Choroidal Effusion Mimicking Uveal Melanoma: A Novel Presentation of Idiopathic Systemic Capillary Leak Syndrome

2021 ◽  
pp. 1-6
Author(s):  
Beatrice Y. Brewington ◽  
Srinivas Kondapalli ◽  
Shaili S. Kothari ◽  
Samir V. Parikh ◽  
Colleen M. Cebulla
Keyword(s):  
Uveal Melanoma ◽  
Medical Condition ◽  
White Male ◽  
Choroidal Melanoma ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Severe Hypotension ◽  
Choroidal Effusion ◽  
Uveal Effusion

<b><i>Background:</i></b> Idiopathic systemic capillary leak syndrome (ISCLS, also known as Clarkson’s disease) is a rare medical condition characterized by episodes of capillary endothelial cell dysfunction with leakage of fluid into the interstitial space resulting in severe hypotension, hemoconcentration, hypoalbuminemia, and generalized edema. Each episode can result in multiorgan failure due to systemic hypoperfusion. <b><i>Case Presentation:</i></b> We report a case of uveal effusion, mimicking uveal melanoma, associated with ISCLS following viral infection. A 74-year-old white male was evaluated in our ocular tumor clinic for a large intraocular mass in the right eye concerning for choroidal melanoma. We completed a review of the literature and list clinical recommendations for these cases. ISCLS, although rare, was a significant diagnostic consideration in this patient. Due to the high mortality rate of this condition, accurate diagnosis and prompt treatment was critical. We hypothesize that the mechanism of choroidal effusion development was due to reduced oncotic pressure from rapid decrease in serum albumin. Increased permeability of choroidal capillaries may be an additional mechanism leading to uveal effusion. <b><i>Conclusion:</i></b> With treatment, the patient had complete resolution of his choroidal effusion with no recurrence of his ISCLS. Further research should be considered on the role of viral infections in the pathogenesis of ISCLS.

Systemic Capillary Leak Syndrome Mimicking Polycythemia Vera.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4909-4909
Author(s):  
Arif H. Kamal ◽  
Philip R. Greipp
Keyword(s):  
Polycythemia Vera ◽  
Fluid Resuscitation ◽  
Low Dose ◽  
Capillary Leak Syndrome ◽  
Laboratory Evaluation ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Monoclonal Protein ◽  
Severe Hypotension ◽  
Patient Reported

Abstract Systemic capillary leak syndrome (SCLS) is a vascular disorder characterized by intermittent episodes of hypotension and edema from increased vascular permeability. Patients present with resultant dramatic shifts in plasma volume from intravascular to extravascular spaces. Diagnosis requires observation of severe hemoconcentration and hypoalbuminemia during acute attacks. Typically, laboratory evaluation of hematocrit and albumin is normal when patients are not symptomatic. Concomitantly, and of unknown significance, a monoclonal protein is also frequently discovered. We present the case of a patient whose increased hematocrit suggested polycythemia vera, but whose total clinical picture of hypotension, hemoconcentration and hypoalbuminemia confirmed the diagnosis of SCLS. A 54-year old gentleman was referred for evaluation of polycythemia vera that was diagnosed after a series of dramatic hospitalizations for near-syncope. The patient had no chronic illnesses, negative family history for hematologic abnormalities, and was otherwise in his usual state of good health when a complaint of sudden lightheadedness and pre-syncope led to an emergency room evaluation. There he was found to be severely hypotensive and was resuscitated with intravenous fluids. Initial laboratory evaluation showed a hematocrit of 60%. Further workup revealed an increased red cell mass. For the presumptive diagnosis of polycythemia vera, the patient was phlebotomized once and, interestingly, did not require any further phlebotomy for almost three years. During this interim, he remained relatively asymptomatic with only occasional bouts of lightheadedness that would abort with increased fluid hydration. Phlebotomy was again used when the patient presented with near-syncope, hypotension, and vomiting accompanied by significant extremity swelling. The hematocrit was found to be greater than 80%. The patient recovered with aggressive fluid resuscitation and was discharged on a low-dose aspirin with the continued empiric diagnosis of polycythemia vera. During that year, the patient recovered from four other similar episodes that resolved after fluid resuscitation and where phlebotomy was used. The most severe episode involved a protracted ICU stay for severe hypotension complicated by severe extremity edema and resultant compartment syndrome and ischemic muscle necrosis. With the concern of adrenocortical dysfunction leading to these hypotensive episodes, the patient was started on low dose daily steroids. While on this, the patient reported less frequency and severity of his almost-daily symptoms of lightheadedness and edema. In fact, attempts at tapering the steroid dose down resulted in a return of the minor symptoms and with continued steroids, no further hospitalizations were needed. During our evaluation, we confirmed a normal hematocrit and albumin while asymptomatic and the presence of a monoclonal protein. This case represents a common clinical history of patients who are ultimately diagnosed with SCLS. Often, the significance of the observed hypotension in concert with laboratory evidence of hemocentration and hypoalbuminemia is not initially appreciated. Although current attack prophylaxis regimens for SCLS use terbutaline and theophylline, steroids had historically shown occasional benefit in some patients. SCLS still carries a significant mortality risk and prompt recognition of this syndrome and initiation of an appropriate prophylaxis regimen is crucial.

scholarly journals Systemic Capillary Leak Syndrome: Is Methylene Blue the Silver Bullet?

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Michele Umbrello ◽  
Marco Gardinali ◽  
Davide Ottolina ◽  
Giancarlo Zanforlin ◽  
Gaetano Iapichino
Keyword(s):  
Methylene Blue ◽  
Endothelial Permeability ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Severe Hypotension ◽  
Complete Reversal ◽  
Sparing Effect ◽  
Classical Triad ◽  
Silver Bullet

Background. Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by unexplained, recurrent episodes of transient, abrupt increase in endothelial permeability, leading to severe hypotension, generalized edema, and hemoconcentration.Case Report. We report the case of a patient suffering from systemic capillary leak syndrome and present a possible interpretation of the pathophysiology of this condition. Besides the classical triad of hypotension, edema, and hemoconcentration, we recorded increased levels of methemoglobin, an index of NO overproduction. We present a possible interpretation of the pathophysiology of this condition based on the fast and complete reversal of symptoms after methylene blue administration (which opposes NO-induced effects) and speculate that increased NO levels could be implicated in the pathophysiology of the capillary leak phase.Why should an emergency physician be aware of this?The safety of this treatment and its fluid- and cathecolamine-sparing effect deserve consideration and further research.

Bilateral simultaneous non-arteritic ischaemic optic neuropathy: a rare complication of idiopathic systemic capillary leak syndrome (SCLS)

2021 ◽  
Vol 14 (5) ◽  
pp. e242847
Author(s):  
Yan Ning Neo ◽  
Manvi Sobti ◽  
Hadi Zambarakji
Keyword(s):  
Optic Nerve ◽  
Visual Field ◽  
Optic Neuropathy ◽  
Capillary Leak Syndrome ◽  
Fluid Replacement ◽  
Visual Field Defects ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Severe Hypotension ◽  
Ischaemic Optic Neuropathy

We report a case of bilateral symmetrical superior visual field defects in a 72-year-old man first reported during the recovery from systemic capillary leak syndrome (SCLS). During the acute illness, he required extensive and prolonged fluid replacement and mechanical ventilation for severe hypotension, shock and multiorgan dysfunction. His visual field defect and optic nerve changes were consistent with a diagnosis of ischaemic optic neuropathy. These remained unchanged over 3 years and he retained excellent 6/7.5 visual acuity bilaterally. We hypothesised the mechanism of bilateral segmental infarction of the optic nerve head to be caused by the hypercoagulable and hypovolaemic state, in addition to pre-existing vascular disease and hypertension. This case highlights the importance of including optic nerve examination in the management plan of SCLS, particularly in individuals with underlying vascular risk factors.

scholarly journals Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome)

Blood ◽  
2012 ◽  
Vol 119 (18) ◽  
pp. 4321-4332 ◽  
Author(s):  
Zhihui Xie ◽  
Chandra C. Ghosh ◽  
Roshni Patel ◽  
Shoko Iwaki ◽  
Donna Gaskins ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Case Series ◽  
Fiber Formation ◽  
Capillary Leak Syndrome ◽  
Actin Stress Fiber ◽  
Vascular Endothelial ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Humoral Factors ◽  
Clarkson Disease

Abstract The systemic capillary leak syndrome (SCLS) is a rare disorder characterized by transient episodes of hypotensive shock and anasarca thought to arise from reversible microvascular barrier dysfunction. Although the high prevalence of a monoclonal gammopathy of unknown significance in SCLS suggests a pathogenic contribution of endogenous immunoglobulins, the mechanisms of vascular hyperpermeability remain obscure. Herein, we report clinical and molecular findings on 23 patients, the largest SCLS case series to date. Application of episodic SCLS sera, but neither the purified immunoglobulin fraction nor sera obtained from patients during remission, to human microvascular endothelial cells caused vascular endothelial cadherin internalization, disruption of interendothelial junctions, actin stress fiber formation, and increased permeability in complementary functional assays without inducing endothelial apoptosis. Intravenous immunoglobulin, one promising therapy for SCLS, mitigated the permeability effects of episodic sera. Consistent with the presence of endogenous, nonimmunoglobulin, circulating permeability factor(s) constrained to SCLS episodes, we found that vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang2), were elevated in episodic SCLS sera but not in remission sera. Ab-based inhibition of Ang2 counteracted permeability induced by episodic SCLS sera. Comparable experiments with anti-VEGF Ab (bevacizumab) yielded less interpretable results, probably because of endothelial toxicity of VEGF withdrawal. Our results support a model of SCLS pathogenesis in which nonimmunoglobulin humoral factors such as VEGF and Ang2 contribute to transient endothelial contraction, suggesting a molecular mechanism for this highly lethal disorder.

scholarly journals A Remarkable Coexistence of Systemic Capillary Leak Syndrome and Diabetes in an 11-Year-Old Boy: A Case Report and Review of the Literature

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Aysun Ata ◽  
Samim Özen ◽  
Damla Gökşen ◽  
Neslihan Edeer Karaca ◽  
Güzide Aksu ◽  
...  
Keyword(s):  
Urine Analysis ◽  
Early Recognition ◽  
Recovery Period ◽  
Capillary Leak Syndrome ◽  
Ivig Treatment ◽  
Acid Decarboxylase ◽  
Diabetic Patients ◽  
Healthy Children ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

Systemic capillary leak syndrome (ISCLS) is a rare disease characterized by unexplained reversible capillary hyperpermeability followed by hypoperfusion, hemoconcentration, and either hypoalbuminemia or total hypoproteinemia. An 11-year-old boy was admitted with vomiting, generalized edema, and hyperglycemia, which was preceded by 5 days of coryzal symptoms, lethargy, and oral aft, without fever. On physical examination, he had tachycardia and hypotension, with severe generalized systemic nonitchy edema, and the laboratory tests supported the conclusion that he had severe hemoconcentration with hemoglobin: 184 g/L, hematocrit: 51.3 %, urea: 20 mmol/L, blood glucose: 11.1 mmol/L, and albumin: 19 gr/L, with normal urine analysis. On the fourth day, the patient was diagnosed with ISCLS, by ruling out other causes of shock and hypoalbuminemia. Intravenous immunoglobulin (IVIG) treatment regimen was administered on two consecutive days (day five and day six). His edema decreased on the fifth day, and the patient was deemed clinically well. There was no compartment syndrome, rhabdomyolysis, or pulmonary edema in the recovery period. However, respiratory virus panel PCR was positive for respiratory syncytial virus (RSV) and enterovirus, which were thought to be the triggering cause of ISCLS. For the differential diagnosis of diabetes, his fasting serum glucose was 13.4 mmol/L, simultaneous C-peptide was 0.44 nmol/L, and HbA1c was 64 mmol/mol, and urine ketone was positive. However, antiglutamic acid decarboxylase, anti-insulin antibody, and islet cell antibody were negative. At the last outpatient visit, 22 months after the diagnosis, his insulin dose was still 0.4 IU/kg/day and HbA1c was 40 mmol/mol, and without prophylaxis, there was no ISCLS attack. Conclusion. Early recognition of ISCLS is important for therapeutic awareness, since it is very rare in childhood and occurs usually without any prior provoking factors in healthy children. With the increase in awareness of the disease, knowledge and experiences about pediatric patients may also increase. We think that our case will contribute to the literature since there have been no pediatric diabetic patients with ISCLS reported.

Masquerading as Septic Shock: Idiopathic Systemic Capillary Leak Syndrome

CHEST Journal ◽  
2014 ◽  
Vol 146 (4) ◽  
pp. 295A
Author(s):  
Kymberly McDonald ◽  
Vanessa Yap ◽  
Stephen Meng ◽  
Prashant Grover ◽  
Daniel Gerardi
Keyword(s):  
Septic Shock ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome

scholarly journals Systemic Capillary Leak Syndrome (Clarkson Syndrome) in Cancer Patients: A Systematic Review

2018 ◽  
Vol 7 (11) ◽  
pp. 418 ◽  
Author(s):  
Jae Shin ◽  
Keum Lee ◽  
I. Lee ◽  
Ji Oh ◽  
Dong Kim ◽  
...  
Keyword(s):  
Cancer Patients ◽  
Case Reports ◽  
Laboratory Data ◽  
Capillary Leak Syndrome ◽  
Treatment Modalities ◽  
Capillary Leak ◽  
Contributing Factors ◽  
Systemic Capillary Leak Syndrome ◽  
Increased Risk ◽  
Anti Cancer

Systemic capillary leak syndrome (SCLS) is a rare disease characterized by shock caused by capillary hyperpermeability. The disease can occur in cancer patients and effective therapeutic strategies have not been established yet. The aim of the study was to analyze the clinical and laboratory data, treatment modalities, and mortality rate of patients and to identify contributing factors leading to mortality of SCLS in cancer. We searched MEDLINE (inception to July 2018) and of 4612 articles, we identified 62 case reports on SCLS associated with cancer or cancer-related drugs in a total of 53 articles. SCLS was associated with cancer itself in 43.6%, with anti-cancer agents in 51.6% and bone marrow transplantation (BMT) in 4.8%. Among anti-cancer agents, granulocyte-colony stimulating factor (G-CSF) was the most frequently associated drug (14.6%), followed by interleukin (IL)-2 (11.4%). The most common associated malignancies were hematologic (61.3%) with non-Hodgkin lymphoma (22.7%) and multiple myeloma (12.9%) being the leading causes. Common symptoms and signs included dyspnea (27.4%), edema (67.7%), hypotension (32.2%), pleural effusion (29.0%), ascites (22.7%), oliguria (22.7%), and weight gain (21.0%). Patients with SCLS were treated with steroids (59.7%), volume replacement (33.8%), diuretics (24.2%), inotropes (9.6%), methylxanthines (12.8%), β2 agonists (4.8%), while intravenous immunoglobulins (IVIG) were administered in 2 patients (3.2%) only. Among sixteen deaths during follow-up, four were directly attributed to SCLS. Hematologic malignancies were associated with an increased risk for mortality (hazard ratio (HR) 8.820, 95% confidence interval (CI) 1.126–69.063, p = 0.038). Taken together, SCLS can be one important adverse event in cancer patients and careful monitoring of fluid volume is required in the management of SCLS.

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