Management strategies in clival and craniovertebral junction chordomas: a 29-year experience

J Neurosurg 138:1640–1652, 2023

Chordomas represent one of the most challenging subsets of skull base and craniovertebral junction (CVJ) tumors to treat. Despite extensive resection followed by proton-beam radiation therapy, the recurrence rate remains high, highlighting the importance of developing efficient treatment strategies. In this study, the authors present their experience in treating clival and CVJ chordomas over a 29-year period.

METHODS The authors conducted a retrospective study of clival and CVJ chordomas that were surgically treated at their institution from 1991 to 2020. This study focuses on three aspects of the management of these tumors: the factors influencing the extent of resection (EOR), the predictors of survival, and the outcomes of the endoscopic endonasal approaches (EEAs) compared with open approaches (OAs).

RESULTS A total of 265 surgical procedures were performed in 210 patients, including 123 OAs (46.4%) and 142 EEAs (53.6%). Tumors that had an intradural extension (p = 0.03), brainstem contact (p = 0.005), cavernous sinus extension (p = 0.004), major artery encasement (p = 0.01), petrous apex extension (p = 0.003), or high volume (p = 0.0003) were significantly associated with a lower EOR. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 52.1% and 75.1%, respectively. Gross-total resection and Ki-67 labeling index < 6% were considered to be independent prognostic factors of longer PFS (p = 0.0005 and p = 0.003, respectively) and OS (p = 0.02 and p = 0.03, respectively). Postoperative radiation therapy correlated independently with a longer PFS (p = 0.006). Previous surgical treatment was associated with a lower EOR (p = 0.01) and a higher rate of CSF leakage after EEAs (p = 0.02) but did not have significantly lower PFS and OS compared with primary surgery. Previously radiation therapy correlated with a worse outcome, with lower PFS and OS (p = 0.001 and p = 0.007, respectively). EEAs were more frequently used in patients with upper and middle clival tumors (p = 0.002 and p < 0.0001, respectively), had a better rate of EOR (p = 0.003), and had a lower risk of de novo neurological deficit (p < 0.0001) compared with OAs. The overall rate of postoperative CSF leakage after EEAs was 14.8%.

CONCLUSIONS This large study showed that gross-total resection should be attempted in a multidisciplinary skull base center before providing radiation therapy. EEAs should be considered as the gold-standard approach for upper/middle clival lesions based on the satisfactory surgical outcome, but OAs remain important tools for large complex chordomas.

Endoscopic endonasal approach for infradiaphragmatic craniopharyngiomas: a multicentric Italian study

J Neurosurg 138:522–532, 2023

Infradiaphragmatic craniopharyngiomas (ICs) represent a distinct subtype, harboring a sellar-suprasellar origin and generally growing in the extra-arachnoidal space contained by the diaphragma sellae. They have been considered ideal for surgical removal through the transsphenoidal approach since the 1960s. The authors present a multicentric national study, intending to selectively analyze IC behavior and the impact of the transsphenoidal endoscopic endonasal approach (EEA) on surgical outcomes.

METHODS Craniopharyngiomas that were intraoperatively recognized as infradiaphragmatic and removed with standard EEA between 2000 and 2021 at 6 Italian neurosurgical departments were included in the study. Clinical, radiological, and surgical findings and outcomes were evaluated and reviewed.

RESULTS In total, 84 patients were included, with 45.23% identified as pediatric cases and 39.28% as having recurrent tumors. The most common presenting symptoms were endocrine (75%), visual (59.52%), and hypothalamic (26.19%) disorders. ICs were classified as extending below (6 intrasellar and 41 occupying the suprasellar cistern) or above (26 obliterating the anterior recesses of the third ventricle and 11 extending up to the foramina of Monro) the chiasmatic cistern. Gross-total resection (GTR) was achieved in 54 cases (64.28%). Tumor extension above the chiasmatic cistern and calcifications were associated with lower likelihood of GTR. The cumulative rate of postoperative complications was 34.53%, with CSF leak being the most common (14.28%). Endocrine, visual, and hypothalamic functions deteriorated postoperatively in 41/78 patients (52.56%), 5/84 (5.95%), and 14/84 (16.67%), respectively. Twenty-eight patients (33.33%) had recurrence during follow-up (mean 63.51 months), with a mean 5-year progression-free survival (PFS) rate of 58%. PFS was greater in patients who achieved GTR than patients with other extent of resection.

CONCLUSIONS This is the largest series in the literature to describe ICs removed with standard EEA, without the need for additional bone and dural opening over the planum sphenoidale. EEA provides a direct route to ICs, the opportunity to manage lesions extending up to the third ventricle without breaching the diaphragma, and high rates of GTR and satisfactory clinical outcomes. Increased surgical complexity and morbidity should be expected in patients with extensive suprasellar extension and involvement of the surrounding vital neurovascular structures.

Septal rhinopharyngeal flap: a novel technique for skull base reconstruction after endoscopic endonasal clivectomies

J Neurosurg 136:1601–1606, 2022

Endoscopic endonasal reconstruction techniques have improved CSF leak rates that were initially reported after surgery for cranial base and intradural lesions. However, wide surgical defects still pose a problem, especially if located in the clival region.

The authors propose and describe a novel reconstruction technique they call a septal rhinopharyngeal flap (SRF) specifically designed to address this issue. The SRF is formed by three components of mucosa: 1) septal, 2) rhinopharyngeal roof, and 3) rhinopharyngeal posterior wall components, which allows for the coverage of the tuberculum/ sellar region, midclivus, and lower clivus, respectively. A step-by-step procedure is described and its results analyzed in cases in which it has been used. The SRF was performed in 8 patients, which included diagnoses of 4 chordomas, 2 petroclival meningiomas, 1 invasive pituitary adenoma, and 1 chondrosarcoma. The size of the flap was considered optimal in all patients (100%). Postoperative MRI revealed contrast enhancement covering the entire surface of the flap. No CSF leaks were encountered after at least 1 postoperative year.

The SRF is a novel vascularized reconstruction technique specifically indicated for wide endosanasal clivectomies focused on the middle clivus with caudal extension into the lower clivus and craniocervical junction, as well as rostral extensions into the tubercular or planum sphenoidale. This new reconstruction technique could be added to the skull base reconstruction armamentarium as a safe and optimal option.

 

Complications after 1002 endoscopic endonasal approach procedures at a single center: lessons learned, 2010–2018

https://doi.org/10.3171/2020.11.JNS202494

The endoscopic endonasal approach (EEA) has evolved into a mainstay of skull base surgery over the last two decades, but publications examining the intraoperative and perioperative complications of this technique remain scarce. A prior landmark series of 800 patients reported complications during the first era of EEA (1998–2007), parallel to the development of many now-routine techniques and technologies. The authors examined a single-institution series of more than 1000 consecutive EEA neurosurgical procedures performed since 2010, to elucidate the safety and risk factors associated with surgical and postoperative complications in this modern era.

METHODS
After obtaining institutional review board approval, the authors retrospectively reviewed intraoperative and postoperative complications and their outcomes in patients who underwent EEA between July 2010 and June 2018 at a single institution.

RESULTS
The authors identified 1002 EEA operations that met the inclusion criteria. Pituitary adenoma was the most common pathology (n = 392 [39%]), followed by meningioma (n = 109 [11%]). No patients died intraoperatively. Two (0.2%) patients had an intraoperative carotid artery injury: 1 had no neurological sequelae, and 1 had permanent hemiplegia. Sixty-one (6.1%) cases of postoperative cerebrospinal fluid leak occurred, of which 45 occurred during the original surgical hospitalization. Transient postoperative sodium dysregulation was noted after 87 (8.7%) operations. Six (0.6%) patients were treated for meningitis, and 1 (0.1%) patient died of a fungal skull base infection. Three (0.3%) patients died of medical complications, thereby yielding a perioperative 90-day mortality rate of 0.4% (4 deaths). High-grade (Clavien-Dindo grade III–V) complications were identified after 103 (10%) EEA procedures, and multivariate analysis was performed to determine the associations between factors and these more serious complications. Extradural EEA was significantly associated with decreased rates of these high-grade complications (OR [95% CI] 0.323 [0.153–0.698], p = 0.0039), whereas meningioma pathology (OR [95% CI] 2.39 [1.30–4.40], p = 0.0053), expanded-approach intradural surgery (OR [95% CI] 2.54 [1.46–4.42], p = 0.0009), and chordoma pathology (OR [95% CI] 9.31 [3.87–22.4], p < 0.0001) were independently associated with significantly increased rates of high-grade complications.

CONCLUSIONS
The authors have reported a large 1002-operation cohort of EEA procedures and associated complications. Modern EEA surgery for skull base pathologies has an acceptable safety profile with low morbidity and mortality rates. Nevertheless, significant intraoperative and postoperative complications were correlated with complex intradural procedures and meningioma and chordoma pathologies.

The rhinopharyngeal flap for reconstruction of lower clival and craniovertebral junction defects

J Neurosurg 135:1319–1327, 2021

The endoscopic endonasal approach (EEA) to the lower clivus and craniovertebral junction (CVJ) has been traditionally performed via resection of the nasopharyngeal soft tissues. Alternatively, an inferiorly based rhinopharyngeal (RP) flap (RPF) can be dissected to help reconstruct the postoperative defect and separate it from the oropharynx. To date, there is no evidence regarding the viability and potential clinical impact of the RPF. The aim of this study was to assess RPF viability and its impact on clinical outcome.

METHODS A retrospective cohort of 60 patients who underwent EEA to the lower clivus and CVJ was studied. The RPF was used in 30 patients (RPF group), and the nasopharyngeal soft tissues were resected in 30 patients (control group).

RESULTS Chordoma was the most common surgical indication in both groups (47% in the RPF group vs 63% in the control group, p = 0.313), followed by odontoid pannus (20% in the RPF group vs 10%, p = 0.313). The two groups did not significantly differ in terms of extent of tumor (p = 0.271), intraoperative CSF leak (p = 0.438), and skull base reconstruction techniques other than the RPF (nasoseptal flap, p = 0.301; fascia lata, p = 0.791; inlay graft, p = 0.793; and prophylactic lumbar drain, p = 0.781). Postoperative soft-tissue enhancement covering the lower clivus and CVJ observed on MRI was significantly higher in the RPF group (100% vs 26%, p < 0.001). The RPF group had a significantly lower rate of nasoseptal flap necrosis (3% vs 20%, p = 0.044) and surgical site infection (3% vs 27%, p = 0.026) while having similar rates of postoperative CSF leakage (17% in the RPF group vs 20%, p = 0.739) and meningitis (7% in the RPF group vs 17%, p = 0.424). Oropharyngeal bacterial flora dominated the infections in the control group but not those in the RPF group, suggesting that the RPF acted as a barrier between the nasopharynx and oropharynx.

CONCLUSIONS The RPF provides viable vascularized tissue coverage to the lower clivus and CVJ. Its use was associated with decreased rates of nasoseptal flap necrosis and local infection, likely due to separation from the oropharynx.

An endoscopic endonasal approach to craniopharyngioma via the infrachiasmatic corridor

Acta Neurochirurgica (2021) 163:2253–2268

The infrachiasmatic corridor is the most important surgical access route for craniopharyngiomas and was identified and used in clinical series. The aims of this study were to describe the characteristics that assist dissection and resection rates in endoscopic surgery of solid, cystic, and recurrent cases and their importance in the infrachiasmatic corridor in endoscopic surgery.

Methods One hundred operations on 84 patients with pathologically identified craniopharyngioma were included in the study. The MRI findings were evaluated, and the location of the lesions was classified as (1) infrasellar; (2) sellar; or (3) suprasellar. In the sagittal plane, we measured the longest diameter of cystic and solid components and the height of chiasm-sella. Images were assessed for the extent of resection and were classified as gross total resection. This was deemed as the absence of residual tumor and subtotal resection, which had residual tumor.

Results The infrasellar location was reported in 7/84 (8.3%) patients, the sellar location in 8/84 (9.5%), and the suprasellar location in 69/84 (82.1%) patients. The narrow and high chiasm-sella were observed in 28/69 (40.5%) and 41/69 patients (59.4%), respectively. The mean distance of the chiasm-sella was 9.46± 3.76. Gross total tumor resection was achieved in 60/ 84 (71.4%) and subtotal tumor resection was performed in 24/84 (28.6%) patients. The results revealed that suprasellar location (OR: 0.068; p = 0.017) and recurrent cases (OR: 0.011; p<0.001) were negative predictive factors on GTR. Increasing the experience (OR: 42,504; p = 0.001) was a positive predictor factor for GTR.

Conclusion An EETS approach that uses the infrachiasmatic corridor is required for skull base lesions extending into the suprasellar area. The infrachiasmatic corridor can determine the limitations of endoscopic craniopharyngioma surgery. This corridor is a surgical safety zone for inferior approaches.

How I do it: endoscopic endonasal resection of tuberculum sellae meningioma

Acta Neurochirurgica (2021) 163:2193–2197

Tuberculum sellae meningiomas (TSMs) adherent to neurovascular structures are particularly challenging lesions requiring delicate and precise microneurosurgery. There is an ongoing debate about the optimal surgical approach.

Method We describe technical nuances and challenges in TSM resection using the endoscopic endonasal approach (EEA) in two cases of fibrous tumors with adherence to neurovascular structures. The cases are illustrated with a video (case 1) and figures (cases 1 and 2).

Conclusion A dedicated team approach and precise microsurgical technique facilitate safe resection of complex TSMs through the EEA.

Endoscopic endonasal approach for suprasellar meningiomas

J Neurosurg 135:113–125, 2021

The endoscopic endonasal approach (EEA) has gained increasing popularity for the resection of suprasellar meningiomas (SSMs). Appropriate case selection is critical in optimizing patient outcome. Long-term outcome data are lacking. The authors systematically identified preoperative factors associated with extent of resection (EOR) and determined the relationship between EOR and long-term recurrence after EEA for SSMs.

METHODS
In this retrospective cohort study, the authors identified preoperative clinical and imaging characteristics associated with EOR and built on the recently published University of California, San Francisco resectability score to propose a score more specific to the EEA. They then examined the relationship between gross-total resection (GTR; 100%), near-total resection (NTR; 95%–99%), and subtotal resection (STR; < 95%) and recurrence or progression with Kaplan-Meier survival analysis.

RESULTS
A total of 51 patients were identified. Radiographic GTR was achieved in 40 of 47 (85%) patients in whom it was the surgical goal. Significant independent risk factors for incomplete resection were prior surgery (OR 25.94, 95% CI < 2.00 to 336.49, p = 0.013); tumor lateral to the optic nerve (OR 13.41, 95% CI 1.82–98.99, p = 0.011); and complete internal carotid artery (ICA) encasement (OR 15.12, 95% CI 1.17–194.08, p = 0.037). Tumor size and optic canal invasion were not significant risk factors after adjustment for other variables. A resectability score based on the multivariable model successfully predicted the likelihood of GTR; a score of 0 had a positive predictive value of 97% for GTR, whereas a score of 2 had a negative predictive value of 87.5% for incomplete resection. After a mean follow-up of 40.6 ± 32.4 months (mean ± SD), recurrence was 2.7% after GTR (1 patient with atypical histology), 44.4% after NTR, and 80% after STR (p < 0.0001). Vision was stable or improved in 93.5% and improved in 67.4% of patients with a preoperative deficit. There were 5 (9.8%) postoperative CSF leaks, of which 4 were managed with lumbar drains and 1 required a reoperation.

CONCLUSIONS
The EEA is a safe and effective approach to SSMs, with favorable visual outcomes in well-selected cases. The combination of postoperative MRI-based EOR with direct endoscopic inspection can be used in lieu of Simpson grade to predict recurrence. GTR dramatically reduces recurrence and can be achieved regardless of tumor size, proximity or encasement of the anterior cerebral artery, or medial optic canal invasion. Risk factors for incomplete resection include prior surgery, tumor lateral to the optic nerve, and complete ICA encasement.

Access to Meckel’s cave for biopsies of indeterminate lesions

Neurosurgical Review (2021) 44:249–259

Accessing Meckel’s cave (MC) is surgically challenging. Open approaches are complex and often correlated with high morbidity. Endoscopic approaches emerged in the last decade as feasible alternatives to open approaches, especially for sampling indeterminate lesions.

This article first analyses available routes to approach Meckel’s cave and presents furthermore an illustrative case. We conducted a systematic review and reported according to the guidelines for preferred reporting items for systematic reviews and meta-analyses (PRISMA). Various surgical approaches identified through the search are evaluated and discussed in detail. Additionally, we report on a case of woman with a lesion in MC, which was accessed through an endoscopic transpterygoid approach subsequently diagnosed as a diffuse large B cell lymphoma. Our search delivered 75 articles that included case reports (n = 21), cadaveric studies (n = 32), clinical articles (n = 16), review of the literatures (n = 3), as well as technical notes (n = 2) and a radiological manuscript (n = 1).

Open routes included lateral approaches with many variations, mainly intra- and extradural pterional approaches and anterior petrosal, as well as a retrosigmoid intradural suprameatal and a lateral transorbital approach.

Endoscopically, MC was reached via approaches that included transpterygoid, transorbital or infraorbital fissure routes.

Percutaneous approaches, e.g. through the foramen ovale, were also described. Multiple surgical approaches to MC are currently available.

Their different characteristics as well as individual patient factors, such as clinical history and the localization of the disease, have to be considered when choosing a surgical corridor.

Studies included in this review highlight the endonasal endoscopic transpterygoidal technique as an excellent corridor for biopsies in the ventral MC.

Endoscopic transorbital and endonasal approach for trigeminal schwannomas

J Neurosurg 133:467–476, 2020

Trigeminal schwannomas are rare neoplasms with an incidence of less than 1% that require a comprehensive surgical strategy. These tumors can occur anywhere along the path of the trigeminal nerve, capable of extending intradurally into the middle and posterior fossae, and extracranially into the orbital, pterygopalatine, and infratemporal fossa. Recent advancements in endoscopic surgery have suggested a more minimally invasive and direct route for tumors in and around Meckel’s cave, including the endoscopic endonasal approach (EEA) and endoscopic transorbital superior eyelid approach (ETOA). The authors assess the feasibility and outcomes of EEA and ETOA for trigeminal schwannomas.

METHODS A retrospective multicenter analysis was performed on 25 patients who underwent endoscopic surgical treatment for trigeminal schwannomas between September 2011 and February 2019. Thirteen patients (52%) underwent EEA and 12 (48%) had ETOA, one of whom underwent a combined approach with retrosigmoid craniotomy. The extent of resection, clinical outcome, and surgical morbidity were analyzed to evaluate the feasibility and selection of surgical approach between EEA and ETOA based on predominant location of trigeminal schwannomas.

RESULTS According to predominant tumor location, 9 patients (36%) had middle fossa tumors (Samii type A), 8 patients (32%) had dumbbell-shaped tumors located in the middle and posterior cranial fossae (Samii type C), and another 8 patients (32%) had extracranial tumors (Samii type D). Gross-total resection (GTR, n = 12) and near-total resection (NTR, n = 7) were achieved in 19 patients (76%). The GTR/NTR rates were 81.8% for ETOA and 69.2% for EEA. The GTR/NTR rates of ETOA and EEA according to the classifications were 100% and 50% for tumors confined to the middle cranial fossa, 75% and 33% for dumbbell-shaped tumors located in the middle and posterior cranial fossae, and 50% and 100% for extracranial tumors. There were no postoperative CSF leaks. The most common preoperative symptom was trigeminal sensory dysfunction, which improved in 15 of 21 patients (71.4%). Three patients experienced new postoperative complications such as vasospasm (n = 1), wound infection (n = 1), and medial gaze palsy (n = 1).

CONCLUSIONS ETOA provides adequate access and resectability for trigeminal schwannomas limited in the middle fossa or dumbbell-shaped tumors located in the middle and posterior fossae, as does EEA for extracranial tumors. Tumors predominantly involving the posterior fossa still remain a challenge in endoscopic surgery.

Clinical features and operative technique of transinfundibular craniopharyngioma

J Neurosurg 133:119–128, 2020

Transinfundibular craniopharyngioma (TC) is one of the 4 subtypes of suprasellar craniopharyngioma. In this study, the authors analyzed the clinical features of and operative technique for TC.

METHODS A total of 95 consecutive cases of suprasellar craniopharyngioma that had been resected via the endoscopic expanded endonasal approach were retrospectively reviewed. Patients were divided into 2 groups: 34 in the TC group and 61 in the nontransinfundibular craniopharyngioma (NC) group. Clinical and radiographic features, intraoperative findings, histopathological and genetic findings, and surgical outcomes were analyzed and compared between groups.

RESULTS Compared with NC, TC was mostly seen in adult patients (97.1%); it was rare in children (2.9%). Clinical presentations tended toward headache, hydrocephalus, and diabetes insipidus. The relatively smaller volume, midline location (consistent with the stalk position), unidentifiable stalk, no shift of the third ventricle, and greater likelihood to involve the third ventricle and cause hydrocephalus were the characteristic features of TC in the preoperative MRI study. According to the degree of vertical extension of the tumor, the 34 TCs could be classified into 3 subtypes: type 1, entity was limited to stalk (n = 2, 5.9%); type 2, tumor extended up to the third ventricle (type 2a) or down to the subdiaphragmatic cavity (type 2b) (n = 23, 67.6%); and type 3, tumor extended in both directions (n = 9, 26.5%). For TC resection, the chiasm–pituitary corridor, lamina terminalis corridor, and pituitary corridor could be used separately or jointly. Most of the TCs originated from the infundibulum–tuber cinereum, grew within and along the long axis of the infundibulum, and the pituitary stalk was not usually preserved in TCs (20.6%), whereas the rate of preservation was higher (80.3%) in NCs. Bilateral hypothalamic injury was found in nearly all TCs if radical resection was performed, whereas the relationship between NCs and hypothalamus was either compression (32.8%) or unilateral invasion (67.2%). Meanwhile, the postoperative endocrine and neuropsychological function outcomes in patients with TC were worse than in patients with NC. The genetic analysis with whole-exome sequencing studies showed no differential mutations of CTNNB1 (b-catenin) and BRAF (V600E) between TC and NC subtypes, but there was a difference between adamantinomatous craniopharyngioma and papillary craniopharyngioma.

CONCLUSIONS TC is a special subtype of suprasellar craniopharyngioma, which is remarkably different from NC. Identification of this type of tumor preoperatively is essential for the planning of appropriate surgical approach and degree of excision.

Endoscopic endonasal treatment of maxillary nerve (V2) painful neuropathy: cadaveric study with clinical correlation

Acta Neurochirurgica (2020) 162:223–229

Surgical access to the second (V2, maxillary) and third (V3, mandibular) branches of the trigeminal nerve (V) has been classically through a transoral approach. Increasing expertise with endoscopic anatomy has achieved less invasive, more efficient access to skull base structures. The authors present a surgical technique using an endoscopic endonasal approach for the treatment of painful V2 neuropathy.

Methods Endoscopic endonasal dissections using a transmaxillary approach were performed in four formalin-fixed cadaver heads to expose the V2 branch of the trigeminal nerve. Relevant surgical anatomy was evaluated and anatomic parameters for neurectomy were identified.

Results Endoscopic endonasal transmaxillary approaches completed bilaterally to the pterygopalatine and pterygomaxillary fossae exposed the V2 branch where it emerged from the foramen rotundum. The anatomy defined for the location of neurectomy was determined to be the point where V2 emerged from the foramen rotundum into the pterygopalatine fossa. The technique was then performed in 3 patients with intractable painful V2 neuropathy.

Conclusions In our cadaveric study and clinical cases, the endoscopic endonasal approach to the pterygopalatine fossa achieved effective exposure and treatment of isolated V2 painful neuropathy. Important surgical steps to visualize the maxillary nerve and its branches and key landmarks of the pterygopalatine fossa are discussed. This minimally invasive approach appears to be a valid alternative for select patients with painful V2 trigeminal neuropathy.

Endoscopic endonasal transoculomotor triangle approach for adenomas invading the parapeduncular space

J Neurosurg 130:1304–1314, 2019

Pituitary adenomas may extend into the parapeduncular space by invading through the roof of the cavernous sinus. Currently, a transcranial approach is the preferred choice, with or without the combination of an endonasal approach. In this paper the authors present a novel surgical approach that takes advantage of the natural corridor provided by the tumor to further open the oculomotor triangle and resect tumor extension into the parapeduncular space.

METHODS Six injected specimens were used to demonstrate in detail the surgical anatomy related to the approach. Four cases in which the proposed approach was used were retrospectively reviewed.

RESULTS From a technical perspective, the first step involves accessing the superior compartment of the cavernous sinus. The interclinoid ligament should be identified and the dura forming the oculomotor triangle exposed. The oculomotor dural opening may be then extended posteriorly toward the posterior petroclinoidal ligament and inferolaterally toward the anterior petroclinoidal ligament. The oculomotor nerve should then be identified; in this series it was displaced superomedially in all 4 cases. The posterior communicating artery should also be identified to avoid its injury. In all 4 cases, the tumor invading the parapeduncular space was completely removed. There were no vascular injuries and only 1 patient had a partial oculomotor nerve palsy that completely resolved in 2 weeks.

CONCLUSIONS The endoscopic endonasal transoculomotor approach is an original alternative for removal of tumor extension into the parapeduncular space in a single procedure. The surgical corridor is increased by opening the dura of the oculomotor triangle and by working below and lateral to the cisternal segment of the oculomotor nerve.

 

Clival chordomas: considerations after 16 years of endoscopic endonasal surgery

J Neurosurg 128:329–338, 2018

In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach.

METHODS All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter. Postoperative adjuvant therapies were considered.

RESULTS Sixty-five patients (male/female ratio 1:0.9) were included in this study. The median age was 48 years (range 9–80 years). Gross-total resection (GTR) was achieved in 47 cases (58.7%). On univariate analysis, primary procedures (p = 0.001), location in the superior or middle third of the clivus (p = 0.043), extradural location (p = 0.035), and histology of conventional chordomas (p = 0.013) were associated with a higher rate of GTR. The complication rate was 15.1%, and there were no perioperative deaths. Most complications did not result in permanent sequelae and included 2 CSF leaks (2.5%), 5 transient cranial nerve VI palsies (6.2%), and 2 internal carotid artery injuries (2.5%), which were treated with coil occlusion of the internal carotid artery without neurological deficits. Three patients (3.8%) presented with complications resulting in permanent neurological deficits due to a postoperative hematoma (1.2%) causing a hemiparesis, and 2 permanent ophthalmoplegias (2.5%). Seventeen patients (26.2%) have died of tumor progression over the course of follow-up (median 52 months, range 7–159 months). Based on Kaplan-Meier analysis, the survival rate was 77% at 5 years and 57% at 10 years. On multivariate analysis, the extent of tumor removal (p = 0.001) and the absence of previous treatments (p = 0.001) proved to be correlated with a longer survival rate.

CONCLUSIONS The EEA was associated with a high rate of tumor removal and symptom control, with low morbidity and preservation of a good quality of life. These results allow for a satisfactory overall survival rate, particularly after GTR and for primary surgery. Considering these results, the authors believe that an EEA can be a helpful tool in chordoma surgery, achieving a good balance between as much tumor removal as possible and the preservation of an acceptable patient quality of life.

Microscopic versus endoscopic approaches for craniopharyngiomas

microscopic-versus-endoscopic-approaches-for-craniopharyngiomas

Neurosurg Focus 41 (6):E5, 2016

Resection remains the mainstay of treatment for craniopharyngiomas with the goal of radical resection, if safely possible, to minimize the rate of recurrence. Endoscopic endonasal and microscopic transcranial surgical approaches have both become standard methods for the treatment for craniopharyngiomas. However, the approach selection paradigm for craniopharyngiomas is still a point of discussion. Choosing the optimal surgical approach can play a significant role in maximizing the extent of resection and surgical outcome while minimizing the risks of potential complications.

Craniopharyngiomas can present with a variety of different sizes, locations, and tumor consistencies, and each individual tumor has distinct features that favor one specific approach over another.

The authors review standard cranial base techniques applied to craniopharyngioma surgery, using both the endoscopic endonasal approach and traditional open microsurgical approaches, and analyze factors involved in approach selection. They discuss their philosophy of approach selection based on the location and extent of the tumor on preoperative imaging as well as the advantages and limitations of each surgical corridor, and they describe the operative nuances of each technique, using a personalized, tailored approach to the individual patient with illustrative cases and videos.

Endoscopic endonasal anatomy of the ophthalmic artery in the optic canal

Endoscopic endonasal anatomy of the ophthalmic artery in the optic canal

Acta Neurochir (2016) 158:1343–1350

The endoscopic endonasal opening of the optic canal has been recently proposed for tumors with medial invasion of this canal, such as tuberculum sellae meningiomas. Injury of the ophthalmic artery represents a dramatic risk during this maneuver. Therefore, the aim of this study was to analyze the endoscopic endonasal anatomy of the precanalicular and canalicular portion of this vessel, discussing its clinical implication.

Methods The course of the ophthalmic artery was analyzed through five endoscopic endonasal dissections, and 40 nonpathological consecutive MRAs were reviewed.

Results The ophthalmic artery arises from the intradural portion of the supraclinoid internal carotid artery, in 93%of cases about 1.9 mm (range: 1–3) posterior to the falciform ligament. At the entrance into the optic canal, the ophthalmic artery is located infero-medially to the optic nerve in 13 % of cases. In 50 % of these cases the artery moves infero-laterally along its course, remaining in a medial position in the others. In cases with an non medial entrance of the ophthalmic artery, it runs infero-lateral to the optic nerve for its entire canalicular portion, with just one exception.

Conclusion The endoscopic endonasal approach gives a direct, extensive and panoramic view of the course of the precanalicular and canalicular portion of the ophthalmic artery. Dedicated high-field neuroimaging studies are of paramount importance in preoperative planning to evaluate the anatomy of the ophthalmic artery, reducing the risk of jeopardizing the vessel, particularly for those uncommon cases with an infero-medial course of the artery.

Endoscopic endonasal treatment of 103 craniopharyngiomas

Endoscopic endonasal treatment of 103 craniopharyngiomas

J Neurosurg 121:100–113, 2014

Despite their benign histological appearance, craniopharyngiomas can be considered a challenge for the neurosurgeon and a possible source of poor prognosis for the patient. With the widespread use of the endoscope in endonasal surgery, this route has been proposed over the past decade as an alternative technique for the removal of craniopharyngiomas.

Methods. The authors retrospectively analyzed data from a series of 103 patients who underwent the endoscopic endonasal approach at two institutions (Division of Neurosurgery of the Università degli Studi di Napoli Federico II, Naples, Italy, and Division of Neurosurgery of the Bellaria Hospital, Bologna, Italy), between January 1997 and December 2012, for the removal of infra- and/or supradiaphragmatic craniopharyngiomas. Twenty-nine patients (28.2%) had previously been surgically treated.

Results. The authors achieved overall gross-total removal in 68.9% of the cases: 78.9% in purely infradiaphragmatic lesions and 66.3% in lesions involving the supradiaphragmatic space. Among lesions previously treated surgically, the gross-total removal rate was 62.1%. The overall improvement rate in visual disturbances was 74.7%, whereas worsening occurred in 2.5%. No new postoperative defect was noted. Worsening of the anterior pituitary function was reported in 46.2% of patients overall, and there were 38 new cases (48.1% of 79) of postoperative diabetes insipidus. The most common complication was postoperative CSF leakage; the overall rate was 14.6%, and it diminished to 4% in the last 25 procedures, thanks to improvement in reconstruction techniques. The mortality rate was 1.9%, with a mean follow-up duration of 48 months (range 3–246 months).

Conclusions. The endoscopic endonasal approach has become a valid surgical technique for the management of craniopharyngiomas. It provides an excellent corridor to infra- and supradiaphragmatic midline craniopharyngiomas, including the management of lesions extending into the third ventricle chamber. Even though indications for this approach are rigorously lesion based, the data in this study confirm its effectiveness in a large patient series.

Endoscopic endonasal surgery for craniopharyngiomas: surgical outcome in 64 patients

Endoscopic endonasal surgery for craniopharyngiomas

J Neurosurg 119:1194–1207, 2013

The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging and controversial management dilemmas in neurosurgery. Endoscopic endonasal surgery (EES) has recently been introduced as a treatment option for both pediatric and adult craniopharyngiomas. The object of the present study was to present the results of EES and analyze outcome in both the pediatric and the adult age groups.

Methods. The authors retrospectively reviewed the records of patients with craniopharyngioma who had undergone EES in the period from June 1999 to April 2011.

Results. Sixty-four patients, 47 adults and 17 children, were eligible for this study. Forty-seven patients had presented with primary craniopharyngiomas and 17 with recurrent tumors. The mean age in the adult group was 51 years (range 28–82 years); in the pediatric group, 9 years (range 4–18 years). Overall, the gross-total resection rate was 37.5% (24 patients); near-total resection (> 95% of tumor removed) was 34.4% (22 patients); subtotal resection (≥ 80% of tumor removed) 21.9% (14 patients); and partial resection (< 80% of tumor removed) 6.2% (4 patients). In 9 patients, EES had been combined with radiation therapy (with radiosurgery in 6 cases) as the initial treatment. Among the 40 patients (62.5%) who had presented with pituitary insufficiency, pituitary function remained unchanged in 19 (47.5%), improved or normalized in 8 (20%), and worsened in 13 (32.5%). In the 24 patients who had presented with normal pituitary function, new pituitary deficit occurred in 14 (58.3%). Nineteen patients (29.7%) suffered from diabetes insipidus at presentation, and the condition developed in 21 patients (46.7%) after treatment. Forty-four patients (68.8%) had presented with impaired vision. In 38 (86.4%) of them, vision improved or even normalized after surgery; in 5, it remained unchanged; and in 1, it temporarily worsened. One patient without preoperative visual problems showed temporary visual deterioration after treatment. Permanent visual deterioration occurred in no one after surgery. The mean follow-up was 38 months (range 1–135 months). Tumor recurrence after EES was discovered in 22 patients (34.4%) and was treated with repeat surgery (6 patients), radiosurgery (1 patient), combined repeat surgery and radiation therapy (8 patients), interferon (1 patient), or observation (6 patients). Surgical complications included 15 cases (23.4%) with CSF leakage that was treated with surgical reexploration (13 patients) and/or lumbar drain placement (9 patients). This leak rate was decreased to 10.6% in recent years after the introduction of the vascularized nasoseptal flap. Five cases (7.8%) of meningitis were found and treated with antibiotics without further complications. Postoperative hydrocephalus occurred in 7 patients (12.7%) and was treated with ventriculoperitoneal shunt placement. Five patients experienced transient cranial nerve palsies. There was no operative mortality.

Conclusions. With the goal of gross-total or maximum possible safe resection, EES can be used for the treatment of every craniopharyngioma, regardless of its location, size, and extension (excluding purely intraventricular tumors), and can provide acceptable results comparable to those for traditional craniotomies. Endoscopic endonasal surgery is not limited to adults and actually shows higher resection rates in the pediatric population.

Surgical nuances for nasoseptal flap reconstruction of cranial base defects with high-flow cerebrospinal fluid leaks after endoscopic skull base surgery

Neurosurg Focus 32 (6):E7, 2012. DOI: 10.3171/2012.5.FOCUS1255

Extended endoscopic endonasal approaches have allowed for a minimally invasive solution for removal of a variety of ventral skull base lesions, including intradural tumors. Depending on the location of the pathological entity, various types of surgical corridors are used, such as transcribriform, transplanum transtuberculum, transsellar, transclival, and transodontoid approaches. Often, a large skull base dural defect with a high-flow CSF leak is created after endoscopic skull base surgery. Successful reconstruction of the cranial base defect is paramount to separate the intracranial contents from the paranasal sinus contents and to prevent postoperative CSF leakage. The vascularized pedicled nasoseptal flap (PNSF) has become the workhorse for cranial base reconstruction after endoscopic skull base surgery, dramatically reducing the rate of postoperative CSF leakage since its implementation.

In this report, the authors review the surgical technique and describe the operative nuances and lessons learned for successful multilayered PNSF reconstruction of cranial base defects with high-flow CSF leaks created after endoscopic skull base surgery. The authors specifically highlight important surgical pearls that are critical for successful PNSF reconstruction, including target-specific flap design and harvesting, pedicle preservation, preparation of bony defect and graft site to optimize flap adherence, multilayered closure technique, maximization of the reach of the flap, final flap positioning, and proper bolstering and buttressing of the PNSF to prevent flap dehiscence. Using this technique in 93 patients, the authors’ overall postoperative CSF leak rate was 3.2%.

An illustrative intraoperative video demonstrating the reconstruction technique is also presented.

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