Neuropathic arthropathy diagnosis and management guidelines

charcots arthropathy diagnosis and management

1. CHARCOTS ARTHROPATHY Anand Leo George

2. Jean Martin Charcot , a French neurologist and professor of anatomical pathology who is known as Founder of modern neurology , in 1868 gave the first detailed description of this disease.

3. chronic progressive degenerative arthropathy affecting one or more peripheral or vertebral articulations Result of a disturbance in the normal sensory innervation of joints. Conditions associated - Diabetes, Syphilis and syringomyelia. Rare conditions associated - Leprosy, Spinal dysraphism, congenital insensitivity to pain, yaws, Spina bifida, myelomeningocele, peripheral nerve injury, amyloid neuropathy, spinal cord injury, Vit B12 deficiency, Phenylbutazone, Indomethacin.

4. Diabetes is the most common cause. 0.1% to 29% of diabetic patients Commonly involved joint- Tarsal(M/C) , midtarsal, tarsometatarsal, metatarsophalangeal and interphalangeal joints

5. The most common cause of upper extremity neuropathy is Syringomyelia. Characterized by monoarticular presentation , mostly involving shoulder and less frequently the elbow.

6. Classification based on anatomical location Type 1 - involves tarsometatarsal joints Type 2 - refers to triple joint complex Type 3A - involves tibiotalar joint Type 3B - pathological fracture of calcaneus.

7. Eichenholtz classification

8. Pathogenesis Neurotraumatic theory - A joint with abnormal sensory innervation, will undergo rapid destruction as a result of minor traumatic event, if unprotected. Neurovascular theory ( A.C.Brower theory )- Neurological changes produced by an underlying medical disorder create a hypervascular region in subchondral bone characterized by increased osteoclastic resorption and osteoporosis.

9. Loss of vasomotor tone leading to severe regional osteopenia followed by Hypervascular flush with marked osteoclastic activity Loss of subchondral bone with loss of support Weight bearing continues due to lack of sensation Leads to mechanical displacement of shards of shredded cartilage and foreign body response.

10. Three distinct phases in progression of neuropathic joint Destructive phase - Characterized by hyperemia, swelling and osteoclastic bone resorption. Reparative phase - Results in the formation of dense fibrous tissue within joint and dense sclerotic bone at joint line and in surrounding tissue. Quiescent phase - Characterized by decreased vascularity, stabilization of periarticular reaction and significant osseous sclerosis.

11. Diagnosis WBC, ESR are usually normal Joint aspiration usually produces a large quantity of yellow fluid MRI to differentiate between soft tissue infection and OM Histology Synovial and bone biopsy specimens is most reliable Finding - Presence of osseous and cartilaginous debris deep within synovium

12. Radiography Atrophic - Characterized by massive bone resorption and virtual disintegration of the joint. Seen in hip, shoulder and foot. Hypertrophic - Characterized by severe joint destruction, periarticular new bone formation, osteophytes, fractures and osseous debris. Seen in knee, elbow and ankle

13. Clinical presentation Diffusely swollen, warm and erythematous joint. Vague or non specific h/o trauma Painless joint Changes in joint similar to osteoarthritis Further progression can cause joint subluxation and rocker bottom foot in diabetes

14. Management Treat underlying disorder Patient education, joint protection and early recognition of fractures. Surgery can be considered in advanced joint destruction when there is significant disability.

15. Tabes dorsalis - Hip, knee, ankle Syringomyelia - Shoulder, elbow, wrist Diabetes - Tarsal and tarsometatarsal joints

16. Regional survey- Spine Involved in Syphilis, syringomyelia, spinal dysraphism, diabetic neuropathy, myelomeningocele and spinal tumors. Symptoms - Thoracolumbar junction and lumbar spine commonly affected Presents with painless, progressive spinal deformity. Investigations - Massive new bone formation is characteristic. Destruction of articular facets initially Large marginal osteophytes formation secondary to instability (Parrot beak appearance) Disc space narrowed and retrolisthesis can occur

17. Treatment Involves immobilisation of hypermobile segment to prevent disastrous neurological sequale. Principles 1. Posterior segment instrumentation and fusion for single level involvement with bone grafting of anterior single level defect. 2. Restoration of normal saggital plane contour with anterior first stage surgery for rigid kyphosis or multiple level involvement.

18. Shoulder Mostly associated with syringomyelia, but also a/w syphilis, DM, Arnold chiari malformation, cervical spondylosis, adhesive arachnoiditis, tuberculous arachnoiditis and post traumatic syringomyelia. Symptoms - Painless swelling of shoulder Active motion limited, but passive motion maintained Investigations - joint aspiration produces large amount of straw coloured fluid with particulate debris.

19. Xray - Osteolysis with osseous fragmentation and destruction Treatment - 1. Protective immobilization 2. Arthrodesis if marked instability present

20. Elbow A/w syringomyelia, syphilis or congenital insensitivity to pain Symptoms - Significant swelling. Deformity and instability as joint destruction progresses. Xray - Destruction and subluxation of radiohumeral and ulnohumeral joint. Sclerosis, extensive osteophytes, periarticular swelling, calcification Treatment - Bracing that allows flexion and extension neutralizing varus and valgus stress, Arthrodesis for resistant cases.

21. Wrist and hand A/w Diabetes, leprosy, syringomyelia and syphilis Symptoms - Swelling and deformity without pain Xray - Narrowing of intercarpal spaces, disorganised carpal alignment and desintegration of carpal bones Treatment - Prolonged immobilization and functional bracing Joint debridement and Arthrodesis when gross instability present

22. Hip A/w Syphilis Symptoms - Two patterns 1. Involves fracture of femoral head or neck due to mild trauma 2. Arthritic type - progressive wear and fragmentation of femoral head and acetabulum. Presents with painless progressive worsening limp Xray - Femoral head and neck resorption

23. Treatment Neuropathic hip fractures(Young patients) - Fix and immobilize in spica cast Operative management in severe disability - Shanz osteotomy if there is head and neck destruction 1. Joint debridement and synovectomy with loose body removal 2. Femoral neck fractures - Hemi arthroplasty. 3. Arthrodesis and THR have high complication rate.

24. Knee A/w Syphilis and diabetes Symptoms - Joint instability, crepitus and pain Xray - Progressive joint destruction, fragmentation, hypertrophic new bone formation and subluxation. Treatment - 1. Bracing 2. Arthrodesis 3. Synovectomy with arthrodesis

25. Foot and ankle A/w long standing diabetic peripheral neuropathy is most common and joint manifestations are unilateral. Symptoms -1. Decreased vibratory sense 2. Anhidrosis 3. Loss of ankle reflexes 4. Warmth, swelling of insidious onset in painless foot or ankle 5. Hammer toes and thinning of fat pad beneath metatarsal head Signs - Ankle : Instability, crepitus, fixed varus or valgus deformity Foot : Shortened, thickened and collapse of longitudinal arch

26. Pathology Pattern of destruction described by Harris and brand . Changes are usually hypertrophic with various stages of fragmentation, resorption, sclerosis, periarticular calcification and new bone formation Navicular dislocations, lisfranc fracture dislocations, calcaneal tuberosity avulsions, cuneiform destruction have been reported. Plantar callosities and ulcerations. Dissolution of metatarsal neck and shaft - pencil in cup deformity. Dissolution of proximal phalanges - hourglass appearance

27. Investigations The best diagnostic study is histologic examination of synovial and bone biopsy specimens. X-rays - Tibiofibular dissociation, subchondral sclerosis, osteophytes, Vascular calcification. D/D - Psoriatic arthritis, Rheumatoid arthritis, Gout, TB, Osteomyelitis

28. Treatment Foot care and daily inspection Vitamin B12, thiamine and pyridoxine supplements Wellpadded cast or polypropylene splint for 6 months Weight bearing with custom molded extra depth shoes or patellar tendon bearing orthoses for 1 year or until osteopenia has resolved. Plantar ulceration needs surgical management.

29. Ankle arthrodesis Goal - To establish normal weight bearing axes, Create plantigrade foot Eliminate need of prolonged bracing Charnley or Calandruccio or other external compression techniques augmented anteriorly by sliding tibial graft are popular. Intramedullary ankle arthrodesis nail If talus disintegrated - excision of remaining necrotic talus followed by tibiocalcaneal fusion Amputation

30. Foot Aggressive removal of plantar ulcers and exostectomy Midfoot and hindfoot osteotomy - to achieve plantigrade foot Amputation

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