Isolated Cleft Palate Research Articles

Cleft Lip and Palate and Isolated Cleft Palate

Cleft Lip and Palate and Isolated Cleft Palate

Smartphone‐based scans of palate models of newborns with cleft lip and palate: Outlooks for three‐dimensional image capturing and machine learning plate tool

AbstractObjectivesTo evaluate the performance of smartphone scanning applications (apps) in acquiring 3D meshes of cleft palate models. Secondarily, to validate a machine learning (ML) tool for computing automated presurgical plate (PSP).Materials and MethodsWe conducted a comparative analysis of two apps on 15 cleft palate models: five unilateral cleft lip and palate (UCLP), five bilateral cleft lip and palate (BCLP) and five isolated cleft palate (ICP). The scans were performed with and without a mirror to simulate intraoral acquisition. The 3D reconstructions were compared to control reconstructions acquired using a professional intraoral scanner using open‐source software.ResultsThirty 3D scans were acquired by each app, totalling 60 scans. The main findings were in the UCLP sample, where the KIRI scans without a mirror (0.22 ± 0.03 mm) had a good performance with a deviation from the ground truth comparable to the control group (0.14 ± 0.13 mm) (p = .653). Scaniverse scans with a mirror showed the lowest accuracy of all the samples. The ML tool was able to predict the landmarks and automatically generate the plates, except in ICP models. KIRI scans' plates showed better performance with (0.22 ± 0.06 mm) and without mirror (0.18 ± 0.05 mm), being comparable with controls (0.16 ± 0.08 mm) (p = .954 and p = .439, respectively).ConclusionsKIRI Engine performed better in scanning UCLP models without a mirror. The ML tool showed a high capability for morphology recognition and automated PSP generation.

Columbia-Suicide Severity Rating Scale (C-SSRS) Reveals High Rates of Suicidality in 602 Patients With Cleft and Craniofacial Conditions.

Little is known about rates of suicidal ideation and behavior among youth with cleft lip and/or palate (CLP) and other craniofacial conditions. Records of patients ages 6 and older who were administered the Columbia-Suicide Severity Rating Scale (C-SSRS) Lifetime Version during routine multidisciplinary cleft or craniofacial team visits between 2019 and 2023 were examined. Demographics information, C-SSRS data, and diagnoses were assessed with statistics including t tests, the Fisher exact test, and odds ratios. A total of 1140 C-SSRS questionnaires across 602 (433 CLP and 169 craniofacial) patients with an average age of 11.2±3.7 years were included. Eighty-four (13.6%) patients endorsed lifetime suicidal ideation, 9 (1.5%) had at least one instance of suicidal behavior, 30 (5.0%) endorsed nonsuicidal self-injury, and 2 (0.3%) engaged in self-injurious behavior. Compared with CLP, those with other craniofacial conditions had similar odds of endorsing suicidal ideation and behavior ( P ≥0.05). Compared with those with isolated cleft palates, CLP had greater odds of endorsing suicidal ideation and behavior, though those differences were not significant ( P ≥0.05). Incidence of suicidality was unchanged before, during, and after the COVID-19 pandemic ( P ≥0.05). Dividing patients by sex or insurance type revealed no difference in suicidality ( P ≥0.05). Patients with CLP and craniofacial conditions have a high incidence of suicidal ideation and behavior, though levels are similar between these groups. Suicidality in these patients was not negatively impacted by the COVID-19 pandemic. Early identification of safety risks and psychosocial challenges through regular screening can facilitate connection with appropriate clinical interventions.

Respiratory outcomes after cleft palate closure in Robin sequence: a retrospective study

ObjectivesThere is a paucity of information about the possible risk factors that could identify patients with Robin sequence (RS) who are more prone to developing obstructive airway complications after palate closure. This study aimed to compare the respiratory complication rates in patients with RS and isolated cleft palate (ICP).Materials and methodsIn this retrospective study, we reviewed the medical records of 243 consecutive patients with RS and ICP who were treated at Amsterdam University Medical Centers over the past 25 years. We collected preoperative data on previous treatment, diagnostic findings, surgical technique, weight, and presence of congenital anomalies.ResultsDuring cleft palate closure, patients with RS were older (11.9 versus 10.1 months; p = 0.001) and had a lower gestational age than those with ICP (37.7 versus 38.5 weeks; p = 0.002). Patients with RS had more respiratory complications (17 versus 5%; p = 0.005), were more often non-electively admitted to the pediatric intensive care unit (PICU) (13 versus 4.1%; p = 0.022), and had a longer hospital stay duration (3.7 versus 2.7 days; p = 0.011) than those with ICP. The identified risk factors for respiratory problems were a history of tongue-lip-adhesion (TLA) (p = 0.007) and a preoperative weight of < 8 kg (p = 0.015). Similar risk factors were identified for PICU admission (p = 0.015 and 0.004, respectively).ConclusionsThe possible risk factors for these outcomes were a low preoperative weight and history of TLA. Closer postoperative surveillance should be considered for patients with these risk factors.Clinical relevanceIdentifying risk factors for respiratory complications could provide clinicians better insight into their patients and allows them to provide optimal care for their patients.

Occlusal Outcomes in Non-Robin Sequence Patients with Isolated Cleft Palate.

1. To assess the skeletal class occlusion and lateral cephalometry in children with isolated cleft palates (non-Robin sequence) and 2. to identify associations between these findings and pre-palatoplasty cleft palate measurements. Retrospective cohort study. North American Institutional Tertiary Paediatric Center. Our cleft database was reviewed, and patients were included if they had an isolated cleft palate without a Robin Sequence diagnosis, had a Furlow palatoplasty and had available per operative cleft palate measurements and available lateral cephalogram between 6 and 8 years old. Thirty-two patients matched to inclusion criteria. Furlow's Palatoplasty. Cleft size at palatoplasty, cephalometric measurements and skeletal occlusal classes were analysed. ANOVA was used to test the association between cephalometric measurements and occlusal classes. Results are presented as means with a 95% confidence interval. The association between cleft measurements and cephalometric parameters was tested with Spearman Correlation (rs). The skeletal occlusal outcome at 7 years old for this series of patients was: Class I: 19%; Class II: 59% and Class III: 22%. No single cleft measurement at palatoplasty was predictive of the skeletal occlusal outcome. A larger hard palate cleft was associated with a shorter antero-posterior maxilla. The skeletal occlusal class outcomes were similar to those found in a previous study in the literature. The occlusal prognosis appears to be better than in patients with Robin Sequence or with an associated cleft lip. No preoperative measurement was found to be associated with the occlusal outcome.

An Adult with Untreated Isolated Cleft Palate Since Birth: A Rare Case Report

Introduction: Orofacial clefts are one of the most common congenital anomalies involving the craniofacial region. An isolated cleft palate is the rarest (33% of all orofacial clefts) among individuals with female predominance. Individuals born with these clefts manifest craniofacial, functional, and psychological anomalies. Sometimes, patients cannot receive the required therapies due to a lack of awareness and lack of medical access or economic factors. Untreated clefts may adversely impact the patient’s life both physically and psychologically.&#x0D; Case Description: This study presents a case report of a 45-year-old male with an untreated cleft palate since birth due to a low socioeconomic background, leading to several oro-dental anomalies, speech defects, and psychological and social challenges.&#x0D; Results: Extraoral examination revealed mild facial asymmetry, dry and incompetent lips, and underdeveloped philtrum. Intraorally, there was a cleft palate, macroglossia, soft and edematous gingiva with bleeding on probing, gingival recession, and periodontal pockets, along with various dental anomalies. Full mouth orthopantomogram showed discontinuity of the palatal line, collapsed maxillary arch, root stumps, and crowding. The final diagnosis of isolated cleft palate was made. The patient was advised for surgery regarding the cleft palate, dental treatment, speech therapy, and psychological counseling. However, the patient refused all treatments except a few dental aids due to economic difficulties.&#x0D; Conclusion: The present case report highlights the need for these patients to receive particular concerns. Patients must be motivated to get proper treatment at a suitable stage to prevent future complications. Cleft teams must be advised to initiate some encouraging steps in imparting treatment aids to such patients, especially in rehabilitation therapy, considering their surrounding environment, psychological makeup, socioeconomic status, and motivation. In addition, social groups should also treat these patients with a positive approach, helping them to live healthy and normal lives just like any other individual.

Classification of Skeletal Phenotypes of Preadolescent Patients With Isolated Cleft Palate Using Principal Component Analysis and Cluster Analysis.

This study aimed to classify the skeletal phenotypes of preadolescent patients with isolated cleft palate using principal component analysis and cluster analysis. Sixty-four preadolescent female patients with isolated cleft palate (incomplete hard palate and complete soft palate cleft group, n=51; complete cleft of the hard and soft palate group, n=13; the mean age when lateral cephalograms were taken, 7.08±0.76y) were included. Ten angular and 2 ratio cephalometric variables were measured on a lateral cephalogram. Cluster analysis was performed using 3 representative variables obtained from principal component analysis (SN-GoMe, SNA, and SNB). The differences in the variables among the clusters were characterized using the Kruskal-Wallis test. As a result of the analysis, 6 clusters were obtained from 3 groups: the retrusive maxilla and mandible group: cluster 3 (14.1%, moderately hyperdivergent pattern), cluster 5 (17.2%, severely hyperdivergent pattern); the normal maxilla and mandible group: cluster 1 (23.4%, normodivergent pattern), cluster 4 (12.5%, moderately hyperdivergent pattern), cluster 6 (20.3%, severely hyperdivergent pattern); the normal maxilla and protrusive mandible group: cluster 2 (12.5%, normodivergent pattern). The distribution of isolated cleft palate types did not differ among the 6 clusters ( P >0.05). Two thirds of the patients (68.7%, clusters 1, 2, 4, and 6) had a normal anteroposterior position of the maxilla, while one third of the patients (31.3%, clusters 3 and 5) showed a retrusive mandible. These results indicate that isolated cleft palate patients have diverse maxillo-mandibular growth patterns compared with patients with cleft lip and palate.

Long-term speech outcome in patients with Robin sequence after cleft palate repair and tongue-lip adhesion: A 21-year retrospective analysis

The purpose of this study was to assess the effect of tongue−lip adhesion (TLA) on the long-term speech and articulation outcomes of patients with Robin sequence (RS) after cleft palate repair. Outcomes were compared to those in patients with RS who required positioning alone and to patients with isolated cleft palate (ICP).All consecutive patients with RS (with or without TLA) versus isolated cleft palate (ICP) who underwent cleft palate repair were retrospectively reviewed. Speech and articulation included all assessments between the age of 3–6 years. Secondary speech operations, velopharyngeal insufficiency (VPI), hypernasality, and articulation errors by cleft-type characteristics (CTC), including 4 categories (passive), non-oral, anterior-oral, and posterior-oral. A total of 41 RS patients and 61 ICP patients underwent repair with sufficient follow-up. Of them, 23 patients underwent a TLA at median age of 12 days. Rates of hypernasality (p = 0.004), secondary speech operations (p = 0.004), and posterior oral CTC (p = 0.042) were higher in RS compared to ICP. Isolated RS had speech outcomes similar to those of ICP; however, syndromic RS patients needed more secondary speech operations compared to isolated RS (p = 0.043). TLA-RS patients did not demonstrate differences in speech outcomes or any CTCs (all p > 0.05) compared to non−TLA-RS patients, except for the anterior oral CTC (74% TLA-RS vs 28% non−TLA-RS, p = 0.005).Within the limitations of the study, it seem that TLA does not affect long-term velopharyngeal function in patients with RS. However, TLA-RS patients demonstrated higher rates of anterior-oral CTC, which might be related to a different positioning of the tongue after TLA. Every effort should be taken to treat patients with RS conservatively instead of with TLA because of this demonstrated a negative effect on one type of articulation error. However, if conservative therapy fails, a TLA is still a valuable adjunct in the treatment of RS, and cleft speech pathologists who treat such patients should be more aware of this phenomenon in order to improve long-term articulation outcomes.

Is There a Relationship between Palatal Asymmetry and Speech in Children with Isolated Cleft Palate after Double-Opposing Z-Plasty?

Although some cleft palates show asymmetric palatal shelf length and/or width intraoperatively, the relationship between palate asymmetry and speech outcomes has not been fully investigated. This study retrospectively reviewed 234 patients who underwent double-opposing Z-plasty (DOZ) for isolated cleft palate (Veau class I and II). Speech outcomes were analyzed to evaluate the association with width and length discrepancy of the palatal shelves using multiple logistic regression adjusting for patient age. The mean age at repair was 14.2 ± 5.26 months. The mean palatal shelf width and length differences were 0.87 ± 0.97 mm and 1.63 ± 1.61 mm, respectively. The mean age at initial and follow-up assessment was 37.6 ± 5.70 months ( n = 234) and 66.2 ± 8.81 months ( n = 120), respectively. Multivariate logistic regression analysis of initial speech outcomes showed odds ratios in width discrepancy of 1.67 ( P = 0.0703), 1.59 ( P = 0.0104), and 2.01 ( P = 0.0051) for nasal emission, hypernasality, and compensatory articulation, respectively. Additional analysis including follow-up outcomes also revealed that width discrepancy of the palatal shelves had higher odds ratios for nasal emission, hypernasality, and compensatory articulation (OR, 1.49, P = 0.0406; OR, 1.36; P = 0.0660; and OR, 1.65; P = 0.0170, respectively). There was no association between length discrepancy and all speech abnormalities. Greater discrepancies in palatal shelf width, rather than in length, were associated with poorer speech outcomes after DOZ. The authors suggest that DOZ is effective for longitudinally asymmetric cleft palates. Risk, III.

Evaluation of consonant development following cleft palate surgery: Comparison of Isolated Cleft Palate Versus Pierre Robin sequence

The optimal management of Cleft palate patient from birth to completion of the treatment continue to present a formidable challenge the professionals of the multidisciplinary cleft clinic (MDCC) specially children with Pierre Robin Sequence (PRS) than the children with Isolated cleft palate (ICP).

Cayler Syndrome with Isolated Cleft Palate: Case Report

Published on:August 2022 Journal of Young Pharmacists, 2022; 14(3):337-338 Case Report | doi:10.5530/jyp.2022.14.66 Authors: Elizabeth Jacob*, Cyril Sajan Department of Pharmacy, Sumandeep Vidyapeeth (Deemed to Be University), Vadodara, Gujarat, INDIA. Abstract: The present study was focused on assessing the clinical Isolated cleft palate is the term used when a cleft occurs only within the palate. In clinical terms it is differentiated from other clefts, affecting the primary palate because it as such doesn’t affect either the alveolar ridge or the perioral musculature. This makes the diagnosis of the cleft, by facial analysis, more challenging, since it is concealed in the palate. Cayler syndrome is an abnormal condition at birth represented by hypoplasia of the depressor angular oris muscle (HDAOM) in an addition to cardiac birth defects. A considerable range of multiple organ system anomalies are also to predicted to be present. Paediatrician must make awareness regarding this minor facial anomaly as it can be related to multiple, cardiac and internal organs abnormalities. I present to you a paediatric rare case of cleft palate which appears to be isolated, along with cayler syndrome. To my knowledge no previous report on situs inverse totalis was reported. Additionally, the patient does not have any major cardiac anomalies associated with the cayler complications.Keywords: Isolated cleft palate, Cayler syndrome, Palatoplasty, Palatogenesis, Clefting.

Monogenic Versus Multifactorial Inheritance in the Development of Isolated Cleft Palate: A Whole Genome Sequencing Study.

Craniofacial morphogenesis is highly complex, as is the anatomical region involved. Errors during this process, resulting in orofacial clefts, occur in more than 400 genetic syndromes. Some cases of cleft lip and/or palate (CLP) are caused by mutations in single genes; however, complex interactions between genetic and environmental factors are considered to be responsible for the majority of non-syndromic CLP development. The aim of the current study was to identify genetic risk factors in patients with isolated cleft palate (CP) by whole genome sequencing. Patients with isolated CP (n = 30) recruited from the Riga Cleft Lip and Palate Centre, Institute of Stomatology, Riga, were analyzed by whole genome sequencing. Pathogenic or likely pathogenic variants were discovered in genes associated with CP (TBX22, COL2A1, FBN1, PCGF2, and KMT2D) in five patients; hence, rare disease variants were identified in 17% of patients with non-syndromic isolated CP. Our results were relevant to routine genetic counselling practice and genetic testing recommendations. Based on our data, we propose that all newborns with orofacial clefts should be offered genetic testing, at least for a panel of known CLP genes. Only if the results are negative and there is no suggestive family history or additional clinical symptoms (which would support additional exome or genome-wide investigation), should multifactorial empiric recurrence risk prediction tools be applied for families.

Prevalence of dental anomalies in different cleft lip and palate groups

Introduction: Patients with orofacial clefts are usually associated with various dental anomalies. These dental aberrations pose a clinical challenge in treatment planning. Thus, this investigation intends to find the prevalence rate of different types of dental anomalies and their correlation among nonsyndromic cleft patients. Methodology: Orthodontic records of 129 cleft patients classified into different cleft groups were assessed to evaluate the presence of different dental anomalies. Results: The most frequent dental anomaly found in Cleft patients was rotations followed by maxillary lateral incisor agenesis with a prevalence rate of 90.4% and 59.2%, respectively. Maxillary second premolar agenesis, peg-shaped laterals, mandibular second premolar agenesis, and supernumerary teeth showed prevalence rates as 25%, 21%, 7%, and 5%, respectively. There were significant differences in the overall distribution of maxillary lateral incisor agenesis and rotations between patients with Unilateral Cleft Lip and Cleft Palate, Bilateral Cleft Lip and Cleft Palate, Cleft Lip (CL), and Isolated Cleft Palate (ICP). Conclusion: There is a high prevalence of dental anomalies like rotations and maxillary lateral incisor agenesis in different CL and palate patients, which were statistically significant. The presence of such anomalies can deteriorate the already existing functional problem and hence, the study of these anomalies and their treatment is an important step in the rehabilitation of such patients.

Stress Distribution and Displacement of Craniofacial Structures Following Rapid Maxillary Expansion in Different Types of Cleft Palate: A Three-Dimensional FEM Study.

To evaluate displacements and stress distributions in finite element models (FEMs) of the craniofacial complex of 13-yearold male patient with complete unilateral cleft palate (UCP), a 15-year-old female patient with complete bilateral cleft palate (BCP), and a 15-year-old female patient with isolated cleft palate (ICP), which may respond differently to expansive forces. The FEMs were based on computed tomography scans of patients with UCP, BCP, and ICP who needed maxillary expansion. Von Mises stress distribution after 0.2 mm of expansion and displacements after 5 mm of expansion were investigated. The highest amount of stress was observed in the ICP model. Surprisingly, no stress was noted around the nose in the BCP model. The amount of dentoalveolar expansion decreased from anterior to posterior on the cleft side of the UCP, BCP, and ICP models. In contrast, on the non-cleft side of the UCP model, the maximum dentoalveolar expansion occurred at the molar area, decreasing toward the anterior parts. Anatomical structures expressed posterior displacement in the UCP model. In the ICP model, structures close to midline showed anterior displacement, while structures in the lateral parts showed posterior displacement. In contrast with the other 2 models, the structures in the BCP model showed anterior displacement. Vertically, all the anatomic structures in the BCP model showed inferior displacement, while in the ICP and UCP models, only the structures close to the midline showed inferior displacement. Maxillary expansion caused different patterns of stress distribution and displacement in different types of clefts. Clinicians should consider the type of the cleft, and may expect differing patterns of widening following maxillary expansion.

How Does Hypodontia Compare in Nonsyndromic Pierre Robin Sequence Versus Isolated Cleft Palate and Isolated Cleft Lip?

To assess the prevalence and patterns of hypodontia in nonsyndromic Pierre Robin sequence (PRS) and compare it with hypodontia in nonsyndromic isolated cleft palates and isolated cleft lips. Retrospective cohort study. Alder Hey Children's Hospital, United Kingdom. Patients with nonsyndromic PRS (group 1), isolated cleft palate (group 2), and isolated cleft lip (group 3). Hypodontia in the permanent dentition assessed from orthopantomographs. A total of 154 patients were included. Group 1 had the highest incidence of hypodontia with 47% having at least one tooth congenitally absent. Groups 2 and 3 had reduced rates of hypodontia with 27% and 19% of the groups missing teeth, respectively; 93% of cases of hypodontia in group 1 involved the absence of at least one second premolar. Of these patients, there was found to be bilateral agenesis of second premolars in 50% of cases. Patients with PRS and cleft palates are more likely to have hypodontia than those with isolated cleft palates or unilateral cleft lips. Patients with PRS have more severe hypodontia than those with isolated cleft palates or unilateral cleft lips. Bilateral agenesis of lower second premolars is a commonly seen pattern among patients with PRS. In this large UK study, a similar prevalence and pattern of hypodontia to other nonsyndromic PRS populations worldwide has been demonstrated.

Characteristics of craniofacial morphology and factors affecting them in patients with isolated cleft palate.

BackgroundMyriad maxillo-mandibular occlusal relationships are observed in patients with isolated cleft palate (ICP), unlike in patients with other cleft types, such as cleft lip and palate.ObjectivesThis study aimed to categorise the characteristics of craniofacial morphology in patients with ICP, and investigate the clinical factors affecting these categorised morphological characteristics.MethodsThirty-six girls with ICP (age (mean ± SD): 5.36 ± 0.36 years) underwent cephalometric measurement. Their craniofacial morphology was categorised using cluster analysis. Profilograms were created and superimposed onto the standard Japanese profilograms to visualise the morphological characteristics of each group (cluster). The mean values and variations in the linear and angular measurements of each group were compared with the Japanese standards and statistically analysed using Dunnett’s test after the analysis of variance. Fisher’s exact test was used to analyse the differences between the cleft types (cleft in the hard and/or soft palate) and skills of the operating surgeons in the groups.ResultsCluster analysis of craniofacial morphologies in patients with ICP resulted in the formation of three categories: the first cluster exhibited a relatively harmonious anteroposterior relationship between the maxilla and the mandible (22.2%); the second cluster exhibited crossbite owing to a significantly smaller maxilla (33.3%); and the third cluster exhibited a smaller mandible with posterior rotation showing skeletal class II malocclusion (44.4%). Differences in cleft types and surgeons were not associated with the distribution of patients in each cluster.ConclusionsPatients with ICP exhibited characteristic morphological patterns, such as bimaxillary retrusion or severe mandibular retrusion, besides the anterior crossbite frequently found in patients with cleft lip and palate . Understanding the typical morphological characteristics could enable better diagnostic categorisation of patients with ICP, which may eventually improve orthodontic treatment planning.

Evaluation of Discipline-Specific Outcomes Through a Multidisciplinary Team Clinic for Patients With Isolated Cleft Palate.

To describe the incidence and timing of provider-specific interventions for children with isolated cleft palate. This was a retrospective cohort study involving review of medical records. Multidisciplinary team care clinic at a tertiary academic children's hospital between January 2000 and July 2019. Patients with isolated nonsyndromic cleft palate seen by an American Cleft Palate-Craniofacial Association-approved team; 138 children were included. Study outcomes included incidence of secondary velopharyngeal management, tympanostomy tube insertion, speech therapy, hearing loss, dental/orthodontic treatment, and psychology interventions. Provider-specific outcomes were calculated for patients at ages 0 to 3, 3 to 5, and >5 years. Median follow-up time was 7.0 years (interquartile range: 3.3-11.8 years). At their last team assessment, 42% of patients still had conductive hearing loss. The rate of tympanostomy tube insertions not done alongside a palatoplasty was highest for ages 3 to 5 and dropped after new American Academy of Otolaryngology-Head and Neck Surgery Foundation guidelines in 2013 (P = .015); 54% of patients received speech-language therapy during follow-up. Palatoplasty, psychology, and dental/orthodontic treatment were all less common than speech or ENT treatment (P < .01). Secondary palatoplasty was performed in 31 patients (22%). Patients who received speech, dental/orthodontic, or psychology intervention followed up longer than those who did not (9.8 vs 2.1 years, P < .001). Half of the patients terminated team follow-up by age 7, suggesting that burden of care outweighed perceived benefits of continued follow-up for many families. These results can be used to adjust protocols for children with isolated cleft palate.

Long-term Speech Outcomes of Cleft Palate Repair in Robin Sequence versus Isolated Cleft Palate.

This retrospective case series of consecutive syndromic and isolated RS- and ICP-patients (1990-2016) includes indications and outcomes of straight-line repair with intravelar veloplasty (SLIV) or Furlow repair depending on cleft and airway characteristics. Seventy-five RS and 83 ICP patients underwent CP repair. Velopharyngeal insufficiency (VPI) occurred in 41% of RS versus 17% of ICP patients (P = 0.012), and in 60% of patients with syndromic RS versus 16% with isolated RS (P = 0.005). In multivariable logistic regression analysis, wider and more severe CP anatomy was the only factor independently associated with VPI (P = 0.028), in contrast to age at repair, syndromic RS compared with isolated RS, and isolated RS compared with ICP and initial tongue-lip adhesion. Secondary Furlow after primary SLIV was used to treat VPI in all groups, and more frequently in syndromic versus isolated RS patients (P = 0.025). Variability of RS anatomy and airway compromise necessitates individualized treatment protocols. Despite differing CP etiology and other variables, our findings demonstrate cleft anatomy as the only independent variable predictive of VPI comparing RS and ICP patients. Patients with isolated RS should ultimately attain similar VPI outcomes compared with ICP patients. Obstructive speech operations in RS patients can be avoided without compromising speech outcome by reserving the prsocedure for secondary cases.

Palatal Re-Repair With Z-Plasty in Treatment of Velopharyngeal Insufficiency of Syndromic and Nonsyndromic Patients With Cleft Palate.

Velopharyngeal insufficiency (VPI) often results from palatal shortening or insufficient levator function after cleft palate repair. To assess the efficacy of palatal re-repair with Z-plasty in treatment of VPI for patients with isolated cleft palate (ICP). This retrospective analysis comprised 130 consecutive patients who had ICP with VPI that required Z-plasty as secondary surgery between 2008 and 2017. Pre- and post-operative evaluation of velopharyngeal function was done perceptually and instrumentally by Nasometer. Median patient age at Z-plasty was 6.8 years (range 3.0-20.1). Of the 130 patients, preoperatively VPI was severe in 73 (56%), mild-to-moderate in 55 (42%), and borderline in 2 (2%). Postoperatively, 105 (81%) of patients achieved adequate (normal or borderline) velopharyngeal competence and 16 (12%) required second operation for residual VPI. The success rate was 84% in nonsyndromic patients, 79% in nonsyndromic Pierre Robin sequence patients, and 58% in syndromic patients. In syndromic children, the speech outcome was significantly worse than in nonsyndromic children (P = 0.014). Complications included wound healing problems in 3 patients (2%), mild infection in 1 patient (1%), postoperative bleeding in 1 (1%), and postoperative fistula in 2 (2%). Palatal re-repair with Z-plasty is a safe operation for VPI correction in patients with ICP with a success rate of 81%. In syndromic patients, the procedure did not seem to work as well as in nonsyndromic patients.

The Likelihood of Orthognathic Surgery After Orofacial Cleft Repair.

Midface hypoplasia is one of the most significant sequelae of cleft lip and/or palate surgery. A complete understanding of the rate of orthognathic surgery across varying cleft phenotypes is a powerful tool for educating patients and families as to the treatment course that the patient will incur during their lifetime. Understanding the average rates of orthognathic intervention also can act to develop metrics for outcome evaluation with different treatment protocols. Attempting to identify the average rates of orthognathic intervention, the authors conducted a systematic review and meta-analysis by combining studies from 1987 to 2016 describing the frequency of orthognathic intervention on the different cleft phenotypes as the primary outcome. Secondary outcomes included identification of surgical protocol, age of patient at orthognathic intervention, and the method by which patients were evaluated for orthognathic intervention. The rate of orthognathic surgery was 38.1% for bilateral cleft lip and palate (BCLP), 30.2% for unilateral cleft lip and palate (UCLP), 4.4% for isolated cleft palate (ICP), and 1.8% for patients with isolated cleft lip (ICL). 71% (n = 10) reported using lateral cephalograms for orthognathic surgery evaluation and only one of those studies reported specific objective cephalometric measurements for orthognathic intervention. Our findings demonstrated that BCLP possessed the highest rate of orthognathic intervention followed by UCLP, ICP, and ICL. ICP and ICL both possessed low rates of orthognathic intervention. By sharing our findings, the authors hope to provide a useful tool for informing patients' families as to their risk of needing orthognathic intervention.