Upper Airway Obstruction Following Molar (Dental) Extraction in a Hemophilia A Patient with High-Titer Inhibitor: A Management Dilemma When Resources are Not Enough
Abstract
Introduction Hemophilia is an X-linked bleeding disorder caused by deficiency of factor VIII (hemophilia A) or factor IX (hemophilia B), leading to prolonged clotting time due to delayed fibrin formation. Over the years, advances in diagnosis, replacement therapy, and personalized care have transformed hemophilia from a life-threatening disease into a manageable condition. However, challenges persist, including inhibitor development, breakthrough bleeding, and limited access to comprehensive treatment. Method We present a 24-year-old male with severe hemophilia A (factor VIII activity level of 0.6%) and high titer inhibitor (25.14 Bethesda Units) who underwent one molar extraction for recurrent dental bleeding requiring transfusions and infusions of BPA. He received recombinant activated factor VII (rFVIIa) and tranexamic acid pre-operatively which resulted in minimal bleeding. Eight hours later, he developed a submandibular hematoma prompting initiation of fresh frozen plasma (FFP) transfusion and a repeat dose of rFVIIa. Neck and cranial scan revealed a left masticator space hematoma compressing the upper airway ([ Fig. 1 ]). The patient eventually developed respiratory distress requiring emergent video-assisted intubation. Hemostasis was escalated with alternating FFP and bypassing agent (total of four doses). Fresh frozen plasma transfusion was then continued thereafter, resulting to gradual resolution of hematoma, allowing extubation after six days. Fig. 1 Neck and Cranial CT Scan of Patient. This imaging shows a left masticator hematoma compressing the upper airway at the oropharyngeal level (green arrows). Results Dental procedures are particularly high-risk, especially in hemophilia patients with high-titer inhibitors. For dental extractions, hemophilia A patients typically need plasma factor levels of 40–80% before the procedure and 20–50% postoperatively. Those with inhibitors require bypassing agents administered at specific intervals until healing. Adequate preparation and maintenance are essential to prevent life-threatening bleeding. In resource-limited settings specially in the Philippines, access to these agents is often restricted due to high cost and limited availability. Local unpublished studies by Fusilero et al. (2019) and Avenido et al. (2024) show the use of plasma products as management of hemophilia patients in the Philippines, with only a few patients availing BPAs from humanitarian donations. Alternating FFP with BPA may offer a practical approach, though further studies are needed to validate its effectiveness [ 1 ] [ 2 ] [ 3 ] [ 4 ] [ 5 ] [ 6 ] [ 7 ] [ 8 ] [ 9 ]. Conclusion While clotting factor replacement and bypassing agents remain the gold standard for preventing and controlling bleeding, their cost and limited availability in resource-constrained settings presents a significant challenge. Alternatives like fresh frozen plasma, combined with antifibrinolytics and local hemostatic measures, may offer a temporary solution—though not without limitations. These challenges highlight the urgent need to expand access to essential therapies and establish practical, locally appropriate protocols that ensure safe, equitable care for individuals with bleeding disorders. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany
Fields of Study & Subjects
Hemophilia Treatment and Research
Case Reports on Hematomas
Hemostasis and retained surgical items
Publication details
- Journal / source
- Hämostaseologie
- Volume
- 46
- Issue
- S 01
- Pages
- S58–S59
- Publication date
- 1 February 2026
- Language
- DE