densedepositscontaininginterspersed TE. A geometric model to predict the change proliferative sickle cell retinopathy is in corneal curvature from the intrastromal cor- collagen fibrils, extracellular empty neal ring (ICR). Invest Ophthalmol Vis Sci. 1994; characterized clinically by a con- spaces (likely clefts formed from lipid 35(suppl):2023. cave tractional retinal elevation, reti- removal during processing), and per- 7. Colin J, Cochener B, Savary G, Malet F. Cor- recting keratoconus with intracorneal rings. nal nonperfusion, inner-layer breaks, sistentlyactivatedkeratocytesaresimi- J Cataract Refract Surg. 2000;26:1117-1122. absorption of laser by the outer layer, lar to these animal findings. The ex- 8. Kymionis GD, Siganos CS, Kounis G, Asty- and a split pattern on optical coher- rakakis N, Kalyvianaki MI, Pallikaris IG. tracellular lipid collections, which Management of post-LASIK corneal ectasia with ence tomography. Two cases of reti- likely correspond to the crystalline de- Intacs inserts: 1-year results. Arch Ophthalmol. nal schisis are described herein, both posits seen clinically, probably arise 2003;121:322-326. 3 9. Twa MD, Ruckhofer J, Kash RL, Costello M, featuring the conjunctival sickle sign from chronic mechanical irritation to Schanzlin DJ. Histologic evaluation of corneal and both eventually complicated by keratocytes that continually strive to stroma in rabbits after intrastromal corneal ring outer-layer breaks and retinal detach- heal the stromal wound. implantation. Cornea. 2003;22:146-152. 10. Rodrigues MM, McCarey BE, Waring GO III, ment that possibly might have been The breaks in the Bowman layer Hidayat AA, Kruth HS. Lipid deposits posterior prevented by timely laser treatment. could correspond to predisposition toimpermeableintracorneallensesinrhesusmon- keys: clinical, histochemical, and ultrastructural to keratoconus and ectasia. Al- studies. Refract Corneal Surg. 1990;6:32-37. Report of Cases. Case 1. A 44-year- though the reinforced stromal bed old African American woman com- was not strong enough to prevent plained that in recent months the vi- further ectasia from developing in sual acuity in her left eye had been this case, longitudinal studies have Schisis in Sickle Cell decreasing as a result of what she de- not yet addressed the overall long- Retinopathy scribed as “a moving veil.” She had term success or stability of ICR seg- been diagnosed with sickle cell dis- ments for corneal ectasia beyond 1 Retinal schisis is a rare but poten- ease, type SS, 17 years earlier. She had year postoperatively. These studies tially serious complication of sickle a history of multiple pulmonary em- are necessary to address whether the cell retinopathy.1 It is related to boli complicated by pulmonary hy- natural history of the ectasia changes chronic low-grade ischemia of the in- pertension, which had necessitated after ICR segment implantation. ner nuclear layer, which houses the placement of an aortic umbrella 2 Marc J. Spirn, MD Muellerian glia, the structural back- years earlier. She took warfarin so- Daniel G. Dawson, MD bone of the retina.2 Schisis as part of dium and folic acid. Her visual acu- Roy S. Rubinfeld, MD Christine Burris, OD A B Jonathan Talamo, MD Henry F. Edelhauser, PhD Hans E. Grossniklaus, MD Correspondence: Dr Grossniklaus, BT428 Emory Eye Center, 1365 Clifton Rd NE, Atlanta, GA 30322 ([email protected]). C D Financial Disclosure: None. Funding/Support: This study was supported in part by grants RO1- EY-00933 and P30-EY-006360 from the National Eye Institute, Bethesda, Md, and an unrestricted grant from Research to Prevent Blindness, Inc, New York, NY. 1. Seiler T, Koufala K, Richter G. Iatrogenic kera- tectasia after laser in situ keratomileusis. J Re- E F fract Surg. 1998;14:312-317. 2. Randleman JB, Russell B, Ward MA, Thomp- son KP, Stulting RD. Risk factors and progno- sis for corneal ectasia after LASIK. Ophthalmology. 2003;110:267-275. 3. Lovisolo CF, Fleming JF. Intracorneal ring seg- ments for iatrogenic keratectasia after laser in situ keratomileusis or photorefractive keratectomy. J Refract Surg. 2002;18:535-541. 4. Rapuano CJ, Sugar A, Koch DD, et al. Intrastro- mal corneal ring segments for low myopia: a re- portbytheAmericanAcademyofOphthalmology. Ophthalmology. 2001;108:1922-1928. 5. Fleming JF, Reynold AE, Kilmer L, Burris TE, Figure 1. Funduscopy, angiography, and optical coherence tomography results in patient 1. A, Drawing Abbott RA, Schanzlin DJ. The intrastromal cor- of the fundus showing the extent of schisis (hatched lines). B, Ocular coherence tomogram of the left neal ring: 2 cases in rabbits. J Refract Surg. 1987; macula. The Muellerian pillars indicate schisis. Photographs of the fundus of the left eye showing 3:227-232. neovascularization of the disc (C) and temporal retinal elevation (D). Fluorescein angiogram of the 6. Silvestrini TA, Mathis ML, Loomas BE, Burris arteriovenous phase focused on the fovea (E) and vessels of retinal elevation (F). (REPRINTED) ARCH OPHTHALMOL / VOL 123, NOV 2005 WWW.ARCHOPHTHALMOL.COM 1607 ©2005 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 A B C D Figure 2. Funduscopy, ophthalmoscopy, and angiography results in patient 2. A, Drawing of the fundus of the right eye demonstrating the extent of schisis (hatched lines) and the location of inner-layer holes (dots) seen through the vitreous hemorrhage. Fluorescein angiogram of the anteriovenous phase focused on the detached macula (B) and elevated nonperfused inferotemporal periphery (C). D, Photograph of the fundus showing an inferior outer-layer break. Note the rolled edges of the break and the glial nodules (arrows) of the inner layer. ity with glasses was 20/30 OD and Visual field testing showed dense eye operated on was 20/80 follow- 20/120 OS; near vision was 20/65 OD scotomas corresponding to the areas ing cataract extraction and repeat and 20/200 OS. Slitlamp examina- of nonperfusion and schisis. Angi- capsulotomy. tion showed the sickle comma sign ography confirmed peripheral reti- Case 2. A 56-year-old African in conjunctival arterioles.3 Intraocu- nal nonperfusion beyond the near pe- American man with sickle cell trait lar pressure was normal. riphery; all areas of schisis received had had repeat vitreous hemor- Funduscopy of the right eye dis- little or no perfusion (Figure 1E rhages in his right eye. Years ear- closed tractional schisis from the and F). Optical coherence tomogra- lier, a closed-funnel retinal detach- 9- to 7-o’clock meridian within the phy of the right macula showed schi- ment had caused blindness in his left inferotemporal arcades. Small inner- sis in the inner layer and, in the left eye. Visual acuity was 20/40 OD; layer breaks were noted posterior to macula, schisis and Muellerian pil- near vision was J2 at 14 in. There was equatorial fibrovascular prolifera- lars toward the temporal periphery light perception in the left eye. The tion. The peripheral retina appeared (Figure 1B). previous winter, the patient had had nonperfused; the macula had a glial After photocoagulation of the pneumonia. He had no other medi- sheen. Funduscopy of the left eye nonperfused attached retina, laser cal problems and was taking no showed equatorial fibrovascular pro- burns were noted in the areas of reti- medications. liferations. The temporal retina, in- nal elevation, corroborating schi- Slitlamp examination of the bul- cluding the fovea, was elevated from sis. Four years of follow-up showed bar conjunctiva showed the comma 12- to 6-o’clock (Figure 1A). The in- the area of schisis to be stable. Vi- sign of sickling.3 Indirect ophthal- ferotemporal fovea was thrown into sion was 20/50 OD and 20/120 OS. moscopy of the right eye showed the striae related to a point of vitreous ad- A rhegmatogenous retinal detach- disc and macula through vitreous hesion just inside the arcades. An ment occurred in the left eye and was hemorrhage. Large fibrovascular ovoid macular vitreous condensa- repaired, improving acuity to 20/ proliferations were noted from 11- tion was present (Figure 1C and D). 50. After 5 years, visual acuity in the to 1-o’clock and from 5- to 8-o’clock. (REPRINTED) ARCH OPHTHALMOL / VOL 123, NOV 2005 WWW.ARCHOPHTHALMOL.COM 1608 ©2005 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/23/2021 Just anterior to them, the retina was tion on the inner layer. In all likeli- concave and elevated and had large hood, it was the chronicity of ische- Periocular Cutaneous inner-layer breaks (Figure 2A). mia that allowed schisis to occur. Pigmentary Changes The patient was told that he had Rapidly progressive proliferative Associated With retinal schisis and was at risk of reti- sickle retinopathy would have been Bimatoprost Use nal detachment but that his hemor- characterized by traction detach- ments because there would not have rhage was likely to clear. He was lost Topical bimatoprost (Lumigan; been enough weakening of the in- to follow-up for 5 months and then Allergan Inc, Irvine, Calif) is a traretinal cohesive forces to permit was seen with 5/200 OD vision. Oph- prostamide analog licensed in the schisis. The pathomechanism of thalmoscopy showed a mild vitre- United States (2001) and in the schisis in patients such as ours may ous hemorrhage and a retinal eleva- United Kingdom (2002) as a second- be similar to that of proliferative dia- tion that was shallow superiorly and line treatment modality for glau- betic retinopathy, in which schisis more prominent inferiorly and tem- coma and ocular hypertension. Un- is found after 20 years of disease ac- porally. Peripheral nonperfusion was like with latanoprost, there is little counting for half of all tractional el- demonstrated angiographically published evidence for any periocu- evations of the retina.6-8 (Figure 2B and C). The retina was lar skin pigmentary effects associ- One can only speculate about the elevated from the disc to the ora ser- ated with bimatoprost.