Impact of upper airway obstruction management in Robin Sequence on need for myringotomy tubes.

OBJECTIVE: Investigate an association between upper airway obstruction (UAO) management in Robin Sequence (RS) and need for bilateral myringotomy and tubes (BMT). METHODS: Retrospective chart review of RS patients treated at a tertiary free-standing pediatric hospital from 1995 to 2020 was performed. Patients were grouped based on airway management: conservative, tracheostomy, tongue-lip adhesion (TLA), and mandibular distraction osteogenesis (MDO). Demographic data, cleft palate (CP) association, numbers of BMT and ear infections, and audiogram data including tympanograms were collected. One-way ANOVA and Chi-square/Fisher's exact tests were used to compare continuous and categorical data, respectively. Multivariable regression analysis was used to compare BMT rates between treatment groups. RESULTS: One hundred forty-eight patients were included, 70.3 % of which had CP. Most patients (67.6 %) had at least one BMT; 29.1 % required two or more BMT. The rate of BMT was higher in patients with CP compared to those with intact palates (p = 0.003; 95 % CI 1.30-3.57) and those treated with tracheostomy (p = 0.043; 95 % CI 1.01, 2.27). Surgically managed patients were more likely to have hearing loss (67.5 % vs. 35.3 %, p = 0.017) and ear infections (42.1 % vs. 20.0 %, p = 0.014) pre-compared to post-procedure for airway management. CONCLUSION: Most RS patients require at least 1 set of BMT. Those with CP and/or treated with tracheostomy had a higher likelihood of needing BMT. Rate of hearing loss and ear infection was higher in surgically managed RS patients. Patients with RS and overt CP require a statistically higher number of BMTs compared to those with either submucous cleft palate or intact palate.

Trends in Natural Decannulation in Patients with Robin Sequence: A Twenty-five Year Retrospective Review.

OBJECTIVE: Robin sequence (RS) consists of micrognathia and glossoptosis that result in upper airway obstruction (UAO). In RS patients who undergo tracheostomy, long-term goals include natural decannulation (ND) without further surgical airway intervention. The objective of this study was to identify long-term trends in the rate and length of time to ND. METHODS: Retrospective chart review on 144 patients with RS treated from 1995 to 2020 at a pediatric tertiary care center. Patients were grouped by year of tracheostomy. Demographic data, UAO management, postoperative care, complications, and time to decannulation were recorded. RESULTS: Thirty-six patients met the inclusion criteria. Tracheostomy was performed at a median age of 45.5 days. 19 (53%) patients experienced ND at a median time of 66.1 months. ND rate was higher in non-syndromic patients (93% non-syndromic vs 27% syndromic; P < .0001) and during the first study period (1995-2006: 78%, 2007-2020: 28%; P = .003). Cox proportional-hazard regression demonstrated that white race [aHR 0.15 (0.03-0.8); P = .023] and higher birthweight [aHR 0.9 (0.8-0.98); P = .018] were associated with a higher likelihood of ND while a syndromic diagnosis had a negative association with ND [aHR 12.5 (3.3-50.0); P < .001]. CONCLUSIONS: Our study documented that ND in patients with RS who underwent tracheostomy was significantly associated with ethnicity, birthweight, and syndromic status. The negative impact on successful ND was most observed in patients with syndromic associations. Additionally, ND rates are lower in the 2007 to 2020 subgroup. We suspect this is because alternative management techniques such as tongue lip adhesion and mandibular distraction osteogenesis became primary surgical treatment in severe RS upper airway obstruction at our institution starting in 2007.

Outcomes and Management of Infants Who Refer Newborn Hearing Screening.

OBJECTIVES: With the implementation of Universal Newborn Hearing Screening, early diagnosis and referral has been expedited. Many patients who refer screening pass subsequent testing with otoacoustic emissions (OAE) or auditory brainstem response (ABR). The objective of our study was to identify the incidence and etiology of hearing loss in infants who refer initial testing in an urban, tertiary care pediatric otolaryngology practice. METHODS: We performed a chart review of infants who were evaluated after referring newborn hearing screening from 2017 to 2021. Data collected included birth history, hospital screening results, subsequent audiology and otolaryngology visit findings, final hearing diagnoses, interventions, and outcomes. RESULTS: Of the 450 patients, 83.8% (n = 377) had normal hearing bilaterally after repeat testing (OAE and/or ABR). Thirty five patients were diagnosed with otitis media with effusion (OME) (7.8%) and 17 patients (3.8%) were diagnosed with sensorineural hearing loss. Twenty seven patients (6.0%) were diagnosed with obstructing cerumen/vernix, many times in addition to another diagnosis. Of the 17 patients with sensorineural hearing loss, 2 had genetic syndromes and 2 had congenital cytomegalovirus. Sensorineural hearing loss was significantly associated with the presence of a deafness syndrome (P = .004) and in-utero infections (P = .04). About 11 (2.4%) underwent myringotomy with tube placement, 5 (1.1%) were fitted with hearing aids, 2 (0.4%) were referred for hearing aids, 4 (0.9%) had both myringotomy with tube placement and hearing aids, 1 child had a soft band/Bone Anchored Hearing Aid (BAHA) (0.2%), and 1 child (0.2%) had a cochlear implant. CONCLUSION: Our incidence of sensorineural hearing loss was 3.8% (95% CI 2.0, 5.5), compared to rates of 0.44 to 68% in the published literature. Most patients had normal hearing, usually identified after only 1 repeat test. OME requiring myringotomy tube insertion was the most common pathology requiring intervention. Close observation for resolution and intervention, if warranted, is critical to prevent sequelae.

Otolaryngologists' Attitudes toward In-Office Pediatric Tympanostomy Tube Placement without General Anesthesia.

OBJECTIVES: Our objectives were to assess attitudes regarding office-based insertion of tympanostomy tubes without general anesthesia, to identify barriers that would discourage in-office procedures, and to highlight opportunities that would potentially facilitate this approach in the future. METHODS: Cross-sectional survey administered to members of the American Society of Pediatric Otolaryngology (ASPO) from March to April 2020 using the Research Electronic Data Capture (Redcap), internet-based data capture platform. The brief, 10-item survey required 3 minutes to complete and used a 5-point Likert scale for primary questions. RESULTS: Respondents included 172 fellowship trained, pediatric otolaryngologists with 14 median years of clinical practice and 25 median tympanostomy tube insertions per month (75% >40 per month). Although tubes, in any setting, were most often inserted in children under age 2 years (95% "often" or "very often") and in those aged 3 to 5 years (93%), the likelihoods of doing this in-office for these age groups were only 8% and 6% respectively. For children aged 6 to 12 years, likelihood of in-office insertion was only 15%. Frequent barriers noted were safety concerns, emotional trauma, physical pain, and inability to suction. Opportunities to facilitate this approach include improved topical anesthesia, availability of conscious sedation, conclusive research on adverse effects of general anesthesia, and availability of an automated tube insertion device. CONCLUSION: Office-based insertion of tympanostomy tubes in children without general anesthesia is performed by a small minority of respondents, but there are discernible barriers and opportunities to promote future uptake. Our results should facilitate ongoing discussion and innovation to better accommodate the preferences of families whose children are candidates for tympanostomy tubes.

A Rare Case of Invasive Mucormycosis in a Diabetic Patient Treated with a Short Course of Dexamethasone.

BACKGROUND Invasive mucormycosis is a rare, life-threatening infection that requires urgent medical management. Here we describe a patient who developed invasive mucormycosis after receiving only a short course of dexamethasone. The purpose is to highlight this atypical presentation of a rare disease. CASE REPORT A 74-year-old woman with a medical history of diabetes mellitus (DM), hypertension, hyperlipidemia, and small cell lung cancer with metastasis to the brain presented to the Emergency Department with altered mental status and a hyperosmolar hyperglycemic state. Three weeks before, she had been diagnosed with DM (hemoglobin A1c [HbA1c] 6.5%) and was started on dexamethasone to treat cerebral edema. On admission, her HbA1c was 10.8%, although she had received only a short course of dexamethasone. Her physical exam was concerning for left eyelid swelling and ophthalmoplegia. Computed tomography of the head and neck revealed signs of left ocular proptosis and invasive rhinomaxillary fungal disease. The patient underwent urgent surgical debridement; subsequent magnetic resonance imaging revealed extensive fungal disease extending into her left inferior frontal lobe. A surgical pathology report was positive for Rhizopus oryzae and Stenotrophomonas maltophilia. Her blood cultures were positive for methicillin-susceptible Staphylococcus aureus. She was treated with antibiotics and amphotericin B. Her clinical course was complicated by hypokalemia. She eventually recovered and was discharged from the hospital. CONCLUSIONS This case highlights an atypical presentation of mucormycosis. Clinicians should remain vigilant for this rare complication of dexamethasone use even when the therapy is given for a short time.

Surgical Management of Sialorrhea: A Systematic Review and Meta-analysis.

OBJECTIVE: Chronic sialorrhea commonly occurs in patients with neurodevelopmental disorders. While conservative management can provide sufficient symptom control, surgical intervention is often required. One of the most common procedures utilized is submandibular gland excision (SMGE), with or without parotid duct ligation or rerouting (PDL or PDR). This study aims to compare these surgical approaches and their outcomes. DATA SOURCES: PubMed, Web of Science, and Embase. REVIEW METHODS: This systematic review includes studies of patients with chronic sialorrhea treated with SMGE alone or SMGE plus PDR or PDL and reports on postintervention outcomes and complications. Two independent investigators assessed study eligibility, rated quality, and extracted data for analysis. A random effects model was used for meta-analysis of pooled data. RESULTS: Of 3186 studies identified, 21 met inclusion criteria, with 708 patients: 103 underwent SMGE alone (15%); 299 (42%), SMGE and PDL; and 306 (43%), SMGE plus PDR. Overall, a majority of patients had significant improvement, with very good to excellent control of symptoms after surgery: SMGE, 82% (95% CI, 73%-89%); SMGE and PDL, 79% (95% CI, 73%-85%); and SMGE and PDR, 85% (95% CI, 75%-92%). Importantly, there was no significant difference in outcomes with the addition of PDL or PDR. Reported complications included sialocele, parotitis, dental caries, and dry mouth. CONCLUSION: Our systematic review identified consistent positive outcomes with SMGE for patients with chronic sialorrhea but no additional benefit when PDR or PDL was performed as a concurrent procedure.

Management of Type 1 Laryngeal Clefts: A Systematic Review and Meta-analysis.

OBJECTIVE: Endoscopic surgical management or injection laryngoplasty of type 1 laryngeal clefts in pediatric patients is used in those who do not respond to conservative treatment. This study compares conservative treatment, endoscopic surgical repair, and injection laryngoplasty for the management of type 1 laryngeal clefts. DATA SOURCES: PubMed, Web of Science, and Embase. REVIEW METHODS: This systematic review included studies of patients with type 1 laryngeal clefts who were managed with conservative treatment, injection laryngoplasty, or endoscopic repair, and all studies reported postintervention outcomes. Two independent investigators assessed study eligibility, rated the quality, and extracted data for analysis. A random effects model was used for meta-analysis of pooled data. RESULTS: Of the 1209 studies identified, 27 met inclusion criteria. There were 543 patients with type 1 laryngeal clefts represented in the studies, with outcomes reported for 537. Conservative therapy had a 52% (95% CI, 37%-66%; I2 = 63%) success rate at improving symptoms, while endoscopic repair had a significantly higher percentage resolution of symptoms (70%; 95% CI, 59%-79%; I2 = 62%, P < .001) as compared with conservative treatment (51%; 95% CI, 36%-65%; I2 = 62%) or injection laryngoplasty (36%; 95% CI, 20%-57%; I2 = 70%). The quality scores of the studies ranged from 7 to 12 out of 16. CONCLUSION: Our systematic review demonstrated significant improvement and resolution of symptoms for patients with type 1 laryngeal clefts treated with endoscopic repair as compared with other modalities. More prospective and controlled studies comparing treatment strategies with validated instruments to measure outcomes are necessary to determine their efficacy in the management of type 1 laryngeal clefts.

Purely endoscopic endonasal surgery of the craniovertebral junction: A systematic review.

BACKGROUND: Endoscopic endonasal surgery (EES) is a relatively novel approach to the craniovertebral junction (CVJ). The purpose of this analysis is to determine the surgical outcomes of patients who undergo purely EES of the CVJ. METHODS: A search for articles related to EES of the CVJ was performed using the MEDLINE/PubMed database. A bibliographic search was done for additional articles. Demographics, presenting symptoms, imaging findings, complications, follow-up, and patient outcomes were analyzed. RESULTS: Eighty-five patients from 30 articles were included. The mean patient age was 47.9 ± 24.8 years (range, 3 to 96 years), with 44.7% being male. The most common presenting symptom was myelopathy (n = 64, 75.3%). The most common indications for surgery were brainstem compression secondary to basilar invagination (n = 41, 48.2%) and odontoid pannus (n = 20, 23.5%). Odontoidectomy was performed in 97.6% of cases. Intraoperative complications occurred in 16 patients (18.8%) and postoperative complications occurred in 18 patients (21.2%). Six patients developed postoperative respiratory failure necessitating a tracheostomy. Neurologic improvement was seen in 89.4% of patients at a mean follow-up of 22.2 months. CONCLUSION: Our analysis found that EES of the CVJ results in a high rate of neurologic improvement with acceptable complication rates. Given its minimally invasive nature and high success rate, this approach appears to be a reasonable alternative to the traditional transoral approach in select cases. This study represents the largest pooled sample size of EES of the CVJ to date. Increasing use of the endoscopic endonasal approach will allow for further studies with greater statistical power.