An augmented patient-specific approach to administration of contrast agent for CT renal angiography

ABSTRACT Purpose This hybrid retrospective and prospective study performed on 200 consecutive patients undergoing renal CTA, investigates the opacification of renal vasculature, radiation dose, and reader confidence. Materials and Methods 100 patients were assigned retrospectively to protocol A and the other 100 were allocated prospectively to protocol B. Both protocols implemented a contrast material and saline flow rate of 4.5 mL/sec. Protocol A utilized a 100 mL of low-osmolar nonionic IV contrast material (Ioversol 350 mg I/mL) while protocol B employed a patient-tailored contrast media formula using iso-osmolar non-ionic (Iodixanol 320 mg I/mL). Results Arterial opacification in the abdominal aorta and in the bilateral main proximal renal arteries demonstrated no statistical significance (p>0.05). Only the main distal renal artery of the left kidney in protocol B was statistically significant (p<0.046). In the venous circulation, the IVC demonstrated a significant reduction in opacification in protocol B (59.39 HU ± 19.39) compared to A (87.74 HU ± 34.06) (p<0.001). Mean CNR for protocol A (22.68 HU ± 13.72) was significantly higher than that of protocol B (14.75 HU ± 5.76 p< 0.0001). Effective dose was significantly reduced in protocol B (2.46 ± 0.74 mSv) compared to A (3.07 ± 0.68 mSv) (p<0.001). Mean contrast media volume was reduced in protocol B (44.56 ± 14.32 mL) with lower iodine concentration. ROC analysis demonstrated significantly higher area under the ROC curve for protocol B (p< 0.0001), with inter-reader agreement increasing from moderate to excellent in renal arterial visualization. Conclusion Employing a patient-tailored contrast media injection protocol shows a significant refinement in the visualization of renal vasculature and reader confidence during renal CTA.

A teenage patient with autosomal recessive polycystic kidney disease, a splenorenal shunt, and congenital hepatic fibrosis: a case report / Doença renal policística autossômica recessiva, shunt esplenorrenal e fibrose hepática congênita em adolescente: relato de caso

ABSTRACT A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an endoscopic procedure to treat the gastrointestinal bleeding. The analysis of the differential diagnosis for chronic liver disease indicated she had a spontaneous splenorenal shunt. Ultrasound-guided biopsy revealed the patient had cirrhosis, as characteristically seen in individuals with ARPKD. She had no symptoms at discharge and was referred for review for a combined transplant.

RESUMO Relato de caso de uma paciente adolescente de 16 anos de idade com diagnóstico prévio de doença renal policística autossômica recessiva (DRPAR), que apresentou quadro agudo de pneumonia bilateral e hemorragia digestiva alta por ruptura de varizes esofágicas, bem como ascite e edema de membros inferiores. Necessitou de estabilização clínica intensiva e tratamento endoscópico do sangramento digestivo. Após investigação dos diagnósticos diferenciais da hepatopatia crônica, diagnosticou-se shunt esplenorrenal espontâneo, e realizou-se biópsia hepática guiada por ecografia com diagnóstico de cirrose, espectro típico da DRPAR. Recebeu alta hospitalar assintomática e foi encaminhada para avaliação de transplante duplo.

Síndrome del cascanueces / Nutcracker syndrome

El síndrome del cascanueces es una anomalía vascular en la que se comprime la vena renal izquierda a su paso entre la unión de la aorta y la arteria mesentérica superior, debido a un ángulo muy cerrado entre ambas arterias. Clínicamente puede presentarse como hematuria macro o microscópica, dolor episódico en flanco izquierdo, dolor pélvico, várices gonadales o simplemente cursar de forma asintomática. Presentamos dos casos clínicos, uno de ellos con dolor abdominal tipo cólico nefrítico y hematuria macroscópica y otro estudiado por hipertensión pero con antecedentes de embolización de la vena renal izquierda por varicocele izquierdo. Discutimos los aspectos clínicos, diagnósticos y terapéuticos de este síndrome.

Nutcracker syndrome is a vascular anomaly consisting in the compression of the left renal vein between the superior mesenteric artery and the aorta. Clinical features in nutcracker syndrome include pelvic pain, flank pain, haematuria, gonadal varices or simply asymptomatic. We are presenting two cases, one of them with macroscopic haematuria and flank pain and the other was studied for hypertension but with previous antecedents of left renal vein embolization in the setting of varicocele. We discuss the clinical presentation as well as diagnostic and therapeutic aspects related to this syndrome.

Cavernoma portal complicada con biliopatía secundario a enfermedad de Gaucher tipo 1: reporte de un caso peruano / Cavernoma complicated with biliopatia secondary to type 1 Gaucher disease: report of a Peruvian case

La enfermedad de Gaucher, es un trastorno autosómico recesivo de depósito lisosomal que se caracteriza por deficiencia de la beta-glucocerebrosidasa que lleva a la acumulación de glucosilceramida principalmente en células del sistema fagocítico mononuclear causando afectaciones sistémicas. Se presenta paciente varón de 20 años que cursa con dolor crónico en hipocondrio izquierdo con episodios de sangrados desde hace 3 años y sensación de alza térmica, al examen físico se identificó ictericia y esplenomegalia masiva, sin afectación neurológica. Como apoyo al diagnóstico se mostró osteoporosis severa, pancitopenia y como hallazgo inesperado la presencia de trombosis de vena porta con transformación cavernomatosa complicada con biliopatía portal simulando un tumor de klatskin, los estudios de médula y enzimáticos eran compatibles con enfermedad de Gaucher, por lo cual recibió tratamiento con imiglucerasa realizando seguimiento. Es un caso poco frecuente, de gran interés, heterogeneidad en sus manifestaciones clínicas e inéditas por su complicación, constituyendo un desafío llegar a su diagnóstico de esta enfermedad huérfana.

Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. he received treatment with imiglucerase, following up. It is a rare case, of great interest, heterogeneity in its clinical manifestations and unpublished by its complication, constituting a challenge to reach its diagnosis of this orphan disease.

Laparoscopic donor nephrectomy in unusual venous anatomy - donor and recepient implications

ABSTRACT Objectives Laparoscopic donor nephrectomy is now a commonly performed procedure in most of renal transplantation centers. However, the suitability of laparoscopy for donors with abnormal venous anatomy is still a subject of debate. Materials and methods Between August 2007 and August 2014, 243 laparoscopic donor nephrectomies were performed in our institution. All donors were evaluated with preoperative three-dimensional spiral computed tomography (CT) angiography Thirteen (5.35%) donors had a left renal vein anomaly. A retrospective analysis was performed to collect donor and recipient demographics and perioperative data. Results Four donors had a type I retroaortic vein, seven had type II retroaortic vein and a circumaortic vein was seen in three donors. The mean operative time was 114±11 minutes and mean warm ischemia time was 202±12 seconds. The mean blood loss was 52.7±18.4mL and no donor required blood transfusion. Mean recipient creatinine at the time of discharge was 1.15±0.18mg/dL, and creatinine at six months and one year follow-up was 1.12±0.13mg/dL and 1.2±0.14mg/dL, respectively. There were no significant differences in operative time, blood loss, warm ischemia time, donor hospital stay or recipient creatinine at 6 months follow-up, following laparoscopic donor nephrectomy in patients with or without left renal vein anomalies. Conclusion Preoperative delineation of venous anatomy using CT angiography is as important as arterial anatomy. Laparoscopic donor nephrectomy is safe and feasible in patients with retroaortic or circumaortic renal vein with good recipient outcome.

Renal Pelvic Urothelial Carcinoma With Vena Caval Thrombus Mimicking Renal Cell Carcinoma

A 61-year-old man presented with a right renal mass with a vena caval thrombus on computed tomography that was consistent with renal cell carcinoma. The results of routine laboratory examinations and urinalysis were within normal limits. Preoperative planning was critical owing to the presence of the vena caval thrombus. A radical nephrectomy, vena caval thrombectomy, and regional lymphadenectomy were done. The pathologic report was consistent with a high-grade, invasive urothelial carcinoma, with sarcomatoid differentiation involving the renal vein and inferior vena cava (Stage IV, T4N0M0). Thus, this was a rare case of upper tract urothelial carcinoma. Adjuvant chemotherapy with the methotrexate, vinblastine, doxorubicin, cisplatinum regimen is scheduled. To our knowledge, this is the first report in Korea of upper tract urothelial carcinoma of the sarcomatoid type with a vena caval thrombus.

Renal Pelvic Urothelial Carcinoma With Vena Caval Thrombus Mimicking Renal Cell Carcinoma

A 61-year-old man presented with a right renal mass with a vena caval thrombus on computed tomography that was consistent with renal cell carcinoma. The results of routine laboratory examinations and urinalysis were within normal limits. Preoperative planning was critical owing to the presence of the vena caval thrombus. A radical nephrectomy, vena caval thrombectomy, and regional lymphadenectomy were done. The pathologic report was consistent with a high-grade, invasive urothelial carcinoma, with sarcomatoid differentiation involving the renal vein and inferior vena cava (Stage IV, T4N0M0). Thus, this was a rare case of upper tract urothelial carcinoma. Adjuvant chemotherapy with the methotrexate, vinblastine, doxorubicin, cisplatinum regimen is scheduled. To our knowledge, this is the first report in Korea of upper tract urothelial carcinoma of the sarcomatoid type with a vena caval thrombus.

CT Angiography for Living Kidney Donors: Accuracy, Cause of Misinterpretation and Prevalence of Variation

OBJECTIVE: To determine the accuracy of the use of multi-detector row CT (MDCT) to predict vascular anatomy in living kidney donors and to reveal the prevalence of vascular variations in a Korean population. MATERIALS AND METHODS: A total of 153 living kidney donors that had undergone preoperative CT and nephrectomy, either with open or laparoscopic surgery, were selected retrospectively. The initial CT results were compared with the surgical findings and repeated review sessions of CT scans were performed to determine the causes of mismatches in discordant cases. RESULTS: The accuracy of CT angiography was 95% to predict the number of renal vessels. Four arteries and two veins were missed during the initial CT interpretation due to perception errors (for two arteries and two veins) and technical limitations (two arteries). The prevalence of multiple renal arteries and veins, early branching of a renal artery and late confluence of a renal vein were 31%, 5%, 12%, 17%, respectively. The circumaortic renal vein and the bilateral inferior vena cava were found in two cases each (1.3%). One case (0.7%) each of a retroaortic renal vein and a supradiaphragmatic originated renal artery were found. CONCLUSION: MDCT provides a reliable method to evaluate the vascular anatomy and variations of living kidney donors.

Hepatic myelopathy as a presenting neurological complication in patients with cirrhosis and spontaneous splenorenal shunt / 대한간학회지

Hepatic myelopathy is a rare complication of chronic liver disease that is associated with extensive portosystemic shunts. The main clinical feature of hepatic myelopathy is progressive spastic paraparesis in the absence of sensory or sphincter impairment. Early and accurate diagnosis of hepatic myelopathy is important because patients with early stages of the disease can fully recover following liver transplantation. Motor-evoked potential studies may be suitable for the early diagnosis of hepatic myelopathy, even in patients with preclinical stages of the disease. Here we describe two patients who presented with spastic paraparesis associated with a spontaneous splenorenal shunt and without any previous episode of hepatic encephalopathy. One patient experienced improved neurologic symptoms after liver transplantation, whereas the other patient only received medical treatment, which did not prevent the progression of spastic paraparesis.

Power Doppler Imaging in Acute Renal Vein Occlusion and Recanalization: a Canine Model

OBJECTIVE: To evaluate the dynamic changes of the power Doppler (PD) in acute renal vein occlusion and recanalization in a canine model. MATERIALS AND METHODS: We performed a PD of the kidney during graded renal vein occlusion and recanalization induced by balloon inflation and deflation in nine dogs. The PD images were transferred to a personal computer, and the PD signals were quantified. RESULTS: We observed the temporal change of the PD signal during renal vein occlusion and recanalization, with a decrease in the PD signal during occlusion and an increase during recanalization. The mean PD signal decreased gradually as the renal vein was occluded, and conversely increased gradually with sequential relief of occlusion. The sequential change of the mean value of the PD signal was statistically significant. CONCLUSION: The PD can detect a change in renal blood flow during acute renal vein occlusion and recanalization in a canine model. The PD may be used as a helpful tool for the early detection of acute renal vein thrombosis and the monitoring of renal perfusion.

Hemostatic profile in nephrotic syndrome.

OBJECTIVE: To evaluate the coagulation profile and its relation to steroid therapy, and the frequency of thromboembolic complications and its correlation with coagulation parameters in nephrotic syndrome (NS). SETTING: Hospital based. SUBJECTS AND METHODS: Forty children with NS were subdivided into four groups, namely, fresh cases, steroid dependent, remission after therapy and steroid resistant. An equal number of age and sex matched children served as controls. In all the study and control subjects, detailed clinical examination, liver function tests, renal function tests and detailed coagulation profile were done. Evaluation of renal veins and inferior vena cava for the presence of thrombosis was also done by abdominal ultrasonography. RESULTS: Thrombocytosis was detected in 57.5% and the degree of thrombocytosis was directly related to the amount of proteinuria. The mean prothrombin and thrombin times were within normal range in the study children. The activated partial thromboplastine time (APTT) was prolonged in six cases (15%) and three out of these six children had thromboembolic complications. Antithrombin-III level was significantly lower (p < 0.001) whereas protein C and S were significantly elevated (p < 0.001) as compared to controls. The levels became normal with remission of the disease. Steroid therapy significantly increased the levels of proteins C, protein S. AT-III and fibrinogen as compared to controls. Thromboembolic complications were seen in 3 cases (7.6%) and were associated with very low levels of AT-III and protein C and all three had serum albumin below 2 g/dl. CONCLUSIONS: The importance of coagulation profile in nephrotic syndrome is highlighted and a high index of suspicion for thromboembolic complications is warranted in patients with thrombocytosis, hyper fibrinogenemia, prolonged APTT and in children with low levels of AT-III, protein C and protein S.