The Cleft Palate Craniofacial Journal

This study examined whether the 2011 Tōhoku earthquake and tsunami affected the birth prevalence of cleft lip and/or cleft palate (CL/P) in Miyagi Prefecture, where the earthquake and tsunami caused severe damage.

This was a retrospective cohort study.

The study was conducted at university and children’s hospitals in Miyagi Prefecture.

The annual and monthly numbers of infants born with CL/P were obtained from medical records. The affected period of birth was defined as 1 year from December 1, 2011, to November 30, 2012. The control period was 5 years from January 1, 2006, to December 31, 2010. The annual and monthly numbers of births in Miyagi Prefecture were obtained using e-Stat, which is a portal for Japanese government statistics.

Main outcome measures were birth prevalence of CL/P during the control and affected periods.

There were no significant differences between the control and affected periods in the prevalence (per 10 000 live births) of cleft lip with or without palate (13.8 vs 16.7; P = .342), isolated cleft palate (5.2 vs 3.2; P = .267), or overall CL/P (19.0 vs 19.9; P = .799).

We did not observe that the 2011 Tōhoku earthquake and tsunami affected the birth prevalence of CL/P in Miyagi Prefecture, even though it severely impacted human health in the area.

The 22q11.2 deletion syndrome (22q11.2DS) is the most common genetic cause of velopharyngeal dysfunction; however, limited information exists regarding variations in velopharyngeal anatomy in this clinically challenging population. The purpose of this study was to examine velopharyngeal characteristics among young children with 22q11.2DS in comparison to a normative cohort using an innovative, nonsedated magnetic resonance imaging (MRI) scanning protocol.

Fifteen children with 22q11.2DS and 15 age- and gender-matched controls with normal velopharyngeal anatomy (ages 4-12) successfully completed the MRI protocol. Eighteen velopharyngeal and 2 related craniofacial measures were examined. Analysis of covariance was used to compare differences between the experimental and the control groups.

The 22q11.2DS group demonstrated a significantly thinner velum (P < .0005) and a larger pharyngeal depth (P = .007) compared to the matched control group. Findings in the current study also demonstrated that the levator veli palatini muscle is significantly shorter (P = .037) and thinner (P = .025) in the 22q11.2DS cohort, with a significantly shorter origin-to-origin distance (P < .0005) and a greater angle of origin (P = .001) compared to healthy peers.

Children with 22q11.2DS demonstrated multiple variations that may contribute to velopharyngeal dysfunction by altering the anatomic characteristics of the velopharyngeal port, the levator muscle, and associated structures. This investigation represents the first and largest attempt to characterize velopharyngeal anatomy in children with 22q11.2DS using a nonsedated MRI protocol.

In high-income countries, many cases of cleft lip are now identified at the 18- to 21-week pregnancy scan. The manner in which a diagnosis is communicated is vital for long-term parental well-being, yet previous studies have been indicative of parental dissatisfaction. The aims of the present study were to examine the experiences of parents who received an antenatal diagnosis of cleft lip in their unborn child and to offer pragmatic suggestions for improving the diagnostic experience in practice.

An online, mixed-methods survey was designed and distributed by the Cleft Lip and Palate Association. Data from 574 eligible parents were analyzed using descriptive statistics and inductive content analysis.

Although survey responses indicated positive diagnostic experiences overall, respondents perceived a lack of sensitivity among sonographers, long waiting times between referrals, and a lack of appropriate and reliable information. Respondents also reported a number of misconceptions about cleft lip and/or palate and its prognosis, as well as a variety of initial concerns about their own ability to cope with the anticipated challenges.

Findings emphasize the importance of providing accurate and individualized information to prospective parents, in a sensitive manner, so they can adjust to their child’s diagnosis and prepare for the birth appropriately. Given that antenatal screening for cleft lip is becoming more fully integrated into routine practice, more training for health-care professionals, improved access to reliable information in a variety of formats, and stronger links between local hospitals and specialist cleft services may be needed.

Assess the weight and contribution of each of the parameters of the Asher-McDade Scale to overall subjective assessment of nasolabial aesthetics following cleft lip repair.

Retrospective cohort evaluation.

Cleft and craniofacial center.

Forty-one patients who underwent unilateral cleft lip repair.

Unilateral cleft lip repair.

Nasolabial rating using the Asher-McDade scale and overall subjective assessment of nasolabial aesthetics using a rank score following unilateral cleft lip repair.

Strong interrater reliability was observed between the 3 raters. Significant association was determined on bivariate analysis between nasal form score (β = 27.06; P < .001), nasal symmetry score (β = 26.41; P < .001), nasal profile score (β = 28.75; P < .001), vermilion border score (β = 13.40; P = .012), and the ranking score. Adjusted β coefficients obtained from multivariate regression analysis were used to develop a modified nasolabial appearance score (over 5), that is, weighted for each of the 4 parameters: nasal form (over 8, adjusted β = 14.33), nasal symmetry (over 5, adjusted β = 7.96), nasal profile (over 5, adjusted β = 9.44), and vermilion (over 2, adjusted β = 3.31). Regression analysis between our modified nasolabial appearance score and patient ranking score demonstrated superior goodness of fit when compared to the Asher-McDade overall nasolabial appearance score (R² = .80; P < .001 vs R² = .69; P < .001).

The parameters evaluated in the Asher-McDade scale have different weights and contribute differently to overall subjective assessment of nasolabial aesthetic outcomes following cleft lip repair. Adjusting for their weights results in a modified score that demonstrates superior correlation with overall subjective assessment of nasolabial aesthetic outcomes.

Anthropometry is a well-established means of measuring facial morphology. Although reliable, direct anthropometry can be time-consuming and not conducive to a busy clinical practice. The Vectra H1 handheld stereophotogrammetric system requires 3 stereophotographs taken from different perspectives to generate a three-dimensional (3D) surface. The time needed to take the 3 stereophotographs can increase the possibility of involuntary movements, precluding its use to assess young patients. To overcome this limitation, we evaluated if accurate linear facial measurements can be obtained from a single stereophotograph and compare these to the measurements taken by direct anthropometry.

Twenty pediatric patients, aged 0 to 10 years, who were undergoing minor surgical procedures at Johns Hopkins All Children’s Hospital were recruited. Fourteen linear facial distances were obtained from each participant using direct anthropometry under general anesthesia. These same distances were measured using Mirror 3D analysis. Intraclass correlation was used to determine intrarater reliability on duplicate 3D images.

Correlation coefficients between 3D imaging in frontal view and direct anthropometric measurements were excellent for 13 measures taken, ranging from 0.8 (subnasale to columella and subnasale to stomion superius) to 0.98 (nasion to subnasale and subnasale to labiale superius). Correlation coefficients between submental view and direct anthropometric measurements were excellent for 13 measures as well, ranging from 0.77 (subnasale to columella) to 0.98 (nasion to subnasale).

Linear anthropometric measurements taken from 3D surfaces generated from a single stereophotograph correlate closely with direct anthropometric measures. This improves workflow and applicability of anthropometric studies to our youngest patients.

Long-term outcomes of patients born with a cleft lip and palate (CLP) are scarcely investigated. Yet, this patient group is of particular interest, as they can provide a valuable retrospective view upon their treatment experiences and psychological adjustment. Qualitative accounts may be especially useful in understanding the patient journey.

The present study set out to evaluate quality of life and satisfaction with treatment in adult patients previously treated for CLP at the Wilhelmina Children’s Hospital. Semistructured interviews were performed.

A total of 22 patients aged 17 to 35 years (mean: 25 years) were interviewed about their experiences of growing up with CLP and of the treatment they received. Interviews were audio-recorded and factors thought to influence psychological adjustment were identified.

Four main themes were identified: background factors, support systems, treatment factors, and coping/internal factors. Each theme was described with illustrative quotes.

This study underlines that psychological adjustment can fluctuate over time and greatly differs between individuals, even during adulthood. Psychological support should therefore be available beyond the finalization of the treatment course. Furthermore, the majority of participants stated they had wanted more psychological support as a child to facilitate resilience and to help them cope with challenges.

The birth of a child with a cleft lip and/or palate (CL/P) can have a significant emotional and social impact on parents. Yet, the impact on the wider family is rarely investigated. Grandparents are becoming increasingly involved in the care of their grandchildren and may therefore have support needs of their own. The aim of the current study was to explore the experiences and support needs of grandparents of children born with CL/P.

Individual semistructured telephone interviews were carried out with 12 grandparents of children born with CL/P and were analyzed using inductive thematic analysis.

Five themes were identified (1) emotional impact of CL/P on grandparents, (2) grandparents’ social experiences, (3) grandparents’ involvement, (4) grandparents’ information needs, and (5) grandparents’ support needs. Participants experienced difficult emotions around the time of diagnosis and were concerned about the child’s treatment and future experiences. Participants played a significant role in supporting the whole family, but received little information or support themselves.

Grandparents reported experiences comparable to published literature on parents. Clinicians and charitable organizations could consider how existing resources could be made more accessible to and/or adapted for wider family members, including grandparents.

The present study investigated how adults with congenital craniofacial anomalies (CFAs) and parents experience the long-term and complex treatment offered by a multidisciplinary team (MDT).

Exploratory-descriptive qualitative study based on individual semistructured interviews.

Centralized national follow-up and treatment of CFAs by a multidisciplinary craniofacial team from which participants were systematically recruited.

The sample included 48 parents of children with CFAs and 16 adults with CFAs (N = 64).

In general, participants reported to be satisfied with the follow-up and treatment they received from the MDT. Still, some aspects of treatment were experienced as demanding such as the large number of health professionals present during the consultation and being the object of their scrutinizing attention. Health professionals’ communication skills were described as central for participants’ involvement in, and satisfaction with, treatment. Participants also expressed a need for more treatment-related information regarding future treatment.

Findings could have implications for the organization of care for parents and patients with rare CFAs. The many advantages of MDTs also create unique challenges for patients and parents that need to be addressed. Patients and families should be prepared for the first consultation with the MDT. Health professionals should be aware of their communication style when interacting with patients and be aware of individual differences and needs regarding treatment-related experiences and expectations.

To test the Brazilian Portuguese velopharyngeal insufficiency (VPI) Effects on Life Outcome (VELO) instrument for reliability and validity.

Cross-sectional methodological study.

Tertiary craniofacial medical center.

Participants with VPI (VPI group, n = 60), with cleft and without VPI (no VPI/cleft group, n = 60), and with no cleft nor VPI (no VPI/no cleft group, n = 60) and their parents (n = 180).

All patients with VPI 8+ years old and their parents completed the Brazilian–Portuguese VELO instrument and other questionnaires (Pediatric Quality of Life Inventory4.0, PedsQL4.0; Pediatric Voice-Related Quality of Life, PVRQOL; and Intelligibility in Context Scale, ICS) at baseline; patients with VPI and their parents completed the VELO instrument again 2 weeks later.

The VELO instrument was tested for internal consistency, test–retest reliability, discriminant validity (participants with VPI against participants with no VPI), concurrent validity against other questionnaires, criterion validity against hypernasality severity, and construct validity against nasal air emission and overall velopharyngeal competence (speech construct) and velopharyngeal gap (anatomic construct).

The VELO had excellent internal consistency (Cronbach α 0.99 for parents and 0.98 for participants with VPI) and test–retest reliability (all intraclass correlation coefficient > 0.87). The VELO discriminated well between VPI group and unaffected groups (all P < .05). The VELO was significantly correlated with the PedsQL^4.0, PVRQOL, and ICS (–r > 0.75; P < .001). The VELO met criterion validity, speech construct validity, and anatomic construct validity (r > 0.7; P < .001).

The Brazilian-Portuguese VELO instrument demonstrated reliability (internal consistency and test–retest) and validity (discriminant, concurrent, criterion, and construct).

Cleft lip and palate are among the common birth defects in the United States. Management is complex and may be difficult for parents and patients to understand. The goal of this study was to evaluate the readability of online educational material provided by the American Society of Plastic Surgeons (ASPS) and the American Cleft Palate-Craniofacial Association (ACPA) websites regarding cleft lip and palate and to assess parents’ comprehension.

Parents of children younger than 10 years of age presenting to the plastic and reconstructive surgery clinic at Johns Hopkins All Children’s Hospital for conditions other than cleft lip and palate were recruited for participation. Parents were given the literature published on the ASPS and ACPA web sites to review and were asked to complete a 5-question test specifically targeted to the content of the passage they reviewed. We tested the readability of the 2 online passages using Readability Studio software (Oleander Software Solutions Ltd).

Sixty parents completed the study. Across all groups, the mean score was 4.35 of a possible 5. The mean score was 4.4 for the ASPS web site and 4.29 for the ACPA web site. Fifty-three percent of participants answered all questions correctly. No significant difference in scores was noted between web sites (P = .48).

Despite containing content of varying reading-level difficulty, the literature posted on the ACPA and ASPS web sites concerning cleft lip and palate conveyed information equally well to the target parent demographic with high levels of comprehension.

Informed decision-making relies on available information, including online resources. We evaluated the content and readability of websites published by American Cleft Palate-Craniofacial Association (ACPA)-approved cleft lip and/or palate (CLP) teams in the United States.

Team websites were reviewed, and teams with no accessible website or <30 sentences of content were excluded. Website content was scored by presence/absence of 20 variables derived from ACPA approval standards. Readability was evaluated with 8 scales. Readability was then compared to American Medical Association (AMA) recommendations. The relationship between website content and readability was assessed.

Content and readability of team websites.

From 167 reviewed teams, 47 (28.1%) had nonfunctional links, 17 (10.2%) had no accessible website, and 39 (23.4%) had <30 sentences. The average content score for all 111 team websites included was 14.5 (2.6) of 20. The combined average reading level across all scales (10.7 [1.9]) exceeded the AMA-recommended sixth-grade reading level; this finding held true for each individual website. Children’s Hospital-affiliated teams (n = 86) had a significantly higher content score (14.8 vs 13.5; P = .03) and better readability as evidenced by lower reading grade level (10.5 vs 11.4; P = .04). On linear regression, a higher content score significantly predicted better readability (β = −0.226; P < .001).

Websites published by ACPA-approved CLP teams vary in accessibility and content and exceed the recommended reading level. These findings could inform future efforts to improve patient-oriented resources.

Limited research has studied the involvement of children in medical decision-making. The aim of the study was to understand the involvement of adolescents with cleft lip and/or palate (CL/P) in decisions about elective surgeries and treatments.

Parents and professionals completed mixed-methods questionnaires about the degree to which children had been involved in choices about elective treatments. Data were analyzed using content analysis. Young people aged 12 to 25 years were asked to take part in semistructured interviews. The data were analyzed using inductive thematic analysis.

Questionnaire data collection took place online, and interview data were collected via messenger or telephone-based interviews.

The study employed 30 participants; 11 young people (3 male, 8 female), 17 parents (13 mothers, 4 fathers), and 5 professionals (2 surgeons, 2 speech and language therapists, and 1 pediatric dentist).

Five main themes were identified. These reflected participants feeling that with increasing age should come increased involvement in decision-making and that it was important for adolescents to “have a voice” during decision-making. Parents, peers, and health professionals were identified as influencing decisions. Most adolescents reported overall satisfaction with their involvement in decision-making but sometimes felt “left in the dark” by professionals or under pressure from parents. A desire to improve speech and/or appearance was as an area where adolescents wanted to be more involved in decision-making.

Shared decision-making is an important factor for psychological well-being by promoting autonomy and self-esteem among adolescents with CL/P.

Childhood is a period of extensive socioemotional development, which can be impacted by the presence of a congenital craniofacial anomaly (CFA). Complex multidisciplinary treatment and long-term follow-up are normally required, yet understanding of children’s treatment experiences is limited. The objective of this study was to investigate children’s experiences of multidisciplinary team (MDT) consultations from the perspective of their parents.

Thirty-eight parents of children with a rare CFA were interviewed in person or over the telephone. Interviews were transcribed verbatim, translated into English, and explored using thematic analysis.

Background factors influencing the child’s experience of the consultation included age, developmental stage, personality, and prior treatment experiences. Participants tried to prepare their child for meeting the MDT, but did not fully understand what to expect themselves. During consultations, participants were acutely focused on their child’s emotional state, making it difficult to balance their desire to protect the child from potentially negative experiences, and the need to engage in a constructive dialogue with health professionals. Participants believed that health professionals’ conduct could considerably influence the child’s well-being and subsequent treatment decisions. Finally, participants highlighted the need to debrief their child to help them adjust positively.

The ultimate goal of craniofacial care is to help children develop into confident adults who are able to cope with the challenges associated with their condition. Multidisciplinary teams play a vital role in creating a safe and supportive environment in which children feel genuinely informed and involved in key aspects of their care.