Preoperative embolization and microsurgical resection of a cerebellar hemangioblastoma: embolization goals and technical nuances of the approaches. Illustrative case.

BACKGROUND: Resection remains the optimal treatment for hemangioblastomas, highly vascular tumors commonly located in the cerebellum or spinal cord. Preoperative embolization can be used with caution to reduce intraoperative blood loss and promote gross-total resection while reducing neurological morbidity. OBSERVATIONS: The authors report a case of a 44-year-old male who presented with worsening dizziness, gait imbalance, and diplopia. Imaging revealed a large vascular cerebellar mass with brainstem compression and hydrocephalus, concerning for hemangioblastoma. The patient underwent preoperative embolization of the main arterial supply from the right superior cerebellar artery, which devascularized the tumor and reduced the early venous shunting, followed by gross-total resection of the tumor. Preoperative embolization helped to facilitate safe microsurgical resection because this main arterial supply was deep to the planned surgical approach, and the Onyx cast served as a landmark of the dorsal midbrain and remaining arterial supply. This resulted in minimal intraoperative blood loss. At 2-year follow-up, the patient's diplopia and vertigo had resolved, and his gait continues to improve. LESSONS: Despite controversy regarding the use of preoperative embolization for hemangioblastoma resection, the authors emphasize its safety and utility during resection of a large cerebellar hemangioblastoma. Onyx embolization provided benefit in both reducing the tumor blood supply and serving as intraoperative visual guidance.

Open surgical ligation of a thoracic spinal epidural arteriovenous fistula causing thoracic myelopathy: illustrative case.

BACKGROUND: Spinal epidural arteriovenous fistulas (eAVFs) are rare spinal vascular malformations characterized by an abnormal connection from the paraspinal and paravertebral system to the epidural venous plexus. This contrasts with the more frequently seen spinal dural AVF, where the fistula is entirely intradural. Although endovascular repair is commonly performed for spinal eAVF, few cases require open surgical ligation. OBSERVATIONS: The authors present a case of a 74-year-old male with progressive thoracic myelopathy secondary to a spinal eAVF. Thoracic magnetic resonance imaging (MRI) showed intramedullary T2 signal hyperintensity from T8 to T12. Spinal angiography revealed a primary arterial supply from the right T11 segmental artery and minor supply from the left T11 branches with drainage into the ventral epidural space. The patient underwent T11-12 laminectomy and complete right T11-12 facetectomy for ligation of the fistula with T11-L1 fusion. A postoperative spinal angiogram showed resolution of the fistula. Postoperatively, the patient's myelopathy improved, and MRI showed a decrease in T2 cord intensity. LESSONS: Spinal eAVFs are rare lesions that differ from the more commonly seen intradural dural AVF in that the abnormal connection is in the epidural space, and they are often associated with a dilated epidural venous pouch. Treatment involves endovascular, open surgical, or combined approaches.

Mutations of the DNA repair gene PNKP in a patient with microcephaly, seizures, and developmental delay (MCSZ) presenting with a high-grade brain tumor.

Polynucleotide Kinase-Phosphatase (PNKP) is a bifunctional enzyme that possesses both DNA 3'-phosphatase and DNA 5'-kinase activities, which are required for processing termini of single- and double-strand breaks generated by reactive oxygen species (ROS), ionizing radiation and topoisomerase I poisons. Even though PNKP is central to DNA repair, there have been no reports linking PNKP mutations in a Microcephaly, Seizures, and Developmental Delay (MSCZ) patient to cancer. Here, we characterized the biochemical significance of 2 germ-line point mutations in the PNKP gene of a 3-year old male with MSCZ who presented with a high-grade brain tumor (glioblastoma multiforme) within the cerebellum. Functional and biochemical studies demonstrated these PNKP mutations significantly diminished DNA kinase/phosphatase activities, altered its cellular distribution, caused defective repair of DNA single/double stranded breaks, and were associated with a higher propensity for oncogenic transformation. Our findings indicate that specific PNKP mutations may contribute to tumor initiation within susceptible cells in the CNS by limiting DNA damage repair and increasing rates of spontaneous mutations resulting in pediatric glioma associated driver mutations such as ATRX and TP53.

The Longissimus Capitis Insertion as a Superficial Landmark for the Sigmoid Sinus: An Anatomical Study.

Objective Many external anatomical landmarks have been used for approximating deeper, intracranial structures. Herein, we evaluate the attachment of the longissimus capitis (LC) on the mastoid process as a landmark for the underlying sigmoid sinus. Methods Adult cadavers underwent dissection of the posterior occiput with special attention to the attachment of the LC muscle. Once the periphery of the muscle's tendon of attachment was determined, a burr hole was made in this area and evaluated internally for its relationship to the sigmoid sinus. Results From an intracranial view, burr holes on all sides were over the sigmoid sinus and just slightly lateral to the center of the sinus. The distance from the midline to the medial border of the insertion of the LC had a mean of 63.0 ± 7.2 mm. The width of the tendon of insertion of the LC on the mastoid process had a mean of 17.6 ± 5.7 mm. The length of the tendon insertion of the LC had a mean of 14.7 ± 4.7 mm. The distance from the inferior border of the insertion of the LC to the tip of the mastoid process had a mean of 6.2 ± 4.5 mm. Conclusion To our knowledge, use of the attachment site of the LC on the mastoid process as an external landmark for the underlying sigmoid sinus has not previously been reported. Based on our cadaveric findings, the sigmoid sinus is centered under the attachment of the LC regardless of the width of its tendon.

Aneurysmal subarachnoid hemorrhage during pregnancy: a comprehensive and systematic review of the literature.

Aneurysmal subarachnoid hemorrhage (aSAH) is an emergent condition requiring rapid intervention and prolonged monitoring. There are few recommendations regarding the management of aSAH in pregnancy. We identified all available literature and compiled management decisions as well as reported outcomes through a systematic literature review without meta-analysis to provide recommendations for management of aSAH during pregnancy. We included a total of 23 articles containing 54 cases of pregnancy-related aSAH in our review. From these reports and other literature, we evaluated information on aSAH pathophysiology, diagnosis, and management with respect to pregnancy. Early transfer to an appropriate facility with neurocritical care, a high-risk obstetric service, and a neurosurgery team available is crucial for the management of aSAH in pregnancy. Intensive monitoring and a multidisciplinary approach remain fundamental to ensure maternal and fetal health.

Transvenous embolization of bilateral indirect carotid-cavernous fistulas via a unilateral transorbital approach.

A 73-year-old man with a complex ophthalmologic history presented with several weeks of worsening diplopia, visual acuity, and proptosis bilaterally. Cerebral angiography demonstrated bilateral indirect Barrow type B carotid-cavernous fistulas (CCFs). Transarterial embolization was not attempted due to small arterial diameter and risk of stroke. Multiple attempts were made to access the fistula via a transfemoral venous approach and were unsuccessful. A transorbital puncture was performed, which allowed access to both cavernous sinuses via a unilateral approach. After embolization with Onyx, there was no residual fistula. The patient had a left-sided retrobulbar hematoma from the access. Right eye vision improved postoperatively.

Anatomic Study Quantifying the Relationship Between the Arcuate Eminence and the Root of the Zygoma: Application to Skull Base Surgery.

OBJECTIVE: The aim of this study was to define the structural relationship between the arcuate eminence (AE) and a known fixed external bony landmark, the root of the zygoma (ZR), and to determine its reliability as a consistent guide for guiding surgical approaches. To our knowledge, this is the only anatomic study to quantify the relationship between the AE and ZR. METHODS: Twenty-one dry temporal bones were measured using digital calipers. The distance from the posterior aspect of the ZR to the midpoint of the AE was measured. Additionally, the anteroposterior distance between the ZR and AE and vertical distance between the 2 structures were measured. Student's t-test was used to compare the left and right sides. RESULTS: An AE was found in every specimen. The mean ZR to AE distance was 30.9 mm. On most sides (91%), the ZR was located more inferiorly than the AE with a mean distance of 3 mm between the 2 structures. The mean distance between the AE and ZR was 17 mm. On all sides, the AE was located posterior to the ZR. No significant differences were found between sides. No anatomic variations or pathologic conditions were noted in any of the specimens. CONCLUSIONS: The ZR is an easily identifiable and consistent bony landmark often used by skull base surgeons. In this investigation, we measured the anatomic relationships between the ZR and AE. Such data might assist in planning surgical trajectories and minimizing complications when skull base pathologies are approached.

Anatomical Considerations for Endovascular Intervention for Extracranial Carotid Disease: A Review of the Literature and Recommended Guidelines.

Patient selection for endovascular intervention in extracranial carotid disease is centered on vascular anatomy. We review anatomical considerations for non-traumatic disease and offer guidelines in patient selection and management. We conducted a systematic literature review without meta-analysis for studies involving anatomical considerations in extracranial carotid intervention for non-traumatic disease. Anatomical considerations discussed included aortic arch variants, degree of vessel stenosis, angulation, tortuosity, and anomalous origins, and atheromatous plaque morphology, composition, and location. Available literature suggests that anatomical risks of morbidity are largely secondary to increased procedural times and difficulties in intervention system delivery. We recommend the prioritization of endovascular techniques on an individual basis in cases where accessible systems and surgeon familiarity provide an acceptable likelihood of rapid access and device deployment.

Laboratory assessments of therapeutic platelet inhibition in endovascular neurosurgery: complication prediction using the VerifyNow P2Y12 assay and thromboelastography with platelet mapping.

OBJECTIVE: Inhibition of platelet aggregation is universally used to prevent thromboembolic complications related to stent placement in endovascular neurosurgery, but excessive inhibition potentiates hemorrhagic complications. Previously, the authors demonstrated that two different commercially available measures of adenosine diphosphate (ADP)-dependent platelet inhibition-the VerifyNow P2Y12 clopidogrel assay (measured in platelet reactivity units [PRU]) and maximal amplitude (MA) attributable to ADP activity (MA-ADP) derived from thromboelastography (TEG) with platelet mapping (PM)-yielded wildly different results. This study sought to analyze observed complications to quantify the ideal therapeutic windows for both tests. METHODS: Ninety-one patients with simultaneous or near-simultaneous PRU and TEG-PM results who underwent craniocervical endovascular stenting at the authors' institution between September 2015 and November 2017 were identified and retrospectively enrolled. From November 2017 until June 2019, 109 additional patients were prospectively enrolled. For this study, in-hospital thrombotic and hemorrhagic complications (both CNS and non-CNS) were tabulated, and receiver operating characteristic (ROC) curve analysis was used to identify threshold values of the PRU and MA-ADP for predicting each type of complication. RESULTS: Of the 200 patients enrolled, 7 were excluded because of anemia or thrombocytopenia outside of the test manufacturer's specified ranges and 1 was excluded because they did not have a TEG-PM result. Including complications of all severities, there were a total of 15 CNS thrombotic complications, 1 access-site thrombotic complication, 3 CNS hemorrhages, 8 access-site hemorrhagic complications, and 3 hemorrhagic complications not affecting either the CNS or the access site. ROC curve analysis yielded therapeutic threshold values of 118-144 PRU. The results demonstrated PRU has a significant dose-dependent effect on the rates of thrombosis and hemorrhage. Logistic regression models did not demonstrate statistically significant relationships between the MA-ADP and either thrombosis or hemorrhage. ROC analysis based on these models is of little value and did not identify significant threshold values for MA-ADP. CONCLUSIONS: There continues to be poor correlation between the results of TEG-PM and PRU. PRU accurately predicted complications, with a relatively narrow ideal value range of 118-144. The MA-ADP alone does not appear able to accurately predict either hemorrhagic or thrombotic complications in this group.

Timing of cranioplasty: a 10.75-year single-center analysis of 754 patients.

OBJECTIVE Despite their technical simplicity, cranioplasty procedures carry high reported morbidity rates. The authors here present the largest study to date on complications after cranioplasty, focusing specifically on the relationship between complications and timing of the operation. METHODS The authors retrospectively reviewed all cranioplasty cases performed at Harborview Medical Center over the past 10.75 years. In addition to relevant clinical and demographic characteristics, patient morbidity and mortality data were abstracted from the electronic medical record. Cox proportional-hazards models were used to analyze variables potentially associated with the risk of infection, hydrocephalus, seizure, hematoma, and bone flap resorption. RESULTS Over the course of 10.75 years, 754 cranioplasties were performed at a single institution. Sixty percent of the patients who underwent these cranioplasties were male, and the median follow-up overall was 233 days. The 30-day mortality rate was 0.26% (2 cases, both due to postoperative epidural hematoma). Overall, 24.6% percent of the patients experienced at least 1 complication including infection necessitating explantation of the flap (6.6%), postoperative hydrocephalus requiring a shunt (9.0%), resorption of the flap requiring synthetic cranioplasty (6.3%), seizure (4.1%), postoperative hematoma requiring evacuation (2.3%), and other (1.6%). The rate of infection was significantly higher if the cranioplasty had been performed < 14 days after the initial craniectomy (p = 0.007, Holm-Bonferroni-adjusted p = 0.028). Hydrocephalus was significantly correlated with time to cranioplasty (OR 0.92 per 10-day increase, p < 0.001) and was most common in patients whose cranioplasty had been performed < 90 days after initial craniectomy. New-onset seizure, however, only occurred in patients who had undergone their cranioplasty > 90 days after initial craniectomy. Bone flap resorption was the least likely complication for patients whose cranioplasty had been performed between 15 and 30 days after initial craniectomy. Resorption was also correlated with patient age, with a hazard ratio of 0.67 per increase of 10 years of age (p = 0.001). CONCLUSIONS Cranioplasty performed between 15 and 30 days after initial craniectomy may minimize infection, seizure, and bone flap resorption, whereas waiting > 90 days may minimize hydrocephalus but may increase the risk of seizure.

Endovascular Coil Retrieval Using Dual Synchronous Solitaire Revascularization Devices.

BACKGROUND AND IMPORTANCE: Coil migration is a potential complication of endovascular aneurysm treatment. Dislodged coils into the parent artery require retrieval to prevent thromboembolic complications. A variety of techniques for coil retrieval have been described, including the use of single stentrievers and aspiration catheters. CLINICAL PRESENTATION: The use of 2 stentrievers, as opposed to a single device, was thought to be advantageous in coil removal by the additional point of friction due to the extensive length of coil stretching and earlier failure of a single device. CONCLUSION: In this report, 2 synchronous Solitaire FR Revascularization Devices (Covidien/Medtronic, Dublin, Ireland) were deployed, 1 distal and 1 proximal, to retrieve an inadvertently deployed coil.

International multicentre validation of the arteriovenous malformation-related intracerebral haemorrhage (AVICH) score.

OBJECTIVE: The recently published arteriovenous malformation-related intracerebral haemorrhage (AVICH) score showed better outcome prediction for patients with arteriovenous malformation (AVM)-related intracerebral haemorrhage (ICH) than other AVM or ICH scores. Here we present the results of a multicentre, external validation of the AVICH score. METHODS: All participating centres (n=11) provided anonymous data on 325 patients to form the Spetzler-Martin (SM) grade, the supplemented SM (sSM) grade, the ICH score and the AVICH score. Modified Rankin score (mRS) at last follow-up (mean 25.6 months) was dichotomized into favourable (mRS 0-2, n=210) and unfavourable (mRS 3-6;n=115). Univariate and AUROC analyses were performed to validate the AVICH score. RESULTS: Except nidus structure and AVM size, all single parameters forming the SM, sSM, ICH and AVICH score and the scores itself were significantly different between both outcome groups in the univariate analysis. The AVICH score was confirmed to be the highest predictive outcome score with an AUROC of 0.765 compared with 0.705 for the ICH score and 0.682 for the sSM grade. CONCLUSION: The multicentre-validated AVICH score predicts clinical outcome superior to pre-existing scores. We suggest the routine use of this score for future clinical outcome prediction and in clinical research. TRIAL REGISTRATION NUMBER: NCT02920645.