Necrotising fasciitis: A sequelae of varicella zoster infection

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1 Journal of Plastic, Reconstructive & Aesthetic Surgery (2011) 64, 123e127 CASE REPORT Necrotising fasciitis: A sequelae of varicella zoster infection Rebecca Shirley a, *, Simon Mackey b, Peter Meagher b a The Blizzard Institute, 4 Newark Street, London E1 2AT, United Kingdom b St George s Hospital, Blackshaw Road, Tooting, United Kingdom Received 2 December 2009; accepted 11 March 2010 KEYWORDS Necrotising fasciitis; Varicella zoster Summary Necrotising fasciitis (NF) can complicate varicella zoster virus in children. This is rare and has not previously been reported in the plastic surgery literature. We report a case of a female toddler who developed necrotising fasciitis secondary to chicken pox. Her presentation and progress are reported, the diagnosis of necrotising fasciitis in children and the small number of case series and case control studies are discussed. ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. Case presentation A 33-month-old female was presented at accident and emergency with a history of development of vesicles characteristic of varicella zoster virus (chicken pox). She had been vomiting for five days and developed cellulitis on her right flank related to a clump of vesicles. She had been treated with regular ibuprofen syrup and deteriorated clinically in the last two days. She had also been prescribed oral flucloxacillin which she had taken for 24 h. On the day of presentation she had vomited five times and her parents commented on reduced urine output: only one wet nappy. * Corresponding author. þ address: rshirley@doctors.net.uk (R. Shirley). Past medical history included pneumococcal meningitis aged one, for which she was treated with intravenous antibiotics for five days. Immunological tests had previously been performed to rule out immunodeficiency: immunoglobulin levels including IgG sub-classes were normal. Vaccination schedule had been carried out as recommended. On presentation she was alert with a temperature 38.1 C pulse of 160/min and peripheral capillary refill of 2 s. In her right flank below her axilla were two welldemarcated areas of cellulitis, tender to palpation with no fluctuance or crepitus; the lower area had a small central area of dark eschar. Blood tests revealed a raised white cell count of /L with a neutrophilia, C-reactive protein (CRP) of 377 mg/l and INR 1.5. Renal function was normal. She was admitted for intravenous cefuroxime, flucloxacillin and maintenance fluids. Nonsteroidal anti-inflammatory medication was stopped. The following morning she remained pyrexial and tachycardic and by the evening further areas of cellulitis developed on /$ - see front matter ª2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi: /j.bjps

2 124 R. Shirley et al. the left chest. Twelve hours later she was lethargic with peripheral capillary refill of 4e5 s and a larger cellulitic area on her right chest with two exquisitely painful pustular spots. She also had a widespread blanching rash to her groin and axillae. The possibility of necrotising fasciitis was raised and she was transferred to our unit for plastic surgery assessment and Paediatric Intensive Care. On assessment at admission temperature was 38.7 C, respiratory rate was 36/min, this had increased from 30/ min. Areas of cellulitis were visible in the right chest extending to the back, right groin and sacral region, these regions were all tender with a purplish dusky hue, these had drastically advanced beyond the demarcated borders over the last twenty-four hours. The throat was also inflamed. Widespread crusted vesicles were also present. On the advice of the microbiologist an empirical regime of highdose penicillin, high-dose clindamycin, gentamicin and flucloxacillin was commenced until culture results became available. Gram stain from wound exudates revealed moderate concentrations of streptococcus and high concentration of white cells. She was taken to theatre for urgent debridement where dish-water fluid was revealed on incising the tissue. An area inferomedial to the right axilla 10 8 cm was debrided with an area of necrotic fascia, a further area of 5 3 cm was debrided at the right iliac fossa. All necrotic tissue was debrided until healthy tissue was reached and after copious wash with both hydrogen peroxide and betadine a VACâ dressing was applied to the area on the right chest. Post-operatively she went to Paediatric Intensive Care Unit (PICU) on Bilevel positive airway pressure (BIPAP). She returned theatre twenty-four hours later for the change of dressing and second look; at this stage areas of cellulitis had shown obvious signs of regressing. At operation pectoralis major, serratus anterior and latissimus dorsi muscles were clearly visualised and not necrotic. She remained intubated and returned to PICU. In total she spent 7 days on PICU, she was intubated for four days. She had five further debridements in theatre and seven days after the initial debridement had split skin graft applied to the defect on the right chest and the defect at the right iliac fossa was closed directly. She subsequently underwent one change of dressing in theatre and was discharged 12 days after the initial debridement. She subsequently has made a complete recovery with no functional deficit (Figures 1e3). Blood culture from time of admission grew Group A streptococcus. Histology from specimens sent at the first debridement from both the chest and the iliac fossa showed an extensive necrotising process in the deep subcutaneous tissue, including necrosis of skeletal muscle fibres, with associated oedema, acute inflammation and secondary vasculitis, abundant gram positive cocci were visualised. Figure 1 Patient after first debridement. potential devastating sequelae and requires prompt diagnosis and surgical management. The condition is difficult to recognise in the early phase, when it is often confused with cellulitis, 2 and although ultrasound has been suggested to aid diagnosis, 3 imaging studies should not delay surgical debridement. 4 Symptoms and signs that can be associated with cellulitis, may suggest necrotising fasciitis: rapid progression, poor therapeutic response, blistering necrosis, cyanosis, extreme local tenderness, high temperature, tachycardia, hypotension and altered level of consciousness. Organisms spread from the subcutaneous tissue along the superficial and deep fascial planes, presumably facilitated by bacterial enzymes and toxins. This deep infection causes vascular occlusion, ischemia, and tissue necrosis. Superficial nerves are damaged, producing the characteristic localised anaesthesia. Septicemia ensues with systemic toxicity. Streptococcal pyrogenic exotoxins (SPEs) A, B, and C are directly toxic and tend to be produced by strains causing necrotising fasciitis. These pyrogenic exotoxins, together with streptococcal superantigen (SSA), lead to the release of cytokines and produce clinical signs such as hypotension. Difficulty diagnosing necrotising fasciitis in children Unlike adult cases, necrotising fasciitis in children often appears in otherwise healthy individuals who have no chronic disease or pre-disposition to infection. In a number Discussion Necrotising fasciitis is a rapidly progressive and often fatal infection of the soft-tissue fascia deep to the skin but superficial to the muscles. It was popularised in the lay media during the 1980s after an increase in prevalence said to be caused by flesh-eating bacteria. 1 Necrotising fasciitis as a complication of varicella zoster infection (chicken pox) is rare; it is a serious condition with Figure 2 Patient after fifth debridement.

3 Necrotising fasciitis 125 apply equally to the diagnosis of toxic shock syndrome. Diagnostic criteria for toxic shock syndrome were summarised by Reingold and Hargrett in 1981 and although this enables a diagnosis to be reached on very specific criteria, just with necrotising fasciitis two of the criteria are pain and hypotension 11 (Table 1). In addition parental and child distress can make assessment of a child more challenging, for example it may take longer to obtain blood samples. Types of necrotising fasciitis Figure 3 Patient at six weeks post-discharge. of studies approximately 75% of affected patients have had an underlying disease processes affecting resistance to infection, including diabetes, peripheral vascular disease, metastatic carcinoma, and chronic renal failure in adults. 5e8 Necrotising fasciitis usually develops after a specific inciting event such as a perforated viscus or severe soft-tissue trauma. 7 Ninety-five percent of one series of children developing the condition 9 and 91% of another have previously been healthy. 10 Severe pain and systemic toxicity reflect the widespread tissue necrosis underlying the apparently viable skin. In young children it is often not possible to obtain a reliable blood pressure and patients are also unable to give a history of extreme pain. These difficulties in obtaining a history The most common type of necrotising fasciitis: Type 1 is a polymicrobial infection with both aerobic and anaerobic organisms such as Clostridium, Proteus, Escherichia coli, Bacteroides, and Enterobacteriaceae. 12 A second form of the disease illustrated in this case, Type 2 is caused by group A streptococci and is seen in approximately 10% of cases. 12,13 Toxic shock syndrome may complicate this in later form 13,14 as exemplified by this case. These types are summarised in Table 2. Necrotising fasciitis associated with chicken pox A literature review revealed a small number or retrospective reviews with relatively small sample sizes: n Z five, 15 eighteen, 13 nineteen 16 and twenty-five. 17 These case series were all from the United States and are suggestive of minor epidemics, probably related to virulent strains of streptococcus. A case control study from 1996 compared 25 patients who developed necrotising fasciitis secondary to chicken pox over a five months period with 25 patients with an Table 1 TSS Case Diagnosis Criteria a I Fever II Rash III Hypotension IV Multisystem dysfunction, at least 3 V Desquamation VI Evidence against an alternative diagnosis, if obtained >38.9 C Diffuse macular erythroderma ( sunburn ) Systolic blood pressure <90 mmhg (adults) or <5th percentile for age (children under 16 years of age), or orthostatic hypotension, dizziness or syncope A) Gastrointestinal: vomiting or diarrhoea at onset of illness B) Muscular: severe myalgias, or serum creatine phosphokinase level (CPK) >twice upper limit of normal C) Mucous membranes: vaginal, oropharyngeal, or conjunctival hyperaemia D) Renal: blood urea nitrogen (BUN) or creatinine >twice upper limit of normal, or pyuria (>5 leukocytes per high power field), in the absence of urinary tract infection E) Hepatic: total serum bilirubin or transaminase level >twice upper limit of normal F) Hematologic: platelets <100,000 L 1 G) Central nervous system: disorientation or alteration in consciousness but no focal neurological signs at a time when fever and hypotension are absent 1 to 2 weeks after the onset of illness (typically palms and soles) Negative cultures of blood, throat, or cerebrospinal fluid b ; absence of a rise in antibody titres to the agents of leptospirosis, measles or Rocky Mountain spotted fever Reingold AL, Hargrett NT, et al. Toxic shock surveillance in the United States, 1980 to Ann Intern Med 1982;96(Part 2):875e880. a Confirmed case meets all six criteria; probable case meets five of the six. b Blood cultures may be positive for Staphylococcus aureus.

4 126 R. Shirley et al. Table 2 Classification of Necrotising fasciitis. Type 1 Polymicrobial May initially be mistaken for a simple wound cellulitis. However, severe pain and systemic toxicity reflect widespread tissue necrosis underlying apparently viable skin. May also be observed in association with urogenital or anogenital infections Type 2 Group A The so-called flesh-eating bacterial infection Streptococcus Type 3 Gas gangrene Clostridial myonecrosis, is gas gangrene. This skeletal muscle infection may be associated with recent surgery or trauma. Anaerobic and facultative bacteria work synergistically. A variant of type I necrotising fasciitis is saltwater necrotising fasciitis in which an apparently minor skin wound is contaminated with saltwater containing a Vibrio species. Varicella infection and the use of non-steroidal anti-inflammatory drugs may be predisposing factors. Usually caused by Clostridium perfringens. When type III necrotising fasciitis occurs spontaneously, Clostridium septicum is more likely to be the etiologic agent; can occur in association with colon cancer or leukemia uncomplicated chicken pox (n Z 65). Association with necrotising fasciitis was made with home childcare, history of asthma, second case within a household and later presentation at their health care provider. 17 Another case control study compared 19 cases of necrotising fasciitis within three weeks of primary varicella infection with 29 cases of uncomplicated chicken pox. After controlling for age, gender and group A streptococcus isolation cases were more likely than controls to have used ibuprofen before hospitalisation. In most children ibuprofen was commenced after diagnosis of the primary infection, those who developed renal insufficiency were more likely to have been treated with ibuprofen. 16 An association between non-steroidal anti-inflammatory drugs (NSAIDs) was also highlighted in a study from the United Kingdom, in which 92% of patients with streptococcal toxic shock syndrome (STSS) were taking NSAIDs at the time of the disease onset. 18 NSAIDs act by reducing the ability of granulocytes to perform chemotaxis, phagocytosis and bacterial killing. It is not clear whether NSAIDs actively participate in the disease or delay the diagnosis and treatment, thus resulting in worse outcome. Necrotising fasciitis is a potentially fatal condition which must be managed promptly including appropriate antibiotics, ITU support and early surgical debridement. The need for surgical intervention is not always clear and explorative surgery must be carried out promptly when there is any clinical suspicion. Diagnosis in children is more difficult because of difficulty eliciting symptoms and signs and because the majority of children do not have premorbid conditions. Delay in diagnosis is not uncommon: delayed diagnosis was found in over half the patients treated for necrotising fasciitis in one retrospective review. 18 patients treated for necrotising fasciitis caused by Group A haemolytic streptococcus. Although the evidence against ibuprofen is limited its use may allow progression of the disease process by suppressing the inflammatory response to streptococcus and therefore should be avoided in suspected necrotising fasciitis. Conflict of interest None. Funding None. References 1. Donnelly L, Frush D, O Hara S, et al. Necrotising fasciitis: an atypical cause of acute abdomen in an immunocompromised child. Pediatr Radiol 1998;28:109e Becker M, Zbaren P, Hermans R, et al. Necrotising fasciitis of the head and neck: role of CT in diagnosis and management. Radiology 1997;202:471e6. 3. Chao H, Kong M, Lin T. Diagnosis of necrotising fasciitis in children. J Ultrasound Med 1999;18:277e Cardinal E, Bureau N, Aubin B, et al. Role of ultrasound in musculoskeletal infections. Radiol Clin North Am 2001;39: 191e9. 5. Wilson HD, Haltalin KC. Acute necrotising fasciitis in childhood: report of 11 cases. Am J Dis Child 1973;125:591e5. 6. Rouse TM, Malangoni MA, Schulte WJ. Necrotising fasciitis: a preventable diaster. Surgery 1982;92:765e Majeski JA, Alexander JW. Early diagnosis, nutritional support, and immediate extensive debridement improve survival in necrotising fasciitis. Am J Surg 1983;145:784e7. 8. Freischlag JA, Ajalat G, Busuttil RW. Treatment of necrotising soft tissue infections: the need for a new approach. Am J Surg 1985;149:751e5. 9. Nathan S, Pang A, Singh Sidhu D, et al. Necrotising soft tissue infections as a complication of chicken pox. Singapore Med J 1995 December;36:656e Moss R, Musemeche C, Kosloske A. Necrotising fasciitis in children: prompt recognition and aggressive therapy improve survival. J Pediatr Surg 1996;31:1142e Reingold AL, Hargrett NT, et al. Toxic shock surveillance in the United States, 1980 to Ann Intern Med 1982;96:875e Barnham M, Weightman N, Anderson A, et al. Streptococcal toxic shock syndrome: a description from North Yorkshire, UK. Clin Microbial Infect 2002;8:174e Zerr D, Alexander E, Duchin J, et al. A case-control study of necrotising fasciitis during primary varicella. Paediatrics 1999 April;4:783e Stevens D. Streptococcal toxic shock syndrome associated with necrotising fasciitis. Ann Rev Med 2000;51:271e Clark P, Davidson D, Letts M, et al. Necrotising fasciitis secondary to chicken pox infection in children. Can J Surg 2003 February;46:9e14.

5 Necrotising fasciitis Waldhausen J, Holterman M, Sawin R. Surgical implications of necrotising fasciitis in children with chicken pox. J Pediatr Surg 1996 August;31:1138e Peterson C, Vugia D, Meyers H, et al. Risk factors for invasive group A streptococcal infections in children with varicella: a case control study. Pediatr Infect Dis J 1996 February;15: 151e Brogan T, Nizet V, Waldhausen J, et al. Group A streptococcal necrotising fasciitis complicating primary varicella: a series of fourteen patients. Pediatr Infect Dis J 1995 July;14:588e94.