Clinical course of pediatric large vascular anomalies located in the extremities

Oktay, Burcu; Kaçar, Ayşe Gonca; Özel, Simge Çınar; Ocak, Süheyla; Çelkan, Tiraje

doi:10.5152/turkarchpediatr.2021.20089

Cited by 5 publications

(4 citation statements)

References 22 publications

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“…This condition can result in elevated D‐dimer levels in extralesional blood, known as localized intralesional coagulopathy (LIC) 3,4 . In some cases, a decrease in fibrinogen levels may precede the development of disseminated intravascular coagulopathy (DIC), 5,6 although very few cases have been described in the literature 7–10 . Asymptomatic lesions are common, but they may cause unpredictable pain crises, mostly due to exacerbation of LIC.…”

Section: Introductionmentioning

confidence: 99%

“…3,4 In some cases, a decrease in fibrinogen levels may precede the development of disseminated intravascular coagulopathy (DIC), 5,6 although very few cases have been described in the literature. [7][8][9][10] Asymptomatic lesions are common, but they may cause unpredictable pain crises, mostly due to exacerbation of LIC. The emotional distress caused by these episodes can lead to a lower quality of life for patients affected by VM compared with the general population.…”

Section: Introductionmentioning

confidence: 99%

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Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study

Nava y Hurtado,

Monzon Manzano,

Viana‐Huete

et al. 2024

Pediatric Blood & Cancer

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ObjectiveThe occurrence of unpredictable pain crises are the principal determinant of the quality of life for patients with venous malformations (VM). A definite coagulation phenomenon, characterized by an increase in D‐dimer levels and the presence of phleboliths within the malformation, has been previously reported. By applying Virchow's triad and evaluating intralesional samples, our objective is to delineate the coagulation profile and the extent of endothelial dysfunction within the malformation.MethodsWith the authorization of the Ethics Committee, a research project was undertaken on intralesional and extralesional blood samples from 30 pediatric patients afflicted with spongiform VM. Thromboelastometry analyses were performed using ROTEM Sigma, and the concentration of syndecan‐1 was determined by ELISA.ResultsIn the ROTEM analyses, the A5, A10, and maximum clot firmness (MCF) values were below the established reference ranges in the intralesional samples in both the EXTEM and INTEM assays, indicating that intralesional clots had significant instability. Furthermore, during the investigation of the delayed fibrinolysis phase using recombinant tissue plasminogen activator (rtPA) in EXTEM analysis, widespread hyperfibrinolysis was observed intralesional. Additionally, analysis of syndecan‐1 showed significant differences between extralesional and intralesional levels (p < .026) and controls (p < .03), suggesting differences in the state of endothelium.ConclusionsFor the first time, we developed a comprehensive understanding of the coagulopathic profile of VM and the role of endothelial dysfunction in its pathogenesis. These findings will enable the implementation of targeted therapies based on the individual coagulation profiles.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study

Nava y Hurtado,

Monzon Manzano,

Viana‐Huete

et al. 2024

Pediatric Blood & Cancer

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“…17,20 Furthermore, the classification of patients often lacks clearly described clinical criteria for diagnosis, the classification used or the handling of unclear diagnoses. 16,17,20,27 As a result, despite the some descriptions of the above-mentioned populations, it becomes almost impossible to compare them.…”

Section: Introductionmentioning

confidence: 99%

Clinical phenotype of adolescent and adult patients with extracranial vascular malformation

Tuleja¹,

Bernhard²,

Hamvas³

et al. 2023

Journal of Vascular Surgery: Venous and Lymphatic Disorders

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“…hemangiomas) and vascular malformations [1]. Vascular malformations are congenital heterogeneous conditions characterized by abnormal vessel development [2]. They can be broadly classified into slow-flow (capillary, venous and lymphatic) and fast-flow (arteriovenous) malformations.…”

Section: Introductionmentioning

confidence: 99%

Successful Management of a Rare Manifestation of Intramuscular Venous Malformation in a Young Adult: A Case Report

Sharma¹,

Bhandari²,

Rai³

2023

Cureus

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Venous malformations are the most common type of congenital vascular lesions resulting from abnormal embryonic development of vessels. Typical venous malformations are easily diagnosed by skin color changes, focal edema, or pain as they are mostly present in the skin and subcutaneous tissue. Venous malformations in the skeletal muscles, however, have the potential to be missed because their involved sites are invisible. We describe a 15-year-old patient with extensive intramuscular venous malformations in the lower extremity with special emphasis on diagnosis and treatment.

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Clinical course of pediatric large vascular anomalies located in the extremities

Cited by 5 publications

References 22 publications

Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study

Assessing coagulopathy and endothelial dysfunction in pediatric venous malformation: A thromboelastometry and syndecan‐1 study

Clinical phenotype of adolescent and adult patients with extracranial vascular malformation

Successful Management of a Rare Manifestation of Intramuscular Venous Malformation in a Young Adult: A Case Report

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