Document Type

Article

Publication Date

3-2025

Identifier

DOI: 10.1016/j.arrct.2024.100416

Abstract

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.

Journal Title

Archives of Rehabilitation Research and Clinical Translation

Volume

7

Issue

1

First Page

100416

Keywords

Connective tissue disease; Pain; Pediatrics; Physical therapy; Rehabilitation

Comments

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Publisher's Link: https://www.archives-rrct.org/article/S2590-1095(24)00129-0/fulltext

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