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Published in: Pediatric Radiology 11/2018

01-10-2018 | Pictorial Essay

Ultrasound with Doppler evaluation of congenital hepatic vascular shunts

Authors: Apeksha Chaturvedi, Nina B. Klionsky, David Saul

Published in: Pediatric Radiology | Issue 11/2018

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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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Literature
1.
go back to reference Bernard O, Franchi-Abella S, Branchereau S et al (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32:273–287CrossRefPubMed Bernard O, Franchi-Abella S, Branchereau S et al (2012) Congenital portosystemic shunts in children: recognition, evaluation, and management. Semin Liver Dis 32:273–287CrossRefPubMed
2.
go back to reference Back SJ, Maya CL, Khwaja A (2017) Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen. Pediatr Radiol 47:1069–1078CrossRefPubMed Back SJ, Maya CL, Khwaja A (2017) Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen. Pediatr Radiol 47:1069–1078CrossRefPubMed
3.
go back to reference Achiron R, Gindes L, Kivilevitch Z et al (2009) Prenatal diagnosis of congenital agenesis of the fetal portal venous system. Ultrasound Obstet Gynecol 34:643–652CrossRefPubMed Achiron R, Gindes L, Kivilevitch Z et al (2009) Prenatal diagnosis of congenital agenesis of the fetal portal venous system. Ultrasound Obstet Gynecol 34:643–652CrossRefPubMed
4.
go back to reference Collardeau-Frachon S, Scoazec JY (2008) Vascular development and differentiation during human liver organogenesis. Anat Rec 291:614–627CrossRef Collardeau-Frachon S, Scoazec JY (2008) Vascular development and differentiation during human liver organogenesis. Anat Rec 291:614–627CrossRef
5.
go back to reference 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 51:322–330PubMed 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 51:322–330PubMed
6.
go back to reference 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 7:100–108PubMedPubMedCentral 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 7:100–108PubMedPubMedCentral
7.
go back to reference White JJ, Brenner H, Avery ME (1969) Umbilical vein collateral circulation: the caput medusae in a newborn infant. Pediatrics 43:391–395PubMed White JJ, Brenner H, Avery ME (1969) Umbilical vein collateral circulation: the caput medusae in a newborn infant. Pediatrics 43:391–395PubMed
8.
go back to reference 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 29:1239–1241CrossRefPubMed 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 29:1239–1241CrossRefPubMed
9.
go back to reference 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 15:37CrossRefPubMedPubMedCentral 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 15:37CrossRefPubMedPubMedCentral
10.
go back to reference Alonso-Gamarra E, Parron M, Perez A et al (2011) Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics 31:707–722CrossRefPubMed Alonso-Gamarra E, Parron M, Perez A et al (2011) Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review. Radiographics 31:707–722CrossRefPubMed
11.
go back to reference Loomba RS, Telega GW, Gudausky TM (2012) Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula. J Pediatr Surg 47:E25–E31CrossRefPubMed Loomba RS, Telega GW, Gudausky TM (2012) Type 2 Abernethy malformation presenting as a portal vein-coronary sinus fistula. J Pediatr Surg 47:E25–E31CrossRefPubMed
12.
13.
go back to reference 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 260:188–198CrossRefPubMed 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 260:188–198CrossRefPubMed
14.
go back to reference Park JH, Cha SH, Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 155:527–528CrossRefPubMed Park JH, Cha SH, Han JK, Han MC (1990) Intrahepatic portosystemic venous shunt. AJR Am J Roentgenol 155:527–528CrossRefPubMed
15.
go back to reference Wu L, Zhao L, Lu Y et al (2016) Interventional embolization of congenital intrahepatic shunts in children. Pediatr Radiol 46:541–547CrossRefPubMed Wu L, Zhao L, Lu Y et al (2016) Interventional embolization of congenital intrahepatic shunts in children. Pediatr Radiol 46:541–547CrossRefPubMed
16.
17.
go back to reference Florio F, Nardella M, Balzano S et al (1998) Congenital intrahepatic portosystemic shunt. Cardiovasc Intervent Radiol 21:421–424CrossRefPubMed Florio F, Nardella M, Balzano S et al (1998) Congenital intrahepatic portosystemic shunt. Cardiovasc Intervent Radiol 21:421–424CrossRefPubMed
18.
go back to reference 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 172:227–229CrossRefPubMed 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 172:227–229CrossRefPubMed
19.
go back to reference Vade A, Lim-Dunham J, Iqbal N (2001) Imaging of the ductus venosus in neonates: from patency to closure. J Ultrasound Med 20:681–687CrossRefPubMed Vade A, Lim-Dunham J, Iqbal N (2001) Imaging of the ductus venosus in neonates: from patency to closure. J Ultrasound Med 20:681–687CrossRefPubMed
20.
go back to reference 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 19:39–46CrossRefPubMed 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 19:39–46CrossRefPubMed
21.
go back to reference 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 184:163–168CrossRefPubMed 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 184:163–168CrossRefPubMed
22.
go back to reference 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–H1263CrossRefPubMed 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–H1263CrossRefPubMed
23.
go back to reference Uquillas KR, Grubbs BH, Prosper AE et al (2017) Doppler US in the evaluation of fetal growth and perinatal health. Radiographics 37:1831–1838CrossRefPubMed Uquillas KR, Grubbs BH, Prosper AE et al (2017) Doppler US in the evaluation of fetal growth and perinatal health. Radiographics 37:1831–1838CrossRefPubMed
24.
go back to reference 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 26:377–382CrossRefPubMedPubMedCentral 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 26:377–382CrossRefPubMedPubMedCentral
26.
go back to reference 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 33:20–23CrossRefPubMed 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 33:20–23CrossRefPubMed
27.
go back to reference Norton SP, Jacobson K, Moroz SP et al (2006) The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr 43:248–255CrossRefPubMed Norton SP, Jacobson K, Moroz SP et al (2006) The congenital intrahepatic arterioportal fistula syndrome: elucidation and proposed classification. J Pediatr Gastroenterol Nutr 43:248–255CrossRefPubMed
28.
go back to reference Vauthey JN, Tomczak RJ, Helmberger T et al (1997) The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy. Gastroenterology 113:1390–1401CrossRefPubMed Vauthey JN, Tomczak RJ, Helmberger T et al (1997) The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy. Gastroenterology 113:1390–1401CrossRefPubMed
29.
go back to reference Teplisky D, Tincani EU, Lipsich J, Sierre S (2012) Congenital arterioportal fistulas: radiological treatment and color Doppler US follow-up. Pediatr Radiol 42:1326–1332CrossRefPubMed Teplisky D, Tincani EU, Lipsich J, Sierre S (2012) Congenital arterioportal fistulas: radiological treatment and color Doppler US follow-up. Pediatr Radiol 42:1326–1332CrossRefPubMed
30.
31.
32.
go back to reference 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 42:62–67CrossRefPubMed 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 42:62–67CrossRefPubMed
34.
go back to reference Rialon KL, Murillo R, Fevurly RD et al (2015) Impact of screening for hepatic hemangiomas in patients with multiple cutaneous infantile hemangiomas. Pediatr Dermatol 32:808–812CrossRefPubMed Rialon KL, Murillo R, Fevurly RD et al (2015) Impact of screening for hepatic hemangiomas in patients with multiple cutaneous infantile hemangiomas. Pediatr Dermatol 32:808–812CrossRefPubMed
35.
go back to reference Kassarjian A, Dubois J, Burrows PE (2002) Angiographic classification of hepatic hemangiomas in infants. Radiology 222:693–698CrossRefPubMed Kassarjian A, Dubois J, Burrows PE (2002) Angiographic classification of hepatic hemangiomas in infants. Radiology 222:693–698CrossRefPubMed
36.
go back to reference Kulungowski AM, Alomari AI, Chawla A et al (2012) Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg 47:165–170CrossRefPubMed Kulungowski AM, Alomari AI, Chawla A et al (2012) Lessons from a liver hemangioma registry: subtype classification. J Pediatr Surg 47:165–170CrossRefPubMed
37.
go back to reference Hughes JA, Hill V, Patel K et al (2004) Cutaneous haemangioma: prevalence and sonographic characteristics of associated hepatic haemangioma. Clin Radiol 59:273–280CrossRefPubMed Hughes JA, Hill V, Patel K et al (2004) Cutaneous haemangioma: prevalence and sonographic characteristics of associated hepatic haemangioma. Clin Radiol 59:273–280CrossRefPubMed
38.
go back to reference Dickie B, Dasgupta R, Nair R et al (2009) Spectrum of hepatic hemangiomas: management and outcome. J Pediatr Surg 44:125–133CrossRefPubMed Dickie B, Dasgupta R, Nair R et al (2009) Spectrum of hepatic hemangiomas: management and outcome. J Pediatr Surg 44:125–133CrossRefPubMed
39.
go back to reference Metry DW, Hawrot A, Altman C, Frieden IJ (2004) Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Arch Dermatol 140:591–596CrossRefPubMed Metry DW, Hawrot A, Altman C, Frieden IJ (2004) Association of solitary, segmental hemangiomas of the skin with visceral hemangiomatosis. Arch Dermatol 140:591–596CrossRefPubMed
40.
41.
go back to reference Anupindi SA, Biko DM, Ntoulia A et al (2017) Contrast-enhanced US assessment of focal liver lesions in children. Radiographics 37:1632–1647CrossRefPubMed Anupindi SA, Biko DM, Ntoulia A et al (2017) Contrast-enhanced US assessment of focal liver lesions in children. Radiographics 37:1632–1647CrossRefPubMed
42.
go back to reference Paltiel HJ, Patriquin HB, Keller MS et al (1992) Infantile hepatic hemangioma: Doppler US. Radiology 182:735–742CrossRefPubMed Paltiel HJ, Patriquin HB, Keller MS et al (1992) Infantile hepatic hemangioma: Doppler US. Radiology 182:735–742CrossRefPubMed
43.
Metadata
Title
Ultrasound with Doppler evaluation of congenital hepatic vascular shunts
Authors
Apeksha Chaturvedi
Nina B. Klionsky
David Saul
Publication date
01-10-2018
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Radiology / Issue 11/2018
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-018-4247-0

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