Preoperative Imaging and Surgical Findings in Pediatric Frontonasal Dermoids.

OBJECTIVE: To review cases of congenital frontonasal dermoids to gain insight into the accuracy of preoperative computed tomography (CT) and magnetic resonance imaging (MRI) in predicting intracranial extension. METHODS: This retrospective study included all patients who underwent primary excision of frontonasal dermoids at an academic children's hospital over a 23-year period. Preoperative presentation, imaging, and operative findings were reviewed. Receiver operating characteristic (ROC) statistics were generated to determine CT and MRI accuracy in detecting intracranial extension. RESULTS: Search queries yielded 129 patients who underwent surgical removal of frontonasal dermoids over the study period with an average age of presentation of 12 months. Preoperative imaging was performed on 122 patients, with 19 patients receiving both CT and MRI. CT and MRI were concordant in the prediction of intracranial extension in 18 out of 19 patients. Intraoperatively, intracranial extension requiring craniotomy was seen in 11 patients (8.5%). CT was 87.5% sensitive and 97.4% specific for predicting intracranial extension with an ROC of 0.925 (95% CI [0.801, 1]), whereas MRI was 60.0% sensitive and 97.8% specific with an ROC of 0.789 (95% CI [0.627, 0.950]). CONCLUSION: This is the largest case series in the literature describing a single institution's experience with frontonasal dermoids. Intracranial extension is rare and few patients required craniotomy in our series. CT and MRI have comparable accuracy at detecting intracranial extension. Single-modality imaging is recommended preoperatively in the absence of other clinical indications. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:1961-1966, 2024.

Pediatric Nasal and Septal Fractures.

Pediatric nasal bone and septal fractures represent a large number of craniofacial injuries in children each year. Due to their differences in anatomy and potential for growth and development, the management of these injuries varies slightly from that of the adult population. As with most pediatric fractures, there is a bias toward less-invasive management to limit disruption to future growth. Often this includes closed reduction and splinting in the acute setting followed by open septorhinoplasty at skeletal maturity as needed. The overall goal of treatment is to restore the nose to its preinjury shape, structure, and function.

Delaying Invasive Treatment in Unilateral Head and Neck Lymphatic Malformation Improves Outcomes.

OBJECTIVES: Large (De Serres stage [IV-V]) head and neck lymphatic malformations (HNLMs) often have multiple, high-risk, invasive treatments (ITs) to address functional compromise. Logically reducing HNLM ITs should reduce treatment risk. We tested whether delaying HNLM ITs reduces total IT number. MATERIALS: Consecutive HNLM patients (n = 199) between 2010 and 2017, aged 0-18 years. METHODS: ITs (surgery or sclerotherapy) were offered for persistent or dysfunction causing HNLMs. Treatment effectiveness categorized by IT number: optimal (0-1), acceptable (2-5), or suboptimal (>5). Clinical data were summarized, and outcome associations tested (χ2 ). Relative risk (RR) with a Poisson working model tested whether HNLM observation or IT delay (>6 months post-diagnosis) predicts treatment success (i.e., ≤1 IT). RESULTS: Median age at HNLM diagnosis was 1.3 months (interquartile range [IQR] 0-45 m) with 107/199(54%) male. HNLM were stage I-III (174 [88%]), IV-V (25 [13%]). Initial treatment was observation (70 [35%]), invasive (129 [65%]). Treatment outcomes were optimal (137 [69%]), acceptable (36 [18%]), and suboptimal (26 [13%]). Suboptimal outcome associations: EXIT procedure, stage IV-V, oral location, and tracheotomy (p < 0.001). Stage I-III HNLMs were initially observed compared with stage I-III having ITs within 6 months of HNLM diagnosis, had a 82% lower relative treatment failure risk ([i.e., >1 IT], RR = 0.09, 95% CI 0.02-0.36, p < 0.001). Stage I-III HNLMs with non-delayed ITs had reduced treatment failure risk compared with IV-V (RR = 0.47, 95% CI 0.33-0.66, p < 0.001). CONCLUSION: Observation and delayed IT in stage I-III HNLM ("Grade 1") is safe and reduces IT (i.e., ≤1 IT). Stage IV-V HNLMs ("Grade 2") with early IT have a greater risk of multiple ITs. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:956-962, 2023.

Somatic activating BRAF variants cause isolated lymphatic malformations.

Somatic activating variants in PIK3CA, the gene that encodes the p110α catalytic subunit of phosphatidylinositol 3-kinase (PI3K), have been previously detected in ∼80% of lymphatic malformations (LMs).1 , 2 We report the presence of somatic activating variants in BRAF in individuals with LMs that do not possess pathogenic PIK3CA variants. The BRAF substitution p.Val600Glu (c.1799T>A), one of the most common driver mutations in cancer, was detected in multiple individuals with LMs. Histology revealed abnormal lymphatic channels with immunopositivity for BRAFV600E in endothelial cells that was otherwise indistinguishable from PIK3CA-positive LM. The finding that BRAF variants contribute to low-flow LMs increases the complexity of prior models associating low-flow vascular malformations (LM and venous malformations) with mutations in the PI3K-AKT-MTOR and high-flow vascular malformations (arteriovenous malformations) with mutations in the RAS-mitogen-activated protein kinase (MAPK) pathway.3 In addition, this work highlights the importance of genetic diagnosis prior to initiating medical therapy as more studies examine therapeutics for individuals with vascular malformations.

Outcomes in Pediatric Thyroidectomy: Results From a Multinational, Multi-institutional Database.

OBJECTIVE: Traditionally, data regarding thyroidectomy were extracted from billing databases, but information may be missed. In this study, a multi-institutional pediatric thyroidectomy database was used to evaluate recurrent laryngeal nerve (RLN) injury and hypoparathyroidism. STUDY DESIGN: Retrospective multi-institutional cohort study. SETTING: Tertiary care pediatric hospital systems throughout North America. METHODS: Data were individually collected for thyroidectomies, then entered into a centralized database and analyzed using univariate and multivariable regression models. RESULTS: In total, 1025 thyroidectomies from 10 institutions were included. Average age was 13.9 years, and 77.8% were female. Average hospital stay was 1.9 nights and 13.5% of patients spent at least 1 night in the pediatric intensive care unit. The most frequent pathology was papillary thyroid carcinoma (42%), followed by Graves' disease (20.1%) and follicular adenoma (18.2%). Overall, 1.1% of patients experienced RLN injury (0.8% permanent), and 7.2% experienced hypoparathyroidism (3.3% permanent). Lower institutional volume (odds ratio [OR], 3.57; 95% CI, 1.72-7.14) and concurrent hypoparathyroidism (OR, 3.51; 95% CI, 1.64-7.53) correlated with RLN injury on multivariable analysis. Graves' disease (OR, 2.27; 95% CI, 1.35-3.80), Hashimoto's thyroiditis (OR, 4.67; 95% CI, 2.39-9.09), central neck dissection (OR, 3.60; 95% CI, 2.36-5.49), and total vs partial thyroidectomy (OR, 7.14; 95% CI, 4.55-11.11) correlated with hypoparathyroidism. CONCLUSION: These data present thyroidectomy information and complications pertinent to surgeons, along with preoperative risk factor assessment. Multivariable analysis showed institutional volume and hypoparathyroidism associated with RLN injury, while hypoparathyroidism associated with surgical indication, central neck dissection, and extent of surgery. Low complication rates support the safety of thyroidectomy in pediatric tertiary care centers.

Clinical Outcomes of Diffuse Sclerosing Variant Papillary Thyroid Carcinoma in Pediatric Patients.

OBJECTIVES/HYPOTHESIS: The diffuse sclerosing variant of papillary thyroid carcinoma (DSV) may be more aggressive than conventional well-differentiated non-DSV related papillary thyroid carcinomas (N-PTC). STUDY DESIGN: Retrospective chart review. METHODS: Retrospective review of clinical outcomes for patients 21 years of age or younger who underwent initial surgery for PTC at a single institution from January 1, 2005 to April 1, 2020. Genomic analysis was performed using targeted next-generation sequencing. Data were analyzed using Fischer's exact test and Kaplan-Meier curve log-rank test. RESULTS: Our cohort consisted of 72 patients, nine with DSV and 63 with N-PTC. Age at diagnosis was comparable (15.4 vs. 16.2 years, respectively, P = .46). DSV were more likely to be in the high-risk American Thyroid Academy pediatric risk group (100% vs. 41.3%, P = .004), to present with regional cervical lymph node metastases (100% vs. 60.3%, P = .036), and to present with distant metastases (67% vs. 22%, P = .005). No mortality seen in either group over 27.5 (interquartile range 14.8, 46.00) months average follow-up. Throughout the follow-up period, DSV were more likely to experience progression than N-PTC (hazard ratio = 5.7 [95% confidence interval 1.7-20.0; P = .0056]). In a subset of 19 patients with aggressive disease who had molecular testing as part of clinical care we detected RET fusions in nearly all DSV compared to a minority of N-PTC (83% vs. 15.4%, P = .0095). CONCLUSIONS: Pediatric patients with DSV have more advanced disease at diagnosis and are more likely to experience progression of disease compared to patients with N-PTC. The prevalence of RET fusions in our cohort recapitulates the frequency of this alteration described in prior studies. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:1132-1138, 2022.

Impact of Eliminating Local Anesthesia on Immediate Postoperative Analgesia in Pediatric Ambulatory Adenotonsillectomy.

Our goal was to standardize intraoperative analgesic regimens for pediatric ambulatory tonsillectomy by eliminating local anesthetic use and to determine its impact on postoperative pain measures, while controlling for other factors. METHODS: We assembled a quality improvement team at an ambulatory surgery center. They introduced a standardized anesthetic protocol, involving American Society of Anesthesiologists Classification 1 and 2 patients undergoing adenotonsillectomy. Local anesthesia elimination was the project's single intervention. We collected pre-intervention data (79 cases) from July 5 to September 17, 2019 and post-intervention data (59 cases) from September 25 to December 17, 2019. The intervention requested that surgeons eliminate the use of local anesthetics. The following outcomes measures were evaluated using statistical process control charts and Shewhart's theory of variation: (1) maximum pain score in the post-anesthesia care unit, (2) total post-anesthesia care unit minutes, and (3) postoperative opioid rescue rate. RESULTS: No special cause variation signal was detected in any of the measures following the intervention. CONCLUSIONS: Our data suggest that eliminating intraoperative local anesthetic use does not worsen postoperative pain control at our facility. The intervention eliminated the added expenses and possible risks associated with local anesthetic use. This series is unique in its standardization of anesthetic regimen in a high-volume ambulatory surgery center with the exception of local anesthesia practices. The study results may impact the standardized clinical protocol for pediatric ambulatory adenotonsillectomy at our institution and may hold relevance for other centers.

Pediatric hyperparathyroidism: review and imaging update.

Hyperparathyroidism, due to increased secretion of parathyroid hormones, may be primary, secondary or tertiary. Most pediatric patients with sporadic primary hyperparathyroidism will be symptomatic, presenting with either end-organ damage or nonspecific symptoms. In younger patients with primary hyperparathyroidism, there is a higher prevalence of familial hyperparathyroidism including germline inactivating mutations of the calcium-sensing receptor genes that result in either neonatal severe hyperparathyroidism or familial hypocalciuric hypercalcemia. Parathyroid scintigraphy and ultrasound are complementary, first-line imaging modalities for localizing hyperfunctioning parathyroid glands. Second-line imaging modalities are multiphase computed tomography (CT) and magnetic resonance imaging. In pediatrics, multiphase CT protocols should be adjusted to optimize radiation dose. Although, the role of these imaging modalities is better established in preoperative localization of hyperfunctioning parathyroid glands in primary hyperparathyroidism, the same principles apply in secondary and tertiary hyperparathyroidism. In this manuscript, we will review the embryology, anatomy, pathophysiology and preoperative localization of parathyroid glands as well as several subtypes of primary familial hyperparathyroidism. While most of the recent imaging literature centers on adults, we will focus on the issues that are pertinent and applicable to pediatrics.

Active Observation as an Alternative to Invasive Treatments for Pediatric Head and Neck Lymphatic Malformations.

OBJECTIVES: An increasing number of treatment modalities for lymphatic malformations are being described, complicating therapeutic decisions. Understanding lymphatic malformation natural history is essential. We describe management of head and neck lymphatic malformations where decisions primarily addressed lesion-induced functional compromise (ie, breathing, swallowing) to identify factors associated with invasive treatment and active observation. We hypothesize that non-function threatening malformations can be observed. STUDY DESIGN: Retrospective case series. METHODS: Retrospective case series of consecutive head and neck lymphatic malformation patients (2000-2017) with over 2 years of follow-up. Patient characteristics were summarized and associations with invasive treatment (surgery or sclerotherapy) tested using Fisher's exact. In observed patients, factors associated with spontaneous regression were assessed with Fisher's exact test. RESULTS: Of 191 patients, 101 (53%) were male, 97 (51%) Caucasian, and 98 (51.3%) younger than 3 months. Malformations were de Serres I-III 167 (87%), or IV-V 24 (12%), and commonly located in the neck (101, 53%), or oral cavity (36, 19%). Initial treatments included observation (65, 34%) or invasive treatments such as primary surgery (80, 42%), staged surgery (25, 13%), or primary sclerotherapy (9, 5%). Of 65 initially observed malformations, 8 (12%) subsequently had invasive treatment, 36 (58%) had spontaneous regression, and 21 (32%) elected for no invasive therapy. Spontaneous regression was associated with location in the lateral neck (P = .003) and macrocystic malformations (P = .017). CONCLUSION: Head and neck lymphatic malformation treatment selection can be individualized after stratifying by stage, presence of functional compromise, and consideration of natural history. Recognizing the spectrum of severity is essential in evaluating efficacy of emerging treatments, as selected malformations may respond to observation. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1392-1397, 2021.

Targeted Oncogene Therapy Before Surgery in Pediatric Patients With Advanced Invasive Thyroid Cancer at Initial Presentation: Is It Time for a Paradigm Shift?

Importance: Initial data suggest the effectiveness of oncogene-specific targeted therapies in inducing tumor regression of diverse cancers in children and adults, with minimal adverse effects. Observations: In this review, preliminary data suggest that systemic therapy may be effective in inducing tumor regression in pediatric patients with unresectable invasive thyroid cancer. Although most pediatric patients with thyroid cancer initially present with operable disease, some children have extensive disease that poses substantial surgical challenges and exposes them to higher than usual risk of operative complications. Extensive disease includes thyroid cancer that invades the trachea or esophagus or encases vascular or neural structures. Previous efforts to manage extensive thyroid cancer focused on surgery with near-curative intent. With the recent development of oncogene-specific targeted therapies that are effective in inducing tumor regression, with minimal drug-associated adverse effects, there is an opportunity to consider incorporating these agents as neoadjuvant therapy. In patients with morbidly invasive regional metastasis or with hypoxia associated with extensive pulmonary metastasis, neoadjuvant therapy can be incorporated to induce tumor regression before surgery and radioactive iodine therapy. For patients with widely invasive medullary thyroid cancer, in whom the risk of surgical complications is high and the likelihood of surgical remission is low, these agents may replace surgery depending on the response to therapy and long-term tolerance. Conclusions and Relevance: With oncogene-specific targeted therapy that is associated with substantial tumor regression and low risk of adverse reactions, there appears to be an opportunity to include children with advanced invasive thyroid cancer in clinical trials exploring neoadjuvant targeted oncogene therapy before or instead of surgery.

Pediatric Otolaryngology Divisional and Institutional Preparatory Response at Seattle Children's Hospital after COVID-19 Regional Exposure.

Coronavirus disease 2019 (COVID-19) is a novel coronavirus resulting in high mortality in the adult population but low mortality in the pediatric population. The role children and adolescents play in COVID-19 transmission is unclear, and it is possible that healthy pediatric patients serve as a reservoir for the virus. This article serves as a summary of a single pediatric institution's response to COVID-19 with the goal of protecting both patients and health care providers while providing ongoing care to critically ill patients who require urgent interventions. A significant limitation of this commentary is that it reflects a single institution's joint effort at a moment in time but does not take into consideration future circumstances that could change practice patterns. We still hope dissemination of our overall response at this moment, approximately 8 weeks after our region's first adult case, may benefit other pediatric institutions preparing for COVID-19.

Preoperative Facial Nerve Mapping to Plan and Guide Pediatric Facial Vascular Anomaly Resection.

Importance: Facial vascular anomalies are surgical challenges due to their vascularity and facial nerve distortion. To assist facial vascular anomaly surgical treatment, presurgical percutaneous facial nerve stimulation and recording of compound motor action potentials can be used to map the facial nerve branches. During surgery, the nerve map and continuous intraoperative motor end plate potential monitoring can be used to reduce nerve injury. Objective: To investigate if preoperative facial nerve mapping (FNM) is associated with intraoperative facial nerve injury risk and safe surgical approach options compared with standard nerve integrity monitoring (NIM). Design, Setting, and Participants: This investigation was a historically controlled study at a tertiary vascular anomaly center in Seattle, Washington. Participants were 92 pediatric patients with facial vascular anomalies undergoing definitive anomaly surgery (from January 1, 1999, through January 1, 2015), with 2 years' follow-up. In retrospective review, a consecutive FNM patient cohort after 2005 (FNM group) was compared with a consecutive historical cohort (1999-2005) (NIM group). Main Outcomes and Measures: Postoperative facial nerve function and selected surgical approach. For NIM and FNM comparisons, statistical analysis calculated odds ratios of nerve injury and operative approach, and time-to-event methods analyzed operative time. Results: The NIM group had 31 patients (median age, 3.3 years [interquartile range, 2.2-11.4 years]; 20 [65%] male), and the FNM group had 61 patients (median age, 4.4 years [interquartile range, 1.5-11.0 years]; 26 [43%] male). In both groups, lymphatic malformation resection was most common (19 of 31 [61%] in the NIM group and 32 of 61 [52%] in the FNM group), and the median anomaly volumes were similar (52.4 mL; interquartile range, 12.8-183.3 mL in the NIM group and 65.4 mL; interquartile range, 18.8-180.2 mL in the FNM group). Weakness in the facial nerve branches at 2 years after surgery was more common in the NIM group (6 of 31 [19%]) compared with the FNM group (1 of 61 [2%]) (percentage difference, 17%; 95% CI, 3%-32%). Anterograde facial nerve dissection was used more in the NIM group (27 of 31 [87%]) compared with the FNM group (28 of 61 [46%]) (percentage difference, 41%; 95% CI, 24%-58%). Treatment with retrograde dissection without identification of the main trunk of the facial nerve was performed in 21 of 61 (34%) in the FNM group compared with 0 of 31 (0%) in the NIM group. Operative time was significantly shorter in the FNM group, and patients in the FNM group were more likely to complete surgery sooner (adjusted hazard ratio, 5.36; 95% CI, 2.00-14.36). Conclusions and Relevance: Facial nerve mapping before facial vascular anomaly surgery was associated with less intraoperative facial nerve injury and shorter operative time. Mapping enabled direct identification of individual intralesional and perilesional nerve branches, reducing the need for traditional anterograde facial nerve dissection, and allowed for safe removal of some lesions after partial nerve dissection through transoral or direct excision.

A clinical pathway for the postoperative management of hypocalcemia after pediatric thyroidectomy reduces blood draws.

OBJECTIVES: Postoperative calcium management is challenging following pediatric thyroidectomy given potential limitations in self-reporting symptoms and compliance with phlebotomy. A protocol was created at our tertiary children's institution utilizing intraoperative parathyroid hormone (PTH) levels to guide electrolyte management during hospitalization. The objective of this study was to determine the effect of a new thyroidectomy postoperative management protocol on two primary outcomes: (1) the number of postoperative calcium blood draws and (2) the length of hospital stay. STUDY DESIGN: Institutional review board approved retrospective study (2010-2016). METHODS: Consecutive pediatric total thyroidectomy and completion thyroidectomy ±â€¯neck dissection cases from 1/1/2010 through 8/5/2016 at a single tertiary children's institution were retrospectively reviewed before and after initiation of a new management protocol. All cases after 2/1/2014 comprised the experimental group (post-protocol implementation). The pre-protocol control group consisted of cases prior to 2/1/2014. Multivariable linear and Poisson regression models were used to compare the control and experimental groups for outcome measure of number of calcium lab draws and hospital length of stay. RESULTS: 53 patients were included (n = 23, control group; n = 30 experimental group). The median age was 15 years. 41 patients (77.4%) were female. Postoperative calcium draws decreased from a mean of 5.2 to 3.6 per day post-protocol implementation (Rate Ratio = 0.70, p < .001), adjusting for covariates. The mean number of total inpatient calcium draws before protocol initiation was 13.3 (±13.20) compared to 7.2 (±4.25) in the post-protocol implementation group. Length of stay was 2.1 days in the control group and 1.8 days post-protocol implementation (p = .29). Patients who underwent concurrent neck dissection had a longer mean length of stay of 2.32 days compared to 1.66 days in those patients who did not undergo a neck dissection (p = .02). Hypocalcemia was also associated with a longer mean length of stay of 2.41 days compared to 1.60 days in patients who did not develop hypocalcemia (p < .01). CONCLUSIONS: The number of calcium blood draws was significantly reduced after introduction of a standardized protocol based on intraoperative PTH levels. The hospital length of stay did not change. Adoption of a standardized postoperative protocol based on intraoperative PTH levels may reduce the number of blood draws in children undergoing thyroidectomy.

First branchial cleft anomalies: otologic manifestations and treatment outcomes.

OBJECTIVE: This study describes the presentation of first branchial cleft anomalies and compares outcomes of first branchial cleft with other branchial cleft anomalies with attention to otologic findings. STUDY DESIGN: Case series with chart review. SETTING: Pediatric tertiary care facility. METHODS: Surgical databases were queried to identify children with branchial cleft anomalies. Descriptive analysis defined sample characteristics. Risk estimates were calculated using Fisher's exact test. RESULTS: Queries identified 126 subjects: 27 (21.4%) had first branchial cleft anomalies, 80 (63.4%) had second, and 19 (15.1%) had third or fourth. Children with first anomalies often presented with otologic complications, including otorrhea (22.2%), otitis media (25.9%), and cholesteatoma (14.8%). Of 80 children with second branchial cleft anomalies, only 3 (3.8%) had otitis. Compared with children with second anomalies, children with first anomalies had a greater risk of requiring primary incision and drainage: 16 (59.3%) vs 2 (2.5%) (relative risk [RR], 3.5; 95% confidence interval [CI], 2.4-5; P<.0001). They were more likely to have persistent disease after primary excision: 7 (25.9%) vs 2 (2.5%) (RR, 3; 95% CI, 1.9-5; P=.0025). They were more likely to undergo additional surgery: 8 (29.6%) vs 3 (11.1%) (RR, 2.9; 95% CI, 1.8-4.7; P=.0025). Of 7 persistent first anomalies, 6 (85.7%) were medial to the facial nerve, and 4 (57.1%) required ear-specific surgery for management. CONCLUSIONS: Children with first branchial cleft anomalies often present with otologic complaints. They are at increased risk of persistent disease, particularly if anomalies lie medial to the facial nerve. They may require ear-specific surgery such as tympanoplasty.

Evaluation and treatment of failed nasolacrimal duct probing in Down syndrome.

PURPOSE: To elucidate the mechanisms underlying failed nasolacrimal duct (NLD) probing in children with Down syndrome (DS) utilizing computed tomography (CT) scans and histopathology of nasal mucosa. METHODS: The medical records of children with DS and NLD obstruction confirmed by dye disappearance testing who failed NLD surgery were retrospectively reviewed. Dimensions of the bony NLD and presence of postductal mucosal obstruction were obtained from CT scans. Histopathology of the nasal mucosa was performed in a subset of patients. Subsequent treatment was topical or intranasal corticosteroids or submucosal corticosteroids alone or combined with surgical reduction of the inferior turbinate. RESULTS: A total of 9 subjects (age range, 8-10 years) and 43 age-matched controls were included. Both groups demonstrated a logarithmic increase in NLD and maxilla dimensions with increasing age; however, the transverse diameter of the NLD was consistently 1-2 mm smaller in children with DS ≤5 years age (n = 4) than in age-matched controls. The transverse diameter in DS children overlapped that of controls after 5 years age. Histopathology revealed abnormal lymphoplasmacytic inflammation of the mucosa in 4 of 5 biopsies of DS patients, consistent with chronic infection, allergic disease, or immune dysregulation. The postductal obstruction was successfully treated with topical or intranasal corticosteroids or by surgical reduction of the inferior turbinate submucosa with corticosteroid injection. CONCLUSIONS: Before 5 years of age, NLD obstruction in children with DS was associated with reduced dimensions of the NLD and hypertrophic nasal mucosa. In DS children older than 5 years of age, the dimensions of the NLD are normal and postductal obstruction due to hypertrophic nasal mucosa should be considered.

Propranolol therapy for reducing the number of nasal infantile hemangioma invasive procedures.

IMPORTANCE: Propranolol therapy is changing the treatment paradigm for infantile hemangioma. This study addresses the effect of propranolol therapy on the treatment of nasal infantile hemangioma (NIH), an area that often does not respond to medical therapy. OBJECTIVE: To determine if propranolol treatment is associated with fewer invasive treatments for NIH. DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort study conducted within a single pediatric institution's multidisciplinary vascular anomaly program for patients with NIH treated between January 1, 2003, and December 31, 2011. Three NIH cohorts were compared: prepropranolol (20 in group 1; 2003-2009), propranolol (25 in group 2; 2009-2011), and nonpropranolol (13 in group 3; 2009-2011) treatment. INTERVENTIONS: Analysis of systemic medical, laser, or surgical therapies for NIH. MAIN OUTCOMES AND MEASURES: The study plan was created to detect a change in invasive therapy for NIH. Data collected included presenting age, sex, affected nasal subunits, infantile hemangioma morphologic characteristics, treatment type and number, and primary treating service. An NIH grading system, based on nasal subunit involvement, helped quantify treatment change. Descriptive statistics summarized data, and a Cox proportional hazards regression model evaluated propranolol use and the likelihood of invasive treatments (surgical excision or laser). RESULTS: Of the 95 patients identified, 58 met inclusion criteria: 20 in group 1 (mean age, 4.8 months), 25 in group 2 (mean age, 4.9 months), and 13 in group 3 (mean age, 4.9 months). Nasal infantile hemangiomas involved the nasal tip subunit in 33 of 58 patients (56.9%). Eight of 13 patients (61.5%) in group 3 frequently had small NIH (grade 1). Patients in group 2 were less likely to undergo any invasive treatments (relative risk, 0.44; 95% CI, 0.27-0.73), have surgical excision only (0.45; 0.15-1.38), or undergo laser treatment only (0.44; 0.27-0.78) compared with those in group 1. Patients with higher-grade NIH had more medical or invasive therapy, but invasive procedures were carried out in each subgroup defined by grade. CONCLUSIONS AND RELEVANCE: Patients with isolated propranolol-treated NIH were less likely to undergo invasive treatment, but despite its implementation, the need for invasive treatment was not totally supplanted by its use.

Lymphatic malformations.

PURPOSE OF REVIEW: To update current knowledge of basic science and clinical care of lymphatic malformations. RECENT FINDINGS: Advances in gene sequencing methods have allowed further elucidation of the genetic pathways involved in vascular development. New cell culture techniques are promising the development of practical models to test novel therapeutic interventions. Clinical treatment trends are continuing to shift more away from early surgery and toward sclerotherapy in appropriate cases. New emphasis has been placed upon quality of life analysis of treatment outcomes. SUMMARY: Basic science is increasing the understanding of vascular anomalies in general and may lead us soon toward more effective nonsurgical therapies. Focus on quality of life measures will help to elucidate the most effective therapeutic interventions.

Propranolol use for infantile hemangiomas: American Society of Pediatric Otolaryngology Vascular Anomalies Task Force practice patterns.

IMPORTANCE: This study provides multi-institutional practice guidelines for the initiation of propranolol hydrochloride treatment of routine infantile hemangiomas. OBJECTIVE: To provide information on current propranolol treatment practices for infantile hemangiomas among a cohort of pediatric otolaryngologists. DESIGN AND SETTING: A survey for initiation of propranolol therapy was created by the American Society of Pediatric Otolaryngology Vascular Anomalies Task Force Subcommittee. After an initial pilot of the survey by 4 task force members, the survey was modified and then distributed by e-mail. Results were transferred to spreadsheet format and analyzed. PARTICIPANTS: All 51 members of the task force. RESULTS: A total of 18 respondents from 15 institutions submitted completed surveys. Data from respondents at the same institution were aggregated and/or averaged to minimize regional bias. Fourteen of 15 responding institutions (93%) treat patients with infantile hemangioma as part of a multidisciplinary vascular anomalies team. Ten institutions (67%) routinely consult cardiology before initiation of propranolol therapy. The median propranolol hydrochloride initiation dosage is 2.00 (mean [SD], 1.65 [0.64]; range, 0.45-2.50) mg/kg/d. Postinitiation monitoring for propranolol therapy includes blood pressure (15 of 15 respondents [100%]), serum glucose levels (7 of 15 [47%]), and pulse oximetry (2 of 15 [13%]). Only 2 institutions routinely admit all patients for initiation of propranolol therapy. Typical duration of therapy ranges from 4 to 8 (5 of 15 [33%]) or 8 to 12 months (10 of 15 [67%]), and cessation of therapy in most cases is based on the clinical response (7 of 14 [50%]) or the age of the patient (6 of 14 [43%]). CONCLUSIONS AND RELEVANCE: Propranolol is a commonly used medication for the treatment of infantile hemangiomas among otolaryngologists in the Vascular Anomalies Task Force. Propranolol therapy is commonly initiated in the outpatient setting and continued for as long as 12 months.

Aspirin sensitivity syndrome (Samter's Triad): an unrecognized disorder in children with nasal polyposis.

Aspirin sensitivity syndrome is an underdiagnosed entity in pediatric otolaryngology. The diagnosis must be considered in a pediatric non-cystic fibrosis patient with florid nasal polyposis. In this small case series, we will describe 2 patient's presentation, work up, allergic and surgical therapies and their postoperative course. In doing so, we hope to increase awareness and to illustrate the details that are involved in its diagnosis and treatment.