Idiopathic Systemic Capillary Leak Syndrome With Shock and Compartment Syndrome due to Influenza A

Introduction: Idiopathic systemic capillary leak syndrome (ISCLS) is a rare, poorly understood condition that leads to extreme pathology with severe but predictable complications. First described in 1960, ISCLS has been identified in about 260 case reports and is marked by a triad of hypotension, hypoalbuminemia, and hemoconcentration often preceded by upper respiratory tract infection. It is divided into distinct phases of prodrome, extravasation, and recovery. We present a case of a middle-aged woman who presented with influenza A infection and developed ISCLS needing ICU level care for shock and bilateral upper extremity compartment syndrome.


Description: Our patient is a 49-year-old woman who presented with syncope and bilateral upper extremity pain in the setting of several days of URI symptoms. She was found to have influenza A, hematocrit 58.9, albumin 2.1, serum lactate 9, and creatine kinase 119,575 requiring dialysis for clearance with creatinine 1.81. She developed refractory shock despite volume resuscitation with significant third spacing resulting in anasarca, respiratory failure leading to intubation, and bilateral upper extremity compartment syndrome requiring fasciotomies. Infection, malignancy, and rheumatologic causes for her course were ruled out, and the triad of hemoconcentration, hypotension, and hypoalbuminemia with preceding URI, and the discovery of a monoclonal gammopathy, ultimately led to diagnosis of ISCLS. Eventually, her shock resolved; her third spacing reversed, and she was taken for fasciotomy closure, discharging with dialysis and intensive rehab.


Discussion: The diagnosis of ISCLS in this case is supported by the triad of hemoconcentration, hypotension, and hypoalbuminemia in the setting of preceding URI and monoclonal gammopathy. Her case followed a typical course with a prodromal phase, fluid extravasation phase, and fluid recruitment phase. It illustrates the need for greater awareness of this rare condition that may be underrecognized. The transition point from extravasation to recruitment, which is marked by a steep drop in fluid and pressor needs, is one of acute danger. In this transitory state complications begin to mount including the mundane such as peripheral edema and more extreme and life threatening like flash pulmonary edema or compartment syndrome.