Dr. Maneesha Mohan Bellala, B18537, Dr. Shashikant Shethy, Dr. Saranya Balakrishnan
Reporting a 14 year old male child who presented with defective vision in both eyes since childhood.On ocular examination,epicanthal folds with nystagmus noted. vision was HM both eyes.Eyes were micro-ophthalmic with microcornea. Pupils were 3mm;not reacting to light and accomodation and non responsive to atropinisation with persistant pupillary membrane in both eyes. USG Bscan revealed normal posterior segment with a cataractous lens. Hallermann-Streiff is a rare syndrome characterized by multiple congenital anomalies, especially in the head and face.Essential features of this syndrome are: dyscephaly with birdface, dental anomalies, proportionate short stature, hypotrichosis, atrophy of skin, bilateral microphthalmos, and cataract. All the above were present in this case. PPM removal with lens extraction was done in the left eye following which his bcva was 6/24.Similar procedure has been planned in other eye. it may not be a permanent fix but every little bit of it is significant.