Arab Journal of Gastroenterology xxx (2015) xxx–xxx
Contents lists available at ScienceDirect
Arab Journal of Gastroenterology journal homepage: www.elsevier.com/locate/ajg
Case Report
Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome Rym Ennaifer a,c,⇑, Saloua B’chir Hamzaoui b,c, Thara Larbi b,c, Hayfa Romdhane a,c, Maya Abdallah b,c, Najet Bel Hadj a,c, Sander M’rad b,c a b c
Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia Department of Internal Medicine, Mongi Slim Hospital, Tunis, Tunisia Université de Tunis El Manar, Faculté de Médecine de Tunis, Tunis, Tunisia
a r t i c l e
i n f o
Article history: Received 27 July 2014 Accepted 2 January 2015 Available online xxxx Keywords: Oesophageal varices Superior vena cava syndrome Behçet’s disease Gastrointestinal bleeding
a b s t r a c t Behçet’s disease (BD) is a multisystemic disorder that involves vessels of all sizes. Superior vena cava (SVC) thrombosis is a rare complication that can lead to the development of various collateral pathways. A 31-year-old man presented with SVC syndrome. He had a history of recurrent genital aphthosis. Computed tomography revealed extensive thrombosis of the right internal jugular, axillary, and subclavian veins with collateral circulation. The patient was diagnosed with BD, and he was started on anticoagulation and immunosuppressive therapy. One week later, he presented with haematemesis. Upper gastrointestinal endoscopy disclosed varices in the upper third of the oesophagus with stigmata of recent bleeding. Portal hypertension was ruled out. Anticoagulation therapy was discontinued. He was discharged on immunosuppressive therapy. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD. Ó 2015 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.
Introduction
Case report
Behçet’s disease (BD) is a chronic inflammatory disease characterised by recurrent oral and genital ulceration as well as ocular involvement. Many other systems can be affected including skin, joints, cardiovascular, gastrointestinal, and nervous systems. Vasculitis is the common denominator in this multisystemic disease, and it is distinctive among the classical vasculitides in that it involves vessels of all sizes and both arteries and veins [1,2]. Vascular involvement is manifested most often as venous thrombosis, occurring in 25–35% of patients [3–5]. Superior vena cava (SVC) syndrome is rare, occurring in 2% of the patients in a large Tunisian multicentric study [5]. Gradual obstruction of the SVC or its major tributaries results in the development of various venous collaterals. We report an unusual pattern of collateral development in a patient with BD-related SVC syndrome causing proximal oesophageal varices that resulted in upper gastrointestinal bleeding.
A 31-year-old male presented with fever, dyspnoea, hoarseness, and progressive facial and cervical swelling for 4 months. He reported no history of thrombosis or a family history of similar conditions, but he had recurrent genital aphthosis for 5 years. Significant physical findings included swelling of the upper trunk, prominent superficial veins of the chest, and multiple scars of scrotal ulcers. Computed tomography (CT) revealed extensive thrombosis involving the right internal jugular, axillary, and subclavian veins; partial occlusion of the left internal jugular and axillary veins; and a profuse amount of cervicothoracic collateral circulation (Fig. 1). No mediastinal mass was observed. The patient was diagnosed with BD. There was no evidence of other skin or systemic involvement, and ophthalmologic examination was normal. Initial laboratory test values included the following: haemoglobin (Hb) 11.7 g/dl, haematocrit 37.3%, platelet count 463,000/mm3, and prothrombin time 13 s. Antinuclear, anticardiolipin, and anti-b2 glycoprotein 1 antibodies were negative. A combination of anticoagulant treatment (enoxaparin followed by acenocoumarol) and immunosuppressive treatment (prednisone 1 mg/kg/day) was initiated, allowing initial improvement of the SVC syndrome. Unfortunately, 1 week later, he presented with upper gastrointestinal
⇑ Corresponding author at: Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia. Tel.: +216 22 764 106; fax: +216 70 938 616. E-mail address: [email protected] (R. Ennaifer).
http://dx.doi.org/10.1016/j.ajg.2015.02.003 1687-1979/Ó 2015 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.
Please cite this article in press as: Ennaifer R et al. Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/j.ajg.2015.02.003
2
R. Ennaifer et al. / Arab Journal of Gastroenterology xxx (2015) xxx–xxx
haemorrhage with hypotension. The Hb level decreased to 7 g/dl. At this time, the international rationalised ratio (INR) was 3.09. Subsequently, the anticoagulant treatment was discontinued, and the patient received units of packed red blood cells. Initial emergency upper gastrointestinal endoscopy disclosed fresh blood in the oesophagus and the stomach, which did not allow accurate exploration. The bleeding source could not be identified. Because of underlying vasculitis, the diagnosis of portal vein or hepatic vein thrombosis and bleeding due to oesophageal varices was considered. The patient received intravenous infusion of octreotide and intravenous infusion of proton pump inhibitors. After stabilisation, the patient underwent a second endoscopy (24 h later) that showed two prominent oesophageal varices in the upper half of the oesophagus with an overlying clot, which correlated with recent bleeding. The presence of chronic liver disease or portal hypertension as well as thrombosis of hepatic veins was ruled out with CT and Doppler sonography. Moreover, liver tests and platelet count were within the normal range, which was not in favour of liver fibrosis. Afterwards, the bleeding stopped, and as no more signs of red colour or stigmata of bleeding were observed at re-endoscopy, we did not perform band ligation (Fig. 2). Nevertheless, anticoagulation therapy was not started again. He was discharged from the hospital with colchicine, prednisone, and azathioprine (150 mg/day) without any further episodes of bleeding with hindsight of 6 months. Discussion This case of downhill bleeding oesophageal varices induced by SVC syndrome is a relatively rare symptom of BD. The classic triad described by Behçet consists of oral ulcerations, genital ulcerations, and uveitis. Even if our patient did not fulfill the International Society Study Group criteria, he was diagnosed with BD. This diagnosis was based on his history of recurrent genital ulcerations and because BD is the main nonneoplastic cause of SVC syndrome in young males in our country. It is, in fact, well known that 3% of patients with BD may not experience oral ulcerations [6], and many incomplete forms have been reported, especially in patients with vascular involvement [7,8]. On the other hand, the diagnosis of downhill variceal bleeding was the most likely, even if active bleeding from the varix was
Fig. 1. Spiral computed tomography showing thrombosis involving the right internal jugular, axillary, and subclavian veins and cervicothoracic collateral circulation.
Fig. 2. Endoscopic finding in upper third of oesophagus: Downhill varices.
not highlighted at the first endoscopy. Indeed, the diagnosis of variceal bleeding is usually made using the following criteria: active bleeding from a varix, the presence of a ‘‘white nipple’’ fibrin clot overlying a varix, a clot on a varix, the presence of varices without other potential sources of bleeding, and fresh blood in the stomach [9]. SVC syndrome is secondary to the obstruction of SVC or its major tributaries. It results in the development of various venous collateral pathways. The most important are azygos–hemiazygos, paravertebral, internal mammary, lateral thoracic, and thoracoepigastric veins, and the anterior jugular venous system [10]. Small mediastinal veins including pericardial and perioesophageal veins were more rarely reported among the collateral pathways [11]. Oesophageal varices associated with SVC thrombosis are called ‘‘downhill’’ oesophageal varices. They develop in the upper or middle third of the oesophagus [12,13]. Downhill varices serve as collateral branches directing blood flow downward, either to bypass superior vena obstruction via the azygos vein or to drain the superior systemic system when both the SVC and the azygos vein are obstructed. Downhill oesophageal varices are mostly due to SVC syndrome secondary to mass effects including lung cancers, intrathoracic goitre, mediastinal lymphoma, thyroid carcinoma, thymoma, and mediastinal lymphadenopathy [14,15]. Downhill oesophageal varices due to SVC syndrome secondary to BD are very rare. In our knowledge, only four previous cases have been reported; among them, only two had bleeding [16–19]. Bleeding from varices associated with the SVC syndrome is far less common than that from varices due to portal hypertension; therefore, they may be underestimated. However, the bleeding could be life-threatening [16]. In our case, anticoagulation may be an additive factor to explain bleeding even if our patient’s INR was in the therapeutic range for the treatment of venous thrombosis. Therefore, we considered that anticoagulation therapy may be harmful as long as the varices persist. There is no agreement on the treatment of major venous disease in BD [20,21]. As the main pathology is the intense inflammation of the vessel walls that lead to thrombi, immunosuppressive agents are usually recommended. However, some authors consider the use of anticoagulants controversial in that the venous thrombi in BD adhere to the vessel wall and pulmonary embolism is rare. Another reason to avoid anticoagulation is the possibility of a coexisting pulmonary aneurysm, which might result in fatal bleeding.
Please cite this article in press as: Ennaifer R et al. Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/j.ajg.2015.02.003
R. Ennaifer et al. / Arab Journal of Gastroenterology xxx (2015) xxx–xxx
In the case of SVC syndrome, even if rare, the possibility of proximal oesophageal varices should also be considered before introducing anticoagulation. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD. Therapeutic management in these cases may be difficult given the risks of anticoagulation, and efforts should rather be directed towards the vasculitis. Conflict of interest The authors declared that there was no conflict of interest. References [1] O’Duffy JD. Vasculitis in Behçet’s disease. Rheum Dis Clin North Am 1990;16: 423–31. [2] Lie JT. Vascular involvement in Behçet’s disease: arterial and venous and vessels of all sizes. J Rheumatol 1992;19:341–3. [3] Houman MH, Ben Ghorbel I, Khiari Ben Salah I. Deep vein thrombosis in Behçet’s disease. Clin Exp Rheumatol 2001;19:S48–50. [4] Wechsler B, Piette JC, Conard J, et al. Les thromboses veineuses profondes dans la maladie de Behçet. 106 localisations sur une série de 177 malades. Presse Med 1987;16:661–4. [5] B0 chir Hamzaoui S, Harmel A, Bouslama K, et al. Behçet’s disease in Tunisia. Clinical study of 519 cases. Rev Med Int 2006;27:742–50. [6] Yazici H, Yazici Y. Criteria for Behçet’s disease with reflections on all disease criteria. J Autoimmun 2014;48–49:104–7. [7] Yu M, Shi A, Jin B, Jiang X, Liang H, Ouyang C. Superior vena cava occlusion caused by Behçet disease. J Vasc Surg 2012;55:1488–91. [8] Vandergheynst F, Francois O, Laureys M, Decaux G. Superior vena cava syndrome without thrombosis revealing Behçet’s disease: two cases. Joint Bone Spine 2008;75:359–61.
3
[9] Cremers I, Ribeiro S. Management of variceal and nonvariceal upper gastrointestinal bleeding in patients with cirrhosis. Therap Adv Gastroenterol 2014;7:206–16. [10] Bettmann MB, Steinberg I. The superior vena cava. In: Abrams HL, editor. Abrams angiography. Vascular and interventional radiology. Little Boston: Brown and Company; 1983. p. 924–35. [11] Bashist B, Parisi A, Frager DH, Suster B. Abdominal CT findings when the superior vena cava, brachiocephalic vein, or subclavian vein is obstructed. AJR Am J Roentgenol 1996;167:1457–63. [12] Palmer ED. Primary varices of the cervical esophagus as a source of massive upper gastrointestinal hemorrhage. Am J Dig Dis 1952;19:375–7. [13] Otto DL, Kurtzman RS. Esophageal varices in superior vena cava obstruction. AJR Am J Roentgenol 1964;92:1000–12. [14] Savoy A, Wolfsen H, Paz-Fumagalli R, Raimondo M. Endoscopic therapy for bleeding proximal esophageal varices: a case report. Gastrointest Endosc 2004;59:310–3. [15] Bedard E, Deslauriers J. Bleeding, ‘‘Downhill’’ varices: a rare complication of intrathoracic goiter. Ann Thorac Surg 2006;81:358–60. [16] Tavakkoli H, Asadi M, Haghighi M, Esmaeili A. Therapeutic approach to downhill esophageal varices bleeding due to superior vena cava syndrome in Behçet’s disease: a case report. BMC Gastroenterol 2006;6:43. [17] Ishikawa R, Noguchi T, Matsumoto K. A case of downhill esophageal varices – Behçet disease associated with abdominal aortic aneurysm and occlusions of the superior and inferior vena cava. Nihon Geka Gakkai Zasshi 1986;7: 1576–82. [18] Orikasa H, Ejiri Y, Suzuki S, et al. A case of Behçet’s disease with occlusion of both caval veins and downhill esophageal varices. J Gastroenterol 1994;29:506–10. [19] Tsuji S, Suzuki Y, Tomii M, et al. Behçet’s disease associated with multiple cerebral aneurysms and downhill esophageal varices caused by superior vena cava obstruction: a case report. Ryumachi 1990;30:375–9. [20] Hatemi G, Silman A, Bang D, et al. Management of Behçet’s disease: a systematic review for the EULAR evidence-based recommendations for the management of Behçet’s disease. Ann Rheum Dis 2008;67:1656–62. [21] Wechsler B, Le Thi Huong DB, Saadoun D. EULAR recommendations for the management of Behçet’s disease: evidence-based or experience-based medicine. Rev Med Int 2009;30:939–41.
Please cite this article in press as: Ennaifer R et al. Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/j.ajg.2015.02.003
Contents lists available at ScienceDirect
Arab Journal of Gastroenterology journal homepage: www.elsevier.com/locate/ajg
Case Report
Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome Rym Ennaifer a,c,⇑, Saloua B’chir Hamzaoui b,c, Thara Larbi b,c, Hayfa Romdhane a,c, Maya Abdallah b,c, Najet Bel Hadj a,c, Sander M’rad b,c a b c
Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia Department of Internal Medicine, Mongi Slim Hospital, Tunis, Tunisia Université de Tunis El Manar, Faculté de Médecine de Tunis, Tunis, Tunisia
a r t i c l e
i n f o
Article history: Received 27 July 2014 Accepted 2 January 2015 Available online xxxx Keywords: Oesophageal varices Superior vena cava syndrome Behçet’s disease Gastrointestinal bleeding
a b s t r a c t Behçet’s disease (BD) is a multisystemic disorder that involves vessels of all sizes. Superior vena cava (SVC) thrombosis is a rare complication that can lead to the development of various collateral pathways. A 31-year-old man presented with SVC syndrome. He had a history of recurrent genital aphthosis. Computed tomography revealed extensive thrombosis of the right internal jugular, axillary, and subclavian veins with collateral circulation. The patient was diagnosed with BD, and he was started on anticoagulation and immunosuppressive therapy. One week later, he presented with haematemesis. Upper gastrointestinal endoscopy disclosed varices in the upper third of the oesophagus with stigmata of recent bleeding. Portal hypertension was ruled out. Anticoagulation therapy was discontinued. He was discharged on immunosuppressive therapy. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD. Ó 2015 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.
Introduction
Case report
Behçet’s disease (BD) is a chronic inflammatory disease characterised by recurrent oral and genital ulceration as well as ocular involvement. Many other systems can be affected including skin, joints, cardiovascular, gastrointestinal, and nervous systems. Vasculitis is the common denominator in this multisystemic disease, and it is distinctive among the classical vasculitides in that it involves vessels of all sizes and both arteries and veins [1,2]. Vascular involvement is manifested most often as venous thrombosis, occurring in 25–35% of patients [3–5]. Superior vena cava (SVC) syndrome is rare, occurring in 2% of the patients in a large Tunisian multicentric study [5]. Gradual obstruction of the SVC or its major tributaries results in the development of various venous collaterals. We report an unusual pattern of collateral development in a patient with BD-related SVC syndrome causing proximal oesophageal varices that resulted in upper gastrointestinal bleeding.
A 31-year-old male presented with fever, dyspnoea, hoarseness, and progressive facial and cervical swelling for 4 months. He reported no history of thrombosis or a family history of similar conditions, but he had recurrent genital aphthosis for 5 years. Significant physical findings included swelling of the upper trunk, prominent superficial veins of the chest, and multiple scars of scrotal ulcers. Computed tomography (CT) revealed extensive thrombosis involving the right internal jugular, axillary, and subclavian veins; partial occlusion of the left internal jugular and axillary veins; and a profuse amount of cervicothoracic collateral circulation (Fig. 1). No mediastinal mass was observed. The patient was diagnosed with BD. There was no evidence of other skin or systemic involvement, and ophthalmologic examination was normal. Initial laboratory test values included the following: haemoglobin (Hb) 11.7 g/dl, haematocrit 37.3%, platelet count 463,000/mm3, and prothrombin time 13 s. Antinuclear, anticardiolipin, and anti-b2 glycoprotein 1 antibodies were negative. A combination of anticoagulant treatment (enoxaparin followed by acenocoumarol) and immunosuppressive treatment (prednisone 1 mg/kg/day) was initiated, allowing initial improvement of the SVC syndrome. Unfortunately, 1 week later, he presented with upper gastrointestinal
⇑ Corresponding author at: Department of Hepato-gastro-enterology, Mongi Slim Hospital, Tunis, Tunisia. Tel.: +216 22 764 106; fax: +216 70 938 616. E-mail address: [email protected] (R. Ennaifer).
http://dx.doi.org/10.1016/j.ajg.2015.02.003 1687-1979/Ó 2015 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.
Please cite this article in press as: Ennaifer R et al. Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/j.ajg.2015.02.003
2
R. Ennaifer et al. / Arab Journal of Gastroenterology xxx (2015) xxx–xxx
haemorrhage with hypotension. The Hb level decreased to 7 g/dl. At this time, the international rationalised ratio (INR) was 3.09. Subsequently, the anticoagulant treatment was discontinued, and the patient received units of packed red blood cells. Initial emergency upper gastrointestinal endoscopy disclosed fresh blood in the oesophagus and the stomach, which did not allow accurate exploration. The bleeding source could not be identified. Because of underlying vasculitis, the diagnosis of portal vein or hepatic vein thrombosis and bleeding due to oesophageal varices was considered. The patient received intravenous infusion of octreotide and intravenous infusion of proton pump inhibitors. After stabilisation, the patient underwent a second endoscopy (24 h later) that showed two prominent oesophageal varices in the upper half of the oesophagus with an overlying clot, which correlated with recent bleeding. The presence of chronic liver disease or portal hypertension as well as thrombosis of hepatic veins was ruled out with CT and Doppler sonography. Moreover, liver tests and platelet count were within the normal range, which was not in favour of liver fibrosis. Afterwards, the bleeding stopped, and as no more signs of red colour or stigmata of bleeding were observed at re-endoscopy, we did not perform band ligation (Fig. 2). Nevertheless, anticoagulation therapy was not started again. He was discharged from the hospital with colchicine, prednisone, and azathioprine (150 mg/day) without any further episodes of bleeding with hindsight of 6 months. Discussion This case of downhill bleeding oesophageal varices induced by SVC syndrome is a relatively rare symptom of BD. The classic triad described by Behçet consists of oral ulcerations, genital ulcerations, and uveitis. Even if our patient did not fulfill the International Society Study Group criteria, he was diagnosed with BD. This diagnosis was based on his history of recurrent genital ulcerations and because BD is the main nonneoplastic cause of SVC syndrome in young males in our country. It is, in fact, well known that 3% of patients with BD may not experience oral ulcerations [6], and many incomplete forms have been reported, especially in patients with vascular involvement [7,8]. On the other hand, the diagnosis of downhill variceal bleeding was the most likely, even if active bleeding from the varix was
Fig. 1. Spiral computed tomography showing thrombosis involving the right internal jugular, axillary, and subclavian veins and cervicothoracic collateral circulation.
Fig. 2. Endoscopic finding in upper third of oesophagus: Downhill varices.
not highlighted at the first endoscopy. Indeed, the diagnosis of variceal bleeding is usually made using the following criteria: active bleeding from a varix, the presence of a ‘‘white nipple’’ fibrin clot overlying a varix, a clot on a varix, the presence of varices without other potential sources of bleeding, and fresh blood in the stomach [9]. SVC syndrome is secondary to the obstruction of SVC or its major tributaries. It results in the development of various venous collateral pathways. The most important are azygos–hemiazygos, paravertebral, internal mammary, lateral thoracic, and thoracoepigastric veins, and the anterior jugular venous system [10]. Small mediastinal veins including pericardial and perioesophageal veins were more rarely reported among the collateral pathways [11]. Oesophageal varices associated with SVC thrombosis are called ‘‘downhill’’ oesophageal varices. They develop in the upper or middle third of the oesophagus [12,13]. Downhill varices serve as collateral branches directing blood flow downward, either to bypass superior vena obstruction via the azygos vein or to drain the superior systemic system when both the SVC and the azygos vein are obstructed. Downhill oesophageal varices are mostly due to SVC syndrome secondary to mass effects including lung cancers, intrathoracic goitre, mediastinal lymphoma, thyroid carcinoma, thymoma, and mediastinal lymphadenopathy [14,15]. Downhill oesophageal varices due to SVC syndrome secondary to BD are very rare. In our knowledge, only four previous cases have been reported; among them, only two had bleeding [16–19]. Bleeding from varices associated with the SVC syndrome is far less common than that from varices due to portal hypertension; therefore, they may be underestimated. However, the bleeding could be life-threatening [16]. In our case, anticoagulation may be an additive factor to explain bleeding even if our patient’s INR was in the therapeutic range for the treatment of venous thrombosis. Therefore, we considered that anticoagulation therapy may be harmful as long as the varices persist. There is no agreement on the treatment of major venous disease in BD [20,21]. As the main pathology is the intense inflammation of the vessel walls that lead to thrombi, immunosuppressive agents are usually recommended. However, some authors consider the use of anticoagulants controversial in that the venous thrombi in BD adhere to the vessel wall and pulmonary embolism is rare. Another reason to avoid anticoagulation is the possibility of a coexisting pulmonary aneurysm, which might result in fatal bleeding.
Please cite this article in press as: Ennaifer R et al. Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/j.ajg.2015.02.003
R. Ennaifer et al. / Arab Journal of Gastroenterology xxx (2015) xxx–xxx
In the case of SVC syndrome, even if rare, the possibility of proximal oesophageal varices should also be considered before introducing anticoagulation. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD. Therapeutic management in these cases may be difficult given the risks of anticoagulation, and efforts should rather be directed towards the vasculitis. Conflict of interest The authors declared that there was no conflict of interest. References [1] O’Duffy JD. Vasculitis in Behçet’s disease. Rheum Dis Clin North Am 1990;16: 423–31. [2] Lie JT. Vascular involvement in Behçet’s disease: arterial and venous and vessels of all sizes. J Rheumatol 1992;19:341–3. [3] Houman MH, Ben Ghorbel I, Khiari Ben Salah I. Deep vein thrombosis in Behçet’s disease. Clin Exp Rheumatol 2001;19:S48–50. [4] Wechsler B, Piette JC, Conard J, et al. Les thromboses veineuses profondes dans la maladie de Behçet. 106 localisations sur une série de 177 malades. Presse Med 1987;16:661–4. [5] B0 chir Hamzaoui S, Harmel A, Bouslama K, et al. Behçet’s disease in Tunisia. Clinical study of 519 cases. Rev Med Int 2006;27:742–50. [6] Yazici H, Yazici Y. Criteria for Behçet’s disease with reflections on all disease criteria. J Autoimmun 2014;48–49:104–7. [7] Yu M, Shi A, Jin B, Jiang X, Liang H, Ouyang C. Superior vena cava occlusion caused by Behçet disease. J Vasc Surg 2012;55:1488–91. [8] Vandergheynst F, Francois O, Laureys M, Decaux G. Superior vena cava syndrome without thrombosis revealing Behçet’s disease: two cases. Joint Bone Spine 2008;75:359–61.
3
[9] Cremers I, Ribeiro S. Management of variceal and nonvariceal upper gastrointestinal bleeding in patients with cirrhosis. Therap Adv Gastroenterol 2014;7:206–16. [10] Bettmann MB, Steinberg I. The superior vena cava. In: Abrams HL, editor. Abrams angiography. Vascular and interventional radiology. Little Boston: Brown and Company; 1983. p. 924–35. [11] Bashist B, Parisi A, Frager DH, Suster B. Abdominal CT findings when the superior vena cava, brachiocephalic vein, or subclavian vein is obstructed. AJR Am J Roentgenol 1996;167:1457–63. [12] Palmer ED. Primary varices of the cervical esophagus as a source of massive upper gastrointestinal hemorrhage. Am J Dig Dis 1952;19:375–7. [13] Otto DL, Kurtzman RS. Esophageal varices in superior vena cava obstruction. AJR Am J Roentgenol 1964;92:1000–12. [14] Savoy A, Wolfsen H, Paz-Fumagalli R, Raimondo M. Endoscopic therapy for bleeding proximal esophageal varices: a case report. Gastrointest Endosc 2004;59:310–3. [15] Bedard E, Deslauriers J. Bleeding, ‘‘Downhill’’ varices: a rare complication of intrathoracic goiter. Ann Thorac Surg 2006;81:358–60. [16] Tavakkoli H, Asadi M, Haghighi M, Esmaeili A. Therapeutic approach to downhill esophageal varices bleeding due to superior vena cava syndrome in Behçet’s disease: a case report. BMC Gastroenterol 2006;6:43. [17] Ishikawa R, Noguchi T, Matsumoto K. A case of downhill esophageal varices – Behçet disease associated with abdominal aortic aneurysm and occlusions of the superior and inferior vena cava. Nihon Geka Gakkai Zasshi 1986;7: 1576–82. [18] Orikasa H, Ejiri Y, Suzuki S, et al. A case of Behçet’s disease with occlusion of both caval veins and downhill esophageal varices. J Gastroenterol 1994;29:506–10. [19] Tsuji S, Suzuki Y, Tomii M, et al. Behçet’s disease associated with multiple cerebral aneurysms and downhill esophageal varices caused by superior vena cava obstruction: a case report. Ryumachi 1990;30:375–9. [20] Hatemi G, Silman A, Bang D, et al. Management of Behçet’s disease: a systematic review for the EULAR evidence-based recommendations for the management of Behçet’s disease. Ann Rheum Dis 2008;67:1656–62. [21] Wechsler B, Le Thi Huong DB, Saadoun D. EULAR recommendations for the management of Behçet’s disease: evidence-based or experience-based medicine. Rev Med Int 2009;30:939–41.
Please cite this article in press as: Ennaifer R et al. Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome. Arab J Gastroenterol (2015), http://dx.doi.org/10.1016/j.ajg.2015.02.003
