Improving Access to Extracranial-Intracranial Bypass Surgery in Border Hispanic Communities Utilizing Collaborative Approach Between Plastics and Neurological Surgery

INTRODUCTION: Hispanic patients such as those with Moyamoya disease are less likely to receive surgical revascularization therapy due to inequities in access (1). Our institution is a located in the Southern Texas- Mexico border region serving a largely Hispanic population. We previously referred patients for EC-IC bypass to other quaternary-care centers in Texas. While referrals were already challenging due to distance, mixed immigration status, and poor socioeconomic background of many patients;COVID-19 further exacerbated this problem with restriction of elective surgical volume. METHOD(S): A consecutive series of EC-IC bypasses performed by authors (SKD and MDLG) were retrospectively reviewed. Baseline clinical, perioperative radiographic, and post-operative outcomes were studied. All patients were offered option of a referral to a quaternary-care centers and also given local option for performing bypass surgery. Further, patients met preoperatively with both the plastic and neurological surgeon. Ultimately, decision was made by patient. RESULT(S): A total of 6 craniotomies for EC-IC bypass were performed during the study period. The diagnoses included Moyamoya in 5 cases and symptomatic intracranial atherosclerosis in one. All patients were Hispanic, female, and nonsmokers with mean age of 35.6 years. Mean preoperative HBa1c was 7.9, preoperative LDL was 82, and mean preoperative hemoglobin was 11.3. Direct bypass was performed in 40% of cases. Mean OR time was 3 hours and 7 minutes. CONCLUSION(S): We have found collaboration between plastic and neurological surgery for surgical revascularization is feasible and improved access to care for Hispanic Moyamoya disease patients residing in a border community.

Post-Transplantation Cyclophosphamide for the Prevention of Graft-Versus-Host Disease Should Not Limit Transplantation for Acute Lymphoblastic Leukemia in People with Trisomy 21

Introduction: Use of hematopoietic cell transplantation (HCT) in patients with trisomy 21 (+21) is infrequent given concerns about increased toxicity with cytotoxic chemotherapy.1 Due to increasing evidence of benefit from post-HCT cyclophosphamide (PTCy) for graft-vs.-host disease (GVHD) prophylaxis and lack of prior descriptions in patients with +21,2-4 we report on 2 patients with +21 and acute lymphoblastic leukemia (ALL) who underwent HCT with PTCy. Method(s): Retrospective data were collected from 2 patients with ALL and +21 who underwent allogeneic HCT with PTCybased GVHD prophylaxis from 2019 to 2021. Data collected included age, disease risk, HCT-CI, GVHD incidence, and survival. Result(s): Patient 1 is a 22-year-old male and patient 2 a 25-year-old female. Both had Ph-negative, B-cell ALL. Patient 1 had ETV6/RUNX1 rearrangement, del 12p, gain of X, and he had recurrence of measurable residual disease (MRD) after initial MRD-negative CR with two lines of therapy pre-HCT. Patient 2 had normal cytogenetics and relapsed disease with 4 prior lines of therapy. Both achieved MRD-negativity pre-HCT. Both received fludarabine and melphalan conditioning, and patient 1 also received thiotepa 2.5 mg/kg. PTCy was given on days +3 and 4 at 50 mg/kg with sirolimus and tacrolimus for GVHD prophylaxis. Patient 1 had a haploidentical donor and received one dose of rabbit ATG (1 mg/kg) on day +5. Patient 2 had a matched unrelated donor. There was no significant delay in engraftment of ANC (day 16-19) or platelets (day 15-16). Patient 2 developed acute GVHD at day 30 (stage I skin, stage II GI) that resolved with steroids which were tapered off by day 96 without recurrence. Sirolimus stopped at day 79 (pt 1) and 103 (pt 2) and tacrolimus was stopped at day 274 (pt 1) and 469 (pt 2). Patient 1 developed a sirolimus-induced pericardial effusion at day 84 which did not recur after sirolimus discontinuation. Patient 2 developed moyamoya 8 months post-HCT during tacrolimus taper without other GVHD symptoms. Response to steroids was noted, so tacrolimus was restarted for residual neurological deficit. Neither patient developed chronic GVHD or left ventricular ejection fraction decline, and neither patient had disease relapse at follow-up of 30 and 16 months respectively. Patient 2 developed COVID pneumonia 16 months post-HCT and died while in CR. Patient 1 remains alive, in CR, and off immunosuppression nearly 3 years post HCT. Conclusion(s): Allogeneic HCT with PTCy at standard doses did not appear prohibitively toxic in patients with +21 when administered after reduced-intensity conditioning. In this case series, GVHD rates seemed consistent with larger series in patients without +21. Moyamoya development is associated with autoimmunity in patients with +21 and hence may have been GVHD-related5. Trisomy 21 should not be a barrier to patients otherwise eligible for HCT, even with PTCy prophylaxis.Copyright © 2023 American Society for Transplantation and Cellular Therapy

Diagnosis and treatment of vasculitis

Primary angiitis of the central nervous system (PACNS) is an inflammatory disease affecting exclusively small and medium-sized vessels of the central nervous system. CNSvasculitis may also occur in systemic diseases like giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis, or Behçet syndrome. The most common presenting symptoms of CNS vasculitis are multifocal symptoms associated with recurrent episodes of ischemia or hemorrhage, encephalopathy-related cognitive and affective abnormalities, and headaches. Diagnostic work up of CNS vasculitis includes MRI, CSF examination, digital subtraction angiography and brain biopsy. High-resolution, contrast-enhanced, compensated and fat-saturated MRI imaging of the cerebral vessel walls (black-blood imaging) may be of some value for the detection of CNS-vasculitis. Patients with normal CSF findings are unlikely to have CNS vasculitis. Brain biopsy should be performed in suspected PACNS. Important differential diagnoses include reversible cerebral vasoconstriction syndrome, moyamoya angiopathy and infectious vasculopathies (VZV, SarsCoV2, borreliosis, bacterial endocarditis). The adherence to diagnostic criteria and the avoidance of inappropriate therapies are essential. Treatment recommendations for CNS-vasculitis include glucocorticoids in combination with cyclophosphamide or rituximab;however, randomized clinical trials of PACNS treatment do not exist. Induction therapy is recommended for 6 to 12 months. After remission is achieved, treatment may be continued with substances as mycophenolate mofetil, methotrexate, or azathioprine. Repeated clinical, CSF- and neuroradiological monitoring is needed to determine the individual duration of maintenance therapy.