MRI phenotypes of localized intravascular coagulopathy in venous malformations

Koo, Kevin S. H.; Dowd, Christopher F.; Mathes, Erin F.; Rosbe, Kristina W.; Hoffman, William Y.; Frieden, Ilona J.; Hess, Christopher P.

doi:10.1007/s00247-015-3389-6

Cited by 27 publications

(25 citation statements)

References 20 publications

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“…It is now well recognised that extensive VMs are commonly associated with systemic coagulation profile abnormalities . The relative slow‐flow within the lesion results in thrombosis through the triggering of the fibrinolytic cascade.…”

Section: Introductionmentioning

confidence: 99%

“…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%

“…Combined adult and paediatric case series have shown patients with large, extensive or multiple VMs have elevated D‐dimer levels in 42–58% of patients. A total of 6–10% of patients have concurrent low fibrinogen levels, usually with normal platelet counts …”

Section: Introductionmentioning

confidence: 99%

“…Most clinical evaluations of this relationship have been via combined adult and paediatric retrospective case series. Most have shown elevated D‐dimers to be correlated with VM surface area, intramuscular involvement, bony involvement, visceral VMs and the presence of phleboliths . However, whilst studies, have shown a correlation between the size and extent of VMs with LIC amongst the general VM population, few have looked at VM volume, multifocal VMs or the specific situation of children with VMs .…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

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%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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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“…However, if the ocular muscle or even the optic nerve is invaded, the symptoms can be serious. [45] In rare cases, ocular venous malformation can affect the orbital bone. [6] In general, ocular venous malformation is believed to be benign, but its invasive behavior can also lead to ocular dysfunction and even blindness.…”

Section: Introductionmentioning

confidence: 99%

Pingyangmycin Pretreatment Influences the Biological Behavior of Ocular Venous Malformation and Relates with Galectin-3 Expression

Qiao

Liu

2017

Chinese Medical Journal

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Background:Galectin-3 (Gal-3) plays a role in the mechanisms underlying ocular venous malformation. We conducted this study to investigate the effect of pingyangmycin pretreatment on the Gal-3 expressions and biological behavior of ocular venous malformation.Methods:Tissue samples were collected from 136 patients with ocular venous malformation. Patients were randomly divided into pingyangmycin (n = 69) and nonpingyangmycin group (n = 67). Patients in the pingyangmycin group received a local injection of 0.02% pingyangmycin once every 2 days for 2 weeks (7 doses) before removal surgery, whereas patients in the nonpingyangmycin group underwent removal surgery without local injection. The protein and messenger RNA (mRNA) expression of Gal-3 were detected by using immunohistochemistry and in situ hybridization.Results:Gal-3 protein was expressed in 35 (52%) of 67 samples in the nonpingyangmycin group and in 19 (28%) of 69 samples in the pingyangmycin group (P < 0.05). Gal-3 mRNA expression was detected in 39 (58%) of 67 samples in the nonpingyangmycin group and 22 (32%) of 69 samples in the pingyangmycin group (P < 0.05). The higher Gal-3 expressions were detected in samples with deeper invasiveness than those with superficial invasiveness before (χ2 = 12.720 and 13.369, respectively, both P < 0.05) and after pingyangmycin treatment (χ2 = 8.429 and 4.590, respectively, both P < 0.05). It was more frequently detected in mesh-like lesions with unclear boundary than round lesions with clear boundary before (χ2 = 30.291 and 41.466, respectively, both P < 0.05) and after pingyangmycin treatment (χ2 = 14.619 and 15.130, respectively, both P < 0.05). Pingyangmycin treatment led to a significant difference in Gal-3 expressions at both protein and mRNA levels (χ2 = 8.664 and 9.524, respectively, both P < 0.05).Conclusions:Gal-3 expression may be involved in the development and invasiveness of ocular venous malformation, and pingyangmycin can inhibit Gal-3 expression, indicating a role of pingyangmycin treatment before the removal of ocular venous malformation.

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Value of Dynamic Contrast‐Enhanced (DCE) MRI in Predicting Response to Foam Sclerotherapy of Venous Malformations

Xia

Yuan

et al. 2021

Magnetic Resonance Imaging

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Background Preoperative imaging assessment of venous malformations (VMs) and prediction of foam sclerotherapy efficacy might be achievable by DCE‐MRI but elaborate quantitive analysis was absent. Purpose To evaluate the value of DCE‐MRI in predicting the effectiveness of foam sclerotherapy in VMs. Study Type Retrospective. Population Fifty‐five patients (M:F = 17:38; mean age ± SD, 15.4 ± 13.0 years) with VMs. Field Strength/Sequence Three Tesla MRI with 3D T1‐weighted volume interpolated body examination. Assessment Patients who underwent pretreatment DCE‐MRI were divided into “effective” and “ineffective” groups according to the response to foam sclerotherapy. Clinical characteristics and morphologic features were assessed. The semiquantitative parameters, such as maximum intensity time ratio (MITR), enhancement ratio (ER), and Slope, were obtained from ROI and volume of interest (VOI). The quartile and mean values of these parameters were acquired from VOI, while mean values denoted as Mean# were acquired from ROI. Establishment of two predictive models was based on ROI and VOI respectively. Model 1 was based on morphologic parameters and ROI semiquantitative parameters, while model 2 was based on morphologic parameters and VOI semiquantitative parameters. Statistical Analysis Mann–Whitney U‐test, Cohen's kappa, multivariate logistic regression analysis (backward stepwise), and ROC analyses. Results The lesion classification, presence of phlebolith, semiquantitative parameters of VOI (quartile and mean of MITR), and semiquantitative parameters of ROI (Slopemean#, MITRmean#) were significantly different between two groups. Lesion classification (P = 0.002) and MITRmean# (P = 0.027) were independent predictors for poor efficacy in model 1 as determined by multivariate binary logistic regression analysis. For model 2, lesion classification (P = 0.006) and MITR25 (P = 0.001) were independent predictors. The predictive model based on VOI (AUC = 0.961) performed better than that based on ROI (AUC = 0.909) in predicting therapeutic response. Data Conclusion DCE‐MRI is promising in predicting the response to foam sclerotherapy for VMs. The whole lesion VOI‐based model showed better performance and could instruct surgical approach in the future. Evidence Level 3 Technical Efficacy Stage 4

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MRI phenotypes of localized intravascular coagulopathy in venous malformations

Abstract: LIC is associated with larger lesion size, visualized phleboliths, truncal location and spongiform morphology on MRI in venous malformations, suggesting that lesions with larger capacitance, slower flow and less physiological compression are more likely to be associated with coagulopathy.

Cited by 27 publications

References 20 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

Pingyangmycin Pretreatment Influences the Biological Behavior of Ocular Venous Malformation and Relates with Galectin-3 Expression

Value of Dynamic Contrast‐Enhanced (DCE) MRI in Predicting Response to Foam Sclerotherapy of Venous Malformations

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