Transcallosal approach for intraventricular gliomas: neuropsychological outcome and functionality of the corpus callosum.

The transcallosal approach is commonly used for surgery of lateral and third ventricle lesions. Cognitive deficits due to the transcallosal approach still remain controversial. Even if enormous efforts have been made in order to understand specific functions of the corpus callosum, still little is known. The present study was aimed to evaluate the neuropsychological results and the functionality of the corpus callosum in transferring visual, auditory and tactile information between the two hemispheres in a group of patients who were treated using the transcallosal approach. The study evaluated the neuropsychological status of five selected patients presenting low-grade lesions of lateral ventricles that had not previously undergone surgical treatments and that did not receive radiotherapy and chemotherapy. All patients were administered an extensive neuropsychological testing postoperatively and the interhemispheric transfer of visual, auditory and tactile information was also evaluated. Two patients were tested preoperatively. Incisions of 2.4 cm maximum of the corpus callosum length were operated. The postoperative cognitive profile was normal. In some patients, a postoperative subnormal performance in memory functions was found but it cannot be attributable to the surgical approach given that it was altered even pre-surgically. Small incisions of the corpus callosum preserved the integrity of this anatomical structure in transferring lateralized information between the two hemispheres. The transcallosal approach is a safe surgical route to lateral ventricles lesions and the neuropsychological evaluation of these cases could give new insights in the comprehension of corpus callosum functions.

Bobble-head doll syndrome: report of 2 cases and a review of the literature, with video documentation of the clinical phenomenon.

Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral ("no-no") involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

Speckle Tracking in Transcranial Ultrasound Allows Noninvasive Analysis of Pulsation Patterns of the Third Ventricle.

Cerebrospinal fluid (CSF) flow is sensitive to many cerebral disorders. We aimed to develop a noninvasive bedside method to detect physiological and pathological CSF phenomena by measuring pulsation patterns of the third ventricle. By transcranial B-mode ultrasound, electrocardiography (ECG)-gated video loops of the third ventricle were acquired. "Speckle tracking" software was used to quantify the relative change of its width. We conducted measurements of nine cardiac cycles in 11 healthy subjects in sitting and in supine position during Valsalva maneuver to investigate the influence of an increased intracranial pressure on the relative deformation of the third ventricle. In one patient with occlusive hydrocephalus, 19 cardiac cycles were measured in sitting position before and after removal of a tumorous obstruction of the aqueduct of Sylvius. Healthy subjects expressed a pulse-related increased width of the third ventricle ([Formula: see text]: +5.69, 95% confidence interval [CI] = [4.38, 7.00]). No significant difference was found between the sitting and the supine position in healthy adults. In the preoperative state of occlusive hydrocephalus, we found a negative, pulse-related deformation ([Formula: see text]: -1.86, 95% CI = [-2.15, -1.58]) with delayed onset. After surgery, the deformation pattern resembled that of our healthy controls. The difference between pre- and postoperative condition was significant (p < 0.001). Transcranial B-mode sonography can be used to record small movements of the sidewalls of the third ventricle. This noninvasive bedside method is suitable to assess CSF pulsatility within the third ventricle and might be able to distinguish between physiological and pathological flows.

Radiographic markers of clinical outcomes after endoscopic third ventriculostomy with choroid plexus cauterization: cerebrospinal fluid turbulence and choroid plexus visualization.

OBJECTIVE Endoscopic third ventriculostomy (ETV) with choroid plexus (CP) cauterization (CPC) represents a viable treatment option for congenital hydrocephalus in infants younger than 2 years. Imaging studies complement clinical data in the evaluation of treatment success or failure. The objectives of this study were to investigate novel radiographic markers-cerebrospinal fluid (CSF) turbulence and CP visualization-and their ability to reflect or predict clinical outcomes following ETV/CPC. METHODS Hydrocephalic patients younger than 2 years who were initially treated by ETV/CPC at the senior authors' institution between March 2013 and February 2014 were retrospectively reviewed. Clinical data, as well as the visualization of CSF turbulence and CP on pre- and postoperative fast-sequence MRI, were recorded. Radiographic images were reviewed by a blinded observer based on specific criteria for the visualization of CSF turbulence and CP. Data were collected and analyzed using descriptive statistics, including Fisher's exact test for comparisons. The research team obtained appropriate institutional review board approval for this study, without the need for informed consent. RESULTS Among the 32 patients (53% male and 47% female) studied, 18 of 32 (56%) responded favorably to initial or repeat ETV/CPC, with 13 of 32 (41%) patients requiring 1 surgery. Of the 19 (59%) patients whose initial ETV/CPC failed, 8 of 19 (42%) patients underwent repeat ETV/CPC, with 5 of 8 (63%) patients responding favorably. Radiographic CSF turbulence appeared more frequently following ETV/CPC failure than after ETV/CPC success (55% vs 18%, respectively; p = 0.02). The sensitivity and specificity of CSF turbulence as a radiographic marker for ETV/CPC failure were 80% and 58%, respectively. The radiographic depiction of CP disappearance following ETV/CPC from pre- to postoperative imaging occurred in 20 of 30 patients (67%). Among the patients who responded unsuccessfully to ETV/CPC and ultimately required secondary shunt insertion, 71% (10 of 14 patients) demonstrated CP persistence on postoperative imaging. In contrast, 6% (1 of 18) of patients who were treated successfully by ETV/CPC demonstrated the presence of CP on follow-up imaging. This difference reached statistical significance (p = 0.0001). The visualization of CP persistence despite ETV/CPC reflected treatment failure with 91% sensitivity and 81% specificity. The sensitivity of either or both radiographic markers to suggest ETV/CPC failure was 77%, while their specificity (both markers absent, thereby indicating ETV/CPC success) was 81%. CONCLUSIONS Radiographic markers correlate with clinical outcomes following the treatment of infantile hydrocephalus with ETV/CPC. Specifically, CSF turbulence may indicate ongoing pathological CSF flow dynamics, while CP absence following ETV/CPC may predict shunt independence. Future studies that incorporate prospective review and formal intra- and interobserver reliability estimates may help corroborate the utility of these radiographic markers.

Takotsubo cardiomyopathy in the setting of acute hydrocephalus secondary to neurocysticercosis.

We report the case of a 45-year-old male who presented with transient neurogenic stunned myocardium, or takotsubo cardiomyopathy, secondary to acute hydrocephalus caused by obstruction of the third ventricle by neurocysticercosis.

Symptomatic colloid cysts in the third ventricle of monozygotic twins.

In this case report we describe colloid cysts in the third ventricles of monozygotic twin sisters. They were 26 years old when their condition was discovered. One woman was admitted to us on an emergency basis, with signs of high intracranial pressure such as unconsciousness and extension posturing. Her sister was also brought to the hospital since she had a history of attacks of headache. They were both operated with removal of the colloid cysts, and the clinical courses are described in the case report. In reviewing the literature another 30 familial cases were found. Of these were two pairs of monozygotic and one pair of dizygotic twins.

CSF dynamics analysis in patients with post-traumatic ventriculomegaly.

OBJECTIVE: The management of post-traumatic ventriculomegaly (PTV) is controversial. This is due to the difficulty to determine whether PTV is related to an atrophic process or to a true "active" hydrocephalus. The purpose of this study is to analyze the CSF-dynamics in patients with PTV and, possibly, to identify parameters that correlate with prognosis. METHODS: 15 patients with PTV were treated following this protocol: 1-frontal ventriculostomy (with Rickham reservoir); 2-CSF-dynamics evaluation; 3-ventriculo-peritoneal shunt. CSF dynamics evaluation was based on an intraventricular infusion test (performed three to five days after ventriculostomy). Outflow Resistance (R-out) and Intracranial Elastance Index (EI, i.e. the reciprocal of intracranial compliance) were calculated. Patients were classified according to response to shunt into: 1-fast responders: rapid clinical improvement, i.e. within days/one month from surgery; 2-slow responders: patients presenting little clinical improvement occurring after months (despite neurorehabilitation); 3-non responders: no clinical improvement. RESULTS: Seven patients (46.7%) were classified as fast-responders, three patients were classified as slow-responders (20%) and five patients were classified as non-responders (33.3%). Opening CSF pressure was less than 15 mmHg for all patients. R-out (cut-off >10 mmHg/ml/min) had 100% sensitivity, 50% specificity, 100% negative predictive value and 63.6% positive predictive value. EI (cut-off value >0.3) had 100% specificity, 42.4% sensitivity, 100% positive predictive value and 66.7% negative predictive value. CONCLUSIONS: Based on these considerations, we can suggest that, for patients with normal pressure PTV, analysis of CSF dynamics could be of help in selecting patients for CSF-shunt. A combination of Intracranial Elastance and of R-out could help predicting shunt responsiveness.

Quiste coloide del tercer ventrículo / Colloid cyst of the third ventricle

Los quistes coloides son lesiones intracraniales con una incidencia calculada en tres individuos por millón de personas en un año. Son tumores benignos de la porción anterosuperior del tercer ventrículo. El flujo normal del líquido cefalorraquídeo puede ser interrumpido por un gran quiste que obstruya el foramen de Monro. Los signos y síntomas asociados engloban un espectro amplio de características, desde una cefalea o específica hasta datos de hipertensión intracraneal; algunos quistes coloides se dan en el comienzo agudo de una hidrocefalia y pueden llevar a una muerte súbita. Caso: presentamos el caso de un adolescente masculino de 13 años de edad, de raza negra, quien dos días antes de su fallecimiento había manifestado cefalea. Al examen de autopsia se evidenció un quiste coloide del tercer ventrículo que produjo una hidrocefalia aguda obstructiva, con edema cerebral severo y herniación de amígdalas cerebelosas. En este artículo comentamos las características principales del quiste coloide y realizamos una breve revisión bibliográfica...

[Identification of highly specific electroencephalographic patterns in patients with neuroepithelial tumors of the III ventricle by algorithmic methods].

An algorithm was suggested for identifying highly specific electroencephalographic (EEG) patterns in neurooncologic patients. The algorithm provides selection of patients with their further classification into main and control groups based on the already existing database of EEG indicators; requests to it; generation of mono-indicator candidates for EEG-patterns on the basis of a 4-dipole table for selecting and verifying sensitive and specific EEG patterns and outlining the best ones. Our material included 368 patients with basal-diencephalic tumors. Algorithmic methods revealed new EEG patterns in patients with different anatomical and topographical variants of neuroepithelial tumors in the III ventricle. We think it reasonable to use the revealed syndromes to improve diagnosis and identify pathophysiological basis of clinical syndromes.

New ependymal cells are born postnatally in two discrete regions of the mouse brain and support ventricular enlargement in hydrocephalus.

A heterogeneous population of ependymal cells lines the brain ventricles. The evidence about the origin and birth dates of these cell populations is scarce. Furthermore, the possibility that mature ependymal cells are born (ependymogenesis) or self-renewed (ependymal proliferation) postnatally is controversial. The present study was designed to investigate both phenomena in wild-type (wt) and hydrocephalic α-SNAP mutant (hyh) mice at different postnatal stages. In wt mice, proliferating cells in the ventricular zone (VZ) were only found in two distinct regions: the dorsal walls of the third ventricle and Sylvian aqueduct (SA). Most proliferating cells were monociliated and nestin+, likely corresponding to radial glial cells. Postnatal cumulative BrdU-labeling showed that most daughter cells remained in the VZ of both regions and they lost nestin-immunoreactivity. Furthermore, some labeled cells became multiciliated and GLUT-1+, indicating they were ependymal cells born postnatally. Postnatal pulse BrdU-labeling and Ki-67 immunostaining further demonstrated the presence of cycling multiciliated ependymal cells. In hydrocephalic mutants, the dorsal walls of the third ventricle and SA expanded enormously and showed neither ependymal disruption nor ventriculostomies. This phenomenon was sustained by an increased ependymogenesis. Consequently, in addition to the physical and geometrical mechanisms traditionally explaining ventricular enlargement in fetal-onset hydrocephalus, we propose that postnatal ependymogenesis could also play a role. Furthermore, as generation of new ependymal cells during postnatal stages was observed in distinct regions of the ventricular walls, such as the roof of the third ventricle, it may be a key mechanism involved in the development of human type 1 interhemispheric cysts.

Endoscopic third ventriculostomy in patients with malfunctioning CSF-shunt.

OBJECTIVE: A less favorable outcome is usually claimed for ETV in formerly shunted patients, and continuous bias exists on using endoscopy in cases with malfunctioning CSF shunts. METHODS: A cohort of 60 patients with obstructive triventricular hydrocephalus (mean age 22 years, range 1-68) underwent an ETV instead of shunt revision. Fourteen patients had a history of multiple shunt-related surgeries (more than three times). Median follow-up lasted 2 years (range 1 month-8 years). Data on patients' preoperative condition and their history, including particularities of the surgery, were studied to define the impact of any given variable on the outcome. The Mann-Whitney U test was used to assess differences among groups. RESULTS: Sixteen patients did not improve and needed permanent shunts anyway. The remaining 44 patients improved and became free of shunt (72%). No reliable correlation has been found regarding final outcome and data, characterizing patients' profile, for example, etiology of hydrocephalus, the history of intraventricular bleeding and/or CNS infection, age at onset and age at the first shunting, number of shunt surgeries, the origin of shunt malfunction, and complicated ventricular anatomy. There were no deaths, and overall cases with morbidity comprised 20% (12 cases); among them, serious complications with neurologic deficit were noted in three (5%) patients. CONCLUSIONS: Patients with obstructive hydrocephalus could benefit from ETV in case of their shunt malfunction and if carefully selected have about 70% probability to become shunt free. In formerly shunted patients, endoscopy has somewhat greater risk of serious complications; thus a wider experience is essential when offering them an ETV.

Endoscopic treatment of intraventricular cerebrospinal fluid cysts: 10 consecutive cases.

INTRODUCTION: The use of the endoscope for lesions of the central nervous system has been progressively widened in the past decades. Among these lesions, the intraventricular cyst is one of the most attractive targets for this less invasive procedure. METHODS: Between 2003 and 2007, ten consecutive patients with IVC underwent endoscopic surgery in our department. The location of the cyst was the lateral ventricle in nine, the lateral and third ventricles in one. The cyst was resected or fenestrated according to the degree of adhesion of the cyst wall with the ventricular wall. The follow-up ranged from 6 months to 54 months (mean: 22.5 months). RESULTS: The cyst was totally removed in three, subtotally removed in one, and fenestrated in six cases. Except for transient fever, there was no post-operative morbidity. During follow-up, all patients were doing well. CONCLUSION: The endoscopic technique is a good treatment option with the advantage of minimal invasiveness and less complications. The extension of the cyst and whether the hemisphere involved is dominant or not, determines the ideal endoscopic trajectory. The long-term efficacy of the endoscopic technique in treatment of IVC needs further evaluation.

Intraventricular fibrinolysis and lumbar drainage for ventricular hemorrhage.

BACKGROUND AND PURPOSE: Both intraventricular fibrinolysis (IVF) and lumbar drainage (LD) may reduce the need for exchange of external ventricular drainage (EVD) and shunt surgery in patients with intracerebral hemorrhage and severe intraventricular hemorrhage. We investigated the feasibility and safety of IVF followed by early LD for the treatment of posthemorrhagic hydrocephalus. METHODS: This prospective study included patients with spontaneous ganglionic intracerebral hemorrhage and severe intraventricular hemorrhage with acute obstructive posthemorrhagic hydrocephalus who received an EVD (n=32). The treatment algorithm started with IVF (4 mg recombinant tissue plasminogen activator every 12 hours) until clearance of the third and fourth ventricles from blood. Thereupon, EVD was clamped and if clamping was unsuccessful, communicating posthemorrhagic hydrocephalus was assumed and LD placed. EVD was removed if there was neither an increase of intracranial pressure nor ventricle enlargement on CT. A ventriculoperitoneal shunt was indicated if "LD weaning" was unsuccessful for >10 days. Outcome was assessed at 90 and 180 days using the modified Rankin Scale. RESULTS: IVF resulted in fast clearance of the third and fourth ventricles (73+/-50 hours). However, early EVD removal was only possible in 4 patients. The remaining 28 patients developed communicating posthemorrhagic hydrocephalus. In all of these patients, early LD was capable to replace EVD. EVD exchange was not necessary and EVD duration was 105+/-59 hours. Only one patient required a ventriculoperitoneal shunt. At 180 days, 20 (62.5%) patients had a good (modified Rankin Scale 0 to 3) outcome and 5 (15.6%) patients had died. One patient had asymptomatic ventricular rebleeding. CONCLUSIONS: In patients with secondary intraventricular hemorrhage and posthemorrhagic hydrocephalus, the combined treatment approach of IVF and early LD is safe and feasible, avoids EVD exchange, and may markedly reduce the need for shunt surgery.

Endoscopic third ventriculostomy for malfunction in previously shunted infants.

INTRODUCTION: The usage of endoscopic third ventriculostomy (ETV) as an alternative to shunt revision in the management of shunt malfunction is gaining popularity. METHODS: We review the clinical data of 45 patients who underwent ETV because of ventriculopritoneal shunt malfunction at Hacettepe University School of Medicine Department of Neurosurgery between January 2002 and August 2007. Medical records of the patients were retrospectively studied. RESULTS: Male-to-female ratio was 23/22. The cause of the hydrocephalus was aqueduct stenosis in 21 (46.9%) patients, newborn meningitis in nine (20%) patients, tumor in six (13.3%) patients, newborn intraventricular hemorrhage in four (8.8%) patients, myelomeningocele in three (6.6%), and trauma in two (2.2%) patients. Of the patients, 27 (60%) had triventricular and 18 (40%) had tetraventricular hydrocephalus at their radiologic evaluation. On admission, all patients had at least one episode of shunt dysfunction prior to ETV. Follow-up duration after surgery was 1-5 years (mean 2.46 +/- 1.64 years). Postoperative cerebrospinal fluid flow studies using the cine-PC MR imaging were performed on all patients. The overall success rate for ETV after shunt malfunction was 80% with 36 patients and failure rate was 20% with nine patients. All of these nine patients had undergone shunt insertion within 10 days-1 month after unsuccessful ETV. CONCLUSION: Endoscopic third ventriculostomy is an effective treatment for shunt malfunction.

Rhinorrhoea from a frontal encephalocele after reduction of high intracranial pressure.

Acquired non-traumatic frontal sinus encephaloceles are very rare lesions that are usually caused by a tumour or hydrocephalus. We present a 31-year-old woman with a frontal sinus encephalocele who developed rhinorrhoea after a ventriculo-peritoneal shunt to treat her hydrocephalus and underwent radiotherapy for a tectum tumour.

Chordoid glioma of the third ventricle.

BACKGROUND: Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle with both glial and chordoid features. It was first described by Brat in 1998. Since there is no detailed information available on the outcome after surgery and adjuvant treatment, we reviewed the literature. METHODS: A literature search through PUBMED revealed 50 cases of chordoid glioma. Most reports were found in pathology journals. Information on the postoperative course was sometimes very limited. We reviewed the available literature and studied in detail the presenting symptoms, mortality and postoperative complications in relation to the extent of resective surgery, as well as the importance of adjuvant treatment. CONCLUSIONS: Mortality in the immediate postoperative period is 32% and is higher after gross total resection as compared to subtotal resection. Non-fatal postoperative complications are hypothalamic disorders and mental alterations. Gross total resection is the treatment of choice since no recurrence has been reported after macroscopically complete resection, but this is often difficult because of the location and adherence to the hypothalamus. The role of postoperative radiotherapy is uncertain. There is some indication that radiosurgery with or without conventional irradiation is superior to conventional radiation alone. Planned subtotal resection followed by stereotactic radiosurgery can be a safe and effective alternative in a patient in whom gross total resection is considered to be too risky. There is no report on the use of chemotherapy in the treatment of chordoid gliomas. More information about the optimal treatment strategy is needed, and more reports are also needed.

Long-term outcome of endoscopic third ventriculostomy in obstructive hydrocephalus.

This multicentric study reports on 140 patients who underwent endoscopic third ventriculostomy for obstructive hydrocephalus in four Italian neurosurgical centers between 1994 and 1999. Its aim is to define the long-term outcome of these patients many years (6-12) after the initial procedure. The study includes both children and adults; the etiology of the hydrocephalus was malformative aqueductal stenosis in 88 cases (62.8%), compression by tumors of the mesencephalic and pineal regions and posterior fossa in 45 (32.2%) and post-infection aqueductal stenosis in 7 (5%). The ETV was performed by using the standard technique. The overall rate of good results (shunt-independent patients with clinical remission or improvement) was 87.1%. Eighteen patients (12.9%) required a shunt because of ETV failure. The long-term outcome of ETV in this study was not influenced by the patient's age and the etiology of the hydrocephalus (although cases secondary to cisternal hemorrhage and infections are not included). Other series including cases with long follow-up are analyzed. In conclusion, ETV results in a high rate of good long-term outcome in patients with obstructive hydrocephalus. Because postoperative failures occur early, clinical and radiological control studies must be performed particularly in the first years after the neuroendoscopic procedure.

Endoscopic treatment of in utero diagnosed suprasellar arachnoid cyst and development of salt wasting.

Suprasellar arachnoid cysts are rare lesions that constitute approximately 5-12.5% of all arachnoid cysts. We present a male infant diagnosed in utero with suprasellar arachnoid cyst and treated with endoscopic ventriculocystocisternotomy on the 38th day of life. The patient developed hyponatremia as a result of cerebral salt wasting after the operation.