Aims: To investigate the prevalence of sickle cell maculopathy (SCM), associations with age, sex, genotype and proliferative sickle cell retinopathy (PSR) stage and the impact on visual acuity
Methods: Age, sex and visual acuity were recorded and spectral domain OCT and ultra-widefield images of the macula and retina were reviewed for a consecutive series of 74 adults with sickle cell disease
Results: The median age was 37 years (range 19-73 years) and 36 cases (48.6%) were male. SCM was present in at least one eye of 40 cases (54.1%) or in 67 of all eyes (42.3%). SCM prevalence was 54.8%, 62.5% and 25% for HbSS, HbSC and HbS/BThal or other genotypes respectively. SCM was observed in 41 (39.4%) of the eyes with PSR stages 0,1,2 and 21 (51.2%) of the eyes with PSR stages 3,4,5. Mild visual impairment or worse was present in 3 eyes (4.8%) with SCM but this was secondary to other pathology.
Conclusion: SCM is a frequent finding in eyes of adults with sickle cell disease. The prevalence is similar for both HbSS and HbSC genotypes and is not related to the PSR stage. High-contrast distance visual acuity is typically preserved.
Purtscher's retinopathy is a rare angiopathy reported in patients with a history of severe trauma and other systemic diseases. The diagnosis is made on clinical grounds, and the severity varies. A 41-year-old gentleman with underlying poorly controlled diabetes mellitus and dyslipidemia was referred to the ophthalmology department for diabetic retinopathy screening. He denied visual complaints. Ocular examination revealed a negative relative afferent pupillary defect with a visual acuity of 6/6 bilaterally. The anterior segment examination was unremarkable. Both eye (oculus uterque, OU) fundus revealed a pink disc with a cup-to-disc ratio of 0.4 and peripapillary flame-shaped hemorrhages. Right eye (oculus dexter, OD) also showed multiple cotton wool spots along the superotemporal arcade involving zones 1 and 2 of the retina, while left eye (oculus sinister, OS) showed a single cotton wool spot along the superotemporal arcade at zone 1 of the retina. Otherwise, there were no visible retinal emboli, dot hemorrhages, or hard exudates, and the macula was normal. The retinal features were not characteristic of diabetic retinopathy. It mimicked hypertensive retinopathy, but the patient was normotensive. The absence of inner retinal thickening and hyperreflectivity on optical coherence tomography of the macula ruled out retinal vein occlusion. This prompted us to elicit further history, and the patient disclosed a recent admission for myocardial infarction in which he received cardiopulmonary resuscitation with chest compressions for seven minutes. Hence, the diagnosis of OU Purtscher's retinopathy was made, and the patient was monitored closely in the clinic. Purtscher's retinopathy remains a diagnostic dilemma and should not be neglected in complex clinical contexts.
Background: Penicillin is the conventional treatment for all stages of syphilis, including ocular and neurosyphilis, according to the recommendations by the Centre for Disease Control and Prevention Sexually Transmitted Disease. This case series highlighted three cases of ocular syphilis which showed prompt treatment response as early as 24 h after the adjunctive intravitreal ceftazidime injection. Methods: Case Series. Results: In case 1, there was significant improvement in the vision and vitritis after 24 h of a single intravitreal ceftazidime injection. In case 2, the patient achieved his best vision after a total of three intravitreal injections in his left eye and one in his right eye. In case 3, there was a further resolution of perivascular sheathing and retinal haemorrhages seen 1 week after a single intravitreal ceftazidime injection. Conclusion: The efficacy of intravitreal ceftazidime injection as a local adjunctive therapy in ocular syphilis treatment is noteworthy. It can be considered in cases which show suboptimal or slower treatment response despite early commencement of systemic penicillin to prevent devastating ocular sequelae.
Hemolacria is a rare condition that causes a person to produce tears that are partially composed of blood. It can be a presenting feature of certain ocular and systemic conditions. Here, the authors describe an interesting case of a 12-year-old boy with an underlying beta-thalassemia trait, who presented with a 2-day history of bilateral blood-stained tears, and an episode of epistaxis. Ocular examination was normal, and syringing showed no nasolacrimal duct blockage. Systemic examination was unremarkable. Laboratory investigations confirmed type 2 von Willebrand disease. Management of hemolacria remains a clinical challenge given the rare occurrence of the disease. In this case report, the authors discuss the differential diagnosis and management approach to hemolacria.
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