Clinical presentation

A 63 year old woman was admitted for diuretic refractory ascites. Her mother was affected by chronic renal failure due to polycystic kidney disease and she suffered from arterial hypertension. Ten months before she had presented with abdominal distension; laboratory tests showed serum creatinine 2 mg/dl, normal liver function, negative hepatitis B surface antigen, and hepatitis C virus antibody. Abdominal ultrasound revealed ascites, hepatomegaly, multiple hepatic and renal cysts, and patent portal and hepatic veins. Laparoscopy excluded cirrhosis or malignancy and upper gastrointestinal endoscopy was normal. Diuretic therapy and a low sodium/protein diet were prescribed but the abdominal distension worsened and required repeated paracenteses. At admission, the patient was confined to bed, her appetite and nutritional condition were poor; physical examination disclosed tender hepatomegaly, tense ascites, and mild lower limb oedema. Liver tests were normal, serum albumin 3.0 g/dl, creatinine 2.4 mg/dl, urea 102 mg/dl, serum electrolytes normal, creatinine clearance 18 ml/min, haemoglobin 10.8 g/dl, and platelets 190 000/mm³. The serum-ascites albumin gradient was 2.0 g/dl. Cytology and microbiological investigations were negative. Ascites was unresponsive to furosemide at a dose of 150 mg/day and recurred quickly after paracentesis.

Question

Abdominal magnetic resonance imaging and cavography images (fig 1) are depicted. What is the diagnosis?

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Figure 1

 Magnetic resonance imaging (MRI) axial and coronal T2 weighted images (A, B). MRI gadolinium enhanced T1 weighted image (C). Sagittal plane reconstruction of image (D). (E) Cavogram.

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