Transorbital anastomotic pathways between the external and internal carotid systems in children affected by intraocular retinoblastoma.

PURPOSE: It is well known that many anastomoses can join the external carotid system and the ophthalmic artery. However, their frequency has never been reported. Since they can be relevant for interventional radiologists operating in the orbit, we decided to illustrate and determine the frequency of the anastomoses that can be found in children. METHODS: A retrospective study of 443 angiographic procedures (via ophthalmic artery and/or external carotid artery) carried out on 97 children affected by intraocular retinoblastoma was made to investigate the arterial anatomy of 106 orbits. RESULTS: Anastomoses were observed in 44.33 % of orbits. However, their true frequency is likely much higher as the rate of visualization increased up to 91.11 % of orbits when the angiographic study was extended to the external carotid artery. In order of frequency we detected the following anastomoses: lacrimal artery-middle meningeal artery, lacrimal artery-anterior deep temporal artery, ophthalmic artery-middle meningeal artery, ophthalmic artery-facial artery, supraorbital artery-superficial temporal artery, supratrochlear artery-superficial temporal artery, supraorbital artery-middle meningeal artery, dorsal nasal artery-infraorbital artery, supraorbital artery-zygomaticoorbital artery, lacrimal artery-zygomaticoorbital artery. CONCLUSION: When properly searched, anastomoses between the ophthalmic artery and the external carotid artery are almost constant in children. Depending on the clinical scenario, they can represent dangers or valuable alternative routes for collateral circulations and intraarterial chemotherapy.

Identification of Intraorbital Arteries in Pediatric Age by High Resolution Superselective Angiography.

PURPOSE: Angiography is a powerful tool to identify intraorbital arteries. However, the incidence by which these vessels can be identified is unknown. Our purpose was to determine such incidence and which angiographic approach is best for the identification of each artery. METHODS: A retrospective study of 353 angiographic procedures (via ophthalmic artery and/or external carotid artery) carried out on 79 children affected by intraocular retinoblastoma was made to investigate the arterial anatomy in 87 orbits. For each intraorbital artery two parameters were calculated: the angiographic incidence, as the percentage of times a given artery was identified, and the visibility index, as the ratio between the angiographic incidence and the true anatomic incidence. RESULTS: All collaterals of the ophthalmic artery could be spotted. Most of them were identified with a high angiographic incidence; some of them were less easily identified because too thin or because frequently shielded. The visibility index paralleled the angiographic incidence of most arteries. However, the lacrimal and meningolacrimal arteries had a higher visibility index suggesting that their identification was more frequent than the angiographic incidence alone could suggest. Statistical analysis demonstrated that the lacrimal artery and some muscular branches had higher chances to be identified if the angiography of the ophthalmic artery was accompanied by the study of the external carotid system. CONCLUSION: This work provides an objective measure of how powerful angiography is to identify intraorbital arteries as well as useful references for professionals who need to operate in the orbit.

Superselective ophthalmic artery infusion of melphalan for intraocular retinoblastoma: preliminary results from 140 treatments.

PURPOSE: To report our experience in superselective ophthalmic artery infusion of melphalan (SOAIM) for intraocular retinoblastoma. METHODS: From June 2008 to October 2010, 38 patients (18 women, 20 men; age range at first treatment, 7 months to 22 years) with 41 eyes with retinoblastoma were scheduled for SOAIM, for 17 newly diagnosed retinoblastomas Tumour, Node and Metastasis (TNM) 7th Edition 1a (n = 1), 1b (n = 1), 2a (n = 7), 2b (n = 4) and 3a (n = 4) and 24 retinoblastomas with partial remission/relapse TNM 7th Edition 1b (n = 13), 2a (n = 1) and 2b (n = 10). Eight patients (ten eyes) have been treated by SOAIM alone. Follow-up was 6-27 months in 28 patients (30 eyes). RESULTS: Ophthalmic artery cannulation failed in two patients. Thirty-six patients underwent 140 treatments by internal (n = 112) or external (n = 28) carotid arteries. No major procedural complications occurred. Two patients have been lost to follow-up. Remaining 34 patients (37 eyes) had no metastatic disease. Four patients suffered permanent ocular complications: chorioretinal dystrophy (n = 2), ptosis (n = 1) and strabismus/exotropia (n = 1). Eight (22%) eyes in eight (24%) patients underwent enucleation: 7/16 (43%) newly diagnosed retinoblastomas and 1/22 (4.5%) retinoblastomas undergoing partial remission/relapse. For all treated eyes, Kaplan-Meier eye enucleation-free rates (K-M) were 85.4% (95% CI, 73.3-97.5%), 74.4% (95% CI, 57-91.8%) and still stable at 6, 12 months and 2 years, respectively. For eyes with partial remission/relapse, and eyes at presentation, K-M at 2 years were 95.5% (95% CI, 86.9-100%) and 45.6% (95% CI, 16.6-74.6%), respectively. CONCLUSION: Superselective ophthalmic artery infusion of melphalan was safe and powerful, especially following other therapies. Superselective ophthalmic artery infusion of melphalan should be added to focal therapies spectrum. In selected cases, melphalan should be combined with other chemotherapeutic agents.

Cerebral neoplastic enhancing lesions: multicenter, randomized, crossover intraindividual comparison between gadobutrol (1.0M) and gadoterate meglumine (0.5M) at 0.1 mmol Gd/kg body weight in a clinical setting.

OBJECTIVE: Two macrocyclic extracellular contrast agents, one-molar neutral gadobutrol and ionic gadoterate meglumine, were compared to determine the overall preference for one or the other in a clinical setting. MATERIALS AND METHODS: Multicenter, randomized, single-blind, intra-individually controlled, comparison study with a corresponding blinded read. Efficacy analysis was based on 136 patients who underwent identical MRI examinations: group A first received 1.0M gadobutrol followed by 0.5M gadoterate meglumine 48 h to 7 days later; group B had a reversed administration order. Three independent blinded readers assessed off-site their overall diagnostic preference (primary efficacy parameter) based on a matched pairs approach. RESULTS: Superiority of gadobutrol over gadoterate meglumine was demonstrated for the qualitative assessment of overall preference across all readers by a statistically significant difference between both contrast agents for this primary endpoint. Preferences in lesion enhancement (secondary endpoint) were also found significantly in favor of gadobutrol. For preference in lesion delineation from surrounding tissue/edema and for internal structure only a trend towards a higher proportion for gadobutrol was found (except for internal structure reported by one reader, which showed a result of statistical significance). Lesion contrast and relative lesion enhancement (quantitative parameters) were statistically significantly higher for gadobutrol compared to gadoterate meglumine. CONCLUSION: Contrast-enhanced MRI of neoplastic brain lesions at a dose of 0.1 mmol Gd/kg body weight, assessed in a standardized off-site blinded reading, results in a significantly higher qualitative and quantitative preference for gadobutrol compared to gadoterate meglumine.

Quantitative ColourDopplerSonography evaluation of cerebral venous outflow: a comparative study between patients with multiple sclerosis and controls.

BACKGROUND: Internal Jugular Veins (IJVs) are the principle outflow pathway for intracranial blood in clinostatism condition. In the seated position, IJVs collapse, while Vertebral Veins (VVs) increase the venous outflow and partially compensate the venous drainage. Spinal Epidural Veins are an additional drainage pathway in the seated position. Colour- Doppler-Sonography (CDS) examination is able to demonstrate IJVs and VVs outflow in different postural and respiratory conditions. The purpose of this study was to evaluate CDS quantification of the cerebral venous outflow (CVF) in healthy subjects and patients with multiple sclerosis (MS). METHODOLOGY/PRINCIPAL FINDINGS: In a group of 27 healthy adults (13 females and 14 males; mean age 37.8 ± 11.2 years), and 52 patients with MS (32 females and 20 males; mean age 42.6 ± 12.1 years), CVF has been measured in clinostatism and in the seated position as the sum of the flow in IJVs and VVs. The difference between CVF in clinostatism and CVF in the seated position (ΔCVF) has been correlated with patients' status (healthy or MS), and a number of clinical variables in MS patients. Statistical analysis was performed by Fisher's exact test, non-parametric Mann-Whitney U test, ANOVA Kruskal-Wallis test, and correntropy coefficient. The value of ΔCVF was negative in 59.6% of patients with MS and positive in 96.3% of healthy subjects. Negative ΔCVF values were significantly associated with MS (p<0.0001). There was no significant correlation with clinical variables. CONCLUSIONS/SIGNIFICANCE: Negative ΔCVF has a hemodynamic significance, since it reflects an increased venous return in the seated position. This seems to be a pathologic condition. In MS patients, a vascular dysregulation resulting from involvement of the autonomous nervous system may be supposed. ΔCVF value should be included in the quantitative CDS evaluation of the cerebral venous drainage, in order to identify cerebral venous return abnormalities.

Subcortical low-intensity and restricted diffusion after first seizure in a child.

Our purpose was to increase the knowledge about subcortical low-intensity images on long repetition time by describing brain magnetic resonance images of a young boy after his first spontaneous seizure. Evident in the epileptogenic area were transient images of reversible subcortical low intensity on long-repetition time, T2*-weighted, and b = 0 s/mm2 diffusion, isointense signal on b = 1000 s/mm2 diffusion, and restricted diffusion. Most likely, mechanism was axonal flow disruption with iron accumulation and free radicals production induced during seizure.

Lung cancer presenting with trigeminal neuropathy.

The purpose of this case report is to describe MR imaging and CT findings of a patient in whom the first clinical and neuroradiological manifestation of a metastatic lung adenocarcinoma was unilateral trigeminal neuropathy caused by a presumed metastasis involving cisternal and Meckel's cave segments of ipsilateral trigeminal nerve. MR imaging and CT scan differential diagnosis of expansive lesions of the intracranial trigeminal nerve must include metastases. Physicians and neuroradiologists must be aware of metastases as an uncommon cause of trigeminal neuropathy, even as the presenting condition of cancer.

Contrast-enhanced magnetic resonance imaging of fibrovascular tissue ingrowth within synthetic hydroxyapatite orbital implants in children.

PURPOSE: To assess the usefulness of magnetic resonance imaging (MRI) in assessing fibrovascularization progression into synthetic hydroxyapatite (HA) implants inserted in anophthalmic sockets of children submitted to enucleation. METHODS: We studied 23 HA orbital implants in 23 children who underwent enucleation for retinoblastoma. Each patient was examined by MRI within to 9 to 69 weeks after implant insertion (mean 34 weeks, median 30 weeks). No patient had received chemotherapy or radiotherapy at MRI examination. From each T1-weighted, fat-suppressed enhanced axial examination, the image depicting the center of the implant was identified. Enhancement was evaluated using a 5-point scale. Additionally, possible associated orbital and intracranial pathologies and implant migration or extrusion were evaluated. RESULTS: All patients showed areas of enhancement of the implant consistent with the presence of fibrovascular ingrowth. There was no grade 1 enhancement in our series. Grade 2 was observed in 1 patient (4.34%), grade 3 in 7 patients (30.43%), grade 4 in 11 patients (47.82%), and grade 5 in 4 patients (17.39%). During follow-up there were no cases of clinically evident orbital infection, implant migration, or implant extrusion. No second tumor, optic nerve invasion, orbital extension, tumor relapse, or leptomeningeal brain seeding were noted. CONCLUSIONS: In this series, enhanced MRI showed satisfactory fibrovascular ingrowth of orbital implants since the 13th week after HA spheres insertion, with a trend towards progressive enhancement during the following weeks. The data also confirm the advice to wait at least 5-6 months after enucleation to perform drilling and peg placement.

Autonomic activity and baroreflex sensitivity in patients submitted to carotid stenting.

Arterial baroreflex and cardiac autonomic control play important roles in hemodynamic instability after carotid artery stenting (CAS). Spontaneous baroreflex sensitivity (BRS), heart rate variability (HRV) and blood pressure variability (BPV) are established tools for the assessment of arterial baroreflex and cardiac autonomic activity. Aim of the study was to evaluate cardiac autonomic activity (by means of HRV, BPV and BRS) after CAS and to explore the impact of internal carotid artery stenosis on BRS changes after CAS. 37 patients (68±10.45 years) with internal carotid stenosis underwent CAS. HRV, BPV and BRS were measured in all subjects before and at 1 and 72h after CAS. ANOVA was performed to compare BRS, HRV and BPV parameters before and after CAS. Spearman analysis was performed to determine a possible correlation between carotid stenosis degree (or carotid plaque diameter) and BRS changes (ΔBRS). LF/HF (index of sympatho-vagal balance) decreased during postoperative period, in comparison with baseline (2.32±1.70 vs 1.65±1.40, p<0.05). There was a significant negative correlation between carotid stenosis degree and ΔBRS (r=-0.35, p=0.03) and between carotid plaques thickness and ΔBRS (r=-0.36, p=0.02). CAS procedure may cause an alteration of carotid wall mechanical properties, increasing baroreflex sensitivity. BRS does not increase in all the patients, because arterial wall damage and nerve destruction determined by atherosclerotic plaque may reduce ΔBRS.

Methadone-induced toxic leukoencephalopathy: diagnosis and follow-up by magnetic resonance imaging including diffusion-weighted imaging and apparent diffusion coefficient maps.

BACKGROUND AND PURPOSE: Effects of methadone misuse have been rarely described. The purpose of this case report is to increase the knowledge of methadone-related leukoencephalopathy. METHODS: We report the long-term follow-up by brain magnetic resonance imaging including isotropic diffusion-weighted imaging and mean apparent diffusion coefficient values of a 49-year-old patient who attempted suicide by intravenous methadone. RESULTS AND CONCLUSIONS: Lesion pattern included subtle cerebellar involvement, mainly reversible extensive bilateral and symmetric brain involvement, cystic degeneration in the periventricular regions, sparing of corpus callosum and subcortical U-fibers, development of diffuse brain atrophy, and clear-cut clinical improvement.

Lateral medullary ischemia presenting with persistent hiccups and vertigo.

This study describes a patient with lateral medullary ischemia (LMI) presenting with persistent hiccups followed by vertigo with horizontal head-shaking-induced contralesional nystagmus (HSN) and discusses pertinent pathophysiology. A 65-year-old man presented with persistent hiccups and disabling spells of vertigo, lasting 30 seconds that became much more frequent and associated with lateropulsion to the right. A strong left beating HSN was evident. Magnetic resonance imaging and angiography, and intra-arterial cerebral digital subtracted angiography showed subacute ischemic lesions in the right lateral medulla and ipsilateral inferior cerebellar hemisphere, and two tight stenoses of the V1 and V4 segments of the right vertebral artery. Patient was treated by intravenous heparin and oral clopidogrel. After 48 hours, hiccups disappeared. One month later, vertigo spells were less frequent but still disabling. Endovascular stenting of the right vertebral artery stenoses was then performed. In the subsequent four years, the patient had no further episodes of hiccups or vertigo. Less intense HSN persisted. Hiccups followed by vertigo, lateropulsion, and HSN had been the clinical presentation of LMI and cerebellar ischemia, without other major neurologic or ocular motor findings. This unusual clinical variant of LMI could mimic a more benign labyrinthine lesion, and possibly leading to a dangerously delayed treatment.

Cavernous malformation of the optic nerve mimicking optic neuritis.

A 30-year-old woman developed acute visual loss and optic disc elevation in the left eye after breastfeeding her second son. The initial diagnosis was optic neuritis. However, MRI showed a lesion in left intraorbital and intracanalicular optic nerve and several cerebral lesions with imaging features of cerebral cavernous malformations (CCMs). Genetic testing was positive for abnormalities known to predispose to CCMs in the patient and her father, who also showed MRI evidence of CCMs. During a 44-month follow-up period in which no intervention took place, the patient's vision in the affected eye fluctuated but eventually became extinguished. Serial MRIs did not always show lesion changes that explained the visual deterioration. In familial CCM, pregnancy might be a "second hit" to genetically predisposed tissue.

Regression of dilated perivascular spaces of the brain.

PURPOSE: Perivascular, or Virchow-Robin, spaces of the brain represent interstitial fluid-filled spaces continuous with subpial spaces, and not invagination of cerebrospinal fluid-filled subarachnoid spaces. Regression of a dilated, or even giant, perivascular space occurs rarely. The purpose of this paper is to describe magnetic resonance imaging evidence of complete regression of dilated perivascular spaces (dPVSs). METHODS: Patient 1 was a 76-year-old woman with right hemiparesis and aphasia from a left cranial vault meningioma infiltrating the superior sagittal sinus, and a left temporal lobe giant perivascular space. Patient 2 was a 70-year-old man with pituitary apoplexy, vasospasm, cerebral ischemia, and two dPVSs, one in the right temporal lobe, and one in the left anterior perforate substance. Patient 3 was a 78-year-old man with a generalized seizure, and a right temporal lobe dPVS. RESULTS: In all the patients, temporal lobe giant or dPVSs underwent regression, following meningioma subtotal resection (patient 1) or pituitary lesion shrinkage (patient 2), or spontaneously (patient 3). In patient 2, the left anterior perforate substance dPVS was unchanged. CONCLUSIONS: Temporal lobe giant or dPVSs may regress, eventually together with resection or regression of intracranial tumors, also distant from the PVSs. Cerebral edema does not seem the only factor influencing dilatation of PVSs.

Growth of congenital malignant teratoid medulloepithelioma of the ciliary body: a case study.

The purpose of this study was to describe the growth pattern of congenital malignant teratoid medulloepithelioma of the ciliary body by reporting clinical and imaging findings with pathological correlation. An 11-month-old little girl presented with a whitish-pink iris mass in the right eye resulting from a small ciliary body mass consistent with medulloepithelioma at both clinical and computed tomography (CT) findings. At CT, the lesion showed heterogeneous attenuation, without intraocular calcifications. Eleven months later, clinical and ultrabiomicroscopy showed a clear enlargement of the mass, which invaded the pupil. At magnetic resonance imaging (MRI), the lesion showed T1-weighted hyperintensity and T2-weighted slight hypointensity when compared to the vitreous and a notch in the anterolateral aspect of the ipsilateral lens. After intravenous gadolinium administration, the lesion showed intense homogeneous enhancement, and there was leakage of gadolinium in the anterior chamber, resulting from impairment of blood-aqueous barrier. Biopsy revealed a malignant teratoid medulloepithelioma. The eye was then enucleated, and histology confirmed the diagnosis. Systemic chemotherapy and radiotherapy were not performed, since there was no extraocular extension. The 57-month clinical and MRI follow-up did not show disease relapse. This uncommon case displays the natural history of congenital malignant teratoid medulloepithelioma of the ciliary body. While the tumour might have been successfully treated by local excision at diagnosis, the delay in surgical treatment led to tumour overgrowth with consequent need for enucleation. The most important prognostic feature is extraocular extension, which carries a risk of local recurrence, eventually resulting in intracranial extension and/or lymphatic spread.

Immediate and 30-day clinical outcome of patients treated with the TwinOne cerebral protection system: multicenter experience in 217 cases.

There are several cerebral protection devices used for carotid artery stenting (CAS). All of them require manipulation and exchange of two separate materials (cerebral protection device ? dilatation balloon), making the procedure longer and posing possible complications such as vasospasm and embolism. The new system described here is an evolution of the cerebral protection concept and allows temporary occlusion of the distal internal carotid during CAS procedures with both materials in one device. We present our experience using this new device. Between January 2007 and March 2008, consecutive patients sent for CAS (symptomatic, with stenosis C50%, or asymptomatic, with stenosis C70%) were treated using TwinOne as a cerebral protection system. All patients were treated using the "simplified" CAS technique, limiting cerebral protection to the poststenting angioplasty phase. Two hundred nine patients have been treated at our institutions; eight underwent bilateral CAS, for a total of 217 CAS procedures performed using the TwinOne for cerebral protection. There have been four periprocedural (within 3 h of procedure) cases of complications (1.8%): three disabling strokes (one in-stent thrombosis, one presumed clotting, one ipsilateral stroke with uncertain root cause) and one transient ischemic attack attributable to heart failure. There have been two in-hospital complications (0.92%): one disabling stroke of unclear origin and one contralateral transient ischemic attack. No additional adverse event has been reported at 30 days after the CAS procedure. In conclusion, this cerebral protection system is simple to use, allows a quick intervention and short occlusion time, and has a low rate of complications.

Absence of the right common carotid artery.

In a 33-year-old male patient with multiple sclerosis, brain magnetic resonance imaging incidentally showed a narrow right intracranial internal carotid artery. Gadolinium-enhanced magnetic resonance angiography showed absence of the right common carotid artery. Right external and internal carotid arteries arose separately from the ipsilateral innominate artery and subclavian artery, respectively. Color-Doppler ultrasound confirmed this finding. Embriologically, the case could be interpreted as the disappearance of the third aortic arch with persistence of the carotid duct connecting the third and fourth aortic arches.

Neuroradiological follow-up of the growth of papillary tumor of the pineal region: a case report.

Papillary tumor of the pineal region (PTPR) is a recently described distinct clinicopathological entity. The purpose of this case report is to increase the knowledge of its neuroradiological findings and natural history by describing the long-term clinical and neuroradiological follow-up of a PTPR occurring in a 56-year-old Italian male patient. At magnetic resonance imaging (MRI) obtained at diagnosis, the lesion showed a subtle high signal intensity on T1-weighted imaging. Twenty-nine months later, MRI showed clearcut enlargement of the lesion, which had only a small area of high signal intensity on T1-weighted images, and a minimum apparent diffusion coefficient of 0.854 x 10(-3) mm(2)/s. Treatment included surgery followed by irradiation. Three-month MRI follow-up did not show disease relapse.

Intracranial involvement in plasmacytomas and multiple myeloma: a pictorial essay.

INTRODUCTION: The purpose of this pictorial essay is to increase awareness of the clinical presentation, neuroradiological findings, treatment options, and neuroradiological follow-up of plasmacytomas and multiple myeloma with intracranial growth. METHODS: This pictorial essay reviews the clinical features and neuroradiological findings in seven patients (four women, three men; age range at diagnosis 62-82 years) followed in two institutions. Six patients, one with IgG-kappa plasmacytoma, and five with IgG-kappa (n = 3), IgG-lambda (n = 1), and nonsecretory (n = 1) multiple myeloma, had been seen over a period of 9 years in one institution, and the other patient with IgG-kappa plasmacytoma had been seen over a period of 3.5 years in the other. RESULTS: Intracranial involvement is rare, most frequently resulting from osseous lesions in the cranial vault, skull base, nose, or paranasal sinuses. Primary dural or leptomeningeal involvement is rarer. Some typical findings of a dural and/or osseous plasmacytoma include iso- to hyperdensity on CT scan, T1 equal to high signal intensity and T2 markedly hypointense signal on MRI, and high vascularity possibly documented on intraarterial digital subtraction angiography. However, the neuroradiological findings generally lack specificity, since they are generally no different from those of meningioma, metastasis, lymphoma, dural sarcoma, plasma cell granuloma, infectious meningitis, and leptomeningeal carcinomatosis. CONCLUSION: The spectrum of clinical and neuroradiological evaluation shows that intracranial involvement from plasmacytoma and multiple myeloma must be taken into account in the differential diagnosis of cranial osseous and meningeal disease.