Our bodies are held together by structures called connective tissue. Connective tissues make up the ligaments and tendons that help your joints move. They also provide structure and flexibility for body parts, such as your skin and blood vessels.

Damage to your connective tissue can cause serious health issues. Normal wear and tear from aging can start to erode connective tissues. So can certain Conditions in which the body’s disease fighting system mistakenly attacks and destroys the body’s own cells. autoimmune diseases, like lupus, and specific connective tissue disorders. The effects of these conditions can range from mild to severe.

Researchers are looking for better ways to diagnose and treat conditions that lead to connective tissue damage. Getting treatment may help improve a person’s quality of life. But connective tissue disorders can be hard to recognize.

Connecting the Dots

Connective tissue disorders aren’t common. Several can be passed down genetically. Those tend to run in families. Each disorder affects different body parts.

One condition, known as Marfan syndrome, can lead to balloon-like bulges called aneurysms in blood vessels. These bulges are especially dangerous when they occur in the body’s largest blood vessel, the aorta, which carries blood to the heart. If it bursts, it can cause sudden death.

Another disorder, called hyper-mobile Ehlers-Danlos Syndrome (or hEDS), can cause pain in the joints and other parts of the body. The condition epidermolysis bullosa (EB) weakens the skin. And the disorder osteogenesis imperfecta can make bones prone to breaking.

Although the symptoms and affected body parts are different, these conditions all stem from damaged connective tissues. The severity of symptoms for these disorders varies widely among patients. They can range from hardly noticeable to life-threatening (see Wise Choices box).

“Milder forms of these conditions are probably under-recognized and underdiagnosed,” says Dr. Anna Bruckner, a skin expert at the University of Colorado.

The symptoms caused by connective tissue disorders can also often arise from many other health conditions. “And symptoms like chronic pain and chronic fatigue are often missed, or dismissed,” explains Dr. Cortney Gensemer, a genetics researcher at the Medical University of South Carolina (MUSC), who has hEDS herself.

Tell your doctor if you have a family history of a connective tissue disorder or if you have a relative who died suddenly at a young age. That information can help with a diagnosis, explains Dr. Jason Cook, a vascular surgeon at the University of Nebraska Medical Center.

Finding the Cause

Sometimes, having a connective tissue disorder in the family can delay diagnosis, Gensemer explains.

“People can get accustomed to thinking that something painful is normal,” she says. “You might say something to your mom and dad, and their answer might be ‘Oh, that happens to me all the time.’”

But you can’t always be sure what’s causing your symptoms. So it’s important to talk with your doctor about any symptoms you have. Your doctor will ask about your personal and family medical history. Some common tests that look for connective disorders include imaging tests or genetic testing.

Genetic testing on its own isn’t enough to rule out some conditions, including Marfan syndrome, Cook says. That’s because the test doesn’t detect all possible genetic factors that can lead to the condition.

Researchers are continuing to search for the causes of connective tissue disorders. Gensemer, working with Dr. Russell Norris from MUSC, has built a registry of families living with hEDS. They’re now carrying out a large study looking for genes that can cause the condition.

“Having a genetic test would provide a definitive, easy, objective diagnosis,” Gensemer says. “It would also let people get a diagnosis earlier.”

Living With Connective Tissue Disorders

Researchers hope that a better understanding of the causes of these disorders will eventually lead to a cure. Currently, treatments for connective tissue disorders focus on managing symptoms.

Both Marfan syndrome and hEDS can affect your blood vessels, which makes them vulnerable to tearing. People with these conditions often need frequent monitoring. Some may eventually need surgery to make sure a blood vessel doesn’t tear.

The same gene changes that affect the blood vessels in Marfan syndrome can also weaken the heart, Cook says. His team is studying how to repair blood vessels without putting extra stress on heart tissue.

Treatment for connective tissue disorders needs to be personalized, Cook explains, because the same condition can affect people very differently. Medications are available to help with some symptoms of these disorders, like high blood pressure and reduced bone strength.

Recently, the first two medications to help heal skin damage in people with EB were approved by the U.S. Food and Drug Administration. “So now having a diagnosis can open the door to treatment for these patients,” Bruckner says.

She and her team are working on ways to repair skin damage for people with severe EB. They’re taking Cells that have the ability develop into many different cell types in the body. stem cells from patients’ skin, fixing the broken gene, and then returning repaired cells to the body. They’re testing whether these cells can heal blisters that arise in people with EB and prevent new ones from forming.

“The skin is visible, so we can just look to see whether this works,” Bruckner explains. “If it does, then we have the potential to change the stem cells into other types of cells that could potentially heal the person’s other organs.”

“It’s potentially one treatment, and you’re done for the rest of your life,” adds Dr. Anthony Oro, a stem cell researcher at Stanford University.

Oro and others are now working on a large NIH-funded project to make it easier to get stem cell therapies manufactured, so the treatment can be used for more patients. They’re starting with skin repair for EB. But other research teams are already looking at how to repair other tissues like bone, muscle, and heart tissue.

“We hope this technology will be applicable to many different disorders,” Bruckner says.

Could You Have a Connective Tissue Disorder?

Common signs and symptoms:

Marfan Syndrome

• Arms, legs, fingers, and toes that are long compared to your body.
• A chest wall that caves in or sticks out.
• A curved spine.
• A long, narrow face.
• Heartbeats that skip, flutter, or feel too hard or fast.
• Vision problems, like blurry vision or extreme nearsightedness.

Hypermobile EDS

• Joints that stretch farther than normal (joint hypermobility).
• Joints that pop out of place (dislocations).
• Skin that is soft or velvety, highly elastic (stretchy), or bruises easily.
• Chronic pain.
• Feeling tired all the time (fatigue).

Epidermolysis Bullosa (EB)and Related Skin Conditions

• Skin that blisters easily.
• Blisters in the mouth.
• Rough, thickened, or missing fingernails or toenails.
• Changes in the color of the skin.

Achondrogenesis is a group of severe disorders that affect cartilage and bone development. These conditions are characterized by a small body, short limbs, and other skeletal abnormalities. As a result of serious health problems, infants with achondrogenesis usually die before birth, are stillborn, or die soon after birth from respiratory failure. However, some infants have lived for a short time with intensive medical support.

Researchers have described at least three forms of achondrogenesis, designated as type 1A, type 1B, and type 2. The types are distinguished by their signs and symptoms, inheritance pattern, and genetic cause. However, types 1A and 1B are often hard to tell apart without genetic testing.

Achondrogenesis type 1A, which is also called the Houston-Harris type, is the least well understood of the three forms. Affected infants have extremely short limbs, a narrow chest, short ribs that fracture easily, and a lack of normal bone formation (ossification) in the skull, spine, and pelvis.

Achondrogenesis type 1B, also known as the Parenti-Fraccaro type, is characterized by extremely short limbs, a narrow chest, and a prominent, rounded abdomen. The fingers and toes are short and the feet may turn inward and upward (clubfeet). Affected infants frequently have a soft out-pouching around the belly-button (an umbilical hernia) or near the groin (an inguinal hernia).

Infants with achondrogenesis type 2, which is sometimes called the Langer-Saldino type, have short arms and legs, a narrow chest with short ribs, and underdeveloped lungs. This condition is also associated with a lack of ossification in the spine and pelvis. Distinctive facial features include a prominent forehead, a small chin, and, in some cases, an opening in the roof of the mouth (a cleft palate). The abdomen is enlarged, and affected infants often have a condition called hydrops fetalis, in which excess fluid builds up in the body before birth.

Dupuytren contracture is characterized by a deformity of the hand in which the joints of one or more fingers cannot be fully straightened (extended); their mobility is limited to a range of bent (flexed) positions. The condition is a disorder of connective tissue, which supports the body's muscles, joints, organs, and skin and provides strength and flexibility to structures throughout the body. In particular, Dupuytren contracture results from shortening and thickening of connective tissues in the hand, including fat and bands of fibrous tissue called fascia; the skin is also involved.

In men, Dupuytren contracture most often occurs after age 50. In women, it tends to appear later and be less severe. However, Dupuytren contracture can occur at any time of life, including childhood. The disorder can make it more difficult or impossible for affected individuals to perform manual tasks such as preparing food, writing, or playing musical instruments.

Dupuytren contracture often first occurs in only one hand, affecting the right hand twice as often as the left. About 80 percent of affected individuals eventually develop features of the condition in both hands.

Dupuytren contracture typically first appears as one or more small hard nodules that can be seen and felt under the skin of the palm. In some affected individuals the nodules remain the only sign of the disorder, and occasionally even go away without treatment, but in most cases the condition gradually gets worse. Over months or years, tight bands of tissue called cords develop. These cords gradually draw the affected fingers downward so that they curl toward the palm. As the condition worsens, it becomes difficult or impossible to extend the affected fingers. The fourth (ring) finger is most often involved, followed by the fifth (little), third (middle), and second (index) fingers. Occasionally the thumb is involved. The condition is also known as Dupuytren disease, and "Dupuytren contracture" most accurately refers to later stages when finger mobility is affected; however, the term is also commonly used as a general name for the condition.

About one-quarter of people with Dupuytren contracture experience uncomfortable inflammation or sensations of tenderness, burning, or itching in the affected hand. They may also feel pressure or tension, especially when attempting to straighten affected joints.

People with Dupuytren contracture are at increased risk of developing other disorders in which similar connective tissue abnormalities affect other parts of the body. These include Garrod pads, which are nodules that develop on the knuckles; Ledderhose disease, also called plantar fibromatosis, which affects the feet; scar tissue in the shoulder that causes pain and stiffness (adhesive capsulitis or frozen shoulder); and, in males, Peyronie disease, which causes abnormal curvature of the penis.

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders caused by abnormalities in the structure, production, and/or processing of collagen. The new classification, from 2017, includes 13 subtypes of EDS. Although other forms of the condition may exist, they are extremely rare and are not well-characterized. The signs and symptoms of EDS vary by type and range from mildly loose joints to life-threatening complications. Features shared by many types include joint hypermobility and soft, velvety skin that is highly elastic (stretchy) and bruises easily. Mutations in a variety of genes may lead to EDS; however, the underlying genetic cause in some families is unknown. Depending on the subtype, EDS may be inherited in an autosomal dominant or an autosomal recessive manner. There is no specific cure for EDS. The treatment and management is focused on preventing serious complications and relieving associated signs and symptoms.

What is lupus?

Lupus is an autoimmune disease. This means that your immune system attacks healthy cells and tissues by mistake. This can damage many parts of the body, including the joints, skin, kidneys, heart, lungs, blood vessels, and brain.

There are several kinds of lupus

• Systemic lupus erythematosus (SLE) is the most common type. It can be mild or severe and can affect many parts of the body.
• Discoid lupus causes a red rash that doesn't go away
• Subacute cutaneous lupus causes sores after being out in the sun
• Drug-induced lupus is caused by certain medicines. It usually goes away when you stop taking the medicine.
• Neonatal lupus, which is rare, affects newborns. It is probably caused by certain antibodies from the mother.

What causes lupus?

The cause of lupus is unknown.

Who is at risk for lupus?

Anyone can get lupus, but women are most at risk. Lupus is two to three times more common in African American women than in Caucasian women. It's also more common in Hispanic, Asian, and Native American women. African American and Hispanic women are more likely to have severe forms of lupus.

What are the symptoms of lupus?

Lupus can have many symptoms, and they differ from person to person. Some of the more common ones are

• Pain or swelling in joints
• Muscle pain
• Fever with no known cause
• Red rashes, most often on the face (also called the "butterfly rash")
• Chest pain when taking a deep breath
• Hair loss
• Pale or purple fingers or toes
• Sensitivity to the sun
• Swelling in legs or around eyes
• Mouth ulcers
• Swollen glands
• Feeling very tired

Symptoms may come and go. When you are having symptoms, it is called a flare. Flares can range from mild to severe. New symptoms may appear at any time.

How is lupus diagnosed?

There is no specific test for lupus, and it's often mistaken for other diseases. So it may take months or years for a doctor to diagnose it. Your doctor may use many tools to make a diagnosis:

• Medical history
• Complete exam
• Blood tests
• Skin biopsy (looking at skin samples under a microscope)
• Kidney biopsy (looking at tissue from your kidney under a microscope)

What are the treatments for lupus?

There is no cure for lupus, but medicines and lifestyle changes can help control it.

People with lupus often need to see different doctors. You will have a primary care doctor and a rheumatologist (a doctor who specializes in the diseases of joints and muscles). Which other specialists you see depends on how lupus affects your body. For example, if lupus damages your heart or blood vessels, you would see a cardiologist.

Your primary care doctor should coordinate care between your different health care providers and treat other problems as they come up. Your doctor will develop a treatment plan to fit your needs. You and your doctor should review the plan often to be sure it is working. You should report new symptoms to your doctor right away so that your treatment plan can be changed if needed.

The goals of the treatment plan are to

• Prevent flares
• Treat flares when they occur
• Reduce organ damage and other problems

Treatments may include drugs to

• Reduce swelling and pain
• Prevent or reduce flares
• Help the immune system
• Reduce or prevent damage to joints
• Balance the hormones

Besides taking medicines for lupus, you may need to take medicines for problems that are related to lupus such as high cholesterol, high blood pressure, or infection.

Alternative treatments are those that are not part of standard treatment. At this time, no research shows that alternative medicine can treat lupus. Some alternative or complementary approaches may help you cope or reduce some of the stress associated with living with a chronic illness. You should talk to your doctor before trying any alternative treatments.

How can I cope with lupus?

It is important to take an active role in your treatment. It helps to learn more about lupus - being able to spot the warning signs of a flare can help you prevent the flare or make the symptoms less severe.

It is also important to find ways to cope with the stress of having lupus. Exercising and finding ways to relax may make it easier for you to cope. A good support system can also help.

Marfan syndrome is a disorder that affects the connective tissue in many parts of the body. Connective tissue provides strength and flexibility to structures such as bones, ligaments, muscles, blood vessels, and heart valves. The signs and symptoms of Marfan syndrome vary widely in severity, timing of onset, and rate of progression.

Because connective tissue is found throughout the body, Marfan syndrome can affect many systems, often causing abnormalities in the heart, blood vessels, eyes, bones, and joints. The two primary features of Marfan syndrome are vision problems caused by a dislocated lens (ectopia lentis) in one or both eyes and defects in the large blood vessel that distributes blood from the heart to the rest of the body (the aorta). The aorta can weaken and stretch, which may lead to a bulge in the blood vessel wall (an aneurysm). Stretching of the aorta may cause the aortic valve to leak, which can lead to a sudden tearing of the layers in the aorta wall (aortic dissection). Aortic aneurysm and dissection can be life threatening.

Many people with Marfan syndrome have additional heart problems including a leak in the valve that connects two of the four chambers of the heart (mitral valve prolapse) or the valve that regulates blood flow from the heart into the aorta (aortic valve regurgitation). Leaks in these valves can cause shortness of breath, fatigue, and an irregular heartbeat felt as skipped or extra beats (palpitations).

Individuals with Marfan syndrome are usually tall and slender, have elongated fingers and toes (arachnodactyly), loose joints, and have an arm span that exceeds their body height. Other common features include a long and narrow face, crowded teeth, an abnormal curvature of the spine (scoliosis or kyphosis), stretch marks (striae) not related to weight gain or loss, and either a sunken chest (pectus excavatum) or a protruding chest (pectus carinatum). Some individuals develop an abnormal accumulation of air in the chest cavity that can result in the collapse of a lung (spontaneous pneumothorax). A membrane called the dura, which surrounds the brain and spinal cord, can be abnormally enlarged (dural ectasia) in people with Marfan syndrome. Dural ectasia can cause pain in the back, abdomen, legs, or head. Most individuals with Marfan syndrome have some degree of nearsightedness (myopia). Clouding of the lens (cataract) may occur in mid-adulthood, and increased pressure within the eye (glaucoma) occurs more frequently in people with Marfan syndrome than in those without the condition.

The features of Marfan syndrome can become apparent anytime between infancy and adulthood. Depending on the onset and severity of signs and symptoms, Marfan syndrome can be fatal early in life; however, with proper treatment, many affected individuals have normal lifespans.

Osteogenesis imperfecta (OI) is a group of genetic disorders that mainly affect the bones. The term "osteogenesis imperfecta" means imperfect bone formation. People with this condition have bones that break (fracture) easily, often from mild trauma or with no apparent cause. Multiple fractures are common, and in severe cases, can occur even before birth. Milder cases may involve only a few fractures over a person's lifetime.

There are at least 19 recognized forms of osteogenesis imperfecta, designated type I through type XIX. Several types are distinguished by their signs and symptoms, although their characteristic features overlap. Increasingly, genetic causes are used to define rarer forms of osteogenesis imperfecta. Type I (also known as classic non-deforming osteogenesis imperfecta with blue sclerae) is the mildest form of osteogenesis imperfecta. Type II (also known as perinatally lethal osteogenesis imperfecta) is the most severe. Other types of this condition, including types III (progressively deforming osteogenesis imperfecta) and IV (common variable osteogenesis imperfecta with normal sclerae), have signs and symptoms that fall somewhere between these two extremes.

The milder forms of osteogenesis imperfecta, including type I, are characterized by bone fractures during childhood and adolescence that often result from minor trauma, such as falling while learning to walk. Fractures occur less frequently in adulthood. People with mild forms of the condition typically have a blue or grey tint to the part of the eye that is usually white (the sclera), and about half develop hearing loss in adulthood. Unlike more severely affected individuals, people with type I are usually of normal or near normal height.

Other types of osteogenesis imperfecta are more severe, causing frequent bone fractures that are present at birth and result from little or no trauma. Additional features of these types can include blue sclerae of the eyes, short stature, curvature of the spine (scoliosis), joint deformities (contractures), hearing loss, respiratory problems, and a disorder of tooth development called dentinogenesis imperfecta. Mobility can be reduced in affected individuals, and some may use a walker or wheelchair. The most severe forms of osteogenesis imperfecta, particularly type II, can include an abnormally small, fragile rib cage and underdeveloped lungs. Infants with these abnormalities may have life-threatening problems with breathing and can die shortly after birth.

Rheumatoid arthritis is a disease that causes chronic abnormal inflammation, primarily affecting the joints. The most common signs and symptoms are pain, swelling, and stiffness of the joints. Small joints in the hands and feet are involved most often, although larger joints (such as the shoulders, hips, and knees) may become involved later in the disease. Joints are typically affected in a symmetrical pattern; for example, if joints in the hand are affected, both hands tend to be involved. People with rheumatoid arthritis often report that their joint pain and stiffness is worse when getting out of bed in the morning or after a long rest.

Rheumatoid arthritis can also cause inflammation of other tissues and organs, including the eyes, lungs, and blood vessels. Additional signs and symptoms of the condition can include a loss of energy, a low fever, weight loss, and a shortage of red blood cells (anemia). Some affected individuals develop rheumatoid nodules, which are firm lumps of noncancerous tissue that can grow under the skin and elsewhere in the body.

The signs and symptoms of rheumatoid arthritis usually appear in mid- to late adulthood. Many affected people have episodes of symptoms (flares) followed by periods with no symptoms (remissions) for the rest of their lives. In severe cases, affected individuals have continuous health problems related to the disease for many years. The abnormal inflammation can lead to severe joint damage, which limits movement and can cause significant disability.

• Linear scleroderma
• Morphea

• Diffuse cutaneous systemic sclerosis
• Limited cutaneous systemic sclerosis (which includes CREST syndrome)
• Limited Systemic Sclerosis

Soft tissue sarcoma is a form of cancer that occurs due to abnormal and uncontrolled cell growth of the "soft tissues" of the body. These tissues connect, support and surround other body parts and may include muscle, fat, blood vessels, lymph vessels, nerves, tendons and the lining of joints. Many people with early soft tissue sarcoma have no signs or symptoms of the condition. When present, symptoms depend on the location and size of the tumor but may include a palpable lump under the skin, pain, or difficulty breathing.

Most cases occur sporadically in people with little to no family history of the condition. People who have previously received radiation therapy and those with certain inherited disorders (such as Gorlin syndrome, Gardner syndrome, Li-Fraumeni syndrome, Tuberous sclerosis, neurofibromatosis type 1, and Werner syndrome) have an increased risk of developing a soft tissue sarcoma. The best treatment options depend on many factors but may include surgery, chemotherapy, and radiation therapy.

Surgical incision on the FASCIA. It is used to decompress compartment pressure (e.g. in COMPARTMENT SYNDROMES; circumferential burns and extremity injuries) or to release contractures (e.g. in DUPUYTREN'S CONTRACTURE).

Introduction

A fasciotomy is an emergency procedure used to treat acute compartment syndrome. Compartment syndrome is when the pressure builds up in a non-compliant osseofascial compartment and causes ischemia leading to muscle and nerve necrosis. It occurs most commonly in the volar compartment of the forearm, deep posterior, or anterior leg compartment. It can, however, happen in any closed space where the muscle is surrounded by substantial fascia, eg, the hand, foot, thigh, or buttock.

Compartment syndrome categorizes as acute or chronic. Acute compartment syndrome often follows high-energy trauma, fractures, circumferential burns, crush injuries, or a tight plaster cast. Chronic compartment syndrome develops with muscular overuse and commonly occurs in the leg of runners or military personnel or the forearm of weightlifters and rowers. Occasionally acute exertional compartment syndrome can be seen after strenuous exertion.

Anatomy and Physiology

The lower leg is the most frequent site of compartment syndrome and associated fasciotomy. The lower leg divides into 4 anatomical compartments: anterior, lateral, superficial posterior, and deep posterior.

Anatomical Structures of the Lower Leg

• Anterior compartment of the lower leg
  • Tibialis anterior
  • Extensor halluces longus
  • Extensor digitorum longus
  • Peroneus tertius
  • Deep fibular nerve
  • Anterior tibial artery
• Lateral compartment of the lower leg
  • Peroneus longus
  • Peroneus tertius
  • Superficial fibular nerve
  • Fibular artery
• Superficial posterior compartment of the lower leg
  • Gastrocnemius
  • Soleus
  • Plantaris
  • Sural nerve
• Deep posterior compartment of the lower leg
  • Tibialis posterior
  • Flexor hallucis longus
  • Flexor digitorum longus
  • Popliteus
  • Tibial nerve
  • Posterior tibial artery

The forearm is the most common site of compartment syndrome in the upper limb. The forearm subdivides into 4 anatomical compartments: superficial volar, deep volar, dorsal compartment, and the mobile wad of Henry. The volar compartments are most commonly affected.

Anatomical Structures of the Upper Limb

• Superficial volar compartment of the upper limb
  • Flexor carpi ulnaris
  • Flexor carpi radialis
  • Pronator teres
  • Palmaris longus
  • Flexor digitorum superficialis of the upper limb (often described as being in an intermediate volar compartment)
  • Ulnar nerve
  • Ulnar artery
• Deep volar compartment of the upper limb
  • Flexor digitorum profundus
  • Flexor pollicis longus
  • Pronator quadratus
  • Median nerve
  • Anterior interosseous artery
  • Anterior interosseous nerve
• Dorsal compartment of the upper limb
  • Extensor digitorum
  • Extensor digiti minimi
  • Extensor carpi ulnaris
  • Supinator
  • Extensor indicis
  • Extensor pollicis longus
  • Extensor pollicis brevis
  • Abductor pollicis longus
  • Posterior interosseous nerve
  • Posterior interosseus artery
• Mobile wad of Henry of the upper limb
  • Brachioradialis
  • Extensor carpi radialis longus
  • Extensor carpi radialis brevis
  • Radial nerve
  • Radial artery

The median and ulnar nerves lie in the volar forearm between the flexor digitorum superficialis and profundus. The radial nerve lies deep to the mobile wad.

Indications

The classical features of compartment syndrome are ischemia, pain out of proportion to the injury, paraesthesia, pallor, paralysis, and pain on passive movement, especially stretch of the concerned compartment. Two-point discrimination can be useful for determining nerve ischemia. Depending on the conscious level, sensory state, and communication ability, these signs and symptoms can be challenging to assess. In these circumstances, monitoring of compartment pressures can be useful..

Measuring compartment pressures is possible via multiple methods, none of which have robust supporting evidence. No universal agreement exists on indications for emergency fasciotomy. Some institutes operate if the difference between the compartment pressure and diastolic pressure is less than 20 mmHg. While some surgeons operate if a compartment pressure is greater than 30 mmHg with the correlation of clinical signs.

Contraindications

No absolute contraindication to performing a fasciotomy exists. This section will explore the relative contraindications. Every decision to perform an emergency fasciotomy should be made by a senior team member and on a case-by-case basis considering the patient's medical condition and the injury sustained.

The primary relative contraindication to performing a fasciotomy is delayed presentation. If the clinician suspects compartment syndrome has been present for more than 12 hours, there is a potential risk of reperfusion injury.

One study demonstrated that fasciotomies performed within 6 hours resulted in almost complete limb function recovery; fasciotomies performed between 6 and 12 hours resulted in a normal functional recovery rate of 68%. However, fasciotomies performed after 12 hours resulted in only 8% of patients regaining normal limb function. Conversely, another more recent study revealed no difference in limb salvage rate when comparing early (12 h) to late (>12 h) fasciotomy. The infection rate was significantly higher in patients with delayed fasciotomies. In a 2008 study, 336 combat patients received 643 fasciotomies (upper and lower limb included). Patients who underwent a delayed fasciotomy had twice the amputation rate and 3 times the mortality rate.

Considerations of irreversible nerve and muscle damage and high risk of infection change the risk-benefit analysis in missed compartment syndrome and negate the necessity for emergency surgery.

Equipment

The equipment used to perform a fasciotomy is listed below.

• Antiseptic solution for skin preparation
• Skin scalpel
• Inside scalpel
• Forceps
• Simple hand-held retractor (Langenbeck)
• Diathermy device
• Dressing

Personnel

The procedure can be carried out by any medical professional with the appropriate training; often this is a surgical resident or attending. The one performing the fasciotomy needs direct assistance from a scrub nurse and an anesthetist at a minimum.

Preparation

Before the procedure begins, the surgical team should complete the WHO Surgical Safety Checklist. For a fasciotomy of the leg, the patient is positioned supine. The leg should be prepped and draped to above the knee. For a forearm fasciotomy, the patient should be positioned supine with the arm abducted to 90 on a table extension. The patient should then be prepped and draped to above the elbow.

Technique or Treatment

Single-Incision Fasciotomy of the Leg (Davey, Rorabeck, and Fowler Technique)

• Make a skin incision beginning at the lateral malleolus and extending proximally along the fibula for the full length of the compartment.
• Develop the subcutaneous plane anteriorly to expose the fascial layer. Be aware of damage to the superficial peroneal nerve at this stage.
• Make a longitudinal incision in the anterior and lateral fascial compartments.
• Develop the subcutaneous plane posteriorly and perform a longitudinal incision into the superficial posterior compartment.
• Identify the soleus in the superficial posterior compartment and begin to develop the plane between the distal third of the soleus and the lateral compartment.
• Remove the soleus and the deeper flexor hallucis longus from the posterior fibula. Be aware the peroneal neurovascular bundle will be immediately medial to the fibula.
• Retract the peroneal vessels posteriorly to expose the fascial attachment of the tibialis posterior to the fibula and make a longitudinal incision.
• Apply appropriate wound dressing.

Double Incision Fasciotomy of the Leg (Mubarak and Harges Technique)

• Anterolateral incision
  • Make a 20 cm anterior skin incision centered between the crest of the tibia and the fibula.
  • Identify the anterior intramuscular septum and make a longitudinal incision on either side into the anterior and lateral compartments.
• Posteromedial incision
  • Make a second skin incision starting 2 cm proximal and 2 cm superior to the medial malleolus of the tibia, extending proximally in line with the tibia longitudinally.
  • Carefully use blunt dissection to identify the fascial layer, the long saphenous vein, and the saphenous nerve; retract these anteriorly.
  • Make an incision along the length of the posterior fascial compartment.
  • Make another fascial incision over the flexor digitorum longus muscle immediately posterior and medial to the tibia to release the posterior compartment.

Forarm Fasciotomy

• Volar Incision
  • Make a large skin incision starting just radial to the flexor carpi ulnaris and extending proximally to the medial epicondyle.
  • Extend the incision distally to the wrist crease, cross the wrist crease diagonally towards the hypothenar eminence and into the palm to facilitate a carpal tunnel release.
  • Make a longitudinal incision into the superficial fascial compartment.
  • Retract the flexor carpi ulnaris and the ulnar neurovascular bundle medially.
  • Retract the flexor digitorum superficialis medially. This exposes the deep fascial compartment.
  • Make a fascial incision onto the flexor digitorum profundus.
  • Extend both fascial incisions to the transverse carpal ligament.
• Dorsal Incision
  • Make a 10-cm incision in the skin between the extensor digitorum communis and extensor carpi radialis brevis starting 2 cm distal to the lateral epicondyle. This incision allows the release of the fascia over the mobile wad immediately.
  • Develop the subcutaneous plane posteriorly to expose the extensor retinaculum and release the fascia to decompress the posterior compartment.

Follow-up

Fasciotomy wound management begins with an inspection at 48 hours. If the compartments are soft, this closure is achieved by primary wound closure, secondary wound healing, or split-thickness skin grafting. Split-thickness grafting is needed in approximately 50% of wounds. Delayed primary closure is also feasible using a vessel loop shoelace stitch. The use of a negative pressure wound management device is another option.

Complications

Due to muscle necrosis, rhabdomyolysis and acute renal failure are common; treatment is with intravenous fluids and dialysis.

Incomplete fasciotomies can create a need for a revision fasciotomy either to extend the fascial opening or open a missed compartment. Compartments are most commonly overlooked when the anatomy is highly distorted, such as in cases of high-energy trauma or patients with previous surgery and scarring. In these patients, the associated mortality increased by 4 times.

Also, patients who underwent a delayed fasciotomy have twice the amputation rate and 3 times the mortality. In some cases, even with timely fasciotomies, the affected limb may not regain normal functionality and may result in an amputation.

Wound Complications are as follows:

• Need for skin grafting
• Scaring
• Tendon tethering
• Muscle herniation
• Recurrent ulceration
• Swollen limbs
• Discolored wounds
• Pruritus
• Dry, scaly skin
• Altered sensation

Clinical Significance

Early recognition and subsequent treatment of compartment syndrome with fasciotomies cause a significant decrease in poor functional outcomes, the need for amputation, and the risk of death. Additionally, the medico-legal burden of delayed fasciotomies is high. The time from symptom onset to fasciotomy is directly linked to an increasing payout in medical negligence claims. Fasciotomy performed early is associated with a successful defense of medico-legal action.

Enhancing Healthcare Team Outcomes

Early recognition of compartment syndrome is best detected by those professionals who have regular contact with the patients in the hospital. The hospital staff, typically attending physicians and nursing staff, has the most contact time with patients and is in the best position to detect increasing symptom severity. These staff members need the most training in recognizing compartment syndrome and need to exercise a high index of suspicion. The ability to escalate the situation in a timely fashion to a senior physician who can initiate early aggressive treatment with fasciotomy is crucial. Therefore, department management is responsible for ensuring all healthcare team members have adequate training in recognizing the symptoms, are familiar with pressure measuring equipment, and can escalate the situation appropriately and promptly. Implementing an interprofessional approach provides the best method for early detection to provide the best patient outcomes.

Layers of connective tissue of variable thickness. The superficial fascia is found immediately below the skin; the deep fascia invests MUSCLES, nerves, and other organs.