Systemic Capillary Leak Syndrome Associated with Compartment Syndrome

Matsumura, Masami; Kakuchi, Yasushi; Hamano, Ryoko; Kitajima, Susumu; Ueda, Akihito; Kawano, Mitsuhiro; Yamagishi, Masakazu

doi:10.2169/internalmedicine.46.0254

Cited by 18 publications

(12 citation statements)

References 13 publications

(8 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This is followed by an extravasation phase where capillary leakage leads to the triad of hypotension, hemoconcentration and hypoalbuminemia [6][7][8][9]. Aggressive fluid resuscitation may lead to a compartment syndrome during the extravasation phase, and rhabdomyolysis may occur, as in this case [20,21]. Acute tubular necrosis, ischemic brain injury or ischemic hepatitis have been reported.…”

Section: Discussionmentioning

confidence: 83%

Systemic Capillary Leak Syndrome Presenting as Apparent Polycythemia Vera: A Critical Diagnostic Dilemma

Wu¹

2014

J Hematol

View full text Add to dashboard Cite

Polycythemia vera presents with true erythrocytosis (elevated red cell mass) and an expanded plasma volume, while apparent erythrocytosis presents with plasma volume contraction and a normal red cell mass. Here we report a case with recurrent episodes of severe apparent erythrocytosis, closely mimicking polycythemia vera, due to a rare condition known as systemic capillary leak syndrome (SCLS). This syndrome typically presents with the triad of hypotension, hemocencentration and hypoalbuminemia, combined with a monoclonal serum M-protein. Empirical treatment with intravenous immune globulin (IVIG) has successfully led to resolution of symptoms and correction of hemoconcentration in several reported cases as well as in our patient's case. It is important for clinicians to differentiate true erythrocytosis from apparent erythrocytosis, and to be aware of this rare syndrome causing the latter, as aggressive phlebotomy and overtreatment with crystalloid fluids could result in serious adverse events and should be avoided.

show abstract

Section: Discussionmentioning

confidence: 83%

Systemic Capillary Leak Syndrome Presenting as Apparent Polycythemia Vera: A Critical Diagnostic Dilemma

Wu¹

2014

J Hematol

View full text Add to dashboard Cite

show abstract

“…Further symptoms observed in patients are polydipsia, dizziness, hypotension, generalized edema (as well as cerebral, pulmonary, macular or epiglottic edema), weight gain, pleural or pericardial effusion, and renal dysfunction that could end in renal failure [5]. Potential laboratory markers found in patients with SCLS are elevated white blood cell count, elevated hematocrit (>55%), low serum total protein and albumin, or monoclonal immunoglobulinopathy such as increased immunoglobulin G (IgG) kappa, IgG lambda, or immunoglobulin A (IgA) lambda levels [5-7]. Most of the patients in the reported cases were previously healthy, indicating that SCLS appears to belong to diseases with an idiopathic genesis.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2014

View full text Add to dashboard Cite

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.

show abstract

“…The hallmark of the systemic capillary leak syndrome is the triad of acute hypovolaemia, hypoalbuminaemia without albuminuria and oedema of the limbs. [1][2][3][4][5][6][7][8][9] Up to 70% of intravascular fluid may shift rapidly into the interstitial space, causing rapid and dramatic rises in the concentrations of marrow elements, which normalise one to four days later when the interstitial fluid is recruited back into the venules. The oedema of the limbs may be severe enough to cause a compartment syndrome.…”

Section: Discussionmentioning

confidence: 99%

Four-limb compartment syndrome associated with the systemic capillary leak syndrome

Simon

Taylor

Bayley

et al. 2010

The Journal of Bone and Joint Surgery. British volume

View full text Add to dashboard Cite

Systemic capillary leak syndrome, or the Clarkson syndrome, is an extremely rare condition in which increased capillary permeability results in a massive shift of fluid into the extravascular space. This is followed rapidly by hypotensive shock, haemoconcentration, and, potentially, substantial oedema of the limbs resulting in an acute compartment syndrome. It is important for orthopaedic surgeons to be aware of this syndrome as our medical colleagues, who initially care for these patients, are less familiar with the diagnosis and the need for emergency management of the associated compartment syndrome should it develop. There have been fewer than 100 cases of this entity reported. This case report is the first to describe the subsequent development of a compartment syndrome in all four limbs. Clinical vigilance and continuous monitoring of intracompartmental pressure is necessary in these patients in order to help reduce limb-threatening complications.

show abstract

Systemic Capillary Leak Syndrome Associated with Compartment Syndrome

Abstract: Abstract

Cited by 18 publications

References 13 publications

Systemic Capillary Leak Syndrome Presenting as Apparent Polycythemia Vera: A Critical Diagnostic Dilemma

Systemic Capillary Leak Syndrome Presenting as Apparent Polycythemia Vera: A Critical Diagnostic Dilemma

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

Four-limb compartment syndrome associated with the systemic capillary leak syndrome

Contact Info

Product

Resources

About