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Case Reports
. 2024 Jan 28;8(2):186-191.
doi: 10.1177/24741264241227680. eCollection 2024 Mar-Apr.

Ophthalmologic Presentations of Incontinentia Pigmenti

Ravneet S Rai  1 , Albert S Li  1   2 , Philip J Ferrone  1   2
Affiliations
Case Reports

Ophthalmologic Presentations of Incontinentia Pigmenti

Ravneet S Rai et al. J Vitreoretin Dis. .

Abstract

Purpose: To characterize treatments and outcomes in incontinentia pigmenti. Methods: Cases of incontinentia pigmenti were consecutively identified from a retina practice. Inclusion criteria were patients with incontinentia pigmenti with at least 6 months of follow-up. All patients had a full ophthalmic examination, including imaging with widefield fundus photography and widefield fluorescein angiography. Eyes with areas of avascular retina were treated with laser photocoagulation (except for 1 eye with mild changes). Results: Thirty-six eyes of 18 patients with incontinentia pigmenti were included. The median age at presentation was 11 months. On presentation, 7 eyes had a visual acuity (VA) of 20/40 or better and 3 eyes had VA of 20/50 to 20/100. The remaining 26 eyes could fix and follow or had at least light perception (LP) VA given the patients' young age. Of the 36 eyes, 20 (56%) had retinal involvement. The mean follow-up for treated patients was 6.9 years. Seventy-four percent of treated eyes required 1 laser session only. No eye that received laser treatment subsequently developed a retinal detachment. Of the 26 eyes with initial fix-and-follow or LP VA, 12 had Snellen or Allen VA testing at follow-up. Nine of these eyes had a follow-up VA of 20/40 or better. Of 10 eyes with a Snellen or Allen VA recorded at the initial visit, 9 had a final VA that was the same or improved. Conclusions: Laser photocoagulation was effective in treating patients with retinal manifestations of incontinentia pigmenti. Except for 1 eye, VA remained stable at the final follow-up.

Keywords: incontinentia pigmenti; neovascularization; photocoagulation; retinal detachment.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Images of Patient 5, who had ischemia and neovascularization in the right eye. (A) Fundus photograph before treatment. (B) Fluorescein angiography before treatment shows area of avascular retina as well as neovascularization leakage.
Figure 2.
Figure 2.
Images of Patient 5. (A) Fundus photograph after treatment. (B) Fluorescein angiography after treatment shows laser scars in the area of avascular retina.
Figure 3.
Figure 3.
Visual outcomes at the final follow-up of patients with initial fix-and-follow (F&F) or light-perception (LP) visual acuity (VA) (n = 26).
See this image and copyright information in PMC

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