FP313

Post operative incidental diagnosis of Glanzmann thrombasthenia in a Child with Stickler Syndrome 

An 11y/o boy presented with sudden diminution of vision in RE since 2 months.He gave h/o recurrent epistaxis since 1yr of age. On GPE he had high arched palate, pectum carinatum, petechial lesions on body, and cleft hands. B scan showed axial length of 28.8mm in RE and 29.89mm in LE. RE FUNDUS showed subtotal RD extending from 2o clock to 10o clock meridian with macular involvement. LE FUNDUS showed myopic fundus. After taking ENT clearance, he underwent RE scleral buckling with PPV under GA which was uneventful. On postop day1 he presented with retrobulbar hemorrhage with protusion of globe and vitreous hemaorrhage. Hematology workup in specialised center showed a deficiency of GPIIb/IIIa receptor and Glanzman thrombasthenia was diagnosed.The retrobulbar and vitreous hemorrhage subsided with platelet infusions but retina has redetached due to PVR.Take away message is preop assessment to decide cause of repeated epistaxis in Stickler syndrome cases should be investigated more robustly.