2/15/17 Resident Morning Report – Budd-Chiari Syndrome

CC: progressive abdominal distension

ID: 70 y/o male with PMH hypertension and hyperlipidemia, previously in excellent health who presents with progressive abdominal distention that started 3 months prior. He additionally notes that his urine was significantly darker with associated nausea/vomiting, anorexia and generalized weakness/fatigue. Physical exam was significant for a fluid wave and shifting dullness; paracentesis revealed a total protein of 2.5 g/dL and high SAAG. Abdominal US showed thrombosed right and middle hepatic veins and an attenuated left hepatic vein, consistent with Budd-Chiari syndrome.

Causes of Portal Hypertension 

Prehepatic

  • Portal vein thrombosis
  • Splenic vein thrombosis
  • Massive splenomegaly

Intrahepatic

  • Schistosomiasis
  • Congenital hepatic fibrosis
  • Cirrhosis
  • Alcoholic hepatitis
  • Venoocclusive syndrome

Posthepatic

  • Constrictive pericarditis
  • Budd-Chiari Syndrome
  • Right-sided heart failure
  • Severe tricuspid regurgitation
  • IVC obstruction

Ascitic Fluid Analysis

 presentation4


Pearls from morning report:

  • Patients with a thrombus in the hepatic vein, portal vein, mesenteric vein or cerebral vein deserve a hypercoagulable work up, including testing for antiphospholipid syndrome, polycythemia vera (PV) and paroxysmal nocturnal hemoglobinuria (PNH).
  • If a patient is diagnosed with Budd-Chiari syndrome, look for any underlying cause or associated conditions such as PV, PNH, ulcerative colitis, Behcet’s disease, oral contraceptive use, or masses causing compression on the hepatic vein (HCC, aneurysms, abscesses, etc.).

Random trivia:

Budd-Chiari is named after British physician Dr. George Budd and Austrian physician Dr. Hans Chiari, who has several other eponyms to his name (Arnold-Chiari malformation, Chiari network).


References: 

MKSAP 17: Medical Knowledge Self-assessment Program. Philadelphia : American College Of Physicians, 2016. Print.

Menon KV, Shah V, Kamath PS. The Budd Chiari Syndrome. N Engl J Med 2004; 350:578-585

Bacon, Bruce R.. “Cirrhosis and Its Complications.” Harrison’s Principles of Internal Medicine, 19e Eds. Dennis Kasper, et al. New York, NY: McGraw-Hill, 2014, http://accessmedicine.mhmedical.com/content.aspx?bookid=1130&sectionid=79748841.

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