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.
Similar content being viewed by others
References
Hartley JL, Davenport M, Kelly DA (2009) Biliary atresia. Lancet 374:1704–1713
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 123:1280–1286
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 261:916–922
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 44:1077–1084
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 24:97–102
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 43:905–919
Choi SO, Park WH, Lee HJ, Woo SK (1996) 'Triangular cord': a sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg 31:363–366
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 32:1555–1559
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 34:1706–1710
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 55:172–175
Lee HJ, Lee SM, Park WH, Choi SO (2003) Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology 229:395–400
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
Kim WS, Cheon JE, Youn BJ et al (2007) Hepatic arterial diameter measured with US: adjunct for US diagnosis of biliary atresia. Radiology 245:549–555
Lee MS, Kim MJ, Lee MJ et al (2009) Biliary atresia: color doppler US findings in neonates and infants. Radiology 252:282–289
El-Guindi MA, Sira MM, Sira AM et al (2014) Design and validation of a diagnostic score for biliary atresia. J Hepatol 61:116–123
Caponcelli E, Knisely AS, Davenport M (2008) Cystic biliary atresia: an etiologic and prognostic subgroup. J Pediatr Surg 43:1619–1624
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 20:465–470
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 31:833–841
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 215:395–401
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 33:853–864
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 24:265–269
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 23:773–778
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
Ohi R, Ibrahim M (1992) Biliary atresia. Semin Pediatr Surg 1:115–124
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 29:808–814
Da Ines D, Essamet W, Montoriol PF (2011) Peribiliary cysts. Hepatology 54:2271–2272
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–W445
Humphrey TM, Stringer MD (2007) Biliary atresia: US diagnosis. Radiology 244:845–851
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 30:69–73
Jiang LP, Chen YC, Ding L et al (2013) The diagnostic value of high-frequency ultrasonography in biliary atresia. Hepatobiliary Pancreat Dis Int 12:415–422
Takamizawa S, Zaima A, Muraji T et al (2007) Can biliary atresia be diagnosed by ultrasonography alone? J Pediatr Surg 42:2093–2096
Li SX, Zhang Y, Sun M et al (2008) Ultrasonic diagnosis of biliary atresia: a retrospective analysis of 20 patients. World J Gastroenterol 14:3579–3582
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 621-622
Zhou LY, Wang W, Shan QY et al (2015) Optimizing the US diagnosis of biliary atresia with a modified triangular cord thickness and gallbladder classification. Radiology. doi:10.1148/radiol.2015142309:142309
Sun Y, Zheng S, Qian Q (2011) Ultrasonographic evaluation in the differential diagnosis of biliary atresia and infantile hepatitis syndrome. Pediatr Surg Int 27:675–679
Aziz S, Wild Y, Rosenthal P, Goldstein RB (2011) Pseudo gallbladder sign in biliary atresia--an imaging pitfall. Pediatr Radiol 41:620–626, quiz 681-622
Matar M, Ayoubi JM, Picone O (2014) Prenatal diagnosis of gallbladder abnormalities: a review. J Gynecol Obstet Biol Reprod (Paris) 43:581–586
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 25:2992–3002
Acknowledgments
The scientific guarantor of this publication is Dr Pariente. The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article. The authors state that this work has not received any funding. One of the authors has significant statistical expertise. Institutional Review Board approval was obtained. Written informed consent was waived by the Institutional Review Board. Methodology: retrospective, diagnostic or prognostic study, performed at one institution.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Koob, M., Pariente, D., Habes, D. et al. The porta hepatis microcyst: an additional sonographic sign for the diagnosis of biliary atresia. Eur Radiol 27, 1812–1821 (2017). https://doi.org/10.1007/s00330-016-4546-5
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00330-016-4546-5