Sirolimus for vascular anomalies in the first year of life: a systematic review.

Evidence on effectiveness and safety of sirolimus in congenital vascular anomalies in infancy is lacking. We aim to systematically review the efficacy and safety of sirolimus in treating congenital VA in infancy. We searched for and included all studies evaluating sirolimus for VA in the first year of life. The primary outcome was effectiveness. The secondary outcome was safety. We included 84 case series and reports (172 participants). Sirolimus decreased the size of the VA in >50% of participants, most of whom had minor transient side effects, and 27% had no adverse effects at all. When categorized by age (<1 month, 1-5 months and 6-12 months), the effectiveness was similar in all age groups. Available evidence suggests that sirolimus is effective and well tolerated. The effectiveness of sirolimus should be evaluated in a well-designed randomized controlled or observational studies.

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.

No Association of Sirolimus with Wound Complications in Children With Vascular Anomalies.

INTRODUCTION: Sirolimus has demonstrated effectiveness as a treatment option for several types of vascular anomalies; however, it has a potential side effect of delayed surgical wound healing. The purpose of this study was to evaluate the association of sirolimus with postoperative complications in the pediatric vascular anomaly population. METHODS: A retrospective cohort study was performed for children with a vascular anomaly who underwent excision or debulking of the anomaly from 2015 to 2020. Patient demographics, vascular anomaly characteristics, operative variables, sirolimus dosing information, and perioperative outcomes were collected. Univariate analysis was performed to compare outcomes based on the administration of sirolimus. RESULTS: Forty-seven patients with vascular anomalies underwent 57 surgical procedures (36 without perioperative sirolimus, 21 with perioperative sirolimus). The median age at the time of surgery was seven years (IQR 1.7-14.0). The most common anomalies were lymphatic and venolymphatic malformations. Of the patients administered perioperative sirolimus, the median preoperative and postoperative sirolimus levels were comparable (preoperative 6.9 ng/mL (IQR 4.9-10.1), postoperative 6.5 ng/mL (IQR 4.7-9.4)). The rate of postoperative complications (sirolimus 19%, without sirolimus 11%; p = 0.45) and wound complications (sirolimus 14%, without sirolimus 6%; p = 0.26) were comparable between the cohorts. CONCLUSION: Our results suggest sirolimus may not significantly increase perioperative complication rates in pediatric patients undergoing resection of their vascular anomaly. LEVEL OF EVIDENCE: Level III.

Update on Vascular Anomalies of the Head and Neck.

Vascular anomalies of the head and neck is an evolving field, with more recent focus on identifying and understanding the underlying genetic and molecular causes for these lesions. Medical therapies for some of these vascular anomalies have been developed. Many complex vascular anomalies require multimodality therapy, and other lesions could be treated with any of a variety of the available therapies. High-quality outcomes research and establishing clinical practice guidelines to help guide management are essential.

Utilization of Alexandrite Laser for Hair Removal in Pediatric Auricular Reconstruction.

Auricular reconstruction with autologous rib cartilage involves using a soft tissue envelope to cover the cartilage framework. In patients with a low hairline, hair-bearing skin may be incorporated on the reconstructed ear, creating a difficult and conspicuous aesthetic problem. A retrospective chart review was conducted to summarize and share the experience of using the Candela GentleMax Pro 755-nm alexandrite laser system (Candela Corp) in children following auricular reconstruction. Nine patients received laser hair removal via the alexandrite laser system with good results. The number of completed sessions ranged from 1 to 5. The procedures were completed without the need for premedication or procedural sedation. Laser hair removal with an alexandrite laser system was safe, fast, and effective. With multistage auricular reconstruction, it was feasible to incorporate laser hair removal between the stages.

Adenoidectomy and persistent velopharyngeal insufficiency: Considerations, risk factors, and treatment.

IMPORTANCE: Persistent velopharyngeal insufficiency (VPI) is a rare but well-recognized complication of adenoidectomy. VPI can have a significant impact on the communication of a child. OBJECTIVE: To describe the pathophysiology of post-adenoidectomy VPI, identify its associated risk factors, and illustrate the techniques used to treat the entity. EVIDENCE REVIEW: A search of English or translated English articles concerning adenoidectomy, partial adenoidectomy, superior adenoidectomy; and velopharyngeal insufficiency, speech and voice from 1980 to 2021 was performed using Pubmed and Embase. Data from prospective and retrospective studies and their relevant references were pooled. RESULTS: By objective measures, hypernasality is noted in many pediatric patients post-adenoidectomy, but this typically resolves in 3-6 months. Risk factors for the development of post-adenoidectomy VPI include low birth weight, family history of hypernasality, and history of speech problems or nasal regurgitation. The cleft palate, submucous cleft palate, poor palate mobility, and a deep pharynx may indicate susceptibility to VPI. Speech therapy is successful in up to 50% of patients, while surgical intervention may be tailored based on the diagnostic evaluation of the velopharynx with videofluoroscopy or nasoendoscopy. CONCLUSION: We present a comprehensive review of the literature on the pathophysiology, risk factors, and treatment of post-adenoidectomy VPI. We hope to bring awareness to the factors that can lead to a rare but potentially devastating complication in one of the most common procedures performed by Otolaryngologists.

The importance of sublingual gland removal in treatment of ranulas: A large retrospective study.

PURPOSE: Investigate the efficacy of sublingual gland removal for the treatment of simple and plunging ranulas. MATERIALS AND METHODS: After IRB approval, a retrospective review was performed on patients treated for a ranula from February 2013 to May 2018 at Texas Children's Hospital in Houston, TX. Clinical data was collected from medical records and patients were contacted in November 2018 to obtain additional information about recurrences or complications. RESULTS: This study included 52 patients with ranulas (10 plunging, 42 simple; M:F 21:31) with a mean age of 9.68 years. Eighteen patients provided information in the extended follow-up period. Simple ranulas were treated with intraoral excision of the cyst and the sublingual gland (27 cases), marsupialization (7 cases), intraoral excision of the cyst alone (7 cases), and intraoral excision of the cystic component and subsequent marsupialization after recurrence (1 case); intraoral excision of the sublingual gland was not associated with any recurrence. Plunging ranulas were treated with intraoral excision of the cyst and/or sublingual gland (7 cases) or with a transcervical approach (3 cases). One patient was initially treated with sclerotherapy before undergoing intraoral excision of the sublingual gland. Two patients treated with transcervical excision of the cyst experienced recurrence compared to no recurrence with intraoral excision of the sublingual gland. CONCLUSION: Intraoral removal of the sublingual gland is the most effective treatment for both simple and plunging ranulas. Plunging ranulas must be considered in patients presenting with a submandibular and submental cystic mass given intraoral extension may not be apparent.

Arteriovenous Malformations of the Head and Neck.

Extracranial arteriovenous malformations (AVMs) are complex vascular malformations to diagnose and treat. They are comprised of congenitally derived arteriovenous shunts with chronic vascular expansion, collateralization, and infiltration of local tissue. Their cause remains unclear, but new genetic and molecular clues are emerging. They may present at any age following an early quiescent period. Diagnosis is based on vascular staining, soft tissue expansion, progressive growth, warmth, and pulsations. Focal lesions can be cured, whereas diffuse AVMs demonstrate highly recidivistic disease. Multimodal therapy with staged interventions can improve treatment outcomes, increase treatment intervals, and control disease. Vigilant follow-up is critical.

The reliability of flexible nasolaryngoscopy in the identification of vocal fold movement impairment in young infants.

OBJECTIVE: Flexible nasolaryngoscopy (FNL) is considered the gold standard for evaluation of vocal fold mobility but there has been no data on the reliability of interpretation in the infant population. Visualization may be limited by excessive movement, secretions, or floppy supraglottic structures that prevent accurate diagnosis of vocal fold movement impairment (VFMI). We sought to evaluate the inter- and intra-rater reliability of FNL for the evaluation of VFMI in young infants. STUDY TYPE: Case-control. METHODS: Twenty infants were identified: 10 with VFMI and 10 normal as seen on FNL. Three pediatric otolaryngologists reviewed the video without sound and rated the presence and/or degree of vocal fold mobility. Twelve videos were repeated to assess intra-rater reliability. RESULTS: There was substantial agreement between the reviewers regarding the identification normal vs. any type of VFMI (kappa = 0.67) but only moderate agreement regarding the degree of vocal fold movement (kappa = 0.49). Intra-rater reliability ranges from moderate to perfect agreement (kappa = 0.48-1). CONCLUSION: FNL in infants is an extremely challenging procedure. Clinically, physicians frequently use the quality of the cry and the past medical and surgical history to help make a judgment of vocal fold movement when the view is suboptimal. These other factors, however, may bias the interpretation of the FNL. Without sound, there is only moderate inter-rater and variable intra-rater reliability for the identification of degree of movement on FNL. Otolaryngologists must be cognizant of the limitations of FNL when using it as a clinical tool or as a "gold standard" against which other modalities are measured.

Pediatric cervical lymphadenopathy.

This article provides an overview for evaluation and management of the pediatric patient with cervical lymphadenopathy. A thorough history and physical examination are crucial in developing a differential diagnosis for these patients. Although infectious causes of lymphadenopathy are more prevalent in the pediatric population compared with adults, neoplasms should also be considered. Judicious use of imaging studies, namely ultrasound, can provide valuable information for accurate diagnosis. Common and uncommon infectious causes of cervical lymphadenopathy are reviewed. Surgical intervention is occasionally necessary for diagnosis and treatment of infections, and rarely indicated for the possibility of malignancy. Indications for surgery are discussed.

Section for residents and fellows-in-training survey results.

OBJECTIVES: (1) To present data from the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) Section for Residents and Fellows-in-Training (SRF) annual survey from 2002 to 2011. (2) To compare and analyze trends in resident demographics, residency experiences, and post-training career choices. STUDY DESIGN: Review of cross-sectional survey data. SETTING: Residents and Fellows registered as Members-in-Training through AAO-HNS. METHODS: A review of data from surveys distributed between 2002 and 2011 was conducted. Respondent demographic data including age, postgraduate year, gender, and geographic distribution were analyzed. Responses about training experiences, fellowship selection, debt burden, and post-training practice choice were studied in order to elicit trends. RESULTS: Respondents have consistently rated otolaryngology, anesthesia, and trauma/critical care as the most important intern rotations for otolaryngology residents. Each year, approximately 70% of respondents have reported interest in pursuing a fellowship. Pediatric otolaryngology fellowship is now the most popular fellowship among respondents. There has been a recent increase in the percentage of respondents who are interested in pursuing a career in academics. Location, family, and lifestyle have consistently been the most important factors in determining choice of practice. Respondents have reported increasing levels of educational debt. CONCLUSION: The AAO-HNS SRF survey collects important data regarding resident/fellow training. Several factors limit the generalizability of these results. Despite its limitations, these unique data provide valuable information for continual evaluation and improvement of physician training in the specialty.

Extremes in otolaryngology resident surgical case numbers.

OBJECTIVE: Understand (1) significance between minimum, mean, and maximum case numbers for otolaryngology resident surgical procedures; (2) weaknesses of Accreditation Council for Graduate Medical Education (ACGME) resident case log system contributing to inaccurate data/case inflation; and (3) when excessive case load may transform education into service. STUDY DESIGN: Cross-sectional survey using a national database. SETTING: Academic otolaryngology residency programs. SUBJECTS/METHODS: Review of otolaryngology resident national data reports from ACGME resident case log system performed from 2004 to 2010. Minimum, mean, standard deviation, and maximum values for total number of resident surgeon cases and for specific surgical procedures were compared. Case unbundling, resident data entry habits, and tracked vs untracked Current Procedural Terminology (CPT) codes were reviewed. RESULTS: Mean total number of resident surgeon cases remained constant (1699.5 ± 424.2 to 1772.2 ± 517). Minimum total number of cases ranged from 730 to 811 (approaching 2 standard deviations below the mean). Zero was reported as the minimum case number for some procedures. Maximum for total number of cases increased (3559 to 4857) and surpassed the mean by 4 to 5 standard deviations. Some procedures have maximums greater than 11 standard deviations above the mean. The authors identified untracked CPT codes and variations in resident case log documentation habits. CONCLUSIONS: Large differences between the minimum, mean, and maximum resident surgeon case numbers exist. Establishing minimum case number requirements for otolaryngology residents should be considered. Educational benefit derived from excessive case load is unclear. Critical examination of the ACGME resident case log system and resident documentation habits is needed to improve accuracy of reporting.

Evaluating the adult patient with a neck mass.

The objective of this article is to provide the internist with general considerations when confronted with an adult patient presenting with a neck mass. A thorough gathering of historical information and a complete physical examination are crucial in developing a differential diagnosis for these patients. Specifically, the location of the mass, its time of onset, and duration are important because of the high likelihood of neoplastic processes in patients older than 40 years. The young adult patient has an increased incidence of inflammatory, congenital, and traumatic processes as causes of their neck mass, but again, neoplasms are not out of the realm of possibility. Judicious use of imaging studies, namely computed tomography scanning with contrast, is a valuable adjunct to the physical examination. Other than infectious etiology, referral to an otolaryngologist is frequently warranted to obtain a definitive diagnosis for the development of an appropriate treatment plan, which is predominantly surgical.

Cell density of the lamina propria of neonatal vocal folds.

OBJECTIVES: We undertook (1) to measure the cell density within the lamina propria of the neonatal vocal folds and (2) to examine changes in cell density in the lamina propria with increasing gestational age of the neonatal vocal folds. METHODS: Intact neonatal larynges were obtained from fresh cadaveric specimens. Hematoxylin and eosin-stained slides were used to visualize the laryngeal structures, and photomicrographs of the vocal folds were taken at 100x magnification. The cell density of the lamina propria was calculated by counting the cells within each of five 100-microm2 regions within the study area, and the totals were then averaged for each area. RESULTS: A total of 62 sections from 14 larynges with gestational ages of 19 to 36 weeks were examined. Histologic analysis revealed a uniform appearance of the vocal fold without apparent layers. The cell density of the lamina propria was 30 or more cells per 100 microm2 for 51.2% of larynges with less than 27 weeks of gestation. However, only 14.3% of the larynges with 27 or more weeks of gestation had an average cell density of 30 or more cells per region (p < 0.005). CONCLUSIONS: As described by previous studies, the lamina propria of the neonatal vocal folds is a hypercellular monolayer. The process of vocal fold maturation appears to occur earlier than previously thought, with decreasing cell density in the lamina propria by 27 weeks' gestation.