Timing of Primary Surgery for Cleft Palate.

BACKGROUND: Among infants with isolated cleft palate, whether primary surgery at 6 months of age is more beneficial than surgery at 12 months of age with respect to speech outcomes, hearing outcomes, dentofacial development, and safety is unknown. METHODS: We randomly assigned infants with nonsyndromic isolated cleft palate, in a 1:1 ratio, to undergo standardized primary surgery at 6 months of age (6-month group) or at 12 months of age (12-month group) for closure of the cleft. Standardized assessments of quality-checked video and audio recordings at 1, 3, and 5 years of age were performed independently by speech and language therapists who were unaware of the trial-group assignments. The primary outcome was velopharyngeal insufficiency at 5 years of age, defined as a velopharyngeal composite summary score of at least 4 (scores range from 0 to 6, with higher scores indicating greater severity). Secondary outcomes included speech development, postoperative complications, hearing sensitivity, dentofacial development, and growth. RESULTS: We randomly assigned 558 infants at 23 centers across Europe and South America to undergo surgery at 6 months of age (281 infants) or at 12 months of age (277 infants). Speech recordings from 235 infants (83.6%) in the 6-month group and 226 (81.6%) in the 12-month group were analyzable. Insufficient velopharyngeal function at 5 years of age was observed in 21 of 235 infants (8.9%) in the 6-month group as compared with 34 of 226 (15.0%) in the 12-month group (risk ratio, 0.59; 95% confidence interval, 0.36 to 0.99; P = 0.04). Postoperative complications were infrequent and similar in the 6-month and 12-month groups. Four serious adverse events were reported (three in the 6-month group and one in the 12-month group) and had resolved at follow-up. CONCLUSIONS: Medically fit infants who underwent primary surgery for isolated cleft palate in adequately resourced settings at 6 months of age were less likely to have velopharyngeal insufficiency at the age of 5 years than those who had surgery at 12 months of age. (Funded by the National Institute of Dental and Craniofacial Research; TOPS ClinicalTrials.gov number, NCT00993551.).

Evaluation of noise excitation as a method for detection of hypernasality.

Hypernasality is a disorder where excess nasal resonance is perceived during speech, often as a result of abnormal coupling between the oral and nasal tracts known as velopharyngeal insufficiency (VPI). The most common cause of VPI is a cleft palate, which affects around 1 in 1650 babies, around ⅓ of whom have persistent speech problems after surgery. Current equipment-based assessment methods are invasive and require expert knowledge, and perceptual assessment methods are limited by the availability of expert listeners and differing interpretations of assessment scales. Spectral analysis of hypernasality within the academic community has resulted in potentially useful spectral indicators, but these are highly variable, vowel specific, and not commonly used within clinical practice. Previous works by others have developed noise excitation technologies for the measurement of oral tract transfer functions using resonance measurement devices (RMD). These techniques provide an opportunity to investigate the structural system abnormalities which lead to hypernasality, without the need for invasive measurement equipment. Thus, the work presented in this study adapts these techniques for the detection of hypernasality. These adaptations include augmentation of the hardware and development of the software, so as to be suitable for transfer function measurement at the nostrils rather than the mouth (nRMD). The new method was tested with a single participant trained in hypernasal production, producing 'normal' and hypernasal vowels, and the recordings validated through a listening test by an expert listener and calculation of nasalance values using a nasality microphone. These validation stages indicated the reliability of the captured data, and analysis of the nRMD measurements indicated the presence of a systematic difference in the frequency range 2 to 2.5 kHz between normal and hypernasal speech. Further investigation is warranted to determine the generalisability of these findings across speakers, and to investigate the origins of differences manifesting in the transfer functions between conditions. This will provide new insights into the effects of nasal tract coupling on voice acoustics, which could in turn lead to the development of useful new tools to support clinicians in their work with hypernasality.

Age at Primary Cleft Lip Repair: A Potential Bellwether Indicator for Pediatric Surgery.

The bellwether procedures described by the Lancet Commission on Global Surgery represent the ability to deliver adult surgical services after there is a clear and easily made diagnosis. There is a need for pediatric surgery bellwether indicators. A pediatric bellwether indicator would ideally be a routinely performed procedure, for a relatively common condition that, in itself, is rarely lethal at birth, but that should ideally be treated with surgery by a standard age. Additionally, the condition should be easy to diagnose, to minimize the confounding effects of delays or failures in diagnosis. In this study, we propose the age at primary cleft lip (CL) repair as a bellwether indicator for pediatric surgery. METHOD: We reviewed the surgical records of 71,346 primary cleft surgery patients and ultimately studied age at CL repair in 40,179 patients from 73 countries, treated by Smile Train partners for 2019. Data from Smile Train's database were correlated with World Bank and WHO indicators. RESULTS: Countries with a higher average age at CL repair (delayed access to surgery) had higher maternal, infant, and child mortality rates as well as a greater risk of catastrophic health expenditure for surgery. There was also a negative correlation between delayed CL repair and specialist surgical workforce numbers, life expectancy, percentage of deliveries by C-section, total health expenditure per capita, and Lancet Commission on Global Surgery procedure rates. CONCLUSION: These findings suggest that age at CL repair has potential to serve as a bellwether indicator for pediatric surgical capacity in Lower- and Middle-income Countries.

Prioritizing Cleft/Craniofacial Surgical Care after the COVID-19 Pandemic.

BACKGROUND: It is anticipated that in due course the burden of emergency care due to COVID-19 infected patients will reduce sufficiently to permit elective surgical procedures to recommence. Prioritizing cleft/craniofacial surgery in the already overloaded medical system will then become an issue. The European Cleft Palate Craniofacial Association, together with the European Cleft and Craniofacial Initiative for Equality in Care, performed a brief survey to capture a current snapshot during a rapidly evolving pandemic. METHODS: A questionnaire was sent to the 2242 participants who attended 1 of 3 recent international cleft/craniofacial meetings. RESULTS: The respondents indicated that children with Robin sequence who were not responding to nonsurgical options should be treated as emergency cases. Over 70% of the respondents indicated that palate repair should be performed before the age of 15 months, an additional 22% stating the same be performed by 18 months. Placement of middle ear tubes, primary cleft lip surgery, alveolar bone grafting, and velopharyngeal insufficiency surgery also need prioritization. Children with craniofacial conditions such as craniosynostosis and increased intracranial pressure need immediate care, whilst children with craniosynostosis and associated obstructive sleep apnea syndrome or proptosis need surgical care within 3 months of the typical timing. Craniosynostosis without signs of increased intracranial pressure needs correction before the age of 18 months. CONCLUSIONS: This survey indicates several areas of cleft and craniofacial conditions that need prioritization, but also certain areas where intervention is less urgent. We acknowledge that there will be differences in the post COVID-19 response according to circumstances and policies in individual countries.

Longitudinal 3D Assessment of Facial Asymmetry in Unilateral Cleft Lip and Palate.

OBJECTIVE: Longitudinal evaluation of asymmetry of the surgically managed unilateral cleft lip and palate (UCLP) to assess the impact of facial growth on facial appearance. DESIGN: Prospective study. SETTING: Glasgow Dental Hospital and School, University of Glasgow, United Kingdom. PATIENTS: Fifteen UCLP infants. METHOD: The 3-D facial images were captured before surgery, 4 months after surgery, and at 4-year follow-up using stereophotogrammetry. A generic mesh which is a mathematical facial mask that consists of thousands of points (vertices) was conformed on the generated 3-D images. Using Procustean analysis, an average facial mesh was obtained for each age-group. A mirror image of each average mesh was mathematically obtained for the analysis of facial dysmorphology. Facial asymmetry was assessed by measuring the distances between the corresponding vertices of the original and the mirror copy of the conformed meshes, and this was displayed in color-coded map. RESULTS: There was a clear improvement in the facial asymmetry following the primary repair of cleft lip. Residual asymmetry was detected around the nasolabial region. The nasolabial region was the most asymmetrical region of the face; the philtrum, columella, and the vermillion border of the upper lip showed the maximum asymmetry which was more than 5 mm. Facial growth accentuated the underlying facial asymmetry in 3 directions; the philtrum of the upper lip was deviated toward the scar tissue on the cleft side. The asymmetry of the nose was significantly worse at 4-year follow-up ( P < .05). CONCLUSION: The residual asymmetry following the surgical repair of UCLP was more pronounced at 4 years following surgery. The conformed facial mesh provided a reliable and innovative tool for the comprehensive analysis of facial morphology in UCLP. The study highlights the need of refining the primary repair of the cleft and the potential necessity for further corrective surgery.

Assessment of regional asymmetry of the face before and after surgical correction of unilateral cleft lip.

This study was carried out on 26 unilateral cleft lip and palate (UCLP) cases with mean age 3.6 ± 0.7 months.3D facial images were captured for each infant 2-3 days before the repair of cleft lip and at 4 months following surgery at a mean age of 8.2 ± 1.8 months, using a stereophotogrammetry imaging system. An iterative closest point (ICP) algorithm was used to superimpose the 3D facial model to its mirror image using VRMesh software. After the superimposition, the face model was divided into seven anatomical regions. Asymmetry of the entire face and of the anatomical regions was calculated by measuring the absolute distances between the 3D facial surface model and its mirror image. Colour maps were used to illustrate the patterns and magnitude of the facial asymmetry before and after surgery. There were significant decreases in the asymmetry scores for the nose, upper lip and the cheeks as a result of the surgical repair of cleft lips. Surgery did not change the magnitude of the asymmetry scores for the lower lip and chin. Residual nasolabial asymmetries were detected. The main outcome of the findings of this innovative study is to inform the required surgical refinement of primary repair of cleft lip in order to minimise facial asymmetry. We have presented a sensitive tool that could be used for comparative analysis of lip repair at various cleft centres and to guide secondary corrective surgery when required.

Effect of palate re-repairs and hynes pharyngoplasties on pediatric airways: an analysis of preoperative and postoperative cardiorespiratory sleep studies.

OBJECTIVES : To determine whether Sommerlad palate re-repairs and Hynes pharyngoplasties cause obstructive sleep apnea/hypopnea or increased upper airway resistance. DESIGN : Prospective before-and-after study. SETTING : Tertiary cleft unit. PATIENTS : A total of 44 patients undergoing a Sommerlad palate re-repair or a Hynes pharyngoplasty. Interventions : Preoperative and postoperative multichannel cardiorespiratory sleep studies. MAIN OUTCOME MEASURES : The main outcome measures were mean arterial oxygen saturation, desaturation index, percentage of time spent with arterial oxygen saturation <90%, mean pulse rate, number of pulse rate rises (arousals) per hour, inspiratory effort derived from pulse transit time, pulse transit time arousals, and snoring. RESULTS : No patient in either group required intervention for airway obstruction or obstructive sleep apnea/hypopnea. Re-repairs caused no significant change in any parameter. Hynes caused an increase in inspiratory effort (P = .04) and obstructive sleep apnea/hypopnea grading (P = .002). All other parameters showed no significant deterioration. No patient developed more than mild/moderate obstructive sleep apnea/hypopnea. Snoring and arterial oxygen saturation levels were not reliable indicators of increased inspiratory effort. CONCLUSIONS : A palate re-repair had no significant adverse effect on the airway. A Hynes, in patients with optimized velar function, caused a significant increase in inspiratory effort and obstructive sleep apnea/hypopnea grade. However, compared with studies on midline flaps and on sphincter pharyngoplasties, a Hynes appears to be less obstructive. Failure to study changes in inspiratory effort in patients undergoing velopharyngeal incompetence surgery may underestimate the obstructive effect of pharyngoplasties.

Surgical anatomy of the levator veli palatini: a previously undescribed tendinous insertion of the anterolateral fibers.

The purpose of this study was to describe the previously unreported tendinous insertion of the anterolateral fibers of the levator veli palatini (levator) and discuss possible implications for levator function and cleft palate repair. The velopharyngeal anatomy in normal adult cadavers was studied, with histologic confirmation of anatomical findings. These findings were compared with a more limited study of levator anatomy in cleft palates at the time of intraoperative muscle dissection. Just before entering the velum, the levator divides into two parts. The smaller bundle of muscle fibers (anterolateral part) runs anteriorly, close to the lateral pharyngeal wall, and inserts into the palatine aponeurosis through a number of fine tendons. The main part of the muscle runs medially into the velum, where it fans out and forms the levator sling with the contralateral levator. The possible function of the anterolateral part of the levator is discussed. Inadequate release of the tendinous insertions at the time of palate repair may tether the levator anteriorly and compromise muscle retropositioning or may result in splitting of the levator, so that only part of the levator is retropositioned.

Surgical significance of abnormal internal carotid arteries in velocardiofacial syndrome in 43 consecutive hynes pharyngoplasties.

OBJECTIVES: To determine: (1) the incidence of surgically significant, abnormal internal carotid arteries (ICAs) in velocardiofacial syndrome (VCFS); (2) the implications for a Hynes pharyngoplasty; (3) the reliability of preoperative investigations in detecting surgically significant abnormal ICAs. DESIGN: Prospective data collection with blind reassessment of nasendoscopy recordings. SETTING: Two-site, tertiary referral cleft unit. PATIENTS: Forty-three consecutive patients with VCFS who underwent a Hynes pharyngoplasty (six had a subsequent revision). INTERVENTIONS: Intraoral examinations, lateral videofluoroscopy, nasendoscopy when possible, and intraoperative palpation of the posterolateral pharyngeal walls. Only one patient had a magnetic resonance angiography (MRA). MAIN OUTCOME MEASURES: Incidence of surgically significant pulsations; modifications to surgical procedure; and correlation of surgical findings with preoperative nasendoscopy and MRA. RESULTS: Five patients (11.6%) had abnormal pulsations noted at the time of the Hynes. In no patient was the decision to perform a Hynes altered as a result of abnormal pulsations. Two patients had minor adjustments to the Hynes flaps to avoid exposing/damaging the ICA. In one patient an abnormal ICA was exposed during elevation of the left Hynes flap. This was covered uneventfully by routine closure of the secondary defect. Pulsations were noted in only 3 of 24 assessable preoperative nasendoscopies. CONCLUSIONS: A Hynes pharyngoplasty is not contraindicated in VCFS, even if abnormal pulsations are present. Examination and palpation of the pharyngeal walls after the patient is positioned for surgery appear to be reliable in detecting abnormal pulsations and allow accurate surgical planning. Routine vascular imaging, even in patients with pulsations on preoperative nasendoscopy is not essential and may not always be reliable, as shown by the variation in endoscopic, MRA, and intraoperative findings. This further re-emphasizes the importance of palpating the pharyngeal walls once the patient is positioned for surgery.

Surgical management of velopharyngeal incompetence in velocardiofacial syndrome.

OBJECTIVES: To analyze the results of surgery for velopharyngeal incompetence (VPI) in velocardiofacial syndrome. DESIGN: Prospective data collection, with randomized, blind assessment of speech and velopharyngeal function on lateral videofluoroscopy and nasendoscopy. SETTING: Two-site, tertiary referral cleft unit. PATIENTS: Forty-two consecutive patients with the 22q11 deletion underwent surgery for symptomatic VPI by a single surgeon. INTERVENTIONS: Intraoral examinations, lateral videofluoroscopy (+/- nasendoscopy) and intraoperative evaluation of the position of the velar muscles through the operating microscope. Based on these findings, either a radical dissection and retropositioning of the velar muscles (submucous cleft palate [SMCP repair]) or a Hynes pharyngoplasty (posterior pharyngeal wall augmentation pharyngoplasty) was performed. As anticipated, a proportion of patients undergoing SMCP repair subsequently required a Hynes. The aim of this staged approach was to maximize velar function, thereby enabling a less obstructive pharyngoplasty to be performed. Thus, there were three surgical groups for analysis: SMCP alone, Hynes alone, and SMCP+Hynes. MAIN OUTCOME MEASURES: Blind perceptual rating of resonance and nasal airflow; blind assessment of velopharyngeal function on lateral videofluoroscopy and nasendoscopy; and identification of predictive factors. RESULTS: Significant improvement in hypernasality in all three groups. The SMCP+Hynes group also showed significant improvement in nasal emission. There were significant improvements in the extended and resting velar lengths following SMCP repair and a trend toward increased velocity of closure. CONCLUSIONS: Depending on velopharyngeal anatomy and function, there is a role for SMCP repair, Hynes pharyngoplasty, and a staged combination of SMCP+Hynes, all of which are procedures with a low morbidity.

Unilateral Moore pharyngoplasty in the treatment of unilateral or asymmetric velopharyngeal incompetence.

OBJECTIVE: To describe the use of a unilateral modification of the Moore pharyngoplasty in the treatment of unilateral or asymmetric velopharyngeal incompetence (VPI) and analyze the results in a consecutive series of patients operated on by a single surgeon. DESIGN: Blind assessment of randomized speech and nasendoscopy recordings. SETTING: A two-site tertiary referral cleft unit. PATIENTS: Eighteen consecutive patients with asymmetrical or unilateral VPI of varying etiology. INTERVENTIONS: A unilateral Moore pharyngoplasty was performed in all patients. Three patients underwent radical dissection and retropositioning of the velar muscles at the same time as the unilateral Moore pharyngoplasty. MAIN OUTCOME MEASURES: Pre- and postoperative nasality and nasal airflow using the CAPS score, assessment of nasendoscopy recordings, and the rate of further surgery. RESULTS: There was a significant improvement in hypernasality (p =.014). There was a highly significant decrease in the size of the velopharyngeal gap on the side on which the Moore pharyngoplasty was performed (p =.004) as well as a highly significant decrease in the total gap size (p =.003). The Moore flap was effective in obliterating the lateral pharyngeal recess in 11 of 12 patients (p =.004). Three patients required further velopharyngeal surgery. CONCLUSIONS: In appropriately selected patients, a unilateral Moore pharyngoplasty is a safe and effective treatment for unilateral or asymmetric VPI. If indicated, a radical dissection and retropositioning of the velar muscles may be combined with a Moore pharyngoplasty.

Gross unilateral abnormalities of the velum and pharynx.

OBJECTIVE: Velopharyngeal incompetence because of gross unilateral velopharyngeal hypoplasia is rare, particularly in patients with no significant hemifacial microsomia or facial asymmetry. We describe the abnormal anatomy and treatment of three patients with gross congenital velopharyngeal asymmetry. RESULTS AND CONCLUSIONS: Following surgery, all three patients showed a good improvement in velopharyngeal function and speech. Nasendoscopy and lateral videofluoroscopy were important in confirming the abnormal anatomy and pathology and in evaluating the degree of movement of the affected side of the velum and pharyngeal walls as well as the size and location of the defect. Muscles from the normal side were radically dissected and mobilized across the midline to reconstruct the hypoplastic hemivelum. Mucosal lengthening was achieved by suturing the normal mucosa to the mucosa of the hypoplastic hemivelum and the lateral pharyngeal wall or by insetting a posterolateral pharyngeal flap (modified Moore pharyngoplasty) into the nasal surface of the hypoplastic side.

Palate re-repair revisited.

OBJECTIVE: To analyze the results of a consecutive series of palate re-repairs performed using the operating microscope and identify predictive factors for outcome. DESIGN: Prospective data collection, with blind assessment of randomized recordings of speech and velar function on lateral videofluoroscopy and nasendoscopy. PATIENTS: One hundred twenty-nine consecutive patients with previously repaired cleft palates and symptomatic velopharyngeal incompetence (VPI) and evidence of anterior insertion of the levator veli palatini underwent palate re-repairs by a single surgeon from 1992 to 1998. Syndromic patients, those who had significant additional surgical procedures at the time of re-repair (23 patients), and all patients with inadequate pre- or postoperative speech recordings were excluded, leaving a total of 85 patients in the study. INTERVENTIONS: Palate re-repairs, with radical dissection and retropositioning of the velar muscles, were performed using the operating microscope with intraoperative grading of anatomical and surgical findings. MAIN OUTCOME MEASURES: Pre- and postoperative perceptual speech assessments using the Cleft Audit Protocol for Speech (CAPS) score, measurement of velar function on lateral videofluoroscopy, and assessment of nasendoscopy recordings. RESULTS: There were significant improvements in hypernasality, nasal emission, and nasal turbulence and measures of velar function on lateral videofluoroscopy, with improvement in the closure ratio, velopharyngeal gap at closure, velar excursion, velar movement angle, and velar velocity. CONCLUSIONS: Palate re-repair has been shown to be effective in treating VPI following cleft palate repair, both in patients who have not had an intravelar veloplasty and those who have had a previous attempt at muscle dissection and retropositioning. Palate re-repair has a lower morbidity and is more physiological than a pharyngoplasty or pharyngeal flap.