The Cleft Palate Craniofacial Journal

To determine associations of demographic, morphologic, and treatment protocol parameters with quality of life (QoL), appearance/speech satisfaction, and psychological adjustment.

Observational study utilizing retrospective report of protocol variables and current outcome variables.

Six North American cleft treatment clinics.

Children, ages 8.0–10.99 years, with Cleft Lip ± Alveolus, Cleft Palate, Cleft Lip and Palate, and parents (N = 284).

Pediatric QoL Inventory (PedsQL): Parent, Child, Family Impact Module (FIM); Patient Reported Outcome Measurement Information System (PROMIS); Child Behavior Checklist (CBCL); CLEFT-Q.

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 (β=.20) but lower CBCL Affective (β = −.16) and PROMIS Stigma scores (β= −.24). Incomplete cleft lip was associated with lower PROMIS Depression, and more positive ratings of CLEFT-Q: Nose, Nostril, Lip Scar; CBCL Competence scores, (βs = −.17 to .17). Younger Age at Lip Closure was associated with higher CBCL School Competence (β= −.18). Younger Age at Palate Closure was associated with higher Child PedsQL Total, Physical, Psychosocial QoL, and better CLEFT-Q Speech Function (βs = −.18 to −.15). Furlow Palatoplasty was associated with more CBCL Externalizing Problems (β = .17) higher CBCL Activities (β = .16). For all diagnoses, fewer Total Cleft-Related Surgeries was associated with lower PROMIS Stigma and higher CBCL Total Competence and Activities (βs = −.16 to .15).

Demographic characteristics, lip morphology, and treatment variables are related to later psychological functioning.

To quantify differences in levator veli palatini (LVP) muscle dimensions based on age, sex, and race and determine the typical range of asymmetry between the left and right sides of the LVP under age 2

Retrospective cohort study

Children's tertiary care hospital

A retrospective chart review of non-cleft patients under 2 who received a volumetric FLAIR MRI sequence within the past 18 months identified 216 patient scans. Exclusion criteria left 200 scans for analysis. Measurements of the LVP were obtained consistent with previous literature.

Corrected age, sex, and race

LVP muscle dimensions

A significant (P < .0001) difference between corrected age groups based on the overall combination of LVP variables after controlling for sex and race. Significant age differences included average extravelar length (P < .0001), average intravelar length (P < .0001), midline thickness (P < .0001), and average (P < .0001) and difference (P = .0413) in insertion thickness. Significant sex differences included average intravelar length (P = .0412) and average insertion thickness (P = .0281). Significant race differences included average insertion thickness (P = .0334) and difference in intravelar length (P = .0473). Differences between left and right total length (P = .0491) and angle of origin (P < .0001) were significant.

Differences in LVP morphology were observed in individuals under 2 years or age related to age, sex, and race. While asymmetry was significant in some dimensions, it varied by age range.

To develop an appropriate Chinese version of the CLEFT-Q through translation and cultural adaptation and to evaluate its reliability and validity.

The English CLEFT-Q was translated into Chinese following the International Society for Pharmacoeconomics and Outcomes Research guidelines, including cognitive debriefing interviews, and its reliability and validity were assessed.

Patients (N = 246) were mostly in active orthodontic treatment, had a mean age of 14.7 ± 4.4 years, 29% were female, and were born with isolated cleft lip ± alveolus (12%), cleft palate (1%), or cleft lip and palate (87%).

The Chinese CLEFT-Q, including 13 subscales covering Appearance, Health-Related Quality of Life (HRQOL), and Facial Function. Criterion validity instruments included the Negative Physical Self, Satisfaction with Life Scale, and Scale of Positive and Negative Experience.

The wording of 67 items was adapted in the final translation. The internal consistency of the Chinese version of the CLEFT-Q was high based on Cronbach's alphas of 0.85 to 0.98 and split-half reliability of 0.85 to 0.92. Exploratory and confirmatory factor analyses yielded three factors, which demonstrated construct validity by broadly matching the structure of the original CLEFT-Q. The Appearance and HRQOL dimensions had weak to moderate correlations (r = −0.35 to 0.67) with the corresponding instruments for criterion validity.

The Chinese version of the CLEFT-Q is a patient-reported outcome measure that can reflect the quality of life of Chinese patients with cleft lip and/or palate with good reliability and validity.

Treacher Collins syndrome is a rare congenital disease characterized by the multiple craniofacial malformations. Although the deformities affecting patients with Treacher Collins syndrome have been well characterized, the effects of these malformations to clinical severity of the syndrome are not well understood.

To determine the association of specific Treacher Collins mandibular malformations with clinical severity.

A retrospective radiographic observational study.

Study conducted at a single institution, a quaternary craniofacial care center

54 patients with Treacher Collins syndrome.

Computed tomography (CT), clinical photographs and medical history were included in this analysis. Mandibles were isolated from CT data and reconstructed in three dimensions using Mimics software. Cephalometric measurements were performed on CT data. Clinical severity was determined by Teber and Vincent scores. Association of craniofacial dysmorphology to clinical severity was determined by Spearman rank coefficient.

The main results obtained were the measurements of the mandibles and the quantification of the malformations of the evaluated patients

Among the most frequent findings in the sample are hypoplasia of the zygomatic complex, descending palpebral cleft and mandibular hypoplasia. Patients with a lower ramus/corpus ratio had a higher (more severe) Teber and Vincent classification.

Patients with the most compromised mandible are also the patients with the highest number of malformations, thus, the most severe patients.

Cleft lip repair (CLR) can be complicated by hypertrophic scar or keloid. Botulinum toxin type A (BTA) may improve postoperative scarring by reducing muscle tension and cytokine activity at the scar site. This systematic review analyzes the available evidence regarding the effect of BTA on scar quality after CLR.

The search was conducted in 6 different databases in accordance with PRISMA guidelines (PubMed, Scielo, Embase, Scopus, Web of Science, and Cochrane) using “botulinum toxin” and “cleft lip” as keywords.

Academic hospital

Exclusive to patients who underwent CLR and BTA injection

Mean visual analog scores (VAS), mean Vancouver scar scale (VSS), scar width, and BTA or CLR-related complications.

Five studies for a total of 216 patients met inclusion criteria. Four studies reported on primary CLR during infancy while 1 study recruited older patients seeking revision. All patients had BTA (range: 1-2 units/kg) injected in the orbicularis oris muscle. One study documented BTA injections in additional perioral muscles. All 4 studies that measured scar width and had a saline control arm found a significant decrease in width with BTA injection. Improvement of VAS and VSS with BTA was reported in 3 of 5 studies and 2 of 5 studies, respectively. There were no reports of complications associated with BTA or CLR.

The existing studies support the use of BTA injection to improve scar quality following CLR with low concern for complication. Further investigations with a greater number of patients are necessary.

To compare lower lip changes after Le Fort I advancement surgery in patients with a cleft.

Single institution, retrospective review.

Academic tertiary referral hospital.

Skeletally mature patients with a cleft who underwent one-piece Le Fort I advancement surgery who had a lateral cephalogram or cone-beam computed tomography (CBCT) scan preoperatively and at least 6 months postoperatively. Patients who underwent concomitant mandibular surgery or genioplasty were excluded. 64 patients were included: 45 male and 19 female, 25 with BCLP and 39 with UCLP. The mean age at surgery was 18.4 years.

Single jaw one-piece Le Fort I advancement surgery.

Standard lateral cephalometric landmarks of the bony skeleton and soft tissue were compared before and after Le Fort I advancement. Pearson correlation coefficients (r) were calculated to measure the correlation between lower lip position and other soft and hard tissue changes.

After comparable maxillary advancements [BCLP: 7.2 mm (95% CI: 6.2-8.3 mm), UCLP: 6.4 mm (95% CI: 5.7-7.0 mm)] the horizontal upper-to-lower lip discrepancy significantly improved in both groups. The lower lip became thinner and more posteriorly positioned. Changes in lower lip position correlated strongly with mandibular bony landmarks and moderately with upper lip position, but poorly with maxillary landmarks.

Le Fort I advancement results in posterior displacement of the lower lip and better lip competence, thereby improving facial harmony. This lower lip change is not predictable by degree of maxillary advancement, and does not differ in patients with BCLP vs. UCLP.

This study investigated the association of MSX1 gene variants rs3821949 and rs12532 with nonsyndromic cleft lip and/or palate (NSCL/P) in the Pakistani population.

Comparative cross-sectional study.

Setting: Multicenter of CL/P malformation.

Patients/Participants: Unrelated Non-Syndromic cleft Lip/Palate patients and healthy controls were enrolled.

One hundred (n = 100) subjects with NSCL/P and n = 50 unrelated healthy controls were enrolled in a multicenter comparative cross-sectional study. A tetra amplification refractory mutation system (ARMS) polymerase chain reaction (PCR) was performed to analyze MSXI gene single nucleotide variants (SNVs).

Among 100 NSCL/P subjects, the majority were males (56%; male: female = 1.27: 1). Most of the cases (74%) had cleft lip and palate (CLP) compared to isolated clefts. Genotyping of MSX1 gene variant rs3821949 showed an increased risk for NSCL/P in various genetic models (P < 0.0001), and the A allele exhibited a more than 4-fold increased risk among cases (OR = 4.22: 95% CI = 2.16–8.22; P < 0.0001). Our investigation found no significant difference between the rs12532 variation and NSCL/P.

Our study findings suggest that MSX1 gene variants may increase predisposition to NSCL/P in the Pakistani population. Further studies comprising large samples are required to identify the genetic aetiology of NSCL/P among our people.

This is a cross sectional study aimed to asses novel semi objectve compter assisted method to asses the symmetry amog unilateral cleft lip patients.

using Standard inferior view nose photos that were uploaded to Digimizer© Version 4.6.1 (2005–2016 MedCalc Software) Image Analysis, symmetry percentage was calculated and compared between cleft and non-cleft neonates. This method was compared to two subjective methods of rating symmetry.

This a cross sectional pilot study performed in the Univresity Medical Center using standard inferior views of unilateral cleft patients which were compared to non-cleft neonates.

Photographs of 71 neonates with unilateral complete and incomplete cleft lip and 30 neonates without facial cleft, which were born at the university medical center, Beer Sheba, Israel, were analyzed in a standard manner to determine nasal symmetry.

the novel method proposed produced a nominal value for percentage of symmetry.

Using Pearson's correlation test we found intra-rater reliability of 91.2% (p < 0.001) and Inter-rater reliability of 82.9% (p < 0.001). The analysis correlated with the surgeons subjective scores in both the numerical ranking method at 73% (p < 0.001) and 72.6% (p < 0.001) and the Ordinal ranking method at 87.6% (p < 0.001) and 77.7% (p < 0.001). The ability of the method to discriminate healthy from Cleft Lip patients overall was 92.3% With high sensitivity and specificity.

This method accurately distinguishes healthy patients from cleft lip patients. We have achieved High levels of inter and intra-rater reliability. Significant correlations were found between our semiobjective method and the subjective scores.

To assess the effect of secondary alveolar bone grafting (SABG) on maxillary growth in patients with unilateral cleft lip or palate (UCL/P)

Systematic review and Meta-analysis

Pubmed, Cochrane Library, Embase, Scopus, Web of Science and manual search was performed to assess the maxillary growth following SABG in UCL/P patients

Non-syndromic patients with UCL/P

Comparison of maxillary growth between patients with UCL/P who underwent SABG and UCL/P patients who had not undergone SABG or non-cleft control

39 of the identified 233 articles were assessed for inclusion and exclusion criteria after duplicate removal and title and abstract reading. 7 articles (1 prospective, and 6 retrospective studies) were included in the qualitative analysis and 2 articles were subjected to quantitative analysis. Four studies had a low risk of bias and three studies had a moderate risk of bias. Meta-analysis revealed a significant reduction of SNA and no significant difference in ANB in the SABG group compared to the non-cleft control group(I²= 0%). There was no significant difference in ANB between SABG and non-cleft control; however, results showed high heterogeneity(I²= 83%). Meta-analysis of SNA and ANB showed no significant difference between SABG and the cleft control group; however, there was high heterogeneity.

The studies showed a low to moderate risk of bias. SABG causes inhibition of maxillary growth in patients with cleft lip or palate when compared to patients with non-cleft control. Due to high heterogeneity, comparison to cleft control showed insufficient evidence.

To analyse the maxillary arch dimensions of children aged 9 and 12 with unilateral cleft lip and palate (UCLP) who received orthodontic treatment and secondary alveolar bone grafting.

This retrospective cohort study was performed on 30 patients with UCLP.

All patients were treated at the Cleft Lip and Palate Centre, which is part of the University Medical Centre Amsterdam and Academic Centre for Dentistry Amsterdam, the Netherlands.

Children with non-syndromic UCLP who received pre- and postsurgical orthodontics combined with secondary alveolar bone grafting between the ages of 9 and 12 years were included.

Maxillary arch dimensions were assessed on 60 digitised dental casts with measurements of the intermolar widths, interpremolar widths, arch perimeters, arch lengths, arch widths, and palatal depths.

The results of a paired-samples t-test revealed a statistically significant increase (P < .05) in intermolar width 1, intermolar width 3, interpremolar width 1, arch perimeter, and arch width between the ages of 9 (T0) and 12 (T1). Intermolar width 2 and the palatal depth decreased statistically significantly between T0 and T1.

Analysis of maxillary arch dimensions of children with UCLP indicates significant changes between 9 and 12 years of age. This suggests that orthodontic treatment and secondary alveolar bone grafting can be effective in improving maxillary arch dimensions. However, there is a need for collaborative research and data collection in order to provide sensible and evidence-based care to patients with cleft lip and palate.

Quantify the cost benefits of otoplasty under local as opposed to general anaesthesia.

A cost analysis of all components of otoplasty surgery under local anaesthesia (LA) in a minor operating room (OR) and general anaesthesia in a main OR was performed.

Our institution, compared to provincial/federal data, with costs converted into 2022 Canadian dollars.

Patients undergoing otoplasty under LA in the last year.

An efficiency analysis was performed by means of an opportunity cost, and the cost of failure was added to the overall LA costs.

Expenses for infrastructure, surgical and anaesthetic material, salaries, and personnel costs were derived from the literature, our hospital OR catalog and federal/provincial salary data, respectively. The cost of failure to tolerate local anaesthesia for such cases was also tabulated.

The true cost of LA otoplasty was computed as the absolute cost ($611.73) added to the cost of failure ($10.80), resulting in a total of $622.53/procedure. The true cost of GA otoplasty was calculated as the absolute cost ($2033.05) added to the opportunity cost ($1108.94), representing 3141.99$/procedure. The total savings when performing LA otoplasty to GA otoplasty are thus 2519.44$/case, with 1 GA otoplasty costing 5.05 LA otoplasties.

Otoplasty under local anaesthesia offers significant cost savings when compared with the same procedure under general anaesthesia. Economic considerations must be given particular attention given the elective nature of this procedure, which is often publicly funded.

Investigate associations between socioeconomic indicators of healthcare access with family compliance with cleft-related otologic and audiologic care within an interdisciplinary model.

Retrospective case series.

Children born 2005–2015 who presented to the Cleft-Craniofacial Clinic (CCC) at a quaternary care children's hospital.

Associations between main outcome measures and Area Deprivation Index (ADI), median household income for zip code, distance from hospital, and insurance status were evaluated.

Cleft types, ages at presentation to outpatient clinic (cleft, otolaryngology, and audiology), and ages at procedures (first tympanostomy tube insertion (TTI), lip repair, and palatoplasty) were measured.

Most patients were male (147/230, 64%) with cleft lip and palate (157/230, 68%). Median age at first cleft, otolaryngology, and audiology visits were 7 days, 86 days, and 5.9 months, respectively. Private insurance predicted lower no-show rates (p = .04). Age at first CCC visit was younger for patients with private insurance (p = .04) and older for those who lived further from the hospital (p = .002). Age at lip repair was positively correlated with national ADI (p = .03). However, no socioeconomic status (SES) proxy or proximity to hospital was associated with delays in first otolaryngology or audiology examination or TTI.

Once children become established within an interdisciplinary CCC, SES appears to bear little influence on cleft-related otologic and audiologic care. Future efforts should aim to elucidate which aspects of the interdisciplinary model maximize multisystem cleft care coordination and increase access for higher risk populations.

To perform a morphometric analysis of the upper cervical spine (UCS) by means of cone-beam computed tomography (CBCT) for the diagnosis of malformations or craniocervical instabilities in patients with cleft lip and palate (CLP) and class III malocclusion.

A total of 72 CBCTs from adult patients (48 male and 24 female) with Angle Class III malocclusion were divided into three groups: 1) Unilateral cleft lip and palate (UCLP) (n = 29; male = 65.5%; age = 24, 2 ± 4.2 years); 2) Bilateral cleft lip and palate (BCLP) (n = 18; male = 83.3%; age = 26.4 ± 6.0 years); 3) Control group (CON) (n = 25; male = 56.0%; age = 27.8 ± 9.3 years). The version 11.7 of the Dolphin® software (Chatsworth, California, USA) was used to evaluate the morphometric measurements and anomalies of the UCS. Data were analyzed by descriptive and inferential statistics (p ≤ 0.05).

For the UCLP, BCLP and CON groups, respectively, the measures were: atlantodental interval (2.1 ± 0.5; 2.1 ± 0.4; 2.0 ± 0.3 mm), basion-opisthion (35.9 ± 3.2; 36.4 ± 3.0; 34.7 ± 1.9 mm), hyoid-C3 (34.5 ± 3.7; 34.5 ± 5.2; 35.3 ± 4.5 mm), and hyoid-sella (108.1 ± 9.8; 111.3 ± 9.2; 109.7 ± 10 mm); clivus-canal angle (152.3 ± 13; 150.3 ± 10; 150.7 ± 10°) and Torg-Pavlov index (1.0 ± 0.2; 1.0 ± 0.1; 1.1 ± 0.2). Potentially unstable anomalies and malformations were more prevalent in the UCLP group (34,4%).

Subjects with UCLP presented compressive or unstable anomalies on upper cervical spine, more frequently than controls and BCLP, despite the lack of statistically significant differences among groups. Future studies could increase the safety of patients and healthcare professionals specialized in craniofacial anomalies.

Assess agreement of hypernasality and audible nasal emission (ANE) ratings between audio-recordings and a clinic setting.

Cross-sectional study using retrospective clinical recordings.

Audio-recording ratings by two trained speech language pathologists.

Percent agreement and intra- and inter-rater reliability of perceptual ratings.

Intra-rater reliability (AC2) of 167 audio-recorded speech samples for the primary and secondary raters, respectively, was 0.82 and 0.79 for hypernasality; for ANE, it was 0.57 and 0.75. Inter-rater reliability was 0.77 for hypernasality and 0.63 for ANE. When comparing ratings made from audio-recording versus the original clinical ratings, intra-rater reliability was 0.85 and 0.61 (primary and secondary rater, respectively) for hypernasality and 0.21 and 0.34 for ANE.

Ratings for hypernasality made from audio recordings were consistent with clinical evaluation, while ratings of ANE were not. ANE ratings made from audio recordings may not be a valid measure of velopharyngeal insufficiency speech characteristics.

To determine the efficacy of hyaluronic acid (HLA) lip filler to correct subtle cleft lip asymmetries in adults using a validated patient reported outcome measure (PROM).

Prospective cross sectional.

UK National Health Service.

Over 18 years old with repaired cleft lip and dissatisfaction of their lip appearance.

HLA lip filler injection.

A validated, cleft specific PROM, the lip module of CLEFT-Q™ prior to treatment and again after six weeks.

24 patients included. A mean total CLEFT-Q™ score pre-procedure was 14.9 (Stand deviation (SD) = 4.91) and 24 (SD = 6.08) post-procedure. Difference in mean total score pre- and post-procedure were statically significant (P = .0001) in all domains on the CLEFT-Q™. No adverse outcomes.

HLA filler to correct subtle cleft lip asymmetries in adults is a simple low risk technique, which can significantly improve the patient's perception of lip appearance.

HLA lip filler to correct subtle cleft lip asymmetries in adults is a simple technique, low risk procedure which can significantly improve the patient's perception of lip appearance.

To introduce a highly innovative imaging method to study the complex velopharyngeal (VP) system and introduce the potential future clinical applications of a VP atlas in cleft care.

Four healthy adults participated in a 20-min dynamic magnetic resonance imaging scan that included a high-resolution T2-weighted turbo-spin-echo 3D structural scan and five custom dynamic speech imaging scans. Subjects repeated a variety of phrases when in the scanner as real-time audio was captured.

Multisite institution and clinical setting

Four adult subjects with normal anatomy were recruited for this study.

Establishment of 4-D atlas constructed from dynamic VP MRI data.

Three-dimensional dynamic magnetic resonance imaging was successfully used to obtain high quality dynamic speech scans in an adult population. Scans were able to be re-sliced in various imaging planes. Subject-specific MR data were then reconstructed and time-aligned to create a velopharyngeal atlas representing the averaged physiological movements across the four subjects.

The current preliminary study examined the feasibility of developing a VP atlas for potential clinical applications in cleft care. Our results indicate excellent potential for the development and use of a VP atlas for assessing VP physiology during speech.