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

[systemic capillary leak syndrome]

Systemic 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 .A 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 .Systemic 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 .

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