Systemic capillary leak syndrome (Clarkson's disease) during elective pylorus-preserving pancreaticoduodenectomy: case report

Abstract

Systemic capillary leak syndrome (SCLS), also called Clarkson's disease is rare and life-threatening disorder of unknown etiology, which is a characteristic triad of hypovolemic shock, hemoconcentration, and hypoalbuminemia. Unexplained capillary leakage from the intravascular to the interstitial space, which has been estimated up to 70% of the intravascular volume, is the proposed mechanism. Because the pathogenesis is unknown, it is diagnosed clinically after exclusion of other diseases that cause systemic capillary leak and no efficacious pharmacological treatment has been clearly established. The mortality rate ranges from 30% to 76%. In Korea, four cases of SCLS (5 cases in adult and 1 case in child) were reported by 2012. We describe a case of severe SCLS that suddenly occurred and rapidly progressed during pylorus preserving pancreaticoduodenectomy and review the literature.

Systemic capillary leak syndrome during cardiac surgery

“…It is well known that idiopathic SCLS may occur during surgical procedures without preceding symptoms or a suggestive history. [1,2] The rarity and sporadic nature of the condition make it difficult to conclude whether CPB-induced systemic inflammation may have a role in triggering the disease, and whether measures to reduce inflammation including coated circuits, mini-circuits, leukocyte and cytokine filters can prevent the attack.…”

“…Surgery may induce SCLS without a previous history. [1,2] To the best of our knowledge, there is no report regarding SCLS occurred during or after adult cardiac surgery in the literature. In this article, we present an adult case who suffered from an acute SCLS attack during cardiac surgery.…”

Refractory shock during the anesthetic and surgical management of an intrahepatic tumor arising from the adrenal cortex: A case report

“…The underlying mechanism is a transient dysfunction of the endothelium, with up to 70% of the plasma shifting from the intravascular to the interstitial space. By contrast to cardiopulmonary bypass surgery, abdominal surgery is a condition that has been exceptionally associated with systemic capillary leak syndrome [ [11] , [12] , [13] , [14] , [15] ]. In all the reported cases after abdominal surgery, the diagnosis was based on the triad of hypotension, hemoconcentration and hypoalbuminemia, after reasonable exclusion of other etiologies of shock.…”