signal, and communication with the right portal vein and right hepatic vein was suspected by an oblique view of coronal MRI (Fig. 4). Superior mesenteric portography demonstrated a 15 X 15 mm bi loculate aneurysm in the right portal vein and an early portal hepatic venous shunt (Fig. 5). Laparoscopy showed non-cirrhotic liver (Fig. 6). A liver needle biopsy speci men indicated a mild hepatic fibrosis, without cirrhosis The patient remained stable and showed no signs of (Fig. 7). Further follow-up by US and CDU two months encephalopathy thus was treated conservatively, later indicated noand further change in size or flow pattern. without surgery.
A previously healthy 66-year-old man was referred to our hospital for examination of a mass in the liver detected by ultrasonography (US) on May 21, 1990. He had consumed 120 ml of ethanol (Japanese sake) per day for the past 10 years. His past and familial history showed no remarkable findings. He has no complaints at present. A mass lesion in the liver was detected by medical examination. On admission, physical examination Discussion was normal, and there were no neurological signs, ab dominal bruits, or indications of encephalopathy. Liver Portosystemic venous collaterals in patients with function tests were normal except that the venous blood portal hypertension are generally located in the extra ammonia level was slightly elevated (Table 1). US de hepatic portion. Postmortem studies occasionally show monstrated a cystic lesion (15mm in diameter) in the minute intrahepatic shunts (1-2mm in diameter) be posterior superior segment of the right hepatic lobe tween the portal and hepatic veins in cirrhotic livers (1), which communicated with the right portal vein and right otherwise portal hepatic venous shunts (PHVS) within hepatic vein (Fig. 1). Color doppler ultrasonography From the Department of Internal Medicine, Yawatahama City General Hospital, Yawatahama (CDU) showed flow in the tracts the aneurysmal Received for publication June 10, entering 1991; Accepted for publication March 13, 1992 Reprint requests should be addressed to Dr. Takenori Sakai, the Department of Internal Medicine, Yawatahama City General Hospital, 1-63 Ohira, Yawatahama, Ehime 796, Japan Internal
Medicine
Vol. 31, No. 7 (July
1992)
899
S ak a i e t a l
T able 1. L abo ratory D ata o n A d m ission U rinaly sis F aeces O ccu lt b loo d
n .p .
(- )
P eri ph eral b lo od RBC 387 x lO4 /m m 3 Hb Ht W BC
12.0 g/dl 35.2 % 4,200 /m m 3
st. seg .
ll % 47 % 3% b aso . 0% ly . 31 % m on o . 8% P lat. 25 .8 x lO4/m m 3 C o agu lation B leeding tim e 2 m in A PT T 22 .7 sec PPT 10 .5 sec H pt 102 .9 %
E SR
25/58 m m
B lo od chem istry T .B i1 0 .5 m g/dl G OT G PT LD H
27 K -U 2 3 K -U 39 6 W -U
ALP y-G T P LA P
4.2 K -A IO m U /m l lO4 G -R
C h -E ZT T TTT T .Pro t A lb . orr glb o-2-glb
0.6 1 A p H 6U 1U 6.4 g/dl 57 % 3.7 % 13.2 %
/3-glb 7 .5 % y-glb 18 .6 % T .C ho i 146 m g /dl S-A m ylase 362 IU /1 U -A m ylase 664 IU /1 NH 3 90 ug/dl Fe ll ug/dl
BUN
13 m g/d l
C reatin ine U ric acid Na
1.O m g/dl 4 .1m g/dl 138 m E q/l
K C1
4 .0 m E q/] 102 m E q/l
Serolo gy CR P H B sA g H B sA b H CV A b A NA A M A L E test IgG IgA IgM IgE A FP C A 19-9 F erri tin R 15 IC G
(ｱ ) (- ) 24 (- ) x 40 (- ) (- ) 1552 m g/d l 35 3 m g/d l 57 m g/d l 2 IU /m l 3.1 n g/m l 9 U /m l 186 n g/m l 5%
Fig. 1. US of the liver from the right transverse view (a) and intercostal view (b) demonstrating a cystic lesion (open arrows) in the posterior superior segment of the right hepatic lobe. The lesion com municated with the posterior superior branch of right portal vein (arrowheads) and right hepatic vein (solid arrows). the hepatic parenchyma are rare. PHVS via portal vein aneurysms (PVA) are extremely rare: only ten cases (2 8) have been reported in the literature (Table 2), seven PHVS were in the right hepatic lobe. Only one had symptoms directly related to the shunt (encephalopathy) , the other nine were asymptomatic. Four cases had hyper The causes of Eight PHVScases are unknown. Kozuka et al ammoniemia. had chronic liver disease. consider the abnormality to be acquired since microscopic 900
Fig. 2. CDU of the liver from the right intercostal view showing flow in the tracts entering the aneurysmai cavity from the posterior superior branch of the right portal vein (red) and draining into the right hepatic vein (blue). Internal
Medicine
Vol. 31, No. 7 (July
1992)
A Case of Portal Hepatic Venous Shunt
Fig. 3. a) Plain CT indicating a biloculate structure of low densityFig. 4. a) Axial MRI showed no signal area (arrows) in the in the posterior segment of the right lobe, b) Biloculate structure was posterior superior segment of the right lobe, b) Oblique view of homogenously enhanced on enhanced CT and its density was the same coronal MRI showed no signal area (open arrows). Communication as that of the inferior vena cava. with the portal vein (arrowheads) and the right hepatic vein (solid arrows) was suspected. examination has shown both the muscular layer and elastic lumellae to disappear abruptly from the wall of the shunt and the cerebral manifestation could be detected only in older-age patients (9). However, con genital origin of this abnormality also has been postulated (4, 10). One embryologic explanation for shunts in the right lobe is the persistence of high flow communication between the omphalomesenteric venous system and the right horn of the sinus venosus (10). Decreasing tolerance toward toxic metabolites with age may possibly explain its late clinical manifestation (4, 10). In the present case, the portal aneurysm may have preceded the venous shunt followed by rupture into the hepatic vein to establish methods the communication. cause of the portal Different were used toThe demonstrate PHVS aneurysm is still uncertain (2). via PVA, including angiography, US, CT, MRI, and, in one case, CDU. Raskin et al found a congenital malfor mation with abnormal communication between branches of the portal hepatic angiography (10).with Minakuchi et and al noted thatveins MRIby shows every image Internal
Medicine
Vol. 31, No. 7 (July
1992)
Fig. 5. Superior mesenteric portography demonstrated a 15 x 15 mm biloculate aneurysm (open arrows) in the right portal vein (arrowheads) and an early portal hepatic venous shunt (solid arrows). 901
Sakai et al
Fig. 6. Laparoscopic findings of the right hepatic lobe (a, d), the left hepatic lobe (b), and close-up view of the right hepatic lobe (c) showed increased lobular markings.
Fig. 7. Histological finding of the liver needle biopsy specimen. Portal fibrosis was observed, (a: Azan stain X20, b: silver impregnation stain xlOO, c: HE stain xlOO, d: HE stain x50). 902
Internal
Medicine
Vol. 31, No. 7 (July
1992)
A Case of Portal Hepatic Venous Shunt Table 2. Reports of Portal Hepatic Venous Shunt Via an Intrahepatic Portal Vein Aneurysm ,.. 1 1 1 hyper-NH3 portal Complicated _ Reporter case age sex liversegment encephalopathy Jr. , à" ,. Treatment , , r emia hypertension diseaseshunt (year) ?
1 75 M rightant.inf. PV5-^MHV
+
T ak ayasu (1984 ) H origu ch i (1984 )
L C ,H C C
?
LC
3 60 M rightpos.sup. PV7^RHV
?
CH
74 F left LPV^ LHV
+
+
63 F right RPV ^ RHV
+
B
61 M left LPV-^ LHV
+
+
2 53 M left LPV-^ LHV
?
alco ho lic liv er injury
9
CH
+
LC
?
?
CH
+
9
hep atic fi b rosis
7 37 F rightpos.sup. PV7^RHV 8 32 F right inf. RPV^ RHV 9 73 F rightant.inf. PV5-^RHV 10 66 M rightpos.sup. PVy^RHV
蝣
C hagno n (1986)
T akeuchi (1987) T suk uda (1988) M ario (1988) M in aku chi (1989) o ur case (199 1)
liver segment; ant: anterior, pos: posterior, sup: superior, inf: inferior shunt; LPV: left portal vein, RPV: right portal vein, PV5: right anterior inferior branch, PV7: right posterior superior branch, MHV: midhepatic vein, RHV: right hepatic vein, LHV: left hepatic vein, Complicated disease; LC is liver cirrhosis, HCC is hepatocellular carcinoma, CH is chronic hepatitis options of the coronal, axial and sagital plane, and provide data on the continuity between the portal vein and aneurysmal cavity (8). CDU is useful for demonstrat ing small fistulous tracts between iatrogenic aneurysmal portal hepatic venous fistula (7). In our case, CDU showed flow in the tracts entering the aneurysmal cavity from the right portal vein and draining into right hepatic vein. In nearly all cases, diagnosis has been made by angiography following all other examinations. The pre sent case was diagnosed by US and CDU. If abnormal communication between branches of the portal and hepatic veins can be demonstrated clearly by US and CDU, will cease to be used for diagnosis in To date,angiography there is no report of serious complication the future. such as rupture or thrombosis, and prognosis is good. Operation has been reported in only one case in which encephalopathy could not be controlled by conservative means (4). The present case with no serious complication or no complaints did not require surgical treatment and will continue to be follow-up by US studies.
Internal
Medicine
Vol. 31, No. 7 (My 1992)
References 1) Popper H, Elias H, Petty DE. Vascular pattern of cirrhotic liver. Am J Clin Pathol 22: 717, 1952. 2) Takayasu K, Moriyama N, Shima Y, et al. Spontaneous portal hepatic venous shunt via an intrahepatic portal vein aneurysm. Gastroenterology 86: 945, 1984. 3) Horiguchi Y, Kitano T, Taguchi H. Two cases of intrahepatic portasystemic shunt. Acta Hepatologica Japonica 25: 794, 1984. 4) Chagnon SF, Vallee CA, Barge J, et al. Aneurysmal portohepatic venous fistula: report of two cases. Radiology 159: 693, 1986. 5) Takeuchi K, Imai Y, Kamisako T, et al. A case ofthe intrahepatic portal vein aneurysm with portal-hepatic venous shunt. Diagnosis and Treatment 75: 1362, 1987. 6) Tsukuda M, Yokomizo Y, Nanba T, et al. Intrahepatic portal vein aneurysm with portal-hepatic venous shunt: Case report. Nippon Acta Radiol 48: 304, 1988. 7) Mario B, Donald GM, Lancence N, et al. Iatrogenic aneurysmal portal-hepatic venous fistula: diagnosis by color doppler imaging. J Ultrasound Med 7: 457, 1988. 8) Minakuchi K, Takada K, Nakamura K. Two cases of venous aneurysm of the intrahepatic portal vein branch: MR demon stration. Osaka CityR, Medical Journal 49, 1989. 9) Kozuka S, Sassa Kakumu S. An35: enormous intrahepatic shunt between portal vein and hepatic one. Angiography 26: 365, 1975. 10) Raskin NH, Price JB, Fishman RA. Portal systemic encephalo pathy due to congenital intrahepatic shunts. N Engl J Med 270: 225, 1964.
903
Introduction Portal hepatic venous shunts (PHVS) within hepatic parenchyma are rare, and PHVS via a portal vein aneurysm (PVA) are extremely rare: only ten cases have been reported to our knowledge. We present a case of PHVS via an intrahepatic PVA. The etiology, clinical features, and treatment are discussed and previously published cases are reviewed. Case Report
cavity from the right portal vein and draining into the right hepatic vein (Fig. 2). Plain computed tomography (CT) indicated a 20 x 30mm well circumscribed, bi loculate, low density area in the right lobe (Fig. 3-a). By enhanced CT, the mass was homogenously enhanced and its density was the same as that of the inferior vena cava (Fig. 3-b). Magnetic resonant imaging (MRI) showed an area with no signal, and communication with the right portal vein and right hepatic vein was suspected by an oblique view of coronal MRI (Fig. 4). Superior mesenteric portography demonstrated a 15 X 15 mm bi loculate aneurysm in the right portal vein and an early portal hepatic venous shunt (Fig. 5). Laparoscopy showed non-cirrhotic liver (Fig. 6). A liver needle biopsy speci men indicated a mild hepatic fibrosis, without cirrhosis The patient remained stable and showed no signs of (Fig. 7). Further follow-up by US and CDU two months encephalopathy thus was treated conservatively, later indicated noand further change in size or flow pattern. without surgery.
A previously healthy 66-year-old man was referred to our hospital for examination of a mass in the liver detected by ultrasonography (US) on May 21, 1990. He had consumed 120 ml of ethanol (Japanese sake) per day for the past 10 years. His past and familial history showed no remarkable findings. He has no complaints at present. A mass lesion in the liver was detected by medical examination. On admission, physical examination Discussion was normal, and there were no neurological signs, ab dominal bruits, or indications of encephalopathy. Liver Portosystemic venous collaterals in patients with function tests were normal except that the venous blood portal hypertension are generally located in the extra ammonia level was slightly elevated (Table 1). US de hepatic portion. Postmortem studies occasionally show monstrated a cystic lesion (15mm in diameter) in the minute intrahepatic shunts (1-2mm in diameter) be posterior superior segment of the right hepatic lobe tween the portal and hepatic veins in cirrhotic livers (1), which communicated with the right portal vein and right otherwise portal hepatic venous shunts (PHVS) within hepatic vein (Fig. 1). Color doppler ultrasonography From the Department of Internal Medicine, Yawatahama City General Hospital, Yawatahama (CDU) showed flow in the tracts the aneurysmal Received for publication June 10, entering 1991; Accepted for publication March 13, 1992 Reprint requests should be addressed to Dr. Takenori Sakai, the Department of Internal Medicine, Yawatahama City General Hospital, 1-63 Ohira, Yawatahama, Ehime 796, Japan Internal
Medicine
Vol. 31, No. 7 (July
1992)
899
S ak a i e t a l
T able 1. L abo ratory D ata o n A d m ission U rinaly sis F aeces O ccu lt b loo d
n .p .
(- )
P eri ph eral b lo od RBC 387 x lO4 /m m 3 Hb Ht W BC
12.0 g/dl 35.2 % 4,200 /m m 3
st. seg .
ll % 47 % 3% b aso . 0% ly . 31 % m on o . 8% P lat. 25 .8 x lO4/m m 3 C o agu lation B leeding tim e 2 m in A PT T 22 .7 sec PPT 10 .5 sec H pt 102 .9 %
E SR
25/58 m m
B lo od chem istry T .B i1 0 .5 m g/dl G OT G PT LD H
27 K -U 2 3 K -U 39 6 W -U
ALP y-G T P LA P
4.2 K -A IO m U /m l lO4 G -R
C h -E ZT T TTT T .Pro t A lb . orr glb o-2-glb
0.6 1 A p H 6U 1U 6.4 g/dl 57 % 3.7 % 13.2 %
/3-glb 7 .5 % y-glb 18 .6 % T .C ho i 146 m g /dl S-A m ylase 362 IU /1 U -A m ylase 664 IU /1 NH 3 90 ug/dl Fe ll ug/dl
BUN
13 m g/d l
C reatin ine U ric acid Na
1.O m g/dl 4 .1m g/dl 138 m E q/l
K C1
4 .0 m E q/] 102 m E q/l
Serolo gy CR P H B sA g H B sA b H CV A b A NA A M A L E test IgG IgA IgM IgE A FP C A 19-9 F erri tin R 15 IC G
(ｱ ) (- ) 24 (- ) x 40 (- ) (- ) 1552 m g/d l 35 3 m g/d l 57 m g/d l 2 IU /m l 3.1 n g/m l 9 U /m l 186 n g/m l 5%
Fig. 1. US of the liver from the right transverse view (a) and intercostal view (b) demonstrating a cystic lesion (open arrows) in the posterior superior segment of the right hepatic lobe. The lesion com municated with the posterior superior branch of right portal vein (arrowheads) and right hepatic vein (solid arrows). the hepatic parenchyma are rare. PHVS via portal vein aneurysms (PVA) are extremely rare: only ten cases (2 8) have been reported in the literature (Table 2), seven PHVS were in the right hepatic lobe. Only one had symptoms directly related to the shunt (encephalopathy) , the other nine were asymptomatic. Four cases had hyper The causes of Eight PHVScases are unknown. Kozuka et al ammoniemia. had chronic liver disease. consider the abnormality to be acquired since microscopic 900
Fig. 2. CDU of the liver from the right intercostal view showing flow in the tracts entering the aneurysmai cavity from the posterior superior branch of the right portal vein (red) and draining into the right hepatic vein (blue). Internal
Medicine
Vol. 31, No. 7 (July
1992)
A Case of Portal Hepatic Venous Shunt
Fig. 3. a) Plain CT indicating a biloculate structure of low densityFig. 4. a) Axial MRI showed no signal area (arrows) in the in the posterior segment of the right lobe, b) Biloculate structure was posterior superior segment of the right lobe, b) Oblique view of homogenously enhanced on enhanced CT and its density was the same coronal MRI showed no signal area (open arrows). Communication as that of the inferior vena cava. with the portal vein (arrowheads) and the right hepatic vein (solid arrows) was suspected. examination has shown both the muscular layer and elastic lumellae to disappear abruptly from the wall of the shunt and the cerebral manifestation could be detected only in older-age patients (9). However, con genital origin of this abnormality also has been postulated (4, 10). One embryologic explanation for shunts in the right lobe is the persistence of high flow communication between the omphalomesenteric venous system and the right horn of the sinus venosus (10). Decreasing tolerance toward toxic metabolites with age may possibly explain its late clinical manifestation (4, 10). In the present case, the portal aneurysm may have preceded the venous shunt followed by rupture into the hepatic vein to establish methods the communication. cause of the portal Different were used toThe demonstrate PHVS aneurysm is still uncertain (2). via PVA, including angiography, US, CT, MRI, and, in one case, CDU. Raskin et al found a congenital malfor mation with abnormal communication between branches of the portal hepatic angiography (10).with Minakuchi et and al noted thatveins MRIby shows every image Internal
Medicine
Vol. 31, No. 7 (July
1992)
Fig. 5. Superior mesenteric portography demonstrated a 15 x 15 mm biloculate aneurysm (open arrows) in the right portal vein (arrowheads) and an early portal hepatic venous shunt (solid arrows). 901
Sakai et al
Fig. 6. Laparoscopic findings of the right hepatic lobe (a, d), the left hepatic lobe (b), and close-up view of the right hepatic lobe (c) showed increased lobular markings.
Fig. 7. Histological finding of the liver needle biopsy specimen. Portal fibrosis was observed, (a: Azan stain X20, b: silver impregnation stain xlOO, c: HE stain xlOO, d: HE stain x50). 902
Internal
Medicine
Vol. 31, No. 7 (July
1992)
A Case of Portal Hepatic Venous Shunt Table 2. Reports of Portal Hepatic Venous Shunt Via an Intrahepatic Portal Vein Aneurysm ,.. 1 1 1 hyper-NH3 portal Complicated _ Reporter case age sex liversegment encephalopathy Jr. , à" ,. Treatment , , r emia hypertension diseaseshunt (year) ?
1 75 M rightant.inf. PV5-^MHV
+
T ak ayasu (1984 ) H origu ch i (1984 )
L C ,H C C
?
LC
3 60 M rightpos.sup. PV7^RHV
?
CH
74 F left LPV^ LHV
+
+
63 F right RPV ^ RHV
+
B
61 M left LPV-^ LHV
+
+
2 53 M left LPV-^ LHV
?
alco ho lic liv er injury
9
CH
+
LC
?
?
CH
+
9
hep atic fi b rosis
7 37 F rightpos.sup. PV7^RHV 8 32 F right inf. RPV^ RHV 9 73 F rightant.inf. PV5-^RHV 10 66 M rightpos.sup. PVy^RHV
蝣
C hagno n (1986)
T akeuchi (1987) T suk uda (1988) M ario (1988) M in aku chi (1989) o ur case (199 1)
liver segment; ant: anterior, pos: posterior, sup: superior, inf: inferior shunt; LPV: left portal vein, RPV: right portal vein, PV5: right anterior inferior branch, PV7: right posterior superior branch, MHV: midhepatic vein, RHV: right hepatic vein, LHV: left hepatic vein, Complicated disease; LC is liver cirrhosis, HCC is hepatocellular carcinoma, CH is chronic hepatitis options of the coronal, axial and sagital plane, and provide data on the continuity between the portal vein and aneurysmal cavity (8). CDU is useful for demonstrat ing small fistulous tracts between iatrogenic aneurysmal portal hepatic venous fistula (7). In our case, CDU showed flow in the tracts entering the aneurysmal cavity from the right portal vein and draining into right hepatic vein. In nearly all cases, diagnosis has been made by angiography following all other examinations. The pre sent case was diagnosed by US and CDU. If abnormal communication between branches of the portal and hepatic veins can be demonstrated clearly by US and CDU, will cease to be used for diagnosis in To date,angiography there is no report of serious complication the future. such as rupture or thrombosis, and prognosis is good. Operation has been reported in only one case in which encephalopathy could not be controlled by conservative means (4). The present case with no serious complication or no complaints did not require surgical treatment and will continue to be follow-up by US studies.
Internal
Medicine
Vol. 31, No. 7 (My 1992)
References 1) Popper H, Elias H, Petty DE. Vascular pattern of cirrhotic liver. Am J Clin Pathol 22: 717, 1952. 2) Takayasu K, Moriyama N, Shima Y, et al. Spontaneous portal hepatic venous shunt via an intrahepatic portal vein aneurysm. Gastroenterology 86: 945, 1984. 3) Horiguchi Y, Kitano T, Taguchi H. Two cases of intrahepatic portasystemic shunt. Acta Hepatologica Japonica 25: 794, 1984. 4) Chagnon SF, Vallee CA, Barge J, et al. Aneurysmal portohepatic venous fistula: report of two cases. Radiology 159: 693, 1986. 5) Takeuchi K, Imai Y, Kamisako T, et al. A case ofthe intrahepatic portal vein aneurysm with portal-hepatic venous shunt. Diagnosis and Treatment 75: 1362, 1987. 6) Tsukuda M, Yokomizo Y, Nanba T, et al. Intrahepatic portal vein aneurysm with portal-hepatic venous shunt: Case report. Nippon Acta Radiol 48: 304, 1988. 7) Mario B, Donald GM, Lancence N, et al. Iatrogenic aneurysmal portal-hepatic venous fistula: diagnosis by color doppler imaging. J Ultrasound Med 7: 457, 1988. 8) Minakuchi K, Takada K, Nakamura K. Two cases of venous aneurysm of the intrahepatic portal vein branch: MR demon stration. Osaka CityR, Medical Journal 49, 1989. 9) Kozuka S, Sassa Kakumu S. An35: enormous intrahepatic shunt between portal vein and hepatic one. Angiography 26: 365, 1975. 10) Raskin NH, Price JB, Fishman RA. Portal systemic encephalo pathy due to congenital intrahepatic shunts. N Engl J Med 270: 225, 1964.
903
