Local Soft Tissue and Bone Displacements Following Midfacial Bipartition Distraction in Apert Syndrome - Quantification Using a Semi-Automated Method.

ABSTRACT: Patients with Apert syndrome experience midfacial hypoplasia, hypertelorism, and downslanting palpebral fissures which can be corrected by midfacial bipartition distraction with rigid external distraction device. Quantitative studies typically focus on quantifying rigid advancement and rotation postdistraction, but intrinsic shape changes of bone and soft tissue remain unknown. This study presents a method to quantify these changes. Pre- and post-operative computed tomography scans from patients with Apert syndrome undergoing midfacial bipartition distraction with rigid external distraction device were collected. Digital Imaging and Communications in Medicine files were converted to three-dimensional bone and soft tissue reconstructions. Postoperative reconstructions were aligned on the preoperative maxilla, followed by nonrigid iterative closest point transformation to determine local shape changes. Anatomical point-to-point displacements were calculated and visualized using a heatmap and arrow map. Nine patients were included.Zygomatic arches and frontal bone demonstrated the largest changes. Mid-lateral to supra-orbital rim showed an upward, inward motion. Mean bone displacements ranged from 3.3 to 12.8 mm. Soft tissue displacements were relatively smaller, with greatest changes at the lateral canthi. Midfacial bipartition distraction with rigid external distraction device results in upward, inward rotation of the orbits, upward rotation of the zygomatic arch, and relative posterior motion of the frontal bone. Local movements were successfully quantified using a novel method, which can be applied to other surgical techniques/syndromes.

Frontofacial Surgery: Reducing Infection with the Development and 6-Year Outcome of a Frontofacial Protocol.

BACKGROUND: Frontofacial surgery (FFS) creates a communication between the cranial and nasal cavities and is associated with significant infection risk. After a cluster of infections affecting patients undergoing FFS, a root cause analysis of index cases was undertaken, but no specifically remedial causes were identified. Basic principles incorporating known risk factors for the prevention of surgical-site infection were then applied to the creation of a perioperative management protocol. This study analyzes infection rates before and after its implementation. METHODS: The protocol was designed around the needs of patients undergoing FFS and consists of three checklists covering their preoperative, intraoperative, and postoperative care. Compliance required the completion of each checklist. All patients undergoing FFS between 1999 and 2019 were studied retrospectively, and infections occurring before and after the implementation of the protocol were analyzed. RESULTS: One hundred three patients underwent FFS (60 monobloc and 36 facial bipartition) before the implementation of the protocol in August of 2013, and 30 patients underwent FFS after its implementation. Compliance with the protocol was 95%. After implementation, there was a statistically significant reduction in infections from 41.7% to 13.3% ( P = 0.005). CONCLUSIONS: Although no specific cause for a cluster of postoperative infection had been identified, the implementation of a bespoke protocol consisting of preoperative, perioperative, and postoperative checklists covering measures known to reduce infection risk was associated with a significant reduction in postoperative infections in patients undergoing FFS. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Two-Center Review of Posterior Vault Expansion following a Staged or Expectant Treatment of Crouzon and Apert Craniosynostosis.

BACKGROUND: The timing of posterior cranial expansion for the management of intracranial pressure can be "staged" by age and dysmorphology or "expectant" by pressure monitoring. The authors report shared outcome measures from one center performing posterior vault remodeling (PCVR) or distraction (PVDO) following a staged approach and another performing spring-assisted expansion (SAPVE) following an expectant protocol. METHODS: Apert or Crouzon syndrome patients who underwent posterior expansion younger than 2 years were included. Perioperative outcomes and subsequent cranial operations were recorded up to last follow-up and intracranial volume changes measured and adjusted using growth curves. RESULTS: Thirty-eight patients were included. Following the expectant protocol, Apert patients underwent SAPVE at a younger age (8 months) than Crouzon patients (16 months). The initial surgery time was shorter but total operative time, including device removal, was longer for PVDO (3 hours 52 minutes) and SAPVE (4 hours 34 minutes) than for PCVR (3 hours 24 minutes). Growth-adjusted volume increase was significant and comparable. Fourteen percent of PCVR, 33% of PVDO, and 11% of SAPVE cases had complications, but without long-term deficits. Following the staged approach, 5% underwent only PVDO, 85% had a staged posterior followed by anterior surgery, and 10% required a third expansion. Following the expectant approach, 42% of patients had only posterior expansion at last follow-up, 32% had a secondary cranial surgery, and 26% had a third cranial expansion. CONCLUSION: Two approaches involving posterior vault expansion in young syndromic patients using three techniques resulted in comparable early volume expansion and complication profiles. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Convolutional mesh autoencoders for the 3-dimensional identification of FGFR-related craniosynostosis.

Clinical diagnosis of craniofacial anomalies requires expert knowledge. Recent studies have shown that artificial intelligence (AI) based facial analysis can match the diagnostic capabilities of expert clinicians in syndrome identification. In general, these systems use 2D images and analyse texture and colour. They are powerful tools for photographic analysis but are not suitable for use with medical imaging modalities such as ultrasound, MRI or CT, and are unable to take shape information into consideration when making a diagnostic prediction. 3D morphable models (3DMMs), and their recently proposed successors, mesh autoencoders, analyse surface topography rather than texture enabling analysis from photography and all common medical imaging modalities and present an alternative to image-based analysis. We present a craniofacial analysis framework for syndrome identification using Convolutional Mesh Autoencoders (CMAs). The models were trained using 3D photographs of the general population (LSFM and LYHM), computed tomography data (CT) scans from healthy infants and patients with 3 genetically distinct craniofacial syndromes (Muenke, Crouzon, Apert). Machine diagnosis outperformed expert clinical diagnosis with an accuracy of 99.98%, sensitivity of 99.95% and specificity of 100%. The diagnostic precision of this technique supports its potential inclusion in clinical decision support systems. Its reliance on 3D topography characterisation make it suitable for AI assisted diagnosis in medical imaging as well as photographic analysis in the clinical setting.

The 3D skull 0-4 years: A validated, generative, statistical shape model.

BACKGROUND: This study aims to capture the 3D shape of the human skull in a healthy paediatric population (0-4 years old) and construct a generative statistical shape model. METHODS: The skull bones of 178 healthy children (55% male, 20.8 ± 12.9 months) were reconstructed from computed tomography (CT) images. 29 anatomical landmarks were placed on the 3D skull reconstructions. Rotation, translation and size were removed, and all skull meshes were placed in dense correspondence using a dimensionless skull mesh template and a non-rigid iterative closest point algorithm. A 3D morphable model (3DMM) was created using principal component analysis, and intrinsically and geometrically validated with anthropometric measurements. Synthetic skull instances were generated exploiting the 3DMM and validated by comparison of the anthropometric measurements with the selected input population. RESULTS: The 3DMM of the paediatric skull 0-4 years was successfully constructed. The model was reasonably compact - 90% of the model shape variance was captured within the first 10 principal components. The generalisation error, quantifying the ability of the 3DMM to represent shape instances not encountered during training, was 0.47 mm when all model components were used. The specificity value was <0.7 mm demonstrating that novel skull instances generated by the model are realistic. The 3DMM mean shape was representative of the selected population (differences <2%). Overall, good agreement was observed in the anthropometric measures extracted from the selected population, and compared to normative literature data (max difference in the intertemporal distance) and to the synthetic generated cases. CONCLUSION: This study presents a reliable statistical shape model of the paediatric skull 0-4 years that adheres to known skull morphometric measures, can accurately represent unseen skull samples not used during model construction and can generate novel realistic skull instances, thus presenting a solution to limited availability of normative data in this field.

Orbital fractures in children.

In children, differences in the properties and proportions of bone in the craniofacial skeleton and the lack of development of the paranasal sinuses result in orbital fractures that present differently from those in adults. Facial growth may be disturbed by such injuries and also by surgical intervention, which should therefore be as conservative as possible. However, urgent operation is needed to prevent irreversible changes when fractures of the orbital floor involve entrapped muscle. We present an approach to such injuries.