Terminology for Retinal Findings in Sickle Cell Disease Research: A Scoping Review.

OBJECTIVE: To review the current sickle cell disease (SCD) literature to assess how "retinopathy" has been defined and to identify ocular outcomes that have been measured and described. DESIGN: A systematic scoping review of SCD literature was completed regarding ocular manifestations of SCD and vision outcomes across all medical specialties. SUBJECTS: Participants with SCD and control patients were included in our data extraction. METHODS: We reviewed English-language literature from 2000 to 2021 for eligible studies by searching PubMed, Google Scholar, Embase, and the Cochrane library using terms to encompass SCD and ocular findings. MAIN OUTCOME MEASURES: Data collection included study information, patient characteristics, vision-related findings (inclusion criteria and/or study outcomes), and retinopathy characteristics (definition, when, how and by whom diagnosed). RESULTS: We identified 4006 unique citations and 111 were included in the analysis. Ophthalmologists were senior authors of about half (59/111; 53.2%) of the articles; most articles were published between 2016 and 2021 (71/111; 70.0%). The studies had been conducted primarily in North America (54/111; 48.6%) or Europe (23/111; 20.7%); designs were cross-sectional (51/111; 45.9%), prospective cohort (28/111; 25.2%), retrospective cohort (27/111; 24.3%), and case-control (4/111; 3.6%). Among studies reporting any retinopathy, it was commonly defined as a combination of nonproliferative sickle cell retinopathy and proliferative sickle cell retinopathy (PSR; 52/87; 59.8%), infrequently as PSR only (6/87; 6.9%), or not defined at all (23/87; 26.4%). The Goldberg classification was used to grade retinopathy in almost half of the studies (41/87; 47.1%). Investigators reporting diagnostic methods used clinical fundus examination (56/111; 50.4%), OCT (24/111; 21.6%), fluorescein angiography (20/111; 18.0%), ultrawidefield fundus photographs (15/111; 13.5%), and OCT angiography (10/111; 9.0%), or did not report methods (28/111; 25.2%). CONCLUSIONS: There are inconsistencies in documentation of methods and outcomes in studies of SCD ophthalmic findings. Particularly concerning is the lack of documentation of ophthalmic examination methods, qualifications of examiners, and clarity and specificity of sickle cell retinopathy definitions. With the increase in SCD treatment research and novel systemic therapies available, it is important to adopt clear and consistent descriptions and rigorous data collection and reporting of ophthalmic outcomes in SCD studies. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.

Erratum: Nurse Practitioner Students' Knowledge Assessment and Perceived Preparedness to Triage Ophthalmology Complaints in a Primary Care Setting: An Educational Intervention.

[This corrects the article DOI: 10.1055/s-0043-1771355.].

Nurse Practitioner Students' Knowledge Assessment and Perceived Preparedness to Triage Ophthalmology Complaints in a Primary Care Setting: An Educational Intervention.

Purpose In this proof-of-concept pilot study, we aimed to increase nurse practitioner (NP) student knowledge of ophthalmology to prepare NPs for encounters in primary care settings. The Association of University Professors of Ophthalmology (AUPO) and the American Academy of Ophthalmology (AAO) endorse core knowledge that medical students should achieve. We assess the effectiveness of an innovative ophthalmologist-led curriculum based on these competencies tailored to issues NPs encounter in primary care. Methods Johns Hopkins University NP students enrolled in a pre-post-cohort study and educational intervention. The didactic program was developed according to AUPO and AAO core ophthalmology content for medical students and was taught in-person by an ophthalmologist. Pre-post-assessments evaluated students' perceived readiness to encounter ophthalmic issues in the clinic and baseline knowledge of core competencies of ophthalmology. Results A total of 42 NP students were included in the analysis. NP students improved in core knowledge and readiness to encounter ophthalmology issues. After the educational event, there was a statistically significant improvement in students' ratings of preparedness to obtain a focused history, exam, perform initial management and decide the urgency of a referral for acute painless vision loss ( p < 0.001), chronic vision loss ( p < 0.001), or a patient with a red/painful eye ( p < 0.001). Students showed a statistically significant improvement in postdidactic event core ophthalmology knowledge assessment scores ( p = 0.002). Conclusion Primary care NPs are increasingly the initial point of contact for patients with ophthalmic complaints, and thus, high-quality and thorough education regarding ophthalmology triage and referral for NPs is necessary. NP student comfort with and knowledge of ophthalmic complaints and triage may be improved by a brief educational intervention taught by an ophthalmologist early in the NP curriculum.

Analyzing the demographics of patients with uveitis in an indigent, urban population.

PURPOSE: To study the types of uveitis examined in a hospital serving indigent populations in need of low-cost care. METHODS: A retrospective chart review examined the electronic medical records of all patients with uveitis-related at Drexel Eye Physicians. Data collected included demographics, anatomic location of the uveitis, systemic disease associations, treatment modalities and insurance. Statistical analysis was performed using χ² or Fischer exact tests. RESULTS: 270 patients (366 eyes) were included for analysis, 67% of patients identified as African American. Most eyes (95.3%, N = 349) were treated with topical corticosteroid drops, and only 6 (1.6%) received an intravitreal implant. Immunosuppressive medications were started in 24 patients (8.9%). Nearly 80% depended to some extent on Medicare or Medicaid Assistance for treatment coverage. There was no association between insurance type and use of biologics or difluprednate. CONCLUSION: We found no association between insurance type and the prescription of medications for uveitis that should be used at home. There was a minimal number of patients prescribed medications for implantation in the office. The adherence of use of medications at home should be investigated.

Clinical Vignettes, Part II: Eyes, Teeth, and Bone.

Microvascular occlusions caused by sickle cell disease (SCD) can affect all ocular and orbital structures. Sickle cell retinopathy (SCR) is the most common ophthalmic manifestation of SCD. Fortunately, most individuals with SCR are visually asymptomatic. Vision loss in SCD most commonly occurs as a consequence of proliferative sickle cell retinopathy (PSR), in which pathologic retinal neovascularization occurs. To prevent significant vision loss and blindness, which can occur from complications of PSR, regular retinopathy surveillance screening examinations and consistent follow-up with a retina specialist are recommended. Scatter laser photocoagulation is the current gold-standard treatment to prevent vision threatening progression of PSR. Patients with sickle cell disease should have regular checkups with their dental care provider. Patients should be educated on the importance of proper dental care, a healthy diet, and the need for early intervention if they suspect any dental problems or are having dental pain. If any dental procedures that involve surgery or sedation are planned, it is critical to consult with the hematologist before the procedure is started. Prophylactic antibiotics may have to be prescribed before invasive dental procedures, such as extractions or periodontal surgery but is best determined by discussions between the dental care provider and the hematologist. Osteonecrosis is a highly prevalent skeletal complication of sickle cell disease that affects all genotypes. Risk factors for osteonecrosis include older age, HbSS genotype with concomitant alpha-thalassemia trait, frequent vaso-occlusive episodes, history of acute chest syndrome, elevated body mass index, and low white blood cell counts. Osteonecrosis causes progressive joint damage and associates with chronic pain, frequent acute care visits, and overall poor health-related quality of life. Current consensus guidelines recommend analgesics, physical therapy, and early consideration of joint arthroplasty in sickle cell-related osteonecrosis, although surgery may be deferred until late adolescence after growth plates have fused.

Thrombocytopenia and clear corneal incision cataract surgery.

PURPOSE: To investigate complications and outcomes of clear corneal incision cataract surgery in patients with thrombocytopenia. SETTING: 1 veterans hospital and 2 academic medical centers. DESIGN: Multicenter retrospective chart review. METHODS: All eyes of thrombocytopenic patients that underwent clear corneal incision cataract surgery with a platelet count of 100 × 103/µL or less measured within 30 days prior to surgery were included. Subject demographics, intraoperative complications, use of pupillary expansion devices, use of local anesthetic injections, and change in corrected distance visual acuity were recorded. RESULTS: 3 sites recorded 40 113 clear corneal incision cataract surgeries, of which 196 eyes (0.49%) of 150 thrombocytopenic patients were recorded. The mean platelet count in the study subjects was 73.0 ± 20.5 × 103/µL. Two cases of intraoperative iris hemorrhage that were readily and controlled occurred in conjunction with pupillary expansion. There were no bleeding complications associated with retrobulbar, peribulbar, or sub-Tenon anesthetic injections. There was a statistically significant improvement (P < .0001) in visual acuity post-operatively. CONCLUSIONS: Clear corneal incision cataract surgery with pupillary expansion devices and local anesthetic injections can be safely performed in patients with thrombocytopenia.