Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital heart disease (Abernethy malformation): A case report and literature review
- Authors:
- Yabin Hao
- Xu Hong
- Xinyan Zhao
-
Affiliations: Department of Endocrinology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, P.R. China, Department of Hepatology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, P.R. China - Published online on: December 4, 2014 https://doi.org/10.3892/ol.2014.2767
- Pages: 695-700
This article is mentioned in:
Abstract
 |
 |
|
Abernethy J and Banks J: Account of two instances of uncommon formation in the viscera of the human body. Philos Trans R Soc Lond. 83:59–66. 1793. View Article : Google Scholar | |
|
Asrani SK, Asrani NS, Freese DK, et al: Congenital heart disease and the liver. Hepatology. 56:1160–1169. 2012. View Article : Google Scholar : PubMed/NCBI | |
|
Schaeffer DF, Laiq S, Jang HJ, John R and Adeyi OA: Abernethy malformation type II with nephrotic syndrome and other multisystemic presentation: an illustrative case for understanding pathogenesis of extrahepatic complication of congenital portosystemic shunt. Hum Pathol. 44:432–437. 2013. View Article : Google Scholar | |
|
Witjes CD, Ijzermans JN, Vonk Noordegraaf A and Tran TK: Management strategy after diagnosis of Abernethy malformation: a case report. J Med Case Rep. 6:1672012. View Article : Google Scholar : PubMed/NCBI | |
|
Daddi G, Cozzi M and Pardi G: Anatomo-pathological considerations on a case of congenital stenosis of the portal vein. Folia Hered Pathol (Milano). 15:1121965.(in Italian). | |
|
Motoori S, Shinozaki M, Goto N and Kondo F: Case report: congenital absence of the portal vein associated with nodular hyperplasia in the liver. J Gastroenterol Hepatol. 12:639–643. 1997. View Article : Google Scholar | |
|
Matsuoka Y, Ohtomo K, Okubo T, Nishikawa J, Mine T and Ohno S: Congenital absence of the portal vein. Gastrointest Radiol. 17:31–33. 1992. View Article : Google Scholar : PubMed/NCBI | |
|
Arana E, Martí-Bonmatí L, Martinez V, Hoyos M and Montes H: Portal vein absence and nodular regenerative hyperplasia of the liver with giant inferior mesenteric vein. Abdom Imaging. 22:506–508. 1997. View Article : Google Scholar : PubMed/NCBI | |
|
Northrup M, Mendez-Castillo A, Sethi Y and Churchill R: Congenital absence of the portal vein with an intrahepatic inferior vena cava branch showing hepatopetal flow. J Ultrasound Med. 21:569–572. 2002.PubMed/NCBI | |
|
Lundstedt C, Lindell G, Tranberg KG and Svartholm E: Congenital absence of the intrahepatic portion of the portal vein in an adult male resected for hepatocellular carcinoma. Eur Radiol. 11:2228–2231. 2001. View Article : Google Scholar : PubMed/NCBI | |
|
De Gaetano AM, Gui B, Macis G, Manfredi R and 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. 29:455–459. 2004. View Article : Google Scholar : PubMed/NCBI | |
|
Appel H, Loddenkemper C, Schirmacher P, et al: Congenital absence of the portal vein with splenomegaly and hypersplenism in a young woman. Digestion. 67:105–110. 2003. View Article : Google Scholar : PubMed/NCBI | |
|
Wojcicki M, Haagsma EB, Gouw AS, Slooff MJ and Porte RJ: Orthotopic liver transplantation for portosystemic encephalopathy in an adult with congenital absence of the portal vein. Liver Transpl. 10:1203–1207. 2004. View Article : Google Scholar : PubMed/NCBI | |
|
Cheung KM, Lee CY, Wong CT and Chan AK: Congenital absence of portal vein presenting as hepatopulmonary syndrome. J Paediatr Child Health. 41:72–75. 2005. View Article : Google Scholar : PubMed/NCBI | |
|
Tsuji K, Naoki K, Tachiyama Y, et al: A case of congenital absence of the portal vein. Hepatol Res. 31:43–47. 2005. View Article : Google Scholar : PubMed/NCBI | |
|
Takagaki K, Kodaira M, Kuriyama S, et al: Congenital absence of the portal vein complicating hepatic tumors. Intern Med. 43:194–198. 2004. View Article : Google Scholar : PubMed/NCBI | |
|
Kornprat P, Langner C, Fritz K and Mischinger HJ: Congenital absence of the portal vein in an adult woman: a case report. Wien Klin Wochenschr. 117:58–62. 2005. View Article : Google Scholar : PubMed/NCBI | |
|
Ohnishi Y, Ueda M, Doi H, et al: Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess. J Pediatr Surg. 40:E1–E3. 2005. View Article : Google Scholar : PubMed/NCBI | |
|
Takeichi T, Okajima H, Suda H, et al: Living domino liver transplantation in an adult with congenital absence of portal vein. Liver Transpl. 11:1285–1288. 2005. View Article : Google Scholar : PubMed/NCBI | |
|
Oei M and Wessling J: Abernethy malformation - congenital aplasia of the portal vein a 29-year-old patient. RoFo. 179:167–169. 2007.(in German). View Article : Google Scholar : PubMed/NCBI | |
|
Collard B, Maleux G, Heye S, et al: Value of carbon dioxide wedged venography and transvenous liver biopsy in the definitive diagnosis of Abernethy malformation. Abdom Imaging. 31:315–319. 2006. View Article : Google Scholar : PubMed/NCBI | |
|
Maekawa S, Suzuki T, Mori K, et al: Congenital absence of the portal vein in an adult woman with liver tumor. Nihon Shokakibyo Gakkai Zasshi. 104:1504–1511. 2007.(in Japanese). PubMed/NCBI | |
|
Kong Y, Zhang H, Liu C, et al: Abernethy malformation with multiple aneurysms: incidentally found in an adult woman with Caroli’s disease. Ann Hepatol. 12:327–331. 2013.PubMed/NCBI | |
|
Matsuura T, Soejima Y and Taguchi T: Auxiliary partial orthotopic living donor liver transplantation with a small-for-size graft for congenital absence of the portal vein. Liver Transpl. 16:1437–1439. 2010. View Article : Google Scholar : PubMed/NCBI | |
|
Chandler TM, Heran MK, Chang SD, Parvez A and Harris AC: Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein. Magn Reson Imaging. 29:881–886. 2011. View Article : Google Scholar : PubMed/NCBI | |
|
Barchetti F, Pellegrino L, Al-Ansari N, De Marco V, Scarpato P and Ialongo P: absence of the portal vein in a middle-aged man. Surg Radiol Anat. 33:369–372. 2011. View Article : Google Scholar | |
|
Caputo S and Manganiello CA: Severe portopulmonary hypertension associated with an unusual pattern of Abernethy malformation: three-dimensional computed tomography view. Echocardiography. 29:E275–E277. 2012. View Article : Google Scholar : PubMed/NCBI | |
|
Scheuermann U, Foltys D and Otto G: Focal nodular hyperplasia proceeds hepatocellular carcinoma in an adult with congenital absence of the portal vein. Transpl Int. 25:E67–E68. 2012. View Article : Google Scholar : PubMed/NCBI | |
|
Badea R, Serban A, Procopet B, et al: Education and imaging: hepatobiliary and pancreatic: Abernethy malformation-congenital portocaval shunt. J Gastroenterol Hepatol. 27:18752012. View Article : Google Scholar : PubMed/NCBI | |
|
Tateishi Y, Furuya M, Kondo F, et al: Hepatocyte nuclear factor-1 alpha inactivated hepatocellular adenomas in patient with congenital absence of the portal vein: a case report. Pathol Int. 63:358–363. 2013. View Article : Google Scholar : PubMed/NCBI | |
|
Alewine TC, Carter WR and Frew MJ: Congenital absence of the portal vein in a patient with urolithiasis. AJR Am J Roentgenol. 189:W150–W152. 2007. View Article : Google Scholar : PubMed/NCBI | |
|
Morgan G and Superina R: Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg. 29:1239–1241. 1994. View Article : Google Scholar : PubMed/NCBI | |
|
Morse SS, Taylor KJ, Strauss EB, Ramirez E and Seashore JH: Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome). Pediatr Radiol. 16:437–439. 1986. View Article : Google Scholar : PubMed/NCBI | |
|
Marois D, van Heerden JA, Carpenter HA and Sheedy PF 2nd: Congenital absence of the portal vein. Mayo Clin Proc. 54:55–59. 1979.PubMed/NCBI | |
|
Yancy AG, Jeffries CP and Miller RM: Congenital absence of the portal vein. J Natl Med Assoc. 53:119–121. 1961.PubMed/NCBI | |
|
Laverdiere JT, Laor T and Benacerraf B: Congenital absence of the portal vein: case report and MR demonstration. Pediatr Radiol. 25:52–53. 1995. View Article : Google Scholar : PubMed/NCBI | |
|
Kamiya S, Taniguchi I, Yamamoto T, et al: Analysis of intestinal flora of a patient with congenital absence of the portal vein. FEMS Immunol Med Microbiol. 7:73–80. 1993. View Article : Google Scholar : PubMed/NCBI | |
|
Woodle ES, Thistlethwaite JR, Emond JC, Whitington PF, Vogelbach P, Yousefzadeh DK and Broelsch CE: Successful hepatic transplantation in congenital absence of recipient portal vein. Surgery. 107:475–479. 1990.PubMed/NCBI | |
|
Nakasaki H, Tanaka Y, Ohta M, Kanemoto T, Mitomi T, Iwata Y and Ozawa A: Congenital absence of the portal vein. Ann Surg. 210:190–193. 1989. View Article : Google Scholar : PubMed/NCBI | |
|
Usuki N and Miyamoto T: A case of congenital absence of the intrahepatic portal vein diagnosed by MR angiography. J Comput Assist Tomogr. 22:728–729. 1998. View Article : Google Scholar : PubMed/NCBI | |
|
Kim SZ, Marz PL, Laor T, Teitelbaum J, Jonas MM and Levy HL: Elevated galactose in newborn screening due to congenital absence of the portal vein. Eur J Pediatr. 157:608–609. 1998. View Article : Google Scholar : PubMed/NCBI | |
|
Guariso G, Fiorio S, Altavilla G, 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. 157:287–290. 1998. View Article : Google Scholar : PubMed/NCBI | |
|
Howard ER and Davenport M: Congenital extrahepatic portocaval shunts - the Abernethy malformation. J Pediatr Surg. 32:494–497. 1997. View Article : Google Scholar : PubMed/NCBI | |
|
Massin M, Verloes A and Jamblin P: Cardiac anomalies associated with congenital absence of the portal vein. Cardiol Young. 9:522–525. 1999. View Article : Google Scholar : PubMed/NCBI | |
|
Taoube KA, Alonso Calderon JL, Yandza T, et al: Congenital absence of portal vein in a girl with biliary atresia treated with liver transplant. Cir Pediatr. 12:38–40. 1999.(in Spanish). PubMed/NCBI | |
|
Wakamoto H, Manabe K, Kobayashi H and Hayashi M: Subclinical portal-systemic encephalopathy in a child with congenital absence of the portal vein. Brain Dev. 21:425–428. 1999. View Article : Google Scholar : PubMed/NCBI | |
|
Kohda E, Saeki M, Nakano M, Masaki H, Ogawa K, Nirasawa M and Hiramatsu K: Congenital absence of the portal vein in a boy. Pediatr Radiol. 29:235–237. 1999. View Article : Google Scholar : PubMed/NCBI | |
|
Grazioli L, Alberti D, Olivetti L, et al: Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol. 10:820–825. 2000. View Article : Google Scholar : PubMed/NCBI | |
|
Andreani P, Srinivasan P, Ball CS, Heaton ND and Rela M: Congenital absence of the portal vein in liver transplantation for biliary atresia. Int J Surg Investig. 2:81–84. 2000. | |
|
Alvarez AE, Ribeiro AF, Hessel G, Baracat J and Ribeiro JD: Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol. 34:391–394. 2002. View Article : Google Scholar : PubMed/NCBI | |
|
Shinkai M, Ohhama Y, Nishi T, et al: Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg. 36:1026–1031. 2001. View Article : Google Scholar : PubMed/NCBI | |
|
Kinjo T, Aoki H, Sunagawa H, Kinjo S and 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. 36:622–625. 2001. View Article : Google Scholar : PubMed/NCBI | |
|
Venkat-Raman N, Murphy KW, Ghaus K, Teoh TG, Higham JM and Carvalho JS: Congenital absence of portal vein in the fetus: a case report. Ultrasound Obstet Gynecol. 17:71–75. 2001. View Article : Google Scholar : PubMed/NCBI | |
|
Tanaka Y, Takayanagi M, Shiratori Y, et al: Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver. J Gastroenterol. 38:288–294. 2003. View Article : Google Scholar : PubMed/NCBI | |
|
Soejima Y, Taguchi T, Ogita K, et al: Auxiliary partial orthotopic living donor liver transplantation for a child with congenital absence of the portal vein. Liver Transpl. 12:845–849. 2006. View Article : Google Scholar : PubMed/NCBI | |
|
Noe JA, Pittman HC and Burton EM: Congenital absence of the portal vein in a child with Turner syndrome. Pediatr Radiol. 36:566–568. 2006. View Article : Google Scholar : PubMed/NCBI | |
|
Sumida W, Kaneko K, Ogura Y, et al: Living donor liver transplantation for congenital absence of the portal vein in a child with cardiac failure. J Pediatr Surg. 41:E9–E12. 2006. View Article : Google Scholar : PubMed/NCBI | |
|
Avila LF, Luis AL, Encinas JL, et al: Congenital portosystemic shunt. The Abernethy malformation. Cir Pediatr. 19:204–209. 2006.(in Spanish). | |
|
Koizumi J, Yamashita T, Dowaki S, et al: Hepatobiliary and pancreatic: Hepatic adenoma, focal nodular hyperplasia and congenital absence of the portal vein. J Gastroenterol Hepatol. 21:6192006. View Article : Google Scholar : PubMed/NCBI | |
|
Schmidt S, Saint-Paul MC, Anty R, Bruneton JN, Gugenheim J and Chevallier P: Multiple focal nodular hyperplasia of the liver associated with congenital absence of the portal vein. Gastroenterol Clin Biol. 30:310–313. 2006.(in French). View Article : Google Scholar : PubMed/NCBI | |
|
Turkbey B, Karcaaltincaba M, Demir H, Akcoren Z, Yuce A and Haliloglu M: Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings. Pediatr Radiol. 36:445–448. 2006. View Article : Google Scholar : PubMed/NCBI | |
|
Ringe K, Schirg E, Melter M, et al: Congenital absence of the portal vein (CAPV). Two cases of Abernethy malformation type 1 and review of the literature. Radiologe. 48:493–502. 2008.(in German). View Article : Google Scholar | |
|
Kawano S, Hasegawa S, Urushihara N, et al: Hepatoblastoma with congenital absence of the portal vein-a case report. Eur J Pediatr Surg. 17:292–294. 2007. View Article : Google Scholar : PubMed/NCBI | |
|
Gocmen R, Akhan O and Talim B: Congenital absence of the portal vein associated with congenital hepatic fibrosis. Pediatr Radiol. 37:920–924. 2007. View Article : Google Scholar : PubMed/NCBI | |
|
Ratnasamy C, Kurbegov A and Swaminathan S: Cardiac anomalies in the setting of the Abernethy malformation of the portal vein. Cardiol Young. 17:212–214. 2007. View Article : Google Scholar | |
|
Goo HW: Extrahepatic portosystemic shunt in congenital absence of the portal vein depicted by time-resolved contrast-enhanced MR angiography. Pediatr Radiol. 37:706–709. 2007. View Article : Google Scholar : PubMed/NCBI | |
|
Morotti RA, Killackey M, Shneider BL, Repucci A, Emre S and Thung SN: Hepatocellular carcinoma and congenital absence of the portal vein in a child receiving growth hormone therapy for turner syndrome. Semin Liver Dis. 27:427–431. 2007. View Article : Google Scholar : PubMed/NCBI | |
|
Emre S, Arnon R, Cohen E, Morotti RA, Vaysman D and Shneider BL: Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report. Liver Transpl. 13:1662–1668. 2007. View Article : Google Scholar : PubMed/NCBI | |
|
Morikawa N, Honna T, Kuroda T, et al: Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation. J Pediatr Surg. 43:E35–E38. 2008. View Article : Google Scholar : PubMed/NCBI | |
|
Tsutsui M, Sugahara S, Motosuneya T, Wada H, Fukuda I, Umeda E and Kazama T: Anesthetic management of a child with Costello syndrome complicated by congenital absence of the portal vein - a case report. Paediatr Anaesth. 19:714–715. 2009. View Article : Google Scholar : PubMed/NCBI | |
|
Peker A, Ucar T, Kuloglu Z, et al: Congenital absence of portal vein associated with nodular regenerative hyperplasia of the liver and pulmonary hypertension. Clin Imaging. 33:322–325. 2009. View Article : Google Scholar : PubMed/NCBI | |
|
Kasahara M, Nakagawa A, Sakamoto S, et al: Living donor liver transplantation for congenital absence of the portal vein with situs inversus. Liver Transpl. 15:1641–1643. 2009. View Article : Google Scholar : PubMed/NCBI | |
|
Sanada Y, Mizuta K, Kawano Y, et al: Living donor liver transplantation for congenital absence of the portal vein. Transplant Proc. 41:4214–4219. 2009. View Article : Google Scholar : PubMed/NCBI | |
|
Hino T, Hayashida A, Okahashi N, et al: Portopulmonary hypertension associated with congenital absence of the portal vein treated with bosentan. Intern Med. 48:597–600. 2009. View Article : Google Scholar : PubMed/NCBI | |
|
Raghuram KA, Bijulal S, Krishnamoorthy KM and Tharakan JA: Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy. Pediatr Cardiol. 4:1882–1885. 2013. View Article : Google Scholar | |
|
Loomba RS, Telega GW and Gudausky TM: Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula. J Pediatr Surg. 47:E25–E31. 2012. View Article : Google Scholar : PubMed/NCBI | |
|
Passalacqua M, Lie KT and Yarmohammadi H: Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated endovascularly with vascular plug shunt closure. Pediatr Surg Int. 28:79–83. 2012. View Article : Google Scholar | |
|
Pathak A, Agarwal N, Mandliya J, Gehlot P and Dhaneria M: Abernethy malformation: a case report. BMC Pediatr. 12:572012. View Article : Google Scholar : PubMed/NCBI | |
|
Gadodia A, Sharma R, Kandpal H and Prashad R: Congenital absence of portal vein with large inferior mesenteric-caval shunt. Trop Gastroenterol. 32:223–226. 2011. | |
|
Asran MK, Loyer EM, Kaur H and Choi H: Case 177: Congenital absence of the portal vein with hepatic adenomatosis. Radiology. 262:364–367. 2012. View Article : Google Scholar | |
|
Chandrashekhara SH, Bhalla AS, Gupta AK, Vikash CS and Kabra SK: Abernethy malformation with portal vein aneurysm in a child. J Indian Assoc Pediatr Surg. 16:21–23. 2011. View Article : Google Scholar : PubMed/NCBI | |
|
Osorio MJ, Bonow A, Bond GJ, Rivera MR, Vaughan KG, Shah A and Shneider BL: Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation. Pediatr Transplant. 15:E149–E151. 2011. View Article : Google Scholar | |
|
Tannuri U, Galvão F, Leal AJ, Gibelli NE and Tannuri AC: Congenital absence of the portal vein: a complex disease with multiple manifestations and types of treatment. Eur J Pediatr Surg. 21:269–272. 2011. View Article : Google Scholar : PubMed/NCBI | |
|
Law YM, Mack CL, Sokol RJ, Rice M, Parsley L and Ivy D: Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: pulmonary hypertension and pulmonary vascular dilatation. Pediatr Transplant. 15:E162–E168. 2011. View Article : Google Scholar : | |
|
Uchida H, Sakamoto S, Shigeta T, et al: Living donor liver transplantation with renoportal anastomosis for a patient with congenital absence of the portal vein. Case Rep Surg. 2012:670289g2012. | |
|
Ogul H, Bayraktutan U, Yalcin A, Turan MI, Ozgokce M, Eren S and Kantarci M: Congenital absence of the portal vein in a patient with multiple vascular anomalies. Surg Radiol Anat. 35:529–534. 2013. View Article : Google Scholar | |
|
Banz V, Olliff S, Taniere P, Mayer D and Isaac J: Liver tumours in patients with Abernethy malformation. ANZ J Surg. 81:640–641. 2011. View Article : Google Scholar | |
|
Shinkai M, Ohhama Y, Nishi T, et al: Living related partial liver transplantation for hyperammonemia due to congenital absence of the portal vein. Transplant Proc. 32:21842000. View Article : Google Scholar : PubMed/NCBI | |
|
Joyce AD and Howard ER: Rare congenital anomaly of the portal vein. Br J Surg. 75:1038–1039. 1988. View Article : Google Scholar : PubMed/NCBI | |
|
Bellah RD, Hayek J and Teele RL: Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration. Pediatr Radiol. 20:115–117. 1989. View Article : Google Scholar : PubMed/NCBI | |
|
Marks C: Developmental basis of the portal venous system. Am J Surg. 117:671–681. 1969. View Article : Google Scholar : PubMed/NCBI | |
|
Moretti G, Staeffen J, Broustet A and Le Bras M: Congenital malformations associated with stenosis of the portal vein. Sem Hop. 44:893–897. 1968.(in French). PubMed/NCBI | |
|
K C S, Matsutani S, Maruyama H, et al: Portal-systemic encephalopathy in two patients without liver cirrhosis and portal hypertension. Hepatol Res. 23:122–129. 2002. View Article : Google Scholar |
