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Retroperitoneal Hematoma Caused by a Ruptured Pelvic Varix in a Patient with Iliac Vein Compression Syndrome
Retroperitoneal Hematoma Caused by a Ruptured Pelvic Varix in a Patient with Iliac Vein Compression Syndrome Sachin Dheer, MD, Allen E. Joseph, MD, and Alain Drooz, MD May-Thurner syndrome, or iliac vein compression syndrome (IVCS), is a rare but well-described entity. It refers to the formation of venous thrombus caused by compression of the left iliac vein, most commonly between the right iliac artery and lumbar vertebrae. Several variants of IVCS have been described, including unusual presenting symptoms, etiologies, and complications. The authors describe an unusual case of IVCS in which the patient presented with a left-sided retroperitoneal hematoma arising from a ruptured collateral venous varix shortly after the development of symptomatic left lower-extremity deep vein thrombosis. Index terms:
Iliac veins, compression
•
May-Thurner syndrome
•
Thrombosis, venous
J Vasc Interv Radiol 2003; 14:387–390 Abbreviation:
IVCS ⫽ iliac vein compression syndrome
ILIAC vein compression and subsequent venous thrombus and spur formation was first described by McMurrich in 1908 (1). Thereafter, May and Thurner (2) described this entity pathologically and Cockett and Thomas (3) described a series of patients in whom symptoms included left-leg edema, pain, varicosities, ulceration, and venous claudication. Several case reports have documented the presentation and pathophysiology of this entity, noting the formation of thrombus, spurs, and collateral venous blood flow that develops in response to venous stenosis (4,5). However, most of the literature has focused on the evolving management of iliac vein compression syndrome (IVCS) (6 –10). From the Department of Radiology, Inova Fairfax Hospital, Falls Church, Virginia. Received June 21, 2002; revision requested August 15; revision received October 15; accepted October 17. Address correspondence to A.E.J., 3299 Woodburn Rd., Suite 110, Annandale, VA 22003; E-mail: aejoseph@ post.harvard.edu A.E.J. is a shareholder in Johnson & Johnson. None of the other authors has identified a potential conflict of interest. © SIR, 2003 DOI: 10.1097/01.RVI.0000058411.01661.2b
Although the exact incidence of IVCS is unknown, it is likely underdiagnosed. IVCS most commonly occurs in women in the second to fourth decades of life and may or may not be associated with factors predisposing to hypercoagulability. If this condition is not recognized, complications arising from venous thrombosis can result in significant morbidity and mortality (4). We present a case of life-threatening hemorrhage from a ruptured varix occurring after acute iliac venous thrombosis in a patient with IVCS.
CASE REPORT A 58-year-old woman with no significant medical history presented to her local hospital with acute left leg pain and swelling. Physical examination revealed edema and cyanosis of the left leg. Computed tomography (CT) demonstrated a right lower-lobe pulmonary embolus, left iliac venous thrombosis, and a large left-sided retroperitoneal hematoma with extravasation of intravascular contrast material (Fig 1). The patient was transported to our hospital for further management. On arrival, the patient’s blood pressure was 74/36 mm Hg, with a pulse rate of 140 bpm. The pa-
tient’s left leg was edematous, cold, mottled, and pulseless. Aggressive fluid resuscitation and transfusion of blood products was continued during the procedures described herein. An arteriogram was first obtained to exclude an arterial source of bleeding. When none was observed, venography was performed via a right internal jugular approach. Occlusive thrombus was present throughout the left iliac vein, extending into the inferior vena cava (Fig 2). Injection into the patent left common femoral vein revealed active extravasation though a collateral venous varix (Fig 3). A brief and unsuccessful attempt was made to cannulate and embolize the bleeding varix. It was elected to decompress the varix by reducing the clot burden and treating any underlying stenosis with a stent. Because thrombolysis was contraindicated as a result of the active pelvic hemorrhage, a TrapEase inferior vena cava filter (Cordis, Miami, FL) was placed in an infrarenal position via a right internal jugular venous approach to protect against further pulmonary embolus. An 8-F, 80-cm vascular sheath was advanced below the filter through the same access. The thrombus was debulked with use of a 6-F
387
388
•
Retroperitoneal Hematoma from a Ruptured Pelvic Varix
Figure 1. CT demonstrates a large, left-sided retroperitoneal hematoma with extravasation of intravenous contrast material (arrow), suggesting active bleeding.
Xpeedior AngioJet mechanical thrombectomy device (Possis, Minneapolis, MN). Empiric stent placement in the left iliac vein was performed with two overlapping 14-mm ⫻ 60-mm Smart stents (Cordis). The unconstrained stents were compressed in the central portion of the left common iliac vein, the characteristic location of IVCS. Balloon angioplasty was performed to expand the stents. Thrombus that had become trapped in the filter was also debulked with the mechanical thrombectomy device. Control left iliac venography revealed restored flow through the left iliac veins (Fig 4). Nonocclusive thrombus remained in the left common iliac vein and below the caval filter. Immediately after the procedure, the left leg was pink and warm, with strongly palpable pulses and multiphasic arterial Doppler signals at the ankle. Two days after the procedure, CT revealed a stable hematoma without evidence of further bleeding and lower-extremity venous duplex ultrasonography (US) demonstrated no recurrent or residual clot. There was symmetrically phasic venous Doppler flow in both legs. The patient underwent anticoagulation with heparin and subsequently warfarin, for a total of 6 months of anticoagulation. Fur-
March 2003
JVIR
Figure 2. Venography via a right internal jugular approach reveals occlusive thrombus in the left iliac vein (arrow).
ther laboratory workup did not reveal any genetic or metabolic cause of the patient’s thrombosis. The patient remains symptom-free at 17 months, with no evidence of recurrent deep vein thrombosis or postphlebitic syndrome. Duplex US at 5 months and contrast-enhanced pelvic CT performed at 14 months showed patent left iliac vein stents with no recurrent or residual clot. Institutional review board approval was obtained for this case review.
DISCUSSION May-Thurner syndrome, or IVCS, has been well reported in the literature (4 –10). Most cases are caused by direct compression of the left iliac vein between the right iliac artery and fifth lumbar vertebra; however, cases have been described in which iliac vein compression can result from more unusual causes (5,11–14). Similarly, authors have reported a broad spectrum of symptoms associated with IVCS (15–18). Our patient presented with acute venous obstruction, or phlegmasia cerulea dolens, which quickly progressed to arterial compromise, or phlegmasia alba dolens, and hemodynamic instability. To our knowledge, concomitant
Figure 3. Active contrast material extravasation from a collateral venous varix (arrow) observed during left common femoral venography.
retroperitoneal hemorrhage has not been described as a complication of acute iliocaval deep vein thrombosis.
Volume 14
Number 3
Dheer et al
Figure 4. Postprocedural venography demonstrates restored flow through the left iliac vein. A TrapEase filter is present in the inferior vena cava (arrow) and two overlapping 14-mm ⫻ 60-mm Smart stents are present in the left iliac vein (arrowheads). Nonocclusive clot is present in the left common iliac vein and below the filter (curved arrows).
Longstanding venous obstruction is known to cause dilation of collateral venous channels in other vascular beds (eg, portal-to-systemic collaterals in portal hypertension and in chronic splenic vein occlusion). Dilated varices, with their thin walls and high radial tension, are prone to rupture. In this case, an underlying iliac venous stenosis likely resulted in the formation of a retroperitoneal collateral varix. Acute venous thrombosis superimposed on the stenosis likely increased venous pressures enough to overwhelm the varix, resulting in its rupture. The severity of our patient’s symptoms dictated the need for aggressive intervention. Most patients with phlegmasia cerulea dolens caused by lowerextremity deep vein thrombosis are, in our practice, treated with catheter-directed thrombolysis followed by treatment of underlying stenoses with primary stent placement. However, given the associated life-threatening hemorrhage, thrombolysis was clearly contraindicated in this patient. We were not able to embolize the bleeding
varix. Experience with primary embolization of portal varices suggests that the treatment of venous hypertension is necessary to prevent recurrent bleeding (19). We therefore sought to reduce the underlying venous hypertension with a combination of mechanical thrombectomy and empiric stent placement in the iliac venous stenosis. Extrapolating from the general experience with transjugular intrahepatic portosystemic stent creation and portal hypertension, we did not believe it necessary to repeat attempts at embolization after relieving the underlying venous hypertension. In our hands, mechanical thrombectomy tends to debulk and not completely remove iliac venous clot. We believed that the restoration of flow occurring after iliac venous stent placement could cause more pulmonary emboli if clot remained in the iliac venous system. Because we could not use thrombolytic therapy to treat this clot, we elected to place an inferior vena cava filter before manipulating the clot and placing a stent in the underlying stenosis. We were limited to
•
389
the use of a permanent inferior vena cava filter at that time. Our approach was immediately effective in restoring circulation to the left leg (we observed resolution of phlegmasia on the table), limiting the volume of clot passing into the pulmonary circulation and preventing further hemorrhage. The patient underwent successful anticoagulation within 72 hours without further episodes of bleeding. She remained asymptomatic 17 months after the event, with no signs of recurrent deep vein thrombosis or postphlebitic syndrome. References 1. McMurrich JP. The occurrence of congenital adhesions in the common iliac veins and their relation to thrombosis of the femoral and iliac veins. Am J Med Sci 1908; 135:342–346. 2. May R, Thurner J. Ein GefBsporn in der v. iliaca com. sin. Als wahrscheinliche ursache der uberwiegend linksseitigen beckenvenenthrombrose. Z Kreisl-Forsch 1956; 45:912. 3. Cockett FB, Thomas ML, Negus D. Iliac vein compression—its relation to iliofemoral thrombosis and the postthrombotic syndrome. BMJ 1967; 2(543):14 –19. 4. O’Sullivan GJ, Semba CP, Bittner CA, et al. Endovascular management of iliac vein compression (May-Thurner) syndrome. J Vasc Interv Radiol 2000; 11:823– 836. 5. Steinberg JB, Jacocks MA. MayThurner syndrome: a previously unreported variant. Ann Vasc Surg 1993; 7:577–581. 6. Alimi YS, DiMauro P, Fabre D, Juhan C. Iliac vein reconstructions to treat acute and chronic venous occlusive disease. J Vasc Surg 1997; 25:673– 681. 7. Binkert CA, Schoch E, Stuckmann G, et al. Treatment of pelvic venous spur (May-Thurner syndrome) with self-expanding metallic endoprostheses. Cardiovasc Intervent Radiol 1998; 21:22– 26. 8. Kasirajan K, Gray B, Ouriel K. Percutaneous AngioJet thrombectomy in the management of extensive deep venous thrombosis. J Vasc Interv Radiol 2001; 12:179 –185. 9. Patel NH, Stookey KR, Ketcham DB, Cragg AH. Endovascular management of acute extensive iliofemoral deep venous thrombosis caused by May-Thurner syndrome. J Vasc Intev Radiol 2000; 11:1297–1302. 10. Verhaeghe R, Stockx L, Lacroix H, Vermylen J, Baert AL. Catheter-directed
390
•
Retroperitoneal Hematoma from a Ruptured Pelvic Varix
lysis of iliofemoral vein thrombosis with use of rt-PA. Eur Radiol 1997; 7:996 –1001. 11. Arrazola L, Sutherland DE, Sozen H, et al. May-Thurner syndrome in renal transplantation. Transplantation 2001; 71:698 –702. 12. Ducharme SE, Herring D, Tripp HF. Unilateral iliac vein occlusion, caused by bladder enlargement, simulating deep venous thrombosis. J Vasc Surg 1999; 29:724 –726. 13. Palma L, Peterson MC, Ingebretsen R. Iliac vein compression syndrome from urinary bladder distention due to
prostatism. South Med J 1995; 88:959 – 960. 14. Shift JS, Rozencwaig R, Wilson MR. Pelvic mass secondary to polyethylene and titanium alloy wear debris resulting in recurrent deep vein thrombosis. J Arthroplasty 1997; 12:946 –949. 15. Hassell DR, Reifsteck JE, Harshfield DL, Ferris EJ. Unilateral left leg edema: a variation of May-Thurner syndrome. Cardiovasc Intervent Radiol 1987; 10:89 –91. 16. Way J, Lopez-Yunez A, Beristain X, Biller J. Paradoxical embolism to the basilar apex associated with May-
March 2003
JVIR
Thurner syndrome. Arch Neurol 2000; 57:1761–1764. 17. Jacob ET, Bar-Nathan N, Heim M, Morag B, Rubistein Z. The “iliac vein compression syndrome” and lower limb vascular access for hemodialysis. J Cardiovasc Surg 1980; 21:155–158. 18. Bomalaski MD, Mills JL, Argueso LR, Fujitani RM, Sago AL, Joseph AE. Iliac vein compression syndrome: an unusual cause of varicocele. J Vasc Surg 1993; 18:1064 –1068. 19. Luketic VA. Management of portal hypertension after variceal hemorrhage. Clin Liver Dis 2001; 5:677–707.
Iliac veins, compression
•
May-Thurner syndrome
•
Thrombosis, venous
J Vasc Interv Radiol 2003; 14:387–390 Abbreviation:
IVCS ⫽ iliac vein compression syndrome
ILIAC vein compression and subsequent venous thrombus and spur formation was first described by McMurrich in 1908 (1). Thereafter, May and Thurner (2) described this entity pathologically and Cockett and Thomas (3) described a series of patients in whom symptoms included left-leg edema, pain, varicosities, ulceration, and venous claudication. Several case reports have documented the presentation and pathophysiology of this entity, noting the formation of thrombus, spurs, and collateral venous blood flow that develops in response to venous stenosis (4,5). However, most of the literature has focused on the evolving management of iliac vein compression syndrome (IVCS) (6 –10). From the Department of Radiology, Inova Fairfax Hospital, Falls Church, Virginia. Received June 21, 2002; revision requested August 15; revision received October 15; accepted October 17. Address correspondence to A.E.J., 3299 Woodburn Rd., Suite 110, Annandale, VA 22003; E-mail: aejoseph@ post.harvard.edu A.E.J. is a shareholder in Johnson & Johnson. None of the other authors has identified a potential conflict of interest. © SIR, 2003 DOI: 10.1097/01.RVI.0000058411.01661.2b
Although the exact incidence of IVCS is unknown, it is likely underdiagnosed. IVCS most commonly occurs in women in the second to fourth decades of life and may or may not be associated with factors predisposing to hypercoagulability. If this condition is not recognized, complications arising from venous thrombosis can result in significant morbidity and mortality (4). We present a case of life-threatening hemorrhage from a ruptured varix occurring after acute iliac venous thrombosis in a patient with IVCS.
CASE REPORT A 58-year-old woman with no significant medical history presented to her local hospital with acute left leg pain and swelling. Physical examination revealed edema and cyanosis of the left leg. Computed tomography (CT) demonstrated a right lower-lobe pulmonary embolus, left iliac venous thrombosis, and a large left-sided retroperitoneal hematoma with extravasation of intravascular contrast material (Fig 1). The patient was transported to our hospital for further management. On arrival, the patient’s blood pressure was 74/36 mm Hg, with a pulse rate of 140 bpm. The pa-
tient’s left leg was edematous, cold, mottled, and pulseless. Aggressive fluid resuscitation and transfusion of blood products was continued during the procedures described herein. An arteriogram was first obtained to exclude an arterial source of bleeding. When none was observed, venography was performed via a right internal jugular approach. Occlusive thrombus was present throughout the left iliac vein, extending into the inferior vena cava (Fig 2). Injection into the patent left common femoral vein revealed active extravasation though a collateral venous varix (Fig 3). A brief and unsuccessful attempt was made to cannulate and embolize the bleeding varix. It was elected to decompress the varix by reducing the clot burden and treating any underlying stenosis with a stent. Because thrombolysis was contraindicated as a result of the active pelvic hemorrhage, a TrapEase inferior vena cava filter (Cordis, Miami, FL) was placed in an infrarenal position via a right internal jugular venous approach to protect against further pulmonary embolus. An 8-F, 80-cm vascular sheath was advanced below the filter through the same access. The thrombus was debulked with use of a 6-F
387
388
•
Retroperitoneal Hematoma from a Ruptured Pelvic Varix
Figure 1. CT demonstrates a large, left-sided retroperitoneal hematoma with extravasation of intravenous contrast material (arrow), suggesting active bleeding.
Xpeedior AngioJet mechanical thrombectomy device (Possis, Minneapolis, MN). Empiric stent placement in the left iliac vein was performed with two overlapping 14-mm ⫻ 60-mm Smart stents (Cordis). The unconstrained stents were compressed in the central portion of the left common iliac vein, the characteristic location of IVCS. Balloon angioplasty was performed to expand the stents. Thrombus that had become trapped in the filter was also debulked with the mechanical thrombectomy device. Control left iliac venography revealed restored flow through the left iliac veins (Fig 4). Nonocclusive thrombus remained in the left common iliac vein and below the caval filter. Immediately after the procedure, the left leg was pink and warm, with strongly palpable pulses and multiphasic arterial Doppler signals at the ankle. Two days after the procedure, CT revealed a stable hematoma without evidence of further bleeding and lower-extremity venous duplex ultrasonography (US) demonstrated no recurrent or residual clot. There was symmetrically phasic venous Doppler flow in both legs. The patient underwent anticoagulation with heparin and subsequently warfarin, for a total of 6 months of anticoagulation. Fur-
March 2003
JVIR
Figure 2. Venography via a right internal jugular approach reveals occlusive thrombus in the left iliac vein (arrow).
ther laboratory workup did not reveal any genetic or metabolic cause of the patient’s thrombosis. The patient remains symptom-free at 17 months, with no evidence of recurrent deep vein thrombosis or postphlebitic syndrome. Duplex US at 5 months and contrast-enhanced pelvic CT performed at 14 months showed patent left iliac vein stents with no recurrent or residual clot. Institutional review board approval was obtained for this case review.
DISCUSSION May-Thurner syndrome, or IVCS, has been well reported in the literature (4 –10). Most cases are caused by direct compression of the left iliac vein between the right iliac artery and fifth lumbar vertebra; however, cases have been described in which iliac vein compression can result from more unusual causes (5,11–14). Similarly, authors have reported a broad spectrum of symptoms associated with IVCS (15–18). Our patient presented with acute venous obstruction, or phlegmasia cerulea dolens, which quickly progressed to arterial compromise, or phlegmasia alba dolens, and hemodynamic instability. To our knowledge, concomitant
Figure 3. Active contrast material extravasation from a collateral venous varix (arrow) observed during left common femoral venography.
retroperitoneal hemorrhage has not been described as a complication of acute iliocaval deep vein thrombosis.
Volume 14
Number 3
Dheer et al
Figure 4. Postprocedural venography demonstrates restored flow through the left iliac vein. A TrapEase filter is present in the inferior vena cava (arrow) and two overlapping 14-mm ⫻ 60-mm Smart stents are present in the left iliac vein (arrowheads). Nonocclusive clot is present in the left common iliac vein and below the filter (curved arrows).
Longstanding venous obstruction is known to cause dilation of collateral venous channels in other vascular beds (eg, portal-to-systemic collaterals in portal hypertension and in chronic splenic vein occlusion). Dilated varices, with their thin walls and high radial tension, are prone to rupture. In this case, an underlying iliac venous stenosis likely resulted in the formation of a retroperitoneal collateral varix. Acute venous thrombosis superimposed on the stenosis likely increased venous pressures enough to overwhelm the varix, resulting in its rupture. The severity of our patient’s symptoms dictated the need for aggressive intervention. Most patients with phlegmasia cerulea dolens caused by lowerextremity deep vein thrombosis are, in our practice, treated with catheter-directed thrombolysis followed by treatment of underlying stenoses with primary stent placement. However, given the associated life-threatening hemorrhage, thrombolysis was clearly contraindicated in this patient. We were not able to embolize the bleeding
varix. Experience with primary embolization of portal varices suggests that the treatment of venous hypertension is necessary to prevent recurrent bleeding (19). We therefore sought to reduce the underlying venous hypertension with a combination of mechanical thrombectomy and empiric stent placement in the iliac venous stenosis. Extrapolating from the general experience with transjugular intrahepatic portosystemic stent creation and portal hypertension, we did not believe it necessary to repeat attempts at embolization after relieving the underlying venous hypertension. In our hands, mechanical thrombectomy tends to debulk and not completely remove iliac venous clot. We believed that the restoration of flow occurring after iliac venous stent placement could cause more pulmonary emboli if clot remained in the iliac venous system. Because we could not use thrombolytic therapy to treat this clot, we elected to place an inferior vena cava filter before manipulating the clot and placing a stent in the underlying stenosis. We were limited to
•
389
the use of a permanent inferior vena cava filter at that time. Our approach was immediately effective in restoring circulation to the left leg (we observed resolution of phlegmasia on the table), limiting the volume of clot passing into the pulmonary circulation and preventing further hemorrhage. The patient underwent successful anticoagulation within 72 hours without further episodes of bleeding. She remained asymptomatic 17 months after the event, with no signs of recurrent deep vein thrombosis or postphlebitic syndrome. References 1. McMurrich JP. The occurrence of congenital adhesions in the common iliac veins and their relation to thrombosis of the femoral and iliac veins. Am J Med Sci 1908; 135:342–346. 2. May R, Thurner J. Ein GefBsporn in der v. iliaca com. sin. Als wahrscheinliche ursache der uberwiegend linksseitigen beckenvenenthrombrose. Z Kreisl-Forsch 1956; 45:912. 3. Cockett FB, Thomas ML, Negus D. Iliac vein compression—its relation to iliofemoral thrombosis and the postthrombotic syndrome. BMJ 1967; 2(543):14 –19. 4. O’Sullivan GJ, Semba CP, Bittner CA, et al. Endovascular management of iliac vein compression (May-Thurner) syndrome. J Vasc Interv Radiol 2000; 11:823– 836. 5. Steinberg JB, Jacocks MA. MayThurner syndrome: a previously unreported variant. Ann Vasc Surg 1993; 7:577–581. 6. Alimi YS, DiMauro P, Fabre D, Juhan C. Iliac vein reconstructions to treat acute and chronic venous occlusive disease. J Vasc Surg 1997; 25:673– 681. 7. Binkert CA, Schoch E, Stuckmann G, et al. Treatment of pelvic venous spur (May-Thurner syndrome) with self-expanding metallic endoprostheses. Cardiovasc Intervent Radiol 1998; 21:22– 26. 8. Kasirajan K, Gray B, Ouriel K. Percutaneous AngioJet thrombectomy in the management of extensive deep venous thrombosis. J Vasc Interv Radiol 2001; 12:179 –185. 9. Patel NH, Stookey KR, Ketcham DB, Cragg AH. Endovascular management of acute extensive iliofemoral deep venous thrombosis caused by May-Thurner syndrome. J Vasc Intev Radiol 2000; 11:1297–1302. 10. Verhaeghe R, Stockx L, Lacroix H, Vermylen J, Baert AL. Catheter-directed
390
•
Retroperitoneal Hematoma from a Ruptured Pelvic Varix
lysis of iliofemoral vein thrombosis with use of rt-PA. Eur Radiol 1997; 7:996 –1001. 11. Arrazola L, Sutherland DE, Sozen H, et al. May-Thurner syndrome in renal transplantation. Transplantation 2001; 71:698 –702. 12. Ducharme SE, Herring D, Tripp HF. Unilateral iliac vein occlusion, caused by bladder enlargement, simulating deep venous thrombosis. J Vasc Surg 1999; 29:724 –726. 13. Palma L, Peterson MC, Ingebretsen R. Iliac vein compression syndrome from urinary bladder distention due to
prostatism. South Med J 1995; 88:959 – 960. 14. Shift JS, Rozencwaig R, Wilson MR. Pelvic mass secondary to polyethylene and titanium alloy wear debris resulting in recurrent deep vein thrombosis. J Arthroplasty 1997; 12:946 –949. 15. Hassell DR, Reifsteck JE, Harshfield DL, Ferris EJ. Unilateral left leg edema: a variation of May-Thurner syndrome. Cardiovasc Intervent Radiol 1987; 10:89 –91. 16. Way J, Lopez-Yunez A, Beristain X, Biller J. Paradoxical embolism to the basilar apex associated with May-
March 2003
JVIR
Thurner syndrome. Arch Neurol 2000; 57:1761–1764. 17. Jacob ET, Bar-Nathan N, Heim M, Morag B, Rubistein Z. The “iliac vein compression syndrome” and lower limb vascular access for hemodialysis. J Cardiovasc Surg 1980; 21:155–158. 18. Bomalaski MD, Mills JL, Argueso LR, Fujitani RM, Sago AL, Joseph AE. Iliac vein compression syndrome: an unusual cause of varicocele. J Vasc Surg 1993; 18:1064 –1068. 19. Luketic VA. Management of portal hypertension after variceal hemorrhage. Clin Liver Dis 2001; 5:677–707.