J Shoulder Elbow Surg (2017) 26, 544–552

www.elsevier.com/locate/ymse

REVIEW ARTICLE

Charcot shoulder and elbow: a review of the

literature and update on treatment
Mark C. Snoddy, MD*, Donald H. Lee, MD, John E. Kuhn, MD

Department of Orthopaedic Surgery, Vanderbilt University Medical Center, Nashville, TN, USA

Charcot arthropathy of the shoulder and elbow is a rare disease process initially described in the 1700s;
however, it was not until the 19th century that physicians understood its association with other disease
processes such as cervical spine pathology and diabetes. A primary complaint is painful or painless joint
dysfunction, meaning the orthopedic surgeon is regularly the first physician to evaluate the patient. Fre-
quently, the condition of these patients is misdiagnosed. Although the pathogenesis of the disease is
controversial, the etiology is commonly due to syringomyelia. The key to successful management is a thor-
ough history and examination along with a workup including specific laboratory testing and imaging to
rule out other disease processes. Most neuropathic shoulders and elbows have historically been managed
conservatively because of poor outcomes with operative interventions. Newer data have emerged hinting
that early neurosurgical intervention can stabilize this degenerative process. If clinical and radiographic
stabilization occurs, recent studies have outlined surgical indications that can provide surgeons with a guide
as to patients in whom successful operative outcomes can be achieved in the face of failed conservative
management.
Level of evidence: Narrative Review
© 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. All rights reserved.
Keywords: Charcot; shoulder; elbow; syrinx; syringomyelia; neuropathic arthropathy

Syringomyelia is the leading cause of neuropathic (Charcot)
arthropathy of the upper extremity, with the shoulder and elbow
being the most commonly affected joints. Although neuro-
logic deficits are occult and easily overlooked, the primary
complaints of most patients are symptoms of neuropathic ar-
thropathy, specifically painless joint swelling.44 Thus, the
orthopedic surgeon is often the first physician to evaluate the
patient with syringomyelia and a Charcot shoulder and/or
elbow. Early diagnosis and proper management of the joint
and neurologic cause are critical. Referral to the neurosurgery
Institutional review board or ethical committee approval was not applica-
ble to this study.
*Reprint requests: Mark C. Snoddy, MD, Medical Center East, Vanderbilt
Orthopaedic Institute, Ste 4200, Nashville, TN 37232, USA.
E-mail address: markcsnoddy@gmail.com (M.C. Snoddy).
department and conservative arthropathy management were
traditionally the mainstay of treatment, as surgical treat-
ments produced unfavorable results. Newer literature has
advocated surgical management of the Charcot shoulder and
elbow and has reported early successful outcomes.
History
The first description of neuropathic arthropathy was by
Musgrave in 1703, when he described the swollen, in-
flamed joints of a patient who was left “flaccid by paralysis.”
In 1831, Mitchell reported on a patient with spinal cord pa-
ralysis due to tuberculosis and noted “bizarre” joint changes.
During the latter part of the 19th century, Jean-Marie Charcot
reviewed patients with tabes dorsalis and provided a detailed

1058-2746/$ - see front matter © 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. All rights reserved.
http://dx.doi.org/10.1016/j.jse.2016.10.015
Charcot shoulder and elbow
description of the rapid development of joint deterioration and
instability.2 It was Sokoloff in 1892 who described the as-
sociation of neuropathic joints of the upper extremity with
syringomyelia. In the mid 1900s, neuropathic changes were
described in association with diabetes and intra-articular cor-
ticosteroid injections.8,24
Etiology
Neuropathic arthropathy typically occurs due to syringomy-
elia but has also been associated with diabetes mellitus, tabes
dorsalis, chronic alcoholism, end-stage renal disease, gigan-
tism, intra-articular steroid injections, peripheral neuropathy,
meningomyelocele, multiple sclerosis, myelodysplasia, leprosy,
amyloidosis, and congenital insensitivity to pain.2,52 Neuro-
pathic arthropathy will develop in 25% of patients with
syringomyelia, with 80% of these cases occurring in the upper
extremity.2,21 Although a few reports have indicated that the
elbow is more commonly affected than the shoulder, most
case reports have described the shoulder as the most com-
monly affected joint of the upper extremity.4,20,25,43 Case reports
of neuropathic arthropathy of the wrist and interphalangeal
joints are rare.12 Twenty percent of patients have multiple joints
involved.2 Cervical syringomyelia is the etiology of shoul-
der neuropathic arthropathy in 75% of cases and is the most
common cause of elbow neuroarthropathy.25
Pathogenesis
Syringomyelia is a chronic and slowly progressive spinal cord
disease with a fluid-containing cavity (syrinx) inside the
spinal cord. The disease can be congenital or may result
from infection, trauma, tumor, vascular abnormalities, or
degeneration.10,14 The decussating fibers of the lateral spino-
thalamic tract that harbor nerve fibers for pain and temperature
sensation are the first structures to be damaged by a syrinx.
This leads to abnormal innervation of the affected joint or
joints10,23; as a result, a condition called dissociative anes-
thesia occurs, in which proprioception and motor function
are preserved while pain and temperature senses are lost.10,49
As the syrinx enlarges, the damage to the dorsal column and
anterior horn will produce areflexia, loss of muscle strength,
and atrophy. The eventual joint destruction can occur early
or late in the disease process.43
There are 3 phases of the neuropathic joint. In the de-
structive phase, the joint is hyperemic and swollen and there
is osteoclastic bone resorption associated with repetitive
trauma. The reparative phase follows with the formation of
dense fibrous tissue and coalescence of the debris. Finally,
the quiescent phase is characterized by decreased vascular-
ity and osseous sclerosis.16
The pathogenesis of syringomyelia and neuropathic ar-
thropathy is not fully understood, and many theories have
been proposed. Mitchell and Charcot hypothesized in the 19th
545
century that damage to the central nervous system trophic
centers disrupts bone and joint nutrition and causes osteolysis.
Volkmann and Virchow theorized that after a loss in pain sense,
joint destruction was caused by years of subclinical trauma.
More recently, neurovascular and neurotraumatic theories for
the development of neuropathic arthropathy were developed.4
According to the neurovascular theory, sensory loss
disrupts normal neurovascular reflexes at the joints. The re-
sulting hyperemia and activation of osteoclasts cause bone
resorption.7,52 The neurotraumatic theory involves loss of
somatic muscle reflexes, which prevents protective proprio-
ception. This leads to recurrent, unnoticed microtrauma,
causing joint destruction from extremes of joint motion. Not
all reported cases have a history of trauma, as neuropathic
arthropathy is also seen in paraplegic, bedridden patients.
Therefore, the most widely accepted current theory is that os-
teolysis starts because of neurovascularity processes and
continues because of neurotraumatic processes.7,22,52
In 1997, Gough et al19 proposed a mechanism of inflam-
mation leading to excess osteoclastogenesis. This is supported
by an elevation in blood marker levels showing osteoclast ac-
tivity (tumor necrosis factor α, IL-6) in patients with acute
Charcot arthropathy and an elevation in proinflammatory
cytokine levels in bone samples from surgery.6,39
Ultimately, there is no widely accepted theory as to why
syringomyelia causes neuropathic arthropathy. A large question
remains as to why this disease process tends to occur uni-
laterally or is monoarticular. Although the syrinx does involve
both halves of the cord, usually only one side of the body is
affected. Multiple studies have reviewed the cervical magnetic
resonance imaging (MRI) scans of the syrinx and confirmed
that it is often slightly asymmetrical, with every patient having
neuropathic arthropathy on the affected side.1,12,27,37
Diagnosis
The clinical findings in neuropathic shoulder arthropathy
are widely variable. Usually, joint symptoms manifest earlier
than neurologic symptoms, with patients presenting initial-
ly to orthopedic clinics.21 Patients may present with painful
or painless joints, joint instability, swelling, and dysfunc-
tion with or without a history of trauma. Most patients with
a neuropathic elbow present with elbow instability and/or
subluxation.11,27,53 Physical examination reveals joint laxity,
crepitus, effusion, and often, decreased muscle strength.2 On
examination, sensory and temperature changes are often re-
vealed, most notably along the patient’s back and arms in a
cape-like distribution. Also noted are a loss of hot or cold hand
sensation and the presence of asymmetrical reflexes.12,23,52
To firmly diagnose a syrinx and neuropathic arthropathy,
other conditions must be ruled out. The differential includes
septic arthritis, arthritis, synovial chondromatosis, soft-
tissue sarcoma, tumoral calcinosis, idiopathic osteolysis,
Winchester syndrome, Gorham disease, trauma, and blood
clot.4,15,25 Another condition in the differential is Milwaukee
546
shoulder syndrome, which is a rapid destruction of the rotator
cuff and glenohumeral joint with intra-articular or periar-
ticular hydroxyapatite crystals.18,34
Neuropathic arthropathy is seen by some clinicians as a
diagnosis of exclusion. Therefore, other more prevalent con-
ditions must be ruled out including diabetes, tabes dorsalis,
infection, neoplasm, and inflammatory arthropathy. Radio-
graphs demonstrate severe joint deterioration with evident bone
loss that may resemble septic arthritis or neoplasia; biopsy
may be required to rule out these diagnoses.44 One clinical
pearl is that a patient’s pain is less than what would be ex-
pected based on imaging studies in patients with neuropathic
arthropathy.17 The serologic evaluation includes complete blood
count, hemoglobin A1c, rapid plasma reagin, erythrocyte sed-
imentation rate, c-reactive protein, rheumatoid factor, and
antinuclear antibody. White blood cell count and erythro-
cyte sedimentation rate are normal in most cases of neuropathic
arthropathy.2 If a joint aspiration is performed, a neuro-
pathic joint will contain a large quantity of clear yellow fluid
with particulate debris.2
In patients with elbow neuroarthropathy, ulnar and pos-
terior interosseous nerve compression can develop about
the elbow. Electromyography and nerve conduction studies
can be used to diagnose acute compression caused by het-
erotopic ossification or joint pathology in addition to the
polyneuropathy caused by a syrinx.3,11
Imaging
Radiographs
Radiographic findings in a neuropathic joint include sclero-
sis, joint destruction, new bone formation, bony fragmentation,
dislocation, subluxation, osteophytes, osseous debris, effu-
sion, heterotopic ossification, disorganization, and periarticular
soft-tissue swelling.27,35 The various joints affected are pre-
disposed to both atrophic and hypertrophic reactions. An
atrophic reaction, described as massive bone resorption with
disintegration of the joint, most commonly occurs in the hip,
shoulder, and foot. Specific radiographic findings in the shoul-
der include superomedial flattening of the humeral head,
periarticular soft-tissue calcification, and glenoid sclerosis.44
Hypertrophic changes such as severe joint destruction,
osteophytes, fractures, osseous debris, and periarticular new
bone formation are found more in the elbow.11
Computed tomography
Computed tomography (CT) scans have no significant role
in the diagnosis of neuropathic arthropathy. However, these
scans may be helpful in evaluating intraosseous gas, corti-
cal destruction, and sequestra. Furthermore, destruction,
deformity, debris, degeneration, and dislocation are more
evident on advanced imaging such as CT scans.30
M.C. Snoddy et al.
Magnetic resonance imaging
MRI is rarely needed to confirm the diagnosis. However, char-
acteristic MRI changes include edema of the surrounding
muscles, bony destruction, and large joint effusions.4
A cervical MRI study should be ordered to rule out
cervical spine pathology. Syringomyelia is the most common
underlying disease.4 The anatomy and size of the syrinx
can be identified using T1-weighted images. The syrinx has
signal intensity equal to or slightly higher than cerebrospi-
nal fluid. With T2-weighted images, pathologic tissue, as
well as cerebrospinal fluid dynamics within a cavity, is
better identified.46 The use of contrast-enhanced versus
non–contrast-enhanced MRI study is debatable. Older studies
recommended using contrast whereas newer studies have
suggested that non–contrast-enhanced imaging with sagittal
and T2 images is sufficient to distinguish a syrinx from a
spinal cord tumor.13,48 A CT myelogram can be obtained if
patients are unable to undergo an MRI study and advanced
imaging is desired.47
Treatment and management
The goals in caring for a patient with neuropathic arthropa-
thy are early diagnosis, treatment of the offending agent or
agents, and maintenance of joint and extremity function. Treat-
ing the underlying disease process will help slow disease
progression and maximize joint function.43 In many cases this
means immediate referral to the neurosurgery department for
syrinx evaluation and possibly to the endocrinology depart-
ment for diabetes management.
As reported in a few case series, a moderate number
of patients have achieved superior results and baseline im-
provement in their arthropathies from neurosurgical
intervention alone.4,20,29,31 Recent literature has shown that
after syrinx decompression, patients can have improvement
of neurologic symptoms, slowing of joint deterioration, and
improvement of bone quality.4,20 Makihara et al29 reviewed
a single patient with a Charcot shoulder who underwent
suboccipital decompression for syringomyelia and showed
improved proximal muscle strength and sensory changes,
decreased hydrarthrosis, and remarkably, bone regrowth
that over time resembled a new articular surface. Deng et
al12 compared 12 patients who had a syrinx and neuropathic
shoulders. Five of the patients underwent neurosurgical de-
compression, and all 5 showed neurologic improvement
without joint deterioration over a period of 30 months. The
remaining 7 patients refused neurosurgical intervention, and
5 of these patients had worsening joint destruction and
neurologic decline. Atalar et al4 reviewed 5 patients with
syringomyelia and neuropathic arthropathy of the shoulder.
Four patients were managed with neurosurgical decompres-
sion and conservative treatment of the neuropathic joint,
whereas one did not undergo syrinx decompression. All
patients undergoing neurosurgical decompression showed
Charcot shoulder and elbow 547

A B

C D E
Figure 1 (A) A 42-year-old right hand–dominant male patient with no medical history presented for evaluation of left elbow swelling for
2 months. He was noted to have first dorsal interosseous atrophy and ulnar-side hand numbness. Treatment and workup at an outside hos-
pital included ulnar nerve decompression, a bone scan, elbow magnetic resonance imaging, elbow aspiration, antibiotics, and pain medication.
Elbow motion was 0° to 130° with 75° of pronation and 85° of supination bilaterally. Positive Froment and Wartenberg signs were noted
with diminished sensation in the ulnar 2 digits. (B, C) Radiographs obtained in the clinic showed evidence of joint destruction with areas of
heterotopic bone suspicious for a Charcot elbow. (D, E) Magnetic resonance imaging was obtained and confirmed suspicion of a cervical
syrinx involving the cervical and upper thoracic cord. The patient was managed in a hinged elbow brace and was deemed not to be a sur-
gical candidate for arthroplasty.

improved range of motion and function of the neuropathic
joint. Atalar et al concluded that all patients should be
referred to the neurosurgery department for syrinx decom-
pression. Three case examples are discussed in Figures 1,
2, and 3.
Nonoperative treatment of neuropathic joint
The main goal of orthopedic management in the neuro-
pathic joint is maintenance of function. Early recognition of
the disease, joint protection, and patient education are im-
perative. Patient education regarding the cause and prognosis
of the disease process is vital. For patients to play an active
role in their care, they must have an understanding of how
their disease affects their ability to sense injury and can lead
to joint destruction. Education will also help establish ap-
propriate expectations regarding treatments and outcomes.2
Regarding the management of the neuropathic shoulder,
restricted weight bearing, immobilization, and functional
bracing were historically the mainstay of treatment.2,23,33 To
prevent the progression of ligamentous and soft-tissue laxity,
aspiration followed by immobilization of an affected joint has
been used.43 More recently, physical therapy, passive stretch-
ing, range-of-motion exercises, and strengthening have been
used to reduce pain and swelling.21,31 In the elbow, authors
encourage mobilization, physical therapy, and functional
bracing.11,27 In neuropathic joints, nonsteroidal anti-inflammatory
medication can mitigate the synovial inflammatory process.45
Although not a widely accepted method of treatment,
alendronate has been used to treat foot arthropathy caused
548 M.C. Snoddy et al.

A B C

D E F

G H I
Figure 2 A 67-year-old right hand–dominant male patient with a medical history significant for spinal cord injury in the mid 1990s pre-
sented for evaluation of right shoulder pain. He had a history of 7 right shoulder operations, including total shoulder arthroplasty (dislocated
8 times), multiple irrigation and débridement procedures, revision hemiarthroplasty, reverse total shoulder arthroplasty, and revision reverse
total shoulder arthroplasty. Examination showed swelling involving the right shoulder (A, B) with bruising extending across the shoulder
and atrophy of the hand with the hand in the intrinsic-minus position (C, D). Range of motion was limited to forward flexion to 50°, ab-
duction to 80°, external rotation to −30°, and internal rotation to the hip. No sensory changes were noted. (E, F) Radiographs showed a
dislocated reverse total shoulder arthroplasty. (G) Magnetic resonance imaging was obtained and confirmed a cervical syrinx. (H, I) The
patient underwent resection arthroplasty and was lost to follow-up.

by diabetes. In a single study, 20 patients with newly diag- received total contact casting for 2 months and a pneumatic
nosed Charcot arthropathy of the foot were randomized to boot for 4 months. Bone density and markers of bone turn-
treatment with 70 mg of alendronate weekly or to a control over such as carboxy-terminal telopeptide of type I collagen
group and were followed up for 6 months.40 All patients and hydroxyproline were measured initially and at 6 months.
Charcot shoulder and elbow 549

A B

C D

E F
Figure 3 A 52-year-old female patient with a medical history significant for Chiari malformation and prior decompression, chronic ob-
structive pulmonary disorder, hemochromatosis, and lupus presented with left shoulder pain for 3 years. Examination showed swelling and
painless range of motion with forward flexion to 90°, external rotation to 15°, and internal rotation to the hip; significant instability of the
humeral head in the glenoid; weakness of the rotator cuff with internal and external rotation; and an abnormal sensation to light tough in
the hands. (A-D) Radiographs and computed tomography scans showed destruction of the glenohumeral joint with fragmentation. (E, F)
Magnetic resonance imaging showed effusion and joint destruction. Unfortunately, the patient’s shunt had clotted, and because of her mul-
tiple medical comorbidities, it could not be drained according to the neurosurgery department. As a result of the inability to decompress the
syrinx, only conservative management of the shoulder was offered.
550
There was a significant reduction in markers of bone turn-
over with improved bone density compared with the control
group. 40 A more recent meta-analysis on the use of
bisphosphonates in the Charcot foot also reported the reduc-
tion of bone turnover markers; however, it did not support
the use of bisphosphonates in Charcot neuroarthropathy
because of the potential for prolonging the resolution phase
and unknown long-term efficacy.42 No data exist regarding
bisphosphonate use for upper extremity Charcot arthropa-
thy. Regarding orthopedic management, nonoperative
modalities should be considered until stabilization of the syrinx
has occurred both clinically and radiographically.4
Surgical treatment of neuropathic joint
Before surgical treatment is considered for the involved joint,
the underlying disease should be treated. Furthermore, it should
be documented that the osteolytic process is not progress-
ing and that the active phase of the disease has ceased.2
Attempted joint reconstruction applied before knowledge of
the natural course of the disease is attained and before treat-
ment of the underlying disease is performed may lead to
catastrophic results.4
Shoulder
In the past, shoulder arthrodesis, hemiarthroplasty, and re-
surfacing operations attempted for the treatment of neuropathic
shoulder arthropathy have led to unsatisfactory results.9,26,38
At one point, reconstruction was thought to be contraindicated
because of high risks of dislocation, loosening, and implant
failure due to muscle weakness and sensory disturbance.26 With
neurosurgical decompression, Makihara et al29 showed there
is improved sensation, albeit not complete sensory normal-
ization, which can potentially provide enough protective
sensation to consider surgical management.
Historically, arthrodesis was the preferred management
strategy, yet there were high rates of failure including pseud-
arthrosis and infection, which led to other surgical options.2,14,32
Previously considered contraindicated as a treatment for
neuroarthropathy, arthroplasty has recently produced favor-
able outcomes for hip and knee neuroarthropathy.5,41 Crowther
and Bell9 presented the case report of a single patient treated
with shoulder resurfacing arthroplasty and soft-tissue bio-
logical resurfacing of the glenoid performed 7 years after syrinx
decompression. Before the arthroplasty, radiographs showed
no humeral head destruction. At 2 years’ follow-up, the pa-
tient’s range of motion was reasonable with forward flexion
to 140° and external rotation to 50°, with no change from the
initial postoperative radiographs. Crowther and Bell re-
ported this procedure was indicated in young patients with
adequate humeral head bone stock with neuropathic shoul-
ders. Resurfacing arthroplasty gives the added benefit of the
protection of existing humeral and glenoid bone for possi-
ble use in future procedures.9
M.C. Snoddy et al.
After initial neurosurgical decompression of the syrinx
cavity, Matsuhashi et al31 performed humeral head replace-
ment with rotator cuff repair in 3 patients with neuropathic
shoulders. After 8 years, patients had improved pain and
increased range of motion. Radiographs showed no signs
of loosening or failure, including no dislocation and no
medialization of the humeral head. Specific indications for
the procedure include absence of sepsis, no obvious limb
palsies, an intact humeral greater tuberosity, and the pa-
tient’s desire for restored shoulder function. By performing
the rotator cuff repair with the arthroplasty, the authors be-
lieved it provided improved head stability contributing to lower
complication rates.
Ueblacker et al50 performed bilateral reverse total shoul-
der arthroplasties, 4 months apart, in a patient with a post-
traumatic syrinx after conservative management had failed
and she had no rotator cuff function. Having incomplete para-
plegia at baseline, she required her arms for mobilization in
her wheelchair. She reported no pain, but instability was her
main concern. No mention was made as to neurosurgical
intervention. At 2 years, she had improved range of motion
without radiographic loosening, no pain, and a stable shoul-
der with functional strength. In the unique patient with a
neuropathic shoulder without rotator cuff function but main-
tenance of deltoid function, Ueblacker et al recommended
surgical management with a reverse total shoulder after con-
servative measures have failed.
Elbow
Indications for neuropathic elbow surgery include persis-
tent pain and the inability to use the arm for activities of daily
living.28 With most of the literature being from small case
series, the results of surgical intervention are often unpre-
dictable with high rates of complications.
Elbow arthrodesis causes significant functional impair-
ment due to inability of the shoulder, wrist, and hand to
compensate for loss of elbow motion. In patients with
neuropathic elbows, the adjacent upper extremity joints are
unlikely to be fully functional.36 Vaishya et al51 performed a
successful elbow arthrodesis in a patient with paraplegia and
a traumatic syrinx. The main indication for the arthrodesis
was elbow instability causing difficulty with activities of daily
living. The arthrodesis was successful, and the patient was
able to perform transfers.
Elbow arthroplasty is considered contraindicated because
of the lack of protective sensation and reflexes, osteopenic
bone, and ligament and muscle weakness.28 Kwon and
Morrey28 have obtained good results with resection arthro-
plasty of the elbow in 3 patients. Although all complained
of instability, each patient was satisfied with the end result
of treatment and had a Mayo Elbow Performance Score >90.
The authors’ recommendations were for functional bracing
over operative stabilization. Despite multiple postoperative
complications, the patients’ pain improved in spite of per-
sistent elbow instability. The patients’ cervical spine pathology
was not discussed, and therefore, it is unclear whether the
Charcot shoulder and elbow
clinical improvements were because of elbow surgery or res-
olution of the disease process.
Yeap and Wallace53 treated a patient with a neuropathic
elbow and an olecranon tip ulcer with an external fixator. The
fixator allowed for daily wound care and provided elbow sta-
bility. Despite 6 weeks in the fixator, the elbow continued to
be unstable.
Ulnar and posterior interosseous nerve palsies can occur
with neuropathic elbows. Deirmengian et al11 reported on 3
patients who underwent neurolysis and decompression that
resulted in recovery of nerve function. Aly et al3 reviewed 1
patient with bilateral ulnar neuropathy with bilateral neuro-
pathic elbows who underwent successful bilateral ulnar nerve
decompressions. Deirmengian et al and Aly et al both sup-
ported surgical nerve decompression.
Conclusion
The key for successful management of the patient with
neuropathic arthropathy is a thorough history and exam-
ination followed by prompt diagnosis. Neurosurgical referral
in patients with syringomyelia should be the first step in
management along with conservative measures that main-
tain limb function, such as splinting and weight-bearing
restriction. Once clinical and radiographic stabilization of
the underlying neurologic process is achieved, surgical in-
tervention to improve joint function can be discussed along
with the goals of management. Given the scarcity of data
regarding outcomes, there is a need for further studies to
provide a treatment algorithm to direct nonoperative in-
terventions and identify patients who could potentially
benefit from operative management.
Disclaimer
The authors, their immediate families, and any research
foundations with which they are affiliated have not re-
ceived any financial payments or other benefits from any
commercial entity related to the subject of this article.
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