Clinical Imaging xxx (2015) xxx–xxx
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Case report
Massive superior mesenteric venous aneurysm with portal venous thrombosis Anna Starikov, Roger J. Bartolotta ⁎ NewYork-Presbyterian Hospital/Weill Cornell Medical College, 525 E. 68th Street, New York, NY 10065
a r t i c l e
i n f o
Article history: Received 8 April 2015 Accepted 3 May 2015 Available online xxxx Keywords: Portal venous aneurysm Portal venous thrombosis Pancreatitis Computed tomography (CT) Magnetic resonance imaging (MRI)
a b s t r a c t Portal venous aneurysm is a rare and sometimes dangerous vascular pathology, which can result in thrombosis or rupture. We present the computed tomography, magnetic resonance, and sonographic imaging of a 27-year-old man with superior mesenteric venous aneurysm and subsequent thrombosis following acute pancreatitis. This multimodality imaging approach can prove useful in the evaluation of these rare aneurysms. © 2015 Published by Elsevier Inc.
1. Introduction Visceral venous aneurysms are much rarer than arterial or nonvisceral venous aneurysms and account for only 3% of all venous aneurysms [1]. The majority of visceral venous aneurysms occur within the portal venous system, particularly within the main portal vein or at the junction of the splenic and superior mesenteric veins (SMVs) [1–3]. In this case report, we present the computed tomography (CT), magnetic resonance (MR), and sonographic imaging of a previously healthy young male discovered to have a massive superior mesenteric venous aneurysm with portal venous thrombosis. Portal venous aneurysms can be congenital or acquired. Congenital aneurysms typically arise from aberrant development of the vitilline veins during the embryonic period or from an inborn weakness in the wall of the vessel and frequently remain stable with follow-up [4,5]. The cause of acquired aneurysms has not been fully elucidated but is thought to be associated with cirrhosis, portal hypertension, pancreatitis, or trauma [1,4,6,7]. One hypothesis proposes that the thrombotic state resulting from these conditions leads to the thrombosis and occlusion of the portal vein, which can result in the formation of aneurysms secondary to hypertension [4]. Most portal venous aneurysms remain stable on follow-up, but when the diameter is found to be increasing or when complications such as thrombosis and/or rupture occur, surgical treatment should be
⁎ Corresponding author. NewYork-Presbyterian Hospital/Weill Cornell Medical College, 520 E. 70th Street, Starr 8A, New York, NY 10021. Tel.: +1-212-746-7839. E-mail address: [email protected] (R.J. Bartolotta).
considered. The most common surgical treatments include splenectomy and aneurysmorrhaphy [4]. 2. Case report A 27-year-old male without significant past medical history presented with sharp, constant, nonradiating epigastric pain. He was found to have an elevated lipase level and underwent abdominal CT with the clinical diagnosis of acute pancreatitis. Of note, he reported being only a social drinker (1–2 drinks per week) and did not have a history of gallstone disease. Abdominal CT confirmed the clinical suspicion of pancreatitis by demonstrating the typical CT findings of pancreatitis, including edematous enlargement of the pancreatic body with adjacent fat stranding (Fig. 1). More remarkably, however, images through the pancreatic neck demonstrated an 8.6 cm×7.6 cm×12.2 cm low-attenuation mass lesion centered at the pancreatic neck with a claw of pancreatic parenchyma at its periphery (Fig. 2). Multiplanar reformats confirmed the tubular configuration of this mass lesion conforming to the expected anatomic orientation of the SMV, compatible with thrombosed massive SMV aneurysm. Thrombus also extended into the splenic vein as well as the main, left, and right portal veins. To confirm nonenhancement of the thrombosed aneurysm (i.e., bland thrombus), the patient underwent contrast-enhanced magnetic resonance imaging (MRI) with magnetic resonance cholangiopancreatography (MRCP), which again demonstrated imaging findings of acute pancreatitis. The SMV aneurysm demonstrated diffuse T1 hyperintensity (not shown) and nonenhancement, compatible with bland thrombus (Fig. 3a and b). Thrombosis of the splenic and portal veins was reconfirmed. MRCP images demonstrated normal caliber of the
http://dx.doi.org/10.1016/j.clinimag.2015.05.001 0899-7071/© 2015 Published by Elsevier Inc.
Please cite this article as: Starikov A, Bartolotta RJ, Massive superior mesenteric venous aneurysm with portal venous thrombosis, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.05.001
2
A. Starikov, R.J. Bartolotta / Clinical Imaging xxx (2015) xxx–xxx
a
b Fig. 1. Axial contrast-enhanced CT image at initial presentation demonstrates edematous enlargement of the pancreatic body/tail (solid arrow) with surrounding fluid, compatible with acute pancreatitis. Note the early cavernous transformation (dashed arrow) at the porta hepatis related to portal venous thrombosis.
pancreatic duct, as well as the intrahepatic and extrahepatic bile ducts. The liver and gallbladder were normal in MR appearance. The patient was treated with heparin for portal venous thrombosis as well as conservative management of his acute pancreatitis. Although the patient appeared to improve clinically, his liver function tests began to rise after 2 weeks of hospitalization. Subsequent right upper quadrant ultrasound redemonstrated the thrombosed SMV aneurysm (Fig. 4) as well as persistent portal venous thrombosis. Follow-up MRI 2 years following the initial presentation demonstrated slight size decrease of the thrombosed SMV aneurysm (Fig. 5) and thrombosed portal veins. The follow-up MRI also showed interval increase in extensive cavernous transformation (Fig. 6), compatible with chronic portal venous thrombosis.
3. Discussion Portal venous aneurysm is a known complication of hepatic cirrhosis and portal hypertension [1,6,7]. This young, otherwise healthy patient had no evidence of primary hepatic disease. The large SMV aneurysm in this case likely arose from portal hypertension related to portal
Fig. 2. Axial contrast-enhanced CT image at initial presentation shows large hyperdense mass lesion (solid arrow) along the expected course of the SMV. Note the claw of pancreatic parenchyma (dashed arrow) surrounding the thrombosed venous aneurysm, which displaces the pancreatic parenchyma rather than invading it.
Fig. 3. (a) Axial 3D LAVA MR image following gadolinium administration confirms nonenhancement of the bland thrombus (solid arrow) within the aneurysmal SMV; (b) coronal 3D LAVA postcontrast MR image confirms the mass lesion conforms to the expected course of the SMV, compatible with thrombosed SMV aneurysm (dashed arrow).
venous thrombosis. Prior studies have reported thrombophilic defects in some patients with portal vein thrombosis [8]. This patient was evaluated for several such disorders. He had two abnormal dilute Russell’s viper venom times, done 3 months apart, which suggested the presence of antiphospholipid antibody. He also had a positive STA Clot LA assay, indicative of lupus anticoagulant. He was negative for Factor V Leiden and abnormalities in Protein C or Protein S activity and was negative for the MTHFR (C677T) mutation, which has been associated with an increased risk of venous thromboembolism [9]. Although portal venous thrombosis and portal venous aneurysms are known complications of pancreatitis [7,8,10], the cause of this patient’s pancreatitis was unclear. Genetic analysis was conducted to determine if there could be an inherited cause of his pancreatitis. The gene analysis was negative for any abnormal serine protease inhibitor Kazal-type 1 (SPINK1) mutations, which have been associated with hereditary pancreatitis [11]. He did have a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene with a singlecopy DNA alteration characterized as 2988GNC. Various abnormal CFTR alleles have been reported in patients with idiopathic chronic pancreatitis [12], but the patient’s specific mutation was not seen in these studies. Thus, it is unclear if this alteration is a benign polymorphism or a pancreatitis-causing mutation.
Please cite this article as: Starikov A, Bartolotta RJ, Massive superior mesenteric venous aneurysm with portal venous thrombosis, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.05.001
A. Starikov, R.J. Bartolotta / Clinical Imaging xxx (2015) xxx–xxx
3
Fig. 4. Sonographic image of the pancreas with color Doppler demonstrates the lack of flow within the thrombosed SMV.
strated a peripheral claw of displaced pancreatic tissue, and close attention to multiplanar reformats was necessary to differentiate this vascular lesion from a primary pancreatic mass. The heterogeneity of the thrombus within this large SMV aneurysm must not be confused for complicated pancreatic pseudocyst given the obvious risks that would be associated with attempted drainage.
4. Conclusion
Fig. 5. Axial 2D FIESTA image from follow-up MRI 2 years after initial presentation demonstrates interval size decrease of the thrombosed SMV aneurysm (solid arrow).
This case reviews the multimodality imaging of a large superior mesenteric venous aneurysm and portal venous thrombosis in a previously healthy young male. It is important to be aware of the multimodality imaging appearances of these aneurysms so that they are not mistaken for pancreatic pseudocyst or other mass lesion within the pancreas. References
Though rare overall, visceral venous aneurysm must be considered in the differential of any mass lesion presenting along the course of the portal venous system. The large SMV aneurysm in this case demon-
Fig. 6. Axial 2D FIESTA image from follow-up MRI 2 years after initial presentation exhibits interval progression of now extensive cavernous transformation (solid arrow) at the porta hepatis related to chronic portal venous thrombosis.
[1] López-Machado E, Mallorquín-Jiménez F, Medina-Benítez A, Ruiz-Carazo E, CuberoGarcía M. Aneurysms of the portal venous system: ultrasonography and CT findings. Eur J Radiol 1998;26(2):210–4. [2] Sfyroeras GS, Antoniou GA, Drakou AA, Karathanos C, Giannoukas AD. Visceral venous aneurysms: clinical presentation, natural history and their management: a systematic review. Eur J Vasc Endovasc Surg 2009;38(4):498–505. [3] Fulcher A, Turner M. Aneurysms of the portal vein and superior mesenteric vein. Abdom Imaging 1997;22(3):287–92. [4] Giannoukas AD, Sfyroeras GS. Current management of visceral venous aneurysms. Phlebolymphology 2010;16(12):130–6. [5] Gallego C, Velasco M, Marcuello P, Tejedor D, De Campo L, Friera A. Congenital and acquired anomalies of the portal venous system. Radiographics 2002;22(1):141–59. [6] Leonsins AJ, Siew S. Fusiform aneurysmal dilatation of the portal vein. Postgrad Med J 1960;36:570–4. [7] Rafiq SA, Sitrin MD. Portal vein aneurysm: case report and review of the literature. Gastroenterol Hepatol 2007;3(4):296–8. [8] Koc Z, Oguzkurt L, Ulusan S. Portal venous system aneurysms: imaging, clinical findings, and a possible new etiologic factor. AJR Am J Roentgenol 2007;189(5): 1023–30. [9] Ray JG, Shmorgun D, Chan WS. Common C677T polymorphism of the methylenetetrahydrofolate reductase gene and the risk of venous thromboembolism: metaanalysis of 31 studies. Pathophysiol Haemost Thromb 2002;32(2):51–8. [10] Heider TR, Azeem S, Galanko JA, Behrns KE. The natural history of pancreatitisinduced splenic vein thrombosis. Ann Surg 2004;239(6):876–80. [11] Weiss FU, Simon P, Witt H, Hlouschek V, Zimmer KP, Schnekenburger J, et al. SPINK1 mutations and phenotypic expression in patients with pancreatitis associated with trypsinogen mutations. J Med Genet 2003;40(4):e40. [12] Weiss FU, Simon P, Bogdanova N, Mayerle J, Dworniczak B, Horst J, et al. Complete cystic fibrosis transmembrane conductance regulator gene sequencing in patients with idiopathic chronic pancreatitis and controls. Gut 2005;54(10):1456.
Please cite this article as: Starikov A, Bartolotta RJ, Massive superior mesenteric venous aneurysm with portal venous thrombosis, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.05.001
Contents lists available at ScienceDirect
Clinical Imaging journal homepage: http://www.clinicalimaging.org
Case report
Massive superior mesenteric venous aneurysm with portal venous thrombosis Anna Starikov, Roger J. Bartolotta ⁎ NewYork-Presbyterian Hospital/Weill Cornell Medical College, 525 E. 68th Street, New York, NY 10065
a r t i c l e
i n f o
Article history: Received 8 April 2015 Accepted 3 May 2015 Available online xxxx Keywords: Portal venous aneurysm Portal venous thrombosis Pancreatitis Computed tomography (CT) Magnetic resonance imaging (MRI)
a b s t r a c t Portal venous aneurysm is a rare and sometimes dangerous vascular pathology, which can result in thrombosis or rupture. We present the computed tomography, magnetic resonance, and sonographic imaging of a 27-year-old man with superior mesenteric venous aneurysm and subsequent thrombosis following acute pancreatitis. This multimodality imaging approach can prove useful in the evaluation of these rare aneurysms. © 2015 Published by Elsevier Inc.
1. Introduction Visceral venous aneurysms are much rarer than arterial or nonvisceral venous aneurysms and account for only 3% of all venous aneurysms [1]. The majority of visceral venous aneurysms occur within the portal venous system, particularly within the main portal vein or at the junction of the splenic and superior mesenteric veins (SMVs) [1–3]. In this case report, we present the computed tomography (CT), magnetic resonance (MR), and sonographic imaging of a previously healthy young male discovered to have a massive superior mesenteric venous aneurysm with portal venous thrombosis. Portal venous aneurysms can be congenital or acquired. Congenital aneurysms typically arise from aberrant development of the vitilline veins during the embryonic period or from an inborn weakness in the wall of the vessel and frequently remain stable with follow-up [4,5]. The cause of acquired aneurysms has not been fully elucidated but is thought to be associated with cirrhosis, portal hypertension, pancreatitis, or trauma [1,4,6,7]. One hypothesis proposes that the thrombotic state resulting from these conditions leads to the thrombosis and occlusion of the portal vein, which can result in the formation of aneurysms secondary to hypertension [4]. Most portal venous aneurysms remain stable on follow-up, but when the diameter is found to be increasing or when complications such as thrombosis and/or rupture occur, surgical treatment should be
⁎ Corresponding author. NewYork-Presbyterian Hospital/Weill Cornell Medical College, 520 E. 70th Street, Starr 8A, New York, NY 10021. Tel.: +1-212-746-7839. E-mail address: [email protected] (R.J. Bartolotta).
considered. The most common surgical treatments include splenectomy and aneurysmorrhaphy [4]. 2. Case report A 27-year-old male without significant past medical history presented with sharp, constant, nonradiating epigastric pain. He was found to have an elevated lipase level and underwent abdominal CT with the clinical diagnosis of acute pancreatitis. Of note, he reported being only a social drinker (1–2 drinks per week) and did not have a history of gallstone disease. Abdominal CT confirmed the clinical suspicion of pancreatitis by demonstrating the typical CT findings of pancreatitis, including edematous enlargement of the pancreatic body with adjacent fat stranding (Fig. 1). More remarkably, however, images through the pancreatic neck demonstrated an 8.6 cm×7.6 cm×12.2 cm low-attenuation mass lesion centered at the pancreatic neck with a claw of pancreatic parenchyma at its periphery (Fig. 2). Multiplanar reformats confirmed the tubular configuration of this mass lesion conforming to the expected anatomic orientation of the SMV, compatible with thrombosed massive SMV aneurysm. Thrombus also extended into the splenic vein as well as the main, left, and right portal veins. To confirm nonenhancement of the thrombosed aneurysm (i.e., bland thrombus), the patient underwent contrast-enhanced magnetic resonance imaging (MRI) with magnetic resonance cholangiopancreatography (MRCP), which again demonstrated imaging findings of acute pancreatitis. The SMV aneurysm demonstrated diffuse T1 hyperintensity (not shown) and nonenhancement, compatible with bland thrombus (Fig. 3a and b). Thrombosis of the splenic and portal veins was reconfirmed. MRCP images demonstrated normal caliber of the
http://dx.doi.org/10.1016/j.clinimag.2015.05.001 0899-7071/© 2015 Published by Elsevier Inc.
Please cite this article as: Starikov A, Bartolotta RJ, Massive superior mesenteric venous aneurysm with portal venous thrombosis, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.05.001
2
A. Starikov, R.J. Bartolotta / Clinical Imaging xxx (2015) xxx–xxx
a
b Fig. 1. Axial contrast-enhanced CT image at initial presentation demonstrates edematous enlargement of the pancreatic body/tail (solid arrow) with surrounding fluid, compatible with acute pancreatitis. Note the early cavernous transformation (dashed arrow) at the porta hepatis related to portal venous thrombosis.
pancreatic duct, as well as the intrahepatic and extrahepatic bile ducts. The liver and gallbladder were normal in MR appearance. The patient was treated with heparin for portal venous thrombosis as well as conservative management of his acute pancreatitis. Although the patient appeared to improve clinically, his liver function tests began to rise after 2 weeks of hospitalization. Subsequent right upper quadrant ultrasound redemonstrated the thrombosed SMV aneurysm (Fig. 4) as well as persistent portal venous thrombosis. Follow-up MRI 2 years following the initial presentation demonstrated slight size decrease of the thrombosed SMV aneurysm (Fig. 5) and thrombosed portal veins. The follow-up MRI also showed interval increase in extensive cavernous transformation (Fig. 6), compatible with chronic portal venous thrombosis.
3. Discussion Portal venous aneurysm is a known complication of hepatic cirrhosis and portal hypertension [1,6,7]. This young, otherwise healthy patient had no evidence of primary hepatic disease. The large SMV aneurysm in this case likely arose from portal hypertension related to portal
Fig. 2. Axial contrast-enhanced CT image at initial presentation shows large hyperdense mass lesion (solid arrow) along the expected course of the SMV. Note the claw of pancreatic parenchyma (dashed arrow) surrounding the thrombosed venous aneurysm, which displaces the pancreatic parenchyma rather than invading it.
Fig. 3. (a) Axial 3D LAVA MR image following gadolinium administration confirms nonenhancement of the bland thrombus (solid arrow) within the aneurysmal SMV; (b) coronal 3D LAVA postcontrast MR image confirms the mass lesion conforms to the expected course of the SMV, compatible with thrombosed SMV aneurysm (dashed arrow).
venous thrombosis. Prior studies have reported thrombophilic defects in some patients with portal vein thrombosis [8]. This patient was evaluated for several such disorders. He had two abnormal dilute Russell’s viper venom times, done 3 months apart, which suggested the presence of antiphospholipid antibody. He also had a positive STA Clot LA assay, indicative of lupus anticoagulant. He was negative for Factor V Leiden and abnormalities in Protein C or Protein S activity and was negative for the MTHFR (C677T) mutation, which has been associated with an increased risk of venous thromboembolism [9]. Although portal venous thrombosis and portal venous aneurysms are known complications of pancreatitis [7,8,10], the cause of this patient’s pancreatitis was unclear. Genetic analysis was conducted to determine if there could be an inherited cause of his pancreatitis. The gene analysis was negative for any abnormal serine protease inhibitor Kazal-type 1 (SPINK1) mutations, which have been associated with hereditary pancreatitis [11]. He did have a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene with a singlecopy DNA alteration characterized as 2988GNC. Various abnormal CFTR alleles have been reported in patients with idiopathic chronic pancreatitis [12], but the patient’s specific mutation was not seen in these studies. Thus, it is unclear if this alteration is a benign polymorphism or a pancreatitis-causing mutation.
Please cite this article as: Starikov A, Bartolotta RJ, Massive superior mesenteric venous aneurysm with portal venous thrombosis, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.05.001
A. Starikov, R.J. Bartolotta / Clinical Imaging xxx (2015) xxx–xxx
3
Fig. 4. Sonographic image of the pancreas with color Doppler demonstrates the lack of flow within the thrombosed SMV.
strated a peripheral claw of displaced pancreatic tissue, and close attention to multiplanar reformats was necessary to differentiate this vascular lesion from a primary pancreatic mass. The heterogeneity of the thrombus within this large SMV aneurysm must not be confused for complicated pancreatic pseudocyst given the obvious risks that would be associated with attempted drainage.
4. Conclusion
Fig. 5. Axial 2D FIESTA image from follow-up MRI 2 years after initial presentation demonstrates interval size decrease of the thrombosed SMV aneurysm (solid arrow).
This case reviews the multimodality imaging of a large superior mesenteric venous aneurysm and portal venous thrombosis in a previously healthy young male. It is important to be aware of the multimodality imaging appearances of these aneurysms so that they are not mistaken for pancreatic pseudocyst or other mass lesion within the pancreas. References
Though rare overall, visceral venous aneurysm must be considered in the differential of any mass lesion presenting along the course of the portal venous system. The large SMV aneurysm in this case demon-
Fig. 6. Axial 2D FIESTA image from follow-up MRI 2 years after initial presentation exhibits interval progression of now extensive cavernous transformation (solid arrow) at the porta hepatis related to chronic portal venous thrombosis.
[1] López-Machado E, Mallorquín-Jiménez F, Medina-Benítez A, Ruiz-Carazo E, CuberoGarcía M. Aneurysms of the portal venous system: ultrasonography and CT findings. Eur J Radiol 1998;26(2):210–4. [2] Sfyroeras GS, Antoniou GA, Drakou AA, Karathanos C, Giannoukas AD. Visceral venous aneurysms: clinical presentation, natural history and their management: a systematic review. Eur J Vasc Endovasc Surg 2009;38(4):498–505. [3] Fulcher A, Turner M. Aneurysms of the portal vein and superior mesenteric vein. Abdom Imaging 1997;22(3):287–92. [4] Giannoukas AD, Sfyroeras GS. Current management of visceral venous aneurysms. Phlebolymphology 2010;16(12):130–6. [5] Gallego C, Velasco M, Marcuello P, Tejedor D, De Campo L, Friera A. Congenital and acquired anomalies of the portal venous system. Radiographics 2002;22(1):141–59. [6] Leonsins AJ, Siew S. Fusiform aneurysmal dilatation of the portal vein. Postgrad Med J 1960;36:570–4. [7] Rafiq SA, Sitrin MD. Portal vein aneurysm: case report and review of the literature. Gastroenterol Hepatol 2007;3(4):296–8. [8] Koc Z, Oguzkurt L, Ulusan S. Portal venous system aneurysms: imaging, clinical findings, and a possible new etiologic factor. AJR Am J Roentgenol 2007;189(5): 1023–30. [9] Ray JG, Shmorgun D, Chan WS. Common C677T polymorphism of the methylenetetrahydrofolate reductase gene and the risk of venous thromboembolism: metaanalysis of 31 studies. Pathophysiol Haemost Thromb 2002;32(2):51–8. [10] Heider TR, Azeem S, Galanko JA, Behrns KE. The natural history of pancreatitisinduced splenic vein thrombosis. Ann Surg 2004;239(6):876–80. [11] Weiss FU, Simon P, Witt H, Hlouschek V, Zimmer KP, Schnekenburger J, et al. SPINK1 mutations and phenotypic expression in patients with pancreatitis associated with trypsinogen mutations. J Med Genet 2003;40(4):e40. [12] Weiss FU, Simon P, Bogdanova N, Mayerle J, Dworniczak B, Horst J, et al. Complete cystic fibrosis transmembrane conductance regulator gene sequencing in patients with idiopathic chronic pancreatitis and controls. Gut 2005;54(10):1456.
Please cite this article as: Starikov A, Bartolotta RJ, Massive superior mesenteric venous aneurysm with portal venous thrombosis, Clin Imaging (2015), http://dx.doi.org/10.1016/j.clinimag.2015.05.001
