What is Necrotizing Fasciitis (NF)??

Necrotizing fasciitis (NF), commonly known as flesh-eating disease or Flesh-eating bacteria syndrome, is a rare infection of the deeper layers of skin and subcutaneous tissues, easily spreading across the fascial plane within the subcutaneous tissue. Necrotizing fasciitis is a quickly progressing and severe disease of sudden onset and is usually treated immediately with high doses of intravenous antibiotics. Necrotizing fasciitis is a term that describes a disease condition of rapidly spreading infection, usually located in fascial planes of connective tissue that results in tissue necrosis (dead and damaged tissue). The disease occurs infrequently, but it can occur in almost any area of the body. Although many cases have been caused by group A beta-hemolytic streptococci (Streptococcus pyogenes), most investigators now agree that many different bacterial genera and species, either alone or together (polymicrobial) can cause this disease. Occasionally, mycotic (fungal) species cause necrotizing fasciitis.

This condition was described by several people in the 1840s to 1870s, and Dr. B. Wilson in 1952 first termed the condition necrotizing fasciitis. It is likely that the disease has been occurring for many centuries before it was first described in the 1800s. Currently, there are many names that have been used loosely to mean the same disease as necrotizing fasciitis: flesh-eating bacterial infection or disease; suppurative fasciitis; dermal, Meleney, hospital, or Fournier's gangrene; and necrotizing cellulitis. Body regions frequently have the term "necrotizing" placed before them to describe the initial localization of necrotizing fasciitis (for example, necrotizing colitis, necrotizing arteriolitis), but they all refer to the same disease process in the tissue. Important in understanding necrotizing fasciitis is the fact that whatever the infecting organism(s), once it reaches and grows in connective tissue, the spread of the infection can be so fast (investigators suggest some organisms can progress about 3 centimeters per hour) that the infection becomes difficult to stop with both antimicrobial drugs and surgery.

Mortality (death) rates have been reported as high as 75% for necrotizing fasciitis associated with Fournier's (testicular) gangrene. Patients with necrotizing fasciitis have an ongoing medical emergency that often leads to death or disability if it is not promptly and effectively treated.

Background
Flesh-eating disease is the common name for necrotizing fasciitis (nek-roe-tie-zing fah-sheeeye-tis), an infection that works its way rapidly through the layers of tissue (the fascia) that surround muscles. It destroys tissue and can cause death within 12 to 24 hours. It is estimated that there are between 90 and 200 cases per year in Canada, and about 20 to 30 percent of these are fatal.

The symptoms of flesh-eating disease include a high fever, and a red, severely painful swelling that feels hot and spreads rapidly. The skin may become purplish and then die. There may be extensive tissue destruction. Sometimes the swelling starts at the site of a minor injury, such as a small cut or bruise, but in other cases there is no obvious source of infection.

Flesh-eating disease can be caused by a number of different bacteria, including group A streptococcus (GAS). GAS is a very common bacteria. Many people carry it in the throat or on their skin without getting sick. It is the same bacteria that causes strep throat, and can also cause impetigo, scarlet fever and rheumatic fever. In rare instances, GAS will cause serious illnesses, including pneumonia, meningitis, blood poisoning (bacteremia), streptococcal toxic-shock syndrome and flesh-eating disease.

Few people who come into contact with GAS will develop a serious disease. The bacteria are normally spread through close personal contact, such as kissing or sharing cutlery with someone who is infected. People who are ill, such as those with strep throat or skin infections, are most likely to spread the bacteria. People who carry the bacteria, but have no symptoms, are much less contagious.

Do different types of necrotizing fasciitis exist?

Variations of necrotizing fasciitis are placed by some investigators into three general groups, roughly based on the genera of organisms causing the infection and some clinical findings that unfortunately vary from patient to patient. Type 1 is either caused by more than two bacterial genera (polymicrobial) or by the infrequently found single bacterial genus such as Vibrio or fungal genera such as Candida. Type 2 is caused by Streptococcus spp, and type 3 (or termed type 3 gas gangrene) is caused by Clostridium spp. One example of clinical findings (gas in tissues) is more often found in type 3, but can be found in types 1 and 2 also. Many investigators elect not to use this typing system and simply identify the organism(s) causing the necrotizing fasciitis.

What causes necrotizing fasciitis?

ost cases of necrotizing fasciitis are caused by bacteria; only rarely do other organisms such as fungi cause this disease. Group A Streptococci and Staphylococci, either alone or with other bacteria, cause many cases of necrotizing fasciitis, although Clostridium spp should be considered as a cause especially if gas is found in the infected tissue. Because of better microbial isolation techniques for anaerobic bacteria, bacterial genera such as Bacteroides, Peptostreptococcus, and Clostridium are often cultured from the infected area. Frequently, culture of tissue involved by necrotizing fasciitis also yields a mixture of other non-anaerobic bacterial genera such as E. coli, Klebsiella, Pseudomonas, and others. Many investigators

conclude that non-anaerobic organisms damage tissue areas enough to cause local areas of hypoxia (reduced oxygen) where anaerobic organisms then can thrive and extend the infection further. This results in polymicrobial infection in which one type of bacteria aids the survival and growth of another type of bacteria (synergy). Infrequently, Vibrio vulnificus causes the disease when a person, usually someone with liver function problems (for example, alcoholics or immunosuppressed patients), eats contaminated seafood or a wound gets contaminated with seawater containing Vibrio vulnificus.

In general, the bacteria that cause necrotizing fasciitis utilize similar methods to cause and advance the disease. Most produce toxins that inhibit the immune response, damage or kill tissue, produce tissue hypoxia, specifically dissolve connective tissue, or do all of the above. In polymicrobic infections, one bacterial genus may produce one toxic factor (for example, E. coli causing tissue hypoxia) while different types of coinfecting bacteria may produce other toxins that lyse (disintegrate) damaged tissue cells or connective tissue.

Prevention
Always clean the skin thoroughly after a cut, scrape, or other skin injury. ecrotizing fasciitis does not begin unless an infection has already started in tissue; immediate effective treatment of any infection is likely to prevent the disease. Further, anything that can help prevent infections will help prevent necrotizing fasciitis. Practices such as hand washing, checking extremities for cuts or wounds if you have diabetes, avoiding physical contact with people who carry MRSA, and good hygiene practices help prevent initial infections that may lead to necrotizing fasciitis. Immunosuppressed patients should be very careful not to get infections, and people with liver disease should avoid eating seafood that may be contaminated with Vibrio vulnificus. People with liver disease should not have any infections or cuts in the skin exposed to warm seawater to avoid necrotizing fasciitis caused by Vibrio vulnificus.

Physicians, surgeons, and other caregivers play an important role in prevention. Cases of necrotizing fasciitis may occur when surgical sites become infected. Consequently, physicians need to use sterile techniques when doing surgery and to adhere to hospital practices such as glove and gown coverage to help prevent infection spread in hospitalized patients. Careful surgical techniques in sites that can easily become contaminated are required. Some examples of such sites are bowel surgery, episiotomy (surgically enlarging the vaginal outlet), and debridement with closure of traumatic wounds.

Necrotizing fasciitis is not usually contagious. However, it is possible for uninfected people to physically come into contact with some patients with the disease and become infected with an organism that may eventually cause necrotizing fasciitis. For example, a person could

come in contact with a lesion containing MRSA organisms causing or contributing to the disease in another person and then become infected with MRSA.

What are the symptoms of necrotizing fasciitis?

The majority of cases begin with an existing infection, most frequently on an extremity or in a wound. The initial infection can be from almost any cause (for example, cuts on the skin, puncture wounds, surgical incisions, or insect bites). Instead of healing, the infected site can show erythema (redness) and swelling. The site may be very sensitive to pain, even past the area of erythema. At the same time, patients often experience fever and chills. Early symptoms resemble those of cellulitis, but progressive skin changes such as skin ulceration, bullae (thin-walled fluid-filled blisters) formation, necrotic eschars (black scabs), gas formation in the tissues, and fluid draining from the site can occur rapidly as the infection progresses. Some patients can become septic (meaning the infection has spread to the bloodstream and throughout the body) before the skin changes are recognized, especially when necrotizing fasciitis begins in deep facial planes. Type 1 often occurs after trauma or surgery and may form little or undetectable amounts of gas. Type 2 usually occurs after more simple skin trauma (cuts, abrasions, and insect bites) and infects more superficial facial planes with almost no gas formation. Type 3 usually occurs after trauma or after wounds become contaminated with dirt that contains Clostridium spp, which produce gas in tissues (gangrene) and necrotic eschars. However, symptoms for types 1-3 are not definitive, and symptoms vary widely which is why some investigators prefer to define the individual patients' disease by the organism(s) isolated from the patient rather than assigning a type label.

One set of patients that is being recognized with a more specific set of symptoms and health history are those infected with Vibrio vulnificus. The organisms occur in the warmer waters in the U.S. (Gulf of Mexico and southern coastal states) and elsewhere in the world with similar water conditions. Either ingesting the organisms or getting Vibrio vulnificus from contaminated seawater into skin abrasions or cuts can cause necrotizing fasciitis. The majority of those affected are either immunosuppressed or have chronic liver problems (for example, alcoholic liver disease, hepatitis, or cirrhosis). Bullae formation and rapid progression of the disease (within hours) on the extremities are hallmarks of Vibrio vulnificus wound infections. Even though this infection is caused by a single type of bacteria, some investigators classify it as a variant of type 1 necrotizing fasciitis.

Necrotizing enterocolitis (also termed NEC; necrosis of gastrointestinal tissue) occurs mainly in premature or sick infants and may be another variant of necrotizing fasciitis. Although investigators suggest that bacterial infection causes this disease, there is no definitive data to prove this and others attribute the disease to a permeable gut tissue. A few individuals think the necrotizing enterocolitis is due to a "leaky gut syndrome," but this concept is not widely accepted by the medical community.

Treatment
Patients are typically taken to surgery based on a high index of suspicion, determined by the patient's signs and symptoms. In necrotizing fasciitis, aggressive surgical debridement (removal of infected tissue) is always necessary to keep it from spreading and is the only treatment available. Diagnosis is confirmed by visual examination of the tissues and by tissue samples sent for microscopic evaluation. Early medical treatment is often presumptive; thus, antibiotics should be started as soon as this condition is suspected. Initial treatment often includes a combination of intravenous antibiotics including penicillin, vancomycin, and clindamycin. Cultures are taken to determine appropriate antibiotic coverage, and antibiotics may be changed when culture results are obtained. As in other maladies characterized by massive wounds or tissue destruction, hyperbaric oxygen treatment can be a valuable adjunctive therapy but is not widely available. Amputation of the affected organ(s) may be necessary. Repeat explorations usually need to be done to remove additional necrotic tissue. Typically, this leaves a large open wound, which often requires skin grafting. The associated systemic inflammatory response is usually profound, and most patients will require monitoring in an intensive care unit. Treatment for necrotizing fasciitis may involve an interdisciplinary care team. For example, in the case of a necrotizing fasciitis involving the head and neck, the team could include otolaryngologists, intensivists, microbiologists and plastic surgeons.