"I can't provide what my child needs": Early feeding experiences of caregivers of children with craniofacial microsomia.

PURPOSE: Craniofacial microsomia (CFM) is a congenital condition that can be associated with feeding challenges in infants. As part of the larger 'Craniofacial microsomia: Accelerating Research and Education (CARE)' program, this study described caregivers' early feeding experiences. DESIGN AND MATERIALS: US-based caregivers of 34 children with CFM participated in remote narrative interviews. Two authors completed inductive thematic analysis in an iterative process until consensus was reached. RESULTS: Caregivers' narratives outlined the inherent challenges of feeding an infant with special healthcare needs. The first theme 'Navigating Challenges and Managing Expectations' describes the distress participants experienced when they were unable to breastfeed and the negative emotional effect of switching to formula. The second theme 'Making Adaptations' outlines the methods participants tried, including breast pumps and feeding tubes. The third theme 'Accessing Support' describes participants' interactions with healthcare providers and challenges accessing feeding support. The final theme 'Growing from Adversity' recounts participants' relief once their child established a feeding pattern and the personal growth gained from their experiences. CONCLUSIONS: Caregivers reported several feeding related challenges associated with CFM, many of which negatively affected their wellbeing. Negative consequences were particularly pronounced in cases where caregivers' feeding experiences differed from their expectations. Participants identified challenges in accessing reliable feeding information and support. Despite difficult experiences, caregivers cited some positive outcomes, including increased confidence and resilience. PRACTICE IMPLICATIONS: Holistic feeding information and support for families affected by CFM should be inclusive of several feeding methods to improve care delivery, child health, and the caregiver experience.

Laryngeal Structural Dysphagia in Children.

The upper aerodigestive system is closely intertwined from an embryologic and functional perspective. Laryngotracheal anatomic abnormalities, such as laryngomalacia, stenosis, vocal cord paralysis, and laryngeal clefts, affect not only the respiratory function but also the swallow function. Laryngotracheal pathology can interfere with the suck-swallow-breathe mechanism in infants. It can also exacerbate gastroesophageal reflux. Chronic aspiration secondary to laryngotracheal anomalies can result in respiratory and pulmonary complications. Surgical treatment of laryngotracheal anomalies can also cause transient or long-term swallow dysfunction. Multidisciplinary approaches and clinical assessment of swallowing are important in patients with laryngotracheal pathology.

Early Experiences of Parents of Children With Craniofacial Microsomia.

OBJECTIVE: To describe the early health care experiences of parents of children with craniofacial microsomia (CFM), a congenital diagnosis often identified at birth. DESIGN: Qualitative descriptive. SETTING: Homes of participants. PARTICIPANTS: Parents of 28 children with CFM from across the United States. METHODS: We interviewed participants (27 mothers individually and one mother and father together) via telephone or teleconference and used reflexive thematic analysis to derive themes that represented early health care experiences of parents of children with CFM. RESULTS: Participants' narratives included detailed recounting of their birth and early care experiences. We identified two overarching themes. The first overarching theme, Stressors, included four subthemes that represented difficulties related to emotional reactions and negative experiences with health care providers. The second overarching theme, Finding Strength, included four subthemes that represented participants' positive adjustment to stressors through independent information seeking about CFM, adaptive coping, positive experiences with health care providers, and drawing on external supports. CONCLUSION: Participants often described early experiences as challenging. Findings have implications for improving early care, including increasing open and supportive communication by health care professionals, expanding access to CFM information, screening for mental health concerns among parents, strengthening coping among parents, and linking families to resources such as reliable online CFM information and early intervention programs.

Body Dysmorphic Disorder in Adult Patients With an Orofacial Cleft: An Unseen Psychological Burden.

OBJECTIVES: Facial dysmorphic disorder (FDD), a variant of body dysmorphic disorder, occurs when individuals are preoccupied with perceived defects in their facial appearance. Cleft lip and/or palate (CL/P) requires many clinical interventions and has significant psychological impacts on a patient's perception of appearance. This study identified psychological burdens related to living as an adult with CL/P and characterizes the degree of FDD symptoms in an adult craniofacial population. METHODS: This was a prospective, single-center, cross-sectional case-control study using semi-structured interviews and symptom assessments at a university-based craniofacial center. Patients without CL/P undergoing non-cosmetic facial surgery were recruited as controls (n = 20). Patients with an orofacial cleft (n = 30) were recruited from medical and dental providers at the University of North Carolina. Body Dysmorphic Disorder-Yale Brown Obsessive Compulsive Scale (BBD-YBOCS) scores were collected from a control population and patients with CL/P to assess FDD severity. RESULTS: Demographic factors such age, biological sex, and ethnicity had no significant impact on FDD symptom scores. Patient with CL/P were more likely to have significant FDD symptoms (BDD-YBOCS greater than 16) than patients without CL/P (OR 10.5, CI95 2.7-41.1), and had a mean difference in FDD symptoms scores of 10.04 (p < 0.0001; CI95 5.5-14.6). Patients with CL/P seen by a mental health provider in the past 3 months had 3-fold lower overall FDD symptom scores (OR 0.081; CI95 0.0085-0.77). CONCLUSIONS: Adults with CL/P would benefit from treatment for cleft-specific needs and psychological support as they face unique stressors related to their appearance, including an increase in FDD-associated symptoms. This study emphasizes the importance of recognizing psychological symptoms and providing ongoing multidisciplinary care to adults with CL/P. LEVEL OF EVIDENCE: 3; Individual case-control study Laryngoscope, 133:818-821, 2023.

Parental Reports of Intervention Services and Prevalence of Teasing in a Multinational Craniofacial Microsomia Pediatric Study.

ABSTRACT: Children with craniofacial microsomia (CFM) are at increased risk for educational and social concerns. This study describes intervention services and frequency of teasing in a multinational population of children with CFM. Caregivers of children with CFM ages 3 to 18 years in the US and South America were administered a questionnaire. Additional information was gathered from medical charts and photographs. Participants (N = 169) had an average age of 10.1 ±â€Š6.2 years, were primarily male (60%), and from the US (46%) or Colombia (32%). Most participants had microtia and mandibular hypoplasia (70%). They often had unilateral (71%) or bilateral (19%) hearing loss and 53% used a hearing aid. In the US, special education services were provided for 48% of participants enrolled in school; however, similar services were rare (4%) in South America and reflect differences in education systems. Access to any intervention service was higher in the US (80%) than in South America (48%). Caregivers reported children showed diagnosis awareness by an average age of 4.4 ±â€Š1.9 years. Current or past teasing was reported in 41% of the children, starting at a mean age of 6.0 ±â€Š2.4 years, and most often took place at school (86%). As half of the US participants received developmental and academic interventions, providers should screen for needs and facilitate access to services. Given diagnosis awareness at age 4 and teasing at age 6, providers are encouraged to assess for psychosocial concerns and link to resources early in treatment.

Haploinsufficiency of SF3B2 causes craniofacial microsomia.

Craniofacial microsomia (CFM) is the second most common congenital facial anomaly, yet its genetic etiology remains unknown. We perform whole-exome or genome sequencing of 146 kindreds with sporadic (n = 138) or familial (n = 8) CFM, identifying a highly significant burden of loss of function variants in SF3B2 (P = 3.8 × 10-10), a component of the U2 small nuclear ribonucleoprotein complex, in probands. We describe twenty individuals from seven kindreds harboring de novo or transmitted haploinsufficient variants in SF3B2. Probands display mandibular hypoplasia, microtia, facial and preauricular tags, epibulbar dermoids, lateral oral clefts in addition to skeletal and cardiac abnormalities. Targeted morpholino knockdown of SF3B2 in Xenopus results in disruption of cranial neural crest precursor formation and subsequent craniofacial cartilage defects, supporting a link between spliceosome mutations and impaired neural crest development in congenital craniofacial disease. The results establish haploinsufficient variants in SF3B2 as the most prevalent genetic cause of CFM, explaining ~3% of sporadic and ~25% of familial cases.

Cognitive, Motor, and Language Development of Preschool Children With Craniofacial Microsomia.

OBJECTIVE: To examine neurodevelopment in preschool-aged children with craniofacial microsomia (CFM) relative to unaffected peers. DESIGN: Multisite, longitudinal cohort study. SETTING: Tertiary care centers in the United States. PARTICIPANTS: We included 92 children with CFM ("cases") through craniofacial centers and clinics. Seventy-six children without CFM (controls) were included from pediatric practices and community advertisements. This study reports on outcomes assessed when participants were an average age of 38.4 months (SD = 1.9). MAIN OUTCOME MEASURES: We assessed cognitive and motor skills using the Bayley Scales of Infant and Toddler Development, third edition (Bayley-III), and language function using subtests from the Clinical Evaluation of Language Fundamentals-Preschool, second edition (CELF-P2). RESULTS: Case-control differences were negligible for Bayley-III cognitive (effect sizes [ES] = -0.06, P = .72) and motor outcomes (ES = -0.19, P = .25). Cases scored lower than controls on most scales of the CELF-P2 (ES = -0.58 to -0.20, P = .01 to .26). Frequency counts for "developmental delay" (ie, one or more scores > 1 SD below the normative mean) were higher for cases (39%) than controls (15%); however, the adjusted odds ratio = 1.73 (P = 0.21) was not significant. Case-control differences were most evident in children with microtia or other combinations of CFM-related facial features. CONCLUSIONS: Cognitive and motor scores were similar for preschool-aged children with and without CFM. However, children with CFM scored lower than controls on language measures. We recommend early monitoring of language to identify preschoolers with CFM who could benefit from intervention.

Behavioral Adjustment of Preschool Children With and Without Craniofacial Microsomia.

OBJECTIVE: The study aim was to assess behavioral adjustment in preschool children with and without craniofacial microsomia (CFM). DESIGN: Multisite cohort study of preschoolers with CFM ("cases") or without CFM ("controls"). PARTICIPANTS: Mothers (89%), fathers (9%), and other caregivers (2%) of 161 preschoolers. OUTCOME MEASURE: Child Behavior Check List (CBCL 1.5-5); linear regressions with standardized effect sizes (ES) adjusted for sociodemographic confounds. RESULTS: Child Behavior Check Lists for 89 cases and 72 controls (average age 38.3 ± 1.9 months). Children were male (54%), white (69%), and of Latino ethnicity (47%). Cases had microtia with mandibular hypoplasia (52%), microtia only (30%), or other CFM-associated features (18%). Nearly 20% of cases had extracranial anomalies. Composite CBCL scores were in the average range compared to test norms and similar for cases and controls. On the subscales, cases' parents reported higher Anxious/Depressed scores (ES = 0.35, P = .04), Stress Problems (ES = 0.40, P = .04), Anxiety Problems (ES = 0.34, P = .04), and Autism Spectrum Problems (ES = 0.41, P = .02); however, the autism subscale primarily reflected speech concerns. Among cases, more problems were reported for children with extracranial anomalies and certain phenotypic categories with small ES. CONCLUSIONS: Behavioral adjustment of preschoolers with CFM was comparable to peers. However, parental reports reflected greater concern for internalizing behaviors; thus, anxiety screening and interventions may benefit children with CFM. Among cases, more problems were reported for those with more complex presentations of CFM. Craniofacial microsomia-related speech problems should be distinguished from associated psychosocial symptoms during developmental evaluations.

Reduced pharyngeal dimensions and obstructive sleep apnea in adults with cleft lip/palate and Class III malocclusion.

Objective: To three-dimensionally evaluate the upper airway of individuals with cleft lip and palate (CLP) and Class III malocclusion and the occurrence of obstructive sleep apnea (OSA).Methods: Twenty-one CLP individuals with Class III malocclusion, 20-29 years of age, who underwent computed tomography for orthognathic surgery planning, were prospectively evaluated. All participants underwent polysomnography, and the apnea-hypopnea index ≥ 5 events/hour was considered indicative of OSA. The total upper airway and its subdivisions volumes, as well as the minimum pharyngeal cross-sectional area (CSA), were assessed using Mimics software.Results: Among the 21 individuals analyzed, 6 (29%) presented with OSA. The total upper airway and the oropharynx mean volumes were significantly decreased in subjects with OSA when compared to individuals without OSA. Mean CSA was not statistically different between groups.Conclusion: CLP individuals with Class III malocclusion and OSA have an upper airway significantly smaller than individuals without OSA.

Microtia and craniofacial microsomia: Content analysis of facebook groups.

OBJECTIVE: An increasing number of patients use social media for health-related information and social support. This study's objective was to describe the content posted on Facebook groups for individuals with microtia and/or craniofacial microsomia (CFM) and their families in order for providers to gain insight into patient and family needs and experiences to inform clinical care. METHODS: Two months of posts, images, comments, and "like" responses from two Facebook groups in the US and the UK were recorded and analyzed using content analysis. A secondary analysis identified statements of emotion. RESULTS: Posts (N = 254) had a total of 7912 "like" responses, 2245 comments, and 153 images. There were three categories of posts: seeking guidance (43%; 9 themes), promoting events/news (33%; 5 themes), and sharing experiences (24%; 3 themes). Across categories, 16% of posts had emotional content. Most comments were responding to posts seeking guidance, including medical care (20%), surgical care (9%), and hearing aids (5%). Promotional posts often aimed to increase CFM awareness. Posts sharing experiences were generally positive, with the highest number of "likes". CONCLUSIONS: Facebook groups members frequently exchanged health-related information, suggesting value placed on input from other families and the convenience of seeking information online. Posts also promoted awareness and shared experiences. Clinical care implications include the need for easily accessible accurate and tailored CFM-related health education. Additionally, providers should demonstrate awareness of health information on social media and may address the potential emotional impact of CFM by facilitating access to resources for social support.

Craniofacial Interventions in Children.

Craniofacial interventions are common and the surgical options continue to grow. The issues encountered include micrognathia, macroglossia, midface hypoplasia, hearing loss, facial nerve palsy, hemifacial microsomia, and microtia. In addition, a unifying theme is complex upper airway obstruction. Throughout a child's life the focus of interventions may change from airway management to speech, hearing, and language optimization, and finally to decannulation and procedures aimed at social integration and self-esteem. Otolaryngologists play an important role is this arena and provide high-quality care while continuing to expand what can be done for our patients.

Evaluation of prenatal diabetes mellitus and other risk factors for craniofacial microsomia.

OBJECTIVES: Craniofacial microsomia (CFM) is a congenital condition that typically involves hypoplasia of the ear and jaw. It is often associated with adverse effects such as hearing loss and sleep-disordered breathing. There is little research on its etiology. METHODS: We conducted a case-control study from maternal interview data collected from mothers of infants with and without CFM. The study included 108 children with and 84 children without CFM. Logistic regression with adjustment for demographic factors was used to evaluate associations between maternal exposures of interest and risk for CFM overall, as well as for different phenotypic sub-groups of children on the CFM spectrum. RESULTS: We found a statistically significant association between diabetes mellitus (DM) and CFM (OR 4.01, 95% CI 1.6-10.5). The association was slightly attenuated after adjustment for BMI. Higher parity was also associated with increased risk for CFM (OR 2.0, 95% CI 1.0-4.0). Vitamin A consumption and/or liver consumption was associated with a 70% lower risk compared with non-users (OR 0.3, 95% 0.1-0.8). Maternal age at the time of pregnancy was not associated with CFM. CONCLUSIONS: These analyses contribute evidence linking maternal DM with an elevated risk of having an infant with CFM, which is consistent with previous research and adds to the body of knowledge about the strength of this association. Further study is warranted to understand the potential mechanisms underlying the effect of DM in the developing embryo.

Methods and Challenges in a Cohort Study of Infants and Toddlers With Craniofacial Microsomia: The Clock Study.

OBJECTIVE: The Craniofacial microsomia: Longitudinal Outcomes in Children pre-Kindergarten (CLOCK) study is a longitudinal cohort study of neurobehavioral outcomes in infants and toddlers with craniofacial microsomia (CFM). In this article, we review the data collection and methods used to characterize this complex condition and describe the demographic and clinical characteristics of the cohort. SETTING: Craniofacial and otolaryngology clinics at 5 study sites. PARTICIPANTS: Infants with CFM and unaffected infants (controls) ages 12 to 24 months were recruited from the same geographical regions and followed to age 36 to 48 months. METHODS: Phenotypic, neurodevelopmental, and facial expression assessments were completed during the first and third waves of data collection (time 1 and time 3, respectively). Medical history data were taken at both of these time points and during an intermediate parent phone interview (time 2). RESULTS: Our cohort includes 108 cases and 84 controls. Most cases and controls identified as white and 55% of cases and 37% of controls identified as Hispanic. Nearly all cases had microtia (95%) and 59% had mandibular hypoplasia. Cases received extensive clinical care in infancy, with 59% receiving care in a craniofacial clinic and 28% experiencing at least one surgery. Study visits were completed at a study site (92%) or at the participant's home (8%). CONCLUSIONS: The CLOCK study represents an effort to overcome the challenges of characterizing the phenotypic and neurodevelopmental outcomes of CFM in a large, demographically and geographically diverse cohort.

Considering a Weight Criterion for Neonatal Tracheostomy: An Analysis of the ACS NSQIP-P.

OBJECTIVES: Neonates weighing < 2.5 kg have known higher rates of surgical mortality and morbidity, but this remains unexamined specifically for tracheostomy. We present outcomes of neonates undergoing tracheostomy stratified by weight. METHODS: Retrospective review of the American College of Surgeons National Surgical Quality Improvement Program-Pediatric from 2012 to 2014. Patients undergoing tracheostomy were included. Thirty-day mortality and major/minor complication rates were stratified by weight (group 1: < 2.5 kg; group 2: ≥ 2.5 kg and < 4 kg; group 3: ≥ 4 kg). Patient comorbidities were assessed for independent risk factors of morbidity and mortality. RESULTS: Of 183,233 patients, 543 underwent tracheostomy. Forty-four patients were group 1 (mean: 2.2 kg ± 0.25); 170 patients were group 2 (mean: 3.31 kg ± 0.42); and 329 patients were group 3 (mean: 6.4 kg ± 2.7). Between groups 1 and 2, there were no significant differences in mortality (P = 0.47), major complication rates (P = 0.99), or minor complication rates (P = 0.64). In comparing all three groups, there were no significant differences in mortality (P = 0.47), major complication rates (P = 0.80), or minor complication rates (P = 0.77). The overall 30-day mortality for all patients was 4.24%. In a multivariate logistical regression model, weight group did not change the odds of all negative outcomes (group 1: odds ratio [OR] of 0.71; 95% confidence interval [CI], 0.33-1.53 and group 2: OR of 0.78; 95% CI, 0.50-1.22). Bronchopulmonary dysplasia was the only independent significant predictor of major complications (OR, 1.69; 95% CI, 1.02-2.79) (P = 0.04). CONCLUSION: Our data indicate that 30-day mortality and morbidity outcomes for neonatal tracheostomy are not affected by weight. Overall 30-day mortality should be discussed with caregivers preoperatively. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:500-505, 2019.

Transoral robotic surgery for upper airway pathology in the pediatric population.

OBJECTIVES/HYPOTHESIS: The purpose of this study is to present one of the largest case series of pediatric transoral robotic surgery (TORS) in the upper airway demonstrating a wide range of ages and indications. STUDY DESIGN: A retrospective case series at an academic tertiary referral center from August 2010 to September 2014. METHODS: The da Vinci surgical robot (Intuitive Surgical, Inc., Sunnyvale, CA) was used on 16 pediatric patients for 18 procedures. A variety of upper airway pathologies and reconstructions in children with a wide range of ages and weights were treated. No lingual tonsillectomies or base-of-tongue reductions were included. RESULTS: Sixteen children (6 males) underwent 18 TORS procedures, including resection of hamartoma (n = 1), repair of laryngeal cleft (n = 7), removal of saccular cyst (n = 2), release of pharyngeal or esophageal strictures (n = 2), and excision of lymphatic malformations (n = 4). Patient ages ranged from 14 days to 15 years. There were no intraoperative complications. All patients had successful robotic access, and no patients had conversions to open or traditional endoscopic surgery. Hospital courses varied with duration ranging from 1 to 20 days. The median follow up was 22 months. CONCLUSION: Applying TORS to the pediatric population can be feasible and safe for appropriate airway pathologies. Because many patients are small in size, there is inherent risk in using robotic instruments and scopes transorally. Pearls in this series include a standardized two-robot experienced attending team and longitudinal airway follow-up. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:247-251, 2017.

Trends in medical malpractice claims in patients with cleft or craniofacial abnormalities in the United States.

OBJECTIVE: To describe medical malpractice trends in patients with cleft and/or craniofacial abnormalities. METHODS AND MATERIALS: A modified Delphi approach was used to gather search terms. Search settings included "all jury verdicts and settlements", with jurisdiction of "all states" and "all federal courts" (by court and circuit). A retrospective review of WestLawNext legal database was conducted. Cases were excluded if they did not have a direct association from the patient's craniofacial anomaly or if they were not related to malpractice. RESULTS: Forty-two cases met inclusion criteria. Cases closed between 1981 and 2014 were included. The mean payment among claims with an indemnity payment was $3.9 million. Of cases brought to trial, 62% were in favor of the plaintiff. Amongst physicians named as co-defendants, pediatricians were most commonly named (24%), followed by plastic surgeons (16%), obstetricians (7.8%), and radiologists (7.8%). "Missed diagnosis" was the most common type of negligent claim (45%), followed by "surgical error" (21%), and "medication error" (17%). "Anoxic brain injury" resulted in the highest median indemnity payment for complication of patient management ($3.5 million), followed by "wrongful birth" ($1.03 million), and "minor physical injury" ($520,000). No specific type of negligent claim (p = 0.764) nor complication of patient management (p = 0.61) was associated with a greater indemnity payment. Mean indemnity payment was $920,000 prior to 2001 and $4.4 million after 2001 (p = 0.058). CONCLUSIONS: Mean indemnity payments were fourteen-fold greater in patients as compared to those in the overall population ($3.9 million versus $274,887) and seven-fold greater than those in the average pediatric population ($3.9 million versus $520,923). All healthcare providers should be aware of the associated medical malpractice claims that may be incurred when treating patients at risk for these conditions.

A Multidisciplinary Children's Airway Center: Impact on the Care of Patients With Tracheostomy.

BACKGROUND: Children with complex airway problems see multiple specialists. To improve outcomes and coordinate care, we developed a multidisciplinary Children's Airway Center. For children with tracheostomies, aspects of care targeted for improvement included optimizing initial hospital discharge, promoting effective communication between providers and caregivers, and avoiding tracheostomy complications. METHODS: The population includes children up to 21 years old with tracheostomies. The airway center team includes providers from pediatric pulmonology, pediatric otolaryngology/head and neck surgery, and pediatric gastroenterology. Improvement initiatives included enhanced educational strategies, weekly care conferences, institutional consensus guidelines and care plans, personalized clinic schedules, and standardized intervals between airway examinations. A patient database allowed for tracking outcomes over time. RESULTS: We initially identified 173 airway center patients including 123 with tracheostomies. The median number of new patients evaluated by the center team each year was 172. Median hospitalization after tracheostomy decreased from 37 days to 26 days for new tracheostomy patients <1 year old discharged from the hospital. A median of 24 care plans was evaluated at weekly conferences. Consensus protocol adherence increased likelihood of successful decannulation from 68% to 86% of attempts. The median interval of 8 months between airway examinations aligned with published recommendations. CONCLUSIONS: For children with tracheostomies, our Children's Airway Center met and sustained goals of optimizing hospitalization, promoting communication, and avoiding tracheostomy complications by initiating targeted improvements in a multidisciplinary team setting. A multidisciplinary approach to management of these patients can yield measurable improvements in important outcomes.

Comprehensive quality of life outcomes for pediatric patients undergoing endoscopic sinus surgery.

BACKGROUND: Limited quality of life data exist for pediatric chronic rhinosinusitis (CRS) patients undergoing endoscopic sinus surgery (ESS). Further exploration of the following areas will enhance understanding and support clinical decision-making: baseline and post-ESS general and disease-specific quality of life, parent vs. child report, and correlation of nasal endoscopy to sinus CT scores. METHODOLOGY: A prospective cohort study evaluated CRS patients age 5-18 undergoing ESS. Surveys were completed at two timepoints: (1) pre-ESS and (2) 30-90 days post-ESS, with parents completing general (PedsQLTM) and CRS-specific (SNOT-16 and SN-5) quality of life surveys and children completing PedsQLTM and SNOT-16 surveys. Preoperative Lund-Kennedy nasal endoscopy and Lund-Mackay sinus CT scores were calculated. Where appropriate, outcomes were stratified by cystic fibrosis status. RESULTS: Impaired preoperative general quality of life was evidenced by parent proxy-report of PedsQLTM scores in 10 cystic fibrosis and 11 non-CF patients. ESS was associated with decreased sinus symptoms at 1-3 months postoperatively with SN-5 change scores of -1.85 and -2.2, in CF and non-CF patients, respectively. Parents reported worse CRS symptoms via higher preoperative SNOT-16 scores than their children did. Nasal endoscopy and sinus CT scores correlated with a Spearman correlation coefficient of 0.51. Scores not reaching statistical significance included CF-related CRS SNOT-16 change scores and PedsQLTM general quality of life change scores. CONCLUSION: In pediatric patients with CRS electing ESS, general quality of life is impaired preoperatively and sinus symptoms improve significantly 1-3 months after sinus surgery. Parents report statistically worse CRS symptom scores than their children do. Nasal endoscopy scores in this cohort correlated with sinus CT scores.