Post-closure With MANTA Fluoroscopic DOT Technique for Emergent Percutaneous Mechanical Circulatory Support and 'Bail-out' for Large Bore Arterial Hemostasis.

BACKGROUND: Large bore arterial hemostasis for emergent MCS initiation in cardiogenic shock and during failures of suture mediated vascular closure devices (VCD) necessitates dry/post-closure technique for successful closure. Use of the alternative MANTA fluoroscopic DOT technique, without depth finder, as post closure and as 'bail-out' in this high-risk patient population is described. METHODS: All patients who underwent emergent percutaneous transfemoral MCS initiation, without use of upfront sutured-mediated pro-glide VCDs, and procedures where proglide-perclose technique (PPT) failed to achieve hemostasis were post-closed with the alternative MANTA fluoroscopic DOT technique (without depth finder) as primary method or as 'bail-out'. Patient related factors, cardiovascular co-morbidities, clinical indication, distribution of 14F versus 18F MANTA, and types of procedures obtained. Primary outcomes of access site related acute flow-limiting limb ischemia or bleeding requiring intervention analyzed. RESULTS: 27 patients met inclusion criteria; mean age 64 years, majority male 19 (70 %), more than half obese (56 %) with mean BMI 31.06 kg/m2. 22 (81 %) had emergent MCS initiation and 5 (19 %) PPT hemostasis failures. Types of percutaneous MCS support included; 11 (44 %) Impella CP, 2 (7 %) 15F arterial ECMO, 6 (22 %) 17F arterial ECMO, 4 (15 %) 19F ECMO. All achieved hemostasis utilizing alternative MANTA fluoroscopic DOT technique without vascular complications of bleeding or acute ischemic limb. CONCLUSION: The alternative MANTA fluoroscopic DOT technique (without depth finder) can be successfully applied as post-closure for emergent MCS support delayed hemostasis and as bail-out for per-close suture mediated VCD failures for large bore arterial hemostasis.

Alternative Application Technique for the MANTA Vascular Closure Device for Percutaneous Large-Bore Arterial Access Closure: The Fluoroscopic DOT Technique.

BACKGROUND: Vascular complications are a cause of increased morbidity and mortality when performing percutaneous procedures requiring large-bore arterial access. MANTA vascular closure device (VCD) is currently the only large-bore VCD using an intraluminal foot plate and an extraluminal collagen plug. The traditional depth locator approach might be compromised in; emergent cases without the required measurements, cases of hematoma formation, or other patient, procedure, or operator-specific variables. Furthermore, this technique can be used for postclosure in cases without the required measurement of depth. We describe vascular outcomes using fluoroscopy (fluoroscopic DOT technique) rather than traditional depth locator approach for vascular closure with the MANTA VCD. METHODS: Fifty patients who underwent transcatheter aortic valve implantation (TAVI) were retrospectively analyzed using fluoroscopic DOT technique with 18F MANTA VCD between May and August 2021. All patients >18 years of age who qualified for transfemoral TAVI were included. Access was obtained with ultrasound guidance with vessel diameter of at least > 6 mm and free from anterior vessel wall calcification. Patient related factors and primary outcomes of access site bleeding and acute flow-limited limb ischemia requiring intervention were prospectively analyzed. Furthermore, 1 patient who failed hemostasis with suture-mediated VCD had successful hemostasis with fluoroscopic DOT technique as dry postclosure after balloon aortic valvuloplasty. RESULTS: In total, 50 patients were analyzed with a mean age of 81 years and majority were male (56%). Majority had comorbidities of hypertension (88%) and hyperlipidemia (94%), 24% had peripheral arterial disease, 38% coronary artery disease, and 58% were former smokers. Importantly, 40% were obese with an average body mass index (BMI) of 29 kg/m2. There were no bleeding or ischemic limb complications post MANTA VCD deployment using the fluoroscopic DOT technique. Furthermore, none of the patients required peripheral intervention from index procedure to 1 month post verified during their 1-month post TAVR follow-up. CONCLUSION: Fluoroscopic DOT technique using the MANTA VCD is highly reproducible and allows hemostasis in a predictable manner for procedures requiring large-bore arterial access in the absence of MANTA depth measurement.

Feasibility and outcomes with subclavian vein access for crescent jugular dual lumen catheter for venovenous extracorporeal membrane oxygenation in COVID-19 related acute respiratory distress syndrome.

INTRODUCTION: Femoral-femoral Veno-Venous ExtraCorporeal Life Support (V-V ECLS) has been associated with higher infections rates, vascular site bleeding complications, and restricted patient mobility. Jugular or bicaval dual lumen V-V ECLS conceptually overcomes some of these adverse factors, but experience has shown that jugular vein cannulation still limits mobility and has increased bleeding complications. Technique and outcomes of subclavian vein single-cannulation with Crescent jugular dual-lumen V-V ECLS is described. METHOD: five patients with COVID-19 related acute respiratory distress syndrome (ARDS) underwent right subclavian vein V-V ECLS placement with the Crescent 32 French jugular dual-lumen V-V ECLS catheter. A standardized percutaneous technique was developed that allowed efficient insertion without need for any specialized imaging (i.e. transesophageal echocardiogram) and outcomes assessed. RESULTS: Mean age of the five patients was 41.2 years, all obese with an average basal mass index of 45.2 kg/m2 and mean days to decannulation of 24.2 days. Outcomes discovered included; improved patient mobility allowing physical rehabilitation, no vascular access site related complications requiring surgery or endovascular intervention, and none had evidence of superior vena cava syndrome. One patient had subclavian/axillary vein thrombosis with resolution after 3 months of direct-acting oral anticoagulants, and one patient had blood cultures positive at day 37, nearing decannulation. CONCLUSION: Subclavian vein access for crescent jugular dual lumen V-V ECLS catheter appears to be safe and feasible with added benefits of decreased bleeding and increased mobility over jugular or femoral-femoral access site for long term V-V ECLS support in COVID-19 related ARDS patients.

Percutaneous transeptal mitral valve endocarditis debulking with AngioVac aspiration system.

Vacuum assisted aspiration with the AngioVac system has been well described for; right sided endocarditis, venous thrombus, lead related infection/thrombus aspiration and right sided cardiac mass evacuation. Percutaneous transeptal debulking with AngioVac for mitral valve endocarditis (MVE) in the inoperable or high surgical risk patient has not been well defined. A significant proportion of high/prohibitive surgical risk patients with left sided infective endocarditis (IE) are not offered valve surgery as patients in the acute active phase of IE have a high surgical mortality. Nonetheless, sequala of acute IE ie stroke, sepsis or hemodynamic instability in itself is associated with high morbidity and mortality without surgical treatment. A case report of an inoperable patient with methicillin sensitive staphylococcus aureus MVE who was offered MV vegetation debulking with the AngioVac Gen3 C 180 MV system is described. Preprocedural planning with attention to; optimal transeptal height puncture, use of sentinel cerebral protection device to decrease risk of procedure related cerebral embolism and venous extracorporeal membrane cannula, rather than arterial cannula for reinfusion, is described to avoid large bore arterial access related vascular complications. Further studies in a randomized manner are warranted to test these procedural techniques and determine outcomes of percutaneous aspiration of left sided IE with the AngioVac system in this highrisk inoperable cohort of patients.

3D Printing of Subclavian Artery: Utility for Preprocedural Planning and Correlation With Subclavian Artery Percutaneous Vascular Interventions.

BACKGROUND: Three-dimensional (3D) printing for subclavian artery (SA) percutaneous vascular interventions (PVI) may allow superior understanding of patient specific complex anatomy and aid with preprocedural planning. METHODS: Five patients with computed tomography angiography (CTA) of the neck who underwent SA PVI were queried retrospectively. 3D printing of aortic arch and great vessels was accomplished with 3D slicer software and painted with acrylic paint to highlight anatomic features. The aortic arch type and implications for preprocedural planning for SA interventions including complex chronic total occlusion (CTO) lesions were determined. Comparisons were made with SA angiograms and 3D-CTA. RESULTS: Of the 5 patients, type I (n = 2), type II (n = 1), and type III (n = 2) aortic arches were identified. Proximal and distal reference vessel size and total lesion length were determined using a digital millimeter caliper and correlated with intraprocedural balloons and stents. In 3D-printed models (3D-PMs) of patients with SA-CTO (n = 2), cap morphology (tapered vs blunt) and distal vessel filling were visualized, permitting optimal arterial access site selection for successful cap crossing. The vertebral arteries (VAs) were also 3D printed which further allowed the ability to delineate optimal stent deployment site (proximal or distal to VA), a common dilemma that is faced intraprocedurally. The 3D-PMs also allowed preprocedural precision in stent and balloon size and length, potentially leading to procedural efficiency and cost-effectiveness. CONCLUSION: 3D printing of aortic arch and great vessel anatomy for SA-PVI allows multiple procedure-related factors to be predicted in advance, translating to decrease in contrast volume, radiation time, procedure and fluoroscopic time, thereby improving procedure and cost efficiency.

3D Printing for Mesenteric Artery Endovascular Interventions: Feasibility and Utility for Preprocedural Planning and Angiographic Correlation.

BACKGROUND: Three-dimensional (3D) printing of mesenteric artery (MA) anatomy preprocedurally for endovascular interventions can allow strategic preprocedure planning and improve procedure-related clinical outcomes. METHODS: Three patients with computed tomography angiography (CTA) of the abdomen and pelvis who subsequently underwent MA interventions were 3D printed retrospectively, and 2 patients with symptoms and severe MA stenosis on CTA, who had not undergone intervention, were 3D printed for procedure-related planning and anatomy-specific implications. The 3D-printed models (3D-PMs) were painted with acrylic paint to highlight anatomy. Reference vessel size, lesion length (LL), and renal artery (RA) to MA distance were determined using a digital millimeter caliper. RESULTS: Each of the 5 patients with variable anatomy, including an MA chronic total occlusion (CTO), were successfully 3D printed. A digital caliper allowed determination of vessel size, LL, and RA to MA distance, which were then compared with intraprocedural MA angiograms and intravascular imaging when available. Further complex anatomies, such as intraprocedural navigation in the setting of prior abdominal aortic endograft and CTO assessment with relevance to cap morphology, small branch arteries, and collateral flow, were also successfully 3D printed. CONCLUSION: Preprocedural 3D printing of MA anatomy for interventions can theoretically lead to decreases in contrast use, radiation dose, and fluoroscopic and procedural times, as well as enhance comprehension of complex patient-specific anatomy.

Safety and outcomes of combined carbon dioxide angiography and OCT-guided femoro-popliteal chronic total occlusion crossing and directional atherectomy in patients with chronic kidney disease.

BACKGROUND: Carbon dioxide angiography with addition of optical coherence tomography imaging may improve procedural success and clinical outcomes in patients with peripheral artery disease and chronic kidney disease. METHODS: Single-center, retrospective analysis of patients with chronic kidney disease who underwent carbon dioxide angiography and optical coherence tomography-guided chronic total occlusion crossing and/or optical coherence tomography-guided directional atherectomy was performed. Patient and procedure-related characteristics, along with peri- and one-year post-procedural major adverse events, were analyzed. RESULTS: A total of 18 vessels in 11 patients, with mean age 70 years were treated. All had co-morbidities such as hypertension, hyperlipidemia, had history or were current smokers with baseline peripheral artery disease. Majority were diabetic with coronary disease (82%); 55% baseline chronic kidney disease IV, 55% Rutherford class III and 45% class IV. Contrast was used in only two patients. Mean total fluoroscopy time and radiation dose was 24.1 min and 249.2 mGY, respectively. Half of the lesions were femoro-popliteal chronic total occlusions, and Ocelot catheter was used to cross seven of nine chronic total occlusions and was successful in six. Adjunctive optical coherence tomography-guided directional atherectomy was performed in 8 of 11 patients. Only two adverse events occurred: one clinically significant event of slow-flow intra-procedurally and one target limb revascularization within one year of index procedure in a vessel different than prior treated. Optical coherence tomography imaging in both chronic total occlusion-crossing and atherectomy resulted in 10-min mean fluoroscopy reduction time and 32 mGY reduction in radiation dose. CONCLUSION: Carbon dioxide angiography with the addition of optical coherence tomography imaging for chronic total occlusion crossing and/or optical coherence tomography-guided directional atherectomy reduced the need for contrast agents, total fluoroscopy time, and radiation exposure in patients with peripheral artery disease and baseline chronic kidney disease.

Robotic Percutaneous Coronary Intervention During COVID-19 Pandemic: Outcomes and Cost Effectiveness With Procedural Distancing.

BACKGROUND: The COVID-19 pandemic raised many questions, including the need to maintain distancing and the importance of full personal protection equipment (PPE) for healthcare workers. Robotic-assisted percutaneous coronary intervention (R-PCI) can be advantageous during a pandemic to facilitate procedural distancing for cath lab personnel and to decrease the cumulative amount of PPE employed. METHODS: All patients who underwent R-PCI during the early phase of the pandemic were evaluated at a single institution. Procedural characteristics, complexity of disease, and use of adjunctive imaging or physiology were queried. Mean R-PCI time, procedure and fluoroscopy times, radiation dose, and contrast volume were collected. Cost of PPE incurred with R-PCI was evaluated in comparison with traditional cases. Furthermore, procedural distancing from patients for operators was objectively quantified to compare with traditional cath lab cases. RESULTS: Thirteen patients were treated using R-PCI. Radial access site was utilized in 54% and the left circumflex artery was treated most frequently (in 50% of cases). The complexity of cases performed was illustrated by revascularization of chronic total occlusions in 2 patients (14%) as well as adjunctive intravascular ultrasound or instantaneous wave-free ratio in 4 patients (29%). Mean R-PCI time was 45.38 minutes, manual time was 25.66 minutes, and total procedure time was 71.08 minutes. Average fluoroscopy time was 18.27 minutes, contrast volume was 143.85 mL, and radiation dose was 747.15 mGy. R-PCI was more cost effective, with total average PPE cost of $330.71 in comparison with $496.08 for traditional PCI (absolute difference, $165.36). Furthermore, R-PCI demonstrated substantial procedural distancing of operators from patients (8.14 feet for R-PCI vs 2.75 feet for traditional PCI; absolute difference, 5.39 feet). CONCLUSION: R-PCI could be a promising strategy during a pandemic by facilitating procedural distancing, minimizing staffing exposure risk, and decreasing PPE cost.

3D Printing of Renal Arteries for Endovascular Interventions: Feasibility, Utility, and Correlation With Renal Arteriograms.

BACKGROUND: Three-dimensional (3D) printing technology is increasingly being utilized for preprocedural planning of interventional procedures. However, utility of 3D models of obstructive and clinically relevant renal artery disease has not been evaluated and could potentially assist in preprocedural planning of renal artery endovascular interventions. METHODS: Five patients with computed tomography angiography (CTA) of abdomen and pelvis who also subsequently underwent renal artery interventions were 3D printed retrospectively. Standard 3D slicer software was used to segment out descending aorta, renal artery, and renal anatomy to create a computer aided image. The 3D-printed models (3D-PMs) were painted with acrylic paint to highlight anatomic features for comparison with renal arteriograms and 3D-CTA to aid in endovascular interventions. RESULTS: 3D-PMs were successfully produced in diverse renal artery pathology: atherosclerotic disease, fibromuscular dysplasia, in-stent restenosis, and bilateral renal artery stenosis. Renal artery ostium angulation and optimal axial guiding catheter engagement were elucidated. Additionally, reference vessel size and lesion length were measured using digital millimeter calipers. Renal arteriogram along with renal interventional devices utilized during each case were compared for size correlation, reproducibility, and clinical utility. CONCLUSION: Preprocedural 3D printing of renal artery anatomy requiring endovascular intervention could allow for better appreciation of renal anatomy and could serve as an adjunctive tool to minimize use of contrast, fluoroscopy, and procedure time.

Risk Prediction in Cardiogenic Shock: Current State of Knowledge, Challenges and Opportunities.

Cardiogenic shock (CS) is a condition associated with high mortality rates in which prognostication is uncertain for a variety of reasons, including its myriad causes, its rapidly evolving clinical course and the plethora of established and emerging therapies for the condition. A number of validated risk scores are available for CS prognostication; however, many of these are tedious to use, are designed for application in a variety of populations and fail to incorporate contemporary hemodynamic parameters and contemporary mechanical circulatory support interventions that can affect outcomes. It is important to separate patients with CS who may recover with conservative pharmacological therapies from those in who may require advanced therapies to survive; it is equally important to identify quickly those who will succumb despite any therapy. An ideal risk-prediction model would balance incorporation of key hemodynamic parameters while still allowing dynamic use in multiple scenarios, from aiding with early decision making to device weaning. Herein, we discuss currently available CS risk scores, perform a detailed analysis of the variables in each of these scores that are most predictive of CS outcomes and explore a framework for the development of novel risk scores that consider emerging therapies and paradigms for this challenging clinical entity.

3D Printing of Carotid Artery and Aortic Arch Anatomy: Implications for Preprocedural Planning and Carotid Stenting.

BACKGROUND: Carotid artery stenting (CAS) has been associated with increased periprocedural stroke in comparison with carotid endarterectomy (CEA). Three-dimensional (3D) printing of aortic arch and carotid artery may aid with preprocedural planning and adaptive learning, possibly reducing procedure-related complications. METHODS: Five CAS cases with available computed tomography angiography (CTA) were retrospectively evaluated and 3D-printed models (3D-PMs) were made. One additional case that was 3D printed preprocedurally provided prospective analysis. Standard 3D printing software was used to create a computer-aided image from CTA series that were 3D printed. The models were painted with acrylic paint to highlight anatomical features. The type of aortic arch, common carotid artery (CCA) to internal carotid artery (ICA) angle, and ICA distal landing zone for embolic protection device (EPD) were analyzed. In addition, stent and EPD sizing was determined preprocedurally for the prospective case. Comparisons of 3D-PM were made with 3D-CTA reconstruction and carotid angiography. RESULTS: Of 6 cases, 2 had type III and 4 had type I aortic arches. One case, a failed endovascular approach from femoral artery access site requiring reattempt via right brachial artery, had a CCA to ICA angle >60° and a tortuous innominate artery and distal ICA for EPD. The remaining 5 cases had straight distal landing zones for EPD and <60° CCA to ICA angles with successful first endovascular attempt. Additionally, vessel-specific stent and EPD sizing was appropriately chosen for the 1 prospective case. CONCLUSIONS: 3D-PM for CAS offers added value compared with CTA by providing improved perceptual and visual understanding of 3D anatomy.

Patient-Specific Coronary Artery Bypass Graft 3D Printing: Implications for Procedural Planning in Complex Percutaneous Coronary Interventions.

BACKGROUND: Three-dimensional (3D) printing technology has seen tremendous growth in augmenting didactics, research, and preprocedural planning with structural heart procedures. Limited investigative efforts have been made in other areas of the cardiovascular spectrum. 3D-printed models (PMs) of anatomically complex coronary artery bypass graft (CABG) patients from coronary computed tomography angiography (CCTA) have implications for adaptive learning and preprocedural planning. METHODS: Five patients with CCTA who underwent subsequent coronary angiography were 3D printed for retrospective comparisons. Standard slicer software was used to create a computer-aided image of the ascending aorta, native coronary arteries, bypass grafts, aortic arch, and great vessels and 3D printed using polylactic acid filament. The models were painted with acrylic paint to highlight anatomical features and comparison was made with coronary angiography and 3D-CTA images. RESULTS: All occluded vein grafts, left and right internal mammary artery (IMA) grafts, patent saphenous vein grafts, along with distal graft anastomotic sites, were accurately 3D printed. In cases with chronic total occlusions (CTOs), ambiguous ostial caps, mid or distal vessel chronic occlusions, and occlusions seen as CTOs on coronary angiography were 3D printed showing either distal vessel reconstitution via collaterals or complete arterial filling seen in a setting of calcification, microchannels, and collateral flow. Lastly, 3D printing of the aortic root and great vessels allowed for better appreciation of vessel tortuosity to aid in the cannulation of IMA grafts and optimizing engagement with diagnostic and guiding catheters. CONCLUSIONS: 3D printing of anatomically complex CABG patients has the potential to assist with preprocedural planning and operator understanding of complex coronary anatomy.

Antegrade and retrograde in-stent tibial artery chronic total occlusion recanalization with double kiss crush (DK crush) stenting of previous stent.

Below the knee (BTK) peripheral arterial disease often presents with critical limb ischemia (CLI) clinically with involvement of more than one tibial vessels. Drug eluting stent (DES) technology for treatment of BTK disease has shown promising long-term durable results; however, currently only coronary DESs are available for application in the United States. Although coronary bifurcation stenting techniques are backed by extensive data in literature, there is a scarcity of data for the treatment of tibial bifurcation disease. Bifurcation angles in the tibials are similar to those in the coronaries and therefore the same two stent bifurcation technique can be applied in BTK disease. Double Kiss crush (DK crush) stenting has superior outcomes when compared to provisional or culotte stenting in randomized coronary trials (based on Medina classification). We present a case of BTK CLI with tibial bifurcation chronic total occlusion treated with two stent DK crush technique using coronary DES.

Recanalization of superior mesenteric artery chronic total occlusion using hybrid algorithm and dissection reentry device.

Chronic total occlusion (CTO) of mesenteric arteries with associated chronic mesenteric ischemia (CMI) is associated with high morbidity and mortality. Endovascular intervention has been associated with high technical success with high rates of freedom from symptoms and long-term patency. However, to achieve high procedural success, use of optimal vascular access and expertise in CTO hybrid algorithm including advanced dissection reentry strategies are essential. We present a case of CMI from severe celiac artery (CA) stenosis and CTO of superior mesenteric artery (SMA) and inferior mesenteric artery (IMA). After treatment of CA stenosis, we were unsuccessful in our first attempt at recanalization of SMA CTO. On second attempt, left brachial artery (BA) access was obtained and the hybrid algorithm along with use of Stingray Reentry balloon (Boston Scientific) for dissection reentry into true lumen was successful in recanalizing the SMA CTO with placement of balloon expandable covered stents (CS). To the best of our knowledge, this is the first case report utilizing Sting-ray Reentry balloon in the mesenteric arteries.

Effect of a low-dose interventional x-ray system on radiation exposure in the higher body surface area patient population.

Low-dose interventional x-ray systems have been shown to substantially reduce radiation dose in the pediatric and adult structural and interventional realm. We evaluated our single-center experience with Philips AlluraClarity software for all cardiovascular procedures; we also compared performance relative to patient body size. A total of 1155 patients were included. Data on dose area product (DAP) for radiation exposure, along with body surface area (BSA) and fluoroscopy time, were retrospectively collected for 467 patients before implementation of the Clarity system and for 688 patients after system implementation. DAP was then compared to BSA and fluoroscopy time. BSA was categorized into four quartiles to assess the relationship between radiation dose across small to large patient size populations. The mean BSA between two groups was similar (2.03 vs 2.02 m2, P = 0.48), with a 44.7% reduction in radiation dose with DAP indexed to BSA. A significant reduction in radiation dose was seen across all quartiles, with the highest reduction in the post-Clarity sample population with the largest BSA. Fluoroscopy time in the pre-Clarity period was lower than in the post-Clarity period (mean of 7.6 vs 10.2 min; P ≤ 0.001), with a total 57.7% radiation dose reduction with DAP indexed to fluoroscopy time (P ≤ 0.001). There was a 45.2% overall decrease in radiation dose with AlluraClarity (P ≤ 0.001). In conclusion, AlluraClarity significantly reduced overall radiation dose, irrespective of BSA. The largest reduction in radiation was seen in patients with the highest BSA, suggesting that obese patients derive the most benefit. To our knowledge, this is the first study to describe this relationship with BSA and AlluraClarity. The Clarity system also substantially reduced radiation dose despite longer fluoroscopy time.

Central Pseudo-Aneurysm Formation Following Arterial Closure with a StarClose SE Device: When a StarClose Doesn't Completely Close.

BACKGROUND: Vascular closure devices (VCDs) are frequently used for hemostasis with endovascular procedures by employing sutures or plug devices (using collagen or hydrogel) or through the use of a metal clip made of nickel and titanium, such as the StarClose SE device. In comparison to manual compression (MC), VCDs are associated with earlier time to discharge and ambulation, improved patient comfort, and better cost-effectiveness. CASE REPORT: A 77-year-old man with history of ischemic cardiomyopathy with non-ST segment elevation myocardial infarction (NSTEMI) underwent diagnostic cardiac catheterization with deployment of a StarClose SE vascular closure device for hemostasis. Upon repeat access 4 days later for coronary intervention, retrograde sheath angiography revealed a pseudo-aneurysm emanating from the center of the StarClose clip. CONCLUSIONS: A review of the literature shows VCDs to be non-inferior to MC, with an overall high success rate. Major and minor complications rates are comparable to those with MC, and pseudo-aneurysm is an infrequent complication.

Refractory postsurgical pyoderma gangrenosum in a patient with Beckwith Wiedemann syndrome: response to multimodal therapy.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that may be difficult to diagnose and treat. We presented a 41-year-old woman who required skin grafting following third-degree burns to her left breast. She suffered recurrent graft dehiscence and infections over many years, prompting elective bilateral reduction mammoplasty. She subsequently developed suture margin ulcerations unresponsive to topical therapies and antibiotics. Skin biopsies were non-specific, and a clinical diagnosis of PG was established. Although initially responsive to corticosteroids, wounds promptly recurred following steroid taper. She was treated unsuccessfully with various immunomodulatory agents and underwent elective bilateral mastectomy. Following a mastectomy, she developed progressive deep chest wall ulcerations. She failed numerous immunomodulatory treatments, surgical wound closure and negative pressure wound therapy. Ultimately, treatment with adalimumab, mycophenolate mofetil and prednisone, in addition to hyperbaric oxygen therapy facilitated progressive healing. Our case highlights the role of collaborative multimodal therapy for the treatment of refractory PG.