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Objective: To present the clinical features of retinal astrocytic hamartoma (RAH) based on the acquired experience Methods: Medical records were analyzed on 10 patients diagnosed with RAH at the S. Fyodorov Eye Microsurgery Federal State Institution, Moscow, Russia, between 2013 and 2023. The average age of the patients was 16 years (range, 1 month – 32 years). Among these patients were 6 men, 1 woman, and 3 children (1 boy and 2 girls). The patients underwent a comprehensive ophthalmological examination, including visometry, tonometry, perimetry, biomicroscopy, ophthalmoscopy, and B-scan ultrasonography to determine the location and size of the lesion, as well as optical coherence tomography (OCT) for secondary retinal detachment Results: The average visual acuity (VA) of patients at the time of diagnosis was 0.7 (range, 0.16-1.0). VA in young children was assessed by tracking moving objects. All children were able to fixate and actively track the object. Most patients (n=8; 80%) were diagnosed with RAH based on the clinical (ophthalmoscopic) picture of the fundus, ultrasound, and OCT data. The average height of the RAH was 1.7 mm (range, 1.0 to 3.2 mm), and the average length was 4.2 mm (range, 0.4 to 7.6 mm). In all cases, the B-scan results showed lesions with hyperechogenicity of high amplitude. 4 patients displayed common signs – thickening of the neuroepithelium with optically empty spaces with a lobulated, "moth-eaten" appearance on the OCT. In contrast, the remaining patients had a homogeneous hyper-reflective thickening of the neuroepithelium in the lesion area. Two patients (20%) had cystoid macular edema in the fovea and secondary flat detachment of the neuroepithelium extending from the hamartoma to the fovea Conclusion: Differential diagnosis of RAH and retinoblastoma (RB) is crucial because, if misdiagnosed, it leads to unnecessary and aggressive treatment

Abstract Aim To investigate the complete clinical spectrum of individuals with paediatric tuberous sclerosis complex in southern Sweden and explore changes over time. Methods In this retrospective observational study, 52 individuals aged up to 18 years at the study start were followed-up at regional hospitals and centres for habilitation from 2000 to 2020. Results Cardiac rhabdomyoma was detected prenatally/neonatally in 69.2% of the subjects born during the latest ten years of the study period. Epilepsy was diagnosed in 82.7% of subjects, and 10 (19%) were treated with everolimus, mainly (80%) for a neurological indication. Renal cysts were detected in 53%, angiomyolipomas in 47%, astrocytic hamartomas in 28% of the individuals. There was a paucity of standardized follow-up of cardiac, renal, and ophthalmological manifestations and no structured transition to adult care. Conclusion Our in-depth analysis shows a clear shift towards an earlier diagnosis of tuberous sclerosis complex in the latter part of the study period, where more than 60% of cases showed evidence of this condition already in utero due to the presence of a cardiac rhabdomyoma. This allows for preventive treatment of epilepsy with vigabatrin and early intervention with everolimus for potential mitigation of other symptoms of tuberous sclerosis complex.

We report a rare case of a 32-year-old Indian male who presented to the retina outpatient department with a history of sudden worsening of vision in the left eye. There was a background history of poor vision and deficient night vision since childhood. At the first presentation, the best corrected visual acuity was 6/36 and 6/60 in the right and left eye, respectively. Ocular examination revealed waxy pale disc, bone spicule pigmentation, attenuated vessels and epiretinal membrane in the right eye in keeping with retinitis pigmentosa. An astrocytic harmatoma was also present in the right eye. Vitreous haemorrhage in the left eye precluded a view of the fundus. He subsequently had a left pars plana vitrectomy, and intravitreal bevacizumab on account of non-resolving vitreous haemorrhage and a vasoproliferative tumour and astrocytic hamartoma were noticed intraoperatively. He had a good immediate post-operative outcome post-left vitrectomy but subsequently developed left neovascular glaucoma 2 years after. Neovascular glaucoma may be a sequela of vasoproliferative tumour; hence, regular follow-up and monitoring are essential in these patients.

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.

Aim: To investigate the complete clinical spectrum of individuals with paediatric tuberous sclerosis complex in southern Sweden and explore changes over time. Methods: In this retrospective observational study, 52 individuals aged up to 18 years at the study start were followed-up at regional hospitals and centres for habilitation from 2000 to 2020. Results: Cardiac rhabdomyoma was detected prenatally/neonatally in 69.2% of the subjects born during the latest ten years of the study period. Epilepsy was diagnosed in 82.7% of subjects, and 10 (19%) were treated with everolimus, mainly (80%) for a neurological indication. Renal cysts were detected in 53%, angiomyolipomas in 47%, astrocytic hamartomas in 28% of the individuals. There was a paucity of standardized follow-up of cardiac, renal, and ophthalmological manifestations and no structured transition to adult care. Conclusion: Our in-depth analysis shows a clear shift towards an earlier diagnosis of tuberous sclerosis complex in the latter part of the study period, where more than 60% of cases showed evidence of this condition already in utero due to the presence of a cardiac rhabdomyoma. This allows for preventive treatment of epilepsy with vigabatrin and early intervention with everolimus for potential mitigation of other symptoms of tuberous sclerosis complex. [ABSTRACT FROM AUTHOR]

We report a rare case of a 32‑year‑old Indian male who presented to the retina outpatient department with a history of sudden worsening of vision in the left eye. There was a background history of poor vision and deficient night vision since childhood. At the first presentation, the best corrected visual acuity was 6/36 and 6/60 in the right and left eye, respectively. Ocular examination revealed waxy pale disc, bone spicule pigmentation, attenuated vessels and epiretinal membrane in the right eye in keeping with retinitis pigmentosa. An astrocytic harmatoma was also present in the right eye. Vitreous haemorrhage in the left eye precluded a view of the fundus. He subsequently had a left pars plana vitrectomy, and intravitreal bevacizumab on account of non‑resolving vitreous haemorrhage and a vasoproliferative tumour and astrocytic hamartoma were noticed intraoperatively. He had a good immediate post‑operative outcome post‑left vitrectomy but subsequently developed left neovascular glaucoma 2 years after. Neovascular glaucoma may be a sequela of vasoproliferative tumour; hence, regular follow‑up and monitoring are essential in these patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Astrocytic hamartoma is a benign glial tumor. It may be associated with tuberous sclerosis and can also be found incidentally on retinal examination as an isolated presentation. Here, we describe multimodal imaging characteristics of astrocytic hamartoma in a patient with retinitis pigmentosa. Spectral domain optical coherence tomography of both eyes showed moth-eaten optically empty spaces and hyperreflective dots along with foveal thinning. Multicolor image highlighted mulberry appearance of the lesion with green shift signifying elevated lesion. In infrared reflectance, lesion was hyporeflective with its margins well delineated. Green reflectance and blue reflectance highlighted calcification as multiple hyperreflective dots. Autofluorescence showed typical hyperautofluorescence.

A clinical and morphological description of a case of pilocytic astrocytoma (astrocytic hamartoma) of the retina and optic nerve in an 11-year old girl is presented. The clinical and instrumental characteristics of the tumor are determined by ultrasound and MRI tests. Detailed cytological and pathomorphological description of the tumor, which is rarely observed, is given. The tumor poses a threat to the eye and requires that the eyeball be removed.

Gene- crumbs 1 –associated retinitis pigmentosa may demonstrate preservation of the para-arteriolar retinal pigment epithelium and slow progression over time. This condition may also be associated with a benign peripheral retinal tumor. Purpose: To report the multimodal imaging findings of a patient with gene- crumbs 1 –associated retinitis pigmentosa (RP) characterized by preservation of para-arteriolar retinal pigment epithelium and a peripheral retinal tumor. Methods: A 27-year-old woman was referred to our center because of progressive decreased vision in both eyes with a diagnosis of gene- crumbs 1 –associated RP. Fundus examination was remarkable for attenuated retinal vessels and bone spicule migration that was bilateral and symmetric. In addition, an elevated yellow–white mass with dilated retinal vessels was noted in the superotemporal midperiphery of the retina in the left eye without any associated exudation. Results: Diffuse retinal pigment epithelium mottling was present but spared the area along the retinal arterioles. Swept-source optical coherence tomography showed diffuse outer retinal atrophy. Optical coherence tomography angiography of the peripheral lesion illustrated extensive vascularity and a possible retinal feeder vessel communicating with the tumor at its inferior margin. The phenotype of the lesion showed overlap with a vasoproliferative tumor or an astrocytic hamartoma. Over a period of 5 years of follow-up, the peripheral tumor was unchanged. No significant progression of the peripheral retinal degeneration was evidenced by autofluorescent imaging over this time period although the central acuity continued to decrease. Conclusion: Gene- crumbs 1 –associated RP may be characterized by preservation of the para-arteriolar retinal pigment epithelium and slow progression and may also feature a benign peripheral retinal tumor. [ABSTRACT FROM AUTHOR]

A 25-year-old woman presented with a juxtafoveal, pigmented lesion in the left eye. Multimodal imaging, including optical coherence tomography angiography, allowed the diagnosis of retinal astrocytic hamartoma. The patient has been followed for 2 years without any modifications of the morphology of the lesion and of visual acuity. Purpose: To report a case of retinal astrocytic hamartoma imaged by optical coherence tomography angiography (OCTA), followed for 2 years. Methods: Observational case report. Results: A 25-year-old woman was referred for an incidental retinal lesion in the left eye (LE). At baseline, the best-corrected visual acuity in the LE was 20/32, and fundus examination showed the presence of a round, pigmented lesion in juxtafoveal region, corresponding, on spectral domain OCT, to a hyperreflective lesion within nerve fiber layer. Optical coherence tomography angiography revealed the presence of a high-flow lesion in the superficial capillary plexus segmentation. The patient was followed up for 2 years: best-corrected visual acuity remained stable and multimodal imaging, including OCTA, confirmed the benign and stable nature of the lesion. At baseline, the total lesion area on OCTA (superficial capillary plexus) was 0.181 mm2, whereas vascular density was 52.080%; the total area was 0.204 mm2, and vascular density was 53.740% at 2-year follow-up. Conclusion: Optical coherence tomography angiography is helpful not only for the diagnosis and follow-up of such rare tumors, but also it gives insights as to how these tumors develop and how they affect surrounding structures. [ABSTRACT FROM AUTHOR]

Congenital disorder of glycosylation-Ia (CDG-Ia) is a rare autosomal recessive genetic disorder, characterized by systemic and ophthalmological abnormalities. Here, we report multiple retinal astrocytic hamartomas as a new retinal finding in an adolescent affected by congenital disorder of CDG-Ia. A 15-year-old boy affected by CDG-Ia underwent full ophthalmic examination, full field electroretinography (ERG) evaluation and retinal multimodal imaging, including: fundus photography, spectral domain optical coherence tomography (SD-OCT) and blue fundus autofluorescence (FAF). Blue FAF showed multiple papillary and iuxtapapillary bilateral hyper-FAF lesions, corresponding to hyperreflective thickening of the retinal nerve fiber layer, with internal optical empty spaces and posterior dense optical shadowing at SD-OCT. These imaging findings were consistent with retinal astrocytic hamartomas. Scotopic ERG response was significantly reduced in both eyes. Macular edema and absence of the retinal outer segments layer were also detectable. Retinal multi-modal imaging provides additional insights about retinal involvement of patients affected by CDG-Ia. In particular, this case shows the presence of multiple retinal astrocytic hamartomas.

A 24-year old female patient presented to our clinic with bilateral visual impairment since birth. Best corrected visual acuity was light perception in both eyes. At fundus examination there were mulberry-like, yellowish-white nodules along the peripapillary margins. There was attenuation of retinal vasculature, bone-spicule-like pigment deposition in the general fundus, macular edema in the right eye and macular atrophy in the left eye. Fundus autofluorescence (FAF) imaging of the bilateral peripapillary retina and optic nerve head showed increased autofluorescence. It is important to clinically detect retinal astrocytic hamartoma (RAH). It may be with Leber's Congenital Amaurosis and optic nerve drusen. [ABSTRACT FROM AUTHOR]

Michael J Wan,1 Ka Lo Chan,1,2 Benjamin G Jastrzembski,1 Asim Ali11Department of Ophthalmology and Vision Sciences, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada; 2School of Medicine, Griffith University, Brisbane, QLD, AustraliaAbstract: Tuberous sclerosis complex (TSC) is a complex, multi-system disorder with a well-described underlying genetic etiology. While retinal findings are common in TSC and important in establishing the diagnosis, TSC also has many potential neuro-ophthalmology manifestations. The neuro-ophthalmology manifestations of TSC can have a significant impact on visual function and are sometimes a sign of serious neurological disease. The purpose of this review is to describe the neuro-ophthalmological manifestations of TSC. These manifestations include optic nerve hamartomas, elevated intracranial pressure, cranial nerve palsies, cortical visual impairment, visual field deficits, and ocular toxicity from vigabatrin treatment of infantile spasms. It is important to be aware of potential neuro-ophthalmological manifestations in these patients in order to detect signs of vision- or life-threatening disease and to optimize visual function and quality-of-life.Keywords: astrocytic hamartoma, cortical visual impairment, intracranial pressure elevation, visual field defect, vigabatrin

Víctor Manuel Asensio-SánchezOphthalmology Department, Clinical University Hospital of Valladolid, Valladolid, SpainAbstract: A 56-year-old woman had an yellow-white retinal lesion superior to the optic disc. Optical coherence tomography demonstrated the mass with a snowball configuration and smooth surface. Autofluorescence disclosed revealed moderate hypoautofluorescence. Ultrasonography showed no calcification. Visual field examination showed an enlargement of the blind spot corresponding to the predominantly superotemporal juxtapapillary extension of the lesion. Eight months later, the lesion spontaneously resolved. Presumed solitary circumscribed retinal astrocytic proliferation (PSCRAP) is a benign stable retinal tumor, but PSCRAP has been reported to resolve spontaneously here. It differs from other white or yellow-white lesions of the retina in important ways that enable the ophthalmologist to reassure the patient as to its benign prognosis.Keywords: presumed solitary circumscribed retinal astrocytic proliferation, benign lesion, astrocytes, acquired retinal astrocytoma, astrocytic hamartoma, spontaneous regress

Purpose: To evaluate the findings of astrocytic hamartoma in the setting of gyrate atrophy, including details of optical coherence tomography angiography (OCTA). Observations: Multimodal imaging was obtained on a 20-year-old woman with genetically-confirmed gyrate atrophy. Dilated fundus exam was performed, followed by ultra-widefield color and green autofluorescence imaging and OCTA of bilateral peripapillary and optic disc lesions. Clinical and imaging findings were consistent with gyrate atrophy. The bilateral peripapillary and optic disc lesions had a glistening, translucent, and mulberry-like appearance. OCTA imaging of these lesions clearly demonstrated an intrinsic vascular network and hyporeflective spaces within the lesion, which could not be seen on routine examination. Conclusions and importance: OCTA was used to noninvasively diagnose astrocytic hamartoma in this patient with gyrate atrophy by showing the intrinsic vasculature and hyporeflective spaces of the lesion. This imaging modality can help differentiate astrocytic hamartoma from other lesions that typically lack intrinsic vascularity, such as optic disc drusen.

Tuberosclerosis is a multisystemic phakomatosis originating from embryonic ectoderm characterized by presence of hamartomatous tumours and central nervous system, skin, visceral organs and eye findings. The aim of this case report is detailed description of systemic and retinal findings of the disease with the help of investigations. The patient was a 4-year-old male under vigabatrin treatment and follow-up by neurology department of pediatrics for epileptic seizures. The patient was consulted to our outpatient clinic for control. Ophtalmologic examination showed visual acuities as 8/10 in both eyes. Chorioretinal depigmented areas were observed on the periphery of the fundus in both eyes. Fundus examination of the right eye showed an astrocytic hamartoma on the inferior temporal arch which was bulgy, hypopigmented, with the size of two optic discs and with no calcifications. Dilated venous vessels were observed on the proximal of this lesion. Also two astrocytic hamartomas with the size of half-disc were observed on the nasal of optic disc. OCT showed surface bulging on hamartomatous lesion, hyper-reflectance and disorganization on the inner retinal layers and shading on the outer retinal layers. In the management of tuberous sclerosis, genetic counseling and multidisciplinary follow up are necessary as the disease is multisystemic. [ABSTRACT FROM AUTHOR]

Background: To report a coincidentally discovered retinal astrocytic hamartoma in an asymptomatic older woman with no history of tuberous sclerosis or neurofibromatosis. Methods: A 63-year-old Turkish woman underwent routine ophthalmic examination. Results: Visual acuity was 20/25 in each eye. On fundus examination, the right eye showed a flat yellow-white superficial retinal lesion in the inferotemporal macula and measuring 1 mm in diameter. The lesion was unmeasurable using B-mode ultrasonography. Spectral domain optical coherence tomography revealed the mass with thickening in the retinal nerve fiber layer, with minor disorganization of the underlying retina and no retinal or vitreoretinal traction. Fluorescein angiography disclosed the mass as early isofluorescence with late mild hyper fluorescence, without leakage. Findings in the left eye were normal. Based on the clinical and imaging results, a diagnosis of noncalcified small retinal astrocytic hamartoma was rendered. The lesion remained unchanged at 1-year follow-up. Conclusion: Small coincidentally discovered retinal astrocytic hamartomas in older patients can be difficult to diagnose and possibly cause diagnostic confusion with other conditions including myelinated nerve fiber and cotton wool spot. Retinal astrocytic hamartoma is characterized by thickening of the retinal nerve fiber layer and mild compression and disorganization of the underlying retinal layers on spectral domain optical coherence tomography. [ABSTRACT FROM AUTHOR]

Background: Some diseases that affect the visual system may show loss of chromatic-achromatic sensitivity before obvious physical signs appear in the usual examination of the eye′s posterior segment. A perimetric study has been conducted with four typical patients with glaucoma and diabetes, at different stages of the disease. Materials and Methods: In addition to the standard white-on-white (standard automated perimetry [SAP]), a test battery has been used to study patient′s contrast sensitivity, using stimuli with different chromatic, spatial, and temporal content (multichannel perimetry). The choice of stimuli tries to maximize the response of different visual mechanisms: Achromatic (parvocellular and magnocellular origin); chromatic red-green (parvocellular origin); and chromatic blue-yellow (koniocellular origin). Results: The results seem to indicate losses in the achromatic-parvocellular perimetry and both chromatic perimetry tests, undetected by conventional SAP. Conclusions: Our results illustrate that our patients without visible retinal alterations show signs of suspicion in multichannel perimetry.

Context: Sunglasses should follow minimum requirements to sufficiently protect eyes. It is not known whether all items obtainable from the market are appropriately designed. Aims: To compare ultraviolet (UV)-protective properties of commercially available sunglasses obtained from authorized and unauthorized Iranian sellers. Settings and Study Design: An analytic-descriptive study performed in a metropolitan area (Tehran). Materials and Methods: Using a UV-visible standard spectrophotometer, the percentage transmittance was scanned between 280 and 400 nm in 348 pairs of nonprescription sunglasses (price range: 20-80 US$) obtained anonymously and randomly from authorized (permitted by the Ministry of Health, 189 pairs) and unauthorized (159 pairs) sellers in the Iranian capital city, Tehran. The Australian/New Zealand Standard (AS/NZS) and the American National Standards Institute [ANSI] standards were followed. Statistical Analysis: Chi-square test, independent samples t-test or Mann-Whitney U-test. Results UV-protective properties of the sunglasses obtained from authorized sellers complied with AS/NZS and ANSI guidelines in 92.6% and 95.2% of items, respectively. The corresponding rates for sunglasses obtained from unauthorized sellers were 0% and 8.2%, respectively (P < 0.001 for both). The rate of defective polarizing capability of lenses was 27.4% in sunglasses obtained from authorized sellers versus 90.4% in sunglasses obtained from unauthorized sellers (P < 0.001). Neither brand nor price played significant contributions to UV protection/lens polarizing capability of sunglasses obtained from authorized sellers. Conclusions: Sunglasses provided by unauthorized sellers are alarmingly unreliable and could be potentially hazardous for the eye. Brand and price do not guarantee optimal protection against UV radiation or polarizing performance of the lens.

Targeted therapy in retinoblastoma (RB) is widely accepted as the current management tool with an aim of increasing drug availability at the tumor location. Inevitably the effect is several times higher compared to systemic delivery of chemotherapeutic drugs and carries less systemic toxicity. Despite tremendous advancement in saving life, eye salvage in advanced RB especially with active vitreous seeds remains a challenge. The hypoxic environment of the vitreous and reduced vitreous concentration of the drugs delivered makes these tumor seeds resistant to chemotherapy. Direct delivery of chemotherapeutic drugs into the vitreous cavity aids to overcome these challenges and is progressively being accepted worldwide. However, intraocular procedure in RB was abandoned due to high risk of extraocular tumor dissemination. Recently, the forbidden therapeutic technique was re-explored and modified for safe use. Although eye salvage rate has tremendously improved after intravitreal chemotherapy (IVitC), retinal toxicity, and vision salvage are yet to be validated. In our preliminary report of intravitreal melphalan in 11 eyes, we reported 100% eye salvage and 0% recurrence with an extended 15 months mean follow-up. In this review, we analyzed published reports on IVitC in RB via PubMed, Medline, and conference proceedings citation index, electronic database search, without language restriction that included case series and reports of humans and experimental animal eyes with RB receiving IVitC.

Human papillomavirus (HPV) infection is strongly associated with several human cancers; the most known genotypes involved being HPV 16 and HPV 18. We report the detection of HPV 52 in a sample taken from a 47-year-old patient with squamous cell carcinoma of the conjunctiva of the left eye. The method used for the detection of HPV was real time polymerase chain reaction. The evolution was favorable after surgical removal of the tumor and the patient was explained that long-term follow-up is essential to avoid recurrence.

Vascular tumors of the retina and choroid can be seen occasionally. In the following article, the key clinical and diagnostic features of the major retinal and choroidal vascular tumors, their systemic associations, and the literature pertaining to the most currently available treatment strategies are reviewed.

Background: Keratoconus is a contraindication for photorefractive keratectomy (PRK). In the recent decade, some efforts have been made to perform PRK in patients with keratoconus whose corneas are stable naturally or by doing corneal collagen crosslinking. These studies have suggested residual central corneal thickness (CCT) ≥450 μm. Aims: The aim was to evaluate the long-term outcomes of PRK in patients with mild to moderate keratoconus in patients older than 40 with residual CCT ≥ 400 μm. Settings and Design: This prospective study was conducted in our Cornea Research Center, Mashhad, Iran. Materials and Methods: Patients over 40 years old, with a grade I/II keratoconus without progression in the last 2 years were recruited. Patients with a predicted postoperative CCT < 400 μm were excluded. PRK with tissue saving protocol was performed with Tecnolas 217 Z. Mitomycin-C was applied after ablation. The final endpoints were refraction parameters the last follow-up visit (mean: 35 months). Paired t-test and Chi-square were used for analysis. Results: A total of 38 eyes of 21 patients were studied; 20 eyes (52.6%) with a grade I and 18 eyes (47.4%) with grade II keratoconus. The mean uncorrected visual acuity, best corrected visual acuity (BCVA), spherical equivalent, cylindrical power and keratometric readings were significantly improved at the final endpoint compared to preoperation measurements (P < 0.001). Two eyes (5%) lost two lines of BCVA at the final visit. No case of ectasia occurred during the follow-up course. Conclusions: PRK did not induce keratoconus progression in patients older than 40 with a grade I/II keratoconus. Residual CCT ≥ 450 μm seems to be sufficient to prevent the ectasia.

Background: Spectral domain (SD) enhanced depth imaging optical coherence tomography (EDI-OCT) is a useful tool for anatomic, cross-sectional imaging of retinal conditions. Aims: The aim was to identify characteristic patterns of retinal and retinal pigment epithelial tumors on EDI-OCT in children and adults. Settings and Design: Retrospective review. Materials and Methods: Analysis of published reports and personal observations using office-based EDI-OCT for adults and portable hand-held SD OCT for infants and children. Results: Using EDI-OCT, retinal tumors such as small retinoblastoma, astrocytic hamartoma, and hemangioblastoma arose abruptly from the retina, immediately adjacent to normal retina. Small exophytic retinoblastoma and retinal hemangioblastoma showed the full-thickness, homogeneous retinal disorganization with surrounding normal retina "draping" over the margins. Retinoblastoma occasionally had intralesional cavities and surrounding subretinal fluid. Hemangioblastoma often had adjacent intraretinal edema and subretinal fluid. Astrocytic hamartoma arose within the nerve fiber layer and sometimes with a "moth-eaten" or cavitary appearance. Retinal pigment epithelial (RPE) lesions such as congenital hypertrophy of RPE appeared flat with shadowing, occasional subretinal cleft, and abrupt photoreceptor loss. Congenital simple hamartoma showed an abrupt elevation from the inner retina with crisp, dark posterior shadowing. Combined hamartoma of the retina/RPE showed vitreoretinal traction causing "sawtooth mini-peak" or gently "maxi-peak" folding of the retina. RPE adenoma often produces remote macular edema or epiretinal membrane and the tumor has an irregular, "rugged" surface with deep shadowing. Conclusions: Enhanced depth imaging optical coherence tomography shows characteristic patterns that are suggestive of certain retinal and RPE tumors.

We report a case of ocular tuberculosis (TB) which initially presented with disc edema and was mistaken for optic neuritis. With no definite pathology being identified, the patient was treated on the lines of optic neuritis with intravenous (IV) steroid with beneficial effect. Ocular TB was suspected when he presented later with a subretinal abscess. Based on positive Mantoux, QuantiFERON TB gold results and radiographic findings, a diagnosis of subretinal abscess of presumed tubercular etiology was made. The patient was successfully treated with anti-tubercular therapy. To the best of our knowledge, this is the first case report of ocular TB presenting as disc edema followed by subretinal abscess.

Aim: To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa. Methods: A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally. Results: Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis. Conclusion: Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%). To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen.

Background: To present a coincidence of macular telangiectasia type 2 and solitary retinal astrocytic hamartoma in one patient. Case presentation: A 50-year-old woman was examined in the Department of Ophthalmology of University hospital Kralovske Vinohrady for complaints of metamorphopsia in her left eye. Her uncorrected visual acuity (VA) was 4/4 on Early Treatment Diabetic Retinopathy Study charts (ETDRS), on the retina of her left eye white, prominent, partially calcified tumour 1 disc diameter in diameter, 1,5 disc diameter from the foveola was detected on the retina. In the macular region of both eyes, parafoveal greying with crystalline deposits and changes in retinal vasculature were visible. We performed following examinations: fluorescein angiography (FA), B-scan ultrasound, spectral domain optical coherence tomography (SD-OCT) including photo documentation. FA showed partial hyperfluorescence of mulberry-like surface of the tumour typical for retinal astrocytic hamartoma. Parafoveally in both eyes, leakage from parafoveal telangiectasia was apparent. SD-OCT showed cystoid space in the macular region of both eyes as well as changes in inner and outer photoreceptor segment junction in left eye. SD-OCT of the tumour showed proliferation in retinal nerve fibre layer with normal structure of underlying retinal layers and choroid. Ultrasound examination of the tumour detected solid, highly echogenic prominent tumour with high reflectivity and acoustic shadow. Conclusion: A coincidence of two relatively rare clinical units, macular telangiectasia type 2 and solitary astrocytic hamartoma was detected as a unique and rare observation. [ABSTRACT FROM AUTHOR]

Background: Spectral domain (SD) enhanced depth imaging optical coherence tomography (EDI-OCT) is a useful tool for anatomic, cross-sectional imaging of retinal conditions. Aims: The aim was to identify characteristic patterns of retinal and retinal pigment epithelial tumors on EDI-OCT in children and adults. Settings and Design: Retrospective review. Materials and Methods: Analysis of published reports and personal observations using office-based EDI-OCT for adults and portable hand-held SD OCT for infants and children. Results: Using EDI-OCT, retinal tumors such as small retinoblastoma, astrocytic hamartoma, and hemangioblastoma arose abruptly from the retina, immediately adjacent to normal retina. Small exophytic retinoblastoma and retinal hemangioblastoma showed the full-thickness, homogeneous retinal disorganization with surrounding normal retina "draping" over the margins. Retinoblastoma occasionally had intralesional cavities and surrounding subretinal fluid. Hemangioblastoma often had adjacent intraretinal edema and subretinal fluid. Astrocytic hamartoma arose within the nerve fiber layer and sometimes with a "moth-eaten" or cavitary appearance. Retinal pigment epithelial (RPE) lesions such as congenital hypertrophy of RPE appeared flat with shadowing, occasional subretinal cleft, and abrupt photoreceptor loss. Congenital simple hamartoma showed an abrupt elevation from the inner retina with crisp, dark posterior shadowing. Combined hamartoma of the retina/RPE showed vitreoretinal traction causing "sawtooth mini-peak" or gently "maxi-peak" folding of the retina. RPE adenoma often produces remote macular edema or epiretinal membrane and the tumor has an irregular, "rugged" surface with deep shadowing. Conclusions: Enhanced depth imaging optical coherence tomography shows characteristic patterns that are suggestive of certain retinal and RPE tumors. [ABSTRACT FROM AUTHOR]

The article presents several cases of multifocal retinal astrocytic hamartomas in patients with suspected tuberous sclerosis complex (TSC). In the case of the 17-year-old Middle Eastern girl, she has shown collagenoma, a connective tissue nevus that was suggestive of TSC following a baseline ophthalmic examination. The other cases involved a 27-year-old Hispanic female and a 9-year-old white male who was presented for assessment of a fibrotic lesion in the left macula.

Neurofibromatosis type 2 (NF2) is a heritable syndrome characterized by multifocal proliferation of neural crest-derived cells. The characteristic and diagnostic finding of NF2 is bilateral vestibular nerve schwannomas (acoustic neuromas). In addition to other tumors involving the central and peripheral nervous systems, ophthalmic manifestations, including posterior subcapsular and peripheral cortical cataracts, optic nerve meningiomas, epiretinal membrane, and combined pigment epithelial and retinal hamartomas, are common to NF2. Herein we present an 8- year-old girl with NF2 and astrocytic hamartoma of the optic disc. This patient had been previously diagnosed with NF1 on the basis of multiple CAL macules and suspected subcutaneous neurofibromas. However, neuroimaging revealed bilateral acoustic neuromas, leading to a clinical diagnosis of NF2. Subsequent molecular genetic analysis confirmed the NF2 diagnosis. Multiple CAL macules and astrocytic hamartomas, while associated with NF1, are rarely associated with NF2. Specifically, we are not aware of any reported cases of optic disc astrocytic hamartoma in the setting of NF2. [ABSTRACT FROM AUTHOR]

Abstract: Background: Retinal astrocytic hamartomas are glial tumors of the retinal nerve fiber layer that arise from retinal astrocytes. Classically they appear as a cream-white, well-circumscribed, elevated lesion that may present as multiple or solitary sites. A lesion is commonly seen with a multilobulated, “mulberry” appearance, but can also appear flat and semitranslucent. It is most frequently associated with tuberous sclerosis (TS) but may also be found rarely in patients with neurofibromatosis. Although the finding may point toward a systemic association, it can also be found incidentally on retinal examination as an isolated presentation. Case Reports: Two cases of solitary retinal astrocytic hamartomas without systemic complications discovered at routine examination are presented. Both patients were found to have a raised, multilobulated retinal lesion consistent with the appearance of a retinal astrocytic hamartoma. In both cases, the patients had no personal or family history of seizures or any signs of unusual dermatologic lesions. B-scan ultrasonography, fluorescein angiography, and magnetic resonance imaging (MRI) were performed as necessary. Conclusion: A retinal astrocytic hamartoma is typically associated with TS but can also present as a spontaneous, idiopathic lesion. Prompt referral to rule out TS or other systemic associations, particularly in young children, is important. Neuroimaging, along with other diagnostic tests, may ensure the proper management and rule out need for further evaluation. Spontaneous lesions without systemic association can initially be monitored closely to rule out possible progression. Once stability has been established, annual follow-up with a primary care eye practitioner is appropriate. [Copyright &y& Elsevier]

Abstract: Retinal glial tumors and pseudotumors can be classified into astrocytic hamartoma, acquired retinal astrocytoma, massive gliosis, and focal nodular gliosis. Each has different clinical manifestations. Astrocytic hamartoma is usually seen patients who have some manifestations of tuberous sclerosis complex (TSC). It can occur as a noncalcified or calcified variety, and often a combination of the two, and has fairly typical features with fluorescein angiography, ultrasonography, and optical coherence tomography. Although it is generally a stationary lesion, an aggressive variant seen in very young children with TSC can lead to exudative retinopathy, retinal detachment, and neovascular glaucoma. Acquired astrocytoma general occurs in somewhat older individuals who do not have TSC. Like the aggressive form of astrocytic hamartoma it can lead to exudative retinopathy and exudative retinal detachment. Pseudoneoplastic diffuse retinal gliosis can occur as massive glial proliferation in eyes with prior trauma, Coats disease, retinal angiomatosis and other conditions and often occurs in blind eyes. Pseudoneoplastic focal retinal gliosis is characterized by a very superficial, white, noncalcified lesion in otherwise normal eye in somewhat older individuals. Recognition of these glial lesions is important because they can resemble malignant tumors and have different clinical courses and complications. [Copyright &y& Elsevier]

Background: Retinal hamartoma is a common finding in tuberous sclerosis, but the symptomatic changes of this lesion have rarely been described. This evidence-based review evaluated the incidence of symptomatic retinal hamartoma and compared possible treatment modalities. Methods: We carried out a review of the literature using MEDLINE. Older publications not listed in MEDLINE were obtained from the reference list of currently published papers. Results: Three observational case series with a follow-up of up to 34 years included 93 patients and reported progression from a flat to a more elevated lesion without visual symptoms in nine patients (9.7%). Additional symptomatic changes were described in 11 case reports published over a period of three decades. The symptomatic alterations were caused by an enlarged tumour with leakage, macular oedema, accumulating lipoid exudates, serous retinal detachment ( n = 8/11) and vitreous haemorrhage ( n = 4/11). Most symptomatic cases involved a retinal hamartoma type 1 ( n = 6/8). Spontaneous resolution of symptomatic exudative hamartomas occurred in three patients within 4 weeks, although a delayed resorption of subretinal fluid caused permanent visual impairment in one patient. Investigational reports described a slow resorption of subretinal fluid after argon laser photocoagulation ( n = 2), although recurrent laser applications induced choroidal neovascularization and destruction of the neurosensory retina ( n = 1). A vitrectomy was used to remove a vitreous haemorrhage in another reported patient. In one case, complete resorption of subretinal fluid and an increase in visual acuity was observed within 2 weeks after a single treatment with photodynamic therapy (PDT). No complications were noted during a follow-up of 4 years. Conclusions: Symptomatic changes are very rare in retinal hamartomas secondary to tuberous sclerosis. Spontaneous resolution of subretinal fluid may occur within 4 weeks. If a macular oedema with increasing lipoid exudates persists over a period of 6 weeks, treatment should be considered. Although previous reports demonstrated possible visual stabilization after argon laser photocoagulation, vision-threatening complications can occur. Current treatment strategies may include PDT based on favourable anatomical and functional results. [ABSTRACT FROM AUTHOR]

Purpose: To report successful treatment of a fine-needle aspiration biopsy--proven acquired retinal astrocytoma with photodynamic therapy. Methods: Case report. Results: A 50-year-old man complaining of decreased vision of his right eye was found to have a visual acuity of 20/100 and an amelanotic juxtapapillary retinal tumor with surrounding subretinal fluid and lipid exudation extending into the fovea. Fine-needle aspiration biopsy showed an astrocytic lesion consistent with retinal astrocytoma. The lesion was treated with one session of photodynamic therapy. At 20 months after photodynamic therapy, visual acuity had improved to 20/20 with complete tumor regression and normal foveal contour on optical coherence tomography. Conclusion: This report confirms that photodynamic therapy of symptomatic acquired retinal astrocytoma may be effective in causing tumor regression and stabilizing or improving visual acuity by reducing tumor leakage. [ABSTRACT FROM AUTHOR]