The challenges in managing a case of refractory and overwhelming shock caused by Systemic Capillary Leak Syndrome

Abstract

Introduction: Systemic Capillary Leak Syndrome (SCLS) or Clarkson syndrome is a rare condition characterised by vascular hyperpermeability. The frequency and severity of disease flares is highly variable. The disease is associated with significant morbidity and mortality. We describe a fulminant episode in a patient known to have the condition. Case Report: A forty-four-year-old woman self-presented with malaise and dyspnoea. Over twelve hours she developed refractory shock despite aggressive volume resuscitation and vasopressor use. She ultimately succumbed to the condition despite aggressive treatment which included fasciotomies of all four limbs for compartment syndrome and a laparostomy for suspected abdominal compartment syndrome. Conclusion: SCLS should be considered in the context of hypotension, haemoconcentration, and hypoalbuminaemia in the absence of albuminuria. Management of acute SLCS episodes can prove extremely challenging for both the intensivist and the anaesthetist. The underlying pathophysiology is unique and fascinating. Supportive care in an intensive care setting is the only established treatment. There are still no proven pharmacotherapies to modulate the course of acute episodes of this disease. Keywords: Shock states; Capillary leak; Compartment syndrome.