Charcot Arthropathy

 As obesity continues to rise, so does the prevalence of type 2 diabetes and its complications.  More than 285 million adults worldwide have been diagnosed with diabetes.1  Foot disorders are a leading cause of hospitalization among people with diabetes, and one of the most potentially destructive is neuropathic osteoarthropathy, or Charcot neuroarthropathy.  This condition can be a devastating complication of diabetic neuropathy.  Early intervention is critical to preventing amputation. Charcot arthropathy is a progressive musculoskeletal condition characterized by joint dislocation, fractures and deformities. It results in progressive destruction of bone and soft tissue of weight-bearing joints, most commonly in the foot and ankle. It is most commonly due to diabetes. A Brief Historical Overview Of Charcot In 1703, William Musgrave identified a disease process of arthralgia associated with venereal disease. Mitchell reported on neuropathic joints with spinal cord lesions in 1831. These disease processes were later noted to be consistent with Charcot deformity. In 1868, neurologist Jean-Marie Charcot described the degeneration of ligaments and joint surfaces in a particular patient population. His work was conducted during his clinical experience with patients of tabes dorsalis and other neurological disorders at the Salpetriere Hospital in Paris. It was concluded that many of the vast changes observed were secondary to a loss of neurologic sensation in these patients. Over the years, it has become clear that any peripheral disorder that results in sensory and/or autonomic neuropathy has the potential to produce a Charcot event (see Causes Of Neuroarthropathy). Syphilis was the most common cause of Charcot arthropathy in the 1800s. In 1936, W.R. Jorden linked Charcot arthropathy to diabetes. The evolving medical management and treatment of syphilis has decreased its incidence and long-term complications. Alternatively, the discovery of insulin and other life-preserving treatments of diabetes have not resulted in an eradication of the disease, but have resulted in a longer life span for this patient group. A longer life expectancy for patients with diabetes is a great victory but there is the challenging flipside of facing a higher incidence of the resulting long-term sequelae of the disease. Despite early diagnosis, stricter management and more aggressive treatment of diabetes, the medical community has not been successful in eliminating the long-term complications that often present in these patients. In our present medical community, diabetes is the leading cause of Charcot arthropathy.

Oblique view X-ray in a 45-year-old male diabetic revealed a divergent, Lisfranc dislocation of the first metatarsal with associated lesser metatarsal fractures.

The same 45-year-old man with diabetes mellitus presented with a diffusely swollen, warm and non-tender left foot due to Charcot arthropathy. There are no changes to the skin itself.

Recognize and refer quickly to prevent foot amputation By Katrina Jackson, MSN, NP Posted on: February 8, 2011 Pathophysiology Charcot neuroarthropathy is a destructive disease process that can cause significant bone and joint destruction in a matter of weeks. The pathophysiology underlying Charcot neuroarthropathy is somewhat unclear. Two theories are widely accepted. The neurovascular theory suggests that an autonomic stimulation resulting in increased peripheral circulation causes bone resorption and demineralization of the weight-bearing extremities.5,6 Increased peripheral circulation is believed to be a result of the loss of vascular sympathetic tone associated with autonomic neuropathy in diabetes.5 The neurotraumatic theory says that chronic, repetitive injury to the foot is masked by loss of sensation in the neuropathic foot.2,5 Because the patient with neuropathy does not feel the pain associated with

the initial injury and consequent inflammation, he or she continues to walk on the affected joint, producing further damage. Recent research has examined proinflammatory cytokine and receptor activator of nuclear factor kappa B ligand (RANKL) on osteopenia and neuropathy.2 RANKL is involved in bone metabolism and is regulated by osteoprotegerin. In patients with Charcot neuroarthropathy, the RANKL-osteoprotegerin balance is most likely disrupted by excessive inflammation, which causes an increase in the production of osteoclasts and, subsequently, osteolysis.2 Proinflammatory cytokines reduce pain, allowing the patient with Charcot neuroarthropathy to continue to bear weight on the affected foot and fueling a vicious cycle that promotes further damage.7 The direct mechanism of Charcot neuroarthropathy is unknown, but a recurrent theme is that trauma is the primary precipitating factor. Even a minor event may initiate the process. The longer the trauma-causing Charcot neuroarthropathy persists, the greater the destruction and consequent deformity.5

An x-ray view of Charcot foot deformity. Copyright University of California, San Diego. Appears with permission.

Managing Ulcers on The Charcot Foot

Causes Of Neuroarthropathy Diabetes Use of steroids Alcoholism Trauma Infection Amyloidosis Pernicious anemia Syphilis

 Poliomyelitis Syringomyelia Spina bifida Myelomenigocele Leprosy (Hansens disease) Multiple sclerosis Congenital vascular disease Charcot-Marie-Tooth disease Cord compression Tuberculosis Asymbolia Connective disorders (RA and scleroderma) Ehlers-Danlos syndrome Raynauds disease Adrenal hypercorticism Thalidomide embryopathy Paraneoplastic sensory neuropathy -Any condition causing sensory or autonomic neuropathy at a joint can lead to a Charcot deformity.

Clinical Presentation Although Charcot neuroarthropathy has been associated with other anatomical sites, it is most common in the foot and ankle.8 The patient with Charcot neuroarthropathy typically presents in the acute stage with a unilateral, warm, edematous foot that may or may not be painful or deformed.5,7 Only about one-third of patients with Charcot neuroarthropathy complain of pain or discomfort in the acute stage, increasing the probability of a missed diagnosis.4 A foot with Charcot neuroarthropathy is generally 2 C hotter than the unaffected foot; this difference can be measured with an infrared thermometer.4 A thorough history is important for any patient, but a more meticulous assessment should be performed on the patient with neuropathy. A history of recent trauma is a red flag for a Charcot process.8 Compare the feet side by side to detect asymmetry, and take the temperature of each foot using an infrared thermometer. Early in the deformity, a patient with Charcot neuroarthropathy may complain of not being able to fit into an everyday shoe. If the condition goes unnoticed or is misdiagnosed at this point, the patient may not present again until a change in foot anatomy has occurred. Patients may complain of altered foot shape or that they hear bones crunching when they walk.4 A common finding at this point is a rocker bottom foot deformity, which increases plantar ulcer risk via the increased pressure applied to plantar aspects of the foot (see image).4,9 Repeated weight bearing on the affected foot leads to abnormal joint alignment and eventual impairment of joint mobility. Once the joint begins subluxating, the cartilage and subchondral bones begin to erode, resulting in bone dislocation and fragmentation.10 Diagnostic Studies The diagnosis of Charcot neuroarthropathy is mainly a clinical one, with objective testing used to exclude other possibilities.3 Charcot neuroarthropathy is often misdiagnosed as cellulitis,

osteomyelitis, deep vein thrombosis or gout.3-5 Observing the extremity while it is elevated can help differentiate this condition from cellulitis. In Charcot foot, erythema often dissipates quickly when the affected extremity is elevated. This is due to the arteriovenous shunting that occurs in this condition. In cellulitis, erythema generally persists despite elevation.5 Another complicated differential surfaces when a patient presents with a concomitant ulceration, making osteomyelitis a top concern. Laboratory studies and x-rays may help differentiate between the two. Deformity is typically present with Charcot neuroarthropathy, but it is rare with osteomyelitis unless underlying Charcot foot is present.8 The deformity associated with Charcot neuroarthropathy, which results from joint subluxation or dislocation, can often present as a flattening of the foot arch as it collapses, or as new bony protrusions as a result of the deformity. For the same reason, radiographs also help exclude gout. It is important to note, however, that x-ray results may be normal if the patient presents very early in the acute phase of Charcot neuroarthropathy. Therefore, it is important never to rule out Charcot foot based on a normal radiograph result.3 MRI or a bone scan should produce unequivocal answers. When Charcot neuroarthropathy is present, a bone scan shows evidence of disease. Similarly, MRI shows microfractures and bone marrow edema even in the earliest stages of the disease process.3 A complete blood count with differential is helpful in distinguishing between an infectious process and Charcot neuroarthropathy. In an infectious process, white blood cells will be elevated and demonstrate a shift to the left. Further testing choices for investigation of infection include a comprehensive metabolic panel, erythrocyte sedimentation rate, C-reactive protein, blood cultures and hemoglobin A1c.5 Because unilateral swelling can occur with deep vein thrombosis, a venous Doppler examination may be needed to rule out this possibility. Swelling associated with Charcot neuroarthropathy is generally more intense than that seen with deep vein thrombosis. Specialist Referral If the presence of Charcot foot seems possible or likely, refer to an orthopedist, podiatrist or a diabetic foot center for prompt evaluation. Immobilize the affected foot and prescribe strict avoidance of weight bearing to prevent further damage.2,10 Use of crutches or a walker is necessary awaiting diagnosis. Treatment options for Charcot neuroarthropathy depend on the stage at diagnosis. In an acute Charcot neuroarthropathy, strict immobilization and stress reduction are the mainstays of treatment.2,3 Non-weight bearing can be achieved with the use of crutches, a walker or a wheelchair. Immobilzation with a splint, cast or removable cast is recommended until hyperemia is resolved.2 In the initial stages, frequent cast changes are necessary to adjust to reductions in foot edema. This usually occurs within the first 48 hours, but it may continue for weeks. During this period of changing foot anatomy, cast changes are needed at least every 2 to 3 weeks.3 Immobilization is typically required for 3 to 6 months.2,3 In addition to the above modalities, the use of bisphosphonates and electrical bone stimulation may help improve healing time and promote rapid consolidation of fractures.2,3,10 In contrast to the acute Charcot foot, the chronically deformed and unstable Charcot foot requires surgical reconstruction.5 This approach encounters less criticism and debate than surgical intervention in the acute stage.5 Surgical management varies greatly depending on the

size, anatomic location and degree of instability in the foot. The patient's health status also plays a role. Surgery usually includes external fixation or intramedullary nails, followed by a period of rest and immobilization.2,5 The overriding goal of treatment is to avoid amputation and prevent further deformity. Good outcomes can be managed with footwear that allows adequate gait and activity, thus sustaining overall quality of life.3

Disease mechanism Two major theories exist explaining the pathophysiology of Charcot arthropathy: 1. Neurotraumatic theory: A joint without proper sensory innervation is subject to repeated injury. The patient is unaware of minor trauma to the joint and continues to damage it over time. 2. Neurovascular theory: Loss of sympathetic vascular tone leads to increased blood flow to the joint, causing an imbalance in bone metabolism. Over time the joint becomes osteopenic Both the neurotraumatic and neurovascular mechanisms are likely to be involved and complement each other. The joint, mechanically weaker due to bone loss is subject to repeated minor injuries, and the patient is unaware of the destruction until the joint is badly damaged. Symptoms and signs The clinical presentation varies depending on the stage of the disease from mild swelling to severe swelling and moderate deformity. Inflammation, erythema, pain and increased skin temperature (3-7 degrees celsius) around the joint may be noticeable on examination. X-rays may reveal bone resorption and degenerative changes in the joint. These findings in the presence of intact skin and loss of protective sensation are pathognomonic of acute Charcot arthropathy. Roughly 75% of patients experience pain, but it is less than what would be expected based on the severity of the clinical and radiographic findings Treatment Treatment is usually non-operative, consisting of reduction of stress on the joint by casting, avoiding weight bearing where possible, and elevation to reduce blood flow (decreasing inflammation and bone loss). Only about 25% of cases require surgery. Outcome Outcomes vary depending on the location of the disease, the degree of damage to the joint, and whether surgical repair was necessary. Average healing times vary from 5597 days depending on location. Up to 12 years may be required for complete healing. Author(s):

By Pamela M. Jensen, DPM, and John S. Steinberg, DPM Eichenholtz described the staging of Charcot arthropathy, which is based on clinical and radiographic changes that progress from the acute phase through the healing phase (calescence) to the resolution phase. One should use radiographs to stage the disease and monitor its progression. In the acute or active stage, you will see persistent or progressive joint effusion, narrowing of the joint space, soft tissue calcification, minimal subluxation, preservation of bone density (unless infected) and fragmentation of eburnated subchondral bone. This is often followed by progressive destruction of articular surfaces, subchondral sclerosis, osteophytosis, intraarticular loose bodies, continued subluxation and fracture/dislocation of the bony architecture. The healing or coalescent stage is observed with serial radiographs that show absorption of fine debris, periosteal new bone formation, fusion of bone fragments, exuberant bone growth, decreased joint mobility, increased stabilization, increased bone density and sclerosis with remaining deformity. The remodeling (reconstructive) stage is significant for repair, consolidation and healing allowing for increased stability with remodeling and ankylosis.

Ancillary Diagnostic Methods You Need To Know There are some optional procedures that can be helpful when diagnosing Charcot. You can use bone scintigraphy when you are unable to differentiate between osteomyelitis and Charcot. Clinical diagnosis can be a dilemma when a patient presents with a chronic nonhealing ulceration secondary to a Charcot deformity. Is the ulceration simply due to the increased pressure of the bony deformity or is there underlying osteomyelitis? This is often difficult to assess using clinical and plain film radiography alone. Confirmatory diagnosis methods can meet with some ambiguity and debate, but there are more tests available now that may suit your particular patient situation. Indium-111 white blood cell scan is more specific than technetium-99 and gallium-67 scans. When positive in the very late phases (up to 24 hours), Indium-111 WBC scans can be used to confirm a diagnosis of osteomyelitis. MRI may be useful in distinguishing between osteomyelitis and Charcot but the sensitivity and specificity are often dependent on the radiologists technical skill. Generally, obtaining a MRI can be helpful for surgical planning as it allows you to assess the proximal extent of possible bone infection. Bone biopsy remains the gold standard for identifying osteomyelitis and differentiating Charcot arthropathy. A microscopic analysis of osteomyelitis will reveal an eroded bone architecture and a field filled with leukocytes. In the case of Charcot arthropathy, a synovial biopsy identifies small fragments of bone and cartilage debris that are embedded in the synovium due to joint destruction rather than infection. How To Differentiate Between Charcot And Osteomyelitis y y Open ulceration in a neuropathic foot always necessitates careful evaluation to ensure a correct diagnosis and to prevent unnecessary amputation. Clinical presentation of an active Charcot event with erythema, swelling and increased warmth can appear very similar to an infection.

y y

When the clinical presentation lacks ulceration, a point of entry or an abscess, the patient most likely is in the early active stages of Charcot arthropathy. However, in the face of a chronic ulceration that presents with concurrent extremity redness, edema and calor, you must carefully scrutinize your clinical suspicion of osteomyelitis.

Managing Ulcers On The Charcot Foot Author(s): By Pamela M. Jensen, DPM, and John S. Steinberg, DPM y Ulcerations with an apparently healthy wound base, no evidence of bone exposure and indefinite radiographic findings may require serial radiographs and ancillary studies, such as a bone scan or bone biopsy, to aid you in the decision-making processes. y Bone imaging is extremely sensitive for bone pathology but it is often less specific and inconclusive. Technetium-99 is effective for localizing the area of bone involved but it is often insufficient to completely differentiate between Charcot and osteomyelitis. y Indium-111 imaging has an overall sensitivity of 90 percent and a specificity of 95 percent for detecting an abscess. However, when the literature reviewed is focused on the ability of Indium testing to differentiate the causative pathology, the sensitivity is noted to range from 62 to 98 percent for osteomyelitis. y Often, one may employ a combination of imaging techniques to localize the activity and identify osteomyelitis versus Charcot arthropathy. Keep in mind that chronic osteomyelitis is noted to have a lower sensitivity for Indium-111 imaging and clinical follow-up is essential. y Lab studies can aid with the clinical diagnosis and differentiation of Charcot arthropathy versus osteomyelitis but there is significant ambiguity. White blood cell count (WBC) with differential will be elevated with a left shift when infection is present, but WBC can also be a marker for inflammation and may be moderately elevated in Charcot arthropathy. y To add to this complex situation, not all people with diabetes are able to mount a significant immune response to infection. Therefore, they may have normal appearing WBC levels in the face of abscess/osteomyelitis. Erythrocyte sedimentation rate (ESR) is elevated in infection and is also a nonspecific marker for inflammation. Significantly increased ESR>70 is highly predictive of osteomyelitis.4 Glycosylated hemoglobin indicates the level of hyperglycemic control. y Hyperglycemia causes nonenzymatic collagen glycosylation, which can lead to laxity in ligaments and unstable joints as well as neuropathy. y In non-diabetics, obtaining B-12/folate deficiency and liver function tests may be necessary to rule out peripheral neuropathy as a result of chronic alcoholism. A Guide To Conservative Treatment Options y When you seek to treat a patient conservatively, you have numerous options. Total contact casting (TCC) is the gold standard of conservative treatment for immobilizing, offloading and supporting the structure of the foot until the acute stage of Charcot arthropathy resolves. Ideally, one should combine a TCC with complete nonweightbearing (NWB) of the affected extremity but NWB status is often difficult to obtain

y y

in this patient population due to concurrent obesity, visual impairment and upper extremity weakness. Patients unable to comply with NWB status will have a longer healing time and risk further deformity and ulceration. When using TCC, be aware that the modality requires weekly cast changes for three to six months with regular debridement of pre-ulcerative or ulcerative lesions. Alternative devices that can assist in partial non-weightbearing include the removable cast walkers, patellar tendon-bearing brace, accommodative footwear with a modified AFO, a Charcot restraint walker (CROW) and double upright AFO. Obtain serial radiographs monthly to evaluate progressio Discontining offloading is dependent on clinical, radiographic and dermal thermometric signs of quiescence. In our practice, we generally combine a return to weightbearing status with progression into a removable cast walker device for two to three weeks. Subsequently, we emphasize appropriate prescriptive shoewear, including custom molded shoes, extra depth with plastazote insoles and steel shank shoes with a rocker bottom if the patient has an ulceration. Long-term management requires life-long protection of the involved extremity or possible surgical intervention to establish a stable weight bearing platform.

Pearls For Surgical Treatment Goals of surgical treatment include correction of the deformity, which prevents the foot from being protected appropriately in accommodative shoewear. In addition, seek to create a stable plantigrade foot that allows for ambulation and minimizes the risk for ulceration.

Managing Ulcers On The Charcot Foot

Conclusion: More favorable outcomes in the face of Charcot arthropathy are noted when there is early/immediate diagnosis and aggressive management. Treatment of concurrent ulceration in Charcot arthropathy includes offloading, immobilization, debridement and local care. Ulcer treatment for the Charcot patient is very much the same as that which would be employed in the non-Charcot neuropathic diabetic foot ulceration. One should consider aggressive use of bioengineered tissues and other advanced healing modalities early in the treatment course due to the high risk of these particular ulcerations. Ruling out underlying infection/osteomyelitis is more complicated in the ulceration due to Charcot but one should employ advanced imaging techniques and bone biopsy in the face of an uncertain clinical scenario. In cases in which offloading and local care for Charcot arthropathy/ulceration is not successful and re-ulceration and worsening of the deformity is present, consider surgical intervention through exostectomy or other reconstructive procedures to provide the best long-term outcome. In the Charcot foot, it is imperative to address the underlying etiology when possible. Ulceration in Charcot can usually be directly attributed to a resultant deformity or a malalignment affecting the plantar weightbearing surface. In the ideal scenario, the active ulceration would first be healed with offloading, debridement and local management. Once you have achieved a closed skin envelope, you can perform the appropriate surgical procedures to address the causative factors and prevent ulcer recurrence. In this manner, one can minimize surgical complications of wound infection and dehiscence.

Dr. Steinberg is an Assistant Professor in the Department of Orthopaedics/Podiatry Service at the University of Texas Health Science Center in San Antonio, Texas. He is a Fellow of the American College of Foot and Ankle Surgeons. Dr. Jensen is a second-year resident at the University of Texas Health Science Center in San Antonio, Texas. CE Exam #110 Choose the single best response to each question listed below: 1. What was the most common cause of Charcot in the 1800s? a) diabetes b) pneumonia c) osteomyelitis d) syphilis 2. Which of the following is the least common modern cause of Charcot arthropathy? a) diabetes b) tabes dorsalis c) pneumonia d) syringomyelia 3. Charcot most commonly presents in which of the following patterns? a) upper extremity, unilaterally b) lower extremity, unilaterally c) upper extremity, bilaterally d) lower extremity, bilaterally 4. Which of the following are the two most commonly accepted theories which describe the pathophysiology of Charcot? a) ischemic and neuromuscular b) neuromuscular and neurovascular c) infectious and neurotraumatic d) neurotraumatic and neurovascular 5. Increased temperature in an extremity can be a clinical finding in Charcot. How many degrees of temperature difference is typically noted in an active Charcot extremity when compared to the unaffected limb? a) 1-2 F b) 3-7 F c) 15-20 F d) 22-30 F 6. Which of the following is the gold standard for differentiating Charcot from osteomyelitis? a) indium-111 b) technetium-99 c) WBC with differential d) bone biopsy 7. Which of the following is the gold standard for offloading the acute Charcot ulceration? a) total contact cast b) removable cast walker c) CROW walker d) custom molded shoe

8. Equinus is problematic in the face of Charcot arthropathy because it a) produces increased plantar forefoot pressures b) can cause midtarsal joint stress and collapse c) increases ulceration risk d) all of the above 9. Which of the following describes a complication directly related to overaggressive bone resection when performing an exostectomy in the Charcot foot? a) destabilization of the remaining joints b) infection c) dehiscence d) gangrene References: