Pathophysiology of long-standing overt ventriculomegaly in adults

2000 ◽  
Vol 92 (6) ◽  
pp. 933-940 ◽  
Author(s):  
Shizuo Oi ◽  
Masami Shimoda ◽  
Masayoshi Shibata ◽  
Yumie Honda ◽  
Kouji Togo ◽  
...  

Object. Long-standing overt ventriculomegaly in adults (LOVA) is a unique form of hydrocephalus that develops during childhood and manifests symptoms during adulthood. The aim of the present study was to analyze the specific pathophysiological characteristics of LOVA.Methods. The specific diagnostic criteria for LOVA include severe ventriculomegaly in adults that is associated with macrocephalus measuring more than two standard deviations in head circumference and/or neuroradiological evidence of a significantly expanded or destroyed sella turcica. Twenty patients who fulfilled these criteria, 14 males and six females, were retrospectively studied. These patients' ages at diagnosis ranged from 15 to 61 years (mean 39.4 years). All had symptoms and/or signs indicating that hydrocephalus first occurred at birth or during infancy in the absence of any known underlying disease. The authors performed a pathophysiological study that included specific variations of magnetic resonance (MR) imaging, such as fluid-attenuated inversion recovery and cardiac-gated cine-mode imaging; intracranial pressure (ICP) monitoring; three-dimensional computerized tomography (CT) scanning; and other techniques.Hydrocephalus was caused by aqueductal stenosis in all patients. Severe ventriculomegaly involving the lateral and third ventricles was associated with a marked expansion or destruction of the sella turcica in 17 cases. Cardiac-gated cine-MR imaging did not reveal any significant movements of cerebrospinal fluid in the aqueduct. Three-dimensional CT ventriculography confirmed that the expanded third ventricle protruded into the sella and, sometimes, extended a diverticulum. Fourteen patients revealed symptoms and signs that indicated increased ICP with prominent pressure waves. Dementia or mental retardation was seen in 11 patients, gait disturbance in 12, and urinary incontinence in eight; all three of these symptoms were observed in seven patients. Thirteen patients experienced visual disturbance. Nine patients underwent ventriculoperitoneal shunt implantation as the initial treatment, leading to postoperative subdural hematoma in all seven cases in which a differential pressure valve was used. Nine patients, three of whom were initially treated by shunt placement, underwent a neuroendoscopic procedure, mainly for third ventriculostomy. Postoperatively, ICP returned to normal, and marked to-and-fro pulsatile movements at the site of ventriculostomy were recognized on cine-MR imaging in patients treated endoscopically. However, the ventriculomegaly was little improved. Consequently, all patients eventually demonstrated improvement in response to either a shunt equipped with a pressure-programmable valve or an endoscopic procedure; however, depression appeared in six patients, who required psychiatric consultation or medication.Conclusions. Such remarkably decreased intracranial compliance but relatively high ICP dynamics are the pathophysiological characteristics of LOVA. The therapeutic regimen should be determined based on the individual's specific pathophysiological makeup.

1999 ◽  
Vol 90 (3) ◽  
pp. 448-454 ◽  
Author(s):  
Giuseppe Cinalli ◽  
Christian Sainte-Rose ◽  
Paul Chumas ◽  
Michel Zerah ◽  
Francis Brunelle ◽  
...  

Object. The goal of this study was to analyze the types of failure and long-term efficacy of third ventriculostomy in children.Methods. The authors retrospectively analyzed clinical data obtained in 213 children affected by obstructive triventricular hydrocephalus who were treated by third ventriculostomy between 1973 and 1997. There were 120 boys and 93 girls. The causes of the hydrocephalus included: aqueductal stenosis in 126 cases; toxoplasmosis in 23 cases, pineal, mesencephalic, or tectal tumor in 42 cases; and other causes in 22 cases. In 94 cases, the procedure was performed using ventriculographic guidance (Group I) and in 119 cases by using endoscopic guidance (Group II). In 19 cases (12 in Group I and seven in Group II) failure was related to the surgical technique. Three deaths related to the technique were observed in Group I. For the remaining patients, Kaplan—Meier survival analysis showed a functioning third ventriculostomy rate of 72% at 6 years with a mean follow-up period of 45.5 months (range 4 days–17 years). No significant differences were found during long-term follow up between the two groups. In Group I, a significantly higher failure rate was seen in children younger than 6 months of age, but this difference was not observed in Group II. Thirty-eight patients required reoperation (21 in Group I and 17 in Group II) because of persistent or recurrent intracranial hypertension. In 29 patients shunt placement was necessary. In nine patients in whom there was radiologically confirmed obstruction of the stoma, the third ventriculostomy was repeated; this was successful in seven cases. Cine phase-contrast (PC) magnetic resonance (MR) imaging studies were performed in 15 patients in Group I at least 10 years after they had undergone third ventriculostomy (range 10–17 years, median 14.3 years); this confirmed long-term patency of the stoma in all cases.Conclusions. Third ventriculostomy effectively controls obstructive triventricular hydrocephalus in more than 70% of children and should be preferred to placement of extracranial cerebrospinal shunts in this group of patients. When performed using ventriculographic guidance, the technique has a higher mortality rate and a higher failure rate in children younger than 6 months of age and is, therefore, no longer preferred. When third ventriculostomy is performed using endoscopic guidance, the same long-term results are achieved in children younger than 6 months of age as in older children and, thus, patient age should no longer be considered as a contraindication to using the technique. Delayed failures are usually secondary to obstruction of the stoma and often can be managed by repeating the procedure. Midline sagittal T2-weighted MR imaging sequences combined with cine PC MR imaging flow measurements provide a reliable tool for diagnosis of aqueductal stenosis and for ascertaining the patency of the stoma during follow-up evaluation.


2000 ◽  
Vol 93 (3) ◽  
pp. 509-512 ◽  
Author(s):  
Philippe Decq ◽  
Caroline Le Guerinel ◽  
Stéphane Palfi ◽  
Michel Djindjian ◽  
Yves Kéravel ◽  
...  

✓ Since its description by Dandy in 1922, several techniques have been used to perform third ventriculostomy under endoscopic control. Except for the blunt technique, in which the endoscope is used by itself to create the opening in the floor of the third ventricle, the other techniques require more than one instrument to perforate the floor of the ventricle and enlarge the ventriculostomy. The new device described is a sterilizable modified forceps that allows both the opening of the floor and the enlargement of the ventriculostomy in a simple and effective way.The new device has the following characteristics: 1) the tip of the forceps is thin enough to allow the easy perforation of the floor of the ventricle; 2) the inner surface of the jaws is smooth to avoid catching vessels of the basal cistern; and 3) the outer surface of the jaws has indentations that catch the edges of the opening to prevent them from slipping along the instrument's jaws. The ventricle floor is opened by gentle pressure of the forceps, which is slowly opened so that the edges of the aperture are caught by the distal outer indentation of the jaws, leading to an approximately 4-mm opening of the floor. This device has been used successfully in 10 consecutive patients.This new device allows surgeons to perform third ventriculostomy under endoscopic control in a very simple, quick, and effective way, avoiding the need for additional single-use instruments.


1999 ◽  
Vol 91 (4) ◽  
pp. 553-562 ◽  
Author(s):  
John D. Heiss ◽  
Nicholas Patronas ◽  
Hetty L. DeVroom ◽  
Thomas Shawker ◽  
Robert Ennis ◽  
...  

Object. Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy.Methods. The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing.Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressure and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape.Conclusions. The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-1 laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.


2000 ◽  
Vol 93 (supplement_3) ◽  
pp. 191-192 ◽  
Author(s):  
Lee Walton ◽  
Anna Hampshire ◽  
Paul Vaughan ◽  
David M. C. Forster ◽  
Andras A. Kemeny ◽  
...  

✓ The purpose of this paper was to note a potential source of error in magnetic resonance (MR) imaging. Magnetic resonance images were acquired for stereotactic planning for GKS of a vestibular schwannoma in a female patient. The images were acquired using three-dimensional sequence, which has been shown to produce minimal distortion effects. The images were transferred to the planning workstation, but the coronal images were rejected. By examination of the raw data and reconstruction of sagittal images through the localizer side plate, it was clearly seen that the image of the square localizer system was grossly distorted. The patient was returned to the MR imager for further studies and a metal clasp on her brassiere was identified as the cause of the distortion.


2005 ◽  
Vol 103 (5) ◽  
pp. 848-852 ◽  
Author(s):  
Mahmoud Hamdy Kamel ◽  
Michael Kelleher ◽  
Kristian Aquilina ◽  
Chris Lim ◽  
John Caird ◽  
...  

Object. Neuroendoscopists often note pulsatility or flabbiness of the floor of the third ventricle during endoscopic third ventriculostomy (ETV) and believe that either is a good indication of the procedure's success. Note, however, that this belief has never been objectively measured or proven in a prospective study. The authors report on a simple test—the hydrostatic test—to assess the mobility of the floor of the third ventricle and confirm adequate ventricular flow. They also analyzed the relationship between a mobile floor (a positive hydrostatic test) and prospective success of ETV. Methods. During a period of 3 years between July 2001 and July 2004, 30 ETVs for obstructive hydrocephalus were performed in 22 male and eight female patients. Once the stoma had been created, the irrigating Ringer lactate solution was set at a 30-cm height from the external auditory meatus, and the irrigation valve was opened while the other ports on the endoscope were closed. The ventricular floor ballooned downward and stabilized. The irrigation valve was then closed and ports of the endoscope were opened. The magnitude of the upward displacement of the floor was then assessed. Funneling of the stoma was deemed to be a good indicator of floor mobility, adequate flow, and a positive hydrostatic test. All endoscopic procedures were recorded using digital video and recordings were subsequently assessed separately by two blinded experienced neuroendoscopists. Patients underwent prospective clinical follow up during a mean period of 11.2 months (range 1 month–3 years), computerized tomography and/or magnetic resonance imaging studies of the brain, and measurements of cerebrospinal fluid pressure through a ventricular reservoir when present. Failure of ETV was defined as the subsequent need for shunt implantation. The overall success rate of the ETV was 70% and varied from 86.9% in patients with a mobile stoma and a positive hydrostatic test to only 14.2% in patients with a poorly mobile floor and a negative test (p < 0.05). The positive predictive value of the hydrostatic test was 86.9%, negative predictive value 85.7%, sensitivity 95.2%, and specificity 66.6%. Conclusions. The authors concluded that the hydrostatic test is an easy, brief test. A positive test result confirms a mobile ventricular floor and adequate flow through the created ventriculostomy. Mobility of the stoma is an important predictor of ETV success provided that there is no obstruction at the level of the arachnoid granulations or venous outflow. A thin, redundant, mobile third ventricle floor indicates a longstanding pressure differential between the third ventricle and the basal cisterns, which is a crucial factor for ETV success. A positive hydrostatic test may avert the need to insert a ventricular reservoir, thus avoiding associated risks of infection.


2000 ◽  
Vol 93 (2) ◽  
pp. 326-329 ◽  
Author(s):  
Takaichi Suehiro ◽  
Takanori Inamura ◽  
Yoshihiro Natori ◽  
Masayuki Sasaki ◽  
Masashi Fukui

✓ The authors report the use of neuroendoscopic third ventriculostomy to treat successfully both hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. A 27-year-old woman presented with dizziness, headache, and nausea. Magnetic resonance (MR) imaging demonstrated dilation of all ventricles, downward displacement of the third ventricular floor, obliteration of the retrocerebellar cerebrospinal fluid (CSF) space, funnellike enlargement of the entrance of the central canal in the fourth ventricle, and syringomyelia involving mainly the cervical spinal cord. Cine-MR imaging indicated patency of the aqueduct and an absent CSF flow signal in the area of the cisterna magna, which indicated obstruction of the outlets of the fourth ventricle. Although results of radioisotope cisternography indicated failure of CSF absorption, neuroendoscopic third ventriculostomy completely resolved all symptoms as well as the ventricular and spinal cord abnormalities evident on MR images. Neuroendoscopic third ventriculostomy is an important option for treating hydrocephalus in patients with fourth ventricle outlet obstruction.


2001 ◽  
Vol 95 (5) ◽  
pp. 783-790 ◽  
Author(s):  
Philippe Decq ◽  
Caroline Le Guérinel ◽  
Jean-Christophe Sol ◽  
Pierre Brugières ◽  
Michel Djindjian ◽  
...  

Object. Hydrocephalus associated with Chiari I malformation is a rare entity related to an obstruction in the flow of cerebrospinal fluid (CSF) in the foramen of Magendie. Like all forms of noncommunicating hydrocephalus, it can be treated by endoscopic third ventriculostomy (ETV). The object of this study is to report a series of five cases of hydrocephalus associated with Chiari I malformation and to evaluate the use of ETV in the treatment of this anomaly. Methods. Five patients (four women and one man with a mean age of 29.6 years) underwent ETV for hydrocephalus associated with Chiari I malformation between April 1991 and February 1997. All patients had presented with paroxysmal headaches, which in two cases were associated with visual disorders. All patients had also presented with hydrocephalus (mean transverse diameter of the third ventricle 12.79 mm; mean sagittal diameter of the fourth ventricle 18.27 mm) with a mean herniation of the cerebellar tonsils at 13.75 mm below the basion—opisthion line. Surgery was performed in all patients by using a rigid endoscope. No complications occurred either during or after the procedure, except in one patient who experienced a wound infection that was treated by antibiotic medications. The mean duration of follow up in this study was 50.39 months. Four patients became completely asymptomatic and remained stable throughout the follow-up period. One patient required an additional third ventriculostomy after 1 year, due to secondary closure, and has remained stable since that time. Postoperative magnetic resonance images demonstrated a significant reduction in the extent of hydrocephalus in all patients (mean transverse diameter of the third ventricle 6.9 mm [p = 0.0035]; mean sagittal diameter of the fourth ventricle 10.32 mm [p = 0.007]), with a mean ascent of the cerebellar tonsils from 13.75 mm below the basion—opisthion line to 7.76 mm below it (p = 0.01). In addition, CSF flow was identified on either side of the orifice of the third ventriculostomy in all patients postoperatively. Conclusions. Results in this series confirm the efficacy of ETV in the treatment of hydrocephalus associated with Chiari I malformation. It is a reliable, minimally invasive technique that also provides a better understanding of the pathophysiology of this malformation.


2000 ◽  
Vol 92 (6) ◽  
pp. 1028-1035 ◽  
Author(s):  
Joshua G. Kouri ◽  
Michael Y. Chen ◽  
Joseph C. Watson ◽  
Edward H. Oldfield

✓ Generally accepted contraindications to using a transsphenoidal approach for resection of tumors that arise in or extend into the suprasellar region include a normal-sized sella turcica, normal pituitary function, and adherence of tumor to vital intracranial structures. Thus, the transsphenoidal approach has traditionally been restricted to the removal of tumors involving the pituitary fossa and, occasionally, to suprasellar extensions of such tumors if the sella is enlarged. However, conventional transcranial approaches to the suprasellar region require significant brain retraction and offer limited visualization of contralateral tumor extension and the interface between the tumor and adjacent structures, such as the hypothalamus, third ventricle, optic apparatus, and major arteries. In this paper the authors describe successful removal of suprasellar tumors by using a modified transsphenoidal approach that circumvents some of the traditional contraindications to transsphenoidal surgery, while avoiding some of the disadvantages of transcranial surgery.Four patients harbored tumors (two craniopharyngiomas and two hemangioblastomas) that arose in the suprasellar region and were located either entirely (three patients) or primarily (one patient) within the suprasellar space. All patients had a normal-sized sella turcica. Preoperatively, three of the four patients had significant endocrinological deficits signifying involvement of the hypothalamus, pituitary stalk, or pituitary gland. Two patients exhibited preoperative visual field defects. For tumor excision, a recently described modification of the traditional transsphenoidal approach was used. Using this modification, one removes the posterior portion of the planum sphenoidale, allowing access to the suprasellar region. Total resection of tumor was achieved (including absence of residual tumor on follow-up imaging) in three of the four patients. In the remaining patient, total removal was not possible because of adherence of tumor to the hypothalamus and midbrain. One postoperative cerebrospinal fluid leak occurred. Postoperative endocrinological function was worse than preoperative function in one patient. No other new postoperative endocrinological or neurological deficits were encountered.This study demonstrates the feasibility of using a modified transsphenoidal approach for resection of certain suprasellar, nonpituitary tumors.


2008 ◽  
Vol 109 (5) ◽  
pp. 931-938 ◽  
Author(s):  
Mathias Kunz ◽  
Gernot Schulte-Altedorneburg ◽  
Eberhard Uhl ◽  
Robert Schmid-Elsaesser ◽  
Karsten Schöller ◽  
...  

Object Endoscopic third ventriculostomy is the treatment of choice in patients with obstructive hydrocephalus caused by aqueductal stenosis. The authors examined the clinical course and results of surgical treatment for obstructive hydrocephalus with pre- and postoperative refined constructive interference in steady-state (CISS) MR imaging. Methods Forty patients with obstructive hydrocephalus underwent pre- and postoperative 3D-CISS imaging and clinical evaluation. Radiological findings were correlated with intraoperative observations of the thickness and transparency of the floor of the third ventricle and the patient's postoperative clinical course. Results Three-dimensional CISS MR imaging provides precise visualization of the basilar/posterior cerebral artery, its distance to the clivus, the diameter of the foramen of Monro, and the extension of and thickness of the floor of the third ventricle. In 71% of patients a flow void was detectable postoperatively on the ventriculostomy. In this group 81.5% had strong and 14.8% moderate clinical benefit, and 3.7% required secondary shunt placement. In the remaining 29% of the patients without a visible flow void, strong improvement was seen in 54.5%, moderate improvement in 18.2%, and stoma failure occurred in 27.3% (p = 0.094). Radiological measurements of the thickness of the third ventricle floor correlated with intraoperative findings (r = 0.35, p = 0.029). Comparison of outcomes showed a statistically significant tendency for a better outcome in patients with thin and easily perforated third ventricle floors (p = 0.04). Conclusions Endoscopic ventriculostomy in patients with obstructive hydrocephalus is safe and mostly successful, and 3D-CISS MR imaging seems to be a valuable diagnostic method for precisely identifying the anatomy of relevant structures. Furthermore, 3D-CISS MR imaging allows judgment of the thickness of the third ventricle floor and display of the ventriculostomy/flow void, which are predictive for intraoperative course and clinical outcome.


2003 ◽  
Vol 99 (1) ◽  
pp. 89-99 ◽  
Author(s):  
Jérôme Yelnik ◽  
Philippe Damier ◽  
Sophie Demeret ◽  
David Gervais ◽  
Eric Bardinet ◽  
...  

Object. The aim of this study was to correlate the clinical improvement in patients with Parkinson disease (PD) treated using deep brain stimulation (DBS) of the subthalamic nucleus (STN) with the precise anatomical localization of stimulating electrodes. Methods. Localization was determined by superimposing figures from an anatomical atlas with postoperative magnetic resonance (MR) images obtained in each patient. This approach was validated by an analysis of experimental and clinical MR images of the electrode, and the development of a three-dimensional (3D) atlas—MR imaging coregistration method. The PD motor score was assessed through two contacts for each of two electrodes implanted in 10 patients: the “therapeutic contact” and the “distant contact” (that is, the next but one to the therapeutic contact). Seventeen therapeutic contacts were located within or on the border of the STN, most of which were associated with significant improvement of the four PD symptoms tested. Therapeutic contacts located in other structures (zona incerta, lenticular fasciculus, or midbrain reticular formation) were also linked to a significant positive effect. Stimulation applied through distant contacts located in the STN improved symptoms of PD, whereas that delivered through distant contacts in the remaining structures had variable effects ranging from worsening of symptoms to their improvement. Conclusions. The authors have demonstrated that 3D atlas—MR imaging coregistration is a reliable method for the precise localization of DBS electrodes on postoperative MR images. In addition, they have confirmed that although the STN is the main target during DBS treatment for PD, stimulation of surrounding regions, particularly the zona incerta or the lenticular fasciculus, can also improve symptoms of PD.


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