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ABDOMINAL IMAGING Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 2  |  Page : 233-236
Recurrence of complex congenital hepatoportal fistula after coil embolisation

Department of Radiology Amrita Institute of Medical Sciences, Amrita Lane , Elamakkara, Cochin-682026, Kerala, India

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Keywords: Intrahepatic shunts, congenital, hepatoportal

How to cite this article:
Prabhu N K, Moorthy S, Sree KK, Pillai A. Recurrence of complex congenital hepatoportal fistula after coil embolisation. Indian J Radiol Imaging 2002;12:233-6

How to cite this URL:
Prabhu N K, Moorthy S, Sree KK, Pillai A. Recurrence of complex congenital hepatoportal fistula after coil embolisation. Indian J Radiol Imaging [serial online] 2002 [cited 2020 Dec 4];12:233-6. Available from:

   Introduction Top

Hepatoportal fistulas are predominantly acquired, secondary to trauma, iatrogenic injury, aneurysm formation, neoplasms or infections. Congenital hepatoportal fistulas are rare lesions, which present during infancy or early childhood with symptoms of portal hypertension, gastrointestinal variceal bleed, ascitis or malabsorption. They are treated surgically or by percutaneous transarterial embolisation. We present a patient with a complex large hepatoportal fistula fed by additional pancreaticoduodenal feeders, which manifested as massive variceal bleed after delivery and was treated by coil embolisation of the hepatic artery. The fistula recurred through common hepatic, pancreaticoduodenal and dorsal pancreatic collateral channels after one year. This case is unusual in view of the late presentation, complex nature of the fistula and recurrence from collateral channels.

   Case Report Top

A 26 year old woman presented two months after a caesarian section, with massive acute hematemesis. Upper Gastrointestinal endoscopy revealed Grade II esophageal varices and a pulsatile actively bleeding large fundal varix, not controlled by sclerosant injections.

There was aneurysmal dilatation of the portal vein confluence and left portal vein with pulsatile flow, on Color Doppler. Angiogram showed a large fast flow complex hepatoportal fistula (with intra and extra hepatic components) from the distal common hepatic artery emptying into the aneurysmally dilated main and left portal venous sacs, with retrograde flow into the splenic vein, superior mesenteric vein, inferior mesenteric vein, coronary veins, fundal and gastroesophageal varices [Figure - 1]a,b. Superior mesenteric artery (SMA) injection showed small collaterals from the proximal superior mesenteric artery and pancreaticoduodenal arcade filling only the intrahepatic left portal venous sac component of the fistula [Figure - 1]c. Her prothrombin time (15.4 seconds INR 1.47) and partial thromboplastin time (APTT 23.2 seconds Control 28.8 seconds) were normal. She gave no history of trauma or iatrogenic intervention in the liver. The distal common hepatic artery just proximal to the fistulous rent was occluded using Gianturco Wallace stainless steel coils (Cook, Bloomington) [Figure - 2]a. The superior mesenteric collaterals were too numerous and small to be selectively catheterized. On hepatic artery injection after embolisation, there was occlusion of the fistula and non-visualization of the portal sacs, while SMA injection showed persistent filling of the left portal vein sac alone [Figure - 2]b. Doppler after 72 hours revealed thrombosis of main portal vein sacs, preserving the vein itself. Her varices collapsed and she was discharged, on expectant management. One year later, she returned with a single self-limited episode of hematemesis. Angiogram revealed collaterals from dorsal pancreatic artery, common hepatic artery and pancreaticoduodenal filling the left portal sac and retrograde filling into the portal system [Figure - 3]. After glue embolisation of the pancreaticoduodenal collaterals, the sac continued to fill from multiple collaterals from the coeliac axis [Figure - 4]. The coeliac collaterals could not be safely embolized and were left as such. Her variceal filling had decreased markedly and she was discharged on follow-up

   Discussion Top

Congenital hepatoportal fistulas are rare entities [1], presenting during infancy or during early childhood with portal hypertension, gastrointestinal bleed secondary to varices, ascitis, malabsorption, anemia and congestion of the bowel [2]. Cardiac failure does not occur due to tamponading by hepatic sinusoids [2]. Life-threatening complications can occur. The diagnosis is confirmed by angiography. Portal vein aneurysmal sac formation can be secondary to hemodynamic changes or could be congenital [3]. Arterialization of the portal vein can lead to progressive hepatoportal sclerosis [4],[5].

Small acquired intrahepatic hepatoportal fistulas may seal spontaneously [4]. Larger fistulas and congenital varieties can be treated by embolisation or by surgical methods. Coexisting portal hypertension and collateral formation can make surgery difficult or unsuccessful [6]. Surgical resection of the fistula is definitive, but failure of resection especially in intrahepatic fistulas is common. Hepatic artery ligation is an alternative [2],[6], but there is a risk of recurrence since the fistula itself is not ablated [6]. Though embolisation as a primary therapeutic modality is widely reported as being curative [1],[7], its long-term benefit is controversial since several reports indicate recurrence of fistulas through collaterals [2],[4],[6]. Its role in controlling acute hemorrhage and in post-surgical recurrence of fistulas is accepted [2],[4]. Embolisation is relatively more effective in solitary fistulas and can be technically impossible in diffuse fistulas [6]. A variety of embolic materials have been used, stainless steel coils being most common. IsoButy1 Cyanoacrylate [8] and Detachable balloons [9] have been used rarely.

The case presented is unusual. The age at presentation is late. Though hepatoportal fistulas have not been reported in association with pregnancy, there are rare reports of splenic artery aneurysms rupturing into the splenic vein leading to formation of an arteriovenous fistula and portal hypertension [5]. The nature of the fistula in our patient is complex, with extrahepatic and intrahepatic components. The common hepatic artery was communicating with the main and left portal vein, with additional feeders from the pancreaticoduodenal arcade supplying the left portal vein sac.

In our patient, embolisation was life saving, resulting in immediate cessation of the variceal bleed and collapse of the varices. She was asymptomatic for one year, before collateralization produced repeat variceal hemorrhage.

In conclusion, congenital hepatoportal fistulas are rare developmental anomalies and experience in treatment is limited. While coil embolisation may be successful in the majority in the short term, complex fistulas with multiple feeders are difficult to treat by embolisation alone, given the rich vascularity of the porta and propensity to develop collateral arteries. On the other hand, difficulties of resecting the fistula in a vascular field with coexisting portal hypertension and reports of recurrence after hepatic artery ligation, present a dilemma in management

   References Top

1.Agarwala S. Dutta H, Bhatnagar V, Gulathi M, Paul S, Mitra. Congenital hepatoportal arteriovenous fistula: report of a case. Surg Today, 2000; 30(3): 268-71. Review.  Back to cited text no. 1    
2.Paley MR, Farrant P, Kane P, Heaton ND, Howard ER, Karani, Developmental intrahepatic shunts of childhood: radiological features and management. Eur Radiol. 1997;7(9): 1377-82.  Back to cited text no. 2    
3.Altuntas B, Erden A, Karakurt C, Kut A, Senbil N, Yurdakul. Severe portal hypertension due to congenital hepatoportal arteriovenous fistula associated with intrahepatic portal vein aneurysm. J Clin Ultrasound. 1998; 26(7):357-60.Vujic I, Meredith HC, Ameriks JA. Embolization for hepatoportal arteriovenous fistula. Am Surg. 1980;46(6):366-70.  Back to cited text no. 3    
4.Pasternak BM, Cohen H. Arteriovenous fistula and forward hypertension in the portal circulation. Angiology. 1978; 29(5): 367-73.  Back to cited text no. 4    
5.Heaton ND, Davenport M, Karani J, Mowat AP, Howard ER. Congenital hepatoportal arteriovenous fistula, Surgery. 1995;117(2): 170-4. Reviewii.  Back to cited text no. 5    
6.Raghuram L. Korah IP, Jaya V. Athyal RP, Thomas A. Thomas. Coil embolization of a solitary congenital intrahepatic hepatoportal fistula. Abdom Imaging. 2001; 26(2): 194-6.  Back to cited text no. 6    
7.Ramchandani P, Goldenberg NJ, Soulen RL, White RI Jr. Isobutyl 2-cyanoacrylate embolization of a hepatoportal fistula. AJR Am J Roentgenol. 1983; 140(1): 137-40.  Back to cited text no. 7    
8.Ridout DL 3rd Bralow SP, Chait A, Nusbaum M. Hepatoportal arteriovenous fistula treated with detachable balloon embolotherapy. Am J Gastroenterol. 1989; 84 (1): 63-66.  Back to cited text no. 8    

Correspondence Address:
N K Prabhu
Department of Radiology Amrita Institute of Medical Sciences, Amrita Lane , Elamakkara, Cochin-682026, Kerala, India

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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