Pediatric unilateral isolated choanal atresia is not associated with mandibular condyle hypoplasia.

PURPOSE: We hypothesized that the ontogeny of unilateral isolated choanal atresia involves a field defect manifesting as ipsilateral mandibular condylar hypoplasia. The topic is important because the mechanism of the unilateral isolated choanal atresia is unknown. MATERIALS AND METHODS: Retrospective self-controlled case series. We included 20 patients (2 males and 18 females, ages 2 weeks to 13 years) with unilateral isolated non-syndromic choanal atresia. We studied their high-resolution computed tomographic scans. Two otolaryngologists measured the largest cross-sectional area of the mandibular condyle in the axial plane perpendicular to the posterior border of each mandibular ramus independently. Statistical significance and inter-rater agreement were calculated with paired Wilcoxon rank sum test and Spearman's non-parametric correlation coefficient respectively. RESULTS: Cross-sectional areas of the condyles ipsilateral to the choanal atresia were not statistically different than those of the contralateral condyle (P = 0.27). Inter-observer agreement of condyle areas was excellent: Spearman's r = 0.85 on the right and r = 0.94 on the left. CONCLUSIONS: In this cohort of children with the rarity of isolated non-syndromic unilateral congenital choanal atresia, no associated mandibular condyle hypoplasia was found. The data suggest that the underlying ontogeny was unlikely attributable to a field defect.

Submental Island Flaps for Lateral Reconstruction: Technical Refinements for Optimal Outcomes and Resource Efficiency.

Objective: To describe our modifications to the submental island flap (SMIF) in a case series that demonstrates improved reproducibility, shortened length of stay (LOS), and reduced utilization of hospital resources. Study Design: This retrospective case series with chart review included adult patients who underwent resection of malignant or benign tumors resulting in lateral facial, parotid, or temporal bone defects, which were reconstructed with SMIF. Setting: A tertiary-care academic referral center. Methods: Retrospective case series included all adult patients who underwent SMIF reconstruction between March 2020 and August 2021. Patient demographic and clinical data were collected. Primary outcomes were measures of hospital utilization including duration of surgery, LOS, and postoperative outcomes. Results: Twenty-eight patients were included with a mean age of 71.7 years. Eighty percent were male. All patients underwent parotidectomy, and the mean operative time was 347 minutes. The median LOS was 2.5 days (range 0-16 days). Seventy-five percent of the flaps drained into the internal jugular vein, and 25% drained into the external jugular vein. No patients required reoperation or readmission. All flaps survived. Conclusion: SMIFs are a safe and effective option for reconstruction of lateral facial, parotid, and temporal bone defects. Compared to free flap reconstruction, SMIFs offer reduced length of surgery, decreased use of health care resources, and lower rate of reoperation. As health care resource allocation is increasingly important, the SMIF offers an excellent alternative to free flap reconstruction of lateral defects.

Obstructive sleep apnea, sleep symptoms, and their association with cardiovascular disease.

OBJECTIVES: To determine the frequency of obstructive sleep apnea (OSA), insomnia, restless legs, and other sleep symptoms in patients with cardiovascular disease and the association of these sleep disorders with quantitative cardiovascular measures. METHODS: Study design was a cross-sectional survey and retrospective chart review. A questionnaire containing validated sleep symptoms was distributed to 202 patients with cardiovascular disease at a tertiary referral cardiology clinic. Following a focused review of these patients' medical charts, their questionnaire responses were examined for associations with clinical cardiovascular parameters. RESULTS: Twenty-one percent of patients reported a prior diagnosis of OSA. A total of 115 patients (60%) had at least one additional sleep symptom. Clinically significant insomnia was significantly associated with heart disease (relative risk [RR] = 1.5, confidence interval [CI] = 1.1 to 2.1), prior myocardial infarction or cerebrovascular accident (RR = 2.1, CI = 1.2 to 3.6), and heart failure (RR = 2.2, CI = 1.3 to 3.8). Left ventricular ejection fraction was significantly associated with insomnia by Insomnia Severity Index (ß = -0.52, CI = -0.89 to -0.13). CONCLUSION: The frequency of OSA in patients in this tertiary cardiology clinic was higher than the general population in the United States, with the majority of patients experiencing at least one sleep symptom. Insomnia symptoms were shown to be associated with multiple cardiovascular measures, including left ventricular ejection fraction. These findings imply an interwoven relationship between cardiovascular and sleep symptoms as captured by validated sleep questionnaires. LEVEL OF EVIDENCE: IV Laryngoscope, 130:1595-1602, 2020.

Craniomaxillofacial Trauma Experience in Otolaryngology Residency: A National Survey of Program Directors.

This article aimed to assess the depth and volume of craniomaxillofacial (CMF) trauma exposure and education in otolaryngology residency training in the United States. This is a cross-sectional survey. A 15-question web-based survey was distributed to program directors of 106 Accreditation Council for Graduate Medical Education (ACGME)-approved otolaryngology residency programs to inquire about program size and demographics, trauma coverage, case volume, and education. Responses were collected anonymously. A total of 77 responses were received, representing 73% of residency programs. Seventy-five programs (97%) reported that their residents rotated at a level 1 trauma center, and 72 (94%) covered CMF trauma. Sixty-one programs (79%) included pediatric CMF trauma. The majority of programs (76%) allocated less than 10% of residency-dedicated didactic lecture time to CMF trauma. Residents in all programs typically logged at least 11 to 20 cases before graduation with 24% of programs averaging more than 50 cases per resident. Ninety percent of respondents described the training as "somewhat" to "very adequate." CMF coverage by the otolaryngology department, number of cases, and dedicated didactic lecture time to CMF trauma were significant factors on the perception of adequate training. The majority of program directors felt that the training in CMF trauma was adequate. Reasons for this may include that most residents rotate at level 1 trauma centers, have exposure to pediatric trauma, encounter an adequate volume of cases, and have dedicated didactic time to CMF education.

The Prevalence of Superior Semicircular Canal Dehiscence in Patients With Mastoid Encephalocele or Cerebrospinal Fluid Otorrhea.

OBJECTIVE: To evaluate the association between mastoid encephalocele or cerebrospinal fluid (CSF) otorrhea and concurrent superior semicircular canal dehiscence (SSCD). STUDY DESIGN: Retrospective case-control study with chart and imaging review. SETTING: University-affiliated tertiary referral center. PATIENTS: A chart review was conducted of all patients greater than 18 years of age who had primary surgery for CSF otorrhea or encephalocele at our university-affiliated tertiary center from 2000 to 2016. Eighty-three patients matched inclusion criteria for case subjects, and 100 patients without CSF otorrhea or encephalocele were included for controls. MAIN OUTCOME MEASURE: High-resolution computed tomography (CT) scans were reviewed to assess superior semicircular canal roof integrity. Student's t test was used to determine significance of continuous variables. Odds ratio (OR) and χ test was used to determine the association between SSCD and concurrent mastoid encephalocele or CSF otorrhea compared with the control population. RESULTS: The mean age of the 83 case subjects was 54 years, and 73% were women. In patients with confirmed encephalocele and CSF otorrhea, the prevalence of SSCD was 35%, which was significantly greater than controls (2%) (OR = 26.1, p < 0.001). In patients with only CSF otorrhea, 21% had concurrent SSCD (OR = 10.3, p = 0.001). In patients with SSCD, 46% had bilateral canal dehiscence. CONCLUSIONS: This is the largest study to evaluate the prevalence of SSCD in patients with concurrent mastoid encephalocele. This study showed a significant association between SSCD and the presence of both mastoid encephalocele and CSF otorrhea. This data suggests that surgeons should have a high suspicion for SSCD in patients presenting with encephalocele or CSF otorrhea.

A non-canonical JAGGED1 signal to JAK2 mediates osteoblast commitment in cranial neural crest cells.

During craniofacial development, cranial neural crest (CNC) cells migrate into the developing face and form bone through intramembranous ossification. Loss of JAGGED1 (JAG1) signaling in the CNC cells is associated with maxillary hypoplasia or maxillary bone deficiency (MBD) in mice and recapitulates the MBD seen in humans with Alagille syndrome. JAGGED1, a membrane-bound NOTCH ligand, is required for normal craniofacial development, and Jagged1 mutations in humans are known to cause Alagille Syndrome, which is associated with cardiac, biliary, and bone phenotypes and these children experience increased bony fractures. Previously, we demonstrated deficient maxillary osteogenesis in Wnt1-cre;Jagged1f/f (Jag1CKO) mice by conditional deletion of Jagged1 in maxillary CNC cells. In this study, we investigated the JAG1 signaling pathways in a CNC cell line. Treatment with JAG1 induced osteoblast differentiation and maturation markers, Runx2 and Ocn, respectively, Alkaline Phosphatase (ALP) production, as well as classic NOTCH1 targets, Hes1 and Hey1. While JAG1-induced Hes1 and Hey1 expression levels were predictably decreased after DAPT (NOTCH inhibitor) treatment, JAG1-induced Runx2 and Ocn levels were surprisingly constant in the presence of DAPT, indicating that JAG1 effects in the CNC cells are independent of the canonical NOTCH pathway. JAG1 treatment of CNC cells increased Janus Kinase 2 (JAK2) phosphorylation, which was refractory to DAPT treatment, highlighting the importance of the non-canonical NOTCH pathway during CNC cells osteoblast commitment. Pharmacologic inhibition of JAK2 phosphorylation, with and without DAPT treatment, upon JAG1 induction reduced ALP production and, Runx2 and Ocn gene expression. Collectively, these data suggest that JAK2 is an essential component downstream of a non-canonical JAG1-NOTCH1 pathway through which JAG1 stimulates expression of osteoblast-specific gene targets in CNC cells that contribute to osteoblast differentiation and bone mineralization.

Surgical Treatment of OSA on Cardiovascular Outcomes: A Systematic Review.

BACKGROUND: OSA is an increasingly prevalent clinical problem with significant effects on quality of life and cardiovascular risk. Surgical therapy represents an important treatment for those unable to use positive airway pressure. This systematic review examines the available cardiovascular risk reduction data for the surgical treatment of OSA. METHODS: A comprehensive literature search was performed. Articles were included if they met the following criteria: (1) the sample population consisted of adults (age ≥ 18 years); (2) OSA was diagnosed according to a sleep study; (3) surgical intervention was performed for OSA; and (4) one or more physical or biochemical cardiovascular and/or cerebrovascular variables was measured preoperatively and at ≥ 14 days postoperatively. RESULTS: Thirty-three articles were included. The majority of studies were case series and cohort studies (42% and 44%, respectively), with wide-ranging follow-up periods (4 weeks-9 years) and sample sizes (range, 6-10,339; median, 34). The following classes of surgical intervention were examined: pharyngeal surgery (n = 23), tracheostomy (n = 6), maxillomandibular advancement (n = 3), and hypoglossal nerve stimulation (n = 1). In total, 19 outcome measures were assessed. Tracheostomy was most consistently associated with improvement in cardiovascular end points. Pharyngeal surgeries (eg, uvulopalatopharyngoplasty) were variably associated with improvement in cardiovascular end points. CONCLUSIONS: The published literature examining cardiovascular end points following surgical treatment of OSA is limited and generally of poor quality. However, available data from mainly small and observational studies suggest that surgical treatment of OSA may provide improvement in some cardiovascular end points. Larger, randomized, and prospective trials with more rigorous study designs are needed. TRIAL REGISTRY: PROSPERO International Prospective Register of Systemic Reviews (PROSPERO 42016040120).

Perceived compliance and barriers to care in sublingual immunotherapy.

BACKGROUND: For allergy immunotherapy to be effective, patient compliance is critical. However, aqueous sublingual immunotherapy (SLIT) may be considered demanding for patients, requiring strict daily dosing and refilling of medication at regular intervals. In this study we sought to determine how patients perceive their own compliance with SLIT and identify barriers that may hinder compliance. METHODS: Using a retrospective review, 46 patients currently undergoing aqueous SLIT were identified to have less-than-excellent compliance based on frequency of clinic visits for SLIT refills. Twenty-eight of these patients completed a phone survey regarding symptom improvement, compliance, and barriers to care. In addition, 56 patients who withdrew from therapy before completion were polled about barriers to adherence. RESULTS: Of the 28 patients participating in the initial phone survey, 24 (86%) reported subjective improvement in symptoms on SLIT, despite less-than-excellent compliance. Nineteen (68%) of these patients perceived their own compliance to be excellent. Eighteen patients (65%) reported the inconvenience of scheduling and attending clinic appointments to be the main reason for noncompliance. The most common reasons for withdrawal from therapy were cost (27%), lack of effectiveness (25%), and difficulty with compliance (14%). CONCLUSION: The majority of aqueous SLIT patients perceived their compliance to be excellent, although, based on a previous study, these patients did not reach excellent compliance benchmarks. Inconvenience of clinic visits and cost of therapy were found to be the most common barriers to care. Despite what providers perceived as less-than-excellent compliance, 82% of patients reported symptom improvement with SLIT.