Chiari I malformation: a rare cause of noncommunicating hydrocephalus treated by third ventriculostomy.

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 I 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.     

Syringomyelia with obstructive hydrocephalus at the foramens of Luschka and Magendie successfully treated by endoscopic third ventriculostomy

BACKGROUND: Syringomyelia rarely accompanies hydrocephalus except for the cases with Chiari malformation, and the efficacy of endoscopic third ventriculostomy for this entity is undetermined. CASE REPORT: A 55-year-old man developed sensory disturbance and discrete movement disturbance on the bilateral upper limb during the past 3 years. On admission, he presented with slight tetraparesis and hyperreflection on his lower limbs with rectovesical dysfunction. Preoperative MR imaging demonstrated marked enlargement of the lateral ventricles as well as the third and fourth ventricle due to obstruction of the foramens of Luschka and Magendie. Spine MR imaging showed longitudinal dilatation of the central canal from the cerebrospinal junction toward the thoracic level with communication to the fourth ventricle, whereas Chiari malformation and tight cisterna magna were absent. He was successfully treated by ETV. The patient significantly recovered from his symptoms postoperatively, and MR imaging after surgery showed apparent shrinkage of the syrinx as well as the ventricles. CONCLUSION: The efficacy of the normalization of cerebrospinal fluid circulation and subsequent improvement of the syringomyelia in the present case may suggest that ETV could be a therapeutic choice for syringomyelia associated with hydrocephalus due to the obstruction of the foramens of Luschka and Magendie, especially when the connection between the ventricular system and the dilated central canal is evident, and may give a clue to the explanation for Gardner's hydrodynamic theory.     

Initial management of hydrocephalus associated with Chiari malformation Type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis

OBJECT: The aim of this study was to evaluate the efficacy of endoscopic third ventriculostomy (ETV) in patients with Chiari malformation Type I (CM-I) and hydrocephalus with or without syringomyelia. METHODS: The authors identified, in a prospective endoscopy database, 16 adults and children (age range 2-68 years) with CM-I and hydrocephalus that had been managed with ETV. They reviewed the clinical features and radiographic findings for all patients. Fifteen patients underwent ETV as a primary treatment, whereas 1 patient underwent the procedure at the time of shunt failure. All patients had symptomatic hydrocephalus with either aqueductal or fourth ventricular outflow obstruction. The mean duration of follow-up was 42 months. RESULTS: Fifteen patients (94%) remain shunt free following ETV for CM-I. Five (83%) of the 6 patients with a syrinx had improvement or resolution of the syrinx following ETV. Six patients (37.5%) underwent foramen magnum decompression for persistent CM-I -- or syrinx-related symptoms. There was no cerebrospinal fluid leakage or intracranial pressure-related problem following foramen magnum decompression. CONCLUSIONS: Endoscopic third ventriculostomy provides a durable method of treatment for hydrocephalus associated with CM-I. It is effective as a primary treatment, and the authors advocate its use as a replacement for routine ventriculoperitoneal shunt insertion in these patients. Management of the hydrocephalus alone is often sufficient and may obviate decompression, although a significant proportion of patients will still need both procedures.     

Endoscopic opening of the foramen of magendie using transaqueductal navigation for membrane obstruction of the fourth ventricle outlets. Technical note

A membrane obstruction of the foramina of Magendie and Luschka is an uncommon origin of hydrocephalus characterized by unusual clinical symptoms of rhomboid fossa hypertension. Various surgical approaches have been proposed to alleviate this obstruction, including opening the obstructed foramen of Magendie using suboccipital craniectomy, shunting procedures, and more recently, endoscopic third ventriculostomy (ETV). In some cases, however, reshaping of the posterior fossa due to the collapse of the prepontine cistern could make ETV difficult for the surgeon and dangerous to the patient. In these cases, endoscopic opening of the foramen of Magendie by transaqueductal navigation of the fourth ventricle is a suitable and feasible therapeutic option.     

Successful neuroendoscopic third ventriculostomy for hydrocephalus and syringomyelia associated with fourth ventricle outlet obstruction. Case report

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 cistema 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.     

Visual field deficit due to downward displacement of optic chiasm

Visual disturbance in hydrocephalus is typically due to raised intracranial pressure. We describe a patient who presented with marked loss of peripheral visual fields, but without features suggestive of raised intracranial pressure. MR scan showed an enlarged third ventricle and a downward displacement of the optic chiasm, Chiari II malformation. These radiological changes and the visual field deficits reversed after endoscopic third ventriculostomy and foramen magnum decompression. These observations support the view that the treatment of the hydrocephalus in such patients can help to reverse the change in the position of the optic chiasm and the visual field deficits.     

Idiopathic acute obstructive hydrocephalus. Case report

Acute hydrocephalus is rarely encountered in adults without venticuloperitoneal or venticuloatrial shunts. The purpose of this report is to describe a case of acute hydrocephalus due to no identifiable cause. A 40-year-old man developed a typical clinical picture of intracranial hypertension within a 24-hours period. On the morning of hospitalization, the patient consulted his physician for severe headache followed quickly by nausea and vomiting. After cerebral CT-scan, the patient was referred to our department. His condition rapidly worsened, with confusion, then drowsiness. Magnetic resonance imaging (MRI) demonstrated quadri-ventricular hydrocephalus with a Chiari I malformation. In view of his rapidly deteriorating clinical condition, emergency endoscopic third ventriculostormy was performed. The patient recovered rapidly and was discharged 8 days after the procedure. Diagnostic work-up included lumbar puncture showing normal cerebrospinal fluid (thereby eliminating multiple sclerosis, low-grade intracranial hemorrhage, and meningitis (bacterial, viral, fungal)), spinal MRI depicting no medullary lesions, and brain angiography revealing no vascular abnormalities. Follow-up brain MRI carried out at six months after hospitalization demonstrated normal ventricles and complete disappearance of the Chiari I malformation. No conclusion can be drawn as to whether the Chiari I malformation was the consequence or cause of hydrocephalus. The explanation of this acute hydrocephalus is the acute decompensation without identifiable cause of idiopathic stenosis of the foramen of Magendie and Luschka. Treatment with endoscopic third ventriculostomy is effective.     

Bilateral occlusion of the foramina of Monro after third ventriculostomy

Occlusion of both foramina of Monro following third ventriculostomy is a very rare complication. The authors present the case of a 30-year-old female who underwent endoscopic third ventriculostomy (ETV) for occlusive hydrocephalus due to aqueductal stenosis. Thirty months after the ETV, she reported recurrent headaches. Magnetic resonance imaging demonstrated bilateral enlargement of the lateral ventricles with a collapsed third ventricle caused by bilateral stenosis of the foramina of Monro. Left-sided endoscopic foraminoplasty and stenting of the left foramen of Monro were performed with immediate neurological improvement.     

Severe parkinsonism following endoscopic third ventriculostomy for non-communicating hydrocephalus--case report

A 23-year-old male with non-communicating hydrocephalus caused by intraventricular hemorrhage from an arteriovenous malformation was treated by endoscopic third ventriculostomy (ETV) after repeated ventricular cerebrospinal fluid drainage and removal of ventriculoperitoneal shunt due to infection. Postoperatively, the patient developed robust rigidity and akinesia with mutism, suggesting secondary parkinsonism. These symptoms showed marked improvement after administration of levodopa, and were not aggravated after discontinuation. The pathophysiology of this case might involve dysfunction of the dopaminergic system caused by repeated compression and release of the paraventricular white matter. Careful perioperative management not leading to excessive dilation of the the ventricles should be considered for ETV for non-communicating hydrocephalus to avoid this unexpected complication.     

Endoscopic third ventriculostomy for patients with hydrocephalus and fourth ventricular cysticercosis: a review of five cases.

The authors evaluate the feasibility of performing endoscopic third ventriculostomy (ETV) in a series of five patients with ventricular outlet obstruction and a prior history of fourth ventricular cysticercosis. All patients underwent fourth ventricular cyst excision and presented at various times afterwards with hydrocephalus. They were all successfully managed with ETV, and no patient has required subsequent surgery during a mean follow-up period of 21 months (range 12-36 months).     

Role of preoperative midsagittal magnetic resonance imaging in endoscopic third ventriculostomy.

We measured the distance between the infundibular recess and the tip of the basilar artery (BA) on midsagittal magnetic resonance (MR) images in 217 subjects (mean age, 54.7 years) without intracranial disorders and 8 patients with hydrocephalus treated neuroendoscopically. The mean distance measured in the 217 subjects without intracranial disorders was 10.5 +/- 2.3 mm. There were no significant differences in this distance among age groups. In 28 of these 217 subjects (12.9%), the tip of the BA had shifted anterior to the mamillary bodies on MR images. Twenty-six of these 28 subjects (93%) were 60 years of age or older. The mean distance between the infundibular recess and the tip of the BA was 12 +/- 3.7 mm in 8 patients with hydrocephalus. There was no significant difference between the subjects without intracranial disorders and the patients with hydrocephalus in this distance. In an elderly patient with aqueductal stenosis for whom the distance between the infundibular recess and the tip of the BA was 6 mm, the tip of the BA had shifted anterior to the mamillary bodies and indented the floor of the third ventricle on preoperative midsagittal MR images, and perforation of the floor of the third ventricle therefore had to be carefully performed in order to avoid injury of the BA. Preoperative midsagittal MR images can clearly reveal neurovascular structures beneath the floor of the third ventricle and increase the safety of endoscopic third ventriculostomy.     

Endoscopic third ventriculostomy for treatment of noncommunicating syringomyelia associated with a Chiari I malformation and hydrocephalus: case report and pathophysiological considerations

OBJECTIVE AND IMPORTANCE: A Chiari I malformation associated with syringomyelia and hydrocephalus is a rare condition. We report the successful use of endoscopic third ventriculostomy for the treatment of this pathological entity. The successful use of this technique in such a case has not been previously described, and the results allow us to speculate on the pathophysiological mechanism involved. CLINICAL PRESENTATION: A 34-year-old woman presented with headaches, a motor deficit of the right upper limb, and gait dyspraxia. Magnetic resonance imaging scans demonstrated dilation of all ventricles, compression of the retrocerebellar cerebrospinal fluid space, downward displacement of the tonsils, and syringomyelia. Syringomyelia involved the cervicodorsal cord below C3, with a syrinx-free segment between C1 and C3 and no enlargement of the rostral part of the central canal. INTERVENTION: Endoscopic third ventriculostomy resulted in prompt improvement of the clinical symptoms. Postoperative magnetic resonance imaging scans demonstrated shrinkage of the syrinx and return of the cerebellar tonsils to their physiological positions. CONCLUSION: This experience demonstrates that endoscopic third ventriculostomy, which is a simple, safe technique, may be the treatment of choice for associated Chiari I malformations, hydrocephalus, and syringomyelia (even the noncommunicating type).     

Endoscopic third ventriculostomy for shunt dysfunction in occlusive hydrocephalus: long-term follow up and review

OBJECT: Endoscopic third ventriculostomy (ETV) is the treatment of choice for occlusive (noncommunicating) hydrocephalus. Nevertheless, its routine use in patients who have previously undergone shunt placement is still not generally accepted. The authors' aim was to investigate the long-term effects of ETV in a group of prospectively chosen patients. METHODS: Patients who underwent ETV and had previously undergone shunt placement for occlusive hydrocephalus were followed prospectively for at least 3 years (range 36-103 months, mean 63.6 months). Nine female and eight male patients ranging from 8 to 54 years of age (mean 32 years) had undergone shunt placement 0.7 to 23.5 years (mean 8.1 years) before ETV. Fifteen patients were admitted with underdrainage and two with overdrainage. In six cases, ETV was performed as an emergency operation. The origin of hydrocephalus was aqueductal stenosis in 12 cases and aqueductal compression by a tumor in two cases. Three patients suffered from a fourth ventricle outlet syndrome, and in two patients an additional malresorptive component was suspected. Thirteen patients underwent ETV with shunt removal and insertion of an external drain in one session. The drain served as a safety measure; it could be opened if raised intracranial pressure or ventricular dilation was observed on postoperative imaging studies. In the other four patients the shunt was initially ligated and then removed during a second operation. Fourteen patients (82%) have remained shunt free. The other three patients, including the two with an additional malresorptive component, needed shunt reimplantation 3 days, 2 weeks, or 7 months after ETV. CONCLUSIONS: Use of ETV is safe and effective for the treatment for shunt dysfunction in patients with obstructive hydrocephalus.     

Traumatic subependymal hematoma during endoscopic third ventriculostomy in a patient with a third ventricle tumor: case report.

Endoscopic third ventriculostomy has become a routine intervention for the treatment of non-communicating hydrocephalus. This technique is largely considered safe and a very low incidence of complications is reported. However, hemorrhage in the course of neuroendoscopy is still a problem difficult to manage. The authors present a case in which endoscopic third ventriculostomy and tumor biopsy were performed in a young patient with a huge tumor growing in the posterior part of the third ventricle. The surgical approach to realize the stoma was difficult because the tumor size reduced the third ventricle diameter. Surgical manipulation produced a traumatic subependymal hematoma. This hematoma drained spontaneously after few minutes into the ventricle and the blood was washed away. The postoperative neurological course was uneventful and the ventriculostomy showed to work well by reducing the size of the lateral ventricles and the intracranial pressure in three days. This complication during endoscopic third ventriculostomy has never been reported before. We emphasize the difficulty of endoscopic procedures in patients with huge tumors in the third ventricle. Where reduction in size of the third ventricle and of the foramen of Monro ist present we suggest a careful approach to the third ventricle.     

Clinical features in patients requiring reoperation after failed endoscopic procedures for hydrocephalus.

The aim of this study was to clarify the clinical features of patients at risk of secondary obstruction following endoscopic fenestration. Clinical notes and endoscopic findings for 15 patients treated with endoscopic procedures were retrospectively reviewed. Endoscopic third ventriculostomy (ETV) was performed as initial treatment in 4 patients with non-communicating hydrocephalus, including a neonate with myelomeningocele, and as an alternative to shunt revision in 4 patients. Two patients with non-communicating hydrocephalus caused by tumor or arachnoid cyst were also managed with third ventriculostomy. Four patients with loculated hydrocephalus underwent endoscopic septostomy. A child with an isolated fourth ventricle was treated with endoscopic aqueductoplasty. Of the 15 patients undergoing endoscopic procedure, 4 required reoperation. Of the 10 patients treated with ETV, only the neonate with myelomeningocele required a ventriculoperitoneal shunt because of failure of the initial procedure. Of the 4 patients treated with endoscopic septostomy, 2 children with loculated hydrocephalus following intraventricular hemorrhage (IVH) underwent a second septostomy. In a patient with an isolated fourth ventricle following posthemorrhagic hydrocephalus, recurrence was noted 8 months after the initial procedure. He underwent a second procedure using a stent implanted into the aqueduct to maintain CSF circulation. Sufficient stomal size or implantation of a stent may be required in the under-2-year age group with hydrocephalus accompanied by IVH and associated with myelomeningocele, in whom the risk of secondary obstruction may be high.     

Hidden aqueductal stenosis associated to bilateral idiopathic foramina of Monro stenosis mimicking a Chiari I malformation? Case report

A 39-year old man came to our outpatient clinic with long history of unspecific symptoms and signs. Cerebral MRI showed herniation of the cerebellar tonsils of more than 1?cm below the foramen magnum and a triventricular hydrocephalus. A diagnosis of Chiari I malformation was retained. After an osteo-dural decompression of the posterior fossa, post-operative MRI revealed an aqueductal stenosis with triventricular hydrocephalus. An endoscopic-third- ventriculostomy showed an idiopathic stenosis of the right foramen of Monro. Residual symptoms and persistence of biventricular hydrocephalus justified a ventriculo-peritoneal shunt. Aqueductal and foramina of Monro stenosis can mimick a Chiari I malformation.     

Anomalies and variants of the endoscopic anatomy for third ventriculostomy.

OBJECTIVE: Endoscopic third ventriculostomy (ETV) is an alternative to shunt placement in occlusive hydrocephalus. The negative impact of anatomic anomalies and variants on ETV have been sporadically reported but not yet investigated systematically. Therefore, the objectives of the present study are 1) to evaluate the frequency of endoscopic anatomic anomalies of the ventricular system, 2) to define their potential to complicate the procedure and to compromise the surgical results, and 3) to investigate the value of preoperative magnetic resonance (MR) imaging for their detection. METHOD: The video recordings, the operative reports, and the preoperative MR images of 25 hydrocephalic patients who underwent ETV were reviewed. The surgical results were classified into completed and successful, completed, but failed, and unsuccessfully attempted ETV and were correlated with the absence or presence of anatomic variants. RESULTS: In 9 of the 25 patients, 10 anatomic anomalies or variants, respectively, were identified, accounting for an incidence rate of 36%. The single most common anatomic anomaly was a thickened third ventricular floor in 4 patients. Anatomic variants extended the operation time (n = 6), increased the stretching of floor and walls of the third ventricle during perforation (n = 4), were related to minor arterial bleeding (n = 3), and obscured the visual control of the basilar artery (n = 2). In 5 of the 9 patients, ETV was completed and successful, but in 2 patients, ETV was finally abandoned, and in an additional 2 patients, ETV was completed, but failed to cure the symptoms of hydrocephalus. In contrast, ETV was completed and successful in all 16 patients with normal anatomy. All anatomic anomalies had been detectable on preoperative MR imaging, with the exception of the thickened floor of the third ventricle. CONCLUSION: Anatomic anomalies are a frequent finding during ETV. Successful perforation and control of the hydrocephalus correlates with the absence of anatomic anomalies. Most anatomic variants have the potential to increase the operative risk. With the exception of the thickened third ventricular floor, MR imaging allows us to identify all anatomic anomalies preoperatively, and enables the neurosurgeon to weigh the operative risk in a patient with an anatomic anomaly against the chance to perform ETV successfully.     

Laser-assisted endoscopic third ventriculostomy for obstructive hydrocephalus: technique and results in a series of 40 consecutive cases

BACKGROUND AND OBJECTIVES: To report a case series of endoscopic third ventriculostomy (ETV) using laser in 40 consecutive patients with obstructive hydrocephalus. STUDY DESIGN/MATERIALS AND METHODS: Under stereotactic and endoscopic guidance, multiple perforations in the ventricular floor using a 1.32 microm neodymium-yttrium/aluminum/garnet (Nd-YAG) or a 0.805 microm diode laser unit and removal of intervening coagulated tissue ensued with a 4-6 mm opening between third ventricle and basilar cisterns. RESULTS: The procedure could be completed in all cases. A transient complication occurred in five cases. In 39 patients (mean follow-up 28 months), 31 (79%) had a favorable outcome. Failure occurred in six patients, requiring permanent shunting leading to complete recovery, and two patients remained in a poor clinical status despite ETV. CONCLUSIONS: Laser-assisted ETV is a safe and efficient procedure for the treatment of obstructive hydrocephalus. Laser is advantageous in cases of distorted anatomy and may reduce technical failures.     

Endoscopic diagnosis and treatment of far distal obstructive hydrocephalus

Purpose  

Obstruction of the CSF circulation distal to the fourth ventricle is a rare cause of noncommunicating hydrocephalus. Endoscopic third ventriculostomy (ETV) represents one of the treatment options, but reports of results are rare.     

Endoscopic third ventriculostomy in obstructive hydrocephalus due to giant basilar artery aneurysm

OBSTRUCTIVE: hydrocephalus due to giant basilar artery (BA) aneurysm is a rare finding, and endoscopic treatment has not been reported. Here the authors present their experience with endoscopic third ventriculostomy (ETV) in obstructive hydrocephalus due to giant BA aneurysm. Between December 2000 and March 2007, 3 patients (2 men and 1 woman; age range 32-80 years) underwent an ETV for the treatment of obstructive hydrocephalus caused by a giant BA aneurysm. All 3 patients presented with cephalgia, nausea, vomiting, and a variable decrease in consciousness. An obstructive hydrocephalus caused by a giant BA aneurysm was found in each case as the underlying pathological entity. Intraoperatively, a narrowing of the third ventricle by upward displacement of the tegmentum was found in all 3 patients. A standard ETV was performed and included an inspection of the prepontine cisterns. The endoscopic treatment was successful in all patients with respect to clinical signs and radiological ventricular enlargement. No complications were observed. In all, the endoscopic ventriculostomy was proven to be a successful treatment option in obstructive hydrocephalus even if it is caused by untreated giant BA aneurysm.