Table 2.
Clinical Features of 10 Patients With Multizonal Outer Retinopathy and Retinal Pigment Epitheliopathy (MORR)
Patient No./Sex/Age, y | Laterality | Follow-Up Duration, y | Initial Visual Acuity | Final Visual Acuity | Medical History | Systemic Work-Up and Genetic Testing | Treatment | Natural Course and Complications |
1/F/43 | Bilateral | 23 | 20/20 OD 20/20 OS |
20/30 OD 20/20 OS |
Graves' disease | Negative laboratory and genetic panel testing | Mycophenolate mofetil | Centripetal and centrifugal progression with foveal sparing OU |
2/F/71 | Bilateral | 17 | 20/25 OD 20/25 OS |
20/40 OD 20/60 OS |
Rheumatoid arthritis, hypertension, asthma | Negative laboratory and whole genome sequencing | None | Centripetal and centrifugal progression with foveal sparing OU |
3/M/69 | Bilateral with marked asymmetry | 10 | 20/40 OD 20/30 OS |
20/200 OD 20/50 OS |
Hypercholesterolemia | Negative laboratory and genetic panel testing | None | Centripetal and centrifugal progression with foveal involvement OD Discrete progression of peripapillary lesion with foveal sparing OS |
4/F/53 | Bilateral with marked asymmetry | 21 | 20/60 OD 20/20 OS |
HM OD HM OS |
Hashimoto thyroiditis, hypercholesterolemia | Negative laboratory and genetic panel testing | Acyclovir | Complete retinal degeneration OU |
5/F/64 | Bilateral | 10 | 20/30 OD 20/40 OS |
20/150 OD 20/200 OS |
Hashimoto's thyroiditis, Type 1 diabetes, hypertension, liver hemangiomas, ovarian cancer | Negative laboratory and whole genome sequencing | None | Centripetal and centrifugal progression with foveal involvement OU |
6/M/41 | Bilateral | 10 | 20/20 OD 20/20 OS |
20/100 OD 20/100 OS |
Ulcerative colitis, primary sclerosing cholangitis | Negative laboratory testing | Repeated IVI of dexamethasone implant 0.7 mg, then of fluocinolone acetonide intravitreal implant (0.19 mg) | Centripetal and centrifugal progression with foveal involvement OU. Temporary cessation of the peripapillary lesion progression and partial regression of the fringe-like hyperautofluorescent border after intravitreal corticosteroids Subretinal fibrosis OD |
7/F/53 | Bilateral | 10 | 20/40 OD 20/20 OS |
20/300 OD 20/40 OS |
Congenital pulmonary valve disease, hypercholesterolemia, Type 2 diabetes | Negative laboratory and genetic panel testing | Repeated IVI of dexamethasone implant 0.7 mg, then of fluocinolone acetonide intravitreal implant (0.18 mg) | Centripetal and centrifugal progression with foveal involvement OD Temporary cessation of the peripapillary lesion progression and partial regression after repeated IVI of dexamethasone 0.7 mg then centripetal and centrifugal progression with foveal involvement OS |
8/F/38 | Bilateral | 13 | 20/20 OD 20/200 OS |
20/40 OD 20/400 OS |
Unremarkable | Negative laboratory and genetic panel testing | Corticosteroid, mycophenolate mofetil, methotrexate, adalimumab, infliximab | Centripetal and centrifugal progression with foveal sparing OD and foveal involvement OS |
9/F/40 | Bilateral | 8 | 20/20 OD 20/20 OS |
20/20 OD 20/20 OS |
Unremarkable | Negative laboratory testing | Corticosteroid, adalimumab | Centripetal and centrifugal progression with foveal sparing OU |
10/F/69 | Bilateral | 9 | 20/20 OD 20/40 OS |
20/200 OD 20/200 OS |
Unremarkable | Negative laboratory and genetic panel testing | Intravitreal injections of bevacizumab | Centripetal and centrifugal progression with foveal involvement OU Choroidal neovascularization OS and subretinal fibrosis OU |
IVI, intravitreal injection; OD, right eye: OS, left eye.