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• W3016770947 abstract "Free AccessLetter To The EditorAgreed diagnostic criteria needed for Budd-Chiari syndromeAndrea MancusoAndrea MancusoMedicina Interna 1, ARNAS Civico - Di Cristina - Benfratelli, Piazzale Leotta 4, Palermo, ItalySearch for more papers by this authorPublished Online:17 Apr 2020https://doi.org/10.1259/bjr.20200259SectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail AboutI read with interest the systematic review and meta-analysis on diagnostic accuracy of Doppler ultrasound, CT and MRI in Budd Chiari syndrome (BCS), recently published in British Journal of Radiology and confirming that overall diagnostic accuracy for diagnosis of BCS is high for all.1 However, despite the commendable effort to define the background of an agreed diagnostic approach for BCS, overall the results add weak evidence to the issue, mostly because of both heterogeneity and low quality of the studies included. Moreover, the referred standard for diagnosis attributed to catheter venography and/or surgery appears debatable. In fact, both catheter venography and surgery are not able to rule out small hepatic veins BCS, a quite frequent condition at least in the West, and generally presenting with an unspecific mosaic enhancement pattern at CT and/or MRI, for whom liver histology is the only way to reach diagnosis.2,3Recent studies have emphasized the main differences of BCS in the West and the East.4,5 In fact, in the West BCS respectively has a low incidence, is mainly characterized by hepatic veins (HV) thrombosis, has an acutely progressive course and there is a relevant role of prothrombotic disorders (PD). Differently, in the East BCS respectively has a higher incidence, is mainly characterized by segmental or membranous inferior vena cava (IVC) obstruction with or without HVs occlusion, has a relatively chronic course, there is a less relevant role of PD. Finally, BCS respectively in the West and in the East, should probably be considered different diseases and, then, different therapeutic strategies seem fully justified.6–10Albeit there are not agreed diagnostic criteria for BCS both in the West and in the East, a pragmatic approach should consider Doppler ultrasound the first diagnostic step. However, BCS diagnosis at Doppler ultrasound is difficult, at least in not experienced settings and when the possibility of such diagnosis is not considered at preliminary clinical evaluation. Moreover, in real life, HV and or IVC involvement is not the main picture at Doppler ultrasound but evidence of intrahepatic collateral circulation, not a specific but a suggestive sign, according to diagnostic Doppler ultrasound criteria.6 The second diagnostic step should be CT or MRI. Finally, liver histology should be considered when CT and/or MRI are not diagnostic.Acknowledgment I declare I contributed to the paper.REFERENCES1. Gupta P, Bansal V, Kumar MP, Sinha SK, Samanta J, Mandavdhare H, et al..;IN PRESS Diagnostic accuracy of Doppler ultrasound, CT and MRI in Budd Chiari syndrome: systematic review and meta-analysis. Br J Radiol 2020. Google Scholar2. Mancuso A, Butera G, Politi F, Maringhini A. Budd Chiari syndrome presenting with hepatic transient mosaic enhancement pattern and treated with early tips. AME Med J 2017; 2: 60. Crossref, Google Scholar3. Ronot M, Kerbaol A, Rautou P-E, Brancatelli G, Bedossa P, Cazals-Hatem D, et al.. Acute extrahepatic infectious or inflammatory diseases are a cause of transient mosaic pattern on CT and MR imaging related to sinusoidal dilatation of the liver. Eur Radiol 2016; 26: 3094–101. doi: https://doi.org/10.1007/s00330-015-4124-2 http://www.ncbi.nlm.nih.gov/pubmed/26615556 Crossref Medline ISI, Google Scholar4. Mancuso A. Budd-Chiari syndrome in the West and the East: same syndrome, different diseases. Liver Int 2019; 39: 2417–9. doi: https://doi.org/10.1111/liv.14131 http://www.ncbi.nlm.nih.gov/pubmed/31066181 Crossref Medline ISI, Google Scholar5. Qi X, Han G, Guo X, De Stefano V, Xu K, Lu Z, et al.. Review article: the aetiology of primary Budd-Chiari syndrome - differences between the West and China. Aliment Pharmacol Ther 2016; 44(11-12): 1152–67. doi: https://doi.org/10.1111/apt.13815 http://www.ncbi.nlm.nih.gov/pubmed/27734511 Crossref Medline ISI, Google Scholar6. Mancuso A. Controversies in the Management of Budd–Chiari Syndrome. In: EditorBudd-Chiari Syndrome. Singapore: Xingshun Qi, Springer Nature; 2020. pp. 245–52. Crossref, Google Scholar7. Mancuso A. An update on the management of Budd-Chiari syndrome: the issues of timing and choice of treatment. Eur J Gastroenterol Hepatol 2015; 27: 200–3. doi: https://doi.org/10.1097/MEG.0000000000000282 http://www.ncbi.nlm.nih.gov/pubmed/25590783 Crossref Medline ISI, Google Scholar8. Mancuso A. Budd-Chiari syndrome hemodynamic: clinical setting more complicated than computational model. Med Eng Phys 2019; 71: 1. doi: https://doi.org/10.1016/j.medengphy.2019.06.021 http://www.ncbi.nlm.nih.gov/pubmed/31324448 Crossref Medline ISI, Google Scholar9. Mancuso A. Timing of TIPS in Budd Chiari Syndrome: An Italian Hepatologist’s Perspective. Journal of Translational Internal Medicine 2017; 5: 194–9. Crossref Medline ISI, Google Scholar10. Mancuso A. An update on management of Budd-Chiari syndrome. Ann Hepatol 2014; 13: 323–6. doi: https://doi.org/10.1016/S1665-2681(19)30860-9 http://www.ncbi.nlm.nih.gov/pubmed/24756006 Crossref Medline ISI, Google ScholarReply to the letter to the editor by Mancuso A—“Agreed diagnostic criteria needed for Budd-Chiari syndrome”1Pankaj Gupta and 1Varun Bansal1Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaDear Editor,We acknowledge the extensive expertise of Mancuso A in the field of Budd-Chiari syndrome (BCS). Regarding the critical comments on our study,1, we would like to make the following remarks.We acknowledge the limitations of the meta-analysis posed by the heterogeneity and low quality of the included studies. These limitations are inherent to the observational studies that were included in the meta-analysis and points towards the lack of convincing data regarding the imaging aspects of BCS. We also agree about the stepwise approach for imaging patients with suspected BCS.2 In this regard, Doppler represents a sensitive screening test. However, we recognize that the diagnosis requires an experienced operator.Moreover, there are technical challenges that may be difficult to overcome while performing Doppler evaluation of hepatic veins.3 We evaluated the role of contrast-enhanced ultrasound (CEUS) of the hepatic veins (HV) and inferior vena cava (IVC) as a complementary modality to Doppler in the initial evaluation of patients with BCS. This study (under peer review for publication) showed that a false-positive diagnosis of BCS is significantly reduced with the use of CEUS. CT is widely available and provides an excellent depiction of the HV, IVC, intrahepatic, and extrahepatic collateral pathways.3 Accurate technique is critical and must include a delayed phase. MRI provides a comprehensive evaluation; however, its availability is limited. Our meta-analysis does provide data regarding the comparative pooled diagnostic accuracy of Doppler, CT, and MRI that may guide physicians, particularly in centers whether all the modalities are available.Regarding the reference standard for diagnosis, we agree that catheter venography or surgery are less than ideal. During transjugular venography, although IVC may be adequately evaluated, evaluation of all the hepatic veins is challenging. Percutaneous venography of the individual HV is an alternative but is more invasive and may be risky in the setting of ascites. Additionally, there are rare instances of small vein BCS, a rare entity that involves smaller hepatic veins.4 The main hepatic veins are normal on Doppler and venography. This entity needs a histological diagnosis on the liver biopsy specimen. However, routine liver biopsy for diagnosis of BCS is not recommended.5REFERENCES1. Gupta P, Bansal V, Kumar-M P, Sinha SK, Samanta J, Mandavdhare H, et al.. Diagnostic accuracy of Doppler ultrasound, CT and MRI in Budd Chiari syndrome: systematic review and meta-analysis. Br J Radiol 2020; 20190847: 20190847. doi: https://doi.org/10.1259/bjr.20190847 http://www.ncbi.nlm.nih.gov/pubmed/32150462 Link ISI, Google Scholar2. Mancuso A. An update on management of Budd-Chiari syndrome. Ann Hepatol 2014; 13: 323–6. doi: https://doi.org/10.1016/S1665-2681(19)30860-9 http://www.ncbi.nlm.nih.gov/pubmed/24756006 Crossref Medline ISI, Google Scholar3. Bansal V, Gupta P, Sinha S, Dhaka N, Kalra N, Vijayvergiya R, et al.. Budd-Chiari syndrome: imaging review. Br J Radiol 2018; 91: 20180441. doi: https://doi.org/10.1259/bjr.20180441 http://www.ncbi.nlm.nih.gov/pubmed/30004805 Link ISI, Google Scholar4. Riggio O, Marzano C, Papa A, Pasquale C, Gasperini ML, Gigante A, et al.. Small hepatic veins Budd-Chiari syndrome. J Thromb Thrombolysis 2014; 37: 536–9. doi: https://doi.org/10.1007/s11239-013-0959-z http://www.ncbi.nlm.nih.gov/pubmed/23813023 Crossref Medline ISI, Google Scholar5. European Association for the Study of the Liver. Electronic address: [email protected]. EASL clinical practice guidelines: vascular diseases of the liver. J Hepatol 2016; 64: 179–202. doi: https://doi.org/10.1016/j.jhep.2015.07.040 http://www.ncbi.nlm.nih.gov/pubmed/26516032 Crossref Medline ISI, Google Scholar Previous article FiguresReferencesRelatedDetailsCited byBudd–Chiari Syndrome Management: Controversies and Open Issues3 November 2022 | Diagnostics, Vol. 12, No. 11Focusing the Controversies in Budd-Chiari Syndrome ManagementThe International Journal of Gastroenterology and Hepatology Diseases, Vol. 1, No. 1 Volume 93, Issue 1110June 2020 © 2020 The Authors. Published by the British Institute of Radiology History ReceivedMarch 17,2020AcceptedApril 01,2020Published onlineApril 17,2020 Metrics Download PDF" @default.
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