Paediatric Fournier gangrene: comprehensive insights into diagnosis, management, and outcomes—a narrative review
Introduction
Fournier gangrene (FG) can be defined as a critical, life-threatening, and rare condition that generally affects the genital, perineal, and abdominal regions and is characterized by the presence of necrotizing fasciitis. FG typically involves the onset of tissue necrosis and is accompanied by the onset of severe pain, as well as systemic symptoms. French surgeon Jean-Alfred Fournier was the first to describe FG in 1883 and initially suggested a link between FG and urethral infections as well as scrotal conditions (1). Thereafter, clinical progress over the years enhanced our understanding of FG, contributing to its recognition as a polymicrobial infection, and drawing a correlation between FG and other chronic conditions such as diabetes, trauma, and immunosuppression (1-4). FG has been predominantly reported in adult males, with only a few incidences among the pediatric population. Nevertheless, despite the rare occurrence of pediatric FG, several cases have been reported in both prepubescent boys as well as girls (1). Various factors have been linked to the prevalence of FG in pediatric patients, including the presence of infections, congenital abnormalities, and surgical complications. Due to the rarity of the condition in the pediatric population, there is limited available literature to determine the prevalence of FG in prepubescent individuals. However, FG remains a crucial aspect to assess pediatric emergency care (5-9).
FG in patients has been characterized by swelling, excruciating pain, and regions of discoloration around the affected areas of skin. FG has been associated with rapid prognosis, resulting in significant morbidity and mortality rates, if not managed cautiously. Consequently, timely diagnosis and prompt medical intervention are essential for favorable medical care and patient outcomes. The initial management of FG involves intravenous administration of broad-spectrum antibiotics followed by immediate surgical resection of necrotic tissue. Considering the high necrotic activity at the infected areas and the associated severity, FG requires early diagnosis and focal recognition of clinical presentation and associated risk factors. A prompt clinical recognition combined with immediate surgical intervention can help in reducing the incidence of disease-related complications and improve survival outcomes (5,10-14). This article is presented in accordance with the Narrative Review reporting checklist (available at https://tp.amegroups.com/article/view/10.21037/tp-2025-112/rc).
Methods
A comprehensive review of pediatric FG was conducted on the existing data on its strategies, clinical features, complications, treatments, and outcomes. A detailed literature search was done on databases of PubMed, Scopus, and Web of Science with keywords such as “Fournier gangrene”, “Pediatric Fournier gangrene”, “Fournier gangrene in children”, and “necrotizing soft tissue infections (NSTI)”. Due to the limited pediatric data, adult FG data were used to explain the diagnostic scoring system, treatment strategies, and pathophysiology. The Infectious Diseases Society of America (IDSA) and the Surgical Infection Society (SIS) were also consulted (15,16). The search strategy summary is presented in Table 1.
Table 1
Items | Specification |
---|---|
Date of search | Dec 1, 2024 |
Databases and other sources searched | PubMed, Scopus, and Web of Science |
Search terms used | Fournier gangrene, Pediatric Fournier gangrene, Fournier gangrene in children, Necrotizing soft tissue infections (NSTI) |
Timeframe | December 2005 to December 2024 |
Inclusion criteria | (I) Fournier gangrene studies using machine learning methods with a population of pediatrics, and (II) English-language articles |
Selection process | To ensure the accuracy of the investigation, K.A.A. meticulously screened the retrieved papers. K.A.A. assessed the titles and abstracts to determine the eligibility of articles in relation to predetermined inclusion and exclusion criteria |
Etiology and pathophysiology
FG is a form of necrotizing fasciitis initially identified as an idiopathic entity, but diligent studies have shown the primary site of infection to be the perineum, genital, and abdominal areas (17-19). FG can be best defined as a severe soft tissue infection characterized by necrosis of the perineal wall and areas around the genitals, with a rapid prognosis. The infection exhibits predominance in adults and is rarely observed in infants. Even though the prevalence rate is low in the pediatric population, it remains crucial to comprehend the underlying complex etiology and pathophysiological mechanism of FG in infants for early diagnosis and effective management. The pathogenesis of FG is characterized by synergism existing between the polymicrobial population colonizing the perineal and genital areas, aided by the host factors, triggering rapid tissue destruction and systemic infection. Mostly microorganisms belonging to bacterial species Staphylococci, Clostridium, Streptococci, Klebsiella, and Bacteroides are commonly isolated from the infection site. Synergism between the polymicrobial population initiates a series of reactions at the infection surface, causing thrombosis, hypoxia, and necrosis of fascial barriers, further promoting the infection.
Underlying causes and risk factors
FG has been observed to occur more often in infants with existing congenital abnormalities of the genitourinary and gastrointestinal systems, primarily due to higher risks from infection-prone environment. A recent report from Setiawan et al. (2024) has observed the early onset of FG in a 1-month-old male infant with an existing perforation in the transverse colon (20). The clinical presentation of the patients involved the presence of scrotal erythema, areas of necrosis, and pus accumulation in the umbilical cord (20). FG has been predominantly observed in immunocompromised infants due to underdeveloped immune systems. Various past studies have also concluded that infants with skin conditions such as dermatitis or trauma have a higher risk of developing FG. Hartoyo et al. (2022) and Delport et al. (2020) have reported the onset of FG in a 30-day-old infant and a ten-month-old baby with a medical history of diaper rash (21,22). Moreover, infant patients with preexisting systemic or local skin infections are presented to be at high risk of developing necrotizing fasciitis. The situation is further aggravated by the presence of medical conditions such as diabetes, endocrine abnormalities, and metabolic disorders. Pajares et al. reported such an occurrence of FG in an 11-year-old boy with a previous medical history of hyperimmunoglobulinemia E (23).
Pathogenesis in pediatric patients
The severity of the infection is determined by the status of the host’s immunity and the virulence of the pathogenic microorganisms. The underdeveloped immune defense mechanism in infants presents a favorable environment for the entry and propagation of pathogenic bacteria, causing a rapid spread of infection. FG in infant stages involve the entry of pathogenic bacteria into the subcutaneous tissue through skin injuries, infection, or congenital defects. Other most commonly reported infection sources are abscesses in the perirectal, perianal, scrotal, and ischiorectal regions and urinary tract infections, promoting the development of FG. The microorganisms are polymicrobial, with a mixed composition of aerobic and anaerobic bacteria that act synergistically in the rapid spread of the infection. The synergism in the release of toxins and enzymes that stimulate thrombosis and coagulation of the vessels leads to hypoxic tension in the surrounding tissues. The hypoxic stress in the tissues aids in the multiplication of anaerobic and aerophilic microorganisms, causing widespread necrosis (24-27). The infection progresses rapidly in infants who lack a well-defined tissue barrier and have immature immune systems. The aggressive nature of the disease, coupled with a weaker immune defense mechanism, can result in septic shock and aggravated outcomes such as multiple organ failure if not promptly diagnosed and managed with medical intervention (28-31).
Differences in etiology compared to adults
The etiology of FG in adults is often associated with metabolic and endocrine disorders and other health conditions such as diabetes, alcoholism, and immunosuppression. Comparatively, in infants, it is mostly found to be linked to congenital anomalies. In recent studies reported by Padilla et al. (2020) and Setiawan et al. (2024), it was observed that the occurrence of FG in a child and a 1-month-old infant was associated with a pre-existing steroid-resistant nephrotic syndrome and anasarca or presence of perforated transverse colon (20,32). The disease prognosis is more aggressive in infants who have a relatively immature immune response system than an adult with a well-developed immune (33-36). The disease-associated microbial flora population also varies significantly in adults, consisting of a mixture of aerobic and anaerobic bacteria as compared to infants with FG, that demonstrates local microbial flora and is highly dependent on the predisposing factors. Managing the disease in infants requires a quick multidisciplinary approach involving surgical debridement, broad-spectrum antibiotics, and supportive care. While the treatment outcomes may vary between adults and infants, early recognition and intervention are crucial for improving the prognosis of the disease (21,37-39).
Clinical presentation of FG in infants
Signs and symptoms
FG in infants is characterized by a sudden onset and rapid progression, leading to distress. The symptoms are localized in the perineal, genital, or abdominal areas. Infants with the disease exhibit redness, swelling, and tenderness in the affected area, which may appear edematous and have a warm, erythematous appearance. Systemic symptoms include high fever, irritability, poor feeding, lethargy, and vomiting. In severe cases, signs of septic shock, such as tachycardia, tachypnea, and hypotension, may occur. Several case studies by De La Torre et al. (2021), Bayileyegn et al. (2022), Hartoyo et al. (2022), and Pajares et al. (2023) have reported occurrences of FG in pediatric patients presenting with fever, painful erythema in both groins, scrotal swelling, irritability, crying during urination, perineal abscess, scrotal erythema, pain on palpation, and purulent exudate in the inguinal region (21,23,38,40). As the infection progresses, signs of necrosis or gangrene become visible in the affected skin areas, manifesting as skin discoloration (e.g., purple or black patches) and gas formation in the affected tissues (crepitus). Delport et al. (2020) reported swelling in the scrotum and penis, changes in skin coloration, and a foul smell in a ten-month-old baby boy diagnosed with FG (22). As the infection advances, a foul odor develops, which is a characteristic feature of FG.
Variability in clinical presentation
Both neonates and older infants differ from each other in terms of the clinical presentation of FG, owing to their obvious age difference. The disease symptoms are less specific in neonates, who generally show signs of general distress, difficulty in feeding, irritability, and less noticeable skin changes at the initial level (38,41). Contrarily, older infants and adults present similar symptoms characterized by erythema and edema in the perineal and genital areas. The clinical onset and presentation of FG are generally more severe in healthy infants, characterized by rapid onset as well as progression. On the other hand, infants suffering from congenital abnormalities generally present with atypical symptoms related to their pre-existing conditions (21,22).
Diagnostic challenges and differential diagnosis
Diagnosis of FG in infants is particularly challenging owing to the presence of typical nonspecific symptoms like fever and generalized irritability. The situation is further complicated by the rapid progression of the disease. Furthermore, the limited exposure of healthcare workers in managing FG cases stems from their limited exposure owing to the rarity of the condition. There is a significant overlap between the clinical symptoms of FG and other skin infections. Both these conditions showcase the presence of cellulitis, abscesses, and dermatologic conditions such as diaper dermatitis and trauma. The similar clinical presentation makes it increasingly difficult to differentiate between FG and other skin conditions, thereby contributing to the inherent complexity in the diagnosis. However, close speculation with focal examination and knowledge of differential clinical characteristics can still be effective in accurately diagnosing the infection type. For example, localized redness and swelling are common symptoms observed in cases of cellulitis and FG. However, cellulitis shows slow progression in comparison to FG. Similarly, localized swelling and tenderness are observed in abscesses, but the systemic symptoms are comparatively less pronounced.
Diagnosis of FG
History and physical examination
Early diagnosis of FG is particularly challenging due to a similar clinical presentation and symptoms to those of other skin infections, leading to misdiagnosis in three-quarters of the cases. Misdiagnosis is often a major cause of increased mortality and morbidity due to widespread infection in such cases. The condition presents difficulty in the differential diagnosis of FG from cellulitis and other NSTIs due to similar clinical signs and symptoms. The most commonly observed symptoms in cases of NSTI are erythema, edema, and pain, and therefore, may often be misdiagnosed as erysipelas and cellulitis, which are moderate-grade infections (42). However, if carefully speculated, a focal understanding of the clinical features of FG can be easily distinguished from other similar infections. FG is associated with a disproportionately severe level of pain relative to its appearance, and in its early stages, it is characterized by poorly demarcated erythema. In later stages, it progresses into the formation of bullae and blisters at the infection site. On the contrary, both cellulitis and erysipelas present with well-demarcated areas of inflammation and erythema (43). Moreover, the onset of FG is marked by systemic toxicity and prognoses to multiple organ failure, while cellulitis and erysipelas display symptoms of generalized infection such as fever and malaise (43-45). Even though FG is an acute condition, the presentation of clinical symptoms may take days to weeks to manifest. The average time interval between the onset of initial symptoms and admission to hospital is approximately 5.1±3.1 days, as reported by Ferreira et al. (2007) (46). In a study by Talwar et al. (2010), researchers outlined the sequence of symptom appearance, which progressed as follows: Initial onset of fever and lethargy (which may take 2–7 days to appear), followed by intense genital pain and tenderness accompanied by edema of the overlying skin. This is accompanied by the onset of erythema with increased intensity of genital pain, subcutaneous crepitation, the dusky appearance of the overlying skin, and purulent drainage from the wound, indicating genital gangrene (47). Therefore, clinicians must conduct thorough examinations to rule out any suspicion while investigating the genitalia and perineum of all patients (48,49).
Laboratory investigations and clinical scoring systems
To date, scientific literature has not identified any specific laboratory tests or biomarkers that can definitively diagnose FG or other NSTIs. However, aggregated laboratory data from multiple tests has proven effective in assessing illness severity and predicting mortality. Several predictive tools for disease diagnosis have been proposed, including:
- Fournier Gangrene Severity Index (FGSI) assesses disease severity based on nine clinical parameters, including temperature, respiratory rate, heart rate, and markers from the basic metabolic panel (serum potassium, serum sodium, serum bicarbonate, and creatinine), as well as blood cell parameters (hematocrit and leukocyte count). A FGSI score >9 predicts a 75% chance of death, while a score ≤9 predicts a 78% chance of survival (50). FGSI helps to quantify the severity of FG based on clinical and laboratory parameters. Each of the clinical parameters mentioned above is assigned a score based on its value. This is followed by the addition of the scores of individual parameters to compute the total score, which can provide valuable information for predicting outcomes and guiding therapeutic decisions. The individual scores of each parameter are evaluated and summed to obtain the total FGSI score. A higher total score indicates a more severe disease and a greater likelihood of complications or mortality. The scoring system is summarized in Table 2.
Recently, a meta-analysis study was done by Tufano et al. analyzed data from 37 studies involving 2,043 patients and demonstrated that non-survivors had significantly higher FGSI scores at admission compared to survivors, with a mean difference (MD) of 5.53 [95% confidence interval (CI): 4.68–6.37] (51). This strongly supports the FGSI’s prognostic value despite notable heterogeneity (I2=91%). Furthermore, in a subset of nine studies that reported diagnostic accuracy, the FGSI exhibited a pooled sensitivity of 84%, specificity of 85%, and an area under the ROC curve (AUC) of 0.90 (95% CI: 0.87–0.92), indicating excellent predictive performance for mortality. Traditionally, a threshold FGSI score ≥10.5 is associated with a 96% mortality rate, while scores <10.5 correspond to a 96% probability of survival. These findings affirm FGSI as a practical and reliable clinical tool for risk (50-52). - Platelet-lymphocyte ratio (PLR), neutrophil-lymphocyte ratio (NLR), and monocyte-lymphocyte ratio (MLR): evaluation of PLR, MLR, and NLR presents as a useful tool in predicting the severity status of the inflammatory and infectious conditions, including FG. Inflammatory response is marked by elevated levels of circulating neutrophils, platelets, and monocytes and decreased levels of lymphocytes in blood. The elevation in blood cell levels can be evaluated by increased levels of NLR, PLR, and MLR. Estimation of these ratios can serve as an indicator for predicting the severity of the infection and aid in monitoring disease progression. These measures can provide useful insights for surgical and medical interventions. PLR values >140 and NLR >8 have been reported to be associated with higher risks of mortality (53).
- Uludag Fournier Gangrene Severity Index (UFGSI) was first introduced by Yilmazlar et al. in 2010 to provide a more defined and accurate assessment of the severity of FG (54). It is a more refined version of FGSI and incorporates information from clinical parameters. UFGSI score is estimated by integrating information from the parameters with white blood count (WBC), organ dysfunction, and tissue architecture. A higher value of UFGSI is an indicator of a severe disease propensity and complications associated with high mortality outcomes. The evaluation of this score enables clinicians to make informed decisions about the patient’s disease status and intensive care requirements. It also guides in preparation for the required level of surgical intervention and enables identification of high-risk patients.
- Age-Adjusted Charlson Comorbidity Index (ACCI) provides a more detailed assessment of patient’s mortality risk by integrating information about the age and associated comorbidities. It is a useful computing tool for determining the patient’s outcome and the success of surgical interventions. It analyzes factors such as surgical risk assessment, prognosis rate in comorbid patients, and probability of hospital readmission to determine the risk levels in patients. A high ACCI score is indicative of higher mortality risk and thus requires immediate medical care and aggressive treatment regimes.
- Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score is estimated by integrating the information from various diagnostic tests such as C-reactive protein (CRP), WBC count, hemoglobin levels, and serum levels of creatinine, sodium, and glucose. Scores are assigned for each of these identified parameters, and then a summed-up value of these individual scores are defined as the LRINEC score. The test score lies between 0 to 13, and a test score ≥6 has been associated with the presence of NSTI (55). The score assigned to each of these parameters is detailed in Table 3.
- If the score lies between 0–4, it indicates a low probability of necrotizing fasciitis, whereas a score of 5–6 indicates an intermediate probability and requires imaging and surgical consultation. A high LRINEC score not only aids in early diagnosis but also correlates with disease severity and the likelihood of adverse outcomes, such as sepsis, multi-organ failure, or mortality. When used in conjunction with clinical assessment, LRINEC serves as a valuable triage and decision-making tool in emergency and critical care settings (9,57).
- Combined Urology and Plastics Index (CUPI): the CUPI index integrates information from both laboratory and clinical parameters. The outcome of FG from this score is calculated by examining several parameters, which include age at admission, and laboratory parameters (e.g., lactate, albumin, serum calcium, and total bilirubin). A higher CUPI score indicates the need for immediate surgical management and higher chances of morbidity or mortality.
- Platelet Mass Index (PMI) score: PMI is computed by multiplying mean platelet volume with platelet count, and PMI values ≥2,076.89 have been associated with increased fatality (58).
Table 2
Parameter | Normal (score =0) |
---|---|
Body temperature, ℃ | 36–38.4 |
Heart rate, breaths/min | 70–109 |
Respiratory rate, breaths/min | 12–24 |
Serum creatinine, mg/dL | 0.6–1.4 |
White blood cell count, ×103/µL | 3–14.9 |
Haematocrit, % | 30–45.9 |
Serum sodium, mmol/L | 130–149 |
Serum potassium, mmol/L | 3.5–5.4 |
Serum bicarbonate, mmol/L | 22–31.9 |
Score calculation | Higher or lower values from the normal range are scored 1–4 based on the degree of deviation |
Total FGSI score | A FGSI score >9 predicts a 75% chance of death, while a score ≤9 predicts a 78% chance of survival (50) |
FGSI, Fournier Gangrene Severity Index.
Table 3
Parameter | Score 0 | Score 1 | Score 2 | Score 4 | Prognostic insight |
---|---|---|---|---|---|
CRP, mg/L | <150 | – | – | ≥150 | High CRP (≥150) is a strong predictor of NSTI |
WBC, ×103/μL | <15.0 | 15–25 | >25 | – | Leukocytosis/leukopenia both reflect severe disease |
Haemoglobin, g/dL | >13.5 | 11–13.5 | <11 | – | Anemia is associated with tissue hypoxia and poor outcomes |
Sodium, mmol/L | ≥135 | – | <135 | – | Hyponatremia (<135) correlates with increased severity |
Creatinine, mg/dL | ≤1.6 | – | >1.6 | – | Renal dysfunction predicts systemic deterioration |
Glucose, mg/dL | ≤180 | >180 | – | – | Hyperglycemia impairs immune response and wound healing |
Total LRINEC score | 0–5 | 6–7 | ≥8 | 0–5: low risk; 6–7: intermediate; ≥8: high risk for NSTI (55,56) |
CRP, C-reactive protein; LRINEC, Laboratory Risk Indicator for Necrotizing Fasciitis; NSTI, Necrotizing Soft Tissue Infection; WBC, white blood count.
Imaging techniques
The diagnosis of FG primarily requires a comprehensive physical examination combined with a detailed medical history and a high level of suspicion. For haemodynamically stable patients suspected of having FG, immediate medical intervention should be initiated without waiting for the completion of imaging studies. Conventional radiography aids in the identification of soft tissue emphysema and tissue edema along fascial planes and can be identified before crepitus is noticeable on physical examination. Conventional radiography may not always show the presence of subcutaneous gas and may show false positive results. The technique is efficient in tracking subcutaneous gas but fails to detect the presence of deep tissue gas.
Point-of-care ultrasound (POCUS) is a highly effective technique for the characterization of soft tissue with high sensitivity (88%) and specificity (93%) via the usage of STAFF (Subcutaneous Thickening, Air, and Fascial Fluid) protocol. POCUS enables easy and effective identification of Intrascrotal Gas, which is a unique characteristic of NSTI, and identification of “cobblestoning” which represents thin lines of fluid between fat globules. Subcutaneous gas is also accompanied by the presence of “dirty shadowing” (hazy shadowing caused by hyperechoic foci with reverberation artifacts) and “snow globe” (heterogeneous swirling matter) (35,59).
Computed tomography (CT) scan and ultrasonography aid in effective diagnosis and surgical planning for the management of FG by providing a detailed picture of fluid collections, asymmetric fascial thickening, abscesses, fat stranding, as well as subcutaneous emphysema. The high specificity (93.3%) and sensitivity (88.5%) of CT scans make them an important tool in the identification of the magnitude and severity of infection. In a recent case report by Kim et al. (2023), the researchers used CT scan and ultrasonography to show the presence of air bubbles in the subcutaneous layer of the suprapubic and inguinal areas, indicative of FG in a 1-month-old girl suffering from fever and skin discoloration in the suprapubic area (37). Similarly, De La Torre et al. (2021) used ultrasonography to show soft tissue involvement in the case of a 24-day-old infant showing fever along with fluctuating and painful erythema in both groins, left hemiscrotum, left anterior femoral region, as well as perineum, indicative of FG (40). In another study, Bayileyegn et al. (2022) ruled out the presence of secondary infections and pathologies in eight-day-old male neonates presented with high-grade fever, scrotal swelling, irritability, and the presence of blackish scrotum with purulent discharge, indicative of FG (38). Magnetic resonance imaging (MRI) is seldom used for the characterization of soft tissue owing to its high cost as well as the longer time duration needed for the examination.
Management of FG in pediatric patients
FG has been observed to progress at a rapid rate, leading to significant morbidity and mortality if not promptly addressed. The management of FG in pediatric patients requires a comprehensive, multidisciplinary approach involving several levels of management strategies.
Immediate management steps upon diagnosis
FG is a life-threatening condition that needs immediate medical intervention to stabilize the patient while also carefully managing life-threatening conditions. FG is typically associated with specific signs and symptoms, which include severe pain, erythema, swelling, fever, and signs of infection. It is important for clinicians to recognize these signs, followed by implementation of patient stabilization measures, such as adequate oxygenation and close monitoring of vital signs.
FG is accompanied by systemic inflammatory response syndrome (SIRS), which results in increased vascular permeability, low blood pressure, and organ dysfunction, leading to a life-threatening condition. In addition, other complications such as acute kidney injury leading to renal failure and electrolyte imbalance may also occur. Adequate hydration and resuscitation help in stabilizing blood pressure, preventing circulatory collapse, restoring cardiovascular stability, alleviation of renal failure, controlling the inflammatory response, and electrolyte management. A multidisciplinary approach involving pediatric surgeons, critical care physicians, and infectious disease specialists is essential for coordinated action (4,60-63).
Medical management
- Fluid resuscitation: this technique involves the administration of isotonic crystalloids, such as normal saline or lactated Ringer’s solution, to restore intravascular volume as well as improve tissue perfusion. This is accompanied by monitoring of vital signs, including urine output, as well as laboratory markers analysis to assess the effects of ongoing fluid management since fluid overload can lead to pulmonary edema or heart failure (64-67). Delport et al. presented the case of a 10-month-old baby boy who presented with progressive swelling in the scrotum and penis, change in skin coloration and foul smell, difficulty in urination, diaper rash, and was treated with surgical debridement, antibiotic treatment, and fluid resuscitation (22).
- Combined broad-spectrum antibiotics: FG, being a polymicrobial infection, requires the administration of broad-spectrum antibiotics involving a mixture of multiple antibiotics to combat a wide spectrum of aerobic and anaerobic bacteria. Synergistic administration of antibiotics not only aids in improving overall treatment efficacy but also ensures enhanced coverage against a huge plethora of pathogens while curtailing the risks associated with antimicrobial drug resistance. Commonly prescribed antibiotics include a combination of piperacillin-tazobactam and vancomycin, ticarcillin-clavulanate and clindamycin, as well as Meropenem and Linezolid. Studies presented cases of 9-month-old (presented with convulsions and sepsis after fistulectomy. Scrotal, gluteal, and perianal edema), a 7-year-old boy (fever with progressive scrotal swelling and presence of penile erythema), 2-month-old male infant (presented with fever and progressive scrotal swelling, penile erythema) and 3-month-old boy [presented with fever (5-day history) with scrotal erythema, skin lesions, necrotizing scrotal fasciitis spreading to abdomen] (41,68-70). All these cases showed that timely diagnosis of the disease, coupled with the administration of broad-spectrum antibiotic treatment, aided in early recovery and favorable treatment outcomes. These cases, along with others, are summarized in Table 4.
Table 4
Details of case studies of pediatric Fournier gangreneNo. Subject detail with presenting symptoms Symptoms Diagnosis Procedure Outcome Conclusion Reference 1 1-month-old girl Fever, skin discoloration in the suprapubic area Ultrasonography and computed tomography showed the presence of air bubbles in the subcutaneous layer of the suprapubic and inguinal areas Emergency operation performing low transverse incision in suprapubic area to reveal subcutaneous tissue along with curettage and irrigation Improvement of necrotizing fasciitis after surgical treatment The study highlights the critical nature of timely diagnosis and clinical intervention in cases of pediatric FG (37) 2 24-day-old boy Fever with painful and fluctuating erythema in both groins, left anterior femoral region, left hemiscrotum, as well as perineum for the past 6 h Blood report revealed the presence of acute phase reactants. US revealed soft tissue involvement (I) Urgent fasciotomy, placement of Penrose drains as well as intensive irrigation; (II) broad-spectrum antibiotic therapy including cefotaxime, clindamycin, and cloxacillin for weeks Discharged on day 24 post-operatively with minimal, inconspicuous scars and absence of any functional sequela Early diagnosis, prompt surgical intervention, and administration of antibiotics play a crucial role in ameliorating the infection without complication (40) 3 8-day-old male neonate High-grade fever (38.8 ℃), irritability, scrotal swelling, and crying during urination for 3 days, presence of blackish scrotum with minimal pus discharge Blood analysis showed leukocytosis while scrotal US ruled out the presence of other pathologies. Pus culture showed a polymicrobial nature (E. coli, Bacteroides, and Staphylococcus aureus) (I) Prompt surgical debridement, incision, and drainage; (II) administration of ceftriaxone and metronidazole The neonate was discharged home safely Prompt surgical debridement, incision, and drainage along with the administration of broad-spectrum antibiotics aid in the reduction of infection and systemic toxicity (38) 4 16 neonates including 3 females and 13 males – Neonatal FG Surgical debridement along with antibiotic therapy 11 neonates successfully recovered while 5 died Early diagnosis along with prompt surgical intervention and antibiotics can help in treating FG (39) 5 30-day-old infant (caesarean delivery) White discoloration of scrotal skin, pain while urination, diaper rash (6 days before admission along with fever, which was treated with topical antifungal and corticosteroid ointment) (I) Leukocyte count: 23,000/µL and CRP 26.8 mg/dL, hemoglobin: 10.6 g/dL, serum sodium: 134 mEq/L, blood glucose: 80 mg/dL, serum urea: 15 mg/dL; (II) chest and abdominal X-rays were normal; (III) histology examination revealed the presence of non-specific granulation tissue correlating with FG; (IV) soft-tissue culture showed the presence of MRSA and ESBL-producing Klebsiella pneumoniae Surgical debridement to get rid of necrotic tissue along with antibiotic therapy The neonate was discharged safely Early diagnosis along with prompt surgical intervention and antibiotics can help in treating FG (21) 6 11-year-old boy with a diagnosis of hyperimmunoglobulinemia E Scrotal necrosis, perineal abscess, scrotal erythema, pain on palpation, purulent exudate in inguinal region Laboratory tests showed a complete blood count showing 19,530 leukocytes/mm3 and elevated expression of acute phase proteins (I) Aggressive surgical debridement with antibiotic therapy with clindamycin and meropenem and hemodynamic support; (II) advancement flap reconstruction surgery of the scrotal region The study showed no recurrence of the infection or complications up to 12 months after the follow-up of surgery Timely diagnosis of the disease along with a multidisciplinary approach and timely follow-up plays a crucial role in disease treatment (23) 7 3-month-old boy Fever (5-day history) with scrotal erythema, skin lesions, necrotizing scrotal fasciitis spreading to abdomen Preliminary laboratory results revealed leukocytosis with a WBC count of 23,200/mm3 with 78% neutrophils, high C-reactive protein, high blood urea, nitrogen 80 mg/dL, creatinine 0.72 mg/dL, high AST, high level of bilirubin. Ultrasonography revealed the presence of deep soft tissue infection with torsion testis Surgical debridement and relieving of testicular torsion with antibiotic therapy Despite antibiotic treatment, the patient developed severe sepsis and liver dysfunction Timely diagnosis of the disease along with a multidisciplinary approach and timely follow up plays a crucial role in disease treatment (68) 8 Fournier gangrene in a child with the presence of steroid-resistant nephrotic syndrome and anasarca with severe scrotal edema – Presence of fever and scrotal pain; laboratory tests revealed WBC =10,600 leukocytes/mm3, high CRP a 156 mg/L, creatinine a 0.3 and hyponatremia Surgical debridement with antibiotic therapy followed by reconstructive plastic surgery The neonate was discharged home safely Early diagnosis along with prompt surgical intervention and antibiotics can help in treating FG (32) 9 2-month-old male infant Fever and progressive scrotal swelling, penile erythema Scrotal US s left-sided hydrocele without evidence of testicular torsion Antibiotic therapy (using ceftazidime and clindamycin) Discharge of patients 5 days after admission. Follow up after 2 weeks, patient achieved a full recovery Broad gram-positive and anaerobic coverage may be beneficial in pediatric scrotal cellulitis, especially when standard SSTI therapy fails (69) 10 7-year-old boy with cerebral palsy Erythema, and swelling of the scrotum physical examination swelling, erythema and necrosis in the skin of the scrotum CT scan revealed the presence of air under the skin and the presence of a hyperdense lesion on the left side of the upper scrotum Surgical debridement with antibiotic therapy followed by reconstructive plastic surgery The patient was discharged after 2 weeks, and the abdominal wound was closed with a delayed primary closure protocol after 30 days Early diagnosis along with prompt surgical intervention and antibiotics can help in treating FG (70) 11 6-month-old female patient with anal stenosis Fever, irritability, and difficulty in feeding Physical examination after 12 hours showed ecchymosis in the anal region and necrotic lesions Surgical debridement with antibiotic therapy Complete recovery after 2 months of admission Early debridement, proper antibiotics treatment, and hyperbaric oxygen treatment can help in the treatment of FG (41) 12 9-month-old male patient Convulsions and sepsis after fistulectomy. Scrotal, gluteal, and perianal edema Physical examination revealed scrotal, gluteal, and perianal edema and ecchymosis (I) Perianal debridement and colostomy followed by broad-spectrum antibiotic therapy; (II) hyperbaric oxygen treatment for perianal lesions Complete recovery after 3 months of admission Early debridement, proper antibiotics treatment, and hyperbaric oxygen treatment can help in the treatment of FG (41) 13 Three patients, premature infants aged 10 days (born in the 27th gestation month), 14, and 17 months Predisposing factors: prematurity, diaper rash, and varicella infection Case I (10-day-old premature infant) showed the presence of perianal hyperemia leading to necrosis. Case II (a 14-month-old male patient) revealed perianal hyperemia with generalized edema, tachycardia, hypotension, and other signs of sepsis. Cellulitis and necrotic lesion were also observed on the penis. Laboratory tests revealed anemia and neutropenia. Case III (17-month-old female patient) showed hyperemia and tenderness in the right groin after a varicella infection. and was diagnosed with soft tissue infection Case I: antibiotic treatment (rifampicin and mupirocin) and protective colostomy. Case II: protective colostomy along with surgical debridement, VAC dressing, and administration of ornidazole and meropenem. Case III: medical treatment by intravenous ampicillin and clindamycin removal of infected tissue by surgical debridement and application of dressing Case I: the wound healed with healthy tissue in 2 weeks, but the patient required anal dilatation for a resulting stricture. Case II: the patient received VAC therapy followed by closure with bilateral V-Y flaps, anoplasty, and circumcision without complications; the colostomy was reversed two months later. Case III: the tissue defect healed naturally by secondary intention Early diagnosis along with prompt surgical intervention and antibiotics can help in treating FG (71) 14 10-month-old baby boy Progressive swelling in scrotum and penis, change in skin coloration and foul smell, difficulty in urination, diaper rash Physical examination: the penis, scrotum and perineum were necrotic with pus and foul smelling, leukocytosis and high CRP (I) Emergency surgical debridement for removal of necrotic tissue; (II) broad-spectrum antibiotic treatment; (III) reconstructive surgery 6 weeks post debridement (including penoplasty, scrotoplasty, and bilateral orchidopexy) No sign of discomfort or genitourinary complaints after 18 months of surgery Management of FG can be done by prompt stabilization, fluid resuscitation, administration of broad-spectrum antibiotics, and early aggressive surgical debridement (22) 15 A 1-month-old male infant presented with FG occurring due to perforation transverse colon Fever, scrotal swelling, scrotal erythema with necrotic tissue, pus in umbilical cord (additional infections: septic shock, pneumonia, and patent processus vaginalis) Radiological findings showed the presence of FG with pneumonia and pneumoperitoneum. Laboratory: thrombocytopenia, renal impairment, high inflammation markers, and metabolic acidosis (I) Incision drainage and peritoneal lavage; (II) exploratory laparotomy, colostomy, necrotomy, and surgical debridement; (III) patent processus vaginalis ligation After 1 month the patient developed respiratory failure due to hospital-acquired pneumonia Prompt diagnosis with a multidisciplinary approach may have a better outcome in the management of FG (20) AST, aspartate transaminase; CRP, C-reactive protein; ESBL, extended-spectrum β-lactamase; FG, Fournier gangrene; MRSA, methicillin-resistant Staphylococcus aureus; US, Ultrasound; VAC, vacuum assisted closure; WBC, white blood count.
Surgical interventions and wound care strategies tailored for pediatric patients
- Surgical debridement: the primary goal of surgical debridement is to control the progression of infection by ensuring complete removal of the entire necrotic and infected tissue. To achieve this, the anesthetic and the other teams should be contacted early to ensure a prompt discussion. Lin et al. (2019), in a recent study, showed that the optimal period between initial diagnosis and surgical debridement should not exceed 14 hours, and prolonged time delay has been associated with increased mortality (72). Similarly, another study highlighted the necessity of early diagnosis in the effective management of FG and has demonstrated a direct correlation between early surgical debridement and a higher recovery rate (32). Multiple rounds of debridement may be required depending on the severity of the infection and the rate of necrosis. However, the debridement process should be performed with utmost care and precision to avoid damaging the surrounding healthy tissues. The debridement process is followed by the placement of a surgical drain to facilitate the elimination of purulent material and prevent fluid accumulation. De La Torre et al. (2021) showcased the exceptional case of a 24-day-old boy diagnosed with FG and underwent early diagnosis followed by administration of broad-spectrum antibiotics and prompt surgical management, leading to early recovery (40). Prompt surgical debridement has been reported to increase the chances of favorable outcomes in pediatric patients, as shown in studies by Pajares et al., 2023, Padilla et al., 2020, Bayileyegn et al., 2022, better recovery was reported in cases involving of an 11-year-old boy, a child with steroid-resistant nephrotic syndrome, an Eight-day-old male neonate (23,32,38).
- Vacuum-assisted closure (VAC) aids in the withdrawal of fluid from the wound area via the usage of negative pressure and is therefore referred to as “negative pressure wound therapy” (NPWT). NPWT accelerates the healing process by reducing edema and improving blood flow to the affected area, thereby preparing the ground for wound closure (73,74). However, VAC shows maximum efficacy if integrated with other wound care strategies. A retrospective study by Iacovelli et al. (2021) comprising data from 92 patients showed higher efficacy of VAC therapy in the treatment of patients with disseminated FG in promoting wound healing at a higher rate in comparison to conventional dressing (75).
- Fecal diversion involves the creation of a temporary ileostomy or colostomy to divert fecal flow away from the gastrointestinal areas affected by FG. This procedure eliminates the risk of further infection and contamination while ensuring proper wound healing of the affected perineal or abdominal regions (76-78). A study by Rouzrokh et al. (2014) showed a report of 7 cases of FG in pediatric patients, two of which required extensive debridement; one underwent both urinary and fecal diversion, while the other received only fecal diversion to reduce contamination and allow wound healing (79).
- Post-operative skin reconstruction is a reformative approach for severely damaged skin due to exhaustive surgical debridement procedures. Several newly developed reconstructive techniques may be used for this purpose, including different types of local flaps to ensure proper wound coverage while preserving function and aesthetics (80). Several pre-disposable factors, such as wound conditions, patients’ medical status, and available surgical options, greatly influence the choice of reconstructive approach. However, the successful outcome of the process with proper healing and functional recovery of the skin areas requires effective post-operative wound management and rehabilitation (22). A case study was reported by Ekingen et al. (2008) of a 14-month-old male patient admitted with necrotizing fasciitis in the perianal region as well as preputial skin. The patient was managed by empirical antibiotic treatment, accompanied by surgical removal of the infected tissue and VAC dressing, followed by closing of the tissue defect with a v-y advancement flap (71). Another case of postoperative skin reconstruction to manage FG was reported by Delport et al. (2020), wherein FG in a 10-month-old boy was managed by reconstructive surgery 6 weeks post debridement (including penoplasty, scrotoplasty, and bilateral orchidopexy) (22).
Non-pharmacological therapies and antibiotic choices in the management of pediatric FG
Hyperbaric oxygen therapy (HBOT) aims to promote wound healing by ensuring oxygenation in hypoxic tissues, which can be particularly instrumental in the management of severe cases of gangrene with compromised wound healing. The usage of HBOT in conjunction with other therapies has been reported to yield promising results in the management of gangrene in pediatric as well as adult patients (81-83). A meta-analysis by Raizandha et al. (2022) of 10 retrospective studies showed the ameliorative role of HBOT in significantly decreasing the mortality rate in patients suffering from FG (84). Sütçü et al. (2016) in a study described the management of cases of FG in two infants (A nine-month-old male patient presented with convulsions and sepsis after fistulectomy, Scrotal, gluteal, and perianal edema and a 6-month-old female patient with anal stenosis presented with fever, irritability, difficulty in feeding and ecchymosis in the anal region and necrotic lesions) and reported better recovery if hyperbaric oxygen was administered after perianal debridement, colostomy and broad-spectrum antibiotic administration (41).
Complications of pediatric FG
Potential complications
FG is a rapidly progressing condition that can result in systemic infection and sepsis. It leads to a systemic inflammatory response characterized by dysfunction in multiple organs, which, if not promptly treated, can potentially be fatal. Severe cases can lead to septic shock, characterized by low blood pressure and organ failure, necessitating immediate medical intervention (1). Sepsis may cause organ dysfunction in the liver, kidneys, and cardiovascular system. This was highlighted in a recent study by Bakalli et al. (2023), wherein the clinicians described the case of a 3-month-old boy presenting with typical symptoms of FG (fever, scrotal erythema) spreading to the abdomen (68). The infant was diagnosed with FG and later underwent surgical debridement, relief of testicular torsion, and antibiotic therapy. However, despite prompt antibiotic treatment, the patient developed severe sepsis as well as liver dysfunction, which further complicated the treatment procedure. Additionally, FG has also been reported to lead to extensive tissue necrosis, characterized by loss of skin and subcutaneous tissue, both of which can cause functional impairment and substantial deformities (85-87).
Long-term outcomes and sequelae
Extensive tissue damage, generating the need for extensive surgical debridement in the affected genital or perineal areas, may lead to functional impairment and loss of sexual and urinary functions. Excessive cosmetic or functional deformity may have a huge psychosocial impact on the well-being of the affected individual. Growth and development of the affected individual may be compromised owing to extensive tissue loss and surgical intervention. The affected may become prone to recurrent infections due to the presence of excessive scarring and tissue damage (88,89).
Strategies for mitigating complications
Timely disease diagnosis with immediate medical intervention and antibiotic treatment can help control disease progression, eliminating the need for extensive surgical debridement. Surgical management involving debridement and reconstructive surgery is vital in controlling infection and averting further complications. Supportive care through fluid resuscitation and nutritional support promotes wound healing and ensures timely recovery. Appropriate management of the condition requires the implementation of a multidisciplinary approach, comprising pediatric surgeons, infectious disease specialists, wound care experts, as well as psychologists. This should be followed by regular follow-ups and proper rehabilitation to ensure swift recovery and address issues related to functional impairment and mental distress (90).
Prognosis and follow-up for pediatric FG
Factors influencing prognosis in pediatric patients
The prognosis of FG depends on the severity of the infection and the grade of tissue damage. The prognosis of the disease is worsened by extensive tissue damage and necrosis. Therefore, early diagnosis of the disease aids in controlling the infection and achieving favorable improved outcomes. The recovery is exacerbated by underlying health issues, such as birth defects, metabolic disorders, and a compromised immune system (91). Also, the age of the patient negatively affects the prognosis. Younger infants with immature immune systems have a lower chance of recovery. Recovery and surgical outcomes are largely dependent on the success of first-line treatments, including surgical debridement and antibiotics. Timely and correct treatment can lead to better results. Maintaining adequate nutrition for speedy recovery since poor nutrition can slow down the wound healing process (63).
Long-term follow-up considerations and outcomes
It is important to closely monitor the rate of wound healing and address remnant wounds to ensure postoperative care for FG. This is important since any delay in wound healing would need additional reconstructive surgeries. Therefore, in order to improve the quality of life of individuals, it is important to assess functional impairments and address them via reconstructive procedures and physical therapy. Affected children often develop psychological issues, which supportive therapies must address. It is important to detect long-term complications and recurrent infections via regular follow-ups (78).
Prevention strategies for pediatric FG
Prevention strategies focusing on pediatric populations
FG poses substantial risks for the pediatric population and, therefore, needs the implementation of prevention strategies. These strategies should be aimed at not just safeguarding children’s health but also achieving favorable treatment outcomes. Prevention of FG requires the conduct of regular screening programs to identify infants with congenital abnormalities and prevent them from developing fatal conditions like FG. This can be achieved by timely management of conditions like diaper dermatitis, as well as managing urological abnormalities in the perineal area. Also, it is important to administer antibiotics judiciously to control the emergence of antibiotic-resistant bacterial strains, which further complicate the management of conditions like FG (5). It is important to conduct community awareness programs in order to impart the necessary knowledge to healthcare workers, caregivers, and parents to enable them to be able to effectively diagnose and manage conditions like FG (34).
Role of vaccination and hygiene practices
Vaccination and following proper hygiene and sanitation practices are important in alleviating the risk of FG among pediatric populations. Vaccine immunization plays a crucial role in strengthening the immune system, as a consequence of which, the likelihood of children contracting FG is alleviated. Not only do these strategies promote individual health, but they also aid in decreasing infection rates in vulnerable populations. So far, no vaccine has been devised to prevent the occurrence of FG, nonetheless, vaccines against Haemophilus influenzae type b, Streptococcus pneumoniae, and other bacterial infections play a crucial role in decreasing the overall occurrence of bacterial infections, hence, indirectly reducing the likelihood of FG in vaccinated children. Gentle wiping of the diaper changing area and frequent diaper changes can play a crucial role in reducing the occurrence of diaper dermatitis and other skin infections in the genital area (71).
Conclusions
FG, despite its rare occurrence in the pediatric population, poses a humongous challenge to the healthcare community owing to its rapid onset, complex diagnosis, and high morbidity and mortality rates. The current review article highlights the importance of early diagnosis coupled with immediate medical intervention in the management of FG in children. Also, the review sheds light on the unique etiology and the uncommon clinical presentation of the disease, both of which require the implementation of a multidisciplinary approach comprising infectious disease specialists, pediatric surgeons, and public health professionals. The review provides a glimpse of the shortcomings of the healthcare system and emphasizes the need for optimization of treatment procedures while also evaluating the long-term effects of FG on affected children.
Acknowledgments
None.
Footnote
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