Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome

Objective: To report chronic fatigue syndrome (CFS) associated with both Eh lers-Danlos syndrome (EDS) and orthostatic intolerance. Study design: Case series of adolescents referred to a tertiary clinic for the evaluation of CFS. All subjects had 2-dimensional echocardiography, tes ts of orthostatic tolerance, and examinations by both a geneticist: and an ophthalmologist, Results: Twelve patients (11 female), median age 15.5 years, met: diagnosti c criteria for CFS and EDS, and all had either postural tachycardia or neur ally mediated hypotension in response to orthostatic stress. Six had classi cal-type EDS and 6 had hypermobile-type EDS. Conclusions: Among patients with CFS and orthostatic intolerance, a subset also has EDS. We propose that the occurrence of these syndromes together ca n be attributed to the abnormal connective tissue in dependent blood vessel s of those with EDS, which permits veins to distend excessively in response to ordinary hydrostatic pressures. This in turn leads to increased venous pooling and its hemodynamic and symptomatic consequences. These observation s suggest that: a careful search for hypermobility and connective tissue ab normalities should be part of the evaluation of patients with CFS and ortho static intolerance syndromes.