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.

'[ ] the situation in the schools still remains the Achilles heel.' Barriers to the implementation of school tobacco policies-a qualitative study from local stakeholder's perspective in seven European cities.

School tobacco policies (STPs) are a crucial strategy to reduce adolescents smoking. Existing studies have investigated STPs predominantly from a school-related 'insider' view. Yet, little is known about barriers that are not identified from the 'schools' perspective', such as perceptions of local stakeholders. Forty-six expert interviews from seven European cities with stakeholders at the local level (e.g. representatives of regional health departments, youth protection and the field of addiction prevention) were included. The analysis of the expert interviews revealed different barriers that should be considered during the implementation of STPs. These barriers can be subsumed under the following: (i) Barriers regarding STP legislature (e.g. inconsistencies, partial bans), (ii) collaboration and cooperation problems between institutions and schools, (iii) low priority of smoking prevention and school smoking bans, (iv) insufficient human resources and (v) resistance among smoking students and students from disadvantaged backgrounds. Our findings on the expert's perspective indicate a need to enhance and implement comprehensive school smoking bans. Furthermore, collaboration and cooperation between schools and external institutions should be fostered and strengthened, and adequate human resources should be provided.

The Natural History of Soft Tissue Hypertrophy, Bony Hypertrophy, and Nodule Formation in Patients With Untreated Head and Neck Capillary Malformations.

BACKGROUND: A percentage of patients with capillary malformation (CM) develop soft tissue hypertrophy, bony hypertrophy, and/or nodule formation. OBJECTIVE: To determine the incidence, age of onset, anatomic distribution of soft tissue/bony hypertrophy, and nodule formation in patients with untreated CM. METHODS: A retrospective medical records review of head and neck CM patients presenting to a tertiary referral center over a 7-year period (2004-2011) was performed. RESULTS: Of the 160 patients with CM, 96 demonstrated progression of disease to include either soft tissue/bony hypertrophy or nodule formation. Of these, 87 patients had not received previous treatment and met the inclusion criteria for analysis. On average, soft tissue hypertrophy began at 9 years of age. The V2/maxillary segment was most commonly involved with upper lip hypertrophy being the most prominent. Fourteen percent of the patients also presented with bony hypertrophy, which began at an average age of 15 years. Nodules were present in 38/87 (44%) of patients with an average age of onset of 22 years. CONCLUSION: This study demonstrates the nature progression of CM and quantifies the clinical characteristics of hypertrophy and nodule formation with untreated head and neck CM. Early and continuous treatment is recommended in hopes of preventing CM progression.

Expression Patterns of HIF-1α Under Hypoxia in Vascular Smooth Muscle Cells of Venous Malformations.

PURPOSE: The molecular pathophysiology of venous malformations (VMs), which are a type of vascular malformation, is poorly understood. Until now, it is known that VM lesions are related to the process of angiogenesis. Because angiogenesis is induced under hypoxic conditions, hypoxia is thought to be important in VM lesion formation. Therefore, we examined the implications of hypoxia on the biological behavior of VM vascular smooth muscle cells (VSMCs). In doing so, we investigated the expression patterns of hypoxia-inducible factor-1α (HIF-1α), which plays a key role in hypoxia-induced angiogenesis, to provide a further understanding of the molecular mechanisms involved in VM. METHODS: Vascular smooth muscle cells from 5 normal veins and 5 VM lesions were cultured under moderate hypoxic conditions (3% O2, 5% CO2). The effects of hypoxia on HIF-1α expression were measured by immunocytochemical staining, reverse transcription-polymerase chain reaction, and real-time reverse transcription-polymerase chain reaction. RESULTS: Overall, the expression of HIF-1α in cells was high after exposure to hypoxia for 6 or 12 hours, but decreased after 24 hours of hypoxia. HIF-1α expression in VM VSMCs was 2 times higher than that in normal VSMCs. Immunocytochemically, HIF-1α was mainly located in the nucleus and the intensity in VM VSMCs was stronger after 6 and 12 hours of hypoxia when compared to the expression pattern of HIF-1α in VSMCs from normal tissue. This suggested that VM tissue is more susceptible to the effects of hypoxia than normal tissue. CONCLUSIONS: These results indicate that the high expression of HIF-1α in VM VSMCs under hypoxic conditions could be an important factor for stimulating downstream angiogenesis in VM. Furthermore, the results of this investigation could provide the basis for future studies of VM pathophysiology, and ultimately lead to the development of new therapeutic approaches.

Surgical Management of Scalp Infantile Hemangiomas.

BACKGROUND: Scalp infantile hemangiomas (IHs) are usually focal lesions that can be both disfiguring and may lead to complications such as ulceration and bleeding. The clinical features of scalp IHs have not been previously studied. This study aims to identify the clinical characteristics associated with scalp IH, the indications for surgical intervention, and results of surgical treatment. METHODS: We performed a retrospective chart review of patients with scalp IH presenting to a tertiary care referral center over the past 7 years. Patients' demographics, clinical features, location, and treatment course were noted. RESULTS: One hundred fifty-one of 1916 total IH patients presented with a diagnosis of scalp IH (8%). The distribution of the scalp lesions was the following: 31.8% frontal, 46.7% parietal, 7.9% occipital, and 9.9% temporal. Fifty-eight percent were solitary and 42% were multifocal lesions. The size range of scalp IH is 1 × 1 cm to 8 × 6 cm. Two percent of patients with scalp IH presented with other facial IH. Primary indications for surgery were secondary to complications such as ulceration (23.2%) and alopecia (51.7%). Surgery included elliptical excision with primary closure (85.7%) or with rotational flap closure (14.3%). The average age of surgery was 3 years (1-8 years). Most patients had a good aesthetic outcome with satisfactory hair growth. CONCLUSION: Scalp IHs are morbid tumors which often cause alopecia and/or ulceration. In our experience, many scalp IHs eventually require surgical intervention. We find that early surgical excision is beneficial, as the tissues are easily manipulated secondary to scalp/soft tissue laxity and scarring is more favorable.

A Biomechanical Evaluation of Auricular Cartilage Autografts in Orbital Floor Defect Repair.

OBJECTIVE: Auricular cartilage is used as a surgical implant in the management of orbital floor fractures. However, no specific parameters exist regarding the use/limitations of this potential graft. In order to determine the mechanical efficacy of adult auricular cartilage grafts, a mechanical model was developed and studied for structural threshold size limits. METHODS: Thirty-seven cadaveric auricular cartilage specimens were tested in a laboratory. A plexiglass baseplate was created with four different sized holes, defined as 1.0×, 1.2×, 1.4×, and 1.6× the mean minor axis of the specimens. Each specimen was used to bridge one hole under increasing loads until mechanical failure. Structural stiffness at three different loading stages, structural failure strength, and percent failure of the entire system for each defect size was calculated. RESULTS: Specimens tested on 1.0×, 1.2×, 1.4× and 1.6× defects demonstrated 0%, 0%, 20%, and 60% system failure rates, respectively. Structural stiffness curves showed a similar trend, with ANOVA demonstrating a significant difference in mechanical properties between defect sizes (p = 0.03). The curve representing 1.6 × defect size demonstrated significantly reduced structural stiffness relative to 1.0×, 1.2×, and 1.4× curves. There was no statistical difference between 1.2× and 1.4× testing sets (p = 0.09). CONCLUSION: A clinically significant biomechanical and functional threshold exists between 1.2×and 1.4× defect sizes. Given a mean minor axis of 2.06 cm, orbital blow-out defects <2.4 cm (1.2 × 2.06 cm) are suitable for auricular cartilage grafts; fractures >2.4 cm may require a more rigid material. Cartilage grafts that allow failure, however, may better protect the globe in subsequent injury.

Advantages of Early Surgical Management of Periorbital Infantile Hemangiomas.

Background: Early evaluation and treatment of periorbital infantile hemangiomas (POIH) were associated with lower rates of ophthalmological complications. Objective: To evaluate age and characteristics associated with improved anisometropic astigmatism (anisoastigmatism) and eye symmetry measured by diopters and a 5-point scale, respectively, in patients with POIH treated with surgical excision. Methods: A retrospective study was performed on patients with POIH. Patient characteristics and eye symmetry were analyzed between patients with resolved and unresolved anisoastigmatism after surgery. Statistical analyses included the Mann-Whitney U tests, chi-square tests, and linear regression models. Results: In total, 54 patients were included (male: 20, female: 34). Upper medial eyelid was the most commonly affected site (resolved: 45%, unresolved: 43%), followed by upper lateral and upper central. Fifty-six percent (31/55) had postoperative resolution of anisoastigmatism, whereas 44% (24/55) did not. Earlier surgical evaluation (median: 4.5 vs. 6.0 months, p = 0.047) and excision (median: 5.0 vs. 12.0 months, p = 0.005) were associated with reversible anisoastigmatism. Good and suboptimal eye symmetry were not associated with earlier surgical excision (median: 6 vs. 6.5 months, p = 0.87). Follow-up ranged from 1 month to 12 years. Conclusion: Earlier surgical excision was associated with reversing anisoastigmatism but was not significant for improving eye symmetry.

Staged endovascular and surgical treatment of slow-flow vulvar venous malformations.

OBJECTIVE: The objective of the study was to report our experience in a rare series of treated symptomatic slow-flow vulvar venous malformations (VVMs) using a staged, multidisciplinary approach. STUDY DESIGN: Consecutive patients with symptomatic lesions treated over a 7 year period (2005-2012) were followed up for technical success, resolution of symptoms, aesthetic outcomes, and complications. Direct endovenous sclerotherapy (DEVS) using sodium tetradecyl sulfate (STS) foam was performed in all patients under ultrasound and contrast-enhanced fluoroscopic guidance. Surgical excision and layered primary closure was performed within 24 hours after the last DEVS session. RESULTS: Eleven patients (mean age, 25 years; range, 4-43 years) were treated. Presenting symptoms included pain (n = 11), soft tissue swelling (n = 11), local heaviness (n = 11), dyspareunia (n = 2), and dysmenorrhea (n = 2). Most were isolated lesions (n = 8). There were 2 cases of Klippel-Trénaunay syndrome and 1 case of Maffucci syndrome. The latter required Nd:YAG laser photocoagulation prior to sclerotherapy. On average, approximately 3 DEVS sessions were required prior to surgical excision (range, 1-6). Mean estimated surgical blood loss was 130 mL (range, 20-400 mL). Mean follow-up was 23 months (range, 3-55 months). Elimination of pain and soft tissue redundancy was achieved in all patients with satisfactory aesthetic outcomes. All patients experienced minor pain and swelling after DEVS. Following surgical excision, there was 1 case of hematoma and wound dehiscence requiring surgical evacuation. No other reinterventions, endovascular or surgical, were required. CONCLUSION: VVMs require increased awareness and appropriate preoperative evaluation for proper identification and treatment. A multidisciplinary approach can provide improvement in clinical signs and symptoms with satisfactory cosmesis and minimal complications.

Distribution and Clinical Characteristics of Periorbital Infantile Hemangiomas.

Background: Periorbital infantile hemangiomas (POIHs) are associated with a high incidence of visual complications. Objective(s): To analyze the sites of predilection of POIHs and to determine whether certain sites require earlier intervention due to their higher rate of visual complications. Methods: A retrospective case series study was conducted on patients from two tertiary care centers for 25 years. The location of POIHs was determined from clinical photographs, medical records, and radiological studies. The presence or absence of anisometropic astigmatism (anisoastigmatism) and amblyopia was recorded. Data were analyzed using a chi-square test. Results: There were 486 patients, of which 302 patients had ophthalmology evaluations and 245 patients had refractive error data. At presentation, 10% of patients already had amblyopia and 44% had anisoastigmatism. Medial eyelid lesions had the highest risk of developing anisoastigmatism (anisoastigmatism correlates with eyelid position, p = 0.0001). Segmental and upper medial lesions had the highest risk of amblyopia at initial evaluation. Conclusion: The site of POIH is an important indicator for developing clinically significant anisoastigmatism and amblyopia, underlining the need for early ophthalmologic assessment and management.

American society of pediatric otolaryngology vascular anomalies task force review of tongue venous malformations: Techniques, pearls, and pitfalls.

Management of tongue venous malformations can be challenging in the pediatric population due to their heterogeneity in presentation, extent of involvement and functional compromise. It is important to recognize the value of various treatment options in order to guide management of each patient in an individualized fashion. Here we describe a series of patients with tongue venous malformations that are managed using diverse modalities to illustrate the relative benefits and risks of each technique. The challenges of venous malformation treatment can be mitigated by tailoring the approach to each individual patient and malformation. This case series also emphasizes the need and importance of working in the setting of a multidisciplinary vascular anomalies team.

Psychometric Validation of the FACE-Q Craniofacial Module for Facial Nerve Paralysis.

Background: Systematic reviews have identified the need for a patient-reported outcome measure for facial nerve paralysis (FNP). The aim of this study was to determine the psychometric properties of FACE-Q Craniofacial module scales when used in a combined sample of children and older adults with FNP. Methods: Data were collected between December 2016 and December 2019. We conducted qualitative interviews with children and adults with FNP. FACE-Q data were collected from patients aged 8 years and older with FNP. Rasch measurement theory analysis was used to examine the reliability and validity of the relevant scales in the FNP sample. Results: Twenty-five patients provided 2052 qualitative codes related to appearance, physical, psychological, and social function. Many patient concerns were common across age. The field-test sample included 235 patients aged 8-81 years. Of the 13 scales examined, all 122 items had ordered thresholds and good item fit to the Rasch model. For 12 scales, person separation index values were ≥0.79 and Cronbach's alpha values were ≥0.82. The 13th scale's reliability values were ≥0.71. Conclusion: The FACE-Q Craniofacial module scales described in this study can be used to collect and compare evidence-based outcome data from children and adults with FNP.

[Diagnosis of laryngopharyngeal Reflux by Impedance with esophageal pHmetry in children with chronic dysphonia]. / Diagnóstico de Reflujo faringolaríngeo mediante Impedanciometría con pHmetría esofágica en niños con disfonía crónica.

INTRODUCTION: Laryngopharyngeal Reflux (LPR) is the retrograde flow of gastric or duodenal contents into the pharynx and larynx, causing inflammation in the upper aerodigestive tract. Traditionally, a pH monitoring study with an acid reflux index was used. The use of multichannel intraluminal impedance testing with pH monitoring (MII-pH) confirms a causal relationship between suspicious symptoms and LPR. OBJECTIVES: To evaluate LPR diagnosed by MII-pH in the pediatric population consulting due to chronic dysphonia and laryngoscopic findings suggestive of LPR, in addition, to measure the concordance between MII-pH and traditional pH monitoring. PATIENTS AND METHOD: Descriptive, prospective study of patients consulting at the Gastroenterology or Otorhinolaryngology polyclinic due to chronic dysphonia, whose nasofibrolaryngoscopy (NFL) was suggestive of LPR. The patients were hospitalized for a 24-hour MII-pH. Patients with a congenital or acquired morbid history were excluded. Pathological LPR was considered if there were 3 or more acid reflux episodes at the pro ximal level in MII-pH. The frequency of traditional pH monitoring and altered MII-pH and the concordance between both methods were evaluated. RESULTS: 12 patients were recruited, 10 men, 6 to 15 years old. On 9/12, pathological LPR was confirmed by MII-pH, of which 2/9 had traditional pH measurements in normal ranges and 7/9 altered pH measurements. In 3 patients, LPR was ruled out by normal proximal MII-pH. The concordance between MII-pH and traditional pH monitoring was acceptable (kappa 0.4). CONCLUSIONS: 75% of the patients with dysphonia and suggestive NFL showed objective evidence of pathological LPR. Since only with the clinical evaluation, NFL and conventional pH monitoring it is not possible to diagnose LPR, we recommend perform MII-pH for greater diag nostic certainty, avoiding unnecessary treatment, and with unwanted effects in 25% of cases.

Differential expression of SKI oncogene protein in hemangiomas.

OBJECTIVE: The pathogenesis for benign tumorigenesis in hemangiomas is unknown. Oncogene proteins may be influential in this process. SKI proteins have been previously described in various malignancies. We investigated the differential expression of the SKI (sarcoma viral oncogene) protein in hemangiomas. STUDY DESIGN: Prospective basic science study. SUBJECTS AND METHODS: Paraffin-embedded hemangioma tissues were obtained from the senior author from 2005 to 2006. We created the first vascular tissue array composed of 12 hemangioma specimens at various stages of growth and anatomic location. Two cores were taken from each sample. Controls were also included. Immunohistochemical studies were performed using SKI, CD31, and Ki67. RESULTS: All 12 hemangioma tissues overexpressed the SKI protein. The staining pattern was perinuclear within the endothelial cells. The intensity of staining was inversely proportional to the growth stage. The endothelial cells that were SKI-positive were involved in active cell division. CONCLUSION: SKI oncogene protein is differentially and specifically expressed in hemangioma tissues. SKI acts as a transcriptional co-repressor and inhibits the TGF-beta pathway, thus leading to uncontrolled cellular proliferation and transformation. All vascular controls were negative for SKI staining. CLINICAL SIGNIFICANCE OF STUDY: The SKI oncogene protein is upregulated by hemangiomas and may play a role in hemangioma tumorigenesis.

MicroRNA Microarray Profiling in Infantile Hemangiomas.

Objective: MicroRNAs are short, noncoding RNA molecules that negatively regulate the stability and translational efficiency of target mRNAs. They are critical regulators of growth and development. Our aim was to identify microRNAs involved in the growth and regulation of infantile hemangiomas. In addition, we searched for the presence of Piwi-interacting RNAs in hemangioma tissue as another regulator of infantile hemangiomas. Methods: RNA was extracted from hemangioma specimens from 3 clinical, age-based categories: proliferative (N = 16), quiescent (N = 8), and involuting (N = 9). RNAs from human dermal microvascular endothelial cells were used as controls. MicroRNA microarray was performed, and the expression profiles of the hemangiomas and endothelial cells were compared using the t test. 5' End-labeling of RNA of our hemangioma specimens was performed for Piwi-interacting RNA detection. Results: Analysis confirmed statistically significant downregulated (N = 18) and upregulated (N = 15) microRNAs. Piwi-interacting RNA analysis did not detect Piwi-interacting RNA transcripts in the hemangioma specimens. Conclusions: The differential expression of microRNAs found in our hemangioma specimens provides insight into the regulation of hemangioma formation and proliferation, quiescence, and fibrofatty involution. Piwi-interacting RNA transcripts were not detected in the hemangioma specimens. These novel findings will help in establishing new therapeutic and diagnostic initiatives for these tumors.

Medical Management of Acute Facial Paralysis.

Acute facial paralysis (FP) describes acute onset of partial or complete weakness of the facial muscles innervated by the facial nerve. Acute FP occurs within a few hours to days. The differential diagnosis is broad; however, the most common cause is viral-associated Bell Palsy. A comprehensive history and physical examination are essential in arriving at a diagnosis. Medical treatment for acute FP depends on the specific diagnosis; however, corticosteroids and antiviral medications are the cornerstone of therapy. Lack of recovery after 4 months should prompt further diagnostic workup.

Multidisciplinary Approach to the Management of Lymphatic Malformations of the Head and Neck.

Lymphatic malformations (LMs) occur in 2.8 to 5 per 100,000 live births. Most involve the head and neck and they are equally common in men and women. They are developmental anomalies of unknown cause, although recent evidence suggests that an upregulation of the mammalian target of rapamycin (mTOR) pathway may be a causal factor leading to the overproduction of abnormal lymph vessels. These vessels are likely dilated lymphatic sacs sequestered from the lymphatic and venous systems. This overproduction results in the accumulation of lymph in dilated cystic spaces, which in turn results in the clinical features of an LM.

The Management of Vascular Malformations of the Airway: Natural History, Investigations, Medical, Surgical and Radiological Management.

Vascular malformations may affect nearly all aspects of the upper airway. Each type of malformation has a characteristic pattern of disease. These lesions may be focal or diffuse, and require directed management strategies. Physicians treating these entities should have a high level of suspicion to consider airway evaluation even in the absence of overt symptoms. However, cutaneous head and neck venous malformations or other lesions affecting the lips, oral cavity, or tongue can herald the presence of coexisting airway lesions. A multidisciplinary approach is critical in achieving comprehensive treatment.

Management of Long-Standing Flaccid Facial Palsy: Static Approaches to the Brow, Midface, and Lower Lip.

Chronic flaccid facial paralysis (FFP>2 years) may be approached with static and dynamic techniques. A horizontal zonal assessment evaluates the upper, middle, and lower thirds of the face. Surgery is tailored to an individual's deficits, goals, and health status. While dynamic reanimation is the gold standard for rehabilitation, there are cases in which static approaches are more appropriate or may be used as an adjunct to dynamic techniques. This article focuses on the surgical management of FFP primarily using static approaches to the individual zones of the face to create resting symmetry.