The Cleft Palate Craniofacial Journal

To compare in-depth speech results in Scandcleft Trial 1 as well as reference data from peers without cleft palate (CP).

A prospective randomized clinical trial.

A Danish and a Swedish CP center.

143 of 148 randomized 5-year-olds with unilateral cleft lip and palate. All received lip and velum closure at 4 months, and hard palate closure at 12 months (arm A) or 36 months (arm B).

A composite measure based on velopharyngeal competence (VPC) or velopharyngeal incompetence (VPI), an overall assessment of VPC from connected speech (VPC-Rate), Percentage of Consonants Correct (PCC-score), and consonant errors. Speech therapy visits, average hearing thresholds, and secondary pharyngeal surgeries documented burden of treatment.

Across the trial, 61.5% demonstrated VPC and 38.5% VPI. Twenty-two percent of participants achieved age appropriate PCC-scores. There were no statistically significant differences between arms or centers for these measures. In the Danish center, arm B: achieved lower PCC-scores (P = .01); obtained PCC-scores without s-errors below 79% (P = .002); produced ≥3 active oral cleft speech characteristics (P = .004) than arm A. In both centers, arm B attended more speech visits.

At age 5, differences between centers and treatment arms were not statistically significant for VPC/VPI, but consonant proficiency differed between treatment arms in the Danish center. Poor speech outcomes were seen for both treatment arms. Variations between centers were observed. As the Swedish center had few participants, intercenter comparisons should be interpreted with caution.

To evaluate attrition rates prior to expected completion of team care for children with complete cleft lip and palate (cleft) or nonsyndromic single-suture craniosynostosis (synostosis).

A single-institution retrospective review of attendance data from 2002 to 2016.

Single cleft and craniofacial center in the United States.

A sample of 983 patients with either cleft or synostosis. Patients who were more than 2 to 3 years from their last visit were considered lost to follow-up. Patients with cleft older than 16 years or synostosis over 11 years were considered graduated from team care.

Survival analysis shows that in our patients with cleft, 25% leave before age 8 and over 60% are lost from team by age 16. In patients with synostosis, 25% leave before age 6 and 45% are lost by age 11. Cox regression showed underrepresented minorities being 1.7 times more likely to become lost in the cleft group (hazard ratio: 1.66, 95% confidence interval [CI]: 1.01-2.74).

Overall, attrition rates were high at our institution. Many patients are lost to follow-up prior to receiving key medical interventions. Improved family education and personalized care are needed to help ensure continuity of care.

This study was to investigate ipsilateral hypertrophy of the mastoid process in the patients with congenital muscular torticollis (CMT).

Retrospective cross-sectional study.

Children with CMT.

A total of 212 surgical cases of patients with CMT (age: 50.9 ± 44.3 months) and 212 age- and gender-matched controls (age: 50.4 ± 44.2 months) were included. The mastoid process volume was calculated and compared for both groups on the computed tomography axial images. A linear regression analysis was performed between the age and the intrasubject volume difference in the mastoid process.

The volume of the mastoid process in the CMT side was significantly larger than that of the non-CMT side in the CMT group (32.2 ± 30.3 cm³ vs 21.9 ± 22.8 cm³; P < .001). In the control group, there was no significant difference between the volume of the right and left mastoid process (21.6 ± 24.6 cm³ vs 21.2 ± 23.8 cm³; P = .472). The intrasubject volume difference in the mastoid process in the CMT group showed a linear increase with the age (adjusted R ² = 0.286; P < .001), and the volumetric asymmetry of the mastoid process became more severe over age compared to the control group.

We showed ipsilateral hypertrophy of the mastoid process in patients with CMT and demonstrated that the volumetric asymmetry increased with age.

The aims of the study were to assess the postoperative oronasal fistula rate after 1-stage and 2-stage cleft palate repair and identify risk factors associated with its development.

Systematic review.

Various primary cleft and craniofacial centers in the world.

Syndromic and nonsyndromic cleft lip, alveolus, and palate patients who had undergone primary cleft palate surgery.

Assessment of oronasal fistula frequency and correlation with staging, timing, and technique of repair, gender, and Veau type. The results obtained in this systematic review were compared with those in previous reports.

The main outcome is represented by the occurrence of the oronasal fistula after 1-stage versus 2-stage palatoplasty.

The mean fistula percentage was 9.94%. In the Veau I, II, III, and IV groups, the respective fistula rates were 2%, 7.3%, 8.3%, and 12.5%. Oronasal fistula locations based on the Pittsburgh Fistula Classification System were soft palate (type II), 16.2%; soft palate–hard palate junction (type III), 29.3%; and hard palate (type IV), 37.3%. There were no statistically significant differences between 1-stage and 2-stage palatoplasty, syndromic and nonsyndromic, or male and female patients. Primary palatoplasty timing was not a significant predictor.

Some disparities arose when comparing studies, mainly regarding location and types of clefting prone to oronasal fistulation. Interestingly, the fistula rate does not differ between 1- and 2-stage closure, and timing of the repair does not play a role.

To investigate whether morphofunctional velopharyngeal aspects may be considered predictors of appearance or worsening of hypernasality in patients with cleft palate after surgical maxillary advancement (MA).

Prospective.

National referral center for cleft lip and palate rehabilitation.

Fifty-two patients with repaired cleft palate, skeletal class III malocclusion, and normal speech resonance completed speech audio recordings and cone-beam computed tomography examination before (T1) and, on average, 14 months after (T2) MA.

Hypernasality was rated by 3 experienced speech-language pathologists using a 4-point scale and morphofunctional aspects on a 3-point scale. Cone-beam computed tomography image measurements were performed using Amira and Dolphin 3D software. For each velopharyngeal morphofunctional aspect analyzed, patients were compared according to the absence (G1) and presence (G2) of postoperative hypernasality.

Comparison of hypernasality scores between T1 and T2 and association between hypernasality and each velopharyngeal morphofunctional aspect.

Significant difference was observed between T1 and T2 for hypernasality ( P = .031) and between G1 and G2 ( P = .015) for velar mobility, with significant association between this variable and hypernasality on T2 (P = .041).

Levator veli palatini mobility influenced the appearance of hypernasality after MA.

(1) To explore orthodontists’ perceptions of nonadherence and related factors in their patients with craniofacial conditions; (2) to examine differences in adherence perceptions by provider characteristics; (3) to evaluate current adherence interventions.

Cross-sectional.

United States-based orthodontists affiliated with a nonprofit association for providers treating oral cleft and craniofacial conditions received survey invitations via list-serv, e-mail, and social media.

Thirty-eight orthodontists participated (mean age = 50.5 ± 10.7 years; 76% Caucasian; 55% male; 54% private practice).

An 80-item survey assessed demographic characteristics; frequencies of various adherence problems and adherence-related prolonged or terminated treatment; importance of adherence to treatment outcomes; factors that may impact adherence; interest in improving adherence; and use of adherence-enhancing interventions.

Adherence problems were common, yet 80.6% of participants rated adherence as “very important” to cleft treatment outcomes. Child behavior and motivation, caregiver reinforcement of behavior, and provider communication with the family were identified as factors that greatly impact adherence. Orthodontists in university-affiliated programs were more likely to rate individual, family, health-care system, and community factors as impacting adherence compared to private practice orthodontists; 80.6% used adherence interventions; these strategies were all rated as “sometimes successful.”

Nonadherence to cleft lip/palate-related orthodontic care is common and potentially detrimental to care. Current interventions are not uniformly successful and could be better tailored. Understanding provider, patient, and family factors that affect adherence can inform individualized treatment planning to improve adherence and ultimately, treatment outcomes.

Measure lower lip thickness and eversion in patients with cleft lip and palate (CLP) and maxillary hypoplasia. The specific aims were to (1) compare lower lip thickness/eversion in patients with CLP to noncleft controls with maxillary hypoplasia, (2) determine differences between patients with unilateral CLP (UCLP) and bilateral CLP (BCLP), and (3) document changes in the lower lip that occur with Le Fort I advancement.

Retrospective case–control study.

Tertiary care center.

Patients with available pre- and postoperative CT scans and 2D lateral photographs who had a Le Fort I advancement between 2009 and 2017. There were 32 patients with CLP (17 females; mean age 17.7 ± 1.9 years) and 33 noncleft controls (21 females; mean age 18.8 ± 2.6 years).

Lower lip thickness and eversion.

Patients with CLP and maxillary hypoplasia have a significantly thicker lower lip (P = .019) and outward rotation of the vermilion border (P = .003) compared to noncleft controls. The lower lip was significantly thicker in patients with BCLP than in those with UCLP (P = .035). Lower lip thickness and rotation did not change after maxillary advancement.

Patients with CLP and maxillary hypoplasia have a thicker and more everted lower lip than noncleft controls. Patients with BCLP have a significantly thicker lower lip than those with UCLP. Strain of the lower lip musculature appears to be an important contributor to the development of the cleft lip lower lip deformity.

The goal of cleft therapy has progressed from simply correcting the deformity to uplifting the patient’s quality of life (QoL). At the end of comprehensive treatment, a patient with cleft lip and palate (CLP) should report with satisfactory QoL scores in all domains such as aesthetics, speech, function, and psychology.

To develop and validate a novel, disease-specific questionnaire designed in 2 regional languages to assess the QoL in young adult patients with CLP of South India following comprehensive treatment.

A preliminary questionnaire was created from the literature review and patient interviews, considering regional sociodemographic conditions. The questionnaire was then validated by subject experts and pilot tested. The resultant tool was implemented on patients at treatment completion. Data collected were assimilated for statistical evaluation.

The questionnaire was deemed reliable (Cronbach α = .854 and test–retest reliability, κ = 0.8) and was administered to 100 young adult patients with CLP (mean age: 22 years). A large majority (83%) of the population felt more confident about themselves, with positive responses to familial relations, social interaction, and self-image. About 25% of the patients faced problems with speech regularly, while a majority of patients did not face problems with chewing and swallowing. Nearly 60% of patients were fully satisfied with their facial appearance, while others had concerns about their lip and nose aesthetics. The results were descriptive of the local population.

Most patients achieved satisfactory QoL in all domains following comprehensive multispeciality therapy. The novel tool is simple, reliable, and can be adapted to homogenous population groups.

Maintaining and improving patients’ quality of life (QOL) are regarded as the most important aims in health-care systems. These are directly associated with intervention of health-care providers across the world. The aim of the present study was to evaluate the impact of teamwork on frequency of care provided to children with cleft lip and palate and their mothers’ QOL.

This analytical epidemiology study was conducted on 101 children with cleft lip and palate and their mothers who were divided into 2 groups: a multidisciplinary team and a group of individual providers. Data were collected using convenience sampling. Quality of Life Questionnaire (Short Form-36) was assessed. The statistical analysis was performed using the χ² test, independent t test, and Mann-Whitney U test in SPSS (20).

Both groups were matched. There was a significant statistical difference among the members of multidisciplinary team who received surgery, genetic counseling, and dental care (P ≤ .043) and the individual providers groups. The 2 groups did not have significant difference in receiving the hearing test, speech therapy, and dental care 12 months before the interview. There were significant differences in QOL scores between the multidisciplinary team and the group of individual providers (P = .013).

The services provided as a multidisciplinary team leads to a better outcome and improves the QOL of our patients and their families. It is recommended that services should be provided in the team approach for patients with cleft lip and palate.

To compare oral health-related quality of life (OHRQoL) before treatment of adults with unilateral cleft lip and palate (UCLP) and surgical Class III malocclusion, and to consider if clefts needing different orthodontic treatment protocols could influence people’s self-perception.

Cross sectional.

Cleft Lip and Palate Center and Clinic of Orthognathic Surgery from a School of Dentistry.

A sample of adults with repaired nonsyndromic UCLP (n = 52) which was age- and sex-matched with a noncleft Class III malocclusion sample seeking orthognathic surgery (n = 51). In turn, the cleft group was subdivided according to treatment planning into nonsurgical orthodontic and surgical orthodontic approaches.

The whole sample was assessed using the short-form oral health impact profile (OHIP-14), with higher scores indicating a poorer OHRQoL. Statistical comparisons were performed with Mann-Whitney U and Kruskal-Wallis tests, and effect size. Bonferroni adjustment was used for post hoc tests (P < .017).

The OHIP-14 scores of the UCLP and Class III groups were significantly different (P = .001, η² = 0.108), and higher in Class III. The largest commitment was in the physical disability, physical pain, and psychological disability domains. In addition, no differences were found when the UCLP treatment planning was considered.

Surgical Class III malocclusion have a poorer OHRQoL when compared to patients with UCLP, irrespective of whether they are treated surgically or orthodontically. Therefore, the greater commitment of OHRQoL appears to be influenced by the etiology of Class III, and not by treatment plan.

To compare the cephalometric characteristics of patients with and without Opitz G/BBB syndrome type I.

Cross-sectional, case–control study.

Tertiary cleft center in Brazil.

Eighteen individuals with Opitz G/BBB syndrome with complete bilateral cleft lip and palate (BCLP), compared to 18 individuals with nonsyndromic complete cleft lip and palate and 18 individuals without malformations, matched for gender and age.

Pretreatment lateral cephalograms of all patients were manually traced and digitized for achievement of linear and angular measurements.

Analysis of variance or Kruskal-Wallis followed by Tukey tests were used for intergroup comparisons at a significance level of P < .05.

Individuals with Opitz G/BBB syndrome exhibited alterations in SNGn, P-Co, and N’-Pr/Po-Or that were not attributable to BCLP. Co-Go, Sella-Nasion-Supramentale, ANB (maxillo-mandibular relationship), and anterior nasal spine-posterior nasal spine (ANS-PNS)/U1A-U1T were significantly different in both G/BBB and BCLP groups compared to control, but not different between G/BBB and BCLP groups. Anterior nasal spine-posterior nasal spine/Go-Gn, ANS-PNS, V-Upper pharyngeal wall, and U-lower pharyngeal wall were different in nonsyndromic BLCP compared to nonsyndromic controls and Opitz G/BBB group.

Patients with Opitz G/BBB syndrome exhibited some unique cephalometric alterations compared to patients with nonsyndromic complete BCLP and controls.

The gold standard for diagnosis of craniosynostosis is a clinical examination and motionless head computed tomography (CT). Computed tomography sedation is associated with increased cost, resource utilization, medical, and possible developmental risks. This study investigates whether a “feed and swaddle” protocol can be used to achieve diagnostic quality craniofacial imaging without the use of infant sedation.

Prospective cohort study.

Tertiary academic medical center.

Ninety patients <18 months of age undergoing evaluation for craniosynostosis from 2012 to 2018.

A feed and swaddle protocol.

Diagnostic level imaging without the use of infant sedation.

Eighty-five (94%) achieved a diagnostic quality craniofacial CT scan using the “feed and swaddle” method. Mean patient age was 24.0 ± 10.0 weeks. Craniosynostosis was diagnosed in 74% of patients. Mean age of patients with successful completion of a CT scan was 23.7 ± 9.6 weeks, compared to 27.2 ± 17.1 weeks for unsuccessful completion. Mean weight for the successful group was 15.6 ± 2.9 pounds and 15.9 ± 2.5 pounds for the unsuccessful group. Mean travel distance was 59.2 ± 66.5 miles for successful patients and 66.5 ± 61.5 miles for unsuccessful patients. For the unsuccessful patients, there were no delays in surgical planning or scheduling.

The “feed and swaddle” protocol described here is an effective alternative to infant sedation for motionless craniofacial CT imaging.

To describe a reliable method utilizing large, rotation flaps to reconstruct a number 10 Tessier cleft.

This is a descriptive clinical case report.

Pediatric teaching hospital affiliated with a university.

One participant in this clinical case report.

Full-thickness excision of the clefted eyelid, brow, and forehead tissue was performed bilaterally to develop medial and lateral eyebrow and forehead flaps. Right: the forehead/brow flap was rotated caudally to reapproximate the eyebrow and an eyelid rotation flap was also used to reapproximate the lid margin. Left: forehead/brow rotation flap allowed realignment of the eyebrow and a series of Z-plasties were used in the eyelid to reapproximate the lid margin and to lengthen the eyelid.

Develop and construct a reliable reconstruction with full-eyelid closure and minimal donor site morbidity.

Complete eyelid closure bilaterally was achieved intraoperatively, and was maintained at 6-month follow-up with no evidence of ocular pathology.

Large, bilateral upper eyelid colobomas require repair to prevent blindness. Although free tarsomarginal grafts and lid-sharing procedures have been described, we demonstrate that large rotation flaps designed along the cleft margin can provide a reliable reconstruction and minimize donor-site morbidity.