Superficial Vascular Malformations Research Articles

Severe Chronic Rectocolitis Associated With a Characteristic Sunflower Magnetic Resonance Imaging Pattern

Severe Chronic Rectocolitis Associated With a Characteristic Sunflower Magnetic Resonance Imaging Pattern

Treatment of superficial benign vascular tumors by high intensity focused ultrasound: Observations in two illustrative cases.

BackgroundExisting therapeutic methods for reduction or removal of superficial vascular malformations and tumors have high risks of scarring and other complications that result in aesthetic appearance less favorable than the baseline. Patients are often cautioned against intervention, which can lead to psychosocial problems and low self‐esteem. Improved treatment modalities are therefore relevant from both medical and aesthetic perspectives.MethodsTwo volunteer subjects were treated with a medical 20 MHz high intensity focused ultrasound device developed for dermatological conditions. One patient was given three treatments to remove a superficial congenital hemangioma on the left middle cheek. The other patient was given a single treatment to remove seven cherry angiomas on the thighs. Handpieces with nominal focal depths of 0.8 – 1.8 mm below the skin surface were used to administer acoustic energy of 1.1 – 1.2 J/dose. An integrated dermoscope in the handpiece was used to monitor the treatment in real‐time.ResultsDuring treatment, blood in the capillary network of the lesions was coagulated immediately, and capillary walls were collapsed due to the thermal and mechanical effects of the high intensity focused ultrasound. During the healing phase, the areas regenerated a normal skin structure with very limited scar or dyspigmentation. At follow‐up, a clear aesthetic improvement was observed over the baseline for all treated targets with the exception of two cherry angiomas, where focal depth and/or dose coverage had not been optimal.ConclusionHigh intensity focused ultrasound is concluded to be a safe and efficient skin treatment for benign superficial vascular malformations and tumors.

Health care transition for patients with vascular malformations: a French multicenter cross-sectional study

BackgroundHealth care transition (i.e., transition from pediatric to adult care) is challenging in chronic conditions but has been poorly studied in rare chronic skin diseases. We investigated the proportion of lost to follow-up among patients with superficial vascular malformations after health care transition. We also collected patients’ opinions. This prospective, multicenter, cross-sectional study was performed at 7 French hospitals. We included patients aged 19–25 years, who were followed for a superficial vascular malformation before age 16, and who had completed the transition period in 2020. Data were collected from medical records and a questionnaire was sent to included patients asking about the health care transition.ResultsAmong the 90 patients included, 41 (46%) were lost to follow-up after health care transition period. The age at diagnosis was significantly higher for lost to follow-up than non- lost to follow-up patients. The lost to follow-up proportion was similar between patients who changed and did not change hospitals during the transition. Responses to the questionnaire were obtained for 47 of 90 patients (52.2% response rate); most were satisfied with their care (n = 31/36, 86.1%); however, a lack of psychological support was reported.ConclusionsHealth care transition is associated to a high rate of lost to follow-up. Early management seems associated to less lost to follow-up. Further studies are needed to better understand risk factors for a failed health care transition and its consequences.

Angiomes superficiels : traitements

Beta-blockers are efficient for treating complicated infantile hemangiomas; propranolol is currently the first-line treatment. Superficial vascular malformations have to be managed by multidisciplinary teams. T2 FAT-SAT MRI is the most interesting sequence to explore superficial vascular malformations. Recent advances in molecular biology allow exploring new genetic mutations which could be involved in vascular malformations and be the target of new drugs. Mammalian target of rapamycin (mTOR) inhibitors are promising drugs for slow-flow malformations. Arteriovenous malformations are aggressive lesions with very few treatment options.

Treatment of voluminous and complicated superficial slow-flow vascular malformations with sirolimus (PERFORMUS): protocol for a multicenter phase 2 trial with a randomized observational-phase design

BackgroundSlow-flow superficial vascular malformations (VMs) are rare congenital anomalies that can be responsible for pain and functional impairment. Currently, we have no guidelines for their management, which can involve physical bandages, sclerotherapy, surgery, anti-inflammatory or anti-coagulation drugs or no treatment. The natural history is progressive and worsening. Mammalian target of rapamycin (mTOR) is a serine/threonine kinase that acts as a master switch in cell proliferation, apoptosis, metabolism and angio/lymphangiogenesis. Sirolimus directly inhibits the mTOR pathway, thereby inhibiting cell proliferation and angio/lymphangiogenesis. Case reports and series have reported successful use of sirolimus in children with different types of vascular anomalies, with heterogeneous outcomes.ObjectiveThe objective of this trial is to evaluate the efficacy and safety of sirolimus in children with complicated superficial slow-flow VMs.Methods/designThis French multicenter randomized observational-phase, phase 2 trial aims to include 50 pediatric patients 6 to 18 years old who have slow-flow (lymphatic, venous or lymphatico-venous) voluminous complicated superficial VM. Patients will be followed up for 12 months. All patients will start with an observational period (no treatment). Then at a time randomly selected between month 4 and month 8, they will switch to the experimental period (switch time), when they will receive sirolimus until month 12. Each child will undergo MRI 3 times: at baseline, at the switch time, and at month 12. For both periods (observational and treatment), we will calculate the relative change in volume of the VM divided by the study period duration. This relative change weighted by the study period duration will constitute the primary endpoint. VM will be measured by MRI images, which will be centralized and interpreted by the same radiologist who will be blinded to the study period. Hence, each patient will be his/her own control. Secondary outcomes will include assessment of safety and efficacy by viewing standardized digital photographs and according to the physician, the patient or proxy; impact on quality of life; and evolution of biological makers (coagulation factors, vascular endothelial growth factor, tissue factor).DiscussionThe main benefit of the study will be to resolve uncertainty concerning the efficacy of sirolimus in reducing the volume of VMs and limiting related complications and the safety of the drug in children with slow-flow VMs. This trial design is interesting in these rare conditions because all included patients will have the opportunity to receive the drug and the physician can maintain it after the end of the protocol if is found efficient (which would not be the case in a classical cross-over study).Trial registrationClinicalTrials.gov Identifier: NCT02509468, first received: 28 July 2015.EU Clinical Trials Register EudraCT Number: 2015-001096-43.

Clinical Characteristics and Treatment Options of Infantile Vascular Anomalies.

To analyze the clinical characteristics and treatment outcomes of vascular anomalies, and determine which therapy is safe and effective.The data of vascular anomalies pediatric patients who arrived at Beijing children's Hospital from January 2001 to December 2014 were analyzed retrospectively, including the influence of gender, age, clinical manifestation, diagnosis, treatment options, and outcomes. As to infantile hemangiomas, the outcomes of different treatments and their adverse reactions were compared. As to spider angioma and cutaneous capillary malformation, the treatment effect of 595 nm pulsed dye laser (PDL) is analyzed.A total number of 6459 cases of vascular anomalies were reclassified according to the 2014 ISSVA classification system. Among them, the gender ratio is 1:1.69, head-and-neck involved is 53.3%, the onset age within the first month is 72.4%, the age of initial encounter that younger than 6 months is 60.1%. The most common anomalies were infantile hemangiomas (42.6%), congenital hemangiomas (14.1%), and capillary malformations (29.9%). In treating infantile hemangiomas, laser shows the lowest adverse reactions rate significantly. Propranolol shows a higher improvement rate than laser, glucocorticoids, glucocorticoids plus laser, and shows no significant difference with propranolol plus laser both in improvement rate and adverse reactions rate. The total improvement rate of 595 nm PDL is 89.8% in treating spider angioma and 46.7% in treating cutaneous capillary malformation. The improvement rate and excellent rate of laser in treating cutaneous capillary malformation are growing synchronously by increasing the treatment times, and shows no significant difference among different parts of lesion that located in a body.Vascular anomalies possess a female predominance, and are mostly occurred in faces. Definite diagnosis is very important before treatment. In treating infantile hemangioma, propranolol is recommended as the first-line agent, and systemic use glucocorticoids should be considered when associated with serious complications. The 595 nm PDL is effective in managing superficial vascular malformations in childhood, and could attempt to increase the treatment times to improve the outcomes.

Large plaque-like glomangioma in a patient with multiple glomus tumours: review of imaging and histology

Glomus tumours are benign tumours of the temperature-sensitive neuromyoarterial glomus body, present within the dermis, which are most commonly seen as solitary subungual lesions on the arms. Multiple glomus tumours or glomangiomas are a less common subtype of extradigital glomus tumours, which very rarely present as large plaque-like dermal lesions. Glomangiomas do not often show the classic triad of symptoms associated with glomus tumours, namely: pain, point tenderness on compression, and temperature sensitivity. As a result of this and of their atypical morphology, they can often be misdiagnosed as vascular malformations (VMs), resulting in delayed diagnosis and inappropriate treatment. We report a 29-year-old man with multiple extradigital glomus tumours that had been present since childhood, with the lesion on the patient's leg being the largest plaque-like glomangioma yet reported, to our knowledge. Spectral greyscale and Doppler shift ultrasonography showed multiple, tubulonodular, ectatic, noncompressible, vascular structures with aberrant flow within the thickened dermis. Using magnetic resonance imaging, low to intermediate signal was seen on T1-weighted images and high signal on T2-weighted images, and there was florid enhancement with gadolinium, with no evidence of extension into muscle or bone. Histology showed abnormal, dilated, thin-walled, vascular channels lined with multiple layers of glomus cells, confirming the diagnosis of a glomangioma. We discuss imaging techniques for plaque-like glomangiomas, and review the clinical, radiological and histological characteristics that help differentiate them from other superficial VMs.

Two Cases of Hemihyperplasia–Multiple Lipomatosis Syndrome and Review of Asymmetric Hemihyperplasia Syndromes

Hemihyperplasia-multiple lipomatosis syndrome (HHML) is a condition characterized by asymmetric nonprogressive overgrowth, multiple lipomas, and superficial vascular malformations. We present two cases of HHML to enhance the diagnostic acumen of dermatologists and avoid potential misdiagnosis of this rare but probably underrecognized entity. We also provide a brief review of asymmetric overgrowth syndromes, which have overlapping yet distinct clinical manifestations.

Malformations vasculaires superficielles : impact sur le suivi de grossesse et prise en charge à l’accouchement

Superficial vascular malformations (MAV), so far called “superficial angioma”, are uncommon and often unknown. The last classification, done by the “International Society for the Study of Vascular Anomalies”, is essential to avoid diagnostic and therapeutic mistakes. Extramedullar localisations are rare. The coexistence of a pregnancy and a MAV states two problems: the medical and paraclinic supervision of the volume of the MAV and its risk of thrombosis with the choice of thrombosis prevention, and the mode of delivery and type of anaesthesia depending on its anatomical location. We revised the management of pregnant women with a MAV illustrating possible troubles to deal with. We reported the cases of two women having for the first one a pharyngolaryngeal MAV, the second a cutaneous MAV located on the leg and needing a multidisciplinary management with obstetricians, anaesthesists, hematologists, dermatologists, ENT, radiologists and pediatricians.

Échec de l’embolisation transartérielle par l’alcool absolu d’une fistule durale du sinus transverse

This is a case report of successful arterial embolization of a dural fistula using absolute alcohol. In this two-part embolization of a dural fistula of the left sagittal venous sinus, the use of n-BCA (n-butyl-cyanoacrylate) was followed by 1 ml of absolute alcohol four months later. The first procedure, using n-BCA via the external carotid, permitted exclusion of the arterial supply coming from the medial meningeal and occipital arteries. Persistence of the blood supply through the internal carotid prompted us to perform the second procedure, to occlude the tentorium marginalis artery, using 1 ml of 95% ethanol. This was followed by a cranial nerve (III) palsy that was reversible. The three-month follow-up was satisfactory, although arteriography after one year showed revascularization of the fistula, which was successfully treated by Onyx. Routinely used in the treatment of superficial vascular malformations, the use of absolute alcohol intracerebrally appears to be unreliable, with results that were only temporary.

Treatment of proliferative haemangiomas with the 585nm pulsed dye laser

Haemangiomas usually develop within the first few weeks of life, most regressing spontaneously before the age of 7 years. Some may ulcerate or compromise a vital function, in which case systemic corticosteroids, surgery or radiotherapy may be helpful. All of these treatment modalities are associated with significant morbidity. Treatment with the 585 nm flashlamp pulsed dye laser is safe and effective in the management of superficial vascular malformations. We report seven patients, under 12 months of age, who presented with proliferative haemangiomas, causing functional impairment. Ulcerated lesions were present in four patients. The patients were treated with the 585 nm pulsed dye laser (fluences 7.0-9.25 J/cm2), at intervals of 4-8 weeks. All of the lesions showed a significant reduction in size, together with improvement in the colour and integrity of the overlying skin. Treatment with the 585 nm pulsed dye laser should be considered in the management of infants with early proliferative haemangiomas, in whom intervention is indicated.

Pulsed color laser for treatment of benign, superficial vascular malformations

The flash lamp-excited dye laser is ideal for treating benign superficial vascular cutaneous lesions. Port-wine stains, telangiectases, spider angiomas, or hemangiomas may be treated without pain. If used properly, complications such as skin texture changes, hypopigmentation, and scarring are extremely rare. The flash lamp-excited dye laser works on the principle of selective photothermolysis, which allows selective damage to the target vessel without affecting surrounding tissue. Lesions like xanthelasma palpebrarum, keloid scars, and warts may also be treated by the flash lamp-excited dye laser. This study presents the results of treatment with this laser in our patients.