Maternal Stressful Life Events During the Periconceptional Period and Orofacial Clefts: A Systematic Review and Meta-Analysis

Objective To assess whether women who experience stressful life events during the periconceptional period are at higher risk of giving birth to a baby with an orofacial cleft (OFC). Design Systematic review and meta-analysis of studies reporting the proportion of babies born with OFC to mothers exposed and unexposed to population-level or personal-level stressful life events during the periconceptional period. Six electronic databases were searched from inception to August 2020. Risk of bias was assessed using the Newcastle-Ottawa scale. Odds ratios (ORs) for the odds of OFC in babies of exposed mothers relative to unexposed controls were extracted and/or calculated. Random effects meta-analysis was undertaken, stratified by cleft subtype. Results Of 12 eligible studies, 8 examined experience of personal events and 4 examined population-level events. Studies demonstrated low-moderate risk of bias and there was indication of publication bias. There was some evidence that personal stressful life events were associated with greater odds of cleft lip and/or palate (six studies, OR 1.63, 95% confidence interval (CI) 1.16, 2.30, P = 0.001) and cleft palate only (six studies, OR 1.45, 95% CI 1.02, 2.06, P = 0.04). Population-level events were associated with higher odds of OFC in studies that did not specify subtype (three studies, OR 1.64, 95% CI 1.19, 2.25, P = 0.002), but subtype stratified analyses were underpowered. Heterogeneity was high. Conclusions Limited evidence indicated a weak positive association between maternal stressful life events during the periconceptional period and risk of OFC in the offspring, but further studies with greater consistency in research design are needed.

Facial Nerve Dysfunction After Mandibular Distraction Osteogenesis in Patients with Robin Sequence: A Systematic Review and Meta-Analysis

Objective Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical intervention is mandibular distraction osteogenesis (MDO). This study analyzes published evidence regarding facial nerve dysfunction (FND) associated with MDO. Design and Setting According to PRISMA guidelines, a systematic review was carried out with databases queried in June 2019 using MESH terms, or equivalent terms, as follows: “distraction osteogenesis” and “Robin Sequence”. A review of original Spanish and English articles, were included. Outcome measures included the prevalence of FND; the affected branches; the rate of permanent vs. transient FND; the use of an internal vs. external device; the daily distraction rate; and finally, the overall distraction length. Subsequently, a meta-analysis was conducted to collate results regarding the prevalence of FND and the factors associated with it. Results Of 239 unique studies identified, 19 studies with 729 patients met inclusion criteria; 52 patients developed FND after MDO. A random-effects meta-analysis yielded a pooled prevalence of FND of 6.40%, with moderately heterogeneous studies (I2 = 41%, τ2 = 0.006). Marginal mandibular nerve involvement was most commonly noted. Nine studies reported transient FND, six permanent, one both, and two unspecified. Internal distractors were used in 8 studies and external in 3 and both in 2. Distraction rate was 1.00 to 2.00 mm/day and total distraction length ranged from 13.00 to 22.3 mm. Sample size was the only parameter inversely associated with rate of FND (p = 0.04). Conclusion This analysis of FND associated with MDO for patients with RS demonstrates a lack of consistent documentation. MDO-associated FND does not appear to be uncommon, and permanent dysfunction can occur. This review underscores the importance of thorough documentation to elucidate the mechanism of FND.

Adapting Elements of Cleft Care Protocols in Low- and Middle-income Countries During and After COVID-19: A Process-driven Review With Recommendations

A consortium of global cleft professionals, predominantly from low- and middle-income countries, identified adaptations to cleft care protocols during and after COVID-19 as a priority learning area of need. A multidisciplinary international working group met on a videoconferencing platform in a multi-staged process to make consensus recommendations for adaptations to cleft protocols within resource-constrained settings. Feedback was sought from a roundtable discussion forum and global organizations involved in comprehensive cleft care. Foundational principles were agreed to enable recommendations to be globally relevant and two areas of focus within the specified topic were identified. First the safety aspects of cleft surgery protocols were scrutinized and COVID-19 adaptations, specifically in the pre- and perioperative periods, were highlighted. Second, surgical procedures and cleft care services were prioritized according to their relationship to functional outcomes and time-sensitivity. The surgical procedures assigned the highest priority were emergent interventions for breathing and nutritional requirements and primary palatoplasty. The cleft care services assigned the highest priority were new-born assessments, pediatric support for children with syndromes, management of acute dental or auditory infections and speech pathology intervention. A collaborative, interdisciplinary and international working group delivered consensus recommendations to assist with the provision of cleft care in low- and middle-income countries. At a time of global cleft care delays due to COVID-19, a united approach amongst global cleft care providers will be advantageous to advocate for children born with cleft lip and palate in resource-constrained settings.

Risk of Malocclusion Among Patients Undergoing Single-Stage Versus Two-Stage Cleft Palate Repair

Objective Evaluate impact of single-stage versus staged palate repair on the risk of developing malocclusion among patients with cleft palate (CP). Design Retrospective cohort study 2000–2016 Setting Academic, tertiary children’s hospital. Patients Patients undergoing CP repair between 1999–2015. Interventions CP repair, categorized as either single-stage or staged. Main Outcome Measure Time to development of Class III malocclusion. Results 967 patients were included; 60.1% had a two-stage CP repair, and 39.9% had single-stage. Malocclusion was diagnosed in 28.2% of patients. In the model examining all patients at ≤5 years ( n = 659), patients who were not white had a higher risk of malocclusion (HR 2.46, p = 0.004) and staged repair was not protective against malocclusion (HR 0.98, p = 0.91). In all patients >5 years ( n = 411), higher Veau classification and more recent year of birth were significantly associated with higher hazard rates ( p < 0.05). Two-staged repair was not protective against developing malocclusion (HR 0.86, p = 0.60). In the model examining patients with staged repair ≤5 years old ( n = 414), higher age at hard palate closure was associated with reduced malocclusion risk (HR 0.67, p < 0.001) and patients who were not white had increased risk (HR 2.56, p = 0.01). In patients with staged repair >5 years old, more recent birth year may be associated with a higher risk of malocclusion (HR 1.06, p = 0.06) while syndrome may be associated with lower risk of malocclusion diagnosis (HR 0.46, p = 0.07). Conclusion Our data suggests that staged CP repair is not protective against developing Class III malocclusion.

The Cleft Lip Education with Augmented Reality (CLEAR) VR Phase 2 Trial: A Pilot Randomized Crossover Trial of a Novel Patient Information Leaflet

Background The Cleft Lip Education with Augmented Reality (CLEAR) project centers around the use of augmented reality (AR) in patient leaflets, as a visual means to overcome the “health literacy” gap. This trial followed Virtual Reality (VR CORE) guidelines for VR Phase 2 (Pilot) trials. Methods Participants included families of children treated for Cleft Lip and Palate at the Royal Hospital for Children, Glasgow. Interventions were AR leaflet or Traditional Leaflet. Objectives were to calculate sample sizes, assess outcome instruments, trial design, and acceptability to patients. Primary outcome measure was Mental Effort Rating Scale, and secondary outcomes were Patient Satisfaction (Visual Analogue Scale), Usefulness Scale for Patient Information Material (USE) scale, and Instructional Materials Motivation Survey (IMMS). Randomization was by block randomization. The trial was single blinded with assessors blinded to group assignment. Results 12 Participants were randomized, with crossover design permitting analysis of 12 per group. Primary outcome with Mental Effort Rating Scale indicated higher mental effort with Traditional compared to AR Leaflet (4.75 vs 2.00, P = .0003). Secondary outcomes for Satisfaction were Traditional 54.50 versus AR 93.50 ( P = .0001); USE scale 49.42 versus 74.08 ( P = .0011); and IMMS 112.50 versus 161.75 ( P = .0003). Subjective interviews noted overwhelmingly positive patient comments regarding the AR leaflet. Outcome instruments and trial design were acceptable to participants. No harms were recorded. Conclusions The CLEAR pilot trial provides early evidence of clinical efficacy of AR leaflets in patient education. It is hoped that this will provide a future paradigm shift in the way patient education is delivered.

Repair of Protruding Bilateral Cleft Lip and Palate With Staged Premaxilla Setback Osteotomy, Cheiloplasty, and Palatoplasty in Trisomy 17p Patient: A Review of Syndromic Clinical Characteristic

Chromosome 17 duplication is correlated with an increased risk of developmental delay, birth defects, and intellectual disability. Here, we reported a female patient with trisomy 17 on the whole short arm with bilateral complete cleft lip and palate (BCLP). This study will review the surgical strategies to reconstruct the protruding premaxillary segment, cleft lip, and palate in trisomy 17p patient. The patient had heterozygous pathogenic duplication of chromosomal region chr17:526-18777088 on almost the entire short arm of chromosome 17. Beside the commonly found features of trisomy 17p, the patient also presented with BCLP with a prominent premaxillary portion. Premaxillary setback surgery was first performed concomitantly with cheiloplasty. The ostectomy was performed posterior to the vomero-premaxillary suture (VPS). The premaxilla was firmly adhered to the lateral segment and the viability of philtral flap was not compromised. Two-flap palatoplasty with modified intravelar veloplasty (IVV) was performed 4 months after. Successful positioning of the premaxilla segment, satisfactory lip aesthetics, and vital palatal flap was obtained from premaxillary setback, primary cheiloplasty, and subsequent palatoplasty in our trisomy 17p patient presenting with BLCP. Postoperative premaxillary stability and patency of the philtral and palatal flap were achieved. Longer follow-up is needed to evaluate the long-term effects of our surgical techniques on inhibition of midfacial growth. However, the benefits that the patient received from the surgery in improving feeding capacity and facial appearance early in life outweigh the cost of possible maxillary retrusion.

Disparities in Access to Cranial Remodeling Orthosis for Deformational Plagiocephaly

Objective Moderate to severe cases of deformational plagiocephaly (DP) may be treated with cranial remolding orthoses (CRO). This study investigated the socioeconomic disparities in access to care for CRO for DP correction. Design This was a retrospective review of medical records from a single CRO company in Connecticut from 2014 to 2020. Methods Demographic variables were collected from all patients. Univariable logistic regressions were used to identify differences for presenting age at consultation, whether CRO was pursued, and length of CRO treatment by insurance payor and household income quartile. Results Of the 5620 patients identified, 4100 (73.0%) received CRO, with 674 (12.0%) receiving a second helmet. Of those receiving CRO, 1536 (37.5%) had Medicaid insurance while 2558 (62.4%) were commercially insured. Patients on Medicaid were 1.30 times more likely to have delayed presentation ( P = .017), while patients from the lowest income quartile were 1.26 1.50 ( P < .001) and 1.58 ( P < .001) times more likely to have a delayed presentation relative to those in the highest and second-highest income quartiles, respectively. Patients in the highest and second-highest income quartiles were also 1.55 ( P < .001) and 1.45 ( P < .001) more likely, respectively, to receive CRO after consultation than those from the lowest income quartile. Conclusions Lower income and Medicaid-insured patients had delayed presentation for CRO consultation. Those from the lowest income quartile were more likely to never receive CRO than those from wealthier backgrounds. Low socioeconomic status and Medicaid insurance, which can have more restrictive coverage policies for CRO, may result in the delayed treatment of DP.

Psychological Adjustment in Apert Syndrome: Parent and Young Person Perspectives

To date, limited research has been carried out into the psychological impact of having a diagnosis of Apert syndrome (AS) and the life experiences of families living with this condition. The aim of the current study was to explore psychological adjustment to AS from the perspectives of young people, and their parents, with the broader goal of informing care, and support for this population. Four young people (2 male) aged 11 to 15 years and their mothers were interviewed in their homes using a semistructured interview guide and photo-elicitation methods. Transcripts were analyzed using Interpretive Phenomenological Analysis. Three superordinate themes were identified from the data: (1) Acceptance and Adjustment: A Cyclical Journey; (2) A Barrier to Adjustment: Navigating Treatment; and (3) Facilitating Adjustment: Social Support. Families described adjustment as a cyclical process, which was sensitive to change, particularly in the context of ongoing medical treatment. Families also utilized many resources, particularly in the form of social support, to adjust to the challenges of AS and build resilience. The findings of this study have important implications for the implementation of patient-centered care within designated craniofacial treatment centers, which should at a minimum include the provision of reliable information throughout the treatment pathway, additional support from health professionals at key times of transition, and the coordination of support across medical teams, and other key organizations in the child's life.

Racial and Ethnic Disparities in Primary Cleft Lip and Cleft Palate Repair

Objective To examine the impact of race/ethnicity on timing and postoperative outcomes of primary cleft lip (CL) and cleft palate (CP) repair. Design Cross-sectional analysis of the National Surgical Quality Improvement Program Pediatric (NSQIP-P) database from 2013 to 2018. Patients and main outcome measures Patients under 2 years of age who underwent primary CL or CP repair were identified in the NSQIP-P. Outcomes were the timing of surgery and 30-day readmission and reoperation rates stratified by race and ethnicity. Results In total, 6021 children underwent CL and 6938 underwent CP repair. Adjusted rates of CL repair over time were 10% lower in Hispanic children (95%CI: 0.84–0.96) and 38% lower for Asian children (95%CI: 0.55–0.70) compared with White infants. CP repair rates over time were 13% lower in Black (95%CI: 0.79–0.95), 17% lower in Hispanic (95%CI: 0.77–0.89), and 53% lower in Asian children (95%CI: 0.43–0.53) than in White infants. Asian patients had the highest rates of delayed surgical repair, with 19.3% not meeting American Cleft Palate-Craniofacial Association (ACPA) guidelines for CL ( P < .001) and 28.2% for CP repair ( P< .001). Black and Hispanic children had 80% higher odds of readmission following primary CL repair (95%CI: 1.16–2.83 and 95%CI: 1.27–2.61, respectively). Conclusions This study of a national database identified several racial/ethnic disparities in primary CL and CP, with reduced receipt of cleft repair over time for non-White children. Asian patients were significantly more likely to have delayed cleft repair per ACPA guidelines. These findings underscore the need to better understand disparities in cleft repair timing and postoperative outcomes.

Cleft Size and Success of Secondary Alveolar Bone Grafting—A Systematic Review

Objective This study aimed to review all research evidence of presurgical cleft size and related factors to success of secondary alveolar bone grafting (SABG). Design and Setting The systematic review searched the OVID-Medline®, PubMed®, Embase®, and Cochrane Central Register of Controlled Trials (CENTRAL) up to August 2020. Two reviewers independently selected potential abstracts for full review. Disagreeements were resolved by consensus. The first author extracted data and assessed the risk of bias using Risk of Bias in Non-randomized studies—of Interventions tool. Patients and Interventions Patients with non-syndromic clefts who received SABG were selected. Presurgical cleft size/volume and treatment results must be available. Main Outcome Measures Level of the grafted bone, achievement of orthodontic tooth movement into the grafted area, need for re-operation. Results From 962 abstracts, 23 publications were included. Mean cleft width was 6.80 ± 1.98 mm, cleft area 20–240 mm2, and mean volume 0.89 ± 0.33 cm3. No definite conclusion was achieved on whether a narrow or wide cleft showed better treatment outcomes, but other potentially related factors were good oral hygiene and eruptive force of the maxillary canines. Lack of a standard definition of cleft size, a small sample size, varying outcome parameters, and moderate-to-high risk of bias contributed to the summary. A meta-analysis could not be performed because of the heterogeneity. Conclusion Due to insufficient evidence, cleft width/volume could not be specified leading to more successful SABG. Care of patients could be improved in both research by following rigorous methodology, and practice by clear communication.