Fournier gangrene, a form of necrotizing fasciitis, is a rapidly progressive disease that affects the deep and superficial planes of the perineal and genital region. Named after Dr. Alfred Fournier, the French dermatology and venereal specialist, it was initially described in 1883 as necrotizing fasciitis of the external genitalia, perineal, and perianal region in five of Dr. Fournier’s patients. Similar to other forms of necrotizing fasciitis, this disease involves the spread of inflammation along fascial planes and adjacent soft tissue; therefore, the disease often goes unnoticed as there are no skin manifestations. The spread of inflammation and infection leads to thrombosis of blood vessels, which in turn leads to ischemia and necrosis of the adjacent soft tissue and fascia. Furthermore, the infectious and inflammatory process spreads along the dartos fascial, Colle’s fascia, and Scarpa’s fascia, which allows for the involvement of the abdominal wall. Due to initial fascial and subcutaneous involvement, clinicians can miss this disease because the overlying soft tissue can often look unremarkable. Therefore, early diagnosis of this potentially fatal disease is key as it can often get misdiagnosed as an otherwise benign process.
This disease process often results from polymicrobial aerobic and anaerobic synergistic infection of the fascia. Similar to necrotizing fasciitis, gram-positive bacteria such as Group A Streptococci and Staphylococcus aureus and gram-negative bacteria such as E. Coli and Pseudomonas aeruginosa are organisms most commonly grown in wound cultures of Fournier gangrene patients. These bacteria can get introduced through several different sources, including urinary, bowel, or dermal. Urinary tract infections and other infectious processes of the perineum, such as perianal abscesses, also provide a nidus for infection. Surgical manipulation of the genital and perineal area similarly can provide the initial insult required in the development of Fournier gangrene. Additionally, trauma to the area leading to skin breakdown can provide bacteria with a path to the fascia and subsequent development of the disease.
Fournier gangrene has a strong predilection for males as opposed to females with a 10 to 1 ratio. The incidence in males 1.6 per 100000 cases. Fournier gangrene is seen most commonly in men ages 50 to 79, with an incidence of 3.3 per 100000 cases. These frequencies appear in all patients both in the US and worldwide.
The pathophysiology of Fournier gangrene involves a synergistic infectious process involving anaerobic and aerobic bacteria. The bacterial infection involves an initial insult such as a urinary tract infection, perineal abscess, recent surgical manipulation of the genital or perineal area. The synergistic activity of the bacterial infection leads to obliterative endarteritis, the micro thrombosis of subcutaneous vessels, ultimately leading to gangrene of the surrounding tissue, which is a result of bacterial production of various endotoxins and enzymes.
While the diagnosis of Fournier gangrene is often predicated on a combination of imaging and history and physical examination, a biopsy of the area in question is obtainable to differentiate Fournier gangrene from severe cellulitis. The histopathology of Fournier gangrene will show ulceration of the epidermis, neutrophilic exudate, thrombosed vessels, and necrosis.
Patients with Fournier gangrene often present with pain to the perineal or genital area. Patients may often report having a medical history of diabetes mellitus, hypertension, malignancy, and/or alcohol abuse, which are all known risk factors for Fournier gangrene as well as being male. Patients may also report being immunocompromised or having recently had trauma to the area as well as recent surgeries. Depending on the time at which they are presenting, they may also report redness and swelling to the perineum and genitals. Patients will often report systemic symptoms such as fever, chills, nausea, vomiting, urinary retention, and malaise.
On physical examination, the patient may have pain out of proportion to findings as there may not be any cutaneous manifestations of the disease. Findings of crepitus, swelling, hyperemia, discoloration of the skin, and putrid smell may be seen on physical examination depending on the time of patient presentation. Other nonspecific physical examination findings include tachycardia, tachypnea, and elevated temperature.
Evaluation of Fournier gangrene requires a combination of both blood work and imaging.
Blood work in Fournier gangrene should include complete blood count(CBC) with differential and comprehensive metabolic panel (CMP). The CBC will often show elevated white blood count (WBC) with the potential for a left shift. The CMP may show any electrolyte abnormalities such as hyponatremia or metabolic acidosis, as well as any concurrent renal failure. Blood cultures and lactate can help to evaluate for associated bacteremia and sepsis. Arterial blood gas may be obtained to assess for acid/base status. Wound cultures are necessary to guide antibiotic treatment.
While traditionally a clinical diagnosis, imaging can be utilized to aid in confirmation of and the extent of disease. Ultrasound imaging helps visualize any subcutaneous air in the underlying soft tissue. Ultrasound may also be used to evaluate for edema and thickness of the affected soft tissue. X-ray imaging of the area may similarly indicate any subcutaneous air in the affected area. The most specific form of imaging in the diagnosis of Fournier gangrene is CT imaging. This form of imaging allows for better evaluation of the extent of the disease when compared to plain films and ultrasound imaging. CT imaging findings include fascial thickening, subcutaneous air, and fluid collections such as an abscess.
Fournier gangrene is a true urological emergency. The disease process is managed with both surgical interventions and medical resuscitation as patients will often be septic.
Medical intervention revolves around the initiation of empiric broad-spectrum antibiotics while awaiting culture sensitivities. Antibiotic therapy has historically involved triple therapy in covering for the previously mentioned gram-positive, gram-negative, and aerobic organisms that are associated with Fournier gangrene. The combination of a third-generation cephalosporin or aminoglycoside, in addition to penicillin and metronidazole, is classically used as triple therapy antibiotic coverage. Current antibiotic regimens include the use of carbapenems or piperacillin-tazobactam. In addition to antibiotic therapy, fluid resuscitation is of great importance as patients may present with hypotension. Vasopressors can be an addition to the patient’s resuscitation efforts should their hypotension not be responsive to fluids alone. Electrolyte abnormalities should also be appropriately corrected. Diabetic patients suffering from Fournier gangrene will need correction of any blood glucose abnormalities.
Surgical intervention is predicated on radical, wide resection of necrotic, gangrenous tissue. The time of presentation to surgical intervention has had associations with improved prognosis; therefore, efforts should be made to minimize this time. Surgical debridement is based on the separation of the skin and subcutaneous tissue with debridement halting when the skin and subcutaneous tissue can no longer be easily separated. Surgical debridement often takes place in stages, with most cases of Fournier gangrene requiring three separate procedures.
Following surgical debridement and formation of granulation tissue, patients will need to undergo reconstructive surgery of the affected area. Skin involvement guides reconstruction effects, which involves one or more of the following: skin plasty with local tissue, stem cell utilization, and/or split autodermoplasty. Vacuum-assisted closure (VAC) system dressing is used following debridement and reconstruction efforts to minimize skin defects and enhance tissue healing. This enhancement is based on exposing the tissue to subatmospheric pressures for prolonged periods, which promotes debridement and healing.
Patients with significant perineal involvement often will undergo diverting colostomy or fecal management systems for feces diversion to aid in healing. Fecal management systems have their basis around bowel management catheters. The catheter system involves the insertion of the catheter into the rectum with a syringe for irrigation and a collection bag. Diverting colostomy is indicated in patients where the infectious process originated from the anorectal area and subsequently involved the anal sphincter. Other indications include rectal perforation, large rectal wound, or systemic sepsis. A diverting colostomy is often performed using the Trephine technique. Using this technique, a flexible sigmoidoscope is used to transilluminate and locate the sigmoid colon. The stoma site is determined intra-operatively. A trephine is performed, and while holding the colon, the sigmoidoscope gets withdrawn. The colon is delivered into the wound and an end colostomy is formed. When compared, fecal management systems have been shown to have decreased length of hospitalization, 24.1 days, when compared to patients who have received a colostomy, 40.5 days.
Use of topical therapy and hyperbaric oxygen are two other treatment modalities that are useful for the treatment of Fournier gangrene; however, medical and surgical treatment takes priority. Hyperbaric oxygen therapy is a controversial supplemental treatment option. The premise being that the hyperbaric environment results in the improvement of tissue oxygenation, thereby improving antibiotic delivery and wound healing. Application of honey to the affected areas has also been reported to improve wound healing, but no studies have thus far been performed to validate this type of therapy.
The differential diagnosis for Fournier gangrene includes testicular torsion, epididymitis, cellulitis, abdominal wall cellulitis, perianal/periurethral abscess, gangrenous balanitis, gangrenous vulvitis, inguinal lymphogranulomatosis, syphilis, chancre, tissue edema, herpes simplex, vasculitis, toxic shock syndrome, toxic epidermal necrolysis, and Stevens-Johnson syndrome. 
The prognosis of Fournier gangrene is multifactorial.
A study published in 1995 by Laor et al. showed that the development of the Fournier Gangrene Severity Index (FGSI) is useful in determining prognosis in patients suffering from the disease. The index used temperature, heart rate, respiratory rate, serum potassium and sodium, creatinine, bicarbonate levels, hematocrit, and white blood count. A score of greater than 9 was associated with a mortality of greater than 75 percent, while patients with a score of less than 9 had a 78 percent chance of survival. Other electrolyte abnormalities that are associated with a worse prognosis include elevated calcium and low magnesium levels.
A patient’s age, as well as the extent of tissue involvement, also determines the prognosis. Patient prognosis declines with advancing age. The larger the degree of tissue involvement is similarly associated with a worse prognosis. Uludag FGSI (UFGSI) scoring system involving both of these factors was developed in 2010 by Yilmazlar et al. Using this newly developed scoring system, a score greater than 9 was associated with a 94% likelihood of death while a score less than 9 correlated with an 81% probability of survival.
The sAPGAR, surgical APGAR score, and the ACCI, age-adjusted Charleston Comorbidity Index, are two general surgical scoring systems that can be used to determine the prognosis of the patient's suffering from Fournier gangrene. ACCI is a prognostic classification system based on assigning points to 19 different comorbidities with each comorbidity assigned a score of 1,2,3, or 6. Higher scores are associated with increased mortality risk. sAPGAR is used to calculate complications in postoperative patients. The score's basis is estimated blood, lowest mean arterial pressure, and lowest heart rate. The lower the sAPGAR score, the higher the risk of complication. While not specific to Fournier gangrene, both sAPGAR and ACCI are equivocal to FGSI and UFGSI in determining patient outcomes.
As previously mentioned, the time of onset of the disease to surgical treatment is key; patients who present earlier often having better outcomes. Diabetic patients with a HgA1c greater than 7 have also been found to have a worse prognosis.
Given the devasting effect that Fournier gangrene has on patients, complications can present both in the short term as well as the long term.
Short term systemic complications of Fournier gangrene are a result of the inflammatory response mounted by the body against this aggressive infectious process. These systemic complications include acute renal failure, acute respiratory distress syndrome, cardiac arrhythmias, heart failure, and bacteremia. This bacteremia can cause acute thromboembolic events such as strokes and arterial occlusion in the lower extremities. Also, patients can develop ileus as a result of the multiple surgeries they undergo. Wound infections following debridement can also occur; however, as previously discussed, adjunctive treatments such as hyperbaric oxygen and topical treatment are aimed at lowering the rate of wound infections.
Patients who suffer from significant disease involvement of the perineum can often develop fecal incontinence as a result of the involvement of anal sphincter, which requires debridement. These patients will require a colostomy to decrease fecal contamination of the wound. The formation of a colostomy itself comes with multiple associated complications. These complications include the evisceration of the stoma and stomal infection. As the penis is often involved, urinary tract infections are common complications of the disease. Urinary retention results from periurethral swelling, which prevents the patient from voiding; treatment includes urinary catheterization as well as cystostomy in certain patient groups.
Psychological complications are prevalent in patients who suffer from Fournier gangrene. Due to the devastating effect the disease has on the genitals, many patients have long term pain as a result. This condition leads to a decreased quality of life, which can cause depression. The disfiguring scars associated with the recovery process also contribute to patients’ psychological problems. Patients will often suffer from sexual dysfunction stemming from impaired penile function, penile deviation, and penile torsion. Loss of penile skin sensitivity and pain associated with erections are two other factors that lead to sexual dysfunction.
Fournier gangrene is a rare but deadly infection of the genital and perineum. Symptoms of this infectious process include swelling to the genitals and perineal area associated with significant pain. Systemic symptoms are not uncommon. While there is no specific way to prevent Fournier gangrene, there are certain actions that can be taken to minimize the chances of suffering the disease. Diabetic patients should carefully and thoroughly assess the groin area for any signs of redness, swelling, or pain. Also, appropriate management of diabetes with medication compliance is beneficial. Diabetic patients who suffer from Fournier gangrene have a better prognosis if the HgA1c is below 7. Maintaining appropriate hygiene and skincare can also prevent Fournier gangrene as break down in skin can be the first step in the development of the disease.
Fournier gangrene is a rapid, life-threatening infection of the genitals and perineum. Special care and attention should be given to patients presenting with groin pain as misdiagnosis can prove fatal. Particular attention is necessary for patients who have diabetes, have a history of hypertension, or are immunocompromised as these factors predispose patients to the development of Fournier gangrene. Treatment revolves around medical resuscitation with intravenous fluids and antibiotics, with priority given to emergent surgical debridement. Despite the devastating complications, many patients are ultimately pleased with their quality of life.
The treatment and management of Fournier gangrene require an interprofessional team. Patients will often present to the emergency department or their primary care physician. Following diagnosis and admission to the hospital, Fournier gangrene management is best by a team of health care professionals, including an intensivist, urologist, pharmacist, infectious disease expert, and nursing staff. In the emergency department, prompt identification of the disease is of great importance. The faster a patient with Fournier gangrene gets evaluated, and their workup initiated, the quicker they are accurately diagnosed and treated. Following an appropriate diagnosis, the patient will require admission and urological consultation for prompt surgical debridement. These patients will often require admission to an intensive care unit where their vitals will be continually monitored by nursing staff who will communicate any concerns regarding patient deterioration to the intensivist. Pharmacy and infectious disease play crucial roles in the determination of the appropriate antibiotics, which should be given to the patient once culture sensitivities result. If the wound is left open, a wound care nurse should be involved with the dressing changes. A stoma nurse should assess the appliance and ensure that the skin around the stoma is protected. This global and systemic interprofessional approach to the treatment of patients suffering from Fournier gangrene is crucial in lowering the associated mortality. [Level 5]
Fournier gangrene is a potentially fatal disease with a mortality ranging from 20% to 30%. Mortality is dependent on age, time to surgical intervention, and predisposing factors such as diabetes mellitus, hypertension, and immunocompromise. If diagnosed and managed appropriately, most survivors of Fournier gangrene are satisfied with their quality of life and cosmetic results despite complications such as chronic pain, sexual dysfunction, and stool incontinence.
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