Solitary idiopathic choroiditis

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1 Optometry (2007) 78, Solitary idiopathic choroiditis Kimberly D. Kohne, O.D., a Victor E. Malinovsky, O.D., a and Hua Gao, M.D., Ph.D. b a School of Optometry and b Department of Ophthalmology, School of Medicine, Indiana University, Bloomington, Indiana. KEYWORDS Intraocular tumors; Posterior inflammatory condition; Optical coherence; Topography; Solitary idiopathic choroiditis Abstract Solitary idiopathic choroiditis (SIC) is often confused with intraocular masses and inflammatory conditions and has only recently been identified as a distinct clinical condition. Jerry Shields, M.D., was the first to put a name on this condition. The clinical findings and features that distinguish it from other conditions are discussed in this report. A comprehensive examination with supplemental testing such as fluoresceine angiography, B scan, and extensive laboratory workup is necessary to rule out the differentials of SIC. These differentials include inflammatory conditions and retinal and choroidal tumors. SIC has both an active and an inactive phase. The treatment for the condition is controversial. Our patient presented with a suspicious intraocular mass that was diagnosed as SIC after an extensive ocular examination and laboratory workup was completed. Optometry 2007;78: An elevated choroidal lesion can be a serious, and, in some cases life threatening, condition. Often the diagnosis is confusing, and the patient requires extensive ocular and medical workup to determine the exact etiology. However, even after extensive workup, the precise diagnosis can still be unclear. A rare condition, solitary idiopathic choroiditis (SIC) has only recently been recognized and reported in literature as an important differential diagnosis for conditions such as choroidal metastasis and choroidal osteoma, as well as inflammatory conditions like sarcoid and histoplasmosis. SIC is a finding that is easily confused with more serious chorioretinal conditions such as intraocular tumors. It has a granulomalike appearance and is located in the choroid. The etiology currently is unknown, and all laboratory and ophthalmic findings are negative. It is a diagnosis of exclusion that is made only after completing extensive systemic and ophthalmic workups in conjunction with careful follow-up examinations. This report reviews a case of an isolated, Corresponding author: Kimberly Kohne, O.D., 800 East Atwater, Bloomington, Indiana kkohne@indiana.edu elevated choroidal lesion in which all diagnostic tests were negative and the elaborate workup required a final diagnosis of SIC. Case summary The patient, a 63-year-old white woman, presented with a chief complaint of near blur. She denied any history of ocular disorder, ocular trauma, or ocular surgery. She had a history of hypertension controlled by hydrochlorothiazide, seasonal allergies treated with patanol, and polycystic ovary syndrome for which she was currently taking metformin. Her best-corrected visual acuities were 20/20 in both eyes. Slit lamp examination showed mild nuclear sclerotic cataract in both eyes. Intraocular pressures (IOPs), taken by Goldmann tonometry, were 18 mmhg in both eyes at 1:40 PM. Fundus examination found her optic nerves to be distinct and round with good color and 0.4/0.4 in both eyes. The vitreous was clear in both eyes. A mild epiretinal membrane was found in the right eye. In the left eye, a yellow, elevated choroidal mass was detected along the superior temporal arcade approximately 3 disc diopters (DD) from the macular area /07/$ -see front matter 2007 American Optometric Association. All rights reserved. doi: /j.optm

Kohne et al Clinical Care 177 Figure 1 A chorioretinal lesion 3 4 DD in size, elevated and 3 DD from the macula with mild subretinal fluid.

The retinal specialist described the lesion as being ¾ DD in size, mildly elevated with subtle subretinal fluid, and with a shunt vessel growing into the mass of the left eye (see Figures 1 and 2).

The following tests were ordered: mammogram, chest x-ray, angiotensin-converting enzyme (ACE) levels, lysozyme levels, liver enzyme levels, toxoplasmosis IgG and IgM, OB/GYN examination, complete

levels, optical coherence topography (OCT), fluorescein angiography (FA), indocyanine green angiography (ICG), and a B-scan. Early phase of fluorescein angiography showing hypofluo- Figure 3 rescence.

2 Kohne et al Clinical Care 177 Figure 1 A chorioretinal lesion 3 4 DD in size, elevated and 3 DD from the macula with mild subretinal fluid. The tentative diagnosis of a suspicious, focal choroidal lesion of the left eye was made. The patient was referred to a retinal specialist for further evaluation. The retinal specialist described the lesion as being ¾ DD in size, mildly elevated with subtle subretinal fluid, and with a shunt vessel growing into the mass of the left eye (see Figures 1 and 2). Systemic and laboratory workups were ordered to aid in determining the etiology of the mass. The following tests were ordered: mammogram, chest x-ray, angiotensin-converting enzyme (ACE) levels, lysozyme levels, liver enzyme levels, toxoplasmosis IgG and IgM, OB/GYN examination, complete blood count, magnetic resonance imaging (MRI) of the abdomen and pelvic region, colonoscopy, bone scan, purified protein derivative (PPD), cat-scratch antibodies, carcinoembryonic antigen (CEA) levels, optical coherence topography (OCT), fluorescein angiography (FA), indocyanine green angiography (ICG), and a B-scan. Early phase of fluorescein angiography showing hypofluo- Figure 3 rescence. The OCT found an elevation of the choroid and retina with disruption of the retinal pigment epithelium (RPE) layer. There was no obvious surrounding or overlying fluid. FA found hypofluorescence in the arterial phase that progressively moved to hyperfluorescence in the late phase. Early FA found no neovascular nets, and the late phase had distinct margins (see Figures 3and 4). ICG indicated an early hypofluorescent spot. The B-scan showed a mild elevation with medium reflectivity and no calcifications. Results of all laboratory tests were normal except for the lysozyme level, alanine aminotransferase (ALT) level, alkaline phosphate level, and the CEA level. The lysozyme value was found to be 25 ug/ml, with normal being between 9 and 17 ug/ml. The CEA level was 7 ng/ml. For a Figure 2 Lesion with abnormal shunt vessels and a distinctive redorange halo. Late phase of fluorescein angiography showing hyperfluo- Figure 4 rescence.

178 Figure 5 Patient 9 months after initial examination, more prominent shunt vessels and well-demarcated margins with no obvious progression. nonsmoker the normal level is 2.5 ng/ml.

The ALT and alkaline phosphate levels indicated the need for an MRI of the abdomen to see if any tumors were present in the liver. The MRI found no lesions in the liver.

3 178 Figure 5 Patient 9 months after initial examination, more prominent shunt vessels and well-demarcated margins with no obvious progression. nonsmoker the normal level is 2.5 ng/ml. These tests were reordered, and, upon further testing, the results were found to be normal. The ALT and alkaline phosphate levels indicated the need for an MRI of the abdomen to see if any tumors were present in the liver. The MRI found no lesions in the liver. Normal laboratory testing reports ruled out inflammatory conditions and metastasis/malignancy. After all of the laboratory and ophthalmic testing was performed, a retinal expert in the area of choroidal and retinal tumors was consulted. After his review of the case, he found a tentative diagnosis of SIC. The patient was informed that all serious tumors of the eye had been ruled out, and the best management at this time would be careful observation. The patient was to return to the clinic in 3 months for follow-up care. At the 3-month follow-up visit, the lesion was stable. Photos were taken for documentation, and the patient then followed up in 6 months. At this visit, the lesion was found to be slightly smaller than it was at the initial visit with a larger halo surrounding the lesion (see Figure 5). An OCT was performed at this visit, and the lesion was still elevated, but appeared to have scarred down from the OCT image taken at the initial visit (see Figure 6). The patient was to continue follow-up visits on a 6-month schedule unless there was a change in vision. Optometry, Vol 78, No 4, April 2007 testing fail to reveal any specific etiology for the choroidal lesion.1 The lesion appears as a discrete, mildly elevated, yellowish-white mass. It can be in either an active or inactive phase. The primary distinguishing feature of an active phase lesion is its ill-defined borders. The active lesions are often characterized by yellow, intraretinal exudation and localized subretinal fluid. There can also be retinal vascular dilation, which can lead to focal retinal hemorrhages. The FA findings will be hypofluorescent in the early vascular filling phase moving progressively to hyperfluorescent in later phases, with possible leakage if subretinal fluid is present. When the lesion causing inflammation subsides, it develops more distinct margins. After an active lesion moves to the inactive phase, it usually stabilizes. Although rare, an inactive lesion can revert back to the active phase.1 This type of lesion has never before been labeled or discussed in the literature. However, Hong et al.7 describe lesions of a similar appearance in They termed the lesions unifocal helioid choroiditis mainly because the lesions have a sunlike appearance. Hong et al.7 saw only 6 patients, and all of these most likely had active lesions with accompanying symptoms. A lesion in the inactive phase has distinct borders. At times there is a red-orange halo around the lesion with ill-defined borders.2 Retinochoroidal shunt vessels in or around the lesion can also be seen. Usually, the RPE is minimally affected with the inactive lesion.1 There are several differential diagnoses that a doctor must consider when evaluating an elevated lesion of the retina or the choroid. These include both retinal and choroidal tumors as well as inflammatory conditions that cause similar-looking choroidal granulomas. Retinal and choroidal tumors are differentiated from SIC by their characteristic appearances (see Table 1). Amelanotic choroidal melanoma is fairly large in diameter and thickness, lacks yellow exudates, and typically has overlying drusen or goldenbrown mottling surrounding the lesion.3 SIC usually lacks Discussion SIC is a rare and highly atypical choroidal finding most often found posterior to the equator. It can occur at any age but is most often found in people between the ages of 20 and 50. It lacks predilection to any race, sex, or unilaterality. The vision of patients with this condition normally is good, except when the lesion is in the juxtapapillary or macular area. IOP and anterior chamber are normal. There are no cells found in the vitreous. Medical, laboratory, radiologic, and ocular examinations with ophthalmic supplementary Figure 6 OCT image of the patient 9 months after initial examination. Note increased reflectivity of the choroidal tissue.

4 Kohne et al Clinical Care 179 Table 1 Tumor differentials to SIC Retinal and choroidal tumors Ocular appearance Differentiation from SIC Amelanotic choroidal melanoma Choroidal metastasis Choroidal osteoma Retinoblastoma Nondistinct borders, yellow-white appearance, fairly large diameter and thickness, can produce overlying drusen, does not produce a red halo surrounding lesion, brown mottling surrounding the lesion. Creamy yellow in appearance, FA in late phase is not intense in appearance, can produce RD, vitreal hemorrhage, proptosis in extreme cases. Yellow-white to red-orange in appearance, the edges are scalloped, high reflectivity on ultrasound scan, can cause serous macular detachments or neovascularization. Leukocoria, dome-shaped lesion that is whitish pink in appearance, can cause nonrhegmatogenous RD, found predominately in children, has early hyperfluorescence that continues to progress in late stages, has high reflectivity on ultrasound scan. SIC is not as large in diameter and thickness, doesn t produce a brown mottling around lesion, although it can cause some pigmentation, doesn t produce overlying drusen. SIC is a clear whitish yellow lesion, the late FA is very intense in appearance, not known to cause RD or vitreal hemorrhage, does not cause proptosis. Systemic testing necessary to completely rule out metastasis. SIC does not have scalloped edges or neovascularization, SIC does not have high reflectivity. SIC typically affects people between the ages of 20 and 50; however, Hong noted similar lesions to SIC in children. Does not cause leukocoria or nonrhegmatogenous RD. SIC has hypofluorescence in early stage that progresses to hyperfluorescence. Does not have high reflectivity. overlying drusen in its appearance. A choroidal metastasis has a creamy yellow appearance with little inflammation associated with the mass. It can cause an exudative retinal detachment (RD). When an FA is performed, it is less intense in the late phase; SIC has an intense hyperflouorescent late phase. SIC is usually a whitish-yellow lesion. Solitary choroidal hemangioma has more of a reddishorange color. During FA, there is a more diffuse mottled Table 2 Inflammatory differentials to SIC Inflammatory condition Ocular manifestation Differentiation from SIC Tuberculosis Histoplasmosis Nodular posterior uveitis Syphilis Granulomatis anterior uveitis, conjunctival granulomas, band keratopathy, candle wax drippings on veins, choroidal granulomas. Yellow-white, multifocal choroidal lesion, granulomatous/nongranulomatous anterior uveitis. Classic triad of punched out lesions, peripapillary atrophy, and neovascularization of the macula. Lacks any uveitic manifestation both anteriorly and posteriorly. Unilateral, multifocal chorioretinal lesion, has severe posterior uveitis response. Retinal or choroidal infiltrates, posterior uveitis, vascular sheathing. Depends on the stage: primary has a chancre; secondary will produce chorioretinitis, uveitis, optic neuritis, conjunctivitis, episcleritis, scleritis, etc.; tertiary will cause optic atrophy, IK, Argyll Robertson pupil. Primarily, SIC will not be positive for ACE laboratory testing. Secondly, SIC has no uveitic manifestations, band keratopathy, or conjunctival granulomas. Primarily, SIC will not have a positive PPD test or an abnormal chest x-ray. Secondly, SIC does not have any uveitic manifestations. SIC does not have the classic triad, but does lack the uveitic manifestation like histoplasmosis. Primarily, SIC will be negative on a Toxocara enzymelinked immunosorbent assay. Secondly, SIC does not have any uveitic manifestations. Laboratory testing will indicate the cause of the posterior uveitis, SIC laboratory results will all be negative. There are no uveitic manifestations with SIC. Primarily, SIC will be negative on the FTA-ABS and VDRL. Secondly, SIC does not cause uveitis, conjunctivitis, Argyll Robertson pupil, etc. FTA-ABS Fluorescent Treponemal Antibody Absorption Test; VDRL Venereal Disease Research Laboratory Test; IK interstitial keratitis.

5 180 Optometry, Vol 78, No 4, April 2007 Table 3 Laboratory tests done for differential diagnosis of SIC Laboratory testing done Complete blood count IgG IgM ALT AP ACE Lysozymes Cat scratch titer CEA Mammogram Chest x-ray OB/GYN examination MRI of abdomen/ pelvis Colonoscopy AP alkaline phosphate level. What the test investigates Abnormal blood counts Inflammation or injury to liver Problems with the bile duct Cat scratch disease Carcinogenic indicator in the blood Abnormal lesions in the breast Abnormal lesions in the lung, tuberculosis, sarcoidosis Abnormal lesions in the lower abdomen Abnormal lesions in abdomen/pelvis region Abnormal lesions in colon appearance in the arterial phase and some late staining, which differentiates it from the SIC FA. Choroidal osteoma has very well-defined margins that are usually scalloped in shape. There are no inflammatory signs with this lesion, and it has high reflectivity on ultrasound scan. It is very likely to cause neovascularization. Retinal astrocytic hamartoma is gray-white in color. The lesion is located in the sensory retina and has yellow foci of calcification. 4 Anumber of laboratory tests were performed on this patient to identify a possible metastatic tumor. These tests included mammogram, chest x-ray, OB/GYN check up, MRI of the abdomen and pelvis, and a colonoscopy. In the differential diagnosis of SIC, it is important to consider the inflammatory conditions that can have lesions that manifest as a solitary granulomalike lesion in the choroid (see Table 2). These granulomalike lesions have an appearance similar to the lesions seen in SIC patients. They include sarcoid and tuberculosis (TB). These conditions generally create a noticeable inflammatory response both posterior and anterior. The patient in this case lacked both. Laboratory testing can usually rule out most inflammatory conditions (see Table 3). 5,6 Additionally, there are inflammatory conditions, such as histoplasmosis and toxoplasmosis, that only superficially resemble SIC. These can be differentiated diagnostically through characteristics specific to each disease. 1,8,9 Histoplasmosis has a classic triad of punched out lesions, peripapillary atrophy, and neovascularization of the macula, none of which were seen in this patient. produces a posterior uveitis, which also was not observed in our patient. Treatment Currently, treatment of this condition is controversial. There has been no research to date to determine the optimal treatment. With inactive lesions, it is best to simply monitor the patient. However, for active lesions, especially lesions near the optic disc or macular region, some retinal specialists have used systemic steroids. With this particular patient, all appropriate diagnostic testing was performed to rule out serious or specific treatable lesions. Her condition has been monitored carefully. There has been a reduction in the size of the lesion, and there have been no signs of an inflammatory process. Conclusion SIC is a rare choroidal finding with no specific etiology that is typically benign but easily confused with serious choroidal findings. SIC is distinguished from other choroidal and retinal conditions through comprehensive systemic, laboratory, and ocular examinations. It is important for the optometrist to be aware of SIC as a possible diagnosis to avoid unnecessary treatment, which could ultimately cause more harm to the patient. References 1. Shields JA, Shields CL, Demirici H, et al. Solitary idiopathic choroiditis. Arch Ophthalmol 2002;120: McDonald R. Diagnostic and therapeutic challenges. Retina 2003; 23(6): Gunduz K, Shields CL, Shields JA, et al. Presumed choroidal granuloma with vitreous hemorrhage resembling choroidal melanoma. Arch Ophthalmol 1998;29: Shields CL, Shields JA, Augsburger JJ. Choroidal Osteoma (review). Surv Ophthalmol 1988;331: Olk RJ, Lipmann MJ, Cundiff HC, et al. Solitary choroidal mass as the presenting symptom in systemic sarcoidosis. BR J Ophthalmol 1983; 67: Jabbour NM, Faris B, Trempe CL. A Case of pulmonary tuberculosis presenting with a choroidal tuberculoma. Ophthalmology 1985;92: Hong PH, Jampol LM, Dodwell DG, et al. Unifocal helioid choroiditis. Arch Ophthalmol 1997;115: Rhee D, Pyfer M. The Wills eye manual: office and emergency room diagnosis and treatment of eye disease, 3rd ed. Philadelphia, PA: Lippincott Williams and Wilkins, Alexander L. Primary care of the posterior segment, 3rd ed. New York: McGraw-Hill, 2002.

Moncef Khairallah, MD

Moncef Khairallah, MD Department of Ophthalmology, Fattouma Bourguiba University Hospital Faculty of Medicine, University of Monastir Monastir, Tunisia INTRODUCTION IU: anatomic form of uveitis involving