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Published on: September 20, 2018
Idiopathic Systemic Capillary Leak Syndrome: Report of a Pediatric Case
João Vasco1, Francisca Albuquerque2, Francisca Costa3
1Serviço de Pediatria. Hospital do Divino Espírito Santo. Ponta Delgada. Portugal.
Insights
Idiopathic systemic capillary leak syndrome (ISCLS) causes sudden fluid loss, leading to shock. This case highlights ISCLS phases in a child, emphasizing diagnostic challenges.
Area of Science:
- Pediatrics
- Internal Medicine
- Critical Care Medicine
Background:
- Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder.
- ISCLS is characterized by recurrent episodes of hypovolemia and shock.
- The exact cause of ISCLS remains unknown.
Observation:
- A previously healthy seven-year-old boy presented with prodromal symptoms including abdominal pain, fatigue, and nausea.
- The patient experienced a fluid extravasation phase with hemoconcentration, hypoproteinemia, and muscular edema.
- Compartment syndrome developed in the abdominal wall and lower limbs due to edema.
Findings:
- The patient exhibited the three distinct phases of ISCLS: prodromal, extravasation, and recovery.
- Inflammatory markers and blood cultures remained negative throughout the illness.
- Spontaneous and rapid clinical and analytical recovery was observed within days.
Implications:
- This case illustrates the typical presentation and course of ISCLS in a pediatric patient.
- Early recognition and diagnosis of ISCLS are crucial due to its potential fatality.
- Differentiating ISCLS from other causes of shock is a significant clinical challenge.
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.
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