Necrotizing fasciitis in children due to minor lesions

Journal of Pediatric Surgery Case Reports 25 (2017) 52e55

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Necrotizing fasciitis in children due to minor lesions €mpfen Viktoria A. Pfeifle*, Stephanie J. Gros, Stefan Holland-Cunz, Alexandre Ka University Children's Hospital Basel UKBB, Spitalstrasse 33, 4056 Basel, Switzerland

a r t i c l e i n f o

a b s t r a c t

Article history: Received 6 June 2017 Accepted 5 August 2017 Available online 8 August 2017

Introduction: Necrotizing fasciitis is a soft tissue infection that can rapidly progress and end lethally if not treated early and radically. With an extremely low prevalence (0.02% of all pediatric in hospital cases), most physicians will probably only see very few cases during their career. Unlike adult patients, the majority of children affected by this disease are healthy individuals. There is no chronic disease and necrotizing fasciitis often arises from minor lesions. Case presentation: We present two cases treated in our clinic within the past year. Our first case of necrotizing fasciitis was a 5 year old Caucasian male patient with a varicella lesion on the back. The second case, a 4 year old Caucasian male patient, presented after an insect bite at the lower limb. Both cases were triggered by a superinfection after scratching. We describe the clinical findings, difficulties in diagnosis, surgical therapy and outcome. Conclusion: Rapid surgical treatment is necessary to reduce morbidity and mortality in cases of necrotizing fasciitis. Due to the rarity of the disease it is often misdiagnosed by physicians. We emphasize the importance of staying alert and to keep necrotizing fasciitis in mind. © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Keywords: Necrotizing fasciitis Soft tissue infection Pediatrics Varicella

1. Introduction Necrotizing Fasciitis (NF) is a severe form of soft tissue infections, which rapidly spreads from subcutaneous tissue leading to necrosis of the fascia and the surrounding soft tissue [1]. It progresses fast and can lead to septic shock, organ failure and death if not diagnosed and treated early and radically. Treatment consists of fluid resuscitation, broad spectrum antibiotics and most importantly rigorous surgical debridement of infected and necrotic tissue. Delay of surgical therapy for more than 24 h doubles the mortality rate [2,3]. Due to the rarity of this disease with a prevalence of 0.02% in the pediatric population [4] NF is often misdiagnosed as cellulitis. This delays the correct diagnosis and increases the risk of a more fulminant course and a higher morbidity. Local early signs for NF are edema, induration and erythema. Skin necrosis is a late sign [5]. Early clinical findings are typical for septicemia: fever, severe pain, tachycardia and elevated white blood count. NF can be caused by a polymicrobial infection with aerobic and anaerobic organisms such as clostridium, proteus, E. coli, bacteroides and

* Corresponding author. Tel.: þ 41 0041 61 7041212; fax: 0041 61 7041213. E-mail addresses: Viktoria.Pfeifl[email protected] (V.A. Pfeifle), Stephanie.Gros@ukbb. ch (S.J. Gros), [email protected] (S. Holland-Cunz), Alexandre. €mpfen). [email protected] (A. Ka

enterobacteriacea or by a single organism such as group A streptococci [6]. Unlike in adults, NF occurs most often in children that are previously healthy individuals [7]. Cases of NF in children have been reported due to minor lesions, e.g. after circumcision, umbilical vein catheterization [8,9], inguinal hernia repair [10,11] or secondary to varicella infection [5,12e14]. We present two cases of NF in children following minor lesions. 2. Case presentation 2.1. Case 1 The first case is a 5 year old Caucasian male patient that was suffering from varicella infection. 6 days after eruption of the first vesicles a painful superinfection of a lesion on the back (Fig. 1) was treated with local antibiotic ointment. When local infection signs increased the family returned to emergencies. Signs of septicemia were noted (39.3
C, pulse rate 156/min) and the lesion on his lower back had increased in size (10  20 cm). There was crepitation, tender palpation and lymph nodes in his right axilla were swollen. Laboratory findings revealed elevated white blood cell count (WBC) (43,4  109/l) with a neutrophilia and C-reactive protein (CRP) 141 mg/l [<10 mg/l]. Renal function was normal. He had received all vaccinations according to the Swiss Vaccination Program. An ultrasound was performed which revealed a thickening of

http://dx.doi.org/10.1016/j.epsc.2017.08.005 2213-5766/© 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

V.A. Pfeifle et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 52e55

Fig. 1. Preoperative findings of necrotizing fasciitis following varicella infection on the back of a 5 year old boy.

subcutaneous fat tissue without abscess formation. Considering the findings NF was suspected and the patient was debrided. The fascia of the latissimus dorsi appeared edematous with a grey-greenish color and was macroscopically highly suspicious for necrotizing fasciitis. Intraoperative fascial biopsies confirmed cocci-like bacteria typical for necrotizing fasciitis. The entire necrotic fascia was debrided until healthy margins were confirmed by biopsies (Fig. 2). After antiseptical irrigation a vacuum wound dressing was established. Postoperatively the patient was monitored on the pediatric intensive care unit. One blood conserve was transfused after a loss of 26 g/l, otherwise the patient stabilized rapidly and a second look 24 h later confirmed no further progression of the disease. Two dressing changes later, ten days after first presentation, a split thickness skin graft (STSG) closed the wound. Initial broad band antibiotic Amoxicillin/Clavulanic acid i.v. and Clindamycin i.v. could be changed to Penicillin 1G i.v. when final results of the tissue culture revealed penicillin sensitive streptococcus pyogenes. 16 days after admission these could be stopped and the patient was discharged with a normal WBC and CRP (Fig. 3). Blood culture from time of admission remained negative. Histology from the biopsies revealed a necrotizing process with acute inflammation and abundant gram positive cocci. Subsequent follow-ups showed a complete recovery. Scars are being corrected by tissue expansion and excision of the STSG one year postoperatively.

Fig. 2. Intraoperatively the entire necrotic fascia was debrided.

hyperdens in the subcuticular fat with hyperemia without collection. With a suspected diagnosis of cellulitis he was admitted to the ward and antibiotic treatment with Amoxicillin/Clavulanic acid i.v. was started. After four hours his clinical condition detoriated and he was presented to the surgeon on call. Necrotizing fasciitis was suspected and he was immediately taken to the operating room for surgical debridement. Antibiotic treatment was extended and intraoperative findings confirmed NF. A second look 24 h post primary intervention revealed a further small part of the fascia that appeared to be infected and this was resected accordingly. The following days the wound was regularly washed with Prontosan (polihexanid and betain). On the 3rd postoperative day a vacuum dressing was applied. Streptococcus pyogenes were isolated from culture and antibiotic treatment could be narrowed to Amoxicillin/ Clavulanic acid and stopped at discharge. On day 12 the wound was closed by secondary closure and STSG. Laboratory findings normalized until discharge on the 15th postoperative day (Fig. 4). The follow up visits showed a full recovery of gait. Scar corrections including STSG excision are considered for the future, but not essential. 3. Discussion Necrotizing fasciitis is a dangerous and severe infection, in which early diagnosis and correct immediate treatment are of utmost importance to limit morbidity and mortality.

2.2. Case 2 The second case of necrotizing fasciitis that presented in our clinic within the same year was a previously healthy 4 year old Caucasian male patient. He presented at the emergency department with high fever (40
C) and a progressive redness and swelling at the right lower leg. He remembered being bitten by an insect, most probably a mosquito a few days earlier. The day before admission his pediatrician had started an antibiotic treatment with Amoxicillin/Clavulanic acid after blood test revealed an extremely elevated CRP (332 mg/l). At admission he presented with a septicemia (pulse 120/min, blood pressure 111/47 mmHg, respiratory rate of 28/min) and progression of local infectious findings, including now the whole lateral lower leg. His parents reported that he had received all vaccinations according to the recommended Swiss vaccination program. Laboratory findings showed a WBC of 10.58  109/l and CRP of 514 mg/l. An ultrasound was

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Fig. 3. Timeline of clinical course and treatment of case 1.

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V.A. Pfeifle et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 52e55

Fig. 4. Timeline of clinical course and treatment of case 2.

Since NF is a rare disease in children it is therefore often misdiagnosed. The most frequent misdiagnosis is cellulitis, but it is also the most frequent differential diagnosis. Fustes-Morales et al. could show that in a review of 39 children affected by NF, 59% of the cases were misdiagnosed with cellulitis [4]. In our cases the initial diagnoses were superinfection of varicella lesion and cellulitis after insect bite. In the initial stage of NF, it is very difficult to distinguish between cellulitis or superinfection and NF. Clinical signs of NF include swelling, erythema, edema and induration e all of which are nonspecific. Crepitation is regarded as a more typical clinical sign of NF resulting from the gas produced by the bacteria. In our first case crepitation was present, however in many cases of NF it cannot be observed. In a series of 11 cases of postoperative NF, only 2 presented with crepitation [11] and in the most recent review of pediatric NF comprising 53 patients, crepitation was not mentioned as an initial finding, but tenderness in 16 cases [15]. “Pain out of proportion” is often stated as a typical clinical sign of NF. We could also observe this in our two cases, as both of the children complained of heavy pain that could usually not be correlated to the local findings. However, the most important indicator for necrotizing fasciitis in children and adults remains rapid clinical deterioration towards septic shock. Typically, minor skin lesions or trauma are the starting point of NF. In neonates usually omphalitis, circumcision and placement for electrodes and catheters are the underlying triggers for NF [7,16,17]. In our patients it was a mosquito bite and a varicella vesicle. Waldhausen et al. reviewed parameters of 30 patients with varicella for whom there was concern for NF. The authors tried to identify parameters to differentiate patients that have an uncomplicated superinfection to patients that developed NF and needed surgery. The authors found, that patients with varicella who present two or three days after the outbreak of the disease with fever, tachycardia, elevated leucocyte and an erythematous, indurated lesion with severe pain are at high risk of developing NF and therefore they recommend immediate surgical exploration. The authors specifically show that out of the investigated parameters increased leucocytes was the most predictive indicator to distinguish between the misdiagnosis of cellulitis and NF [5]. We could also observe this in our first case, where the patient with varicella infection had an extremely high WBC of 43,4  109/l. Overall laboratory findings to our mind are not the most relevant parameters to decide whether NF is to be suspected or not. They might be false negative in light of a rapidly evolving disease and might lull the treating team into wrong security.

Once NF is suspected, the patient should be taken to theatre for surgical exploration. Typical intraoperative findings include a grayish, non-bleeding subcutaneous tissue with the necrotic tissue being easily stripped off from the fascia [15]. The fascial infection leads to thrombosis in skin nourishing perforators which then leads to patchy skin necrosis. Diagnosis is made on account of surgical findings but can be confirmed by intraoperative fascial biopsies. During initial surgery it is important to excise all necrotic and infected tissue. Postoperatively, patients most often need monitoring on an intensive care unit for optimal support until systemic recovery. Broad spectrum antibiotic treatment should be continued until results of culture of the lesion confirm the causative bacteria to securely cover type II NF. As far as the causative bacteria is concerned, there are major differences in the adult and pediatric population. In general, NF can be caused by poly- or monomicrobial infections. The most common type in adults is a polymicrobial infection with anaerobic and aerobic organisms, such as clostridium, proteus, E. coli, bacteroides and enterobacteriacea [3,6,10,18].Unlike in adults, NF in the pediatric population is usually a monomicrobial infection [19]. Besides Streptococci and Staphylococci being the most frequent bacterial genera, Pseudomonas aeruginosa was the third most frequently reported single causative organism [15]. We could also observe this in our two cases. In both cases Streptococcus pyogenes was isolated. As in one of our cases varicella can be the starting point for NF in children we reviewed the literature more thorough regarding this problem. A recent review of the epidemiology of varicella confirmed that over 90% of seropositive tested persons are younger than 10 years of age [20]. The disease process is usually mild and self-limiting with initial fever, a characteristic rash with vesicles and pruritus. Complications like bacterial superinfection of the vesicular lesion, but also pneumonia, encephalitis, toxic shock syndrome and necrotizing fasciitis have been published [20]. Concerning necrotizing fasciitis, a recently published review of 10 pediatric cases in a university hospital in Switzerland revealed that the majority of initial lesions were caused by Varicella, mostly on the trunk [21]. It is also known, that children who have recently been infected by varicella have a 58 times increased risk of invasive group A streptococcal disease [22]. Severe complications may lead to hospitalisation. In Switzerland, a hospitalisation rate of 130/ 100000 cases of varicella in children is reported [23]. In Germany the rate is 271/100000 of varicella cases [24]. To reduce morbidity and mortality of complications from varicella infection, a vaccine was developed. The first varicella vaccination program was established in the USA in 1995. As a consequence, disease burden has significantly decreased. In Germany the vaccine was introduced in 2004. The introduction of a universal vaccination program showed epidemiological impact and decreased the number of varicella infections by 63% and varicella-associated complications by 81% [20]. In Switzerland, varicella vaccination is recommended in immunocompromised individuals, young adults and health care workers, that have not had varicella infection before [25]. With increasing incidence of invasive group A streptococci infections [12,26] it is to discuss if varicella vaccination programs should be universally implemented. At least in one of our cases NF might have been avoided. Insufficient treatment of NF may lead to severe morbidity and could end lethally. Mortality was reported most often as a result of sepsis, disseminated intravascular coagulation (DIC) or organ failure [16]. The mortality rate of NF in adults is reported to be higher with a cumulative rate of 35% (6%e76%) than in the pediatric population, where a rate of as low as 5.4% was reported [27]. However, in the neonatal period the mortality rate is again higher with a reported range of 36%e88% [7,22,23]. In conclusion, physicians need to be aware of the life-

V.A. Pfeifle et al. / Journal of Pediatric Surgery Case Reports 25 (2017) 52e55

threatening disease of necrotizing fasciitis. Children that present with septicemia, local swelling, erythema and pain that is out of proportion to the clinical findings should be observed closely. If varicella infection is present suspicion should be even greater. Misdiagnosing necrotizing fasciitis as cellulitis is common and delays the correct treatment. Once NF is suspected, we recommend early surgical exploration and fascial biopsy which would be followed by radical debridement if necessary.

[3]

[4]

[5] [6]

Ethics approval [7]

N/A. [8]

Consent to participate N/A. Consent for publication Written informed consent was obtained from the patient's legal guardian(s) for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

[9] [10]

[11] [12] [13]

[14]

Availability of data and material

[15]

Data sharing not applicable to this article as no datasets were generated or analysed during the current study.

[16] [17]

Competing interests

[18]

The authors declare that they have no competing interests. Funding

[19]

[20]

We received no funding. [21]

Authors' contributions [22]

VP reviewed the data and literature and wrote the manuscript as first draft. SG gave support in writing the manuscript. SHC and AK gave intellectual input and revised the manuscript. All authors read and approved the final manuscript. Acknowledgements N/A.

[23]

[24]

[25] [26]

References [27] [1] Bisno AL, Stevens DL. Streptococcal infections of skin and soft tissues. N Engl J Med 1996;334(4):240e5. [2] Barton LL, Jeck DT, Vaidya VU. Necrotizing fasciitis in children: report of two

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cases and review of the literature. Arch Pediatr Adolesc Med 1996;150(1): 105e8. Childers BJ, Potyondy LD, Nachreiner R, Rogers FR, Childers ER, Oberg KC, et al. Necrotizing fasciitis: a fourteen-year retrospective study of 163 consecutive patients. Am Surg 2002;68(2):109e16. Fustes-Morales A, Gutierrez-Castrellon P, Duran-Mckinster C, OrozcoCovarrubias L, Tamayo-Sanchez L, Ruiz-Maldonado R. Necrotizing fasciitis: report of 39 pediatric cases. Arch Dermatol 2002;138(7):893e9. Waldhausen JH, Holterman MJ, Sawin RS. Surgical implications of necrotizing fasciitis in children with chickenpox. J Pediatr Surg 1996;31(8):1138e41. Barnham MR, Weightman NC, Anderson AW, Tanna A. Streptococcal toxic shock syndrome: a description of 14 cases from North Yorkshire, UK. Clin Microbiol Infect 2002;8(3):174e81. Abbott RE, Marcus JR, Few JW, Farkas AM, Jona J, iz-Maldonado R. Necrotizing fasciitis in infancy: an uncommon setting and a prognostic disadvantage. J Pediatr Surg 1999;34(9):1432e4. Goldberg GN, Hansen RC, Lynch PJ. Necrotizing fasciitis in infancy: report of three cases and review of the literature. Pediatr Dermatol 1984;2(1):55e63. Weinberger M, Haynes RE, Morse TS. Necrotizing fasciitis in a neonate. Am J Dis Child 1972;123(6):591e4. Moss RL, Musemeche CA, Kosloske AM. Necrotizing fasciitis in children: prompt recognition and aggressive therapy improve survival. J Pediatr Surg 1996;31(8):1142e6. Farrell LD, Karl SR, Davis PK, Bellinger MF, Ballantine TV. Postoperative necrotizing fasciitis in children. Pediatrics 1988;82(6):874e9. Clark P, Davidson D, Letts M, Lawton L, Jawadi A. Necrotizing fasciitis secondary to chickenpox infection in children. Can J Surg 2003;46(1):9e14. Kwak BO, Lee MJ, Park HW, Song MK, Chung S, Kim KS, ldonado R. Necrotizing fasciitis and streptococcal toxic shock syndrome secondary to varicella in a healthy child. Korean J Pediatr 2014;57(12):538e41. Shirley R, Mackey S, Meagher P. Necrotising fasciitis: a sequelae of varicella zoster infection. J Plast Reconstr Aesthet Surg 2011;64(1):123e7. chal A, Kaiser P, Szavay P. Diagnosis and treatment of peZundel S, Lemare diatric necrotizing fasciitis: a systematic review of the literature. Eur J Pediatr Surg 2017 Apr;27(2):127e37. Nazir Z. Necrotizing fasciitis in neonates. Pediatr Surg Int 2005;21(8):641e4. Hsieh WS, Yang PH, Chao HC, Lai JY. Neonatal necrotizing fasciitis: a report of three cases and review of the literature. Pediatrics 1999;103(4):e53. Wong CH, Chang HC, Pasupathy S, Khin LW, Tan JL, Low CO. Necrotizing fasciitis: clinical presentation, microbiology, and determinants of mortality. J Bone Jt Surg Am 2003;85-A(8):1454e60. €l-Kologlu M, Yildiz RV, Alper B, Yag murlu A, Ciftçi E, Go € kçora IH, et al. Bingo Necrotizing fasciitis in children: diagnostic and therapeutic aspects. J Pediatr Surg 2007;42(11):1892e7. Helmuth IG, Poulsen A, Suppli CH, Mølbak K. Varicella in Europe-A review of the epidemiology and experience with vaccination. Vaccine 2015;33(21): 2406e13. Rüfenacht MS, Montaruli E, Chappuis E, Posfay-Barbe KM, La Scala GC. Skinsparing debridement for necrotizing fasciitis in children. Plast Reconstr Surg 2016;138(3). 489e-97e. Laupland KB, Davies HD, Low DE, Schwartz B, Green K, McGeer A. Invasive group A streptococcal disease in children and association with varicella-zoster virus infection. Ontario Group A Streptococcal Study Group. Pediatrics 2000;105(5):E60. Bonhoeffer J, Baer G, Muehleisen B, Aebi C, Nadal D, Schaad UB, et al. Prospective surveillance of hospitalisations associated with varicella-zoster virus infections in children and adolescents. Eur J Pediatr 2005;164(6):366e70. Liese JG, Grote V, Rosenfeld E, Fischer R, Belohradsky BH, v Kries R, et al. The burden of varicella complications before the introduction of routine varicella vaccination in Germany. Pediatr Infect Dis J 2008;27(2):119e24. Heininger U, Seward JF. Varicella. Lancet 2006;368(9544):1365e76. Tyrrell GJ, Lovgren M, Kress B, Grimsrud K. Varicella-associated invasive group A streptococcal disease in Alberta, Canadae2000-2002. Clin Infect Dis 2005;40(7):1055e7. Eneli I, Davies HD. Epidemiology and outcome of necrotizing fasciitis in children: an active surveillance study of the Canadian Paediatric Surveillance Program. J Pediatr 2007;151(1):79e84. 84 e1.