Cross-Sectional Analysis of Neurocognitive Function, Retinopathy, and Retinal Thinning by Spectral-Domain Optical Coherence Tomography in Sickle Cell Patients.

PURPOSE: The purpose was to examine the relationship between neurocognitive function and two distinct forms of retinopathy in sickle cell disease. MATERIALS AND METHODS: Patients with sickle cell disease (n = 44, age range: 19-56 years, 70% female) were prospectively recruited for this cross-sectional study. Retinopathy was characterized by: (1) Presence of focal retinal thinning on spectral domain optical coherence tomography and (2) determination of the sickle retinopathy stage on funduscopic exam based on Goldberg classification. Neurocognitive function was assessed using the Philadelphia Brief Assessment of Cognition (PBAC), a validated test of cognition. Univariate and multivariate analyses for PBAC score outcomes were performed. Retinal thinning and retinopathy stage were primary variables of interest and age, gender, genotype, education, and history of stroke were covariates. RESULTS: Univariate analysis revealed associations with total PBAC score and age (P = 0.049), history of stroke (P = 0.04), and genotype (P < 0.001). Focal retinal thinning and Goldberg retinopathy stage were not associated with each other in this sample. Neither the presence of focal retinal thinning nor degree of retinopathy was associated with total PBAC score in univariate or multivariate analyses. CONCLUSIONS: We find an association between lower cognitive function and older age, history of stroke and sickle cell genotype SS in patients with sickle cell disease. Our data do not provide evidence to support an association between cognitive function and retinopathy in sickle cell patients.

Bilateral Congenital Posterior Capsular Defects and Ectopic Cataracts.

Preexisting posterior capsular defects are rare and can be associated with infantile cataracts. The authors review possible etiologies of this condition and emphasize the importance of cautious preoperative planning and surgery.

The effect of rectus muscle recession, resection and plication on anterior segment circulation in humans.

BACKGROUND: Plication is an alternative tightening procedure to resection. In monkeys, plication has been shown to preserve anterior segment circulation compared with full-tendon tenotomy, but this is unconfirmed in humans. PURPOSE: To evaluate anterior segment circulation by iris angiography before and after strabismus surgery in humans. METHODS: Prospective, blinded study of 14 patients (mean age (SD), 58.6 (14.3)) undergoing plication and/or full tendon tenotomy (resection or recession) from August 2013 to March 2014. Eight patients (mean age (SD), 59.0 (13.3)) underwent plication of one muscle with or without recession of a second muscle on the same eye and six patients (mean age (SD), 58.2 (16.8)) underwent tenotomy of one to two muscles on the same eye. Preoperative and postoperative iris angiograms were compared for changes in perfusion by a masked examiner. In patients undergoing binocular surgery, one eye was chosen preoperatively to be the study eye. RESULTS: Postoperative iris filling defects were present in four patients (67%) after tenotomy and one patient (12.5%) after plication (p=0.09). Of the seven total vertical rectus muscles operated (three tenotomies and four plications), filling defects were present after three tenotomies and one plication (100% vs 25%; p=0.14). Of the 13 total horizontal rectus muscles operated (eight tenotomies and five plications), filling defects were present after one tenotomy and none of the plications (13% vs 0%; p=0.99). CONCLUSIONS: Rectus muscle plication spares the ciliary vessels and may be considered a safer alternative to resection for patients at risk for anterior segment ischaemia, especially when surgery involves a vertical rectus muscle.

Bilateral traumatic expulsive aniridia after phacoemulsification.

We report a case of bilateral traumatic expulsive aniridia after uneventful phacoemulsification through small clear corneal incisions. Phacoemulsification was performed 8 and 13 months prior to the trauma in the left and right eyes, respectively. In both eyes, the intraocular lens and capsular bag were undisturbed after trauma. After resolution of hyphema, transient elevated intraocular pressure, and anterior chamber inflammation, best corrected visual acuity returned to 20/25 in each eye 6 months later. Self-sealing clear corneal wounds likely serve as a decompression valve during blunt trauma, thus preventing devastating intraocular damage and globe rupture. The intraocular lens may absorb the external force, therefore preventing damage to the capsular bag and zonules as well as preventing prolapse of posterior structures. A review of previously reported cases of traumatic aniridia is also presented.

Chronic anterior uveitis in common variable immunodeficiency.

PURPOSE: To describe patients with uveitis and common variable immunodeficiency (CVID). DESIGN: Retrospective observational case series. METHODS: Retrospective review of patients with uveitis and CVID, specifically focusing on clinical presentation and treatment. RESULTS: Three patients with CVID and uveitis were identified. All patients had chronic anterior uveitis. Two required systemic immunosuppression to control uveitis, with one of these patients relapsing after discontinuation of immunoglobulin therapy. One improved on topical steroid therapy; however, follow-up on this patient did not extend beyond 4 months. CONCLUSIONS: CVID can be associated with chronic anterior uveitis. Patients presenting with uveitis should be questioned about frequent infections and autoimmune disorders as part of the uveitis evaluation.