G-tube placement in patients with robin sequence undergoing mandibular distraction osteogenesis: A multi-institutional review.

The goal of this study was to investigate the relationship between mandibular distraction osteogenesis (MDO) and rates of postoperative gastrostomy tube (G-tube) placement among patients with isolated and syndromic Robin sequence (RS). This study was a multi-institutional retrospective chart review of patients with RS who underwent MDO at one of three different pediatric tertiary medical centers. The primary aim of the study was to compare rates of G-tube placement following MDO among the three institutions. The primary outcome was analyzed using Fischer's exact test. The secondary aim of the study was to assess for other contributing factors to G-tube placement such as demographic differences, length of hospital stay, and age at MDO. Analysis of secondary outcomes was assessed using multiple logistic regression models. A total of 125 patients met the inclusion criteria, which required RS diagnosis, completion of MDO between 2004 and 2019, and adequate medical record availability. Sixty percent (n = 75) of subjects were categorized as isolated RS (iRS) and forty percent (n = 50) as syndromic RS (sRS). After MDO, 20% (n = 25) of all patients had G-tubes placed. Of the iRS group, 14.7% (n = 11) required a G-tube, while 28% (n = 14) of the sRS group required a G-tube. The post-operative G-tube rate was similar between institutions when considering all patients. When considering only those patients with iRS, the post-MDO G-tube rate at one center was significantly higher than the other two. Overall, most patients with RS did not require a G-tube after MDO, regardless of diagnosis. However, the significant differences in rates of G-tube placement among patients with iRS may indicate differing practice philosophies, surgical protocols, thresholds for G-tube placement, or regional influences between institutions.

Reduction of Antibiotic-Associated Conditions After Tympanostomy Tube Placement in Children.

OBJECTIVE: Tympanostomy tube placement has been shown to decrease systemic antibiotics usage in patients with recurrent acute otitis media. Systemic antibiotics in children are associated with an increase in antibiotic-associated conditions (asthma, allergic rhinitis, food allergy, atopic dermatitis, celiac disease, overweight/obesity, attention-deficit hyperactivity disorder [ADHD], autism, learning disability, and Clostridium difficile colonization) later in life. The objective of this study is to estimate whether tympanostomy tube placement is associated with a reduction in antibiotic-associated conditions in children with recurrent acute otitis media (RAOM). METHODS: A retrospective cohort review of electronic medical records from 1991 to 2011 at a large pediatric hospital system was performed identifying 27,584 patients under 18 years old with RAOM, defined by 3 or more episodes of AOM. Antibiotic-associated conditions were defined using ICD-9 and ICD-10 codes. RESULTS: The enrollment population was largely composed of White patients (28.9%), Black patients (30.1%), and Hispanic/Latino patients (16.4%). The number of systemic antibiotics prescribed per encounter was significantly lower in children who pursued tympanostomy tubes (0.14 antibiotics per encounter) versus those who did not (0.23 antibiotics per encounter) (p < 0.001). Patients with RAOM who received tympanostomy tubes were less likely to have diagnoses of overweight/obesity (OR. 0.62 [0.55, 0.68]; p < 0.001), asthma (OR 0.8 [0.74, 0.87]; p < 0.001), allergic rhinitis (OR 0.72 [0.65, 0.81]; p < 0.001), and atopic dermatitis (0.78 [0.71, 0.86]; p < 0.001). CONCLUSIONS AND RELEVANCE: Tympanostomy tube placement is associated with less systemic antibiotic administration and a decreased incidence of overweight/obesity, asthma, allergic rhinitis, and atopic dermatitis in children diagnosed with RAOM. LEVEL OF EVIDENCE: 4 Laryngoscope, 2024.

The Effect of Palatoplasty on Growth.

OBJECTIVES: To assess the effect of palatoplasty on growth velocity in undernourished and nourished orally fed children with cleft palate. METHODS: A retrospective chart review was conducted using a data query with cleft-associated ICD-10 and ICD-9 codes to identify children who underwent cleft palate repair between 2006 and 2022 at a tertiary pediatric hospital. Data gathered included demographics, admission variables, weight gain, and weight percentile as per the WHO growth chart data. Inclusion was limited to patients with complete growth data and without parenteral feeding support. Nutritional status was defined as undernourished (≤3rd percentile) and nourished (>3rd percentile). Between and within-group comparisons were made using nonparametric tests. RESULTS: A total of 192 patients met the study criteria. Among undernourished patients, the median pre- and postsurgical weekly weight gain was 0.09 kg and 0.05 kg (p = <0.0001), and the median growth percentiles were 0.67% and 1.1%, respectively (p = 0.03). Among nourished patients, the median pre- and postsurgical weekly weight gain was 0.12 kg and 0.07 kg (p = <0.0001), and the median growth percentiles were 25.4% and 29.5%, respectively (p = <0.0001). The postsurgical reduction in weight gain per week was similar across nutritional status (p = 0.43), however, the nourished group demonstrated a larger increase in median growth percentile (3.3% vs. 0.23%; p = 0.03). CONCLUSION: This study demonstrates that palatoplasty does not independently improve growth velocity in children who were able to maintain an oral preoperative diet. In the case of undernourished children, there was no clinically significant improvement in postoperative growth percentiles. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

Timing of First Set of Pressure Equalization Tubes in Pediatric Patients With Cleft Deformities.

OBJECTIVES: Children with cleft palates often have comorbid eustachian tube dysfunction requiring pressure equalization tubes (PETs). PETs can relieve middle ear effusions that impede hearing. Ideal PET placement timing in this population is controversial. In this study, the audiologic exam passing rates of patients with cleft palate prior to and following PET insertion were assessed. Rates for patients receiving PETs at different ages were compared. It was hypothesized that earlier PET placement may benefit patients with additional months of improved hearing. METHODS: A retrospective chart review was performed of patients with cleft palate between November 22, 2016 and November 22, 2021 at a tertiary center. Statistical analysis compared passing/normal audiologic exams in patients receiving PETs at different ages. RESULTS: A total of 348 patients had cleft palate diagnoses, received PETs, and had adequate hearing data for inclusion. Those with PETs inserted at 3 months of age or less had an increase in percent of patients passing audiologic exams following versus prior to PET insertion of 13% (1.3 times improvement). Those receiving PETs between 7- and 12-months had the largest rate of improvement (42%) (2.4 times improvement); other groups had changes in passing rates between 31% and 40%. The rate of passing audiologic exams following PET insertion was high across all groups, ranging from 66% to 81%. CONCLUSION: This is one of the first studies exploring the timing of PET placement in this population and showed that patients receiving PETs at 3 months of age or younger passed subsequent audiologic exams at similar rates relative to those receiving PETs later in life. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:3391-3394, 2024.

Children's Visual Perception of Facial Scarring and Cleft Lip Deformity Using Eye Tracking Data.

OBJECTIVE: The purpose of this study is to determine children's perception of secondary cleft lip deformity (SCLD) using objective eye tracking technology and subjective responses on a survey to understand pediatric perceptions of facial scarring. DESIGN: Cross-sectional study of participants aged 5-17 years old. METHODS: Participants viewed images of children's faces with an eye tracking device. Sixteen images were displayed, 12 with unilateral SCLD and four with no facial scarring. Eye tracking data were obtained. Gaze samples were analyzed for areas of interest (AOIs). Immediately after viewing each image, participants answered two survey questions relating to facial asymmetry attitude toward the child pictured. For analysis, participants were divided into age groups. RESULTS: A total of 259 participants were enrolled (42.5% female). Mean age was 10.5 years and 78% identified as White. In all age groups, total fixation time was greater for SCLD compared to control images. Early elementary age children spent significantly less time assessing the nose AOI compared to other groups, and also spent the least total fixation time and had the lowest visit count on all AOIs. Subjective survey questions showed similar trends with elementary age not noticing facial asymmetry compared to older age groups. CONCLUSION: This study demonstrates the successful use of eye tracking technology in children as young as 5 years old. This study suggests that SCLD is perceived as less noticeable in elementary age children and becomes more noticeable to older groups. Understanding peer perception on SCLD from this study may impact decision on revision surgery for SCLD. LEVEL OF EVIDENCE: IV Laryngoscope, 134:2726-2733, 2024.

Risk factors and outcomes in patients with cochlear nerve deficiency.

INTRODUCTION: Cochlear nerve deficiency (CND) is a cause of sensorineural hearing loss made by radiologic criteria. There is sparse literature involving audiological outcomes and cochlear implantation (CI) success in patients with CND. METHODS: A retrospective chart review of all patients with sensorineural hearing loss at a tertiary children's hospital from 2000 to 2020 was conducted. Patients with CND on radiographic imaging were included and categorized as hypoplastic, aplastic, or indeterminate. RESULTS: In this study, 53 patients were identified with CND, totaling 70 ears. Of the 53 patients, 30 (56.6 %) were male, 8 (16.0 %) had a family history of childhood hearing loss, 6 (11.3 %) were born preterm, and 11 (23.4 %) required neonatal intensive care admission. The median maternal age was 29 years old [IQR: 27, 35], and 8 (15 %) patients were born to mothers with diabetes. Of the 70 ears, 49 (70 %) utilized conventional hearing aids, 12 (17.1 %) utilized a bone-anchored hearing aid, and 10 (14.3 %) underwent CI. Of the 10 ears implanted, 4 (40 %) ears had nerves classified as hypoplastic, 3 (30 %) as aplastic, and 3 (30 %) as indeterminate. Improvement in pure tone averages compared to preoperative testing was demonstrated in 8 (80 %), and 6 (60 %) displayed improved speech awareness thresholds. CONCLUSION: This study demonstrates that there may be an association between CND and maternal diabetes and NICU admission. There are variable results with hearing amplification options in patients with CND, and further research is needed to better describe the role of CI, bone-anchored hearing aids and conventional hearing aids in patients with CND.

Head and neck venous thrombosis secondary to pediatric otolaryngologic infection.

OBJECTIVES: Head and neck venous thrombosis is a rare but potentially devastating complication of childhood otolaryngologic infections. This study examines the presentation and management of this condition. METHODS: A retrospective chart review was performed on all pediatric patients with otolaryngologic infections complicated by cranial and cervical venous thrombosis at a tertiary children's hospital from 2007 to 2018. Patient demographics, presentation, site of infection, thrombosis location, implicated pathogen, length of hospital stay, need for surgery, and anticoagulant regimen were assessed. RESULTS: This study included 33 patients (mean age, 7.5 years; age range, 0.8-17 years; 19 [58%] male). The most common infection source was otologic (n = 20), followed by ophthalmic and sinonasal pathology (n = 9), and neck infections (n = 4). The most common site of thrombosis secondary to ear pathology was the sigmoid sinus. The ophthalmic veins were the most common site of thrombosis for ophthalmic/sinonasal infections. Nine CN VI palsies, one CN VII palsy, and one CN III palsy were observed. Twenty-six subjects (79%) required surgical intervention. All those who experienced a nerve palsy required surgery. Length of hospitalization significantly differed with the stay for a neck infection complicated by thrombosis longer compared to otologic and sinonasal infections (F[2,30] = 7.08, p = 0.003). Length of hospital stay was significantly correlated with admission temperature (r = 0.506, p = 0.003) and CRP (r = 0.400, p = 0.03) but not WBC (r = 0.181, p = 0.31). Culture growth predominantly isolated a single causative organism rather than polymicrobial involvement. Forty-eight species were identified, most (n = 41/48, 85%) being Gram-positive bacteria. Alpha-hemolytic Streptococcus was the most common isolate from children with vessel thrombosis secondary to ear infections, with Streptococcus pyogenes predominant in sinonasal infections and Staphylococcus aureus the most common in neck abscesses. There was significant variability in anticoagulation management within the patient population, but no bleeding complications were documented. Most patients had no evidence of underlying thrombophilia (n = 15); for those with positive hypercoagulability screens, the most common positive marker was the presence of lupus inhibitor (n = 6). CONCLUSION: Venous thrombosis resulting from adjacent otolaryngologic infection is a serious complication requiring proper recognition and management. The involved vasculature and cranial nerve findings are dependent on the anatomic location of the underlying infection. Cranial neuropathies in the presence of these infections should prompt evaluation for possible thrombosis.

Update on congenital ear molding.

PURPOSE OF REVIEW: Congenital ear deformities are characterized by distorted, but still present, landmarks of the auricle. Interventions include early treatment with ear molding or delayed surgical treatment. The purpose of this review is to provide an update on the latest information related to ear molding for congenital ear deformities. RECENT FINDINGS: Various ear molding techniques date back to the 1980s, but with the availability of commercially available molding systems, interest and use has blossomed in recent years. As more longitudinal research results are obtained, ear molding has been proven to be a highly effective treatment, although the best technique and length of treatment remains unclear. SUMMARY: While it is clear that earlier intervention is ideal, due to lack of public awareness, patients often present later, and it remains controversial what age to offer ear molding interventions to and still expect success. Ear molding interventions are becoming increasingly covered by insurance due to evidence of preventing long-term psychological morbidities and the need for future surgical interventions.

Pediatric peanut aspirations before and after 2015 recommendation for early peanut exposure.

OBJECTIVES: To investigate if there has been an increase in peanut foreign body aspirations (FBA) in children since the publication of the Learning Early About Peanut Allergy (LEAP) trial, which revealed that early exposure to peanut-containing foods prevented peanut allergies in children at risk of atopic disease. METHODS: Retrospective chart reviews were conducted separately at two pediatric institutions. Institutions One and Two reviewed children less than 7 years old who underwent bronchoscopy for FBA over ten-year periods between January 2007 and September 2017 and November 2008 and May 2018, respectively. The proportion of FBAs attributed to peanuts was compared before and after the publication LEAP. RESULTS: Out of 515 reviewed cases, there was no change in pediatric peanut aspirations prior to and following the LEAP trial and AAP guideline change (33.5% vs 31.4%, p = 0.70). At Institution One, 317 patients met inclusion criteria. When comparing FBAs before and after LEAP, there were no significant changes in the rate of peanut aspiration (53.5% vs. 45.1%, p = 0.17). Institution Two also found no significant increase in the rate of peanut aspirations before and after the Addendum Guidelines (41.4% vs. 28.6%, p = 0.65) upon review of 198 cases. CONCLUSIONS: Multiple institutions demonstrated a non-significant change in the rate of peanut FBAs following the AAP recommendation. Given that peanuts comprise a large proportion of FBAs, it is important to continue to track peanut aspirations. Longer term data tracking is needed from more institutions to further understand how recommendations from other specialties and the media impacts pediatric aspiration outcomes.

Prenatal Consultation Outcomes for Infants With Cleft Lip With and Without Cleft Palate.

To assess the clinical impacts of prenatal consultation with a multidisciplinary cleft team on infants with cleft lip with or without cleft palate (CL ± P).Retrospective cases series.Tertiary pediatric hospital.Infants with CL ± P whose mothers received prenatal consultation with a pediatric otolaryngology team from June 2005 to December 2019 were identified. A random sample of infants with CL ± P without prenatal consultation from June 2005 to December 2019 was also identified.The primary outcomes were the length of hospitalization during the first 12 weeks of life, timing of surgical repair, length of postsurgical hospitalization, and number of unplanned clinic visits and phone calls for feeding evaluation.Time to cleft lip repair differed significantly between the 2 groups with repair performed at 13.4 (±0.9) weeks for the prenatal consultation group (n = 73) and 15.3 (±2.1) weeks for the control group (n = 80), (P < .05). If hospitalization was required for feeding difficulties during the first 12 weeks of life, length of stay was 4.9 (± 1.7) days for infants with prenatal consultation and 11.5 (± 7.2) days for control infants (P < .05). Unplanned clinic visits with a speech-language pathologist (SLP) for feeding difficulties were needed for 2.7% of prenatal consultation infants and 11.3% of control infants (P < .05).Prenatal consultation regarding CL ± P resulted in infants with decreased duration of early hospitalizations, earlier cleft lip repair, and decreased engagement with the SLP feeding clinic for feeding difficulties when compared with infants without prenatal consultation.

Formula Otorrhea in Pediatric Patients With Cleft Palate.

The case series details 2 unusual cases of male newborns with cleft lip and palate (CLP) that later developed formula otorrhea. Both patients underwent bilateral myringotomies with the insertion of pressure equalizing (PE) tubes for chronic otitis media with effusion (OME). Chronic otorrhea associated with feeding occurred post-PE tube insertion and the otorrhea was later confirmed to be due to reflux of formula. Patients were treated with antibiotic ear drops, routine ear cleaning, anti-reflux medication, and reflux precautions. After definite cleft palate repair, formula otorrhea completely resolved. When patients with CLP develop chronic OME or otorrhea following PE tube placement, reflux of formula into the middle ear should be considered and treated accordingly.

Unplanned Intensive Care Unit Admission Following Cleft Palate Repair by Head and Neck Surgeons.

OBJECTIVE: To examine the incidence of pediatric intensive care unit (PICU) admission following primary repair of cleft palate by otolaryngologist-head and neck surgeons at 2 tertiary centers. To identify potential diagnoses associated with admission or unanticipated PICU transfer. STUDY DESIGN: Multi-institutional case series with chart review. SETTING: Two tertiary pediatric medical centers. METHODS: Children who underwent primary repair of cleft palate at 2 cleft centers over a 10-year period were identified. Charts were reviewed for demographics, comorbidities, and whether PICU admission was required. RESULTS: From 2009 to 2019, 464 patients underwent primary repair of a cleft palate by 1 of 6 otolaryngologist-head and neck surgeons with subspecialty training in cleft surgery; 459 patients had sufficient postoperative documentation and 443 children met inclusion criteria. The incidence of PICU admission was 9.3% (41/443), with 33 (7.4%) planned admissions and 8 (1.8%) unexpected PICU transfers. Syndromic conditions were associated with both planned and unanticipated PICU admissions. CONCLUSION: The incidence of unanticipated postoperative PICU admission following cleft palate repair by otolaryngologist-head and neck surgeons was low. Risk stratification by surgeons with expertise in airway management may inform decisions regarding postoperative disposition of patients with medical or airway complexity who are undergoing cleft palate repair.

Oral Premalignant and Malignant Lesions in Fanconi Anemia Patients.

OBJECTIVE: There is a lack of data supporting cancer surveillance in pediatric Fanconi Anemia patients. We sought to describe the rates of upper aerodigestive lesions and malignancy in this population to augment current management guidelines. METHODS: A retrospective cohort study of patients with Fanconi Anemia from a quaternary referral center between 2007-2021 was completed for head and neck cancer risk. RESULTS: One hundred and five FA patients were reviewed. Average age at presentation was 11.3 years old and 90.5% of patients underwent hematopoietic stem cell transplant (HSCT). A total of 8.6% of patients had leukoplakia or erythroplakia and 3.8% developed malignancy. The standardized incidence ratio of head and neck malignancy was 483.8. Patients presented with leukoplakia and malignancy at an average age of 14.6 and 25.1 years old, respectively. Malignancies were aggressive and marked by recurrence. There were no premalignant or malignant lesions found on flexible laryngoscopy. This series represents the largest longitudinal series of pediatric FA head and neck lesions. CONCLUSIONS: Fanconi Anemia patients should begin screening for head and neck cancer at age 10 or after HSCT. LEVEL OF EVIDENCE: Level 4 Laryngoscope, 133:1745-1748, 2023.

Pediatric Otolaryngology fellowship virtual interview sentiments.

BACKGROUND: Interviews for Pediatric Otolaryngology fellowship rapidly transitioned to virtual interviews mid-cycle in March 2020 due to the COVID-19 pandemic. OBJECTIVE: This study aims to describe perspectives on virtual versus in-person interviews for both applicants and program directors. METHODS: Cross-sectional study. Surveys were conducted of all Pediatric Otolaryngology fellowship applicants participating in the San Francisco Match and program directors in 2020 and 2021. RESULTS: Out of 32 U.S. trained fellowship applicants, 24 completed the survey in 2020 and 18 in 2021. While 70% of applicants felt they did not get the same experience with virtual interviews, 75% did not feel it changed how they ranked programs. Applicant perception of virtual interviews improved in 2021, with the majority (56%) preferring virtual interviews if provided an option. Twenty out of 36 fellowship directors completed the survey in 2020, and eighteen in 2021. While fellowship directors continued to prefer in-person in 2021, an increased number (10% in 2020, 30% in 2021) felt continuing with virtual interviews may increase the number of applicants in the future. CONCLUSION: Based on the survey, both applicants and fellowship directors had a less favorable perception of virtual interviews compared to in-person interviews initially; however, applicant perception favored virtual interviews in 2021, while Program Directors continued to prefer in-person.

The Incidence of Velopharyngeal Insufficiency in Stickler Syndrome.

OBJECTIVE: Stickler Syndrome (SS) is an inherited collagenopathy characterized by heterogenous orofacial, ocular, auditory, and skeletal abnormalities. The orofacial manifestations are variable and some patients present with cleft palate and velopharyngeal insufficiency (VPI). The incidence of VPI in SS is poorly studied and no studies have compared the incidence of VPI between Type I (COL2A1) and Type II (COL11A1) SS. The objective of this study is to compare the incidence of VPI between SS subtypes and discuss the surgical techniques used to treat them. DESIGN: Single-institution, retrospective chart review. SETTING: Tertiary pediatric hospital. PATIENTS/PARTICIPANTS: Forty-three children were diagnosed with SS between January 2003 and December 2018. Genetic testing results, genetics notes, craniofacial clinic notes, and operative reports were reviewed. Patients without genetic testing or craniofacial/otolaryngologic evaluation were excluded. Thirty-one patients met criteria and were included. MAIN OUTCOME MEASURE: Primary outcome was VPI incidence. RESULTS: There were 18 patients with Type I SS and 13 with Type II SS. Five (16%) patients had VPI, 2 (11%) with Type I SS compared to 3 (23%) with Type II SS (P > .05). All patients with VPI underwent surgery with either sphincter pharyngoplasty (3) or pharyngeal flap (2). Two patients with Type II SS underwent revision sphincter pharyngoplasty, with one conversion to pharyngeal flap. CONCLUSION: VPI is common for patients with SS. In this study, there was no significant difference in the incidence of VPI between SS subtypes. Future studies are needed to confirm these findings, which could be important for patient counseling and treatment planning.

A national pediatric otolaryngology fellowship virtual dissection course using 3D printed simulators.

OBJECTIVE: Our objective was to create and evaluate a novel virtual platform dissection course to complement pediatric otolaryngology fellowship training in the setting of the COVID-19 pandemic. METHODS: A four-station, four-simulator virtual course was delivered to pediatric otolaryngology fellows virtually using teleconferencing software. The four stations consisted of microtia ear carving, airway graft carving, cleft lip repair, and cleft palate repair. Fellows were asked to complete pre- and post-course surveys to evaluate their procedural confidence, expertise, and attitudes towards the course structure. RESULTS: Statistical analysis of pre-course survey data showed fellows agreed that simulators should play an important part in surgical training (4.59 (0.62)); would like more options for training with simulators (4.31 (0.88)); and would like the option of saving their simulators for later reference (4.41 (0.85)). Fellows found the surgical simulators used in the course to be valuable as potential training tools (3.96 (0.96)), as competency or evaluation tools (3.91 (0.98)), and as rehearsal tools (4.06 (0.93)). Analysis showed a statistically significant improvement in overall surgical confidence in performing all four procedures. CONCLUSION: This virtual surgical dissection course demonstrates 3D printed surgical simulators can be utilized to teach fellows advanced surgical techniques in a low-risk, virtual environment. Virtual platforms are a viable, highly-rated option for surgical training in the setting of restricted in-person meetings and as a mechanism to increase access for fellows by reducing costs and travel requirements during unrestricted periods.

Etiologies of hearing loss in Fanconi Anemia.

OBJECTIVES: We aim to describe types of hearing loss associated with Fanconi anemia patients who underwent a bone marrow transplant (BMT) to identify possible etiologies of hearing loss. Additionally, we hope to investigate hearing loss early in life as a potential predictor of needing a BMT surgery. Fanconi anemia is a rare autosomal recessive disease that is the most common inherited bone marrow failure syndrome, characterized by bone marrow failure and multiple congenital anomalies, including hearing loss. This is the largest study to date reviewing types of hearing loss in patients with Fanconi anemia, specifically in those who have undergone BMTs. METHODS: A retrospective chart review of patients diagnosed with Fanconi anemia at a single institution, tertiary, referral-based children's hospital with a bone marrow transplant team specializing in Fanconi anemia was conducted from 4/19/1976 to 10/19/2015. History, physical examination, audiometry, and imaging findings were reviewed in patients with and without history of bone marrow transplant. Patient hearing levels, as measured by pure tone audiometry at 500 Hz, 1, 2, and 4 kHz, were evaluated. Patients were grouped by transplant status and results and were assessed to determine type and degree of hearing loss. Statistical analysis was performed to compare the likelihood of bone marrow transplant procedures in Fanconi anemia patients with normal and abnormal hearing. RESULTS: There were 252 patients with Fanconi anemia identified via diagnosis search in institutional electronic medical records using CPT codes and cross referencing with the Fanconi Anemia database, 58 of whom had available audiometric data. Of the 58 Fanconi anemia patients with available audiograms, 21 (36%) had abnormal audiograms; 37 patients had normal audiograms. Twenty out of 21 (95%) patients who had abnormal audiograms had undergone bone marrow transplants. Thirty-one of 37 (84%) patients with normal audiograms had received bone marrow transplants. Statistical analysis showed that patients with hearing loss were more likely to require a BMT in the future (OR = 3.87, p = 0.05). Of the patients with abnormal audiograms and a bone marrow transplant (n = 20), 14 (70%) had conductive hearing loss, 5 (25%) had mixed hearing loss, and 1 patient (5%) had sensorineural hearing loss. 13 of 20 patients (65%) had bilateral hearing loss and eight of 20 (40%) had unilateral hearing loss. Of those patients with conductive hearing loss (n = 15), the most common etiologies were Eustachian tube dysfunction (47%), external auditory canal stenosis (33%), and abnormal middle ear anatomy (13%). CONCLUSIONS: Hearing loss is a common finding in Fanconi anemia patients who have undergone BMTs with conductive hearing loss being the most common audiologic manifestation in our cohort of patients. This demonstrates the necessity of frequent hearing screenings in this population and close collaboration with audiology throughout patient care. Our study indicates that hearing status early in life may be a predictor of needing a bone marrow transplant in the future. Further studies should explore the long-term impact of BMT surgery on hearing status.

The Safety and Efficacy of Mandibular Distraction Osteogenesis Among Infants Less Than 3 kg.

OBJECTIVES/HYPOTHESIS: Mandibular distraction osteogenesis (MDO) is a safe and effective surgery to address respiratory and feeding issues due to micrognathia in patients with Robin Sequence (RS). Previous studies examining postoperative complications in neonates receiving MDO have considered 4 kg as the cut-off for low weight; however, an increasing number of MDO interventions are performed in infants <4 kg. To determine if a weight <3 kg at time of MDO is a risk factor for postoperative complications or need for subsequent tracheostomy or gastrostomy tube (G-tube). STUDY DESIGN: Retrospective chart review. METHODS: A retrospective review of all infants <6 months of age undergoing MDO at two tertiary pediatric hospitals from 2008 to 2018. Demographic data, syndromic status, weight, and age at time of surgery, length of postoperative hospital stay, and postoperative outcomes were recorded including tracheostomy placement, G-tube placement, hardware infection, reintubation, facial/marginal mandibular nerve damage, and need for revision MDO. RESULTS: Sixty-nine patients with RS were included. The mean age at MDO was 25 ± 20 days and mean weight was 3.32 ± 0.44 kg. There was no statistically significant correlation between weight (P = .699) or age (P = .422) and unfavorable postoperative outcomes. No patients (0%) underwent tracheostomy pre-MDO. Two patients (2.9%) required tracheostomy postsurgery; neither was <3 kg. Eight patients (11.6%) required a G-tube postoperatively. CONCLUSION: Newborns <3 kg who undergo MDO experience the same rates of success and complication as larger infants, suggesting that MDO is a safe and efficacious procedure in infants less than 3 kg. Laryngoscope, 132:1295-1299, 2022.