Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome

BMC Pediatr. 2019 Jan 21;19(1):28. doi: 10.1186/s12887-019-1401-2.


Background: Idiopathic systemic capillary leak syndrome (ISCLS) is rare, and there has been about 32 cases reported in children worldwide since this disorder was first described in 1960. Clinical guidelines on the management approach stemming from robust scientific evidence are lacking. This case report presents the first reported paediatric case of severe ISCLS with significant myocardial oedema and emphasizes this disease's impact on a child's cardiac function.

Case presentation: A Chinese boy had his first attack of severe hypovolaemic shock that responded to fluid resuscitation when he was 6 years of age. His second attack developed at 8 years of age. He was then transferred to our cardiac unit for refractory hypotensive shock. The patient's echocardiogram revealed ventricular wall thickening with significant cardiac dysfunction requiring extracorporeal membrane oxygenation support. Subsequently, he made a full recovery, including his myocardial wall thickness and function. The echocardiographic findings suggested myocardial oedema that was transient in nature. Clinical and laboratory investigation from both episodes were compatible with ISCLS.

Conclusion: ISCLS is rare, and therefore there is only a limited understanding on the pathophysiology of this disorder. The current treatment approach is based on a few case reports and series. During the acute phase, optimal supportive management is paramount. Our case highlights the importance of early recognition and consideration for extracorporeal membrane oxygenation support in patients with a life-threatening presentation, as it was lifesaving for this child who suffered myocardial oedema and ventricular dysfunction.

Keywords: Clarkson’s disease; ECMO; Extracorporeal membrane oxygenation; Myocardial oedema; Paediatrics; Shock; Systemic capillary leak syndrome.

Publication types

  • Case Reports

MeSH terms

  • Asians
  • Capillary Leak Syndrome / complications*
  • Cardiomyopathies / diagnosis
  • Cardiomyopathies / etiology*
  • Cardiomyopathies / therapy
  • Child
  • Edema / diagnosis
  • Edema / etiology*
  • Edema / therapy
  • Humans
  • Male