VALUE OF DOPPLER SONOGRAPHY IN THE ASSESSMENT OF PATIENTS WITH CAROLIS-DISEASE

Purpose. We examined the intracystic flow pattern and portal hemodynam ics with Doppler sonography in patients with Caroli's disease. Methods . Sonographic characteristics and portal hemodynamics were studied by color Doppler sonography and spectral analysis in 5 patients (4 childr en and 1 adult) with Caroli's disease using high-frequency transducers . Caroli's disease was associated with infantile polycystic kidney dis ease in 4 patients and congenital hepatic fibrosis in 2 patients. Four patients had no clinical or imaging evidence of portal hypertension. The adult patient had congenital hepatic fibrosis and portal hypertens ion. Results. Color Doppler sonograms and spectral analyses disclosed distinctive hepatic arterial and portal venous flow within the fibrova scular projections in the bile ducts of all the children. The adult wi th advanced portal hypertension presented with a no-flow state in the intracavitary part of the portal vein and a strong arterial signal rel ated to disturbed hemodynamics in the arterialized liver. Conclusions. The fact that portal hemodynamics change over time should be taken in to account when Doppler assessment of Caroli's disease is attempted. D oppler sonographic monitoring of the portal system to indirectly diagn ose and follow the progression of so-called congenital hepatic fibrosi s may be an effective alternative to liver biopsy. (C) 1998 John Wiley & Sons, Inc.