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Published in: Pediatric Radiology 6/2010

01-06-2010 | Review

Paediatric chronic liver diseases: how to investigate and follow up? Role of imaging in the diagnosis of fibrosis

Authors: Danièle Pariente, Stéphanie Franchi-Abella

Published in: Pediatric Radiology | Issue 6/2010

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Abstract

Chronic liver diseases are rare in children, but encompass a wide spectrum of disorders that may all be complicated by liver fibrosis and therefore by portal hypertension. They may be classified according to the level of portal flow obstruction: prehepatic, intrahepatic or suprahepatic. Most of them, except presinusoidal diseases, may progress to cirrhosis that carries additional risks of impaired liver function and development of hepatocellular carcinoma. Imaging plays an important role in guiding the diagnosis and biopsy and for follow-up during treatment. US, with high-frequency transducers and Doppler, is the first modality of choice, directs the rest of the investigations and guides interventional radiology. MDCT has made great progress and has replaced angiography for diagnostic purposes. MRI is indicated for parenchyma and nodule characterization and for biliary tract evaluation. To avoid liver biopsy, several elasticity imaging techniques have been developed and have to be evaluated for accuracy and convenience in children. The role of each modality with main imaging findings is described in extrahepatic portal vein obstruction, hepatoportal sclerosis, congenital hepatic fibrosis, cirrhosis and Budd-Chiari syndrome.
Literature
1.
go back to reference Bernard O, Alvarez F, Brunelle F et al (1985) Portal hypertension in children. Clin Gastroenterol 14:33–55PubMed Bernard O, Alvarez F, Brunelle F et al (1985) Portal hypertension in children. Clin Gastroenterol 14:33–55PubMed
2.
go back to reference Leonis MA, Balistreri WF (2008) Evaluation and management of end-stage liver disease in children. Gastroenterology 134:1741–1751CrossRefPubMed Leonis MA, Balistreri WF (2008) Evaluation and management of end-stage liver disease in children. Gastroenterology 134:1741–1751CrossRefPubMed
4.
go back to reference Kruskal JB, Newman PA, Sammons LG et al (2004) Optimizing Doppler and color flow US: application to hepatic sonography. Radiographics 24:657–675CrossRefPubMed Kruskal JB, Newman PA, Sammons LG et al (2004) Optimizing Doppler and color flow US: application to hepatic sonography. Radiographics 24:657–675CrossRefPubMed
5.
go back to reference Patriquin H, Tessier G, Grignon A et al (1985) Lesser omental thickness in normal children: baseline for detection of portal hypertension. AJR 145:693–696PubMed Patriquin H, Tessier G, Grignon A et al (1985) Lesser omental thickness in normal children: baseline for detection of portal hypertension. AJR 145:693–696PubMed
6.
go back to reference Chan FP, Rubin GD (2005) MDCT angiography of pediatric vascular diseases of the abdomen, pelvis, and extremities. Pediatr Radiol 35:40–53CrossRefPubMed Chan FP, Rubin GD (2005) MDCT angiography of pediatric vascular diseases of the abdomen, pelvis, and extremities. Pediatr Radiol 35:40–53CrossRefPubMed
7.
go back to reference Mendichovszky IA, Marks SD, Simcock CM et al (2008) Gadolinium and nephrogenic systemic fibrosis: time to tighten practice. Pediatr Radiol 38:489–496, quiz 602–483CrossRefPubMed Mendichovszky IA, Marks SD, Simcock CM et al (2008) Gadolinium and nephrogenic systemic fibrosis: time to tighten practice. Pediatr Radiol 38:489–496, quiz 602–483CrossRefPubMed
8.
go back to reference Chavhan GB, Babyn PS, Manson D et al (2008) Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics 28:1951–1962CrossRefPubMed Chavhan GB, Babyn PS, Manson D et al (2008) Pediatric MR cholangiopancreatography: principles, technique, and clinical applications. Radiographics 28:1951–1962CrossRefPubMed
9.
go back to reference Chavhan GB, Babyn PS, Jankharia BG et al (2008) Steady-state MR imaging sequences: physics, classification, and clinical applications. Radiographics 28:1147–1160CrossRefPubMed Chavhan GB, Babyn PS, Jankharia BG et al (2008) Steady-state MR imaging sequences: physics, classification, and clinical applications. Radiographics 28:1147–1160CrossRefPubMed
10.
go back to reference Habdank K, Restrepo R, Ng V et al (2003) Combined sonographic and fluoroscopic guidance during transjugular hepatic biopsies performed in children: a retrospective study of 74 biopsies. AJR 180:1393–1398PubMed Habdank K, Restrepo R, Ng V et al (2003) Combined sonographic and fluoroscopic guidance during transjugular hepatic biopsies performed in children: a retrospective study of 74 biopsies. AJR 180:1393–1398PubMed
11.
12.
go back to reference Abd El-Hamid N, Taylor RM, Marinello D et al (2008) Aetiology and management of extrahepatic portal vein obstruction in children: King’s College Hospital experience. J Pediatr Gastroenterol Nutr 47:630–634CrossRefPubMed Abd El-Hamid N, Taylor RM, Marinello D et al (2008) Aetiology and management of extrahepatic portal vein obstruction in children: King’s College Hospital experience. J Pediatr Gastroenterol Nutr 47:630–634CrossRefPubMed
13.
go back to reference Gauthier-Villars M, Franchi S, Gauthier F et al (2005) Cholestasis in children with portal vein obstruction. J Pediatr 146:568–573CrossRefPubMed Gauthier-Villars M, Franchi S, Gauthier F et al (2005) Cholestasis in children with portal vein obstruction. J Pediatr 146:568–573CrossRefPubMed
14.
go back to reference Gauthier F (2005) Recent concepts regarding extra-hepatic portal hypertension. Semin Pediatr Surg 14:216–225CrossRefPubMed Gauthier F (2005) Recent concepts regarding extra-hepatic portal hypertension. Semin Pediatr Surg 14:216–225CrossRefPubMed
15.
go back to reference Guerin F, Porras J, Fabre M et al (2009) Liver nodules after portal systemic shunt surgery for extrahepatic portal vein obstruction in children. J Pediatr Surg 44:1337–1343CrossRefPubMed Guerin F, Porras J, Fabre M et al (2009) Liver nodules after portal systemic shunt surgery for extrahepatic portal vein obstruction in children. J Pediatr Surg 44:1337–1343CrossRefPubMed
16.
go back to reference Puppala S, Patel J, Woodley H et al (2009) Preoperative imaging of left portal vein at the Rex recess for Rex shunt formation using wedged hepatic vein carbon dioxide portography. J Pediatr Surg 44:2043–2047CrossRefPubMed Puppala S, Patel J, Woodley H et al (2009) Preoperative imaging of left portal vein at the Rex recess for Rex shunt formation using wedged hepatic vein carbon dioxide portography. J Pediatr Surg 44:2043–2047CrossRefPubMed
17.
go back to reference Sarin SK, Kumar A, Chawla YK et al (2007) Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment. Hepatol Int 1:398–413CrossRefPubMed Sarin SK, Kumar A, Chawla YK et al (2007) Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment. Hepatol Int 1:398–413CrossRefPubMed
18.
go back to reference Bernard PH, Le Bail B, Balabaud C et al (1996) Hepatoportal sclerosis, nodular regenerative hyperplasia, incomplete septal cirrhosis: lesions of hepatic microcirculation? Gastroenterol Clin Biol 20:335–338PubMed Bernard PH, Le Bail B, Balabaud C et al (1996) Hepatoportal sclerosis, nodular regenerative hyperplasia, incomplete septal cirrhosis: lesions of hepatic microcirculation? Gastroenterol Clin Biol 20:335–338PubMed
19.
go back to reference Girard M, Amiel J, Fabre M et al (2005) Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism? Am J Med Genet A 135:186–189PubMed Girard M, Amiel J, Fabre M et al (2005) Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism? Am J Med Genet A 135:186–189PubMed
20.
go back to reference Gurkaynak G, Yildirim B, Aksoy F et al (1998) Sonographic findings in noncirrhotic portal fibrosis. J Clin Ultrasound 26:309–313CrossRefPubMed Gurkaynak G, Yildirim B, Aksoy F et al (1998) Sonographic findings in noncirrhotic portal fibrosis. J Clin Ultrasound 26:309–313CrossRefPubMed
21.
go back to reference Turkbey B, Ocak I, Daryanani K et al (2009) Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol 39:100–111CrossRefPubMed Turkbey B, Ocak I, Daryanani K et al (2009) Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF). Pediatr Radiol 39:100–111CrossRefPubMed
22.
go back to reference Veigel MC, Prescott-Focht J, Rodriguez MG et al (2009) Fibropolycystic liver disease in children. Pediatr Radiol 39:317–327CrossRefPubMed Veigel MC, Prescott-Focht J, Rodriguez MG et al (2009) Fibropolycystic liver disease in children. Pediatr Radiol 39:317–327CrossRefPubMed
23.
go back to reference Brancatelli G, Federle MP, Vilgrain V et al (2005) Fibropolycystic liver disease: CT and MR imaging findings. Radiographics 25:659–670CrossRefPubMed Brancatelli G, Federle MP, Vilgrain V et al (2005) Fibropolycystic liver disease: CT and MR imaging findings. Radiographics 25:659–670CrossRefPubMed
24.
go back to reference Besnard M, Pariente D, Hadchouel M et al (1994) Portal cavernoma in congenital hepatic fibrosis. Angiographic reports of 10 pediatric cases. Pediatr Radiol 24:61–65CrossRefPubMed Besnard M, Pariente D, Hadchouel M et al (1994) Portal cavernoma in congenital hepatic fibrosis. Angiographic reports of 10 pediatric cases. Pediatr Radiol 24:61–65CrossRefPubMed
25.
go back to reference Tuney D, Aribal ME, Ertem D et al (1998) Diagnosis of liver cirrhosis in children based on colour Doppler ultrasonography with histopathological correlation. Pediatr Radiol 28:859–864CrossRefPubMed Tuney D, Aribal ME, Ertem D et al (1998) Diagnosis of liver cirrhosis in children based on colour Doppler ultrasonography with histopathological correlation. Pediatr Radiol 28:859–864CrossRefPubMed
26.
go back to reference Tsai A, Paltiel HJ, Sena LM et al (2009) Neonatal hemochromatosis and patent ductus venosus: clinical course and diagnostic pitfalls. Pediatr Radiol 39:823–827CrossRefPubMed Tsai A, Paltiel HJ, Sena LM et al (2009) Neonatal hemochromatosis and patent ductus venosus: clinical course and diagnostic pitfalls. Pediatr Radiol 39:823–827CrossRefPubMed
27.
go back to reference Shiha G, Sarin SK, Ibrahim AE et al (2009) Liver fibrosis: consensus recommendations of the Asian Pacific Association for the Study of the Liver (APASL). Hepatol Int 3:323–333CrossRefPubMed Shiha G, Sarin SK, Ibrahim AE et al (2009) Liver fibrosis: consensus recommendations of the Asian Pacific Association for the Study of the Liver (APASL). Hepatol Int 3:323–333CrossRefPubMed
28.
go back to reference Khong PL, Ooi CG, Saing H et al (2002) Portal venous velocity in the follow-up of patients with biliary atresia after Kasai portoenterostomy. J Pediatr Surg 37:873–876CrossRefPubMed Khong PL, Ooi CG, Saing H et al (2002) Portal venous velocity in the follow-up of patients with biliary atresia after Kasai portoenterostomy. J Pediatr Surg 37:873–876CrossRefPubMed
29.
go back to reference Kardorff R, Klotz M, Melter M et al (1999) Prediction of survival in extrahepatic biliary atresia by hepatic duplex sonography. J Pediatr Gastroenterol Nutr 28:411–417CrossRefPubMed Kardorff R, Klotz M, Melter M et al (1999) Prediction of survival in extrahepatic biliary atresia by hepatic duplex sonography. J Pediatr Gastroenterol Nutr 28:411–417CrossRefPubMed
30.
go back to reference Wildhaber BE, Rubbia-Brandt L, Majno P et al (2008) Focal ischemic necrosis in advanced biliary atresia cirrhosis. Pediatr Transplant 12:487–491CrossRefPubMed Wildhaber BE, Rubbia-Brandt L, Majno P et al (2008) Focal ischemic necrosis in advanced biliary atresia cirrhosis. Pediatr Transplant 12:487–491CrossRefPubMed
31.
go back to reference Caruso S, Miraglia R, Milazzo M et al (2009) Multidetector computed tomography hepatic findings in children with end-stage biliary atresia. Eur Radiol Dec 17 [Epub ahead of print] Caruso S, Miraglia R, Milazzo M et al (2009) Multidetector computed tomography hepatic findings in children with end-stage biliary atresia. Eur Radiol Dec 17 [Epub ahead of print]
32.
go back to reference Hanna RF, Aguirre DA, Kased N et al (2008) Cirrhosis-associated hepatocellular nodules: correlation of histopathologic and MR imaging features. Radiographics 28:747–769CrossRefPubMed Hanna RF, Aguirre DA, Kased N et al (2008) Cirrhosis-associated hepatocellular nodules: correlation of histopathologic and MR imaging features. Radiographics 28:747–769CrossRefPubMed
33.
go back to reference Hussain SM, Reinhold C, Mitchell DG (2009) Cirrhosis and lesion characterization at MR imaging. Radiographics 29:1637–1652CrossRefPubMed Hussain SM, Reinhold C, Mitchell DG (2009) Cirrhosis and lesion characterization at MR imaging. Radiographics 29:1637–1652CrossRefPubMed
34.
go back to reference Knisely AS, Strautnieks SS, Meier Y et al (2006) Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 44:478–486CrossRefPubMed Knisely AS, Strautnieks SS, Meier Y et al (2006) Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 44:478–486CrossRefPubMed
35.
go back to reference Brunati A, Feruzi Z, Sokal E et al (2007) Early occurrence of hepatocellular carcinoma in biliary atresia treated by liver transplantation. Pediatr Transplant 11:117–119CrossRefPubMed Brunati A, Feruzi Z, Sokal E et al (2007) Early occurrence of hepatocellular carcinoma in biliary atresia treated by liver transplantation. Pediatr Transplant 11:117–119CrossRefPubMed
36.
go back to reference Liang JL, Cheng YF, Concejero AM et al (2008) Macro-regenerative nodules in biliary atresia: CT/MRI findings and their pathological relations. World J Gastroenterol 14:4529–4534CrossRefPubMed Liang JL, Cheng YF, Concejero AM et al (2008) Macro-regenerative nodules in biliary atresia: CT/MRI findings and their pathological relations. World J Gastroenterol 14:4529–4534CrossRefPubMed
37.
go back to reference Hermeziu B, Franchi-Abella S, Plessier A et al (2008) Budd-Chiari syndrome and essential thrombocythemia in a child: favorable outcome after transjugular intrahepatic portosystemic shunt. J Pediatr Gastroenterol Nutr 46:334–337CrossRefPubMed Hermeziu B, Franchi-Abella S, Plessier A et al (2008) Budd-Chiari syndrome and essential thrombocythemia in a child: favorable outcome after transjugular intrahepatic portosystemic shunt. J Pediatr Gastroenterol Nutr 46:334–337CrossRefPubMed
38.
go back to reference Brancatelli G, Vilgrain V, Federle MP et al (2007) Budd-Chiari syndrome: spectrum of imaging findings. AJR 188:W168–176CrossRefPubMed Brancatelli G, Vilgrain V, Federle MP et al (2007) Budd-Chiari syndrome: spectrum of imaging findings. AJR 188:W168–176CrossRefPubMed
39.
go back to reference Cauchi JA, Oliff S, Baumann U et al (2006) The Budd-Chiari syndrome in children: the spectrum of management. J Pediatr Surg 41:1919–1923CrossRefPubMed Cauchi JA, Oliff S, Baumann U et al (2006) The Budd-Chiari syndrome in children: the spectrum of management. J Pediatr Surg 41:1919–1923CrossRefPubMed
40.
go back to reference Friedrich-Rust M, Ong MF, Herrmann E et al (2007) Real-time elastography for noninvasive assessment of liver fibrosis in chronic viral hepatitis. AJR 188:758–764CrossRefPubMed Friedrich-Rust M, Ong MF, Herrmann E et al (2007) Real-time elastography for noninvasive assessment of liver fibrosis in chronic viral hepatitis. AJR 188:758–764CrossRefPubMed
41.
go back to reference Faria SC, Ganesan K, Mwangi I et al (2009) MR imaging of liver fibrosis: current state of the art. Radiographics 29:1615–1635CrossRefPubMed Faria SC, Ganesan K, Mwangi I et al (2009) MR imaging of liver fibrosis: current state of the art. Radiographics 29:1615–1635CrossRefPubMed
42.
go back to reference Foucher J, Chanteloup E, Vergniol J et al (2006) Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study. Gut 55:403–408CrossRefPubMed Foucher J, Chanteloup E, Vergniol J et al (2006) Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study. Gut 55:403–408CrossRefPubMed
43.
go back to reference de Ledinghen V, Vergniol J (2008) Transient elastography (FibroScan). Gastroenterol Clin Biol 32:58–67CrossRefPubMed de Ledinghen V, Vergniol J (2008) Transient elastography (FibroScan). Gastroenterol Clin Biol 32:58–67CrossRefPubMed
44.
go back to reference de Ledinghen V, Le Bail B, Rebouissoux L et al (2007) Liver stiffness measurement in children using FibroScan: feasibility study and comparison with Fibrotest, aspartate transaminase to platelets ratio index, and liver biopsy. J Pediatr Gastroenterol Nutr 45:443–450CrossRefPubMed de Ledinghen V, Le Bail B, Rebouissoux L et al (2007) Liver stiffness measurement in children using FibroScan: feasibility study and comparison with Fibrotest, aspartate transaminase to platelets ratio index, and liver biopsy. J Pediatr Gastroenterol Nutr 45:443–450CrossRefPubMed
45.
go back to reference Friedrich-Rust M, Wunder K, Kriener S et al (2009) Liver fibrosis in viral hepatitis: noninvasive assessment with acoustic radiation force impulse imaging versus transient elastography. Radiology 252:595–604CrossRefPubMed Friedrich-Rust M, Wunder K, Kriener S et al (2009) Liver fibrosis in viral hepatitis: noninvasive assessment with acoustic radiation force impulse imaging versus transient elastography. Radiology 252:595–604CrossRefPubMed
46.
go back to reference Muller M, Gennisson JL, Deffieux T et al (2009) Quantitative viscoelasticity mapping of human liver using supersonic shear imaging: preliminary in vivo feasibility study. Ultrasound Med Biol 35:219–229CrossRefPubMed Muller M, Gennisson JL, Deffieux T et al (2009) Quantitative viscoelasticity mapping of human liver using supersonic shear imaging: preliminary in vivo feasibility study. Ultrasound Med Biol 35:219–229CrossRefPubMed
47.
go back to reference Razek AA, Abdalla A, Omran E et al (2009) Diagnosis and quantification of hepatic fibrosis in children with diffusion weighted MR imaging. Eur J Radiol Nov 17 [Epub ahead of print] Razek AA, Abdalla A, Omran E et al (2009) Diagnosis and quantification of hepatic fibrosis in children with diffusion weighted MR imaging. Eur J Radiol Nov 17 [Epub ahead of print]
48.
go back to reference Sandrasegaran K, Akisik FM, Lin C et al (2009) Value of diffusion-weighted MRI for assessing liver fibrosis and cirrhosis. AJR 193:1556–1560CrossRefPubMed Sandrasegaran K, Akisik FM, Lin C et al (2009) Value of diffusion-weighted MRI for assessing liver fibrosis and cirrhosis. AJR 193:1556–1560CrossRefPubMed
Metadata
Title
Paediatric chronic liver diseases: how to investigate and follow up? Role of imaging in the diagnosis of fibrosis
Authors
Danièle Pariente
Stéphanie Franchi-Abella
Publication date
01-06-2010
Publisher
Springer-Verlag
Published in
Pediatric Radiology / Issue 6/2010
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-010-1600-3

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