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Ultrasound with Doppler evaluation of congenital hepatic vascular shunts

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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

  1. 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

    Article  CAS  PubMed  Google Scholar 

  2. 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

    Article  PubMed  Google Scholar 

  3. 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

    Article  CAS  PubMed  Google Scholar 

  4. Collardeau-Frachon S, Scoazec JY (2008) Vascular development and differentiation during human liver organogenesis. Anat Rec 291:614–627

    Article  Google Scholar 

  5. 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

    PubMed  Google Scholar 

  6. 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

    PubMed  PubMed Central  Google Scholar 

  7. White JJ, Brenner H, Avery ME (1969) Umbilical vein collateral circulation: the caput medusae in a newborn infant. Pediatrics 43:391–395

    CAS  PubMed  Google Scholar 

  8. 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

    Article  CAS  PubMed  Google Scholar 

  9. 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

    Article  PubMed  PubMed Central  Google Scholar 

  10. 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

    Article  PubMed  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. Kwapisz L, Wells MM, AlJudaibi B (2014) Abernethy malformation: Congenital absence of the portal vein. Can J Gastroenterol Hepatol 28:587–588

    Article  PubMed  PubMed Central  Google Scholar 

  13. 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

    Article  PubMed  Google Scholar 

  14. Park JH, Cha SH, Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 155:527–528

    Article  CAS  PubMed  Google Scholar 

  15. Wu L, Zhao L, Lu Y et al (2016) Interventional embolization of congenital intrahepatic shunts in children. Pediatr Radiol 46:541–547

    Article  PubMed  Google Scholar 

  16. Gallego C, Miralles M, Marin C et al (2004) Congenital hepatic shunts. Radiographics 24:755–772

    Article  PubMed  Google Scholar 

  17. Florio F, Nardella M, Balzano S et al (1998) Congenital intrahepatic portosystemic shunt. Cardiovasc Intervent Radiol 21:421–424

    Article  CAS  PubMed  Google Scholar 

  18. 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

    Article  CAS  PubMed  Google Scholar 

  19. 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

    Article  CAS  PubMed  Google Scholar 

  20. 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

    Article  CAS  PubMed  Google Scholar 

  21. 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

    Article  PubMed  Google Scholar 

  22. 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

    Article  CAS  PubMed  Google Scholar 

  23. Uquillas KR, Grubbs BH, Prosper AE et al (2017) Doppler US in the evaluation of fetal growth and perinatal health. Radiographics 37:1831–1838

    Article  PubMed  Google Scholar 

  24. 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

    Article  PubMed  PubMed Central  Google Scholar 

  25. Billing JS, Jamieson NV (1997) Hepatic arterioportal fistula: a curable cause of portal hypertension in infancy. HPB Surg 10:311–314

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. 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

    Article  PubMed  Google Scholar 

  27. 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

    Article  PubMed  Google Scholar 

  28. Vauthey JN, Tomczak RJ, Helmberger T et al (1997) The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy. Gastroenterology 113:1390–1401

    Article  CAS  PubMed  Google Scholar 

  29. 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

    Article  PubMed  Google Scholar 

  30. Foley WJ, Turcotte JG, Hoskins PA et al (1971) Intrahepatic arteriovenous fistulas between the hepatic artery and portal vein. Ann Surg 174:849–855

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  31. Agha HM, Zakaria R, Mostafa FA, Hamza H (2015) Regression of a large congenital hepatic arteriovenous malformation. Tex Heart Inst J 42:184–187

    Article  PubMed  PubMed Central  Google Scholar 

  32. 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

    Article  PubMed  Google Scholar 

  33. Isaacs H Jr (2007) Fetal and neonatal hepatic tumors. J Pediatr Surg 42:1797–1803

    Article  PubMed  Google Scholar 

  34. 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

    Article  PubMed  Google Scholar 

  35. Kassarjian A, Dubois J, Burrows PE (2002) Angiographic classification of hepatic hemangiomas in infants. Radiology 222:693–698

    Article  PubMed  Google Scholar 

  36. Kulungowski AM, Alomari AI, Chawla A et al (2012) Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg 47:165–170

    Article  PubMed  Google Scholar 

  37. Hughes JA, Hill V, Patel K et al (2004) Cutaneous haemangioma: prevalence and sonographic characteristics of associated hepatic haemangioma. Clin Radiol 59:273–280

    Article  CAS  PubMed  Google Scholar 

  38. Dickie B, Dasgupta R, Nair R et al (2009) Spectrum of hepatic hemangiomas: management and outcome. J Pediatr Surg 44:125–133

    Article  PubMed  Google Scholar 

  39. 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

    Article  PubMed  Google Scholar 

  40. Makin E, Davenport M (2010) Fetal and neonatal liver tumours. Early Hum Dev 86:637–642

    Article  PubMed  Google Scholar 

  41. Anupindi SA, Biko DM, Ntoulia A et al (2017) Contrast-enhanced US assessment of focal liver lesions in children. Radiographics 37:1632–1647

    Article  PubMed  Google Scholar 

  42. Paltiel HJ, Patriquin HB, Keller MS et al (1992) Infantile hepatic hemangioma: Doppler US. Radiology 182:735–742

    Article  CAS  PubMed  Google Scholar 

  43. Peddu P, Huang D, Kane PA et al (2008) Vanishing liver tumours. Clin Radiol 63:329–339

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Apeksha Chaturvedi.

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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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