Ventricular Size Involvement in Neuropsychological Outcomes in Pediatric Hydrocephalus

The current literature is inconclusive regarding the direct association between large
ventricle size and poorer cognitive outcomes. This uncertainty stems from relatively few
studies, problems with study design (possible confounding factors), varying study populations
of hydrocephalus, and conflicting evidence. If the association is proven then aggressive
strategies must be developed to maximally decrease ventricular size in these children to
preserve long-term cognitive functioning. However, if the association is disproven then
previous reservations for procedures such as Endoscopic Third Ventriculostomy (ETV) or
higher-resistance shunting to prevent overdrainage complications will be further minimized.
Either finding has a major potential to further improve the quality of life in our pediatric
hydrocephalus patients. This study is unique as it will include pediatric patients presenting
with hydrocephalus from a wide range of etiologies. This study is also unique in that we
propose to conduct a brief pre-operative neuropsychological examination to act as internal
controls along with the outcomes obtained at a more extensive neuropsychological examination
at six months after initial hydrocephalus treatment surgery.

Inclusion Criteria: Patients will be eligible for enrollment if they:

- are 5 years of age or older; and

- have been newly diagnosed with hydrocephalus to be managed surgically with either a
cerebrospinal fluid (CSF) shunt or an ETV; and

- have one of the following etiologies for hydrocephalus: aqueductal stenosis,
supratentorial and posterior fossa tumors both benign and malignant, post-traumatic,
myelomeningocele, tectal gliomas, post-infectious, and following spontaneous
intraventricular hemorrhage (IVH). Malignant tumors have been included as the
neuropsychological deficits secondary to adjuvant chemotherapy and radiation have not
been demonstrated within the proposed study's 6 month window.

Exclusion Criteria: Patients will be ineligible for enrollment if ANY of the following is
true or anticipated:

- present with a Glasgow Coma Scale (GCS) score of less than 14/15, cerebellar mutism,
or cognitive deficits so severe as to make neuropsychological testing impossible; OR

- have etiologies of diffuse intrinsic pontine glioma, atypical teratoid rhabdoid tumor,
or any tumor with cerebral or spinal metastases (these patients' clinical course and
survival are highly unpredictable); OR

- are not expected to survive for 6 months; OR

- are unable or unwilling to participate in the study and with the neuropsychological
exam; OR

- due to limitations of neuropsychological testing, blind and deaf children will be
excluded. Children for whom English is not their primary language will be included if
they have attended 1 or more years of English language based schooling.