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Kevin Carr, Scott L. Zuckerman, Luke Tomycz and Matthew M. Pearson

The endoscopic resection of intraventricular tumors represents a unique challenge to the neurological surgeon. These neoplasms are invested deep within the brain parenchyma and are situated among neurologically vital structures. Additionally, the cerebrospinal fluid system presents a dynamic pathway for resected tumors to be mobilized and entrapped in other regions of the brain. In 2011, the authors treated a 3-year-old girl with a third ventricular mass identified on stereotactic brain biopsy as a WHO Grade IV CNS primitive neuroectodermal tumor. After successful neoadjuvant chemotherapy, endoscopic resection was performed. Despite successful resection of the tumor, the operation was complicated by mobilization of the resected tumor and entrapment in the atrial horn of the lateral ventricle. Using a urological stone basket retriever, the authors were able to retrieve the intact tumor without additional complications. The flexibility afforded by the nitinol urological stone basket was useful in the endoscopic removal of a free-floating intraventricular tumor. This device may prove to be useful for other practitioners performing these complicated intraventricular resections.

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Jonathan A. Forbes, Adam S. Reig, Luke D. Tomycz and Noel Tulipan

Object

Intracranial hypertension resulting from compression of the superior sagittal sinus (SSS) by an overlying depressed calvarial fracture is a rare condition. Primary surgical treatment for the symptomatic patient in this setting traditionally involves elevation of the fracture, which often carries significant associated morbidity.

Methods

The authors report a case involving a 6-year-old boy who suffered a closed, depressed, parietooccipital fracture as the result of an unhelmeted all-terrain vehicle accident. This fracture caused compression and subsequent thrombosis of the SSS, which resulted in CSF malabsorption and progressive intracranial hypertension. Initially headache free following the injury, he had developed severe and unremitting headaches by postinjury Day 7. A CT angiography study of the head obtained at this time exhibited thrombosis of the SSS underlying the depressed calvarial fracture. Subsequent lumbar puncture demonstrated markedly elevated intrathecal pressures. Large volumes of CSF were removed, with temporary improvement in symptoms. After medical management with anticoagulation failed, the decision was made to proceed with image-guided ventriculoperitoneal shunt insertion.

Results

The patient's headaches resolved immediately following the procedure, and anticoagulation therapy was reinstituted. Follow-up images obtained 4 months after the injury demonstrated evidence of resolution of the depressed fracture, with recanalization of the SSS. The anticoagulation therapy was then discontinued. To the authors' knowledge, this report is the first description of ventriculoperitoneal shunt insertion as the primary treatment of this infrequent condition.

Conclusions

This report demonstrates that select patients with this presentation can undergo CSF diversion in lieu of elevation of the depressed skull fracture—a surgical procedure shown to be associated with increased risks when the depressed fracture overlies the posterior SSS. The literature on this topic is reviewed and management of this condition is discussed.

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Mayshan Ghiassi, Mahan Ghiassi, Elyne Kahn, Luke Tomycz, Michael Ayad and Oran Aaronson

The authors report on the case of a 24-year-old man who presented with back pain and radiculopathy due to epidural venous engorgement in the setting of a congenitally absent inferior vena cava. Despite initial improvement after steroid administration, the patient's health ultimately declined over a period of weeks, and signs and symptoms of cauda equina syndrome manifested. Lumbar decompression was performed and involved coagulation and resection of the compressive epidural veins. No complications occurred, and the patient made a full neurological recovery.

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Travis R. Ladner, Michael C. Dewan, Matthew A. Day, Chevis N. Shannon, Luke Tomycz, Noel Tulipan and John C. Wellons III

OBJECT

Osseous anomalies of the craniocervical junction are hypothesized to precipitate the hindbrain herniation observed in Chiari I malformation (CM-I). Previous work by Tubbs et al. showed that posterior angulation of the odontoid process is more prevalent in children with CM-I than in healthy controls. The present study is an external validation of that report. The goals of our study were 3-fold: 1) to externally validate the results of Tubbs et al. in a different patient population; 2) to compare how morphometric parameters vary with age, sex, and symptomatology; and 3) to develop a correlative model for tonsillar ectopia in CM-I based on these measurements.

METHODS

The authors performed a retrospective review of 119 patients who underwent posterior fossa decompression with duraplasty at the Monroe Carell Jr. Children’s Hospital at Vanderbilt University; 78 of these patients had imaging available for review. Demographic and clinical variables were collected. A neuroradiologist retrospectively evaluated preoperative MRI examinations in these 78 patients and recorded the following measurements: McRae line length; obex displacement length; odontoid process parameters (height, angle of retroflexion, and angle of retroversion); perpendicular distance to the basion-C2 line (pB–C2 line); length of cerebellar tonsillar ectopia; caudal extent of the cerebellar tonsils; and presence, location, and size of syringomyelia. Odontoid retroflexion grade was classified as Grade 0, > 90°; Grade I,85°–89°; Grade II, 80°–84°; and Grade III, < 80°. Age groups were defined as 0–6 years, 7–12 years, and 13–17 years at the time of surgery. Univariate and multivariate linear regression analyses, Kruskal-Wallis 1-way ANOVA, and Fisher’s exact test were performed to assess the relationship between age, sex, and symptomatology with these craniometric variables.

RESULTS

The prevalence of posterior odontoid angulation was 81%, which is almost identical to that in the previous report (84%). With increasing age, the odontoid height (p < 0.001) and pB–C2 length (p < 0.001) increased, while the odontoid process became more posteriorly inclined (p = 0.010). The pB–C2 line was significantly longer in girls (p = 0.006). These measurements did not significantly correlate with symptomatology. Length of tonsillar ectopia in pediatric CM-I correlated with an enlarged foramen magnum (p = 0.023), increasing obex displacement (p = 0.020), and increasing odontoid retroflexion (p < 0.001).

CONCLUSIONS

Anomalous bony development of the craniocervical junction is a consistent feature of CM-I in children. The authors found that the population at their center was characterized by posterior angulation of the odontoid process in 81% of cases, similar to findings by Tubbs et al. (84%). The odontoid process appeared to lengthen and become more posteriorly inclined with age. Increased tonsillar ectopia was associated with more posterior odontoid angulation, a widened foramen magnum, and an inferiorly displaced obex.

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Travis R. Ladner, Michael C. Dewan, Matthew A. Day, Chevis N. Shannon, Luke Tomycz, Noel Tulipan and John C. Wellons III

OBJECT

The clinical significance of radiological measurements of the craniocervical junction in pediatric Chiari I malformation (CM-I) is yet to be fully established across the field. The authors examined their institutional experience with the pB–C2 line (drawn perpendicular to a line drawn between the basion and the posterior aspect of the C-2 vertebral body, at the most posterior extent of the odontoid process at the dural interface). The pB–C2 line is a measure of ventral canal encroachment, and its relationship with symptomatology and syringomyelia in pediatric CM-I was assessed.

METHODS

The authors performed a retrospective review of 119 patients at the Monroe Carell Jr. Children's Hospital at Vanderbilt University who underwent posterior fossa decompression with duraplasty, 78 of whom had imaging for review. A neuroradiologist retrospectively evaluated preoperative and postoperative MRI examinations performed in these 78 patients, measuring the pB–C2 line length and documenting syringomyelia. The pB–C2 line length was divided into Grade 0 (< 3 mm) and Grade I (≥ 3 mm). Statistical analysis was performed using the t-test for continuous variables and Fisher's exact test analysis for categorical variables. Multivariate logistic and linear regression analyses were performed to assess the relationship between pB–C2 line grade and clinical variables found significant on univariate analysis, controlling for age and sex.

RESULTS

The mean patient age was 8.5 years, and the mean follow-up duration was 2.4 years. The mean pB–C2 line length was 3.5 mm (SD 2 mm), ranging from 0 to 10 mm. Overall, 65.4% of patients had a Grade I pB–C2 line. Patients with Grade I pB–C2 lines were 51% more likely to have a syrinx than those with Grade 0 pB–C2 lines (RR 1.513 [95% CI 1.024–2.90], p = 0.021) and, when present, had greater syrinx reduction (3.6 mm vs 0.2 mm, p = 0.002). Although there was no preoperative difference in headache incidence, postoperatively patients with Grade I pB–C2 lines were 69% more likely to have headache reduction than those with Grade 0 pB–C2 lines (RR 1.686 [95% CI 1.035–2.747], p = 0.009). After controlling for age and sex, pB–C2 line grade remained an independent correlate of headache improvement and syrinx reduction.

CONCLUSIONS

Ventral canal encroachment may explain the symptomatology of select patients with CM-I. The clinical findings presented suggest that patients with Grade I pB–C lines2, with increased ventral canal obstruction, may experience a higher likelihood of syrinx reduction and headache resolution from decompressive surgery with duraplasty than those with Grade 0 pB–C2 lines.