Posterior Fusion in a Case of Lenke Type 2 Adolescent Idiopathic Scoliosis with Severe Factor VII Deficiency: A Case Report
,
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.
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[PubMed: 5560054].
3. Alexander B, Goldstein R, Landwehr G, Cook CD. Congenital
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with hemorrhage rectified by serum and serum fractions. J
Clin Invest. 1951;30(6):596-608. doi: 10.1172/jci102477. [PubMed:
14841260]. [PubMed Central: PMC436294].
4. Perry DJ. Factor VII Deficiency. Br J Haematol. 2002;118(3):
689-700. doi: 10.1046/j.1365-2141.2002.03545.x. [PubMed:
12181036].
5. Lane A, Cruickshank JK, Mitchell J, Henderson A, Humphries S,
Green F. Genetic and environmental determinants of factor VII
coagulant activity in ethnic groups at differing risk of
coronary heart disease. Atherosclerosis. 1992;94(1):43-50. doi:
10.1016/0021-9150(92)90186-k. [PubMed: 1632858].
6. Fauci A, Longo D, Kasper D, Jameson J, Loscalzo J, Hauser S.
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NY: McGraw Hill Medical; 2022.
7. Silberstein LE, Anastasi J. Hematology: Basic Principles and
Practice. 6th ed. Philadelphia, PA: Elsevier Health Sciences; 2017.
8. McPherson RA, Pincus MR. Henry's Clinical Diagnosis and
Management by Laboratory Methods. 24nd ed. Philadelphia,
PA: Elsevier Health Sciences; 2021.9. Seligsohn U, White G. Inherited Deficiencies of Coagulation
Factors II, V, VII, X, XI, and XIII and Combined Deficiencies of
Factor V and VIII and of Vitamin K–Dependent Factors. In:
Lichtman MA, Kaushansky K, Prchal JT, Levi MM, Burns LJ,
Linch DC, editors. Williams Hematology. 10th ed. New York, NY:
McGraw Hill Medical; 2022. p. 1617-38.
10. Giansily-Blaizot M, Biron-Andreani C, Aguilar-Martinez P, de
Moeloose P, Briquel ME, Goudemand J, et al. Inherited factor
VII deficiency and surgery: clinical data are the best criteria to
predict the risk of bleeding. Br J Haematol. 2002;117(1):172-5.
doi: 10.1046/j.1365-2141.2002.03408.x. [PubMed: 11918551].
11. Butenas S, Brummel KE, Branda RF, Paradis SG, Mann KG.
Mechanism of factor VIIa–dependent coagulation in
hemophilia blood. Blood. 2002;99(3):923-30. doi:
10.1182/blood.v99.3.923. [PubMed: 11806995].
12. Herrmann FH, Wulff K, Auberger K, Aumann V, Bergmann F,
Bergmann K, et al. Molecular biology and clinical manifestation
of hereditary factor VII deficiency. Semin Thromb Hemost.
2000;26(4):393-400. doi: 10.1055/s-2000-8458. [PubMed: 11092214].
13. Jain S, Donkin J, Frey MJ, Peltier S, Gunawardena S, Cooper DL.
Phenotypical variability in congenital FVII deficiency follows
the ISTH-SSC severity classification guidelines: a review with
illustrative examples from the clinic. J Blood Med. 2018;9:211-8.
doi: 10.2147/jbm.S157633. [PubMed: 30510462]. [PubMed
Central: PMC6250109].
14. Chaudhry R, Killeen RB, Babiker HM. Physiology, Coagulation
Pathways. 2025 Jun 2. In: StatPearls [Internet]. Treasure Island,
FL: StatPearls Publishing; 2025. [PubMed: 29489185].
15. Carew JA, Pollak ES, High KA, Bauer KA. Severe factor VII
deficiency due to a mutation disrupting an Sp1 binding site in
the factor VII promoter. Blood. 1998;92(5):1639-45. [PubMed:
9716591].
16. Cutler JA, Patel R, Mitchell MJ, Savidge GF. The significance of
published polymorphisms in 14 cases of mild factor VII
deficiency. Blood Coagul Fibrinolysis. 2005;16(2):91-5. doi:
10.1097/01.mbc.0000161561.51517.31. [PubMed: 15741795].
17. Kaushansky K, Lichtman MA, Prchal JT, Levi MM, Press OW,
Burns LJ, et al. Williams hematology. 9th ed. New York, NY:
McGraw-Hill Education; 2016.
18. Greer MD JP, Foerster J, Rodgers GM, Paraskevas F, Glader BE,
Arber DA, et al. Wintrobe's clinical hematology. 12th ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 2009.
19. Napolitano M, Di Minno MN, Batorova A, Dolce A, Giansily-
Blaizot M, Ingerslev J, et al. Women with congenital factor VII
deficiency: clinical phenotype and treatment options from
two international studies. Haemophilia. 2016;22(5):752-9. doi:
10.1111/hae.12978. [PubMed: 27338009].
20. Geng Y, Verhamme IM, Sun MF, Bajaj SP, Emsley J, Gailani D.
Analysis of the factor XI variant Arg184Gly suggests a
structural basis for factor IX binding to factor XIa. J Thromb
Haemost. 2013;11(7):1374-84. doi: 10.1111/jth.12275. [PubMed:
23617568]. [PubMed Central: PMC4158700].
21. Yoshida A, Kimoto Y, Ejiri K, Mitani Y, Kawamata T. Anesthetic
management of a patient with factor VII deficiency
undergoing laparoscopic colectomy: a case report. JA Clin Rep.
2016;2(1):30. doi: 10.1186/s40981-016-0059-0. [PubMed:
29492425]. [PubMed Central: PMC5814789].
22. Gruppo RA, Kessler CM, Neufeld EJ, Cooper DL. Assessment of
individual dose utilization vs. physician prescribing
recommendations for recombinant activated factor VII
(rFVIIa) in paediatric and adult patients with congenital
haemophilia and alloantibody inhibitors (CHwI): the Dosing
Observational Study in Hemophilia (DOSE). Haemophilia.
2013;19(4):524-32. doi: 10.1111/hae.12113. [PubMed: 23551918].
23. Blechman S, Fruchtman Y, Perry ZH, Mazar J, Ben Harosh M,
Abed A, et al. Clinical and Laboratory Findings in Jewish and
Bedouin Patients in Southern Israel Who Were Diagnosed with
Factor VII Deficiency. Isr Med Assoc J. 2019;21(5):318-21. [PubMed:
31140222].
24. Marder VJ, Shulman NR. Clinical Aspects of Congenital Factor
vii Deficiency. Am J Med. 1964;37:182-94. doi: 10.1016/0002-
9343(64)90004-x. [PubMed: 14206752].
25. Rajpurkar M, Cooper DL. Continuous infusion of recombinant
activated factor VII: a review of data in congenital hemophilia
with inhibitors and congenital factor VII deficiency. J Blood
Med. 2018;9:227-39. doi: 10.2147/jbm.S184040. [PubMed:
30568523]. [PubMed Central: PMC6276614].
26. Friederich PW, Henny CP, Messelink EJ, Geerdink MG, Keller T,
Kurth KH, et al. Effect of recombinant activated factor VII on
perioperative blood loss in patients undergoing retropubic
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| Files | ||
| Issue | Vol 11 No 3 (2025) | |
| Section | Case Report | |
| DOI | https://doi.org/10.18502/jost.v11i3.19407 | |
| Keywords | ||
| Adolescent Idiopathic Scoliosis (AIS)- Lenke- factor VII deficiency | ||
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How to Cite
1.
Malekzadeh M, Mrzashahi B, Shabani S. Posterior Fusion in a Case of Lenke Type 2 Adolescent Idiopathic Scoliosis with Severe Factor VII Deficiency: A Case Report. J Orthop Spine Trauma. 2025;11(3):124-8.
