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Congenital intrahepatic arterioportal fistula is a rare but treatable cause of portal hypertension for which early recognition may lead to successful radiological management. We report an infant presenting with severe failure to thrive, melena and splenomegaly due to a congenital intrahepatic arterioportal fistula, successfully ablated after multiple trials of superselective transarterial embolization. Comprehensive review of congenital cases provides an understanding of the key clinical features defining this syndrome. A classification system is proposed, upon which treatment decisions may be based.

We report a case of an intrahepatic arterioportal fistula in a 61-year-old female liver transplant recipient. The patient presented with massive ascites 7 months after a percutaneous liver biopsy. A large fistula between the right hepatic artery and the right portal vein was diagnosed on color Doppler ultrasound and confirmed on arteriography. The fistula was successfully embolized with the detachable balloon technique and the ascites resolved. Symptomatic intrahepatic arterioportal fistula in a liver transplant recipient following percutaneous biopsy is rare. Clinical manifestations, surgical or endovascular therapy, and outcome are discussed. The literature on this subject is reviewed.

Congenital hepatic arterioportal fistula is a treatable cause of portal hypertension. Transarterial embolization is a widely accepted endovascular treatment approach. Here, a case of a recanalized congenital hepatic arterioportal fistula after prior transarterial coil embolization that was successfully treated with percutaneous transhepatic embolization with coils and N-butyl 2-cyanoacrylate is reported. The percutaneous transhepatic approach can be an effective alternative for patients with recanalized hepatic arterioportal fistula who have undergone prior transarterial coil embolization, and N-butyl 2-cyanoacrylate can be used as an embolic material in addition to metallic coils to achieve quick and total occlusion.

Arterioportal fistulae (APFs) are rare. An asymptomatic APF was suspected by computed tomography. Multiplanar, maximum intensity projection, and surface shaded display reconstructions showed its anatomy. To our knowledge, this is the first report using such reconstructions to analyze the architecture of an extrahepatic APF. Complete assessment of APF can be achieved noninvasively, and initial endovascular treatment can be planned.

Traumatic arterioportal fistulas are rare lesions in the pediatric age group. This case highlights the safe and effective management of intrahepatic arterioportal fistulas by transcatheter coil embolization.

Transarterial embolization is one of the treatment choices for symptomatic hepatic arterioportal fistula that has low mortality and morbidity. Proper selection of the technique and embolic material is very important for the success of the procedure. We present a case with high-flow arterioportal fistula treated with transarterial embolization using detachable balloons.

We report a case of congenital arterioportal fistula presenting with upper gastrointestinal bleeding from oesophageal varices. The fistula was successfully treated with surgical ligation of the left hepatic artery.

Ultrasonography is a rapid, accurate, noninvasive diagnostic test for primary (congenital) and secondary (acquired) portosystemic shunting in dogs and cats. Two-dimensional, gray-scale ultrasonography alone enables diagnosis of most congenital portosystemic shunts and determination of intra- versus extrahepatic location. Use of duplex- and color-flow Doppler ultrasonography aids detection of congenital and acquired extrahepatic portosystemic shunts. The underlying cause of acquired portosystemic shunting is portal hypertension; this may be documented by finding either hepatofugal or reduced velocity hepatopetal portal blood flow by duplex-Doppler. Also, ultrasonography may enable detection of lesions involved in the pathogenesis of portal hypertension, for example, hepatic arterioportal fistula, hepatic parenchymal lesions, and portal vein thrombosis.

A unique case with superior mesenteric artery aneurysm and arterioportal fistula resulting from mesenteric arteriovenous malformation is presented, and the relevant literature is reviewed.

Intrahepatic arterioportal fistula (APF) was found in five out of 65 consecutive patients following blunt hepatic trauma. In four patients the fistula was located peripherally and the blood flow was small. These fistulas closed spontaneously within 3 months. However, a centrally located fistula with early visualization of the trunk of the portal vein persisted in one patient and necessitated transcatheter embolization. The APF in this patient caused portal dilatation which was detectable by CT scan. We conclude that spontaneous closure can be expected when an APF is located peripherally and the shunt flow is small, while centrally located APF with large flow require active treatment, preferably by transcatheter embolization. An APF detectable by CT scan suggests the need of intervention.

Large fistulas associated with impaired liver function should be treated by direct obliteration or removal of the shunt orifice. In a large shunt with the portal branch lying on the arterial branch, identification of the exact site of the fistula can be a challenge. We report a case of impaired liver function due to a large intrahepatic arterioportal fistula. The site of the shunt orifice could not be located accurately by preoperative imaging. However, intraoperative color Doppler ultrasonography and the simple regulation of arterial inflow clearly demonstrated the shunt orifice. This original technique has allowed the precise definition of the problem and has optimized the surgical treatment for this critical condition. Consequently, it should be considered a new option for the definition and management of large intrahepatic arterioportal fistulas.

Twenty-three angiographies were performed in 20 rats with a totally arterialized liver. The arterialization was obtained by the construction of an end-to-side portacaval shunt (PCS) and an arteriovenous (AV) fistula between the left gastric artery and the portal stump. Microsurgical technique was necessary because of the small diameter of the AV fistula (0.5 mm). The angiographic examinations showed an AV fistula patency rate of 70% and a PCS rate of 100%. A successively increasing diameter of the fistula with dilation of the intrahepatic portal vessels was demonstrated in the rats with a patent arterioportal fistula. Tortuous and irregular vessels were also observed. All these signs suggest an overarterialization which developed despite the use of a very small AV fistula. None of the three rats examined histologically had cirrhosis of the liver. Further research is needed to attempt to avoid or minimize the deleterious effects of overarterialization of the portal tree, while utilizing the advantages of a dual liver blood supply in patients with decompressive PCS.

A huge spontaneous intrahepatic arterioportal shunt with regurgitation through the portal vein trunk was demonstrated by computed tomography and confirmed by angiography in 2 patients with liver cirrhosis. Rapid sequential computed tomographic scanning was very useful in the diagnosis and follow-up after arterial embolization. Early visualization of the portal vein and increased attenuation in the ipsilateral lobe bearing the fistula were demonstrated in 1 case, and the same findings were also demonstrated by computed tomography and angiography in the other case.

Hepatic metastases produce a spectrum of angiographic alterations of the portal vein that mimicks that of primary liver neoplasms. In 11 patients with varied primary malignancies, hepatic arteriograms revealed seven instances of opacification of the portal vein from arterioportal shunting, two cases of intraluminal tumor thrombus, and two cases with intrahepatic portal vein occlusion. Another patient with liver metastases developed an arterioportal fistula following percutaneous liver biopsy. Although uncommon, metastatic involvement of the portal vein is not necessarily indicative of a poor prognosis since one patient's arterioportal shunting resolved following intraarterial chemotherapy infusion and tumor regression.