Systemic capillary leak syndrome associated with a rare abdominal and four-limb compartment syndrome: a case report

Abstract

IntroductionSystemic capillary leak syndrome is a rare and life threatening disease characterized by periodic episodes of hypovolemic shock due to leakage of plasma from the intravascular to the extravascular space. It is associated with hemoconcentration, hypoalbuminemia, and generalized edema. We report the case of a patient with idiopathic systemic capillary leak syndrome who developed an unexpected and potentially fatal abdominal and four-limb compartment syndrome. This was successfully treated with fasciotomies and medical treatment including terbutaline, theophylline, and corticosteroids. To the best of our knowledge this is the first report of this kind in the literature.Case presentationA previously healthy 54-year-old Caucasian man presented to the emergency department of our internal medicine ward with a medical history of aggravation of general health related to dizziness, weight gain, and two syncopal attacks. Due to a massive emission of fluids and proteins from the intravascular to the extracellular compartments, he developed compartment syndromes in his upper and lower limbs and the abdominal compartment. The abdomen and all four limbs required decompression by a fasciotomy of both forearms, both thighs, both lower legs, and the abdomen within 24 hours after admission. After 60 days of treatment he was dismissed from the clinic. He was able to return to his previous occupation and reached the same level of athletic activity as before the illness.ConclusionsSystemic capillary leak syndrome is a very rare disease that can lead to a fatal clinical outcome. It is important to be aware of the fatal complications that can be caused by this disease. Despite the fact that systemic capillary leak syndrome represents a very rare disease it is still important to be aware of life threatening complications, like compartment syndromes, which need surgical intervention. However, early diagnosis and interdisciplinary treatment can lead to a good clinical outcome.

Idiopathic systemic capillary leak syndrome: a case report

“…Previous case reports have described a range of complications associated with ICSLS [ 2 , 7 , 11 ]. One such report documenting a case of severe chronic ISCLS requiring daily hemodialysis, stated AKI as one of the most common manifestations of capillary leak syndrome [ 7 ].…”

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.…”

Multiple limb compartment syndrome as a manifestation of capillary leak syndrome secondary to metformin and dipeptidyl peptidase IV inhibitor overdose

“…This patient developed compartment syndrome of all 4 limbs, which is a highly uncommon presentation of compartment syndrome other than CLS, and only idiopathic CLS [ 23 , 24 ] cases were reported so far. The symmetrical change of intramuscular pressure also suggests that the trigger was a systemic effect rather than compression and atraumatic crush syndrome.…”