Interdisciplinary Management of Vascular Anomalies in the Head and Neck. / Interdisziplinäres Management vaskulärer Anomalien im Kopf-Hals-Bereich.

Vascular anomalies in the head and neck area are usually rare diseases and pose a particular diagnostic and therapeutic challenge. They are divided into vascular tumours and vascular malformations. A distinction is made between benign tumours, such as infantile haemangioma, and rare malignant tumours, such as angiosarcoma. Vascular malformations are categorised as simple malformations, mixed malformations, large vessel anomalies and those associated with other anomalies. Treatment is interdisciplinary and various modalities are available. These include clinical observation, sclerotherapy, embolisation, ablative and coagulating procedures, surgical resection and systemic drug therapy. Treatment is challenging, as vascular anomalies in the head and neck region practically always affect function and aesthetics. A better understanding of the genetic and molecular biological basis of vascular anomalies has recently led to clinical research into targeted drug therapies. This article provides an up-to-date overview of the diagnosis, clinic and treatment of vascular anomalies in the head and neck region.

Drainage Pattern of Craniofacial Veins With Emphasis on its Influence on Facial Reconstruction Procedures.

As the facial transplantation procedures are becoming more popular and frequent in recent years, for repairing facial trauma, variations in the veins of head and neck needs to be reported time and again. This study was undertaken to examine the course and drainage pattern of the facial vein and external jugular vein on this context and emphasize its surgical implications. The authors studied the head and neck region of 50 embalmed cadavers of both sexes to document normal and variant anatomy of facial, retromandibular, and external jugular veins. In 30% of the head and neck regions, different draining pattern of the above-mentioned veins were observed. One of the rare variation discovered was the splitting of the retromandibular vein to embrace the external carotid artery within the parotid gland. The data about variations in the termination of facial vein, retromandibular vein, and external jugular vein, as observed in the present study might be useful in avoiding accidental injury to these vessels during any surgical intervention in the face as well as neck. Level of Evidence: IV.

Reconstruction after ablative treatment of arteriovenous malformations of the head and neck.

Arteriovenous malformations are high-flow congenital vascular malformations that are characterized by abnormal connections between arteries and veins across an abnormal capillary bed. The head and neck region is the most common location for extracranial arteriovenous malformations. Due to their highly invasive, aggressive, and locally destructive nature, arteriovenous malformations are difficult to treat, and management through a multimodal, multidisciplinary approach is recommended. The mainstay of treatment is embolization and surgical excision. With the advent of genetic research, adjuvant targeted medical therapy is starting to play a larger role. Successful surgical treatment with or without embolization requires removal of the nidus. Large, focal, or diffuse lesions involving multiple anatomic areas usually necessitates surgical reconstruction. Free tissue transfer has many advantages over more conservative methods with restoration of function and contour. Herein, the authors describe the surgical treatment and reconstruction in head and neck arteriovenous malformations.

Current classification of vascular anomalies of the head and neck.

Vascular anomalies of the head and neck comprise a wide spectrum of phenotypically diverse lesions. Optimal diagnosis and management of these lesions are critically dependent upon establishment of uniform and well-defined histopathologic, clinical, and radiological criteria, but these remain subject of debate. In this paper, we describe the International Society for the Study of Vascular Anomalies classification scheme, which was first published in 1996 and updated in 2014. The strength of this proposal rests on its distinction between vascular malformations and tumors, and is responsible for its wide adoption. This paradigm serves as a developing platform for diagnosis, inter-collegial communication, and treatment, and adhering to it will help clinicians to improve the management of vascular anomalies.

Multimodality treatment of vascular anomalies of the head and neck.

BACKGROUND: Vascular anomalies affect up to 5% of children with the majority affecting the head and neck. They present at different ages as a wide variety of lesions. A careful evaluation with history, physical examination, and imaging assists in the proper diagnosis. Depending upon the condition treatment options for vascular anomalies include topical therapy, selective photothermolysis, sclerotherapy, embolization, surgical excision, and targeted systemic therapy. CONCLUSION: Staged multimodal therapeutic regimens have proven to best control disease and allow for the preservation of function and aesthetics. The timing, sequence, and combination of therapies are best determined by a multidisciplinary vascular anomalies team. Patients and families need to be counseled on anticipated positive outcomes following a protracted course of treatment for the majority of vascular anomalies.

Surgical management of vascular tumors and malformations of the head and neck in adults.

Vascular tumors and malformations present a diagnostic and therapeutic challenge to many physicians. Because these lesions are rare, few surgeons have enough experience with them other than those practicing in tertiary vascular anomaly treatment centers. Some patients may have been misdiagnosed or mistreated during childhood and present in adult age with either recurrence or with an untreated lesion. Ideally, a multidisciplinary treatment team should be involved to discuss management with the patient including specialists in surgery, interventional radiology, pathology, hematology, genetics, and dermatology. As our understanding of the pathogenesis of these lesions grows, novel therapies are being employed which may decrease the need for surgery. Nevertheless, some lesions need definitive treatment with surgery. Improving understanding of the surgical management of vascular anomalies will improve cosmetic and functional outcomes for patients.

Intralesional therapies for vascular malformations of the head and neck.

Vascular malformations of the head and neck represent a spectrum of complex vascular anomalies, requiring a multidisciplinary approach toward diagnosis and treatment. Several intralesional therapeutic agents have been devised and pioneered over the years, some of which are now primary and standard of care for the management of these lesions. In this article, the authors discuss the currently available intralesional therapeutic agents for the management of vascular malformations in the head and neck region.

Head and Neck CTA Utilization: Analysis of Ordering Frequency and Nonroutine Results Communication, With Focus on the 50 Most Common Emergency Department Clinical Presentations.

BACKGROUND. Utilization of head and neck CTA in the emergency department (ED) has grown disproportionately to other neuroimaging examinations. OBJECTIVE. The purpose of this article was to characterize utilization of head and neck CTA in the ED, comparing utilization and frequency of nonroutine results communication among patients' chief concerns. METHODS. All adult ED visits for a single health care system from January 2014 to December 2017 were retrospectively reviewed. Variables recorded included chief concerns, whether head and neck CTA was performed, and, if so, whether the report documented nonroutine results communication. The 50 chief concerns resulting in the highest number of head and neck CTA examinations were identified. Frequencies of head and neck CTA ordering and of nonroutine results communication were calculated. A subset of reports documenting nonroutine communication were manually reviewed. RESULTS. Head and neck CTA was ordered in 2.5% (17,903) of 708,145 ED visits in 236,476 patients (mean age, 49.8 ± 20.5 [SD] years; 110,952 men, 125,521 women, 3 unknown sex). Head and neck CTA was ordered for 833 distinct chief concerns. Nonroutine results communication was documented for 17.6% (3155/17,903) of examinations. Among the 50 chief concerns associated with the highest number of examinations, frequency of ordering head and neck CTA ranged from less than 0.5% (five concerns) to 55.2% (stroke code), and frequency of nonroutine communication ranged from 5.6% (transient ischemic attack) to 67.5% (unresponsive). Chief concerns not among the 50 most common accounted for 50.0% (8956/17,903) of examinations; these exhibited a collective frequency of nonroutine communication of 4.8% (429/8956). Manual review of 11.1% (350/3155) of reports with a nonroutine communication indicated an acute finding related to the indication in 51.1%, nonemergent but potentially explanatory finding in 14.0%, incidental finding in 28.0%, and communication of negative results in 6.9%. CONCLUSION. Head and neck CTA is ordered in 2.5% of ED visits for a wide range of chief concerns. Frequencies of ordering and of nonroutine results communication are highly variable among chief concerns. Acute indication-related findings account for half of nonroutine radiologist communications. CLINICAL IMPACT. Insight into patterns regarding head and neck CTA ordering and nonroutine results may help optimize patient selection and radiologist communications in the ED setting.

Domestic pig head and neck arteries from the viewpoint of imaging methods and experimental surgery

The aim of this paper is to summarize the current knowledge of the anatomy of domestic pig head and neck arteries for the needs of experimental surgery and imaging methods in biomedical research and translational medicine. The potential of this large animal model seems to be valuable also for the xenotransplantation of certain organs. Demands for the knowledge of morphological differences between analogous human structures and particular breeds are growing also in connection with the need for more precise planning of experiments or interpretation of the results. Deepening anatomical knowledge is allowed also by the development of imaging methods. The search was performed using the keywords "domestic pig" and "arteries of the head and neck" in the MEDLINE database, PubMed interface.

The modified 5-item frailty index is a predictor of perioperative risk in head and neck microvascular reconstruction: An analysis of 3795 cases.

BACKGROUND: The modified frailty index 5 (mFI-5)-a scale based on the five variables diabetes, hypertension, chronic obstructive pulmonary disease, congestive heart failure, and functional dependency-has been shown to be a valid predictor of surgical outcomes. In this study we sought to evaluate the ability of the mFI-5 to predict the postoperative outcomes of head and neck microvascular reconstruction. METHODS: Review of the 2015-2019 American College of Surgeons, National Surgical Quality Improvement Program (ACS NSQIP) database identified 5323 cases of microvascular reconstruction, of which 3795 were head and neck cases that provided parameters necessary to calculate the mFI-5. The groups were compared in terms of demographics and comorbidities. Post-operative outcomes assessed included mortality, average operative time and length of hospital stay, surgical and medical complications, and non-home discharge. RESULTS: Increases in the mFI were associated with longer hospitalization periods (10.5 ± 7.5 days in mFI 0 vs 14.9 ± 15.4 in mFI ≥ 3; p < 0.0001) higher rates of mortality (1% in mFI 0 vs 3.1% in mFI ≥ 3; p = 0.02), reoperation (15.4% in mFI 0 vs 17.2% in mFI ≥ 3; p = 0.002) and unplanned readmission (7.6% in mFI 0 vs 18.8% in mFI ≥ 3; p = 0.001). Rates of any complications (p < 0.0001), as well as surgical (p < 0.002) and medical (p < 0.0001) complications specifically were higher with greater mFI scores. Higher mFI scores also predicted decreased home discharge (p < 0.0001). Differences remained significant on multivariate analysis and subgroup analysis by age. CONCLUSION: The mFI-5 is a significant predictor of risk in microvascular head and neck reconstruction. Subgroup analysis by age highlights that the tool can help identify younger patients who are frail and hence at risk. Through appropriate pre-operative identification of frail patients surgeons can prospectively modify their operative and discharge planning as well as post-operative support.

Standing Waves on Computed Tomography Angiography in Multiple Vessels in a Young Trauma Patient.

ABSTRACT: Standing waves are a phenomenon of uncertain etiology seen on imaging. We present the first case demonstrating standing waves on computed tomography angiography in multiple vessels in a single patient with imaging evidence of resolution in some of the vessels. Our case further supports the literature that standing waves are a physiologic phenomenon, likely because of flow mechanics, rather than modality.

Peri-procedural Anticoagulation in Patients with Head and Neck Versus Extremity Venous Malformations.

OBJECTIVE: (1) Review a multidisciplinary vascular anomalies center's practice regarding periprocedural anticoagulation for venous malformations (VM) and the associated risk of thromboembolic and disseminated intravascular coagulation (DIC) events. (2) Compare the risk of thromboembolic events and DIC post-procedure between head and neck (H&N) and extremity VM patients. METHODS: An Institutional Review Board (IRB)-approved, retrospective chart review was performed on 120 VM patients. A thromboembolic event was defined as a thrombus formation post-sclerotherapy or post-surgery within 2 months in a distant or local venous structure not directly addressed by the procedure. RESULTS: There were 39 cases involving the H&N and 81 cases based at the extremities. There were eight cases of post-procedure thrombus formation within the extremity VM group (8/71; 11.3%) as opposed to 0 cases in the H&N group (OR: 0, 95% CI .00-.09), p = .049. There was no difference in incidence of post-procedure thromboembolic events between those with elevated D-dimer (H&N: 0%, extremity: 22.7%, 5/22) and normal D-dimer values (H&N: 0%, extremity: 6.3% [1/16], P = .370). There was no difference in incidence of post-procedure thromboembolic events between those who received periprocedural anticoagulation (H&N: 0%, extremity: 21%, 4/19) and those who did not (H&N: 0%, extremity: 8.2%, 4/49), (Extremity: OR: 3.00, .67-13.50, P = .206). CONCLUSION: Post-procedure thromboembolism is rare in the treatment of venous malformations, especially in the head and neck subsite. Regardless of anticoagulation use, there were no thromboembolic events for H&N VM patients. Such events are rare, and the odds may approach zero, especially with small sample size. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1163-1167, 2021.

Outcomes of Venous End-to-Side Microvascular Anastomoses of the Head and Neck.

OBJECTIVES/HYPOTHESIS: The literature on outcomes of end-to-side (ETS) anastomoses for microvascular reconstruction of the head and neck is limited. This series reviews ETS in free tissue transfer (FTT) across multiple institutions to better understand their usage and associated outcomes. STUDY DESIGN: Retrospective review of 2482 consecutive patients across three tertiary institutions. METHODS: Adult patients (> 18) who received a FTT from 2006 to 2019 were included. RESULTS: Two hundred and twenty-one FTT were identified as requiring at least one ETS anastomosis. These ETS cases had a failure rate of 11.2% in comparison to 3.8% in a cohort of end-to-end (ETE) cases (P < .001). ETS cases were significantly more likely to have a prior neck dissection (P < .001), suggesting the ETS method was utilized in select circumstances. A second ETS anastomosis improved survival of the FTT (P = .006), as did utilization of a coupler over suture (P = .002). Failure due to venous thrombosis was significantly more common with one ETS anastomosis instead of two ETS anastomoses (P = .042). CONCLUSIONS: ETS is effective but is often used as a secondary technique when ETE is not feasible; as such, in this series, ETS was associated with higher failure. A second anastomosis and the use of the coupler for completing the anastomoses were associated with lower rates of failure. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:1286-1290, 2021.

Contemporary Management of Vascular Anomalies of the Head and Neck-Part 1: Vascular Malformations: A Review.

Importance: Vascular anomalies of the head and neck are relatively rare lesions. Management is challenging because of the high likelihood of involvement of functionally critical structures. Multiple modalities of treatment exist for vascular anomalies of the head and neck, including medical therapies, sclerotherapy and embolization procedures, and surgery. This review focuses on the accurate diagnosis and the relative roles of the various therapeutic options. Observations: Vascular anomalies are classified by the International Society for the Study of Vascular Anomalies into 2 major groups: vascular tumors and vascular malformations. Vascular tumors encompass proliferative lesions ranging from infantile and congenital hemangiomas to kaposiform hemangioendothelioma. Alternatively, vascular malformations are embryologic errors in vasculogenesis. This article focuses on the management of vascular malformations. The 3 primary vascular malformation subclassifications are lymphatic, venous, and arteriovenous. The burden of disease, diagnosis, and current management options are discussed in detail for each subtype. Conclusions and Relevance: Most vascular malformations of the head and neck require a multidisciplinary approach. Available medical, interventional radiologic, and surgical interventions are constantly evolving. Optimization of function and cosmesis must be balanced with minimization of treatment-associated morbidity. Otolaryngologists-head and neck surgeons must remain up to date regarding options for diagnosis and management of these lesions.

Genetic landscape of common venous malformations in the head and neck.

OBJECTIVE: Common venous malformations (VMs) are a frequent sporadic subtype of vascular malformations. Given the TEK and PIK3CA mutations identified, this study aims to investigate the genetic landscape of VMs in the head and neck. METHODS: Patients from published sequencing studies related to common VMs were reviewed. Detailed data regarding clinical characteristics, sequencing strategies, and mutation frequency were synthesized. Lesion distribution of common VMs in the head and neck were further retrospectively analyzed by the pathologic database of the Department of Oral and Maxillofacial-Head and Neck Oncology, Shanghai Ninth People's Hospital. For the frequently affected sites in the head and neck, patients were selected for targeted sequencing with a designed vascular malformation-related gene panel or whole exome sequencing. Detected variants were analyzed by classical bioinformatic algorithms (SIFT23, PolyPhen-2 HDIV, LRT, MutationTaster, Mutation Assessor, and GERP++). To confirm the expression pattern of particular candidate gene, specimens were examined histochemically. Gene ontology enrichment analysis and a protein-protein interaction network were also constructed. RESULTS: Three hundred patients from eight sequencing studies related to common VMs were reviewed. The total prevalence rates of TEK and PIK3CA mutations were 41.3% and 26.7%, respectively. The most frequent TEK/PIK3CA mutations were TEK-L914F/PIK3CA-H1047R. TEK/PIK3CA mutations existed in 70.3% and 2.7% of VMs in the head and neck. In retrospective data from 649 patients carrying cervicofacial VMs at Shanghai Ninth Hospital, the most frequent sites were the maxillofacial region (lips, cheek, parotid-masseteric region, submandibular region) and the oral and oropharyngeal region (buccal mucosa, tongue). Targeted sequencing for 14 frequent lesions detected TEK variants in three patients (21.4%), but no PIK3CA mutations. On whole exome sequencing of two patients without TEK/PIK3CA mutations, CDH11 was the only shared deleteriously mutated gene. Bioinformatic analyses of CDH11 implied that genes involved in cellular adhesion and junctions formed a significant portion. CONCLUSIONS: Common VMs of the head and neck have a unique genetic landscape. Novel CDH11 and TEK variants imply that pathogenesis is mediated by the regulatory relationship between endothelial cells and extracellular components.

Abnormal Vasculature Development in Zebrafish Embryos with Reduced Expression of Pantothenate Kinase 2 Gene.

Mutations in pank2 gene encoding pantothenate kinase 2 determine a pantothenate kinase-associated neurodegeneration, a rare disorder characterized by iron deposition in the globus pallidus. To extend our previous work, we performed microinjections of a new pank2-specific morpholino to zebrafish embryos and thoroughly analyzed vasculature development. Vessels development was severely perturbed in the head, trunk, and tail, where blood accumulation was remarkable and associated with dilation of the posterior cardinal vein. This phenotype was specific as confirmed by p53 expression analysis and injection of the same morpholino in pank2-mutant embryos. We can conclude that pank2 gene is involved in vasculature development in zebrafish embryos. The comprehension of the underlining mechanisms could be of relevance for understanding of pantothenate kinase-associated neurodegeneration.

Image-guided percutaneous sclerotherapy of venous malformations of the head and neck: Clinical and MR-based volumetric mid-term outcome.

OBJECTIVE: To report the clinical and MRI-based volumetric mid-term outcome after image guided percutaneous sclerotherapy (PS) of venous malformations (VM) of the head and neck. METHODS: A retrospective analysis of a prospectively maintained database was performed, including patients with VM of the head and neck who were treated with PS. Only patients with available pre- and post-interventional MRI were included into this study. Clinical outcome, which was subjectively assessed by the patients, their parents (for paediatric patients) and/or the physicians, was categorized as worse, unchanged, minor or major improvement. Radiological outcome, determined by MRI-based volumetric measurements, was categorized as worse (>10% increase), unchanged (≤10% increase to <10% decrease), minor (≥10% to <25% decrease), intermediate (≥25% to <50% decrease) or major improvement (≥50% decrease). RESULTS: Twenty-seven patients were treated in 51 treatment sessions. After a mean follow-up of 31 months, clinical outcome was worse for 7.4%, unchanged for 3.7% of the patients, while there was minor and major improvement for 7.4% and 81.5%, respectively. In the volumetric imaging analysis 7.4% of the VMs were worse and 14.8% were unchanged. Minor improvement was observed in 22.2%, intermediate improvement in 44.4% and major improvement in 11.1%. The rate of permanent complications was 3.7%. CONCLUSION: PS can be an effective therapy to treat the symptoms of patients with VMs of the head and neck and to downsize the VMs. MRI-based volumetry can be used to objectively follow the change in size of the VMs after PS. Relief of symptoms frequently does not require substantial volume reduction.

Posture-induced changes in the vessels of the head and neck: evaluation using conventional supine CT and upright CT.

Since the venous system is affected by gravity, upright computed tomography (CT) in addition to conventional supine CT has great potential for evaluating postural changes in the venous system. We evaluated the morphological differences in the head and neck vessels by performing a contrast CT study in both the supine and the sitting positions. In this study, the 20 included participants (10 men and 10 women) were healthy adults aged 30 to 55 years. The cross-sectional area of the cervical vessels, craniocervical junction veins, and intracranial vessels were obtained quantitatively. Venous sinuses and venous plexuses that were difficult to measure were evaluated qualitatively. The average change in areas from a supine to an upright posture was - 77.87 ± 15.99% (P < 0.0001) in the right internal jugular vein (IJV), - 69.42 ± 23.15% (P < 0.0001) in the left IJV, - 61.52 ± 12.81% (P < 0.0001) in the right external jugular vein (EJV), and - 58.91 ± 17.37% (P < 0.0001) in the left EJV. In contrast, the change in the anterior condylar vein (ACV) from a supine to an upright posture was approximately + 144% (P < 0.005) on the right side and + 110% (P < 0.05) on the left side. In addition, according to the qualitative analysis, the posterior venous structures including the anterior condylar confluence (ACC) of the craniocervical junction became more prominent in an upright posture. Despite these changes, the intracranial vessels showed almost no change between postures. From a supine to an upright position, the IJVs and EJVs above the heart collapsed, and venous channels including the ACCs and ACVs opened, switching the main cerebral venous drainage from the IJVs to the vertebral venous system. Upright head CT angiography can be useful for investigating physiological and pathophysiological hemodynamics of the venous system accompanying postural changes.

Vascular anomalies of the head and neck.

The head and neck are the most common site of involvement for vascular tumors and malformations, with more than half of all vascular anomalies seen in this region. Lesions in this location can cause significant disfigurement and can be associated with airway obstruction, impairment in vision or hearing, swallowing disorders and hemorrhage. Accurate diagnosis is critical in determining treatment, and interdisciplinary care is essential for optimal management. We review clinical and imaging features that are key to establishing the correct diagnosis, and review treatment modalities, with emphasis on interventional and surgical procedures.