Management of a case of Enhanced S-cone syndrome with massive foveoschisis treated with pars plana vitrectomy with silicone oil tamponade.

Introduction: Goldmann Favre Syndrome (GFS) is a vitreoretinal degenerative disease with macular retinoschisis. The current treatment of foveoschisis is topical and oral carbonic anhydrase inhibitors.Case: A 22-year-old male diagnosed with GFS presented a progressive decrease in vision of the right eye. The optical coherence tomography showed a significant macular schisis. A medical treatment with topical and oral carbonic anhydrase inhibitors was ineffective. We performed a pars plana vitrectomy and silicone oil placement which led to an improvement of the visual acuity and a reduction of the foveoschisis.Conclusion: We describe here the first case of surgical treatment for macular schisis in a patient with GFS.

Contribution of Fourier-domain optical coherence tomography to the diagnosis of keratoconus progression.

PURPOSE: To determine the anatomic criteria for diagnosing keratoconus progression by corneal optical coherence tomography (OCT). SETTING: Quinze-Vingts National Ophthalmology Hospital, Paris, France. DESIGN: Prospective case series. METHODS: Scanning-slit corneal topography (Orbscan II) and Fourier-domain corneal OCT (RTVue) were performed in eyes with mild to moderate keratoconus (progressive or nonprogressive [stable] ectasia) at each examination to assess the keratoconus. Disease progression was defined as an increase of at least 1.0 diopter (D) in the steepest keratometry (K) measurement over 6 months. RESULTS: Of the 134 eyes of 134 patients with mild to moderate keratoconus, 98 had had progressive ectasia and 36 nonprogressive ectasia. The mean maximum K increased significantly in the progressive group (2.1 D ± 1.2 [SD], P < .0001) and remained constant in the stable group (-0.03 ± 0.39 D, P = .31). The mean thinnest corneal thickness increased significantly in the progressive group (-7.98 ± 9.3 µm, P < .0001) and remained constant in the stable group (-0.52 ± 4.21 µm, P = .22). The change in maximum K was significantly correlated with changes in the thinnest corneal thickness (r = -0.61, P < .0001). A cutoff value of -5 µm for the change in thinnest corneal thickness was identified on receiver operating characteristic curves as a threshold separating cases of progressive and stable keratoconus (area under the curve, 0.79; sensitivity, 68%; specificity, 89%). CONCLUSIONS: Topographic data partly reflected the structural changes occurring during the progression of corneal ectasia. Based on the pachymetric parameters provided by OCT, corneal and epithelial thinning was correlated with corneal deformation. The use of corneal OCT might therefore improve the diagnostic sensitivity for keratoconus progression.

Early Epithelial Remodeling After Standard and Iontophoresis-Assisted Corneal Cross-linking as Evaluated by Spectral-Domain Optical Coherence Tomography.

PURPOSE: To evaluate and compare epithelial remodeling after standard corneal cross-linking (S-CXL) and iontophoresis-assisted CXL (I-CXL) in eyes with keratoconus by anterior segment optical coherence tomography (AS-OCT). METHODS: In this prospective, observational study, AS-OCT and corneal topography were performed before and 1, 3, and 6 months after surgery. Corneal pachymetry was performed and epithelial and stromal thicknesses (central and 16 points on the 6-mm central zone) and the maximum simulated keratometry value (Kmax) were measured. RESULTS: Two groups of 30 patients (60 eyes total) with progressive keratoconus underwent S-CXL or I-CXL. Before surgery, both groups of patients presented with an irregular epithelial thickness profile. At 6 months postoperatively, localized corneal epithelial thinning was observed in the superior (P = .026), inferior (P = .034), inferonasal (P = .024), and nasal (P = .035) areas at 3 mm and in the superior (P = .039), nasal (P = .043), and inferior (P = .01) areas at 6 mm in the SCXL group. Localized corneal epithelial thinning was observed at 3 (P = .04) and 6 (P = .01) mm in the inferior area in the I-CXL group. No significant stromal changes were observed for either group. Kmax was stable in both groups at 6 months. CONCLUSIONS: Significant epithelial remodeling occurs after S-CXL, resulting in a thinner, more regular thickness profile than after I-CXL. Epithelial thickness profile measurements after I-CXL could be used as an adjunctive follow-up tool for monitoring the efficacy of CXL in progressive keratoconus. [J Refract Surg. 2018;34(8):551-558.].

Unilateral corneal ectasia after small-incision lenticule extraction in a 43-year-old patient.

Unilateral corneal ectasia developed after small-incision lenticule extraction for mild myopia in a 43-year-old man with preoperative asymmetric astigmatism. The ectasia was diagnosed 4 years postoperatively. Preoperative data showed asymmetric astigmatism with no signs of forme fruste keratoconus. Inferior anterior curvature steepening exceeded 2.00 diopters without bulging of the posterior curvature, and pachymetric thickness exceeded 515 µm. Corneal ectasia can occur after small-incision lenticule extraction in patients older than 40 years with preoperative asymmetric astigmatism.