General Introduction: Why They Exist, Incidence, Social and Economic Costs, and Quality of Life


Hardware-related complications

 Obstruction, raised intracranial pressure

 Breakage

 Malposition

 Migration

 Infection

Fluid dynamic-related complications

 Overdrainage

 Slit ventricles/slit ventricle syndrome

 Subdural collections

Intra-abdominal complications

 Infection, peritonitis, abscess

 CSF pseudocyst

 Visceral complications (bowel obstruction, perforation)



The impact of these complications on affected individuals and societies is tremendous. The current introductory chapter explores the effects of shunt-related complications on patients and healthcare systems. For individuals, shunt-related complications may lead to disability or mortality. Furthermore, a greater impetus has been placed in recent years on understanding the effects of hydrocephalus and procedure-related complications on the quality of life of affected patients. The societal impact of shunt-related complications is also important to consider. We review the relevant literature in the context of both developing and developed countries. We underscore that prevention, early identification, and treatment are necessary to mitigate the personal and social costs of shunt-related complications.



1.2 Heterogeneity in Practice and Complications


In order to appreciate the impact of shunt-related complications on the individual and society, it is important to recognize that considerable heterogeneity exists in surgical decision-making concerning the management of hydrocephalus [43]. There is no consensus on the optimal methods of CSF diversion, much less the specific devices, procedures, and protocols that should be adopted uniformly to decrease the risk of shunt-related complications. In order to mitigate the burden of complications on individuals and societies, rigor must be applied in identifying modifiable patterns that result in subsequent shunt dysfunction. Indeed, this remains the mission of many organizations and consortia worldwide.

In patients who require insertion of a VP shunt, it is known that various non-modifiable factors may predispose to subsequent complications. For example, premature neonates are more likely develop shunt infections than term equivalents [24]. Furthermore, the underlying etiology of the hydrocephalus has been associated with complications, with obstructive hydrocephalus associated with a disproportionately greater incidence of complications than communicating hydrocephalus [35, 45, 52]. The few studies that have examined novel ways to mitigate shunt complications have largely yielded disappointing results; therefore, there is little evidence on the basis of which surgical decision-making can be standardized. For instance, studies have failed to demonstrate superiority of more complex or expensive shunt device over simpler alternatives [39]. Treatment decisions are often at the discretion of the attending surgeon and tailored towards the specific clinical and radiographic phenotypes of individual patients.

There is some evidence, however, to suggest that procedure-related changes may mitigate certain complications [21]. For example, it has been shown that performing shunt insertions at the beginning of the day, limiting personnel in the operating theater, and administration of periprocedural antibiotics result in considerably lower rates of shunt infections [20]. More recently, intrathecal antibiotics, minimizing exposure of shunt tubing to breached surgical gloves [24], and antibiotic-impregnated shunts [13] have been shown or suggested to decrease complications. It remains imperative for clinicians to be cognizant of heterogeneity in practice and to strive to identify best practices to mitigate shunt-related complications.


1.3 Impact on the Individual


Heterogeneity also exists in the underlying pathological conditions leading to shunt-dependent hydrocephalus. They may range from congenital malformations such as aqueductal stenosis and myelomeningocele to acquired conditions such as post-infectious or post-subarachnoid hemorrhage hydrocephalus. Importantly the underlying etiologies of hydrocephalus may be independently associated with poor outcome or poorer health-related quality of life (QOL), and indeed, disentangling the effects of shunt-related complications from other putative factors often proves difficult.

Evaluating the effects of shunt-related complications on an individual’s health is furthermore complicated by the fact that numerous external factors moderate his/her well-being when faced with medical or surgical complications. For example, lower socioeconomic status, worse family functioning, and lower parental education have been shown to correlate with lower quality of life in studies of childhood hydrocephalus [22]. The multifaceted interplay between multiple variables must therefore be considered when evaluating the impact of shunt-related complications.

Concrete data on mortality, disability, and time lost provide less biased estimates of the impact of shunt-related complications on the lives of patients. More robust measures such as QOL indices may, however, provide a more in-depth picture of ways in which shunt complications affect different dimensions of a patient’s well-being. In the subsequent sections, we provide a summary of various indicators used to index the effects of procedure-related complications on a patient’s well-being. Namely, we review data on mortality, disability, and time lost as well as QOL and cognitive function.


1.3.1 Mortality and Hospital Length of Stay


Perhaps the most succinct ways to quantify the impact of shunt complications are to review data on mortality, disability, and length of stay in a hospital. These metrics are important as they represent salient endpoints for outcomes and often inform system-level decision-making regarding the allocation of resources. They are also useful summary statistics to measure the effect of interventions and policies in a longitudinal manner. They are, however, limited in that they do not adequately describe how shunt-related complications affect individuals and do not attempt to explain the cascade of events that lead to poor outcomes. For example, mortality due to shunt-related complications may be a result of delayed diagnosis or delayed transfer to a neurosurgical center. These endpoints also represent a summary of aggregate population-based data; therefore, it is imperative for individual neurosurgeons to record and monitor the rates of complications and adverse events in their own practice in order to strive to improve patient care.

Shunt failure may lead to raised intracranial pressure, herniation syndromes, and sudden death [17]. A large population-based study determined that among all admissions primarily involving a shunt-related procedure, in-patient mortality was 2.7 % [37]. In the same series, the most common diagnosis was shunt malfunction (40.7 %) with 42.8 % of admissions resulting in shunt replacement, suggesting that shunt-related complications are responsible for a considerable proportion of hydrocephalus-associated mortality. Indeed, a 20-year longitudinal study of 138 children with shunted hydrocephalus identified 4 deaths (2.9 %) that were directly attributable to shunt failure [38]. Furthermore, shunt infection has been shown to be significantly associated with a higher likelihood of death following CSF diversion [46]. In the literature, comparable shunt-related mortality rates of 2–5 % have been published on long-term follow-up of shunted patients [3, 16, 17, 18]. Importantly, while some deaths related to shunt malfunction occur suddenly, a considerable proportion of patients also have symptoms for hours or weeks prior to death, highlighting the importance of vigilance and education of patients and front-line healthcare workers [17].

Patients with shunt-related procedures also often have a protracted stay in an in-patient hospital unit. Nearly 50 % of patients admitted for shunt insertions had a length of stay greater than 5 days in hospital [37]. A Spanish study also showed that the mean ICU stay for shunt insertion was 8.2 days [11]. It has been shown that patients admitted with shunt-related complications have a disproportionately longer length of stay in hospital compared to those undergoing primary shunt insertion. For example, patients undergoing shunt revisions due to infection, it has been estimated that the mean duration of hospital stay adjusted for days attributable to CSF infection was 16.3 days [50]. Undoubtedly, prolonged in-patient hospital stays have a profound impact on individuals, as well as societies given the lost productivity as well as the associated healthcare costs.


1.3.2 Quality of Life


In order to adequately discuss, quantify, and ultimately mitigate the impact of shunt-related complications on the individual patient, clinically meaningful and measureable outcomes must be defined beyond standard measures, such as mortality and length of stay in hospital. Quality of life (QOL) is a multidimensional concept that describes the perceived quality of an individual’s daily life, which encompasses their emotional, social, and physical states. Health-related QOL is an evaluation of how an individual’s well-being may be affected over time by a disease, disability, or disorder. As previously described, one difficulty that is encountered in benchmarking the impact of shunt-related complications is that the QOL with hydrocephalus is already diminished, for example, by underlying etiologies of hydrocephalus. When evaluating how shunt-related complications further depress this baseline level, one may encounter a “floor effect,” whereby depressions below the baseline become difficult to measure.

When evaluating an individual’s QOL, it is also important to consider the lens from which this measurement is captured. In some studies, QOL may refer to an assessment of an individual’s abilities by an external rater. For example, a physician may evaluate a patient’s ability to perform a specific task and make inferences about their QOL as a result, or a parent may speak on behalf of a child and describe what they may or may not be capable of doing. Increasingly, the perspective of the patient themselves is considered valuable in evaluating their QOL. The importance of the patient’s perspective is underscored by studies demonstrating the prevalence and morbidity of depression, dependence, substance abuse, unemployment, and inability to drive in this patient population [14].

QOL can be summarized into two separate categories, generic instruments (i.e., SF-36; short form with 36 questions) and disease-specific questionnaires. While the former aims to measure generalizable effects of illness on the individual, the latter focuses on issues that are most important for a given patient population. Using the generic SF-36 instrument, patients with shunted hydrocephalus reported poorer perceived health in 2 of 8 SF-36 domains, physical functioning (covering walking, self-care ability, and strenuous activity), and general health [38]. It is unclear from the literature whether shunt-related complications, such as the number of shunt revisions, are associated with worse perceived QOL on the SF-36.

Kulkarni and colleagues have developed a 51-item questionnaire to specifically quantify the physical, cognitive, and social-emotional health of children with hydrocephalus, the Hydrocephalus Outcome Questionnaire (HOQ) [25, 26]. This instrument has been assessed for validity (i.e., the degree to which it measures what it claims to measure) and reliability (the reproducibility of responses on multiple occasions). The HOQ also shows good correlations with several independent measures of health, including the Strengths and Difficulties Questionnaire [12] and Functional Independence Measure for Children [36]. Interestingly, data from QOL studies using this instrument have demonstrated considerable heterogeneity in the distribution of QOL scores [25]. For instance, 5 % of children with shunted hydrocephalus had QOL scores, which could be interpreted as worse than dead, whereas 20 % were within the normal ranges for the population.

From such disease-specific measures of QOL, which may better capture a patient’s subjective experience with the illness, the impact of shunt-related complications on the individual also becomes more apparent. On multivariate analysis of QOL data from a large cohort of 346 children, increased length of stay in hospital for shunt infection and shunt overdrainage, as well as the number of proximal shunt catheters in situ were significantly associated with worse QOL [25]. Furthermore, more recent studies suggest that more severe shunt infections are associated with poorer QOL than less severe infections [28, 22].


1.3.3 Cognitive Outcome


Disorders involving the central nervous system are unique from those affecting other organs due to the premium placed on the brain, which is the substrate of identity, agency, and cognitive faculties. It is not therefore surprising that cognitive deficits in patients with hydrocephalus significantly impair their QOL [25]. Cognitive function in children with hydrocephalus, for instance, is considerably lower than other dimensions of well-being. Cognition may be impaired to varying extents in upwards of 60 % of affected individuals [44] and may encompass numerous neuropsychological domains, including language, memory, and learning.

The majority of studies evaluating cognitive function in shunted patients have focused on pediatric populations [44]. Indeed, the effects of hydrocephalus on the developing brain are an issue of importance and active scientific inquiry. As many as half of children with hydrocephalus have intelligence quotients (IQ) lower than 70 [16, 29, 32], and an equivalent proportion of infants with hydrocephalus also had severe cognitive impairment [10]. Furthermore, even individuals with IQ scores greater than 70 may possess poor learning, memory, and executive function [33]. While it remains controversial whether shunt complications contribute to cognitive impairment [29], some studies have suggested that central nervous system infections may be associated with worse intelligence outcomes [7]. Further research is, however, required to determine whether a causal relationship exists between cognitive dysfunction and shunt-related complications.

Epilepsy is also an important predictor of poor cognitive outcome, particularly in children with shunted hydrocephalus [2, 32]. The incidence of seizures in shunted patients ranges from 20 to 50 % [40]. The frequency of seizure activity has also been associated with worse QOL in patients with shunted hydrocephalus [28, 22]. It remains unclear whether seizure activity is a marker of worse underlying brain function (and therefore worse outcomes), a result of the etiology of hydrocephalus, or due to the shunt insertion. Data from one study suggest that the position of the ventricular catheter may be associated with the incidence of epilepsy, with patients implanted with frontal catheters more prone to seizures than those with parietal catheters [4]. The number of ventricular catheter revisions may also be associated with increased incidence of seizures [4], although these finding have not been corroborated by other studies.


1.4 Societal Impact


Shunt-related complications are also associated with an enormous social burden. The true impact of shunt complications on society is difficult to glean from the literature. Patients with shunted hydrocephalus are less likely to access higher education and often possess poorer social functioning and employment prospects than their peers [38], resulting in an enormous loss of productivity. It is expected that shunt-related complications exacerbate these challenges by extending time lost from work, extended lengths of hospital stay, and health-related economic costs. The effect of complications on families and communities is also substantial. The extent of the social impact of shunt-related complications is also related to the degree in which they result in impairments for the individual. For example, cognitive impairments, childhood onset, and protracted disease course may be associated with a greater cost to society [15].

There are several ways in which the societal impact of hydrocephalus and shunt-related complications may be measured. One salient measure is the cost of intervention for shunting and revisions. Healthcare costs in general comprise a large proportion of any nation’s budget and have repercussions for societies, particularly with socialized healthcare systems [15]. More importantly, disease and disability associated with hydrocephalus and shunt-related complications exact a high societal price, which may be more difficult to quantify. Metrics such as years of potential life lost (YPLL), valued years of potential life lost (VYPLL), years of potential productivity lost (YPPL), and lifetime years of potential life lost (LYPLL) may be used to attempt to quantify this impact [30]. Measures such as quality-adjusted life years (QALY) also take into account the quality of life affected by the disease burden. These are often used in cost-utility analyses to evaluate the impact of a particular intervention on mitigating the cost of a disease on society. Unfortunately, there is a paucity of studies that have examined how shunt-related complications affect these metrics. Given that health economics is a burgeoning field of research, it is expected that greater emphasis will be placed on understanding the impact of shunt-related complications on these measures of society in the future.

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Jun 22, 2017 | Posted by in NEUROSURGERY | Comments Off on General Introduction: Why They Exist, Incidence, Social and Economic Costs, and Quality of Life

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