Ophthalmic Surgery, Lasers and Imaging Retina

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Case Report 

Nodular Sclerochoroidopathy Simulating Choroidal Malignancy

Elham Hatef, MD; Jianmin Wang, MD; Mohamed Ibrahim, MD; Peykan Turkcuoglu, MD; Afsheen Khwaja, MD; Roomasa Channa, MD; Eric P. Suan, MD, FACS; Cathy DiBernado, RN, CDOS, ROUB; Yasir J. Sepah, MB, BS; Diana V. Do, MD; Quan Dong Nguyen, MD, MSc

Abstract

A 55-year-old man who had multiple subretinal and choroidal yellowish lesions and episcleral nodules for 1.5 years was diagnosed as having nodular sclerochoroidopathy after developing classic features of posterior scleritis with choroidal and serous retinal detachment. Long-term therapy with steroids in combination with mycophenolate mofetil resulted in regressed posterior scleritis and nodular lesions, as well as improved visual acuity. Nodular sclerochoroidopathy should be suspected in patients with subretinal and choroidal lesions and should be distinguished from choroidal neoplasm.

Abstract

A 55-year-old man who had multiple subretinal and choroidal yellowish lesions and episcleral nodules for 1.5 years was diagnosed as having nodular sclerochoroidopathy after developing classic features of posterior scleritis with choroidal and serous retinal detachment. Long-term therapy with steroids in combination with mycophenolate mofetil resulted in regressed posterior scleritis and nodular lesions, as well as improved visual acuity. Nodular sclerochoroidopathy should be suspected in patients with subretinal and choroidal lesions and should be distinguished from choroidal neoplasm.

Nodular Sclerochoroidopathy Simulating Choroidal Malignancy

From the Wilmer Eye Institute (EH, JW, MI, PT, AK, RC, CD, DVD, QDN), Johns Hopkins University School of Medicine, Baltimore, Maryland; the Department of Ophthalmology (JW), The Second Hospital of Hebei Medical University Shijiazhuang, Hebei Province, China; the Department of Ophthalmology (PT), Inonu University School of Medicine, Malatya, Turkey; and the Retina Care Center (EPS), Baltimore, Maryland.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Quan D. Nguyen, MD, MSc, Johns Hopkins Hospital, 600 North Wolfe Street, Maumenee 745, Baltimore, MD 21287. E-mail: qnguyen4@jhmi.edu

Received: December 19, 2009
Accepted: May 20, 2010
Posted Online: August 30, 2010

Introduction

Nodular posterior scleritis is an inflammatory process of the posterior sclera and is one of the causes of choroidal detachment. The condition is often idiopathic and unilateral.1 This nodular form of scleral inflammation may simulate other subretinal and choroidal lesions, such as melanotic choroidal melanoma.2 We describe a case of nodular scleritis with choroidopathy that resembled choroidal malignancy.

Case Report

A 55-year-old man with a history of diabetes mellitus was referred to the Wilmer Eye Institute in January 2007 complaining of wavy vision after cataract surgery in his right eye. The patient had a history of removal of a cutaneous pectoral lesion in 2001. Pathology report revealed “cytologic atypia” not diagnostic of melanoma. On examination, visual acuity was 20/40 in the right eye and 20/20 in the left eye. Funduscopic examination of the right eye was remarkable for multiple large, deep-orange lesions protruding from the subfoveal area and several yellow-orange lesions with retinal pigment epithelium (RPE) changes that were scattered throughout the posterior pole of the retina and extended temporally. Intraretinal hemorrhages were noted in the periphery (Fig. 1A). There were few intraretinal hemorrhages in the peripheral retina of the left eye (Fig. 1C). Fluorescein angiography was performed and demonstrated diffuse leakage. Optical coherence tomography (OCT) was also obtained and significant subretinal fluid was noted in the right macula (Fig. 1B). There were no abnormalities in the left eye (Fig. 1D).

(A) Right Eye at Initial Presentation. Fundus Photograph Illustrates Deep Orange-Yellow Protruding Lesions at the Inferior Temporal Arcade and Multiple Large Retinal Pigment Epithelium (RPE) Clumping Around the Fovea and Chorioretinal Folds Extending Periphery Starting from the Optic Disc (left). RPE Clumping and Chorioretinal Folds Were Noticed More Easily at the Early Phase of Fluorescein Angiography (FA) with Pinpoint Hyperfluorescences Surrounding the Fovea (middle). In the Late Phase of FA, Oozing of Fluorescein Was Noticed at the Inferior Temporal Arcade, Which Corresponds to the Lesion Site (right). (B) Optical Coherence Tomography (OCT) and Thickness Map of the Right Eye at Initial Presentation. On the OCT, Localized Subretinal Fluid and Retinal Thickening Surrounding the Fovea Were Noticed. RPE Contour Had a Mild Undulating Course with Localized RPE Detachment. Foveal Contour Was Preserved. (C) Left Eye at Initial Presentation. Fundus Photograph and Mid and Late Phases of FA Revealed Normal Macula with Normal Vasculature. (D) OCT and Thickness Map of the Left Eye at Initial Presentation. There Were No Abnormalities in the Left Eye.

Figure 1. (A) Right Eye at Initial Presentation. Fundus Photograph Illustrates Deep Orange-Yellow Protruding Lesions at the Inferior Temporal Arcade and Multiple Large Retinal Pigment Epithelium (RPE) Clumping Around the Fovea and Chorioretinal Folds Extending Periphery Starting from the Optic Disc (left). RPE Clumping and Chorioretinal Folds Were Noticed More Easily at the Early Phase of Fluorescein Angiography (FA) with Pinpoint Hyperfluorescences Surrounding the Fovea (middle). In the Late Phase of FA, Oozing of Fluorescein Was Noticed at the Inferior Temporal Arcade, Which Corresponds to the Lesion Site (right). (B) Optical Coherence Tomography (OCT) and Thickness Map of the Right Eye at Initial Presentation. On the OCT, Localized Subretinal Fluid and Retinal Thickening Surrounding the Fovea Were Noticed. RPE Contour Had a Mild Undulating Course with Localized RPE Detachment. Foveal Contour Was Preserved. (C) Left Eye at Initial Presentation. Fundus Photograph and Mid and Late Phases of FA Revealed Normal Macula with Normal Vasculature. (D) OCT and Thickness Map of the Left Eye at Initial Presentation. There Were No Abnormalities in the Left Eye.

Ultrasonography (contact B-scan and standardized A-scan) was performed and showed diffuse, irregular thickening of the ocular coats with focal areas of low reflective infiltration in the orbit adjacent to the globe wall (Fig. 2). The clinical and echographic differential diagnoses included diffuse melanoma, lymphoma, lymphoid hyperplasia, leukemia, or a metastatic process.

Echography of Right Eye at Initial Examination. Transverse (cross-Section) B-Scans Showed Thickening of the Ocular Coats (right Arrows) Nasally and Inferiorly (top: Left and Right). There Were Focal Areas of Low Reflectivity in the Orbit Adjacent to the Inferior Globe Wall (left Arrows). Longitudinal B-Scans Showed the Radial Extent of the Ocular Coats Thickening Nasally and Inferiorly (right Arrows) and the Low Reflective Lesions in the Orbit Adjacent to the Inferior Globe Wall (left Arrows) (center: Left and Right). Standardized A-Scans Showed the Areas of Low Reflectivity Within the Globe Wall Thickening (down Arrow, Left) and the Low Reflectivity of the Orbital Lesions Adjacent to the Inferior Globe Wall (down Arrow, Right) (bottom:left and Right).

Figure 2. Echography of Right Eye at Initial Examination. Transverse (cross-Section) B-Scans Showed Thickening of the Ocular Coats (right Arrows) Nasally and Inferiorly (top: Left and Right). There Were Focal Areas of Low Reflectivity in the Orbit Adjacent to the Inferior Globe Wall (left Arrows). Longitudinal B-Scans Showed the Radial Extent of the Ocular Coats Thickening Nasally and Inferiorly (right Arrows) and the Low Reflective Lesions in the Orbit Adjacent to the Inferior Globe Wall (left Arrows) (center: Left and Right). Standardized A-Scans Showed the Areas of Low Reflectivity Within the Globe Wall Thickening (down Arrow, Left) and the Low Reflectivity of the Orbital Lesions Adjacent to the Inferior Globe Wall (down Arrow, Right) (bottom:left and Right).

Orbital magnetic resonance imaging showed diffuse scleral enhancement in the right eye associated with mild focal thickening of the posterior medial orbital wall measuring 12 × 3 mm. Neuroimaging evaluation revealed thickening of the wall of the globe but no discrete lesions. Diagnostic transconjunctival anterior orbitotomy and diagnostic vitrectomy were performed in March 2007. During the orbitotomy, no scleral nodules were found externally that could be biopsied. Histopathological studies of the vitreous specimens did not reveal any malignant cells. Oncologic evaluation showed no evidence for a systemic malignancy, except for a moderate FDG (fluorine-18 2-fluoro-2-deoxy-D-glucose) uptake in a small nodular opacity in the right lung that was noted on positron emission tomography/computed tomography scan, which was thought to be inflammatory in nature. The nodular opacity in the right lung was observed and showed a decrease in size after 18 months. Based on the clinical ocular findings and evaluations, the diagnosis of nodular sclerochoroidopathy was made and the patient was monitored.

Visual acuity stabilized at 20/25 in the right eye for 14 months following orbitotomy and vitrectomy. No evidence of growth was noted by ultrasonography and no significant changes were noted on OCT and fluorescein angiography. In May 2008, the patient reported new onset of episodic right-sided frontal headache, as well as redness, photophobia, and blurriness of vision. On examination, visual acuity had decreased to 20/40 in the right eye. There was significant choroidal detachment and localized inferior exudative retinal detachment. Fundus photography confirmed clinical findings (Fig. 3). Fluorescein angiography detected early hyperfluorescence with multiple pinpoint foci of late leakage throughout the macula (Fig. 3). Ultrasonography of both anterior and posterior segments revealed diffuse posterior fundus thickening and choroidal detachments (Fig. 4); there was no anterior scleritis. Clinical and ancillary findings were consistent with posterior scleritis. Pulse intravenous methylprednisolone was administered (1,000 mg daily) for 3 days followed by 60 mg oral prednisone daily. The patient was also prescribed mycophenolate mofetil at 2,500 mg daily. The choroidal and exudative retinal detachment quickly resolved. Prednisone was tapered over a period of 11 months to 6 mg a day and the mycophenolate mofetil was maintained at 2,500 mg daily. During this period, visual acuity remained stable in both eyes. At the most recent visit, visual acuity was 20/20 in both eyes. Funduscopic examination of the right eye revealed no choroidal detachment, RPE changes in the macula, and a normal appearing optic disc. Scattered intraretinal hemorrhages consistent with diabetic retinopathy were noted in the left eye.

Fundus Photograph and the Early and Late Phases of Fluorescein Angiography of the Right Eye at the Time of Diagnosis of Posterior Scleritis. Fundus Photograph Illustrated Pronounced Chorioretinal Folds with Choroidal Detachment in This Acute Exacerbation (A). On Fluorescein Angiography (B and C), There Was Early Hyperfluorescence with Multiple Pinpoint Foci of Late Staining and Leakage Throughout the Macula and Throughout the Posterior Pole as Evidenced by Sweep Views. There Was Staining at the Margin of the Disc Rim More Prominent Temporally in the Late Views.

Figure 3. Fundus Photograph and the Early and Late Phases of Fluorescein Angiography of the Right Eye at the Time of Diagnosis of Posterior Scleritis. Fundus Photograph Illustrated Pronounced Chorioretinal Folds with Choroidal Detachment in This Acute Exacerbation (A). On Fluorescein Angiography (B and C), There Was Early Hyperfluorescence with Multiple Pinpoint Foci of Late Staining and Leakage Throughout the Macula and Throughout the Posterior Pole as Evidenced by Sweep Views. There Was Staining at the Margin of the Disc Rim More Prominent Temporally in the Late Views.

Echography of the Right Eye at Time of Diagnosis of Posterior Scleritis. Transverse and Longitudinal B-Scans and Standardized A-Scan Showed that the Thickening of the Ocular Coats Remained Nasally (arrows) but the Reflectivity Was Higher than at the Initial Visit (down Arrow) (left Column). Transverse and Longitudinal B-Scans Showed What Appeared to Be Choroidal Detachments (side and up Arrow) (right Column). The Echo Dense Signals in the Suprachoroidal Space Signified that This Material Was Non-Serous in Nature. The Standardized A-Scan Showed Low Amplitude Signals (down Arrow) Produced by the Suprachoroidal Substance, Which could be Consistent with Hemorrhage.

Figure 4. Echography of the Right Eye at Time of Diagnosis of Posterior Scleritis. Transverse and Longitudinal B-Scans and Standardized A-Scan Showed that the Thickening of the Ocular Coats Remained Nasally (arrows) but the Reflectivity Was Higher than at the Initial Visit (down Arrow) (left Column). Transverse and Longitudinal B-Scans Showed What Appeared to Be Choroidal Detachments (side and up Arrow) (right Column). The Echo Dense Signals in the Suprachoroidal Space Signified that This Material Was Non-Serous in Nature. The Standardized A-Scan Showed Low Amplitude Signals (down Arrow) Produced by the Suprachoroidal Substance, Which could be Consistent with Hemorrhage.

Discussion

Posterior scleritis is not uncommon in clinical practice. However, our index case of nodular sclerochoroidopathy associated with posterior scleritis is among the few cases that have been reported. Given the initial clinical findings complemented by findings on ultrasonography in a patient with a history of skin lesion that had atypical cellular appearance, we were concerned with a malignancy process. B-scan ultrasonography revealed the fundus lesions with low-medium reflection in contrast to the high internal reflectivity typically noted in posterior scleritis.

Intraocular involvement appears to be the initial manifestation of nodular sclerochoroidopathy in this patient. Foci of nodular scleral inflammation led to multifocal choroidal elevations that resembled choroidal neoplasms.3 Another unusual feature was the absence of anterior scleritis during the course of the disease, which is often present in typical cases of posterior scleritis. The patient also did not experience pain at the initial onset, but later suffered from significant discomfort with full extent of the posterior scleritis along with serous retinal detachment. Often, individuals with posterior scleritis experience ocular pain proportional to the severity of the anterior scleritis, which was not present in this patient.3,4

On exacerbation of the disease, the symptoms and signs were more typical of posterior scleritis. The most common fluorescein angiographic findings of mottled background fluorescence in the early phase, numerous pinpoint spots of leakage in the middle phase, and late intense staining of the subretinal fluid were present in this patient.3 At this stage, ultrasonography was a useful ancillary test in the diagnosis. Combined A-scan and B-scan features can be diagnostic of posterior scleritis and help to rule out primary and metastatic choroidal neoplasms.5 Proper diagnosis is important because it can aid in proper management, which is often through a medical approach for the serous detachment. The employment of low-dose systemic corticosteroids, in conjunction with immunomodulatory therapy, has allowed the disease to be in remission and visual acuity to improve and be maintained.

Unusual atypical features, such as nodular sclerochoroidopathy, can accompany common conditions such as posterior scleritis. An appropriate, step-ladder approach should be employed in the evaluation and management to achieve the best possible outcome.

References

  1. Pérez-Campagne E, Guex-Crosier Y, Schalenbourg A, Uffer S, Zografos L. Giant nodular posterior scleritis compatible with ocular sarcoidosis simulating choroidal melanoma [aticle in Spanish]. Arch Soc Esp Oftalmol. 2007;82:563–566. doi:10.4321/S0365-66912007000900010 [CrossRef]
  2. Arevalo JF, Shields CL, Shields JA. Giant nodular posterior scleritis simulating choroidal melanoma and birdshot retinochoroidopathy. Ophthalmic Surg Lasers Imaging. 2003;34:403–405.
  3. Benson WE. Posterior scleritis. Surv Ophthalmol. 1988;32:297–316. doi:10.1016/0039-6257(88)90093-8 [CrossRef]
  4. Benson WE, Shields JA, Tasman W, Crandall AS. Posterior scleritis: a cause of diagnostic confusion. Arch Ophthalmol. 1979;97:1482–1486.
  5. Brod RD, Saul RF. Nodular posterior scleritis. Arch Ophthalmol. 1990;108:1170–1171.
Authors

From the Wilmer Eye Institute (EH, JW, MI, PT, AK, RC, CD, DVD, QDN), Johns Hopkins University School of Medicine, Baltimore, Maryland; the Department of Ophthalmology (JW), The Second Hospital of Hebei Medical University Shijiazhuang, Hebei Province, China; the Department of Ophthalmology (PT), Inonu University School of Medicine, Malatya, Turkey; and the Retina Care Center (EPS), Baltimore, Maryland.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Quan D. Nguyen, MD, MSc, Johns Hopkins Hospital, 600 North Wolfe Street, Maumenee 745, Baltimore, MD 21287. E-mail: qnguyen4@jhmi.edu

10.3928/15428877-20100830-06

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