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

Brenda D Barajas1, Angela Sun, David L Rimoin

  • 1Medical Genetics Institute, Cedars-Sinai Medical Center, Los Angeles, CA 90048, USA.

American Journal of Medical Genetics. Part A
|May 2, 2013
PubMed

Insights

Acute compartment syndrome (ACS) is a rare complication of vascular type Ehlers-Danlos syndrome (EDS). This case suggests ACS may occur in other heritable connective tissue disorders, highlighting the need for broader diagnostic considerations.

Area of Science:

  • Vascular Medicine
  • Genetics
  • Orthopedics

Background:

  • Arterial complications are frequent in vascular Ehlers-Danlos syndrome (EDS), with acute compartment syndrome (ACS) reported post-vascular rupture.
  • Vascular fragility in other connective tissue disorders can lead to arterial aneurysms and rupture, but ACS has not been previously documented.

Observation:

  • A female patient presented with recurrent compartment syndrome and features overlapping with hypermobile and vascular EDS.
  • She did not meet the diagnostic criteria for either hypermobile or vascular EDS, clinically or molecularly.

Findings:

  • This case expands the known complications of heritable connective tissue disorders.
  • The literature review indicates that compartment syndrome is a rare but potential complication in these conditions.

Implications:

  • Compartment syndrome should be considered in the differential diagnosis of patients with unexplained recurrent ACS, especially those with features suggestive of heritable connective tissue disorders.
  • Further research is warranted to understand the link between various heritable connective tissue disorders and the risk of developing ACS.

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