Tunneled internal jugular catheters in adult patients: comparison of outcomes in hemodialysis versus infusion catheters.

BACKGROUND: Tunneled central venous catheters placed by interventional radiologists are now widely used for hemodialysis and infusion therapies throughout the world. However, complications such as infections and malfunctions still remain a major concern in oncology and hemodialysis patients. PURPOSE: To evaluate the long-term follow-up results of tunneled central venous catheters in an adult population in terms of infectious complications and malfunction rates in dialysis and oncology patients. MATERIAL AND METHODS: We retrospectively reviewed the hospital charts and our electronic database for 434 tunneled internal jugular catheters in 335 consecutive patients between December 2002 and March 2006. Mean patient age was 57 years (range 23-86 years) in the hemodialysis group and 45 years (range 18-83 years) in the infusion group. A total of 224 hemodialysis catheters were placed in 168 patients (68 females, 100 males) and 210 infusion catheters in 167 patients (48 females, 119 males). RESULTS: Technical success rate was 100%. Mean duration of catheter use was 86 days (1-652 days) and 60 days (2-686 days) for hemodialysis and infusion catheters, respectively. A total of 107 hemodialysis (47%) and 95 infusion catheters (45%) were electively removed due to completion of therapy and resolution of need for dialysis. Revisions were performed 0.22 and 0.11 per 100 catheters days in the hemodialysis and infusion groups, respectively. Our total infection rate was 0.10 episodes per 100 catheter days, and the rate of infections necessitating catheter removal was 0.05 episodes per 100 catheter days in the hemodialysis group, which is lower than that reported in other big series. However, in the infusion group, the rate of infections necessitating catheter removal was 0.28 episodes per 100 catheter days. CONCLUSION: Long-term central venous accesses using tunneled internal jugular catheters appeared to be safe and effective for both hemodialysis and long-term infusion therapies, with relatively higher infection rates in oncologic patients.

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.

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.

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.

Antiphospholipid syndrome presenting as portopulmonary hypertension.

The association of pulmonary hypertension with portal hypertension, also called portopulmonary hypertension, is a well-described condition. The pathogenesis of this association remains unclear. We describe a 34-year-old female patient with "primary antiphospholipid syndrome" and portopulmonary hypertension. Our finding supports that in situ microthrombosis associated with the presence of anticardiolipin antibodies could be the pathophysiologic explanation for both portal and pulmonary hypertension.

Disappearance of "pseudocholangiocarcinoma sign" in a patient with portal hypertension due to complete thrombosis of left portal vein and main portal vein web after web dilatation and transjugular intrahepatic portosystemic shunt.

The main portal vein web is probably a consequence of portal vein thrombosis, which is a very rare cause of portal hypertension. Principal manifestations are related to the degree of portal hypertension. In the literature, no data has been found for the treatment modality of portal vein web. We report, herein, the clinical and laboratory findings of a 38-year-old woman with angiographically proven incomplete main portal vein web and complete thrombotic occlusion of the left portal vein causing pseudocholangiocarcinoma sign (PCCS) on the common bile duct. She was treated by transjugular intrahepatic portosystemic shunt (TIPS) and membrane dilatation, which resulted in complete disappearance of collaterals and PCCS. It appears that TIPS and balloon dilatation of the portal vein web via transjugular approach was effective in decreasing portal pressure and its consequences.

Hepatic artery aneurysm in a patient with Behçets disease and segmental pancreatitis developing after its embolization.

Segmental pancreatitis is an unusual form of acute pancreatitis mostly seen in the head of pancreas. We present the CT findings of a segmental pancreatitis in the body and tail of the pancreas developed following endovascular embolization of a giant hepatic artery aneurysm and arterioportal fistula in a patient with Behçet's disease.

Pelvic kidney with an unusual blood supply: angiographic findings.

A right pelvic kidney was observed in a patient, who presented with hypertension. On angiograms, the left kidney was normally positioned and had a single renal artery, whereas the right pelvic kidney received three arteries, which arose from bilateral common iliac arteries and from ipsilateral internal iliac artery. The renal arteries from the ipsilateral internal iliac artery and the contralateral common iliac artery supplied the medial half of the pelvic kidney. In the present case, the blood supply from both the right and left sides appeared to be related to the medial position of the right pelvic kidney. As the incidence of unilateral renal ectopia is not extremely low, it is possible to encounter in a surgical or cancer treatment case. Variations in the positional anatomy of the kidney and its vascular supply are of clinical importance and our case illustrates a different kind of blood supply that a pelvic kidney may possess.

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.

Guglielmi detachable coil treatment of a partially thrombosed giant basilar artery aneurysm in a child.

We report a partially thrombosed giant of the aneurysm basilar artery with prominent mass effect, diagnosed in an 11 year-old child who presented with neurological deficits due to brain stem compression. After the patent portion of the aneurysm was embolised with Guglielmi detachable coils, remarkable clinical improvement occurred. Angiography demonstrated complete occlusion of the aneurysm and MRI revealed dramatic shrinkage of the aneurysm at 6-month and 1-year follow-up.

Renal vascular abnormalities in Bardet-Biedl syndrome.

Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder. Specific diagnostic criteria for BBS have now been defined. At least four of the five cardinal signs of mental retardation, obesity, hypogenitalism in men, distal limb anomalies, and progressive tapetoretinal degeneration of the retina are required for the diagnosis. Renal involvement has been described as a sixth cardinal feature. Chronic renal failure occurs in 30%-60% of patients. Hypertension has been noted in 50%-66% of cases. Renal abnormalities reflect a defect in maturation of the kidneys. We present a patient with BBS who had bilateral microaneurysms and occlusions in renal arterioles.

Treatment of a TIPS-biliary fistula by stent-graft in a 9-year-old boy.

We report a 9-year-old male cirrhotic patient with acute occlusion of a transjugular intrahepatic portosystemic shunt (TIPS) due to a biliary-to-TIPS fistula which occurred 9 hr after the TIPS procedure. Immediate TIPS revision was performed and the fistula was treated by placement of an endoluminal stent-graft. At 12-month follow-up color Doppler examination demonstrated a patent shunt.

Arterial thrombosis leading to intestinal infarction in a patient with Behçet's disease associated with protein C deficiency.

Behçet's disease may be a possible cause of both occlusive and aneurysmal arterial involvement as well as recurrent venous thrombosis. A case of Behçet's disease complicated with vascular involvement leading to intestinal infarction is presented. A 41-yr-old man suffering from Behçet's disease for 15 yr presented with a 2-day history of severe abdominal pain and bloody diarrhea. Intestinal infarction secondary to thrombosis of the superior mesenteric artery had been diagnosed during surgical exploration 3 yr previously. He was started on anticoagulation with nutritional support. The patient was readmitted with severe diarrhea and malabsorption symptoms 3 yr after intestinal resection. A thrombus located in the posterior wall of the infrarenal portion of aorta was detected by aortography and ultrasonography. Although thrombosis is a relatively common complication of Behçet's disease caused by vasculitis, protein C deficiency, which is a pertinent laboratory finding in this case, might be a secondary factor in the thrombotic event. This is the first case reported of mesenteric artery thrombosis leading to bowel infarction and abdominal aorta thrombosis associated with protein C deficiency.

One calyx one ureter.

A case of duplication of the renal collecting system in which a single calyx is drained by a single ureter is presented. A second ureter draining a single calyx was not reported in the literature before.

Does hepatic vein outflow obstruction contribute to the pathogenesis of hepatocellular carcinoma?

Hepatocellular carcinoma (HCC) is one of the more common malignant diseases in the world. Here we have investigated role of hepatic venous outflow obstruction in the development of HCC. During a 10-year period from November 1986 to December 1996, 1,748 patients with clinical evidence of either portal hypertension, hepatic venous outflow obstruction, or inferior vena cava obstruction without Behçet's disease (BD) and 512 patients with Behçet's disease were examined at Hacettepe University Hospital. The presence of and the effect of hepatic venous obstruction on the subsequent development of HCC was assessed. In each case, hepatic vein thrombosis was assessed by hepatic venography and by digital subtraction angiography (DSA), computed tomography (CT), ultrasonography (US), and liver biopsy. Coagulation factors, including protein C, protein S, anti-thrombin III, and routine laboratory studies assessing the coagulability of blood were also investigated. The role of hepatic venous outflow obstruction on the subsequent development of HCC was determined by periodic laboratory investigations that included alpha-fetoprotein (AFP), ultrasonography, and when indicated liver biopsy. During the same time period all patients diagnosed as having HCC were investigated to identify all potential etiologic factors responsible for the HCC. Fifty-five (10.7%) of the 512 patients with BD were found to have one or more large vein thromboses. Sixteen of these 55 (29%) patients had hepatic vein thrombosis. During the follow-up period HCC developed in 2 of these 16 patients (12.5%), 34 and 21 months after a diagnosis of hepatic vein thrombosis was established. Forty patients from a total of 1,748 patients with clinical evidence of portal hypertension and cirrhosis, but without BD, were found to have evidence of hepatic vein thrombosis. Twenty-one of these 40 patients had an identifiable underlying disorder responsible for their hepatic vein thrombosis. Despite a full clinical and laboratory investigation in the other 19 patients, the etiologic factor responsible for the hepatic vein thrombosis remained obscure. Only one of these 19 patients, who also had portal vein thrombosis, developed HCC during a 9-year follow-up. Thus, a total of three of the 56 (5.36%) of cases of hepatic vein thrombosis developed an HCC. All of the hepatic tumors were of the multicentric, nodular, rapidly growing type. Despite the presence of hepatic vein thrombosis, there was no clear-cut histologic evidence for cirrhosis. Our experience suggests that hepatic vein thrombosis may be a contributing factor responsible for HCC development. Moreover, we advise that individuals with hepatic vein thrombosis should be assessed periodically for the development of HCC.

Budd-Chiari syndrome: a common complication of Behçet's disease.

OBJECTIVE: The Budd-Chiari syndrome is characterized by venous outflow obstruction of the liver, usually occurring as a consequence of thrombosis of the hepatic veins. Vasculitis is a major component of Behçet's syndrome. The aim of this study was to determine the incidence of hepatic vein thrombosis in patients with Behçet's disease and to estimate the effect of this entity upon the clinical features and course of Behçet's syndrome. METHODS: During an 8-yr period from 1985 to 1994, from a total of 493 patients with Behçet's disease seen at Hacettepe University Hospital, the incidence and effect of hepatic vein thrombosis on the clinical course of Behçet's syndrome was investigated. The hepatic vein thrombosis in each case was documented by hepatic venography and confirmed by digital subtraction angiography, computed tomography, ultrasonography, and liver biopsy. Coagulation parameters including protein C, protein S, and anti-thrombin III levels were easured in each case. The survival of cases with Behçet's syndrome complicated by Budd-Chiari syndrome and the effect of the Budd-Chiari syndrome on the survival of individuals with Behçet's syndrome were determined using the Kaplan-Meier technique. RESULTS: Of the 493 cases of Behçet's syndrome, 53 (10.8%) were found to have one or more large vessel thrombosis. Of these 53 patients, 14 (26.4%) had hepatic vein thrombosis. Of these 14 patients, 8 had an additional inferior vena cava thrombosis and 4 had portal vein as well as total inferior vena cava thrombosis. Only two patients with isolated hepatic vein thrombosis were identified. These two patients and two additional patients with hepatic vein thrombosis plus thrombosis of the hepatic portion of the inferior vena cava are currently alive. Of the 10 patients with total inferior vena cava and hepatic vein thrombosis (4 also had portal vein thrombosis), all 10 died with a mean survival of 10.3 months. During the same time period, 37 patients obtained from a total of 1494 patients with clinical evidence of either portal hypertension, hepatic venous outflow obstruction or inferior vena caval obstruction without Behçet's syndrome were found to have a Budd-Chiari syndrome. Of these 37 patients, 19 (51%) had an identifiable underlying disorder responsible for their hepatic vein thrombosis. CONCLUSION: Based upon this experience, it appears as if Budd-Chiari syndrome is a relatively frequent complication of Behçet's disease. When individuals with Behçet's syndrome have BCS, concurrent thrombosis of the portal vein and inferior vena cava are often found, if the patency of these vessels is assessed. The clinical course of patients with Behçet's syndrome complicated by Budd-Chiari syndrome is poor. The extent of the vascular thrombosis within the inferior vena cava rather than the presence of the hepatic vein thrombosis per se is the major determinant of survival.

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.