Fergus Robertson

Efficacy and Complications of Super-selective Intra-ophthalmic Artery Melphalan for the Treatment of Refractory Retinoblastoma

PURPOSE To report the efficacy of super-selective intra-ophthalmic artery melphalan (IAM) for the treatment of refractory retinoblastoma and any associated complications of this treatment. DESIGN A prospective case series. PARTICIPANTS Eyes with retinoblastoma that had been treated with systemic chemotherapy or local therapy and had a relapse of their condition. METHODS All patients receiving IAM between May 2009 and September 2010 were included in the study. Intra-ophthalmic artery melphalan was offered to patients who had failed to respond adequately to systemic chemotherapy and local treatment where appropriate or because of a new recurrence of retinoblastoma that could not be treated with local therapies. None of the patients were excluded because of central nervous system abnormalities. Patients received 2 treatments of IAM given 4 weeks apart. All patients received an orthoptic assessment 3 weeks after each treatment and an examination under anesthesia (EUA). A third treatment was given if an unsatisfactory response was observed on EUA after 2 treatments. MAIN OUTCOME MEASURES The response of the retinoblastoma tumor(s) and any associated local side effects from the treatment. RESULTS A total of 15 eyes in 14 patients were treated with IAM during the study period. The mean age at the time of IAM was 31.5 months (median 17.3, range 11.2-150.7 months), and the mean follow-up was 8.7 months (3-16.3 months). Tumor control was achieved in 12 eyes (80%), and 12 eyes (80%) had local side effects that included third cranial nerve palsy in 6 (40%), orbital edema in 3 (20%), permanent retinal detachment in 1 (7%), and vitreous hemorrhage in 4 (27%). Seven eyes (47%) developed significant retinal pigment epithelium changes. CONCLUSIONS Intra-ophthalmic artery melphalan is an effective treatment for retinoblastoma, achieving a high level of remission in refractory tumors. It can be associated with significant local side effects that can result in loss of vision and possible amblyogenesis. Clinicians and parents need to consider the benefits and potential local side effects before embarking on treatment.

Preoperative Particle and Glue Embolization of Meningiomas: Indications, Results and Lessons Learned from 117 Consecutive Patients.

BACKGROUND: Preoperative embolization of meningiomas remains contentious, with persisting uncertainty over the safety and efficacy of this adjunctive technique. OBJECTIVE: To evaluate the safety of presurgical embolization of meningiomas and its impact on subsequent transfusion requirement with respect to the extent of embolization and technique used. METHODS: One hundred seventeen consecutive patients between 2001 and 2010 were referred for embolization of presumed intracranial meningioma before surgical resection. Glue and/or particles were used to devascularize the tumor in 107 patients, all of whom went on to operative resection. The extent and nature of embolization-related complications, degree of angiographic devascularization, and the intraoperative blood transfusion requirements were analyzed. RESULTS: Mean blood transfusion requirement during surgery was 0.8 units per case (range, 1-14 units). Blood transfusion was significantly lower in patients whose meningiomas were completely, angiographically devascularized (P = .035). Four patients had complications as a direct result of the embolization procedure. These included intratumoral hemorrhage in 2, sixth cranial nerve palsy in 1, and scalp necrosis requiring reconstructive surgery in 1 patient. CONCLUSION: The complication rate was 3.7%. No relationship between the embolic agent and the degree of devascularization was observed. Achieving a complete devascularization resulted in a lower blood transfusion requirement, considered an indirect measure of operative blood loss. This series demonstrates that preoperative meningioma embolization is safe and may reduce operative blood loss. We present distal intratumoral injection of liquid embolic as a safe and effective alternative to more established particle embolization techniques.

Spinal Dural Arteriovenous Fistulas: Clinical Experience with Endovascular Treatment as a Primary Therapy at 2 Academic Referral Centers

BACKGROUND AND PURPOSE: Spinal dural arteriovenous fistulas are a rare entity that, if left untreated, can lead to considerable morbidity with progressive spinal cord symptoms. The aim of this study was to evaluate the clinical outcome of patients with spinal dural arteriovenous fistulas that were primarily treated with endovascular embolization. MATERIALS AND METHODS: A retrospective review was performed of all patients from 1997–2010 who underwent treatment at 2 academic referral centers for a spinal dural arteriovenous fistula. Follow-up was performed by clinical examination, and functional status was measured by use of the Aminoff-Logue Disability Scale, McCormick classification grading, and mRS scores. The nonparametric Wilcoxon signed rank test was used to compare pretreatment and posttreatment Aminoff-Logue Disability Scale gait and micturition scores, McCormick classification grading, and mRS scores. P values < .05 were considered significant. RESULTS: A total of 38 patients were included. Five patients (2 endovascular, 3 surgical) were lost to follow-up and therefore were excluded from the analysis, 29 patients were initially treated from an endovascular approach (9 Onyx, 20 cyanoacrylate), and 4 patients were treated from a standard surgical approach. Five patients in the endovascular group subsequently underwent surgery for various reasons. The clinical improvements in the Aminoff-Logue Disability Scale gait and micturition scores, McCormick classification grading, and the mRS scores were statistically significant (P < .05, Wilcoxon signed rank test). CONCLUSIONS: We conclude that endovascular treatment of spinal dural arteriovenous fistulas can result in good clinical outcomes. Surgery remains the treatment of choice when safe embolization of the proximal radicular draining vein cannot be obtained or because the shunting artery of the spinal dural arteriovenous fistula also supplies the anterior spinal, posterior spinal, or a radiculomedullary artery.

Pediatric Cerebral Aneurysms

Childhood intracranial aneurysms differ from those in the adult population in incidence and gender prevalence, cause, location, and clinical presentation. Endovascular treatment of pediatric aneurysms is the suggested approach because it offers both reconstructive and deconstructive techniques and a better clinical outcome compared with surgery; however, the long-term durability of endovascular treatment is still questionable, therefore long-term clinical and imaging follow-up is necessary. The clinical presentation, diagnosis, and treatment of intracranial aneurysms in children are discussed, and data from endovascular treatments are presented.

Autonomic cardio‐respiratory reflex reactions and superselective ophthalmic arterial chemotherapy for retinoblastoma

To describe our experience with superselective ophthalmic artery chemotherapy (SOAC) in retinoblastoma and to report the serious adverse cardio‐respiratory reactions we have observed.

Stability of ruptured intracranial aneurysms treated with detachable coils: is delayed follow-up angiography warranted?

The optimal strategy for monitoring the stability of ruptured intracranial aneurysms following coil embolisation is unclear. The value of delayed follow-up angiography in detecting new recurrences or progression of residual lesions visualised on earlier angiographic studies was determined in the light of the increasing use of non-invasive imaging techniques such as time of flight magnetic resonance angiography (TOF-MRA) for the evaluation of intracranial aneurysm occlusion. Ninety-seven patients with 105 ruptured aneurysms treated with detachable coils in 2005 and 2006 were included. The presence of a residual neck or aneurysm was assessed on catheter angiograms performed at 6 months and 2 years using the Raymond criteria (Class I = completely occluded, class II = small residual neck, class III = aneurysm sac filling). At 6-month follow-up, 32% of class I aneurysms progressed to class II and 6% of these aneurysms required re-treatment. A further 2-year angiogram was obtained in 59 patients with 65 aneurysms. Ninety-six per cent of class I, 100% of the class II and class III aneurysms remained unchanged at 2 years compared to 6 months. In our series, most recurrences were apparent at 6-month follow-up. The vast majority of coiled ruptured aneurysms that were class I or II at 6 months remained stable at 2-year follow-up. In the absence of a residual lesion in the early angiographic study, a further delayed catheter angiogram may not be warranted. The use of non-invasive strategies such as TOF-MRA should be considered.

Screening for Cerebrovascular Disease in Microcephalic Osteodysplastic Primordial Dwarfism Type II (MOPD II): An Evidence-Based Proposal

Microcephalic osteodysplastic primordial dwarfism type II (OMIM 210720) is a rare autosomal recessive condition frequently associated with early-onset cerebrovascular disease. Presymptomatic detection and intervention could prevent the adverse consequences associated with this. We reviewed published cases of microcephalic osteodysplastic primordial dwarfism type II to ascertain prevalence and characteristics of cerebrovascular disease and use these data to propose an evidence-based approach to cerebrovascular screening. Of 147 cases identified, 47 had cerebrovascular disease (32%), including occlusive arteriopathy (including moyamoya) and cerebral aneurysmal disease. Occlusive disease occurred in younger individuals, and progression can be both rapid and clinically silent. A reasonable screening approach would be magnetic resonance imaging and angiography of the cervical and intracranial circulation at diagnosis, repeated at yearly intervals until 10 years, and every 2 years thereafter, unless clinical concerns occur earlier. At present it would appear that this needs to be life-long. Families and professionals should be alerted to the potential significance of neurologic symptoms and measures should be taken to maintain good vascular health in affected individuals.

An automated multiscale ensemble simulation approach for vascular blood flow

This work has received funding from the CRESTA project within the EC-FP7 (ICT-2011.9.13) under Grant Agreements no. 287703, and from EPSRC Grants EP/I017909/1 (www.2020science.net) and EP/I034602/1.

Early single centre experience of flow diverting stents for the treatment of cerebral aneurysms.

Abstract Introduction. The flow diverting stent (FDS) is a relatively new endovascular therapeutic tool specifically designed to reconstruct the parent artery and divert blood flow along the normal anatomical course and away from the aneurysm neck and dome. Methods. Retrospective review of prospectively built clinical and imaging database of patients treated with FDS at the National Hospital for Neurology and Neurosurgery, Queen Square, London, UK was done. Results. Between 18/03/2008 and 10/11/2011, 80 patients underwent 84 FDS insertion procedures for various indications. Mean duration of clinical follow-up was 11.3 ± 9.3 months and of imaging follow-up was 10.6 ± 9.3 months. Sixty-seven had anterior circulation aneurysms while 17 had posterior circulation aneurysms. Seven (8.3%) patients died (two probably not related, giving a procedure-related mortality of 5.9%), eight had permanent new deficit (9.5%), 20 had transient deficit (23%) and 49 (58%) had no complications. There was a trend towards bad outcome with larger posterior circulation aneurysms. Angiographic follow-up showed 38% cure rate at 6 months and 61% at 12 months. Conclusion. FDS should only be used following multidisciplinary discussion in selected patients. Further data is required regarding long-term safety, efficacy and indications.

Idiopathic intracranial hypertension: 120-day clinical, radiological, and manometric outcomes after stent insertion into the dural venous sinus

OBJECTIVE Idiopathic intracranial hypertension (IIH) is commonly associated with venous sinus stenosis. In recent years, transvenous dural venous sinus stent (DVSS) insertion has emerged as a potential therapy for resistant cases. However, there remains considerable uncertainty over the safety and efficacy of this procedure, in particular the incidence of intraprocedural and delayed complications and in the longevity of sinus patency, pressure gradient obliteration, and therapeutic clinical outcome. The aim of this study was to determine clinical, radiological, and manometric outcomes at 3-4 months after DVSS in this treated IIH cohort. METHODS Clinical, radiographic, and manometric data before and 3-4 months after DVSS were reviewed in this single-center case series. All venographic and manometric procedures were performed under local anesthesia with the patient supine. RESULTS Forty-one patients underwent DVSS venography/manometry within 120 days. Sinus pressure reduction of between 11 and 15 mm Hg was achieved 3-4 months after DVSS compared with pre-stent baseline, regardless of whether the procedure was primary or secondary (after shunt surgery). Radiographic obliteration of anatomical stenosis correlating with reduction in pressure gradients was observed. The complication rate after DVSS was 4.9% and stent survival was 87.8% at 120 days. At least 20% of patients developed restenosis following DVSS and only 63.3% demonstrated an improvement or resolution of papilledema. CONCLUSIONS Reduced venous sinus pressures were observed at 120 days after the procedure. DVSS showed lower complication rates than shunts, but the clinical outcome data were less convincing. To definitively compare the outcomes between DVSS and shunts in IIH, a randomized prospective study is needed.