ArticlePDF Available

An Instrument to Measure the Health Status of Children with Hydrocephalus: The Hydrocephalus Outcome Questionnaire

Authors:
Abhaya V Kulkarni
Abhaya V Kulkarni
Abhaya V Kulkarni
  • This person is not on ResearchGate, or hasn't claimed this research yet.
Download full-text PDF
Read full-text
Download citation
Available via license: CC BY 4.0
Content may be subject to copyright.
BioMed Central
Page 1 of 1
(page number not for citation purposes)
Cerebrospinal Fluid Research
Open Access
Oral presentation
An Instrument to Measure the Health Status of Children with
Hydrocephalus: The Hydrocephalus Outcome Questionnaire
Abhaya V Kulkarni*
Address: Division of Neurosurgery, Hospital for Sick Children 555 University Avenue Toronto, Ontario CANADA M5G 1X8
Email: Abhaya V Kulkarni* - abhaya.kulkarni@sickkids.ca
* Corresponding author
Background
The measurement of clinical outcome in pediatric hydro-
cephalus frequently ignores the substantial effect that the
condition can have on a child's physical, emotional, cog-
nitive and social health. Therefore, our group developed a
quantitative health status measure, called the Hydroceph-
alus Outcome Questionnaire (HOQ), designed specifi-
cally for children with hydrocephalus. This was designed
as a simple questionnaire to be completed by the child or
child's parents.
Materials and methods
The standardized steps in the development of a health sta-
tus measure were followed. Item generation involved
health professionals and focus groups with parents of
children with hydrocephalus. This created a comprehen-
sive list of 165 unique health status items. To reduce this
list, questionnaires were sent to 69 sets of parents to assess
what they felt were the most important of these health
issues. The 51 most important items were then selected to
represent the following health domains: Physical, Social-
Emotional and Cognitive Health. The 51-item question-
naire was then tested for reliability and construct validity,
in another cohort of 90 sets of parents, against the follow-
ing independent measures of specific components of
health: Health Utilities Index (HUI-2), Wide Range
Achievement Reading Test (WRAT), Strengths and Diffi-
culties Questionnaires (SDQ), Functional Independence
Measure for Children (WeeFIM)
Results
The HOQ took approximately 10–15 minutes for the par-
ents to complete and demonstrated excellent test-retest
reliability (0.93, 95% confidence interval (CI) 0.88–
0.96), inter-rater reliability (0.88, 95% CI 0.79–0.93), and
internal consistency (Cronbach's alpha 0.94). Construct
validity was demonstrated by very good Pearson correla-
tions of domain scores with their respective independent
measures. The child-completed version of the HOQ also
demonstrated very good reliability properties.
Conclusion
The HOQ for children with hydrocephalus has been
developed and has demonstrated excellent reliability and
validity properties. This will provide a valuable measure-
ment tool for a wide range of clinical research projects in
pediatric hydrocephalus. Using the HOQ, our group has
begun a large-scale study to explore the determinants of
health outcome in this population of children.
from 49th Annual Meeting of the Society for Research into Hydrocephalus and Spina Bifida
Barcelona, Spain, 29 June – 2 July 2005
Published: 30 December 2005
Cerebrospinal Fluid Research 2005, 2(Suppl 1):S42 doi:10.1186/1743-8454-2-S1-S42
<supplement> <title> <p>49th Ann ual Meeting of the S ociety for Research in to Hydrocephalus and Spina Bifida</p> </ti tle> <note>Meeting abstracts – A single PDF containing all abstracts in this Supplement is available <a href="http://www.biomedcentral.com/con-tent/files/p df/1743-8454-2-S1-full.p df">here</a>.</note> </supplement>
Quality of life and functional vision in adolescents with surgically treated hydrocephalus in infancy
Article
  • Jun 2024
  • ACTA PAEDIATR
Aim To evaluate health‐ and vision‐related quality of life (HR‐ and VR‐QoL) and perceptual visual dysfunction (PVD) in adolescents with hydrocephalus surgically treated in infancy. Method s In total, 23 adolescents (15 males and 8 females; median age 14.9 years) with hydrocephalus and 31 controls were evaluated using validated instruments to measure HR‐QoL and VR‐QoL. PVDs were reported by history taking in five areas: recognition, orientation, depth, movement and simultaneous perception. Results Adolescents with hydrocephalus and the parent proxy reports showed lower mean total Paediatric Quality of Life Inventory 4.0 scores (75.8 and 63.7, respectively) compared with controls (87.6 and 91.5), p = 0.016 and p < 0.0001. Parent‐reported scores were lower than self‐reported scores ( p = 0.001). Adolescents with myelomeningocele ( n = 10) showed lower physical health scores ( p = 0.001). No VR‐QoL difference was found between groups. PVDs were reported in ≥1 area by 14/23 hydrocephalus participants and 2/31 controls ( p < 0.0001). Associations were found in the hydrocephalus group between VR‐QoL and HR‐QoL ( r s = 0.47, p = 0.026) and number of PVD areas ( r s = −0.6, p = 0.003). Conclusion Adolescents with hydrocephalus and their parents reported lower HR‐QoL and more PVDs. These problems indicate the need for not only ophthalmological follow‐ups but also evaluation of QoL and PVDs in individuals with infantile hydrocephalus.
View
Natural History, Treatment Outcomes and Quality of Life in Idiopathic Normal Pressure Hydrocephalus (iNPH)
Article
  • Nov 2021
Background: The natural history and treatment outcomes in adult patients with hydrocephalus is a broad and heterogeneous topic that encompasses the natural history of the various subtypes of adult hydrocephalus with or without treatment; their surgical operative results, including symptom improvements, treatment failure, short- and long-term complications, and reoperations; and morbidity, mortality, and patient-centered health-related quality of life (HRQoL). Objective, methods, and materials: The objective of this review is to present a current update on the natural history and treatment outcomes, including QoL, for adults with hydrocephalus with a focus on patients with idiopathic normal pressure hydrocephalus (iNPH). A nonsystematic review of relevant literature was summarized. Results and conclusions: The natural history for untreated patients with iNPH is poor, with both increased mortality and morbidity. It is strongly recommended that practitioners follow established guidelines to select patients with suspected iNPH while using objective measures of gait, balance, and cognition for consideration of treatment with a CSF shunt. Other factors such as patient-related medical comorbidities or frailty may need to be factored into the decision-making process before surgical treatment is offered. As a rule, failure to select patients based on the identified guidelines will result in a significantly lower positive response to treatment with a CSF shunt. Over 90% of iNPH patients who undergo CSF-shunt treatment demonstrate symptomatic relief after surgery, and long-term studies have shown that in most patients, the clinical improvements are long-lasting, with over 70% demonstrating improvement longer than 6 years after treatment. There is no evidence to support the routine use of endoscopic third ventriculostomy (ETV) to treat patients with iNPH. There is limited data regarding HRQoL in patients with iNPH. In addition to objective measures of outcomes focused on gait and cognition, it is equally important for future studies to assess patient-centered subjective measures of HRQoL.
View
The Impact of Hydrocephalus Shunt Devices on Quality of Life
Article
  • Apr 2021
Background: Despite advances in hydrocephalus shunt technology and improvement in hydrocephalus management, many patients have chronic disability and require multiple surgeries throughout their lifetime. There is limited data from patients' perspective regarding the impact of shunt devices on quality-of-life. Methods: A cross-sectional survey was developed to evaluate the impact of shunt devices on patient quality-of-life. The survey was distributed via social media platforms of the Hydrocephalus Association, and patients self-selected to anonymously complete the online questionnaire. A literature review was performed to contextualize the findings from the survey. Results: A total of 562 survey responses were obtained from a network encompassing 35,000 members. The mean age was 30 years old (0.5-87), and 65% identified as female. Eighty one percent underwent at least 1 shunt revision surgery, with a reported average of 10 shunt revision surgeries per patient (1-200 surgeries). Occlusion, shunt migration and infection were the leading causes for revision at 60%, 47%, and 35%, respectively. In addition, 72% of patients reported pain and discomfort from the device, and 68% expressed avoidance of certain activities due to "fear of bumping shunt." Despite numerous articles discussing shunt technology, a review of the literature indicated a paucity of studies specifically evaluating the burden of shunt devices from a patient/caregiver perspective. Conclusions: The findings from this study suggest long-term physical and psychosocial burden associated with shunt devices. Importantly, this study highlights the need for concerted efforts to develop validated tools to study patient reported outcomes as it relates to neurocranial implanted devices.
View
MR Elastography Demonstrates Reduced White Matter Shear Stiffness in Early-Onset Hydrocephalus
Article
Full-text available
  • Feb 2021
Introduction Hydrocephalus that develops early in life is often accompanied by developmental delays, headaches and other neurological deficits, which may be associated with changes in brain shear stiffness. However, noninvasive approaches to measuring compliance are limited. Magnetic Resonance Elastography (MRE) of the brain is a relatively new noninvasive imaging method that provides quantitative measures of brain tissue stiffness. Herein, we aimed to use MRE to assess brain stiffness in hydrocephalus patients compared to healthy controls, and to assess its associations with ventricular size, as well as demographic, shunt-related and clinical outcome measures. Methods MRE was collected at two imaging sites in 39 hydrocephalus patients and 33 healthy controls, along with demographic, shunt-related, and clinical outcome measures including headache and quality of life indices. Brain stiffness was quantified for whole brain, global white matter (WM), and lobar WM stiffness. Group differences in brain stiffness between patients and controls were compared using two-sample t-tests and multivariable linear regression to adjust for age, sex, and ventricular volume. Among patients, multivariable linear or logistic regression was used to assess which factors (age, sex, ventricular volume, age at first shunt, number of shunt revisions) were associated with brain stiffness and whether brain stiffness predicts clinical outcomes (quality of life, headache and depression). Results Brain stiffness was significantly reduced in patients compared to controls, both unadjusted (p≤0.002) and adjusted (p≤0.03) for covariates. Among hydrocephalic patients, lower stiffness was associated with older age in temporal and parietal WM and whole brain (WB) (beta (SE): -7.6 (2.5), p=0.004; -9.5 (2.2), p=0.0002; -3.7 (1.8), p=0.046), being female in global and frontal WM and WB (beta (SE): -75.6 (25.5), p=0.01; -66.0 (32.4), p=0.05; -73.2 (25.3), p=0.01), larger ventricular volume in global, and occipital WM (beta (SE): -11.5 (3.4), p=0.002; -18.9 (5.4), p=0.0014). Lower brain stiffness also predicted worse quality of life and a higher likelihood of depression controlling for all other factors. Conclusions Brain stiffness is reduced in hydrocephalus patients compared to healthy controls, and is associated with clinically-relevant functional outcome measures. MRE may emerge as a clinically-relevant biomarker to assess the neuropathological effects of hydrocephalus and shunting, and may be useful in evaluating the effects of therapeutic alternatives, or as a supplement, of shunting.
View
View
Ventriculo-peritoneal shunting devices for hydrocephalus
Article
  • Jun 2020
Background: Hydrocephalus is a common neurological disorder, caused by a progressive accumulation of cerebrospinal fluid (CSF) within the intracranial space that can lead to increased intracranial pressure, enlargement of the ventricles (ventriculomegaly) and, consequently, to brain damage. Ventriculo-peritoneal shunt systems are the mainstay therapy for this condition, however there are different types of shunt systems. Objectives: To compare the effectiveness and adverse effects of conventional and complex shunt devices for CSF diversion in people with hydrocephalus. Search methods: We searched the Cochrane Central Register of Controlled Trials (2020 Issue 2); Ovid MEDLINE (1946 to February 2020); Embase (Elsevier) (1974 to February 2020); Latin American and Caribbean Health Science Information Database (LILACS) (1980 to February 2020); ClinicalTrials.gov; and World Health Organization International Clinical Trials Registry Platform. Selection criteria: We selected randomised controlled trials or quasi-randomised trials of different types of ventriculo-peritoneal shunting devices for people with hydrocephalus. Primary outcomes included: treatment failure, adverse events and mortality. Data collection and analysis: Two review authors screened studies for selection, assessed risk of bias and extracted data. Due to the scarcity of data, we performed a Synthesis Without Meta-analysis (SWiM) incorporating GRADE for the quality of the evidence. Main results: We included six studies with 962 participants assessing the effects of standard valves compared to anti-syphon valves, other types of standard valves, self-adjusting CSF flow-regulating valves and external differential programmable pressure valves. All included studies started in a hospital setting and offered ambulatory follow-up. Most studies were conducted in infants or children with hydrocephalus from diverse causes. The certainty of the evidence for most comparisons was low to very low. 1. Standard valve versus anti-syphon valve Three studies with 296 randomised participants were included under this comparison. We are uncertain about the incidence of treatment failure in participants with standard valve and anti-syphon valves (very low certainty of the evidence). The incidence of adverse events may be similar in those with standard valves (range 0 to 1.9%) and anti-syphon valves (range 0 to 2.9%) (low certainty of the evidence). Mortality may be similar in those with standard valves (0%) and anti-syphon valves (0.9%) (RD 0.01%, 95% CI -0.02% to 0.03%, low certainty of the evidence). Ventricular size and head circumference may be similar in those with standard valves and anti-syphon valves (low certainty of the evidence). None of the included studies reported the quality of life of participants. 2. Comparison between different types of standard valves Two studies with 174 randomised participants were included under this comparison. We are uncertain about the incidence of treatment failure in participants with different types of standard valves (early postoperative period: RR 0.41, 95% CI 0.13 to 1.27; at 12 months follow-up: RR 1.17, 95% CI 0.72 to 1.92, very low certainty of the evidence). None of the included studies reported adverse events beyond those included under "treatment failure". We are uncertain about the effects of different types of standard valves on mortality (range 2% to 17%, very low certainty of the evidence). The included studies did not report the effects of these interventions on quality of life, ventricular size reduction or head circumference. 3. Standard valve versus self-adjusting CSF flow-regulating valve One study with 229 randomised participants addressed this comparison. The incidence of treatment failure may be similar in those with standard valves (42.98%) and self-adjusting CSF flow-regulating valves (39.13%) (low certainty of the evidence). The incidence of adverse events may be similar in those with standard valves (range 0 to 1.9%) and those with self-adjusting CSF flow-regulating valves (range 0 to 7.2%) (low certainty of the evidence). The included study reported no deaths in either group in the postoperative period. Beyond the early postoperative period, the authors stated that nine patients died (no disaggregated data by each type of intervention was available, low certainty of the evidence). The included studies did not report the effects of these interventions on quality of life, ventricular size reduction or head circumference. 4. External differential programmable pressure valve versus non-programmable valve One study with 377 randomised participants addressed this comparison. The incidence of treatment failure may be similar in those with programmable valves (52%) and non-programmable valves (52%) (RR 1.02, 95% CI 0.84 to 1.24, low certainty of the evidence). The incidence of adverse events may be similar in those with programmable valves (6.19%) and non-programmable valves (6.01%) (RR 0.97, 95% CI 0.44 to 2.15, low certainty of the evidence). The included study did not report the effect of these interventions on mortality, quality of life or head circumference. Ventricular size reduction may be similar in those with programmable valves and non-programmable valves (low certainty of the evidence). Authors' conclusions: Standard shunt valves for hydrocephalus compared to anti-syphon or self-adjusting CSF flow-regulating valves may cause little to no difference on the main outcomes of this review, however we are very uncertain due to the low to very low certainty of evidence. Similarly, different types of standard valves and external differential programmable pressure valves versus non-programmable valves may be associated with similar outcomes. Nevertheless, this review did not include valves with the latest technology, for which we need high-quality randomised controlled trials focusing on patient-important outcomes including costs.
View
Development and Validation of the Japanese Version of Quality of Life Assessment of Spina Bifida in Teenagers (QUALAS‐T‐J)
Article
  • Sep 2019
Background: Spina bifida (SB) is the second-most common birth defect in Japan. In recent years, health-related quality of life measurements have been used to assess the psychosocial status of children with SB. The Quality of Life Assessment of Spina Bifida in Teenagers (QUALAS-T) is a self-reported questionnaire for subjects aged 13 to 17 years with SB. It focuses particularly on factors related to independence, bladder and bowel. The purpose of this study was to develop and validate a Japanese version of QUALAS-T (QUALAS-T-J). Methods: Three urologists specialized in SB, 2 nurses, 1 statistician, and the author of the original version developed the QUALAS-T-J. Subjects with SB completed the QUALAS-T-J and the Japanese version of KIDSCREEN-27 (J-KIDSCREEN-27) independently from their parents. Results: A pilot study included 7 adolescents with SB and confirmed face and content validity. Sixty-nine adolescents with SB participated in the main survey. The intraclass correlation coefficient in the retest was 0.77, and Cronbach's alpha in each domain was 0.83 and 0.79. Validity was verified by factor analysis and convergent/divergent validity. 5 items converged with 1 domain same as Bladder and Bowel. The remaining 5 items divided into 2 domains, which are included in the concept of Family and Independence. The correlation between each domain of the QUALAS-T-J and J-KIDSCREEN-27 was low to moderate. Conclusion: The reliability and validity of the QUALAS-T-J were verified in Japanese adolescents with SB. The QUALAS-T-J would be a useful tool for communication between adolescents with SB and medical staff. This article is protected by copyright. All rights reserved.
View
Diffusion tensor imaging and ventricle volume quantification in patients with chronic shunt-treated hydrocephalus: a matched case-control study
Article
  • Jan 2018
  • J NEUROSURG
OBJECTIVE The object of this study was to use diffusion tensor imaging (DTI) and tract-based spatial statistics (TBSS) to characterize the long-term effects of hydrocephalus and shunting on white matter integrity and to investigate the relationship of ventricular size and alterations in white matter integrity with headache and quality-of-life outcome measures.METHODS Patients with shunt-treated hydrocephalus and age- and sex-matched healthy controls were recruited into the study and underwent anatomical and DTI imaging on a 3-T MRI scanner. All patients were clinically stable, had undergone CSF shunt placement before 2 years of age, and had a documented history of complaints of headaches. Outcome was scored based on the Headache Disability Inventory and the Hydrocephalus Outcome Questionnaire. Fractional anisotropy (FA) and other DTI-based measures (axial, radial, and mean diffusivity; AD, RD, and MD, respectively) were extracted in the corpus callosum and internal capsule with manual region-of-interest delineation and in other regions with TBSS. Paired t-tests, corrected with a 5% false discovery rate, were used to identify regions with significant differences between patients and controls. Within the patient group, linear regression models were used to investigate the relationship between FA or ventricular volume and outcome, as well as the effect of shunt-related covariates.RESULTSTwenty-one hydrocephalus patients and 21 matched controls completed the study, and their data were used in the final analysis. The authors found significantly lower FA for patients than for controls in 20 of the 48 regions, mostly posterior white matter structures, in periventricular as well as more distal tracts. Of these 20 regions, 17 demonstrated increased RD, while only 5 showed increased MD and 3 showed decreased AD. No areas of increased FA were observed. Higher FA in specific periventricular white matter tracts, tending toward FA in controls, was associated with increased ventricular size, as well as improved clinical outcome.CONCLUSIONS The study shows that TBSS-based DTI is a sensitive technique for elucidating changes in white matter structures due to hydrocephalus and chronic CSF shunting and provides preliminary evidence that DTI may be a valuable tool for tailoring shunt procedures to monitor ventricular size following shunting and achieve optimal outcome, as well as for guiding the development of alternate therapies for hydrocephalus.
View
Functional outcome in pediatric hydrocephalus: Results of applying the Spanish version of the Hydrocephalus Outcome Questionnaire
Article
  • Jan 2018
OBJECTIVE The aims of the study were to assess whether the Spanish version of the Hydrocephalus Outcome Questionnaire (HOQ) could be useful for the evaluation and comparison of outcomes in the patients in the authors’ region with those in patients in other institutions and to analyze predictors of functional outcome in patients with a shunt. METHODS The authors performed this cross-sectional study between March 2015 and April 2016. All the parents of a pediatric patient with hydrocephalus who attended the Hospital Regional Universitario de Málaga neurosurgery outpatient clinic were invited to enroll in the study and complete the HOQ–Spanish version. Age at diagnosis and at the time of the study, clinical data, shunt complications, and socioeconomic factors were also recorded. A descriptive analysis was performed, and independent variables related to the HOQ scores were studied. RESULTS A total of 132 patients participated in the study (100% participation rate). The mean ages were 16.74 months (range 0–142 months) at diagnosis and 10.45 years (range 5–16 years) at the time of the study. The mean overall HOQ score was 0.68 (on a scale from 0 [worse] to 1.0 [best]). Factors related to a worse quality of life were seizures, motor or visual impairment, shunt infection, need for shunt revision, need for more than 2 shunt revisions (range 0–8 revisions), symptomatic overdrainage, and older age at the time of the study. CONCLUSIONS The HOQ–Spanish version is useful in the authors’ region; the dimension scores found in this study were comparable to those previously reported by referral centers. Future goals should be to prevent shunt complications, routinely get children to reach functional status, and refer patients for adjuvant therapy promptly.
View
Ventriculo-peritoneal shunting devices for hydrocephalus: Protocols
Article
  • Jul 2017
This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To compare the effectiveness and safety of conventional and complex shunt devices for CSF diversion in people with hydrocephalus.
View
The Health Utilities Index (HUI®) System for Assessing Health-Related Quality of Life in Clinical Studies
Article
Full-text available
  • Aug 2001
This study examines health differences between first-generation immigrant and Canadian-born persons who share the same the ethnocultural origin, and the extent to which such differences reflect social structural and health-related behavioural contexts. Data from the 2000/2001 Canadian Community Health Survey show that first generation immigrants of Black and French race/ethnicity tend to have better health than their Canadian-born counterparts, while the opposite is true for those of South Asian, Chinese, and south and east European and Jewish origins. West Asians and Arabs and other Asian groups are advantaged in health regardless of country of birth. Health differences between ethnic foreign- and Canadian-born persons generally converge after adjusting for socio-demographic, SES, and lifestyle factors. Implications for health care policy and program development are discussed.
View
Cerebrospinal fluid shunt infection: A prospective study of risk factors
Article
  • Mar 2001
  • J NEUROSURG
Hydrocephalus is a common condition of childhood that usually requires insertion of a cerebrospinal fluid (CSF) shunt. Infection is one of the most devastating complications that may arise from the presence of CSF shunts. In this study, the authors prospectively analyzed perioperative risk factors for CSF shunt infection in a cohort of children. Between 1996 and 1999, 299 eligible patients underwent CSF shunt operations (insertions and revisions) that were observed by a research nurse at a tertiary care pediatric hospital. Several perioperative variables were recorded. All cases were followed postoperatively for 6 months to note any development of CSF shunt infection. A Cox proportional hazards model was used to analyze the relationship between the variables and the development of shunt infection. Thirty-one patients (10.4%) experienced shunt infection. Three perioperative variables were significantly associated with an increased risk of shunt infection: 1) the presence of a postoperative CSF leak (hazard ratio [HR] 19.16, 95% confidence interval [CI] 6.96-52.91); 2) patient prematurity (< 40 weeks' gestation at the time of shunt surgery: HR 4.72, 95% CI 1.71-13.06), and 3) the number of times the shunt system was inadvertently exposed to breached surgical gloves (HR 1.07, 95% CI 1.02-1.12). Three variables associated with an increased incidence of shunt infection have been identified. Changes in clinical practice should address these variables, as follows. 1) Great care should be taken intraoperatively to avoid a postoperative CSF leak. 2) Alternatives to placement of a CSF shunt in premature infants should be studied. 3) Surgeons should minimize manual contact with the shunt system and consider the use of double gloves.
View