Introduction
Background
In 1883, the French venereologist Jean Alfred Fournier described a series in which 5 previously healthy young men suffered from a rapidly progressive gangrene of the penis and scrotum without apparent cause. This condition, now known as Fournier gangrene, is defined as a polymicrobial necrotizing fasciitis of the perineal, perianal, or genital areas. In contrast to Dr Fournier's initial description, doctors today know that the disease is not limited to young or male patients, and a causative etiology usually is identified.
Pathophysiology
Localized infection adjacent to a portal of entry is the inciting event in the development of Fournier gangrene.
Wound cultures from patients with Fournier gangrene reveal that it is a polymicrobial infection with an average of 4 isolates per case. The bacteria involved act synergistically, via collagenases, hyaluronidases, and other enzymes to invade and destroy fascial planes. See Causes section for specific microbes involved and specific inciting factors.
Ultimately, an obliterative endarteritis develops, and the ensuing cutaneous and subcutaneous vascular necrosis leads to localized ischemia and further bacterial proliferation. Rates of fascial destruction as high as 2-3 cm/h have been described. Infection of superficial perineal fascia (Colles fascia) may spread to the penis and scrotum via Buck and dartos fascia, or to the anterior abdominal wall via Scarpa fascia, or vice versa. Colles fascia is attached to the perineal body and urogenital diaphragm posteriorly and to the pubic rami laterally, thus limiting progression in these directions. Testicular involvement is rare, as the testicular arteries originate directly from the aorta and thus have a blood supply separate from the affected region.
Frequency
United States
Fournier gangrene is relatively uncommon. The true incidence of the disease is unknown. A retrospective case review revealed 1726 cases documented in the literature from 1950-1999.1 An average of 97 cases per year were reported from 1989-1998.
Mortality/Morbidity
- The reported mortality rate for Fournier gangrene varies widely from 4-75%.
- Factors associated with high mortality include an anorectal source, advanced age, extensive disease (involving abdominal wall or thighs), shock or sepsis at presentation, renal failure, and hepatic dysfunction.
- Death usually results from systemic illness, such as sepsis, coagulopathy, acute renal failure, diabetic ketoacidosis, or multiple organ failure.
Sex
Male-to-female ratio is approximately 10:1. Lower incidence in females may be caused by better drainage of the perineal region through vaginal secretions. Men who have sex with men may be at higher risk, especially for infections caused by community-associated methicillin-resistant Staphylococcus aureus (MRSA).
Age
Most reported cases occur in patients aged 30-60 years. A 1997 literature review found only 56 pediatric cases, with 66% of those in infants younger than 3 months.
Clinical
History
Obtain a thorough review of systems, including history of diabetes, alcohol abuse, cancer, colorectal or urogenital disease or surgery, steroid use, sexual history, and HIV status.
- Fournier gangrene usually begins with an insidious onset of pruritus and discomfort of the external genitalia.
- Patients wait an average of 5 days before seeking medical attention.
- Early in the course of the disease, pain may be out of proportion to physical findings.
- Swelling and erythema of the region follow pain, and patients may complain of systemic symptoms such as fever or chills.
- As gangrene develops, pain actually may subside as nerve tissue becomes necrotic.
Physical
- Skin overlying the affected region may be normal, erythematous, edematous, cyanotic, bronzed, indurated, blistered, and/or frankly gangrenous. Skin appearance often underestimates the degree of underlying disease.
- A feculent odor may be present secondary to infection with anaerobic bacteria.
- Crepitus may be present, but its absence does not exclude the presence of Clostridium species or other gas-producing organisms.
- Systemic symptoms, eg, fever, tachycardia, hypotension, may be present.
- A thorough genital and perianal examination is required to detect potential portal of entry.
Causes
Idiopathic cases still are reported, but causative factors are found in more than 75% of patients.
- Inciting events: Localized infection adjacent to a portal of entry is often the inciting event in the development of Fournier gangrene. Colorectal, genitourinary, and dermatologic sources are implicated in the pathogenesis of the disease. Trauma, recent surgery, and the presence of foreign bodies may also lead to the disease. Examples include the following:
- Perianal, perirectal, and ischiorectal abscesses; anal fissures; colonic perforations; urethral strictures with urinary extravasation; chronic urinary tract infections; epididymitis; orchitis; or hidradenitis may lead to the disease.
- Urethral instrumentation, prosthetic penile implants, superficial soft-tissue injuries, intramuscular injections, genital piercings, steroid enemas (used for the treatment of radiation proctitis), blunt thoracic trauma, and penile self-injection with cocaine have been reported in the literature as causative factors.
- In women, septic abortions, vulvar or Bartholin gland abscesses, hysterectomy, and episiotomy are documented sources.
- In men, anal intercourse may increase risk of perineal infection, either from blunt trauma to the area or by spread of rectally carried microbes.
- In children, circumcision, strangulated inguinal hernia, omphalitis, insect bites, trauma, urethral instrumentation, perirectal abscesses, systemic infections, and burns have led to the disease.
- Inability to practice adequate perineal hygiene or the presence of chronically indwelling catheters, such as in paraplegic patients, poses an increased risk.
- Causative microbes isolated in wound cultures: Wound cultures from patients with Fournier gangrene reveal that it is a polymicrobial infection with an average of 4 isolates per case.
- Escherichia coli is the predominant aerobe, and Bacteroides is the predominant anaerobe.
- Other common microflora include Proteus, Staphylococcus, Enterococcus, aerobic and anaerobic Streptococcus, Pseudomonas, Klebsiella, and Clostridium.
- Incidence of methicillin-resistant Staphylococcus aureus may be increasing .
- Predisposition to disease: Any condition with depressed cellular immunity may predispose a patient to the development of Fournier gangrene. Examples include the following:
- Diabetes mellitus (present in up to 60% of cases)
- Alcoholism
- Extremes of age
- Malignancy
- Chronic steroid use
- Malnutrition
- HIV infection
More on Fournier Gangrene |
 Overview: Fournier Gangrene |
| Differential Diagnoses & Workup: Fournier Gangrene |
| Treatment & Medication: Fournier Gangrene |
| Follow-up: Fournier Gangrene |
| References |
| Next Page » |
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Further Reading
Keywords
Fournier gangrene, Fournier's gangrene, gangrene of the penis and scrotum, polymicrobial necrotizing fasciitis, infection of superficial perineal fascia
