State of the art: The Unilateral Cleft lip and Nose Deformity and Anatomic Subunit Approximation.

The anatomic subunit approximation approach to unilateral cleft lip repair was developed over 20 years ago. While the underlying principles of the repair are unchanged, its description has been simplified, additional landmarks and creases have been added, and objective analysis of perioperative changes have provided better clarity regarding goals and desired alterations. We review recent insights regarding the deformity; describe the repair in a simplified manner; and link a stepwise approach to foundation-based primary rhinoplasty as a part of the avenue to creating nasolabial balance and harmony.

L'approximation de la sous-unité anatomique pour réparer la fissure palatine unilatérale a été mise au point il y a plus de 20 ans. Les principes fondamentaux de la réparation n'ont pas changé, mais la description est simplifiée et des repères et des plis ont été ajoutés, sans compter qu'une analyse objective de la malformation et des changements chirurgicaux ont permis de mieux comprendre les objectifs ainsi que les modifications souhaitées. Les auteurs analysent la malformation dans le contexte de récentes études objectives, décrivent la réparation selon des concepts contemporains et relient une approche graduelle à la rhinoplastie primaire fondamentale dans le cadre de la trajectoire visant à créer une harmonie et un équilibre nasolabiaux.

Bilateral Cleft lip Simulation.

OBJECTIVE: To evaluate the features, anatomic accuracy, and educational value of a high-fidelity bilateral cleft lip simulator. DESIGN: Evaluation of the simulator by expert cleft surgeons after performing a simulated bilateral cleft lip repair. SETTING: The simulator was evaluated by the surgeons during the Latin American Craniofacial Association meeting. PARTICIPANTS: Eleven experienced cleft surgeons evaluated the simulator. The cleft surgeons were selected based on their availability during the meeting. INTERVENTIONS: The participants performed a simulated bilateral cleft lip repair. They were each provided with a questionnaire assessing the simulator's features, realism and value as a training tool. MAIN OUTCOME MEASURE (S): The main outcome measure are the scores obtained from a Likert-type questionnaire assessing the simulators features, realism and value. RESULTS: Overall, the surgeons agreed with the simulator's realism and anatomic accuracy (average score of 3.7 out of 5). Overall, the surgeons strongly agreed with the value of the simulator as a training tool (average score of 4.6 out of 5). CONCLUSIONS: A high-fidelity bilateral cleft lip simulator was developed that is realistic and valuable as a training tool. The simulator provides a comprehensive training platform to gain hands-on experience in bilateral cleft lip repair before operating on real patients.

Selective nerve transfers to restore shoulder abduction and flexion in acute flaccid myelitis: A case report.

Acute flaccid myelitis (AFM) is a polio-like condition predominantly affecting children that is characterized by acute-onset, asymmetric flaccid paralysis, often preceded by a prodromal fever or viral illness. With prompt diagnosis and early surgical referral, nerve transfers may be performed to improve function. Highly selective nerve transfers are ideal to preserve existing functions while targeting specific deficits. In this report, we present a case of a double fascicular nerve transfer of median and ulnar nerve fascicles to the axillary nerve, combined with selective transfer of the spinal accessory nerve to the supraspinatus branch of the suprascapular nerve, performed for a 5-year-old girl who developed AFM after an upper respiratory infection. Six months after the onset of the patient's symptoms, the patient had continued weakness of shoulder flexion and abduction, atrophy of the deltoid, and supraspinatus muscles, though needle electromyography revealed a functioning infraspinatus muscle. The patient had no post-operative complications and at 2 years of postoperative follow up achieved shoulder abduction and flexion Active Movement Scale scores of 7/7 compared to preoperative scores of 2/7, with no loss of function in the donor nerve domains. The patient showed active shoulder abduction against gravity to 90° from 30° preoperatively and shoulder flexion to 180° from 15° preoperatively. This case report shows that highly selective nerve transfers may preserve existing functions while targeting specific deficits. A double fascicular transfer from the median and ulnar nerves to axillary nerve may provide abundant axons for functional recovery.

Unraveling a Major Burden of Orofacial Clefts Analyses: Classification of Cleft Palate Fistulas by Cleft Surgeons.

OBJECTIVE: The objective of this study was to investigate how cleft surgeons classify palatal fistulas. We focused on three different anatomical locations (ie, hard palate, soft palate, junction hard/soft palate) to analyze agreement/disagreement at various anatomical locations. DESIGN: Cross-sectional survey study. PARTICIPANTS: Participants in an international webinar that focused on palatal fistula treatment were included. INTERVENTION: Participants were presented with a survey pre- and post-webinar. MAIN OUTCOMES: Frequency of used classification systems for classifying oronasal fistulas and the inter-rater reliability of the Pittsburgh classification system. RESULTS: A total of 141 participants completed the questionnaires prior to the webinar and 109 participants completed the survey after the webinar. In total, four classification systems were used (ie, Pittsburgh, Pakistan Comprehensive Fistula Classification [PCFC], anatomical and 'other'). The Pittsburgh classification was the most commonly used system in all cases. However, Pittsburgh inter-rater reliability was low (κ = 0.136 pre-webinar, and κ = 0.174 post-webinar). Surprisingly, a substantial shift was observed from the anatomical to Pittsburgh classification after the webinar, indicating increased awareness of the usability of the Pittsburgh classification system. CONCLUSIONS: This study demonstrates a large heterogeneity with regards to the classification of cleft palate fistulas. Interestingly, a shift was observed from the anatomical to Pittsburgh classification after the webinar. However, the inter-rater reliability for using the Pittsburgh classification was low. Classifying palatal fistulas in a homogenous fashion could enhance comparison of primary palate repair and could improve treatment of palatal fistulas.

Measuring the Unilateral Cleft Lip Nasal Deformity: Lateral Deviation of Subnasale Is a Clinical and Morphologic Index of Unrepaired Severity.

OBJECTIVE: Objective measurement of pre-operative severity is important to optimize evidence-based practices given that the wide spectrum of presentation likely influences outcomes. The purpose of this study was to determine the correlation of objective measures of form with a subjective standard of cleft severity. DESIGN: 3D images were ranked according to severity of nasal deformity by 7 cleft surgeons so that the mean rank could be used as the severity standard. PATIENTS: 45 patients with unilateral cleft lip and 5 normal control subjects. INTERVENTIONS: Each image was assessed using traditional anthropometric analysis, 3D landmark displacements, and shape-based analysis to produce 81 indices for each subject. MAIN OUTCOME: The correlation of objective measurements with the clinical severity standard. RESULTS: Lateral deviation of subnasale from midline was the best predictor of severity (0.86). Other strongly-correlated anthropometric measurements included columellar angle, nostril width ratio, and lateral lip height ratio (0.72, 0.80, 0.79). Almost all shape-based measurements had tight correlation with the severity standard, however, dorsum deviation and point difference nasolabial symmetry were the most predictive (0.84, 0.82). CONCLUSIONS: Quantitative measures of severity transcend cleft type and can be used to grade clinical severity. Lateral deviation of subnasale was the best measure of severity and may be used as a surrogate of uncoupled premaxillary growth; it should be recorded as an index of pre-operative severity with every cleft lip repair. The correlation of other measures evaluated clarify treatment priorities and could potentially be used to grade outcomes.

Surgery for Velopharyngeal Insufficiency Following Cleft Palate Repair: An Audit of Contemporary Practice and Proposed Schema of Techniques and Variations.

OBJECTIVE: Surgical treatment of velopharyngeal insufficiency (VPI) includes a wide array of procedures. The purpose of this study was to develop a classification for VPI procedures and to describe variations in how they are performed.Design/participants/setting/outcomes: We completed an in-depth review of the literature to develop a preliminary schema that encompassed existing VPI procedures. Forty-one cleft surgeons from twelve hospitals across the USA and Canada reviewed the schema and either confirmed that it encompassed all VPI procedures they performed or requested additions. Two surgeons then observed the conduct of the procedures by surgeons at each hospital. Standardized reports were completed with each visit to further explore the literature, refine the schema, and delineate the common and unique aspects of each surgeon's technique. RESULTS: Procedures were divided into three groups: palate-based surgery; pharynx-based surgery; and augmentation. Palate-based operations included straight line mucosal incision with intravelar veloplasty, double-opposing Z-plasty, and palate lengthening with buccal myomucosal flaps. Many surgeons blended maneuvers from these three techniques, so a more descriptive schema was developed classifying the maneuvers employed on the oral mucosa, nasal mucosa, and muscle. Pharynx-based surgery included pharyngeal flap and sphincter pharyngoplasty, with variations in design for each. Augmentation procedures included palate and posterior wall augmentation. CONCLUSIONS: A comprehensive schema for VPI procedures was developed incorporating intentional adaptations in technique. There was substantial variation amongst surgeons in how each procedure was performed. The schema may enable more specific evaluations of surgical outcomes and exploration of the mechanisms through which these procedures improve speech.

Promoting Recovery Following Birth Brachial Plexus Palsy.

Neonatal brachial plexus palsies (NBPP) occur in 1.74 per 1000 live births with 20% to 30% having persistent deficits. Dysfunction can range from mild to severe and is correlated with the number of nerves involved and the degree of injury. In addition, there are several comorbidities and musculoskeletal sequelae that directly impact the overall functional development. This review addresses the nonsurgical and surgical management options and provides guidance for pediatricians on monitoring and when to refer for specialty care.

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.

The Yin and Yang of Primary Unilateral Cleft Lip and Nose Repair: Balance through Opposing Cleft and Noncleft Side Changes.

BACKGROUND: Balance is achieved through opposing interactions. Objective three-dimensional assessment of changes during surgical treatment of the unilateral cleft lip and nasal deformity are limited, and false assumptions may prevent optimal management. METHODS: The authors performed anthropometric analysis on the immediate preoperative and postoperative images (captured under anesthetic) of patients undergoing primary repair ( n = 36). Changes in dimensions and measures of balance were assessed ( P < 0.05). RESULTS: Angles and ratios that reflect cleft to noncleft side balance normalized, although alterations occurred in opposing ways. Centralization of the columella narrowed the cleft nasal base and widened the noncleft nasal base. As the cleft columellar height elongated, the noncleft columellar height shortened. With these changes and correction of cleft alar base retrusion, the cleft alar dome was raised. The cleft and noncleft lateral lip heights and widths elongated. Meanwhile, the Cupid's bow broadened as the commissures were drawn closer together. Whereas the cleft philtral height lengthened, the noncleft philtral height shortened. Reduction in noncleft philtral height averaged 20% but varied with measures of preoperative severity including columellar angle ( R = 0.67), the difference in philtral heights ( R = 0.65), and lateral deviation of the subnasale ( R = 0.74). CONCLUSIONS: Tissue does not need to be added to "lengthen" the columella, the noncleft philtral height shortening can be estimated, and the contours of anatomic subunits change with surgery on both cleft and noncleft sides. It is inadequate to focus on correction of the cleft side alone without considering corresponding noncleft side changes. Achieving balance through opposing alterations should be the principal goal of treatment. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Protocol for a Prospective Observational Study of Revision Palatoplasty Versus Pharyngoplasty for Treatment of Velopharyngeal Insufficiency Following Cleft Palate Repair.

OBJECTIVE: To present the design and methodology for an actively enrolling comparative effectiveness study of revision palatoplasty versus pharyngoplasty for the treatment of velopharyngeal insufficiency (VPI). DESIGN: Prospective observational multicenter study. SETTING: Twelve hospitals across the United States and Canada. PARTICIPANTS: Individuals who are 3-23 years of age with a history of repaired cleft palate and a diagnosis of VPI, with a total enrollment target of 528 participants. INTERVENTIONS: Revision palatoplasty and pharyngoplasty (either pharyngeal flap or sphincter pharyngoplasty), as selected for each participant by their treatment team. MAIN OUTCOME MEASURE(S): The primary outcome is resolution of hypernasality, defined as the absence of consistent hypernasality as determined by blinded perceptual assessment of a standard speech sample recorded twelve months after surgery. The secondary outcome is incidence of new onset obstructive sleep apnea. Statistical analyses will use propensity score matching to control for demographics, medical history, preoperative severity of hypernasality, and preoperative imaging findings. RESULTS: Study recruitment began February 2021. As of September 2022, 148 participants are enrolled, and 78 have undergone VPI surgery. Enrollment is projected to continue into 2025. Collection of postoperative evaluations should be completed by the end of 2026, with dissemination of results soon thereafter. CONCLUSIONS: Patients with VPI following cleft palate repair are being actively enrolled at sites across the US and Canada into a prospective observational study evaluating surgical outcomes. This study will be the largest and most comprehensive study of VPI surgery outcomes to date.

Establishing a Clinical Protocol for Velopharyngeal MRI and Interpreting Imaging Findings.

Traditional imaging modalities used to assess velopharyngeal insufficiency (VPI) do not allow for direct visualization of underlying velopharyngeal (VP) structures and musculature which could impact surgical planning. This limitation can be overcome via structural magnetic resonance imaging (MRI), the only current imaging tool that provides direct visualization of salient VP structures. MRI has been used extensively in research; however, it has had limited clinical use. Factors that restrict clinical use of VP MRI include limited access to optimized VP MRI protocols and uncertainty regarding how to interpret VP MRI findings. The purpose of this paper is to outline a framework for establishing a novel VP MRI scan protocol and to detail the process of interpreting scans of the velopharynx at rest and during speech tasks. Additionally, this paper includes common scan parameters needed to allow for visualization of velopharynx and techniques for the elicitation of speech during scans.

Weight Status Change in Chinese American Children over a Ten-Year Period: Retrospective Study of a Primary Care Pediatric Population.

Weight change from childhood to adolescence has been understudied in Asian Americans. Known studies lack disaggregation by Asian subgroups. This retrospective study assessed the weight status change in 1500 Chinese American children aged 5−11 years from an urban primary care health center between 2007 and 2017. Weight status was categorized using the 2000 CDC growth charts into "underweight/normal weight" and "overweight/obese." The overweight/obesity prevalence in 2007 and 2017 were determined. McNemar's test and logistic regression were performed. The prevalence of overweight/obesity decreased from 29.9% in 2007 to 18.6% in 2017. Children who were overweight/obese at 5−11 years had 10.3 increased odds of staying overweight/obese over time (95% CI = 7.6−14.0, p < 0.001) compared to their underweight/normal weight counterparts. Of the children who were overweight/obese in 2007, 45.7% remained overweight/obese ten years later. Childhood overweight/obesity strongly predicts adult overweight/obesity in Chinese Americans. Targeted education and intervention are warranted to prevent adult obesity.

Partial tibial nerve transfer for foot drop from deep peroneal palsy: Lessons from three pediatric cases.

Peroneal nerve palsy with resultant foot drop has significant impacts on gait and quality of life. Traditional management includes ankle-foot-orthosis, tendon transfer, and arthrodesis-each with certain disadvantages. While nerve transfers for peroneal nerve injury have been reported in adults, with variable results, they have not been described in the pediatric population. We report the use of partial tibial nerve transfer for foot drop from deep peroneal nerve palsy in three pediatric patients. The first sustained a partial common peroneal nerve laceration and underwent transfer of a single tibial nerve branch to deep peroneal nerve 7 months after injury. Robust extensor hallucis longus and extensor digitorum longus reinnervation was obtained without satisfactory tibialis anterior function. The next patient sustained a thigh laceration with partial sciatic nerve injury and underwent transfer of two tibial nerve branches directly to the tibialis anterior component of deep peroneal nerve 9 months after injury. The final patient sustained a blast injury to the posterior knee and similarly underwent a double fascicular transfer directly to tibialis anterior 4 months after injury. The latter two patients obtained sufficient strength (MRC 4-5) at 1 year to discontinue orthosis. In all patients, we used flexor hallucis longus and/or flexor digitorum longus branches as donors without postoperative loss of toe flexion. Overall, our experience suggests that early double fascicular transfer to an isolated tibialis anterior target, combined with decompression, could produce robust innervation. Further study and collaboration are needed to devise new ways to treat lower extremity nerve palsies.

A Comparative Assessment of Nasal Appearance following Nasoalveolar Molding and Primary Surgical Repair for Treatment of Unilateral Cleft Lip and Palate.

BACKGROUND: Although many cleft teams have adopted nasoalveolar molding to improve nasal form, few comparative studies have assessed the postoperative benefits of this treatment. Given that reported outcomes have been contradictory and that treatment involves considerable burden to families, the purpose of this study was to assess objective and subjective changes from nasoalveolar molding at approximately 5 years of age. METHODS: All patients with complete unilateral cleft lip and palate who underwent primary cheiloplasty performed by a single surgeon over a 7-year period were reviewed. Patient results were grouped into nasoalveolar molding or no-nasoalveolar molding. Cleft severity and aesthetic outcomes were assessed by panels of raters who independently ranked subject images at presentation, immediately preoperative (after molding), and at 5-year follow-up. Objective symmetry was measured using standard anthropometric analysis on three-dimensional images. RESULTS: Among 41 patients included, 16 successfully completed nasoalveolar molding. Both groups were similar at presentation; however, the nasoalveolar molding group had improved appearance following molding (p < 0.05). After surgery, at 5 years of age, the nasoalveolar molding group had better rank scores for overall appearance (p < 0.05), cleft nostril height, and cleft medial lip height (p < 0.05). Regression analysis revealed that nasoalveolar molding treatment was the most significant predictor of overall nasal appearance at 5 years, but that treatment team experience and initial severity were also significant predictors (p < 0.05). Qualitative audit following analysis identified favorable and unfavorable features of nasoalveolar molding. CONCLUSION: In children with complete unilateral cleft lip and palate, nasoalveolar molding was associated with better overall nasal aesthetics and improved cleft nostril height and cleft medial lip height at approximately 5 years of age. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Revisiting the unrepaired unilateral cleft lip and nasal deformity using 3D surface image analysis: A data-driven model and its implications.

BACKGROUND: Current descriptions of the unilateral cleft lip and nasal deformity (uCLND) are based upon limited sample sets and subjective observations. While those descriptions are inconsistent and contradictory, theoretical models, including Hogan's "tilted tripod" and Fisher's "nasal arch forms", have never been tested. Given that favorable outcomes of treatment remain elusive, detailed study of the deformity is critical in devising better treatments. The purpose of this study was to develop a data-driven three-dimensional (3D) model of uCLND that spans the spectrum of presentation and involves a pervasive underlying mechanism. METHODS: We studied 3D images of 100 infants with unrepaired cleft lip at 6 months of age. Objective assessment included the landmark positions, anthropometric dimensions, and shape-based measures. Cleft severity was stratified by the lateral displacement of subnasale, so that a model could be developed using linear regression. RESULTS: With progressive deviation of subnasale, the non-cleft alar base moved lateral, the cleft alar base was left posterior, and the nasal dorsum followed the caudal septum (deviating towards the non-cleft side). The "twist" resulted in opposing cleft alar dome collapse, non-cleft alar ring constriction (the non-cleft nasal sill narrowed, lateral genu rose, and alar-cheek junction became more acute), and displacement of the philtrum from midline. CONCLUSIONS: Our study not only supports theoretical models of uCLND but also clarifies vectors of change and reveals significant non-cleft side alterations. On the basis of our findings, the objectives of treatment should involve centralization of the columella and philtrum and rebalancing the nose by untwisting the orthogonal displacement of the alar bases.

A Toolbox of Surgical Techniques for Palatal Fistula Repair.

OBJECTIVE: To provide an inventory of oronasal fistula repair techniques alongside expert commentary on which techniques are appropriate for each fistula type. DESIGN: A 4-stage approach was used to develop a consensus on surgical techniques available for fistula repair: (1) in-person discussion of oronasal fistula cases among cleft surgeons, (2) development of a schema for fistula management using transcripts of the in-person case discussion, (3) evaluation of the preliminary schema via a web-based survey of additional cleft surgeons, and (4) revision of the management schema using survey responses. PARTICIPANTS: Six cleft surgeons participated in the in-person case discussion. Eleven additional surgeons participated in the web-based survey. Participants had diverse training experiences, having completed residency and fellowship at 20 different hospitals. RESULTS: A schema for fistula management was developed, organized by fistula location. The schema catalogues all viable approaches for each location. For fistulae involving the soft palate, the schema stresses the importance of evaluating for velopharyngeal insufficiency (VPI) and incorporating VPI management into fistula repair. For fistulae involving the hard palate, the schema separately enumerates the techniques available for nasal lining repair and for oral lining repair in each region. The schema also catalogues the diversity of approaches to lingual- and labioalveolar fistula, including variation in timing, orthodontic preparation, and simultaneous alveolar bone grafting. CONCLUSIONS: This study employed consensus methods to create a comprehensive inventory of available fistula repair techniques and to identify preferential techniques among a diverse group of surgeons.

Crowdsourcing to Assess Speech Quality Associated With Velopharyngeal Dysfunction.

OBJECTIVE: To assess crowdsourced responses in the evaluation of speech outcomes in children with velopharyngeal dysfunction (VPD). DESIGN: Fifty deidentified speech samples were compiled. Multiple pairwise comparisons obtained by crowdsourcing were used to produce a rank order of speech quality. Ratings of overall and specific speech characteristics were also collected. Twelve speech-language pathologists (SLPs) who specialize in VPD were asked to complete the same tasks. Crowds and experts completed each task on 2 separate occasions at least 1 week apart. SETTING: On-line crowdsourcing platform. PARTICIPANTS: Crowdsource raters were anonymous and at least 18 years of age, North American English speakers with self-reported normal hearing. Speech-language pathologists were recruited from multiple cleft/craniofacial teams. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): Correlation of repeated assessments and comparison of crowd and SLP assessments. RESULTS: We obtained 6331 lay person assessments that met inclusion criteria via crowdsourcing within 8 hours. The crowds provided reproducible Elo rankings of speech quality, ρ(48) = .89; P <.0001, and consistent ratings of intelligibility and acceptability (intraclass correlation coefficient [ICC] = .87 and .92) on repeated assessments. There was a significant correlation of those crowd rankings, ρ(10) = .86; P = .0003, and ratings (ICC = .75 and .79) with those of SLPs. The correlation of more specific speech characteristics by the crowds and SLPs was moderate to weak (ICC < 0.65). CONCLUSIONS: Crowdsourcing shows promise as a rapid way to obtain large numbers of speech assessments. Reliability of repeated assessments was acceptable. Large groups of naive raters yield comparable evaluations of overall speech acceptability, intelligibility, and quality, but are not consistent with expert raters for specific speech characteristics such as resonance and nasal air emission.