SESSION TITLE: Critical Care Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 26, 2015 at 03:15 PM - 04:15 PM
INTRODUCTION: Shock is a common presentation to the ICU, however uncommon causes should be considered when no etiology is found.
CASE PRESENTATION: A 22 year old man presented with syncope & bilateral lower extremity edema. He was hypotensive & afebrile with poor peripheral perfusion. Studies revealed erythrocytosis, leukocytosis, acute kidney injury, lactic acidosis & hypoalbuminemia. Volume resuscitation, empiric antibiotics, & vasopressors were administered. Infectious, cardiac, hepatic, & hematologic work-ups were unrevealing. Renal studies showed acute tubular necrosis. On hospital day two he lost distal lower extremity pulses & was brought for emergent arteriogram, popliteral artery exploration & bilateral fasciotomies. By hospital day three his shock resolved but he developed acute hypoxemic respiratory failure from cardiogenic pulmonary edema. Concurrent severe anemia occurred but no bleeding source was found. He ultimately recovered but suffered three similar illnesses in the following year. The combination of hemoconcentration, hypoalbuminemia & edema with swift resolution but resultant fluid accumulation leading to respiratory failure & compartment syndrome is highly suggestive of Systemic Capillary Leak Syndrome, or Clarkson’s Disease.
DISCUSSION: First described in 1960, 126 cases of Systemic Capillary Leak Syndrome have been reported. It presents with recurrent episodes of hypovolemic shock due to sudden leakage of plasma into the extravascular space & subsequent cardiovascular collapse. Spontaneous resolution occurs within days leading to rapid intravascular fluid redistribution. Initial complications include multi-organ failure related to hypoperfusion and arteriovenous thrombi. Mortality is highest in the post-resuscitation phase due to complications of rhabdomyolysis, compartment syndrome, pulmonary edema & subsequent respiratory failure from fluid reaccumulation. Treatment consists of judicious fluid resuscitation with toleration of markers of hypoperfusion in order to prevent post-resuscitation complications. Data on preventive therapies including terbutaline & theophylline is limited.
CONCLUSIONS: The presentation of hemoconcentration, hypoalbuminemia & edema in a patient with shock of unclear etiology is suggestive of Systemic Capillary Leak Syndrome. Judicious fluid administration is advised to decrease the risk of compartment syndrome & cardiopulmonary collapse in the post-leak phase.
Reference #1: Druery K, Greipp PR, Narrative review:the systemic capillary leak syndrome. Ann Intern Med. 2010; 153:90-8
DISCLOSURE: The following authors have nothing to disclose: Christine Bielick, Ryan Shipe, Craig Lilly
No Product/Research Disclosure Information