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.     

Role of endoscopic third ventriculostomy in patients with communicating hydrocephalus: an evaluation by MR ventriculography

Post-tubercular meningitic hydrocephalus (TBMH) and post-traumatic hydrocephalus (PTH) is often considered a contraindication for endoscopic third ventriculostomy (ETV), as it is mostly of communicating type in these cases. The aim of the present study was to define the role of ETV in patients with communicating hydrocephalus. Ten consecutive patients of TBMH, PTH and postneurocysticercus (NCC) hydrocephalus were formed the study group. Diagnosis of communicating hydrocephalus was made using magnetic resonance ventriculography (MRV). If contrast was seen coming out from the ventricular system into the basal cisterns, it was considered as communicating hydrocephalus. Patients with clinical and imaging evidence of raised intracranial pressure and failed medical treatment were taken up for ETV. All patients were studied by preoperative and postoperative MRV. Success of the procedure was assessed by the improvement in clinical and imaging parameters on postprocedure follow-up in all these cases. Technically successful ETV was performed in all 10 patients. Overall success rate of ETV in communicating hydrocephalus was 70% (n = 7). The shunt surgery was performed in the remaining three patients with ETV failure. One patient developed complication following postoperative MRV and was managed conservatively. We conclude that ETV is effective in post-TBM, post-traumatic communicating and post-NCC communicating hydrocephalus and should be considered as initial surgical option for cerebrospinal fluid diversion in these patients. MRV is a relatively safe technique to ascertain the patency of subarachnoid space as well as ETV stoma.     

Death after late failure of third ventriculostomy in children. Report of three cases

Late failure following successful third ventriculostomy for obstructive hydrocephalus is rare, and death caused by failure of a previously successful third ventriculostomy has been reported only once. The authors present three patients who died as a result of increased intracranial pressure (ICP) after late failure of a third ventriculostomy. Through a collaborative effort, three patients were identified who had died following third ventriculostomy at one of the authors' institutions. A 13-year-old girl with neurofibromatosis Type 1 underwent third ventriculostomy for obstructive hydrocephalus caused by a tectal lesion. Three years later her condition deteriorated rapidly over the course of 6 hours and she was found dead at home. A 4-year-old boy treated with third ventriculostomy for aqueductal stenosis presented 2 years postoperatively with symptoms of increased ICP. This patient suffered a cardiorespiratory arrest while under observation and died despite external ventricular drainage. A 10-year-old boy with previous ventriculoperitoneal (VP) shunt placement underwent conversion to a third ventriculostomy and shunt removal. Eight months after the procedure his condition deteriorated. with evidence of raised ICP, and he underwent emergency insertion of another VP shunt, but remained in a vegetative state and died of complications. Neuropathological examinations in two cases demonstrated that the third ventriculostomy was not patent, and there was also evidence of increased ICP. Late failure of third ventriculostomy resulting in death is a rare complication. Delay in recognition of recurrent ICP symptoms and a false feeling of security on the part of family and caregivers because of the absence of a shunt and the belief that the hydrocephalus has been cured may contribute to fatal complications after third ventriculostomy. Patients with third ventriculostomies should be followed in a manner similar to patients with cerebrospinal fluid shunts.     

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.     

Endoscopic ventriculostomy versus shunt operation in normal pressure hydrocephalus: diagnostics and indication.

In contrast to the shunt operation the indication for an endoscopic ventriculostomy in patients diagnosed for normal pressure hydrocephalus is not scientifically established. Between September 1997 and December 1999 we operated on 48 patients diagnosed for normal pressure hydrocephalus. The diagnosis was established by means of the intrathecal lumbar or ventricular infusion test, the cerebrospinal fluid tap test and MRI-CSF flow studies pre- and postoperatively. In 37 patients (77%) we have implanted a ventriculo-peritoneal shunt, and in 11 patients (23%) we performed the endoscopic assisted third ventriculostomy. With our created NPH recovery rate and use of the clinical grading for normal pressure hydrocephalus created by Kiefer and Steudel we compared the operative results of both groups of patients. In patients with a pathologically increased resistance to CSF outflow in the lumbar infusion test a shunt implantation is indicated. Patients whose outflow resistance is increased in the ventricular infusion test but with a physiological lumbar infusion test are suspected for a functional aqueduct stenosis and should be treated by means of endoscopic assisted ventriculostomy.     

Third Ventriculostomy in Late-onset Idiopathic Aqueductal Stenosis Treatment: A Focus on Clinical Presentation and Radiological Diagnosis

Endoscopic third ventriculostomy (ETV) is considered the gold standard treatment for obstructive hydrocephalus due to partial or complete obstruction of cerebrospinal fluid (CSF) ventricular pathways caused by mass lesions. However long-term efficacy of this procedure remains controversial as treatment of chronic adult hydrocephalus due to stenosis of Sylvian acqueduct [late-onset idiopathic aqueductal stenosis (LIAS)]. The authors describe clinical presentation, diagnostic investigations in patients affected by LIAS, and define their clinical and radiological outcome after ETV. From January 2003 to December 2008, 13 consecutive LIAS patients treated by ETV were retrospectively reviewed. Pre- and post-operative clinical and radiological findings, including conventional and phase-contrast (PC) cine magnetic resonance imaging (MRI) were investigated. ETV was successfully performed in all patients. Patient''s neurological condition improved. No one required a second ETV procedure or shunt implantation. Clinical and radiological results reveal a satisfactory outcome of LIAS patients treated by ETV. At follow-up a clinical improvement could be demonstrated in all cases. Selection criteria of LIAS patients seem to be crucial to obtain satisfactory and long-lasting results. Even in elderly patients with chronic hydrocephalus, ETV can be considered the treatment of choice.     

Ventricular failure as a cause of unsuccessful endoscopic third ventriculostomy.

INTRODUCTION: In spite of extensive studies on cerebrospinal fluid (CSF) dynamics, the mechanism of its circulation is still obscure. The aim of this study is to evaluate the effect of an insult to the brain tissue on the success or failure of an endoscopic procedure. METHODS: During the period from May 1995 to December 1998 we studied 21 children, ages from 2 to 48 months (mean 15 months) with non-communicating hydrocephalus. The intracranial pressure was measured at the time of endoscopic surgery. The follow-up period was from 2 - 5 years. They were divided into 2 groups. Group I were 8 patients with no history of brain insult. Group II (13 cases) had a previous brain insult (infective and/or vascular). Endoscopic third ventriculostomy (ETV) was done in 17 cases; 5 in Group I and 12 in Group II. Fenestration of cyst/s was done in all of Group I and in 9 cases of Group II. Failure was considered whenever shunt implantation was required. RESULTS: The mean intracranial pressure in Group I was 13.0 mmHg as compared to 9.7 mmHg in Group II (p = 0.015). The failure rate of the endoscopic procedure/s was 25 % and 54 % in Groups I and II, respectively. DISCUSSION: The success of ETV and/or fenestration of cyst/s depends on a sufficient pressure gradient. The pressure gradient is generated by a normal or compensated ventricular function. CONCLUSION: Brain tissue damage can result in ventricular failure. The quality of ventricular wall function has a significant role on the success of ETV.     

Endoscopic third ventriculostomy in the treatment of idiopathic normal pressure hydrocephalus: a review study

Idiopathic normal pressure hydrocephalus is a hydrodynamic disorder whose etiology remains unclear. The diagnosis is mainly clinical and the traditional treatment is cerebrospinal fluid shunt diversion. With the introduction of modern management strategies, endoscopic third ventriculostomy has become a viable alternative to shunting and constitutes a well-established method of treatment for obstructive hydrocephalus. The new hydrodynamic concept of hydrocephalus suggests that endoscopic third ventriculostomy (ETV) may be an effective treatment for communicative hydrocephalus. In our current review, the authors focus on the up-to-date knowledge regarding the consideration of endoscopic third ventriculostomy as a safe surgical option in the management of idiopathic normal pressure hydrocephalus.     

Management of hydrocephalus associated with occipital encephalocoele using endoscopic third ventriculostomy: report of two cases

BACKGROUND: Occipital encephalocoele is the most common cranial dysraphism in the western hemisphere and is often complicated by hydrocephalus. Management of hydrocephalus and reducing the CSF pressure is crucial in preventing dehiscence at the site of the encephalocoele repair. METHODS: Two female patients had presented with occipital encephalocoeles. The first patient (aged 42 days) had undergone repair of the occipital encephalocoele and then developed hydrocephalus with recurrence of the encephalocoele. The second patient (aged 12 months) had hydrocephalus associated with an occipital encephalocoele at initial presentation.Both the patients underwent endoscopic third ventriculostomy (ETV) through a right frontal burr hole. In the first patient, ETV was performed after shunt dysfunction at the age of 9 months. Because she presented with recurrence of the encephalocoele 15 months later, a repeat endoscopic third ventriculostomy was performed. She required a ventriculoperitoneal shunt during the same admission because of the early failure of the ventriculostomy. In the second patient, it was performed before the encephalocoele repair, both ETV and the repair being conducted under the same anesthesia. ETV was performed using a rigid scope and the perforation in the third ventricular floor was enlarged using a No. 4 Fogarty catheter. RESULTS: The first patient had no recurrence of encephalocoele at follow-up of 10 months but she presented with recurrence of the occipital encephalocoele after 15 months. The second patient had no evidence of recurrence at follow-up after 16 months. The lateral and third ventricular volumes had decreased in both the patients at initial follow-up. CONCLUSION: ETV can be an effective treatment option for encephalocoele-associated hydrocephalus, even in children under the age of 1 year. It may obviate the need for placement of CSF shunts that have a risk of infection and dysfunction. However, delayed failure of ETV may occur as seen in our first patient, indicating the need for careful and long-term follow-up.     

Endoscopic third ventriculostomy for chronic hydrocephalus after tuberculous meningitis

BACKGROUND: Cerebrospinal fluid diversion procedures are indicated in patients with hydrocephalus after tuberculous meningitis (TBM). We present 2 patients with hydrocephalus after TBM who were successfully treated with endoscopic third ventriculostomy (ETV). METHODS: Two patients had been diagnosed with hydrocephalus after TBM and had undergone ventriculoperitoneal shunt surgery for the same. They presented with multiple episodes of shunt dysfunction. Endoscopic third ventriculostomy was performed (twice for one patient), and the patients were evaluated clinically and radiologically after the procedure. RESULTS: On long-term clinical follow-up (3 and 2 years, respectively), both patients were asymptomatic after the ETV. The first patient was radiologically evaluated 7 months after the procedure and the second patient 2 years after the procedure. The first patient showed a decrease in ventricular size. The second patient did not show any significant change in the ventricular size. CONCLUSION: Endoscopic third ventriculostomy can be considered as a safe and long-lasting solution for hydrocephalus after chronic TBM.     

Sudden death secondary to delayed closure of endoscopic third ventriculostomy

An 11-year-old boy who underwent an initially successful endoscopic third ventriculostomy (ETV) died 4 months later secondary to acute hydrocephalus. This is the first report of sudden death caused by delayed closure of ETV. Guidelines to patients, parents and primary caregivers should include the possibility of ETV failure and encourage early neurosurgical consultation when symptoms of raised intracranial pressure occur.     

Changes in cerebrospinal fluid hydrodynamics following endoscopic third ventriculostomy for shunt-dependent noncommunicating hydrocephalus

OBJECT: The aim of this study was to analyze physiological changes in cerebrospinal fluid (CSF) dynamics following endoscopic third ventriculostomy (ETV) for shunt-dependent noncommunicating hydrocephalus. METHODS:. Clinical data obtained in 15 patients treated with ETV for shunt malfunction were analyzed. Magnetic resonance imaging studies demonstrated the obstruction of the ventricular system preoperatively. After ETV, the existing shunt system was removed and a continuous extraventricular drain, set at 30 cm H2O in height, was installed to measure daily amounts of CSF outflow. Cerebrospinal fluid dynamics after ETV were also evaluated using 111In-diethylenetriamine pentaacetic acid radioisotope cisternography in six of 15 patients within 1 month of the procedure. Three patients underwent cisternography at 6 months after ETV. Cisternograms were obtained at 1, 5, 24, and 48 hours after injection of the radioisotope. To study CSF absorptive capacity, ratios of radioisotope counts at 48 and 5 hours after injection were calculated (C48:C5). Seven of 15 patients had daily outflows of CSF of less than 20 ml; this volume decreased quickly within a few days. The other eight patients demonstrated an outflow of more than 150 ml of CSF for several days, three of whom had signs of transiently increased intracranial pressure. Their CSF outflow volume decreased gradually and symptoms improved within 1 week. Ratios of C48:C5 were within normal limits in five of six patients who had undergone cisternography 1 month after ETV. These ratios were decreased in all three patientswho had undergone cisternography at 6 months after ETV compared with that measured at 1 month after the procedure. CONCLUSIONS: Our data suggest that CSF dynamics convert from a shunt-dependent state to a shunt-independent state within I week following ETV in patients with shunt-dependent noncommunicating hydrocephalus. Nonetheless, intraventricular pressure does not decrease quickly in certain cases. Cerebrospinal fluid absorptive capacity or CSF circulation through the subarachnoid space may show further improvement several months after ETV.     

Hydrocephalus: overdrainage by ventricular shunts. A review and recommendations

Selected literature review of the clinical course of patients with ventricular shunts for hydrocephalus shows that the effects of cerebrospinal fluid overdrainage are subdural hematoma, craniosynostosis, slit ventricle syndrome, and low intracranial pressure syndrome. These occur sequentially at different age groups, but approximate averages of incidence and time of occurrence after first shunt reveal an overall incidence of 10%-12% for at least one of these appearing at 6.5 years after shunting. The basic etiology, diagnosis, and variety of treatment modalities available are reviewed, including the need for shunt closing intracranial pressure control. Included is a hydrocephalus program designed to minimize the need for long-term extracranial shunts and to maximize therapeutic intracranial procedures for hydrocephalus.     

Long-term control of hydrocephalus via endoscopic third ventriculostomy in children with tectal plate gliomas

OBJECTIVE: We report the control of hydrocephalus in children with presumed tectal plate gliomas by the use of endoscopic third ventriculostomy (ETV). METHODS: The hospital records, office charts, and imaging studies from children who underwent ETV at the Children's Hospital of Alabama were reviewed. Thirteen children with the diagnosis of tectal plate glioma and hydrocephalus were identified. ETV was the primary therapy instituted for all but one of these children. Successful treatment outcome was defined as shunt freedom, improvement in symptoms, and reduced ventricular size. RESULTS: Thirteen children underwent a total of 15 ETVs, and all children were shunt-free at their most recent follow-up examinations. One child underwent successful secondary ETV, one child underwent shunt removal concomitant with the initial ETV, and one child underwent shunt removal concomitant with secondary ETV. Symptoms and signs resolved in all patients. All postoperative cranial imaging studies revealed normal or reduced ventricular size as compared with preoperative cranial imaging scans. The median follow-up period was 31 months. CONCLUSION: In our surgical experience, ETV has been uniformly successful in the management of hydrocephalus caused by tectal plate gliomas in children. ETV should be considered the treatment of choice for hydrocephalus in pediatric patients with tectal plate gliomas.     

First experiences with a 2.0-microm near infrared laser system for neuroendoscopy

Nd:YAG, argon and diode lasers have been used in neurosurgical procedures including neuroendoscopy. However, many neurosurgeons are reluctant to use these lasers because of their inappropriate wavelength and uncontrollable tissue interaction, which has the potential to cause serious complications. Recently, a 2.0-microm near infrared laser with adequate wavelength and minimal tissue penetration became available. This laser was developed for endoscopic neurosurgical procedures. It is the aim of the study to report the initial experiences with this laser in neuroendoscopic procedures. We have performed 43 laser-assisted neuroendoscopic procedures [multicompartmental congenital, posthaemorrhagic or postinfectious hydrocephalus (n = 17), tumour biopsies (n = 6), rescue of fixed and allocated ventricular catheters (n = 2), endoscopic third ventriculostomy (ETV, n = 17) and aqueductoplasty (n = 1)] in 41 patients aged between 3 months and 80 years. The laser beam was delivered through a 365-microm bare silica fibre introduced through the working channel of a rigid endoscope. It was used for the opening of cysts, perforating the third ventricular floor, and for coagulation prior to and after biopsy. The therapeutic goals [creating unhindered cerebrospinal fluid (CSF) flow between cysts, ventricles and cisterns, sufficient tissue samples for histopathological diagnosis and catheter rescue] were achieved in 40 patients by the first and in 2 patients by a second neuroendoscopic operation. In one child, a CSF shunt was later required despite patency of the created stoma proven by magnetic resonance imaging (MRI). In another patient ETV was abandoned due to a tiny third ventricle. There was neither mortality nor transient or permanent morbidity. The authors conclude that the use of the 2.0-microm near infrared laser enables safe and effective procedures in neuroendoscopy.     

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.     

Management of cerebrospinal fluid disorders

Cerebrospinal fluid (CSF) disorders generally result in an excess volume of CSF within the neuraxis and an increase in intracranial pressure, defined as hydrocephalus. The treatment of hydrocephalus remains surgical today with the aim of CSF diversion, but the best technique and the best assessment of adequacy of treatment remains undecided in many cases. While shunt insertion remains the mainstay of treatment, neuroendoscopic interventions such as endoscopic third ventriculostomy (ETV) have proven successful for specific pathological conditions. Improved understanding of CSF dynamics and hydrocephalus pathophysiology together with technological advances in neuroimaging, neuronavigation and shunt hardware is leading to a more bespoke, patient-specific approach to this complex and multifactorial pathological entity. In this article we discuss the basics of our current understanding in CSF physiology, the presenting signs and symptoms of hydrocephalus and the modalities best employed to diagnose it. We also discuss treatment modalities and the tailored approach required to specific CSF disorders.     

Management of cerebrospinal fluid disorders

Cerebrospinal fluid (CSF) disorders generally result in an excess volume of CSF within the neuraxis and an increase in intracranial pressure, defined as hydrocephalus. The treatment of hydrocephalus remains surgical with the aim of CSF diversion, but the best technique and the best assessment of adequacy of treatment remains undecided in many cases. While shunt insertion remains the mainstay of treatment, neuroendoscopic interventions such as endoscopic third ventriculostomy (ETV) have proven successful for specific pathological conditions. Improved understanding of CSF dynamics and hydrocephalus pathophysiology together with technological advances in neuroimaging, neuronavigation, and shunt hardware is leading to a more bespoke, patient-specific approach to this complex and multifactorial pathological entity. In this paper we discuss the basics of our current understanding in CSF physiology, the presenting signs and symptoms of hydrocephalus and the modalities best employed to diagnose it. We also discuss treatment modalities and the tailored approach required to specific CSF disorders.     

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.