Fournier Gangrene (FG) is a rare kind of necrotising fasciitis, which is a ‘flesh-eating disease’ caused by a variety of bacteria. It mainly affects the genitals and perineum (skin between the anus and vulva, or anus and scrotum). It is life threatening and requires urgent treatment with surgery and antibiotics. Males are 10 times more likely to develop FG compared to females. Cases in minors are incredibly rare, and how the disease manifests in childhood is poorly understood. However, cases in those as young as 8 days old have been reported. Fortunately, paediatric cases have a better survival rate and require less aggressive approaches.¹

Understanding FG

Fournier is the French doctor who first identified this kind of necrotising fasciitis of the perineal and genital region in males².  Gangrene means tissue death either due to bacterial infection or a lack of blood reaching the area. FG can affect the muscle fascia and subcutaneous tissue:

• Fascia is a kind of soft connective tissue that surrounds bones, organs, blood vessels, nerves and muscles to keep them in place
• Subcutaneous tissue is a deep layer under the skin that is made of fat cells and connective tissue. It helps keep us warm by acting as insulation, and can be used as an energy store

Our bodies carry around 60,000 miles worth of blood vessels, which include arteries, veins, and capillaries. Arteries are essential for transporting oxygen and nutrients in the blood from the heart to other parts of the body. The pudendal artery moves blood towards the perineum, scrotum, and the reproductive organs. In FG, bacteria enter the muscle fascia and subcutaneous tissue, which can begin to destroy parts of the pudendal artery. When this occurs, the bacteria can begin to spread even further, destroying the blood vessels of the perineum and sex organs. If the disease is left untreated, it can even begin to affect the stomach, chest, and thighs. By the time that you’re able to see the disease on the skin surface, the tissue underneath the skin has already been destroyed.

FG can be fatal and requires immediate medical attention, even a few hours of inaction can lead to death. FG can be caused by a variety of bacteria, and in very few cases, fungi.³ The most common bacteria found in paediatric FG are Streptococci and Staphylococci.⁴

Risk Factors

Paediatric cases of FG are incredibly rare, with only 56 being reported in scientific literature.¹ However, there are risk factors associated with paediatric FG. These include:^1-4

• Having a weakened immune system (immunocompromised). This could be from HIV, types of blood cancers, and chemotherapy⁵
• Being born premature 
• Nappy rash 
• Chickenpox 
• Bug bite 
• UTI or a bladder infection
• Circumcision or genital mutilation³

Symptoms

FG tends to develop over a course of 2-7 days, and can be split into about five stages:²

• Fatigue and fever within 2-7 days
• Swollen, or tender genitals or perineum. You might also begin to see fluid collecting in the affected area(s)
• Increased pain and tenderness, with an increasing red colour
• The skin can begin to darken, taking on a purple colour. Crepitus can also occur, which is when you hear a crackling or popping sound when pressure is applied to the affected area. This is due to the bacteria producing gas that gathers in the tissue³
• A thick, milky fluid can begin to leak from the tissue. At this point, the skin can even turn black

Other symptoms of FG might include:

• A foul odour from the genitals or perineum
• Nausea and vomiting
• Sepsis
• A quick heartbeat
• Itching in the affected areas

If your child has red, tender or swollen genitals or perineum AND a fever above 38℃ or is generally ill, you should go to A&E immediately. 

Diagnosis

Diagnosis mainly involves a doctor looking at the child’s skin and assessing symptoms. In some cases, additional imaging and tests may be performed to confirm a diagnosis. These include:

• CT scan: this is used to look for fluids and gases in parts of the body, and see where the infection has come from
• Ultrasound: this can help differentiate FG from other conditions like cellulitis and help identify air in soft tissues
• X-ray: this can help your healthcare provider see where the gas has spread in the soft tissue
• Blood tests: your doctor might order blood tests to help identify increased white blood cell count, which can indicate an active immune response to bacterial infection. These tests can also identify septic shock and atypical electrolyte levels, which are common in FG

If your healthcare provider strongly suspects a case of FG, they may put your child on antibiotics before the results for the tests come back. If it’s a severe case, your doctor may skip diagnosis entirely and go straight to treatment.

Treatment

Early treatment is essential for the best outcome. FG always requires hospitalisation, and the duration depends on the severity of the infection.

Surgery

Unless the necrotic tissue has fallen off on its own, it will need to be removed during surgery. This is known as debridement, and is the main treatment method for FG. This typically requires about 3 surgeries, and only the damaged tissue will be removed. In severe cases, the entire penis and scrotum may need to be removed. Afterwards, reconstructive surgery can be performed to help return the skin to how it used to look. In some cases, a colostomy or cystostomy may be required to prevent the surgical wound being contaminated with more bacteria.¹

Hyperbaric oxygen therapy

This is an adjunct to the other treatment methods, meaning it can help treat FG, but is not used as a primary form of treatment. This involves inhaling pure oxygen in a pressurised room. Although the efficacy of this adjunct is not clear, it has been reported to help with:

• Wound healing after surgery
• Reducing the damage to the blood vessels
• Preventing further growth of bacteria
• Antibiotics reaching the area²

Antibiotics

Your child will likely be injected with antibiotics through their veins and/or muscles for about 2 weeks in total, starting before surgery.² Typical antibiotic treatment involves administration of three antibiotics to cover a range of bacteria that could be causing the infection. Triple therapy involves:²

• A third generation cephalosporin
• An aminoglycoside or penicillin
• Metronidazole or clindamycin³

Culture tests may also be performed, where a sample is taken from the tissue to identify the specific bacteria causing your child’s FG. If specific bacteria can be identified, the course of antibiotics may be changed by your doctor to target these specifically. 

Third generation cephalosporins⁷

These antibiotics work by preventing the bacteria from forming their cell wall, which gives the bacterial cell its structure and shape. Once this cell wall is compromised, the bacterial cells die due to the cellular contents leaking out. Examples of these drugs include cefixime, ceftriaxone, and ceftazidime. Side effects of these drugs include:

• Diarrhoea
• A hypersensitivity reaction, which occurs when your immune system overreacts. There are many kinds of hypersensitivity reactions, and they can occur immediately or over time
  

Aminoglycosides

These antibiotics work by weakening the bacterial cell membrane.⁸ This means that the cell can no longer control what is entering and exiting the cell, and can lead to death of the cell. The production of proteins is inhibited by binding to the ribosome of the bacterial cell, which is like a ‘protein production factory'.⁸ This type of antibiotic includes streptomycin and gentamicin. Side effects of aminoglycosides include:

• Nausea and vomiting
• Kidney damage
• Hearing loss

Penicillins⁹

These are one of the most commonly used antibiotics. They work by preventing the bacterial cell from forming its cell wall. Side effects include:

• Nausea and vomiting
• Seizures, which are more common in those with kidney problems 
• Diarrhoea

Metronidazole

This antibiotic is thought to prevent DNA and RNA from being made. These nucleic acids serve as the instructions for cells, and when they are no longer made, the cells die. Side effects of this medication can include:

• Nausea and vomiting
• Headaches
• Drowsiness 
• Urine turning a darker colour

Clindamycin

This antibiotic prevents bacteria from making proteins needed for their growth,  thus preventing further infection.¹⁰ Side effects include:

• Diarrhoea
• Nausea and vomiting
• Stomach pain 

If your doctor suspects a fungus may play a role in your child’s FG, they may also be put on an antifungal medication.^2,3

Prevention

You can help prevent your child from developing FG by:

• Cleaning the genital area and perineum well after each nappy change
• Teaching your child the importance of keeping their genital and perineal area clean
• Keeping an eye on any open wounds in the genital or perianal region and making sure they are cared for

Summary

FG is a rare and life-threatening form of necrotising fasciitis that can affect the genitals and perineum of children as well as adults. It progresses rapidly, destroying tissue and requiring urgent treatment. Fortunately, survival rates are higher in paediatric cases and treatment is less aggressive than in adults. Symptoms include severe pain, swelling, and discoloration in the affected areas, often accompanied by fever. Surgical removal of dead tissue, antibiotic therapy, and supportive measures like hyperbaric oxygen therapy are usually enough to treat this disease.