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

Abstract

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.

Figure 1

Physical findings in patient with recurrent compartment syndrome A - Normal body habitus with non-dysmorphic facial features. B – Non-healing ulcer located superior to right medial malleolus

Figure 2

Surgical scars, following faciotomies A – Lateral aspect of right leg B – Anterior aspect of left leg C – Left posterior forearm D – Right medial thigh E – Dorsum of left hand