Vertical Parasagittal Hemispherotomy in a Pediatric Case of Epilepsy Due to Rasmussen Encephalitis: 2-Dimensional Operative Video.

Hemispherotomy is an effective disconnection technique for the treatment of different forms of drug-resistant epilepsy due to encephalopathies with unilateral hemispheric involvement.1-8 We describe the case of a 6-year-old child affected by Rasmussen encephalitis who underwent right vertical parasagittal hemispherotomy.9 The goal of the procedure was to isolate the basal ganglia region by interrupting the interhemispheric and intrahemispheric white matter connectivity. The main surgical steps include the transcortical approach to the lateral ventricle, the posterior callosotomy, the fimbria-fornix incision, the laterothalamic vertical incision, the anterior callosotomy, the frontobasal disconnection, and the transcaudate lateral incision to the anterior temporal horn.7,10 At 10-month follow-up, the patient was seizure free with a stable left hemiparesis. The antiepileptic therapy was progressively interrupted. The video describes the main surgical steps, using both intraoperative videos and advanced three-dimensional modeling of neuroimaging pictures. Patient' parents consented to the procedure. The participants and any identifiable individuals consented to publication of his/her image. Approval from the ethics committee was acquired.

Vertical Hemispherotomy: Contribution of Advanced Three-Dimensional Modeling for Presurgical Planning and Training.

Vertical hemispherotomy is an effective treatment for many drug-resistant encephalopathies with unilateral involvement. One of the main factors influencing positive surgical results and long-term seizure freedom is the quality of disconnection. For this reason, perfect anatomical awareness is mandatory during each step of the procedure. Although previous groups attempted to reproduce the surgical anatomy through schematic representations, cadaveric dissections, and intraoperative photographs and videos, a comprehensive understanding of the approach may still be difficult, especially for less experienced neurosurgeons. In this work, we reported the application of advanced technology for three-dimensional (3D) modeling and visualization of the main neurova-scular structures during vertical hemispherotomy procedures. In the first part of the study, we built a detailed 3D model of the main structures and landmarks involved during each disconnection phase. In the second part, we discussed the adjunctive value of augmented reality systems for the management of the most challenging etiologies, such as hemimegalencephaly and post-ischemic encephalopathy. We demonstrated the contribution of advanced 3D modeling and visualization to enhance the quality of anatomical representation and interaction between the operator and model according to a surgical perspective, optimizing the quality of presurgical planning, intraoperative orientation, and educational training.

Calcium phosphate and titanium cranioplasty after total angular craniopagus separation.

Craniopagus separation requires careful planning and a multi-staged surgical approach, managed by a multidisciplinary team. Despite growing experience on surgical management, the long-term restoration of the cranial defect has been rarely discussed so far. Several alloplastic materials have been proposed as effective and safe solutions for cranioplasty even for pediatric patients, allowing for bone generation, good aesthetic results, and low complication rates. We report, for the first time, on a separated craniopagus child who underwent successful implant of a tailor-made system based on the combination of calcium phosphate ceramic reinforced with a titanium mesh.

Magnetic resonance imaging based neurosurgical planning on hololens 2: A feasibility study in a paediatric hospital.

Objective: The goal of this work is to show how to implement a mixed reality application (app) for neurosurgery planning based on neuroimaging data, highlighting the strengths and weaknesses of its design. Methods: Our workflow explains how to handle neuroimaging data, including how to load morphological, functional and diffusion tensor imaging data into a mixed reality environment, thus creating a first guide of this kind. Brain magnetic resonance imaging data from a paediatric patient were acquired using a 3 T Siemens Magnetom Skyra scanner. Initially, this raw data underwent specific software pre-processing and were subsequently transformed to ensure seamless integration with the mixed reality app. After that, we created three-dimensional models of brain structures and the mixed reality environment using Unity™ engine together with Microsoft® HoloLens 2™ device. To get an evaluation of the app we submitted a questionnaire to four neurosurgeons. To collect data concerning the performance of a user session we used Unity Performance Profiler. Results: The use of the interactive features, such as rotating, scaling and moving models and browsing through menus, provided by the app had high scores in the questionnaire, and their use can still be improved as suggested by the performance data collected. The questionnaire's average scores were high, so the overall experiences of using our mixed reality app were positive. Conclusion: We have successfully created a valuable and easy-to-use neuroimaging data mixed reality app, laying the foundation for more future clinical uses, as more models and data derived from various biomedical images can be imported.

Bleeding in patients with continuous-flow left ventricular assist devices: acquired von Willebrand disease or antithrombotics?

OBJECTIVES: To evaluate the competing pro-haemorrhagic contribution of acquired von Willebrand (vW) disease and antithrombotic therapy in patients implanted with continuous-flow left ventricular assist devices (LVADs). METHODS: We compared the extent of vW factor (vWf) degradation [vWf antigen (vWf:Ag)] and a decrease of functional activity of large vWf multimers [vWf collagen binding (vWf:CB)] in LVAD patients who did and did not suffer from bleeding. Data were measured pre-implant, at short-term (t1: <3 months) and long-term (t2: >12 months) follow-up. The occurrence of primary bleeding events, as well as bleeding recurrence, was correlated with patient-specific vWf profile and antithrombotic regimen. Indeed, patients were discharged on warfarin (international normalized ratio: 2-2.5) and aspirin, with the latter withhold after a first bleeding episode. RESULTS: Fifty-three patients were enrolled. The median follow-up was 324 (226-468) days. We recorded 25 primary bleeding events (47% of patients). All primary events occurred in patients on warfarin and aspirin. Both vWf:Ag and vWf:CB decreased significantly post-implant (P = 0.0003 and P < 0.0001), and patients showing pathological vWf:CB/vWf:Ag ratio (<0.7) increased progressively over the time of support (pre-implant = 26%, t1 = 58%, t2 = 74%; P < 0.0001). Of note, activity of large vWf multimers of bleeders was significantly lower at t2 with respect to non-bleeders (vWf:CB: 61 (36-115) vs 100 (68-121), P = 0.04; vWf:CB/vWf:Ag ratio: 0.36 (0.26-0.61) vs 0.58 (0.33-0.96), P = 0.04). Despite these marked differences in the vWf profile, following aspirin discontinuation only 3 patients had bleeding recurrence. CONCLUSIONS: Aspirin contributes significantly to haemorrhagic events in the background of acquired vW disease; its discontinuation significantly reduces bleeding recurrence. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov/ct2/show/NCT03255928; ClinicalTrials.gov Identifier: NCT03255928.

Successful staged separation of total angular craniopagus.

BACKGROUND: Total craniopagus is an exceedingly rare condition in which surgical treatment is complex and potentially fatal. Over the last decades, a multistep surgical approach, which allows development of venous collateral circulation, has fostered a dramatic improvement of successful separation rates and neurological outcomes. Most of the experience derives from management of vertical craniopagus, the angular form being rarer and less amenable to successful surgical separation. METHODS: We present a case of total angular craniopagus twins observed at our Institution. Specific features included a large occipital fusion area with a bone defect, complete separation of brain and arterial vessels and a complex configuration of dural venous sinuses. The superior sagittal sinus of each twin preferentially drained to a single transverse sinus through a shared torcular. RESULTS: After an extensive diagnostic phase, including neuroimaging, tridimensional and virtual reality modelling, neurological, neurophysiological and rehabilitation assessment, a detailed multistep surgical plan, was proposed to a wide multidisciplinary team. The venous system was managed by taking advantage of the fact each twin's superior sagittal sinus was drained preferentially by the transverse sinus on the twin's left. The transverse sinuses were thus separated accordingly. CONCLUSIONS: Successful separation was achieved in three surgical steps over one year, with an excellent outcome for both twins.

Surgical management of pediatric intracranial CCM: a 10-year single center experience.

BACKGROUND: Cerebral cavernous malformations (CCMs) are low-flow vascular malformations made up of dilated vascular spaces without intervening parenchyma that can occur throughout the central nervous system. CCMs can occur sporadically or in familial forms. Presentation is diverse, ranging from asymptomatic discoveries to drug-resistant epilepsy and hemorrhages. METHODS: We describe the surgical management of CCMs in pediatric patients at Bambino Gesù Children's Hospital in Rome over the last 10 years. The cases have been stratified based on the clinical presentation and the relevant literature is discussed accordingly. RESULTS: We discuss the rationale and technique used in these cases based on their presentation, as well as the generally positive outcomes we achieved with early surgical management, use of intra-operative ultrasound (ioUS) and intraoperative neuromonitoring. CONCLUSIONS: Surgical management of pediatric CCMs is a safe and effective strategy, low rates of postoperative morbidity and partial resection were observed.