Atypical case of choroidal osteoma associated to polypoidal choroidal vasculopathy and preretinal neovascular membrane.

PURPOSE: To report a very rare and atypical case of an elderly Caucasian female patient who developed perilesional multiple polypoidal choroidal vasculopathy (PCV) as a probable complication of choroidal osteoma (CO), associated to preretinal neovascular membrane overlying the lesion. METHODS: Observational case report. CASE OBSERVATION: A 60-year-old Caucasian woman presented with blurred vision in her right eye (RE). Fundus examination revealed a round white-yellowish calcified deep lesion in the juxta-papillary superior area, measuring 4 disc-diameters, with well-defined scalloped margins and an irregular surface. B-scan ultrasonography and orbital tomography confirmed the diagnosis of choroidal osteoma (CO). Further investigation with multimodal imaging including infracyanine green angiography, fluorescein angiography, swept source optical coherence tomography and angiography highlighted the presence of multiple aneurysmal choroidal dilations around the CO, corresponding to PCV. We also noted the presence of a preretinal neovascular membrane overlying the CO. The patient was monitored with regular follow-up since no signs of activity were detected on multimodal imaging. CONCLUSION: Our case report represents an exceptional and atypical association between pre-retinal neovascularization, PCV and choroidal osteoma. While the mechanisms underlying the development of PCV and pre-retinal neovascularization in the setting of CO are not well understood, it is imperative for ophthalmologists to recognize this association as a potential cause of sudden vision loss in patients with CO, and to consider appropriate diagnostic and management strategies.

Bilateral choroidal osteoma: long-term follow-up of secondary choroidal neovascularization in a child using antiangiogenic therapy.

Choroidal osteoma is a rare condition, and its treatment is not well established, especially in the pediatric population, where use of antiangiogenics for choroidal neovascularization is poorly studied. Few studies have reported the long-term follow-up of pediatric patients with bilateral choroidal osteomas. We report the case of a girl who was diagnosed at the age of 3, with the appearance of bilateral secondary choroidal neovascularization, and has been under strict observation for 12 years. The effectiveness of antiangiogenic agents as a long-term therapeutic option for secondary choroidal neovascularization in pediatric patients with symptomatic choroidal osteomas is discussed.

Importance of multimodal imaging diagnostics in acute angle closure glaucoma: Case report.

To describe the natural history and management of an acute angle closure secondary to choroidal melanoma. A 70-year-old female presented with pain, elevated intraocular pressure, mature cataract, and angle closure in right eye. With further studies she was found to have a choroidal melanoma causing the acute angle closure. Enucleation was performed and the patient is currently in close postoperative surveillance by ophthalmology and oncology. The importance of early identification and treatment of intraocular tumors to decrease incidence of metastasis. In secondary acute angle-closure glaucoma treatment should be targeted towards resolving the triggering factor of glaucoma.

Rhegmatogenous retinal detachment in choroidal melanoma: clinical features and surgical outcomes.

PURPOSE: To describe the clinical features, prognostic factors, safety and rate of success of surgery and visual outcomes in patients with rhegmatogenous retinal detachment (RRD) and choroidal melanoma (CM). METHODS: A retrospective, observational case-series of 21 patients with rhegmatogenous retinal detachment or combined tractional-rhegmatogenous retinal detachment in patients with choroidal melanoma over a period of 20 years. RESULTS: Nineteen patients were included in the final analysis. The mean elevation of CM was 4.0 mm and the mean largest diameter was 11.0 mm. RRD occurred after the CM treatment in 14 eyes at a mean interval of 44.2 months. The RRD was macula-on RRD in 6 eyes, there was posterior vitreous detachment (PVD) in 15 and PVR in 7 eyes. BCVA at presentation was 0.71 logMAR and final was 1.5 logMAR (p = 0.01). The primary surgical success rate was 59%. No intraocular or extraocular tumour dissemination occurred. Mean follow-up was 66 months. CONCLUSION: RRD in patients with CM is uncommon but requires multidisciplinary management. Anatomical results are favourable but visual outcomes are poor due to a combination of factors related to melanoma treatment, macular retinal detachment and PVR. Vitrectomy as a surgical intervention for RRD in treated CM appears to be safe in terms tumour dissemination.

Serous Retinal Detachment in a Patient With a Port-Wine Birthmark Associated With Prostaglandin Analog Therapy.

An 11-year-old girl with a port-wine birthmark (PWB), diffuse choroid hemangioma (DCH), and glaucoma experienced decreased vision upon starting treatment with bimatoprost. The patient was switched to latanoprostene bunod. Her vision remained reduced. Shortly after, she was diagnosed with serous retinal detachment (SRD). Both SRD and vision improved following prostaglandin analog (PGA) cessation. Patients with PWB are likely to have DCH and glaucoma. DCH itself poses a risk factor for SRD. Certain glaucoma management modalities may further increase this risk. This report highlights the importance of regular surveillance for SRD in patients with DCH who are receiving PGA. [Ophthalmic Surg Lasers Imaging Retina 2023;54:723-729.].

Isolated diffuse choroidal hemangioma without systemic symptoms: a case report.

BACKGROUND: Sturge-Weber syndrome is a nonhereditary congenital neurocutaneous syndrome characterized by a distinctive facial capillary malformation,neurological abnormalities, and ocular abnormalities such as glaucoma and choroidal hemangioma.It can be divided into different subtypes according to different clinical manifestations. It is rare for a patient to present with isolated difuse choroidal hemangioma and ipsilateral abnormal conjunctival and episcleral vessels without other systemic symptoms. CASE PRESENTATION: We report a 30-year-old man with isolated diffuse choroidal hemangioma in his right eye without systemic symptoms, such as vascular malformations in the skin or leptomeningeal angiomatosis. The only additional ophthalmic finding was ipsilateral abnormal conjunctival and episcleral vessels without glaucoma. However, there was no evidence of leptomeningeal angiomatosis or port-wine stain on the right side of the face, or glaucoma, which are common clinical manifestations of the Sturge-Weber syndrome (SWS).The absence of these characteristic symptoms did not preclude the diagnosis, and the patient could be diagnosed with a particular subtype of SWS. CONCLUSIONS: This is a rare case of documented isolated difuse choroidal hemangioma with ipsilateral abnormal conjunctival and episcleral vessels without glaucoma which we think it is belonging to a particular subtype of SWS.In addition to the traditional clinical manifestations, more and more atypical clinical manifestations are also accompanied by SWS, which requires our clinicians to continuously discover and report, so as to help more clinicians understand this disease.

Clinical features and therapeutic management of choroidal osteoma: A systematic review.

Choroidal osteoma is a benign ossifying tumor within the choroid. Complications associated with choroidal osteoma, including disruption of retinal pigment epithelium, atrophy of photoreceptors, subretinal fluid, and choroidal neovascularization, present challenges for clinicians, and management remain controversial. We performed a comprehensive search in the PubMed, EMBASE, and Ovid databases for published studies and case reports relating to the management of choroidal osteoma. Since it was first described in 1978, various case reports of ocular complications associated with choroidal osteoma have been documented, and various therapies have yielded different outcomes. We systematically evaluate the literature published on this rare entity.

Corneal Perforation as a Rare and Late Manifestation of Choroidal Melanoma.

PURPOSE: To report a case of corneal perforation as a rare and late manifestation of choroidal melanoma and to highlight the major histopathological findings of this unusual combined clinical presentation. METHODS: A 74-year-old male patient presented to our department due to corneal perforation of the right eye with the absence of light perception for 6 months. The intraocular pressure was hard on palpation. Because of the protracted finding and reduced visual prognosis, primary enucleation was performed. RESULTS: The histopathological examination revealed choroidal melanoma with epithelioid and spindle cell components at the posterior pole, which was positive for Melan-A, Human Melanoma Black 45 (HMB45), BAP1, and SOX10. The anterior segment showed complete anterior chamber hemorrhage and blood remnants in the trabecular meshwork. The cornea displayed diffuse blood staining with hemosiderin and hemosiderin-loaded macrophages and keratocytes. No inflammatory cells were present near the corneal perforation, which had a width of 3 mm. Intraocular heterotopic ossification was indicative of a long-standing condition. Postoperative cancer staging was normal. CONCLUSION: Corneal perforation should be considered as a very rare and late manifestation of advanced choroidal melanoma and may result from interaction between intraocular hemorrhage, elevated IOP, and its secondary signs such as corneal blood staining.

Occurrence of circumscribed choroidal hemangioma accompanied by polypoidal choroidal vasculopathy and branch retinal vein occlusion: a case report.

BACKGROUND: Circumscribed Choroidal hemangioma (CCH) is a kind of hamartoma that is caused by congenital vascular malformation. And, polypoidal choroidal vasculopathy (PCV) is an exudative maculopathy. There is no literature indicating that there is a correlation between the occurrences of CCH and PCV. CASE PRESENTATION: A 66-year-old male presented with decreased vision of his left eye for 4 years. Fundus photograph showed that the branches of blood vessels at the supratemporal retina were occluded in white lines, an orange lesion could be seen in the subnasal retina and mottled, yellowish white lesions were accompanied by punctate hard exudation in the macular in the left eye. The Fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and Spectral domain optical coherence tomography (OCT) were done. There was a diagnosis of CCH, PCV and branch retinal vein occlusion accompanied with retinoschisis of the left eye. CONCLUSION: This article reports on a case of an elderly male Chinese patient with CCH and PCV accompanied by branch retinal vein occlusion with retinoschisis in the left eye. The common lesions are choroidal vascular abnormalities. Whether hypertension is related to CCH, PCV and branch retinal vein occlusion remains to be further studied.

[Photodynamic therapy in Germany-Quo vadis?] / Photodynamische Therapie in Deutschland ­ Quo vadis?

BACKGROUND: Photodynamic therapy (PDT) was originally approved for the treatment of neovascular age-related macular degeneration (nAMD) and secondary choroidal neovascularization in myopia (mCNV). In addition, it is used as an off-label treatment in patients with choroidal hemangioma, polypoidal choroidal vasculopathy (PCV), and central serous chorioretinopathy (CSC). OBJECTIVE: To track the development of PDT treatment numbers in Germany between 2006 and 2021 and to investigate the composition of the therapeutic indications. METHODS: In this retrospective study the quality reports of German hospitals were evaluated in the period from 2006 to 2019 and the number of PDTs performed was recorded. In addition, the range of indications for PDT was determined exemplarily for the Eye Center at Medical Center, University of Freiburg and the Eye Center at St. Franziskus Hospital in Münster between 2006 and 2021. Finally, the estimated prevalence of CSC and an estimate of cases requiring treatment were used to calculate the number of patients in need of PDT treatment in Germany. RESULTS: The number of PDTs performed in Germany decreased from 1072 in 2006 to 202 in 2019. While PDT was used in 86% of cases in patients with nAMD and in 7% of cases with mCNV in 2006, it was mainly performed in patients with CSC (70%) and choroidal hemangiomas (21%) from 2016 to 2021. With an estimated incidence of CSC of 1:10,000 and assuming that 16% of patients develop chronic CCS requiring treatment, approximately 1330 PDTs would need to be performed per year in Germany for patients with newly diagnosed chronic CSC alone. CONCLUSION: The decreasing numbers of PDT treatment performed in Germany is mainly due to a change to intravitreal injections as the preferred treatment for nAMD and mCNV. As PDT is currently the recommended treatment of choice for chronic CSC, an underprovision of PDT in Germany can be assumed. To enable an appropriate treatment for patients, a reliable verteporfin production, a simplified approval process by health insurance companies and a close cooperation between ophthalmologists in private practice and larger centers are urgently needed.

The role of choriocapillaris vessel density in the pathogenesis of macular neovascularization associated with choroidal osteoma.

PURPOSE: To detect the vessel density of choriocapillaris (CC) vascular network in eyes affected by choroidal osteoma and in eyes complicated by macular neovascularization (MNV), using optical coherence tomography angiography (OCTA). METHODS: In this retrospective study, twenty-eight eyes of 28 patients were divided into three groups: group 1 including patients with calcified choroidal osteoma, group 2 including patients with decalcified choroidal osteoma, and group 3 including patients with decalcified choroidal osteoma complicated by MNV. OCTA analyzed the vessel density of CC in these lesions localized in the peripapillary region. RESULTS: We enrolled 12 eyes with calcified choroidal osteoma, 11 eyes with decalcified choroidal osteoma, and 5 eyes with decalcified choroidal osteoma complicated by MNV. The eyes with decalcified choroidal osteoma and MNV revealed a statistically significant reduction in vessel density of the CC respect to the other groups (p < 0.001). Moreover, the vessel density of CC in decalcified choroidal osteoma was significantly reduced compared to calcified choroidal osteoma (p < 0.001). CONCLUSIONS: OCTA allowed a quantitative evaluation of choriocapillaris vessel density in choroidal osteoma, in order to detect the changes of this vascular network, which could lead to the development of MNV. Therefore, OCTA could be a new diagnostic tool in the clinical management of the choroidal osteoma. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT05342324.

Long-term visual outcomes after ruthenium plaque brachytherapy for posterior choroidal melanoma.

BACKGROUND: To assess the long-term visual outcomes in patients with posteriorly located choroidal melanoma treated with ruthenium plaque brachytherapy between January 2013 and December 2015. METHODS: A retrospective review was conducted on consecutive patients treated with ruthenium plaque brachytherapy for post-equatorial choroidal melanoma with available Snellen visual acuity before and after treatment, and the development and treatment of radiation complications. RESULTS: There were 219 patients with posterior choroidal melanoma treated with ruthenium plaque brachytherapy. Median follow up was 56.5 months, range 12-81 months. Final visual acuity was ≥6/12 in 97 (44.3%) patients, 6/12 to 6/60 in 57 (26.0%), <6/60 in 55 (25.1%) and 10 (4.6%) eyes were enucleated. Radiation maculopathy was the most common radiation complication encountered, occurring in 53 (24.2%) patients. Of these, final visual acuity was 6/12 in 10 patients (18.9%), 6/12 to 6/60 in 26 (49.1%), <6/60 in 16 (30.2%) and 1 eye (1.9%) was enucleated. Twenty-five (47%) with radiation maculopathy were treated with intravitreal anti-angiogenic therapy, 27 (51%) were monitored and one (2%) was treated with scatter photocoagulation. Eyes treated with intravitreal anti-angiogenic therapy had better final vision than those observed or treated with retinal laser (chi-square, p = 0.04). On multivariate analysis, close proximity to the optic nerve and fovea, and large or notched plaque type was associated with final vision worse than 6/12. CONCLUSION: Most patients treated with ruthenium plaque brachytherapy for posterior choroidal melanoma retain 6/60 vision, with almost half retaining 6/12 vision at long term follow up.

Low-dose proton radiotherapy for pediatric choroidal hemangioma: A case series.

Management of pediatric choroidal hemangioma complicated by large exudative retinal detachment can be challenging, with few options available. Limited data have been published on outcomes following proton radiotherapy (PRT) for management of these patients. In this retrospective case series, nine patients were treated with a low-dose PRT regimen of 20 Gy(relative biological effectiveness [RBE]) in 10 fractions, and two were treated with 15 Gy(RBE) in four fractions. Visual acuity improved in seven patients (64%) and remained stable in the remaining four (36%). In patients with imaging follow-up (10 patients), subretinal fluid resolved in nine patients (90%) and tumor thickness decreased or remained stable in 10 (100%). Complications were observed in eight of 11 patients (73%). One patient developed grade 2 cataract; otherwise, no grade ≥2 complications were observed.

Relentlessly Progressive Sweet Syndrome of the Eye with Scleritis and Choroidal Infiltration.

PURPOSE: To describe a case of Sweet syndrome, a dermatologic inflammatory disease, with progressive, unrelenting ocular findings. METHODS: Case report. RESULTS: A 73-year-old male was evaluated with a six-month history of Sweet syndrome, manifesting as cutaneous erythematous edematous papules on the dorsal arms and shins and confirmed with biopsy demonstrating neutrophil infiltration with nuclei fragmentation and lack of vasculitis. He initially noted a unilateral red eye with ocular pain and was found to have scleritis and choroidal infiltration. The patient's ocular disease progressed despite treatment with systemic corticosteroids, intraocular Ozurdex ®, systemic dapsone, and subtenons triamcinolone. Systemic evaluation was negative for malignancy or other inflammatory syndromes. Following 7 months of non-manageable ocular pain enucleation was offered to the patient, but he declined. CONCLUSION: Sweet syndrome, a dermatologic condition, can be associated with unilateral scleritis and choroidal infiltration that are relentlessly progressive despite maximal systemic and ocular corticosteroid therapy.