J Pediatr Intensive Care 2018; 07(02): 094-096
DOI: 10.1055/s-0037-1607342
Case Report
Georg Thieme Verlag KG Stuttgart · New York

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

Anna Sofi E. Asmundsson
1  Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, United States
,
Ashley R. Bjorklund
1  Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, United States
2  Department of Pediatrics, Walter Reed National Military Medical Center, Bethesda, Maryland, United States
,
Gwenyth A. Fisher
1  Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, United States
› Author Affiliations
Further Information

Publication History

20 January 2017

13 September 2017

Publication Date:
23 October 2017 (eFirst)

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.

Note

The views expressed in this article are those of the authors and do not reflect the official policy of the Navy, Department of Defense, or U.S. Government.