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.

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.

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 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.

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.

Anatomic landmark approach to reconstruction of asymmetric midline cleft lip due to Pai syndrome.

Midline clefts of the upper lip are rare, and it is therefore important that surgeons have access to a methodical approach for when these presentations are encountered. We adapted principles of the anatomic subunit approximation for unilateral cleft lip, to the repair of midline clefts. The overt use of anatomic landmarks to define the repair results in a design that inherently adjusts to varying degrees of clefts and can accommodate asymmetries. The "measure twice, cut once" style is an advantage to new surgeons and to surgeons who seldom encounter this presentation. We describe the details of surgical repair in the context of a patient with Pai syndrome and associated nasal hamartomas that resulted in nasolabial asymmetry. This is the first report of surgical outcome following treatment of Pai syndrome and includes early and 5-year follow-up. The system of repair that we describe is applicable to both symmetric and asymmetric midline clefts.

Shoulder Release and Tendon Transfer following Neonatal Brachial Plexus Palsy: Gains, Losses, and Midline Function.

BACKGROUND: Shoulder release and tendon transfer is frequently performed to address persistent weakness from neonatal brachial plexus palsy. Although postoperative improvements in motion are well described, associated deficits are poorly documented, and functional assessments are lacking. Loss of ability to reach midline can occur with surgery and may result in impairment. The purpose of this study was to comprehensively assess the gains, losses, functional changes, and patient-reported outcome associated with the authors' surgical approach. METHODS: Consecutive patients undergoing surgery with 2-year follow-up were included (n = 30). Prospectively recorded assessments by therapists were reviewed. Changes were assessed by t test and Wilcoxon rank sum (p < 0.05). RESULTS: Active external rotation and abduction improved and internal rotation diminished. Aggregate modified Mallet score increased with improvements in all subscales, except that hand to spine was unchanged and hand to belly decreased. Functional assessment using the Brachial Plexus Outcome Measure revealed an increase of aggregate score, with no decline in any subscales. Improvements were in hand to back of head, forward overhead reach, holds plate with palm up, opening large container, and strings bead. Aggregate patient self-report of appearance and function increased (from 18 to 23). Loss of ability to reach midline occurred in three patients (10 percent) who had extended Erb or total palsy and preoperative limitations of internal rotation. CONCLUSIONS: Secondary reconstruction rebalances shoulder motion by increasing external rotation and abduction and reducing internal rotation. In this study, a conservative surgical approach results in overall improvement in task-based abilities and self-reported outcomes and preservation of internal rotation within a functional range. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Feasibility of Surgeon-Delivered Audit and Feedback Incorporating Peer Surgical Coaching to Reduce Fistula Incidence following Cleft Palate Repair: A Pilot Trial.

BACKGROUND: Improving surgeons' technical performance may reduce their frequency of postoperative complications. The authors conducted a pilot trial to evaluate the feasibility of a surgeon-delivered audit and feedback intervention incorporating peer surgical coaching on technical performance among surgeons performing cleft palate repair, in advance of a future effectiveness trial. METHODS: A nonrandomized, two-arm, unblinded pilot trial enrolled surgeons performing cleft palate repair. Participants completed a baseline audit of fistula incidence. Participants with a fistula incidence above the median were allocated to an intensive feedback intervention that included selecting a peer surgical coach, observing the coach perform palate repair, reviewing operative video of their own surgical technique with the coach, and proposing and implementing changes in their technique. All others were allocated to simple feedback (receiving audit results). Outcomes assessed were proportion of surgeons completing the baseline audit, disclosing their fistula incidence to peers, and completing the feedback intervention. RESULTS: Seven surgeons enrolled in the trial. All seven completed the baseline audit and disclosed their fistula incidence to other participants. The median baseline fistula incidence was 0.4 percent (range, 0 to 10.5 percent). Two surgeons were unable to receive the feedback intervention. Of the five remaining surgeons, two were allocated to intensive feedback and three to simple feedback. All surgeons completed their assigned feedback intervention. Among surgeons receiving intensive feedback, fistula incidence was 5.9 percent at baseline and 0.0 percent following feedback (adjusted OR, 0.98; 95 percent CI, 0.44 to 2.17). CONCLUSION: Surgeon-delivered audit and feedback incorporating peer coaching on technical performance was feasible for surgeons.