Abstract
Objective. To describe a single case of Systemic Capillary Leak Syndrome (SCLS) with a rare complication of compartment syndrome. Patient. Our patient is a 57-year-old male, referred to our hospital due to polycythemia (hemoglobin (Hgb) of 220 g/L), hypotension, acute renal failure, and bilateral calf pain. Measurements and Main Results. The patient required bilateral forearm, thigh, and calf fasciotomies during his ICU stay and continuous renal replacement therapy was instituted following onset of acute renal failure and oliguria. Ongoing hemodynamic (Norepinephrine and Milrinone infusion) and respiratory (ventilator) support in the ICU was provided until resolution of intravascular fluid extravasation. Conclusions. SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space. Patients present with significant hypotension, hemoconcentration, hypovolemia, and oliguria. Severe edema results from leakage of fluid and proteins into tissue. The most important part of treatment is maintaining stable hemodynamics, ruling out other causes of shock and diligent monitoring for complications. Awareness of the clinical syndrome with the rare complication of compartment syndrome may help guide investigations and diagnoses of these critically ill patients.
Highlights
Systemic Capillary Leak Syndrome (SCLS, Clarkson Syndrome) was first described by Clarkson et al in 1960 [1]
SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space
In the 5 weeks prior to presentation, he had begun an exercise program, which included running on a treadmill, and over the last week he complained of progressive bilateral leg swelling and pain
Summary
To describe a single case of Systemic Capillary Leak Syndrome (SCLS) with a rare complication of compartment syndrome. Our patient is a 57-year-old male, referred to our hospital due to polycythemia (hemoglobin (Hgb) of 220 g/L), hypotension, acute renal failure, and bilateral calf pain. The patient required bilateral forearm, thigh, and calf fasciotomies during his ICU stay and continuous renal replacement therapy was instituted following onset of acute renal failure and oliguria. SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space. Severe edema results from leakage of fluid and proteins into tissue. Awareness of the clinical syndrome with the rare complication of compartment syndrome may help guide investigations and diagnoses of these critically ill patients
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