Type III Ehlers-Danlos syndrome ... most debilitating form ... article

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1: Clin Orthop. 2000 Jul;(376):213-21. Related Articles, Links

 

Orthopaedic manifestations of Ehlers-Danlos syndrome.

Stanitski DF, Nadjarian R, Stanitski CL, Bawle E, Tsipouras P.

Medical University of South Carolina, ton, USA.

Ehlers-Danlos syndrome is the most prevalent heritable disorder of connective

tissue. Musculoskeletal problems include joint pain, swelling and

instability, and spinal deformity. This study was undertaken to assess

functional

orthopaedic problems of patients with Ehlers-Danlos syndrome. Sixty patients

with

genetically verified Ehlers-Danlos syndrome (range, 8-60 years; mean, 34 years)

who attended a National Ehlers-Danlos Syndrome Foundation learning conference

were evaluated by questionnaire, clinical examination, and when indicated,

radiographs. A database of 250 items per patient was constructed and

statistically

assessed using analysis of variance. Because of rarity of Types VII and VIII,

these two patients were dropped from the analysis. Fifty-eight patients had

Ehlers-Danlos syndrome Types I, II, III, or IV and form the study cohort. Among

these four types, there were no significant differences in history of joint

dislocation, swelling, or types of orthopaedic surgical procedures experienced.

Thirty patients with Type III Ehlers-Danlos syndrome reported joint pain more

frequently than did patients with Types I, II, or IV. Ambulation was impaired

significantly in patients with Type III disorder as a whole, as was

functional hand strength and upper extremity function. Back or neck pain was a

common

(67.2%) report among patients with all types of disease but did not correlate

with the presence or absence of spinal deformity. Contrary to most previous

reports, the patients in this study showed that Type III Ehlers-Danlos syndrome

was the most debilitating form with respect to musculoskeletal function.

PMID: 10906878 [PubMed - indexed for MEDLINE]