|Year : 2021 | Volume
| Issue : 4 | Page : 830-832
Swept-source optical coherence tomography in Oguchi disease
Devashish Dubey1, Mahesh Shanmugam2, Rajesh Ramanjulu2
1 Sankara Eye Hospital, Jaipur, Rajasthan, India
2 Sankara Eye Hospital, Bengaluru, Karnataka, India
|Date of Submission||07-Apr-2021|
|Date of Acceptance||20-May-2021|
|Date of Web Publication||09-Oct-2021|
Dr. Rajesh Ramanjulu
Sankara Eye Hospital, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
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
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)|
Click here to view
|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|
Click here to view
| Discussion|| |
Oguchi disease is characterized by difficulty in night vision and a golden-yellow sheen (Mizuo–Nakamura phenomenon) which disappears after dark adaptation. Autosomal recessive mutations in S-antigen (SAG) or GPK1 have been identified; both encode a protein that inactivates photoactivated rhodopsin, resulting in rod dysfunction.
Yamada et al. 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. reported pachychoroid neovasculopathy in a patient with retinitis pigmentosa. SAG mutation has been identified in retinitis pigmentosa (RP) as well. Cases having an overlap of RP with Oguchi have also been reported. 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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Mizuo G. A new discovery in dark adaptation in Oguchi disease. Acta Soc Ophthalmol Jpn 1913;17:1148-50.
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.
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.
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.
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.
[Figure 1], [Figure 2]