Life-Threatening Pharyngolaryngeal Hematoma in a Patient With Hemophilia A

Although airway hematomas are a rare complication of hemophilia, emergency treatment is required in these cases since airway obstruction can cause asphyxia. While the main treatment for hematomas in the airway entails replacing the coagulation factor products, in cases with a high risk of asphyxia, deciding whether or not to perform a tracheotomy can be difficult. We report the case of a patient with severe hemophilia A with rapidly worsening dyspnea due to hematomas caused by an acute upper respiratory tract infection and covering the airway at the upper part of the epiglottis and the tongue base. Laryngoscopy revealed a hematoma of the tongue base, but the hypopharynx and larynx were not visible owing to the hematoma. A CT scan also showed narrowing of the airway due to the hematoma. Given the high risk of obstruction, a tracheotomy was performed under general anesthesia. Efraloctocog alfa, a recombinant coagulation factor VIII (FVIII) product with an extended half-life, was administered to maintain trough levels of coagulation FVIII at 80% or above for eight days after tracheotomy. A repeat CT scan performed four days after the tracheotomy confirmed that the hematoma had improved. The patient was discharged 18 days after the tracheotomy with the tracheostoma closed. If patients with hemophilia complain of throat discomfort or dysphagia, the airway should be assessed via laryngoscopy. Emergency tracheotomy and treatment with a recombinant coagulation factor product by a medical team comprising emergency physicians, hematologists, and otolaryngologists is required for hemophilia patients with a high risk of airway obstruction.