Ocular manifestations of incontinentia pigmenti

PURPOSE: The study aimed to evaluate the ocular manifestations in patients with incontinentia pigmenti (IP). METHODS: Thirty patients from different parts of Sweden participated. Orthoptic and ocular examinations were performed as well as evaluation of refraction and visual acuity. RESULTS: Ocular manifestations, probably associated with IP, were found in 77% (23/30) of the patients. Thirteen had serious or vision-threatening eye manifestations in one eye, of whom 7 were totally blind in that eye from retinal detachments. Ten patients had minor retinal and/or corneal changes. CONCLUSION: Ocular lesions in patients with IP may be serious and lead to blindness because of retinal disease. Ophthalmological follow-up is essential in the neonatal period and such a programme is recommended.