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Congenital Anomalies of Inferior Vena Cava in Young Patients with Iliac Deep Venous Thrombosis
Congenital Anomalies of Inferior Vena Cava in Young Patients with Iliac Deep Venous Thrombosis Gabrielle Sarlon,1 Michel Alain Bartoli,1 Cyril Muller,2 Souad Acid,2 Jean-Michel Bartoli,2 Serge Cohen,1 Philippe Piquet,1 and Pierre-Edouard Magnan,1 Marseille, France
Venous thromboembolism (VTE) in young patients is frequently associated with hereditary biological thrombophilia, autoimmune disorders, or neoplasia. Advances in venous ultrasound and contrast-enhanced computed tomography have allowed for the identification of inferior vena cava (IVC) anomalies as newly considered etiologic factor. We present two cases of VTE in young patients: the first case involves left IVC in a 22-year-old man and the second involves IVC atresia in a 39-year-old man. IVC anomalies should be identified in young patients with spontaneous VTE involving the iliac veins because they are at a high risk for thrombotic recurrence and adaptation to long periods of antithrombotic therapy.
Venous thromboembolism (VTE) is known to be frequently associated with hereditary biological thrombophilia, autoimmune disorders, or neoplasia. In this study, we present two cases of inferior vena cava (IVC) anomalies in young patients with spontaneous VTE that represents an ‘‘anatomic’’ thrombophilia because of the high risk involved for thrombotic recurrence.
CASE REPORTS Case 1 A 22-year-old man was presented to our facility in July 2009 with a complaint of sudden pain in the right thigh after being involved in an intensive sporting activity. A venous ultrasound was performed immediately, which detected a recent thrombus in the right iliac venous axes and a dilated and totally occluded left IVC. He had a primary history of deep venous thrombosis of the right 1 Vascular Medicine and Surgery, Timone Hospital, Marseille, France. 2 Radiology, Timone Hospital, Marseille, France.
Correspondence to: Gabrielle Sarlon, MD, Vascular Medicine and Surgery, Timone Hospital, 264 Rue Saint Pierre, 13385 Marseille Cedex 5, France, E-mail: [email protected] Ann Vasc Surg 2011; 25: 265.e5-265.e8 DOI: 10.1016/j.avsg.2010.07.007 Ó Annals of Vascular Surgery Inc. Published online: October 4, 2010
iliac vein secondary to a venous catheterism for a junctional tachycardia ablation. This first VTE episode was treated for a period of 3 months by the administration of fluindione. A venous computed tomography (CT) confirmed the presence of a second episode of VTE: a fresh thrombus occluded the right iliac veins (external, internal, and common) and the left IVC until drainage occurred in the left renal vein (Fig. 1A). The venous confluence at the level of the renal veins was free of thrombus and it crossed over the midline region in a retroaortic position joining the azygos system up to the superior vena cava (Fig. 1B). The retrohepatic segment of the IVC was found to be absent and the hepatic veins drained directly into the right atrium (Fig. 1C). Biological risk factors for thrombophilia including heterozygote factor V Leiden Arg 506-Gln mutation and hyperhomocysteinemia (22.7 mmoles/L) with heterozygote MTHFR gene mutation were found to be present without any other anomalies. Results from the total body CT scan eliminated the presence of either evolutive neoplasia or lymphoma. After we conducted a multidisciplinary discussion regarding different treatment options, an in-situ thrombolysis with urokinase was performed with partial repermeabilization of the vena cava, but right iliac vein occlusion still continued to persist (Fig. 1D). Anticoagulation therapy including nonfractioned heparin followed by administration of fluindione was initiated. One month after the IVC was completely free of thrombus, the right common iliac vein was found to be still occluded and the external iliac vein was partially recanalized. Recurrence of VTE and persistence of IVC anomalies in this young patient necessitated prolonged anticoagulation therapy.
265.e5
265.e6 Case reports
Annals of Vascular Surgery
Fig. 1. Case 1: Venous computed tomographic scans of the coronal plane (A, B, C) and cavography (D) showing the presence of a fresh clot in the right iliac veins and in the left inferior vena cava (IVC) (arrow in panel A), azygos continuation (arrow in panel B) with absence of
IVC prerenal segment, hepatic veins directly draining into the right atrium (arrow in panel C), and partial repermeabilization of left IVC during in-situ thrombolysis (arrow in panel D).
Case 2
CT and biomarkers, neither hereditary or immunologic disorders nor neoplasia were diagnosed. Before curative anticoagulation therapy was started, a cerebral CT was performed to eliminate immediate intracerebral hemorrhagic risk because of neurosurgical intervention. Standard anticoagulation therapy with low molecular weight heparin was initiated without thrombolysis because of the possibly aging thrombus, and was followed by bilateral venous bandaging and oral administration of fluindione. We agreed on a minimum of 1 year of anticoagulation therapy because of possible neurological hemorrhagic risk, IVC anomalies, and mobility reduction.
A 39-year-old man was presented to our facility in September 2009 with complaint of persistent low back pain and recent bilateral leg edema and pain. A venous ultrasound led to the diagnosis of a bilateral deep venous thrombosis of the femoral and iliac veins without any observed complication in the IVC. His medical history was relevant as it included the following: a neurosurgical ablation carried out 4 years back for a cerebral arteriovenous malformation with monoparesia sequela of the left leg, a recent depressive syndrome with significant reduction of physical mobility, and an active smoking status. A CT scan led to an anatomic diagnosis of a recent bilateral iliac venous thrombosis (Figs. 2A, B) at the confluence of the two common iliac veins. After this confluence the IVC was reported to be absent, namely because of either IVC atresia or agenesia (postrenal, renal, and retrohepatic segments) (Fig. 2C) with several collaterals being issued from the thoracic, lumbar, pelvic, and abdominal veins. These collaterals joined the azygos and the hemiazygos systems which then drained into the superior vena cava (Fig. 2D). Using
DISCUSSION Overall incidence rate for VTE is about one per 1,000 persons per year, with an exponential increase after 50 years reaching a rate of 1/100 per year after 75 years. VTE is known to occur rarely in the younger population ranging in ages from 20 to 40 years, with an incidence rate that is 10 times lower
Vol. 25, No. 2, February 2011
Case reports 265.e7
Fig. 2. Case 2: Venous computed tomographic scan of the coronal (A) and axial planes (B, C, D) showing thrombosis of the two iliac venous axes up to the confluence (arrows in panels A and B), IVC agenesia in the three segments (postrenal, renal, and retrohepatic)
(arrow in panel C) and collaterals issuing from the thoracic, lumbar, pelvic, and abdominal veins (panel C), and the azygos and hemiazygos systems (arrows in panel D) joined with the collaterals leading to venous drainage into the superior vena cava.
than the older population, and it is frequently associated with predisposing conditions, such as hereditary biological thrombophilia, autoimmune disorders, neoplasia, or hematological diseases. IVC anomalies are reported infrequently in published data and only about <1% of the overall studies related to IVC anomalies are on VTE; none of the previously published data on IVC anomalies have reported any gender differences.1 There are essentially four anomalies because of embryologic disorders in the period between the fifth and the seventh week of gestation: duplicated or double IVC at the postrenal level of IVC secondary to persistence of the left supracardinal vein in the embryo, left IVC or transposition of IVC secondary to regression of the right supracardinal vein and persistence of the left supracardinal vein (case 1),2 atresia or agenesia of a segment or of the total IVC which is habitually divided into three segments (retrohepatic, renal, and postrenal segments) (case 2),3 and azygos and/ or hemiazygos continuation frequently associated with the aforementioned conditions and allowed drainage into the caudal segment up to the right atrium because of a connection with the superior
vena cava. The pathophysiology of VTE associated with IVC anomalies includes inadequate blood return; increased blood pressure in the lower extremities veins; venous stasis; and subsequent deep vein thrombosis (bilateral in more than 50% of the cases).4 As reported in this study, performance and accessibility of venous ultrasound and contrast enhanced venous CT scanning, in most cases, have helped in diagnosing venous anomalies. Generally, vascular ultrasound is the initial diagnostic method used in patients with suspected congenital vascular disease; however, it is otherwise of limited use, particularly in diagnosing thoracic abnormalities with narrowed acoustic windows. Technical improvements including multidetectors, multiplanar reformation, threedimensional volume rendering properties, and venous acquisition time have helped in improving the role of CT in the evaluation of vena cava anomalies.1-4 Previously published studies have favored complications, such as minor biological thrombophilia in case 1 or immobilization in case 2 to provide an explanation for the VTE episode associated with IVC anomalies. The possibility of recurrent thrombosis is high when anticoagulation treatment is withdrawn
265.e8 Case reports
even after the removal of precipitating triggers, similar to that observed in case 1. The most appropriate therapeutic approach is to continue anticoagulation therapy for more than 6 months until the principal factor provoking thrombosis continues.5 Patients who are known to have IVC anomalies should be advised to avoid additional thrombogenic risk factors, such as unusual physical activity or oral contraceptive use, and additionally graduate compression stockings and preventive anticoagulation therapy should be applied during prolonged immobilization.6
CONCLUSIONS An initial episode of proximal VTE, especially iliac, bilateral, and spontaneous, in the younger population promotes a screening for congenital IVC anomalies; this is important as it helps in deciding on a therapeutic approach including follow-up, duration of anticoagulation therapy, and advice related to lifestyle choices.
Annals of Vascular Surgery
The authors of this manuscript have certified that they have no conflict of interest.
REFERENCES 1. Onbas x O, Kantarci M, Koplay M, et al. Congenital anomalies of the aorta and vena cava: 16-detector-row CT imaging findings. Diagn Interv Radiol 2008;13:163-171. 2. Milani C, Constantinou M, Berz D, Butera J, Colvin GA. Left sided inferior vena cava duplication and venous thromboembolism: case report and review of literature. J Hematol Oncol 2008;1:24. 3. D’Aloia A, Faggiano P, Fiorina C, et al. Absence of inferior vena cava as a rare cause of deep venous complicated by liver and lung embolism. Int J Cardiol 2003;88:327-329. 4. Gayer G, Luboshitz J, Hertz M, et al. Congenital anomalies of the inferior vena cava revealed on CT in patients with deep vein thrombosis. Am J Radiol 2003;180:729-732. 5. Obernosterer A, Aschauer M, Schnedl W, Lipp RW. Anomalies of the inferior vena cava in patients with iliac venous thrombosis. Ann Intern Med 2002;136:37-41. 6. Chee YL, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol 2001;114:878-880.
Venous thromboembolism (VTE) in young patients is frequently associated with hereditary biological thrombophilia, autoimmune disorders, or neoplasia. Advances in venous ultrasound and contrast-enhanced computed tomography have allowed for the identification of inferior vena cava (IVC) anomalies as newly considered etiologic factor. We present two cases of VTE in young patients: the first case involves left IVC in a 22-year-old man and the second involves IVC atresia in a 39-year-old man. IVC anomalies should be identified in young patients with spontaneous VTE involving the iliac veins because they are at a high risk for thrombotic recurrence and adaptation to long periods of antithrombotic therapy.
Venous thromboembolism (VTE) is known to be frequently associated with hereditary biological thrombophilia, autoimmune disorders, or neoplasia. In this study, we present two cases of inferior vena cava (IVC) anomalies in young patients with spontaneous VTE that represents an ‘‘anatomic’’ thrombophilia because of the high risk involved for thrombotic recurrence.
CASE REPORTS Case 1 A 22-year-old man was presented to our facility in July 2009 with a complaint of sudden pain in the right thigh after being involved in an intensive sporting activity. A venous ultrasound was performed immediately, which detected a recent thrombus in the right iliac venous axes and a dilated and totally occluded left IVC. He had a primary history of deep venous thrombosis of the right 1 Vascular Medicine and Surgery, Timone Hospital, Marseille, France. 2 Radiology, Timone Hospital, Marseille, France.
Correspondence to: Gabrielle Sarlon, MD, Vascular Medicine and Surgery, Timone Hospital, 264 Rue Saint Pierre, 13385 Marseille Cedex 5, France, E-mail: [email protected] Ann Vasc Surg 2011; 25: 265.e5-265.e8 DOI: 10.1016/j.avsg.2010.07.007 Ó Annals of Vascular Surgery Inc. Published online: October 4, 2010
iliac vein secondary to a venous catheterism for a junctional tachycardia ablation. This first VTE episode was treated for a period of 3 months by the administration of fluindione. A venous computed tomography (CT) confirmed the presence of a second episode of VTE: a fresh thrombus occluded the right iliac veins (external, internal, and common) and the left IVC until drainage occurred in the left renal vein (Fig. 1A). The venous confluence at the level of the renal veins was free of thrombus and it crossed over the midline region in a retroaortic position joining the azygos system up to the superior vena cava (Fig. 1B). The retrohepatic segment of the IVC was found to be absent and the hepatic veins drained directly into the right atrium (Fig. 1C). Biological risk factors for thrombophilia including heterozygote factor V Leiden Arg 506-Gln mutation and hyperhomocysteinemia (22.7 mmoles/L) with heterozygote MTHFR gene mutation were found to be present without any other anomalies. Results from the total body CT scan eliminated the presence of either evolutive neoplasia or lymphoma. After we conducted a multidisciplinary discussion regarding different treatment options, an in-situ thrombolysis with urokinase was performed with partial repermeabilization of the vena cava, but right iliac vein occlusion still continued to persist (Fig. 1D). Anticoagulation therapy including nonfractioned heparin followed by administration of fluindione was initiated. One month after the IVC was completely free of thrombus, the right common iliac vein was found to be still occluded and the external iliac vein was partially recanalized. Recurrence of VTE and persistence of IVC anomalies in this young patient necessitated prolonged anticoagulation therapy.
265.e5
265.e6 Case reports
Annals of Vascular Surgery
Fig. 1. Case 1: Venous computed tomographic scans of the coronal plane (A, B, C) and cavography (D) showing the presence of a fresh clot in the right iliac veins and in the left inferior vena cava (IVC) (arrow in panel A), azygos continuation (arrow in panel B) with absence of
IVC prerenal segment, hepatic veins directly draining into the right atrium (arrow in panel C), and partial repermeabilization of left IVC during in-situ thrombolysis (arrow in panel D).
Case 2
CT and biomarkers, neither hereditary or immunologic disorders nor neoplasia were diagnosed. Before curative anticoagulation therapy was started, a cerebral CT was performed to eliminate immediate intracerebral hemorrhagic risk because of neurosurgical intervention. Standard anticoagulation therapy with low molecular weight heparin was initiated without thrombolysis because of the possibly aging thrombus, and was followed by bilateral venous bandaging and oral administration of fluindione. We agreed on a minimum of 1 year of anticoagulation therapy because of possible neurological hemorrhagic risk, IVC anomalies, and mobility reduction.
A 39-year-old man was presented to our facility in September 2009 with complaint of persistent low back pain and recent bilateral leg edema and pain. A venous ultrasound led to the diagnosis of a bilateral deep venous thrombosis of the femoral and iliac veins without any observed complication in the IVC. His medical history was relevant as it included the following: a neurosurgical ablation carried out 4 years back for a cerebral arteriovenous malformation with monoparesia sequela of the left leg, a recent depressive syndrome with significant reduction of physical mobility, and an active smoking status. A CT scan led to an anatomic diagnosis of a recent bilateral iliac venous thrombosis (Figs. 2A, B) at the confluence of the two common iliac veins. After this confluence the IVC was reported to be absent, namely because of either IVC atresia or agenesia (postrenal, renal, and retrohepatic segments) (Fig. 2C) with several collaterals being issued from the thoracic, lumbar, pelvic, and abdominal veins. These collaterals joined the azygos and the hemiazygos systems which then drained into the superior vena cava (Fig. 2D). Using
DISCUSSION Overall incidence rate for VTE is about one per 1,000 persons per year, with an exponential increase after 50 years reaching a rate of 1/100 per year after 75 years. VTE is known to occur rarely in the younger population ranging in ages from 20 to 40 years, with an incidence rate that is 10 times lower
Vol. 25, No. 2, February 2011
Case reports 265.e7
Fig. 2. Case 2: Venous computed tomographic scan of the coronal (A) and axial planes (B, C, D) showing thrombosis of the two iliac venous axes up to the confluence (arrows in panels A and B), IVC agenesia in the three segments (postrenal, renal, and retrohepatic)
(arrow in panel C) and collaterals issuing from the thoracic, lumbar, pelvic, and abdominal veins (panel C), and the azygos and hemiazygos systems (arrows in panel D) joined with the collaterals leading to venous drainage into the superior vena cava.
than the older population, and it is frequently associated with predisposing conditions, such as hereditary biological thrombophilia, autoimmune disorders, neoplasia, or hematological diseases. IVC anomalies are reported infrequently in published data and only about <1% of the overall studies related to IVC anomalies are on VTE; none of the previously published data on IVC anomalies have reported any gender differences.1 There are essentially four anomalies because of embryologic disorders in the period between the fifth and the seventh week of gestation: duplicated or double IVC at the postrenal level of IVC secondary to persistence of the left supracardinal vein in the embryo, left IVC or transposition of IVC secondary to regression of the right supracardinal vein and persistence of the left supracardinal vein (case 1),2 atresia or agenesia of a segment or of the total IVC which is habitually divided into three segments (retrohepatic, renal, and postrenal segments) (case 2),3 and azygos and/ or hemiazygos continuation frequently associated with the aforementioned conditions and allowed drainage into the caudal segment up to the right atrium because of a connection with the superior
vena cava. The pathophysiology of VTE associated with IVC anomalies includes inadequate blood return; increased blood pressure in the lower extremities veins; venous stasis; and subsequent deep vein thrombosis (bilateral in more than 50% of the cases).4 As reported in this study, performance and accessibility of venous ultrasound and contrast enhanced venous CT scanning, in most cases, have helped in diagnosing venous anomalies. Generally, vascular ultrasound is the initial diagnostic method used in patients with suspected congenital vascular disease; however, it is otherwise of limited use, particularly in diagnosing thoracic abnormalities with narrowed acoustic windows. Technical improvements including multidetectors, multiplanar reformation, threedimensional volume rendering properties, and venous acquisition time have helped in improving the role of CT in the evaluation of vena cava anomalies.1-4 Previously published studies have favored complications, such as minor biological thrombophilia in case 1 or immobilization in case 2 to provide an explanation for the VTE episode associated with IVC anomalies. The possibility of recurrent thrombosis is high when anticoagulation treatment is withdrawn
265.e8 Case reports
even after the removal of precipitating triggers, similar to that observed in case 1. The most appropriate therapeutic approach is to continue anticoagulation therapy for more than 6 months until the principal factor provoking thrombosis continues.5 Patients who are known to have IVC anomalies should be advised to avoid additional thrombogenic risk factors, such as unusual physical activity or oral contraceptive use, and additionally graduate compression stockings and preventive anticoagulation therapy should be applied during prolonged immobilization.6
CONCLUSIONS An initial episode of proximal VTE, especially iliac, bilateral, and spontaneous, in the younger population promotes a screening for congenital IVC anomalies; this is important as it helps in deciding on a therapeutic approach including follow-up, duration of anticoagulation therapy, and advice related to lifestyle choices.
Annals of Vascular Surgery
The authors of this manuscript have certified that they have no conflict of interest.
REFERENCES 1. Onbas x O, Kantarci M, Koplay M, et al. Congenital anomalies of the aorta and vena cava: 16-detector-row CT imaging findings. Diagn Interv Radiol 2008;13:163-171. 2. Milani C, Constantinou M, Berz D, Butera J, Colvin GA. Left sided inferior vena cava duplication and venous thromboembolism: case report and review of literature. J Hematol Oncol 2008;1:24. 3. D’Aloia A, Faggiano P, Fiorina C, et al. Absence of inferior vena cava as a rare cause of deep venous complicated by liver and lung embolism. Int J Cardiol 2003;88:327-329. 4. Gayer G, Luboshitz J, Hertz M, et al. Congenital anomalies of the inferior vena cava revealed on CT in patients with deep vein thrombosis. Am J Radiol 2003;180:729-732. 5. Obernosterer A, Aschauer M, Schnedl W, Lipp RW. Anomalies of the inferior vena cava in patients with iliac venous thrombosis. Ann Intern Med 2002;136:37-41. 6. Chee YL, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol 2001;114:878-880.