ORIGINAL ARTICLE. Francesco Marongiu, Federico Buggi, Matteo Mingozzi, Annalisa Curcio & Secondo Folli

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1 International Wound Journal ISSN ORIGINAL ARTICLE A rare case of primary necrotising fasciitis of the breast: combined use of hyperbaric oxygen and negative pressure wound therapy to conserve the breast. Review of literature Francesco Marongiu, Federico Buggi, Matteo Mingozzi, Annalisa Curcio & Secondo Folli Breast Unit, Morgagni-Pierantoni Hospital, Forlì, Italy Key words Breast conservation; Hyperbaric oxygen; Negative pressure wound therapy; Primary necrotising fasciitis of the breast Correspondence to F Marongiu, MD Breast Unit Morgagni-Pierantoni Hospital Via Carlo Forlanini 34 Forlì (FC) Italy marongiufra@gmail.com doi: /iwj Marongiu F, Buggi F, Mingozzi M, Curcio A, Folli S. A rare case of primary necrotising fasciitis of the breast: combined use of hyperbaric oxygen and negative pressure wound therapy to conserve the breast. Review of literature. Int Wound J 2017; 14: Abstract Necrotising fasciitis is a rare but potentially fatal disease. It is even more unusual as a primary disease of the breast. Surgical treatment is required in order to gain control over the spreading infection and mastectomy is reported to be the most common procedure. We report the first case of an otherwise healthy woman exhibiting a primary necrotising fasciitis of the breast, which was treated combining conservative surgery with hyperbaric oxygen (HO) and negative pressure wound therapy (NPWT). A 39-year-old woman presented to the emergency room with fever and swelling of her right breast. The physical examination showed oedema and erythema of the breast, with bluish blisters on the lower quadrant. Ultrasound and CT scans showed diffuse oedema of the entire right breast, with subdermal gas bubbles extending to the fascial planes. Few hours later the necrotic area extended regardless an IV antibiotic therapy; a selective debridement of all breast necrotic tissue was performed and repeated 7 days later. The HO was started immediately after the first surgery and repeated daily (2 8 Bar, 120 min) for 18 days and then a NPWT ( mmhg) was applied. Forty-five days after the last debridement, the breast wound was covered with a full-thickness skin graft. Several months later, an excellent cosmetic result was observed. This is the first case of primary necrotising fasciitis of the breast treated associating HO and NPWT to surgical debridement only; this combination resulted in a complete recovery with the additional benefit of breast conservation. Such result is discussed in light of the available literature on the treatment of primary necrotising fasciitis of the breast. Introduction Necrotising fasciitis (NF) is a rare but potentially fatal disease. It is even more unusual as a primary disease of the breast, with no previous invasive procedure or other comorbid conditions. To the best of our knowledge, only 11 cases of spontaneous necrotising infection of the breast have been reported in the literature (1 11), and 5 of these were otherwise healthy women (3 5,8,11). Mastectomy is reported to be the main treatment in the published literature. We present here the first case of a healthy patient exhibiting primary necrotising fasciitis of the breast (PNFB) treated by combining conservative surgery with hyperbaric oxygen (HO) and negative pressure wound therapy (NPWT). We describe the details of the case, our management to conserve the breast, the outcome and a review of the literature. Key Messages necrotising fasciitis of the breast is a rare but potentially fatal disease mastectomy is reported to be the main treatment in the published literature we present here the first case of a healthy patient exhibiting primary necrotising fasciitis of the breast treated combining conservative surgery with hyperbaric oxygen (HO) and negative pressure wound therapy (NPWT) 2016 Medicalhelplines.com Inc and John Wiley & Sons Ltd 349

2 A rare case of primary necrotising fasciitis of the breast treated conservatively F. Marongiu et al. the antibiotic therapy and adequate wound debridement is the mainstay of treatment, but adjunctive use of HO and NPWT may reduce the number of debridements and may allow for breast conservation in our case, this association resulted in a complete recovery and breast conservation with the additional benefit of facilitated reconstruction, obtaining also a satisfactory cosmetic result the multi-modal approach is necessary in all phases of the treatment Case report A 39-year-old woman presented to the emergency room with fever and swelling of her right breast. The patient s history showed no previous use of drugs and oral contraceptives, no recent pregnancy, no trauma, no thromboembolic disease or other comorbidities. She was non-obese and a non-smoker. No known family history of breast cancer or other malignancies was noted. She had never undergone surgery or any other invasive procedure of the breast. She reported only having fever during the night; upon waking, she noted a redness on the superior pole of her right breast. After 12 hours, this area occupied the whole breast surface. Upon admission to the emergency unit, she was febrile. The physician examination showed swelling, erythema and oedema on her right breast, with a ecchymosis and bluish bulla on the lower quadrant (Figure 1). The left breast was normal, and there was no axillary lymphadenopathy bilaterally. Blood pressure was normal, and the electrocardiogram showed sinus tachycardia only. Blood tests results were normal. Ultrasound examination showed diffuse oedema of the entire right breast, with few subdermal gas bubbles in the outer lower quadrant and several undefined hypoechoic areas. The provisional diagnosis was breast mastitis, and IV broad-spectrum antibiotic therapy (Ceftriaxone) was started. Several hours later, the patient exhibited high fever (39 8 C), a new area of necrosis appeared on the outer lower quadrant of the right breast, and a rapid extension of the skin redness to the ipsilateral hemi-thorax was evident (Figure 2). Blood workup showed leukocytosis of cells/mm 3, with 92% Neutrophils, Hb 11 3 g/dl, INR 1 50, Na 129 mm, K 3 1 mm and PCR mg/l. Glycemia, Platelet and other results were normal. The CT scan showed skin thickening and subcutaneous gas in the deep tissues of the breast and beneath the skin surface, extending down the pectoral and latissimus dorsi fascial planes until the second lumbar vertebra (Figure 3). The patient was prepared for emergency surgery. In the operating room, following incision, necrosis and purulent infiltrate of the breast tissue was noted, involving mainly the lower outer quadrant, extending to the lower inner quadrant but sparing the nipple areola complex; the infection appeared to reach the fascial planes of the pectoralis major and latissimus dorsi muscles until the second lumbar vertebra, confirming the CT report. We performed a selective debridement, and apparently, all breast necrotic tissue was removed. Several drains were left in place in the breast and in the ipsilateral hemi-thorax (Figures 4 and 5). After surgery, the patient was admitted to the intensive care unit and remained intubated. The HO was immediately started and continued every day (once/day Figure 1 The appearance of the breast on initial examination. Figure 2 New area of necrosis with rapid extension of the skin redness to the ipsilateral hemi-thorax. 2 8 Bar 120 min). Culture results demonstrated Group A Streptococcus pyogenes, and adequate IV antibiotic treatment was initiated with Vancomicina-Meropenem-Clindamicina. The wound dressing was changed every other day. After 3 days, the patient s condition stabilised, the fever resolved, and the white blood cell count began to normalise. She was extubated with no further complications and was transferred to our surgical department. The pathology reports indicated acute necrotising inflammation of the dermis and breast tissue, with bacterial colonies in the areas of necrosis and no evidence of malignancy. One week later, we made a second selective debridement under general anaesthesia. Former overtly necrotic areas in the breast were maintained open to heal secondarily, while counterincisions were surrounded by healthy granulation tissue and were approximated, and new drains were positioned (Figure 6). After 18 days, the Medicalhelplines.com Inc and John Wiley & Sons Ltd

3 F. Marongiu et al. A rare case of primary necrotising fasciitis of the breast treated conservatively (A) (B) Figure 3 CT scan showing skin thickening and subcutaneous gas of the breast and beneath the skin surface, extending down the pectoral (A) and latissimus dorsi fascial planes (B) until the second lumbar vertebra. Figure 4 Intraoperative photograph after selective debridement of breast necrotic tissue. Several drains were left in place in the breast and in the ipsilateral hemi-thorax. The hyperbaric therapy was immediately started and continued every day (once/day 2 8 Bar 120 min). HO was suspended, and an NPWT that provided a continuous negative pressure to the wound ( mmhg) was applied (Figure 7). Following that, the patient s condition continued to improve. We progressively removed the drains; the wound started to granulate nicely; no further tissue necrosis was seen; and the breast began to exhibit signs of healing (Figure 8), so 45 days after the last debridement, we removed the NPWT and performed a new operation to cover the breast wound using a full-thickness skin graft taken from the abdomen region (Figures 9 and 10). After 5 days, no complications occurred, so the drain was removed, and the patient was discharged. Several months later, an excellent cosmetic result was observed (Figure 11). Discussion The term necrotising fasciitis was coined by Wilson in the 1950s to describe necrosis of the fascia and subcutaneous tissue Figure 5 The breast after 10 days showed only superficial marginal skin loss with small residual islands of necrosis, but the deep tissue appeared healthy. with sparing of the muscle (12). FN is one of the fastest spreading infections known, determined by an aggressive bacteria group that gets into the body and stealthily hides itself from the body s innate immune system. Bacteria quickly reproduce, spread rapidly along tissue planes and give off toxins and enzymes that destroy the soft tissues and fascias, which quickly become gangrenous. Furthermore, because of the systemic toxicity, the bacteria may cause multi-organ failure and demise (13). For this reason, the timeliness of diagnosis and treatment is crucial to save the patients lives. NF can attack any part of the 2016 Medicalhelplines.com Inc and John Wiley & Sons Ltd 351

4 A rare case of primary necrotising fasciitis of the breast treated conservatively F. Marongiu et al. Figure 8 Forty-five days after the last debridement, no further tissue necrosis was seen, and the breast began to exhibit signs of healing, so we removed the negative pressure wound therapy (NPWT) and performed a new operation to cover the wound. Figure 6 Postoperative photograph demonstrating the vitality of the wound tissue after the second selective debridement under general anaesthesia. Figure 9 Postoperative photograph showing a full-thickness skin graft reconstruction taken from the abdomen region. Figure 7 After 18 days, the hyperbaric oxygen therapy was suspended, and the wound defect closed with negative pressure wound therapy (NPWT) ( mmhg). body and primarily affects people with high comorbid index scores (trauma, surgeries, diabetes mellitus, obesity, smoking and every immunocompromised condition), although It can happen to the young, old, adult, child, any race, any size, healthy or not (13). Therefore, nobody is out of danger. Based on the aetiology, NF may be categorised into two main types. Typically, patients who develop type 1 (polymicrobial) are immunocompromised in some way. With the type 2 (monomicrobial) variety, patients are usually immunocompetent, with a history of trivial trauma. The type 2 is less common than the type 1 variety, but it is more dangerous because it is caused by group A beta-haemolytic Streptococci with very rapid necrosis, and people can go from perfectly healthy to death s door in a matter of day. For this reason, it is given the name flesh-eating bacteria (13). We performed a PubMed search using the terms necrotising fasciitis of the breast and primary necrotising fasciitis of the breast ; an additional extensive manual search on the same topic was conducted using references from relevant published papers. The first case of NF of the breast was described by Shah in 2001 (1), and only 11 cases of PNFB (i.e. with no previous trauma or intervention) have been reported in the literature so far (1 11) (Table 1); among these, only 5 cases without any associated comorbidity are reported (3 5,8,11). The current case is the first described Italian case of PNFB in otherwise healthy patients, the second in Europe and the sixth reported in the English literature (Table 1). The Medicalhelplines.com Inc and John Wiley & Sons Ltd

5 F. Marongiu et al. A rare case of primary necrotising fasciitis of the breast treated conservatively Figure 10 Skin graft donor site from the abdomen region. diagnosis of NF is mainly clinical and should be suspected when the skin appears red or darkened, tender or swollen, with formation of bluish bullae (Figure 2), and the local picture is associated with fever and severe pain disproportionate to the obvious physical signs (13). However, clinical suspicion may not be enough because these equivocal signs may initially mimic simple cellulitis, or mastitis, delaying diagnosis; the use of imaging, (Ultrasound, MRI or CT scan) may help in this context because findings such as irregularity of the fascias, abnormal fluid collections or gas tracking along fascial planes, fat stranding and diffuse thickening of the fascias outline the presence of necrosis that demands aggressive surgical management (13 15). Blood tests also proved useful, and Wong (4) proposed the use of the Laboratory Risk Indicator for Necrotising Fasciitis score may help in suspicious cases as a score of 6 7 should indicate early imaging or surgical exploration to rule out NF (13). In our case, clinical signs showed fever but no associated pain, swelling, erythema or oedema, with a ecchymosis and bluish bulla on the patient s right breast. The Laboratory Risk Indicator score was 8 and indicated the use of CT scan that, in turn, suggested a surgical exploration. Unfortunately, as no signs, imaging or tests are pathognomonic, today, the mortality rate is still 25 35% Figure 11 Cosmetic result after 4 months. because the time is the most important factor in survival (13,16). The standard management includes fluid resuscitation, intensive care support if indicated, intravenous broad-spectrum antibiotics and early surgical aggressive debridement leaving open wounds; based on the blood culture results, a specific intravenous antibiotics therapy must be started in association with multiple debridement if indicated (13,16). As far as NF affecting the breast is concerned, the mastectomy has been reported to be the most common surgical procedure in the published literature (13,16,17) (Table 1), but the most effective treatment is not well established because of the lack of a large case series. In this context, some authors have suggested breast conservative treatment (8,11), and only one paper reported about wound management using NPWT (10) (Table 1). HO is widely used in the treatment of acute and chronic infections because of numerous profitable actions: direct antimicrobial effect, increased antibiotic drug s efficacy, reduction of oedema, enhanced angiogenesis and fibroblasts proliferation (18). These benefits may theoretically impact positively on all the phases in the treatment of NF, synergistically improving both infection control and wound healing (18). However, to the best of our knowledge, nobody reported so far on the use of HO in the management of PNFB; On the Table 1 Summary of cases of primary necrotising fasciitis of the breast Authors Country Year Comorbidity Cultures Treatment Shah India 2001 Diabetes Polymicrobial Mastectomy Nizami Pakistan 2006 Steroid treatment Polymicrobial Mastectomy + skin graft Rajakannu India 2006 None Polymicrobial Mastectomy + skin graft Wong Singapore 2008 None Unknown Quadrantectomy + secondary suture Keune USA 2008 None Polymicrobial Mastectomy Venkatramani India 2009 HIV positive Polymicrobial Mastectomy Vishwanath India 2011 Lactating Unknown Mastectomy Soliman Kuwait 2011 None Polymicrobial Multiple selective debridements + skin graft Yaji India 2014 Diabetes Polymicrobial Wide debridement Lee Korea 2015 Pregnant Monomicrobial Wide debridement + NPWT and secondary suture Yang Europe 2015 None Polymicrobial Selective debridement + skin graft Present case Europe 2015 None Monomicrobial Selective debridement + hyperbaric oxygen + NPWT and skin graft NPWT, negative pressure wound therapy 2016 Medicalhelplines.com Inc and John Wiley & Sons Ltd 353

6 A rare case of primary necrotising fasciitis of the breast treated conservatively F. Marongiu et al. basis of these considerations, we performed a selective surgical debridement (addressing only the overtly necrotic tissue), and the HO was immediately associated to exploit its additional effect of infection control and enhanced wound healing. This allowed us to obtain an excellent clearance of the infection from the breast tissue in association with a healthy, lively growing surrounding tissues sustained by NPWT; thus, the number of debridement procedures was minimal. Once the main goal of survival was granted, some room for pursuing an aesthetic outcome was created; when the clinical condition was stable and the breast tissue was definitively healthy, the NPWT could be removed, and the wound was covered with a split skin graft. We decided to take the skin from the abdomen region because the patient had a skin excess, thus optimising the cosmetic results. Conclusion Early diagnosis and timely treatment are crucial to save the patients life. The multi-modal approach is necessary in all phases of the treatment and may also allow the balancing of surgical aggressiveness to conserve the breast. This is the first case of PNFB case successfully treated conservatively, associating HO with selective surgical debridement and NPWT. This association resulted in a complete recovery and breast conservation, with the additional benefit of facilitated reconstruction and satisfactory cosmetic result. We recommend more prospective clinical studies to be carried out to analyse this kind of approach to such life-threatening diseases. Acknowledgements The authors wish to thank Dr Julie-Ann Smith for revising the manuscript. None of the authors have a financial interest in any of the products, devices or drugs mentioned in this manuscript. The authors declare that they have no conflict of interest. References 1. Shah J, Sharma AK, O Donoghue JM, Mearns B, Johri A, Thomas V. Necrotizing fasciitis of the breast. Br J Plast Surg 2001;54: Nizami S, Mohiuddin K, Azam M, Zafar H, Memon MA. Necrotizing fasciitis of the breast. Breast J 2006;12: Rajakannu M, Kate V, Ananthakrishnan N. Necrotizing infection of the breast mimicking carcinoma. Breast J 2006;12: Wong CH, Tan BK. Necrotizing fasciitis of the breast. Plast Reconstr Surg 2008;122:151e Keune J, Melby S, Kirby J, Aft R. Shared management of a rare necrotizing soft tissue infection of the breast. Breast J 2009;15: Venkatramani V, Pillai S, Marathe S, Rege SA, Hardikar JV. Breast gangrene in an HIV-positive patient. Ann R Coll Surg Engl 2009;91:W Vishwanath G, Basarkod SI, Katageri GM, Mirji P, Mallapur AS. Necrotizing fasciitis of the breast with shock and postpartum psychosis. J Clin Diagn Res 2011;5: Soliman MO, Ayyash EH, Aldahham A, Asfar S. Necrotizing fasciitis of the breast: a case managed without mastectomy. Med Princ Pract 2011;20: Yaji P, Bhat B, Harish E. Primary necrotising fasciitis of the breast: case report and brief review of literature. J Clin Diagn Res 2014;8:ND Lee J, Ju Lee K, Sun WY. Necrotizing fasciitis of the breast in a pregnant woman successfully treated using negative-pressure wound therapy. Ann Surg Treat Res 2015;89: Yang B, Connolly S, Ball W. Necrotising fasciitis of the breast: a rare primary case with conservation of the nipple and literature review. JPRAS Open 2015;6:15e Wilson B. Necrotising fasciitis. Am Surg 1952;18: Puvanendran R, Huey JC, Pasupathy S. Necrotizing fasciitis. Can Fam Physician 2009;55: Brothers TE, Tagge DU, Stutley JE, Conway WF, Del Schutte H Jr, Byrne TK. Magnetic resonance imaging differentiates between necrotizing and non-necrotizing fasciitis of the lower extremity. JAm Coll Surg 1998;187: Tsai CC, Lai CS, Yu ML, Chou CK, Lin SD. Early diagnosis of necrotizing fasciitis by utilization of ultrasonography. Kaohsiung J Med Sci 1996;12: Sarani B, Strong M, Pascual J, Schwab CW. Necrotizing fasciitis: current concepts and review of the literature. J Am Coll Surg 2009;208: Angarita FA, Acuna SA, Torregrosa L, Tawil M, Sánchez EF, Heilbron O, Domínguez LC. Bilateral necrotizing fasciitis of the breast following quadrantectomy. Breast Cancer 2014;21: Bhutani S, Vishwanath G. Hyperbaric oxygen and wound healing. Indian J Plast Surg 2012;45: Medicalhelplines.com Inc and John Wiley & Sons Ltd