Midfacial Growth Research Articles

The impact of spheno-occipital synchondrosis exposure via extended endoscopic endonasal surgery on midface growth in pediatric patients.

Pediatric extended endonasal procedures pose significant surgical challenges. Lesions from the suprasellar region to the lower clivus necessitate extensive exposure. This study examined whether drilling the spheno-occipital synchondrosis (SOS) to remove the posterior clinoid process and dorsum sellae (DS) for greater exposure affects pediatric midfacial growth. From 2014 to 2020, the authors performed endoscopic endonasal surgery (EES) in 14 patients aged 12 years or younger. The lesions consisted of 11 cases of craniopharyngioma, 1 pituitary neuroendocrine tumor, 1 Rathke's cleft cyst, and 1 Langerhans cell histiocytosis. In 8 of the 14 cases, an extended EES procedure was used by exposing the SOS to remove the posterior clinoid process and DS. Measurement of the central face was based on head MRI before and after surgery. Measuring points were the sellae-nasion (SN) plane, the foremost points of the anterior maxilla (point A), and the maximum concavity point of the mandibular symphysis (point B). The authors measured and evaluated the SNA angle (angle created by the SN plane and the NA [a line connecting point A and the nasion] plane), SNB angle (angle created by the SN plane and the NB [a line connecting point B and the nasion] plane), and the ANB angle (angle created by the NA plane and the NB plane). In addition, a comparison was made with 6 pediatric cases in which transcranial surgery was performed for craniopharyngiomas. In the extended EES group, the average preoperative age was 7 years, and the average postoperative age was 12 years. Mean preoperative angles in this group were 84° (SNA), 80.9° (SNB), and 3.1° (ANB); mean postoperative angles were 83.5° (SNA), 83.9° (SNB), and -0.4° (ANB). In the standard EES group, the average preoperative age was 9 years, and the average postoperative age was 14.5 years. Average preoperative angles in the standard EES group were 83° (SNA), 80.3° (SNB), and 2.7° (ANB); average postoperative angles were 82.7° (SNA), 81° (SNB), and 1.6° (ANB). In the transcranial surgery group, the average preoperative age was 4.5 years, and the average postoperative age was 9.8 years. Mean preoperative angles were 83.8° (SNA), 80.3° (SNB), and 3° (ANB); mean postoperative angles were 83.8° (SNA), 82.6° (SNB), and 1.2° (ANB). The only significant difference between groups was the postoperative ANB angle, which was negative in the extended EES group compared to the standard EES group, indicating the maxilla was positioned posteriorly compared to the mandible. The measurement values of the EES groups and the transcranial surgery group exhibited minimal differences, except for a significant decrease in the postoperative ANB angle in the extended EES group compared with the standard EES group. These results show that extended EES may impact midface growth. Further research is required to understand the long-term impact of SOS exposure.

Orthognathic Surgery Rates in Furlow Double-Opposing Z-Plasty Versus Straight-Line Repair: A Review of Three Decades of Experience.

The influence of different surgical techniques on maxillary growth remains unclear. This study investigates the long-term impact of Furlow double-opposing Z-plasty versus straight-line repair (SLR) techniques on midface growth and subsequent orthognathic surgery. Retrospective cohort study. Tertiary children's hospital. This study evaluated patients who underwent primary palatoplasty with Furlow or SLR techniques from 1994-2023. Patients were >14 years old at their most recent follow-up. No interventions were performed. Primary outcomes were orthognathic surgery and orthognathic surgery recommendation rates to correct midface hypoplasia (MFH). Cephalometrics at the time of orthognathic surgery recommendation were traced to validate MFH. In total, 1857 patients underwent palatoplasty, of which 335 met inclusion criteria (49 SLR, 286 Furlow). Average age at last follow-up was 18.5±2.6 years. Patients who underwent Furlow versus SLR showed no significant difference in orthognathic surgery rates (p=0.428) or recommendation for orthognathic surgery rates (p=0.900). Patients recommended to undergo orthognathic surgery had more negative ANB angles (p<0.001) and smaller SNA angles (p<0.001) than patients not recommended for orthognathic surgery, demonstrating maxillary hypoplasia. Upon multivariate regression, patients with Veau III and IV clefts had an increased need for orthognathic surgery, p=0.047 and p=0.008, respectively. Our findings suggest that higher cleft severity contributes to future orthognathic surgery. However, palatoplasty technique did not influence orthognathic surgery rates. Our results provide valuable data when surgeons are considering the impact of palatoplasty technique on sagittal growth restriction.

Septoplasty During Primary Cleft Lip Reconstruction: A Historical Perspective and Scoping Review.

Traditional surgical approaches excluded septoplasty at primary cleft lip reconstruction due to concerns about restricted nasal and midfacial growth. Modern opinion in the treatment of cleft lip has increasingly employed primary septoplasty; this scoping review and historical perspective aims to chronicle the evolution of septoplasty in patients born with cleft lip and palate and discuss current evidence. The historical perspective explicitly contrasts American and European perceptions of septoplasty in cleft lip deformity and the competing anatomical theories of the role of the septum on midfacial and nasal growth. For the scoping review, articles were extracted from Embase, PubMed, and Medline, as well as manual searches of reference lists. Results were compiled, grouped, and appraised by date, outcomes, and historical significance. Inclusion criteria consisted of children who underwent primary septoplasty for any indication and were followed up on outcomes of facial growth and nasal function. Literature reviews, opinion articles, case reports, guidelines, or studies not available in English or online were excluded. Evolving anatomical theories relating to midfacial growth in the mid-late 20th century underpinned a progressive ideological shift on the safety and efficacy of septoplasty in children. This is supported by our scoping review, which included 23 articles mutually selected for inclusion by 2 blinded assessors. Several competing methods have been employed to measure endpoints on facial growth and nasal function, but generally indicate primary septoplasty is successful in improving nasal function and preserving midfacial growth. Perceptions towards septoplasty on facial growth in the pediatric population have transformed significantly and suggest a growing acceptance of primary septoplasty techniques in patients born with a cleft lip.

Palatal segment contributions to midfacial anterior-posterior growth.

Anterior-posterior (A-P) elongation of the palate is a critical aspect of integrated midfacial morphogenesis. Reciprocal epithelial-mesenchymal interactions drive secondary palate elongation that is coupled to the periodic formation of signaling centers within the rugae growth zone (RGZ). However, the relationship between RGZ driven morphogenetic processes, the differentiative dynamics of underlying palatal bone mesenchymal precursors, and the segmental organization of the upper jaw has remained enigmatic. A detailed ontogenetic study of these relationships is important, because palatal segment growth is a critical aspect of normal midfacial growth, can be modified to produce dysmorphology, and is a likely basis for evolutionary differences in upper jaw morphology. Variation in palatal-segment specific growth may also underlie known differences in palatal segment proportions between inbred mouse strains. We completed a combined whole mount gene expression and morphometric analysis of normal murine palatal growth dynamics and their association with palatal segment elongation and resulting upper jaw morphology. Our results demonstrated that the first formed palatal ruga (ruga 1), found just posterior to the RGZ, maintained an association with important nasal, neurovascular and palatal structures throughout early midfacial development; suggesting that these features are positioned at a proximal source of embryonic midfacial directional growth. Our detailed characterization of midfacial morphogenesis revealed a one-to-one relationship between palatal segments and upper jaw bones during the earliest stages of palatal elongation. Growth of the maxillary anlage within the anterior secondary palate is uniquely coupled to RGZ-driven morphogenesis that more than doubles the length of this palatal segment prior to palatal shelf fusion. Our results also demonstrate that the future maxillary-palatine suture, approximated by the position ruga 1 and consistently associated with the palatine anlage, forms predominantly via the posterior differentiation of the maxilla within the expanding anterior secondary palate. Our complementary ontogenetic comparison of three inbred mouse strains identified small but significant strain-specific differences in early embryonic palatal segment contributions to the upper jaw. Although early palatal segment specific growth is not primarily responsible for adult differences in upper jaw morphology between these strains, our ontogenetic series of measurements provide a useful foundation for understanding the impact of background genetic effects on facial shape and elongation. In combination, our results provide a novel and particularly detailed picture of the earliest spatiotemporal dynamics of intramembranous midfacial skeletal specification and differentiation within the context of the surrounding palatal segment A-P elongation and associated rugae formation.

Distraction Osteogenesis and Endoscopic Suturectomy in the Treatment of Rachitic Craniosynostosis: A Case Series and Updated Literature Review With Discussion of Cephalometrics

Background: Individuals with genetic or nutritional rickets frequently suffer from craniosynostosis. While cranial vault remodeling is most often performed, other approaches, such as distraction osteogenesis and endoscopic suturectomy, are infrequently reported. Here, we discuss the status of the current literature and describe 2 patients with rachitic craniosynostosis, one of whom had previously undergone endoscopic suturectomy, who both successfully underwent distraction osteogenesis. Case description: Two male patients with rickets-associated craniosynostosis, 1 genetic and 1 nutritional, underwent posterior-vault distraction osteogenesis (PVDO). The patient with genetic rickets previously underwent endoscopic-assisted cranial suturectomy of the sagittal suture with barrel-staving at an outside hospital and subsequently re-fused all cranial sutures 19 months postoperatively necessitating reintervention. Complete distraction was confirmed by X-ray and both patients recovered without complication and without re-synostosis at 18 month follow-up. The patient with nutritional rickets required subsequent fronto-facial advancement because of poor midface growth. Conclusion: PVDO effectively treats rachitic craniosynostosis associated with both genetic and nutritional etiologies. Long-term follow up is warranted to monitor for skull-base involvement and associated midface retrusion in these patients.

The Influence of Sidedness in Unilateral Cleft Lip and Palate on Mid Facial Growth at Five Years of Age.

To determine whether facial growth at five years is different for children with a left versus right sided cleft lip and palate. Retrospective cohort study. Seven UK regional cleft centres. Patients born between 2000-2014 with a complete unilateral cleft lip and palate (UCLP). 5-Year-Old's Index scores. 378 children were included. 256 (68%) had a left sided UCLP and 122 (32%) had a right sided UCLP. 5-Year-Old's index scores ranged from 1 (good) to 5 (poor). There was a higher proportion of patients getting good scores (1 and 2) in left UCLP (43%) compared to right UCLP (37%) but there was weak evidence for a difference (Adjusted summary odds ratio 1.27, 95% CI 0.87 to 1.87; P = .22). Whilst maxillary growth may be different for left versus right sided UCLP, definitive analysis requires older growth indices and arch forms.

Long-Term Outcomes in Pediatric Midfacial Growth Following Expanded Endonasal Skull Base Surgery for Craniopharyngioma.

Long-Term Outcomes in Pediatric Midfacial Growth Following Expanded Endonasal Skull Base Surgery for Craniopharyngioma.

Symmetrical Midfacial Growth After Pediatric Mandibular Reconstruction With Free Fibula Flap

Free fibula is the workhorse flap for mandibular reconstruction and is increasingly being used in pediatric patients. However, craniomaxillofacial growth and development involve interdependent processes, and it remains unknown whether mandibular reconstruction with free fibula allows symmetric growth of the midface. The study evaluated midfacial symmetry after pediatric mandibular defect reconstruction. This retrospective cohort study included pediatric patients aged ≤14years who underwent mandibular reconstruction with free fibula flap. Postoperative computed tomography data were obtained at predefined follow-up time points. Midfacial symmetry was evaluated based on 3-dimensional (3D) cephalometry. The predictor variable was the side of the midface (affected or healthy side relative to the mandibular defect). The primary outcome variable was postoperative midfacial symmetry (at 1week, 6months, 1year, 2years, and >3years, or after the age of 18years), assessed in horizontal, vertical, and anteroposterior dimensions using 3D cephalometry. Another outcome variable was patient satisfaction based on a self-evaluation using visual analog scoring. Sex, age, diagnosis, and type of denture restoration. Paired t tests were performed to assess the relationship between the predictor and outcome variables, with the significance level of P<.05. A total of 13 patients were included in this study (9 males and 4 females; mean age: 12.23±2.39years). The average distance from upper first molar point (U6) to the horizontal plane on the affected side became greater than on the healthy side (difference: 0.7±0.5mm to 1.6±1.4mm, P<.05), while the average distance from pterygomaxillary fissure to coronal plane on affected side became shorter than that on the healthy side (difference: 0.6±0.6mm to 1.2±1.1mm, P<.05) from 1year after the surgery. There were no statistically significant differences in the remaining measurements between the 2 sides (P>.05). All the patients were satisfied with their postoperative facial symmetry. There were no severe midface deformities after pediatric mandibular reconstruction with free fibula flap. Meanwhile, pediatric mandibular reconstruction and proper occlusion could promote midfacial growth and symmetry.

The Effects of Gingivoperiosteoplasty and Cleft Palate Repair on Facial Growth.

Gingivoperiosteoplasty (GPP) can avoid secondary alveolar bone graft in up to 60% of patients. The effects of GPP on maxillary growth are a concern. However, palatoplasty can also negatively impact facial growth. This study quantifies the isolated effects of GPP and cleft palate repair on maxillary growth at the age of mixed dentition. A single-institution, retrospective study of all patients undergoing primary reconstruction for unilateral cleft lip and alveolus (CLA) or cleft lip and palate (CLP) was performed. Study patients had lateral cephalograms at the age of mixed dentition. Patients were stratified into four groups: CLA with GPP (CLA + GPP), CLA without GPP (CLA - GPP), CLP with GPP (CLP + GPP), and CLP without GPP (CLP - GPP). Cephalometric measurements included the following: sella-nasion-point A (SNA), sella-nasion-point B (SNB), and A point-nasion-B point. Landmarks were compared between patient groups and to Eurocleft center D data. One hundred ten patients met inclusion criteria: seven CLA - GPP, 16 CLA + GPP, 24 CLP - GPP, and 63 CLP + GPP patients. There were no significant differences in SNA, SNB, and A point-nasion-B point between CLA + GPP and CLA - GPP, or between CLP + GPP and CLP - GPP groups. In patients who did not receive GPP, SNA was significantly lower in patients with a cleft palate compared with patients with an intact palate ( P < 0.05). There were no significant differences in SNA or SNB of CLP - GPP or CLP + GPP groups when compared with Eurocleft data. When controlling for the effects of cleft palate repair, GPP does not appear to negatively affect midface growth at the age of mixed dentition. Therapeutic, III.

The nasal septum and midfacial growth.

The nasal septum is the only element of the chondrocranium which never completely ossifies. The persistence of this nonarticular cartilage has given rise to a variety of theories concerning cranial mechanics and growth of the midface. Previously, using pigs, we demonstrated that the septum is not a strut supporting the snout and that septal growth seems capable of stretching the overlying nasofrontal suture, a major contributor to snout elongation. Here we investigate whether abnormalities of the septum are implicated in cases of midfacial hypoplasia, in which growth of the midface is inadequate. Mild midfacial hypoplasia is common in domestic pig breeds and often severe in the Yucatan minipig, a popular laboratory breed. Normal-snouted and midfacial hypoplastic heads of standard (farm mixed breed) and minipigs ranging in age from perinatal to 12 months were dissected, imaged by CT, and/or prepared for histology. Even at birth, Yucatan minipigs with midfacial hypoplasia exhibited greater caudal ossification than normal; the ventral cartilaginous sphenoidal "tail" was diminished or missing. In addition, cells that morphologically appeared to have divided recently were less numerous than in newborn standard pigs. Juvenile Yucatan minipigs lacked caudal cartilaginous growth zones almost completely. In standard newborns, the ventral caudal septum was more replicative than the dorsal, but this trend was not seen in Yucatan newborns. In conclusion, accelerated maturation of the caudal septum was associated with midfacial hypoplasia, a further indication that the septum, particularly its ventral portion, is important for midfacial elongation.

Clinical Outcomes of Bilateral Cleft Lip and Palate Repair with Nasoalveolar Molding and Gingivoperiosteoplasty to Facial Maturity.

The long-term effects of nasoalveolar molding (NAM) on patients with bilateral cleft lip and palate (BCLP) are unknown. The authors report clinical outcomes of facially mature patients with complete BCLP who underwent NAM and gingivoperiosteoplasty (GPP). A single-institution retrospective study of nonsyndromic patients with complete BCLP who underwent NAM between 1991 and 2000 was performed. All study patients were followed to skeletal maturity, at which time a lateral cephalogram was obtained. The total number of cleft operations and cephalometric measures was compared with a previously published external cohort of patients with complete and incomplete BCLP in which a minority (16.7%) underwent presurgical orthopedics before cleft lip repair without GPP. Twenty-four patients with BCLP comprised the study cohort. All patients underwent GPP, 13 (54.2%) underwent alveolar bone graft, and nine (37.5%) required speech surgery. The median number of operations per patient was five (interquartile range, two), compared with eight (interquartile range, three) in the external cohort ( P < 0.001). Average age at the time of lateral cephalogram was 18.64 years (1.92). There was no significant difference between our cohort and the external cohort with respect to sella-nasion-point A angle (SNA) [73 degrees (6 degrees) versus 75 degrees (11 degrees); P = 0.186] or sella-nasion-point B angle (SNA) [78 degrees (6 degrees) versus 74 degrees (9 degrees); P = 0.574]. Median ANB (SNA - SNB) was -3 degrees (5 degrees) compared with -1 degree (7 degrees; P = 0.024). Twenty patients (83.3%) underwent orthognathic surgery. Patients with BCLP who underwent NAM and GPP had significantly fewer total cleft operations and mixed midface growth outcomes at facial maturity compared with patients who did not undergo this treatment protocol. Therapeutic, III.

Impact of Minor Suture Fusion on Midface Hypoplasia in Syndromic Craniosynostosis

Purpose: Early onset of minor suture fusion in syndromic craniosynostosis is associated with midface dysplasia and is a common indication for craniofacial surgery. However, the potential effects of fusion severity on craniofacial growth patterns are not well understood. This study seeks to describe the impact of minor suture fusion severity on midface morphology in Crouzon syndrome. Methods: Pre-operative computed tomography images (CT) of 63 patients with Crouzon syndrome and 63 normocepahlic controls were included. Degree of suture fusion was assessed for the frontosphenoidal, sphenethmoidal, sphenosquamosal, sphenopetrosal, spheno-occipital synchondrosis, frontoethmoidal, and zygomaticosphenoidal sutures. Each suture was graded on a 5-point scale. The sella (S), nasion (N), A point (A), basion (BA), and anterior nasal spine (ANS) landmarks were used to calculate the SNA angle, BA-ANS length of the lower midface, and N-S length of the upper midface. Multiple linear regressions were used to analyze data. Results: The mean age was 43 months and 44% were female. The control group was significantly older ( P &lt; .01) than the patients with Crouzon syndrome. Advanced fusion of the spheno-occipital synchondrosis in Crouzon syndrome correlates with regression of the BA-ANS length by 0.563 mm per incremental increase in suture fusion ( P &lt; .01). Additionally, the lower midface (BA-ANS) was restricted to a greater degree than the upper midface (N-S) with progressive suture fusion in all patient types with ratios of these rates ranging between 0.602 and 0.89 for the 7 sutures analyzed. Suture fusion severity did not impact the SNA angle in any of the analyses performed. Conclusion: The severity of sheno-occipital synchondrosis fusion in Crouzon syndrome contributes to midface hypoplasia. Similarly, all anterior skull base sutures limited lower midface growth to a greater degree than the upper midface.

Pediatric septorhinoplasty: Current attitudes and practices by facial plastic and reconstructive surgeons

ObjectiveThere is hesitation to offer pediatric patients rhinoplasty due to concerns about postoperative effect on midface growth. A cross-sectional survey of members of the American Academy of Facial Plastic and Reconstructive Surgery was conducted regarding practice information and attitudes towards pediatric septorhinoplasty. The goal of the study is to describe the current attitudes on pediatric septorhinoplasty. Study designCross-sectional survey. SettingCommunity members of the American Academy of Facial Plastic and Reconstructive Surgery society. MethodsA 19-question survey was distributed to surgeons surveying background information and current attitudes towards pediatric septorhinoplasty practices. Fisher's exact tests were implemented using Monte Carlo methods. ResultsThere were 94 total respondents. A majority believed septorhinoplasty is safe in patients <16 years of age (n = 68, 72.34 %) with most choosing either 16 years (n = 30, 31.91 %) or 14 years (n = 29, 30.85 %) as the minimum age to consider the procedure. A majority of respondents would not perform any nasal procedures in patients ≤12 years (n = 40, 43.48 %). ConclusionTrends in pediatric rhinoplasty practices have evolved overtime. Despite prior beliefs and studies cautioning against performing septorhinoplasty in pediatric patients (<16 years of age), a majority of practicing facial plastic surgeons believe that pediatric septorhinoplasty can be performed in patients >14 years old.Level of evidence.IV.

Sexual dimorphism in human midfacial growth patterns from newborn to 5 years old based on computed tomography.

Recent studies have supported the presence and varying nature of craniofacial sexual dimorphism (SD) from the very first stages of ontogeny. But the exact patterns of between-sex differences during the first years of life remain obscure despite the importance of these data for craniofacial surgery treatment and forensic studies. Our study employs a large dataset of clinical computed tomography scans of individuals of East Slavonic descent from birth to 5 years of age (247 males and 184 females) to address the pattern of age-related between-sex differences in 22 linear measurements of the mid-face. At birth, SD of most dimensions is low, but it increases significantly during the first year of life. The level of SD of most variables fluctuates in both directions during the second year and peaks during the third and fourth years of life. During the sixth year, SD of about half of the variables markedly decreases. In adults, SD of all variables increases, but to a very different extent: from 2% to 13%. Most sexually dimorphic features of the facial skeleton begin to develop early in postnatal ontogeny and then may or may not become accentuated during puberty. Importantly, the patterns of age changes in the level of SD differ strongly between various dimensions, and so cannot be expressed by a single value for the whole face. Additionally, the level of SD for a particular variable is not ontogenetically stable during the first years of life.

Interrelationship Between Microstructure &amp; Macrostructure in the Growing Hard Palate

Bone strength is the result of microstructure (material properties) and macrostructure (size and shape), and deficiencies in either can produce skeletal fragilities with increased likelihood of injury. The micro‐ and macro‐architecture work together during re/modeling, ensuring that bone is resistant to repetitive loading and fracture. Bone is particularly responsive to loading during the rapid growth occurring in early life. Within the craniofacial complex, the hard palate is a unique structure that undergoes continuous loading due to resting/active tongue pressure and bite force. Because of this, palatal growth is theorized to drive midfacial growth. The aim of this study is to investigate longitudinal changes in palatal micro‐ and macro‐structure in the context of a perturbation to type I collagen (Col1). Col1 is the structural protein that forms osteoid (the precursor to bone), forms a scaffold for bone mineralization, and determines bone toughness. Here we use the OIM mouse (B6C3FE a/a‐Col1a2OIM/J), a strain with a Col1 mutation. We hypothesize that OIM and unaffected wild‐type (WT) mice will be most similar in bone micro‐ and macro‐structure in regions of the palate that experience high loading and therefore necessitate high rates of bone modeling or remodeling to form a structure which can withstand the stress.Mice were micro‐CT scanned at two timepoints: week 4 (juvenile) and week 16 (adult). BMD was collected at 3 regions (anterior, mid, and posterior palate). Mann‐Whitney U tests were used to compare BMD between genotypes. 28 fixed and 10 sliding landmarks were placed across the palate. Geometric morphologic analyses were conducted to determine variation between genotypes in overall shape and curvature throughout growth.Average BMD decreased along an anteroposterior gradient for all mice. While juvenile OIM mice had lower palate BMD than WT mice at all regions, adult OIM mice had significantly lower BMD at only the anterior and posterior regions but not the midpalate. Procrustes ANOVA revealed significant differences in palatal shape between the genotypes at both the juvenile (p=0.001) and adult (p=0.0015) stage. Principal component analyses revealed that juvenile OIM mice had a shorter anterior palate and broader palate compared to WT mice, while adult OIM mice had a shorter posterior palate than WT mice. OI mice had flatter palatal arches in the coronal plane as juveniles, and in the midsagittal plane as juveniles and adults.Results show significant differences in palatal BMD and morphology between genotypes throughout growth, with fewer significant differences at the adult stage. Increased loading of the anterior palate during incisive gnawing may result in the convergence of bone macrostructure among adult mice, while increased strain at the midpalatal suture may necessitate increased BMD even in the presence of a biomineralization defect. Further research is needed to understand the functional significance of the anteroposterior palatal gradient and its relation to soft tissue attachments, particularly given the transition in oral behaviors between juveniles and adults.

Long-term Management of a Patient with Apert Syndrome

To present an Apert syndrome patient with midfacial growth deficiency treated with Le Fort III distraction osteogenesis and subsequent two-jaw surgery. Apert syndrome is expressed as a severe and irregular craniosynostosis, midfacial hypoplasia, and symmetric syndactyly in the fingers and toes. For craniosynostosis syndromes, treatment planning is complex due to the disharmony between facial profile and occlusion. A 4-year-and-5-month-old boy, diagnosed with Apert syndrome, showed a concave profile accompanied with midfacial hypoplasia, moderate exorbitism, a reversed occlusion of -10.0 mm, an anterior open bite of -5.0 mm, and skeletal class III jaw-base relationship. The patient, aged 15 years and 4 months, underwent a Le Fort III osteotomy, and subsequent osteodistraction was performed via a rigid external distraction (RED) device. His midfacial bone was advanced by approximately 7.0 mm. One year after the distraction, preoperative treatment with 0.018-in preadjusted edgewise appliances was initiated. Two-jaw surgery with a Le Fort I osteotomy and bilateral sagittal split ramus osteotomy was performed after 42 months of preoperative orthodontic treatment. At the age of 20 years and 9 months, his facial profile dramatically changed to a straight profile, and an acceptable occlusion with an adequate interincisal relationship was obtained. A functional occlusion with an excellent facial profile was maintained throughout the 2-year retention period, although the upper dental arch width was slightly decreased, resulting in the recurrence of the left posterior crossbite. Our report indicates the necessity of long-term follow-up in patients with craniosynostosis because of syndrome-specific growth and methodologically induced relapse. The two-stage operation combining early distraction osteogenesis and postgrowth orthognathic surgery proves to be an effective therapy for correcting midfacial hypoplasia and skeletal mandibular protrusion caused by Apert syndrome.

Surgically assisted rapid palatal expansion for transverse maxillary discrepancy in adults - Case report.

Introduction and importanceTransverse maxillary deficiency is one of the most detrimental problems to midfacial growth and the integrated dentoalveolar structures. Early diagnosis and proper treatment of such cases is most important to maintain the balance between the basal bones and stable occlusion.Case presentationIn our case, a 17-year-old male had irregular upper front teeth with an unpleasant smile. Detail examination revealed a symmetrical face with an orthognathic profile, mild malar deficiency, competent lips, asymmetrical arches, Class I molar and canine relationships bilaterally. Crowding was present in the upper anterior arch with 2 mm of anterior open bite and posterior cross bite present in the premolar region and molar region bilaterally. Lefort-1 osteotomy, midpalatal split, pterygomandibular disjunction without down fracture was done. The HYRAX appliance was cemented. Distraction started after four days of surgery. One mm distraction per day was done for 10 days. The patient was transferred to fixed orthodontic treatment to relive the anterior crowding. Records were taken after 1 year of follow up and analyzed. Skeletal relationships were in harmony. Dental crowding, anterior open bite and posterior crossbite were corrected.Clinical discussionThe zygomatic buttress and the pterygomaxillary junction are considered as the critical areas of resistance for maxillary expansion. Literature claims lefort-1 osteotomy in combination with palatal distraction results in more displacement and less stress in the maxilla.ConclusionSARPE has proved to be clinically effective and stable for the correction of transversely deficient maxilla after cessation of growth in adult patients.

Gingivosupraperiosteoplasty following Presurgical Maxillary Orthopedics Is Associated with Normal Midface Growth in Complete Unilateral and Bilateral Cleft Patients at Mixed Dentition.

Passive orthodontic appliances and gingivosupraperiosteoplasty are adjuncts that can be used by surgeons at the time of primary cleft lip repair. These treatments, along with the surgical technique of cleft lip and palate repair, may impact midface growth. The objective of this study was to describe the authors' protocol for unilateral and bilateral cleft lip repair and to evaluate midfacial growth in a cohort of patients at mixed dentition who had undergone presurgical passive orthodontic appliance therapy and gingivosupraperiosteoplasty at the time of unilateral and bilateral cleft lip repair. Fifteen complete unilateral and 15 complete bilateral cleft lip and palate patients underwent passive orthodontic appliance treatment and primary lip repair with gingivosupraperiosteoplasty. Lateral cephalograms were analyzed by three blinded reviewers. Mean cephalometric measurements at mixed dentition were compared to cephalometric values for noncleft patients, unilateral cleft lip and palate patients who did not undergo gingivoperiosteoplasty or presurgical treatment, and unilateral cleft lip and palate patients who underwent gingivoperiosteoplasty/nasoalveolar molding with independent samples t tests. Mean cephalometric values were within age-specific normal values for sella-nasion-A point, sella-nasion-B point, A point-nasion-B point, and facial axis. Eighty-seven (13/15) percent of unilateral cleft lip and palate patients and 93 percent (14/15) of bilateral cleft lip and palate patients did not exhibit skeletal class III malocclusion. There was no significant difference between cephalometric values for our patients and patients who did not receive gingivosupraperiosteoplasty or presurgical treatment or who underwent the gingivoperiosteoplasty/nasoalveolar molding protocol. Presurgical passive orthodontic appliances, combined with gingivosupraperiosteoplasty at the time of lip repair, leads to normal maxillary development in most patients at mixed dentition. Assessment of midface growth at skeletal maturity is required. Therapeutic, IV.

Discussion: Gingivosupraperiosteoplasty following Presurgical Maxillary Orthopedics Is Associated with Normal Midface Growth in Complete Unilateral and Bilateral Cleft Patients at Mixed Dentition.

Discussion: Gingivosupraperiosteoplasty following Presurgical Maxillary Orthopedics Is Associated with Normal Midface Growth in Complete Unilateral and Bilateral Cleft Patients at Mixed Dentition.

Midface Growth Potential in Unoperated Clefts: A Systematic Review and Meta-Analysis.

Competing hypotheses for the development of midface hypoplasia in patients with cleft lip and palate include both theories of an intrinsic restricted growth potential of the midface and extrinsic surgical disruption of maxillary growth centers and scar growth restriction secondary to palatoplasty. The following meta-analysis aims to better understand the intrinsic growth potential of the midface in a patient with cleft lip and palate unaffected by surgical correction. A systematic review of studies reporting cephalometric measurements in patients with unoperated and operated unilateral cleft lip and palate (UCLP), bilateral cleft lip and palate (BCLP), and isolated cleft palate (iCP) abstracted SNA and ANB angles, age at cephalometric analysis, syndromic diagnosis, and patient demographics. Age and Region-matched controls without cleft palate were used for comparison. SNA angle for unoperated UCLP (84.5 ± 4.0°), BCLP (85.3 ± 2.8°), and ICP (79.2 ± 4.2°) were statistically different than controls (82.4 ± 3.5°), (all P ≤ 0.001). SNA angles for operated UCLP (76.2 ± 4.2°), BCLP (79.8 ± 3.6°), and ICP (79.0 ± 4.3°) groups were statistically smaller than controls (all P ≤ 0.001). SNA angle in unoperated ICP (n = 143) was equivalent to operated ICP patients (79.2 ± 4.2° versus 79.0 ± 4.3° P = 0.78). No unoperated group mean SNA met criteria for midface hypoplasia (SNA < 80). Unoperated UCLP/BLCP exhibit a more robust growth potential of the maxilla, whereas operated patients demonstrate stunted growth compared to normal phenotype. Unoperated ICP demonstrates restricted growth in both operated and unoperated patients. As such, patients with UCLP/BCLP differ from patients with ICP and the factors affecting midface growth may differ.Level of Evidence: IV.