Indocyanine Green Angiography of Juvenile X-linked Retinoschisis

Indocyanine Green Angiography of Juvenile X-linked Retinoschisis

Late reopening and spontaneous closure of previously repaired macular holes are rare events. Only three cases have been reported, and only one case ha...

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Late reopening and spontaneous closure of previously repaired macular holes are rare events. Only three cases have been reported, and only one case had OCT confirmation.1,2,4 Possible mechanisms of reopening of macular holes after vitrectomy include any process that modifies the foveolar anatomy, such as progressive epiretinal membrane formation, cystoid macular edema after cataract surgery or inflammation, or intraretinal and preretinal cellular remodeling and resultant traction.1–3 It is unlikely that residual vitreous remnants play a role, because the vitreous has been surgically removed; however, the internal limiting membrane may be involved in the reopening.2 Possible mechanisms for spontaneous closure of a macular hole after previous vitrectomy include epiretinal membrane contracture and bridging glial cell proliferation.1,4 There are similarities between the OCT images in the two case descriptions and reports from spontaneous closure after traumatic macular holes and idiopathic holes.5 There is a bridging of neuroretinal tissue over an optically empty space (that most likely represents residual vitreous fluid) combined with reduction of the cystoid spaces in the rim of tissue surrounding the hole. The subfoveal fluid gradually reabsorbs with eventual reformation of the normal foveal contour. Although the incidence is rare, the two cases described here illustrate that a 6- to 8-week period of observation with serial OCT measurements may be warranted to allow spontaneous closure of reopened macular holes.

PURPOSE: In juvenile X-linked retinoschisis (XLRS), fluorescein angiography is usually unremarkable and contributes poorly to the diagnosis. However, indocyanine green (ICG) angiography features in eyes that are affected with XLRS were not yet described. DESIGN: Retrospective observational case series. METHODS: A complete ophthalmologic examination that included ICG angiography was performed on three unrelated male patients (six eyes) who were 15, 22, and 48 years old. RESULTS: A distinct hyperfluorescent stellate pattern in the macular area that was associated with radial lines of hypofluorescence that were centered on the foveola was observed on the early phase of ICG examination (six of six eyes). This feature disappeared on the late phase of ICG examination. CONCLUSION: On these six XLRS eyes, early phases of ICG examination revealed an unusual radial aspect on the macula. This finding suggests that ICG angiography may be useful for the diagnosis of XLRS. (Am J Ophthalmol 2005;140:558 –561. © 2005 by Elsevier Inc. All rights reserved.)

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most common causes of juvenile macular degeneration that leads to early-onset visual loss in male patients.1 Genetically, it is related to the XLRS1 gene that is located on chromosome Xp22 and that codes for the protein retinoschisin.2 The fundoscopy reveals bilateral foveal schisis characterized by folds that radiate in a subtle spoke-wheel pattern.1,3 Optical coherent tomography is helpful for diagnosis.4 The cross section of the foveola shows the cystic cavities progressively decreasing in size toward the periphery, located in the inner layers of the neurosensory retina, sparing the retinal pigment epithelium and the external segment of the photoreceptors. On the contrary, fluorescein angiography does not contribute to the diagnosis of XLRS.1,5 Fluorescein angiography is usually normal or only reveals a vaguely hyperfluorescent circular zone at the edges of the macular area. Faintly hyperfluorescent cysts can be seen sometimes, but without late hyperfluorescence, which helps in the differential diagnosis with cystoid macular edema. Here, we report indocyanine green (ICG) angiography features in 3 unrelated male patients who were affected with XLRS. Case 1 was a 15-year-old boy whose visual acuity was 20/100 right eye (OD) and 20/63 left eye (OS). Case 2 was a 22-year-old man whose visual acuity was 20/63 OD and 20/50 OS. Case 3 was a 48-year-old man whose visual acuity was 20/50 OD and 20/200 OS. Familial

REFERENCES

1. Duker JS, Wendel R, Patel AC, Puliafito CA. Late re-opening of macular holes after initially successful treatment with vitreous surgery. Ophthalmology 1994;101:1373–1378. 2. Paques M, Massin P, Blain P, Duquesnoy AS, Gaudric A. Long-term incidence of reopening of macular holes. Ophthalmology 2000;107:760 –766. 3. Christmas NJ, Smiddy WE, Flynn HW Jr. Reopening of macular holes after initially successful repair. Ophthalmology 1998;105:1835–1838. 4. Shaikh S, Garretson B. Spontaneous closure of a recurrent macular hole following vitrecomy corroborated by optical coherence tomography. Ophthalmic Surg Lasers Imaging 2003;34:172–174. 5. Menchini U, Virgili G, Giacomelli G, Cappelli S, Giansanti F. Mechanism of spontaneous closure of traumatic macular hole: OCT study of one case. Retina 2003;23:104 –106.

Indocyanine Green Angiography of Juvenile X-linked Retinoschisis

Accepted for publication Mar 19, 2005. From the Clinique Ophtalmologique Universitaire de Créteil, Créteil, France (E.H.S., A.G., G.Q., G.C., G.S.). Inquiries to Eric H. Souied, Clinique Ophtalmologique Universitaire de Créteil, 40 Avenue de Verdun, 94010 Créteil, France; fax: 33 1 45 17 52 27; e-mail: [email protected]

Eric H. Souied, MD, PhD, Anna Goritsa, MD, Giuseppe Querques, MD, Gabriel Coscas, MD, and Gisele Soubrane, MD, PhD 558

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FIGURE 1. Imaging of juvenile X-linked retinoschisis (XLRS). Case 1; right eye. On the color picture, we distinguish a subtle spoke-wheel pattern (top left). Red free frame and localization of the optical coherent tomography (OCT) scans are shown on the central top panel. On the 5-mm horizontal linear scan (top right, upper panel), we observe cystic-like spaces within the neurosensory retina that progressively decrease in size toward the periphery. On the 1.8-mm concentric ring (top right, middle panel), the cystic cavities are demarcated by several anteroposterior linear columns that cross these hyporeflective spaces. On the 3.6-mm concentric ring (top right, upper panel), the cystic-like lesions are much smaller and are still demarcated by thin walls. On the arterial phase of indocyanine green (ICG) angiography (0=18=), we discern a hyperfluorescent lesion in the macular area that is associated with hypofluorescent radial lines (middle left). At approximately minute 5, we clearly see on this composite panel the hyperfluorescent pattern, which simulates a starfish appearance (bottom right). The lesion is localized only in the macular area, without peripheral defect. Comparison with OCT scans seems to indicate that the hyperfluorescence pattern corresponds with the cavities of the cystic cavities, although the hypofluorescent lines could correspond to the thin walls of the cystic cavities. On the late phase of ICG examination (minute 30), the macula area is hypofluorescent, and the stellate aspect is almost not visible (bottom left).

history of XLRS was noted in the three individuals. No peripheral schisis was present at the time of examination. ICG angiography was performed after informed VOL. 140, NO. 3

consent was obtained from the three patients, in agreement with our local ethical committee and French legislation, with the Heidelberg Retina Angiograph

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FIGURE 2. ICG features of XLRS. Case 2; left eye. On the infrared frame (top left), the subtle spoke-wheel pattern of XLRS is subtly visible. At 40 seconds of ICG examination, we distinguish a macular hyperfluorescence that harbors a starfish appearance (top right). The hyperfluorescence contrasts with radial lines of hypofluorescence that is centered on the foveola. This aspect remains visible at minute 5 of the ICG examination (bottom left). At minute 25, ICG angiography appears normal in this patient, which suggests a wash out of the dye inside the cystic cavities (bottom right).

Cases 1 and 3; Figure 1). It is notable that no other hyper- or hypofluorescent lesion was observed in any other part of the retina. In conclusion, in these three patients, early phases of ICG examination revealed an unusual radiate pattern that was centered on the foveola. The hyperfluorescence in ICG angiography might suggest an affinity of the dye to the inside of the cysts. Further analysis, with a larger number of patients and with other ICG angiographs, could confirm these preliminary results and establish the relevance of ICG examination for the diagnosis of XLRS.

(Heidelberg Engineering, Heidelberg, Germany). On the arterial and venous phase of ICG examination (0=15= to 0=45=), we observed a hyperfluorescent pattern in the macular area that harbored a starfish appearance and that contrasted with radial lines of hypofluorescence that was centered on the foveola (Figures 1 and 2). The clearest perception of this pattern lasted from the arterial phase to approximately minute 5 after the injection, which corresponds to the early phases of the ICG examination (Figures 1 and 2). It is notable that this peculiar aspect on the early phase of ICG examination was found in six of the six eyes. Then it gradually became less defined until the late phase of the ICG examination (20 to 30 minutes). On the late-phase frames, we observed either a normal aspect (two eyes, Case 2; Figure 2) or a moderate and ill-defined hypofluorescence in the macular area, which contrasted with the background of fluorescence of the retina (four eyes, 560

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REFERENCES

1. Tantri A, Vrabec TR, Cu-Unjieng A, Frost A, Annesley WH Jr, Donoso LA. X-linked retinoschisis: a clinical and molecular genetic review. Surv Ophthalmol 2004;49:214 –230. OF

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2. Reid SN, Yamashita C, Farber DB. Retinoschisin, a photoreceptor-secreted protein, and its interaction with bipolar and muller cells. J Neurosci 2003;23:6030 – 6040. 3. George ND, Yates JR, Moore AT. Clinical features in affected males with X-linked retinoschisis. Arch Ophthalmol 1996; 114:274 –280. 4. Ozdemir H, Karacorlu S, Karacorlu M. Optical coherence tomography findings in familial foveal retinoschisis. Am J Ophthalmol 2004;137:179 –181. 5. Krausse U, Vainio-Mattila B, Erkisson A, Forsius H. Fluorescein angiography studies on X-chromosomal retinoschisis. Acta Ophthalmol 1970;48:794 – 807.

Polycystic Echinococcosis of the Orbit Ramesh Murthy, FRCS, Santosh G. Honavar, MD, FACS, Geeta K. Vemuganti, MD, Milind Naik, MD, and Sanghamitra Burman, MD, FRCS PURPOSE: To report a case of polycystic echinococcosis of the orbit caused by Echinococcus oligarthrus. DESIGN: Interventional case report METHODS: A 14-year-old girl presented with a massive proptosis of the right eye of 9 years duration and loss of vision. The eye was phthisic, and a cystic mass was palpable. Computed tomography scan showed closely packed multiple orbital cysts with destruction of the medial orbital wall. Surgical exploration was performed. RESULTS: The phthisic eye was enucleated. Dissection of the outer fibrous capsule yielded approximately 30 intact translucent white cysts of various sizes that were suggestive of polycystic echinococcosis. The diagnosis of echinococcosis was confirmed on histopathologic evaluation. The specific diagnosis of E oligarthrus was made on the basis of the presence of straight-backed hooks and central guards. CONCLUSION: Polycystic echinococcosis of the orbit caused by E oligarthrus is rare. It may be an entirely new entity or hitherto unrecognized form of orbital echinococcosis. (Am J Ophthalmol 2005;140:561–563. © 2005 by Elsevier Inc. All rights reserved.)

E

CHINOCOCCOSIS OR HYDATID DISEASE IS A ZOONOSIS

caused by the larval stage of the cestode, genus Echinococcus.1,2 Within the genus Echinococcus, four species are recognized: E granulosus that causes cystic echinococcosis, E multilocularis that causes alveolar echinococcosis, and

Accepted for publication Mar 19, 2005. From the Division of Ophthalmic Plastic Surgery, Orbit, and Ocular Oncology (R.M., S.G.H., M.N.), the Ophthalmic Pathology Service (G.K.V.), and the Ocular Immunology Service (S.B.), LV Prasad Eye Institute, Hyderabad, India. Inquiries to Santosh G. Honavar, MD, FACS, Division of Ophthalmic Plastic Surgery, Orbit, and Ocular Oncology, LV Prasad Eye Institute, LV Prasad Marg, Banjara Hills, Hyderabad 500 034, India; fax: ⫹91-4023548271; e-mail: [email protected]

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FIGURE 1. Polycystic echinococcosis in a 14-year-old girl: (Top) Axial computed tomography scan shows closely packed multiple cysts with bony effacement and phthisis bulbi with calcification. (Bottom) Multiple white translucent cysts that have been freshly extracted from the orbit are shown.

E oligarthrus and E vogeli that cause polycystic echinococcosis1,2 Orbital echinococcosis predominantly is caused by E granulosus, which typically manifests as a unilocular cyst.3,4 Polycystic echinococcosis is rare.1–5 We report polycystic orbital echinococcosis caused by E oligarthrus. A 14-year-old girl presented with painless progressive protrusion of the right eye of 9 years duration that was associated with loss of vision. Examination revealed a large nontender cystic mass with severe proptosis. The phthisic eye was displaced supranasally. Computed tomography scan showed closely packed multiple cysts with thinning of the lateral orbital wall (Figure 1, Top) and destruction of the medial wall. Computed tomography scans of the brain, ultrasound scans of the abdomen and x-ray of the chest were normal. A clinical diagnosis of cystic echinococcosis was made. A transconjunctival approach was coupled with BerkeReese lateral orbitotomy incision. The phthisic eye was enucleated. A fibrous layer that appeared to delimit the mass was dissected to reveal closely packed multiple cysts. Approximately 30 intact translucent white cysts of various

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