Abstract
Clinical features, images, complications, treatments, and prognosis of 10 children with congenital portosystemic shunt (CPSS) were reviewed. Nine children were diagnosed with intrahepatic shunts while one presented with extrahepatic shunt. CPSS was detected by prenatal ultrasonography in four infants. Three infants presented with galactosemia without an enzyme deficiency. Two children presented with mental retardation and attention deficit hyperactivity disorder. Pulmonary hypertension developed in two patients. Spontaneous closure occurred in four infants with intrahepatic shunts including patent ductus venosus. The shunts were closed using transcatheter embolizations in four patients with intrahepatic shunts. Conclusion: Intrahepatic shunts may close spontaneously. Transcatheter embolization is effective for the treatment of symptomatic intrahepatic shunts.
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Delle Chiaie L, Neuberger P, Von Kalle T (2008) Congenital intrahepatic portosystemic shunt: prenatal diagnosis and possible influence on fetal growth. Ultrasound Obstet Gynecol 32:233–235
Evans WN, Galindo A, Acherman RJ, Rothman A, Berthoty DP (2009) Congenital portosystemic shunts and AMPLATZER vascular plug occlusion in newborns. Pediatr Cardiol 30:1083–1088
Ferrero GB, Porta F, Biamino E, Mussa A, Garelli E, Chiappe F, Veltri A, Silengo MC, Gennari F (2010) Remittent hyperammonemia in congenital portosystemic shunt. Eur J Pediatr 169:369–372
Franchi-Abella S, Branchereau S, Lambert V, Fabre M, Steimberg C, Losay J, Riou JY, Pariente D, Gauthier F, Jacquemin E, Bernard O (2010) Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 51:322–330
Gitzelmann R, Forster I, Willi UV (1997) Hypergalactosaemia in a newborn: self-limiting intrahepatic portosystemic venous shunt. Eur J Pediatr 156:719–722
Kamata S, Kitayama Y, Usui N, Kuroda S, Nose K, Sawai T, Okada A (2000) Patent ductus venosus with a hypoplastic intrahepatic portal system presenting intrapulmonary shunt: a case treated with banding of the ductus venosus. J Pediatr Surg 35:655–657
Kono T, Hiki T, Kuwashima S, Hashimoto T, Kaji Y (2009) Hypergalactosemia in early infancy: diagnostic strategy with an emphasis on imaging. Pediatr Int 51:276–282
Konstas AA, Digumarthy SR, Avery LL, Wallace KL, Lisovsky M, Misdraji J, Hahn PF (2010) Congenital portosystemic shunts: imaging findings and clinical presentations in 11 patients. Eur J Radiol [Epub ahead of print]
Lautz TB, Tantemsapya N, Rowell E, Superina RA (2011) Management and classification of type II congenital portosystemic shunts. J Pediatr Surg 46:308–314
Nishimura Y, Tajima G, Dwi Bahagia A, Sakamoto A, Ono H, Sakura N, Naito K, Hamakawa M, Yoshii C, Kubota M, Kobayashi K, Saheki T (2004) Differential diagnosis of neonatal mild hypergalactosaemia detected by mass screening: clinical significance of portal vein imaging. J Inherit Metab Dis 27:11–18
Oguz B, Akata D, Balkanci F, Akhan O (2003) Intrahepatic portosystemic venous shunt: diagnosis by colour/power Doppler imaging and three-dimensional ultrasound. Br J Radiol 76:487–490
Ohno T, Muneuchi J, Ihara K, Yuge T, Kanaya Y, Yamaki S, Hara T (2008) Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 121:e892–899
Ono H, Mawatari H, Mizoguchi N, Eguchi T, Sakura N (1998) Clinical features and outcome of eight infants with intrahepatic porto-venous shunts detected in neonatal screening for galactosaemia. Acta Paediatr 87:631–634
Park JH, Cha SH, Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 155:527–528
Sakura N, Mizoguchi N, Eguchi T, Ono H, Mawatari H, Naitou K, Ito K (1997) Elevated plasma bile acids in hypergalactosaemic neonates: a diagnostic clue to portosystemic shunts. Eur J Pediatr 156:716–718
Stringer MD (2008) The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 21:147–157
Takehara Y, Mori K, Edagawa T, Sugimoto M, Takehara H, Ito M, Kuroda Y (2004) Presumed hypoplastic intrahepatic portal system due to patent ductus venosus: importance of direct occlusion test of ductus venosus under open laparotomy. Pediatr Int 46:484–486
Tanoue S, Kiyosue H, Komatsu E, Hori Y, Maeda T, Mori H (2003) Symptomatic intrahepatic portosystemic venous shunt: embolization with an alternative approach. AJR Am J Roentgenol 181:71–78
Uchino T, Matsuda I, Endo F (1999) The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 135:254–256
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This study was supported by grant no. 04–20071070 from the Seoul National University Hospital Research Fund.
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Kim, M.J., Ko, J.S., Seo, J.K. et al. Clinical features of congenital portosystemic shunt in children. Eur J Pediatr 171, 395–400 (2012). https://doi.org/10.1007/s00431-011-1564-9
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DOI: https://doi.org/10.1007/s00431-011-1564-9