Correction of metopic synostosis utilizing an in situ bandeau approach.
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ABSTRACT: Metopic craniosynostosis is a common growth disturbance in the infant cranium, second only to sagittal synostosis. Presenting symptoms are usually of a clinical nature and are defined by an angular forehead, retruded lateral brow, bitemporal narrowing, and a broad-based occiput. These changes create the pathognomonic trigonocephalic cranial shape. Aesthetic in nature, these morphological changes do not constitute the only developmental issues faced by children who present with this malady. Recent studies and anecdotal evidence have also demonstrated that children who present with metopic synostosis may face issues with respect to intellectual and/or psychological development. The authors present an elegant approach to the surgical reconstruction of the trigonocephalic cranium using an in situ bandeau approach.
Project description:ObjectiveSurgical correction of unicoronal synostosis (UCS) entails extensive cranioplasties which do not address facial scoliosis. This paper presents the first results with springs that motivated the shift from extensive cranioplasties to dynamic techniques for surgical correction of UCS.MethodsTwo cases of UCS were operated with a linear osteotomy combined with springs. The deviation in facial symmetry (orbital dystopia angle) and skull base angles were measured on pre and postoperative computed tomography scans until 3 years of age.ResultsThe facial scoliosis was corrected. At spring removal, the orbital dystopia angle had gone from a 9.2 to 13.2-degree deviation preoperatively to a 0.5 to 0.9-degree overcorrection compared with the ideal 0-degree deviation. Also, the skull base deviation improved.ConclusionLinear osteotomy combined with springs corrects the facial scoliosis in UCS. These cases indicate that dynamic methods may be beneficial for improving the results of surgical correction of UCS.
Project description:Nasal asymmetry is widely acknowledged to be one of the most difficult deformities to manage. Most reports in the literature pertain to corrective methods in relation to isolated deformity of the dorsum in the posttraumatic patient. There is a paucity of literature relating to management of nasal radix asymmetry, and still less in the context of severe panfacial asymmetry.
Project description:Sagittal synostosis is the most commonly treated form of craniosynostosis exhibiting an incidence of 1:5000 births.(1) Early closure of the sagittal suture is the cause of the classic phenotypic presentation of an elongated head, which is characterized by a narrow and low occiput, a saddle-shaped parietal region, and noticeable frontal bossing. Early surgical intervention is the treatment of choice and surgical options range from simple suture excision to total cranial vault remodeling. Noting that a significant proportion of patients present with limited frontal bossing and asymmetry that is more pronounced in the posterior cranium, the author developed the functional aesthetic approach to reconstruction. This operative technique addresses the changes noted in the posterior two-thirds of the cranial vault, completely correcting the sequelae associated with early closure of the sagittal suture. As an added advantage, the reconstructive paradigm is designed to create a dynamic temporal-occipital component that will activate when a child rests in the supine position. This enhances widening of the posterior cranium thus improving long-term results.
Project description:The clinical management of prostate cancer is challenging and currently relies primarily on staging, histological grading, and tumor size. In this study, we take advantage of the propensity of prostate cancer to be multifocal and categorize aggressiveness of individual prostate cancer foci based on DNA methylation patterns in primary and metastatic tumors.
Project description:Despite their remarkable charge carrier mobility when forming well-ordered fibers, supramolecular transistors often suffer from poor processability that hinders device integration, resulting in disappointing transconductance and output currents. Here, a new class of supramolecular transistors, π-ion gel transistors (PIGTs), is presented. An in situ π-ion gel, which is an unprecedented composite of semiconducting nanofibers and an enclosed ionic liquid, is directly employed as an active material and internal capacitor. In comparison to other supramolecular transistors, a PIGT displays a high transconductance (133 µS) and output current (139 µA at -6 V), while retaining a high charge-carrier mobility (4.2 × 10-2 cm2 V-1 s-1 ) and on/off ratio (3.7 × 104 ). Importantly, the unique device configuration and the high ionic conductivity associated with the distinct nanosegregation enables the fastest response among accumulation-mode electrochemical-based transistors (<20 µs). Considering the advantages of the absence of dielectric layers and the facile fabrication process, PIGT has great potential to be utilized in printed flexible devices. The device platform is widely applicable to various supramolecular assemblies, shedding light on the interdisciplinary research of supramolecular chemistry and organic electronics.
Project description:We present the successful surgical treatment and management of metacarpal synostosis in a near-skeletally mature 15-year-old patient, the significance of which is underscored by an updated review of the literature. We additionally outline a reliable surgical approach for patients with similar clinical presentations and disease severity.
Project description:We propose a landmark-based, virtual coordinate system, specifically designed for assessment of asymmetrical craniofacial anatomy associated with unicoronal synostosis.MethodCT scans of 33 patients with nonsyndromic unicoronal synostosis were included. Proposed mid-sagittal plane was compared with commonly used sagittal planes: (1) nasion, sella, and basion (N-S-BA); (2) midplane of bilateral frontozygomatic sutures (midFZ); and (3) the skull gravity center plane, to evaluate reliability and validity in the assessments of the anterior and posterior skull base.ResultsThe proposed midplane is similar to the midFZ plane in describing the direction of the anterior skull base. However, it has less bias than the N-S-BA (P < 0.001), and the gravity center planes (P < 0.001). The proposed midplane measures the direction of the posterior skull base plane, similar to the midFZ and gravity center planes, but it has less measurement deviation than the N-S-BA plane (P < 0.001). The most protrusive point on the frontal bone in unicoronal patients is contralateral to the fused suture and distant from the mid-sagittal plane by 13.93 ± 4.01 mm. In addition, it is more anteriorly positioned, by 5.32 mm (P < 0.001), when compared with the corresponding point on the synostotic side. The uppermost point of the supraorbital rim on the synostotic side is cephalic to that of the contralateral side by 4.09 mm (P < 0.001).ConclusionsPrioritized orientation of an averaged Frankfort horizontal plane, followed by the location of the mid-sagittal and coronal planes, can generate a reliable and valid coordinate framework for the assessment of asymmetric skull shape in unicoronal synostosis.
Project description:The TP53 c.359A>G mutation severely impairs expression of the major TP53 transcript variant encoding p53 K120R by creating a new splicing donor site. Antisense morpholino oligomer (AMO) targeting the mutation site restored normal splicing and expression of the major variant. Despite the recovery of mutant TP53 mRNA levels, the functional restoration as the tumor suppressor of p53 K120R was unclear. Given that p53 exerts its tumor suppressor function through regulation of target genes that mediate growth arrest or apoptosis, the p53 K120R mutant exhibits impaired transcriptional regulation of CDKN1A, a key target gene in growth arrest, while maintaining normal induction of the pro-apoptotic BBC3 gene. Consequently, the p53 K120R mutant protein demonstrates a phenotype characterized by defective cell growth arrest but retained apoptotic induction capacity, suggesting that the p53 K120R mutant protein may retain some tumor suppressor functionality. In addition, Lysine 120 provides a critical acetylation site linked to p53 activation. Although other studies of the oncogenic effects of p53 K120R highlight the relevance of acetylation for tumor suppression, our findings indicate that targeting mutant TP53 mRNA is a prerequisite for restoration of p53 function. In particular, despite the presence of mutations, it showed sufficient corrective effect to restore p53 functions, including apoptosis induction. In conclusion, this study highlights the potential of AMO-mediated splice correction as a therapeutic approach for TP53 mutations.
Project description:BackgroundMetopic synostosis patients have a high prevalence of orthoptic anomalies, including hyperopia, astigmatism, and amblyopia. We hypothesized altered orbital anatomy contributes to suboptimal visual outcomes by adversely affecting eye anatomy and growth from early life onward. Therefore, we aimed to investigate eye and orbital anatomy in metopic synostosis.MethodsWe conducted a retrospective study in nonsyndromic metopic synostosis patients (n = 134, median age 0.43 years [IQR 0.45]) with nonsyndromic sagittal synostosis patients (n = 134, median age 0.27 years [IQR 0.23]) as controls. Primary analyses focused on eye dimensions (axial length, width, and globe height) and orbital dimensions, correcting for sex and age. Measurements were obtained from preoperative computed tomography scans.ResultsAxial length and width in metopic synostosis patients did not differ from sagittal synostosis patients, but globe height was significantly smaller (P = 0.0002). Lateral wall interorbital length, lateral orbital wall length, anterior medial interorbital length, and maximal medial interorbital length were significantly smaller, and anterior vertical orbital height and maximal vertical orbital height were significantly larger (P < 0.001). The central orbital axis and interorbital angle were significantly narrower, and medial-to-lateral orbital wall angle was wider (P < 0.001).ConclusionsMetopic synostosis patients have more shallow, wider, and higher orbits. Eye dimensions are similar in sagittal synostosis patients, although globe height was smaller. Altered orbital and eye dimensions in metopic synostosis probably have a causal relation with an unknown order of development. How these dimensions relate to future orthoptic anomalies (eg, refractive error) needs further investigation.
Project description:To provide appropriate treatment, neurosurgeons and pediatricians must understand the chronological changes of the cranial morphology in normal children and the differences between normal and sagittal synostosis children. However, this issue has remained unresolved due to the traditional methods of analyzing cranial morphology. Therefore, we applied a new and precise method, geometric morphometrics, to understand cranial morphology in children with normal and sagittal synostosis. This study analyzed morphological differences in the cranium of 30 control children and 14 children with sagittal synostosis using geometric morphometrics and the landmark method on the lateral view of the three-dimensional computed tomography of the skull. The results showed that morphological changes in the cranium of normal children with age could be visualized (p < 0.01), with the parietooccipital and suboccipital areas being the main areas of change. Morphological analysis revealed different cranial morphologies among the control, scaphocephalic sagittal synostosis, and non-scaphocephalic sagittal synostosis groups (p < 0.01), as evidenced by a fan-like spread and reduced cranial height in the sagittal synostosis group compared to the control cranium. Visualization in wireframes could reveal morphological differences, even if small, with statistical differences. This study clarified 3 issues regarding the cranial morphology in normal and sagittal children. The study showed evident age-related differences and changes in the cranial morphology in normal children. Differences in cranial morphologies among the 3 groups were identified. Finally, the study demonstrates that geometric morphometrics is a precise and appropriate method for analyzing morphological differences and changes.