Capillary leak syndrome

Capillary leak syndrome
DiseasesDB	34090
MeSH	D019559

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and Keywords: Systemic Capillary Leak Syndrome, SCLS

Overview

Capillary Leak Syndrome is a rare medical condition where the number and size of the pores in the capillaries are increased which leads to a leakage of fluid from the blood to the interstitial fluid, resulting in dangerously low blood pressure (hypotension), edema and multiple organ failure due to limited perfusion.

Historical Perspective

The syndrome was first described by Clarkson in 1960.

Diagnosis

Symptoms

Generalized swelling may be present.

Physical Examination

Vitals

• Low blood pressure (hypotension)

Skin

Generalized edema or anasarca

Laboratory Studies

• Hemoconcentration
• Hypoalbuminemia without albuminuria

Natural hisotry

The episode usually consists of two phases

1. The capillary leak phase (1-4 days) The initial phase is the capillary leak phase, lasting from 1 to 4 days. Clinical features are abdominal pain, nausea, generalized edema and hypotension that may result in cardiopulmonary collapse. Acute renal failure is due to acute tubular necrosis consequent to hypovolemia and rhabdomyolysis.

2. Recruitment of the interstitial fluid The second phase results in the recruitment of the initially extravasated fluid. Intravascular overload with polyuria and pulmonary edema often occur. Edema may be more severe due to massive fluid supply in the initial phase. It's necessary to monitor the patient in order to switch to depletion treatment with diuretics or hemofiltration.

Treatment

A few prophylactic treatments have been tried with variable results, principally terbutaline and theophylline.

Prognosis

Mortality is reported in 21% of the 57 cases described. However, better management of this condition has recently led to lower mortality.

References

• Dorlands Medical Dictionary 30th Ed.
• Cancer.gov
• Case report in Oxford Journals
• Orphanet (2003)

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