Localised intravascular coagulation complicating venous malformations in children: Associations and therapeutic options

Zhuo, Kevin Y; Russell, Susan; Wargon, Orli; Adams, Susan

doi:10.1111/jpc.13461

Cited by 45 publications

(70 citation statements)

References 34 publications

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“…Venous malformations and any of the combined malformations with a prominent venous or lymphatic component demonstrate slow blood flow. Slow‐flow vascular malformations are affected by intravascular stagnation and clotting that causes localized intravascular coagulopathy (LIC), a condition characterized and measured systemically by elevated D‐dimer levels, and when severe by concomitant low fibrinogen and less often by platelet reductions . These lab abnormalities reflect the extent of the activation and consumption of coagulation factors caused by the slow flow within the enlarged venous channels of a malformation triggering the coagulation cascade .…”

Section: Introductionmentioning

confidence: 99%

“…Slow‐flow vascular malformations are affected by intravascular stagnation and clotting that causes localized intravascular coagulopathy (LIC), a condition characterized and measured systemically by elevated D‐dimer levels, and when severe by concomitant low fibrinogen and less often by platelet reductions . These lab abnormalities reflect the extent of the activation and consumption of coagulation factors caused by the slow flow within the enlarged venous channels of a malformation triggering the coagulation cascade . This derangement in coagulation can lead to localized thrombosis and/or bleeding that results in pain, functional limitations, and, in severe cases, progression to disseminated intravascular coagulopathy (DIC).…”

Section: Introductionmentioning

confidence: 99%

“…This derangement in coagulation can lead to localized thrombosis and/or bleeding that results in pain, functional limitations, and, in severe cases, progression to disseminated intravascular coagulopathy (DIC). DIC can be a serious risk or problematic during surgical or interventional procedures when the malformed vessels are disturbed or manipulated . While all patients with a vascular malformation containing a venous component are at risk of LIC, risk is increased in correlation with increased size and extent.…”

Section: Introductionmentioning

confidence: 99%

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Effect of sirolimus on coagulopathy of slow‐flow vascular malformations

Mack

Verkamp

Richter

et al. 2019

Pediatric Blood & Cancer

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Background/Objectives: Stagnant blood flow present in slow-flow vascular malformations can lead to localized intravascular coagulopathy (LIC), measured by elevated D-dimer levels, low fibrinogen, and/or thrombocytopenia. LIC can lead to localized thrombosis and/or bleeding, resulting in pain, swelling, and functional limitations. Patients with complex vascular malformations treated with sirolimus show clinical improvement in these symptoms. We hypothesized that the clinical benefits of sirolimus may correlate with improvements in coexisting LIC. Design/Methods: A retrospective chart review was performed, including D-dimer, fibrinogen, and platelet count, in patients with slow-flow vascular malformations treated with sirolimus. Laboratory values were assessed at three time points (presirolimus, 1-3 months postsirolimus, and last clinic visit). Clinical response, as defined by decreased pain and swelling, was extracted from the record.Results: Thirty-five patients at our vascular anomalies center had been prescribed sirolimus between 2014 and 2017. Fifteen patients (12 combined slow-flow vascular malformations and three pure venous malformations) remained after excluding patients that did not have adequate records or a venous component to their vascular malformation. Patients who did not adhere to the treatment were also excluded. All 15 had elevated D-dimer levels prior to treatment and there was a statistically significant decrease in D-dimer levels following treatment with sirolimus. Symptomatic improvement of pain and swelling was reported after 3 months of starting sirolimus in 13/15 patients. Conclusion:This study suggests that sirolimus improves coagulopathy in slow-flow vascular malformations, as evidenced by reduced D-dimer levels. Improvement in LIC symptoms also correlates with sirolimus-corrected coagulopathy.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Effect of sirolimus on coagulopathy of slow‐flow vascular malformations

Mack

Verkamp

Richter

et al. 2019

Pediatric Blood & Cancer

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“…Since this initial success, the use of sirolimus has expanded rapidly (8). While initially explored for KHE and lymphatic malformation (LM) (26)(27)(28), sirolimus is now being used broadly throug hout the VA spectrum and is showing promising results in both systemic administration (10,(29)(30)(31) and topical administration (32)(33)(34).…”

mentioning

confidence: 99%

Oral and Topical Sirolimus for Vascular Anomalies: A Multicentre Study and Review

Sandbank¹,

Molho‐Pessach²,

Farkas³

et al. 2019

Acta Derm Venerol

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This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Acta Derm Venereol 2019; 99: 990-996 990 SIGNIFICANCEIn recent years, sirolimus, an mTOR inhibitor, has become a new therapeutic option for patients with vascular anomalies that do not respond to other treatments. We report here a retrospective series of 19 young adults, children and neonates with complex vascular anomalies treated with sirolimus. Overall, clinical improvement was demonstrated in 15 patients (79%). In addition, an up-to-date literature review was performed and 150 cases of vascular anomalies treated with sirolimus were analysed. The results suggest that sirolimus is an effective and safe treatment. Further study is needed into the early use of sirolimus in cases of low-flow lesions, and overgrowth syndromes with low-flow components.Vascular anomalies (VAs) may be associated with significant morbidity and mortality. The aim of this study was to evaluate the efficacy and safety of sirolimus (rapamycin) in the treatment of children and young adults with complicated VAs. A retrospective chart was created that included 19 patients treated with sirolimus for complicated VAs. Concurrently, a search of the PubMed database for VA cases treated with sirolimus was conducted. Descriptive analysis was performed and the efficiency rate of sirolimus was calculated. This retrospective study included 19 patients, 17 of whom were treated with oral sirolimus and 2 with topical sirolimus. Clinical improvement occurred in 15 patients (79%). One patient experienced near-complete resolution. Only 2 patients showed poor response and discontinued treatment. The literature review analysed 150 cases of VA treated with sirolimus. Sirolimus was efficient in 85% of cases, including 5 cases of complete resolution. Sirolimus appears to be an effective and safe treatment for children and young adults with complicated VAs.

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“…Anticoagulation therapy, such as subcutaneous low-molecular weight heparin (LMWH), can be administered to patients with a risk of thromboemboli. Surgery may enable a permanent improvement in patients with LIC by reducing the blood pooling arising from venous malformations; however, such procedures should be performed prior to the development of LIC or DIC [ 6 ]. An appropriate treatment for LIC or DIC associated with vascular malformations remains to be determined, as only low-level evidence is available.…”

Section: Introductionmentioning

confidence: 99%

Successful treatment with dabigatran for consumptive coagulopathy associated with extensive vascular malformations

Yasumoto

Ishiura

Narushima

et al. 2017

Blood Coagulation &Amp; Fibrinolysis

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Vascular malformation is occasionally complicated by consumptive coagulopathy, known as localized intravascular coagulopathy (LIC), which is characterized by a reduced fibrinogen level, an elevated D-dimer level and a normal platelet count. We report the case of a 17-year-old Japanese girl who presented with LIC secondary to extensive vascular malformations, whose condition had progressed to disseminated intravascular coagulation (DIC). She suddenly presented with severe anaemia, despite the absence of obvious bleeding, and she began to require regular red blood cell (RBC) transfusions. As she was suffering from paroxysmal atrial fibrillation, we treated her with dabigatran, after obtaining informed consent. Immediately after the administration of dabigatran, the results of clotting tests improved dramatically. Seven months later, she has not required any RBC transfusions, and the dabigatran treatment has been well tolerated. The present case report suggests that dabigatran may be a useful treatment option for patients with DIC associated with vascular malformations.

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Localised intravascular coagulation complicating venous malformations in children: Associations and therapeutic options

Cited by 45 publications

References 34 publications

Effect of sirolimus on coagulopathy of slow‐flow vascular malformations

Effect of sirolimus on coagulopathy of slow‐flow vascular malformations

Oral and Topical Sirolimus for Vascular Anomalies: A Multicentre Study and Review

Successful treatment with dabigatran for consumptive coagulopathy associated with extensive vascular malformations

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