Fournier Gangrene

What is Fournier Gangrene?

Fournier gangrene is a rare and fast-moving disease that affects the skin and deep tissues in the private areas, including the area around the genitals, anus, and perineum (the area between the anus and the genitals). It was first identified in 1883 by Dr. Alfred Fournier, a French doctor. This disease, also known as necrotizing fasciitis, can have severe inflammation and infection that rapidly spread along certain tissue layers, affecting nearby soft tissue. Because there might be little or no visible effects on the skin in the early stages, it sometimes goes unnoticed.

Fournier gangrene is often linked with general signs of serious infection, rapid tissue damage, and a high fatality rate of 40%. The infection and inflammation can cause blood clots in the vessels, which in turn causes a lack of blood supply leading to tissue death in the surrounding soft tissue and layers of tissue. These infection and inflammation spread quickly along three specific layers of tissue (Dartos, Colles, and Scarpa’s fascias), which could also affect the abdominal wall early on. Doctors might overlook this disease in its early stages because the tissue covering the affected area might look normal or just appear inflamed.

Recognizing and treating this potentially deadly disease early on is crucial because it could be easily mistaken for a less serious condition at first. An early diagnosis can make a significant difference and could potentially be lifesaving.

What Causes Fournier Gangrene?

This condition happens due to an infection caused by diverse types of bacteria affecting the fascia (a band of tissue beneath the skin) and the soft tissues just beneath the skin. Common bacteria like Group A Streptococci, Staphylococcus aureus (both gram-positive bacteria), E. Coli, and Pseudomonas aeruginosa (both gram-negative bacteria) are often found in the infections of patients with Fournier gangrene.

These bacteria can be introduced from multiple sources. They can come from urinary tract infections, bowel infections, skin infections, or even infection starting from as small as a pimple. These bacteria can start an infection which can grow and develop into Fournier gangrene.

Surgeries or procedures in the genital and perineal area (the area between the anus and the genitals) can sometimes provide the starting point for bacteria to enter the tissues. Any injury or break in the skin in the perineal or scrotum area can give bacteria access to the tissues there, beginning the process which in some cases may lead to Fournier gangrene. It’s important to know that about 25% of cases occur without a clear cause.

Risk Factors and Frequency for Fournier Gangrene

Fournier gangrene is a rare infection that makes up less than 0.02% of all hospital admissions. It is more commonly seen in males, with a ratio of 10 males to every female affected. Men between the ages of 50 to 79 are the most commonly affected, especially in the southeastern United States, which has the highest reported rate of this disease. However, this condition is not exclusive to males; it also affects females, who often become severely ill when affected by it.

While some healthy individuals can develop Fournier gangrene, it is often associated with people who have weak or compromised immune systems. This condition is most often seen in males over 50 years of age who have diabetes and a history of excess alcohol consumption. Those with multiple other health issues tend to have worse outcomes.

Fournier gangrene has a number of known risk factors:

• Hardening of the arteries (Atherosclerosis)
• Chemotherapy
• Chronic alcohol abuse (seen in 25% to 50% of cases)
• Chronic use of steroids
• Colorectal cancer
• Diabetes (seen in 20% to 70% of cases)
• Drug abuse (especially injecting drugs into groins or penis)
• HIV infection
• Having a compromised immune system
• Inflammatory bowel disease
• Leukaemia
• Liver failure or cirrhosis
• Being male (seen in 90% of cases)
• Malnutrition
• Having a neurogenic bladder
• Obesity
• Perianal abscess
• Peripheral vascular disease
• Prostate cancer

Further risk factors include trauma or surgery, rectal fistula, renal failure, significant heart disease, spinal cord injury, urethral strictures, and use of certain diabetic medications.

Despite improvements in treating sepsis, the mortality rate for Fournier gangrene remains around 40%. Delay in diagnosis and treatment can increase the mortality rate to 88%. The most crucial factor affecting mortality is the timing of surgical intervention; early surgery can halve the death rate. Conditions such as severe diabetes, heart disease, and renal failure significantly increase the mortality rate.

Signs and Symptoms of Fournier Gangrene

Fournier gangrene is a severe infection that affects the genital or perineal areas. Because the early symptoms may be minor and only get severe over time, it can be hard to diagnose early. Medical professionals should be vigilant if they see any infection in the perineal or scrotal areas, especially if there’s disproportionate pain compared to how the infected area looks. Such infections may quickly get worse and lead to life-threatening complications.

Males and those with diabetes, high blood pressure, cancer, immune system disorders, and alcohol addiction are at a higher risk of contracting Fournier gangrene. Other risk factors include a history of trauma to the genital area, recent surgeries, and systemic symptoms such as fever, chills, nausea, vomiting, urinary retention, and fatigue.

The earliest indications of this disease might include itching, tenderness, edema (swelling), and erythema (redness), mainly involving the skin of the perineum, scrotum, or labia. These signs can easily be mistaken for simpler infections like cellulitis, erysipelas, and impetigo. However, Fournier gangrene typically shows up with sporadic bulla (fluid-filled blisters), widespread swelling, and patchy redness. Some visible physical signs include abrasions or soft, fluctuating swollen areas on the skin.

Although primarily a disease for men, women also face an increasing risk. Also, the incidence is higher in females who are severely overweight. In women, it usually affects the labial or vulvar regions with the presentation as cellulitis.

Fournier gangrene can be challenging to diagnose early, as approximately 40% of patients show no symptoms, and up to 75% of cases can be misdiagnosed at first. This misdiagnosis contributes to its high mortality rate. A typical indication of Fournier gangrene is an intense pain that seems disproportionate to the visible physical findings.

As the infection progresses, further signs include crepitus on touch, purpura (purple skin spots due to bleeding), bulla, and patchy dark tissue discoloration.

Testing for Fournier Gangrene

If your doctor suspects you might have Fournier gangrene, a severe infection that affects the genital area, they’ll use a combination of blood tests, visual inspection, and medical imaging to diagnose it. This is especially true for men who are older, diabetic, or at a high risk for infections.

Your doctor will use a complete blood count (CBC) to check the number of different cells, including white blood cells, in your blood. Elevated levels of specific white blood cells can indicate an infection. A comprehensive metabolic panel (CMP) is another test that helps your doctor check if there are any abnormalities in your body’s electrolyte and acid levels. It can also point out if you have kidney problems. Other tests, like serum lactate, c-reactive protein, and procalcitonin, can help determine if the infection has spread to your bloodstream or caused sepsis, a life-threatening reaction to infection. These and other tests may be used to guide the type of antibiotics to be used for your treatment.

Despite all these tests, imaging is key in confirming the severity and extent of the infection. However, it’s important to note that imaging can’t definitively diagnose Fournier gangrene on its own. Moreover, if you are not stable or if there’s a delay in getting your images, your doctor won’t wait for imaging results to start treatment.

One of the quick ways your doctor can get images and evaluate the case is by ultrasound. It helps show any gas or air pockets in the soft tissues that are beneath the skin. Ultrasound can detect changes in tissues or fluid collections that cannot be seen or felt in a physical examination. In fact, if gas is seen in the scrotum, it’s a strong sign of Fournier gangrene.

X-ray imaging can also show gas in the affected area. While not everyone with Fournier gangrene will have gas in the tissues, it’s found in 90% of the cases. It’s also important to note that the absence of gas does not rule out the possibility of Fournier gangrene.

CT scans are exceptionally useful in diagnosing Fournier gangrene. They are considered the best way to identify changes around the structures involved and abnormal fluid collections. CT scans can also help locate the origin of the disease and rule out other diseases. After surgery, doctors often use CT scans to check for new infection pockets and monitor how the wounds are healing.

While MRI scans provide detailed images of the soft tissues involved, they are not recommended as the primary diagnostic tool for suspected Fournier gangrene since they are costly and take longer to perform.

In summary, while diagnosing Fournier gangrene involves several steps, the key is not to delay treatment, especially if the patient is unstable. If Fournier gangrene is suspected, it’s better to start treating it immediately, even if that means retrospectively discovering that the treatment may have been unnecessary.

Treatment Options for Fournier Gangrene

Fournier gangrene is a serious condition that is generally regarded as a medical emergency. Treatment involves a combination of surgical procedures and medication. Usually, patients affected by Fournier gangrene are already in a critical condition, and likely have severe infections and shock.

Medical treatment for this disease starts with administering a broad range of antibiotics to combat the infection while waiting for the results of specific tests. The antibiotics are usually given in a combination to fight against a variety of bacteria like staphylococcus, streptococcus, Pseudomonas, and clostridium, which can all contribute to Fournier gangrene. The patient typically remains on these medications for at least two weeks.

Apart from antibiotic treatments, it’s vital to restore fluid balance in the patient, as many arrive with low blood pressure and septic shock. In some cases, medications to raise blood pressure may be needed if the person’s blood pressure doesn’t normalize with fluids alone. Any abnormalities in electrolytes should also be corrected, and for diabetic patients, any blood sugar abnormalities need to be fixed.

Crucially, while these medical treatments are being initiated, there should be no delay in carrying out urgent surgery to remove the infected tissue. Any time wasted can increase the risk of harm and death in affected patients.

In the case of surgical interventions, the first step is to thoroughly remove, or debride, all the dead and diseased tissue. A team of specialized surgeons may undertake this procedure, including urologists, general surgeons, plastic surgeons, colorectal surgeons, and specialists in female reproductive health. The choice of specialists depends on the patient’s particular needs. The surgical procedure should be completed as quickly as possible after the patient’s arrival at the hospital, as rapid intervention has been linked to better patient outcomes.

In some cases, the testicles can be temporarily placed in a protective pocket, typically constructed in the upper inner thighs, to safeguard their function and hydration. In case the rectum or anus is involved in the infection, a procedure called a colostomy can be performed to divert feces away from the area to aid in healing.

A few additional treatments can help manage Fournier gangrene, although they are secondary to medical and surgical treatments. For instance, hyperbaric oxygen therapy, which involves exposing the patient to pure oxygen at high pressure, can be beneficial after surgery and is shown to reduce disease-related complications and enhance survival. However, it should not delay surgical intervention. Other treatments like application of honey, intravenous immunoglobulins, and therapeutic plasma exchange have been suggested, but there is insufficient evidence on their effectiveness.

In conclusion, treatment of Fournier gangrene centers on immediate surgical intervention combined with broad-spectrum antibiotics. Understanding the urgency of this disease can significantly determine survival outcomes.

What else can Fournier Gangrene be?

When a doctor is trying to diagnose Fournier gangrene, they must also consider other conditions that can present with similar symptoms. These may include the following:

• Acute renal colic, urolithiasis (Kidney stones)
• Cellulitis (skin infection)
• Chancre (a symptom of syphilis)
• Deep vein thrombosis (blood clot)
• Epididymitis (inflammation of the epididymis behind the testicle)
• Erysipelas (skin infection)
• Gangrenous balanitis (Gangrene of the penis)
• Gangrenous vulvitis (Gangrene of the vulva)
• Gas gangrene (particularly severe skin infection)
• Herpes simplex (a viral infection)
• Inguinal lymphogranulomatosis (a sexual transmitted infection)
• Invasive candidiasis (a fungal infection)
• Mucormycosis (a type of fungus infection)
• Orchitis (inflammation of one or both testicles)
• Perianal/periurethral abscess (collection of pus at the back side or around the urethra)
• Pyoderma gangrenosum (Rare, ulcerative skin condition)
• Pyomyositis (a bacterial infection inside a muscle)
• Pyonephrosis (condition when pus accumulates in the kidneys)
• Scrotal abscess (collection of pus in scrotum)
• Scrotal edema (swelling of the scrotum due to fluid accumulation)
• Stevens-Johnson syndrome (a severe skin reaction)
• Syphilis (a sexually transmitted disease)
• Testicular torsion (twisting of the spermatic cord)
• Toxic epidermal necrolysis (a serious skin disorder)
• Toxic shock syndrome (an infection caused by certain bacteria)
• Vasculitis (inflammation of the blood vessels)

What to expect with Fournier Gangrene

The severity and potential outcomes of Fournier gangrene, a severe infection, can be influence by many factors.

A study from 1995 showed that the Fournier Gangrene Severity Index (FGSI) – a score created from several health indicators like temperature, heart rate, breathing rate, and certain blood tests – can be helpful in predicting the patient’s outcome. People with a FGSI score greater than 9 had a more than 75% chance of dying from the condition, while those with a score less than 9 had a 78% chance of surviving it. Other blood abnormalities like high calcium and low magnesium also suggested a worse outcome.

Factors like patient age and the extent of tissue affected by the disease also impact the outlook. As a person gets older, their chances of recovery decrease. Similarly, the more tissue is infected, the worse the potential outcome. A scoring system that included both of these factors, called the Uludag FGSI (UFGSI), was developed in 2010. It shows that a score greater than 9 corresponds to a 94% risk of death, while a score less than 9 suggests an 81% chance of survival.

The sAPGAR and ACCI scores are two other systems for predicting how a patient might fare during and after surgery. The ACCI score, which assigns points to 19 different health conditions, predicts that higher scores are associated with greater risk of death. The sAPGAR score predicts the risk of complications after surgery based on estimated blood, the lowest mean blood pressure, and the lowest heart rate. Both scores can also be used for estimating outcomes in Fournier gangrene, even though they aren’t specific to this disease. A comparison of these systems showed that the ACCI was the most accurate, as well as being among the easiest to use.

The timing of disease onset to surgical treatment plays an important role in the outcome. Patients who get treated earlier in the course of the disease have better outcomes. Additionally, diabetic patients with a blood sugar control measure (HgA1c) greater than 7 tend to have a worse prognosis.

Possible Complications When Diagnosed with Fournier Gangrene

Fournier gangrene is a very serious condition with devastating effects on patients. It has a high death rate and can cause problems short-term and long-term.

Short-term problems are caused by the body’s response to this aggressive infection. This can lead to a wide range of issues like:

• Acute kidney failure
• Severe lung distress
• Heart irregularities and failure
• Failure of multiple organs
• Bacteria in the blood

Bacteria in the blood can cause clotting problems leading to strokes and blocked blood vessels in the legs that might need amputation. The multiple surgeries can cause things like wound infection and intestinal blockage. Treatments like hyperbaric oxygen are aimed at reducing the risk of wound infections.

In cases where the infection has significantly spread to the anal area, it can lead to a loss of control over bowel movements. This requires the formation of an artificial opening for the discharge of body wastes (colostomy), which can have its own complications. Common issues include:

• Protrusion of internal organs through the stoma (open wound)
• Infection in the stoma

Since the disease often affects the penis, urinary tract infections are common, and there could be difficulty passing urine due to swelling near the urethra (tube that carries urine out of the body). Treating this involves methods like urinary catheterization and cystostomy for some patients.

Patients with Fournier gangrene also experience psychological problems due to the damaging effect on their genitals. This can lead to:

• Long-term pain
• Decreased quality of life
• Depression
• Painful, disfiguring scars
• Sexual dysfunction due to impaired penile function and twisted penis
• Loss of penile sensitivity and pain during erections

Preventing Fournier Gangrene

Fournier gangrene is a rare but very serious infection that occurs in the genital and perineum area (the region between the anus and the genitals). This condition can cause symptoms such as swelling and significant pain in the genital and perineum area. It is also common for patients to experience overall body symptoms as well.

Even though there’s no sure way to prevent Fournier gangrene, there are certain steps that can help reduce the chances of getting it. For example, if you have diabetes, you should regularly check your groin area for any signs of redness, swelling, or pain. Managing your diabetes well by taking your medication as prescribed can also be helpful. If a person with diabetes gets Fournier gangrene, they tend to have a better outcome if their HgA1c levels (a blood test that measures average blood glucose control over the past 2 to 3 months) are below 7.

Practicing good personal hygiene and taking care of your skin can also help prevent Fournier gangrene. This is important because when your skin breaks down, it may increase the risk of developing this disease.