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Purpose : Reporting a case of Incontinentia pigmenti in preterm with fundus fluorescein angiography findings
Methods : A female born at 36 weeks of gestation with birth weight of 1800 grams, and history of seizures and respiratory distress at 3 weeks after birth, was referred for retinopathy of prematurity screening at the age of 4 weeks.
On general physical examination, multiple grey brown vesiculo-papular skin lesions were noted on the flexor aspects of both upper and lower limbs as well as on the sides of the abdomen and in the genital area .There were a few lesions that had resolved with brownish pigmentation. Ocular examination revealed normal conjunctiva, clear cornea, clear lens and normal other anterior segment structures, while dilated indirect ophthalmoscopic examination of fundus revealed, tortuous and dilated vessels at posterior pole along with large avascular vascular loops in zone 1.
FFA revealed delayed arm to retina circulation time of more than 5 seconds. The retinal arteries were seen only till posterior to equator with large mid-peripheral avascular loops. There was gross peripheral capillary non-perfusion and peripapillary capillary plexus could be seen for about two to four disc diameters in both eyes. The right eye showed leakage from the disc and the left eye showed leakage from the tortous vessels.
Results : Patient was subsequently diagnosed and managed as case of Incontinentia pigmenti
Conclusion : Features like uncommon retinal vasculature presentation of the disease, association with skin lesions, have previously never been reported. We describe the management of the case and subsequent prognosis of the patient in our presentation.
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