Gorka W, Lewall D B
Department of Medical Imaging, King Fahad National Guard Hospital, Riyadh, Saudi Arabia.
J Clin Ultrasound. 1998 Jul-Aug;26(6):283-7. doi: 10.1002/(sici)1097-0096(199807/08)26:6<283::aid-jcu1>3.0.co;2-e.
We examined the intracystic flow pattern and portal hemodynamics with Doppler sonography in patients with Caroli's disease.
Sonographic characteristics and portal hemodynamics were studied by color Doppler sonography and spectral analysis in 5 patients (4 children and 1 adult) with Caroli's disease using high-frequency transducers. Caroli's disease was associated with infantile polycystic kidney disease 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 hypertension.
Color Doppler sonograms and spectral analyses disclosed distinctive hepatic arterial and portal venous flow within the fibrovascular projections in the bile ducts of all the children. The adult with advanced portal hypertension presented with a no-flow state in the intracavitary part of the portal vein and a strong arterial signal related to disturbed hemodynamics in the arterialized liver.
The fact that portal hemodynamics change over time should be taken into account when Doppler assessment of Caroli's disease is attempted. Doppler sonographic monitoring of the portal system to indirectly diagnose and follow the progression of so-called congenital hepatic fibrosis may be an effective alternative to liver biopsy.
我们利用多普勒超声检查了卡罗里病患者的囊内血流模式和门静脉血流动力学。
使用高频探头，通过彩色多普勒超声和频谱分析对5例卡罗里病患者（4例儿童和1例成人）的超声特征和门静脉血流动力学进行了研究。4例患者的卡罗里病与婴儿型多囊肾病相关，2例患者与先天性肝纤维化相关。4例患者无门静脉高压的临床或影像学证据。成年患者患有先天性肝纤维化和门静脉高压。
彩色多普勒超声检查和频谱分析显示，所有儿童胆管内纤维血管突起内有独特的肝动脉和门静脉血流。患有晚期门静脉高压的成年患者门静脉腔内部分呈无血流状态，与动脉化肝脏血流动力学紊乱相关的动脉信号较强。
在尝试对卡罗里病进行多普勒评估时，应考虑门静脉血流动力学随时间变化这一事实。对门静脉系统进行多普勒超声监测以间接诊断和跟踪所谓先天性肝纤维化的进展，可能是肝活检的一种有效替代方法。
