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Open Access Case report

10-year-old girl with life-threatening idiopathic systemic capillary leak syndrome: a case report

Tadashi Iwasa1*, Hiroyuki Ohashi1, Kentaro Kihira1, Yuhki Koike2, Kohei Otake2, Mikihiro Inoue2, Hirofumi Sawada1, Hidemi Toyoda1 and Yoshihiro Komada1

Author Affiliations

1 Department of Pediatrics, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu City, Mie Prefecture 514-8507, Japan

2 Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu City, Mie Prefecture 514-8507, Japan

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BMC Pediatrics 2014, 14:137  doi:10.1186/1471-2431-14-137

Published: 31 May 2014

Abstract

Background

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disorder, characterized by episodic life-threatening hypotension, hypoalbuminemia, and hemoconcentration.

Case presentation

A 10-year-old girl presented with abdominal pain, vomiting, diarrhea, fever and developed generalized edema a day after admission. Clinical and laboratory findings were consistent with ISCLS. She received aggressive fluid replacement, methylprednisolone pulse (30 mg/kg/day), high-dose intravenous immunoglobulin (IVIG, 2 g/kg/day) and plasma exchange in acute phase. She received fasciotomy of bilateral lower extremities as she developed complications of compartment syndrome. Since there were two episodes of ISCLS attacks, theophylline and terbutaline were initiated for prevention of attacks and then the remission is currently maintained. Because of high fatality rate in ISCLS, prompt diagnosis and intervention are very important.

Conclusion

We describe here, a rare case of pediatric ISCLS. ISCLS should be considered as a differential diagnosis, when the patient presents with unexplained or sudden hypovolemic shock. Reports on pediatrics ISCLS are very few, and accumulation of similar case reports is needed.

Keywords:
Idiopathic systemic capillary leak syndrome; Vascular endothelial damage; Methylprednisolone pulse; Theophylline; Terbutaline