Extra-Hepatic portal venous obstruction with portal biliopathy in infant presenting as neonatal cholestasis: a rare case report and review of literature
Keywords:
Extra-hepatic portal obstruction, Jaundice, Portal biliopathy
Abstract
Extra-hepatic portal venous obstruction is most common cause of portal hypertension in children. Apart from variceal formation in gastric and esophageal mucosal layer, it has been postulated that external pressure and or ischemic injury play a role in portal biliopathy. We report an unusual case of a 6 month old infant who presented to us with clinical features suggestive of extra-hepatic biliary atresia; diagnosed as EHPVO with portal biliopathy, managed surgically.
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References
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24. Petren T. The veins of the extrahepatic biliary system and their pathologic anatomic significance. Vert Anat Ges. 1932;41:139–143.
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28. Gulati G, Pawa S, Chowdhary V, Kumar N, Mittal SK. Colour Doppler flow imaging findings in portal biliopathy. Trop Gastroenterol. 2003 Jul-Sep;24(3):116-9. [PubMed]
29. Khuroo MS, Rather AA, Khuroo NS, Khuroo MS. Portal biliopathy. World J Gastroenterol. 2016 Sep 21;22(35):7973-82. doi: 10.3748/wjg.v22.i35.7973. [PubMed]
30. Besa C, Cruz JP, Huete A, Cruz F. Portal biliopathy: a multitechnique imaging approach. Abdom Imaging. 2012 Feb;37(1):83-90. doi: 10.1007/s00261-011-9765-2. [PubMed]
31. Magnuson TH, Bender JS, Duncan MD, Ahrendt SA, Harmon JW, Regan F. Utility of magnetic resonance cholangiography in the evaluation of biliary obstruction. J Am Coll Surg. 1999 Jul;189(1):63-71; discussion 71-2.
2. Poddar U, Thapa BR, Rao KL, Singh K. Etiological spectrum of esophageal varices due to portal hypertension in Indian children: is it different from the West? J Gastroenterol Hepatol. 2008 Sep;23(9):1354-7. [PubMed]
3. Poddar U, Thapa BR, Puri P, Girish CS, Vaiphei K, Vasishta RK, Singh K. Non-cirrhotic portal fibrosis in children. Indian J Gastroenterol. 2000 Jan-Mar;19(1):12-3. [PubMed]
4. Dhiman RK, Behera A, Chawla YK, Dilawari JB, Suri S. Portal hypertensive biliopathy. Gut. 2007 Jul;56(7):1001-8. [PubMed]
5. Ho C, Gunn A, Noordin M. Portal biliopathy. Med J Malaysia. 2014 Oct;69(5):236-7. [PubMed]
6. Dilawari JB, Chawla YK. Pseudosclerosing cholangitis in extrahepatic portal venous obstruction. Gut. 1992 Feb;33(2):272-6. [PubMed]
7. Sarin SK, Agarwal SR. Extrahepatic portal vein obstruction. Semin Liver Dis. 2002 Feb;22(1):43-58. [PubMed]
8. Khuroo MS, Yattoo GN, Zargar SA, Javid G, Dar MY, Khan BA, Boda MI. Biliary abnormalities associated with extrahepatic portal venous obstruction. Hepatology. 1993 May;17(5):807-13.
9. Bayraktar Y, Balkanci F, Kayhan B, Ozenç A, Arslan S, Telatar H. Bile duct varices or "pseudo-cholangiocarcinoma sign" in portal hypertension due to cavernous transformation of the portal vein. Am J Gastroenterol. 1992 Dec;87(12):1801-6. [PubMed]
10. Sezgin O, Oğuz D, Altintaş E, Saritaş U, Sahin B. Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein. Gastrointest Endosc. 2003 Oct;58(4):602-8. [PubMed]
11. Hanif FM, Soomro GB, Akhund SN, Luck NH, Laeeq SM, Abbas Z, Hassan SM, Mubarak M. Clinical presentation of extrahepatic portal vein obstruction: 10-year experience at a tertiary care hospital in Pakistan. J Transl Int Med. 2015 Apr-Jun;3(2):74-78. [PubMed]
12. Agarwal AK, Sharma D, Singh S, Agarwal S, Girish SP. Portal biliopathy: a study of 39 surgically treated patients. HPB (Oxford). 2011 Jan;13(1):33-9. [PubMed]
13. Chattopadhyay S, Nundy S. Portal biliopathy. World J Gastroenterol. 2012 Nov 21;18(43):6177-82. [PubMed]
14. GIBSON JB, JOHNSTON GW, FULTON TT, RODGERS HW. EXTRAHEPATIC PORTAL-VENOUS OBSTRUCTION. Br J Surg. 1965 Feb;52:129-39. [PubMed]
15. Webb LJ, Sherlock S. The aetiology, presentation and natural history of extra-hepatic portal venous obstruction. Q J Med. 1979 Oct;48(192):627-39. [PubMed]
16. Rangari M, Gupta R, Jain M, Malhotra V, Sarin SK. Hepatic dysfunction in patients with extrahepatic portal venous obstruction. Liver Int. 2003 Dec;23(6):434-9. [PubMed]
17. Narendra K. Arora, Manoja K. Das. Extra Hepatic Portal Venous Obstruction in Children, Portal Hypertension - Causes and Complications, Prof. Dmitry Garbuzenko (Ed.), Available from: http://www.intechopen.com/books/portal-hypertension-causes-and-complications/extra-hepaticportal- venous-obstruction-in-children. 2012th ed. InTech; 2012.
18. El-Matary W, Roberts EA, Kim P, Temple M, Cutz E, Ling SC. Portal hypertensive biliopathy: a rare cause of childhood cholestasis. Eur J Pediatr. 2008 Nov;167(11):1339-42. [PubMed]
19. Khuroo MS, Yattoo GN, Zargar SA, Javid G, Dar MY, Khan BA, Boda MI. Biliary abnormalities associated with extrahepatic portal venous obstruction. Hepatology. 1993 May;17(5):807-13.
20. Malkan GH, Bhatia SJ, Bashir K, Khemani R, Abraham P, Gandhi MS, Radhakrishnan R. Cholangiopathy associated with portal hypertension: diagnostic evaluation and clinical implications. Gastrointest Endosc. 1999 Mar;49(3 Pt 1):344-8.
21. Condat B, Vilgrain V, Asselah T, O'Toole D, Rufat P, Zappa M, Moreau R, Valla D. Portal cavernoma-associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study. Hepatology. 2003 Jun;37(6):1302-8.
22. Nagi B, Kochhar R, Bhasin D, Singh K. Cholangiopathy in extrahepatic portal venous obstruction. Radiological appearances. Acta Radiol. 2000 Nov;41(6):612-5.
23. SAINT JH. The epicholedochal venous plexus and its importance as a means of identifying the common duct during operations on the extrahepatic biliary tract. Br J Surg. 1961 Mar;48:489-98.
24. Petren T. The veins of the extrahepatic biliary system and their pathologic anatomic significance. Vert Anat Ges. 1932;41:139–143.
25. Dhiman RK, Puri P, Chawla Y, Minz M, Bapuraj JR, Gupta S, Nagi B, Suri S. Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic? Gastrointest Endosc. 1999 Nov;50(5):646-52. [PubMed]
26. Valla DC, Condat B, Lebrec D. Spectrum of portal vein thrombosis in the West. J Gastroenterol Hepatol. 2002 Dec;17 Suppl 3:S224-7. [PubMed]
27. Nunoi H, Hirooka M, Ochi H, Koizumi Y, Tokumoto Y, Abe M, Tada F, Ikeda Y, Matsuura B, Tanaka H, Tsuda T, Mochizuki T, Hiasa Y, Onji M. Portal biliopathy diagnosed using color Doppler and contrast-enhanced ultrasound. Intern Med. 2013;52(10):1055-9. [PubMed]
28. Gulati G, Pawa S, Chowdhary V, Kumar N, Mittal SK. Colour Doppler flow imaging findings in portal biliopathy. Trop Gastroenterol. 2003 Jul-Sep;24(3):116-9. [PubMed]
29. Khuroo MS, Rather AA, Khuroo NS, Khuroo MS. Portal biliopathy. World J Gastroenterol. 2016 Sep 21;22(35):7973-82. doi: 10.3748/wjg.v22.i35.7973. [PubMed]
30. Besa C, Cruz JP, Huete A, Cruz F. Portal biliopathy: a multitechnique imaging approach. Abdom Imaging. 2012 Feb;37(1):83-90. doi: 10.1007/s00261-011-9765-2. [PubMed]
31. Magnuson TH, Bender JS, Duncan MD, Ahrendt SA, Harmon JW, Regan F. Utility of magnetic resonance cholangiography in the evaluation of biliary obstruction. J Am Coll Surg. 1999 Jul;189(1):63-71; discussion 71-2.
CITATION
DOI: 10.17511/ijpr.2016.i12.06
Published: 2016-12-31
How to Cite
Dr Ajeet Kumar Yadav, Dr Ramkesh Meena, Dr Bhavika YM, Dr Guruayyappa Ramsingh, & Dr Vivek Dewan. (2016). Extra-Hepatic portal venous obstruction with portal biliopathy in infant presenting as neonatal cholestasis: a rare case report and review of literature. Pediatric Review: International Journal of Pediatric Research, 3(12), 889-892. https://doi.org/10.17511/ijpr.2016.i12.06
Section
Case Report
Copyright (c) 2016 Author (s). Published by Siddharth Health Research and Social Welfare Society
This work is licensed under a Creative Commons Attribution 4.0 International License.
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