Massive Localized Lymphedema: Wound Reconstruction With Extracellular Matrix

Massive localized lymphedema (MLL) is a benign overgrowth of lymphoid tissue first described in 1998 by pathologists Weiss and Farshid in Ann Arbor, Michigan.¹ Since then, it has become a topic of considerable interest given its predilection for obese patients and the rising obesity epidemic.^2-4 While the precise pathophysiology of MLL remains undefined, the development is likely related to secondary lymphedema.^2,5 The mechanical forces from the excessive tissue mass may result in obstruction, causing lymph stasis.^6,7 Lymphatic hypertension then promotes extravasation of fluid and protein into the interstitium, creating a local inflammatory response which perpetuates the cycle of impaired drainage.^6,7

Clinically, MLL lesions initially appear as large, pendulous, painless masses that have been present for months to years.^1,2,4,8,9 Weighing upwards of 50 pounds, these lesions frequently impair functional status.^1,2,4,10 In contrast to generalized lymphedema, MLL is often well circumscribed. It most commonly involves the lower limb; however, cases of the abdomen, scrotum, and vulva have been reported.^1,2,4 On physical examination MLL has a hyperkeratotic, peau d’orange appearance.^1,2,4 Ulceration, non-pitting edema, and superimposed cellulitis are common.^1,2,4 Imaging is particularly difficult given the large average body habitus, thus emphasizing the importance of a thorough physical examination.⁴ When imaging is conducted, computerized tomography or magnetic resonance imaging will demonstrate a subcutaneous edema with a reticular pattern within the lesion and characteristic lack of muscular involvement.^1,6,8,11

When the mass interferes with lifestyle, raises concerns for malignant conversion, or becomes infected, surgical care should be strongly considered.^8,11,12 After excision, primary closure is commonly attempted, and postoperative complications are not infrequent.^6,8 With the advent of advanced wound care technologies such as negative pressure wound therapy (NPWT) and biological matrices, the surgeon has acquired a new armamentarium in the effort to treat MLL. To this end, the authors report 2 cases of successful management of MLL with wide local excision, NPWT, and acellular biologic matrix.

Two patients presented to Crozer-Chester Medical Center (Chester, PA) with findings compatible with MLL. The senior surgeons on staff evaluated these patients and agreed that they would benefit from definitive treatment by operative excision. Written informed consent was obtained and patients were counseled on the role of extracellular matrix (ECM) use for wound reconstruction. Preoperative and postoperative pictures were taken, and the patients were followed closely throughout the wound healing process. The study was conducted in accordance with the Institutional Review Board at Crozer-Chester Medical Center. Operative details, wound reconstruction, and postoperative courses are discussed in detail. 

Case 1. A 58-year-old man with a history of hypertension, gout, hyperlipidemia, and morbid obesity (body mass index [BMI] 78) presented to the authors’ facility with complaints of a large left posterior thigh mass (Figure 1). The mass had been increasing in size and had developed foul-smelling drainage. On examination, the mass appeared brawny, erythematous, and edematous and was covered by an exudate (Figure 2). The patient was started on 1.5 g intravenous (IV) vancomycin twice daily and the decision was made to provide definitive treatment through surgical excision (Figure 3). A resultant soft-tissue defect of 57 cm x 45 cm remained and was treated initially with a NPWT dressing changed 3 times per week during the patient’s hospital stay (Figure 4). The patient returned to the operating room 6 days later, and sharp debridement with split-thickness skin grafting (STSG) was performed. The STSG was secured with netting, staples, and an Acticoat (Smith & Nephew, Fort Worth, TX) dressing and was again covered with a NPWT dressing. Multiple areas of graft loss were noted at the 2-week postoperative follow-up, and the patient required 2 further debridements during which MatriStem Multilayer Wound Matrix (ACell, Inc, Columbia, MD) and NPWT were applied (Figure 5). After 2 applications of the multilayer wound matrix, the wound achieved complete tertiary closure by 2 months post initial matrix application (Figure 6). 

Case 2. A 46-year-old man with a significant history of diabetes mellitus, hypertension, hyperlipidemia, and morbid obesity (BMI 75) presented to the authors’ facility with complaints of recurrent bouts of cellulitis involving a right posterior thigh mass. Clinically, the mass appeared consistent with MLL. The patient was started on 1.5 g IV vancomycin every 12 hours, and the decision was made to undergo definitive surgical excision. The mass was excised, and the wound initially was closed primarily after raising skin flaps. Within the first postoperative month, the patient suffered from incisional dehiscence and developed nonhealing wounds. He was subsequently taken back to the operating room on 2 separate occasions for sharp debridement and application of a multilayer wound matrix. Negative pressure wound therapy was initiated, and after 2 applications of the multilayer wound matrix the patient proceeded to heal his wound within 4 months’ time and without any further complications. 

Both patients in this series were male, middle-aged, and morbidly obese with BMIs exceeding 70. Both lesions were localized to the posterior thigh and had superinfection on presentation. In both cases, the lesions were treated by excision and the clinical diagnosis was confirmed histologically. In case 1, the patient’s wound was closed with STSG, while in case 2 the patient underwent attempted primary closure. Both options were met with postoperative complications requiring multiple return visits to the operating room. After application of a multilayer wound matrix, both patients were able to successfully achieve full wound closure within 2 to 4 months postoperatively and regain baseline functional status. 

While MLL is a relatively new disease,¹ its incidence will likely continue to increase considering the rising obesity epidemic.^8,11 Given the potential for MLL to degenerate into lymphangiosarcoma, clinical recognition and early treatment is paramount.^2,4,13 Evidence-based treatment recommendations are lacking; however, surgery appears to be the evolving standard of care for lesions that impair mobility, become superinfected, or appear clinically indistinguishable from a malignant process.^2,8,11,12,14 Nonoperative therapies, such as compression garments, are frequently unsuccessful, and the consequences of malignant degeneration can be devastating.⁴ Excision of MLL frequently results in a massive wound. The literature indicates that almost all of these lesions have been reconstructed using primary closure with or without flap creation and skin grafting.¹⁵ 

While primary closure appears to be the most prevalent form of MLL wound reconstruction, these incisions are frequently disrupted by infection and excessive lymphatic exudate.^1,8,16 Uncomplicated tension-free primary closure is often not possible given the tremendous defect left in the wake of these lesions. Edematous, draining tissues pose a particular threat to wound healing and skin graft take. Dehiscence is common, and mechanical complications are likely compounded by a concomitant local oxygen deficiency resulting from the impaired lymphatic drainage.^6,8 Wound care focuses on drainage, debridement of nonviable tissue, and infection control.^8,17 Long-term follow-up with these patients is essential given the protracted course of wound healing and the potential for recurrence and/or malignant degeneration.^2,12

Emerging literature on MLL management supports the use of NPWT to improve postoperative healing and control wound exudate.³ The use of NPWT has been steadily increasing since its conception, and indications for use have continued to broaden. The system constantly removes wound exudate, decreases bacterial accumulation, and enhances local blood flow by creating a microdeformation strain, which is thought to stimulate angiogenesis.^4,18-20 It has also been used for skin grafting and dermal matrix stabilization, which facilitates the neovascularization process.²⁰ 

Acellular dermal matrices (ADMs) and ECMs are frequently used in the field of wound reconstruction. They provide a scaffold that promotes cellular ingrowth and angiogenesis.^21,22 Several studies conducted over the past 15 years have shown that initial ADM grafting benefits from concomitant NPWT.^20-24 During the first stage of wound reconstruction, ADM grafts undergo incorporation and vascular ingrowth, which can be enhanced and even exaggerated using NPWT.^20-24 The ECM selected for this study was a multilayer wound matrix, derived from porcine urinary bladder and has been shown to promote healing in especially ischemic wound beds.²⁵ This multilayer wound matrix is composed of a basement membrane layer which protects the underlying tissues while the lamina propria layer promotes wound bed incorporation.²⁵  

This study reports 2 additional cases of MLL that fit the patient profile established in the literature. In addition to augmenting the clinical acumen on this newly emerging disease, this study presents a novel approach towards wound reconstruction. Using a combination of multilayer wound matrix to improve the wound bed vascularization and NPWT to control exudate and stabilize the multilayer wound matrix system, both patients achieved complete wound closure and return to baseline function.

Evidence-based recommendations regarding the clinical management of MLL are lacking, and reconstructive techniques for the postoperative massive wounds continue to evolve. In the cases presented herein, the authors employed a number of modalities to ultimately close difficult MLL sites that would have been inappropriate for primary closure. The authors’ approach yielded satisfactory delayed wound closure and return to baseline function upon completion of the surgical wound course. These methods should be considered in the developing reconstructive paradigm for MLL. 

From the Department of General Surgery, Drexel University College of Medicine, Philadelphia, PA; Nathan Speare Regional Burn Treatment Center, Crozer-Chester Medical Center, Chester, PA; and Department of Surgery, Crozer-Chester Medical Center

Address correspondence to:
Matthew E. Pontell, MD
Department of Surgery
Drexel University College of Medicine
245 N 15th Street
Philadelphia, PA 19146
MatthewPontellMD@gmail.com

^*Both authors contributed equally as first author.

Disclosure: The authors disclose no financial or other conflicts of interest.