A Man With Dyspnea and Hepatomegaly
Article Outline
- Answer to the Clinical Challenges and Images in GI Question: Image 1 (page 732): Polycystic Liver Disease Resulting in Compromised Cardiopulmonary Function
- References
- Copyright
Question: A 55-year-old man presented with progressive dyspnea on exertion for the past month. He denied any previous systemic disease. On physical examination, an engorged jugular vein without basal rales in bilateral lung and hepatomegaly was noted. Blood tests for cell count and biochemistry were normal. A chest x-ray showed extreme volume reduction in the right lung field (Figure A). Plain abdominal radiography revealed a huge mass in right upper abdomen with leftward shift of bowel gas pattern (Figure B). What is the most likely diagnosis?
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Answer to the Clinical Challenges and Images in GI Question: Image 1 (page 732): Polycystic Liver Disease Resulting in Compromised Cardiopulmonary Function
Abdominal computed tomography depicted multiple, variable-sized cysts in both lobes of the liver with the largest being 9.6 × 8.8 × 8.1 cm3 in the right lobe, which caused a mass effect in compressing of the right heart and volume reduction of the right lung (Figure C). A 2-dimensional echocardiogram further confirmed a small and compressed chamber of the right atrium by the giant cyst (Figure D). Mildly compromised cardiac function was also identified with estimated left/right ventricular ejection fraction of 48% and 46%, respectively. Severe restrictive pattern was also demonstrated on the pulmonary function test with 35% and 41% of predicted forced vital capacity and forced expiratory volume in 1 second. The dyspnea was soon relieved by ultrasonography-guided pigtail drainage of the giant cyst.
Polycystic liver disease (PLD) is usually asymptomatic. However, PLD can rarely cause hepatomegaly with compression to the adjacent organs, which induces symptoms such as pain, abdominal distention, and dyspnea. Cardiac compression has been rarely reported in patients with hepatic hydatid cyst and simple hepatic cysts associated with polycystic kidney disease.1, 2 In the present case, PLD concurrently compressed the right diaphragm and heart, and resulted in progressive dyspnea on exertion, which was confirmed by abdominal computed tomography, pulmonary function testing, and echocardiography.
Medical treatment of PLD has limited effect. Selective symptomatic patients with massive hepatomegaly from PLD may benefit from operative intervention.3 The type of operation performed mainly depends on the distribution of the cysts, coincident sectoral vascular patency, parenchymal preservation, and hepatic reserve. Hepatic resection with cyst fenestration was advised by our surgeons, but the patient refused.
References
- A case of cardiac compression by hepatic cyst in a woman with polycystic kidney disease. Intern Emerg Med. 2008;3:69–71
- Atrial flutter resulting from right atrial compression by a hepatic hydatid cyst. Int J Cardiol. 2000;73:87–89
- Polycystic liver disease: a critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann Surg. 2009;250:112–118
Conflicts of interest The authors disclose no conflicts.
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PII: S0016-5085(09)02199-4
doi:10.1053/j.gastro.2009.09.079
© 2010 AGA Institute. Published by Elsevier Inc. All rights reserved.
