Surgical outcomes and revision rates for velopharyngeal insufficiency (VPI) in syndromic and non-syndromic children: A systematic review and meta-analysis.

PURPOSE: To evaluate pre- and post-operative resonance, surgical technique, revision rate, and revision indication among syndromic and non-syndromic children with velopharyngeal insufficiency (VPI). MATERIALS AND METHODS: A systematic review was conducted through July 2022. Children surgically treated for VPI were included. A meta-analysis of single means, proportions, comparison of proportions, and mean differences with 95 % confidence interval [CI] was conducted. RESULTS: Twenty-three articles (n = 1437) were included in the analysis. The most common surgery was Sphincter Pharyngoplasty (SP), 62.6 % [31.3-88.9] for syndromic and 76.3 % [37.5-98.9] for non-syndromic children. Among all surgical techniques, for syndromic and non-syndromic children, 54.8 % [30.9-77.5] and 73.9 % [61.3-84.6] obtained normal resonance post-operatively, respectively. Syndromic patients obtained normal resonance post-operatively in 83.3 % [57.7-96.6] of Combined Furlow Palatoplasty and Sphincter Pharyngoplasty (CPSP), 72.6 % [54.5-87.5] of Pharyngeal Flap (PF), and 45.1 % [13.2-79.8] of Sphincter Pharyngoplasty (SP) surgeries. Non-syndromic patients obtained normal resonance post-operatively in 79.2 % [66.4-88.8] of PF and 75.2 % [61.8-86.5] of SP surgeries. The revision rate for syndromic and non-syndromic patients was 19.9 % [15.0-25.6] and 11.3 % [5.8-18.3], respectively. The difference was statistically significant, 8.6 % [2.9-15.0, p = 0.003]. Syndromic patients who underwent PF were least likely to undergo revision surgery as compared to SP and CPSP, 7.7 % [2.3-17.9] vs. 23.7 % [15.5-33.1] and 15.3 % [2.8-40.7], respectively. CONCLUSIONS: Syndromic children had higher revision rates and were significantly less likely to obtain normal resonance following primary surgery than non-syndromic patients. Among syndromic children, PF and CPSP have been shown to improve resonance and reduce revision rates more so than SP alone.

Cleft Lip and/or Palate Repair in Children With Hypopituitarism: Analysis of the Kids' Inpatient Database.

OBJECTIVE: Children with hypopituitarism (CwHP) can present with orofacial clefting, frequently in the setting of multiple midline anomalies. Hypopituitarism (HP) can complicate medical and surgical care; the perioperative risk in CwHP during the traditionally lower risk cleft lip and/or palate (CL/P) repair is not well described. The objective of this study is to examine the differences in complications and mortality of CL/P repair in CwHP compared to children without hypopituitarism (CwoHP). DESIGN: A retrospective cross-sectional analysis. SETTING: The 1997 to 2019 Kids' Inpatient Databases (KID). PATIENTS: Children 3 years old and younger who underwent CL/P repair. MAIN OUTCOME MEASURE(S): Complications and mortality. RESULTS: A total of 34 106 weighted cases were analyzed, with 86 having HP. CwHP had a longer length of stay (3.0 days [IQR 2.0-10.0] vs 1.0 day [IQR 1.0-2.0], P < .001) and higher rates of complications and mortality (12.8% vs 2.9%, P < .001) compared to CwoHP. Controlling for demographic factors, CwHP had 6.61 higher odds of complications and mortality than CwoHP (95% CI 3.38-12.94, P < .001). CONCLUSIONS: CwHP can present with a CL/P and other midline defects that can increase the complexity of their care. These data show a significant increase in length of stay, complications, and mortality in CwHP undergoing CL/P repair. Increased multidisciplinary attention and monitoring may be needed for these children peri- and postoperatively, especially if additional comorbidities are present. Further studies on perioperative management in this population are warranted to reduce morbidity and mortality.

Racial and Ethnic Disparities in Primary Cleft Lip and Cleft Palate Repair.

OBJECTIVE: To examine the impact of race/ethnicity on timing and postoperative outcomes of primary cleft lip (CL) and cleft palate (CP) repair. DESIGN: Cross-sectional analysis of the National Surgical Quality Improvement Program Pediatric (NSQIP-P) database from 2013 to 2018. PATIENTS AND MAIN OUTCOME MEASURES: Patients under 2 years of age who underwent primary CL or CP repair were identified in the NSQIP-P. Outcomes were the timing of surgery and 30-day readmission and reoperation rates stratified by race and ethnicity. RESULTS: In total, 6021 children underwent CL and 6938 underwent CP repair. Adjusted rates of CL repair over time were 10% lower in Hispanic children (95%CI: 0.84-0.96) and 38% lower for Asian children (95%CI: 0.55-0.70) compared with White infants. CP repair rates over time were 13% lower in Black (95%CI: 0.79-0.95), 17% lower in Hispanic (95%CI: 0.77-0.89), and 53% lower in Asian children (95%CI: 0.43-0.53) than in White infants. Asian patients had the highest rates of delayed surgical repair, with 19.3% not meeting American Cleft Palate-Craniofacial Association (ACPA) guidelines for CL (P < .001) and 28.2% for CP repair (P< .001). Black and Hispanic children had 80% higher odds of readmission following primary CL repair (95%CI: 1.16-2.83 and 95%CI: 1.27-2.61, respectively). CONCLUSIONS: This study of a national database identified several racial/ethnic disparities in primary CL and CP, with reduced receipt of cleft repair over time for non-White children. Asian patients were significantly more likely to have delayed cleft repair per ACPA guidelines. These findings underscore the need to better understand disparities in cleft repair timing and postoperative outcomes.

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.

Association of Patient Factors and Follow-Up in an International Volunteer Cleft Lip and Palate Repair Clinic in El Salvador.

BACKGROUND: In countries which lack robust health care systems, congenital conditions such as cleft lip and/or palate deformities are often untreated in certain individuals. Many volunteer organizations have stepped in to fill this gap but certain factors, such as continuity of care, are yet to be studied for these clinics. METHODS: This is a retrospective cohort study of 167 pediatric patients with cleft lip and/or palate residing in El Salvador treated by a nongovernmental organizations between 2011 and 2020. This data was used in univariate and multivariable models to associate particular patient factors to their likelihood of following up to their annual clinic visits. RESULTS: Each 1-year increase in duration of follow-up was associated with a 27% decrease in the odds of attending a visit. In addition, 33.7% of cleft lip and 49.7% of cleft palate/cleft lip and palate patients returned at least once. Males had 36% higher odds of attending a return visit compared with females but this difference was not statistically significant. Time spent travelling to the clinic had no effect on follow-up rates. CONCLUSION: Nongovernmental organizations utilizing a diagonal care model should consider using more strategies to maximize continuity of care by increasing communication with patients and emphasizing the need of following up during clinic visits. Continued and increased collaboration with the local team is also of great importance.

Follow-Up Care Barriers for Patients with Orofacial Clefts.

OBJECTIVE: The aim of this study was to evaluate whether a patient with a cleft's age, associated syndrome, cleft phenotype or travel distance affects their follow-up rate. DESIGN: This study is a retrospective review of patients with CL/P treated by a craniofacial clinic. SETTING: The setting was a craniofacial clinic at a tertiary care university hospital. PATIENTS, PARTICIPANTS: Candidates were patients seen by the craniofacial clinic between January 2007 and December 2019. An initial pool of 589 patients was then reduced to 440 due to exclusion criteria. INTERVENTIONS: None. MAIN OUTCOME MEASURE(S): The outcome measure was actual patient attendance to the craniofacial team compared to the team goal expectation of annual return visits. RESULTS: The mean age of participants at the end of the study was 9.0 ± 5.4 years with a mean follow-up period (total possible follow-up period length based on patient age at presentation and study window) of 5.5 ± 3.6 years. There was no association between cleft phenotype, type of syndrome, or distance to the clinic with attendance. Children with syndromes had an 11% decrease in the odds of attending follow-up visits with each 1-year increase in age compared to a 4% decrease in children without syndromes. CONCLUSIONS: The only significant factors determining patient attendance were the presence of a syndrome and increasing age.

Perceived Barriers to Comprehensive Cleft Care Delivery: Results From A Capacity-Building Educational Initiative and Implications.

INTRODUCTION: We analyzed the perceptions of participants and faculty members in simulation-based comprehensive cleft care workshops regarding comprehensive cleft care delivery in developing countries. METHODS: Data were collected from participants and faculty members in 2 simulation-based comprehensive cleft care workshops organized by Global Smile Foundation. We collected demographic data and surveyed what they believed was the most significant barrier to comprehensive cleft care delivery and the most important intervention to deliver comprehensive cleft care in developing countries. We also compared participant and faculty responses. RESULTS: The total number of participants and faculty members was 313 from 44 countries. The response rate was 57.8%. The majority reported that the most significant barrier facing the delivery of comprehensive cleft care in developing countries was financial (35.0%), followed by the absence of multidisciplinary cleft teams (30.8%). The majority reported that the most important intervention to deliver comprehensive cleft care was creating multidisciplinary cleft teams (32.2%), followed by providing cleft training (22.6%). We found no significant differences in what participants and faculty perceived as the greatest barrier to comprehensive cleft care delivery (P = 0.46), or most important intervention to deliver comprehensive cleft care in developing countries (P = 0.38). CONCLUSIONS: Our study provides an appraisal of barriers facing comprehensive cleft care delivery and interventions required to overcome these barriers in developing countries. Future studies will be critical to validate or refute our findings, as well as determine country-specific roadmaps for delivering comprehensive cleft care to those who need it the most.

Simulation-Based Comprehensive Cleft Care Workshops: A Reproducible Model for Sustainable Education.

OBJECTIVE: Evaluate simulation-based comprehensive cleft care workshops as a reproducible model for education with sustained impact. DESIGN: Cross-sectional survey-based evaluation. SETTING: Simulation-based comprehensive cleft care workshop. PARTICIPANTS: Total of 180 participants. INTERVENTIONS: Three-day simulation-based comprehensive cleft care workshop. MAIN OUTCOME MEASURES: Number of workshop participants stratified by specialty, satisfaction with the workshop, satisfaction with simulation-based workshops as educational tools, impact on cleft surgery procedural confidence, short-term impact on clinical practice, medium-term impact on clinical practice. RESULTS: The workshop included 180 participants from 5 continents. The response rate was 54.5%, with participants reporting high satisfaction with all aspects of the workshop and with simulation-based workshops as educational tools. Participants reported a significant improvement in cleft lip (33.3 ± 5.7 vs 25.7 ± 7.6; P < .001) and palate (32.4 ± 7.1 vs 23.7 ± 6.6; P < .001) surgery procedural confidence following the simulation sessions. Participants also reported a positive short-term and medium-term impact on their clinical practices. CONCLUSION: Simulation-based comprehensive cleft care workshops are well received by participants, lead to improved cleft surgery procedural confidence, and have a sustained positive impact on participants' clinical practices. Future efforts should focus on evaluating and quantifying this perceived positive impact, as well reproducing these efforts in other areas of need.

Sustainable Cleft Care Through Education: The First Simulation-Based Comprehensive Workshop in the Middle East and North Africa Region.

OBJECTIVE: To describe the conduct of the first multidisciplinary simulation-based workshop in the Middle East/North Africa region and evaluate participant satisfaction. DESIGN: Cross-sectional survey-based evaluation. SETTING: Educational comprehensive multidisciplinary simulation-based cleft care workshop. PARTICIPANTS: Total of 93 workshop participants from over 20 countries. INTERVENTIONS: Three-day educational comprehensive multidisciplinary simulation-based cleft care workshop. MAIN OUTCOME MEASURES: Number of workshop participants, number of participants stratified by specialty, satisfaction with workshop, number of workshop staff, and number of workshop staff stratified by specialty. RESULTS: The workshop included 93 participants from over 20 countries. The response rate was 47.3%, and participants reported high satisfaction with all aspects of the workshop. All participants reported they would recommend it to colleagues (100.0%) and participate again (100.0%). No significant difference was detected based on participant specialty or years of experience. The majority were unaware of other cleft practitioners in their countries (68.2%). CONCLUSION: Multidisciplinary simulation-based cleft care workshops are well received by cleft practitioners in developing countries, serve as a platform for intellectual exchange, and are only possible through strong collaborations. Advocates of international cleft surgery education should translate these successes from the regional to the global arena in order to contribute to sustainable cleft care through education.

Conscious Perception of Facial Asymmetry in a Unilateral Cleft Lip Model.

OBJECTIVE: Lip asymmetry after a unilateral cleft lip repair can be perceived as an unsatisfactory result. The objective of this study is to determine the degree of upper lip asymmetry and/or nasal alar hooding required for recognition of asymmetry in a simulated model of unilateral cleft lip. DESIGN: A model of unilateral cleft lip was created using digital morphing software to simulate asymmetries in vermilion height and nasal hooding in photographs of children. Volunteers were shown photographs for different time intervals and with varying degrees of asymmetry. Ability to detect facial asymmetry was recorded and analyzed. SETTING: This study was conducted by surveying layperson volunteers in public community settings. PARTICIPANTS: 108 layperson volunteers were randomly surveyed. MAIN OUTCOME MEASURES: The primary outcome measure was a reported lip or nose asymmetry by the volunteers. Proportions and corresponding 95% confidence intervals were obtained to estimate the probability of reporting an asymmetry at 3- and 10-second intervals. RESULTS: After 3- and 10-second exposure, labial asymmetry was perceived by ≥50% of subjects at 2 mm (62%, P = .001) and 1 mm (89%, P < .0001), respectively. Nasal asymmetry was detected by <50% of subjects at 3 seconds, but ≥50% perceived a 3-mm alteration at 10 seconds (64%, P < .0001). Photographs with combined nasal and labial modification did not lower the threshold for asymmetry perception compared to either deformity alone. CONCLUSIONS: This study is the first to determine a predictable millimeter threshold for perceived asymmetry in cleft lip deformity using a digital model.

Melanotic Neuroectodermal Tumor of Infancy: A Systematic Review.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor, usually diagnosed within the first year of age, with a predilection for the maxilla. Although the tumor is usually benign, its rapidly growing nature and ability to cause major deformities in surrounding structures necessitate early diagnosis and intervention. It is important that medical and dental specialists are prepared to make the diagnosis and proceed with appropriate intervention. The authors performed a systematic review of the 472 reported cases from 1918 through 2013 and provided a comprehensive update on this rare entity that can have devastating effects on young patients. This investigation uncovered age at diagnosis as an important prognostic indicator, because younger age correlated with a higher recurrence rate. The authors also present a case report of a 5-month-old girl diagnosed with MNTI and review her clinical presentation and imaging and histopathologic findings.

Esophageal adenocarcinoma and urothelial carcinoma orbital metastases masquerading as infection.

Orbital metastases can masquerade as other orbital processes. We present two cases of orbital metastases, the first being the first reported adenocarcinoma of the esophagus presenting as an orbital metastasis prior to the primary being known, and the other as the first urothelial carcinoma to present as orbital cellulitis. The first patient presented with left upper eyelid pain. CT scan identified a superolateral subperiosteal fluid collection without concomitant sinus disease, which was drained in the operating room. Two weeks later repeat CT scan showed recurrent orbital subperiosteal fluid. It was drained and a biopsy showed necrotic adenocarcinoma. The second case presented with a painless right proptosis, decreased vision, and globally decreased ocular motility 3 days after bladder resection for urothelial carcinoma. CT scan demonstrated pan sinusitis with a soft tissue mass in the apex of the right orbit with extension through the superior orbital fissure. After no improvement on antibiotics endoscopic drainage was performed. Pathology revealed metastatic urothelial carcinoma within the orbital fat.

Use of safety measures, including the modified world health organization surgical safety checklist, during international outreach cleft missions.

International surgical outreach missions have become increasingly common within the surgery community. Untoward events in this setting, although rare, can be prevented by careful planning and the use of quality assurance guidelines designed to prevent such complications. The surgical safety checklist is widely accepted in most developed health care practices, but is used variably by international mission groups. This article outlines the quality assurance guidelines used, including a modified World Health Organization safety checklist and illustrated patient instruction forms, to provide a standardized means of delivering sound surgical care in the setting of short-term international cleft lip and/or palate missions.

Structural Support for Large to Total Nasal Reconstruction.

When large defects of the nose are present, it is imperative to address all 3 layers: the external skin envelope, the osteocartilaginous support, and the inner mucosal lining. The middle structural framework is the primary factor in determining the overall shape of the nose, in addition to facilitating a functional and patent airway. As such, its reconstruction must be robust enough to provide lasting osteocartilaginous support while minimizing disfiguring bulk. The goal is replacement of missing tissue with grafts of similar strength, size, and shape. This article will review approaches to the reconstruction of structural support in large nasal defects.

High-Grade Pleomorphic Sarcoma in Postauricular Keloid Scar After Surgical Excision and Radiation.

Keloid scars can cause significant morbidity to the patient including substantial cosmetic disfigurement, particularly in the head and neck region. Surgical excision followed by immediate postoperative radiation therapy has been shown to be more effective than single modality treatment. Radiation therapy increases risk for radiation-induced malignancy, though very few cases have been reported in the treatment of keloids. We report the case of a patient with a large postauricular keloid treated with excision and immediate post-operative radiation who developed a high-grade pleomorphic sarcoma in the scar bed. Laryngoscope, 134:3143-3145, 2024.

Speech Intelligibility in Patients with Facial Paralysis.

Background: Patients with facial paralysis often report frustration with communication; however, there are limited data regarding intelligibility deficiencies. Objective: To compare speech intelligibility in patients with severe and non-severe facial paralysis, and in patients with or without synkinesis. Methods: Video and audio data were reviewed retrospectively. Groups were stratified as follows: Group A - severe paralysis (Sunnybrook 0-20) without synkinesis; Group B - non-severe (Sunnybrook >20) paralysis without synkinesis; and Group C - non-severe paralysis with synkinesis. Intelligibility was assessed by lay-people and a speech and language pathologist (SLP) using the Frenchay Dysarthria Assessment Version 2 (FDA-2). A receiver operating characteristic (ROC) curve was used to determine a Sunnybrook cutoff for intelligibility. Results: Eighty cases were reviewed with mean age 55.6, 53.8% female. 25.0% were in Group A, 30.0% Group B, and 45.0% in Group C. Lay-people rated 15.0% and the SLP rated 28.7% as having intelligibility deficiency. An ROC curve demonstrated that patients with Sunnybrook ≤18.5 were more likely to have intelligibility abnormality. Conclusion: Patients with Sunnybrook ≤18.5 are more likely to demonstrate intelligibility deficiency. Clinicians with a more trained ear are more likely to identify intelligibility abnormality compared with lay-people. Those with synkinesis are more intelligible compared with those without it.

Preserving Nasal Tip Rotation and Projection in Open Septorhinoplasty.

Background: Open septorhinoplasty is a common facial plastic surgery procedure that requires extensive planning and knowledge to achieve predictable outcomes. Many patients want to keep their nasal tip characteristics, and the surgeon's task is to reliably meet this expectation and provide stable long-term results. Techniques used to reconstruct nasal tip support include the tongue-in-groove, caudal septal extension graft, and caudal septal replacement graft procedures. Methods: We assessed the 1-year reliability of tongue-in-groove, caudal septal extension graft, and caudal septal replacement graft procedures in maintaining nasal tip rotation and projection in open septorhinoplasty. We conducted a retrospective case series review of septorhinoplasty cases between 2015 and 2019 at the Medical University of South Carolina. Cases with intention to change nasal tip rotation or projection were excluded. Two blinded reviewers analyzed standardized preoperative and 1-year postoperative photographs. Results: Fifty-seven patients fit the inclusion criteria and were included in the analysis. Mean preoperative and postoperative nasal tip rotations and projection ratios were similar (P=0.62, P=0.22, respectively). Twenty-six patients underwent a tongue-in-groove procedure, 24 had a caudal septal extension graft, and 7 had a caudal septal replacement graft with preoperative nasal tip rotations of 98.93°, 99.35°, and 96.89°, respectively (P=0.73). At 1 year, patients who received a tongue-in-groove procedure had a significant increase in nasal tip rotation to 101.24° (P=0.013), while patients who received a caudal septal extension graft had a significant decrease in nasal tip rotation to 97.25° (P=0.009). Patients who received a caudal septal replacement graft had no significant change in nasal tip rotation (P=0.117). The preoperative and postoperative projection ratios were not significantly different among the 3 techniques. Conclusion: Tongue-in-groove, caudal septal extension graft, and caudal septal replacement graft are reliable techniques for maintaining nasal tip projection in open septorhinoplasty. In our experience, when attempting to maintain preoperative nasal tip rotation, the tongue-in-groove technique resulted in a significant increase in tip rotation of 2.31°, while the caudal septal extension graft resulted in a significant decrease of 2.1° at 1 year postoperatively.

Patient and Observer Graded Rhinoplasty Scar Outcomes: A Randomized Controlled Trial of Fast Absorbing Versus Permanent Columellar Suture Closure.

Background: When performing an open rhinoplasty, surgeons commonly use nonabsorbable skin sutures to close the columellar incision. These are believed to minimize scarring. However, removal can be associated with patient discomfort and outcomes may not be superior to using absorbable sutures. Objective: To compare difference in scar appearance for columellar closure after rhinoplasty with absorbable and nonabsorbable sutures. Methods: We performed a prospective randomized control trial with 61 patients. Forty-one patients completed follow-up and were included in final analysis: 23 whose columellar incision was closed with absorbable sutures and 18 with nonabsorbable sutures. A blinded surgeon performed Stony Brook Evaluation Scale (SBES) and a patient performed Patient Scar Assessment Questionnaire (PSAQ) was completed for each suture type. Results: Our results did not reject the null hypothesis that there is no difference in SBES or PSAQ scores between absorbable and nonabsorbable suture types. Conclusions: No difference was detected in scar outcomes between absorbable and nonabsorbable sutures for closure of the columellar incision created during an open rhinoplasty as rated by both patients and blinded clinicians.

Treating Nasal Valve Collapse in Facial Paralysis: What I Do Differently.

Patients with facial paralysis require a systematic zonal assessment. One frequently overlooked region is the effect of facial paralysis on nasal airflow. Patients with flaccid paralysis experience increased weight of the cheek and loss of muscle tone in the ala and sidewall; this significantly contributes to nasal valve narrowing and collapse. These specific findings are often not adequately corrected with traditional functional rhinoplasty-grafting techniques. Flaccid paralysis typically results in inferomedial displacement of the alar base, which must be restored with suspension techniques to fully treat the nasal obstruction. Multiple surgical options exist and are discussed in this article.