Pulmonary thromboembolic events in patients with congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities and Klippel-Trénaunay syndrome

Reis, Joseph; Alomari, Ahmad I.; Trenor, Cameron C.; Adams, Denise M.; Fishman, Steven J.; Spencer, Samantha A.; Shaikh, Raja; Lillis, Anna P.; Surnedi, Murali K.; Chaudry, Gulraiz

doi:10.1016/j.jvsv.2018.01.015

Cited by 43 publications

(22 citation statements)

References 14 publications

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“…The presence of LIC in an individual patient is heralded by the presence of palpable phleboliths – small calcific venous wall or intra‐luminal deposits, and has been shown to be proportional to VM size and extent . VMs involving the muscle, bone and viscera, as opposed to just skin and subcutaneous fat, are also significantly more likely to be associated with LIC, as are VMs affecting the persistent embryonic veins associated with PIKC3A Related Overgrowth Spectrum and Klippel‐Trenaunay syndrome . The exact reason why many of these embryonic veins persist beyond fetal life is undefined .…”

Section: Introductionmentioning

confidence: 99%

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

Zhuo

Russell

Wargon

et al. 2017

J Paediatrics Child Health

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Venous malformations are slow-flow congenital vascular malformations that enlarge as the child ages and may be associated with localised intravascular coagulation, a consumptive coagulopathy characterised by elevated D-dimer and decreased fibrinogen levels. The authors review the known correlations between localised intravascular coagulation and venous malformation number, size and planes involved, and call attention to the concept of the progression of localised intravascular coagulopathy as the child ages and their venous malformations enlarge. The authors also discuss the identified therapeutic options for its investigation, management and treatment, including compression garments, anti-coagulation therapy, sclerotherapy, endovascular laser, surgical excision and sirolimus (rapamycin). Evidence for protocol improvements that may be instigated for the optimal physical and medical therapy of venous malformations complicated by localised intravascular coagulopathy is reviewed.

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Section: Introductionmentioning

confidence: 99%

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

Zhuo

Russell

Wargon

et al. 2017

J Paediatrics Child Health

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“…Slow-flow vascular malformations, especially those with a large venous component can frequently be complicated by intralesional thrombosis and if ectatic vessels are connected to larger feeding veins this can put patients at risk for venous thromboembolism (VTE) [5][6][7][8][9][10][11] . Although the mechanism is not fully elucidated, venous stagnation can lead to activation and consumption of coagulation factors and platelets and result in localized intravascular coagulopathy (LIC).…”

Section: Localized Intravascular Coagulopathymentioning

confidence: 99%

How we approach coagulopathy with vascular anomalies.

Mack¹,

Crary²

2021

Preprint

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Some vascular anomalies can present with challenging hematologic aberrations. Kaposiform hemangioendothelioma (KHE) may be complicated with Kasabach-Merritt phenomenon (KMP) and stagnant blood flow in slow flow malformations can promote activation and consumption of coagulation factors which results in bleeding and clotting known as localized intravascular coagulopathy (LIC). These patients can experience significant morbidity secondary to pain due to thrombosis and are at higher risk of hematologic complications during surgical procedures. No standard of care has been established to prevent or manage these complications. This review focuses on the management of coagulopathy in children and adults with vascular anomalies.

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“…Thrombin is generated and localized thrombosis can occur with the presence of phleboliths on imaging, leading to significant pain and functional limitations. Thrombosis can lead to thromboembolic disease such as pulmonary embolism and phlebitis 3 . Severe LIC may even progress to disseminated intravascular coagulopathy (DIC), marked by systemic consumption of platelets and coagulation factors, which can be life‐threatening and result in severe bleeding, especially during invasive surgical procedures 1,4,5 …”

Section: Introductionmentioning

confidence: 99%

Analyzing coagulation dynamics during treatment of vascular malformations with thromboelastography

Mack

Pierce

Richter

et al. 2020

Pediatric Blood & Cancer

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Background/Objectives: Slow-flow vascular malformations are abnormal vessels that can lead to activation and consumption of coagulation factors and thrombosis, known as localized intravascular coagulopathy (LIC). Most clinical and research evidence of vascular malformation hemostasis relies on conventional coagulation studies, which may not provide a complete picture. Thromboelastograpy (TEG) is a tool that can provide real-time assessment of a patient's coagulation dynamics, and may allow for a more individualized treatment approach. We hypothesized that patients with slowflow vascular malformations will have changes in TEG parameters peri-procedure that will help predict blood product or medication administration. Design/Methods: Institutional Review Board approved prospective study of patients with slow-flow vascular malformations undergoing a sedated, minor procedure. TEG and conventional coagulation studies were obtained preprocedure, 15 min, and when possible, at 30 min after the start of the procedure. Results: Twenty-five patients were enrolled. Median age was 15 years (range 3-47 years). Procedures included laser and/or sclerotherapy. There were no changes in TEG parameters from baseline to 15 min or 30 min. The following decreased from baseline to 15 min: fibrinogen 313 to 287 mg/dL (P = .001), D-dimer 1.3 to 1.1 mg/L (P = .02), hemoglobin 12.8 to 11.8 g/dL (P = .001), and platelet count 272 000 to 256 000 (P = .006). No patient had a bleeding/thrombotic complication during or within 1 week postprocedure. Conclusion: We saw no change in TEG parameters or bleeding or clotting complications despite significant numerical changes in conventional coagulation profiles, suggesting that conventional studies may not be as useful in determining risks of bleeding or thrombotic complications peri-procedure for minor procedures.

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Pulmonary thromboembolic events in patients with congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities and Klippel-Trénaunay syndrome

Cited by 43 publications

References 14 publications

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

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

How we approach coagulopathy with vascular anomalies.

Analyzing coagulation dynamics during treatment of vascular malformations with thromboelastography

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