Atypical Bilateral Multifocal Congenital Toxoplasmosis Retinochoroiditis: Case Report With Literature Review.

BACKGROUND: Toxoplasmosis gondii is ubiquitously present on earth and infection, including congenital infection, is common. Neurological, developmental, and ocular effects can be devastating in the congenital toxoplasmosis population. At present, there is no standard, nation-wide neonatal screening for this disease in the United States. CASE PRESENTATION: A 17-month-old Caucasian female presented to our institution by way of referral for macular scarring. She was diagnosed with intrauterine growth retardation and born with low birth weight and microcephaly at an outside institution, but no systemic workup was conducted at that time. On ocular examination, she was found to have nystagmus and extensive multifocal chorioretinal pigmented scars involving the macula and peripheral retina in both eyes with fibrous vitreous strands extending between scars in the right eye. Toxoplasmosis immunoglobulin G was found to be highly positive. Magnetic resonance imaging of the brain showed supratentorial intracranial calcifications. CONCLUSIONS: Our patient presented with severe chorioretinal lesions, microcephaly, and nystagmus with a positive immunoglobulin G toxoplasmosis titer. She did not receive any evaluation, including TORCH infectious panel workup, on being born with low birth weight and microcephaly. There are currently no national programs in place for toxoplasmosis to be included in routine neonatal screening, despite the grave sequelae of congenital infection or that studies in other countries have shown cost-effectiveness in early screening and treatment.

Hydrocephalus research funding from the National Institutes of Health: a 10-year perspective.

OBJECT: Funding of hydrocephalus research is important to the advancement of the field. The goal of this paper is to describe the funding of hydrocephalus research from the National Institutes of Health (NIH) over a recent 10-year period. METHODS: The NIH online database RePORT (Research Portfolio Online Reporting Tools) was searched using the key word "hydrocephalus." Studies were sorted by relevance to hydrocephalus. The authors analyzed funding by institute, grant type, and scientific approach over time. RESULTS: Over $54 million was awarded to 59 grantees for 66 unique hydrocephalus proposals from 48 institutions from 2002 to 2011. The largest sources of funding were the National Institute of Neurological Disease and Stroke and the National Institute of Child Health and Human Development. Of the total, $22 million went to clinical trials, $15 million to basic science, and $10 million to joint ventures with small business (Small Business Innovation Research or Small Business Technology Transfer). Annual funding varied from $2.3 to $8.1 million and steadily increased in the second half of the observation period. The number of new grants also went from 15 in the first 5 years to 27 in the second 5 years. A large portion of the funding has been for clinical trials. Funding for shunt-device development grew substantially. Support for training of hydrocephalus investigators has been low. CONCLUSIONS: Hydrocephalus research funding is low compared with that for other conditions of similar health care burden. In addition to NIH applications, researchers should pursue other funding sources. Small business collaborations appear to present an opportunity for appropriate projects.

Long-term change in ventricular size following endoscopic third ventriculostomy for hydrocephalus due to tectal plate gliomas.

OBJECT: Endoscopic third ventriculostomy (ETV) is an alternative to shunt placement in children with hydrocephalus due to tectal plate gliomas (TPGs). However, controversy remains regarding the amount of ventricular size reduction that should be expected after ETV. This study investigates ventricular size change after ETV for TPGs. METHODS: Twenty-two children were identified from a 15-year retrospective database of neuroendoscopic procedures performed at the authors' institution, Children's Hospital of Alabama, in patients with a minimum of 1 year of follow-up. Clinical outcomes, including the need for further CSF diversion and symptom resolution, were recorded. The frontal and occipital horn ratio (FOR) was measured on pre- and postoperative, 1-year, and last follow-up imaging studies. RESULTS: In 17 (77%) of 22 children no additional procedure for CSF diversion was required. Of those in whom CSF diversion failed, 4 underwent successful repeat ETV and 1 required shunt replacement. Therefore, in 21 (96%) of 22 patients, CSF diversion was accomplished with ETV. Preoperative and postoperative imaging was available for 18 (82%) of 22 patients. The FOR decreased in 89% of children who underwent ETV. The FOR progressively decreased 1.7%, 11.2%, and 12.7% on the initial postoperative, 1-year, and last follow-up images, respectively. The mean radiological follow-up duration for 18 patients was 5.4 years. When ETV failed, the FOR increased at the time of failure in all patients. Failure occurred 1.6 years after initial ETV on average. The mean clinical follow-up period for all 22 patients was 5.3 years. In all cases clinical improvement was demonstrated at the last follow-up. CONCLUSIONS: Endoscopic third ventriculostomy successfully treated hydrocephalus in the extended follow-up period of patients with TPGs. The most significant reduction in ventricular size was observed at the the 1-year followup, with only modest reduction thereafter.

The economic impact of ventriculoperitoneal shunt failure.

OBJECT: Detailed costs to individuals with hydrocephalus and their families as well as to third-party payers have not been previously described. The purpose of this study was to determine the primary caregiver out-of-pocket expenses and the third-party payer reimbursement rate associated with a shunt failure episode. METHODS: A retrospective study of children born between 2000 and 2005 who underwent initial ventriculoperitoneal (VP) shunt placement and who subsequently experienced a shunt failure requiring surgical intervention within 2 years of their initial shunt placement was conducted. Institutional reimbursement and demographic data from Children's Hospital of Alabama (CHA) were augmented with a caregiver survey of any out-of pocket expenses encountered during the shunt failure episode. Institutional reimbursements and caregiver out-of-pocket expenses were then combined to provide the cost for a shunt failure episode at CHA. RESULTS: For shunt failures, the median reimbursement total was $5008 (interquartile range [IQR] $2068-$17,984), the median caregiver out-of-pocket expenses was $419 (IQR $251-$1112), and the median total cost was $5411 (IQR $2428-$18,582). Private insurance reimbursed at a median rate of $5074 (IQR $2170-$14,852) compared with public insurance, which reimbursed at a median rate of $4800 (IQR $1876-$19,395). Caregivers with private insurance reported a median $963 (IQR $322-$1741) for out-of-pocket expenses, whereas caregivers with public insurance reported a median $391 (IQR $241-$554) for out-of-pocket expenses (p = 0.017). CONCLUSIONS: This study confirmed that private insurance reimbursed at a higher rate, and that although patients had a shorter length of stay as compared with those with public insurance, their out-of-pocket expenses associated with a shunt failure episode were greater. However, it could not be determined if the significant difference in out-of-pocket expenses between those with private and those with public insurance was due directly to the cost of shunt failure. This model does not take into consideration community resources and services available to those with public insurance. These resources and services could offset the out-of-pocket burden, and therefore should be considered in future cost models.

Evaluating the Children's Hospital of Alabama endoscopic third ventriculostomy experience using the Endoscopic Third Ventriculostomy Success Score: an external validation study.

OBJECT: Endoscopic third ventriculostomy (ETV) success is dependent on patient characteristics including age, origin of hydrocephalus, and history of shunt therapy. Using these factors, an Endoscopic Third Ventriculostomy Success Score (ETVSS) model was constructed to predict success of therapy. This study reports a single-institution experience with ETV and explores the ETVSS model validity. METHODS: A retrospective chart review identified 151 consecutive patients who underwent ETV at a pediatric hospital between August 1995 and December 2009. Of these 151, 136 patients had at least 6 months of clinical follow-up. Data concerning patient characteristics, operative characteristics, radiological findings, complications, and success of ETV were collected. The actual success rates were compared with those predicted by the ETVSS model. RESULTS: The actual success rate of ETV at 6 months was 68.4% (93 of 136 patients), which compared well to the predicted ETVSS of 76.5% ± 12.5% (± SD). The C-statistic was 0.74 (95% CI 0.65-0.83), suggesting that the ability of the ETVSS to discriminate failures from successes was good. Secondary ETV was found to have a hazard ratio for failure of 4.2 (95% CI 2.4-7.2) compared with primary ETV (p < 0.001). The complication rate was 9.3% with no deaths. At the first radiological follow-up, the increased size of ventricles had a hazard ratio for failure of 3.0 (95% CI 1.5-6.0) compared with patients in whom ventricle size either remained stable or decreased (p = 0.002). CONCLUSIONS: The ETVSS closely predicts the actual success of ETV, fitting the statistical model well. Shortcomings of the model were identified in overestimating success in patients with ETVSS ≤ 70, which may be attributable to the poor success of secondary ETVs in the authors' patient population.

Small-ventricle neuroendoscopy for pediatric brain tumor management.

OBJECT: The use of intraventricular endoscopy to achieve diagnosis or to resect accessible intraventricular or paraventricular tumors has been described in the literature in both adults and children. Traditionally, these techniques have not been used in patients with small ventricles due to the perceived risk of greater morbidity. The authors review their experience with the effectiveness and safety of endoscopic brain tumor management in children with small ventricles. METHODS: Between July 2002 and December 2009, 24 children with endoscopically managed brain tumors were identified. Radiological images were reviewed by a radiologist blinded to study goals and clinical setting. Patients were categorized into small-ventricle and ventriculomegaly groups based on frontal and occipital horn ratio. Surgical success was defined a priori and analyzed between groups. Trends were identified in selected subgroups, including complications related to pathological diagnosis and surgeon experience. RESULTS: Six children had small ventricles and 18 had ventriculomegaly. The ability to accomplish surgical goals was statistically equivalent in children with small ventricles and those with ventriculomegaly (83% vs 89%, respectively, p = 1.00). There were no complications in the small-ventricle cohort, but in the ventriculomegaly cohort there were 2 cases of postoperative hemorrhages and 1 case of infection. All hemorrhagic complications occurred in patients with high-grade tumor histopathological type and were early in the surgeon's endoscopic career. CONCLUSIONS: Based on our experience, endoscopy should not be withheld in children with intraventricular tumors and small ventricles. Complications appear to be more dependent on tumor histopathological type and surgeon experience than ventricular size.

Small ventricular access prior to rigid neuroendoscopy.

The authors describe a new technique that may be used in conjunction with neuronavigation or freehand techniques for obtaining small ventricular access. Using this modification, the introducer sheath and trocar can be guided down a ventriculostomy tract with endoscopic visual control. With increasing focus on endoscopic therapies in patients without hydrocephalus, this adjunct, based on the authors' experience, may provide an additional technique for safely treating patients.