Budd-Chiari syndrome differential diagnosis: Difference between revisions

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==Overview==
==Overview==
==Differential diagnosis==
==Differential diagnosis==
Budd-Chiari syndrome should be differentiated from other causes of [[hepatic failure]] like [[viral hepatitis]] and [[liver cirrhosis]] that may have similar presentation with right upper quadrant abdominal pain and ascites. The differential diagnosis is as follows:
Budd-Chiari syndrome should be differentiated from other causes of [[hepatic failure]] like [[viral hepatitis]] and [[liver cirrhosis]], [[splenic vein thrombosis]], [[portal vein thrombosis]] and [[inferior vena cava obstruction]] that may have similar presentation with right upper quadrant abdominal pain and [[ascites]]. The differential diagnosis is as follows:
 


{| class="wikitable"
{| class="wikitable"
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! style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating signs and symptoms
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating signs and symptoms
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating Tests
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Differentiating Tests
|-
| style="background:#DCDCDC; + " | '''Budd-Chiari Syndrome'''
|
* [[Abdominal pain]]
* [[Diarrhea]]
* [[Ascites]]
|
* [[Doppler ultrasound]] and [[CT]] of the [[abdomen]] shows absence of the [[hepatic vein]] filling
* [[Abdominal]] [[Computed tomography|CT scan]] will show a rapid clearing of the [[Caudate lobe of liver|caudate lobe]] of the [[liver]]
|-
|-
| style="background:#DCDCDC; + " |'''Cirrhosis'''
| style="background:#DCDCDC; + " |'''Cirrhosis'''
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*Findings on transient [[elastography]] may show reasonable correlation with the severity of cirrhosis:<ref>{{cite journal |author=Foucher J, Chanteloup E, Vergniol J, ''et al'' |title=Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study |journal=Gut |volume=55|issue=3 |pages=403-8 |year=2006 |pmid=16020491 |doi=10.1136/gut.2005.069153}}</ref><ref name="pmid22733854">{{cite journal |author=Xie L, Chen X, Guo Q, Dong Y, Guang Y, Zhang X |title=Real-time elastography for diagnosis of liver fibrosis in chronic hepatitis B |journal=[[Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine]] |volume=31 |issue=7 |pages=1053–60 |year=2012|pmid=22733854 |doi= |url=}}</ref>
*Findings on transient [[elastography]] may show reasonable correlation with the severity of cirrhosis:<ref>{{cite journal |author=Foucher J, Chanteloup E, Vergniol J, ''et al'' |title=Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study |journal=Gut |volume=55|issue=3 |pages=403-8 |year=2006 |pmid=16020491 |doi=10.1136/gut.2005.069153}}</ref><ref name="pmid22733854">{{cite journal |author=Xie L, Chen X, Guo Q, Dong Y, Guang Y, Zhang X |title=Real-time elastography for diagnosis of liver fibrosis in chronic hepatitis B |journal=[[Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine]] |volume=31 |issue=7 |pages=1053–60 |year=2012|pmid=22733854 |doi= |url=}}</ref>
**Increasing [[Scar|scarring]] of the [[liver]] is associated with increasing "stiffness" of the [[Tissue (biology)|tissue]].
**Increasing [[Scar|scarring]] of the [[liver]] is associated with increasing "stiffness" of the [[Tissue (biology)|tissue]].
|-
| style="background:#DCDCDC; + " | '''Constrictive pericarditis'''
|
* Increased [[jugular venous pressure]]
* [[Atrial fibrillation]]
* [[Tachycardia]]
* Faint [[heart sounds]] with a [[third heart sound]] ([[ventricular]] knock)
|
* [[EKG]] will show:
** [[Tachycardia]]
** [[Atrial fibrillation]]
** Low-voltage [[QRS complexes]]
** [[T wave]] abnormalities
* [[Doppler ultrasound]] shows [[Ventricle (heart)|ventricular]] filling abnormalities
|-
| style="background:#DCDCDC; + " | '''Budd-Chiari Syndrome'''
|
* [[Abdominal pain]]
* [[Diarrhea]]
* [[Ascites]]
|
* [[Doppler ultrasound]] and [[CT]] of the [[abdomen]] shows absence of the [[hepatic vein]] filling
* [[Abdominal]] [[Computed tomography|CT scan]] will show a rapid clearing of the [[Caudate lobe of liver|caudate lobe]] of the [[liver]]
|-
|-
| style="background:#DCDCDC; + " | '''Splenic vein thrombosis'''
| style="background:#DCDCDC; + " | '''Splenic vein thrombosis'''
Line 142: Line 127:
|
|
* MR or direct [[Angiogram|angiography]] shows a normal [[Hepatic vein|hepatic venous]] pressure gradient
* MR or direct [[Angiogram|angiography]] shows a normal [[Hepatic vein|hepatic venous]] pressure gradient
|-
| style="background:#DCDCDC; + " | '''Sarcoidosis'''
|
* [[Dry cough]] with [[dyspnea]]
* Anterior or posterior [[uveitis]]
* [[Dry eyes]]
* [[glaucoma]]
* [[Skin]] findings may include:
** [[Maculopapular]] lesions on the [[face]], [[back]], arms and [[legs]]
** [[Erythema nodosum]] on the legs
|
* Chest x ray may show:
** [[Hilar lymphadenopathy]]
** Upper lobe [[fibrosis]]
** Diffuse reticulonodular shadowing
** [[Liver biopsy]] shows non-[[necrotizing]], non-caseating [[Granuloma|granulomas]]
|-
|-
| style="background:#DCDCDC; + " | '''Inferior vena cava obstruction'''
| style="background:#DCDCDC; + " | '''Inferior vena cava obstruction'''
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|
|
* [[Liver biopsy]] shows no evidence of cirrhosis
* [[Liver biopsy]] shows no evidence of cirrhosis
|-
| style="background:#DCDCDC; + " | '''Vitamin A intoxication, arsenic, and vinyl chloride toxicity'''
|None
|
* History generally reveals exposure
|}
|}


Revision as of 19:51, 5 January 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2] Amandeep Singh M.D.[3]

Overview

Differential diagnosis

Budd-Chiari syndrome should be differentiated from other causes of hepatic failure like viral hepatitis and liver cirrhosis, splenic vein thrombosis, portal vein thrombosis and inferior vena cava obstruction that may have similar presentation with right upper quadrant abdominal pain and ascites. The differential diagnosis is as follows:

Condition Differentiating signs and symptoms Differentiating Tests
Budd-Chiari Syndrome
Cirrhosis

Ultrasound findings in cirrhosis are as follows:[1][2][3][4][5][6][7][8]

Abdominal MRI may also be helpful in the diagnosis of portal hypertension. Findings on MRI suggestive of cirrhosis with portal hypertension include:[9][10][11][12]

Transient elastography and the Acoustic Radiation Force Impulse (ARFI) technique are well-established methods for the staging of fibrosis in various liver diseases: [13][14][15][16][17][18][19][20][21][22][23] 

  • The FibroScan (transient elastography) uses elastic waves to determine liver stiffness which theoretically may be converted into a liver score.
  • The FibroScan produces an ultrasound image of the liver (from 20-80mm) along with a pressure reading (in kPa).
  • Transient elastography is much faster than a biopsy (usually lasts 2.5-5 minutes) and is completely painless.
  • Findings on transient elastography may show reasonable correlation with the severity of cirrhosis:[24][25]
Splenic vein thrombosis Signs and symptoms of:
Portal vein thrombosis
Schistosomiasis
Inferior vena cava obstruction
Nodular regenerative hyperplasia None
Idiopathic portal hypertension (hepatoportal sclerosis) None

References

  1. ↑ Udell JA, Wang CS, Tinmouth J, FitzGerald JM, Ayas NT, Simel DL, Schulzer M, Mak E, Yoshida EM (2012). "Does this patient with liver disease have cirrhosis?". JAMA. 307 (8): 832–42. doi:10.1001/jama.2012.186. PMID 22357834.
  2. ↑ Becker CD, Scheidegger J, Marincek B (1986). "Hepatic vein occlusion: morphologic features on computed tomography and ultrasonography". Gastrointest Radiol. 11 (4): 305–11. PMID 3533689.
  3. ↑ Di Lelio A, Cestari C, Lomazzi A, Beretta L (1989). "Cirrhosis: diagnosis with sonographic study of the liver surface". Radiology. 172 (2): 389–92. doi:10.1148/radiology.172.2.2526349. PMID 2526349.
  4. ↑ Sanford NL, Walsh P, Matis C, Baddeley H, Powell LW (1985). "Is ultrasonography useful in the assessment of diffuse parenchymal liver disease?". Gastroenterology. 89 (1): 186–91. PMID 3891495.
  5. ↑ Giorgio A, Amoroso P, Lettieri G, Fico P, de Stefano G, Finelli L, Scala V, Tarantino L, Pierri P, Pesce G (1986). "Cirrhosis: value of caudate to right lobe ratio in diagnosis with US". Radiology. 161 (2): 443–5. doi:10.1148/radiology.161.2.3532188. PMID 3532188.
  6. ↑ Simonovský V (1999). "The diagnosis of cirrhosis by high resolution ultrasound of the liver surface". Br J Radiol. 72 (853): 29–34. doi:10.1259/bjr.72.853.10341686. PMID 10341686.
  7. ↑ Trinchet JC, Chaffaut C, Bourcier V, Degos F, Henrion J, Fontaine H, Roulot D, Mallat A, Hillaire S, Cales P, Ollivier I, Vinel JP, Mathurin P, Bronowicki JP, Vilgrain V, N'Kontchou G, Beaugrand M, Chevret S (2011). "Ultrasonographic surveillance of hepatocellular carcinoma in cirrhosis: a randomized trial comparing 3- and 6-month periodicities". Hepatology. 54 (6): 1987–97. doi:10.1002/hep.24545. PMID 22144108.
  8. ↑ "EASL-EORTC clinical practice guidelines: management of hepatocellular carcinoma". J. Hepatol. 56 (4): 908–43. 2012. doi:10.1016/j.jhep.2011.12.001. PMID 22424438.
  9. ↑ Procopet, Bogdan; Berzigotti, Annalisa (2017). "Diagnosis of cirrhosis and portal hypertension: imaging, non-invasive markers of fibrosis and liver biopsy". Gastroenterology Report. 5 (2): 79–89. doi:10.1093/gastro/gox012. ISSN 2052-0034.
  10. ↑ Aagaard, J; Jensen, LI; Sorensen, TI; Christensen, U; Burcharth, F (1982). "Recanalized umbilical vein in portal hypertension". American Journal of Roentgenology. 139 (6): 1107–1110. doi:10.2214/ajr.139.6.1107. ISSN 0361-803X.
  11. ↑ Cho, K C; Patel, Y D; Wachsberg, R H; Seeff, J (1995). "Varices in portal hypertension: evaluation with CT". RadioGraphics. 15 (3): 609–622. doi:10.1148/radiographics.15.3.7624566. ISSN 0271-5333.
  12. ↑ Bandali, Murad Feroz; Mirakhur, Anirudh; Lee, Edward Wolfgang; Ferris, Mollie Clarke; Sadler, David James; Gray, Robin Ritchie; Wong, Jason Kam (2017). "Portal hypertension: Imaging of portosystemic collateral pathways and associated image-guided therapy". World Journal of Gastroenterology. 23 (10): 1735. doi:10.3748/wjg.v23.i10.1735. ISSN 1007-9327.
  13. ↑ Castera L, Pinzani M (2010). "Biopsy and non-invasive methods for the diagnosis of liver fibrosis: does it take two to tango?". Gut. 59 (7): 861–6. doi:10.1136/gut.2010.214650. PMID 20581229.
  14. ↑ Friedrich-Rust M, Nierhoff J, Lupsor M, Sporea I, Fierbinteanu-Braticevici C, Strobel D, Takahashi H, Yoneda M, Suda T, Zeuzem S, Herrmann E (2012). "Performance of Acoustic Radiation Force Impulse imaging for the staging of liver fibrosis: a pooled meta-analysis". J. Viral Hepat. 19 (2): e212–9. doi:10.1111/j.1365-2893.2011.01537.x. PMID 22239521.
  15. ↑ Friedrich-Rust M, Ong MF, Martens S, Sarrazin C, Bojunga J, Zeuzem S, Herrmann E (2008). "Performance of transient elastography for the staging of liver fibrosis: a meta-analysis". Gastroenterology. 134 (4): 960–74. doi:10.1053/j.gastro.2008.01.034. PMID 18395077.
  16. ↑ Ziol M, Handra-Luca A, Kettaneh A, Christidis C, Mal F, Kazemi F, de Lédinghen V, Marcellin P, Dhumeaux D, Trinchet JC, Beaugrand M (2005). "Noninvasive assessment of liver fibrosis by measurement of stiffness in patients with chronic hepatitis C". Hepatology. 41 (1): 48–54. doi:10.1002/hep.20506. PMID 15690481.
  17. ↑ Sandrin L, Fourquet B, Hasquenoph JM, Yon S, Fournier C, Mal F, Christidis C, Ziol M, Poulet B, Kazemi F, Beaugrand M, Palau R (2003). "Transient elastography: a new noninvasive method for assessment of hepatic fibrosis". Ultrasound Med Biol. 29 (12): 1705–13. PMID 14698338.
  18. ↑ Bamber J, Cosgrove D, Dietrich CF, Fromageau J, Bojunga J, Calliada F, Cantisani V, Correas JM, D'Onofrio M, Drakonaki EE, Fink M, Friedrich-Rust M, Gilja OH, Havre RF, Jenssen C, Klauser AS, Ohlinger R, Saftoiu A, Schaefer F, Sporea I, Piscaglia F (2013). "EFSUMB guidelines and recommendations on the clinical use of ultrasound elastography. Part 1: Basic principles and technology". Ultraschall Med. 34 (2): 169–84. doi:10.1055/s-0033-1335205. PMID 23558397.
  19. ↑ "EASL-ALEH Clinical Practice Guidelines: Non-invasive tests for evaluation of liver disease severity and prognosis". J. Hepatol. 63 (1): 237–64. 2015. doi:10.1016/j.jhep.2015.04.006. PMID 25911335.
  20. ↑ Castera L, Bedossa P (2011). "How to assess liver fibrosis in chronic hepatitis C: serum markers or transient elastography vs. liver biopsy?". Liver Int. 31 Suppl 1: 13–7. doi:10.1111/j.1478-3231.2010.02380.x. PMID 21205132.
  21. ↑ Chou R, Wasson N (2013). "Blood tests to diagnose fibrosis or cirrhosis in patients with chronic hepatitis C virus infection: a systematic review". Ann. Intern. Med. 158 (11): 807–20. doi:10.7326/0003-4819-158-11-201306040-00005. PMID 23732714.
  22. ↑ Khallafi H, Qureshi K (2015). "Imaging Based Methods of Liver Fibrosis Assessment in Viral Hepatitis: A Practical Approach". Interdiscip Perspect Infect Dis. 2015: 809289. doi:10.1155/2015/809289. PMC 4686715. PMID 26779260.
  23. ↑ Singh S, Fujii LL, Murad MH, Wang Z, Asrani SK, Ehman RL, Kamath PS, Talwalkar JA (2013). "Liver stiffness is associated with risk of decompensation, liver cancer, and death in patients with chronic liver diseases: a systematic review and meta-analysis". Clin. Gastroenterol. Hepatol. 11 (12): 1573–84.e1–2, quiz e88–9. doi:10.1016/j.cgh.2013.07.034. PMC 3900882. PMID 23954643.
  24. ↑ Foucher J, Chanteloup E, Vergniol J; et al. (2006). "Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study". Gut. 55 (3): 403–8. doi:10.1136/gut.2005.069153. PMID 16020491.
  25. ↑ Xie L, Chen X, Guo Q, Dong Y, Guang Y, Zhang X (2012). "Real-time elastography for diagnosis of liver fibrosis in chronic hepatitis B". Journal of Ultrasound in Medicine : Official Journal of the American Institute of Ultrasound in Medicine. 31 (7): 1053–60. PMID 22733854.

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