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Oday Atallah1, Amr Badary2, Fatma A. Monib3, Yasser F. Almealawy4, Aalaa Saleh5, Francesco Lioi6, Souhaila Fathallah7, Apil Sapkota8, Mrinmoy Kundu9, Vivek Sanker10, Joe M. Das11
  1. Department of Neurosurgery, Hannover Medical School, Hannover, Germany
  2. Department of Neurosurgery, Klinikum Dessau, Dessau-Rosslau, Germany,
  3. House Officer, Faculty of Medicine, Assiut University, Assiut, Egypt,
  4. Department of Medicine, University of Kufa, Najaf, Iraq,
  5. Faculty of Medical Sciences, Lebanese University, Beirut, Lebanon,
  6. Department of Neurosurgery, Sapienza University of Rome, Rome, Italy,
  7. Faculty of Medicine, Mohammed V University, Rabat, Morocco,
  8. Department of Psychiatry, University of Birmingham, Birmingham, United Kingdom,
  9. Department of Neurosurgery, Institute of Medical Sciences and SUM Hospital, Bhubaneswar, Odisha, India
  10. 0Department of Neurosurgery, Trivandrum Medical College, Trivandrum, Kerala, India,
  11. 1Department of Neurosurgery, Imperial College Healthcare National Health Services Trust, Charing Cross Hospital, London, United Kingdom.

Correspondence Address:
Oday Atallah, Department of Neurosurgery, Hannover Medical School, Hannover, Germany.

DOI:10.25259/SNI_215_2024

Copyright: © 2024 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Oday Atallah1, Amr Badary2, Fatma A. Monib3, Yasser F. Almealawy4, Aalaa Saleh5, Francesco Lioi6, Souhaila Fathallah7, Apil Sapkota8, Mrinmoy Kundu9, Vivek Sanker10, Joe M. Das11. Ventriculoperitoneal shunt extrusion in pediatric patients, clinical patterns and therapeutic strategies: A scoping review. 05-Jul-2024;15:226

How to cite this URL: Oday Atallah1, Amr Badary2, Fatma A. Monib3, Yasser F. Almealawy4, Aalaa Saleh5, Francesco Lioi6, Souhaila Fathallah7, Apil Sapkota8, Mrinmoy Kundu9, Vivek Sanker10, Joe M. Das11. Ventriculoperitoneal shunt extrusion in pediatric patients, clinical patterns and therapeutic strategies: A scoping review. 05-Jul-2024;15:226. Available from: https://surgicalneurologyint.com/?post_type=surgicalint_articles&p=12982

Date of Submission
23-Mar-2024

Date of Acceptance
01-May-2024

Date of Web Publication
05-Jul-2024

Abstract

Background: Ventriculoperitoneal shunts (VPSs) are frequently employed in neurosurgery to treat hydrocephalus, with a particular focus on pediatric patients. Although VPSs are commonly utilized, they are not exempt from difficulties, such as shunt extrusion. The main aim of this study is to enhance comprehension regarding the occurrence, causes contributing to, and consequences of VPS extrusion in pediatric patients.

Methods: A comprehensive search approach was implemented, including electronic databases, including PubMed, Google Scholar, and Scopus, to locate pertinent articles published between January 1950 and May 2023. The utilization of keywords such as “ventriculoperitoneal shunt” and “extrusion,” “ventriculoperitoneal shunt” and “migration,” and “ventriculoperitoneal shunt” and “perforation” was employed. Data on patient demographics, underlying diseases, origin of extrusion, presenting symptoms, treatment, and follow-up were gathered. Statistical studies were conducted to identify potential risk factors connected with the occurrence of shunt extrusion.

Results: A study analyzed 80 studies on 120 individuals with extruded VPS catheters. The majority of patients (55.8%) had symptoms such as cerebrospinal fluid leakage and irritation. Hydrocephalus was categorized into congenital (40%), obstructive (36.7%), and communicating (11.7%) groups. Catheter extrusion sites varied, with most from the anal or rectal site. Preoperative meningitis or peritonitis was present in 20% of patients. Treatments ranged from shunt removal to endoscopic third ventriculostomy, resulting in a 90% recovery rate, 1.7% mortality, and 5% follow-up loss.

Conclusion: Extrusion of the distal catheter in VPSs is a critical medical situation that necessitates urgent surgical intervention. The presence of an infection raises the likelihood of complications; hence, it is vital to promptly address the issue through the administration of antibiotics and the replacement of the shunt. Timely intervention enhances results.

Keywords: Child, Children, Extrusion, Hydrocephalus, Neurosurgery, Pediatric, Perforation, Ventriculoperitoneal shunt

INTRODUCTION

Ventriculoperitoneal shunts (VPSs) have evolved into a crucial and widely used neurosurgical intervention for managing hydrocephalus, a neurological disorder characterized by an abnormal cerebrospinal fluid (CSF) accumulation within the cerebral ventricles.[ 41 , 59 , 61 , 100 ] These implantable devices are a lifesaving solution for people suffering from hydrocephalus. They are composed of a proximal catheter that is positioned within the cerebral ventricles and a distal catheter that is routed beneath the patient’s skin, frequently following a subcutaneous pathway down the neck, chest, and abdomen before ending within the peritoneal cavity.[ 5 , 17 , 88 ]

The primary function of these components is to allow excess CSF to drain away from the cranial vault, reducing intracranial pressure (ICP) and alleviating associated neurological symptoms. This intricate system relies on pressure differentials to allow CSF to flow from the cerebral ventricles, through the proximal catheter, along the subcutaneous path, and finally into the peritoneal cavity, where the body’s natural mechanisms reabsorb it. The regulation of CSF flow is further facilitated by a valve mechanism, often located along the distal catheter or within the shunt’s programmable unit, which can be adjusted to optimize CSF diversion based on individual patient needs.[ 17 , 34 ]

Although VPSs have significantly transformed the management of hydrocephalus, it is crucial to acknowledge that intricacies and potential consequences accompany their utilization.[ 14 , 80 ] Shunt component extrusion is a significant complication of shunt elements’ unintentional movement or protrusion through anatomical structures.[ 2 ] This poses considerable clinical difficulty in the management of hydrocephalus.[ 102 ]

This review aims to provide a comprehensive analysis of shunt extrusion, encompassing its occurrence, underlying pathophysiological causes, clinical symptoms, diagnostic techniques, and the range of therapeutic options currently utilized.

MATERIALS AND METHODS

The aims and objectives of the study were carefully considered, and a protocol was written to reflect that. Methods for conducting the search, extracting data, synthesizing data, determining which should be included, evaluating the quality of the studies, and ultimately screening them were all documented in the protocol. This review follows the guidelines established by Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA).

All of the studies reported in the literature in English with participants younger than 18 years were considered for inclusion. Patients older than 18 years old, studies that reported only the migration of the VPS without extrusion, studies that provided an overview, and studies conducted in languages other than English were not considered.

We identified a database of studies on the extrusion of VPSs in the pediatric population through various orifices by conducting a systematic review and searching the relevant literature using PubMed, Google Scholar, and Scopus. The search terms “ventriculoperitoneal shunt” and “extrusion,” “ventriculoperitoneal shunt” and “migration,” and “ventriculoperitoneal shunt” and “perforation” were employed.

To find the articles that met the systematic review’s inclusion criteria, we screened their titles and abstracts and then read them in their entirety. The studies that met the inclusion and exclusion criteria were subjected to a thorough critical review by two reviewers, O.A. and Y.A. We first looked at the titles with keywords, then at the abstracts, and finally at the full texts of those that seemed relevant. At the full-text screening stage, all studies were reviewed by an independent reviewer, A.B., to ensure eligibility. Articles selected or included differently by each of the three reviewers were briefly discussed until a consensus was reached [ Figure 1 ].


Figure 1:

Flow diagram of articles included in the systematic review.

 

The third review resolved any discrepancies that emerged following critical analysis, while the first two reviews independently determined the risk of bias and carried out data extraction using a designed protocol by the Cochrane Handbook.[ 55 ]

Statistical analysis was carried out using Microsoft Excel and the statistical software package Statistical Package for the Social Sciences 26. Categorical data were compared and tested for significance using the Chi-square test. Statistical significance was defined as P < 0.05.

RESULTS

Following a comprehensive screening process, a selection of 80 studies was undertaken, employing rigorous criteria during the initial search across reputable databases, including PubMed, Scopus, and Google Scholar. This extensive study analyzed patient-level data derived from a defined cohort comprising 120 individuals, all grappling with the challenging clinical scenario of the extruded distal end of the VPS catheter. Our age distribution revealed that patients within our study were administered their first shunt at a median age of 3 months, while 75% of participants were below 18 months [ Figure 2 ]. Furthermore, the median interval between the initial shunt implantation and their presentation with shunt extrusion was calculated at 10 months, when 75% of patients presented with the extrusion in <24 months from the first VPS.


Figure 2:

A histogram showing age by the first presentation to ventriculoperitoneal shunt surgery.

 

Gender analysis demonstrated that out of these individuals, 64 were unequivocally identified as male, constituting 53.3% of the total cohort. In contrast, 48 were unequivocally identified as females, accounting for 40% of the cohort, while the genders of the remaining 8 patients (6.7%) were unknown.

Remarkably, the gender of the remaining participants remained enigmatic, and our analysis disclosed no statistically significant gender preferences. It is essential to underscore that a substantial proportion of our cohort, precisely 67 patients, making up a significant 55.8%, presented with a spectrum of symptoms. These symptoms manifested diversely and were likely attributable to localized effects, including the leakage of CSF and irritation of the mucosal lining of the affected organ due to the infected distal catheter. These localized effects often manifest as distressing symptoms such as vomiting, skin erosion, and purulent discharge. Conversely, some of the reported symptoms may have originated from systemic issues linked to VPS dysfunction or infection, resulting in altered mental status, fever, and sepsis. A comprehensive and thoughtful compilation of imaging findings for each individual was organized [ Table 1 ].


Table 1:

Patient characteristics of the related articles.

 

The classification of hydrocephalus represents a pivotal facet of our study, and we categorized it into three principal groups for our statistical analysis: congenital (n = 48, 40%), obstructive (n = 44, 36.7%), and communicating (n = 14, 11.7%), while the type in the rest 14 patients (11.7%) was unknown. This deliberate categorization provides valuable insights into our study’s broad spectrum of conditions.

The origin of catheter extrusion emerged as a significant aspect of our analysis. Our findings strongly suggest that in 44 patients (36.7%) of the cases, the catheter extruded from the anal or rectal site. Conversely, the remaining 17 patients (14.2%) experienced extrusion from the oral site, 14 patients (11.7%) extruded from the abdominal wall, 9 patients (7.5%) from the vagina, 8 patients (6.7%) from the urethra, 7 patients (5.8%) from the umbilicus, and 6 patients (5%) from an inguinal site. The remaining 12 patients (12.5%) exhibited extrusion from random sites [ Figure 3 ].


Figure 3:

The distribution of the extrusion sites.

 

An important variable in our study is the presence of preoperative meningitis or peritonitis in only 24 patients (20%). Our analysis found no statistically significant difference in peritonitis or meningitis presentation between patients presenting across the three primary extrusion sites (oral or rectal, abdominal wall, and urethra), with P = 0.743.

Irrespective of the presenting symptoms, all patients underwent invasive management procedures. The spectrum of treatments employed was diverse and included various approaches: 62 patients (51.7%) underwent the removal of the VPS with subsequent replacement during the same hospital admission; 32 patients (26.7%) had the VPS removed exclusively; 11 patients (9.2%) underwent distal catheter revision alone; 4 patients (3.3%) underwent VPS removal followed by endoscopic third ventriculostomy (ETV); 3 patients (2.5%) underwent external ventricular drainage (EVD) without mentioning the further management; and 3 patients (2.5%) had the VPS removed and replaced with a ventriculoatrial (VA) shunt. The remaining 3 patients (2.5%) underwent alternative treatment management strategies. In addition, there was one patient for whom the therapy remained enigmatic [ Figure 4 ]. After the management, as mentioned above, a noteworthy 108 patients (90%) made a complete recovery, while 2 patients (1.7%) regrettably succumbed to their conditions, and 6 patients (5%) were lost to follow-up.


Figure 4:

The treatment modalities.

 

DISCUSSION

The distal catheter of the VPS typically resides serenely within the peritoneal cavity, navigating its course amidst the labyrinthine folds of the intestines without causing any disruption. However, under certain predisposing conditions, it embarks on a transformative odyssey, migrating to atypical locations where it adheres and initiates an inflammatory cascade. Abdominal wall contractions can exert force on the catheter, propelling it into the encircling fibrous tract. This phenomenon may follow episodes of elevated intra-abdominal pressure or may result from anchoring to a calcified point along the catheter’s path, thereby prompting its migration toward subcutaneous tissues.[ 29 , 42 , 71 , 92 ] By adherence of the shunt distal end to the viscera or body wall, an inflammatory response will be provoked with the release of the cytokine interleukin-13,[ 57 ] and chemotaxis of Th1 cells, CD4+ cells, macrophages, and dendritic cells leading to cytolysis and necrosis of the organ wall with gradual extrusion of the catheter.[ 3 ] A firm type of catheter may provoke migration[ 61 ] because using a softer catheter reduces the risk rate of migration.[ 61 ] Poor immunity, wound dehiscence, and insufficient surgical technique can lead to extrusion[ 25 , 32 ] [ Figure 5 ].


Figure 5:

Overview of the ventriculoperitoneal shunt extrusion. IL-13: Interleukin 13, TGF: Transforming Growth Factor, CTGF: Connective Tissue Growth Factor, ECM: Extracellular Matrix, MMP: Matrix Metalloproteinease, ICP: Intracranial Pressure, ETV: External Third Ventriculostomy, VP Shunt: Ventriculoperitoneal shunt

 

This journey through uncharted territories triggers intricate foreign body tissue responses – inflammatory cells, collagen deposition, and fibrous tissue encapsulation – that are intricately linked to the risk of catheter extrusion, ultimately culminating in one of the most prevalent complications – obstruction, a frequent impetus for revision surgeries.[ 3 , 60 ] The formation of granulation tissue and fibrotic encapsulation catalyzes the migration and then extrusion of the peritoneal terminus of the VPS, contributing substantively to the constellation of shunt-related complications and constituting approximately 10.81% of the total shunt complications encountered in clinical practice.[ 81 ] We posit that this process of distal catheter migration represents the initial phase in the intricate narrative of catheter extrusion. Importantly, this pathophysiological sequence aligns harmoniously with extant findings in the scientific literature, some of which have been substantiated through investigative studies employing canine models.[ 21 ]

When delving into the realm of other potential predisposing factors, infection emerges as a prominent contender when localized around the shunt components. This infectious component holds the potential to exert a considerable influence on the likelihood of shunt extrusion. This revelation harmonizes seamlessly with the prevailing body of neurosurgical literature, which underscores the pivotal role of infection in the landscape of shunt-related complications.[ 34 , 66 , 95 ] Shunt infection increases the risk of extrusion due to the formation of biofilms on catheter surfaces.[ 22 ] Biofilms can serve as a nidus for chronic inflammation and tissue reactions, ultimately causing more weakness in tissue integrity and increasing the likelihood of catheter migration.[ 22 ]

The clinical presentation of distal catheter extrusion in a VPS can vary depending on the extent of catheter displacement and associated complications. Common clinical signs and symptoms associated with distal catheter extrusion may include visible protrusion of the catheter from the abdomen, usually at the site where it was initially placed;[ 5 , 39 ] patients may experience localized pain or discomfort at the extrusion site or redness and swelling in the area around the extruded catheter.[ 9 ] There may be drainage of CSF or clear fluid from the extrusion site, which can indicate shunt malfunction.[ 39 ]

The extrusion site can become a potential entry point for infection, leading to symptoms such as fever, increased pain, or redness.[ 39 , 103 ] Depending on the extent of CSF diversion disruption, patients may experience symptoms related to increased ICP or shunt malfunction, such as headaches, nausea, vomiting, or changes in mental status.[ 38 , 39 ]

When it comes to the management of extrusion of the distal catheter from a VPS through the abdominal wall, the prompt administration of prophylactic antibiotics is essential, followed by complete replacement of the entire shunt system.[ 25 , 32 ] Shunt revision in the first 6 months after implantation constitutes around 24% of the total shunt complications.[ 81 ] Pulling the extruded distal end distally avoids the potential spread of infection; cases of inexistent peritonitis, laparotomy, and peritoneal lavage are unneeded.[ 64 ] In the event of peritoneal inflammation or catheter attachment to peritoneal contents, the necessity arises for a laparotomy. Once the existing shunt system has been completely removed, reinserting a fresh shunt system is dependent on the CSF analysis results. When they are negative, it may be contemplated within the same surgical session; however, in scenarios where an infection is suspected, the standard procedure entails installing an EVD system accompanied by initiating intravenous antibiotic treatment.[ 82 , 94 ] Following this, sequential CSF sampling is carried out until two consecutive negative culture results are obtained. At this juncture, the option to reintroduce a new shunt system becomes a feasible consideration.

Some studies investigated the outcomes of pediatric patients who underwent VPS revision for various complications, including extrusion, and found that early intervention and meticulous surgical techniques were associated with improved outcomes and reduced recurrence rates.[ 1 , 81 ]

In recurring infections and complications associated with VPSs, a potential solution may involve transitioning to a VA shunt. This approach, as reported in some studies, has been linked to enhanced clinical outcomes and a reduction in ventricular size,[ 108 ] which was done in three cases in our review with two recoveries and one death.[ 45 ] ETV – with postoperative EVD as a detector for ETV failure – is an alternative intervention for patients experiencing shunt malfunction, revealing that 68.4% of cases achieved shunt independence,[ 69 ] which is done in four cases in our review with a 100% recovery rate. The statistically quantifiable success rate supports VPS removal and replacement efficacy with a new VPS. Alternative drainage replacement approaches, such as ETV and VA shunt, should be reserved.

CONCLUSION

Distal catheter extrusion in VPSs involves the unintended protrusion of the catheter from its intended exit site, causing clinical symptoms such as visible protrusion, localized pain, redness, swelling, CSF drainage, and signs of infection. Prompt management is crucial, typically involving prophylactic antibiotics and complete shunt system replacement, with the timing of reinsertion determined by CSF analysis results, indicating feasibility for immediate reinsertion.

Extrusion sites vary, including the anal or rectal area, abdominal wall, urethra, vagina, umbilicus, and inguinal region, each presenting unique management challenges. Distal catheter extrusion is a medical emergency, necessitating immediate medical attention, surgical intervention, and shunt revision to prevent complications such as infection or intracranial hypertension. Alternative drainage options, such as VASs and ETV with postoperative EVD, may be considered based on the clinical scenario and patient characteristics. Managing distal catheter extrusion has diverse outcomes, with early intervention, meticulous surgical techniques, and appropriate strategies associated with improved outcomes and reduced recurrence rates. Continued research is essential to refine treatment approaches for the varied distribution of extrusion sites in VPSs.

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.

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