Multimodal imaging evaluation of combined hamartoma of the retina and retinal pigment epithelium.

PURPOSE: Combined hamartoma of the retina and retinal pigment epithelium is a rare benign tumor characterized by a variable combination of glial, vascular, and pigmented components. The purpose of our study was to analyze the features of combined hamartoma of the retina and retinal pigment epithelium on optical coherence tomography angiography. METHODS: Small case series of two cases of combined hamartoma of the retina and retinal pigment epithelium with macular and optic nerve involvement, evaluated with multimodal imaging including optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography. RESULTS: On optical coherence tomography, combined hamartoma of the retina and retinal pigment epithelium is characterized by disruption of the inner neurosensory retina and a variable degree of involvement of the external retina. Optical coherence tomography angiography showed diffuse alterations of the retinal vessels of the superficial and deeper layers, extended to the peripapillary area. Vessel abnormalities included increased tortuosity and caliber of vessels, vascular traction, and vessel stretching within the lesion. CONCLUSION: Optical coherence tomography angiography allows in-depth multilayer analysis of tumor vascular network, highlighting the fine abnormalities of retinal vasculature characteristic of combined hamartoma of the retina and retinal pigment epithelium.

Morpho-functional evaluation of torpedo maculopathy with optical coherence tomography angiography and microperimetry.

PURPOSE: To report the case of a 13-year-old girl with torpedo maculopathy, evaluated with multimodal morpho-functional retinal imaging, including fundus photography, infra-red and blue fundus autofluorescence, swept-source optical coherence tomography (OCT), en face OCT, OCT angiography and microperimetry (MP). OBSERVATIONS: On fundus examination, a torpedo-like hypopigmented lesion was observed temporal to the fovea in the left eye. OCT showed disruption of outer retinal layers and the presence of a subretinal cleft. On OCTA, a diffuse attenuation of signal from choriocapillaris was observed along the lesion. Functional analysis with MP revealed a reduction of retinal sensitivity over the lesion. CONCLUSIONS: and importance: On OCTA, torpedo maculopathy is characterized by vascular alterations of the choriocapillaris along the lesion.

ARCUATE NERVE FIBER LAYER CHANGES AFTER INTERNAL LIMITING MEMBRANE PEELING IN IDIOPATHIC EPIRETINAL MEMBRANE.

PURPOSE: To analyze the relationship between swelling of the arcuate nerve fiber layer (SANFL) and long-term decrease of retinal nerve fiber layer thickness after internal limiting membrane peeling for idiopathic epiretinal membrane, and to investigate if SANFL is related to a mechanical surgical damage. METHODS: Prospective, interventional consecutive case series of 46 eyes that underwent combined epiretinal membrane/internal limiting membrane peeling for idiopathic epiretinal membrane. Infrared, blue autofluorescence, color fundus imaging and measurement of retinal nerve fiber layer thickness in six peripapillary sectors by spectral-domain optical coherence tomography were performed preoperatively and at 2 weeks, 1, 3, 6, and 12 months after surgery. The presence of SANFL was checked postoperatively on infrared and blue autofluorescence fundus imaging, and the extent of each SANFL was measured on infrared fundus images. RESULTS: Areas of SANFL were identified in 39 eyes (84.8%) at 2-week follow-up. Retinal nerve fiber layer thickness significantly decreased in the temporal sectors at 1, 6, and 12 months (P < 0.0001). The linear extent of SANFL was significantly correlated with the percentage of reduction in retinal nerve fiber layer thickness in the temporal (R = 0.45; P < 0.0001) and infero-temporal (R = 0.23; P = 0.0008) sectors at 12 months of follow-up. Correspondence between sites of surgical grasping and the points of origin of SANFL was demonstrated on blue autofluorescence fundus images superimposed on intraoperative surgical frames. CONCLUSION: Early postoperative SANFL is correlated with late focal retinal nerve fiber layer thinning in the temporal sectors. Intraoperative surgical grasping seems to be a leading factor for the onset of SANFL.

Combined deep sclerectomy and descemet stripping automated endothelial keratoplasty.

PURPOSE: The aim of this study was to evaluate a novel surgical combination of Descemet stripping automated endothelial keratoplasty (DSAEK) and deep sclerectomy (DS) for the management of concomitant corneal endothelial decompensation and uncontrolled glaucoma. METHODS: This retrospective case series noncomparative study included 9 eyes of 6 consecutive patients with coexistence of corneal edema resulting from Fuchs dystrophy or pseudoexfoliation keratopathy and medically uncompensated glaucoma; these patients underwent combined DSAEK and DS with mitomycin C and an absorbable collagen implant. Corneal graft clarity, endothelial cell density, visual acuity, intraocular pressure (IOP), and identification of complications were assessed over a 2-year follow-up. RESULTS: All eyes obtained graft clarity throughout the follow-up, with a final average endothelial cell decrease of -36% from baseline, and showed improved vision and good IOP control without hypotensive therapy. Measured at 3 and 24 months postoperation, the mean visual acuity improvement was 154% and 372% and IOP decrease was 51.1% and 46.4%, respectively. Two anterior segment complications occurred in 2 (22%) patients' eyes. This consisted of a graft dislocation and a modest IOP elevation, treated successfully. CONCLUSIONS: Combined DSAEK and DS was longitudinally associated with good corneal graft survival and IOP control, with few complications. These findings suggest that this surgical approach is a viable option for patients with coexisting glaucoma and corneal endothelial dysfunction. Our study should stimulate a multicenter, randomized, controlled trial of our technique.