ncontinentia pigmenti (IP), or Bloch-Sulzberger syndrome, is a rare X-linked dominant disorder mainly seen in females because a single allele in male embryos is usually fatal. 1,2 Incontinentia pigmenti usually presents with a characteristic skin rash that leaves hypopigmented patches on the trunk and limbs. 3 The most serious events involve the retina and central nervous system (CNS) in 18% to 30%. [2][3][4][5] Loss-offunction mutations in IKBKG/NEMO impair nuclear factorkappa B (NF-κB) signaling and are responsible for most cases of IP. 6,7 Although NF-κB signaling is ubiquitous, effects from the IKBKG/NEMO mutation do not manifest in all tissues.Vision loss has been associated with vascular occlusions, secondary extraretinal neovascularization (NV), tractional retinal detachments, 2,5,8 and foveal hypoplasia. 8 Herein, we report findings from spectral-domain optical coherence tomography (SD-OCT) in children with IP examined before age 5 years. Poor visual behavior corresponded with inner retinal structural abnormalities.IMPORTANCE 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).OBSERVATIONS 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 AND RELEVANCE 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.

PRB exchange can transiently alleviate persistent or recurrent post-AUS SUI in the absence of mechanical failure or urethral pathology. Caution is warranted in patients with prior radiation as this was a risk factor for urethral erosion. Although many patients may require device revision within 2 years, it can be a temporizing solution that avoids urethral manipulation and periprocedural device deactivation.

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