Neuropathic (Charcots) joints

Neuropathic Arthropathy

Dr.Subodh Pathak

Jean-Martin Charcot

Jean-Martin Charcot  29 November 1825 – 16 August 1893) was a French neurologist and professor of anatomical pathology. He is known as "the founder of modern neurology"

Definition

Neuropathic arthropathy , neuropathic osteoarthropathy, Charcot joint refers to progressive condition of the musculoskeletal system that is characterized by joint dislocations, pathologic fractures, and debilitating deformities.

Charcot Arthropathy :James K DeOrio, MD Associate Professor of Orthopedic Surgery, Duke University School of Medicine .

Walter Panis, MD Clinical Instructor, Department of Physical Medicine and Rehabilitation, Spaulding Rehabilitation Hospital, Harvard Medical School

History

The first description of neuropathic arthropathy was by Musgrave in 1703, in his book De Arthritide Symptomatica.He described a neuropathic joint as an athralgia.

1868 Jean-Martin Charcot gave the first detailed description of this disease.

In 1892, Sokoloff --upper extremity with syringomyelia.

In 1927 Leriche stated that a lesion of sympathetic led to Hyperaemia and bone resorption. In 1936, Jordan -diabetes mellitus ---neuropathic changes in the foot and ankle.Associated with intra-articular corticosteroid injections by Chandler and Wright in 1958.A.C Brower—Neurovascular theory

Etiology

Any condition that causes sensory or autonomic neuropathy

Diabetes mellitus neuropathy  Multiple Sclerosis Alcoholic Neuropathy Syringomyelia Cerebral palsy Leprosy

Tabes Dorsalis Spinal cord injury Myelomeningocele Intra-articular steroid injections Congenital insensitivity to pain CMTD Familial interstial Polyneuropathy Amyloidosis Pernicious Anemia

Vitamin B12 Deficiency Phenylbutazone ,Indomethacin Ethyl Alcohol.

Diabetes mellitus is currently the most common cause of neuropathic arthropathy.

Neuropathic joint destruction develops in approximately 0.1% of patients with diabetes and 5% of those with peripheral neuropathy

Neuroarthropathy among all pts with tabes dorsalis ranges b/w 5 to 10%

75% of this 5-10% involve lower extremities and 25% upper extremities.

Pathophysiology

Major theories

– Neurotraumatic theory

– Neurovascular theory

– Most probably both

Neurotraumatic Theory

Loss of peripheral sensation and proprioception leads to repetitive micro trauma to the joint in question

This damage goes unnoticed by the neuropathic patient, and the resultant inflammatory resorption of traumatized bone renders that region weak and susceptible to further trauma.

Poor fine motor control generates unnatural pressure on certain joints, leading to additional microtrauma.

Neurovascular theory

A.C.Brower theory Postulates that neurologic changes produced by

an underlying medical disorder create a hypervascular region in the subchondral bone that is characterized by increased osteoclastic resorption and osteoporosis.

More recent theories implicate the role of inflammatory cytokines such as TNF-α and IL-1 in the pathogenesis of Charcot neuroarthropathy.

On the molecular level, these factors lead to increased expression of nuclear transcription factor-κB, which in turn stimulates osteoclast formation.

Joint destruction in the neuropathic joint is probably brought on by a combination of factors that include damage to the nociceptors of the joint and the periarticular tissues.

The activity of peptides such as substance P, calcium gene related peptide, and vasoactive intestinal peptide (VIP) could result in increased vascularity and inflammation, contributing to further joint destruction.

Substance P can enhance the cellular synthesis of collagenase and prostaglandin-E; activate T lymphocytes, monocytes, and neutrophils; and take an active part in inflammation

The initial pathologic changes occur in the underlying bone and cartilage. Recurrent effusions occur due to hyperplasia of the synovium.

The articular cartilage is slowly destroyed by a pannus, which helps distinguish Charcot's joints from other forms of osteoarthritis.

Gough et al concluded that…..

The serum carboxyterminal telopeptide of type 1 collagen, a marker of osteoclastic bone resorption, had significantly increased levels in the acute Charcot foot.

The lack of an associated increase in osteoblastic activity supports the idea that excess osteoclast activity is a feature of the early stages of Charcot's neuroarthropathy

Clinical History

A careful history may reveal an unrecognized traumatic event.

Charcot neuroarthropathy most frequently presents in the fifth decade, after an average duration of diabetes of 20 to 24 years; in those with type 2 diabetes.

Presentation

DEPENDS OF DURATION OF DISEASE Mild swelling w/o deformity-Moderate

deformity with extreme swelling. Signs of inflammation. Profound unilateral swelling. WBC and

ESR may be normal

Increase in localized temp Erythema, Joint effusion. 75% pt. have pain. The deep tendon reflexes

at the knee are absent in a majority of patients.

Acute Charcot neuropathy

On Examination

Marked Irregularities identified as bony projections.

Bone formation in soft tissues.

Bag of Bones:

Joint can be passively and painlessly moved in all Directions

Diagnosis

Xrays. Indium-111 WBC scan. Gallium scan. USG MRI Radionuclide scans

Lab Studies

Inflammatory markersESR and WBC– elevated in both infection and Charcot arthropathy

Serum albumin >3.0g/dL

IMAGING Early Changes similar to OA Nontraumatic dislocations may be an early

sign. LaterRadiographic evidence of joint

distention caused by fluid, hypertrophic synovitis, osteophytes, and subluxation.

The normal architecture of the joint is lost, with dislocation, fragmentation, attempted repair by osteophytes, and sclerosis

Atrophic Stage:

Rapid joint destruction Loose bodies Subchondral bone erosions Subluxation Pathological#

Hypertrophic Stage

Reduced jt space. Subchondral bone sclerosis Pathological # healing with callus Multiple osteophyte formation with exoxtosis

formation. Dislocations of joints

Radiographic features6D’s Yochum and Rowe

Dense bones (subchondral sclerosis) Degeneration  Destruction of articular cartilage Deformity (pencil-point deformity of

metatarsal heads) Debris (loose bodies) Dislocation

Commonly Affected Joints

Foot Involvment Knee involvement Hip involvement Shoulder Elbow

Anatomic Classification (Sanders and Frykberg, 1991)

I - forefoot, 10-30% II - Lisfranc’s joint, most

common III - midtarsal joint, often

including naviculocuneiform joint IV - ankle and subtalar joints, 8-

10% V - (“posterior pillar”) fractures

of calcaneus, 2%

Classification ( Brodsky and Rouse)

Type 1 Midfoot

Type 2 Hindfoot

Type 3a Ankle

3b Calcis tubercleType4 Combination

Type 5 Forefoot

Neuropathic Joints

Hypertophic or

Productive

Hypertophic or

Productive

MIXED

Atrophic or

Resorptive

Brailsford

Stage of Hydrasthrosis:Distension of joint by serosanguinous effusion

Stage of atrophy:Destruction of affected articular cartilage and then the bone

Stage of hypertrophy:Massive hyperrophy of bone at periphery of articular cartilage

Radiographic Staging (Eichenholtz, 1966)

I Developmental (acute) stage

II Coalescence (quiescent) stage

III Consolidation (resolution) stage

Modified Eichenholtz Classification for the Progression of Charcot Neuroarthropathy

Stage 0(Shibata and Schon)

Swelling and erythema

No Radiographic Changes

Eichenholtz Classification

Stage I - Developmental (acute)

– Hyperemia due to autonomic neuropathy weakens bone and ligaments

– Diffuse swelling, joint laxity, subluxation, frank dislocation, fine periarticular fragmentation, debris formation

Radiographs

Stage I

Charcot Neuroarthropathy

Eichenholtz Classification

Stage II - Coalescence (quiescent)

– Absorption of osseous debris, fusion of larger fragments

– Dramatic sclerosis– Joints become less mobile and more stable– Aka the “hypertrophic”, or “subacute” phase of

Charcot

Radiographs

Stage II

Radiographs

Stage II

Eichenholtz Classification

Stage III - Consolidation (resolution)

– Osseous remodeling – for clinical purposes, stage I is regarded as the

acute phase, while stages II and III are regarded as the chronic or quiescent phase

Radiographs

Stage III

Charcot Arthropathy

HIP

Charcot neuroarthropathy in the hip is rare. Painless and Functional: no treatment Try conservative management 50% of fractures of the femoral neck in

diabetics developed Charcot's joints.

KNEE

Most Commonly secondary to Syphilis. Results in Gross Instability If only one knee is involved and destruction

is severe, fusion is indicated.

Total knee arthroplasty ???

Shoulder

Treatment

Primarily nonoperative. Consists of Acute and Postacute phases.

– Acute– Casting along with crutches and walkers.

– Postacute– Include bracing, ankle-foot orthotics(AFO),

specialized shoes.

Treatment

Casting- changed every 1-2weeks, if ulcerations are present changed every week for wound care, duration from 3-6 months.

Shoes, bracing, and orthotics- duration from 6-24 months.

Typical total healing time 1-2 years.

Early stage

Total Contact cast.

CROW boots

Surgical options

Arthrodesis Exostosectomy of bony prominences Osteotomies Reconstructive Surgeries Autologous bone Grafting Amputations

Surgical treatmentAnkle:

Arthrodesis of ankle to place the foot Plantigrade.

IM nail/Charnley/Ilizarov External Fixators

Average time for Fusion:20 months(IM nail).

Talus -- fragmented and avascular--talectomy and tibiocalcaneal arthrodesis.

Internal or External Fixation??

Hindfoot neuroarthropathy

Mainstay of Treatment is NONSURGICAL.

Arthrodesis indicated for… Hindfoot valgus with subluxation of the

subtalar joint or midtarsals to prevent ulceration and infection.

Principles outlined by Papa et al.

Careful removal of cartilage and debris.Thorough removal of sclerotic bone.Adequate fashioning of congruent bone surfaces for apposition.Rigid fixation of the arthrodesis site.Complete resection of fibrotic capsular tissue and synovium

Complication

Ulcers Osteomyelitis Gross Deformity of the foot Gangrene.

TH

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With the Lisfranc pattern, breakdown initially occurs along the medial column, with late changes progressing to the lateral column. The arch abducts and prominences develop, leading to deformity, fullness, and ulceration. The naviculocuneiform pattern leads to collapse at the naviculocuneiform joint, with development of a lateral rocker-bottom deformity. The perinavicular pattern is caused by osteonecrosis or fracture of the navicular. The lateral arch height decreases, leading to a lateral rocker and shortening of the medial column. Eventually this breakdown progresses to the central aspect of the foot, with severe plantar flexion of the talus and eventual ulceration. The transverse tarsal pattern is caused by lateral subluxation of the navicular on the talus and abduction of the foot with a valgus calcaneus. Calcaneal pitch eventually decreases, and a central rocker develops at the calcaneocuboid joint. In late stages, the talus is completely dislocated from the navicular, and ulceration develops at the calcaneocuboid interval. All four patterns eventually progress to a rocker-bottom deformity and chronic ulceration.