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 Table of Contents  
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 830-832

Swept-source optical coherence tomography in Oguchi disease

1 Sankara Eye Hospital, Jaipur, Rajasthan, India
2 Sankara Eye Hospital, Bengaluru, Karnataka, India

Date of Submission07-Apr-2021
Date of Acceptance20-May-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Rajesh Ramanjulu
Sankara Eye Hospital, Bengaluru, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_787_21

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Keywords: Mizuo–Nakamura phenomenon, Oguchi disease, pachychoroid, swept-source optical coherence tomography

How to cite this article:
Dubey D, Shanmugam M, Ramanjulu R. Swept-source optical coherence tomography in Oguchi disease. Indian J Ophthalmol Case Rep 2021;1:830-2

How to cite this URL:
Dubey D, Shanmugam M, Ramanjulu R. Swept-source optical coherence tomography in Oguchi disease. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 26];1:830-2. Available from: https://www.ijoreports.in/text.asp?2021/1/4/830/327707

A 30-year-old male patient presented with complaints of difficulty of vision at night since early childhood. He had a best-corrected visual acuity of 6/6, N6 in both eyes with normal intraocular pressure. On examination, anterior segment was unremarkable. On fundus evaluation, a golden-yellow sheen was visible throughout the entire retina, being more evident in the mid-peripheral areas. Swept-source optical coherence tomography (SS-OCT) (TOPCON-Japan Inc.) revealed indistinct outer retinal hyper-reflective bands along with the presence of pachychoroid. The patient was re-evaluated after 45 min of dark adaptation. The golden yellow sheen seen earlier had disappeared. SS-OCT showed improved delineation of the outer retinal hyper-reflective bands with a distinct IS-OS line [Figure 1] and [Figure 2]. Electroretinography was not done to avoid contact procedure during the ongoing pandemic.
Figure 1: Color and red-free fundus photograph showing the presence of golden-yellow sheen (a), which disappears after 45 min of dark adaptation (b). SS-OCT picture in dark adapted state showing distinct outer retinal bands along with the presence of pachychoroid (c)

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Figure 2: SS-OCT picture before (a) and after dark adaptation (b) showing distinct visibility of all four outer retinal hyper-reflective bands along with emergence of a distinct IS-OS band after dark adaptation

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  Discussion Top

Oguchi disease is characterized by difficulty in night vision and a golden-yellow sheen (Mizuo–Nakamura phenomenon) which disappears after dark adaptation.[1] Autosomal recessive mutations in S-antigen (SAG) or GPK1 have been identified; both encode a protein that inactivates photoactivated rhodopsin, resulting in rod dysfunction.[2]

Yamada et al.[3] reported the reappearance of the IS-OS line and better delineation of outer retinal bands after 4 hours of dark adaptation on Spectral domain (SD)-OCT in Oguchi disease. We observed a similar change in our case. In our case, we observed the dilatation of vessels in Haller's layer with compression of the overlying Sattler's layer. The subfoveal choroidal thickness was 387 μm.

Attanasio et al.[4] reported pachychoroid neovasculopathy in a patient with retinitis pigmentosa. SAG mutation has been identified in retinitis pigmentosa (RP) as well.[2] Cases having an overlap of RP with Oguchi have also been reported.[5] The cause for this choroidal hemodynamic change rarely seen in retinal dystrophies is still unknown. The presence of pachychorid in these cases may be incidental.

Our case highlights the SS-OCT features, which are evident after a short period of dark adaptation of 45 min itself, along with yet undescribed association with pachychoroid.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Mizuo G. A new discovery in dark adaptation in Oguchi disease. Acta Soc Ophthalmol Jpn 1913;17:1148-50.  Back to cited text no. 1
Nishiguchi KM, Ikeda Y, Fujita K, Kunikata H, Akiho M, Hashimoto K, et al. Phenotypic features of Oguchi disease and retinitis pigmentosa in patients with S-antigen mutations: A long-term follow-up study. Ophthalmology 2019;126:1557-66.  Back to cited text no. 2
Yamada K, Motomura Y, Matsumoto CS, Shinoda K, Nakatsuka K. Optical coherence tomographic evaluation of the outer retinal architecture in Oguchi disease. Jpn J Ophthalmol 2009;53:449-51.  Back to cited text no. 3
Attanasio M, Maggio E, Arena F, Pertile G. Swept-source optical coherence tomography angiography findings in a case of pachychoroid neovasculopathy in retinitis pigmentosa. Retin Cases Brief Rep 2020. doi: 10.1097/ICB.0000000000000962.  Back to cited text no. 4
Nakamachi Y, Nakamura M, Fujii S, Yamamoto M, Okubo K. Oguchi disease with sectoral retinitis pigmentosa harboring adenine deletion at position 1147 in the arrestin gene. Am J Ophthalmol 1998;125:249-51.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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