EN PL
REVIEW PAPER
Figure from article: Ehlers-Danlos syndrome and...
 
KEYWORDS
TOPICS
ABSTRACT
Ehlers-Danlos syndromes (EDS) are complex connective tissue disorders, with hypermobile EDS (hEDS) being the most common subtype, characterised by joint hypermobility. Diagnosis relies primarily on clinical assessment and family history, as definitive genetic testing is not yet available. Patients with hEDS frequently experience shoulder instability, often involving bidirectional patterns, posing substantial management challenges. Conservative management remains the cornerstone of treatment for hEDS-related shoulder instability. Evidence supports structured, progressive physiotherapy programmes that emphasise strengthening of dynamic stabilisers, including higher-load exercise where tolerated, alongside neuromuscular and proprioceptive training. Progressive home-based programmes with monitoring and selective adjunctive interventions, such as compression garments and taping for short-term improvements in pain or range of motion, have demonstrated feasibility and potential benefit, enhancing accessibility and long-term adherence. Surgical interventions, including arthroscopic techniques such as posterior glenohumeral capsular reconstruction with allografts, and open procedures such as inferior capsular shift or shoulder arthroplasty, have shown promise in carefully selected patients. Long-term outcomes remain limited, and surgical decision-making should be individualised, performed in specialised centres, and integrated with structured postoperative rehabilitation. This narrative review summarises and compares available surgical options and evaluates current evidence supporting conservative management for shoulder instability in hEDS.
REFERENCES (41)
1.
Nourissat G, Vigan M, Hamonet C, et al. Diagnosis of Ehlers- Danlos syndrome after a first shoulder dislocation. J Shoulder Elbow Surg 2018; 27: 65–69, DOI: 10.1016/J.JSE.2017.05.028.
 
2.
Demmler JC, Atkinson MD, Reinhold EJ, et al. Diagnosed prevalence of Ehlers-Danlos syndrome and hypermobility spectrum disorder in Wales, UK: a national electronic cohort study and case-control comparison. BMJ Open 2019; 9: e031365, DOI: 10.1136/BMJOPEN-2019-031365.
 
3.
Malfait F, Francomano C, Byers P, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet 2017; 175: 8–26, DOI: 10.1002/AJMG.C.31552.
 
4.
Song B, Yeh P, Harrell J. Systemic manifestations of Ehlers- Danlos syndrome. Proc (Bayl Univ Med Cent) 2020; 34: 49–53, DOI: 10.1080/08998280.2020.1805714.
 
5.
Tinkle B, Castori M, Berglund B, et al. Hypermobile Ehlers- Danlos syndrome (a.k.a. Ehlers-Danlos Syndrome type III and Ehlers-Danlos syndrome hypermobility type): clinical description and natural history. Am J Med Genet C Semin Med Genet 2017; 175: 48–69, DOI: 10.1002/AJMG.C.31538.
 
6.
Tschernogobow A. Cutis laxa. Moscow Dermatologic and Venereologic Society. Monatsh Prakt Dermatol 1892; 14–76.
 
7.
Ehlers E. Cutis Laxa, Neigung Zu Hemorrhagien in Der Haut, Lockerung Mehrerer Artikulation. Dermatologie 1901; 8: 173–178.
 
8.
Danlos HA. Un cas de cutis laxa avec tumeurs par comtusion chronique des coudes et des genoux. Bull Soc Fr Dermatol Syphiligr 1908; 19: 70-72.
 
9.
Bicca Ede B, de Almeida FB, Pinto GM et al. Classical Ehlers-Danlos syndrome: clinical, histological and ultrastructural aspects. An Bras Dermatol 2011; 86: 164–167, DOI: 10.1590/S0365-05962011000700043.
 
10.
Homere A, Bolia IK, Juhan T, et al. Surgical management of shoulder and knee instability in patients with Ehlers- Danlos syndrome: joint hypermobility syndrome. Clin Orthop Surg 2020; 12: 279, DOI: 10.4055/CIOS20103.
 
11.
Johannessen EC, Reiten HS, Løvaas H, et al. Shoulder function, pain and health related quality of life in adults with joint hypermobility syndrome/Ehlers-Danlos syndrome-hypermobility type. Disabil Rehabil 2016; 38: 1382–1390, DOI: 10.3109/ 09638288.2015.1102336.
 
12.
Ericson WB, Wolman R. Orthopaedic management of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet 2017; 175: 188–194, DOI: 10.1002/AJMG.C.31551.
 
13.
Abihssira S, Benistan K, Nourissat G. Functional benefit of joint surgery in patients with non-vascular Ehlers-Danlos syndrome: results of a retrospective study. Orphanet J Rare Dis 2024; 19: 351, DOI: 10.1186/S13023-024-03261-3.
 
14.
Legato JM, O’Connell M, Fuller DA. Shoulder arthrodesis. J Orthop Trauma 2018; 32: S4–S5, DOI: 10.1097/BOT.0000000000001212.
 
15.
Rogers T, Werthel JD, Crowe MM, et al. Shoulder arthroplasty is a viable option in patients with Ehlers-Danlos syndrome. J Shoulder Elbow Surg 2021; 30: 2484–2490, DOI: 10.1016/ J.JSE.2021.03.146.
 
16.
Kishan A, Thomas K, Kubsad S, et al. Trends in surgical procedures for shoulder instability among patients with Ehlers- Danlos syndrome or joint hypermobility syndrome. JSES Int 2024; 8: 1164–1168, DOI: 10.1016/J.JSEINT.2024.08.178.
 
17.
Karpyshyn J, Gordey EE, Coady CM, Wong IH. Posterior glenohumeral capsular reconstruction using an acellular dermal allograft. Arthrosc Tech 2018; 7: e739, DOI: 10.1016/J.EATS.2018.03.011.
 
18.
Gallagher F, Wong IHB. Anterior capsule augmentation and posterior glenohumeral capsular reconstruction with human dermal allograft for multidirectional shoulder instability. Arthrosc Tech 2020; 9: e657–e662, DOI: 10.1016/J.EATS.2020.01.020.
 
19.
Mihata T, Lee TQ, Watanabe C, et al. Clinical results of arthroscopic superior capsule reconstruction for irreparable rotator cuff tears. Arthroscopy 2013; 29: 459–470, DOI: 10.1016/ J.ARTHRO.2012.10.022.
 
20.
Petri M, Warth RJ, Horan MP, et al. Outcomes after open revision repair of massive rotator cuff tears with biologic patch augmentation. Arthroscopy 2016; 32: 1752–1760, DOI: 10.1016/ J.ARTHRO.2016.01.037.
 
21.
Pogorzelski J, Hussain ZB, Lebus GF, et al. Anterior capsular reconstruction for irreparable subscapularis tears. Arthrosc Tech 2017; 6: e951–e958, DOI: 10.1016/J.EATS.2017.03.008.
 
22.
Housset V, Nourissat G. Arthroscopic capsular plication for multidirectional shoulder instability in hypermobile Ehlers-Danlos syndrome patients. Arthrosc Tech 2021; 10: e2767, DOI: 10.1016/ J.EATS.2021.08.024.
 
23.
Misamore GW, Sallay PI, Didelot W. A longitudinal study of patients with multidirectional instability of the shoulder with seven- to ten-year follow-up. J. Shoulder Elbow Surg 2005; 14: 466–470, DOI: 10.1016/J.JSE.2004.11.006.
 
24.
Boulu X, Karam JD, Schmidt J. Compression garments in hypermobile Ehlers-Danlos syndrome and hypermobility spectrum disorders: a retrospective cohort study. BMC Musculoskelet Disord 2025; 26: 1066, DOI: 10.1186/S12891-025-09318-Z.
 
25.
Tudini F, Jordon M, Levine D, et al. Evaluating the effects of two different kinesiology taping techniques on shoulder range of motion and proprioception in patients with hypermobile Ehlers-Danlos syndrome: a randomized controlled trial. Front Rehabil Sci 2024; 5: 1383551, DOI: 10.3389/fresc.2024.1383551.
 
26.
Armstrong MD, Smith B, Coady C, Wong IH. Arthroscopic anterior and posterior glenoid bone augmentation with capsular plication for Ehlers-Danlos syndrome with multidirectional instability. Arthrosc Tech 2018; 7: e541, DOI: 10.1016/ J.EATS.2018.01.011.
 
27.
Provencher MT, Frank RM, Golijanin P, et al. Distal tibia allograft glenoid reconstruction in recurrent anterior shoulder instability: clinical and radiographic outcomes. Arthroscopy 2017; 33: 891–897, DOI: 10.1016/J.ARTHRO.2016.09.029.
 
28.
Khawaja SR, Hussain ZB, Cooke H, et al. Dynamically rebalancing the unstable shoulder in Ehlers-Danlos syndrome: latissimus dorsi transfer for posterior shoulder instability. JSES Int 2024; 8: 954–962, DOI: 10.1016/j.jseint.2024.05.008.
 
29.
Anastasopoulos PP, Alexiadis G, Spyridonos S, Fandridis E. Latissimus dorsi transfer in posterior irreparable rotator cuff tears. Open Orthop J 2017; 11: 77–94, DOI: 10.2174/1874325001711010077.
 
30.
Broida SE, Sweeney AP, Gottschalk MB, et al. Clinical outcomes of latissimus dorsi tendon transfer and superior capsular reconstruction for irreparable rotator cuff tears: a systematic review. Eur J Orthop Surg Traumatol 2022; 32: 1023–1043, DOI: 10.1007/s00590-021-03046-5.
 
31.
Vavken P, Tepolt FA, Kocher MS. Open inferior capsular shift for multidirectional shoulder instability in adolescents with generalized ligamentous hyperlaxity or Ehlers-Danlos syndrome. J Shoulder Elbow Surg 2016; 25: 907–912, DOI: 10.1016/J.JSE.2015.10.010.
 
32.
Neer CS, Foster CR. Inferior capsular shift for involuntary inferior and multidirectional instability of the shoulder. A preliminary report. J Bone Joint Surg Am 1980; 62: 897–908.
 
33.
Bacle G, Nové-Josserand L, Garaud P, Walch G. Long-term outcomes of reverse total shoulder arthroplasty. J Bone Joint Surg Am 2017; 99: 454–461, DOI: 10.2106/JBJS.16.00223.
 
34.
Simovitch RW, Friedman RJ, Cheung EV, et al. Rate of improvement in clinical outcomes with anatomic and reverse total shoulder arthroplasty. J Bone Joint Surg Am 2017; 99: 1801–1811, DOI: 10.2106/JBJS.16.01387.
 
35.
Clare DJ, Wirth MA, Groh GI, Rockwood CA. Shoulder arthrodesis. J Bone Joint Surg Am 2001; 83: 593–600, DOI: 10.2106/00004623-200104000-00018.
 
36.
Chaudhury S, Gasinu S, Rodeo SA. Bilateral anterior and posterior glenohumeral stabilization using achilles tendon allograft augmentation in a patient with Ehlers-Danlos syndrome. J Shoulder Elbow Surg 2012; 21: e1–e5, DOI: 10.1016/j.jse.2011.10.033.
 
37.
Higo A, Palmer S, Liaghat B, et al. The effectiveness of conservative interventions on pain, function, and quality of life in adults with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorders and shoulder symptoms: a systematic review. Arch Rehabil Res Clin Transl 2024; 6: 100360, DOI: 10.1016/j.arrct.2024.100360.
 
38.
Liaghat B, Pedersen JR, Husted RS, et al. Diagnosis, prevention and treatment of common shoulder injuries in sport: grading the evidence – a statement paper commissioned by the Danish Society of Sports Physical Therapy (DSSF). Br J Sports Med 2023; 57: 408–416, DOI: 10.1136/BJSPORTS-2022-105674.
 
39.
Spanhove V, De Wandele I, Malfait F, et al. Home-based exercise therapy for treating shoulder instability in patients with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorders. A randomized trial. Disabil Rehabil 2023; 45: 1811–1821, DOI: 10.1080/09638288.2022.2076932.
 
40.
Chaléat-Valayer E, Denis A, Zelmar A, et al. VETCOSED study: efficacy of compressive garments for patients with hypermobile Ehlers-Danlos syndrome on shoulder stability and muscles strength. Disabil Rehabil 2022; 44: 3165–3172, DOI: 10.1080/09638288.2020.1860142.
 
41.
Benistan K, Foy M, Gillas F, et al. Effects of compression garments on balance in hypermobile Ehlers-Danlos syndrome: a randomized controlled trial. Disabil Rehabil 2024; 46: 1841–1850, DOI: 10.1080/09638288.2023.2209742.
 
Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
eISSN:2084-9834
ISSN:0034-6233
Journals System - logo
Scroll to top