Charcot neuropathy.

Neuropathic
osteoarthropathy
Dr.Bahaa Ali Kornah
Prof. Of Orthopedic
Al-Azhar University
Cairo -Egypt
bahaa Ali Kornah-Al-Azhar Un. Cairo -EGYPT

Dr. Bahaa Ali Kornah
Prof.. Of Orthopedic
Al-Azhar University
Cairo - Egypt
Bahaa Kornah. Al-Azhar Un. Cairo EGYPT
‫وبركاته‬ ‫هللا‬ ‫ورحمة‬ ‫عليكم‬ ‫السالم‬

Definition
1. Neuropathic arthropathy ,
2. Charcot joint
3. neuropathic osteoarthropathy,>>>>> A chronic
and progressive joint disease following loss of
protective sensation leads to destruction of joints,
pathologic fractures, surrounding bony structures
and debilitating deformities may lead to
amputation if left untreated

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.
❖ Steindler, Fleming Moller, Fried, and Floyd claim
that Mitchell J. K. of Philadelphia was the first to
report neuropathic joints in 183 1.
❖ 1868 Jean-Martin Charcot gave the first detailed
description of this disease.
❖ In 1892, Sokoloff --upper extremity with
syringomyelia.

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

❖ Volkman and Virchow mechanical theory 1886
❖ Eloesser in 1917. He assessed the role of trauma in
the development of neuropathic joints.
❖ 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.
❖ Chandler and Wright in 1958. Associated with
intra-articular corticosteroid injections
❖ Brower A.C —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.

Epidemiology
• incidence
– 0.1-1.4% of patients with diabetes
– 7.5% of patients with diabetes and neuropathy
• demographics
– age bracket
• type 1 diabetes
– typically presents in 5th decade (20-25 years following diagnosis)
• type 2 diabetes
– typically presents in 6th decade (5-10 years following diagnosis)

• foot and ankle (diabetic Charcot foot)
• 9-35% have bilateral disease

• Neuroarthropathy among all pts with tabes
dorsalis ranges b/w 5 to 10%
•75% lower
extremities
25% upper
extremities.

Risk factors
•diabetic neuropathy
•alcoholism
•leprosy
•myelomeningocele
•tabes dorsalis/syphilis
•syringomyelia

Mechanism and pathophysiology
• Major theories
– Neurotraumatic theory
– Neurovascular theory
– Most probably both

Neurotraumatic Theory
• Loss of deep sensation leads to repetitive micro
trauma to the joint
• insensate joints subjected to repetitive
microtrauma
• body unable to adopt protective mechanisms to
compensate for microtrauma due to abnormal
sensation

Neurovascular theory
• Neurovascular autonomic dysfunction
increases blood flow through AV shunting
• leads to bone resorption and weakening by
increased osteoclastic resorption and
osteoporosis.

Molecular biology
•inflammatory cytokines may cause destruction
•IL-1 and TNF-alpha lead to increased
production of
•transcription factor-kB
•RANK/RANKL/OPG triad pathway
•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
❖Symptoms swollen foot and ankle
❖pain in 50%, painless in 50%
❖loss of function
• Mild swelling w/o deformity-Moderate
deformity with extreme swelling.
• Signs of inflammation.
WBC and
ESR may
be normal

acute Charcot neuropathy
•inspection
•swollen
•warm
•average of 3.3 degrees C warmer
than contralateral side
•erythema
•often confused with infection
•erythema will decrease with
elevation in Charcot arthropathy,
but is unchanged in infection
• Joint effusion.

Acute Charcot neuropathy

chronic Charcot neuropathy
•inspection
•structurally deformed foot
•bony prominences
•rocker bottom deformity
•collapse of medial arch
•motion may be ligamentously unstable,
Joint can be passively and painlessly
moved in all Directions
•neurovascular
•Semmes Weinstein 5.07(10g) Sensory
Testing Monofilaments

On Examination
Marked Irregularities identified as bony
projections.
Bone formation in soft tissues. Bag of Bones:
•50% pt. have pain.
•The deep tendon reflexes at the knee are
absent in a majority of patients.

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

•Laboratory
•inflammatory markers
•ESR and WBC
•elevated in both infection and Charcot arthropathy
•wound healing levels
•absolute lymphocyte count >1500/mm3
•serum albumin >3.0g/dL
•Biopsy
•may be used to guide antibiotic therapy in cases of associated
osteomyelitis or soft tissue abscess
•Histology
•synovial hypertrophy
•detritic synovitis (cartilage and bone distributed in
synovium)

IMAGING
• Early Changes similar to OA
• Nontraumatic dislocations may be an early
sign.
• obliteration of joint space
• fragmentation of both articular surfaces of a joint
leading to subluxation or dislocation
• scattered "chunks" of bone in fibrous tissue
• surrounding soft tissue edema
• joint distension by fluid
• heterotopic ossification

• 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 features
6D’s Yochum and Rowe
• Dense bones (subchondral sclerosis)
• Degeneration
• Destruction of articular cartilage
• Deformity (pencil-point deformity of
metatarsal heads)
• Debris (loose bodies)
• Dislocation

•Bone scan indications
• useful to help determine presence of
superimposed osteomyelitis
•type of study
• technetium bone scan
• may be positive for a neuropathic joint
and osteomyelitis
• indium WBC scan
• negative (cold) for neuropathic joints and
positive (hot) for osteomyelitis

MRI
•indications
•best for differentiating
abscess from soft-tissue
swelling
•most sensitive in
diagnosing soft tissue
and/or osteomyelitis
•limitations
•difficult to differentiate
infection from Charcot
arthropathy on MRI

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 3aAnkle
❖ 3b Calcis tubercle
❖ Type4 Combination
❖ Type 5 Forefoot

Neuropathic Joints
Hypertrophic 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

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

Charcot Neuroarthropathy
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

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

Modified Eichenholtz Classification for
the Progression of Charcot
Neuroarthropathy

Stage 0(Shibata and Schon)
• Swelling and erythema
• No Radiographic
Changes

Charcot Arthropathy

• PREVENTION
• TREAMENT THE PRIMARY CAUSES
• TREAMENT OF NEUROPATHIC PAIN

• Every six minutes, somewhere in the United
States, someone loses a limb due to
amputation because of peripheral neuropathy.
• Most foot problems that people with diabetes
face arise from two serious complications of
the disease: nerve damage and poor
circulation. The most effective treatment,
however, is prevention.(AAOS)

• Neuropathy is a universal feature of the
affected limb

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.

Nonoperative
•total contact casting, shoewear modifications, medications
•indications
•first line of treatment
•technique
•contact casting
•casts changed every 1-2 weeks for 3-4 months
•orthotics
•Charcot restraint orthotic walker (CROW) boot can be used
after contact casting
•shoe modifications
•in Eichenholtz stage 3 double rocker shoe modifications will
best reduce risk for ulceration at the plantar apex of the
deformity

Nonoperative
•total contact casting, shoewear modifications,
medications
•medications
•bisphosphonates
•neuropathic pain medications
•antidepressants
•topical anesthetics
•outcomes
•75% success rate

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

Operative
resection of bony prominences
(exostectomy) and TAL
•indications
•"braceable" foot with equinus
deformity and focal bony
prominences causing skin breakdown
•technique
•goal is to achieve plantigrade foot
that allows ambulation without skin
compromise

Operative
•deformity correction, arthrodesis +/-
osteotomies
•indications
•severe deformity that is not
"braceable"
•outcomes
•very high complication rate (up to
70%)

Surgical treatment
Ankle:
• Arthrodesis of ankle to place the foot
Plantigrade.
•fixation techniques
•internal fixation
•screw, pins, plates, tibiocalcaneal nail
•external fixation
•used when bone quality is poor or soft
tissues are compromised
• Average time for Fusion:20 months(IM
nail).
• Talus -- fragmented and avascular--
talectomy and tibiocalcaneal arthrodesis.

Operative
•deformity correction, arthrodesis +/-
osteotomies
•indications
•severe deformity that is not "braceable"
•outcomes
•very high complication rate (up to 70%)
•amputations
•indications
•failed previous surgery (unstable
arthrodesis)
•recurrent infection
•technique
•goal is for a partial or limited
amputation if vascularity allows

Internal
or External Fixation??

Case presentation
female DM –Charcot joint

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.

Surgical 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.

Take Home Message
• High degree of suspicion to diagnose acute Charcot
arthropathy.
• Take the diabetic foot seriously
• Prevention is better treatment of causes neuropathic pain.
• MANAGEMENT OF THE DIABETIC FOOT is Team Approach
• Ensure referrals are timely and appropriate
• ALL PATIENTS WITH DIABETIC FOOT ULCERS SHOULD
BE REFERRED ON FOR SPECIALIST CARE
• Immobilization
• Bisphosphonate.
• Customized Foot Wear

Bahaa Ali Kornah
bkornah@gmail.com
‫د‬/‫قرنة‬ ‫بهاء‬bahaa Ali Kornah-Al-Azhar Un. Cairo -EGYPT

Charcot neuropathy.

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