Review A Review of Endoscopic Treatment of Hydrocephalus in Paediatric and Adult Patients Ji Min Ling, MB.ChB (UK), MMed Surgery (NUS), Rajendra Tiruchelvarayan, MBBS, FRCS (Neurosurgery) (Ireland) Department of Neurosurgery, National Neuroscience Institute, Singapore Abstract Endoscopic treatment for hydrocephalus started in the early 20th century, but could not thrive due to poor illumination and magnification of the scope. In the 1950s, ventriculoperitoneal (VP) shunt became widely acceptable as standard treatment for hydrocephalus owing to the invention of well-designed valves and discovery of silicone, a biocompatible material for manufacturing shunt catheters. However, shunting is still far from being an ideal treatment because of its associated complications such as catheter malposition, blockage, and over- or under-drainage of cerebrospinal fluid. The shunt revision rates remained high in recent series. At the same time, endoscopy has undergone tremendous improvement in the latter half of the century and has emerged as an attractive alternative since the early 1990s. The article described the usage of endoscopy in the treatment of hydrocephalus, such as endoscopic third ventriculostomy, fenestration of multi-loculated hydrocephalus, and fenestration of septum pellucidum prior to VP shunting. Keywords: Endoscopy, Hydrocephalus, Shunt, Third ventriculostomy HISTORY OF NEUROENDOSCOPY AND VENTRICULOSCOPY IN THE TREATMENT OF HYDROCEPHALUS Endoscopy has been employed by neurosurgeons since the early 20th century. In 1918, Walter Dandy was one of the first surgeons who used an endoscope to perform choroid plexectomy in four patients with communicating hydrocephalus1.The results were poor, as three out of four patients died. The disappointing result led Dandy to develop a new technique of fenestrating the floor of the third ventricle via a subfrontal approach2. However, this technique did not become popular because of the need to sacrifice an optic nerve to afford the exposure. In 1923, Fay and Grant were able to visualise and successfully photograph the interior of the ventricles of a child with hydrocephalus by using a cystoscope3. That same year, William Mixter, a urologist, became the first surgeon to perform an endoscopic third ventriculostomy (ETV). He used a urethroscope to examine the ventricles of a child with obstructive hydrocephalus, and fenestrated the floor of the third ventricle during this procedure4. In 1934, Putnam reattempted cauterisation of the choroid plexus with an endoscopic device for seven patients. The procedure was successful in at least three cases, and resulted in two deaths5. Nine years later Putnam reported his series of endoscopic choroid plexectomy in 42 patients. There were 10 perioperative deaths (25%) and 15 patients failed to respond, although 17 had successful relief of increased intracranial pressure6,7. Despite the various efforts that showed the potential utility of endoscope in the treatment of hydrocephalus, endoscopy did not gain favour in general neurosurgical practice because of the poor magnification and illumination, which made this technique unreliable and frustrating to perform. The beginning of the1950s marked the decline of endoscopic treatment of hydrocephalus when Nulsen and Spitz published their landmark report of cerebrospinal fluid (CSF) shunting with a ball-andcone valve as a viable treatment for hydrocephalus8. Proceedings of Singapore Healthcare Volume 22 Number 3 2013 203 Subsequent developments of improved valve designs and discovery of biocompatible and fatigue-free silicone as constructional material for the tubing resulted in higher success rates and lower mortality. Eventually, ventriculo-peritoneal (VP) shunting became the standard treatment for hydrocephalus. There was therefore no perceived need to develop neuroendoscopy for the treatment of hydrocephalus. While the utility of endoscopy in neurosurgery became sparse from the 1960s onwards, a few key technological advances have occurred that further improved the quality of the endoscope. In 1966, Hopkins and Storz developed a rigid endoscope that used a new type of lens, the SELFOC lens. This new technology obviated the need for the relay lenses while preserving light transmission9,10. These lenses also created a wider effective field of vision. The advent of charge-coupled devices (CCDs) marked another technological breakthrough. In 1969, George Smith and Willard Boyle invented the first CCDs at Bell Laboratories11. The CCDs are solid-state devices, usually a silicon chip, which are capable of converting optical data into electrical current, resulting in both improved quality of the transmitted images and decreased size of the endoscopic systems. Another important technological breakthrough was the development of fibre optics. Fibre optics allowed the light source to be separated from the rest of the endoscope12. Light could also be emitted from the tip of the endoscope without significant heating through one set of cables; while other specialised and coherently arranged cables could be constructed to conduct images without a loss of luminescence. The combination of brighter light sources and improved camera resolution significantly advanced the quality of endoscopy, which led to the subsequent reconsideration of its usage in neurosurgery. On the other hand, the dramatic success of CSF shunting was later on met with various complications such as hardware infection, improper placement of catheter, obstruction, and hydraulic mismanagement, i.e. over- or under-drainage13,14. Shunt failure rates remained high even in modern series. In recent publications with long follow-up periods of six to twenty years, shunt revision rates ranged from 32.5% in adult patients15 to 84.5% in the paediatric population16. This has resulted in a renewed interest in ETV in the 1990s, a then viable 204 option owing to the improved imaging capabilities of the endoscope17. A successful ETV negates the need for a permanent implant and all its associated problems mentioned above. ENDOSCOPIC THIRD VENTRICULOSTOMY Endoscopic third ventriculostomy (ETV) aimed to create a new communication between the ventricular system and the subarachnoid space, by fenestrating the floor of the third ventricle. Below is an example of a patient with obstructive hydrocephalus secondary to a pineal tumour treated with ETV. A middle aged lady presented with headache and unsteadiness for one month. Physical examination revealed papilloedema and an unsteady gait. In Figure 1 (please see overleaf ), the sagittal T1 post-contrast magnetic resonance imaging (MRI) showed an enhanced tumour in the pineal region causing obstructive hydrocephalus. The lateral and third ventricles were dilated but the forth ventricle was of normal size. The diagnosis was obstructive hydrocephalus secondary to a pineal region tumour. The dilated third ventricle and patency of the prepontine cistern made this case suitable for third ventriculostomy. The patient underwent ETV. Her symptoms improved after the operation. An MRI CSF flow study done on post-operative day three demonstrated net forward motions through the ventriculostomy, thus confirming its patency. Indications for ETV The pioneers of third ventriculostomy such as Hirsh18, Hoffman19, Patterson and Bergland20, recommended that the procedure should only be reserved for patients with obstructive or non-communicating hydrocephalus, in whom the normal mechanism of CSF absorption still exists at the arachnoid granulations. Indeed, the literature showed that ETV is more effective if the hydrocephalus is due to aqueductal stenosis21,22 or compression of aqueduct or fourth ventricle by tumours23–25. Causes of hydrocephalus such as meningitis, subarachnoid haemorrhage, and intraventricular haemorrhage have been used by some to exclude patients from ETV. However, these have recently become relative contraindications in view of the reduction of the morbidity and mortality of ETV. Recent publications have shown acceptable results of ETV compared to VP shunt when its indication was extended to patients with CSF infection26, normal Proceedings of Singapore Healthcare Volume 22 Number 3 2013 Endoscopic Third Ventriculostomy and Fenestration Fig. 1. A: Sagittal MRI, T1 sequence post-contrast showing an enhancing tumour in the pineal region causing obstructive hydrocephalus. The lateral and third ventricles were dilated but the forth ventricle was of normal size. B: Coronal MRI, T1 post-contrast showing similar findings. C: Axial MRI, FLAIR sequence, showing dilated lateral ventricles and CSF transudation. D: Axial MRI, FLAIR sequence, showing dilated third ventricle which made third ventriculostomy possible. MRI: Magnetic resonance imaging CSF: Cerebrospinal fluid FLAIR: Fluid attenuated inversion recovery Fig. 2. Snapshot of the navigation screen showing the selection of an entry point (lateral/ superficial crosshair) at the frontal region and a trajectory that will bring the endoscope to the foramen of Monro (medial/deep crosshair) and then to the floor of the third ventricle with minimum angulation. Proceedings of Singapore Healthcare Volume 22 Number 3 2013 205 Review pressure hydrocephalus (NPH)27, intraventricular haemorrhage28, subarachnoid haemorrhage29, and other communicating hydrocephalus30. Gangemi et al31 published ETV success rates of 73.4% (33/45) in patients with NPH and 60% (12/20) in patients with hydrocephalus secondary to infection or haemorrhage. The latest preliminary result of a randomised controlled trial in Brazil where patients with NPH were randomised to VP shunt (with a fixed pressure valve) or ETV, showed that the 12-months improvement rate for VP shunt was 77%, whereas only 50% of patients who underwent ETV showed improvement. The difference was statistically significant32. Therefore at this point, it remains difficult to advocate ETV as a standard or routine treatment for NPH. More work still needs to be done to refine the selection criteria and improve the understanding of the pathophysiology of communicating hydrocephalus. Apart from the aetiology of hydrocephalus, the success rate of ETV is also dependent on the age of patients. In particular, children who undergo ETV at less than six months of age are shown to have higher failure rate33–35. Surgical Anatomy and Technique Familiarity with the ventricular anatomy is essential in performing an ETV. A burr hole is made on a point at the frontal region that will project a straight line linking the foramen of Monro and the tuber cinereum, the part of the floor of the third ventricle to be perforated. Stereotactic navigation is often employed to select the entry point and determine the trajectory of the endoscope. Figure 2 is a snapshot of the navigation screen showing the selection of an entry point at the frontal region and a trajectory that will bring the endoscope to the foramen of Monro and then to the floor of the third ventricle with minimum angulation. The foramen of Monro is usually the first structure visualised after entry of the endoscope into the frontal horn of the lateral ventricle. The standard view of the foramen of Monro is illustrated in Figure 3. The choroid plexus of the lateral ventricle projects forward to the foramen, through which it passes before turning posteriorly to lie under the roof of the third ventricle. The septal vein is located anteromedially along the septum pellucidum. It joins the thalamostriate vein, located posterolaterally, at the posterior rim of the foramen of Monro to form the internal cerebral vein36. The scope is then advanced through the foramen of Monro. The endoscopic view of the floor of the third ventricle is illustrated in Figure 4 (please see overleaf ). The paired mammillary bodies marked the posterior limit of the floor of the third ventricle. The target of fenestration is the tuber cinereum, the area between the mammillary bodies and the infundibulum of the pituitary gland. It is important to note that the basilar artery and the oculomotor nerves are situated directly under the tuber cinereum. A probe is then passed through the working channel of the endoscope. Once the tip of the probe appears in view of the endoscope, it is Fig. 3. Standard endoscopic view of the right foramen of Monro. 206 Proceedings of Singapore Healthcare Volume 22 Number 3 2013 Endoscopic Third Ventriculostomy and Fenestration Fig. 4. Endoscopic view of the floor of the third ventricle. Table 1. Selected Publications of the Success Rates of ETV from 1990 to 2013. ^ success rate at 36 months; * 65% at one year, 52% at five years; f only included patients with tumour-related obstructive hydrocephalus. guided to the tuber cinereum where a fenestration is made. This has to be done carefully in order to avoid bleeding from the basilar artery or damage to the oculomotor nerve. The hole is then enlarged with a 3 mm Fogarty balloon. Figure 4 showed the fenestration being made by the probe. Once haemostasis is achieved, the endoscope is withdrawn gently. Success Rates According to Literature When Jones37 published his ETV series in 1990, the success rate was only 50%. Success is defined as adequate treatment of hydrocephalus without the need for further VP shunt. The subsequent two decades saw the improvement of success rates ranging from 65% to 78% due to better patient selection and improved techniques. Table 1 summarises some of the selected publications of ETV success rates from 1990 to 2013. As a result, ETV has become an attractive alternative to VP shunt. It obviates the complications associated with an implant such as infection, blockage, and overor under-drainage. The senior author’s personal experience in performing ETV is from 2007 to 2013. The commonest indications for surgery were brain tumours causing obstructive hydrocephalus. In the 10 adult patients who underwent ETV, there was a success rate of 70%. Surgical aids such as computer-based neuro-navigation were used to enhance the safety of surgery. In his experience with eight paediatric patients, the success rate was 75%. Importantly, there were no ETV related complications of haemorrhage or infection. Thus, ETV has been found to be a viable alternative to VP Proceedings of Singapore Healthcare Volume 22 Number 3 2013 207 Review shunting in carefully selected cases. fenestration followed by VP shunt. COMPLICATIONS OF ENDOSCOPIC THIRD VENTRICULOSTOMY The common complications associated with ETV include bleeding and infection. Bleeding could occur from a number of sources, such as injury of the basilar artery, intraventricular vessels, and brain parenchyma. Minor bleeding will usually stop with irrigation. Heavy bleeding may necessitate abandonment of the procedure and placement of an external ventricular drain. Infection can be subdivided into superficial skin infection and meningitis. The latter requires treatment with a longer duration of intravenous antibiotic. The rate of infection is approximately 2%38. The less common complications associated with ETV include leakage of cerebrospinal fluid, seizure, and hypothalamic injury leading to diabetes insipidus. According to a series of 193 ETVs published by Schroeder et al39, the mortality rate was 1%, permanent morbidity rate of 1.6%, and transient morbidity rate was 7.8%. In this series, the mortality and permanent morbidity occurred in the author’s early experience, thus indicating the steep learning curve associated with this procedure. A young girl presented with acute left hemiparesis associated with a headache. On physical examination, her power was 3/5. Computed tomography of the brain showed a large right basal ganglia haemorrhage which also discharged into the ventricle resulting in intraventricular haemorrhage. She underwent decompressive craniectomy, evacuation of the haematoma, and insertion of an external ventricular drain. Unfortunately, she developed secondary bacterial infection which finally led to a frank ventriculitis. As a result, she developed loculated hydrocephalus. In Figure 5, the MRI on the left shows a loculation within the right frontal horn. Doing a VP shunt required insertion of three intracranial catheters: one in the left lateral ventricle, one in the right lateral ventricle, and one in the loculated collection within the right frontal horn. This surgical option was not ideal, as more catheters would expose the patient to a higher risk of catheter malposition, blockage, and infection. Therefore, the patient underwent an endoscopic fenestration of the right frontal loculation and the septum pellucidum followed by placement of a ventricular catheter at the right frontal horn. In Figure 5, the MRI on the right taken post-operatively shows that both the lateral ventricles were adequately decompressed. UTILITY OF ENDOSCOPE IN MULTILOCULATED HYDROCEPHALUS Neuroendoscopy is also a useful treatment for multiloculated hydrocephalus. Endoscopy allowed fenestration of the loculations with minimal invasion. Below is an example of a patient with loculated hydrocephalus treated with endoscopic The common causes of multiloculated hydrocephalus are ventriculitis and intraventricular haemorrhage. Tumours that grow in the CSF pathway may also result in obstruction of CSF Fig. 5. Left: T2 sequence axial magnetic resonance imaging (MRI) showing loculation of the right frontal horn. Right: MRI taken after the endoscopic fenestration of the right frontal loculation and septum pellucidum. Both lateral ventricles were adequately decompressed. 208 Proceedings of Singapore Healthcare Volume 22 Number 3 2013 Endoscopic Third Ventriculostomy and Fenestration flow and isolated hydrocephalus. It is a difficult condition to treat and in most patients multiple surgical revisions are required. Historically, multiloculated hydrocephalus was usually treated with multiple intracystic and intraventricular catheters, and occasionally with microsurgical open cyst fenestration49–52. The placement of multiple catheters is associated with many problems. The ventricular anatomy is frequently distorted in patients with multiloculated hydrocephalus. Malposition of the catheter is therefore common, especially without the aid of computerised surgical navigation tools. Even when navigation is employed, drainage of CSF after insertion of the first catheter may result in a significant shift of the intracranial structures, thus rendering the navigation unreliable for subsequent catheters insertion. The cost of implants also increases significantly when more shunt tubings and valves are required. When a patient with multiple shunts presents with infection, all the shunts need to be removed. It becomes especially complicated when a patient with multiple shunts present with a blocked shunt, as it is very difficult to diagnose which catheter is blocked. It minimises the size of skull opening; a burr hole is usually sufficient. It reduces brain retraction and trauma and facilitates deep access mainly through preformed and existing cavities53. Combined with navigation, fenestration of the membrane could be made at the appropriate locations and the ventricular catheter could also be guided into a good position. In patients with tumour in the third ventricle causing a blockage of the bilateral foramen of Monro, fenestration of the septum pellucidum could re-establish the communication of both lateral ventricles, thus avoiding placement of two shunts. Multiple shunts can be avoided if the multiloculated hydrocephalus is converted to a unilocular hydrocephalus. Open microsurgical fenestration is an option, but it is invasive with associated complications. A craniotomy is required and a large corticectomy is frequently needed to access the ventricles. The advantages of using an endoscope to examine the ventricle are obvious. A middle aged lady presented with headache associated with features of increased intracranial pressure. An MRI showed a large thalamic tumour causing an obstruction of the bilateral foramen of Monro. She underwent endoscopic fenestration of the septum pellucidum followed by insertion of VP shunt. Figure 6 shows the steps involved in the insertion of a ventriculoscope for fenestration ENDOSCOPIC FENESTRATION OF THE SEPTUM PELLUCIDUM In cases where both the foramina of Monro are blocked, endoscopic fenestration of the septum pellucidum could be performed prior to shunting, thus avoiding the insertion of two intraventricular catheters. Below is an example of a patient who has obstructive hydrocephalus secondary to a large thalamic tumour, treated with endoscopic fenestration of the septum pellucidum followed by VP shunt. Fig. 6. A: Picture showing a rigid ventriculoscope. The patient has been positioned and draped for a ventriculoperitoneal shunt surgery. B: A burr hole has been made. The lateral ventricle was first cannulated with a Dandy needle. C: The ventriculoscope was introduced through the same path created by the Dandy needle. D: The camera was introduced. Proceedings of Singapore Healthcare Volume 22 Number 3 2013 209 Review Fig. 7. The ventriculoscope has been registered to the navigation system. The axial and coronal views of the navigation screen showed that the structure seen from the scope camera was the septum pellucidum. Fig. 8. Endoscopic picture showing fenestration of the septum pellucidum. After the septal fenestration was done, the thalamic tumour was endoscopically biopsied. of septum pellucidum. We preferred to use the rigid ventriculoscope because of superior image quality. The patient was positioned and draped as for VP shunt insertion. A frontal burr hole was made. The lateral ventricle was first cannulated by a Dandy needle. The rigid ventriculoscope was then introduced, following the same path created by the Dandy needle. The camera was then introduced. The endoscopic view confirmed the successful placement of the scope within the frontal horn of the lateral ventricle. Prior to 210 inserting the ventriculoscope, the scope was registered to the navigation system. In Figure 7, the axial and coronal MRI on the navigation screen showed that the structure seen on the endoscopic camera was the septum pellucidum. The septum was then fenestrated with a monopolar probe and dilated with the scope itself. Figure 8 shows the fenestration made at the septum pellucidum. After the septal fenestration was done, the thalamic tumour was biopsied endoscopically. Her headache resolved after the surgery. Proceedings of Singapore Healthcare Volume 22 Number 3 2013 Endoscopic Third Ventriculostomy and Fenestration CHALLENGES IN PROMOTING THE USE OF ENDOSCOPY IN THE TREATMENT OF HYDROCEPHALUS The current recognised indications of endoscopic treatment of hydrocephalus are ETV for obstructive hydrocephalus, and endoscopic fenestration in multiloculated hydrocephalus. Recent publications have shown acceptable results of ETV compared to VP shunt when its indication was extended to patients with CSF infection26, NPH27, intraventricular haemorrhages28, subarachnoid haemorrhage29, and other communicating hydrocephalus30. With a shunt free rate of 65% to 75%, ETV has shown superior results compared to VP shunt, when used for good indications. The volume of endoscopic work in a typical neurosurgery department is relatively low. It is also associated with a steep learning curve. As a result, endoscopic surgery is only practiced by a few neurosurgeons who have developed subspecialised interest. Most neurosurgeons would still prefer to treat hydrocephalus with VP shunt, an established surgery with relatively simple technique. However, as the literature on endoscopy usage and its advantages are further disseminated, it is likely that more neurosurgeons will consider it as an option. Capital investment is needed in the purchase of the endoscopic system, including the endoscopic camera, telescope, light source, and recording device. The huge sum of money required often serves as an obstacle for small neurosurgical departments to employ neuroendoscopy. Nevertheless, the capital investment could be offset by the money saved from not using additional implants. Potential savings could be derived from the avoidance of treatment complications of VP shunt such as infection, and revision surgeries required for blockage and over- or under-drainage. CONCLUSION The improvement of the image quality of endoscope, as well as the endoscopic tools such as diathermy and scissors have revived the use of endoscopy in treatment of hydrocephalus. The aim is to treat hydrocephalus without the use of an implant, such as a shunt, thus avoiding the complications associated with a permanent implant such as infection and malfunction. Endoscopic surgeries employed in the treatment of hydrocephalus are ETV, fenestration of multi-loculated hydrocephalus, and fenestration of septum pellucidum before shunting. Endoscopic treatment of hydrocephalus may become more widely accepted and practiced with the increase in success rate, reduction of morbidity and mortality, and emergence of new evidences supporting its role in treatment of communicating hydrocephalus. Further research is required to refine the selection criteria in order to identify patients with communicating hydrocephalus who will likely respond to ETV. REFERENCES 1. Dandy WE. 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Multiloculated hydrocephalus in infants. Neurosurgery 1981;8(6):641–6. 50. Jamjoom AB, Mohammed AA, al-Boukai A, Jamjoom ZA, Rahman N, Jamjoom HT. Multiloculated hydrocephalus related to cerebrospinal fluid shunt infection. Acta Neurochir (Wien) 1996;138(6):714–9. 51. Nida TY, Haines SJ. Multiloculated hydrocephalus: Craniotomy and fenestration of intraventricular septations. J Neurosurg 1993;78(1):70–6. 52. Sandberg DI, McComb JG, Krieger MD. Craniotomy for fenestration of multiloculated hydrocephalus in pediatric patients. Neurosurgery 2005;57:100–6. 53. Schulz M, Bohner G, Knaus H, Haberl H, Thomale UW. Navigated endoscopic surgery for multiloculated hydrocephalus in children. J Neurosurg Pediatr 2010;5(5):434–42. Proceedings of Singapore Healthcare Volume 22 Number 3 2013
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