The Burden of Care of Nasoalveolar Molding: An Institutional Experience.

Nasoalveolar molding (NAM) is an early presurgical intervention to facilitate primary cleft lip repair by reducing cleft severity and improving labial and nasal form. However, it continues to be associated with the burden of care that influences access and completion of therapy. The authors, therefore, aim to determine the burden of care of NAM therapy for families seeking treatment at a high-volume urban cleft center. A retrospective study of all patients undergoing primary cleft repair between 2012 and 2020 was performed. Patients were grouped based on whether or not NAM therapy was offered. Variables including physical, psychosocial, and financial factors were assessed. Two hundred and thirty patients underwent primary cleft repair between 2012 and 2020. Of these, 176 patients were indicated for NAM, with 4% discontinuing, and 54 patients did not undergo NAM. The 169 patients who completed NAM had a mean duration of treatment of 13.6±8.8 wks consisting of 15±6 scheduled NAM adjustment visits and 1±1 unscheduled visit made urgently to assess caregiver concerns. The mean travel distance was 28.6±37.1 miles. Eighty-four percent of caregivers were married, and 16% did not have English as a primary language. Though 57% had private insurance, 43% of patients received charity support for their treatment. NAM is a finite presurgical intervention that requires caregivers to participate in patient care for approximately three months of their early life. The decision to pursue NAM should be considered alongside the burden of care for caregivers to complete treatment.

Abbe Flap Division Before Two Weeks' Time: Harmful or Advantageous?

INTRODUCTION: The Abbe flap is a standard intervention to treat upper lip deformities in patients with bilateral cleft lip. This two-stage procedure requires a 2 to 3-week period in which the superior and inferior lips remain connected. This study evaluates the safety of Abbe flap division and inset prior to 14 days' time. MATERIALS AND METHODS: A single institution, 8-year review of all patients with a bilateral cleft lip who underwent Abbe flap reconstruction was performed. Patients were classified into two groups: those whom division was performed 14 days or later and those with division earlier than 14 days. RESULTS: A total of 26 patients were identified. Patients who underwent Abbe flap division in less than 14 days (n = 10) demonstrated an average time to division of 9.7 days (range 7-13 days) with no evidence of flap loss, wound breakdown or infection. Patients who underwent Abbe flap division within 14 days or more (n = 16) demonstrated an average time to division of 15 days with four minor complications and no flap loss. CONCLUSION: Dividing the Abbe flap after the first postoperative week appears to be safe and without additional risk to flap loss or wound breakdown. A shorter time to Abbe flap division may decrease the burden of care on patients and their caregivers.

Quantifying Sagittal Lip Changes in Patients with Bilateral Cleft Lip Post Abbe Flap Reconstruction.

OBJECTIVES: To objectively quantify results of sagittal lip changes following Abbe flap reconstruction in patients with bilateral cleft lip. DESIGN: Retrospective, observational study. SETTING: Single institution, 8-year retrospective review. PATIENTS/PARTICIPANTS: In total, 17 patients with bilateral cleft lip that underwent Abbe flap reconstruction were included in this study. INTERVENTION: Patients had lateral photographs taken prior to Abbe flap reconstruction and at least 8 months post-Abbe flap. MAIN OUTCOME MEASUREMENTS: Vegter's index, Sushner's S2 line and Burstone's B line reference lines were used for evaluation of sagittal lip changes. Wilcoxon signed rank tests were used in analysis. RESULTS: The mean pre-Abbe flap upper to lower lip ratio, defined as Vegter's Lip Index, was 0.906 compared to a mean of 0.946 following Abbe flap reconstruction. The mean upper to lower lip ratio for Sushner's S2 and Burstone's B line exhibited an increase in upper lip sagittal projection from -0.164 to 1.459 and 0.259 to 0.957, respectively (P < 0.001). CONCLUSIONS: This study quantifies sagittal changes to upper and lower lip position after Abbe flap reconstruction. These findings may aid in operative planning and patient/caregiver expectations during counseling.

Same-day Discharge for Cleft Palate Repair: A Single-Surgeon Retrospective Analysis.

OBJECTIVE: To evaluate the safety of same-day discharge for patients undergoing primary cleft palate repair. DESIGN: Single-surgeon retrospective review. SETTING: Tertiary care institution. PATIENTS/PARTICIPANTS: 40 consecutive patients that underwent primary cleft palate repair by a single surgeon from September 2018 to June 2023. INTERVENTIONS: Same-day discharge versus overnight admission after primary palatoplasty. MAIN OUTCOME MEASURES: 30-day readmission, reoperation, wound and all-cause complication rate and 1-year fistula incidence. RESULTS: Of 40 total cases, 20 patients were discharged on the same calendar day and 20 patients were admitted for overnight stay following primary cleft palate repair. In the same-day discharge group, readmission incidence was 10%(n = 2), wound complication incidence was 5%(n = 1), and postoperative complication incidence was 15%(n = 3). In comparison, patients admitted overnight had a readmission incidence of 5%(n = 1, P = 1.00), wound complication incidence of 10%(n = 2, P = 1.00), and postoperative complications of 20%(n = 4, P = 1.00) No patients had 30-day reoperations or fistulas at 1 year. A higher proportion of admitted patients held a preoperative diagnosis of unilateral cleft palate and alveolus (Veau 3) as compared to patients discharged on the same day (P = .019). During the postoperative hospital course, admitted patients received significantly more oxycodone at median of 2 doses (IQR 1.00-3.75) and acetaminophen at a median of 4 doses (IQR 3.00-5.00) than patients with same-day discharge with a median of 1 dose (IQR 0.00 -1.00, P < .001). CONCLUSIONS: In a low-risk patient population, same-day discharge following primary cleft palate repair may be safely undertaken and result in similar short-term outcomes and 1-year fistula incidence as patients admitted for overnight stay.

Defining the Treatment Gap in Nasoalveolar Molding: Factors Affecting the Utilization of NAM in an Urban Cleft Center.

BACKGROUND: Many cleft centers incorporate NasoAlveolar Molding (NAM) into their presurgical treatment protocols. However, there are limited data on eligible patients who do not receive or complete NAM. This study characterizes the demographics associated with non-utilization or completion of NAM. METHODS: A single-institution retrospective review was performed of all patients with cleft lip and alveolus undergoing primary unilateral and bilateral cleft lip repair from 2012-2020. Patients were grouped based on utilization or non-utilization of NAM. Demographic and treatment data were collected, including documented reasons for not pursuing or completing NAM. RESULTS: Of 230 eligible patients, 61 patients (27%) did not undergo or complete NAM (no-NAM). In this group, 37 (60.7%) received no presurgical intervention, 12 (19.7%) received presurgical nostril retainers, 3 (4.9%) received lip taping, 1 (1.6%) received a combination of taping/nostril retainers, and 8 (13.1%) discontinued NAM. The most common reasons for not receiving NAM were sufficiently aligned cleft alveolus (21.3%), medical complexity (16.4%), late presentation (16.4%), and alveolar notching (18%). Compared to the NAM group, the no-NAM group had significantly lower rates of prenatal cleft diagnosis/consult, and significantly higher proportion of non-married and non-English speaking caregivers. Multivariable analysis controlling for insurance type, primary language, prenatal consult, marital status, and age at first appointment found that age at first appointment is the only statistically significant predictor of NAM utilization (P < .001). CONCLUSIONS: Common reasons for non-utilization of NAM include well-aligned cleft alveolus, medical complexity, and late presentation. Early presentation is an important modifiable factor affecting rates of NAM utilization.

De novo mutations in the BMP signaling pathway in lambdoid craniosynostosis.

Lambdoid craniosynostosis (CS) is a congenital anomaly resulting from premature fusion of the cranial suture between the parietal and occipital bones. Predominantly sporadic, it is the rarest form of CS and its genetic etiology is largely unexplored. Exome sequencing of 25 kindreds, including 18 parent-offspring trios with sporadic lambdoid CS, revealed a marked excess of damaging (predominantly missense) de novo mutations that account for ~ 40% of sporadic cases. These mutations clustered in the BMP signaling cascade (P = 1.6 × 10-7), including mutations in genes encoding BMP receptors (ACVRL1 and ACVR2A), transcription factors (SOX11, FOXO1) and a transcriptional co-repressor (IFRD1), none of which have been implicated in other forms of CS. These missense mutations are at residues critical for substrate or target sequence recognition and many are inferred to cause genetic gain-of-function. Additionally, mutations in transcription factor NFIX were implicated in syndromic craniosynostosis affecting diverse sutures. Single cell RNA sequencing analysis of the mouse lambdoid suture identified enrichment of mutations in osteoblast precursors (P = 1.6 × 10-6), implicating perturbations in the balance between proliferation and differentiation of osteoprogenitor cells in lambdoid CS. The results contribute to the growing knowledge of the genetics of CS, have implications for genetic counseling, and further elucidate the molecular etiology of premature suture fusion.

Free Fibula Flap for the Treatment of Agnathia in a 10-Year-Old With Severe Agnathia-Otocephaly Complex.

Agnathia-otocephaly complex (AOC), a first branchial arch defect, is characterized by mandibular hypoplasia or aplasia, ear abnormalities, microstomia, and macroglossia and is a rare and often fatal diagnosis. Herein, the technical considerations and details of mandibular reconstruction using virtual surgical planning (VSP) and a vascularized free fibula flap for further mandibular reconstruction in a 10-year-old boy are presented. The patient's preoperative examination was consistent with agnathia (absence of mandibular symphysis, bilateral mandibular bodies, condyles, coronoids, rami, and temporomandibular joint), severe microstomia, and a Tessier # 30 cleft (maintained to allow oral access until later in treatment). Virtual surgical planning was utilized to plan a 3-segment fibula for the reconstruction of the mandibular symphysis and bilateral body segments, and bilateral costochondral grafts were planned for the rami. To the authors' knowledge, this represents the first application of virtual surgical planning for mandibular reconstruction with a vascularized free fibula flap in a pediatric patient with severe agnathia-otocephaly complex.

Reconstructive Approaches Following Sphenoorbital Meningioma Resection.

Sphenoorbital meningiomas are a challenge to access and reconstruct. Although there is much neurosurgical literature on resection of such tumors, there is little discussion on the best methods for the reconstruction of consequent defects, which are often extensive due to large areas of hyperostosis requiring resection. We performed a retrospective analysis of patients who underwent resection and reconstruction of a sphenoorbital meningioma by the senior authors (C.S. and D.A.S.) between 2010 and 2020. Surgical access in all cases included an orbitozygomatic osteotomy. The study cohort consisted of 23 patients (20 female, 3 male) with an average age of 50 (range: 37-72) years at the time of surgery. Most patients had progressive proptosis before the ablative operation. Orbital reconstruction was with a combined titanium-Medpor implant in 18 patients, split calvarial bone graft in 3 patients, and a Medpor implant in 2 patients. Calvarial reconstruction was performed with titanium mesh in 21 patients, split calvarial bone graft and titanium mesh in 1 patient, and craniotomy bone and titanium plate in 1 patient. Reoperation was required in 7 patients due to hypoglobus or enophthalmos (N=2), orbital implant malposition (N=1), abscess (N=1), pain (N=1), intracranial fat graft modification (N=1), and soft tissue deformities (N=2). Our experience demonstrates that sphenoorbital meningiomas can require broad areas of resection of the skull base and calvarium and necessitate comprehensive reconstruction of the anterior cranial fossa, orbital walls, and cranium. Collaboration between craniofacial surgeons and neurosurgeons can achieve optimal results.

Simultaneous Le Fort III and Le Fort I Osteotomy: Surgical Outcomes and Clinical Parameters.

INTRODUCTION: Simultaneous Le Fort III/I (LF III/I) osteotomies are often performed when a differential advancement of the upper and lower midface is needed. This study aims to evaluate midface position preoperative and 1 week postoperative in patients with severe midface hypoplasia. In addition, this study aims to compare the planned surgical movements to the actual postoperative movements. MATERIALS AND METHODS: A retrospective review was conducted using cephalometry for patients treated with a simultaneous LF III/I osteotomy at a single institution. Osteotomies were performed during 1980-2018 on skeletally mature patients with a craniofacial syndrome, with clinical and radiographic follow-up available. RESULTS: Twelve patients met the inclusion criteria with a mean age of 20.2±6.4 years. Treatment resulted in statistically significant anterior movements related to Orbitale, anterior nasal spine, A Point, and the upper incisor tip, and inferior movements related to anterior nasal spine, A Point, upper and lower incisor tips, B point, and pogonion. Stability after 1 year showed only statistically significant changes at ANB. The predictable error for planned movements versus actual movements was greater in the vertical plane than the horizontal plane. CONCLUSIONS: A simultaneous LF III/I osteotomy significantly improved the midface position and occlusal relationship in syndromic patients with midface hypoplasia in a predictable manner. Further multicenter studies with larger sample sizes are needed to validate the conclusions.

LeFort I Horizontal Osteotomy: Defining the Feasibility of the "High Osteotomy".

OBJECTIVE: To define "high osteotomy" and determine the feasibility of performing this procedure. DESIGN: Single institution, retrospective review. SETTING: Academic tertiary referral hospital. PATIENTS, PARTICIPANTS: 34 skeletally mature, nonsyndromic patients with unilateral CLP who underwent Le Fort I osteotomy between 2013 and 2020. Patients with cone-beam computed tomography (CBCT) scans completed both pre- (T1) and post-operatively (T2) were included. Patients with bilateral clefts and rhinoplasty prior to post-operative imaging were excluded. INTERVENTIONS: Single jaw one-piece Le Fort I advancement surgery. MAIN OUTCOME MEASURES: Measurements of the superior ala and inferior turbinates were taken from the post-operative CBCT. RESULTS: The sample included 26 males and 8 females, 12 right- and 22 left-sided clefts. The inferior turbinates are above the superior alar crease at a rate of 73.53% and 76.48% on the cleft and non-cleft sides, respectively. One (2.9%) osteotomy cut was above the level of the cleft superior alar crease, and no cuts were above the level of the non-cleft superior ala. On average, the superior ala was 2.63 mm below the inferior turbinates. The average vertical distances from the superior alar crease and the inferior turbinates to the base of the non-cleft side pyriform aperture were 12.17 mm (95% CI 4.00-20.34) and 14.80 mm (95% CI 4.61-24.98), respectively. To complete a "high osteotomy," with 95% confidence, the cut should be 20.36 mm from the base of the pyriform aperture. CONCLUSIONS: A "high" osteotomy is not consistently possible due to the relationship between the superior alar crease and the inferior turbinate.

"Septoplasty" Performed at Primary Cleft Rhinoplasty: A Systematic Review of Techniques and Call for Accurate Terminology.

OBJECTIVE: Primary cleft nasal repair can include septal reconstruction. We hypothesize that primary cleft septoplasty and adult septoplasty have fundamental differences that render these procedures as distinct surgical entities. DESIGN: Systematic review of the PubMed, Cochrane, and Embase databases performed on pediatric cleft and general adult septoplasty techniques through December 2021. (PROSPERO ID CRD42022295763). MAIN OUTCOME MEASURES: Collected data included information on septal dissection, septal detachment, and management of the bony and cartilaginous septum. RESULTS: Twenty-eight pediatric cleft septoplasty and 229 adult septoplasty studies were included. Dissection in primary cleft septoplasty was limited to the anterocaudal septum, while secondary cleft septoplasty and adult septoplasty techniques entailed wide exposures of the cartilaginous septum with or without exposure of the perpendicular plate of the ethmoid. In primary cleft septoplasty, detachment of the septum was mostly limited to the nasal spine and anterior base of the cartilaginous septum, while secondary cleft septoplasty and adult septoplasty included detachment from the vomer, and ethmoid. In the few reports of cartilage excision during primary cleft septoplasty, removal was limited to the anterior inferior border of the septum, while secondary cleft septoplasty and adult septoplasty included excision of the cartilaginous and bony septum. CONCLUSION: Primary cleft septoplasty is distinct from septoplasty performed on facially mature patients. More specifically, septal dissection and detachment are limited to the anterior caudal area during primary lip repair, with rare removal of cartilage or bone. Given these differences, the authors suggest the term "septal reset" to describe septoplasty performed during primary cleft nasal repair.

Implementation of an Ambulatory Cleft Lip Repair Protocol: Surgical Outcomes.

OBJECTIVES: Cleft lip repair has traditionally been performed as an inpatient procedure. There has been an interest toward outpatient cleft lip repair to reduce healthcare costs and avoid unnecessary hospital stay. We report surgical outcomes following implementation of an ambulatory cleft lip repair protocol and hypothesize that an ambulatory repair results in comparable safety outcomes to inpatient repair. DESIGN/SETTING: This is a single-institution, retrospective study. PATIENTS/PARTICIPANTS: Patients undergoing primary unilateral (UCL) and bilateral (BCL) cleft lip repair from 2012 to 2021 with a minimum 30-day follow-up. A total of 226 patients with UCL and 58 patients with BCL were included. INTERVENTION: Ambulatory surgery protocol in 2016. OUTCOME MEASURES: Variables include demographics and surgical data including 30-day readmission, 30-day reoperation, and postoperative complications. RESULTS: There were no differences in rates of 30-day readmission, reoperation, wound complications, or postoperative complications between the pre- and post-protocol groups. Following ambulatory protocol implementation, 80% of the UCL group and 56% of the BCL group received ambulatory surgery. Average length of stay dropped from 24 h pre-protocol to 8 h post-protocol. The 20% of the UCL group and 44% of the BCL group chosen for overnight stay had a significantly higher proportion of congenital abnormalities and higher American Society of Anesthesiology (ASA) class. Reasons for overnight stay included cardiac/airway monitoring, prematurity, and monitoring of comorbidities. There were no differences in surgical outcomes between the ambulatory and overnight stay groups. CONCLUSIONS: An ambulatory cleft lip repair protocol can significantly reduce average length of stay without adversely affecting surgical outcomes.

Characterizing Cleft Rhinoplasty Across Skeletal Maturity: A Systematic Review of Terminology and Surgical Techniques.

OBJECTIVE: The purpose of this study is to assess cleft rhinoplasty terminology across phases of growth.Design/Setting: A systematic review was performed on cleft rhinoplasty publications over 20 years.Interventions: Studies were categorized by age at surgical intervention: infant (<1 year); immature (1 to 14 years); mature (>15 years).Main Outcome Measures: Collected data included terminology used and surgical techniques. RESULTS: The 288 studies included demonstrated a wide range of terminology. In the infant group, 51/54 studies used the term "primary." In the immature group, 7/18 studies used the term "primary," 3/18 used "secondary." In the mature group, 2/33 studies used the term "primary," 16/33 used "secondary," 2/33 used "definitive," 5/33 used terms such as "mature," "adult," and "late," and 8/33 did not use terminology.Surgical technique assessment demonstrated: cleft rhinoplasty at infancy used nostril rim or no nasal incision, immature rhinoplasty used closed and open rhinoplasty incisions; and mature rhinoplasty used a majority of open rhinoplasty. Infant and immature cleft rhinoplasty incorporated septal harvest or spur removal in <10% of cases, whereas these procedures were common in mature rhinoplasty. No studies in infants or immature patients used osteotomies or septal grafts, common techniques in mature rhinoplasty. CONCLUSIONS: Current terminology for cleft rhinoplasty is varied and inconsistently applied across stages of facial development. However, cleft rhinoplasty performed at infancy, childhood, and facial maturity are surgically distinct procedures. The authors recommend the terminology "infant," "immature," and "mature" cleft rhinoplasty to accurately describe this procedure within the context of skeletal growth.

Mutations in TFAP2B and previously unimplicated genes of the BMP, Wnt, and Hedgehog pathways in syndromic craniosynostosis.

Craniosynostosis (CS) is a frequent congenital anomaly featuring the premature fusion of 1 or more sutures of the cranial vault. Syndromic cases, featuring additional congenital anomalies, make up 15% of CS. While many genes underlying syndromic CS have been identified, the cause of many syndromic cases remains unknown. We performed exome sequencing of 12 syndromic CS cases and their parents, in whom previous genetic evaluations were unrevealing. Damaging de novo or transmitted loss of function (LOF) mutations were found in 8 genes that are highly intolerant to LOF mutation (P = 4.0 × 10-8); additionally, a rare damaging mutation in SOX11, which has a lower level of intolerance, was identified. Four probands had rare damaging mutations (2 de novo) in TFAP2B, a transcription factor that orchestrates neural crest cell migration and differentiation; this mutation burden is highly significant (P = 8.2 × 10-12). Three probands had rare damaging mutations in GLI2, SOX11, or GPC4, which function in the Hedgehog, BMP, and Wnt signaling pathways; other genes in these pathways have previously been implicated in syndromic CS. Similarly, damaging de novo mutations were identified in genes encoding the chromatin modifier KAT6A, and CTNNA1, encoding catenin α-1. These findings establish TFAP2B as a CS gene, have implications for assessing risk to subsequent children in these families, and provide evidence implicating other genes in syndromic CS. This high yield indicates the value of performing exome sequencing of syndromic CS patients when sequencing of known disease loci is unrevealing.

Skeletal and Dental Correction and Stability Following LeFort I Advancement in Patients With Cleft Lip and Palate With Mild, Moderate, and Severe Maxillary Hypoplasia.

OBJECTIVE: This study evaluates skeletal and dental outcomes of LeFort I advancement surgery in patients with cleft lip and palate (CLP) with varying degrees of maxillary skeletal hypoplasia. DESIGN: Retrospective study. METHOD: Lateral cephalograms were digitized at preoperative (T1), immediately postoperative (T2), and 1-year follow-up (T3) and compared to untreated unaffected controls. Based on the severity of cleft maxillary hypoplasia, the sample was divided into 3 groups using Wits analysis: mild: ≤0 to ≥-5 mm; moderate: <-5 to >-10 mm; and severe: ≤-10 mm. PARTICIPANTS: Fifty-one patients with nonsyndromic CLP with hypoplastic maxilla who met inclusion criteria. INTERVENTION: LeFort I advancement. MAIN OUTCOME MEASURE: Skeletal and dental stability post-LeFort I surgery at a 1-year follow-up. RESULTS: At T2, LeFort I surgery produced an average correction of maxillary hypoplasia by 6.4 ± 0.6, 8.1 ± 0.4, and 10.7 ± 0.8 mm in the mild, moderate, and severe groups, respectively. There was a mean relapse of 1 to 1.5 mm observed in all groups. At T3, no statistically significant differences were observed between the surgical groups and controls at angle Sella, Nasion, A point (SNA), A point, Nasion, B point (ANB), and overjet outcome measures. CONCLUSIONS: LeFort I advancement produces a stable correction in mild, moderate, and severe skeletal maxillary hypoplasia. Overcorrection is recommended in all patients with CLP to compensate for the expected postsurgical skeletal relapse.

Three-Dimensional Nasolabial Changes After Nasoalveolar Molding and Primary Lip/Nose Surgery in Infants With Bilateral Cleft Lip and Palate.

OBJECTIVE: Utilize 3-dimensional (3D) photography to evaluate the nasolabial changes in infants with bilateral cleft lip and palate (BCLP) who underwent nasoalveolar molding (NAM) and primary reconstructive surgery. DESIGN: This is a retrospective serial longitudinal study of consecutively enrolled infants from September 2012 to July 2016 with BCLP who underwent NAM before primary lip and nose reconstructive surgery. It included infants who had digital 3dMD stereophotogrammetry records at initial presentation (T1), completion of NAM (T2), and 3 weeks following primary repair (T3). Twelve infants fulfilled the inclusion criteria. 3dMD Vultus software was used to orient images and plot 16 nasolabial points with x, y, z coordinates to obtain the linear and angular measurements. Nasal form changes were measured and analyzed between T1 (0.5 months old), T2 (5 months old), and T3 (6 months old). Intraclass correlation coefficient was performed for intrarater reliability. Averaged data from the 3D images was statistically analyzed from T1 to T2 and T2 to T3 with Wilcoxon tests. Unaffected infant norms from the Farkas publication were used as a control sample. RESULTS: After NAM therapy, statistically significant changes in the position of subnasale and labius superius improved nasolabial symmetry. Both retruded after NAM were displaced downward after NAM and surgical correction with respect to soft tissue nasion. The nasal tip's projection was maintained with NAM and surgical correction. The columella lengthened from 1.4 to 4.71 mm following NAM. CONCLUSIONS: There was a significant improvement in the nasolabial anatomy after NAM, and this was further enhanced after primary reconstructive surgery.

Clinical Outcomes in Orthognathic Surgery for Craniofacial Microsomia Following Mandibular Distraction Using CBCT Analysis: A Retrospective Study.

OBJECTIVE: The aim of this study was to evaluate the outcomes of orthognathic surgery (OGS) in patients with craniofacial microsomia (CFM) who had previously undergone mandibular distraction osteogenesis (MDO). DESIGN: A retrospective cohort study was performed including all patients with CFM who were treated with OGS at a single institution between 1996 and 2019. The clinical records, operative reports, and cone beam computed tomography (CBCT) scans were reviewed. CBCT data before OGS (T1), immediately after OGS (T2), and at long-term follow-up (T3) were analyzed using Dolphin three-dimensional software to measure the occlusal cant and chin point deviation. RESULTS: The study included 12 patients with CFM who underwent OGS (6 underwent OGS without MDO and 6 underwent OGS after MDO). There was a statistically significant improvement in occlusal cant and chin point deviation in both groups postoperatively. Occlusal cant relapsed by a mean of 0.6° (standard deviation [SD] 1.1°) in the patients who had OGS alone compared with 0.7° (SD 1.2°) in the patients who had OGS after MDO (P = .745) between T2 and T3. There was no statistically significant difference in chin point relapse between patients who had OGS alone compared with those who had OGS after MDO (0.1 mm [SD 2.5mm] vs 0.7mm [SD 2.2mm]; P = .808). CONCLUSIONS: Within the limitations of this study, these findings suggest that OGS after MDO in patients with CFM can produce stable results.

Skeletal and Dental Stability Following Different Magnitude of Le Fort I Advancement in Patients With Cleft Lip and Palate.

PURPOSE: The purpose of this study was to measure the association between the magnitude of advancement and dental and skeletal relapse in patients with cleft lip and palate (CLP). METHODS: A single-institution retrospective cohort study of skeletally matured patients with CLP who underwent isolated Le Fort I advancement surgery between 2013 and 2019 was studied. Patients were included if they had lateral cephalograms or cone-beam computed tomography (CBCT) at preoperative (T1), immediately postoperative (T2), and 1-year follow-up (T3). Lateral cephalometric landmarks were digitized and measured. The sample was divided on the basis of the magnitude of skeletal advancement: minor (<5 mm), moderate (≥5 but <10 mm), and major (≥10 mm) advancement groups. The mean advancement and relapse were compared between groups using 1-way ANOVA. Correlation between the amount of surgical advancement and relapse was evaluated. RESULTS: Forty-nine patients with nonsyndromic CLP with hypoplastic maxilla met inclusion criteria and the sample consisted of 36 males and 13 females with the mean age of 19.5 years. In the minor, moderate, and major advancement groups, the mean advancement at point A was +4.1 ± 0.4, + 7.5 ± 1.4, and +11.3 ± 1.3 mm, respectively. At 1-year follow-up, the mean relapse at point A was -1.3 ± 1.2, -1.1 ± 1.2, and -1.7 ± 1.5 mm, respectively. There was no significant difference in the relapse amount between all surgical groups. No correlation between the magnitude of advancement and relapse was found. CONCLUSIONS: This study demonstrated no statistically significant difference in skeletal stability between a minor (<5 mm), moderate (≥5 but <10 mm), and major (≥10 mm) Le Fort I advancement groups in patients with clefts. Regardless of the degree of advancement, mild skeletal relapse was observed in all 3 groups.

Ian Jackson's Sphincter Pharyngoplasty.

ABSTRACT: Velopharyngeal insufficiency (VPI) after cleft palate repair remains an intriguing problem for the cleft surgeon. While other options for the treatment of VPI, in many ways the sphincter pharyngoplasty has become a reliable and satisfying operation. When the applied to the properly selected patient, it rearranges the palatopharyngeus muscles to provide dynamic closure of the newly created central velopharngeal port. The dynamic action is particularly satisfying to the surgeon. The surgery evolved in part because of the dedication and creativity of Dr Ian Jackson who's description is closest to the design used today. In his memory we felt it fitting to review Dr Jackson's involvement with the surgery over the decades as well as include our own thoughts on the advantages of the procedure.

Crouzon Syndrome and Acanthosis Nigricans With Fibrous Dysplasia of the Maxilla: An Unreported Suggested Triad.

ABSTRACT: The aim of this report is to describe the combination of Crouzon syndrome and acanthosis nigricans with fibrous dysplasia of the maxilla. The diagnosis of fibrous dysplasia was confirmed clinically and pathologically during Le Fort III osteotomy and midface advancement with distraction osteogenesis. Crouzon syndrome with acanthosis nigricans is a known syndrome with an incidence of 1:1,000,000. This is the first report in the literature of Crouzon syndrome and acanthosis nigricans combined with fibrous dysplasia. As all 3 pathologies are related to fibroblasts, they may be different manifestations of malfunction of a single molecular pathway. The detection of fibrous dysplasia in a patient with Crouzon syndrome and acanthosis nigricans is important because it may complicate midface osteotomies and fixation of the hardware on the bones during craniofacial surgery.