Outcomes of hydrocephalus secondary to congenital toxoplasmosis

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OBJECTIVE

Hydrocephalus occurs in children with congenital toxoplasmosis and can lead to severe disability. In these cases, the decision to intervene is often influenced by the expectation of neurological recovery. In this study, clinical responses to neurosurgical intervention in children with hydrocephalus secondary to congenital toxoplasmosis are characterized.

METHODS

Sixty-five participants with hydrocephalus due to congenital Toxoplasma gondii infection were evaluated as part of the National Collaborative Chicago-based Congenital Toxoplasmosis Study, and their neuroradiographic findings were reviewed. Clinical outcomes were scored on the basis of cognition and motor skills through the use of IQ scores and Gross Motor Function Classification System (GMFCS) level. Outcomes were then analyzed in relation to approach to management, anatomy of hydrocephalus, and time from diagnosis of hydrocephalus to surgical intervention.

RESULTS

There was considerable variation in the outcomes of patients whose hydrocephalus was treated in early life, ranging from normal cognitive and motor function to profound developmental delay and functional limitation. Of the 65 participants included in the study, IQ and GMFCS level were available for 46 (70.8%). IQ and motor score were highly correlated (r = −0.82, p < 0.001). There were people with differing patterns of hydrocephalus or thickness of cortical mantle on initial presentation who had favorable outcomes. Time to neurosurgical intervention data were available for 31 patients who underwent ventriculoperitoneal (VP) shunt placement. Delayed shunt placement beyond 25 days after diagnosis of hydrocephalus was associated with greater cognitive impairment (p = 0.02). Motor impairment also appeared to be associated with shunt placement beyond 25 days but the difference did not achieve statistical significance (p = 0.13). Among those with shunt placement within 25 days after diagnosis (n = 19), the mean GMFCS level was 1.9 ± 1.6 (range 1–5). Five (29.4%) of 17 of these patients were too disabled to participate in formal cognitive testing, after excluding 2 patients with visual difficulties or language barriers that precluded IQ testing. Of the patients who had VP shunt placement 25 or more days after diagnosis (n = 12), the mean GMFCS level was 2.7 ± 1.4 (range 1–4). Of these, 1 could not participate in IQ testing due to severe visual difficulties and 8 (72.7%) of the remaining 11 due to cognitive disability.

CONCLUSIONS

VP shunt placement in patients with hydrocephalus caused by congenital toxoplasmosis can contribute to favorable clinical outcomes, even in cases with severe hydrocephalus on neuroimaging. Shunt placement within 25 days of diagnosis was statistically associated with more favorable cognitive outcomes. Motor function appeared to follow the same pattern although it did not achieve statistical significance.

ABBREVIATIONS ETV = endoscopic third ventriculostomy; GMFCS = Gross Motor Function Classification System; NCCCTS = National Collaborative Chicago-based Congenital Toxoplasmosis Study; VP = ventriculoperitoneal; WISC-III = Wechsler Intelligence Scale for Children–III; WPPSI-R = Wechsler Preschool and Primary Scale of Intelligence–Revised.

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Article Information

Correspondence Rima McLeod: The University of Chicago, Chicago, IL. rmcleod@uchicago.edu.

INCLUDE WHEN CITING Published online September 6, 2019; DOI: 10.3171/2019.6.PEDS18684.

D.M. and D.F. contributed equally to this work.

Disclosures Dr. Penn is a consultant for Arkis Biosciences, Inc., and receives options on shares.

© AANS, except where prohibited by US copyright law.

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Figures

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    Distribution of anatomical patterns of hydrocephalus. Figure is available in color online only.

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    IQ scores and GMFCS levels of patients with VP shunt placement. An IQ score of 0 indicates a person too disabled to participate in cognitive testing. Each dot represents the outcome of an individual person in the study. The black cross represents the mean value among patients with VP shunt placement. The box over the outcomes marks the 25th percentile (bottom of the box) and 75th percentile (top of the box). The bottom and top black lines mark the minimum and maximum, respectively. Figure is available in color online only.

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    IQ scores and GMFCS levels of patients with no neurosurgical intervention. An IQ score of 0 indicates a person too disabled to participate in cognitive testing. Each dot represents the outcome of an individual patient in the study. The black cross represents the mean value among patients with no neurosurgical intervention. The box over the outcomes marks the 25th percentile (bottom of the box) and 75th percentile (top of the box). The bottom and top black lines mark the minimum and maximum, respectively. *Those not requiring neurosurgical intervention had cortical mantle thickness of at least several centimeters, no progression, and development that was normal. Figure is available in color online only.

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    Outcomes by anatomy of hydrocephalus. IQ scores and GMFCS levels of patients with aqueductal obstruction (AO), foraminal obstruction (FO), mixed aqueductal/foraminal obstruction (MO), and no obvious intraventricular obstruction (NO). An IQ score of 0 indicates a person too disabled to participate in cognitive testing. Each symbol represents the outcome of an individual person in the study. The black cross represents the mean value among patients with each anatomical pattern of hydrocephalus. The box over the outcomes marks the 25th percentile (bottom of the box) and 75th percentile (top of the box). The bottom and top black lines mark the minimum and maximum, respectively. Figure is available in color online only.

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    Brain CT images of patients with hydrocephalus before and after VP shunt placement. A: Image of a patient with hydrocephalus caused by aqueductal obstruction. B: Image of the same patient in panel A after shunt placement, showing improvement. C: Image of a patient with hydrocephalus caused by obstruction of the foramina of Monro. D: Image of the same patient in panel C after shunt placement, showing improvement. E: Image of a patient with a pattern of hydrocephalus with no obvious evidence of intraventricular obstruction causing ventricular enlargement. From Hutson et al.: Patterns of hydrocephalus caused by congenital Toxoplasma gondii infection associate with parasite genetics. Clin Infect Dis 2015: 61(12) 1831–1834, by permission of Oxford University Press on behalf of the Infectious Diseases Society of America. F: Image of the same patient in panel E after shunt placement, showing improvement.

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    Time interval from diagnosis of hydrocephalus to surgical intervention and associated outcomes. Each small square represents the time to intervention and outcome of an individual person in the study. An IQ score of 0 indicates a person too disabled to participate in cognitive testing. The green box indicates those with an IQ > 40 and shunt placement within 25 days, and the blue box indicates those with a GMFCS level of 1–2 and shunt placement within 25 days. Figure is available in color online only.

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