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Case Reports
. 2018 Jun;7(2):94-96.
doi: 10.1055/s-0037-1607342. Epub 2017 Oct 23.

Diagnosis of Systemic Capillary Leak Syndrome in a Young Child Treated with Intravenous Immunoglobulin in the Acute Phase

Anna Sofi E Asmundsson  1 , Ashley R Bjorklund  1   2 , Gwenyth A Fisher  1
Affiliations
Case Reports

Diagnosis of Systemic Capillary Leak Syndrome in a Young Child Treated with Intravenous Immunoglobulin in the Acute Phase

Anna Sofi E Asmundsson et al. J Pediatr Intensive Care. 2018 Jun.

Abstract

Systemic capillary leak syndrome (SCLS) is a potentially life-threatening disorder characterized by distributive shock, hypoalbuminemia, and hemoconcentration. It is exceedingly rare in children with less than 20 cases reported to date. The underlying cause for this syndrome remains largely unknown and acute treatment has remained mainly supportive. Prophylaxis with intravenous immunoglobulin (IVIG) has been shown to successfully prevent further episodes in both adults and children. We present a case of a 2-year-old previously healthy male admitted to the pediatric intensive care unit with a clinical course consistent with SCLS. His shock was refractory to aggressive fluid and vasopressor support. Reversal of SCLS with IVIG given in the acute phase had been described in three adult subjects, and for this reason, the decision was made to administer IVIG. Within an hour of administration, hemodynamics stabilized and vasopressor support could be weaned. He has had no further episodes on prophylactic infusions of IVIG. Although the exact mechanism of IVIG in SCLS is unknown, it has proven to be an effective and safe prophylactic therapy, and in our patient, it drastically reversed the acute capillary leak. We suggest that IVIG should be considered as acute therapy in pediatric patients with refractory shock and a clinical course suggestive of SCLS.

Keywords: hemoconcentration; hypoalbuminemia; intravenous immunoglobulin; systemic capillary leak syndrome.

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Conflict of interest statement

Conflict of Interest None.

Figures

Fig. 1
Fig. 1
Massive fluid resuscitation was given until the time of IVIG administration, after which blood pressure stabilized and no further fluid boluses were needed. HR, heart rate; IVIG, intravenous immunoglobulin; MAP, mean arterial pressure.
See this image and copyright information in PMC

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