Ophthalmic evaluation, treatment, and follow-up of two cases of incontinentia pigmenti / Avaliação oftalmológica, tratamento e seguimento de dois casos de incontinentia pigmenti

Incontinentia pigmenti (IP) is an X-linked dominant disorder affecting the skin, teeth, eyes, and central nervous system. Ocular changes are common and may lead to severe vision loss. We report on the ocular manifestations in two young girls with IP, with emphasis on the asymmetry of this condition in both eyes and associated retinal problems. The outcomes of laser treatment of the ischemic peripheral retina were good and resulted in stability of vision.

Incontinentia pigmenti (IP) é uma desordem ligada ao X dominante afetando a pele, dentes, olhos e sistema nervoso central. Alterações oculares são comuns e podem levar a severa perda visual. Nós relatamos manifestações oculares de duas jovens pacientes com IP, enfatizando a assimetria da condição em cada olho e também alterações retinianas que possam ocorrer. Tratamento a laser na periferia isquêmica da retina gera bons resultados e estabiliza a visão.

Cryotherapy for retinopathy of incontinentia pigmenti

A case with typical skin lesions of incontinentia pigmeti showed progressing retinal vascular abnormalities. So cryotherapy was done on avascular peripheral retina, and retinal vascular changes ceased. We propose that cryotherapy may be a good treatment method for progressing retinal vascular lesions of incontinentia pigmenti.