Objective To explore factors associated with caregiver psychological distress and family quality of life (QoL) in craniofacial microsomia (CFM). Design Online cross-sectional survey. Participants: Forty caregivers (38 mothers) of children with CFM. Outcome Measures: Pediatric Quality of Life Inventory (Family Impact Module), Perceived Stress Scale, Hospital Anxiety and Depression Scale, and PTSD Checklist. Results Most caregivers (75%) were in “borderline” or “concern” risk categories for QoL. Caregivers reported significantly more symptoms of anxiety, depression, stress, and PTSD than norms. Factors associated with caregiver distress and family QoL included the number of child CFM characteristics and prior surgeries, and caregivers’ healthcare satisfaction, life orientation, resilience, and relationship satisfaction. Conclusions This study represents a small, self-selected, clinically impacted group of caregivers. Yet, findings align with similar studies and highlight a subset of families in need of psychological support. The findings can be tentatively used to inform larger studies, screening practices, and intervention targets.
Treatment decision-making is an integral but complex part of healthcare, particularly in the context of craniofacial surgeries. The aim of the current study was to explore caregiver narratives to inform future surgical care delivery and best practice. 'Life Story' narrative interviews were conducted with US English-and Spanishspeaking caregivers (n = 62) of children aged 3-17 years with craniofacial microsomia (CFM). Extracts relating to treatment decision-making were inductively coded using Reflexive Thematic Analysis. Four themes were identified: 1) 'Grappling with Difference' exemplifies how participants dealt with having a child who was different; 2) 'Seeking Authoritative Guidance' illustrates how participants proactively pursued information about treatment options over several years; 3) 'In the Driving Seat' describes participants' beliefs about whether and how much to involve their child in treatment decisions; and 4) 'Post-Treatment Reflections' depicts participants' reflections of the decision-making experience. Surgeons and other healthcare providers are encouraged to use neutral and accessible language, to ensure families and children have a thorough understanding of all treatment pathways, and to engage in effective shared decision-making practices. Content predominantly focused on surgeries for ear reconstruction and hearing amplification. Future studies would benefit from examining other treatment decisions that caregivers are required to make.
Caregiver psychosocial adjustment can be impacted by their children's early treatment for craniofacial microsomia (CFM). A deeper understanding of caregiver early experiences is needed to advance family-centered clinical care. This study aimed to describe the range of caregiver experiences of CFM care for their young children to inform recommendations for providers. As part of the "Craniofacial Microsomia: Accelerating Research and Education" (CARE) program, health histories and narrative interviews were completed with a national sample of US caregivers (N=42) of children with CFM aged 3 to 17 years (mean=10.1, SD=4.5). Interviews (average 71 min) were in English (n=30) or Spanish (n=12) and primarily with mothers (93%). Thematic analysis was used to describe early care experiences. By age 3, 91% of children were diagnosed with hearing loss, 64% had participated in early developmental services, 38% had undergone at least one surgery, and 24% used hearing aids. Caregivers' experiences fit within themes of: (1) Burden of Care; (2) Adaptation; (3) Perceived Quality of Care; (4) Child's Physical Health; and (5) Early Surgical Interventions. CFM care in early childhood presented challenges for caregivers, which may be mitigated by health care providers reducing burdens of care, coordinating care, building trust, increasing CFM and hearing education, instilling hope, assisting with hearing aid access, referring to early interventions, screening for caregiver distress, linking caregivers to support services, promoting coping and social support, and preparing families thoroughly for surgeries.
ObjectiveTo describe characteristic CLEFT-Q response profiles and patterns in patients with cleft palate and/or lip (CP ± L).DesignRetrospective analysis using latent profile analysis (LPA) to categorize patient-reported outcome responses into distinct profiles.SettingTertiary care pediatric hospital with multidisciplinary cleft team.Patients, ParticipantsPatients aged 8-29 years with CP ± L completing CLEFT-Q questionnaires from September 2021 to June 2025 (n = 596). Total of 923 responses analyzed after excluding 94 responses from patients under 8 years.InterventionsAdministration of the 119-item CLEFT-Q questionnaire prior to clinic visits, measuring appearance, function, and quality of life.Main Outcome Measure(s)Six latent profiles identified through LPA based on CLEFT-Q scores. Secondary outcomes included profile stability and surgical effects.ResultsSix profiles of CLEFT-Q responses emerged among subjects: Universally Affected-Moderate (UA1, n = 228, 24.7%), Universally Affected-Severe (UA2, n = 119, 12.9%), Facial Appearance-Moderate (FA1, n = 183, 19.8%), Facial Appearance-Severe (FA2, n = 56, 6.1%), Social/School/Speech distress (SSS, n = 95, 10.3%), and Minimally Distressed (MD, n = 242, 26.2%). Patients in severe profiles (UA2 and FA2) were older (P < .001) and more likely to have complete cleft lip (P < .001), while patients in the MD profile were younger and more likely to have isolated cleft palate (P < .001). Among 279 patients with longitudinal CLEFT-Q data, 47.5% remained in the same profile at subsequent CLEFT-Q response, with longer follow-up intervals predicting profile change (P = .049).ConclusionsLatent profile analysis categorizes CLEFT-Q responses into six clinically meaningful profiles correlating with demographics and treatment outcomes. This approach may guide personalized cleft care, though additional studies are needed for validation.
ObjectiveTo evaluate how patient-reported outcomes change from pre- to post-operation after common procedures in patients with cleft lip and/or palate (CL/P), as well as compared to patients who did not undergo surgery.DesignRetrospective chart review.SettingTertiary care hospital in the United States.PatientsAll patients with CL/P who had multiple CLEFT-Q completions from 2021 to 2025.Main Outcome MeasureCLEFT-Q scores.ResultsTotally, 256 patients with 594 CLEFT-Q responses were included. Sixty (23.4%) patients received 66 craniofacial operations between CLEFT-Q completions. Of these operations, 16 (24.2%) were dental extractions/exposures, 15 (22.7%) rhinoplasties, 10 (15.2%) velopharyngeal insufficiency repairs, 6 (9.1%) alveolar bone grafts, 6 (9.1%) orthognathic surgeries, and 4 (6.1%) oronasal fistula repairs. Surgical patients had lower first CLEFT-Q scores across nearly all domains compared to those in the non-surgical cohort (P < .05). Patients who underwent rhinoplasty (n = 15) demonstrated increased face (64.1 vs 49.6, P = .006), nose (64.8 vs 43.3, P = .003), nostrils (62.0 vs 34.2, P = .001), teeth (59.5 vs 41.3, P = .008), and lips (63.5 vs 48.4, P = .007) scores post-operatively. Jaw scores increased following orthognathic surgery, though this did not reach significance (71.2 vs 55.8, P = .100). No other operation had a statistically significant impact on CLEFT-Q scores (P > .05).ConclusionsPatients who underwent surgery demonstrated improvements in post-operative CLEFT-Q scores compared to patients who did not undergo surgery during this surveyed period. Of those who underwent surgery, rhinoplasty was associated with the most significant improvements in patient facial perception post-operatively.
ObjectiveWhile studies have analyzed the quality of life (QOL) in children with craniofacial microsomia (CFM), little is known about QOL in adults with CFM. This study compares QOL in adults with CFM to a comparative group using a validated questionnaire.DesignCross-sectional.SettingTertiary care center.Patients, ParticipantsAdults aged 18 years or older with CFM were contacted and administered the World Health Organization (WHO) QOL Biomedical Research and Educating Foundation questionnaire.Main Outcome Measure(s)Quality of life in adults with CFM.ResultsOne hundred and fourteen patients met inclusion criteria, 40 (35.1%) were successfully contacted, and 28 (24.6%) completed the questionnaire in 8.6 ± 2.6 min. Respondents were 23.3 ± 4.6 years old (minimum 18.4, maximum 32.8 years) with 16 (57.1%) females and 12 (42.9%) males. This cohort of adults with CFM scored lower in the physical domain (13.8 ± 1.7 vs 15.5 ± 3.2, P < .01), similar in the psychological (14.7 ± 1.9 vs 13.8 ± 3.2, P > .05), and higher in both the social (16.0 ± 3.2 vs 13.2 ± 3.2, P < .01) and environmental (16.8 ± 1.6 vs 11.7 ± 2.7, P < .01) domains than the WHO norm sample. There was no association between overall phenotypic severity or craniofacial surgical burden and QOL (P > .05).ConclusionsAdults treated for CFM perceive their physical health QOL to be lower than the WHO norms but report higher QOL in the social and environmental domains. Adult patients with CFM may demonstrate resilience and can live high-quality lives. These results underscore that their lives are not defined by CFM.
Objective To describe how the psychosocial status of patients with cleft lip and/or palate (CL/P) relates to patient-reported outcomes (PROs). Design Cross-sectional retrospective chart review. Setting Tertiary care pediatric hospital. Patients/Participants Patients aged 8 to 29 years attending cleft team evaluations during a 1-year period. Main Outcome Measures CLEFT-Q. Results Patients (N = 158) with isolated or syndromic CL/P and mean age 13.4 ± 3.0 years were included. Fifteen (9%) patients had siblings who also had CL/P. Of 104 patients who met with the team psychologist, psychosocial concerns were identified in 49 (47%) patients, including 25 (24%) with Attention-Deficit/Hyperactivity Disorder or behavior concerns, 28 (27%) with anxiety, and 14 (13%) with depression or mood concerns. Younger age and having siblings with cleft were associated with better PROs, while psychosocial concerns were associated with worse PROs on Speech, Psychosocial, and Face Appearance scales. Conclusions Patient perception of cleft outcomes is linked to psychosocial factors.
INTRODUCTION:Little is known about rates of suicidal ideation and behavior among youth with cleft lip and/or palate (CLP) and other craniofacial conditions. METHODS:Records of patients ages 6 and older who were administered the Columbia-Suicide Severity Rating Scale (C-SSRS) Lifetime Version during routine multidisciplinary cleft or craniofacial team visits between 2019 and 2023 were examined. Demographics information, C-SSRS data, and diagnoses were assessed with statistics including t tests, the Fisher exact test, and odds ratios. RESULTS:A total of 1140 C-SSRS questionnaires across 602 (433 CLP and 169 craniofacial) patients with an average age of 11.2±3.7 years were included. Eighty-four (13.6%) patients endorsed lifetime suicidal ideation, 9 (1.5%) had at least one instance of suicidal behavior, 30 (5.0%) endorsed nonsuicidal self-injury, and 2 (0.3%) engaged in self-injurious behavior. Compared with CLP, those with other craniofacial conditions had similar odds of endorsing suicidal ideation and behavior ( P ≥0.05). Compared with those with isolated cleft palates, CLP had greater odds of endorsing suicidal ideation and behavior, though those differences were not significant ( P ≥0.05). Incidence of suicidality was unchanged before, during, and after the COVID-19 pandemic ( P ≥0.05). Dividing patients by sex or insurance type revealed no difference in suicidality ( P ≥0.05). CONCLUSION:Patients with CLP and craniofacial conditions have a high incidence of suicidal ideation and behavior, though levels are similar between these groups. Suicidality in these patients was not negatively impacted by the COVID-19 pandemic. Early identification of safety risks and psychosocial challenges through regular screening can facilitate connection with appropriate clinical interventions.
Objective Children with craniofacial microsomia (CFM) have complex healthcare needs, resulting in evaluations and interventions from infancy onward. Yet, little is understood about families’ treatment experiences or the impact of CFM on caregivers’ well-being. To address this gap, the NIH-funded ‘Craniofacial microsomia: Accelerating Research and Education (CARE)’ program sought to develop a conceptual thematic framework of caregiver adjustment to CFM. Design Caregivers reported on their child's medical and surgical history. Narrative interviews were conducted with US caregivers ( n = 62) of children aged 3-17 years with CFM. Transcripts were inductively coded and final themes and subthemes were identified. Results Components of the framework included: 1) Diagnostic Experiences, including pregnancy and birth, initial emotional responses, communication about the diagnosis by healthcare providers, and information-seeking behaviors; 2) Child Health and Healthcare Experiences, including feeding, the child's physical health, burden of care, medical decision-making, surgical experiences, and the perceived quality of care; 3) Child Development, including cognition and behavior, educational provision, social experiences, and emotional well-being; and 4) Family Functioning, including parental well-being, relationships, coping strategies, and personal growth. Participants also identified a series of “high” and “low” points throughout their journey and shared their priorities for future research. Conclusions Narrative interviews provided rich insight into caregivers’ experiences of having a child with CFM and enabled the development of a conceptual thematic framework to guide clinical care and future research. Information gathered from this study demonstrates the need to incorporate evidence-based psychological support for families into the CFM pathway from birth onward.
Objective To identify associations between scores on the CLEFT-Q and Columbia-Suicide Severity Rating Scale (C-SSRS) Lifetime Version in patients with cleft lip and/or palate (CLP). Design Prospective. Setting Tertiary care center. Patients, Participants Patients ages six and older administered both the CLEFT-Q questionnaire and C-SSRS survey between 2019 and 2024. Interventions Multidisciplinary care coordination facilitated by the team psychologist. Main Outcome Measure(s) Associations among demographics, CLEFT-Q responses, and suicidality. Results A total of 305 patients were included, 141 females (46.2%) and 164 males (53.8%). Fifty-one (16.7%) endorsed lifetime incidence of suicidal ideation, four (1.3%) endorsed suicidal behavior, 12 (3.9%) endorsed non-suicidal self-injury (NSSI), and one (0.3%) endorsed self-injurious behavior, intent unknown. Patients endorsing suicidal ideation had lower PROs in 12/13 categories on the CLEFT-Q questionnaire (p < 0.001). Those with suicidal behavior had lower PROs in three health-related quality of life categories (psychological function, p = 0.018; social function, p = 0.005; school function, p = 0.007), but no difference in other domains. A cutoff of ≤70 in the CLEFT-Q psychological function domain identified suicidal ideation with 72.9% sensitivity and 65.9% specificity and suicidal behavior with 100.0% sensitivity and 62.2% specificity. Conclusions Patients with cleft lip and/or palate have increased risks for psychosocial challenges that are often missed by healthcare providers. This study reveals that patient-reported outcomes are worse in those with CLP who endorsed suicidal ideation and behavior. Low PRO responses identify suicidality with moderate sensitivity and specificity. Patients with low scores should be offered safety screenings and psychosocial support, ideally by mental healthcare professionals.
Objective Measuring disability as a concept of impaired global function enables beneficiaries of treatment, the impact of treatment, and targets of health system investment to be rigorously assessed. Measures of disability are not well established for cleft lip and palate. This study aims to systematically review disability weight (DW) studies pertaining to orofacial clefts (OFCs) and identify methodological strengths and shortcomings of each approach. Design Systematic literature review of studies that met the following criteria: (1) peer-reviewed publication, (2) focus on disability valuation, (3) mention orofacial clefts, and (4) publication January 2001-December 2021. Setting None. Patients/Participants None. Interventions None. Main Outcome Measure(s) Disability weight method of valuation and the value itself. Results The final search strategy yielded 1,067 studies. Seven manuscripts were ultimately included for data extraction. The disability weights used in our studies, including those newly generated or taken from the Global Burden of Disease Studies (GBD), ranged widely for isolated cleft lip (0.0-0.100) and cleft palate with or without cleft lip (0.0-0.269). The GBD studies limited their consideration of cleft sequelae informing disability weights to impact on appearance and speech-related concerns, while other studies accounted for comorbidities such as pain and social stigma. Conclusions Current measures of cleft disability are sparse, inadequately reflect the comprehensive impact of an OFC on function and socialization, and are limited in detail or supporting evidence. Use of a comprehensive health state description in evaluating disability weights offers a realistic means of accurately representing the diverse sequelae of an OFC.
OBJECTIVE:To compare patient-reported outcomes (PROs) in internationally adopted patients with cleft lip and palate to those in non-adopted peers. DESIGN:Cross-sectional study. SETTING:Multidisciplinary cleft team at tertiary care hospital. PATIENTS:Patients aged ≥ 8 with cleft lip and palate attending routine cleft team evaluations September 2021 - September 2022. MAIN OUTCOME MEASURE:CLEFT-Q PRO scores. RESULTS:Sixty-four internationally adopted patients and 113 non-adopted patients with a mean age of 13 years were included. Compared to non-adopted peers, adopted patients demonstrated worse satisfaction with face appearance (mean 59 vs. 66, p = .044), speech function (mean 69 vs. 78, p = .005), and speech distress (mean 80 vs. 84, p = .032). No significant differences were observed on the nose, nostrils, teeth, lips, lip scar, jaws, psychological function, or social function scales (p > .05). Objective clinical evaluation corroborated these findings, with adopted patients demonstrating worse Pittsburgh Weighted Speech scores (mean 3.0 vs 1.9, p = .027) and greater incidence of articulation errors (64% vs 46%, p = .021). No significant differences were observed in rates of mood, anxiety, or behavior concerns identified on psychosocial assessment (p = .764). Among adopted patients, undergoing palatoplasty prior to adoption was associated with worse satisfaction with speech, appearance, school, and social function (p < .05). CONCLUSIONS:Patient-reported outcomes among internationally adopted adolescents and young adults with cleft lip and palate show slightly lower satisfaction with facial appearance and speech but otherwise demonstrate similar results to non-adopted peers on most appearance and psychosocial measures. PRO data correlated well with objective speech assessment and did not portend worse psychosocial function.
Abstract Introduction Pediatric Graves’ disease (GD) is associated with hyperthyroid symptoms broadly impacting psychosocial functioning. Total thyroidectomy (TT) is a definitive treatment option that replaces long-term antithyroid medication (ATD) for GD. While studies have examined health-related quality of life (hrQOL) in adults, there are no data describing impacts of definitive surgery in pediatrics. In this prospective, longitudinal study, we explored the impact of definitive surgery on hrQOL and psychosocial functioning in adolescent patients with GD. Methods Patients 12-19 years old pursuing definitive surgery for GD and their parents were recruited to complete surveys before and at least six months following TT. Parents pre-operatively completed a demographic questionnaire, parent rating scale assessing perceived symptom severity, and motivations for pursuing TT questionnaire. Patients pre- and post-operatively completed the Pediatric Quality of Life Scales (PedsQL), Quality of Life Questionnaire for Patients with Thyroid Disease (ThyPRO), Perceived Stigmatization Questionnaire (PSQ), Body Esteem Scale for Adolescents and Adults (BESAA), and European Group on Graves’ Orbitopathy GO Quality of Life Questionnaire (EUGOGO-QOL). Patients and parents post-operatively completed the Patient and Observer Scar Assessment Scale (POSAS) and a post-surgical satisfaction scale. Longitudinal paired scores were compared using Wilcoxon rank-sum test with Bonferroni-Holm correction. Survey subscore associations were assessed using Spearman tests with Benjamin-Hochberg correction. P-values < 0.10 for survey association analyses and all other p-values < 0. 05 were considered statistically significant. Results Thirty-six patient/parent dyads completed baseline surveys, including 19 patient/parent dyads completing both pre- and post-TT surveys. Families reported that patients’ GD and ATD significantly interfered with quality of life. Motivations for definitive surgery included ease of monitoring hypothyroidism as opposed to hyperthyroidism, access to a 'high-volume' surgeon, desire to reduce goiter symptoms, and opposition to radioactive medication. At baseline, patients reported high physical and cognitive GD symptomology via ThyPRO, specifically hyperthyroid symptoms, tiredness, anxiety, and emotional susceptibility. Psychosocial functioning via PedsQL, specifically in school settings, was depressed. Disease-specific hrQOL (ThyPRO) significantly improved following TT, with notable improvements in goiter (p=0. 002), hyperthyroid symptoms (p=0. 002), tiredness (p=0. 003), and anxiety (p=0. 003). Physical (p=0. 006) and school-related functioning (p=0. 003) improved post-TT (PedsQL), while variables captured by PSQ, BESAA, and EUGOGO-QOL were not significant pre- and post-TT. Reported GD-associated eye symptomology was minimal at baseline and further improved following surgery (p=0. 007). ThyPRO and PedsQL subscores were significantly associated at baseline and change after surgery, suggesting interconnectedness between disease-specific hrQOL and psychosocial functioning. Patients and parents reported median recovery of two months and high satisfaction with the outcomes of definitive surgery, with minimal scar appearance concerns. Conclusions In the setting of a high-volume surgeon with low complication rates, definitive surgery for GD in pediatric populations has substantial, beneficial effects on disease-specific hrQOL and psychosocial functioning, with minimal adverse scar appearance complaints. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m., Sunday, June 12, 2022 12:48 p.m. - 12:53 p.m.
BACKGROUND:Secondary alveolar bone grafting of autologous bone from the iliac crest is a widely accepted modality for repair of residual alveolar cleft in patients with mixed dentition with previously repaired complete cleft lip and palate. There remains debate regarding surgical timing and preoperative, perioperative, and postoperative management of these cases.METHODS:This retrospective study reviewed patient demographic information in addition to preoperative, perioperative, and postoperative course to evaluate how patient and practice factors impact graft outcomes as assessed by three-dimensional cone beam computed tomographic evaluation at the 6-month postoperative visit.RESULTS:On univariate analysis, age at operation older than 9 years, history of oronasal fistula, history of cleft lip or palate revision, and history of international adoption were all found to significantly increase likelihood of graft failure (p < 0.05). On multivariate analysis, age older than 9 years was found to be the single most significant predictor of graft failure (p < 0.05). There was no significant difference in graft outcomes between patients with unilateral or bilateral cleft lip and palate, and no single variable was found to significantly correlate to increased complication rates. The graft success rate overall of the authors' practice was 86.2 percent, with a complication rate of 7.7 percent.CONCLUSIONS:Secondary alveolar bone grafting is an integral part of the cleft lip and palate surgical treatment series; this study identified several outcome predictors for both graft failure and adverse events, the most significant of which was age at operation. Although the mixed dentition phase often extends to 12 years of age, it is recommended that bone grafting be performed before 9 years of age to optimize outcomes.CLINICAL QUESTIONS/LEVEL OF EVIDENCE:Risk, III.
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.
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.
Background: Facial palsy can have significant functional and psychological impact. Dynamic facial reanimation methods have provided means of restoration of smile. There remains a dearth of quality of life data in children. The authors aim to assess patient reported outcomes in this underreported population. Methods: A retrospective review was performed of pediatric patients with facial palsy who had dynamic facial reanimation from 2009 to 2017 at a single institution. Patients were sent a digital Facial Clinimetric Evaluation (FaCE) questionnaire, a validated tool to assess patient quality of life. FaCE statements are grouped into 6 categories: facial movement, facial comfort, oral function, eye comfort, lacrimal control, and social control. Results: Twenty patients met inclusion criteria. Fourteen successfully completed the survey. With normal function being 100, our cohort scored: facial movement 38.7 +/- 14.3; facial comfort 67.3 +/- 23, oral function 75 +/- 22.7, eye comfort 68.75 +/- 18.2, lacrimal control 53.6 +/- 32.5, social function 64.3 +/- 25.6. The overall FaCE score was 61.07 +/- 10.85. The facial movement subscores were lower for brow raise 16.1 +/- 33.4 relative to smiling 53.6 +/- 29.2 and lip pucker, 46.4 +/- 23.7. No correlation was found between facial movement scores and total scores (R-2 < 0.01). There was a strong negative correlation between total scores and time since procedure (Spearman's rho R = -0.45). Conclusion: Our pediatric facial reanimation cohort demonstrated excellent social function, in contrast to a relatively lower facial movement score. The low facial movement score was mediated by low score for brow raise and was not correlated to overall FaCE score. Interestingly, overall FaCE score tended to decrease with time from procedure.
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.