Outcomes of alveolar cleft repair in complete cleft lip and palate after tertiary gingivoperiosteoplasty and segmental Le Fort I osteotomy.

OBJECTIVES: Gingivoperiosteoplasty is often used for reconstruction of alveolar defects in infants with cleft lip and palate. This study aimed to examine outcomes of tertiary gingivoperiosteoplasty, which has not previously been investigated. MATERIALS AND METHODS: This prospective study included 11 adults with complete cleft lip and palate (n = 12 sites) who consecutively underwent segmental Le Fort I osteotomy and concomitant gingivoperiosteoplasty for correction of skeletal class III deformity, nasoalveolar fistula and alveolar cleft. Outcomes included clinical and radiographic evaluations of gingivoperiosteoplasty at the time of osteotomy (presence of nasoalveolar fistula, residual cleft defect and unsupported root ratio of cleft-adjacent teeth), and determination of influencing factors for the clinical success of alveolar cleft repair. Study variables included age, gender, pre-surgical orthodontic treatment and alveolar cleft width of cleft-adjacent canine and angulation between cleft-adjacent teeth before surgery and 1-week postsurgery. RESULTS: Posttreatment, no nasoalveolar fistula remained. The residual cleft defect decreased significantly (p < 0.01). The unsupported root ratio of cleft-adjacent teeth did not differ (p > 0.05); eight cleft sites reached Bergland I or II (67% success). One-week postsurgery, the minimal alveolar cleft width of cleft-adjacent canine was significantly less in the success group compared with the failed group (p = 0.01). CONCLUSIONS: Tertiary gingivoperiosteoplasty and segmental Le Fort I osteotomy decreased nasoalveolar fistulas and induced alveolar bone formation. The minimal alveolar cleft width immediately after surgery was the major influencing factor of clinical success. CLINICAL RELEVANCE: Segmental Le Fort I osteotomy with simultaneous gingivoperiosteoplasty efficaciously repairs adult alveolar clefts.

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.

Extensive Gingivoperiosteoplasty Versus Secondary Alveolar Bone Grafting in Patients With Complete Bilateral Cleft Lip and Palate During the Mixed Dentition Period: A Retrospective Outcome Analysis.

BACKGROUND: The concept of gingivoperiosteoplasty (GPP) in the mixed dentition stage as compared with secondary alveolar bone grafting (ABG) in management of alveolar cleft has not been much discussed upon. The authors present the experience with extensive GPP and ABG in the mixed dentition stage in complete bilateral alveolar cleft cases. METHODS: A retrospective review of nonsyndromic patients with complete bilateral alveolar cleft operated on with either GPP or ABG (iliac crest) in the mixed dentition stage with at least 1-year follow-up was performed. Dental occlusal radiographs were evaluated for level of bone gain using Bergland and Witherow scales. Statistical evaluation of clinical success and procedure-related complications was conducted using χ2 test and odds ratio. RESULTS: Twenty-four patients in the GPP group and 20 in the ABG group were comparatively studied. Clinical success rate as indicated by Bergland scales I and II (87.5% in GPP vs 82.5% in ABG; P = 0.731), complication rate (20.83% in GPP vs 30% in ABG; P = 0.484), and status of canine eruption showed no significant differences in clinical outcomes in both groups. CONCLUSIONS: The technique of extensive GPP as described by authors shows equal efficacy to secondary ABG for management of bilateral alveolar clefts during the mixed dentition period.

Comparison of stability and outcomes of surgery-first bimaxillary surgery for skeletal class III deformity between unilateral and bilateral cleft lip and palate.

OBJECTIVES: Some adults with cleft lip and palate (CLP) require orthognathic surgery due to skeletal deformity. This prospective study aimed to (1) compare skeletal stability following bimaxillary surgery for correction of class III deformity between patients with unilateral CLP (UCLP) and bilateral CLP (BCLP), and (2) identify risk factors of stability. MATERIALS AND METHODS: Adults with CLP and skeletal class III deformities who underwent surgery-first bimaxillary surgery were divided into two groups according to cleft type: UCLP (n = 30) and BCLP (n = 30). Skeletal stability was assessed with measures from cone beam computed tomography images of the maxilla and mandible taken before treatment, 1-week and ≥ 1 year postsurgery for translation (left/right, posterior/anterior, superior/inferior) and rotation (yaw, roll, pitch); multiple regression analysis examined risk factors. RESULTS: At follow-up, the maxilla moved upwards in both groups, and backwards in the UCLP group. The mandible moved forward and upward, shifted to the cleft (deviated) side, and rotated upward in both groups. The amount of surgical advancement was a risk factor for sagittal stability in the maxilla (ß = -0.14, p < 0.05). The mandible had three risk factors for sagittal stability: age (ß = -0.23, p < 0.05), surgical team (ß = -1.83, p < 0.05), and amount of surgical setback (ß = -0.32, p = 0.001). CONCLUSIONS: Two years after bimaxillary surgery, patients with UCLP had a higher sagittal relapse of the maxilla compared with patients with BCLP, which was due to a greater surgical advancement in the patients with UCLP. CLINICAL RELEVANCE: Surgery-first bimaxillary surgery results in favorable treatment outcomes for correction of cleft-related class III deformity. Severity of jaw discrepancy and surgeons should be considered in the surgical design of overcorrection.

Social Media and Instant Messaging: An Online Platform for the Treatment of Cleft Lip and Palate Patients; Taipei Medical University Hospital Experience.

BACKGROUND: Social media and instant messaging can improve patient education, follow-up, and outcome. The utilization of these modalities in cleft lip/palate care is not fully described. We formed an online platform consisting of a dedicated website, Facebook group and LINE group through which we provide information and counseling to our patient population. Our objective is to evaluate the impact and patient satisfaction of our online platform. METHODS: In this retrospective study, we designed a questionnaire aimed at evaluating the quality of information and patient satisfaction of the online platform. The questionnaire was distributed online to 732 cases connected to us through the LINE application. RESULTS: The response rate was 26%. Website information was found very necessary and sufficient by 75% and 46% of the cases, respectively. Regarding Facebook, the medical team's response was found very professional and practical by 81% and 68% of the cases, respectively. Patient-patient interaction was helpful to 96% of the cases. Regarding the LINE application, the medical team's response was found very immediate and helpful in 73% of the cases, and 91% felt that LINE saved them time from returning to the clinic on unscheduled occasions. The website, Facebook and LINE were used over 10 times per month by 17%, 32%, and 50% of the cases, respectively. A decrease in unscheduled "pop-up" visits to the clinic (from 25 to 6 per month) was registered. CONCLUSIONS: Adapting social media and instant messaging into our cleft lip/palate care has been welcomed with high satisfaction among our patient population, increasing their knowledge, saving them time from returning to the clinic, and allowing them beneficial interaction with other families coping with similar conditions.

Videofluoroscopic Findings as Predictors of Velopharyngeal Insufficiency After Orthognathic Surgery in Patients With Cleft Lip and Palate.

BACKGROUND: Midface retrusion is a common problem in patients with cleft lip and palate owing to the lack of adequate growth of the maxilla. Midface advancement surgery is a definite treatment for midface retrusion; however, the subsequent oropharyngeal airway changes might result in velopharyngeal insufficiency (VPI) in affected patients. Determining the predictors of VPI beforehand and modifying the surgical procedure would be beneficial. MATERIALS AND METHODS: A retrospective review of 42 patients with cleft lip and palate who underwent orthognathic surgery from 2013 to 2014 was performed. A total of 25 patients met the inclusion criteria and had undergone complete pre- and postsurgery videofluoroscopy, nasopharyngoscopy (NPS), and speech assessment. We compared 2 groups of patients, those who showed NPS finding changes of more than 0.1 (8 patients) and those without changes (17 patients), by measuring the distances of the contact point to the tip of the velum, gap size at maximum closure (MC) and rest, contact area length, lateral wall closure rate, closure velocity, and 2 angles (from the velum to the hard palate and genu) at MC and rest. The amount of maxillary advancement was also recorded. RESULTS: Among the 8 patients with NPS finding changes, 3 underwent Furlow palatoplasty for VPI. No significant difference was found in the amount of maxillary advancement between the groups (5.8 vs 5.7 mm). Significant differences were found in the preoperative gap size at MC and angle at the genu at MC (P = 0.035 and .012). These could be considered as the predictors before surgery. After surgery, a significant difference was found in the contact area, lateral wall closure rate, and gap size at MC (P = 0.005, 0.018, and 0.01). CONCLUSION: Videofluoroscopy is a relatively applicable method of determining dynamic changes in the velum function. By performing videofluoroscopy before midface advancement surgery in patients with cleft lip and palate, we may predict the risk of VPI and consider alternative surgical strategies.

Unilateral Cleft Lip Repair Using the Chang Gung Technique: An Outcome Analysis.

OBJECTIVE: This retrospective study analyzed the outcomes of patients undergoing complete or incomplete unilateral cleft lip repair using the Chang Gung technique. The goal was to compare the symmetry and change of the technique through the measurement of anthropometric points on digital photographs. METHODS: From 2010 to 2016, a total of 274 complete and incomplete cleft lip patients without other craniofacial deformities were included in the study. All included patients had a minimum 1-year follow-up with frontal view photographs taken. The vermilion area, lip width, vermilion height, lateral lip length, lip height, and Cupid's bow width of both cleft and noncleft sides were measured for all patients. The Cleft Lip Component Symmetry Index was used to determine the symmetry of the cleft and noncleft sides in both incomplete and complete cleft groups. RESULTS: A total of 152 complete and 122 incomplete cleft lip patients were included in the study. The mean Cleft Lip Component Symmetry Index values showed that the vermilion area, lip height, and Cupid's bow width were symmetric on both cleft and noncleft sides. Lip width and lateral lip length were noted to be shorter, whereas the vermilion height was thicker on the cleft side than on the noncleft side. Significant differences between the complete and incomplete cleft group measurements were found for lateral lip length and lip height, and complete cleft measurements were shorter than those for incomplete clefts. There were no significant differences in vermilion area, lip width, vermilion height, and Cupid's bow width. CONCLUSIONS: The outcome analysis showed that vermilion height reduction and modifications could be made for both complete and incomplete groups. The complete cleft lip has been found to have a significantly shorter lateral lip length and lip height, reflecting a more severe anatomical soft tissue deficiency in this group. A long-term outcome anthropometric point study may further verify the results of this surgical technique.

The Impact of Facial Growth in Unilateral Cleft Lip and Palate Treated With 2 Different Protocols.

BACKGROUND AND AIM: It is well known that palatoplasty can often cause disturbances in maxillary growth. The use of a single-layer vomer flap for the early closure of the hard palate is controversy among surgeons. The aim of this study is to compare the 10-year facial growth of 2 surgical protocols in the treatment of patients with unilateral cleft lip and palate performed by a single surgeon. METHODS: This retrospective analysis includes 43 nonsyndromic patients with complete unilateral cleft lip with or without a vomer flap for the closure of the hard palate during cleft-lip repair. Lateral cephalograms were obtained at the age of 5, 7, and 9 years old, and angular measurements were used to assess patient's facial growth. The Mann-Whitney U test was used to compare 2 treatment protocol groups. RESULT: A total of 23 patients in protocol 1 group (16 male, 7 female) and 20 patients in protocol 2 group (10 male, 10 female) were included. At the age of 5 and 7, there was no significant difference of maxillary and mandibular growth in both groups. At the age of 9 years, all the angular measurement revealed statistical significance with SNA (P = 0.02), SNB (P = 0.05), ANB (P < 0.01), and SNPg (P = 0.05). CONCLUSIONS: The present study has shown that early anterior palate repair for 3-month-old cleft patients have better maxillary growth and less mandibular prognathism.

Aesthetic Durable Forehead Contouring in Asians With Fat Grafting and Botulinum Toxin.

BACKGROUND: Augmentative forehead contouring to create a full rounded smooth forehead is increasingly popular worldwide especially in East Asia where a flat and/or concave irregular forehead is considered unaesthetic. We present our outcomes performing fat transfer to the forehead in combination with botulinum toxin (Botox) corrugator-frontalis injection where indicated in East Asians. METHODS: From 2013 to 2017, 62 consecutive patients with flat and/or concave irregular unaesthetic foreheads underwent forehead fat transfer using a microautologous fat transplantation gun. Concomitant Botox injection to minimize corrugator and frontalis contraction-mediated fat displacement was indicated in 23 patients (thin forehead skin and/or excessive forehead animation or rhytides) to prevent forehead vertical crease formation. Thirty-nine subjects with a mean follow-up of 16 months (range = 3-44 months) had their pregraft and postgraft forehead contours assessed on an aesthetic grading scale. Of these 39, 17 completed forehead fat graft volumetric analysis using preoperative and postoperative 3D photography. RESULTS: Aesthetic results were durable at latest follow-up, and all patients were satisfied with their final appearance. The mean amount of fat transferred was 7.72 mL (range = 1.5-33 mL). One subject required a repeat fat grafting (with Botox) because of vertical crease formation after the index procedure (without Botox). Another 12 needed a second fat-grafting session to optimize the contour. All who received Botox did not develop vertical creases. The mean ± SD forehead contour grade improved from 2.29 ± 0.77 to 3.24 ± 0.67 (P < 0.001). The percentage mean ± SD retention of grafted fat was 46.71% ± 5.77% with Botox versus 39.12% ± 5.24% without Botox (P = 0.006). CONCLUSIONS: Fat grafting to the forehead using our technique with selective Botox administration is an effective, simple, fast, inexpensive, and safe strategy for aesthetic forehead contouring especially for flat and/or concave foreheads. Forehead fat survival rate is objectively improved with simultaneous Botox injection. There is minimum downtime and long-lasting results with high patient satisfaction rates.

Masseter Muscle Volume Changes Evaluated by 3-Dimensional Computed Tomography After Repeated Botulinum Toxin A Injections in Patients With Square Facial Morphology.

INTRODUCTION: Botulinum neurotoxin A (BoNT-A) is a minimally invasive and technically straightforward treatment of masseter muscle (MM) volume reduction and facial contouring, but the literature on its long-term effect on MM volume remains unclear. OBJECTIVE: This study aimed to assess quantitatively for progressive volume changes of lower facial contour after 3 BoNT-A injections in patients with bilateral MM hypertrophy causing square facial morphology using 3-dimensional computed tomographic scans. MATERIALS AND METHODS: Ten female patients with square facial morphology due to bilateral MM hypertrophy were recruited to, and 6 completed, this clinical study. Each received 24 U of BoNT-A into the inferior portion of each MM on both sides, repeated 6 monthly to complete 3 treatments. Masseter muscle volume changes were assessed using 3-dimensional computed tomography at pretreatment (before injections) and posttreatment (1 year after the third injection). RESULTS: Mean MM volume significantly reduced from 26.39 ± 4.18 cm before treatment to 23.26 ± 4.31 cm 1 year after treatment (P = 0.002). CONCLUSION: Three consecutive 6-monthly BoNT-A injections into the MMs reduced their volume by 12% when assessed 1 year after completion of treatment.

Minimal Invasive Rhinoplasty: Fat Injection for Nasal Dorsum Contouring.

PURPOSE: To determine the graft retention rate of fat injection rhinoplasty in the nasal dorsum region using 3dMD System. MATERIALS AND METHODS: Thirteen consecutive patients (12 women and 1 man) treated by the corresponding author (F.C.S.C.) between April of 2014 and December of 2015 for autologous fat injection rhinoplasty at the nasal dorsum region to improve facial esthetics were included in this study. Preoperative and postoperative 3-dimensional (3-D) photography images taken 3 months after fat injection rhinoplasty were superimposed at the level of forehead to measure the volume changes at the nasal dorsum region. The same evaluator repeated the measurements 1 week after the initial measurements. Pearson correlation test was used to determine the intraobserver correlation. RESULTS: Twenty-six scans from 13 patients were analyzed. The amount of fat injected for nasal dorsum augmentation was 1.67 ± 0.95 mL (range, 0.6-3.3 mL). The volume changes calculated using the 3dMD system was 0.74 ± 0.42 mL for the first measurements and 0.74 ± 0.43 mL for the second measurements. The Intraobserver consistency was high with Cronbach α = 0.96 (P < 0.001). The mean volume change according to 3-D photography was 0.74 ± 0.42 mL (range, 0.21-1.53 mL). The mean retention rate was 44.54% (range, 21-74%). CONCLUSIONS: In this pilot study, we reported the fat graft retention rate for nasal dorsum augmentation using 3-D photography. Future studies with a larger sample size, longer-term follow-up, and different anatomical regions will be beneficial to better understand the long-term fat graft retention rate.

Monobloc Frontofacial or Le Fort III Distraction Osteogenesis in Syndromic Craniosynostosis: Three-Dimensional Evaluation of Treatment Outcome and the Need for Central Distraction.

BACKGROUND: The objectives of this study were to investigate the treatment effect, morphology, and volumetric outcomes of monobloc frontofacial or Le Fort III distraction osteogenesis in syndromic craniosynostosis by 3-dimensional evaluation. MATERIALS AND METHODS: Nine consecutive patients underwent monobloc frontofacial or Le Fort III distraction during 2003 to 2012 were included and evaluated. The patient's evaluation is a minimum of a 4-year follow-up. Pretreatment, post-treatment advancement, and relapse were quantified. The changes in intracranial volume, upper airway volume, globe protrusion, advancement at bilateral malar eminence, and central face were calculated from computerized tomography before and after treatment. RESULTS: After distraction, the intracranial volume was increased by 16.4% in average, and the upper airway volume increased by 64.1%. Orbital protrusion improved by 9.9 mm in the left eye and 10.5 mm in the right eye in comparison to the preoperative status. Bilateral malar eminences advancement was greater than the dorsum advancement by 7.1 mm. CONCLUSION: The external distraction osteogenesis device caused significant technical difficulties with advancement of the midface in growing bones. The central midface did not advance as hoped for. This finding may suggest a need for modification in the distraction device in order to improve the central facial distraction vector.

The effect of cleft maxillary distraction osteogenesis on the levator veli palatini and velopharyngeal function.

BACKGROUND: Le Fort I maxillary distraction with the rigid external distraction (RED) device is performed to correct severe midface retrusion in cleft patients, but it may adversely affect velopharyngeal function. OBJECTIVES: This study aims to investigate the angular changes in the levator veli palatini (LVP) and its influence on velopharyngeal function after maxillary distraction using 3-dimensional computed tomography (3D CT) scan volume rendered images. METHODS: This was a retrospective study of 12 patients. Group 1 had no velopharyngeal function deterioration and group 2 had velopharyngeal function deterioration. Preoperative and 1 year postoperative CT scans were analyzed with Mimics v10 software. Segmentation of the LVP and the nasopharyngeal airway was performed and volumetric images were obtained. Six measurements were made: (1) the angle between the levator plane and the Frankfort horizontal, (2) the angle between the levator plane and the soft palate plane, (3) the angle between the 2 LVP muscles, (4) the pharyngeal depth, and (5, 6) the movement of the inferior pharyngeal point with respect to the horizontal and vertical planes. The independent samples t test, Mann-Whitney test, and paired t tests were used for statistical analyses (P < 0.05). RESULTS: Group 2 had statistically significant reduction in the angle between the levator plane and Frankfort horizontal as well as the soft palate plane. Group 1 had a statistically significant increase in the pharyngeal depth and movement of the inferior pharyngeal point with respect to the horizontal plane. CONCLUSION: A decrease in the angle between the levator plane and the Frankfort horizontal or the soft palate plane was associated with velopharyngeal function deterioration.

Vomer flap for hard palate repair is related to favorable maxillary growth in unilateral cleft lip and palate.

OBJECTIVE: Vomer flap repair is assumed to improve maxillary growth because of reduced scarring in growth-sensitive areas of the palate. Our aim was to evaluate whether facial growth in patients with unilateral cleft lip and palate was significantly affected by the technique of hard palate repair (vomer flap versus two-flap). MATERIALS AND METHODS: For this retrospective longitudinal study, we analyzed 334 cephalometric radiographs from 95 patients with nonsyndromic complete unilateral cleft lip and palate who underwent hard palate repair by two different techniques (vomer flap versus two-flap). Clinical notes were reviewed to record treatment histories. Cephalometry was used to determine facial morphology and growth rate. The associations among facial morphology at age 20, facial growth rate, and technique of hard palate repair were assessed using generalized estimating equation analysis. RESULTS: The hard palate repair technique significantly influenced protrusion of the maxilla (SNA: ß = -3.5°, 95 % CI = -5.2-1.7; p = 0.001) and the anteroposterior jaw relation (ANB: ß = -4.2°, 95 % CI = -6.4-1.9; p = 0.001; Wits: ß = -5.7 mm, 95 % CI = -9.6-1.2; p = 0.01) at age 20, and their growth rates (SNA p = 0.001, ANB p < 0.01, and Wits p = 0.02). CONCLUSIONS: The results suggest that in patients with unilateral cleft lip and palate, vomer flap repair has a smaller adverse effect than two-flap on growth of the maxilla. This effect on maxillary growth is on the anteroposterior development of the alveolar maxilla and is progressive with age. We now perform hard palate closure with vomer flap followed by soft palate closure using Furlow palatoplasty. CLINICAL RELEVANCE: These findings may improve treatment outcome by modifying the treatment protocol for patients with unilateral cleft lip and palate.

Surgery-First Orthognathic Approach for Correction of Dentofacial Deformity in Unilateral Cleft Lip and Palate.

BACKGROUND: Although several studies have reported the advantages of the surgery-first approach for orthognathic correction of class III deformity, there is no report of the success of this approach for patients with cleft lip and palate. Therefore, the purpose of this study was to evaluate the stability and outcome of bimaxillary surgery for cleft-related dentofacial deformity using a surgery-first approach. METHODS: Forty-one patients with unilateral cleft lip and palate who consecutively underwent Le Fort I and bilateral sagittal split osteotomies for skeletal class III deformity were included. Cone-beam computed tomographic scans before surgery, 1 week after surgery, and after orthodontic treatment were used to measure the surgical and postsurgical changes in jaw position by landmarks, and outcomes of jaw protrusion and relation, incisor angle and occlusion, and menton deviation after treatment. Self-report questionnaires regarding satisfaction with overall appearance of the face and seven facial regions were administered after treatment. RESULTS: A clinically insignificant relapse was found in the maxilla (<1 mm) and mandible (<2 mm). There was a significant improvement in the jaw protrusion and relation, incisor angle and occlusion, and menton deviation. Responses from the self-report questionnaires completed after treatment indicated that patient satisfaction was high. CONCLUSION: These findings demonstrate surgical-orthodontic treatment with a surgery-first approach can successfully improve cleft-related dentofacial deformity in patients with unilateral cleft lip and palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Soft tissue management of orbitotemporal neurofibromatosis.

PURPOSE: The aim of this study was to provide an overview of a single-institution, 30-year surgical experience with the soft tissue management of orbitotemporal neurofibromatosis. Lessons learned are highlighted in case presentations. METHODS: From 1981 to 2011, all patients who presented to the Chang Gung Memorial Hospital Craniofacial Center with craniofacial neurofibromatosis and orbitotemporal involvement were retrospectively reviewed. The medical records of those patients who underwent surgical correction were reviewed for age, extent of involvement, procedures performed, histologic confirmation, and acute complications. All patients were grouped according to the Jackson Classification. The electronic photobank was queried to evaluate results. RESULTS: Thirty-five patients presented to our center with orbitotemporal neurofibromatosis during the study period. Thirty-one patients underwent surgical management of their disease. The average age was 25 years (range 4 to 57 years). Over half of our patients (n = 18) presented with concomitant disease of the cheek. The 2 most common procedures performed were lateral canthopexy (n = 24) and upper eyelid excision (n = 24). The only acute complication recorded was a postoperative hematoma on the fourth postoperative day following simultaneous lateral canthopexy and upper eyelid excision which required operative evacuation. CONCLUSIONS: In orbitotemporal neurofibromatosis, tissue hyperextensibility and tumor weight adversely affect outcomes. Treatment of concomitant disease of the cheek should be prioritized in order to provide periorbital support prior to addressing the delicate structures of the eyelids. Preservation of the lateral canthal unit and levator muscle, despite neurofibroma infiltration, is critical to maximize outcomes following debulking procedures of the eyelid and orbit.

The FGF and FGFR Gene Family and Risk of Cleft Lip With or Without Cleft Palate.

Background : Isolated, nonsyndromic cleft lip with or without cleft palate is a common human congenital malformation with a complex and heterogeneous etiology. Genes coding for fibroblast growth factors and their receptors (FGF/FGFR genes) are excellent candidate genes. Methods : We tested single-nucleotide polymorphic markers in 10 FGF/FGFR genes (including FGFBP1, FGF2, FGF10, FGF18, FGFR1, FGFR2, FGF19, FGF4, FGF3, and FGF9) for genotypic effects, interactions with one another, and with common maternal environmental exposures in 221 Asian and 76 Maryland case-parent trios ascertained through a child with isolated, nonsyndromic cleft lip with or without cleft palate. Results : Both FGFR1 and FGF19 yielded evidence of linkage and association in the transmission disequilibrium test, confirming previous evidence. Haplotypes of three single-nucleotide polymorphisms in FGFR1 were nominally significant among Asian trios. Estimated odds ratios for individual single-nucleotide polymorphic markers and haplotypes of multiple markers in FGF19 ranged from 1.31 to 1.87. We also found suggestive evidence of maternal genotypic effects for markers in FGF2 and FGF10 among Asian trios. Tests for gene-environment (G × E) interaction between markers in FGFR2 and maternal smoking or multivitamin supplementation yielded significant evidence of G × E interaction separately. Tests of gene-gene (G × G) interaction using Cordell's method yielded significant evidence between single-nucleotide polymorphisms in FGF9 and FGF18, which was confirmed in an independent sample of trios from an international consortium. Conclusion : Our results suggest several genes in the FGF/FGFR family may influence risk for isolated, nonsyndromic cleft lip with or without cleft palate through distinct biological mechanisms.

Craniofacial characteristics in unilateral complete cleft lip and palate patients with congenitally missing teeth.

INTRODUCTION: Congenitally missing permanent teeth are common in patients with clefts. This retrospective study was conducted to evaluate the craniofacial characteristics in patients with unilateral complete cleft lip and palate with congenitally missing permanent teeth. METHODS: A series of 73 consecutive patients with nonsyndromic unilateral complete cleft lip and palate were enrolled. Evaluation of congenitally missing permanent teeth was based on the panoramic films taken from 7 to 11 years of age. The cephalometric films taken around 9 years of age were used to compare the craniofacial morphology in patients with no congenitally missing permanent teeth (n = 20) and 1 (n = 25), 2 (n = 18), and 3 (n = 10) congenitally missing permanent teeth. The Spearman correlation coefficient was used to assess the association of increased numbers of congenitally missing permanent teeth with each cephalometric parameter. RESULTS: Anterior facial height, distance from the maxillary incisor and first molar to the palatal plane, and overjet decreased as the number of congenitally missing permanent teeth increased in patients with unilateral cleft lip and palate. CONCLUSIONS: Unilateral cleft lip and palate patients with congenitally missing permanent teeth have a unique craniofacial morphology with a reduced vertical dimension.

Rationale for white skin roll flap in unilateral cleft lip repair: A retrospective anthropometric measurement analysis.

BACKGROUND: Although the "white skin roll" of the lip is often considered a line, it is better defined as the subunit between the vermilion border and the upper lip horizontal groove. In many unilateral cleft lip repair techniques, this structure is approximated between both sides of the cleft without restoration. This study aimed to analyze the white skin roll height in patients with unilateral cleft lip. METHODS: This retrospective cohort study included 134 consecutive infants with unilateral cleft lip aged 3-6 months who underwent lip repair in a single institution between January 2019 and July 2021. White skin roll heights at the peak of the Cupid's bow on the non-cleft side (CPHIR), cleft medial element (CPHIL), and cleft lateral element (CPHIL') were measured, and differences in their averages were analyzed. RESULTS: The mean height was 1.70 ± 0.30 mm at CPHIR, 0.98 ± 0.33 mm at CPHIL, and 1.28 ± 0.32 mm at CPHIL.' The mean difference in height between CPHIR-CPHIL, CPHIR-CPHIL,' and CPHIL-CPHIL' groups was significant for each paired sample (p < 0.01). No difference was found between the complete and incomplete clefts or left and right clefts (p > 0.01). CONCLUSIONS: A significantly reduced mean height of the white skin roll was present more markedly on the cleft medial element than on the cleft lateral element. Therefore, we strongly support using a white skin roll flap on the cleft lateral element for unilateral cleft lip repair, embracing the concepts of subunits and lip contour lines.

Comparison of Facial Growth between Two Nasoalveolar Molding Techniques in Patients with Unilateral Complete Cleft Lip.

BACKGROUND: Nasoalveolar molding (NAM) has become standard treatment in the authors' craniofacial center. There are two types of presurgical NAM: the Grayson and Figueroa techniques. The Grayson method involves active alveolar molding, and the Figueroa method involves passive alveolar molding. The authors previously found no differences in number of clinic visits, costs, or 6-month postoperative outcome between the two techniques. The authors extended the previous study to evaluate facial growth between these two groups. METHODS: In this randomized single-blind study, conducted between May of 2010 and March of 2013, the authors recruited 30 patients with unilateral complete cleft lip and palate and randomized them for Grayson or Figueroa presurgical NAM. Standard lateral cephalometric measurements at 5 years were used to determine facial growth. RESULTS: Twenty-nine patients completed 5 years of follow-up. There were no statistically significant differences in facial cephalometric measurements between the two groups. CONCLUSION: Presurgical NAM using either a passive or active NAM technique produced similar facial growth patterns after unilateral cleft lip and palate repair. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, II.