Life-Threatening Tongue and Retropharyngeal Hemorrhage in a Patient with Hemophilia A with Inhibitors

Abstract

Patient: Male, 71 Final Diagnosis: Hemophilia A Symptoms: Dysarthria • dysphagia • dyspnea Medication: — Clinical Procedure: Cricothyrotomy • tracheotomy Specialty: Hematology Objective: Unusual clinical course Background: Massive tongue hemorrhage in patients with hemophilia is a medical emergency because it can lead to airway obstruction. However, managing bleeding in patients with inhibitors is more difficult than in patients without inhibitors. We report a case of life-threatening massive tongue and retropharyngeal hematoma in a patient with hemophilia A who had inhibitors. Case Report: The patient was a 71-year-old man with severe hemophilia A with high-responding inhibitors. He was admitted to our hospital with dysarthria and dysphagia secondary to a massive tongue hematoma. Although bypassing therapy was started immediately after admission, he rapidly developed an airway obstruction and cardiopulmonary arrest secondary to suffocation. Cardiopulmonary resuscitation and surgical cricothyrotomy were performed, which restored his pulse and breathing. On day 5 of hospitalization, he underwent tracheotomy under inhibitor-neutralizing therapy, and we began emicizumab on day 19 of hospitalization to prevent further bleeding events. He recovered and was transferred to another hospital for rehabilitation on day 64 of hospitalization. Conclusions: Because tongue hematomas progress dramatically within a few days, prompt airway maintenance by tracheotomy under appropriate hemostatic therapy must be considered. Furthermore, emicizumab induction after primary hemostasis prevents further bleeding. We suggest that initiating emicizumab therapy is a good choice to prevent further bleeding after critical bleeding events if the patient has not received the drug previously.

Retropharyngeal hematoma in a patient with chronic alcoholism

“…Bleeding in this space is rare; however, it may cause airway obstruction and sometimes requires emergency intubation. Most reported cases have been related to head and neck trauma, use of anticoagulants, or underlying bleeding diathesis [1][2][3]. In our case, two mechanisms of injury were suspected.…”

“…Retropharyngeal hematoma is a potentially life-threatening condition due to the risk of airway obstruction. Previous reports have suggested causative factors, such as head and neck trauma, the use of anticoagulants, or an underlying bleeding diathesis [1][2][3]. Herein, we report a case of retropharyngeal hematoma in a patient with chronic alcoholism for whom we could not confirm any predisposing factors at the time of examination.…”

Airway compromise in a patient with hemophilia A

“…Maintaining constant vigilance over the patient's airway is imperative, especially in the context of hemophilia, where even minor bleeds can rapidly escalate into life-threatening situations. 1 Replenishing factor VIII was complicated in this case because the patient had a factor inhibitor. Thus, he required a bypassing product, which was Factor eight inhibitor bypass activity in this case.…”

“…This clinical scenario underscores the vital significance of immediate recognition and interdisciplinary collaboration to avert potential airway compromise. Maintaining constant vigilance over the patient's airway is imperative, especially in the context of hemophilia, where even minor bleeds can rapidly escalate into life‐threatening situations 1 …”

Management of acute hemorrhage in patients with hemophilia or von Willebrand disease in the emergency department

“…Nasal intubation is contraindicated in any hemophilic patient needing intubation [14]. If a patient develops progressive tongue hematomas, prompt tracheostomy in an operative room must be considered [15]. For neck trauma and cervical hematoma, ultrasound is helpful to detect the cricothyroid membrane, and awake fiberoptic intubation can be successfully performed [15,16].…”