Case of concurrent factor VII and factor XI deficiencies manifesting as spontaneous lower extremity compartment syndrome

Marshalek, Joseph P.; Yashar, David; Huynh, Karen; Tomassetti, Sarah

doi:10.1002/ccr3.5710

Cited by 2 publications

(1 citation statement)

References 24 publications

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“…There is a limited number of reported cases of factor VII+XI deficiency in the literature. [19][20][21] Our patient who was diagnosed with factor VII+XI deficiency preoperatively at the age of 51 when he was investigated due to preoperative aPTT and PT prolongation. He was planned to underwent a transcatheter aortic valve implantation.…”

Section: Discussionmentioning

confidence: 99%

Perioperative Management of Patients with Rare Congenital Factor Deficiency

Can

2023

EJMI

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Objectives: Fibrinogen, prothrombin, factor V, VII, X, XI, XIII and V+VIII deficiencies are defined as rare congenital factor deficiencies (RCD). Due to small number of patients and lack of data, perioperative management can be challeging. We aimed to evaluate the RCD patients who underwent surgery. Methods: Thirteen women, eight men, totally 21 patients was retrospectively analyzed. Results: Ten patients were diagnosed incidentally by investigating the bleeding tests performed before the procedure and routine bleeding tests. Two patients had diagnosed with fibrinogen disorder, one patient factor V deficiency, 9 patients factor VII deficiency, 2 patients factor X deficiency, 4 patients factor XI deficiency, 3 patients combined factor deficiency. Six patients underwent gynecological intervention, 4 patients dental procedures, 2 patients orthopedic surgery, 2 patients appendectomy, 2 patients urological intervention, one patient gastrectomy, one patient rhinoplasty, one retinal surgery, one lipoma excision, one cardiological intervention. Two of the patients were treated with only antifibrinolytic therapy before the procedure/surgery, while 19 patients were treated with replacement therapy appropriate to their disease. Ten patients were continued with replacement therapy after the procedure/surgery. None of the patients had bleeding. Conclusion: Perioperative management of patients with rare factor deficiency is crucial in terms of bleeding and related complications.

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Section: Discussionmentioning

confidence: 99%