4/5/2020 RP's Ortho Notes – Passion for excellence and perfection…..

RP's Ortho Notes

Passion for excellence and perfection…..

TOPICS July 31, 2019

Charcot Osteoarthropathy

(h ps://learningorthopaedics.com/2019/07/31/charcot-osteoarthropathy/)
Charcot neuropathic osteoarthropathy (CNO) is a noninfective, inflammatory condition affecting
periarticular soft tissue and bone in patients with peripheral neuropathy which if not properly
treated may lead to progressive resorption of bone, disruption of soft tissues and disorganization
of joints resulting in permanent deformity, altered biomechanics, predisposition to skin
ulceration, infection and osteomyelitis.
It most commonly affects the foot and ankle region.
In the early stages there are local inflammatory changes followed by progressive bone loss, tissue
disruption, joint dislocation and development of deformities.
The deformities lead to abnormal loading pa erns, skin break down, infection and ultimately
result in osteomyelitis.
Most common cause is diabetic peripheral neuropathy.
Lifetime prevalence of CNO in diabetic patients is 0.1-10% which increases to 29-35% if there is
peripheral neuropathy.
The prevalence in diabetics, depend upon the diagnostic method, with MRI showing positive
findings in up to 75% and x-ray findings in 30%.
28% mortality rate has been reported within 5 years of diagnosis (Sohn 2009).
In the early phase, differentiation from acute osteomyelitis is difficult.
Natural history (Sal man 2005)
Risk of amputation increased 15-40 fold.
2.7% annual amputation rate.
40% chance of ulceration.
28% mortality within 5 years of diagnosis (Sohn 2009).

History

1703 – Musgrave described CNO as an arthralgia caused by venereal disease.

1831 – JK Mitchell described the relationship with spinal lesion.

1868 – Jean Martin Charcot described the neuropathic aspect.

1881 – JM Charcot at the 7th International Medical Congress described the association with tabes
dorsalis.

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1936 – WR Jordan described CNO in association with diabetes mellitus.

Pathogenesis

Development of CNO is due to interplay between several pathways leading to dysregulation

of bone formation and resorption, persistent inflammatory response, increased glycation of
collagen and accumulation of advanced glycation end products (AGLEPs) in the tissues.
In genetically predisposed individuals with peripheral neuropathy, decreased neuropeptides such
as nitrous oxide and calcitonin gene related peptide leads to increased levels of receptor activator
nuclear factor kappa beta ligand (RANKL). Increased RANKL potentiates osteoclastogenesis
resulting in uncoupling of bone formation and resorption.
3 theories –
Neuro-traumatic theory – Damage to sensory feedback results in repeated trauma. Repeated
trauma leads to increased proinflammatory cytokines such as interleukin-1β, interleukin-6,
tumour necrosis factor α which causes bone resorption.
Neurovascular theory – Due to changes in vascularity caused by dysregulation of vasomotor
and trophic nerve supply.
Neuro-inflammatory theory – Abnormal persistence of inflammatory response and inability to
terminate the inflammatory response are thought to be important in the pathogenesis.
Unregulated inflammatory process triggers increased expression of receptor activator of
nuclear kappa ligand (RANKL) in susceptible individuals. RANKL increases production of
nuclear factor kappa beta (NF-κβ) which stimulates maturation of osteoclast precursor cells to
osteoclasts. RANKL also stimulates synthesis of osteoprotogerin (OPG) by the osteoblasts. The
decreased secretion of calcitonin gene related peptide (CGRP) which is an antagonist of
RANKL by the damaged nerve endings is also theorized as a cause. Dysfunction of Wnt/
βcatenin pathway which regulate bone and vascular metabolism is also proposed as a cause.
Increased RANKL expression is thought to be mediated by advanced glycation end products
(AGEs), reactive oxygen species and oxidized lipids. Increased AGEs in diabetes is due to
hyperglycemia as well as increased oxidative stress. Increased blood glucose and decreased
circulating receptor for AGEs leads to nonenzymatic glycation of collagen and accumulation of
AGEs in the tissues. AGEs induce apoptosis in the mesenchymal cells and hence may affect the
mechanical parameters of type I collagen.
Causes of CNO
Diabetes mellitus
Leprosy
Peripheral neuropathy
Syringomyelia
Poliomyelitis
Multiple sclerosis
Tabes dorsalis
Toxins
Rheumatoid arthritis

Clinical features

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Physical findings may be neurological, musculoskeletal and vascular abnormalities.

The onset may be following a triggering event, which may be trauma, surgery or infection.
Clinical findings depend on the stage of disease.
There are 3 stages clinico-radiologically.
Dissolution stage
Coalescence stage
Resolution stage
Patients present with acute onset unilateral swelling of foot and ankle which may extend up to the
knee.
Pain is absent in 50% of patients. (Brodsky 1993)
Some patients may complain of mild pain or discomfort.
Initial stages show marked inflammation evidenced by erythema, edema, warmth and more than
20C temperature difference when compared to opposite side.
Skin temperature measurement using surface temperature sensing devices such as infrared
thermometer is useful in assessing severity of inflammation due to neuropathy.
Erythema due to CNO will dissipate if the limb is elevated above the level of heart for 10-15
minutes, while erythema due to infection will not.
During the coalescence stage, swelling and inflammation begins to dissipate but deformities start
developing.
During the resolution stage, the signs of inflammation resolves completely and deformities
persist.
Deformities lead to marked alteration of load bearing pa ern of the sole of foot predisposing the
high pressure areas to ulceration.
Ulceration lead to infection which may progress to deep infection and osteomyelitis.
Deformities affect the forefoot, midfoot, hindfoot or the ankle.
Forefoot deformity may involve the first metatarsophalangeal joint in the form of dorsal or plantar
dislocation.
Mid foot is affected in more than 60% of patients. Patients may develop abduction or adduction
deformity at the Lisfranc joint or plantar dislocation of the tarsometatarsal joint leading to
classical rocker bo um foot.
In the ankle, equinus or calcaneus deformity may develop.
Sagi al instability of foot assessed by Assal and Stern method. The ankle is locked by dorsiflexion,
pressure on the forefoot demonstrates instability leading to collapse of longitudinal arch.
Contracture of the tendoachilles leads to plantar flexion of the calcaneus and midfoot collapse
leads to rocker bo om foot with dorsiflexion of forefoot.
In severe cases the joints may be dislocated and unstable.

Diagnosis

Diagnosis needs establishment of the presence of peripheral sensory neuropathy with reduced
pain perception and establishment of arthropathy by clinical findings and imaging studies,
Peripheral neuropathy diagnosed by
Decreased reflexes, reduced vibration sense and weakness
Decreased sensation on Semmes-Weinstein monofilament examination of sensation.
Pinprick sensation
Neurometer test
Electrophysiological studies
Diagnosis of osteomyelitis done by
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