Developmental Risk for Infants with Cleft Lip with or Without Cleft Palate Based on Caregiver-Proxy Reports.

OBJECTIVES: (1) Assess caregiver-reported development in infants born with cleft lip ± alveolus (CL ± A) and cleft lip and palate (CLP); (2) determine factors associated with increased developmental risk; and (3) determine consistency of developmental risk before and after surgery for cleft lip. DESIGN: Prospective, longitudinal assessment of development. Time (T) 1, prior to lip closure; T2, 2 months post lip closure. SETTING: Three US craniofacial teams and online parent support groups. PARTICIPANTS: 123 total caregivers (96% mothers); 100 at T1, 92 at T2, and 69 at both T1 and T2. MEASURE: Ages and Stages Questionnaire-3 (ASQ-3): Communication, Gross Motor, Fine Motor, Problem Solving, Personal Social Domains. RESULTS: At T1 47%; at T2 42% passed all 5 Domains; 36% of infants pass all 5 Domains at both T1 and T2. Infants with CLP were at greatest risk on Communication [B = 1.449 (CI = .149-20.079), p = .038; Odds Ratio (OR) = 4.3 (CI = .923-19.650)] and Gross Motor Domains [B = 1.753 (CI = .316-20.605), p = .034; OR = 5.8 (CI = 1.162-28.671)]. Male infants were at greatest risk on Fine Motor [B = 1.542 (CI = .495-20.005), p = .009; OR = 4.7 (CI = 1.278-17.101)] and Problem Solving Domains [B = 1.200 (CI = .118-19.708), p = .044; OR = 3.3 (CI = .896-12.285)]. CONCLUSIONS: Based on caregiver report, infants with CL ± A and CLP meet referral criteria at a high rate. Infants with CLP and male infants were at greatest risk. Regular developmental screening is recommended.

Screening for Academic Risk Among Students With Cleft Lip and/or Palate: Patterns of Risk and Qualities of Effective Tools.

This study evaluated the effectiveness of academic screening measures in relation to parent-reported diagnoses.Multicenter, retrospective cohort study including structured interviews, questionnaires, and chart reviews.Six North American cleft centers.Children (n = 391) with cleft lip and/or palate, ages 8 to 10 years of age (192 male) and their guardians were recruited during regular clinic visits.Parent and child ratings on the Pediatric Quality of Life Inventory (PedsQL) School Scale, child report on CleftQ School Scale, parent report on the Adaptive Behavior Assessment System-Third Edition Functional Academics (ABAS-FA) Scale and Child Behavior Checklist (CBCL) School Competency Scale, parent interview, and medical chart review.Risk for concerns ranged from 12% to 41%, with higher risk reflected on the CBCL-SC compared to other measures. Males with cleft palate were consistently at the highest risk. Only 9% of the sample had a parent-reported diagnosis of a learning or language disability. Ratings from the ABAS-FA and CBCL-SC had the highest utility in identifying those with language and/or learning concerns.As cleft teams work to develop standardized batteries for screening and monitoring of patients, it is important to evaluate the effectiveness of measures in identifying those at highest risk. When screening for language and learning disorders, questions related to potential academic struggles, such as increased school effort or increased school distress, are most useful. Referrals for follow-up evaluation are recommended for those identified at high risk.

A Multisite Study Investigating Child and Parent Proxy Reported Quality of Life in Children With Cleft Lip and/or Palate.

This observational, multisite cohort study explored health-related quality of life (HRQoL) in children with cleft lip and/or palate (CL/P), including interrater agreement and ratings for this group relative to clinical cutoff scores and published means for healthy and chronically ill children.Participants (338 children ages 8-10 years, 45.9% male and their parents, 82.0% female) across 6 sites completed the Pediatric Quality of Life Inventory Generic Core Scales (PedsQL).Intraclass correlation revealed poor interrater agreement for most HRQoL domains. Although ratings were generally higher than those expected for children with a chronic illness, child ratings were below healthy means for school functioning, and parent proxy ratings were below healthy means for all domains except physical functioning. Lower ratings consistent with chronic illness means were found for self-reported emotional and psychosocial functioning in children with cleft lip and palate (CLP), as well as parent proxy-reported emotional, school, and psychosocial functioning for children with cleft palate (CP). Scores were most likely to be in the clinical range for children with CP for social, school, and total functioning.Although parent proxy report provides important information about observed functioning, poor interrater agreement indicates that both child and parent proxy reported HRQoL should be included in outcomes assessment for CL/P. HRQoL ratings may be higher for children with CL/P compared to youth with other chronic illnesses, but psychosocial functioning may be negatively impacted when compared with healthy youth, particularly for emotional, social, and school functioning in children with CLP or CP.

Psychosocial Outcomes in Children with Cleft Lip and/or Palate: Associations of Demographic, Cleft Morphologic, and Treatment-Related Variables.

OBJECTIVE: To determine associations of demographic, morphologic, and treatment protocol parameters with quality of life (QoL), appearance/speech satisfaction, and psychological adjustment. DESIGN: Observational study utilizing retrospective report of protocol variables and current outcome variables. SETTING: Six North American cleft treatment clinics. PARTICIPANTS: Children, ages 8.0-10.99 years, with Cleft Lip ± Alveolus, Cleft Palate, Cleft Lip and Palate, and parents (N = 284). OUTCOME MEASURES: Pediatric QoL Inventory (PedsQL): Parent, Child, Family Impact Module (FIM); Patient Reported Outcome Measurement Information System (PROMIS); Child Behavior Checklist (CBCL); CLEFT-Q. RESULTS: Outcome scores were average with few differences by cleft type. Multiple regression analyses yielded significant associations (Ps < .05) between socioeconomic status, race, and age at assessment and parent- and self-reported measures. Females had higher PROMIS Depression (ß=.20) but lower CBCL Affective (ß = -.16) and PROMIS Stigma scores (ß= -.24). Incomplete cleft lip was associated with lower PROMIS Depression, and more positive ratings of CLEFT-Q: Nose, Nostril, Lip Scar; CBCL Competence scores, (ßs = -.17 to .17). Younger Age at Lip Closure was associated with higher CBCL School Competence (ß= -.18). Younger Age at Palate Closure was associated with higher Child PedsQL Total, Physical, Psychosocial QoL, and better CLEFT-Q Speech Function (ßs = -.18 to -.15). Furlow Palatoplasty was associated with more CBCL Externalizing Problems (ß = .17) higher CBCL Activities (ß = .16). For all diagnoses, fewer Total Cleft-Related Surgeries was associated with lower PROMIS Stigma and higher CBCL Total Competence and Activities (ßs = -.16 to .15). CONCLUSIONS: Demographic characteristics, lip morphology, and treatment variables are related to later psychological functioning.

Observations by Caregivers Using the Infant with Clefts Observation Outcomes Instrument (iCOO): A Comparison of Three Versus Seven-day Daily Diaries.

OBJECTIVE: Our goal was to compare data collected from 3- and 7-day Infant with Clefts Observation Outcomes (iCOO) diaries. DESIGN: Secondary data analysis of an observational longitudinal cohort study. Caregivers completed the daily iCOO for 7 days before cleft lip surgery (T0) and for 7 days after cleft lip repair (T1). We compared 3- and 7-day diaries collected at T0 and 3- and 7-day diaries collected at T1. SETTING: United States. PARTICIPANTS: Primary caregivers of infants with cleft lip with and without cleft palate (N = 131) planning lip repair and enrolled in original iCOO study. MAIN OUTCOMES MEASURE(S): Mean differences and Pearson correlation coefficients. RESULTS: Correlation coefficients were high for global impressions (>0.90) and scaled scores (0.80-0.98). Mean differences were small across iCOO domains at T0. T1 comparisons reflected the same pattern. CONCLUSIONS: Three-day diary data is comparable to 7-day diaries for measuring caregiver observations using iCOO across T0 and T1.

Exploration of Caregiver Interrater Agreement and Test-Retest Reliability on the Infant Cleft Observer Outcomes (iCOO).

Caregiver and observer-reported measures are frequently used as outcomes for research on infants and young children who are unable to report on their own health. Our team developed the Infant with Clefts Observation Outcomes Instrument (iCOO) for infants with cleft lip with or without cleft palate. This exploratory study compared test-retest and interrater reliabilities to inform whether differences in caregiver perspective might affect the iCOO.This study is a secondary analysis comparing caregiver interrater agreement to test-retest reliability. Twenty-five pairs of caregivers completed the iCOO before surgery, 1 week later for test-retest reliability, 2 days after surgery, and 2 months after surgery. Reliability was assessed using intraclass correlations (ICCs) and t-tests were used to compare ratings between caregivers.Infants had cleft lip (28%) or cleft lip and palate (72%). Primary caregivers were predominantly mothers (92%) and secondary caregivers were predominantly fathers (80%). Test-retest reliability met psychometric standards for most items on the iCOO (81%-86% of items). Caregiver agreement on the iCOO items was lower than test-retest reliability (33%-46% of items met psychometric standards). Caregivers did not systematically differ in whether they rated infants as healthier or less healthy than the other caregiver (5%-16% of items had statistically significant differences).Caregivers used the measure consistently, but had different experiences and perceptions of their infant's health and functioning. Future studies are needed to explore mechanisms for the differences in test-retest and interrater reliability. Whenever possible, the same caregiver should provide ratings of the infant, including on the iCOO.

Infant with Clefts Observation Outcomes Instrument (iCOO): A New Outcome for Infants and Young Children with Orofacial Clefts.

OBJECTIVE: We evaluated the measurement properties for item and domain scores of the Infant with Clefts Observation Outcomes Instrument (iCOO). DESIGN: Cross-sectional (before lip surgery) and longitudinal study (preoperative baseline and 2 days and 2 months after lip surgery). SETTING: Three academic craniofacial centers and national online advertisements. PARTICIPANTS: Primary caregivers with an infant with cleft lip with or without cleft palate (CL ± P) scheduled to undergo primary lip repair. There were 133 primary caregivers at baseline, 115 at 2 days postsurgery, and 112 at 2 months postsurgery. MAIN OUTCOME MEASURE(S): Caregiver observation items (n = 61) and global impression of health and function items (n = 8) across eight health domains. RESULTS: Mean age at surgery was 6.0 months (range 2.7-11.8 months). Five of eight iCOO domains have scale scores, with Cronbach's alphas ranging from 0.67 to 0.87. Except for the Facial Skin and Mouth domain, iCOO scales had acceptable intraclass correlation coefficients (ICCs) ranging from 0.76 to 0.84. The internal consistency of the Global Impression items across all domains was 0.90 and had acceptable ICCs (range 0.76-0.91). Sixteen out of 20 (nonscale) items had acceptable ICCs (range 0.66-0.96). As anticipated, iCOO scores 2 days postoperatively were generally lower than baseline and scores 2 months postsurgery were consistent with baseline or higher. The iCOO took approximately 10 min to complete. CONCLUSIONS: The iCOO meets measurement standards and may be used for assessing the impact of cleft-related treatments in clinical research and care. More research is needed on its use in various treatment contexts.

Caregiver Observations of Infant Well-Being Before and After Cleft Lip Surgery.

OBJECTIVE: To evaluate the sensitivity to change of daily ratings of the comfort (COMF) and behavioral/emotional health (BEH) domains of the Infants with Clefts Observation Outcomes Instrument (iCOO) at 3 time points, and to assess the association of post-surgical interventions on iCOO ratings. DESIGN: The COMF and BEH domains were completed by caregivers before (T0), immediately after (T1), and 2-months after (T2) cleft lip (CL) surgery. Analyses included descriptive statistics, correlations, t-tests, and generalized estimating equations. PARTICIPANTS: Caregivers (N = 140) of infants with CL with/without cleft palate. MAIN OUTCOME MEASURES: The COMF and BEH domain scores of the iCOO: Scale (SCALE), a summary of observable signs; and Global Impression (IMPR), a single item measuring caregivers' overall impression. RESULTS: Daily COMF and BEH SCALE and IMPR scores changed significantly during T1 (P's < 0.001) but not T0 or T2. Day 1 and 7 T0 scores were significantly higher than Day 1 and 7 T1 scores (P's <0.001 to <0.012) but similar at T2 (P's > 0.05). After CL surgery, the combined use of immobilizers and nasal stents and the use of multiple feeding methods with treatment for gastroesophageal reflux were associated with lower daily scores in COMF and BEH SCALE and IMPR (P's: 0.040 to <0.001). CONCLUSIONS: COMF and BEH iCOO scores were sensitive to daily changes in infant well-being following CL surgery. Future studies should further investigate impact of post-surgical treatments on infant well-being.

Retrospective Evaluation of Number of Surgeries and Parent Ratings of Academic and Behavioral Functioning Among Children With Isolated Oral Clefts.

OBJECTIVE: The purpose of this study was to evaluate possible relationships between number of surgeries and parent ratings of academic functioning among children with isolated oral clefts. DESIGN: Multicenter, retrospective cohort study including structured interviews, questionnaires, and chart reviews. SETTING: Completion of questionnaires occurred during clinical visits at 6 different cleft centers across North America. PARTICIPANTS: Parents of 285 children with isolated clefts of the lip and/or palate, aged 8 to 10 years old, participated in structured interviews and completed questionnaires regarding the academic and behavioral functioning of their children. MAIN OUTCOME MEASURES: Parent interview and medical chart review of number of surgeries to date and parent ratings on the Adaptive Behavior Assessment System, Third Edition-Functional Academics Scale (ABAS-FA) and Child Behavior Checklist (CBCL) Total Competency Scale. RESULTS: Parent ratings of ABAS-FA were at or above normative expectations, while ratings across CBCL Competency Scales were lower than normative expectations. Socioeconomic status (SES), age, and race were consistent predictors of parent ratings (higher SES, older age, and Caucasian race were associated with better functioning). Number of surgeries did not add significantly to academic ratings but did significantly contribute to ratings of social and activity participation. Patients with more surgeries were rated with lower functioning in these domains. CONCLUSIONS: Findings do not support a connection between number of surgeries and later ratings of academic functioning but do support a connection to social and activity involvement. Recommendations for conducting direct studies of the connection between surgeries and academic functioning as well as clinical considerations for surgeries and impact on social and activity involvement are discussed.

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.

Development of an Outcome Measure of Observable Signs of Health and Well-Being in Infants With Orofacial Clefts.

OBJECTIVE: To develop an outcomes instrument that assesses observations that can be reliably reported by caregivers and can be used to assess health of infants with a cleft lip or cleft lip and cleft palate (CL±P) and impacts of treatments. DESIGN: Cross-sectional, mixed methods study. SETTING: Caregivers and health-care providers were recruited from 3 academic craniofacial centers and national advertisements. Most interviews were conducted by telephone, and surveys were completed online. PARTICIPANTS: Caregivers had a child less than 3 years of age with CL±P and spoke either English or Spanish. Health-care providers were members of a cleft team. Caregivers (n = 492) and health-care professionals (n = 75) participated in at least one component of this study. MAIN OUTCOME MEASURE(S): Caregivers and health-care providers participated in tasks related to instrument development: concept elicitation for items within relevant health domains, prioritization of items, and item review. RESULTS: We identified 295 observations of infant well-being across 9 health areas. Research staff and specialists evaluated items for clarity, specificity to CL±P, and responsiveness to treatment. Caregivers and health-care providers rated the resulting list of 104 observations and developed the final instrument of 65 items. CONCLUSIONS: In this phase of development of the Infant with Clefts Observation Outcomes (iCOO) instrument, items were developed to collect caregiver observations about indicators of children's health and well-being across multiple domains allowing for psychometric testing, sensitivity to changes associated with treatment, and documentation of the effects of treatment.

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.

Neurodevelopment of Infants with and without Craniofacial Microsomia.

OBJECTIVES: To determine whether infant cases with craniofacial microsomia (CFM) evidence poorer neurodevelopmental status than demographically similar infants without craniofacial diagnoses ("controls"), and to examine cases' neurodevelopmental outcomes by facial phenotype and hearing status. STUDY DESIGN: Multicenter, observational study of 108 cases and 84 controls aged 12-24 months. Participants were assessed by the Bayley Scales of Infant and Toddler Development-Third Edition and the Preschool Language Scales-Fifth Edition (PLS-5). Facial features were classified with the Phenotypic Assessment Tool for Craniofacial Microsomia. RESULTS: After adjustment for demographic variables, there was little difference in Bayley Scales of Infant and Toddler Development-Third Edition or Preschool Language Scales-Fifth Edition outcomes between cases and controls. Estimates of mean differences ranged from -0.23 to 1.79 corresponding to standardized effect sizes of -.02 to 0.12 (P values from .30 to .88). Outcomes were better among females and those with higher socioeconomic status. Among cases, facial phenotype and hearing status showed little to no association with outcomes. Analysis of individual test scores indicated that 21% of cases and 16% of controls were developmentally delayed (OR 0.68, 95% CI 0.29-1.61). CONCLUSIONS: Although learning problems have been observed in older children with CFM, we found no evidence of developmental or language delay among infants. Variation in outcomes across prior studies may reflect differences in ascertainment methods and CFM diagnostic criteria.

Speech Production Skills in Children With Cleft Palate Who Were Internationally Adopted.

OBJECTIVE: The objective of this study was to investigate the impact of international adoption (IA), age at palatoplasty (PR age), and velopharyngeal sufficiency (VPS) on articulation outcomes. DESIGN: This was a cross-sectional, prospective, observational study. SETTING: Outpatient hospital clinic. PARTICIPANTS: Fifty-one IA and 65 not-adopted (NA) children between the ages of 3 and 9 with nonsyndromic cleft palate with or without cleft lip. MAIN OUTCOME MEASURE(S): The Goldman-Fristoe Test of Articulation-2nd Edition (GFTA-2) standard score and cleft-related articulation errors (CREs). RESULTS: Articulation impairment was observed for 40% to 76% of NA children and 71% to 92% IA children, depending on age. PR age mean IA = 2.07 (0.86) years; NA = 1.23 (0.71) years. Children who were IA had poorer performance on the GFTA-2 ( B = -13.82, P = .015). Children who were IA were not significantly more likely to make CRE; rather, age at the time of assessment ( B = -.10, P = .002) and VPS ( B = .24, P = .021) were associated with CRE. CONCLUSIONS: Children who were IA demonstrated poorer articulation skills. Although primary palatoplasty was accomplished later among children who were IA, age at assessment and VP status (not PR age) were significantly correlated with articulation outcomes. Implications for timing of surgical intervention are discussed.

Predicting developmental delay in a longitudinal cohort of preschool children with single-suture craniosynostosis: is neurobehavioral assessment important?

AIM: The aim of this study was to determine whether neurobehavioral assessment before and after cranial vault surgery can improve prediction of developmental delay in children with single-suture craniosynostosis (SSC), after accounting for 'baseline' demographic and clinical variables (SSC diagnosis and surgery age). METHOD: Children with SSC were referred by the treating surgeon or pediatrician before surgery. Neurobehavioral assessments were performed at ages of approximately 6, 18, and 36 months. Iterative models were developed to predict delay, as determined by one or more tests of cognitive, motor, and language skills at 36 months. We selected from groups of variables entered in order of timing (before or after corrective surgery), and source of information (parent questionnaire or psychometric testing). RESULTS: Good predictive accuracy as determined by area under the receiver operating characteristic curve (AUC), was obtained with the baseline model (AUC=0.66), which incorporated age at surgery, sex, and socio-economic status. However, predictive accuracy was improved by including pre- and post-surgery neurobehavioral assessments. Models incorporating post-surgery neurobehavioral testing (AUC=0.79), pre-surgery testing (AUC=0.74), or both pre- and post-surgery testing (AUC=0.79) performed similarly. However, the specifity of all models was considered to be moderate (≤0.62). INTERPRETATION: Prediction of delay was enhanced by assessment of neurobehavioral status. Findings provide tentative support for guidelines of care that call for routine testing of children with SSC.

Sex differences in the neurodevelopment of school-age children with and without single-suture craniosynostosis.

PURPOSE: Previous studies have indicated that infants and school-age children with single-suture craniosynostosis (SSC, cases) score modestly but consistently lower than unaffected children (controls) on neurodevelopmental tests. However, sex differences in these functions rarely have been examined, and it is unknown whether potential sex differences vary by case status (cases vs. controls) or location of suture fusion. METHODS: We tested 182 cases and 183 demographically matched controls at a mean age of 7.4 years. We measured intellectual abilities with the Wechsler Scale of Intelligence for Children-Fourth Edition. We assessed reading, spelling, and math with a combination of the Wide Range Assessment Test-Fourth Edition, the Test of Word Reading Efficiency, and the Comprehensive Test of Phonological Processing. RESULTS: Among both cases and controls, males scored lower on all measures than females with standard score differences ranging from -1.2 to -7.8 for controls (p values from <0.001 to 0.55) and -2.3 to -8.5 for cases (p values from <0.001 to 0.33). For all but one measure, sex differences were slightly larger for cases than controls. Among cases, males were more likely than females to have learning problems (50 vs. 30%, respectively), with the highest level observed among males with unicoronal synostosis (86%). CONCLUSIONS: Sex differences in neurodevelopmental abilities among children with SSC are substantial, but not a unique correlate of this disorder as similar differences were observed among controls. Girls and those with sagittal synostosis have the lowest risk for academic problems. Boys with unicoronal synostosis warrant close developmental surveillance.

Shared Surgical Decision Making and Youth Resilience Correlates of Satisfaction With Clinical Outcomes.

The aim of this study was to identify factors associated with youth satisfaction with surgical procedures performed to address oral cleft or craniofacial conditions (CFCs). It was hypothesized that youth mental health, participation in decision making, perceived consequences of living with a CFC, and coping strategies would be associated with satisfaction with past surgeries. A total of 203 youth between the ages of 11 and 18 years (mean age = 14.5, standard deviation = 2.0, 61% male participants, 78% oral cleft) completed a series of questionnaires measuring depression, self-esteem, participation in decision making, condition severity, negative and positive consequences of having a CFC, coping, and satisfaction with past surgeries. Multiple regression analysis using boot-strapping techniques found that youth participation in decision making, youth perception of positive consequences of having a CFC, and coping accounted for 32% of the variance in satisfaction with past surgeries (P < 0.001). Youth age, sex, and assessment of condition severity were not significantly associated with satisfaction with surgical outcome. Depression, self-esteem, and negative consequences of having a CFC were not associated with satisfaction with past surgeries. Youth should be actively involved in the decision for craniofacial surgery. Youth who were more satisfied with their surgical outcomes also viewed themselves as having gained from the experience of living with a CFC. They felt that having a CFC made them stronger people and they believed that they were more accepting of others and more in touch with others' feelings because of what they had been through.

Longitudinal analysis of parenting stress in mothers and fathers of infants with and without single-suture craniosynostosis.

OBJECTIVE: To examine longitudinal differences in reported stress between parents of children with and without single-suture craniosynostosis and to compare the stress reports of mothers and fathers. DESIGN: Multi-site, nonrandomized prospective study. SETTING/PARTICIPANTS: Children with single-suture craniosynostosis (cases) were identified via referral of the treating surgeon or physician at the time of diagnosis, and those without single-suture craniosynostosis (controls) were recruited from pediatric practices, birthing centers, and announcements in print media. When children were aged 6, 18, and 36 months (on average), mothers and fathers of children with and without single-suture craniosynostosis completed the Parenting Stress Index. For cases, 247 mothers and 211 fathers completed the Parenting Stress Index at the first visit; corresponding numbers for controls were 254 and 220, respectively. MAIN OUTCOME MEASURES: The Parenting Stress Index Parent and Child Domains and subscales scores. RESULTS: We found few differences between parents of infants with and without single-suture craniosynostosis, regardless of parent gender. Irrespective of case status, mothers consistently reported higher stress than fathers on the Parent Domain. Within the Parent Domain, mothers reported more stress than fathers on the Role Restriction and Spousal Support subscales. CONCLUSIONS: The parents of children with single-suture craniosynostosis reported levels of stress similar to those reported by parents of same-aged, unaffected children. Mothers reported greater stress than fathers, and these differences remained remarkably stable over time. This may reflect widely held perceptions of gender differences in parenting roles.

The Association of Speech/Language Risk With Phonological Awareness, Rapid Naming, and Reading Ability in Children With Cleft Lip and/or Palate.

Children with cleft lip and/or palate were assessed for speech, language, phonological awareness (PA), rapid naming (RN) and reading ability using standardized instruments at baseline (T1; N = 142, Mage = 6.14 years, 51% males) and 2-year follow-up (T2; 89% retention, Mage = 8.38). Children with no speech or language risk scored higher for T1 and T2 PA, RN, and reading than children with both speech and language risk [Adjusted Mean Difference (AMD) ranged from 11.79 to 21.25]; language risk (AMD 8.37 to 13.58); and speech risk (0.51 to 6.87). No significant differences by cleft type or child sex.