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Binder's syndrome is a rare congenital deformity characterized by midface hypoplasia, particularly around the nasomaxillary area. Genetic etiology or developmental failure caused by prenatal exposure to teratological agents has been considered. In this article, we present 3 related rhesus monkeys born with orofacial deformities similar to those found in infants with the Binder phenotype. For the first time, a primate biomodel for this condition is presented. The clinical description and association with management and environmental factors are discussed. These findings reinforce the knowledge about the relationship between possible vitamin K metabolism interference and Binder's syndrome.

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OBJECTIVE: To characterize the epidemiology, identify trends in prevalence, seasonality, and risk factors for orofacial clefts (OFC), selecting the São Paulo state (SPS) population database. DESIGN: A population-based study to estimate the OFC prevalence trends in recent years, stratified by maternal age and SPS geographical clusters. SETTING: All live births (LB) with OFC in SPS from 2008-2019. PATIENTS: 5342 cases of OFC among 7 301 636 LB. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: OFC prevalence trends, annual percent change (APC) with a 95% confidence interval, and seasonality. RESULTS: We found an OFC prevalence of 7.3/10 000LB in SPS, Brazil. Among all the cases, the majority were male (57.1%), Caucasian (65.4%), 77.8% born at term, 75.8% weight >2500 g, 97.1% singleton, and 63.9% of births were by cesarean section. From 2008-2019, SPS presented a stationary OFC prevalence trend; in São Paulo city, the highest APC was observed (0.05%); the maternal age group with the highest OFC prevalence rate was ≥35 years (9.2/10 000LB). We identified the existence of seasonal variation based on the conception date in the final months of the year, corresponding to the spring season (P < .001). CONCLUSION: OFC had a stationary prevalence trend in recent years, with the highest prevalence in the Central North Cluster and ≥35 years maternal age group. Seasonality was observed in the spring season, and congenital malformation of lips was the most common associated pathology. This population-based study is the first to summarize the current epidemiology of OFC in SPS.

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OBJECTIVE: To evaluate the influence of filling material and timing of surgery on radiograph outcomes of alveolar grafting with premaxillary osteotomy. The null hypothesis was that radiographic outcomes would be similar with both rhBMP-2 (rhBMP-2G) and cancellous bone from the iliac crest (IG), regardless of the timing of surgery. DESIGN: Cross-sectional study with consecutive sampling of 56 periapical or occlusal radiographs taken 12 months after surgery. SETTING: A single tertiary craniofacial center. PATIENTS/PARTICIPANTS: Twenty-eight patients with complete bilateral cleft lip and palate and mean age of 13 years. The individuals underwent bilateral alveolar grafting associated with premaxillary osteotomy (AG + PO) with rhBMP-2 or cancellous bone from the iliac crest. INTERVENTIONS: Experienced maxillofacial surgeons used the same surgical technique in both groups. AG + PO were assigned as success or failure by 3 blinded raters based on modified Bergland and SWAG scales. MAIN OUTCOME MEASURES: The influence of filling materials and timing of surgery on radiographic outcomes was verified by Fisher's exact test and chi-square test (P < .05). RESULTS: There was no significance variation between the mean age of participants in the rhBMP-2G and IG (P = .471). Scales showed almost perfect reliability (agreement rate = 96.4%; K = 0.85). rhBMP-2G and IG had similar success rates with modified Bergland scale (85.7% and 82.1%) and SWAG scale (92.9% and 82.1%), respectively. However, only modified Bergland scale found influence of age on radiographic outcomes (P = .025). CONCLUSIONS: AG + PO performed with rhBMP-2 and iliac crest bone showed similar radiographic success rates, regardless of the timing of surgery.

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To evaluate and compare the dental arch symmetry of individuals with and without cleft lip and palate after orthodontic/rehabilitation treatment.Cross-sectional study.Tertiary cleft center in Brazil.Fifty-five participants aged between 18 and 30 years were divided into 3 groups according to treatment. Patients that received either a fixed partial denture (FPD) or implant-supported crown (ISC) in the cleft area or only orthodontic treatment, noncleft patients (NC).An analysis was performed using digitized dental casts scanned by laser and software. The following linear measurements were evaluated: incisor-canine; canine-molar; incisor-molar; surface and volume of the palatal region.Three-way ANOVA was used to compare the study factors: group (FPD/ISC/NC) and side (right/left) followed by the Tukey test to verify their interaction (α = .05).The results showed statistically significant differences among groups for the maxillary linear measurements canine-molar and incisor-molar, but not for incisor-canine. No statistically significant differences were found regarding the side for the maxillary measurements, while the factor interaction showed similarity only for incisor-canine. The mandibular measurements showed no statistical differences among groups, sides, or factor interactions. In surface and volume, all values in patients with cleft presented lesser than in without cleft patients.Regardless of the rehabilitation, arch symmetry can be achieved in the incisor-canine dimension in the cleft area.

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To evaluate nasal cavity (NC) dimensions of individuals with cleft lip and/or palate (CL/P), obstructive sleep apnea (OSA), and primary snoring, by tomographic image analysis, compared to individuals with OSA without CL/P (N-CL/P).Cross-sectional and retrospective.Tertiary referral center.Patients were divided into 2 groups: (G1) CL/P + OSA or primary snoring, n = 11; (G2) N-CL/P + OSA, n = 13.NC tomographic images were reconstructed using ITK-SNAP software, and measurements were obtained from these three-dimensional models using SpaceClaim software.Total NC volumes, right and left NC volumes, and volumes of the nostril to the nasal valve (V1) and from the nasal valve to the superior limit of the nasopharynx (V2), cross-sectional areas, and perimeters.NC volumes (total, right, and left sides), V1, and V2, though smaller in the CL/P + OSA, did not differ significantly from the N-CL/P + OSA. Cross-sectional areas and perimeters of the superior limit of the nasopharynx, in the CL/P + OSA, presented significantly higher values compared to the N-CL/P + OSA (P ≤ .05).The internal nasal dimensions of patients with CL/P do not seem to be part of the etiopathogenesis, nor constitute a risk factor for OSA with greater severity, in this special group of patients.

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OBJECTIVE: An inspiring early result with no oronasal fistula formation was recently described for a modified medial incision small double-opposing Z-plasty (MIsDOZ) for treating Veau type I cleft palate. This study describes an early single-surgeon experience in applying this newly proposed surgical approach. DESIGN: Retrospective single-surgeon study. PATIENTS: Consecutive nonsyndromic patients (n = 27) with Veau I cleft palate. INTERVENTIONS: Topographic anatomical-guided MIsDOZ palatoplasty with pyramidal space dissection (releasing of the ligamentous fibers in the greater palatine neurovascular bundle and pyramidal process region, in-fracture of the pterygoid hamulus, and widening of space of Ernst) performed by a novice surgeon (RD). MEAN OUTCOME MEASURES: Age at surgery, the presence of cleft lip, palatal cleft width, use of lateral relaxing incision, and 6-month complication rate (bleeding, dehiscence, fistula, and flap necrosis). A published senior surgeon-based outcome dataset (n = 24) was retrieved for comparison purposes. RESULTS: Twenty-two (81.5%) and 5 (18.5%) patients received the medial incision only technique and lateral incision technique, respectively (P = .002). Age, presence of cleft lip, and cleft width were not associated (all P > .05) with the use of lateral incision. Comparative analysis between the novice surgeon- and senior surgeon-based datasets revealed no significant differences for sex (females: 74.1% vs 62.5%; P = .546), age (10.2 ± 1.7 vs 9.6 ± 1.2 months; P = .143), rate of lateral incision (18.5% vs 4.2%; P = .195), and postoperative complication rate (0% vs 0%). CONCLUSION: This modified DOZ palatoplasty proved to be a reproducible procedure for Veau I cleft palate closure, with reduced need for lateral incision and with no early complication.

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OBJECTIVE: To describe the population of patients with cleft lip and/or palate (CL/P) in terms of cleft phenotypes, gender, age, ethnic group, family history, clinical presentation (syndromic vs nonsyndromic), some environmental and behavioral factors, and some clinical features. DESIGN: Descriptive retrospective study. SETTING: Patients attending the genetics counseling practice in Operation Smile Foundation, Bogotá, Colombia, for over 8 years. PARTICIPANTS: No screening was conducted. All patients requiring clinical genetics assessment in Operation Smile Foundation were included in the study. RESULTS: Left cleft lip and palate (CLP) and nonsyndromic forms were the most frequent types of malformations in this population. Psychomotor retardation and heart disease were the most frequent comorbidities in these patients. A low proportion of mothers exposed to passive smoking during pregnancy was observed and low birth weight accounted for an important number of cases. Aarskog, velocardiofacial, and orofaciodigital syndromes were the most frequent syndromic forms of CLP in this population. CONCLUSIONS: In this study, the most frequent type of CL/P was the nonsyndromic complete left CLP. Aarskog, velocardiofacial, and orofaciodigital syndromes were the most frequent syndromic forms of CL/P in this population.

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Congenital midline cervical cleft is a rare and generally isolated malformation of the ventral neck region with no clear etiology established. Mandibular deformities, such as micrognathia, could be considered as a consequence of a cleft cervical contracture. Complete surgical excision of the subcutaneous fibrous cord at an early age is the primary treatment modality, minimizing growth development problems on surrounding affected tissue. The aim of this study is to describe the clinical, surgical, and histological findings in a female child with congenital midline cervical cleft along with a relevant literature review. Three years follow-up after surgery exhibited satisfactory functional and cosmetic results.

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OBJECTIVE: To compare occlusal relationship in patients undergoing neonate versus conventional lip surgery (LS) with and without infant orthopedics (IO) by assessment of dental arch relationship in individuals with complete unilateral cleft lip and palate. MATERIAL AND METHODS: Three groups treated by different protocols; Group I: neonate LS (1-15 days) + IO and palatoplasty (13-31 months); Group II: LS (3-12 months) + IO and palatoplasty (15-35 months); and Group III: LS (3-6 months) without IO and palatoplasty (12-18 months). The 112 intraoral photographs of individuals of all groups, obtained between 6 and 12 years of age, were assessed by the occlusal index for intraoral photograph rating. The groups were compared by the χ2 test. The correlation between surgical timing and the scores was tested by the Spearman test (P < .05). RESULTS: Group I presented the highest percentage of score 5, group II exhibited highest percentage of score 1, and group III presented the lowest percentage of score 5 according to the χ2 test (P = .029). The Spearman correlation test revealed statistically significant difference between timing of LS and the occlusal index. The earlier the surgical timing, the higher the occlusal index (P = .019). CONCLUSIONS: Infant orthopedics has demonstrated the possibility of postponing primary plastic surgeries. Patients submitted to late lip and palate repair had the best prognosis, while patients undergoing lip repair from 1 to 15 days of life, even operating the palate later, had the worst prognosis. Neonate LS negatively influenced the occlusal relationships.

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OBJECTIVE: The aim of this study was to use dental development as a tool to subphenotype oral clefts and investigate the association of MMP2 with dentin-pulp complex anomalies, in order to identify dental anomalies that are a part of a "cleft syndrome." DESIGN: Two hundred and ninety individuals born with cleft lip and palate were evaluated and several clinical features, such as cleft completeness or incompleteness, laterality, and presence of dental anomalies were used to assess each individual's cleft status. We tested for overrepresentation of MMP2 single nucleotide polymorphism rs9923304 alleles depending on individuals having certain dental anomalies. Chi-square and Fisher exact tests were used in all comparisons (α = .05). RESULTS: All individuals studied had at least one dental anomaly outside the cleft area. Significant differences between individuals born with clefts with and without talon cusp (P = .04) were observed for the frequency of the MMP2 less common allele. CONCLUSION: All individuals born with cleft lip and palate had alterations of the dentition, and a quarter to half of the individuals had alterations of the internal anatomy of their teeth, which further indicates that dental anomalies can be considered as an extended phenotype for clefts. MMP2 was associated with talon cusp in individuals born with oral clefts.

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Rare facial clefts are characterized by facial involvement that is not restricted to the lip, palate, and alveolus as in traditional cleft lip and palate. The craniofacial skeleton and the orbital structures are frequently affected in these conditions. Exposure of the eyeball, when not early treated, puts the function and the preservation of the eye at risk. We report the case of a 2-month-old boy admitted to our service with an extensive oral-ocular cleft and exposure of the eyeball with melting corneal ulcer treated with a conjunctival closure with a purse-string suture.

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