Anatomical Step-by-Step Dissection of Complex Skull Base Approaches for Trainees: Surgical Anatomy of the Far Lateral Approach.

Introduction Skull base neuroanatomy is classically taught using surgical atlases. Although these texts are critical and rich resources for learning three-dimensional (3D) relationships between key structures, we believe they could be optimized and complemented with step-by-step anatomical dissections to fully meet the learning needs of trainees. Methods Six sides of three formalin-fixed latex-injected specimens were dissected under microscopic magnification. A far lateral craniotomy was performed by each of three neurosurgery resident/fellow at varying stages of training. The study objective was the completion and photodocumentation of the craniotomy to accompany a stepwise description of the exposure to provide a comprehensive, intelligible, and anatomically oriented resource for trainees at any level. Illustrative case examples were prepared to supplement approach dissections. Results The far lateral approach provides a wide and versatile corridor for posterior fossa operation, with access spanning the entire cerebellopontine angle (CPA), foramen magnum, and upper cervical region. Key Steps Include The study includes the following steps: positioning and skin incision, myocutaneous flap, placement of burr holes and sigmoid trough, fashioning of the craniotomy bone flap, bilateral C1 laminectomy, occipital condyle/jugular tubercle drilling, and dural opening. Conclusion Although more cumbersome than the retrosigmoid approach, a far lateral craniotomy offers unparalleled access to lesions centered lower or more medially in the CPA, as well as those with significant extension into the clival or foramen magnum regions. Dissection-based neuroanatomic guides to operative approaches provide a unique and rich resource for trainees to comprehend, prepare for, practice, and perform complex cranial operations, such as the far lateral craniotomy.

Foundations and guidelines for high-quality three-dimensional models using photogrammetry: a technical note on the future of neuroanatomy education.

Hands-on dissections using cadaveric tissues for neuroanatomical education are not easily available in many educational institutions due to financial, safety and ethical factors. Supplementary pedagogical tools, for instance 3D models of anatomical specimens acquired with photogrammetry are an efficient alternative to democratize the 3D anatomical data. The aim of this study was to describe a technical guideline for acquiring realistic 3D anatomic models with photogrammetry and to improve the teaching and learning process in neuroanatomy. Seven specimens with different sizes, cadaveric tissues, and textures were used to demonstrate the step-by-step instructions for specimen preparation, photogrammetry setup, post-processing, and display of the 3D model. The photogrammetry scanning consists of 3 cameras arranged vertically facing the specimen to be scanned. In order to optimize the scanning process and the acquisition of optimal images, high-quality 3D models require complex and challenging adjustments in the positioning of the specimens within the scanner, as well as adjustments of the turntable, custom specimen holders, cameras, lighting, computer hardware and its software. MeshLab® software was used for editing the 3D model before exporting it to MedReality® (Thyng, Chicago, IL) and SketchFab® (Epic, Cary NC) platforms. Both allow manipulation of the models using various angles and magnifications and are easily accessed using mobile, immersive, and personal computer devices free of charge for viewers. Photogrammetry scans offer a 360° view of the 3D models ubiquitously accessible on any device independent of operating system and should be considered as a tool to optimize and democratize the teaching of neuroanatomy.

Interventional radiology in obstetric patients: A population-based record linkage study of use and outcomes.

INTRODUCTION: Interventional radiology (IR) is a technique for controlling hemorrhage and preserving fertility for women with serious obstetric conditions such as placenta accreta spectrum (PAS) or postpartum hemorrhage. This study examined maternal, pregnancy and hospital characteristics and outcomes for women receiving IR in pregnancy and postpartum. MATERIAL AND METHODS: A population-based record linkage study was conducted, including all women who gave birth in hospital in New South Wales or the major tertiary hospital in the neighboring Australian Capital Territory, Australia, between 2003 and 2019. Data were obtained from birth and hospital records. Characteristics and outcomes of women who underwent IR in pregnancy or postpartum are described. Outcomes following IR were compared in a high-risk cohort of women: those with PAS who had a planned cesarean with hysterectomy. Women were grouped by those who did and those who did did not have IR and were matched using propensity score and other factors. RESULTS: We identified IR in 236 pregnancies of 1 584 708 (15.0 per 100 000), including 208 in the delivery and 26 in a postpartum admission. Two-thirds of women receiving IR in the birth admission received a transfusion of red cells or blood products, 28% underwent hysterectomy and 12.5% were readmitted within 6 weeks. Other complications included: severe maternal morbidity (29.8%), genitourinary tract trauma/repair (17.3%) and deep vein thrombosis/pulmonary embolism (4.3%). Outcomes for women with PAS who underwent planned cesarean with hysterectomy were similar for those who did and did not receive IR, with a small reduction in transfusion requirement for those who received IR. CONCLUSIONS: Interventional radiology is infrequently used in pregnant women. In our study it was performed at a limited number of hospitals, largely tertiary centers, with the level of adverse outcomes reflecting use in a high-risk population. For women with PAS undergoing planned cesarean with hysterectomy, most outcomes were similar for those receiving IR and those not receiving IR, but IR may reduce bleeding.

Vernix Caseosa Peritonitis Causing Acute Abdomen After Cesarean Section: A Case Series.

BACKGROUND Vernix caseosa peritonitis (VCP) is a rare complication that typically presents following an otherwise uneventful cesarean section. Leakage of vernix caseosa into the peritoneum is thought to elicit a granulomatous foreign body reaction. Symptoms can be similar to other acute abdominal conditions, and diagnosis is confirmed by intraoperative findings and histological examination. Peritoneal lavage with supportive measures is the mainstay of treatment and recovery. CASE REPORT Case 1 was a 30-year-old woman who developed right iliac fossa pain, fever, tachycardia, and tachypnea less than a week after her lower segment cesarean section (LSCS). She underwent a laparoscopy for a peritonitic abdomen and concern for intra-abdominal sepsis. A peritoneal biopsy demonstrated histological changes consistent with VCP. Case 2 was a 39-year-old woman who underwent a LSCS. After discharge, she re-presented with generalized abdominal pain. With computed tomography (CT) scan findings suggestive of appendicitis, an appendectomy was performed, and vernix caseosa was detected in all quadrants. Case 3 was a 33-year-old woman who presented with fever, vomiting, diarrhea, and iliac fossa pain 9 days following an LSCS. She was given analgesia and antibiotics for a pelvic fluid collection noted on CT scan. She re-presented with tense swelling and pain above her cesarean section incision. Laparoscopy revealed adhesions over the lower abdomen and pelvis and white plaques suggestive of vernix caseosa along the peritoneal side walls. CONCLUSIONS The rising incidence of cesarean births worldwide creates the potential for increased numbers of VCP cases. Greater recognition of VCP is warranted to prevent unnecessary procedures.

Development and validation of a recursive partitioning analysis-based pretreatment decision-making tool identifying ideal candidates for spine stereotactic body radiation therapy.

BACKGROUND: This study was aimed at developing and validating a decision-making tool predictive of overall survival (OS) for patients receiving stereotactic body radiation therapy (SBRT) for spinal metastases. METHODS: Three hundred sixty-one patients at one institution were used for the training set, and 182 at a second institution were used for external validation. Treatments most commonly involved one or three fractions of spine SBRT. Exclusion criteria included proton therapy and benign histologies. RESULTS: The final model consisted of the following variables and scores: Spinal Instability Neoplastic Score (SINS) ≥ 6 (1), time from primary diagnosis < 21 months (1), Eastern Cooperative Oncology Group (ECOG) performance status = 1 (1) or ECOG performance status > 1 (2), and >1 organ system involved (1). Each variable was an independent predictor of OS (p < .001), and each 1-point increase in the score was associated with a hazard ratio of 2.01 (95% confidence interval [CI], 1.79-2.25; p < .0001). The concordance value was 0.75 (95% CI, 0.71-0.78). The scores were discretized into three groups-favorable (score = 0-1), intermediate (score = 2), and poor survival (score = 3-5)-with 2-year OS rates of 84% (95% CI, 79%-90%), 46% (95% CI, 36%-59%), and 21% (95% CI, 14%-32%), respectively (p < .0001 for each). In the external validation set (182 patients), the score was also predictive of OS (p < .0001). Increasing SINS was predictive of decreased OS as a continuous variable (p < .0001). CONCLUSIONS: This novel score is proposed as a decision-making tool to help to optimize patient selection for spine SBRT. SINS may be an independent predictor of OS.

Point of Care Virtual Surgical Planning and 3D Printing in Facial Feminization Surgery.

The use of virtual surgical planning (VSP) and three-dimensional printing (3DP) technologies in the routine facial feminization surgery practice has gained a significant popularity over the past few years. The clinical applications of them are claimed to improve safety, accuracy, and efficiency of facial feminization surgeries. In this article, we review and discuss the current applications of VSP and 3DP in different facial feminization procedures.

Virtual Planning and 3D Printing in the Management of Acute Orbital Fractures and Post-Traumatic Deformities.

Virtual surgical planning (VSP) and three-dimensional (3D) printing have advanced surgical reconstruction of orbital defects. Individualized 3D models of patients' orbital bony and soft tissues provide the surgeon with corrected orbital volume based on normalized anatomy, precise location of critical structures, and when needed a better visualization of the defect or altered anatomy that are paramount in preoperative planning. The use of 3D models preoperatively allows surgeons to improve the accuracy and safety of reconstruction, reduces intraoperative time, and most importantly lowers the rate of common postoperative complications, including over- or undercontouring of plates, orbital implant malposition, enophthalmos, and hypoglobus. As 3D printers and materials become more accessible and cheaper, the utility of printing patient-specific implants becomes more feasible. This article summarizes the traditional surgical management of orbital fractures and reviews advances in VSP and 3D printing in this field. It also discusses the use of in-house (point-of-care) VSP and 3D printing to further advance care of acute orbital trauma and posttraumatic deformities.

Establishing a Point-of-Care Virtual Planning and 3D Printing Program.

Virtual surgical planning (VSP) and three-dimensional (3D) printing have become a standard of care at our institution, transforming the surgical care of complex patients. Patient-specific, anatomic models and surgical guides are clinically used to improve multidisciplinary communication, presurgical planning, intraoperative guidance, and the patient informed consent. Recent innovations have allowed both VSP and 3D printing to become more accessible to various sized hospital systems. Insourcing such work has several advantages including quicker turnaround times and increased innovation through collaborative multidisciplinary teams. Centralizing 3D printing programs at the point-of-care provides a greater cost-efficient investment for institutions. The following article will detail capital equipment needs, institutional structure, operational personnel, and other considerations necessary in the establishment of a POC manufacturing program.

Virtual surgical planning and mirrored, 3-dimensionally printed guides for corrective clavicle osteotomies in clavicle malunions and nonunions.

BACKGROUND: The objective of this study was to retrospectively review clinical and radiographic outcomes of patients who underwent corrective osteotomies for clavicle malunion and internal fixation for nonunion using a combination of virtual surgical planning, patient-specific 3-dimensional (3D)-printed clavicles, and 3D-printed cutting guides manufactured at the point of care. METHODS: Between 2015 and 2021, 18 patients underwent corrective osteotomy for a clavicle malunion (7 shoulders) or internal fixation for a clavicle nonunion (11 shoulders). There were 11 male and 7 female individuals with an average patient age of 43.9 (range 19-76) years. All patients underwent computed tomography evaluation of both clavicles. The DICOM files were manually segmented, virtual surgical planning was performed selectively using commercially available software, and a mirrored version of the normal clavicle was 3D printed along with a 3D-printed replica of the affected clavicle. Three-dimensionally printed mirrored clavicles were used in all cases to ensure adequate restoration of the shape and length of the clavicle and to precontour fixation plates. Virtual surgical planning and 3D-printed cutting guides for osteotomy were used in 4 of 18 (22%) patients. Either cancellous or structural intercalary bone grafting was used in 15 of 18 (83%) cases. Patients were contacted postoperatively to determine clinical outcome scores. Preoperative, early postoperative, and late postoperative radiographs were reviewed to assess for union and complications. The average follow-up time was 24.9 months. RESULTS: Radiographic evaluation at the most recent follow-up demonstrated adequate restoration of length and successful union for all shoulders. There were no complications or reoperations. Postoperative patient-reported outcomes could be obtained in 16 of 18 (88.9%) patients. At the most recent follow-up, the mean visual analog scale for pain was 2.38 points (range, 1-7), the mean shoulder American Shoulder and Elbow Surgeons score was 73.2 points (range, 25-100), and the mean Patient-Reported Outcome Measurement Information System Upper Extremity score was 26 points (range, 7-35). All (100%) the patients were satisfied with their outcome (9 very satisfied, 7 satisfied), and their mean subjective shoulder value was 73% (range, 10%-100%). However, 2 patients complained of hardware-related symptoms, and 1 patient had return of preoperative symptoms after an interim 2 years of pain relief. CONCLUSION: The use of mirrored 3D-printed clavicles combined with virtual surgical planning and patient-specific 3D guides provides a reliable technique for restoring native anatomy when performing corrective osteotomies for clavicle malunion or internal fixation for clavicle nonunion, with a high rate of satisfactory clinical and radiographic outcomes.

Single and Multifraction Spine Stereotactic Body Radiation Therapy and the Risk of Radiation Induced Myelopathy.

Purpose: This study reports on the risk of radiation-induced myelitis (RM) of the spinal cord from a large single-institutional experience with 1 to 5 fraction stereotactic body radiation therapy (SBRT) to the spine. Methods and Materials: A retrospective review of patients who received spine SBRT to a radiation naïve level at or above the conus medullaris between 2007 and 2019 was performed. Local failure determination was based on SPIne response assessment in Neuro-Oncology criteria. RM was defined as neurologic symptoms consistent with the segment of cord irradiated in the absence of neoplastic disease recurrence and graded by Common Toxicity Criteria for Adverse Events, version 4.0. Rates of adverse events were estimated and dose-volume statistics from delivered treatment plans were extracted for the planning target volumes and spinal cord. Results: A total of 353 lesions in 277 patients were identified that met the specified criteria, for which 270, 70, and 13 lesions received 1-, 3-, and 5-fraction treatments, respectively, with a median follow-up of 46 months (95% confidence interval [CI], 41-52 months) for all surviving patients. The median overall survival was 33.0 months (95% CI, 29-43). The median D0.03cc to the spinal cord was 11.7 Gy (interquartile range [IQR], 10.5-12.4), 16.7 Gy (IQR, 12.8-20.6), and 26.0 Gy (IQR, 24.1-28.1), for 1-, 3-, 5-fractions. Using an a/b = 2Gy for the spinal cord, the median single-fraction equivalent-dose (SFED2) was 11.7 Gy (IQR, 10.2-12.5 Gy) and the normalized biological equivalent dose (nBED2/2) was 19.9 Gy (IQR, 15.4-22.8 Gy). One patient experienced grade 2 RM after a single-fraction treatment. The cumulative probability of RM was 0.3% (95% CI, 0%-2%). Conclusions: Spine SBRT is safe while limiting the spinal cord (as defined on treatment planning magnetic resonance imaging or computed tomography myelogram) D0.03cc to less than 14 Gy, 21.9 Gy, and 30 Gy, for 1, 3, and 5-fractions, consistent with standard guidelines.

Utility and Costs During the Initial Year of 3D Printing in an Academic Hospital.

OBJECTIVE: There is a paucity of utility and cost data regarding the launch of 3D printing in a hospital. The objective of this project is to benchmark utility and costs for radiology-based in-hospital 3D printing of anatomic models in a single, adult academic hospital. METHODS: All consecutive patients for whom 3D printed anatomic models were requested during the first year of operation were included. All 3D printing activities were documented by the 3D printing faculty and referring specialists. For patients who underwent a procedure informed by 3D printing, clinical utility was determined by the specialist who requested the model. A new metric for utility termed Anatomic Model Utility Points with range 0 (lowest utility) to 500 (highest utility) was derived from the specialist answers to Likert statements. Costs expressed in United States dollars were tallied from all 3D printing human resources and overhead. Total costs, focused costs, and outsourced costs were estimated. The specialist estimated the procedure room time saved from the 3D printed model. The time saved was converted to dollars using hospital procedure room costs. RESULTS: The 78 patients referred for 3D printed anatomic models included 11 clinical indications. For the 68 patients who had a procedure, the anatomic model utility points had an overall mean (SD) of 312 (57) per patient (range, 200-450 points). The total operation cost was $213,450. The total cost, focused costs, and outsourced costs were $2,737, $2,180, and $2,467 per model, respectively. Estimated procedure time saved had a mean (SD) of 29.9 (12.1) min (range, 0-60 min). The hospital procedure room cost per minute was $97 (theoretical $2,900 per patient saved with model). DISCUSSION: Utility and cost benchmarks for anatomic models 3D printed in a hospital can inform health care budgets. Realizing pecuniary benefit from the procedure time saved requires future research.

A Radiologist's Guide to the 2021 WHO Central Nervous System Tumor Classification: Part I-Key Concepts and the Spectrum of Diffuse Gliomas.

The fifth edition of the World Health Organization (WHO) classification of tumors of the central nervous system, published in 2021, contains substantial updates in the classification of tumor types. Many of these changes are relevant to radiologists, including "big picture" changes to tumor diagnosis methods, nomenclature, and grading, which apply broadly to many or all central nervous system tumor types, as well as the addition, elimination, and renaming of multiple specific tumor types. Radiologists are integral in interpreting brain tumor imaging studies and have a considerable impact on patient care. Thus, radiologists must be aware of pertinent changes in the field. Staying updated with the most current guidelines allows radiologists to be informed and effective at multidisciplinary tumor boards and in interactions with colleagues in neuro-oncology, neurosurgery, radiation oncology, and neuropathology. This review represents the first of a two-installment review series on the most recent changes to the WHO brain tumor classification system. This first installment focuses on the changes to the classification of adult and pediatric gliomas of greatest relevance for radiologists.

Three-Dimensional-Printed Vaginal Molds for Use After McIndoe Neovagina Creation.

BACKGROUND: Surgical vaginoplasty is a highly successful treatment for congenital absence of the vagina. One key to long-term success is the use of an appropriate vaginal mold in the immediate postoperative period. We present the use of a three-dimensional (3D)-printed vaginal mold, customizable to the anatomy of individual patients. TECHNIQUE: Vaginal molds were designed using a 3D modeling software program. The design included narrowing around the urethra, holes for egress of secretions, and a knob for insertion and removal. Dental resin was 3D-printed into various-sized vaginal molds, and postprocessing was performed. EXPERIENCE: We present the use of the 3D-printed mold for a patient with a history of cloacal exstrophy and a unique pelvic shape. Two prior neovagina surgeries in this patient had been unsuccessful due to ineffective handheld dilator use; the patient experienced success with the 3D-printed intravaginal mold. CONCLUSION: The use of the 3D-printed vaginal mold is an alternative to the limited commercially available models today and allows for customization to user anatomy. With 3D printers becoming more widely accessible, we believe this method could become universally accepted, with hopes of contributing to increased patient satisfaction and decreased complications.

Three-Dimensional Modeling for Augmented and Virtual Reality-Based Posterior Fossa Approach Selection Training: Technical Overview of Novel Open-Source Materials.

BACKGROUND: Selection of skull base approaches is a critical skill for complex cranial surgery, which demands nuanced understanding of neuroanatomy and pathology. OBJECTIVE: To develop novel pedagogical resources for approach selection education and assessment. METHODS: A prospectively maintained skull base registry was screened for posterior fossa tumors amenable to 3-dimensional (3D) modeling of multiple operative approaches. Inclusion criteria were high-resolution preoperative and postoperative computed tomography and MRI studies (≤1 mm) and consensus that at least 3 posterior fossa craniotomies would provide feasible access. Cases were segmented using Mimics and modeled using 3-Matic. Clinical Vignettes, Approach Selection Questionnaire, and Clinical Application Questionnaire were compiled for implementation as a teaching/testing tool. RESULTS: Seven cases were selected, each representing a major posterior fossa approach group. 3D models were rendered using clinical imaging for the primary operative approach, as well as a combination of laboratory neuroanatomic data and extrapolation from comparable craniotomies to generate 2 alternative approaches in each patient. Modeling data for 3D figures were uploaded to an open-sourced database in a platform-neutral fashion (.x3d) for virtual/augmented reality and 3D printing applications. A semitransparent model of each approach without pathology and with key deep structures visualized was also modeled and included for comprehensive understanding. CONCLUSION: We report a novel series of open-source 3D models for skull base approach selection training, with supplemental resources. To the best of our knowledge, this is the first such series designed for pedagogical purposes in skull base surgery or centered on open-source principles.

3D printing in aortic endovascular therapies.

Endovascular treatment of aortic disease, including aneurysm or dissection, is expanding at a rapid pace. Often, the specific patient anatomy in these cases is complex. Additive manufacturing, also known as three-dimensional (3D) printing, is especially useful in the treatment of aortic disease, due to its ability to manufacture physical models of complex patient anatomy. Compared to other surgical procedures, endovascular aortic repair can readily exploit the advantages of 3D printing with regard to operative planning and preoperative training. To date, there have been numerous uses of 3D printing in the treatment of aortic pathology as an adjunct in presurgical planning and as a basis for training modules for fellows and residents. In this review, we summarize the current uses of 3D printing in the endovascular management of aortic disease. We also review the process of producing these models, the limitations of their applications, and future directions of 3D printing in this field.

Infrapedicular approach to CT-guided thermal ablation in the thoracic spine.

OBJECTIVES: The aim of this study is to introduce the infrapedicular approach to CT-guided spine interventions, a specialized technique that can safely expand the scope of spine lesions amenable to treatment, and to document the feasibility in a variety of procedural scenarios to the extent possible with a retrospective case series. METHODS: Data from 24 cases performed at a single institution over a 10-year period were retrospectively reviewed to assess the technical feasibility and safety profile of the technique. RESULTS: The infrapedicular approach enabled a technically satisfactory procedural result in 24 cases (mean age 63.9 years, range 35-83 years). Two peri-procedural complications occurred, including a small pneumothorax and a cerebrospinal fluid leak, both of which resolved with conservative treatment. No lasting injurious effects or additional complications were identified. The infrapedicular approach was found to be particularly useful in multiple technically challenging scenarios: it facilitates access to lesions in the inferior vertebral body, allows biopsy, cement augmentation, or ablation of high thoracic lesions difficult to treat due to limitations of steep angulation of fluoroscopy and CT scanners, and enables treatment of large lesions by using multiple overlapping probes.

Accuracy and Precision of the Computed Tomographic Angiography Perforator Localization Technique for Virtual Surgical Planning of Composite Osteocutaneous Fibular Free Flaps in Head and Neck Reconstruction.

BACKGROUND: Virtual surgical planning (VSP), computer aided design/computer aided modeling, and 3-dimensional printing technology have been shown to improve surgical accuracy and efficiency in head and neck reconstruction. However, persisting criticism of the technology is that it does not adequately address the soft tissue-related aspects of reconstructive surgery. Prior publication on the computed tomographic angiography (CTA) perforator localization technique has demonstrated how soft tissue planning can be incorporated directly into existing VSP workflows. PURPOSE: The aim of this study is to prospectively assess the accuracy, precision, negative predictive value (NPV), and positive predictive value (PPV) of the CTA perforator localization technique for VSP of osteocutaneous fibular free flaps. MATERIALS AND METHODS: A prospective observational study in a consecutive cohort of subjects undergoing VSP of osteocutaneous fibular free flaps at Mayo Clinic between 2018 and 2020 was completed. All cutaneous perforators from the peroneal system of the selected donor leg were identified preoperatively through a previously reported CTA tracing method and registered into the VSP. Perforators were classified as primary or secondary based on whether the perforators were targeted for use in the final reconstructive plan. Perforator measurements obtained from the VSP were cross-referenced with intraoperatively obtained measurements of actual perforator locations to calculate accuracy, precision, NPV, PPV, sensitivity, and specificity of the CTA localization technique. RESULTS: Sixty consecutive subjects were enrolled in the study. A total of 141 perforators were identified preoperatively on CTA and 145 perforators were identified on operative exposure. One perforator identified on preoperative CTA was not identified on surgical exposure (false positive perforator). Six perforators were identified on operative exposure alone without recognition on preoperative CTA (false negative perforators). The accuracy of CTA perforator identification was 96.52%. Median precision of perforator localization was 0.3 cm (standard deviation 0.40) between CTA and operatively identified locations. PPV of the technique was 99.29% and NPV was 90.00%. CONCLUSION: The CTA localization technique for identifying and incorporating cutaneous perforator locations into VSP of osteocutaneous fibular free flaps is a reliable, accurate, and precise technique to employ in the modern paradigm of guided surgery for head and neck reconstruction.

MRI enhancement patterns in 28 cases of clival chordomas.

Clival chordomas are classically thought of as locally aggressive tumors of the skull base and differentiate themselves from their benign counterparts by demonstrating moderate to marked contrast enhancement, reported as 95-100% in prior studies. The purpose of this review was to evaluate the imaging characteristics of lesions from a single institution classified as clival chordomas with an emphasis of highlighting lesions that do not follow the prevalent current description for chordoma. We searched our institutional databases for all patients with pathologically proven clival chordomas from 1997 to 2017 who had pre-operative imaging available. The images were evaluated for degree of contrast enhancement, MRI signal characteristics, osseous involvement, location, aggressiveness of appearance, and presence of calcifications. 28 cases were identified that had preoperative imaging available for review. Over half of the patients demonstrated either no/minimal (11/28, 39%) or mild enhancement (7/28, 25%). The remaining cases demonstrated moderate (4/28, 14%) and marked enhancement (6/28, 21%). The 4 lesions measuring less than 20 mm all had mild to minimal/no enhancement and lacked aggressive features on CT. Our experience finds that over half (64%) of clival chordomas will demonstrate mild or no enhancement at all. These findings suggest that the lack of MRI contrast enhancement should not be synonymous with a benign clival mass.

Dysregulation of Oxygen Sensing/Response Pathways in Pregnancies Complicated by Idiopathic Intrauterine Growth Restriction and Early-Onset Preeclampsia.

Preeclampsia (PE) and intrauterine growth restriction (IUGR) are the leading causes of maternal and fetal morbidity/mortality. The central deficit in both conditions is impaired placentation due to poor trophoblast invasion, resulting in a hypoxic milieu in which oxidative stress contributes to the pathology. We examine the factors driving the hypoxic response in severely preterm PE (n = 19) and IUGR (n = 16) placentae compared to the spontaneous preterm (SPT) controls (n = 13) using immunoblotting, RT-PCR, immunohistochemistry, proximity ligation assays, and Co-IP. Both hypoxia-inducible factor (HIF)-1α and HIF-2α are increased at the protein level and functional in pathological placentae, as target genes prolyl hydroxylase domain (PHD)2, PHD3, and soluble fms-like tyrosine kinase-1 (sFlt-1) are increased. Accumulation of HIF-α-subunits occurs in the presence of accessory molecules required for their degradation (PHD1, PHD2, and PHD3 and the E3 ligase von Hippel-Lindau (VHL)), which were equally expressed or elevated in the placental lysates of PE and IUGR. However, complex formation between VHL and HIF-α-subunits is defective. This is associated with enhanced VHL/DJ1 complex formation in both PE and IUGR. In conclusion, we establish a significant mechanism driving the maladaptive responses to hypoxia in the placentae from severe PE and IUGR, which is central to the pathogenesis of both diseases.