Management of Velopharyngeal Dysfunction (VPD) Following Cleft Palate Repair: A Comprehensive Decision-Making Process Based on Severity and Structural Deficiencies.

OBJECTIVE: To describe a comprehensive decision-making process for surgical correction of structural Velopharyngeal Dysfunction (VPD) following cleft palate repair and evaluate its efficacy. DESIGN: Retrospective study. SETTING: Tertiary care hospital. PATIENTS: 300 consecutive patients with unilateral or bilateral cleft lip and palate (CLP) or isolated cleft palate (CP) diagnosed with clinical VPD following cleft palate repair between 2009 and 2014. Of these 206 patients had structural VPD and underwent surgical correction. INTERVENTIONS: Surgical corrections were carried out according to the comprehensive two stage decision making process developed by the investigators. Step 1 of decision-making involved visualisation of the VP sphincter function by nasoendoscopy. This was followed by step 2 which involved per-operative identification of scarring, tissue loss, hypoplasia and other structural deficiencies in the soft palate and septal mucoperiosteum. The choice of operation was then made from a repertoire of interrelated and escalating surgical procedures consisting of palate revision and pharyngoplasties ranging from most anatomical to the least. MAIN OUTCOME MEASURES: Evidence of postoperative restoration of VP function on nasoendoscopy, evaluation of speech for hypernasality, understandability, acceptability and symptoms of obstructive sleep apnea. RESULTS: Complete VP closure was demonstrated in 94% of patients treated using this algorithm. There was significant improvement in all speech parameters (p < 0.00001). CONCLUSION: Our comprehensive decision-making process is designed to effectively correct structural VPD according to the severity of structural and functional deficiencies in the soft palate and avoid over treatment.

Optimization of wear behavior of heat-treated Ti-6Al-7Nb biomedical alloy by response surface methodology.

Titanium-based metals are used most often in biomedical implant studies because they have good qualities like being biocompatible, not being poisonous, Osseo-integration, high specific properties, wear resistance, etc. The main goal of this work is to improve the wear resistance of Ti-6Al-7Nb biomedical metal by using a mix of Taguchi, ANOVA, and Grey Relational Analysis. The effect of changeable control process factors like applied load, spinning speed, and time on wear reaction measures like wear rate (WR), coefficient of friction (COF), and frictional force. The optimal combinations of wear rate, COF, and frictional force minimise wear characteristics. The L9 Taguchi orthogonal array was used to plan the experiments, which were done on a pin-on-disc set-up according to ASTM G99. To find the best set of control factors, Taguchi, ANOVA, and Grey relationship analysis were used. The results show that a load of 30 N, a speed of 700 rpm, and a time of 10 min are the best control settings.

The First Hybrid International Educational Comprehensive Cleft Care Workshop.

OBJECTIVE: Describe the first hybrid global simulation-based comprehensive cleft care workshop, evaluate impact on participants, and compare experiences based on in-person versus virtual attendance. DESIGN: Cross-sectional survey-based evaluation. SETTING: International comprehensive cleft care workshop. PARTICIPANTS: Total of 489 participants. INTERVENTIONS: Three-day simulation-based hybrid comprehensive cleft care workshop. MAIN OUTCOME MEASURES: Participant demographic data, perceived barriers and interventions needed for global comprehensive cleft care delivery, participant workshop satisfaction, and perceived short-term impact on practice stratified by in-person versus virtual attendance. RESULTS: The workshop included 489 participants from 5 continents. The response rate was 39.9%. Participants perceived financial factors (30.3%) the most significant barrier and improvement in training (39.8%) as the most important intervention to overcome barriers facing cleft care delivery in low to middle-income countries. All participants reported a high level of satisfaction with the workshop and a strong positive perceived short-term impact on their practice. Importantly, while this was true for both in-person and virtual attendees, in-person attendees reported a significantly higher satisfaction with the workshop (28.63 ± 3.08 vs 27.63 ± 3.93; P = .04) and perceived impact on their clinical practice (22.37 ± 3.42 vs 21.02 ± 3.45 P = .01). CONCLUSION: Hybrid simulation-based educational comprehensive cleft care workshops are overall well received by participants and have a positive perceived impact on their clinical practices. In-person attendance is associated with significantly higher satisfaction and perceived impact on practice. Considering that financial and health constraints may limit live meeting attendance, future efforts will focus on making in-person and virtual attendance more comparable.

A Normative Observational Study of the Relationship of the Philtral Ridges to the Columellar Base in South-Indian Population and its Significance on Cleft Lip Repair.

Introduction The philtral ridges form a prominent visual landmark in the upper lip. An aesthetically pleasing cleft lip repair should restore this preferably without any scars cutting across it. Although there are several scientific publications on morphology of this structure and its variations, very few studies on the Indian population have been published. Aim To study the morphology of the philtral ridges and their relationship to the columellar base in normal Indian children and its significance in cleft lip repair. Methods 115 normal healthy children from southern India aged between one and 12 years were studied based on direct observation of the relationship of the superior end of the philtral ridge to the columellar base and nasal sill. In type A, the philtral ridge terminates at the nasal sill just lateral to the columellar base, and in type B, it either reaches or fades before reaching the columellar base. All observations were performed by the first author by examining standardized two-dimensional (2D) photographs of the upper lip-nose complex. Results In this study, 74% of the subjects had the philtral column extending lateral to the columellar base. Conclusion These finding assume a significance since, in a substantial proportion of the studied population, techniques of cleft lip repair such as the rotation advancement method, place a scar that crosses the upper third of the philtral ridge which may be undesirable.

A Technical Innovation in Midface Advancement With the External Frame Distraction Device.

BACKGROUND: Maxillary advancement with a rigid external distractor (RED) II is a commonly performed procedure for correcting midface hypoplasia. While there are various methods of anchoring the osteotomized maxillary segment to the halo device, the looped transpyriform wire is the simplest and most cost-effective. However, a common complication with this is the cutting through of the stainless steel traction wire across the pyriform buttress during distraction. The patient with cleft undergoing midface distraction is particularly vulnerable since the force needed for distraction is higher due to scarring from previous operations. INNOVATION AND DESIGN: A single-hole titanium plate is introduced as an interface between the bone of the pyriform buttress and the looped stainless steel traction wire attached to the external frame device. EVALUATION: Twenty-four patients with cleft who underwent midface advancement using the external frame distractor before the introduction of the innovation were compared with 26 patients who underwent the same procedure after its introduction. The former group had 12.5% complication in the form of cut through of the traction wire, while the latter group had only 3.8% complication rate. CONCLUSION: Introduction of the titanium eyelet as an interface between the traction wire and the bone is a simple technical addition for a more reliable distraction using the RED II device in patients with cleft even in the presence of extensive scarring.

Management of post midface distraction occlusal discrepancy using temporary anchorage devices in a cleft patient.

Open bite deformity following a successful midface advancement by distraction osteogenesis is a common complication. Temporary anchorage devices can be deployed during the distraction and post-distraction settling phases for restoring the occlusion even in severe cases. The following report describes the management of severe anterior open bite following maxillary distraction.

A multi-centric, single-blinded, randomized, parallel-group study to evaluate the effectiveness of nasoalveolar moulding treatment in non-syndromic patients with complete unilateral cleft lip, alveolus and palate (NAMUC study): a study protocol for a randomized controlled trial.

BACKGROUND: Cleft lip and palate (CLP) are among the most common congenital anomaly that affects up to 33,000 newborns in India every year. Nasoalveolar moulding (NAM) is a non-surgical treatment performed between 0 and 6 months of age to reduce the cleft and improve nasal aesthetics prior to lip surgery. The NAM treatment has been a controversial treatment option with 51% of the cleft teams in Europe, 37% of teams in the USA and 25 of cleft teams in India adopting this methodology. This treatment adds to the already existing high burden of care for these patients. Furthermore, the supporting evidence for this technique is limited with no high-quality long-term clinical trials available on the effectiveness of this treatment. METHOD: The NAMUC study is an investigator-initiated, multi-centre, single-blinded randomized controlled trial with a parallel group design. The study will compare the effectiveness of NAM treatment provided prior to lip surgery against the no-treatment control group in 274 patients with non-syndromic unilateral complete cleft lip and palate. The primary endpoint of the trial is the nasolabial aesthetics measured using the Asher McDade index at 5 years of age. The secondary outcomes include dentofacial development, speech, hearing, cost-effectiveness, quality of life, patient perception, feeding and intangible benefits. Randomization will be carried out via central online system and stratified based on cleft width, birth weight and clinical trial site. DISCUSSION: We expect the results from this study on the effectiveness of treatment with NAM appliance in the long term along with the cost-effectiveness evaluation can eliminate the dilemma and differences in clinical care across the globe. TRIAL REGISTRATION: ClinicalTrials.gov CTRI/2022/11/047426 (Clinical Trials Registry India). Registered on 18 November 2022. The first patient was recruited on 11 December 2022. CTR India does not pick up on Google search with just the trial number. The following steps have to be carried out to pick up. How to search: ( https://ctri.nic.in/Clinicaltrials/advsearch.php -use the search boxes by entering the following details: Interventional trial > November 2022 > NAMUC).

Reducing the burden of orthodontic care for children with clefts: evaluating the effectiveness of pre-alveolar bone graft orthodontics in unilateral non-syndromic cleft patients (PABO study)- A study protocol for a multicentric randomised controlled trial.

BACKGROUND: An alveolar cleft commonly affects 75% of cleft lip and palate patients. While it is common practice to provide a course of orthodontic treatment before alveolar bone grafting, there are no previous high-quality studies reporting on the benefits of this type of treatment. AIM: The aim of the study is to evaluate the effectiveness of pre-alveolar bone graft orthodontics for unilateral non-syndromic cleft palate patients. METHOD: The PABO trial is a multicentric, parallel, two-arm, single-blinded randomised controlled trial. The inclusion criteria include unilateral cleft alveolus patients requiring bone graft and between the age group of 8 and 13 years with erupted upper central incisors. Participants will be recruited at three centres across India. Participants will be randomised to orthodontic treatment or no orthodontic treatment group. Both groups of participants will have alveolar bone graft surgery and will be followed up for 6 months after surgery. The primary outcome will be the success of the alveolar bone graft measured by anterior oblique radiograph and secondary outcomes include quality of life, cost analysis and quality of the dento-occlusal outcome. Data analysis will be carried out by an independent statistician at the end of the study. DISCUSSION: This study is the first to evaluate the effect of orthodontics on alveolar bone graft success. The increased burden of care for these patients with multiple treatments required from multiple specialists from birth to adult life highlights the need for reducing unnecessary treatment provision. TRIAL REGISTRATION: Clinical Trials Registry - India, CTRI/2020/10/028756 . Trial prospectively registered on 29 October 2020. .

Genetic variations at 10q26 regions near FGFR2 gene and its association with non-syndromic cleft lip with or without cleft palate.

OBJECTIVES: In our study, we focussed on three SNPs in the non-coding regions near FGFR2 gene, as studies on non-coding variants in the genome are the novel trends to identify the susceptible loci for nonsyndromic cleft lip with or without cleft palate (NSCL/P). FGFR2 gene is selected as a candidate gene based on knock out animal models and the role played in syndromic forms of clefting. FGFR2 gene also plays an important role in FGF signaling pathway during craniofacial development. METHODS: In the present study 148 case-parent triads were assessed for three SNPs rs10749408, rs11199874 and rs10788165 near FGFR2 gene by using TaqMan allelic discrimination method. Transmission disequilibrium test (TDT) was used to find the allelic association. Linkage disequilibrium (LD) between the markers was analysed using Haploview program 4.2. Haplotype transmission effects were estimated using FAMHAP package. The possible parent-of-origin effects were assessed by likelihood based approach. RESULTS: TDT analysis of three SNPs failed to show significant transmission disortion from heterozygous parents to the affected child and are not associated with NSCL/P. Linkage disequilibrium analysis showed strong LD between rs11199874 and rs10788165 SNPs. In the haplotype TDT analysis, GG haplotype of rs11199874-rs10788165 showed significant undertransmission to affected child. No significant parent-of-origin effects were observed. CONCLUSION: The present study on noncoding variants near FGFR2 gene is not associated with NSCL/P. As the numbers of triads included in the study are less, further studies are needed including large sample size to find association with NSCL/P.

Speech outcome following late primary palate repair.

OBJECTIVE: Early palate repair is recommended to minimize the development of disordered speech. We studied the speech outcome of late palate repair in 131 patients. The success of late palate repair is questioned because of the persistence of learned, compensatory misarticulations that are difficult to correct in spite of the establishment of correct palatal anatomy and a competent velopharyngeal mechanism. The objective of this study is to highlight the speech results following late primary repair of the palate. SETTINGS AND DESIGN: Retrospective analysis of speech outcomes in 131 patients with cleft lip and palate who underwent primary palate repair after the age of 10 years between November 2000 and December 2004. None of the patients had received supervised institution-based speech therapy. However, all patients were counseled and oriented and demonstrated the correct place and manner of articulation for the phonemes misarticulated by them. Preoperative and 6- to 12-month postoperative speech samples were assessed within the parameters of articulation, hypernasality, nasal air emission, and speech intelligibility. OUTCOME: The analysis indicated improvement in all speech parameters leading to an overall improvement in postoperative intelligibility for most patients. CONCLUSION: Although definite improvement occurs in all parameters of speech following late primary palate repair, residual speech problems persist in most patients, requiring further evaluation and appropriate treatment.

Role of sialic acid and sulfate groups in cervical mucus physiological functions: study of Macaca radiata glycoproteins.

The influence of charged groups in glycoproteins was investigated to assess their effect on the physiological functions of bonnet monkey cervical mucus. The macromolecular glycoproteins from peri-ovulatory, midcycle phase cervical mucus were treated with Pronase, trypsin and chymotrypsin and the enzyme-resistant glycoproteins purified by gel filtration on Sepharose 4B and a high molecular weight component containing carbohydrates, proteins and sulfate groups was recovered in high yield. This material still reacted with an antiserum directed against purified midcycle glycoprotein but not against another antiserum directed against luteal phase purified glycoproteins. Upon treatment with Pronase, trypsin and chymotrypsin, asialoglycoproteins and desulfated asialoglycoproteins released fragments of low molecular sizes, none of which reacted with the anti-midcycle glycoprotein antiserum. Cervical mucus collected from the estrogenic phase displayed a morphology supporting sperm migration, and this mucus retains the same morphology and reacts with the anti-midcycle glycoprotein antiserum following mild treatment with sialidase and subsequently with Pronase. These results imply that charged carbohydrate groups help maintain the structural and functional integrity of the mucus glycoprotein in its biological environment.

Polymorphic variants near 1p22 and 20q11.2 loci and the risk of non-syndromic cleft lip and palate in South Indian population.

BACKGROUND: Recent genome-wide association studies (GWAS) have reported multiple genetic risk loci for non-syndromic orofacial clefts (NSOFCs) in many populations. However, the contribution of these loci to NSOFC in India, which comprises one-fifth of the global population, is completely lacking. Our aim was to replicate the association of the SNPs located on 1p22 chromosomal loci (rs540026, rs481931) and 20q11.2 (rs13041247, rs11696257) in the aetiology of NSOFCs, in South Indian populations. METHODS: The SNPs were genotyped by using KBiosciences KASPar SNP genotyping chemistry in 173 cases and 176 controls for NSOFCs in South India. To estimate the association between these SNPs and NSOFCs, chi-square test was adopted. Odds ratios (OR) with 95% confidence intervals (CI) were also calculated in order to assess the risk. RESULTS: Single nucleotide polymorphisms located at chromosomal region 1p22 are not found to be associated with cleft lip with or without non-syndromic cleft palate (NSCL/P) and non-syndromic cleft palate only (NSCPO) at either the genotype or allele levels. Further, there is no LD observed between these variants. The polymorphic variants near 20q11.2 (rs13041247, rs11696257) are in complete linkage disequilibrium (LD) and are significantly associated with only NSCL/P in genotypic (p=0.013) and allelic models (p=0.029). In the genotypic model significance persisted even after Bonferroni correction (p<0.016). CONCLUSION: These results suggest that 20q11.2 SNPs could play a contributory role in the pathophysiology and risk of NSCL/P, while the variations in 1p22 do not underlie the pathophysiology of NSOFCs in South Indian populations.

External frame distraction osteogenesis of the midface in the cleft patient.

Distraction osteogenesis has established itself as an accepted form of treatment in the management of midface deficiency in cleft patients. However, it is well known that some amount of relapse is inevitable in patients who undergo this procedure. Like most surgical techniques, it has its specific indications, limitations, and complications. The problems are amplified in some patients because of severe fibrosis resulting from previous palate and lip operations. This article reviews treatment planning, pre- and postoperative orthodontic management, operative technique, and mechanics of distraction. It also discusses long-term changes following distraction and protocols to optimize the results and minimize complications.

A comparison of bilateral infraorbital nerve block with intravenous fentanyl for analgesia following cleft lip repair in children.

BACKGROUND: The efficacy of analgesia with bilateral infraorbital nerve block and intravenous (i.v.) fentanyl were compared for cleft lip surgery in children. METHODS: Eighty-two children aged 3 months to 10 years undergoing cleft lip repair were prospectively randomized to one of two groups: bilateral infraorbital nerve block (Group B), or i.v. fentanyl (Group F). Group B (n = 41) received bilateral infraorbital injection of 1 ml 0.25% bupivacaine and 2 ml i.v. saline as control. Group F (n = 41) received 2 microg x kg(-1) i.v. fentanyl, and bilateral infraorbital injection of 1 ml saline as control. Pain was evaluated by the incidence of tachycardia, hypertension, and/or modified pain score > or =4. The time to awakening, time to first cry and time to feeding were noted. RESULTS: Thirty four children (82.9%) in Group B had adequate analgesia compared with 15 (36.6%) in Group F (P < 0.0001, RR of failure 0.27 for Group B). Group B had a mean time to awakening of 5.65 +/- 2.52 min (Group F: 9.37 +/- 4.50 min; P < 0.0001), time to first cry 32.14 +/- 18.22 min (Group F: 28.00 +/- 16.27 min; P = 0.3), time to feed 62.05 +/- 20.06 min (Group F: 72.44 +/- 17.72; P = 0.015), and pain score 2.81 +/- 1.38 (Group F: 4.71 +/- 1.89; P < 0.0001). There were no major complications. CONCLUSIONS: Bilateral infraorbital block is superior to fentanyl in terms of analgesia, and time to awakening and feeding.

Synchronous palatal closure and premaxillary setback in older children with bilateral complete cleft of lip and palate.

BACKGROUND: Achieving good results after primary repair in older children with complete bilateral cleft lip and palate is a Herculean task, particularly when the premaxilla is protruding and twisted. The authors faced the problems of protruding premaxilla in children older than 5 years of age with bilateral complete cleft lip and palate who presented late for primary repairs. Due to financial constraints and the distances traveled, these patients prefer to come for fewer procedures with maximum benefits. These factors encouraged the authors to carry out synchronous palatal closure and premaxillary setback in older children with bilateral complete cleft of the lip and palate. METHODS: Synchronous palatal repair and premaxillary setback were carried out in 14 patients. During palate repair, good exposure of the vomer made premaxillary setback easy without compromising the blood supply to it. The premaxilla, after osteotomy, was immobilized in all patients using the simple technique of Kirschner wire fixation; an additional gingivoperioplasty was performed in a few patients. Palatal closure was achieved with two flap techniques with intravelar veloplasty. RESULTS: Proper positioning of the premaxilla was achieved in all patients, with good labial repair 6 months after the primary palate closure. There were no major complications, such as loss of the premaxilla or vascular compromise. Two patients had an anterior open bite and one patient had a postalveolar fistula. CONCLUSIONS: This technique had a few minor complications, but the overall surgical outcome was satisfactory. This protocol proved advantageous in achieving good results with fewer procedures and in reducing the total expenditures and the length of the patient's hospital stay.

Anesthesia for cleft lip surgery in a child with Seckel syndrome--a case report.

We describe the anesthetic management of a 5-year-old girl diagnosed with Seckel syndrome, for cleft lip surgery. The syndrome is characterized by severe intrauterine and postnatal growth retardation with proportional dwarfism, typical beak-like triangular nose, and mental retardation, accounting for its various synonyms such as bird-headed dwarfism, microcephalic primordial dwarfism, nanocephalic dwarfism and Seckel-type dwarfism.