Eur J Orthop Surg Traumatol (2008) 18:229231

DOI 10.1007/s00590-007-0291-4

C A S E RE P O RT

Charcot joint of shoulder

Korhan Ozkan Umut Yavuz Budak Akman
Namik Kemal Ozkan Levent Eralp

Received: 18 January 2007 / Accepted: 10 September 2007 / Published online: 22 November 2007
Springer-Verlag 2007

Abstract Neuropathic arthropathy involving the shoulder

is a relatively rare chronic disorder characterized by the
destruction of the joint which is also associated with
decreased sensory innervation (Lequesne et al. in Rev
Rhum Mal Osteoartic 49(6):427437, 1982; Yank et al. in
Rheumatol Int 24(4):238241, 2004). Here, we report a
case of neuropathic arthropathy of the shoulder (charcot
shoulder), secondary to syringomyelia with classic clinical
and radiographic features of the entity.
Keywords Charcot joint Shoulder Syringomyelia
Sarcoma Biopsy

Epaule de Charcot, propos dun cas

Rsum Les arthropathies neurologiques aVectant lpaule sont des aVections rares associant une destruction articulaire une perte de sensibilit. Nous rapportons ici un cas
dpaule de Charcot secondaire une syringomylie avec
ses caractriqtiques cliniques et radiologiques.
Mots cls Arthropathie de Charcot paule
Syringomylie Sarcome Biopsie

K. Ozkan (&) U. Yavuz B. Akman N. K. Ozkan

Department of Orthopaedics and Traumatology,
Goztepe Training and Research Hospital,
veysipasa sok barbaros mah. Atayurt sit B
blok daire 19 Uskudar, Istanbul, Turkey
e-mail: korhanozkan@hotmail.com
L. Eralp
Department of Orthopedics and Traumatology,
University of Istanbul, Istanbul, Turkey

Case history
A 36-year-old right hand dominant female, with a history
of acute onset pain 2 weeks ago and progressive left shoulder swelling with no history of trauma was referred to our
clinic from one of the state hospital with the suspicion of
soft tissue sarcoma. The patients past medical and surgical
histories were otherwise unremarkable.
Physical examination of patients left upper extremity
revealed generalized swelling and diminished function of her
left shoulder with 30 active forward elevation, 20 active
external rotation and 40 abduction. Her internal rotation was
to the level of sacrum. Neurological examination displayed
decreased sensation to light touch and pinprick throughout
the upper extremity. Although triceps jerk was found to be
normal, her biceps and supinator jerk was hypoactive. Proprioception and vibration sensation were preserved.
Her left shoulder anteroposterior plain radiographs
revealed destruction of humeral head associated with soft
tissue calciWcations and ossous debris (Fig. 1). MRI of the
patients left shoulder showed 10 7 cm soft tissue mass
mimicking soft tissue sarcoma (Fig. 2).
A Wne needle biopsy of the soft tissue mass was negative
for any evidence of malignancy or infection and it simply
represented a nonspeciWc inXammatory reaction with synovitis. So, open biopsy was performed which revealed a
large soft tissue mass and necrotic bone was excised completely. The pathological results were consistent with the
frozen samples taken during operation with hypervascular
synovial tissue (Fig. 3). Physical examination of patients
left upper extremity 10 days after the open biopsy, revealed
45 active forward elevation, 35 active external rotation
and 45 abduction. Her internal rotation was to the level of
sacrum with no diVerence comparing to pre-operative
value. This small increase in the range of motion of the

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Eur J Orthop Surg Traumatol (2008) 18:229231

Fig. 3 Hypervascular synovial tissue

onance imaging was performed that showed a large syrinx

in the cervical region.
The patient was put on a rehabilitation program that
emphasized on passive stretching and strengthening exercises for the deltoid and scapular stabilizers after than
referred to the neurosurgical department for shunting operation for the cervical syrinx.

Fig. 1 Destruction of humeral head associated with soft tissue calciWcations and osseous debris

Fig. 2 Soft tissue mass mimicking soft tissue sarcoma

shoulder was attributed to pain relief of the patient after

complete excision of the soft tissue mass.
Keeping in mind the decreased sensation to light touch
and pinprick throughout the upper extremity, magnetic res-

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Discussion
Neuropathic arthropathy involving the shoulder is a relatively rare chronic disorder characterized with the destruction of the joint, which is also associated with decreased
sensory innervation [13].
The diVerential diagnosis of the shoulder includes neoplasm, tuberculosis, osteonecrosis and microbial infection which can cause bone fragmentation with collapse
and gorham stout disease [46]. Gorham stout disease is
a very rare disorder characterized by uncontrolled,
destructive proliferation of vascular and lymphatic capillaries within bone and surrounding soft tissue. Generally,
laboratory studies are within normal limits. Histopathologic Wndings are distinct for Gorham disease and
includes vascular or lymphatic proliferation in early
stages or Wbrous tissue in later stages in histologic specimens of the aVected bone. The natural history of
Gorhams disease is unpredictable with spontaneous
regression. However, in many patients the disease is progressive and involvement of vital structures like pleural
cavity or thoracic duct may be fatal. Though, the neuropathic arthropathy usually become symptomatic over
many years [7], rapid progressive destruction pattern can
be seen as in our patient.
Charcot and Mitchell emphasized on the destruction of
CNS trophic centers that control the bone and joint nutrition, but still the exact mechanism of association between

Eur J Orthop Surg Traumatol (2008) 18:229231

syringomyelia and neuropathic arthropathy remains controversial. Brower and Allman postulated a neurovascular
mechanism that neurally mediated vascular reXex secondary to the impairment of sympathetic vascular modulation
leads to the increase of bone blood Xow which in turn
leads to active resorption of bone by osteoclasts with fracture and joint damage occurring secondary to the degree
of weight bearing and sensory impairment aVecting the
involved joint [1].
In our patient, we emphasized on the maintenance of
function with stretchening and strengthening exercises of
deltoid and shoulder stabilizer muscles rather than performing surgical procedures like arthrodesis and prostetic
replacement of the damaged joint due to high failure rates
[2, 5].

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References
1. Lequesne M, Fallut M et al (1982) Rapid destructive arthropathy of
the shoulder. Rev Rhum Mal Osteoartic 49(6):427437
2. Yank B, Tuncer S et al (2004) Neuropathic arthropathy caused by
Arnold-Chiari malformation with syringomyelia. Rheumatol Int
24(4):238241
3. Richte L, Frigelli S et al (2002) Neuropathic shoulder arthropathy
associated with syringomyelia and Arnold-Chiari malformation
(type 1). J Rheumatol 29(3):638639
4. Jones J, Wolf S (1998) Neuropathic shoulder arthropathy (charcot
joint) associated with syringomyelia. Neurology 50(3):825827
5. Hatzis N, Kaar TK et al (1998) Neuropathic arthropathy of the
shoulder. J Bone Joint Surg Am 80(9):13141319
6. Rao P, Kotwal PP et al (2001) Painless destruction of the shoulder
joint:case. Clin Rheumatol 20(1):143146
7. Turkiewicz AM, Kerr G (2004) Clinical images: syrinx-induced
Charcot shoulder. Arthritis Rheum 50(7):2380

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