The Asymmetry Within: A Renewed Look at Cupids Bow in Unilateral Cleft Lip.

The purpose of this study is to analyze the angular variations within Cupid's bow in patients with unoperated unilateral cleft lip (UCL). Angular features of Cupid's bow were quantified in standardized presurgical photographs of children with UCL by 5 medical professionals specializing in craniofacial anomalies. The peaks and valley of Cupid's bow were identified. A cleft side (CSA) and a noncleft side angle (NCSA) were delineated and measured by each expert. The data was pooled, and the angles were analyzed for symmetry. Cupid's bow asymmetry was defined as a difference between NCSA and CSA ≥3°. Of the 37 patients studied, 29 were found to have asymmetry of Cupid's bow with an average angle difference of 8.0° (95% CI: 6.6°-9.5°). Within this group,15 patients were found with acute asymmetry and 14 with obtuse asymmetry. Geometric analysis was performed on an example of a patient with acute asymmetry to demonstrate how correction of asymmetry can be considered during surgical repair. There is an asymmetry that exists in the Cupid's bow of a significant number of patients with unoperated UCL. This finding not only adds to our understanding of UCL but may also have important implications when selecting the method/technique of surgical repair.

Testing the physiological capacity of the mussel Mytilus chilensis to establish into the Southern Ocean.

The Southern Ocean and the Antarctic Circumpolar Current create environmental conditions that serve as an efficient barrier to prevent the colonization of non-native species (NNS) in the marine ecosystems of Antarctica. However, warming of the Southern Ocean and the increasing number of transport opportunities are reducing the physiological and physical barriers, increasing the chances of NNS arriving. The aim of this study was to determine the limits of survival of the juvenile mussels, M. chilensis, under current Antarctic conditions and those projected under climate change. These assessments were used to define the mussels potential for establishment in the Antarctic region. Experimental mussels were exposed to four treatments: -1.5 °C (Antarctic winter), 2 °C (Antarctic summer), 4 °C (Antarctic projected) and 8 °C (control) for 80 days and a combination of physiological and transcriptomics approaches were used to investigate mussel response. The molecular responses of mussels were congruent with the physiological results, revealing tolerance to Antarctic winter temperatures. However, a higher number of regulated differentially expressed gene (DEGs) were reported in mussels exposed to Antarctic winter temperatures (-1.5 °C). This tolerance was associated with the activation of the biological processes associated with apoptosis (up regulated) and both cell division and cilium assembly (down regulated). The reduced feeding rate and the negative scope for growth, for a large part of the exposure period at -1.5 °C, suggests that Antarctic winter temperatures represents an environmental barrier to M. chilensis from the Magellanic region settling in the Antarctic. Although M. chilensis are not robust to current Antarctica thermal conditions, future warming scenarios are likely to weaken these physiological barriers. These results strongly suggest that the West Antarctic Peninsula could become part of Mytilus distributional range, especially with dispersal aided by increasing maritime transport activity across the Southern Ocean.

The Role of Digital Surgical Planning and Surgical Guides in the Treatment of Unilateral Condylar Hyperplasia.

Unilateral condylar hyperplasia (UCH) results in facial asymmetry, malocclusion, and temporomandibular joint dysfunction. Treatment consists of both surgical and orthodontic intervention. A review was performed for 4 patients with UCH who underwent digital surgical planning (DSP)-assisted condylectomy. All patients were female, aged 14 to 35 years at the time of operation with facial asymmetry and class III malocclusion. None of the patients had prior treatment and all had perioperative orthodontic appliances to provide fixation and postoperative elastic therapy. All patients underwent DSP-guided condylectomy, and intraoperative surgical cutting guides were used for 3 of the patients. All had significant improvement in facial symmetry and occlusion. None had recurrence, and additional intervention has not been required. If UCH is recognized before marked secondary changes in the maxilla, mandible, and occlusion, future orthognathic surgery may be potentially obviated. Craniomaxillofacial surgeons should consider using DSP and surgical guides in the treatment of UCH.

Crouzon Syndrome Spanning Three Generations: Advances in the Treatment of Syndromic Midface Deficiency.

Background: Crouzon syndrome is an autosomal dominant genetic disorder characterized by craniosynostosis, midface retrusion, and exophthalmos. Over the past century, the treatment of craniofacial disorders like Crouzon syndrome has evolved significantly. Methods: An institutional review board-approved retrospective study was conducted to ascertain the treatment of three individuals with Crouzon syndrome from one family, complemented with a series of literature searches to examine the evolution of craniofacial surgical history. Results: Dr. David Williams Cheever developed the Le Fort I level to correct malocclusion, maxillomandibular malformations, and midface hypoplasia. Later, Dr. Paul Tessier introduced the Le Fort II and III osteotomies to treat syndromic midface hypoplasia. In 1978, Dr. Fernando Ortiz-Monasterio and Dr. Antonio Fuente del Campo published the first series of monobloc osteotomies, allowing for simultaneous correction of supraorbital and midface malformations, although complicated by blood loss and high infection rates. In 1992, McCarthy et al introduced the concept of gradual distraction to the craniofacial skeleton. In 1995, Polley et al performed the first monobloc advancement using external distraction. Subsequently, in 1997, Polley and Figueroa introduced a rigid external distraction device with multiple vector control to manage severe cleft maxillary hypoplasia. The technique was further refined and applied to treat syndromic midface hypoplasia, reducing complication rates. Currently, either external or internal distraction approaches are used to safely treat this challenging group of patients. Conclusion: The treatment of syndromic midface deficiency has significantly evolved over the past 50 years, as evidenced by this report of three generations of Crouzon syndrome.

TMJ Ankylosis Following Mandibular Distraction Osteogenesis: Management With Simultaneous Midface External Distraction and Bilateral Temporomandibular Joint Replacement.

Early mandibular distraction osteogenesis (MDO) can decrease upper airway and feeding complications in pediatric patients with micrognathia; however, temporomandibular joint (TMJ) complications like TMJ ankylosis (TMJA) may occur. TMJA can disturb pediatric patients' function and craniofacial growth, resulting in significant physical and psychosocial consequences. Additional surgical procedures may also be required, increasing the burden of care on patients and their families. CMF surgeons must discuss the potential complications of early MDO surgery with families as well as potential solutions should these problems occur. This report presents the case of a 17-year-old male with a severe craniofacial anomaly with features of Treacher-Collins syndrome (TCS) and a surgical history of tracheostomy, cleft palate repair, mandibular reconstruction with harvested costochondral grafts, and MDO with resultant bilateral TMJA and limited mouth opening. The patient Was treated with bilateral custom alloplastic TMJ replacements and simultaneous maxillary DO using a Rigid External Distraction (RED) device.

Acclimation of the Antarctic sea urchin Sterechinus neumayeri to warmer temperatures involves a modulation of cellular machinery.

Global warming is threatening marine Antarctic fauna, which has evolved in isolation in a cold environment for millions of years. Facing increasing temperatures, marine Antarctic invertebrates can either tolerate or develop adaptations to these changes. On a short timescale, their survival and resistance to warming will be driven by the efficiency of their phenotypic plasticity through their capacity for acclimation. The current study aims at evaluating the capacity for acclimation of the Antarctic sea urchin Sterechinus neumayeri to predicted ocean warming scenarios (+2, RCP 2.6 and + 4 °C, RCP 8.5, IPCC et al., 2019) and deciphering the subcellular mechanisms underlying their acclimation. A combination of transcriptomics, physiological (e.g. growth rate, gonad growth, ingestion rate and oxygen consumption), and behavioral-based approaches were used on individuals incubated at 1, 3 and, 5 °C for 22 weeks. Mortality was low at warmer temperatures (20%) and oxygen consumption and ingestion rate seemed to reach a stable state around 16 weeks suggesting that S. neumayeri might be able to acclimate to warmer temperatures (until 5 °C). Transcriptomic analyses highlighted adjustments of the cellular machinery with the activation of replication, recombination, and repair processes as well as cell cycle and division and repression of transcriptional and signal transduction mechanisms and defense processes. These results suggest that acclimation to warmer scenarios might require more than 22 weeks for the Antarctic Sea urchins S. neumayeri but that projections of climate change for the end of the century may not strongly affect the population of S. neumayeri of this part of the Antarctic.

Long-Term Outcomes After External Maxillary Distraction Surgery in Patients With Down Syndrome.

BACKGROUND: Patients with Down syndrome have severe facial deformities that can precipitate functional consequences and social stigmatization. Craniofacial surgical intervention can play a role in improving these symptoms and patient quality of life. The objective of this study was to investigate the long-term outcomes of distraction osteogenesis and orthognathic surgical intervention in patients with Down syndrome. MATERIALS AND METHODS: Charts of 3 patients with Down syndrome who were treated with external maxillary distraction osteogenesis were retrospectively reviewed. The patients' caregivers were prospectively interviewed between 10 and 15 years after surgery to determine surgical stability, long-term function, and quality of life status. RESULTS: All patients and their caregivers reported excellent results with improvements in function and quality of life. Facial skeletal changes have been stable over time. The cephalometric analysis demonstrated significant maxillary advancement in all 3 patients and mandibular changes to correct mandibular prognathism and asymmetry in the patient who underwent finishing orthognathic surgery. CONCLUSIONS: External maxillary distraction osteogenesis and orthognathic surgery may be considered in select patients with Down syndrome as part of their multidisciplinary health care. These interventions can result in long-term improvements in patient function and quality of life.

Spontaneous Closure of Congenital Cranial Defect: Is Early Surgical Intervention Warranted?

Infantile cranial development typically occurs in a predictable sequence of events; however, less is known about how the development occurs in isolated, nonsyndromic congenital craniofacial anomalies. Furthermore, the timing of pediatric cranioplasty has been extrapolated from adult studies. Thus, the management of nonsyndromic congenital craniofacial anomalies presents with unique challenges to the craniofacial surgeon. The authors describe the case of a baby girl who was born with right Tessier 3 cleft, cleft palate, anophthalmos, and severe left craniofacial microsomia with Pruzansky grade III left mandibular anomaly. By analyzing 3-dimensional chronological models of the patient, the authors found that her abnormal fontanelle initially increased in size until 22 weeks of age, with subsequent spontaneous closure at a rate of 60.53 mm2/y. Although similar cranial anomalies are typically surgically corrected early in life, delaying treatment until after 2 years of age may be appropriate in some patients, obviating surgical morbidity in the newborn period.

Differential gene expression analysis in the scallop Argopecten purpuratus exposed to altered pH and temperature conditions in an upwelling-influenced farming area.

Increased carbon dioxide in the atmosphere and its absorption across the ocean surface will alter natural variations in pH and temperature levels, occurring in coastal upwelling ecosystems. The scallop Argopecten purpuratus, one of the most economically important species farmed in northern Chile, has been shown to be vulnerable to these environmental drivers. However, the regulatory responses at the gene-level of scallops to these climate stressors remain almost unknown. Consequently, we used an orthogonal experimental design and RNAseq approach to analyze the acute effects of variability in pH and temperature on gene expression in the muscle tissue of A. purpuratus. In respect to control conditions (pH ~ 8.0/ 14 °C), the influence of low pH (~ 7.7) and temperature (14 °C) induced the activation of several genes associated with apoptotic signaling pathways and protein localization to plasma membrane. Elevated temperature (18 °C) and pH (~8.0) conditions increased the expression of transcripts associated with the activation of muscle contraction, regulation, and sarcomere organization effects on muscle tissue. In scallops exposed to low pH and elevated temperature, the genes expressed were differentially associated with the oxidation-reduction process, signal translation, and positive regulation of GTPase activity. These results indicated that the differentially expressed genes under the experimental conditions tested are mainly related to the mitigation of cellular damage and homeostasis control. Our results add knowledge about the function of the adductor muscle in response to stressors in scallops. Furthermore, these results could help in the identification of molecular biomarkers of stress necessary to be integrated into the aquaculture programs for the mitigation of climate change.

Developing a core outcome set for anthropometric evaluation for presurgical infant orthopaedics for unilateral cleft lip and palate: e-Delphi consensus.

INTRODUCTION: Presurgical infant orthopaedics (PSIO) in infants with cleft lip and palate focuses on improving the anatomical conditions of the lip, palate and nose before the first lip surgery; however, its effectiveness has not been proven. OBJECTIVE: To develop a core outcome set for reporting anthropometry-based outcomes in studies appraising the PSIO before primary cleft lip repair in unilateral cleft lip palate (UCLP). METHOD: Literature search to identify anthropometric measures. The operational definition and schematic representation of each were elaborated, grouping those apparently the same. By using Delphi methodology with a consensus of 10 subject-matter experts, three rounds were conducted to select a core outcome set of anthropometric measures with a validity V coefficient ≥80% among considered necessary to evaluate the PSIO in UCLP. RESULTS: A total of 101 anthropometric measures were identified in the literature to evaluate PSIO in UCLP. Of these, consensus validated the content of the core outcome set, which comprises 18 anthropometric measures, including columella height, nasal tip projection, projection alar length, width of nostril, nasal basal width, angle of columella, cleft lip segment, height of the non-cleft lip, height of the cleft lip, intersegment distance, arch length, greater segment length, lesser segment length, lateral deviation of the incisal point, posterior width of palatal cleft, arch width, grater segment rotation and lesser segment rotation. CONCLUSIONS: Standardised outcome measures are necessary to evaluate and ensure the quality of treatment in CLP. The core outcome set for anthropometric evaluation validated by consensus subject-matter experts is a clinically useful and low-cost tool for PSIO effectiveness studies.

Monobloc Differential Distraction Osteogenesis.

ABSTRACT: Midface advancement at the monobloc level can be the seminal life event for patients with craniofacial dysostosis. Monobloc reconstruction, when planned appropriately, can simultaneously and definitively address multiple functional and aesthetic deficiencies in these patients. The application of distraction has reduced the morbidity experienced with traditional monobloc surgery. The purpose of this study is to report on the outcomes, stability, and growth in younger patients after monobloc advancement in syndromic craniosynostosis patients. The authors report a consecutive series of thirty patients with craniofacial dysostosis treated through monobloc differential distraction osteogenesis. Detailed history, photographic, and long-term radiographic data are reviewed, including a subset of patients who were skeletally immature at the time of their treatment. Differential distraction allows control of midface pitch, roll, and yaw, optimizing functional and aesthetic outcomes. There were no infectious complications requiring reoperation. The average surgical age for all patients was 12.5 years. For the 7 patients age <7 years, average age was 6 years. For all patients, the mean horizontal movement was 12 mm at nasion and 10 mm at A-point. At mean follow-up (4.8 years entire group and 6.2 years age <7 years group) a positive horizontal advancement of 1.1 mm at nasion and 0.8 mm at A-point was observed. More pronounced positive horizontal changes were seen in the age <7 years group. Monobloc differential distraction osteogenesis affords safe and precise repositioning of the midface. The advancement is skeletally stable and young patients show moderate continued growth.

Using Active-Phonation Cone-Beam Computed Tomography to Evaluate Pediatric Patients With Suspected Velopharyngeal Dysfunction, a Pilot Study.

ABSTRACT: Velopharyngeal dysfunction (VPD) is described as the incomplete closure of the velopharyngeal port during a speech production. Nasopharyngoscopy and/or multiplanar videofluoroscopy have been utilized for decades to assess the degree and nature of the dysfunction. Cone-beam computed tomography (CBCT) is presented as an additional diagnostic tool, allowing for clear visualization of the affected structures and the ability to obtain accurate measurements (within 100 microns) of the involved anatomy and defect. This prospective pilot study aims to test the feasibility of using "active-phonation" CBCT to assess suspected VPD in the pediatric and young adult populations and compare the results to nasopharyngoscopy; the current standards of care.Six patients, ages 6 to 26 years, with suspected VPD, defined as the inability to completely close off the nasal airway during an oral speech, seen at an urban medical outpatient craniofacial care center, served as subjects for this pilot study. Each patient received a comprehensive speech evaluation and participated in both active-phonation CBCT and nasopharyngoscopy.Both active-phonation CBCT and nasopharyngoscopy revealed incomplete closure of the velopharyngeal port during a speech in all 6 patients (100%). Two patients (33%) were unable to tolerate a complete nasendoscopic examination. There was no difference between CBCT or nasopharyngoscopy in determining the presence of VPD and noting the severity on a 3-point scale, (P = 0.61) as judged by 4 experienced clinicians.As a functional imaging modality, active-phonation CBCT is a useful adjunct tool for accurate diagnosis of VPD and may be more easily tolerated during a thorough VPD assessment than nasopharyngoscopy. It also provides quantitative data that is useful to augment treatment optimization and surgical planning in this population. Further studies are needed to validate these results.

Alloplastic Temporomandibular Joint Reconstruction Following Recurrent Ameloblastoma Resection.

ABSTRACT: Ameloblastomas are benign tumors that most commonly affecting the mandible. The current standard of treatment for ameloblastomas is resection followed by reconstruction that has historically been accomplished through the use of a microsurgical vascularized flaps taken from the iliac crest or fibula. Alloplastic reconstruction methods have gained popularity over recent years with success reported in the reconstruction of many pathologies, including ankylosis, condylar fracture, neoplasia involving extensive resection, severe inflammatory/degenerative temporomandibular joint (TMJ) disease, and congenital TMJ abnormalities. The authors present a patient who successfully underwent ameloblastoma resection and TMJ reconstruction with a custom TMJ Concepts alloplastic implant. The authors also present a review of the literature on alloplastic TMJ reconstruction following ameloblastoma resection. To our knowledge, this is the second report in the literature on the use of a TMJ Concepts implant after ameloblastoma resection.

Intra-Lesional Injection of Triamcinolone to Palatoplasty Scar to Aid Reversal of Transverse Maxillary Relapse after Orthognathic Surgery.

ABSTRACT: A 32-year-old female with a repaired right unilateral cleft lip and palate underwent several surgical and orthodontic procedures during the rehabilitation process of her condition. Nine years after this extensive treatment she underwent transverse relapse of her maxilla and requested a consultation for its correction as she felt her speech and chewing were negatively affected. She presented with a transverse maxillary arch collapse on the cleft side with significant palatal scarring secondary to multiple palate procedures. A course of maxillary expansion and dental alignment with fixed orthodontic appliances was carried out. in addition, she had 2 triamcinolone injections 7 months apart while undergoing orthodontic treatment and one 10 months after completion to soften the scarred palatal tissues. The maxillary arch was successfully expanded and aligned. She was retained with a removable chrome cobalt palatal frame to be used full-time and assure stability of the correction. She has been followed for 4 years with no clinical evidence of relapse. Triamcinolone injection into significant palatal scarring in cleft palate patients with a decreased transverse maxillary dimension can be considered an adjunct procedure in conjunction with orthodontic treatment.

RNA-Seq reveals divergent gene expression between larvae with contrasting trophic modes in the poecilogonous polychaete Boccardia wellingtonensis.

The polychaete Boccardia wellingtonensis is a poecilogonous species that produces different larval types. Females may lay Type I capsules, in which only planktotrophic larvae are present, or Type III capsules that contain planktotrophic and adelphophagic larvae as well as nurse eggs. While planktotrophic larvae do not feed during encapsulation, adelphophagic larvae develop by feeding on nurse eggs and on other larvae inside the capsules and hatch at the juvenile stage. Previous works have not found differences in the morphology between the two larval types; thus, the factors explaining contrasting feeding abilities in larvae of this species are still unknown. In this paper, we use a transcriptomic approach to study the cellular and genetic mechanisms underlying the different larval trophic modes of B. wellingtonensis. By using approximately 624 million high-quality reads, we assemble the de novo transcriptome with 133,314 contigs, coding 32,390 putative proteins. We identify 5221 genes that are up-regulated in larval stages compared to their expression in adult individuals. The genetic expression profile differed between larval trophic modes, with genes involved in lipid metabolism and chaetogenesis over expressed in planktotrophic larvae. In contrast, up-regulated genes in adelphophagic larvae were associated with DNA replication and mRNA synthesis.

Cleft Lip and Palate: LeFort I Distraction with Halo and Hybrid Internal Maxillary Distractors.

Distraction osteogenesis is a viable treatment option for patients with a cleft associated with severe maxillary retrusion. A rigid external distraction device and a hybrid internal maxillary distractor have been used to advance the maxilla allowing for predictable and stable results. These techniques can be applied by itself or as an adjunct to traditional orthognathic procedures. The technical aspects are presented. These procedures tend to be simpler and demonstrate great stability compared to traditional surgical methods. The reasons for stability are discussed.

Skeletal Stability and Airway Changes After Maxillary Advancement Using a Rigid External Distraction System in Non-Growing Cleft Patients.

ABSTRACT: Distraction osteogenesis (DO) is a highly effective technique for correction of severe maxillary hypoplasia, especially in patients with orofacial clefts and craniofacial syndromes. The purpose of this retrospective, longitudinal study was to assess long-term airway alterations after maxillary advancement using a rigid external distraction system (RED) in non growing cleft patients. Fifteen cleft patients (8 males and 7 females) aged from 14 to 25 years were included in this study. All of them were treated with a rigid external distraction system for maxillary advancement after a high Le Fort I osteotomy. To analyse airway changes lateral cephalograms were obteined before distraction (T0), immediately after distraction (T1) and 1 to 3 years and 3 months after distraction (T2). All the measurements were describled by means of median, minimum and maximum. In order to evaluate differences between each time interval, a Wilcoxon test associated to a Delta Cliff test was used to evaluate the effect size (level of significance adopted was 5%). A significant maxillary advancement and increased upper airway antero-posterior dimensions were observed after the distraction osteogeness process, as demonstrated by the difference between T1 and T0. No significant relapse at T2 was found. Lower airway and the airway at tip of uvula region did not display significant alterations. A significant maxillary advancement and increased antero-posterior upper airway dimension was measured immediately after maxillary distraction with rigid external distraction in non growing cleft patients. The findings were stable three years after distraction.