JBR 2010-5

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Volume 93 Page 235-284

September- October

Bimonthly

2010

DIAGNOSTIC AND INTERVENTIONAL IMAGING, RELATED IMAGING SCIENCES, AND CONTINUING EDUCATION

ORGANE DE LA SOCIETE ROYALE BELGE DE RADIOLOGIE (SRBR) ORGAAN VAN DE KONINKLIJKE BELGISCHE VERENIGING VOOR RADIOLOGIE (KBVR)


JBR-BTR ♦ 93/5 ♦ 2010 Journal Belge de ♦ Belgisch Tijdschrift voor ♦ RADIOLOGIE

Founded in 1907 A bimonthly journal devoted to diagnostic and interventional imaging, related imaging sciences, and continuing education Contents A review of the imaging and intervention of liver transplant complications. S. Mc Evoy, H. Stunell, T. Ramadan, N. Campbell, S. Barrett, T. Geoghegan, W.C. Torreggiani . . . . . . . . . . . . . . . . Pathologic assessment of non palpable probably benign breast masses at sonography : can instant intervention be avoided and is follow-up adequate? C. Yucesoy, N. Aydin Oktay, M. Oktay, S. Hucumenoglu, M. Alper, B. Hekimoglu . . . . . . . . . . . . . . . . . . . . . . . . . . MR imaging findings of haemophilic arthropathy of the elbow in children. L. Jans, M. Ditchfield, A. Gomez, S. Madhala, K. Verstraete . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . CT angiography evaluation of the renal vascular pathologies: a pictorial review. M. Oz, T. Hazirolan, B. Turkbey, A.D. Karaosmanoglu, M. Canyigit, B. Peynircioglu . . . . . . . . . . . . . . . . . . . . . . . . . Familial renal retroperitoneal lymphangiomatosis: personal experience and review of the literature. P. Antonopoulos, G. Charalampopoulos, F. Constantinidis, K. Tavernaraki, A. Skolarikos . . . . . . . . . . . . . . . . . . . . Metallic staples line mimicking a retained surgical sponge. L. Cardinale, C. Fava, N. Dervisci, N. Najada, P. Borasio, F. Ardissone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Wegener granulomatosis masquerading as pneumonia. D.J. Theodorou, S.J. Theodorou, K. Mpougias, M. Mastora, S. Stofanaki, N.C. Akritidis . . . . . . . . . . . . . . . . . . . . .

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HISTORICAL ARTICLE The roots of radiology in Greece. Ch. S. Baltas, A.P. Balanika, N. Kelekis, I.V. Fezoulidisl . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267

IMAGES IN CLINICAL RADIOLOGY Epiploic appendagitis within a Spigelian hernia. B. Coulier, B. Broze . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Extra-adrenal retroperitoneal ganglioneuroma. E. Janssens, L. Van Hoe, T. De Beule, P. D’haenens . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Unusual vacuum phenomenon suggesting occult vertebral instability. B. Coulier, M. Theodorescu, B. Vander Elst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Terson’s syndrome. M. Eyselbergs, M.H. Voormolen, A. Snoeckx, P.M. Parizel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Pellegrini Stieda disease. J.B. De Vis, P. Kersemans . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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In memoriam Prof. E.G. Van de Velde . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Prof. L. Jeanmart . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . News from the Universities. Doctoral thesis J. Vandevenne . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Forthcoming courses and meetings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . News from the Museum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Classified services . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Grants of the RBRS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Instructions to Authors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Subscribers information . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Advertising index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

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The terms used for indexation of subjects were developed by the Radiological Society of North America (RSNA) over a period of years. Their use here is by permission of the RSNA. The terms may not be used in any other index, print or electronic, except by specific permission of RSNA. ◆◆ Indexed in Index Medicus and in Zentralblatt Radiologie. Evaluated for Medline User, EMBASE and CANCERNET. Abstracted in Excerpta Medica Journals. ◆


JBR–BTR, 2010, 93: 235-241.

A REVIEW OF THE IMAGING AND INTERVENTION OF LIVER TRANSPLANT COMPLICATIONS S. McEvoy1, H. Stunell1, T. Ramadan1, N. Campbell1, S. Barrett1, T. Geoghegan2, W.C. Torreggiani1 Liver transplantation has become a successful surgical solution to a variety of medical and oncological parenchymal liver diseases. As a result, these patients are being encountered more frequently within diagnostic imaging departments which may be remote from the transplant centre. Radiologists must therefore be proficient in identifying normal post-transplant anatomy which involves the anastomosis of four structures between the donor and recipient, namely the hepatic artery, the main portal vein, the retro-hepatic inferior vena cava and the extra-hepatic bile ducts. A number of potential complications can arise involving any or all of these structures, which can be potentially devastating and lead to graft failure. Radiologists must familiarise themselves with the normal post-operative appearances of liver transplantation and become competent in diagnosing post-transplant complications. Where possible, complications should be treated using interventional radiological techniques, thus avoiding the need for repeat surgical intervention or retransplantation. Key-word: Liver, transplantation.

Since the first liver transplant was performed in Colorado in 1963, advances in surgical techniques, perioperative and postoperative care, and immunosuppression have made this procedure a successful surgical solution to a wide range of medical and oncological parenchymal liver diseases. As transplantation has become widely available, organ supply has become the major limiting factor, although transplant research continues to extend boundaries to combat this through initiatives such as split-liver transplanting and living lobe donation. Such progress has resulted in transplant patients being frequently encountered in the hospital system, and in particular, within diagnostic imaging departments which may be remote from the transplant centre. Therefore radiologists must become proficient in identifying both the normal posttransplant anatomy and potential associated complications. Complications of liver transplant may be classified into mechanical and nonmechanical. Nonmechanical complications include graft rejection, disease recurrence and complications of immunosuppresion, with imaging playing a limited role in the investigation of these complications. Mechanical complications refer to technical problems involving anastomotic structures. Liver transplantation surgery involves the anastomosis of four anatomical structures

between the donor and recipient, namely arterial (hepatic artery) anastomosis, venous inflow (portal vein) anastomosis, venous outflow (hepatic vein and inferior vena cava) anastomosis, and biliary anastomosis. A number of complications can arise involving any of these structures leading to graft failure, and radiology thus plays a crucial role in the diagnosis and potential treatment of these complications. Non-invasive imaging of liver transplant Non-invasive imaging of the posttransplant liver is performed using ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI). Ultrasound utilising both grey-scale assessment of the liver parenchyma and biliary tree and Doppler evaluation of hepatic and portal vasculature, is an ideal initial screening tool. Its widespread availability and ease of use, in addition to its lack of ionizing radiation make it an ideal imaging modality for use in these patients. Furthermore, it offers real-time imaging and can be performed perioperatively or at the bedside post-operatively. The quality of the images depends on patient size, available windows, and operator experience. For optimal views a curved transducer is used and transabdominal, subcostal and intercostal

From: 1. Department of Radiology, Adelaide & Meath Hospital, Tallaght, Dublin, Ireland 2. Department of Radiology, University of British Columbia Hospital, Vancouver, Canada. Address for correspondence: Dr W. Torreggiani, M.D., Department of Radiology, Adelaide & Meath Hospital, Tallaght, Dublin 24, Ireland. E-mail: William.torreggiani@amnch.ie

windows are scanned (1). The normal hepatic artery waveform on Doppler US shows a rapid systolic upstroke with continuous diastolic flow (Fig. 1). The normal portal vein Doppler waveform is a continuous flow pattern towards the liver with mild respiratory induced velocity variations (2). In cases where ultrasound images are suboptimal or inconclusive, further imaging using CT or MRI may be warranted. Multidetector CT (MDCT) has diffused rapidly into clinical imaging practice in recent years and compared with single-detector scanners, it allows faster image acquisition and multiplanar and 3D reconstruction. Imaging uses both the vascular and parenchymal phases of contrast material enhancement to provide a combined angiographic and organ-directed study (3). MR imaging including MR cholangiopancreatography (MRCP) and MR angiography also provide a comprehensive evaluation of the transplanted liver including its vascular structures and bile ducts. An important consideration is the relatively low toxicity of its contrast agents and the lack of associated radiation exposure. Invasive procedures such as conventional angiographic and cholangiographic studies are now generally reserved for nonsurgical treatment of certain complications. The transplant hepatic artery Vascular mechanical complications occur most frequently in the transplanted hepatic artery and may be divided into steno-occlusive and nonocclusive. Steno-occlusive is a collective term describing arterial obstruction including hepatic artery thrombosis, stenosis and kinking.


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Fig. 1. — The normal hepatic arterial waveform demonstrates a rapid systolic upstroke with continuous diastolic flow. The systolic acceleration time (SAT) is the time from end-diastole to the first systolic peak should be less than 0.08 seconds.

Hepatic artery thrombosis Hepatic artery thrombosis is the most common arterial complication, occurring in 5-12% of adult transplants and twice as many paediatric transplant recipients (4, 5). Causes may be related to the surgical technique, prolonged operative and cold ischaemia time of the liver, ischaemia reperfusion injury, coagulation abnormalities, secondary cholangitis, or periarterial inflammation secondary to prior transarterial chemoembolisation. The clinical spectrum of hepatic artery thrombosis ranges from mild abnormalities of liver biochemistry to fulminant hepatic necrosis and graft failure. It is associated with a significant mortality of up to 60% if left untreated and is the one of the leading causes of graft failure in the early postoperative period (5, 6). Early hepatic artery thrombosis occurs within one month of transplantation. Diagnosis can be accurately made using Doppler US and spectral analysis, identifying absent arterial flow with 90% sensitivity (7). Absent diastolic flow or dampening of the systolic flow may indicate impending thrombosis. Collaterals resulting in intrahepatic flow may produce false negative studies while complete thrombosis leads to the total absence of both proper hepatic and intra-hepatic flow. Newer techniques such as CT angiography may be used to evaluate vascular patency and parenchyma followed by formal hepatic artery angiography if thrombolysis proce-

Fig. 2. — Coeliac axis angiogram in the arterial phase demonstrating filling defect just distal to the origin of the common hepatic artery.

dures are contemplated (Fig. 2). Treatment options include retransplantation or revascularisation via surgical exploration with thrombectomy or anastomotic revision, transcatheter thrombolysis or systemic anticoagulation. Transcatheter thrombolysis is controversial due to the high risk of intra-abdominal bleeding reported in the limited number of case studies on this procedure (8). The majority of patients will require retransplantation (5). If conservative management is employed radiological intervention can used to treat secondary complications of ischaemia as they occur, namely biliary necrosis requiring percutaneous transhepatic cholangiography and liver parenchymal breakdown and abscess formation requiring percutaneous drainage. Hepatic artery stenosis Hepatic artery stenosis occurs in approximately 5% of liver transplants, most frequently at the anastomotic site (9). Causes include clamp injury, intimal injury due to perfusion catheters, or anastomotic ischaemia. It is classified based on the anatomical relationship to the anastomosis as proximal, anastomotic or distal (intra-hepatic or extra-hepatic). Digital subtraction angiography is the gold standard diagnostic modality (Fig. 3). Both Doppler US and multidetector CT angiography may also be used to accurately depict areas of hepatic artery stenosis.

The consequences of haemodynamically significant hepatic artery stenosis (>50% stenosis) are essentially identical to those of hepatic artery thrombosis. However in contrast to hepatic artery stenosis, reestablishment of arterial flow is of value even in cases of established ischaemia. Treatment should be instigated before the onset of biliary necrosis. Treatment options include anticoagulation, surgical revascularisation and re-transplantation. Revascularisation techniques require a multidisciplinary approach including endovascular and open surgical management. First line interventional radiological procedures are used to treat amenable stenoses and percutaneous transluminal angioplasty can maintain hepatic arterial patency in 60-70% of cases. Access is obtained using a 67 Fr vascular sheath and 3000-5000 IU of Heparin are given prior to crossing the stenosis with a 0.0140.035 inch guidewire (Fig. 4). Angioplasty is performed distal to proximal using a 4-6 mm balloon for adult patients and a 2-2.5 mm balloon for paediatric patients. The rate of intraprocedural complication occurrence is 7-10%, with more complex lesions, including kinked, tortuous, or multiple lesions, incurring an increased risk of complications and a higher failure rate (10, 11). Open surgical techniques are employed to treat technical failures or complications of endovascular techniques as well as treating complex lesions de novo.


LIVER TRANSPLANT COMPLICATIONS — MC EVOY et al

Fig. 3. — Coeliac axis angiogram with catheter at origin of coeliac axis demonstrates focal area of stenosis involving a short segment of the common hepatic artery.

Fig. 4. — Hepatic artery angiogram demonstrating 0.035 inch guidewire across focal area of stenosis which was successfully treated using balloon angioplasty.

Hepatic artery kinking Hepatic artery kinking refers to an acute arterial bend of the hepatic artery (Fig. 5) and occurs in 0.4% of transplant recipients. It is generally due to redundancy of donor or recipient vessels, and infrequently occurs because of external compression from surgical drains. Treatment depends on the cause. If kinking is secondary to arterial redundancy, surgical revision is advised. Endoluminal management of hepatic artery kinking is largely inadvisable due to the high risk of arterial dissection or subsequent thrombosis (11). Non-occlusive hepatic artery disease represents less than 5% of

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Fig. 5. — Hepatic artery angiogram with catheter at origin of coeliac axis demonstrates a focal, acute bend in the hepatic artery.

Fig. 6. — Doppler ultrasound demonstrating pseudoaneurysm of the intra-hepatic portion of the hepatic artery.

transplant artery complications. Examples include hepatic artery pseudoaneurysms and arteriovenous fistulae, and also non-occlusive diminished flow and arterial rupture. Hepatic artery pseudoaneurysm Hepatic artery pseudoaneurysm formation occurs in 0.4% of patients post liver transplantation. They are classified as either intra-hepatic (30%) (Fig. 6) or extra-hepatic (70%) (Fig. 7, 8) and are considered as distinct clinical entities. Intra-hepatic hepatic artery pseudoaneurysm formation typically affects the right lobe of the liver and is related to percutaneous biopsy procedures and drain placement. It is usually asympto-

matic and has no association with hepatic artery thrombosis and is managed conservatively with either transcatheter embolisation or percutaneous thrombin injection. Embolisation techniques utilize a coaxial microcatheter. Coiling of the aneurysm sac or selective embolisation of the distal or proximal aspect of the involved arterial branch is undertaken. There is an increased incidence of atrophy or necrosis of the involved hepatic segment relative to native livers. Extra-hepatic hepatic artery pseudoaneurysm formation is related to the surgical anastomosis and is considered to be the same clinical entity as mycotic pseudoaneurysm formation (arteritis). In contrast to intra-hepatic


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Fig. 7. — Contrast-enhanced axial CT demonstrates pseudoaneurysm of the extra-hepatic portion of the hepatic artery.

Fig. 9. — Doppler ultrasound of the portal vein demonstrating a focal area of stenosis of the middle branch of the portal vein.

denal and left gastric steal syndrome have also been reported (12). Diagnosis can be made accurately using angiography and treatment options include embolisation of the ‘steal’ artery. The transplant portal vein

Fig. 8. — Hepatic artery angiogram demonstrating pseudoaneurysm formation of the hepatic artery.

hepatic artery pseudoaneurysms, they are frequently symptomatic and may present with fever, gastrointestinal bleeding related to haemobilia and biliary obstruction and are frequently associated with the development of subhepatic collections. Extra-hepatic hepatic artery pseudoaneurysm formation is associated with hepatic artery thrombosis in up to 44% of cases. Arterial steal syndrome Arterial steal syndrome is a recently described complication of

orthotopic liver transplantation characterized by arterial hypoperfusion of the graft resulting from a shift in blood flow into other arteries that originate from the same trunk. The pathogenesis of the steal is unknown. It is a separate entity to HAS and HAT; it causes hypoperfusion secondary to reduced blood flow through the hepatic artery rather than obstruction. It is thought to be an under-recognised cause of graft ischaemia. Most cases of steal are associated with the splenic artery. However, cases of gastroduo-

Post-transplant portal venous complications, including stenosis and thrombosis, are relatively uncommon in comparison with arterial complications, yet they can be even more destructive (5). They occur more frequently in split liver and living related donor transplants. Stenosis usually occurs at the donorrecipient anastomosis and is most commonly seen in paediatric cases due to size mismatch between donor and recipient portal veins (13, 14). It most frequently occurs late following transplant (15). Most patients are asymptomatic, and are diagnosed on routine follow-up ultrasonography (Fig. 9). The diagnosis can also be made reliably using contrast enhanced CT (Fig. 10) or MRI. Patients with clinical evidence of portal hypertension require careful assessment to assess its significance. To measure portal venous pressure a right-sided percutaneous transhepatic approach is employed under ultrasound guidance and puncture of a second or third degree portal branch with a 22 guage needle


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Fig. 11. — Doppler ultrasound demonstrating thrombosis of the left branch of the portal vein.

Fig. 10. — Contrast enhanced CT scan of the liver demonstrating a focal stenosis of the portal vein.

is performed. The traditional management of portal vein stenosis has included reconstruction of the anastomosis, or retransplantation. However, percutaneous balloon angioplasty and tract embolisation has been shown to have a 71% patency at 24 months and improve survival in this population (13). Stent placement may be performed using a 10mm self-expanding stent in cases of residual or recurrent stenosis. The main complications that have been described with this procedure include haemoperitoneum and intrahepatic pseudoaneurysm (16). Portal vein thrombosis has an incidence of 1-2% post liver transplant and most frequently occurs in the early post transplant period (5, 17). Presentation can be with elevated transaminases, with signs of portal hypertension or thrombosis can be detected incidentally on US (Fig. 11). Treatment options include retransplantation, systemic anticoagulation, transcatheter thrombolysis and port-systemic shunting. Several case reports have validated the safety and efficacy of transjugular intrahepatic portosystemic shunt in recanalising the portal venous system post OLT (18-20). Surgical interventions remain more commonly utilised than interventional radiological techniques currently (5, 15).

The transplant venous outflow tract Venous outflow complications are relatively uncommon. Stenosis occurs more frequently in living donor and split liver transplants and hepatic vein stenosis occurs more commonly than inferior vena cava (IVC) stenosis (21). Hepatic vein stenosis may present with weight gain, ascites, hydrothorax, varices and may mimic portal hypertension. In the acute setting it generally arises from technical problems such as tight anastomosis, abnormal intimal flap or twisting of the veins. Stenosis presenting in delayed fashion is more likely to be secondary to perivascular fibrosis or intimal hyperplasia. Doppler ultrasound interrogation demonstrates decreased mean velocities in the hepatic veins and portal vein with accelerated flow and aliasing distal to the anastomosis and can be confirmed using venography. In one study of 26 patients suspected of having hepatic vein stenosis on Doppler ultrasound, only 20 (77%) had an abnormal gradient at subsequent venography (22). IVC stenosis occurs in < 2% of liver transplants and usually occurs above the hepatic vein anastomosis, thus presenting in an identical fashion to hepatic vein stenosis. Isolated IVC stenosis

below the hepatic veins is unusual and may present with lower limb oedema and ascites without hepatic dysfunction or hepatomegaly. CT with intravenous contrast can also accurately depict IVC stenosis post liver transplant (Fig. 12). Endovascular repair with balloon angioplasty or stent placement is safer than surgery and gives good results, although re-stenosis is frequent (22, 23). Angioplasty can be achieved via a transjugular, transfemoral, or direct transhepatic approach. The transplant biliary tract The most frequent mechanical complication of orthototic liver transplantation occurs within the transplant biliary tract; strictures and bile leaks occur in 10-35% of transplant patients (24). The majority occur in the first six months post transplantation. Biliary strictures may occur at the anastomotic site secondary to scar tissue causing retraction and narrowing of the common bile duct at the suture site (Fig. 13). Bile duct strictures occurring secondary to ischaemia involve only the biliary tree of the graft, starting at the hilum and progressing to the intra-hepatic bile ducts. These nonanastomotic strictures are associated with prolonged allograft cold time (25). Methods of imaging the transplant biliary tract include ultrasound, T-tube cholangiography, MR cholangiography and invasive procedures such as endoscopic retrograde cholangio-pancreatography (ERCP) or percutaneous transhepatic cholangiography (PTC). Ultrasound is not the optimal imaging modality


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Fig. 12. — CT scan of the liver post I-V contrast demonstrates stenosis of the IVC. Fig. 14. — Percutaneous transhepatic cholangiogram (PTC) demonstrating balloon dilatation of CBD stricture occurring post liver transplant.

Fig. 13. — Image from MRCP study in a patient post liver transplant demonstrating a focal short segment of strictured common bile duct with post-stenotic dilatation.

to investigate for biliary strictures as many liver transplants do not develop bile duct dilatation even in cases of high grade stenosis (26). MRCP has been shown to be a reliable technique for detecting post transplant biliary complications as it can provide information about the biliary tract that is nearly as diagnostic as that offered by invasive procedures (27). The majority of strictures can be managed by percutaneous dilatation (Fig. 14) with or without transhepatic biliary drainage. Bile leakage carries a significant morbidity and most

Fig. 15. — CT post I-V contrast demonstrating large low attenuation fluid collection adjacent to the stomach representing a large bile collection or biloma.

commonly occurs at the site of T-tube placement. Small bile leaks may close spontaneously although surgical revision of the anastomosis is often necessary. Bile collections are easily visualised on ultrasound or CT (Fig. 15) and may be managed with percutaneous catheter placement and drainage. Post-transplant malignancy Liver transplant recipients are at increased risk of developing malignancy, especially non-Hodgkin’s lym-

phoma (Fig. 16) and squamous cell carcinoma and a high index of suspicion is necessary in evaluating these patients. Epstein Barr virus has been associated with post-transplantation lymphoproliferative disease in patients receiving cyclosporine therapy. In addition, recurrence of a primary malignancy e.g. hepatocellular carcinoma may occur in the transplanted liver. Conclusion Liver transplantation is a successful treatment for a variety of


LIVER TRANSPLANT COMPLICATIONS — MC EVOY et al

13.

14.

15.

16.

17.

18.

Fig. 16. — Axial contrast-enhanced CT of the abdomen demonstrating a large area of para-aortic lymphadenopathy in a patient two years post liver transplant. The final diagnosis was non-Hodgkin’s lymphoma.

parenchymal liver diseases. Advances in surgical technique, postoperative care and immunological therapy have decreased the previously high mortality and morbidity associated with the procedure, leading to an increase in the number of procedures performed. Radiologists play a vital role in the postoperative care of this patient group and must be familiar with both normal and abnormal transplant appearances in order to identify and treat associated complications. Where possible, complications should be treated using minimally invasive interventional radiological techniques, avoiding retransplantation, as these techniques are associated with a significantly lower mortality rate.

5.

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Transplantation. Transplantation Proceedings, 2006, 38: 3651-3655 Buell J.F., et al.: Long term venous complications after full size and segmental paediatric liver transplantation. Ann Surg, 2002, 236: 658-666. Woo D.H., et al.: Management of portal venous complications after liver transplantation. Techniques in Vascular and Interventional Radiology, 2007, 10: 233-239. Kyoden Y., et al.: Portal vein complications after adult-to-adult living donor liver transplantation. Transplant International, 2008, 21: 1136-1144. Glanemann M.: Portal vein angioplasty using a transjugular, intrahepatic approach for treatment of extrahepatic portal vein stenosis after liver transplantation. Transplant International, 2001, 14:48-51. Cavallari A., et al.: Treatment of vascular complications following liver transplantation: multidisciplinary approach. Hepatogastroenterology, 2001, 48: 179-183. Tepetes K., et al.: Portosystemic shunt for the treatment of portal vein thrombosis following orthotopic liver transplantation. Transplant International, 1994, 7: S117-118 Ciccarelli O., et al.: Transjugular intrahepatic portosystemic shunt approach and local thrombolysis for treatment of early posttransplant portal vein thrombosis. Transplantation, 2001, 72: 159-161. Lodhia N., et al.: Transjugular intrahepatic portosystemic shunt with thrombectomy for the treatment of Portal Vein Thrombosis After Liver Transplantation. Digestive Diseases and Sciences, 2010, 55: 529-534. Darcy M.D.: Management of venous outflow complications after liver transplantation. Techniques in Vascular and Interventional. Radiology, 2007, 10: 240-245. Kubo T., et al.: Outcome of percutaneous transhepatic venoplasty for hepatic venous outflow obstruction after living donor liver transplantation. Radiology, 2006, 235: 285-290. Zajko A.B., et al.: Percutaneous transluminal angioplasty of venous anastomotic stenoses complicating liver transplantation: intermediate-term results. J Vasc Intervent Radiol, 1994, 5: 121-126. Everson G.T., et al.: Immediate postoperative care. In: Transplantation of the Liver. 3rd ed. Lipponcott Williams & Wilkins, 2001: 131-162. McDonald V., et al.: Biliary strictures in hepatic transplantation. J Vasc Intervent Radiol, 1991, 2: 533-538. Keogan M.T., et al.: The role of imaging in the diagnosis and management of biliary complications after liver transplantation. AJR, 1999, 173: 215219. Boraschi P.: MR cholangiography in orthotopic liver transplantation: sensitivity and specificity in detecting biliary complications. Clinical Transplantation, 2009, DOI: 10.1111/j.13990012.2009.01190.x.


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PATHOLOGIC ASSESSMENT OF NON-PALPABLE PROBABLY BENIGN BREAST MASSES AT SONOGRAPHY: CAN INSTANT INTERVENTION BE AVOIDED AND IS FOLLOW-UP ADEQUATE? C. Yücesoy1, N. Aydin Oktay1, E. Öztürk1, M. Oktay2, S. Hücümenog˘lu2, M. Alper2, B. Hekimog˘lu1 Aim of the study: To evaluate the pathologic results, determine the negative predictive value of non-palpable probably benign lesions at ultrasound and asses whether follow-up is adequate and immediate biopsy can be avoided. Materials and methods: Four hundred and eight cases which were referred to our breast imaging unit between 2004 and 2008 for biopsy evaluation were enrolled into the study. Two hundred and thirteen probably benign solid masses are classified as BI-RADS 3 in 205 of the enrollees. All masses were sonographically detectable and were classified through the guidelines of BI-RADS lexicon for sonography before the final pathological examination. All pathologic results were evaluated and the negative predictive value, false negativity rate and 95% confidence interval were calculated. Results: Of the 213 masses, fine needle aspiration cytology was performed in 120. US-guided wire localization and eventual surgery were carried out in the remaining 93 masses. Finally, 211 of the punctured lesions turned out to be benign and only two malignant lesions were detected. The resulting negative predictive value was found to be 99.1% while the false negative rate value was 0.9%. Conclusion: With the results provided, we think that in the patients with sonographically detected probably benign breast lesions, short term follow-up seems to be a strong alternative to immediate biopsy with its reliable high negative predictivity as well as low false negativity. Key-words: Breast, biopsy – Breast neoplasms, US.

Although mammography is the most widely used modality for early detection of breast cancer, with current advances in high-frequency transducers, US has become an important method for breast imaging. Traditionally, sonography has been used for solid-cystic differentiation as an adjunct to mammography, but today US can also be used to describe reliable signs to differentiate between benign and malignant masses. In addition, recent reports have pointed out that cancer can be obscured by dense breast tissue, and combination of both mammography and US may result in more malignant lesions being detected than with a single method alone (14). The Breast Imaging Reporting and Data System (BI-RADS) developed by the American College of Radiology (ACR) in 1993 has mostly standardized the assessment and reporting of lesions on mammography. Both lesion description and patient management have become more consistent through the guidelines of BI-RADS for mammography. ACR also has developed a lexicon for breast masses for US to improve the efficacy of the modality and to standardize the lesion characterization

and reporting. The lexicon includes features such as shape, orientation, margin and posterior acoustic shadowing, and a solid mass is classified as probably benign (BI-RADS 3) with a circumscribed margin, oval shape and parallel orientation without posterior acoustic shadowing (5). Although for probably benign masses that are visible on mammography, follow-up is mostly accepted as a standard method and supported by scientific data (6-10), there are limited information about the outcome of probably benign lesions that are classified only on US and it has not been proven whether the follow-up is the best strategy (11-14). The aim of our study was to evaluate the pathologic results of nonpalpable probably benign masses (BI-RADS 3) that were classified only on US and to determine whether follow-up US is adequate and thus, immediate biopsy may be avoided. Materials and methods The study was approved by the ethics committee of the hospital. Four hundred and eight cases which were referred to our breast imaging unit between 2004 and 2008 for breast biopsy were classified. We

From: 1. Department of Radiology, 2. Department of Pathology S.B. Ankara Diskapi Training and Research Hospital, Ankara, Turkey. Address for correspondence: Dr Cüneyt Yücesoy, M.D., Mutluköy Sitesi 12. Sok. No. 17 Ümitköy Ankara, Türkiye 06530. E-mail: yucecun2000@yahoo.com

identified 213 probably benign solid masses classified as BI-RADS 3 went to pathologic evaluation in 205 patients and included in the study. The mean patient age was 44.2 years, with a range of 1874 years. As a routine protocol at our institution, either the patient is referred by the departments of our hospital or other centers for biopsy, US evaluation repeated on the admission day by one of the two radiologists who have more than 6 years of experience in breast imaging. All the masses are categorized based on the criteria of BI-RADS and noted on the biopsy procedure form of the patient. The sonographic evaluation was carried out using either GE Logiq S6 (GE Heathcare, Milwaukee, Wisc.) with a 7-12 MHz high frequency linear transducer or Toshiba, Powervision 6000 SSA-370A (Tokyo, Japan) with 6-11 MHz high frequency linear transducer. The following US criteria were used to define a probably benign solid breast mass: shape (oval or macrolobulated fewer than four); circumscribed margins of the lesion; width greater than height (long axis parallel to the skin surface); echogenicity (isoechoic or mildly hypoechoic); and no posterior acoustic shadowing (Fig. 1) (Table I). To be included in the probably benign category, a mass needed to meet all the criteria regarding shape, margins, echogenicity, axis, and posterior acoustic shadowing


ASSESSMENT OF PROBABLY BENIGN BREAST MASSES — YÜCESOY et al

Fig. 1. — US shows an oval, circumscribed, hypoechoic typical probably benign mass in a 28 year-old woman classified as BI-RADS category 3 which was diagnosed as fibroadenoma after FNAC.

Table I. — Sonographic criteria for probably benign masses. Shape Margin Echogenicity Orientation Lesion boundary Posterior echoes

oval circumscribed Iso-hypoechoic Parallel Abrupt interface Enhancement - no change (no shadowing)

mentioned above. Typically, benign masses (BI-RADS 2) such as cysts or intramammarian lymph nodes and suspicious malignant masses (BIRADS 4-5) were excluded from the study.

The patients were informed about the probable malignancy risk of the masses that were assessed on US. Informed consent of all the patients was obtained. Biopsy was achieved not more than 3 days after the bleed-

ing parameters were checked. Indications for biopsy were either family history and/or patient anxiety. The technique used for pathologic evaluation was either fine-needle aspiration cytology (FNAC) or USguided wire localization (USGWL) and surgical excision. Choice of the biopsy technique was based on the preference and experience of the radiologist, the surgeon, and the patient. Cytology was considered negative for malignancy, when a definite benign diagnosis (fibroadenoma, papilloma, cyst content etc.) or a negative result such as benign cytology or no evidence of malignancy, was established. In the patients with indefinite result of FNAC or inadequate sample, the FNAC was repeated. The routine strategy at our breast unit for probably benign lesions without a definite diagnosis with benign cytology is short-interval follow up and we schedule a follow up protocol every 6 months for at least 2 years for these patients. Statistical analysis The false negative rate (FNR), the negative predictive value (NPV) and 95% confidence interval (CI) were calculated by using SPSS statistical program version 14.0 for Windows. To address the study hypothesis that fewer than 2% of the masses were malignant, frequencies were described using percentages and a corresponding 95% CI was calculated.

B

A

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Fig. 2. – A. US demonstrates a circumscribed, well-defined, oval shaped solid mass with no posterior acoustic shadowing classified as probably benign in a 50-year-old woman. The needle is observed within the lesion during FNAC. B. Cytology of the lesion shows pleomorphic, hyperchromatic malignant cells in a disorganized architecture (pap x 200).


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A

B

Fig. 3. – A. Non-palpable solid mass classified as probably benign in a 61-year-old woman. Note that the A-P diameter of the mass is minimal increased in the second look. After USGWL and surgery pathology revealed infiltrative ductal carcinoma. B. Low magnification of tumor shows haphazardly arranged tubules infiltrating adjacent adipose tissue (HE x 100).

Results All the 213 masses classified as BIRADS category 3 were visible on US. FNAC was performed in 120, and USGWL and excision carried out in the remaining 93 masses. The procedure was repeated in 11 masses that were reported as inadequate material or indefinite results in the first FNAC, and 8 fibroadenomas and 3 benign cytology were determined in the second intervention. Of the 205 patients with the 213 probably benign masses, 198 had a single lesion and the other 7 had multiple lesions. Although some of the patients had mammography examinations and some of the masses were detectable on mammography, all the biopsy procedures were guided by US because all the masses were visible on sonography. The pathological evaluation of 213 probably benign masses revealed 211 benign and two malignant lesions. One of the patients with a malignant mass was a 50year-old woman whose malignancy was diagnosed with FNAC and whose histopathology was reported as infiltrative ductal carcinoma after surgery (Fig. 2A, B). The other patient with a malignant mass was a 61year-old woman with infiltrative ductal carcinoma determined by USGWL and surgery (Fig. 3A, B). Both malignant masses were visible on mammography, but for the convenience of the patients, both procedures were achieved with the guidance of sonography. The FNR was calculated as 0.9% considering two malignant cases. The pathological evaluation revealed fibroadenomas in 99, fibrocystic changes in 37, fat

necrosis in 2, intraductal hyperplasia and papilloma in 1, adenosis in 5, and benign cytology in 67 of the remaining benign masses. The NPV was determined as 99.1%. The results of the pathological evaluation are summarized in Table II. Of the 211 masses that were diagnosed as benign, 92 masses were completely removed by USGWL and surgical excision. In 52 of the 119 masses, the biopsy procedures of which was performed through FNAC, a definite benign diagnosis was obtained and in the remaining 67 masses, the pathological evaluation revealed benign cytology without a definite diagnosis. As it is a routine protocol at our breast imaging unit, for the group with benign cytology without a definite diagnosis a short-interval follow up was scheduled. However, only 41 of the 67 masses could be followed for a mean 16.6 months (range 6-36 months) and no morphological or dimensional changes and no late malignancy were determined during the follow-up period in this group. Discussion Although mammography is not the perfect tool of screening for breast cancer, it is the widely accepted modality for early detection of breast cancer. In addition, despite lack of scientific evidence that sonography can be used for breast cancer screening (15), US has nearly become a standard imaging procedure due to recent advances in ultrasound and transducer technology. Since it is known that US plays many different roles in breast imaging

such as solid-cystic differentiation or determination of the exact location of the masses, it is obvious that its major promising role is its ability of malignant versus benign discrimination and of characterization of the masses (2, 3). BI-RADS classified the breast masses into different categories according to their morphologic characteristics, and BI-RADS category 3 is defined as morphologic features suggesting that a lesion is probably benign (5). It has been estimated that the probability of being malignant is below 2%, and it has been suggested that these lesions may be managed with periodic imaging, which should lead to reduction of biopsy rates. This strategy has been mostly agreed and supported by scientific data for mammography (610); however, for US, BI-RADS have a shorter history and this has not been proved yet. To the best of our knowledge, there are few reports of the probably benign lesions determined on sonography. In the first of the two reports by Graf et al. on probably benign breast masses at US, 157 masses were classified as probably benign and in these 157 masses no cancer was diagnosed. In the second report, 448 probably benign masses were determined, and 445 probably benign masses were followed up. Of the 445 masses, 442 remained stable. Two masses that increased in size were fibroadenomas based on biopsy evaluation, and one mass became palpable and cancer was diagnosed. The FNR was 0.2% and NPV was 99.8 % (11, 12). In another study, Mainero et al. categorized 148 masses as BI-RADS 3 and


ASSESSMENT OF PROBABLY BENIGN BREAST MASSES — YÜCESOY et al

Table II. — Pathologic evaluation in BI-RADS 3 masses. FNAC

USGWL

Total

Fibroadenoma Fibrocytic changes Benign cytology Fat necrosis Adenosis Intraductal papilloma-hyperplasia Malignancy

44 7 67 1 – – 1

55 30 – 1 5 1 1

99 37 67 2 5 1 2

Total

120

93

213

FNAC: Fine-needle aspiration cytology USGWL: US guided wire localization.

Table III. — Review of the literature regarding BI-RADS category 3 lesions at US.

Graf et al. (1) Graf et al. (2) Mainero et al. Park et al. Present study

No of masses (BI-RADS 3)

No of malignancies

NPV (%)

FNR (%)

157 448 148 312 213

0 1 1 2 2

100 99.8 99.3 99.4 99.1

0 0.2 0.7 0.6 0.9

NPV: Negative predictive value FNR: False negative rate.

also declared a high NPV with a rate of 99.3% (13). Likewise, in a recently published report on the same subject by Park et al., of the 312 probably benign masses, 2 malignancies were found, and the NPV and FNR were reported to be 99.4% and 0.6% respectively (14). Compared with the limited literature on the issue, the results of our study were similar. Of the 213 masses, two malignancies were found in the histopathologic examination, and the NPV and FNR were calculated as 99.1% and 0.9% respectively (Table III). In the previous and the present studies based on BI-RADS category 3 lesions at US, the probability of being malignant has been reported to be below 2%, which is the same rate for mammography reported in previous studies Therefore, in the light of the results of the previous reports and the present study, it can be said that shortinterval follow up seems an adequate strategy for probably benign masses. The slight differences in statistical rates reported by similar studies are possibly due to the operator dependent nature of US and might be the interobserver variability in classifying the masses or various numbers

of masses included in the studies. The effect of inter and intraobserver variabilities was not calculated in the present study. However, considering the facts that two radiologists with more than 6-years of experience in breast imaging and the good intra and interobserver agreement stated in BI-RADS for US in the earlier reports (16, 17), observer related variability in this study may be disregarded. Another point of criticism in our study may be the high rate of pathology diagnosed through FNAC and surgical excision rather than the core needle biopsy. Surgical excision is an alternative diagnostic technique in the management of breast lesions. Although using of excision for diagnostic purpose is expensive and invasive, it is reliable for diagnosing all types of breast lesions (including atypical hyperplasia, papillary lesions, mucinous lesion and phyllodes tumor). Another issue is that, whether surgical excision affects sentinel lymph node biopsy results. Although there are conflicting reports about the prior excisional biopsy affecting sentinel lymph node biopsy, the recent thought in the clinical literature is that excision-

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al biopsies have no adverse impact on the process (18, 19). It is known that core needle biopsy is increasingly being used as a faster, less invasive, and less expensive alternative to surgical biopsy for the histologic assessment of breast lesions (20). The reliability increases and in order to achieve a high diagnostic efficiency, minimum three cores per lesion is advisable (20, 21). Core needle biopsy has also some benefits such as being familiar to pathologists during histopathological assessment of the material (especially if experienced cytopathologists are not accessible), and the high ability to discriminate invasive cancer from in-situ carcinoma. However core needle biopsy may lead to histological changes in the main lesion including hemorrhage, granulation tissue formation, hemosiderin deposition, fibrosis, foreignbody reaction and infarction. Besides reduction of tumor size is another consequence of core needle biopsy (22). There are many studies published on FNAC of the breast masses, especially in benign lesions that FNAC is sufficient and still maintain the validity (23-25). It is a reasonably rapid, less invasive and less expensive procedure. It is accepted to be very useful for evaluation of breast lesions by some authors especially for very small lesions, lesions located under the skin or close to the chest wall compared with core biopsy. On the other hand its ability to differentiate invasive cancer from in-situ carcinoma is limited and the tissue obtained may be inadequate to assess tumor grade and hormonal receptor status (26). Regarding the issues above, the patients with indefinite and inadequate results on FNAC underwent a second intervention, and the patients with benign cytology were scheduled for short-interval follow up for every 6 months in the present study. It is also clear that the selection of the biopsy procedure can be based on the personal experience or choice of the radiology-pathology team. Although the possibility of malignancy for BI-RADS category 3 lesions is below 2%, follow up seems essential even in benign cytology without a definite diagnosis obtained with FNAC. While no standard interval and duration has been established for benignity of probably benign lesions, a minimum 2 years has been commonly established as the optimal time (11, 12, 14). We also perform and suggest that the follow-


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up protocol must be scheduled at 6month intervals for at least 2 years for patients with benign cytology without a definite diagnosis if FNAC was performed and for patients who do not undergo biopsy due to probably benign masses on US. Unfortunately, the mean follow-up period was under 2 years in the present study, and it was speculated that pathology results of the masses with benign cytology were explained to the patients by physicians as goodnatured, which led to decrease in the number of control visits. We believe that the patients should be warned about the probability of malignancy and the importance of strict followup, especially if there was not a definite diagnosis. In conclusion, the present study with a high NPV and a low FNR compatible with the limited literature confirmed that in circumscribed solid masses, which fulfill the criteria for BI-RADS category 3, short-term follow up is an adequate alternative strategy to immediate histopathologic examination. However, further studies with larger series and multicenter trials are needed to confirm efficacy of follow-up and to define the exact value of US in breast cancer. References 1. Baker J.A., Soo M.S.: Breast US: assessment of technical quality and image interpretation. Radiology, 2002, 223: 229-238. 2. Stavros A.T., Thickman D., Rapp C.L., Dennis M.A., Parker S.H., Sisney G.A.: Solid breast nodules: use of sonography to distinguish between benign and malignant lesions. Radiology, 1995, 196: 123-134. 3. Rahbar G., Sie A.C., Hansen G.C., et al.: Benign versus malignant solid breast masses: US differentiation. Radiology, 1999, 213: 889-894. 4. Crystal P., Strano S.D., Shcharynski S., Koretz M.J.: Using sonography to screen women with mammographically dense breasts. AJR, 2003 , 181: 177-182 .

JBR–BTR, 2010, 93 (5) 5. American College of Radiology: Breast imaging reporting and data system, breast imaging atlas. 4th ed. Reston, Va: American College of Radiology, 2003. 6. Sickles E.A.: Periodic mammographic follow-up of probably benign lesions: results in 3,184 consecutive cases. Radiology, 1991, 179: 463-468. 7. Varas X., Leborgne F., Leborgne J.H.: Nonpalpable, probably benign lesions: role of follow-up mammography. Radiology, 1992, 184: 409-414. 8. Sickles E.A.: Probably benign breast lesions: when should follow-up be recommended and what is the optimal follow-up protocol? Radiology, 1999, 213: 11-14. 9. Varas X., Leborgne J.H., Leborgne F., Mezzera J., Jaumandreu S., Leborgne F.: Revisiting the mammographic follow-up of BIRADS category 3 lesions. AJR, 2002, 179: 691-695. 10. Vizcaino I., Gadea L., Andreo L., et al.: Shortterm follow-up results in 795 nonpalpable probably benign lesions detected at screening mammography. Radiology, 2001, 219: 475-483. 11. Graf O., Helbich T.H., Fuchsjaeger M.H., et al.: Follow-up of palpable circumscribed noncalcified solid breast masses at mammography and US: can biopsy be averted? Radiology, 2004, 233: 850-856. 12. Graf O., Helbich T.H., Hopf G., Graf C., Sickles E.A.: Probably benign breast masses at US: is follow-up an acceptable alternative to biopsy? Radiology, 2007, 244: 87-93. 13. Mainiero M.B., Goldkamp A., Lazarus E., et al.: Characterization of breast masses with sonography: can biopsy of some solid masses be deferred? J Ultrasound Med, 2005, 24: 161-167. 14. Park Y.M., Kim E.K., Lee J.H., et al.: Palpable breast masses with probably benign morphology at sonography: can biopsy be deferred? Acta Radiol, 2008, 49: 1104-1111. 15. Kopans D.B.: Sonography should not be used for breast cancer screening until its efficacy has been proven scientifically. AJR, 2004, 182: 489-491. 16. Lazarus E., Mainiero M.B., Schepps B., Koelliker S.L., Livingston L.S.: BI-RADS lexicon for US and mammography: interobserver variability and positive predictive value. Radiology, 2006, 239: 385-391.

17. Lee H.J., Kim E.K., Kim M.J., et al.: Observer variability of Breast Imaging Reporting and Data System (BI-RADS) for breast ultrasound. Eur J Radiol, 2008, 65: 293-298. 18. Yararbas U., Argon A.M., Yeniay L., Kapkac M.: Problematic aspects of sentinel lymph node biopsy and its relation to previous excisional biopsy in breast cancer. Clin Nucl Med, 2009, 34: 854-858. 19. Wong S.L., Edwards M.J., Chao C.: The effect of prior biopsy method and concurrent definitive breast procedure on success and accuracy of sentinel lymph node biopsy. Ann Surg Oncol, 2002, 9: 272-277. 20. Youk J.H., Kim E.K., Kim M.J., Oh K.K.: Sonographically guided 14gauge core needle biopsy of breast masses: a review of 2,420 cases with long-term follow-up. AJR, 2008, 190: 202-207. 21. Bolívar A.V., Alonso-Bartolomé P., García E.O., Ayensa F.G.: Ultrasoundguided core needle biopsy of nonpalpable breast lesions: a prospective analysis in 204 cases. Acta Radiol, 2005, 46: 690-695. 22. Chaiwuna B., Thorner P.: Fine needle aspiration for evaluation of breast masses. Current Opinion in Obstetrics and Gynecology, 2007, 19: 48-55. 23. Boerner S., Fornage B.D., Singletary E., Sneige N.: Ultrasoundguided fine-needle aspiration (FNA) of nonpalpable breast lesions: a review of 1885 FNA cases using the National Cancer Institute-supported recommendations on the uniform approach to breast FNA. Cancer, 1999, 87: 19-24. 24. Liao J., Davey DD., Warren G., Davis J., Moore AR., Samayoa LM.: Ultrasound-guided fine-needle aspiration biopsy remains a valid approach in the evaluation of nonpalpable breast lesions. Diagn Cytopathol, 2004, 30: 325-331. 25. Kocjan G., Bourgain C., Fassina A., et al.: The role of breast FNAC in diagnosis and clinical management: a survey of current practice. Cytopathology, 2008, 19: 271-278. 26. Lieske B., Ravichandran D., Wright D.: Role of fine-needle aspiration cytology and core biopsy in the preoperative diagnosis of screen-detected breast carcinoma. Br J Cancer, 2006, 95: 62-66.


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MR IMAGING FINDINGS OF HAEMOPHILIC ARTHROPATHY OF THE ELBOW IN CHILDREN L. Jans1,2, M. Ditchfield1, A. Gomez1, S. Madhala1, K. Verstraete2 Haemophilic arthropathy of the elbow is a rare cause of elbow pain in children and adolescents. The purpose of this study is to determine the MR appearance of the spectrum of lesions found in haemophilic arthropathy of the elbow at initial MR imaging. It is important to be aware of the early changes in this entity, since early diagnosis and treatment of the disease may prevent progressive joint destruction. Key-words: Children, skeletal system – Elbow, MR.

Haemophilic arthropathy (HA) of the elbow is an uncommon cause of elbow pain and swelling in children and adolescents. HA due to recurrent haemarthrosis is the most common musculoskeletal manifestation of haemophilia and one of the most disabling complications of this disease. Children with severe haemophilia suffer from recurrent and acute joint haemorraghe. The therapy consists of factor VIII or IX replacement infusions (treatment on demand). The aim of this therapy is to keep the deficient factor > 1% of its normal value to convert severe haemophilia into a milder form of the disorder. The importance of starting replacement therapy early has been stressed in recent literature (13). The aim of this study is to define the MR imaging features of HA arthropathy of the elbow at initial MR imaging. Materials and methods A retrospective study was performed in a pediatric tertiary care centre. Institutional ethics approval was obtained. Subjects with documented HA of the elbow were identified using a radiology information system database keyword search of final radiology reports over a 10 year period. Patients with haemophilia presenting with elbow lesions in the setting of acute musculoskeletal trauma were excluded (Fig. 1-5). If the subject had undergone more than one MRI examination, only the first set of MRI images was reviewed. Twelve

Fig. 1. — Early stage haemophilic arthropathy of the elbow in a 15 year-old boy. Sagittal T2 PD-weighted MR image. There is a small joint effusion of the elbow predominantly in the coronoid fossa (long arrow) and anterior elbow joint recess (short arrow).

patients were identified meeting inclusion criteria for the study. The mean age was 12.2 years (range 4.1–16.6 years). All patients had been diagnosed with haemophilia within

From: 1. Department of Medical Imaging, University of Melbourne, Royal Children’s Hospital, Melbourne, Vic, Australia, 2. Department of Radiology and Medical Imaging Ghent University Hospital, Gent, Belgium. Address for correspondence: Dr L. Jans, M.D., Royal Children’s Hospital, Department of Medical Imaging, Flemington Road, Parkville 3052, Melbourne, Vic, Australia. E-mail: lennart.jans@hotmail.com

the first age of life and received factor substitution therapy since. The images were reviewed by two pediatric radiologists. Both reviewing radiologists were not blinded to the original reported findings but used the report only as a means to identify patients for inclusion into the study. The presence of joint effusion and synovial hypertrophy was assessed. Synovial haemosiderin deposition was considered present if blooming artefact was evident on gradient


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A

Fig. 2. — Haemophilic arthropathy of the elbow in a 16-yearold boy. Sagittal PD-weighted MR image demonstrates joint haemarthrosis (small arrows). A large subchondral bone erosion of the olecranon is present (arrow).

echo MR imaging. Joint effusion was graded to be moderate or extensive (extension of all joint spaces). Synovial hypertrophy was graded to be small (< 2 mm), moderate (25 mm) or large (> 5 mm). Synovial haemosiderin deposition was determined to be present or absent. The presence of associated findings including osteochondral lesions, bone erosions, subchondral cysts and presence of loose bodies was analysed. Joint alignment, joint narrowing and epiphyseal overgrowth were assessed. MR examination All studies were performed on 1.5 Tesla system (Avanto, Siemens Medical, Erlangen, Germany) with the affected elbow imaged in a small flex extremity four- channel phased circular polarised or array coil (Siemens Medical, Erlangen, Germany). Sequences obtained during MR included sagittal, and coronal oblique fat-saturated T1-

B Fig. 3. — Haemophilic arthropathy of the elbow in a 13- yearold boy. A: Coronal FS T2-weighted MR image. An ill defined area of hyperintense signal abnormality is present on the articular side of the trochlea and ulna (small arrows), in keeping with focal edema. Extensive edema of the radial head is identified (long arrow). B: Coronal FS GRE MR image demonstrates small focal subchondral osseous defects in the articular side of the trochlea and ulna (short arrows), as well as an osteochondral defect of the medial aspect of the radial head (long arrow).


MR IMAGING FINDINGS OF HAEMOPHILIC ARTHROPATHY OF THE ELBOW — JANS et al

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B

Fig. 4. — Haemophilic arthropathy of the elbow in a 7-year- old boy. A: Sagittal PD-weighted MR image demonstrates extensive joint haemarthrosis (stars) and subchondral bone erosion in the olecranon (arrow) B: Sagittal FS PD-weighted MR image demonstrates subchondral bone in the capitellum of the humeral physis and in the radial head (arrows).

B

A

Fig. 5. — Haemophilic arthropathy of the elbow in a 4-year-old boy. A: Coronal FS PD-weighted MR image demonstrates haemosiderin deposition in the synovium of the elbow joint (long arrows). Haemosiderin is identified on the articular surface of capitellum and radial head (short arrow). B: Axial T2- weighted image demonstrates discrete hypointense signal return from the synovium in keeping with haemosiderin deposition (arrows). No synovial thickening is identified. Moderate joint effusion is present.


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Table I. — Petterson radiologic classification of haemophilic arthropathy. Possible joint score: 0-13 points. Radiologic change

Finding

Score (points)

Osteoporosis

Absent Present Absent Present Absent Slight Pronounced Absent < 50% > 50% Absent 1 cyst > 1 cyst Absent Present Absent Slight Pronounced Absent Slight Pronounced

0 1 0 1 0 1 2 0 1 2 1 1 2 0 1 0 1 2 0 1 2

Enlargement of epiphysis Irregularity of subchondral surface Narrowing of joint space Subchondral cyst formation Erosions at joint margins Incongruence between joint margins Deformity (angulation and/ or displacement of articulating bones)

Table II. — The Denver MRI scale classifying the arthropathy in different stages in relation to the most severe finding. The score is progressive, the maximum score is 10. Score

Stage

0 Normal joint Effusion/haemarthrosis 1 Small 2 Moderate 3 Large Synovial hypertrophy/haemosiderin 4 Small 5 Moderate 6 Large Cyst/erosion 7 One cyst or partial surface erosion 8 More than one cyst or full surface erosion Cartilage loss 9 < 50% 10 > 50%

weighted-images; axial and coronal oblique fat-saturated PD (TE = 27 ms) and 3D volumetric spoiled gradient echo sequence. No direct MR arthrogram or IV contrast administration was performed. Results No normal elbow joints were identified. In all patients, MR imaging findings in keeping with HA of the elbow were identified (Fig. 1-5). Joint effusion was identified in 10 patients (83%). Of these patients,

7 presented with moderate and 3 with large joint effusion. Synovial haemosiderin deposition was present in 10 patients (83%). Synovial hypertrophy was present in 11 patients (92%). The hypertrophy was small (< 2 mm) in 3 patients, moderate in 2 patients (2-5 mm) and extensive (> 5 mm) in 6 patients. Chondral lesions were identified in 11 patients (92%). Partial thickness cartilage loss was present in 6 patients; complete cartilage loss was present in 5 patients. Changes of subchondral bone or joint margins were identified in 7 patients (58%).

Subchondral cysts were present in 7 patients (58%), all demonstrating multiple cysts in at least one bone of the joint. No patients with articular loose bodies were identified. Bone marrow edema was present in 8 patients (75%). Focal bone marrow edema in only one bone was identified in 4 patients; extensive multifocal bone marrow edema was identified in 4 patients. The joint alignment was anatomic in 7 patients (58%) and abnormal in 5 patients (42%). In 3 patients, irregularity of the articular surfaces was identified. Severe misalignment of the elbow joint was demonstrated in 2 patients, with epiphyseal overgrowth in 1 patient. Discussion Our study has shown that MRI clearly depicts early changes in haemophiliac arthropathy of the elbow. In haemophilia, arthropathy predominantly occurs in only a few joints, most notably the knees, ankles and elbows (4). MRI delineates soft tissue, both cartilage and bone and is accurate in the detection of joint effusion and haemosiderin deposition in HA of the elbow. This is why MRI is a precise non- invasive tool for the assessment of early joint changes still undetectable by physical examination or conventional radiography in the haemophilic setting (5). Early diagnosis of HA of the elbow is mandatory, since it has been stressed that factor substitution therapy from early age decreases the number of rebleeds of the synovial and thus slows down the progressive severity of the arthropathy. Development of late stage disease, with radiosynoviorthesis and ultimately arthroplasty of the elbow, may be prevented (6-7). Several schemes for the radiological diagnosis, classification and follow- up of HA have been proposed. The Petterson scoring system of radiological evaluation of haemophilic arthropathy (Table I) is widely recognized (8). This scoring system, however, is based on radiographic findings only. It has been well demonstrated that MR depicts subtle early joint changes in HA that are not depicted by conventional radiography (5). Several MR scoring systems have been presented that entail two different strategies: the pathology is scored either according to the characteristic stages of development or


MR IMAGING FINDINGS OF HAEMOPHILIC ARTHROPATHY OF THE ELBOW — JANS et al

Table III. — The European MRI score given in the format A(e:s:h). The A component is the sum of values for changes in following categories: subchondral cysts, irregularity/erosion of subchondral cortex and chondral destruction. The statements are evaluated to weather they are present, for each present feature 1 point is added to the A component. The factors e (effusion), s (synovial hypertrophy) and h (haemosiderin deposition) are evaluated according to a five- point scale. The maximum score is 16 (4:4:4). Subchondral cysts (part of A) Present in at least one bone Present in at least two bones More than three cysts in at least one bone More than three cysts in at least two bones Largest size more than 4 mm in at least one bone Largest size more than 4 mm in at least two bones Irregularity/erosion of subchondral cortex (part of A) Present in at least one bone Present in at least two bones Involves more than half of joint surface in at least one bone Involves more than half of joint surface in at least two bones Chondral destruction (part of A) Present in at least one bone Present in at least two bones Full-thickness defect in at least one bone Full-thickness defect in at least two bones Full-thickness defect involves more than one third of the joint surface in at least one bone Full-thickness defect involves more than one third of the joint surface in at least two bones Effusion/Haemarthrosis (e) Hypertrophic synovial (s) Haemosiderin (h) 0 Absent 1 Equivocal 2 Small 3 Moderate 4 Large

by summation of specific changes, and thus are respectively ‘progressive’ and ‘additive’ methods. The Denver MRI scale shown in Table II represents a progressive scoring system, whereas the European MRI score shown in Table III represents an additive scoring system (9-10). In recent literature combined progressive and additive scales are been developed, resulting in ‘compatible scales’ (11). In HA of the elbow, other findings of clinical significance may be present. These features include sprain and tear of ligaments, strain of muscles, presence of soft tissue pseudotumor and late arthropathic degenerative changes. Our study did not focus on these associated findings. However, since these associated findings may be of importance in the clinical setting, awareness of these entities is mandatory.

Gadolinium enhanced MRI may play a role in the differentiation of synovial hypertrophy versus joint fluid. However, contrast administration in the pediatric age group remains a traumatic experience. Given the fact that non enhanced MR imaging provides information of the most common imaging features of HA of the elbow, arthrography was not routinely performed in this study. Conclusion The most common findings in haemophiliac arthropathy of the elbow consist of joint effusion, the presence of (osteo)chondral lesions, synovial hypertrophy and synovial haemosiderin deposits. MRI clearly depicts these early changes. Early diagnosis of joint haemorrhage alters the therapeutic management

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and may prevent progressive joint destruction and surgery. Acknowledgement The research was supported by the 2009 research grant of the Royal Belgian Radiology Society (RBRS).

References 1. Soler R., Lopez- Fernandez F., Rodriguez E., Marini M.: Haemophilic arthropathy. A scoring system for magnetic resonance imaging. Eur Radiol, 2002, 12: 836-843. 2. Pergantou H., Matsinos G., Papadopoulos A., Platokouki H., Aronis S.: Comparative study of clinical, x-ray and magnetic resonance imaging in evaluation and management of hemophilic arthropathy in children. Haemophilia, 2006, 12: 241247. 3. Utukuri M.M., Goddard N.J.: Haemophilic arthropathy of the elbow. Haemophilia, 2005, 11: 565-570. 4. Maclachlan J., Gough-Palmer A., Hargunani R., Farrant J., Holloway B.: Haemophila imaging: a review. Skeletal Radiol, 2009, 38: 949-957. 5. Dobon M., Lucia J., Aguilar C., Mayayo E., Roca M., Solano V., Pena S., Giralt M., Ferrandez A.: Value of magnetic resonance imaging for the diagnosis and follow-up of haemophilic arthropathy. Haemophilia, 2003, 9: 76-85. 6. Nuss R., Kilcoyne R., Rivard G.; et al.: Late clinical, plain x-ray and magnetic resonance imaging findings in haemophilic joints treated with radiosynoviorthesis. Haemophilia, 2000, 6: 658-663. 7. Chapman-Sheath P.J., Giangrande P., Carr A.J.: Arthroplasty of the elbow in haemophilia. J Bone Joint Surg, 2003, 85: 1138-1140. 8. Petterson H., Ahlberg A., Nilsson I.M.: A radiological classification of haemophilic arthropathy. Clin Orthop, 1980, 149:153-159. 9. Lundin B., Babyn P., Doria A., Kilcoyne R., Ljung R., Miller S., Nuss R., Rivard G.E., Petterson H.: Compatible scales for progressive and additive MRI assessments of haemophilic arthropathy. Haemophilia, 2005, 11: 109-205. 10. Funk M.B., Schmidt H., Becker S., Escuriola C., Klarmann D., Klingebiel T., Kreuz W.: Modified magnetic resonance imaging score compared with orthopaedic and radiological scores for the evaluation of haemophilic arthropathy. Haemophilia, 2002, 8: 98-103. 11. Doria A.S., Lundin D., Miller S., et al.: Reliability and construct validity of the compatible MRI scoring system for evaluation of elbows in haemophilic children. Haemophilia, 2008, 14: 303-314.


JBR–BTR, 2010, 93: 252-257

CT ANGIOGRAPHY EVALUATION OF THE RENAL VASCULAR PATHOLOGIES: A PICTORIAL REVIEW M. Oz, T. Hazirolan, B. Turkbey, A.D. Karaosmanoglu, M. Canyigit, B. Peynircioglu The emergence of CT angiography (CTA) has a groundbreaking impact on the evaluation of renal vessels and is gradually replacing the conventional catheter angiography as the standard imaging procedure. In this review, we aimed to describe the renal CTA technique and imaging findings of several renal arterial (i.e. atherosclerosis, fibromuscular dysplasia, aneurysms of the renal arteries, dissection, vasculitidis, follow-up of patients with renal arterial stent) and venous (i.e. nut-cracker syndrome, pelvic congestion syndrome) pathologies. Key-words: Renal veins, CT – Renal arteries, CT.

CT angiography, which is a rapid and non-invasive method, has become a widely used imaging modality in imaging of renal vascular pathologies (1, 2). The most common renal vascular pathologies include renal artery stenosis, renal artery aneurisms, dissection, vasculitiditis and fibromuscular dysplasia. At renal CT angiography, not only vascular pathologies, but also related secondary parenchymal alterations of kidneys such as infarction and atrophy can be demonstrated. Indications of renal CT angiography include imaging work-up of the diagnosis of occlusive diseases of the renal artery and related hypertension, follow up of balloon angioplasty or renal stenting, preoperative assessment of the patients that will undergo partial or total nephrectomy, post-operative followup, preoperative assessment of renal transplant candidate donors and acute onset flank pain in some occasions (3, 4). Image acquisition The diagnostic accuracy of renal CTA relies on the quality of the raw data acquired. MDCT protocol for the assessment of renal vessels consists of both unenhanced and enhanced CT scans (at arterial and venous phases). Unenhanced scan of kidneys with contiguous sections of 3-mm thickness is necessary for evaluation of vascular calcifications, and renal calculi. The optimal coverage for arterial phase scan should cover the area between the celiac artery and the terminal part of the main iliac artery (Fig. 1), but in patients with ectopic or transplanted kidney the

Fig. 1. — The coverage of the arterial phase, which should include abdominal aorta and main iliac arteries, where main and accessory renal arteries have their origins.

coverage can be increased. 1-1,5 mm thickness slices are obtained after intravenous injection of a 100-mL bolus of 300-400 mg/ml non-ionic iodinated contrast at a rate of 4cc/sec and 70-mL bolus at a rate of 56 cc/sec at 16 and 64-Channel MDCT scanners, respectively. Image acquisition is started after a delay of 4-5 and 6-7 seconds at 16 and 64-channel MDCT scanners, respectively after a

threshold enhancement of 100 HU is reached within the abdominal aorta under bolus tracking. For evaluation renal venous structures and abdominal viscera whole abdomen is scanned with a slice thickness of 5 mm following a delay of 60-80 sec after IV contrast injection. The scanning parameters are summarized in table I. Renal artery pathologies Renal artery stenosis

From: Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey. Address for correspondence: Dr B. Turkbey, Department of Radiology, Hacettepe University School of Medicine, 06500, Sihhiye, Ankara, Turkey. E-mail: bturkbey@yahoo.com

Occlusive diseases of the renal artery can result in hypertension, renal dysfunction and renal failure, ultimately. Renal artery stenosis


CT ANGIOGRAPHY EVALUATION OF THE RENAL VASCULAR PATHOLOGIES — OZ et al

Table I. — MDCT scanning parameters used in renal CT angiography. 16 MDCT

64 MDCT

Unenhanced Arterial Venous Unenhanced Arterial Venous KV/effective mass

120/140

140/140 120/140

120/160

120/150 120/160

Rotation time (sec)

0.5

0.5

0.5

0.5

0.33

0.5

Detector collimation (mm)

1.5

0.75

1.5

1.2

0.6

1.2

Slice thickness (mm)

3

1

5

3

1

5

Pitch

1.25

1.5

1.25

1.4

1.4

1.4

Reconstruction interval

3

1

5

3

0.5

5

Scan delay (sec)

0

4-5

60

0

7-8

60

Dose-length product (DLP) (mGy × cm)

6-7

5-6

Fig. 2. — 56-year-old female with hypertension. The left renal artery stenosis is seen on volume rendered CT image (arrow).

A (RAS) is responsible for secondary hypertension in 5% of adults and atherosclerosis is the most common etiology among elder population (> 55 years old), whereas fibromuscular dysplasia (FMD) is the most common cause among young individuals (5, 6). In atherosclerotic RAS, the stenosis typically occurs due to calcification and atherosclerotic plaque(s) localized to the proximal portion of the renal artery just close to the orifice, which can be bilateral

Fig. 3. — 36-year-old female with hypertension. Coronal CT angiography (A) and conventional angiography (B) images demonstrates the typical “rosary beads” pattern.

B

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in up to 30% of patients (7) (Fig. 2). The pathophysiology of the hypertension is the reduction in the renal perfusion associated with constriction of the renal artery resulting in the activation of the reninangiotensin-aldosteron pathway and the sympathetic nervous system, which ultimately leads to renovascular hypertension, renal dysfunction, even to renal failure (8). FMD is the second common cause of RAS, which is most commonly seen in females of 2nd to 5th decades (9). The distinction of FMD lesions from atherosclerotic disease is typically made by their location which is usually at mid to distal portion of the main renal artery. The disease is generally bilateral (10). In FMD, intima, media and adventitial layers of the vessel walls are all affected and in approximately 90% of cases; however, the media layer is mainly affected. The socalled “rosary beads”, representing


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A Fig. 4. — 37-year-old female with hypertension secondary to renal artery stenosis. Post stenotic dilatation (arrowhead) is seen at the distal flow tract of the stenotic segment in the middle left renal artery (arrow) at the coronal MIP images.

B Fig. 6. — Imaging follow-up after placement of an endovascular stent for right renal artery stenosis in a 51-year-old female with atherosclerotic stenosis. Curved-MPR image demonstrates the patency of the stent (arrow).

Fig. 5. — 56-year-old male with hypertension secondary to left renal artery stenosis. Coronal CT angiography image demonstrates the difference in perfusion and the dimensions between kidneys due to chronic left renal artery stenosis.

the alternating dilated and stenosed segments, is the typical imaging finding (10, 11). Even though there are publications stressing the excellent diagnostic accuracy of CT angiography (12); careful evaluation should be made in every patient and further imaging studies (e.g. catheter angiography) can be needed to con-

firm the diagnosis, specifically in young individuals and middle aged women (13) (Fig. 3). CT angiography is a non-invasive and reliable imaging modality in the detection of RAS with sensitivity values close up to 100% in diagnosis of the severe stenosis (> 50%) (14-16). Maximum intensity projection (MIP)

and volume rendering techniques are both useful and complementary in the assessment of the stenosis at CT angiography. Indeed, axial images are not solely sufficient due to the tortuous nature of arteries, their variable anatomical course and presence of accessory renal arteries (14, 16). Moreover, secondary signs of the arterial stenosis such as post-stenotic dilatation (Fig. 4), differences in the parenchymal perfusion and morphological alterations (atrophy, contour changes etc.) can also be accurately assessed on CT (Fig. 5). MIP images provide angiography-like


CT ANGIOGRAPHY EVALUATION OF THE RENAL VASCULAR PATHOLOGIES — OZ et al

Fig. 7. — 67-year-old male with atherosclerosis. Curved MPR CT angiography image demonstrates a calcified saccular aneurysm at the left renal hilum (arrow).

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A

B A

Fig. 8. — 48-year-old man with type B aortic dissection. Axial CT angiography images demonstrate an aortic dissection flap extending to right (A) and left (B) renal arteries (arrows).

images with an excellent overview of vascular anatomy and their variable projection angles should be used for accurate interpretation of stenotic lesions. Multiplanar and curved multiplanar reconstruction images are particularly useful for correct evaluation of the arterial luminal diameter for accurate depiction and quantification of the arterial stenosis, specifically in FMD.

B

Fig. 9. — 19-year old female with Takayasu arteritis. Axial MIP image demonstrates Takayasu arteritis affecting bilateral renal arteries (arrows) (A). Late venous phase image shows significant thickening of the aortic wall as an indicator of the active disease process (arrows) (B).


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A

A

B Fig. 10. — 48 year-old woman with a history of microscopic hematuria. The compression of the left renal vein at the aorticomesenteric forks in axial unenhanced (A) and reformatted oblique sagittal CT angiography images (arrows) (B).

The endovascular treatment with balloon angioplasty and/or stents is almost always the first therapeutic intervention for RAS. The outcome of endovascular treatment (stent lumen patency) can be accurately assessed at CT angiography by using raw and reconstructed images (Fig. 6). Acute occlusion of renal artery Acute renal infarction (ARI) is a cause of acute flank pain. The most common cause is thromboem-

B Fig. 11. — 39-year-old woman with an ongoing pelvic pain of two years. coronal MIP (A) and axial (B) Ct angiography images show the dilatation of the left ovarian vein (A) and pelvic varices (B) (arrows).

bolism. The most common clinical manifestation is acute flank or back pain. Hematuria, proteinuria, fever, leukocytosis and an elevated serum lactate dehydrogenase may also accompany. Parenchymal changes in the kidney depend on the size of the embolus, the location of the arterial occlusion, and its age. MDCT easily

demonstrates absence of enhancement in the affected renal tissue, which typically appears as wedgeshaped low attenuation areas (17). An additional CT finding of ARI is the cortical rim sign which represents preserved perfusion in the outer renal cortex which is supplied by renal capsular arteries (18).


CT ANGIOGRAPHY EVALUATION OF THE RENAL VASCULAR PATHOLOGIES — OZ et al

Renal artery aneurysms Renal artery aneurysms (RAA) are detected approximately in the 0.1% of the patients undergoing imaging studies, mostly in the 4th and 5th decades of the life (11, 19). The most common causes of RAA are atherosclerosis, polyarteritis nodosa, FMD and trauma (11). Morphologically, they can be saccular or fusiform in shape; a rim calcification can be also seen in 18% of them (19, 20) (Fig. 7). Less than 10% of the atherosclerotic aneurysms are symptomatic and again less than 5% of them undergo rupture, however the risk of rupture is higher in ones with a diameter of greater than 2 cm. Non-calcified RAAs and RAAs in pregnant patients are believed to be more prone to rupture (21, 22). Renal arterial dissections Renal artery dissection (RAD) is generally stenotic and/or occlusive in nature. Individuals with hypertension with an underlying atherosclerosis or FMD are generally more prone to have RAD. Aortic dissections may also cause constriction or occlusion by extending towards renal arteries. Acute dissections can occur as a complication of diagnostic and therapeutic catheter angiography; whereas chronic dissections may either be spontaneous or secondary to renovascular hypertension (23) (Fig. 8). Vasculitis Renal CT angiography is used as the first line renal vascular imaging modality in the diagnosis and followup of vasculitic conditions such as Takayasu disease, polyarteritis nodosa (PAN). In Takayasu disease, the thickening of the vessel wall and the luminal stenosis can be accurately assessed at CT angiography and detection of the contrast enhancement within the vessel wall can be postulated as the indicator of an active disease (Fig. 9). In PAN, microaneurysms at the interlobar or arcuate artery bifurcations, and infarct areas at different ages can be seen (24). Renal venous pathologies Nutcracker syndrome and pelvic congestion syndrome Nutcracker syndrome occurs secondary to occlusion of the left renal vein between the aorticomesenteric

fork (Fig. 10). The disease usually presents with asymptomatic hematuria due to increased peri-glomerular or peri-ureteral capillary pressure. In males, the reflux to the testicular vein might cause the varicosity of the pampiniform venous plexus, that may lead to secondary infertility; whereas, in females the retrograde venous flow into the ovarian veins may result in the varicose dilatation in the ovarian and parametrial venous plexuses (25). Pelvic congestion syndrome is characterized by the dilatation pelvic venous structures secondary to retrograde blood flow from the left renal vein. It may be the cause of pelvic pain which is more prominent in erect or semi-erect postures (26) (Fig. 11).

11.

12.

13.

14.

15.

Conclusion MDCT angiography is currently the main imaging method in assessment of the renal vasculature. It provides highly valuable and concluding data in patients with vascular pathologies involving the renal vessels non-invasively.

16.

17.

References 1. Kuszyk B.S., Heath D.G., Ney D.R., et al.: CT angiography with volume rendering: imaging findings. AJR, 1995, 165: 445-448. 2. Smith P.A., Fishman E.K.: Threedimensional CT angiography: renal applications. Semin Ultrasound CT MR, 1998, 19: 413-424. 3. Urban B.A., Ratner L.E., Fishman E.K.: Three-dimensional Volume-rendered CT Angiography of the Renal Arteries and Veins: Normal Anatomy, Variants, and Clinical Applications. Radiographics, 2001, 21: 373-386. 4. Fleischmann D.: Multiple detectorrow CT angiography of the renal and mesenteric vessels. Eur J Radiol, 2003, 45: 79-87. 5. Thomsen H.S., Sos T.A., Nielsen S.: Renovascular hypertension: diagnosis and intervention. Acta Radiol, 1990, 30: 113-120. 6. Pickering T.G.: Renovascular hypertension: etiology and pathophysiology. Semin Nucl Med, 1989, 19: 79-88. 7. Bookstein J.J., Maxwell M.H., Abrams H.L., et al.: Cooperative study of radiologic aspects of renovascular hypertension. JAMA, 1977, 237: 17061709. 8. Uder M., Humke U.: Endovascular therapy of renal artery stenosis: where do we stand today? Cardiovasc Intervent Radiol, 2005, 28: 139-47. 9. Youngberg J.P., Sheps S.G., Strong C.G.: Fibromuscular disease of the renal arteries. Med Clin North Am, 1977, 61: 623-641. 10. Harrison E.G. Jr, Hunt J.C., Bernatz P.E.: Morphology of fibro-

18.

19.

20.

21.

22. 23.

24.

25.

26.

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muscular dysplasia of the renal artery in renovasclar hypertension. Am J Med, 1967, 43: 97-112. Kadir S.: Angiography of the kidneys. In: Kadir S., eds. Diagnostic angiography. Philadelphia, Pa: Saunders, 1986, 445-495. Beregi J.P., Louvegny S., Gautier C., et al.: Fibromuscular dysplasia of the renal arteries: compari-son of helical CT angiography and arteriography. AJR, 1999, 172: 27-34. Das C.J., Neyaz Z., Thapa P., Sharma S., Vashist S.: Fibromuscular dysplasia of the renal arteries: a radiological review. Int Urol Nephrol, 2007, 39: 233-238. Johnson P.T., Halpern E.J., Kuszyk B.S., et al.: Renal artery stenosis: CT angiography-comparison of real-time volume rendering and maximum intensity projection algorithms. Radiology, 1999, 211: 337-343. Qanadli S.D., Mesurolle B., Coggia M., et al.: Abdominal aortic aneurysm: pretherapy assessment with dual-slice helical CT angiography. AJR, 2000, 174: 181-187. Coulier B.: Multidetector-row CT of renal arteries: review of the performances in normo- and hypertensive patients. JBR-BTR, 2005, 88: 311-321. Akpinar E., Turkbey B., Eldem G., Karcaaltincaba M., Akhan O.: When do we need contrast-enhanced CT in patients with vague urinary system findings on unenhanced CT? Emerg Radiol, 2009, 16: 97-103. Wong W.S., Moss A.A., Federle M.P., Cochran S.T., London S.S.: Renal infarction: CT diagnosis and correlation between CT findings and etiologies. Radiology, 1984, 150: 201-5. Tham G., Ekelund L., Herrlin K., et al.: Renal artery aneurysms: natural history and prognosis. Ann Surg, 1983, 197: 348-352. Tan W.A., Chough S., Saito J., Wholey M.H., Eles G.: Covered stent for renal artery aneurysm. Catheter Cardiovasc Interv, 2001, 52: 106-109. Rundback J.H., Rizvi A., Rozenbilt G.N., et al.: Percutaneous stent-graft management of renal artery aneurysms. J Vasc Interv Radiol, 2000, 11: 1189-1193. Yang J.C., Hye R.J.: Ruptured renal artery aneurysm during pregnancy. Ann Vasc Surg, 1996, 10: 370-372. Williams D.M., Lee D.Y., Hamilton B.H., et al.: The dissected aorta.III.Anatomy and radiologic diagnosis of branch vessel compromise. Radiology, 1997, 203: 37-41. Kawashima A., Sandler C.M., Ernst R.D., Tamm E.P., Goldman S.M., Fishman E.K.: CT evaluation of renovascular disease. Radiographics, 2000, 20: 1321-1340. Beard R.W., Highman J.H., Pearce S., Reginald P.W.: Diagnosis of pelvic varicosities in women with chronic pelvic pain. Lancet, 1984, 2: 946-949. Beard R.W., Reginald P.W., Wadsworth J.: Clinical features of women with chronic lower abdominal pain and pelvic congestion. Br J Obstet Gynaecol, 1988, 95: 153-161.


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FAMILIAL RENAL RETROPERITONEAL LYMPHANGIOMATOSIS: PERSONAL EXPERIENCE AND REVIEW OF LITERATURE P. Antonopoulos1, G. Charalampopoulos1, F. Constantinidis1, K. Tavernaraki1, A. Skolarikos2 Lymphangiomatosis of the kidneys and perirenal-retroperitoneal tissues is a rare disease of unknown etiology. We present two cases affecting members of the same family, supporting the familial nature of the disease. The natural history and related urological and systematic complications of the disease during a long-term follow-up are highlighted, while a comprehensive literature review is presented. Key-words: Lymphangiomatosis – Kidney, diseases.

Lymphangiomatosis of the kidneys and perirenal-retroperitoneal tissues is an extremely rare disease of unknown etiology. Currently, only Meredith et al. (1) have supported the familial nature of the disease, having found exacerbation of renal lymphangiomatosis during pregnancy in two sisters. We present the cases of two siblings affected by the disease, supporting its familial predisposition, and we highlight the diagnosis, the natural history and the potential complications related to this rare entity. A comprehensive literature review also enlightens the reader’s knowledge. Material and methods We present the cases of two siblings diagnosed with renal lymphangiomatosis. Following the diagnosis of the first case we have examined the whole family discovering one more affected patient. After establishing the diagnosis, we have followed the two patients to depict the natural history of the disease and its potential urological and systematic effects. Due to the rarity of the disease we have searched the Medline, Pubmed and EmBase databases to systematically review the literature published on this entity.

grade fever (37.3 ºC). Routine blood tests and blood pressure measurement were normal. Urinalysis revealed microscopic haematuria (12-20 RBC / HPF), and leucocyturia (WBC 20-50/HPF) without proteinuria. Urine culture was positive for Klebsiella for which antibiotic therapy was given. Abdominal ultrasonography (US) showed multiple parapelvic cystic lesions with bilateral low-grade dilatation of the renal pelvicalyceal systems. Abdominal computed tomography (CT) before and after the intravenous (IV) contrast material (CM) administration demonstrated multiple cystic lesions of low attenuation (0-10HU) in both renal pelvises. Similar lesions were also observed in the perirenal, pararenal and paraortic space. There was no contrast material uptake by the cysts. Some of the aforementioned cystic lesions demonstrated an irregular shape while others were tubular-shaped consistent with lymphangiectasias.

The lesions communicated with those on the contralateral side across the midline. The renal outline was not distorted and the renal parenchymal thickness was normal (Fig. 1). Based on the previously mentioned CT findings renalretroperitoneal lymphangiomatosis (RRL) was the likely diagnosis. It was confirmed by fine needle aspiration (FNA) of the retroperitoneal lesions which confirmed the fluid to be lymph. Following the establishment of diagnosis in patient A, her whole family (brother, sister and parents) was clinically examined and radiologically evaluated. A male sibling (patient B), aged 37, with a positive 17-year history of hypertension on ACE inhibitors, showed multiple bilateral parapelvic renal cystic lesions and a moderate dilatation of the pelvicalyceal system. Renal function was normal. Computed tomography revealed additional perirenal and paraortic lesions of various

Results In 1998, a 39 year-old woman (patient A), with a negative pastmedical and past-surgical history, presented to the emergency department with a right lower quadrant abdominal pain radiating to the hypogastrium. There were no other concomitant symptoms. Physical examination revealed diffuse abdominal tenderness and a low Fig. 1. — Transverse contrast enhanced abdominal CT (CECT) demonstrates a tubular low attenuation lesion in the anterior pararenal and paraFrom: 1. First I.K.A CT Department, Sismanoglio Hospital, Athens, 2. Athens Medical ortic space. There is a communication School, 2nd Department of Urology, Sismanoglio Hospital, Athens, Greece. between the pararenal lesions across the Address for correspondence: Dr P. Antonopoulos, M.D., Director of 1st I.K.A. CT midline. Parapelvic cysts are demonstratDepartment, Sismanoglio Hospital, Athens, Greece. ed in both kidneys. E-mail: axonikos@sismanoglio.gr, paopetros@yahoo.gr


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A Fig. 3. — CECT of patient A. In comparison to the previous CT (Fig 1) no changes were identified, apart from a slight increase in the size of the lymphangiectasias.

B

Fig. 4. — CECT section at the level of the upper pole of the left kidney shows dilatation and clubbing of the upper calyceal moiety (white arrow) with concomitant parenchymal thinning, findings consistent with focal chronic pyelonephritis. The patient suffered from frequent bouts of UTI.

C Fig. 2 A-C. — Abdominal CECT demonstrates dilated lymphatic vessels involving the right perirenal space and parapelvic cystic lesions in both kidneys. In comparison with previous CT examinations the parapelvic cysts increased in size. Perivascular lyphangiectasias surrounding the abdominal aorta and the inferior vena cava are also present. CECT demonstrated a moderate dilatation of the renal collecting system in both kidneys and a low attenuation lesion located in the right paravertebral space, extending to the thorax, representing dilated lymphatic vessels (white arrow).

shapes (lobulated, tubular-shaped) and of low attenuation (0-10HU), as well as a right paravertebral lesion with low attenuation which extended to the thorax. The pararenal lesions communicated with those on the contralateral side across the midline. Renal parenchymal thickness was normal (Fig. 2A-C). The aforementioned CT findings were diagnosed as lymphangiectasias and a diagnosis of familiar renal-retroperitoneal lymphangiomatosis (FRRL) was made. Based on the lack of urinary tract complications on both patients at presentation and the long-lasting hypertension of the male patient, no further treatment was decided and


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Fig. 5. — CECT of patient B at the level of the kidneys shows bilateral large parapelvic cystic lesions, compression of the contrast enhanced renal cortex and dilatation of the renal collecting systems (black arrows). In comparison with previous CT examinations the parapelvic cysts increased in size.

the patients were advised for regular follow-up. During the first two years of follow-up, patient A remained asymptomatic and no further changes in comparison to the previous CT were identified, apart from a slight increase in the size of the lymphangiectasias (Fig. 3). On occasion of recurrent urinary tract infections started in 2005 (2-3 bouts per year), an ultrasound confirmed the slight increase in the pelvicalyceal system dilatation, more prominent in the left kidney. In 2007, during a new UTI episode, CT scan findings were indicative of chronic pyelonephritis with clubbing of the upper calyceal moiety of the left kidney and concomitant parenchymal thinning (Fig. 4). A lesser pelvis fluid collection was also seen. The urine culture was positive for Escherichia Coli at that time for which the patient received the appropriate antibiotic therapy. A follow-up US performed one month later showed a marked decrease of the pelvic fluid collection. Patient’s B compliance to followup was not strict and he underwent a repeat CT scan in 2004, which showed thinning of the renal parenchyma, a greater bilateral pelvicalyceal dilatation as well as a slight increase in the size of the perirenal, paraortic and paravetebral lymphangiectasias in comparison to the previous CT examination performed six years ago (Fig. 5). Despite the worsened CT findings the patient’s renal function remained

stable. The patient declined a renogram and no further treatment was initiated, while he denied further radiological follow-up. Discussion RRL is a rare disease of unknown origin (1, 2). Although the familial nature of the disease has been previously inferred (3), the affection of siblings has been presented only once in the literature previously (1). Our report adds to the presumption of RRL’s familial nature, strengthens the anatomical findings and the extension of the disease, adds to the understanding of the natural history, and finally recognizes factors that may exacerbate the disease. Disease pathogenesis indicates that a failure of the developing lymphatic tissue to establish normal communication with the rest of the lymphatic system results in an abnormal lymphatic drainage with subsequent cyst formation (1, 4-9). These cysts are lined by endothelium (2), indicative of their vascular origin, and contain lymph and proteinaceous material (2). The anatomical distribution of renal lymphatics, its drainage to the retroperitoneal lymphatic system and the connections to the contralateral site explain the findings in RRL. Involvement of the intrarenal medullary and cortical lymphatics, of the big renal lymph node system and the paraaortic lymph nodes lead to lymphatic vessel obstruction and finally to the formation of parapelvic,

perirenal, pararenal, retroperitoneal and paravertebral cysts (8). Autopsy studies showed that kidney’s lymphatics also reach distant lymph nodes (10). This may explain the lack of anatomical consistency of findings in all patients as well as a cyst location distally to kidneys. The findings in our cases are relevant to the anatomical distribution and drainage of renal and retroperitoneal lymphatics, support the bilateral nature of RRL, while for the second time in literature show a midline connection between the pararenal lymphangiectatic lesions. The later can be explained by autopsy studies showing a connection across the midline between the anterior pararenal spaces in contrast to the perirenal spaces (11, 12). While RRL can be asymptomatic and incidentally diagnosed by imaging modalities (6, 13), clinical manifestations include abdominal pain and distention, a palpable abdominal mass (8, 13), and haematuria and/or proteinuria (2, 6). Early at diagnosis renal function tests are within normal limits (8). CT findings in combination with clinical and laboratory tests are considered sufficient to establish the diagnosis of RRL (2, 7, 14). FNA of the lesions may also be helpful, but it is not always diagnostic for the disease (9, 15). RRL’s differential diagnosis includes polycystic kidney disease (6, 8); tumors such as multilocular cystic nephroma; cystic renal cell carcinoma; lymphoma; sarcomas (5, 6); renal abscess; urinoma (6) and renal pelvis lipomatosis (2). Magnetic Resonance Imaging (MRI) is also considered a useful diagnostic tool for RRL and several imaging findings have been described (13, 16-18). Cystic lesions in RRL are demonstrated with the typical fluid signal intenstity, that is low on T1-weighted and high on T2-weighted images (16). However, varying signal intensities of cysts have also been described, probably due to different internal ratios of fat, fluid or chyle (17). MRI is also helpful in demonstrating thin retroperitoneal perivascular fluid-filled lymphatic channels[18). Furthermore, rare but possible complications of RRL may be depicted with MRI, such as haemorrhage inside the cysts, demonstrated as hyperintense foci on T1-weighted images, or cystic rupture with ascites representing an indirect finding (13, 16, 18). Advantages of MRI over CT include primarily the lack of patient


FAMILIAL RENAL RETROPERITONEAL LYMPHANGIOMATOSIS — ANTONOPOULOS et al

irradiation and also administration of iodinated intravenous contrast agent. Considering repeatability of imaging during follow up of patients with RRL as well as the possible contraindication of contrast enhanced CT due to potential renal impairment, MRI is probably considered safer than CT. Multiplanal MRI is also helpful in revealing the exact extent of renal and retroperitoneal disease as well as the non-parenchymal origin of the cysts (18). Additionally, MRI with MR urography (MRU) images obtained, with or without iv contrast administration, may evaluate both urinary tract morphology and renal function, while MR angiography (MRA) may yield information about renal vessels (18). The latter, is of great importance in patients with RRL complicated with arterial hypertension. The natural history of the disease has not been well documented due to its rarity. In our cases the followup was 11 years for the first patient and 6 years for the second patient, which constitutes the larger followup ever documented in literature. During the course of the disease increase in cyst size, infection of the cystic fluid (14), obstructive uropathy and abnormal renal function due to renal parenchymal compression from the cystic lesions (19), bilateral renal vein thrombosis (15), and perirenal fluid collections and ascites may develop (1, 14). Radiological findings of development of chronic pyelonephritis were also shown as a complication of the disease in our series. Although we were unable to unfold the etiology of longstanding hypertension in one of our cases, the latter may be secondary to the activation of rennin-angiotensin system due to ischemia caused by renal parenchyma compression from the cystic lesions (Page kidney) (20). The later explains the successful treatment of hypertension with ACE inhibitors in our case. Disease exacerbating factors are also not well known. Pregnancy was correlated with increase in perirenal fluid collection, superinfection of the fluid collections and the development of ascites (14). Our study shows for the first time in literature that urinary tract infection is another significant triggering factor of increase of parapelvic lymphatic vessels’ size, deterioration of the pelvicaliceal dilatation and increase in fluid collection. Asymptomatic RRL does not require treatment (13, 18). On the

other hand, percutaneous drainage (1, 14), laparoscopic cauterization (21) and surgical excision (2, 19) of the cystic lesions should be the treatment options in symptomatic patients. Surgery should be considered an option only in severe cases due to its high risk of nephrectomy and haemorrhage (2). Nephrectomy is no longer considered a choice, since in case of asymmetrical bilateral involvement, the cysts in the contralateral kidney may increase in size (2, 20). Antibiotic treatment is reserved for the case of superinfection of the lesions (14) and may lead to significant decrease of cystic and perirenal fluid accumulation in the case of a concomitant urinary tract infection, as shown in our case. Conclusions Renal lymphangiomatosis may be a disease of familiar origin. Most characteristic CT findings are parapelvic cystic lesions and perirenal and pararenal, hypodense lesions of various shapes, often tubular, corresponding to lymphangiectasias. During the course of the disease the dilated lymphatic vessels may increase in size, obstruct the urinary collecting system and in time result in chronic pyelonephritis and hypertension. Concomitant urinary tract infections may exacerbate the disease and necessitate appropriate treatment. References 1. Meredith W.T., Levine E., Ahlstrom N.G., Grantham J.J.: Exacerbation of familial renal lymphangiomatosis during pregnancy. AJR, 1988, 151: 965-966. 2. Murray K.K., McLellan G.L.: Renal peripelvic lymphangiectasia: appearance at CT. Radiology, 1991, 180: 455456. 3. Fis’kova L.B., Prokopchuk V.S.: Congenital bilateral lymphangiomatosis of the renal fibrous capsule. Urol Nefrol (Mosk), 1979, 54-56. 4. Younathan C.M., Kaude J.V. Renal peripelvic lymphatic cysts (lymphangiomas) associated with generalized lymphangiomatosis Urol Radiol, 1992, 14: 161-164. 5. Leder R.A., Frederich M.G., Hall B.P., Elenberger C.D.: Genitourinary case of the day. Renal lymphangiomatosis. AJR, 1995, 165: 197-198. 6. Varela J.R., Bargiela A., Requejo I., Fernandez R., Darriba M., Pombo F.: Bilateral renal lymphangiomatosis: US and CT findings. Eur Radiol, 1998, 8: 230-231. 7. Ramseyer L.T.: Case 34: renal lymphangiectasia. Radiology, 2001, 219: 442-444.

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8. Lindsey J.R.: Lymphangiectasia simulating polycystic disease. J Urol, 1970, 104: 658-662. 9. Davidson A.J., Hartman D.S.: Lymphangioma of the retroperitoneum: CT and sonographic characteristics. Radiology, 1990, 175: 507510. 10. Assouad J., Riquet M., Foucault C., Hidden G., Delmas V.: Renal lymphatic drainage and thoracic duct connections: implications for cancer spread. Lymphology, 2006, 39: 26-32. 11. Raptopoulos V., Lei Q., Touliopoulos P., Vrachliotis T., Marks S. Jr.: Why perirenal disease does not extend into the pelvis: the importance of closure of the cone of renal fasciae. AJR, 1995, 164: 11791184. 12. Raptopoulos V., Touliopoulos P., Lei Q., Vrachliotis T., Marks S. Jr.: Medial border of the perirenal space: CT and anatomic correlation. Radiology, 1997, 205: 777-784. 13. Mani N.B., Sodhi K.S., Singh P., Katariya S., Poddar U., Thapa B.R. Renal lymphangiomatosis: a rare cause of bilateral nephromegaly. Australas Radiol, 2003, 47: 184-187. 14. Ozmen M., Deren O., Akata D., Akhan O., Ozen H., Durukan T.: Renal lymphangiomatosis during pregnancy: management with percutaneous drainage. Eur Radiol, 2001, 11: 37-40. 15. Riehl J., Schmitt H., Schäfer L., Schneider B., Sieberth H.G.: Retroperitoneal lymphangiectasia associated with bilateral renal vein thrombosis. Nephrol Dial Transplant, 1997, 12: 1701-1703. 16. Kiresi D., Emlik D., Kivrak A.S., Odev K.: Retroperitoneal lymphangiectasia associated with mediastinal extension: Radiologic findings. Eur J Radiol Extra, 2007, 63: 61-63. 17. Cutillo D.P., Swayne L.C., Cucco J., Dougan H.: CT and MR imaging in cystic abdominal lymphangiomatosis. J Comput Assist Tomogr, 1989, 13: 534-536. 18. Kocaoglu M., Bulakbasi N., Ilica T., Somuncu I.: MRI findings of renal lymphangiectasia. J Magn Reson Imaging, 2005, 22: 681-683. 19. Chiu J.S., Wu C.J., Sun G.H., Lin S.H.: Obstructive uropathy associated with bilateral renal lymphangiomatosis. Nephrol Dial Transplant, 2004, 19: 2923. 20. Schwarz A., Lenz T., Klaen R., Offermann G., Fiedler U., Nussberger J.: Hygroma renale: pararenal lymphatic cysts associated with rennindependent hypertension (Page kidney). Case report on bilateral cysts and successful therapy by marsupialization. J Urol, 1993, 150: 953-957. 21. Wadhwa P., Kumar A., Sharma S., Dogra P.N., Hemal A.K.: Renal lymphangiomatosis: imaging and management of a rare renal anomaly. Int Urol Nephrol, 2007, 39: 365-368.


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METALLIC STAPLES LINE MIMICKING A RETAINED SURGICAL SPONGE L. Cardinale1, C. Fava1 , N. Dervisci, D. Najada1, P. Borasio2, F. Ardissone2 The inadvertent loss of surgical sponges remains a dreadful hazard of surgery. We report the case of a patient with a medical history of myotonic dystrophy type 1 who had received a right upper lobectomy for the treatment of a stage IIA (pT1N1M0) well differentiated neuroendocrine carcinoma. In the early postoperative period, aspiration of gastric contents occurred and the patient underwent endotracheal intubation and mechanical ventilation. A follow-up multidetector computed tomography (MDCT) scan of the chest showed a complex mass in interlobar position with an internal radiopaque serpiginous thread of metallic density which was assumed to represent a retained surgical sponge. Upon surgical exploration, no retained foreign body was found and a zone of recent hemorrhagic infarction, bordered by the line of the mechanical staples used to complete the minor fissure, was removed from the middle lobe. When evaluating patients suspected of having a retained surgical sponge, thoracic surgeons and radiologists should be aware of this potential source of confusion. Key-word: Foreign bodies.

Retained surgical sponges (also called gossypibomas or textilomas) are a rare but well recognized and dreaded complication of surgery. Several authors have highlighted the inconsistency and variability of the radiologic appearances of intrathoracic gossypibomas (1, 2). In this report we describe a peculiar case where, paradoxically enough, modern imaging techniques introduced unanticipated confounding factors into this diagnostic problem. Case report A 57-year-old man with a medical history of myotonic dystrophy type 1 (Steinert disease) underwent right upper lobectomy and systematic lymphadenectomy for a 20-mm well differentiated neuroendocrine carcinoma with lobar and interlobar lymph nodes involvement, staged as a T1N1 tumor. Multiple firings (n = 4) of a gastrointestinal anastomosis stapler with a 55 mm 3.85 mm load of titanium staples were used to complete both minor and major fissures. The surgical procedure was straightforward: no safety-compromising events known to be associated with a risk of retention of a foreign body occurred (3, 4) and repeated counting procedures of instruments and surgical sponges were correct. On postoperative day 2, hemodynamic compromise and acute respiratory failure developed following aspiration of gastric contents. The patient underwent endotracheal intubation and mechanical ventilation. Anteroposterior portable chest radiographs demonstrated opacification

Fig. 1. — Anteroposterior portable chest radiograph obtained on postoperative day 8 shows opacification of the right paratracheal zone.Note also a pleural fluid collection in the left lower lung zone.

of the right paratracheal zone which was interpreted as postoperative pulmonary bleeding and inadequate ventilation of the middle lobe (Fig. 1). However, owing to a poor response to treatment, a multi–detector computed tomography (MDCT) scan was obtained on postoperative day 10, which showed a complex mass in the right upper hemithorax. A serpiginous linear opacity of metallic density was seen within the mass (Fig. 2). The findings were thought most likely to represent a retained surgical sponge and thoracotomy for removal was recommended. At operation, no

From: 1. Radiology Unit, 2. Thoracic Surgery Unit, University of Turin, Department of Clinical & Biological Sciences, Orbassano, Turin, Italy. Address for correspondence: Dr L. Cardinale, University of Turin, Department of Clinical & Biological Sciences, Radiology Unit, San Luigi Hospital, I-10043 Orbassano (Torino), Italy. E-mail: luciano.cardinale@gmail.com

retained surgical sponge was found and a dark bluish zone of hepatization in the middle lobe, bordered by the line of the staples used to complete the minor fissure, was removed. Pathologic evaluation showed recent hemorrhagic infarction. The postoperative course was complicated by persistent disorders of deglutition and impaired cough mechanism. The patient was discharged to a long-term care facility with tracheostomy and feeding jejunostomy on postoperative day 35. Discussion Despite numerous published reports, there is little consensus in the literature on the incidence of retained surgical sponges, with estimates ranging from 1 in 1.000 to 1 in over 18.000 procedures (3). Most


METALLIC STAPLES LINE MIMICKING A RETAINED SURGICAL SPONGE — CARDINALE et al

A

B

263

C

Fig. 2. — MDCT. Coronal MIP image (A) shows a heterogeneous mass in the right upper hemithorax with an internal radiopaque thread of metallic density which was assumed to be a retained surgical sponge. Additional sagittal oblique MIP (B) and volumerendered (C) images show the interrupted serpiginous pattern of metallic density, compatible with the multiple firings of the staple device used to divide interlobar fissures.

likely, the difficulty in ascertaining the true incidence of retained surgical sponges results from the lack of established reporting systems for such adverse occurrences, the concern for potentially serious medicolegal implications, and the number of asymptomatic retained surgical sponges remaining undiscovered for years or decades (5). Indeed, the inadvertent loss of surgical sponges continues to be a dreadful hazard of surgery. Surgical sponges are routinely supplied with radiopaque markers allowing them to be readily recognized on imaging studies. However, in the early postoperative period, proper identification of radiopaque sponge markers on plain radiographs is hampered by several factors including location and orientation of the foreign body, marker distortion by folding or twisting, and presence of metallic artifacts from surgically placed staple lines or clips (6-8). Besides, detection of radiopaque sponge markers may not be as easy as claimed owing to the less than ideal diagnostic quality of anteroposterior portable chest film obtained in the perioperative period. In our case, also retrospectively, was the staple lines specific pattern not recognizable in bedside radiography. Finally, the most CT characteristic signs for retained surgical sponges, namely a spongiform or whirl-like pattern with gas bubbles in a mass with a thin enhancing capsule, may be absent or may be confused with loculated infection and organized hematoma or seroma (9). All of these factors can lead to diagnostic dilemmas and misinterpretations.

Recent advances in MDCT technology have greatly improved the quality of three-dimensional (3D) renderings. However, combining our findings with those of two prior case reports (7, 8) suggests that the reconstruction of images by using maximum intensity projection (MIP) technique, besides lacking accurate 3D perspective, results in increased opacity of the line of the mechanical staples used to complete interlobar fissures, which simulates the radiopaque marker of a supposedly retained surgical sponge. In retrospect, closer examination of additional images generated with both MIP and volume rendering techniques (Fig. 2B,C) or the creation of cine loops from MIP images in multiple planes revealed the interrupted pattern of metallic density, consistent with the multiple firings of the gastrointestinal anastomosis stapler used to separate the lobes. On the other hand, disintegration of the radiopaque marker embedded in a retained surgical sponge was to be excluded in view of the short time that had elapsed since surgery. We reiterate the opinion of others (6, 10) that optimal use of imaging techniques (native axial CT data and multiplanar reformation with MIP for the reconstruction of two-dimensional images, and volume rendering for the creation of 3D images) is required for achieving a confident understanding of equivocal imaging findings. Besides, referral to a photoradiographic atlas of surgical items that can be left, either intentionally or unintentionally, in surgical wounds would contribute to making a prompt diagnosis and facilitating appropriate treatment.

References 1. Suwatanapongched T., Boonkasem S., Sathianpitayakul E., Leelachaikul P.: Intrathoracic gossypiboma: radiographic and CT findings. Brit J Radiol 2005, 78: 851-853. 2. Madan R., Trotman-Dickenson B., Hunsaker A.R.: Intrathoracic gossypiboma. AJR 2007, 189: W90-W91. 3. Gawande A.A., Studdert D.M., Orav E.J., Brennan T.A., Zinner M.J.: Risk factors for retained instruments and sponges after surgery. N Engl J Med 2003, 348: 229-235. 4. Christian C.K., Gustafson M.L., Roth E.M., Sheridan T.B., Gandhi T.K., Dwyer K., et al.: A prospective study of patient safety in the operating room. Surgery 2006, 139: 159-173. 5. Kaiser C.W., Friedman S., Spurling K.P., Slowick T., Kaiser H.A.: The retained surgical sponge. Ann Surg 1996, 224: 79-84. 6. de Lacey G.: Retained surgical swabs: possible causes for errors in X-ray detection and an atlas to assist recognition. Brit J Radiol 1978, 51: 691-698. 7. Poncelet A.J., Watremez C., Tack D., Noirhomme P.: Paracardiac opacity following inferior- and middle-lobe resection for bronchogenic carcinoma. Unsuspected diagnosis. Chest 2005, 128: 439-441. 8. Granetzny A., Holtbecker N., Thomas H., Klein K., Boseila A.: Misinterpretation of a pulmonary GI anastomosis stapler line as a retained foreign body. Chest 2008, 133: 281283. 9. Kopka L., Fischer U., Gross A.J., Funke M., Oestmann J.W., Grabbe E.: CT of retained surgical sponges (textilomas): pitfalls in detection and evaluation. J Comput Assist Tomogr 1996, 20: 919-923. 10. Wolfson K.A., Seeger L.L., Kadell B.M., Eckardt J.J.: Imaging of surgical paraphernalia: what belongs in the patient and what does not. RadioGraphics 2000, 20: 1665-1673.


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WEGENER GRANULOMATOSIS MASQUERADING AS PNEUMONIA D.J. Theodorou1, S.J. Theodorou1, K. Mpougias1, M. Mastora2, S. Stefanaki3, N.C. Akritidis2 We report a case of an elderly patient with a limited form of Wegener granulomatosis, which simulated the clinical and imaging features of organizing pneumonia. Here we call attention to this atypical case presentation that eloquently illustrates the many faces of Wegener granulomatosis. Key-word: Wegener granulomatosis.

Report of the case A 76-year-old woman presented at the emergency room with malaise, chest pain, dyspnoea and cough, and a 2-day history of fever along with transient mental status changes. On examination, her body temperature was 39.4°C, blood pressure was 115/70 mmHg, heart rate 112 beats/min, and respiratory rate 28 breaths/min. The patient had bronchial breathing and crepitations, especially over the right lung. Laboratory test results are shown in Table I. Initial chest radiography and CT scan (Fig. 1) showed bilateral parenchymal consolidations, containing air bronchograms. A bacterial pneumonia was suspected and the patient received a treatment with intravenous (IV) broad-spectrum antibiotics. She was discharged one week later based on clinical recovery. After two days, the patient was febrile again and was admitted to the hospital. Repeat chest radiography showed bilateral, multifocal, solid and cavitary masses confluent at the lung bases. A cavitary mass with an air-fluid level was present in the left upper lobe (Fig. 2A). A new chest CT scan revealed multiple cavitary lesions in both lungs (Fig. 2B). The patient was treated with levofloxacin hemihydrate and vancomycin hydrochloride for 10 days. Methylprednisolone was empirically added to the theurapeutic regimen on day four, and clinical response to treatment was dramatic. The peripheral white blood cell count dropped at 6,470/mm3 with 77% neutrophils and 12% lymphocytes, and CRP was 0.74 mg/L. Because of the aggressive course of the disease, an open lung biopsy was performed. Histopathologic

Fig. 1. — Chest CT scan (lung window) on first admission demonstrates bilateral, extensive parenchymal consolidations in the right (R) upper and middle lobes abutting the fissure, and the left (L) upper lobe. Air bronchogram (arrowheads) is evident particularly on the right. Pneumonia was the diagnosis favored at the time.

analysis showed multiple geographic areas of necrosis and granulomatous inflammation (Fig. 3). There was vasculitis of the medium-sized and small pulmonary arteries, veins and capillaries. The diagnosis established was Wegener granulomatosis (WG) of the lung. The absence of systemic disease involvement was suggestive of “limited” disease, which represents a “forme fruste” of Wegener granulomatosis (1, 2). After two weeks of treatment, symptoms resolved and the patient was discharged. Discussion WG is a multi-organ system disease characterized by granuloma-

From: Department of 1. Radiology, 2. Internal Medicine, 3. Pathology, Ioannina General Hospital, Ioannina, Greece. Address for correspondence: Dr D. J. Theodorou, M.D., 13 Papadopoulos street, Ioannina, 45444 Greece. E-mail: daphne_theodorou@hotmail.com, rjtheodorou@hotmail.com

tous inflammation, tissue necrosis, and vasculitis (1). Although this autoimmune disorder may affect virtually any organ, it has a predilection for the upper respiratory tract, the lungs, and the kidneys (3). Multiple pulmonary nodules or masses that are frequently cavitated are the hallmarks of lung disease. As previous studies have shown, however, WG involving the lungs may also present with non-specific pulmonary infiltrates, which can be initially misdiagnosed as pneumonia (1, 3). In our patient, the clinical manifestations of pulmonary disease were largely derived from extensive infiltrates within the lungs. The presence of pneumonia as the one and only manifestation of WG is very rare (2). Our case is unusual in that involvement of the lungs -in the form of isolated air-space consolidation- was the sole manifestation of disease. It is well known that the ANCAs may amplify inflammation, affecting primarily the neutrophils. Reportedly, only 10-20% of patients with active, untreated WG are ANCA


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A

265

B

Fig. 2. — A. Posteroanterior chest radiograph two days after discharge on the second admission shows bilateral, multiple pulmonary masses with areas of cavitation. A large cavitary mass with air-fluid level (arrow) is seen in the left paracardial area. B. Chest CT scan (lung window) demonstrates bilateral, multiple solid and cystic lesions. Large cavitary mass on the left (L) contains air-fluid level (arrow). Note the variable thickness of the walls of the cavities.

Table I. — Laboratory data at first and second admission. Results Parameter

1st admission

2nd admission

Normal value

Haemoglobin, g/dl

12.4

12.7

12-16

White cells x 103, /mm3

13

27,12

4,5-11

85 6 215

92 3 203

55-65 20-35 200-350

Differential count, % Neutrophils Lymphocytes Platelets x 103, /mm3 Erythrocyte sedimentation rate (ESR), mm/1 h

51

62

6-12

C-reactive protein (CRP), mg/ l

5.7

21.4

0-0,8

Ferritin, ng/ml Urea, mg/dl Creatinine, mg/dl

119 76 1.1

135 79 1.3

80-200 11-54 0,5-1,4

Rheumatoid factor (RF)

N/E

Normal

anti-doublestranded DNA antibodies (anti-dsDNA)

N/E

Normal

antinuclear antibody (ANA)

N/E

Positive

antineutrophil cytoplasmic antibodies (ANCAs)

N/E

Negative

N/E = Not evaluated.

negative (1), while up to 30% of patients with limited WG are ANCA negative (1). The specificity (99%) and sensitivity (91%) of ANCA testing for active disease is high (4, 5). Our case is challenging in that WG was suspected not because it caused pneumonia as the sole manifestation of disease or was ANCA negative but, rather, because of its remarkable response to steroid treatment.

Limited WG, as in our case, certainly poses a difficult diagnostic problem. Other conditions including infection (mycobacteria, fungi, actinomycosis and syphilis), malignancy (squamous cell carcinoma, extranodal lymphoma, plasmacytoma and metastasis), and various immunological disorders (amyloidosis, rheumatoid arthritis, systemic lupus erythematosus, microscopic polyangiitis and the Churg-Strauss

syndrome) may mimic WG in its presenting manifestations (1-3). Although limited WG has a more favorable prognosis than its classical counterpart, recognition of disease is essential because untreated disease is almost uniformly fatal (2). In our patient, serial imaging studies demonstrated progressive and destructive lung changes, indicating severe disease. The additional monitoring of inflammatory markers


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pneumonia after classical therapy with antibiotics is strongly suggestive of WG, even in the absence of generalized disease or ANCA positivity. Histologic proof is often required because pulmonary carcinoma and infection can present an identical clinical and imaging pattern of involvement. References 1.

2. Fig. 3. — Photomicrograph of lung biopsy specimen shows irregular areas of parenchymal necrosis (arrows) surrounded by inflammatory granulation tissue (hematoxylin and eosin, x 40). 3.

reflected disease activity over time. Although histopathologic proof was available later on in our case, WG was suspected and included in the differential diagnosis because of the patient’s significant and rapid clinical improvement following initial empirical treatment with antibiotics, and, most importantly, steroids. In previous studies, steroids have been associated with complete remission of disease in 75% of the patients (6). An interesting finding in our case is this patient’s limited involvement of WG confined to the lung, without the typical upper airway involvement, systemic vasculitis, or glomerulonephritis. To our knowl-

edge, isolated WG of the lung comprises an unusual manifestation of disease that can easily go undiagnosed. We present this case, not only for its rarity and its benign course after successful treatment with steroids, but foremost for the challenging differential diagnosis of WG masquerading as pneumonia. Indeed, this case of an atypical presentation of disease underscores the clinical versatility of WG that clinicians may need to confront with. In conclusion, the presence of pulmonary consolidations as the first and only manifestation of WG is extremely rare. However, the persistence and relapse of symptoms in

4.

5.

6.

Stone J., Hoffman G.: Wegener’s granulomatosis and lymphomatoid granulomatosis. In: Hochberg M., Silman A., Smolen J., Weinblatt M., Weisman M., eds. Rheumatology, 3rd ed. Edinburgh: Mosby, Elsevier Ltd, 2003, pp 1635-1648. Frazier A., Rosado-de-Christenson M., Galvin J., Fleming M.: From the archives of the AFIP. Pulmonary angiitis and granulomatosis: radiologic-pathologic correlation. RadioGraphics, 1998, 18: 687-710. Mayberry J., Primack S., Müller N.: Thoracic manifestations of systemic autoimmune diseases: radiographic and high-resolution CT findings. RadioGraphics, 2000, 20: 1623-1635. Rao J., Weinberger M., Oddone E., Allen N., Landsman P., Feussner J.: The role of antineutrophil cytoplasmic antibody (c-ANCA) testing in the diagnosis of Wegener granulomatosis: a literature review and metaanalysis. Ann Intern Med, 1995, 123: 925-932. Wiik A.: Testing for ANA and ANCAdiagnostic value and pitfalls. In: Hochberg M., Silman A., Smolen J., Weinblatt M., Weisman M., eds. Rheumatology, 3rd ed. Edinburgh: Mosby, Elsevier Ltd, 2003, pp 215-26. Langford C.: Update on Wegener granulomatosis. Cleveland Clin J Med, 2005, 72: 689-697.


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HISTORICAL ARTICLE THE ROOTS OF RADIOLOGY IN GREECE C. S. Baltas1, A. P. Balanika2, N. Kelekis3, I. V. Fezoulidis4 This article presents as reliably as possible the roots of the Radiology specialty in Greece, from the time of the discovery of X-rays by WC Roentgen. It mentions the most important dates relevant to the foundation of the specialty of Radiology in Greece. Key-word: Radiology, history.

Wilhelm Conrad Professor Roentgen (1845-1923) was the discoverer of X-rays and who first used the term “X rays”, utilizing the Greek letter X - the Greek mathematical symbol representing the unknown. His discovery was initially presented to the Institute of Physics at the University of Wurzburg on December 28, 1895. The present article reviews the first steps of Radiology in Greece. The first X- rays, which were taken in Athens in 1896 by Timoleon Argyropoulos, Professor of Physics at the National and Kapodistrian University of Athens. Two years later, in 1898, the first X-ray equipments were installed in hospitals in both Patras and Athens. The first Greek publication concerning the use of Xrays was written by Professor Timoleon Argyropoulos and was published in the French scientific journal “Comptes rendus Hebdomadaires des séances de l’ Académie des Sciences” in 1896. The first Greek textbook by Konstantinos Maltezos, Professor of Engineering at the National and Kapodistrian University of Athens was published in 1897. The present article recounts the establishment of the Hellenic Radiological Society as well as the developments in the field of Radiology in Greece up to the establishment of the first Chair of Radiology at the National and

Kapodistrian University of Athens in 1947. The first X-rays in Greece The father of Radiology in Greece is considered to be the Professor of Physics at the National and Kapodistrian University of Athens Timoleon Argyropoulos (1847-1912), who conducted experiments with Xrays as far back as 1896. In the Laboratory of Experimental Physics of the University of Athens, in the Chemistry Institute on Solonos street, on February 20, 1896*, he was able to “photograph” bones on radiological plates which were first reproduced on a negative plate and then reprinted on a positive plate. K. Basias and K. Botsis were his assistants in this successful experiment (1). In reality, the first X-ray machine installed in Greece was a mobile radiological unit which was brought to Greece from Great Britain. It was part of the medical supplies provided to the Greek Army by the British people for those injured in the Greek-Turkish War of 1897. The radiological unit, manufactured by the London Company Miller and Woods, was sent to Greece in 15 packages with the HMS Prince Crown. The unit’s power supply was provided by generators charged aboard the HMS Rodney, which had docked in the port of Piraeus. Head of the medical

From: 1. Consultant Radiologist, Radiology Imaging Department, General Hospital of Athens “G. Gennimatas”, Athens, Greece, 2.Consultant Radiologist, Computed Tomography Department, General Hospital of Athens “Asklipieio Voulas”, Athens, Greece, 3. Associated Professor of Radiology, 2nd Radiology Department, University General Hospital “Attikon”, Athens, Greece, 4.Professor of Radiology, Department of Radiology, University of Thessaly, Larisa, Greece Address for correspondence: Dr C. S Baltas, M.D., Radiology Imaging Department, General Hospital of Athens “G. Gennimatas”, 28th Octovriou Str, N. Psychiko 15451, Athens, Greece. E-mail: mpaltas@hotmail.com

team was Francis Charles Abbott, a surgeon at St Thomas’ Hospital in London and a member of the Royal College of Surgeons. F.C. Abbott departed from London’s Victoria Station on April 30, 1897, and was accompanied by Mrs Bedford Fenwick (in charge of organizing the nursing staff) and Henry Alford Moffatt, a surgeon at Guy’s Hospital. They arrived in Athens on May 4th of the same year. Robert Fox Symons, a surgeon at St Thomas’ Hospital, was in charge of the X-ray unit (2). The X-ray unit was housed in Faliro (Athens), in a building granted for this purpose by Queen Olga. In a written report, F.C. Abbott and R.F. Symons noted the fear Greek soldiers expressed when examination was required by this new machine, pointing out that the soldiers crossed themselves not only before but also during their examination, which lasted as much as 35 minutes. The patients’ behaviour was based on fear because they considered the machine to be “the work of the devil” (2, 3). Abbott published a report based on his experience from Greco-Turkish War which was published in “The Lancet” in two issues, those of January 14 and 21, 1899 (4, 5). The German Red Cross sent X-ray equipment to a military hospital in Istanbul. This was listed as one of the first times radiological equipment was used worldwide during times of war. The first medical presentations of X-rays took place at the Athens Medical Society. On March 12, 1898*, the surgeon D. Kokkoris presented a case regarding: “The reproduction of a woman’s hand, which was produced in the Physics Laboratory by Professor Argyropoulos using a Roentgen machine, and another X-ray of a stomach tumor (6, 7). On March 19 of the same year the physician G. Koromilas - gave a speech “On


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the subject of X-rays” (6) with three cases using X-rays - once again presented at the Athens Medical Society. It was in 1898 that the first X-ray equipment was installed in Greek public hospitals. The first was installed at the General Public Hospital of Patras as a donation from Andreas Kollas. On March 31st 1898*, the use of the machine was demonstrated by Timoleon Argyropoulos, Professor of Physics at the National and Kapodistrian University in Athens, when he was able to show a foreign object (bullet) in the wounded leg of Efstathios Samartzis. Despite the capabilities of this new technique, due to the absence of a physician trained to use the equipment, it was rarely used over the next few years (8). The second X-ray machine was installed in the biggest hospital in Athens, “Evangelismos” (7). The equipment remained at “Evangelismos” Hospital up until 1923. The management of the makeshift radiology department was originally assigned to Professor Marinos Geroulanos. In 1912, Christos Kalantidis was appointed chairman, followed by Manos Karzis in 1918 and Isidoros Gounaris in 1922 (9). The first private lab was set up in Athens in 1903 by Ioannis Chrisospathis as published in an advertisement in the Athenian newspaper “Akropolis” (7). The first scientific medical papers and textbooks and training in Radiology The Professor of Physics at the National and Kapodistrian University of Athens, Timoleon Argyropoulos, published the first foreign language article in France, which was presented on May 18th 1896 at the French Academy of Science by the Academic A. Cornu and published in the weekly scientific journal “Comptes rendus Hebdomadaires des séances de l’ Académie des Sciences” in the January-June 1896 issue, page 1119, and titled: “Observations sur les rayons X” Note de M.T. Argyropoulos présentée par M.A. Cornu (Fig. 1). The following are mentioned in the publication: “En expérimentant avec différentes substances fluorescentes aux rayons X j’ ai constaté que le platinocyanure de potassium et de sodium et aussi le platinocyanure de potassium et de lithium deviennent bien plus lumineux que celui de baryum. La

Fig. 1. — M.T. Argyropoulos: Observations sur les rayons X. Comptes rendus Hebdomadaires des séances de l’Académie des Sciences, 1896, 122: 1119.

fluorescence des premières était bien visible à une distance de 5 mètres, tandis qu’avec la même intensité des rayons X le platinocyanure de baryum n’était visible qu’ à une petite distance” (10, 11). The first presentation concerning X-rays at a Greek medical convention was made on April 16th, 1906*, within the framework of the 5th Panhellenic Medical Convention by Ioannis Chrisospathis. The convention was held in the lecture hall of the National and Kapodistrian University and the speaker presented the subject of X-rays in his speech entitled “Roentgen’s rays in medicine”, which was held on the 7th day of the convention (12, 13).

The first textbook of Radiology written in Greek was a monograph by the Professor of Engineering at the National and Kapodistrian University of Athens, Konstantinos Maltezos (1869-1951), entitled: “Cathode rays and new forms of radiation” published in 1897 by “Estia” Publications (Fig. 2). It contained an introduction and three chapters, totaling 55 pages (14). In 1917, the book entitled “Determining doses in Radiotherapy” was written by Manos Karzis and was published by I. Vartsos Publications, followed two years later by a 114 page book by the same author entitled “Radium and its properties”, by “Ermou”


THE ROOTS OF RADIOLOGY IN GREECE — BALTAS et al

Fig. 2. — The first textbook of Radiology written in Greek by the Professor Konstantinos Maltezos, entitled: “Cathode rays and new forms of radiation” published in 1897.

Publications. In 1919, the prolific writer Manos Karzis wrote a chapter which was included in the book “Guide to Medical Surgery by the Medical Department at the Ministry of the Military” entitled “Defining and determining a foreign object in the body using X-rays” (15). The first Greek medical journal dealing with Radiology was titled “Deltion Karkinologias” and circulated in Athens from 1929 to 1932. Its Chief Editor was Radiologist Athanasios Lambadaridis. It was published every two months and contained articles by Athanasios Lambadaridis, as well as translated articles on extremely specialized subjects for that time period by distinguished University Professors as well as heads of hospitals from abroad (16, 17). “Hellenic Radiology”, the official radiological journal of Greek radiologists, was published much later, in 1968, which is to say 36 years after “Deltion Karkinologias” stopped being published (18). Unfortunately, the subject of Radiology was taught at a much later

date in Greece. The first official classes in Radiology began to be taught at the “Andreas Syggros” Hospital in 1923 by Felix-Eftyhios Hart, who was to become the first Professor of Radiology at the National and Kapodistrian University of Athens, and more specifically to fifth year students of the University’s Clinic in Skin Diseases (7). The establishment of the Hellenic Radiological Society and the first University Chair in Radiology The Hellenic Radiological Society was established on September 20, 1933. Its memorandum of association consisted of 38 articles. It was signed by its first President Dimitrios Vasilidis and the General Secretary Athanasios Lambadaridis, and was approved by the Athenian Court of First Instance with the number 8266 ruling of October 31st, 1933. The first Executive Board of the Society consisted of the following people, in alphabetical order: Vasilidis Demetrios, Vidalis Evangelos, Georgakopoulos Andreas, Gounaris

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Isidoros, Grigoratos Panagiotis, Throuvalas Antonios, Kalantidis Christos, Karzis Manos, Kontopoulos M., Kratsas Georgios, Kiniras Michael, Lambadaridis Athanasios, Lapatsanis Panagiotis, Kope Joseph, Petrohilos Stefanos, Prapopoulos Takis, Tsaggaris Konstantinos, Tsarouhas Vagias, Farmakidou Lia, Hart Felix-Eftyhios (19, 20). The specialty of Radiology was established five years later in 1938 by mandatory Law 1461 (21). In 1947, the newly-established, although temporary, Chair of Radiology and Physiotherapy was established at the National and Kapodistrian University of Athens, and the following year Felix-Eftyhios Hart (1885-1954) was promoted to full Professor of Radiology in Greece (22). And so, 50 or so years after the discovery of X-rays, the National and Kapodistrian University of Athens acquired a full Chair and full Professor of Radiology and the opportunity was finally given to young doctors who wanted to specialize in Radiology to do so in their own country instead of going abroad for this purpose. It was not until 1972 that the first Panhellenic Radiological Convention took place in Athens, 39 years after the establishment of the Hellenic Radiological Society (23). (*It must be pointed out that the Gregorian calendar is used throughout most of Europe, while the Julian calendar was still used in Greece. It wasn’t until March 1st 1923 that Greece switched over to the Gregorian calendar, which means that 12 additional days must be added to European dates). References 1. Livadas G., Tsachalis A.: Timoleon Argyropoulos, the unknown father of Greek radiology. Hellen Radiol, 1996, 27: 207-210. 2. Ramoutsaki I.A., Giannacos E.N., Livadas G.N.: Birth of battlefield radiology: Greco-Turkish War of 1897. Radiographics, 2001, 21: 263-266. 3. Livadas G.: The first medical radiographs in Greece. Hellen Radiol, 1995, 26: 347-348. 4. Abbott F.C.: Surgery in the GrecoTurkish War. Lancet, 1899, 14: 80-81. 5. Abbott F.C.: Surgery in the GrecoTurkish War. Lancet, 1899, 14: 152156. 6. Pournaropoulos K.G.: Radiology in the Athens Medical Society during its first twenty years (1835-1934). Hellen Radiol, 1979, 12: 167-173. 7. Vrakatselis Th.: Chronology. Hellen Radiol, 1995, 26: 335-337.


270 8. Zavras G.M.: The first stages of Radiology in Patras. Hellen Radiol, 1995, 26: 387-389. 9. Damilakis J.E.: The first stages of the evolution of medicine of the ionizing radiation in Greece. Hellen Radiol, 1995, 26: 349-353. 10. Argyropoulos M.T.: Observations sur les rayons X. Comptes rendus Hebdomadaires des séances de l’Académie des Sciences, 1896, 122: 1119. 11. Baltas C.: The History of Greek Radiology. PhD thesis, University of Thessaly, Department of Radiology, 2006, pp 46. 12. Chrisospathis I.: Roentgen’s rays in medicine. Minutes of the 5th Panhellenic Medical Conference, Athens, April 10-18, 1906.

JBR–BTR, 2010, 93 (5) 13. Baltas C.: The History of Greek Radiology. PhD thesis, University of Thessaly, Department of Radiology, 2006, pp 48-49. 14. Papadaki P.J.: Hellen Radiol, 1996, 27: 403. 15. Papadaki P., Vrakatselis Th.: The first Greek publications on Radiodiagnostics and Radiotherapy. Hellen Radiol, 1995, 26: 382-386. 16. Deltion Karkinologias. Years 1929, 1930, 1931, 1932, Hellenic National Library. 17. Baltas C.: The History of Greek Radiology. PhD thesis, University of Thessaly, Department of Radiology, 2006, pp 87-89. 18. Baltas C.: The History of Greek Radiology. PhD thesis, University of

19. 20.

21. 22.

23.

Thessaly, Department of Radiology, 2006, pp 89-90. Book of Minutes of the Hellenic Radiological Society. Baltas C.S., Balanika A.P.: Evangelia Farmakidou (1890-1982): the first female Greek radiologist. J Med Biogr, 2010, 18: 41-43. Pappas A.: The mysterious X-rays. The first years. Athens, Al Pappas (eds), 2002, pp 107. Livadas G.: Felix Hart. The first teacher of Radiology in Greece. Hellen Radiol, 1995, 26: 362366. Baltas C.: The History of Greek Radiology. PhD thesis, University of Thessaly, Department of Radiology, 2006, pp 94-98.

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JBR–BTR, 2010, 93: 271.

IMAGES IN CLINICAL RADIOLOGY Epiploic appendagitis within a Spigelian hernia B. Coulier1, B. Broze2

A

A 49-year-old female presented to the emergency room with a one week history of continuous pain in the left iliac fossa. Local tenderness was present and a painful olive-shaped mass was clearly palpable in this area. Ultrasound revealed an 18 x 10 mm painful hyperechoic avascular mass (Fig. A, sagittal and axial view) which was trespassing the abdominal wall through a 7-millimetre orifice. It was prolonged by a small intra abdominal pedicle ending on the sigmoid colon (white arrow). The herniating mass was surrounded by a small amount of fluid and interstitial fat and this complex remained covered by the aponeurosis of the great oblique muscle (white arrowhead). The orifice was situated exactly along the spigelian line constituted by the external border of the left rectus muscle and the internal border of the lateral abdominal muscles. An epiploic appendage incarceration – with secondary appendagitis – through a parietal Spiegelian hernia was diagnosed and confirmed by unenhanced MDCT (Fig. B (axial view) and C (oblique sagittal and coronal view). The patient underwent laparoscopic repair of the hernia. The post operative period was uneventful. Comment

Spigelian hernia (SH) classically develops under the level of the arcuate line of Douglas where a physiologic anatomic change of the posterior rectus sheath produces resulting in an area of relative “weakness” more prone to develop parietal hernias. Under this line of Douglas, all three aponeurosis of the lateral abdominal muscles do not more equally distribute anteriorly and posteriorely around the rectus muscle to constitute a firm circumferential sheath but abruptly pass exclusively anteriorly to the muscle, thus creating a weakness of the B posterior rectus sheath. This weakness is amplified by the fact that, at this level, the internal oblique and transverse muscles do not more constitute solid muscular bands but only fascias or thin musculo-aponeurotic bands separated by fascias and running in a parallel instead of perpendicular way. 95% of SH produce through these slitting defects. Transversally this area is situated between the rectus muscle and the lateral abdominal muscles on a curvilinear vertical line called the line of Spiegel. SH always contain a peritoneal sac, rarely exceed a diameter more than 2 or 3 cm and remain covered by the aponeurosis of the external oblique. The high percentage of complications is essentially due to the small size of their orifice – 0,5 to 2 cm – which predisposes to incarcerations and strangulations C with occlusive syndrome. Presentation and symptoms are variable and related to the nature of the incarcerating structure. Moreover SH are also clinically difficult to diagnose because a typical mass is not always palpable. The greater omentum, small bowel, sigmoid colon or caecum are the most implicated organs but cases implicating a strangulated appendice, the gallbladder, an ovary or a testis, the round ligament or the fallopian tube, the stomach, the Meckel’s diverticulum, an uterine fibroma or endometric nodules have also rarely been reported. Incarceration of an epiploic appendage (EA) – with secondary appendagitis – in a SH has only exceptionally been described with only three previous cases described in the literature and only one case with ultrasound and CT correlations. Classically acute epiploic appendagitis (AEA) results from twisting, kinking or stretching of EA along their long axis with impairment of their vascular supply, subsequent venous thrombosis and necrosis. Patients describe a localized, strong, non-migratory, sharp pain with localised abdominal tenderness. When localised on the right it may mimic acute appendicitis, but more often it is found on the left side of the abdomen mimicking acute sigmoid diverticulitis. Ultrasound shows an oval, avascular and non-compressible hyperechoic mass with a subtle hypoechoic rim directly under the site of maximum tenderness. Although CT findings also appear very typical US has specific advantages on CT to diagnose the incompressibility of the lesion, visualise its adherence to the parietal peritoneum and establish a precise correlation between the location of the lesion and that of the maximal parietal tenderness. In the reported case ultrasound rapidly focused on the elective site of tenderness and unambiguously recognized the typical intermingled ultrasound semeiology of both epiploic appendagitis and spigelian hernia. CT confirmed the ultrasound diagnosis but finally didn’t carry any complementary decisive data for the diagnosis and therapeutic decision.

Department of 1. Diagnostic Radiology and 2. Abdominal Surgery, Clinique St Luc, Bouge, Belgium.


JBR–BTR, 2004, 87: 272.

IMAGES IN CLINICAL RADIOLOGY Extra-adrenal retroperitoneal ganglioneuroma E. Janssens, L. Van Hoe, T. De Beule, P. D’haenens1

A

B

C1

C2

A 74-year-old woman presented to the hospital complaining of fatigue and atypical epigastric discomfort. Physical examination revealed epigastric tenderness. She had no relevant clinical history. Laboratory findings were within normal limits. A CT scan of the abdomen was performed and showed a large well-defined, lobulated hypodense mass in the retroperitonium (Fig. A, arrows), containing small punctate calcifications (Fig. A, arrowheads). After intravenous contrast administration the mass showed slight and inhomogeneous enhancement. The mass surrounded the celiac trunc (Fig. B, arrowhead) and superior mesenteric artery, without evidence of luminal narrowing. On MRI, the lesion was homogeneous and hypointense relatively to the liver on T1, and heterogeneous and hyperintense on T2. Axial contrast-enhanced dynamic T1weighted MR images obtained 40 seconds (Fig. C1, arrows) and 100 seconds (Fig. C2, arrows) after contrast material injection showed gradual but heterogeneous enhancement of the mass. The radiological differential diagnosis included lymphadenopathy and neurogenic tumor. No malignancy was found elsewhere in the body. CT guided percutaneous biopsy of the mass was performed and pathology revealed an extra-adrenal ganglioneuroma. Encasement of the vascular structures made radical excision impossible. Followup by imaging after 6 months was recommended. Comment Ganglioneuroma is a rare, benign neurogenic tumor, composed of mature Schwann cells, ganglion cells and nerve fibers, arising typically along the paravertebral sympathic plexus and occasionally from the adrenal medulla. The two most common sites are the retroperitonium and posterior mediastinum, followed by the cervical region and pelvis. The tumor has been most often described in adolescents and young adults, but all ages can be affected. Clinical presentation is often asymptomatic. Most abdominal ganglioneuromas are hormone silent and detected incidentally by abdominal imaging studies. The prognosis is excellent and recurrence is rare after surgical resection. CT and MRI can be used to develop a differential diagnosis. Retroperitoneal or adrenal ganglioneuromas appear as a well-circumscribed oval or lobulated mass. CT images show a homogeneous, hypo-attenuating mass containing discrete and punctate calcifications in 20% of the cases. Typically, slight or inhomogeneously enhancement is reported on delayed scans. On MR imaging, the lesions are relatively homogenous with low signal intensity on T1 WI and intermediate to high signal intensity on T2 WI. The tumor signal intensity on T2 WI is influenced by the portion of myxoid stroma to cellular components and collagen fibers. Dynamic contrast-enhanced T1-WI shows lack of early enhancement, and gradually increasing, inhomogeneous enhancement. Curvilinear bands of low SI on T2 WI, giving the tumor a whorled appearance, are characteristic. The imaging appearance, including the anatomic location, shape and internal architecture support the correct diagnosis of this tumor but only biopsy can reveal the diagnosis of a ganglioneuroma.

1. Department of Radiology, OLVZ, Aalst, Belgium.


JBR–BTR, 2010, 93: 273.

IMAGES IN CLINICAL RADIOLOGY Unusual vacuum phenomenon suggesting occult vertebral instability B. Coulier, M. Theodorescu, B. Vander Elst

A

A 70-year-old female was referred to our department with complaints of bilateral lumbar sciatalgia. Lumbar CT was performed. Bilateral spondylolysis of L5 with secondary olisthesis had been previously surgically stabilised by anterior arthodesis. Ankylosis appeared effective at this level. On sagittal MPR the vertebra L4 and L5 seemed lined up well. Nevertheless our attention was retained by an unusual large diastasis of the L4-L5 joints (A, black arrowhead) and this diastasis was occupied by massive vacuum phenomenon. An unusual central vacuum phenomenon was also visible in the L4-L5 disk (A & B, black arrow). It was suggested that this diastasis could be due to a realignment of the vertebra L4 in the supine position (B, white arrowhead). Complementary upright plain film of the lumbar spine were performed in flexion and extension and revealed a sharp instability of L4 with olisthesis (C, white arrowhead) but also subsidence of the L4-L5 disk when compared with supine CT. Comment

B

C

Many radiological manifestations of vacuum phenomenon (VP) only represent snapshots of a complex dynamic hydropneumatical continuum extending from true VP to gas and/or fluid and vice versa. In the majority of cases VP remains an incidental or anecdotal finding but, nevertheless, it occasionally represents a useful clinical or radiological sing of critical importance for the understanding, clinical diagnosis, prognosis and therapeutic of several spinal diseases. VP is frequently observed in facet joints during supine CT especially in association with articular arthritis with or without olisthesis. It only represents an acute transitory phenomenon produced by distraction of joint during supine CT or MR examination. Sometimes, however, when the distraction is unusually large, the VP can alert the radiologist that there is instability at that level and that measurement of antero-posterior diameter of the central canal will seriously underestimate the true level diameter. As with discs and vertebrae gas can progressively replace VP in distracted joint and may consequently be forced within cysts of the ligamenta flava, extraspinal expansions of the facet joint or subchondral bone erosions. In sufficiently prolonged supine position a progressive replacement of gas by fluid may also produce and this replacement has been reported by MR.

Department of Diagnostic Radiology, Clinique St Luc, Bouge, Belgium.


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IMAGES IN CLINICAL RADIOLOGY

Terson’s syndrome M. Eyselbergs1, M. H. Voormolen1, A. Snoeckx1, P.M. Parizel1 A 55-year-old-woman suddenly woke up with the worst headache she ever experienced. The clinical picture deteriorated fast and by the time she was admitted to the emergency department, she developed anisocoria and was in a coma (Glasgow Coma Scale, 3/15). A non enhanced computed tomography of the brain was performed and revealed a subarachnoid hemorrhage (Fig. A) caused by a ruptured aneurysm of the anterior communicating artery (Fig. B, black arrow). The aneurysm was successfully treated by endovascular occlusion with coils. On the axial non enhanced CT-scan on admission a radiopaque linear density was noted in the left eye located in close proximity to the retina (Fig. C, white arrow). Ultrasound investigation of the left eye confirmed a vitreal hemorrhage but no retinal detachment. Clinically the patient had loss of visual acuity of the left eye. The diagnosis of Terson’s syndrome was made. Comment Terson’s syndrome is a vitreal or retinal hemorrhage as result of an acute increased intracranial pressure occurring consequently to subarachnoid hemorrhage. It can be seen in 10% to 20% of patients with spontaneous or traumatic subarachnoid hemorrhage and generally begins as bleeding between the internal limiting membrane and the retina. The pathophysiology of this syndrome is still debated. Disturbed circulation in the retinal vessels consequent to increased intracranial pressure may lead to retinal venous hypertension and eventually to hemorrhage. Early diagnosis can lead to a more accurate ophthalmologic follow up as this pathology can result in blindness. In the acute setting this syndrome can easily be overlooked as other pathology stands more on the foreground. The presence of a vitreous hemorrhage can also be seen as an adverse prognostic factor in terms of clinical outcome which can be related to rebleeding or the (re)appearance of coma. Although an ophthalmologic examination is the golden standard to diagnose this syndrome, careful CT evaluation by the radiologist can sometimes identify the ocular abnormalities in an early stage. Imaging findings on computed tomography can be very subtle and include nodularity, thickening or crescent-shaped increased density of the retinal surface relative to the vitreous body often along the temporal retinal side and adjacent to the optic nerve as described. These findings can be identified within the first few days after intracranial hemorrhage. Pitfalls in diagnosis can be volume averaging at the rectus muscle insertions or tangential sectioning of superior and inferior aspects of the eye. A crescentic increased density can be mimicked in this way. Differential diagnosis includes melanoma, metastasis and hemangioma but in the clinical setting of a subarachnoid hemorrhage a Terson’s syndrome is the most likely diagnosis. Patients with a subarachnoid hemorrhage are prone to a vitreal or retinal hemorrhage. Radiologists should be aware of the potential diagnosis of Terson’s syndrome since early diagnosis may prevent long-term vision loss.

1. Department of Radiology, Antwerp University Hospital, Edegem, Belgium.


JBR–BTR, 2010, 93: 275.

IMAGES IN CLINICAL RADIOLOGY

Pellegrini Stieda disease J.B. De Vis, P. Kersemansš A 52-year-old woman presented with load-dependent pain on the right knee. Pressure pain was conceived at the medial femoral condyle and manual valgus stress as well as rotation provoked severe pain at the medial side of the knee. Plain radiographs showed an ossification at the proximal part of the medial collateral ligament (Fig. A). Further imaging occurred. Magnetic Resonance Imaging (MRI) showed the ossification as a signal void on T2weighted images (Fig. B-C). There was also a hyper-intense signal surrounding the medial collateral ligament on T2-weighted images, consistent with an acute component. The diagnosis Pellegrini Stieda disease was made. Therapy consisted of rest and the use of non-steroidal anti-inflammatory drugs to treat the acute inflammatory component. No further follow-up of the patient occurred. Comment Pellegrini Stieda is a condition where ossification around the medial femoral condyle occurs. This may develop posttraumatically, different traumatic events having been described, eg. avulsion injury of the medial collateral ligament at the medial femoral condyle, tear or avulsion of the posterior cruciate ligament with stripping of the femoral periosteum proximal to the femoral attachment of the medial collateral ligament, ‌ Recent studies have shown that ossification has not necessarily to be in the medial collateral ligament. Ossification may occur as well in the medial collateral ligament, as in the adductor magnus tendon, or in both. Clinical symptoms are pain, limitation of motion, swelling and tenderness on pressure over the internal condyle of the femur. Diagnosis can be made by radiographic examination, which typically shows ossification at the proximal part of the medial collateral ligament. It is possible that early cases show abnormal 99 m technetium MDP uptake before evolution of plain radiographic changes. MR imaging shows ossification as a signal void on T2weighted image, T1 shows a high signal intensity area within the fragment consistent with the fatty marrow of the fragment and an indication of its maturity. Therapy consists of exercises in combination with pain medication, with this, pain mostly disappears after a few months. In refractory cases infiltration with corticosteroids or surgery, to remove the calcifications, may be performed.

1. Department of Radiology, St. Elisabeth Hospital, Zottegem, Belgium.


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09-11.12.10 COURS INTENSIFS DE TDM MULTICOUPE DU THORAX Lille, France Information: secrétariat du Serv. de Radiologie, Hôp. Calmette, Bld du Pr Leclerc, F-59037 Lille Cedex Tel.: 03-20-44 43 11 Fax: 03-20-44 47 20 e-mail: mremy-jardin@chru-lille.fr 16.01.11 EUROPEAN SOCIETY FOR MOLECULAR IMAGING : EMERGING MEDICINE 2011 LES HOUCHES, FRANCES Information: http://www.e-smi.eu 24-28.01.11 ERASMUS COURSE HEAD & NECK MRI LISBON, PORTUGAL Information: www.erasmus2011.com.pt 10.02.11 JOINT INTERVENTIONAL MEETING ROME, ITALY INFORMATION: WWW.JIMVASCULAR.COM

23.02.11 ANNUAL SYMPOSIUM OF THE AMERICAN SOCIETY OF SPINE RADIOLOGY 2011 USA, Hawaii Information: http://theassr.org 09-11.06.11 CRETE, GREECE 18TH EUROPEAN SOCIETY OF MUSCULOSKELETAL RADIOLOGY MEETING (ESSR) Main theme refresher course: Bone marrow. Additional refresher courses on different MSK topics. Scientific papers on all aspects of MSK Radiology Congress president: Prof.dr. A. Karantanas Information: 30-2810392078 08.11.11 11TH WORLD FEDERATION OF INTERVENTIONAL AND THERAPEUTIC NEURORADIOLOGY CONGRESS SOUTH AFRICA, CAPE TOWN Information: www.wfitn2011.org


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