Orbital Edema Secondary to a Sphenoidal Mass as the Presenting Symptom of High-Risk Precursor B-Cell Acute Lymphoblastic Leukemia.

Introduction: Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy, known to present with ocular manifestations in rare cases. Case Presentation: We describe the case of a 9-year-old previously healthy male who developed a 2-day history of periocular swelling and was found on MRI to have a large sphenoidal mass. Further work up showed involvement of the spinal cord, iliac crests, and kidneys. His initial blood work showed no hematological abnormalities. A bone marrow biopsy taken from the iliac crest demonstrated >90% B lymphoblasts and flow cytometry was positive for CD19. Overall, his investigations were consistent with a diagnosis of precursor B-cell ALL (pre B-ALL). His neuro-ophthalmic exam showed right-sided subtle periocular edema, decreased palpebral fissure height, and proptosis. Posterior exam showed mild nasal elevation of the right optic disc without vessel obscuration and mild tortuosity of the peripheral vessels. He otherwise had no overt signs of afferent or efferent dysfunction despite the proximity of the mass to his optic nerve and globe. Conclusion: This case demonstrates that high-risk pre B-ALL, a childhood cancer not commonly associated with orbital manifestations, can present with orbital edema and normal leukocyte count in an otherwise healthy child.

A Case Report of an Isolated Cardiac Metastasis in a Patient with Esophageal Carcinoma.

A 76-year-old Caucasian male presented with syncope, intermittent melena, anemia, and unexplained weight loss. Esophagogastroduodenoscopy revealed a friable non-obstructing esophageal tumor that appeared thickened on computed tomography (CT). Biopsies confirmed a poorly differentiated carcinoma. Fluorine-18 fluorodeoxyglucose positron emission tomography/CT (F-18 FDG PET/CT) showed intense FDG avidity with a maximum standardized uptake value (SUVmax) of 23. Although CT did not identify any lymphadenopathy or distant metastases, a mildly enhancing lobulated circumscribed mass with no internal calcification was incidentally identified in the left atrium. Cardiac magnetic resonance imaging (MRI) favored myxoma over thrombus given the signal characteristics and mild enhancement; however, F-18 FDG PET/CT showed an SUVmax of 18, more consistent with a metastasis. The cardiac mass was resected and shown to be a metastatic focus of poorly differentiated carcinoma, histologically identical to the esophageal mass. He received a single 8 Gray (Gy) fraction of urgent hemostatic radiotherapy for his primary tumor followed by palliative chemotherapy with cisplatin, capecitabine, and pembrolizumab. He was readmitted for transfusion due to recurrent bleeding from his primary tumor and given a second urgent hemostatic fraction of 8 Gy for stabilization. Systemic therapy was eventually discontinued due to declining performance status. He received consolidative palliative radiotherapy (20Gy in five fractions) but continued to deteriorate over the next three months and died in hospice, ten months from the time of his initial presentation.

Patient Personality and Illness Perceptions in Relation to Follow-Up Appointment Adherence in Neuro-Ophthalmology.

BACKGROUND: Improving patient attendance at medical follow-up visits may have a notable impact on disease and overall health outcomes. Understanding factors contributing to poor attendance is important for identifying at-risk patients and designing interventions to improve clinical outcomes. Our objective was to identify personality and illness perception factors associated with attendance at recommended follow-up visits in a neuro-ophthalmology practice. METHODS: New or established patients (≥18 years) with scheduled neuro-ophthalmology (study) or glaucoma (comparison) appointments at a tertiary care academic medical center completed the Brief Illness Perception Questionnaire and Ten-Item Personality Inventory. Physician recommendations made during the visit were recorded (medications, referrals, follow-up, testing, and procedures). A chart review was performed 18 months after enrollment to assess attendance at follow-up appointment and adherence with other physician recommendations. Multiple variable logistic regression models studied associations between follow-up appointment attendance and demographic factors, appointment factors, and survey responses. RESULTS: Among 152 respondents (97% response rate (152 of 157), aged 19-97 years, 58% female, 34% new, 80 neuro-ophthalmology, 72 glaucoma), neuro-ophthalmology subjects were younger, more likely to be White, non-Hispanic, female and new to the practice than subjects with glaucoma. They reported higher emotional impact, identity, and consequences related to their illness (P = 0.001-0.03). Neuro-ophthalmology physician recommendations included more referrals to other services (17.5% vs 1.4%, P = 0.001, chi-square) and more radiology studies (15% vs 0%, P = 0.001, chi-square), but fewer follow-up visits (75% vs 97%, P < 0.0005, chi-square). Among those with recommended follow-up visits, neuro-ophthalmology subjects had lower rates of on-time appointment attendance (55% vs 77%, P = 0.009, chi-square). In a multiple variable model, on-time follow-up attendance was associated with shorter recommended follow-up interval (≤90 days, P < 0.0005), established (vs new) patient status at enrollment visit (P = 0.04), and glaucoma (P = 0.08), but not subject demographics, illness perception, or personality factors. CONCLUSIONS: Patient demographics, illness perception, and personality traits were not associated with follow-up appointment attendance and therefore unlikely to be useful for identifying patients at risk of being lost to follow-up. New neuro-ophthalmology patients with a follow-up recommended ≥90 days in advance may benefit from targeted interventions to improve follow-up appointment adherence.