Phacomatosis pigmentovascularis- A case series

Abstract

Purpose To observe and describe a rare disease Phacomatosis pigmentovascularis as a case series To describe a rare disease in ophthalmology called Phacomatosis pigmentovascularis and to observe it in 8 patients in a series. Method Detailed history taking and examination was done in the glaucoma clinic of Chittagong Eye Infirmary and Training Complex 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 Result Total of 8 cases were included. Five male and three female. Average age group was 4 +/-2 years. All of them had hemangiomatous lesion in the hand and feet.5 of them had hemangioma in the both side of the face.5 cases had bilateral and rest two has 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 & CT-brain revealed calcification in the brain. All cases needed trabeculectomy surgery to reduce IOP. 2 cases needed valve implant. Conclusion: Phacomatosis pigmento vascularis is a rare entity. Early diagnosis and appropriate measurement can save their vision as well as life

Medivision J. of Med. and Health Sci. Vol 1(1), Jul 2026; p 85-87