Choice of suture technique may mitigate figure-of-8 deformity in slide tracheoplasty.

OBJECTIVES: To demonstrate varying degrees of immediate postoperative figure of 8 deformity following three different methods of tracheal suturing. STUDY DESIGN: Comparative ex-vivo study. METHODS: A fresh, unfixed rabbit trachea was harvested and bisected at its midpoint. The posterior membranous trachea was excised from both segments to mimic the anatomy of complete tracheal rings following longitudinal release. A slide tracheoplasty was performed on the same tracheal segments as an internal control, using three different methods of closure: simple running, running horizontal mattress, and a modified baseball stitch. Endoscopic and external photographs were taken immediately following repair to document the effect of closure technique on external and endoluminal anatomy. RESULTS: A simple running closure, in which traveling occurs between needle passes, showed the greatest degree of postoperative figure of 8 deformity. A running horizontal mattress suture closure showed less deformity and a modified running closure, in which traveling occurs within needle passes, showed the least deformity. CONCLUSIONS: Our study suggests that a minor modification of standard running closure techniques may help limit the common figure-of-8 deformity often noted following slide tracheoplasty. LAY SUMMARY: Figure of-8 deformity is a problem that can occur when repairing a narrow trachea referred to as tracheal stenosis. This deformity may be prevented using particular suture techniques which may decrease the likelihood that the trachea becomes blocked again following repair.

Identification of micrognathia by prenatal ultrasound may improve timely diagnosis and management of infants with Robin sequence.

OBJECTIVE: Robin sequence (RS) is a craniofacial anomaly characterized by small jaw (micrognathia) with associated tongue base airway obstruction. With advances in fetal imaging, micrognathia may be detected prenatally. This study aims to determine if prenatal recognition of micrognathia offers any advantage over being unaware of the condition until after delivery and to assess if prenatal consultation for micrognathia adds benefits beyond merely noting the presence of the condition. METHOD: Retrospective chart review examining cases from 01/01/2010 to 12/31/2020 at an urban tertiary medical center. RESULTS: Forty seven infants with RS were included. 40.4% (n = 19) had micrognathia/retrognathia noted on prenatal ultrasound. 47.4% (n = 9) of those 19 pregnancies saw a maternal fetal medicine (MFM) program with craniofacial consultation. Compared to 28 infants not diagnosed with micrognathia until after birth, the 19 infants identified prenatally required fewer transfers from birth hospital (p = 0.02). Additionally, those referred to MFM with craniofacial consultation had shorter lengths of stay when airway intervention was required (p = 0.05). CONCLUSION: Prenatal recognition of micrognathia may lead to early detection and management of RS. When RS is suspected, prenatal consultation with MFM and craniofacial team may further optimize care of the infant following delivery.

Acquired nasopharyngeal stenosis after radiation treatment for nasopharyngeal carcinoma.

INTRODUCTION: Nasopharyngeal stenosis (NPS) is a rare and morbid complication following radiation therapy for nasopharyngeal carcinoma. This review provides an update on management and prognosis. METHODS: A comprehensive PubMed review using the terms "nasopharyngeal stenosis," "choanal stenosis," and "acquired choanal stenosis" was performed. RESULTS: Fourteen studies identified 59 patients who developed NPS after radiotherapy for NPC. 51 patients underwent endoscopic nasopharyngeal stenosis excision by cold technique (80-100% success). The remaining 8 underwent carbon dioxide (CO2) laser excision with balloon dilation (40-60% success). Adjuvant therapies included postoperative topical nasal steroids in 35 patients. The need for revision was 62% in the balloon dilation group, vs 17% in the excision group (p-value <0.01). CONCLUSION: When NPS occurs after radiation, primary excision of scarring is the most effective method of management with less need for revision surgery relative to balloon dilation.

Pharyngeal wall and soft palate motion after two common speech surgeries.

PURPOSE: To assess how pharyngeal wall and soft palate motion are affected after two common interventions for velopharyngeal insufficiency. MATERIALS AND METHODS: A retrospective observational study was performed. A database of patients who had undergone Furlow palatoplasty or pharyngeal flap surgery between 2011 and 2019 and had video-archived preoperative and postoperative nasopharyngoscopy recordings was created. Recordings were deidentified and randomized, with 5 randomly-selected videos duplicated to determine intra-rater reliability. The videos were scored by 3 experienced raters using a modified Golding-Kushner scale. Pre- and postoperative scores were compared using paired t-test. Inter- and intra-rater reliability were estimated using intra-class correlation (ICC). RESULTS: There were 17 patients who met inclusion criteria. The mean age was 6.9 years (range 3-22 years, 59% male). In the Furlow palatoplasty group (n = 9), an increase in left soft palate motion was noted postoperatively (t(8) = 2.71, p = 0.02). In the pharyngeal flap group (n = 8), increases in lateral pharyngeal wall motion (left: t(7) = 3.58, p = 0.008, right: t(7) = 3.84, p = 0.006) and right soft palate motion (t(6) = 2.49, p = 0.04) were identified. Intra-rater reliability and inter-rater agreement were lower than prior studies utilizing the Golding Kushner scale. CONCLUSIONS: Our results provide objective evidence that Furlow palatoplasty and pharyngeal flap surgeries achieve velopharyngeal closure by increasing movement at different anatomical sites. Palatal and pharyngeal wall motion observed during preoperative nasopharyngoscopy may influence a surgeon's choice of intervention.

Ambient noise limits efficacy of smartphone-based screening for hearing loss in children at risk.

OBJECTIVE: To determine if hearScreen®, a smartphone-based pure tone audiometric screen, serves as an effective hearing screen for identifying hearing loss in children at risk, such as those with chronic otitis media and/or craniofacial anomalies. METHODS: A cross-sectional, single-center, prospective study at an urban tertiary care hospital was completed. From June to October 2019, a total of 208 pediatric ears at risk for hearing loss were evaluated by both hearScreen® and conventional pure tone audiometry. The efficacy of hearScreen® for detecting hearing loss in a pediatric population at risk was determined. RESULTS: A total of 208 pediatric ears at risk for hearing loss were screened. HearScreen® demonstrated a sensitivity of 85%, a specificity of 41%, a positive predictive value of 36%, and a negative predictive value of 87%. Subgroup analysis stratified by hearing loss type showed that hearScreen® screens for conductive hearing loss with increased sensitivity. CONCLUSIONS: This investigation suggests that hearScreen® may serve as a an accessible, low-cost, and sensitive pure tone audiometric screen for hearing loss in children at risk, particularly those with conductive hearing loss, with the caveat that it may generate a high proportion of false positives due to the influence of ambient noise, especially when screening at 500 Hz. Future investigations should weigh the utility of including 500 Hz in the screening protocol as well as assess methods that may mitigate the influence of ambient noise during a pure-tone audiometric screen.

The spiral flap: A novel approach to scalp closure in children.

BACKGROUND: The spiral rotational advancement flap is a single-stage technique that has been described for the closure of nasal ala defects and in nipple reconstruction. Herein we present our experience using the spiral flap for closure of moderately sized defects of the scalp vertex in children. Compared to other scalp reconstructive approaches, such as a simple rotational flap or primary linear closure, the spiral flap design offers improved cosmesis through recreation of the natural whorl pattern within the hair-bearing scalp. Scar camouflage is especially important in the pediatric population, as increased skin elasticity and continued cranial growth impart a high risk of scar widening in children. METHODS: The electronic patient database at a single, urban, academic, tertiary pediatric otolaryngology and facial plastic surgery practice was queried to identify children who had undergone scalp reconstruction. The medical records of three children who had undergone spiral rotational advancement flap closure by a single surgeon were reviewed. Indications for scalp reconstruction in these patients were limited to the excision of benign, congenital lesions, such as infantile hemangioma unresponsive to propranolol and hypertrophic scarring and alopecia from aplasia cutis congenita A case example of a 15-month-old boy with scarring of the scalp, secondary to aplasia cutis congenita, is chosen for demonstration of the spiral flap design in step-by-step instructional video. RESULTS: A spiral rotational advancement flap was conceived for multi-layer repair in three pediatric patients. By reproducing the main elements of the logarithmic spiral flap design, the apex of the flap is rotated in on itself to complete a spiral closure pattern. There were no operative or post-operative complications. At various post-operative timepoints, the wounds appeared well-healed without evidence of flap necrosis, skin discoloration, or residual alopecia. CONCLUSION: We report satisfactory results using the spiral rotational advancement flap to address moderately sized defects of the scalp vertex in three pediatric patients. Although this flap has primarily been used in the reconstruction of the nasal ala in adults, we feel that this technique is ideally suited for scalp reconstruction, particularly in young children with highly elastic tissues in areas with decreased pliability. This approach has the added advantage of simulating the natural hair whorl in a cosmetically sensitive region and may serve as an effective alternative to linear reconstruction methods. The technique described can be added to the armamentarium of the pediatric plastic surgeon.

TIA: algorithms for development of identity-linked SNP islands for analysis by massively parallel DNA sequencing.

BACKGROUND: Single nucleotide polymorphisms (SNPs) located within the human genome have been shown to have utility as markers of identity in the differentiation of DNA from individual contributors. Massively parallel DNA sequencing (MPS) technologies and human genome SNP databases allow for the design of suites of identity-linked target regions, amenable to sequencing in a multiplexed and massively parallel manner. Therefore, tools are needed for leveraging the genotypic information found within SNP databases for the discovery of genomic targets that can be evaluated on MPS platforms. RESULTS: The SNP island target identification algorithm (TIA) was developed as a user-tunable system to leverage SNP information within databases. Using data within the 1000 Genomes Project SNP database, human genome regions were identified that contain globally ubiquitous identity-linked SNPs and that were responsive to targeted resequencing on MPS platforms. Algorithmic filters were used to exclude target regions that did not conform to user-tunable SNP island target characteristics. To validate the accuracy of TIA for discovering these identity-linked SNP islands within the human genome, SNP island target regions were amplified from 70 contributor genomic DNA samples using the polymerase chain reaction. Multiplexed amplicons were sequenced using the Illumina MiSeq platform, and the resulting sequences were analyzed for SNP variations. 166 putative identity-linked SNPs were targeted in the identified genomic regions. Of the 309 SNPs that provided discerning power across individual SNP profiles, 74 previously undefined SNPs were identified during evaluation of targets from individual genomes. Overall, DNA samples of 70 individuals were uniquely identified using a subset of the suite of identity-linked SNP islands. CONCLUSIONS: TIA offers a tunable genome search tool for the discovery of targeted genomic regions that are scalable in the population frequency and numbers of SNPs contained within the SNP island regions. It also allows the definition of sequence length and sequence variability of the target region as well as the less variable flanking regions for tailoring to MPS platforms. As shown in this study, TIA can be used to discover identity-linked SNP islands within the human genome, useful for differentiating individuals by targeted resequencing on MPS technologies.

Neonatal Macroglossia: Demographics, Cost of Care, and Associated Comorbidities.

OBJECTIVES: To examine the birth prevalence of congenital macroglossia and identify demographic variables and comorbidities that may influence length of stay and cost of care. STUDY DESIGN: Retrospective cross-sectional study using the Kids' Inpatient Database 2003, 2006, 2009, and 2012. METHODS: Demographics were analyzed. Linear regression modeling and multivariate analyses were performed. RESULTS: The birth prevalence of congenital macroglossia was 4.63/100 000 births. Patients were classified as isolated (n = 349, 48.1%) or syndromic (n = 377, 51.9%). A higher incidence of isolated macroglossia was seen in females (odds ratio, OR [95% confidence interval, 95% CI]: 1.93 [1.45-2.56] and African Americans (2.02 [1.41-2.88]). Length of stay was higher for syndromic patients than for nonsyndromic patients (22.6 days [18.6-26.6] vs 7.93 days [5.95-9.90], as were inpatient costs ($3619USD [$27 628-$44 754] vs $10 168USD [$6272-$14 064]. After accounting for gender, race, location, and socioeconomic status, the presence of macroglossia alone increased length of stay by 4.07 days (0.42-7.72 days) in nonsyndromic patients and 12.02 days (3.63-20.4 days) in syndromic patients. The cost of care increased by $6207USD ($576-$11 838) among nonsyndromic newborns and $17 205USD ($374-34 035) among syndromic patients. CONCLUSION: The birth prevalence of congenital isolated macroglossia appears to vary by sex and race. Prolonged length of stay and increased costs are associated with both isolated macroglossia and syndromic macroglossia, even after controlling for other syndromic comorbidities.

Surgical Management of Pierre Robin Sequence: Using Mandibular Distraction Osteogenesis to Address Hypoventilation and Failure to Thrive in Infancy.

Mandibular hypoplasia may present in isolation or in the context of glossoptosis and a U-shaped, incomplete cleft palate. This latter triad is referred to as Pierre Robin sequence. Deleterious effects of micrognathia that may present during infancy are due primarily to glossoptosis or posterior displacement of the tongue. This tongue base prolapse may cause varying degrees of upper airway obstruction. A surgical option for management of tongue base airway obstruction secondary to mandibular hypoplasia is neonatal mandibular distraction osteogenesis. Herein, the author seeks to outline the benefits and limitations of early mandibular distraction osteogenesis as a way of managing airway obstruction and feeding difficulty in newborns with micrognathia. A description of the author's operative technique as well as potential complications and pitfalls will also be discussed.

Presentation, Management, and Outcomes of Pathologic Scars Within a Pediatric Otolaryngology Practice.

OBJECTIVES: This study aims to review the spectrum of scarring that may present to an urban, pediatric otolaryngology practice and determine if associations exist between race, scar location, treatment modality, and outcomes following interventions for scarring. METHODS: Retrospective cohort study among 115 pediatric patients with 138 unique keloids or hypertrophic scars (HTS), and 141 children presenting for tonsillectomy at Tufts Medical Center. Age at presentation and sex assigned at birth were collected for both populations. For those presenting for pathologic scars, income quintile, self-identified race/ethnicity, anatomical location, treatment number and type, and clinical outcome were also analyzed. Multivariate analyses calculated adjusted odds ratios (aORs) and 95% confidence intervals to assess associations between scar subsite, intervention type, and persistence after treatment. RESULTS: Compared to individuals presenting for tonsillectomy, a disproportionate percentage of patients presenting for scarring identified as Black (26.6% vs. 13.5%) or Asian (17.4% vs. 7.1%, p = 0.016) or were male (61.7% vs. 49.7%, p = 0.053). Individuals identifying as Black or Asian were more likely to present with ear lobe and neck scars, respectively (50.0% vs. 45.5%, p = <0.001). Ear scars were significantly more likely to receive excision at initial treatment (aOR = 5.86 [1.43-23.96]) compared to other subsites, and were more likely to require >1 treatment (aOR = 5.91 [1.53-22.75]). CONCLUSION: Among pediatric patients presenting with keloids or HTS, children who identified as Black or Asian were more likely to present with ear and neck scars, respectively. Ear scars were frequently treated with excision and appear more likely to require adjuvant treatments and multiple interventions. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:3127-3135, 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.

Symptomatic Lingual Thyroglossal Duct Cyst in Children: A Laryngomalacia Phenotype.

OBJECTIVES: Patients with lingual thyroglossal duct cyst (TGDC) can present as symptomatic with obstructive airway and feeding difficulties. METHODS: We present 3 cases of symptomatic lingual TGDC. RESULTS: All 3 patients were diagnosed with laryngomalacia and underwent further concurrent or delayed airway intervention, in addition to cyst removal. CONCLUSIONS: We reason that there is a phenotype of laryngomalacia in the symptomatic lingual thyroglossal duct cyst patients who present with symptoms due to disruption in laryngeal anatomy rather than the cyst itself causing obstructive symptoms. Distinguishing this phenotype, especially in comparison to other pathologies such as vallecular cysts, may better allow for planning of concurrent or delayed airway procedures and overall counseling of parents.