Fibrodysplastic implications for transvenous embolization of a high-flow pelvic arteriovenous malformation in Osler-Weber-Rendu syndrome

Abstract

Osler-Weber-Rendu syndrome is a rare genetic disorder that commonly features high-flow arteriovenous malformations (AVM) within the pulmonary, intracranial, and visceral circulation. We present a patient with a unique case of OslerWeber-Rendu syndrome featuring a high-flow pelvic AVM in addition to fibromuscular dysplasia affecting multiple vascular beds. This required a unique modification of our embolic therapeutic approach for adequate treatment of the AVM. (J Vasc Surg Cases 2015;1:16-9.) Osler-Weber-Rendu (OWR) syndrome is a rare genetic disorder that commonly features high-flow arteriovenous malformations (AVM) within the pulmonary, intracranial, and visceral circulation. We present a patient with a unique case of OWR syndrome featuring a high-flow pelvic AVM in addition to fibromuscular dysplasia (FMD) affecting multiple vascular beds. Consent to publish this case report was obtained from patient. CASE REPORTA 59-year-old woman with a history of OWR syndrome was evaluated in consultation for a symptomatic left pelvic AVM discovered on a computed tomography (CT) scan. Symptoms included a dull, throbbing ache, waxing and waning in severity, with exacerbation of symptoms after activity. She also reported a personal and first-degree relative history of chronic, persistent epistaxis. Her physical examination was remarkable for multiple, widespread erythematous macules (telangiectasias) throughout the volar aspect of the acral digits, tongue, and buccal mucosa (Fig 1, A and B). Left lower quadrant tenderness to deep palpation and a faint local bruit was appreciated.The CT scan confirmed the presence of large-caliber, tortuous arterial feeders supplying the nidus of a high-flow left pelvic AVM with prompt shunting into an aneurysmal draining vein, which eventually emptied into the left gonadal venous system (Fig 1, C). The arterial supply seemed to arise from the hypogastric or the gonadal arteries, or both. Results of the laboratory evaluation were unremarkable aside from mild microcytic anemia.The patient was offered a diagnostic angiography with the option for transcatheter embolization of this symptomatic, high-flow AVM.Technical details. The procedure was performed in a hybrid operating suite, with the patient under general anesthesia. No systemic anticoagulation was given; however, generous and frequent heparinized saline irrigation of sheaths and catheters was performed throughout the case. Flush aortography confirmed the presence of a high-flow AVM supplied by an enlarged, redundant, and extremely tortuous left gonadal artery, with prompt shunting into aneurysmal draining veins, which eventually emptied into the left gonadal venous system. No significant left renal vein hemodynamic abnormalities were noted. Selective catheterization of the left hypogastric artery revealed no association with the AVM. Also noted were diffuse, medial fibrodysplastic lesions with a characteristic chain of beads appearance involving the bilateral external iliac arteries and the left renal artery (Fig 2, A and B).Selective...

Evaluation and management of congenital peripheral arteriovenous malformations

“…26,28 AVM nidus access can be performed via transarterial, transvenous, or direct puncture routes (Figs 1, 2, 4, and 5). 9,48,49 Sometimes, a combination of these approaches may be necessary (Fig 1). Although transarterial access is the most common and straightforward, it may not always be achievable owing to extreme tortuosity of feeding vessels, vasospasm, and innumerable arterial feeders (Figs 2 and 4).…”

“…9,[49][50][51][52][53] The use of liquid embolic agents via the transvenous route is technically challenging given directionality of blood flow. 48,49 This may require adjunctive, flow-limiting techniques such as balloon-facilitated outflow and/or inflow control, and deliberate use of polymerizing embolic agents so as to minimize nontarget embolization. 9,48,49,[51][52][53] Direct, percutaneous access into the AVM nidus is another viable option that can be used alone or in conjunction with other techniques (Figs 1 and 5).…”

“…48,49 This may require adjunctive, flow-limiting techniques such as balloon-facilitated outflow and/or inflow control, and deliberate use of polymerizing embolic agents so as to minimize nontarget embolization. 9,48,49,[51][52][53] Direct, percutaneous access into the AVM nidus is another viable option that can be used alone or in conjunction with other techniques (Figs 1 and 5). 9 The location of the nidus plays an important role in determining the feasibility of this option and is not always possible.…”

“…Alternatively, nidus delivery via direct stick embolization and/or retrograde, transvenous approach are also achievable depending on the angioarchitecture of the AVM. 9,[48][49][50] n-BCA is an acrylic adhesive that rapidly polymerizes and expands via an exothermic reaction when exposed to an ionic medium such as blood, thereby forming an almost instantaneous cast. The polymerization rate can be prolonged by the addition of iodized ethyl esters (Lipiodol; Guerbet USA, Bloomington Ind), which can allow for more distal penetration of n-BCA.…”

“…29 Alternatively, these devices may provide a scaffold upon which further embolization via liquid embolic agents can be performed, thereby decreasing the volume of embolic agent needed to shut down the AVM. 26,48 They can also be deployed as flow rate control adjunctive modalities typically within aneurysmal draining veins so as to minimize risk of nontarget embolization of liquid embolic agents during transarterial or direct access delivery. 29…”