A 12-month-old boy with a history of bilateral retinoblastoma refractory to systemic chemotherapy, laser therapy and cryotherapy, with excellent response to previous intra-arterial melphalan infusion, presents with active tumour deposits in the right eye. Repeat intra-arterial chemotherapy was recommended. Previous bilateral melphalan infusion was uneventful using flow-guided catheterisation technique. Direct catheterisation of the right ophthalmic artery was unsuccessful despite employment of several flow-guided and over-the-wire catheters. Superselective catheterisation of the ipsilateral middle meningeal artery was unable to identify an anastomotic connection to the ophthalmic artery; however, angiography of the anterior deep temporal artery identified an alternate route for chemotherapy infusion. The anterior deep temporal artery was successfully and safely catheterised to infuse chemotherapy into the ophthalmic artery. The anterior deep temporal artery is an important potential anastomotic connection to the ophthalmic artery that can be used safely and effectively for central retinal artery chemotherapy infusion for retinoblastoma treatment.

Object

Management of unruptured arteriovenous malformations (AVMs) is controversial. In the first randomized trial of unruptured AVMs (A Randomized Trial of Unruptured Brain Arteriovenous Malformations [ARUBA]), medically managed patients had a significantly lower risk of death or stroke and had better outcomes. The University of California, San Francisco (UCSF) was one of the participating ARUBA sites. While 473 patients were screened for eligibility, only 4 patients were enrolled in ARUBA. The purpose of this study is to report the treatment and outcomes of all ARUBA-eligible patients at UCSF.

Methods

The authors compared the treatment and outcomes of ARUBA-eligible patients using prospectively collected data from the UCSF brain AVM registry. Similar to ARUBA, they compared the rate of stroke or death in observed and treated patients and used the modified Rankin Scale to grade outcomes.

Results

Of 74 patients, 61 received an intervention and 13 were observed. Most treated patients had resection with or without preoperative embolization (43 [70.5%] of 61 patients). One of the 13 observed patients died after AVM hemorrhage. Nine of the 61 treated patients had a stroke or died. There was no significant difference in the rate of stroke or death (HR 1.34, 95% CI 0.12-14.53, p = 0.81) or clinical impairment (Fisher's exact test, p > 0.99) between observed and treated patients.

Conclusions

The risk of stroke or death and degree of clinical impairment among treated patients was lower than reported in ARUBA. The authors found no significant difference in outcomes between observed and treated ARUBA-eligible patients at UCSF. Results in ARUBA-eligible patients managed outside that trial led to an entirely different conclusion about AVM intervention, due to the primary role of surgery, judicious surgical selection with established outcome predictors, and technical expertise developed at high-volume AVM centers.

We describe a unique case of bilateral cervical spinal dural arteriovenous fistulas mimicking an intracranial dural arteriovenous fistula near the foramen magnum. We review its detection via MRI and digital subtraction angiography and subsequent management through surgical intervention. Pitfalls in diagnostic angiography are discussed with reference to accurate location of the fistula site. The venous anastomotic connections of the posterior midline spinal vein to the medial posterior medullary vein, posterior fossa bridging veins, and dural venous sinuses of the skull base are discussed with reference to problem-solving in this complex case. The mechanism of myelopathy through venous hypertension produced by spinal dural fistulas is also emphasized.

Contrast staining of brain parenchyma identified on non-contrast CT performed after DSA in patients with acute ischemic stroke (AIS) is an incompletely understood imaging finding. We hypothesize contrast staining to be an indicator of brain injury and suspect the fate of involved parenchyma to be cerebral infarction. Seventeen years of AIS data were retrospectively analyzed for contrast staining. Charts were reviewed and outcomes of the stained parenchyma were identified on subsequent CT and MRI. Thirty-six of 67 patients meeting inclusion criteria (53.7%) had contrast staining on CT obtained within 72 hours after DSA. Brain parenchyma with contrast staining in patients with AIS most often evolved into cerebral infarction (81%). Hemorrhagic transformation was less likely in cases with staining compared with hemorrhagic transformation in the cohort that did not have contrast staining of the parenchyma on post DSA CT (6% versus 25%, respectively, OR 0.17, 95% CI 0.017 - 0.98, p = 0.02). Brain parenchyma with contrast staining on CT after DSA in AIS patients was likely to infarct and unlikely to hemorrhage.

Background and purpose

The three largest adult stroke trials investigating mechanical embolectomy retrieval devices in acute stroke (the Merci, Multi Merci and Penumbra Pivotal Stroke Trials) excluded children. There is a need to expand the literature on mechanical embolectomy in large vessel pediatric arterial ischemic stroke. This paper reports the use of two mechanical embolectomy devices cleared by the Federal Drug Administration (FDA) in four consecutive cases.

Methods

Our pediatric stroke database from 2002 to the present was reviewed retrospectively. Patients were included if they were diagnosed with an acute large vessel occlusion, were <18 years of age and underwent recanalization with a device cleared by the FDA. Clinical and radiographic results were abstracted from medical record review. The Pediatric National Institutes of Health Stroke Scale (PedNIHSS) score at presentation and at discharge and a pediatric-modified Rankin Scale (Ped-mRS) at approximately 90 days were scored retrospectively based on documented examinations.

Results

Four patients aged 4-17 years with a PedNIHSS score at presentation ranging from 2 to 17 points underwent mechanical embolectomy for reperfusion of the basilar artery (n=3), M1 segment of the right middle cerebral artery (n=1) and right internal carotid artery terminus (n=1). Thrombolysis in cerebral infarction (TICI) grade 3 was achieved in four vessels and TICI grade 2A was achieved in one vessel; there was one asymptomatic intraparenchymal hemorrhage. Intra-arterial tissue plasminogen activator was administered in two vessels. The PedNIHSS score at discharge ranged from 0 to 16 points and the Ped-mRS score at approximately 90 days ranged from 0 to 3 with 75% achieving a Ped-mRS score of ≤2.

Conclusion

Mechanical embolectomy using the Merci and Penumbra systems may be a feasible therapeutic option in the treatment of large vessel pediatric arterial ischemic stroke.

Background

The treatment of dolichoectatic basilar trunk aneurysms has been ineffectual or morbid due to nonsaccular morphology, deep location, and involvement of brainstem perforators. Treatment with bypass surgery has been advocated to eliminate malignant hemodynamics and to stabilize aneurysm growth.

Objective

To validate that flow alteration with bypass and parent artery occlusion favorably impacts aneurysm progression.

Methods

Surgical management evolved in 3 phases, each with different hemodynamic alterations.

Results

During a 17-year period, 37 patients with dolichoectatic basilar trunk aneurysms were retrospectively identified, of whom 21 patients were observed, 12 treated immediately, and 4 selected for treatment after clinical progression. In phase 1, flow reversal was overly thrombogenic, despite heparin (N = 5, final mortality, 100%). In phase 2, flow reduction with intracranial-to-intracranial bypass was safer than flow reversal, but did not prevent progressive aneurysm enlargement (N = 3, final mortality 67%). In phase 3, distal clip occlusion of the basilar trunk aneurysm preserved anterograde flow in the aneurysm without rupture, but reduced flow threatened perforator patency, despite treatment with clopidogrel (N = 8, final mortality 62%).

Conclusion

Shifting treatment strategy for dolichoectatic basilar trunk aneurysms improved surgical (80% to 50%) and final mortalities (100% to 62%), with stabilization of aneurysms in the phase 3 survivors. Good outcomes are determined by perforator preservation and mitigating aneurysm thrombosis. Occlusion techniques with increased distal run-off seem to benefit perforators. The treatment of dolichoectatic basilar trunk aneurysms can advance through concentrated management in dedicated centers, concerted efforts to study morphology and hemodynamics with computational methods, and widespread collection of registry data.

Abbreviations

4D PC-MRI, time-resolved phase-contrast MRIAICA, anterior inferior cerebellar arteryCE-MRA, high-resolution contrast-enhanced MR angiographyEC-IC, extracranial-to-intracranial bypassMCA, middle cerebral arteryMR, magnetic resonancemRS, modified Rankin ScalePCA, posterior cerebral arteryPICA, posterior inferior cerebellar arterySCA, superior cerebellar arterySTA, superficial temporal arteryVA, vertebral artery.

Objective

To describe a pediatric stroke syndrome with chronic focal vertebral arteriopathy adjacent to cervical abnormalities.

Methods

At a single pediatric stroke center, we identified consecutive children with stroke and vertebral arteriopathy of the V3 segment with adjacent cervical bony or soft tissue abnormalities. We abstracted clinical presentation, treatment, and follow-up data from medical charts.

Results

From 2005 to 2019, 10 children (all boys, ages 6-16 years) presented with posterior circulation strokes and vertebral arteriopathy with adjacent cervical pathology. Two children had bony abnormalities: one had a congenital arcuate foramen and one had os odontoideum with cervical instability. In children without bony pathology, vertebral artery narrowing during contralateral head rotation was visualized by digital subtraction angiography. Eight boys had recurrent ischemic events despite anti-thrombotic treatment (including 5 with multiple recurrences) and were treated surgically to prevent additional stroke. Procedures included vertebral artery decompression (n = 6), endovascular stent and spinal fusion (n = 1), and vertebral artery endovascular occlusion (n = 1). In boys treated with decompression, cervical soft tissue abnormalities (ruptured atlantoaxial bursa, ruptured joint capsule, or connective tissue scarring) were directly visualized during open surgery. No other etiology for stroke or dissection was found in any of the cases. Two boys without recurrent stroke were treated with activity restriction and antithrombotics. At a median follow-up of 51 months (range 17-84), there have been no additional recurrences.

Conclusions

Children with V3 segmental vertebral arteriopathy frequently have stroke recurrence despite antithrombotics. Cervical bone imaging and angiography with neck rotation can identify underlying pathology.

Background

The authors recently reported a series of children with vertebral artery (VA) compression during head turning who presented with recurrent posterior circulation stroke. Whether VA compression occurs during head positioning for cranial surgery is unknown.

Observations

The authors report a case of a child with incidental rotational occlusion of the VA observed during surgical head positioning for treatment of an intracranial arteriovenous fistula. Intraoperative angiography showed dynamic V₃ occlusion at the level of C2 with distal reconstitution via a muscular branch "jump" collateral, supplying reduced flow to the V₄ segment. She had no clinical history or imaging suggesting acute or prior stroke. Sequential postoperative magnetic resonance imaging scans demonstrated signal abnormality of the left rectus capitus muscle, suggesting ischemic edema.

Lessons

This report demonstrates that rotational VA compression during neurosurgical head positioning can occur in children but may be asymptomatic due to the presence of muscular VA-VA "jump" collaterals and contralateral VA flow. Although unilateral VA compression may be tolerated by children with codominant VAs, diligence when rotating the head away from a dominant VA is prudent during patient positioning to avoid posterior circulation ischemia or thromboembolism.

Large, wide-necked basilar apex aneurysms are difficult to treat. Microsurgical clipping can result in neurologic morbidity and mortality. Endovascular treatment often leaves remnants that need retreatment and/or stent placement with dual antiplatelet therapy. The Woven EndoBridge (WEB) is an intrasaccular flow disruption device that can be used without dual antiplatelet therapy. However, the WEB cannot typically be used in large or giant aneurysms > 10 mm because the largest diameter device is the 11 × 9.6 mm single layer sphere (SLS). We present a case in which we use a PulseRider aneurysm neck reconstruction device in the basilar artery to assist in WEB deployment within a 22 mm basilar apex aneurysm with 14 mm neck, thereby permitting aspirin monotherapy postoperatively.

OBJECT Large arteriovenous malformations (AVMs) remain difficult to treat, and ideal treatment parameters for volume-staged stereotactic radiosurgery (VS-SRS) are still unknown. The object of this study was to compare VS-SRS treatment outcomes for AVMs larger than 10 ml during 2 eras; Era 1 was 1992-March 2004, and Era 2 was May 2004-2008. In Era 2 the authors prospectively decreased the AVM treatment volume, increased the radiation dose per stage, and shortened the interval between stages. METHODS All cases of VS-SRS treatment for AVM performed at a single institution were retrospectively reviewed. RESULTS Of 69 patients intended for VS-SRS, 63 completed all stages. The median patient age at the first stage of VS-SRS was 34 years (range 9-68 years). The median modified radiosurgery-based AVM score (mRBAS), total AVM volume, and volume per stage in Era 1 versus Era 2 were 3.6 versus 2.7, 27.3 ml versus 18.9 ml, and 15.0 ml versus 6.8 ml, respectively. The median radiation dose per stage was 15.5 Gy in Era 1 and 17.0 Gy in Era 2, and the median clinical follow-up period in living patients was 8.6 years in Era 1 and 4.8 years in Era 2. All outcomes were measured from the first stage of VS-SRS. Near or complete obliteration was more common in Era 2 (log-rank test, p = 0.0003), with 3- and 5-year probabilities of 5% and 21%, respectively, in Era 1 compared with 24% and 68% in Era 2. Radiosurgical dose, AVM volume per stage, total AVM volume, era, compact nidus, Spetzler-Martin grade, and mRBAS were significantly associated with near or complete obliteration on univariate analysis. Dose was a strong predictor of response (Cox proportional hazards, p < 0.001, HR 6.99), with 3- and 5-year probabilities of near or complete obliteration of 5% and 16%, respectively, at a dose < 17 Gy versus 23% and 74% at a dose ≥ 17 Gy. Dose per stage, compact nidus, and total AVM volume remained significant predictors of near or complete obliteration on multivariate analysis. Seventeen patients (25%) had salvage surgery, SRS, and/or embolization. Allowing for salvage therapy, the probability of cure was more common in Era 2 (log-rank test, p = 0.0007) with 5-year probabilities of 0% in Era 1 versus 41% in Era 2. The strong trend toward improved cure in Era 2 persisted on multivariate analysis even when considering mRBAS (Cox proportional hazards, p = 0.055, HR 4.01, 95% CI 0.97-16.59). The complication rate was 29% in Era 1 compared with 13% in Era 2 (Cox proportional hazards, not significant). CONCLUSIONS VS-SRS is an option to obliterate or downsize large AVMs. Decreasing the AVM treatment volume per stage to ≤ 8 ml with this technique allowed a higher dose per fraction and decreased time to response, as well as improved rates of near obliteration and cure without increasing complications. Reducing the volume of these very large lesions can facilitate a surgical approach for cure.