Charcot Joint Disease – Symptoms, Causes, Treatment

Charcot Joint Disease is a non-infectious, progressive bone and soft tissue degeneration disorder, most commonly observed in the weight bearing joints like the foot, as a complication of diabetes mellitus. Hence, it is also called Diabetic Neuropathic Arthropathy or Neuropathic Osteoarthropathy. The disease eventually causes severe foot deformity due to loss of sensation (also called diabetic neuropathy) in the region and may require amputation in severe cases.

Seldom, the etiology may be tertiary syphilis, leprosy, spinal cord injury, multiple sclerosis, poliomyelitis or rheumatoid arthritis, chronic alcoholism, congenital insensitivity to pain with anhidrosis (CIPA) or cerebral palsy. Basically, any condition resulting in decreased peripheral sensation.

Symptoms:

Charcot’s foot is characterized by joint swelling (edema), increase in local skin temperature, erythema (redness) and loss of protective sensation: all symptoms consistent with inflammation, except pain. The patient does not feel pain in the swollen joint, which distinguishes Charcot’s disease from osteomyelitis. The patient may also present with instability, loss of joint function and joint crepitus (grinding in the involved joint which represents actual unstable bone fragments rubbing against each other).

Due to unnatural dislocation and relocation of bones in the joint, chronic sores (ulcers) may develop from the abnormal pressure and weight shifting in the region.

The affected areas are the weight bearing joints like the forefoot, ankle, knee, hip and, sometimes, shoulder.

Further investigation through X-Ray imaging may reveal joint deformity, the severity of which varies depending on the stage of the disease. In the initial stages, there may also be no visible deformity in the scans. A bone scan/indium scan is a nuclear medicine test that is done to rule out bone infection. An MRI or bone scan is rarely required to check for inflammation or infection due to abscess formation.

Causes:

There are mainly three underlying, interlinked causes for Charcot’s foot to develop in a patient:

Neurotrauma

Severe diabetes mellitus is known to cause to peripheral neuropathy or nerve damage causing loss of sensation which leads to repetitive microtrauma to the joint; this damage, however, goes unnoticed by the patient. Subsequent inflammatory response and resorption of the bone in the region renders the area weak and prone to further trauma.

Neurovascular

Desensitized joints receive significantly greater blood flow due to a dysregulated autonomic nervous system in neuropathic patients. This leads to higher than normal bone resorption and degeneration which further makes the joint susceptible to injury due to mechanical stress.

Bone injury – A key component to developing Charcot’s disease is a preceding history of injury or trauma to the joint or extremity. This injury often goes undetected due to pre-existing neuropathy. Else, it may not be perceived alarmingly due to loss of sensation. Consequently, the patient would have continued normal or near normal activity leading to further fracture, dislocation and trauma of the bone in the joint.

Treatment:

The choice of treatment depends on the severity of damage to the joint and whether the damage done can be reversed non-surgically.

Casting

The primary treatment is non-operative. Immobilization and pressure reduction (off-loading) of the joint is the mainstay of treatment to prevent any further injury to the same joint. Casting, braces or orthoses are used for immobilization and reducing the pressure.

A cast may be required for as long as three to six months. The swelling, redness and high temperature usually subsides in six to nine months with decreased joint crepitus.

The cast boot is used for completely immobilizing the foot and ankle. It prevents any movement in the toes and ankle, even rotation of the ankle is restricted. It requires that no pressure be put on the joint till it is completely healed, hence the cast is shaped to take pressure away from the joints to the leg. Crutches, walker or wheelchairs are given to help people be mobile with the cast.  The joint is X-Rayed approximately every month to monitor progress of healing.

Even after the joint is completely healed, the patient will be asked to wear special custom shoes that take off the pressure from bony prominences to reduce occurrence of pressure ulcers or to allow an existing ulcer to heal, as the case may be. Some people may not be able to wear normal footwear as it won’t fit the deformed foot. Hence, specially designed diabetic boots are suggested.

Braces and orthoses may be used if a cast cannot be used for some reason, say an open ulcer. They are also used for post-cast pressure off-loading.

The patient is asked to avoid laying further stress on it by keeping the joint non-weight bearing or partial weight bearing with the help of crutches and walkers, and periodically monitor the limb for higher than normal skin temperature, redness or swelling, recurrence of joint crepitus and prevent further injury.

Complete recovery can take a year to two years; however, lifelong self-examination, vigilance, monitoring and pressure reduction is needed.

Surgery

A major contradiction to surgery is active inflammation of the location and lower healing capability in diabetics. But unstable fractures and deformities, complex dislocations, fractures that occur in the softer bones of diabetic and appearance of bony prominences in the foot require surgery to treat. The surgery may involve removal of the bony prominence, screw and plate fixation, reconstructive fusion and repositioning surgery, bone grafting and amputation.

Depending on the amount of bone destruction, poor quality of soft tissue and the risk of infection of post-operative sutures, surgery may need to salvage the healthy tissue.

Post-operative period is crucial as the healing time for diabetics is naturally slow and chances of infection are greater. Use of crutches, off-loading and casting is required even after successful surgery.

The most common complication of Charcot’s disease is foot and ankle deformity even following early and appropriate treatment. This occurs due to such significant bone and joint destruction that the functional integrity of the foot is sacrificed. Instability of the foot and ankle during ambulation is another complication that requires major reconstructive surgery to treat this complication.

To ensure the best outcome from the treatment, the patient should ensure that the doctor’s instructions are followed regarding preventive monitoring and continuous use of walking aids to avoid putting pressure on the injured foot. Diabetic patients must check both their legs daily and lower their blood sugar to prevent further recurrences and for the treatment to work effectively. In addition, the sooner a disease is diagnosed, the better it can be treated and with minimum complications. Hence, both responsibilities of the patient are extremely important as preventive measures.

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