Feasibility of intra-arterial chemotherapy for retinoblastoma: experiences in a large single center cohort study.

PURPOSE: In the last 10 years, intra-arterial chemotherapy (IAC) has been increasingly used in the clinical management of retinoblastoma. It is reported to provide tumor control even in advanced stage disease that might have previously required enucleation. In our clinical experience, there are three conditions that may impair the use of IAC: (1) significant collaterals to meningeal arteries, (2) technical failure of ophthalmic artery catheterization, or (3) retina blood supply from collaterals different to the ophthalmic artery. In the current study, we assessed the rate of IACs that could not be carried out in our institution due to these three reasons. METHODS: All patients admitted for IAC in our hospital were retrospectively assessed by chart review. Non-application rate of IAC was assessed and classified according to the three abovementioned criteria. Complication rate of both finalized and interrupted interventions was recorded. RESULTS: Ninety-eight patients (median age 21.4 months, range 5.3 months-10.5 years) were identified. IAC was performed in 69 (70.4%) patients and interrupted in 12 (12.2%) cases because of meningeal collaterals, in 8 (8.2%) because of technical failure to cannulate the ophthalmic artery, and in 9 (9.2%) because of alternative blood supply of the retina. CONCLUSION: The rather defensive approach that is pursued in our center resulted in an overall non-application rate of IAC around 30%. The relatively high probability of conditions that impair the use of IAC needs to be addressed adequately in the patient conversation prior to the procedure. Our rate of 8% of abstention from IAC due to technical limitations might be reduced by the application of more rigorous therapeutic approaches such as balloon occlusion of the distal internal carotid artery. More research is finally needed to determine if IAC can be safely performed in the presence of meningeal collaterals and via branches of the external carotid artery.

The Role of Whole-Body Magnetic Resonance Imaging (WB-MRI) in Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH).

In PNH thromboembolic events (TEs) represent the leading cause of morbidity and mortality. Between Dec 2013 and Jan 2016 37 PNH patients (pts) (23 PNH, 14 AA/PNH; 51% (19/37) females; median age 44 years, median D-dimer levels 0.22 mg/l) were examined with a whole-body magnetic resonance imaging (WB-MRI) scan at 1.5 T to detect TEs. Pts were treated according to German PNH guidelines, including eculizumab therapy. 64% (24/37) of the pts had no documented TEs prior to observation. Two pts had suspected TEs in their clinical history. 29% of the pts (11/37) had a known history of venous thromboses (deep venous thrombosis (DVT) (5/11), portal venous thrombosis (PVT) (4/11), vena caval thrombosis (VCT) (2/11). A myocardial infarction was reported in one pt, and two had a cerebral venous sinus thrombosis (CVST) or a thalamic infarction. Six pts (16%) had at least two prior TEs. In pts with prior TEs no progression of the existing TEs was observed. In pts on eculizumab and prior TEs as well as treatment-naïve pts silent bone and renal infarctions were detected. Furthermore, a clinically non-critical arterial occlusion was identified. WB-MRI scans present a novel, non-invasive method to assess the complete vascular status of PNH pts and allow the detection of previously undiagnosed vascular complications, affecting treatment indications and regimens.