Background: Vascular malformations are a rare and complex group of lesions which may present serious pitfalls in diagnosis and management. We sought to evaluate the efficacy and safety of our imaging protocol and therapeutic algorithm in the treatment of low-flow and high-flow vascular malformations in a large series of patients.
Methods: A prospective database of all patients treated by the multidisciplinary vascular malformation team at our institution between 2006 and 2011 was reviewed. Management decisions were based on patients' clinical profile as well as critical lesion characteristics, and included conservative care, sclerotherapy, embolization, surgical resection, or a combination of these modalities. Treatment goals and expectations were established by the patient and physician at the time of initial evaluation. An outcomes grading system based on patient- and physician-derived treatment goals and assessment of response to management was applied (1 = worse; 2 = unchanged, 3 = significantly improved, 4 = completely resolved), and postprocedural complications were identified.
Results: The 136 vascular malformations in 135 patients included 59 (43.7%) males and 76 (56.3%) females, ranging in age from under 1 year to 68 years (mean, 25.3 ± 17.0 years). In order to facilitate application of the therapeutic algorithm, all patients underwent dynamic contrast-enhanced magnetic resonance imaging to determine critical lesion characteristics, including flow quality and lesion extension. Of the 105 low-flow vascular malformations (LFVM), 23 (21.9%) were managed conservatively, 38 (36.2%) were treated with sclerotherapy (sodium tetradecyl sulfate, polidocanol, and/or ethanol), 18 (17.1%) were surgically resected, and eight (7.6%) were managed with a combination of modalities. Of the 31 high-flow vascular malformations (HFVM), eight (25.8%) were managed conservatively, eight (25.8%) were treated with transcatheter arterial embolization, six (19.4%) required embolization followed by sclerotherapy, and five (16.1%) were primarily resected. Patients in all groups managed conservatively had minimal alteration in status. Response to sclerotherapy in the LFVM group resulted in improvement in 32 (84.2%) patients, surgical resection resulted in improvement in 16 (88.9%) patients, and combination therapy resulted in improvement in eight (100%) patients. Treatment with embolization in the HFVM group resulted in improvement in seven (87.5%) patients, while combination therapy resulted in improvement in six (100%), and surgical resection led to improvement in four (80%). Complications were observed in six (6.8%) patients treated for LFVMs (0 with sodium tetradecyl sulfate or polidocanol, four with ethanol, two with resection), and two (7.4%) patients treated for HFVMs with embolization or combination therapy.
Conclusions: In this large cohort of vascular malformation patients, implementation of the proposed diagnostic and therapeutic algorithms in a multidisciplinary setting resulted in favorable outcomes with an acceptable complication rate in this challenging patient population.
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http://dx.doi.org/10.1016/j.jvs.2012.04.066 | DOI Listing |
The central nervous system (CNS) parenchyma has conventionally been believed to lack lymphatic vasculature, likely due to a non-permissive microenvironment that hinders the formation and growth of lymphatic endothelial cells (LECs). Recent findings of ectopic expression of LEC markers including Prospero Homeobox 1 (PROX1), a master regulator of lymphatic differentiation, and the vascular permeability marker Plasmalemma Vesicle Associated Protein (PLVAP), in certain glioblastoma and brain arteriovenous malformations (AVMs), has prompted investigation into their roles in cerebrovascular malformations, tumor environments, and blood-brain barrier (BBB) abnormalities. To explore the relationship between ectopic LEC properties and BBB disruption, we utilized endothelial cell-specific overexpression mutants.
View Article and Find Full Text PDFActa Neurochir (Wien)
January 2025
Department of Neurosurgery, Kanazawa University, 13-1 Takara-machi, Kanazawa, 9208641, Ishikawa, Japan.
A 54-year-old man presented with gait disturbances, urinary incontinence, and headache for 6 months. Head computed tomography indicated several high-density mass lesions in the quadrigeminal cistern, causing occlusive hydrocephalus. Digital subtraction angiography confirmed tentorial dural arteriovenous fistulae (AVF).
View Article and Find Full Text PDFInt J Hematol
January 2025
Associated Department With Mie Graduate School of Medicine, Mie Prefectural General Medical Center, Yokkaichi, Japan.
This study discusses disseminated intravascular coagulation (DIC) associated with solid cancers and various vascular abnormalities, both of which generally exhibit chronic DIC patterns. Solid cancers are among the most significant underlying diseases that induce DIC. However, the severity, bleeding tendency, and progression of DIC vary considerably depending on the type and stage of the cancer, making generalization difficult.
View Article and Find Full Text PDFCureus
December 2024
Neurosurgery, Southmead Hospital, North Bristol NHS, Bristol, GBR.
Cerebral arteriovenous malformations (AVMs) are tangles of abnormal vessels with early arteriovenous (AV) shunting that can lead to intracerebral hemorrhage, seizures, neurologic deficit, or headache. To date, only a few cases of carcinomas metastasizing to pre-existing cerebral AVMs have been reported in the literature. However, renal clear cell carcinoma (RCC) brain metastases that exhibit early AV shunting, where AVM pathology is not present, are extremely rare.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
September 2024
Division of Cardio-thoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine at Christus Children's Hospital of San Antonio, Texas.
Aneurysms of the superior vena cava are rare vascular malformations of systemic veins. This report presents the case of a 27-year-old woman with an incidental finding of mediastinal shadow widening on the chest roentgenogram that was confirmed by computed tomographic angiography to be a superior vena cava saccular aneurysm >4 cm in diameter. Surgical resection was recommended on the basis of aneurysmal size and shape and was performed through median sternotomy by using cardiopulmonary bypass.
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