Idiopathic Systemic Capillary Leak Syndrome: Report of a Pediatric Case

Abstract

The idiopathic systemic capillary leak syndrome is characterized by recurrent episodes of hypovolemia, with an unknown cause, presenting as a distributive and hypovolemic shock, due to fluid loss to the extravascular space. We describe a case of a previously healthy seven-year-old boy, who started with prodromal symptoms (abdominal pain, fatigue, nausea), followed by a fluid extravasation phase, with hemoconcentration, hypoproteinemia, and muscular edema in the abdominal wall and lower limbs, accompanied by pain – compartment syndrome. After a couple of days, spontaneous and fast recovery was noted, with clinical and analytic improvement. The inflammatory markers were always normal, and the blood cultures were negative. In this case, it is possible to distinguish the three idiopathic systemic capillary leak syndromes phases, as described in the literature. Although rare, this syndrome can be fatal, and the differential diagnosis with other causes of shock represents a challenge.