The diagnosis of renovascular hypertension with technetium-99m-ethylenedicysteine captopril scintigraphy.

RATIONALE AND OBJECTIVES: The authors present preliminary findings on the diagnosis of renovascular hypertension with technetium-99m-ethylenedicysteine (99mTc-EC). METHODS: Thirty-nine patients referred to the nuclear medicine department with clinical evidence of renovascular hypertension were included in the study. Baseline and captopril scintigraphies were done on separate days after the injection of 185 MBq of 99mTc-EC. All patients had angiographic correlation and 9 patients were shown to have renal artery stenosis. RESULTS: Quantitative analysis of the data showed no significant changes of perfusion index (PI), split renal function (SRF), and effective renal plasma flow (ERPF) values between pre- and postcaptopril studies in patients with significant renal artery stenosis (P > 0.05). Baseline and postcaptopril values for PI, SRF, and ERPF were measured as 128 +/- 21 and 116 +/- 12 mL/minute, 47 +/- 1 and 50 +/- 2 mL/minute, and 250 +/- 18 and 231 +/- 20 mL/minute, respectively. However, time to maximum activity (Tmax), time to half maximum activity (T 1/2), time to two thirds of maximum activity (T 2/3), and residual cortical activity (RCA) values showed marked changes with a rising renogram curve (P < 0.05). Baseline and postcaptopril values for Tmax, T 1/2, T 2/3, and RCA were measured as 3.1 +/- 0.1 and 20.2 +/- 1 minute, 5.4 +/- 0.4 and 45.4 +/- 3.1 minutes, 3.1 +/- 0.2 and 33.7 +/- 4.1 minutes, and 27 +/- 4 and 215 +/- 34 minutes, respectively. All scintigraphic studies showed good correlation with angiography and no false-positive or false-negative results were observed. CONCLUSIONS: This preliminary study demonstrates that 99mTc-EC has good potential for the diagnosis of renovascular hypertension and that a single diagnostic criteria, specifically a rising renogram curve, seems adequate. However, the authors' initial results should be confirmed in a broader patient population.

Technetium-99m-ethylenedicysteine in the diagnosis and follow-up of renovascular hypertension.

Technetium-99m-ethylenedicysteine (99mTc-EC) captopril scintigraphy performed in a patient with severe hypertension revealed increased parenchymal retention in the left kidney, suggesting renal artery stenosis. After angiographic confirmation of renal artery stenosis, percutaneous transluminal angioplasty (PTA) was performed on the left renal artery. Captopril scintigraphy after PTA showed normal findings with no evidence of parenchymal retention, consistent with reversal to normal kidney functions. In light of this case of renal artery stenosis, it was concluded that 99mTc-EC can be used successfully as a potential renal agent in the diagnosis and follow-up of renovascular hypertension.

Interlocking detachable coil occlusion in the endovascular treatment of intracranial aneurysms: preliminary results.

PURPOSE: To present our preliminary experience with the recently developed interlocking detachable coils in the treatment of intracranial aneurysms. METHODS: Two aneurysms of the basilar tip, two of the internal carotid artery, and one of the posterior inferior cerebellar artery were treated by an endovascular technique using interlocking detachable coils. Three of the patients had undergone unsuccessful surgical clipping. Three-month and 1-year control angiograms were obtained. RESULTS: In all patients but one, who had an aneurysm of the internal carotid artery, the aneurysmal sac was occluded with preservation of the parent artery and did not show recanalization on the follow-up control angiograms. In the other patient who had a wide-necked aneurysm of the internal carotid artery, the sac could not be totally obliterated and showed contrast filling in the neck remnant at 3-month angiography. None of the patients experienced neurologic deficit after treatment. CONCLUSION: Because they are soft and retrievable, interlocking detachable coils, with their immediate coil release design, may provide an alternative to surgery in the future treatment of endovascular aneurysms.

Case report: interruption of the inferior vena cava with anomalous intrahepatic continuation.

Infrahepatic interruption of the inferior vena cava (IVC) with azygos and hemiazygos substitution is a rare IVC anomaly which has been documented in the literature (Anderson et al, 1961; Chuang et al, 1974; Floyd & Nelson, 1976; Mayo et al, 1983). Recently Bercoff et al and Ghossain et al reported two cases with portal continuation of the interrupted IVC (Bercoff et al, 1985; Ghossain et al, 1988). In our case, we describe a left sided IVC with infrahepatic interruption which continues by both the azygos system and the hepatic veins via anomalous intrahepatic vessels. To our knowledge such a continuation with intrahepatic anomalous vessels has not been described before.

Intrahepatic portosystemic venous shunt: diagnosis by colour/power Doppler imaging and three-dimensional ultrasound.

Intrahepatic portosystemic venous shunt, considered to be a rare disease, can lead to hepatic encephalopathy. With recent advances in diagnostic imaging techniques, the number of reports of intrahepatic portosystemic venous shunts identified incidentally in patients without symptoms are increasing. We report an intrahepatic portosystemic venous shunt that was diagnosed incidentally by real-time ultrasound and colour Doppler imaging, including the use of three-dimensional ultrasound using minimum intensity projections and power Doppler.

Behçet's disease with severe arterial involvement in a child.

Behçet's disease is a vasculitis characterised by its thrombotic tendency. In some patients, manifestations of vascular lesions may dominate the clinical picture instead of the classic triad. We describe a 13-year-old boy with a 1.5-year history of Behçet's disease presenting with complaints of acute abdominal pain, severe headache and decreased vision. His work-up revealed a saccular aneurysm of the distal abdominal aorta, thrombosis in the right common iliac, external iliac and femoral arteries, and thrombosis of the superior sagittal sinus. Aortoiliac bypass with a Dacron graft was performed with success. He also received cyclosporin A and anticoagulant therapy. Severe vasculitis may become overt at any age in patients with Behçet's disease. Early diagnosis and management is important to prevent morbidity and mortality.

The factor V Leiden mutation: a possible contributor to the hepatic artery thrombosis encountered after liver transplantation in a child.

A 10-year-old girl has experienced 3 recurrences of hepatic artery thrombosis (HAT) after a liver transplantation. She responded to intraarterial administrations of urokinase after the first 2 attacks. However, the restoration of the arterial flow was not possible after the third attack. The child and her father were both heterozygous for factor V Leiden mutation. In addition to the technical factors, the factor V Leiden mutation should be considered as a factor that plays a role in HAT.

A rare vascular malformation, Klippel-Trenaunay syndrome. Report of a case with deep vein agenesis and review of the literature.

The Klippel-Trenaunay syndrome (KTS) is a rare anomaly characterized by naevus, soft tissue hypertrophy and varicosities which is mainly unknown etiology. Associated deep venous system abnormalities have been reported with KTS. Here, a 4-year-old-boy with KTS was reported, because of the associated popliteal vein, common femoral vein with its deep and superficial branches, and external iliac vein agenesis. The originality of the presented case is that we have never observed such a case before.

Coarctation of the abdominal aorta with left renal artery and splenic artery aneurysms.

Both coarctation of the abdominal aorta, and splenic artery aneurysm reside in the hilus, a rare but extremely important pathological condition. Herein a 36-year-old woman hospitalized for uncontrolled hypertension is presented. Aortography demonstrated segmental abdominal aortic coarctation with left renal artery and splenic artery aneurysms. Medical control of hypertension was not possible and thoracoabdominal bypass with a prosthetic graft was undertaken. Left renal artery aneurysm was resected and aorto-renal saphenous vein bypass was performed. But splenic artery aneurysm was left in situ because it was asymptomatic and less than 2 cm in diameter. Postoperatively the patient was normotensive. Although the pathogenesis of such lesions remain unknown, it may be to congenital or related medial degeneration as demonstrated histologically.

Cavernous transformation of the portal vein is associated with pancreatic duct atrophy.

BACKGROUND/AIMS: To determine whether or not portal venous obstruction affects pancreatic duct size. MATERIAL AND METHODS: The ERCP findings of 43 of 51 subjects with cavernous transformation of the portal vein (CTPV) were studied prospectively. In three, the ERCP failed to demonstrate the pancreatic duct (PD) for technical reasons and in 8 of the original 51 subjects, the ERCP was not attempted because of a lack of indications and unwillingness of the subject to undergo the procedure. The ERCP findings of the remaining 40 subjects were compared with those of 23 normal concurrent controls and those of reference controls. RESULTS: In 21 of the 51 patients, an underlying disorder responsible for portal vein thrombosis and cavernous transformation was identified. Despite a full clinical, radiologic, hematologic and biochemical evaluation, no etiology for CTPV was found in the remaining 30 patients. The pancreatic duct diameter was measured at the widest portion of the duct where it traverses the head of the pancreas. In 34 of the 40 patients with CTPV and a successful ERCP, the pancreatic duct was smaller (less than 2.28 mm in diameter) than that of concurrent control subjects and a reference control group (p < 0.05). In only 6 subjects with CTPV was the pancreatic duct size within normal limits. In 3 of these 6, a confounding common bile duct stone was shown to be present and may have accounted for the pancreatic duct enlargement as compared to the other 34 subjects with CTPV. CONCLUSIONS: These results indicate that a smoothly narrowing pancreatic duct hypoplasia or atrophy can be demonstrated in 85% of cases with CTPV. It is speculated that this finding is a consequence of the obstructed pancreatic venous drainage that occurs with CTPV.

Congenital hepatic fibrosis associated with cavernous transformation of the portal vein.

BACKGROUND/AIMS: Congenital hepatic fibrosis (CHF), which is one of the fibropolycystic diseases, occurs in various forms. Portal hypertension, a very common clinical feature of this condition, has been attributed to the compression of portal vein radicles in the fibrous bands. We investigated whether there are any other contributing factors in the development of portal hypertension in patients with CHF. METHODOLOGY: A total of 1285 patients with portal hypertension of different etiologies were studied using ultrasonography as the screening test. Forty-seven (including portal vein involvement and/or CHF) of these 1285 patients were prospectively studied to evaluate the etiology of the portal hypertension by portography, abdominal computed tomography, exploratory laparotomy, peritonoscopy, liver biopsy and laboratory tests. The patients with CHF were divided into two groups, according to whether or not they had portal vein involvement. RESULTS: Eleven (0.8%) of the 1285 patients with portal hypertension had CHF, and 41 (3.2%) had cavernous transformation of the portal vein (CTPV), resulting from different or unknown etiologies. Five patients had both pathologies (CTPV and CHF). In the 11 patients with CHF, there was CTPV in 5 patients, Caroli's disease in 2 patients, cholangiocarcinoma in 1 patient, inferior vena caval obstruction in 1 patient, and CHF in only 2 patients. There were statistically significant differences in the age of the CHF patients at clinical onset, the incidence of bleeding from esophageal varices, and laboratory findings between the 2 groups with and without CTPV. Despite a thorough investigation, we could not distinguish any predisposing factor in 25 of the 41 patients with CTPV. The incidence of CTPV was 48% in patients with CHF and 3.2% in patients with portal hypertension. CONCLUSIONS: These results suggest that the association of CTPV with CHF is not coincidental, but that CTPV may be associated with CHF and a new possible factor in portal hypertension, and that it can be a major factor in the manifestation of esophageal bleeding from varices.

A comparison of chemoembolization with conventional chemotherapy and symptomatic treatment in cirrhotic patients with hepatocellular carcinoma.

BACKGROUND/AIMS: The results of transcatheter arterial chemoembolization (TACE) in 28 cirrhotic patients with advanced hepatocellular carcinoma (HCC) were compared to those obtained in 15 cirrhotic patients with HCC treated with systemic chemotherapy, and 14 patients with HCC receiving no specific anti-cancer treatment. MATERIALS AND METHODS: From November, 1986 through May 1994, 235 patients with HCC were seen by the investigators. Twenty eight of these patients actually received TACE. The chemotherapeutic agent used was mitomycin C mixed with Lipiodol and arterial embolization was achieved using Gelfoam. In 19 of 28 cases treated with TACE, the embolization was limited to an artery feeding the tumor, thereby avoiding liver cell injury in non-tumor tissue. In 9 others, the main left or right hepatic artery was embolized. The results obtained in these 28 cases were compared to those obtained in 15 patients with HCC larger than 5 cm. who received systemic mitomycin C and doxorubicin chemotherapy and with those obtained in 14 patients who received no specific anti-tumor therapy. RESULTS: One patient died of liver failure related to the TACE and three patients died of bleeding from esophageal varices within weeks of the TACE procedure. Two of the remaining 24 patients are alive with a follow-up of 6 and 22 months. Twenty-six of the 28 patients treated with TACE died within 1 and 28 months of the initiation of the TACE therapy. The mean survival of those receiving TACE was 13.0 months. Chemotherapy without embolization yielded a mean survival of 7.2 months. The mean survival of the patients receiving no specific anti-cancer treatment was only 6.9 months. There was no statistical differences between the survival of those receiving systemic chemotherapy and those receiving no specific treatment. In contrast, the mean survival of the group receiving TACE was significantly greater than that of either of the other two groups (p < 0.005). CONCLUSIONS: These data suggest that TACE provides the best survival for individuals with larger hepatocellular carcinomas (> 5 cm) not eligible for surgery or percutaneous ethanol injection (PEI).

Rare anomaly of the aortic arch complex with extreme degenerative changes. A case report.

A forty-one-year-old woman with a rare anomaly of the aortic arch complex is described. The case closely resembles a combination of right cervical aortic arch and right aortic arch with aberrant left subclavian artery with additional tortuous right-sided aortic arch structures, absent left common carotid artery, and right common carotid artery originating from the left side of the aortic arch.

Pericardiac extravasation during intravenous digital subtraction angiography: a case report.

The authors reported a case, pericardiac extravasation of contract medium during an intravenous angiographic (IV DSA) procedure. To their knowledge this is the first such case in the literature. They suggest modifications of the IV DSA method to avoid this complication.

A digital splenoportography: more sensitive method of detecting spontaneous splenorenal shunt.

Splenoportography was performed upon 195 pediatric patients, including 137 by conventional technique and 58 by digital subtraction technique. The results obtained were compared, and it was found that the percentage of diagnosis of spontaneous splenorenal shunt was higher with digital than with conventional technique. In conclusion digital technique is a more sensitive and safer method than the conventional method.

Behçet's disease in the etiology of Budd-Chiari disease.

Hepatic venous outflow obstruction (Budd-Chiari syndrome-BCS) was diagnosed in 30 patients during the period from March, 1987, to May, 1991, in Hacettepe University Hospital, Turkey. Patients with Behçet's disease constituted the major group (12/30) in the etiologic distribution. Although the vascular manifestations of Behçet's disease, including venous and arterial occlusions, varices, and aneurysms, are well known, the hepatic vein involvement has been reported in only 22 cases in the literature so far. The authors present 30 cases of BCS, of whom patients with Behçet's disease comprise the largest group (40%). The angiographic findings are not specific to Behçet's disease. It is noted, however, that inferior vena cava involvement is more common in these patients (83.3%) than it is in the rest of the group (61.1%). It is suggested that Behçet's disease should be included among the diagnostic possibilities in a case of BCS, particularly in the countries in which it is prevalent.

Budd-Chiari syndrome: analysis of 30 cases.

The authors report their experience with 30 adult patients with Budd-Chiari syndrome (BCS), which is a rare and serious disorder, characterized by hepatic outflow obstruction caused by many different conditions. The diagnosis was based on the clinical data, ultrasonography (US), vena cavography and hepatic venography, computed tomography (CT), and liver biopsy. Behçet's disease (BD) was found in 10 patients with BCS as an underlying disease. Two patients used oral contraceptive drugs, 2 had liver tumor hepatocellular carcinoma and liver lymphoma, and 1 patient had chronic lymphocytic leukemia. Despite full investigation, the authors could not find any obvious underlying cause in the other 15 patients. The results suggest that (1) BCS must be considered as a possible complication in patients with Behçet's disease when they have hepatomegaly even if there were no cardinal manifestations of the disease at the time of admission, and BD is the most common etiologic factor in BCS (33%) in Turkey, where the incidence of Behçet's disease is relatively high. (2) Anti-aggregant treatment seems to be effective in many instances. (3) There were space-occupying lesion-like appearances in the liver of 7 cases by CT and US examination in the acute stage, and these disappeared on the follow-up CT and US in 5 patients but continued in 2. BCS should thus be differentiated from other liver lesions. (4) There were other great-vessel involvements in 43% of the cases, mostly venous, but only 1 pulmonary arterial occlusion.

Aneurysmatic arteriovenous fistula complicating splenic injury.

A child with a posttraumatic aneurysmatic arteriovenous (AV) fistula located in the spleen is presented. The fact that the increased use of more conservative diagnostic and therapeutic approaches in splenic injuries might lead to underestimation of some uncommon complications is discussed.

Renovascular hypertension due to Takayasu's arteritis demonstrated by Tc-99m ethylenedicysteine captopril scintigraphy.

A 34-year-old woman with severe hypertension underwent Tc-99m ethylenedicycteine (Tc-99m EC) captopril renal scintigraphy. There was bilateral parenchymal retention of the tracer suggesting renal artery stenosis. Angiography confirmed bilateral renal artery stenosis and also demonstrated contour irregularities of the abdominal aorta, and stenosis in mesenteric and subclavian arteries with increased collateral circulation consistent with Takayasu's arteritis. In light of this case of renal artery stenosis, it was concluded that Tc-99m EC can be used successfully as a potential renal agent in the diagnosis of renal involvement in patients with Takayasu's arteritis.

Congenital tracheoesophageal fistula without atresia: an incidental finding.

A four-year-old boy who had a long history of upper respiratory tract infections and growth retardation was admitted because of recurrent abdominal pain. During upper gastrointestinal series to search for a gastric or duodenal ulcer, the examiner noticed a minute amount of contrast medium within the trachea. Repeat esophagography on an angiographic table led to the correct diagnosis of a congenital H-type fistula. The patient did not have the classical symptoms of a history of choking and cyanosis after feeding during infancy or recurrent lower respiratory tract infections. The only finding consistent with a fistula was growth retardation, and the diagnosis was established incidentally during a work-up for abdominal pain.