Polycystic liver disease: Quantitation of parenchymal and cyst volumes from computed tomography images and clinical correlates of hepatic cysts

Abstract

Polycystic liver disease is a common manifestation of autosomal dominant polycystic kidney disease. However, factors that regulate hepatic cystogenesis have not been defined, and the effect of cyst formation on hepatic parenchymal mass has not been studied. We validated computed tomographic methods for measuring volumes from computed tomographic images using plastic-agar models and demonstrated that measured volumes were within 10% of actual volumes. The validated methods were used to measure hepatic parenchymal, hepatic cyst and kidney volumes in 25 subjects with polycystic liver disease and nine controls without autosomal dominant polycystic kidney disease. Hepatic cyst volume varied considerably in the 25 subjects with polycystic liver disease (20 to 7,148 ml), but hepatic parenchymal volume was not altered by hepatic cysts and was similar to that of controls (polycystic liver disease vs. controls: 1,357 +/- 185 vs. 1,319 + 340 ml). Total liver volume increased linearly as cyst volume increased (slope = 1.02 +/- 0.05, r = 0.994). Nine of 18 women with polycystic liver disease had massive hepatic cysts (cyst: parenchymal volume greater than 1; range of cyst volumes from 1,354 to 7,148 ml), and the other nine had cyst volumes (20 to 399 ml) similar to men with polycystic liver disease (25 to 1,107 ml). Total kidney volume, a measure of renal cystic disease, did not correlate with either total liver volume or the volume of hepatic cysts. The data indicate that hepatic parenchymal volume is preserved in polycystic liver disease despite massive cystic involvement and that women are uniquely susceptible to massive hepatic cystic disease.

Biliary Dysgenesis in the PCK Rat, an Orthologous Model of Autosomal Recessive Polycystic Kidney Disease

“…Computed tomographic methods to measure cystic volume in plastic agar models (in this model plastic cysts were embedded in agar) and in patients with ADPKD (without intravenous injection of contrast) had shown that this method is valid and accurate. 41,42 Our published data 43 also suggest that micro-CT images could be used to precisely measure the total liver tissue volume. In the PCK rat, liver parenchyma and cysts (based on differences in their densities) were easily defined by micro-CT images.…”

Polycystic disease of the liver

“…Patients with PCLD can be arbitrarily divided into two groups, massive or minimal, based on a definition for mass of total liver cyst/parenchymal volume ratio > 1. 76 Using this definition, most symptomatic cases are restricted to patients with massive hepatic cystic disease with abdominal pain and discomfort and shortness of breath correlating with severity of hepatic cystic disease. l8 Typically, liver parenchymal volume is preserved despite extensive hepatic cystic disease and extraordinary distortion of hepatic architecture (Fig.…”

VEGF receptor inhibition blocks liver cyst growth in pkd2(WS25/−) mice

“…The most common extrarenal manifestation, accounting for up to 10% of ADPKD morbidity and mortality, is the development of liver cysts. Within a clinically affected liver the parenchymal volume does not change significantly (7), but the liver can have hundreds of distinct cysts, with cysts reaching several centimeters in diameter. In the liver, the clinical manifestations of ADPKD are associated directly with the compression of the surrounding tissues, organs, and vasculature.…”