Otogenic Sinus Thrombosis and Hemophilia: A Clinical Case

Abstract

Background. Patients with hemophilia are protected from thrombosis by a deficiency of one of coagulation factors, therefore thrombotic complications are rare in them. Currently, few descriptions of spontaneous venous thrombosis in adult hemophiliacs can be found in the literature. Even fewer data of thromboembolic complications are reported in pediatric practice. At present, no clear recommendations are given for the management of thrombotic complications in children with hemophilia, which requires further study of this issue to develop prevention and treatment regimens, including otogenic sinus thrombosis in this category of patients.Case description. A 7-year-old boy was transferred from the Somatic Hospital to the Intensive Care Unit of Regional Clinical Hospital No. 2 in Tyumen (Russia) with an Otorhinolaryngology Unit. He was diagnosed with non-perforative form of acute right-sided suppurative otitis media, acute right-sided mastoiditis, thrombosis of the right sigmoid sinus. The anamnesis shows that the child is diagnosed with hemophilia A, of moderate severity (factor VIII level — 5%), for which he has been receiving factor VIII replacement therapy for the last year. Upon admission, a general blood test revealed no abnormalities, with a slight increase in acute-phase parameters: C-reactive protein, fibrinogen, signs of hypocoagulation. Due to negative dynamics, the child underwent anthromastoidotomy against the background of intensive replacement therapy. Surgery revealed the presence of granulation tissue with hemorrhagic thrombi in the antrum and aditus. In the postoperative period, replacement therapy was accompanied with antibacterial and anticoagulant therapy.Conclusion. The management of patients with otogenic sinus thrombosis against the background of hereditary factor VIII deficiency is a difficult task. On the one hand, it requires surgical intervention and anticoagulant therapy in order to prevent further thrombosis, on the other hand — intensive replacement therapy in order to reduce the risks of hemorrhagic complications. The described case demonstrates the need for an interdisciplinary approach to the diagnosis and treatment of a patient with otogenic thrombosis of lateral sinus against the background of hemophilia A. The approach is to minimize the risks of intraoperative complications and insure a favorable outcome.