# Life-Threatening Pharyngolaryngeal Hematoma in a Patient With Hemophilia A

## TL;DR

A patient with hemophilia A developed a life-threatening airway hematoma, requiring a tracheotomy and extended coagulation factor treatment to prevent asphyxia.

## Contribution

The case highlights the importance of early laryngoscopy and multidisciplinary emergency intervention in managing airway hematomas in hemophilia patients.

## Key findings

- Tracheotomy under general anesthesia was performed due to high risk of airway obstruction.
- Efraloctocog alfa maintained FVIII levels above 80% for eight days, aiding hematoma resolution.
- The patient recovered and was discharged with a closed tracheostoma after 18 days.

## Abstract

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.