Prevalence of Pain and Effect of Joint Strength on Perceived Physical Function in Pediatric Patients With Ehlers–Danlos Syndrome

Objective

To identify how pediatric patients with hypermobile-type Ehlers–Danlos syndrome (hEDS) present to physical therapy (PT) and better understand their musculoskeletal dysfunction.

Design

Patients were included in the study if they had a diagnosis of hEDS and at least 1 completed PT evaluation after diagnosis. A chart review was conducted to assess joint strength and pain characteristics at the initial PT assessment. Physical Function Mobility (PF-Mobility; 4 domains with 1-5 scale with higher score representative of better physical function) as part of the Patient-Reported Outcome Measurement Information System and pain intensity score (visual analog scale, 0-10 scale where a lower number indicates less pain) was completed by participants.

Setting

Midwestern tertiary care center.

Participants

Sixty-nine participants were included in the study with an average age of 16.4 years (SD 2.8) at the initial PT visit and 96% were female individuals.

Intervention

Not applicable.

Main Outcome Measures

Joint strength, pain intensity and location, and PF-Mobility.

Results

The knee was the most reported location with pain (68%) followed by the shoulder (46%), hip (41%), and back (39%). Average pain intensity was 5.0 (SD 1.8), joint strength scores ranged from 8.2 (SD 0.9) to 9.9 (SD 0.4), and the PF-Mobility mean was 3.3 (SD 0.6). No correlation was noted between joint pain intensity and strength at the joint.

Conclusions

Although the participants perceived poor mobility and significant pain, PT-assessed strength did not reveal significant muscle weakness. Additional work is needed to explore this discrepancy, including altered biomechanics, evaluation methods, and nonmusculoskeletal factors such as neuropathic and psychological components.