De novo pediatric choroidal Osteoma: A longitudinal observation since inception and through treatment

Choroidal osteoma (CO), also known as osseous choristoma, is a rare benign osseous tumour of the choroid. It is diagnosed based on the presence of a yellowish-white to orange lesion deep in the retinal pigment epithelium (RPE) with well-defined margins and bone density in ultrasonography (Pellegrini et al. 2014). Patients are usually asymptomatic and the lesion is found incidentally. Choroidal neovascularization (CNV) is the most frequent complication of CO and leads to severe visual impairment. Anti-vascular endothelial growth factor (Anti-VEGF) therapy has been recommended for CNV-related CO (Song & Roh 2009). Here, we report a case of extrafoveal CO associated with CNV. A 75-year-old woman, incidentally diagnosed with CO at the age of 68 years, presented with a two-month history of metamorphopsia in the left eye. Best-corrected visual acuity (BCVA) was 0 logMar in the right eye and 0.7 logMar in the left eye. Anterior segment findings were unremarkable in both eyes. A fundus examination of the right eye was unremarkable, whereas, in the left eye, it revealed a yellowish-white area with small haemorrhages in the superior temporal retina that suggested an extrafoveal CO associated with CNV (Fig. 1A,B). Baseline evaluation of the right eye. (A, B) Colour fundus photography and multicolour image showing the choroidal osteoma with subretinal haemorrhage. The black circle underlines macular haemorrhage spots. (C−E) Optical coherence tomography (OCT) angiography, fluorescein angiography and indocyanine green angiography revealed late-stage choroidal neovascularization within the tumour. The red circle underlines macular haemorrhage spots (F, G) A and B scan echography showed a slightly elevated choroidal mass with high reflectivity and acoustic shadowing. (H) Enhanced depth imaging optical coherence tomography (EDI-OCT) showed a hyper-reflective mass in the subretinal space associated with a neurosensory detachment and detected a sponge-like structure of the choroid. (I, J) Fluorescein and indocyanine angiography of the superotemporal region of the contralateral normal eye. (K) Optical coherence tomography (OCT) scan of the macular region without any pathological feature. (L−P) Colour fundus photography, multicolour image, fluorescein angiography and indocyanine green angiography and OCT angiography showing regression of the choroidal neovascularization after four months of treatment. (Q) Enhanced depth imaging optical coherence tomography (EDI-OCT) revealed resolution of the subretinal fluid. [Correction added on 17 June 2016 after first online publication: Figure 1 has been replaced to correct the labelling of Fig. 1E and 1O]. Optical coherence tomography (OCT) angiography (angiovue software RTVue; Optovue Inc., Fremont, CA, USA) showed a hyporeflective area surrounded by a hyper-reflective-edged ring in direct relation to the osteoma. It also showed a dense irregular vascular network within the tumour in the outer retinal layer and choroid capillary layers. Moreover, the CNV detected in the choroidal layer had fine glomerular-like hyper-reflection (Fig. 1C). Visualization of vessels is better with non-invasive OCT angiography than with fluorescein angiography (FA) and Indocyanine green angiography (ICGA); in fact, with the latter two techniques, the leaking vessels of the CNV usually are overshadowed by the dye in the late phases (Wang et al. 2015). Fluorescein angiography (FA) showed early hyperfluorescence due to leakage surrounded by an area of blocked fluorescence, and late staining of the lesion showed well-defined borders (Fig. 1D). Indocyanine green angiography (ICGA) confirmed the presence of an active CNV secondary to CO (Fig. 1E). Standardized A and B scan echography revealed a slightly elevated choroidal mass with high reflectivity and acoustic shadowing (Fig. 1F,G). Enhanced depth imaging optical coherence tomography (EDI-OCT) performed with the Heidelberg Spectralis HRA + OCT (Heidelberg Engineering, Heidelberg, Germany) showed a hyper-reflective mass in the subretinal space associated with neurosensory detachment and elongation of the outer segments. Enhanced depth imaging optical coherence tomography (EDI-OCT) also revealed a sponge-like structure of the choroid with horizontal tubules (Fig. 1H). The findings obtained in our patient strongly support the diagnosis of OC as opposed to sclerochroidal calcification (SCC). In fact, unlike OC, SCC does not feature growth, decalcification or intralesional vessels of CNV, as recently reported by Shields et al. (2015). After obtaining informed consent, we administered three monthly intravitreal injections of ranibizumab in the left eye. Four months later, the patient's BCVA was 0.1 logMar, and a fundus examination revealed resolution of the subretinal haemorrhage (Fig. 1L,M). Fluorescein angiography (FA) and ICGA showed regression of the CNV (Fig. 1N,O) and spectral domain OCT revealed no subretinal fluid (Fig. 1Q). Optical coherence tomography (OCT) angiography demonstrated a smaller and more rarified vascular network inside a capsular formation (Fig. 1P). These findings remained unchanged during the 12-month follow-up. In summary, OCT angiography reveals unique features in the vascular changes of CNV in CO not visualized with other imaging methods. However, additional studies are needed to verify whether this technique can partially replace FA and ICGA in the diagnosis and follow-up of CNV secondary to CO.

Inhibition of choroidal osteoma progression using bisphosphonate and RANKL-inhibitory treatment

To report a case of choroidal osteoma that developed subretinal hemorrhage after photodynamic therapy (PDT). Interventional case report. An 8-year-old boy was brought to our attention because of mild visual loss in his left eye. The visual acuity was 20/25. A subfoveal yellow-orange-colored lesion, measuring 5.0 mm × 4.5 mm in base diameter was detected in the left eye. B-mode ultrasonography showed an acoustically solid 2.0-mm-thick mass with orbital shadowing. Orbital computed tomography confirmed the presence of calcium, and choroidal osteoma was diagnosed. As there was a decrease in the best-corrected visual acuity (20/30) and the lesion showed growth (6.0 mm × 6.0 mm), PDT was performed at 6 months follow-up. The day after PDT, the visual acuity was counting fingers at 1 m and a subretinal hemorrhage over the lesion was detected. Two weeks after PDT, subfoveal hemorrhage dissappeared with some retinal pigment epithelial hyperplasia and visual acuity increased back to 20/30. Although PDT can induce decalcification in choroidal osteomas, it should not be performed in subfoveal osteomas unless there is evidence to treat the lesion such as the presence of choroidal neovascularization.

To describe a patient with a choroidal osteoma treated with photodynamic therapy to prevent tumor growth in whom choroidal neovascularization (CNV) developed after being treated with photodynamic therapy. Case report. A 5-year-old Hispanic woman presented with an asymptomatic choroidal osteoma, temporal to the macula of her right eye. According to the patient's mother, her medical, surgical, and family history was unremarkable. At examination, best-corrected visual acuity was 20/30 in both eyes. After 11 months of follow-up, signs of tumor growth toward the fovea without any signs of CNV was noted. Photodynamic therapy was performed to prevent invasion of the foveola. Two months thereafter, the patient developed CNV in the macula region in the right eye, decreasing visual acuity to 20/200. The patient was treated with four total intravitreal injections of 1.25 mg of bevacizumab over 24 weeks, which resulted in inactivation of the CNV and improved visual acuity to 20/20. Choroidal neovascularization had been never reported in her past history and her follow-up visits over 7 years. In addition, no evidence of recurrent neovascular activity or tumor growth was reported. Choroidal osteoma is a benign tumor that can result in vision-threatening complications, caused by tumor growth and tumor decalcification. Photodynamic therapy is an effective modality in inducing choroidal osteoma decalcification and stabilization; however, CNV due to reperfusion following photodynamic therapy can be seen in the retina.

Dear Editor, Retinal capillary hemangioma (RCH) is a benign capillary angiomatous hamartoma that occurs on the peripheral or juxtapapillary retina. The condition in which RCH is accompanied by intracranial hemangioblastomas and cysts in abdominal organs such as kidneys or pancreas is termed von Hippel-Lindau (VHL) disease. Photodynamic therapy (PDT) with anti-vascular endothelial growth factor agents is an effective treatment for RCH [1], although exudative complications can occur following PDT in rare cases [2,3,4]. Herein, we present a case of massive exudative retinal detachment involving the entire retina following the administration of PDT with intravitreal injection of bevacizumab in a patient with VHL disease. A 24-year-old man was referred to our hospital with a history of impaired vision in his left eye. On admission, his visual acuity was 20 / 100 in the left eye, and his medical history revealed a previous diagnosis of VHL disease with multiple cerebellar hemangioblastomas and renal cell carcinoma. Furthermore, the patient had a family history of VHL disease, and genetic analyses revealed a mutation in the VHL tumor suppressor gene (VHL gene). Two months prior to the development of visual symptoms, he had undergone suboccipital craniectomy and excision of the cerebellar hemangioblastomas. Fundus examination revealed an inferonasal, 2-disc diameter-sized RCH with dilated feeder vessels (Fig. 1A). The patient was recommended PDT, which he declined. Fig. 1 Fundus photographs of the left eye of our 24-year-old patient with confirmed von Hippel-Lindau disease obtained at the initial visit (A) and two years later (B). A peripheral retinal capillary hemangioma with dilated feeder vessels was observed. A fundus ... Two years later, the patient revisited our clinic with a complaint of double vision. The visual acuity in his left eye was 20 / 1,000, and fundus examination revealed an enlarged RCH with exudation (Fig. 1B and 1C). Optical coherence tomography revealed subretinal fluid accumulation with macular involvement (Fig. 1D). PDT with a 6 mg/m2 (body surface area) dose of verteporfin over a period of 10 minutes and a light exposure of 50 J/cm2 5 minutes after intravenous verteporfin injection were administered in combination with an intravitreal injection of 1.25 mg/0.05 mL bevacizumab (Avastin; Genentech-Roche, South San Francisco, CA, USA). Fourteen days after PDT and intravitreal bevacizumab injection, the patient's visual acuity in the left eye progressed to no light perception. Funduscopy revealed total retinal detachment with massive exudation and hemorrhage from the RCH (Fig. 1E and 1F). After 21 days, his visual acuity showed no improvement, and total retinal detachment was still apparent. Consequently, we performed pars plana vitrectomy, internal subretinal fluid drainage retinotomy, endodiathermy, cryotherapy and endolaser therapy on the RCH, and injection of silicone oil and bevacizumab. Two months after surgery, his visual acuity in the left eye recovered slightly to enable the detection of hand motion. Funduscopy revealed the absorption of subretinal fluid in the left eye (Fig. 1G and 1H). Six months after vitrectomy, the silicone oil was removed. One year later, the patient's visual acuity in the left eye had improved to 20 / 500 (Fig. 1I and 1J). VHL disease is a dominantly inherited multi-systemic cancer syndrome associated with a mutation in the VHL gene. VHL protein dysfunction leads to upregulation of hypoxia inducible factors, which in turn leads to increased levels of angiogenic cytokines. Angiomatous proliferation is a target for RCHs and is aided by anti-angiogenic treatments. Furthermore, the treatment of RCH is variable and depends on the size, location, and secondary effects. Laser photocoagulation or cryotherapy is the standard treatment for small and medium-sized RCH. PDT for the treatment of peripheral RCH has shown effective results in terms of decreasing regional exudation from large lesions [1,5]. Furthermore, the complication of massive exudative retinal detachment after PDT is extremely rare; only two cases have been reported worldwide, one of which was managed by emergency vitrectomy [3,4]. The surgical outcomes of our case and the previous case [3] indicated successful removal of the subretinal fluid and restoration of vision. Unlike previous cases, we also administered an intravitreal injection of bevacizumab; however, this did not prevent massive exudation following PDT. In summary, our case indicates that treatments for large RCHs in patients with VHL disease must be carefully selected. Although PDT is considered one of the useful treatment options for RCH, PDT combined with an intravitreal injection of bevacizumab can lead to permanent and severe deterioration of vision because of massive exudative retinal detachment. Therefore, frequent fundus examinations should be performed after PDT.

A choroidal osteoma (CO) is a relatively rare, benign tumor with ossification that develops in the choroid and undergoes enlargement and decalcification in its natural course. Photodynamic therapy (PDT) is used to induce decalcification, but there are few reports on individual cases treated with PDT. A 47-year-old Japanese man who had reduced decimal visual acuity (VA) of the right eye to 0.7 due to a CO away from the fovea was treated with PDT. The PDT resulted in a partial decalcification of CO, and the visual acuity improved to 1.0. However, the tumor slowly expanded, eventually reaching the central fovea. Decalcification and focal choroidal excavation occurred during this natural course of the disease. Although his metamorphopsia worsened, his VA was maintained at 1.0. This case highlights that a CO partially decalcified after PDT can still enlarge and decalcify over several years. These findings indicate the need for careful and continuous monitoring of eyes with a CO.

To report a case of macular choroidal osteoma treated with photodynamic therapy. A 34-years old woman with decreased visual acuity in her left eye came to our observation for assessment of an amelanotic choroidal tumor in the left eye. On the basis of ophthalmoscopic and echographic features the tumor was diagnosed as choroidal osteoma. Imaging examination revealed subretinal fluid involving the foveal area associated with alterations of outer neuroepithelial layers and photoreceptors without evidence of choroidal neovascularization. Foveal sparing standard fluence rate photodynamic therapy was performed. After treatment, subretinal fluid reabsorption and visual acuity recovery was noted with progressive restoration of foveal architecture. Due to the relapse of fluid and visual impairment, 1 year after treatment, a second PDT session was made using the same parameters and protocol of treatment. Despite a complete subretinal fluid reabsorption and visual acuity recovery the second treatment was complicated by the development of subretinal fibrosis. PDT is effective to induce subretinal fluid reabsorption and visual recovery in choroidal osteoma located in the macular area. However, the risk of possible complications related to the treatment have to be considered.

Choroidal osteoma is an unusual form of intraocular calcification seen in otherwise healthy eyes. It is a benign idiopathic osseous tumor of the choroid, typically seen in young females. Choroidal neovascular membrane (CNVM) is a complication seen in one-third of these patients and carries a poor visual outcome. We report a case of a 25-year-old hyperthyroid female with choroidal osteoma and subfoveal CNVM in her left eye which was successfully treated using low-fluence photodynamic therapy (PDT) with verteporfin followed by a single injection of intravitreal ranibizumab.

Choroidal osteoma is a rare benign ossifying tumor of the choroid, predominantly affecting healthy young females. This report presents a case of a 73-year-old female with no significant systemic or ocular history, except for prior cataract surgery. The patient presented with irritation and itching, and examination revealed a hypopigmented lesion inferior to the optic disc in the left eye, consistent with choroidal osteoma. Diagnosis was confirmed via fundus examination, B-scan ultrasonography, and OCT. The lesion displayed elevated retinal pigment epithelium but no subretinal fluid or membrane. Management included cataract surgery with guarded visual prognosis, and regular follow-up for potential choroidal neovascularization. The patient’s visual acuity improved postoperatively. Choroidal osteoma typically results in vision loss through atrophy of the overlying retinal pigment epithelium or choroidal neovascularization. Treatment options for associated complications include photodynamic therapy and anti-VEGF agents. This case emphasizes the importance of monitoring for progressive changes in patients with choroidal osteoma.

Talaporfin Sodium–Mediated Photodynamic Therapy Alone and in Combination with Pulsed Dye Laser on Cutaneous Vasculature

Purpose:To describe a case of choroidal osteoma with choroidal neovascularization (CNV) that was successfully treated with two intravitreal injections of bevacizumab (IVB).Design and methods:Case report on a 12-year-old Japanese girl who presented with a sudden decrease in vision in her left eye. At the first visit, 2 days after the onset of her symptoms, her visual acuity (VA) in her left eye was 0.2. Ophthalmoscopy showed a hemorrhage of 5 disc diameters under the retinal pigment epithelium and a serous retinal detachment at the posterior pole of the left eye. These findings were confirmed by optical coherence tomography. Fluorescein angiography (FA) and indocyanine green angiography (ICGA) showed several points of leakage around the fovea, which suggested a CNV. From these findings, the patient was diagnosed with choroidal osteoma with a CNV. The submacular hemorrhage was from the CNV associated with the choroidal osteoma. We treated her with two injections of 1.25 mg/0.05 mL IVB with a 4-month interval.Results:The patient’s VA in her left eye improved to 0.7, and this vision was maintained for 4 years. The CNV disappeared in the FA and ICGA images and no recurrence was observed after 4 years.Conclusion:Our findings indicate that IVB is effective in resolving CNV in eyes with an osteoma and prevents a decrease of vision in eyes with a choroidal osteoma with a CNV.

To describe fundus autofluorescence (FAF) characteristics associated with choroidal osteomas and their secondary complications. Retrospective descriptive case series of six eyes of five patients with choroidal osteomas. Findings of FAF correlated with visual acuity, clinical features, lesion characteristics, and findings from other imaging modalities. All 6 choroidal osteomas (100%) had totally or partially calcified, orange portions that were isoautofluorescent. Partial decalcification also produced areas of hyperautofluorescence and granular hypoautofluorescence corresponding to overlying retinal pigment epithelium mottling in 3 eyes (50%). Total decalcification with retinal pigment epithelial atrophy produced decreased FAF in 2 eyes (33%). Serous retinal detachment was present in 3 eyes (50%). When the overlying retinal pigment epithelium was viable, hyperautofluorescence as a result of elongation of the outer segments of photoreceptor was observed. In one eye where geographic atrophy of the retinal pigment epithelium was present, FAF was decreased even in the presence of serous retinal detachment. Portions of three partially or totally decalcified osteomas within the treatment field of photodynamic therapy for choroidal neovascularization were hypoautofluorescent. Four eyes (67%) had reduced foveal FAF and visual acuity <20/20, while both eyes with foveal isoautofluorescence had normal (20/20) visual acuity. Calcified portions of choroidal osteomas not previously treated with photodynamic therapy were isoautofluorescent. Decalcification and secondary complications of serous retinal detachment, choroidal neovascularization, and geographic atrophy altered foveal autofluorescence and were associated with reduced visual acuity.

We share a case of a 16-year-old girl who presented with blurring of vision in her left eye since 1 year, with fundus examination suggestive of choroidal osteoma. The detailed clinical, complimentary examinations, and multimodal imaging – fundus photo, optical coherence tomography, fundus fluorescein angiography, and B-scan ultrasonography – of the case are discussed. Choroidal osteoma is a rare benign ossifying tumor of unknown etiology characterized by mature bone replacing choroid, more commonly unilateral and predominantly affecting young females, typically manifesting in the teenage years or in the early 20s. Although choroidal osteoma is a rare benign tumor, accurate diagnosis is important because it is similar to amelanotic choroidal melanoma and metastatic choroidal carcinoma which require treatment and it is important to keep patients under observation for early detection of treatable choroidal neovascularization and other complications.

A 24-year-old man visited for imagedistortion in his left eye on 10 Decem-ber 2004. His past medical and familyhistory were negative. Visual acuity(VA) was 20⁄20 in the right eye and20⁄25 in the left. Fundus examinationand ultrasonogram of the left eyerevealed a choroidal osteoma (CO).On 20 July 2005, image distortionworsened. Lesion size enlarged andsubfoveal haemorrhage was noted. Asingle session of photodynamic ther-apy (PDT) with verteporfin for thischoroidal neovascularization (CNV)was performed at another hospital.After 3 months, vision decreased tohand motion and the size of the CNVincreased.When the patient visited our hospi-tal on 27 June 2006, VA of his lefteye was finger counting at 20 cmbecause of the CO with juxtafovealCNV and subfoveal haemorrhage.After a relevant discussion with thepatient and considering the locationof CNV, treatment by intravitrealbevacizumab (Avastin ; Genentech)1.25 mg, 0.05 ml injection was chosen.It was performed on 18 October and27 December 2006 without any com-plications. The size and leakage of theCNV reduced and subfoveal haemor-rhage decreased (Fig. 1). VA increasedto 20⁄200 and stabilized until thepatient’s last visit at 8 August 2007.

Optical Coherence Tomography of Choroidal Osteoma in 22 Cases: Evidence for Photoreceptor Atrophy over the Decalcified Portion of the Tumor