WBR0711

Author	PageAuthor::William J Gibson (Reviewed by Serge Korjian)
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Pathophysiology
Sub Category	SubCategory::Gastrointestinal, SubCategory::Hematology
Prompt	[[Prompt::A 74-year-old woman presents to the emergency department with severe upper abdominal pain for the past four hours. Past medical history is notable for hypertension, diabetes and polycythemia vera for which she receives hydroxyurea. The patient reports being non-compliant with her medications because of their many side effects. On physical examination, the liver can be palpated 5 cm below the costal margin and an abdominal fluid wave is present. What is the most likely diagnosis?]]
Answer A	AnswerA::Budd-Chiari syndrome
Answer A Explanation	AnswerAExp::Budd-Chiari syndrome is often caused by hepatic vein thrombosis. One of the most common causes of these thromboses is polycythemia vera.
Answer B	AnswerB::Choledocholithiasis
Answer B Explanation	[[AnswerBExp::Choledocholithiasis refers to the condition which occurs when gallstones have migrated into the biliary tract. This may lead to obstruction of the biliary tree and infection. Choledocholithiasis may present as acute upper abdominal pain, but it would not cause hepatomegaly and ascites.]]
Answer C	AnswerC::Nonalcoholic steatohepatitis
Answer C Explanation	[[AnswerCExp::Nonalcoholic steatohepatitis (NASH) is characterized by inflammation of the liver with concurrent fat accumulation. It may progress to cirrhosis, but it tends to progress silently over many years. A presentation this acute is not consistent with NASH.]]
Answer D	AnswerD::Pancreatitis
Answer D Explanation	AnswerDExp::Pancreatitis may present with upper abdominal pain radiating to the back, but it would not cause hepatomegaly or ascites.
Answer E	AnswerE::Splenic infarction
Answer E Explanation	AnswerEExp::While splenomegaly is associated with polycythemia vera and splenic infarction has been observed, the triad of abdominal pain, ascites and hepatomegaly is characteristic of Budd-Chiari syndrome.
Right Answer	RightAnswer::A
Explanation	[[Explanation::The patient in this vignette has Budd-Chiari syndrome secondary to polycythemia vera. Budd–Chiari syndrome is caused by occlusion of the hepatic veins leading to venous outflow obstruction. It presents with the classical triad of abdominal pain, ascites, and hepatomegaly. While most cases (75%) are due to thrombotic obstruction of the hepatic vein, any obstruction of the hepatic venous vasculature can cause a similar clinical picture. For example, cancers of the liver may obstruct hepatic venous blood flow. In all cases, the increased portal pressure causes increased filtration of vascular fluid and subsequent fluid leakage and ascites. In chronic, slow developing cases of Budd-Chiari, collateral hepatic venous circulations may develop that give the appearance of a "spider's web" on imaging. Collateral venous flow through alternative veins can eventually lead to esophageal, gastric and rectal varicose. Budd-chiari can be treated with surgical shunting of blood flow around the offending obstruction(s). Educational Objective: Budd-Chiari syndrome is caused by thrombosis of the hepatic vein and can present with hepatomegaly, abdominal pain, and ascites. References: First Aid 2014 page 362]]
Approved	Approved::Yes
Keyword	WBRKeyword::Budd-Chiari syndrome, WBRKeyword::Hepatic vein, WBRKeyword::Liver, WBRKeyword::Hepatomegaly, WBRKeyword::Abdominal pain, WBRKeyword::Ascites, WBRKeyword::Polycythemia, WBRKeyword::Polycythemia vera
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