A rare case of occlusive juxtafoveolar retinal telangiectasias associated with lesions of the central nervous system: A cerebroretinal vasculopathy like phenotype without mutations in the TREX1 gene.

PURPOSE: To report a rare case of bilateral occlusive juxtafoveolar retinal telangiectasias associated with central nervous system lesions and renal impairment. OBSERVATIONS: A 47-year-old woman presented to clinic with subjective vision loss in the right eye with best-corrected visual acuity (BCVA) 20/80. Fundoscopic examination, fluorescein angiography (FA), and optical coherence tomography with adjunct angiography (OCT/OCT-A) revealed macular microhemorrhages, enlarged foveal avascular zones (FAZ), and occlusive juxtafoveal telangiectasis with pruning of the macular capillaries in both eyes. Patient subsequently developed memory loss, dizziness, nystagmus, and diplopia secondary to intermittent exotropia. She was found to have a two-millimeter aneurysm of the proximal posterior cerebellar artery along with several scattered white matter changes on brain magnetic resonance imaging (MRI). Genetic workup revealed no mutations in the TREX1 gene. With continued surveillance over 18 months, the patient's BCVA deteriorated to 20/200 OU and she developed mild renal impairment, without further CNS complications. CONCLUSION AND IMPORTANCE: Patients who present with vision loss secondary to occlusive juxtafoveolar telangiectasias should undergo imaging of the central nervous system (CNS) for architectural abnormalities in cerebral vasculature and white matter. Further investigation of patients with the Gass-Blodi type 3 macular telangiectasia - cerebroretinal vasculopathy phenotype is required to optimize management protocols for both retinal and CNS lesions. At this time, no interventions have demonstrated clear benefit in vision preservation or recovery.

A unique case of chronic myeloid leukemia presenting as monocular vision loss with unilateral retinopathy.

PURPOSE: To report a case of unilateral leukemic retinopathy secondary to chronic myeloid leukemia (CML). OBSERVATIONS: Patient presented to clinic with a visual acuity (VA) of 20/200 in the right eye (OD) after several months of progressive monocular vision loss and was found to have dense pre-retinal hemorrhage. Patient underwent 23-gauge pars plana vitrectomy to clear the preretinal hemorrhage along with a complex macula-off retinal detachment repair to address retinal tear and multilayer retinal hemorrhage. The patient was subsequently diagnosed with CML as she was found to be positive for the fusion protein of break point cluster gene (BCR) with Abelson tyrosine kinase (ABL1), BCR-ABL1, upon systemic work-up. Imatinib therapy resulted in complete hematologic and cytogenetic resolution after one month, however, the patient's vision remained unchanged six months after surgery. CONCLUSION AND IMPORTANCE: To the authors' knowledge, this is the first reported case of unilateral leukemic retinopathy secondary to low risk CML, as determined by the Sokal and Hasford prognostic scoring systems. CML should be included in the differential diagnosis of patients with progressive monocular vision loss with suspicious multi-layer retinal compromise.

Impact of Direct to Consumer Store-and-Forward Teledermatology on Access to Care, Satisfaction, Utilization, and Costs in a Commercial Health Plan Population.

PURPOSE: Store-and-forward teledermatology can improve access to dermatology by enabling asynchronous consults. This study assesses it on access, satisfaction, utilization, and costs in a commercial health plan setting. METHODOLOGY: In this prospective observational study with matched control, 47,411 individuals were provided access to teledermatology services staffed by board-certified, licensed dermatologists for 6 months. Two hundred forty-three individuals used the service. One hundred fifty-two participants successfully matched to similar users of in-person services for comparison. Average number of dermatology-related visits and dermatology-related costs in 30, 60, and 90 days postvisit, most frequent diagnoses, time to consult, patient satisfaction, and number of procedures were measured. RESULTS: Average time to consult for the teledermatology group was 16.31 h. Patient satisfaction was 4.38/5. Total dermatology utilization in the postvisit period for the teledermatology and the control groups respectively was 9 and 21 visits at 30 days (p = 0.074), 15 and 46 visits at 60 days (p = 0.005), and 26 and 74 at 90 days (p = 0.001). The dermatology-related spend for the teledermatology and control groups, respectively, was $59 and $113 on the day of the initial consult (p < 0.01), $70 and $202 for 30 days (p = 0.03), $78 and $ 221 for 60 days (p = 0.02), and $86 and $307 for 90 days (p = 0.08) following initial visit. Total number of procedures conducted in the control group at the index visit was 26. In the postvisit period, the total number of procedures in the study and control groups, respectively, were 5 and 15 at 30 days (p = 0.053), 10 and 26 at 60 days (p = 0.088), and 14 and 32 at 90 days (p = 0.082). CONCLUSIONS: Teledermatology services are accessible within hours and associated with high patient satisfaction. There is no evident increased utilization or costs postvisit.

Practice Guidelines for Teledermatology.

Previous American Telemedicine Association (ATA) Teledermatology Practice Guidelines were issued in 2007. This updated version reflects new knowledge in the field, new technologies, and the need to incorporate teledermatology practice in a variety of settings, including hospitals, urgent care centers, Federally Qualified Health Centers, school-based clinics, public health facilities, and patient homes.