Malignant Hemispheric Cerebral Infarction Associated with Idiopathic Systemic Capillary Leak Syndrome

Abstract

Idiopathic systemic capillary leak syndrome (ISCLS) is a rare condition that is characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse general swelling, fetal hypovolemic shock, hypoalbuminemia, and hemoconcentration. Although ISCLS rarely induces cerebral infarction, we experienced a patient who deteriorated and was comatose as a result of massive cerebral infarction associated with ISCLS. In this case, severe hypotensive shock, general edema, hemiparesis, and aphasia appeared after serious antecedent gastrointestinal symptoms. Progressive life-threatening ischemic cerebral edema required decompressive hemicraniectomy. The patient experienced another episode of severe hypotension and limb edema that resulted in multiple extremity compartment syndrome. Treatment entailed forearm and calf fasciotomies. Cerebral edema in the ischemic brain progresses rapidly in patients suffering from ISCLS. Strict control of fluid volume resuscitation and aggressive diuretic therapy may be needed during the post-leak phase of fluid remobilization.

Severe cerebral involvement due to idiopathic systemic capillary leak syndrome

“…There is one study describing a cerebral involvement in a child with ISCLS 10. To the best of our knowledge, only one other case with cerebral involvement leading to unilateral malignant cerebral infarction in adults has been published to date 11. IVIG seems to be a rational treatment option for the acute phase in patients with ISCLS and cerebral involvement 12.…”

Systemic capillary leak syndrome requiring fasciotomy for limb compartment syndrome: A case report and literature review

“…4 Patients with SCLS typically develop prodromal symptoms, such as weakness, malaise, myalgias, or abdominal pain, followed by a leakage phase represented by hypotensive shock and edema. This phase, which lasts for 1-3 days, 1 is the critical period where patients are prone to hypoperfusion-related multiorgan dysfunction and LCS as severe complications [2][3][4][5][6][7][8][9][10] ; patients require the administration of fluids (in massive quantities), vasopressors for resuscitation, and additional treatments for complications. The leakage phase is followed by the postleakage phase, in which vascular permeability starts to be restored with fluid recruitment into the intravascular circulation.…”

Systemic capillary leak syndrome induced by influenza type A infection

“…Extremity compartment syndrome and ischemic cerebral stroke are caused by the hyperviscosity state and intravascular volume depletion during the shock and acute leak phase [ 12 , 13 ]. In a recent pediatric SCLS case associated with influenza A, a boy treated medically without decompressive fasciotomy recovered without sequelae, despite the initial painful calves and thighs with elevated CPK [ 14 ].…”