Posterior Fusion in a Case of Lenke Type 2 Adolescent Idiopathic Scoliosis with Severe Factor VII Deficiency: A Case Report

Siamak Shabani; Maziar  Malekzadeh; Babak Mirzashahi

doi:10.18502/jost.v11i3.19407

Siamak Shabani Spine Fellowship, Department of Spine Surgery, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Maziar Malekzadeh Spine Fellowship, Department of Spine Surgery, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Babak Mirzashahi Assistant Professor, Department of Spine Surgery, Imam Khomeini Hospital Complex, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/jost.v11i3.19407

Keywords: Scoliosis; Factor VII Deficiency; Adolescent; Spinal Fusion

Abstract

Background: Congenital factor VII (FVII) deficiency is a rare autosomal recessive coagulation disorder that poses significant challenges during major surgeries, particularly high-risk procedures like instrumented spinal fusion for adolescent idiopathic scoliosis (AIS).

Case Report: We present the case of a 15-year-old girl diagnosed with Lenke type 2 AIS and severe congenital FVII deficiency. She was referred to Imam Khomeini Hospital Complex, Tehran, Iran, for corrective spinal fusion. Preoperatively, a single dose of recombinant activated FVII (rFVIIa) at 20 mcg/kg was administered to maintain hemostasis. The instrumented spinal fusion was performed under general anesthesia using meticulous surgical techniques. Notably, no additional doses of rFVIIa were required during the procedure.

Conclusion: This case demonstrates that with appropriate preoperative factor replacement and careful intraoperative hemostatic management, patients with severe FVII deficiency can safely undergo corrective spinal fusion. It underscores the critical importance of sustaining hemostasis in managing high-risk surgical patients with coagulopathies.