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Neuropathic Arthropathy of the Glenohumeral Joint: A Review of the Literature.

Neuropathic arthropathy (NA) is a destructive joint process postulated to result from loss of nociception, pain sensation, or both. (1,2) First described by Mitchell in 1831, neuropathic arthropathy is actually named after Jean-Marie Charcot for his association of tabes dorsalis with severe arthritic changes and joint destruction in 1868. (1,2) In 1892, Sokoloff reported the association between upper extremity neuropathic arthropathy with the joints of the upper extremity and syringomyelia. (1,3) A syrinx is believed to be the most common underlying etiology of the neuropathic shoulder, being identified in 80% of upper extremity Charcot and 25% of neuroarthropathic joints overall. (4-6) Arnold-Chiari malformations, post-traumatic syringomyelia, cervical spondylosis, infection, and diabetes are other potential causes. (1) Historically, syphilitic myelopathy and tuberculosis spondylitis were common etiologies, although aggressive public health initiatives have substantially decreased the prevalence of these conditions in North America. (1)

Despite being described over a century ago, the optimal management of the neuropathic shoulder remains unclear and is heavily influenced by historical data. The purpose of this review is to report on the current understanding of the pathogenesis, clinical presentation, and treatment of neuropathic arthropathy of the glenohumeral joint (NAGHJ). To clarify the role of surgical reconstruction in the neuropathic shoulder, a literature search was performed with a focus on modern orthopedic management.

Pathogenesis

A comprehensive understanding of NAGHJ remains elusive. The vast majority of basic science research to date has attempted to understand the underlying pathophysiology of diabetic neuropathic joints. Nonetheless, available evidence supports two prevailing theories: the neurotraumatic and neurovascular theories. The "neurotraumatic theory" proposes an underlying neurologic disorder that leads to decreased joint sensory innervation necessary for innate joint protection. Repetitive instability and microtrauma then lead to progressive joint destruction. (1,2,7,8) The most archetypal clinical example of this is syringomyelia. Afluid filled cavity (or syrinx) forms in the subarachnoid space of the cervical or upper thoracic spinal cord due to an interference in the flow of cerebrospinal fluid. (9) The presence of this syrinx leads to disruption of the decussating fibers of the spinothalamic tracts that mediate pain and thermal sensation. (10-12) Progressive shoulder destruction with varying levels of pain ensues. Alternatively, the "neurovascular theory" proposes a loss of autonomic vascular control that results in increased blood flow into the joint. This hyperemia allows for excessive recruitment of osteoclasts and monocytes that lead to an escalation of bone resorption. The weakened bone structure is then destroyed by repetitive microtrauma and progresses to joint collapse. (1,2,7,8,13)

In all likelihood, neuropathic arthropathy (NA) is caused by a combination of these theories sharing the mechanism of some degree of neurological derangement. Furthermore, microtrauma from loss of protective sensation can intuitively increase blood flow to a joint, just as edema from hyperemia may impair sensation or predispose a joint to instability and injury. Regardless of the inciting etiology, the resultant inflammation is considered essential to the pathophysiology of NA. (8,14) The increase in pro-inflammatory cytokines leads to activation of the receptor activator of nuclear factor-[kappa][beta](NF- [kappa][beta]) ligand (RANKL), (8,15) which is essential for osteoclast activation, function, and survival. The ligand's expression is induced by osteoblasts and binds to the RANK receptor on mononuclear precursor osteoclasts. This RANK-RANKL bond induces the formation of transcription factor NF-[kappa][beta], leading to the maturation of the osteoclasts and subsequently increasing osteoclastogenesis. (8,15) A known inhibitor of the above processes is osteoprotegerin (OPG), which acts as a decoy receptor that also binds to the RANK ligand. OPG-RANKL binding leads to inhibition of osteoclastogenesis and decreases survival of pre-existing osteoclasts (Fig. 1). (8,15)

In addition to the RANK-RANKL pathway, monocytes involved show decreased secretion of anti-inflammatory cytokines as well as increased resistance to apoptosis. (8,15) This process may be further accelerated or augmented through the interaction of certain neuropeptides that are found to be altered in joints with neuropathic arthropathy. For example, peripheral and autonomic neuropathies decrease the release of neuropeptide calcitonin gene-related peptide (CGRP). Calcitonin gene-related peptide is located in nerve fibers within bone tissue that affects both osteoclasts and osteoblasts and has been implicated in bone remodeling and repair, (8,16,17)

Much remains to be understood about the underlying pathophysiology of this disease. However, inflammation-induced activation of the RANK-RANKL pathway appears to be central to the profound osteoclastic response observed clinically. Ongoing basic science research will determine if targeting this pathway with RANKL inhibitors like osteoprotegerin will ultimately allow for pharmacological management of neuropathic joints.

Clinical Presentation

History

Patients with NAGHJ most often present with generalized pain and swelling about the shoulder region associated with limited range of motion (ROM) and weakness. (18-20) These symptoms are often gradual and progressive. Neurologic complaints such as paresthesias or numbness may be present in the ipsilateral upper extremity. (21,22) The patient may or may not report a history of recent trauma to the affected shoulder joint.

Physical Examination

Physical exam findings canbe quite variable, which likely reflects the differing degrees of joint degeneration. Inspection may show generalized swelling about the shoulder region with or without erythema. The presence of erythema often raises suspicion for infection and septic arthritis has to be considered part of the differential diagnosis. There can be varying amounts of tenderness to palpation and the shoulder may feel warm to the touch. Loss of active shoulder motion is evident, although passive ROM is usually intact. There is often crepitus associated with ROM. Generalized weakness of the upper extremity with or without muscular atrophy is common. (12) Neurovascular evaluation of the extremities may show asymmetrical deficits in deep tendon reflexes and temperature and pain sensation. (23) Classically, syringomyelia is known to produce a "cape-like distribution" of sensory abnormalities along the back and arms. (2,19,24)

Differential Diagnosis

The differential diagnosis of NAGHJ is broad and includes: diabetes, tabes dorsalis, tuberculosis, septic joint, chronic alcoholism, syringomyelia, spinal cord injury, and Gorham disease (vanishing bone disease). (18,22) Obtaining fasting blood glucose or hemoglobin A1c levels, venereal disease research laboratory (VDRL) tests, and quantiferon beta can help identify diabetes mellitus, syphilis, or tuberculosis, respectively. As NA may be confused with septic arthritis, a C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and white blood cell (WBC) count should be obtained. Additionally, aspiration of the joint should be considered and specimens sent for cell count (with differential), and crystals as well as microbiology for aerobic, anaerobic, acid-fast bacilli, and fungal culture. (18,22) Skin testing should be performed if a tuberculous joint is being considered in the differential diagnosis.

Imaging Studies

Radiographically, there are two patterns of disease: atrophic and hypertrophic. (25,26) Atrophic neuroarthropathy is characterized by bone resorption, while hypertrophic neuroarthropathy is frequently mistaken for hypertrophic osteoarthritis with findings of sclerosis, debris, fragmentation, and the presence of exuberant osteophytes. (1,5,24,27) Although the above classification does not appear directly relevant for prognostication or treatment decision making, physicians should be aware that NA of the glenohumeral joint can have a varied radiographic presentation.

In addition to radiographs, magnetic resonance imaging (MRI) of the glenohumeral joint may be considered in order to confirm the diagnosis of NA (effusion, soft tissue inflammation, and glenohumeral joint destruction) or to examine for associated pathology (i.e., rotator cuff tears). (27) An MRI or computed tomography (CT) myelogram of the cervical spine should be strongly considered given the known association between syringomyelia and NAGHJ. (4)

Classification

In addition to the various descriptive classification systems mentioned above, the Eichenholtz system is applicable to the neuropathic shoulder even though it was developed for NA of the foot and ankle. The Eichenholtz system describes three stages of progression of disease: 1. development, 2. coalescence, and 3. reconstruction and reconstitution. (28) Stage 1 is characterized by localized warmth, erythema and swelling about the joint on clinical exam, in addition to fragmentation joint subluxation and dislocation, and bony debris formation on radiographs. Stage 2 shows a decrease of warmth and swelling with radiographs demonstrating absorption of the bony debris and sclerosis. Stage 3 has a complete absence of warmth, erythema, and swelling on examination, while radiographic images show osteophyte formation, decreased sclerosis, joint fusion, and uniform bone fragments. This classification system was later expanded by Shibata et al. (29) to include Stage 0 or a prodromal stage presenting with mild warmth, erythema, and swelling on exam with normal radiographic images.

The utility of this classification system in the shoulder is largely unknown. It is believed that protection from ongoing trauma and identification of inciting cause should be the clinical approach in Stages 1 and 2, with any operative reconstructive effort delayed until Stage 3, if at all. These generalities remain largely unproven and may be more appropriate in the foot and ankle, which is more amenable to bracing and operative management.

Case Report

A 59-year-old right-hand dominant female presented for evaluation of worsening left shoulder pain of 3 years duration. In October, 2012, she sustained a mechanical trip and fall at work, and sustained an injury to her left shoulder. She was evaluated at an outside hospital but was unsure about imaging performed on her shoulder. Since that time, she had continued pain, aggravated with overhead activities such as lifting, pushing, or pulling. The shoulder pain interfered with sleep. She also reported a decrease in fine motor skills, such as buttoning pants or shirts, and increasing difficulty with use of a keyboard. She complained of numbness and tingling in both upper extremities along with an electric shock-type pain that was worse on the left. She also noted impaired temperature sensitivity in both upper extremities and had sustained multiple burns and injuries over the past few years as a result. Her past medical history was significant for eczema. The patient utilized ibuprofen as needed for pain relief. She was employed as a contract manager.

Physical examination revealed a healthy-appearing, middle-aged female. Examination of the upper extremities showed mildly decreased range of motioninboth shoulders. Examination of the right shoulder was significant for active forward elevation to 85[degrees] with crepitus, external rotation to 25[degrees], and internal rotation to lumbosacral area. Examination of the left shoulder was significant for active forward elevation to 90[degrees] with crepitus, external rotation to 30[degrees], and internal rotation to the lumbosacral area. Strength testing of both upper extremities was similar with deltoid, supraspinatus, and infraspinatus and teres minor strength of 4/5 and grip strength of 3/5. Distal sensory exam was intact to soft touch testing.

Radiographs of both shoulders demonstrated an erosive and destructive end-stage arthritis with marked deformity. There was significant glenoid and humeral head erosion, with acetabulization of the glenoid that suggested NAGHJ (Figs. 2 and 3). Magnetic resonance imaging of the spine demonstrated a large syrinx extending from C1-C2 to T10-T11 and Chiari I malformation with tonsillar herniation (Fig. 4).

After the diagnosis of neuropathic arthropathy of the glenohumeral joints secondary to syringomyelia was made, the patient was referred to both neurology and neurosurgery. She proceeded to undergo decompression of cervical and thoracic syringomyelia and is currently participating in occupational therapy. Treatment of the shoulders has been deferred until she recovers from her spinal surgery.

Orthopedic Management

Management of NA of the upper extremity depends on the specific joint involved, the underlying cause, and the extent of the disease process and deformity. (10) Often, the primary goal of treatment is to identify and treat the underlying cause in an attempt to slow or prevent further destruction of the involved joint. (18,19) The mainstay of initial orthopedic management should be non-operative in the vast majority of cases including anti-inflammatory medications to mitigate synovial inflammation, (2,10,22) joint aspiration as needed to provide symptomatic relief and prevent further soft tissue destruction, (12) patient education on minimization of mechanical trauma, (22) and physical therapy to retain motion and strength. (1,12,30) Pharmacologic therapies such as bisphosphonates and calcitonin have been investigated to manage diabetic foot and ankle NA, but the utility of these medications in the upper extremity are currently considered investigational. (31-34)

If non-operative management is unsuccessful and symptoms persist, surgical intervention might include arthrodesis, hemiarthroplasty, or total joint arthroplasty. However, surgical options are predicated on the underlying condition and whether or not progressive degeneration is likely to continue after surgical intervention. Furthermore, underlying sensory and motor deficits limit functional expectations and increase the risk of surgical complications including infection, dislocation, and component failure from loosening or progression of disease. (4,10,35,36)

Literature Search

Although the above treatment approach is intuitive, the evidence supporting it is limited at best and generally historic in nature. Therefore, we performed a literature search that focused on articles published after 1987. PubMed was searched to identify English language articles using the following search terms: neuropathic arthropathy, neuroarthropathy, Charcot arthropathy, syringomyelia, glenohumeral joint, and shoulder. This search produced 27 articles for review.

Of these, three were excluded because they reported cases of NAGHJ but did not specify a treatment modality or patient outcomes. (7,37,38)

The remaining 24 studies (2,4,5,10-12,18,23,25,27,35,36,39-46) represented a total of 50 patients with an average age of 50 years (range: 29 to 82 years). Of these patients, 58% were male (N = 29) and 8% (N = 4) had bilateral disease. Non-operative treatment of the shoulder joint was used in 19 patients (Table 1) and operative management was used in 22 patients (Table 2). Five studies did not describe treatment directed to the shoulder but focused on the syrinx decompression with drainage or shunting, posterior fossa decompression, or cervical laminectomy. (23,5,36,39,43) For this review, we consider operative management to consist of procedures performed on the glenohumeral joint.

Non-Operative Management

Some attempt at non-operative management of NAGHJ was described in 54.2% (N = 13) of the studies (Table 1). (11,12,18,19,21,22,25,27,40-44) Our literature search returned only Level IV evidence. In addition, shoulder function was frequently not described in detail. For example, 38.5% (N = 5) of these studies did not quantify preoperative function, 53.8% (N = 7) had unclear post-intervention function, and 23.1% (N = 3) lacked both preoperative and postoperative outcomes. In general, results after non-operative interventions were variable with often moderate to poor results. Of these patients, the majority of studies reported small improvements in active range of motion (AROM) and mild reductions in pain about the shoulder. Often, the patients were treated using more than one non-operative treatment modality. Physical therapy was utilized in 63.2% of patients, most commonly along with neurosurgical intervention for a syrinx and other therapies. (12,18,22,27,41,42,44) Similarly, NSAIDs were utilized in 36.8% of patients, (21,22,25,27,40,43) but only one patient received this as an isolated treatment. (40) Other reported therapies included bracing or immobilization (N = 4), (11,12,18,27) bisphosphonates (N = 1), (43) and steroid injection (N = 1). (19) Limited, and at times incomplete, data prevented us from reaching meaningful conclusions regarding the efficacy of any non-surgical modality.

Hemiarthroplasty

Three of the 24 studies described performing hemiarthroplasty for NA of the shoulder. (4,35,46) Crowther and Bell (4) described a 40-year-old female who underwent two syrinx drainages over 5 years without relief of her progressive right shoulder pain. She had 100[degrees] of active forward elevation, and imaging showed destruction of the right glenohumeral joint. She ultimately underwent an uncemented shoulder hemiarthroplasty to manage her pain. At 2-year follow-up, active forward elevation was 140[degrees] and external rotation to 50[degrees]. Her radiographs demonstrated progressive glenoid sclerosis without humeral component medialization. (4)

Matsuhashi et al. (35) reported three patients who underwent uncemented hemiarthroplasty combined with open rotator cuff repair after undergoing syrinx decompression. All patients had glenohumeral destruction from NAGH due to syringomyelia. The investigators' indications for shoulder hemiarthroplasty in this series were: 1. no evidence of joint sepsis, 2. no severe palsies in the affected upper extremity, 3. intact greater tuberosity, and 4. patient desired treatment to improve shoulder function. The investigators did not mention the attempted utilization of nonoperative management for the NAGHJ prior to performing surgery. All patients were placed in an abduction brace postoperatively for a total of 8 weeks in addition to rehabilitation with passive followed by active ROM and isometric exercises performed for 12 weeks after surgery. Importantly, the investigators did not mention the non-operative measures utilized prior to performing surgery. All three patients noted an improvement in pain and active ROM after 8 to 10 years of follow-up. All patients were satisfied with their treatment and follow-up imaging did not demonstrate any evidence of prosthetic loosening. (35)

Schoch et al. (46) presented 10 Charcot shoulders they treated with hemiarthroplasty, total shoulder arthroplasty (TSA), or reverse TS A from January 2000 through December 2011. The minimum follow-up was 2 years. Hemiarthroplasty was performed in 6 of the 10 patients (60%).

Total Shoulder Arthroplasty

Three of the 24 studies described utilizing reverse total shoulder arthroplasty in the treatment of NAGHJ. (20,43,46) Ueblacker et al. (20) described a 62-year-old woman with a history of cervicothoracic and lumbar syringomyelia due to spinal trauma who developed bilateral NAGHJ. At presentation, she complained only of instability and denied pain in either shoulder. Her right shoulder showed active forward elevation to 30[degrees] and external rotation to 0[degrees]; the left shoulder showed active forward elevation to 20[degrees] and external rotation to 0[degrees]. Imaging demonstrated glenohumeral joint destruction and massive rotator cuff tearing bilaterally. Her deltoid function was found to be satisfactory preoperatively. The investigators do not mention any attempts at non-operative treatment. The patient ultimately underwent staged bilateral reverse total shoulder arthroplasties with 6 months between the procedures. Two months postoperatively, a screw within the right glenoid baseplate component was changed due to loosening. No further complications were noted. At 24 months postoperatively, the AROM about the right shoulder had a forward elevation of 110[degrees] and external rotation to 45[degrees], and AROM of the left shoulder had a forward elevation of 110[degrees] and external rotation to 70[degrees]. Overall, the patient was satisfied with her treatment and denied pain about either shoulder with improvement in stability. (20)

Atalar et al. (43) described a 46-year-old male who presented for evaluation of right shoulder pain of 1 week duration. Examination demonstrated 30[degrees] of active forward flexion without active external or internal rotation. Imaging showed osteolysis of the humeral head. Ultimately, the patient underwent debridement and resection arthroplasty. As a result of poor function, additional surgery was performed consisting of shoulder arthroplasty utilizing a tumor prosthesis. Infection developed requiring prosthesis removal 10 months postoperatively. Follow-up function was poor with active forward flexion of 20[degrees] and absence of active external or internal rotation. (43)

In Schoch et al.'s series, (46) three patients underwent anatomic total shoulder arthroplasty (aTSA) and one patient reverse total shoulder arthroplasty (rTSA). Notably, two of the aTSAs were later revised to rTSAs at an average of 5 months after the patient's initial surgery. The investigators noted a statistical improvement in pain (p = 0.008), although they did not find improvement in ROM. (46)

Arthrodesis

Although three studies "recommended" arthrodesis for treatment of NAGHJ, (2,21,44) only one study provided the results of this procedure performed. (44) Kuur (44) described a 43-year-old male who presented with destruction of the right shoulder with complaints of pain, swelling, and decreased ROM. Imaging demonstrated resorption of the humeral head. An arthrodesis was performed and follow-up demonstrated union 5 months postoperatively. At the 14-year follow-up, the patient was noted to be working full-time as a laborer and remained asymptomatic. (44)

NAGHJ and Septic Arthritis

Two of the studies described the relationship between NAGHJ and septic arthritis. Gomez-Rodriguez et al. (45) described septic arthritis of the shoulder in two patients with a history of NAGHJ secondary to syringomyelia. Aspiration in the first patient grew Staphylococcus aureus susceptible to vancomycin and cloxacillin. The patient was treated with surgical drainage and debridement along with cloxacillin and gentamicin antibiotic therapy. At 2-year follow-up, there was no evidence of continued sepsis although shoulder function was poor secondary to rotator cuff tear and humeral head destruction. The second patient developed a respiratory infection with Staphylococcus epidermidis and positive blood cultures with the same organism. Ultimately, Staphylococcus epidermidis was isolated from joint aspiration. Surgical debridement and drainage was performed, followed by 6 weeks of vancomycin and rifampin therapy. At the time of last follow-up shoulder function was poor AROM was less than 20[degrees] abduction and less than 10[degrees] external rotation. (45)

Ruette et al. (12) described a 46-year-old male with past medical history of NAGHJ secondary to syringomyelia who later presented with septic arthritis after pneumonia. Treatment consisted of surgical debridement followed by parenteral antibiotics for 1 month with resolution of the infection.

Other Operative Management

Two of the studies utilized other surgical modalities not mentioned above. (5,10) Alai et al. (10) described a 49-year-old male with NAGHJ secondary to syringomyelia in which treatment consisted of neurolysis of the right infraclavicular brachial plexus with synovectomy of the right shoulder. Six weeks postoperatively the patient noted improvement in pain and function about the shoulder. At the 5-year follow-up the patient had 140[degrees] of active forward flexion and 80[degrees] of external rotation with minimal pain. However, at the 7-year follow-up, the patient had developed a synovial cyst arising from the acromioclavicular joint requiring aspirations and indicative of rotator cuff deterioration. Active ROM declined to forward flexion of 70[degrees] and external rotation to 20[degrees]. (10)

Richards and Delaney (5) described two patients who presented following failed shoulder instability surgery with a missed diagnosis of NAGHJ secondary to syringomyelia. Both patients underwent multiple procedures to treat instability before arriving at the diagnosis of NAGHJ. These reports stress the importance of maintaining NAGHJ in the differential diagnosis. It is very unlikely that either of these two patients would have undergone shoulder stabilization procedures if the diagnosis of NAGHJ had been recognized initially.

Discussion

Neuropathic arthropathy of the glenohumeral joint is very rare, with sparse evidence available to help guide treatment. Our results support the finding by Floyd et al. (3) in 195 9: 80% of the joints with neuropathic involvement secondary to syringomyelia occur in the upper extremity and the most common joint in the upper extremity affected by syringomyelia is the shoulder, followed by the elbow and then the wrist and hand. (2,12,21,27,43) Patients may present with a wide variety of symptoms such as shoulder pain or discomfort, swelling in the glenohumeral joint, and decreased ROM. A detailed history and physical exam including both musculoskeletal and neurologic examinations are vital to determining etiology. Based upon the literature review, the only clearly supported recommendation is that the underlying cause of NAGHJ must be identified and adequately treated. The role and effectiveness of non-operative and operative orthopedic management remains unclear, with no general conclusions possible due to limited evidence and poor quality studies. Nonetheless, it seems intuitive that non-operative management of NAGHJ should be the first step, including: anti-inflammatory medications, joint immobilization with splinting or bracing, patient education, and if possible physical therapy to maintain ROM and strength. (2,7,22)

Historically, operative management has been discouraged in the treatment of NAGHJ. Patients with neuroarthropathies are known to have an underlying sensory deficit with destructive changes of surrounding bone and soft tissues. Postoperative complications such as dislocation, loosening of the components, and implant failure are more common. (4,10,35,36) However, the literature review also indicates that operative treatment can be beneficial if used in appropriate patients. We recommend only considering operative management in patients who have had treatment for their primary condition (syringomyelia), demonstrate little or no changes in glenohumeral joint destruction over a minimum 1 year period, have reasonable expectations regarding the goals of surgical management (mainly pain control), and who do not have persistent neurological symptoms and, of course, any evidence of infection. (4,10,20,35,43,44) Synovectomy, shoulder hemiarthroplasty, and reverse shoulder arthroplasty can all be considered based on symptoms and the degree of bone and soft tissue compromise. We caution against the use of aTSA in this population, as two of the three aTSAs identified in our literature search ultimately required conversion to a rTSA for early failure. In our 59-year-old patient, we are considering staged rTSAs after her recovery from treatment of the underlying syringomyelia.

Pharmaceuticals may ultimately play a greater role in the management of NAGHJ as our understanding of the pathophysiology behind Charcotjoints continues to evolve. The importance of the RANK-RANKL in initiating bony absorption and OPGs inhibitor action on this pathway may ultimately open up more therapeutic targets for non-operative management. (44) However, it remains to be seen if therapies like OPG have a role in maintaining bone stock and anatomy, especially in the context of syringomyelia. (47) A better understanding of the proinflammatory cytokines that activate the RANKL pathway may ultimately identify alternative medications to prevent further joint destruction, such as glucocorticoids or TNFa inhibitors. (14) However, the bulk of the above research is being performed using a diabetic NA model, which rarely affects the shoulder. As such, caution should remain when attempting to extrapolate any future findings to NAGHJ.

Conclusion

Neuropathic arthropathy of the glenohumeral joint can be a rapidly destructive and debilitating condition. Early identification and diagnosis is considered critical to the proper management of this condition. This review identified relatively poor quality evidence to recommend either non-operative or operative interventions. Nonetheless, we still believe an initial attempt at anti-inflammatory medications, physical therapy, bracing, or joint injections may provide satisfactory results once the underlying cause of the disease is addressed. In select patients, some evidence suggests that arthroplasty of the glenohumeral joint can lead to satisfactory results with a relatively high complication rate.

Disclosure Statement

None of the authors have a financial or proprietary interest in the subject matter or materials discussed, including, but not limited to, employment, consultancies, stock ownership, honoraria, and paid expert testimony.

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Lauren Santiesteban, MD, Brent Mollon, MD, FRCSC, and Joseph D. Zuckerman, MD

Lauren Santiesteban, MD, Brent Mollon, MD, FRCSC, and Joseph D. Zuckerman, MD, Department of Orthopedic Surgery, NYU Langone Orthopedic Hospital, NYU Langone Health, New York, New York, USA.

Correspondence: Joseph D. Zuckerman, MD, Department of Orthopedic Surgery, NYU Langone Orthopedic Hospital, NYU Langone Health, 301 East 17th Street, New York, New York 10003, USA; joseph.zuckerman@nyumc.org.

Caption: Figure 1 RANK-RANKL pathway.

Caption: Figure 2 Anterior-posterior, axillary, and scapular Y views of left shoulder.

Caption: Figure 3 Anterior-posterior, axillary, and scapular Y views of right shoulder.

Caption: Figure 4 Sagittal view of spinal MRI demonstrating large syrinx from C1/C2 to T10/T11.
Table 1 Non-Operative Management of Neuropathic Arthropathy of the
Glenohumeral Joint

               No. of
Author         pts     Age & Sex     Laterality  Etiology

Panagariya     1       62 y.o. M     L,R         Syringomyelia
& Sharma
Gaskins et     1       52 y.o. F     R           Syringomyelia
al.
(*) Grahovac   1       62 y.o. F     L           Syringomyelia
et al.
Butala et al.  1       53 y.o. F     L           Syringomyelia
Kuur           1/2     39 y.o. M     L           Syringomyelia
Atalar et al.  3/5     47 y.o. F     R>L         Syringomyelia
                       35 y.o. F     L           Syringomyelia
                       54 y.o. F     L           Syringomyelia
Hatzis et al.  3/6     66 y.o. M     R           Syringomyelia
                       29 y.o. M     L           Syringomyelia
                       53 y.o. M     R           Chronic
                                                 alcoholism
Clayton &      1       77 y.o. F     R           T2DM
Taylor
Panda et al.   1       56 y.o. M     L           Syringomyelia
Ruette et al.  3       3  68y.o. M   R           Syringomyelia
                        46 y.o. M    L           Syringomyelia
                        34 y.o. M    L           Syringomyelia
Kumar et al.   1       38 y.o. F     R           Syringomyelia
Ekim &         1       54 y.o. M     R           Syringomyelia
Armagan
Rao et al.     1       40 y.o. M     R           No clear etiology

               ROM at
Author         Presentation          Treatment

Panagariya     FF (R): 40[degrees]   "Custom made shoulder abduction
& Sharma       IR (R): Sacrum        brace" and physical therapy
               AFF (L): 30[degrees]
               IR (L): Sacrum
Gaskins et     FF: 50[degrees]       Subacromial steroid injection,
al.            ER: none              Decompression and T1-T2 laminectomy
               IR: none
(*) Grahovac                         NSAIDs
et al.                               Sub-occipital craniotomy and C1-C3
                                     laminectomy
Butala et al.  FF: 80[degrees]       NSAIDs, protective joint education,
               ER: 50[degrees]       physical therapy
               IR: 20[degrees]
Kuur                                 Physical therapy of L shoulder
Atalar et al.  FF (R): 95[degrees]   NSAIDs
               ER (R): 30[degrees]   (Posterior fossa decompression
               IR (R): buttock       performed 20 years prior to visit)
               FF: 30[degrees]       Posterior fossa decompression and
               ER: none              laminectomy,
               IR: none              Zoledronic acid x 1 year (then
                                     discontinued),
                                     Repeat decompression
               FF: 10[degrees]       NSAIDs
               ER: none              Hx of laminectomy and decompression
               IR: none              x 2 + Baclofen tx
Hatzis et al.  FF: 40[degrees]       NSAIDs, physical therapy
               ER: 30[degrees]
               IR: sacrum
               FF: 40[degrees]       Passive exercises
               ER: 25[degrees]
               IR:L5
               FF: 40[degrees]       Passive and strengthening exercise
               ER: 20[degrees]
               IR:L5
Clayton &      Limited AROM          NSAIDs
Taylor
Panda et al.                         Immobilization, reduction of trauma
Ruette et al.  FF: 45[degrees]       Physical therapy
               ER:0[degrees]
               IR: sacrum
               FF: 80[degrees]       Physical therapy and orthosis
                                     Joint debridement for later septic
                                     joint
               FF: 85[degrees]       Decompression, physical therapy
Kumar et al.   Restricted shoulder   NSAIDs, passive exercises, sling
               movements
Ekim &                               Rehabilitation and physical
Armagan                              therapy
Rao et al.                           Physical therapy

Author         Follow-up          Results

Panagariya     2 yrs              Remained asymptomatic, able to
& Sharma                          perform ADLs
Gaskins et     2 yrs              80% improvement in pain
al.
(*) Grahovac   5 yrs              No progression of syringomyelia
et al.
Butala et al.  6 mo               60% reduction in her symptoms
Kuur           2 yrs              Patient satisfied with ability to
Atalar et al.  1 yr               use arm without limitations
               3 yrs              FF (R): 100[degrees]
               13 mo              ER (R): 40[degrees]
                                  IR (R): lumbar region
                                  FF: 80[degrees]
                                  ER: none
                                  IR: lower lumbar region
                                  Continues to have pain in L
                                  shoulder at rest and with movement
                                  FF: 30[degrees]
                                  ER: none
                                  IR: lower lumbar region
Hatzis et al.                     Improved shoulder pain and swelling
Clayton &      13 mo
Taylor                            No change in functionality or pain
Panda et al.
Ruette et al.
               Improvement in
               pain and mobility
Kumar et al.
Ekim &
Armagan
Rao et al.

All measurements reflect active ROM. FF: active forward
flexion/elevation; ER: external rotation; IR: internal rotation;
M: male; F: female; (R): right; (L): left. (*) Grahovac et al.: authors
state they recommended arthrodesis although unclear if patients
underwent this procedure.

Table 2 Operative Management of Neuropathic Arthropathy of the
Glenohumeral Joint

                   No. of  Age&
Author             pts     Sex        Laterality  Etiology

Crowther &         1       47 y.o. F  R           Syringomyelia
Bell
Matsuhasi          3       54 y.o. F  R           Syringomyelia
etal.
                           55 y.o. M  R           Syringomyelia
                           64 y.o. F  R           Syringomyelia
Ueblacker          1       62 y.o. F  R,L         Syringomyelia
etal.
(*) Atalar et al.  1/5     46 y.o. M  R           Syringomyelia
Kuur               1/2     43 y.o. M  R
Ruette et al.      1/3     46 y.o. M  L           Syringomyelia
Gomez              2       39 y.o. M  L           Syringomyelia +
Rodriguez                                         Septic arthritis
etal.                      59 y.o. F  R           Syringomyelia +
                                                  Septic arthritis
Alai et al.        1       49 y.o. M  R           Syringomyelia
Richards           2/3     54 y.o. F  L           Syringomyelia
etal.
                           51y.o  F   R           Syringomyelia

                   ROM at
Author             Presentation

Crowther &         FF: 100[degrees]
Bell
Matsuhasi          FF: 45[degrees]
etal.              ER: -20[degrees]
                   IR: buttock
                   FF:15[degrees]
                   ER: 15[degrees]
                   IR:L3
                   FF: 45[degrees]
                   ER:0[degrees]
                   IR: buttock
Ueblacker          FF (R): 30[degrees]
etal.              ER (R): 0[degrees]
                   IR (R): 10[degrees]
                   FF (L): 20[degrees]
                   ER (L): 0[degrees]
                   IR(L): 15[degrees]
(*) Atalar et al.  FF: 30[degrees]
                   ER: none
                   IR: none
Kuur
Ruette et al.      FF: 80[degrees]
Gomez              Limited AROM
Rodriguez
etal.              Limited AROM
                   Abd.: <15[degrees]
                   ER: <5[degrees]
Alai et al.        FF: 90[degrees]
                   ER: none
                   IR: none
Richards
etal.
                   FF: 45[degrees]

Author             Treatment                              Follow-up

Crowther &         Hx of drainage of syrinx x2             2 yrs
Bell               Hemiarthroplasty with subscapularis
                   tenotomy
Matsuhasi          Syrinx decompression followed by       10 yrs
etal.              hemiarthroplsty with rotator cuff
                   repair
                   Syrinx decompression followed by        8 yrs
                   hemiarthroplsty with rotator cuff
                   repair
                   Syrinx decompression followed by        8 yrs
                   hemiarthroplsty with rotator cuff
                   repair
Ueblacker          Staged bilateral reverse total          2 yrs
etal.              shoulder arthroplasties
                   Post-op treatment: physical therapy
(*) Atalar et al.  Debridement and resection              31 mo
                   arthroplasty
                   Followed by tumor prosthesis
                   Removal of tumor prosthesis 2/2
                   infection
Kuur               Arthrodesis                            14 yrs
Ruette et al.      Physical therapy and orthosis
                   Joint debridement for later septic
                   joint
Gomez              Surgical drainage & debridement,        2 yrs
Rodriguez          vancomycin and cloxacillin
etal.              Surgical drainage & debridement,       NA
                   vancomycin and rifampin
Alai et al.        Neurolysis of right infraclavicular     5 yrs
                   brachial plexus and synovectomy,
                   Decompression
                                                           7 yrs
Richards           Glenoid osteotomy w/ bone graft x2,
etal.              carpal tunnel release, decompression,
                   cervical laminectomy, and shunt
                   placement
                   Putti-Platt procedure

Author             Results

Crowther &         FF: 140[degrees]
Bell               ER: 50[degrees]
                   Mild pain with activity
Matsuhasi          FF: 110[degrees]
etal.              ER: 20[degrees]
                   IR: T10
                   Decreased pain
                   FF: 80[degrees]
                   ER: 20[degrees]
                   IR:L3
                   FF: 120[degrees]
                   ER: 20[degrees]
                   IR: T12
Ueblacker          FF(R): 110[degrees]
etal.              ER (R): 45[degrees]
                   IR (R): 80[degrees]
                   FF(L): 110[degrees]
                   ER (L): 70[degrees]
                   IR (L): 80[degrees]
                   Patient satisfied, no pain, improvement
                   in stability
(*) Atalar et al.  FF: 20[degrees]
                   ER: none
                   IR: none
Kuur               Working full-time as laborer and
                   remained asymptomatic
Ruette et al.
Gomez              Poor left shoulder function
Rodriguez
etal.              Poor right shoulder function
                   Abd.: < 20[degrees]
                   ER: < 10[degrees]
Alai et al.        FF: 140[degrees]
                   ER: 80[degrees]
                   Minimal pain
                   FF: 70[degrees]
                   ER: 20[degrees]
                   Course complicated by acromioclavicular
                   joint cyst
                   Pain varied
Richards           Continues to have pain and decreased
etal.              mobility
                   No change in her symptoms

FF: active forward flexion/elevation; ER: external rotation; IR:
internal rotation; M: male; F: female; (R): right; (L): left.
(*) Atalar et al.: patient underwent shoulder debridement, resection
arthroplasty, followed by placement of tumor prosthesis at outside
hospital.


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Author:Santiesteban, Lauren; Mollon, Brent; Zuckerman, Joseph D.
Publication:Bulletin of the NYU Hospital for Joint Diseases
Article Type:Report
Date:Apr 1, 2018
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