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.

Spontaneous compartment syndrome and endovascular repair of tibioperoneal trunk pseudoaneurysm in Ehlers-Danlos syndrome

“…In vEDS, compartment syndrome will typically occur secondary to spontaneous arterial bleeding, which rapidly increase the compartment pressures. 5 , 8 However, the initial CTA noted no contrast extravasation or hematoma ( Fig 3 ). Each compartment's musculature was grossly edematous, and no source of arterial bleeding was encountered.…”

“…Our patient presented with compartment syndrome without preceding trauma. In vEDS, compartment syndrome typically occurs secondary to spontaneous arterial bleeding, rapidly increasing compartment pressures (5,8). However, the initial CTA noted no J o u r n a l P r e -p r o o f contrast extravasation or hematoma (Figure 3).…”

Recurrent Lower-Extremity Compartment Syndrome after Four-Compartment Fasciotomy Secondary to Acute Limb Ischemia

“…A patient with an unclassified connective tissue disorder that displayed some features of EhlerseDanlos syndrome was reported to have had an estimated 60e70 fasciotomies performed within a span of 10 years. 7 In another case, a 34-year-old man developed compartment syndrome, requiring lateral compartment fasciotomies of the same leg, twice in the same year, 2 years after he had a 4-compartment fasciotomy for acute compartment secondary to a closed injury of the leg. In both instances of his recurrent lateral compartment syndrome, no cause was identified.…”

Chronic Exertional Compartment Syndrome in a Healthy Young Man

“…This is the definitive test and the criterion standard for making the diagnosis of exercise-induced compartment syndrome. 18,[22][23][24][25][26] The orthopedic surgeon chose to continue conservative therapy for 12 weeks prior to considering the patient for an initial diagnostic testing.…”