Journal List > Korean J Pediatr Gastroenterol Nutr > v.14(2) > 1043497

Korean J Pediatr Gastroenterol Nutr. 2011 Jun;14(2):122-129. Korean.
Published online June 30, 2011.  https://doi.org/10.5223/kjpgn.2011.14.2.122
Copyright © 2011 The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition
Early Exclusive Diagnosis of Biliary Atresia among Infants with Cholestasis
Byung-Ho Choe, M.D.
Department of Pediatrics, Kyungpook National University School of Medicine, Daegu, Korea.

Corresponding author (Email: bhchoi@knu.ac.kr )
Received May 26, 2011; Revised June 07, 2011; Accepted June 10, 2011.

Abstract

The persistence of jaundice beyond the first 2 weeks of life require further investigation and this can be determined if the conjugated bilirubin levels are greater than 1.5 mg/dL or greater than 20% of the total bilirubin level. There is a diverse differential diagnosis for the cause of neonatal cholestasis due to hepatobiliary disease including biliary atresia, which eventually leads to liver cirrhosis if uncorrected before 60~80 days of life. Long-established initial studies include abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy, but better diagnostic methods are needed. Promising new options are described including MRCP (magnetic resonance cholangiography), ERCP (endoscopic retrograde cholangiography), and PCC (percutaneous cholecysto-cholangiography). Though no single test can differentiate biliary atresia from other neonatal cholestasis with confidence, a combination of diagnostic methods is usually consistently beneficial. By excluding biliary atresia as early as possible, the risk of unnecessary explolaparotomy with intraoperative cholangiography is decreased. Further evaluation would be required for the diagnosis of neonatal cholestasis after excluding biliary atresia.

Keywords: Newborn; Cholestasis; Jaundice; Biliary atresia; Hepatitis; Diagnosis; Cholangiography

Figures


Fig. 1
Classification of cholestasis in infants. *NH: neonatal hepatitis; Ds: disease; UTI: urinary tract infection; PFIC: progressive familial intrahepatic cholestasis; TPN: total parental nutrition.
Click for larger image


Fig. 2
Suggested approach for the exclusion of biliary atresia in infants with cholestasis. *Bil (D): direct bilirubin; IBEM: inborn error of metabolism; MS: mass spectrometry; AA: amino acid analysis; USG: ultrasonograpghy; NS: nonspecific finding; Bx: biopsy; Op: operation; BA: biliary atresia.
Click for larger image

References
1. Choe BH, Bezerra JA, Balistreri WF. Transplantation for cholestatic liver disease in the pediatric patient. In: Busuttil RW, Klintmalm GB, editors. Transplantation of the Liver. 2nd ed. Philadelphia: Elsevier Saunders; 2005. pp. 303-321.
2. Danks DM, Campbell PE, Smith AL, Rogers J. Prognosis of babies with neonatal hepatitis. Arch Dis Child 1977;52:368–372.
3. Mowat AP, Psacharopoulos HT, Williams R. Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants. Arch Dis Child 1976;51:763–770.
4. Tazawa Y, Kobayashi K, Abukawa D, Nagata I, Maisawa S, Sumazaki R, et al. Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients. Mol Genet Metab 2004;83:213–219.
5. Ko JS, Song JH, Park SS, Seo JK. Neonatal intrahepatic cholestasis caused by citrin deficiency in Korean infants. J Korean Med Sci 2007;22:952–956.
6. Alagille D, Estrada A, Hadchouel M, Gautier M, Odievre M, Dommergues JP. Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases. J Pediatr 1987;110:195–200.
7. Deprettere A, Portmann B, Mowat AP. Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty; severe morbidity throughout early childhood. J Pediatr Gastroenterol Nutr 1987;6:865–871.
8. Oda T, Elkahloun AG, Pike BL, Okajima K, Krantz ID, Genin A, et al. Mutations in the human Jagged1 gene are responsible for Alagille syndrome. Nat Genet 1997;16:235–242.
9. Bates MD, Bucuvalas JC, Alonso MH, Ryckman FC. Biliary atresia: pathogenesis and treatment. Semin Liver Dis 1998;18:281–293.
10. Mieli-Vergani G, Howard ER, Portman B, Mowat AP. Late referral for biliary atresia--missed opportunities for effective surgery. Lancet 1989;1:421–423.
11. Grosfeld JL, Fitzgerald JF, Predaina R, West KW, Vane DW, Rescorla FJ. The efficacy of hepatoportoenterostomy in biliary atresia. Surgery 1989;106:692–700.
discussion-1.
12. Volpert D, White F, Finegold MJ, Molleston J, Debaun M, Perlmutter DH. Outcome of early hepatic portoenterostomy for biliary atresia. J Pediatr Gastroenterol Nutr 2001;32:265–269.
13. Schoen BT, Lee H, Sullivan K, Ricketts RR. The Kasai portoenterostomy: when is it too late? J Pediatr Surg 2001;36:97–99.
14. Carceller A, Blanchard H, Alvarez F, St-Vil D, Bensoussan AL, Di Lorenzo M. Past and future of biliary atresia. J Pediatr Surg 2000;35:717–720.
15. Dolgin SE. Answered and unanswered controversies in the surgical management of extra hepatic biliary atresia. Pediatr Transplant 2004;8:628–631.
16. Maller ES. Liver function tests. In: Altschuler SM, Liacouras CA, editors. Clinical pediatric gastroenterology. 1st ed. Philadelphia: Churchill Livingstone; 1998. pp. 575-578.
17. Park WH, Choi SO, Lee HJ, Kim SP, Zeon SK, Lee SL. 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 1997;32:1555–1559.
18. Park WH, Choi SO, Lee HJ. The ultrasonographic 'triangular cord' coupled with gallbladder images in the diagnostic prediction of biliary atresia from infantile intrahepatic cholestasis. J Pediatr Surg 1999;34:1706–1710.
19. Park WH, Choi SO, Lee HJ. Technical innovation for noninvasive and early diagnosis of biliary atresia: the ultrasonographic "triangular cord" sign. J Hepatobiliary Pancreat Surg 2001;8:337–341.
20. Lee HJ, Lee SM, Park WH, Choi SO. Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology 2003;229:395–400.
21. Kanegawa K, Akasaka Y, Kitamura E, Nishiyama S, Muraji T, Nishijima E, et al. Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction. AJR Am J Roentgenol 2003;181:1387–1390.
22. Tan Kendrick AP, Phua KB, Ooi BC, Subramaniam R, Tan CE, Goh AS. Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length. Pediatr Radiol 2000;30:69–73.
23. Kim JM, Choe BH, Jang YC, Oh KW, Cho MH, Lee KH, et al. Diagnostic limitation and usefulness of (99m)Tc-DISIDA hepatobiliary scanning on neonatal cholestasis. Korean J Pediatr 2006;49:737–744.
24. Norton KI, Glass RB, Kogan D, Lee JS, Emre S, Shneider BL. MR cholangiography in the evaluation of neonatal cholestasis: initial results. Radiology 2002;222:687–691.
25. Ryeom HK, Choe BH, Kim JY, Kwon S, Ko CW, Kim HM, et al. Biliary atresia: feasibility of mangafodipir trisodium-enhanced MR cholangiography for evaluation. Radiology 2005;235:250–258.
26. Ohnuma N, Takahashi T, Tanabe M, Yoshida H, Iwai J. The role of ERCP in biliary atresia. Gastrointest Endosc 1997;45:365–370.
27. Petersen C, Meier PN, Schneider A, Turowski C, Pfister ED, Manns MP, et al. Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia. J Hepatol 2009;51:1055–1060.
28. Shanmugam NP, Harrison PM, Devlin J, Peddu P, Knisely AS, Davenport M, et al. Selective use of endoscopic retrograde cholangiopancreatography in the diagnosis of biliary atresia in infants younger than 100 days. J Pediatr Gastroenterol Nutr 2009;49:435–441.
29. Hadzic N, Harrison PM. Selective rather than routine approach to endosopic retrograde cholangio-pancreatography in diagnosis of biliary atresia. J Hepatol 2010;52:777.
30. Shin KM, Ryeom HK, Choe BH, Kim KC, Kim JY, Lee JM, et al. Ultrasound-guided Percutaneous Cholecysto-Cholangiography for the Exclusion of Biliary Atresia in Infants. J Korean Radiol Soc 2006;55:177–182.
31. Nwomeh BC, Caniano DA, Hogan M. Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography. Pediatr Surg Int 2007;23:845–849.
32. Lee SY, Kim GC, Jang YJ, Kim HJ, Ryeom HK, Choe BH, et al. The efficacy of ultrasound-guided percutaneous cholecysto-cholangiography for the early exclusion and type determination of biliary atresia. Radiology. 2011
(forthcoming).
33. Yamamoto H, Yoshida M, Ikeda S, Terakura H, Sera Y. Laparoscopic cholecystcholangiography in a patient with biliary atresia. Surg Laparosc Endosc 1994;4:370–372.
34. Hay SA, Soliman HE, Sherif HM, Abdelrahman AH, Kabesh AA, Hamza AF. Neonatal jaundice: the role of laparoscopy. J Pediatr Surg 2000;35:1706–1709.
35. Balistreri WF. Pediatric hepatology. A half-century of progress. Clin Liver Dis 2000;4:191–210.
36. Finegold MJ. Common diagnostic problems in pediatric liver pathology. Clin Liver Dis 2002;6:421–454.
37. Kahn E. Paucity of interlobular bile ducts. Arteriohepatic dysplasia and nonsyndromic duct paucity. Perspect Pediatr Pathol 1991;14:168–215.
38. Choe BH, Kim KM, Kwon S, Lee KS, Koo JH, Lee HM, et al. The pattern of differentially expressed genes in biliary atresia. J Korean Med Sci 2003;18:392–396.
39. Choi B, Choe BH, Chung EJ, Kim KM, Kim HM, Park JY, et al. Patterns of Intrahepatic Gene Expression in Neonatal Cholestasis. Korean J Pediatr Gastroenterol Nutr 2005;8:177–193.
40. Wang H, Malone JP, Gilmore PE, Davis AE, Magee JC, Townsend RR, et al. Serum markers may distinguish biliary atresia from other forms of neonatal cholestasis. J Pediatr Gastroenterol Nutr 2010;50:411–416.