Double Optic Discs, Optic Disc Coloboma, And
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Figure 2. Optical coherence tomographic image of the right eye Figure 3. Microperimetry of the right eye with a scanning laser corresponding to the scan line indicated by the arrow. Some of the outer ophthalmoscope. (size, Goldmann I; brightness, −15 dB; background, 10 retinal layers other than the nerve fiber layer appear to be absent in the areas candelas/m2). The patient did not recognize the stimulus at the positions of the cleavages. indicated by the red P s. profile of the inner retinal cleavages never see small bundles of nerve fi- (Figure 2). The mean±SD thick- ber passing across the RNFL de- Double Optic Discs, Optic ness of the retina at the site of the fect. However, there should be many Disc Coloboma, and Pit: cleavages was 106.4±17.7 m and that cases in which we cannot make a Spectrum of Hybrid Disc of the adjacent unaffected retina was clear distinction between them just Anomalies in a Single Eye 254.2±25.8 m. The mean thickness by fundoscopic appearance. Opti- of the retina at the site of the cleav- cal coherence tomographic imag- ages was significantly thinner than ing should be very useful in such Doubling of the optic disc is rare and that of the adjacent unaffected retina cases in making a correct diagnosis. is seen as true doubling or pseudo- (PϽ.001, Mann-Whitney U test). The The optical coherence tomo- doubling. We describe a peculiar case with multiple anomalies involv- fluorescein angiograms showed no graphic images showed the cleav- ing a single optic disc: a coloboma abnormalities. The results of stan- ages clearly along with the possibil- involving the optic nerve along with dard automated perimetry (Hum- ity that these cleavages extended a pit and sensory detachment, and phrey Field Analyzer 30-2 program; deeper than the nerve fiber layer. Al- 2 pseudodoubling of the optic disc Carl Zeiss Meditec, Dublin, Calif) though Chihara and Chilhara re- ported that the retinal sensitivity in with a disclike lesion within the were normal, but microperimetry coloboma. with a scanning laser ophthalmo- the area of the cleavage was not de- pressed, we detected a relative sco- scope (Scotometry program, ver- Report of a Case. A 34-year-old man sion 3.0; Rodenstock Instruments, toma in that area in our case. These findings indicated there may be some was referred to our institute as a case Ottobrunn, Germany) revealed a rela- of a “peculiar optic disc.” Examina- tive scotoma in the area of the reti- abnormalities in nerve conduction 1 in the area of the cleavage. tion revealed a right eye that was nal cleavages (Figure 3). No reti- within normal limits and a left eye that nal cleavage was found in her parents Keiichi Komeima, MD, PhD had a coloboma involving the optic or her elder brother. Masato Kikuchi, MD disc. A small, craterlike depression Yasuki Ito, MD, PhD was seen in the center of the colo- 2 Comments. Chihara and Chilhara Hiroko Terasaki, MD, PhD boma. A thin blood vessel along with reported a cleavage of the retinal Yozo Miyake, MD, PhD a tuft of whitish glial tissue extended nerve fiber layer in eyes with high down from the optic disc and en- myopia, but there has been only 1 Correspondence: Dr Komeima, De tered the crater on its superior as- report of retinal cleavages around partment of Ophthalmology, Nagoya pect. Several thin vessels emerged blood vessels in highly myopic eyes. University Graduate School of Medi- from the lesion, thereby simulating They used the term “cleavage” to dis- cine, 65 Tsuruma-cho, Showa-ku, another optic disc (Figure A and B). tinguish the cleavages from the “de- Nagoya, 466-8550, Japan (kkome Since we found it difficult to photo- fect” seen in glaucomatous eyes. @med.nagoya-u.ac.jp). graph the entire optic disc and colo- It is clinically important to dif- Financial Disclosure: None. boma without loss of detail of the ferentiate the cleavage from the reti- simulated optic disc with a standard nal nerve fiber layer (RNFL) de- 1. Timberlake GT, Mainster MA, Webb RH, Hughes fundus camera (VISUPAC FF 450Plus fect. The cleavage is often spindle GW, Trempe CL. Retinal localization of scoto- mata by scanning laser ophthalmoscopy. Invest IR; Carl Zeiss Meditec, Jena, Ger- shaped and we can sometimes see Ophthalmol Vis Sci. 1982;22:91-97. many) (Figure, A), another fundus small bundles of nerve fiber pass- 2. Chihara E, Chihara K. Apparent cleavage of the photograph using a digital slitlamp retinal nerve fiber layer in asymptomatic eyes ing across the cleavage, whereas the with high myopia. Graefes Arch Clin Exp camera (VISUPAC Digital Archiving RNFL defect is wedge shaped and we Ophthalmol. 1992;230:416-420. system; Carl Zeiss Meditec) and a (REPRINTED) ARCH OPHTHALMOL / VOL 123, OCT 2005 WWW.ARCHOPHTHALMOL.COM 1450 ©2005 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 A C B D Figure. A, Color fundus photograph of patient’s left eye taken with standard fundus camera, showing optic disc (arrowhead) with coloboma in its inferior aspect. Central disclike lesion (arrow) with vessel clothed in glial tissue entering it in its superior aspect from optic disc and thin vessels emerging from lateral and inferior aspects are seen, as is sensory detachment confined to temporal part of coloboma (asterisk). Inset, optic pit (arrow) in inferotemporal part of coloboma. B, Color fundus photograph of patient’s left eye taken with digital slitlamp camera and Mainster ultrawide field lens in place, showing optic disc and coloboma in their entirety. Arrow denotes central disclike lesion. Vessels in relation to lesion are not as clear as in earlier fundus photograph. Asterisk denotes sensory detachment confined to temporal part of coloboma, which is also less clear than in earlier fundus photograph. Inset, optic pit (arrow) in inferotemporal aspect of coloboma, which is clearer than in earlier fundus photograph. C, Optical coherence tomographic picture of patient’s left eye (oblique line scan through disclike lesion and temporal aspect of coloboma), showing coloboma and craterlike depression (arrow) corresponding to disclike lesion. Glial tissue surrounding vessel entering lesion superiorly (arrowhead) and sensory detachment in temporal aspect of coloboma (asterisk) are obvious. D, Optical coherence tomographic picture of patient’s left eye (horizontal line scan through temporal aspect of coloboma and adjacent temporal peripapillary retina), showing sensory detachment in temporal aspect of coloboma (asterisk). Arrow denotes confinement of sensory detachment to temporal margin of coloboma, with sparing of adjacent peripapillary retina. Mainster ultrawide field lens (West- evidence of twin optic nerves.1 Our injury to the proximal embryonic fis- ern Ophthalmics, Lynnwood, Wash) patient had only a single optic nerve sure. The origin of the pit is debated, placed over the patient’s cornea was as imaged by B-scan ultrasonogra- with some speculating it to be owing taken with satisfactory results (Figure, phy, though magnetic resonance im- to an improper closure of the supe- B). The inferotemporal portion of the aging would have been more infor- rior end of the embryonic fissure.4 coloboma lodged an optic pit. Sen- mative. Pseudodoubling of the optic Our patient had a spectrum of hy- sory detachment was confined to the disc is also rare and is commonly brid disc anomalies, suggesting an in- temporal part of the coloboma. Peri- caused by optic disc colobomas or stance of early embryonic injury in- papillary pigmentary changes sur- peripapillary colobomas.1-3 Our pa- volving both the proximal embryonic rounded the temporal aspect of the tient had the interesting finding of an fissure and the distal optic stalk. To disc and the coloboma. The macula optic disc–like lesion within a colo- our knowledge, there is no other such was normal. Optical coherence to- boma. Additional abnormalities such case described in the literature. mography disclosed a craterlike de- as an optic pit, serous detachment pression corresponding to the simu- confined to the coloboma, peripapil- Vasumathy Vedantham, MS, DNB, lated optic disc, which suggested a lary altered pigmentation, and a glial FRCS doubling of the papilla (Figure, C and tuft were observed. The latter 2 ab- D). A B-scan ultrasonogram showed normalities are usually seen in asso- Correspondence: Dr Vedantham, a single optic nerve head shadow. ciation with the morning glory syn- Retina-Vitreous Service, Aravind Eye drome that occurs owing to Hospital & Postgraduate Institute of Comment. A diagnosis of true dou- embryologic insults to the distal op- Ophthalmology, 1, Anna Nagar, bling is made with direct or indirect tic stalk.4 The coloboma involves an Madurai 625-020, Tamilnadu, In- (REPRINTED) ARCH OPHTHALMOL / VOL 123, OCT 2005 WWW.ARCHOPHTHALMOL.COM 1451 ©2005 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/30/2021 dia ([email protected]). Financial Disclosure: None. 1. Kamath GG, Prasad S, Patwala YJ, Watts MT. Peripapillary coloboma simulating double op- tic disc. Br J Ophthalmol. 1999;83:1207. 2. Barboni P, Deluigi M, Bonis CD, Monetti G, Savini G, Zanini M. Pseudodoubling of the optic disc. Arch Ophthalmol. 1998;116:1400-1401. 3. Donoso LA, Magargal LE, Eiferman RA, Meyer D. Ocular anomalies simulating double optic discs. Can J Ophthalmol. 1981;16:85-87. 4. Brodsky MC. Congenital optic disc anomalies. Surv Ophthalmol. 1994;39:89-112. Orbital Emphysema Leading to Blindness Following Routine Functional Endoscopic Sinus Surgery Figure. Computed tomographic scan. Black arrows show bony defect in the ethmoidal wall of the left orbit post–functional endoscopic sinus surgery. The white arrow indicates air in the orbit. Functional endoscopic sinus surgery (FESS) is a procedure commonly per- patent central retinal artery and no However, if the vision deteriorates, formed for sinus pathologic exami- disc swelling.