Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis.

[systemic capillary leak syndrome]

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 .