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.

Acta Medica Portuguesa
|August 23, 2024
PubMed

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.

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