An intrahepatic arteriovenous fistula is usually ac-quired as a result of trauma, including a liver biopsy, orin association with hepatic tumors and rupturedaneurysms of the hepatic artery (1). A congenital hepato-portal arteriovenous fistula is extremely rare and thereare very few reports. The common clinical manifesta-tions are the features of portal hypertension such asbowel congestion, gastrointestinal bleeding, malabsorp-tion, ascites and splenomegaly (2). An intrahepatic por-tovenous shunt is also rare (3). Duplex Doppler sonogra-phy is the modality of choice for both diagnosing andevaluating abnormal vascular communications alongwith their hemodynamic consequences in the liver,which can be confirmed by angiography (4). We de-scribe a case of a male neonate who was diagnosed by

duplex Doppler sonography with a congenital hepato-portal arteriovenous fistula and portohepatic venousshunt associated with an intrahepatic portal veinaneurysm, which was confirmed by angiography.

Case Report

A 3740-g male neonate that was born after 41 weeks’gestation via a normal spontaneous vaginal delivery wasreferred to this hospital for an assessment of his tachyp-nea. A chest radiogram at birth showed a mild car-diomegaly, and the echocardiography revealed grade IIItricuspid regurgitation and a mild right-side heart en-largement. At one week of age, the baby exhibited mele-na, which required transfusions. There was no historyof jaundice and the examination showed no he-patosplenomegaly and cyanosis. The liver function testswere normal, but the hemoglobin level was 6.4 g/dl.Gray-scale ultrasonography of the abdomen demon-strated a 1.5 cm in diameter saccular dilatation of theright portal vein (Fig. 1A), and the color Doppler exami-

J Korean Radiol Soc 2005;53:103-106

─ 103 ─

Congenital Hepatoportal Arteriovenous Fistula associatedwith Intrahepatic Portal Vein Aneurysm and

Portohepatic Venous Shunt: Case Report 1

Jin-Hwa Lee, M.D., Tae Beom Shin, M.D., Myong Jin Kang, M.D.,Seong-Kuk Yoon, M.D., Jong-Young Oh, M.D., Jong Cheol Choi, M.D.,Byeong Ho Park, M.D., Sunseob Choi, M.D., Kyung Jin Nam, M.D.

1Department of Diagnostic Radiology, College of Medicine, Dong-AUniversity Received May 21, 2004 ; Accepted July 4, 2005Address reprint requests to : Tae Beom Shin, M.D., Department ofDiagnostic Radiology, Dong-A University Hospital, 1,3-ga, Dongdaeshin-dong, Seo-gu, Pusan 602-715, Korea. Tel. 82-51-240-5356 Fax. 82-51-253-4931

A congenital hepatoportal arteriovenous fistula associated with an intrahepatic por-tal vein aneurysm is a rare type of arteriovenous malformation. Only 14 pediatric cas-es have been reported to the best of the authors’ knowledge. An intrahepatic shunt be-tween the portal and systemic veins is also relatively rare. We report a case of a con-genital hepatoportal arteriovenous fistula associated with an intrahepatic portal veinaneurysm and a portohepatic venous shunt in a neonate who presented with tachyp-nea and melena.

Index words : Congenital hepatoportal arteriovenous fistula Portal vein, aneurysm Portohepatic venous shunt Ultrasound (US) Angiography

nation showed mixed colors and turbulent flow (Fig.1B). The blood flow in the portal vein was hepatopetalbut the duplex Doppler examination revealed an arteri-alized pattern (Fig. 1C). There were no features of portalhypertension including hepatosplenomegaly, venous

collaterals and ascites. A diagnosis of an intrahepaticportal vein aneurysm and arterioportal shunt betweenthe portal vein aneurysm and the hepatic artery was es-tablished. Digital subtraction abdominal aortographyconfirmed an intrahepatic arterioportal fistula with a

Jin-Hwa Lee, et al : Congenital Hepatoportal Arteriovenous Fistula associated with Intrahepatic Portal Vein Aneurysm and Portohepatic Venous Shunt

─ 104 ─

A B

C D

Fig. 1. A male neonate presenting with melena and tachypnea. A. Gray-scale sonography of the liver demonstrates an aneurys-mal dilatation of the right portal vein (arrow). B. Color Doppler sonography shows mixed colors and turbulentflow in the portal vein aneurysm. C. Duplex Doppler sonography depicts arterialized hepatopetalflows in the portal vein. D, E. Abdominal aortography shows an intrahepatic arteriopor-tal fistula with the portal vein aneurysm (arrow) in the earlyphase (D) and a portovenous shunt between portal veinaneurysm and hepatic vein (arrow) in late phase (E).

E

portal vein aneurysm (Fig. 1D). A portovenous shunt be-tween the portal vein aneurysm and the hepatic veinwas also shown on late phase of angiography (Fig. 1E).An attempt was made to embolize the feeder right he-patic artery using microcoils but the hepatic angiogramimmediately after embolization showed additional feed-ing vessels communicating with the portal veinaneurysm.

Discussion

A hepatoportal arteriovenous fistula may be congeni-tal or acquired. The acquired type is more common withcongenital lesions being extremely rare. A congenital he-patoportal fistula is a very rare cause of infantile portalhypertension (2). Most of pediatric patients with congen-ital arterioportal fistula show symptoms such as waterydiarrhea, melena and features of portal hypertension, in-cluding esophageal varices, splenomegaly and ascites(1). Poor circulation and necrotic changes in the smallbowel mucosa are believed to be due to a steal phenom-enon from the superior mesenteric artery to the arterio-portal venous fistula (5). Our patient exhibited melena,but did not show the signs of portal hypertension. Noneof the previously reported patients had heart failure,which might be due to the flow restrictions as a result ofthe hepatic sinusoids interposed between the lesion andthe right heart. Our case exhibited mild tachypnea andmild cardiomegaly but did not show the signs of heartfailure.

A portal vein aneurysm is also rare and the etiology ofthis lesion is believed to be congenital or secondary toportal hypertension, or it is associated with abnormalweakness of the vein wall (6). In our case, the portalvein aneurysm was demonstrated by gray-scale sonog-raphy, and duplex Doppler sonography identified the ar-teriovenous fistula to be an etiologic factor. Therefore,the portal vein aneurysm was considered to be sec-ondary to the hemodynamic changes in the portal ve-nous system by the hepatoportal arteriovenous fistula.

Duplex Doppler sonography is a rapid and noninva-sive initial diagnostic imaging modality (7). It maydemonstrate a saccular dilatation of the portal vein andshow high flow velocities with turbulence, an arterialfeeder from an enlarged hepatic artery branch with ahigh flow velocity, and pulsatile reversed hepatofugalflow in the portal vein. Gray-scale sonography demon-strates the features of portal hypertension, which in-clude splenomegaly, venous collaterals and ascites (4).

Hepatic arteriography confirms the diagnosis, anddemonstrates the vascular anatomy as well as the pre-cise characteristics and location of the fistula (2).

Various therapeutic approaches for an arterioportalfistula have been reported. Many believe that direct sur-gical closure is the most suitable technique for treatingextrahepatic fistulas, and embolization is a safe and ef-fective method for treating intrahepatic fistulas (4). Inour case, we failed to embolize the feeding hepaticartery on account of new additional feeding vesselscommunicating with the portal vein aneurysm. In ouropinion, the sac of the portal vein aneurysm may be em-bolized using the Guglielmi detachable coils (GDCs) inthe same manner used for the cerebral aneurysm em-bolization.

Pediatric portovenous fistulas are always associatedwith many other malformations (8). Park at al. (9) arbi-trarily categorized portohepatic venous shunts into fourdifferent morphologic types. The first and most com-mon type is a single large tube with a constant diameter,which connects the right portal vein to the inferior venacava. Most patients with this type of intrahepatic por-tosystemic shunt show clinical evidence of liver cirrho-sis and portal hypertension. The second type is a local-ized peripheral shunt in the branches of the portal andhepatic veins in a single hepatic segment. The third typeis the aneurysmal type, where the peripheral portal andhepatic veins are connected through the aneurysm. Thefourth type has diffuse multiple communications be-tween the peripheral portal veins and the hepatic veinsin both lobes. Our case was the third type because theportal aneurysm preceded the venous shunt and rup-tured in the hepatic vein to make the communication,and a hepatoportal arteriovenous fistula caused the por-tal aneurysm .

In summary, even though this is a rare anomaly in in-fants, a congenital hepatoportal arteriovenous fistulamay be included in a differential diagnosis when an in-fant presents with the features of portal hypertension.Therefore, in order to evaluate any intrahepatic abnor-mal vascular communications, it may be necessary toperform a careful sonographic examination, includingboth gray-scale and duplex Doppler sonography.

References

1. Agarwala S, Dutta H, Bhatnagar V, Gulathi M, Paul S, Mitra D.Congenital hepatoportal arteriovenous fistula: report of a case.Surg Today 2000;30:268-271

2. Raghuram L, Korah IP, Jaya V, Athyal RP, Thomas A, Thomas G.

J Korean Radiol Soc 2005;53:103-106

─ 105 ─

Coil embolization of a solitary congenital intrahepatic hepatoportalfistula. Abdom Imaging 2001;26:194-196

3. Lewis AM, Aquino NM. Congenital portohepatic vein fistula thatresolved spontaneously in a neonate. AJR Am J Roentgenol1992;159:837-838

4. Taourel P, Perney P, Bouvier Y, Dauzat M, Bricquir YL,Domergue J, et al. Angiographic embolization of intrahepatic arte-rioportal fistula. Eur Radiol 1996;6:510-513

5. Gryboski JD, Clemett A. Congenital hepatic artery aneurysm withsuperior mesenteric artery insufficiency: a steal syndrome.Pediatrics 1967;39:344-347

6. Altuntas B, Erden A, Karakurt C, Kut A, Senbil N, Yurdakul M.

Severe portal hypertension due to congenital hepatoportal arteri-ovenous fistula associated with intrahepatic portal vein aneurysm.J Clin Ultrasound 1998;26:357-360

7. Heaton ND, Davenport M, Karani J, Mowat AP, Howard ER.Congenital hepatoportal arteriovenous fistula. Surgery 1995;117:170-174

8. Chagnon SF, Vallee CA, Barge J, Chevalier LJ, Gal JL, Blery MV.Aneurysmal portahepatic venous fistula: report of two cases.Radiology 1986;159:693-695

9. Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic ve-nous shunt. Case report. AJR Am J Roentgenol 1990;155:527-528

Jin-Hwa Lee, et al : Congenital Hepatoportal Arteriovenous Fistula associated with Intrahepatic Portal Vein Aneurysm and Portohepatic Venous Shunt

─ 106 ─

대한영상의학회지 2005;53:103-106

간내 문맥류 및 문맥간정맥단락을 동반한 선천성 간동맥문맥단락: 증례 보고1

1동아대학교의과대학영상의학과학교실

이진화·신태범·강명진·윤성국·오종영·최종철·박병호·최순섭·남경진

간내 문맥류를 동반한 선천성 간동맥문맥단락은 드문 형태의 동정맥기형이며, 지금까지 보고된 예도 14예에 불과하

다. 또한, 문맥과 간정맥사이의 단락 역시 흔하지 않다. 이에 저자는 빠른 호흡과 흑색변을 주소로 내원한 신생아에서

간내 문맥류 및 문맥간정맥단락을 동반한 선천성 간동맥문맥단락으로 진단된 1예를 보고하고자 한다.