Details
Category: Volume 10 Issue 11 November 2022
Hits: 516

Title: A Prospective Study on Etiology Based Prevalence, Clinical Spectrum and Outcome of Budd Chiari Syndrome in Southern India

Authors: Arnab Dey, K. Narayanasamy, K. Premkumar, A. Santhi Selvi

 DOI: https://dx.doi.org/10.18535/jmscr/v10i11.09

Abstract

Aim: Budd–Chiari syndrome, or Hepatic venous outflow obstruction (HVOO) is defined as the obstruction of hepatic venous outflow regardless of its causative mechanism or level of obstruction. Indian studies on BCS date back to the 1970s. It was suggested that in contrast to western world where hepatic vein (HV) obstruction is a commoner IVC obstruction is a commoner in the far east. This study was done to evaluate the aetiology-based prevalence, clinical spectrum with pattern of obstruction, outcome and non-surgical treatment in patients with BCS from southern part of India.

Methods: All the consecutive cases of Budd Chiari syndrome (BCS) or Hepatic Vein Outflow Obstruction (HVOO) in the study period from September 2018 to July 2021 were prospectively evaluated. Diagnosis was based on angiographic evidence of HVOTO (i.e., obstruction of IVC and/or HV). All the patients with BCS were subjected to tests available for hypercoagulable state before starting any treatment and if required was confirmed in the first degree relative for any hypercoagulable state. After the confirmation of the diagnosis patients were taken up for appropriate radiological intervention after discussion with the intervention radiology and anaesthesia team.

Results: Seventy-eight patients, forty-three female and thirty-five males had radiologically confirmed BCS. The mean age at presentation was 35.86 years (range 10–65 years). Twenty-seven patients had acute presentation and fifty-one had chronic illness. Abdominal pain and jaundice were more common in patients with acute disease than the chronic patients but distended veins were more commonly seen in the later. Right upper quadrant pain or epigastric pain (65.38%), distension of the abdomen (75.64%), pedal oedema (41%), UGIB (24.35%) were the primary symptoms and distended veins (66.67%), hepatomegaly (69.2%) were the most common presenting signs. Hypercoagulable state was the etiology in 14 (18%) patients. 48 (61.53%) patients had both IVC and HV obstruction. Associated portal venous thrombosis was seen in 8 (10.25%) patients. Out of the 27 patients that presented with the acute onset of the disease, 2 (7.4%) patients underwent balloon angioplasty of the IVC obstruction.3 (11.1%) patients underwent angioplasty with stenting and they had pure hepatic vein obstruction. 5 (18.5%) patients underwent TIPS procedure. Out of the 51 patients that presented with chronic disease, two third of the patients had radiological features suggestive of established cirrhosis. 12 (23.5%) patients with combined IVC and hepatic vein obstruction underwent angioplasty with stenting.

Conclusion: Combined obstruction is more common in India in contrast to the west. Hypercoagulable states are a common cause of etiology in Indian patients which was reported earlier in very few studies. Combined IVC and HV obstruction can be treated with angioplasty with stenting as demonstrated in our study which is usually difficult to treat. Significant mortality seen with surgical procedures is not seen in patients treated with non-surgical methods. TIPS has been useful in patients with complete HV obstruction with IVC obstruction.    

Keywords: Budd Chiari syndrome; HVOO; TIPS in BCS; Angioplasty in BCS; Acute BCS; Chronic BCS.

References

  1. Hernandez-Guerra M, Turnes J, Rubinstein P, Olliff S, Elias E, Bosch J, Garcia-Pagan JC. PTFE‐covered stents improve TIPS patency in Budd‐Chiari syndrome - Hernández‐Guerra - 2004 - Hepatology - Wiley Online Library [Internet]. [cited 2021 Aug 13]. Available from: https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/hep.20436
  2. Dilawari JB, Bambery P, Chawla Y, Kaur U, Bhusnurmath SR, Malhotra HS, et al. Hepatic outflow obstruction (Budd-Chiari syndrome). Experience with 177 patients and a review of the literature. Medicine. 1994;73(1):21–36.
  3. Kohli V, Pande GK, Reddy KS, Nundy S, Dev V, Kaul U. Management of hepatic venous outflow obstruction. The Lancet. 1993;342(8873):718–22.
  4. Datta DV, Vashishta S, Samanta AK, Chhuttani PN. Diagnostic value of combined transhepatic venography and inferior vena cavography in chronic Budd-Chiari syndrome. The American journal of digestive diseases. 1978;23(11):1031–41.
  5. Datta DV, Saha S, Singh SA, Gupta BB, Aikat BK, Chugh KS, Chhuttani PN. Chronic Budd-Chiari syndrome due to obstruction of the intrahepatic portion of the inferior vena cava | Gut [Internet]. [cited 2021 Aug 13]. Available from: https://gut.bmj.com/content/13/5/372.abstract
  6. Taneja A, Mitra SK, Moghe PD, Rao PN, Samanta N, Kumar L. Budd-Chiari Syndrome in Childhood Secondary to Inferior Vena Caval Obstruction | American Academy of Pediatrics [Internet]. [cited 2021 Aug 13]. Available from: https://pediatrics.aappublications.org/content/63/5/808
  7. AIKAT B, SR B, PN C, DV D. HEPATIC VEIN OBSTRUCTION. A RETROSPECTIVE ANALYSIS OF 72 AUTOPSIES AND BIOPSIES. HEPATIC VEIN OBSTRUCTION A RETROSPECTIVE ANALYSIS OF 72 AUTOPSIES AND BIOPSIES. 1978;
  8. Singh V, Sinha SK, Nain CK, Bambery P, Kaur U, Verma S, et al. Budd–Chiari syndrome: our experience of 71 patients. Journal of gastroenterology and hepatology. 2000;15(5):550–4.
  9. De BK, Biswas PK, Sen S, Das D, De KK, Das U, et al. Management of the Budd-Chiari syndrome by balloon cavoplasty. Indian J Gastroenterol. 2001 Jul 1;20(4):151–4.
  10. Aikat BK, Bhusnurmath SR, Pal AK, Chhuttani PN, Datta DV. Amoebic liver abscess--a clinicopathological study. Indian J Med Res. 1978 Mar;67:381–91.
  11. Khuroo MS, Datta DV. Budd-Chiari syndrome following pregnancy: Report of 16 cases, with roentgenologic, hemodynamic and histologic studies of the hepatic outflow tract. The American Journal of Medicine. 1980 Jan 1;68(1):113–21.
  12. Koshy A, Bhusnurmath SR, Mitra SK, Mahajan KK, Datta DV, Aikat BK, Bhagwat AG. EBSCOhost | 16276257 | Hydatid Disease Associated with Hepatic Outflow Tract Obstruction.
  13. Valla DC. The diagnosis and management of the Budd‐Chiari syndrome: Consensus and controversies - Valla - 2003 - Hepatology - Wiley Online Library
  14. Parker RGF. Occlusion of the hepatic veins in man. Medicine. 1959;38(4):369–402.
  15. Mohanty D, Shetty S, Ghosh K, Pawar A, Abraham P. Hereditary thrombophilia as a cause of Budd-Chiari syndrome: a study from Western India. Hepatology. 2001;34(4):666–70.
  16. Kumar SI, Kumar A, Srivastava S, Saraswat VA, Aggarwal R. Low frequency of factor V Leiden and prothrombin G20210A mutations in patients with hepatic venous outflow tract obstruction in northern India: a case-control study. Indian Journal of Gastroenterology. 2005;24(5):211.
  17. Jain P, Rai RR, Nijhawan S, Singla D, Mathur A. BCS associated with portal vein and superior mesenteric vein thrombosis.J. Indian Association for study of the liver, Vellore 2007, Abstract no L44.
  18. Shukla A, Rathod K, Bapat MR, Goyal R, Deshmukh HL. Profile of hepatic venous outflow obstruction in Mumbai. Indian Association for study of the liver, Vellore 2007, Abstract no L43.
  19. De BK, Sen S, Biswas PK, Mandal SK, Das D, Das U, et al. Occurrence of hepatopulmonary syndrome in Budd–Chiari syndrome and the role of venous decompression. Gastroenterology. 2002 Apr 1;122(4):897–903.
  20. Bhattacharyya M, Makharia G, Kannan M, Ahmed RP, Gupta PK, Saxena R. Inherited Prothrombotic Defects in Budd-Chiari Syndrome and Portal Vein Thrombosis | American Journal of Clinical Pathology | Oxford Academic
  21. De BK, De KK, Sen S, Biswas PK, Das TK, Das S, et al. Etiology based prevalence of Budd-Chiari syndrome in eastern India. J Assoc Physicians India. 2000 Aug 1;48(8):800–3.
  22. Dayal S, Pati HP, Pande GK, Sharma MP, Saraya AK. Multilineage hempoietic stem cell defects in Budd Chiari syndrome. Journal of Hepatology. 1997 Feb 1;26(2):293–7.
  23. Aggarwal R, Ravishankar B, Misra R, Aggarwal A, Dwivedi S, Naik SR. Significance of elevated igg anticardiolipin antibody levels in patients with budd-chiari syndrome. The American Journal of Gastroenterology. 1998 Jun 1;93(6):954–7.
  24. DAS R, GAREWAL G, CHAWLA Y, DHIMAN R. Prevalence of the factor V Leiden mutation in portal and hepatic vein thrombosis. Gut. 1998 Jul;43(1):147.
  25. Sood A, Midha V, Sood N, Kaushal V. Hepatic vein thrombosis with ulcerative colitis. Indian J Gastroenterol. 2000 Sep;19(3):145–6.
  26. MOHANTY S, SAXENA R, Kumar AS. Activated Protein C Resistance in Budd-Chiari Syndrome. International journal of hematology. 2000 Aug;72(2):255.
  27. Das H, Punamiya S, Kalokhe S, Desai N, Amarapurkar D, Sawant P. Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt. The Journal of the Association of Physicians of India. 2003 Apr 1;51:309–10.
  28. Mahmoud AE, Mendoza A, Meshikhes AN, Olliff S, West R, Neuberger J, et al. Clinical spectrum, investigations and treatment of Budd-Chiari syndrome. QJM: monthly journal of the Association of Physicians. 1996;89(1):37–43.
  29. Hernández-Gea V, De Gottardi A, Leebeek FWG, Rautou P-E, Salem R, Garcia-Pagan JC. Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis. Journal of Hepatology. 2019 Jul 1;71(1):175–99.
  30. European Association for the Study of the Liver. Electronic address: This email address is being protected from spambots. You need JavaScript enabled to view it.. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016 Jan;64(1):179–202.
  31. Amarapurkar DN, Punamiya SJ, Patel ND. Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment. World journal of gastroenterology: WJG. 2008;14(2):278.
  32. Murad SD, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Annals of internal medicine. 2009;151(3):167–75.
  33. Seijo S, Plessier A, Hoekstra J, Dell’Era A, Mandair D, Rifai K, et al. Good long-term outcome of Budd-Chiari syndrome with a step-wise management. Hepatology. 2013;57(5):1962–8.
  34. Okuda K. Inferior vena cava thrombosis at its hepatic portion (obliterative hepatocavopathy). In: Seminars in liver disease. Copyright\copyright 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New ; 2002. p. 015–26.
  35. Valla D-C. Hepatic vein thrombosis (Budd-Chiari syndrome). In: Seminars in liver disease. Copyright\copyright 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New …; 2002. p. 005–14.
  36. Millener P, Grant EG, Rose S, Duerinckx A, Schiller VL, Tessler FN, et al. Color Doppler imaging findings in patients with Budd-Chiari syndrome: correlation with venographic findings. AJR American journal of roentgenology. 1993;161(2):307–12.
  37. Powell-Jackson PR, Melia W, Canalese J, Pickford RB, Portmann B, WILLIAMS R. Budd-Chiari syndrome: clinical patterns and therapy. QJM: An International Journal of Medicine. 1982;51(1):79–88.
  38. Tilanus HW. Budd-Chiari syndrome. Journal of British Surgery. 1995;82(8):1023–30.
  39. Shrestha SM. Hepatic venous outflow obstruction in Nepal. Tropical gastroenterology: official journal of the Digestive Diseases Foundation. 1996;17(3):165–71.
  40. Mitchell MC, Boitnott JK, Kaufman S, Cameron JL, Maddrey WC. Budd-Chiari syndrome: etiology, diagnosis and management. Medicine. 1982;61(4):199–218.
  41. Valla D, Le MG, Poynard T, Zucman N, Rueff B, Benhamou JP. Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. A case-control study. Gastroenterology. 1986 Apr;90(4):807–11.
  42. Pande GK. Hepatic venous outflow obstruction–where east meets west. Tropical gastroenterology: official journal of the Digestive Diseases Foundation. 1996;17(3):151–2.
  43. Tavill AS, Wood EJ, Kreel L, Jones EA, Gregory M, Sherlock S. The Budd-Chiari syndrome: correlation between hepatic scintigraphy and the clinical, radiological, and pathological findings in nineteen cases of hepatic venous outflow obstruction. Gastroenterology. 1975;68(3):509–18.
  44. Murad SD, Valla D-C, de Groen PC, Zeitoun G, Haagsma EB, Kuipers EJ, et al. Pathogenesis and Treatment of Budd-Chiari Syndrome Combined with Portal Vein Thrombosis. Official journal of the American College of Gastroenterology | ACG. 2006 Jan;101(1):83–90.
  45. Deutsch V, Rosenthal T, Adar R, Mozes M. Budd-chiari syndrome. American Journal of Roentgenology. 1972 Oct 1;116(2):430–9.
  46. Bolondi L, Gaiani S, Bassi SL, Zironi G, Bonino F, Brunetto M, et al. Diagnosis of Budd—Chiari Syndrome by Pulsed Doppler Ultrasound. Gastroenterology. 1991 May 1;100(5, Part 1):1324–31.
  47. Hosoki T, Kuroda C, Tokunaga K, Marukawa T, Masuike M, Kozuka T. Hepatic venous outflow obstruction: evaluation with pulsed duplex sonography. Radiology. 1989 Mar 1;170(3):733–7.
  48. Vogelzang RL, Anschuetz SL, Gore RM. Budd-Chiari syndrome: CT observations. Radiology. 1987;163(2):329–33.
  49. Stark DD, Hahn PF, Trey C, Clouse ME, Ferrucci Jr JT. MRI of the Budd-Chiari syndrome. American Journal of Roentgenology. 1986;146(6):1141–8.
  50. Bayraktar UD, Seren S, Bayraktar Y. Hepatic venous outflow obstruction: Three similar syndromes. World J Gastroenterol. 2007 Apr 7;13(13):1912–27.
  51. Min AD, Atillasoy EO, Schwartz ME, Thiim M, Miller CM, Bodenheimer HC. Reassessing the role of medical therapy in the management of hepatic vein thrombosis. Liver Transplantation and Surgery. 1997;3(4):423–9.
  52. Zeitoun G, Escolano S, Hadengue A, Azar N, El Younsi M, Mallet A, Boudet MJ, Hay JM, Erlinger S, Benhamou JP, Belghiti J, Valla D. Outcome of Budd‐Chiari syndrome: A multivariate analysis of factors related to survival including surgical portosystemic shunting - Zeitoun - 1999 - Hepatology - Wiley Online Library
  53. Menon KVN, Shah V, Kamath PS. The Budd–Chiari Syndrome. New England Journal of Medicine. 2004 Feb 5;350(6):578–85.
  54. Valla DC. Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West - VALLA - 2004 - Journal of Gastroenterology and Hepatology - Wiley Online Library [Internet].
  55. Bismuth H, Sherlock DJ. Portasystemic shunting versus liver transplantation for the Budd-Chiari syndrome. Ann Surg. 1991 Nov;214(5):581–9.
  56. Victor S, Jayanthi V, Kandasamy I, Ratnasabapathy A, Madanagopalan N. Retrohepatic cavoatrial bypass for coarctation of inferior vena cava with a polytetrafluoroethylene graft. The Journal of Thoracic and Cardiovascular Surgery. 1986 Jan 1;91(1):99–105.
  57. Victor S, Jayanthi V, Madanagopalan N, Dhala B, Krishnan null, Gajaraj A. Inflow occlusion technique for correction of coarctation of the inferior vena cava causing the Budd Chiari syndrome. Trop Gastroenterol. 1986 May;7(2):49–53.
  58. Shah SR, Narayanan TS, Nagral SS, Mathur SK. Surgical management of the Budd-Chiari syndrome: early experience. Indian J Gastroenterol. 1999 Apr 1;18(2):60–2.
  59. Klein AS, Molmenti EP. Surgical treatment of Budd-Chiari syndrome. Liver Transplantation. 2003 Sep 1;9(9):891–6.
  60. Zhang C-Q, Fu L-N, Xu L, Zhang G-Q, Jia T, Liu J-Y, et al. Long-term effect of stent placement in 115 patients with Budd-Chiari syndrome. World J Gastroenterol. 2003 Nov 15;9(11):2587–91.
  61. Pisani-Ceretti A, Intra M, Prestipino F, Ballarini C, Cordovana A, Santambrogio R, et al. Surgical and Radiologic Treatment of Primary Budd-Chiari Syndrome. World J Surg. 1998 Jan 1;22(1):48–54.
  62. Baijal SS, Roy S, Phadke RV, Agrawal DK, Kumar S, Choudhuri G. Management of Idiopathic Budd-Chiari Syndrome with Primary Stent Placement: Early Results. Journal of Vascular and Interventional Radiology. 1996 Jul 1;7(4):545–53.
  63. Gasparini D, Del Forno M, Sponza M, Branca B, Toniutto P, Marzio A, et al. Transjugular intrahepatic portosystemic shunt by direct transcaval approach in patients with acute and hyperacute Budd–Chiari syndrome. European Journal of Gastroenterology & Hepatology. 2002 May;14(5):567–71.
  64. Perelló A, Garcı́a-Pagán JC, Gilabert R, Suárez Y, Moitinho E, Cervantes F, et al. TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapy. Hepatology. 2002 Jan 1;35(1):132–9.
  65. Cejna M, Peck-Radosavljevic M, Schoder M, Thurnher S, Ba-Ssalamah A, Angermayr B, et al. Repeat Interventions for Maintenance of Transjugular Intrahepatic Portosystemic Shunt Function in Patients with Budd-Chiari Syndrome. Journal of Vascular and Interventional Radiology. 2002 Feb 1;13(2):193–9.
  66. Ochs A, Sellinger M, Haag K, Noldge G, Herbst EW, Walter E, Gerok W, Rossle M. Transjugular intrahepatic portosystemic stent-shunt (TIPS) in the treatment of Budd-Chiari syndrome - ScienceDirect [Internet]. [cited 2021 Aug 13]. Available from: https://www.sciencedirect.com/science/article/abs/pii/S0168827805802491
  67. Rogopoulos A, Gavelli A, Sakai H, McNamara M, Huguet C. Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome After Failure of Surgical Shunting. Archives of Surgery. 1995 Feb 1;130(2):227–8.
  68. Seki S, Sakaguchi H, Kobayashi S, Kitada T, Nakamura K, Yamada R. Transjugular intrahepatic portosystemic shunt in combination with oral anticoagulant for Budd-Chiari syndrome. Hepatogastroenterology. 2001 Sep 1;48(41):1447–9.
  69. Blum U, Rössle M, Haag K, Ochs A, Blum HE, Hauenstein KH, et al. Budd-Chiari syndrome: technical, hemodynamic, and clinical results of treatment with transjugular intrahepatic portosystemic shunt. Radiology. 1995 Dec 1;197(3):805–11.
  70. Molmenti EP, Segev DL, Arepally A, Hong J, Thuluvath PJ, Rai R, et al. The Utility of TIPS in the Management of Budd-Chiari Syndrome. Ann Surg. 2005 Jun;241(6):978–83.
  71. Kavanagh PM, Roberts J, Gibney R, Malone D, Hegarty J, Mccormick PA. Acute Budd–Chiari syndrome with liver failure: The experience of a policy of initial interventional radiological treatment using transjugular intrahepatic portosystemic shunt. Journal of Gastroenterology and Hepatology. 2004;19(10):1135–9.
  72. Franchis R de. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension. Journal of Hepatology. 2015 Sep 1;63(3):743–52.
  73. Garcia–Pagán JC, Heydtmann M, Raffa S, Plessier A, Murad S, Fabris F, et al. TIPS for Budd-Chiari Syndrome: Long-Term Results and Prognostics Factors in 124 Patients. Gastroenterology. 2008 Sep 1;135(3):808–15.
  74. Rössle M, Olschewski M, Siegerstetter V, Berger E, Kurz K, Grandt D. The Budd-Chiari syndrome: outcome after treatment with the transjugular intrahepatic portosystemic shunt. Surgery. 2004 Apr;135(4):394–403.

Corresponding Author

Dr Arnab Dey

DM Hepatology Postgraduate, Institute of Hepatobiliary Sciences, Madras Medical College, Chennai, Tamil Nadu