2. Case Presentation
A 10-year-old, 34 kg female with no significant medical history was admitted with recently diagnosed acute myeloid leukemia. She was treated with etoposide 150 mg/m2/dose on days 1-5, cytarabine 200 mg/m2/dose on days 6-12, mitoxantrone 5 mg/m2/dose on days 6-10, and triple intrathecal therapy on day 6 as induction therapy (JPLSG AML-05 protocol2). Following chemotherapy, her course was complicated by continued febrile neutropenia and oral mucositis. Broad spectrum antibiotics including meropenem, teicoplanin, and caspofungin with granulocyte colony-stimulating factor (G-CSF) was initiated. As multiplex polymerase chain reaction proved positive for Herpes simplex viral infection, acyclovir was added. She complained of severe throat pain and dysphagia due to worsening mucositis and required continuous infusion of morphine.
On day 24, the patient complained of increasing throat pain and difficulty breathing. She was found sitting up in bed, leaning forward, drooling. Pan-inspiratory stridor and mild effort of breathing was observed on physical examination. She was alert with her vital signs otherwise stable. Examination on nasolaryngoscopy revealed extensive desquamation of the oropharyngeal mucosa and multiple, confluent ulcerative lesions with significant edema extending down to the arytenoid cartilage (Fig. 1). The patient was taken to the operating room for elective intubation with otolaryngology team on standby for tracheostomy placement. A size 5.0 mm internal diameter endotracheal tube was successfully inserted by video laryngoscopy with anesthesia maintaining spontaneous ventilation.
The patient was then transported to the pediatric intensive care unit. As infection being considered as an aggravating factor, antimicrobiotic agents and G-CSF was continued, and granulocyte transfusion from paternal donor was attempted.
On day 28, she developed significant spontaneous bleeding from the gingiva and the oropharyngeal ulcers, resulting in hemorrhagic shock. Platelet transfusions and vasoconstrictors were warranted for resuscitation. Nasolaryngoscopy revealed further aggravation of the ulcerative lesions with active oozing and worsening edema of the oropharyngeal tissues showing complete obstruction of the retropharyngeal space. Packing of the oropharyngeal space was performed as electrocoagulation of bleeding vessels failed to control bleeding.
Surgical tracheostomy was considered taking the above finding into account. After thorough discussion with the oncologists and the otolaryngologists a decision was made to wait for resolution of neutropenia considering the elevated risk of procedure in the nadir phase and expected improvement of mucositis following recovery of neutrophil counts.
On day 29, absolute neutrophil count exceeded 500 /µL and G-CSF was discontinued. Her bleeding was well controlled. On day 31, defervescence was achieved. On day 32, improvement of the ulcerative lesions and airway swelling was confirmed on nasolaryngoscopy. Adequate leakage around the endotracheal tube was verified. The patient was able to cough, swallow efficiently requiring minimum ventilatory support. Extubation was successful on first attempt. Repeated blood cultures obtained throughout her course were found to be negative. Her course after extubation was uneventful.