Abstract
Congenital aberrant hepatic vascular communications result from intrahepatic or extrahepatic errors in vascular development or involution during the transition from fetal to newborn hepatic circulation. These hepatic vascular shunts can be fortuitously discovered and asymptomatic, or can cause symptoms of varying severity, often presenting diagnostic dilemmas. Some hepatic vascular shunts resolve spontaneously while others require interventional radiologic or surgical closure. Affected patients are often first studied with real-time and Doppler ultrasound, so radiologists should familiarize themselves with the expected ultrasound findings of these vascular shunts for effective diagnosis, triage and management. In this review, the authors focus on ultrasound and Doppler findings of hepatic vascular shunts with underlying embryology, clinical features and management strategies. Broadly, these aberrant hepatic vascular communications include portosystemic venous shunts (which can be intra- or extrahepatic and include persistent patent ductus venosus), arterioportal, arteriovenous or mixed shunts.
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References
Bernard O, Franchi-Abella S, Branchereau S et al (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32:273–287
Back SJ, Maya CL, Khwaja A (2017) Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen. Pediatr Radiol 47:1069–1078
Achiron R, Gindes L, Kivilevitch Z et al (2009) Prenatal diagnosis of congenital agenesis of the fetal portal venous system. Ultrasound Obstet Gynecol 34:643–652
Collardeau-Frachon S, Scoazec JY (2008) Vascular development and differentiation during human liver organogenesis. Anat Rec 291:614–627
Franchi-Abella S, Branchereau S, Lambert V et al (2010) Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 51:322–330
Abernethy J (1797) Account of two instances of uncommon formation in the viscera of the human body: Philos. Trans. Roy. Soc. London. Med Facts Obs 7:100–108
White JJ, Brenner H, Avery ME (1969) Umbilical vein collateral circulation: the caput medusae in a newborn infant. Pediatrics 43:391–395
Morgan G, Superina R (1994) Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 29:1239–1241
Nacif LS, Paranagua-Vezozzo DC, Galvao FH et al (2015) Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation. BMC Med Imaging 15:37
Alonso-Gamarra E, Parron M, Perez A et al (2011) Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics 31:707–722
Loomba RS, Telega GW, Gudausky TM (2012) Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula. J Pediatr Surg 47:E25–E31
Kwapisz L, Wells MM, AlJudaibi B (2014) Abernethy malformation: Congenital absence of the portal vein. Can J Gastroenterol Hepatol 28:587–588
Blanc T, Guerin F, Franchi-Abella S et al (2014) Congenital portosystemic shunts in children: a new anatomical classification correlated with surgical strategy. Ann Surg 260:188–198
Park JH, Cha SH, Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 155:527–528
Wu L, Zhao L, Lu Y et al (2016) Interventional embolization of congenital intrahepatic shunts in children. Pediatr Radiol 46:541–547
Gallego C, Miralles M, Marin C et al (2004) Congenital hepatic shunts. Radiographics 24:755–772
Florio F, Nardella M, Balzano S et al (1998) Congenital intrahepatic portosystemic shunt. Cardiovasc Intervent Radiol 21:421–424
Loberant N, Herskovits M, Barak M et al (1999) 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 172:227–229
Vade A, Lim-Dunham J, Iqbal N (2001) Imaging of the ductus venosus in neonates: from patency to closure. J Ultrasound Med 20:681–687
Mavrides E, Moscoso G, Carvalho JS et al (2002) The human ductus venosus between 13 and 17 weeks of gestation: histological and morphometric studies. Ultrasound Obstet Gynecol 19:39–46
Gorincour G, Droulle P, Guibaud L (2005) Prenatal diagnosis of umbilicoportosystemic shunts: report of 11 cases and review of the literature. AJR Am J Roentgenol 184:163–168
Bellotti M, Pennati G, De Gasperi C et al (2000) Role of ductus venosus in distribution of umbilical blood flow in human fetuses during second half of pregnancy. Am J Physiol Heart Circ Physiol 279:H1256–H1263
Uquillas KR, Grubbs BH, Prosper AE et al (2017) Doppler US in the evaluation of fetal growth and perinatal health. Radiographics 37:1831–1838
Chacko A, Kock C, Joshi JA et al (2016) Patent ductus venosus presenting with cholestatic jaundice in an infant with successful trans-catheter closure using a vascular plug device. Indian J Radiol Imaging 26:377–382
Billing JS, Jamieson NV (1997) Hepatic arterioportal fistula: a curable cause of portal hypertension in infancy. HPB Surg 10:311–314
Kumar N, de Goyet Jde V, Sharif K et al (2003) Congenital, solitary, large, intrahepatic arterioportal fistula in a child: management and review of the literature. Pediatr Radiol 33:20–23
Norton SP, Jacobson K, Moroz SP et al (2006) The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr 43:248–255
Vauthey JN, Tomczak RJ, Helmberger T et al (1997) The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy. Gastroenterology 113:1390–1401
Teplisky D, Tincani EU, Lipsich J, Sierre S (2012) Congenital arterioportal fistulas: radiological treatment and color Doppler US follow-up. Pediatr Radiol 42:1326–1332
Foley WJ, Turcotte JG, Hoskins PA et al (1971) Intrahepatic arteriovenous fistulas between the hepatic artery and portal vein. Ann Surg 174:849–855
Agha HM, Zakaria R, Mostafa FA, Hamza H (2015) Regression of a large congenital hepatic arteriovenous malformation. Tex Heart Inst J 42:184–187
Christison-Lagay ER, Burrows PE, Alomari A et al (2007) Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. J Pediatr Surg 42:62–67
Isaacs H Jr (2007) Fetal and neonatal hepatic tumors. J Pediatr Surg 42:1797–1803
Rialon KL, Murillo R, Fevurly RD et al (2015) Impact of screening for hepatic hemangiomas in patients with multiple cutaneous infantile hemangiomas. Pediatr Dermatol 32:808–812
Kassarjian A, Dubois J, Burrows PE (2002) Angiographic classification of hepatic hemangiomas in infants. Radiology 222:693–698
Kulungowski AM, Alomari AI, Chawla A et al (2012) Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg 47:165–170
Hughes JA, Hill V, Patel K et al (2004) Cutaneous haemangioma: prevalence and sonographic characteristics of associated hepatic haemangioma. Clin Radiol 59:273–280
Dickie B, Dasgupta R, Nair R et al (2009) Spectrum of hepatic hemangiomas: management and outcome. J Pediatr Surg 44:125–133
Metry DW, Hawrot A, Altman C, Frieden IJ (2004) Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Arch Dermatol 140:591–596
Makin E, Davenport M (2010) Fetal and neonatal liver tumours. Early Hum Dev 86:637–642
Anupindi SA, Biko DM, Ntoulia A et al (2017) Contrast-enhanced US assessment of focal liver lesions in children. Radiographics 37:1632–1647
Paltiel HJ, Patriquin HB, Keller MS et al (1992) Infantile hepatic hemangioma: Doppler US. Radiology 182:735–742
Peddu P, Huang D, Kane PA et al (2008) Vanishing liver tumours. Clin Radiol 63:329–339
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Chaturvedi, A., Klionsky, N.B. & Saul, D. Ultrasound with Doppler evaluation of congenital hepatic vascular shunts. Pediatr Radiol 48, 1658–1671 (2018). https://doi.org/10.1007/s00247-018-4247-0
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DOI: https://doi.org/10.1007/s00247-018-4247-0