Soft Tissue Vascular Malformations Research Articles

Prospective Observational Study of Pain Severity and Pain Interference Outcomes Following Percutaneous MRI-guided Laser Ablation or Cryoablation for Focal Painful Peripheral, Soft Tissue Vascular Malformations: 12-month Outcomes

Background: Percutaneous image-guided thermal ablation has emerged as a treatment option for vascular malformations (VM). However, prospective data on intermediate to long-term effectiveness with uniform follow-up are lacking. Purpose: To prospectively evaluate the 12-month pain severity and pain interference outcomes following percutaneous MRI-guided laser ablation and cryoablation of focal painful, peripheral soft tissue VM. Materials and Methods: Participants undergoing clinically indicated MRI-guided laser ablation and cryoablation for focal painful (worst pain ≥4 out of 10 on Visual Analog Scale), peripheral soft tissue vascular malformations (VM) were enrolled in an IRB-approved prospective, observational study after giving informed consent. Participants completed the Brief Pain Inventory questionnaire prior to ablation and 1, 3, 6, and 12-month postablation. Differences in pain severity and interference outcomes from baseline on a 0 to 10 scale were compared using a paired t-test. Results: Seventeen participants (15 female; mean age 28.3 ± 9.3 years) underwent 24 MRI-guided and monitored ablation sessions including laser (n = 21), cryoablation (n = 2), or both (n = 1) for treatment of painful slow-flow (n = 16) or high-flow (n = 1) VM. Median maximal VM diameter was 5.7 cm (range 1.1 to 31.0 cm). Mean (±SD) preablation worst pain score was 7.9 ± 1.4. There was a significant decrease in worst pain at 1-month postablation (–3.5 ± 2.9; P = .0007) that was sustained at 3 (–3.4 ± 3.2; P = .0007), 6 (–4.2 ± 3.6; P = .0003), and 12 months (–3.6 ± 3.9; P = .002), with similar results for average, current, and least pain scores. There was a significant improvement in pain interference outcomes at 12-month postablation: general activity (P = .018), walking ability (P = .008), work (P = .003), sleep (P = .007), and enjoyment of life (P = .033). Conclusions: These data suggest that MRI-guided and monitored thermal ablation is safe and provides early and sustained significant improvements in pain severity and pain interference outcomes during the first year of follow-up in participants with focal painful peripheral soft tissue VM.

Percutaneous MR Imaging–Guided Laser Ablation for the Treatment of Symptomatic Cervicofacial Vascular Malformations

PurposeTo evaluate the safety and effectiveness of percutaneous magnetic resonance (MR) imaging–guided laser ablation for the treatment of symptomatic soft tissue vascular malformations (VMs) in the face and neck. Materials and MethodsAn institutional review board–approved retrospective review was undertaken of all consecutive patients who underwent MR imaging–guided and monitored laser ablation for treatment of symptomatic, cervicofacial soft tissue VM. Preablation and postablation MR imaging findings were independently reviewed. Preablation and postablation VM sizes were documented. Preablation T2 signal characteristics and enhancement patterns as well as postablation change in both signal and enhancement were semiquantitatively assessed. Changes in VM size were compared using a paired t test. ResultsThirteen patients (women, 9; age, 14.5–69.5 years) with 13 VMs were treated for moderate-to-severe pain (n = 4), swelling/mass effect (n = 8), or predominantly cosmesis (n = 1) with 22 total ablation sessions. The baseline maximum VM diameter was 5.7 cm ± 4.2. At baseline, all VMs (100%) demonstrated variable T2-weighted signal hyperintensity and enhancement. For painful VM, the baseline pain score was 8 ± 1. Clinical follow-up was available for 10 patients. Of patients with available follow-up, 3 (100%) treated for moderate-to-severe pain and 7 (100%) treated for swelling/mass effect reported subjective complete or partial symptomatic relief. The patient treated predominantly for cosmetic reasons was lost to follow-up. Two patients (15.4%) experienced minor adverse events by the Society of Interventional Radiology standards. There were no major adverse events. ConclusionsMR imaging–guided and monitored percutaneous laser ablation is safe and effective for the treatment of symptomatic, cervicofacial VMs.

Cryoablation of Soft Tissues Low-Flow Vascular Malformations: Clinical Outcomes and Safety.

To report the clinical effectiveness and the safety of cryoablation in first or second-line therapy in symptomatic soft tissues vascular malformation, a mini-invasive therapeutic alternative to sclerotherapy or surgery. This retrospective and monocentric study included patients with symptomatic low-flow vascular malformation. The interventions were carried out under computed tomography (CT) scan, Cone-beam CT (CBCT) and/or USA guidance. Clinical response was evaluated by collecting the Numerical Rating Scale (NRS) for symptoms before and after the ablation. Safety was assessed based on criteria proposed by the Cardiovascular and Interventional Radiological Society of Europe. Imaging response was evaluated with post-ablation Magnetic Resonance Imaging. Twenty-one patients were included. Cryoablation was the first operative treatment for 12 patients (12/21, 57%). The remaining patients had already undergone surgery (2/21, 9%) or one or more sclerotherapy procedures (7/21, 33%).Symptoms assessed by Numerical Rating Scale dropped from a median of 7 [IQR 6-8] before the procedure to a median of 1 [IQR 0-2] after cryoablation (p < 0.001). Half of the patients declared a full effectiveness of cryoablation on their symptoms (11/21). No major complications and four minor adverse events (two skin lesions, two patients with neuropathic pain) were reported (19%). Lesional volume significantly decreased after cryoablation (median from 3.7 cm3 [1-10.4] to 0.25 cm3 [0-2.0], p < 0.001). Cryoablation seems to be a safe and effective first- or second-line therapy for soft tissue vascular malformations.

Endovascular balloon-assisted liquid embolisation of soft tissue vascular malformations: technical feasibility and safety

PurposeArteriovenous malformations (AVMs) are abnormal communications between arteries and veins without an intervening capillary system. The best endovascular treatment option for these is unclear and may involve multiple staged procedures using a variety of embolic materials. We report our initial experience using a modified version of a previously published neurointerventional technique to treat soft tissue AVMs with single-stage curative intent.Materials and methodsSoft tissue AVMs treated endovascularly using either sole arterial or combined arterial and venous balloon-assisted techniques with liquid embolic agents were retrospectively identified over a 3.5 year period (January 2017 to June 2020)) at two centres. Clinical, pre-operative radiological, procedural technical and post treatment details were recorded.ResultsSeven patients were treated for symptomatic soft tissue arteriovenous malformations. These AVMs were located in the peripheral limbs (five), tongue (one) and uterus (one). Curative treatment was achieved in 6/7 patients with one patient requiring a second treatment approximately 1 year later. A variety of liquid embolisation agents (LEAs) including sclerosants and polymers were used. Clinical success rate was 100% following treatment. One patient experienced expected temporary post-operative tongue swelling requiring tracheostomy occurred following embolisation of the lingual AVM. A minor complication in a second patient was due to an access site haematoma developed following treatment of the hand AVM requiring surgical intervention. No long-term sequelae or additional complications were observed.ConclusionEndovascular arterial and venous balloon assisted LEA embolization of soft tissue AVMs with curative intent is feasible. This technique may provide an alternative treatment option for achieving durable occlusion for complex soft tissue AVMs.

MRI evaluation of soft tissue vascular malformations.

Background:Soft tissue vascular malformations are not uncommonly encountered in clinical practice and are often mistaken for other pathologies. Vascular anomalies are divided into vascular tumors and malformations. Vascular malformations progressively increase in size with increasing age and are classified into low-flow (venous, lymphatic, veno-lymphatic, capillary and capillary venous) and high-flow malformations (arteriovenous malformations (AVM) and arteriovenous fistula (AVF)) depending on the presence or absence of arterial flow. Aim:Aim of this study is to evaluate the Magnetic resonance imaging (MRI) features of the soft tissue vascular malformations and to classify the lesions depending on flow pattern. Materials and Methods:A total of 52 patients of soft tissue vascular malformations were included in this study over a period of 3 years. All patients underwent detailed clinical examination and dynamic post contrast MRI evaluation. Doppler and ultrasound were done as an adjunct. Results:There were 31 females and 21 male patients with age ranging from 9 to 34 years. In total 33 venous, 11 lymphatic, 8 veno-lymphatic malformations were seen. No case of high-flow malformation was seen. Majority of the lesions involved the head and neck region and the extremities. Fat saturated T2WI, STIR, and 3D dynamic post contrast sequences were found to be the most useful MRI sequences. Conclusion:MRI is the modality of choice for evaluating the soft tissue vascular malformations. It depicts the extent of the lesion, classifies the lesions into low or high flow and helps in treatment planning.

Soft-tissue vascular malformations and tumors. Part 1: Classification, role of imaging and high-flow lesions

Vascular malformations and tumors, also known as “vascular anomalies”, comprise an extensive variety of lesions involving all parts of the body. Knowledge of their classification and imaging characteristics is paramount. Whereas hemangiomas are benign vascular tumors, characterized by cellular proliferation and hyperplasia; vascular malformations are not real tumors and characteristically exhibit normal endothelial turnover. Vascular malformations are classified according to the predominant vascular channel as arterial, capillary, venous, lymphatic, or mixed. Ultrasound and MRI are the main imaging modalities used in the diagnosis and classification of the vascular anomalies. In this series of two articles we review the classification of vascular anomalies, describe the role of imaging, summarize their distinctive histopathogenic, clinical and imaging features, and discuss the treatment options. On the first article we discuss the high-flow lesions, whereas the slow-flow lesions will be reviewed on the second. Complex syndromes with associated vascular tumors and malformations will be also presented.

Discrepancy between the clinical and histopathologic diagnosis of soft tissue vascular malformations

Soft tissue vascular malformations are generally diagnosed clinically, according to the International Society for the Study of Vascular Anomalies (ISSVA) classification. Diagnostic histopathologic examination is rarely performed. We sought to evaluate the validity of the current diagnostic workup without routinely performed diagnostic histopathology. We retrospectively determined whether there were discrepancies between clinical and histopathologic diagnoses of patients with clinically diagnosed vascular malformations undergoing therapeutic surgical resections in our center (2000-2015). Beforehand, a pathologist revised the histopathologic diagnoses according to the ISSVA classification. Clinical and histopathologic diagnoses were discrepant in 57% of 142 cases. In these cases, the pathologist indicated the diagnosis was not at all a vascular malformation (n=24; 17%), a completely different type of vascular malformation (n=26; 18%), or a partially different type with regard to the combination of vessel-types involved (n=31; 22%). Possible factors associated with the discrepancies were both clinician-related (eg, diagnostic uncertainty) and pathology-related (eg, lack of immunostaining). Retrospective analysis of a subgroup of patients undergoing surgery. The large discrepancy between clinical and histopathologic diagnoses raises doubt about the validity of the current diagnostic workup for vascular malformations. Clear clinical and histopathologic diagnostic criteria might be essential for a uniform diagnosis.

Immunohistochemical and histochemical characterization of intraosseous arteriovenous malformations of the jaws: analysis of 16 cases with emphasis on GLUT-1 immunophenotype

Intraosseous vascular lesions of the craniofacial region are rare and may cause diagnostic and therapeutic challenges. The purpose of this study was to characterize 16 cases of intraosseous arteriovenous malformations (AVMs) affecting the jaws. Immunohistochemical evaluation was performed using antibodies against α-smooth muscle actin (α-SMA), desmin, CD31, D2-40, and glucose transporter 1 (GLUT-1). Staining with elastic Verhoeff-Van Gieson and Masson trichrome histochemical stains was also performed. No gender predilection (female:male ratio=1:1) was observed, with patients' mean age being 50.8years (SD of ±13.9; range 28-71years). Predilection for the mandible was observed (12 of 16 [75%]). Immunohistochemically, diffuse endothelial CD31 staining was noted, and α-SMA strongly highlighted smooth muscle cells and pericytes. Desmin-positive vessels were identified in 9 of 16 AVMs (56.3%). D2-40 was uniformly negative in all specimens. AVMs were negative for GLUT-1 (11 of 14 [78.6%]) except for 2 cases (2 of 14 [14.3%]) exhibiting focal limited cytoplasmic GLUT-1 immunoreactivity. One case was equivocal for GLUT-1. Masson trichrome highlighted smooth muscle cells, and elastic fibers were identified in thick-walled arteries. AVMs of the jaws generally lack expression of GLUT-1, similar to soft tissue vascular malformations. Clinicoradiographic features of intraosseous AVMs in the present study were consistent with the findings of previous studies, although mean age was higher.

Síndrome De Klippel-Trenaunay-Weber: Estudo De 80 Casos Klippel-Trenaunay-Weber Syndrome: Study of 80 Cases

Brief Description of the Purpose of the StudyKlippel-Trenaunay-Weber Syndrome (KTWS) is a rare congenital disease. The clinical diagnostic criteria consists of the classic triad: capillary malformations in the skin, bone or soft tissue hypertrophy and vascular malformations. Complex vascular anomalies may occur (venous, arterial, lymphatic) as well as arteriovenous fistulas (AVFs), a frequent cause of pain. The purpose of this study is to report clinical and sonographic findings encountered in KTWS.MethodsBetween June 2009 and November 2012, 80 patients (ages ranging from 5 months to 45 years, 61% women) with a clinical diagnosis of KTWS were examined. Clinical evaluation and usual Ultrasound (US) Doppler techniques were used with an exam protocol developed for KTWS.Main ResultsMain clinical findings: port-wine skin patches(80%);lesions on lower limb(78%); on upper limb(18%); bilateral(10%), on all limbs(5%); increase in the length of the limb (50%); increase of limb volume (72%). Main sonographic findings: deep venous malformations (49%); lateral marginal vein (35%); anomalous arterial branches (96%); AVFs (88%); venous aneurisms (23%, among which 35% with AVFS); thrombosis (15%) and lymphatic cysts (19%).Importance of the ConclusionsWhen performed sistematically, the US-Doppler provides morphologic and hemodynamic evaluation of complex vascular malformations, especially of AVFs, indispensable in the treatment of KTWS. Brief Description of the Purpose of the StudyKlippel-Trenaunay-Weber Syndrome (KTWS) is a rare congenital disease. The clinical diagnostic criteria consists of the classic triad: capillary malformations in the skin, bone or soft tissue hypertrophy and vascular malformations. Complex vascular anomalies may occur (venous, arterial, lymphatic) as well as arteriovenous fistulas (AVFs), a frequent cause of pain. The purpose of this study is to report clinical and sonographic findings encountered in KTWS. Klippel-Trenaunay-Weber Syndrome (KTWS) is a rare congenital disease. The clinical diagnostic criteria consists of the classic triad: capillary malformations in the skin, bone or soft tissue hypertrophy and vascular malformations. Complex vascular anomalies may occur (venous, arterial, lymphatic) as well as arteriovenous fistulas (AVFs), a frequent cause of pain. The purpose of this study is to report clinical and sonographic findings encountered in KTWS. MethodsBetween June 2009 and November 2012, 80 patients (ages ranging from 5 months to 45 years, 61% women) with a clinical diagnosis of KTWS were examined. Clinical evaluation and usual Ultrasound (US) Doppler techniques were used with an exam protocol developed for KTWS. Between June 2009 and November 2012, 80 patients (ages ranging from 5 months to 45 years, 61% women) with a clinical diagnosis of KTWS were examined. Clinical evaluation and usual Ultrasound (US) Doppler techniques were used with an exam protocol developed for KTWS. Main ResultsMain clinical findings: port-wine skin patches(80%);lesions on lower limb(78%); on upper limb(18%); bilateral(10%), on all limbs(5%); increase in the length of the limb (50%); increase of limb volume (72%). Main sonographic findings: deep venous malformations (49%); lateral marginal vein (35%); anomalous arterial branches (96%); AVFs (88%); venous aneurisms (23%, among which 35% with AVFS); thrombosis (15%) and lymphatic cysts (19%). Main clinical findings: port-wine skin patches(80%);lesions on lower limb(78%); on upper limb(18%); bilateral(10%), on all limbs(5%); increase in the length of the limb (50%); increase of limb volume (72%). Main sonographic findings: deep venous malformations (49%); lateral marginal vein (35%); anomalous arterial branches (96%); AVFs (88%); venous aneurisms (23%, among which 35% with AVFS); thrombosis (15%) and lymphatic cysts (19%). Importance of the ConclusionsWhen performed sistematically, the US-Doppler provides morphologic and hemodynamic evaluation of complex vascular malformations, especially of AVFs, indispensable in the treatment of KTWS. When performed sistematically, the US-Doppler provides morphologic and hemodynamic evaluation of complex vascular malformations, especially of AVFs, indispensable in the treatment of KTWS.

566 Klippel Treunanay Syndrome in Differential Diagnosis of Cerebral Palsy

In cerebral palsy (CP) atrophy of the paretic body half results in disturbed growth. Disturbed growth is also a feature of a rare disorder; Klippel Treunanay syndrome (KTS). Here we...

MR Imaging of Soft-Tissue Vascular Malformations: Diagnosis, Classification, and Therapy Follow-up

Vascular malformations and tumors comprise a wide, heterogeneous spectrum of lesions that often represent a diagnostic and therapeutic challenge. Frequent use of an inaccurate nomenclature has led to considerable confusion. Since the treatment strategy depends on the type of vascular anomaly, correct diagnosis and classification are crucial. Magnetic resonance (MR) imaging is the most valuable modality for classification of vascular anomalies because it accurately demonstrates their extension and their anatomic relationship to adjacent structures. A comprehensive assessment of vascular anomalies requires functional analysis of the involved vessels. Dynamic time-resolved contrast material-enhanced MR angiography provides information about the hemodynamics of vascular anomalies and allows differentiation of high-flow and low-flow vascular malformations. Furthermore, MR imaging is useful in assessment of treatment success and establishment of a long-term management strategy. Radiologists should be familiar with the clinical and MR imaging features that aid in diagnosis of vascular anomalies and their proper classification. Furthermore, they should be familiar with MR imaging protocols optimized for evaluation of vascular anomalies and with their posttreatment appearances. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.315105213/-/DC1.

Ultrasound Diagnosis of Soft Tissue Vascular Malformations and Tumours

Soft tissue vascular malformations and tumours are a heterogeneous group of lesions. Some of them may only be transient, such as vascular birthmarks in newborns. In about one percent of children however a vascular soft tissue lesion needs further diagnostic work-up. Angiomateous lesions may even have a malignant potential and so an exact assessment is especially needed to define the prognosis of a lesion, as well as for planning of efficient treatment. Highresolution ultrasound (HR-US) together with Color Doppler ultrasound (CDUS) and spectral wave analysis (SWA) - at least as a first line modality - allow for an exact and reliable differentiation of such lesions in most cases. In addition HRUS is well tolerated and is suitable for follow-up assessment especially in children. Besides its diagnostic impact, HR-US is also well suited for guided interventional procedures. HR-US guided percutaneous sclerotherapy seems very promising in this context also with regard to other therapeutic options. In this review the diagnostic importance and value of HR-US are discussed in detail. Keywords: Soft tissue neoplasm, vascular malformation, –, ultrasound

Diagnostic Ultrasound in the Head and Neck Region

Improvements in ultrasound (US) resolution have led to the application of this non-ionizing imaging modality in the diagnosis of soft tissue disorders affecting the head and neck region. Although operator experience is paramount in image interpretation, US has the advantage of being inexpensive, safe and widely available. Ultrasound is the first line imaging modality for suspected salivary gland tumours and is useful in evaluating superficial neck swellings, such as lymph node disease, soft tissue cysts and vascular malformations. Ultrasound also improves the success rate when used for guidance during fine-needle aspiration cytology (FNAC). This paper aims to provide the general practitioner with an overview of the basic principles of image formation, the advantages and disadvantages of US, and an outline of its indications for conditions that might present to the dental practitioner.

Laser management for oral soft tissue haemangiomas and vascular malformations

Congenital vascular anomalies have been and remain poorly understood. Since 1982 haemangiomas and vascular malformations have been recognised as distinct entities that exhibit unique characteristics and demand appropriately tailored treatment plans.

Laser management for oral soft tissue haemangiomas and vascular malformations

Laser management for oral soft tissue haemangiomas and vascular malformations

Cosmetic treatments: An emerging field of interest for interventional radiologists

The current trend in medical care in the 21st century is evolving into a minimally invasive specialty. The interest of Interventional Radiology (IR) in cosmetic is increasing particularly for outsetting patients, in the treatment of soft tissue vascular malformations, us-guided injections of Botox ® and varicous veins management. Advantages of cosmetic IR treatments are many: treatments takes less than an hour and provides immediate relief of symptoms; no scaring, because the procedure does not require a surgical incision; an immediate return to normal activity with little or no pain; and high success rate and low recurrence rate compared to surgery.

Multiple Ipsilateral Lower Extremity Masses in a 46-year-old Man

Multiple Ipsilateral Lower Extremity Masses in a 46-year-old Man

Acute presentation of soft tissue vascular malformations: diagnosis with MR imaging

Acute presentation of soft tissue vascular malformations: diagnosis with MR imaging

Radiofrequency Ablation for Treatment of Symptomatic Low-flow Vascular Malformations after Previous Unsuccessful Therapy

Three patients with low-flow soft-tissue vascular malformations were treated with radiofrequency (RF) ablation. Other treatment options had proven unsuccessful. After RF ablation treatment, two patients were free of symptoms and one reported decreased symptoms followed by a return of symptoms within 1 year. No procedure-related complications occurred. It is suggested that RF ablation may be offered as an alternative treatment option for symptomatic vascular malformations when others have failed or are not possible.

MR and MR angiography characterization of soft tissue vascular malformations

Hemangioma is an abnormal proliferation of blood vessels that may occur in any vascularized tissue. Different classifications separate vascular lesions of soft tissues into hemangiomas and vascular malformations on the basis of their natural history, location, cellular turnover, and histology. Soft-tissue vascular malformations are relatively common. These lesions can be categorized on MR imaging because of their typical appearance as multiple lobules with fat overgrowth and serpentine channels, depending on the vascular flow. The combination of conventional MR and MR angiography (MRA) enable the differentiation between low-flow and high-flow vascular malformations and allows a noninvasive diagnostic strategy. This article reviews the MR and MRA imaging characteristics of soft-tissue hemangiomas to provide a helpful guide for radiologists to perform a more specific diagnosis and better management of these anomalies.