» Articles » PMID: 32967103

Pain Symptomatology and Management in Pediatric Ehlers-Danlos Syndrome: A Review

Overview
Specialty Health Services
Date 2020 Sep 24
PMID 32967103
Citations 2
Authors
Affiliations
Soon will be listed here.
Abstract

Ehlers-Danlos syndromes (EDS) are a group of connective tissue disorders that manifest with hyperextensibility of joints and skin, and general tissue fragility. While not a major criterion for clinical diagnosis, pain is a frequently endorsed symptom across subtypes of EDS. As such, the present review aims to summarize research to date on pain characteristics and management, and the relationship between such pain symptomatology and quality of life in pediatric EDS. Characteristics of pain, including theorized etiology, relative intensity and extent of pain are described, as well as descriptions of frequently endorsed pain sites (musculoskeletal, and non-musculoskeletal). Interventions related to the management of musculoskeletal (e.g., pharmaceutical intervention, physical therapy) and non-musculoskeletal pain (e.g., pharmaceutical and psychological interventions) are discussed, highlighting the need for additional research related to pediatric pain management in the context of hypermobility syndromes. In addition, the relationship between pain in pediatric EDS and quality of life is described. Finally, limitations of literature to date are described and recommendations for future lines of research are outlined.

Citing Articles

The Relationship Between Fatigue, Pain Interference, Pain-Related Distress, and Avoidance in Pediatric Hypermobile Ehlers-Danlos Syndrome.

Sokol O, Biggs E, Berger A, Simons L, Bhandari R Children (Basel). 2025; 12(2).

PMID: 40003271 PMC: 11854175. DOI: 10.3390/children12020170.


The Impact of Pain on Everyday Activities of People With Hypermobility Spectrum Disorders or Hypermobility Ehlers Danlos Syndrome.

Lindholm S, Petersson S, Molander P, Bjork M Eur J Pain. 2025; 29(3):e70000.

PMID: 39945031 PMC: 11822559. DOI: 10.1002/ejp.70000.


Comorbidities of Rural Children and Adolescents with Migraine and without Migraine.

Walter S, Dai Z, Wang K Children (Basel). 2023; 10(7).

PMID: 37508629 PMC: 10378174. DOI: 10.3390/children10071133.


State of the art and future directions in assessing the quality of life in rare and complex connective tissue and musculoskeletal diseases.

Trieste L, Cannizzo S, Palla I, Triulzi I, Turchetti G Front Med (Lausanne). 2022; 9:986218.

PMID: 36213631 PMC: 9537631. DOI: 10.3389/fmed.2022.986218.

References
1.
Rose J, Finkel J, Himelstein B, Schreiner M, Medve R . Oral tramadol for the treatment of pain of 7-30 days' duration in children. Anesth Analg. 2002; 96(1):78-81, table of contents. DOI: 10.1097/00000539-200301000-00016. View

2.
Gaisl T, Giunta C, Bratton D, Sutherland K, Schlatzer C, Sievi N . Obstructive sleep apnoea and quality of life in Ehlers-Danlos syndrome: a parallel cohort study. Thorax. 2017; 72(8):729-735. DOI: 10.1136/thoraxjnl-2016-209560. View

3.
Fatoye F, Palmer S, Macmillan F, Rowe P, van der Linden M . Proprioception and muscle torque deficits in children with hypermobility syndrome. Rheumatology (Oxford). 2008; 48(2):152-7. DOI: 10.1093/rheumatology/ken435. View

4.
Gedalia A, BREWER Jr E . Joint hypermobility in pediatric practice--a review. J Rheumatol. 1993; 20(2):371-4. View

5.
Sacheti A, Szemere J, Bernstein B, Tafas T, Schechter N, Tsipouras P . Chronic pain is a manifestation of the Ehlers-Danlos syndrome. J Pain Symptom Manage. 1997; 14(2):88-93. DOI: 10.1016/s0885-3924(97)00007-9. View