Congenital Portosystemic Shunts in Children: Recognition, Evaluation, and Management

Abstract

Congenital portosystemic shunts are present in one in 30,000 children. Among the associated risks of severe complications are neonatal cholestasis, benign and malignant liver tumors, hepatopulmonary syndrome, portopulmonary hypertension, and encephalopathy. They can be detected on prenatal ultrasonograms, during the investigation of a positive galactosemia screening test in neonates or of a complication, or be found fortuitously on an abdominal ultrasound. Small intrahepatic shunts may resolve spontaneously within one year of age, but other shunts such as extrahepatic, persistent ductus venosus or persisting intrahepatic shunts, must be closed in one or two steps, by interventional radiology techniques or surgically. The plasticity of the intrahepatic portal system allows revascularization of the liver after shunt closure, even when no intrahepatic portal structures can be detected on imaging studies. This leaves little or no place for liver transplantation in the management of these children.

Keywords

portosystemic shunts - children - liver tumors - hepatopulmonary syndrome - pulmonary hypertension

• References

• 1 Collardeau-Frachon S, Scoazec JY. Vascular development and differentiation during human liver organogenesis. Anat Rec (Hoboken) 2008; 291 (6) 614-627
• 2 Loberant N, Herskovits M, Barak M , et al. Closure of the ductus venosus in premature infants: findings on real-time gray-scale, color-flow Doppler, and duplex Doppler sonography. AJR Am J Roentgenol 1999; 172 (1) 227-229
• 3 Marois D, van Heerden JA, Carpenter HA, Sheedy II PF. Congenital absence of the portal vein. Mayo Clin Proc 1979; 54 (1) 55-59
• 4 Morse SS, Taylor KJ, Strauss EB, Ramirez E, Seashore JH. Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome). Pediatr Radiol 1986; 16 (5) 437-439
• 5 Barton III JW, Keller MS. Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein. Pediatr Radiol 1989; 20 (1-2) 113-114
• 6 Joyce AD, Howard ER. Rare congenital anomaly of the portal vein. Br J Surg 1988; 75 (10) 1038-1039
• 7 Bellah RD, Hayek J, Teele RL. Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration. Pediatr Radiol 1989; 20 (1-2) 115-117
• 8 Nakasaki H, Tanaka Y, Ohta M , et al. Congenital absence of the portal vein. Ann Surg 1989; 210 (2) 190-193
• 9 Woodle ES, Thistlethwaite JR, Emond JC , et al. Successful hepatic transplantation in congenital absence of recipient portal vein. Surgery 1990; 107 (4) 475-479
• 10 Jabra AA, Taylor GA. Ultrasound diagnosis of congenital intrahepatic portosystemic venous shunt. Pediatr Radiol 1991; 21 (7) 529-530
• 11 Gitzelmann R, Arbenz UV, Willi UV. Hypergalactosaemia and portosystemic encephalopathy due to persistence of ductus venosus Arantii. Eur J Pediatr 1992; 151 (8) 564-568
• 12 Kieran MW, Vekemans M, Robb LJ, Sinsky A, Outerbridge EW, Der Kaloustian VM. Portohepatic shunt in a Down syndrome patient with an interchange trisomy 47,XY,-2, + der(2), + der(21)t(2;21)(p13;q22.1)mat. Am J Med Genet 1992; 44 (3) 288-292
• 13 Kitagawa S, Gleason Jr WA, Northrup H, Middlebrook MR, Ueberschar E. Symptomatic hyperammonemia caused by a congenital portosystemic shunt. J Pediatr 1992; 121 (6) 917-919
• 14 Lalonde L, Van Beers B, Trigaux JP, Delos M, Melange M, Pringot J. Focal nodular hyperplasia in association with spontaneous intrahepatic portosystemic venous shunt. Gastrointest Radiol 1992; 17 (2) 154-156
• 15 Lewis AM, Aquino NM. Congenital portohepatic vein fistula that resolved spontaneously in a neonate. AJR Am J Roentgenol 1992; 159 (4) 837-838
• 16 Maisawa S, Takasago Y, Oyake Y, Maeta H, Fujiwara T. Patent ductus venosus with hypoplastic right hepatoportal system in a young child born with asymmetric intra-uterine growth retardation. Eur J Pediatr 1992; 151 (8) 569-571
• 17 Shah R, Ford E, Wooley M. Congenital portocaval shunt. Pediatr Surg Int 1992; 7: 216-217
• 18 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 1993; 7 (1) 73-80
• 19 Papagiannis J, Kanter RJ, Effman EL , et al. Polysplenia with pulmonary arteriovenous malformations. Pediatr Cardiol 1993; 14 (2) 127-129
• 20 Kawakatsu M, Vilgrain V, Belghiti J, Flejou JF, Nahum H. Association of multiple liver cell adenomas with spontaneous intrahepatic portohepatic shunt. Abdom Imaging 1994; 19 (5) 438-440
• 21 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 (9) 1239-1241
• 22 Laverdiere JT, Laor T, Benacerraf B. Congenital absence of the portal vein: case report and MR demonstration. Pediatr Radiol 1995; 25 (1) 52-53
• 23 Mboyo A, Lemouel A, Sohm O , et al. Congenital extra-hepatic portocaval shunt. Concerning a case of antenatal diagnosis. Eur J Pediatr Surg 1995; 5 (4) 243-245
• 24 Yanai S, Minami T, Sonoda K , et al. Patent ductus venosus associated with a hyperintense globus pallidum on T1-weighted magnetic resonance imaging and pulmonary hypertension. Eur J Pediatr 1995; 154 (7) 526-529
• 25 Matsubara T, Sumazaki R, Saitoh H, Imai H, Nakayama J, Takita H. Patent ductus venosus associated with tumor-like lesions of the liver in a young girl. J Pediatr Gastroenterol Nutr 1996; 22 (1) 107-111
• 26 Santamaría G, Pruna X, Serres X, Inaraja L, Zuasnabar A, Castellote A. Congenital intrahepatic portosystemic venous shunt: sonographic and magnetic resonance imaging. Eur Radiol 1996; 6 (1) 76-78
• 27 Uchino T, Endo F, Ikeda S, Shiraki K, Sera Y, Matsuda I. Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus. Gastroenterology 1996; 110 (6) 1964-1968
• 28 Howard ER, Davenport M. Congenital extrahepatic portocaval shunts—the Abernethy malformation. J Pediatr Surg 1997; 32 (3) 494-497
• 29 Orii T, Ohkohchi N, Kato H , et al. Liver transplantation for severe hypoxemia caused by patent ductus venosus. J Pediatr Surg 1997; 32 (12) 1795-1797
• 30 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 1998; 157 (4) 287-290
• 31 Usuki N, Miyamoto T. A case of congenital absence of the intrahepatic portal vein diagnosed by MR angiography. J Comput Assist Tomogr 1998; 22 (5) 728-729
• 32 Yoon HK, Choo SW, Do YS, Choo IW, Han BK. Congenital intrahepatic portosystemic shunts in the neonate: coil embolization via the umbilical vein. J Vasc Interv Radiol 1998; 9 (3) 509-511
• 33 Ihara K, Hijii T, Kuromaru R , et al. High-intensity basal ganglia lesions on T1-weighted images in two toddlers with elevated blood manganese with portosystemic shunts. Neuroradiology 1999; 41 (3) 195-198
• 34 Ikeda S, Sera Y, Yoshida M , et al. Reversibility of hyperintense globus pallidus on T 1-weighted MRI following surgery for a portosystemic shunt in an 8-year-old girl. Pediatr Radiol 1999; 29 (6) 449-450
• 35 Ikeda S, Sera Y, Ohshiro H, Uchino S, Uchino T, Endo F. Surgical indications for patients with hyperammonemia. J Pediatr Surg 1999; 34 (6) 1012-1015
• 36 Kohda E, Saeki M, Nakano M , et al. Congenital absence of the portal vein in a boy. Pediatr Radiol 1999; 29 (4) 235-237
• 37 Massin M, Verloes A, Jamblin P. Cardiac anomalies associated with congenital absence of the portal vein. Cardiol Young 1999; 9 (5) 522-525
• 38 Schwartz YM, Berkowitz D, Lorber A. Transvenous coil embolization of a patent ductus venosus in a 2-month-old child. Pediatrics 1999; 103 (5 Pt 1) 1045-1047
• 39 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 (6) 425-428
• 40 Ikeda S, Sera Y, Yoshida M , et al. Successful coil embolization in an infant with congenital intrahepatic portosystemic shunts. J Pediatr Surg 1999; 34 (6) 1031-1032
• 41 Broere D, van Gemert WG, Kneepkens CM , et al. A 6-year-old boy with hyperammonaemia: partial N-acetylglutamate synthase deficiency or portosystemic encephalopathy?. Eur J Pediatr 2000; 159 (12) 905-907
• 42 Courtens W, Segers V, Johansson A, Avni FE. Association between Down syndrome and portohepatic shunt. Am J Med Genet 2000; 93 (2) 166-168
• 43 Grazioli L, Alberti D, Olivetti L , et al. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver. Eur Radiol 2000; 10 (5) 820-825
• 44 Kamata S, Kitayama Y, Usui N , et al. Patent ductus venosus with a hypoplastic intrahepatic portal system presenting intrapulmonary shunt: a case treated with banding of the ductus venosus. J Pediatr Surg 2000; 35 (4) 655-657
• 45 Karashima S, Hattori S, Nakazato H , et al. Membranoproliferative glomerulonephritis in congenital portosystemic shunt without liver cirrhosis. Clin Nephrol 2000; 53 (3) 206-211
• 46 Kim IO, Cheon JE, Kim WS , et al. Congenital intrahepatic portohepatic venous shunt: treatment with coil embolisation. Pediatr Radiol 2000; 30 (5) 336-338
• 47 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 2000; 32 (7) 2184
• 48 Valls E, Ceres L, Urbaneja A, Muñoz R, Alonso I. Color Doppler sonography in the diagnosis of neonatal intrahepatic portosystemic shunts. J Clin Ultrasound 2000; 28 (1) 42-46
• 49 Yonemitsu H, Mori H, Kimura T , et al. Congenital extrahepatic portocaval shunt associated with hepatic hyperplastic nodules in a patient with Dubin-Johnson syndrome. Abdom Imaging 2000; 25 (6) 572-575
• 50 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 (4) 622-625
• 51 Marx M, Huber WD, Crone J , et al. Interventional stent implantation in a child with patent ductus venosus and pulmonary hypertension. Eur J Pediatr 2001; 160 (8) 501-504
• 52 Satoh M, Yokoya S, Hachiya Y , et al. Two hyperandrogenic adolescent girls with congenital portosystemic shunt. Eur J Pediatr 2001; 160 (5) 307-311
• 53 Scheer I, Kivelitz D, Taupitz M , et al. Patent ductus venosus: diagnosis by MR angiography. Pediatr Radiol 2001; 31 (4) 279-282
• 54 Shinkai M, Ohhama Y, Nishi T , et al. Congenital absence of the portal vein and role of liver transplantation in children. J Pediatr Surg 2001; 36 (7) 1026-1031
• 55 Venkat-Raman N, Murphy KW, Ghaus K, Teoh TG, Higham JM, Carvalho JS. Congenital absence of portal vein in the fetus: a case report. Ultrasound Obstet Gynecol 2001; 17 (1) 71-75
• 56 Alvarez AE, Ribeiro AF, Hessel G, Baracat J, Ribeiro JD. Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 2002; 34 (5) 391-394
• 57 Badler R, Price AP, Moy L, Katz DS. Congenital portacaval shunt: CT demonstration. Pediatr Radiol 2002; 32 (1) 28-30
• 58 Ersch J, Bänziger O, Braegger C, Arbenz U, Stallmach T. An infant with pulmonary hypertension due to a congenital porto-caval shunt. Eur J Pediatr 2002; 161 (12) 660-662
• 59 Araki T, Kamada M, Okamoto Y, Arai S, Oba O. Coil embolization of a patent ductus venosus in a 52-day-old girl with congenital heart disease. Ann Thorac Surg 2003; 75 (1) 273-275
• 60 Kanamori Y, Hashizume K, Kitano Y, Sugiyama M, Motoi T, Tange T. Congenital extrahepatic portocaval shunt (Abernethy type 2), huge liver mass, and patent ductus arteriosus—a case report of its rare clinical presentation in a young girl. J Pediatr Surg 2003; 38 (4) E15
• 61 Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts. Pediatr Radiol 2003; 33 (9) 614-620
• 62 Pipitone S, Garofalo C, Corsello G , et al. Abnormalities of the umbilico-portal venous system in Down syndrome: a report of two new patients. Am J Med Genet A 2003; 120A (4) 528-532
• 63 Pohl A, Jung A, Vielhaber H , et al. Congenital atresia of the portal vein and extrahepatic portocaval shunt associated with benign neonatal hemangiomatosis, congenital adrenal hyperplasia, and atrial septal defect. J Pediatr Surg 2003; 38 (4) 633-634
• 64 Suga K, Ogasawara N, Matsunaga N, Sugio Y, Shimizu R. Reversed intrapulmonary right-to-left shunt after banding of the patent ductus venosus. Clin Nucl Med 2003; 28 (10) 827-833
• 65 Tanaka Y, Takayanagi M, Shiratori Y , et al. Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver. J Gastroenterol 2003; 38 (3) 288-294
• 66 Alonso J, Sierre S, Lipsich J, Questa H, Faella H, Moguillansky S. Endovascular treatment of congenital portal vein fistulas with the Amplatzer occlusion device. J Vasc Interv Radiol 2004; 15 (9) 989-993
• 67 Charre L, Roggen F, Lemaire J , et al. Hematochezia and congenital extrahepatic portocaval shunt with absent portal vein: successful treatment by liver transplantation. Transplantation 2004; 78 (9) 1404-1406
• 68 Eroglu Y, Donaldson J, Sorensen LG , et al. Improved neurocognitive function after radiologic closure of congenital portosystemic shunts. J Pediatr Gastroenterol Nutr 2004; 39 (4) 410-417
• 69 Kakuno Y, Matsuki M, Kani H, Narabayashi I, Katayama H. Intrahepatic portosystemic venous shunt: occurrence in a child patient without liver cirrhosis. Radiat Med 2004; 22 (3) 195-197
• 70 Kim T, Murakami T, Sugihara E, Hori M, Wakasa K, Nakamura H. Hepatic nodular lesions associated with abnormal development of the portal vein. AJR Am J Roentgenol 2004; 183 (5) 1333-1338
• 71 Kimura T, Soh H, Hasegawa T , et al. Laparoscopic correction of congenital portosystemic shunt in children. Surg Laparosc Endosc Percutan Tech 2004; 14 (5) 285-288
• 72 Kinane TB, Westra SJ. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 31-2004. A four-year-old boy with hypoxemia. N Engl J Med 2004; 351 (16) 1667-1675
• 73 Manning N, Impey L, Lindsell D, Lakhoo K. Prenatally diagnosed portocaval shunt and postnatal outcome: a case report. Prenat Diagn 2004; 24 (7) 537-540
• 74 Saxena AK, Sodhi KS, Arora J, Thapa BR, Suri S. Congenital intrahepatic portosystemic venous shunt in an infant with Down syndrome. AJR Am J Roentgenol 2004; 183 (6) 1783-1784
• 75 Takagaki K, Kodaira M, Kuriyama S , et al. Congenital absence of the portal vein complicating hepatic tumors. Intern Med 2004; 43 (3) 194-198
• 76 Takehara Y, Mori K, Edagawa T , et al. Presumed hypoplastic intrahepatic portal system due to patent ductus venosus: importance of direct occlusion test of ductus venosus under open laparotomy. Pediatr Int 2004; 46 (4) 484-486
• 77 Yoshimoto Y, Shimizu R, Saeki T , et al. Patent ductus venosus in children: a case report and review of the literature. J Pediatr Surg 2004; 39 (1) E1-E5
• 78 Yagi H, Takada Y, Fujimoto Y , et al. Successful surgical ligation under intraoperative portal vein pressure monitoring of a large portosystemic shunt presenting as an intrapulmonary shunt: report of a case. Surg Today 2004; 34 (12) 1049-1052
• 79 Cheung KM, Lee CY, Wong CT, Chan AK. Congenital absence of portal vein presenting as hepatopulmonary syndrome. J Paediatr Child Health 2005; 41 (1-2) 72-75
• 80 Digilio MC, Capolino R, Marino B, Sarkozy A, Dallapiccola B. Congenital intrahepatic portosystemic venous shunt: an unusual feature in LEOPARD syndrome and in neurofibromatosis type 1. Am J Med Genet A 2005; 134 (4) 457-458
• 81 Ikeda H, Aotsuka H, Nakajima H, Sawada M. Portosystemic shunt with polysplenia and hypoplastic left heart syndrome. Pediatr Cardiol 2005; 26 (4) 446-448
• 82 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 2005; 40 (5) e1-e3
• 83 Tsuji K, Naoki K, Tachiyama Y , et al. A case of congenital absence of the portal vein. Hepatol Res 2005; 31 (1) 43-47
• 84 Gillespie MJ, Golden A, Sivarajan VB, Rome JJ. Transcatheter closure of patent ductus venosus with the Amplatzer vascular plug in twin brothers. Pediatr Cardiol 2006; 27 (1) 142-145
• 85 Noe JA, Pittman HC, Burton EM. Congenital absence of the portal vein in a child with Turner syndrome. Pediatr Radiol 2006; 36 (6) 566-568
• 86 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 2006; 12 (5) 845-849
• 87 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 2006; 41 (11) e9-e12
• 88 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 (2) e1-e3
• 89 Turkbey B, Karcaaltincaba M, Demir H, Akcoren Z, Yuce A, Haliloglu M. Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings. Pediatr Radiol 2006; 36 (5) 445-448
• 90 Emre S, Arnon R, Cohen E, Morotti RA, Vaysman D, Shneider BL. Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report. Liver Transpl 2007; 13 (12) 1662-1668
• 91 Goo HW. Extrahepatic portosystemic shunt in congenital absence of the portal vein depicted by time-resolved contrast-enhanced MR angiography. Pediatr Radiol 2007; 37 (7) 706-709
• 92 Kandpal H, Sharma R, Arora NK, Gupta SD. Congenital extrahepatic portosystemic venous shunt: imaging features. Singapore Med J 2007; 48 (9) e258-e261
• 93 Kawano S, Hasegawa S, Urushihara N , et al. Hepatoblastoma with congenital absence of the portal vein - a case report. Eur J Pediatr Surg 2007; 17 (4) 292-294
• 94 Peretta P, Carlino C, Gennari F , et al. Spontaneous occlusion of brainstem arteriovenous malformation following ligature of a hepatic patent ductus venosus. Case report and review of the literature. J Neurosurg 2007; 106 (2, Suppl) 147-152
• 95 Ratnasamy C, Kurbegov A, Swaminathan S. Cardiac anomalies in the setting of the Abernethy malformation of the portal vein. Cardiol Young 2007; 17 (2) 212-214
• 96 Sagiv-Friedgut K, Witzling M, Dalal I, Vinkler C, Someh E, Levine A. Congenital patent ductus venosus: an association with the hyper IgE syndrome. J Pediatr 2007; 150 (2) 210-212
• 97 Yamagami T, Yoshimatsu R, Matsumoto T , et al. Successful embolization using interlocking detachable coils for a congenital extrahepatic portosystemic venous shunt in a child. J Pediatr Surg 2007; 42 (11) 1949-1952
• 98 Chiu SN, Chien YH, Wu MH, Wang JK, Chen SJ. Transcatheter closure of portal-systemic shunt combining congenital double extrahepatic inferior vena cava with vascular plug. J Pediatr 2008; 153 (5) 723
• 99 Hoover W, Ackerman V, Schamberger M, Kumar M, Marshalleck F, Hoyer M. The congenital porto-caval fistula: a unique presentation and novel intervention. Pediatr Pulmonol 2008; 43 (2) 196-199
• 100 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 2008; 43 (2) e35-e38
• 101 Ohno T, Muneuchi J, Ihara K , et al. Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 2008; 121 (4) e892-e899
• 102 Singhal M, Lal A, Thapa BR, Prakash M, Shanbhogue KP, Khandelwal N. Congenital atresia of portal vein with portocaval shunt associated with cardiac defects, skeletal deformities, and skin lesions in a boy. J Pediatr Surg 2008; 43 (8) e25-e28
• 103 Stringer MD. The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 2008; 21 (2) 147-157
• 104 Elias N, Scirica CV, Hertl M. Liver transplantation for the Abernathy malformation. N Engl J Med 2008; 358 (8) 858
• 105 Alomari AI, Chaudry G, Fox VL, Fishman SJ, Buchmiller TL. Atypical manifestation of patent ductus venosus in a child: intervening against a paradoxical presentation. J Vasc Interv Radiol 2009; 20 (4) 537-542
• 106 Cho YK, Chang NK, Ma JS. Successful transcatheter closure of a large patent ductus venosus with the Amplatzer vascular plug II. Pediatr Cardiol 2009; 30 (4) 540-542
• 107 Evans WN, Galindo A, Acherman RJ, Rothman A, Berthoty DP. Congenital portosystemic shunts and AMPLATZER vascular plug occlusion in newborns. Pediatr Cardiol 2009; 30 (8) 1083-1088
• 108 Gupta V, Kalra N, Vyas S, Sodhi KS, Thapa BR, Khandelwal N. Embolization of congenital intrahepatic porto-systemic shunt by n-butyl cyanoacrylate. Indian J Pediatr 2009; 76 (10) 1059-1060
• 109 Hino T, Hayashida A, Okahashi N , et al. Portopulmonary hypertension associated with congenital absence of the portal vein treated with bosentan. Intern Med 2009; 48 (8) 597-600
• 110 Iida T, Ogura Y, Doi H , et al. Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation. Transpl Int 2010; 23 (1) 105-109
• 111 Kasahara M, Nakagawa A, Sakamoto S , et al. Living donor liver transplantation for congenital absence of the portal vein with situs inversus. Liver Transpl 2009; 15 (11) 1641-1643
• 112 Kono T, Hiki T, Kuwashima S, Hashimoto T, Kaji Y. Hypergalactosemia in early infancy: diagnostic strategy with an emphasis on imaging. Pediatr Int 2009; 51 (2) 276-282
• 113 Nii A, Takehara HO, Kuyama H, Shimada M. Successful preemptive surgical division of type 2-congenital extrahepatic portosystemic shunt in children. J Med Invest 2009; 56 (1-2) 49-54
• 114 Peker A, Ucar T, Kuloglu Z, Ceyhan K, Tutar E, Fitoz S. Congenital absence of portal vein associated with nodular regenerative hyperplasia of the liver and pulmonary hypertension. Clin Imaging 2009; 33 (4) 322-325
• 115 Singhal A, Srivastava A, Goyal N , et al. Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting. Pediatr Transplant 2009; 13 (8) 1041-1047
• 116 Sista AK, Filly RA. Type 1 congenital extrahepatic portosystemic shunt. J Ultrasound Med 2009; 28 (5) 703-705
• 117 Tsutsui M, Sugahara S, Motosuneya T , et al. Anesthetic management of a child with Costello syndrome complicated by congenital absence of the portal vein—a case report. Paediatr Anaesth 2009; 19 (7) 714-715
• 118 Franchi-Abella S, Branchereau S, Lambert V , et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 2010; 51 (3) 322-330
• 119 Ferrero GB, Porta F, Biamino E , et al. Remittent hyperammonemia in congenital portosystemic shunt. Eur J Pediatr 2010; 169 (3) 369-372
• 120 Fuchs J, Warmann SW, Sieverding L , et al. Impact of virtual imaging procedures on treatment strategies in children with hepatic vascular malformations. J Pediatr Gastroenterol Nutr 2010; 50 (1) 67-73
• 121 Kamimatsuse A, Onitake Y, Kamei N , et al. Surgical intervention for patent ductus venosus. Pediatr Surg Int 2010; 26 (10) 1025-1030
• 122 Koga H, Miyako K, Sakemi Y, Hidaka T, Suga N, Takahashi N. Congenital portosystemic shunt in a child with Wolf-Hirschhorn syndrome. Am J Med Genet A 2010; 152A (9) 2387-2389
• 123 Kuo MD, Miller FJ, Lavine JE, Peterson M, Finch M. Exploiting phenotypic plasticity for the treatment of hepatopulmonary shunting in Abernethy malformation. J Vasc Interv Radiol 2010; 21 (6) 917-922
• 124 Lee SA, Lee YS, Lee KS, Jeon GS. Congenital intrahepatic portosystemic venous shunt and liver mass in a child patient: successful endovascular treatment with an Amplatzer vascular plug (AVP). Korean J Radiol 2010; 11 (5) 583-586
• 125 Matsuura T, Soejima Y, Taguchi T. Auxiliary partial orthotopic living donor liver transplantation with a small-for-size graft for congenital absence of the portal vein. Liver Transpl 2010; 16 (12) 1437-1439
• 126 Nagasaka H, Okano Y, Aizawa M , et al. Altered metabolisms of mediators controlling vascular function and enhanced oxidative stress in asymptomatic children with congenital portosystemic venous shunt. Metabolism 2010; 59 (1) 107-113
• 127 Newman B, Feinstein JA, Cohen RA , et al. Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia. Pediatr Radiol 2010; 40 (7) 1222-1230
• 128 Osorio MJ, Bonow A, Bond GJ , et al. Abernethy malformation complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation. Pediatr Transplant 2011; 15 (7) E149-E151
• 129 Hori T, Yonekawa Y, Okamoto S , et al. Pediatric orthotopic living-donor liver transplantation cures pulmonary hypertension caused by Abernethy malformation type Ib. Pediatr Transplant 2011; 15 (3) e47-e52
• 130 Kobayashi D, Edwards HD, Singh J, Nadkarni MD, Lantz PE, Cook AL. Portopulmonary hypertension secondary to congenital extrahepatic portosystemic shunt with heterotaxy and polysplenia: a cause of sudden death in an infant. Pediatr Pulmonol 2011; 46 (10) 1041-1044
• 131 Konstas AA, Digumarthy SR, Avery LL , et al. Congenital portosystemic shunts: imaging findings and clinical presentations in 11 patients. Eur J Radiol 2011; 80 (2) 175-181
• 132 Lautz TB, Tantemsapya N, Rowell E, Superina RA. Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 2011; 46 (2) 308-314
• 133 Law YM, Mack CL, Sokol RJ, Rice M, Parsley L, Ivy D. Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: pulmonary hypertension and pulmonary vascular dilatation. Pediatr Transplant 2011; 15 (8) E162-E168
• 134 Sakamoto S, Kasahara M, Shigeta T , et al. Living donor liver transplantation for multiple intrahepatic portosystemic shunts after involution of infantile hepatic hemangiomas. J Pediatr Surg 2011; 46 (6) 1288-1291
• 135 Sanada Y, Urahashi T, Ihara Y , et al. The role of operative intervention in management of congenital extrahepatic portosystemic shunt. Surgery 2012; 151 (3) 404-411
• 136 Stewart JK, Kuo WT, Hovsepian DM, Hofmann LV, Bonham CA, Sze DY. Portal venous remodeling after endovascular reduction of pediatric autogenous portosystemic shunts. J Vasc Interv Radiol 2011; 22 (8) 1199-1205
• 137 Grimaldi C, Monti L, Falappa P, d'Ambrosio G, Manca A, de Ville de Goyet J. Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review. J Pediatr Surg 2012; 47 (2) e27-e31
• 138 Kim MJ, Ko JS, Seo JK , et al. Clinical features of congenital portosystemic shunt in children. Eur J Pediatr 2012; 171 (2) 395-400
• 139 Nakamura Y, Aoki M, Hagino I, Nakajima H. Severe cyanosis after Kawashima operation resulting from congenital portosystemic shunt. Ann Thorac Surg 2012; 93 (4) 1315-1317
• 140 Passalacqua M, Lie KT, Yarmohammadi H. Congenital extrahepatic portosystemic shunt (Abernethy malformation) treated endovascularly with vascular plug shunt closure. Pediatr Surg Int 2012; 28 (1) 79-83
• 141 Park JH, Cha SH, Han JK, Han MC. Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 1990; 155 (3) 527-528
• 142 Lisovsky M, Konstas AA, Misdraji J. Congenital extrahepatic portosystemic shunts (Abernethy malformation): a histopathologic evaluation. Am J Surg Pathol 2011; 35 (9) 1381-1390
• 143 Bioulac-Sage P, Saric J, Boussarie L, Balabaud C. Congenital portacaval shunt in rats: liver adaptation to lack of portal vein—a light and electron microscopic study. Hepatology 1985; 5 (6) 1183-1189
• 144 Gitzelmann R, Forster I, Willi UV. Hypergalactosaemia in a newborn: self-limiting intrahepatic portosystemic venous shunt. Eur J Pediatr 1997; 156 (9) 719-722
• 145 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 (6) 631-634
• 146 McElhinney DB, Marx GR, Newburger JW. Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: a case series and literature review. Congenit Heart Dis 2011; 6 (1) 28-40
• 147 Acherman RJ, Evans WN, Galindo A , et al. Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion. J Ultrasound Med 2007; 26 (8) 1077-1082
• 148 Contratti G, Banzi C, Ghi T, Perolo A, Pilu G, Visentin A. Absence of the ductus venosus: report of 10 new cases and review of the literature. Ultrasound Obstet Gynecol 2001; 18 (6) 605-609
• 149 Shen O, Valsky DV, Messing B, Cohen SM, Lipschuetz M, Yagel S. Shunt diameter in agenesis of the ductus venosus with extrahepatic portosystemic shunt impacts on prognosis. Ultrasound Obstet Gynecol 2011; 37 (2) 184-190
• 150 Shiomi S, Kuroki T, Kurai O , et al. Portal circulation by technetium-99m pertechnetate per-rectal portal scintigraphy. J Nucl Med 1988; 29 (4) 460-465
• 151 Inoue E, Hori S, Narumi Y , et al. Portal-systemic encephalopathy: presence of basal ganglia lesions with high signal intensity on MR images. Radiology 1991; 179 (2) 551-555
• 152 Krieger S, Jauss M, Jansen O , et al. MRI findings in chronic hepatic encephalopathy depend on portosystemic shunt: results of a controlled prospective clinical investigation. J Hepatol 1997; 27 (1) 121-126
• 153 Uchino A, Noguchi T, Nomiyama K , et al. Manganese accumulation in the brain: MR imaging. Neuroradiology 2007; 49 (9) 715-720
• 154 Mizoguchi N, Nishimura Y, Ono H, Sakura N. Manganese elevations in blood of children with congenital portosystemic shunts. Eur J Pediatr 2001; 160 (4) 247-250
• 155 Uchino T, Matsuda I, Endo F. The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 1999; 135 (2 Pt 1) 254-256
• 156 Raskin NH, Price JB, Fishman RA. Portal-systemic encephalopathy due to congenital intrahepatic shunts. N Engl J Med 1964; 270: 225-229
• 157 Spencer LT, Langham MR, Hoyer MH, Jones DA, Caridi JG, Chesrown SE. Resolution of hypoxemia in a liver transplant recipient after ligation of a portosystemic shunt. J Pediatr 2000; 137 (4) 575-577
• 158 Hervé P, Le Pavec J, Sztrymf B, Decante B, Savale L, Sitbon O. Pulmonary vascular abnormalities in cirrhosis. Best Pract Res Clin Gastroenterol 2007; 21 (1) 141-159
• 159 Weinbren K, Washington SL. Hyperplastic nodules after portacaval anastomosis in rats. Nature 1976; 264 (5585) 440-442
• 160 Braissant O. Ammonia toxicity to the brain: effects on creatine metabolism and transport and protective roles of creatine. Mol Genet Metab 2010; 100 (Suppl. 01) S53-S58
• 161 Racette BA, Aschner M, Guilarte TR, Dydak U, Criswell SR, Zheng W. Pathophysiology of manganese-associated neurotoxicity. Neurotoxicology 2012; 33 (4) 881-886