Charcot Neuropathic Osteoarthropathy (Charcot Joint)


Article Author:
Amelia Harris


Article Editor:
Melanie Violand


Editors In Chief:
Sisira Reddy
Joseph Nahas
Chokkalingam Siva


Managing Editors:
Avais Raja
Orawan Chaigasame
Khalid Alsayouri
Kyle Blair
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beenish Sohail
Hajira Basit
Phillip Hynes
Sandeep Sekhon


Updated:
1/27/2019 8:29:04 AM

Introduction

Charcot neuropathic osteoarthropathy is a destructive joint disorder initiated by trauma to a neuropathic extremity. It can lead to dislocations and fractures of the foot. Correct diagnosis and treatment of acute Charcot are imperative to decrease permanent foot deformity and allow for a stable and plantigrade foot that is suitable for ambulation. This review article of Charcot discusses etiology and pathogenesis of the disease, presentation, and treatment options.[1][2][3]

Etiology

Charcot neuropathic osteoarthropathy is seen in the lower extremity and is characterized by bone and joint fragmentation of the foot and ankle in individuals with various peripheral neuropathies. Diabetes, neuropathy, trauma, and metabolic abnormalities of the bone result in an acute localized inflammatory condition. The inflammatory response can permanently disrupt the bony architecture of the foot resulting in abnormal plantar pressures that are at risk for ulceration and amputation.[4][5][6][7]

Charcot is a debilitating condition affecting the lower extremity of patients with established peripheral neuropathy caused by many complicated etiologies; however, diabetic neuropathy has become the most common etiology.

Epidemiology

It has been reported that Charcot affects between 0.1% to 0.9% of people with diabetes. An estimated 63% of patients with Charcot neuropathic osteoarthropathy will develop a foot ulcer. McEwen et al. found a significant association between elevated body mass index and Charcot.

Pathophysiology

There are two commonly accepted theories used to describe the pathogenesis of Charcot: neuro-traumatic and neurovascular. The neuro-traumatic theory proposes neuropathy, and repeated trauma produces joint destruction. The neurovascular theory suggests the increased peripheral blood flow results in osteolysis and demineralization.

Histopathology

Pathophysiologically, key inflammatory markers have been identified that contribute to the development of an acute Charcot foot. Calcitonin gene-related peptide (CGRP) acts typically at the nerve terminal to antagonist the synthesis of nuclear factor-kB ligand (RANKL), a cytokine involved in the inflammatory process. However, in the neuropathic foot, the CGRP is not functioning, and therefore RANKL is synthesized without inhibition. RANKL is a very important marker in the development of Charcot as it is responsible for the proliferation of osteoclastogenesis. The RANKL osteoclastic relationship is normally moderated by osteoprotegerin (OPG) by acting as a decoy receptor to RANKL binding. In the Charcot foot, this relationship between RANKL and OPG is disrupted. The unregulated synthesis of RANKL accounts for the excessive bony turnover and accumulation that is seen in the Charcot limb. Additionally, CGRP may have a further role in Charcot in that it is involved in upholding the integrity of the joint capsule.  Therefore, in its absence, it is presumed to lead to joint destruction and dislocation that is characteristic of the Charcot foot.

History and Physical

A high suspicion for Charcot needs to be present to diagnose the condition accurately. Charcot presents as an erythematous foot with edema and color. Often, it is unilateral with a sudden onset of symptoms. Charcot may be initially misdiagnosed as a deep venous thrombosis or cellulitis. Charcot can also be confused with osteomyelitis due to the similar clinical appearance of a red, hot swollen foot with skeletal lysis and often unilateral presentation.  Although distinct and separate processes, there is evidence that osteomyelitis can lead to Charcot. This is due to the inflammatory cascade of cytokine release osteomyelitis triggers in the body.  This risk of osteomyelitis evolving into Charcot is further escalated if surgical intervention was performed to remove the infected bone.  This is because both osteomyelitis and surgery are forms of trauma that promote an inflammatory response which triggers the opportunity for Charcot to manifest.

In addition to the trauma caused by surgery, the changes to the bony architecture of the foot post-surgery can also lead to inflammatory triggering events due to biomechanical and gait alterations in plantar pressures. For example, amputations and other surgical interventions of the foot can lead to altered pressure points for which the foot is unable to compensate. These changes in plantar pressure, even if mild, can cause microtrauma which initiates the inflammatory events that lead to an acute Charcot.[8][9][10][11]

Evaluation

Stages of Charcot

Common classifications that map the phases of Charcot include the Eichenholtz classification and the Sanders and Frykberg classification.  Eichenholtz classifications describe the three stages of disease progression based on clinical and radiographic findings. The Sanders/Frykberg classification is used to type and class the five common anatomical locations of Charcot in the foot.

Eichenholtz

Development:

Clinically: Erythema, foot edema, elevated temperature, no pain

Radiographically: Boney debris at joints, fragmentation of subchondral bone, joint subluxation and or fracture dislocation

Coalescence:

Clinically: Decreased signs of inflammation

Radiographically: Worsening of stage 1 features.  Absorption of boney debris with new bone formation. Coalescence of large fragments with sclerosis of bone ends.  Some increased stability

Consolidation:

Clinically:  Resolution of inflammation.  Changes in overall foot architecture due to underlying final bony remodeling that can lead to new pressure points which are at risk of ulceration

Radiographically: Remodeling of affected bones and joints

Sanders and Frykberg

  1. Metatarsophalangeal to interphalangeal joints: 15%
  2. Tarsometatarsal joints: 40%
  3. Naviculocuneiform joint, navicular-cuneiform, talonavicular and calcaneocuboid joints: 30%
  4. Ankle and subtalar joints: 10%
  5. Calcaneus: 5%

Treatment / Management

Treatments of Charcot aim to decrease permanent foot deformity and ultimately allow for a stable and plantigrade foot that is stable and suitable for ambulation. In the acute phase, it is imperative to immobilize the foot and restrict weight bearing to prevent permanent deformity. Non-operatively, this is achieved through off weight-bearing via total non-weight bearing or protective weight bearing devices. Assistive devices such as crutches and wheel chairs can aid with non-weight bearing. Total contact casts (TCC) with a controlled ankle motion (CAM) walker can provide protected weight bearing. TCC redistribute and reduce pressures on the plantar foot while allowing ambulation. The hyperemic phase of the Eichenholtz classification can last for weeks. By using these types of protective measures, the foot may heal the fractures in a stable position if the stress does not exceed the rate of healing.

Pharmacological treatments also exist to control the osteoclastic activity including bisphosphonates and calcitonin supplements.  Bisphosphonates may help the acute phase of Charcot as they inhibit osteoclastic reabsorption. Calcitonin also serves as an antiresorptive agent. Alternative agents include pamidronate or zoledronic acid which act on new hydroxyapatite crystal by blocking osteoclast precursors in the newly formed bone matrix.

Pearls and Other Issues

Charcot is a rare but serious complication of peripheral neuropathy that can mimic many other disease processes common in the foot of a patient with diabetes. Correctly diagnosing Charcot osteoarthropathy is imperative due to the risk of permanent foot deformity that can be at risk for ulceration and amputation. For the acute phase, treatment endorses non-weight bearing with immobilization of the affected limb to prevent permanent deformity as the disease progresses through the Eichenholtz stages. However, total non-weight bearing and immobilization cannot always be achieved. Common reasons for nonadherence include the length of time required for non-weight bearing, lack of physical ability, and employment restrictions. Alternative treatment options to total non-weight bearing include administration of offloading ambulating devices. Many offloading ambulating devices exist, such as the controlled ankle motion walker with an offloading insole, total contact cast or assistive devices like knee scooters.

Enhancing Healthcare Team Outcomes

The management of charcot joint is an interprofessional; any error in the diagnosis or failure to treat it can lead to the progression of damage and amputation. The aim of treatment is to prevent progression of the neuropathic joint and avoid amputation. Acutely, immobilization may help. Later the patient may be asked to limit weight bearing. Most of these patients may benefit from an ambulatory device. Total contact casts (TCC) with a controlled ankle motion (CAM) walker can provide protected weight bearing. TCC redistribute and reduce pressures on the plantar foot while allowing ambulation. The hyperemic phase of the Eichenholtz classification can last for weeks. By using these types of protective measures, the foot may heal the fractures in a stable position if the stress does not exceed the rate of healing. 

Pharmacological treatments also exist to control the osteoclastic activity including bisphosphonates and calcitonin supplements.  Finally, patients must be educated on foot safety and wear proper shoe wear both inside and outside the home. Unfortunately, once charcot joint has developed, the risk of amputation is always present.[3] (Level V)


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Charcot Neuropathic Osteoarthropathy (Charcot Joint) - Questions

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In what condition is Charcot joint most commonly seen?



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A 45-year-old southeast Asian male presents with a long history of painless swelling and deformity of his right ankle. On physical examination, the ankle is swollen and the lower limb reflexes are absent. His pupils also fail to react to light. Blood work reveals normal blood glucose and a normal white blood cell count. Which of the following is the most likely diagnosis?



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Charcot joint deformity in a 44-year-old is associated with which of the following disorders?



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Patients with which condition most commonly have Charcot joint deformity?



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A patient with diabetes mellitus has a foot deformity accompanied by erythema and joint pain. What is the most likely diagnosis?



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According to the Eichenholtz classification system describing the disease progression of Charcot, which stage leads to the most destruction of the arch of the foot?



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Charcot Neuropathic Osteoarthropathy (Charcot Joint) - References

References

Kampen WU,Westphal F,Van den Wyngaert T,Strobel K,Kuwert T,Van der Bruggen W,Gnanasegaran G,Jens JH,Paycha F, SPECT/CT in Postoperative Foot and Ankle Pain. Seminars in nuclear medicine. 2018 Sep;     [PubMed]
Figueiredo A,Ferreira R,Alegre C,Fonseca F, Charcot osteoarthropathy of the knee secondary to neurosyphilis: a rare condition managed by a challenging arthrodesis. BMJ case reports. 2018 Aug 20;     [PubMed]
Dixon J,Coulter J,Garrett M,Cutfield R, A retrospective audit of the characteristics and treatment outcomes in patients with diabetes-related charcot neuropathic osteoarthropathy. The New Zealand medical journal. 2017 Dec 15;     [PubMed]
Zhao HM,Diao JY,Liang XJ,Zhang F,Hao DJ, Pathogenesis and potential relative risk factors of diabetic neuropathic osteoarthropathy. Journal of orthopaedic surgery and research. 2017 Oct 2;     [PubMed]
Sinacore DR,Bohnert KL,Smith KE,Hastings MK,Commean PK,Gutekunst DJ,Johnson JE,Prior FW, Persistent inflammation with pedal osteolysis 1year after Charcot neuropathic osteoarthropathy. Journal of diabetes and its complications. 2017 Jun;     [PubMed]
Trieb K, The Charcot foot: pathophysiology, diagnosis and classification. The bone     [PubMed]
Miller RJ, Neuropathic Minimally Invasive Surgeries (NEMESIS):: Percutaneous Diabetic Foot Surgery and Reconstruction. Foot and ankle clinics. 2016 Sep;     [PubMed]
Płaza M,Nowakowska-Płaza A,Walentowska-Janowicz M,Chojnowski M,Sudoł-Szopińska I, Charcot arthropathy in ultrasound examination - a case report. Journal of ultrasonography. 2016 Jun;     [PubMed]
Fosbøl M,Reving S,Petersen EH,Rossing P,Lajer M,Zerahn B, Three-phase bone scintigraphy for diagnosis of Charcot neuropathic osteoarthropathy in the diabetic foot - does quantitative data improve diagnostic value? Clinical physiology and functional imaging. 2017 Jan;     [PubMed]
Mautone M,Naidoo P, What the radiologist needs to know about Charcot foot. Journal of medical imaging and radiation oncology. 2015 Aug;     [PubMed]
Hofstaetter SG,Trieb K, [Diagnostic for Charcot foot]. Der Orthopade. 2015 Jan;     [PubMed]

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