scholarly journals Sirolimus for the treatment of vascular anomalies in children

2020 ◽  
Vol 7 (3) ◽  
pp. 22-31
Author(s):  
E. K. Donyush ◽  
Z. A. Kondrashova ◽  
Yu. A. Polyaev ◽  
R. V. Garbuzov
Keyword(s):  
Vascular Malformations ◽  
Venous Malformation ◽  
Antiangiogenic Therapy ◽  
Pneumocystis Pneumonia ◽  
Response To Therapy ◽  
Vascular Anomalies ◽  
Vascular Tumors ◽  
Treatment Regimens ◽  
Transplant Patients ◽  
Results Of Treatment

Vascular anomalies (VA) comprise a heterogeneous group of diseases associated with congenital angiogenesis disorder. There are no currently developed unified protocols and treatment regimens for systemic forms of VA. Numerous advantages show sirolimus, an mTOR inhibitor, as a well tolerated and effective antiproliferative and antiangiogenic therapy in patients with VA. The article presents the results of treatment of 211 patients with VA (6 patients with vascular tumors and 205 patients with vascular malformations) aged 2 months to 17 years (median – 9 years), who received sirolimus therapy for 1–86 months (median – 24 months). Sirolimus was administered at a starting dose of 0.8 mg/m2/day orally in two doses with an interval of 12 hours. The concentration of the blood preparation was maintained in the therapeutic range of 6–15 ng/ml. Since 2015, concomitant therapy with co-trimoxazole for the prevention of Pneumocystis pneumonia has been prescribed only to tracheostomy carriers. When infectious episodes occurred in patients, sirolimus therapy continued without changes in the dose of the drug and did not affect the disease, provided that the therapeutic concentration was maintained. A positive response to therapy was observed in 89.1 % of patients with VA in the form of the size of the vascular mass according to the data of visual examination and instrumental control. All patients showeda clinical response to therapy in the form of relief of painsyndrome, reduction/relief of lymphorrhea, reduction/improvement of hemostasis parameters, and an increase in functional activity and quality. For the entire observation period 2012–2020, when taking sirolimus, not a single severe adverse event occurring in post-transplant patients has been reported that would require discontinuation of the drug. The article presents two clinical cases of sirolimus use in the treatment of patients with kaposiform hemangioendothelioma and extensive venous malformation. Parents are encouraged to use the information in scientific research and publications.

scholarly journals Cavernous Hemangioma of the Tongue

2015 ◽  
Vol 6 (3) ◽  
pp. 109-111 ◽  
Author(s):  
Jatin P Shah ◽  
Ivana Petrovic ◽  
Ben Roman
Keyword(s):  
Cavernous Hemangioma ◽  
Soft Tissues ◽  
Vascular Malformations ◽  
Surgical Excision ◽  
Vascular Anomalies ◽  
Preoperative Imaging ◽  
Vascular Tumors ◽  
Adult Onset ◽  
Complete Excision ◽  
Intramuscular Hemangioma

ABSTRACT Background Vascular anomalies are divided into vascular tumors, hemangiomas being the most common, and vascular malformations. Most vascular anomalies are noticed at birth or occur during infancy, and generally involve skin or subcutaneous soft tissues. Adult onset hemangiomas are rare, and intramuscular location is extremely rare. Surgical excision is recommended for hemangiomas in adults, if they are symptomatic, or manifest growth. Materials and methods We report a rare case of a 51-year-old woman, with an intramuscular hemangioma of the tongue, presenting as a submental mass. Preoperative imaging for assessment of tumor extent was followed by a successful surgical excision. Results Postoperative course was uneventful with primary healing of the wound, and with no functional deficit of tongue function. Conclusion Although a variety of treatment approaches are reported for childhood hemangiomas, surgical excision is the preferred treatment for adult onset symptomatic hemangiomas. Preoperative work up should include imaging preferably with contrast enhanced magnetic resonance imaging (MRI). Embolization may be considered for larger lesions. Intraoperative hypotension should be avoided to ensure identification of the entire lesion to ensure complete excision. How to cite this article Petrovic I, Roman B, Shah JP. Cavernous Hemangioma of the Tongue. Int J Head Neck Surg 2015;6(3):109-111.

scholarly journals Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies

2018 ◽  
Vol 190 (09) ◽  
pp. 825-835 ◽  
Author(s):  
Maliha Sadick ◽  
René Müller-Wille ◽  
Moritz Wildgruber ◽  
Walter Wohlgemuth
Keyword(s):  
Classification System ◽  
Vascular Malformations ◽  
Color Doppler ◽  
Vascular Anomalies ◽  
Endothelial Cell Proliferation ◽  
Vascular Tumors ◽  
Interdisciplinary Management ◽  
Venous Malformations ◽  
Medical Evaluation ◽  
Malformation Syndromes

Background Vascular anomalies are a diagnostic and therapeutic challenge. They require dedicated interdisciplinary management. Optimal patient care relies on integral medical evaluation and a classification system established by experts in the field, to provide a better understanding of these complex vascular entities. Method A dedicated classification system according to the International Society for the Study of Vascular Anomalies (ISSVA) and the German Interdisciplinary Society of Vascular Anomalies (DiGGefA) is presented. The vast spectrum of diagnostic modalities, ranging from ultrasound with color Doppler, conventional X-ray, CT with 4 D imaging and MRI as well as catheter angiography for appropriate assessment is discussed. Results Congenital vascular anomalies are comprised of vascular tumors, based on endothelial cell proliferation and vascular malformations with underlying mesenchymal and angiogenetic disorder. Vascular tumors tend to regress with patient’s age, vascular malformations increase in size and are subdivided into capillary, venous, lymphatic, arterio-venous and combined malformations, depending on their dominant vasculature. According to their appearance, venous malformations are the most common representative of vascular anomalies (70 %), followed by lymphatic malformations (12 %), arterio-venous malformations (8 %), combined malformation syndromes (6 %) and capillary malformations (4 %). Conclusion The aim is to provide an overview of the current classification system and diagnostic characterization of vascular anomalies in order to facilitate interdisciplinary management of vascular anomalies. Key Points  Citation Format

Vascular Anomalies

2019 ◽  
pp. 241-260
Author(s):  
Lara Wine Lee ◽  
Cameron C. Trenor
Keyword(s):  
Functional Impairment ◽  
Vascular Tissue ◽  
Vascular Malformations ◽  
Diagnostic Procedures ◽  
Vascular Anomalies ◽  
Surgical Approaches ◽  
Vascular Tumors ◽  
Vascular Abnormalities ◽  
The Aesthetic ◽  
Excessive Proliferation

The chapter provides a description of the main anomalies characterized by excessive proliferation of the vascular tissue and the major syndromes associated with them. The predominant types of vascular anomalies are vascular tumors and vascular malformations. Vascular tumors are further subdivided in benign, locally aggressive, and malignant, according to the clinical course of the lesions, while vascular abnormalities are classified as simple (capillary, lymphatic, venous, arteriovenous, and arteriovenous fistula) or combined. For each subgroup of vascular anomalies, clinical presentation and diagnostic procedures are discussed, as well as pathogenic mechanisms and potential comorbidities. The management of vascular anomalies may be oriented to treat the aesthetic discomfort as well as the functional impairment and may involve both pharmaceutical and surgical approaches.

scholarly journals Peripheral Vascular Anomalies – Essentials in Periinterventional Imaging

2019 ◽  
Vol 192 (02) ◽  
pp. 150-162 ◽  
Author(s):  
Maliha Sadick ◽  
Daniel Overhoff ◽  
Bettina Baessler ◽  
Naema von Spangenberg ◽  
Lena Krebs ◽  
...  
Keyword(s):  
Therapeutic Approach ◽  
Vascular Malformations ◽  
Vascular Anomaly ◽  
Flow Dynamics ◽  
Vascular Anomalies ◽  
Vascular Tumors ◽  
Slow Flow ◽  
Peripheral Vascular ◽  
Fast Flow ◽  
Selection Of

Background Peripheral vascular anomalies represent a rare disease with an underlying congenital mesenchymal and angiogenetic disorder. Vascular anomalies are subdivided into vascular tumors and vascular malformations. Both entities include characteristic features and flow dynamics. Symptoms can occur in infancy and adulthood. Vascular anomalies may be accompanied by characteristic clinical findings which facilitate disease classification. The role of periinterventional imaging is to confirm the clinically suspected diagnosis, taking into account the extent and location of the vascular anomaly for the purpose of treatment planning. Method In accordance with the International Society for the Study of Vascular Anomalies (ISSVA), vascular anomalies are mainly categorized as slow-flow and fast-flow lesions. Based on the diagnosis and flow dynamics of the vascular anomaly, the recommended periinterventional imaging is described, ranging from ultrasonography and plain radiography to dedicated ultrafast CT and MRI protocols, percutaneous phlebography and transcatheter angiography. Each vascular anomaly requires dedicated imaging. Differentiation between slow-flow and fast-flow vascular anomalies facilitates selection of the appropriate imaging modality or a combination of diagnostic tools. Results Slow-flow congenital vascular anomalies mainly include venous and lymphatic or combined malformations. Ultrasound and MRI and especially MR-venography are essential for periinterventional imaging. Arteriovenous malformations are fast-flow vascular anomalies. They should be imaged with dedicated MR protocols, especially when extensive. CT with 4D perfusion imaging as well as time-resolved 3D MR-A allow multiplanar perfusion-based assessment of the multiple arterial inflow and venous drainage vessels of arterio-venous malformations. These imaging tools should be subject to intervention planning, as they can reduce procedure time significantly. Fast-flow vascular tumors like hemangiomas should be worked up with ultrasound, including color-coded duplex sonography, MRI and transcatheter angiography in case of a therapeutic approach. In combined malformation syndromes, radiological imaging has to be adapted according to the dominant underlying vessels and their flow dynamics. Conclusion Guide to evaluation of flow dynamics in peripheral vascular anomalies, involving vascular malformations and vascular tumors with the intention to facilitate selection of periinterventional imaging modalities and diagnostic and therapeutic approach to vascular anomalies. Key Points:  Citation Format

HEAD AND NECK VASCULAR ANOMALIES OUR CLINICAL EXPERIENCE AND LITERATURE REVIEW.

2020 ◽  
Vol VOLUME 8 (ISSUE 1) ◽  
pp. 1-8
Author(s):  
Arulalan Mathialagan
Keyword(s):  
Literature Review ◽  
Head And Neck ◽  
Venous Malformation ◽  
Neck Region ◽  
Vascular Anomaly ◽  
Vascular Anomalies ◽  
Active Management ◽  
Vascular Tumors ◽  
Tertiary Referral Centre ◽  
Prompt Diagnosis

Abstract Background- Head and neck vascular anomalies are a spectrum of lesion ranging from simple asymptomatic malformations to life-threatening airway vascular tumors. Management of these lesion poses a real challenge to even most experienced otolaryngologists.Systematic evaluation and prompt diagnosis and judicial choice of treatment are essential for proper management of these lesions. Material and methods-A retrospective review of the head and neck vascular anomalies over three years, from 2017 to 2019 in a tertiary referral centre. Clinical features, radiology, treatment and follow-up data were studied.We did a literature review to give a comprehensive analysis regarding the diagnosis and treatment of the vascular anomalies of the head and neck region. Results- A total of 25 patients were managed in our department in two years. Of these 25 patients, three patients had infantile haemangioma(12%), six patient had congenital haemangioma(24%), six patients had lymphatic malformation(24%), four patient had an arteriovenous malformation (16%), and six patient had venous malformation (24%). Sixpatients (24%) underwent surgery as treatment, which includes one case of noninvoluting congenital hemangioma (NICH) of the temporal bone, one facial AVM, twolateral neck lymphangiomas, one skull base lymphangioma and a venous neck malformation. All the infantile h a ema n gi oma s we rema n a g e d w i t h o ra l propranolol, three lymphatic malformations underwent sclerotherapy, while the three Arteriovenous malformations (AVM), 􀃶ve congenital haemangiomas and four Venous malformations (VM) were under observation. Conclusion-Managing a vascular anomaly requires a prompt diagnosis based on the ISSVA classi􀃶cation, patients' symptoms and the location of the lesion. Not all vascular anomaly requires active management. Careful case selection and a multidisciplinary team are essential for adequate management of the vascular anomalies. Key Words: Vascular Malformation, Hemangioma, Lymphangioma

Intraoral Venous Malformation-A Case report

2021 ◽  
pp. 80-82
Author(s):  
B.V Subhash ◽  
Alba Dinesh ◽  
Seema Patil ◽  
Asha R Iyengar ◽  
Revan Kumar Joshi ◽  
...  
Keyword(s):  
Case Report ◽  
Oral Cavity ◽  
Vascular Malformations ◽  
Venous Malformation ◽  
Vascular Anomalies ◽  
Venous Malformations ◽  
Wide Spread ◽  
Facial Area ◽  
Ct And Mri

The vascular anomalies consist of vascular tumours and vascular malformations. One among these are the venous malformations which constitute about 40% of all vascular malformations. Usually, the venous malformations present as localized lesions in the form of swellings/nodules with phleboliths, are diagnosed by ultrasonography, CT and MRI. Summary: This is a report of a rare longstanding case of wide spread venous malformations of oral cavity and facial area with phleboliths.

scholarly journals Glut 1, S-100, and Nerve Bundle Study in Vascular Anomalies

2021 ◽  
Author(s):  
Ajay K. Khanna ◽  
Akhilesh Kumar ◽  
Soumya Khanna ◽  
Amrita Kar ◽  
Puneet Kumar ◽  
...  
Keyword(s):  
Vascular Malformation ◽  
Vascular Malformations ◽  
Vascular Anomalies ◽  
Vascular Lesions ◽  
Histological Evaluation ◽  
Vascular Tumors ◽  
Nerve Bundle ◽  
Glut 1 ◽  
S 100 ◽  
Hematoxylin And Eosin

Abstract Vascular anomalies grouped into vascular tumors (hemangioma) (HI) and vascular malformation (VM) are benign vascular lesions that are difficult to distinguish from one another clinically and often confused with each other at histopathology. This confusing terminology leads to improper diagnosis, illogical treatment, and misdirected research. This study aimed to study GLUT 1, S-100, and nerve bundle to differentiate hemangioma and vascular malformation. Thirty two cases of vascular lesions (26 vascular malformations and 6 hemangiomas) were taken into the study. For histological evaluation and immunohistochemistry (IHC), samples of vascular lesions were collected in formalin. All the hematoxylin and eosin-stained slides were evaluated under light microscope for histology and nerve bundles. Immunohistochemical staining was performed by streptavidin–biotin method for GLUT 1 and S-100. GLUT 1 was positive in all 6 cases of hemangiomas (100%) and only in 1 case of vascular malformation. Nerve bundle was present in 24 cases of vascular malformation (92.3%) out of 26 cases but not in any cases of hemangioma and S-100 was found positive in all vascular malformation cases (100%) but not in hemangioma. So GLUT 1 expression, S-100, and presence of nerve bundle in vascular lesions can help to differentiate hemangioma and vascular malformation.

scholarly journals A Step-by-step Sonographic Approach to Vascular Anomalies in the Pediatric Population: A Pictorial Essay

2021 ◽  
Author(s):  
Anushka Mittal ◽  
Rama Anand ◽  
Richa Gauba ◽  
Subhasis Roy Choudhury ◽  
Pooja Abbey
Keyword(s):  
Vascular Malformations ◽  
Pediatric Population ◽  
Vascular Supply ◽  
Clinical Findings ◽  
Vascular Anomalies ◽  
Doppler Imaging ◽  
Low Flow ◽  
Vascular Tumors ◽  
Pictorial Essay ◽  
Soft Tissue Masses

AbstractVascular anomalies are a common cause of soft-tissue masses in children and often referred for ultrasonographic (USG) evaluation. They are broadly classified as vascular tumors (hemangiomas, hemangioendotheliomas, and angiosarcomas) or vascular malformations (venous malformations, lymphatic malformations, and arteriovenous malformations). Findings on USG and Doppler imaging can be used to categorize vascular anomalies into high- or low-flow lesions, which forms the basis for further workup, diagnosis, and management. On careful evaluation of various sonographic features, in conjunction with clinical findings, an accurate clinicoradiological diagnosis can be made in most cases. Further imaging with magnetic resonance (MR) imaging or computed tomography (CT) helps in delineation of lesion extent, whereas MR or CT angiography is useful to map the vascular supply of high-flow lesions. We have illustrated and discussed a step-by-step approach to diagnose vascular anomalies using ultrasound and Doppler imaging.

scholarly journals Vascular Birthmarks as a Clue for Complex and Syndromic Vascular Anomalies

2021 ◽  
Vol 9 ◽  
Author(s):  
Andrea Diociaiuti ◽  
Guglielmo Paolantonio ◽  
Mario Zama ◽  
Rita Alaggio ◽  
Claudia Carnevale ◽  
...  
Keyword(s):  
Vascular Malformation ◽  
Early Recognition ◽  
Vascular Malformations ◽  
Clinical Signs ◽  
Vascular Anomalies ◽  
Vascular Lesions ◽  
Vascular Tumors ◽  
Capillary Malformation ◽  
Wide Range ◽  
Vascular Birthmarks

Vascular birthmarks are common in neonates (prevalence: 20–30%) and mostly incidental findings sometimes with spontaneous regression (salmon patch and nevus simplex). Capillary malformations are found in about 1% and infantile hemangiomas are found in 4% of mature newborns. Vascular malformations are classified according to their most prominent vessel type. The term “capillary malformation” (port wine stain) includes a wide range of vascular lesions with different characteristics; they may be isolated or part of specific syndromic conditions. Part of the infantile hemangiomas and of the vascular malformations may require treatment for functional or cosmetic reasons, and in rare cases, investigations are also necessary as they represent a clue for the diagnosis of complex vascular malformation or tumors associated with extracutaneous abnormalities. Complex vascular malformations are mostly mosaicism due to early somatic mutations. Genetic advances have led to identify the main pathogenic pathways involved in this disease group. Diffuse capillary malformation with overgrowth, Klippel–Trenaunay syndrome, CLAPO syndrome, CLOVES syndrome, and megalencephaly-capillary malformation belong to the PIK3CA-related overgrowth. Capillary malformation–arteriovenous malformation underlies a fast-flow vascular malformation, sometimes manifesting as Parkes–Weber syndrome. Recognition of these different types of capillary vascular stains is sometimes difficult; however, associated findings may orient the clinicians while genetic testing may confirm the diagnosis. Lymphatic malformation frequently manifests as large masses that compress and/or infiltrate the surrounding tissues, representing a neonatal emergency when airways are involved. Infantile hemangiomas may cause functional and/or permanent esthetical damage, depending on their localization (such as periorbital area, lip, nose); large (more than 5 cm) infantile hemangiomas with a segmental distribution can be associated with obstruction or malformations of the underneath organs with complications: PHACE syndrome, LUMBAR/SACRAL syndrome, and beard infantile hemangioma. In our review, we discuss controversies regarding the international classification and emerging concepts in the field of vascular anomalies. Finally, we discuss potential developments of new, non-invasive diagnostic techniques and repurposing of target therapies from oncology. Complex and/or life-threatening vascular tumors and malformations are extremely rare events and they represent a considerable therapeutic challenge. Early recognition of clinical signs suggestive for a specific disease may improve therapeutic outcomes and avoid severe complications.

scholarly journals Application of the adenosine triphosphate sensitivity assay in infantile vascular anomalies

2019 ◽  
Author(s):  
Li Li ◽  
Bin Yang ◽  
Li Wei ◽  
Bin Zhang ◽  
Xiao-feng Han ◽  
...  
Keyword(s):  
Adenosine Triphosphate ◽  
Comprehensive Evaluation ◽  
Vascular Malformations ◽  
Inhibitory Effect ◽  
Vascular Anomalies ◽  
Vascular Tumors ◽  
Inhibitory Effects ◽  
Inhibition Effect ◽  
Multiple Drugs

Abstract BACKGROUND: The term vascular anomalies include various vascular tumors and vascular malformations, among them infantile hemangiomas and capillary malformations are the most well-known associated diseases in early ages. Multiple drugs have been introduced for intervention, but susceptibility test in vitro were scarcely reported.OBJECTIVE: To evaluate the inhibition effect of adenosine triphosphate sensitivity assay in vitro before the treatment of infantile hemangiomas and capillary malformations. METHODS: Specimens were selected from 5 cases of infantile hemangiomas and 11 cases of capillary malformations. Propranolol, rapamycin, sildenafil and itraconazole were tested for their growth inhibition effect by using the adenosine triphosphate sensitivity assay. RESULTS: Propranolol demonstrated inhibitory effects on infantile hemangiomas cells. Rapamycin and itraconazole both showed inhibitory effects on infantile hemangiomas cells and capillary malformations cells. Sildenafil has no growth inhibitory effect on infantile hemangiomas cells or capillary malformations cells. CONCLUSION: Adenosine triphosphate sensitivity assay is a sensitive and useful testing method before the management of vascular anomalies, and individualized medication suggestions for the choice of therapeutic drugs were offered based on the testing result and together with a comprehensive evaluation of each infant.

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