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Uveal melanoma is the most common intraocular malignancy in adults. Despite advances in local treatments, approximately 50% of all cases eventually die from metastatic disease. In cases with metastasis, 2- and 5-year survival rates are approximately 10% and <1%, respectively. Advances in molecular biology have led to the identification of a number of promising drugs including immune checkpoint inhibitors (ICIs). Ipilimumab and nivolumab are ICIs targeting the cytotoxic T-lymphocyte-associated antigen-4 and the programmed-cell death protein-1, respectively. Herein, we present a case of choroidal melanoma having liver metastasis treated with nivolumab and ipilimumab and transarterial radioembolization, achieving a 3-year survival.
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Retinoblastoma (RB) is the most common primary intraocular malignant tumor of childhood. Persistent or recurrent vitreous seeding is the most common reason for therapeutic failure in advanced RB. Intravitreal chemotherapy has emerged as an effective therapy for vitreous seeding in RB, with a generally acceptable safety profile. However, intravitreal chemotherapeutics, especially melphalan, can cause toxicity that may progress to total retinal atrophy. In this report, we present two cases with retinal melphalan toxicity that had varied clinical findings. One of the cases had extensive retinal atrophy that was demonstrated by hand-held spectral domain optical coherence tomography (HHSD-OCT), while the other had normal retinal anatomy on HHSD-OCT but markedly diminished retinal function on flash electroretinography.
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PURPOSE: To investigate the swept-source optical coherence tomography (SS-OCT) and SS-OCT angiography (SS-OCTA) findings in circumscribed choroidal hemangioma (CCH) before and after treatment with transpupillary thermotherapy (TTT). METHODS: The clinical records of 21 eyes having CCH imaged with SS-OCT/SS-OCTA between September 2018 and December 2022 were evaluated. RESULTS: SS-OCT examination in CCH showed dome-shaped appearance (100%), choroidal shadowing (100%), expansion of choroidal structures (100%), subretinal fluid (66.7%), intraretinal edema/schisis (33.3%), retinal pigment epithelium (RPE) atrophy (19.0%), hyperreflective dots (19.0%), and epiretinal membrane (4.8%). Internal arborizing tumor vessels showing hyperreflectivity were observed in the choriocapillaris slab on SS-OCTA in all eyes. In the deep capillary plexus (DCP), flow void changes were seen in 7 eyes with intraretinal schisis/cystoid macular edema. Four CCHs > 2 mm in thickness showed outer retinal involvement due to unmasking of flow in intratumoral vessels related to RPE atrophy. Following TTT/indocyanine green-enhanced TTT (ICG-TTT) of CCH, SS-OCT findings included total/partial resolution of subretinal fluid (57.1%), complete/partial regression of the tumor (52.4%), and RPE atrophy (33.3%). After treatment; loss of choriocapillaris, decrease in tumor vascularity together with increase in the fibrous component and flow void areas were detected on SS-OCTA. CONCLUSIONS: SS-OCT/SS-OCTA are useful non-invasive tools for imaging the structural/vascular changes in CCHs managed with TTT or ICG-TTT. On SS-OCTA, hyporeflective spaces localizing to edema/schisis in the DCP and arborizing tumor vessels within a hyporeflective stromal background in the choriocapillaris slab were observed. After TTT/ICG-TTT, a decrease in tumor vessels and an increase in the fibrous component and flow-void areas inside the CCH were detected on SS-OCTA.
Subject(s)
Choroid Neoplasms , Hemangioma , Hyperthermia, Induced , Tomography, Optical Coherence , Humans , Tomography, Optical Coherence/methods , Choroid Neoplasms/therapy , Choroid Neoplasms/diagnostic imaging , Choroid Neoplasms/pathology , Female , Middle Aged , Male , Hemangioma/therapy , Hemangioma/diagnostic imaging , Hemangioma/pathology , Adult , Hyperthermia, Induced/methods , Aged , Fluorescein Angiography/methods , Retrospective Studies , Choroid/diagnostic imaging , Choroid/blood supply , Choroid/pathologyABSTRACT
OBJECTIVES: Differentiation of iris and ciliary body lesions as benign or malignant and cystic or solid is important. The aim of this study was to compare anterior segment swept-source optical coherence tomography (AS SS-OCT) and ultrasound biomicroscopy (UBM) findings in iris and ciliary body tumors. RESEARCH DESIGN AND METHODS: Forty-two eyes of 38 cases with iris and ciliary body tumors imaged with UBM and AS SS-OCT between September 2018 and September 2023 were evaluated retrospectively. RESULTS: Of 42 eyes, 14 had melanoma, 14 iris pigment epithelial (IPE) cysts, 7 nevi, 3 Lisch nodules, 2 iris stromal cysts, 1 pars plana cysts, and 1 iris mammillations. An equivalent (100%) visualization of the anterior tumor margin was obtained with both techniques. Compared to AS SS-OCT, UBM was superior for posterior margin visualization in melanocytic tumors and IPE cysts. Bland-Altman plots demonstrated good agreement between UBM and AS SS-OCT for melanocytic tumors < 2.5 mm in base diameter and < 2 mm in thickness. CONCLUSIONS: Although, UBM is the gold standard for ciliary body and iridociliary tumors. AS SS-OCT should be considered as an excellent alternative to UBM, especially in minimally elevated iris lesions.
Subject(s)
Ciliary Body , Microscopy, Acoustic , Tomography, Optical Coherence , Humans , Tomography, Optical Coherence/methods , Microscopy, Acoustic/methods , Ciliary Body/diagnostic imaging , Ciliary Body/pathology , Male , Female , Middle Aged , Adult , Retrospective Studies , Aged , Uveal Neoplasms/diagnostic imaging , Uveal Neoplasms/pathology , Anterior Eye Segment/diagnostic imaging , Anterior Eye Segment/pathology , Iris Neoplasms/diagnostic imaging , Young Adult , Iris Diseases/diagnostic imaging , Iris/diagnostic imaging , Iris/pathology , AdolescentABSTRACT
Sclerochoroidal calcification (SCC) is a rare disease which is characterized by calcium deposition in the sclera. The choroid is secondarily involved. Typical localization is in the midperipheral region, outside the vascular arcades. SCC is mostly located in the superotemporal quadrant. Often times, the patients are referred with the diagnosis of an amelanotic tumor. SCC may be dystrophic or metastatic. Metastatic SCC lesions are associated with conditions altering calcium and phosphate metabolism including primary and secondary hyperparathyroidism, vitamin D intoxication, renal failure, hyperphosphatemia, and destructive bony lesions. SCC lesions have a characteristic appearance and appear as distinct, ill-defined, yellow-white, elevated scleral/choroidal masses funduscopically. The purpose of this literature review is to review the current knowledge on SCC, highlight the imaging features, and discuss the differential diagnosis as well as management options.
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PURPOSE: To evaluate clinical and demographic characteristics and factors affecting recurrence, metastasis, and survival in conjunctival melanoma (CM). METHODS: The clinical records of 45 patients who were treated for CM between October 1998 and June 2022 were retrospectively evaluated. Age, gender, presence of underlying conjunctival nevus-primary acquired melanosis (PAM), tumor stage according to the 8th edition of the American Joint Committee on Cancer (AJCC) staging system, tumor basal diameter, tumor thickness, lymph node (LN) involvement, metastasis, presence of tumor at the surgical margin, treatment method, need for adjuvant therapy, local tumor control, recurrence, and survival were recorded. RESULTS: Twenty-one (46.7%) patients were female and 24 (53.3%) patients were male. The mean age at diagnosis was 53.2 ± 16.1 years. Median follow up time was 12 (1-300) months. Fifteen (33.3%) patients had conjunctival PAM; 2 (4.4%) patients had conjunctival nevus. The tumor stage was T1 in 24 (55.8%), T2 in 13 (30.2%), and T3 in 6 (14.0%) of the cases. The T stage in 2 cases could not be determined. For stage T1 and T2 CM, in addition to excisional biopsy (EB) and cryotherapy, alcohol epitheliectomy (AE) was performed in 17 cases (37.8%), superficial sclerectomy (SS) was performed in 7 (15.6%), and amnion membrane transplantation (AMT) due to a large conjunctival defect in 9 (20.0%). Six (14.0%) T3 cases underwent primary exenteration. Positive surgical margins were observed in 23 (51.1%) of the excised tumors at histopathologic examination. Adjuvant topical mitomycin-C (MMC) was used in 7 (30.4%) and strontium-90 episcleral brachytherapy in 4 (17.4%) of the 23 cases with tumor-positive borders. During the follow-up, recurrence was seen in 14 (31.1%) cases. According to Kaplan Meier analysis, the mean time to recurrence development was 90.5 ± 16.1 months and the 5-year recurrence free rate was 52.0%. Fourteen of the recurrent cases underwent EB + cryotherapy, 3 underwent AE + SS, and 3 underwent secondary exenteration. Metastasis and LN involvement occurred in 11 (24.4%) and 8 (17.8%) of the cases, respectively. Four (8.9%) cases expired during follow-up. According to Kaplan-Meier analysis, the mean time to metastasis was 106.2 ± 17.3 months and the 5-year metastasis free rate was 52.0%. While recurrence was more frequent in CM developing from PAM/nevus, metastasis was more frequent in men and those with LN involvement. CONCLUSION: Conjunctival melanoma was a malignant tumor with high recurrence and metastasis rates. Precursor nevus/PAM is a risk factor for recurrence, while male gender and regional LN involvement were risk factors for metastasis in this study.
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Bone Neoplasms , Conjunctival Neoplasms , Melanoma , Nevus , Skin Neoplasms , Humans , Female , Male , Adult , Middle Aged , Aged , Retrospective Studies , Melanoma/diagnosis , Melanoma/therapy , Conjunctival Neoplasms/therapyABSTRACT
Limbal dermoid is a congenital benign tumor of the limbus which is often managed by surgery if necessary. In dermoid lesions involving the deep stroma, tumor excision and reconstruction of the anterior segment with amniotic membrane transplantation or keratoplasty may be required. Herein, we present a case of deep limbal dermoid treated with surgical resection and lamellar keratoplasty using microkeratome-assisted anterior lamellar graft.
Subject(s)
Corneal Transplantation , Dermoid Cyst , Eye Neoplasms , Humans , Dermoid Cyst/diagnosis , Dermoid Cyst/surgery , Dermoid Cyst/pathology , Eye Neoplasms/diagnosis , Eye Neoplasms/surgery , Amnion/transplantationABSTRACT
PURPOSE: To report a rare case of atopic keratoconjunctivitis (AKC) presenting with bilateral corneal panni associated with limbal inclusion cysts in the left eye. MATERIALS & METHODS: Retrospective case report. RESULTS: A 19-year-old female with AKC presented with bilateral corneal panni and limbal inclusion cysts in the left eye. Anterior segment swept-source optical coherence tomography showed bilateral hyperreflective epicorneal membrane and a lobulated cystic lesion in the left eye. Ultrasound biomicroscopy demonstrated a dense membrane overriding the cornea in both eyes and hyporeflective spaces separated by medium reflective septa in the cyst. The patient underwent excision of the limbal inclusion cyst and pannus in the left eye. Histopathological examination revealed subepithelial cystic lesion surrounded by non-keratinizing epithelium; areas of acanthosis, hyperkeratosis, parakeratosis, and hyperplasia in the epithelium of the pannus; as well as inflammation, fibrosis, and increase in vascularization in the stroma. CONCLUSION: To our knowledge, this is the first case of corneal pannus associated with limbal inclusion cysts in AKC. Surgical excision was done to establish the diagnosis as well as to improve vision in our case.
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PURPOSE: To report RetCam fluorescein angiographic (FA) changes and correlation with clinical findings in the follow-up of retinoblastoma (RB) after frontline intravenous chemotherapy and other treatments. METHODS: Patients having RB who underwent post-treatment RetCam fundus photography and FA under general anesthesia between February 2020 and February 2022 were retrospectively analyzed. RESULTS: 78 eyes of 70 patients with RB were included. In 55 (70.5%) eyes with type 1, 2 or 3 regression patterns, the main tumor started to show hyperfluorescence in arterial phase in 24 eyes (43.6%), in early venous phase in 24 (43.6%) eyes, and in late venous phase in 7 (12.7%) eyes. Of thirty-six (46.2%) eyes with chorioretinal scars developing after focal treatments (cryotherapy and transpupillary thermotherapy), window defects and visible choroidal vessels were found in all eyes, scleral staining in 10 (27.8%), and gliosis on scar tissue in 8 (22.2%). Vitreous seeds showed hyperfluorescence if they were calcified (6 eyes, 7.7%) and hypofluorescence if non-calcified (11 eyes, 14.1%). Retrohyaloid seeds (11 eyes, 14.1%) were hypo/isofluorescent while subretinal seeds (6 eyes, 7.7%) were hyperfluorescent. Leakage from peripheral retinal vessels was detected in 14 (17.9%) eyes and non-perfusion in 6 (7.7%) eyes. CONCLUSION: RetCam wide-angle FA is useful to evaluate the tumor and associated vascular and retinal changes after treatment in RB. Regressed tumors demonstrate later fluorescein uptake in venous phases. Fluorescein angiographic changes in chorioretinal scars include window defects, visibility of choroidal vessels, scleral staining, and leakage from vessels. Retinal vascular leakage and peripheral non-perfusion can be seen in eyes with regressed stable tumors.
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PURPOSE: To determine the effect of patient and tumor features and different treatments on eye removal (enucleation or exenteration) and metastasis in posterior uveal melanoma (PUM). METHODS: Retrospective analysis. Patient age (≤60 vs >60 years), sex (female vs male), visual acuity (VA, ≤20/40 vs >20/40), largest tumor basal diameter (LTBD), tumor thickness, tumor stage according to American Joint Committee on Cancer (AJCC) 8th edition, ciliary body involvement, distance to optic disc (OD)/fovea (≤3â mm vs >3â mm), OD involvement, and histopathology were evaluated. Primary treatment options were transpupillary thermotherapy, plaque radiotherapy, Cyberknife radiosurgery, exoresection, and eye removal. Risk factors for primary eye removal were determined using logistic regression test and those for secondary eye removal and metastasis with Cox regression analysis. RESULTS: Of 387 cases, 153 (39.5%) underwent primary eye removal. Multivariable risk factors for primary eye removal included AJCC tumor stage (p = 0.001, OR:4.586; p < 0.001, OR:34.545; p < 0.001, OR:103.468 for stages T2, T3, and T4 vs stage T1, respectively), and VA≤20/40 (p = 0.014, OR:2.597). Multivariable risk factors for secondary eye removal were VA≤20/40 (p = 0.019, RR:2.817) and AJCC stage T3 vs T1 (p = 0.021, RR:2.666). Eye preservation rates in patients undergoing eye-conserving treatments were 80.3%, 69.6%, and 51.5% at 5, 10, and 15 years, respectively. Metastasis-free survival rates were 81.0%, 73.0%, and 56.7% at 5, 10, and 15 years, respectively. Multivariable risk factors for metastasis included eye removal as primary treatment (p = 0.005, RR:2.828) and mixed type histopathology (p < 0.001, RR:4.804). DISCUSSION: Early diagnosis is crucial for both eye preservation and survival in PUM. Increasing AJCC tumor stage and lower VA were risk factors for eye removal in this study. Mixed type histopathology and primary eye removal were risk factors for metastasis.
Subject(s)
Brachytherapy , Melanoma , Uveal Neoplasms , Humans , Male , Female , Middle Aged , Retrospective Studies , Uveal Neoplasms/surgery , Eye Enucleation , Melanoma/surgery , Melanoma/pathologyABSTRACT
Optical coherence tomography (OCT) is a non-invasive method that provides the opportunity to examine tissues by taking cross-sectional images. OCT is increasingly being used to evaluate anterior segment (AS) pathologies. Swept-source (SS) OCT allows greater penetration and achieves better visualization of the internal configuration of AS tissues due to the longer wavelength employed and high scan speeds. We reviewed the utilization of AS SS-OCT in various conditions including glaucoma, ocular surface pathologies, iris tumors, refractive surgery, cataract surgery, and scleral diseases. A systematic literature search was carried out on PubMed, Scopus, and Web of Science databases between January 1, 2008, and September 1, 2022 using the following keywords: AS SS-OCT; dry eye and SS-OCT; ocular surface and SS-OCT; cornea and SS-OCT; dystrophy and SS-OCT; glaucoma and SS-OCT; ocular surface tumors and SS-OCT; conjunctival tumors and SS-OCT; refractive surgery and SS-OCT; cataract and SS-OCT; biometry and SS-OCT; sclera and SS-OCT; iris and SS-OCT; ciliary body and SS-OCT; artificial intelligence and SS-OCT. A total of 221 studies were included in this review. Review of the existing literature shows that SS-OCT offers several advantages in the diagnosis of AS diseases. Exclusive features of SS-OCT including rapid scanning, deeper tissue penetration, and better image quality help improve our understanding of various AS pathologies.
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Cataract , Glaucoma , Photochemotherapy , Humans , Tomography, Optical Coherence/methods , Artificial Intelligence , Photochemotherapy/methods , Photosensitizing Agents , Glaucoma/diagnostic imagingABSTRACT
This study aims to systematically review the reported literature on the use of anterior segment optical coherence tomography (AS-OCT) in ocular surface tumours and simulating lesions. A systematic literature search was done using PubMed, Scopus, and Web of Science databases between January 2002 and December 2021. On AS-OCT, ocular surface squamous neoplasia typically demonstrate epithelial thickening, epithelial hyperreflectivity, and an abrupt transition between normal and abnormal epithelium. Conjunctival nevi usually show mildly hyperreflective epithelium of normal thickness, internal hyperreflectivity, and intralesional cysts which is the hallmark of this tumour. Primary acquired melanosis presents with normal thickness epithelium, basal epithelial hyperreflectivity, and absence of cysts. Conjunctival melanoma demonstrates hyperreflective normal/thickened epithelium, hyperreflective basal epithelium, internal hyperreflectivity, and absence of intralesional cysts. Conjunctival lymphoma shows homogenous, low-medium reflective subepithelial lesions with smooth borders, and dot-like infiltrates. Benign reactive lymphoid hyperplasia findings are similar to lymphoma but the infiltrates are more hyperreflective compared to lymphoma. Pterygium shows thickened conjunctival epithelium, epithelial hyperreflectivity, and subepithelial wedge-shaped hyperreflective tissue separated from the overlying epithelium by a cleavage plane. Pinguecula demonstrates mildly thickened epithelium and similar findings with pterygium but does not extend beyond the corneal limbus. This review shows that AS-OCT, as a noninvasive tool, has potential uses in the differential diagnosis of ocular surface tumours and simulating lesions. Major limitations of AS-OCT include limited visualization of the posterior border of thick, keratinized, and pigmented tumours and lack of assessment of large conjunctival tumours in a single cut.
Subject(s)
Conjunctival Neoplasms , Corneal Diseases , Cysts , Eye Neoplasms , Lymphoma , Pterygium , Humans , Pterygium/pathology , Corneal Diseases/pathology , Tomography, Optical Coherence/methods , Eye Neoplasms/diagnostic imaging , Conjunctival Neoplasms/diagnostic imaging , Conjunctival Neoplasms/pathologyABSTRACT
PURPOSE: To report wide-field fundus imaging and fluorescein angiography findings in conditions mimicking retinoblastoma (pseudoretinoblastoma). METHODS: The clinical and imaging records of 28 patients (36 eyes) imaged with RetCam 3 (Clarity Medical Systems, Inc) wide-field fundus photography and fluorescein angiography who were diagnosed as having various pseudoretinoblastoma disorders between February 2020 and August 2021 were retrospectively evaluated. RESULTS: Most patients were referred with suspicion of retinoblastoma. Other patients had unresolved eye conditions and received initial treatment elsewhere and they were referred later for exclusion of retinoblastoma. On RetCam fundus photography and fluorescein angiography, pseudoretinoblastoma conditions were diagnosed as Coats disease (9 eyes), congenital glaucoma (3 eyes), persistent fetal vasculature (3 eyes), familial exudative vitreoretinopathy (2 eyes), retinopathy of prematurity (2 eyes), myelinated retinal nerve fibers (2 eyes), optic nerve hypoplasia (2 eyes), oculocutaneous albinism (2 eyes), meridional fold (2 eyes), combined hamartoma of retina and retinal pigment epithelium (2 eyes), grouped congenital hypertrophy of the retinal pigment epithelium (1 eye), retinal astrocytic hamartoma (1 eye), morning glory syndrome (1 eye), optic glioma (1 eye), giant choroidal nevus (1 eye), vasculitis (1 eye), and chorioretinitis (1 eye). RetCam imaging and fluorescein angiography demonstrated telangiectasias, microaneurysms, submacular exudation/fibrosis, capillary non-perfusion, and exudation posterior to telangiectasias in Coats disease. Hypofluorescent optic discs, peripheral non-perfusion, and vascular leakage were observed in congenital glaucoma. Large areas of capillary non-perfusion, poor foveal morphology/formation, elongated ciliary processes with hyperfluorescent central core, and hyperfluorescent fibrovascular stalk at the posterior pole were hallmarks of persistent fetal vasculature. CONCLUSIONS: RetCam wide-field fundus imaging and fluorescein angiography play an important role in the diagnosis of conditions simulating retinoblastoma. [J Pediatr Ophthalmol Strabismus. 2023;60(1):60-74.].
Subject(s)
Hydrophthalmos , Persistent Hyperplastic Primary Vitreous , Retinal Neoplasms , Retinal Telangiectasis , Retinoblastoma , Infant, Newborn , Humans , Fluorescein Angiography/methods , Retrospective Studies , Persistent Hyperplastic Primary Vitreous/diagnosis , Retinal Neoplasms/diagnosisABSTRACT
PURPOSE: To investigate the distribution of pseudoretinoblastoma (PSRB) cases based on gender, age, and laterality. MATERIALS AND METHODS: The clinical records of 607 patients (851 eyes) who were referred for diagnosis of retinoblastoma or simulating conditions between October 1998 and May 2021 were retrospectively evaluated. Patients were stratified by age as follows: ≤1 year, >1-3 years, >3-5 years, and >5 years. RESULTS: Of 190/607 PSRB patients, 129 (67.9%) were males and 61 (32.1%) females (p = 0.001). The 3 most common diagnoses in males were Coats disease (20.2%), persistent fetal vasculature (PFV, 14.0%), and chorioretinal coloboma (6.2%). In females, the 3 most common diagnoses included PFV (21.3%), retinal dysplasia, congenital glaucoma, and combined hamartoma (each 6.6%). PFV was the most common diagnosis in ≤1 year old patient group (26.6%). Coats disease and PFV were the most common diagnoses in >1-3 years old patient group (each 16.7%). Coats disease was the most common diagnosis in >3-5 years old (30.8%) and >5 years old patient groups (13.1%). PSRBs were unilateral in 121/190 (63.7%) patients. Coats disease usually presented unilaterally (p < 0.001) while PFV, optic nerve head drusen, and retinopathy of prematurity as bilateral diseases (p = 0.019, p = 0.001, and p = 0.001 respectively). DISCUSSION: PSRB diagnoses show differences depending on gender, age, and laterality. In our study, the most common PSRB lesions were Coats disease in males and PFV in females. PFV was the most frequent diagnosis in ≤3 years and Coats disease in >3 years of age groups. Coats disease and PFV were the most common unilateral and bilateral PSRB diagnoses respectively.
Subject(s)
Retinal Neoplasms , Retinal Telangiectasis , Retinoblastoma , Infant , Infant, Newborn , Male , Female , Humans , Child, Preschool , Retrospective Studies , Retinoblastoma/diagnosis , Retinoblastoma/epidemiology , Vitreous Body/pathology , Retinal Neoplasms/diagnosis , Retinal Neoplasms/epidemiologyABSTRACT
Hamartomas are local malformation of cells that demonstrate abnormal proliferation in the area where they are normally present. Retinal and optic disc hamartomas include astrocytic hamartoma, congenital hypertrophy of the retinal pigment epithelium (CHRPE), simple congenital hamartoma of the retinal pigment epithelium (CSHRPE), combined hamartoma of the retina and retinal pigment epithelium (CHRRPE), retinal hemangioblastoma (retinal capillary hemangioma), and retinal cavernous hemangioma. Retinal and optic disc hamartomas can be observed sporadically as well as with systemic associations. Astrocytic hamartoma usually appears as a flat, transparent yellowish lesion. CHRPE is a round, pigmented, and flat lesion. CSHRPE usually presents as a dark black macular tumor. CHRRPE consists of vascular, glial, and pigment epithelial components, which can demonstrate peripapillary, macular, and peripheral localization. Retinal hemangioblastoma is a vascular tumor, red-pink in color with tortuous and dilated afferent and efferent vessels, typically located in the peripheral retina or optic disc. Retinal cavernous hemangioma is characterized by the formation of thin-walled saccular angiomatous structures in the retina or optic nerve head resembling concord grapes. Ultrasonography, fundus autofluorescence, optical coherence tomography, optical coherence tomography angiography, and fluorescein angiography methods are used in the diagnosis of retinal and optic disc hamartomas. Some retinal and optic disc hamartomas do not require treatment. However, complications including vitreous hemorrhage, macular exudation, retinal detachment, macular hole, epiretinal membrane, and choroidal neovascularization require treatment.
Subject(s)
Eye Abnormalities , Eye Neoplasms , Hamartoma , Hemangioblastoma , Hemangioma, Cavernous , Optic Disk , Retinal Diseases , Retinal Neoplasms , Humans , Hemangioblastoma/diagnosis , Hemangioblastoma/complications , Retina , Retinal Diseases/diagnosis , Retinal Neoplasms/diagnosis , Hamartoma/diagnosis , Hamartoma/complications , Hemangioma, Cavernous/complicationsABSTRACT
We report a case of bilateral optic nerve head drusen complicated by choroidal neovascularization (CNV) in the left eye at presentation. The presence of optic disc and macular edema in addition to exudation led to the misdiagnosis of neuroretinitis at an outside medical center. Swept-source optical coherence tomography (SS-OCT) and SSOCT angiography were critical in establishing the diagnosis and follow-up in a noninvasive manner. Secondary CNV associated with optic nerve head drusen responded well to intravitreal injections of anti-vascular endothelial growth factor in the left eye. Asymptomatic nonexudative CNV that developed in the right eye during follow-up regressed spontaneously without treatment. [Ophthalmic Surg Lasers Imaging Retina 2022;53:518-521.].
Subject(s)
Chorioretinitis , Choroidal Neovascularization , Optic Disk Drusen , Optic Disk , Papilledema , Retinitis , Chorioretinitis/complications , Choroidal Neovascularization/diagnosis , Choroidal Neovascularization/drug therapy , Choroidal Neovascularization/etiology , Diagnostic Errors/adverse effects , Endothelial Growth Factors , Humans , Optic Disk Drusen/complications , Optic Disk Drusen/diagnosis , Papilledema/complications , Papilledema/etiology , Retinitis/complicationsABSTRACT
A 71-year-old male presented with painless and slowly progressive proptosis of the right eye. The patient had no history of cancer and was in good health. Orbital magnetic resonance imaging showed a well-demarcated, slightly lobulated intraconal orbital mass hypo-isointense on T1- weighted and isointense on T2-weighted images, demonstrating contrast enhancement in the right orbit. The lesion had a central cavitary region. An anterior orbitotomy with lower lid crease incision was performed and the lesion was excised in toto. Histopathologic examination revealed diffuse infiltration of monomorphic population of small lymphoid cells. Immunohistochemical staining demonstrated diffuse and strong positivity for CD20, CD5, and cyclin D1. Histopathological and immunohistochemical findings were consistent with mantle cell lymphoma. Positron emission tomography-computed tomography did not show systemic involvement. The patient underwent orbital external beam radiotherapy (36 Gy). There was no recurrence and/or systemic involvement at 2.5 years follow-up.
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PURPOSE: We report a case of idiopathic sclerochoroidal calcification (SCC) evaluated with multimodal imaging including swept source optical coherence tomography angiography (SS-OCTA). CASE PRESENTATION: A 52-year-old man was diagnosed with sclerochoroidal calcification in the left eye. Multimodal imaging revealed mixed hypoautofluorescence and hyperautofluorescence on fundus autofluorescence (FAF) imaging; an acoustically solid lesion with hyperreflective deposits on ultrasonography (USG); a rocky configuration with marked disruption of outer retinal layers and abrupt transition from normal to tumor tissue on swept source optical coherence tomography (SS-OCT); hyporeflective plexus in the outer retinal and choroidal slabs and a retinochoroidal shunt vessel on SS-OCTA; and central hypofluorescence with intervening patchy hyperfluorescent areas, diffuse peripheral window defects, and a retinochoroidal shunt vessel on fluorescein angiography (FA). Systemic evaluation including serum parathyroid hormone, calcium, phosphorus, albumin levels, and urine analysis were normal. CONCLUSION: Sclerochoroidal calcification is a rare ocular tumor that may be idiopathic or secondary to several nephrological or endocrinological diseases involving calcium and phosphorus mechanism. Multimodal imaging with FAF, USG, OCT, OCTA, and FA help to identify the distinguishing features of the tumor and differentiate it from other entities.