Download PDF
CC BY-NC-ND 4.0 · Indian J Radiol Imaging 2016; 26(03): 364-372
DOI: 10.4103/0971-3026.190420
Pediatric

The Abernethy malformation-myriad imaging manifestations of a single entity

Samarjit S Ghuman
Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
,
Saumya Gupta
Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
,
T B S Buxi
Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
,
Kishan S Rawat
Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
,
Anurag Yadav
Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
,
Naimish Mehta
Department of Surgical Gastroenterology, Sir Gangaram Hospital, New Delhi, India
,
Seema Sud
Department of Radiodiagnosis, Sir Gangaram Hospital, New Delhi, India
› Author Affiliations Financial support and sponsorship Nil.
› Further Information
Also available at
  PDF Download Permissions and Reprints

Abstract

Abernethy malformation, also known as congenital extrahepatic portosystemic shunts (CEPS) is a rare clinical entity and manifests with different clinical symptoms. CEPS are abnormalities of vascular development where there is shunting of portal blood into the systemic venous system. Multidetector computed tomography (MDCT) is a fast and effective modality for evaluation of CEPS. CT displays all the information desired by the surgeon as well as the clinician including the anatomy of the splenic and superior mesenteric veins, size and site of the shunt, presence or absence of the portal vein radicles, and helps to plan the therapy and even the follow-up of these patients. Contrast-enhanced magnetic resonance imaging (MRI) has also emerged as a promising tool for the evaluation of liver lesions associated with the malformation. The Radiologist should be aware of the various imaging appearances of this entity including its complications. In this article, we describe the imaging appearances of CEPS, their complications, and their imaging appearances on CT and MRI. We have also described various associated anomalies.

Keywords

Abernethy malformation - congenital extrahepatic portosystemic shunts - portosystemic shunts


Publication History

Article published online:
30 July 2021

© 2016. Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Thieme Medical and Scientific Publishers Private Ltd.
A-12, Second Floor, Sector -2, NOIDA -201301, India

 

  • References

  • 1 Abernethy J. Account of two instances of uncommon formation, in the viscera of the human body. Philos Trans R Soc Lond B Biol Sci 1793;83:59-66.
  • 2 Hu GH' Shen LG, Yang J' Mei JH' Zhu YF. Insight into congenital absence of the portal vein: Is it rare? World J Gastroenterol 2008;14:5969-79.
  • 3 Hales MR, Allan JS, Hall EM. Injection-corrosion studies of normal and cirrhotic livers. Am J Pathol 1959;35:909-41.
  • 4 Alonso-Gamarra E, Parrón M, Pérez A, Prieto C, Hierro L, López-Santamaría M. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: A comprehensive review. Radiographics 2011;31:707-22.
  • 5 Doehner GA, Ruzicka FF Jr, Rousselot LM, Hoffman G. The portal venous system: On its pathological roentgen anatomy. Radiology 1956;66:206-17.
  • 6 Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 1990;155:527-8.
  • 7 Morgan G, Superina R. Congenital absence of the portal vein: Two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 1994;29:1239-41.
  • 8 Marks C. Developmental basis of the portal venous system. Am J Surg 1969;117:671-81.
  • 9 Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 2008; 21:147-57.
  • 10 Komatsu S, Nagino M, Hayakawa N, Yamamoto H, Nimura Y. Congenital absence of portal venous system associated with a large inferior mesenteric-caval shunt: A case report. Hepatogastroenterology 1995;42:286-90.
  • 11 Niwa T, Aida N, Tachibana K, Shinkai M, Ohhama Y, Fujita K, et al. Congenital absence of the portal vein: clinical and radiologic findings. J Comput Assist Tomogr 2002;26:681-6.
  • 12 Dickson AD. The development of the ductus venosus in man and the goat. J Anat 1957;91:358-68.
  • 13 Howard ER, Davenport M. Congenital extrahepatic portocaval shunts—the Abernethy malformation. J Pediatr Surg 1997;32:494-7.
  • 14 Oei M, Wessling J. Abernethy malformation—Congenital aplasia of the portal vein a 29-year-old patient. Rofo 2007;179:167-9.
  • 15 Witters P, Maleux G, George C, Delcroix M, Hoffman I, Gewillig M, et al. Congenital veno-venous malformations of the liver: Widely variable clinical presentations. J Gastroenterol Hepatol 2008;23(Pt 2):e390-4.
  • 16 Asran MK, Loyer EM, Kaur H, Choi H. Case 177: Congenital absence of the portal vein with hepatic adenomatosis. Radiology 2012;262:364-7.
  • 17 Porres-Aguilar M, Altamirano JT, Torre-Delgadillo A, Carlton MR, Duarte-Rojo A. Portopulmonary hypertension and hepatopulmonary syndrome: A clinician-oriented overview. Eur Respir Rev 2012;21:223-33.
  • 18 Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD. Abernethy malformation: One of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 2002;34:391-4.
  • 19 Tercier S, Delarue A, Rouault F, Roman C, Bréaud J, Petit P. Congenital portocaval fistula associated with hepatopulmonary syndrome: Ligation vs liver transplantation. J Pediatr Surg 2006;41:e1-3.
  • 20 Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, et al. Complications of congenital portosystemic shunts in children: Therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 2010;51:322-30.
  • 21 Ono H, Mawatari H, Mizoguchi N, Eguchi T, Sakura N. Clinical features and outcome of eight infants with intrahepatic porto-venous shunts detected in neonatal screening for galactosaemia. Acta Paediatr 1998;87:631-4.
  • 22 Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol 2003;33:614-20.
  • 23 Satoh M, Yokoya S, Hachiya Y, Hachiya M, Fujisawa T, Hoshino K, et al. Two hyperandrogenic adolescent girls with congenital portosystemic shunt. Eur J Pediatr 2001;160:307-11.
  • 24 Goo HW. Extrahepatic portosystemic shunt in congenital absence of the portal vein depicted by time-resolved contrast-enhanced MR angiography. Pediatr Radiol 2007;37:706-9.
  • 25 Wakamoto H, Manabe K, Kobayashi H, Hayashi M. Subclinical portal-systemic encephalopathy in a child with congenital absence of the portal vein. Brain Dev 1999;21:425-8.
  • 26 Uchino T, Matsuda I, Endo F. The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 1999;135(Pt 1):254-6.
  • 27 Ikeda S, Sera Y, Yoshida M, Ohshiro H, Uchino S, Seguchi S, et al. Reversibility of hyperintense globus pallidus on T1-weighted MRI following surgery for a portosystemic shunt in an 8-year-old girl. Pediatr Radiol 1999;29:449-50.
  • 28 Kumar A, Kumar J, Aggarwal R, Srivastava S. Abernethy malformation with portal vein aneurysm. Diagn Interv Radiol 2008;14:143-6.
  • 29 Massin M, Verloes A, Jamblin P. Cardiac anomalies associated with congenital absence of the portal vein. Cardiol Young 1999;9:522-5.
  • 30 Ratnasamy C, Kurbegov A, Swaminathan S. Cardiac anomalies in the setting of the Abernethy malformation of the portal vein. Cardiol Young 2007;17:212-4.
  • 31 Gocmen R, Akhan O, Talim B. Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr Radiol 2007;37:920-4.
  • 32 Le Borgne J, Paineau J, Hamy A, Dupas B, Lerat F, Raoul S, et al. Interruption of the inferior vena cava with azygos termination associated with congenital absence of portal vein. Surg Radiol Anat 2000;22:197-202.
  • 33 De Gaetano AM, Gui B, Macis G, Manfredi R, Di Stasi C. Congenital absence of the portal vein associated with focal nodular hyperplasia in the liver in an adult woman: Imaging and review of the literature. Abdom Imaging 2004;29:455-9.
  • 34 Kinjo T, Aoki H, Sunagawa H, Kinjo S, Muto Y. Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst: A case report. J Pediatr Surg 2001;36:622-5.
  • 35 Guariso G, Fiorio S, Altavilla G, Gamba PG, Toffolutti T, Chiesura-Corona M, et al. Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney. Eur J Pediatr 1998;157:287-90.
  • 36 Appel H, Loddenkemper C, Schirmacher P, Dienes HP, Sieper J, Rudwaleit M, et al. Congenital absence of the portal vein with splenomegaly and hypersplenism in a young woman. Digestion 2003;67:105-10.
  • 37 Grazioli L, Alberti D, Olivetti L, Rigamonti W, Codazzi F, Matricardi L, et al. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 2000;10:820-5.
  • 38 Kondo F. Benign nodular hepatocellular lesions caused by abnormal hepatic circulation: Etiological analysis and introduction of a new concept. J Gastroenterol Hepatol 2001;16:1319-28.
  • 39 Brancatelli G, Federle MP, Grazioli L, Golfieri R, Lencioni R. Benign regenerative nodules in Budd-Chiari syndrome and other vascular disorders of the liver: Radiologic–pathologic and clinical correlation. Radiographics 2002;22:847-62.
  • 40 Arana E, Martí-Bonmatí L, Martínez V, Hoyos M, Montes H. Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. Abdom Imaging 1997;22:506-8.
  • 41 Kawano S, Hasegawa S, Urushihara N, Okazaki T, Yoshida A, Kusafuka J, et al. Hepatoblastoma with congenital absence of the portal vein—a case report. Eur J Pediatr Surg 2007;17:292-4.
  • 42 Barton JW 3rd, Keller MS. Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein. Pediatr Radiol 1989;20:113-4.
  • 43 Banz V, Olliff S, Taniere P, Mayer D, Isaac J. Liver tumours in patients with Abernethy malformation. ANZ J Surg 2011;81:640-1.
  • 44 Pichon N, Maisonnette F, Pichon-Lefièvre F, Valleix D, Pillegand B. Hepatocarcinoma with congenital agenesis of the portal vein. Jpn J Clin Oncol 2003;33:314-6.
  • 45 Lundstedt C, Lindell G, Tranberg KG, Svartholm E. Congenital absence of the intrahepatic portion of the portal vein in an adult male resected for hepatocellular carcinoma. Eur Radiol 2001;11:2228-31.
  • 46 Joyce AD, Howard ER. Rare congenital anomaly of the portal vein. Br J Surg 1988;75:1038-9.
  • 47 Kim SZ, Marz PL, Laor T, Teitelbaum J, Jonas MM, Levy HL. Elevated galactose in newborn screening due to congenital absence of the portal vein. Eur J Pediatr 1998;157:608-9.
  • 48 Bonington SC, Hodgson DI, Mehta S, Lynch N, Chalmers N. A congenital venous anomaly, with a portal-systemic shunt into a previously undescribed intra-thoracic vein. Clin Radiol 2002;57:658-60.
  • 49 Koizumi J, Yamashita T, Dowaki S, Tobita K, Shimamura K, Hirabayashi K, et al. Hepatobiliary and pancreatic: Hepatic adenoma, focal nodular hyperplasia and congenital absence of the portal vein. J Gastroenterol Hepatol 2006;21:619.
  • 50 Konno K, Ishida H, Uno A, Ohnami Y, Masamune O. Large extrahepatic portosystemic shunt without portal hypertension. Abdom Imaging 1997;22:79-81.
  • 51 Prokop M. Multislice CT angiography. Eur J Radiol 2000;36:86-96.
  • 52 Morgan H, Braunstein D. A computed tomography dose index for infants and young children. Med Phys 2001;28:1818.
  • 53 Trenschel GM, Schubert A, Dries V, Benz-Bohm G. Nodular regenerative hyperplasia of the liver: Case report of a 13-year-old girl and review of the literature. Pediatr Radiol 2000;30:64-8.
  • 54 Krajina A, Lojik M, Chovanec V, Raupach J, Hulek P. Wedged hepatic venography for targeting the portal vein during TIPS: Comparison of carbon dioxide and iodinated contrast agents. Cardiovasc Intervent Radiol 2002;25:171-5.
  • 55 Martinez-Cuesta A, Elduayen B, Vivas I, Delgado C, Gonzalez-Crespo I, Bilbao JI. CO (2) wedged hepatic venography: Technical considerations and comparison with direct and indirect portography with iodinated contrast. Abdom Imaging 2000;25:576-82.
  • 56 Sura PA, Tobias KM, Morandi F, Daniel GB, Echandi RL. Comparison of 99mTcO4(-) trans-splenic portal scintigraphy with per-rectal portal scintigraphy for diagnosis of portosystemic shunts in dogs. Vet Surg 2007;36:654-60.
  • 57 Shiomi S, Kuroki T, Ueda T, Takeda T, Ikeoka N, Nishiguchi S, et al. Clinical usefulness of evaluation of portal circulation by per rectal portal scintigraphy with technetium-99m pertechnetate. Am J Gastroenterol 1995;90:460-5.
  • 58 Gallego C, Miralles M, Marín C, Muyor P, González G, García-Hidalgo E. Congenital hepatic shunts. Radiographics 2004;24:755-72.
  • 59 Akahoshi T, Nishizaki T, Wakasugi K, Mastuzaka T, Kume K, Yamamoto I, et al. Portal-systemic encephalopathy due to a congenital extrahepatic portosystemic shunt: Three cases and literature review. Hepatogastroenterology 2000;47:1113-6.
  • 60 Inomata Y, Kiuchi T, Kim I, Uemoto S, Egawa H, Asonuma K, et al. Auxiliary partial orthotopic living donor liver transplantation as an aid for small-for-size grafts in larger recipients. Transplantation 1999;67:1314-9.