Neuroendoscopic Navigated One-Step Removal of Crossed Ventricular Catheter Fragments: Technical Note

Introduction Neuroendoscopy plays an important role in minimally invasive neurosurgery. The authors reported an interesting case of a pediatric patient with multiple ventriculoperitoneal shunt (VPS) revision surgeries, presenting with VPS infection and with two crossed intraventricular floating catheter fragments, successfully removed through a neuroendoscopic navigated one-step procedure. A literature review regarding this exceedingly rare condition has also been discussed. Case Description An 11-year-old female patient with a history of congenital hydrocephalus was admitted to the emergency department with symptoms of intracranial hypertension, psychomotor agitation, and tetraparesis. She had a history of previous multiple VPS revisions. She had an urgent brain computed tomography scan that documented hydrocephalus; the VPS's intraventricular catheter tip was sited at the level of the right frontal horn. Two small floating catheter fragments, not connected to the VPS, were identified: the first close to the right lateral ventricle at the level of the right occipital horn, the second one between the right occipital horn and the third ventricle. First, she underwent an exteriorization of the distal catheter for VPS. Cerebrospinal fluid examination documented hyperproteinorrachia and a positive culture for Staphylococcus aureus. Then a navigated right transfrontal endoscopic approach to the right lateral ventricle was performed extending to the previous burr hole and achieving a wide range of working angle with a rigid 0-degree lens endoscope. Intermittent irrigation generating convective flow was performed such as to mobilize the catheters tip gently upward, to remove them by grasping. Finally, a whole VPS replacement has been performed. Conclusion Persistence of intraventricular floating catheter fragments can lead to subacute or chronic infections. Neuroendoscopic retrieval represents a safe and effective alternative to a more extensive and invasive surgical approach. However, the exact catheter tip identification, grasping, and removal can be difficult to achieve, due to the technical instrumentation characteristics and altered intraventricular anatomy in chronic congenital hydrocephalus. In our experience, endoscopic convective flow induction through saline irrigation can determine floating intraventricular catheter fragments movement aiming to their identification and subsequent successful endoscopic retrieval.

Radiological imaging of choroid plexus tumors: Systematic approach toward diagnosing a choroid plexus tumor

Choroid plexus tumors (CPTs) are rare intraventricular tumors comprising approximately 1% of all brain tumors. The common locations are the lateral ventricle (most common location in children), the third and fourth ventricles (most common location in adults), and cerebellopontine angle. Here, we present the case of a 10-month-old child with complaints of fever (99.6F), abnormal eye movements, and bilateral papilledema. Ultrasound cranium of the child revealed a well-defined hyperechoic lesion adjacent to the trigone and occipital horn of the right lateral ventricle. No vascularity could be appreciated on color Doppler. Non-contrast computed tomography of the brain showed a well-defined lobulated mass lesion epicentered at the choroid plexus of the right lateral ventricle which was hyperdense relative to the brain parenchyma with specks of calcification. Contrast-enhanced magnetic resonance imaging showed a solid intensely enhancing lobulated mass lesion with frond-like morphology originating from the choroid plexus of the occipital horn of the right lateral ventricle. Surgical excision of the CPT was done under aseptic conditions. We try to reinforce the ultrasound (USG), CT, and MRI findings of a CPT which ultimately came out to be a choroid plexus papilloma (CPP) on histopathological examination.

Ventriculomegaly and postoperative lateral/third ventricular blood as predictors of cerebrospinal fluid diversion following posterior fossa tumor resection

OBJECTIVE Postoperative hydrocephalus occurs in one-third of children after posterior fossa tumor resection. Although models to predict the need for CSF diversion after resection exist for preoperative variables, it is unknown which postoperative variables predict the need for CSF diversion. In this study, the authors sought to determine the clinical and radiographic predictors for CSF diversion in children following posterior fossa tumor resection. METHODS This was a retrospective cohort study involving patients ≤ 18 years of age who underwent resection of a primary posterior fossa tumor between 2000 and 2018. The primary outcome was the need for CSF diversion 6 months after surgery. Candidate predictors for CSF diversion including age, race, sex, frontal occipital horn ratio (FOHR), tumor type, tumor volume and location, transependymal edema, papilledema, presence of postoperative intraventricular blood, and residual tumor were evaluated using a best subset selection method with logistic regression. RESULTS Of the 63 included patients, 26 (41.3%) had CSF diversion at 6 months. Patients who required CSF diversion had a higher median FOHR (0.5 vs 0.4) and a higher percentage of postoperative intraventricular blood (30.8% vs 2.7%) compared with those who did not. A 0.1-unit increase in FOHR or intraventricular blood was associated with increased odds of CSF diversion (OR 2.9 [95% CI 1.3–7.8], p = 0.02 and OR 20.2 [95% CI 2.9–423.1], p = 0.01, respectively) with an overfitting-corrected concordance index of 0.68 (95% CI 0.56–0.80). CONCLUSIONS The preoperative FOHR and postoperative intraventricular blood were significant predictors of the need for permanent CSF diversion within 6 months after posterior fossa tumor resection in children.

Efficacy and safety of the Miethke programmable differential pressure valve (proGAV®2.0): a single-centre retrospective analysis

Purpose Achieving decompression without CSF over-drainage remains a challenge in hydrocephalus. Differential pressure valves are a popular treatment modality, with evidence suggesting that incorporation of gravitational units helps minimise over-drainage. This study seeks to describe the utility of the proGAV®2.0 programmable valve in a paediatric population. Methods Clinical records and imaging of all patients fitted with proGAV®2.0 valves and Miethke fixed-pressure valves between 2014 and 2019 at our tertiary centre were analysed. Patient demographics, indication for shunt and valve insertion/revision and time to shunt/valve revision were collected. Ventricular linear metrics (fronto-occipital horn ratio (FOHR) and fronto-occipital horn width ratio (FOHWR)) were collected pre- and post-valve insertion. Microsoft Excel and SPSS v24 were used for data collection and statistical analysis. Results Eighty-eight proGAV®2.0 valves were inserted in a population of 77 patients (n = 45 males (58%), mean age 5.1 years (IQR: 0.4–11.0 years)). A total of 102 Miethke fixed-pressure valves were inserted over the same time period. Median follow-up was 17.5 months (1.0–47.3). One (1.1%) proGAV®2.0 was revised due to over-drainage, compared to 2 (1.9%) fixed-pressure valves (p > 0.05). ProGAV®2.0 insertion resulted in a significant decrease in the mean number of revisions per patient per year (1.77 vs 0.25; p = 0.01). Overall shunt system survival with the proGAV®2.0 was 80.4% at 12 months, and mean time to revision was 37.1 months, compared to 31.0 months (95%CI: 25.7–36.3) and 58.3% in fixed-pressure valves (p < 0.01). Significant decreases were seen following proGAV®2.0 insertion in both FOHR and FOHWR, by 0.014 (95%CI: 0.006–0.023, p = 0.002) and 0.037 (95%CI: 0.005–0.069, p = 0.024) respectively. Conclusion The proGAV®2.0 provides effective decompression of hydrocephalic patients, significantly reduces the number of valve revisions per patient and had a significantly greater mean time to revision than fixed-pressure valves.

Occipital Horn Syndrome as a Result of Splice Site Mutations in ATP7A. No Activity of ATP7A Splice Variants Missing Exon 10 or Exon 15

Disease-causing variants in ATP7A lead to two different phenotypes associated with copper deficiency; a lethal form called Menkes disease (MD), leading to early death, and a much milder form called occipital horn syndrome (OHS). Some investigators have proposed that an ATP7A transcript missing exon 10 leads to a partly active protein product resulting in the OHS phenotype. Here, we describe an individual with OHS, a biology professor, who survived until age 62 despite a splice site mutation, leading to skipping of exon 15. ATP7A transcripts missing exon 10, or exon 15 preserve the reading frame, but it is unknown if either of these alternative transcripts encode functional protein variants. We have investigated the molecular consequence of splice site mutations leading to skipping of exon 10 or exon 15 which have been identified in individuals with OHS, or MD. By comparing ATP7A expression in fibroblasts from three individuals with OHS (OHS-fibroblasts) to ATP7A expression in fibroblasts from two individuals with MD (MD-fibroblasts), we demonstrate that transcripts missing either exon 10 or exon 15 were present in similar amounts in OHS-fibroblasts and MD-fibroblasts. No ATP7A protein encoded from these transcripts could be detected in the OHS and MD fibroblast. These results, combined with the observation that constructs encoding ATP7A cDNA sequences missing either exon 10, or exon 15 were unable to complement the high iron requirement of the ccc2Δ yeast strain, provide evidence that neither a transcript missing exon 10 nor a transcript missing exon 15 results in functional ATP7A protein. In contrast, higher amounts of wild-type ATP7A transcript were present in the OHS-fibroblasts compared with the MD-fibroblasts. We found that the MD-fibroblasts contained between 0 and 0.5% of wild-type ATP7A transcript, whereas the OHS-fibroblasts contained between 3 and 5% wild-type transcripts compared with the control fibroblasts. In summary these results indicate that protein variants encoded by ATP7A transcripts missing either exon 10 or exon 15 are not functional and not responsible for the OHS phenotype. In contrast, expression of only 3-5% of wild-type transcript compared with the controls permits the OHS phenotype.

MR-Brain Causing Confusion

This is a T2 weighted image (T2WI). In T2WI compartments filled with fluid appear brighter (as is the case of the CSF in the lateral ventricles). On the contrary, tissues with a high fat content appear as dark. This T2WI demonstrates layering of debris (figure 2- marked red star) in the occipital horn of the lateral ventricles. In this particular patient, the complete MRI report additionally demonstrated that the debris did not show a high T1 signal, demonstrated diffusion restriction, and a high FLAIR sequence. There was also restricting material observed in the fourth ventricle and the sylvian fissures bilaterally. There were no parenchymal changes or pathological contrast enhancements within the brain tissue. Whilst this appearance could represent blood, the appearance of the debrinous material itself was more in keeping with infective/pus material within the ventricles suggestive of ventriculitis.

Detailed Analysis of Hydrocephalus and Hindbrain Herniation After Prenatal and Postnatal Myelomeningocele Closure: Report From a Single Institution

BACKGROUND The Management of Myelomeningocele Study (MOMS) demonstrated that fetal myelomeningocele (fMMC) closure results in improved hydrocephalus and hindbrain herniation when compared to postnatal closure. OBJECTIVE To report on the outcomes of a single institution's experience in the post-MOMS era, with regard to hydrocephalus absence and hindbrain herniation resolution. METHODS A single-center retrospective study of a subset of post-MOMS patients who underwent fetal/postnatal myelomeningocele closure was performed. Primary outcomes included cerebrospinal fluid (CSF) diversion status and hindbrain herniation resolution. Families were contacted via telephone for outcome information if care was transitioned to outside institutions. Univariate/multivariable analyses were performed using several prenatal and postnatal variables. RESULTS From January 2011 to May 2016, data were reviewed from families of 62 postnatal and 119 fMMC closure patients. In the postnatal group, 80.6% required CSF diversion compared to 38.7% fetal cases (P &lt; .01). Hindbrain herniation resolution occurred in 81.5% fetal repairs compared to 32.6% postnatal (P &lt; .01). In the fetal group, fetal/premature neonatal demise occurred in 6/119 (5.0%) patients. There was a 42.0% decrease (95% CI –55.2 to –28.8) and 48.9% increase (95% CI 33.7 to 64.1) in risk difference for CSF diversion and hindbrain herniation resolution, respectively, in the fetal group. On univariate analysis for both groups, prenatal atrial diameter, frontal–occipital horn ratio, and hindbrain herniation resolution were significantly associated with the absence of clinical hydrocephalus. The treatment of hydrocephalus was significantly delayed in the fetal group compared to the postnatal group (10 mo vs 13.8 d). CONCLUSION This study demonstrates the benefits of fMMC closure with regard to CSF dynamics.