Use of Topical Phenol in Awake Young Children for Tympanostomy Tube Placement.

Importance: Phenol kits cleared by the Food and Drug Administration (FDA) are indicated as a topical anesthetic for the tympanic membrane (TM) in adults. However, there is no existing literature that reports outcomes to support the safety and use of phenol on the TM of awake children. Objective: Determine if topical phenol is safe and at low risk for complications and therefore be used effectively in awake children to facilitate office otologic procedures as in adults. Design, Setting, and Participants: Children under 21 years of age whose parents agreed to participate in an awake office setting for tympanostomy tube (TT) placement. All children had TT placement after phenol placement on the TM prior to insertion. Main Outcomes and Measures: TM perforation or other signs of TM complications through a minimum of 6-month clinical follow-up, along with assessment of the tolerability of the procedure by the child. Results: A total of 228 children with an age range of 6 months to 15.9 years and 435 TMs completed TT placement using phenol as a local anesthetic while awake in the office. There were no complications reported in the 204 children at the first follow-up visit post TM placement within 3 to 10 weeks. Of the 93 children followed up at least 6 months, there were no TM complications reported. Conclusions: This is the first study to report the outcomes on the use of phenol in an office setting in children. In this large experience, phenol appears to be tolerable and safe for use in young children in the office and is a potential safe choice of topical anesthesia for surgeons if they choose to perform office procedures such as myringotomies or TT placement on children.

Triple Layer Oronasal Fistula Repair Using Local Endonasal Flaps: Case Series of Three Patients.

Oronasal fistula (ONF) is a common complication encountered after palatoplasty. Repair is indicated when symptoms impact speech and swallowing. In spite of the variety of surgical approaches described to repair these defects, recurrence rates remain high. Traditionally, successful closure is said to be achieved in using a double-layered approach due to the three-dimensional aspect of the defect. The extent of the fistula into the nasal cavity has incited an increased curiosity in using local endonasal flaps. In recent years, endonasal reconstructive procedures have seen increased interest and application, from cranial base defect repairs to orbital reconstruction and beyond. The nasoseptal (NSF) and inferior turbinate flaps (ITF) possess a robust arterial supply and an exceptional reach with excellent results demonstrated in large defect repair. However, the use of these flaps in ONF repair is scarcely discussed in the literature, and their effectiveness is relatively undetermined. In this manuscript, we present a series of three patients who underwent a triple layer ONF closure, with the oral portion incorporating a turn-in mucosal flap plus a local palate rotation flap or greater palatine artery pedicled-rotation flap, and a NSF or an ITF for the nasal portion of the defect.

The Clinical Utility of Continuous Laryngoscopy During Exercise: A Report of Two Cases.

Exertional dyspnea is a common and disabling symptom in otherwise healthy children and adolescents, as well as in children with baseline airway abnormalities. It impairs the quality of life and may be associated with fatigue and underperformance in sports. Exertional dyspnea can be caused by a wide variety of structural and psychogenic causes. Exercise-induced laryngeal obstruction (EILO) is a relatively prevalent entity in young people that usually presents with exertional stridor, coughing, and dyspnea caused by transient closure of the larynx. In more complex cases where conventional tests such as pulmonary function tests (PFTs), chest imaging, ECG, and echocardiography are unrevealing, continuous laryngoscopy during exercise (CLE) tests may provide diagnostic utility. In addition to the baseline abnormalities visualized by conventional laryngoscopy, CLE can assess dynamic laryngeal responses during exercise. This article describes the clinical characteristics of two pediatric patients with various degrees of laryngeal dysfunction at baseline and the utility of CLE testing in tailoring management strategies.

Telehealth as an effective method of follow-up for pediatric post tonsillectomy patients.

PURPOSE: To determine if pediatric patients can be safely and effectively managed postoperatively with nurse led telehealth communication. METHODS: This is a retrospective case series conducted at a tertiary academic medical center pediatric otolaryngology practice. Retrospective chart review was conducted on patients <18 years old who underwent tonsillectomy from January 2017 to December 2019. Patients were managed postoperatively with a telehealth communication on postoperative day (POD) 3-5 and again at 4-6 weeks. Patient demographics, satisfaction with follow-up, number of office visits, and postoperative complications were recorded. RESULTS: 829 tonsillectomy patients were identified. Average patient age was 5.7 years (range 10 months-16 years). Successful contact was made with the patient's caregiver on POD 3-5 for 511 patients. 322 patients successfully completed 4-6 weeks telehealth follow-up. 292 patients (91 %) reported improvement in pre-operative symptoms at 4-6 weeks. Overall, 98 % of patients who completed telehealth follow-up were satisfied with this method and did not desire an additional office appointment. 62 patients (21 %) participated in an office follow-up in 2017, 54 patients in 2018 (19 %), and 36 patients (14 %) in 2019. Only 61 of these visits were routinely scheduled postoperative tonsillectomy office visits. 53 patients (6 %) had a postoperative tonsillectomy bleed and 31 patients (4 %) required return to the operating room for cauterization. CONCLUSION: Telehealth is successful in reducing the number of post-tonsillectomy office visits for pediatric patients without a subsequent increase in complications. Reduction in office visits can lead to cost reduction and increased availability of pediatric otolaryngology appointments.

Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus recommendations.

BACKGROUND: Cystic fibrosis (CF) is a multisystem disease that often requires otolaryngology care. Individuals with CF commonly have chronic rhinosinusitis but also present with hearing loss and dysphonia. Given these manifestations of CF, otolaryngologists are frequently involved in the care of patients with CF; however, there is limited consensus on optimal management of sinonasal, otologic, and laryngologic symptoms. METHODS: The Cystic Fibrosis Foundation convened a multidisciplinary team of otolaryngologists, pulmonologists, audiologists, pharmacists, a social worker, a nurse coordinator, a respiratory therapist, two adults with CF, and a caregiver of a child with CF to develop consensus recommendations. Workgroups developed draft recommendation statements based on a systematic literature review, and a ≥80% consensus was required for acceptance of each recommendation statement. RESULTS: The committee voted on 25 statements. Eleven statements were adopted recommending a treatment or intervention, while five statements were formulated recommending against a specific treatment or intervention. The committee recommended eight statements as an option for select patients in certain circumstances, and one statement did not reach consensus. CONCLUSION: These multidisciplinary consensus recommendations will help providers navigate decisions related to otolaryngology consultation, medical and surgical management of CF-CRS, hearing, and voice in individuals with CF. A collaborative and multidisciplinary approach is advocated to best care for our patients with CF. Future clinical research is needed utilizing standardized, validated outcomes with comprehensive reporting of patient outcome, effects of modulator therapies, and genetic characteristics to help continue to advance care, decrease morbidity, and improve the quality of life for individuals with CF.

Feasibility of an Innovative Absorbable Ventilation Tube Designed to Provide Intermediate-Term Middle Ear Ventilation.

Myringotomy with ventilation tube placement is a common surgical procedure performed in children and adults to remove fluid buildup behind the tympanic membrane. However, retention of tubes beyond achievement of therapeutic response increases risk for complications and additional intervention. This small feasibility study was conducted to demonstrate proof of concept of a novel bioabsorbable ventilation tube that provides the necessary duration of ventilation with absorption shortly thereafter. Tubes were placed in 15 ears of 14 patients meeting indications for short or intermediate duration of middle ear ventilation. Two independent examiners documented tube patency and tube absorption status at 3, 6, and 12 weeks or until absorption was complete. Results indicate that average ventilation time was 12 weeks (range, 3 weeks to 18 months). There was no observation of blockage. These findings support the feasibility of a novel bioabsorbable ventilation tube.

Assessing Pediatric Feeding Disorders by Domain in Complex Aerodigestive Patients.

OBJECTIVE: Pediatric feeding disorder (PFD) is defined as impaired oral intake, associated with dysfunction in at least one of four domains: medical, nutritional, feeding skill, and/or psychosocial. The pediatric aerodigestive patient presents with conditions impacting airway, breathing, feeding, swallowing, or growth. The objective of the study was to determine the prevalence of PFD and dysfunctional domain, in the aerodigestive patient presenting to a tertiary aerodigestive clinic. METHODS:  Twenty-five charts from patients enrolled in Mayo Clinic Children's Center Aerodigestive Program were retrospectively reviewed for documentation of dysfunction within the four feeding disorder domains. Results from the aerodigestive triple scope, functional endoscopic evaluation of swallow (FEES), and videofluoroscopic swallow study (VFSS) were recorded. Height and weight z-scores were compared between the initial assessment and 6-12 months later. RESULTS: Median age was 20 months (range 2-81 months). Of the patients, 100% (n = 25) had dysfunction in at least one PFD domain. The domain identified most frequently was medical dysfunction (96%; n = 24). Feeding dysfunction was observed in 76% (n = 19). Psychosocial dysfunction was observed in 76% (n = 19). Nutritional dysfunction was observed in 60% (n = 15). Dysfunction in three or greater domains was seen in 80% (n = 20). Weight z-score increased in 76% (n = 19) of patients 6 to 12 months after the initial aerodigestive evaluation. CONCLUSION:  Aerodigestive patients frequently have PFD and utilizing the consensus definition of PFD at intake may enhance clinical assessment and therapeutic evaluation, and provide a framework to measure outcomes in this heterogeneous patient population.

In-office insertion tympanostomy tubes in children using single-pass device.

OBJECTIVES: Insertion of tympanostomy tubes (TT) is generally accomplished in children in the operating room under general anesthesia. We report on 229 children treated in-office with a novel device. METHODS: Investigators participated in an IRB-approved, prospective, single arm, multisite investigation of in-office TT placement in awake children. Topical anesthetic was applied, and protective restraint was used. TT placement was performed with a single-pass TT insertion device. Safety was assessed by monitoring procedural events. RESULTS: Four hundred and forty-four ears were treated in 229 children at 10 sites. Children were in age groups 6-24 months (n = 211, mean = 13 months) and 5-12 years (n = 18, mean = 8.3 years). Two hundred and fifteen children received bilateral TT placement, and 14 received unilateral placement. Overall, 226/229 (98.7%) children had successful TT placement in the office (209/211 in 6-24 months and 17/18 in 5-12 years). Three children were rescheduled for the operating room due to anatomical challenges or patient movement. Median procedure time for bilateral cases in both age groups was 4:53. Two minor adverse events (AEs) were reported in one patient. Per independent assessment of 30 procedure videos by clinicians, TT placement was tolerated acceptably by all children. CONCLUSION: In-office TT placement in awake young children using topical anesthetic, enabled by a single pass delivery device, was safe, successful and well tolerated. The American Academy of Otolaryngology (AAO) recently released a Position Statement supporting in-office TT placement in appropriate children. These results affirm an in-office alternative for clinicians and parents who have concerns with the risk, inconvenience and cost of surgery in an operating room under general anesthesia.Level of Evidence: 2c.Clinical Trials Registration Number: NCT03544138.

Endotracheal metallic stent removal: A novel ABC (airway balloon collapse) technique.

To illustrate a previously unreported method of tracheal stent removal that appears to cause less mucosal injury we present a case of a 9-year-old Down syndrome patient with a history of tracheoesophageal fistula, brought to our attention after recurrent bouts of exacerbating cough and tracheo-bronchitis. Endoscopic examination under general anesthesia noted the presence of severe tracheomalacia with inspiratory collapse, and a 10-mm balloon expandable metallic stent (BEMS) was deployed and symptomatic improvement was noted. The initial stent was then removed to consider a definitive procedure using the typical grasping fashion with an alligator forceps and expected mucosal excoriation was noted. Due to symptom recurrence, the patient underwent placement of a second BEMS stent. Initial improvement was noted followed by recurrent episodes of respiratory distress due to granulation tissue formation and stent compression and a decision to remove the stent was made. A new method of stent removal deemed ABC (airway balloon collapse) method was utilized where an expandable airway balloon is placed outside the stent between the stent and tracheal wall and then inflated to collapse the stent, facilitating easy removal.

Innovative 2-Step Management Strategy Utilizing EXIT Procedure for a Fetus With Hypoplastic Left Heart Syndrome and Intact Atrial Septum.

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (HLHS-IAS) carries a high risk of mortality and affects about 6% of all patients with HLHS. Fetal interventions, postnatal transcatheter interventions, and postnatal surgical resection have all been used, but the mortality risk continues to be high in this subgroup of patients. We describe a novel, sequential approach to manage HLHS-IAS and progressive fetal hydrops. A 28-year-old, gravida 4 para 2 mother was referred to Mayo Clinic for fetal HLHS. Fetal echocardiography at 28 weeks of gestation demonstrated HLHS-IAS with progressive fetal hydrops. The atrial septum was thick and muscular with no interatrial communication. Ultrasound-guided fetal atrial septostomy was performed with successful creation of a small atrial communication. However, fetal echocardiogram at 33 weeks of gestation showed recurrence of a pleural effusion and restriction of the atrial septum. We proceeded with an Ex uteroIntrapartum Treatment (EXIT) delivery and open atrial septectomy. This was performed successfully, and the infant was stabilized in the intensive care unit. The infant required venoarterial extracorporeal membrane oxygenator support on day of life 1. The patient later developed hemorrhagic complications, leading to his demise on day of life 9. This is the first reported case of an EXIT procedure and open atrial septectomy performed without cardiopulmonary bypass for an open-heart operation and provides a promising alternative strategy for the management of HLHS-IAS in select cases.

Multicenter Interrater Reliability in the Endoscopic Assessment of Velopharyngeal Function Using a Video Instruction Tool.

OBJECTIVE: Assess interrater agreement of endoscopic assessment of velopharyngeal (VP) function before and after viewing the video instruction tool (VIT). We hypothesized improvement in interrater agreement using the Golding-Kushner scale (GKS) after viewing the VIT. STUDY DESIGN: Prospective study. SETTING: Multi-institutional. METHODS: Sixteen fellowship-trained pediatric otolaryngologists who treat velopharyngeal insufficiency (VPI) rated 50 video segments using the GKS before and after watching the VIT. Raters assessed gap size percentage and lateral pharyngeal wall (LPW), soft palate (SP), and posterior pharyngeal wall (PPW) movement. Intraclass correlation coefficient was estimated for these continuous measures. Raters also indicated the presence of a palatal notch, Passavant's ridge, and aberrant pulsations (categorical variables). Fleiss κ coefficient was used for categorical variables. Wilcoxon signed-rank test was performed on the difference between the pre/post individual video ratings. RESULTS: Reliability improved for all continuous variables after watching the instructional video. The improvement was significant for PPW (0.22-0.30, P < .001), SP (left: 0.63-0.68, P < .001 and right: 0.64-0.68, P = .001), and LPW (left: 0.49-0.54, P = .01 and right: 0.49-0.54, P = .09) but not significant for gap size (0.65-0.69, P = .36). Among categorical variables, agreement on Passavant's ridge significantly improved (0.30-0.36, P = .03). CONCLUSION: Exposure to a video instruction tool improves interrater agreement of endoscopic assessment of VP function. Significant improvement was observed in our primary end points, specifically posterior pharyngeal wall movement, soft palate movement, and lateral pharyngeal wall movement. There was less impact of the VIT on the interrater agreement of the categorical variables, palatal notch, Passavant's ridge, and aberrant pulsations.

Outcome measures for pediatric laryngotracheal reconstruction: International consensus statement.

OBJECTIVES: Develop multidisciplinary and international consensus on patient, disease, procedural, and perioperative factors, as well as key outcome measures and complications, to be reported for pediatric airway reconstruction studies. METHODS: Standard Delphi methods were applied. Participants proposed items in three categories: 1) patient/disease characteristics, 2) procedural/intraoperative/perioperative factors, and 3) outcome measures and complications. Both general and anatomic site-specific measures were elicited. Participants also suggested specific operations to be encompassed by this project. We then used iterative ranking and review to develop consensus lists via a priori Delphi consensus criteria. RESULTS: Thirty-three pediatric airway experts from eight countries in North and South America, Europe, and Australia participated, representing otolaryngology (including International Pediatric Otolaryngology Group members), pulmonology, general surgery, and cardiothoracic surgery. Consensus led to inclusion of 19 operations comprising open expansion, resection, and slide procedures of the larynx, trachea, and bronchi as well as three endoscopic procedures. Consensus was achieved on multiple patient/comorbidity (10), disease/stenosis (7), perioperative-/intraoperative-/procedure-related (16) factors. Consensus was reached on multiple outcome and complication measures, both general and site-specific (8 general, 13 supraglottic, 15 glottic, 17 subglottic, 8 cervical tracheal, 12 thoracic tracheal). The group was able to clarify how each outcome should be measured, with specific instruments defined where applicable. CONCLUSION: This consensus statement provides a framework to communicate results consistently and reproducibly, facilitating meta-analyses, quality improvement, transfer of information, and surgeon self-assessment. It also clarifies expert opinion on which patient, disease, procedural, and outcome measures may be important to consider in any pediatric airway reconstruction patient. LEVEL OF EVIDENCE: 5 Laryngoscope, 129:244-255, 2019.

Interdisciplinary aerodigestive care model improves risk, cost, and efficiency.

OBJECTIVE: This study sought to evaluate the impact of an interdisciplinary care model for pediatric aerodigestive patients in terms of efficiency, risk exposure, and cost. METHODS: Patients meeting a standard clinical inclusion definition were studied before and after implementation of the aerodigestive program. RESULTS: Aerodigestive patients seen in the interdisciplinary clinic structure achieved a reduction in time to diagnosis (6 vs 150 days) with fewer required specialist consultations (5 vs 11) as compared to those seen in the same institution prior. Post-implementation patients also experienced a significant reduction in risk, with fewer radiation exposures (2 vs 4) and fewer anesthetic episodes (1 vs 2). Total cost associated with the diagnostic evaluation was significantly reduced from a median of $10,374 to $6055. CONCLUSION: This is the first study to utilize a pre-post cohort to evaluate the reduction in diagnostic time, risk exposure, and cost attributable to the reorganization of existing resources into an interdisciplinary care model. This suggests that such a model yields improvements in care quality and value for aerodigestive patients, and likely for other pediatric patients with chronic complex conditions.

Observational study of laryngoscopy plus flow-volume loops during exercise.

Laryngoscopy is the gold standard to diagnose exercise-induced laryngeal obstruction, though inspiratory flow-volume loop may provide a clue. We combined tidal flow-volume loop analysis plus laryngoscopy during exercise and found that cigar-shaped - not flattened - inspiratory loops are associated with obstruction. Pursed-lip breathing slows inhalation thereby reducing vocal fold adduction.

Impact of age on the anatomy of the pediatric pterygopalatine fossa and its relationship to the suprazygomatic maxillary nerve block.

OBJECTIVES: The suprazygomatic maxillary nerve block is associated with improved post-operative pain management after select craniofacial surgical procedures. This study's objective is to better define the impact of pediatric facial skeletal growth on techniques for accessing the pterygopalatine fossa (PPF). METHODS: Pediatric patients with prior thin-slice maxillofacial computed tomography imaging were identified in an institutional radiology database. Aquarius image-processing software (Ver. 4.4.11, TeraRecon, Inc., Foster City, CA) was used to measure from the suprazygomatic skin to the greater wing of the sphenoid where the needle is then re-oriented in an anterior and inferior trajectory allowing it to advance into the PPF. RESULTS: A total of 90 patients ranging from 0 to ≤18 years of age were included in the study. The mean distance from the suprazygomatic skin to the foramen rotundum in patients 0 to ≤12 months of age and >13 to ≤18 years of age was 38.6 (SD ±â€¯4.7) and 47.1 (SD ±â€¯3.2) mm, respectively (p < .0001). The statistical analysis demonstrated a positive correlation between age in years and all of the measured distances (p = .0001). With respect to the plane of the needle entry site, the anterior and inferior angles required for passage into the PPF in the 0 to ≤12 months age group were 11 (SD ±â€¯2.1) and 9.0 (SD ±â€¯2.5) degrees, respectively, compared to those in the >13 to ≤18 years of age group at 12.4 (SD ±â€¯1.9) and 12.1 (SD ±â€¯3.2) degrees, respectively. These data reveal that patients in the oldest compared to the youngest pediatric age groups require significantly greater needle insertion, yet the angles of needle re-orientation are clinically similar between these two pediatric age groups varying by up to only 3°. CONCLUSION: As expected, the distance from the skin to the foramen rotundum increases significantly with age; however, the angles of re-orientation with respect to the original needle entry site demonstrated up to only 3° of variability between the youngest and oldest age groups evaluated in this pediatric cohort.

Structure and Functions of Pediatric Aerodigestive Programs: A Consensus Statement.

Aerodigestive programs provide coordinated interdisciplinary care to pediatric patients with complex congenital or acquired conditions affecting breathing, swallowing, and growth. Although there has been a proliferation of programs, as well as national meetings, interest groups and early research activity, there is, as of yet, no consensus definition of an aerodigestive patient, standardized structure, and functions of an aerodigestive program or a blueprint for research prioritization. The Delphi method was used by a multidisciplinary and multi-institutional panel of aerodigestive providers to obtain consensus on 4 broad content areas related to aerodigestive care: (1) definition of an aerodigestive patient, (2) essential construct and functions of an aerodigestive program, (3) identification of aerodigestive research priorities, and (4) evaluation and recognition of aerodigestive programs and future directions. After 3 iterations of survey, consensus was obtained by either a supermajority of 75% or stability in median ranking on 33 of 36 items. This included a standard definition of an aerodigestive patient, level of participation of specific pediatric disciplines in a program, essential components of the care cycle and functions of the program, feeding and swallowing assessment and therapy, procedural scope and volume, research priorities and outcome measures, certification, coding, and funding. We propose the first consensus definition of the aerodigestive care model with specific recommendations regarding associated personnel, infrastructure, research, and outcome measures. We hope that this may provide an initial framework to further standardize care, develop clinical guidelines, and improve outcomes for aerodigestive patients.

Trans-nasal flexible bronchoscopy in wheezing children: Diagnostic yield, impact on therapy, and prevalence of laryngeal cleft.

AIM: Persistent or recurrent wheezing is a common indication for flexible bronchoscopy, as anatomic and infectious or inflammatory changes are highly prevalent. We sought to evaluate the prevalence of anatomic, infectious, and inflammatory disease in a cohort of children undergoing flexible bronchoscopy for wheezing or poorly controlled asthma. METHODS: We retrospectively reviewed all children <18 years old who underwent flexible bronchoscopy at our center from October 29, 2012-December 31, 2016 for the primary or secondary indication of wheezing (persistent, frequently recurring, or atypical) or poorly controlled asthma. RESULTS: A total of 101 procedures were identified in 94 patients, aged 3 months to 18 years. Potential anatomic causes for wheezing identified in 45.7% of patients and inflammatory changes in 49.5% of procedures. This included the identification of a laryngeal cleft in 17% for which half required medical or surgical management. Tracheobronchomalacia was the most commonly identified anatomic lesion. Thirty children from this cohort had poorly controlled asthma. Among this subgroup, 54% had increased neutrophils on BAL and 30% had an anatomic contributor to wheezing, including one with a laryngeal cleft. Based on findings from flexible bronchoscopy, management changes made in 63.8% of patients. This included medication changes in 54 and surgical intervention in 9. DISCUSSION: We conclude that transnasal flexible bronchoscopy has high yield in children with recurrent, persistent, or atypical wheezing and those with poorly controlled asthma. Laryngeal cleft has a reasonably high prevalence that warrants specific evaluation in this population.

Hyperbaric oxygen therapy for wound breakdown after oronasal fistula and cleft palate repair: four cases.

We offer the first report of hyperbaric oxygen (HBO2) therapy to treat early surgical wound breakdown after oronasal fistula and cleft palate repair in the pediatric population. We present four patients' experiences after undergoing HBO2 therapy. HBO2 was initiated as soon as an oronasal fistula was identified. Three of the children underwent 10 HBO2 treatments with the fourth undergoing 11 treatments. There were no adverse effects during treatment; none of the patients required decompressive myringotomy. Follow-up shows portions of the repair have been salvaged by initiating HBO2 at the first sign of fistula formation. In our case series, the fistulas all decreased in size over the course of treatment and demonstrated granulation tissue at the wound edges. Given the positive outcomes from our preliminary results, our results suggest concurrent usage of HBO2 therapy for wound breakdown after cleft palate repair is an effective treatment option.

Otorhinolaryngologic manifestations of Hartsfield syndrome: Case series and review of literature.

Diagnosis of Hartsfield syndrome includes recognition of three distinct clinical anomalies: holoprosencephaly, ectrodactyly, and bilateral cleft-lip and palate syndrome. A family including three male siblings all affected by Hartsfield syndrome presented to our institution for care. An autosomal dominant variant in Fibroblast Growth Factor Receptor 1 (FGFR1) was identified. This report focuses on otorhinolaryngologic manifestationsof Hartsfield syndrome, previously undescribed, including midline defects of holoprosencephaly, bilateral cleft-lip and palate, retrognathia, gastroesophageal reflux disease, external ear anomalies, eustachian tube dysfunction, and midface abnormalities, in addition to multidisciplinary, long-term management strategies. Multidisciplinary management is imperative in the care of these children with modification of approach based on their medical complexity.