title: Liver Transplantation for Budd-Chiari Syndrome With Large Solitary Focal Nodular Hyperplasia of the Liver in a Patient With Essential Thrombocythemia: Case Report. [Corrected].
abstract: Budd-Chiari syndrome is a rare condition caused by interrupted hepatic venous outflow in the hepatic veins, inferior vena cava, or right atrium. Reports from the literature have delineated on focal nodular hyperplasia (FNH)-like lesions in association with Budd-Chiari Syndrome. To our knowledge, there are no reports about true FNH lesions in patients with Budd-Chiari Syndrome. Focal nodular hyperplasia develops in disorders with aberrant circulation and vasculature. We report a case of Budd-Chiari syndrome in association with large solitary FNH in a 22-year-old man who was referred to our institution with sudden intermittent right upper quadrant abdominal pain, vomiting, diarrhea with pale stool, decreased appetite, dark urine, and abdominal distention for 15 days. Laboratory investigations revealed anemia, thrombocytosis, and abnormal liver function tests and coagulation profile. Imaging revealed hepatic vein thrombosis, confirming Budd-Chiari syndrome, and a 6.2 × 6.1 × 6.8 cm lesion in segment 8 of the liver. Primary cause of Budd-Chiari syndrome was essential thrombocythemia according to bone marrow biopsy and molecular testing results. The patient was treated medically and underwent transjugular intrahepatic portosystemic shunt insertion. The lesion in segment 8 continued to enlarge. Cadaveric liver transplantation was carried out. On gross and histologic examination of the explanted liver, the lesion was found to be a true FNH.
authors.affiliation: College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
authors.forename: A
authors.initials: A
authors.lastname: Alnajjar
authors.affiliation: Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors.forename: H
authors.initials: H
authors.lastname: Al-Hussaini
authors.affiliation: Department of Liver and Small Bowel Transplantation and Hepatobiliary and Pancreatic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors.forename: M
authors.initials: M
authors.lastname: Al Sebayel
authors.affiliation: College of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
authors.forename: W
authors.initials: W
authors.lastname: Al-Kattan
authors.affiliation: Department of Liver and Small Bowel Transplantation and Hepatobiliary and Pancreatic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. Electronic address: helsiesy@gmail.com.
authors.forename: H
authors.initials: H
authors.lastname: Elsiesy
mesh_terms: D001706:Biopsy / Q000009:adverse effects; D006502:Budd-Chiari Syndrome / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D020518:Focal Nodular Hyperplasia / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D013921:Thrombocytopenia / Q000209:etiology / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D055815:Young Adult