Luke Tomycz

Evaluating the relationship of the pB–C2 line to clinical outcomes in a 15-year single-center cohort of pediatric Chiari I malformation

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. Childrens 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 Fishers 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.

“Real-world” comparison of non-invasive imaging to conventional catheter angiography in the diagnosis of cerebral aneurysms

Background: Based on numerous reports citing high sensitivity and specificity of non-invasive imaging [e.g. computed tomography angiography (CTA) or magnetic resonance angiography (MRA)] in the detection of intracranial aneurysms, it has become increasingly difficult to justify the role of conventional angiography [digital subtraction angiography (DSA)] for diagnostic purposes. The current literature, however, largely fails to demonstrate the practical application of these technologies within the context of a “real-world” neurosurgical practice. We sought to determine the proportion of patients for whom the additional information gleaned from 3D rotational DSA (3DRA) led to a change in treatment. Methods: We analyzed the medical records of the last 361 consecutive patients referred to a neurosurgeon at our institution for evaluation of “possible intracranial aneurysm” or subarachnoid hemorrhage (SAH). Only those who underwent non-invasive vascular imaging within 3 months prior to DSA were included in the study. For asymptomatic patients without a history of SAH, aneurysms less than 5 mm were followed conservatively. Treatment was advocated for patients with unruptured, non-cavernous aneurysms measuring 5 mm or larger and for any non-cavernous aneurysm in the setting of acute SAH. Results: For those who underwent CTA or MRA, the treatment plan was changed in 17/90 (18.9%) and 22/73 (30.1%), respectively, based on subsequent information gleaned from DSA. Several reasons exist for the change in the treatment plan, including size and location discrepancies (e.g. cavernous versus supraclinoid), or detection of a benign vascular variant rather than a true aneurysm. Conclusions: In a “real-world” analysis of intracranial aneurysms, DSA continues to play an important role in determining the optimal management strategy.

Use of endovascular embolization to treat a ruptured arteriovenous malformation in a pregnant woman: a case report

IntroductionPregnancy has been linked to increased rates of arteriovenous malformation rupture. This link remains a matter of debate and very few studies have addressed the management of arteriovenous malformation in pregnancy. Unruptured arteriovenous malformations in pregnant woman generally warrant conservative management due to the low rupture risk. When pregnant women present with ruptured arteriovenous malformation, however, surgery is often indicated due to the increased risk of re-rupture and associated mortality. Endovascular embolization is widely accepted as an important component of contemporary, multimodal therapy for arteriovenous malformations. Although rarely curative, embolization can facilitate subsequent surgical resection or radiosurgery. No previous reports have been devoted to the endovascular management of an arteriovenous malformation in a pregnant woman.Case presentationA 23-year-old Caucasian woman presented with headache and visual disturbance after the rupture of a left parieto-occipital arteriovenous malformation in the 22nd week of her pregnancy. After involving high-risk obstetric consultants and taking precautions to shield the fetus from ionizing radiation, we proceeded with a single stage of endovascular embolization followed soon after by open surgical resection of the arteriovenous malformation. There were several goals for the angiography in this patient: to better understand the anatomy of the arteriovenous malformation, including the number and orientation of feeding arteries and draining veins; to look for associated pre-nidal or intra-nidal aneurysms; and to partially embolize the arteriovenous malformation via safely-accessible feeders to facilitate surgical resection and minimize blood loss and operative morbidity.ConclusionFrom our experience and review of the literature, we maintain that ruptured arteriovenous malformations in pregnancy may be managed in a similar manner to those in non-gravid women. Precautions should be taken to reduce the operative time and exposure of the fetus to ionizing radiation and contrast agents.

Findings on Preoperative Brain MRI Predict Histopathology in Children with Cerebellar Neoplasms

Background/Aims: The majority of pediatric patients with cerebellar neoplasms harbor pilocytic astrocytomas (PAs), medulloblastomas, or ependymomas. Knowledge of a preoperative likelihood of histopathology in this group of patients has the potential to influence many aspects of care. Previous studies have demonstrated hyperintensity on diffusion-weighted imaging to correlate with medulloblastomas. Recently, measurement of T2-weighted signal intensity (T2SI) was shown to be useful in identification of low-grade cerebellar neoplasms. The goal of this study was to assess whether objective findings on these MRI sequences reliably correlated with the underlying histopathology. Methods: We reviewed the radiologic findings of 50 pediatric patients who underwent resection of a cerebellar neoplasm since 2003 at our institution. Region of interest placement was used to calculate the relative diffusion-weighted signal intensity (rDWSI) and relative T2SI (rT2SI) of each neoplasm. Results: Tukey’s multiple comparison test demonstrated medulloblastomas to have significantly higher rDWSIs than PAs/ependymomas, and PAs to have significantly higher rT2SIs than medulloblastomas/ependymomas. A simple method consisting of sequential measurement of rDWSI and rT2SI to predict histopathology was then constructed. Using this method, 39 of 50 (78%) tumors were accurately predicted. Conclusion: Measurement of rDWSI and rT2SI using standard MRI of the brain can be used to predict histopathology with favorable accuracy in pediatric patients with cerebellar tumors.

Endoscopic removal of an intraventricular primitive neuroectodermal tumor: retrieval of a free-floating fragment using a urological basket retriever.

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.

The spectrum of management practices in nontraumatic subarachnoid hemorrhage: A survey of high-volume centers in the United States.

Background: There is a considerable variety of management practices for nontraumatic subarachnoid hemorrhage (ntSAH) across high-volume centers in the United States. We sought to design a survey which would highlight areas of controversy in the modern management of ntSAH and identify specific areas of interest fo further study. Methods: A questionnaire on management practices in ntSAH was formulated using a popular web-based survey tool (SurveyMonkey™, Palo Alto, CA) and sent to endovascular neurointerventionists and cerebrovascular surgeons who manage a high volume of these patients annually. Two-hundred questionnaires were delivered electronically, and after a period of 2 months, the questionnaire was resent to nonresponders. Results: Seventy-three physicians responded, representing a cross-section of academic and other high-volume centers of excellence from around the country. On average, the responding interventionists in this survey each manage approximately 100 patients with ntSAH annually. Over 57% reported using steroids to treat this patient population. Approximately 18% of the respondents use intrathecal thrombolytics in ntSAH. Over 90% of responding physicians administer nimodipine to all patients with ntSAH. Over 40% selectively administer antiepileptic drugs to patients with ntSAH. Several additional questions were posed regarding the methods of detecting and treating vasospasm, as well as the indications for CSF diversion in patients with ntSAH further demonstrating the great diversity in management. Conclusion: This survey illustrates the astonishing variety of treatment practices for patients with ntSAH and underscores the need for further study.

Open thoracic cordotomy as a treatment option for severe, debilitating pain.

OBJECTIVE The treatment of patients with debilitating lower extremity or medically refractory quadrant pain presents a challenge for management. Contemporary neuromodulatory therapies may not be affordable or practical, especially in patients with limited life expectancy or from countries with limited resources. We present a small retrospective series to evaluate the role of open thoracic cordotomy as a practice option in the treatment of patients with severe, unilateral, medically refractory pain of the lower abdominal quadrant, hip, or leg. Technical aspects of the procedure, anatomic pathways within the spinal cord, and intraoperative maneuvers are described. METHODS The medical records of 9 patients (7 men, 2 women; median age, 57 years) treated between 1998 and 2010 were reviewed. Each patient underwent open thoracic cordotomy after 1998 for severe lower quadrant or lower extremity pain refractory to multiple other treatment modalities. The indications for surgery included cancer-related pain (4 of 9), postherpetic neuralgia (2 of 9), post-spinal cord injury pain (2 of 9), and multiple sclerosis (1 of 9). RESULTS Six of nine patients reported improvement in their postoperative level of pain after a median follow-up of 31 weeks. Complications included ipsilateral lower extremity weakness, urinary incontinence, and the development of new postcordotomy pain. A higher incidence of complications, including ipsilateral motor weakness, was observed in this series than with previous reports of percutaneous cervical cordotomy (PCC). CONCLUSIONS Although open thoracic cordotomy may be cautiously recommended as a treatment option in certain settings, this procedure should be viewed only as a second-line treatment option in settings where the technology and expertise to perform PCC are available.

Posterior odontoid process angulation in pediatric Chiari I malformation: an MRI morphometric external validation study

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. Childrens 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 Fishers 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.

Time to First Shunt Failure in Pediatric Patients over 1 Year Old: A 10-Year Retrospective Study

Studies comparing alternatives to ventriculoperitoneal (VP) shunting for treatment of hydrocephalus have often relied upon data from an earlier era that may not be representative of contemporary shunt survival outcomes. We sought to determine the shunt survival rate of our cohort and compare our results to previously published shunt survival and endoscopic third ventriculostomy (ETV) success rates. We identified 95 patients between 1 and 18 years of age, who underwent initial VP shunt placement between January 2001 and December 2010. Our study shows a shunt survival rate of 85% at 6 months and 79% at 2 years, for initial shunts in pediatric patients over 1 year of age in this cohort. The overall infection rate was 3%. This compares favorably with published success rates of ETV at similar time points as well as with the rate of infection. This suggests that ventricular shunting remains a viable alternative to ETV in the older child.

Cauda equina syndrome secondary to an absent inferior vena cava managed with surgical decompression.

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 patients 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.