Skip to main content
Top
Published in: Pediatric Radiology 9/2017

01-08-2017 | Pediatric Ultrasound

Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen

Authors: Susan J. Back, Carolina L. Maya, Asef Khwaja

Published in: Pediatric Radiology | Issue 9/2017

Login to get access

Abstract

Ultrasound is often the initial imaging examination performed of the solid organs of the pediatric abdomen. The sonographic appearance of the hepatobiliary system, pancreas and spleen changes with growth and development. This article reviews the normal US appearance of these organs in children and illustrates, through case examples, congenital and inherited conditions that affect them.
Literature
1.
go back to reference Rozel C, Garel L, Rypens F et al (2011) Imaging of biliary disorders in children. Pediatr Radiol 41:208–220CrossRefPubMed Rozel C, Garel L, Rypens F et al (2011) Imaging of biliary disorders in children. Pediatr Radiol 41:208–220CrossRefPubMed
2.
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
3.
go back to reference Yoneyama H, Kondo C, Yamasaki A et al (2015) Comparison of situs ambiguous patterns between heterotaxy syndromes with polysplenia and asplenia. Eur J Radiol 84:2301–2306CrossRefPubMed Yoneyama H, Kondo C, Yamasaki A et al (2015) Comparison of situs ambiguous patterns between heterotaxy syndromes with polysplenia and asplenia. Eur J Radiol 84:2301–2306CrossRefPubMed
4.
go back to reference American Institute of Ultrasound in Medicine (2008) AIUM practice guideline for the performance of an ultrasound examination of the abdomen and/or retroperitoneum. J Ultrasound Med 27:319–326CrossRef American Institute of Ultrasound in Medicine (2008) AIUM practice guideline for the performance of an ultrasound examination of the abdomen and/or retroperitoneum. J Ultrasound Med 27:319–326CrossRef
5.
go back to reference Holder LE, Strife J, Padikal TN et al (1975) Liver size determination in pediatrics using sonographic and scintigraphic techniques. Radiology 117:349–353CrossRefPubMed Holder LE, Strife J, Padikal TN et al (1975) Liver size determination in pediatrics using sonographic and scintigraphic techniques. Radiology 117:349–353CrossRefPubMed
6.
go back to reference Konus OL, Ozdemir A, Akkaya A et al (1998) Normal liver, spleen, and kidney dimensions in neonates, infants, and children: evaluation with sonography. AJR Am J Roentgenol 171:1693–1698CrossRefPubMed Konus OL, Ozdemir A, Akkaya A et al (1998) Normal liver, spleen, and kidney dimensions in neonates, infants, and children: evaluation with sonography. AJR Am J Roentgenol 171:1693–1698CrossRefPubMed
7.
go back to reference Dittrich M, Milde S, Dinkel E et al (1983) Sonographic biometry of liver and spleen size in childhood. Pediatr Radiol 13:206–211CrossRefPubMed Dittrich M, Milde S, Dinkel E et al (1983) Sonographic biometry of liver and spleen size in childhood. Pediatr Radiol 13:206–211CrossRefPubMed
8.
go back to reference Soyupak SK, Narli N, Yapicioglu H et al (2002) Sonographic measurements of the liver, spleen and kidney dimensions in the healthy term and preterm newborns. Eur J Radiol 43:73–78CrossRefPubMed Soyupak SK, Narli N, Yapicioglu H et al (2002) Sonographic measurements of the liver, spleen and kidney dimensions in the healthy term and preterm newborns. Eur J Radiol 43:73–78CrossRefPubMed
9.
go back to reference Kahramaner Z, Erdemir A, Cicek E et al (2013) Reference ranges for sonographic dimensions of the liver and spleen in preterm infants. Pediatr Radiol 43:1464–1474CrossRefPubMed Kahramaner Z, Erdemir A, Cicek E et al (2013) Reference ranges for sonographic dimensions of the liver and spleen in preterm infants. Pediatr Radiol 43:1464–1474CrossRefPubMed
10.
go back to reference McGahan JP, Phillips HE, Cox KL (1982) Sonography of the normal pediatric gallbladder and biliary tract. Radiology 144:873–875CrossRefPubMed McGahan JP, Phillips HE, Cox KL (1982) Sonography of the normal pediatric gallbladder and biliary tract. Radiology 144:873–875CrossRefPubMed
11.
go back to reference Chafetz N, Filly RA (1979) Portal and hepatic veins: accuracy of margin echoes for distinguishing intrahepatic vessels. Radiology 130:725–728CrossRefPubMed Chafetz N, Filly RA (1979) Portal and hepatic veins: accuracy of margin echoes for distinguishing intrahepatic vessels. Radiology 130:725–728CrossRefPubMed
12.
go back to reference Shorbagi A, Bayraktar Y (2010) Experience of a single center with congenital hepatic fibrosis: a review of the literature. World J Gastroenterol 16:683–690CrossRefPubMedPubMedCentral Shorbagi A, Bayraktar Y (2010) Experience of a single center with congenital hepatic fibrosis: a review of the literature. World J Gastroenterol 16:683–690CrossRefPubMedPubMedCentral
13.
go back to reference Hanquinet S, Rougemont AL, Courvoisier D et al (2013) Acoustic radiation force impulse (ARFI) elastography for the noninvasive diagnosis of liver fibrosis in children. Pediatr Radiol 43:545–551CrossRefPubMed Hanquinet S, Rougemont AL, Courvoisier D et al (2013) Acoustic radiation force impulse (ARFI) elastography for the noninvasive diagnosis of liver fibrosis in children. Pediatr Radiol 43:545–551CrossRefPubMed
14.
15.
go back to reference Mittal V, Saxena AK, Sodhi KS et al (2011) Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days. AJR Am J Roentgenol 196:W438–W445CrossRefPubMed Mittal V, Saxena AK, Sodhi KS et al (2011) Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days. AJR Am J Roentgenol 196:W438–W445CrossRefPubMed
16.
go back to reference Zhou L, Shan Q, Tian W et al (2016) Ultrasound for the diagnosis of biliary atresia: a meta-analysis. AJR Am J Roentgenol 206:W73–W82CrossRefPubMed Zhou L, Shan Q, Tian W et al (2016) Ultrasound for the diagnosis of biliary atresia: a meta-analysis. AJR Am J Roentgenol 206:W73–W82CrossRefPubMed
17.
go back to reference Leschied JR, Dillman JR, Bilhartz J et al (2015) Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases. Pediatr Radiol 45:366–375CrossRefPubMed Leschied JR, Dillman JR, Bilhartz J et al (2015) Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases. Pediatr Radiol 45:366–375CrossRefPubMed
18.
go back to reference Wang X, Qian L, Jia L et al (2016) Utility of shear wave elastography for differentiating biliary atresia from infantile hepatitis syndrome. J Ultrasound Med 35:1475–1479CrossRefPubMed Wang X, Qian L, Jia L et al (2016) Utility of shear wave elastography for differentiating biliary atresia from infantile hepatitis syndrome. J Ultrasound Med 35:1475–1479CrossRefPubMed
19.
go back to reference Hanquinet S, Courvoisier DS, Rougemont AL et al (2015) Contribution of acoustic radiation force impulse (ARFI) elastography to the ultrasound diagnosis of biliary atresia. Pediatr Radiol 45:1489–1495CrossRefPubMed Hanquinet S, Courvoisier DS, Rougemont AL et al (2015) Contribution of acoustic radiation force impulse (ARFI) elastography to the ultrasound diagnosis of biliary atresia. Pediatr Radiol 45:1489–1495CrossRefPubMed
20.
go back to reference Subramaniam P, Knisely A, Portmann B et al (2011) Diagnosis of Alagille syndrome — 25 years of experience at King's College Hospital. J Pediatr Gastroenterol Nutr 52:84–89CrossRefPubMed Subramaniam P, Knisely A, Portmann B et al (2011) Diagnosis of Alagille syndrome — 25 years of experience at King's College Hospital. J Pediatr Gastroenterol Nutr 52:84–89CrossRefPubMed
21.
go back to reference Debray D, Kelly D, Houwen R et al (2011) Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros 10:S29–S36CrossRefPubMed Debray D, Kelly D, Houwen R et al (2011) Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros 10:S29–S36CrossRefPubMed
22.
go back to reference Bodewes FA, van der Doef HP, Houwen RH et al (2015) Increase of serum gamma-glutamyltransferase associated with development of cirrhotic cystic fibrosis liver disease. J Pediatr Gastroenterol Nutr 61:113–118PubMed Bodewes FA, van der Doef HP, Houwen RH et al (2015) Increase of serum gamma-glutamyltransferase associated with development of cirrhotic cystic fibrosis liver disease. J Pediatr Gastroenterol Nutr 61:113–118PubMed
23.
go back to reference Friedrich-Rust M, Schlueter N, Smaczny C et al (2013) Non-invasive measurement of liver and pancreas fibrosis in patients with cystic fibrosis. J Cyst Fibros 12:431–439CrossRefPubMed Friedrich-Rust M, Schlueter N, Smaczny C et al (2013) Non-invasive measurement of liver and pancreas fibrosis in patients with cystic fibrosis. J Cyst Fibros 12:431–439CrossRefPubMed
24.
go back to reference Mortele KJ, Rocha TC, Streeter JL et al (2006) Multimodality imaging of pancreatic and biliary congenital anomalies. Radiographics 26:715–731CrossRefPubMed Mortele KJ, Rocha TC, Streeter JL et al (2006) Multimodality imaging of pancreatic and biliary congenital anomalies. Radiographics 26:715–731CrossRefPubMed
25.
go back to reference Borghei P, Sokhandon F, Shirkhoda A et al (2013) Anomalies, anatomic variants, and sources of diagnostic pitfalls in pancreatic imaging. Radiology 266:28–36CrossRefPubMed Borghei P, Sokhandon F, Shirkhoda A et al (2013) Anomalies, anatomic variants, and sources of diagnostic pitfalls in pancreatic imaging. Radiology 266:28–36CrossRefPubMed
26.
go back to reference Walsh E, Cramer B, Pushpanathan C (1990) Pancreatic echogenicity in premature and newborn infants. Pediatr Radiol 20:323–325CrossRefPubMed Walsh E, Cramer B, Pushpanathan C (1990) Pancreatic echogenicity in premature and newborn infants. Pediatr Radiol 20:323–325CrossRefPubMed
27.
go back to reference Siegel MJ, Martin KW, Worthington JL (1987) Normal and abnormal pancreas in children: US studies. Radiology 165:15–18CrossRefPubMed Siegel MJ, Martin KW, Worthington JL (1987) Normal and abnormal pancreas in children: US studies. Radiology 165:15–18CrossRefPubMed
28.
go back to reference Chao HC, Lin SJ, Kong MS et al (2000) Sonographic evaluation of the pancreatic duct in normal children and children with pancreatitis. J Ultrasound Med 19:757–763CrossRefPubMed Chao HC, Lin SJ, Kong MS et al (2000) Sonographic evaluation of the pancreatic duct in normal children and children with pancreatitis. J Ultrasound Med 19:757–763CrossRefPubMed
29.
go back to reference Yu J, Turner MA, Fulcher AS et al (2006) Congenital anomalies and normal variants of the pancreaticobiliary tract and the pancreas in adults: part 2, pancreatic duct and pancreas. AJR Am J Roentgenol 187:1544–1553CrossRefPubMed Yu J, Turner MA, Fulcher AS et al (2006) Congenital anomalies and normal variants of the pancreaticobiliary tract and the pancreas in adults: part 2, pancreatic duct and pancreas. AJR Am J Roentgenol 187:1544–1553CrossRefPubMed
30.
go back to reference Jimenez JC, Emil S, Podnos Y et al (2004) Annular pancreas in children: a recent decade's experience. J Pediatr Surg 39:1654–1657CrossRefPubMed Jimenez JC, Emil S, Podnos Y et al (2004) Annular pancreas in children: a recent decade's experience. J Pediatr Surg 39:1654–1657CrossRefPubMed
31.
go back to reference Johnston DW (1978) Annular pancreas: a new classification and clinical observations. Can J Surg 21:241–244PubMed Johnston DW (1978) Annular pancreas: a new classification and clinical observations. Can J Surg 21:241–244PubMed
32.
go back to reference Vijayaraghavan SB (2002) Sonography of pancreatic ductal anatomic characteristics in annular pancreas. J Ultrasound Med 21:1315–1318CrossRefPubMed Vijayaraghavan SB (2002) Sonography of pancreatic ductal anatomic characteristics in annular pancreas. J Ultrasound Med 21:1315–1318CrossRefPubMed
34.
go back to reference Nijs E, Callahan MJ, Taylor GA (2005) Disorders of the pediatric pancreas: imaging features. Pediatr Radiol 35:358–373CrossRefPubMed Nijs E, Callahan MJ, Taylor GA (2005) Disorders of the pediatric pancreas: imaging features. Pediatr Radiol 35:358–373CrossRefPubMed
35.
go back to reference Thoeni RF, Gedgaudas RK (1980) Ectopic pancreas: usual and unusual features. Gastrointest Radiol 5:37–42CrossRefPubMed Thoeni RF, Gedgaudas RK (1980) Ectopic pancreas: usual and unusual features. Gastrointest Radiol 5:37–42CrossRefPubMed
36.
go back to reference Prasad TR, Gupta SD, Bhatnagar V (2001) Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia. Pediatr Surg Int 17:552–554CrossRefPubMed Prasad TR, Gupta SD, Bhatnagar V (2001) Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia. Pediatr Surg Int 17:552–554CrossRefPubMed
37.
go back to reference Hough DM, Stephens DH, Johnson CD et al (1994) Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical significance, and CT findings. AJR Am J Roentgenol 162:1091–1094CrossRefPubMed Hough DM, Stephens DH, Johnson CD et al (1994) Pancreatic lesions in von Hippel-Lindau disease: prevalence, clinical significance, and CT findings. AJR Am J Roentgenol 162:1091–1094CrossRefPubMed
38.
go back to reference von Rohden L, Mohnike K, Mau H et al (2011) Intraoperative sonography: a technique for localizing focal forms of congenital hyperinsulinism in the pancreas. Ultraschall Med 32:74–80CrossRef von Rohden L, Mohnike K, Mau H et al (2011) Intraoperative sonography: a technique for localizing focal forms of congenital hyperinsulinism in the pancreas. Ultraschall Med 32:74–80CrossRef
39.
go back to reference Agrons GA, Corse WR, Markowitz RI et al (1996) Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. Radiographics 16:871–893CrossRefPubMed Agrons GA, Corse WR, Markowitz RI et al (1996) Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. Radiographics 16:871–893CrossRefPubMed
40.
go back to reference Swobodnik W, Wolf A, Wechsler JG et al (1985) Ultrasound characteristics of the pancreas in children with cystic fibrosis. J Clin Ultrasound 13:469–474CrossRefPubMed Swobodnik W, Wolf A, Wechsler JG et al (1985) Ultrasound characteristics of the pancreas in children with cystic fibrosis. J Clin Ultrasound 13:469–474CrossRefPubMed
41.
go back to reference Donnelly LF, Emery KH, Bove KE et al (1996) Normal changes in the MR appearance of the spleen during early childhood. AJR Am J Roentgenol 166:635–639CrossRefPubMed Donnelly LF, Emery KH, Bove KE et al (1996) Normal changes in the MR appearance of the spleen during early childhood. AJR Am J Roentgenol 166:635–639CrossRefPubMed
43.
go back to reference Rosenberg HK, Markowitz RI, Kolberg H et al (1991) Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol 157:119–121CrossRefPubMed Rosenberg HK, Markowitz RI, Kolberg H et al (1991) Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol 157:119–121CrossRefPubMed
44.
go back to reference Megremis SD, Vlachonikolis IG, Tsilimigaki AM (2004) Spleen length in childhood with US: normal values based on age, sex, and somatometric parameters. Radiology 231:129–134CrossRefPubMed Megremis SD, Vlachonikolis IG, Tsilimigaki AM (2004) Spleen length in childhood with US: normal values based on age, sex, and somatometric parameters. Radiology 231:129–134CrossRefPubMed
45.
go back to reference Nemati M, Hajalioghli P, Jahed S et al (2016) Normal values of spleen length and volume: an ultrasonographic study in children. Ultrasound Med Biol 42:1771–1778CrossRefPubMed Nemati M, Hajalioghli P, Jahed S et al (2016) Normal values of spleen length and volume: an ultrasonographic study in children. Ultrasound Med Biol 42:1771–1778CrossRefPubMed
46.
go back to reference Loftus WK, Metreweli C (1998) Ultrasound assessment of mild splenomegaly: spleen/kidney ratio. Pediatr Radiol 28:98–100CrossRefPubMed Loftus WK, Metreweli C (1998) Ultrasound assessment of mild splenomegaly: spleen/kidney ratio. Pediatr Radiol 28:98–100CrossRefPubMed
47.
go back to reference Mortele KJ, Mortele B, Silverman SG (2004) CT features of the accessory spleen. AJR Am J Roentgenol 183:1653–1657CrossRefPubMed Mortele KJ, Mortele B, Silverman SG (2004) CT features of the accessory spleen. AJR Am J Roentgenol 183:1653–1657CrossRefPubMed
48.
go back to reference Gayer G, Hertz M, Strauss S et al (2006) Congenital anomalies of the spleen. Semin Ultrasound CT MR 27:358–369CrossRefPubMed Gayer G, Hertz M, Strauss S et al (2006) Congenital anomalies of the spleen. Semin Ultrasound CT MR 27:358–369CrossRefPubMed
49.
go back to reference Paterson A, Frush DP, Donnelly LF et al (1999) A pattern-oriented approach to splenic imaging in infants and children. Radiographics 19:1465–1485CrossRefPubMed Paterson A, Frush DP, Donnelly LF et al (1999) A pattern-oriented approach to splenic imaging in infants and children. Radiographics 19:1465–1485CrossRefPubMed
50.
go back to reference Lombardi R, Menchini L, Corneli T et al (2014) Wandering spleen in children: a report of 3 cases and a brief literature review underlining the importance of diagnostic imaging. Pediatr Radiol 44:279–288CrossRefPubMed Lombardi R, Menchini L, Corneli T et al (2014) Wandering spleen in children: a report of 3 cases and a brief literature review underlining the importance of diagnostic imaging. Pediatr Radiol 44:279–288CrossRefPubMed
51.
go back to reference Dodds WJ, Taylor AJ, Erickson SJ et al (1990) Radiologic imaging of splenic anomalies. AJR Am J Roentgenol 155:805–810CrossRefPubMed Dodds WJ, Taylor AJ, Erickson SJ et al (1990) Radiologic imaging of splenic anomalies. AJR Am J Roentgenol 155:805–810CrossRefPubMed
52.
go back to reference Fulcher AS, Turner MA (2002) Abdominal manifestations of situs anomalies in adults. Radiographics 22:1439–1456CrossRefPubMed Fulcher AS, Turner MA (2002) Abdominal manifestations of situs anomalies in adults. Radiographics 22:1439–1456CrossRefPubMed
53.
go back to reference Lee FT Jr, Pozniak MA, Helgerson RB (1993) US case of the day. Polysplenia syndrome. Radiographics 13:1159–1162 Lee FT Jr, Pozniak MA, Helgerson RB (1993) US case of the day. Polysplenia syndrome. Radiographics 13:1159–1162
54.
go back to reference Applegate KE, Goske MJ, Pierce G et al (1999) Situs revisited: imaging of the heterotaxy syndrome. Radiographics 19:837–852CrossRefPubMed Applegate KE, Goske MJ, Pierce G et al (1999) Situs revisited: imaging of the heterotaxy syndrome. Radiographics 19:837–852CrossRefPubMed
55.
56.
go back to reference McCarville MB, Rogers ZR, Sarnaik S et al (2012) Effects of chronic transfusions on abdominal sonographic abnormalities in children with sickle cell anemia. J Pediatr 160:281–285CrossRefPubMed McCarville MB, Rogers ZR, Sarnaik S et al (2012) Effects of chronic transfusions on abdominal sonographic abnormalities in children with sickle cell anemia. J Pediatr 160:281–285CrossRefPubMed
57.
go back to reference Gale HI, Bobbitt CA, Setty BN et al (2016) Expected sonographic appearance of the spleen in children and young adults with sickle cell disease: an update. J Ultrasound Med 35:1735–1745CrossRefPubMed Gale HI, Bobbitt CA, Setty BN et al (2016) Expected sonographic appearance of the spleen in children and young adults with sickle cell disease: an update. J Ultrasound Med 35:1735–1745CrossRefPubMed
58.
go back to reference Donkol RH, Monib S, Eltounsi I (2006) Tumorlike, iron-sparing foci of splenic hemosiderosis in a child with sickle cell anemia. J Ultrasound Med 25:1607–1609CrossRefPubMed Donkol RH, Monib S, Eltounsi I (2006) Tumorlike, iron-sparing foci of splenic hemosiderosis in a child with sickle cell anemia. J Ultrasound Med 25:1607–1609CrossRefPubMed
Metadata
Title
Ultrasound of congenital and inherited disorders of the pediatric hepatobiliary system, pancreas and spleen
Authors
Susan J. Back
Carolina L. Maya
Asef Khwaja
Publication date
01-08-2017
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Radiology / Issue 9/2017
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-017-3869-y

Other articles of this Issue 9/2017

Pediatric Radiology 9/2017 Go to the issue