Divergent growth of the transient brain compartments in fetuses with nonsyndromic isolated clefts involving the primary and secondary palate.

Cleft lip/palate is a common orofacial malformation that often leads to speech/language difficulties as well as developmental delays in affected children, despite surgical repair. Our understanding of brain development in these children is limited. This study aimed to analyze prenatal brain development in fetuses with cleft lip/palate and controls. We examined in utero MRIs of 30 controls and 42 cleft lip/palate fetal cases and measured regional brain volumes. Cleft lip/palate was categorized into groups A (cleft lip or alveolus) and B (any combination of clefts involving the primary and secondary palates). Using a repeated-measures regression model with relative brain hemisphere volumes (%), and after adjusting for multiple comparisons, we did not identify significant differences in regional brain growth between group A and controls. Group B clefts had significantly slower weekly cerebellar growth compared with controls. We also observed divergent brain growth in transient brain structures (cortical plate, subplate, ganglionic eminence) within group B clefts, depending on severity (unilateral or bilateral) and defect location (hemisphere ipsilateral or contralateral to the defect). Further research is needed to explore the association between regional fetal brain growth and cleft lip/palate severity, with the potential to inform early neurodevelopmental biomarkers and personalized diagnostics.

Compound heterozygous variants in RAB34 in a rare skeletal ciliopathy syndrome.

Skeletal ciliopathies are a heterogenous group of congenital disorders characterized by multiple internal abnormalities, and distinct radiographic presentation. Pathogenic variants in at least 30 cilia genes are known to cause skeletal ciliopathies. Here we report a fetus with an atypical skeletal ciliopathy phenotype and compound heterozygous variants in the RAB34 gene. The affected fetus had multiple malformations, including posterior neck edema, micrognathia, low-set and small ears, auricular hypoplasia, cleft lip and palate, short extremities, and a combination of rarely occurring pre- and postaxial polydactyly. Genome sequencing identified compound heterozygous variants in the RAB34 gene: maternal c.254T>C, p.(Ile85Thr), and paternal c.691C>T, p.(Arg231*) variants. Only the paternal variant was present in the unaffected sibling. Evidence in the literature indicated that Rab34-/- mice displayed a ciliopathy phenotype with cleft palate and polydactyly. These features were consistent with malformations detected in our patient supporting the pathogenicity of the identified RAB34 variants. Overall, this case report further expands genetic landscape of human ciliopathy syndromes and suggests RAB34 as a candidate gene for skeletal ciliopathies.

The Hard Palate Sweep: a multiplanar 2-dimensional sonographic method for the prenatal detection of cleft palate.

BACKGROUND: Prenatal diagnosis of cleft palate is challenging. Numerous 2-dimensional and 3-dimensional methods have been proposed to assess the integrity of the fetal palate, yet detection rates remain relatively low. We propose the "Hard Palate Sweep," a novel 2-dimensional method that enables clear demonstration of the entire fetal palate throughout pregnancy, in a single sweep, avoiding acoustic shadows cast by surrounding bones. OBJECTIVE: This study aimed to assess the feasibility and performance of the Hard Palate Sweep, performed throughout pregnancy. STUDY DESIGN: This was a prospective cross-sectional study performed between 2018 and 2022 in pregnant patients referred for a routine or targeted anomaly scan between 13 and 40 weeks of gestation. The presence or absence of a cleft palate was determined using the "Hard Palate Sweep." This was compared with the postnatal palate integrity assessment. Test feasibility and performance indices, including sensitivity, specificity, and positive and negative predictive values were calculated. Offline clips were reviewed by 2 investigators for the assessment of inter- and intraoperator agreement, using Cohen's kappa formula. The study protocol was approved by the institutional ethics committee. All participating patients were informed and provided consent. RESULTS: A total of 676 fetuses were included in the study. The Hard Palate Sweep was successfully performed in all cases, and 19 cases were determined to have a cleft palate. Of these, 13 cases were excluded because postmortem confirmation was not performed, leaving 663 cases available for analysis. Six cases determined to have a cleft palate were confirmed postnatally. In 655 of 657 cases prenatally determined to have an intact palate, this was confirmed postnatally. In the 2 remaining cases, rare forms of cleft palate were diagnosed postnatally, rendering 75% sensitivity, 100% specificity, 100% positive predictive value, and 99.7% negative predictive value for the Hard Palate Sweep (P<.001). There was complete intra- and interoperator agreement (kappa=1; P<.0001). CONCLUSION: The Hard Palate Sweep is a feasible and accurate method for prenatally detecting a cleft palate. It was successfully performed in all attempted cases between 13 and 40 weeks of gestation. This method is reproducible, offering high sensitivity and specificity. Implemented routinely, the Hard Palate Sweep is expected to increase the prenatal detection of cleft palate.

Prenatal detection of orofacial clefts in Denmark from 2009 to 2018.

OBJECTIVE: To investigate the overall and type-specific prenatal detection rates (DRs) of orofacial clefts in a national cohort in Denmark. METHODS: This study was based on data from the Danish Fetal Medicine Database and included all fetuses and children from singleton pregnancies diagnosed with an orofacial cleft prenatally and/or postnatally between 2009 and 2018. The types of cleft included unilateral, bilateral or median cleft lip (CL); unilateral, bilateral or median cleft lip with secondary cleft palate (CLP); and cleft palate (CP). The clefts were grouped as cleft lip with or without cleft palate (CL(P)) or as all clefts (including CP). All cases with discordance between prenatal and postnatal diagnoses were validated in the local patient files (Astraia). Cases without prenatal validation of the postnatal diagnosis were marked as undetected. Postnatally diagnosed cases with a strong prenatal suspicion of a cleft but without an International Classification of Diseases-10 code were registered as prenatally detected. Termination of pregnancy and intrauterine death were registered as true positives even if no autopsy could be performed. Liveborn cases with a prenatal diagnosis but without a postnatal validation were excluded. RESULTS: A total of 994 cases were included in the study, of which 933 were liveborn. The prevalence of orofacial cleft was 1.6 per 1000 live births. There were no differences in the baseline characteristics between detected and undetected cases. The DR for CL(P) was 71.7% (95% CI, 64.8-78.9%), with an increase from 60.0% in 2009 to 73.0% in 2018 (P = 0.018). The type-specific DRs for the entire period were 56.4% (95% CI, 45.0-67.6%) for unilateral CL; 76.6% (95% CI, 71.7-82.9%) for unilateral CLP; 70.5% (95% CI, 52.1-87.6%) for bilateral CL; 82.3% (95% CI, 70.6-93.6%) for bilateral CLP; 0% (0/6) for median CL; 75.0% (3/4) for median CLP; and 3.3% (95% CI, 0.6-5.7%) for CP. A total of 20.9% (208/994) of the cases had associated findings, of which 33.2% (69/208) were genetic aberrations. CONCLUSIONS: The DR for CL(P) has improved in Denmark over the last decade. The DR for CLP is high, with the highest DR for bilateral CLP. However, prenatal detection of CP remains a challenge. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

A novel MED12 pathogenic variant in a female fetus with facial cleft and cardiac defects identified in the first trimester.

Trio exome sequencing was performed on a female fetus with an increased nuchal translucency, along with nasal bone hypoplasia, suspected cleft palate and abnormal outflow tract of the heart. A de novo heterozygous variant c.5500_5507del, p.(Tyr1834Argfs × 58) in the MED12 gene was detected. Loss-of-function variants in MED12 in females are associated with Hardikar syndrome (HS). A follow-up ultrasound at 15+5 weeks of gestation identified multiple fetal anomalies including bilateral cleft lip and palate, diaphragmatic hernia, atrioventricular septal defect, persistent truncus arteriosus, and bilateral renal pelvis dilation. Fetal autopsy confirmed the prenatal sonographic findings, and the MED12 variant was discussed by our multidisciplinary team to be the cause of fetal anomalies. Our case is the first prenatal one in which HS was diagnosed due to first trimester structural malformations. This case report presents another example of early identification of a major anomaly which allows earlier genetic diagnosis and more time for clinical management.

An effective ultrasound fetal palate screening software based on the "sequential sector scan through the oral fissure" and three-dimensional ultrasound.

BACKGROUND: Orofacial clefts are one of the most common congenital malformations of the fetal face and ultrasound is mainly responsible for its diagnosis. It is difficult to view the fetal palate, so there is currently no unified standard for fetal palate screening, and the diagnosis of cleft palate is not included in the relevant prenatal ultrasound screening guidelines. Many prenatal diagnoses for cleft palate are missed due to the lack of effective screening methods. Therefore, it is imperative to increase the display rate of the fetal palate, which would improve the detection rate and diagnostic accuracy for cleft palate. We aim to introduce a fetal palate screening software based on the "sequential sector scan though the oral fissure", an effective method for fetal palate screening which was verified by our follow up results and three-dimensional ultrasound and to evaluate its feasibility and clinical practicability. METHODS: A software was designed and programmed based on "sequential sector scan through the oral fissure" and three-dimensional ultrasound. The three-dimensional ultrasound volume data of the fetal face were imported into the software. Then, the median sagittal plane was taken as the reference interface, the anterior upper margin of the mandibular alveolar bone was selected as the fulcrum, the interval angles, and the number of layers of the sector scan were set, after which the automatic scan was performed. Thus, the sector scan sequential planes of the mandibular alveolar bone, pharynx, soft palate, hard palate, and maxillary alveolar bone were obtained in sequence to display and evaluate the palate. In addition, the feasibility and accuracy of the software in fetal palate displaying and screening was evaluated by actual clinical cases. RESULTS: Full views of the normal fetal palates and the defective parts of the cleft palates were displayed, and relatively clear sequential tomographic images and continuous dynamic videos were formed after the three-dimensional volume data of 10 normal fetal palates and 10 cleft palates were imported into the software. CONCLUSIONS: The software can display fetal palates more directly which might allow for a new method of fetal palate screening and cleft palate diagnosis.

Maxillary arch dimensions in bilateral cleft lip and palate in the age 0-5 months.

INTRODUCTION: The complete bilateral cleft lip and palate (BCLP) divides the maxillary arch into three segments, separated from each other, resulting in abnormal growth of the alveolar arch. This study evaluated the maxillary arch dimensions in BCLP and compared them with neonates without craniofacial anomalies. METHODS: This retrospective study was conducted in a tertiary cleft centre. Sixty-six neonates aged 0-5 months were divided into two groups: cleft group-children with BCLP (23 boys and 18 girls) and control group-children without craniofacial deformities (15 boys and 10 girls). The dental models were processed by a 3D scanner. Landmarks were marked to achieve inter-canine distance, inter-tuberosity distance and arch length measurements. t-Tests were used for intergroup comparisons (p < .05). RESULTS: The maxillary cleft arch was demonstrated to be wider and longer in the posterior region compared to the control group. The inter-canine distance did not present differences between the cleft and controls. The inter-canine distance of the control group was the only measurement influenced by the variable sex. CONCLUSIONS: The cleft significantly interfered with the arch posterior width and arch sagittal length, making them larger. There was no statistical difference in the measurements between sex in the cleft group.

Three-dimensional evaluation of secondary alveolar bone grafting in patients with unilateral cleft lip and palate: A 2-3 year post-operative follow-up.

OBJECTIVES: The present study aims to quantitatively assess secondary alveolar bone graft (SABG) resorption in unilateral cleft lip, alveolus and palate (UCLAP) patients in a 2-3 year longitudinal follow-up setting by using a validated 3D protocol. Furthermore, the potential relation of SABG resorption with maxillary canine position and a number of patient-related factors was investigated. METHODS: UCLAP patients who underwent SABG and had good quality CBCT images at the following timepoints were included in the study: pre-operative (T0), immediate (T1), 6 months (T2) and either 1-2 years (T3) or 2-3 years (T4) post-operative. The final bone grafted region was defined on the T1 scans and refined in the registered T0 scans. The bone graft after resorption was determined by applying threshold-based segmentation on the registered T2, T3 or T4 scans within the segmented bone graft volume. The position of the canines was determined at every timepoint at the cleft and non-cleft side. RESULTS: Forty-five UCLAP patients (mean age 9.0 ± 1.3 years) were included. In the first 6 months after SABG, 43.6% bone resorption was recorded. 2-3 years post-operative, 56% bone resorption was found if the maxillary canine was not yet erupted and 42.7% if it erupted through the graft. The vertical position of the canines was significantly higher on the cleft side at T3. CONCLUSIONS: The present study reports significant SABG resorption over time. However, no correlation was found between SABG resorption and canine position, nor between other patient-related factors.

What is the Success Rate of Repeat Alveolar Bone Graft?

BACKGROUND: Success rates for alveolar bone grafting range from 30 to 96%. There is limited information regarding the success of repeat grafts. PURPOSE: The purpose of this study was to determine the radiographic success rate of repeat alveolar bone grafts. STUDY DESIGN: The study designs was a retrospective cohort study of patients who underwent repeat grafting by 1 surgeon over 15 years. To be included, subjects had to have: cleft lip and alveolus and a cone-beam computed tomography (CBCT) scan obtained >6 months after repeat graft. Patients were excluded if CBCT was inadequate. PREDICTOR VARIABLE: Predictor variables were sex, age at repeat graft, cleft type, presence of an erupted canine, premaxillary osteotomy at time of repeat graft, presence of a visible oronasal fistula, size of bony defect, presence of a bony palatal bridge, and whether the surgeon who performed the repeat graft also performed the initial graft. MAIN OUTCOME VARIABLE: The outcome variable was graft success determined using CBCT assessment and defined as a score of >3 out of 4 in each domain: vertical bone level, labiopalatal thickness, and piriform symmetry. COVARIATES: The covariates were time from bone graft to CBCT (months) and age at time of CBCT (years). ANALYSES: Frequency distributions, relative risk with 95% confidence intervals, medians, and interquartile ranges were calculated. Pearson c2 and Fisher exact tests were performed to determine predictors of outcome. A P < .05 was considered statistically significant. RESULTS: Fifty subjects (54% male) who had repeat bone grafting to 59 cleft sites were included. Median follow-up time from repeat graft to CBCT was 7.0 months (interquartile range: 5.9 months). The radiographic success rate was 81.4%:91.7% if the same surgeon performed both initial and repeat grafts, but 78.7% if initial graft was completed by another surgeon (P = .43). CONCLUSION AND RELEVANCE: Despite being a multifactorial issue, extensive and bilateral clefts, the presence of an erupted tooth in the cleft area, a visible oronasal fistula, and concomitant osteotomy of the premaxilla are warning signs of the possibility of failure. Performing repeat alveolar bone grafting by an experienced surgeon appears to increase the chance of success.

Cortical Block Grafting Successfully Augments Alveolar Cleft Sites for Dental Implant Placement.

BACKGROUND: Supplemental bone grafting is regularly required before dental implant placement in patients with cleft lip and palate (CLP). PURPOSE: The study purpose was to measure and compare implant osseointegration and changes in graft dimensions following lateral incisor onlay cortical bone grafting in CLP and non-CLP patients. STUDY DESIGN, SETTING, SAMPLE: Retrospective cohort study composed of patients who presented to Boston Children's Hospital and underwent autogenous onlay cortical block bone grafting at lateral incisor sites from 2015 through 2023. Patients were excluded if the cone beam computed tomography (CBCT) quality was insufficient for accurate measurements. PREDICTOR VARIABLE: The predictor variable was CLP status coded as CLP or non-CLP. MAIN OUTCOME VARIABLE: The primary outcome variable was successful implant osseointegration confirmed by a torque of 35 N/cm or more after 3 months of implant healing. Secondary outcomes were change in bone width between preoperative and postoperative CBCT scans at lateral incisor sites and the need for additional bone augmentation prior to or during implant placement. COVARIATES: Covariates were age, sex, cleft location, and time from bone graft to postoperative CBCT and implant placement. ANALYSES: Data analyses were performed using t-tests, Fisher's exact tests, Mann-Whitney U tests, and Pearson's correlation. P < .05 was considered statistically significant. RESULTS: A total of 22 subjects (16 with CLP) were evaluated. The mean age at the time of graft was 19.3 ± 2.4 years with 52.6% males. Implants were osseointegrated at 20 of 22 lateral incisor sites (1 CLP failure, 1 non-CLP failure). There was significant change in bone width after grafting for patients with CLP (P < .001). Patients with CLP experienced a 3.32 (± 1.80) mm and 2.99 (± 1.61) mm increase in bone width at 2 different levels. Patients with CLP achieved greater boney changes near the alveolar crest than noncleft patients (P = .008) but the change was not significantly different more apically (P = .86). One subject with CLP required additional grafting during implant placement. CONCLUSION AND RELEVANCE: Cortical block onlay bone grafting is a predictable technique to augment lateral incisor sites in patients with CLP for placement of a dental implant.

Accuracy of prenatal detection of facial clefts and relation between facial clefts, additional malformations and chromosomal abnormalities: a large referral-center cohort.

PURPOSE: Facial clefts belong to the most common congenital malformations and their prenatal diagnosis is a constant challenge. The aim of this study was to determine the accuracy of prenatal ultrasound in correctly classifying facial clefts. Furthermore, we aimed to specify the distribution of the type of clefts and underlying genetic conditions. METHODS: All fetuses seen with suspected facial cleft in the Department of Obstetrics, Charité - Universitätsmedizin Berlin during a period of 23 years (1999-2022) were included in this retrospective study. Clefts were classified according to the classification of Nyberg. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed. RESULTS: 292 patients were included in the study. The most common type of clefts were unilateral cleft lip and palate (CL-P) (53.6%) and bilateral CL-P (30.6%), followed by CL (8.1%), CP (5.1%) and median CL-P (2.6%). The overall pre- and postnatal concordance rate corresponding to a correct prenatal diagnosis was high, 88.9%, ranging from 73.7% (CL) to 93.7% (unilateral CL-P). Most of the median clefts (95.2%) and CP (93.3%) were associated with other sonographic abnormalities, as well as 52.2% of bilateral CL-P. Chromosomal abnormalities, mostly trisomy 13 and trisomy 18, were observed in in the median CL-P (47.6%), bilateral CL-P (31.1%) and CP (26.7%) groups, in contrast to the CL (9.1%) and unilateral CL-P (12.9%) groups. It was exceptional to have a chromosomal abnormality without additional malformations (4.8%). The mortality rate including one late miscarriage, 5 IUFD's, 74 TOPs and 6 palliative cares at birth was 29.8%, particularly high for median clefts (90.5%). CONCLUSION: Prenatal ultrasound exhibited a high accuracy to assess the type of facial clefts with an average rate of 88.9% (73.7%-93.7%) and a concordance rate of up to 93.7%, depending on the type of cleft. The search for additional malformations as well as clarifying underlying genetic conditions is essential. This allows for a targeted counseling of the parents and to best prepare for postnatal care, including surgery by the maxillofacial team.

Comparison of craniomaxillofacial morphology in children with unilateral cleft lip and palate treated with/without presurgical nasoalveolar molding - a retrospective study with a mean age of 5 years.

OBJECTIVES: To three-dimensionally assess differences in craniomaxillofacial skeletal development in patients with operated unilateral cleft lip and palate (UCLP) treated with/without presurgical nasoalveolar molding (PNAM) with a mean age of 5 years. MATERIALS AND METHODS: Cone-beam CT radiographs of 30 patients with UCLP who had undergone PNAM and 34 patients with UCLP who did not receive PNAM were analyzed. The data were stored in DICOM file format and were imported into the Dolphin Imaging program for 3D image reconstruction and landmark identification. 33 landmarks, 17 linear and three angular variables representing craniofacial morphology were analyzed and compared by using the Mann-Whitney U tests. RESULTS: The vast majority of linear variables and 3D coordinates of landmark points reflecting craniofacial skeletal symmetry were not significantly different between the two groups. In terms of craniofacial skeletal development, the PNAM group had a significantly smaller anterior nasal spine offset in the midsagittal plane and a greater maxillary length compared to the non-PNAM group. CONCLUSIONS: Evaluations performed in early childhood showed that treatment with/without PNAM in the neonatal period was not a major factor influencing craniomaxillofacial hard tissue development in patients with UCLP; moreover, PNAM treatment showed significant correction of skeletal deviation at the base of the nose. CLINICAL RELEVANCE: Follow-up in early childhood has shown that PNAM treatment administered during the neonatal stage does not impede maxillary development and has benefits in correcting nasal floor deviation. It is a viable option for improving nasal deformity in children with unilateral cleft lip and palate.

Three-dimensional Assessment of Longitudinal Surgical Outcome in Patients with Unilateral Cleft Lip and Palate: A Modified Rotation Advancement Technique.

BACKGROUND: Optimization of a modified rotation advancement technique is hampered by lack of objective measures to quantify the longitudinal surgical outcome. METHODS: We collected and assessed facial 3D images of 115 consecutive patients who underwent primary repair between 2017 and 2019. Photogrammetry was performed preoperatively, immediately postoperatively and at a first and second follow-up interval, occurring at an average year of 0.6 and 5.3 years, respectively. 10 additional age-matched noncleft control subjects were also included. RESULTS: Growth lag in cleft side lateral lip and gradual elongation of medial lip height on the cleft side caused continuous deviation of philtrum towards the cleft side. The columellar length on the cleft side continued to grow slower, accompanied by a persistent widening of alar base width on the cleft side, leading to in the gradual deviation of columella towards the cleft side. The pre-operative and post-operative nasolabial asymmetry would increase with greater degree of postoperative deficiencies. Right clefts presented with greater degrees of deficiencies in lateral lip height in preoperative measurement, but this discrepancy of the laterality of clefts was not observed in the two follow-up periods. CONCLUSION: The surgical outcome of this modified rotational advancement technique in unilateral cleft lip primary repair is promising. Growth lag in lateral lip and lateral displacement of alar base cause continuous deviation of philtrum towards the cleft side. Pre-operative severity does predict post-operative outcomes. Laterality of oral clefts does not significantly affect the long-term outcomes of surgery. PRACTICAL IMPLICATION: This surgical technique meets the current trend of cleft lip and palate primary repair and is worth promoting.

Stability between single and segmental maxillary osteotomies in bimaxillary surgery for cleft-associated class III deformity: a CBCT study.

OBJECTIVE: Segmental Le Fort I osteotomy through the cleft is a common strategy to narrow the alveolar cleft in adults. This study compared skeletal stability between single and segmental Le Fort I osteotomies in patients with unilateral cleft lip and palate (UCLP). MATERIALS AND METHODS: This retrospective analysis examined 45 adults with complete UCLP-associated class III deformities who underwent bimaxillary surgery with either single (n = 30) or segmental (n = 15) Le Fort I advancement. Cone beam computed tomography (CBCT) scans of the facial skeleton were acquired before surgery, 1-week postsurgery, and at follow-up. Measures of landmarks from the CBCT images for the two treatment groups were compared for translation (left/right, posterior/anterior, superior/inferior) and rotation (yaw, roll, pitch). RESULTS: Postsurgery, the downward movement of the maxilla was larger in the segmental group than the single group. At follow-up, the maxilla moved backward in both groups, and upward in the segmental group. The mandible moved forward and upward and rotated upward in both groups. The amount of upward movement and rotation was larger in the segmental group than the single group. CONCLUSIONS: Two years after bimaxillary surgery in patients with UCLP-associated class III deformity, greater relapse was found after segmental Le Fort I osteotomies in vertical translation of the maxilla and mandible, and pitch rotation of the mandible compared with single Le Fort I osteotomies. CLINICAL RELEVANCE: The vertical relapse of the maxilla was larger after segmental Le Fort I advancement compared with single Le Fort I advancement in clefts.

Quantitative Assessment of Lip Morphology in Patients With and Without Cleft Lip and Palate Using 3-Dimensional Stereophotogrammetry.

This study aimed to assess and quantify the morphologic characteristics of the lips and the lower third of the face in cleft and noncleft patients, utilizing three-dimensional (3D) stereophotogrammetry. Sixty patients were included in the study, comprising 30 unilateral cleft lip and palate patients (G1, 24 female, 6 male; aged 20 to 60 y, mean age 44.0±12.0 y) and 30 noncleft patients (G2, 23 female, 7 male; aged 20 to 59 y, mean age 43.5±12.0 y). Anthropometric landmarks were identified on the facial surface. Three-dimensional stereophotogrammetry was employed to capture images. Statistical analysis was conducted to compare the groups, with a significance level set at 0.05. The comparative analysis revealed statistically significant differences in 5 linear and 6 angular measurements. Linear measurements such as philtrum width, upper and lower cutaneous lip height, mandibular ramus length, and midfacial depth exhibited significant differences between cleft and noncleft patients. Similarly, angular measurements, including upper lip angle, Cupid's bow angle, lower/medium face convexity, lip seal, nasolabial angle, and left gonial angle, displayed statistically significant disparities. These findings underscore the ongoing surgical challenges in the comprehensive rehabilitation of patients with clefts, highlighting the critical need for continued advancements in treatment strategies.

Correlating Jaw Bone Growth and Nasal Morphology Among Adult Patients With Cleft Lip and Palate: A Retrospective Cephalometric Study.

This retrospective cross-sectional study reviewed adult patients with operated cleft lip and/or palate (CL/P) and normal control, and performed comprehensive craniofacial and nasal morphological analyses based on lateral cephalometric radiographs. Pearson or Spearman correlation coefficient assessed intraclass correlation. Seven hundred fifty-seven operated patients with CL/P, and 165 noncleft normal controls were enrolled. Among the normal and CL/P groups, S-N-A angle registered positive correlations with nasal base prominence (S-N'-Sn, degrees). Upper facial height (N-ANS, mm) had positive correlations with nasal dorsum length (N'-Prn, mm) and nasal bone length (N-Na, mm). Although in patients with bilateral cleft lip and palate, there were moderate negative correlations ( r =-0.541, P <0.05) with soft tissue facial profile angle (FH-N'Pog', degree) and nasolabial angle (Cm-Sn-ULA, degree). Correlation exists between the morphology of jaw bones and external nose among patients with CL/P. Maxillary sagittal insufficiency is associated with concave nasal profile, and maxilla height is associated with nasal length.

Three-dimensional Analysis of Skeletal Stability in Cleft Lip and Palate Patients Undergoing Bimaxillary Surgery With Le Fort I Osteotomy and Intraoral Vertical Ramus Osteotomy.

Orthognathic surgery in patients with lip and palate clefts is challenging owing to scar tissue from primary repairs and severe deformities. In this study, we evaluated the stability of Le Fort I osteotomy with intraoral vertical ramus osteotomy (IVRO) in patients with cleft lip and palate using 3-dimensional (3D) analysis. This retrospective study comprised 14 cleft lip and palate patients (3 females, 11 males; the average age at surgery: 23.8 y) who underwent bimaxillary orthognathic surgery involving Lefort I osteotomy and IVRO. Cone-beam computed tomography (CBCT) images were obtained at preoperative (T0), 1-month postoperative (T1), and 1-year follow-up (T2) periods. The 3D analysis assessed the transitions and rotations of the maxilla and mandible using a virtual triangle. The maxilla showed stability in all directions at the 1-year follow-up with minimal relapse. The distal segment of the mandible exhibited clinically acceptable anterior (1.15 mm relapse) movements and pitch rotation (2.88° counterclockwise relapse) during the first year postoperatively. The proximal mandibular segment underwent anterior-inferior transition (1.21 and 2.01 mm, respectively) and lateral-outward rotation (4.90° and 7.51°, respectively) postsurgery, remaining unchanged 1 year postoperation. Le Fort I osteotomy with IVRO provides skeletal stability in patients with cleft lips and palates. This study demonstrated a minimal maxillary relapse and clinically acceptable movements in the distal mandibular segment during the first postoperative year. The proximal mandibular segment moved anteriorly and inferiorly, rotated laterally and outward after surgery, and remained unchanged at the 1-year follow-up.

Three-dimensional nasal asymmetry analysis between adolescence and adulthood in postoperative patients with unilateral cleft lip and palate using computed tomography.

AIM: To investigate the differences of the nasal soft and hard tissue asymmetry in postoperative patients with unilateral cleft lip and palate (UCLP) between adolescence and adulthood, and to explore the correlation of nasal soft and hard tissue asymmetry. METHODS: CT data from 47 repaired UCLP patients were included and divided into two groups:1. adolescent group: 23 patients (15 males, 8 females; age: 10-12 years old). 2. adult group: 24 patients (16 males, 8 females; age:18-32 years old). The three-dimensional asymmetry in nasal soft and hard tissues was analyzed. Additionally, the correlation between nasal soft and hard tissue asymmetry was also analyzed. RESULTS: Both the adolescent group and adult group showed asymmetries in nasal soft and hard tissues. Compared to the adolescent group, the adult group had a significantly increased horizontal asymmetry of nasal soft tissues Sbal (P < 0.05). Furthermore, the sagittal asymmetry of soft tissue Glat (P < 0.05), Sbal (P < 0.001), Sni (P < 0.001) and hard tissue LPA (P < 0.05) also increased significantly. In the adult group, there were more landmarks with a correlation between the asymmetry of nasal hard tissue and soft tissue compared to the adolescent group. There were moderate to strong correlations between nasal hard and soft tissue symmetries in the horizontal and sagittal directions (0.444 < r < 764), but no correlation in the vertical direction in the adult group (P > 0.05). CONCLUSIONS: The asymmetry of nasal soft and hard tissues in patients with repaired UCLP becomes more apparent in the horizontal and sagittal dimensions from adolescence to adulthood. The correlation between the asymmetry of nasal hard tissue and soft tissue becomes stronger in the horizontal and sagittal dimensions. These factors should be taken into account when performing treatment for repaired UCLP patients in adolescence and adulthood.

Optimization of orofacial cleft imaging protocols using device-specific low-dose cone-beam computed tomography.

OBJECTIVE: The aim of this study was to present optimized device-specific low-dose cone-beam computed tomography (CBCT) protocols with sufficient image quality for pre-surgical diagnostics and three-dimensional (3D) modelling of cleft defects. METHODS: Six paediatric skulls were acquired, and an artificial bony cleft was created. A high-resolution CBCT scan acted as a reference standard (Accuitomo 170, Morita, Kyoto, Japan) for comparing eight low-dose protocols of Newtom VGi-evo (QR Verona, Cefla, Verona, Italy), which included Eco and Regular protocols with different field of views (FOVs). Delineation of lamina dura, cementoenamel junction (CEJ), trabecular bone and bony bridge were assessed. A 3D model of the defect was also evaluated. RESULT: The dose area product of low-dose protocols ranged from 31 to 254 mGy*cm2. Despite the dose difference of up to eight times between applied protocols, trabecular bone and CEJ exhibited appropriate image quality in all scans. However, Regular small FOV protocols (5 × 5 and 8 × 5 cm2), for both lamina dura and bony bridge, demonstrated a significant improvement in image quality compared to Eco FOV counterparts. Based on 3D defect analysis, no significant difference existed between low-dose protocols and the reference standard. CONCLUSION: The findings highlight the possibility of achieving a considerable reduction (up to eight times) in the radiation dose using low-dose CBCT protocols while maintaining sufficient image quality for assessing anatomical structures and 3D modelling in cleft cases.

Comparison of Alveolar Bone Grafting Outcomes using CBCT in Individuals with UCLP Based on the Presurgical Orthodontic Treatment Methods.

OBJECTIVE: The purpose is to evaluate outcomes of alveolar bone grafting based on the pre-grafting orthodontic preparation methods. DESIGN: Retrospective analysis of individuals with unilateral cleft lip and palate. SUBJECTS AND SETTINGS: 28 individuals with non-syndromic UCLP from two craniofacial centers, 14 individuals each from XXXX and XXXX. INTERVENTIONS: The alignment group underwent maxillary expansion with incisors alignment while the non-alignment group underwent only maxillary expansion for presurgical orthodontic preparation. METHODS: Initial and post-surgical CBCT scans were compared to observe changes in angulation of the incisor adjacent to the cleft site, alveolar bony root coverage, and bone graft outcomes. RESULTS: In the alignment group, the buccolingual rotation decreased by 32.35 degrees (p = .0002), the anteroposterior inclination increased by 14.01 degrees (p = .0004), and the mesiodistal angulation decreased by 17.88 degrees (p = .0001). Alveolar bony coverage did not change after bone graft in both groups, and no difference was observed between the groups. Chelsea scale showed satisfactory bone graft outcome (category A, C) in 12 cases (85.71%) in the alignment group and 11 cases (78.51%) in the non-alignment group. The volumetric measurement showed the alignment group had better bone fill of 69.85% versus 51.45% in the non-alignment group (p = .0495). CONCLUSIONS: Alveolar bony coverage on the tooth adjacent to cleft sites did not change with alveolar bone grafting surgery in either of the alignment and non-alignment group. Presurgical orthodontic alignment does not induce root exposure nor poorer bone grafting outcome.