Idiopathic Systemic Capillary Leak Syndrome: Report of a Pediatric Case

Authors

  • João Vasco Serviço de Pediatria. Hospital do Divino Espírito Santo. Ponta Delgada. https://orcid.org/0009-0009-1784-019X
  • Francisca Albuquerque Serviço de Pediatria. Unidade Local de Saúde do Alentejo Central. Évora.
  • Francisca Costa Unidade de Cuidados Intensivos e Especiais Pediátricos. Unidade Local de Saúde Amadora/Sintra. Amadora.
  • Ana Dias Curado Unidade de Infeciologia e Imunodeficiências. Serviço de Pediatria. Hospital de Santa Maria. Unidade Local de Saúde de Santa Maria. Lisboa.
  • Carlos Escobar Unidade de Cuidados Intensivos e Especiais Pediátricos. Unidade Local de Saúde Amadora/Sintra. Amadora.

DOI:

https://doi.org/10.20344/amp.21531

Keywords:

Capillary Leak Syndrome, Child, Shock

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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References

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Published

2024-08-23

How to Cite

1.
Vasco J, Albuquerque F, Costa F, Dias Curado A, Escobar C. Idiopathic Systemic Capillary Leak Syndrome: Report of a Pediatric Case. Acta Med Port [Internet]. 2024 Aug. 23 [cited 2024 Dec. 12];37(11):802-4. Available from: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/21531

Issue

Section

Case Report