Comparing Feedback Techniques in Bilobe Flap Simulation Using 3D-Printed Facial Models.

Objective: To compare live versus delayed feedback on trainee performance of bilobe flaps using 3-dimensional (3D)-printed facial simulators and determine whether these effects are sustained on repeat performance. Study Design: Cohort study. Setting: University of Arkansas for Medical Sciences. Methods: 3D-printed facial models with a nasal ala defect were provided to 18 subjects. Subjects were stratified and randomized based on their training level into 1 of 3 groups corresponding to live feedback (Group 1), delayed feedback (Group 2), and no feedback (Group 3). Subjects performed a bilobe flap following a structured lecture. Four weeks later, subjects independently repeated the exercise on the contralateral ala. Likert surveys were used to assess subjective parameters. Objective grading was performed by a plastic surgeon, which included a point system and score for the overall appearance. Results: Following exercise 1, Group 1 reported a significant improvement in knowledge (P < .001), which was sustained after exercise 2 (P < .001); Group 2 reported a significant improvement after exercise 1 (P = .03) but was not sustained (P = .435). After the second exercise, Group 1 and Group 2 improved their confidence in bilobed repair (P = .001 and P = .003, respectively), but this was greater for Group 1. Group 1 showed a significant improvement in their design time following exercise 2 (P = .007). There were no significant differences between groups on total time for repair, total score, and appearance. Conclusion: 3D-printed models are valuable in teaching the bilobe flap for nasal defects, with live feedback providing the greatest level of improvement in self-reported knowledge and confidence.

Intraosseous resection of mandibular arteriovenous malformations: A mandible sparing multi-disciplinary case series.

Mandibular arteriovenous malformations (AVMs) are high flow vascular malformations that can cause pain, hypertrophy, deformity, malocclusion, jaw asymmetry, bone destruction, tooth loss, and severe bleeding [1]. Although general principles apply, the rarity of mandibular AVMs limits definitive agreement on the best course of treatment. Current treatment options include embolization, sclerotherapy, surgical resection, or some combination of techniques [2]. [3]. An alternative multidisciplinary technique of embolization with mandibular-sparing resection is presented. This technique aims to mitigate bleeding with effective removal of the AVM, while preserving mandibular form, function, dentition, and occlusion.

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.

Impact of Oral Steroids on Tonsillectomy Postoperative Complications and Pain.

OBJECTIVES: To analyze the impact of steroids on postoperative tonsillectomy recovery and implement findings for improvement in postoperative management. METHODS: Institutional review board approved prospective study with retrospective analysis of private practice setting tonsillectomy patients (November 2015 to January 2017). A questionnaire was provided postoperatively to patients undergoing tonsillectomy with or without adenoidectomy. The study population was separated into 2 groups: patients who received steroids (3 days of either dexamethasone or prednisolone), postoperative steroid (POS), versus patients who did not receive steroids (PONS). RESULTS: The questionnaire had a return rate of 27.3% (254/931). Nine of the 254 responses were disqualified for lack of information; therefore, the total number of responses was 245. Of these, 115 were POS and 130 were PONS. The groups were similar in mean age (POS: 13.2 ± 10.4 years, PONS: 14.7 ± 12.1 years, P = .32) and sex (POS: Male 40.0%, PONS: Male 40.0%, P = .97). There was an overall decrease of pain and nausea/vomiting (N/V) in the steroid group (P = .0007). There was reduction in pain (P < .05) from postoperative day (POD) 2, 3, 4, and 6 in the POS group. Otherwise, there was no significant reduction in pain from POD 7 to 14, day-by-day rate of N/V, bleeding, or rate of emergency department (ED) or clinic visit (P > .05). CONCLUSION: Postoperative steroid reduced overall pain and N/V, as well as daily pain on POD 2, 3, 4, and 6. Pain from POD 7 to 14, rate of ED or clinic visit, or daily N/V and bleeding rate were not significantly different between cohorts.

Blue Rubber Bleb Nevus Syndrome: A Rare Case of Gastrointestinal Hemorrhage Necessitating Bowel Resection.

Blue Rubber Bleb Nevus Syndrome is a congenital rarity that manifests as vascular malformations throughout the body, including the gastrointestinal tract. With fewer than 300 cases reported, the etiology and clinical course is poorly understood; however, the literature suggests TEK mutations on chromosome 9 result in unregulated angiogenesis. We present the case of a young female treated for anemia of unknown etiology who presented in hemorrhagic shock due to gastrointestinal hemorrhage necessitating small bowel resection, with cutaneous, intestinal, hepatic, and lingual vascular malformations associated with a single somatic pathologic TEK mutation. Although uncommon, this case suggests that Blue Rubber Bleb Nevus Syndrome should be considered in the differential of a patient with persistent anemia and cutaneous lesions, carrying the potential for multiple gastrointestinal vascular malformations progressing to hemorrhage necessitating operative management. Additionally, a severe phenotype can occur without a double-hit TEK mutation.

Characterizing Pediatric Bilateral Vocal Fold Dysfunction: Analysis with the Pediatric Health Information System Database.

OBJECTIVES: The purpose of this study was to characterize pediatric bilateral vocal fold dysfunction and to examine the overall inpatient mortality. METHODS: Retrospective cohort analysis. Data from the Pediatric Health Information System was gathered for all pediatric patients with a diagnosis of bilateral vocal fold dysfunction between January 2008 and September 2020. Univariate and multivariate analyses were performed using Cox proportional hazard models. RESULTS: 2395 patients accounted for 4799 hospitalizations with bilateral vocal fold dysfunction. Inpatient mortality occurred in 2.9% of the study sample. Chiari 2 was found in 2.8% of patients. The most common associated diagnoses were related to comorbid respiratory conditions (61.1%). The median adjusted ratio of cost to charges was $76,569. Aspiration was noted in 28 patients (1.2%). Gastrostomy was performed in 607 patients (25.3%). Tracheostomy was performed in 27% of patients. The overall 90-day readmission rate was 61%. On multivariate analysis, prognostic factors associated with increased hospital survival include gastrointestinal comorbidities (hazard ratio [HR]: 0.29; 95% confidence interval [CI]: 0.18-0.49) and tracheostomy (HR: 0.21; 95% CI: 0.12-0.37). CONCLUSION: This database study represents the largest cohort analysis to date characterizing bilateral vocal fold dysfunction. Favorable prognostic indicators of overall hospital survival include gastrointestinal comorbidities and the presence of tracheostomy. Tracheostomy is associated with an increase in hospital costs, comorbidities, gastrostomy tube placement, and Chiari diagnosis. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:1228-1233, 2023.

Extracranial arteriovenous malformations demonstrate dysregulated TGF-ß/BMP signaling and increased circulating TGF-ß1.

Extracranial arteriovenous malformations (AVMs) are characterized by anomalous arterial-to-venous connections, aberrant angiogenesis, local inflammation and hypoxia, and disorganized histological architecture; however, the precise molecular perturbations leading to this phenotype remain elusive. We hypothesized that extracranial AVM tissue would demonstrate deregulation of the TGF-ß/BMP signaling pathway, which may serve as a potential target in the development of molecular-based therapies for AVMs. AVM tissue was harvested during resection from 10 patients with AVMs and compared to control tissue. Blood was collected from 14 AVM patients and 10 patients without AVMs as controls. Expression of TGF-ß/BMP pathway components was analyzed using RT-PCR, western blotting, and immunohistochemistry. Circulating levels of TGF-ß1 were analyzed by ELISA. Paired t tests were utilized to perform statistical analysis. The mRNA levels of TGF-ß1, ALK1, Endoglin (ENG), Smad6, Smad7, and Smad8 were significantly elevated in AVM tissue when compared to controls. Protein levels of TGF-ß1 and Smad3 were elevated in AVM tissue while protein levels of BMP-9, ALK1, Smad1, Smad6, and Smad8 were significantly decreased in AVMs. Immunohistochemistry demonstrated increased TGF-ß1 in the perivascular cells of AVMs compared to normal controls, and circulating levels of TGF-ß1 were significantly higher in AVM patients. Patients with AVMs demonstrate aberrant TGF-ß/BMP expression in AVM tissue and blood compared to controls. Targeting aberrantly expressed components of the TGF-ß/BMP pathway in extracranial AVMs may be a viable approach in the development of novel molecular therapies, and monitoring circulating TGF-ß1 levels may be a useful indicator of treatment success.

Laser Treatment of Vascular Anomalies.

Lasers are a safe and effective tool for the treatment of vascular anomalies. There are many laser options available. Matching laser parameters with the characteristics of the vasculature in these lesions can selectively deliver energy to the abnormal tissue. This can lead to reduction in size and symptoms of vascular malformations and hemangiomas.

Infantile Hemangioma Treated with Propranolol Readmission Trends, Complications of Therapy, and Cost: A PHIS Database Study.

Objective: To examine admission trends, complications, and costs for inpatient infantile hemangioma (IH) associated with propranolol therapy utilizing the Pediatric Health Information System (PHIS) database. Study Design. A retrospective cohort study was completed using the PHIS database. The PHIS database was queried from 2008 to 2020 for children without cardiac disease and between the ages of three weeks and one year who were admitted with a diagnosis of IH and administered propranolol. Admissions were trended annually and by geographic region. Primary outcomes were length of stay (LOS), readmission, mortality, propranolol-related complications, and costs. Bivariate and multivariable analyses were employed to identify predictors of the primary outcomes. Results: A total of 2290 unique patient encounters were identified. Admissions steadily decreased after 2011, with variations by geographic region. There was no mortality and only 60 (2.6%) propranolol-related complications. African-American race (odds ratio (OR) 1.20 [95% CI: 1.02-1.41]), respiratory comorbidities (OR 2.04 [95% CI: 1.42-2.93]), neurologic conditions (OR 1.34 [95% CI: 1.09-1.59]), admission to an intensive care unit (OR 1.31 [95% CI: 1.09-1.59]), bronchospasm (OR 1.37 [95% CI: 1.22-1.55]), and hyperkalemia (OR 1.86 [95% CI: 1.08-3.20]) were associated with increased LOS. Neurologic conditions (OR 2.87 [95% CI: 1.76-4.67]) and respiratory comorbidities (OR 2.48 [CI: 1.43-4.30]) were associated with readmission. Average cost per admission was $5,158 ($3,259 to $8,560 range). Conclusion: There is an overall national decline in rate of admissions for IH propranolol therapy. Inpatient admission may be beneficial for patients with neurologic or respiratory conditions.

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.

Pharmacokinetics of bleomycin sclerotherapy in patients with vascular malformations.

Bleomycin, a chemotherapy agent that inhibits synthesis of DNA, has been increasingly utilized in sclerotherapy for patients with vascular malformations. A serious long-term risk of intravenous bleomycin is dose-dependent interstitial pneumonitis. Little is known about absorption and circulating levels of bleomycin when used in sclerotherapy for patients with vascular malformations. This is an Institutional Review Board (IRB)-approved prospective study on patients receiving bleomycin sclerotherapy in the management of vascular malformations. Depending on the type of vascular malformation, bleomycin was administered either in the lumen or interstitial space of the involved lesion. A bleomycin assay measured serum bleomycin plasma concentrations versus time at seven intervals following treatment. Pharmacokinetic parameters were obtained for each participant and included peak plasma concentration (Cmax ), time to reach peak plasma concentration (Tmax ), volume of distribution (Vd ), elimination half-life (t1/2 ), the volume of plasma cleared of the drug per unit time (CL), and total systemic exposure area under the curve (AUC). Fifteen patients were enrolled (5: lymphatic, 4: venous, 6: arteriovenous malformations). Bleomycin was administered interstitially (IS) in 11 patients and intraluminal (IL) in four; median age of 13 years (range: 2-67). Pharmacokinetic analysis revealed terminal elimination half-life (t1/2λz ) of 88.51 (±23.09) and 111.61 (±37.75) minutes for the IS and IL groups, respectively. Vd was 4.86 L (±6.74) and 1.55 L (±0.54) for the IS and IL groups, respectively. AUC was 53.9 (±23.45) and 129.17 (±93.57) mg min/L for the IS and IL groups, respectively. There were no statistically significant differences in t1/2λz , Vd , or AUC parameters between groups. Bleomycin is absorbed systemically when used as a sclerosant for vascular malformations when injected either IS or IL.

Autonomous diagnosis of pediatric cutaneous vascular anomalies using a convolutional neural network.

OBJECTIVES: Design and validate a novel handheld device for the autonomous diagnosis of pediatric vascular anomalies using a convolutional neural network (CNN). STUDY DESIGN: Retrospective, cross-sectional study of medical images. Computer aided design and 3D printed manufacturing. METHODS: We obtained a series of head and neck vascular anomaly images in pediatric patients from the database maintained in a large multidisciplinary vascular anomalies clinic. The database was supplemented with additional images from the internet. Four diagnostic classes were recognized in the dataset - infantile hemangioma, capillary malformation, venous malformation, and arterio-venous malformation. Our group designed and implemented a convolutional neural network to recognize the four classes of vascular anomalies as well as a fifth class consisting of none of the vascular anomalies. The system was based on the Inception-Resnet neural network using transfer learning. For deployment, we designed and built a compact, handheld device including a central processing unit, display subsystems, and control electronics. The device focuses upon and autonomously classifies pediatric vascular lesions. RESULTS: The multiclass system distinguished the diagnostic categories with an overall accuracy of 84%. The inclusion of lesion metadata improved overall accuracy to 94%. Sensitivity ranged from 88% (venous malformation) to 100% (arterio-venous malformation and capillary malformation). CONCLUSIONS: An easily deployed handheld device to autonomously diagnose pediatric skin lesions is feasible. Large training datasets and novel neural network architectures will be required for successful implementation.

Cutaneous hyperpigmentation following bleomycin sclerotherapy for vascular malformations.

Systemic bleomycin therapy is associated with pulmonary fibrosis and cutaneous side effects. While it is believed that there is little to no systemic distribution of bleomycin when utilized to treat vascular malformations (VMs), we present a case series in which cutaneous, adhesive-related hyperpigmentation suggests that there is systemic egress of bleomycin following direct puncture sclerotherapy (DPS). This risk of hyperpigmentation after intralesional bleomycin should be discussed with patients, and steps to minimize the chances of it occurring should be implemented.

Utilizing process improvement strategies to generate clinic templates and improve patient flow in pediatric otolaryngology.

OBJECTIVES: To examine outcomes from process improvement strategies aimed to: 1) develop computer generated physician clinic templates using captured and historic clinical data, and, 2) introduce said new template designs while maintaining historic daily patient volumes. METHODS: An Institutional Review Board approved retrospective review of time stamped data collection in a tertiary facility pediatric otolaryngology clinic. RESULTS: A discrete-event simulation was built from timestamps associated with clinic interaction milestones. The data were analyzed to develop standard clinic templates with the goal to reduce patient overall visit length by 10%. A total of 12,052 clinic visits were analyzed, 8,045 before (avg. of 62.9 visits/day) and 4,007 after (avg. of 65.7 visits/day) template standardization. The change led to a 10.5% (5.5 min, p < 0.001) decrease in total clinic visit time from 52.3 ± 25.9 min to 46.8 ± 25.0 min. This data extrapolated over a year is estimated to save 1,567 clinic hours. Secondarily, it was found that patient experience was not affected as a result of this change. CONCLUSION: Discrete-event simulation, using the principles of process improvement, is effective in guiding clinic operational redesign. This quality improvement project decreased the average length of clinic visit by 10% with no impact on historic high clinic volumes. Patient flow can improve in high volume pediatric otolaryngology practices by using process improvement strategies and discrete-event simulations to create standardized provider templates. Theoretically, this strategy can lead to improved patient and physician experiences along with an increase in patient visits over time.

Venous malformation vessels are improperly specified and hyperproliferative.

Venous malformations (VMs) are slow-flow malformations of the venous vasculature and are the most common type of vascular malformation with a prevalence of 1%. Germline and somatic mutations have been shown to contribute to VM pathogenesis, but how these mutations affect VM pathobiology is not well understood. The goal of this study was to characterize VM endothelial and mural cell expression by performing a comprehensive expression analysis of VM vasculature. VM specimens (n = 16) were stained for pan-endothelial, arterial, venous, and endothelial progenitor cell proteins; proliferation was assessed with KI67. Endothelial cells in the VM vessels were abnormally orientated and improperly specified, as seen by the misexpression of both arterial and endothelial cell progenitor proteins not observed in control vessels. Consistent with arterialization of the endothelial cells, VM vessels were often surrounded by multiple layers of disorganized mural cells. VM endothelium also had a significant increase in proliferative endothelial cells, which may contribute to the dilated channels seen in VMs. Together the expression analysis indicates that the VM endothelium is misspecified and hyperproliferative, suggesting that VMs are biologically active lesions, consistent with clinical observations of VM progression over time.

Presence of estrogen and progesterone receptors in proliferating and involuting infantile hemangiomas.

BACKGROUND: Studies in the literature have demonstrated the presence of sex hormone receptors in infantile hemangiomas (IHs), but further investigation is needed to determine the role of these receptors in their proliferation and involution. To date, there are no studies in the literature that aimed to quantitatively examine the expression of sex hormone receptors throughout the different phases of hemangioma development. OBJECTIVE: The objective of our study was to quantitatively evaluate the expression of estrogen (ER) and progesterone (PR) receptors in the proliferative and involuting phases of IHs through the use of real-time polymerase chain reaction (RT-PCR). METHODS: Twenty IHs (10 proliferating and 10 involuting) were harvested and prepared for molecular investigation. ER receptor alpha (ERα) and beta (ERß) and the PR expression were examined by RT-PCR and western blot. RESULTS: RT-PCR analysis demonstrated that mRNA expression of ERα, ERß, and PR was significantly lower in proliferating versus involuting IH. Western blot analysis revealed increased protein expression of ERα in involuting hemangiomas as compared to proliferating ones. CONCLUSIONS: Our study demonstrates the variable expression of ER and PR receptors in proliferating and involuting hemangiomas. Further studies are needed to determine the exact role of these hormone receptors in the growth and involution of IHs.

Propranolol for Treatment of Infantile Hemangioma: Efficacy and Effect on Pediatric Growth and Development.

PURPOSE: Propranolol has been successful in treating problematic infantile hemangiomas (IH) but concerns regarding its effect on normal growth and development have been raised. This study examines physical growth, developmental milestones, and human growth hormone (hGH) levels in infants receiving propranolol for problematic IH. METHOD: Monthly heights and weights of children undergoing propranolol therapy for IH were prospectively collected and tabulated. Data analysis and comparison to World Health Organization (WHO) weight-for-age and weight-to-length z-scores was performed. Questionnaires regarding milestones, efficacy, and guardian satisfaction were performed, and a combination of both chart results and phone conducted surveys was tabulated. Serum from a small representative cohort of age-matched children with IH treated and not treated with propranolol was collected. RESULTS: A total of 185 children receiving propranolol therapy between 2008 and 2013 for IH were assigned to this study. The children were divided into two cohorts based on the presence of comorbidities or risk factors that may affect growth and development (n = 142 no comorbidities, n = 43 with comorbidities). Neither cohort demonstrated deviation from normal weight in comparison to WHO normative data. There was a significant deviation for BMI-for-age and weight-for-age z-scores in our population, especially in patients on propranolol for more than 7 months. Based on data from participants, via either completed questionnaires or chart results, most children met their developmental milestones at or before target ages, regardless of the presence of comorbidities. Eighty percent of guardians noticed clinical improvement of the IH, with 91% either happy about or neutral to using the medication. hGH levels were higher in patients receiving propranolol therapy, but not significantly different. CONCLUSION: Propranolol therapy is effective and well tolerated in the treatment of infantile hemangiomas. This study suggests that propranolol does not impair growth and has no impact on normal pediatric development.

Update on Pediatric Tracheostomy: Indications, Technique, Education, and Decannulation.

PURPOSE OF REVIEW: Tracheostomy in a child demands critical pre-operative evaluation, deliberate family education, competent surgical technique, and multidisciplinary post-operative care. The goals of pediatric tracheostomy are to establish a safe airway, optimize ventilation, and expedite discharge. Herein we provide an update regarding timing, surgical technique, complications, and decannulation, focusing on a longitudinal approach to pediatric tracheostomy care. RECENT FINDINGS: Pediatric tracheostomy is performed in approximately 0.2% of inpatient stays among tertiary pediatric hospitals. Mortality in children with tracheostomies ranges from 10-20% due to significant comorbidities in this population. Tracheostomy-specific mortality and complications are now rare. Recent global initiatives have aimed to optimize decision-making, lower surgical costs, reduce the length of intensive care, and eliminate perioperative wound complications. The safest road to tracheostomy decannulation in children remains to be both patient and provider dependent. SUMMARY: Recent literature provides guidance on safe, uncomplicated, and long-term tracheostomy care in children. Further research is needed to help standardize decannulation protocols.