Choroidal Osteoma Research Articles

Detection of choroidal calcium by computed tomography scan quantitative computerized tomography: a clinically useful diagnostic tool

Objective Quantitative computerized tomography (QCT), a scanning method routinely used to classify tissues based on their radio density, was used to detect choroidal calcification. Design Case reports. Participants Two patients with known or suspected choroidal calcium-choroidal osteoma (CO) and pseudoxanthoma elasticum (PXE) and five normal control subjects were assessed. Intervention The CT scan of choroid, B-scan ultrasound, fluorescein angiography. Main outcome measures The QCT scan used to detect the presence of calcium based on a readout greater than 90. Results Calcium was detected bilaterally, as predicted, in PXE, and in both eyes of CO, although only one eye had a clinically noted lesion. No control eye gave a scale readout consistent with calcium. No findings suggestive of calcium were noted with B-scan ultrasound or fluorescein angiography in PXE or CO. Conclusion The QCT can detect calcium in the choroid when B-scan ultrasonography and CT scan fine sectioning fail to identify calcium despite high clinical suspicion.

Presence of bilateral limbal dermoids and choroidal osteomas in a family with inherited limbal dermoids

We report a case of bilateral limbal dermoids and bilateral choroidal osteomas in a 14-year-old girl with no extraocular anomalies. Histopathological examination of a limbal lesion confirmed the clinical diagnosis of dermoid. Computerized tomography and ultrasonography were compatible with a diagnosis of choroidal osteoma. Limbal dermoids were present in the patient's mother, in a brother with Down syndrome, and in an aunt with no choroidal osteoma. The present pedigree is compatible with autosomal dominant inheritance of bilateral limbal dermoids. The same gene may be involved in the pathogenesis of ocular choristomas in same patients.

A long-term follow-up of choroidal osteoma.

To provide long-term follow-up information on a large series of patients with choroidal osteoma. Review of patients with a diagnosis of choroidal osteoma who had been examined at the Bascom Palmer Eye Institute, Miami, Fla, or known to one of us (J.D.M.G.). Information was obtained from hospital medical records or by a questionnaire sent to referring ophthalmologists. Life-table analysis was used to study the loss of vision and development of choroidal neovascularization. We followed up 36 patients, 31 (89%) were female, mean age, 21 years (range, 5-54 years) for a mean of 10 years (range, 2-22 years). Growth was observed for 9 (41%) of 22 well-documented osteomas. The probability of loss of visual acuity to 20/200 or worse was 58% by 10 years and 62% by 20 years. The probability of developing choroidal neovascularization was 47% by 10 years and 56% by 20 years. Successful treatment of the choroidal neovascularization with laser photocoagulation was performed for 5 (25%) of 20 patients. Most patients with choroidal osteomas maintain good vision in at least 1 eye, but they have a high risk of developing choroidal neovascularization. When this occurs, only a minority can be successfully treated with laser photocoagulation.

Choroidal Osteoma in an Infant

To report an 8-month-old infant with choroidal osteoma. Case report. The patient, who had bilateral yellow stippling at the posterior pole, was followed up for 8 years. Both fundi developed creamy, irregular scalloped lesions. Computed tomography showed a bony plate at the posterior pole bilaterally. We believe that this is the youngest patient reported to have choroidal osteoma.

Ophthalmic features of the organoid nevus syndrome

The organoid nevus (sebaceous nevus) syndrome is characterized primarily by cutaneous sebaceous nevus, seizures, and epibulbar choristomas. On the basis of ophthalmoscopic and computed tomographic studies, a yellow fundus lesion recently observed in this syndrome has been called a coloboma by some investigators and a choroidal osteoma by others. This study was undertaken to review our personal experience with the organoid nevus syndrome, to review the English language literature on the subject, and to address some misconceptions regarding its ocular manifestations. We reviewed the records of patients with the organoid nevus syndrome who were personally evaluated by the investigators. The ocular findings were studied in more detail, with emphasis on the epibulbar and fundus lesions. We identified five patients with the organoid nevus syndrome. Four had a classic sebaceous nevus in the facial and scalp area, and 2 had seizures and arachnoid cysts. All 5 patients had an epibulbar tumor, which proved to be a complex choristoma in one case that was studied histopathologically. A characteristic ophthalmoscopic feature, observed in the 4 patients with clear ocular media, was a flat yellow discoloration of the posterior fundus, of variable size and shape, which appeared to correlate with a dense plaque noted on ultrasonography and computed tomography. In 1 case, histopathologic examination showed that this posterior lesion contained intrascleral cartilage. Our observations and a review of the literature indicated that the organoid nevus syndrome has varied manifestations. Like the closely related phakomatoses, it often occurs as a forme fruste, without full expression of the syndrome. The most important ocular manifestations are an epibulbar mass, compatible with a complex choristoma, and focal yellow discoloration in the fundus, probably related to intrascleral cartilage.

Ocular Manifestations of the Organoid Nevus Syndrome

The organoid nevus (sebaceous nevus) syndrome is characterized primarily by cutaneous sebaceous nevus, seizures, and epibulbar choristomas. Based on ophthalmoscopy and computed tomography (CT), a yellow fundus lesion recently observed in this syndrome has been called a coloboma by some authors or a choroidal osteoma by others. This study was undertaken to review the authors' personal experience with the organoid nevus syndrome, to review the English language literature on the subject, and to address some misconceptions regarding its ocular manifestations. The authors reviewed the records of patients with the organoid nevus syndrome who were personally evaluated by the authors. The ocular findings were studied in more detail, with emphasis on the epibulbar and fundus lesions. The authors identified five patients with the organoid nevus syndrome. Four had a classic sebaceous nevus in the facial and scalp area and two had seizures and arachnoid cysts. All five patients had an epibulbar tumor, which proved to be a complex choristoma in one case that was studied histopathologically. A characteristic ophthalmoscopic feature, observed in the four patients with clear ocular media, was a flat, yellow discoloration of the posterior fundus, of variable size and shape, that appeared to correlate with a dense plaque noted on ultrasonography and CT. In one case, histopathologic studies showed that this posterior lesion contained intrascleral cartilage. The authors' observations and a review of the literature indicated that the organoid nevus syndrome has varied manifestations. Just like the closely related phakomatoses, it often occurs as a forme fruste, without full expression of the syndrome. The most important ocular manifestations are an epibulbar mass, compatible with a complex choristoma, and focal, yellow discoloration in the fundus, probably related to intrascleral cartilage.

Choroidal Osteoma

The choroidal osteoma is a benign, ossifying tumor of the choroid that typically is found in young, healthy women in the second or third decade of life. Its pathogenesis is unknown. This tumor is clinically unilateral in 75% of cases and tends to be located in the juxtapapillary region. Choroidal neovascularization may occur in up to one-third of cases. The choroidal osteoma must be differentiated from other intraocular tumors as well as cases of dystrophic and metastatic calcification. Ultrasonography and computed tomography may help in the diagnosis by demonstrating a calcified plaque at the level of the choroid. Fluorescein angiography and ICG videoangiography may demonstrate choroidal neovascularization, which may be amenable to treatment by laser photocoagulation.

Atlas of Intraocular Tumors

In Atlas of Intraocular Tumors , authors Sanborn, Gonder, and Shields have put together a pictorial review of a wide variety of intraocular tumors of the eye. Chapters 1 and 2 cover tumors of the iris and ciliary body, including various types of nevi, cysts, melanocytomas, melanomas, adenomas, and metastatic and lymphoid tumors. The third chapter reviews neoplasms of the retina, including retinoblastoma, retinocytoma, hamartomas of the retina and retinal pigment epithelium (RPE) as well as the whole spectrum of hemangiomas of the retinal vascular system, arteriovenous malformations, and retinal macroaneurysms. Choroidal tumors, including nevi, melanoma, osteomas, hemangiomas, neurilemoma, and metastatic tumors of the choroid, are presented in chapter 4. Chapter 5 presents primary and metastatic optic nerve tumors, ie, melanocytoma, sarcoid, and leukemic infiltration. The last three chapters are devoted to neoplasms and lesions affecting the RPE, vitreous, and sclera, mainly RPE hypertrophy, adenocarcinoma of RPE, leukemic and lymphoid

Choroidal osteoma: an atypical presentation

Choroidal osteomas are benign osseous tumors of the choroid. These tumors typically occur unilaterally in the juxtapapillary region in young, healthy, white females. The fundus appearance is a yellow-white or orange-red elevated lesion with sharply demarcated irregular borders. Potential complications include growth and visual compromise secondary to choroidal neovascularization. Management involves photodocumentation with annual funduscopic examination and daily Amsler Grid self assessment. Laser photocoagulation is advocated in the presence of choroidal neovascularization within the arcades. A 70-year-old male presented with a unilateral subretinal elevated yellow-orange mass. Although atypical in presentation, the lesion was diagnosed as a choroidal osteoma based on the funduscopic appearance, computed tomography, ultrasonography and serologic testing.

Patterns of indocyanine green videoangiography of choroidal tumours.

Indocyanine green video-angiography is a recently popularised technique for imaging the retina and choroid. Little is known about the value of this technique in the evaluation of choroidal tumours. Indocyanine green video-angiograms of 51 patients with choroidal tumours, including 32 with choroidal melanoma, five with choroidal naevus, four with choroidal metastasis, four with choroidal haemangioma, one with choroidal osteoma, one with choroidal vortex vein varix, and four with irradiated choroidal melanoma were reviewed in a masked fashion. The choroidal melanoma group achieved maximal fluorescence at an average of 18.2 (range 0.4 to 60) minutes after injection. At maximum fluorescence, the pattern varied from hypofluorescent in 17 cases, to isofluorescent in eight cases, and hyperfluorescent in seven cases. Non-pigmented choroidal melanoma generally showed an earlier average onset of fluorescence than the pigmented choroidal melanoma (mean 28 versus 185 seconds, respectively) (p = 0.04). The two subgroups otherwise overlapped substantially in angiography characteristics. The choroidal metastasis group demonstrated maximal fluorescence at an average of 8.9 (range 1.7 to 13) minutes. All choroidal metastases had a homogeneous diffuse fluorescence with late isofluorescence. The choroidal metastases allowed a subtle blurred perception of the normal choroidal pattern through the tumour. The choroidal haemangioma group revealed very characteristic findings with an onset of filigree fluorescence at an average of 0.6 (range 0.4 to 0.7) minutes with little variation. The maximal fluorescence was typically hyperintense in all cases and was achieved at an average of 1.2 minutes and was reached by 1.8 minutes in all cases. In these cases the fluorescence appeared as a lacy diffuse 'fluorescent mulberry' pattern with visibility of the vascular channels and demonstrated 'washout' of the dye in the late frames. Indocyanine green may be a useful adjunct in the differentiation of amelanotic choroidal melanoma, choroidal metastasis, and choroidal haemangioma. The different patterns of fluorescence may be explained by the intrinsic choroidal vascular architecture in these three tumours.

The role of fat-suppression technique and gadopentetate dimeglumine in magnetic resonance imaging evaluation of intraocular tumors and simulating lesions.

Recent studies have shown that contrast (gadopentetate dimeglumine)-enhanced magnetic resonance imaging is useful in evaluating intraocular tumors and differentiating uveal melanoma from other simulating lesions. The purpose of this study was to study prospectively the role of fat-suppression technique and gadopentetate dimeglumine-enhanced magnetic resonance imaging in the evaluation of intraocular neoplasia. Forty-three uveal melanomas and 20 other simulating intraocular lesions from 63 patients were prospectively evaluated on non-contrast-enhanced and post-contrast-enhanced T1-weighted images with and without fat-suppression technique and on T2-weighted images. Forty-one uveal melanomas (95%) were detected with standard pulse sequences and showed the characteristic hyperintense signal on non-contrast-enhanced T1-weighted images and hypointense signal with respect to the vitreous on T2-weighted images. All 33 uveal melanomas evaluated on non-contrast-enhanced T1-weighted images with fat-suppression technique were detected on non-contrast-enhanced images with fat-suppression technique. The intensity of the signal was statistically associated with the degree of pigmentation of the tumor on T1-weighted images with fat-suppression technique (P = .03). On post-contrast-enhanced T1-weighted images with or without fat-suppression technique, the 43 uveal melanomas showed enhancement. The degree of tumor enhancement was not statistically related to the degree of tumor pigmentation or the location of the tumor. Among the other simulating intraocular lesions, choroidal metastasis, retinoblastoma, choroidal leiomyoma, and medulloepithelioma demonstrated the same features on magnetic resonance imaging studies as uveal melanoma demonstrated on non-contrast-enhanced and post-contrast-enhanced T1-weighted images with or without fat-suppression technique. In our series, choroidal hemangioma, choroidal osteoma, posterior scleritis, retinal hemangioma, and Coats' disease can be differentiated from other amelanotic intraocular tumors by their characteristics on magnetic resonance imaging studies. We concluded that pre-and post-contrast-enhanced T1-weighted images with fat-suppression technique are most helpful in detecting small intraocular tumors with a thickness of more than 1.8 mm and in evaluating intraocular neoplasms and simulating lesions, particularly when T2-weighted images are not available. Moreover, in juxtapapillary choroidal or retinal tumor, fat-suppression technique may help in the detection of possible optic nerve or orbital extension by improving the conspicuousness of the tumor.

An Atypical Fulminant Course of Choroidal Osteoma in Two Siblings

We studied two cases of bilateral choroidal osteoma in an otherwise healthy 5-year-old boy and his only sibling, a 7-year-old sister. Both children were known to have normal fundus appearances at younger ages. The tumor showed slow growth in all four eyes, but severe visual acuity loss developed in three eyes because of neovascular complications that could not be effectively treated by photocoagulation. Secondary retinal cysts developed in three eyes.

Idiopathic Sclerochoroidal Calcification: New Observations

Two cases of idiopathic sclerochoroidal calcification are reported with follow-up of two and ten years. In addition we have reviewed 102 cases of choroidal osteoma, including six misleading case reports which actually described idiopathic sclerochoroidal calcification and not choroidal osteoma. Clinical manifestation and the angiographic features of idiopathic sclerochoroidal calcification are outlined. The differential diagnosis of intraocular deposition of calcium salts is discussed in detail.

Idiopathic Sclerochoroidal Calcification

AbstractIdiopathic sclerochoroidal calcification is a disorder that is often overlooked, occurring at the level of the posterior sclera and choroid in older patients. Clinically, it may be confused with choroidal osteoma, choroidal metastasis, amelanotic choroidal nevus, or choroiditis. The authors present seven cases of this unusual entity. The patients ranged in age from 58 to 80 years. In all cases, the yellow lesions were clinically estimated to be at the level of the choroid and sclera, and they were usually located superior to the superotemporal retinal vascular arcade. Systemic investigation of calcium and phosphate metabolism was normal in all patients. The classic clinical manifestations and differentiation of this unusual entity from other similar fundus lesions is discussed.

In Vivo Phosphorus 31 Magnetic Resonance Spectroscopy of Human Uveal Melanomas and Other Intraocular Tumors

We studied the feasibility of using the surface coil probe technique for the noninvasive in vivo study of ocular tumors by phosphorus 31 magnetic resonance spectroscopy. The characteristic organophosphate metabolites of suspected uveal melanomas before proton beam irradiation were determined qualitatively by phosphorus 31 magnetic resonance spectroscopy in vivo using a three-turn surface coil. Spectra of choroidal hemangioma, osteoma, and metastasis were also obtained in vivo and compared with those of uveal melanomas. Analysis of spectra performed at 1.5 T showed significant peaks of phosphomonoesters, inorganic phosphate, phosphodiesters, phosphocreatine, and adenosine 5'-triphosphates. The unusually high concentration of phosphodiesters may be considered as a marker for uveal melanomas and other choroidal tumors. By analyzing the ratio of phosphocreatine to phosphodiesters spectral area values, we interpreted qualitatively spectra of intraocular tumors to differentiate malignant tumors from benign lesions. Nevertheless, the main limitation of interpreting the spectra was their contamination by signals from surrounding tissues.

MAGNETIC RESONANCE IMAGING IN CHOROIDAL OSTEOMA

High resolution magnetic resonance imaging (MRI) with surface coil and contrast material was used to demonstrate the presence of bone in choroidal osteoma. Contrary to initial impressions, choroidal osteoma does not show typical negative image of the bone in MRI scans. The ossifying tumor of the choroid appears as a bright (hyperintense) signal on T1-weighted images and as an area of relative low intensity on T2-weighted images. On contrast T1-weighted scans, the tumor showed gadolinium-DPTA (Gd-DPTA) enhancement. These MRI findings are discussed in relation with histopathologic features of choroidal osteoma and the presence of fat marrow in the intertrabecular spaces of the bone at the level of the choroid.

ARGON LASER PHOTOABLATION OF A CHOROIDAL OSTEOMA

Choroidal osteomas are rare, juxtapapillary choroidal tumors, which are usually unilateral but can be bilateral in as many as 30% of patients. Choroidal neovascularization may complicate this condition and be associated with severe visual loss. A patient treated with argon laser photocoagulation for SRNVM is described, and clinical and radiographic evidence of destruction of the osteoma during an 8-year follow-up is presented. The possible mechanisms of laser's ablative effect on bone, in addition to clinical application for choroidal osteoma treatment, are discussed.

Decalcification of a choroidal osteoma.

A 56-year-old man presented with a clearly defined orange tumour in the posterior pole of his left eye. A choroidal osteoma was suspected, and ultrasonography confirmed the diagnosis. Fluorescein angiography demonstrated subretinal neovascularisation on the nasal edge of the tumour, which was treated with krypton laser photocoagulation twice. Recurrent subretinal neovascularisation occurred one year later and was not amenable to treatment. Three years after the patient first presented, thinning of the tumour was noted on follow-up examination. During the next 15 months the tumour completely disappeared, leaving an area of retinal pigment epithelial and choroidal atrophy. Total decalcification of the choroidal osteoma was demonstrated by ultrasonography.

Spontaneous Involution of a Choroidal Osteoma

To the Editor. —Spontaneous involution, a rarely observed occurrence in a choroidal osteoma, was noted in the eye of a 25-year-old woman who had been followed up for 8 years. Report of a Case. —In 1978 a 17-year-old white woman noted blurred vision in her right eye. A yellow-red choroidal lesion measuring 3×4 disc diameters was found in the right posterior pole. It involved the superior half of the macula, reducing visual acuity to 20/30 (Fig 1, top). Ultrasonographic (Fig 1, bottom) and computed tomographic findings supported the clinical diagnosis of a choroidal osteoma. In 1979, while the patient participated in a basketball game, her visual acuity suddenly decreased to 20/60 due to the development of a serous retinal detachment associated with a hemorrhage under the retinal pigment epithelium overlying the center of the lesion (Fig 2, top). The hemorrhage and detachment slowly resolved, leaving rather mottled-appearing retinal pigment epithelium

Prinzipien der intraokularen Ossifikation am Beispiel der sekundären Aderhautverknöcherung

Intraocular ossification seems to follow general pathogenic principles. Ossification requires besides local changes in carbon dioxide tension and local increase of phosphate ions first a rich vascular supply--therefore the peripapillary region is always involved, alone or in conjunction with other sites--and second probably a direct or indirect influence of the Retinal Pigment Epithelium (RPE). The growth of the bone occurs slowly and leads to a relative uniform morphology. Osteogenesis is triggered by chronic intraocular changes (of the RPE), and ossification is almost always combined with a longstanding retinal detachment. The primary lesion (e.g. trauma, inflammation or tumor) plays a minor or no role. Differences between secondary intraocular bone formation (following a well known lesion) and primary choroidal osteomas could be explained by a different strength of the osteogenic (RPE-)stimulus. In the light of our investigations (29 phthitic or chronically hypotonic eyes, 12 of them with intraocular bone formation) and the literature primary osteomas of the choroid have to be interpreted more likely as secondary processes, possibly following a (birth-)trauma, than as congenital choristomas.