From the Retina-Vitreous Service, Aravind Eye Hospital & Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Dhananjay Shukla, Aravind Eye Hospital & Postgraduate Institute of Ophthalmology, 1 Anna Nagar, Madurai 625 020, Tamil Nadu, India.
Macular edema is a well-known sequel of uveitis; when chronic (6 to 9 months), it may develop degenerative changes which lead to permanent visual impairment. Rarely, such changes can lead to a full-thickness macular hole.1–4 As the vision is already impaired severely, further treatment is either not considered or frequently futile when attempted.1–4 We report the formation and successful management of a macular hole secondary to chronic uveitis.
A 44-year-old healthy woman had been under treatment for bilateral, recurrent anterior uveitis with topical, periocular and systemic corticosteroids at the Uveitis Service of our hospital for 31 months. Her best-corrected Snellen visual acuity (BCVA) was 6/36 in the right eye and 6/18 in the left, with chronic cystoid macular edema (CME) in both eyes and a lamellar macular hole in the right eye, documented by optical coherence tomography (OCT 3, Carl Zeiss Meditec., Dublin, CA) (Figs. 1A to 1D). She was referred to the Retina-Vitreous Service when her vision dropped to 4/60 in the better (left) eye. BCVA of the right eye remained stable at 6/36. She had already been examined and investigated by the in-house physician, who found no evidence of any systemic infective or rheumatic inflammatory disease affecting skin, joints or organ systems. On slit lamp biomicroscopy, the anterior segments were quiet, with old keratic precipitates in both the eyes. Fundus examination showed status quo in the right eye; the view was hazy due to cataract in the left eye. OCT showed an unaltered lamellar macular defect in the right eye. OCT revealed vitreomacular traction with a full-thickness macular hole in the left eye, preceded by a perifoveal vitreous detachment, which was observed three months earlier (Figs. 2A and 2B). With informed consent of the patient and approval of the Institutional Review Board, vitrectomy, internal limiting membrane peeling and perfluoropropane gas (C3F8, 15%) tamponade with simultaneous cataract extraction and intraocular lens implantation was performed in the left eye. One month postoperatively, the macular hole was closed, and BCVA had improved to 6/36 (Figs. 2C and 2D). However, three months later, CME recurred in the left eye (central macular thickness: 340 μ) and BCVA dropped to 6/60 (Figs. 2E and 2F). After checking intra-ocular pressure (IOP: 16 mm Hg), an intravitreal injection of triamcinolone acetonide (4.0 mg/0.1 mL) was administered in the left eye, resulting in a rapid resolution of CME (182 μ). BCVA improved to 6/24 1 month after injection. During the post-injection follow-up, no steroid-induced rise of intra-ocular pressure was noted (IOP: 14 mm Hg). She was maintained on topical ketorolac and prednisolone eye drops with a slow taper, which kept the macular anatomy and function stable over the next 4 months.
Figure 1. Pre-Macular Hole Status in Bilateral Recurrent Uveitis. (A) Fundus View of the Patient’s Right Eye Shows Cystoid Macular Changes with a Central Excavation Suggestive of a Macular Hole. (B) Left Eye View is Marred by the Cataractous Changes, but Macular Cystic Changes are Faintly Discernible. Also Note the Pigment Dusting on the Optic Disc. (C) Optical Coherence Tomography (OCT) of the Right Eye (10 mm, Horizontal) Shows a Lamellar Macular Hole, the More Common Sequel of Chronic Macular Edema. The Complete Vitreomacular Separation Indicates that Status Quo is Likely to Prevail. (D) OCT of the Left Eye Demonstrates Macular Cystic Changes More Clearly than the Fundus Picture. There is No Vitreomacular Separation Yet.
Figure 2. Evolution and Management of a Post-Uveitis Macular Hole. (A) OCT (horizontal 5 mm Scan Through Fovea) of the Left Eye Demonstrates Parafoveal Vitreous Detachment, Without Significant Central Traction. The Previously Documented Macular Edema is Under Resolution, with only a Small Parafoveal Inner Retinal Cyst Remaining. (B) OCT (repeat Mode) Six Months Later is Obscured by the Secondary Cataract, but Aggravated Vitreofoveal Traction with a Full-Thickness Macular Hole in Classic “pregnant Drawbridge” Configuration is Evident. The Central Hyper-Reflectivity at the Level of Retinal Pigment Epithelium Confirms the Full-Thickness Defect. (C) A Month After Vitrectomy, Macular Hole Is Closed; Maculorhexis Margins are Visible. The Previously Noted Pigment Flecks on the Optic Disc Have Reduced in Density. (D) OCT of the Left Eye (10 mm, Horizontal) Shows Closure of the Macular Hole and Normalized Macular Contours. (E) Four Months Postoperatively, the Vision has Dropped in the Left Eye with Recurrence of Cystoid Macular Edema, Faintly Seen in the Red-Free Photograph; (F) Confirmed by OCT (central Macular Thickness: 340 μ). An Intravitreal Triamcinolone Injection Resolved the Macular Edema and Restored the Foveal Contours, Maintained for the Next 4 Months (not Shown).
Chronic intraocular inflammation may compromise central vision through sequels like pre-macular membranes and cystoid macular edema. Rarely, severe posterior uveitis has been reported to a result in a post-inflammatory macular hole (fungal endophthalmitis, cat-scratch disease, Behcet’s disease and immune-recovery uveitis in CMV retinitis), almost invariably precipitated by an inflammatory focus flush at the fovea.1–4 However, vitreomacular separation in a mild-moderate but chronic inflammatory disease can also dehisce a macula weakened by longstanding cystoid edema, as observed in our patient. Such macular holes are probably more important to recognize than in the aforementioned examples of severe uveitis,1–4 where severe background disease has already rendered the macular hole management irrelevant to visual prognosis. After surgery, our patient did not improve to the extent usual in an idiopathic macular hole, though she was definitely better-off than the patients in the aforementioned reports.1–4 We observed a postoperative recurrence of CME, which has been reported to re-open a surgically closed macular hole.5 Prompt anti-inflammatory treatment resulted in restoration and maintenance of the foveal contours, and prevented a potential surgical failure.
There is only one more report of successful outcome in an inflammatory macular hole, in the setting of Behcet’s disease.6 However, the authors could not rule out a coincidental idiopathic macular hole as there were no visits documenting the macular changes preceding the hole formation. Further, the macular hole had a partly flattened configuration, probably indicating that the hole was already closing spontaneously. A course of anti-inflammatory treatment alone might have helped in macular hole closure in their case, as previously reported by Halkiadakis et al.7 Vitreomacular traction in our patient mandated vitrectomy, though corticosteroids did help in the postoperative course.
While cystoid macular edema secondary to severe uveitis may per se dehisce into a full-thickness macular hole, associated vitreous membranes may add vitreomacular traction to this milieu, precipitating macular hole formation even in the presence of less severe uveitis. Though a uveitic macular hole can be successfully closed with vitrectomy and ILM peeling; one must beware of the risk of re-opening of the hole through recurrent inflammation and macular edema. A previous report7 and the present study underline the importance of anti-inflammatory treatment to avert or treat this complication. This study further highlights the benefit of following up an inflammatory CME by OCT to anticipate the untoward sequels, and reports successful management of a postinflammatory macular hole with a combination of surgery and pharmacotherapy.
- Kusaka S, Hayashi N, Ohji M, Ikuno Y, Gomi F, Tano Y. Macular hole secondary to fungal endophthalmitis. Arch Ophthalmol. 2003;121:732–733. doi:10.1001/archopht.121.5.732 [CrossRef]
- Alnini TA, Lakhanpal RR, Foroozan R, Holz ER. Macular hole in cat scratch disease. Am J Ophthalmol. 2005;140:149–151. doi:10.1016/j.ajo.2004.12.082 [CrossRef]
- Shew SJ, Yang CA. Macular hole in Behcet’s disease. Kaohsiung J Med Sci. 2004;20:558–562 doi:10.1016/S1607-551X(09)70258-X [CrossRef]
- Arevalo JF, Mendoza AJ, Ferretti Y. Immune recovery uveitis in AIDS patients with cytomegalovirus retinitis treated with highly active anti-retroviral therapy in Venezuela. Retina. 2003;23:495–502. doi:10.1097/00006982-200308000-00009 [CrossRef]
- Bhatnagar P, Kaiser PK, Smith SD, Meisler DM, Lewis H, Sears JE. Reopening of previously closed macular holes after cataract extraction. Am J Ophthalmol. 2007;144:252–259. doi:10.1016/j.ajo.2007.04.041 [CrossRef]
- Wu TT, Hong MC. Pars plana vitrectomy with internal limiting membrane removal for a macular hole associated with Behcet’s disease. Eye. (in press)
- Halkiadakis I, Pantelia E, Giannakopoulos N, Koutsandrea C, Markomichelakis NN. Macular hole closure after peribulbar steroid injection. Am J Ophthalmol. 2003;136:1165–1167. doi:10.1016/S0002-9394(03)00668-8 [CrossRef]