title: Structural Abnormalities of the Inner Macula in Incontinentia Pigmenti.
abstract: OBJECTIVE
  This report presents evidence from spectral-domain optical coherence tomography and fluorescein angiography of inner foveal structural abnormalities associated with vision loss in incontinentia pigmenti (IP).
  
  
  METHODS
  Two children had reduced visual behavior in association with abnormalities of the inner foveal layers on spectral-domain optical coherence tomography. Fluorescein angiography showed filling defects in retinal and choroidal circulations and irregularities of the foveal avascular zones. The foveal to parafoveal ratios were greater than 0.57 in 6 eyes of 3 patients who had extraretinal neovascularization and/or peripheral avascular retina on fluorescein angiography and were treated with laser. Of these, 3 eyes of 2 patients had irregularities in foveal avascular zones and poor vision.
  
  
  CONCLUSIONS
  Besides traction retinal detachment, vision loss in IP can occur with abnormalities of the inner foveal structure seen on spectral-domain optical coherence tomography, consistent with prior descriptions of foveal hypoplasia. The evolution of abnormalities in the neural and vascular retina suggests a vascular cause of the foveal structural changes. More study is needed to determine any potential benefit of the foveal to parafoveal ratio in children with IP. Even with marked foveal structural abnormalities, vision can be preserved in some patients with IP with vigilant surveillance in the early years of life.
authors.affiliation: University of Utah School of Medicine, Salt Lake City.
authors.forename: Jacob
authors.initials: J
authors.lastname: Basilius
authors.affiliation: Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, University of Utah, Salt Lake City.
authors.forename: Marielle P
authors.initials: MP
authors.lastname: Young
authors.affiliation: University of Utah School of Medicine, Salt Lake City.
authors.forename: Timothy C
authors.initials: TC
authors.lastname: Michaelis
authors.affiliation: Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, University of Utah, Salt Lake City.
authors.forename: Ronald
authors.initials: R
authors.lastname: Hobbs
authors.affiliation: Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, University of Utah, Salt Lake City.
authors.forename: Glen
authors.initials: G
authors.lastname: Jenkins
authors.affiliation: Department of Ophthalmology and Visual Sciences, John A. Moran Eye Center, University of Utah, Salt Lake City.
authors.forename: M Elizabeth
authors.initials: ME
authors.lastname: Hartnett
mesh_terms: D001766:Blindness / Q000209:etiology* / Q000473:pathology; D002675:Child, Preschool; D015862:Choroid Diseases / Q000175:diagnosis / Q000209:etiology; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications* / Q000175:diagnosis; D007223:Infant; D008266:Macula Lutea / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D012163:Retinal Detachment / Q000175:diagnosis / Q000209:etiology; D012164:Retinal Diseases / Q000175:diagnosis / Q000209:etiology*; D015861:Retinal Neovascularization / Q000175:diagnosis / Q000209:etiology; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D014786:Vision Disorders / Q000175:diagnosis / Q000209:etiology*; D014792:Visual Acuity