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Endovascular repair of multiple infrageniculate aneurysms in a patient with vascular type Ehlers-Danlos syndrome

2011; Elsevier BV; Volume: 54; Issue: 3 Linguagem: Inglês

10.1016/j.jvs.2011.01.035

1097-6809

Natalie Domenick, Jae S. Cho, Ghassan Abu Hamad, Michel S. Makaroun, Rabih A. Chaer,

Aortic Disease and Treatment Approaches

Patients with vascular type Ehler-Danlos syndrome can develop aneurysms in unusual locations. We describe the case of a 33-year-old woman with vascular type Ehlers-Danlos syndrome who developed metachronous tibial artery aneurysms that were sequentially treated with endovascular means. Patients with vascular type Ehler-Danlos syndrome can develop aneurysms in unusual locations. We describe the case of a 33-year-old woman with vascular type Ehlers-Danlos syndrome who developed metachronous tibial artery aneurysms that were sequentially treated with endovascular means. Patients with vascular type Ehlers-Danlos syndrome (EDS), previously Ehlers-Danlos type IV, can develop aneurysms in unusual locations.1Pepin M.G. Byers P.H. Ehlers-Danlos syndrome, vascular type.http://www.genetests.orgGoogle Scholar Unfortunately, vascular rupture or dissection is a common presentation reported to occur in 77% of affected patients.1Pepin M.G. Byers P.H. Ehlers-Danlos syndrome, vascular type.http://www.genetests.orgGoogle Scholar, 2Oderich G.S. Panneton J.M. Bower T.C. Lindor N.M. Cherry K.J. Noel A.A. et al.The spectrum, management and clinical outcome of Ehlers-Danlos type IV: a 30-year experience.J Vasc Surg. 2005; 42: 98-106Abstract Full Text Full Text PDF PubMed Scopus (305) Google Scholar We describe the case of a young woman with vascular EDS who developed metachronous tibial artery aneurysms sequentially treated with coil embolization and stent graft exclusion. A 33-year-old woman first presented to our institution in 2001 with severe abdominal pain following a recent appendectomy at an outside hospital. Her past medical history was significant for coronary artery disease of unclear etiology, myocardial infarction, and factor V Leiden deficiency. She had no known family history of collagen vascular disease. An exploratory laparotomy was performed under the presumptive diagnosis of intestinal ischemia. The laparotomy was negative, but 6 days into recovery, the patient was re-explored for shock and abdominal distention. A ruptured splenic artery aneurysm was noted and treated with ligation and splenectomy. Within 1 week, the patient developed acute swelling of her left arm with a compartment syndrome secondary to rupture of an aneurysm of a large branch of the brachial artery. This was temporally related to a blood pressure cuff inflation and was treated with ligation and compartment release. A skin biopsy was performed for suspected vasculitis, and biochemical testing of fibroblast collagen secretion, followed by DNA evaluation, confirmed the diagnosis of vascular EDS. Eight months later, the patient was diagnosed with bilateral anterior tibial artery aneurysms, which were successfully coil embolized in a staged fashion. Her posterior tibial and peroneal vessels were not aneurismal at that time. Seven years after her initial presentation, the patient reported to the emergency department with hypotension and a spontaneous, painful, rapidly expanding lump in her right upper thigh. Emergency surgery revealed a hemorrhaging venous malformation at and beyond the level of the femoral vein confluence with the saphenous vein. Pressure control was obtained and wide suture ligation performed. Postoperatively, the patient developed deep vein thrombosis of the right lower extremity and was anticoagulated with coumadin. Her lower extremity swelling resolved with compression stockings. The patient was also known to have a left posterior tibial artery (PTA) aneurysm (Fig 1). Over 2 years, duplex ultrasound evaluation demonstrated an increase in diameter from 2 to 3 cm at which time the patient developed a pulsatile calf mass on clinical examination. Though asymptomatic, need for repair was based on the absolute size and enlarging diameter. To maintain two-vessel runoff to the foot, the decision was made to attempt stent graft repair, given that the diameter of the nonaneurismal portion of the PTA was large enough and was 4 mm by CT measurement. Through contralateral up and over transfemoral access with a 7F sheath, a 5 × 10 mm self-expanding Viabahn stent graft (W. L. Gore and Associates, Flagstaff, Ariz) was deployed across the aneurysm into the PTA outflow. Poststenting angioplasty with a 5 mm balloon was performed. A completion angiogram showed good apposition of the stent and complete exclusion of the aneurysm (Fig 2). Access site hemostasis was achieved with manual compression after immediate failure of attempted closure with an 8F angioseal (Saint Jude Medical, St. Paul, Minn). The patient developed a hematoma at the puncture site that spontaneously resolved and was acutely investigated by duplex imaging and CT scan. She maintained a palpable PTA pulse through her hospital stay and was discharged home on clopidogrel. No evidence of pseudoaneurysm was noted on follow-up duplex scanning at 1 and 4 weeks. At 1-month follow-up, the PTA pulse was lost, and the stent graft was completely occluded by duplex ultrasound, with a patent PTA at the ankle but no flow into the PTA aneurysm. The patient was asymptomatic with normal ambulation status. At 1-year follow-up, the patient continued to be asymptomatic, with an excluded PTA aneurysm.Fig 2A, Initial angiogram demonstrating a posterior tibial artery (PTA) aneurysm with coils noted (small arrows) in the anterior tibial artery distribution at the site of a previous aneurysm. B, A large PTA aneurysm is noted with mural thrombus (large arrow) and significant outflow tortuosity. C, Complete exclusion of the PTA aneurysm is noted following deployment of a Viabahn (W. L. Gore and Associates) stent graft.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Infrapopliteal aneurysms are exceedingly rare and have a poorly characterized natural history.3Cappendijk V.C. Mouthaan P.J. A true aneurysm of the tibioperoneal trunk: case report and literature review.Eur J Vasc Endovasc Surg. 1999; 18: 536-537Abstract Full Text PDF PubMed Scopus (15) Google Scholar Prior to this report, only 41 true aneurysms below the popliteal fossa have been reported in the English literature,3Cappendijk V.C. Mouthaan P.J. A true aneurysm of the tibioperoneal trunk: case report and literature review.Eur J Vasc Endovasc Surg. 1999; 18: 536-537Abstract Full Text PDF PubMed Scopus (15) Google Scholar, 4Agarwal M. Harkless L. Hagino R.T. Toursarkissian B. Lateral plantar artery aneurysm: a case report.J Am Podiatr Med Assoc. 2007; 97: 480-482PubMed Google Scholar, 5Ferrero E. Ferri M. Viazzo A. Gaggiano A. Berardi G. Piazza S. et al.Rupture of a true giant aneurysm of the posterior tibial artery: a huge size of 6 cm on diameter.Ann Vasc Surg. 2010; 24: 1134.e9-1134.e13Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar, 6Kato T. Takagi H. Sekino S. Manabe H. Matsuno Y. Furuhashi K. et al.Dorsalis pedis artery true aneurysm due to atherosclerosis: case report and literature review.J Vasc Surg. 2004; 40: 1044-1048Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 7Maydew M.S. Dorsalis pedis aneurysm: ultrasound diagnosis.Emerg Radiol. 2007; 13: 277-280Crossref PubMed Scopus (23) Google Scholar, 8Patel S. D'Souza N. Gurjar S.V. Hewes J.C. Edrees W. Mycotic aneurysm of the posterior tibial artery–a rare complication of bacterial endocarditis: a case report.J Med Case Rep. 2008; 2: 341Crossref PubMed Scopus (8) Google Scholar, 9Robaldo A. Colotto P. Palombo D. True atherosclerotic pedis artery aneurysm.Interact Cardiovasc Thorac Surg. 2010; 11: 216-217Crossref PubMed Scopus (9) Google Scholar, 10Tshomba Y. Papa M. Marone E.M. Kahlberg A. Rizzo N. Chiesa R. A true posterior tibial artery aneurysm: a case report.Vasc Endovasc Surg. 2006; 40: 243-249Crossref PubMed Scopus (16) Google Scholar 14 of those involving posterior tibial arteries,5Ferrero E. Ferri M. Viazzo A. Gaggiano A. Berardi G. Piazza S. et al.Rupture of a true giant aneurysm of the posterior tibial artery: a huge size of 6 cm on diameter.Ann Vasc Surg. 2010; 24: 1134.e9-1134.e13Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar and none with concomitant vascular EDS. Since rupture is rare,5Ferrero E. Ferri M. Viazzo A. Gaggiano A. Berardi G. Piazza S. et al.Rupture of a true giant aneurysm of the posterior tibial artery: a huge size of 6 cm on diameter.Ann Vasc Surg. 2010; 24: 1134.e9-1134.e13Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar small and asymptomatic infrapopliteal aneurysms may be observed safely, while symptomatic aneurysms require prompt surgical intervention, most commonly direct repair or ligation.5Ferrero E. Ferri M. Viazzo A. Gaggiano A. Berardi G. Piazza S. et al.Rupture of a true giant aneurysm of the posterior tibial artery: a huge size of 6 cm on diameter.Ann Vasc Surg. 2010; 24: 1134.e9-1134.e13Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar, 6Kato T. Takagi H. Sekino S. Manabe H. Matsuno Y. Furuhashi K. et al.Dorsalis pedis artery true aneurysm due to atherosclerosis: case report and literature review.J Vasc Surg. 2004; 40: 1044-1048Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar, 11Kanaoka T. Matsuura H. A true aneurysm of the posterior tibial artery: a case report.Ann Thorac Cardiovasc Surg. 2004; 10: 317-318PubMed Google Scholar These generalizations must be carefully evaluated in light of the overall vascular fragility of patients with vascular EDS.2Oderich G.S. Panneton J.M. Bower T.C. Lindor N.M. Cherry K.J. Noel A.A. et al.The spectrum, management and clinical outcome of Ehlers-Danlos type IV: a 30-year experience.J Vasc Surg. 2005; 42: 98-106Abstract Full Text Full Text PDF PubMed Scopus (305) Google Scholar In fact, 44% of vascular EDS patients with arterial rupture die before repair, necessitating strategic management of vascular complications prior to rupture.12Sugawara Y. Ban K. Imai K. Okada K. Watari M. Orihashi K. et al.Successful coil embolization for spontaneous arterial rupture in association with Ehlers–Danlos syndrome type IV: report of a case.Sur Today. 2004; 34: 94-96Crossref PubMed Scopus (21) Google Scholar In light of our patient's history of multiple ruptured aneurysms prior to development of her PTA aneurysm, and given the size and enlarging diameter of her aneurysm, intervention was decided. Since a single vessel runoff is sufficient if the pedal arch is patent, it is generally acceptable to sacrifice an aneurismal tibial vessel.10Tshomba Y. Papa M. Marone E.M. Kahlberg A. Rizzo N. Chiesa R. A true posterior tibial artery aneurysm: a case report.Vasc Endovasc Surg. 2006; 40: 243-249Crossref PubMed Scopus (16) Google Scholar As in severe atherosclerotic disease associated with tibial aneurysm,11Kanaoka T. Matsuura H. A true aneurysm of the posterior tibial artery: a case report.Ann Thorac Cardiovasc Surg. 2004; 10: 317-318PubMed Google Scholar this practice also requires personalized deliberation in a patient with vascular EDS. Our patient had already lost ATA runoff from prior coil embolization, and coiling of her PTA aneurysm would leave her with a single vessel runoff that is potentially at risk for future aneurismal degeneration. While stent graft repair is generally accepted for popliteal aneurysms,13Cina C.S. Endovascular repair of popliteal aneurysms.J Vasc Surg. 2010; 51: 1056-1060Abstract Full Text Full Text PDF PubMed Scopus (46) Google Scholar it is not well established for tibial aneurysms. Only four accounts of stent graft repair of an ATA pseudoaneurysm and one of a PTA pseudoaneurysm, all successful, have been reported in the English literature.14Joglar F. Kabutey N.K. Maree A. Farber A. The role of stent grafts in the management of traumatic tibial artery pseudoaneurysms: case report and review of the literature.Vasc Endovasc Surg. 2010; 44: 407-409Crossref PubMed Scopus (31) Google Scholar Stents used included JOSTENT (Abbott Vascular Devices, Abbot Park, Ill) or Symbiot stent graft (Boston Scientific, Natick, Mass),14Joglar F. Kabutey N.K. Maree A. Farber A. The role of stent grafts in the management of traumatic tibial artery pseudoaneurysms: case report and review of the literature.Vasc Endovasc Surg. 2010; 44: 407-409Crossref PubMed Scopus (31) Google Scholar and only short-term patency was reported. No reports exist describing stent grafting of true tibial artery aneurysms or of vascular EDS complications.2Oderich G.S. Panneton J.M. Bower T.C. Lindor N.M. Cherry K.J. Noel A.A. et al.The spectrum, management and clinical outcome of Ehlers-Danlos type IV: a 30-year experience.J Vasc Surg. 2005; 42: 98-106Abstract Full Text Full Text PDF PubMed Scopus (305) Google Scholar Moreover, stenting presents a potential risk of arterial wall injury and pseudoaneurysm development at the access site in these patients.14Joglar F. Kabutey N.K. Maree A. Farber A. The role of stent grafts in the management of traumatic tibial artery pseudoaneurysms: case report and review of the literature.Vasc Endovasc Surg. 2010; 44: 407-409Crossref PubMed Scopus (31) Google Scholar Nevertheless, endovascular repair was chosen due to the potential advantage of maintaining a two-vessel runoff and given the possibility of future peroneal aneurysm formation. The Viabahn (W. L. Gore and Associates) stent was selected because of the suitable large landing zone diameter, the stent's proven performance in popliteal aneurysms, and its superior flexibility compared with balloon expandable stent grafts. Surgical bypass was not considered given the associated risks in vascular EDS patients in general1Pepin M.G. Byers P.H. Ehlers-Danlos syndrome, vascular type.http://www.genetests.orgGoogle Scholar and the patient's history of symptomatic coronary artery disease. Although tibial artery stent grafting has been described, the durability of covered stents in this vascular bed is not well characterized and could be adversely affected by small target vessel diameter and excessive mobility from the calf musculature. This could explain the graft failure in this patient, along with excessive vessel tortuosity and her coexisting hypercoagulable state. While coiling is probably the safest intervention in vascular EDS patients,2Oderich G.S. Panneton J.M. Bower T.C. Lindor N.M. Cherry K.J. Noel A.A. et al.The spectrum, management and clinical outcome of Ehlers-Danlos type IV: a 30-year experience.J Vasc Surg. 2005; 42: 98-106Abstract Full Text Full Text PDF PubMed Scopus (305) Google Scholar stent grafting is a consideration when faced with preservation of runoff and limb viability. Given her history, this patient has the potential to develop future tibial aneurysms requiring intervention, making maintenance of a two-vessel runoff a sensible goal. Patients with vascular EDS can present with multiple tibial artery aneurysms, limiting the options of runoff preservation. Endovascular treatment is safe and can be achieved with coil embolization. Stent grafting may be attractive to maximize runoff, but patency of current grafts in the tibial bed is unproven.