Embolization Techniques for Arteriovenous Malformations in Parkes-Weber Syndrome.

BACKGROUND: Multiple high-flow arteriovenous malformations (AVMs) are the hallmark of Parkes-Weber syndrome (PWS). Surgical resection has historically shown poor outcomes with PWS. The aim of this study was to assess the management of PWS in the current era. METHODS: A retrospective review was performed from 2004 to 2017 on all patients presenting to a single institution for the management of PWS. Presentation, techniques, complications, and outcomes were reviewed. RESULTS: Fourteen patients (50% male) with PWS were seen at our institution, and mean age was 19.9 years (range, 4.7-68.8). The lower extremity was affected in 12 (86%) and the upper extremity in 2 (14%) patients. All patients presented with pain and swelling in the affected limb. Seven (50%) patients presented with ulcers, of which 3 (43%) had extensive wounds. Five (36%) patients had echocardiographic evidence of high-output cardiac failure. All patients underwent angiography with the intention to treat. Three (21%) patients were found to have diffuse arteriovenous communication with no discrete AVM nidus and thus did not undergo intervention. The remaining 11 (79%) patients underwent transcatheter embolization of the AVM's arterial inflow. Six (55%) patients required multiple or staged inflow embolization procedures, with a mean of 3.3 (range, 1-10) interventions. Thirty-two arterial embolization procedures were performed in total. n-Butyl-2-cyanoacrylate (nBCA) adhesive was used in 22 (69%), microspheres in 8 (25%), and a combination of coils and nBCA adhesive in 2 (6%) cases. Technical angiographic success was seen in all patients. Six (55%) patients also had interventions to treat the venous component of the malformation, either concomitantly or during a separate procedure. This included radiofrequency ablation in 1 (17%), coil embolization in 1 (17%), sodium tetradecyl sulfate (STS) sclerotherapy in 2 (33%), and a combination of STS, coil embolization, and vein stripping in 2 (33%) patients. Ten (91%) patients experienced a partial response and 1 (9%) patient experienced no response to treatment. No patients had a complete response, as expected with the diffuse nature of this disease. There were no periprocedural complications. Two of 3 patients with complex wounds required major amputations for gangrene, including one above-knee and one below-knee amputation at 128 months and 66 months after the index procedure, respectively. CONCLUSIONS: AVMs in PWS can be successfully treated by a transcatheter approach. Multiple interventions are usually required. Patients with extensive wounds remain at risk for loss of limb.

Major Limb Amputations in Patients with Congenital Vascular Malformations.

BACKGROUND: The need for major amputations in patients with vascular malformations is rare. This study reviews our contemporary experience with major amputations in patients with vascular malformations. METHODS: A retrospective review from April 2014 to November 2018 identified 993 patients undergoing management of a vascular malformation involving the upper or lower extremity at a tertiary center. This population was analyzed to identify those requiring either a transfemoral or transhumeral amputation. This cohort was investigated for clinical course, surgical procedures, and outcomes. RESULTS: Five patients (0.5%) underwent major amputation, including 3 transhumeral and 2 above-knee amputations. The median age was 37.8 years (interquartile range (IQR): 25.4-40.2), and 2 (40%) were male. Four (80%) patients had high-flow arteriovenous malformations, including 1 (20%) with Parkes-Weber syndrome. One (20%) patient had a low-flow venous malformation associated with Klippel-Trénaunay syndrome. All patients had malformation extending into the chest or pelvis, with the amputation being at the level of residual malformation. As such, amputation had been initially felt to be high risk because of the proximal extent of the lesions. Before amputation, a median of 11 procedures (IQR: 4-39) were performed per patient. This included 29 transarterial embolizations, 4 transvenous embolizations, 20 direct stick embolizations, 3 debulking procedures, 38 debridements, 6 skin grafts or muscle flaps, and 4 minor amputations. The median time course of treatment before amputation was 117 months (IQR: 44-171). Indications for major amputation included chronic pain and recurrent bleeding in all 5 (100%) patients, loss of function in 2 (40%), nonhealing wounds in 2 (40%), and sepsis in 1 (20%) patient. There were no perioperative deaths. The median blood loss was 1,000 mL (IQR: 650-2,750). All patients required transfusion of packed red blood cells with a mean of 1.6 units (standard deviation: 0.54). Transhumeral amputation was facilitated by transcatheter embolization in 1 (33%) and an occlusion balloon within the subclavian artery in 2 (66%) patients. The median length of stay was 6 days (IQR: 5-13). The median length of follow-up was 132 months (IQR: 68-186) from initial intervention and 12 months (IQR: 8-31) from amputation. Two patients (40%) who had undergone transhumeral amputation required revision of the amputation site for recurrent ulceration at 2 and 38 months. Of these, 1 patient underwent 3 transcatheter embolization procedures before revision and 1 underwent 1 embolization after revision. CONCLUSIONS: Although rare, successful amputation at the level of residual malformation can be performed in select patients with refractory complications of vascular malformations including intractable pain, bleeding, or nonhealing wounds. Specific preoperative and intraoperative measures may be critical to achieve satisfactory outcomes, and endovascular techniques continue to play a role after amputation.

Complexity of Aortic Arch Anatomy Affects the Outcomes of Transcarotid Artery Revascularization Versus Transfemoral Carotid Artery Stenting.

BACKGROUND: Transcarotid artery revascularization (TCAR) has been shown to have half the rates of transient ischemic attack (TIA), stroke, and death compared with transfemoral carotid artery stenting (TFCAS). Successful outcomes of TFCAS require careful patient selection. The aim of this study was to determine the outcomes of TFCAS versus TCAR in both simple (type I) and complex (type II and III) aortic arches. METHODS: A retrospective cohort study was performed comparing the outcomes of patients undergoing TFCAS versus TCAR with simple and complex aortic arches using the Vascular Quality Initiative registry from August 2011 to May 2019. The primary outcome was a composite of in-hospital TIA/stroke/death. RESULTS: About 6,108 carotid artery interventions were analyzed, including 3,536 (57.9%) patients with type I, 2,013 (33.0%) with type II, and 559 (9.2%) with type III aortic arch. In 3,535 patients with a simple arch, 1,917 underwent TFCAS and 1,619 had TCAR. Mean age was 70.6 (±9.5) years, and 2,382 (67.4%) patients were males. The primary outcome of postoperative TIA/stroke/death was seen significantly less frequently in those undergoing TCAR compared with TFCAS in simple arches (odds ratio [OR], 0.63; 95% confidence interval [95% CI], 0.43-0.94; P = 0.0236). Although the individual outcome of death occurred less often in TCAR (P = 0.0025), there was no difference in the occurrence of in-hospital stroke (P = 0.8836) or TIA (P = 0.4608). On multivariable analysis, TCAR was associated with improved outcomes (P = 0.0062). A worse outcome was associated with increasing age (P < 0.001), a prior stroke (P < 0.0001), and increasing number of stents (P = 0.0483). In 2,572 patients with a complex arch, 1,416 underwent TFCAS and 1,156 had TCAR. Mean age was 73.0 (±9.1) years, and 1,655 (64.4%) were males. In complex arch anatomy, the primary outcome of in-hospital TIA/stroke/death was seen significantly less frequently in TCAR compared with TFCAS (OR, 0.49; 95% CI, 0.31-0.77; P = 0.0022). Again noted was a significant difference in death, with better outcomes in TCAR (P = 0.0133). Although the occurrence of in-hospital TIA was no different between the 2 approaches (P = 0.6158), there were significantly fewer strokes in those treated with TCAR (P = 0.0132). TCAR (P = 0.0146) was associated with improved outcomes. A worse outcome was seen with advancing age (P = 0.0003), prior strokes (P = 0.01), and a left-sided lesion (P = 0.0176). CONCLUSIONS: TCAR has improved outcomes of TIA/stroke/death compared with TFCAS in both simple and complex aortic arch anatomy. In simple aortic arches, there is no difference in neurologic outcomes between both approaches. In complex arch anatomy, TCAR has fewer strokes.

Cross-clamp location affects short-term survival in patients undergoing open abdominal aortic aneurysm repair.

INTRODUCTION: Open abdominal aortic aneurysm (oAAA) repair in the era of advanced endovascular aortic techniques is used in challenging anatomy. The impact of the location of the proximal aortic cross-clamp (suprarenal [SR] vs infrarenal [IR]) on outcomes remains to be determined. The aim of this study was to analyze the effect of proximal aortic cross-clamp location on short-term and overall survival after oAAA repair in a contemporary series. METHODS: A retrospective cohort study was performed comparing the outcomes of patients undergoing oAAA repair with SR and IR aortic cross-clamping using the Vascular Quality Initiative registry from January 2003 to September 2018. Our primary end point was short-term mortality. RESULTS: There were 7601 patients who underwent oAAA repair. Their mean age was 69.3 ± 8.5 years and 5555 patients (73.1%) were male. The aortic cross-clamp location was IR in 4044 patients (53.2%). The SR group had increased maximum AAA diameter (58 mm vs 56 mm; P < .0001), hypertension (85.5% vs 82.0%; P < .0001), preoperative creatinine (1.11 vs 1.08; P = .001), and were more likely to be in American Society of Anesthesiologists class IV (37.4% vs 30.6%; P < .0001). Postoperative renal failure occurred significantly more often in the SR group (24.4 vs 11.4%; P < .0001). Short-term mortality was 2.7% in the IR group and 4.7% in the SR group (P < .0001). Kaplan-Meier survival estimates were 93.7% and 83.8% in the IR group and 90.9% and 81.2% in the SR group at 1 and 5 years, respectively (P = .007). Multivariable analysis demonstrated that SR cross-clamping was significantly associated with short-term mortality (hazard ratio, 1.38; 95% confidence interval, 1.07-1.78; P = .01); however, it did not affect overall survival (hazard ratio, 1.13; 95% confidence interval, 1.00-1.28; P = .06). CONCLUSIONS: A SR cross-clamp location is associated with an increased short-term mortality in patients undergoing oAAA repair. Overall survival is not affected by a SR cross-clamp location.