Induced Acute Hereditary Angioedema: A Life-Threatening Condition

Abstract

A 19-year-old man presented to the emergency room complaining of dysphagia, as well as swelling of the right side of his face that had spread to his right lower eyelid. The patient had been referred by his general dental practitioner who suspected a dental abscess when the patient returned to him several hours following extirpation of the upper right first premolar nerve. The patient’s medical history was unremarkable. His vital signs were: blood pressure 130/55, pulse 84, oral temperature 36.5°C. The patient was tachypnoeaic and spoke with a “hot potato” speech. He had an oxygen saturation of 92%. Examination revealed edematous swelling on the right side of his face, especially in the periorbital area and lips. The swelling was not sensitive to palpation, was not warm to the touch, and there was no evidence of rubor. Laboratory tests showed: white blood cell count 8.95 10/L; eosinophils 4.5%; lymphocytes 28.8%; neutrophils 56.4%; red blood cells 26 10/mm; hemoglobin 17.8g/dL. Mouth opening was unrestricted and there was no evidence of submandibular lymphadenopathy. Intraorally, the maxillary right first premolar was temporarily filled, was not sensitive to percussion, with no buccal swelling or sensitivity to palpation. No sign of inflammation was present. The uvula, however, was swollen and edematous. X-ray examination (orthopantomogram) showed a deep carious lesion in the lower left second molar, but no signs of dentoalveolar abscess were present anywhere in the maxilla or mandible. Computerized tomography scan, performed to try to determine the cause of the dysphagia and respiratory distress, showed massive edema of the oropharynx and hypopharynx, resulting in almost complete closure of the airway (Fig 1). On further questioning, the patient’s father informed us that he himself suffered from C1 esterase deficiency (C1 INH) and was treated on a regular basis with danazol (Danocrine; Sanofi Winthrop, Bridgewater, NJ). A tentative diagnosis of C1 INH was made. Because of decreasing oxygen saturation, the patient was administered 2 doses of 0.5 mL adrenaline 1:1000 intravenously 15 minutes apart and 125 mg methylprednisolone sodium succinate (Solu-Medrol; Pharmacia and Upjohn, Allegan, MI) intravenously. This treatment yielded no signs of improvement. The patient was then given 2 units of fresh frozen plasma (FFP), and an almost immediate improvement was noted. The patient’s condition improved significantly approximately 40 minutes following the start of treatment, expressed by decreased swelling and improved swallowing and speech. The patient was released from the hospital 24 hours after arrival, in good health and without any of the symptoms that had been present on admission. He was released with a recommendation to undergo further tests, under the care of his general medical practitioner. Approximately 1 week after his release from hospital the patient returned to our department for results of laboratory tests, which revealed a quantitative defect of C1 INH. Received from the Department of Oral and Maxillofacial Surgery,