Eur J Pediatr Surg 2009; 19(4): 251-253
DOI: 10.1055/s-2008-1038825
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© Georg Thieme Verlag KG Stuttgart · New York

Congenital Intrahepatic Arterioportal Fistula: An Unusual Cause of Portal Hypertension Treated by Coil Embolization in an Infant

İ. Karnak1 , B. E. Çil2 , H. Akay3 , M. Haliloglu2 , A. O. Ciftci1 , M. E. Şenocak1 , A. Besim2
  • 1Department of Pediatric Surgery, Hacettepe University, Faculty of Medicine, Ankara, Turkey
  • 2Department of Radiology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
  • 3Department of Radiology, Dicle University, Diyarbakir, Turkey
Further Information

Publication History

Publication Date:
08 December 2008 (online)

Introduction

Hepatic arterioportal fistula is an unusual cause of portal hypertension (PH) and massive gastrointestinal bleeding in an infant. Arterioportal fistulas can be located in the liver or extrahepatically. They are commonly acquired and develop secondary to cirrhosis, hepatic neoplasms, blunt or penetrating trauma, percutaneous liver biopsy, transhepatic cholangiography, Kasai portoenterostomy, segmental liver transplantation, Ehlers-Danlos syndrome, Rendu-Osler-Weber syndrome and hereditary hemorrhagic telangiectasia [2], [6], [8].

Congenital arterioportal fistulas are defined as an intrahepatic communication between the systemic, usually hepatic, arterial system and the portal venous system, without any communication to the systemic venous circulation, secondary cause or primary hepatic or biliary disease, presenting before 18 years of age [6]. They are rare and constitute less than 10 % of all hepatic arterioportal fistulas.

We describe an infant with congenital intrahepatic arterioportal fistula (IHAPF) who presented with massive gastrointestinal bleeding episodes and was treated successfully by coil embolization to stress the importance of prompt diagnosis and effective treatment.

References

  • 1 Chae E J, Goo H W, Kim S C, Yoon C H. Congenital intrahepatic arterioportal and portosystemic venous fistulae with jejunal arteriovenous malformation depicted on multislice spiral CT.  Pediatr Radiol. 2004;  34 428-431
  • 2 Gallego C, Miralles M, Marin C, Muyor P, Gonzalez G, Garcia-Hidalgo E. Congenital hepatic shunts.  Radiographics. 2004;  24 755-772
  • 3 Lamireau T, Chateil J F, Petit P, Portier F, Panuel M, Grenier N. Successful embolization of congenital intrahepatic arterioportal fistula in two infants.  J Pediatr Gastroenterol Nutr. 1999;  29 211-214
  • 4 Mallant M PJH, Van den Berg F G, Verbeke J IML, Bökenkamp A. Pulsatile hepatofugal flow in the portal vein: hallmark of a congenital hepatoportal arteriovenous fistula.  J Pediatr Gastroenterol Nutr. 2007;  44 143-145
  • 5 Mooney C S, Honaker A D, Griffin W O. Influence of the liver on arteriovenous fistula.  Arch Surg. 1970;  100 154-156
  • 6 Norton S P, Jacobson K, Moroz S P, Culham G, Ng V, Turner J, John P. The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification.  J Pediatr Gastroenterol Nutr. 2006;  43 248-255
  • 7 Raghuram L, Korah I P, Jaya V, Athyal R P, Thomas A, Thomas G. Coil embolization of a solitary congenital intrahepatic hepatoportal fistula.  Abdom Imaging. 2001;  26 194-196
  • 8 Stringer M D, McClean P, Heaton N D, Karani J, Mieli-Vergani G. Congenital hepatic arterioportal fistula.  J Pediatr Gastroenterol Nutr. 1999;  29 487-488

Prof. Dr. İbrahim Karnak

Department of Pediatric Surgery
Hacettepe University
Faculty of Medicine

06100 Ankara

Turkey

Email: ikarnak@hacettepe.edu.tr