Clostridial Myonecrosis

Disease Overview

Clostridial myonecrosis is a serious, life-threatening bacterial infection caused by a small number of Clostridium species of bacteria. Clostridial myonecrosis is broken down into two main forms:

Traumatic form, mainly caused by Clostridium perfringens species, most often arise in individuals who have suffered an injury or trauma that hinders or limits blood supply to the area (vascular compromise).

Spontaneous form, mainly caused by the Clostridium septicum species and most often arising in people with a weak or suppressed immune system, due to an underlying medical condition, most often cancer of the colon or rectum or other conditions such as blood (hematological) cancers like leukemia, diabetes mellitus and cyclic neutropenia.

Clostridium septicum is not part of the normal human gut microbiota. It is thought that the associated illnesses cause maladaptation and imbalance (dysbiosis) allowing Clostridium septicum to populate the gut. The bacteria can exist within the gastrointestinal system but are kept under control by the immune system. In some immune-suppressed individuals, the infection can become dangerous.

Clostridial myonecrosis is the term used when these clostridial infections spread to affect deep muscle tissue. There are many complications that can develop including pain, gas gangrene (a condition in which gas production and build up within muscle tissue leads to tissue death [necrosis]) and sepsis, a severe blood infection. These infections eventually progress to cause toxic shock and multiple organ failure.

Clostridial myonecrosis is a rapidly progressive, medical emergency that can be fatal despite treatment. Prompt diagnosis and aggressive treatment are essential.

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Synonyms

• Clostridium myonecrosis
• Clostridium septicum infection
• Clostridium perfringens infection

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Signs & Symptoms

Clostridial myonecrosis initial signs and symptoms may include:

• Pain, which is often the presenting symptom and may be severe and develop rapidly.
  • In individuals with Clostridium perfringensinfection following surgery, pain may arise shortly after the surgical procedure and be disproportionately strong.
  • In Clostridium septicuminfection, there is often sudden, severe onset of muscle pain. Sometimes, there is a sensation of heaviness, pressure, or numbness.
  • Some individuals with the spontaneous form may initially show signs of confusion or exhibit a general feeling of poor health (malaise)
• Fever
• Fatigue
• Dehydration

Gas gangrene and clostridial myonecrosis are interchangeable terms used to describe an infection of muscle tissue by toxin-producing clostridia. Clostridial infection produces toxins in the body. These toxins produce gas. This gas becomes trapped within deep muscle tissue, specifically the soft tissues. This contributes to tissue death and decay called necrosis.

Because clostridial myonecrosis affects deep muscle tissue initially, the skin near the infection site may be unaffected at first. Therefore, with time, other symptoms may include

• Skin that may become pale and over time usually darkens to reddish or brownish color
• Formation of bullae, air- or fluid-filled sacs that form under a thin layer of skin and that may become air- or blood-filled blisters called blebs
• Skin that may turn a black or dark green color
• Pain and tenderness of the affected area with a grating or crackling sound (crepitation) from underneath the skin
• Foul-smelling discharge from the infection site
• Localized hardening of affected soft tissue (induration)
• Swelling due to fluid accumulation (edema)
• Broken and melting of the skin before shedding off (sloughing)
• Aortitis, the spreading of the infection to the aorta, the main artery of the body
  • The wall of the artery may balloon or bulge outward (aneurysm) and there is a risk of the aorta rupturing.
• Sepsis, a widespread blood infection affecting other parts of the body which may occur as the infection progresses
• In a sepsis, the body responds by releasing chemicals to fight off the infection and these chemicals bring about an inflammatory response in the body, which may result in the following symptoms:
• Rapid heartbeat (tachycardia)
• Abnormally rapid breathing (tachypnea)
• Low blood pressure (hypotension)
• Changes in mental status including greatly dulled awareness or consciousness (stupor)
• Toxic shock, a life-threatening condition characterized by a sudden, high fever, sore throat, vomiting, diarrhea, muscle aches, headaches, confusion, disorientation, rash and seizures, and, which, ultimately may lead to the failure of multiple organs

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Causes

Clostridial myonecrosis is caused by infection with species of the Clostridium genera of bacteria. Clostridia are found throughout nature. They are found in soil and marine sediments and can be found in animal and human gastrointestinal tracts. Two specific species, Clostridium perfringens and Clostridium septicum are the cause of most cases of clostridial myonecrosis. These two species produce toxins that cause certain cells to breakdown prematurely and damage blood vessels. They may promote the formation of blood clots and decrease or depress heart muscle contractions. These toxins also produce a gas that becomes trapped within deep muscle tissue causing tissue death and decay (myonecrosis or gas gangrene).

Clostridium infection can occur in individuals who have recently experienced trauma or surgery. This is known as the trauma and or wound-related form. It is most often caused by Clostridium perfringens but can also be caused by Clostridium histolyticum Clostridium novyi, Clostridium bifermentans and Clostridium fallax. A person is exposed to the bacteria through the open wound. However, the bacteria will not grow and spread unless the environment around the wound supports its growth and spread. These bacteria do not need oxygen to survive and if the injury or wound limits or hinders blood supply to the affected area, this can create an environment that promotes the growth and spread of the bacteria. The bacteria will spread to surrounding tissue including deep muscle tissue. The traumatic form of clostridial myonecrosis is associated with a variety of traumatic conditions including gunshot or deep knife wounds, compound fractures and intramuscular injections. It may also be associated with bowel and biliary tract surgery or abortions. Pregnant women who have a retained placenta or prolonged rupture of fetal membranes or experience the death of a fetus (intrauterine fetal demise) may also be at risk.

The spontaneous form of infection occurs when there is no obvious route of infection. It is also called the nontraumatic or idiopathic form. It is usually caused by Clostridium septicum and usually develops in individuals with a suppressed immune system and cancer of the colon or rectum. It also occurs in individuals with blood cancers like leukemia, inflammatory bowel disease, diverticulitis, lymphoproliferative disorders, cyclic neutropenia, or acquired immune deficiency syndrome (AIDS). Individuals who have undergone radiation therapy or gastrointestinal surgery may also be at risk. It is thought that the infection enters the bloodstream through an injury or lesion within the gastrointestinal system where it is carried to other tissues of the body including deep muscle tissue (hematogenous muscle seeding). Clostridium septicum does not need a low oxygen environment to grow and spread in the body and it is more aerotolerant than Clostridium perfringens.

In addition, a toxic shock–like syndrome associated with Clostridium sordellii infection has been increasingly recognized in people skin-popping black tar heroin and in women undergoing childbirth or other gynecologic procedures, including medically induced abortion.

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Affected populations

Clostridial myonecrosis is a rare infection. Approximately, one in 1,000-3,000 people are reported each year in the United States. Most affected individuals are adults, but these infections can occur in individuals of any age including children. About 80% of these infections are traumatic clostridial myonecrosis. Of these, about 70% are caused by Clostridium perfringens.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of clostridial myonecrosis. Comparisons may be useful for a differential diagnosis.

Necrotizing fasciitis (NF) is a rare infection that means “decaying infection of the fascia,” which is the soft tissue that is part of the connective tissue system that runs throughout the body. NF is caused by one or more bacteria that attacks the skin, the tissue just beneath the skin (subcutaneous tissue) and the fascia causing these tissues to die (necrosis). These infections can be sudden, vicious and fast spreading. If not treated quickly with antibiotics and/or debridement of the infected tissue, the patient may develop toxic shock syndrome or toxic shock-like syndrome, which may lead to multiple organ failure and death.

Fournier gangrene is an acute necrotic infection of the scrotum; penis; or perineum. It is characterized by scrotum pain and redness with rapid progression to gangrene and sloughing of tissue. Fournier gangrene is usually secondary to perirectal or periurethral infections associated with local trauma, operative procedures, or urinary tract disease. Since 1950, more than 1,800 cases for study have been reported in English language medical literature. This disease occurs worldwide and, although it is recognized more frequently among male adults, has been identified also among females and children. Treatment usually consists of the surgical removal (debridement) of extensive areas of dead tissue (necrosis, necrotic) and the administration of broad-spectrum intravenous antibiotics. Surgical reconstruction may follow where necessary.

There are several infectious diseases or conditions that can resemble clostridial myonecrosis. These conditions include infection due to group A Streptococcus bacteria and Staphylococcus aureus; wound infection due to Vibrio vulnificus; muscle infection characterized by muscle abscesses (pyomyositis); muscle infection due to viral agents (viral myositis) and rhabdomyolysis, a general condition of skeletal muscle breakdown due to a variety of causes.

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Diagnosis

A diagnosis of clostridial myonecrosis is based upon identification of characteristic symptoms, a detailed patient history, a thorough clinical evaluation and a variety of specialized tests including a culture positive patient sample.

Certain physical signs may support a diagnosis of traumatic clostridial myonecrosis. These include pain at the site of a wound or surgery and signs of systemic toxicity or gas in the soft tissues such as a grating or crackling sound (crepitation) from underneath the skin. Spontaneous clostridial myonecrosis may be suspected in individuals with rapid onset of pain and fever, particularly in the arms and legs, in the absence of trauma or injury. The traumatic form is easier to diagnose.

Clinical Testing and Workup
Blood tests can reveal findings that are indicative of clostridial myonecrosis. Muscle damage and loss can lead to elevated levels of aldolase, potassium, lactate dehydrogenase and creatine phosphokinase levels. White blood cell levels may be low. Sometimes, anemia (low levels of red blood cells) is seen.

X-rays may reveal fine gas bubbles in affected soft tissue and skin tissue. Specialized imaging techniques called computerized tomography (CT) scanning and magnetic resonance imaging (MRI) can reveal gas outside of the gastrointestinal tract (extraluminal gas). During CT scanning, a computer and X-rays are used to create a film showing cross-sectional images of certain tissue structures. An MRI uses a magnetic field and radio waves to produce cross-sectional images of organs and bodily issues. CT scanning and MRIs can also be used to determine whether an infection is localized to one site of the body or has spread to other areas.

Laboratory technicians will also run a test called a gram stain. Bacteria may be classified as gram negative” or “gram positive”, depending upon the results of the staining test. This method involves staining bacteria with various solutions and dyes that help to identify and classify the bacteria based on the composition of their cellular components. This rapid staining technique when combined with microscopy gives physicians early clues about the underlying cause of their patient’s conditions. In individuals with clostridial myonecrosis, a gram stain of discharge from the wound may reveal “gram positive” rods (rod-shaped bacteria) and the absence of polymorphonuclear cells. These cells are inflammatory cells that are normally found at the sites of injury within the body but are not found when this clostridial infection is involved. Researchers believe that toxins produced by some members of the infectious Clostridia prevent polymorphonuclear cells from reaching infected tissue.

Blood cultures, which are tests that can detect and classify bacteria found in the blood, may be useful for diagnosing spontaneous clostridial myonecrosis and Clostridial septicum infection. Bacteria in the blood usually precedes skin signs by several hours.

Exploratory surgery of the wound may reveal characteristic findings including muscles that do not bleed or contract upon stimulation, swelling and discoloration. A tissue sample may be removed and studied under a microscope (biopsy sample) to other characteristic changes in muscle tissue.

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Standard Therapies

Clostridial myonecrosis, commonly called gas gangrene, is a fast-moving and life-threatening infection. Immediate treatment is crucial for survival, as the infection can spread rapidly through the body. Early diagnosis and prompt action are vital, with surgical consultation and aggressive antibiotic therapy being the first steps.

Treatment may require a team of specialists including a specialist in diagnosing and treating infectious diseases (infectious disease specialist); a specialist in diagnosing and treating skin disorders (dermatologists); general, trauma, or burn surgeons; plastic surgeons; social workers and other healthcare professionals may need to systematically and comprehensively plan treatment. Psychosocial support for the entire family is essential as well.

Treatment may include:

• Initial treatment: Affected people should receive broad-spectrum IV antibiotics immediately, without waiting for lab cultures, since delaying treatment could be fatal. Antibiotics like vancomycin, tazobactam, or carbapenem are recommended. If gas gangrene or necrotizing fasciitis is suspected, a combination of penicillin and clindamycin is added. Clindamycin is essential as it blocks bacterial toxins from spreading, even though it needs to be combined with another antibiotic since it’s bacteriostatic (it stops bacteria from growing rather than killing them outright).
• Surgical debridement: Debridement (removing dead tissue) is essential. The surgeon focuses on cutting away all necrotic (dead) and infected tissue to stop the infection from spreading. This may require multiple surgeries, depending on how quickly the infection progresses. In severe cases, large amounts of tissue or even limbs may need to be amputated to save the person’s life. Thorough cleaning (irrigation) of the wounds with sterile saline is also done during surgery.
• Hyperbaric oxygen therapy (HBO): Some studies have reported good results with HBO therapy when combined with antibiotics and surgical debridement. The affected person is placed in a pressurized chamber where they breathe 100% oxygen, which helps inhibit toxin production, promotes healing and reduces tissue swelling by encouraging blood vessel growth. It also enhances the effectiveness of antibiotics. The process typically starts with two treatments a day, which are then reduced to once a day as the affected person stabilizes. However, the use of HBO is still controversial, and it is not without risks, as moving away from the intensive care unit (ICU) for this therapy can be dangerous, and side effects like oxygen toxicity (which can cause seizures) and ear or lung injuries from pressure changes are possible.
• Supporting therapies: IV fluids and ICU monitoring are necessary to manage the patient’s overall condition. In certain cases, tetanus shots are given if the patient hasn’t been vaccinated recently. Additionally, negative pressure wound therapy (NPWT), where a vacuum dressing is used to promote healing, might be considered after the infection is controlled. If affected individuals have not received immunization against tetanus within 10 years, then a booster vaccination against tetanus may be recommended.
• Multidisciplinary care: Because gas gangrene affects multiple systems, a team of specialists is often involved. This might include a general surgeon, orthopedic surgeon, infectious disease expert, plastic surgeon and others depending on the location of the infection. For example, a urologist would be involved if the infection affects the genital area (Fournier’s gangrene) and a gynecologist might be necessary if uterine gas gangrene is involved.
• Psychosocial support: Given the severe nature of gas gangrene, emotional and psychological support for both the patient and their family is crucial throughout the treatment process.

In conclusion, gas gangrene is a true surgical emergency. Early antibiotic therapy, rapid surgical intervention to remove infected tissue and possibly hyperbaric oxygen therapy can significantly reduce the fatality rate. Comprehensive care with a multidisciplinary team is essential to managing this serious condition.

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at https://clinicaltrials.gov/. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/

For information about clinical trials sponsored by private sources, contact:
https://www.centerwatch.com/

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

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References

JOURNAL ARTICLES
Hussain H, Fadel A, Garcia E, Hernandez RJ, Saadoon ZF, Naseer L, Casmartino E, Hamad M, Schnepp T, Sarfraz R, Angly S, Jayakumar AR. Clostridial myonecrosis: a comprehensive review of toxin pathophysiology and management strategies. Microorganisms. 2024 Jul 18;12(7):1464. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11278868/

Mallozzi, Michael JG, et al. Trusting yourgGut: Diagnosis and management of clostridium septicum infections. Clinical Microbiology Newsletter, Volume 38, Issue 23, 187 – 191

Srivastava I, Aldape MJ, Bryant AE, Stevens DL. Spontaneous C. septicum gas gangrene: A literature review. Anaerobe. 2017;48:165-71.

Burnham JP, Kollef MH. Treatment of severe skin and soft tissue infections: a review. Curr Opin Infect Dis. 2018;31:113-119. https://www.ncbi.nlm.nih.gov/pubmed/29278528

Jessamy K, Ojevwe FO, Ubagharaji E, et al. Clostridium septicum: an unusual link to a lower gastrointestinal bleed. Case Rep Gastroenterol. 2016;10:489-493. https://www.karger.com/Article/FullText/448881

Shah A, Yousuf T, Rachid M, et al. Clostridium septicum aortitis of the infrarenal abdominal aorta. J Clin Med. 20016;8:168-174. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4701074/

Stevens DL, Bisno AL, Chambers HF, et al. Practice guidelines for the diagnosis and management of skin and soft tissue infectious: 2014 update by the Infectious Diseases Society of America. Clin Infect Dis. 2014;59:e10-52. https://academic.oup.com/cid/article/59/2/e10/2895845

El Sayad M, Chikate A, Ramesh B. Gas gangrene presenting with back pain. BMJ Case Rep. 2014;2014. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4025197/

Stevens DL, Aldape M, Bryant AE. Life-threatening clostridial infections. Anaerobe. 2012;18:254-259. https://www.ncbi.nlm.nih.gov/pubmed/22120198

Khalid M, Lazarus R, Bowler ICJW, Darby C. Clostridium septicum and its implications. BMJ Case Rep. 2012;2012. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4543009/

Bryant AE, Stevens DL. Clostridial myonecrosis: new insights in pathogenesis and management. Curr Infect Dis Rep. 2010;12:383-391. https://www.ncbi.nlm.nih.gov/pubmed/21308521

Smith-Slatas CL, Bourque M, Salazar JC. Clostridium septicum infections in children: a case report and review of the literature. Pediatrics. 2006;117:e796-805. https://www.ncbi.nlm.nih.gov/pubmed/16567392

INTERNET
Stevens DL, Bryant A. Clostridial Myonecrosis. UpToDate, Inc. Aug 24, 2023. Available at: https://www.uptodate.com/contents/clostridial-myonecrosis Accessed Sept 17, 2024.

Tidy C. Patient website. Gas Gangrene. May 9, 2023. Available at: https://patient.info/doctor/gas-gangrene Accessed Sept 17, 2024.

Mayo Clinic for Medical Education and Research. Hyperbaric Oxygen Therapy. Dec 2, 2023. Available at: https://www.mayoclinic.org/tests-procedures/hyperbaric-oxygen-therapy/about/pac-20394380 Accessed Sept 17, 2024.

Buboltz JB, Murphy-Lavoie HM. Gas Gangrene. [Updated 2023 Jan 30]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537030/ Accessed Sept 17, 2024.

Qureshi S. Clostridial Gas Gangrene. Medscape Reference.  February 01, 2023. https://emedicine.medscape.com/article/214992-overview Accessed Sept 17, 2024.

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Infectious Diseases Society of America

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GARD Disease Summary

The Genetic and Rare Diseases Information Center (GARD) has information and resources for patients, caregivers, and families that may be helpful before and after diagnosis of this condition. GARD is a program of the National Center for Advancing Translational Sciences (NCATS), part of the National Institutes of Health (NIH).

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