Abstract
Introduction
Over the past two decades the number of pediatric cerebrospinal shunts placed annually has nearly doubled.1,2 The increasing utilization of pediatric shunts correlates with the growing number of children surviving prematurity and other congenital conditions. A substantial number of those children will experience a shunt malfunction in infancy or early childhood; cerebrospinal shunts in children have failure rates of 30–40% at one year and 50% at two years.3,4 Patients under 6 months old 5 and those with intraventricular hemorrhage 6 are at even higher risk. Detecting shunt malfunction in a timely manner is essential because untreated shunt failure can result in brain injury and mortality rates of 2% or higher7–9 in a population of children already at high risk for neurodevelopmental impairment and comorbidities.
Infants and young children cannot reliably self-report symptoms of shunt malfunctions, leaving home caregivers with full responsibility for detecting possible shunt failure. Additionally, early childhood is characterized by constant developmental changes, evolving baseline behaviors related to sleep, eating, and activity, and frequent minor infections, illnesses and injuries. Despite the unique challenges of detecting shunt malfunction in this context, and despite the substantial number of shunt malfunctions that occur during early childhood, data focused on this population are limited to studies performed two decades ago. Those decades have coincided with expanding surgical approaches to pediatric hydrocephalus and a greater diversity of underlying medical complexity among infants and young children receiving shunts.10–12 It is important to assess whether the presentation of shunt malfunction in infants and young children is changing in this era.
In 2021, we published results from a small national cohort of caregivers whose child was previously diagnosed with neonatal hydrocephalus. Multiple caregivers reported that their child's symptoms of shunt malfunction differed from what clinicians had told them to expect; this finding was most pronounced among caregivers whose child was an infant or young child. 13 Any potential mismatch between clinician counseling and a child's presentation may reflect the reality that few studies have focused solely on malfunction signs and symptoms in infants and young children. The Pediatric Shunt Design Trial is among the most contemporary and complete descriptions of shunt malfunction in early childhood. 14 In this 2001 cohort of 431 young patients, factors like decreased consciousness and wound erythema occurred rarely but were always indicative of shunt failure. More common factors like irritability, nausea, vomiting, and bulging fontanelle were predictive of shunt malfunction soon after shunt insertion (median time 4.6 months) but became less predictive 9 or more months after insertion.
Most studies of signs and symptoms of shunt malfunction include children from infancy to young adulthood, making it difficult to tailor counseling for families of the youngest patients. Importantly, there is a lack of detailed data from families, even though families are the first line adjudicators of whether a child is experiencing a sign or symptom of concern.
Here we aimed to characterize the signs and symptoms of shunt malfunction in children ≤5 years old and the caregiver experience of identifying and seeking care for shunt malfunction. We report results of a national survey of caregivers of children ≤5 years old with shunts. This contemporary population of infants and young children contributes important information about the initial signs and symptoms of shunt failure, to reinforce clinician counseling and family education. Family education is a key aspect of improving child outcomes following cerebrospinal shunt placement, as improved family education is associated with reduced mortality related to shunt malfunction.8,9,15
Methods
English-speaking caregivers of children ≤5 years of age were recruited in collaboration with the Hydrocephalus Association. An anonymous cross-sectional survey was offered over a predetermined 6 week timeline from 9/23/21-11/1/21 over various Hydrocephalus Association platforms including the website, via email listservs, and public social media platforms (Facebook, Instagram, Twitter). The survey was created by content experts in pediatric neurology, neonatology, and pediatric palliative care. The survey was pilot tested by board members from the Hydrocephalus Association, including a parent member, and then edited accordingly based on feedback before finalizing. Survey data was collected and managed using REDCap electronic data capture tools16,17 hosted by the Children's Hospital of the King's Daughters.
Survey questions included a comprehensive list of shunt malfunction signs or symptoms that was created to be inclusive of the symptoms noted by Garton 14 and Ackerman 15 ; caregivers were asked to indicate which of the signs or symptoms their child exhibited during their first, and when applicable, subsequent shunt malfunctions. In addition, caregivers were asked targeted questions regarding sources of education about shunt malfunctions and what actions they took when they suspected a shunt malfunction (See supplemental material).
All statistical tests were performed using SPSS.26 (Chicago, IL). 18 Continuous variables are presented as mean, standard deviation, median, 25th and 75th percentiles. Categorical variables are presented as frequency and percentage. The Kruskal-Wallis test was used to compare continuous variables. Chi-square test was used to compare categorical variables. All statistical tests were two-sided and p < 0.05 was considered statistically significant.
The Eastern Virginia Medical School (IRB #21-07-EX-0175) approved the study; caregivers gave consent on REDCap before beginning the survey.
Results
Participant Characteristics
A total of 240 caregivers responded to the survey; 12 records were incomplete or truncated due to exclusion criteria (eg current age of child). 228 complete caregiver responses were analyzed, representing 228 children. Table 1 describes the characteristics of caregivers: most were white females, were the mother of the child with the shunt, were employed full time and had at least some college education.
Caregiver Characteristics.
Table 2 describes the characteristics of the children. Most received their shunt when they were <6 months old, and almost half had a confirmed shunt malfunction before 6 months of age.
Child Characteristics.
Presenting for Shunt Evaluation
When worried that their child might have signs or symptoms consistent with a shunt malfunction, just under half (45%) of caregivers first contacted their neurosurgeon's on-call line. They were as likely to call family or friends for advice as to contact their pediatrician (18% vs 20%, respectively). Over half of families (55%) took their child to the emergency department for evaluation, while 27% went to their neurosurgery clinic.
Table 3 describes the frequency of caregiver-reported signs and/or symptoms during any shunt malfunction up through five years of age. The signs and/or symptoms of vomiting (23.1%), irritability (20.8%), and sleepiness (17.2%) were most common. Most children presented with ≥ 2 signs/symptoms, but 15% of shunt malfunctions reportedly presented with a single sign or symptom. A “full fontanelle” and leaking shunt were the most commonly reported isolated signs/symptoms.
Symptoms of Shunt Malfunction in Children ≤5 Years Old.
* = 7 respondents did not check any symptoms.
For those caregivers whose children had at least 2 shunt malfunctions, we asked them to report their child's symptoms in the first versus later malfunctions (Table 4). The signs and/or symptoms of vomiting (47.4%), irritability (47.4%), sleepiness (45.2%), poor eating and/or drinking (32.5%), and full fontanelle (30.3%) were reported in at least one third of initial shunt malfunctions. Those same signs and/or symptoms, in addition to nausea (34.3%), decreased consciousness (33.0%), and change in eye movements (32.4%) were reported for at least one third of subsequent malfunctions. Most (86%) caregivers reported that their child's first and subsequent malfunctions had at least one sign or symptom in common, with vomiting (47.2%), irritability (42.6%), and sleepiness (35.2%) most likely to occur during >1 malfunction.
Frequency of Symptoms in First Malfunction, Later Malfunctions, and “False Alarms”.
A minority (15%) of caregivers reported that their child had “other” symptoms at the time of their shunt malfunction. The most common “other’ symptoms were behavior change (ie “not acting himself”), altered skin color, low heart rate, a distended scalp vein, and chronic symptoms that ebbed and flowed for weeks to months. For 5 caregivers, the “other” symptoms described overlap with those on the list (eg “crying” vs “irritability,” “leaking incision sites” vs “leaking shunt”).
The majority (75%) of caregivers had experienced at least one “false alarm,” worrying their child had a shunt malfunction when there was not one. Table 4 also shows the frequency of symptoms during “false alarms.” The same three symptoms that were most common during confirmed shunt malfunctions were also most common in false alarms: vomiting (45.6%), irritability (46.5%), and sleepiness (37.7%). Compared with actual shunt malfunctions, irritability (OR = 1.39, 95% CI [1.05, 1.85], p = 0.022) and fever (OR = 2.22, 95% CI [1.44, 3.44], p < 0.001) were more likely to be a false alarm. In this survey there were no caregiver-reported symptoms that were statistically more likely to be associated with true malfunction as opposed to a “false alarm.”
Parent Preparation to Detect Shunt Malfunction
Most caregivers reported that they received counseling and education about how to detect a shunt malfunction in multiple locations within their health system: a neonatal or pediatric intensive care unit (ICU) (68%), an outpatient clinic (65%), a general pediatric ward (46%), and an emergency department (42%). The vast majority (95%) reported this counseling/education from a neurosurgeon. Under half learned about shunt malfunction from an ICU clinician (44%), pediatrician (30%), or neurologist (22%). Notably, one third of respondents reported learning from other parents, and over two-thirds reported finding their own resources for education about shunt malfunctions.
When asked, “How many of your child's symptoms had health care providers told you to expect?” slightly over half (55%) of caregivers said “most” while a minority responded “few” (12%) or “none” (8%). Caregivers who were counseled about “most” of the signs and symptoms were significantly more confident in their ability to detect a shunt malfunction than were those who were informed about “some” symptoms (p = 0.036).
Approximately half of caregivers reported that the difficulty of detecting shunt malformations remained the same over time (52%), while under one-third (30%) reported that their child's shunt malfunctions became easier to detect over time (Table 5). Notably, detecting shunt malfunctions became harder for a number of caregivers once their child's anterior fontanelle closed.
Why Detecting Shunt Malfunctions Became Easier Versus Harder*.
* = selected caregiver quotes
The majority (85%) of caregivers reported it was either “very” or “somewhat” difficult to differentiate symptoms of shunt malfunction from their child's regular development.
Discussion
Vomiting, irritability, and sleeping more than usual were the most common symptoms of initial and subsequent shunt malfunction for children ≤ 5 years old in our cohort. These same signs were also the most prevalent in subsequent malfunctions and were the most common triggers of evaluations that revealed no shunt malfunction (“false alarms”). In the most recent comparable cohort of young children with shunts, published by Garton et al in 2001, 14 increased head circumference (18%), bulging fontanelle (14.6%), irritability (13%) and nausea/vomiting (12%) were noted as the most common signs of shunt failure. Data from older children suggest that shunt malfunction after early childhood is more likely to present with lethargy or shunt site swelling. 7 It is notable in our cohort that 15% of shunt malfunctions reportedly presented with “other” symptoms; “not acting him/herself” was the most common “other” sign. In addition, 14% of shunt malfunctions among infants and young children in our cohort were reported to present with a single sign or symptom; no note is made in the Garton study of how often symptoms were clustered versus isolated for individual patients.
Parent-reported signs and symptoms in this cohort of young children are different from those reported by Garton et al and are different than those reported for older children. Notably, the most common signs or symptoms completely overlapped with the most common triggers of false alarms. Vomiting, irritability, and sleeping changes are common in all infants and young children, including those without shunts, related to typical developmental changes and illnesses of early childhood. That non-specific symptoms are unreliable in distinguishing pediatric shunt malfunction from false alarms was recently highlighted by Razmara et al 19 and our data suggest that this challenge may be particularly problematic for the youngest patients.
Taken together, these data underscore that symptom tracking alone is not adequate to detect shunt malfunction in infants and young children. Expanded access to objective measures of intracranial pressure and potential malfunctions is critical. In addition to decreasing radiation exposure, MRI is superior to CT at detecting shunt malfunction.20,21 Recent data demonstrates that despite a nearly 20% increase in emergency department visits between 2006–2017 among children with shunts, overall fewer children received surgical interventions because of access to MRIs. 22 Caregivers can be encouraged to bring their children to attention with concerning symptoms, as evaluation will result in neither excess radiation exposure nor unnecessary interventions. Additionally, rapid MRI is increasingly available 23 though associated with a longer time to imaging (53 min) and a longer emergency department length of stay (52 min). 24 Machine learning also shows potential to combine clinical, radiologic, surgical, and shunt-design factors to improve detection of shunt malfunction. 25
Continuing to evolve objective tools is essential, but we also must help “first responder” caregivers prepare for their essential role in bringing children to medical attention for neuroimaging and for detailed medical exams. In a recent large study of >9000 children of all ages who received a shunt revision, peritonitis, papilledema and oculomotor palsies were the most accurate indicators of shunt malfunction. 19 The utility of these signs is somewhat limited by the fact that they can only be detected by medical professionals, once a caregiver has raised a concern.
Our national sample confirms that neurosurgeons are doing the bulk of family counseling and preparation for shunt malfunctions. The caregivers that received the most counseling felt the most prepared. However, the majority (85%) of this well-educated cohort still found it difficult to distinguish young childhood development or minor illnesses from a shunt malfunction. A minority felt that detecting malfunctions got easier over time; many felt it got more difficult as the fontanelle closed. Other studies conflict about whether parent accuracy in detecting shunt malfunctions increases 26 or decreases 27 as parents gain more experience. Not quite half of the caregivers in our study contacted their neurosurgeon on-call line when worried, the remainder were as likely to call a family or friend as to call their neurologist. This suggests ongoing opportunities to strengthen family preparation for when to worry about shunt malfunction, and what to do when they are worried. Neonatal neurocritical care programs, which focus on the provision of brain-focused care and partnership with neurosurgical teams, may be uniquely suited to meet this need if infants receive their first shunt in the hospital.28–30
Approximately half of caregivers reported that their infant or young child had signs and/or symptoms that they had not been counseled about previously. We cannot confirm whether these caregivers did receive this information; however, information delivery was nonetheless not salient enough to meaningfully guide caregiver decision-making. These results suggest an opportunity for educational methods to complement clinician counseling. The Agency for Healthcare Research and Quality (AHRQ) advocates the teach-back method to bridge differing levels of health literacy 31 ; with this method, the clinician delivers information to a caregiver and asks them to “teach back” what they heard, using their own words. This strategy might overcome the challenge of caregivers not understanding that their child's sign and/or symptom (eg “crying”) is the same one their clinician counseled them about (eg “irritability”).
Caregivers frequently interacted with other healthcare providers (ICU clinicians, neurologists, pediatricians), but report they rarely received counseling about shunt malfunctions from these providers. This may represent a missed opportunity for enhanced involvement of neurologists, who often see patients with hydrocephalus alongside neurosurgeons. It may be that other clinician types, like primary care clinicians, would benefit from continuing medical education about cerebrospinal shunts and the signs and/or symptoms of malfunction, so that they can reinforce caregiver counseling. Many medical certification boards (eg American Academy of Pediatrics 32 ), for example, are now requiring online learning; a module about shunts/shunt malfunctions delivered in this setting could reach a national group of physicians. Caregivers frequent multiple sites within healthcare systems, from inpatient units to outpatient clinics to emergency departments. Distributing educational materials in these locations may be useful for both clinicians and caregivers, eg via quick response (QR) codes linked to computerized content, a strategy with growing evidence for effectiveness in health education. 33
There are also opportunities to leverage the finding that caregivers commonly reached out to other families (33%) and non-clinical resources (69%) to learn about shunt malfunction. Multiple studies confirm that families of children with medical complexity connect with each other and educate each other via social media. When this peer mentorship occurs via non-monitored platforms (eg Facebook34,35), it is difficult to assess information quality. Caregivers should be provided with options for high-quality resources. The Hydrocephalus Association is a non-profit organization with several family supports, including information about symptoms of shunt malfunction, 36 a helpline caregivers can call for resources, and a peer support network 37 that pairs parents with trained parent volunteers to share experiences and supports relevant to pediatric hydrocephalus. High-quality information sources are especially important for caregivers whose infants or young children are having their first malfunctions. Figure 1 summarizes these opportunities to reinforce education about shunt malfunction for caregivers and clinicians.

Education Opportunities for Shunt Malfunction “First Responders”: Caregivers and Healthcare Providers.
We recognize several limitations to this study. The data presented here about the signs and symptoms of shunt malfunction come from caregiver report and can certainly have limitations, although Watkins et al noted that caregivers commonly have greater accuracy in this context than practitioners or hospitals. 38 The children were all ≤5 years old but at different ages at the time of the study, so not all contribute 5 years of data. The finding that the signs and/or symptoms of initial shunt malfunctions differed somewhat from subsequent ones suggests room for studies with the power to isolate the signs and/or symptoms of these distinct groups. The caregivers were mostly well-educated, white females. Potential disparities in shunt care, 4 as in other areas of medicine, demand broader research with heterogeneous populations.
Conclusions
Vomiting, irritability, and/or sleeping more than usual were the presenting symptoms of shunt malfunction for children ≤5 years old in this national cohort. These same symptoms were just as likely to be false alarms. Despite advances in objective measures of shunt malfunction, caregivers at home remain the “first responders” for determining when there is a concern. Nearly all caregivers struggled to distinguish young childhood development or minor illnesses from a shunt malfunction and only a minority felt that detecting malfunctions got easier over time. Future interventions should leverage these opportunities to reinforce education of families of young children with shunts.
Supplemental Material
sj-doc-1-cno-10.1177_2329048X231153513 - Supplemental material for Symptoms of Cerebrospinal Shunt Malfunction in Young Children: A National Caregiver Survey
Supplemental material, sj-doc-1-cno-10.1177_2329048X231153513 for Symptoms of Cerebrospinal Shunt Malfunction in Young Children: A National Caregiver Survey by Rebecca A. Dorner, Monica E. Lemmon, Turaj Vazifedan, Erin Johnson and Renee D. Boss in Child Neurology Open
Footnotes
Acknowledgements
We are very grateful to the parents who shared their time and experiences as a part of study participation. We also want to thank the Hydrocephalus Association for their help in distributing our study recruitment materials.
Declaration of Conflicting Interests
The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr Dorner’s work was supported by the Children’s Hospital of the King’s Daughters’ Department of Pediatrics Chairman’s Grant. The authors have no conflicts of interest. Dr Lemmon receives salary support from the National Institute for Neurological Disorders and Stroke (K23NS116453).
Ethics Approval
Ethical approval to report this study was obtained from The Eastern Virginia Medical School (IRB #21-07-EX-0175).
Funding
The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This work was supported by the Children’s Hospital of the King’s Daughters’ Department of Pediatrics Chairman’s Grant.
Informed Consent
Written informed consent on REDCap was obtained from caregivers for their anonymized information to be published in this article.
Supplemental Material
Supplemental material for this article is available online.
References
Supplementary Material
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