SHM Converge Scientific Abstract ePoster Gallery-A Rare Case of Factor Vii Deficiency and How We Managed Spine Surgery

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This case describes a 46-year-old woman with hypertension, morbid obesity, thoracic spine stenosis, and known Factor VII (FVII) deficiency who presented with bilateral lower extremity weakness. CT myelogram showed severe spinal canal stenosis with cord compression at T10-11 and T11-12. Preoperative labs showed prolonged PT and INR with normal aPTT; a mixing study corrected the PT, and FVII activity was low at 24%, confirming FVII deficiency.<br /><br />Because spine surgery carries significant bleeding risk, hematology recommended perioperative replacement with recombinant Factor VII (NovoSeven). The patient received 2 mg immediately before surgery and 1 mg 8 hours afterward. Her FVII level normalized to 89%, and she underwent T10-12 revision decompression with posterior instrumented fusion without bleeding complications.<br /><br />The report also reviews FVII deficiency, a rare bleeding disorder also known as Alexander’s disease. It may be inherited or acquired, and clinical severity does not always correlate with factor level. Inherited deficiency is autosomal recessive, while acquired causes include liver disease, vitamin K deficiency, and sepsis. Typical laboratory findings are prolonged PT with normal aPTT. Treatment options include recombinant Factor VII, prothrombin complex concentrate, and fresh frozen plasma.<br /><br />Overall, the case highlights the importance of preoperative bleeding risk assessment and timely hematology-guided correction in patients with rare coagulation disorders undergoing high-risk surgery.

Asset Subtitle

Nithin G. Thomas

Meta Tag

Author List Andreea S. Xavier, Nithin G. Thomas

Category Clinical Vignettes

Concept Factor VII Deficiency

Concept Factor VII Level

Concept Prothrombin Time

Concept Recombinant Factor VII

Concept Clinical Presentation

Distinguished Non-Finalist

Presenter Organization Department of Emergency Medicine, McGovern Medical School at UT Health Science Center, Houston

Presenting Author Nithin G. Thomas

Track Adult

Keywords

Factor VII deficiency

spine surgery

recombinant Factor VII

prolonged PT

normal aPTT

thoracic spinal stenosis

cord compression

perioperative bleeding risk

hematology consultation

coagulation disorder

Factor VII Deficiency

Factor VII Level

Prothrombin Time

Recombinant Factor VII

Clinical Presentation