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.

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.

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.

Percutaneous Enteral Feeding in Patients With 22q11.2 Deletion Syndrome.

OBJECTIVE: To describe the incidence of percutaneous enteral feeding in patients with 22q11.2 deletion syndrome (22q11.2 DS) and determine factors associated with the need for percutaneous enteral feeding tube placement. DESIGN: Retrospective chart review. METHODS: The records of a 22q11.2 DS clinic and pediatric otolaryngology clinic at a tertiary pediatric hospital were reviewed from January 1, 2009, to December 31, 2019. All patients with confirmed 22q11.2 deletion were identified. Cardiac, otolaryngological, and feeding characteristics were recorded along with surgical history. A patient was defined to have a G-tube if the history was significant for any percutaneous gastric feeding tube placement, including a gastrostomy tube, gastrostomyjejunostomy tube, or a Mickey button. RESULTS: One hundred ninety patients with confirmed 22q11.2 DS by genetic testing were included. Thirty-three percent (n = 63) required G-tube placement. G-tube placement was associated with cardiac diagnosis (P < .01), history of cardiac surgery (P < .01), aspiration (P < .01), nasopharyngeal reflux (P < .01), subglottic stenosis (P < .01), laryngeal web (P = .003), and tracheostomy (P < .01). This suggests these conditions are associated with higher rates of G-tube placement in the 22q11.2 DS population. CONCLUSIONS: Patients with 22q11.2 DS often require supplemental nutritional support in the form of G-tube feeding, most often in the first year of life. Congenital heart abnormalities and surgery along with tracheostomy, subglottic stenosis, laryngeal web, aspiration, and nasopharyngeal reflux are significantly associated with the need for G-tube placement. Understanding associations between comorbid conditions and G-tube placement, especially those involving the head and neck, may assist with counseling of patients with 22q11.2 DS.

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.

Incidence of mandibular distraction osteogenesis in Stickler Syndrome: Variation due to COL2A1 and COL11A1.

OBJECTIVE: To determine whether the two most common genetic mutations seen in Stickler Syndrome (SS) (COL2A1 and COL11A1) affect the incidence of mandibular distraction osteogenesis (MDO) and what impact Robin sequence (RS) has on diagnosis. SS is an autosomal dominant connective tissue disorder characterized by almost complete penetrance. COL2A1 and COL11A1 are the two most common mutations seen in SS patients. SS often presents at birth with RS, which is characterized by the triad of micrognathia, glossoptosis, and tongue-based airway obstruction. MDO is one surgical intervention that has been shown to be successful in relieving tongue base obstruction and is the surgical intervention of choice for this condition. METHODS: A retrospective chart review was performed on all patients with a diagnosis of SS at a tertiary pediatric hospital between January 1, 2003 and December 31, 2018. The included patient charts were reviewed for demographic information, SS mutation, and history of MDO. Forty-six patients had a clinical diagnosis of SS. Of those, 31 met inclusion criteria which involved having a molecular diagnosis of SS and sufficient follow up information to determine if MDO was indicated or performed. Twenty-two of the 31 included patients had a diagnosis of RS (70.96%). Thirteen of the 31 patients (41.94%) included in this study required MDO as a neonate. RESULTS: Fifty-percent of patients with type I (COL2A1) required MDO as a neonate compared to only 31% of patients with type II (COL11A1), though the difference between the two groups was not statistically significant. CONCLUSION: The findings of this study suggest that patients with type I mutation may have a higher incidence of MDO than patients with a type II mutation, though further research with larger sample sizes is needed. This information is helpful in counseling those with SS or family history of SS about what they can expect related to RS and need for MDO based on genetic findings. LEVEL OF EVIDENCE: 3.

Nasal septal hematoma in children: Time to diagnosis and resulting complications.

OBJECTIVES: To determine the time from initial injury to diagnosis of nasal septal hematoma (NSH). Additional objectives included determining number of medical evaluations prior to diagnosis and long-term complications. METHODS: A retrospective chart review was performed on all patients diagnosed with NSH at a tertiary pediatric hospital between January 1, 2003 and April 1, 2019 were identified. Time to diagnosis was defined as time between initial trauma to date of diagnosis. Number of evaluations was defined as all medical evaluations prior to diagnosis. RESULTS: Of 2762 charts that were reviewed, 13 patients with NSH were identified. Of those, 92% were male and trauma was the cause in 85% of patients. Median time to diagnosis was 7 days (0-21 days), with an average of 2.2 evaluations (1-4 evaluations). Settings where diagnosis were missed included EDs (N = 9, 82%), primary care (N = 6, 55%), urgent care (N = 1, 9%) and otolaryngology clinic (N = 2, 18%). Four patients (31%) were evaluated by an otolaryngologist in the ED. The median time to otolaryngology outpatient visit was 7.5 days. In five patients (46%), septal hematomas were missed in multiple clinical settings. Seven patients (54%) experienced complications, including saddle nose deformity (N = 3, 23%). CONCLUSIONS: NSH is a rare but serious emergency. To avoid multiple visits and delay in diagnosis, additional education and awareness is needed for providers who evaluate these patients. Early diagnosis will reduce the risk of abscess formation and saddle nose deformity.

Submucous Cleft Palate Repair in Patients With 22q11.2 Deletion Syndrome.

OBJECTIVE: To determine whether surgical intervention for submucous cleft palate (SMCP) is more common in children with 22q11.2 deletion syndrome (22q DS) compared to children without 22q DS. DESIGN: Retrospective chart review. SETTING: Tertiary pediatric hospital and 22q11.2 DS specialty clinic. PARTICIPANTS: One hundred forty-two children seen at the tertiary hospital or clinic during a 20-year period (June 1999-June 2019) with documented SMCP with and without 22q DS. MAIN OUTCOME MEASURE: Percentage of children with SMCP with and without 22q DS requiring surgical intervention for velopharyngeal insufficiency. RESULTS: Patients with 22q DS had a significantly higher frequency of SMCP repair than those without 22q DS (89.7% vs 32.0%, P < .001, χ2 = 37.75). The odds of requiring SMCP repair were 18.6 times higher in those with 22q DS compared to those without (odds ratio = 18.6, CI = 6.1-56.6). CONCLUSIONS: This study provides new evidence suggesting patients with 22q DS require SMCP surgical repair for velopharyngeal insufficiency at a significantly higher rate than those without 22q DS. As the majority of patients with 22q DS with SMCP require surgical intervention, future prospective studies looking at early versus late repair of SMCP in patients with 22q DS are needed to guide the surgical repair timeline in this population.

Malnutrition as a Risk Factor in Cleft Lip and Palate Surgery.

OBJECTIVES/HYPOTHESIS: To assess the prevalence of acute and chronic malnutrition at the time of surgery in patients with cleft lip and/or palate (CLP) at our institution, and to quantify nutrition as a risk factor for postsurgical complications following CLP surgery. STUDY DESIGN: Retrospective cohort study. METHODS: Retrospective review of 855 children undergoing initial cleft lip or palate surgery, or revision surgery after fistula/dehiscence of initial cleft repair. We measured acute and chronic malnutrition using World Health Organization Z-scores of weight-for-age and height-for-age, respectively, and noted any postsurgical fistula or dehiscence. RESULTS: Among patients with cleft lip, 22.3% were at least moderately chronically malnourished at the time of initial repair, and 17.5% were at least moderately acutely malnourished. Among patients undergoing initial repair of cleft palate, 20.9% were at least moderately chronically malnourished, and 8.1% were at least moderately acutely malnourished. Increasing nutritional status, as measured by height-for-age, predicts decreased odds of fistula (OR 0.78, P = .01) after cleft palate surgery. CONCLUSIONS: Chronic malnutrition significantly increases the risk of fistula formation in patient with cleft palate. Preoperative strategies to manage this risk and influence surgical timing can avoid morbid and costly postoperative complications. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2060-E2065, 2021.

Comparing autologous versus allogenic rib grafting in pediatric cleft rhinoplasty.

BACKGROUND: Patients with cleft lip with or without cleft palate suffer from varying degrees of nasal deformity, often requiring nasal reconstruction to provide improved form and function. Rib cartilage is an excellent source of grafting material for nasal reconstruction and is available either as an autologous or allogenic graft. There is a paucity of literature comparing outcomes of autologous and allogenic rib grafts in pediatric cleft rhinoplasty. METHODS: A retrospective chart review was performed on patients who underwent cleft rhinoplasty with autologous or allogenic rib grafting at a tertiary pediatric hospital between January 1, 2003 and December 31, 2017. Outcome data were gathered over a 6-month postoperative period. RESULTS: There were 23 cleft rhinoplasties performed with rib graft, 12 with autologous rib and 11 with allogenic rib. Those in the autologous group tended to be older than those in the allogeneic group (15.6 ± 4.4 v 12.4 ± 5.2 years, p = 0.13). The most common types of grafts used were columellar strut (20/23), shield graft (9/23), and unilateral or bilateral batten grafts (7/23). Length of stay was significantly longer for patients who underwent autologous rib grafting compared with those with allogenic rib grafting (25.8 ± 4.7 v 11.9 ± 7.2 h, p < 0.05). Each group reported one complication. CONCLUSION: Autologous and allogenic rib grafts are safe and effective in pediatric rhinoplasty. The most common grafts used in this sample were columellar strut, batten, and shield grafts. Autologous rib grafts were more likely to be used in older patients and require longer hospital stay compared to allogenic grafts.

Developing an Eye-Tracking Protocol to Determine Children's Visual Perception of Secondary Cleft Lip Deformity.

OBJECTIVE: To develop a protocol that will be used to measure children's perception of secondary cleft lip deformity (SCLD) using objective eye-tracking technology. DESIGN: Cross-sectional study. Data collection May and June of 2018. SETTING: Single tertiary care pediatric hospital with a well-established cleft team. PARTICIPANTS: Participants were recruited from a general pediatric otolaryngology clinic. Sixty participants from 4 age groups (5-6, 10, 13, and 16 years) were enrolled on a voluntary basis. INTERVENTION: Pediatric participants viewed images of children's faces while wearing eye-tracking glasses. Ten images with unilateral SCLD and 2 control images with no facial scarring were viewed as gaze was assessed. MAIN OUTCOME AND MEASURE: Successful gaze fixation was recorded across all age groups. RESULTS: This article illustrates the types of data generated from glasses-based eye tracking in children. All children, regardless of age, spent more time with their gaze on a SCLD images (mean = 4.23 seconds; standard deviation [SD] = 1.41 seconds) compared to control images (mean = 3.97 seconds; SD = 1.42). Younger age groups spent less time looking at specific areas of interest in SCLD images. CONCLUSION: In this pilot study, we were able to successfully use eye-tracking technology in children to demonstrate gaze preference and a trend toward visual perception of SCLD changing with age. This protocol will allow for a future study, with larger and more diverse populations. Better understanding of how SCLD is perceived among children and adolescents has the potential to guide future interventions for SCLD and other facial deformities in pediatric patients.

Vincristine-induced vocal cord paresis and paralysis in children.

OBJECTIVES: To describe three new cases of vincristine-induced vocal cord paresis or paralysis (VIVCPP) in children and to review the diagnosis and management of this neuropathy. METHODS: Retrospective case series. Diagnosis of VIVCPP was confirmed by laryngoscopy in all children. RESULTS: Less than 20 cases of VIVCPP in children have been previously documented in the literature. Of the three children in our case series, one had unilateral vincristine-induced vocal cord paresis and two had bilateral VIVCPP. The first two patients each had two separate episodes of paresis, lasting 4 months and 1 month respectively. In the last patient, whose medical course was complicated by many additional factors, vocal cord paralysis persisted for over three years. CONCLUSIONS: Clinicians must evaluate children with suspected VIVCPP for concomitant symptoms and signs of vincristine neuropathies and examine the vocal cords via laryngoscopy. The effects of vincristine neurotoxicity can be waxing and waning, demonstrate delayed onset and persist well beyond drug cessation. Further studies are needed to identify effective neuroprotectants and delineate appropriate vincristine dosing in patients with vincristine neurotoxicity and cancer.

Associations of sex, age and adiposity in endothelium-independent dilation in children.

OBJECTIVE: To examine the association of age, sex, and obesity status on endothelial-independent dilation (EID) among children and adolescents. STUDY DESIGN: This study examined 264 children (143 males) between 8 to 18 years old (mean ± SD: age = 14.3 ± 2.7 years). Endothelial-independent dilation was assessed via ultrasound imaging of the brachial artery following administration of 0.3 milligrams of sublingual nitroglycerin. A one-way analysis of variance with Bonferroni post hoc comparisons assessed sex-differences in percent peak EID dilation (EID%-peak) and EID area under the curve (EID%-AUC), while analysis of covariance (ANCOVA) adjusted for race, age, body mass index percentile (BMI-percentile), and brachial artery diameter. Multiple linear regression evaluated the association of sex, age, BMI-percentile, percent body fat (%BF), and brachial artery diameter on EID. RESULTS: Prior to adjustment, EID%-peak was significantly higher among females than males (mean ± SE: 26.9 ± 0.5% versus 22.9 ± 0.6%, p < 0.001, respectively); similar findings for EID%-AUC (4214% ± 105%·s versus 3398% ± 97%·s, p < 0.001) were observed. After adjusting for covariates, EID%-AUC remained consistent (p = 0.03) while EID%-peak was not significantly different between sexes (p = 0.21). EID%-peak was significantly higher among normal weight compared to obese participants (p = 0.04), while no differences were observed between obesity status after adjustment for brachial artery diameter (p = 0.64). Both unadjusted (p = 0.16) and adjusted EID%-AUC (p = 0.24) was not significantly different between obesity status. BMI-percentile was not associated with EID%-peak (p = 0.76) or EID%-AUC (p = 0.30). Additionally, %BF was not associated with EID%-peak (p = 0.56) or EID%-AUC (p = 0.15). After adjusting for brachial artery diameter, BMI-percentile, and age, males had lower EID%-AUC (p = 0.03) and lower but not significant EID%-peak (p = 0.21). SIGNIFICANCE: Vascular smooth muscle function was significantly lower among male children and adolescents, which is suggestive that impaired EID and increased cardiovascular disease risk among males may begin in childhood. Interestingly, obesity status and BMI-percentile was not associated with EID in children and adolescents after adjusting for brachial artery diameter.

Age and sex relationship with flow-mediated dilation in healthy children and adolescents.

Flow-mediated dilation (FMD) is a noninvasive technique used to measure conduit artery vascular function. Limited information is available on normative FMD values in healthy children and adolescents. The objective of this study was to assess relationships between age and sex with FMD across childhood and adolescence. Nine hundred and seventy-eight asymptomatic children (12 ± 3 yr, range 6-18 yr, 530 male) underwent ultrasonic brachial artery assessment before and after 5 min of forearm ischemia. Sex differences in FMD and baseline artery diameter were assessed using mixed linear models. Baseline artery diameter was smaller in females than males [2.96 mm (95% CI: 2.92-3.00) vs. 3.24 mm (3.19-3.28), P < 0.001] and increased with age across the cohort (P < 0.001). Diameter increased between ages 6 and 17 yr in males [from 2.81 mm (2.63, 3.00) to 3.91 mm (3.68, 4.14)] but plateaued at age 12 yr in females. Males had a lower FMD [7.62% (7.33-7.91) vs. 8.31% (7.95-8.66), P = 0.024], specifically at ages 17 and 18 yr. There was a significant effect of age on FMD (P = 0.023), with a reduction in FMD apparent postpuberty in males. In conclusion, the brachial artery increases structurally with age in both sexes; however, there are sex differences in the timing and rate of growth, in line with typical sex-specific adolescent growth patterns. Males have a lower FMD than females, and FMD appears to decline with age; however, these findings are driven by reductions in FMD as males near maturity. The use of age- and sex-specific FMD data may therefore not be pertinent in childhood and adolescence.