508648
research-article2013
IJLXXX10.1177/1534734613508648The International Journal of Lower Extremity WoundsJun et al
Case Report
A Case of Fatal Necrotizing Fasciitis Arising From Chronic Lymphedema
The International Journal of Lower Extremity Wounds 12(4) 293–296 © The Author(s) 2013 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1534734613508648 ijl.sagepub.com
Young Joon Jun, MD, PhD1, In Sook Kang, MD1, Jung Ho Lee, MD, PhD1, Sue Min Kim, MD1, and Young Jin Kim, MD, PhD1
Abstract Chronic lymphedema and lymphangitis are common adverse effects following treatment for gynecological cancer. Because the early symptoms of necrotizing fasciitis are similar to those of lymphangitis, fatal outcome can occur if patients or physicians underestimate this condition. Here, we present a case of necrotizing fasciitis in a patient with chronic lymphedema. Keywords chronic lymphedema, necrotizing fasciitis, magnetic resonance imaging Lower limb lymphedema is one of the most disabling adverse effects of both surgical and radiological treatments for gynecological cancer. Its incidence varies according to the type of gynecological cancer and treatment modalities, and it is high in cases of vulvar cancer (35.8%) and cervical cancer (12.2%).1 Lymphedema patients feel a fullness or heaviness in the affected limb and suffer from generalized discomfort and a decreased range of motion. In addition, patients often experience infectious complications such as lymphangitis and erysipelas due to decreased local immunity, which occurs as a result of increased interstitial fluid pressure and lymphatic fluid retention.2,3 Whereas most cases of lymphangitis can be managed by administration of antibiotics, deep tissue infection such as necrotizing fasciitis (NF) must be treated through prompt and radical surgical debridement. However, because patients with chronic lymphedema often experience recurrent lymphangitis, with symptoms similar to those of NF, the severity of infection may be overlooked. In this article, we report a case of fatal NF arising from a chronic edematous limb. Because the patient assumed the condition to be due to a recurrence of lymphangitis, the diagnosis was delayed until hospitalization was required for septic shock. We believe that patients with chronic lymphedema should be informed that even in the absence of trauma or penetrating wound, fatal deep tissue infection may occur in an otherwise healthy patient.
Case Report A 52-year-old woman presented to the emergency department of our hospital with skin color change in both lower legs and a decreased level of consciousness. On physical
Figure 1. Clinical photographs of the patient obtained during the initial visit.
There are severe edema, bullae, and skin color changes in both lower legs.
examination, severe edema, bullae, skin color changes to red purple, and severe tenderness of the lower extremities were observed (Figure 1). The patient had a history of 1
Catholic University of Korea, Gyeonggi-do, South Korea
Corresponding Author: Jung Ho Lee, Department of Plastic and Reconstructive Surgery, Bucheon St Mary’s Hospital, College of Medicine, Catholic University of Korea, 2 Sosa-dong, Wonmi-gu, Bucheon-si, Gyeonggi-do 420-717, South Korea. Email: [email protected]
Downloaded from ijl.sagepub.com at University of Otago Library on November 16, 2015
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The International Journal of Lower Extremity Wounds 12(4)
Figure 2. Initial magnetic resonance images of the left lower leg.
(A) Axial section. (B) Coronal section. The deep fascia and subcutaneous tissue in the T2-weighted images are enhanced. Thickening of the skin associated with the bullae of varying sizes is also noted (arrow).
chronic lymphedema in both lower extremities, which developed following a radical hysterectomy, with pelvic lymph node dissection and chemoradiotherapy for cervical cancer 7 years earlier. She had no other comorbidities such as diabetes, malnutrition, alcohol abuse, or renal failure. Three days before the visit, fever, rashes, and tenderness of the lower extremities developed without an obvious history of trauma. Because she had experienced lymphangitis several times, the patient thought that it had recurred and took antibiotics that had been prescribed for lymphangitis. On arrival in the hospital, the patient’s vital signs were as follows: blood pressure, 70/50 mm Hg; heart rate, 107 beats/ min; temperature, 37°C; and respiratory rate, 40 breaths/ min. The peripheral blood cell count test revealed a white blood cell count of 3440/mm3, hemoglobin level of 16.1 g/ dL, and platelet count of 20 000/mm3. The C-reactive protein (CRP) level was ≥34.1 mg/dL, lactate dehydrogenase level was 914 U/L, and creatine phosphate kinase level was 1749 U/L. Magnetic resonance imaging revealed diffuse edema in the subcutaneous tissue and muscles of all compartments of the lower leg. In addition, the subcutaneous tissue and deep fascia were enhanced, which suggested NF (Figure 2). Empirical antibiotics (vancomycin and tazocin) were immediately administered, and emergency surgery (fasciotomy and debridement) was performed (Figure 3).
Figure 3. Clinical photograph obtained after surgical debridement.
(A) Right leg. (B) Left leg. Necrotic tissue including the skin, subcutaneous layer, and muscle fascia is debrided.
Downloaded from ijl.sagepub.com at University of Otago Library on November 16, 2015
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Jun et al
Figure 4. Immediate postoperative photographs.
(A) Right leg. (B) Left leg. The right lower leg is covered with splitthickness skin graft, and the left leg is covered with free latissimus dorsi muscle flap and split-thickness skin graft.
Figure 5. Postoperative 3 months photograph.
Escherichia coli was cultured from the initial blood culture and lower leg tissue, and the antibiotics were changed to a combination of vancomycin, ceftriaxone, clindamycin, and doxycycline, then to a combination of vancomycin and meropenem, and finally to ceftriaxone monotherapy. One month after admission, the patient underwent a reconstructive operation with free latissimus dorsi muscle flap and split-thickness skin graft (Figure 4). During the 6 months of follow-up, no complications occurred (Figure 5).
Discussion Necrotizing fasciitis is a rare and potentially fatal infection characterized by a rapidly progressive necrosis of the subcutaneous tissue and fascia.4 After microbes invade the subcutaneous tissue through external trauma or direct spread from a perforated viscus, they track subcutaneous tissue, producing endo- and exotoxins that cause microvascular thrombosis, tissue ischemia, liquefactive necrosis, and often systemic illness, which can lead to septic shock and death.4-6 Generally, NF occurs as a result of some precipitating event such as laceration, burn, bite, or subcutaneous injection. However, idiopathic NF can also occur in the absence of known or identifiable factors.7 Old age (>55 years), diabetes mellitus, and chronic renal failure are known to be
The flap and grafted skin has survived completely; no complication was observed.
associated with idiopathic NF. In patients with lymphedema, local immunity is decreased because inadequate lymphatic drainage disrupts the regularity of lymphocyte and Langerhans cell trafficking, resulting in ineffective removal of foreign antigens.2,3 Hence, lymphedematous patients are prone to infections such as lymphangitis, erysipelas, and rarely NF, even without direct trauma. The clinical presentation of NF includes severe edema, tenderness, and a rapidly progressive necrosis of the skin, possibly resulting in death due to multiple organ dysfunction and shock.4-6 Antibiotics alone can mask the severity of infection, and therefore, early aggressive surgical debridement is mandatory to decrease mortality. In a previous study by McHenry et al, the average time from admission to operation was 90 hours in nonsurvivors and 25 hours in survivors.8 However, the absence of NF-specific symptoms makes early diagnosis of NF difficult. Moreover, in chronic lymphedematous patients, diagnosis might easily be delayed because the early symptoms may be masked in the edematous limb and be regarded as cellulitis or lymphangitis, as in our case. Hence, high level of awareness is important for survival of the patient. If patients fail to respond to broad
Downloaded from ijl.sagepub.com at University of Otago Library on November 16, 2015
296
The International Journal of Lower Extremity Wounds 12(4)
spectrum antibiotics, develop cutaneous manifestation, or feel pain that is out of proportion to the apparent severity of the lesion, the physician should suspect NF.9 Performing advanced imaging modalities such as computed tomography or magnetic resonance imaging is helpful for early diagnosis.10 Frozen section biopsy is also useful for a quick and accurate diagnosis by checking for the presence of abundant granulocytes in the fascia and subcutis layer.11 In addition, several diagnostic adjuncts such as laboratory risk indicator for necrotizing fasciitis score or transcutaneous tissue oxygen saturation monitoring are useful in diagnosing NF.12 Finally, the role of the physician as a patient educator is essential in preventing delayed treatment. Patients should be aware that life-threatening infection can develop in an otherwise healthy individual, and a high index of suspicion is necessary for survival. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Beesley V, Janda M, Eakin E, Obermair A, Battistutta D. Lymphedema after gynecological cancer treatment: prevalence, correlates, and supportive care needs. Cancer. 2007;109:2607-2614.
2. Ruocco V, Schwartz RA, Ruocco E. Lymphedema: an immunologically vulnerable site for development of neoplasms. J Am Acad Dermatol. 2002;47:124-127. 3. Ruocco V, Brunetti G, Puca RV, Ruocco E. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol. 2009;23:1364-1373. 4. Al Shukry S, Ommen J. Necrotizing fasciitis—report of ten cases and review of recent literature. J Med Life. 2013;6:189194. 5. Khanna AK, Tiwary SK, Kumar P, Khanna R, Khanna A. A case series describing 118 patients with lower limb necrotizing fasciitis. Int J Low Extrem Wounds. 2009;8:112-116. 6. Cainzos M, Gonzalez-Rodriguez FJ. Necrotizing soft tissue infections. Curr Opin Crit Care. 2007;13:433-439. 7. Taviloglu K, Cabioglu N, Cagatay A, et al. Idiopathic necrotizing fasciitis: risk factors and strategies for management. Am Surg. 2005;71:315-320. 8. McHenry CR, Piotrowski JJ, Petrinic D, Malangoni MA. Determinants of mortality for necrotizing soft-tissue infections. Ann Surg. 1995;221:558-563. 9. Naqvi GA, Malik SA, Jan W. Necrotizing fasciitis of the lower extremity: a case report and current concept of diagnosis and management. Scand J Trauma Resusc Emerg Med. 2009;17:28. 10. Oelze L, Wu S, Carnell J. Emergency ultrasonography for the early diagnosis of necrotizing fasciitis: a case series from the ED. Am J Emerg Med. 2013;31:632e5-e7. 11. Stegeman SA, Nijhuis I, van Leeuwen AM, Bonsing BA, Steenvoorde P. The value of frozen section biopsy in diagnosing necrotizing fasciitis: proposal of a new grading system. J Tissue Viability. 2012;21:13-16. 12. Wong CH, Wang YS. The diagnosis of necrotizing fasciitis. Curr Opin Infect Dis. 2005;18:101-106.
Downloaded from ijl.sagepub.com at University of Otago Library on November 16, 2015
research-article2013
IJLXXX10.1177/1534734613508648The International Journal of Lower Extremity WoundsJun et al
Case Report
A Case of Fatal Necrotizing Fasciitis Arising From Chronic Lymphedema
The International Journal of Lower Extremity Wounds 12(4) 293–296 © The Author(s) 2013 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1534734613508648 ijl.sagepub.com
Young Joon Jun, MD, PhD1, In Sook Kang, MD1, Jung Ho Lee, MD, PhD1, Sue Min Kim, MD1, and Young Jin Kim, MD, PhD1
Abstract Chronic lymphedema and lymphangitis are common adverse effects following treatment for gynecological cancer. Because the early symptoms of necrotizing fasciitis are similar to those of lymphangitis, fatal outcome can occur if patients or physicians underestimate this condition. Here, we present a case of necrotizing fasciitis in a patient with chronic lymphedema. Keywords chronic lymphedema, necrotizing fasciitis, magnetic resonance imaging Lower limb lymphedema is one of the most disabling adverse effects of both surgical and radiological treatments for gynecological cancer. Its incidence varies according to the type of gynecological cancer and treatment modalities, and it is high in cases of vulvar cancer (35.8%) and cervical cancer (12.2%).1 Lymphedema patients feel a fullness or heaviness in the affected limb and suffer from generalized discomfort and a decreased range of motion. In addition, patients often experience infectious complications such as lymphangitis and erysipelas due to decreased local immunity, which occurs as a result of increased interstitial fluid pressure and lymphatic fluid retention.2,3 Whereas most cases of lymphangitis can be managed by administration of antibiotics, deep tissue infection such as necrotizing fasciitis (NF) must be treated through prompt and radical surgical debridement. However, because patients with chronic lymphedema often experience recurrent lymphangitis, with symptoms similar to those of NF, the severity of infection may be overlooked. In this article, we report a case of fatal NF arising from a chronic edematous limb. Because the patient assumed the condition to be due to a recurrence of lymphangitis, the diagnosis was delayed until hospitalization was required for septic shock. We believe that patients with chronic lymphedema should be informed that even in the absence of trauma or penetrating wound, fatal deep tissue infection may occur in an otherwise healthy patient.
Case Report A 52-year-old woman presented to the emergency department of our hospital with skin color change in both lower legs and a decreased level of consciousness. On physical
Figure 1. Clinical photographs of the patient obtained during the initial visit.
There are severe edema, bullae, and skin color changes in both lower legs.
examination, severe edema, bullae, skin color changes to red purple, and severe tenderness of the lower extremities were observed (Figure 1). The patient had a history of 1
Catholic University of Korea, Gyeonggi-do, South Korea
Corresponding Author: Jung Ho Lee, Department of Plastic and Reconstructive Surgery, Bucheon St Mary’s Hospital, College of Medicine, Catholic University of Korea, 2 Sosa-dong, Wonmi-gu, Bucheon-si, Gyeonggi-do 420-717, South Korea. Email: [email protected]
Downloaded from ijl.sagepub.com at University of Otago Library on November 16, 2015
294
The International Journal of Lower Extremity Wounds 12(4)
Figure 2. Initial magnetic resonance images of the left lower leg.
(A) Axial section. (B) Coronal section. The deep fascia and subcutaneous tissue in the T2-weighted images are enhanced. Thickening of the skin associated with the bullae of varying sizes is also noted (arrow).
chronic lymphedema in both lower extremities, which developed following a radical hysterectomy, with pelvic lymph node dissection and chemoradiotherapy for cervical cancer 7 years earlier. She had no other comorbidities such as diabetes, malnutrition, alcohol abuse, or renal failure. Three days before the visit, fever, rashes, and tenderness of the lower extremities developed without an obvious history of trauma. Because she had experienced lymphangitis several times, the patient thought that it had recurred and took antibiotics that had been prescribed for lymphangitis. On arrival in the hospital, the patient’s vital signs were as follows: blood pressure, 70/50 mm Hg; heart rate, 107 beats/ min; temperature, 37°C; and respiratory rate, 40 breaths/ min. The peripheral blood cell count test revealed a white blood cell count of 3440/mm3, hemoglobin level of 16.1 g/ dL, and platelet count of 20 000/mm3. The C-reactive protein (CRP) level was ≥34.1 mg/dL, lactate dehydrogenase level was 914 U/L, and creatine phosphate kinase level was 1749 U/L. Magnetic resonance imaging revealed diffuse edema in the subcutaneous tissue and muscles of all compartments of the lower leg. In addition, the subcutaneous tissue and deep fascia were enhanced, which suggested NF (Figure 2). Empirical antibiotics (vancomycin and tazocin) were immediately administered, and emergency surgery (fasciotomy and debridement) was performed (Figure 3).
Figure 3. Clinical photograph obtained after surgical debridement.
(A) Right leg. (B) Left leg. Necrotic tissue including the skin, subcutaneous layer, and muscle fascia is debrided.
Downloaded from ijl.sagepub.com at University of Otago Library on November 16, 2015
295
Jun et al
Figure 4. Immediate postoperative photographs.
(A) Right leg. (B) Left leg. The right lower leg is covered with splitthickness skin graft, and the left leg is covered with free latissimus dorsi muscle flap and split-thickness skin graft.
Figure 5. Postoperative 3 months photograph.
Escherichia coli was cultured from the initial blood culture and lower leg tissue, and the antibiotics were changed to a combination of vancomycin, ceftriaxone, clindamycin, and doxycycline, then to a combination of vancomycin and meropenem, and finally to ceftriaxone monotherapy. One month after admission, the patient underwent a reconstructive operation with free latissimus dorsi muscle flap and split-thickness skin graft (Figure 4). During the 6 months of follow-up, no complications occurred (Figure 5).
Discussion Necrotizing fasciitis is a rare and potentially fatal infection characterized by a rapidly progressive necrosis of the subcutaneous tissue and fascia.4 After microbes invade the subcutaneous tissue through external trauma or direct spread from a perforated viscus, they track subcutaneous tissue, producing endo- and exotoxins that cause microvascular thrombosis, tissue ischemia, liquefactive necrosis, and often systemic illness, which can lead to septic shock and death.4-6 Generally, NF occurs as a result of some precipitating event such as laceration, burn, bite, or subcutaneous injection. However, idiopathic NF can also occur in the absence of known or identifiable factors.7 Old age (>55 years), diabetes mellitus, and chronic renal failure are known to be
The flap and grafted skin has survived completely; no complication was observed.
associated with idiopathic NF. In patients with lymphedema, local immunity is decreased because inadequate lymphatic drainage disrupts the regularity of lymphocyte and Langerhans cell trafficking, resulting in ineffective removal of foreign antigens.2,3 Hence, lymphedematous patients are prone to infections such as lymphangitis, erysipelas, and rarely NF, even without direct trauma. The clinical presentation of NF includes severe edema, tenderness, and a rapidly progressive necrosis of the skin, possibly resulting in death due to multiple organ dysfunction and shock.4-6 Antibiotics alone can mask the severity of infection, and therefore, early aggressive surgical debridement is mandatory to decrease mortality. In a previous study by McHenry et al, the average time from admission to operation was 90 hours in nonsurvivors and 25 hours in survivors.8 However, the absence of NF-specific symptoms makes early diagnosis of NF difficult. Moreover, in chronic lymphedematous patients, diagnosis might easily be delayed because the early symptoms may be masked in the edematous limb and be regarded as cellulitis or lymphangitis, as in our case. Hence, high level of awareness is important for survival of the patient. If patients fail to respond to broad
Downloaded from ijl.sagepub.com at University of Otago Library on November 16, 2015
296
The International Journal of Lower Extremity Wounds 12(4)
spectrum antibiotics, develop cutaneous manifestation, or feel pain that is out of proportion to the apparent severity of the lesion, the physician should suspect NF.9 Performing advanced imaging modalities such as computed tomography or magnetic resonance imaging is helpful for early diagnosis.10 Frozen section biopsy is also useful for a quick and accurate diagnosis by checking for the presence of abundant granulocytes in the fascia and subcutis layer.11 In addition, several diagnostic adjuncts such as laboratory risk indicator for necrotizing fasciitis score or transcutaneous tissue oxygen saturation monitoring are useful in diagnosing NF.12 Finally, the role of the physician as a patient educator is essential in preventing delayed treatment. Patients should be aware that life-threatening infection can develop in an otherwise healthy individual, and a high index of suspicion is necessary for survival. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Beesley V, Janda M, Eakin E, Obermair A, Battistutta D. Lymphedema after gynecological cancer treatment: prevalence, correlates, and supportive care needs. Cancer. 2007;109:2607-2614.
2. Ruocco V, Schwartz RA, Ruocco E. Lymphedema: an immunologically vulnerable site for development of neoplasms. J Am Acad Dermatol. 2002;47:124-127. 3. Ruocco V, Brunetti G, Puca RV, Ruocco E. The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites. J Eur Acad Dermatol Venereol. 2009;23:1364-1373. 4. Al Shukry S, Ommen J. Necrotizing fasciitis—report of ten cases and review of recent literature. J Med Life. 2013;6:189194. 5. Khanna AK, Tiwary SK, Kumar P, Khanna R, Khanna A. A case series describing 118 patients with lower limb necrotizing fasciitis. Int J Low Extrem Wounds. 2009;8:112-116. 6. Cainzos M, Gonzalez-Rodriguez FJ. Necrotizing soft tissue infections. Curr Opin Crit Care. 2007;13:433-439. 7. Taviloglu K, Cabioglu N, Cagatay A, et al. Idiopathic necrotizing fasciitis: risk factors and strategies for management. Am Surg. 2005;71:315-320. 8. McHenry CR, Piotrowski JJ, Petrinic D, Malangoni MA. Determinants of mortality for necrotizing soft-tissue infections. Ann Surg. 1995;221:558-563. 9. Naqvi GA, Malik SA, Jan W. Necrotizing fasciitis of the lower extremity: a case report and current concept of diagnosis and management. Scand J Trauma Resusc Emerg Med. 2009;17:28. 10. Oelze L, Wu S, Carnell J. Emergency ultrasonography for the early diagnosis of necrotizing fasciitis: a case series from the ED. Am J Emerg Med. 2013;31:632e5-e7. 11. Stegeman SA, Nijhuis I, van Leeuwen AM, Bonsing BA, Steenvoorde P. The value of frozen section biopsy in diagnosing necrotizing fasciitis: proposal of a new grading system. J Tissue Viability. 2012;21:13-16. 12. Wong CH, Wang YS. The diagnosis of necrotizing fasciitis. Curr Opin Infect Dis. 2005;18:101-106.
Downloaded from ijl.sagepub.com at University of Otago Library on November 16, 2015
