■ CASE REPORT ■

Spectral Domain Optical ease. Various ocular changes, including angioid streaks, peau d’orange, optic nerve drusen, salmon patches, and Coherence Tomography and choroidal neovascularization, have been reported. His- Fundus Autofl uorescence tology of the angioid streaks has shown mineralization and fragmentation of elastic fi bers. Arvas et al. described Findings in Pseudoxanthoma fi ndings on time domain optical coherence tomography Elasticum (TD-OCT), including hyperrefl ective areas correspond- ing to the optic nerve drusen and hyperrefl ective areas Kaori Sayanagi, MD with masking effects that seemed to represent calcium Sumit Sharma, BS deposits in the angioid streaks.2 Peter K. Kaiser, MD Spectral domain OCT (SD-OCT) offers dramati- cally faster acquisition times and broader band light ABSTRACT sources than TD-OCT. Thus, SD-OCT improves axial A 39-year-old man with image resolution and provides more detailed views of who had a loss of vision was examined using digital im- the intraretinal microstructures. We report the SD- aging. Spectral domain optical coherence tomography OCT and fundus autofl uorescence fi ndings in a pa- (SD-OCT) detected subfoveal deposits that coincided tient with subretinal deposits and pseudoxanthoma with hyperautofl uorescent areas on fundus autofl uo- elasticum. rescence imaging. These deposits seemed to cause the patient’s rapid visual loss because no angioid streaks or CASE REPORT choroidal neovascularization were present in the fovea. A 39-year-old man with pseudoxanthoma elasti- These deposits were not observed on fundus and infrared cum was referred with visual loss in his right . On photography. SD-OCT combined with fundus autofl u- examination, his best-corrected visual acuity (BCVA) orescence imaging may be a useful tool for diagnosis of was 20/200. After Institutional Review Board approv- the atypical deposits in patients with pseudoxanthoma al, digital imaging was performed. Fundus and infrared elasticum, which could lead to visual loss. [Ophthalmic photography revealed the angioid streaks in the poste- Surg Lasers Imaging 2008;39:S108-S110.] rior pole with slight foveal mottling (Fig. 1, A and B). showed mild mottled foveal INTRODUCTION hyperfl uorescence without leakage in any phase (Fig. 1, Pseudoxanthoma elasticum is a hereditary disease C and D). On TD-OCT, 6-mm horizontal and vertical that causes systematic disorders mainly affecting the skin, linear cross-hair scans detected slightly raised, hyperre- , and cardiovascular system.1 Recently, a mutation fl ective lesions and one subretinal deposit at the fovea in the ABCC6 gene has been associated with the dis- (Fig. 1, E and F). Two weeks later, the BCVA decreased to 20/400. SD- From the Cole Eye Institute, Cleveland Clinic Foundation, Cleveland, Ohio. OCT, combined with fundus autofl uorescence, infrared photography, and fl uorescein angiography, was performed Accepted for publication March 5, 2008. Mr. Sharma has received travel funding from Reichert, Inc., and Dr. Kaiser with the Heidelberg Spectralis HRA+OCT (Heidelberg serves on the Scientifi c Advisory Boards of Heidelberg Engineering and Carl Engineering; Heidelberg, Germany). Fluorescein angiog- Zeiss Meditec, Inc. Address correspondence to Peter K. Kaiser, MD, Cole Eye Institute, The Cleve- raphy was unchanged with linear hypofl uorescence pre- land Clinic Foundation, 9500 Euclid Avenue, Desk i3, Cleveland, OH 44195. sumably corresponding to angioid streaks in the late phase

S108 OPHTHALMIC SURGERY, LASERS & IMAGING · JULY/AUGUST 2008 · VOL 39, NO 4 (SUPPLEMENT) Figure 1. A and B, Fundus photograph and infrared images show a mild mot- tled foveal appearance (arrow) and angioid streaks (arrowheads). C, Fluo- rescein angiography shows mottled hyperfl uorescence in the early phase without leakage in the late phases (ar- row). D, Fluorescein angiography in the late phase shows hypofl uorescence corresponding to angioid streaks (ar- rowheads). E and F, TD-OCT image of the fovea. The horizontal scan (E) shows the slightly raised hyperrefl ec- tive areas, and the vertical scan (F) demonstrates one subfoveal deposit. G, The angioid streak is clearly observed on the infrared image (arrowheads) with green lines (a-c) indicating the in- dividual scan lines depicted on the SD- OCT image. H, SD-OCT shows normal retinal structure.

Figure 2. A At the follow-up exami- nation 2 weeks after the first visit, fundus autofluorescence shows sev- eral subfoveal hyperautofluorescent areas (arrows) and hypoautofluores- cence corresponding to the edges of the angioid streaks (arrowhead). The green lines (a-d) indicate the individual locations on SD-OCT. B, SD-OCT shows subfoveal deposits in the photoreceptor inner and outer segment layer and the retinal pig- ment epithelium (RPE) complex and a normal external limiting membrane. The individual deposits correspond to hyperautofluorescent areas on the fundus autofluorescence image. C, An infrared-red image shows the angioid streaks inferior to the fovea. D, SD- OCT shows the subretinal deposits. These deposits did not correspond to the angioid streaks. and no evidence of a choroidal neovascular membrane to the edges of the angioid streaks. SD-OCT showed several explain the vision loss (Fig. 1, G and H). Fundus autofl u- subretinal deposits beneath the external limiting mem- orescence showed distinct dots of hyperautofl uorescence brane in the fovea, which seemed to be the cause of the at the fovea and hypoautofl uorescence corresponding to visual deterioration (Fig. 2, A and B). Because the loca-

CASE REPORT S109 tions of landmarks on the fundus autofl uorescence image edges were hypoautofl uorescent. The fl uorescein angiog- and SD-OCT are precisely mapped with the Heidelberg raphy was normal in the areas of normal fundus autofl uo- HRA+OCT, the subretinal deposits did not correspond rescence and hypofl uorescent in the hypoautofl uorescent to any of the angioid streaks (Fig. 2, C and D). areas corresponding to the angioid streaks. The SD-OCT showed normal retinal structure over the angioid streaks. DISCUSSION Because Bruch’s membrane is only 1- to 4-µm thick, the To our knowledge, this is the fi rst report of sub- SD-OCT used in this case with an axial resolution of 7 µm retinal deposits unrelated to angioid streaks in a pa- may not be able to identify Bruch’s membrane or detect tient with pseudoxanthoma elasticum. These deposits changes from the angioid streaks. However, the SD-OCT appeared hyperautofl uorescent on fundus autofl uores- did show subfoveal deposits that corresponded to the hy- cence imaging and did not appear on fl uorescein angi- perautofl uorescent areas on fundus autofl uorescence that ography. These microstructural changes were not ob- were present in the fovea. served on TD-OCT images. SD-OCT showed that the The precise mechanism of this patient’s visual loss deposits represented excrescence of the photoreceptor remains unclear, and because no SD-OCT images were inner and outer segment layers and the retinal pigment obtained at baseline, no reason for the decline can be epithelium complex with a normal external limiting proven. However, it is likely that the deposits were the membrane, similar to senile drusen.3 However, the proximal cause of our patient’s visual loss because no composition of these deposits probably differs from angioid streaks or choroidal neovascularization were that of senile drusen because senile drusen appears as present in the fovea. Thus, SD-OCT combined with a central hypoautofl uorescent area surrounded by an fundus autofl uorescence imaging may be useful for annulus of hyperautofl uorescence.4 Drusen or drusen- diagnosis of angioid streaks and atypical deposits that like deposits are common in pseudoxanthoma elasti- could lead to visual loss in these patients. Further in- cum. Hyper-autofl uorescence in drusen or drusen-like vestigation is needed to determine the etiology and pa- deposits has been described.5 However, their thology of these deposits. has not been clarifi ed. Pseudoxanthoma elasticum is associated with a mu- REFERENCES tated ABCC6 gene and may lead to a metabolic disorder 1. Chassaing N, Martin L, Calvas P, Le Bert M, Hovna- of elastic fi bers and proliferation of various tissue compo- nian A. Pseudoxanthoma elasticum: a clinical, patho- nents, possibly related to the subfoveal deposits, including physiological and genetic update including 11 novel abnormal fi broblasts, abnormal extracellular matrix, and ABCC6 mutation. J Med Genet. 2005;42:881-892. abnormal elastic fi bers. Pathological studies have revealed 2. Arvas S, Akar S, Yolar M, Yetik H, Kizilkaya M, Ozkan retinal pigment epithelium changes in angioid streaks.6 S. Optical coherence tomography (OCT) and angiogra- Although it is possible that these deposits resulted from phy in patients with angioid streaks. Eur J Ophthalmol. RPE changes that are related to the angioid streaks, there 2002;12:473-481. was no relation between the angioid streaks and the sub- 3. Pieroni CG, Witkin AJ, Ko H, et al. Ultrahigh reso- retinal deposits in our case. lution optical coherence tomography in non-exudative Several reports have been published about fun- age related macular degeneration. Br J Ophthalmol. dus autofl uorescence image fi ndings in angioid streaks 2006;90:191-197. and pseudoxanthoma elasticum. They usually appear 4. Delori FC, Fleckner MR, Goger DG, Weiter JJ, Dorey hypoautofl uorescent and are believed to represent the CK. Autofl uorescence distribution associated with dru- linear breaks of Bruch’s membrane with fi brovascular sen in age-related macular degeneration. Invest Ophthal- ingrowth. However, Shiraki et al.5 reported that some mol Vis Sci. 2000;41:496-504. segments of angioid streaks can show varying degrees 5. Shiraki K, Kohno T, Moriwaki M, Yanagihara N. Fun- of fundus autofl uorescence, ranging from hypoautofl u- dus autofl uorescence in patients with pseudoxanthoma orescence to normal, depending on the degree of RPE elasticum. Int Ophthalmol. 2001;24:243-248. degeneration. 6. Dreyer R, Green WR. The pathology of angioid streaks: In our case, most of the angioid streak segments a study of twenty-one cases. Trans Pa Acad Ophthalmol showed normal fundus autofl uorescence and only the Otolaryngol. 1978;31:158-167.

S110 OPHTHALMIC SURGERY, LASERS & IMAGING · JULY/AUGUST 2008 · VOL 39, NO 4 (SUPPLEMENT)