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Charcot neuro osteoarthropathy is a devastating complication of peripheral neuropathy, primarily effecting the lower limbs. Its commonest cause in western populations currently is Diabetes Mellitus. Its acute presentation is characterised by a combination of destructive features including fracture, dislocation, severe loss of bone density and associated soft tissue swelling and often ulceration. It has a complex etiopathogenesis. Neuro-traumatic mechanisms with activation of complex biochemical pathways coupled with autonomic dysfunction on a background of cumulative microtrauma are generally thought to be the precipitating factors. If neglected, patients eventually present with severe deformity which is either fixed or unstable. This can lead to ulceration due to an abnormal weight bearing profile of the sole of the foot and can be a cause of loss of limb. Observational studies following Charcot patients recurrently reflect that limb loss is a poor prognostic indicator.
The traditional classification for the stages of Charcot arthropathy has been Eichenholtz’s system which is based on clinical and radiological evaluation of the limb in question. Originally described in 1966 by Eichenholtz in 3 stages (Eichenholtz SN. Charcot Joints. Springfield, IL, USA: Charles C. Thomas; 1966), a third stage, 0 ,was added in by Shibata et al(The results of arthrodesis of the ankle for leprotic neuroarthropathy. Shibata T, Tada K, Hashizume CJ Bone Joint Surg Am. 1990 Jun; 72(5):749-56.)
Treatment of Charcot disease is complex difficult frustrating and unpredictable. The deformities caused by this disease are often multi-level and multi-axial and operative planning is difficult upon the background of ulceration, osteomyelitis and bone destruction. The importance of timing of the operation cannot be overemphasised as in the earlier phases of the disease operation can have devastating sequlae secondary to almost unfixable bone and unclosable soft tissues.
It is generally accepted that any surgical intervention for stabilisation or deformity correction is best done after the stage of repair and remodelling are complete (Eichenholtz stage 3). It is also of paramount importance that the deformity correction is done when any ulcers are rendered infection free or healed for fear of devastating infection which can often lead to loss of limb. Earlier operation is though on occasion required, in particular in the context of an uncontrollable and progressing deformity or impending soft tissue breakdown.
Choosing the method of stabilisation is equally important as conventional methods of treating these deformities fail regularly both due to altered bone healing with the production of abnormal collagen as well as non-compliance with non weight bearing by patients who can’t feel their feet.
The choice of hardware in fixing these difficult fusions is also of significance as conventional hardware, such as small fragment plates and screws, are almost sure to fail. Specially designed implants are now available for use which utilises robust designs with highly secure fixation screws, low profile yet strong and malleable plates as well as the use of intra-medullary beams and bolts which allow compression as is found with the Wright Medical Salvation system of fixation. It is also essential to use bone stimulation techniques including the use of osteogenic materials such as bone graft, bone marrow aspirate concentrates, Bone Morphogenic Protein, external and internal bone stimulators. The success of the operation depends on thorough preparation, robust fixation and protracted protection postoperatively.
External fixation is also used with innovative techniques such as minimally invasive surgery to create a treatment algorithm that is specifically of use in patients with poor skin or peripheral vascular disease. It is the case that many of these procedures are refusions of failed operations and the quality of skin is often compromised with multiple previous incisions where external fixation techniques with fine wires can be of great use.

INDICATIONS
Medial stabilisation with arthrodoesis of multiple joints along the medial ray of the foot is best reserved for significant deformity arising from Charcot or other destructive conditions such as infection, post traumatic deformity and instability, severe adult acquired flat foot with arthritis and instability, the rocker bottom foot due to various causes, and occasionally congenital deformities of the medial ray
SYMPTOMS & EXAMINATION
The classic patient is a diabetic patient with reasonable peripheral circulation of longstanding with almost universally a history of peripheral neuropathy and Charcot arthropathy. Deformity is the primary indication and can be either fixed or unstable and dynamic deformity. The patient may present with or without an ulcer. The presence of an ulcer is not a contraindication for surgery as sometimes the only way to heal the ulcer is to correct the deformity. It is however of paramount importance that the ulcer is sterile and proven so with deep tissue samples showing negative microbiology. Many patients describe pain which is more of a deep seated ache and is seemingly different to somatic pain felt by patients with normal sensation. Many patients will have presented previously with their symptoms of acute or sub-acute Charcot ( Eichenholtz stages 1 &2) or may present with infections as a result of ulceration and subsequent tissue loss. many of them present with indolent ulcers which haven’t healed in spite of extensive treatment by other health care professionals such as podiatry and tissue viability specialists. There is sometimes a history of osteomyelitis is not uncommon.
Examination is extremely important so that subtle features of instability, deficiency of tendon mechanisms, peripheral neuropathy, occult deformity such as equinus, and circulatory insufficiency are not missed.
Local examination should include mapping of sensation in the foot as well as assessing circulation. The use of the 1 gram Monofilament to assess sensation, tools to assess position and vibration sense and a hand held Doppler ultrasound probe to assess circulation is almost indispensable as part of routine examination of the diabetic foot. The various joints in the foot are carefully examined for instability or fixed deformity. The sole of the foot is also of great importance as bony prominences in the other rays may also have to be dealt with to avoid a recurrence of ulceration. If an ulcer is present then a careful assessment and classification of the ulcer is essential. This will have to be coupled with taking deep tissue specimens for microbiological culture to establish the sterility of the ulcer before considering operation. Joints such as the ankle joint that are seemingly not involved in the deformity should also be carefully examined. The commonest finding is that of equinus with true Achilles tendon contracture. This is as a result of a lax short and adynamic tendon as the foot suffers a midfoot break often in the 1st tarso-metatarsal, naviculo-cuneiform, or talonavicular joint which over a period of time leads to shortening and contracture of the Achilles tendon. This results in the lack of range of movement in the ankle joint for propulsive ambulation. If this equinus is not identified it is almost certain to cause the procedure to fail as the fusion sites will suffer an enormous amount of stress through the mid whilst walking leading to failure of fusion even months after the operation or the emergence of instability or deformity in other joints in the midfoot. It is also necessary to examine the rest of the limb to ensure other parameters such as the anatomic and mechanical axis of the limb or not abnormal so that fusion will not result in an abnormal position of the weight-bearing foot which is disastrous as the foot no longer has the ability to compensate for the position.
One must not forget taking into consideration other co-morbidities in the patient and optimisation of renal and cardiac function is very important. The multidisciplinary team approach is almost indispensable with optimal treatment of the glycemic status playing a pivotal role in post operative management of the patient. It is well recognised that optimisation of the Hb1Ac is extremely important prior to operation and may well play a major role in the success of the operation and avoiding complications.
IMAGING
Plain radiographs of the weight bearing foot and the ankle in Anteroposterior and lateral projections, subtalar views (Broden) special views such as the Salzman view are required and in the absence of infection are most useful in determining the nature of the deformity. This may be supplemented by CT and/or MRI if additional information about soft tissues or 3D reconstructions are required for the purpose of reconstruction.
If there is severe deformity then I would almost always ask for a vascular surgical opinion which often includes a CT angiogram as the medial neurovascular bundle is often severely distorted by the deformity. If there is a history of past infection then a White Cell Labelled Scan is very useful to assess the presence of residual or occult infection.
During the course of examination the process of consent should also begin. Planning the appropriate operation and explaining in detail the various options available to the patient following examination is extremely important as the complications are life changing including limb loss. Non union rates are high as well as problems with metalwork and wound healing and this should be carefully highlighted to the patient
CLASSIFICATION
The Modified Eichenholtz Classification is the one most commonly used to stage patients and provide a broad idea on the timing of surgical treatment. The Eichenholtz classification has not been validated though is in common usage. It also has its limitations in that it does not take into account the anatomic site or patient co-morbidity which are also important for decision making in the treatment of Charcot disease.
Stage 0 (prodromal)
Clinical: Swelling, erythema, and increased limb temperature. Xray: Normal radiographs.
Stage 1 (Acute)
Clinical: Swelling, erythema, increased limb temperature and Ligament Laxity. Xray: Osteopenia, fractures, fragmentation and subluxation.
Stage 2 (Coalescence)
Clinical: Decrease in swelling, limb temperature and oedema. Xray: Callus formation, fracture healing, sclerotic areas.
Stage 3 (Consolidation)
Clinical: Normal colour temperature and girth. Fixed or unstable deformity. Xray: Remodelling of deformity, mature callus, Fixed deformity
Other classifications including the Sanders and the Brodsky classification use the anatomic site of bony destruction to describe the types of Charcot arthropathy. These are also limited in their ability to either prognosticate or plan treatment. Lew Schon’s classification further subdivides midfoot Charcot into sites with the most significant involvement. He also stages the condition based on the severity of deformation and collapse in the sagittal plane of the foot.
However none of the classifications are comprehensive and it is generally the case that Eichenholtz classification continues to enjoy the widest usage in current diabetic practice.
ALTERNATIVE OPERATIVE TREATMENT
Fine wire frame stabilisation of the fusion can also be used and when done with minimally invasive osteotomy and sequential correction of the deformity is a biologically friendly method of correction of deformity particularly in the presence of poor soft tissue or vascular compromise.
NON-OPERATIVE MANAGEMENT
This largely consists of orthotic devices or custom made footwear to accommodate the deformity if fixed or to correct it if it is flexible. This can be employed in patients whose deformity is braceable, elderly patients or patients whose compliance post surgery is poor. If the deformity is fixed it may be possible to “de-profile” the foot by resection of bony prominences.(exostectomy).
CONTRAINDICATIONS
The main contraindication is the presence of active infection , severe peripheral vascular insufficiency or a lack of confidence in the compliance of the patient post operatively. The procedure must be approached with caution in patients with severe concomitant systemic disease such as renal disease, cardiac and cerebral disease. As a rule the operation is contraindicated in patients with a poor glycaemic control as evidenced by high blood sugars and a high HbA1C as it has significant implications on wound healing post operatively as also the success of fusion.

I do not use a tourniquet for this operation in a diabetic for fear of injuring the somewhat tenuous and often calcified vasculature. If I am confident that the use of the tourniquet will not compromise the patients vascular supply then i would elevate and exsanguinate the limb and use and above knee tourniquet. The patient is placed supine without a sandbag as most of the operation is conducted from the medial side. If a Tendo-achilles lengthening is required I use the percutaneous 3 cut lengthening as was done in this patient. I use diathermy for haemostasis.
The limb is prepped to above the patella as this is required to access the patella for the purposes of assessing the alignment of the limb during and at the end of the operation. Bone graft may also be taken from the proximal tibia. A sand bag is placed initially under the contralateral buttock to make medial access easy if required.

The postoperative protocol for fusion of the Charcot midfoot remains a challenging aspect of the treatment. It is my practice that the patient is kept non weight bearing for at least 10 weeks and sometimes longer. This can be very difficult for a patient for very practical reasons but its importance has to be emphasised.Other issues also arise as a consequence of non-weight bearing, in particular a lack of mobility makes glycemic control very difficult.
I review the patient at 1 week to assess the wound and then every week thereafter to change the plaster as there is a significant risk of plaster induced ulceration due to the peripheral neuropathy.
Patients are often converted to a Beagle Bohler Walker(Beagle Orthopaedics Ltd) at 6 weeks to help them ambulate which helps with glycemic control. This is a significant advantage in being able to keep the patient’s morale up whilst having its biomechanical advantages as well as the psychological gains for the patient who can gain a degree of independence at least whilst at home.
A radiograph AP, Lateral and oblique of the foot is procured at 6 weeks, 3 months and eventually if required at 6 months. The patient is also measured for a semi-rigid diabetes friendly orthosis at 6 weeks so that this can be used at 3 months when the patient is finally ready to mobilise weight bearing.

The results of fusion of the midfoot in Charcot disease is variable with a high risk of complications including metalwork failure, non union, wound breakdown, infection and amputation. Meticulous preparation of the joint, use of osteoinductive adjuncts, full correction of deformity, and the use of robust and Charcot specific implants are the main tenets of such fixation. As mentioned before a multidisciplinary approach is indispensable for the success of such formidable procedures. There are no long term Level 1 studies that can rely on a particular system of fixation to offer predictably good results due to the variety of implants that are required for this challenging condition. Confounding variables make the provision of such results almost impossible. An “A la Carte” approach is often required with a wide variety of operative techniques, implants and strategies being available for use in different clinical settings.
Some results of fixation bolts and complex fixation with a variety of implants are given below:
Journal of Foot and Ankle Surgery 2013 Mar-Apr;52(2):235-8. doi: 10.1053/j.jfas.2012.12.003. Epub 2013 Jan 11. Early results with the use of midfoot bolts in Charcot Arthropathy. Cullen BD et al.
Orthopaedic Proceedings Feb 21st 2018 Online publication: Corrective fusion for Charcot Neuroarthropathy: The Kings Experience
Foot Ankle Int. 2019 Jan;40(1):18-23. doi: 10.1177/1071100718799966. Epub 2018 Oct 4. Clinical Outcomes and Complications of Midfoot Charcot Reconstruction With Intramedullary Beaming. Ford SE1, Cohen BE2, Davis WH2, Jones CP2.
Reference
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