Revealing an Uncommon Presentation of Chiari I Malformation With Diverse Craniovertebral Anomalies in the Absence of Syringomyelia and Atlanto-Occipital Subluxation: A Case Report.

A Chiari I malformation is a frequently encountered anomaly of the posterior fossa, occurring in a notable percentage of the population. It often coexists with various other craniovertebral junction abnormalities, albeit less frequently with Klippel-Feil syndrome. Interestingly, the majority of individuals with Chiari I malformation do not exhibit any symptoms. We present a rare case of a 25-year-old male with chronic neck and occipital pain, along with progressive weakness and sensory disturbances in all four limbs, urinary urgency, and elevated left shoulder. Examination unveiled spasticity, weakness, and brisk reflexes. On extensive radiological evaluation (X-ray, CT, and MRI), findings revealed various anomalies in the craniovertebral junction, including complete atlanto-occipital assimilation, basilar invagination, and platybasia. Furthermore, cervical segmentation abnormalities indicative of Klippel-Feil syndrome were observed, along with Sprengel's deformity. MRI confirmed Chiari I malformation with tonsillar herniation and myelomalacia, as well as compression at the cervico-medullary junction. This patient underwent a surgical procedure that included transoral odontoidectomy combined with occipito-cervical fixation, after which a good clinical response was observed. It emphasizes the necessity of radiological imaging for the diagnosis of Chiari and other associated abnormalities in the craniovertebral junction.

Removal method of a Supera interwoven stent invaginated during its implantation in endovascular procedure: a case report.

BACKGROUND: Supera interwoven stents (IWS) have a unique interwoven structure; thus, precise stent placement can be challenging as they are prone to elongation, shortening, and invagination. Particularly, invagination limits long-term patency. This proposed method aims to remove invaginated IWS. CASE PRESENTATION: A 70-year-old man presented with intermittent claudication in his left lower limb. Endovascular therapy was conventionally performed, and a 5.5 × 40 mm IWS was placed after balloon dilatation; however, invagination occurred. The invaginated IWS was successfully removed by a threading 0.014" wire through the outside of the stent strut, and a snare catheter was used to hold it in place from the inside. Then, while still in place, the 0.014" wire and snare catheter were driven into the guiding sheath. CONCLUSIONS: This practical and easy approach to remove invaginated IWS from the body relies on the particular structural characteristics.

Evaluation of Craniometric Parameters and Efficacy of Posterior Surgical Intervention Types in Basilar Invagination Patients.

OBJECTIVE: Basilar invagination is one of the most frequently observed abnormalities at the craniovertebral junction, in which the odontoid process of C2 prolapses into the foramen magnum. METHODS: The current study included 27 patients who underwent surgery for basilar invagination between October 2013 and January 2023. The study group was divided into 2 groups according to basilar invagination types; type I (the presence of type A atlantoaxial instability and instability is the main pathology) and type II (the presence of type B and C atlantoaxial instability and skull base dysgenesis is the main pathology). Craniometric parameters included in the study were atlantodental interval, posterior atlantodental interval, Chamberlain's line violation, clivus-canal angle, Welcher's basal angle, and Boogaard angle. RESULTS: The mean age of the patients was 24.30 ± 14.36 years (5-57 years). Fourteen patients (51.9%) were female, and 13 patients (48.1%) were male. Ten patients (37%) had type I basilar invagination, and 17 patients (63%) had type II basilar invagination. Preoperative and postoperative atlantodental interval and Boogaard angle were significantly higher in type I basilar invagination, as preoperative and postoperative posterior atlantodental interval and clivus-canal angle were significantly higher in type II basilar invagination. There was a positive strong correlation between Chamberlain's line violation and Boogaard angle. Postoperative Chamberlain's line violation was significantly higher in occipitocervical fixation (P = 0.035). C1 lateral mass screw fixation was found more successful in Chamberlain's line violation correction than occipital plates. Occipitocervical fixation was found to be associated with higher postoperative Nurick scores (P = 0.015) and complication rates (P = 0.020). Cages applied to the C1-C2 joint space were found to be associated with higher fusion rates (P = 0.023) and lower complication rates (P = 0.024). CONCLUSIONS: In the present study, it was found that C1-C2 fixation was more successful in correcting craniometric parameters and had lower complication rates than occipitocervical fixation. In appropriate patients, it was determined that cage application increased the success rates of the operations.

A Global Bibliometric and Visualization Analysis of Craniovertebral Junction Bony Abnormalities Based on VOSviewer and Citespace.

OBJECTIVE: Recent years have witnessed a rapidly growing interest in CVJ bony abnormalities, and a qualitative and quantitative analysis of relevant literatures is necessary. This study aims to identify and summarize the published articles related to craniovertebral junction bony abnormalities, to analyze and visualize the current research trends and major contributors. METHODS: We collected data from Web of Science, excluding certain article types. Two researchers screened articles for relevance. Data were organized with EndnoteX9, and analyzed using VOSviewer and CiteSpace for co-authorship, co-occurrence, keyword burst, and co-citation analyses to identify research trends and collaborations. RESULTS: A total of 2,776 articles were included, revealing an increasing trend in annual publications of CVJ bony abnormalities. The USA was the leading country. King Edward Memorial Hospital was the most prolific institution, and Seth GS Medical College had the most citations. The Spine is the most popular journal with the highest number of publications and citations. Professor Goel Atul from India emerged as the most influential pioneer in this field. Keyword analysis highlighted surgical techniques, diagnosis, and anatomy as the primary research hotspots and Fixation, Placement, and Basilar invagination gradually become the new research trend. However, there is a relative weakness in basic research and epidemiology. CONCLUSIONS: This study provides valuable insights into the current research trends and critical contributors in CVJ bony abnormalities, guiding evidence-based decisions and fostering international collaborations to advance knowledge in this field.

A Case of Long-Tract Ileocolic Intussusception Secondary to Well-Differentiated Cecal Adenocarcinoma.

Intussusception denotes the intricate phenomenon wherein one segment of the bowel undergoes invagination or telescoping into its contiguous distal segment. The ensuing invaginated segment may be propelled forward through peristaltic movements, potentially precipitating bowel obstruction or ischemia, culminating in necrosis of the affected bowel segment. Although the precise etiology of intussusception remains elusive, particularly in cases devoid of an identifiable lead point, dysrhythmic contractions and lymphoid hyperplasia have been implicated in the pathophysiology of this condition. We present the case of an 86-year-old African American female with a past medical history of hypertension and asthma who presented to our emergency room with a seven-day history of worsening abdominal. The pain was described as sharp and intermittent, and it would worsen with every meal or drink. A physical exam demonstrated the right lower quadrant with vague abdominal tenderness, especially below the umbilical region. Computed tomography of the abdomen and pelvis revealed a long segment of ileocolic obstructing intussusception in the ascending colon, with a 2.6 cm solid mass serving as a lead point. Swift intervention ensued with an urgent exploratory laparotomy, culminating in a right hemicolectomy to excise the intussuscepted segment of the bowel. The pathological examination identified a well-differentiated adenocarcinoma of the cecum, categorized as T1N0M0, with all 20 resected lymph nodes yielding negative results. This illustrative case presents a unique insight into a patient with ileocolic obstructing intussusception, caused by a well-differentiated adenocarcinoma acting as the lead point, a relatively uncommon occurrence in adults. Diagnosing intussusception in adults is challenging due to its nonspecific symptoms, which are similar to those of various other gastrointestinal disorders. Therefore, it is crucial for medical providers to be acutely aware of the possibility that adenocarcinoma can trigger obstructing intussusception in various parts of the bowel.

C1–C2 intraarticular distraction with anterior cervical cages for basilar invagination realignment: Operative technique nuances and review of literature

Neurosurgical management of basilar invagination (BI) has traditionally been aimed at direct cervicomedullary decompression through transoral dens resection or suboccipital decompression with supplemental instrumented fixation. Dr. Goel introduced chronic atlantoaxial dislocation (AAD) as the etiology in most cases of BI and described a technique for distracting the C1–C2 joint with interfacet spacers to achieve reduction and anatomic realignment. We present our modification to Goel’s surgical technique, in which we utilize anterior cervical discectomy (ACD) cages as C1–C2 interfacet implants. A young adult male presented to our institution with BI, cervicomedullary compression, occipitalization of C1, and Chiari 1 malformation. There was AAD of C1 over the C2 lateral masses. This reduced some with preoperative traction. He underwent successful C1–C2 interfacet joint reduction and arthrodesis with anterior cervical discectomy (ACD) cages and concomittant occiput to C2 instrumented fusion. BI can be effectively treated through reduction of AAD and by utilizing ACD cages as interfacet spacers. (AU)

El tratamiento neuroquirúrgico tradicional para la impresión basilar es principalmente a través de un abordaje trans-oral para la resección del proceso odontoide, seguido de una descompresión suboccipital con instrumentación posterior cervical. Dr. Goel presenta la dislocación atlanto-axial (AA) como una de las etiologías principales en los casos de impresión basilar. A su vez, describió la técnica quirúrgica que incluye la distracción de la articulación AA con cajas para fusión permitiendo la reducción y reajuste anatómico cervical. En este artículo presentamos una variación a la técnica quirúrgica del Dr. Goel en el cual utilizamos implantes utilizados en la discectomía y fusión cervical anterior (DFCA) para la articulación facetaria de C1–C2. Presentamos un paciente adulto masculino que evaluamos en nuestra institución con impresión basilar, compresión cérvico-medular, fusión occipital con el atlas y malformación de Chiari tipo 1. En adición, el paciente tenía evidencia radiográfica de dislocación AA. Se logro obtener reducción mínima de la impresión basilar con tracción cervical pre-operatoria. Luego, se sometió al tratamiento quirúrgico que consistió en el uso de implantes cervicales para la reducción y fusión de la articulación facetaria de C1–C2 complementado por instrumentación y fusión craneocervical. Esta técnica presentada sugiere que la reducción y reajuste anatómico cervical de la dislocación AA con implantes utilizados para DFCA puede ser efectivo para el tratamiento de impresión basilar. (AU)

Neurological outcome following delayed traction and fixation in severe tetraparesis consecutive to posterior decompression for Chiari malformation: a case report.

BACKGROUND: Chiari malformation type 1 (CM1) is a congenital hindbrain malformation characterized by herniation of the cerebellar tonsils below the foramen magnum. The term Chiari type 1.5 is used when herniation of the brainstem under the McRae line and anomalies of the craniovertebral junction are also present. These conditions are associated with several symptoms and signs, including headache, neck pain, and spinal cord syndrome. For symptomatic patients, surgical decompression is recommended. When radiographic indicators of craniovertebral junction (CVJ) instability or symptoms related to ventral brainstem compression are present, CVJ fixation should also be considered. CASE DESCRIPTION: We report the case of a 13-year-old girl who presented with severe tetraparesis after posterior decompression for Chiari malformation type 1.5, followed 5 days later by partial C2 laminectomy. Several months after the initial surgery, she underwent two fixations, first without and then with intraoperative cervical traction, leading to significant neurological improvement. DISCUSSION AND CONCLUSION: This case report underscores the importance of meticulous radiological analysis before CM surgery. For CM 1.5 patients with basilar invagination, CVJ fixation is recommended, and C2 laminectomy should be avoided. In the event of significant clinical deterioration due to nonadherence to these guidelines, our findings highlight the importance of traction with increased extension before fixation, even years after initial destabilizing surgery.

C1-C2 intraarticular distraction with anterior cervical cages for basilar invagination realignment: Operative technique nuances and review of literature.

Neurosurgical management of basilar invagination (BI) has traditionally been aimed at direct cervicomedullary decompression through transoral dens resection or suboccipital decompression with supplemental instrumented fixation. Dr. Goel introduced chronic atlantoaxial dislocation (AAD) as the etiology in most cases of BI and described a technique for distracting the C1-C2 joint with interfacet spacers to achieve reduction and anatomic realignment. We present our modification to Goel's surgical technique, in which we utilize anterior cervical discectomy (ACD) cages as C1-C2 interfacet implants. A young adult male presented to our institution with BI, cervicomedullary compression, occipitalization of C1, and Chiari 1 malformation. There was AAD of C1 over the C2 lateral masses. This reduced some with preoperative traction. He underwent successful C1-C2 interfacet joint reduction and arthrodesis with anterior cervical discectomy (ACD) cages and concomittant occiput to C2 instrumented fusion. BI can be effectively treated through reduction of AAD and by utilizing ACD cages as interfacet spacers.

A novel classification and management scheme for craniocervical junction disorders with ventral neural element compression.

OBJECTIVE: Craniocervical junction (CCJ) pathologies with ventral neural element compression are poorly understood, and appropriate management requires accurate understanding, description, and a more uniform nomenclature. The aim of this study was to evaluate patients to identify anatomical clusters and better classify CCJ disorders with ventral compression and guide treatment. METHODS: A retrospective review of adult and pediatric patients with ventral CCJ compression from 2008 to 2022 at a single center was performed. The incidence of anatomical abnormalities and compressive etiologies was assessed. Surgical approach, radiographic data, and outcomes were recorded. Association rules analysis (ARA) was used to assess variable clustering. RESULTS: Among 51 patients, the main causes of compression were either purely bony (retroflexed dens [n = 18]; basilar invagination [BI; n = 13]) or soft tissue (degenerative pannus [n = 16]; inflammatory pannus [n = 2]). The primary cluster in ARA was a retroflexed dens, platybasia, and Chiari malformation (CM), and the secondary cluster was BI, C1-2 subluxation, and reducibility. These, along with degenerative pannus, formed the three major classes. In assessing the optimal treatment strategy, reducibility was evaluated. Of the BI cases, 12 of the 13 patients had anterolisthesis of C1 that was potentially reducible, compared with 2 of the 18 patients with a retroflexed dens (both with concomitant BI), and no pannus cases. The mean C1-2 facet angle was significantly higher in BI at 32.4°, compared with -2.3° in retroflexed dens and 8.1° in degenerative pannus (p < 0.05). Endonasal decompression with posterior fixation was performed in 48 (94.0%) of the 51 patients, whereas posterior reduction/fixation alone was performed in 3 patients (6.0%). Of 16 reducible cases, open posterior reduction alone was successful in 3 (60.0%) of 5 cases, with all successes containing isolated BI. Reduction was not attempted if vertebral anatomy was unfavorable (n = 9) or the C1 lateral mass was absent (n = 5). The mean follow-up was 28 months. Symptoms improved in 88.9% of patients and were stable in the remaining 11.1%. Tracheostomy and percutaneous G-tube placement occurred in 7.8% and 11.8% of patients, respectively. Reoperation for an endonasal CSF leak repair or posterior cervical wound revision both occurred in 3.9% of patients. CONCLUSIONS: In classifying, one cluster caused decreased posterior fossa volume due to an anatomical triad of retroflexed dens, platybasia, and CM. The second cluster caused pannus formation due to degenerative hypertrophy. For both, endonasal decompression with posterior fixation was ideal. The third group contained C1 anterolisthesis characterized by a steep C1-2 facet angle causing reducible BI. Posterior reduction/fixation is the first-line treatment when anatomically feasible or endonasal decompression with in situ posterior fixation when anatomical constraints exist.

The role of sphenoid bone in basilar invagination pathophysiology.

Basilar invagination (BI) is characterized by rostral dislocation of the cervical spine toward the skull base. The craniometrics of the skull base have shown significant differences among craniocervical junction malformations. The sphenoid bone is the center of the skull base; however, no study has evaluated this bone in cases of BI. This was a cross-sectional study of MRI databanks from two institutions of the author's practice between 1985 and 2020. The craniometrics of the sphenoid bone were measured in BI patients and controls. Fifty-eight MRIs were selected, including 28 BI patients and 30 controls. The mean sphenoid crest-clivus length was 32.66 ± 4.7 mm in the BI group and 29.98 ± 3.0 mm in the control group (p = 0.01). The mean sphenoid planum-top of Dorsum sellae length was 28.53 ± 3.7 mm in the BI group and 26.45 ± 3.2 mm in the control group (p = 0.02). The mean tuberculum sellae-sphenoid floor height was 18.52 ± 4.4 mm in the BI group and 21.32 ± 2.9 mm in the control group (p = 0.00). The mean sella turcica-sphenoid floor height was 10.35 ± 3.8 mm in the BI group and 12.24 ± 3.5 mm in the control group (p = 0.05). The mean clivus length was 29.81 ± 6.3 mm in the BI group and 40.86 ± 4.2 mm in the control group (p = 0.00). The mean sphenoid length was 58.34 ± 7.4 mm in the BI group and 67.31 ± 6.0 mm in the control group (p = 0.00). The mean sphenoid angle was 116.33 ± 8.7° in the BI group and 112.36 ± 6.9° in the control group (p = 0.05). The BI sphenoid bone has shorter vertical dimensions and longer horizontal measures. This morphology promotes a flattening of the sphenoid angle. The sphenoid bone is significantly altered in BI, favoring the congenital hypothesis in the pathophysiology of this disease.

Structural and functional damage to neuronal nuclei caused by extracellular tau oligomers.

INTRODUCTION: Neuronal nuclei are normally smoothly surfaced. In Alzheimer's disease (AD) and other tauopathies, though, they often develop invaginations. We investigated mechanisms and functional consequences of neuronal nuclear invagination in tauopathies. METHODS: Nuclear invagination was assayed by immunofluorescence in the brain, and in cultured neurons before and after extracellular tau oligomer (xcTauO) exposure. Nucleocytoplasmic transport was assayed in cultured neurons. Gene expression was investigated using nanoString nCounter technology and quantitative reverse transcription polymerase chain reaction. RESULTS: Invaginated nuclei were twice as abundant in human AD as in cognitively normal adults, and were increased in mouse neurodegeneration models. In cultured neurons, nuclear invagination was induced by xcTauOs by an intracellular tau-dependent mechanism. xcTauOs impaired nucleocytoplasmic transport, increased histone H3 trimethylation at lysine 9, and altered gene expression, especially by increasing tau mRNA. DISCUSSION: xcTauOs may be a primary cause of nuclear invagination in vivo, and by extension, impair nucleocytoplasmic transport and induce pathogenic gene expression changes. HIGHLIGHTS: Extracellular tau oligomers (xcTauOs) cause neuronal nuclei to invaginate. xcTauOs alter nucleocytoplasmic transport, chromatin structure, and gene expression. The most upregulated gene is MAPT, which encodes tau. xcTauOs may thus drive a positive feedback loop for production of toxic tau.

Sigmoid-rectal intussusception in an elderly patient: A case report of an unusual presentation of intestinal obstruction.

INTRODUCTION: Sigmoid-rectal intussusception or invagination is an infrequently documented condition in the adult population, with only a handful of cases reported in the medical literature. The underlying pathological mechanism involves impaired peristalsis, often attributed to a malignant tumor. CASE PRESENTATION: A 78-year-old patient, with a history of abdominal pain and lower gastrointestinal bleeding, sought care at our emergency department with evident symptoms indicative of large bowel obstruction. Abdominal examination revealed distension and rectal examination found a mass mimicking an internal rectal prolapse. Subsequently, imaging studies confirmed the diagnosis of sigmoid-rectal intussusception. The patient underwent an emergency open sigmoid resection with Hartman's procedure. The postoperative course was uneventful. Anatomopathological analysis revealed the presence of stage I adenocarcinoma. A restoration of digestive continuity was scheduled six months later. One-year follow-up assessments showed no indications of local recurrence or distant metastasis. DISCUSSION: Sigmoid rectal intussusception stands as a unique and infrequently reported medical entity. The absence of distinct clinical symptoms often renders diagnosis a challenging task, with confirmation typically relying on radiological findings. In contrast to the non-surgical approaches employed in pediatric cases, intussusception in adults necessitates surgical intervention due to its predominantly malignant underpinnings. CONCLUSION: While sigmoid-rectal intussusception is an exceedingly rare occurrence, its manifestation with a multitude of non-specific symptoms can complicate clinical recognition. Nevertheless, it should be duly considered as a potential etiological factor in cases of large bowel obstruction, particularly when suggestive signs are found on rectal examination.

Facet-occiput slope angle: A novel predictor of cage placement feasibility during surgery in basilar invagination patients.

Background and aim: Direct posterior reduction and manipulation of the C1-2 joints, accompanied by placement of spacers, is the state-of-the-art technique for treating basilar invagination (BI) and atlantoaxial dislocation (AAD). The hindrance of occiput to reaching up to the true atlantoaxial facets (AAF) during the surgery remains challenging for cage placement. The aim of this study was to explore an objective and precise method of measuring the effect of the hindrance of occiput to reaching up to the true AAF and cage placement during surgery. Method: We collected the clinico-imaging data of 58 patients with BI and AAD (Group A) who underwent surgery in our hospital, and 78 control cohorts (Group B) were retrieved retrospectively. We measured facet-occiput slope angle (FOSA) in midsagittal CT. Patients were positioned prone for surgery based on preoperative flexion O-C2a, and access to the true AAF was observed intraoperatively. The cut-off value of FOSA for the feasibility of cage placement in BI and AAD patients was appointed when access to the true AAF was impossible due to the hindrance of occiput during surgery. Results: The cut-off value of FOSA for the feasibility of cage placement was 34o with an area under the curve AUC of 0.800 (95 % CI: 0.672-0.928, P < 0.001) and the Youden index of 0.607. In patients with FOSA >34o, reaching up to the true AAF and 3D-printed cage placement was impossible. FOSA was negative in Group A and positive in Group B, significantly larger in females compared to males in both groups and significantly larger postoperatively in Group A. Conclusion: FOSA can objectively measure the feasibility of cage placement when the patient is positioned prone per preoperative flexion O-C2a. A FOSA >34o is contraindication for cage placement.

Cranial vault suspension for basilar invagination in patients with open cranial sutures: technique and long-term follow-up. Illustrative case.

BACKGROUND: Hajdu-Cheney syndrome (HCS) is an extremely rare genetic disorder characterized by severe osteoporosis, scoliosis, and persistent open cranial sutures (POCSs). Neurological complications include hydrocephalus, Chiari I malformations, and basilar invagination (BI). Surgical intervention in HCS is challenging due to severe osteoporosis, ligamentous laxity, POCSs, and extreme skeletal deformities. Herein, the authors present a case of BI repair in a patient with HCS and POCSs, requiring a novel technique of cranial vault suspension, with long-term follow-up. OBSERVATIONS: A 20-year-old female with HCS and progressive symptomatic BI, initially managed with posterior fossa decompression and occipital to cervical fusion, subsequently required cranial vault expansion due to symptomatic shifting of her cranium secondary to POCS. This custom construct provided long-term stabilization and neurological improvement over a follow-up duration of 9.5 years. A literature review performed revealed three other cases of surgical intervention for BI in patients with HCS and clinicopathological characteristics of each case was compared to the present illustrative case. LESSONS: POCSs in patients with BI complicate traditional surgical approaches, necessitating more invasive techniques to secure all mobile cranial parts for optimal outcomes. Using this cranial vault suspension and fusion technique results in lasting neurological improvement and construct stability.

CCDC183 is essential for cytoplasmic invagination around the flagellum during spermiogenesis and male fertility.

Sperm flagellum plays a crucial role in male fertility. Here, we generated Ccdc183 knockout mice using the CRISPR/Cas9 system to reveal the protein function of the testis-specific protein CCDC183 in spermiogenesis. We demonstrated that the absence of CCDC183 causes male infertility with morphological and motility defects in spermatozoa. Owing to the lack of CCDC183, centrioles after elongation of axonemal microtubules do not connect the cell surface and nucleus during spermiogenesis, which causes subsequent loss of cytoplasmic invagination around the flagellum. As a result, the flagellar compartment does not form properly and cytosol-exposed axonemal microtubules collapse during spermiogenesis. In addition, ectopic localization of accessory structures, such as the fibrous sheath and outer dense fibers, and abnormal head shape as a result of abnormal sculpting by the manchette are observed in Ccdc183 knockout spermatids. Our results indicate that CCDC183 plays an essential role in cytoplasmic invagination around the flagellum to form functional spermatozoa during spermiogenesis.

Basilar Invagination With Chiari Type I Malformation and Atlanto-Axial Instability: A Rare Case Report.

Basilar invagination (BI) and Chiari malformation type I (CM-I) are important anomalies involving the craniovertebral junction (CVJ) involving the skull base and occipitocervical region. The incidence of BI is rare involving < 1% of the general population worldwide. They present with varied and complex clinical-radiological features. We present a 36-year-old male who displayed complaints of persistent reeling sensation at our center. Clinical examination revealed bilateral cerebellar signs along with nystagmus and restricted neck movements. Imaging revealed evidence of BI with cerebellar tonsil herniation of ~14.7 mm. Atlantodens interval of 6 mm was noted. The unexpected findings of C1-C2 fusion and instability were also noted. We describe a rare case of BI with C1 prolapse into the foramen magnum along with CM-1 malformation and congenital fusion of C1-C2. We conclude that the treatment algorithm for these rare cases is not very well established and is individually dependent.

Chiari Type 1 Malformation and Syringomyelia in Children: Classification and Treatment Options.

Chiari type 1 malformation (CIM) is defined as tonsillar ectopia of >5 mm, while syringomyelia (SM) is defined as a cerebrospinal fluid (CSF)-filled cavity larger than 3 mm dissecting the spinal cord. Over the last decades, our understanding of these pathologies has grown; however, many controversies still exist almost in every aspect of CIM and SM, including etiology, indication for treatment, timing of treatment, surgical technique, follow-up regime, and outcome. This chapter provides a comprehensive overview on different aspects of CIM and SM and on the still existing controversies, based on the evidence presently available. Future directions for clinical research concerning CIM and SM treatment and outcome are elaborated and discussed as well.

Hierarchical morphogenesis of swallowtail butterfly wing scale nanostructures.

The study of color patterns in the animal integument is a fundamental question in biology, with many lepidopteran species being exemplary models in this endeavor due to their relative simplicity and elegance. While significant advances have been made in unraveling the cellular and molecular basis of lepidopteran pigmentary coloration, the morphogenesis of wing scale nanostructures involved in structural color production is not well understood. Contemporary research on this topic largely focuses on a few nymphalid model taxa (e.g., Bicyclus, Heliconius), despite an overwhelming diversity in the hierarchical nanostructural organization of lepidopteran wing scales. Here, we present a time-resolved, comparative developmental study of hierarchical scale nanostructures in Parides eurimedes and five other papilionid species. Our results uphold the putative conserved role of F-actin bundles in acting as spacers between developing ridges, as previously documented in several nymphalid species. Interestingly, while ridges are developing in P. eurimedes, plasma membrane manifests irregular mesh-like crossribs characteristic of Papilionidae, which delineate the accretion of cuticle into rows of planar disks in between ridges. Once the ridges have grown, disintegrating F-actin bundles appear to reorganize into a network that supports the invagination of plasma membrane underlying the disks, subsequently forming an extruded honeycomb lattice. Our results uncover a previously undocumented role for F-actin in the morphogenesis of complex wing scale nanostructures, likely specific to Papilionidae.

Characteristics and evaluation of C1 posterior arch variation for transpedicular screw placement between patients with and without basilar invagination.

BACKGROUND: C1 transpedicular screw (C1TS) placement provided satisfactory pullout resistance and 3D stability, but its application might be limited in patients with basilar invagination (BI) due to the high incidences of the atlas anomaly and vertebral artery (VA) variation. However, no study has explored the classifications of C1 posterior arch variations and investigated their indications and ideal insertion trajectories for C1TS in BI. PURPOSE: To investigate the bony and surrounding arterial characteristics of the atlas, classify posterior arch variations, identify indications for C1TS, evaluate ideal insertion trajectories for C1TS in BI patients without atlas occipitalization (AO), and compare them with those without BI and AO as control. METHODS: A total of 130 non-AO patients with and without BI (52 patients and 78 patients, respectively) from two medical centers were included at a 1:1.5 ratio. The posterior arch variations were assessed using a modified C1 morphological classification. Comparisons regarding the bony and surrounding arterial characteristics, morphological classification distributions, and ideal insertion trajectories between BI and control groups were performed. The subgroup analyses based on different morphological classifications were also conducted. In addition, the factors possibly affecting the insertion parameters were investigated using multiple linear regression analyses. RESULTS: The BI group was associated with significantly smaller lateral mass height and width, sagittal length of posterior arch, pedicle height, vertical height of posterior arch, and distance between VA and VA groove (VAG) than control group. Four types of posterior arch variations with indications for different screw placement techniques were classified; Classifications I and II were suitable for C1TS. The BI cohort showed a significantly lower rate of Classification I than the control cohort. In the BI group, the subgroup of Classification I had significantly larger distance between the insertion point (IP) and inferior aspect of the posterior arch. In addition, it had the narrowest width along ideal screw trajectory, but a significantly more lateral ideal mediolateral angle than the subgroup of Classification II. Multiple linear regression indicated that the cephalad angle was significantly associated with the diagnosis of BI (B = 3.708, P < 0.001) and sagittal diameter of C1 (B = 3.417, P = 0.027); the ideal mediolateral angle was significantly associated with BMI (B = 0.264, P = 0.031), sagittal diameter of C1 (B = - 4.559, P = 0.002), and pedicle height (B = - 2.317, P < 0.001); the distance between the IP and inferior aspects of posterior arch was significantly associated with age (B = - 0.002, P = 0.035), BMI (B = - 0.007, P = 0.028), sagittal length of posterior arch (B = - 0.187, P = 0.032), pedicle height (B = - 0.392, P < 0.001), and middle and lower parts of posterior arch (B = 0.862, P < 0.001). CONCLUSION: The incidence of posterior arch variation in BI patients without AO was remarkably higher than that in control patients. The insertion parameters of posterior screws were different between the morphological classification types in BI and control groups. The distance between VA V3 segments and VAG in BI cohort was substantially smaller than that in control cohort. Preoperative individual 3D computed tomography (CT), CT angiography and intraoperative navigation are recommended for BI patients receiving posterior screw placement.

A Rare Case of Obstructive Uropathy in an Elderly Male From Rural India - A Case Report.

Ureterocele is a rare congenital anomaly often believed to be caused due to incomplete dissolution of the Chwalla membrane. In this pathology, the distal end of the ureter is invaginated in the bladder and is dilated. We present a case of an 81-year-old male from rural India who came with complaints of hematuria and was diagnosed with ureterocele.