title: Systemic capillary leak syndrome associated with a rare abdominal and four-limb compartment syndrome: a case report.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.forename: Hayat
authors.initials: H
authors.lastname: Lamou
authors.affiliation: Department of Trauma and Handsurgery, Heinrich Heine University Hospital Düsseldorf, Moorenstr, 5, 40225 Düsseldorf, Germany. Jan.Grassmann@med.uni-duesseldorf.de.
authors.forename: Jan-Peter
authors.initials: JP
authors.lastname: Grassmann
authors.forename: Marcel
authors.initials: M
authors.lastname: Betsch
authors.forename: Michael
authors.initials: M
authors.lastname: Wild
authors.forename: Mohssen
authors.initials: M
authors.lastname: Hakimi
authors.forename: Joachim
authors.initials: J
authors.lastname: Windolf
authors.forename: Pascal
authors.initials: P
authors.lastname: Jungbluth
mesh_terms: D058666:Adrenergic beta-2 Receptor Agonists / Q000627:therapeutic use; D019559:Capillary Leak Syndrome / Q000150:complications* / Q000188:drug therapy; D003161:Compartment Syndromes / Q000209:etiology / Q000601:surgery; D019299:Decompression, Surgical; D018495:Fluid Shifts; D005542:Forearm / Q000601:surgery; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D059325:Intra-Abdominal Hypertension / Q000209:etiology* / Q000601:surgery; D007866:Leg / Q000601:surgery; D060046:Maintenance Chemotherapy; D008297:Male; D008875:Middle Aged; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction; D013726:Terbutaline / Q000627:therapeutic use; D013806:Theophylline / Q000627:therapeutic use; D013848:Thigh / Q000601:surgery
chemical_list: D058666:Adrenergic beta-2 Receptor Agonists; D005938:Glucocorticoids; D010726:Phosphodiesterase Inhibitors; D011239:Prednisolone; D013806:Theophylline; D013726:Terbutaline