Case #15 – Bietti with Outer Retinal Tubulations

References Continued Y. Wada, T. Itabashi, H. Sato, M. Kawamura, A. Tada, and M. Tamai. 2005 Screening for Mutations in CP4V2 Gene in Japanese Patients With Bietti's Crystalline Corneoretinal Dystrophy. J Ophthalmol. 139: 894-899 K.Y.C Lee, A.H.C. Koh, T. Aung, V.H.K. Yong, K. Yeung, C.-L. Ang, and E.N. Vithana. 2005 Characterization of Bietti Cyrstalline Dystrophy

References S.A. Zweifel, M. Engelbert, K. Laud, R. Margolis, R. Spaide, and K.B. Freund. Outer Retinal Tubulation: a Novel Optical Coherence Tomography Finding. Archives of Ophthalmology. In press K. Drenser, D. Sarraf, A. Jain, and K. Small. 2006 Crystalline Retinopathies. Surv Ophthalmol. 51(6): 535-549 A. Ayata, S. Tatlipinar, M. Ünal, D. Ersanli, and A.H.

Special Acknowledgement K. Bailey Freund, MD Acknowledgements Sanjeev Nath Jeremy Whitney Alyssa Taussig Ketan Laud Larry Yannuzzi Krishna Morar Sherry Bass Andrea Yang

Jerome Sherman, OD, FAAO Jerome Sherman, O.D., is perhaps optometry's most prolific writer, publishing over 650 clinical articles, research manuscripts, book chapters and two CDs. He is senior author of three books that were published in 2007, and has delivered over three thousand lectures both nationally and internationally. He has served as a contributing

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Comments Bietti crystalline corneoretinal dystrophy (BCD) is an autosomal recessive retinal dystrophy first described by Bietti in 1937.3 Numerous tiny glistening yellow-white crystals in the posterior pole with RPE atrophy and hypertrophy along with choroidal sclerosis characterize the disorder.2 About half the cases have similar crystals in the superficial peripheral cornea.4 The disorder has pigmentary

Select Crystalline Retinopathies2 Crystals Tamoxifen Canthaxanthine Talc Bietti Cystinosis Oxalosis Calcified Drusen Exam Yellow central clustered intra-retinal crystals Golden intra-retinal crystals encircling the fovea Yellow or white opaque deposits within perifoveal vessel Refractile yelllowish crystals associated with RPE atrophy in posterior pole Diffuse mottling of RPE with glistening retractile deposits

Cirrus™ HD-OCT Anterior OS At least some of the hyperreflective dots in these corneal sections are likely due to crystals.

Cirrus™ HD-OCT En Face OSAs on the previous page, the color correspondence denotes the location of theORTs on the en face and the cross sectional images.

Cirrus™ HD-OCT En Face ODThe en face image on the left is color as to location in thecross sectional images on the right.