FP1522 : Diagnosis While Operating : on Table Diagnosis of Brittle Cornea Syndrome

Dr. Ronak Solanki, S16597

Presenting a rare case of a 36-year-old male who presented to us with a Right eye corneal tear after a trivial trauma with fingernail while playing with his child. Patient had a similar trauma in childhood and had lost his sight in the left eye.

On examination patient had pthysis bulbi in the left eye , in the presenting eye patient a documented blue sclera on his previous visit to the hospital. Patient now had a full thickness corneal tear with congestion and proptosis. General examination revealed prognathism , short nose and clinodactyly of the fifth finger . During corneal tear repair there was cheese wiring noticed and on making the side port there was spontaneous extension into a larger linear wound.These on table findings lead to the confirmation of the diagnosis of Brittle Cornea Syndrome in the patient.Loosening of sutures was seen. Family screening was advised.

Conclusion : Brittle Cornea syndrome is a diagnosis of exclusion and corneal tear repair can be a challenging task.

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