Keywords
endoscopic third ventriculostomy - shunt malfunction - hydrocephalus
Introduction
            Shunt placement was a standard treatment for patients with hydrocephalus. It is indicated
               for both communicating and noncommunicating types of hydrocephalus and for various
               etiologies of hydrocephalus, including infection; congenital malformations, such as
               aqueductal stenosis, congenital cysts, mega cisterna magna, and Arnold-Chiari malformation;
               hemorrhage; and tumor. The risk of shunt malfunction is quite high: 25 to 40% in the
               first year after shunt placement, 4 to 5% per year thereafter, and 81% of shunted
               patients require revision after 12 years. Therefore, it is considered that shunt failure
               is almost inevitable during a patient's life.[1]
               [2]
               [3]
               
            Endoscopic third ventriculostomy (ETV) for hydrocephalus is an important advancement
               for patients with hydrocephalus. The results are different when it is performed after
               shunt failure (secondary ETV) than when it is performed as an initial treatment modality
               for hydrocephalus (primary ETV). Complications that are reported with ETV include
               herniation syndromes and arrhythmia at the time of ETV, injury to the hypothalamic-pituitary
               axis and structures adjacent to floor of the third ventricle, including cranial nerves
               and major vessels, resulting in subarachnoid hemorrhage or ischemic stroke, with creation
               of ventriculostomy, as well as remote intracranial hemorrhage and infection and severe
               cognitive and psychiatric sequelae. In this study we assessed the usefulness of ETV
               in cases of ventriculoperitoneal shunt malfunction.
         Materials and Methods
            Ours was a prospective study. We enrolled 21 patients who underwent ETV for shunt
               malfunction presenting to our institution's neurosurgical service between January
               2011 and December 2012. All patients had a minimum of 9 months of follow-up. In this
               study shunt malfunction was diagnosed in all cases with increased ventricular size
               in comparison with baseline investigations of computed tomographic (CT) or magnetic
               resonance imaging (MRI) findings associated with at least one symptom or sign of increased
               intracranial pressure (headache, vomiting, deterioration of conscious level) or shunt
               obstruction (shunt chamber not compressible or refilling). Patients who had earlier
               undergone shunt surgery and now presented with shunt malfunction were enrolled in
               the study. Patients who underwent ETV as a primary procedure were excluded from the
               study. Outcome was considered successful if the patient became shunt independent.[4]
               
            The choice to proceed with ETV was based on discussion on the risks and benefits of
               the procedure with the patient and attendants. An incision was made over Kocher's
               point and burr hole was performed. A ventricular catheter was then used to cannulate
               the lateral ventricle. This track was then followed under direct visualization with
               a 0-degree scope. The floor of the third ventricle was then perforated and dilated
               with a 3F/4F Fogarty catheter. Bipolar cautery and irrigation were used as necessary
               for hemostasis. The interpeduncular and pontine cisterns were inspected for the Liliequist
               membrane or any other arachnoid adhesions. Liliequist membrane, if present, was punctured.
               The scope was then removed, the burr hole was plugged with gel foam, and a layered
               closure was subsequently performed. The correlation between the success of ETV and
               patient's age at surgery, etiology of hydrocephalus, number of shunt revisions, third
               ventricle anatomy, and third ventricle floor were tested with the chi-square test,
               with p < 0.05 indicating statistical significance.
         Results
            Over the study period, 21 patients underwent ETV for the treatment of shunt malfunction.
               Of these patients, 17 were males and 4 were females. The age range of patients in
               this study was 2 months to 53 years. The causes of hydrocephalus were aqueductal stenosis
               in seven patients, tumor in two patients, neurocysticercosis (NCC) in one patient,
               tuberculous meningitis (TBM) in six patients, posttraumatic in one patient, and postmeningitic
               in four patients. Communicating hydrocephalus occurred in 11 patients and noncommunicating
               hydrocephalus 10 patients.
            Success and Failure
            
            Total 13 patients (61.90%) underwent successful ETV whereas ETV failure was seen in
               8 (38.1%) patients .VP shunt insertion was done in all these patients.
            
            Variables Affecting Endoscopic Third Ventriculostomy Failure
            
            We evaluated different variables for significant effect on ETV failure. We evaluated
               success and failure with respect to age, number of shunt revisions, etiology of hydrocephalus,
               third ventricle anatomy, and third ventricle floor.
            
            
               
               - 
                  
                  
                     Effect of age: ETV was successful in 33.34% of patients aged ≤ 2 years and in 73.33% of patients
                     aged > 2 years. However, the difference between success rates in both the groups was
                     not statistically significant (p = 0.088). 
- 
                  
                  
                     Effect of shunt revisions: Patients were divided into two groups: (1) patients who underwent one shunt surgery
                     prior to ETV and (2) patients who underwent two or more shunt surgeries prior to ETV.
                     In the first group, 50% patients had successful outcome, whereas in the second group
                     77.78% patients had successful outcome after ETV. The difference between the two groups
                     was not statistically significant (p = 0.399). [Table 1] shows distribution of patients according to age and number of shunt revisions prior
                     to ETV. 
- 
                  
                  
                     Effect of etiology of hydrocephalus: Total 11 patients were found to have communicating hydrocephalus whereas 10 patients
                     had noncommunicating hydrocephalus. In communicating hydrocephalus group, 45.45% patients
                     had successful ETV. In noncommunicating group, the success rate was higher (80%).
                     However, the difference between success rates in both the groups was not statistically
                     significant (p = 0.104). 
               
                  Table 1 
                     Distribution of patients according to age and number of shunt revisions prior to ETV
                     
                  
                     
                     
                        
                        |  | ETV Success | ETV Failure | 
                     
                  
                     
                     
                        
                        | No. of patients (n = 21) | 13 | 8 | 
                     
                     
                        
                        | Age (y) | < 2 | 2 | 4 | 
                     
                     
                        
                        | > 2 | 11 | 4 | 
                     
                     
                        
                        | Shunt revisions prior to ETV | 1 | 6 | 6 | 
                     
                     
                        
                        | > 1 | 7 | 2 | 
                     
               
               
               
               Abbreviation: ETV, endoscopic third ventriculostomy.
               
                
            
            
            
            In communicating hydrocephalus group, 66.67% patients who presented with TBM with
               hydrocephalus had successful outcome after ETV. Nearly 25% of patients with pyogenic
               meningitis as a cause of hydrocephalus had successful outcome. ETV failed in patient
               who developed hydrocephalus following trauma (head injury).
            
            In noncommunicating hydrocephalus group, the causes for hydrocephalus in patients
               enrolled in our study were aqueductal stenosis, tumor, and fourth ventricle NCC.
            
            Nearly 71.42% patients with aqueduct stenosis who presented with shunt malfunction
               had a successful outcome after ETV. Nearly 100% patients in whom tumor was the cause
               of hydrocephalus had successful outcome. One patient presented with fourth ventricle
               NCC with shunt malfunction and had a successful outcome after ETV. [Table 2] shows distribution of patients according to etiology of hydrocephalus
            
            
               
                  Table 2 
                     Distribution of patients according to etiology of hydrocephalus
                     
                  
                     
                     
                        
                        |  | ETV success | ETV failure | 
                     
                  
                     
                     
                        
                        | Communicating hydrocephalus (11) | 5 | 6 | 
                     
                     
                        
                        | TBM | 4 | 2 | 
                     
                     
                        
                        | Postpyogenic meningitis | 1 | 3 | 
                     
                     
                        
                        | Posttraumatic | 0 | 1 | 
                     
                     
                        
                        | Noncommunicating hydrocephalus (10) | 8 | 2 | 
                     
                     
                        
                        | Congenital | 5 | 2 | 
                     
                     
                        
                        | Tumor | 2 | 0 | 
                     
                     
                        
                        | 4th ventricle NCC | 1 | 0 | 
                     
               
               
               
               Abbreviations: ETV, endoscopic third ventriculostomy; NCC, neurocysticercosis; TBM,
                  tuberculous meningitis.
               
                
            
            
            
            Endoscopic Findings
            
            
               
               - 
                  
                  
                     Effect of third ventricle anatomy: Endoscopic observations made during the procedure showed normal anatomy of the third
                     ventricle in 9 patients and indistinct anatomy in 12 patients. Nearly 77.78% patients
                     who had normal third ventricle anatomy observed during endoscopy had successful outcome
                     whereas 50% patients who were having indistinct anatomy observed during the procedure
                     had successful outcome. The difference between the two groups was not statistically
                     significant (p = 0.195). 
- 
                  
                  
                     Effect of third ventricle floor: Thickened third ventricle floor was found in 15 patients while performing ETV. Nearly
                     53.33% patients with thickened third ventricle floor had successful outcome. Six patients
                     had normal third ventricle floor. Nearly 83.33% patients in this group had a successful
                     outcome after the procedure. The difference between the two groups was not statistically
                     significant (p = 0.201). [Table 3] shows distribution of patients according to endoscopic findings. 
               
                  Table 3 
                     Distribution of patients according to endoscopic findings
                     
                  
                     
                     
                        
                        |  | ETV success | ETV failure | 
                     
                  
                     
                     
                        
                        | 3rd ventricle anatomy | 
                     
                     
                        
                        | Normal | 7 | 2 | 
                     
                     
                        
                        | Indistinct | 6 | 6 | 
                     
                     
                        
                        | 3rd ventricle floor | 
                     
                     
                        
                        | Normal | 5 | 1 | 
                     
                     
                        
                        | Thickened | 8 | 7 | 
                     
               
               
               
               Abbreviation: ETV, endoscopic third ventriculostomy.
               
                
            
            
            Discussion
            ETV is a safe and effective procedure for the treatment of appropriately selected
               patients. Our overall success rate of 61.90% patients is comparable with other studies.
               Buxton et al[5] reported overall success rates of 52%. Cinalli et al[4] reported ETV success in 76% patients whereas Marton et al[6] reported 64% overall ETV success.
            In our study the procedure was successful in 80% of noncommunicating hydrocephalus
               and 45.45% of communicating hydrocephalus. This result is comparable with the previous
               studies. Buxton et al[5] reported success rate of 73% in noncommunicating hydrocephalus and 46% in communicating
               hydrocephalus. In our study patients with aqueductal stenosis who presented with shunt
               malfunction had high success rate (80%).This is at par with that reported by the previous
               studies. In a study by Boschert et al,[7] 82% of their patients remained shunt free after procedure for aqueductal stenosis.
               In another study, O'Brien et al[1] reported a success rate of 68% with the patients having aqueductal stenosis. In
               our study, a history of pyogenic meningitis was associated with low success rate (25%).
               Our results matched those reported by Fukuhara et al[8] who also reported low success rate in these cases. One of the most commonly cited
               preoperative factors that predict outcome is the etiology of the hydrocephalus. However,
               Lee et al[3] categorized hydrocephalus according to etiology, including neoplasm, infection,
               trauma, malformation, and other causes, and found no statistical significance between
               hydrocephalus etiology and ETV outcome. In our study we also did not find significant
               correlation between etiology and ETV outcome. This can be attributed to fewer number
               of patients enrolled in the study.
            Patients who had TBM and presented with shunt malfunction had a success rate of 66.67%
               in our study. This rate is at par with the rate ranging from 41 to 81% reported by
               studies.[9]
               [10] Success rates reported for ETV in patients aged ≤ 2 years vary from 0 to 83% with
               a mean of 47.8%, which is significantly lower than the success rate in older children.[11]
               [12]
               [13]
               [14] Marton et al[6] reported that age at the time of secondary ETV has no statistically significant
               effect. In our series ETV was successful in 33.34% patients aged ≤ 2 years. In our
               study age was not found to be significant predictor of outcome of success of ETV in
               patients with shunt malfunction. This is in accordance to the aforementioned study.
            Defining Success after Endoscopic Third Ventriculostomy
            
            Successful outcome was considered when the patient became shunt independent. In the
               existing literature, success of ETV has been most commonly defined as enduring shunt
               independence after the procedure.[11]
               [13]
               [14]
               15
               
            
            Removal of Shunt
            
            We removed ventriculoperitoneal shunt in all patients who underwent ETV. In our study
               we encountered shunt tract hematoma in one patient after shunt removal. Removal of
               shunt can be decided during ETV as we can look for whether shunt tip is free or is
               struck in the choroid plexus. Shunt removal in patients in whom shunt is present for
               long time is prone for difficult removal, so shunt can be ligated in such cases.
            
            Complications of Endoscopic Third Ventriculostomy
            
            There were three complications (14.2%) associated with ETV and shunt removal in our
               series. Other series have reported complication rates ranging from 6 to 14%.[11]
               [13]
               [14] Hemorrhage was seen in two patients intraoperatively during ETV, which was managed
               with irrigation and cautery. One patient had hemorrhage in shunt tract, which occurred
               while removal of shunt. Our complication rate is comparable with that reported by
               the previous studies.
            Conclusion
            In our study the use of ETV in patients with shunt malfunction resulted in shunt independence
               in 61.90% patients. Age, etiology, type of hydrocephalus, and number of shunt revisions
               did not have a significant impact on the outcome of ETV in our study. Study with more
               number of patients will further elucidate the relation of these factors with ETV outcome.
               ETV is a good procedure for patients who present with shunt malfunction. It is a technically
               demanding procedure and needs expertise. It has got its own complications, but it
               relieves a patient from the everlasting complications of shunt surgery.