Columbia-Suicide Severity Rating Scale (C-SSRS) Reveals High Rates of Suicidality in 602 Patients With Cleft and Craniofacial Conditions.

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.

A Conceptual Thematic Framework of Psychological Adjustment in Caregivers of Children with Craniofacial Microsomia.

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.

Psychosocial Status and Self-Perception in Patients with Cleft Lip and/or Palate.

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.

Long-Term Patient-Reported Outcomes in Internationally Adopted Children with Cleft Lip and Palate.

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.

Disability Caused by Cleft Lip and Palate: A Systematic Review and Critical Valuation Appraisal.

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.

Impact of Definitive Surgery for Graves' Disease on Adolescent Disease-Specific Quality of Life and Psychosocial Functioning.

Introduction: Pediatric Graves' disease (GD) is associated with hyperthyroid symptoms that impact psychosocial and physical functioning. Total thyroidectomy (TT) is a definitive treatment option that replaces antithyroid medication. While studies have examined health-related quality of life (QOL) in adults, there are no data describing impacts of TT in pediatrics. In this prospective longitudinal study, we explored the impact of TT on disease-specific QOL and satisfaction with TT and scar appearance in adolescent patients with GD undergoing TT. Methods: Patients 12-19 years old pursuing TT for GD and their parents were recruited to complete surveys before and at least 6 months after TT. Surveys assessed motivations for pursuing TT, QOL, perceived stigmatization, self-esteem, scar appearance, and surgery satisfaction. Paired scores were compared using Wilcoxon signed-rank tests, and subscore associations were assessed using Spearman association tests. Results: Thirty-seven patient-parent dyads completed baseline surveys, including 20 patient-parent dyads completing pre- and post-TT surveys. At baseline, patients reported physical and cognitive symptomology, including tiredness, anxiety, and emotional susceptibility through ThyPRO. Psychosocial functioning at school was low through PedsQL. Disease-specific QOL significantly improved after TT, with notable improvements associated with resolution of goiter (median change = -26.14, p = 0.003), hyperthyroid symptoms (median change = -43.75, p = 0.002), tiredness (median change = -26.79, p = 0.017), cognitive impairment (median change = -14.58, p = 0.035), anxiety (median change = -33.33, p = 0.010), and emotional susceptibility (median change = -28.99, p = 0.035). Physical (median change = 18.75, p = 0.005) and school-related functioning (median change = 30.00, p = 0.002) also significantly improved post-TT. Reported GD-associated eye symptomology (thyroid eye disease) was the second lowest scoring ThyPRO subscore at baseline and improved after surgery (median change = 14.06, p = 0.03). Families reported median recovery by two months, high satisfaction with the outcomes of TT, and minimal concerns over scar appearance. No permanent surgical complications (i.e., recurrent laryngeal nerve damage or hypoparathyroidism) were sustained. Conclusions: In the setting of a high-volume surgeon with low complication rates, TT for GD in pediatric populations may have substantial beneficial effects on disease-specific QOL and psychosocial functioning, with minimal adverse complaints about scar appearance.

Trends in insurance coverage for adolescent reduction mammaplasty.

BACKGROUND: Reduction mammaplasty is an effective intervention for symptomatic macromastia. Studies of insurance policy criteria for adult patients have demonstrated nonuniformity. This study assesses trends in insurance preauthorization for reduction mammaplasty in the adolescent population. METHODS: A retrospective cohort study of patients aged ≤18 years at a pediatric breast clinic between January 1, 2010 and December 31, 2020. Demographic information and clinical variables submitted to insurers were analyzed, as well as third-party payer company, provider network type, and preauthorization criteria. RESULTS: 249 preauthorization requests were studied, with an approval rate of 79.5%. Submissions increased from 6 in 2010 to 59 in 2020. Variables deemed significantly associated with preauthorization denial included submission before 2015 (OR 2.04, 95% CI 1.04-3.95, p = .038), billing zip code median income <$60,000 (OR 2.11, 95% CI 1.12-3.98 p = .02), predicted resection mass below Schnur Sliding Scale threshold (OR 1.97, 95% CI 1.01-3.83 p = .047), and insurance company. CONCLUSIONS: Insurance criteria for preauthorization of reduction mammaplasty in adolescents differ from adults, are not supported by clinical studies, and may exclude low-income patients from receiving care.

Secondary Alveolar Bone Grafting in Patients with Unilateral and Bilateral Complete Cleft Lip and Palate: A Single-Institution Outcomes Evaluation Using Three-Dimensional Cone Beam Computed Tomography.

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.

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.

Evaluating Quality of Life After Facial Reanimation Using the Facial Clinimetric Evaluation Scale in a Series of Pediatric Patients.

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 ±â€Š14.3; facial comfort 67.3 ±â€Š23, oral function 75 ±â€Š22.7, eye comfort 68.75 ±â€Š18.2, lacrimal control 53.6 ±â€Š32.5, social function 64.3 ±â€Š25.6. The overall FaCE score was 61.07 ±â€Š10.85. The facial movement subscores were lower for brow raise 16.1 ±â€Š33.4 relative to smiling 53.6 ±â€Š29.2 and lip pucker, 46.4 ±â€Š23.7. No correlation was found between facial movement scores and total scores (R2 < 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.

Sociodemographic Factors That Influence the Choice to Pursue Nasoalveolar Molding: One Pediatric Hospital's Experience.

OBJECTIVE: To identify demographic factors that influence choosing nasoalveolar molding (NAM) in the treatment of cleft lip with or without cleft palate (CL±P), and NAM treatment compliance. DESIGN: This work is a retrospective cohort study. SETTING: Tertiary pediatric hospital. PATIENTS, PARTICIPANTS: One hundred forty-nine patients with a diagnosis of unilateral complete CL±P receiving treatment when NAM was offered (January 1, 2008-July 26, 2016). MAIN OUTCOME MEASURE(S): Demographic variables collected included race, ethnicity, ZIP code, number of caregivers, caregiver employment status, and health insurance status. Medical variables collected included diagnosis, treatment pursued, compliance with NAM, completion of NAM, and the treating cleft surgeon and orthodontist. Data were analyzed via Fisher exact tests, χ2 tests, and multivariate logistic regression to identify factors that influence the decision to pursue NAM and treatment compliance. RESULTS: Univariate analyses identified the following significant factors predicting the pursuit of NAM: race and insurance type (both P < .001), surgeon (P = .005), income level (P = .009), comorbidities (P = .015), and syndromic diagnosis (P = .033). Driving distance trended toward significance (P = .078). Multivariate regression analyses indicated that Asian race (P = .047), insurance type (P = .046), driving distance (P = .019), and surgeon (P = .017) were significant predictors of pursuit of NAM. CONCLUSIONS: There are disparities in patient choice of NAM at our center for children with complete cleft lip. African American patient families were less likely to pursue this intervention. A stronger understanding of the barriers that lower income and minority patients face is needed in order to better characterize disparities in cleft care.

The Reducing Opioid Use in Children with Clefts Protocol: A Multidisciplinary Quality Improvement Effort to Reduce Perioperative Opioid Use in Patients Undergoing Cleft Surgery.

BACKGROUND: Cleft repair requires multiple operations from infancy through adolescence, with repeated exposure to opioids and their associated risks. The authors implemented a quality improvement project to reduce perioperative opioid exposure in their cleft lip/palate population. METHODS: After identifying key drivers of perioperative opioid administration, quality improvement interventions were developed to address these key drivers and reduce postoperative opioid administration from 0.30 mg/kg of morphine equivalents to 0.20 mg/kg of morphine equivalents. Data were retrospectively collected from January 1, 2015, until initiation of the quality improvement project (May 1, 2017), tracked over the 6-month quality improvement study period, and the subsequent 14 months. Metrics included morphine equivalents of opioids received during admission, administration of intraoperative nerve blocks, adherence to revised electronic medical record order sets, length of stay, and pain scores. RESULTS: The final sample included 624 patients. Before implementation (n =354), children received an average of 0.30 mg/kg of morphine equivalents postoperatively. After implementation (n = 270), children received an average of 0.14 mg/kg of morphine equivalents postoperatively (p < 0.001) without increased length of stay (28.3 versus 28.7 hours; p = 0.719) or pain at less than 6 hours (1.78 versus 1.74; p = 0.626) or more than 6 hours postoperatively (1.50 versus 1.49; p = 0.924). CONCLUSIONS: Perioperative opioid administration after cleft repair can be reduced in a relatively short period by identifying key drivers and addressing perioperative education, standardization of intraoperative pain control, and postoperative prioritization of nonopioid medications and nonpharmacologic pain control. The authors' quality improvement framework has promise for adaptation in future efforts to reduce opioid use in other surgical patient populations. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Facilitating Positive Psychosocial Outcomes in Craniofacial Team Care: Strategies for Medical Providers.

OBJECTIVE: Psychosocial issues associated with craniofacial diagnoses and the ongoing burden of care can impact the quality of life of patients and families, as well as treatment adherence and outcomes. Utilizing available literature and clinical expertise across 6 centers, the present article summarizes key psychosocial issues for the benefit of nonmental health medical providers and offers suggestions as to how all members of craniofacial teams can promote positive psychosocial outcomes. RESULTS: Family adjustment across developmental phases is outlined, with strategies to support adaptive parental coping. Teasing is a common concern in craniofacial populations and medical providers can promote coping and social skills, as well as link families to mental health services when needed. Academic issues are described, alongside suggestions for medical providers to assist families with school advocacy and ensure access to appropriate services within the school setting. Medical providers are key in preparing patients and families for surgery, including consideration of medical, social, and logistical supports and barriers. As craniofacial care spans infancy to adulthood, medical providers are instrumental in assisting patients and families to navigate treatment transition periods. In addition to ongoing clinical team assessments, medical providers may utilize screening measures to identify and track patient and family adjustment in multiple areas of team care. CONCLUSIONS: Multidisciplinary providers play an important role in supporting positive adjustment in patients affected by craniofacial conditions and their families.

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.

Management of velopharyngeal dysfunction in patients with 22q11.2 deletion syndrome: A survey of practice patterns.

OBJECTIVE: To determine demographics and practice patterns of surgeons treating velopharyngeal dysfunction (VPD) in patients with 22q11.2 deletion syndrome (22q11.2DS). METHODS: An anonymous electronic survey study was administered to the surgical membership of the American Cleft Palate-Craniofacial Association and the Society for Ear Nose and Throat Advances in Children. The survey queried surgeon demographics and differences in management practices for submucous cleft palate (SMCP), pharyngoplasty algorithms, and self-reported complications for nonsyndromic versus 22q11.2DS patients. RESULTS: 126 surveys were returned from 9 international regions with the majority from the United States (73%), followed by Western Europe (9.5%) and Canada (7.9%). Plastic surgery was the most common specialty (61.9%), followed by otolaryngology (27.8%). 88.1% reported fellowship training, and 33% completed multiple fellowships. Prior to proceeding with pharyngoplasty in 22q11.2DS patients, surgeons required the following assessments: speech evaluation (79.4%), velopharyngeal imaging (51.6%), cardiac evaluation (50.0%), carotid artery MRI (29.4%), and cervical spine x-rays (11.1%). Nasoendoscopy was the most common modality used for imaging the velopharynx. Overall, providers managed patients with 22q11.2DS similarly to nonsyndromic patients, with several significant exceptions including that they were more likely to perform SMCP repair alone as a first approach in nonsyndromic patients (p = 0.031) and posterior pharyngeal flap without SMCP repair in those with 22q11.2DS (p = 0.017). CONCLUSIONS: Practice patterns for the management of VPD in patients with 22q11.2DS vary across providers. Further collaborative studies are needed to develop optimal treatment paradigms for VPD in patients with 22q11.2 DS.