OSLI Retina

September 2020

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September 2020 · Vol. 51, No. 9 533 ■ I M A G E S I N O P H T H A L M O L O G Y ■ Unilateral Situs Inversus of the Optic Disc in a Patient With Retinal Arteriolar Tortuosity Chia-Chen Hsu, MD; Kathy Ming Feng, MD; Yun-Hsiang Chang, MD ABSTRACT: Familial retinal arteriolar tortuosity (fRAT) is a rare autosomal dominant disorder that is characterized by tortuosity of the second- and third-order arterioles around the posterior pole of retina. Most cases with fRAT lack associated sys- temic vascular abnormality. Situs inversus, a con- genital anomaly, features emergence of retinal ves- sels from the optic nerve head in a nasal direction, usually occurs bilaterally. In this case report, the authors describe a unique case of fRAT with unilat- eral situs inversus. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:533-535.] A 52-year-old male with hyperlipidemia present- ed for accidently noted retinal vascular anomaly. He received cataract surgery in his right eye (OD) 2 years ago and had experienced exercise-related vitreous hemorrhage with spontaneous recovery in his teens. He denied of diabetes mellitus or hypertension. Ab- normal tortuosity of the retinal arterioles was already noted at that time. Both his father and brother have tortuous retinal arterioles, as well. On examination, the bilateral best-corrected vi- sual acuity was 20/20. The anterior segments were unremarkable except for right pseudophakia. Fundus examination demonstrated spiral arterioles of the sec- ond and third order at the macular and peripapillary area of bilateral eyes without periphery involvement. Furthermore, situs inversus of the optic disc in his left eye (OS) was observed (Figures 1A and 1B). The left eye with situs inversus of the optic disc exhib- ited thicker retinal nerve fiber layer (RNFL) in the nasal side, larger rim area (OD/OS: 1.34/2.59 mm 2 ), and smaller cup-to-disc area ratio than the right eye as measured by optical coherence tomography (Fig- ure 2). After a thorough investigation from imaging to blood tests, no other vascular abnormalities or sys- temic diseases could be found. A diagnosis of familial retinal arteriolar tortuosity (fRAT) accompanied by situs inversus of optic disc was made based on his retinal image and hereditary condition. From the Department of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, R.O.C. Originally submitted April 10, 2020. Revision received April 10, 2020. Accepted for publication July 9, 2020. The authors report no relevant financial disclosures. Address correspondence to Yun-Hsiang Chang, MD, Department of Ophthalmology, Tri-Service General Hospital, National Defense Medical Center, No. 325, Section 2, Cheng-Kung Road, Neihu District, Taipei City, 11490, Taiwan, R.O.C.; email: yun.siang@me.com. doi: 10.3928/23258160-20200831-09

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