Biomechanical behavior of three maxillary expanders in cleft lip and palate: a finite element study.

This study evaluated the stress distribution in the dentoalveolar and palatal bone structures during maxillary expansion in a 17-year-old male patient with bilateral cleft lip and palate (BCLP) using expanders with dental (HYRAX) and skeletal anchorage (MARPE). For the generation of the specific finite element models, cone-beam computed tomography was used, and the DICOM files were exported to Mimics 3-Matic (Materialise) and Patran (MSC Software) software. Three specific three-dimensional models were generated: A) HYRAX: conventional four-banded hyrax screw (9 mm); B) MARPE-DS: 3 miniscrews (1.8 mm diameter - 5.4 mm length) and four-banded dental anchorage; and C) MARPE-NoDS: 3 miniscrews without dental anchorage. Maxillary expansion was simulated by activating the expanders transversely 1 mm on the "X" axis. HYRAX resulted in higher levels of deformation predominantly in the dentoalveolar region. MARPE-DS showed stress in the dentoalveolar region and mainly in the center of the palatal region, at approximately 4,000 µÎµ. MARPE-NoDS exhibited evident stress only in the palatal region. High stress levels in the root anchoring teeth were observed for HYRAX and MARPE-DS. In contrast, MARPE-NoDS cause stress on the tooth structure. The stress distribution from the expanders used in the BLCP showed asymmetric expansive behavior. During the initial activation phase of expansion, the HYRAX and MARPE-DS models produced similarly high strain at the dentoalveolar structures and upper posterior teeth displacement. The MARPE-NoDS model showed restricted strain on the palate.

Congenital midline upper lip sinus in an infant.

Congenital lip sinus is a rare entity with upper lip sinus being rarer than the lower lip sinus. It can be an isolated entity or associated with cleft lip, palate or Van der Woude syndrome. Syndromic association requires proper evaluation and aggressive surgical treatment. Preoperative delineation of the sinus tract with ultrasound sonography or MRI is mandatory. Simple excision is sufficient in cases of isolated sinuses. In this article, we report an infant with upper lip sinus managed successfully with simple excision and reviewed the literature.

Dental and skeletal maturation of Chinese male children with unilateral cleft lip and palate.

The aim of this study was to determine whether the relationship between dental age (DA), cervical stage (CS) and chronological age (CA) in Chinese male children with unilateral cleft lip and palate (UCLP) is similar to that of children without clefts. Panoramic and cephalometric radiographs of 105 male UCLP patients, aged from 8 to 16 years, were collected and compared to 210 age-matched healthy control males. The Demirjian and cervical vertebral maturation (CVM) methods were used to visually examine the radiographs and Spearman's correlation analysis was used to identify differences between the two groups with regards to CS, DA and CA. There was a significant positive correlation between DA and CA in both groups and the mean CA-DA difference was significantly higher in children with UCLP when compared to controls (0.319 vs. 0.003, p < 0.05). A significant delay in tooth development was detected in UCLP children from 10 to 12 years-of-age. Both the UCLP and control groups showed high correlations between CS and DA. Calcification stage D appeared only before CS3; however, from CS5 to 6, all teeth have almost completed their maturation phase. Chinese male UCLP patients are likely to experience delayed tooth development compared to healthy controls, especially during the fast-growing period. Evaluating the stages of tooth mineralization could represent a rapid method to assess growth potential.

3D evaluation of the maxillary sinus volumes in patients with bilateral cleft lip and palate.

One of the most common congenital anomalies of the head and neck region is a cleft lip and palate. This retrospective case-control research aimed to compare the maxillary sinus volumes in individuals with bilateral cleft lip and palate (BCLP) to a non-cleft control group. The study comprised 72 participants, including 36 patients with BCLP and 36 gender and age-matched control subjects. All topographies were obtained utilizing Cone Beam Computed Tomography (CBCT) for diagnostic purposes, and 3D Dolphin software was utilized for sinus segmentation. Volumetric measurements were taken in cubic millimeters. No significant differences were found between the sex and age distributions of both groups. Additionally, there was no statistically significant difference observed between the BCLP group and the control group on the right and left sides (p > 0.05). However, the mean maxillary sinus volumes of BCLP patients (8014.26 ± 2841.03 mm3) were significantly lower than those of the healthy control group (11,085.21 ± 3146.12 mm3) (p < 0.05). The findings of this study suggest that clinicians should be aware of the lower maxillary sinus volumes in BCLP patients when planning surgical interventions. The utilization of CBCT and sinus segmentation allowed for precise measurement of maxillary sinus volumes, contributing to the existing literature on anatomical variations in BCLP patients.

Mandibular condyle volumes are associated with facial asymmetry in patients with cleft lip and palate: A retrospective cohort study.

This study compares condylar volumetric asymmetry and facial asymmetry in patients with cleft lip and/or palate (CLP) and controls. The mandibular condyle is important to facial growth, but its role in facial asymmetry for those with CLP has not been described. Condylar volumes and mandibular asymmetry were retrospectively calculated using Mimics Version 23.0 (Materialise, Leuven, Belgium) from patients with CLP undergoing computed tomography (CT) imaging and a cohort of controls. A total of 101 participants, 60 with CLP and 41 controls, had mean condylar volumetric asymmetry of 16.4 ± 17.4 % (CLP) and 6.0 ± 4.0 % (controls) (p = 0.0002). Patients with CLP who had clinically significant chin deviation (>4 mm) had more asymmetric condyles than those without significant chin deviation (p = 0.003). The chin deviated toward the smaller condyle in patients with facial asymmetry more often than in patients without facial asymmetry (81 % vs. 62 %, p = 0.033). While controls had some degree of condylar asymmetry, it tended to be milder and not associated with facial asymmetry. There is a greater degree of condylar volumetric asymmetry in patients with CLP compared to individuals in the general population. Clinically significant facial asymmetry in CLP is associated with a higher degree of condylar asymmetry, with the facial midline deviating toward the smaller condyle.

Validation and comparison of 2D grading scales and 3D volumetric measurements for outcome assessment of bone-grafted alveolar clefts in children.

BACKGROUND: Several methods have been proposed to assess outcome of bone-grafted alveolar clefts on cone beam computed tomography (CBCT), but so far these methods have not been compared and clinically validated. OBJECTIVES: To validate and compare methods for outcome assessment of bone-grafted clefts with CBCT and provide recommendations for follow-up. METHODS: In this observational follow-up study, two grading scales (Suomalainen; Liu) and the volumetric bone fill (BF) were used to assess the outcome of 23 autogenous bone-grafted unilateral alveolar clefts. The mean age at bone grafting was 9 years. The volumetric BF was assessed in five vertical sections. The bone-grafted cleft outcome was based on a binary coding (success or regraft) on a clinical multidisciplinary expert consensus meeting. Grading scales and volumetric assessment were compared in relation to the bone-grafted cleft outcome (success or regraft). Reliability for the different outcome variables was analyzed with intra-class correlation and by calculating kappa values. LIMITATIONS: The study had a limited sample size. Clinical CBCT acquisitions had a varying tube current and exposure time. RESULTS: Volumetric 3D measurements allowed for outcome assessment of bone-grafted alveolar clefts with high reliability and validity. The two grading scales showed highly reliable outcomes, yet the validity was high for the Suomalainen grading scale but low for the Liu grading scale. CONCLUSIONS: Volumetric 3D measurement as well as the Suomalainen grading can be recommended for outcome assessment of the bone-grafted cleft. Yet, one must always make a patient-specific assessment if there is a need to regraft.

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.

Quantitative assessment of cleft volume and evaluation of cleft's impact on adjacent anatomical structures using CBCT imaging.

OBJECTIVES: To determine pre-operative cleft volume and evaluate cleft´s impact on surrounding anatomical structures in children and adolescents with orofacial clefts using cone bean computed tomography (CBCT) imaging. METHODS: The present retrospective study retrieved CBCT examinations of 68 patients from a previous study. The examinations had been exposed either before (n = 53) or after (n = 15) alveolar bone grafting. Pre-operative volume of cleft was determined, and type and location were evaluated. Morphological changes on the adjacent anatomical structures, including the incisive foramen, the nasal septum and floor, and the inferior turbinate, were assessed. RESULTS: Mean bilateral cleft volume was 0.76 cm3, while mean unilateral cleft volume was 1.08 cm3; the difference was significant (p < 0.001). Variation in cleft volume, however, was large. The incisive foramen was not visible in the majority of cases with bilateral clefts (71%); the difference was significant (p = 0.001). In cases with unilateral clefts, the nasal septum in 87% was curved towards the cleft or graft side. Also, the mean size of the widest part of the inferior turbinate was 8.8 mm on the cleft or graft side and 10.4 mm on the non-cleft side. The difference was significant (p < 0.001). CONCLUSIONS: When required, CBCT is a feasible method for quantitatively illustrating alveolar clefts and their impact on the morphological development of surrounding structures. Variation in cleft volume was large.

Comprehensive repair of the alveolar cleft using cortical and cancellous bone layers: A retrospective study.

To retrospectively review the clinical effect of comprehensive treatment of alveolar cleft (CTAC) using the mandible as the bone source. Patients with alveolar clefts who met the inclusion criteria were subjected to a CTAC protocol that included the following: (1) preoperative orthodontic treatment for creating good soft-tissue conditions; (2) 'area-like grafting' with subperiosteal osteogenic chin bone instead of cartilaginous osteogenic iliac bone; (3) simulation of normal bone anatomy via a sandwich-like bone graft consisting of 'cortical bone + cancellous bone + cortical bone'; and (4) strong internal fixation to ensure initial bone block stability. At 6 months postoperatively, the titanium plate was removed and cone-beam computed tomography was performed to evaluate the surgical results. A total of 54 patients underwent treatment with the CTAC protocol. The average age at the initial operation was 10.3 ± 2.1 years, and the average hospital stay was 2.8 ± 0.6 days. At 6 months postoperatively, 49 patients (90.7%) showed good clinical results. The transplanted bone block formed a 'cortical bone + cancellous bone + cortical bone' structure similar to that of the normal jawbone. A mature bone bridge formed, and the impacted permanent teeth continued to erupt and enter the bone graft area. CTAC is a comprehensive restorative solution for alveolar cleft repair that integrates multiple concepts, including orthodontics, embryology, anatomy, and improvements to surgical methods. The method is easy to perform, causes little surgical trauma, and shows a stable success rate, and is thus worth promoting.

Objective and subjective evaluation of the efficacy of trans-sutural distraction osteogenesis: A correlation analysis between computed tomography measurements and FACE-Q scores.

Previous studies on the efficacy of trans-sutural distraction osteogenesis (TSDO) to treat midface hypoplasia caused by cleft lip and palate (CLP) have mainly focused on objective measurements while ignoring the subjective feelings of patients. This study aimed to analyse the changes in and correlation between computed tomography (CT) measurements and FACE-Q scores in patients who underwent TSDO by performing a comprehensive evaluation from both objective and subjective perspectives. This retrospective study included 25 patients with an average age of 10.7 years who had midface hypoplasia caused by CLP and underwent TSDO between August 2018 and December 2022. The average follow-up time was 18.8 ± 7.7 months. Facial morphology and CT measurements, including A-CR, N-A⊥HR, the SNA angle and the L-ZA, indicated significant improvements in midface concavity (all p < 0.0001). All FACE-Q scores (except for facial function) exhibited a significant increase. The ΔA-CR, ΔN-A⊥HR, and ΔSNA angle were strongly correlated with specific aspects of the FACE-Q-Appearance items, including the ΔFACE-Q-Appearance of the cheeks (all p < 0.0001), the ΔFACE-Q-Appearance of the face (all p < 0.0001), the ΔFACE-Q-Appearance of the jaws (all p < 0.01), the ΔSatisfaction with decision (all p < 0.0001) and the ΔSatisfaction with outcome (all p < 0.001). However, the ΔA-CR, ΔN-A⊥HR, and ΔSNA were weakly correlated with other FACE-Q-Health-related quality of life and function items. These findings suggest that both CT findings and FACE-Q scores have their own emphases and advantages. It is necessary to establish an integrated curative effect evaluation model that combines FACE-Q scores with CT measurements to evaluate both the physical health and psychological status of patients.

The evaluation of superior semicircular canal in patients with unilateral cleft lip and palate using CBCT.

OBJECTIVES: The present study aims to evaluate the thickness and radiological patterns of the superior semicircular canal (SSC) in patients with unilateral cleft lip and palate (CL/P). METHODS: Cone beam computed tomography (CBCT) images of the patients were evaluated in axial and Pöschl planes. CBCT images of 84 patients with unilateral CL/P and 168 healthy individual controls were included in the study. Three study groups were established: the CS-CL/P group (cleft side temporal bones of the CL/P patients), NCS-CL/P (non-cleft side temporal bones of the CL/P patients) and the control group. The radiological patterns of SSCs were categorized as dehiscence, papyraceous, normal, pneumatised and thick. The minimum bone thickness of SSC was measured. RESULTS: It was found that the CS-CL/P group had a higher prevalence for SSCD compared to both the NCS-CL/P group and the control group. CS-CL/P group had a higher prevalence of dehiscence type and papyraceous type compared to the control group. The SSC thickness on the CS-CL/P patients was thinner than the NCS-CL/P patients and the control group sides (p = 0.033 and p < 0.001, respectively). CONCLUSIONS: The mean thickness of SSC was found significantly lower in the CS-CL/P group compared to both the NCS-CL/P group and the control group. The elevated prevalence of dehiscence and papyraceous types in the CS-C/LP group compared to the control group implies that the presence of a cleft may be a predisposing factor for these types.

Surgery-First Orthognathic Approach for Correction of Dentofacial Deformity in Unilateral Cleft Lip and Palate.

BACKGROUND: Although several studies have reported the advantages of the surgery-first approach for orthognathic correction of class III deformity, there is no report of the success of this approach for patients with cleft lip and palate. Therefore, the purpose of this study was to evaluate the stability and outcome of bimaxillary surgery for cleft-related dentofacial deformity using a surgery-first approach. METHODS: Forty-one patients with unilateral cleft lip and palate who consecutively underwent Le Fort I and bilateral sagittal split osteotomies for skeletal class III deformity were included. Cone-beam computed tomographic scans before surgery, 1 week after surgery, and after orthodontic treatment were used to measure the surgical and postsurgical changes in jaw position by landmarks, and outcomes of jaw protrusion and relation, incisor angle and occlusion, and menton deviation after treatment. Self-report questionnaires regarding satisfaction with overall appearance of the face and seven facial regions were administered after treatment. RESULTS: A clinically insignificant relapse was found in the maxilla (<1 mm) and mandible (<2 mm). There was a significant improvement in the jaw protrusion and relation, incisor angle and occlusion, and menton deviation. Responses from the self-report questionnaires completed after treatment indicated that patient satisfaction was high. CONCLUSION: These findings demonstrate surgical-orthodontic treatment with a surgery-first approach can successfully improve cleft-related dentofacial deformity in patients with unilateral cleft lip and palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

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.

Long-term radiographic and periodontal evaluations of the bone-grafted alveolar cleft region in young adults born with a UCLP.

BACKGROUND: Studies addressing the periodontal health of the teeth surrounding the bone-grafted cleft in patients born with unilateral cleft lip and palate disagree on whether periodontal health is compromised. OBJECTIVES: To determine periodontal health differences between the cleft and the non-cleft sides nearly a decade after secondary alveolar bone grafting. METHODS: This prospective, controlled (split-mouth design) study comprised an intraoral apical radiographic and a periodontal examination of 40 consecutive patients from one centre (n = 26 males) who had undergone bone grafting at mean age of 10.2 years (±1.6). Probing pocket depth, gingival index, gingival recession, and radiographic bone support were assessed. RESULTS: No significant difference occurred in probing pocket depth between teeth at cleft and non-cleft sites (OR 1.8, P = .488). Gingival recession was present at 6.6% of all examined sites on the cleft side and at 1.7% on the non-cleft side (OR 17.3, P < .001). Gingival recession occurred most often on the buccal and disto-buccal surfaces of the central incisor on the cleft side. The gingival index was significantly higher on the cleft side (OR 8.0, P < .001). The Bergland index was I or II in most patients (87%). LIMITATIONS: Recruitment of eligible patients was lengthy. CONCLUSION: The teeth on the cleft side had high levels of gingival inflammation. Few pathological gingival pockets, however, were found. Shallow gingival recessions frequently occurred around the central incisor on the cleft side. Teeth in the bone-grafted cleft region generally had good bone support.

Idiopathic Fistula in an Unrepaired Submucous Cleft Palate.

Palatal fistulae are a recognised complication in individuals who have undergone surgical repair of a cleft palate, however, congenital or idiopathic palatal fistulae are rare. This report discusses the presentation and treatment of a 16-year-old female with a submucous cleft palate, who presented with a recent onset change in speech and evidence of a new palatal fistula. There was no history of recent infection or known trauma, and the patient had not undergone any previous palatal surgery. This report discusses the clinical presentation, recommended management and relevant literature for this rare phenomenon.

Prevalence and Features of Maxillary Sinus Septa in Patients with Cleft Lip and Palate: Cone Beam Computed Tomography Imaging Technique.

OBJECTIVE: The development of the maxillary sinus is different in patients with cleft lip and palate (CLP) compared to non-CLP individuals. To investigate the prevalence and features of maxillary sinus septa (MSS) in patients with CLP in comparison with the non-CLP population. DESIGN: Retrospective study. INTERVENTION: Cone beam computed tomography (CBCT) evaluation. SETTING: CLP center in Shiraz faculty of dentistry, Iran. PATIENTS: A total 306 sinuses (88 cleft and 218 noncleft) on 153 images (CLP group: n = 66; control group: n = 87) were examined to determine the prevalence of septa and characterize them. MAIN OUTCOME MEASURES: Sinus septa were characterized according to height, orientation, angle, origin, and location. The chi-square test, Mann-Whitney U test, and Fisher's exact test were used for statistical analysis. RESULTS: The prevalence of septa was 28.9% and 32.1% in the CLP and control groups, respectively. No significant difference was found between the study groups in terms of prevalence, location, and orientation of MSS. The average height and angle of septa were significantly higher in the control group compared to the CLP group. Inferior origin was significantly more prevalent in the control group than in the CLP group (P = .004). CONCLUSION: There was no difference in the prevalence of MSS between patients with CLP and non-CLP individuals. However, certain features of the septa were different in patients with CLP.

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.

Does cleft lip and palate affect the severity of malocclusion?

OBJECTIVE: The purpose of this study was to evaluate the 3D anatomical features of unilateral (UCLP) and bilateral (BCLP) complete cleft lip and palate with those of skeletal Class III dentofacial deformities. MATERIALS AND METHODS: In total, 92 patients were divided into cleft and noncleft groups. The cleft group comprised 29 patients with UCLP and 17 patients with BCLP. The noncleft group comprised 46 patients with Class III dentofacial deformities. 3D anatomical landmarks were identified and the corresponding measurements were made on the cone-beam computed tomography (CBCT). RESULTS: The differences between the affected and unaffected sides of the patients with UCLP were nonsignificant. The differences between the patients with UCLP and BCLP were nonsignificant except for the SNA angle. Significant differences between the patients with clefts and Class III malocclusion were identified for the SNA, A-N perpendicular, and A-N Pog line, indicating that the maxillae of the patients in the cleft group were more retrognathic and micrognathic. Relative to the noncleft group patients, the cleft group patients had a significantly smaller ramus height. CONCLUSION: The affected and unaffected sides of the patients with UCLP did not exhibit significant differences. The maxillae of the patients with UCLP were significantly more retrognathic than those of the patients with BCLP. The maxillae and mandibles of the patients in the cleft group were more micrognathic and retropositioned relative to those of the noncleft Class III patients. CLINICAL RELEVANCE: The maxillary and mandibular findings indicated greater deficiencies in the patients with UCLP or BCLP than in those with skeletal Class III malocclusion. Appropriate surgical design should be administered.

[Assessment of the structural features of the hard palate in endonasal surgery]. / Otsenka anatomii tverdogo neba v khirurgii peregorodki nosa.

Data on the features of the anatomical structure of the hard palate are little described in the scientific literature, and therefore are not taken into account when planning surgical treatment. One of the intraoperative complications during intervention on the lower part of the nasal septum is perforation of the bottom of the nasal cavity, which can develop during a christotomy. This complication mainly depends on the features of the anatomical structure of the hard palate. OBJECTIVE: To study the anatomical structure of the hard palate from the point of view of rhinosurgery, using vector analysis of multispiral computed tomography (MSCT), and to establish anatomical features that should be taken into account when performing surgical interventions on the nasal septum. MATERIAL AND METHODS: 107 patients (30 men, 77 women) were examined without congenital cleft palate and surgical interventions on the structures of the nasal cavity and hard palate. All patients underwent MSCT of the nose and paranasal sinuses (PNS) followed by multiplanar image reconstruction. The key point relative to which the measurements were carried out was the posterior wall of the incisor canal from the side of the nasal cavity. The line corresponding to the bottom of the nasal cavity was chosen as the main vector. In the work, measurements of the thickness of the hard palate (THP) at the level of the palatal suture and the width of the palatal suture (WPS) were carried out. RESULTS: Statistical analysis of the obtained results showed that the THP is 1.74 mm [min 0.28; max 6.46], the WPS is 0.9 mm [min 0.2; max 2.51] (conditional norm). In 19 patients (17.8%), the THP was 0.82 mm, in 2 patients (1.9%) - 0.2 mm. In 3 patients (2.8%), the WPS was equal to 2.5 mm. CONCLUSION: Thus, the data obtained by us indicate that the surgical anatomy of the hard palate is characterized by significant variability, while in some patients the THP can be reduced by 8.8 times, and the WPS increased by 2.7 times compared to normal values. Such anatomical features of the structure of the hard palate should be taken into account when planning septoplasty, since this contingent of patients has an increased risk of developing iatrogenic perforation of the nasal floor during surgical intervention on the lower floor of the nasal septum.

Prenatal diagnosis of Hartsfield syndrome with a novel genetic variant.

A G2P0, 24-year-old woman presented at 17 weeks 3 days gestation for a fetal anatomy scan. Ultrasound identified bilateral upper and lower extremity ectrodactyly, semilobar holoprosencephaly, midface hypoplasia, and cleft lip and palate. Amniocentesis for a chromosome microarray demonstrated no significant copy number changes. Whole exome sequencing was subsequently completed, which revealed a de novo, likely pathogenic variant in FGFR1, c.2044G>A (D682N), consistent with FGFR1-related Hartsfield syndrome. This case highlights the first presumed molecularly confirmed prenatal diagnosis of Hartsfield syndrome and identifies a new pathogenic variant.