|Year : 2020 | Volume
| Issue : 4 | Page : 652
Bilateral macular hole in gyrate atrophy: A rare association
Deepika C Parameswarappa, Komal Agarwal
Smt Kanuri Santhamma Center for Vitreo-Retina Diseases, L V Prasad Eye Institute, Hyderabad, Telangana, India
|Date of Web Publication||16-Mar-2020|
Dr. Komal Agarwal
Smt. Kanuri Santhamma Centre for Vitreo-Retinal Diseases, L V Prasad Eye Institute, Banjara Hills, Hyderabad - 500 034, Telangana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Parameswarappa DC, Agarwal K. Bilateral macular hole in gyrate atrophy: A rare association. Indian J Ophthalmol 2020;68:652
|How to cite this URL:|
Parameswarappa DC, Agarwal K. Bilateral macular hole in gyrate atrophy: A rare association. Indian J Ophthalmol [serial online] 2020 [cited 2020 Apr 4];68:652. Available from: http://www.ijo.in/text.asp?2020/68/4/652/280697
An 18-year-old female presented with gradually progressive reduced vision in both eyes for 8 years. Her BCVA was 20/125p, N18 and 20/200, N18 in right and left eye respectively. The fundus examination showed scalloped areas of choroidal and retinal atrophy with a macular hole [Figure 1]a and [Figure 1]b. Optical coherence tomography (OCT) showed epiretinal membrane and lamellar hole in both. The left eye had different separation planes of outer and inner layers [Figure 1]c and [Figure 1]d. The plasma ornithine level was raised (554.68 mmol/ml). Gyrate atrophy with a lamellar macular hole was diagnosed and the patient underwent sequential internal limiting membrane peeling with inverted flap for hole closure. Ornithine restricted diet and pyridoxine supplement was advised. At 8 months follow-up, the BCVA was maintained and the OCT showed closed holes [Figure 1]e and [Figure 1]f. The macular hole in this case is due to probable de-roofing of cystoid spaces. They are a rare finding in gyrate atrophy.
|Figure 1: Widefield fundus photos (a: Right eye; b: Left eye) showing areas of choroidal and retinal atrophic areas with scalloped margins 360° with macular hole. Pre-operative OCT of the right eye showing a macular pseudo hole with disorganized outer retinal layers at the base (c) and OCT of left eye showing a full-thickness macular hole with different separation planes of outer and inner layers (d). One-month postoperative OCT of the right eye showing closed hole (e) and a closing macular hole with ILM flap in the left eye (f)|
Click here to view
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.