Replacement Therapy for Surgery in FVII Deficiency: The Ster Experience Concerning 63 Interventions.

Abstract

Background: Excessive bleeding represents a major complication of surgical interventions, and its control is especially relevant in patients with Congenital Bleeding Disorders. In FVII deficiency, scanty data is available in surgery to guide treatment strategies. Methods: The STER (Seven Treatment Evaluation Registry) is a multi-centre, prospective, observational, web-based registry providing the frame for an extensive and structured data collection. Results: As of Dec. 2008, sixty-three surgical operations (36 "major" and 27 "minor") were performed in 54 subjects (29 females and 25 males) with a FVII deficiency (31 previously symptomatic; 9 with FVIIc 1%, 9 with 1 to 5% and 36 with > 5%). Cases reported were allocated into the following surgical categories: i. Oral surgery (n=13), ii. Orthopaedic surgery (n=12), iii. Neuro-, Head & Neck-, Eye- and ENT-surgery (n=15), iv: Abdominal and Obsterical & Gynecological surgery (n=12), v. Invasive procedures (n=10), vi. Cardiosurgery (n=1). Replacement Therapy (RT) was carried out using recombinant Factor VIIa (rFVIIa) in 52 interventions and a plasma-derived FVII (pdFVII) concentrate in 11. RT was conducted for a mean of 4.22 days (range 1-20) in major surgery and 2.15 days (range 1-10) in minor surgery. Bleeding occurred in 3 major orthopaedic interventions (two patients) during the operation but rFVIIa-based RT was performed with a very low dose in each case (6 to 13 µg/kg/b.w.) (Figure).