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To assess volumetric changes in the spinal cord at the cervicomedullary junction, diameter of the cervicomedullary cord, and width of the brainstem following posterior fossa decompression (PFD). A retrospective analysis of adult patients with Chiari malformation who underwent PFD was performed. Segmentations were done on clinical quality T2-weighted cervical magnetic resonance images obtained before and after decompression using ITK-SNAP. Volumes of neural tissue within the cervicomedullary junction were evaluated from 10 mm cranial to the medullary beak to the cervical spinal cord at the level of the caudal endplate of the second cervical vertebra. The diameter of the cervicomedullary cord was calculated perpendicular to the spinal cord. The width of the brainstem was measured perpendicular to the clivus at the level of the basion. Twenty adult patients, a mean age of 49.55 years, were included. The cervical cord increased in volume by 13 mm3 to 338 mm3, with an average increase of 155 mm3 (P -value of 0.00002). The diameter of the cervicomedullary cord increased 10.30% 7 mm superior to the beak (P -value of 0.00074), 11.49% at the apex of the beak (P -value of 0.00082), 8.29% 7 mm inferior to the beak (P -value of 0.00075), and the brainstem increased 14.46% perpendicular to the clivus (P -value of 0.00109). The spinal cord at the inferior aspect of the C3 vertebra changed insignificantly (P -value of 0.10580). The volume of the cervical cord at the cervical-medullary junction, width of the cervicomedullary cord, and diameter of the brainstem increase following PFD. [ABSTRACT FROM AUTHOR]

Hemangioblastoma is a rare, benign, highly vascularized tumour, that usually presents a muriform nodule. The most frequent localization is the posterior cranial fossa. The frequency of this spinal lesion is very low, representing less than 5% of spinal cord tumours. The presentation of hemangioblastoma can widely vary, from a solid tumour to a lesion with a cystic component. We present the case of a 43 years old patient, admitted to our clinic for left hemiparesis, swallowing disorders, and dysphonia. An MRI is performed and it showed a cervicomedullary junction tumour with a solid nodule and an anterior cystic cavity pushing towards the 4th ventricle. The patient underwent surgery for the total removal of the tumour. Post-operative CT confirmed the total ablation of the tumour. The patient is discharged with improved symptomatology. In the last 10 years, in our clinic were admitted 23 cases of hemangioblastoma, 14 males (61% of the patients) and 9 females (39%) with an age at presentation varied from 14 to 78 years (mean 48,2 years). Only 3 patients (13%) out of the 23 had associated von Hippel-Lindau syndrome, with hemangioblastomas also present in other locations. The most common location was the posterior fossa, in 13 cases (56,6%). Headache (69,5%), ataxia (56%) and balance disorders (52%) were the most common symptoms accused by patients. The followup varied from 6 to 84 months (mean 20 months). Only one patient died during hospitalization and one tumour recurrence was noted. Most patients improved or remained clinically stable postoperatively. [ABSTRACT FROM AUTHOR]

Pilocytic astrocytomas are well-circumscribed, predominantly cystic mass lesions that have a discrete mural nodule. Giant, solid pilocytic astrocytoma is uncommon. Its characteristic imaging features are described.

Hemangioblastoma is a rare, benign, highly vascularized tumour, that usually presents a muriform nodule. The most frequent localization is the posterior cranial fossa. The frequency of this spinal lesion is very low, representing less than 5% of spinal cord tumours. The presentation of hemangioblastoma can widely vary, from a solid tumour to a lesion with a cystic component. We present the case of a 43 years old patient, admitted to our clinic for left hemiparesis, swallowing disorders, and dysphonia. An MRI is performed and it showed a cervicomedullary junction tumour with a solid nodule and an anterior cystic cavity pushing towards the 4th ventricle. The patient underwent surgery for the total removal of the tumour. Post-operative CT confirmed the total ablation of the tumour. The patient is discharged with improved symptomatology. In the last 10 years, in our clinic were admitted 23 cases of hemangioblastoma, 14 males (61% of the patients) and 9 females (39%) with an age at presentation varied from 14 to 78 years (mean 48,2 years). Only 3 patients (13%) out of the 23 had associated von Hippel–Lindau syndrome, with hemangioblastomas also present in other locations. The most common location was the posterior fossa, in 13 cases (56,6%). Headache (69,5%), ataxia (56%) and balance disorders (52%) were the most common symptoms accused by patients. The follow-up varied from 6 to 84 months (mean 20 months). Only one patient died during hospitalization and one tumour recurrence was noted. Most patients improved or remained clinically stable postoperatively.

Background: Cervicomedullary glioblastoma is an extremely rare clinical entity and the principles of its management are not well understood. Case Description: We report two cases of cervicomedullary glioblastoma in young patients aged 12 and 30 years with contrasting clinical presentation and outcomes. The 12-year-old child had rapid onset bulbar symptoms, with frank infiltration of the medulla due to which the patient succumbed within 4 weeks of surgery. The 30-year-old adult had a relatively slow disease onset and progression and made a good neurological recovery without disease progression at 16 months after surgery. To the best of our knowledge, we also report only the second adult patient in the literature with a dorsally exophytic cervicomedullary glioblastoma. Difficulties in diagnosis and management are discussed with a review of the pertinent literature. Conclusion: The overall outcome depends on the rapid progression and severity of preoperative symptoms and the degree of tumor infiltration noted in imaging and during surgery. [ABSTRACT FROM AUTHOR]

Neurenteric cysts are rare congenital lesions of benign nature that can be encountered at any level of the neuraxis, starting from the cranium down to coccyx. Rewarding outcome can be achieved with early diagnosis and complete removal of these benign lesions. Here, we report a case of a huge neurenteric cyst in an 11‑year‑old boy at the ventral craniocervical junction, a rarely reported entity with literature review. In this article, we focus on the clinical presentation, pathogenesis, radiological findings, surgery, and surgical outcome of this benign lesion, as we succeeded to have gratifying result following surgery in our instance. [ABSTRACT FROM AUTHOR]

Ependymoma is a rare tumor central nervous system that arises from the ependymal lining of the ventricles or the central canal of the spinal cord. They are of neuroectodermal in origin and constitute about 30%–86% of tumors arising in the spinal cord. The occurrence of these tumors in the cervicomedullary region is very rare. Sudden symptomatic neurologic presentations due to hemorrhage in cervicomedullary ependymoma is very rare and so far have never been reported. Mostly presenting as neurologic deficits involving limbs, these tumors pose a technical challenge in their removal. We present a patient who presented with sudden-onset dysesthesia of the upper and lower limbs. On imaging, he was found to have a cystic medullary tumor extending to the cervical region with hemorrhage. We discuss the epidemiology, surgical challenges, and outcome along with review of literature of these rare tumors located in this precarious location. [ABSTRACT FROM AUTHOR]

Background: Intramedullary primitive neuroectodermal tumors (PNETs) are tumors found rarely in the cervical region, with only five such cases described in the literature. The available literature contains only one report regarding cervicomedullary junction PNET. Case Description: The authors present a case report of a 45-year-old male patient who had undergone urgent hospitalization owing to progressive tetraparesis and subtle impairment of respiratory function. He underwent magnetic resonance imaging, which showed an extensive enhancing cervical intramedullary tumor extending from C5 to the bulbar region. Since he developed severe impairment of respiratory function, he required tracheostomy. He then underwent microsurgery 2 days after his admission, and a partial tumor resection was performed. The pathological diagnosis of PNET of the cervicomedullary junction (CMJ) was made. He had slight worsening of strength after surgery with subsequent deterioration over the next 3 weeks. The tumor displayed aggressive growth; thus, radiotherapy was indicated. Unfortunately, he developed severe febrile neutropenia and died after 2 weeks of radiotherapy. Given the rarity of the condition, we wish to review the epidemiology, pathophysiology, and treatment options of his population. Conclusion: Intramedullary PNETs of the cervical spine and CMJ are exceedingly rare in adults; treatment of such patients remains a challenge, despite the modern neurosurgical armamentarium that is available. [ABSTRACT FROM AUTHOR]

Pilocytic astrocytomas are well-circumscribed, predominantly cystic mass lesions that have a discrete mural nodule. Giant, solid pilocytic astrocytoma is uncommon. Its characteristic imaging features are described. [ABSTRACT FROM AUTHOR]

Tumors arising in the brainstem comprise 10–20% of all pediatric central nervous system (CNS) tumors and account for a small percentage in adults. The prognosis for these tumors was considered uniformly poor prior to the era of modern neuroimaging and the location was fraught with disaster being considered a ‘no man’s land’ for neurosurgeons. Following the introduction of advanced imaging modalities and neurophysiological monitoring, striking progress has occurred in the management of these lesions. Brainstem tumors are presently classified based on their anatomic location, focality, and histopathology. This article reviews the current classification of brainstem tumors, current management options, and future directions in the treatment for these rare tumors.

Canine atlantooccipital overlapping (AOO) is characterized by a decreased distance between the dorsal arch of the atlas and the supraoccipital bone. Current diagnostic criteria for this condition are subjective and clinician-dependent; objective criteria are needed to allow a reliable and reproducible diagnosis in clinical and research settings and assess clinical significance. We propose four standardized MRI-based measurements to objectively assess the proximity of the atlas to the foramen magnum. Inclusion criteria for dogs in this study were bodyweight <15 kg, age >5 months, and availability of a complete MRI study performed with the craniocervical junction in extension. Exclusion criteria were space-occupying lesions and poor image quality (i.e. unclear bony margins). Measurements also included blinded determinations of skull type, presence of craniocervical junction anomalies and presence and severity of syringomyelia. Clinical status at the time of imaging was noted. Measurements were obtained in 271 dogs; these were reproducible and reliable. Findings varied by skull type: dolichocephalic dogs had smaller foramen magnum, whereas brachycephalic dogs had more cranially and dorsally positioned atlas bodies in comparison to the other skull types. Measurements also increased with increases in bodyweight. This study demonstrated a close association between AOO, syringomyelia and clinical signs. Toy and small breed dogs (including Cavalier King Charles spaniels) showed higher than previously reported prevalence of AOO; its occurrence was also associated with lower bodyweights within the study population of <15 kg toy/small breed dogs. [ABSTRACT FROM AUTHOR]

Background We reviewed the current literature pertaining to extended endoscopic endonasal approaches to the craniovertebral junction. Methods A systematic literature review was utilized to identify published surgical cases of endoscopic endonasal approaches to the craniovertebral junction. Full-text manuscripts were examined for various measures of surgical indications, patient characteristics, operative technique, and surgical outcomes. Results We identified 71 cases involving endoscopic endonasal approaches for surgical management of a variety of pathologies located within the craniovertebral junction. Patient ages ranged from 3 to 87 years, with 40 females and 31 males. Five patients required tracheostomy, two were reintubated, and all others experienced an average intubation duration of 0.54 days following surgery. Fifty-eight patients (81.7%) underwent an additional posterior decompression or fusion either before or after the endonasal procedure. A complete resection of the pathologic lesion was reported in 57 cases (83.8%), another five were successful biopsies, and four resulted in partial resection. The follow-up time ranged from 0.5 to 57 months. Conclusion Although the transoral approach has been the standard for anterior surgical management for the past several decades, our systematic review illustrates that the extended endoscopic endonasal approach is a safe and effective alternative for most pathologies affecting the craniovertebral junction. [ABSTRACT FROM AUTHOR]

Background Medullary elevation (ie, medullary kinking) at the craniocervical junction ( CCJ) is reported in dogs with Chiari-like malformations ( CM), but its diagnostic criteria and clinical relevance are unclear. Objective To describe the position of the medulla at the CCJ in mature cavalier King Charles spaniels ( CKCS), and evaluate its relationship with clinical status and the presence of syringomyelia. Animals Thirty-six CKCS, 5-12 years of age, including 16 asymptomatic dogs. Methods Dogs were assigned a neurologic grade; magnetic resonance imaging ( MRI) of the CCJ then was performed. The presence of a CM and syringomyelia was recorded and syringomyelia severity was quantified. Medullary position was quantified using the medullary kinking index, the elevation angle and obex position relative to the foramen magnum. The relationship between medullary position measures and presence and severity of neurologic signs and syringomyelia was investigated. Results Chiari-like malformation was found in 33 dogs; 26 of them had syringomyelia. Mean medullary kinking index was 46.4% ( SD, 10.3), elevation angle was 132° ( SD, 12) and obex position was 3.5 mm ( SD, 0.8). A higher medullary kinking index was associated with the presence of neurologic signs ( P = .0368). Obex position was associated with the presence ( P = .0018) and severity of syringomyelia ( P = .0164). Conclusions and clinical importance There is a significant association between medullary elevation and clinical signs, whereas more caudal brainstem positions appear related to the presence of syringomyelia. [ABSTRACT FROM AUTHOR]

Background: A small subset of patients with adult Chiari I malformation without basilar invagination (BI) and instability show ventral cervicomedullary distortion/compression and have symptoms pertaining to that. The cause of this ventral compression remains speculative. Additionally, it is unclear if these patients would require ventral decompression with posterior fusion or only posterior decompression would suffice. Methods: Sixteen adult patients with Chiari I malformation with significant ventral cervicomedullary compression, in the absence of BI, were included in the study. Atlantoaxial dislocation (AAD) was excluded in these patients by flexion-extension craniovertebral junction X-rays and computed tomography (CT). Their clinical profile, especially symptoms pertaining to cervicomedullary compression, i.e. dysphagia, dysarthria and spasticity, were graded. The ventral cervicomedullary compression (VCMC) was quantified using pBC2 (maximum perpendicular distance to the basion-infero posterior point of the C2 body) on sagittal magnetic resonance imaging (MRI) and only those patients with pBC2 ≥9 mm were included. Furthermore, retroversion of dens and retro odonotid tissue thickness was calculated in each patient. Fifteen patients underwent posterior decompression alone and one refused surgery. Follow-up was done every 3 months. Repeat MRI was done at 1 year following surgery to look for pBC2. Results: The mean pBC2 was 11 ± 0.2 mm. Retroversion of dens was responsible for VCMC in three patients and periodontoid crown in 13. There was no correlation between the tonsillar descent, age and the pBC2. All patients improved in symptoms of cervicomedullary compression following surgery. One patient worsened 6 months after initial improvement. The pBC2 did not change, as seen on follow-up MRI done in five patients. Conclusions: VCMC in adult patients with Chiari I malformation in the absence of BI and/or AAD is due to periodontoid tissue (crown) or retroverted dens. Though a long-term study is required, it appears that all patients with Chiari I malformation, irrespective of the VCMC, can be given a chance with posterior decompression alone. Transoral decompression with posterior fusion may be required in a small subset of patients who fail to improve or worsen following posterior decompression only. [ABSTRACT FROM AUTHOR]

Objectives  Dural arteriovenous fistulas (DAVFs) at the cervicomedullary junction are uncommon and often accompanied by subarachnoid hemorrhage (SAH). We aim to illustrate in detail the microsurgical procedure for treating a DAVF located at the cervicomedullary junction. Design  We present a two-dimensional operative video that includes clinical history, preoperative imaging, surgical strategy, still images with labels, clinical course, and postoperative imaging. Setting  The microsurgery was performed at an academic medical center. Participant  The patient is a 55-year-old female who presented with SAH, acute onset headache, nausea, and vomiting. Angiography demonstrated right vertebral artery vasospasm and a persistent arteriovenous shunt at the cervicomedullary junction supplied by small perforating arteries of the right vertebrobasilar junction (Fig. 1). Main Outcome Measures  The patient was placed in the park-bench position with the head turned to the contralateral side. A hockey stick incision was made, followed by a right-side far-lateral transcondylar approach. Indocynanine green videoangiography was performed to help identify the areas of arteriovenous shunting. Multiple clips were placed to interrupt vessels that corresponded to arterial feeders at the level of the C1 and C2 nerve root sleeves (Fig. 2). The dura was closed in a water tight fashion and the posterior fossa was reconstructed with a titanium mesh. Results  Postoperative imaging showed no evidence of continued arteriovenous shunting. The patient was discharged in good clinical condition with an uneventful postoperative course. Conclusion  A deep understanding of the microsurgical vascular anatomy is necessary for successful occlusion of a cervicomedullary DAVF. The link to the video can be found at: https://youtu.be/-LfOcNB05BY. [ABSTRACT FROM AUTHOR]

Pilocytic astrocytoma (PA) is a rare glioma, which generally occurs in children and young adults. In adult patients, the majority of PA tumors are supratentorial. Due to the low morbidity rate of the disease in adults, PA is frequently misdiagnosed and mistreated. In the present study, this rare disease was successfully treated. The study reported the case of a 48-year-old patient with a cervicomedullary occupying lesion, who complained of numbness and pain of the right limbs that persisted for >10 years, with aggravation for 1 month. A magnetic resonance imaging scan showed a sharp cervicomedullary mass extending from the lower medulla to the cervical vertebra C3 level. Intraoperatively, the medulla and upper cervical cord were found to be well-stacked. Immediately after ingression into the spinal cord along the dorsal median sulcus, the tumor mass was detected and had a gray fish-like appearance, moderate blood supply and clear boundary. After intratumoral decompression, total excision was achieved. Postoperative pathological examination confirmed that the tumor was a PA. Following discharge, the patient did not suffer from any symptoms of the lower cranial nerves and was able to walk with limited assistance. [ABSTRACT FROM AUTHOR]

Solid cervicomedullary haemangioblastomas represent a small but technically challenging subset of posterior fossa haemangioblastomas because of their site, high vascularity and tendency to bleed. We present our experience with preoperative embolisation of two solid cervicomedullary haemangioblastomas. In both cases the main feeding artery was the posterior inferior cerebellar artery. Arterial pedicles were catheterised with a microcatheter. The embolic material was small particles (150–250 μm) of polyvinyl alcohol. The result was almost complete preoperative obliteration of the vessels of the tumours. The procedure was well tolerated and facilitated easier bloodless, successful resection in both cases, 24 h after embolisation. [ABSTRACT FROM AUTHOR]

Dural arteriovenous fistulas (DAVFs) at the cervicomedullary junction are rare and have a wide variation in presentation. We report a case of occipital neuralgia (ON) as a rare presenting symptom of cervicomedullary DAVF causing intramedullary hemorrhage at the C1 level. It is important to consider the underlying causes of ON, and precise neurological examinations and radiological evaluations are needed. [ABSTRACT FROM AUTHOR]

Ependymomas are the most common adult intramedullary spinal tumors. 1 Although uncommon in the brainstem, ependymomas make up a large proportion of tumors of this location. 2-8 We present an operative video case report of an intrinsic ependymoma at the cervicomedullary junction. The purpose of this report is to present the clinical picture, operative setup, and surgical technique involved in resection of an intramedullary tumor of this region. For best outcome for intramedullary ependymomas, the goal should be gross total resection. 1,9,10 These tumors have a relatively distinct plane between tumor and normal parenchyma, making a gross total resection more probable than cases of infiltrative intramedullary astrocytomas. 11 Despite this, significant morbidity can be associated with treatment. 1 Proper microsurgical technique with use of operative adjuncts can maximize resection while minimizing neurologic injury to optimize outcomes in patients. We present the case of a 42-year-old man presenting with neck and shoulder pain, upper extremity paresthesias, and gait instability. Magnetic resonance imaging of the neuroaxis revealed a heterogeneously enhancing expansile lesion in the lower medulla and multiple lesions in the thecal sac, representing drop metastases. Due to symptoms and mass effect from the lesion, the patient underwent a suboccipital craniotomy for tumor resection. We highlight operative techniques in our case, including use of neurophysiologic monitoring, intraoperative ultrasound, ultrasonic aspirator, and dissection with microcottonoid pledgets and bimanual technique. Pathology revealed a World Health Organization grade II ependymoma. Postoperative magnetic resonance imaging revealed a small amount of residual. He underwent full craniospinal proton beam therapy with boost to the residual and metastases with good outcome. Patient provided consent for this report. [ABSTRACT FROM AUTHOR]

A 49-year-old woman presented with sudden-onset headache and meningismus. Computed tomography scan revealed a posterior fossa subarachnoid hemorrhage extending from the foramen magnum to the ambient cistern. Angiography showed a pial extramedullary arteriovenous malformation (AVM) at the lateral ventral surface of the cervicomedullary junction with primary supply from the left posterior inferior cerebellar artery (PICA) and dominant drainage into the anterior median perimedullary vein. Embolization of the AVM was considered a high-risk procedure, as feeding arteries originated from the proximal lateral medullary segment of the left PICA. A far lateral approach with suboccipital craniotomy and C1-C2 laminectomy was performed (Video 1). The PICA was disconnected from the AVM, and multiple small feeders were interrupted with bipolar cautery and aneurysm clips to achieve an in situ occlusion of the pial AVM. Intraoperative indocyanine green video angiography was used to define the AVM and critical en passant vessels before disconnection and to demonstrate no residual early venous filling after the in situ occlusion. Postoperative angiography demonstrated no residual arteriovenous shunting or nidiform vessels, with preservation of patency of the left PICA. The patient had an uneventful postoperative course and was discharged with no significant neurologic deficits. [ABSTRACT FROM AUTHOR]

Study Design: A study of a series of consecutive full-endoscopic uniportal decompressions of the anterior craniocervical junction with retropharyngeal approach.Objective: The aim of this study was to evaluate the direct anterior decompression of the craniocervical junction in patients with bulbomedullary compression using a full-endoscopic uniportal technique via an anterolateral retropharyngeal approach.Summary Of Background Data: Acute or progressive myelopathy may necessitate direct anterior decompression of the craniocervical junction and odontoidectomy. Different techniques with individual advantages and disadvantages can be used. In addition to the gold standard - the transoral approach - there is increasing experience with the endoscopic transnasal technique. Other alternative procedures are also being developed.Methods: Between 2013 and 2016, eight patients with basilar impression, retrodental pannus, or retrodental infection were operated in the full-endoscopic uniportal technique with a retropharyngeal approach. Anterior decompression of the bulbomedullary junction with odontoidectomy was performed. All patients additionally underwent posterior stabilization. Imaging and clinical data were collected in follow-up examinations for 1 year.Results: The bulbomedullary junction was adequately decompressed. No problems due to swelling of pharyngeal soft tissue occurred. One patient required revision due to secondary bleeding. No other complications were observed. All patients had a good clinical outcome with stable regression of the myelopathy symptoms and/or healing of the infection. The imaging follow-up showed sufficient decompression of bone and soft tissue in all cases. No evidence was found of increasing instability or failure of posterior fusion.Conclusion: In the operated patients, the full-endoscopic uniportal surgical technique with anterior retropharyngeal approach was found to be a sufficient and minimally invasive method with the known advantages of an endoscopic procedure under continuous irrigation. It should not be viewed only as competition for other surgical techniques - due to its individual technical parameters, it can also be considered to be an alternative or complementary procedure.Level Of Evidence: 4. [ABSTRACT FROM AUTHOR]