Management Challenges in a Case of Type 1 Von Willebrand Disease with bilateral Retinal Detachment.

1. Jaideep Tyagi
2. Arun Singhvi
3. Shashank Gang
4. Navneet Kumar
5. Manoj Kumar

ASG Group of Eye Hospitals, Shashtri Nagar, Jodhpur

Purpose – Management of a case of Type 1 Von willebrand disease with bilateral retinal detachment.

Methods – A 18 year old patient, a known case of haemophilia, presented to us with bilateral retinal detachment. Patient's APTT was 63.10 sec (control blood -34.89) and factor VIII essay was 11 % of normal level. Bilateral retinal buckling surgery was planned. patient was transfused 2000 IU of factor VIII and was operated within next 12 hours. Patient was transfused 1000 IU of factor VIII every 24 hours for next 3 days.

Result - Both eye buckling surgery was successful with retinal attachment in both eyes. Patient developed large subconjunctival hematoma in left eye on 1 post operative day. patient had an uneventful recovery for next 1 week, when he developed right eye subconjunctival hematoma. he was again transfused 1000IU of factor VIII for next 2 days

Conclusion – Retinal Detachment with haemophilia can be managed after transfusing factor VIII both pre- and post –operatively. Even with all the precautions increased risk of bleeding and hematoma formation persists.