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Item Indocyanine Green as a Marker for Tissue Ischemia in Spinal Tumor Resections and Extended Revisions: A Technical Note(MDPI, 2025-01-30) Ward, Max; Schneider, Daniel; Brown, Ethan D. L.; Maity, Apratim; Obeng-Gyasi, Barnabas; Ber, Roee; Elsamadicy, Aladine A.; Sciubba, Daniel M.; Knobel, Denis; Lo, Sheng-Fu Larry; Neurological Surgery, School of MedicineBackground/Objectives: The increasing complexity of spinal oncology procedures, particularly in en-bloc tumor resections, creates challenges in tissue perfusion assessment due to extended operative times and extensive surgical dissection. Real-time visualization of tissue perfusion can be achieved with ICG using commercially available handheld imaging systems, offering potential advantages in spinal oncology cases. This study assessed the utility of ICG in analyzing soft-tissue viability during complex spine procedures extending beyond 7.5 h, with a particular focus on oncologic resections. Methods: Three cases that required over 7.5 h of operative time were chosen for ICG utilization. These cases included an en-bloc malignant peripheral nerve sheath tumor resection, an en-bloc resection of a malignant epithelioid neoplasm, and a long-segment fusion revision for pseudoarthrosis. At the conclusion of the critical portion of the procedure, a handheld intraoperative fluorescence camera was utilized to visualize the tissue penetration of intravenous ICG. Results: Prior to injecting ICG, devascularized tissue was not clearly visible. Injecting ICG allowed clear separation of vascularized (fluorescing) and devascularized (non-fluorescing) tissues. One region of non-florescent tissue was later confirmed to be devascularized with MRI and experienced postoperative infection. Conclusions: As the complexity of spinal oncology procedures increases, ICG fluorescence imaging offers a novel method for real-time assessment of tissue perfusion. This technique may be particularly valuable in extensive tumor resections, post-radiation cases, and revision surgeries where tissue viability is at risk. Further investigation in the spinal oncology population could help establish whether early identification of poorly perfused tissues impacts wound healing outcomes.Item Lessons learned from 5 years of pegvaliase in US clinics: A case series(Elsevier, 2024-12-26) Cooney, Erin; Ammous, Zineb; Bender, Tricia; Clague, Gillian E.; Clifford, Marilyn; Crutcher, Angela; Davis-Keppen, Laura; Havens, Kirsten; Lah, Melissa; Sacharow, Stephanie; Sanchez-Valle, Amarilis; Vucko, Erika; Wardley, Bridget; Wessenberg, Leah; Andersson, Hans C.; Medical and Molecular Genetics, School of MedicineObjective: To provide insights and strategies for pegvaliase management in challenging cases with phenylketonuria (PKU) based on the first 5 years of experience with pegvaliase in real-world clinical practice. Methods: Twelve PKU experts gathered during a one-day, in-person meeting to discuss clinical cases illustrating important lessons from their experiences treating patients with pegvaliase in real-world clinical practice. Challenges with pegvaliase experienced prior to and during treatment and corresponding strategies to overcome them were discussed. Results: Twelve cases of adults with PKU (eight females and four males, aged 18 to 68 years) receiving pegvaliase were reviewed and discussed. Challenges of the cases included medical or mental health comorbidities, executive function deficits, challenging social or socioeconomic situations, logistical or geographic barriers, or a combination of these; one was considering pregnancy. Despite challenges, pegvaliase was initiated successfully in most cases. Strategies to overcome barriers included individualized education, including side effect action plans, help from support organizations, collaboration with local providers, and use of telemedicine. Recommendations from the clinicians included that comorbid conditions should be monitored closely after treatment initiation and may require collaboration with other healthcare providers. A collaborative relationship with the clinic, ongoing education, and supportive relatives or friends can help individuals to remain adherent to pegvaliase. Suboptimal adherence may be addressed by a daily reminder system, telemedicine, in-home support, or a modified titration plan. Treated individuals with eating disorders require additional follow-up and support to achieve a healthy relationship with food. In most cases, including late-diagnosed individuals, reduced blood Phe levels resulted in improved PKU-related symptoms, including neurological issues. Conclusion: Experience from the presented cases and 5 years of expert experience with pegvaliase in the real-world setting provide insight and guidance for healthcare professionals initiating and managing pegvaliase treatment in complex PKU cases. These cases demonstrate that, through comprehensive assessment and addressing barriers, pegvaliase treatment can be successful in adults with PKU, regardless of prior treatment success, age, socioeconomic, cognitive, or executive function challenges, as well as in those with comorbidities or considering pregnancy. Ongoing documentation of clinical experience is crucial for advancing the management of individuals receiving this treatment.