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

Abstract

BackgroundIdiopathic 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 presentationA 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.ConclusionISCLS 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.

Reversible Myocardial Edema Secondary to Tagraxofusp-Induced Capillary Leak Syndrome

“…Myocardial edema secondary to CLS has been reported in several cases of Clarkson disease, with manifestations ranging from echocardiographic ventricular wall thickening without systolic dysfunction to cardiogenic shock with severe LV dysfunction necessitating extracorporeal life support ( 3 , 4 , 5 , 6 , 7 ). Critically, live and postmortem cardiac biopsies have uniformly demonstrated diffuse edematous changes without evidence of acute inflammation or myocyte necrosis to implicate underlying myositis or infarction ( 3 , 8 ).…”

A deadly capillary leak attack. Clarkson’s disease: a narrative review

“…In addition, albumin levels can be in normal range at presentation and appear to drop when serial measurements are taken the following days. 2,3,5,8,16,17 In a case review of Eo et al, where 161 ISCLS case reports were scrutinized, there was no information on the albumin level at presentation in 48.4% of cases. In cases where there was a mentioned albumin level, hypoalbuminemia was absent in 15.7% of them.…”

“…For this reason, serial measurements of serum albumin during the episode are warranted to confirm protein leakage. 2,3,5,8,16,17 A serum protein electrophoresis to detect a paraprotein can also be performed. However, a monoclonal gammopathy cannot be considered a primary criterion since it is not consistently present.…”

“…1 Less than 500 adults and 32 paediatric cases of ISCLS have been reported in the literature since 1960. 2 Unfortunately, this disease is underdiagnosed and it's 10-year mortality is estimated between 25% and 34%. 3 ISCLS can affect the entire vascular system and consists of flares of varying intensity.…”

Utilización de membrana de circulación extracorpórea en síndrome de leak capilar idiopático: reporte de un caso

“…El paciente logró por medio de este dispositivo mantener una adecuada oxigenación, al tiempo que se brindó soporte hemodinámico y cardiaco durante la etapa de shock. Si bien existe el reporte de un caso de uso de ECMO en SLCI en pediatría 9 , en un paciente que también desarrolló edema miocárdico secundario a la enfermedad, se trata del primer caso de requerimiento de ECMO en SLCI en adultos. Aunque si bien a la fecha no existen indicaciones formales para su uso como soporte cardiorrespiratorio en esta patología, se podría considerar como una herramienta útil para el intensivista en situaciones críticas en pacientes con alto potencial de recuperación.…”