UP-TO DATE REVIEW AND CASE REPORT

Bilateral subluxation of the knees secondary to neuroarthropathy:an unusual case

Neeraj Purohit • Geoffrey Channon

Received: 9 February 2010 / Accepted: 11 March 2010 / Published online: 2 April 2010

� Springer-Verlag 2010

Abstract We describe an unusual case of a 68-year-old

woman who developed acute bilateral neuropathic knee

joints. The patient presented with a lower respiratory tract

infection, loss of power and had altered sensation in her

legs. She was diagnosed with Guillain-Barre Syndrome.

After improvement of her neurology, the patient was found

to have a painful, subluxed knee joints. Conservative

treatment was not successful and she underwent bilateral

staged constrained rotating platform total knee arthropla-

sties. 6 months following her most recent total knee

arthroplasty, the patient has pain free movements in both

her knees and mobilises with a frame.

Keywords Guillain-Barre � Charcot � Arthroplasty �Neuropathic � Joint � Knee � Neuroarthropathy

Introduction

Jean-Martin Charcot first described neuroarthropathy in

1868 in patients with tabes dorsalis. Neuropathic arthritis,

also known as Charcot joint, is a destructive condition that

is frequently associated with loss of joint proprioception

[1, 2]. This leads to poor protection of that joint and quite

often, a rapidly progressing arthropathy. Patients with

diabetes mellitus, syringomyelia and other neuropathies

such as Guillain–Barre syndrome are at risk of developing

Charcot joints [2]. Radiological features include joint

degeneration, destruction and dislocation [2]. The man-

agement of patients with Charcot joints involves early

diagnosis and prevention of further joint destruction. Forms

of external immobilisation of the joint in the acute phase

with contact casting have been described most commonly

for the foot and ankle [3], with indications for surgery

being limited.

Guillain–Barre syndrome (GBS) is a post-infectious

immune-mediated condition. Clinically, it is characterised

most commonly by weakness, areflexia and limb paras-

thesia [4, 5]. Pain is a common feature of early GBS with

its intensity being a poor predictor of prognosis [6]. Several

variants of GBS are recognised with the axonal form

characterised by an acute motor and sensory neuropathy

[7]. When neuropathic osteoarthropathy is suspected, a

careful assessment of the patient must be undertaken to find

the cause. In the presence of an acute dislocation, or when

conservative treatment has failed, surgical intervention is

an option.

We describe an unusual case of a patient who developed

bilateral Charcot knees following an acute episode of

Guillain–Barre syndrome. To our knowledge, this is the

first case reported of its kind.

Case report

A 68-year-old woman presented to a local hospital with one-

week history of cough, fevers, rigours and a 2-day history of

progressive weakness in her legs. The patient denied any

back pain, but she had generalised leg pain bilaterally and

had urinary retention. There was no significant past medical

history, and the patient was fully mobile, active and inde-

pendent prior to her admission. On clinical examination, the

patient had weakness with grade 1/5 power (MRC grade) in

the L1-2 myotomes and grade 3/5 in L3-S1 myotomes

bilaterally. Reflexes were absent, and sensation was

N. Purohit (&) � G. Channon

Buckinghamshire Trust, Wycombe Hospital, High Wycombe,

HP11 2TT Buckinghamshire, UK

e-mail: neerajpurohit@hotmail.com

123

Eur J Orthop Surg Traumatol (2010) 20:587–590

DOI 10.1007/s00590-010-0624-6

diminished in both legs. Upper limb neurology was unre-

markable, and perianal sensation and tone were intact. The

patient had a urinary catheter inserted, and a chest radio-

graph was taken. A right-lower-lobe pneumonia was diag-

nosed. An urgent MRI scan of the spine revealed chronic

degenerative changes in the lumbar spine with no cauda

equina. Following a neurology assessment, the diagnosis of

Guillain–Barre syndrome variant secondary to pneumonia

was made, and the patient was started on intravenous

immunoglobulins. No other cause for the patient’s poly-

neuropathy was found, and an MRI of brain and cerebro-

spinal fluid analysis was normal.

Two weeks following admission, the patient remained

non-ambulatory and needed to be hoisted from bed to

chair. The neurological status of her legs had not improved.

The following week, nerve conduction studies were per-

formed to reveal mixed sensory and motor axonal poly-

neuropathy affecting the lower limbs with no evidence of

demyelination. Over the subsequent weeks, the patient’s

neurology improved with normal sensation and lower limb

power of 3/5 bilaterally. The patient was referred for

rehabilitation and intensive physiotherapy.

Six weeks following her admission, the patient had

recovered from pneumonia but still was unable to mobi-

lise due to weakness and pain in her legs. The patient

complained of increasing pain in her knees, worse in her

left. Over the subsequent weeks, pain had increased in the

patients left knee and a deformity was noticed as she

attempted to mobilise. Clinical examination revealed a

cool, non-erythematous left knee with a fixed-flexion

deformity of 40 degrees and a posteriorly subluxed tibia.

Passive range of movement was stiff with an arc of

painful flexion from 40 to 80 degrees with marked

crepitation. The collateral ligaments were stable but

anterior and posterior draw tests could be performed due

to pain and stiffness. Sensation was normal, and the foot

pulses were intact. Examination of the right knee revealed

crepitation, a grade 3 anterior draw and passive flexion of

100 degrees. A radiograph of the left knee revealed a

posteriorly subluxed knee joint with degenerative chan-

ges, Fig. 1a. The right knee was X-rayed as a comparison

and revealed a posteriorly subluxed joint, but to a lesser

extent than that of the left knee, Fig. 1b. Upon re-ques-

tioning, the patient denied any prior history of joint pain,

stiffness or difficulty in walking. After 10 weeks follow-

ing admission, the patient was taken to theatre for an

examination under anaesthetic. The posterior subluxation

was irreducible. Collateral ligaments were stable, and

there was no anterior or posterior draw elicited due to

rigidity. The patient’s left knee was braced for 6 weeks in

an above knee cast and was allowed to mobilise with

physiotherapy. An MRI of both knees was performed and

revealed bilateral cartilaginous destruction, chondrolysis,

deficient anterior cruciate ligaments and a stretched pos-

terior-cruciate ligament, worse on the left, Fig. 2. The

patient underwent staged bilateral constrained rotating

platform total knee arthroplasties, Fig. 3.

Six months following the right arthroplasty and

14 months following the left, the patient lives indepen-

dently and ambulates with a frame. The patient is happy

with her level of mobility and has an arc of flexion from 0

to 120 degrees bilaterally, with no instability.

Fig. 1 Lateral plain

radiographs. a Left knee

showing gross posterior

subluxation of the tibia and

degenerative changes. b Right

knee showing posterior

subluxation of the tibia to a

lesser extent

588 Eur J Orthop Surg Traumatol (2010) 20:587–590

123

Discussion

In typical cases of Guillain–Barre syndrome, there are

mixed motor and sensory features together with autonomic

signs, such as urinary retention. The condition reaches it

nadir at 4 weeks in most cases. Mortality rates at 1 year are

between 4% and 15% with 20% of patients having some

form of disability at 1 year [8]. Charcot joints are well

recognised in conditions that cause neuropathy, such as

diabetes mellitus. The common site for Charcot joints are

in the foot and ankle, with the knee being rarely affected.

Bilateral Charcot knees secondary to Guillain–Barre syn-

drome has not previously been reported. Based on elec-

trophysiological studies, examination findings, the variant

of Guillain–Barre syndrome that most closely fits our case

is acute motor-sensory axonal neuropathy (AMSAN).

Surgery in the management of Charcot knees is con-

troversial and poorly defined. Arthrodesis has been the

popular surgical option for the management of Charcot

knees [9] and generally sparks less controversy than

arthroplasty does [10]. Fullerton et al. described a case of

bilateral Charcot knees secondary to diabetes mellitus. The

patient underwent a highly constrained rotating knee

implant for one side and an arthrodesis on the other [11].

Parvizi et al. performed a retrospective study of 40 knees in

29 patients who underwent a total knee arthroplasty for

Charcot joints [12]. Survivorship at 8 years was 85%. Their

recommendation was that total knee arthroplasty should

not be contraindicated in Charcot knees and suggested a

low threshold for using long stem, more constrained

devices for deformed unstable knees. The aetiologies for

the neuroarthropathy described in this study included dia-

betes mellitus, neurosyphilis and idiopathic. They com-

mented that the outcome following surgery related to the

underlying cause of Charcot knee. This was due to further

neurological deterioration following surgery which may be

detrimental to the arthroplasty.

Kim et al. looked at neurosyphilitic Charcot joints in 10

patients (19 knees) [13]. At their final follow-up (mean

5.2 years), just over half were functioning satisfactorily

(53%), with a 47% complication rate. Despite the severity

of the cases included into this study, the authors discour-

aged the use of constrained hinged prostheses due to the

higher rates of aseptic loosening and the decrease of

available bone stoke for subsequent revisions. Their advice

for managing preoperatively subluxed or dislocated knees

was to immobilise the knees in a long leg brace for at least

6 weeks post operatively. Bae et al. [14] followed 11 knees

(9 patients) for a mean period of 12.3 years (10–22 years).

All 11 knees had rotating hinge prosthesis for Charcot joint

secondary to neurosyphilis. They reported 2 dislocations

and 1 deep infection. They strongly recommended the use

of rotating hinge prosthesis in managing Charcot knees

Fig. 2 T2-weighted sagittal

MRI scans. a Right knee

showing posterior subluxation

of the tibia with a stretched

PCL. b Left knee showing a

100% displacement posteriorly

of the tibia with a severely

stretched PCL and disruption to

the articular surfaces

Fig. 3 Post-operative radiographs. a Right knee. b Left knee

Eur J Orthop Surg Traumatol (2010) 20:587–590 589

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despite their 3 complications. In addition, they recom-

mended the use of a knee brace post operatively, and they

advised in educating patients about limiting over activity.

Good to excellent results have been shown in other

studies [15, 16]. They mention the importance of good

surgical technique, the repair of bone defects, ligamentous

balancing and the use of long stem implants.

In our case, the patient had an acute neurological insult

resulting in a bilateral, painful, destructive arthropathy.

There was also marked joint subluxation, with the left

being more severe. The Guillain–Barre syndrome had

resolved, so there was very little potential risk for contin-

ued neurological deterioration with possible detriment to

the arthroplasties, such as the development of ataxia.

The overwhelming sentiments that total knee arthro-

plasties should be contraindicated in Charcot joints have

been replaced by one that favours them. However, funda-

mental principles of arthroplasty need to be followed

coupled with meticulous surgical technique and the use of a

constrained implant where possible. A total knee arthro-

plasty provides good pain relief and improvement in

function for these patients. Based on our experience in this

case, a constrained rotating platform total knee replace-

ment appears to be a good option in the management of

Charcot knees.

Conflict of interest statement No funds were received in support

of this study. No benefits in any form have been or will be received

from a commercial party related directly or indirectly to the subject of

this manuscript.

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