Neck Vascular Anomalies Research Articles

Molecular pathways and possible therapies for head and neck vascular anomalies.

Vascular anomalies are a heterogenous group of vascular lesions that can be divided, according to the International Society for the Study of Vascular Anomalies Classification, into two main groups: vascular tumors and vascular malformations. Vascular malformations can be further subdivided into slow-flow and fast-flow malformations. This clinical and radiological classification allows for a better understanding of vascular anomalies and aims to offer a more precise final diagnosis. Correct diagnosis is essential to propose the best treatment, which traditionally consists of surgery, embolization, or sclerotherapy. Since a few years, medical treatment has become an important part of multidisciplinary treatment. Genetic and molecular knowledge of vascular anomalies are increasing rapidly and opens the door for a molecular classification of vascular anomalies according to the underlying pathways involved. The main pathways seem to be PI3K/AKT/mTOR and RAS/RAF/MEK/ERK. Knowing the underlying molecular cascades allows us to use targeted medical therapies. The first part of this article aims to review the vascular anomalies seen in the head and neck region and their underlying molecular causes and involved pathways. The second part will propose an overview of the available targeted therapies based on the affected molecular cascade. This article summarizes theragnostic treatments available in vascular anomalies.

Vascular anomalies of the head and neck: diagnosis and treatment.

Vascular malformations and vascular tumors comprise the two specific subsets of vascular anomalies that arise as a result of disorganized angiogenesis and neoplasm, respectively. Malformations are separate entities from vascular tumors (e.g., hemangiomas) and are recognized by the International Society for the Study of Vascular Anomalies (ISSVA) as such. Vascular malformations are classified into four main groups: simple, combined, anomalies of major vessels, and those associated with other vascular anomalies. Vascular tumors are neoplastic growths of blood vessels and are morphologically and molecularly distinct from malformations but can arise in the head and neck and have syndromic association. Head and neck vascular anomalies are not uncommon in the pediatric population and require special care in the workup, diagnostic imaging and clinical care. The purpose of this manuscript is to discuss the diagnosis and management of the most common intracranial and extracranial vascular malformations and tumors in the head and neck in children and adolescents.

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

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

Evaluation of digital image analysis as a supportive tool for the stratification of head and neck vascular anomalies

BackgroundThe histological differentiation of individual types of vascular anomalies (VA), such as lymphatic malformations (LM), hemangioma (Hem), paraganglioma (PG), venous malformations (VeM), arteriovenous malformations (AVM), pyogenic granulomas (GP), and (not otherwise classified) vascular malformations (VM n.o.c.) is frequently difficult due to the heterogeneity of these anomalies. The aim of the study was to evaluate digital image analysis as a method for VA stratificationMethodsA total of 40 VA tissues were examined immunohistologically using a selection of five vascular endothelial-associated markers (CD31, CD34, CLDN5, PDPN, VIM). The staining results were documented microscopically followed by digital image analyses based quantification of the candidate-marker-proteins using the open source program ImageJ/Fiji.ResultsDifferences in the expression patterns of the candidate proteins could be detected particularly when deploying the quotient of the quantified immunohistochemical signal values. Deploying signal marker quotients, LM could be fully distinguished from all other tested tissue types. GP achieved stratification from LM, Hem, VM, PG and AVM tissues, whereas Hem, PG, VM and AVM exhibited significantly different signal marker quotients compared with LM and GP tissues.ConclusionAlthough stratification of different VA from each other was only achieved in part with the markers used, the results of this study strongly support the usefulness of digital image analysis for the stratification of VA. Against the background of upcoming new diagnostic techniques involving artificial intelligence and deep (machine) learning, our data serve as a paradigm of how digital evaluation methods can be deployed to support diagnostic decision making in the field of VAs.

Virome Analysis Reveals No Association of Head and Neck Vascular Anomalies with an Active Viral Infection.

Vascular anomalies encompass different vascular malformations [arteriovenous (AVM), lymphatic (LM), venous lymphatic (VLM), venous (VM)] and vascular tumors such as hemangiomas (HA). The pathogenesis of vascular anomalies is still poorly understood. Viral infection was speculated as a possible underlying cause. A total of 13 human vascular anomalies and three human skin control tissues were used for viral analysis. RNA derived from AVM (n=4) and normal skin control (n=3) tissues was evaluated by RNA sequencing. The Virome Capture Sequencing Platform for Vertebrate Viruses (VirCapSeq-VERT) was deployed on 10 tissues with vascular anomalies (2×AVM, 1×HA, 1×LM, 2×VLM, 4×VM). RNA sequencing did not show any correlation of AVM with viral infection. By deploying VirCapSeq-VERT, no consistent viral association was seen in the tested tissues. The analysis does not point to the presence of an active viral infection in vascular anomalies. However, transient earlier viral infections, e.g. during pregnancy, cannot be excluded with this approach.

Magnetic resonance imaging of head and neck vascular anomalies

Purpose: The aim of this study was to describe typical MRI features of the head and neck vascular anomalies and the possible diagnostic pitfalls. Patients and methods: Patients with extracranial vascular anomalies of the head and neck, who underwent MRI examinations between January 2013 and January 2016, were included in the study. Precontrast and postcontrast T1-WI,T2-WI, with and without fat saturation were acquired. When indicated, a noncontrast MR angiography was performed. Dynamic postcontrast MRI techniques were available in six children. Results: The study included 33 patients (age ranged from 10 to 20 years, mean: 49 months). MRI confirmed the clinical diagnosis in equivocal cases, and provided proper determination of lesion extension and/or associated intracranial anomalies. The study included 10 cases of vascular tumors (hemangioma), whereas the remaining 23 cases had the diagnosis of vascular malformations (one patient with arteriovenous malformation, one with capillary malformation, seven with venous, nine with macrocystic lymphatic, and five with microcystic lymphatic malformations). Conclusion: Vascular anomalies in the head and neck are mostly diagnosed on clinical basis; however, when the history is uncertain or the diagnosis is equivocal, a well-tailored MR examination can be a single valuable diagnostic tool providing structural and functional information. Keywords: children, head and neck, hemangioma, lymphatic malformation, magnetic resonance angiography, venous malformation

Interdisciplinary Management of Head and Neck Vascular Anomalies: Clinical Presentation, Diagnostic Findings and Minimalinvasive Therapies

ObjectivesVascular anomalies are included in the 30 000 rare diseases worldwide affecting less than 5/10 000 people. Depending on their morphology and biological properties, they can cause varied disorders with organ involvement. Almost 60% of vascular anomalies have a predilection for the head and neck region in children. Clinical and scientific effort to establish interdisciplinary management concepts for vascular anomalies is increasing worldwide. MethodsEspecially in the head and neck region, clinical impairment and organ dysfunction is associated with cosmetic issues that may represent a physical and psychological issue for the patient. Correct diagnosis, based on clinical presentation and symptoms, is a prerequisite for appropriate therapy, ranging from conservative management to a spectrum of minimally invasive treatment options. We searched PubMed for German and English language published data until December 2016 with focus on clinical studies, review articles and case reports on vascular anomalies with a focus on the head and neck region. ResultsThe last ISSVA update in 2014 has contributed to a better understanding of vascular anomalies, classifying them in vascular tumors and vascular malformations. The predominant representatives of vascular tumors are congenital and infantile hemangiomas. Infantile hemangiomas have the ability of spontaneous regression in more than 80%. Patients with symptomatic growing hemangiomas with ulcerations, bleeding complications and restriction of hearing, swallowing disorder, impairment of vision, or cosmetic dysfigurement require treatment. Therapies include oral propanolol, transcatheter embolization and surgery. Vascular malformations tend to progress with patientś age and are subdivided in slow flow and fast flow lesions. Symptomatic slow flow lesions, e.g. venous and lymphatic malformations, benefit from percutaneous sclerotherapy. Fast flow lesions, as arteriovenous malformations, are rare but undoubtedly therapeutically the most challenging vascular anomaly. Depending on location and size, they may require multiple transcatheter embolization procedures for successful occlusion of the AVM. ConclusionsThis review provides knowledge on the current ISSVA classification of vascular anomalies, their clinical presentation, diagnostic evaluation and minimally invasive therapy options to encourage the establishment of a comprehensive interdisciplinary management for head and neck vascular anomalies.

Book Review: Review of Head and Neck Vascular Anomalies: A Practical Case-Based Approach

Book Review: Review of <i>Head and Neck Vascular Anomalies: A Practical Case-Based Approach</i>

Management of Low Flow Vascular Malformations of the head and neck: the Birmingham experience

Introduction: Vascular malformations comprise a wide, heterogeneous spectrum of lesions and patients may suffer from significant aesthetic and functional impairment, representing a diagnostic and therapeutic challenge to the clinician. The adult Head and Neck Vascular Anomalies service was established within the Regional Maxillofacial Unit at the Queen Elizabeth Hospital, Birmingham in 2003. This team established a procedure pathway for all patients undergoing percutaneous sclerotherapy with the detergent sclerosing agent sodium tetradeycl sulphate (STS) 3%.

Head and Neck Vascular Anomalies in the Pediatric Population

Head and Neck Vascular Anomalies in the Pediatric Population

Surgical management of head and neck vascular anomalies in children: a retrospective analysis of 42 patients

This article aimed to present a series of surgically treated head and neck vascular anomalies during a 12-year period, highlighting the epidemiology, diagnostic approach, indications for surgery, and final clinical outcome. The medical records of all patients with head and neck vascular anomalies, surgically treated at our department from 1998 to 2010, were reviewed retrospectively. A total of 42 patients with 46 vascular anomalies were identified. Patients' diagnoses included vascular tumors, hemangiomas mainly (18 cases), and various vascular malformations (26 cases). All patients were submitted to surgical treatment (excision-resection) to resolve main clinical symptoms (ulceration, bleeding, impaired mastication and feeding, airway obstruction) and/or esthetic issues. Recurrence was noted in 3 patients. Accurate differential diagnosis based on history, physical examination, and imaging, is the key to optimal treatment. Surgical intervention is warranted for small to moderately extended lesions to avoid complications and/or esthetic concerns and psychosocial distress.

The Birmingham algorithm for the diagnosis and management of vascular malformations

Introduction: The adult Head and Neck Vascular Anomalies Clinic was established in 2003 within the Maxillofacial Unit at the University Hospital Birmingham. The service was set up to satisfy hitherto unmet treatment needs in a group of patients with significant facial deformity. Currently, it receives referrals from all over the United Kingdom and beyond. The multidisciplinary team includes maxillofacial surgeons, a plastic and craniofacial surgeon and interventional radiologists.

Vascular Lesions: GLUT-1 Expression as a Diagnostic Tool to Discriminate Tumors From Malformations

Vascular lesions constitute a widely heterogeneous group of tumors and malformations. For head and neck vascular anomalies, most studies have not attempted to make the differential diagnosis between true hemangiomas and vascular malformations, because an accurate diagnosis remains a challenge for physicians. The successful treatment of vascular anomalies depends on a profound knowledge of their biologic behavior and correct classification. Recently, specific immunohistochemical markers such as erythrocyte-type glucose transporter protein 1 have been described to differentiate hemangiomas from vascular malformations. This report describes 2 cases of intramuscular vascular anomalies involving the masseter muscle histologically diagnosed primarily as cavernous hemangiomas and presents the imaging and pathologic findings. Ample surgical excision was performed through an intraoral approach. Immunohistochemistry showed no uptake of glucose transporter protein 1. The literature was reviewed and the designation intramasseteric vascular malformation for this entity is proposed.

Safety and Clinical Efficacy of Onyx for Embolization of Extracranial Head and Neck Vascular Anomalies: Fig 1.

Onyx was developed for embolization of central nervous system AVMs but is increasingly used extracranially because of its unique physical properties. We review our experience and results with the use of Onyx for the treatment of fast-flow extracranial vascular lesions. We retrospectively analyzed clinical and imaging records of 22 patients who underwent 71 extracranial embolizations from March 2007 through January 2010. The diagnoses were the following: cervicofacial AVM (n = 18), traumatic fistula (n = 3), and vessel laceration (n = 1). In 62 of 71 procedures (87%), Onyx was the sole embolic agent; it was delivered transarterially in 67/71 and percutaneously in 4/71 procedures. Clinical goals included amelioration of pain and control of bleeding. The clinical efficacy of embolization was judged by symptom control, and adverse events were assessed by clinical examination and history, both postembolization and 4 weeks postprocedure. Cessation of acute bleeding was achieved in 13/14 cases, with 1 case of immediate recurrent massive epistaxis prompting reintubation and further embolization. Control of subacute bleeding episodes and pain was achieved for all patients. Following staged embolization, 7 patients underwent surgical resection without significant blood loss. Surgeons reported high satisfaction with the intraoperative handling properties of Onyx. Transient swelling, local tenderness, or numbness was encountered after 7 procedures. There were no stuck catheters, vessel dissections, or vessel ruptures and no skin discoloration. Staged Onyx embolization was clinically efficacious in managing extracranial fast-flow vascular malformations and lesions, with low associated morbidity.

Neck ultrasound for prediction of right nonrecurrent laryngeal nerve

Nonrecurrent laryngeal nerve (NRLN) is 1 of the important causes for nerve damage during neck surgery. The anomaly is almost associated with congenital vascular abnormally. Most neck vascular anomalies can be detected by ultrasound. Both 3.5-MHz and 10-MHz probe neck ultrasound scans were performed for 2330 patients undergoing thyroidectomy preoperatively. Absence of innominate artery (INA) was defined as positive with right NRLN. Of 13 positive patients found by 10-MHz probe, 11 were also identified by 3.5-MHz probe, and proved to be with right NRLN during operation. Two false-positive patients (18%) found by 10-MHz probe were due to short INA and tortuous INA, respectively. The incidence of right NRLN was 0.47% in Chinese people. Both the sensitivity and specificity for predicting right NRLN by 3.5-MHz probe were 100%. A 3.5-MHz probe neck ultrasound scan can accurately demonstrate right NRLN. Applying this tool for neck surgery to reduce the nerve damage is highly advised.

Head and neck vascular anomalies in children

Vascular lesions are the most common congenital and neonatal abnormalities. The aim of this work is to point out differences between various vascular anomalies, in order to define accurate diagnosis, and to present different therapeutic options now used for the treatment of the vascular lesions in children. According to biological classification described by the work of Mulliken and Glowacki there are two major types of vascular abnormality: haemangioma and vascular malformation. Haemangioma is a distinct biologic tumour entity characterised by rapid endothelial proliferation shortly after birth. The lesion is absent at birth and growth in early infancy, followed by a spontaneous resolution in childhood. Vascular malformations are structural anomalies that have a normal growth rate and endothelial turnover. According to the morphology of the vessels and the flow rate we distinguish: slow-flow and fast-flow vascular malformation. The authors document their personal experience in diagnosis, clinical evaluation, treatment and follow-up of the vascular lesions.

Forty-one Cervicofacial Vascular Anomalies and Their Surgical Treatment – Retrospection and Review

Haemangiomas in children usually involute spontaneously and surgical treatment is exceptional. Vascular malformations do not regress spontaneously and resection may become necessary. We present a series of surgically treated face and neck vascular anomalies during a 9-year period, assessing the epidemiology, presenting signs and symptoms, diagnostic modalities, indications for surgery, treatment methods and clinical outcome post-treatment. The medical and pathological records of all patients with cervicofacial vascular anomalies treated surgically at our department from 1997 to 2005 were retrospectively reviewed in relation to current evidence. Forty-one patients were identified. Of these, 9 patients had haemangiomas and the remaining 32 had a variety of vascular malformations. Cervicofacial vascular anomalies were most commonly located at the lip. Atypical looking vascular anomalies like masseteric intramuscular haemangiomas and parotid malformations were diagnostic problems. All 41 had surgical excision of their vascular anomalies for troubling symptoms, cosmesis or diagnostic purpose. For cervicofacial arteriovenous malformations, 28% were classified as Schobinger stage I, 50% stage II, and the remainder stage III. Combined embolisation-resection was used to treat 6 arteriovenous malformations (stage II to III) and of these, 3 required flap reconstruction. Accurate diagnosis distinguishing between cervicofacial haemangiomas and vascular malformations is key to best treatment. The diagnosis can usually be made by history and physical examination aided by early magnetic resonance imaging (MRI). Although cervicofacial haemangiomas can be managed conservatively or with medical therapy, surgery is indicated for preventing psychological distress and in cases of chronic aesthetic alteration resulting from partial regression. Aesthetic concerns and prevention of psychosocial distress point to early excision of venous malformation as the treatment of choice. Lymphatic malformations are best treated by excision. Outcome after excision of localised cervicofacial haemangiomas and low-flow vascular malformations is excellent. Large extensive low-flow malformations as well as those located at the lips may require multiple procedures including reconstruction; patients should be informed that the outcome is generally not as good. Combined embolisation-resection is definitive treatment for arteriovenous malformations and flap reconstruction may prevent their recurrence. Tissue expansion is a useful reconstructive tool after the excision of large vascular anomalies.

Excision of mandibular vascular malformations via a lateral corticotomy approach

Statement of the Problem: Traditional methods of treating “high flow” vascular lesions have often involved aggressive, cosmetically deforming surgery. Following embolization, ligation of feeder vessels, and sclerosis, the majority of literature recommends en bloc resection of the mandible with adequate surgical margins. This has been followed by immediate or delayed bone grafting. In a retrospective review analysis, 4 patients with high flow mandibular vascular lesions were treated with a lateral corticotomy technique and curettage of the tumor was then performed without creating a continuity defect of the mandible. Materials and Methods: Due to the paucity of these types of lesions, specifically within the mandible, the study ranged from 1997 to 2004. The 5 patients ranged in age from 1 to 38. Patients were all diagnosed with “high flow” vascular lesions of the mandible, mostly arteriovenous malformations. At the time of surgery, each patient had prior embolization by interventional radiology. During the procedure, direct puncture embolization also took place in all patients. A window of lateral bone was removed in all patients and the lesions removed by curettage, along with resection of the involved nerves, arteries, and veins. The lingual cortex and inferior border were left intact. All surgical cavities were left to fill in on their own except for one where allogeneic bone graft putty was placed. One patient required a reconstruction plate that was later removed. Follow-up was carried out for each of the patients at regular intervals, and a Panorex film was taken at several points in time. A follow up angiogram, CT, or biopsy was obtained if recurrence was suspected. Follow up times ranged from 2 weeks to 3 years. Results: All five patients were compliant with follow up. Patients followed for longer than 6 months had adequate bone fill. In one case a recurrence was noted at 3 years after surgery. This required a second lateral corticotomy slightly more anterior to the first. The remaining patients have not showed recurrence to date. Conclusion: Our study shows 80 percent success after the first attempt of treatment. The literature supports 67–75 percent success. In our series we avoided creating a continuity defect of the mandible, which allowed avoidance of some of the morbidity associated with complete resection. Traditional complications associated with mandibular plates were not present, because they were not used in the majority of our patients. Similarly, bone grafts were not placed, and so morbidity from a second surgical site was avoided as well. References Kaban LB, Bleaser B, Perrott DH: Head and neck vascular anomalies. Select Readings Oral Maxillofac Surg 5, 1997 Kademani D, Costello BJ, Ditty D, et al: An alternative approach to maxillofacial arteriovenous malformations with transosseous direct puncture embolization. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 97, 2004 (submitted for publication) Kohout MP, Hansen M, Pribaz JJ, et al: Arteriovenous malformations of the head and neck: Natural history and management. Plast Reconstr Surg 102:643, 1998

Application of Doppler ultrasonography in congenital vascular lesions of the head and neck.

To evaluate the value of Doppler ultrasonography in the assessment of congenital vascular lesions of the maxillofacial region. 27 new patients with 36 congenital vascular lesions in the maxillofacial region as well as 10 patients previously treated with cryotherapy underwent ultrasonography (US) with a 6.0-10.0 MHz broadband linear transducer (reference Doppler frequency 7.5 MHz). Initial grey-scale US was followed by Color Doppler, Power Doppler and spectral Doppler studies. The presence or absence of flow was noted. In areas of high vascularity the number of vessels within a 1 cm(2) restricted region of interest were increased by spectral Doppler. US allowed differentiation of seven hemangiomas from other congenital vascular lesions and thus appropriate treatment of patients. Low flow vessels were demonstrated within the scars of five lesions previously treated with cryotherapy, requiring further intervention. Doppler US is a widely available, non-invasive and relatively inexpensive technique which can be used to characterize the flow of head and neck vascular anomalies and thus differentiate hemangiomas from other vascular malformations.

Embolization of a life‐threatening mandibular vascular malformation by direct percutaneous transmandibular puncture

Vascular malformations of the mandible are uncommon, but often present with significant hemorrhage. Transarterial vessel occlusion has become a valuable primary or adjunctive treatment for such lesions, as well as for most other symptomatic congenital and acquired head and neck vascular anomalies. Permanent embolic obliteration of the malformation requires placement of occlusive material directly into the nidus (core) of the lesion. Prohibitively complex proximal vasculature may prevent successful catheter positioning and lead to failure of traditional embolotherapy. Even optimal placement of arterial embolic material may fail to fully obliterate the nidus, allowing eventual restoration of flow to the lesion due to arterial recanalization. Under such circumstances it may be possible to obliterate the malformation and control lesional hemorrhage by occlusion of the malformation or its venous drainage by direct percutaneous mandibular puncture. In our case, multiple transarterial embolizations failed to sufficiently manage a symptomatic vascular malformation. Successful embolotherapy was performed via direct puncture of the venous side of the malformation through the mandibular cortex. Venous thrombosis induced concomitant occlusion of abnormal arteriovenous shunts, resulting in long-term control of life-threatening oral hemorrhage.