Severity of sialorrhea and tracheal secretions in infants and toddlers with a tracheostomy with a focus on quality of life.

OBJECTIVE: Sialorrhea and tracheal secretions can be present in patients with a tracheostomy. The current study seeks to survey the severity of sialorrhea and tracheal secretions in infants and toddlers with a tracheostomy, and to correlate this severity with quality of life. METHODS: Prospective cross-sectional survey pilot study: 26 patients (ages 0.33 years - 4.09 years) were given the Infant/Toddler Quality of Life Questionnaire Short Form 47, the Drooling Impact Scale and assessed for severity of tracheal secretions with a Tracheostomy Secretion Severity Scale. Spearman's correlation and Mann Whitney U tests were used to assess correlation between and contributors to quality of life, drooling severity, and tracheostomy secretion severity, number of outpatient visits, and respiratory related hospitalizations. RESULTS: Average health perception quality of life was 46.7 and average parental impact quality of life was 58.85. The average for the Drooling Impact Scale overall was 19.7, for frequency 4.16, for severity 3.12 and for the Tracheostomy Secretion Severity Scale 2.4. There was strong correlation between the Drooling Impact Scale and the Tracheostomy Secretion Severity Scale (R = 0.432 p = 0.03) overall, and moderate correlation between Drooling Impact Scale and Tracheostomy Secretion Severity Scale (R = 0.39956 p = 0.047) frequency. There was no correlation between health perception quality of life or parental impact quality of life and Drooling Impact Scale or Tracheostomy Secretion Severity Scale. CONCLUSION: Drooling severity does not have significant impacts on health perception and parental impact quality of life for infants and toddlers who have a tracheostomy. Overall drooling impact scores and drooling frequency are related to tracheostomy secretion severity.

Quantifying the effect of resident education on outcomes in pediatric appendicitis.

BACKGROUND/PURPOSE: Surgical residents are involved in the care of patients in a climate where quality of care is an important outcome measure. The purpose of this study was to evaluate the effect of resident involvement on appendectomy outcomes. METHODS: We retrospectively reviewed appendectomies, ages 0-18, from January 2016 to December 2018. Operative time, operative charges, and postoperative outcomes were evaluated for cases with and without a resident. Data were analyzed using Wilcoxon rank and Fisher's exact tests. RESULTS: Of 1842 appendectomies (1267 resident present and 575 no resident present), there was no difference in postoperative stay, abscess formation, readmission, or emergency room visits for simple or complex appendicitis. Operative time was significantly longer for cases of simple appendicitis by 10 min (p = <0.0001) and charges significantly higher by $600 (p = <0.0001) when a resident was involved in the case. These differences held true for complex appendicitis (time longer by 9 min, p = <0.0001 and charges higher by $500, p = 0.03). CONCLUSION: Resident involvement results in an increase in operative time and charges, with no difference in length of stay or complications. These results highlight the cost of resident involvement, without an increase in complications experienced by patients. LEVEL OF EVIDENCE: Level III evidence.

Image-guided surgical navigation for bone-conduction hearing device implant placement.

INTRODUCTION: For pediatric patients, bone-conduction hearing devices (BCHD) have demonstrated excellent outcomes. Unique to this population, BCHD implant surgeries can be technically challenging in children due to thinner, developing bone and syndromes with atypical anatomy. Image-guided surgical navigation (IGSN) clarifies underlying skull structure, potentially improving outcomes. IGSN is commonly used in otorhinolaryngologic surgeries, but current use in BCHD placement surgeries remains unprecedented. We report favorable results of IGSN in BCHD implantation for three children with complex otologic anatomy: two syndromic patients with variable temporal bone thickness, and one with prior mastoidectomies. The three patients each underwent a successful hearing implant surgery without significant intra- or post-surgical complications. All patients had good audiologic outcomes. METHODS AND MATERIALS: We report using IGSN to assist in BAHA or BONEBRIDGE™ implant surgery for three medically complicated patients. For stereotactic imaging, the patients each received pre-operative high-resolution CT scans using the paranasal sinus fusion protocol without contrast. The first patient was a 6-year-old male with CHARGE-associated abnormal temporal bone anatomy, atretic left auditory nerve, and bilateral chronic tympanic membrane perforation and otorrhea resulting in bilateral mixed conductive and sensorineural hearing loss. The patient thus was unable to consistently tolerate hearing aids. The second patient was an 18-year-old male with Rosai-Dorfman disease, history of bilateral chronic mastoiditis and middle ear infections, bilateral mastoidectomies, and bilateral malleus and incus removal resulting in mixed conductive and sensorineural hearing loss. The third patient was an 11-year-old male with Treacher Collins Syndrome, bilateral microtia, and bilateral atresia of the external auditory canals resulting in bilateral conductive hearing loss. RESULTS: The patients each underwent a successful hearing implant surgery without significant intra- or post-surgical complications. All patients had good audiologic outcomes. CONCLUSION: Intraoperative IGSN can be a beneficial adjunct to BCHD implant placement surgeries for pediatric patients with abnormal temporal bone anatomy. IGSN can help identify the optimal surgical implantation sites, thereby reducing the risk for major morbidities associated with BCHD implantations. Furthermore, our findings expand application of IGSN use to placement of both BAHA and BONEBRIDGE™.

A case of rare isolated agnathia and literature review.

Agnathia is a rare congenital malformation with unknown etiology characterized by absence of the mandible, microstomia, and tongue aplasia, often found to have other anomalies including holoprosencephaly. The purpose of this paper was to describe the symptoms and imaging of a case of isolated agnathia and to conduct a comprehensive literature review of reported patients with isolated agnathia. Case reports of isolated agnathia are very rare, with most infants as stillborn. We report a child's management of isolated agnathia with microstomia and tongue aplasia. A literature review was performed with focus on diagnosis, airway, and feeding management of isolated agnathia. Polyhydramnios was a common pregnancy complication reported in 25 out of the 39 patients in the case study. Five infants were stillborn, while 23 died within the neonatal period. Of the deceased infants within the neonatal period, 19 died within minutes to hours while four died within days to weeks. There are nine patients with agnathia that survived past infancy. The results of this study suggest that isolated agnathia is a rare malformation which requires a multi-disciplinary approach for airway and feeding management.

Acquisition limitations of bone conduction hearing devices in children with unilateral microtia and atresia.

OBJECTIVE: To characterize the use of bone conduction hearing devices (BCHD) for hearing management in children with unilateral congenital aural atresia (CAA) at a tertiary pediatric center's microtia clinic while assessing challenges in acquisition. BCHD generally provides better audiologic outcomes than atresiaplasty in pediatric CAA. BCHD is formally recommended for only bilateral CAA, although literature has begun to show benefit in unilateral CAA. METHODS: A retrospective case series of patients born between 2014 and 2018 with unilateral microtia at an urban tertiary care children's hospital collected information on demographics, CAA laterality, hearing loss (HL) severity, management, and acquisition. Statistical analysis aided characterization of BCHD use. RESULTS: 131 patients (65% males) were included with a mean age of 3.5 years. 108 (82%) patients with unilateral microtia were used for further analysis, and right sided microtia was most common (67.6%). Of patients with auditory brain response (ABR) testing available, 70% demonstrated conductive HL, 16% mixed HL, 1% sensorineural HL, 6% no HL secondary to grade 1 microtia, and 7% were pending evaluation. Overall, 46 (42.6%) patients with unilateral microtia obtained a BCHD. 68.7% of those offered a BCHD after ABR opted for acquisition. The average length of time from offer to fit was about 6 months. CONCLUSION: Even with thorough consultation and follow up, less than half of the patients with unilateral CAA received a BCHD. Missed initial ABR appointments that lead to BCHD recommendation seem to be the greatest limiting factor, while demographics did not play a confounding role. Family education and future research on obstacles preventing BCHD use in unilateral CAA will help standardize management and improve hearing within this critical auditory period.

Implementation of auricular malformation screenings in the newborn population.

BACKGROUND AND OBJECTIVE: Research has shown that it is important to initiate ear molding early for children with auricular malformations in order to achieve the best results. Currently our institute relies on the traditional primary care physician (PCP) referral system, which does not recognize the time sensitivity of the visit in patients with auricular malformations. The purpose of the current research is to implement a new screening protocol for identifying auricular malformations in the newborn population and thus expedite the clinic visit and necessary intervention. METHODS: The hearing screen technicians (HSTs) were trained to identify some of the most common auricular malformations. A picture guide of 11 types of auricular malformations were given to the HSTs to use as a reference. At the time of the newborn hearing screen, the HSTs examined the pinnas of each baby. When an auricular malformation was identified, the auricular malformation team was immediately alerted and a bedside consultation with ENT occurred. RESULTS: Comparison was made of the referral rate between pre- and post-implementation of the protocol which showed an increased rate of identification (five referrals in the 12-month period pre-implementation versus eighteen referrals in the 15-month period post-implementation). CONCLUSION: We successfully implemented an auricular malformation screening protocol that was linked to newborn hearing screenings. The frequency of identification has increased with the implementation of the new screening protocol and has resulted in earlier initial ENT consultations for ear molding with the goal of improving patient satisfaction and results.

Renal anomalies and microtia: Determining the clinical utility of screening affected children.

OBJECTIVES: Microtia is a congenital ear anomaly that hinders quality of life. Microtia patients, both syndromic and in isolation, may be at-risk for significant renal anomalies that can go undetected at the time of birth. The goal of this study was to characterize the prevalence of renal anomalies among microtia patients at our institution in order to guide optimal patient management. Current guidelines suggest performing a renal ultrasound when there is presence of preauricular pits and ear anomalies in association with dysmorphic features, but not in cases of isolated microtia and atresia. DESIGN: A retrospective review of 237 children with microtia was conducted from 2001 through 2018 at our tertiary-care pediatric institution, of which 98 also had a documented renal ultrasound. Patients were identified as syndromic or non-syndromic. Data endpoints included renal ultrasounds performed, structural anomalies found, and follow-up. RESULTS: Among the 237 patients, 98 had received renal ultrasounds. 12% of the total cohort was found to be syndromic, the most common being Goldenhar. Structural anomalies were detected in 24% of the 98 patients that underwent ultrasound and included disorders such as pelviectasis, renal ectopia, duplicated collecting systems, and renal agenesis. A third of patients with anomalies required follow-up with nephrology for chronic kidney disease or renal failure. Of note, 21% of non-syndromic patients and 43% of syndromic patients screened had an abnormality on ultrasonography. CONCLUSIONS: Children with microtia are at a significant risk of structural renal abnormalities, even when isolated outside of a genetic syndrome. We recommend the strong consideration of performing a screening renal ultrasound in all patients with microtia. Prospective data would be helpful in developing future clinical guidelines regarding the utility of screening ultrasonography.