An atypical course of Caroli's disease in a renal transplant patient--case report and review of the literature.

This is a rare case of Caroli's disease, diagnosed following renal transplantation in a patient with autosomal recessive polycystic kidneys. Despite advanced cystic transformation of the biliary tree with striking architectural changes, there was no evidence of portal hypertension or hepatic fibrosis. Moreover, the patient did not suffer a single episode of cholangitis, a most interesting feature of this case. Her clinical course was punctuated by repeated episodes of gastrointestinal and urinary tract infections with resistant organisms; but fortunately, she had no evidence of septicemia. Recurrent Salmonella gastroenteritis indicated a chronic carrier state with the dilated bile ducts possibly acting as a potential reservoir. This has significant implications considering the immune suppression associated with renal transplantation. In general, Caroli's disease is rare. Therefore, a high index of suspicion for the diagnosis of Caroli's disease is warranted especially in patients with ARPKD or ADPKD. Once confirmed, affected patients with end-stage renal disease such as our patient, should ideally undergo combined liver-kidney transplantation.

Radiology in the neonatal intensive care unit: dose reduction and image quality.

This paper describes a prospective study of the diagnostic radiation doses received in a neonatal intensive care unit (NICU) for a representative radiological technique used at our institution for a number of years and a "low dose" technique similar to that recommended by the Commission of the European Communities (CEC). A 400 speed film-screen combination was used in both techniques. A total of 363 anteroposterior (AP) chest and abdominal films of 77 neonates were accrued. For each radiograph, the entrance skin dose (FSD), energy imparted (EI) and mean whole body dose were determined. For a neonatal AP chest, there was an 18% reduction in the mean ESD per radiograph from 20.0 muGy for the representative technique to 16.4 muGy for the low dose technique (p < 0.0005). The reduction in the mean EI per radiograph values for the two techniques from 7.9 muJ to 7.1 muJ (10%) was statistically significant at the p < 0.017 level, after compensating for the difference in mean field dimensions between the two patient cohorts. The mean whole body dose per radiograph reduction from 4.4 to 3.5 muGy (20%) was statistically significant at the p < 0.0028 level. It was determined that the ESD and EI could be fitted by an exponential function in the equivalent patient diameter, a single parameter indicative of neonate size. Absolute excess childhood cancer mortality risk per film was estimated using risk factors derived for fetal exposures. A "worst case" absolute excess mortality risk per chest radiograph was estimated to be 1.40 x 10(-7) for the conventional technique and was further reduced to 1.11 x 10(-7) for the low dose technique. A blind comparison of patient-matched film pairs for each technique was performed by three radiologists using criteria similar to those specified by the CEC. No statistically significant difference in clinical image quality was found between the two techniques.

Value of Doppler ultrasound hemodynamics in the assessment of renal artery stenosis in transplanted kidneys: an assessment of patients after percutaneous transluminal angioplasty.

UNLABELLED: Doppler ultrasound (USS) may allow a non-invasive early diagnosis of transplant renal artery stenosis (TRAS). Adequate Doppler sampling of the transplant renal artery is difficult, time consuming and highly operator dependent. As a result, there has been increased attention focused on the intrarenal vessels and downstream changes that occur secondary to TRAS. We evaluated Doppler USS parameters in nine patients with TRAS confirmed on angiography (significant stenosis defined as > 60% diameter narrowing). Doppler USS correctly identified all the nine stenoses. Mean peak systolic velocity (PSV) was 3.6 m/s + 1.09. Mean end-diastolic velocity (EDV) was 1.75 m/s + 0.25 with an acceleration time (AT) of 0.14 + 0.04 sec and resistivity index (RI) of 0.42 + 0.12. Early systolic peak (ESP) was lost in all cases. Percutaneous transluminal angioplasty (PTA) was successfully done in five patients with significant improvement in Doppler parameters. PSV decreased from 4.04 m/s + 1.35 to 0.76 m/s + 0.42 (p = 0.01). Similarly EDV improved from 1.71 m/s + 0.28 to 0.30 m/s + 0.17 (p = 0.001). AT improved from 0.13 + 0.01 to 0.05 + 0.01 sec (p = 0.001). RI normalized from 0.34 + 0.07 to 0.73 + 0.09 (p = 0.008). ESP was restored in all the patients. IN CONCLUSION: our results show that the Doppler USS analysis of segmental arteries is an excellent tool for the diagnosis of TRAS and follow-up of patients post PTA.

Value of Doppler sonography in the assessment of patients with Caroli's disease.

PURPOSE: We examined the intracystic flow pattern and portal hemodynamics 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 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. RESULTS: 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. CONCLUSIONS: 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.

Doppler sonographic diagnosis of severe portal vein pulsatility in constrictive pericarditis: flow normalization after pericardiectomy.

This case report describes the noninvasive assessment of hepatic and portal vein hemodynamics in a patient with constrictive pericarditis before and after pericardiectomy. Doppler sonography of the hepatic veins demonstrated a typical W-shaped pattern with pronounced late diastolic flow reversal that disappeared after surgery. Preoperatively, we observed severe pulsatility of the portal vein with flow reversal in systole; after pericardiectomy, portal venous flow was normal. We concluded that the high right atrial pressure in this patient might have led to increased hepatic venous outflow resistance, with subsequent trans-sinusoidal shunting between the hepatic artery and portal vein causing severe portal vein pulsatility. After pericardiectomy and a decrease in right atrial pressure, portal vein flow normalized.

Doppler ultrasound evaluation of advanced portal vein pulsatility in patients with normal echocardiograms.

The hypothesis tested that mechanisms other than retrograde transsinusoidal fluid wave transfer reported in patients with right heart failure are responsible for the ultrasonographic sign of advanced portal vein pulsatility (APP). Within a time-period of 3 years we have seen 13 patients with APP, defined as temporary portal flow reversal in the face of a normal echocardiogram. Nine of these patients had biopsy-proven liver cirrhosis and four with liver disease were without cirrhosis or cardiac pathology. A randomly selected control group of 18 healthy subjects was studied. Doppler ultrasound evaluation of the hepatic veins as well as the intra and extrahepatic portal vein territories was performed in both groups. Hepatopetal portal flow with APP reversed to hepatofugal flow in follow up studies in two patients. In another two hepatopetal flow with APP in the main portal vein and hepatofugal flow in the intrahepatic portal radicles was recorded during the same examination. The remaining group displayed APP in the intra and extrahepatic portal vein territories. None of the normal subjects presented with APP. Hepatic venous outflow obstruction associated with excessive arterioportal shunting is likely to account for APP of all of our patients. Based on a causal link between angiographic 'to-and-fro' flow pattern and the sonographic APP sign in patients with sinusoidal outflow obstruction we suggest, that APP expresses a short, transitional period of portal hypertension just before the occurence of flow reversal.

Diagnostic value of Doppler ultrasound in the assessment of liver cirrhosis in children: histopathological correlation.

We have correlated flow abnormalities in the hepatic vasculature with histological findings in the liver to determine the accuracy of Doppler ultrasound in the diagnosis of liver cirrhosis in children. Eighteen children admitted for evaluation of unknown liver disease were examined prospectively and blindly with Doppler ultrasound prior to liver biopsy. Biopsy specimens showed established cirrhosis in 9 of 18, early cirrhosis in 5 of 18, and no cirrhosis in 4 of 18 children. Doppler studies were also performed on 20 control subjects. The portal vein velocity was decreased (p < 0.0005) and the arterio-portal velocity ratio was increased (p < 0.0005) in the established cirrhosis cohort relative to the controls. For the criteria of the established cirrhosis cohort, the sensitivities of the loss of the reverse flow component in the hepatic veins, the arterio-portal velocity ratio being greater than 3.0, the portal vein velocity being less than 20 cm/s, and the existence of focal flow acceleration in the hepatic veins were 100%, 78%, 67%, and 44%, respectively. The specificity of all these criteria was 100%. The indicators were not useful in the diagnosis of early cirrhosis.

Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver.

We describe an Arab girl with complete absence of phosphorylase b kinase activity in the liver, symptomatic hypoglycemia, and persistently elevated serum aminotransferase values whose symptoms did not lessen with age; sequential liver biopsies showed progression to cirrhosis. Cirrhosis could not be ascribed to any other known cause. We conclude that type IX glycogenosis is not always associated with a benign outcome.

Qualitative hepatic venous Doppler sonography versus portal flowmetry in predicting the severity of esophageal varices in hepatitis C cirrhosis.

OBJECTIVE: The purpose of this study was to compare the diagnostic accuracy of simple recognition of hepatic vein waveform abnormalities using Doppler sonography with portal Doppler flowmetry for the noninvasive assessment of esophageal varices in patients with hepatitis C cirrhosis. SUBJECTS AND METHODS: Fifty patients with biopsy-proven liver cirrhosis caused by hepatitis C who were being examined for possible liver transplantation were studied prospectively with Doppler sonography by a single observer. Hepatic vein waveforms were classified as normal triphasic, abnormal biphasic, monophasic, and those with loss of the reverse-flow component. Portal flow indicators included the maximum values of portal flow velocity, portal vein flow volume, diameter of the portal vein, and congestion index. For the purposes of this study, we simplified the endoscopic grading of varices by classifying F1 and F2 varices as small and F3 as large. None of the patients had clinical or echocardiographic signs of failure of the right side of the heart. RESULTS: Sensitivity for the detection of large varices was 92% for monophasic waves, 76% for waves with loss of the reverse flow component, and 62% for biphasic waves. Overall specificity was 100%. Portal vein diameter and congestion index were higher (p < .02) and portal vein velocity was lower (p < .05) in patients with varices than in patients without varices, but these indicators were not useful in determining the size of varices. Portal vein flow volume did not differ in the presence of varices or ascites and was independent of the morphology of the hepatic vein wave. CONCLUSION: Simple recognition of patterns seen in hepatic vein waveform morphology in patients with liver cirrhosis caused by hepatitis C is superior to portal Doppler flowmetry for predicting the size of esophageal varices.

Severe hepatic injury associated with lipid formulations of amphotericin B.

A 14-year-old girl with leukemia had Doppler ultrasound evidence of hepatic necrosis, thought to be caused by a lipid formulation of amphotericin B, which has not been previously reported. It seems prudent to exert caution when retreating patients with previous hepatocellular damage with lipid formulations of amphotericin B.

Doppler ultrasound evaluation of hemodialysis vascular access.

Brescia-Cimino arteriovenous fistulas (AVF) ad synthetic grafts are the usual forms of vascular accesses for hemodialysis. Although angiography has been the traditional means of imaging these vascular systems, colour Doppler flow imaging (CDFI) offers a non-invasive method of evaluating AVF dysfunction. We retrospectively evaluated 22 patients with clinical evidence of access dysfunction who were studied by CDFI and of whom 14 also underwent angiography. We analyzed the results of whom the 14 patients who had both CDFI and angiography as angiography was impossible in the remaining eight patients due to difficulty with cannulation. Eight patient had thrombosis on CDFI and angiopraphy in all eight patients confirmed these findings. CDFI showed six stenoses, all of which were proven on angiopraphy. Overall,, CDFI correctly identified all lesions that were seen angiographically ginging a sensitivity and specifificity of 100%. Moreover, CDFI detected two cases of pseudoaneurysms which were missed by angiography. CDFI provides an adequate means of evaluating AVF dysfunction and should be the initial imaging technique of choice.