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Purpose: The study's objectives were to assess the effect of articulation therapy for bilabials on SODA errors, cleft type errors (CTEs), and percentage of correct consonants-revised (PCC-R) in children with repaired cleft lip and palate. Methods: Single-subject with multiple baselines research design was used to investigate the changes in bilabials across four-time points. Four participants with repaired cleft lip and palate (RCLP) between 4 and 7.11 years were considered. For the assessment, pictures of six words were visually presented, and the participants were asked to name them. Three speech-language pathologists identified SODA errors and cleft type errors (CTE), based on which PCC-R was calculated. Participants underwent ten intensive articulation therapy sessions: phase I focused on auditory discrimination training and phase II on production training. The production training mainly focused on the phonetic placement approach, shaping the target sound, and improving the oral airflow. Results: Overall, SODA error analysis revealed substitution and distortion errors during the baseline assessment. CTE analysis indicated weak oral pressure consonant followed by a glottal stop, nasalization of voiced pressure, nasal consonants for oral pressure consonants, and voicing errors. PCC-R scores ranged from 0% to 83.33%. Assessment 4 indicated only distortion errors during SODA error analysis, weak oral pressure consonants during CTE analysis PCC-R was 100%. The obtained results indicate an improvement in the articulation placement and oral airflow; thus, the participants benefited from the intervention program.

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Introduction: Automatic speech processing (ASP) software is a nasality assessment tool. ASP studies focusing on investigating sentences to find nasality and correlating ASP scores with other objective assessment scores measuring nasality are scarce. Hence, the present study aimed at comparing the nasalance values of the ASP software with the nasometer in typically developing children (TDC) and children with repaired cleft palate (RCP) across different stimuli. Methods: Participants included 30 Kannada speaking TDC and 10 children with RCP (9–12 years). Speech stimuli (oral, nasal, and oronasal sentences) were recorded and the values were obtained from the ASP software as well as the nasometer. The following statistical tests were applied: mixed ANOVA, repeated measures ANOVA, paired samples t-test, independent samples t-test and Pearson's correlation. Results: Like nasometer, the nasalance values of ASP software were high for the nasal sentences followed by the oronasal sentences and the oral sentences, for both the populations. Higher nasalance values were found for children with RCP than for TDC across all the stimuli. Significant differences were found in nasalance values between the instruments in oral and oronasal sentences in TDC and nasal sentences and oronasal sentences in RCP. The nasalance values across the stimuli between nasometer and ASP software in both the groups showed no significant correlations. Conclusions: ASP software was successful in identifying nasalance in TDC and children with RCP. However, a major issue needs to be addressed concerning the dynamic range of the software and it has to be validated on a large number of populations.

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Introduction: Evaluating the length of orthodontic-surgical treatment is a key factor to properly inform and guide the patient about the duration of treatment and consequently achieve agreement and adherence to treatment, as well as for health-care institutions that sponsor this type of treatment. Objective: The aim of the present study was to investigate the duration of orthodontic treatment before orthognathic surgery, in patients with nonsyndromic oral clefts, as well as the number of consultations required for that purpose. Materials and Methods: This was an observational, retrospective, descriptive study conducted on data obtained from the patients' medical records. The study analyzed 485 medical records and, after applying the inclusion criteria, the sample included 227 medical records. All data were tabulated in an Excel spreadsheet for calculation of means and standard deviations (SDs), for the time period analyzed. Results: The time for orthodontic preparation was 8.2 years with a range of attendance from 1 to 130 visits and a mean of 37 visits (SD ± 24). Conclusion: The results suggest that there is a longer time than reported in the literature for orthodontic preparation for orthognathic surgery, which could contribute to the increase in total time for orthodontic-surgical treatment.

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Children with cleft lip and palate often suffer from nutritional deficiencies and subsequent growth problems, both of which are largely attributed to feeding difficulties due to the structural defect as well as the numerous surgical procedures that they undergo. The aim of this review article is to assess the nutritional needs of such children and to emphasize the role of nutrition in their long-term growth and development along with parental education on nutrition and various alternative feeding practices as an important aspect in the management of cleft lip and palate which may otherwise be overlooked. For this review, various articles on cleft lip and palate from plastic surgery to dental surgery journals were studied and more articles were based on subsequent bibliographic reviews of the above. It was concluded that to achieve optimal health in these children, the dietary changes required per day were minuscule. Rather, it is the delivery method of breast milk or formula which often needs to be changed to reduce effort and resultant caloric loss by the newborn, indirectly boosting the caloric intake and resultant weight gain and growth.

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The cleft lip and palate (CLP) anomaly involves treatment right from birth till adulthood. A lot of treatment modalities are also time and age sensitive, that if not performed at the correct time, the results are sub-optimal or compromised. One such treatment modality is Presurgical Infant Orthopaedics (PSIO) which is among the first corrective and therapeutic procedures performed in the infants with CLP anomaly. The role of an orthodontist is pivotal in guiding the two cleft segments into a relatively normal position before the surgical repair is performed. Over the course of history, this procedure has seen many modifications, arguments over its utility, and counter arguments of the potential harms. The present articles shed light over the initial inception of the procedure, its development and improvement, long-term changes seen in the patients treated with PSIO technique, and finally the latest advancements in technique.

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Over the last few decades, there is a paramount shift in the implementation of pediatric anesthesia. Although for most of the time, difficult airway scenarios are well-planned and executed, occasionally an unanticipated difficult situation may still arise. This is more common in patients with orofacial anomalies such as cleft lip and palate which constitute one of the most common craniofacial anomalies. Various approaches to difficult airway management in an infant undergoing cleft repair have been described in the literature. However, no approach is considered ideal. It depends on age, associated anomalies, at hand resources, and the skill of the anesthesiologist. The unique anatomical and physiological features of pediatric airways impose additional challenges. The evolution of advanced airway modalities such as video laryngoscopes (VLs) and fiber-optic bronchoscopes had a major impact on the practice of anesthesia and the management of difficult airway scenarios in children and neonates. This article focuses on airway assessment and various current techniques and equipment used in airway management of cleft anomalies. Pediatric airway devices such as supraglottic airways, VLs, and fiber-optic bronchoscopes are briefly discussed with the benefits and limitations of each device. Recent studies describe specific problems as well as advantages with the usage of these devices.

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Introduction: Congenital cleft earlobe is a common congenital deformity of earlobe. Various techniques have been described for its repair, but cosmetic deformity usually persists. We present our result using triangular flap repair for simple longitudinal cleft of earlobe. Materials and Methods: The procedure was done in five cases who presented between 2017 and 2020. The technique uses the same principles as in Randall-Tennison triangular flap repair for cleft lip. Results: Earlobes were near-symmetrical, postoperative scar minimal and patient satisfaction was very good in all cases. Ear piercing was done without any complications at least 6 months after surgery. After a follow-up of 1 year, no complications were noted. Conclusions: Triangular flap repair technique for cleft earlobe follows the principles of Randall-Tennison triangular flap repair for cleft lip. It provides very good results with esthetic contour, minimal scar with no complications in case of simple longitudinal cleft.

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Children born with cleft lip/palate need regular dental care from birth till adulthood. Special feeding requirements, maintenance of oral hygiene, prevention and treatment of dental disease are essential components of oral healthcare provided by dental health care personnel. Malaligned teeth and dental anomalies should be addressed by orthodontics, guided by the developmental stage of the child. For the unmet need of dental care to be mitigated, it is essential that the core and coordinating teams be aware of the need for regular dental care visits. Each team needs to formulate sustainable oral health policies in their management protocol.

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Bilateral cleft lips have traditionally been a challenge to repair and the results have not been very favorable. However, advances in the recent past in the understanding of the nature of the deformity, especially the nasal component have brought a revolutionary change in the approach to these patients, with remarkable improvement in the outcome. The management of the premaxilla and its alignment with the maxillary shelves presurgically is crucial for the optimal treatment of such patients. The treatment protocol and technique followed at our center are described. It is a modification of the Mulliken technique. The philtrum is designed to be small in keeping with its rapid growth potential. The philtral flap is raised and the orbicularis oris muscles from the two sides are mobilized and brought together in the mid-line. Adequate mobilization of the muscles is necessary to avoid closure under tension. The evolution of the concept of primary rhinoplasty is described. The technique of preschool columellar lengthening and open rhinoplasty that is followed at our center is described. Unfavorable results include scarring, dehiscence, and central vermillion deficiency.

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Nasal deformities can vary in the form of severity. Several different factors such as congenital, burn and traumatic nasal fractures or soft-tissue injury/loss, neoplastic: defects resulting from surgery for the treatment of cancers causing compromise the structural architecture and functional framework of the nose. This case report describes the prosthetic rehabilitation of two female patients, one who was reported with congenital arrhinia and another one who had accidental electrical burns of the face. The surgeon's team as well as the maxillofacial prosthetics team discussed the treatment options with the parents and decided to rehabilitate with a nasal prosthesis. This clinical report is imparting an accessible and economic method for prosthetic rehabilitation of a patient.

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Introduction: There are only a few centers providing care to orofacial cleft patients across Nepal. However, no adequate data have been reported regarding the epidemiology, pattern, and association of cleft in children from eastern Nepal. The aim of the study was to study the epidemiological profile of cleft lip palate (CLP) patients reporting to the department of oral and maxillofacial surgery (OMFS) in Dharan, Eastern Nepal. Materials and Methods: Data retrospectively retrieved from the cleft registry in the department of OMFS were tabulated and entered into Microsoft Excel 2000. Data of all the diagnosed nonsyndromic orofacial cleft patients attending BP Koirala Institute of Health Sciences for treatment from August 2011 to August 2017 were used for the analysis. Incompletely filled records and syndromic cases were excluded. Results: Data of 220 orofacial clefts were analyzed which included 119 males and 101 females. Among these children with cleft, only 7 patients had direct family members and 18 patients had distant family members having the same condition. Among patients with CL (n = 151), left-sided CL (n = 85) was a more common presentation. Amongst patients with a cleft of the hard plate (n = 153), bilateral (n = 87) presentation was more common. A similar pattern was observed for cleft alveolus (n = 117) with the left side (n = 60) being common. In clefts affecting the soft palate (n = 192), complete cleft (n = 189) was a more common occurrence than incomplete. The majority of our patients had a CLP (n = 133) with isolated CL being the least commonly seen (n = 18). Conclusion: The majority of our patients had a CLP with isolated CL being the least common. This observation is in concordance with reports from other Caucasian and Asian populations both from developed and developing countries.

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We report a case of a patient with premature fusion of bilateral squamous sutures and perform a literature review of similar cases. We have noted in our case and in other reports that bilateral squamous synostosis does not lead to significant clinical effects such as deformed shape of the skull or raised intracranial pressure.

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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.

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Background: Suture removal in infants and children operated for cleft lip is one of the cumbersome task for the surgeons because of missing compliance. For this reason, tissue adhesives have gained popularity among the surgeons as well as the patients over the past two decades for skin wound closure. Although there are several published reports of case series using tissue adhesive for skin closure during cleft lip repair, very few have established its advantage over conventional suture technique. Objective: The objective of this study was to compare the esthetic outcome of skin closure in cleft lip repair with suture technique and tissue adhesive, with respect to the quality of scar. Methodology: A retrospective observational study was conducted by analyzing the photographic records of patients who underwent surgical repair of cleft lip between January 2015 and December 2017. The patients were divided into two groups, one for whom skin closure performed with 5-0 monofilament polyamide suture and the other with tissue adhesive. Esthetic outcome in terms of the quality of scar was assessed using a 5 point Likert's scale by 10 independent observers who remained blind of the surgeon performed the surgery, to reduce the assessor-related bias to minimum. The statistical analysis was performed using the SPSS version 27.0 and the difference between mean scores of both the groups was calculated. Results: A total of 70 patients were included in this study with 35 in each group. The mean follow-up period of adhesive and the suture group was 13.97 and 17.85 months, respectively. Overall mean of the total scores of the entire patient population was 3.92 (+/‒0.38). For the tissue adhesive group, the mean score was 3.88 (+/‒0.367) and for the suture group, the mean score was 3.96 (+/‒0.401). The difference between mean scores of individual parameters of both the groups as well as the difference between the overall mean scores of both the groups was not found to be statistically significant. Conclusion: The use of tissue adhesive for the closure of skin during cleft lip repair is as good as sutures, with respect to esthetic outcome of scar. Hence, any of these two techniques can be used for this purpose depending on the surgeon's preference.

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Objective: The objective of this study is to evaluate cleft patients, both unilateral and bilateral clefts and compare them with Skeletal base Class I patients for symmetry using orthopantomogram. Materials and Methods: Three groups of patients within age range of 15–25 years: Group I – Normal participants (mean age = 18.33 ± 2.84); Group II – Unilateral cleft lip and cleft palate participants (mean age = 18.74 ± 3.36); and Group III – Bilateral cleft lip and cleft palate participants (mean age = 17.64 ± 3.10) were assessed and compared to each other. Orthopantomogram was analyzed for vertical symmetry. Habets asymmetry index and the Kjellgren's asymmetry index were applied to assess the relative intensity of asymmetry. Results: Significant difference was seen in condylar height (P = 0.029), ramal height (P = 0.046), mandibular body length (P = 0.014), gonial angle (P = 0.035) and condylion–gonian distance (P = 0.044). Kjellberg's symmetry index/condylar ratio was maximally affected by the groups when compared with the Habets asymmetry index. Conclusion: Mandibular asymmetry has been expressed in unilateral cleft lip and palate (CLP) group. Normal group and bilateral CLP group indicated a symmetrical posterior vertical height of the mandible.

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Van der Woude syndrome is a rare congenital condition with autosomal dominant traits. This autosomal dominant syndrome had been reported to have a penetrance of 80% with variable expression.^[1] This genetic disorder is characterized by the combination of lip pits, cleft lip with or without cleft palate, and cleft palate only. Even within the families, there is a wide variability of affected gene expressions. It is very important to establish the correct diagnosis so that genetic counseling can be recommended in such cases. Here, we report cases of Van der Woude syndrome in the same family having the same genetic inheritance with variable expression.

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Orofacial clefts are common birth defects and Van der Woude syndrome (VWS) is the most common form of orofacial cleft syndrome, accounting for approximately 2% of patients with a cleft.^[1],[2] The cardinal features of VWS are lower lip pits associated with cleft lip and/or palate.^[2],[3],[4] Lip pits are reported to occur in over 80% of individuals with VWS.^[2] Most reported cases of VWS have been linked to chromosome 1q32-q41.^[5],[6],[7] The interferon regulatory factor 6 (IRF6) gene, which is located at 1q32-p41 region, has been implicated in several studies.^[2],[8] There are over 300 IRF6 variants that have been identified in patients with VWS, with approximately 50% of these being missense variants.^[9] This paper describes a 7.5-year-old male patient that is heterozygous for a missense variant in C.101A >C p.(Lys34Thr) which is likely to be pathogenic.^[9] This patient has the cardinal features for VWS but also has duodenal atresia. Neither the truncating variant identified in this patient nor other variants associated with VWS have been previously linked to duodenal atresia.

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Restoration of missing oral tissues in patients with cleft lip and palate is the biggest challenge in their rehabilitation. In this report, we described the postdistraction rehabilitation and 1-year follow-up of the regenerated bone by implant-supported prosthesis and orthodontic tooth movement. A novel distraction appliance regenerated natural bone which is compatible for both implant placement and orthodontic tooth movement, thereby restoring adequate function and esthetics.

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