Volumetric segmentation in the context of posterior fossa-related pathologies: a systematic review.

BACKGROUND: Segmentation tools continue to advance, evolving from manual contouring to deep learning. Researchers have utilized segmentation to study a myriad of posterior fossa-related conditions, such as Chiari malformation, trigeminal neuralgia, post-operative pediatric cerebellar mutism syndrome, and Crouzon syndrome. Herein, we present a summary of the current literature on segmentation of the posterior fossa. The review highlights the various segmentation techniques, and their respective strengths and weaknesses, employed along with objectives and outcomes of the various studies reported in the literature. METHODS: A literature search was conducted in PubMed, Embase, Cochrane, and Web of Science up to November 2023 for articles on segmentation techniques of posterior fossa. The two senior authors searched through databases based on the keywords of the article separately and then enrolled joint articles that met the inclusion and exclusion criteria. RESULTS: The initial search identified 2205 articles. After applying inclusion and exclusion criteria, 77 articles were selected for full-text review after screening of titles/abstracts. 52 articles were ultimately included in the review. Segmentation techniques included manual, semi-automated, and fully automated (atlas-based, convolutional neural networks). The most common pathology investigated was Chiari malformation. CONCLUSIONS: Various forms of segmentation techniques have been used to assess posterior fossa volumes/pathologies and each has its advantages and disadvantages. We discuss these nuances and summarize the current state of literature in the context of posterior fossa-associated pathologies.

The role of surgical disconnection for posterior fossa pial arteriovenous fistulas and dural fistulas with pial supply: an illustrative case series.

BACKGROUND: Pial arteriovenous fistulas (pAVFs) are rare vascular malformations characterized by high-flow arteriovenous shunting involving a cortical arterial supply directly connecting to venous drainage without an intermediate nidus. Dural arteriovenous fistulas (dAVFs) can infrequently involve additional pial feeders which can introduce higher flow shunting and increase the associated treatment risk. In the posterior fossa, arteriovenous fistula (AVF) angioarchitecture tends to be particularly complex, involving either multiple arterial feeders-sometimes from both dural and pial origins-or small caliber vessels that are difficult to catheterize and tend to be intimately involved with functionally critical brainstem or upper cervical cord structures. Given their rarity, published experience on microsurgical or endovascular treatment strategies for posterior fossa pAVFs and dAVFs with pial supply remains limited. METHODS: Retrospective chart review from 2019-2023 at a high-volume center identified six adult patients with posterior fossa pAVFs that were unable to be fully treated endovascularly and required microsurgical disconnection. These cases are individually presented with a technical emphasis and supported by comprehensive angiographic and intraoperative images. RESULTS: One vermian (Case 1), three cerebellopontine angle (Cases 2-4) and two craniovertebral junction (Cases 5-6) posterior fossa pAVFs or dAVFs with pial supply are presented. Three cases involved mixed dural and pial arterial supply (Cases 1, 4, and 6), and one case involved a concomitant microAVM (Case 2). Endovascular embolization was attempted in four cases (Cases 1-4): The small caliber and tortuosity of the main arterial feeder prevented catheterization in two cases (Cases 1 and 3). Partial embolization was achieved in Cases 2 and 4. In Cases 5 and 6, involvement of the lateral spinal artery or anterior spinal artery created a prohibitive risk for endovascular embolization, and surgical clip ligation was pursued as primary management. In all cases, microsurgical disconnection resulted in complete fistula obliteration without evidence of recurrence on follow-up imaging (mean follow-up 27.1 months). Two patients experienced persistent post-treatment sensory deficits without significant functional limitation. CONCLUSIONS: This illustrative case series highlights the technical difficulties and anatomical limitations of endovascular management for posterior fossa pAVFs and dAVFs with pial supply and emphasizes the relative safety and utility of microsurgical disconnection in this context. A combined approach involving partial preoperative embolization-when the angioarchitecture is permissive-can potentially decrease surgical morbidity. Larger studies are warranted to better define the role for multimodal intervention and to assess associated long-term AVF obliteration rates in the setting of pial arterial involvement.

[Endoscopic transnasal resection of clival meningiomas]. / Rezul'taty endoskopicheskogo transnazal'nogo udaleniya meningiom oblasti skata.

BACKGROUND: Surgical treatment of ventral and ventrolateral meningiomas of posterior cranial fossa is difficult in modern neurosurgery. This is due to peculiarities of approach to these areas and concentration of critical structures (cranial nerves and great vessels). Currently, endoscopic transnasal approach to these meningiomas allows partial, and in some cases, total resection. However, this technique is not widespread. OBJECTIVE: To analyze the world literature data on postoperative outcomes in patients with clival and petroclival meningiomas after endoscopic transnasal resection. MATERIAL AND METHODS: We analyzed 22 articles representing treatment of 61 patients with clival and petroclival meningiomas. RESULTS: Total or near-total resection was achieved in 22.9% of cases, subtotal resection - 40.9%, partial resection - 26.2% (data were not provided in other cases). Even partial and subtotal resection leads to significant regression of symptoms. CONCLUSION: Endoscopic transnasal surgery is a full-fledged alternative to transcranial approaches in surgical treatment of clival meningiomas. It is also an additional option for patients with petroclival meningiomas after ineffective transcranial approaches. Transnasal tumor shrinkage and devascularization lead to brainstem decompression, regression of hydrocephalus and baseline clinical symptoms.

Step-by-Step Dissection of the Extreme Lateral Transodontoid Approach to the Anterior Craniovertebral Junction: Surgical Anatomy and Technical Nuances.

BACKGROUND: Multicompartmental lesions of the anterior craniovertebral junction require aggressive management. However, the lesions can be difficult to reach, and the surgical procedure is difficult to understand. The aim of this study was to create a procedural, stepwise microsurgical educational resource for junior trainees to learn the surgical anatomy of the extreme lateral transodontoid approach (ELTOA). METHODS: Ten formalin-fixed, latex-injected cadaveric heads were dissected under an operative microscope. Dissections were performed under the supervision of a skull base fellowship-trained neurosurgeon who has advanced skull base experience. Key steps of the procedure were documented with a professional camera and a high-definition video system. A relevant clinical case example was reviewed to highlight the principles of the selected approach and its application. The clinical case example also describes a rare complication: a pseudoaneurysm of the vertebral artery. RESULTS: Key steps of the ELTOA include patient positioning, skin incision, superficial and deep muscle dissection, vertebral artery dissection and transposition, craniotomy, clivus drilling, odontoidectomy, and final extradural and intradural exposure. CONCLUSIONS: The ELTOA is a challenging approach, but it allows for significant access to the anterior craniovertebral junction, which increases the likelihood of gross total lesion resection. Given the complexity of the approach, substantial training in the dissection laboratory is required to develop the necessary anatomic knowledge and to minimize approach-related morbidity.

Endoscopic Endonasal Far-Medial Approach: 2-Dimensional Operative Video.

INDICATIONS CORRIDOR AND LIMITS OF EXPOSURE: Endoscopic endonasal far-medial approach provides an effective and safe corridor to access the parasagittal structures of the lower clivus such as the medial jugular tubercle (JT) and occipital condyle (OC) for lesions that displace neurovascular structures laterally. ANATOMIC ESSENTIALS NEED FOR PREOPERATIVE PLANNING AND ASSESSMENT: Parapharyngeal internal carotid arteries (ICAs) run posterolateral to the eustachian tubes and lateral to the OC. The supracondylar groove is a superficial landmark for the hypoglossal canal, which divides the lateral extension of clivus into the JT and OC. ESSENTIAL STEPS OF THE PROCEDURE: Typically, approach starts with opening of the sphenoid sinus to localize the paraclival ICA. An "inverted U" rhinopharyngeal (RP) flap exposing the supracondylar groove and lower clivus. Doppler and navigation can confirm the course of the ICA. Drilling is started in the midline in the lower clivus and extended laterally to expose the hypoglossal canal, JT, and OC. PITFALLS/AVOIDANCE OF COMPLICATIONS: Neurovascular injuries can be avoided by using intraoperative Doppler and nerve stimulator. Multilayer reconstruction with vascularized nasoseptal (NSF) and RP flaps minimize postoperative cerebrospinal fluid leak. VARIANTS AND INDICATIONS FOR THEIR USE: The contralateral transmaxillary approach provides an increased angle of access behind foramen lacerum and the petrous ICA.The endoscopic endonasal far-medial approach can be used for a variety of pathologies, including petroclival or JT meningiomas, chordomas and chondrosarcomas, and hypoglossal schwannomas, inferiorly extending cholesterol granulomas and even rare, ventral posterior inferior cerebellar artery aneurysms.The patients consented to the procedure.

The Submandibular Approach: A Descriptive Perspective of the Retropharingeal Corridor to the Craniocervical Junction (Microscopic- vs. Endoscopic-Assisted Dissections).

The craniocervical junction refers to an area from the line separating the middle and lower third of the clivus to the base of the dens (anteriorly) and from the posterior edge of the occipital foramen to the spinous process of C2 (posteriorly). Here, the clival region is a challenging surgical target surrounded by a complex neurovascular architecture. Historically, mainly the complex, and high-risk, transmucosal approaches have been the corridors of choice when targeting this region. Nevertheless, the inherent broad anatomic and pathological variants have shown the need for more-malleable and wider approaches. Thus, MacAfee's established retropharyngeal approach has been simplified in parallel to the application of endoscopic surgery, therefore providing access to the clival region through a low-risk retropharyngeal space when compared to homologous anterior transmucosal approaches. The following review analyzes the literature that has specifically described the craniocervical junction after reaching the clivus (or at least after odontoidectomy) through the retropharyngeal corridor, from the perspective of the open approach or the endoscopic submandibular approach.

Radical resection of intracavernous chondrosarcoma using an intradural keyhole approach: how I do it.

BACKGROUND: Skull base chondrosarcoma is rare, arising from the clivus or petroclival junction, and usually presents as ocular motility disorders. Endonasal transsphenoidal surgery may be performed in some cases of midline clivus chondrosarcoma. Chondrosarcoma is located within the cavernous sinus and presents a softer/gelatinous mass and can be removed with suctions and curettage. We have been using a simple intradural keyhole transcavernous approach, avoiding a complex extradural transcavernous dissection. METHODS: The intracavernous chondrosarcoma was removed via a 5 mm keyhole opening over the Parkinson's triangle using a standard frontotemporal intradural approach. CONCLUSION: Minimally invasive keyhole surgical resection can be performed to eradicate skull base chondrosarcomas, avoiding complex extradural cranial base approaches.

The Effect of Posterior Fossa Decompression Surgery on Brainstem and Cervical Spinal Cord Dimensions in Adults with Chiari Malformation Type 1.

OBJECTIVE: Posterior fossa decompression (PFD) surgery creates more space at the skull base, reduces the resistance to the cerebrospinal fluid motion, and alters craniocervical biomechanics. In this paper, we retrospectively examined the changes in neural tissue dimensions following PFD surgery on Chiari malformation type 1 adults. METHODS: Measurements were performed on T2-weighted brain magnetic resonance images acquired before and 4 months after surgery. Measurements were conducted for neural tissue volume and spinal cord/brainstem width at 4 different locations; 2 width measurements were made on the brainstem and 2 on the spinal cord in the midsagittal plane. Cerebellar tonsillar position (CTP) was also measured before and after surgery. RESULTS: Twenty-five adult patients, with a mean age of 38.9 ± 8.8 years, were included in the study. The cervical cord volume increased by an average of 2.3 ± 3.3% (P = 0.002). The width at the pontomedullary junction increased by 2.2 ± 3.5% (P < 0.01), while the width 10 mm caudal to this junction increased by 4.2 ± 3.9% (P < 0.0001). The spinal cord width at the base of second cervical vertebra and third cervical vertebra did not significantly change after surgery. The CTP decreased by 60 ± 37% (P < 0.0001) after surgery, but no correlation was found between CTP change and dimension change. CONCLUSIONS: The brainstem width and cervical cord volume showed a modest increase after PFD surgery, although standard deviations were large. A reduction in compression after PFD surgery may allow for an increase in neural tissue dimension. However, clinical relevance is unclear and should be assessed in future studies with high-resolution imaging.

Full-Endoscopic Technique for Posterior Fossa Decompression in Chiari Malformation.

BACKGROUND AND IMPORTANCE: Full-endoscopic techniques are well-described for spinal procedures. Although endoscopic-assisted techniques are reported for posterior fossa decompression (PFD) in Chiari malformation (CM), a full-endoscopic technique is yet to be reported in these patients. The aim of this study was to present and describe a full-endoscopic technique for PFD in patients with CM. CLINICAL PRESENTATION: Two patients diagnosed with CM were operated on by the full-endoscopic PFD technique. The patients consented to the procedure and to the publication of their image. An endoscope with an oval shaft cross-section with a diameter of 9.3 mm, a working length of 177 mm, a viewing angle of 20°, and a working channel of 5.6 diameters were used. Operative videos were recorded. The surgical steps were easily applied after the clear anatomic landmarks, such as the C1 posterior tubercle and the rectus capitis posterior minor muscles. The patients were followed up for 6 months. Both patients were symptom-free with a significant decrease in Visual Analog Scale score and a good functional outcome assessed by Chicago Chiari Outcome Scale after surgery without any complications. CONCLUSION: All the steps of the full-endoscopic technique for PFD described by the authors in their previous human cadaveric study were also feasible on patients with CM.

Axial Convex-Shaped Hematoma was Associated with Poor Curative Effect of Surgical Treatment for Traumatic Posterior Fossa Epidural Hematoma in Children.

BACKGROUND: Posterior fossa epidural hematoma (PFEDH) is rare which accounts for just 4-12.9% of all EDH cases. Since its frequently subtle and nonspecific clinical presentation, CT scan has great importance for early diagnosis and treatment of PFEDH. However, indications for surgery depending on the findings of CT image are still controversial. METHODS: We retrospectively analyzed 40 pediatric cases of PFEDH. Their baseline characteristic, clinical presentation, imaging findings and outcomes were collected and analyzed. The ellipsoid volume equation X × Y × Z/2 was used to measure the hematoma volume. The Glasgow Outcome Scale (GOS) was used to assess the neurologic functional outcome. RESULTS: A total of 40 pediatric PFEH patients were included with 8 patients having poor outcome and 32 patients having a relatively good prognosis. GCS score showed a significant difference between good and poor outcome groups (p < 0.001). Y value on CT image was significantly bigger in poor outcome group than good outcome group (p < 0.01). Similar results were got in X/Z value (p < 0.05) and Y/Z value (p < 0.01) which reflected the shape of hematoma. A predictive model with Y + X/Z showed the largest area under the ROC curve with a sensitivity of 75.0% and specificity of 93.7%. CONCLUSIONS: GCS score at admission was closely related to the prognosis of the pediatric patients with PFEDH. The morphometry of PFEDH has a crucial role in judging the prognosis. Axial convex-shaped hematoma was associated with poor curative effect of surgical treatment.

Operational Analysis of Trautman's Triangle in Petroclival Region Approaches: Cone-Beam Computed Tomography Study.

OBJECTIVE: This study was conducted to evaluate the anatomical and clinical features of Trautman's triangle (TT) and to better understand the possible surgical corridor for other surgical approaches involving the petroclival region, especially the presigmoid retrolabyrinthine approach. METHODS: In this study, morphological analysis of structures related to TT was performed from cone beam computed tomography images of 134 female and 206 male individuals aged 18-65 years. RESULTS: The TT area was observed as 5.6% (n = 19) type I, 63.2% (n = 215) type II, and 31.2% (n = 106) type III. It was determined that 87.6% of the sigmoid sinus (SS) was lateral to the posterior semicircular canal and 12.4% was medial. It was determined that the TT area showed a positive correlation with petrous slope and a negative correlation with mastoid aeration. In other words, as the TT area increased, the petrous inclination angle also increased, but the mastoid aeration decreased. It was also found that the TT area was associated with the location of the SS and the largest TT area (164.84 ± 42.29 mm2) was observed in the posteriorly located SS. CONCLUSIONS: The relationship between TT and SS, petroclival angle, mastoid aeration, and subarcuate fossa has a very dynamic structure. Understanding the variations and clinical significance of these structures in the petroclival region is critical in determining the surgical approaches to be applied and understanding the etiology of vestibular system diseases.

Combined retrosigmoid and middle fossa approach for a small, superiorly located petroclival meningioma: how I do it.

BACKGROUND: Petroclival meningiomas pose significant surgical challenges because of their deep location and complex surrounding neurovasculature. The use of multiple surgical approaches can optimize safe tumor removal from multiple anatomic compartments. METHOD: We describe a patient with a growing superior petroclival meningioma centered at the posterior clinoid with extension into Meckel's cave that was successfully removed with a combined retrosigmoid and subtemporal middle fossa approach. This strategy avoided the need for anterior petrous bone drilling and tentorial splitting. CONCLUSION: A combined retrosigmoid and subtemporal middle fossa approach can provide safe access to tumors spanning the supra- and infratentorial compartments.

Combined and staged retrosigmoid, extended middle fossa, and endoscopic transnasal approach to a petroclival chondrosarcoma: how I do it.

BACKGROUND: Tumors of the petroclival region with multicompartment extension can be difficult to address with a single surgical approach. METHOD: We present the case of a patient with a large chondrosarcoma centered at the right petroclival fissure with extension into the cavernous sinus, the region beneath the cavernous sinus, cerebellopontine angle with deformation of the pons, and prevertebral space. A staged complete resection was performed using a stage 1 single-incision combined right retrosigmoid craniotomy and extended middle fossa craniotomy, followed by a stage 2 endoscopic transnasal approach. CONCLUSION: A combined approach to selected petroclival tumors can maximize safe resection.

Immunohistochemical markers predicting long-term recurrence following clival and spinal chordoma resection: a multicenter study.

OBJECTIVE: Chordomas are rare tumors from notochordal remnants and account for 1%-4% of all primary bone malignancies, often arising from the clivus and sacrum. Despite margin-negative resection and postoperative radiotherapy, chordomas often recur. Further, immunohistochemical (IHC) markers have not been assessed as predictive of chordoma recurrence. The authors aimed to identify the IHC markers that are predictive of postoperative long-term (≥ 1 year) chordoma recurrence by using trained multiple tree-based machine learning (ML) algorithms. METHODS: The authors reviewed the records of patients who had undergone treatment for clival and spinal chordomas between January 2017 and June 2021 across the Mayo Clinic enterprise (Minnesota, Florida, and Arizona). Demographics, type of treatment, histopathology, and other relevant clinical factors were abstracted from each patient record. Decision tree and random forest classifiers were trained and tested to predict long-term recurrence based on unseen data using an 80/20 split. RESULTS: One hundred fifty-one patients diagnosed and treated for chordomas were identified: 58 chordomas of the clivus, 48 chordomas of the mobile spine, and 45 chordomas sacrococcygeal in origin. Patients diagnosed with cervical chordomas were the oldest among all groups (58 ± 14 years, p = 0.009). Most patients were male (n = 91, 60.3%) and White (n = 139, 92.1%). Most patients underwent resection with or without radiation therapy (n = 129, 85.4%). Subtotal resection followed by radiation therapy (n = 51, 33.8%) was the most common treatment modality, followed by gross-total resection then radiation therapy (n = 43, 28.5%). Multivariate analysis showed that S100 and pan-cytokeratin are more likely to predict the increase in the risk of postoperative recurrence (OR 3.67, 95% CI 1.09-12.42, p= 0.03; and OR 3.74, 95% CI 0.05-2.21, p = 0.02, respectively). In the decision tree analysis, a clinical follow-up > 1897 days was found in 37% of encounters and a 90% chance of being classified for recurrence (accuracy = 77%). Random forest analysis (n = 500 trees) showed that patient age, type of surgical treatment, location of tumor, S100, pan-cytokeratin, and EMA are the factors predicting long-term recurrence. CONCLUSIONS: The IHC and clinicopathological variables combined with tree-based ML tools successfully demonstrated a high capacity to identify recurrence patterns with an accuracy of 77%. S100, pan-cytokeratin, and EMA were the IHC drivers of recurrence. This shows the power of ML algorithms in analyzing and predicting outcomes of rare conditions of a small sample size.

Rational sacrifice of the superior petrosal vein.

During surgeries of the posterior cranial fossa, sacrificing the superior petrosal vein (SPV) can enhance visualization of the operative field but may also lead to postoperative venous infarction with potentially fatal consequences. We previously proposed that the safety of SPV sacrifice depends on whether there are collaterals that can take on the venous flow of the SPV after its sacrifice. Here, we propose several practical strategies that may be able to evaluate the presence of collaterals to the SPV, so that the safety of SPV sacrifice can be assessed.

Ecchordosis Physaliphora: Does It Even Exist?

The term ecchordosis physaliphora (EP) has been used historically to describe a benign notochordal remnant with no growth potential, most commonly occuring in the central clivus. Unfortunately, the radiologic appearance of EP overlaps considerably with the appearance of low-grade chordomas, which do have the potential for growth. In this article, we review new pathologic terminology that better describes this family of diseases, and we propose new radiologic terms that better address the uncertainty of the radiologic diagnosis. The surgical importance of accurate terminology and the implications for patient care are discussed.

[Surgical Strategy for Skull Base Chordoma:Recent Advent of Neuroendoscopic Surgery].

Skull base chordoma is a rare bone tumor that is predominantly found in the clival area and considered to originate from the notochordal remnants. Chordoma is characterized by an aggressive nature and has a high risk of repeated recurrence despite multimodal treatments, including extensive surgical resection and high-dose radiotherapy. Thus, extensive surgical resection of the tumor and adjacent bony structures is strongly recommended. However, surgery was difficult because of the deep location of the lesion and involvement of important anatomies such as the cranial nerves and internal carotid arteries. Recent advent of endoscopic technology has changed visibility of the surgical field and accessibility, and surgical outcomes of this intractable tumor have dramatically changed. In this article, we present our surgical strategy of skull base chordoma aiming for radical surgical resection, focusing on the neuroendoscopic skull base surgery.

Pedunculated Chiari 3 malformation with proatlas defect.

Chiari III malformation is considered to be a rare congenital abnormality in human with very high mortality rates. Seventy percent of Chiari III is found to be associated with C1 arch defect as reported by Cakirer (Clin Imaging 27:1-4, 2003). The herniation of posterior fossa elements or dysplastic neural tissue is a must to stamp it as Chiari 3 malformation. The malformation is a result of the abnormal development of craniovertebral junction (CVJ). The CVJ developed from the occipital somites and the first spinal sclerotome. The major role in the development of the CVJ is played by the fourth occipital somite, which is otherwise known as "proatlas." The Chiari III anomalies are due to a result of proatlas defect, which results from failures of segmentation, failures of fusion of different components of each bone, or hypoplasia and ankylosis. We are presenting a case of a 1-year 4-month-old female child who presented with pedunculated swelling at the suboccipital region. The swelling was cystic and with pulsation. On evaluation, we found Chiari III anomaly with C1 posterior arch deficiency (proatlas defect). He was surgically managed. The outcome of the patient was good. Despite literature concluding Chiari 3 malformation with an unfavorable outcome, however, meticulous management and good pre- and postoperative care, physical therapy, and follow-up are necessary for good outcome.

Chiari I malformation: management evolution and technical innovation.

BACKGROUND AND DEFINITION: In recent years thanks to the growing use of radiological assessment, Chiari I malformation became one of the major diseases for a neurosurgeon to deal with. CIM can be classified according to the extent of cerebellar tonsil tip into the foramen magnum being a protrusion over five mm considered pathological. Such a disease is a heterogeneous condition with a multifactorial pathogenetic mechanism that can subdivided into a primary and secondary form. Regardless of the form, it seems that CIM is the result of an imbalance between the volume of the braincase and its content. Acquired CIMs are secondary to conditions causing intracranial hypertension or hypotension while the pathogenesis of primary forms is still controversial. PATHOGENESIS AND TREATMENT: There are several theories in the literature but the most accepted one implies an overcrowding due to a small posterior cranial fossa. While asymptomatic CIM do not need treatment, symptomatic ones prompt for surgical management. Several techniques are proposed being the dilemma centered in the need for dural opening procedures and bony decompression ones. CONCLUSION: Alongside the paper, the authors will address the novelty presented in the literature on management, diagnosis and pathogenesis in order to offer a better understanding of such a heterogeneous pathology.