OBJECTIVE: After cleft lip and/or palate (CL/P) repair, children may develop velopharyngeal insufficiency (VPI) leading to speech imperfections, necessitating additional speech correcting surgery. This study examines the incidence of VPI and speech correcting surgery after Sommerlad\'s palatoplasty for CL/P, and its association with various clinical features.
METHODS: A retrospective cohort study was performed in the Wilhelmina Children\'s Hospital in Utrecht and child records from 380 individuals with CL/P registered from 2008 to 2017 were retrospectively reviewed. Inclusion criteria comprised the diagnosis of CL/P, primary palatoplasty according to Sommerlad\'s technique, and speech assessment at five years or older. Association between cleft type and width, presence of additional genetic disorders and postoperative complications (palatal dehiscence, fistula) were assessed using odds ratios and chi squared tests.
RESULTS: A total of 239 patients were included. The VPI rate was 52.7% (n = 126) and in 119 patients (49.8%) a speech correcting surgery was performed. Severe cleft type, as indicated by a higher Veau classification, was associated with a significant higher rate of speech correcting surgeries (p = 0.033). Significantly more speech correcting surgeries were performed in patients with a cleft width >10 mm, compared to patients with a cleft width ≤10 mm (p < 0.001). Patients with oronasal fistula underwent significantly more speech correcting surgeries than those without fistula (p = 0.004). No statistically significant difference was found in the incidence of speech correcting surgery between patients with and without genetic disorders (p = 0.890).
CONCLUSIONS: Variations in cleft morphology, cleft width and complications like oronasal fistula are associated with different speech outcomes. Future research should focus on creating a multivariable prediction model for speech correcting surgery in CL/P patients.

OBJECTIVE: We aim to verify velopharyngeal sphincter function in 22q11.2 deletion syndrome patients (22q11.2DS) to establish correlations between aerodynamic and perceptual measures of nasality, and to identify aerodynamic measures differentiating typical from atypical velopharyngeal behavior.
METHODS: Eleven subjects with 22q11.2DS and twenty similar-age control subjects were recruited. The aerodynamic measures were mean Sound Pressure Level, air pressure peak, pressure wave duration, airflow pattern and nasal airflow during the sequence /pi/. The nasality perceptual measures were rhinolalia, rhinophony and nasal air escape.
RESULTS: Airflow patterns and perceptual measures were statistically different in the two groups. Pressure wave duration and air pressure peak were lower in study subjects than in controls. Air pressure peak and nasal airflow were negatively correlated with rhinolalia; pressure wave duration was negatively correlated with nasal air escape and rhinolalia in 22q11.2DS patients.
CONCLUSIONS: This aerodynamic study identified velopharyngeal qualitative and quantitative dysfunctions, suggesting heterogeneous models of velopharyngeal function in syndromic subjects as compared to controls.

Velopharyngeal insufficiency is a disorder where the soft palate directs the air through the nose. It is often present in patients with previous cleft or short palate, but also in many other conditions. Symptoms are primarily to be found in speech, with very distinct nasal sound. After clinical evaluation and nasal endoscopy, surgery is considered. Several surgical techniques are in use, with posterior pharyngeal flap pharyngoplasty being most widely used. This method leaves the base of the posterior pharyngeal flap attached to the posterior pharyngeal wall, with two lateral ports on each side of the flap. Permanent nasopharyngeal obstruction is a very challenging pathology for anesthesiologists in case of mandatory nasal intubation since it is a relative contraindication for nasal intubation. Patients with previous palatoplasty will regularly appear in our routine anesthetic practice, in all surgical segments. The high risk of damage to the flap with possible bleeding can put the anesthesiologist in a very unpleasant situation if not aware of the permanent effect of this surgery. During preanesthetic assessment, if there is information on a previous pharyngoplasty, one should consider alternative options for nasotracheal intubation. All nasal insertion procedures must be either avoided or carried out with great caution, under fiberoptic visual control.

Background  Velopharyngeal insufficiency (VPI) is a major complication of cleft palate repair. The purpose of this study was to evaluate the incidence and predictive factors of VPI after cleft palate repair based on 27 years of one surgeon\'s experience. Methods  Medical records were retrospectively reviewed for 652 patients who underwent cleft palate repair between 1995 and 2021. After exclusion of those with other syndromes or developmental disorders, the study included 374 patients with sufficient follow-up until the age of 4 years, when language evaluation was possible. VPI status was categorized through subjective and objective tests into normal, VPI, and borderline. We analyzed potential differences in VPI incidence by multiple factors. Factors with significance were analyzed to confirm the relationships between subvariables. Results  Of the 374 patients, 311 (83.2%) exhibited normal pronunciation, 51 (13.6%) had VPI, and 12 (3.2%) were borderline. Primary cleft palate repair performed after 18 months was associated with a higher incidence of VPI than repair conducted before 18 months ( p  = 0.005). The incidence of VPI was higher in cases of submucous cleft palate than in the other types based on the Veau classification ( p  = 0.011). However, in the multivariable analysis, only the submucous type showed statistically significant results ( p  = 0.026). Conclusion  A total of 374 people underwent primary cleft palate repair, and 13.6% of those with VPI required secondary therapy. The incidence of VPI was relatively high among patients with primary cleft palate repair after 18 months and patients with submucous cleft palate.

UNASSIGNED: During articulation the velopharynx needs to be opened and closed rapidly and a tight closure is needed. Based on the hypothesis that patients with cleft lip and palate (CLP) produce lower pressures in the velopharynx than healthy individuals, this study compared pressure profiles of the velopharyngeal closure during articulation of different sounds between healthy participants and patients with surgically closed unilateral CLP (UCLP) using high resolution manometry (HRM).
UNASSIGNED: Ten healthy adult volunteers (group 1: 20-25.5 years) and ten patients with a non-syndromic surgically reconstructed UCLP (group 2: 19.1-26.9 years) were included in this study. Pressure profiles during the articulation of four sounds (/i:/, /s/, /ʃ/ and /n/) were measured by HRM. Maximum, minimum and average pressures, time intervals as well as detection of a previously described 3-phase-model were compared.
UNASSIGNED: Both groups presented with similar pressure curves for each phoneme with regards to the phases described and pressure peaks, but differed in total pressures. An exception was noted for the sound /i:/, where a 3-phase-model could not be seen for most patients with UCLP. Differences in velopharynx pressures of 50% and more were found between the two groups. Maximum and average pressures in the production of the alveolar fricative reached statistical significance.
UNASSIGNED: It can be concluded that velopharyngeal pressures of patients with UCLP are not sufficient to eliminate nasal resonance or turbulence during articulation, especially for more complex sounds. These results support a general understanding of hypernasality during speech implying a (relative) velopharyngeal insufficiency.
UNASSIGNED: Während der Artikulation öffnet und schließt sich der velopharyngeale Abschluss in schnellen Bewegungsabfolgen und für die Bildung einiger Laute ist ein vollständiger Abschluss notwendig. Für Patient_innen mit Lippen-, Kiefer-, Gaumenspalte (LKG) ist bekannt, dass die Verschlussdrücke im Velopharynx beim Schlucken reduziert sind. Ziel dieser Studie war es, die Druckprofile von Patient_innen mit unilateraler LKG und gesunden Proband_innen mittels Hochauflösungsmanometrie (HRM) zu vergleichen und zu evaluieren, ob bei der Produktion verschiedener Sprachlaute ebenfalls Druckunterschiede im Velopharynx bestehen.
UNASSIGNED: Es wurden 10 gesunde Erwachsene (Gruppe 1: 20–25,5 Jahre) und 10 Patient_innen mit einer nicht-syndromalen operativ verschlossenen unilateralen LKG (Gruppe 2: 19,1–26,9 Jahre) untersucht. HRM-Verschlussdruckprofile wurden während der Artikulation von 4 Sprachlauten (/i:/, /s/, /ʃ/ und /n/) aufgezeichnet. Es wurden Maximal-, Minimal- und Durchschnittsdrücke sowie Zeitintervalle ermittelt. Zudem wurden die Druckprofile mit einem zuvor beschriebenen 3-Phasen-Phonationsmodell verglichen.
UNASSIGNED: In beiden Gruppen zeigten sich in Bezug auf die Phonationsphasen ähnliche Druckprofile für die produzierten Laute, allerdings lagen unterschiedliche Gesamtdrücke vor. Eine Ausnahme stellte die Realisation des Lautes /i:/ dar, bei der sich bei den meisten Patient_innen mit LKG keine drei Phonationsphasen erkennen ließen. Zwischen den beiden Gruppen existierten Druckunterschiede in der Velopharynxregion von 50% und mehr. Es fanden sich statistisch signifikante Unterschiede bei den Maximaldruck- und durchschnittlichen Druckwerten bei der Produktion von alveolaren Frikativen.
UNASSIGNED: Die reduzierten velopharyngealen Verschlussdrücke bei Patient_innen mit LKG tragen wahrscheinlich dazu bei, dass nasale Resonanzen und Turbulenzen nicht ausreichend eliminiert und komplexere Laute dadurch nicht korrekt gebildet werden. Die hier erhobenen Ergebnisse unterstützen die Annahme, dass die bei Patient_innen mit LKG häufig beobachtete Hypernasalität während der Sprachproduktion auf eine (relative) velopharyngeale Insuffizienz zurückzuführen ist.

UNASSIGNED: Rhinophonia aperta may result from velopharyngeal insufficiency. Neuromuscular electrical stimulation (NMES) has been discussed in the context of muscle strengthening. The aim of this study was to evaluate in healthy subjects whether NMES can change the velopharyngeal closure pattern during phonation and increase muscle strength.
UNASSIGNED: Eleven healthy adult volunteers (21-57 years) were included. Pressure profiles were measured by high resolution manometry (HRM): isolated sustained articulation of /a/ over 5 s (protocol 1), isolated NMES applied to soft palate above motor threshold (protocol 2) and combined articulation with NMES (protocol 3). Mean activation pressures (MeanAct), maximum pressures (Max), Area under curve (AUC) and type of velum reactions were compared. A statistical comparison of mean values of protocol 1 versus protocol 3 was carried out using the Wilcoxon signed rank test. Ordinally scaled parameters were analyzed by cross table.
UNASSIGNED: MeanAct values measured: 17.15±20.69 mmHg (protocol 1), 34.59±25.75 mmHg (protocol 3) on average, Max: 37.86±49.17 mmHg (protocol 1), 87.24±59.53 mmHg (protocol 3) and AUC: 17.06±20.70 mmHg.s (protocol 1), 33.76±23.81 mmHg.s (protocol 3). Protocol 2 produced velum reactions on 32 occasions. These presented with MeanAct values of 13.58±12.40 mmHg, Max values of 56.14±53.14 mmHg and AUC values of 13.84±12.78 mmHg.s on average. Statistical analysis comparing protocol 1 and 3 showed more positive ranks for MeanAct, Max and AUC. This difference reached statistical significance (p=0.026) for maximum pressure values.
UNASSIGNED: NMES in combination with articulation results in a change of the velopharyngeal closure pattern with a pressure increase of around 200% in healthy individuals. This might be of therapeutic benefit for patients with velopharyngeal insufficiency.
UNASSIGNED: Offene Rhinophonien können aufgrund einer velopharyngealen Insuffizienz entstehen. Die neuromuskuläre Elektrostimulation (NMES) wird seit einiger Zeit als Therapieform zur Muskelkräftigung angewendet. Ziel dieser Studie war es, an gesunden Proband_innen zu untersuchen, ob NMES das velopharygeale Verschlussmuster während der Phonation verändern und die Muskelkraft erhöhen kann.
UNASSIGNED: In die Studie wurden 11 gesunde Proband_innen eingeschlossen (21–57 Jahre). Mit der Hochauflösungsmanometrie wurden Druckprofile unter verschiedenen Bedingungen aufgezeichnet: die isolierte Phonation des Lautes /a/ über 5 s (Protokoll 1), die isolierte, motorisch überschwellig applizierte NMES auf das Velum (Protokoll 2) sowie die Phonation und die NMES in Kombination (Protokoll 3). Verglichen wurden der durchschnittliche Druck während der Aktivierung (MeanAct), die durchschnittlichen Maximaldrücke (Max), die durchschnittlichen Integrale unter der Druckkurve (Area under curve (AUC)) und die Art der Velumreaktion. Der Vergleich der Durchschnittswerte von Protokoll 1 und 3 wurden mithilfe des Wilcoxon-Vorzeichen-Rang-Tests durchgeführt. Ordinär skalierte Parameter wurden über eine Kreuztabelle analysiert.
UNASSIGNED: MeanAct Mittelwerte lagen bei 17,15±20,69 mmHg (Protokoll 1), 34,59±25,75 mmHg (Protokoll 3), Max bei 37,86±49,17 mmHg (Protokoll 1), 87,24±59,53 mmHg (Protokoll 3) und AUC bei 17,06±20,70 mmHg.s (Protokoll 1), 33.76±23.81 mmHg.s (Protokoll 3). Im Protokoll 2 zeigten sich 32 Velumreaktionen mit durchschnittlichen MeanAct-Werten von 13,58±12,40 mmHg, Maximalwerten von 56,14±53,14 mmHg und Mean-AUC-Werten von 13,84±12,78 mmHg.s. Der Vergleich der Protokolle 1 und 3 ergab mehr positive Ränge für die Kombination aus Phonation und NMES und für die Parameter MeanAct, Max und AUC. Dieser Unterschied zeigte mit p=0.026 statistische Signifikanz in Bezug auf den Parameter Max.
UNASSIGNED: NMES in Kombination mit Phonation führt bei Gesunden zu einer Veränderung des velopharyngealen Verschlussmusters mit einer Druckerhöhung von etwa 200%. Dies könnte für Patient_innen mit velopharyngealer Insuffizienz von therapeutischem Nutzen sein.

OBJECTIVE: The main purpose of this study was evaluation of the effectiveness of secondary furlow palatoplasty with buccal myomucosal flap (FPBF) for the treatment of velopharyngeal insufficiency (VPI) in patients with a cleft palate who were treated with two flap palatoplasty (TFP) in their primary palate repair.
METHODS: Twenty-three medically free children aged 4-8 years with non-syndromic and previously repaired cleft palate via TFP participated in the study. All patients received secondary surgery following the technique of FPBF. Preoperative speech evaluation was done before the secondary repair and 3 months after the surgery using a hypernasal speech scale, speech intelligibility scale, and nasopharyngoscopy.
RESULTS: A statistically significant improvement was observed regarding the degree of hypernasality and speech intelligibility while comparing the preoperative scores after the primary surgery to the postoperative scores after the secondary surgery. In addition, a statistically significant improvement was found in the nasopharyngoscopic assessment.
CONCLUSIONS: The incorporation of a buccal myomucosal flap with Furlow palatoplasty was successful in improving hypernasality, speech intelligibility, and nasopharyngoscopic scores in patients with cleft palate.
BACKGROUND: clinicaltrials.gov (NCT05626933).
CONCLUSIONS: This technique might be the surgical technique of choice while treating patients who are suffering from VPI after cleft palate repair.

OBJECTIVE: The clinical effects and surgical procedures of Hogan posterior pharyngeal flap in the treatment of the older patients with velopharyngeal insufficiency (VPI) after cleft palate repair were investigated.
METHODS: A total of 33 patients (aged 10-35 years; average of 20.4 years) with VPI secondary to cleft palate were included. They underwent Hogan posterior pharyngeal flap to improve velopharyngeal closure function. The clinical efficacy of the ope-ration was evaluated with Chinese speech clarity measurement and nasopharyngeal fiberscope (NPF), and the velopharyngeal closure was graded. The average follow-up time was 13.3 months.
RESULTS: The wounds of all patients were healed by first intention, and speech assessment showed that the consonant articulation increased and the rate of hypernasality and nasal emission decreased significantly (P<0.05). NPF examination showed that the postoperative velopharyngeal closure function significantly improved, 30 cases (91%) were gradeⅠ, and 3 cases (9%) were grade Ⅱ.
CONCLUSIONS: Hogan posterior pharyngeal flap for VPI secondary to cleft palate can significantly improve velopharyngeal closure.
目的: 探讨使用Hogan法咽后壁组织瓣转移术治疗大龄腭咽闭合不全（VPI）患者的临床疗效。方法: 收集33例腭裂术后VPI患者，年龄10~35岁，平均年龄20.4岁。所有患者均行Hogan法咽后壁组织瓣转移术治疗腭咽闭合不全。采用汉语语音清晰度测定法评估患者语音情况，鼻咽纤维镜（NPF）评估腭咽闭合程度，并进行分级。平均随访时间13.3个月。结果: 33例患者术后创口均达到Ⅰ期愈合。术后语音清晰度明显提高，鼻漏气及高鼻音减少，与术前相比差异有统计学意义（P<0.05）。NPF检查示，术后腭咽闭合功能明显改善，30例（91%）患者腭咽闭合率达到Ⅰ级，3例（9%）患者达到Ⅱ级。结论: Hogan法咽后壁组织瓣转移术可显著改善大龄VPI患者的腭咽闭合状况，减少鼻漏气和高鼻音。.

Speech is the most basic yet invaluable mode of expression for an individual. Alterations in speech can have vast effects on the psychological well-being of a person, hampering social interactions. Congenital or traumatic defects of the hard and soft palate result in velopharyngeal dysfunction, which often results in abnormal and aberrant speech. Apart from these, it is also a common outcome following surgical repair of cleft palate. Prosthodontic management of such cases with velopharyngeal obturators to improve speech and function is well documented and known to give optimal results. In this case report, we are presenting the rehabilitation of residual velopharyngeal insufficiency post-cleft palate closure using a speech bulb prosthesis attached to a complete denture. As the speech bulb would add to the weight of the existing prosthesis, a hollow complete denture was planned. The prosthesis resulted in a decrease in nasal air emissions and hypernasality, thus improving the patient\'s communication skills and overall quality of life.

The condition known as 22q11.2 deletion syndrome (MIM #188400) is a rare disease with a highly variable clinical presentation including more than 180 features; specific guidelines for screening individuals have been used to support clinical suspicion before confirmatory tests by Brazil\'s Craniofacial Project. Of the 2568 patients listed in the Brazilian Database on Craniofacial Anomalies, 43 individuals negative for the 22q11.2 deletion syndrome were further investigated through whole-exome sequencing. Three patients (6.7%) presented with heterozygous pathogenic variants in the KMT2A gene, including a novel variant (c.6158+1del) and two that had been previously reported (c.173dup and c.3241C>T); reverse phenotyping concluded that all three patients presented features of Wiedemann-Steiner syndrome, such as neurodevelopmental disorders and dysmorphic facial features (n = 3), hyperactivity and anxiety (n = 2), thick eyebrows and lower-limb hypertrichosis (n = 2), congenital heart disease (n = 1), short stature (n = 1), and velopharyngeal insufficiency (n = 2). Overlapping features between 22q11.2 deletion syndrome and Wiedemann-Steiner syndrome comprised neuropsychiatric disorders and dysmorphic characteristics involving the eyes and nose region; velopharyngeal insufficiency was seen in two patients and is an unreported finding in WDSTS. Therefore, we suggest that both conditions should be included in each other\'s differential diagnoses.