Abstract

Background: The objective of this research article is to compare endoscopic treatment versus shunting procedures for failed endoscopic third ventriculostomies (ETVs) in pediatric patients with hydrocephalus.

Methods: We did a systematic review based on preferred reporting items for systematic reviews and meta-analyses guidelines on Studies involving pediatric patients (aged 0–18 years) diagnosed with hydrocephalus, reporting on the use of repeat ETV (Re-ETV) or Ventriculoperitoneal shunting (VPS) as a treatment option following failed ETV. Comparative studies, including randomized controlled trials, cohort studies, and any prospective studies, are included. Studies published in the English language conducted between 2001 and 2023 are included.

Results: Forty articles were selected for full-text review. Out of which nine articles that clearly addressed the topic of Re-ETV and/or VPS placement after failure of primary ETV were deemed suitable for analysis. A data set of 663 patients was analyzed. Re-ETV was done in 220 patients (33.18%) and VPS Placement was done in 443 patients (66.81%). The primary ETV failure rates ranged from 16.6 to 60.89%. There was a higher failure rate of Re-ETV (74.98%) compared to VPS (22.26%) indicating that VPS is generally more successful as a secondary intervention. The presence of hemorrhage during primary ETV suggested more benefit from VPS placement rather than Re-ETV (P

Conclusion: Our systematic review suggests that VPS placement is the more prevalent choice after primary ETV failure, likely due to its higher overall success rate and the nature of complications. The wide variability in failure rates and follow-up durations suggests that treatment outcomes can differ greatly between patients and studies. Decisions regarding secondary interventions should be individualized, considering patient-specific factors such as age, complications, and timing of intervention.

Keywords: Endoscopic third ventriculostomy, Endoscopic third ventriculostomy failure, Hydrocephalus, Pediatric neurosurgery, Ventriculoperitoneal shunt

INTRODUCTION

Hydrocephalus, characterized by the abnormal accumulation of cerebrospinal fluid (CSF) within the ventricles of the brain, poses a significant challenge in pediatric neurosurgery.[ 1 ] Hydrocephalus is one of the most common diseases treated by pediatric neurosurgeons. Being more common in developing countries, the prevalence of pediatric hydrocephalus is roughly one case per 1000 births.[ 2 ] Although hydrocephalus is usually managed by endoscopic third ventriculostomy (ETV) or ventriculoperitoneal shunt (VPS) placement, depending on various factors, the choice of treatment for cases of failed ETV remains a subject of ongoing debate.

ETV has emerged as a minimally invasive alternative to shunting for the treatment of hydrocephalus, particularly in pediatric patients.[ 3 ] It involves creating a permanent CSF diversion pathway by creating an opening in the floor of the third ventricle, allowing CSF flow to bypass the obstruction. Although ETV has shown promising results as an initial treatment option, a significant proportion of patients experience failure or inadequate CSF diversion.[ 4 ] Age, etiology, and the surgeon’s experience are significant factors that affect the success and complication rates of ETV, according to prior publications.[ 5 ] In certain trials, infants with aqueductal stenosis had higher success rates of up to 90%.[ 6 , 7 ] Patients with post-infectious and post-hemorrhagic hydrocephalus, as well as those who had previously experienced VPS failures, showed lower success rates.[ 8 - 10 ]

The placement of a VPS is a routine neurosurgery procedure, with approximately 30,000 shunt operations performed annually in the United States.[ 11 ] However, the rate of complications is high. Within the 1^st year following the first shunt insertion, VPS failure rates have been estimated at 11–25%.[ 12 , 13 , 36 ] Shunt blockage and infection are the most frequent causes of shunt failure in both pediatric and adult populations and more so in the pediatric population.[ 14 , 15 , 31 ] Early shunt failures are typically caused by infections, whereas late shunt failures are typically caused by catheter occlusion.[ 16 ]

ETV failure can be attributed to a variety of factors, including ventriculostomy stoma closure by new arachnoid granulation tissues, second membrane relics within the stoma, CSF absorption failure, CSF infection/high protein, and improper selection of patients.[ 17 ] In such cases, revision surgery is often required to ensure adequate CSF drainage and to alleviate the symptoms associated with hydrocephalus. Two primary approaches are commonly employed: repeat ETV (Re-ETV) or placement of a VPS.[ 18 , 28 ]

The aim of this research study is to compare the endoscopic treatment versus shunting procedures for failed ETVs in pediatric patients with hydrocephalus. By critically analyzing the existing literature and sharing our findings, we sought to provide evidence-based insights into the optimal management strategy for this challenging patient population. Nevertheless, shunting procedures remain a well-established and reliable option for CSF diversion in cases in which endoscopic treatment is unsuccessful.[ 19 , 29 ] By identifying the strengths and limitations of both techniques, we hope to guide clinical decision-making and optimize treatment strategies for pediatric patients with hydrocephalus with failed ETVs.

METHODOLOGY

This systematic review aimed to compare the effectiveness of Re-ETV and VPS as therapeutic interventions in pediatric patients with hydrocephalus who have experienced failed ETV. Our study follows a systematic and comprehensive approach to review the existing evidence on this topic.

Search strategy

A thorough literature search was conducted to identify relevant studies published up to the date of the initiation of the review. Databases including PubMed, Embase, and Cochrane Library were systematically searched. The search strategy incorporated a combination of keywords and Medical Subject Headings terms related to pediatric hydrocephalus, ETV failure, Re-ETV, and VPS.

The search strategy specified in Table 1 along with the search results.

Table 1:

Search strategies for different databases.

Inclusion criteria

Studies involving pediatric patients (aged 0–18 years) diagnosed with hydrocephalus reported the use of ReETV or VPS as a treatment option following failed ETV. Comparative studies, including randomized controlled trials (RCTs), cohort studies, and prospective studies, were included. Studies published in English between 2001 and 2023 were included in the study.

Exclusion criteria

Non-comparative studies, case reports, and case series studies involving adult or mixed populations without separate pediatric data were excluded from the study. Studies with insufficient data, reporting, or published in languages other than English were also excluded from the study.

Study selection

Two independent reviewers screened titles, abstracts, and full-text articles for eligibility. Discrepancies were resolved through discussion or consultation with a third reviewer.

Data extraction

For the extraction of data from the studies, and therefore, the evaluation of each study’s significance in the context of our review, a three-step approach was used. After conducting a literature database search, two authors exported studies found using the EndNote Reference Library program. Furthermore, we identified and eliminated duplicate studies. Second, the remaining full-text papers were carefully screened, and only studies that met our inclusion criteria were included for further analysis. We started the screening process by shortlisting each study based on its title and abstract.

Finally, after shortlisting the studies, we read them in full to ensure their relevance and compliance with our predefined inclusion criteria. Any disagreements or doubts in the selection process were addressed through discussions among the authors. The outcomes of each included study (e.g., success rates, complications, and reoperation rates) were then extracted. We extracted data using Microsoft Excel, and all data and values were logged on a sheet for later use.

Quality assessment

Quality assessment, which was required to ensure the credibility and authenticity of our research, was performed using the Cochrane Collaboration’s risk of bias (RoB 2.0) method for RCTs and Critical Appraisal Skills Program (CASP) for prospective studies (CASP for RCTs as well as prospective studies). Quality assessment tools analyze internal validity and a variety of potential biases, including randomization, allocation concealment and publishing, attrition, reporting, selection, detection, and others. TheCASP is a well-known collection of tools and standards for evaluating the quality of research, particularly prospective studies. Many components or domains of research are considered when evaluating the quality of prospective studies using CASP. These factors included study objectives and Design, Sample Size and Selection, Data collection, Bias and Confounding, Ethical Considerations, Data Analysis, Results and Conclusions, and Applicability. The overall evaluation provides an overall evaluation of the quality of the study, including its strengths and faults. We utilized and filled the CASP assessment in PDF forms for each study which are attached as the supplementary files. Each study’s summary provided information about its design, population and demographics, interventions, and results.

Data synthesis

The findings from individual studies were assessed narratively to provide a comprehensive overview of the evidence. The key similarities and differences between the Re-ETV and VPS are summarized in a tabulated form.

RESULTS

In our study, we selected 40 articles for full-text review from a combined database of PubMed, EMBASE, and Cochrane. Among these, nine articles specifically addressed the topic of Re-ETV and/or VPS placement following the failure of primary ETV, rendering them suitable for analysis. A total of 31 articles were excluded due to ambiguity concerning the primary outcome. The selection criteria for the articles is summarized in the Figure 1 .

Figure 1:

Preferred reporting items for systematic reviews and meta-analyses flow diagram.

Our systematic review included 663 pediatric patients who experienced primary ETV failure. The data indicated that 220 patients (33.18%) underwent Re-ETV after the failure of primary ETV, while 443 patients (66.81%) received VPS placement. Table 2 summarizes the major studies included in our review, revealing a predominant preference for VPS placement in response to failed ETV in children.

Table 2:

Study characteristics and demographics along with management of primary ETV failure.

A review of the secondary outcomes demonstrated notable variability across studies. The primary ETV failure rate ranged from 16.66% to 60.89%, with a mean failure rate of 40.58%, suggesting differences in the effectiveness of primary ETV among studies.

The time to failure of primary ETV varies significantly, ranging from <2 months to 28.92 months, with a mean duration of 6.94 months. Similarly, the duration until failure of the secondary procedure showed variability, spanning from 0.19 to 65.3 months, with a mean of 15.77 months. Our pooled data set analysis showed that a higher number of Re-ETVs (n = 112) failed compared to VPSs (n = 55), which was statistically significant (OR = 4.86 CI = 3.205– 7.383, P < 0.05).

A comparative analysis between Re-ETV and VPS following ETV failure is shown in Table 3a and b . The incidence of infection and hemorrhage varied among studies for both ReETV and VPS post-ETV, reflecting the heterogeneous nature of complications associated with ETV failure. Nonetheless, the data in Table 4 indicate that a previous history of hemorrhage emerged as a factor influencing the trend toward VPS over Re-ETV.

Table 3a:

Secondary outcomes.

Table 3b:

Secondary procedures.

Table 4:

Comparison of Re-ETV and VPS post-ETV failure.

DISCUSSION

This systematic review aimed to compare the efficacy and outcomes of Re-ETV versus VPS as secondary interventions following the failure of primary ETV in pediatric patients with hydrocephalus. The findings of our review suggest that while both Re-ETV and VPS are utilized as secondary interventions, there are notable differences in the reasons for choosing them and their success rates. Notably, Kulkarni et al. (2017)[ 19 ] reported comparable outcomes between primary VPS and VPS post-ETV, while Arynchyna-Smith et al. (2022)[ 1 ] observed a 29.5% 1-year Re-ETV success rate. Our analysis revealed that VPS placement was more prevalent (66.81%) than Re-ETV (28.95%) after primary ETV failure. This observation aligns with previous studies indicating a higher adoption of VPS in pediatric hydrocephalus management due to its established efficacy and safety profile.[ 20 , 21 ] However, it is crucial to note that the decision between Re-ETV and VPS should be guided by patient-specific factors, including age, comorbidities, and anatomical considerations.

Variability in the success rates of Re-ETV and VPS was evident in our analysis, echoing the heterogeneous nature of outcomes reported in the literature. Warf et al. (2005)[ 34 ] identified post-infectious etiology as a predictor of Re-ETV failure (62%), whereas Javadpour et al. (2001)[ 12 ] emphasized etiology over age in ETV success. The available data have reported favorable outcomes with Re-ETV,[ 22 ] whereas others have indicated higher success rates with VPS placement.[ 23 ] Notably, our findings align with the notion that while ReETV may offer a less invasive option, it comes with a higher risk of failure, as suggested by the wide range of failure rates observed. Lam et al. (2014)[ 20 ] and Torres et al. (2022)[ 33 ] further illustrated this variability, with VPS preference post-failure (Lam) and 61.53% Re-ETV success in select cohorts (Torres). Similarly, the variability in VPS failure rates after ETV failure underscores the complexity of comparing the efficacy of the two interventions. However, it is evident that both interventions (Re-ETV and VPS placement) face challenges in achieving consistent success, emphasizing the need for further research to elucidate the factors influencing treatment outcomes and guide clinical decision-making.[ 24 , 25 ]

Furthermore, our analysis revealed significant variability in the timing of primary ETV failure and subsequent interventions, corroborating existing literature.[ 26 ] Romeo et al. (2013)[ 27 ] noted 77% primary ETV success with Re-ETV preference for failures, while Kono et al. (2022)[ 16 ] advocated Re-ETV with choroid plexus cauterization as salvage therapy. This variability underscores the unpredictable nature of hydrocephalus progression and the importance of individualized management strategies tailored to each patient’s unique circumstances. The decision-making process regarding the choice between Re-ETV and VPS post-ETV failure is multifaceted,[ 20 , 22 ] emphasizing the importance of considering patient age, anatomical factors, and the history of complications when selecting the appropriate intervention. Gallo et al. (2011)[ 8 ] highlighted age’s role in primary ETV success (39.1%), aligning with this need for personalized approaches. In addition, the variability in complication rates reported in our analysis further emphasizes the need for personalized management strategies to optimize patient outcomes.[ 27 , 32 ]

In addition, it is essential to acknowledge the influence of socioeconomic and geographical factors on the choice and outcome of interventions for pediatric hydrocephalus. Furthermore, there are disparities in access to healthcare services and specialized neurosurgical expertise, particularly in resource-limited settings.[ 20 , 22 ] These disparities may impact the availability of advanced procedures, such as Re-ETV and VPS, and contribute to variations in treatment outcomes among different patient populations. Addressing these disparities through initiatives aimed at improving access to neurosurgical care and enhancing healthcare infrastructure is crucial for ensuring equitable treatment outcomes for all pediatric patients with hydrocephalus. Future studies should address the limitations of the existing literature, particularly the lack of sufficient data on VPS failure rates post-ETV failure, to guide clinicians in selecting the most appropriate intervention for each patient.

Limitations

This systematic review has some limitations. The included studies varied significantly in design, patient groups, and follow-up periods, which hindered direct comparisons and data analysis. Furthermore, there was inconsistent reporting of VPS failure rates after ETV, making it difficult to make a thorough comparison between Re-ETV and VPS. The absence of uniform data on complications as well as the unaccounted-for influence of socioeconomic and geographical factors may mask discrepancies in access to care and outcomes. Many studies were retrospective, and follow-up lengths varied greatly, limiting the validity of our conclusions. Potential publication bias due to reliance on specific databases may have influenced the results. Finally, considerable gaps in the research, particularly regarding VPS failure rates post-ETV, highlight the need for more prospective trials with standardized outcome parameters.

Future implications

Future studies should aim to address the limitations of existing literature, particularly the lack of sufficient data on VPS failure rates post-ETV failure, to guide clinicians in selecting the most appropriate intervention for each patient.

CONCLUSION

This study offers valuable insights into the comparative efficacy and outcomes of Re-ETV versus VPS as secondary interventions following primary ETV failure in pediatric patients with hydrocephalus. Although shunting is performed more frequently in current clinical practice following failed ETV, available evidence does not definitively support one approach over the other. Further research is needed to identify optimal patient selection criteria for ReETV versus shunt.

Ethical approval:

The Institutional Review Board approval is not required.

Declaration of patient consent:

Patient’s consent was not required as there are no patients in this study.

Financial support and sponsorship:

Nil.

Conflicts of interest:

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation:

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Arynchyna-Smith A, Rozzelle CJ, Jensen H, Reeder RW, Kulkarni AV, Pollack IF. Endoscopic third ventriculostomy revision after failure of initial endoscopic third ventriculostomy and choroid plexus cauterization. J Neurosurg Pediatr. 2022. 30: 8-17

2. Bondurant CP, Jimenez DF. Epidemiology of cerebrospinal fluid shunting. Pediatr Neurosurg. 1995. 23: 254-58 discussion 259

3. Brockmeyer D, Abtin K, Carey L, Walker ML. Endoscopic third ventriculostomy: An outcome analysis. Pediatr Neurosurg. 1998. 28: 236-40

4. Cinalli G, Spennato P, Nastro A, Aliberti F, Trischitta V, Ruggiero C. Hydrocephalus in aqueductal stenosis. Childs Nerv Syst. 2011. 27: 1621-42

5. Drake JM, Kulkarni AV, Kestle J. Endoscopic third ventriculostomy versus ventriculoperitoneal shunt in pediatric patients: A decision analysis. Childs Nerv Syst. 2009. 25: 467-72

6. Duru S, Peiro JL, Oria M, Aydin E, Subasi C, Tuncer C. Successful endoscopic third ventriculostomy in children depends on age and etiology of hydrocephalus: Outcome analysis in 51 pediatric patients. Childs Nerv Syst. 2018. 34: 1521-8

7. Dusick JR, McArthur DL, Bergsneider M. Success and complication rates of endoscopic third ventriculostomy for adult hydrocephalus: A series of 108 patients. Surg Neurol. 2008. 69: 5-15

8. Gallo P, Szathmari A, De Biasi S, Mottolese C. Endoscopic third ventriculostomy in obstructive infantile hydrocephalus: Remarks about the so-called ‘unsuccessful cases’. Pediatr Neurosurg. 2011. 46: 435-41

9. Gangemi M, Donati P, Maiuri F, Longatti P, Godano U, Mascari C. Endoscopic third ventriculostomy for hydrocephalus. Minim Invasive Neurosurg. 1999. 42: 128-32

10. Gianaris TJ, Nazar R, Middlebrook E, Gonda DD, Jea A, Fulkerson DH. Failure of ETV in patients with the highest ETV success scores. J Neurosurg Pediatr. 2017. 20: 225-31

11. Hopf NJ, Grunert P, Fries G, Resch KD, Perneczky A. Endoscopic third ventriculostomy: Outcome analysis of 100 consecutive procedures. Neurosurgery. 1999. 44: 795-804 discussion 804-6

12. Javadpour M, Mallucci C, Brodbelt A, Golash A, May P. The impact of endoscopic third ventriculostomy on the management of newly diagnosed hydrocephalus in infants. Pediatr Neurosurg. 2001. 35: 131-5

13. Jeng S, Gupta N, Wrensch M, Zhao S, Wu YW. Prevalence of congenital hydrocephalus in California, 1991-2000. Pediatr Neurol. 2011. 45: 67-71

14. Jones RF, Kwok BC, Stening WA, Vonau M. Neuroendoscopic third ventriculostomy. A practical alternative to extracranial shunts in non-communicating hydrocephalus. Acta Neurochir Suppl. 1994. 61: 79-83

15. Khan F, Shamim MS, Rehman A, Bari ME. Analysis of factors affecting ventriculoperitoneal shunt survival in pediatric patients. Childs Nerv Syst. 2013. 29: 791-802

16. Kono M, Tsuda K, Yamashita M, Ihara S. Repeat endoscopic third ventriculostomy combined with choroid plexus cauterization as salvage surgery for failed endoscopic third ventriculostomy. Childs Nerv Syst. 2022. 38: 1313-9

17. Kulkarni AV, Drake JM, Kestle JR, Mallucci CL, Sgouros S, Constantini S. Endoscopic third ventriculostomy vs cerebrospinal fluid shunt in the treatment of hydrocephalus in children: A propensity score-adjusted analysis. Neurosurgery. 2010. 67: 588-93

18. Kulkarni AV, Riva-Cambrin J, Browd SR, Drake JM, Holubkov R, Kestle JR. Endoscopic third ventriculostomy and choroid plexus cauterization in infants with hydrocephalus: A retrospective hydrocephalus clinical research network study. J Neurosurg Pediatr. 2014. 14: 224-9

19. Kulkarni AV, Sgouros S, Constantini S. Outcome of treatment after failed endoscopic third ventriculostomy (ETV) in infants with aqueductal stenosis: Results from the international infant hydrocephalus study (IIHS). Childs Nerv Syst. 2017. 33: 747-52

20. Lam S, Harris D, Rocque BG, Ham SA. Pediatric endoscopic third ventriculostomy: A population-based study. J Neurosurg Pediatr. 2014. 14: 455-64

21. Limbrick DD, Baird LC, Klimo P, Riva-Cambrin J, Flannery AM. Pediatric hydrocephalus: Systematic literature review and evidence-based guidelines. Part 4 Cerebrospinal fluid shunt or endoscopic third ventriculostomy for the treatment of hydrocephalus in children. J Neurosurg Pediatr. 2014. 14: 30-4

22. McGirt MJ, Leveque JC, Wellons JC, Villavicencio AT, Hopkins JS, Fuchs HE. Cerebrospinal fluid shunt survival and etiology of failures: A seven-year institutional experience. Pediatr Neurosurg. 2002. 36: 248-55

23. Rahman MM, Khan SI, Khan RA, Islam R, Sarker MH. Endoscopic third ventriculostomy in children: Problems and surgical outcome: Analysis of 34 cases. Chin Neurosurg J. 2021. 7: 3

24. Reddy GK, Bollam P, Caldito G. Long-term outcomes of ventriculoperitoneal shunt surgery in patients with hydrocephalus. World Neurosurg. 2014. 81: 404-10

25. Riva-Cambrin J, Detsky AS, Lamberti-Pasculli M, Sargent MA, Armstrong D, Moineddin R. Predicting postresection hydrocephalus in pediatric patients with posterior fossa tumors. J Neurosurg Pediatr. 2009. 3: 378-85

26. Riva-Cambrin J, Shannon CN, Holubkov R, Whitehead WE, Kulkarni AV, Drake J. Center effect and other factors influencing temporization and shunting of cerebrospinal fluid in preterm infants with intraventricular hemorrhage. J Neurosurg Pediatr. 2012. 9: 473-81

27. Romeo A, Naftel RP, Griessenauer CJ, Reed GT, Martin R, Shannon CN. Long-term change in ventricular size following endoscopic third ventriculostomy for hydrocephalus due to tectal plate gliomas. J Neurosurg Pediatr. 2013. 11: 20-5

28. Sainte-Rose C, Cinalli G, Roux FE, Maixner R, Chumas PD, Mansour M. Management of hydrocephalus in pediatric patients with posterior fossa tumors: The role of endoscopic third ventriculostomy. J Neurosurg. 2001. 95: 791-7

29. Schulz M, Bührer C, Pohl-Schickinger A, Haberl H, Thomale UW. Neuroendoscopic lavage for the treatment of intraventricular hemorrhage and hydrocephalus in neonates. J Neurosurg Pediatr. 2014. 13: 626-35

30. Smith ER, Butler WE, Barker FG. 2nd In-hospital mortality rates after ventriculoperitoneal shunt procedures in the United States 1998 to 2000: Relation to hospital and surgeon volume of care. J Neurosurg. 2004. 100: 90-7

31. Stone JJ, Walker CT, Jacobson M, Phillips V, Silberstein HJ. Revision rate of pediatric ventriculoperitoneal shunts after 15 years. J Neurosurg Pediatr. 2013. 11: 15-9

32. Stone SS, Warf BC. Combined endoscopic third ventriculostomy and choroid plexus cauterization as primary treatment for infant hydrocephalus: A prospective North American series. J Neurosurg Pediatr. 2014. 14: 439-46

33. Torres JL, López BR, Moroño SI, Sanjuán ÁR, Rodríguez AS, Larrazábal LC. Re-Do endoscopic third ventriculostomy. Retrospective analysis of 13 patients. Neurocirugía (Astur Engl Ed). 2022. 33: 111-9

34. Warf BC. Hydrocephalus in Uganda: The predominance of infectious origin and primary management with endoscopic third ventriculostomy. J Neurosurg. 2005. 102: 1-15

35. Warf BC, Tracy S, Mugamba J. Long-term outcome for endoscopic third ventriculostomy alone or in combination with choroid plexus cauterization for congenital aqueductal stenosis in African infants. J Neurosurg Pediatr. 2012. 10: 108-11

36. Wu Y, Green NL, Wrensch MR, Zhao S, Gupta N. Ventriculoperitoneal shunt complications in California: 1990 to 2000. Neurosurgery. 2007. 61: 557-62 discussion 562-3