Slide Tracheoplasty for Tracheal Cartilaginous Sleeve in a Patient With Apert Syndrome.

Apert syndrome is a form of acrocephalosyndactyly involving craniosynostosis, syndactyly, and less commonly, tracheal cartilaginous sleeve (TCS), a potential cause of tracheal stenosis. Slide tracheoplasty is performed in children with tracheal stenosis. No reports exist for its application in stenosis related to TCS. We present a case in which slide tracheoplasty was used for the expansion of long segment tracheal stenosis owing to TCS in a newborn with Apert syndrome. Using this technique, a safe and durable airway was achieved without tracheostomy.

Socioeconomic and clinical factors influencing treatment selection in microtia and aural atresia.

OBJECTIVES: Patients with microtia and aural atresia have multiple options for treatment of conductive hearing loss (CHL) and auricle reconstruction; however, little is known about the factors influencing treatment selection. This study aims to review the socioeconomic and clinical data of microtia/atresia patients to evaluate congruency with national data and whether these factors affect treatment decisions. METHODS: Retrospective review of patients evaluated in the microtia and atresia multidisciplinary clinic (MDC) at a tertiary academic children's hospital between 2008 and 2018. Outcomes included demographic, socioeconomic and clinical factors associated with hearing surgery and framework surgery. RESULTS: 373 patients were seen in the Microtia MDC: 193 (51.7%) were male, 187 (50.1%) identified as Hispanic and 23 (6.2%) identified as Asian. 267 (75.6%) patients received a nonsurgical bone conduction hearing device (BCHD); fitting at a younger age was associated with better nonsurgical BCHD compliance. Multivariate analysis was performed on the patients that were eligible for surgery based on age and appropriate follow-up. 70 (18.8%) patients had placement of an osseointegrated BCHD; inconsistent compliance with nonsurgical BCHD decreased the odds of proceeding with osseointegrated BCHD placement. 60 (16.1%) patients underwent framework surgery for external reconstruction. Placement of osseointegrated BCHD was the only factor that was associated with proceeding with framework surgery. Other assessed demographic and socioeconomic factors were statistically not associated with selection of surgical intervention. CONCLUSION: Fitting a nonsurgical BCHD at a younger age is associated with higher likelihood of nonsurgical BCHD compliance, that is in turn associated with patients and families proceeding with osseointegrated BCHD and framework surgery.

Cost comparison of different treatment approaches of dacryocystitis and dacrocystocele.

PURPOSE: Congenital dacrocystocele with potential for dacryocystitis are common ophthalmic findings in children. There are multiple surgical approaches to open the mucocele. In this study, we look at the financial impact of these different approaches. METHODS: A retrospective chart review of 17 patients with dacrocystocele or dacryocystitis was performed. We examined four approaches: (1) bedside nasal endoscopy with marsupialization of nasolacrimal duct (NLD) cyst, (2) surgically performed nasal endoscopy with marsupialization of NLD cyst, (3) NLD probe, and (4) a combination of procedures. Cost of the procedure and length of anesthesia were collected. Reoccurrence of symptoms and disease post-procedure were also collected. RESULTS: The lowest cost billed procedure was bedside nasal endoscopy performed by an otolaryngologist (US$435; n = 1). A nasal endoscopy (n = 2) performed in the operating room (OR) had an average OR fee of US$14,557 [standard deviation (SD): US$7598] for 108.5 (SD: 87.0) min of operating time. An NLD probe (n = 5) performed by pediatric ophthalmologists resulted in an average OR fee of US$5540 (SD: US$1752) for 31.0 min (SD: 8.6 min) of operating time. A combination of both nasal endoscopy and NLD probing (n = 9) had an average OR fee US$10,325 (SD: US$4137) for 69 min (SD: 34.5 min) of operating time. CONCLUSION: This is the first study looking at cost benefit of four different approaches to treating dacrocystoceles/dacryocystitis. A NLD probe was a low-cost OR intervention and had the shortest operating time. The combination procedure was more cost-effective than nasal endoscopy or NLD probing alone.

Outcomes of Airway Management in Micrognathia and Retrognathia Patients Born at Fetal versus Nonfetal Centers.

OBJECTIVES: There is a paucity of evidence to guide the perinatal management of difficult airways in fetuses with micrognathia. We aimed to (1) develop a postnatal grading system based on the extent of airway intervention required at birth to assess the severity of micrognathic airways and (2) compare trends in airway management and outcomes by location of birth [nonfetal center (NFC), defined as a hospital with or without an NICU and no fetal team, versus fetal center (FC), defined as a hospital with an NICU and fetal team]. METHODS: We retrospectively reviewed the prenatal and postnatal records of all neonates diagnosed with micrognathia from January 2010 to April 2018 at a quaternary children's hospital. We developed a novel grading scale, the Micrognathia Grading Scale (MGS), to grade the extent of airway intervention at birth from 0 (no airway intervention) to 4 (requirement of EXIT or advanced airway instrumentation for airway securement). RESULTS: We identified 118 patients with micrognathia. Eighty-nine percent (105/118) were eligible for grading using the MGS. When the MGS was applied, the airway grades were as follows: grade 0 (30%), grade 1 (10%), grade 2 (9%), grade 3 (48%), and grade 4 (4%). A quarter of micrognathic patients with grade 0-2 airways had postnatal hospital readmissions for airway obstruction after birth, of which all were born at NFC. Over 40% of patients with grade 3-4 micrognathic airways required airway intervention within 24 h of birth. Overall, NFC patients had a readmission rate of (27%) for airway obstruction after birth compared to FC patients (17%). CONCLUSIONS: Due to the high incidence of grade 3-4 airways on the MGS in micrognathic patients, fetuses with prenatal findings suggestive of micrognathia should be referred to a comprehensive fetal care center capable of handling complex neonatal airways. For grade 0-2 airways, infants frequently had postnatal complications necessitating airway intervention; early referral to a multidisciplinary team for both prenatal and postnatal airway management is recommended.

Benign tumoral melanosis of a cervical lymph node in a child.

We present a pediatric case of benign tumoral melanosis in a cervical lymph node arising from congenital pigmented lesions of the anterior neck. This is the first reported pediatric case not associated with regressed melanoma or trauma. Tumoral melanosis is a rare phenomenon which describes a pigmented lesion like melanoma; however, histology demonstrates melanin-laden macrophages without malignant cells. In the few reported cases, tumoral melanosis has arisen in the skin or lymph node and associated with a regressed melanoma, pigmented basal cell carcinoma or mycosis fungoides. We discuss the pathology of this disease process, and the work up in a pediatric patient.

Dornase Alfa Ototoxic Effects in Animals and Efficacy in the Treatment of Clogged Tympanostomy Tubes in Children: A Preclinical Study and a Randomized Clinical Trial.

Importance: Many treatments for clogged tympanostomy tubes (TTs) have been proposed, but none have met scientific rigor for safety and efficacy, including the popular empirical use of ototopical antibiotic drops. Dornase alfa, a recombinant molecule with the unique property of cleaving DNA, may be ideal in treating clogged TTs because both middle-ear effusion and the plug are abundant with DNA. Objective: To investigate the ototoxic effects of dornase alfa in a chinchilla model and its efficacy in a clinical trial in children with clogged TTs. Design, Setting, and Participants: The safety profiles of dornase alfa (full-strength and 1:10 strength) were evaluated in chinchilla middle ears using serial auditory brainstem response. The efficacy of ototopical dornase alfa (full-strength) was evaluated in children with clogged TTs in a prospective, single-blind randomized clinical trial. The animal study included 21 chinchillas and was conducted at Loma Linda University, Loma Linda, California, and the clinical trial was conducted at Children's Hospital Colorado, Aurora. A total of 40 children (50 ears with tubes) were enrolled. Interventions: In the animal study, chinchillas were assigned to 3 groups: controls (saline), full-strength dornase alfa, or 1:10 dornase alfa dilution. Children were randomly assigned to receive either topical dornase alfa or ofloxacin for clogged TT, 5 drops each ear twice a day for 7 days. Main Outcomes and Measures: Animal study: Auditory brainstem responses. Randomized trial of children participants: The primary outcome was patency of TT at day 14 assessed by otoscopy and tympanometry. Results: The chinchilla study showed similar auditory brainstem response degradation during a 6-hour period between the control (n = 5) and treatment groups (n = 21). In the clinical trial, a total of 40 clogged TTs (in 33 children, including 25 boys [76%]; mean age, 4.3 years; median [range] age, 3.4 [1.0-14.3] years) were analyzed. The number of unclogged TTs was higher in the dornase alfa group (13 [59%]) compared with the ofloxacin group (8 [44%]), with a difference of 15% (odds ratio, 1.8; 95% CI, 0.54-6.72). Conclusions and Relevance: The chinchilla model suggests that dornase alfa is likely nonototoxic. The pilot clinical trial failed to show efficacy of dornase alfa to unclog TTs. With the difference seen between the treatment groups, a sample size estimate could be calculated for a future large-scale trial. Trial Registration: ClinicalTrials.gov identifier: NCT00419380.

Parapharyngeal and skull base yolk sac tumor: a case report with lessons in diagnosis and management.

Yolk sac tumors are rare in the head and neck. A previously healthy 2-year-old female presented with a large parapharyngeal mass. Pathology was pathognomonic for yolk sac tumor, with glandular differentiation and focal mucin production, which has not been reported in a yolk sac tumor. She was treated aggressively with chemotherapy followed by endoscopic exploration with planned resection, but no viable tumor was encountered. Yolk sac tumors can be difficult to diagnose in the head and neck, but complete clinical response can be achieved. New endoscopic approaches to skull base tumors are applicable to the pediatric population with some technical modifications.

Sedation-related outcomes in postoperative management of pediatric laryngotracheal reconstruction.

OBJECTIVE: Examine outcomes of varied postoperative sedation management in pediatric patients recovering from single stage laryngotracheal reconstruction. DESIGN: Retrospective review of 34 patients treated with single stage laryngotracheal reconstruction from 2001 through 2011. SETTING: Tertiary children's hospital. METHODS: Patients were divided into 2 groups: those managed postoperatively with sedation, with or without paralysis (group 1), and those managed awake with narcotic pain medication as needed for primary management (group 2). Outcomes were measured as a function of sedation management. Outcomes investigated focused on those related to the success of the airway reconstruction, and those related to sedation management. RESULTS: Out of 68 cases of laryngotracheal reconstruction reviewed from 2001 to 2011, 34 were single stage reconstructions. Nineteen patients were sedated postoperatively (group 1) and fifteen patients were left awake (group 2). There were no significant differences between groups in airway-related outcomes, including risk of accidental decannulation, revision rates, and need for secondary airway procedures such as balloon dilation. Sedation-related outcomes, specifically focusing on differences in medical management, showed significant increases in rates of withdrawal (p<0.0001), nursing concerns of withdrawal (p<0.0001) and sedation level (p<0.0001), pulmonary complications (OR 7.7, p=0.008), and prolonged hospital stay due to withdrawal (p=0.0005) in patients managed with sedation with or without paralysis. Multivariable regression analysis revealed that duration of sedation was the primary risk factor for increased postoperative morbidity, while younger age, lower weight, and use of a posterior graft were also significant variables assessed. CONCLUSION: Avoiding sedation as the standard for postoperative management of single stage laryngotracheal reconstruction airway patients leads to an overall decreased risk of morbidity without increasing risk of airway-specific morbidity. This is specifically as related to withdrawal, pulmonary complications, concerns about sedation level and prolonged hospital course, all of which increase significantly with increased level and duration of sedation.

Microtia and congenital aural atresia.

Microtia and congenital aural atresia (CAA) are congenital anomalies that are so common that every otolaryngologist should be familiar with the initial evaluation and care of the patient. When one ear hears normally, speech and language development should be normal. The gross and fine motor development of the baby or child is not expected to be affected in isolated cases of microtia and CAA. Current technologies allow for reconstruction or habilitation of the microtic ear when the child is several years of age. The hope is that tissue engineering can eliminate donor site morbidity. Temporary prosthetic ears will remain an option. Aural atresia work continues to be very dependent on the patient anatomy and the need or desire for better hearing in the affected ear.