Necrotizing fasciitis in a pediatric patient: Successful management in the inguinal area - A case report

Abstract

Introduction

Necrotizing fasciitis (NF) is a rare life-threatening medical and surgical emergency. We present a case of a pediatric patient diagnosed with necrotizing fasciitis in the inguinal area which required debridement and post-operative management of the residual wound using a combination of wound closure techniques.

Case presentation

A 10-months-old girl with a history of Food-protein induced enterocolitis syndrome (FPIES) presented with septic shock and ulcerative lesions of the inguinal area. She was admitted to the Intensive Care Unit (ICU) of our hospital, antibiotic treatment was administered and a diverting colostomy was performed. Serial surgical debridement of the affected tissues resulted in an extensive tissue deficit. Management of the residual skin and soft tissues deficit included application of Negative Pressure Wound Therapy (NPWT) with Vacuum-Assisted Closure (VAC) and was followed by Oasis® extracellular matrix (ECM) graft placement. After 65 days of hospitalization, the wound surface was completely epithelialized.

Discussion

A challenging aspect of NF management is the closure of the residual skin and soft tissue deficit after surgical debridement. Considering the patient's characteristics, a less invasive reconstructive technique was sought. Application of NPWT followed by ECM graft placement are two effective options that can be combined in different stages of wound healing.

Conclusions

The management of the residual wound after surgical debridement of the affected tissue in NF requires a patient – specific approach and constant reevaluation of the management plan. NPWT and ECM graft placement can significantly contribute to wound closure and epithelization of the residual deficit in children.

Highlights

• •NF requires timely extensive surgical debridement.
• •Management of the tissue deficit is challenging.
• •Combination of wound closure techniques is favorable.
• •NPWT had a positive outcome in a pediatric patient with NF in the inguinal area.
• •ECM graft promoted epithelization of an extensive wound area.

1. Introduction

Necrotizing fasciitis (NF) is a rapidly progressive infection of the skin and subcutaneous tissue, extending to the fascia and adjacent muscle. The estimated incidence is 0.08 per 100,000 children per year but the mortality and morbidity remain high because of the simultaneous rapidly progressive nature of the disease and the delay of diagnosis [1]. Imaging and laboratory tests might be useful tools to increase suspicion for NF, but the diagnosis remains clinical [2]. Risk scoring systems for NF in pediatric patients, such as Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) in adults are not applied in pediatric patients [3].

NF most commonly presents in children with malnutrition, immunosuppression, sepsis, skin infections, concurrent use of nonsteroidal anti-inflammatory drugs and malignancy [4]. The underlying cause has been attributed to infection due to one or multiple bacteria such as Staphylococcus aureus, Streptococcus pyogenes, Enterococci, Escherichia coli, Pseudomonas aeruginosa and Klebsiella [1].

NF is considered a medical and surgical emergency [5]. Management of NF includes hemodynamic support, immediate initiation of empirical antibiotic treatment with a broad-spectrum β-lactam, clindamycin and an anti-MRSA antibiotic and aggressive surgical debridement of all necrotic and at-risk appearing tissue [6]. When the perigenital or perineal area is affected, a diverting colostomy is deemed necessary [5].

After the control of necrosis, optimal wound care should be prioritized. Management options for extensive wound management after full-thickness debridement include Negative Pressure Wound Therapy (NPWT) with Vacuum-Assisted Closure (VAC) application and Hyperbaric Oxygen (HBO) use [5]. In cases of extensive skin deficit, skin grafts, flaps, or free flaps should be considered but in pediatric patients with limited donor-site availability, alternative skin and tissue scaffolding methods like extracellular matrix graft application are suggested as wound care options [7].

We present a case of a 10-months-old female patient with NF of the inguinal area which was subjected to necrotic tissue debridement resulting in an extensive skin and soft tissue deficit. A combination of wound closure methods was included in the wound management plan. This case was reported in accordance with the SCARE guidelines and is compliant with the SCARE criteria [8].

2. Case presentation

A 10-months-old female from the Gypsy Roma Traveler (GRT) community presented with fever, vomiting and voice hoarseness and was admitted to the Intensive Care Unit (ICU) of our hospital. 4 days ago, she underwent Oral Food Challenge (OFC) with extensively hydrolized milk. She had history of hospitalization 3 months ago due to diarrhea, vomiting and flatulence after milk intake. The symptoms' pattern set the clinical diagnosis of atypical Food Protein-Induced Enterocolitis Syndrome (FPIES). A complete laboratory evaluation had been carried out to detect a possible IgE-mediated food allergy and to investigate the immunologic profile of the patient due to suspicion of an underlying immunodeficiency but came out negative.

The patient appeared dehydrated, lethargic with multiple rashes of the inguinal area, exudative lesions of the oral cavity, anuria, hepatosplenomegaly and abdominal distention. The diagnosis of septic shock was set. The laboratory work-up at the time of admission is presented in Table 1. During the following days of hospitalization, the ulcerative lesions of the inguinal area were aggravated and signs of necrotic tissue appeared, setting high suspicion for a necrotizing soft tissue infection (Fig. 1A).

Table 1.

Patient's laboratory work-up results on admission.

Test	Value	Laboratory reference range
WBC	0.7 × 10^3/μl	4.5–13.5 × 10^3/μl
NE	0.04 × 10^3/μl	1.5–8.0 × 10^3/μl
LY	0.6 × 10^3/μl	1.5–6.5 × 10^3/μl
PLT	205 × 10^3/μl	130–400 × 10^3/μl
CRP	165 mg/l	<5 mg/l
Glu	261 mg/dl	70–100 mg/dl
Blood urea	49 mg/dl	5–45 mg/dl
K	2.7 mEq/l	3.5–5.5 mEq/l
Na	133 mEq/l	134–148 mEq/l
Serum protein	4.9 g/dl	5.9–8.0 g/dl
Serum albumin	2.7 g/dl	3.8–5.4 g/dl

WBC: White blood cell count, NE: neutrophil count, LY: lymphocyte count, PLT: platelet count, CRP: C-reactive protein, Glu: blood glucose, K: serum potassium, Na: serum sodium.

Fig. 1.

Necrotic area before (A) and after (B) the first surgical debridement.

The blood cultures and lesion cultures were both positive for Staphylococcus aureus and Pseudomonas aeruginosa and the Film Array respiratory panel assay revealed the simultaneous presence of Adenovirus, Enterovirus and Rhinovirus.

2.1. Management

The patient was intubated and initially treated with intravenous antibiotics (meropenem, vancomycin), antifungal (fluconazole) and antiviral (acyclovir) agents. Intravenous immunoglobulin (IVIG) of 2 g/kg was administered, and methylprednisolone tapering schedule was initiated.

During the 5th day of hospitalization in the ICU, serial surgical debridement of the affected perigenital and perineal areas was decided and a diverting sigmoid colostomy was performed to avoid contamination. The ulcerative lesions extended beyond the skin to the subcutaneous tissue, fascia planes and adjacent muscles and the diagnosis of NF was confirmed (Fig. 1B). The procedure was followed by Negative Pressure Wound Therapy (NPWT) with Vacuum-Assisted Closure (VAC) at the 17th day of hospitalization. The continuous suction pressure applied was 30 mmHg and VAC dressings were changed when deemed necessary. During the 31st day of hospitalization the patient was afebrile, hemodynamically stable and the criteria of ICU discharge were met. The previous antibiotic combination treatment was terminated, and amikacin was initiated instead as the cultures revealed an increased bacterial growth of Pseudomonas aeruginosa and Proteus mirabilis, sensitive to amikacin. The NPTWT was paused after 3 weeks, and the new bacterial cultures obtained came out negative. The clinical presentation of the lesion was improved, hypergranulation tissue was formed in the wound bed, the borders of healthy skin formation were still deviated and the granulation tissue appeared friable in specific areas. (Fig. 2A–B).

Fig. 2.

Improved lession fotos after Negative Pressure Wound Therapy (NPWT) with Vacuum-Assisted Closure (VAC).

The decision to apply a layer of Oasis® extracellular matrix (ECM) graft in the wound bed was made taking into consideration the extent of the wound bed and skin deficit and the limited options of skin graft donor sites. The patient underwent surgical wound debridement, excision of hypergranulation tissue seen in Fig. 3A and application of one layer of ECM [9]. After application, the graft was cleansed with normal saline solution according to the instructions for use. The wound and the graft were covered with non-adherent gauze dressings precoated with Vaseline and flexible gauze roll bandage to ensure adequate moisture, sterility, and stable covering. The wound remained closed for the next 3 days, when the dressings were changed and the outcome on the wound appearance is shown in Fig. 3B.

Fig. 3.

Wound before (A) and 1 week after Oasis® extracellular matrix (ECM) graft placement.

2.2. Outcome and follow-up

The patient was afebrile, with no clinical signs of infection and the laboratory inflammation markers were gradually decreased. The appearance of the wound surface on the 74th day of hospitalization (Fig. 4A) was remarkably improved; the wound margins were completely closed, re-epithelialization of wound surface was present and the pigmentation of the wound area resembles to pigmentation of healthy skin surface. Our patient was discharged. Topical application of hydrating cream was advised.

Fig. 4.

Appearance of the wound surface on the 74th day of hospitalization (A) and 24 days post - discharge (B).

The epithelialized wound surface 24 days post - discharge is shown in Fig. 4B. The scar was evaluated using the modified Vancouver Scar Scale (mVSS) with a score of 3/16 (vascularity: 1, pigmentation: 1, pliability: 1, height: 0, pain: 0, pruritus: 0). This modified scale was used for semiquantitative assessment of the scar considering the patient's pigmentation as well [10].

The patient is followed by our surgical team with a monthly appointment at the outpatient clinic. Topical application of hydrating cream and suncream 50+ was advised for the following 6 months to reduce the possibility of keloid formation and the need of a reoperation.

3. Discussion

The available data on treatment of NF is limited because of the low incidence of the disease and the small number of reported cases. The most crucial factor that decreases morbidity and mortality is the immediate surgical intervention with aggressive debridement of non-viable necrotic tissue. This often entails surgical excision of extensive tissue and may result in disability, disfigurement, amputation and skin deficits requiring reconstruction. In the literature there has been also conservative management of NF mentioned but there are only two reports with limited patients and uncertain results [1,15].

In our case we choose the surgical intervention due to the crucial condition of our little patient in ICU. As mentioned in the literature early aggressive surgical treatment is the one key factor to reduce mortality. We performed serial surgical debridement in multiple operations every 48–72 h, in order to remove the whole necrotic area each time. NF is a progressive disease and the necrotic margins change rapidly every day. After 10 days the wound area was clear and the procedure was followed by Negative Pressure Wound Therapy (NPWT) with Vacuum-Assisted Closure (VAC) [1].

A challenging aspect of patient management is closure of the remaining skin and soft tissue defect after surgical debridement. The challenge of this case is that in a pediatric patient with reduced body weight and background of malnutrition, the options for skin graft donor sites were restricted. Furthermore, a flap or autologous skin graft couldn't be included in the management plan and a less invasive wound healing option was examined.

The residual wound healing of extensive skin and soft tissue defects requires a combination of different methods and the management plan should be re-evaluated according to the patient's characteristics and response to treatment. In this patient, we initially used NPWT with VAC application which approximated the viable tissue margins, decreased tissue edema, promoted granulation, and significantly contributed to the restriction of the patient's defect size and depth [11]. It provides a sterile environment with adequate moist that prevents bacterial growth and allowed safe ECM graft application [12].

ECM has been used as a safe and effective alternative scaffolding material for skin and soft tissue defects when skin grafts or flaps are not a favorable option [13]. The ECM graft application provided adequate structural support and contributed as a scaffold for the formation of granulation tissue, epithelialization, and eventual wound closure. It is worth mentioning that there were initial doubts about the safety of the application of a porcine small intestine submucosa (SIS) graft to our patient with a background of FPIES, as it has been associated with high atopic comorbidity and the prospect of susceptibility to the graft's allergens was considered [14]. However, the ECM graft was well-tolerated by our patient with no signs of allergic reaction.

4. Conclusions

Management of rare cases of NF requires timely decision making about aggressive surgical debridement and excision of non-viable tissue as well as antibiotic regimens and potential adjuvant treatment. The closure of the residual wound area with extensive tissue defect is challenging as it requires a combination of various techniques according to the wound location and characteristics and the patient's response to treatment. NPWT is an effective option that has significantly contributed to the elimination of wound infection which made the application of a graft a viable option for further management. The application of ECM graft promotes granulation, epithelialization, and eventual wound closure of extensive wound surfaces and was well-tolerated by a pediatric patient with multiple comorbidities and limited donor-site availability.

Our decision to combine these two different techniques, the NRWT with Vacuum-Assisted Closure (VAC) and ECM graft was really successful for our patient. Our case is the first in the literature that such a combination is used for NF treatment in a child. It provides the best benefit by using the less invasive techniques in our little patients and should be considered as the first treatment choice in such cases in children.

Author contribution

Anna Ntanika: Conceptualized the case report, drafted and revised the manuscript and oversaw data collection and analysis.

Adelais Tzortzopoulou: Revised the manuscript, ensured the accuracy and integrity of the reported work, corresponded with the journal and addressed reviewers' comments.

Ioanna Argyri: Provided specialized knowledge and wrote specific sections of the manuscript.

Artemis Dendi: Provided clinical expertise and assisted with data collection.

Orthodoxos Achilleos: Provided critical feedback and revisions.

Consent

Written informed consent was obtained from the patient's legal guardian for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Parental consent for minors

Written informed consent was obtained from the patient's parents for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Information gathering

Patient information was collected from the patient's medical records' file and was anonymized according to the hospital's policy.

Ethical approval

Ethical approval for this study was provided by the Ethical Committee of P. & A. Kyriakou Children's Hospital on 22 February 2024.

Guarantor

Adelais Tzortzopoulou

Orthodoxos Achilleos

Research registration number

Not applicable.

Funding

None.

Conflict of interest statement

We declare no conflict of interest.