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Related Experiment Videos

Systemic capillary leak syndrome presenting as recurrent shock.

Christos Karatzios1, France Gauvin, E Patricia Egerszegi

  • 1Division of Infectious Diseases, Department of Pediatrics, Montreal Children's Hospital, McGill University Health Center, Montreal, QC, Canada.

Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
|June 2, 2006
PubMed
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Systemic capillary leak syndrome (SCLS) is rare in children. This case report highlights SCLS as a crucial consideration for recurrent hypovolemic shock in pediatric patients, emphasizing prompt fluid resuscitation.

Area of Science:

  • Pediatric critical care medicine
  • Rare diseases
  • Intensive care unit management

Background:

  • Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent episodes of hypotension, hemoconcentration, and edema.
  • While SCLS is well-documented in adults, pediatric cases are infrequently reported.

Observation:

  • A 6-year-old girl experienced two episodes of severe shock with significant edema, requiring intensive care.
  • Initial symptoms included abdominal pain, vomiting, and syncope, progressing to shock unresponsive to initial treatments.
  • Investigations ruled out cardiogenic and septic shock; the second episode, following influenza A infection, led to a diagnosis of SCLS.

Findings:

  • The patient presented with recurrent hypovolemic shock and generalized edema.

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  • Aggressive fluid resuscitation, mechanical ventilation, and vasopressors were critical for managing the shock.
  • Diagnosis of SCLS was established after excluding other potential causes and considering the clinical presentation.

    • Implications:

    • SCLS should be considered in the differential diagnosis of pediatric patients with unexplained recurrent hypovolemic shock and edema.
    • Early recognition and aggressive intravenous fluid resuscitation are vital for improving outcomes in pediatric SCLS.
    • Further research into pediatric SCLS is warranted to improve diagnostic and therapeutic strategies.