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Arterioportal Fistula—An Unusual and Treatable Cause of Portal Hypertension

Mahimarangaiha, Jayaranganath; Sastry, Usha M. Kodandarama; Rai, Maneesh; Nanjappa, Manjunath C.

Journal of Pediatric Gastroenterology and Nutrition: June 2014 - Volume 58 - Issue 6 - p e54–e55
doi: 10.1097/MPG.0b013e31827bf0c4
Case Reports

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Sri Jayadeva Institute of Cardiovascular Sciences And Research, Department of Pediatric Cardiology, Jayanagar 9 Th Block, Bannerghatta Road, Bangalore 560 069, India.

Address correspondence and reprint requests to Usha MK, MD, Sri Jayadeva Institute of Cardiology and Research Sciences, Bangalore 560 069, India (e-mail:

Received 24 June, 2012

Accepted 31 October, 2012

Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (

The authors report no conflicts of interest.

Portal hypertension in neonates and infants has enumerable causes. The treatment is frustrating with dismal results in many instances. Arterioportal fistulas (APFs) are a rare cause of portal hypertension. Successful catheter closure is possible when the anatomy is suitable. Here we present an arterioportal fistula in a 40-day-old infant successfully closed by catheter intervention.

A 40-day-old female infant presented with progressive abdominal distension since 20 days of life. Child was born by normal delivery with weight of 3.5 kg. Antenatal and immediate postnatal period was uneventful. Since day 20 of life parents noticed progressive abdominal distension, irritability, and reduced feeding. The baby had diarrhea with weight loss. There was no melena or hematochezia. On examination the baby weighed 3.5 kg, with a pulse of 110 beats/min and normal volume. Abdomen was tense, grossly distended with prominent veins on anterior abdominal wall (Fig. 1A). There was significant hepatosplenomegaly with ascites. There was no bruit over the abdomen. Other systems were normal. The liver function tests and hemogram were within normal limits (Table 1). Abdominal ultrasound with Doppler showed dilated right portal vein measuring 13 mm with portal hypertension. There was reversal of flow with an arterial pattern. A large artery was directly opening into the aneurysmally dilated portal vein. Computed tomograpgy angiogram of the abdomen revealed a large vessel arising from aorta and directly joining the right portal vein (Fig. 1B). A diagnosis of solitary large arterioportal fistula was made. Child was taken for conventional angiogram. Heparin (100 U/kg) was administered and the fistula imaged with contrast injection into the descending aorta just above the renal artery. Retrograde filling of left and main portal vein was seen (Fig. 1C/Video S1, (Video S1: Angiographic demonstration of arterioportal fistula). There was narrowing at the arteriovenous junction of the fistula. Hepatic vein wedge pressure measured was 13 mmHg. An Amplatzer Duct Occluder II (AGA Medical Corporation, North Plymouth, MN) measuring 5/4 mm was chosen. This device has a waist with retention skirts on either side to prevent embolization. It can be easily deployed from the arterial side and can be negotiated into a 5-F sheath. The procedural time is less and the injury sustained to femoral artery is minimal. The device was loaded onto the cable and passed into a 5-F sheath and deployed at the narrowest portion of the fistula (Fig. 1d/Video S2, [Video S2: Device deployed at the narrowest portion of the fistula]). Check angiogram revealed complete occlusion of the fistula (Video S3, (Video S3: Postdeployment no residual flow)). The abdominal distension, dilated veins, and hepatosplenomegaly reduced during a period of 7 to 10 days. The child was given aspirin at antithrombotic doses for 6 months. The child was healthy, thriving at the end of 12 months, and was weighing 11 kg. On follow-up ultrasound the portal vein (5.5 mm) was reduced in size with normal flow pattern with thrombosis of the anomalous arterial branch.





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APFs are a rare, treatable cause of portal hypertension. A branch of hepatic artery communicates directly with portal vein radicals without communicating with systemic veins. They may be congenital or acquired owing to neoplasms, liver biopsy, or surgery (1,2). There is no underlying hepatic or biliary disease.

APFs have been classified by Norton et al (3) as type I—unilateral (supplied by right, left, or main hepatic artery), type II—bilateral, and type III–complex plexiform vascular nidus. So far 30 cases of congenital APF have been reported with majority of them (75%) being unilateral or bilateral.

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Clinical Features and Pathophysiology

Back flow in portal veins lead to increased pressure with splenomegaly, ascites, and esophageal varices (1,2). Continuous murmur may be heard over the site of the fistula. Superior mesenteric artery steal can cause small bowel angina and infarction. Bleeding because of varices or bowel infarction can occur. Congestive failure does not occur because hepatic sinusoids buffer the shunt. Chronic diarrhea can occur as a result of protein-losing enteropathy and fat malabsorption because of venous congestion of bowel. Chronic exposure of portal venous system to arterial flow causes irreversible portal hypertension and late portal vein thrombosis (1).

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Any child with unexplained portal hypertension should be investigated for arterioportal fistula. Ultrasound with Doppler will provide the initial clue to the diagnosis. Computed tomography angiography will delineate the fistula, differentiate from other vascular malformations, and help in deciding on the strategy of intervention. Conventional angiography will confirm the diagnosis and will be the first step toward definitive intervention (3).

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Closure of the fistula is the definitive treatment. Embolization should be attempted in all fistulas before surgery. Catheter-directed closure of solitary or bilateral large fistula is simple, effective, and safe. It has the advantage of significantly reducing the morbidity and hospital stay. It is cost-effective and time sparing (<30 minutes) in the hands of a skilled operator. Availability of materials and skill of the interventionist are the limiting factors. Surgery should be the last option when repeated embolization fails to close the fistula.

Complex fistulas may be closed by repeated embolization or a combination of surgery and catheter intervention. Surgery includes ligation, hepatectomy, transplantation, or portocaval shunt (4,5). Materials that have been used for embolization are gel foam, gianturco coils, vascular plugs, cyanoacrylate glue, and amplatzer duct occluder. The choice of the device depends on size, number, and complexity of fistula. Large devices (duct occluder/vascular plug) (6,7) or balloon (8) are suitable for high-flow lesions and coils, glue, gel foam should be used for low-flow lesions (1,3).

Recurrence is known after initial closure because the dormant fistulous connections open after the major fistulous artery has been sealed. Portal vein thrombosis can occur because of migration of coils. Closing the fistula at the origin of the arterial feeder and achieving total occlusion may prevent both complications (1,4). Long-term clinical and ultrasound follow-up is required.

Amplatzer duct occluder I has been used in 1 child for closure of APF (6). This is the second case where duct occluder of a different kind (ADO II) has been used. Our case is unique in the young age at which a large solitary fistula has presented, the device used, and the earliest age at which the fistula has been completely closed by simple and effective method. To the best of our knowledge, this has been the youngest child in the literature with arterioportal fistula to be diagnosed and successfully treated with device closure.

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1. Marchand V, Uflacker R, Baker S, et al. Congenital arteriohepatic fistula in a 3-year-old child. J Pediatr Gastroenterol Nutr 1999; 28:435–441.
2. Vauthey JN, Tomczak RJ, Helmberger T. The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy. Gastroenterology 1997; 113:1390.
3. Norton SP, Jacobson K, Moroz SP, et al. The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr 2006; 43:248–255.
4. Meunier C, Dabadie A, Darnault P, et al. Congenital intrahepatic arterio-portal fistula: diagnostic and therapeutic aspects. Pediatrics 1993; 48:211–216.
5. Sutcliffe R, Mieli-Vergani G, Dhawan A, et al. A novel treatment of congenital hepatoportal arteriovenous fistula. J Pediatr Surg 2008; 43:571–573.
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7. Osman Koc, Barbaros EC, Bora P, et al. Complementary use of NBCA with the Amplatzer vascular plug for embolization of a high-flow traumatic hepatic arteriovenous fistula. Cardiovasc Intervent Radiol 2009; 32:1105–1107.
8. Akpek S, Ilgit ET, Cekirge S, et al. High-flow arterioportal fistula: treatment with detachable balloon occlusion. Abdom Imaging 2001; 26:277–280.

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