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, 2024.

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.

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.

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.

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.

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.

Demographic Risk Factors for Malnutrition in Patients With Cleft Lip and Palate.

OBJECTIVES/HYPOTHESIS: Patients with cleft lip and/or palate (CLP) are at increased risk of malnutrition. Acute and chronic malnutrition have been associated with elevated risk of postsurgical wound complications, adding morbidity and cost to patients and their families. To study the association between demographic factors, including insurance type, race, and median neighborhood income (MNI), and malnutrition in patients with CLP. STUDY DESIGN: Retrospective cohort study. METHODS: Retrospective review was performed in patients undergoing their first cleft-related surgery at a large tertiary pediatric hospital from 2006 to 2018. Demographic data, weight and height at surgery, type of insurance, race, and primary residential address were collected. Geocoded information on MNI was generated using patient address. World Health Organization Z-scores for weight-for-age (WFA) and height-for-age (HFA) were used as proxies for acute and chronic malnutrition, respectively. Linear regression models were generated to analyze the relationship of insurance type, race, and MNI on WFA and HFA Z-scores. RESULTS: About 313 patients met inclusion criteria. Increasing MNI predicted increasing WFA Z-score (0.05 increase in WFA per $1,000 increase, P = .047) as well as HFA Z-score (0.09 increase in HFA per $1,000 increase, P = .011). The effect of MNI was not independently modified by race for either WFA (P = .841) nor HFA (P = .404). Race and insurance type did not predict WFA or HFA. CONCLUSIONS: Lower MNI is a significant independent risk factor for acute and chronic malnutrition in children with CLP. Combined with previous investigation linking malnutrition to surgical outcomes in this population, this offers a target area for intervention to improve patient outcomes. LEVEL OF EVIDENCE: 3 Laryngoscope, 132:1482-1486, 2022.

Hearing Outcomes in Stickler Syndrome: Variation Due to COL2A1 and COL11A1.

OBJECTIVES: Stickler syndrome (SS) is a heterogeneous inherited connective tissue disorder, often due to a mutation in COL2A1 or COL11A1. Mutations in these genes cause collagen abnormalities affecting ocular, auditory, orofacial, and skeletal systems, including hearing loss, micrognathia, and cleft palate. Understanding the variability of hearing phenotypes based on genetic mutation has a significant impact on treatment and long-term care. DESIGN: A retrospective chart review of pediatric patients with a confirmed diagnosis of SS between January 2003 and December 2018 at a tertiary pediatric hospital was performed. Patients were excluded if they did not have genetic evaluation, craniofacial/ear, nose, and throat evaluation, and/or audiologic testing. Charts were reviewed for the following information: age, race, sex, SS diagnosis, genetic variant of SS, and audiological testing data. RESULTS: There were 29 confirmed patients with SS who met criteria, 16 with type I (COL2A1) and 13 with type II (COL11A1). Of the 13 patients with type II, 12 (92%) demonstrated hearing loss, ranging in severity from mild to severe. In type I, 25% of patients had mild or resolved hearing loss. CONCLUSION: Results suggest that patients with type II SS are more likely to have congenital hearing loss than type I. Data also suggest that the COL11A1 mutation shows consistently more severe hearing loss than the COL2A1 mutation.

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.

Detection of Chronic Hypoventilation Among Infants With Robin Sequence Using Capillary Blood Gas Sampling.

OBJECTIVES/HYPOTHESIS: To describe the use of capillary blood gas (CBG) sampling to detect and quantify hypoventilation in infants with Robin sequence (RS). METHODS: Case series with chart review at two institutions. Infants with RS presenting over a 10-year period were identified using departmental databases. CBG values obtained during infancy or until airway intervention (AI) were reviewed. RESULTS: From 2008 to 2018, 111 infants with RS were identified as having had been assessed and managed from birth or transfer until discharge home and having CBG data available. In most cases, CBG sampling was obtained every other day until intervention or discharge. A total of 81 (73%) infants required AI: 72 (89%) underwent mandibular distraction osteogenesis, five (6%) underwent tracheotomy, and four (5%) were discharged home with a nasopharyngeal airway. The mean PCO2 at day of life (DOL) 7-30 for the AI group was 52.7 mmHg (95% confidence interval: 51.7-53.7) and for the no AI group was 45.9 mmHg (44.8-47.0; P < .0001). The mean HCO3 at DOL 7-30 for the AI group was 29.8 mEq/L (29.4-30.1) and for the no AI group was 27.0 mEq/L (26.5-27.4; P < .0001). Receiver operating characteristic curves were created for maximum PCO2 and HCO3 values and cutoffs were established by optimizing a balance of sensitivity and specificity. Infants requiring AI surpassed the PCO2 and HCO3 cutoff at a median of DOL 9. CONCLUSIONS: Among infants with RS and hypoventilation, objective measures of respiratory acidosis may be apparent by DOL 9. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:2789-2794, 2021.

Cleft Palate Repair by Otolaryngologist-Head and Neck Surgeons: Risk Factors for Postoperative Fistula.

OBJECTIVES/HYPOTHESIS: To assess the incidence of palatal fistula after primary repair of the cleft palate among two cohorts of Otolaryngologist-Head and Neck Surgeons and to identify patient and surgeon characteristics that may predict fistula development. STUDY DESIGN: Retrospective case series with chart review. METHODS: Children who underwent primary repair of cleft palate at one of two multidisciplinary cleft centers over a 10 year period were identified. Charts were reviewed for the presence of palatal fistula; chi square test and multivariate logistic regression analysis were performed to determine variables associated with fistula formation. RESULTS: From 2007 to 2017, 477 patients underwent primary repair of cleft palate by one of 6 Otolaryngologist-Head and Neck Surgeons. Twenty-four children had incomplete charts, allowing 453 patients to be included in the final analysis. The pooled mean incidence of palatal fistula was 6.6% (P = .525) and varied significantly by cleft type. Logistic regression analysis controlling for multiple variables, showed that Veau IV classification had the highest risk of fistula (OR = 10.582; P = .004). Repair by a specific surgeon was not a significant risk factor for fistula development (P > .07 for each surgeon). CONCLUSIONS: Among six Otolaryngologist-Head and Neck Surgeons with fellowship training in cleft palate repair postoperative fistula rates were consistent and compared favorably to standards in the Cleft and Craniofacial surgery literature established by other surgical specialties. Consistent with larger database studies involving multiple surgical specialties, Veau IV classification was the strongest predictor of palatal fistula development, even after adjusting for multiple variables, including differing levels of experience. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1281-1285, 2021.

Multimodal Imaging Characteristics and Diagnostic Approach to Ancient Schwannoma in a Pediatric Patient.

Ancient schwannoma is an extremely rare benign, peripheral nerve sheath tumor. Despite its benign nature, its characteristic heterogeneous appearance and degenerative changes commonly lead to misdiagnosis of malignancy. Although schwannomas are extremely uncommon in the pediatric population, these neoplasms have been associated with underlying conditions such as neurofibromatosis type two, and appropriate recognition is important to ensure close monitoring of potential future symptoms secondary to other tumors. We report the imaging and laboratory findings of an ancient schwannoma of the vagus nerve in a 10-year-old female, the first documented case of such a tumor in a pediatric patient, and discuss its characteristic findings and diagnostic considerations. Awareness of this rare tumor can help promote correct diagnosis and avoidance of costly, high-risk diagnostic methods.

The use of videos in preparation for pediatric otolaryngology cases-a national survey.

OBJECTIVE: 1) Review surgical preparation methods for pediatric otolaryngology fellows and fellowship directors, focusing on surgical video usage. STUDY DESIGN: Cross sectional survey. METHODS: Structured survey querying preparation methods for surgical cases was distributed to current pediatric otolaryngology fellows and fellowship program directors (FD's). RESULTS: 84 surveys were distributed (47 fellows, 37 FD). Overall response rate was 44% (37/84); fellow response rate was 55% (26/47) and FD response rate was 30% (11/37). Most respondents used videos (84%) and textbooks (95%) to prepare for surgery; fellows were more likely than FD's to use videos (96% vs. 55%, p < 0.01). 89% of respondents used YouTube to prepare; C-videos was the next most common platform used (27%). Fellows were more likely to have used YouTube than FD's (100% vs 63%, p < 0.01). 45% of FD's did not know or did not think their fellows use videos to prepare for cases. Mean helpfulness of surgical videos on a 5 point scale was 3.41 (95% CI 3.0-3.8). Videos were considered most helpful for illustrating technical portions of cases (51%), visualizing the case (27%) and reviewing anatomy (24%). Survey respondents mentioned poor quality (59%) and irrelevance to a particular institutions approach (19%) as weaknesses of available surgical videos. CONCLUSIONS: Surgical videos are commonly used by pediatric otolaryngology fellows to prepare for cases, and can assist in building anatomic knowledge and illustrating technical details of complex cases. YouTube is the most commonly utilized platform accessed by fellows, but poor quality and limited generalizability may restrict the usefulness of current video resources. LEVEL OF EVIDENCE: 4.

Fourth branchial cleft sinus with foreign material within thyroid gland.

Fourth branchial cleft anomalies are rare lesions that present diagnostic dilemmas to otolaryngologists. The report presented is a case of a 17-year-old female with food matter and abscess within the thyroid gland that were associated with a 4th branchial cleft sinus. A thyroid ultrasound revealed a 3.0 x 1.5 x 2.5-cm lesion with abnormal echogenicity that was concerning for early abscess. The patient subsequently underwent direct laryngoscopy and right thyroid lobectomy. Her symptoms resolved after surgery. This case demonstrates an unusual presentation in which food passed through the tract to the thyroid gland itself.

Surgical Outcomes for the Treatment of Velopharyngeal Insufficiency in 22q11.2 Deletion Syndrome.

This study aimed to compare outcomes of concomitant palatoplasty and sphincter pharyngoplasty with pharyngeal flap and sphincter pharyngoplasty alone for the treatment of velopharyngeal insufficiency in patients with 22q11.2 deletion syndrome. Thirty-one cases were identified for inclusion in the study. Patients were separated into 3 surgical groups: combined palatoplasty and sphincter pharyngoplasty (n = 11), pharyngeal flap (n = 7), and sphincter pharyngoplasty (n = 13). Outcome measures included perceptual speech analyses, surgical complications, and revision rates. There were no differences in preoperative speech analysis scores (P = .31). The combined palatoplasty and sphincter pharyngoplasty procedure had similar speech outcomes compared to pharyngeal flap, and both were significantly better than sphincter pharyngoplasty alone. Complication rates (P = .61) and the need for revision surgery (P = .25) were similar among all 3 groups. Concomitant palatoplasty and sphincter pharyngoplasty may be an alternative treatment for velopharyngeal insufficiency in children with 22q11.2 deletion syndrome.

Sleep and Speech Outcomes After Superior Adenoidectomy in Children with Cleft Palate.

Objective To describe the sleep and speech outcomes in patients with cleft palate who underwent superior adenoidectomy. Subjectives and methods This is a case series with chart review of patients with diagnoses of cleft palate and sleep disordered breathing (SDB), obstructive sleep apnea (OSA) or nasal obstruction treated with superior adenoidectomy from 1991-2015 at the Children's Hospital of Minnesota. Postoperative clinic notes documented the changes in symptoms following surgery. All speech outcomes were recorded. Results Fifty patients (23 females, 27 males) aged 11 months to 17 years were included. Forty-six patients (92%) had improvement of sleep symptoms including snoring, nighttime restlessness, and witnessed apnea events, following superior adenoidectomy. Forty-two of the 46 patients (91%) had stable speech postoperatively with either no development or no worsening of velopharyngeal insufficiency (VPI). Conclusion Superior adenoidectomy is an effective procedure to alleviate symptoms of sleep disordered breathing in patients with cleft palate without significantly affecting speech.