Recurrent compartment syndrome in a patient with clinical features of a connective tissue disorder

Brenda D. Barajas, Angela Sun, David L. Rimoin, Eyal Reinstein*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Arterial complications are common in vascular type Ehlers-Danlos syndrome (EDS), accounting for 66% of first complications. Several cases in the literature have documented acute compartment syndrome (ACS) following vascular rupture in vascular type EDS. Other disorders of connective tissue have also demonstrated vascular fragility, leading to arterial aneurysm and rupture, but there have been no documented cases of ACS. Here, we report on a female patient with a history of recurrent compartment syndrome who exhibits some clinical findings seen in hypermobile and vascular EDS; however she does not meet clinical and molecular diagnostic criteria for either of them. We further review the literature on ACS in heritable connective tissue disorders and suggest that compartment syndrome may rarely complicate other heritable disorders of connective tissue.

Original languageEnglish
Pages (from-to)1442-1446
Number of pages5
JournalAmerican Journal of Medical Genetics, Part A
Issue number6
StatePublished - Jun 2013
Externally publishedYes


FundersFunder number
National Institute of General Medical SciencesT32GM008243
National Center for Research ResourcesM01RR000425


    • Compartment syndrome
    • Connective tissue disorders
    • Ehlers-Danlos syndrome


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