Diagnosis and Treatment of Type II Necrotizing Fasciitis in a Child Presenting With Minor Abrasion, Edema, and Apparent Bruising

A healthy, 14-year-old girl presented with what was initially a minor hand abrasion with edema and apparent ecchymosis. Over the next 12 hours, dusky rings developed over the forearm. Key features leading to diagnosis were pain out of proportion to the injury and a CT scan that was positive for gas in the tissues. Optimal limb salvage was obtained with an integrated, multidisciplinary approach that included hyperbaric medicine, surgery, infectious disease, wound care, rehabilitation services, and behavioral health. Multiple surgeries included debridement, layered matrix dressings, and a split-thickness skin graft. The following case report discusses the etiology, diagnosis, mechanism of injury, and treatment of Type II necrotizing fasciitis. Unique problems that are encountered when treating pediatric patients with this problem are discussed and keys to successful outcomes are proposed.

A healthy, 14-year-old girl presented to the emergency room 10 days after playing the “ABC game.” The game requires the participant to think of words starting with the letter called out by other players while fingernails or other sharp objects are used to scratch the hand. The patient also recalled moving a heavy dresser 2 days earlier, which had fallen on her arm. The morning after moving the dresser she noted a circular bruise on the distal part of her forearm, but had full use of the arm until about 5 pm the night of admission. Her mother brought her to the emergency room just before midnight with an extremely painful and swollen left hand. Examination of the dorsal surface showed a healed 4-cm linear scar, minor erythema, moderate edema, and slight ecchymosis. She was afebrile, plain radiograph did not show any fracture or intraosseous abnormality, and she was admitted to the pediatrics floor. The pediatrician and social worker interviewed the patient and excluded non-accidental trauma. There was no evidence of bruising anywhere else on her body. Initial laboratory findings were: white blood cell count (WBC) of 14.6 K/uL, erythrocyte sedimentation rate (ESR) of 4 mm/h, C-reactive protein (CRP) of .4 mg/dL, partial thromboplastin time (PT) of 13 seconds, D-Dimer 846 ng/mL, and fibrinogen 309 mg/dL. All basic metabolic profile values were normal. Blood pressure was 114/77, respirations were 18 breaths per minute, and pulse was 69. She had developed some nausea and vomiting, but did not appear to be systemically ill or in any pain unless the arm was palpated. Pediatric hematology consultation was obtained. Differential diagnosis included traumatic injury with rapidly spreading hematoma, vasculitis, bleeding disorder, or infection. Vancomycin and ceftriaxone were started and she immediately developed a red-man reaction to the vancomycin with itching and hives. This resolved with Benadryl, and the ceftriaxone was continued. Over the next 12 hours, the patient developed progressive dusky-purple rings over the lower and upper arm (Figure 1). At this time, a CT scan of the arm was taken showing evidence of gas in the tissues. She was emergently taken to the operating room by the orthopedic and plastic surgeons.

Extensive Type II necrotizing fasciitis involving the entire lower arm and the distal half of the upper arm was found (Figure 2). A small amount of “soupy fluid,” but no frank pus, was found. The subcutaneous tissue and skin on the volar aspect of the forearm was thrombosed and nonviable. The muscles, nerves, tendons, and vessels were viable but were enclosed in a thin, adherent, necrotic sheath. The necrotic sheath was very tenacious and had to be scraped and laboriously excised off all the tendons, blood vessels, and nerves over the course of 6 debridements during the first 12 days of hospitalization.

During the fourth debridement (6 days after the first debridement), the portion of the incision from the axilla to the antecubital fossa was able to be stapled closed with a Penrose drain in place. A negative pressure wound therapy (NPWT) device (V.A.C® Therapy, KCI, San Antonio, Tex) was then placed over the remaining forearm wound. She was now left with a deficit from the antecubital fossa to the wrist that included most of the subcutaneous tissue and a section of skin measuring 18 cm x 5 cm.

At the time of the sixth debridement (12 days after the first debridement) the upper arm was deemed totally closed and the Penrose drain was removed. All the necrotic tissue in the lower arm was finally debrided. Most of the muscle was intact, but without subcutaneous tissue or fascial sheaths the tendons were in danger of becoming adherent to one another.

To fill in this large tissue deficit and to prevent the tendons from becoming overly adherent, two bilayer membrane dressing (BMD) sheets (Integra™, Integra Lifesciences, Plainsboro, NJ) were placed in the open forearm. The BMD consists of a bovine collagen matrix that has chondroitin added. One sheet of BMD was wrapped around and between the exposed muscle beds and tendons. A second sheet consisting of the collagen matrix with silicone overlay was placed over the top of the first sheet and the NPWT device reapplied.

Three weeks after the initial BMD was placed (34 days after the first debridement) she was taken back to surgery. About 25% of the BMD was not viable and another sheet containing the BMD with the silicone overlay was placed. A week later, a third BMD sheet was placed so that the defect was filled to the level of the adjacent skin. Sixty days after the original injury (19 days after the third BMD), a split-thickness skin graft was placed over the wound in the forearm. The NPWT was continued for an additional 10 days after split-thickness skin grafting.

Hyperbaric medicine and infectious disease were consulted at the time of her initial operative diagnosis. A Gram stain done during the initial surgery demonstrated rare gram-positive cocci. Imipenem, linezolid, and clindamycin were started postoperatively. Linezolid and ceftriaxone were discontinued when final culture results returned with negative finding. Her ASO titer was 200 IU/mL on postoperative day 5, and negative on postoperative day 19. The infectious disease consultant suggested the inciting organism was probably Group A Streptococcus from the fingernail scratching during the schoolyard game. The imipenem and clindamycin were continued for 21 days. She received her initial hyperbaric oxygen therapy (HBOT) of 100% oxygen at 2.5 atmospheres absolute (ATA) for a total treatment time of 2 hours within 1 hour after the first debridement. The patient was started on twice daily HBOT for the first 10 days, and then changed to daily therapy for an additional 28 days. HBOT was paused until the split-thickness skin grafting after which she had an additional 8 HBOT treatments for a total of 56.

She did lose some dexterity, but retained a functional upper extremity with good cosmetic appearance (Figure 3). Her forearm remains healed with good functionality and appearance 1-year later.

Her care required a team approach that involved surgery, hyperbaric medicine, wound care, infectious disease service, pediatric dietitian, child life specialist, occupational therapy, and physical therapy. Behavioral medicine was consulted when she became significantly depressed at the possibility of limb loss or deformity. She was able to return to school 6 months after injury and is doing well both academically and socially 1-year post injury.

Historically, necrotizing fasciitis was associated with a greater than 60% mortality rate.^1,2 Recent studies show this has dropped to 20%–33%.^1,2 The Centers for Disease Control and Prevention (CDC) estimate there are only 500–1500 cases of necrotizing fasciitis in the United States each year and that most cases involve the elderly, immunocompromised, or diabetic populations. These types of infections are extremely rare in healthy, young adults following minor injury.^3,4 Most cases in children occur after varicella infection.⁵

Necrotizing fasciitis is a general term for a life-threatening, soft tissue infection, but is comprised of three distinct groups.⁵

Most studies describe Type I infections as polymicrobial and proceeded by cellulitis.^2–5 Copious exudate resembling “murky dishwater” is described.

Patients are often septic with severe organ dysfunction. An elevated WBC count (> 15,000), elevated blood urea nitrogen (BUN), and low serum sodium are helpful in diagnosis. These patients have a rapid, fulminant course over hours, not days.⁵

Type II infections are chiefly caused by Group A Streptococcus alone, and Type III usually is caused by marine Vibrio Gram-negative rods. Type II infections are more likely to be found in children and neonates without underlying comorbidities. These patients will have symptoms that develop over several days and may not present with systemic involvement, but might have large areas of skin involved.⁵

The present case is consistent with Type II infection. The purplish-black lesions on the skin represent thrombosis of the skin’s underlying vessels by a platelet/polymorphonuclear leukocyte complex stimulated by extracellular toxins.^6,7 Destruction of tissues and thrombosis is caused both by the toxins, antigens, the enzymes of Group A Streptococcus, and the host response to the antigens. Virulent mechanisms include cell wall attached proteins, proteases, exotoxins, and super-antigens. T-helper lymphocytes are activated, in turn activating cytokines, clotting factors, and complement factors. Cytokines include tumor necrosis factor, interleukin-1β, interleukin-2, and interferon.⁵ This may explain the selective effect on the fascial tissues with sparing of the muscles, tendons, nerves, and deep vessels. Thrombosis (the purplish-black lesions seen in Figure 1) and pain are often seen before gas in the tissues or systemic organ failure. Gas in the tissues is a late finding and may be better appreciated on CT scan than with plain radiographs.

The inciting injury is often minor with scant culture results.⁴ For all types of necrotizing fasciitis the primary presenting symptom is pain out of proportion to examination before the purplish-black skin lesions appear.⁴ In a retrospective study from Singapore, this was present 97.8% of the time.⁸ Warm skin to palpation was present in 96.6% of patients, swelling in 92.1% of patients, and fever in 79.8%.⁸ Only 14.6% of the patients in the study were diagnosed as having necrotizing fasciitis at time of admission. No causative organism was identified in 18% of patients.⁸ Definitive diagnosis is made at the time of surgery for the majority of these patients.

Historically, the treatment of necrotizing fasciitis has included resection of involved tissue or amputation of the limb and intravenous (IV) antibiotics with intensive care support of multi-organ system failure. Until cultures are read, the recommendation is broad-spectrum coverage against gram positive and gram-negative organisms, methicillin resistant staphylococcus and anaerobic organisms. The earlier the treatment can be started, the better the chance for survival.⁸ A few studies have used IV antibiotics with delayed debridement after demarcation of nonviable tissue.⁵ Multiple debridements are usually needed to remove non-viable tissue.

Adjunctive therapies include HBOT and use of IVIG (intravenous immune globulin). Combining HBOT with surgical debridement and IV antibiotics resulted in reduction of mortality rates to 11.9%.⁹ Hyperbaric oxygen at or above 2.5 ATA is the only modality that neutralizes the alpha toxin of clostridium.¹⁰ Hyperbaric oxygen enhances the killing ability of neutrophils, reduces tissue edema, increases antibiotic transmembrane transport via oxygen dependent pathways in tissues with poor blood supply, and increases angiogenesis and fibroblast growth.¹⁰ Hyperbaric oxygen also helps delineate viable from non-viable tissue.¹⁰

Intravenous immune globulin neutralizes the super antigens in Streptococcal toxic shock syndrome.⁵ Intravenous immune globulin also reduces T cells, which decreases tumor necrosis factor (TNF). One study reported a significant decrease in mortality with IVIG.¹¹

The extensive tissue loss due to necrosis and repetitive surgical debridements results in a challenging clinical dilemma as to the best approach for reconstruction. Combining multiple, new technologies and wound care techniques can give better, faster results. The layered applications of BMD (collagen-glycosaminoglycan biodegradable matrix) are an effective strategy to fill large tissue deficits, preserve function, retain maximal functionality, and obtain a good cosmetic result. The concurrent use of HBOT and NPWT have been shown to shorten the time needed to mature the BMD, as well as improve outcome in split-thickness skin grafting.^12,13 In this case, we believe that combining HBOT with the NPWT was responsible for successful healing.

Necrotizing fasciitis among children presents unique problems. Hyperbaric oxygen may require myringotomy to prevent the risk of barotraumas to the child and a family member may need to accompany an anxious child in the hyperbaric chamber. Fear of disfigurement and non-acceptance by peers can cause lifelong anxiety and depression. Such emotions can cause inadequate nutritional intake, nausea, crying at dressing changes or hyperbaric treatments, and/or suicide gestures. The teenage girl expressed that she “would rather die than lose an arm.” It is helpful to have a team approach that includes a dietitian, child life specialist, and psychiatrist. Alleviating pain during a process that involves months of care with multiple surgeries and physical and occupational therapy requires an innovative approach when treating children. New techniques utilize virtual reality therapy and alternatives to narcotics. Judicious use of narcotics will also be incorporated into an individualized plan for each patient.^14,15

Necrotizing fasciitis in children presents several dilemmas. First is the dilemma of diagnosis as often the injury is minor, the patients do not appear febrile or ill, laboratory findings may be initially normal and cultures may be negative. The second dilemma is how much tissue to debride, selection of adjunct therapies and how to combine reconstruction techniques for best cosmetic result in the shortest time. The third dilemma is how to assemble a multidisciplinary team to meet the unique emotional, social, and medical needs of children. Since this case, we have developed a limb salvage team and a comprehensive order, as it is critical that emergency departments quickly recognize necrotizing fasciitis and initiate a rapid, multidisciplinary treatment plan. Despite the challenges of treating pediatric necrotizing fasciitis, a successful outcome can be achieved with prompt multidisciplinary management and a creative approach to reconstruction.

From the Department of Hyperbaric Medicine and Surgery, Intermountain Dixie Regional Health Center, St. George, Utah

The authors thank Dr. William Cobb, Department of Infectious Disease, Intermountain Dixie Regional Medical Center, for his valuable consultation and guidance in the clinical management of this case.

Address correspondence to: Joan Eggert, MD, MPH Dept. of Hyperbaric Medicine Intermountain Dixie Regional Health Center 544 S 400 E St. George, UT 84790 Phone: 453-669-2148 E-mail: lveggert@msn.com