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.

Case report of primary dural lymphoma mimicking a cerebellar meningioma and brief review of literature.

Primary dural lymphoma (PDL) is an extremely rare subtype of primary central nervous system lymphoma arising from the dura mater in absence of systemic disease. The most common histological type is the low-grade marginal zone lymphoma, whereas high-grade lymphomas are unusual. We present a case of primary diffuse large B-cell lymphoma, presenting as PDL in the posterior fossa, originating from the dura mater of the petrous bone covering the surface of the left cerebellum, a location not previously described. A 65-year-old woman presented with sudden onset of severe dizziness was admitted in otolaryngology department then transferred to neurosurgery ward. CT scan revealed a large lesion involving left cerebellum, subsequent MRI of the brain demonstrated an enhancing mass suggestive for petrous bone meningioma. The tumor was excised, and the histopathological examination unexpectedly revealed a diffuse large B-cell lymphoma. The patient received postoperative chemoradiotherapy. 20 months after surgery a good outcome was registered. Due to the rarity of primary dural lymphomas no standard treatment is available, however, gross total or subtotal resection followed by adjuvant therapy seems to be a good choice to manage the pathology.

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.

Neuroendoscopic biopsy of ventricular tumors: a multicentric experience.

OBJECT: Although neuroendoscopic biopsy is routinely performed, the safety and validity of this procedure has been studied only in small numbers of patients in single-center reports. The Section of Neuroendoscopy of the Italian Neurosurgical Society invited some of its members to review their own experience, gathering a sufficient number of cases for a wide analysis. METHODS: Retrospective data were collected by 7 centers routinely performing neuroendoscopic biopsies over a period of 10 years. Sixty patients with newly diagnosed intraventricular and paraventricular tumors were included. No patient harboring a colloid cyst was included. Data regarding clinical presentation, neuroimaging findings, operative techniques, pathological diagnosis, postoperative complications, and subsequent therapy were analyzed. RESULTS: In all patients, a neuroendoscopic tumor biopsy was performed. In 38 patients (64%), obstructive hydrocephalus was present. In addition to the tumor biopsy, 32 patients (53%) underwent endoscopic third ventriculostomy (ETV), and 7 (12%) underwent septum pellucidotomy. Only 2 patients required a ventriculoperitoneal shunt shortly after the endoscopy procedure because ETV was not feasible. The major complication due to the endoscopy procedure was ventricular hemorrhage noted on the postoperative images in 8 cases (13%). Only 2 patients were symptomatic and required medical therapy. Infection occurred in only 1 case, and the other complications were all reversible. In no case did clinically significant sequelae affect the patient's outcome. Tumor types ranged across the spectrum and included glioma (low- and high-grade [27%]), pure germinoma (15%), pineal parenchymal tumor (12%), primary neuroectodermal tumor (4%), lymphoma (9%), metastasis (4%), craniopharyngioma (6%), and other tumor types (13%). In 10% of patients, the pathological findings were inconclusive. According to diagnosis, specific therapy was performed in 35% of patients: 17% underwent microsurgical removal, and 18% underwent chemotherapy or radiotherapy. CONCLUSIONS: This is one of the largest series confirming the safety and validity of the neuroendoscopic biopsy procedure. Complications were relatively low (about 13%), and they were all reversible. Neuroendoscopic biopsy provided meaningful pathological data in 90% of patients, making subsequent tumor therapy feasible. Cerebrospinal fluid pathways can be restored by ETV or septum pellucidotomy (65%) to control intracranial hypertension. In light of the results obtained, a neuroendoscopic biopsy should be considered a possible alternative to the stereotactic biopsy in the diagnosis and treatment of ventricular or paraventricular tumors. Furthermore, it could be the only surgical procedure necessary for the treatment of selected tumors.

Xanthomatous hypophysitis mimicking a pituitary adenoma: case report and review of the literature.

Background. Hypophysitis is an inflammatory disease of the pituitary gland that may mimic pituitary tumors clinically and radiologically. Case Description. We report a case of a xanthomatous hypophysitis initially diagnosed as pituitary adenoma. A 31-year-old woman presented with headache, diabetes insipidus, and amenorrhea. A head CT scan showed no intrasellar changes, while an MRI scan showed a sellar cystic mass. An endocrinological work up revealed mild hypocortisolism and diabetes insipidus (DI). Transsphenoidal surgery was performed. The intraoperative histological examination suggested a pituitary adenoma. The removed tissue showed central necrosis surrounded by accumulation of foamy cells and xanthomatous epithelioid cells. The patient made an uneventful postoperative recovery, Nevertheless, DI persisted and the adenohypophysis hypofunction did not recover. Conclusion. We describe an unusual inflammatory lesion of the pituitary gland mimicking an adenoma. A high level of clinical suspicion of inflammatory disorders is necessary for correct diagnosis and optimal management.

Application of neuroendoscopy to intraventricular lesions.

We present an overview of the history, development, technological advancements, current application, and future trends of cranial endoscopy. Neuroendoscopy provides a safe and effective management modality for the treatment of a variety of intracranial disorders, either tumoral or non-tumoral, congenital, developmental, and degenerative, and its knowledge, indications, and limits are fundamental for the armamentarium of the modern neurosurgeon.

Endoscopic third ventriculostomy in idiopathic normal pressure hydrocephalus: an Italian multicenter study.

OBJECTIVES: The aim of the report is to define the indications and results of endoscopic third ventriculostomy (ETV) in idiopathic normal pressure hydrocephalus and to discuss the physiopathological mechanism of this procedure. METHODS: The cases of 110 patients with idiopathic normal pressure hydrocephalus who underwent ETV in four Italian neurosurgical centers were retrospectively reviewed. The postoperative outcome was correlated with patient age, length of clinical history, preoperative clinical score, symptoms of clinical onset, type of hydrocephalus, and intraoperative findings. RESULTS: The follow-up period ranged from 2 to 12 years (average, 6.5 yr). The outcome evaluation was made 2 years after the procedure. Postoperative clinical improvement occurred in 76 (69.1%) of 110 patients. There was no correlation between success rate and patient age or type of ventricular enlargement (normal or enlarged fourth ventricle). Conversely, the rate of neurological improvement was higher in patients with shorter clinical history, better preoperative neurological score, and clinical onset with gait disturbances. Moreover, the intraoperative finding of the sudden reappearance of normal cerebral pulsations and significant downward and upward movements of the third ventricular floor after ETV was also correlated with a good outcome. CONCLUSION: ETV results in a relatively high rate of clinical improvement and a low complication rate in patients with idiopathic normal pressure hydrocephalus. Therefore, it may be easily performed with the same approach used for intracranial pressure monitoring with low morbidity. However, our data must be confirmed by additional studies.

Endoscope-controlled microneurosurgery for the treatment of intracranial fluid cysts.

OBJECTIVE: This study assessed the role of endoscope-controlled microneurosurgery (ECM) in the treatment of intracranial cerebrospinal fluid (CSF) cysts. METHODS: Twelve patients affected by symptomatic arachnoid cysts (11 in the sylvian fissure and 1 in the cerebellopontine angle) underwent ECM, in which the endoscope is used to view the operating field while microsurgical instruments can be passed alongside the endoscope for surgical maneuvers. All the cysts were superficially located and could be reached directly through a burr hole on their surface, so that the endoscope was inserted into the cyst without passing through the brain parenchyma. We used a rigid endoscope, performing a wide fenestration of the cyst in the basal cisterns (cystocisternostomy). The excellent endoscopic vision facilitated the surgical procedure with safe maneuvers through a minimally invasive approach. In 11 patients, we observed the resolution of symptoms. A radiological reduction in the cyst was observed in 7 cases. No mortality or major complications were observed. CONCLUSIONS: Endoscope-controlled microsurgery is a valid minimally invasive procedure for treating superficially located intracranial arachnoid cysts.