Idiopathic Systemic Capillary Leak Syndrome, A Case Report of an Eleven Years Old Girl with Review of Literature

Abstract

Idiopathic Systemic Capillary Leak Syndrome (ISCLS), also known as Clarkson disease, is a rare disorder characterized by episodes of severe hypotension, hypoalbuminemia and hemoconcentration. Approximately 200 adult cases have been previously described in the medical literature. To our knowledge only ten pediatric cases have been previously reported in the medical literature. We present here the eleventh pediatric case, an eleven year old girl who first presented during menarche with severe hypotension during the leak phase in early adolescence. Management initially included supportive measures and cardiopulmonary resuscitation. Preventative measures we have instituted include therapeutic trials of theophylline, montelukast, immunoglobulin (IVIG), and oral birth control pills to create anovulatory cycles - which have proven to be clinically successful for > 5 years since her initial presentation. We describe her initial clinical presentation and clinical course, as well as a review of the literature below.