A 36-year-old woman comes to the emergency department with abdominal pain and distention that started 5 days earlier and has become progressively worse. She initially thought she was experiencing bloating but the symptoms persisted despite poor appetite. The patient has a history of migraines. She underwent ovarian cyst resection 5 years ago. Her medications include over-the-counter analgesics, oral contraceptive pills, and daily multivitamins. She does not use tobacco, alcohol, or illicit drugs.
Her temperature is 37.2° C (99° F) , blood pressure is 110/70 mm Hg, and pulse is 98/min. BMI is 25 kg/m². The estimated jugular venous pressure is 7 mm H₂O. Lungs are clear to auscultation. No murmurs are heard. The abdomen is distended and there is positive fluid shift test. The liver edge is tender and is palpated 4 cm below the right costal margin. The spleen is not palpable. There is trace lower-extremity edema.
Liver function test results are as follows:

Ultrasound-guided paracentesis yields 1.5 L of clear fluid. Fluid analysis shows a white cell count of 120/mm³ and albumin of 2.4 g/L. Cytology is pending.
Which of the following is the most likely diagnosis?

Question

A 36-year-old woman comes to the emergency department with abdominal pain and distention that started 5 days earlier and has become progressively worse. She initially thought she was experiencing bloating but the symptoms persisted despite poor appetite. The patient has a history of migraines. She underwent ovarian cyst resection 5 years ago. Her medications include over-the-counter analgesics, oral contraceptive pills, and daily multivitamins. She does not use tobacco, alcohol, or illicit drugs.
Her temperature is 37.2° C (99° F) , blood pressure is 110/70 mm Hg, and pulse is 98/min. BMI is 25 kg/m². The estimated jugular venous pressure is 7 mm H₂O. Lungs are clear to auscultation. No murmurs are heard. The abdomen is distended and there is positive fluid shift test. The liver edge is tender and is palpated 4 cm below the right costal margin. The spleen is not palpable. There is trace lower-extremity edema.
Liver function test results are as follows:

Ultrasound-guided paracentesis yields 1.5 L of clear fluid. Fluid analysis shows a white cell count of 120/mm³ and albumin of 2.4 g/L. Cytology is pending.
Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_960e_c292_853d_058e2d296708_MD0002_00 This patient's clinical presentation suggests Budd-Chiari syndrome. Budd-Chiari syndrome is due to thrombosis of the hepatic/intrahepatic veins or suprahepatic inferior vena cava and occurs most commonly in women in their 30s and 40s. The condition may be idiopathic or due to etiologies such as malignancy (eg, hepatocellular carcinoma is most common), myeloproliferative diseases, oral contraceptive use, pregnancy, or hypercoagulable states. Acute Budd-Chiari syndrome (20% of patients) presents with severe right upper quadrant abdominal pain, hepatomegaly, jaundice, ascites, and possible variceal bleeding. Serum aminotransferases are usually elevated, but not as high as in acute viral hepatitis (Choice B). Untreated disease may progress to encephalopathy and fulminant liver failure. Subacute and chronic Budd-Chiari syndrome may be asymptomatic for weeks to several months due to partial hepatic vein occlusion. Chronic hepatic vein occlusion may lead to hypertrophy of the caudate lobe of the liver, which eventually compresses the infrahepatic inferior vena cava to cause ascites and lower-extremity edema. Patients eventually develop cirrhosis, portal hypertension, and normal to mildly elevated liver function studies. However, jaundice and encephalopathy are less common. Diagnosis can be made by Doppler ultrasound with flow, CT scan, or magnetic resonance angiography showing occlusion or poorly visualized flow in the hepatic veins. Venography (gold standard) is suggested if these tests are nondiagnostic and may show venous occlusion with a "spider-web" pattern of hepatic collaterals. Ascites fluid analysis usually shows a high serum to ascites albumin gradient (SAAG) > 1.1 g/dL, indicating portal hypertension. Liver biopsy is reserved for patients with inconclusive initial investigations. CT scan Treatment involves diuretics, anticoagulation, angioplasty with stenting for select patients, and possible thrombolytics (only in acute/subacute disease). Patients not responding to these measures may require transjugular intrahepatic portosystemic shunt, surgical shunts, or liver transplant. (Choice A) In acute acetaminophen overdose, serum aminotransferase levels are markedly elevated (> 3000 IU/L) with more rapid progression of hepatic injury. This patient's clinical presentation of abdominal distention and pain for 5 days, as well as low levels of aminotransferase elevation, are less consistent with acetaminophen overdose. (Choice D) Peritoneal carcinomatosis usually presents with large weight loss, shortness of breath, and abdominal pain. Ascites often develops due to large tumor burden; fluid studies without liver involvement often show SAAG < 1.1 g/dL. (Choice E) Primary biliary cirrhosis usually presents with fatigue, pruritus, markedly elevated alkaline phosphatase, normal to slightly elevated serum aminotransferases, and possible hyperpigmented skin. Educational objective: Budd-Chiari syndrome (thrombosis of hepatic veins) should be suspected in young women who present with rapid onset of abdominal pain and ascites. Diagnosis is made by ultrasound with Doppler flow, computed tomography scan, or magnetic resonance angiography. Treatment includes anticoagulation, treatment of liver complications, angioplasty, transjugular intrahepatic portosystemic shunts, or liver transplant. __________ References: An update on the diagnosis and management of Budd-Chiari syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/23237258) Budd-Chiari syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/22997363)

A 36-Year-Old Woman Comes to the Emergency Department with Abdominal