Abstract
Incontinentia Pigmenti is a complex disease with both systemic and ocular findings. It requires early ophthalmological evaluation given risk of progressive retinal ischemia, neovascularization, and complex tractional and/or rhegmatogenous retinal detachments. Due to its rarity, there are no large-scale clinical trials to establish preferred practice patterns. Nevertheless, management guidelines can be adapted from other proliferative retinopathies that present in infancy. In particular, early identification of retinal ischemia through dilated fundus exams with fluorescein angiography is required to guide treatment course. Laser photocoagulation, cryotherapy, anti-vascular endothelial growth factor medications, scleral buckling, and vitrectomy are all used to curb disease progression. Despite advancements in care, outcomes in Incontinentia Pigmenti can be highly variable.
| Original language | English (US) |
|---|---|
| Title of host publication | Pediatric Vitreoretinal Surgery |
| Publisher | Springer International Publishing |
| Pages | 385-398 |
| Number of pages | 14 |
| ISBN (Electronic) | 9783031145063 |
| ISBN (Print) | 9783031145056 |
| DOIs | |
| State | Published - Jan 1 2023 |
Bibliographical note
Publisher Copyright:© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2023.
Keywords
- Bloch-Sulzberger syndrome
- Incontinentia pigmenti
- Neovascularization
- Proliferative retinopathy
- Rhegmatogenous retinal detachment
- Tractional retinal detachment