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The differential diagnosis of mediastinal masses visible on plain chest radiography does not usually include oesophageal varices.
A man aged 28 with congenital hepatic fibrosis and secondary biliary cirrhosis was on the waiting list for liver transplantation. In the past, oesophageal varices had been treated endoscopically on many occasions by band ligation. On the present occasion he was admitted from the emergency department having become short of breath and nauseated, with abdominal pain and increasing confusion. He was deeply jaundiced (bilirubin 594 μmol/L) and had gross ascites; Glasgow Coma Scale score was 8. A chest radiograph showed a left pleural effusion with atelectasis of the basal segments of the underlying left lower lobe; but in addition there was a retrocardiac density with a smooth left lateral border and no fluid level (Figure 1). Comparison with a previous CT image indicated that this mediastinal abnormality consisted of oesophageal varices (Figure 2). The patient was treated for hepatic encephalopathy with oral L-ornithine L-aspartate and lactulose. His mental state improved rapidly and he was discharged after five days to resume his wait for a liver transplant.
Congenital hepatic fibrosis is characterized on liver biopsy by ductal plate malformations and the persistence of excess embryonic bile duct structures in the portal tracts.1 The condition may be associated with gross bile duct abnormalities, including cystic dilatation in the intrahepatic biliary tree (Caroli’s syndrome), and also renal tract disease, such as medullary sponge kidney.1 If patients survive to later life, congenital hepatic fibrosis is usually associated with a secondary biliary cirrhosis of early onset,2 as in our patient, who was diagnosed by liver biopsy in his late teenage years after sudden upper gastrointestinal haemorrhage had led to identification of oesophageal varices. Although extensive varices are common complications of chronic liver disease of any cause, it is very unusual for a density seen through the cardiac silhouette on a plain chest radiograph to be due to varices.3 In a patient with cirrhosis who has a mediastinal mass, the possibility of oesophageal varices needs to be borne in mind.