Nonsurgical Orthodontic Airway Plate Treatment for Newborns With Robin Sequence.

Despite promising outcomes for >50 years, nonsurgical orthodontic airway plates (OAP) are only infrequently offered for babies with Robin sequence in a few parts of the world. This article demonstrates possibility of providing functional improvement using an OAP to help these babies overcome their functional and structural difficulties on their own. Two consecutively treated cases are presented exemplifying that OAP treatment that had originated from Europe is reproducible and effective in an institution in the United States.

A Systematic Review of Mandibular Distraction Osteogenesis Versus Orthodontic Airway Plate for Airway Obstruction Treatment in Pierre Robin Sequence.

OBJECTIVE: Mandibular distraction osteogenesis (MDO) is frequently performed to address airway obstruction in patients with Pierre Robin sequence (PRS), though more recently the technique of orthodontic airway plating (OAP) has gained traction. We aimed to evaluate OAP compared to MDO for airway obstruction in PRS. DESIGN: A systematic literature search across PubMed, Embase, and Google Scholar identified all studies published in English, which involved MDO or any form of OAP as treatments for PRS. All relevant articles were reviewed in detail and reported on, adhering to PRISMA guidelines. MAIN OUTCOME MEASURES: Airway (tracheostomy avoidance, decannulation rate), feeding (full oral feeding tolerance). RESULTS: Literature search identified 970 articles, of which 42 MDO studies and 9 OAP studies met criteria for review. A total of 1159 individuals were treated with MDO, and 322 individuals were treated with OAP. Primary outcomes appear similar for MDO and OAP at face value; however, this must be interpreted with different pretreatment contexts in mind. CONCLUSIONS: Orthodontic airway plating may be considered for airway obstruction in PRS, as some airway-related and feeding-related outcomes appear similar with MDO, per existing evidence in the literature. However, since PRS severity differed between studies, OAP cannot be uniformly considered a replacement for MDO. Further research is required to more comprehensively assess these treatment modalities inclusive of metrics that allow for direct comparison.

Neurofibromatosis of the larynx causing stridor and sleep apnea.

BACKGROUND: Neurofibromatosis type 1 can rarely present in the larynx. Patients typically do not present with complete obstructive symptoms, but partial obstruction and stridor. We review our health centers' case series of two patients, the first of whom presented with persistent sleep apnea post tonsillectomy and adenoidectomy, and the second who presented with noisy breathing. Additionally, we will review the literature on the management and treatment options for children with this rare clinical entity. METHODS: Retrospective case review. CASE REPORT & RESULTS: A two-year old male underwent a sleep endoscopy following persistent evidence of obstructive sleep apnea on polysomnography after initial tonsillectomy and adenoidectomy. Family elicited concerns about noisy breathing at night and an accompanying video documented stridor while sleeping during the monitored polysomnography. Flexible fiberoptic laryngoscopy in the operating room revealed what appeared to be a cystic mass along the right aryepiglottic fold causing deviation of the laryngeal introitus towards the contralateral side. Subsequent direct laryngoscopy and excisional biopsy revealed pathology results consistent with a plexiform neurofibroma. A six-month-old patient with stertor and stridor was found to have a laryngeal mass, subglottic stensosis, and progressive airway obstruction due to plexiform neurofirboma in the supraglottis, subglottis, and trachea. We present a series of two patients incidentally diagnosed with neurofibromatosis type 1 by way of a laryngeal neurofibroma and review the literature on management options. Both patients were found to have accompanying café au lait spots. Both patients required tracheostomy for airway management, and one was successfully decannulated. CONCLUSION: Laryngeal neurofibroma is a rare anomaly that can manifest with airway obstruction. Both patients presented here subsequently were noted to have café au lait spots on physical examination. The Otolaryngologist should be reminded of this anomaly when evaluating a child with evidence of a submucosal laryngeal mass. We present our series including that of a patient whose diagnosis was prompted by persistent sleep apnea following adenotonsillectomy tonsillectomy and a patient with airway obstruction and subglottic stenosis due to a neurofibroma. The treatment of choice is complete excision of the neurofibroma while maintaining functionality of the larynx. This can lead to successful decannulation.

Gaze Patterns of Normal and Microtia Ears Pre- and Post-Reconstruction.

OBJECTIVES: To understand attentional preferences for normal and microtia ears. METHODS: Eye-tracking technology was used to characterize gaze preferences. A total of 71 participants viewed images of 5 patients with unilateral microtia. Profile images of patient faces and isolated ears including normal, microtia, and post-reconstruction microtia ears were shown. Total time of fixation in predefined areas of interest (AOI) was measured. Inferential statistics were used to assess significance of fixation differences between AOIs within and between facial or auricular features. RESULTS: The ear received most visual attention in lateral view of the face (1.91 s, 1.66-2.16 s) [mean, 95% CI], followed by features of the "central triangle"-the eyes (1.26 s, 1.06-1.46), nose (0.48 s, 0.38-0.58), and mouth (0.15 s, 0.15-0.20). In frontal view, microtia ears received less attention following surgical reconstruction (0.74 s vs. 0.4 s, p < 0.001). The concha was the most attended feature for both normal (2.97 s, 2.7-3.23) and reconstructed microtia ears (1.87 s, 1.61-2.13). Scars on reconstructed ears altered the typical visual scanpath. CONCLUSION: The ear is an attentional gaze landmark of the face. Attention to microtia ears, both pre- and post-reconstruction, differs from gaze patterns of normal ears. The concha was the most attended to subunit of the ear. Attentional gaze may provide an unbiased method to determine what is important in reconstructive surgery. LEVEL OF EVIDENCE: NA Laryngoscope, 134:3136-3142, 2024.

Use of Convolutional Neural Networks to Evaluate Auricular Reconstruction Outcomes for Microtia.

OBJECTIVES: The objective of this study is to determine whether machine learning may be used for objective assessment of aesthetic outcomes of auricular reconstructive surgery. METHODS: Images of normal and reconstructed auricles were obtained from internet image search engines. Convolutional neural networks were constructed to identify auricles in 2D images in an auto-segmentation task and to evaluate whether an ear was normal versus reconstructed in a binary classification task. Images were then assigned a percent score for "normal" ear appearance based on confidence of the classification. RESULTS: Images of 1115 ears (600 normal and 515 reconstructed) were obtained. The auto-segmentation task identified auricles with 95.30% accuracy compared to manually segmented auricles. The binary classification task achieved 89.22% accuracy in identifying reconstructed ears. When the confidence of the classification was used to assign percent scores to "normal" appearance, the reconstructed ears were classified to a range of 2% (least like normal ears) to 98% (most like normal ears). CONCLUSION: Image-based analysis using machine learning can offer objective assessment without the bias of the patient or the surgeon. This methodology could be adapted to be used by surgeons to assess quality of operative outcome in clinical and research settings. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:2413-2416, 2023.

Post-Tonsillectomy Ibuprofen: Is There a Dose-Dependent Bleeding Risk?

OBJECTIVES/HYPOTHESIS: Post-tonsillectomy hemorrhage (PTH) is a potentially life-threatening complication. A recent meta-analysis suggests that ibuprofen may increase the risk of PTH. The aims of this study were to 1) re-evaluate the effect of ibuprofen on PTH given additional recent evidence and 2) to evaluate a potential dose effect of ibuprofen. STUDY DESIGN: Meta-analysis and meta-regression; single-institution retrospective review. METHODS: We conducted a systematic review of the literature and a meta-analysis of 12 studies comparing postoperative ibuprofen analgesia to non-nonsteroidal anti-inflammatory drug (NSAID) controls. Next, we performed a meta-regression analysis to assess for an effect of dose, if any, on rates of PTH. Five studies specifying a dose of 5 mg/kg (828 patients, 1,411 controls) and 7 studies using 10 mg/kg (5,633 patients, 7,656 controls) were included. We then conducted a novel single-institution, retrospective review of data for 1,046 patients prescribed intermediate-dose 7.5 mg/kg ibuprofen. RESULTS: Ibuprofen was not associated with an increased rate of PTH (log odds ratio [OR], 0.21; 95% confidence interval [CI] -0.15, 0.57). Meta-regression showed that ibuprofen dose (5 and 10 mg/kg) did not have a statistically significant effect on PTH (OR, 1.32; 95% CI 0.30, 5.84). Uncontrolled, aggregate rates of PTH across all studies were 2.29% (N = 828) for 5 mg/kg and 4.65% (N = 5,633) for 10 mg/kg dosing. The rate of secondary hemorrhage in patients prescribed 7.5 mg/kg ibuprofen was 3.10% (N = 1,046). CONCLUSION: We found no statistically significant increased risk of PTH when ibuprofen is prescribed at the low or high range of commonly used clinical dosages, compared to a non-ibuprofen regimen. Further studies with less heterogeneity are needed to determine if there is a clinically relevant dose-dependent difference in PTH with ibuprofen. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1473-1481, 2022.

Integrated microtia and aural atresia management.

Objectives: To present recommendations for the coordinated evaluation and management of the hearing and reconstructive needs of patients with microtia and aural atresia. Methods: A national working group of 9 experts on microtia and atresia evaluated a working document on the evaluation and treatment of patients. Treatment options for auricular reconstruction and hearing habilitation were reviewed and integrated into a coordinated care timeline. Results: Recommendations were created for children with microtia and atresia, including diagnostic considerations, surgical and non-surgical options for hearing management and auricular reconstruction, and the treatment timeline for each option. These recommendations are based on the collective opinion of the group and are intended for otolaryngologists, audiologists, plastic surgeons, anaplastologists, and any provider caring for a patient with microtia and ear canal atresia. Close communication between atresia/hearing reconstruction surgeon and microtia repair surgeon is strongly recommended.

The Social Perception of Microtia and Auricular Reconstruction.

OBJECTIVES: To examine the social perception of microtia and quantify the effect of reconstruction on socially perceived attributes. METHODS: Parental consent was obtained for peri-reconstruction photographs in a patient with unilateral grade 3 microtia without an underlying craniofacial syndrome. With computer simulation, the normal, preoperative microtia, and postoperative reconstruction ear were isolated and blended into the oblique and lateral views of that volunteer's face to isolate ear morphology as a variable against a constant facial baseline. These photographs were embedded into Web-based surveys with visual analogue scales to capture social perception data and then were sourced to general population adults. RESULTS: Survey respondents totaled 631. On average, the face with the microtia ear was perceived to be less friendly (P = .015), less healthy (P = .022), and less successful (P = .005) than the same face with the "normal" ear. There were no statistically significant differences in socially perceived attributes between the face with the normal ear and the face with the reconstructed ear. CONCLUSION: This is the first study to examine the social perception consequences of microtia and microtia reconstruction in children. These findings may explain the significant psychosocial distress experienced by these patients by exploring the social perception of specific attributes perceived. Lastly, this study may better inform microtia patients and their physicians on the impact of auricular reconstruction on third party's perception of social attributes. LEVEL OF EVIDENCE: N/A Laryngoscope, 131:195-200, 2021.

Social Perceptions of Pediatric Hearing Aids.

OBJECTIVES: To determine whether hearing aid (HA) use affects social perceptions of general public adults and age-matched peers and if so, determine if effects are modulated by lack of societal representation of pediatric HAs. METHODS: A 10-year-old boy was presented in six photographic conditions with and without HAs and eyeglasses (a worn sensory aid with wider societal representation). HAs were presented in neutral skin tone and bright blue colors. Photographic conditions were embedded into web-based surveys with visual analog scales to capture social perceptions data and sourced to 206 adults (age 18-65) and 202 peers (age 10) with demographic characteristics representative of the general US population. Mean differences in scores for each condition compared to control images were computed using two-tailed t-tests. RESULTS: In both adult and child respondents, HAs were associated with decreased athleticism, confidence, health, leadership, and popularity. Glasses were associated with decreased athleticism and popularity but increased intelligence, overall success, and in the child respondents, friendliness. When worn in combination, the beneficial effects of glasses were mitigated by brightly colored but not neutrally colored HAs. CONCLUSION: Negative effects of pediatric HAs on social perceptions may be influenced by poor societal representation of HAs. These results suggest that greater representation of pediatric HAs is necessary to make society more inclusive for children with hearing loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2387-E2392, 2021.

Propranolol for the treatment of a life-threatening subglottic and mediastinal infantile hemangioma.

An infant with a subglottic hemangioma remained in respiratory distress after multiple treatments failed and was found to have an enlarging mediastinal infantile hemangioma compressing the trachea. Treatment with oral propranolol resulted in resolution of symptoms within 2 days and a 50% reduction in lesion size within 1 week.

Major complications after tongue-tie release: A case report and systematic review.

INTRODUCTION: The diagnosis of ankyloglossia, or tongue-tie, and the number of frenotomies performed has increased over 10-fold from 1997 to 2012 in the United States. The sharpest increase has been in neonates. For parents considering frenotomy for their breastfeeding newborn, there is controversy surrounding the evaluation of tongue-tie and the benefit of a frenotomy. Complications from tongue-tie procedures are thought to be low, though it is not well reported nor studied. OBJECTIVES: The aim of this study is to describe a case of a sublingual mucocele after laser frenotomy in a neonate with tongue-tie and to investigate major complications reported after tongue-tie release in pediatric patients through a systematic review of the literature. CASE REPORT: We present a 6-week-old female who underwent a laser frenotomy procedure performed by a dentist who presented with a new cyst under her tongue. MATERIAL AND METHODS: A systematic literature search of articles published from 1965 to April 2020 was conducted in Ovid MEDLINE(R), Ovid EMBASE, and Scopus. Citations were uploaded into a systematic review software program (DistillerSR, Ottawa, ON, Canada), followed by full text screening. RESULTS: 47 major complications were reported in 34 patients, including our patient. Most of the cases were located in the United States and Europe. The most frequent indications for the procedure were breastfeeding problems (n = 18) and speech impediment (n = 4). The procedure was performed by dentists (n = 6), lactation consultants (n = 5), and otolaryngologists (n = 4). The bulk of the major complications after frenotomy included poor feeding (n = 7), hypovolemic shock (n = 4), apnea (n = 4), acute airway obstruction (n = 4), and Ludwig angina (n = 2). CONCLUSIONS: Reporting of complications after frenotomy is lacking. Risks to neonates may be different than risks to older children and adults. Practitioners across different specialties should be monitoring and studying this more rigorously to better guide patients and families on the risks and benefits of this procedure.

Renal ultrasound abnormalities in children with syndromic and non-syndromic microtia.

OBJECTIVE: Renal abnormalities are commonly considered in the work up of pediatric patients with external ear malformations. However, there is little consensus regarding an appropriate renal screening protocol for patients with microtia. We sought to characterize renal abnormalities detected on ultrasonography in pediatric patients with microtia. METHODS: We conducted a retrospective cohort study of pediatric patients diagnosed with microtia who underwent renal ultrasound from 1991 to 2014 at a single tertiary academic institution. Renal ultrasound reports and medical records were reviewed to assess for renal abnormalities and to determine whether patients required specialist follow-up or interventions. Audiograms and otolaryngology notes were used to determine patterns of hearing loss. The following additional information was recorded from the electronic medical records: patient sex, microtia grade (I-IV), microtia laterality, and known associated syndromes. Characteristics were compared between those who did and did not have renal ultrasound findings using Fisher's exact test. Univariate logistic regression analysis was performed to determine factors associated with renal ultrasound findings. RESULTS: The majority of patients in this cohort were syndromic (n = 51, 64%) with grade III microtia (n = 46, 58%) and conductive hearing loss (n = 58, 72%). Syndromic children with microtia demonstrated a higher crude rate of renal ultrasound abnormalities (22%) than children with isolated microtia (7%). Of these patients, 69% required specialist follow-up. Univariate logistic regression analysis did not identify predictors that were significantly associated with renal ultrasound findings. CONCLUSION: Fairly high rates of abnormalities in syndromic and non-syndromic patients may warrant screening renal ultrasound in all patients with microtia, especially given the high percentage of findings requiring renal follow-up. A prospective study to formally evaluate screening efficacy is needed.

Management of Complex Arteriovenous Malformations Using a Novel Combination Therapeutic Algorithm.

Importance: Current therapeutic options for patients with extracranial head and neck arteriovenous malformations are limited. Surgical intervention, such as sclerotherapy or resection, often result in rapid recurrence and progression of disease. Objective: To assess the efficacy and tolerability of sirolimus as an adjuvant therapy for endovascular embolization in the management of complicated extracranial head and neck arteriovenous malformations. Design, Setting, and Participants: This case series examined 6 patients with extracranial head and neck arteriovenous malformations treated from January 1, 2013, to December 31, 2017, at a multidisciplinary vascular anomalies clinic within Stanford Hospital and Clinics. Intervention: Initiation of sirolimus at least 1 month prior to endovascular embolization, targeting a trough level of 10 to 15 ng/mL throughout the course of the endovascular embolization series and continued for at least 1 month after the series. Main Outcomes and Measures: Clinical manifestations; disease progression and overall response to treatment were assessed via clinical evaluation and radiographic imaging. Results: All 6 patients (4 male and 2 female patients; mean age, 24.5 years [range, 9-44 years]) responded favorably to the combination of sirolimus therapy followed by endovascular embolization, and 4 patients exhibited a near-complete response. The median duration of follow-up was 19 months (range, 6-40 months). One patient discontinued sirolimus soon after embolization and experienced regrowth of the arteriovenous malformation after 1 year. Sirolimus was resumed, which has stabilized his disease for more than 2 years. Mild adverse effects were noted in 4 patients. The combination therapy was well tolerated in all patients. One patient developed skin ulceration after embolization and required surgical debridement. Another patient developed pulmonary microthrombi after embolization with cyanoacrylate glue that resolved with a brief course of anti-inflammatory therapy. Conclusions and Relevance: Although further prospective trials are needed, this report suggests the benefit of a mammalian target of rapamycin inhibitor as an adjuvant therapy for surgical embolization of complex, extracranial head and neck arteriovenous malformations. The optimal dosing and therapeutic duration of sirolimus treatment before and after embolization remain to be determined.

Pediatric vocal fold paralysis after cardiac surgery: rate of recovery and sequelae.

OBJECTIVE: To determine the rate of recovery of pediatric vocal fold paralysis (VFP) after cardiac surgery. STUDY DESIGN AND SETTING: Retrospective case series from January 2000 to 2005 at 4 tertiary care pediatric hospitals. RESULTS: A total of 109 children with VFP were identified. Of 80 patients with follow-up >3 months, 28 (35%) recovered vocal fold function with a median time to diagnosis of recovery of 6.6 months. Fifty-two (65%) patients had persistent vocal fold paralysis with a median follow-up time of 16.4 months. Twenty-five (45%) of 55 patients demonstrated aspiration or laryngeal penetration with modified barium swallow. Twenty-nine (27%) of the 109 patients underwent surgical intervention for their airway, feeding, or voice. CONCLUSIONS: Pediatric VFP is not an uncommon complication after cardiac surgery and can result in serious sequelae. This study demonstrates a 35% rate of recovery, 45% rate of aspiration, and 27% rate of complications that require surgical intervention.

Recovery from cisplatin-induced ototoxicity: a case report and review.

We present a pediatric case report of cisplatin-induced ototoxicity with subsequent recovery. The patient experienced tinnitus and fluctuating mild high-frequency sensorineural hearing loss (SNHL) with a concomitant decrease in distortion product otoacoustic emissions (DPOAE). There was recovery of hearing loss and return of DPOAE at 1 year after completion of cisplatin therapy. Reports of recovery from cisplatin-induced ototoxicity in humans are limited in the literature, especially in the pediatric population. A review of cisplatin ototoxicity and mechanisms of recovery are discussed, with an emphasis on the particular chemotherapy regimen and dosing schedule in this case, given at 4-11 week intervals.

The Superior Labial Frenulum in Newborns: What Is Normal?

Introduction and Objectives: There has been an emergence of procedures to release the superior labial frenula in infants, yet little is known about the normal appearance or incidence of severe attachment, or "lip-tie." The objective of this article was to develop a classification system for superior labial frenula and to estimate the incidence of different degrees of attachment. Methods: A prospective cross-sectional study. Newborns were examined and had photographs taken of their upper frenula. Relevant medical professionals rated the appearance of the labial frenula using a previously described Kotlow classification system. The raters assessed each photograph twice and were blinded to their previous rating and to other raters' scores. Results: All newborns have a labial frenula, with most attached at the gingival margins (83%). Raters had poor intra- and interrater reliability (64% to 74% and 8%, respectively), using the Kotlow classification system, which improved when the classification system was simplified. Conclusions: The Kotlow classification of lip-tie fails to be reproducible by relevant experts. The majority of infants had a significant level of attachment of the labial frenulum. As more procedures are done to release the upper lip frenulum, it is important to understand what degree of attachment is normal, or more common.

A child with silent sinus syndrome and spontaneous improvement after sinus surgery.

Silent sinus syndrome is characterized by an asymptomatic hypoplastic maxillary sinus with progressive enophthalmos and hypoglobus. This is a disease rarely affecting children with the majority of reported cases involving adult patients. Treatment is primarily surgical with endoscopic sinus surgery to restore aeration of the sinus along with orbital reconstruction to restore facial symmetry. In this report, we describe a 7 year old child with facial asymmetry and no sinonasal symptoms. CT showed an opacified hypoplastic right maxillary sinus. One year after endoscopic sinus surgery, there was spontaneous improvement of facial asymmetry and relative maxillary sinus size.

Supraglottoplasty for occult laryngomalacia to improve obstructive sleep apnea syndrome.

OBJECTIVE: To evaluate the polysomnographic outcomes after supraglottoplasty (SGP) performed for obstructive sleep apnea syndrome (OSAS) associated with occult laryngomalacia. DESIGN: Retrospective case series with medical chart review. SETTING: Tertiary pediatric medical center. PATIENTS: Twenty-two patients aged 2 to 17 years met the inclusion criteria of polysomnography-proven OSAS and occult laryngomalacia seen on flexible fiber-optic sleep endoscopy. Infants with congenital laryngomalacia were excluded. INTERVENTION: Carbon dioxide laser SGP was performed either alone or in conjunction with other operations for OSAS. MAIN OUTCOME MEASURE: Preoperative and postoperative nocturnal polysomnographic data were paired and analyzed statistically. RESULTS: Supraglottoplasty for occult laryngomalacia resulted in statistically significant reduction in the apnea-hypopnea index (AHI) (from 15.4 to 5.4) (P <.001). Subgroup analysis of children who underwent either SGP alone or in combination with other interventions showed comparable reductions in AHI. Medical comorbidities were associated with worsened postoperative outcomes, although still significantly improved compared with baseline. Overall, 91% of children had an improvement in AHI, and 64% had only mild or no residual OSAS after SGP. CONCLUSION: Supraglottoplasty is an effective technique for the treatment of OSAS associated with occult laryngomalacia.

Sleep endoscopy as a diagnostic tool in pediatric obstructive sleep apnea.

OBJECTIVES: Ten to twenty percent of children have persistent obstructive sleep apnea (OSA) after adenotonsillectomy (T&A). We hypothesize that sleep endoscopy, a flexible fiberoptic examination of the pharynx under anesthesia, is an effective tool for identifying sites of persistent obstruction. METHODS: In this retrospective cohort study, we reviewed records of children who had symptoms consistent with OSA and a positive polysomnogram (PSG) who underwent sleep endoscopy followed by sleep endoscopy directed surgery. Data collection included age, BMI and co-morbidities. Apnea-hypopnea index (AHI) was compared to pre and post surgery for each child using a paired t-test. RESULTS: Of the 80 children who underwent sleep endoscopy followed by directed surgery, 65% were male, mean age was 6 years (SD 3.75 years), average BMI was 19 (SD 0.43 years) and 28% had co-morbidities. For the 51% of patients who had persistent OSA after T&A, the mean AHI after sleep endoscopy directed surgery was significantly lower then before surgery (7.9 vs. 15.7, p<.01). For the 49% of patients who had never undergone surgery for OSA, or who were surgically naïve, and underwent sleep endoscopy directed surgery, the mean AHI was significantly lower then before surgery (8.0 vs. 13.8, p<.01). CONCLUSIONS: Sleep endoscopy is a consistently reliable tool for identifying the sites of obstruction in both surgically naive children and those with persistent OSA after T&A.