Septostomy and Monroplasty in Isolated Lateral Ventricle After Removal of a Third Ventricle Colloid Cyst: 2-Dimensional Operative Video.

Isolated unilateral hydrocephalus (IUH) is a condition caused by unilateral obstruction of the foramen of Monro.1 Etiopathogenic causes include tumors, congenital lesions, infective ventriculitis, intraventricular haemorrhage, and iatrogenic causes such as the presence of contralateral shunts.2,3 Neuroendoscopic management is considered the "gold-standard" treatment in IUH.4 Even if endoscopic septostomy and foraminoplasty in IUH are well-known procedures,5,6 IUH after an interhemispheric transcallosal transchoroidal approach for removal of a III ventricle colloid cyst is a complication barely described in literature. Video 1 describes this rare complication and the neuroendoscopic treatment adopted, including the operative room setup, patient's positioning, instrumentation needed, and a series of intraoperative tips for the performance of septostomy and Monroplasty via a single, precoronal burr hole. The scalp entry point and endoscope trajectory, homolateral to the dilated ventricle, were planned on the neuronavigation system. The avascular septal zone away from the septal veins and body of the fornix was reached, and the ostomy was performed. At the end of the procedure, Monroplasty was performed, too. The procedure was effective in solving the hydrocephalus and patient's clinical picture. No surgical complications occurred. Imaging demonstrated an evident and progressive reduction of enlarged lateral ventricle. In authors' opinion, the single burr-hole approach, ipsilateral to the enlarged ventricle, provides an optimal identification the intraventricular anatomy and allows Monroplasty to be performed, if deemed feasible during surgery. The patient consented to the procedure. The participants and any identifiable individuals consented to publication of their images.

Microsurgical transcranial approaches to the posterior surface of petrosal portion of the temporal bone: quantitative analysis of surgical volumes and exposed areas.

Different microsurgical transcranial approaches (MTAs) have been described to expose the posterior surface of the petrous bone (PPB). A quantitative, anatomical comparison of the most used MTAs, for specific areas of the PPB, is not available. Anatomical dissections were performed on five formalin-fixed, latex-injected cadaver heads (10 sides). Six MTAs were analyzed: Kawase approach (KWA), retrosigmoid approach (RSA), retrosigmoid approach with suprameatal extension (RSAS), retrolabyrinthine approach (RLA), translabyrinthine approach (TLA), and transcochlear approach (TCA). Surgical volumes and exposed areas of each approach were quantified with a dedicated neuronavigation system (ApproachViewer, part of GTx-Eyes II, University Health Network, Toronto, Canada) and adjuvant software (ITK-SNAP and Autodesk Meshmixer 3.5). Areas and volumes were compared using linear mixed models. TCA provided the best exposure of Trautmann's triangle and the retromeatal, suprameatal, meatal, and premeatal regions. RSAs provided the best exposure of the inframeatal region, with RSAS gaining significant exposure of the suprameatal region. KWA had the highest surgical volume, and RLA the lowest. Transpetrosal approaches offer the widest exposure of PPB proportionally to their invasiveness. Retrosigmoid approaches, which get to the studied region through a postero-lateral path, are paramount for the exposure of the inframeatal and suprameatal region and, given the adequate exposure of the remaining PPB, represent an effective approach for the cerebellopontine angle (CPA). These anatomical findings must be considered with approach-related morbidity and the pathological features in order to choose the most appropriate approach in clinical practice.

De novo cavernous angiomas associated with developmental venous anomaly: a mini-series and literature review.

BACKGROUND: Despite being previously considered as congenital lesions, recent studies agree to classify cerebral cavernous malformations (CCM) as acquired forms with clear correlations with other pathological affections of the central nervous system (CNS). In addition, a special subgroup, notably known as de novo CCMs (dnCCM), are associated in a significant number of cases with developmental venous anomalies (DVAs) and, in other cases, with Radiotherapy treatments. METHODS: A mini-series of 4 patients with clinical history characterized by developing dnCCM is reported. In three patients, the dnCCM was associated with the presence of an isolated DVA. In one case, no DVA was detected, but the patient underwent brain radiotherapy. In three cases, the dnCCM was clinically symptomatic, and the patients were submitted to a surgical procedure for lesion removal. In one case, the dnCCM was detected during MRI follow-up. RESULTS: Adding a review of the literature, we describe 47 patients who presented dnCCMs. The most common presentation is a sporadic CCM with a DVA, and the onset presentation was bleeding in 4 out of 47 cases (8.5%). Bleeding of dnCCM was observed in 9 out of 47 cases (19%), and the choice treatment was surgical in 24 out of 47 cases (51%). CONCLUSIONS: We present our series with a review of the recent literature and discuss the "de novo" cavernous malformation pathogenesis. A throughout review of recent literature is reported to clarify the predisposing factors that may lead to dnCCM development in patients carrying specific genetic and molecular features. Considering the high risk of bleeding, strict follow-up and aggressive treatment should be evaluated in dnCCM management.

Idiopathic normal pressure hydrocephalus: postoperative patient perspective and quality of life.

BACKGROUND: Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible disease. Surgical results have been well described in the literature, but only a few studies investigated the subjective outcome. This study aimed to investigate the patient's expectations about surgery, the perceived improvement after treatment, and its impact on the quality of life (QoL). METHODS: A new dedicated survey was created to investigate subjectively different aspects of the treatment pathway of iNPH (diagnosis, symptoms, expectations from surgery, surgical operation, surgical results, and postoperative QoL), together with the SF-12 and EQ-5D as validated, standardized tools. RESULTS: Forty-five patients were included. Forty-three percent of cases received the diagnosis after at least 1 year, with symptoms worsening in 73%, and frustration in 93%. Reaching a diagnosis was important for 100% of patients, with high expectations from surgery; 86% of them hoped to return to a normal life. Seventy-two percent of patients reported a significant postoperative improvement (walking 68%, mood 57%). Memory and incontinence did not improve in 64% of cases. Subjectively, QoL improved in 72% of cases. The SF-12 score is comparable to controls >75 years, but lower than the 65-75 years group. The EQ-5D index was 0.66 (lower than those of the 65-75 years group = 0.823, and >75 years group = 0.724). Pain and discomfort, instead, were lower compared to the healthy population (43% vs 56%). The idea of having an implanted device and of long-term follow-up is not worrying for 80% of patients; approximately two-thirds of them reported a regained control of their lives. CONCLUSIONS: The importance of early diagnosis and patients' perspective, alongside clinical evaluation, is highlighted. The self-reported evaluations on symptoms and QoL, along with the balance between postoperative worries and benefits, should be discussed preoperatively with patients and relatives, and included postoperatively to comprehensively assess the surgical outcome.

Preliminary Validation of FoRCaSco: A New Grading System for Cerebral and Cerebellar Cavernomas.

OBJECTIVE: Surgical indications for cerebral cavernous malformations (CCMs) remain significantly center- and surgeon-dependent; available grading systems are potentially limited, as they do not include epileptologic and radiologic data. Several experienced authors proposed a new grading system for CCM and the first group of patients capable of providing its statistical validation was analyzed. METHODS: A retrospective series of 289 CCMs diagnosed between 2008 and 2021 was collected in a shared anonymous database among 9 centers. The new grading system ranges from -1 to 10. For each patient with cortical and cerebellar cavernous malformations the grading system was applied, and a retrospective outcome analysis was performed. We proposed a score of 4 as a cutoff for surgical indication. RESULTS: Operated patients with a score ≥4 were grouped with non-operated patients with a score <4, as they constituted the group that received correct treatment according to the new grading system. Patients with a score ≥4, who underwent surgery and had an improved outcome, were compared to patients with a score ≥4 who were not operated (P = 0.04), and to patients with a score <4 who underwent surgery (P < 0.001). CONCLUSIONS: This preliminary statistical analysis demonstrated that this new grading would be applicable in surgical reality. The cutoff score of 4 correctly separated the patients who could benefit from surgical intervention from those who would not. The outcome analysis showed that the treated patients in whom the grading system has been correctly applied have a better outcome than those in whom the grading system has not been applied.

An international call for a new grading system for cerebral and cerebellar cavernomas.

Surgical indications for cerebral cavernous malformations remain significantly center- and surgeon-dependent. Available grading systems are potentially limited, as they do not include epileptological and radiological data. A novel grading system is proposed for supratentorial and cerebellar cavernomas: it considers neuroradiological features (bleeding, increase in size), neurological status (focal deficits and seizures), location of the lesion and age of the patient. The score ranges from -1 to 10; furthermore, surgery should be considered when a score of 4 or higher is present. Based on neuroradiological characteristics, 0 points are assigned if the CCM is stable in size at different neuroradiological controls, 1 point if there is an increase in volume during follow-up, 2 points if intra- or extra-lesional bleeding <1 cm is present and 3 points if the CCM produced a hematoma >1 cm. Regarding focal neurological deficits, 0 points are assigned if absent and 2 points if present. For seizures, 0 points are assigned if absent, 1 point if present, but controlled by medications, and 2 points if drug resistant. We considered the site of the CCM, and in case of deep-seated lesions in a critical area (basal ganglia, thalamus) 1 point (-1) is subtracted, while for subcortical or deep cerebellar lesions 0 points are assigned, for CCMs in a cortical critical area 1 point is assigned and in case of lesions in cortical not in critical area or superficial cerebellar area, 2 points are assigned. As far as age is concerned, 0 points are assigned for patients older than 50 years and 1 point for patients younger than 50. In conclusion, a novel grading for surgical decision making in cerebral cavernomas, based on the experience of selected neurosurgeons, basic scientists, and patients, is suggested with the aim of further improving and standardizing the treatment of CCMs. The aim of this paper was also to call for both retrospective and prospective multicenter studies with the aim of testing the efficacy of the grading system in different centers.

Choroid Plexus Cyst of the Fourth Ventricle Associated with Intermittent Obstructive Hydrocephalus.

BACKGROUND: Choroid plexus cysts (CPCs) are a type of neuroepithelial cysts, benign lesions located more frequently in the supratentorial compartment. Symptomatic CPCs in the posterior fossa are extremely rare and can be associated with obstructive hydrocephalus. CASE DESCRIPTION: A previously healthy elderly woman suffered intermittent attacks of headache and vomiting associated with gait instability. Magnetic resonance imaging documented a large cystic lesion occupying all the fourth ventricle. An endoscope-assisted fenestration of the lesion through a telovelar approach determined only temporary improvement, hence a second surgery with gross total resection of the cyst was performed, with successful long-term clinical and radiologic resolution. Histology revealed CPC. CONCLUSIONS: Fourth ventricle symptomatic CPCs are extremely rare lesions, especially in the elderly. Their presence must be carefully evaluated as a possible rare cause of intermittent obstructive hydrocephalus. Even though cyst fenestration with restoration of the cerebrospinal fluid pathway represents the best treatment in the majority of cases, a more aggressive resection is sometimes necessary.

Rathke's cleft cyst associated with pituitary granulomatosis with polyangiitis: An unusual combination of hypothalamus-pituitary region pathologies.

The authors present an unusual case of a patient suffering from visual deficit due to pituitary granulomatosis with polyangiitis (GPA) associated with Rathke's cleft cyst (RCC). The patient was referred to our Neurosurgery Department presenting right eye amaurosis, third cranial nerve palsy, and left temporal hemianopsia. Magnetic resonance imaging documented a sellar or suprasellar lesion with solid and cystic components. The dura mater of the skull base was also strongly enhanced. The patient underwent surgery. Histologic examination revealed RCC associated with pituitary GPA. To our knowledge, this is the first reported case of concomitant pituitary GPA and RCC. Pituitary involvement in GPA is rare, usually diagnosed in hormonal dysfunctions. The patient in case first presented optic chiasm compression, probably due to inflammation of both the pituitary gland and the previously asymptomatic RCC. We focus on the symptoms that led us to diagnose GPA pituitary involvement and on the peculiar and unusual Magnetic resonance imaging of the case presented.

Is Complex Sphenoidal Sinus Anatomy a Contraindication to a Transsphenoidal Approach for Resection of Sellar Lesions? Case Series and Review of the Literature.

BACKGROUND: The transsphenoidal approach is considered the gold standard for resection of pituitary adenomas and other sellar region lesions. This approach is guided by a few fundamental anatomic landmarks that conduct the surgeon toward the sellar floor. Some anatomic structures may vary a lot (e.g., intrasphenoidal septa, intercarotid distance) and may be difficult to identify. Pneumatization and conformation of the sphenoidal sinus (SS) plays a key role in accessing the floor of the sella and other skull base structures. A poorly pneumatized SS may be a relative contraindication to the transsphenoidal approach. We analyzed outcome and complications in transsphenoidal surgery for sellar lesions with a difficult SS. METHODS: We analyzed 243 consecutive patients who underwent a transsphenoidal approach for sellar lesions. Patients with poor pneumatization of the SS were included. Neurosurgical and endocrinologic outcomes were reported. RESULTS: Successful treatment using a transsphenoidal approach with neuronavigation and Doppler ultrasound was achieved in 15 patients with a low degree of pneumatization of the SS. A pituitary adenoma was present in 13 of 15 patients. Endocrinologic and neurosurgical outcomes were similar to patients with normal pneumatization of the SS, showing a cure of disease in 6 of 9 patients with functioning adenomas and an improvement of symptoms in cases of nonfunctioning adenomas. CONCLUSIONS: Patients with a poorly pneumatized SS can be treated safely with a transsphenoidal approach using image guidance techniques to avoid major neurovascular complications.

Third Ventriculostomy in Late-onset Idiopathic Aqueductal Stenosis Treatment: A Focus on Clinical Presentation and Radiological Diagnosis.

Endoscopic third ventriculostomy (ETV) is considered the gold standard treatment for obstructive hydrocephalus due to partial or complete obstruction of cerebrospinal fluid (CSF) ventricular pathways caused by mass lesions. However long-term efficacy of this procedure remains controversial as treatment of chronic adult hydrocephalus due to stenosis of Sylvian acqueduct [late-onset idiopathic aqueductal stenosis (LIAS)]. The authors describe clinical presentation, diagnostic investigations in patients affected by LIAS, and define their clinical and radiological outcome after ETV. From January 2003 to December 2008, 13 consecutive LIAS patients treated by ETV were retrospectively reviewed. Pre- and post-operative clinical and radiological findings, including conventional and phase-contrast (PC) cine magnetic resonance imaging (MRI) were investigated. ETV was successfully performed in all patients. Patient's neurological condition improved. No one required a second ETV procedure or shunt implantation. Clinical and radiological results reveal a satisfactory outcome of LIAS patients treated by ETV. At follow-up a clinical improvement could be demonstrated in all cases. Selection criteria of LIAS patients seem to be crucial to obtain satisfactory and long-lasting results. Even in elderly patients with chronic hydrocephalus, ETV can be considered the treatment of choice.

Percutaneous vertebral augmentation in metastatic disease: state of the art.

Improvements in diagnosis and treatment have prolonged cancer survival, with a consequent increase in the incidence of spinal metastases and vertebral compression fractures with associated axial pain, progressive radiculomyelopathy, and mechanical instability. Pain relief in malignant vertebral compression fractures is key to achieving a better quality of life in patients under palliative care. The gold standard for pain relief is nonsteroidal anti-inflammatory drugs and opioids. Nonresponsive cases are then treated with radiotherapy, which may require 2-4 weeks to take effect and in most cases does not provide complete pain relief. Percutaneous vertebroplasty and percutaneous kyphoplasty can in particular give relief in patients with vertebral body compression fractures that do not cause neurological deficits but severely compromise quality of life because of intractable pain.

Human glioblastoma tumours and neural cancer stem cells express the chemokine CX3CL1 and its receptor CX3CR1.

Human gliomas represent an unmet clinical challenge as nearly two-thirds of them are highly malignant lesions with fast progression, resistance to treatment and poor prognosis. The most severe form, the glioblastoma multiforme, is characterised by a marked and diffuse infiltration through the normal brain parenchyma. Given the multiple effects of chemokines on tumour progression, aim of this study was to analyse the expression of the chemokine CX3CL1 and of its specific receptor CX3CR1 in 36 human surgical glioma samples, with different degrees of histological malignancy and in glioblastoma-derived neurospheres. Herein we show that both ligand and receptor are expressed at the mRNA and protein levels in most specimens (31/36). While receptor expression was similarly detected in low or high grade tumours, the uppermost scores of CX3CL1 were found in grades III-IV tumours: oligodendrogliomas, anaplastic astrocytomas and glioblastomas. Accordingly, the expression of CX3CL1 was inversely correlated with patient overall survival (p = 0.01). Glioblastoma-derived neurospheres, containing a mixed population of stem and progenitor cells, were positive for both CX3CR1 and for the membrane-bound chemokine, which was further up-regulated and secreted after TNF-IFNγ stimulation. Confocal microscopy of 3D neurospheres showed that the ligand was primarily expressed in the outer layer cells, with points of co-localisation with CX3CR1, indicating that this ligand-receptor pair may have important intercellular adhesive functions. The high expression of CXC3L1 in the most severe forms of gliomas suggests the involvement of this chemokine and its receptor in the malignant behaviour of these tumours.