Abstract
Systemic Capillary Leak Syndrome (SCLS) is a rare disorder characterized by increased capillary hyperpermeability leading to hypovolemic shock due to a markedly increased shift of fluid and protein from the intravascular to the interstitial space. Hemoconcentration, hypoalbuminemia and a monoclonal gammopathy are characteristic laboratory findings. Here we present a patient who suffered from SCLS with hypovolemic shock and compartment syndrome of both lower legs and thighs. Volume and catecholamine management was guided using transpulmonary thermodilution. Extended hemodynamic monitoring for volume and catecholamine management as well as monitoring of muscle compartment pressure is of crucial importance in SCLS patients.
Highlights
Systemic Capillary Leak Syndrome (SCLS) is a rare disorder characterized by increased capillary hyperpermeability leading to hypovolemic shock due to a markedly increased shift of fluid and protein from the intravascular to the interstitial space
Backround Systemic Capillary Leak Syndrome (SCLS) is a rare disorder characterized by unexplained, often recurrent, non sepsis-related episodes of increased capillary hyperpermeability leading to hypovolemic shock due to a markedly increased shift of fluid and protein from the intravascular to the interstitial space
SCLS is a very rare disorder with a high mortality rate. It is characterized by increased capillary permeability resulting in hypovolemic shock due to a marked shift of fluid and protein from the intravascular to the extravascular space
Summary
SCLS is a very rare disorder with a high mortality rate. It is characterized by increased capillary permeability resulting in hypovolemic shock due to a marked shift of fluid and protein from the intravascular to the extravascular space. In general optimization of intravascular volume status under consideration of pulmonary hydration is of central importance in the treatment of critically ill patients. There is increasing data that pulmonary artery wedge pressure is not appropriate for assessment of preload and prediction of volume responsiveness, in ICU patients with invasive mechanical ventilation and/or increased intra-abdominal pressure [17]. In numerous studies transpulmonary thermodilutionderived dynamic and volumetric variables of preload have been demonstrated as superior indicators of volume responsiveness as compared to pressures such as pulmonary artery wedge pressure and central venous pressure [14,18,19]. In addition to catecholamine administration, transpulmonary thermodilution-guided volume-management regarding decreased GEDVI as valuable marker of volume deficiency and increased EVLWI as "upper threshold" for further volume resuscitation proved as very useful tool in this patient who's hydration status was difficult to judge using clinical criteria.
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