Journal d'ophtalmologie clinique et expérimentale
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Phacomatosis Pigmentovascularis: A Case Report

Shams Mohammed Noman, Umme Salma Akbar, Mehjabin Haque, Muzahidul Islam, Dipankar Sarkar, Shabiha Kaoser

Purpose: To observe and describe a rare disease Phacomatosis Pigmentovascularis as case series.

Case: Detailed history taking and examination was done in the glaucoma clinic of Chittagong Eye Infirmary and Training Complex (CEITC) for the diagnosis of those suspected cases. Detailed systemic examinations of skin, cardiac and nervous system were done. Ocular examinations included visual acuity, intraocular pressure, gonioscopy (while possible), torch light and slit lamp examination and fundus evaluation were done.

Results: Total of 8 cases were included (Five male and three female). Average age group was 4+/-2 years. All of them had haemangiomatous lesion in the hand and feet. Five of them had haemangioma in the both side of the face. Five cases had bilateral and rest two had unilateral naevus of ota in the eye. Three cases had bilateral and rest five cases had unilateral mild buphthalmos and raised intraocular pressure (IOP). There was history of convulsion in two cases and CT brain revealed calcification in the brain. All cases needed trabeculectomy surgery to reduce IOP. Two cases needed Ahmed valve implant.

Conclusion: Phacomatosis pigmentovascularis is a rare entity. Early diagnosis and appropriate measurement can save their vision as well as life.