Gamma knife radiosurgery for clival metastasis: case series and systematic review.

PURPOSE: Clival metastatic cancer is rare and has limited literature to guide management. We describe management of clival metastasis with Gamma Knife radiosurgery (GKRS). We augment our findings with a systematic review of all forms of radiation therapy for clival metastasis. METHODS: Records of 14 patients with clival metastasis who underwent GKRS at the University of Pittsburgh Medical Center from 2002 to 2023 were reviewed. Treatment parameters and clinical outcomes were assessed. A systematic review was conducted using evidence-based guidelines. RESULTS: The average age was 61 years with male predominance (n = 10) and average follow-up of 12.4 months. The most common primary cancers were prostate (n = 3) and lung (n = 3). The average time from cancer diagnosis to clival metastasis was 34 months. The most common presenting symptoms were headache (n = 9) and diplopia (n = 7). Five patients presented with abducens nerve palsies, and two presented with oculomotor nerve palsies. The median tumor volume was 9.3 cc, and the median margin dose was 15 Gy. Eleven patients achieved tumor control after one procedure, and three with progression obtained tumor control after repeat GKRS. One patient recovered abducens nerve function. The median survival from cancer diagnosis and GKRS were 49.7 and 15.3 months, respectively. The cause of death was progression of systemic cancer in six patients, clival metastasis in one, and unknown in four. The systematic review included 31 studies with heterogeneous descriptions of treatment and outcomes. CONCLUSION: Clival metastasis is rare and associated with poor prognosis. GKRS is a safe, effective treatment for clival metastasis.

Pediatric retroclival hematomas.

BACKGROUND: Traumatic retroclival hematomas (RCHs) are infrequent occurrences among the pediatric population. The existing body of research pertaining to these hematomas primarily consists of case reports or small case series, which do not provide adequate guidance for managing this condition. OBJECTIVE: This study aims to present a report on four cases of RCHs. Additionally, we aim to conduct a systematic review to consolidate the existing literature on pediatric RCHs. METHODS: The authors conducted a systematic review in accordance with the PRISMA and CARE guidelines. A multivariate logistic regression model was developed to evaluate the potential impact of various clinical variables on clinical outcomes. The study also documented four of our cases, one of which was a rare occurrence of spontaneous subdural RCH. RESULTS: A total of 62 traumatic RCHs have been documented in the literature. We documented three cases of traumatic RCHs and one case of spontaneous RCH. A systematic analysis of 65 traumatic RCHs was performed. Of trauma cases, 64.6% demonstrated craniocervical junction instability with 83.3% ligamentous involvement. Thirty-five patients were males. 50.7% were aged between 5 and 9 years. Cranial nerve palsies occurred in 29 patients (27 had abducent palsy), 26 of which resolved within 6 months of trauma. 23.5% underwent surgery, and 76.5% were conservatively managed. Surgeries targeted hematomas, hydrocephalus, or craniocervical instability. Approaches to hematomas included transclival and far/extreme lateral suboccipital approaches. Clinical outcome was good in 75.4% and intermediate or poor in 24.6%. Logistic regression suggested an association between craniocervical junction injuries and poor or intermediate outcomes (OR 4.88, 95% CI (1.17, 27.19), p = 0.04). CONCLUSION: Pediatric RCHs are mostly traumatic and extradural. Children between 5 and 9 years old are most vulnerable. Craniocervical junction injuries, mainly ligamentous, are common in RCHs and are associated with intermediate or poor outcomes. Cervical MRI could be important in cases of trauma to rule out ligamentous injuries of the craniocervical junction. The small size of RCHs should not exempt the careful assessment of craniocervical junction instability. Cranial nerve palsies are common and usually resolve within 6 months. Conservative treatment is typical unless brainstem compression, hydrocephalus, or craniocervical junction instability exists.

Acute myeloid leukemia associated with CHARGE syndrome.

CHARGE syndrome is a malformation disorder with diverse phenotypes that shows autosomal dominance with heterozygous variants in the chromodomain helicase DNA-binding 7 (CHD7) gene. Only a few cases of CHARGE syndrome accompanied by neoplasm during childhood have been reported. We report the case of a girl with CHARGE syndrome who developed acute myelogenous leukemia at 12 years old. She had mild intellectual disability, and hearing loss with inner ear malformation, myopia, astigmatism, laryngotracheal malacia, hypogonadism, and clival hypoplasia, with a history of patent ductus arteriosus. The patient was genetically diagnosed with CHARGE syndrome based on the detection of a novel heterozygous frameshift pathogenic variant in the CHD7 gene. We review the reported pediatric cases of CHARGE syndrome with malignancy and suggest a possible molecular mechanism of carcinogenesis involving pathogenic variants of the CHD7 gene.

Assessing survival outcomes and complication profiles following surgical excision and radiotherapy as interventions for skull base chordoma: a systematic review of operative margins and surgical approaches.

INTRODUCTION: Despite their precarious behavioral classification (benign and low grade on histopathology yet behaviorally malignant), great strides have been taken to improve prognostication and treatment paradigms for patients with skull base chordoma. With respect to surgical techniques, lateral transcranial (TC) approaches have traditionally been used, however endoscopic endonasal approaches (EEA) have been advocated for midline lesions. Nonetheless, due to the rarity of this pathology (0.2% of all intracranial neoplasms), investigations within the literature remain limited to small retrospective series. Furthermore, radiotherapeutic treatments investigated to date have proven largely ineffective. METHODS: Accordingly, we performed a systematic review in order to profile surgical and survival outcomes for skull base chordoma. Fixed and random-effect meta-analyses were performed for categorical variables including GTR, STR, 5-year OS, 10-year OS, 5-year PFS, and 10-year PFS. Additionally, we pooled eligible studies for formal meta-analysis to compare outcomes by surgical approach (lateral versus midline). Statistical analyses were performed using R Studio 'metafor' package or Cochrane Review Manager. Furthermore, meta-analysis of pooled mortality rates and sub-analyses of operative margin and surgical complications were used to compare midline versus lateral approaches via the Mantel-Haenszel method. We considered all p-values < 0.05 to be statistically significant. RESULTS: Following the systematic search and screen, 55 studies published between 1993 and 2022 reporting data for 2453 patients remained eligible for analysis. Sex distribution was comparable between males and females, with a slight predominance of male-identifying patients (0.5625 [95% CI: 0.5418; 0.3909]). Average age at diagnosis was 42.4 ± 12.5 years, while average age of treatment initiation was 43.0 ± 10.6 years. Overall, I2 value indicated notable heterogeneity across the 55 studies [I2 = 56.3% (95%CI: 44.0%; 65.9%)]. With respect to operative margins, the rate of GTR was 0.3323 [95% CI: 0.2824; 0.3909], I2 = 91.9% [95% CI: 90.2%; 93.4%], while the rate of STR was significantly higher at 0.5167 [95% CI: 0.4596; 0.5808], I2 = 93.1% [95% CI: 91.6%; 94.4%]. The most common complication was CSF leak (5.4%). In terms of survival outcomes, 5-year OS rate was 0.7113 [95% CI: 0.6685; 0.7568], I2 = 91.9% [95% CI: 90.0%; 93.5%]. 10-year OS rate was 0.4957 [95% CI: 0.4230; 0.5809], I2 = 92.3% [95% CI: 89.2%; 94.4%], which was comparable to the 5-year PFS rate of 0.5054 [95% CI: 0.4394; 0.5813], I2 = 84.2% [95% CI: 77.6%; 88.8%] and 10-yr PFS rate of 0.4949 [95% CI: 0.4075; 0.6010], I2 = 14.9% [95% CI: 0.0%; 87.0%]. There were 55 reported deaths for a perioperative mortality rate of 2.5%. The relative risk for mortality in the midline group versus the lateral approach group did not indicate any substantial difference in survival according to laterality of approach (-0.93 [95% CI: -1.03, -0.97], I2 = 95%, (p < 0.001). CONCLUSION: Overall, these results indicate good 5-year survival outcomes for patients with skull base chordoma; however, 10-year prognosis for skull base chordoma remains poor due to its radiotherapeutic resistance and high recurrence rate. Furthermore, mortality rates among patients undergoing midline versus lateral skull base approaches appear to be equivocal.

A 13-year patient journey of infant giant clival chordoma: case report and literature review.

Chordomas are rare malignant bone tumours that develop from the ectopic remnants of the embryonic notochord. In contrast to adults, the majority in children under 16 present intra-cranially (63%). In 2006, we reported the youngest case of a large clival chordoma, a 15-week old baby, the second case to present without skull base involvement and the fourth case of chordoma in a patient with tuberous sclerosis (TS) Kombogiorgas (Childs Nerv Syst 22(10):1369-1374, 2006). In this report, we provide an update on this patient's journey through a range of therapeutic options and summarize an update of the literature, since 2006, for this patient group.

[Endoscopic transnasal resection of clival meningiomas]. / Endoskopicheskoe transnazal'noe udalenie meningiom skata.

Treatment of clival meningiomas is still one of the unresolved issues in modern neurosurgery. There are several treatment strategies. These ones include various combinations of follow-up, surgical CSF drainage, tumor resection and radiotherapy. OBJECTIVE: To assess postoperative outcomes in patients with clival meningiomas. MATERIAL AND METHODS: We analyzed 18 patients with large or giant clival meningiomas. RESULTS: We assessed extent of resection using the scale by G. Frank and E. Pasquini (2002): total resection - 95-100%, subtotal - 80-95%, partial - 50-80%, extended biopsy - <50% of tumor. Total resection was achieved in 1 patient (5.5%), subtotal - 5 (27.8%), partial - 12 (66.7%). At the same time, brainstem decompression and regression of hydrocephalus were observed in all cases. Fourteen patients were followed-up. Median follow-up was 8.5 months. Seventeen patients underwent radiotherapy due to predominant partial and subtotal resection. Total focal dose ranged from 50 to 57 Gy in standard fractionation mode. None patient had residual tumor enlargement throughout the follow-up period. There were no lethal outcomes. CONCLUSION: Endoscopic transnasal access to clival meningiomas in appropriate anatomical features of tumor and surrounding structures is a full-fledged alternative to transcranial treatment in these patients. This approach provides total resection and brainstem decompression. These facts increase life expectancy without deterioration of the quality of life.

The impact of expanded endoscopic approaches on oncologic and functional outcomes for clival malignancies:a case series.

INTRODUCTION: Clival malignancies pose particular surgical challenges due to complex skull base anatomy and the involvement of vital neurovascular structures. While endoscopic endonasal approached are widely used, the outcomes for clival malignancies remain poorly understood. In this study we assessed the impact of endoscopic and open surgical approaches on PFS, time to initiation of radiotherapy, KPS, and GTR rates for clival malignancies. METHODS: A retrospective case series for clival malignancies operated between 1993 and 2019 was conducted. Inclusion criteria were age over 18 and a follow-up of at least a 6 months. Statistical analyses were conducted using STATA version 15 statistical software package StataCorp. RESULTS: For the whole cohort (113 patients), and for upper and middle lesions, open surgical approaches increased odds of disease progression, compared to EEA (HR 2.10 to HR 2.43), p < 0.05. EEA had a shorter time interval from surgery to initiation of radiotherapy. No difference in 6 and 12 month KPS was found between surgical groups. Patients undergoing open surgery were less likely to achieve GTR for upper clival lesions. CONCLUSIONS: EEA was found to be associated with increased PFS, for upper and middle clival malignancies. The time to initiation of radiotherapy was shorter for patients undergoing EEA compared to open surgery for patients with middle clival involvement. GTR rates were found to be significantly better with EEA for patients with upper clival malignancies.

Godtfredsen syndrome - recurrent clival chondrosarcoma with 6 years follow up: a case report and literature review.

BACKGROUND: We report a rare case of Godtfredsen syndrome caused by clival chondrosarcoma and perform a review of literatures. This article also explains the clinico-anatomical correlation of this rare neurological syndrome. CASE PRESENTATION: A 22-year-old gentleman presented with binocular diplopia. Clinical examination revealed an isolated right abducent nerve and right hypoglossal nerve palsy, with other cranial nerves intact. Neuroimaging revealed a right clival mass. Supraorbital craniotomy and tumour debulking were done in the same year. Histopathological examination showed low-grade chondrosarcoma. After 5-years of default, he came back with the tumour enlarged. He underwent a right orbitozygomatic craniotomy and tumour excision with 33 cycles of radiotherapy. Despite two surgeries and radiotherapy, the abducent nerve and hypoglossal nerve did not improve throughout 6 years of follow-up. Cranial nerve VI palsy is not always a false localizing sign, in Godtfredsen syndrome it serves as a localizing sign. CONCLUSION: To the best of our knowledge, this is the first case report of Godtfredsen Syndrome secondary to clival chondrosarcoma. Cranial nerve VI and XII palsy with no involvement of other cranial nerves, most likely the pathology is located at the clivus.

Skull base osteomyelitis mimicking skull base tumor in immunocompetent pediatric patient: case report.

Central skull base osteomyelitis (CSBO) is a rare complication of infection in pediatric patients, especially when there are no comorbidities like immunosuppression or metabolic illness. The diagnosis of CSBO is a challenge in children, and imaging findings can mimic skull base tumor. We describe the clinical history and image diagnosis of a case in a 6-year-old girl with no relevant history who presented an extensive skull base lesion. She underwent tumor resection surgery. The intraoperative finding confirmed clivus osteomyelitis, and the histopathological studies discarded malignancy. After diagnosis, the patient completed 6-week antibiotic treatment with adequate evolution. In conclusion, CSBO should be considered within the differential diagnoses due to the fact that it can mimic skull base lesions and it may present without relevant history.

Microsurgical versus endoscopic trans-sphenoidal approaches for clivus chordoma: a pooled and meta-analysis.

Chordoma is a rare slow-growing neoplastic bone lesion. However, they show an invasive local growth and high recurrence rate, leading to an overall survival rate of 65% at 5 years and 35% at 10 years. We conducted a pooled and meta-analysis comparing recurrence rate, post-operative-complications, and survival in patients undergoing either microsurgical (MA) or endoscopic approaches (EA). Search of literature was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines to identify surgical series of clivus chordomas published between January 1990 and March 2018 on Pubmed, Scopus, and Cochrane. Two different statistical analyses have been performed: a pooled analysis and a single-arm meta-analysis of overall recurrence rate and subgroup meta-analysis of complications in the subgroups open surgery and endoscopic surgery. After full-text screening, a total of 58 articles were included in the pooled analysis and 27 studies were included for the study-level meta-analysis. Pooled analysis-the extent of resection was the only association that remained significant (subtotal: HR = 2.18, p = 0.004; partial: HR = 4.40, p < 0.001). Recurrence was more prevalent among the surgical patients (45.5%) compared to endoscopic ones (23.7%). Meta-analysis-results of the cumulative meta-analysis showed an overall rate of recurrence of 25.6%. MA recurrence rate was 31.8% (99% CI 14-52.8), EA recurrence rate was 19.4% (5.4-39.2). CSF leak rate for the endoscopic group was 10.3% (99%CI 5-17.3) and 9.5% (99%CI 1.2-24.6) for the open surgery group. The partial removal versus total removal has an influence on recurrence rate (p < 0.001). MA recurrence rate was 31.8%; EA recurrence rate was 19.4%. The extent of resection is confirmed as a statistically significant factor affecting the risk for recurrence both with the pooled analysis and with the meta-analysis. Meta-analysis demonstrated that older patients tend to recur more than young patients, especially in surgical group.

Endoscopic transsphenoidal drainage of a petrous apex cholesterol granuloma using a frontal sinus nasal stent: a case report with long term follow up.

BACKGROUND: Cholesterol granulomas of the petrous apex are benign cysts affecting 0.6 patients per 1 million. The pathophysiology involves cholesterol crystals and lipids that are surrounded by giant cells. The cholesterol deposits induce an inflammatory response likely from acute hemorrhage leading to the formation of a fibrous capsule. The capsule expands over time compressing nearby cranial nerves (VI-VIII), which can cause worsening symptoms. Surgical resection has been shown to provide excellent improvement in symptoms. Historically, open approaches such as the infracochlear, infralabyrinthine, and middle fossa have been used. Herein we present a case showing the feasibility and clinical utility of using an endoscopic endonasal transclival approach for treatment of these tumors. CASE: A 44-year-old female presented with history of intermittent double vision, dizziness, nausea, and headaches for 3 years. She developed a partial left CN6 palsy with significant diplopia and episodes of left facial weakness. The worsening symptoms prompted presentation to the ED where MRI and CT scan revealed a left petrous apex lesion (1.8 × 1.7 cm) with hyperintensity on T1 and T2 imaging, suggestive of cholesterol granuloma. She underwent an endoscopic transclival resection of the lesion: drainage of left petrous apex cholesterol granuloma and stent placement from left petrous apex into sphenoid sinus (novel technique). This was done to allow continued communication and drainage of the tumor bed from the petrous apex into the sphenoid sinus with the intent to minimize the risk of recurrence. At the postop visit both 6th nerve palsy and diplopia had resolved. Imaging is stable 2 years after the surgery. DISCUSSION: The placement of the stent in this case was done to prevent symptom recurrence. 11% of patients that do not receive a stent will have symptom recurrence within one year. From the historical literature, only 4% of patients who had stent placement developed cyst recurrence or expansion on follow-up imaging. Stent placement has been shown to prevent cyst enlargement within the first few months after surgery. We demonstrate that the endoscopic endonasal transclival approach provided good visualization of the tumor, allowed for an adequate working window for resection, and provided a sufficient approach for stent placement.

Effect of clival bone growth on the localization of the abducens nerve at the petroclival region: a postmortem anatomical study.

PURPOSE: To investigate the effect of the clival bone pattern on the abducens nerve (AN) localization in the petroclival region between the Pediatric and Adult Groups. METHODS: This study used 12 pediatric and 17 adult heads obtained from the autopsy. The length and width of the clivus and the length of the petrosphenoidal ligaments (PSLs) were measured. The ratio of the length and width of the clivus was accepted as the clival index (CI). The localization of the AN at the petroclival region below the PSL, classified as lateral and medial, were recorded. RESULTS: The average length of the clivus was 26.92 ± 2.88 mm in the Pediatric Group, and 40.66 ± 4.17 mm in the Adult Group (p < 0.001). The average width of the clivus was 22.35 ± 2.88 mm in the Pediatric Group, and 29.96 ± 3.86 mm in the Adult Group (p < 0.001). The average value of the CI was 1.20 in the Pediatric Group and 1.36 in the Adult Group (p = 0.003). The length of the PSL was 7.0 ± 1.47 mm in the Pediatric Group and 11.05 ± 2.95 mm in the Adult Group (p < 0.001). The nerve was located below the medial side of the PSL in the Pediatric Group and below the lateral side in the Adult Group (p = 0.002). CONCLUSIONS: The petrous apex localization of the AN in adults compared with pediatric subjects could be related to the increased growth in the length of the clivus than its width.

Ectopic GH-secreting pituitary adenoma of the clivus: systematic literature review of a challenging tumour.

PURPOSE: Acromegaly is usually due to growth hormone (GH)-secreting pituitary adenomas, but it may be exceptionally caused by GH-secreting ectopic pituitary adenomas (EPA). EPA are defined as extra-sellar pituitary tumours, extra- or intra-cranially sited, entirely separated from the pituitary stalk and gland. The aim of the study is to address the challenges in the management of clival GHEPA. METHODS: We reported a case of a 53-year-old acromegalic patient with a primary clival GHEPA and reviewed systematically the relevant English literature between 1975 and 2019, in keeping with the PRISMA guidelines. RESULTS: Four cases of primary clival GHEPA have been described in literature apart from ours. All patients presented with acromegalic features, elevated circulating GH and/or insulin-like growth factor-1 levels. Hyperprolactinemia and empty sella were described in two cases, respectively. These tumours show the typical imaging characteristics of pituitary adenomas, but their neuroradiological diagnosis may be challenging due to their sizes and the difficulty in defining the absence of connections with the pituitary fossa. CONCLUSION: Although primary clival GHEPA are exceedingly rare, even if likely under-reported in literature, they should be considered in the differential diagnosis of clival tumours because of their specific management. Surgery represents the first-line treatment option, while medical and radiation therapies can be adopted as neo-adjuvant, adjuvant or primary treatments according to tumour and patient characteristics.

Clinical classification of clival chordomas for transnasal approaches.

Endoscopic endonasal approaches (EEAs) are ideal for most chordomas, but there is little information regarding the practical clinical classification of clival chordomas to guide surgery with EEAs. This article investigates a relatively concise and practical clinical classification system for clival chordomas and summarizes the clinical characteristics and operative key points of different clinical types. Here, 55 patients with clival chordomas treated through EEAs from 2012 to 2017 were retrospectively reviewed. Depending on the origin of the notochord and the growth pattern of the tumor, with our introduced Wang's line, these cases of clival chordoma were divided into types I-IV. There were 14 cases of type I-A, 7 cases of type I-B, 10 cases of type II, 10 cases of type III-A, 7 cases of type III-B, and 7 cases of type IV. The gross total resection (GTR) rate of primary and recurrent type I tumors was 64% and 25%, and residual tumors were found mainly in cases with involvement of the cavernous sinus or the posterior upper part of the dorsum sella. The GTR rate of primary and recurrent type II tumors was 85% and 66.6%, respectively. Residual tumors were found in cases with involvement of the petrous apex. The GTR rate of primary and recurrent type III tumors was 75% and 20%, and residual tumors were found in cases with involvement of the parapharyngeal space and dorsal side of C1-2. Residual type I-B and type III-B tumors were found when there was BA or VA adhesion or brain stem invasion. Our new classification method proposed here can be used to guide the resection of clival chordomas through EEAs.

The timing of fusion surgery for clival chordoma with occipito-cervical joint instability: before or after tumor resection?

Clival chordoma with occipito-cervical (OC) joint invasion can result in preoperative and postoperative instability. The authors investigate the appropriate timing of OC fusion to prevent instability-, fusion-, and surgery time-related morbidity. Twenty-two consecutive patients underwent surgery for clival chordoma from December 2008 to September 2014. OC fusion was performed for patients with OC joint invasion and instability due to preoperative destruction of the occipital condyle or extensive postoperative condylectomy. The data in relation to OC joint instability, fusion, and surgery time were analyzed retrospectively and compared between OC fusion before and after tumor resection. Of the 22 patients, 8 with tumor invasion of the OC joint underwent OC fusion. OC fusion was performed after tumor resection in one-stage for four patients and before tumor resection in two-stage for four patients. There was OC joint instability from tumor destruction of the occipital condyle in seven patients (87.5%). Patients with OC fusion after tumor resection encountered complications such as surgery site wound dehiscence, encephalitis, and cardiac arrest with consequent mortality in one patient. These complications were avoided in subsequent patients where OC fusion was performed before tumor resection. There were no differences in the extent of tumor resection between OC fusion before and after tumor resection. Two-stage OC fusion before tumor resection can reduce instability-, fusion-, and surgery time-related morbidity and achieve feasible tumor resection when OC joint instability is expected. The extent of tumor invasion and brain stem compression should be considered when fusion precedes tumor resection.

Comparative anatomical study on the role of zygomatic osteotomy in the extradural subtemporal approach to the clival region, when less is more.

PURPOSE: A great concern in performing the extradural subtemporal approach (ESTA) is the evaluation of the actual advantage provided by zygomatic osteotomy (ZO). Complications related to zygomatic dissection have been widely reported in the literature, making it of paramount importance to balance the actual need to perform it, against the risk of maneuver-related morbidity. Authors comparatively analyze the putative advantage provided by ZO in the ESTA in terms of anatomic exposure and surgical operability. Technical limits and potentials are critically revised and discussed. METHODS: A comparative microanatomical laboratory investigation was conducted. The operability score (OS) was applied for quantitative analysis of surgical operability. RESULTS: ZO was found to provide a weakly significant improvement in the surgical angle of attack (p value 0.01) (mean increase 3°). Maneuverability arch (MAC) increase related to ZO did not reach statistical significance (p value 0.09) (mean increase 2°). The variations provided by MAC increase on the conizing effect (CE) did not lead to an actual advantage in the real surgical scenario, modifying the vision area (VA) in terms of reduction of central vision area (CA) in favor of an increase of peripheral vision area (PA) only in the most caudal part of the surgical field. Ultimately, ZO did not influence the overall OS, scoring both ESTA-ZO+ and ESTA-ZO- 2 out of 3. CONCLUSION: In the ESTA, ZO does not provide an actual significant advantage in terms of surgical operability on clival and paraclival areas.

Anatomical location of the abducens nerves (VI) in the ventral approach of clival tumors.

The aim of this work was to determine reliable anatomical landmarks for locating and preserving the abducens nerves (6th cranial nerves) during trans-facial or trans-nasal endoscopic approaches of skull base tumors involving the clivus and the petrous apex. In order to describe this specific anatomy, we carefully dissected 10 cadaveric heads under optic magnification. Several measurements were taken between the two petro-sphénoidal foramina, from the bottom of the sella and the dorsum sellae. The close relationship between the nerves and the internal carotid artery were taken into account. We defined a trapezoid area that allowed drilling the clivus safely, preserving the 6th cranial nerve while being attentive to the internal carotid artery. The caudal part of this trapezium is, on average, 20 mm long at mi-distance between the two petro-sphenoidal foramina. The cranial part is at the sella level, a line between both paraclival internal carotid arteries. Oblique lateral edges between the cranial and caudal parts completed the trapezium.

Ectopic pituitary adenomas: common presentations of a rare entity.

PURPOSE: Ectopic pituitary adenomas (EPA) are rare tumors thought to arise from embryological remnants along the route of normal pituitary migration. Clinically, these tumors vary in hormonal activity and can exert mass effect based on location and size. METHODS: We performed a single-center retrospective analysis of patients with diagnosis of ectopic pituitary adenoma from 2001 to 2018. RESULTS: Five patients were identified with EPA: a 48-year-old woman with suprasellar EPA treated with transsphenoidal removal of the tumor, a 44-year-old woman with cavernous EPA treated with transsphenoidal removal of the tumor, a 48-year-old woman with sphenoid EPA treated with cabergoline, a 45-year-old man with clival EPA treated with cabergoline and transsphenoidal surgical resection, and a 54-year-old man with clival EPA treated with cabergoline therapy. CONCLUSIONS: EPA should be considered as a differential diagnosis of juxta-sellar lesions. Appropriate hormonal testing may lead to early diagnosis, avoidance of unnecessary biopsy or surgery, and improved outcomes.

Clival screw and plate fixation by the transoral approach for the craniovertebral junction: a CT-based feasibility study.

PURPOSE: A clivus screw and plate was invented and proved to strengthen the stability of the craniovertebral junction (CVJ). However, it is unclear whether the clivus screw and plate could be placed onto the CVJ by transoral approach. Therefore, the present study aims to evaluate the feasibility of clivus screw and plate placement by transoral approach and investigate its relative anatomic parameters. METHODS: A total of 80 normal adults (40 males/40 females) with an average age of 60.4 ± 11.6 years old were enrolled in this study. All parameters were measured in a supposed maximums mouth-opening status on computed tomography images, where the vertex of lower incisor was defined as Point A. The vertical intersection from Point A to extracranial clivus was defined as Point B, and its distance to the bottom of clivus was measured as B length. Point B was considered as ideal screw entry point. All the cases were divided into three types based on the location of Point B: above the top portion (Type 1), between the top and bottom portion (Type 2), and below the bottom portion (Type 3) of extracranial clivus. The B Length was defined as a minus value if the case belonged to Type 3. The anterior skull base angle, the angles between tangent of extracranial clivus and the lines from Point A to different parts of clivus, and distances between Point A and clivus and C1-3 vertebra were also measured. RESULTS: One in eighty cases (1.2%) belonged to Type 1 with a B Length of 32.12 mm. Most cases (61.3%) were Type 2 with a B Length of 8.7 mm, while Type 3's was - 9.7 mm occupying for 37.5%. Significant statistic differences were found in anterior skull base angle between these three types (128.9°, 122.7° and 118.5° for Type 1, 2 and 3, respectively). The distances from Point A to the top and bottom portion of the clivus and the pharyngeal tubercle were 97.5, 96.0 and 96.8 mm, respectively. The angles between the tangent of the clivus and the lines from Point A to the above three structures were 75.7°, 92.3° and 84.0°, respectively. The distances from Point A to the middle point of anterior margin of C1 anterior tubercle, C2 vertebra and C3 vertebra were 79.1, 73.4 and 61.5 mm, respectively. CONCLUSION: The clivus screw and plate placement could be accomplished with optimal screw angle by transoral approach in most of patients. Mandibular splitting would be needed in patients with greater anterior skull angle. These slides can be retrieved under Electronic Supplementary Material.

An Incidental Finding of Ecchordosis Physaliphora in a Case of Abducens Nerve Palsy: Case Report.

Ecchordosis physaliphora (EP) is a rare non-malignant mass that originates from remains of the notochord and is typically asymptomatic. A 42-year-old man presented with sudden onset of painless horizontal diplopia and his neurological exam showed sixth cranial nerve palsy. Magnetic resonance imaging (MRI) identified a non-enhanced retroclival mass (EP) with increased signal intensity on T2 and decreased signal intensity on T1-weighted sequences. He was treated with methylprednisolone, completely recovered in four weeks and has remained symptom free. Conservative management should be attempted before surgery in all cases since symptoms can resolve spontaneously and EP could be an incidental finding.