संज्ञानश्वासन और निदान शोध जर्नल
खुला एक्सेस

अमूर्त

Anaesthetic Management of a Patient with Congenital Factor VII Deficiency Undergoing Spine Surgery for a Tethered Cord: A Case Report

Faizah Mufti*, Bashir Ahmad Dar, Majid Khan, Shehla Bashir

Background: Congenital factor VII deficiency is a rare bleeding disorder that can lead to excessive bleeding during the intra-operative period and sometimes also during the postoperative period, which can lead to overall increase in morbidity and mortality. These patients may present with different clinical scenarios which may vary from being asymptomatic to bleeding that can be mild to life threatening. The recombinant form of activated factor VII (rFVIIa, NovoSeven®) has been used in the management of hemorrhage in the patients with factor VII deficiency.

Case: Patient’s factor VII levels were determined. We administered recombinant factor VIIa, two hours prior to surgery. Repeat sample for coagulation profile was taken 1 hour after administration. Post operatively dose was to be repeated 4 hourly until adequate hemostasis was achieved. Throughout the procedure patient was thoroughly monitored and surgery was completed uneventfully in 2 hours and 15 mins.

Results: Our patient’s factor assay showed a severe factor VII deficiency (0.3% of normal). However, the surgery was performed successfully without any bleeding or thrombogenic complications.

Conclusion: Patients with congenital FVII deficiency who require surgical intervention can be effectively managed with rFVIIa. Our patient received the dose pre operatively prior to intervention and was well tolerated and helped in achieving effective hemostasis both during surgery and in the post-operative period.