Ferhun Balkanci

Embolization of a high-output postnephrectomy aortocaval fistula with Gianturco coils and cyanoacrylate.

The authors describe the endovascular treatment of a high-output, large-caliber, postnephrectomy aortocaval fistula using a mixture of cyanoacrylate and lipiodol combined with Gianturco coil embolization. Thirtynine coils were used to decrease the flow through the fistula so that a fast-polymerizing glue mixture could be injected into the fistula. During rapid polymerization, the N-butyl-2-cyanoacrylate (NBCA) mixture was trapped within the coils, providing an easily controllable glue cast in the fistula, thereby preventing inadvertent embolization into the lungs. This approach can be of considerable benefit for the endovascular treatment of central high-output fistulas.

Is Congenital Hepatic Fibrosis a Pure Liver Disease

OBJECTIVES:An association between congenital hepatic fibrosis (CHF) and several different conditions is being increasingly recognized. We aimed to investigate, prospectively, these associated disorders and the clinical consequences for patients with CHF.MATERIALS AND METHODS:CHF was diagnosed using liver biopsy, abdominal ultrasound (US), Doppler US, upper endoscopy, and abdominal computed tomography (CT) in 19 patients (13 women, 6 men). CT portography and splenoportography with digital subtraction angiography were performed if indicated. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to investigate the extent of portal vein involvement of the common bile duct if it existed, to remove a stone located in the common bile duct when documented, and to confirm the diagnosis of Carolis syndrome. Cranial MRI was done when clinical findings suggested brain involvement.RESULTS:The mean age of the patients was 29.47 ± 12.06, ranging from 13 to 57. CHF-associated diseases were Carolis syndrome, polycystic kidney disease, cavernous transformation of the portal vein, Jouberts syndrome, von Meyenburg complex, polydactyly, medullary sponge kidney, and pancreatic duct atrophy. In two cases, cholangiocarcinoma had developed. There was only one case with pure CHF. Portosystemic shunt, TIPS, or splenectomy were performed in some cases to control bleeding from esophageal varices. Papillotomy and stone extraction from the common bile duct were performed in four patients with Carolis syndrome complicated by cholangitis. Three patients died of complications of CHF. Two patients with Carolis syndrome underwent liver transplantation.CONCLUSION:In this prospective study, it seems that CHF is not a pure liver disease but rather a multiorgan disorder involving the brain, portal vein, kidneys, and bile ducts. In most cases, the clinical picture includes other organ involvement, rather than purely the liver parenchyma.

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 Behçets disease constituted the major group (12/30) in the etiologic distribution. Although the vascular manifestations of Behçets 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 Behçets disease comprise the largest group (40%). The angiographic findings are not specific to Behçets 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 Behçets disease should be included among the diagnostic possibilities in a case of BCS, particularly in the countries in which it is prevalent.

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

Behçets disease may be a possible cause of both occlusive and aneurysmal arterial involvement as well as recurrent venous thrombosis. A case of Behçets disease complicated with vascular involvement leading to intestinal infarction is presented. A 41-yr-old man suffering from Behçets 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 malabsorbtion 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 Behçets 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.

JAK2V617F Mutation in Patients with Portal Vein Thrombosis

In a retrospective cohort, we investigated the presence of the JAK2V617F mutation in chronic non-cirrhotic portal vein thrombosis (PVT) patients, irrespective of the presence or absence of myeloproliferative diseases (MPDs). We identified 25 patients in whom thrombophilia workup was completed. The diagnoses of MPDs were made according to the World Health Organization (WHO) criteria. JAK2V617F mutation analysis was performed by allele-specific polymerase chain reaction (PCR). There were 9 male and 16 female patients. Prior to JAK2V617F analysis, there were one or more thrombophilic risk factors in 19 patients (76%). The JAK2V617F mutation analysis revealed the presence of this mutation (all in the heterozygote state) in six patients (24%; two male, four female). Five of the six cases with prior clinical diagnosis of MPDs were found to have wild-type JAK2. We found that the addition of JAK2V617F analysis into the thrombophilia workup in patients with chronic PVT contributes to a 4% increase in the diagnosis of thrombophilic conditions.

ENDOVASCULAR MANAGEMENT OF LIFE-THREATENING RETROPERITONEAL BLEEDING

Background:  The aim of this study was to evaluate efficiency of embolotherapy in management of retroperitoneal bleedings.

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 bi opsy. Behçets disease (BD) was found in 10 patients with BCS as an underlying disease. Two patients used oral contraceptive drugs, 2 had liver tumor hepato cellular carcinoma and liver lymphoma, and 1 patient had chronic lymphocytic leukemia. Despite full investigation, the authors could not find any obvious un derlying cause in the other 15 patients. The results suggest that (1) BCS must be considered as a possible complica tion in patients with Behçets 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 inci dence of Behçets 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.

Radioembolization for the treatment of unresectable liver cancer: initial experience at a single center.

PURPOSE Radioembolization with yttrium-90 microsphere (Y-90) therapy with SIR-Spheres (Sirtex Medical, Lane Cove, Australia) was approved by the Turkish Ministry of Health in April 2008. In this study, we present the preliminary experience at a tertiary care center with early follow-up results of Y-90 therapy, as well as a review of the related literature. MATERIALS AND METHODS Complete evaluation for radioembolization was performed in 10 patients (8 males, 2 females; mean age, 52.3 years) during an 8-month period at a single center, of which 9 were actually treated with SIR-Spheres(R). All patients underwent meticulous pre- and post-procedural imaging studies to document the therapy response. RESULTS; In order to isolate the target hepatic arterial circulation, following branches were embolized as they were considered as potential gastrointestinal shunts: the gastroduodenal artery (n = 5), right gastric artery (n = 1), and supraduodenal artery (n = 1). Radioembolization therapy could not be performed only in one patient because of a hepatogastric shunt of unknown origin. No significant hepatopulmonary shunting was identified (maximum, 9% shunting). The body surface area method was used to calculate the Y-90 dose in all patients (mean dose, 1.24 GBq). All patients had at least partial response of the targeted liver lesions, according to RECIST (Response Evaluation Criteria in Solid Tumors). CONCLUSION In comparison to chemoembolization, radioembolization has less systemic toxicity and can be performed as an outpatient procedure, which makes it more attractive to both patients and physicians. From our limited experience, the radioembolization procedure is a promising first-line treatment in unresectable liver cancer; randomized controlled multi-center studies, however, are needed.

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.

Imaging of iliac horns in Nail-Patella syndrome

Hereditary osteo-onychodysplasia, also known as the nail-patella syndrome (NPS), consists of a clinical tetrad of nail dysplasia, hypoplastic or absent patella, radial head dislocation, and iliac horns. Bilateral posterior iliac horns are observed only in NPS and present in >80% of patients. We describe the appearance, location, and structure of iliac horns by CT, MRI, and 3-dimensional CT reconstruction.