Chronic liver disease, cirrhosis and portal hypertension: Case

This boy presents the complex picture of decompensated chronic liver disease with portal hypertension and probable hepatopulmonary syndrome. The history of biliary atresia with a Kasai portoenterostomy marks him as a child at high risk for progressive fibrosis, and his current findings indicate that the disease has advanced significantly. The growth faltering, splenomegaly with thrombocytopenia, hypoalbuminaemia, coagulopathy, and ascites together paint the picture of decompensated cirrhosis, while the digital clubbing, cyanosis, exertional dyspnoea, and orthodeoxia strongly suggest hepatopulmonary syndrome. ^[1]

Clinical findings

The clinical picture is that of advanced portal hypertension with decompensated cirrhosis and a pulmonary complication. The markedly enlarged spleen with a platelet count of 62,000 per cubic millimetre indicates clinically significant portal hypertension with hypersplenism. The hypoalbuminaemia and INR of 1.7 reflect impaired hepatic synthetic function, and the ascites with shifting dullness confirms decompensation. The growth faltering from the 25th to the 3rd centile is a major concern and itself worsens the PELD score and transplant priority. ^[1]

The respiratory findings demand attention. The digital clubbing and central cyanosis are physical signs of chronic hypoxaemia, and the oxygen saturation of 88 per cent on room air falling to 82 per cent on standing is the classic orthodeoxia of hepatopulmonary syndrome. The exertional dyspnoea described by the mother is platypnoea in its clinical manifestation. Together these findings fulfil two of the three criteria for hepatopulmonary syndrome: chronic liver disease and arterial deoxygenation. The third criterion, intrapulmonary vascular dilatation, must be confirmed by contrast echocardiography or a technetium-99m macroaggregated albumin brain uptake scan. ^[2]