Predicting endoscopic third ventriculostomy success in adult hydrocephalus: preliminary assessment of a modified ETV success score for adults (ETVSS-A) in a series of 47 patients.

BACKGROUND: Endoscopic third ventriculostomy is an established treatment for non-communicating hydrocephalus. In carefully selected patients, it can be adopted for the management of communicating variant; however controversy exists in regards to the definition of the appropriate candidates. Predictive score of Endoscopic Third Ventriculostomy Success (ETVSS) has been reported for pediatric and mixed populations only. Our purpose was to define an ETV success score for adult population (ETVSS-A), measuring the strength of correlation between preoperative score retrospectively evaluated and the success rates achieved in a class of adult patients. METHODS: A retrospective analysis of 47 cases which received ETV procedure at our Institution between 2015 and 2018 was run. Demographic data,clinical history,preoperative and postoperative signs were reviewed and ETVSS-A was calculated.Thereafter ETVSS-A results were compared with the actual success rates. RESULTS: Twenty-nine patients (61.7%) presented unchanged or improved clinical status with a mean ETVSS-A of 54.5%; 18 patients (38.3%) worsened with mean ETVSS-A of 37.7%. We found that age, type of hydrocephalus and symptoms of admission are each apart important factors in predicting ETV success: older patients and those with non-obstructive hydrocephalus had the lowest predicted ETV success. In patients in whom ETV was actually successful, the preoperative ETVSS-A was significantly higher as compared to those patients in whom we observed a poor surgical outcome. CONCLUSIONS: From the results of this series, though small and retrospectively analyzed, it seems that ETVSS-A can be considered as a useful instrument to help neurosurgeon in predicting the ETV success and though define a more accurate surgical strategy in cases of hydrocephalus. Wider series and prospective studies are attended to validate these preliminary results.

Hydrocephalus onset after microsurgical or endovascular treatment for acute subarachnoid hemorrhage. Retrospective italian multicenter study.

BACKGROUND: Chronic shunt-dependent hydrocephalus is a complication of aneurysmal subarachnoid hemorrhage (aSAH). Its incidence and risk factors have been described while the hydrocephalus onset in terms of days after treatment (microsurgical or endovascular) has not been yet analyzed. MATERIALS AND METHODS: 45 patients, treated for aSAH in 4 Italian Neurosurgical Departments, were retrospectively analyzed. It was calculated the time that elapses between treatment and hydrocephalus onset in 36 patients. RESULTS: Of the 45 shunted patients, 15 (33.3%) were included in the microsurgical group (group A) and 30 (66.6%) were in the endovascular one (group B). There was no difference of the hydrocephalus onset between the two groups (24,1 days, group A vs. 27,7 days, group B). The presence of intracerebral hematoma (ICH) caused a delay in the hydrocephalus onset after endovascular treatment in terms of 11,5 days compared to microsurgical group as well the absence of vasospasm determined a delay of 13,7 days (not statistically significant). CONCLUSION: No difference in terms of hydrocephalus onset after microsurgical or endovascular treatment has been demonstrated. Only the presence of ICH or the absence of vasospasm can cause a slight delay in the time of hydrocephalus onset in the endovascular series (not statistically significant). Long-term follow-up studies involving higher numbers of subjects are needed to better demonstrate this issue.

Treatment of Chiari malformation: who, when and how.

There is no effective alternative to surgery in the treatment of the symptomatic cases of chiari malformation. Nonetheless, in literature there is no unanimous consensus about what is the surgical "gold standard" and which are the candidates for surgery. No doubt that intracranial hypertension and ventricular dilatation have to be investigated and treated before considering decompression. It is also very important to keep in mind that a surgery does not guarantee a complete recovery from every symptoms. We report our experience about who are the candidates for surgery, which is the most appropriate surgical technique and when is the correct time for surgery along the natural history of the disease.

Endoscopy versus microsurgical cyst excision and shunting for treating intracranial arachnoid cysts.

OBJECT: Endoscopic surgery is routinely used to treat intracranial arachnoid cysts. However, the indications and results with respect to the different cyst locations, compared with those of microsurgical fenestration and cyst shunting, deserve to be discussed. METHODS: The authors review 18 patients with intracranial arachnoid cysts treated by pure endoscopic technique in their neurosurgical department. There were 10 male and 8 female patients ranging in age from 2 months to 48 years (median age 19.4 years). The cyst location was suprasellar in 5 cases, quadrigeminal in 5, cortical hemispheric in 2, sylvian region in 3, and posterior fossa in 3. The authors also reviewed the literature, comprising 61 reports for an overall number of 645 patients with intracranial arachnoid cysts treated by different surgical techniques. These techniques included microsurgical excision or fenestration by craniotomy, cyst shunting, and endoscopic fenestration. The surgical results of the different techniques according to the different cyst locations underwent statistical analysis. RESULTS: The overall success rate (complete or partial clinical remission) in the authors' endoscopic series was 83.3% (15 of 18 cases), which is rather similar to that of 222 patients treated endoscopically and reported on in the literature (84.2%). In the overall endoscopic group, a higher success rate was found for cysts in the suprasellar (89.7%), quadrigeminal (88.5%), and posterior cranial fossa (83.3%) regions compared with sylvian (70%) and cortical and interhemispheric (75%) regions. The statistical comparison of the results of the endoscopic series with those of craniotomy and shunting revealed no significant differences for suprasellar, quadrigeminal, or posterior cranial fossa cysts, whereas the success rate of endoscopy is lower than that of other techniques for sylvian and cortical cysts. CONCLUSIONS: Endoscopy is a safe and effective therapeutic modality for patients with intracranial arachnoid cysts. Cysts of the suprasellar and quadrigeminal regions and posterior fossa are the best indications for neuroendoscopy; on the other hand, cortical cysts are best treated by microsurgical fenestration or shunting. For sylvian cysts, the endoscopic procedure may be advocated in most cases.

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.

Recurrent acute obstructive hydrocephalus as clinical onset of cerebral Whipple's disease.

Whipple's disease is a rare multisystemic infection caused by the intracellular bacteria Thropheryma whippelii. Central nervous system (CNS) involvement is not rare. The most frequent CNS manifestations are cognitive and behavioural changes, sopranuclear ophtalmoplegia, myoclonus, epilepsy, ataxia, meningitis and focal cerebral palsy. We report one case of cerebral localization of Whipple's disease with a clinical presentation of recurrent endocranic hypertension and hydrocephalus, and uncommon neurological symptoms, successfully treated by endoscopic third ventriculostomy and antibiotic therapy with ceftriaxone and Trimethoprim-Sulfamethoxazole.

Combined endoscopic and microsurgical removal of a giant cystic craniopharyngioma in a six-year-old boy.

Giant cystic craniopharyngiomas are rare lesions whose clinical and surgical management is extremely challenging, often requiring more than one craniotomy before obtaining a satisfying removal. We report one case of a giant cystic craniopharyngioma completely excised with a two-step combined use of a minimally invasive endoscopic approach followed by a single microsurgical transcranial procedure. A six-year-old boy presented with symptoms of increased intracranial pressure and posterior fossa involvement. Preliminary imaging revealed a large para- and suprasellar cystic tumor bulging superiorly into the third ventricle, and extending posteriorly from the retroclival region into the posterior fossa to the level of the foramen magnum. The suprasellar cystic quota was initially approached endoscopically through a right precoronal-transventricular approach and the cyst drained, while the remaining tumor was macroscopically totally removed one week later by a right pterional approach. A combined technique--endoscopic drainage followed by microsurgical removal--can be a valid alternative for the removal of giant cystic craniopharyngiomas in cases in which the cystic compartment bulges within the ventricular spaces, and may avoid multiple craniotomies.

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.

Clinical progression and familial occurrence of cerebral cavernous angiomas: the role of angiogenic and growth factors.

OBJECT: The authors studied the expression of angiogenic and growth factors and various proliferative indices in cavernous angiomas of the brain. The goal was to define whether the often progressive clinical course of both sporadic and familial forms of the lesion is correlated with different expression of these factors. METHODS: Forty-three cavernomas of the brain were investigated with immunohistochemical studies and stained for four growth factors (vascular endothelial growth factor [VEGF], tenascin, transforming growth factor-b [TGFb], and platelet-derived growth factor [PDGF]), and for Ki-67 and bcl-2. The intensity of expression was tested in all cases in the walls of cavernoma vessels, in the perivascular tissue, and in the perilesional brain parenchyma. Among the 43 cavernomas, 32 were stable and sporadic single lesions less than 2 cm in size, whereas 11 were cavernomas larger than 2 cm (up to 6 cm). These larger cavernomas had more aggressive behavior (documented growth in five cases, mass effect in eight, significant hemorrhage in four), familial occurrence (six cases), and/or multiple lesions (five cases). The expression of VEGF, tenascin, and PDGF in cavernomas did not significantly differ in the two groups of patients, whereas TGFb expression was higher in the more aggressive forms of cavernomas. The expression of Ki-67 and bcl-2 was always absent in stable lesions, and it was positive in eight (72.7%) of 11 aggressive lesions. The perilesional brain parenchyma showed a significantly higher expression of TGFb, PDGF, and tenascin in more aggressive cavernomas. CONCLUSIONS: The familial occurrence and more aggressive clinical behavior of cavernous angiomas of the brain are associated with higher expression of Ki-67 and bcl-2 in the cavernoma tissue, as in other proliferative lesions. These features are also associated with higher expression of some growth factors (excluding VEGF) in the perilesional brain parenchyma, suggesting that the neighboring vasculature and glia may be predisposed to and recruited for further growth and progression.

Endoscopic third ventriculostomy in idiopathic normal pressure hydrocephalus.

OBJECTIVE: To define the role and indications for an endoscopic third ventriculostomy (ETV) in patients with idiopathic normal pressure hydrocephalus (INPH). A series of 25 patients treated by endoscopic technique was analyzed, and the results were compared with those of 14 studies reporting patients treated by shunting. METHODS: Twenty-five patients with INPH were treated by ETV from January 1994 through December 2000. All were younger than 75 years of age, had a preoperative clinical history of 1 year or less, had prevalence of gait disturbance with scarce or mild dementia, had marked ventricular enlargement on magnetic resonance imaging (MRI), and had intracranial pressure values ranging from 8 to 12 mm Hg. All were studied by a phase-contrast MRI flow study 1 month after ETV. The 14 reviewed series of patients treated by shunting (all published after 1980) each include more than 25 patients, for a total of 777 patients. RESULTS: The overall rate of neurological improvement after ETV in our series was 72% (including two patients reoperated on because of absence of flow in the MRI scan); this percentage is slightly higher than that found in the 14 series of shunted patients (66%). Gait disturbance showed a high rate of improvement when compared with other symptoms, both in our ETV study and in other shunting series. Postoperative complications occurred only in one patient (4%) with an intracerebral frontal hemorrhage and in 37.9% of patients from the series including shunted patients. CONCLUSION: In patients with INPH showing short duration of symptoms, prevalence of gait disturbance, and slight mental impairment, ETV provides similar results to those of shunting. We suggest performing ETV in these patients and reserving shunting only for those who do not improve after ETV, despite the presence of cerebrospinal fluid flow through the ventriculostomy on MRI flow studies. The good results after ETV in our series indirectly confirm that the cerebrospinal fluid absorption is good or at least sufficient in selected patients with INPH.