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

Wangsrimongkol, Buddhathida; Flores, Roberto L; Staffenberg, David A; Rodriguez, Eduardo D; Shetye, Pradip R
PURPOSE/OBJECTIVE: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.
PMID: 34153247
ISSN: 1531-5053
CID: 4918192

Haploinsufficiency of SF3B2 causes craniofacial microsomia

Timberlake, Andrew T; Griffin, Casey; Heike, Carrie L; Hing, Anne V; Cunningham, Michael L; Chitayat, David; Davis, Mark R; Doust, Soghra J; Drake, Amelia F; Duenas-Roque, Milagros M; Goldblatt, Jack; Gustafson, Jonas A; Hurtado-Villa, Paula; Johns, Alexis; Karp, Natalya; Laing, Nigel G; Magee, Leanne; Mullegama, Sureni V; Pachajoa, Harry; Porras-Hurtado, Gloria L; Schnur, Rhonda E; Slee, Jennie; Singer, Steven L; Staffenberg, David A; Timms, Andrew E; Wise, Cheryl A; Zarante, Ignacio; Saint-Jeannet, Jean-Pierre; Luquetti, Daniela V
Craniofacial microsomia (CFM) is the second most common congenital facial anomaly, yet its genetic etiology remains unknown. We perform whole-exome or genome sequencing of 146 kindreds with sporadic (n = 138) or familial (n = 8) CFM, identifying a highly significant burden of loss of function variants in SF3B2 (P = 3.8 × 10-10), a component of the U2 small nuclear ribonucleoprotein complex, in probands. We describe twenty individuals from seven kindreds harboring de novo or transmitted haploinsufficient variants in SF3B2. Probands display mandibular hypoplasia, microtia, facial and preauricular tags, epibulbar dermoids, lateral oral clefts in addition to skeletal and cardiac abnormalities. Targeted morpholino knockdown of SF3B2 in Xenopus results in disruption of cranial neural crest precursor formation and subsequent craniofacial cartilage defects, supporting a link between spliceosome mutations and impaired neural crest development in congenital craniofacial disease. The results establish haploinsufficient variants in SF3B2 as the most prevalent genetic cause of CFM, explaining ~3% of sporadic and ~25% of familial cases.
PMCID:8333351
PMID: 34344887
ISSN: 2041-1723
CID: 4988632

Craniosynostosis: Le Fort III Distraction Osteogenesis

Mittermiller, Paul A; Flores, Roberto L; Staffenberg, David A
The Le Fort III advancement was first described in 1950 and has since become a key technique in the armamentarium of craniofacial surgeons. The application of distraction osteogenesis to the craniofacial skeleton has allowed for large movements to be performed safely in young patients. This technique is valuable for correcting exorbitism, airway obstruction owing to midface retrusion, and class III malocclusion. It can be performed with either an external distractor or internal distractors. Although serious complications have been reported, these occur rarely when performed by experienced providers.
PMID: 34051899
ISSN: 1558-0504
CID: 4890662

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

Mancini, Laura; Avinoam, Shayna; Grayson, Barry H; Flores, Roberto L; Staffenberg, David A; Shetye, Pradip R
OBJECTIVE/UNASSIGNED: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/UNASSIGNED: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/UNASSIGNED: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/UNASSIGNED:There was a significant improvement in the nasolabial anatomy after NAM, and this was further enhanced after primary reconstructive surgery.
PMID: 34032145
ISSN: 1545-1569
CID: 4887702

Ian Jackson's Sphincter Pharyngoplasty

Mittermiller, Paul A; Staffenberg, David A
ABSTRACT/UNASSIGNED: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.
PMID: 33481472
ISSN: 1536-3732
CID: 4761012

The Nasoalveolar Molding Cleft Protocol: Long-Term Treatment Outcomes from Birth to Facial Maturity

Yarholar, Lauren M; Shen, Chen; Wangsrimongkol, Buddhathida; Cutting, Court B; Grayson, Barry H; Staffenberg, David A; Shetye, Pradip R; Flores, Roberto L
BACKGROUND:The authors present outcomes analysis of the nasoalveolar molding treatment protocol in patients with a cleft followed from birth to facial maturity. METHODS:A single-institution retrospective review was conducted of cleft patients who underwent nasoalveolar molding between 1990 and 2000. Collected data included surgical and orthodontic outcomes and incidence of gingivoperiosteoplasty, alveolar bone grafting, surgery for velopharyngeal insufficiency, palatal fistula repair, orthognathic surgery, nose and/or lip revision, and facial growth. RESULTS:One hundred seven patients met inclusion criteria (69 with unilateral and 38 with bilateral cleft lip and palate). Eighty-five percent (91 of 107) underwent gingivoperiosteoplasty (unilateral: 78 percent, 54 of 69; bilateral: 97 percent, 37 of 38). Of those patients, 57 percent (52 of 91) did not require alveolar bone grafting (unilateral: 59 percent, 32 of 54; bilateral: 54 percent, 20 of 37). Twelve percent (13 of 107) of all study patients underwent revision surgery to the lip and/or nose before facial maturity (unilateral: 9 percent, six of 69; bilateral: 18 percent, seven of 38). Nineteen percent (20 of 107) did not require a revision surgery, alveolar bone grafting, or orthognathic surgery (unilateral: 20 percent, 14 of 69; bilateral: 16 percent, six of 38). Cephalometric analysis was performed on all patients with unilateral cleft lip and palate. No significant statistical difference was found in maxillary position or facial proportion. Average age at last follow-up was 20 years (range, 15 years 4 months to 26 years 10 months). CONCLUSIONS:Nasoalveolar molding demonstrates a low rate of soft-tissue revision and alveolar bone grafting, and a low number of total operations per patient from birth to facial maturity. Facial growth analysis at facial maturity in patients who underwent gingivoperiosteoplasty and nasoalveolar molding suggests that this proposal may not hinder midface growth. CLINICAL QUESTION/LEVEL OF EVIDENCE/METHODS:Therapeutic, IV.
PMID: 33890899
ISSN: 1529-4242
CID: 4847552

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

Wangsrimongkol, Buddhathida; Flores, Roberto L; Staffenberg, David A; Rodriguez, Eduardo D; Shetye, Pradip R
OBJECTIVE/UNASSIGNED: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/UNASSIGNED:Retrospective study. METHOD/UNASSIGNED:: ≤-10 mm. PARTICIPANTS/UNASSIGNED:Fifty-one patients with nonsyndromic CLP with hypoplastic maxilla who met inclusion criteria. INTERVENTION/UNASSIGNED:LeFort I advancement. MAIN OUTCOME MEASURE/UNASSIGNED:Skeletal and dental stability post-LeFort I surgery at a 1-year follow-up. RESULTS/UNASSIGNED: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/UNASSIGNED: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.
PMID: 33722088
ISSN: 1545-1569
CID: 4817482

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

Olshinka, Asaf; Tal, David; Gillman, Leon; Ad-El, Dean; Kalish, Eyal; Kropach, Nesia; Yaacobi, Dafna Shilo; Kornreich, Liora; Staffenberg, David A
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.
PMID: 33156176
ISSN: 1536-3732
CID: 4664472

Feasibility and Perception of Cross-sex Face Transplantation to Expand the Donor Pool

Sosin, Michael; Robinson, Isabel S; Diep, Gustave K; Alfonso, Allyson R; Maliha, Samantha G; Ceradini, Daniel J; Levine, Jamie P; Staffenberg, David A; Saadeh, Pierre B; Rodriguez, Eduardo D
Background/UNASSIGNED:A major challenge in face transplantation (FT) is the limited donor allograft pool. This study aimed to investigate the feasibility of cross-sex FT (CSFT) for donor pool expansion by: (1) comparing craniomaxillofacial metrics following CSFT versus same-sex FT (SSFT); and (2) evaluating the public and medical professionals' perception of CSFT. Methods/UNASSIGNED:Seven cadaveric FTs were performed, resulting in both CSFT and SSFT. Precision of bony and soft tissue inset was evaluated by comparing pre- versus post-operative cephalometric and anthropometric measurements. Fidelity of the FT compared to the virtual plan was assessed by imaging overlay techniques. Surveys were administered to medical professionals, medical students, and general population to evaluate opinions regarding CSFT. Results/UNASSIGNED:< 0.001). On non-blinded and blinded assessments, 62.9% and 79% of responses rated the CSFT superior or equal to SSFT, respectively. Conclusions/UNASSIGNED:Our study demonstrates similar anthropometric and cephalometric outcomes for CSFT and SSFT. Participants were more reticent to undergo CSFT, with increased willingness if supported by research. CSFT may represent a viable option for expansion of the donor pool in future patients prepared to undergo transplantation.
PMCID:7544392
PMID: 33133951
ISSN: 2169-7574
CID: 4655852

The Effects of Nasoalveolar Molding on Nasal Proportions at the Time of Nasal Maturity

Maliha, Samantha G; Kantar, Rami S; Gonchar, Marina N; Eisemann, Bradley S; Staffenberg, David A; Shetye, Pradip R; Grayson, Barry H; Flores, Roberto L
BACKGROUND/UNASSIGNED:The aim of this study is to assess the effect of nasoalveolar molding (NAM) versus no-NAM on nasal morphology in patients with unilateral cleft lip and palate (UCLP) at the time of nasal maturity. METHODS/UNASSIGNED:A retrospective, single-institution review was conducted on all non-syndromic patients with UCLP. Inclusion criteria included age 14 years or above, unilateral cleft repair at the time of infancy, and adequate photography taken at nasal maturity and prior to rhinoplasty. Exclusion criteria included age less than 14 years, syndromic diagnosis, and rhinoplasty prior to nasal maturity. Ten parameters were measured twice from standardized clinical photographs using the Dolphin Imaging Software for establishment of intrarater reliability. Subjective analysis was achieved through completion of the Asher McDade grading scale by 3 expert cleft practitioners. RESULTS/UNASSIGNED:Nostril height, columellar angle, alar cant, vertical alar height, alar height angle, nasofacial angle, and nasolabial angle were found to be significantly less severe in patients who had undergone NAM in conjunction with surgical repair when compared with those who had undergone surgical repair alone. Asher McDade grading revealed significant improvement in nasal form, nasal symmetry/deviation, nasal profile, vermillion border, and overall score in patients who underwent NAM compared to no-NAM. CONCLUSION/UNASSIGNED:The use of presurgical NAM during infancy can improve nasal symmetry and nasal proportions at the time of nasal maturity.
PMID: 32851868
ISSN: 1545-1569
CID: 4575782