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PURPOSE: To investigate hand-held optical coherence tomography (HH-OCT) characteristics of small (<1 mm thickness) retinoblastoma. DESIGN: Retrospective observational case series. METHODS: Patient and tumor data were extracted from the medical record and analyzed along with HH-OCT scans. Determination of tumor layer of origin was performed using a layer-by-layer analysis of HH-OCT data and specific HH-OCT-related features were described. RESULTS: There were 20 sub-millimeter retinoblastomas from 16 eyes of 15 patients. Mean largest tumor basal diameter by HH-OCT was 2.2 mm (median, 1.9; range, 0.7-4.1 mm), and mean tumor thickness was 468 µm (median, 441; range, 151-998 µm). In all cases, the retinoblastoma caused discontinuity or disruption of the inner nuclear (INL), outer plexiform (OPL), outer nuclear (ONL), and external limiting membrane (ELM) layers (20/20, 100%). Tumor origin was in the INL in 19/20 (95%) and equivocal (INL vs ONL) in 1/20 (5%). Intratumoral microcalcification was present in 14/20 tumors (70%). There were 2 characteristic findings (signs) on HH-OCT including the INL "fish tail" sign with splaying of the INL at the tumor margin (19/20, 95%) and the ONL "shark fin" sign with folding of the ONL and OPL, conforming to the lateral tumor margins (15/20, 75%). Both signs were concurrently present in 15 tumors (15/20, 75%). CONCLUSIONS: HH-OCT demonstrated that sub-millimeter retinoblastoma seems to originate from the INL, with tumor base and thickness growth progressing in a linear relationship. Characteristic HH-OCT findings included intratumoral microcalcification, INL "fish tail" sign, and ONL "shark fin" sign.

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IMPORTANCE: Although mostly asymptomatic, patients with choroidal nevi carry a moderate risk for malignant transformation and visual loss. A novel noninvasive imaging assessment could change the current clinical evaluation of choroidal nevi. OBSERVATION: Three patients with a recent diagnosis of choroidal nevi underwent a novel adaptive optical assessment that detected potential photoreceptor abnormalities in the retina overlying the choroidal nevi. CONCLUSIONS AND RELEVANCE: Adaptive optics imaging may provide high-resolution en face images of retinal structural changes in the photoreceptor mosaic overlying the choroidal nevi. Cone attenuation may be an important component of structural damage in choroidal nevi and may correlate and possibly predict functional visual loss.

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PURPOSE: To describe a tiny subclinical choroidal melanoma visualized only with enhanced depth imaging optical coherence tomography in a newly symptomatic patient with known oculodermal melanocytosis. METHODS: Case report. RESULTS: A 52-year-old white man with heterochromia and known oculodermal melanocytosis of the right eye, followed for 2 years without change, developed blurred vision and was referred for possible central serous chorioretinopathy. On examination, visual acuity was 20/20 in each eye. There was oculodermal melanocytosis in the right eye involving the periocular skin, episclera, iris, and choroid. On ophthalmoscopy and ocular ultrasonography, there was no appreciable mass, but subtle subfoveal fluid and perifoveal orange pigment were detected, as well as equatorial drusen. Enhanced depth imaging optical coherence tomography demonstrated a subtle optically dense focal choroidal mass measuring 4.5 mm in basal dimension and 0.7 mm in enhanced depth imaging optical coherence tomography thickness. There was choroidal vascular compression, obliteration of choroidal details, and related overlying subretinal fluid with shaggy photoreceptors, consistent with early choroidal melanoma in an eye with oculodermal melanocytosis. The patient elected early treatment considering the risk factors for growth and the risk for metastasis associated with melanoma in the setting of oculodermal melanocytosis. Plaque radiotherapy was performed with complete tumor regression clinically and by enhanced depth imaging optical coherence tomography. At 2-year follow-up, visual acuity remains 20/20, with regressed tumor and no systemic metastasis. CONCLUSION: Enhanced depth imaging optical coherence tomography is a useful tool in the evaluation of eyes with oculodermal melanocytosis, permitting high-resolution visualization of the choroid and detection of submillimeter early melanoma that might not be apparent with indirect ophthalmoscopy or ultrasonography.

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To describe a case of dome-shaped macula simulating a choroidal hemangioma and discuss multimodal imaging features to distinguish between these disorders. A 52-year-old myopic male with a refraction of -8.00 D in both eyes developed blurred vision in the left eye OS) over 4 years. Fundus examination of the right eye (OD) was unremarkable. In OS, there was a subtle orange-colored mass in the macular region with subretinal fluid, suggestive of choroidal hemangioma. Upon referral, our examination disclosed an echodense mass on ultrasonography of OS, measuring 1.0 mm thickness, and additional staphyloma was noted. Enhanced depth imaging optical coherence tomography (EDI-OCT) confirmed subfoveal fluid over a dome-shaped mass that originated in the sclera and not the choroid, consistent with the dome-shaped macula. The OD showed similar features, but to a lesser degree. Both eyes demonstrated choroidal thinning on EDI-OCT, related to high myopia. Dome-shaped macula can masquerade as choroidal hemangioma, especially when associated with subretinal fluid. In these cases, EDI-OCT can document "tumor" origin from within the sclera and not the choroid.