Michael V. DeFazio

Outcomes after combined radical resection and targeted biologic therapy for the management of recalcitrant hidradenitis suppurativa.

BackgroundTrials demonstrating the efficacy of biologic therapy for moderate to severe hidradenitis suppurativa (HS) have inspired new multidisciplinary treatment strategies. We present our experience with combined biologic and surgical therapy for recalcitrant HS. MethodsBetween 2011 and 2014, 21 patients (57 cases) with Hurley Stage III HS underwent radical resection with delayed primary closure alone, or in combination with adjuvant biologic therapy. Demographic data, treatment regimen, outcomes, and complications were retrospectively reviewed for all cases. ResultsEleven patients underwent combined surgical and biologic therapy, whereas radical resection alone was performed in 10 patients. The average soft tissue deficit, before closure, for the combined and surgery-only patients was 56 cm2 and 48.5 cm2, respectively (P = 0.66). Biologic agents including infliximab (n = 8) and ustekinumab (n = 3) were initiated 2 to 3 weeks after closure and were continued for an average of 10.5 months. Recurrence was noted in 19% (4/29) and 38.5% (10/26) of previously treated sites for combined and surgery-only patients (P < 0.01). For the combined cohort, the disease-free interval was approximately 1 year longer on average (P < 0.001); however, this difference was reduced to 4.5 months when considering time to recurrence after cessation of biologic therapy (P = 0.09). New disease developed in 18% (2/11) and 50% (5/10) of combined and surgery-only patients, respectively (P < 001). No adverse events were noted among patients who received biologic therapy. ConclusionsLower rates of recurrence and disease progression, as well as a longer disease-free interval may be achieved with the use of adjuvant biologic therapy after radical resection for recalcitrant HS.

What Heals Hidradenitis Suppurativa: Surgery, Immunosuppression, or Both?

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the skin that is a significant source of impaired quality-of-life for affected individuals. For patients with refractory disease, radical resection of all involved tissue may offer the only potential option for a cure. However, high rates of recurrence after wide local excision have led investigators to seek alternative therapeutic combinations. We present our experience and a literature review. Methods: A comprehensive literature review (1982–2016) was performed on PubMed and Ovid MEDLINE, using key words relevant to HS. Results: A total of 1842 publications were identified, and 15 publications were included in this review on: surgical intervention (n = 10), biologic therapy (n = 4), and joint surgical and biologic treatment (n = 1) of HS. The treatment type, level of evidence, and key findings are assessed and discussed. Conclusions: Recent trials demonstrating the safety and efficacy of biologic therapy alone in patients with moderate-to-severe HS have spurred questions regarding the potential utility of these agents as adjuncts to more traditional surgical interventions. The results of the literature search advocate a role for radical resection and biologic therapy in the setting of moderate-to-severe disease. Preliminary results from limited case series suggest a potential role for combined management in the setting of advanced, recalcitrant disease.

Current Concepts in Debridement: Science and Strategies

Background: The establishment of a healthy wound bed through adequate debridement of infected, senescent, and/or devitalized tissue is central to the progression of normal wound healing. Although a variety of surgical and nonsurgical strategies have been proposed, none have proven completely effective in all settings. This review focuses on the principles and techniques of modern debridement practices employed in the management of complex wounds. Methods: A comprehensive review of the PubMed/Medline and Ovid databases was performed to identify basic science and clinical studies using key words most relevant to biofilm, debridement, and wound healing. English language articles that were peer reviewed and that met the standard of evidence-based medicine were included. Level of evidence for various debridement approaches was rated utilizing the American Society of Plastic Surgeons Rating Levels of Evidence and Grading Recommendations. Results: The value of both operative and nonoperative debridement techniques, their indications, and limitations are described. With an emphasis placed on surgical debridement, this review highlights technical adjuncts that can be used to optimize wound bed preparation, including preoperative topical staining of the wound, as well as the use of color-guided endpoints to prevent removal of excess healthy tissue. The indications for using temporizing measures for wound control such as negative pressure wound therapy with and without installation are also discussed. Conclusion: Optimal management requires a multimodal approach that centers around operative debridement and incorporates the use of adjunctive measures to facilitate the removal of infected tissue, biofilm, and/or senescent cells that impede the progression of normal wound healing.

Management of advanced plexiform neurofibromatosis of the foot presenting with skeletal deformation and intractable pain: an indication for proximal amputation.

Plexiform neurofibromas of the foot are rare and often present with significant pain, deformity, and functional impairment secondary to their locally invasive behavior. While treatment has traditionally focused on attempts at radical resection, a lack of consensus among surgeons has hindered the establishment of a well-defined algorithm to guide the management of these highly co-morbid peripheral nerve sheath tumors. We present the case of an advanced plexiform neurofibroma of the right foot in a 24-year-old male with neurofibromatosis type 1. The patient presented following accelerated tumor growth with extensive osseous erosion, intractable pain, and progressive ankle instability that limited his capacity to ambulate and wear shoes. A modified transtibial amputation with a vascularized fibular bone graft (Ertl procedure) was performed without complication. Following graduated rehabilitation, postoperatively, the patient regained functional independence and was able to ambulate without pain in a customized prosthesis after 3 months. Plexiform neurofibromas of the foot present a complex challenge for foot and ankle surgeons. On the basis of our experience and previously reported cases, we advocate for amputation over aggressive attempts at advanced limb salvage for patients with extensive skeletal destruction, joint instability, and/or intractable pain caused by tumor mass effect.

Free Tissue Transfer after Targeted Endovascular Reperfusion for Complex Lower Extremity Reconstruction: Setting the Stage for Success in the Presence of Mutlivessel Disease

Recent advances in both microsurgical and endovascular techniques have redefined our approach to complex lower extremity reconstruction in high-risk patients with multivessel disease. When used as an adjunct to free tissue transfer, targeted endovascular therapy has been suggested to optimize recipient-site hemodynamics and promote flap survival in regions of compromised distal blood flow. With a paucity of published outcomes to support this approach, however, the perceived benefits derived from adjunctive endovascular therapy remain largely anecdotal. In this report, we present the outcomes and complications for 3 patients in whom preoperative endovascular therapy was used to improve both direct and collateral distal perfusion and recipient-vessel caliber. Flap survival in this series was 100%, and all patients remained ambulatory at a mean final follow-up of 24.7 months. Minor flap necrosis, necessitating debridement and skin grafting, was seen in one patient, who ultimately required a contralateral below-knee amputation secondary to nonsalvageable arterial disease. All patients were satisfied with respect to pain, function, and cosmetic appearance. On the basis of our experience and previously reported cases, we advocate a role for prereconstructive endovascular therapy as part of a multidisciplinary approach to functional limb salvage in patients with advanced arterial disease.

Functional Reconstruction of a Combined Tendocutaneous Defect of the Achilles Using a Segmental Rectus Femoris Myofascial Construct: A Viable Alternative

The composite anterolateral thigh flap with vascularized fascia lata has emerged as a workhorse at our institution for complex Achilles defects requiring both tendon and soft tissue reconstruction. Safe elevation of this flap, however, is occasionally challenged by absent or inadequate perforators supplying the anterolateral thigh. When discovered intraoperatively, alternative options derived from the same vascular network can be pursued. We present the case of a 74-year-old male who underwent composite Achilles defect reconstruction using a segmental rectus femoris myofascial free flap. Following graduated rehabilitation, postoperatively, the patient resumed full activity and was able to ambulate on his tip-toes. At 1-year follow-up, active total range of motion of the reconstructed ankle exceeded 85% of the unaffected side, and donor site morbidity was negligible. American Orthopaedic Foot and Ankle Society and Short Form-36 scores improved by 78.8% and 28.8%, respectively, compared to preoperative baseline assessments. Based on our findings, we advocate for use of the combined rectus femoris myofascial free flap as a rescue option for reconstructing composite Achilles tendon/posterior leg defects in the setting of inadequate anterolateral thigh perforators. To our knowledge, this is the first report to describe use of this flap for such an indication.

Lower Extremity Flap Salvage in Thrombophilic Patients: Managing Expectations in the Setting of Microvascular Thrombosis

Background Undiagnosed thrombophilia is a risk factor for flap failure; however, its prevalence in patients undergoing microsurgical reconstruction is unknown. We present our experience with free tissue transfer (FTT) in a high-risk population of lower extremity patients with documented thrombophilia, identified through preoperative screening. Methods Between January 2012 and April 2014, 41 patients underwent 43 free flaps for nontraumatic, lower extremity reconstruction by a single surgeon. Patients were preoperatively screened for thrombophilia using historical information and standardized laboratory testing. Demographic data, perioperative management, outcomes, and salvage rates for thrombophilic and nonthrombophilic cohorts were compared. Results Routine preoperative screening identified 52 thrombophilic traits among 25 patients in this series (61%). The most common traits were the plasminogen activator inhibitor-1 4G/5G variant (n = 12) and the methylenetetrahydrofolate reductase A1298C (n = 10) and C677T (n = 9) polymorphisms. While success rates were similar between thrombophilic and nonthrombophilic patients (84 vs. 94%; p = 0.15), thrombotic complications (25 vs. 14%; p = 0.09) and flap failure following postoperative thrombosis (100 vs. 33%; p = 0.05) appeared to be more common in patients with thrombophilia. On average, microvascular complications manifested later in the setting of thrombophilia (mean 4.8 days vs. 18 hours; p = 0.20) and were associated with a worse overall prognosis (salvage rate, 0 vs. 67%; p = 0.05). Conclusions Despite high success rates, thrombophilia appears to increase the risk of nonsalvageability following lower extremity FTT. This information should be used to help counsel patients regarding the risks and benefits of microsurgical reconstruction, as salvage rates following postoperative thrombotic events approach 0% in the presence of thrombophilia.

Pedicle-to-Perforator Bypass Using Supermicrosurgical Technique for Deep Inferior Epigastric Artery Perforator Flap Salvage

1 Sir: A perforator selection is critical to successful deep inferior epigastric artery perforator flap harvest. Although the optimal number of perforators to be included is often debated, commitment to a single, dominant perforator simplifies dissection, reduces operative time, and minimizes donor-site morbidity when compared with multi-perforator flap designs.1 A potential drawback of this approach, however, relates to inadvertent perforator injury, which has a reported incidence as high as 4.3% and bears devastating prognostic implications.2 When discovered in-situ, conversion to a muscle-sparing alternative may facilitate flap salvage. However, the majority (71%) of these injuries are attributed to perforator mishandling (i.e., traction-induced) during flap transfer/ inset, rather than technical error of dissection, itself.2 As such, perforator-level injuries often go unnoticed until arterial insufficiency and/or venous congestion become evident following microvascular anastomosis. When this scenario is encountered intraoperatively, emergent microsurgical intervention offers the only reliable solution to avoid catastrophic flap loss. To this end, strategies that incorporate the superficial inferior epigastric artery/vein, with or without vein grafting and/or pedicle turbocharging, have been proposed to redirect flap inflow/outflow, respectively.3,4 These techniques, however, require preemptive planning and preservation of viable superficial inferior epigastric artery/vein, which are present in fewer than 30% of cases.5 In situations where the superficial system is inadequate/unavailable, segmental resection and anastomosis of the injured perforator to itself may aid in the reestablishment of nutritive perfusion.2 Although high success rates (83%) have been reported with this approach, perforator-to-perforator anastomosis in the setting of prior trauma can be technically challenging and increases the likelihood of thrombotic complications.2 A safer alternative to restore microcirculation involves interperforator bypass between previously ligated, nontraumatized perforators within the flap and preserved pedicle side branches (Fig. 1). After ligation/ division of the injured perforator, the undersurface of the flap and pedicle are each investigated to identify suitable perforator remnants. Donor/recipient perforators are preferentially selected on the basis of vessel caliber (0.3–0.5 mm diameter), length (4–6 mm), and absence of prior trauma (i.e., cauterization). Perforator dissection/anastomosis are performed under microscopic guidance utilizing meticulous supermicrosurgical technique with interrupted 10-0 nylon sutures (Fig. 2). After anastomotic patency is confirmed, the pedicle is sutured to the flap to avoid traction and/or accidental avulsion during inset. The advent of supermicrosurgical technique has expanded the indications and options available for salvage of perforator-level injuries. Successful pedicle-to-perforator bypass, as described herein, is dependent on the exclusion of injured vascular segments that pose unjustified thrombogenic risk. Preservation of perforator length during primary flap/pedicle dissection and use of hemoclip ligation optimize the quality/quantity of perforators available for anastomosis.3,4 In many cases, intraflap dissection and donor spatulation are required to lengthen the vascular cuff and improve size concordance between donor perforators and recipient pedicle side branches.5 It is our preference to incorporate perforators with accompanying venae comitans (VC), which simplifies the orientation of the arterial/venous pedicles supplying the flap. In the absence of reliable VC, the recipient venous pedicle can be separated along its length from the artery and transferred independently to an adjacent donor VC or the superficial inferior epigastric vein to facilitate flap outflow. Although described in the context of single-perforator deep inferiPedicle-to-Perforator Bypass Using Supermicrosurgical Technique for Deep Inferior Epigastric Artery Perforator Flap Salvage

Systematic Review and Comparative Meta-Analysis of Outcomes Following Pedicled Muscle versus Fasciocutaneous Flap Coverage for Complex Periprosthetic Wounds in Patients with Total Knee Arthroplasty

Background In cases of total knee arthroplasty (TKA) threatened by potential hardware exposure, flap-based reconstruction is indicated to provide durable coverage. Historically, muscle flaps were favored as they provide vascular tissue to an infected wound bed. However, data comparing the performance of muscle versus fasciocutaneous flaps are limited and reflect a lack of consensus regarding the optimal management of these wounds. The aim of this study was to compare the outcomes of muscle versus fasciocutaneous flaps following the salvage of compromised TKA. Methods A systematic search and meta-analysis were performed to identify patients with TKA who underwent either pedicled muscle or fasciocutaneous flap coverage of periprosthetic knee defects. Studies evaluating implant/limb salvage rates, ambulatory function, complications, and donor-site morbidity were included in the comparative analysis. Results A total of 18 articles, corresponding to 172 flaps (119 muscle flaps and 53 fasciocutaneous flaps) were reviewed. Rates of implant salvage (88.8% vs. 90.1%, P=0.05) and limb salvage (89.8% vs. 100%, P=0.14) were comparable in each cohort. While overall complication rates were similar (47.3% vs. 44%, P=0.78), the rates of persistent infection (16.4% vs. 0%, P=0.14) and recurrent infection (9.1% vs. 4%, P=0.94) tended to be higher in the muscle flap cohort. Notably, functional outcomes and ambulation rates were sparingly reported. Conclusions Rates of limb and prosthetic salvage were comparable following muscle or fasciocutaneous flap coverage of compromised TKA. The functional morbidity associated with muscle flap harvest, however, may support the use of fasciocutaneous flaps for coverage of these defects, particularly in young patients and/or high-performance athletes.

Giant Congenital Sclerosing Blue Nevus of the Scalp Presenting with Rapidly Disseminated Fatal Metastases in a Pediatric Patient.

1 Michael V. DeFazio, MD Jerry W. Chao, MD Albert K. Oh, MD Gary F. Rogers, MD Division of Plastic and Reconstructive Surgery Children’s National Medical Center Wash. Sir: G congenital blue nevi (GCBN) are rare dermal melanocytic proliferations that are distinguished by their large size, aggressive behavior, and propensity for malignant degeneration.1 To date, only 18 cases of GCBN involving the scalp have been reported. Among these, local invasion into the calvaria/dura (n = 10), malignant transformation (n = 6), and disseminated metastases (n = 5) were observed.2 Although the precise nature of malignant change is unclear, adults with histopathologically cellular GCBN are most commonly affected.2 We report the first case of a sclerosing GCBN of the scalp presenting with rapidly progressive fatal metastases in a toddler. A 17-month-old girl presented for evaluation of a 15 × 20-cm blue-gray plaque of the scalp that had grown commensurately with the child since birth. The patient’s history was notable for posterior encephalocele repair during infancy, resulting in an occipital bone defect and chronic/stable ulceration at the vertex. Initial magnetic resonance imaging demonstrated a noninvasive parietooccipital mass with an underlying 12-cm2 calvarial defect. Multiple incisional biopsies from ulcerated and nonulcerated regions revealed histopathologic features of sclerosing GCBN with extensive dermal/subcutaneous infiltration (ie, spindle-shaped melanocytes/dense collagenous stroma). Given these benign findings, conservative observation/local wound care was pursued in the hopes that dural-induced calvarial regeneration would improve the safety of tumor resection from the underlying scarred dura. However, the patient presented 7 months later with rapidly enlarging subcutaneous nodules, necrosis, and new-onset pain, vomiting, and ataxia (Fig. 1). Repeated magnetic resonance imaging demonstrated interval development of 2 sublesional masses with intracranial extension and calvarial/dural invasion (Fig. 2). A histopathologic diagnosis of melanoma (ie, atypical epithelioid melanocytes, large/pleomorphic nuclei, necrosis, numerous mitoses) arising within the areas reminiscent of sclerotic blue nevus was confirmed. Immunostains for BRAF V600 were negative; however, homozygous deletion of the 9p21 locus (CDKN2A/P16 tumor suppressor) was identified in 40% of cells analyzed by fluorescent in situ hybridization. Total body positron emission tomography revealed disseminated visceral metastases. The patient subsequently died 3 weeks later from widespread metastatic disease. This case presents several unique findings that deviate from previously reported trends. Morphologically, the majority of GCBN display cellular histopathologic features, which correlate well with their proclivity for invasive growth and malignant degeneration.1,2 In contrast, sclerosing blue nevi are predominantly benign, solitary, well-circumscribed nodules. Focal malignant melanoma arising within this subtype has been reported only once; however,