Sitzman, Thomas J

To determine the sensitivity and specificity of velar notching seen on nasopharyngoscopy for levator veli palatini (LVP) muscle discontinuity and anterior positioning. Nasopharyngoscopy and MRI of the velopharynx were performed on patients with VPI as part of their routine clinical care. Two speech-language pathologists independently evaluated nasopharyngoscopy studies for the presence or absence of velar notching. MRI was used to evaluate LVP muscle cohesiveness and position relative to the posterior hard palate. To determine the accuracy of velar notching for detecting LVP muscle discontinuity, sensitivity, specificity, and positive predictive value (PPV) were calculated. A craniofacial clinic at a large metropolitan hospital. Participants Thirty-seven patients who presented with hypernasality and/or audible nasal emission on speech evaluation and completed nasopharyngoscopy and velopharyngeal MRI study as part of their preoperative clinical evaluation. Among patients with partial or total LVP dehiscence on MRI, presence of a notch accurately identified discontinuity in the LVP 43% (95% CI 22-66%) of the time. In contrast, the absence of a notch accurately indicated LVP continuity 81% (95% CI 54-96%) of the time. The PPV for the presence of notching to identify a discontinuous LVP was 78% (95% CI 49-91%). The distance from the posterior edge of the hard palate to the LVP, known as effective velar length, was similar in patients with and without notching (median 9.8 mm vs 10.5 mm, P = 1.00). The observation of a velar notch on nasopharyngoscopy is not an accurate predictor of LVP muscle dehiscence or anterior positioning.

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.

Objective To describe current postoperative management practices following cleft palate repair. Design A survey was administered to cleft surgeons to collect information on their demographic characteristics, surgical training, surgical practice, and postoperative management preferences. Setting Eighteen tertiary referral hospitals across the United States. Participants: Surgeons (n = 67) performing primary cleft palate repair. Results Postoperative diet restrictions were imposed by 92% of surgeons; pureed foods were allowed at one week after surgery by 90% of surgeons; a regular diet was allowed at one month by 80% of surgeons. Elbow immobilizers and/or mittens were used by 85% of surgeons, for a median duration of two weeks. There was significant disagreement about postoperative use of bottles (61% allow), sippy cups (68% allow), pacifiers (29% allow), and antibiotics (45% prescribe). Surgeon specialty was not associated with any aspect of postoperative management ( p > 0.05 for all comparisons). Surgeon years in practice, a measure of surgeon experience, was associated only with sippy cup use ( p < 0.01). The hospital at which the surgeon practiced was associated with diet restrictions ( p < 0.01), bottle use ( p < 0.01), and use of elbow immobilizers or mittens ( p < 0.01); however, many hospitals still had disagreement among their surgeons. Conclusions Surgeons broadly agree on diet restrictions and the use of elbow immobilizers or mittens following palate repair. Almost all other aspects of postoperative management, including the type and duration of diet restriction as well as the duration of immobilizer use, are highly individualized.

This clinical case study describes the velopharyngeal anatomy and physiology in a patient who presented with SATB2-associated syndrome (SAS) and velopharyngeal insufficiency (VPI) in the absence of an overt cleft palate. The clinical presentation, treatment, outcome, and the contribution of anatomical findings from MRI to surgical treatment planning for this rare genetic disorder, SAS, are described. This case study contributes to our current understanding of the anatomy and physiology of the velopharyngeal mechanism in an individual born with SAS and non-cleft VPI. It also details the changes following bilateral buccal myomucosal flaps in this patient.

Background Non-sedated MRI is gaining traction in clinical settings for visualization of the velopharynx in children with velopharyngeal insufficiency. However, the behavioral adaptation and training aspects that are essential for successful pediatric MRI have received limited attention. Solution We outline a program of behavioral modifications combined with patient education and provider training that has led to high success rates for non-sedated velopharyngeal MRI in children.

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.

Background: Palate re-repair has been proposed as an effective treatment for velopharyngeal insufficiency (VPI) with a low risk of obstructive sleep apnea (OSA). The authors conducted a systematic review and meta-analysis to determine the proportion of patients achieving normal speech resonance following palate re-repair for VPI, the proportion developing OSA, and the criteria for patient selection that are associated with increased effectiveness. Methods: PubMed, Embase, and Scopus were searched from inception through April 2018 for English language articles evaluating palate re-repair for the treatment of VPI in patients with a repaired cleft palate. Inclusion criteria included reporting of hypernasality, nasal air emission, nasometry, additional VPI surgery, and/or OSA outcomes. Meta-analysis was conducted using random effects models. Risk of bias was assessed regarding criteria for patient selection, blinding of outcome assessors, and validity of speech assessment scale. Results: Eighteen studies met inclusion criteria. The incidence of achieving no consistent hypernasality follow palate re-repair was 61% (95% confidence interval [CI]: 44%-75%). The incidence of additional surgery for persistent VPI symptoms was 21% (95% CI: 12%-33%). The incidence of OSA was 28% (95% CI: 13%-49%). Criteria for selecting patients to undergo re-repair varied, with anterior/sagittal position of palatal muscles (33%) and small velopharyngeal gap (22%) being the most common. No specific patient selection criteria led to superior speech outcomes ( P = .6572). Conclusions: Palate re-repair achieves normal speech resonance in many but not all patients with VPI. Further research is needed to identify the specific examination and imaging findings that predict successful correction of VPI with re-repair.

Objective: To identify child-, surgeon-, and hospital-specific factors at the time of primary cleft lip repair that are associated with the use of secondary cleft lip surgery. Design: Retrospective cohort study. Setting: Forty-nine pediatric hospitals. Participants: Children who underwent cleft lip repair between 1999 and 2015. Main outcome measure: Time from primary cleft lip repair to secondary lip surgery. Results: By 5 years after primary lip repair, 24.0% of children had undergone a secondary lip surgery. In multivariable analysis, primary lip repair before 3 months had a 1.22-fold increased hazard of secondary surgery (95% confidence interval [CI]: 1.02-1.46) compared to repair at 7 to 12 months of age, and children with multiple congenital anomalies had a 0.77-fold decreased hazard of secondary surgery (95% CI: 0.68-0.87). After adjusting for cleft type, age at repair, presence of multiple congenital anomalies, and procedure volume, there remained substantial variation in secondary surgery use among surgeons and hospitals ( P < .01). For children with unilateral cleft lip repaired at 3 to 6 months of age, the predicted proportion of children undergoing secondary surgery within 5 years of primary repair ranged from 4.9% to 21.8% across surgeons and from 4.5% to 24.7% across hospitals. Conclusions: There are substantial differences among surgeons and hospitals in the rates of secondary lip surgery. Further work is needed to identify causes for this variation among providers.