Atlantoaxial dislocation in the setting of NMLFS.

BACKGROUND: Nablus mask-like facial syndrome (NMFLS) is an extremely rare genetic syndrome characterized by facial dysmorphia as well as developmental delay. In the present report we describe a potential association between non-traumatic atlanto-occipital dislocation and NMFLS in an 11-year old female lacking typical facial features of NMFLS. CASE DESCRIPTION: An 11-year-old female with autism presented with symptoms of persistent headache and vomiting as well as neck stiffness. Further investigation and CT imaging revealed congenital malformation of the skull base and craniocervical junction with complete posterior subluxation of the left occipital condyle. MRI findings later corroborated the findings on CT. CONCLUSIONS: The patient was successfully treated with occipitocervical fusion. The findings in this case suggest the possibility that atlanto-occipital instability and generalized occipitocervical may be associated with NMFLS.

Pediatric Prostatic Alveolar Rhabdomyosarcoma Presenting with Metastatic Spinal Cord Compression in the Thoracic Spine: A Case Report and Review of the Literature.

Rhabdomyosarcoma (RMS) is a pediatric malignancy with a variable prognosis depending on tumor stage and genotype. There has been a significant improvement in survival rates over the past decades. However, aggressive variants can metastasize to locations that are difficult to treat. We report a case of prostatic alveolar rhabdomyosarcoma with metastases to the bone marrow and thoracic spine in a child. The patient was treated with a multimodal approach that included surgical resection of the epidural mass; the administration of vincristine, dactinomycin, and cyclophosphamide; and radiotherapy. Unfortunately, after six months, the patient showed disease progression and was started on secondary-line treatment. This case illustrates the difficulties in managing end-stage metastatic rhabdomyosarcoma and is the first report of prostatic rhabdomyosarcoma presenting with spinal cord compression in a child.

Optimization of novel exoscopic blue light filter during fluorescence-guided resection of Glioblastoma.

PURPOSE: Operative guidelines and use optimization for new surgical exoscopes are not well described in the literature. In this study, we evaluated use of the ORBEYE (Olympus) surgical exoscope system during 5-ALA fluorescence-guided resection of GBMs to optimize workflow and exoscope settings. METHODS: The ORBEYE exoscope system was fitted with a blue light filter for 5-ALA mediated fluorescence-guided surgery (FGS). Intraoperative images were obtained during 5-ALA FGS in 9 patients with primary or recurrent GBM. The exoscope was set up at constant, increasing focal distances from the target tissue, and light source intensity varied. High-resolution 4 K images were captured and analyzed. Comparisons of fluorescence to background were then generated for use optimization. RESULTS: Light intensity did not significantly influence tumor fluorescence (P = 0.878). However, focal distance significantly impacted relative fluorescent intensity (P = 0.007). Maximum average fluorescence was seen consistently at a focal length of 220 mm and a light intensity of approximately 75% maximum. Decreasing focal distance from 400 mm to 220 mm significantly increased visualized fluorescence (P = 0.0038). CONCLUSIONS: The ORBEYE surgical exoscope system with blue light filter is a powerful imaging tool for 5-ALA FGS in GBM. The ORBEYE blue filter performs optimally at shorter focal distance with moderate light intensity. Similar to microscope systems, decreasing focal distance significantly influences visualized fluorescence.

Image Segmentation of Operative Neuroanatomy Into Tissue Categories Using a Machine Learning Construct and Its Role in Neurosurgical Training.

BACKGROUND: The complexity of the relationships among the structures within the brain makes efficient mastery of neuroanatomy difficult for medical students and neurosurgical residents. Therefore, there is a need to provide real-time segmentation of neuroanatomic images taken from various perspectives to assist with training. OBJECTIVE: To develop the initial foundation of a neuroanatomic image segmentation algorithm using artificial intelligence for education. METHODS: A pyramidal scene-parsing network with a convolutional residual neural network backbone was assessed for its ability to accurately segment neuroanatomy images. A data set of 879 images derived from The Neurosurgical Atlas was used to train, validate, and test the network. Quantitative assessment of the segmentation was performed using pixel accuracy, intersection-over-union, the Dice similarity coefficient, precision, recall, and the boundary F1 score. RESULTS: The network was trained, and performance was assessed class wise. Compared with the ground truth annotations, the ensembled results for our artificial intelligence framework for the pyramidal scene-parsing network during testing generated a total pixel accuracy of 91.8%. CONCLUSION: Using the presented methods, we show that a convolutional neural network can accurately segment gross neuroanatomy images, which represents an initial foundation in artificial intelligence gross neuroanatomy that will aid future neurosurgical training. These results also suggest that our network is sufficiently robust, to an unprecedented level, for performing anatomic category recognition in a clinical setting.

Intracranial Vasospasm After Evacuation of Acute Spontaneous Subdural Hematoma.

Cerebral vasospasm is a well-known entity following aneurysmal subarachnoid hemorrhage. While it has been described in trauma, it has been much less studied. There have been no previous reports of cerebral vasospasm following spontaneous subdural hematoma or after subdural hematoma evacuation. In this case report, we present a 38-year-old otherwise healthy female who suffered an acute spontaneous subdural hematoma. After surgical evacuation of her hematoma, she developed neurologic decline. Computer tomography angiography demonstrated intracranial vasospasm. She was treated with blood pressure augmentation and nimodipine. She went on to make a full neurologic recovery.To our knowledge, this is the first reported case of cerebral vasospasm after acute spontaneous subdural hematoma or after subdural hematoma evacuation, and the patient recovered without sequelae. The promising outcome of this case may provide a framework for future similar cases. Neurosurgeons and intensivists should keep cerebral vasospasm in their differentials for patients who have neurologic decline after craniotomy for acute subdural hematoma and have an otherwise negative scan for new acute abnormality.

Cerebellopontine Angle Primary Choroid Plexus Carcinoma Present in an Adult: Case Report and Literature Review.

Choroid plexus tumors (CPTs) are rare intraventricular neoplasms that primarily occur in children and are rare in adults. Of the CPT subtypes, choroid plexus carcinomas (CPC) are highly aggressive and malignant and of World Health Organization (WHO) Grade III. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. In this case report, we present a 33-year-old female with a past medical history notable for schizophrenia and bipolar disease who suffered from left-sided acute vision loss and hearing loss. Magnetic resonance imaging (MRI) demonstrated multiple enhancing masses found in the left cerebellopontine angle (CPA), right internal auditory canal, the atrium of the left ventricle, and the left foramen of Monroe. After surgical decompression of the CPA tumor, the permanent final pathology was consistent with CPC. To our knowledge, this is the first reported case of a primary CPC occurring within the CPA in an adult. The unique presentation and progression of this rare adult-onset CPC provide insight for the diagnosis and treatment of other rare instances of CPTs.

Resection of a pineal region papillary tumor using robotic exoscope: improved visualization and ergonomics for deep seeded tumor.

Surgery for pineal region tumors is technically challenging due to their deep location and close proximity to critical deep venous structures, midbrain, and thalamus. A high-definition video exoscope was recently proposed as an alternative to the operating microscope. The authors illustrate a case of the midline supracerebellar infratentorial approach to resect a pineal region tumor using the Modus V exoscope and demonstrate the improved visualization of critical structures in this deep location. Additionally, the marked improvement in surgeon comfort suggests that this system may have significant advantages over traditional microscope-based surgery for tumors of the pineal region. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2127.

A Confirmed Case of Zoonotic Aortitis and Successful Repair with an Allograft.

INTRODUCTION: Streptococcus equi subspecies zooepidemicus is a ß-haemolytic group C streptococcus that is a highly contagious infection in horses and other equines. Streptococcus equi infection of the native human aorta is a rare form of aortitis that has a high mortality owing to rupture. Surgical intervention with debridement and broad spectrum intravenous antibiotics are essential to improve the patient's status. REPORT: In this case study, a 71 year old man with horse contact presented with left groin pain related to S. equi aortitis and mycotic aneurysm. He was successfully treated by excision of the infected abdominal aorta and iliac vessels with a biological graft reconstruction, debridement of the retroperitoneum, and application of antibiotic beads. DISCUSSION: Biological grafts have never been reportedly used in group C streptococcus infection of the native aorta, and the patient recovered without sequelae. The promising outcome of this case may provide a framework for future similar cases.

Virtual Exploration of Safe Entry Zones in the Brainstem: Comprehensive Definition and Analysis of the Operative Approach.

BACKGROUND: A detailed and accurate understanding of the intrinsic brainstem anatomy and the interrelationship between its internal tracts and nuclei and external landmarks is of paramount importance for safe and effective brainstem surgery. Using anatomical models can be an important step in increasing such understanding. In the present study, we have shown the applicability of our developed virtual 3-dimensional (3D) model in depicting the safe entry zones (SEZs) to the brainstem. METHODS: Accurate 3D virtual models of brainstem elements were created using high-resolution magnetic resonance imaging and computed tomography to depict the brainstem SEZs. RESULTS: All the described SEZs to different parts of the brainstem were successfully depicted using our 3D virtual models. CONCLUSIONS: The virtual models provide an immersive experience of brainstem anatomy, allowing users to understand the intricacies of the microdissection that is necessary to appropriately work through the brainstem nuclei and tracts toward a particular target. The models provide an unparalleled learning environment to understand the SEZs into the brainstem that can be used for training and research.

Fluorescent imaging of protein myristoylation during cellular differentiation and development.

Protein post-translational modifications (PTMs) serve to give proteins new cellular functions and can influence spatial distribution and enzymatic activity, greatly enriching the complexity of the proteome. Lipidation is a PTM that regulates protein stability, function, and subcellular localization. To complement advances in proteomic identification of lipidated proteins, we have developed a method to image the spatial distribution of proteins that have been co- and post-translationally modified via the addition of myristic acid (Myr) to the N terminus. In this work, we use a Myr analog, 12-azidododecanoic acid (12-ADA), to facilitate fluorescent detection of myristoylated proteins in vitro and in vivo. The azide moiety of 12-ADA does not react to natural biological chemistries, but is selectively reactive with alkyne functionalized fluorescent dyes. We find that the spatial distribution of myristoylated proteins varies dramatically between undifferentiated and differentiated muscle cells in vitro. Further, we demonstrate that our methodology can visualize the distribution of myristoylated proteins in zebrafish muscle in vivo. Selective protein labeling with noncanonical fatty acids, such as 12-ADA, can be used to determine the biological function of myristoylation and other lipid-based PTMs and can be extended to study deregulated protein lipidation in disease states.

Incorporation of non-canonical amino acids into the developing murine proteome.

Analysis of the developing proteome has been complicated by a lack of tools that can be easily employed to label and identify newly synthesized proteins within complex biological mixtures. Here, we demonstrate that the methionine analogs azidohomoalanine and homopropargylglycine can be globally incorporated into the proteome of mice through facile intraperitoneal injections. These analogs contain bio-orthogonal chemical handles to which fluorescent tags can be conjugated to identify newly synthesized proteins. We show these non-canonical amino acids are incorporated into various tissues in juvenile mice and in a concentration dependent manner. Furthermore, administration of these methionine analogs to pregnant dams during a critical stage of murine development, E10.5-12.5 when many tissues are assembling, does not overtly disrupt development as assessed by proteomic analysis and normal parturition and growth of pups. This successful demonstration that non-canonical amino acids can be directly administered in vivo will enable future studies that seek to characterize the murine proteome during growth, disease and repair.