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.
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).
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).
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.
1. Marchand V, Uflacker R, Baker S, et al. Congenital arteriohepatic fistula in a 3-year-old child. J Pediatr Gastroenterol Nutr
2. Vauthey JN, Tomczak RJ, Helmberger T. The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy. Gastroenterology
3. Norton SP, Jacobson K, Moroz SP, et al. The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr
4. Meunier C, Dabadie A, Darnault P, et al. Congenital intrahepatic arterio-portal fistula: diagnostic and therapeutic aspects. Pediatrics
5. Sutcliffe R, Mieli-Vergani G, Dhawan A, et al. A novel treatment of congenital hepatoportal arteriovenous fistula. J Pediatr Surg
6. Kumar N, de Goyet Jde V, Sharif K, et al. Congenital, solitary,large, intrahepatic arterioportal fistula in a child: management and review of the literature. Pediatr Radiol
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
8. Akpek S, Ilgit ET, Cekirge S, et al. High-flow arterioportal fistula: treatment with detachable balloon occlusion. Abdom Imaging
Supplemental Digital Content
© 2014 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,