Cleft Type Research Articles

Genome-wide analysis of copy-number variation in humans with cleft lip and/or cleft palate identifies COBLL1, RIC1, and ARHGEF38 as clefting genes

Cleft lip with or without cleft palate (CL/P) is a common birth defect with a complex, heterogeneous etiology. It is well established that common and rare sequence variants contribute to the formation of CL/P, but the contribution of copy-number variants (CNVs) to cleft formation remains relatively understudied. To fill this knowledge gap, we conducted a large-scale comparative analysis of genome-wide CNV profiles of 869 individuals from the Philippines and 233 individuals of European ancestry with CL/P with three primary goals: first, to evaluate whether differences in CNV number, amount of genomic content, or amount of coding genomic content existed within clefting subtypes; second, to assess whether CNVs in our cohort overlapped with known Mendelian clefting loci; and third, to identify unestablished Mendelian clefting genes. Significant differences in CNVs across cleft types or in individuals with non-syndromic versus syndromic clefts were not observed; however, several CNVs in our cohort overlapped with known syndromic and non-syndromic Mendelian clefting loci. Moreover, employing a filtering strategy relying on population genetics data that rare variants are on the whole more deleterious than common variants, we identify several CNV-associated gene losses likely driving non-syndromic clefting phenotypes. By prioritizing genes deleted at a rare frequency across multiple individuals with clefts yet enriched in our cohort of individuals with clefts compared to control subjects, we identify COBLL1, RIC1, and ARHGEF38 as clefting genes. CRISPR-Cas9 mutagenesis of these genes in Xenopus laevis and Danio rerio yielded craniofacial dysmorphologies, including clefts analogous to those seen in human clefting disorders.

The Incidence and Outcomes for Children with Cleft Palate and/or Lip and CHARGE Syndrome.

Published literature on children with cleft palate and/or lip (CP + /-L) and CHARGE syndrome (CS) is limited. This study investigated cleft characteristics including surgery, and feeding and communication outcomes in children identified with CP + /-L and CS. Retrospective cross-sectional review. Regional Referral Centre for Paediatric Cleft Surgery. All children diagnosed with CP + /-L and CS (based on clinical features and/or CHD7 mutation testing) between 1989-2019. Cleft type, timing of CP + /-L repair, reasons for 'delayed' repair, feeding methods and communication modality. Twenty-two children with CP + /-L and CS were identified. Cleft sub-types (%) were: Eleven (50%) had bilateral cleft lip and palate (BCLP), six (27%) had unilateral cleft lip and palate (UCLP) and five (23%) had cleft palate (CP). Cleft repair was delayed compared to protocol care for non-syndromic children with CP + /-L. Median age for lip repair + /- vomerine flap was 9 months (range 4-22 months), and palate repair was 21 months (range 11-40 months). Median age for isolated CP repair was 13 months (range 7-23). Surgery for cardiac anomalies (36%) before cleft repair, and (59%) were classed as having severe systemic disease at the time of cleft surgery. Only 27% of the children in this study had both full oral feeding and verbal communication. Children with CP + /-L and CS had severe cleft types and complex medical problems leading to delayed cleft surgery. Feeding and speech outcomes were better in the children aged over ten years.

A Cross-Sectional Study of the Nutritional Status of Infants with Orofacial Clefts in the First 6 Months of Life

To estimate nutritional status in a large cohort of infants with orofacial clefts in the US, overall and by cleft type from birth to 6months of age. We conducted a cross-sectional study in infants with orofacial clefts by examining growth by month between birth and 6months of age. Infants with at least one weight measurement at a single US regional tertiary care pediatric hospital with an interdisciplinary cleft team between 2010 and 2020 were included. We calculated the average weight-for-age z scores and weight-for-length z scores. We calculated the proportion of infants underweight and wasting with z scores below -2 SDs monthly from birth to 6months of age. We used t tests to compare the distribution of weight for age z-score and weight for length z-score among children with orofacial clefts to a normal distribution. We included 883 infants with orofacial clefts. Compared with expected proportion of underweight infants (2.3%), a larger proportion of infants with orofacial clefts were underweight between birth and 1month (10.6%), peaking between 2 and 3months (27.1%), and remaining high between 5 and 6months (16.3%). Compared with the expected proportion of infants with wasting (2.3%), a higher proportion of infants with orofacial clefts experienced wasting between birth and 1month (7.3%), peaking between 2 and 3months (12.8%), and remaining high between 5 and 6months (5.3%). Similar findings were observed for all cleft types and regardless of comorbidities. A substantial proportion of infants with orofacial clefts compared with normative peers have malnutrition in the first 6months of life in the US.

Immunohistochemical Evaluation of BARX1, DLX4, FOXE1, HOXB3, and MSX2 in Nonsyndromic Cleft Affected Tissue.

Nonsyndromic craniofacial clefts are relatively common congenital malformations which could create a significant negative effect on the health status and life quality of affected individuals within the pediatric population. Multiple cleft candidate genes and their coded proteins have been described with their possible involvement during cleft formation. Some of these proteins like Homeobox Protein BarH-like 1 (BARX1), Distal-Less Homeobox 4 (DLX4), Forkhead Box E1 (FOXE1), Homeobox Protein Hox-B3 (HOXB3), and Muscle Segment Homeobox 2 (MSX2) have been associated with the formation of craniofacial clefts. Understanding the pathogenetic mechanisms of nonsyndromic craniofacial cleft formation could provide a better knowledge in cleft management and could be a possible basis for development and improvement of cleft treatment options. This study investigates the presence of BARX1, DLX4, FOXE1, HOXB3, and MSX2 positive cells by using immunohistochemistry in different types of cleft-affected tissue while determining their possible connection with cleft pathogenesis process. Craniofacial cleft tissue material was obtained during cleft-correcting surgery from patients with nonsyndromic craniofacial cleft diagnosis. Tissue material was gathered from patients who had unilateral cleft lip (n=36), bilateral cleft lip (n=13), and cleft palate (n=26). Control group (n=7) tissue material was received from individuals without any craniofacial clefts. The number of factor positive cells in the control group and patient group tissue was evaluated by using the semiquantitative counting method. Data was evaluated with the use of nonparametric statistical methods. Statistically significant differences were identified between the number of BARX1, FOXE1, HOXB3, and MSX2-containing cells in controls and cleft patient groups but no statistically significant difference was found for DLX4. Statistically significant correlations between the evaluated factors were also notified in cleft patient groups. HOXB3 could be more associated with morphopathogenesis of unilateral cleft lip during postnatal course of the disorder. FOXE1 and BARX1 could be involved with both unilateral and bilateral cleft lip morphopathogenesis. The persistence of MSX2 in all evaluated cleft types could indicate its possible interaction within multiple cleft types. DLX4 most likely is not involved with postnatal cleft morphopathogenesis process.

How Early Can We Predict the Need for VPI Surgery?

This retrospective study reviewed documentation of children with moderate-to-severe VPI at age 3 years who did not undergo VPI surgery until age 5 years. Based on the national cleft register, 959 patients with syndromic and nonsyndromic CP were treated by the craniofacial team at Helsinki University Hospital, Finland between 2000 and 2014. Eighty-six patients fulfilled the study inclusion criteria. The speech pathologist evaluated velopharyngeal function at age 3, 5, and 8 years. Of the 86 children presenting with moderate-to-severe VPI at age 3 years, 94% still had moderate-to-severe VPI at age 5 years, even though speech therapy was offered to 77%. Of those whose velopharyngeal function improved by age 5 years, function regressed to incompetent over time. Overall, 93% underwent VPI surgery and 82% underwent VPI surgery between ages 5 and 8 years. Only 23% at age 8 years still had moderate-to-severe VPI. Speech therapy alone did not improve VPI speech characteristics. Moderate-to-severe VPI did not improve from 3 to 5 years or improved but subsequently relapsed. This suggests that the decision for VPI surgery can be made for children aged 3 years with moderate-to-severe VPI.

Symmetry of the Vermillion Height after Modified Rotation-Advancement Cheiloplasty.

(1) Background: This study aimed to determine the postoperative vermillion symmetry between the cleft and non-cleft sides of patients with unilateral cleft lip during the early and late postoperative periods. (2) Methods: 57 patients with complete and 38 with incomplete unilateral cleft lips operated on between 2010 and 2014 were retrospectively evaluated within 1 month (T1), 9 months to 1 ½ years (T2), and more than 4 years (T3). Vermilion heights of the cleft and non-cleft sides were measured from frontal photographs. The Cleft Lip Component Symmetry Index (CLCSI) was used to determine the symmetry of the cleft and non-cleft sides and was then analyzed. (3) Results: Among the 95 patients studied, vermilion height was excessive on the cleft side throughout the three time periods. There was a significant increase in CLCSI from T1 to T2 for both complete and incomplete types, and a significant increase from T1 to T3 only in the incomplete group and no difference from T2 to T3 for both the groups. (4) Conclusions: Even with efforts to obtain a symmetric vermilion height during the primary cheiloplasty, vermilion height excess was noted with time in complete and incomplete cleft types. Secondary revisional vermilion surgery may be performed to achieve symmetry.

Palate Re-Repair for Velopharyngeal Insufficiency Treatment: A Long-Term Auditory-Perceptual Assessment of Speech

Purpose:The purpose of this study was to assess the effectiveness of the palate re-repair by the auditory-perceptual assessment of speech.Method:Nonsyndromic individuals with operated cleft palate ± lip and velopharyngeal insufficiency (VPI), aged 6–22 years (10 men and 10 women), were referred to secondary palate surgery by the Sommerlad technique. Speech characteristics related to velopharyngeal function (VPF) were analyzed by auditory-perceptual assessment using digital audio recording 5 days before and 13 months after the palate re-repair, on average. Hypernasality, global impression of VPF, and active speech symptoms were scored and blindly rated by three experienced speech-language pathologists before and after surgery. The effect of age and the type of cleft on the outcomes was also analyzed.Results:Surgical success, interpreted as the elimination or reduction of hypernasality, was observed in 13 of the 20 patients analyzed (65%). Adequate nasality was observed in 55% of the patients, and the degree of hypernasality decreased from moderate to mild in 10% of the patients. Postoperative normal nasality was only seen among the children (73%). There was a tendency for better outcomes in patients with unilateral cleft lip and palate.Conclusions:The auditory-perceptual assessment of speech showed that re-repairs were effective in eliminating hypernasality and normalizing VPF in the majority of children analyzed, but not among the adults. In view of these results, the Sommerlad surgery should be prioritized in children as a secondary intervention in order to eliminate VPI.

Bikol clefts and topics and the Austronesian extraction restriction

Many Austronesian languages exhibit an extraction restriction whereby only one particular DP—the “pivot” argument, the choice of which is reflected by morphology on the verb—can be Ā-extracted. We show that such extraction restrictions can vary between different Ā-constructions in Bikol: local clefting is limited to the pivot, whereas topicalization can target pivots and non-pivot agents but not non-pivot themes of transitive verbs. Following the phase-theoretic, locality-based approach to such extraction asymmetries in related Austronesian languages, we propose that clefting and topicalization differ in the featural specifications of their probes, with clefting necessarily targeting the closest DP and topicalization simply seeking the closest topic constituent. Evidence for this approach comes from the behavior of long-distance clefting, which may target certain non-pivot arguments and involve gaps or resumptive pronouns. The inventory of different long-distance cleft types is explained by the possibility of embedded topicalization and hanging topic left dislocation feeding higher clefting. Our study strengthens the view that the classic Austronesian pivot-only extraction restriction is best characterized in terms of syntactic locality, rather than as a restriction on the grammatical function or morphological case of movement targets.

Craniofacial and three-dimensional palatal analysis in cleft lip and palate patients treated in Spain

Growth alterations have been described in patients operated on for oral clefts. The purpose of this work was to analyze the craniofacial and palate morphology and dimensions of young adults operated on for oral clefts in early childhood in Spain. Eighty-three patients from eight different hospitals were divided into four groups based on their type of cleft: cleft lip (CL, n = 6), unilateral cleft lip and palate (UCLP, n = 37), bilateral cleft lip and palate (BCLP, n = 16), and cleft palate only (CPO, n = 24). A control group was formed of 71 individuals. Three-dimensional (3D) digital models were obtained from all groups with an intraoral scanner, together with cephalometries and frontal, lateral, and submental facial photographs. Measurements were obtained and analyzed statistically. Our results showed craniofacial alterations in the BCLP, UCLP, and CPO groups with an influence on the palate, maxilla, and mandible and a direct impact on facial appearance. This effect was more severe in the BCLP group. Measurements in the CL group were similar to those in the control group. Cleft characteristics and cleft type seem to be the main determining factors of long-term craniofacial growth alterations in these patients. Prospective research is needed to clearly delineate the effects of different treatments on the craniofacial appearance of adult cleft patients.

Characterization of hearing status in children under 3 years of age with cleft palate.

To describe the hearing thresholds of children under 3 years of age with cleft palate with or without cleft lip. This retrospective study from a tertiary care children's hospital included children with cleft palate±cleft lip born between January 01, 2008, and December 31, 2015. All patients who had the initial palate repair at our institution and at least one behavioral audiogram were included. Behavioral audiograms collected either in the sound field or under insert earphones showed results from the better hearing ear. We collected demographic information, cleft type, parent report of newborn hearing screen, syndrome diagnosis, comorbid conditions, and dates of tympanostomy tube placement and palatoplasty. The main outcome measure was hearing status as measured by behavioral audiograms, and, for a subset of patients, brainstem auditory evoked responses (BAER). Results were grouped by test type (behavioral vs BAER) and characteristics at time of testing (pre- or post-palatoplasty, with or without tympanostomy tube placement) to evaluate the range of severity of hearing loss. Three hundred and sixty patients were included in the cohort. The cohort had two groups: Group 1 included 37 patients who had BAER results prior to surgery and behavioral audiogram results post-surgery; Group 2 included 322 patients who had behavioral audiograms pre-surgery (n=206) and/or post-surgery (n=215). The median age of patients at time of BAER in Group 1 was 3.00 months [IQR: 1.00,3.75]; pre-surgery BAER results showed mild (45.9%, n=17) or moderate hearing loss (29.7%, n=11) by this age. Patients in Group 2 with pre-surgery behavioral audiograms had a median age of 12.00 months [IQR: 11.00, 14.00] at time of audiogram. Most patients had a mild (33.5%, n=69) or moderate hearing loss (31.1%, n=64). Post-surgery, patients had a median age of 13.00 months [IQR: 11.00, 15.00] at time of hearing assessment and typically had normal hearing (86.0%, n=185). In most patients, hearing improved after palate repair with concurrent tympanostomy tube placement. Most patients with cleft palate±cleft lip had evidence of mild or moderate hearing loss prior to cleft palate repair, and hearing loss was evident from early infancy. Hearing improved after concurrent tympanostomy tube placement and palatoplasty. Further research is needed to understand the clinical significance of untreated transient hearing loss during the first year of life in this patient population.

Clinical Epidemiological Study of Children with Cleft Lip and Palate

Aim: To determine the frequency of different types of cleft lip and palate and to investigate the possible relation of some risk factors to cleft lip and palate. Study design: Descriptive study. Place and duration of study: Peshawar Dental College, Peshawar Pakistan from 1st August 2017 to 31st January 2019. Methodology: One hundred and seven patients with congenital cleft lip and cleft palate deformities, either gender and age ranged from 1 day to 720 days were enrolled. Cleft types were categorized as bilateral cleft lip and palate (BCLP), left cleft lip and palate (LCLP), right cleft lip and palate (RCLP), cleft alveolus (CLA) and bilateral incomplete cleft (BICLP). Results: There were 73(68.2%) males and 34(31.8%) females. The highest number of patients from Peshawar 30(28%) followed by Swat 21(19.8%) and Charssada by 11(10.3%). Forty two (39.3%) patients’ parents had consanguineous marriages. There were 89(83.2%) of mother haven't used folic acid during their pregnancy. Forty 40(37.4%) cases had a positive family history of cleft deformities. Most common systemic disease was anemia 25(23.2). Eighty five (79.4%) of cleft lip and palate patients were belonging to poor class family. Conclusion: The households of cleft lip and palate children demonstrated low socioeconomic status. The incidence of cleft lip and palate should be reduced to enhance the conditions of women during pregnancy by maintaining a balanced diet and giving them folic acid. Keywords: Epidemiology, Children, Cleft lip, Palate, Risk factors

Influence of Primary Palatal Surgery on Craniofacial Morphology in Patients with Cleft Palate Only (CPO)-Systematic Review with Meta-Analysis.

Cleft palate only (CPO) is the second most prevalent cleft type. Both the cleft and palatal scarring may affect craniofacial growth. The aim of this systematic review was to summarize scientific evidence on effect of palatal surgery on craniofacial morphology in CPO. A search was conducted in PubMed, PMC, WoS, Scopus, Embase, using the keywords: "cleft palate" AND ("craniofacial morphology" OR "cephalometric analysis") NOT "lip" with inclusion and exclusion criteria ensuring confident, direct comparison between study groups. The quality assessment was performed with Arrive's scale for radiologic examinations. Of 713 potential articles, 19 were subjected to qualitative analysis and 17 to meta-analysis, which confirmed reduced SNA in unoperated CPO versus non-cleft individuals. No scientific evidence was found directly assessing the effect of surgery on craniofacial morphology. The negative effect of palatal surgery was seen indirectly: in treated CPO versus non-cleft, the size effect of SNA is bigger than in untreated CPO versus non-cleft. A high heterogeneity came from a few non-European publications. CPO is associated with sagittal maxillary deficiency resulting both from the cleft and from primary surgery, disregarding cleft severity in operated CPO patients. Ethnic differences influence craniofacial morphology in CPO. This research received no external funding. Study protocol number in PROSPERO database: CRD42021268957.

Early Cleft Palate Repair by a Modified Technique Without Relaxing Incisions.

This study sought to evaluate a modified palatoplasty technique (MPT) concerning the postoperative outcomes and associated influencing factors. A retrospective cohort study. One hundred forty-three consecutive patients with non-syndromic cleft palate, who received MPT before one year of age within an oral and maxillofacial surgery department of a university-affiliated tertiary hospital between 2011-2017, were reviewed. The postoperative wound healing and velopharyngeal function (VPF) were the primary outcome measures. The sex, age at surgery, cleft type, cleft width, palatal width, soft palate length, pharyngeal cavity depth, and operation duration were preselected as influencing factors. Univariate and multivariate analyses were conducted. The mean age at surgery was 9 ± 1.31 months (5-11), and the average cleft width was 9.03 ± 2.41 mm (4-15). The rate of incomplete cleft palate was 84.6% while the complete cleft palate was 15.4%. Complete wound healing was reported in 96.5% while the others (3.5%) had persistent oronasal fistula. About 90.2% of cases have shown normal velopharyngeal function whereas the others (9.8%) had sustained velopharyngeal insufficiency. The wound healing appeared to be significantly impacted by cleft width and pharyngeal cavity depth (P = .015 and 0.049, respectively). However, none of the factors had a significant association with VPF. The present modified palatoplasty technique has obtained a low fistula rate and appropriate speech outcome. Therefore, this MPT could be promoted for early repairing cleft palate of different severities.

Relationship between cleft palate width and otitis media.

To investigate the relationship between cleft width and otitis media (OM) and to determine whether a wide cleft palate (CP) is a risk factor of the incidence, type, amount of middle ear effusion, and prolonged morbidity in OM. Retrospective cohort study. Children with CP who underwent palatoplasty between 2014 and 2018 were analyzed. Cleft width was measured at palatoplasty. The incidence of otitis media with effusion (OME) and acute otitis media (AOM), the type and amount of middle ear effusion, and OME duration and age at resolution were assessed in relation to cleft width. One hundred eighteen children were included. The CP types were Veau I in 16, II in 35, III in 48, and IV in 19 patients. The incidence of OME and AOM before palatoplasty was 83.1% and 49.2%, respectively. Cleft width did not differ significantly between patients with or without OME but was significantly greater in those with, than in those without, AOM (p < .001), in those with mucoid, than in those with serous, effusion (p=.012), and in those with complete, than in those with partial, effusion (p=.01). Regardless of cleft width or type, OME persisted for a median duration of 50 months. Cleft width was significantly associated with the incidence of AOM and the type and amount of middle ear effusion before palatoplasty. However, it was not significantly related to the incidence, age at resolution, or duration of OME. Regardless of cleft width or type, OM in children with CP requires long-term follow-up. Level of Evidence: 2b.

Evaluation of the Nasopalatine Canal of Patients With and Without Cleft Lip and Palate in CBCT Exams.

To identify and compare the morphology and dimensions of the nasopalatine canal (NPC) of individuals with and without cleft lip and palate using Conical Beam Computed Tomography (CBCT) images and to relate the results to the type of cleft and tooth absences in the region. This is a cross-sectional, analytical study with a retrospective sample. 100 CBCT exams from patients with cleft lip and palate and 100 CBCT exams from patients without cleft lip and palate were used. The NPC was evaluated for its morphology and measurements in the coronal, sagittal, and axial reformatting of CBCT exams. It was obtained as a result that in the group of patients with a cleft, the shapes of funnel, banana, and needle of the NPC were more frequent than in the control group. Patients with cleft lip and palate had significantly a larger nasopalatine foramen and a larger NPC diameter, besides a significantly shorter NPC, compared to controls. In both groups, edentulous patients had less anterior maxillary bone thickness, when compared to patients with maxillary anterior teeth. Patients with clefts had a bone thickness buccal to the NPC less than in the control group. This study confirms the anatomical variability of NPC in patients with cleft lip and palate. This finding reinforces the need for thorough surgical planning of the anterior region of the maxilla, in CBCT exams, by the dentist.

Speech Outcomes following Mandibular Distraction Osteogenesis for Robin Sequence: Midchildhood Results.

The aim of this study was to compare midchildhood speech outcomes in patients with nonsyndromic Robin sequence with cleft palate (RSCP) treated with mandibular distraction osteogenesis (MDO) to patients with nonsyndromic Veau class I and Veau class II cleft palate (CP). The authors performed a retrospective review of patients with nonsyndromic Robin sequence from 2000 to 2017, comparing those who underwent MDO to patients with nonsyndromic CP. Demographics, operative details, length of hospital stay, complications, and Pittsburgh Weighted Speech Scale scores were collected. Thirty-three patients met inclusion criteria in the MDO group with 127 patients as controls. Despite similar median age (RSCP, 4.5 years; CP only, 4.6 years) and Veau cleft type at early evaluation, there was a significant increase in composite Pittsburgh Weighted Speech Scale score within the MDO cohort ( P ≤ 0.002); specifically, with worse visible nasal emission ( P ≤ 0.007), hypernasality ( P ≤ 0.001), and compensatory articulation ( P ≤ 0.015). However, these differences were not present at age-matched midchildhood evaluation (median, RSCP, 6.5; CP only, 7.1; P ≥ 0.092). Median age-matched follow-up was 6.4 years in the MDO group and 7.1 years in the control group ( P ≥ 0.136). There was also no difference in the rate of secondary speech surgery at midchildhood evaluation ( P ≥ 0.688). The authors' retrospective comparison of speech outcomes in RSCP versus CP only demonstrates no difference in midchildhood speech, conflicting with recent reports. Although patients with Robin sequence treated with MDO had worse visible nasal emission, hypernasality, and compensatory articulation in early childhood, this appears to have resolved in the interim without additional intervention. Longitudinal follow-up is needed to fully understand the speech ramifications of RSCP. Risk, II.

The Sommerlad-Furlow Modified Palatoplasty Technique: Postoperative Complications and Implicating Factors.

To explore the incidence of postoperative complications and investigate the impact of preselected factors on functional and quality of life outcomes following the Sommerlad-Furlow modified palatoplasty technique. Retrospective cohort. A total of 429 patients with cleft palate, who received Sommerlad-Furlow modified technique between 2011-2017 were enrolled. The postoperative complications including oronasal fistula (ONF), velopharyngeal insufficiency (VPI), and inadequate quality of life (QOL) were collected. Data of preselected factors including gender, age at palatoplasty, cleft type, cleft width, palatal width, pharyngeal cavity depth, and operation duration were also collected. Among 429 patients, 40.1% were males whereas 59.9% were females. The mean age at palatoplasty was 1.23 ± 0.69 (0.42-4) years, and the average cleft width was 10.15 ± 2.95 (4-27) mm. The cleft types had recorded rates of about 6.8%, 69.5%, 17.7%, and 6.1% of Veau I, II, III, and IV, respectively. The overall incidence rates of ONF, VPI, and inadequate QOL were 2.3%, 19.4%, and 31.3%, respectively. In both the univariate and multivariate analyses, the cleft type was significantly implicated in ONF formation (p= 0.023 and 0.032, respectively) whereas the velopharyngeal function was impacted by the palatoplasty age (p˂0.001). The receiver operating characteristic curve indicated that age of palatoplasty ≥1.3 years (area under the curve=0.611, p= 0.002) was the cutoff value for predicting the incidence of VPI. The Sommerlad-Furlow modified technique appears to have appropriate postoperative outcomes, even in the wide cleft palate. The older age at palatoplasty has a major impact on the overall postoperative outcomes. 4 Laryngoscope, 133:822-829, 2023.

Correlation of Dental Anomalies with Cleft Type and Gender in Non-Syndromic Oral Cleft Patients: A Cross-Sectional Study.

To investigate the distribution of dental anomalies between non-syndromic orofacial cleft patients and their association with the cleft type and gender. Retrospective cross-sectional study involving examination of intraoral dental records and radiographs. 300 non-syndromic orofacial cleft patients included (161 males and 139 females, mean age: 10.23 ± 2.3 years). Variables analyzed: tooth agenesis, microdontia, and supernumerary teeth. Data analysis: Chi-square and Fisher's exact tests to investigate the correlation between dental anomalies, cleft type, and gender. Agenesis occurred in 66% of the subjects, supernumerary teeth in 19.6%, and microdontia in 18.3%. In females with unilateral left cleft lip and palate (CLP), agenesis was significantly higher compared to males. Both genders presented significant agenesis of maxillary lateral incisors and right central incisor. Significant agenesis of central incisor, canine and second premolar on the upper left side and lower second premolars were observed only in males, with significant microdontia for maxillary lateral incisors and left central incisor. Significant agenesis of maxillary first premolars was seen only in females. Agenesis in maxillary quadrants was significant for patients with unilateral right and left CLP, and bilateral CLP. In mandibular quadrants, agenesis was substantially higher for patients with isolated CP. Results suggest that tooth agenesis and microdontia might not be directly associated with the cleft area's anatomical irregularity but are affected by gender and other genetic factors that regulate the development of the anomaly and the orofacial clefting mutually.

Tessier cleft numbers 3 and 4: Presentation of soft tissue and bony deformities in a select South African population

BackgroundTessier cleft numbers 3 and 4 are rare congenital craniofacial malformations. Tessier cleft number 3 runs from the philtrum of the superior lip, traverses the ala of the nose and ends at the medial canthus of the eye. Tessier cleft number 4 commences between the philtrum and oral commissure in the superior lip, and ascends into the orbit, remaining medial to the infraorbital foramen. The associated clinical manifestation of each cleft type varies amongst patients. This study documented the soft tissue and bony deformities of Tessier cleft numbers 3 and 4 in a select South African population. MethodsThe study group was selected from retrospectively reviewed computed tomography (CT) scan records, which were made available via the repository of the Department of Clinical Anatomy at higher education institution in South Africa. Seven out of fifty CT scans that met the inclusion criteria were assessed and analysed. The patient records associated with the selected scans were reviewed. The soft tissue and bony deformities characteristic of Tessier cleft numbers 3 and 4 were documented [Ethical approval number: BE363/19]. Descriptive statistics were used to summarise the results. ResultsThe bony deformities presented in the cases of this study included alveolar clefts, nasal septum deviation, hypertelorism and maxillary bone defects. The soft tissue deformities included cleft lip, nasolacrimal abnormalities, eye dystopia, anophthalmia and nasal ala defects. ConclusionsThe extent of the associated deformities of each case varied; however, the primarily affected regions remained constant i.e., the orbital, nasal and oral regions. The variation in soft tissue and bony presentation warranted the need to document these clefts. This study will contribute to the anatomical interpretation of Tessier cleft numbers 3 and 4 in South Africa, and aid medical professionals in the evaluation, differentiation, and treatment of such clefts.

Middle Ear Status in Cleft Lip and Palate Patients: A Five-Year Follow-Up.

Introduction:The best strategy to treat otitis media with effusion in cleft lip/palate patients is still under debate. This research aimed to evaluate the otologic outcomes in children at least five years post-repair.Materials and Methods:A retrospective study was conducted on 40 children who underwent palatoplasty between January 1, 2012, and January 1, 2014, at Children’s Medical Center (Tehran, Iran). Patients had intervelar veloplasty under magnification (Sommerlad’s Technique). Based on patients’ charts, their age, gender, cleft type, date of palatoplasty, as well as the date and the frequency of ventilation tube (VT) insertion, were recorded. Furthermore, otomicroscopy, middle ear status, and tympanometry were assessed five years postoperatively.Results:There was no significant difference in middle ear status between children with complete and incomplete cleft palates. The mean age at the time of study and the mean follow-up duration were significantly higher in the normal middle ear group, compared to the abnormal middle ear group (7.7±1.6 vs. 6.8±0.9, P=0.03 and 6±1.15 vs. 5.42±0.9, P=0.04, respectively). Middle ear status was not significantly different between early or late palatoplasty patients. In addition, the frequency and timing of VT insertion were not significantly different between the two groups. Conclusions:Middle ear status improved as patients grew older; however, the age of palatoplasty and the frequency of VT insertion were not significant prognostic factors in patients who underwent intervelar veloplasty under magnification.