What is Volkmann Contracture?
Volkmann contracture, also known as Volkmann ischemic contracture, is a medical condition that causes a claw-like shape in your hand. It was named after a German doctor from the 19th century, Richard von Volkmann. “Ischemic” indicates that it happens because of inadequate blood supply, while “contracture” tells us that it involves a shortening of muscle tissue. This condition results in a permanent bend in the wrist and fingers making the hand look claw-like. This shape is due to the permanent tightness of certain muscles in your forearm, which may cause discomfort when you stretch your wrist and fingers.
Your upper limb, or arm, is made up of three sections: the upper arm, the forearm, and the hand. The significant bone in your upper arm, the humerus, connects to two bones in your forearm (the ulna and radius) at one end, and to a bone in the shoulder (the scapula) at the other. Your forearm extends from your elbow to your wrist and has two groups of muscles, the flexor group and the extensor group, which help you move your wrist, fingers, and hand joints. These muscles get their blood supply through the brachial artery, which runs down your upper arm, goes through your elbow, and then splits into two ending arteries, the radial and ulnar arteries. Of these two ending arteries, the ulnar artery supplies blood to the flexor group of muscles.
Within your forearm, the group of flexor muscles, which help you fold your hand and fingers, is divided into a superficial (closer to the skin) group and a deep group. The superficial group is made of five kinds of muscles:
1. Pronator teres
2. Flexor carpi radialis
3. Flexor digitorum superficialis
4. Palmaris longus
5. Flexor carpi ulnaris
The deep group includes:
1. Flexor pollicis longus
2. Pronator quadratus
3. Flexor digitorum profundus
All these muscles are controlled by a nerve called the median nerve. The exceptions are the muscles called the flexor carpi ulnaris (which is regulated by the ulnar nerve) and the flexor digitorum profundus (which gets signals from both the ulnar and median nerves). Both these groups of muscles can be affected by Volkmann ischemic contracture.
What Causes Volkmann Contracture?
Volkmann’s ischemic contracture, a condition that makes your hand curl into a fist that you can’t straighten, is usually related to a specific type of upper arm fracture known as a supracondylar fracture. Though, it can happen with any broken bone in the arm or elbow. The issue stems from a problem called compartment syndrome, which occurs due to loss of blood flow to the forearm.
Compartment syndrome happens when pressure increases within a closed space in your body. Two main things can trigger this – either the space becomes smaller or the contents inside the area increase in size. This condition can potentially lead to Volkmann’s ischemic contracture.
There are a number of things which can cause this increased pressure, such as:
1. Tight bandages and wraps
2. Animal bites
3. Burns
4. Overzealous or intensive exercise
Excessive muscle growth, tumors, bleeding into a closed space due to blood vessel injury or even a disease you were born with, or obtaining injections in the forearm can also cause this increased pressure. Surgery on the forearm too can possibly lead to compartment syndrome.
Moreover, Volkmann’s ischemic contracture might be a result of sudden lack of blood supply to the arms. Something like blood clots getting stuck in the arteries can cause this lack of blood flow, leading to muscle damage and eventually causing the limb to permanently contract.
Risk Factors and Frequency for Volkmann Contracture
Volkmann ischemic contracture is a rare condition often showing up after an injury to a child’s upper arm. One research found that it only makes up 0.105% of all orthopedic conditions, and it’s usually seen in young men in their twenties or thirties. In another study looking at children with severe arm fractures over a 13 year span, they found that out of 33 patients with a specific type of fracture called a supracondylar fracture, 3 developed Volkmann ischemic contracture. More importantly, if the break in the upper arm bone was displaced, there was a 33% chance of getting Volkmann contracture. That’s why, it’s crucial to watch out for warning signs of compartment syndrome—an urgent and painful condition that can lead to Volkmann contracture—in patients with certain types of upper arm bone fractures.
Signs and Symptoms of Volkmann Contracture
Volkmann’s ischemic contracture is a condition typically associated with a particular injury known as a supracondylar fracture of the humerus. Before the development of a contracture (an abnormal shortening of muscle tissue) and fibrosis (hardening or formation of excess tissue), there are signs and symptoms indicating the onset of compartment syndrome, a serious condition that involves increased pressure in a muscle compartment that can lead to muscle and nerve damage.
The initial symptom of compartment syndrome is pain, which later expands to include symptoms commonly known as the 5 Ps of compartment syndrome:
- Pain
- Pallor (a pale color of skin)
- Pulselessness (lack of a detectable pulse)
- Paresthesias (sensations of pins and needles or numbness)
- Paralysis (loss of muscle function)
Additional signs of compartment syndrome include increased pain during passive extension of the hands and fingers on the affected limb, firmness of the tissues in the forearm, and abnormal pulse volume and character when palpating the radial artery. Therefore, a detailed medical history and physical examination are crucial in identifying and preventing the development of Volkmann contracture.
Volkmann contracture can be categorized into three grades according to the Tsuge system: mild, moderate, and severe.
- Mild: This type of contracture is localized, affecting parts of the flexor digitorum profundus muscle and causing the flexion of 2 or 3 fingers, typically the middle and ring fingers. Sensory loss is minimal or not present.
- Moderate: This is a classic type of Volkmann contracture where both the flexor digitorum profundus and the flexor pollicis longus muscles are primarily involved. The superficial muscles may also be affected. This results in all five digits and the wrist flexing, leading to the appearance of a “claw hand”. Sensory impairment in parts of the hand supplied by the median and the ulnar nerves is common.
- Severe: This severe form of contracture is seen in extreme cases and involves both flexors and extensors, resulting in severe contractures with pronounced sensory deficits. Long-lasting moderate Volkmann contractures may eventually develop into this severe form.
Testing for Volkmann Contracture
Volkmann contracture is a condition that’s typically easy to identify based on the common symptoms and a physical examination. For most people, medical tests like lab investigations are not necessary to confirm it. However, if additional insights are needed, doctors might check for another condition called acute compartment syndrome.
This involves various tests, including measures of creatine phosphokinase (a chemical involved in energy production), blood urea nitrogen and creatinine (which can give insights on kidney function), serum electrolytes and calcium (important for proper body functioning), and urine analysis including a specific protein called myoglobin. If there’s suspicion of a blood clotting problem contributing to compartment syndrome, clotting tests might be undertaken. A complete blood check can also help to rule out anemia which potentially can make compartment syndrome worse by reducing oxygen supply in the affected compartment.
One definitive way of diagnosing compartment syndrome is measuring the pressure within the affected compartment of the body (osteofascial compartment). This is typically done when a clear diagnosis can’t be made based on symptoms and examination alone, or to confirm a diagnosis. Care should be taken not to rule out compartment syndrome based on single normal pressure reading as conditions can change. There are various ways to measure this pressure. One common tool used for this is the Stryker tonometer. If it shows a compartmental pressure above 30 to 40 mmHg, it typically means compartment syndrome is present and immediate surgery is needed. This specific type of surgery is called a fasciotomy. Any delays in surgery could worsen the patient condition significantly.
A tool called Near-Infrared Spectroscopy (NIRS) can also help to assess and manage acute compartment syndrome. However, its usage is limited due to high cost and limited sensor availability, despite being a reliable non-invasive way of investigating Volkmann ischemic contracture.
Regarding imaging studies such as X-rays, MRIs, and CT scans, these can be helpful in supporting the diagnosis of Volkmann contracture, but they are not usually the first choice for investigations. X-rays, in particular, could be very useful for identifying displaced fractures in the upper arm bone. CT and MRI scans can also help to rule out other potential causes of compartment syndrome that are unrelated to fractures of the upper limb. An ultrasound can also be used as an additional tool to help rule out acute compartment syndrome.
Treatment Options for Volkmann Contracture
If a doctor suspects that you may have compartment syndrome or early Volkmann contracture, they will likely first remove all external bandages, splints, or casts from your affected limb. This simple step is crucial because it can halt the progression of the condition if it’s caused by an extrinsic compartment syndrome (pressure build-up due to external factors, like a too-tight cast). Research shows that casts can hinder the expansion of the compartment in your limb by up to 40% and should always be removed, a step that can reduce the increased pressure by about 40 to 60%.
Taking off these enclosures also makes it easier for doctors to conduct necessary investigations and physical examinations. Raising the limb can decrease swelling, but it shouldn’t be elevated beyond the level of the heart, as this can reduce blood flow to the limb and increase the risk of tissue death due to lack of blood supply. Other measures that can help slow down the progression of acute compartment syndrome include allowing high blood pressure and correcting anemia. Painkillers may be prescribed depending on the level of pain, but it’s important not to become relaxed because the pain has eased, as the underlying problem may still exist.
If compartment syndrome has led to Volkmann contracture, an emergency fasciotomy (surgical procedure to relieve pressure) may be needed. This is usually the case when the pressure inside the compartment rises above 30 to 40 mmHg. The surgery can be performed from either the front (volar) or rear (dorsal) aspect of the limb, both of which can relieve the compartment’s pressure. The median nerve, which runs down your forearm and into your hand, may need to be relieved, especially in certain high-risk areas.
Doctors also need to watch out for a condition called myoglobinuria, which can occur due to prolonged lack of blood supply (ischemia). It can cause severe body-wide effects and lead to multiple organ failure. If this occurs, you would need to have proper hydration, continuous monitoring of your arterial blood gases, renal (kidney) function tests, and management of any electrolyte imbalances (disruptions in body chemicals that regulate bodily functions).
If Volkmann contracture has already developed, the treatment depends on how severe it is. Mild cases may be treated with physical therapy, dynamic splinting, tendon lengthening, and slide procedures, which usually have a relatively good outcome. Moderate contractures (limb deformities) might require tendon-slide procedures, neurolysis (nerve release), and extensor transfer (moving a muscle to replace a damaged one) operations. For severe contractures that affect both flexor and extensor groups (groups of muscles that move parts of a joint), extensive surgical removal of scar tissue and tendon transfer procedures may be needed to restore movement to the fingers. A muscle transfer may also be performed if the muscle tissue is necrotic (dead). A dead muscle is identified by its color, contractility (ability to contract), firmness, and capacity to bleed.
What else can Volkmann Contracture be?
When a medical professional is trying to diagnose Volkmann’s contracture, they might also consider other conditions that look similar. These include:
- Dupuytren’s contracture
- Pseudo Volkmann’s contracture
Pseudo Volkmann’s contracture relates to a condition where a person can’t fully straighten their fingers. This is often due to a mechanical obstacle in patients who have had a forearm fracture. It’s worth noting that in these cases, there are no signs of inadequate blood supply (ischemia) to the muscles. This is quite a rare situation, more often seen in children, due to an obstruction by a broken bone in the forearm.
On the other hand, Dupuytren’s contracture is a condition that gradually causes one or more fingers to curl into the palm. The exact cause is unknown, but risk factors include chronic liver disease, alcoholism, smoking, and previous injury. It starts off as hard lumps in the palm and over time, restricts the movement of the fingers.
In some instances, the term ‘Volkmann’s contracture’ is used to describe not just conditions affecting the hand. However, in most cases, and for the purposes of this discussion, we are only referring to the condition characterized by muscle damage in the forearm causing a stiff, claw-like hand.
What to expect with Volkmann Contracture
The results of a procedure known as fasciotomy, used to treat a severe condition called acute compartment syndrome which can lead to another condition known as Volkmann contracture, often depends on how long and how severe the compartment syndrome was. If this procedure is done quickly—usually within 4 hours—it typically leads to minor long-term effects.
However, the outcomes for individuals with Volkmann contracture can vary greatly from person to person. It’s worth noting that in most cases, children who develop this contracture often end up with a disproportionately short limb.
An operation called Functioning Free Muscle Transfer (FFMT) has been found to significantly improve hand function. On the other hand, another operation known as tendon lengthening often causes return of contractures in many cases. There are other procedures—tenolysis, neurolysis, tendon transfer, and the removal of nonhealthy (necrotic) muscle—which typically lead to good hand function for individuals who still have enough muscle remaining.
Possible Complications When Diagnosed with Volkmann Contracture
The treatment for Volkmann contracture can result in different complications, depending on the specific procedure. For example, with fasciotomy wounds, some of the issues can be changes in how things feel to the touch (77% of cases), skin that becomes dry and flaky (40% of cases), itching (33% of cases), and discoloration of the wounds (30% of cases). Unfortunately, the look of the wounds can distress patients, with almost a quarter of them changing their behavior to hide the scars.
Tendon transfer surgery also has its potential problems. Both over-tightening and under-tightening can happen, even when performed by expert hands, because the knots that are used may loosen or slip over time. Hematomas (accumulated blood due to internal bleeding) and wound separation might worry patients after flexor origin slide surgery. Another procedure, functional free muscle transfer surgery, also has potential downsides, including the risk of loss of the flap, infection, scarring and attachment of tendons to surrounding tissue.
- Altered sensations
- Dry and flaky skin
- Itching
- Discolored wounds
- Altered behaviour due to distress
- Over-tightening or under-tightening during tendon transfer surgery
- Hematomas and wound separation after flexor origin slide surgery
- Flap loss, infection, scarring, and tendon adhesion following functional free muscle transfer surgery
Recovery from Volkmann Contracture
When treating acute compartment syndrome, cuts made during surgery are always left open. If the major blood vessels and nerves are not exposed, a special kind of dressing that uses negative pressure can be applied to the wound. This dressing should be changed in a clean and sterile manner every 24 to 48 hours to protect the wound from getting infected. About one to two weeks after the surgery, the wound can be closed once the swelling has significantly gone down.
The process of recovery from Volkmann ischemic contracture, a condition which causes muscle shortening and can lead to hand deformity, depends on the specific surgery. For about 2 to 4 weeks after surgery, the affected limb is usually immobilized in a cast or splint. After this period, patients can gradually start using the limb again, slowly increasing their activity level over time.
Preventing Volkmann Contracture
Educating patients the right way can help in early recognition and treatment of conditions like Volkmann’s ischemic contracture and acute compartment syndrome, which could develop beforehand. It’s crucial for patients to understand in simple terms the signs and symptoms of acute compartment syndrome. If left untreated, this condition can have severe long-term effects. They should be told about how important it is to get treated for these conditions early on and the benefits that can come from it.
Patients who have suffered from specific injuries, such as displaced supracondylar fractures, need to be made aware of the high chance of developing Volkmann contracture. They should know not to ignore or delay medical attention if they start to see symptoms of compartment syndrome.
Patients who have to use casts, splints, or bandages also need to be clear about the potential problems that might happen if these are applied too tightly. These issues include increased pain, decreased blood flow, and potentially even acute compartment syndrome, if the pressure inside the muscles becomes too high from the tight bandages or casts. By adequately informing patients about these risks, they can better participate in their own care and seek help if they notice any concerning symptoms.