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Budd-Chiari syndrome associated with congenital afibrinogenaemia reversed after orthotopic liver transplant
  1. Vinayak G Nikam1,
  2. Vijay Waman Dhakre2,
  3. Kanchan Motwani1 and
  4. Somnath Chattopadhyay1
  1. 1HPB and Liver Transplant Surgery, Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra, India
  2. 2Department of General Surgery, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India
  1. Correspondence to Dr Vijay Waman Dhakre; vddrvijayd{at}gmail.com

Abstract

A woman in her mid-20s, a known case of congenital afibrinogenaemia, presented with abdominal pain and distension. She was diagnosed with decompensated liver cirrhosis due to Budd-Chiari syndrome. She underwent deceased donor liver transplantation. Preoperatively, her serum fibrinogen level was undetectable and prothrombin time and international normalised ratio (INR) were unrecordable. Intraoperatively, she was given thromboelastography-guided human fibrinogen concentrate. Postoperatively, her fibrinogen, prothrombin time and INR normalised rapidly. This report summarises the rare occurrence of a complication of hypercoagulability (Budd-Chiari syndrome) in the setting of congenital hypocoagulability (congenital afibrinogenaemia). In this report, we discuss the simultaneous management of these two clinical problems and the curative role of liver transplantation.

  • Transplantation
  • Thrombosis

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Footnotes

  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: VWD, VGN, KM, SC. The following authors gave final approval of the manuscript: VWD, VGN, KM, SC.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.