- Email: [email protected]
An Endovascular Option Is the Final Treatment for a Giant Arteriovenous Malformation
An Endovascular Option Is the Final Treatment for a Giant Arteriovenous Malformation Filippo Benedetto, Narayana Pipito, David Barilla, Domenico Spinelli, Francesco Stilo, and Francesco Spinelli, Messina, Italy
We report the case of a 58-year-old man presented with bleeding ulcer of the left arm caused by a high-flow type-C arteriovenous malformation (AVM), feed by branches from both the subclavian arteries. He had been previously treated with AVM sclerotherapy, embolization, humeral artery endografting, and open surgery. We urgently performed coil embolization of the left vertebral artery, and we covered the huge subclavian artery by a thoracic endograft. Then, we embolized the right tyrocervical trunk. The result was an immediate interruption of bleeding. At 12 months, the patient had no neurologic complications, and the upper limb continued to decompress.
Arteriovenous malformations (AVMs) are an extremely heterogeneous group of vascular disorders characterized by different clinical presentations, systemic hemodynamic effects, and possible treatments. AVMs are divided into low-flow and high-flow (subgroups A, B, and C) AVMs.1e4 Complete eradication of the nidus is the only potential therapy, but this, however, is not always possible. Various authors have published that only 20% of malformations may be amenable to complete extirpation with surgery.5e7 Partial resections can cause a good initial clinical response that may last for some time. However, very often the patient’s presenting symptoms recur or worsen at follow-up.5,8,9 Endovascular treatment may be a therapeutic option especially where surgery would be
Department of Vascular Surgery, Policlinico Universitario ‘‘G. Martino’’ of Messina, Messina, Italy. Correspondence to: David Barilla, MD, Department of Vascular Surgery, Policlinico Universitario ‘‘G. Martino’’ of Messina, Via Consolare Valeria, Messina 98100, Italy; E-mail: [email protected] Ann Vasc Surg 2014; 28: 1932.e5e1932.e8 http://dx.doi.org/10.1016/j.avsg.2014.06.069 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: March 16, 2014; manuscript accepted: June 16, 2014; published online: July 11, 2014.
impossible because of extensive involvement beyond the deep fascia to muscle, tendon, bone, and joints. Preoperative endovascular techniques improve the overall safety and efficacy of surgery, reducing morbidity and complications.10 We report the case of a 58-year-old man presented with bleeding ulcers of the left arm caused by a giant, type-C, high-flow AVM, feed by branches originating from both the subclavian arteries involving the left shoulder and the upper limb. The patient required staged endovascular treatments to arrest recurrent massive bleeding as lifesaving procedure.
CASE REPORT A 58-year-old man was treated for a previous small high-flow AVM on the left shoulder and upper limb, in other institutions. Over 15 years, the patient underwent multiple surgical and endovascular procedures which let a small high-flow AVM to evolve into a giant, type-C, high-flow AVM, resulting in limb function impairment and high-output cardiac state, with subsequent congestive heart failure and atrial fibrillation. Patient’s clinical history was reconstructed from previous clinical documentation. He underwent, over the years, ultrasound- and fluoroscopic-guided adjuvant sclerotherapy with polyvinyl alcohol, embolization with fibered coils and glues, and covered stent’s placement. 1932.e5
1932.e6 Case reports
Annals of Vascular Surgery
Fig. 1. Preoperative clinical presentation and angio-CT scan.
The surgical time was limited to ligation of feeding arteries; total excision of the mass was never possible because of massive bleeding, so the nidus was always left intact. Recently because of the massive proliferation and bleeding of his AVM, patient was referred to our center’s emergency room. At presentation, the left upper limb was warm and hypertrophic with several abnormal vessels clearly evident on the anterior chest wall up to the wrist, and there were bleeding ulcers on the posterolateral surface of the arm. Computed tomographic (CT) angiography demonstrated an irregular vascular network with huge arteriovenous shunts; the left subclavian and vertebral arteries and their branches were dilated and from these originated aberrant branches afferent to several vascular nidi (Fig. 1). We urgently submitted the patient to endovascular treatment. The first step was to catheterize the left vertebral artery, pushing the catheter flush to the origin of the posterior inferior cerebellar artery (PICA). Then, with the vertebral catheter still in place, a thoracic stent graft (Conformable GORE TAG Thoracic Endoprosthesis 21-21-10) was deployed into the aneurysmal subclavian artery from its origin up to previous stent grafts with 3 cm overlapping, covering all its branches. Afterward, with the origin of the subclavian artery already blocked by the thoracic endograft, we performed retrograde coil embolization (Fibered Platinum Coil; Boston Scientific) of the vertebral artery between the PICA and the origin, thus avoiding a possible distal migration of the coils (Fig. 2). At the end of the procedure, partial collapse of venous nidi and stop of bleeding were achieved and no side branches were evident (Fig. 2).
Seventy-two hours later, the patient underwent postoperative CT angiography that showed a persistent feeding vessel to the AVM from the right subclavian via an aberrant tyrocervical trunk and residual feeding from the brachial bifurcation. Right tyrocervical trunk embolization was performed, while the brachial bifurcation feeders were left intact to prevent the occlusion of the extensively covered proximal limb arteries. During a 12-month follow-up, we have observed a gradual thrombosis of the AVM aberrant vessels and a gradual decrease in volume of the upper limb with the resolution of bleeding episodes. As we achieved cessation the active bleeding we were eventually satisfied with the result and we estimated that the venous collectors from the nidus were too large to attempt any safe sclerotherapy (Fig. 3).
DISCUSSION The origin of AVMs has been controversial, and it has generated several theories and classification systems.1e4 Treatment remains a challenge. They have a wide range of clinical presentation and an unpredictable course.5,8,9 High morbidity has been related to both surgical and nonsurgical treatments10e12, with an associated high recurrence rate. They form multiple macroscopic and microscopic arteriovenous shunts, from which origins massive congestion of tissues.13 The primary effects are compression and erosion of surrounding tissues. Secondary hemodynamic effects include potential arterial steal
Vol. 28, No. 8, November 2014
Case reports 1932.e7
Fig. 2. (A) Selective angiography of the left vertebral artery documenting aberrant branches feeding the nidus. (B) Left vertebral artery embolization with coils. (C) Postoperative angiography.
Fig. 3. (A) Angio-CT scan. (B) Postoperative clinical presentation.
phenomena. Heart can be affected by high-output cardiac failure. Peripheral tissues can be affected in a wide range of changes from distal ischemia to gangrene, venous stasis dermatitis, and ulcer or gangrene caused by venous hypertension.11e14 The treatment depends on the type, extent, and location of AVMs. Lee suggests that treatment has to be set up separately for the ‘‘primary
malformation’’ itself first from their ‘‘secondary disorders’’ along the vascular system and/or skeleton and soft tissues, considering absolute indications of treatment of life-treating lesions and relative indications of various conditions to affect the quality of life significantly beside the lesions with potentially high risk of complications, vascular bone syndrome, and cosmetically severe deformity.15
1932.e8 Case reports
The case reported had an absolute indication of treatment, for the presence of recurrent massive bleeding, high-output heart failure, and patchy limb ulcerations. Nowadays, the treatment is based on multidisciplinary approaches, including conservative therapy, nonsurgical interventional treatment, and traditional surgery.15,16 Conservative therapy involves compressive garments and acetyl salicylic acid.17 Nonsurgical interventional treatment includes scleroembolization, whereas surgical treatment is only performed when the complete exeresis of the lesion is achievable. Surgical treatment aims to the complete excision of the nidus of the AVM. Its feasibility relies on the anatomy of the lesion. However, this treatment may expose the patient to a risk of massive intraoperative hemorrhages, as it had occurred in our case, and of possible neurologic deficiency.5,8 Although eradication of the nidus is necessary to obtain a definitive healing of the lesion,10 many AVMs require to fold back on a more conservative approach for the unfeasible complete excision of the lesion for its anatomic features. A surgical procedure that does not achieve a complete excision may result in worsening of the pathology because of early recurrence and collateralization of feeding arteries.18 In our case, the attempt at surgical therapy has been compromised by massive hemorrhage. The incomplete surgical treatment has been followed by progressive growth of the mass that expanded to the root of the limb and to the lateral surface of thorax, thus precluding an amputation/disarticulation of the left upper limb. As reported by many authors, the gold standard for unresectable lesions, which had low rate of improvement from embolization, is still the endovascular treatment.19 Our case is an example of palliative treatment after failure of multidisciplinary attempts of lesion eradication. The previous treatment was based both on surgery and on nonsurgical treatment. Literature reports that the use of covered stents to occlude aberrant branches, as part of a multidisciplinary approach, is feasible and safe.20 According to the experience we reported, in palliative care, endovascular treatment should be as aggressive as possible, to achieve a long-lasting improvement of the symptomatology. Management of giant, high-flow, type-C AVM remains often challenging. An aggressive endovascular treatment allows stabilizing the disease more than scleroembolization, if surgical procedure cannot achieve a complete excision.
Annals of Vascular Surgery
REFERENCES 1. Jackson IT, Carrenco R, Potparic Z, et al. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg 1993;91:1216e30. 2. Garzon MC, Huang JT, Enjolras O, et al. Vascular malformations: part I. J Am Acad Dermatol 2007;56:353e70. quiz 371e374. 3. Enjolras O. Classification and management of the various superficial vascular anomalies: hemangiomas and vascular malformations. J Dermatol 1997;24:701e10. 4. Vaidya S, Cooke D, Kogut M, et al. Imaging and percutaneous treatment of vascular anomalies. Semin Intervent Radiol 2008;25:216e33. 5. Szilagyi DE, Smith RF, Elliott JP, et al. Congenital arteriovenous anomalies of the limbs. Arch Surg 1976;111:423e9. 6. Halliday AW, Smith EJ, Jackson J, et al. Indications for surgery for arteriovenous malformations. Br J Surg 1992;79:361e2. 7. Halliday AW, Mansfield AO. Arteriovenous malformations: current management approaches. Br J Hosp Med 1989;42: 196e202. 8. Decker DG, Fish CR, Juergens JL. Arteriovenous fistulas of the female pelvis: a diagnostic problem. Surg Obstet Gynecol 1968;31:799e805. 9. Flye MW, Jordan BP, Schwartz MZ. Management of congenital arteriovenous malformations. Surgery 1983;94:740e7. 10. Lee BB, Do YS, Yakes W, et al. Management of arterialvenous shunting malformations (AVM) by surgery and embolosclerotherapy. A multidisciplinary approach. J Vasc Surg 2004;3:596e600. 11. Hemingway AP, Allison DJ. Cardiac failure secondary to giant systemic arteriovenous malformations: treatment by embolization. In: St Belov, Loose DA, Weber J eds. Vascular malformations. Reinbek, Germany: Einhorn-Presse Verlag GmbH, 1989. pp 275e8. 12. Malan E. Hemodynamic phenomena in congenital arteriovenous fistulae. In: Malan E ed. Vascular malformations. Milan, Italy: Carlo Erba, 1974. pp 38e58. 13. Kurelio LJ, Kopchick JH, Irwin JL, et al. Congenital pelvic arteriovenous malformation. Urology 1986;27:165e7. 14. Mulliken JB. Cutaneous vascular anomalies. Semin Vasc Surg 1993;6:204e18. 15. Lee BB. Advanced management of congenital vascular malformation (CVM). Int Angiol 2002;21:209e13 [Editorial]. 16. Yamaki T. Prospective randomized efficacy of ultrasoundguided foam sclerotherapy compared with ultrasoundguided liquid sclerotherapy in the treatment of symptomatic venous malformations. J Vasc Surg 2008;47:578e84. 17. Lee BB. What is new in venous disease: new approach to old problem of venous diseasedcongenital vascular malformation. In: Angelides NS ed. Advances in phlebology. Limassol: Hadjigeogiou Printing and Co, 1998. pp 59e64. 18. Park UJ, Do YS, Park KB, et al. Treatment of arteriovenous malformation involving the hand. Ann Vasc Surg 2012;26: 643e8. 19. Richter GT, Friedman AB. Hemangiomas and vascular malformations: current theory and management. J Vasc Surg 2001;33:51e5. 20. Mendonca CT, Miyamotto M. Multidisciplinary approach in the management of a giant axillary arteriovenous malformation. J Vasc Surg 2008;47:657.
We report the case of a 58-year-old man presented with bleeding ulcer of the left arm caused by a high-flow type-C arteriovenous malformation (AVM), feed by branches from both the subclavian arteries. He had been previously treated with AVM sclerotherapy, embolization, humeral artery endografting, and open surgery. We urgently performed coil embolization of the left vertebral artery, and we covered the huge subclavian artery by a thoracic endograft. Then, we embolized the right tyrocervical trunk. The result was an immediate interruption of bleeding. At 12 months, the patient had no neurologic complications, and the upper limb continued to decompress.
Arteriovenous malformations (AVMs) are an extremely heterogeneous group of vascular disorders characterized by different clinical presentations, systemic hemodynamic effects, and possible treatments. AVMs are divided into low-flow and high-flow (subgroups A, B, and C) AVMs.1e4 Complete eradication of the nidus is the only potential therapy, but this, however, is not always possible. Various authors have published that only 20% of malformations may be amenable to complete extirpation with surgery.5e7 Partial resections can cause a good initial clinical response that may last for some time. However, very often the patient’s presenting symptoms recur or worsen at follow-up.5,8,9 Endovascular treatment may be a therapeutic option especially where surgery would be
Department of Vascular Surgery, Policlinico Universitario ‘‘G. Martino’’ of Messina, Messina, Italy. Correspondence to: David Barilla, MD, Department of Vascular Surgery, Policlinico Universitario ‘‘G. Martino’’ of Messina, Via Consolare Valeria, Messina 98100, Italy; E-mail: [email protected] Ann Vasc Surg 2014; 28: 1932.e5e1932.e8 http://dx.doi.org/10.1016/j.avsg.2014.06.069 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: March 16, 2014; manuscript accepted: June 16, 2014; published online: July 11, 2014.
impossible because of extensive involvement beyond the deep fascia to muscle, tendon, bone, and joints. Preoperative endovascular techniques improve the overall safety and efficacy of surgery, reducing morbidity and complications.10 We report the case of a 58-year-old man presented with bleeding ulcers of the left arm caused by a giant, type-C, high-flow AVM, feed by branches originating from both the subclavian arteries involving the left shoulder and the upper limb. The patient required staged endovascular treatments to arrest recurrent massive bleeding as lifesaving procedure.
CASE REPORT A 58-year-old man was treated for a previous small high-flow AVM on the left shoulder and upper limb, in other institutions. Over 15 years, the patient underwent multiple surgical and endovascular procedures which let a small high-flow AVM to evolve into a giant, type-C, high-flow AVM, resulting in limb function impairment and high-output cardiac state, with subsequent congestive heart failure and atrial fibrillation. Patient’s clinical history was reconstructed from previous clinical documentation. He underwent, over the years, ultrasound- and fluoroscopic-guided adjuvant sclerotherapy with polyvinyl alcohol, embolization with fibered coils and glues, and covered stent’s placement. 1932.e5
1932.e6 Case reports
Annals of Vascular Surgery
Fig. 1. Preoperative clinical presentation and angio-CT scan.
The surgical time was limited to ligation of feeding arteries; total excision of the mass was never possible because of massive bleeding, so the nidus was always left intact. Recently because of the massive proliferation and bleeding of his AVM, patient was referred to our center’s emergency room. At presentation, the left upper limb was warm and hypertrophic with several abnormal vessels clearly evident on the anterior chest wall up to the wrist, and there were bleeding ulcers on the posterolateral surface of the arm. Computed tomographic (CT) angiography demonstrated an irregular vascular network with huge arteriovenous shunts; the left subclavian and vertebral arteries and their branches were dilated and from these originated aberrant branches afferent to several vascular nidi (Fig. 1). We urgently submitted the patient to endovascular treatment. The first step was to catheterize the left vertebral artery, pushing the catheter flush to the origin of the posterior inferior cerebellar artery (PICA). Then, with the vertebral catheter still in place, a thoracic stent graft (Conformable GORE TAG Thoracic Endoprosthesis 21-21-10) was deployed into the aneurysmal subclavian artery from its origin up to previous stent grafts with 3 cm overlapping, covering all its branches. Afterward, with the origin of the subclavian artery already blocked by the thoracic endograft, we performed retrograde coil embolization (Fibered Platinum Coil; Boston Scientific) of the vertebral artery between the PICA and the origin, thus avoiding a possible distal migration of the coils (Fig. 2). At the end of the procedure, partial collapse of venous nidi and stop of bleeding were achieved and no side branches were evident (Fig. 2).
Seventy-two hours later, the patient underwent postoperative CT angiography that showed a persistent feeding vessel to the AVM from the right subclavian via an aberrant tyrocervical trunk and residual feeding from the brachial bifurcation. Right tyrocervical trunk embolization was performed, while the brachial bifurcation feeders were left intact to prevent the occlusion of the extensively covered proximal limb arteries. During a 12-month follow-up, we have observed a gradual thrombosis of the AVM aberrant vessels and a gradual decrease in volume of the upper limb with the resolution of bleeding episodes. As we achieved cessation the active bleeding we were eventually satisfied with the result and we estimated that the venous collectors from the nidus were too large to attempt any safe sclerotherapy (Fig. 3).
DISCUSSION The origin of AVMs has been controversial, and it has generated several theories and classification systems.1e4 Treatment remains a challenge. They have a wide range of clinical presentation and an unpredictable course.5,8,9 High morbidity has been related to both surgical and nonsurgical treatments10e12, with an associated high recurrence rate. They form multiple macroscopic and microscopic arteriovenous shunts, from which origins massive congestion of tissues.13 The primary effects are compression and erosion of surrounding tissues. Secondary hemodynamic effects include potential arterial steal
Vol. 28, No. 8, November 2014
Case reports 1932.e7
Fig. 2. (A) Selective angiography of the left vertebral artery documenting aberrant branches feeding the nidus. (B) Left vertebral artery embolization with coils. (C) Postoperative angiography.
Fig. 3. (A) Angio-CT scan. (B) Postoperative clinical presentation.
phenomena. Heart can be affected by high-output cardiac failure. Peripheral tissues can be affected in a wide range of changes from distal ischemia to gangrene, venous stasis dermatitis, and ulcer or gangrene caused by venous hypertension.11e14 The treatment depends on the type, extent, and location of AVMs. Lee suggests that treatment has to be set up separately for the ‘‘primary
malformation’’ itself first from their ‘‘secondary disorders’’ along the vascular system and/or skeleton and soft tissues, considering absolute indications of treatment of life-treating lesions and relative indications of various conditions to affect the quality of life significantly beside the lesions with potentially high risk of complications, vascular bone syndrome, and cosmetically severe deformity.15
1932.e8 Case reports
The case reported had an absolute indication of treatment, for the presence of recurrent massive bleeding, high-output heart failure, and patchy limb ulcerations. Nowadays, the treatment is based on multidisciplinary approaches, including conservative therapy, nonsurgical interventional treatment, and traditional surgery.15,16 Conservative therapy involves compressive garments and acetyl salicylic acid.17 Nonsurgical interventional treatment includes scleroembolization, whereas surgical treatment is only performed when the complete exeresis of the lesion is achievable. Surgical treatment aims to the complete excision of the nidus of the AVM. Its feasibility relies on the anatomy of the lesion. However, this treatment may expose the patient to a risk of massive intraoperative hemorrhages, as it had occurred in our case, and of possible neurologic deficiency.5,8 Although eradication of the nidus is necessary to obtain a definitive healing of the lesion,10 many AVMs require to fold back on a more conservative approach for the unfeasible complete excision of the lesion for its anatomic features. A surgical procedure that does not achieve a complete excision may result in worsening of the pathology because of early recurrence and collateralization of feeding arteries.18 In our case, the attempt at surgical therapy has been compromised by massive hemorrhage. The incomplete surgical treatment has been followed by progressive growth of the mass that expanded to the root of the limb and to the lateral surface of thorax, thus precluding an amputation/disarticulation of the left upper limb. As reported by many authors, the gold standard for unresectable lesions, which had low rate of improvement from embolization, is still the endovascular treatment.19 Our case is an example of palliative treatment after failure of multidisciplinary attempts of lesion eradication. The previous treatment was based both on surgery and on nonsurgical treatment. Literature reports that the use of covered stents to occlude aberrant branches, as part of a multidisciplinary approach, is feasible and safe.20 According to the experience we reported, in palliative care, endovascular treatment should be as aggressive as possible, to achieve a long-lasting improvement of the symptomatology. Management of giant, high-flow, type-C AVM remains often challenging. An aggressive endovascular treatment allows stabilizing the disease more than scleroembolization, if surgical procedure cannot achieve a complete excision.
Annals of Vascular Surgery
REFERENCES 1. Jackson IT, Carrenco R, Potparic Z, et al. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg 1993;91:1216e30. 2. Garzon MC, Huang JT, Enjolras O, et al. Vascular malformations: part I. J Am Acad Dermatol 2007;56:353e70. quiz 371e374. 3. Enjolras O. Classification and management of the various superficial vascular anomalies: hemangiomas and vascular malformations. J Dermatol 1997;24:701e10. 4. Vaidya S, Cooke D, Kogut M, et al. Imaging and percutaneous treatment of vascular anomalies. Semin Intervent Radiol 2008;25:216e33. 5. Szilagyi DE, Smith RF, Elliott JP, et al. Congenital arteriovenous anomalies of the limbs. Arch Surg 1976;111:423e9. 6. Halliday AW, Smith EJ, Jackson J, et al. Indications for surgery for arteriovenous malformations. Br J Surg 1992;79:361e2. 7. Halliday AW, Mansfield AO. Arteriovenous malformations: current management approaches. Br J Hosp Med 1989;42: 196e202. 8. Decker DG, Fish CR, Juergens JL. Arteriovenous fistulas of the female pelvis: a diagnostic problem. Surg Obstet Gynecol 1968;31:799e805. 9. Flye MW, Jordan BP, Schwartz MZ. Management of congenital arteriovenous malformations. Surgery 1983;94:740e7. 10. Lee BB, Do YS, Yakes W, et al. Management of arterialvenous shunting malformations (AVM) by surgery and embolosclerotherapy. A multidisciplinary approach. J Vasc Surg 2004;3:596e600. 11. Hemingway AP, Allison DJ. Cardiac failure secondary to giant systemic arteriovenous malformations: treatment by embolization. In: St Belov, Loose DA, Weber J eds. Vascular malformations. Reinbek, Germany: Einhorn-Presse Verlag GmbH, 1989. pp 275e8. 12. Malan E. Hemodynamic phenomena in congenital arteriovenous fistulae. In: Malan E ed. Vascular malformations. Milan, Italy: Carlo Erba, 1974. pp 38e58. 13. Kurelio LJ, Kopchick JH, Irwin JL, et al. Congenital pelvic arteriovenous malformation. Urology 1986;27:165e7. 14. Mulliken JB. Cutaneous vascular anomalies. Semin Vasc Surg 1993;6:204e18. 15. Lee BB. Advanced management of congenital vascular malformation (CVM). Int Angiol 2002;21:209e13 [Editorial]. 16. Yamaki T. Prospective randomized efficacy of ultrasoundguided foam sclerotherapy compared with ultrasoundguided liquid sclerotherapy in the treatment of symptomatic venous malformations. J Vasc Surg 2008;47:578e84. 17. Lee BB. What is new in venous disease: new approach to old problem of venous diseasedcongenital vascular malformation. In: Angelides NS ed. Advances in phlebology. Limassol: Hadjigeogiou Printing and Co, 1998. pp 59e64. 18. Park UJ, Do YS, Park KB, et al. Treatment of arteriovenous malformation involving the hand. Ann Vasc Surg 2012;26: 643e8. 19. Richter GT, Friedman AB. Hemangiomas and vascular malformations: current theory and management. J Vasc Surg 2001;33:51e5. 20. Mendonca CT, Miyamotto M. Multidisciplinary approach in the management of a giant axillary arteriovenous malformation. J Vasc Surg 2008;47:657.