Shoulder complications in sickle cell disease: Challenges, management strategies, and future directions

Table 1 Cruess classification of shoulder avascular necrosis

Stage	Radiographic features	Additional features/details
Stage 1	Radiographs appear normal	Magnetic resonance imaging detects early changes in the bone marrow signal, indicating the onset of the disease without structural alterations
Stage 2	Reparative process with sclerotic or mottled osteopenia	The sphericity of the humeral head is preserved
Stage 3	Appearance of the "crescent sign"	Subchondral radiolucent line signifies a subchondral fracture, with minor joint surface depressions due to localized subchondral collapse
Stage 4	Complete collapse of the articular surface	Destruction of the trabecular pattern and compromise of joint structural integrity
Stage 5	Articular changes in the glenoid	Joint incongruity with osteo-cartilaginous flaps detaching and becoming loose bodies within the joint

Table 2 Arthroplasty management in sickle cell disease studies summary

Ref.	Population	Procedure (n)	Follow-up	Outcomes	Complications	Notes
Marigi et al[67]	17 shoulders (SCD) compared with 34 shoulders in a matched cohort of non-SCD patients	HA (9), TSA (7), RSA (1)	5.9 years	VAS: 9.1→3.8a; ASES: 48.6→73.5a; ROM: FE: 95° to 128°a, ER: 24° → 38°a, IR score:3.2 → 5.2a ASES 48.6 → 73.5a. significant improvement in strength (FE: 4.2 to 4.8a, ER: 4.1 → 4.7a, IR: 4.1 → 4.7a)	29% complication rate (glenoid loosening, RCT, hematoma, and fracture); 18% reoperation rate	Compared with non-SCD, SCD group has higher pre and post pain VAS 9.1 vs 7.4b, 3.8 vs 1.3b and higher complication rate 29% vs 12%
Colegate-Stone et al[65]	7 shoulders (Stage 4 HHAVN)	Glenoid-sparing HA (2), RSA (1)	NA	VAS: 9.5→4.1; satisfaction: 8.5/10	No significant complications noted in arthroplasty subgroup	Glenoid-sparing HA for younger patients with intact rotator cuffs and minimal glenoid changes, while RSA was indicated for older, lower-demand patients with rotator cuff deficiency
Kennon et al[7]	9 shoulders (7 SCD)	Resurfacing (7) for Stages II and III, TSA (2) for Stage IV	2-year	UCLA: 9.6→29b; ASES: 19.7→81.4a; Constant: 28→87a	3 resurfacing cases required revision (glenoid wear, stiffness, subscapular insufficiency)	Further studies needed to assess the long-term effectiveness of humeral head resurfacing
Lau et al[15]	8 shoulders (all SCD)	HA (7), TSA (1)	NA	Varied (excellent: 2, acceptable: 4, poor: 2)	Sickle cell crises, stiffness, glenoid wear	Excellent: 2 patients with high ASES scores with excellent pain relief, full functional recovery, and high satisfaction. Acceptable: 4 patients demonstrating improved function but little to no improvement in pain. Poor: 2 patients experienced decreased ASES scores, reduced activities of daily living (ADL) scores, and no pain relief
Ristow et al[70]	29 shoulders (8 SCD)	HA (NA), TSA (NA)	3.9 years	ASES: 27.3→84.2b; UCLA: 11.5→25b; Constant: 42.6→96.6b	Combined complication rate: 6.9%	HA for less glenoid wear while TSA for more arthritic glenoid. TSA tended toward better outcomes in functionality and pain relief
Feeley et al[71]	64 shoulders (4 SCD)	HA (2/37), TSA (2/27)	4.8 years	Improved ASES, L’Insalata, ROM, satisfaction (except one patient). No differences between HA and TSA in SCD subgroup	TSA: 22% (reoperation, loosening); HA: 5% (glenoid wear)	The SCD subgroup showed better scores compared to other etiologies, but these differences were not statistically significant, likely due to the small sample size

^aP < 0.001.

^bP < 0.05.

HHAVN: Humeral head avascular necrosis; FE: Forward elevation; ER: External rotation; IR: Internal rotation; SCD: Sickle cell disease; HA: Hemiarthroplasty; TSA: Total shoulder arthroplasty; RSA: Reverse shoulder arthroplasty; RCT: Rotator cuff tear; VAS: Visual analogue scale; ASES: American shoulder and elbow surgeon score; UCLA: University of California, Los Angeles shoulder score; ROM: Range of motion; VAS: Visual analogue scale.