Early diagnosis saves life in flesh-eating disease

Coconino National Forest in Arizona. (Dr. Noorali Bharwani)
Coconino National Forest in Arizona. (Dr. Noorali Bharwani)

Recently, an Edmonton lady was diagnosed with flesh-eating disease while on a holiday in Mexico. Media reports say the lady underwent three surgeries to remove the dead tissues secondary to the infection. The good news is she survived and is now back in Canada.

Medically speaking, this condition is known as necrotizing fasciitis. It is not a new disease. It is often called flesh-eating disease because it kills muscle and skin as it spreads through the tissue.

In Canada, in 1954, the famous case of flesh-eating disease was Loucien Bouchard, the former premier of Quebec. He luckily survived and had to have one leg amputated.

It is an uncommon, rapidly progressive and life-threatening infection that requires urgent diagnosis and medical and surgical treatment. Fortunately, the disease is not that common. In Canada, it is estimated 90 to 200 cases of necrotizing fasciitis occur each year. And, unfortunately, approximately 20 to 30 per cent of these cases lead to death. From time to time the disease hits the headlines. Then there is a long silence before another case is reported. This leaves people wondering what is this all about.

The term flesh-eating refers to the toxins produced by a bacterial infection that can destroy muscles, skin and fatty tissues. The infection spreads rapidly. It progressively destroys fat, fascia and muscles. Shock and multi-organ failure are common.

The flesh-eating infections have been described as early as the fifth century B.C. during the time of Hippocrates. More than 2,000 cases of this condition were reported among soldiers during the Civil War.

In 1871, the same disease was recorded by Dr. Joseph Jones, who was an army surgeon for the Confederate Army (in the American Civil War). He called it ‘hospital gangrene’, and 46 per cent of his patients who suffered the condition died.

Who is susceptible to the infection?

Certain illnesses can make people prone to this infection: diabetes mellitus, intravenous drug users and those who are immunocompromised. However, these infections can arise in people who are healthy.

What kind of organisms are involved?

Streptococci (invasive group A hemolytic streptococci) may act alone or in combination with other bacteria.

The most common sites of infection are perineal and groin areas and post-surgical wounds. Infection around the umbilicus in a new born can be life-threatening. Infection can occur in the trunk and the limbs.

What are the signs and symptoms?

Pain, warmth, skin redness, or swelling at a wound, especially if the redness is spreading rapidly. Skin blisters, sometimes with a “crackling” sensation under the skin. Pain from a skin wound that also has signs of a more severe infection, such as chills and fever. Grayish, smelly liquid draining from the wound would suggest the diagnosis.

Diagnosis

For diagnosis, it is important to be clinically alert to the condition. Early expert advice when necrotizing fasciitis is suspected should be considered. But early diagnosis of this condition is not always easy. If the diagnosis is in doubt, then surgical exploration is the best way to go.

Here are four key points from the Canadian Medical Association Journal (CMAJ May 23, 2017):

  1. Necrotizing fasciitis can occur in otherwise healthy individuals.
  2. Treatment involves emergency debridement in the operating theatre, broad spectrum antimicrobial drugs, local wound care and eventual free flap reconstruction.
  3. If the diagnosis is not clear, it is important to reassess the patient frequently and seek early expert advice in suspected cases.
  4. Imaging examination is ancillary and should never delay surgical treatment of necrotizing fasciitis.

(Ref: Necrotizing fasciitis after scalpel injury sustained during postmortem examination. Michal Brichacek, Robert Strazar, Kenneth A Murray and Avinash Islur CMAJ May 23, 2017 189 (20))

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Early Diagnosis Saves Life in Flesh-Eating Disease

Dear Dr. B: My son has developed serious infection in his leg. How do I know if this is flesh-eating disease?

The most famous case of flesh-eating disease (FED) in Canada is Loucien Bouchard, the former premier of Quebec. He luckily survived and had to have one leg amputated.

FED is a very serious kind of infection which spreads rapidly in the body. It is deep seated under the skin and progressively destroys fat, fascia and muscles. The condition is also known as necrotizing fasciitis.

FED was first described by Hippocrates around 500 B.C. The condition is not that common. In Canada, it is estimated that 90 to 200 cases of FED occur each year. In the US, approximately 1000 cases are seen in a year. Since the condition is not common, a physician would probably see one or two cases in his career.

The death rate in FED has not changed in the last 30 years and remains around 25 to 35 per cent. Death rate is directly related to early diagnosis and surgical intervention.

The most common sites of infection are perineal and groin areas and post surgical wounds. Infection around the umbilicus in a new born can be life-threatening. Among children the FED can be a serious complication of varicella infection. Infection can occur in the trunk and the limbs. Patient who are diabetic, intravenous drug abusers, immuno-compromised or have peripheral vascular disease are also prone to FED.

How to diagnose FED?

The diagnosis is clinical. It is not always easy to make a diagnosis. But the condition should be kept in mind in any kind of skin infection.

Initially, it is hard to differentiate from ordinary infection of the skin. But gradually patients get very sick. The pain is more severe than the clinical findings. There are only minor changes in the skin in early phases.

The factors that help distinguish FED from ordinary skin infection include a generalized rash, toxic appearance, fever and low platelet count. Plain x-ray can reveal gas under the skin or soft-tissue swelling but cannot show deeper gas under the fascia. CAT scan is more sensitive because it can show inflammatory changes like fascial swelling, thickening, abscesses and gas formation. MRI can add more information but ultrasound has poor sensitivity and specificity in this condition.

“The main diagnostic tool, however, is surgical exploration”, says an editorial in the British Medical Journal (BMJ). The characteristic finding at surgery is of grey, edematous fat, which strips off the underlying fascia with a sweep of the finger. Deeper changes are invariably more widespread than the skin changes. If FED is suspected then surgical exploration should be undertaken and can be life saving.

Intravenous antibiotic therapy has an important role in reducing generalized infection and spread of bacteria in the body. The BMJ editorial says that no evidence exists that antibiotics halt the infection in FED and their use may tempt the surgeon to perform less mutilating and less effective surgery. Nevertheless, broad spectrum antibiotic cover is routine and should specifically target anaerobes and streptococci.

Hyperbaric oxygen is strongly advocated by some. But there are no controlled studies to prove its usefulness. Using it would seem reasonable if it was readily available but not if the need for inter-hospital transfer delayed definitive surgery, says the editorial.

In summary, the diagnosis of FED is mainly clinical. Surgery is the mainstay of management. Patient should be taken to the operating room as soon as flesh-eating disease is suspected. Massive removal of dead and dying tissue is undertaken. Sometimes amputation becomes necessary. Patient may require surgery more than once.

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A Dirty Wound Can Be Dangerous

During my recent family holiday, we took a day trip to a very small island of Barbuda. Barbuda is a flat coral island with an area of only 68 square miles. The island’s population of 1500 live in the village of Codrington. It is renowned for its beaches which are natural, sprinkled with pink sand, and miles long.

We were with a group of other tourists including a family doctor from England who stayed at the same hotel as we did and knew I was a surgeon. So, here we are, climbing rocks and exploring a cave. I turned around to take a picture, tripped and fell on a rock. My right leg sustained a deep nasty dirty wound with blood gushing all over the place and a piece of my precious skin and flesh lying on the bloody ground.

There was momentary panic amongst my fellow tourists when the English doctor announced with confidence, “Don’t worry, he is a surgeon!” That lightened the situation with several people offering their shirts to use as a bandage to stop the profuse bleeding.

In my mind I knew this was a serious situation. I immediately thought of two very life threatening outcomes from this episode, loss of limb or life from tetanus or flesh-eating disease. Before my trip, I had a booster dose of tetanus toxoid vaccine. But what about flesh-eating disease?

Flesh-eating disease is a form of streptococcal infection that leads to death of tissues. It is a deep-seated infection under the skin. It progressively destroys fat, fascia and muscles. The condition is also known as necrotizing fasciitis.

Streptococci (invasive group A hemolytic streptococci) may act alone or in combination with other bacteria. The infection spreads rapidly. Shock and multi-organ failure are common. Mortality is high, up to 58 percent.

Fortunately, the disease is not that common. In Canada, it is estimated that 90 to 200 cases of necrotizing fasciitis occur each year. From time to time the disease hits the headlines. Then there is long silence before another case is reported. This leaves people wondering what is this all about.

The most famous case of flesh-eating disease was Loucien Bouchard, the former premier of Quebec. He luckily survived and had to have one leg amputated.

It is not a new disease. The condition was first described in a specific body region by Fournier in 1883 and as a more generalized condition by Meleney in 1924.

Early diagnosis of this condition is not always easy. The diagnosis is clinical. Initially, it is hard to differentiate from ordinary infection of the skin. But gradually patients get very sick. The pain is more severe than the clinical findings. There are only minor changes in the skin in early phases. Unfortunately, the current state of knowledge on diagnoses and management is limited.

If the diagnosis is in doubt then surgical exploration is the best way to go. The characteristic finding is of grey, edematous fat, which strips off the underlying fascia with a sweep of the finger. Deeper changes are invariably more widespread than the skin changes.

Tetanus and flesh-eating disease are two extreme examples of how small things can get out of hand if no action is taken right away. There are many other kinds of infections which can affect dirty wounds. Even clean wounds like surgical incisions can get infected. Although the incidence of that happening is very low compared to a dirty wound.

If you sustain a dirty wound, including stepping on a dirty nail, make sure the area is thoroughly cleaned, your tetanus vaccine is up-to-date, you receive appropriate antibiotics to prevent infection and carefully monitor the progress of the wound. Before you travel abroad, make sure all your vaccines are up-to-date. Freaky things happen when you least expect it.

As for my leg, it is still there. The healing process is slow but I think I will be alright.

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Flesh-eating Disease

Dear Dr. B: What is flesh-eating disease?

Flesh-eating disease is a form of streptococcal infection that leads to death of tissues. It is a deep-seated infection under the skin. It progressively destroys fat, fascia and muscles. The condition is also known as necrotizing fasciitis.

Streptococci (invasive group A hemolytic streptococci) may act alone or in combination with other bacteria. The infection spreads rapidly. Shock and multiorgan failure are common. Mortality is high – up to 58 percent.

Fortunately, the disease is not that common. In Canada, it is estimated that 90 to 200 cases of necrotizing fasciitis occur each year. From time to time the disease hits the headlines. Then there is long silence before another case is reported. This leaves people wondering what is this all about.

The most famous case of flesh-eating disease was Loucien Bouchard, the former premier of Quebec. He luckily survived and had to have one leg amputated.

An editorial in British Medical Journal (BMJ) says that the condition was first described in a specific body region by Fournier in 1883 and as a more generalized condition by Meleney in 1924.

The most common sites of infection are perianal and groin areas and post surgical wounds. Infection around the umbilicus in a new born can be life-threatening. Among children the necrotizing fasciitis can be a serious complication of varicella infection.

Is it possible to make an early diagnosis before things get worse?

Unfortunately, no! The diagnosis is clinical. Initially, it is hard to differentiate from ordinary infection of the skin. But gradually patients get very sick. The pain is more severe than the clinical findings. There are only minor changes in the skin in early phases.

An article in the Canadian Medical Association Journal says the factors that help distinguish necrotizing fasciitis from ordinary skin infection include a generalized rash, toxic appearance, fever and low platelet count. Unfortunately, the current state of knowledge on diagnoses and management is limited. More research is required.

“The main diagnostic tool, however, is surgical exploration”, says the BMJ editorial. The characteristic finding is of grey, edematous fat, which strips off the underlying fascia with a sweep of the finger. Deeper changes are invariably more widespread than the skin changes.

Patient requires immediate hospitalization for medical and surgical management. Three specific treatments have been suggested:
-antibiotics,
-hyperbaric oxygen, and
-surgery.

The BMJ editorial says that no evidence exists that antibiotics halt the infection in necrotizing fasciitis and their use may tempt the surgeon to perform less mutilating but less effective surgery. Nevertheless, broad spectrum cover is routine and should specifically target anaerobes and streptococci.

Hyperbaric oxygen is strongly advocated by some. But there are no controlled studies to prove its usefulness. Using it would seem reasonable if it was readily available but not if the need for interhospital transfer delayed definitive surgery, says the editorial.

Surgery is the mainstay of management. Patient should be taken to the operating room as soon as flesh-eating disease is suspected. Massive removal of dead and dying tissue is undertaken. Sometimes amputation becomes necessary. Patient may require surgery more than once.

Start reading the preview of my book A Doctor's Journey for free on Amazon. Available on Kindle for $2.99!