Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis

Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent spontaneous episodes of hypovolaemic shock due to marked plasma shifts from the intravascular to the extravascular space. This presents as the characteristic triad of hypotension, haemoconcentration and hypoalbuminemia often with an associated monoclonal gammopathy. We describe a patient with SCLS who required aggressive fluid resuscitation and emergency fasciotomies for compartment syndrome with rhabdomyolysis. At presentation the patient was considered to have severe erythrocytosis and was therefore initially referred to a haematologist, which appears to be a frequent sequence of presentation for patients with SCLS. This patient also highlights the importance of muscle compartment pressure monitoring during volume resuscitation in patients with SCLS.