Trends in surgical procedures for shoulder instability among patients with Ehlers-Danlos syndrome or joint hypermobility syndrome

Abstract

Background

Joint hypermobility syndrome (JHS) and Ehlers-Danlos Syndrome (EDS) are connective tissue disorders characterized by increased joint laxity, affecting musculoskeletal health and quality of life. In this study, we explored recent trends in surgical treatment of shoulder instability among patients with these disorders.

Methods

We searched the PearlDiver Mariner database, which includes deidentified US all-payer claims data from 2010 to 2020. We used procedure and diagnostic codes for EDS and JHS to select patients. The primary outcome was the yearly trend in relative utilization of the following 4 shoulder instability procedures: arthroscopic stabilization, Latarjet coracoid transfer, open capsulolabral repair, and open capsulolabral shift.

Results

Among 109,274 patients with EDS and 453,885 with JHS, 3.4% and 0.8% underwent shoulder instability procedures, respectively. Arthroscopic stabilization was the predominant treatment, with a mean utilization rate of 78% for EDS and 83% for JHS. Notably, the age at surgery increased for EDS patients but decreased for JHS patients. Female patients represented large proportions of those undergoing procedures in both the EDS group (83%) and the JHS group (77%).

Conclusions

Our findings indicate a consistent preference for arthroscopic stabilization in treating shoulder instability in patients with EDS and JHS. The trends in age suggest shifts in treatment strategies, possibly influenced by advancements in nonoperative interventions or varying symptom severity. The higher proportion of female patients aligns with the known prevalence of connective tissue disorders in women. Future research should explore outcomes, complications, and specific EDS subtypes to guide optimal treatment strategies for these challenging connective tissue disorders.

Joint hypermobility occurs when ligaments and connective tissues surrounding a synovial joint permit greater than normal range of motion.^5,11 Although the degree of hypermobility depends on genetic factors, approximately 30% of the world’s population has generalized joint hypermobility.^5,12 Nearly 10% of these individuals are symptomatic and experience orthopedic conditions, such as arthralgia, ankle sprains, and subluxations.¹ According to the Beighton criteria, hypermobile joints that cause prolonged arthralgia and recurrent complications and involve different organ systems are classified as joint hypermobility syndrome (JHS).¹¹ JHS can reduce quality of life, especially in young children and adolescents and can impede motor and social development. Features of JHS are also seen in heritable disorders of connective tissue, such as Marfan syndrome and Ehlers-Danlos Syndrome (EDS).

EDS refers to a group of heritable connective tissue disorders caused by aberrant genetic expression of collagen I, III, V, and XII and enzymes involved in the extracellular matrix.⁹ Nineteen of the involved genes create a constellation of clinical manifestations, leading to the creation of 13 subtypes of EDS. Although each subtype has a unique clinical presentation, they all share features of abnormal skin texture, hypermobile joints, and vascular fragility.^9,14

The glenohumeral joint is one of the most commonly dislocated joints, and this holds true for patients with EDS.^2,17 The static stability of the shoulder joint provided by the glenoid labrum, glenohumeral ligaments, and coracohumeral ligaments is compromised in EDS.^2,15,17 Diffuse laxity in stabilizing ligaments leads to chronic shoulder instability and recurrent shoulder dislocations. Shoulder stabilization procedures can provide an effective treatment for joint instability, though little is known regarding surgical trends in patients with joint hypermobility. Riff et al¹³ documented an increase in the use of arthroscopic stabilization and Latarjet procedures from 2007 to 2015, although their study did not focus specifically on shoulder instability in patients with EDS.

In this study, we analyzed trends in surgical shoulder instability procedures for patients with EDS or JHS. Understanding such trends is crucial for tailoring treatment approaches to the unique challenges posed by these heritable connective tissue disorders. By examining how preferences for specific procedures have evolved, we can gain insights into the safety and effectiveness of different interventions and identify which methods align best with the needs and complexities associated with EDS and JHS. This knowledge is essential for improving resource allocation and ensuring that health care resources and expertise are used effectively. Additionally, documenting these trends helps identify gaps in care, driving future research to address these gaps and refine treatment strategies. Ultimately, a thorough understanding of these trends enables more informed decision-making, potentially improving patient outcomes and advancing efforts to manage complex connective tissue disorders.

Materials and methods

Data source and patient selection

The PearlDiver Mariner database (PearlDiver Technologies, Colorado Springs, CO, USA) contains deidentified US all-payer claims data. As a result, this study was deemed exempt from institutional review board approval (IRB00387181). We queried the database from January 2010 to December 2020 using Current Procedural Terminology, International Classification of Diseases, Ninth Revision, and International Classification of Diseases, Tenth Revision codes to identify patients with EDS or JHS (Supplementary Table S1). The first instance of each procedure was queried for each patient, enabling us to target only primary procedures. We also sought to include patients with Marfan syndrome; however, the yearly sample sizes were too small to enable trend modeling, so these patients were excluded.

Outcome measures

The primary outcome measure was the yearly trend in the relative utilization of the following 4 shoulder instability procedures: 1) arthroscopic stabilization (which also encompasses capsular plication), 2) Latarjet coracoid transfer, 3) open capsulolabral repair, and 4) open capsulolabral shift. Because of limitations of the database we used, we were unable to assess which procedures were used for which directions of instability. We defined “relative utilization” as the percentage utilization of a given procedure relative to the total utilization of all 4 shoulder instability procedures within a given year. In addition to quantifying procedural trends, analysis was conducted in the cohorts stratified by age and sex.

Patient characteristics

We identified 109,274 patients with EDS and 453,885 with JHS. Of the patients diagnosed with EDS and JHS, 3679 (3.4%) patients and 3653 (0.8%) patients underwent shoulder instability procedures, respectively. Female patients represented a large proportion of the patients undergoing procedures in both the EDS group (83%) and the JHS group (77%). The largest proportion of patients undergoing these procedures was younger than 25 years in both the EDS group (44%) and the JHS group (41%).

Statistical analysis

We used linear regression for P value determination, with an alpha level set at 0.05. To quantify changes in procedure utilization, we calculated the compounded annual growth rate (CAGR) by using the following equation:

$${(\mathrm{Y}2/\mathrm{Y}1)}^{1/\left(\mathrm{Y}2-\mathrm{Y}1\right)-1}\text{,}$$

where Y1 is the first year of the analysis and Y2 is the final year. The CAGR is validated for the analysis of annual trends because of its ability to reduce the impact of short-term fluctuations on overall trends. Trend analyses were performed using R software (R version 4.0.3; R Foundation for Statistical Computing, Vienna, Austria) provided within the PearlDiver environment.

Results

Procedure utilization in EDS

Arthroscopic stabilization was the predominant treatment, with a mean utilization rate of 78% (95% confidence interval [CI]: 77%, 79%). Utilization rates for the other 3 procedures were 9.8% for open capsulolabral shift (95% CI: 8.8%, 11%), 7.0% for Latarjet coracoid transfer (95% CI: 6.5%, 7.5%), and 5.3% for open capsulolabral repair (95% CI: 4.2%, 6.3%) (Fig. 1). No significant changes in utilization were observed for arthroscopic stabilization (CAGR: 0.3%, P = .99), Latarjet coracoid transfer (CAGR: −1.1%, P = .36), open capsulolabral repair (CAGR: −2.4%, P = .92), or open capsulolabral shift (CAGR: −0.21%, P = .52).

Figure 1.

Trends in utilization of shoulder instability procedures for patients in the United States with Ehlers- Danlos syndrome from 2010 to 2020. Compounded annual growth rates were 0.3% for arthroscopic stabilization, −1.1% for Latarjet coracoid transfer, −2.4% for open capsulolabral repair, and −0.2% for open capsulolabral shift.

Procedure utilization in JHS

Arthroscopic stabilization was predominant, with a utilization rate of 83% (95% CI: 81%, 85%). Similar to our findings for the EDS group, the utilization rates were 8% for open capsulolabral shift (95% CI: 6.1%, 9.9%), 4.6% for Latarjet coracoid transfer (95% CI: 4.0%, 5.1%), and 4.4% for open capsulolabral repair (95% CI: 3.6%, 5.1%) (Fig. 2). The utilization of both open capsulolabral shift (CAGR: −3.7%, P = .02) and open capsulolabral repair (CAGR: −4.6%, P < .01) showed significant declines during the study period. The utilization of arthroscopic stabilization showed a significant increase during the study period (CAGR: 0.7%, P = .01). No significant change in utilization was observed for Latarjet coracoid transfer (CAGR: −1.8%, P = .10).

Figure 2.

Trends in utilization of shoulder instability procedures for patients in the United States with joint hypermobility syndrome from 2010 to 2020. Compounded annual growth rates were 0.7% for arthroscopic stabilization, −1.8% for Latarjet coracoid transfer, −4.6% for open capsulolabral repair, and −3.7% for open capsulolabral shift.

Trends in age and sex

In the EDS group, the percentage of patients younger than 25 years decreased from 2010 to 2020 (CAGR: −4.9%, P = .001) (Table I). During the same period, the proportion of patients aged 25-35 increased (CAGR: 4.4%. P = .02), whereas the proportion of those older than 35 did not change significantly (CAGR: 3.5%, P = .07). In the JHS group, the percentage of patients older than 35 years decreased from 2010 to 2020 (CAGR: −4.6%, P < .001). During the same period, the proportions of patients younger than 25 and those aged 25-35 increased, with CAGRs of 2.2% and 3.2%, respectively (both, P = .02). In regard to sex, the proportion of females increased in the EDS group (CAGR: 1.1%, P < .001) and remained stable in the JHS group (CAGR: 0.29%, P = .93) (Table II).

Table I.

Trends in age of patients with hyperlaxity syndromes undergoing shoulder instability procedures from 2010 to 2020, PearlDiver Mariner database (PearlDiver Technologies, Colorado Springs, CO, USA).

Year	% By age group (y)
	Ehlers-Danlos syndrome			Unspecified hyperlaxity disorder
	<25	25-35	>35	<25	25-35	>35
2010	56	20	24	41	16	43
2011	50	25	25	33	21	46
2012	52	27	21	30	23	47
2013	48	35	18	38	20	42
2014	48	29	23	37	23	40
2015	45	30	26	36	25	40
2016	48	30	22	49	21	30
2017	46	28	27	52	23	26
2018	47	32	21	45	23	33
2019	40	31	29	40	29	31
2020	32	34	35	51	23	26
CAGR (%)	−4.9	4.4	3.5	2.2	3.2	−4.6
P value	.001	.02	.07	.02	.02	<.0001

CAGR, compound annual growth rate.

Table II.

Trends in sex of patients with hyperlaxity syndromes undergoing shoulder instability procedures from 2010 to 2020, PearlDiver Mariner database (PearlDiver Technologies, Colorado Springs, CO, USA).

Year	% By sex
	Ehlers-Danlos syndrome		Unspecified hyperlaxity disorder
	Male	Female	Male	Female
2010	24	76	27	73
2011	21	79	23	77
2012	23	77	20	80
2013	20	81	22	78
2014	18	82	24	76
2015	14	86	22	78
2016	15	85	23	77
2017	12	88	21	79
2018	14	87	24	76
2019	14	86	25	76
2020	15	85	24	76
CAGR (%)	−4.4	1.1	−0.8	0.3
P value		<.001		.93

CAGR, compound annual growth rate.

Discussion

We found arthroscopic stabilization to be by far the most common surgery performed for shoulder instability. As demonstrated in our study, open procedures are less commonly used. The wide spectrum of pathology and presentations of shoulder instability, as well as the range of surgical options, reiterates the need for careful consideration of surgical and patient factors before proceeding with surgery. Our study reinforces the concept that many patients and surgeons may first choose to proceed with arthroscopic stabilization, which encompasses various techniques, including capsular plication, labral repair, and other adjunct procedures tailored to the patient’s instability pattern, reserving open procedures as secondary options even in patients with EDS or JHS. The techniques for JHS and EDS may differ because of the specific ligamentous laxity and joint instability patterns in these conditions. Systematic reviews and meta-analyses comparing arthroscopic vs. open treatment of shoulder instability note similar rates of complications, recurrent instability, revision, and return to sport.^3,8 This study contributes to the understanding of surgical trends in patients with EDS and JHS, highlighting the predominance of arthroscopic stabilization. It is likely that some trends identified in the JHS population reflect broader advancements in surgical techniques for shoulder instability, as supported by studies such as that of Riff et al,¹³ who reported an increase in arthroscopic stabilization and Latarjet procedures in the general population.

A similar study by Haskoor et al⁷ evaluated trends in shoulder instability procedures in the general population and found lower utilization of arthroscopic repair in patients with EDS (78% [95% CI: 77%, 79%]) and JHS (83% [95% CI: 81%, 85%]) compared with 91% in the general population. Furthermore, we found higher utilization of Latarjet coracoid transfer in patients with EDS (7.0% [95% CI: 6.5%, 7.5%]) and JHS (4.6% [95% CI: 4.0%, 5.1%]), whereas Haskoor et al reported 3.5% in the general population. While arthroscopic stabilization remains the predominant instability procedure for patients with EDS and JHS, it is performed at much lower rates for these patients compared with the general population, suggesting that other considerations play a role in preoperative and intraoperative planning.

We also found that from 2010 to 2020, the age of EDS patients undergoing surgery increased modestly. This increase is likely attributable to several factors. The decision for surgical stabilization in patients with EDS may be influenced by multiple factors, including historical surgical failures, delayed care, and physician reluctance to operate in light of past outcomes. These factors, along with the effectiveness of physical therapy, contribute to the complex decision-making process for surgical intervention in this population. During this period, increasing evidence demonstrated the effectiveness of physical therapy for shoulder instability.¹⁶ Most patients with shoulder instability, including those with EDS or JHS, now undergo physical therapy for at least 6-9 months before surgical treatment is considered.⁴ Typically, physical therapy includes rotator cuff strengthening, proprioceptive training, and management of scapulothoracic dyskinesia and weakness. The importance of an ongoing maintenance program is also recognized to prevent recurrence.¹⁶

In contrast to the increasing age of surgically treated EDS patients, the average age of patients with JHS undergoing surgery decreased during the study period. This trend toward a younger age at surgery may reflect that surgeons are treating shoulder instability more aggressively.

Our study has several limitations. We used data from the PearlDiver Mariner dataset, which is based on national all-payer insurance claims. This dataset does not capture all relevant clinical details, and the accuracy of coding for EDS and hyperlaxity disorder diagnoses may be subject to error and variation. For example, Neer and Foster¹⁰ originally defined the condition as anterior and posterior instability with symptomatic subluxation or dislocation, along with involuntary inferior subluxation. More recently, multidirectional shoulder instability has been described as instability in 2 or 3 directions, with recent definitions requiring one of the directions being inferior.⁶ Other limitations are that, we did not differentiate EDS into subtypes, and the database lack information on nonoperative interventions and patient symptoms. These factors limited our ability to draw conclusions about the duration of nonoperative treatment and the observed age trends. Finally, we did not analyze outcomes or complications of the included treatment modalities; thus, we cannot characterize the effectiveness of these interventions.

Several strengths of our study should be noted. We analyzed a large cohort of patients diagnosed with EHS and hyperlaxity disorder during a period of 10 years. The inclusion of multiple interventions enabled a thorough investigation of trends in shoulder instability treatment within these patient populations. To our knowledge, no previous studies have described trends in the utilization of these procedures in these challenging-to-treat patient populations.

Conclusion

This analysis of trends in shoulder instability procedures among patients with EDS and hyperlaxity disorder during the past decade characterizes recent practice patterns and surgical patient demographic characteristics. Arthroscopic stabilization emerged as the predominant choice for addressing shoulder instability in both patient groups. The observed increase in the age of EDS patients suggests advancements in nonoperative interventions and preference for exhausting nonsurgical options before pursuing surgery. In contrast, hyperlaxity disorder patients may opt for surgery at an earlier age.

Acknowledgment

For editorial assistance, the authors thank Rachel Walden, MS, in the Editorial Services group of The Johns Hopkins Department of Orthopaedic Surgery.

Disclaimers:

Funding: No funding was received in support of this study.

Conflicts of interest: Dr. Srikumaran serves as a board or committee member for AAOS, American Shoulder and Elbow Surgeons, and IASES, reports stock or stock options from ROM3, Sonogen, and Tigon Medical, is a paid consultant for and receives intellectual property royalties from Fx Shoulder and Tigon Medical, receives other financial or material support from Arthrex, DePuy–a Johnson & Johnson Company, and Thieme; and has been a paid presenter or speaker for and received research support from Fx Shoulder. All the other authors, their immediate families, and any research foundations with which they are affiliated have not received any financial payments or other benefits from any commercial entity related to the subject of this article.