AIM:To analyze the ocular clinical manifestations especially retinopathy of pediatric patients with incontinentia pigmenti(IP).

METHODS: Clinical data of 33 patients diagnosed with IP from January 2014 to December 2018 in Hunan Children's Hospital were retrospectively analyzed. The follow-up was 1a. All the patients underwent ocular examinations and systemic physical examinations. Patients received treatments according to their fundus condition.

RESULTS:Totally 31 female patients and 2 male patients, who aged from 5d-23mo at first visit, with the average age 3.38±5.02mo. There were 14 patients had ocular anomalies, including corneal opacity(1 case, 1 eye), cataract(1 case, 1 eye), exotropia(2 cases, 2 eyes)and retinopathy(14 cases, 23 eyes). In the 14 patients with ocular anomalies, one patient died due to neuropathy, one patient underwent vitrectomy in the right eye and fundus fluorescence angiography(FFA)combined with retinal laser photocoagulation in the left eye, one patient had lensectomy and vitrectomy in the right eye and FFA combined with retinal laser photocoagulation in the left eye during the follow-up. At the last follow-up, one of the 13 surviving patients with ocular anomalies had retinopathy of IP stage 1, while retinal lesions regressed in other patients.

CONCLUSION:The ocular manifestations of IP are diverse, which is identified with retinopathy. Standard ophthalmic fundus screening and regular follow-up are of great significance.