Abhaya V. Kulkarni and Ruth Donnelly
Abhaya V. Kulkarni, Ruth Donnelly, Donald J. Mabbott, and Elysa Widjaja
Larger-than-normal ventricles can persist in children following hydrocephalus treatment, even if they are asymptomatic and clinically well. This study aims to answer the following question: do large ventricles result in brain injuries that are detectable on diffusion tensor imaging (DTI) and/or in measurable neurocognitive deficits in children with stable, treated hydrocephalus that are not seen in children with small ventricles?
For this prospective study, we recruited 23 children (age range 8–18 years) with hydrocephalus due to aqueductal stenosis or tectal glioma who were asymptomatic following hydrocephalus treatment that had been performed at least 2 years earlier. All patients underwent detailed DTI and a full battery of neuropsychological tests. Correlation analysis was performed to assess the relationship between DTI parameters, neurocognitive tests, and ventricular size. The false-discovery rate method was used to adjust for multiple comparisons.
The median age of these 23 children at the time of assessment was 15.0 years (interquartile range [IQR] 12.1–17.6 years), and the median age at the first hydrocephalus treatment was 5.8 years (IQR 2.2 months–12.8 years). At the time of assessment, 17 children had undergone endoscopic third ventriculostomy and 6 children had received a shunt. After adjusting for multiple comparisons, there were no significant correlations between any neurocognitive test and ventricular volume, any DTI parameter and ventricular volume, or any DTI parameter and neurocognitive test.
Our data do not show an association between large ventricular size and additional white matter injury or worse neurocognitive deficits in asymptomatic children with stable, treated hydrocephalus caused by a discrete blockage of the cerebral aqueduct. Further investigations using larger patient samples are needed to validate these results.
Abhaya V. Kulkarni, Ruth Donnelly, and Iffat Shams
The Hydrocephalus Outcome Questionnaire (HOQ) is an established means of measuring quality of life, but the cognitive component of this questionnaire has never been formally compared with gold-standard neuropsychological test scores. The authors hypothesized that the HOQ Cognitive Health score would demonstrate a relatively strong correlation with neuropsychological test scores, whereas much weaker correlations would be seen for HOQ Physical and Social-Emotional Health scores.
A cross-sectional study of children with long-standing hydrocephalus presenting to The Hospital for Sick Children's Neurosurgery Clinic was performed between July 2006 and September 2008. Participating children and families completed the HOQ and a battery of 21 standard neuropsychological tests and questionnaires. Pearson correlation analysis was then performed.
A total of 83 patients (81% participation) was accrued; the mean age was 11.5 ± 3.4 years (mean ± SD) at the time of assessment. The mean age at hydrocephalus treatment was 1.3 ± 2.6 years. The mean overall HOQ score was 0.69 ± 0.21. The HOQ Cognitive score had a moderate or strong correlation with 19 (90%) of 21 neuropsychological test scores, much more so than the HOQ Social-Emotional score (5 moderate or strong correlations, 24%) and the HOQ Physical score (1 moderate correlation, 5%). For 19 neuropsychological tests (90%), the HOQ Cognitive score had a stronger correlation than the other scores. The HOQ Cognitive score had particularly strong correlations with the Verbal IQ, List Learning, Behavior Problems, and Metacognitive Abilities components.
Data from a wide-ranging representative sample of children with long-standing hydrocephalus provide added evidence of the validity of the HOQ Cognitive score and the overall domain structure of the HOQ itself.
Benjamin Warf, Solomon Ondoma, Abhaya Kulkarni, Ruth Donnelly, Miriam Ampeire, Joan Akona, Collin R. Kabachelor, Ronald Mulondo, and Brian Kaaya Nsubuga
Despite lower failure and infection rates compared with shunt placement, it has not been known whether endoscopic third ventriculostomy/choroid plexus cauterization (ETV/CPC) might be inferior in regard to neurocognitive development. This study is the first to describe neurocognitive outcome and ventricle volume in infants with hydrocephalus due to myelomeningocele that was treated primarily by ETV/CPC.
The modified Bayley Scales of Infant Development (BSID-III) test was administered to 93 children with spina bifida who were 5–52 months of age. Fifty-five of these children had been treated by ETV/CPC, 19 received ventriculoperitoneal (VP) shunts, and 19 had required no treatment for hydrocephalus. Raw scores were converted to scaled scores for comparison with age-corrected norms. Ventricular volume was assessed by frontal/occipital horn ratio (FOR) calculated from late postoperative CT scans. The mean values between and among groups of patients were compared using independent samples t-test and ANOVA. The comparison of mean values to population normal means was performed using the single-sample t-test. Linear regression analyses were performed using BSID scores as the dependent variables, with treatment group and ventricular size (FOR) as the independent variables. Probability values < 0.05 were considered significant.
. There was no significant difference in mean age at assessment among groups (p = 0.8). The mean scale scores for untreated patients were no different from normal (all p > 0.27) in all portions of the BSID (excluding gross motor), and were generally significantly better than those for both VP shunt–treated and ETV/CPC groups. The ETV/ CPC-treated patients had nonsignificantly better mean scores than patients treated with VP shunts in all portions of the BSID (all p > 0.06), except receptive communication, which was significantly better for the ETV/CPC group (p = 0.02). The mean FOR was similar among groups, with no significant difference between the untreated group and either the VP shunt or ETV/CPC groups. The FOR did not correlate with performance.
The ETV/CPC and VP shunt groups had similar neurocognitive outcomes. Neurocognitive outcomes for infants not requiring treatment for hydrocephalus were normal and significantly better than in those requiring treatment. The mean ventricular volume was similar among all 3 groups, and significantly larger than normal. There was no association between FOR and performance. Stable mild-to-moderate ventriculomegaly alone should not trigger intervention in asymptomatic infants with spina bifida.
Steven J. Schiff, Abhaya V. Kulkarni, Edith Mbabazi-Kabachelor, John Mugamba, Peter Ssenyonga, Ruth Donnelly, Jody Levenbach, Vishal Monga, Mallory Peterson, Venkateswararao Cherukuri, and Benjamin C. Warf
Hydrocephalus in infants, particularly that with a postinfectious etiology, is a major public health burden in Sub-Saharan Africa. The authors of this study aimed to determine whether surgical treatment of infant postinfectious hydrocephalus in Uganda results in sustained, long-term brain growth and improved cognitive outcome.
The authors performed a trial at a single center in Mbale, Uganda, involving infants (age < 180 days old) with postinfectious hydrocephalus randomized to endoscopic third ventriculostomy plus choroid plexus cauterization (ETV+CPC; n = 51) or ventriculoperitoneal shunt (VPS; n = 49). After 2 years, they assessed developmental outcome with the Bayley Scales of Infant Development, Third Edition (BSID-III), and brain volume (raw and normalized for age and sex) with CT scans.
Eighty-nine infants were assessed for 2-year outcome. There were no significant differences between the two surgical treatment arms in terms of BSID-III cognitive score (p = 0.17) or brain volume (p = 0.36), so they were analyzed together. Raw brain volumes increased between baseline and 2 years (p < 0.001), but this increase occurred almost exclusively in the 1st year (p < 0.001). The fraction of patients with a normal brain volume increased from 15.2% at baseline to 50.0% at 1 year but then declined to 17.8% at 2 years. Substantial normalized brain volume loss was seen in 21.3% patients between baseline and year 2 and in 76.7% between years 1 and 2. The extent of brain growth in the 1st year was not associated with the extent of brain volume changes in the 2nd year. There were significant positive correlations between 2-year brain volume and all BSID-III scores and BSID-III changes from baseline.
In Sub-Saharan Africa, even after successful surgical treatment of infant postinfectious hydrocephalus, early posttreatment brain growth stagnates in the 2nd year. While the reasons for this finding are unclear, it further emphasizes the importance of primary infection prevention and mitigation strategies along with optimizing the child’s environment to maximize brain growth potential.
Jessica R. Lane, Paddy Ssentongo, Mallory R. Peterson, Joshua R. Harper, Edith Mbabazi-Kabachelor, John Mugamba, Peter Ssenyonga, Justin Onen, Ruth Donnelly, Jody Levenbach, Venkateswararao Cherukuri, Vishal Monga, Abhaya V. Kulkarni, Benjamin C. Warf, and Steven J. Schiff
This study investigated the incidence of postoperative subdural collections in a cohort of African infants with postinfectious hydrocephalus. The authors sought to identify preoperative factors associated with increased risk of development of subdural collections and to characterize associations between subdural collections and postoperative outcomes.
The study was a post hoc analysis of a randomized controlled trial at a single center in Mbale, Uganda, involving infants (age < 180 days) with postinfectious hydrocephalus randomized to receive either an endoscopic third ventriculostomy plus choroid plexus cauterization or a ventriculoperitoneal shunt. Patients underwent assessment with the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III; sometimes referred to as BSID-III) and CT scans preoperatively and then at 6, 12, and 24 months postoperatively. Volumes of brain, CSF, and subdural fluid were calculated, and z-scores from the median were determined from normative curves for CSF accumulation and brain growth. Linear and logistic regression models were used to characterize the association between preoperative CSF volume and the postoperative presence and size of subdural collection 6 and 12 months after surgery. Linear regression and smoothing spline ANOVA were used to describe the relationship between subdural fluid volume and cognitive scores. Causal mediation analysis distinguished between the direct and indirect effects of the presence of a subdural collection on cognitive scores.
Subdural collections were more common in shunt-treated patients and those with larger preoperative CSF volumes. Subdural fluid volumes were linearly related to preoperative CSF volumes. In terms of outcomes, the Bayley-III cognitive score was linearly related to subdural fluid volume. The distribution of cognitive scores was significantly different for patients with and those without subdural collections from 11 to 24 months of age. The presence of a subdural collection was associated with lower cognitive scores and smaller brain volume 12 months after surgery. Causal mediation analysis demonstrated evidence supporting both a direct (76%) and indirect (24%) effect (through brain volume) of subdural collections on cognitive scores.
Larger preoperative CSF volume and shunt surgery were found to be risk factors for postoperative subdural collection. The size and presence of a subdural collection were negatively associated with cognitive outcomes and brain volume 12 months after surgery. These results have suggested that preoperative CSF volumes could be used for risk stratification for treatment decision-making and that future clinical trials of alternative shunt technologies to reduce overdrainage should be considered.