Type 3 Laryngeal Clefts Presenting with Upper Airway Obstruction without Aspiration.

Traditionally, otolaryngologists are taught that the defining clinical feature of a laryngeal cleft is aspiration. However, in a small subset of patients-even those with extensive clefts-the sole presenting feature may be airway obstruction. Here, we report two cases of type III laryngeal clefts that presented with upper airway obstruction without aspiration. The first patient was a 6-month-old male with history of tracheoesophageal fistula (TEF) who presented with noisy breathing, initially thought to be related to tracheomalacia. Polysomnogram (PSG) demonstrated moderate OSA and modified barium swallow (MBS) was negative for aspiration. In-office laryngoscopy was notable for a mismatch of tissue in the interarytenoid region. A type III laryngeal cleft was identified on bronchoscopy, and airway symptoms resolved after endoscopic repair. The second patient was a 4-year-old male with a diagnosis of asthma who presented with progressive exercise-induced stridor and airway obstruction. In-office flexible laryngoscopy revealed redundant tissue in the posterior glottis and MBS was negative for aspiration. He was found to have a type III laryngeal cleft on bronchoscopy and his stridor and upper airway obstruction resolved after endoscopic repair. While aspiration is the most common presenting symptom of a laryngeal cleft, it is important to consider that patients can have a cleft in the absence of dysphagia. Laryngeal cleft should be included in the differential diagnosis for patients with obstructive symptoms not explained by other etiologies and in those with suspicious features on flexible laryngoscopy. Laryngeal cleft repair is recommended to restore normal anatomy and relieve obstructive symptoms. Laryngoscope, 134:977-980, 2024.

Incidence of asymptomatic SARS-CoV-2 infection in children undergoing elective otolaryngologic surgery throughout the COVID-19 pandemic.

Objective: Children infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are less clinically affected than adults, with most cases presenting as asymptomatic or mildly symptomatic. However, true rates of asymptomatic SARS-CoV-2 infection in children remain unclear. We sought to examine rates of SARS-CoV-2 in asymptomatic children and the role of children in transmission. Methods: We performed a retrospective review of patients between 6 months and 17 years of age who underwent elective or semi-elective otolaryngologic surgery with physicians affiliated with Weill Cornell Medicine between May 15, 2020 and March 31, 2022. Patients were included if they received molecular assay testing for SARS-CoV-2 without SARS-CoV-2 symptoms within 5 days of scheduled surgery. SARS-CoV-2 infection status, exposure, clinical symptoms, demographic data, and insurance status were recorded. Results: 1047 patients met inclusion criteria. Thirteen positive cases (1.24%) were identified in the study population. Six cases occurred between December 2021 and February 2022 following the classification of the omicron variant as a variant of concern in November 2021. Five of the 13 cases occurred in children under 2 years of age. Seven patients were male, and five were female. Residences spanned all five boroughs of New York City and the surrounding metropolitan area. Conclusion: Throughout the pandemic, children have had a low rate of asymptomatic disease and likely pose a low risk of transmission of SARS-CoV-2 to the general population. Our results suggest that testing of asymptomatic children is a low-yield practice that is unlikely to influence rates of SARS-CoV-2 in the general population. Level of Evidence: 3.

Endoscopic management of intralingual thyroglossal duct cysts: Case series and systematic review.

OBJECTIVE: To discuss our institutional experience with endoscopic management of intralingual thyroglossal duct cyst (TGDC) and review cases in the published literature in a systematic review. METHODS: Pediatric patients with intralingual TGDC treated with endoscopic surgery at our institution from 2009-2019 were identified. Metrics from our case series were then compared to those in the literature in a systematic review to assess pooled outcomes of endoscopic or transoral management. Patient demographics, age of presentation, presenting symptomatology, size of cyst on imaging, type of surgery, and post-operative outcomes were assessed. RESULTS: We identified 5 institutional cases of intralingual TGDC and 48 cases of intralingual TGDC described in the literature. The average age of presentation was 20.36 months. 69.8% (N=37) of patients presented with at least one respiratory symptom, 22.6% (N=12) presented with dysphagia, 9.4% (N=5) presented with an identified mass in the oropharynx, and 15.1% (N=8) had the cyst discovered as an incidental finding. Three patients required revision surgeries due to prior incomplete TGDC excisions and one patient experienced a recurrence >6 months after primary excision requiring a second procedure. Our data pooled with published case series in systematic review confirms that endoscopic or transoral management are excellent options for definitive management of intralingual TGDC. CONCLUSIONS: Intralingual TDGC is a potentially life-threatening variant of TGDC. Our results pooled with published series in a systematic review suggest that endoscopic or transoral management of intralingual TGDC are excellent minimally invasive treatments with a low risk of recurrence. Postoperative surveillance up to one year is recommended.

A 12-Day-Old Female with Hypothermia, Hypoglycemia and Hyperbilirubinemia.

A 12-day-old, full-term female, born small for gestational age, presented to the emergency department with a 1-week history of worsening hyperbilirubinemia, intermittent hypoglycemia, and episodic hypothermia. The baby's emergency department evaluation revealed transaminitis, pneumatosis intestinalis, indirect hyperbilirubinemia, and hypoglycemia. She was admitted to the ICU and received intravenous glucose, bowel rest, and phototherapy. Thyroid-stimulating hormone, thyroxine, and cortisol levels were low, and growth hormone was undetectable. The patient was hospitalized for a total of 19 days and was discharged from the hospital.

Polysomnography-guided mandibular distraction osteogenesis in Pierre Robin sequence patients.

STUDY OBJECTIVES: Craniofacial malformations with micrognathia cause high grades of obstructive sleep apnea (OSA) measured by polysomnography (PSG). Mandibular distraction osteogenesis is a novel procedure for upper airway obstruction relief. Our primary objective was to describe the utilization of PSGs to improve obstruction in patients undergoing mandibular distraction. METHODS: This is a retrospective study. Patients with micrognathia and severe upper airway obstruction, presenting with severe OSA diagnosed by PSG, were included from a single tertiary care center between 2015 and 2019. PSGs were done (1) prior to surgery, (2) once the cosmetic goal was achieved (Post-Op 1), and (3) if residual moderate-to-severe OSA was seen, every 2 nights until mild or no OSA was achieved (Post-Op 2). RESULTS: Thirteen patients were included. The median age at surgery was 1.1 months (10 days-3 months). All 13 patients had baseline severe OSA, with a median obstructive apnea-hypopnea index of 33 events/h and a median O2 nadir of 73%. Post-Op 1 PSG was done at a median of 6 days after surgery. Median first postoperative obstructive apnea-hypopnea index in all 13 patients was 6.8 events/h, with a median O2 nadir of 87%. A median additional distraction of 3 mm was needed beyond the traditionally recommended advancement. Long-term follow-up studies at or after 1 year were done in 5 patients, all showing persistent nonsevere OSA. CONCLUSIONS: This is the first case series utilizing PSGs as a guide for mandibular distraction osteogenesis in patients with micrognathia showing the need for jaw overcorrection to achieve resolution of OSA. CITATION: Kochhar R, Modi V, de Silva N, et al. Polysomnography-guided mandibular distraction osteogenesis in Pierre Robin sequence patients. J Clin Sleep Med. 2022;18(7):1749-1755.

Tympanostomy tubes in the age of quarantine.

OBJECTIVES: After state-mandated COVID-19 quarantine measures were lifted in 2020, pediatric otolaryngologists noticed that many children who were previously scheduled for tympanostomy tube (TT) placement for indications of acute otitis media (AOM) or chronic middle ear effusions (OME) no longer required surgery. This study aims to describe the effect of home-quarantine on pediatric patients with recurrent AOM and OME to increase our understanding of these conditions. METHODS: This was a retrospective review of pediatric patients that were originally scheduled for TT for recurrent AOM and/or OME, but had their procedure cancelled due to COVID-19. The chi-square test was used to compare the proportion of patients who no longer met indications for surgery stratified by original indication. Data was also collected for TT volume in the months before and after the start of the pandemic. RESULTS: Of 59 patients originally scheduled for TT, 31.0% of the 42 patients who returned for follow-up still met indications for a procedure after a period of home-quarantine. Of these, 76.9% had persistent OME, 61.5% had recurrent AOM, and 69.2% had persistent conductive hearing loss. After elective surgery resumed, there was a substantial decrease in the number of TT procedures performed compared to pre-pandemic data. CONCLUSION: After a period of quarantine, many patients previously scheduled for TT experienced resolution of their AOM or OME. Despite a nationwide recovery in outpatient surgical volume across otolaryngology practices, TT volumes remain low one year after the start of the pandemic, suggesting that continued COVID-19 precautionary measures are contributing to this lingering effect.

Polysomnography use in complex term and preterm infants to facilitate evaluation and management in the neonatal intensive care unit.

STUDY OBJECTIVES: (1) To determine the characteristics of term and preterm infants for whom polysomnography (PSG) was used as a primary diagnostic tool in infants with recurrent desaturation episodes, suspected obstructive apnea, or both, and the prevalence of abnormal studies. (2) To identify the interventions following PSGs. (3) To assess the added value of airway and swallow evaluations. METHODS: Retrospective cohort study of infants evaluated by PSG in the Neonatal Intensive Care Unit at New York-Presbyterian Hospital-Weill Cornell from January 2012 to April 2018. RESULTS: PSGs were performed on 31 infants; 15 (48%) term and 16 (52%) preterm infants. Indications for PSG were persistent desaturations (n = 24), suspected obstructive apnea (n = 15), and stridor (n = 2). Primary comorbid conditions were respiratory (n = 11), craniofacial (n = 9), airway anomalies (n = 6), and neurologic (n = 5). The apnea-hypopnea index was abnormal in 30 (97%) infants. Of those, 23 (74%) were severe, 7 (23%) were moderate, and 1 was normal (3%). Apneic events were predominantly obstructive in 23 infants and predominantly central in 6. The apnea-hypopnea index improved in all but 1 follow-up PSG. The PSG findings resulted in interventions in 24 (77%) infants, in addition to concomitant otolaryngology evaluations (abnormal in 20/25) and swallow studies (abnormal in 9/14). Clinical signs completely resolved in 22 (71%) infants. CONCLUSIONS: This is one of the first reports on the diagnostic value of inpatient PSGs in the neonatal intensive care unit in infants with recurrent desaturation episodes, suspected obstructive apnea, or both. Our findings indicate that PSG is an important tool in evaluating and targeting therapies in complex term and preterm infants with a wide variety of comorbidities. CITATION: Kim J, Gueye-Ndiaye S, Mauer E, Modi VK, Perlman J, Veler H. Polysomnography use in complex term and preterm infants to facilitate evaluation and management in the neonatal intensive care unit. J Clin Sleep Med. 2021;17(8):1653-1663.

Establishing an Office-Based Framework for Resuming Otolaryngology Care in Academic Practice During the COVID-19 Pandemic.

OBJECTIVE: The COVID-19 health crisis abruptly disrupted the practice of otolaryngology. This article aims to define the changes needed to operate an academic otolaryngology practice safely and efficiently from within the epicenter of the pandemic. We define the areas of normal patient workflow that have been affected by COVID-19, and we offer mitigation strategies with attention paid to the specific needs of subspecialties. DATA SOURCES: The article includes data specific to the office practice metrics of the Weill Cornell Medicine Department of Otolaryngology-Head and Neck Surgery, as well as publically available data from New York Presbyterian Hospital system and the New York Times. REVIEW METHODS: Expert opinion. CONCLUSIONS: Through careful planning and execution, it is possible to reestablish safe otolaryngologic patient care during the COVID-19 pandemic. It will require a significant change from prior practice models for successful implementation. Additionally, telemedicine can be positively integrated into the treatment of otolaryngology diseases for new and established patients. IMPLICATIONS FOR PRACTICE: The information conveyed in this review can be used as a guide by large and small otolaryngology groups to identify aspects of the patient visit that are "at risk" due to COVID-19, and it suggests sensible responses that can be made without a significant disruption to normal practice. The methods used to identify vulnerabilities with the patient visit process can be applied to future unforeseen crises, such as a resurgence of COVID-19 or a novel pandemic.

IVORY Guidelines (Instructional Videos in Otorhinolaryngology by YO-IFOS): A Consensus on Surgical Videos in Ear, Nose, and Throat.

OBJECTIVES/HYPOTHESIS: Otolaryngology instructional videos available online are often of poor quality. The objective of this article was to establish international consensus recommendations for the production of educational surgical videos in otolaryngology. STUDY DESIGN: DELPHI survey. METHODS: Twenty-seven international respondents participated in this study from 12 countries. Consensus was reached after three rounds of questionnaires following the Delphi methodology. The proposals having reached the 80% agreement threshold in the third round were retained. RESULTS: The main recommendations are as follows: 1) Ethics: patients must be anonymized and unrecognizable (apart from plastic surgery if necessary). A signed authorization must be obtained if the person is recognizable. 2) Technical aspects: videos should be edited and in high-definition (HD) quality if possible. Narration or subtitles and didactic illustrations are recommended. 3) Case presentation: name of pathology and procedure must be specified; the case should be presented with relevant workup. 4) Surgery: surgical procedures should be divided into several distinct stages and include tips and pitfalls. Pathology should be shown if relevant. Key points should be detailed at the end of the procedure. 5) Organ-specific: type of approach and bilateral audiometry should be specified in otology. Coronal plane computed tomography scans should be shown in endonasal surgery. It is recommended to show pre- and postoperative videos in voice surgery and preoperative drawings and photos of scars in plastic surgery, as well as the ventilation method in airway surgery. CONCLUSIONS: International recommendations have been determined to assist in the creation and standardization of educational surgical videos in otolaryngology and head and neck surgery. LEVEL OF EVIDENCE: 5 Laryngoscope, 131:E732-E737, 2021.

Pseudo-cobra neck deformity due to aplasia cutis congenita.

Aplasia cutis congenita is a rare but heterogeneous group of conditions characterized by the absence of one or more areas of skin sometimes extending to deeper tissues. The disorder as a solitary neck lesion has yet to be reported in the literature. Our patient presented with a pseudo-cobra neck deformity secondary to aplasia cutis congenita. Guidance on optimal management for non-scalp and more "benign" presentations of this disorder is lacking.

Pediatric flexible laryngoscopy: Trends in diagnostic abilities throughout training.

OBJECTIVES: Our objectives were to evaluate the ability of residents to diagnose pathology of the pediatric larynx on laryngoscopy, to trend this ability throughout training, to compare their skills to pediatric otolaryngologists, and to determine whether reviewing digitally captured videos in slow motion, as opposed to a live exam, enhanced diagnostic abilities. In addition, we identified pathologies and anatomical sub-sites that posed diagnostic challenges. METHODS: Qualtrics was used to design and distribute an online test, which included 15 pediatric laryngoscopy videos. Participants selected a diagnosis for each video after (1) watching it once at full speed and (2) watching the video multiple times in slow motion. Anonymous responses were exported into excel for statistical analysis, including T-test, ANOVA, and descriptive statistics. RESULTS: There were 21 total participants. Median scores for full speed versus slow motion video review were 47% and 60% respectively. When analyzed by training level, there was no significant difference in scores for full speed videos, but there was a significant difference across groups for slow motion review (p = 0.04). Post Graduate Year (PGY) 4 residents and pediatric otolaryngologists performed best with an average of 69% and 77% respectively. Base of tongue, subglottic, and laryngeal cleft lesions were the most difficult to diagnose. Motor, laryngeal, and vallecular pathologies were more accurately identified. CONCLUSIONS: The ability to diagnose pathology on pediatric laryngoscopy tends to improve throughout residency training. Slow motion review enhances diagnostic skills. Laryngeal and vallecular pathologies are more easily diagnosed than base of tongue and subglottic lesions.

Recalcitrant chronic rhinosinusitis in the setting of fucosidosis, a rare lysosomal storage disorder.

Fucosidosis is an autosomal recessive lysosomal storage disorder caused by the deficiency of alpha-L-fucosidase. We present the case of an affected female in the second decade of life with chronic rhinosinusitis (CRS) including recalcitrant polypoid inflammation, which has not been previously reported in the literature. With the advancement of life-prolonging measures, children with lysosomal storage disorders may suffer increasingly from CRS due to the lymphohistiocytic and macrophage infiltrate of the paranasal sinus mucosa that resembles severe polypoid inflammation.

Surgery of the head and neck in patient with Kniest dysplasia: Is wound healing an issue?

Kniest dysplasia is a type II collagen disorder that arises from a genetic mutation of the COL2A1 gene that results in short stature, midface anomalies, tracheomalacia, and hearing loss. Disruption of the normal collagen pathway can lead to many changes given its critical role in the body, and can cause complications with respect to wound healing. We present a case in which a patient with Kniest dysplasia successfully underwent multiple procedures in the head and neck region including cochlear implantation, mandibular distraction, palatoplasty, and laryngotracheal reconstruction. All procedures did not have any associated complications with respect to wound healing, indicating that surgery in this population can take place as indicated and surgery should not be contraindicated or delayed.

Histopathological effect of balloon dilation in a live rabbit: Implications for the pediatric airway.

OBJECTIVE/HYPOTHESIS: To examine the short- and long-term histopathologic changes that occur in the subglottis in response to airway balloon dilation (ABD) with different balloon diameters and inflation pressures. STUDY DESIGN: Prospective animal study using forty-two 8-month old New Zealand white rabbits at an academic animal research facility. METHODS: Thirty-nine live New Zealand rabbits underwent a single ABD with diameters ranging from 6.0 mm to 10.0 mm and with pressures between 5.0 atmospheres (atm) to 15.0 atm. Animals were euthanized on postoperative days (POD) 1, 7, and 30, and the histopathological changes of the subglottis were examined. Three rabbits served as controls and underwent no ABD. RESULTS: The subglottic airway diameter of all specimens measured 5.4 mm. When examining the fracture rate by balloon diameter, we found the following: 0 of 6 (0%) at 6 mm, 0 of 9 (0%) at 7 mm, 6 of 9 (67%) at 8 mm, 8 of 9 (89%) at 9 mm, and 6 of 6 (100%) at 10 mm. There was a statistically significant relationship with the rate of cricoid fracture as balloon diameter increased (P < .0001). All fractures occurred at the anterior cricoid lamina. On POD 1, we found mild ulceration in 5 of 6 (83%) using a 6-mm or 7-mm balloon and in 0 of 6 (0%) using an 8-mm, 9-mm, or 10-mm balloon; and moderate/severe ulceration in 1 of 6 (16.67%) using a 6-mm or 7-mm balloon and in 11 of 11 (100%) using an 8-mm, 9-mm, or 10-mm balloon (P < .0001). Also on POD 1, we found mild edema in 6 of 6 (100%) using a 6-mm or 7-mm balloon and in 5 of 11 (45%) using an 8-mm, 9-mm, or 10-mm balloon; and moderate/severe edema in 0 of 6 (0%) using a 6-mm/7-mm balloon and in 6 of 11 (55%) using an 8-mm, 9-mm, or 10-mm balloon (P =.048). On POD 7, we found fibroplasia in 5 of 6 (83%) using a 6-mm or 7-mm balloon and in 1 of 7 (14%) using an 8-mm, 9-mm, or 10-mm balloon; and moderate/severe fibroplasia in 1 of 6 (17%) using a 6-mm or 7-mm balloon and in 6 of 7 (86%) using an 8-mm, 9-mm, or 10-mm balloon (P =.029). Also on POD7, we found granulation tissue in 0 of 6 (0%) using a 6-mm or 7-mm balloon and in 5 of 7 (71%) using an 8-mm, 9-mm, or 10-mm balloon (P = .021). On POD 30, we found no fibrosis in 0 of 3 (0%) using a 7-mm balloon, mild fibrosis in 1 of 6 (16.67%), and moderate/severe fibrosis in 5 of 6 (83%) using an 8-mm or 9-mm balloon (P = .048). Also on POD 30, we found the mean subglottic cross-sectional luminal area was 23.79 mm(2) with a 7-mm balloon and 29.28 mm(2) with an 8-mm or 9-mm balloon (P = .019). Inflation pressure alone had no correlation with mucosal injury or probability of cricoid fracture. CONCLUSIONS: Airway balloon dilation with balloon diameters that exceeded the airway diameter by 2.6 mm was associated with cricoid fractures. All cricoid fractures localized to the anterior cricoid lamina. Balloon diameters larger than the airway diameter by 2.6 mm resulted in a larger subglottic cross-sectional luminal area on POD 30. Airway balloon dilation with balloon diameters that could generate a cricoid fracture created more mucosal injury on POD 0, 1, and 7 than smaller balloon diameters. The histopathological effects of airway balloon dilation observed on POD 0, 1, and 7 resolved by POD 30. When balloon diameter is kept constant, inflation pressure alone had no correlation with mucosal injury or probability of cricoid fracture. LEVEL OF EVIDENCE: NA (animal study). Laryngoscope, 125:S1-S11, 2015.

Thyroglossal duct cyst and ectopic thyroid: surgical management.

The embryology, presentation, imaging, and treatment of the thyroglossal duct cyst will be reviewed. Anatomic features and surgical technique to prevent complications and recurrence will be discussed. Included in the discussion will be the management of thyroglossal duct cyst malignancy and ectopic thyroid.

Tracheal cartilaginous sleeve association with syndromic midface hypoplasia.

IMPORTANCE: We report 2 new cases of tracheal cartilaginous sleeve and perform a systematic literature review of all documented cases of this condition. We aim to increase awareness of this anomaly and provide recommendations for both intraoperative and postoperative management of patients with tracheal cartilaginous sleeve undergoing tracheostomy. OBSERVATIONS: We studied the clinical records of 2 children with tracheal cartilaginous sleeves and short tracheas treated at our institution. One of these patients had Beare-Stevenson syndrome, and the other had Crouzon syndrome. Both patients required tracheostomy for persistent upper airway obstruction, and both required custom-length tracheostomy tubes. Bronchoscopy and needle localization were beneficial in performing tracheostomy on these patients. All documented cases of tracheal cartilaginous sleeve in the literature were found to be associated with midface hypoplasia secondary to syndromic craniosynostosis. Seventy-five percent of cases required tracheostomy. CONCLUSIONS AND RELEVANCE: Tracheal cartilaginous sleeve can be associated with Beare-Stevenson syndrome. In infants with midface hypoplasia associated with a craniosynostosis syndrome undergoing tracheostomy, the surgeon must be prepared to encounter and manage tracheal cartilaginous sleeve intraoperatively. Bronchoscopy and needle localization can be beneficial when performing tracheostomy in these patients. Customized, shorter-length tracheostomy tubes should be considered for these patients.