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Published in: European Radiology 5/2017

01-05-2017 | Hepatobiliary-Pancreas

The porta hepatis microcyst: an additional sonographic sign for the diagnosis of biliary atresia

Authors: Mériam Koob, Danièle Pariente, Dalila Habes, Béatrice Ducot, Catherine Adamsbaum, Stéphanie Franchi-Abella

Published in: European Radiology | Issue 5/2017

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Abstract

Objectives

To describe and evaluate an additional sonographic sign in the diagnosis of biliary atresia (BA), the microcyst of the porta hepatis, in comparison with previously described signs.

Methods

Ultrasound performed in 321 infants (mean age 55 days) with cholestasis were retrospectively analyzed. BA was surgically confirmed in 193 patients and excluded in 128. US evaluated gallbladder type (1: normal; 2: consistent with BA; 3: suspicious), triangular cord sign (TCS), microcyst and macrocyst, polysplenia syndrome, portal hypertension, and bile duct dilatation. T test and Pearson χ2 test were used to compare US signs between the two groups, followed by univariate regression analysis.

Results

The highest specificity and sensitivity for BA (p < 0.001) were respectively obtained with non-visible gallbladder (100 %-13 %), macrocyst (99 %-10 %), polysplenia (99 %-11 %), microcyst (98 %-20 %), type 2 gallbladder (98 %-34 %), and TCS (97 %-30 %). Combination of signs (macro or microcyst; cyst and no bile duct dilatation; microcyst and/or TCS; type 2 gallbladder and/or cyst) provided better sensitivities (25-49 %) with similar specificities (95-98 %) (p < 0.001).
On univariate analysis, the single US signs most strongly associated with BA were polysplenia (odds ratio, OR 16.3), macrocyst (OR 14.7), TCS (OR 13.4) and microcyst (OR 8).

Conclusions

Porta hepatis microcyst is a reliable US sign for BA diagnosis.

Key Points

The porta hepatis microcyst is a specific sign of biliary atresia.
It was found in 31 (16.1 %) of 193 patients with biliary atresia.
Its specificity was 98 % (p < 0.001).
High frequency transducer and color Doppler can show the porta hepatis microcyst.
Literature
2.
go back to reference Serinet MO, Wildhaber BE, Broue P et al (2009) Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics 123:1280–1286CrossRefPubMed Serinet MO, Wildhaber BE, Broue P et al (2009) Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics 123:1280–1286CrossRefPubMed
3.
go back to reference Lee SY, Kim GC, Choe BH et al (2011) Efficacy of US-guided percutaneous cholecystocholangiography for the early exclusion and type determination of biliary atresia. Radiology 261:916–922CrossRefPubMed Lee SY, Kim GC, Choe BH et al (2011) Efficacy of US-guided percutaneous cholecystocholangiography for the early exclusion and type determination of biliary atresia. Radiology 261:916–922CrossRefPubMed
4.
go back to reference Siles P, Aschero A, Gorincour G et al (2014) A prospective pilot study: can the biliary tree be visualized in children younger than 3 months on magnetic resonance cholangiopancreatography? Pediatr Radiol 44:1077–1084CrossRefPubMed Siles P, Aschero A, Gorincour G et al (2014) A prospective pilot study: can the biliary tree be visualized in children younger than 3 months on magnetic resonance cholangiopancreatography? Pediatr Radiol 44:1077–1084CrossRefPubMed
5.
go back to reference Rastogi A, Krishnani N, Yachha SK, Khanna V, Poddar U, Lal R (2009) Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries. J Gastroenterol Hepatol 24:97–102CrossRefPubMed Rastogi A, Krishnani N, Yachha SK, Khanna V, Poddar U, Lal R (2009) Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries. J Gastroenterol Hepatol 24:97–102CrossRefPubMed
6.
go back to reference Kianifar HR, Tehranian S, Shojaei P et al (2013) Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature. Pediatr Radiol 43:905–919CrossRefPubMed Kianifar HR, Tehranian S, Shojaei P et al (2013) Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature. Pediatr Radiol 43:905–919CrossRefPubMed
7.
go back to reference Choi SO, Park WH, Lee HJ, Woo SK (1996) 'Triangular cord': a sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg 31:363–366CrossRefPubMed Choi SO, Park WH, Lee HJ, Woo SK (1996) 'Triangular cord': a sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg 31:363–366CrossRefPubMed
8.
go back to reference Park WH, Choi SO, Lee HJ, Kim SP, Zeon SK, Lee SL (1997) A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg 32:1555–1559CrossRefPubMed Park WH, Choi SO, Lee HJ, Kim SP, Zeon SK, Lee SL (1997) A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg 32:1555–1559CrossRefPubMed
9.
go back to reference Park WH, Choi SO, Lee HJ (1999) The ultrasonographic 'triangular cord' coupled with gallbladder images in the diagnostic prediction of biliary atresia from infantile intrahepatic cholestasis. J Pediatr Surg 34:1706–1710CrossRefPubMed Park WH, Choi SO, Lee HJ (1999) The ultrasonographic 'triangular cord' coupled with gallbladder images in the diagnostic prediction of biliary atresia from infantile intrahepatic cholestasis. J Pediatr Surg 34:1706–1710CrossRefPubMed
10.
go back to reference Imanieh MH, Dehghani SM, Bagheri MH et al (2010) Triangular cord sign in detection of biliary atresia: is it a valuable sign? Dig Dis Sci 55:172–175CrossRefPubMed Imanieh MH, Dehghani SM, Bagheri MH et al (2010) Triangular cord sign in detection of biliary atresia: is it a valuable sign? Dig Dis Sci 55:172–175CrossRefPubMed
11.
go back to reference Lee HJ, Lee SM, Park WH, Choi SO (2003) Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology 229:395–400CrossRefPubMed Lee HJ, Lee SM, Park WH, Choi SO (2003) Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology 229:395–400CrossRefPubMed
12.
go back to reference Roquete ML, Ferreira AR, Fagundes ED, Castro LP, Silva RA, Penna FJ (2008) Accuracy of echogenic periportal enlargement image in ultrasonographic exams and histopathology in differential diagnosis of biliary atresia. J Pediatr (Rio J) 84:331–336 Roquete ML, Ferreira AR, Fagundes ED, Castro LP, Silva RA, Penna FJ (2008) Accuracy of echogenic periportal enlargement image in ultrasonographic exams and histopathology in differential diagnosis of biliary atresia. J Pediatr (Rio J) 84:331–336
13.
go back to reference Kim WS, Cheon JE, Youn BJ et al (2007) Hepatic arterial diameter measured with US: adjunct for US diagnosis of biliary atresia. Radiology 245:549–555CrossRefPubMed Kim WS, Cheon JE, Youn BJ et al (2007) Hepatic arterial diameter measured with US: adjunct for US diagnosis of biliary atresia. Radiology 245:549–555CrossRefPubMed
14.
go back to reference Lee MS, Kim MJ, Lee MJ et al (2009) Biliary atresia: color doppler US findings in neonates and infants. Radiology 252:282–289CrossRefPubMed Lee MS, Kim MJ, Lee MJ et al (2009) Biliary atresia: color doppler US findings in neonates and infants. Radiology 252:282–289CrossRefPubMed
15.
go back to reference El-Guindi MA, Sira MM, Sira AM et al (2014) Design and validation of a diagnostic score for biliary atresia. J Hepatol 61:116–123CrossRefPubMed El-Guindi MA, Sira MM, Sira AM et al (2014) Design and validation of a diagnostic score for biliary atresia. J Hepatol 61:116–123CrossRefPubMed
16.
go back to reference Caponcelli E, Knisely AS, Davenport M (2008) Cystic biliary atresia: an etiologic and prognostic subgroup. J Pediatr Surg 43:1619–1624CrossRefPubMed Caponcelli E, Knisely AS, Davenport M (2008) Cystic biliary atresia: an etiologic and prognostic subgroup. J Pediatr Surg 43:1619–1624CrossRefPubMed
17.
go back to reference Suzuki T, Hashimoto T, Hussein MH, Hara F, Hibi M, Kato T (2013) Biliary atresia type I cyst and choledochal cyst [corrected]: can we differentiate or not? J Hepatobiliary Pancreat Sci 20:465–470CrossRefPubMed Suzuki T, Hashimoto T, Hussein MH, Hara F, Hibi M, Kato T (2013) Biliary atresia type I cyst and choledochal cyst [corrected]: can we differentiate or not? J Hepatobiliary Pancreat Sci 20:465–470CrossRefPubMed
18.
go back to reference Zhou LY, Guan BY, Li L et al (2012) Objective differential characteristics of cystic biliary atresia and choledochal cysts in neonates and young infants: sonographic findings. J Ultrasound Med 31:833–841CrossRefPubMed Zhou LY, Guan BY, Li L et al (2012) Objective differential characteristics of cystic biliary atresia and choledochal cysts in neonates and young infants: sonographic findings. J Ultrasound Med 31:833–841CrossRefPubMed
19.
go back to reference Kim MJ, Park YN, Han SJ et al (2000) Biliary atresia in neonates and infants: triangular area of high signal intensity in the porta hepatis at T2-weighted MR cholangiography with US and histopathologic correlation. Radiology 215:395–401CrossRefPubMed Kim MJ, Park YN, Han SJ et al (2000) Biliary atresia in neonates and infants: triangular area of high signal intensity in the porta hepatis at T2-weighted MR cholangiography with US and histopathologic correlation. Radiology 215:395–401CrossRefPubMed
20.
go back to reference Shin NY, Kim MJ, Lee MJ et al (2014) Transient elastography and sonography for prediction of liver fibrosis in infants with biliary atresia. J Ultrasound Med 33:853–864CrossRefPubMed Shin NY, Kim MJ, Lee MJ et al (2014) Transient elastography and sonography for prediction of liver fibrosis in infants with biliary atresia. J Ultrasound Med 33:853–864CrossRefPubMed
21.
go back to reference Tainaka T, Kaneko K, Nakamura S, Ono Y, Sumida W, Ando H (2008) Histological assessment of bile lake formation after hepatic portoenterostomy for biliary atresia. Pediatr Surg Int 24:265–269CrossRefPubMed Tainaka T, Kaneko K, Nakamura S, Ono Y, Sumida W, Ando H (2008) Histological assessment of bile lake formation after hepatic portoenterostomy for biliary atresia. Pediatr Surg Int 24:265–269CrossRefPubMed
22.
go back to reference Lal R, Prasad DK, Krishna P et al (2007) Biliary atresia with a "cyst at porta": management and outcome as per the cholangiographic anatomy. Pediatr Surg Int 23:773–778CrossRefPubMed Lal R, Prasad DK, Krishna P et al (2007) Biliary atresia with a "cyst at porta": management and outcome as per the cholangiographic anatomy. Pediatr Surg Int 23:773–778CrossRefPubMed
23.
go back to reference Gezer HO, Oguzkurt P, Temiz A, Ince E, Ezer SS, Hicsonmez A (2015) Choledochal cysts in children: Intrahepatic ductal dilatation does not indicate true intrahepatic biliary duct disease. Turk J Gastroenterol. doi:10.5152/tjg.2015.150211 PubMed Gezer HO, Oguzkurt P, Temiz A, Ince E, Ezer SS, Hicsonmez A (2015) Choledochal cysts in children: Intrahepatic ductal dilatation does not indicate true intrahepatic biliary duct disease. Turk J Gastroenterol. doi:10.​5152/​tjg.​2015.​150211 PubMed
24.
go back to reference Ohi R, Ibrahim M (1992) Biliary atresia. Semin Pediatr Surg 1:115–124PubMed Ohi R, Ibrahim M (1992) Biliary atresia. Semin Pediatr Surg 1:115–124PubMed
25.
go back to reference Tan CE, Driver M, Howard ER, Moscoso GJ (1994) Extrahepatic biliary atresia: a first-trimester event? Clues from light microscopy and immunohistochemistry. J Pediatr Surg 29:808–814CrossRefPubMed Tan CE, Driver M, Howard ER, Moscoso GJ (1994) Extrahepatic biliary atresia: a first-trimester event? Clues from light microscopy and immunohistochemistry. J Pediatr Surg 29:808–814CrossRefPubMed
26.
go back to reference Da Ines D, Essamet W, Montoriol PF (2011) Peribiliary cysts. Hepatology 54:2271–2272PubMed Da Ines D, Essamet W, Montoriol PF (2011) Peribiliary cysts. Hepatology 54:2271–2272PubMed
27.
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
29.
go back to reference Tan Kendrick AP, Phua KB, Ooi BC, Subramaniam R, Tan CE, Goh AS (2000) Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length. Pediatr Radiol 30:69–73CrossRefPubMed Tan Kendrick AP, Phua KB, Ooi BC, Subramaniam R, Tan CE, Goh AS (2000) Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length. Pediatr Radiol 30:69–73CrossRefPubMed
30.
go back to reference Jiang LP, Chen YC, Ding L et al (2013) The diagnostic value of high-frequency ultrasonography in biliary atresia. Hepatobiliary Pancreat Dis Int 12:415–422CrossRefPubMed Jiang LP, Chen YC, Ding L et al (2013) The diagnostic value of high-frequency ultrasonography in biliary atresia. Hepatobiliary Pancreat Dis Int 12:415–422CrossRefPubMed
31.
go back to reference Takamizawa S, Zaima A, Muraji T et al (2007) Can biliary atresia be diagnosed by ultrasonography alone? J Pediatr Surg 42:2093–2096CrossRefPubMed Takamizawa S, Zaima A, Muraji T et al (2007) Can biliary atresia be diagnosed by ultrasonography alone? J Pediatr Surg 42:2093–2096CrossRefPubMed
32.
go back to reference Li SX, Zhang Y, Sun M et al (2008) Ultrasonic diagnosis of biliary atresia: a retrospective analysis of 20 patients. World J Gastroenterol 14:3579–3582CrossRefPubMedPubMedCentral Li SX, Zhang Y, Sun M et al (2008) Ultrasonic diagnosis of biliary atresia: a retrospective analysis of 20 patients. World J Gastroenterol 14:3579–3582CrossRefPubMedPubMedCentral
33.
go back to reference Saxena AK, Mittal V, Sodhi KS (2012) Triangular cord sign in biliary atresia: does it have prognostic and medicolegal significance? Radiology 263:621, author reply 621-622CrossRefPubMed Saxena AK, Mittal V, Sodhi KS (2012) Triangular cord sign in biliary atresia: does it have prognostic and medicolegal significance? Radiology 263:621, author reply 621-622CrossRefPubMed
35.
go back to reference Sun Y, Zheng S, Qian Q (2011) Ultrasonographic evaluation in the differential diagnosis of biliary atresia and infantile hepatitis syndrome. Pediatr Surg Int 27:675–679CrossRefPubMed Sun Y, Zheng S, Qian Q (2011) Ultrasonographic evaluation in the differential diagnosis of biliary atresia and infantile hepatitis syndrome. Pediatr Surg Int 27:675–679CrossRefPubMed
36.
go back to reference Aziz S, Wild Y, Rosenthal P, Goldstein RB (2011) Pseudo gallbladder sign in biliary atresia--an imaging pitfall. Pediatr Radiol 41:620–626, quiz 681-622CrossRefPubMedPubMedCentral Aziz S, Wild Y, Rosenthal P, Goldstein RB (2011) Pseudo gallbladder sign in biliary atresia--an imaging pitfall. Pediatr Radiol 41:620–626, quiz 681-622CrossRefPubMedPubMedCentral
37.
go back to reference Matar M, Ayoubi JM, Picone O (2014) Prenatal diagnosis of gallbladder abnormalities: a review. J Gynecol Obstet Biol Reprod (Paris) 43:581–586CrossRef Matar M, Ayoubi JM, Picone O (2014) Prenatal diagnosis of gallbladder abnormalities: a review. J Gynecol Obstet Biol Reprod (Paris) 43:581–586CrossRef
38.
go back to reference Peng SS, Jeng YM, Hsu WM, Yang JC, Ho MC (2015) Hepatic ADC map as an adjunct to conventional abdominal MRI to evaluate hepatic fibrotic and clinical cirrhotic severity in biliary atresia patients. Eur Radiol 25:2992–3002CrossRefPubMed Peng SS, Jeng YM, Hsu WM, Yang JC, Ho MC (2015) Hepatic ADC map as an adjunct to conventional abdominal MRI to evaluate hepatic fibrotic and clinical cirrhotic severity in biliary atresia patients. Eur Radiol 25:2992–3002CrossRefPubMed
Metadata
Title
The porta hepatis microcyst: an additional sonographic sign for the diagnosis of biliary atresia
Authors
Mériam Koob
Danièle Pariente
Dalila Habes
Béatrice Ducot
Catherine Adamsbaum
Stéphanie Franchi-Abella
Publication date
01-05-2017
Publisher
Springer Berlin Heidelberg
Published in
European Radiology / Issue 5/2017
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-016-4546-5

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