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J Shoulder Elbow Surg (2009) 18, e13-e16

1058-2746/2009

doi:10.1016/j.jse

www.elsevier.com/locate/ymse

Arthrodesis in a neuropathic elbow after posttubercularspine syrinx

Raju Vaishya, MS, MCh(Ortho)a, Ajay Pal Singh, MS(Ortho)b,*,Arun Pal Singh, MS(Ortho)b

aDepartment of Orthopaedics, Inderprastha Apollo Hospital, Sarita Vihar, Delhi, IndiabDepartment of Orthopaedics, University College of Medical Sciences and Associated Guru Bahadur Hospital,Dilshad Garden, Delhi, India

A neuropathic (Charcot) elbow joint due to syringomy-elia is a rare condition, and very few reports in the Englishlanguage literature have highlighted this entity2,3,9,10 Wereport a case of Charcot elbow due to syrinx formation thatwas successfully managed with arthrodesis. To the best ofour knowledge, this has not been reported hitherto inEnglish language literature.

Case report

A 46-year-old man, who was left-hand dominant with knownparaplegia due to tuberculosis spondylitis, presented withcomplaints of progressively increasing weakness and instability ofthe left upper limb for the past 2 years. The patient had difficultyusing his left upper limb for activities of daily living such aseating, combing his hair, and maneuvering his wheelchair. Onexamination, the left elbow was swollen, but not warm, and wasnot tender. Range of motion was 25� to 100�, with palpable andaudible crepitation. There was grade 3 weakness of the inteross-eous muscles and digital flexors of the ipsilateral hand. A sensoryexamination revealed sensory loss in the ulnar nerve distribution.Examination showed the median, radial, and musculocutaneousnerves were normal.

Radiographs of the left elbow showed severe destruction of thedistal humerus and proximal radioulnar joint with dislocation, andsevere erosion into the intercondylar area left both condyles in an

uests: Dr. Ajay Pal Singh, D-13, Residential Complex,

ge of Medical Sciences and Associated Guru Teg Bahadur

d Garden, New Delhi.

ss: Delhi.docajaypal@boneandspine.com (A.P. Singh).

/$36.00 - see front matter � 2009 Journal of Shoulder and Elbo

.2009.01.024

inverted U-shape. The proximal radioulnar joint was dislocatedposterolaterally but no dissociation was noted in the proximalradioulnar bones. Diffuse soft-tissue swelling, especially in theposterior elbow and heterotopic ossification, was noted in theadjacent soft tissue (Figure 1).

The patient gave a history of tuberculosis of the thoracic spineinvolving the second, third, and fourth thoracic vertebrae, whichwas treated with anterolateral decompression and antituberculardrugs 4 years previously. He remained paraplegic and wheelchair-bound, but bladder and bowel functions were spared. Previousmagnetic resonance imaging of his cervicothoracic spine revealeda cervicodorsal syrinx extending from C6 to T4, which wasdiagnosed 3 years previously (Figure 2). The patient had refusedany surgical intervention for syrinx at that time.

Results of blood investigations of patient were within normallimits. Computed tomography (CT)-guided aspiration of fluidfrom the elbow joint was investigated for gram stain and acid-fastbacilli stain as well as culture and polymerase chain reaction testfor tuberculosis. All the results were negative. We discussed thetreatment options, both conservative and surgical, including thehigh risks of failure in the latter with patient. Our patient insistedon having a stable elbow.

Through a posterior approach, we performed ulnar nervedecompression and anterior transposition with arthrodesis of theelbow joint. Intraoperatively, the ulnar nerve was markedly dis-placed to the radial side (Figure 3). The radial head was excised.The articular margins of the humerus and ulna were freshened,and a prebent 12-hole locking plate (AO, Synthes Inc, WestChester, PA) was applied with the elbow in a functional position at70� flexion. An anterior submuscular transposition of the ulnarnerve was performed. Results of gram stains, acid-fast bacillistain, and culture of removed tissue were negative.

Postoperatively, the limb was supported in an above elbowsplint for 4 weeks. The patient was followed up at monthly

w Surgery Board of Trustees.

Figure 1 (Left) Anteroposterior and (Right) lateral views of the elbow joint show massive soft-tissue shadow, and dislocation of theulnohumeral joint and the radial head. Articular margins are destroyed, and osseous debris can be seen.

e14 R. Vaishya et al.

intervals with physical examination and radiographs. Aftera follow-up of 1 year, the arthrodesis showed good union(Figure 4), with complete regression of ulnar nerve symptoms.

Discussion

Neuropathic arthropathy of the elbow is a rare conditionand constitutes 3% to 8% of neuropathic joints.2 Syringo-myelia, syphilis, diabetes mellitus, syringohydromyelia,Charcot-Marie-Tooth disease, congenital insensitivity topain, and systemic sclerosis are reported causes of neuro-pathic arthropathy of elbow.3

Syringomyelia is a tubular cavitation of the spinal cordextending longitudinally over many segments and trans-versely across the cord, more often involving the posteriorparts of cord. It causes dissociated segmental anesthesiaover the neck, shoulder, and arms in a cape or hemicapedistribution.4 Syringomyelia is known to be most commoncause of Charcot arthropathy in the upper limb,14 buta combination of Charcot joint and syringomyelia is rare,with only limited literature available on the topic.7,9

Syringomyelia often occurs in association with hind-brain malformations, trauma, tumors, or arachnoiditis.12

Syrinx after tuberculosis is rare.8 In our patient syringo-myelia developed as a sequelae of tuberculosis of the spine.This case is unique because Charcot arthropathy in theelbow after syrinx formation as a sequela of tuberculosis ofthe spine with paraplegia has not previously been reported,to our knowledge.

The pathogenesis of neuropathic joints is uncertain. Twomain theories have been advanced.10 In the neurovasculartheory, initial damage to the joint is a result of damage tothe autonomic nerves and subsequent disruption of thenormal neurovascular reflexes around the joint. This results

in hyperemia and activation of osteoclasts. The resultingbone resorption makes the joint prone to pathologic frac-tures. Alternatively, the loss of protective somatic muscularreflexes can lead to repetitive trauma and, ultimately, tojoint destruction (neurotraumatic theory).10 The neuro-pathic joint in our patient probably resulted due to thesyrinx and repetitive trauma caused by overuse of thedominant limb because he was paraplegic and moredependent on his upper limbs to perform most of hisactivities.

Usual presenting complaints in Charcot joint areswelling, tenderness, and limitation of range of motion ofthe joint. The neuropathic joint is painful in the early stagesof the disease in a minority of patients.7 In the present case,the patient never had pain; his main complaints wereswelling and instability of the elbow. The patient presentedto us after 2 years of onset of the symptoms. Clinicalexamination revealed an elbow that was swollen, but notwarm and tender. Because the patient had been treated fortuberculosis in the past, it was important to rule outtuberculosis as an etiology. The results of all investigationsto rule out tuberculosis were negative. The clinicalpresentation did not raise any suspicion of pyogenicinfection or malignancy, which may also be considered ifclinical findings such as pain, warmth, and tenderness arepresent.

The synovial fluid in Charcot joint may be serous orhemorrhagic. The synovial biopsy is nonconclusive.5 Plainradiographs would reveal soft-tissue calcification, intra-articular fractures, bone fragmentation, and dislocationsalong with severe destruction of the joint. A neuropathicarthropathy can be classified as atrophic or hypertrophicThe atrophic form is characterized by massive boneresorption accompanied by total disintegration of the joint,which causes instability. The hypertrophic form is

Figure 2 Magnetic resonance image shows syrinx from C6 toT4.

Figure 3 Intraoperative photograph shows lateral displacementof ulnar nerve (black arrow).

Figure 4 Elbow radiograph at 1 year of follow-up. Thearthrodesis angle is 70�.

Arthrodesis in neuropathic elbow e15

characterized by severe joint destruction, periarticular newbone formation, osteophytes, sclerosis, fractures, andosseous debris that finally leads to a loss of normal jointarchitecture.10 Hypertrophic osseous sclerosis and

osteophytosis is a feature noted in central spinal cordlesions and atrophic types in peripheral nerve lesion.13 Afew reports suggest that upper limb neuropathy is atrophictype,1 whereas others suggest that hypertropic and atrophicforms are only different forms of natural progression ofdisease.6 Evidence from radiologic findings of massive softtissue shadow, destruction of articular margins, osseousdebris, heterotopic ossification, and dislocation of the

Table I Surgical techniques and outcomes for neuropathic elbow reported in the literature

First author,year

Presentingcomplaint Etiology Treatment Outcome

Kwon,7 2006 Pain, Instability Post traumatic syrinx Total elbow arthroplasty Deep infection developedresulting in resectionarthroplasty

Kwon,7 2006 Pain, instability Congenital neuropathy Resection arthroplasty Joint subluxation neededrevision resection arthroplasty

Deirmengian,3

2001Monteggiafracture-dislocation,ulnar nonunion

Insulin-dependentdiabetes, peripheralpolyneuropathy

Decompression of posteriorinterosseous nerve, internalfixation and bone-grafting of ulnar nonunion

Pain- free elbow motion of 120�

of flexion with a 30� flexioncontracture.

Unnanuntana,13

2006Septic arthritis Diabetes mellitus Incision and drainage and

external fixatorInstability of joint and goodelbow function

Yeap,14 2003 Infection,neuropathic ulcer

Syringomyelia DebridementDebridement andacross elbow external fixator

Flail and grossly deformedelbow with passive range ofmovement of 25� to 150�

Present case Instability, uUlnarnerve compression

Syrinx afterfollowingtubercular spondylitis

Arthrodesis in 110� degreesusing locking compressionplateLCP

Fused elbow with completerecovery of ulnar nervesymptoms.

e16 R. Vaishya et al.

ulnohumeral joint and radial head, led to the considerationthat case was of the hypertrophic type.

Most authors recommend conservative treatment forCharcot joint of elbow due to the unpredictable outcome ofsurgical procedures.3,7,10 Analgesics, splinting, joint aspi-ration, and physical rehabilitation are the modalities used inconservative treatment. The reasons to favor conservativetreatment in all series is good patient satisfaction andacceptance of instability.3,7,10 The very few reports oftreatment by surgery have shown variable results (Table I).

Our patient presented a unique challenge. His mainproblem was instability of the elbow. Being paraplegic, heneeded a stable elbow for wheel chair mobilization andother daily activities. He also had long-standing ulnar nervepalsy that compounded his problem by frequent injuries tothe area of distribution. The association of ulnar nerveinjury and neuropathic arthropathy of the elbow has beendescribed in several reports.3 Ulnar neuropathy is mostlikely due to distortion of the cubital tunnel as a result ofelbow deformity and instability.3 We did forewarn him ofthe high risk of failure of surgical treatment and that motorrecovery for ulnar nerve palsy was unlikely.

Most reports that pertain to the lower limb suggest thatarthrodesis is often the best surgical method to treata neuropathic joint. Total elbow arthroplasty is consideredunsuitable because of lack of protective pain sensation andreflexes, the presence of osteopenic bone, and the weaknessof the surrounding ligaments and muscular tisssues.7

Arthrodesis in a neuropathic elbow is exceedingly diffi-cult.11 We chose internal fixation because that would havegiven the maximum immobilization required.

The patient has shown good union of arthrodesis andimproved sensation in ulnar nerve area. The patient has beenfollowed up for more than 1 year and can use his affected

upper limb to maneuver his wheel chair effectively, cansupport himself into and out of bed and the wheel chair, andwith little assistance can perform daily activities of self-care.

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