Multimodal Imaging in Retinitis Pigmentosa: Correlations among Microvascular Changes, Macular Function and Retinal Structure.

PURPOSE: To analyze microvascular changes in patients with retinitis pigmentosa (RP) with relatively preserved visual acuity (VA), using swept source optical coherence tomography (SS-OCT) angiography to correlate results to macular function and structure. METHODS: This was a case-control study conducted over 70 eyes of 35 RP patients with relatively preserved VA. All patients underwent a complete ophthalmic examination, including SS-OCT, OCT angiography (OCT-A), fundus autofluorescence (FAF), and multifocal electroretinogram (mfERG). Thirty-four eyes of 34 healthy controls of age-, sex-, and axial length-matched (control group), were also analyzed. The main outcome measures were foveal and parafoveal vascular densities (FVDs and PFVDs) in the superficial capillary plexus (SCP) and deep capillary plexus (DCP), foveal avascular zone (FAZ) and its enlargement coefficient and their correlation with macular function (by means of VA and mfERG), and structure (by means of FAF and SS-OCT). RESULTS: In the RP group, PFVD was 25.99 ± 5.2% in the SCP and 34.47 ± 2.37% in the DCP and were significantly lower as compared to control group (P < 0.0001; P = 0.0026, respectively). Enlargement coefficient of FAZ was 1.78 ± 0.79. We found a statistically significant correlation between VA and PFVD in the DCP (P < 0.0001), FAZ disruption in the SCP (P = 0.006) and enlargement coefficient of FAZ (P = 0.01). The parafoveal DCP density was significantly correlated with P1 amplitude (P = 0.005) in rings 2, 3, 4, and 5 of the mfERG. We found a statistically significant correlation between parafoveal density in the DCP, thickness of ganglion cell complex (GCC) (P = 0.001), and the width of ellipsoid band (P = 0.041). Parafoveal SCP density was also correlated to GCC (P = 0.033). CONCLUSIONS: We showed that vascular alteration in RP begins at the level of the DCP, which affects the outer retina and leads to a narrowing of the ellipsoid. The alteration of the SCP would occur later in the evolution of the disease. Vascular changes occur early during RP and were highly correlated to retinal function and structure. OCT-A seems to be a good tool to quantify vascular network loss and could play a central role in staging, prognosis, and monitoring disease progression.

Multimodal imaging in a case of a congenital retinal macrovessel associated with a retinal cavernous hemangioma: a case report.

BACKGROUND: To report the results of multimodal imaging in a case of a congenital retinal macrovessel associated with a retinal cavernous hemangioma. CASE PRESENTATION: A 52-year-old female patient presented with progressive vision loss in the right eye. BCVA was 8/20 in the right eye and 18/20 in the left eye. Fundus examination of the right eye showed an aberrant retinal macrovessel arising from the inferior temporal major vein. It crossed the foveal area and overstepped to the superior retina. A "brunch of grapes" shaped retinal lesions arised from the macrovessel. Fluorescein angiography showed saccular lesions that filled slowly during the venous phase and became brightly hyperfluorescent saccular caps. SS-OCT of the right eye revealed a highly back-scattering hyper-reflective vessel across the fovea with shadow effect and adhesions between the vitreous and the aberrant macrovessel. It also revealed hypo reflective saccules with hyperreflective borders located in the inner retina corresponding to the cavernous retinal hemangioma. Retinal pigment epithelium undulations and vascular dilations at the level of Haller's layer were observed in both eyes. Indocyanine green angiography revealed chroidal vascular dilatations in both eyes in the late phase. OCT-A showed the aberrant vessel emerging from the inferior temporal vein and splitting the foveal avascular zone horizontally. RCH appeared as small heterogeneous saccular flow areas associated with focal capillary hypo perfusion areas. Asymmetry and distorsion of the foveal avascular zone were also noticed. A diagnosis of retinal macrovessel associated with a retinal cavernous hemangioma was made. CONCLUSIONS: Congenital retinal macrovessels and retinal cavernous hemangioma are benign lesions. Their association is rare. Abnormal vascular development is likely to be responsible for their association. Swept source OCT and OCT angiography may be of a great contribution to better evaluate these retinal vascular disorders.

Unilateral MALT conjunctival lymphoma: a case report.

INTRODUCTION: The conjunctival localization of MALT-type lymphoma is rare although it is the most common tumor of the ocular adnexa. OBSERVATION: We report a case of a 57 years old female patient who presented with a unilateral primitive MALT lymphoma, confined to the conjunctiva, she consulted for a protrusion of her left eye associated with a painless conjunctival lesion. Morphological and immunohistochemical analysis led to the diagnosis. CONCLUSION: Conjunctival MALT lymphoma is the most frequent tumor of ocular adnexa. It may be primary or secondary. Presentation is not specific, but the observation of a salmon colored conjunctival mass may lead to the diagnosis.

Ophtalmoplegia complicating sino-orbital mucormycosis.

PURPOSE: To report a case of complete ophthalmoplegia and blindness in sino-orbital mucormycosis. CASE REPORT: A 68-year-old woman with history of diabetes and breast cancer presented with sudden onset of fever, orbital pain and blindness in the right eye. The patient was found to have no light perception, complete ophthalmoplegia, chemosis, corneal ulcer and optic nerve atrophy. In rhinologic examination, necrosis was noticed. Brain and orbit magnetic resonance imaging showed diffuse sinusitis with orbital involvement. The result of the clinical examination was reported as sino-orbitalmucormycosis. The diagnosis was confirmed by pathological specimens. The patient underwent extensive surgical debridement and systemic antifungal treatment. Despite treatment, visual acuity did not recover. CONCLUSION: The sino-orbital form of mucormycosis is a rare and insidious fungal infection. Ocular findings may range from orbital pain to ophtalmoplegia and blindness such as in our case. Black scar tissues seen on the nasal mucosa are pathognomonic. Delay in treatment due to late presentation and complications were major determinants in ocular prognosis and survival outcome.