We report a clinical case of a patient with hereditary angioedema (HAE, Type III) in which, by implementing careful preoperative preparations to prevent the occurrence of edema, good course was achieved without the prophylactic administration of C1-inhibitor (C1–INH). We recommended the prophylactic administration of Berinert P, a human plasma-derived C1–INH formulation; however, due to the risk of developing a viral infection, both the patient and her mother refused to receive Berinert P. In cases where C1–INH cannot be administered as a prophylactic measure, it is essential to make sufficient preparations, including urgent tracheotomy, to prevent sudden occurrence of edema, with the cooperation of the anesthesiologist.
We report a clinical case in which, with careful preparations preoperatively taken in case of the appearance of edema.
Because of the risk of viral infection, both the patient and her mother refused prophylactic administration of Berinert P.
This patient had undergoneimpacted mandibular wisdom tooth extraction without prophylactic administration of C1-INH.
It is important to make sufficient preparations for a response to the urgent appearance of edema in this case.
Hereditary angioedema (HAE) is an extremely rare autosomal dominant inherited disorder, in which edema occurs due to the reduction or dysfunction of the C1-inhibitor (C1–INH) [ ]. It is also known that this disease causes angioedema associated with stress resulting from dental therapy and other treatments. Administration of a C1–INH formulation is recommended at the onset of edema and as a prophylactic measure [ ]. Some studies have demonstrated the effectiveness of C1–INH administration as a short-term prophylactic treatment for patients with HAE undergoing procedures such as wisdom tooth extraction [ , , ]. However, this drug is highly expensive and a blood-derived product, which poses the risk of developing a viral infection and can lead to anaphylaxis. Therefore, controversy on whether C1–INH administration should be administered in oral surgery exists. In addition, there is no definitive evidence to indicate whether C1–INH is effective as a prophylactic agent.
Herein, we report a case in which, by implementing careful preoperative preparations to prevent the occurrence of edema, we obtained good clinical course without the prophylactic administration of C1–INH in a patient with HAE, who had undergone bilaterally impacted mandibular wisdom tooth extraction under intravenous anesthesia.
A 22-year-old female patient was referred to the Department of Oral Medicine in Hokkaido University Hospital with a complaint of experiencing frequent spontaneous pain in the left mandibular wisdom tooth. There was no past history of C1–INH administration or short- and long-term preventive administration of any other agent.
The spontaneous pain intensified since December 2014, and therefore, the patient was referred to another hospital. However, the anesthesiologist refused to perform general anesthesia in that hospital. Thereafter, she was referred to our department in January 2015.
From around 16 years of age, the patient had started experiencing repeated anaphylactic-like reactions (facial edema, coughing, difficulty breathing, etc.), and she felt uncomfortable and repeatedly visited the emergency room. At 18 years of age, the patient was transported by an ambulance to the department of pediatrics for treating seizure-like symptoms, ranging from abdominal pain to coughing, pruritus, dyspnea, marked facial edema, and edema of the oral mucosa. At that time, she was diagnosed with a suspicion of hereditary angioneurotic edema. However, examinations conducted for the most common Type I HAE (decreases in CH50 and C1–INH levels) resulted in negative findings, and hence, she was clinically diagnosed with HAE of unknown type (Type III) in the pediatrics department. The levels of C1–INH activity, C3, and C4 were within normal range ( Table .1 ). The symptoms primarily occurred during times of overwork before her menstrual period. Regarding the family medical history, neither the patient’s mother, nor the father had been diagnosed with HAE. The patient was also not treated with short- or long-term preventive administration of any other agent till the age of 18 years. There were no notable extraoral findings during the initial examination. However, intraoral findings included impacted left and right mandibular wisdom teeth, spontaneous pain, and swelling. All test values were within standard ranges, including complement levels in the clinical laboratory findings. The root apices of the bilateral mandibular wisdom teeth were found to be in close proximity to the mandibular canals in the radiographic findings ( Fig. 1 A). We made a clinical diagnosis of bilateral horizontal impaction and pericoronitis of the mandibular wisdom teeth. We believed that an invasion of wisdom tooth extraction could be severe. It was decided to extract the horizontally impacted mandibular wisdom teeth under intravenous anesthesia using propofol. Due to the risk of developing a viral infection, both the patient and her mother refused prophylactic administration of Berinert P, a human plasma-derived C1–INH formulation. However, both agreed for plasma-derived C1–INH administration in case urgent laryngeal edema occurs during and after the operation. On the day of the surgery, the bilaterally impacted mandibular wisdom teeth were extracted without the prophylactic administration of C1–INH using a combination of intravenous anesthesia with propofol and infiltration anesthesia with propitocaine hydrochloride and 7.2 ml felypressin (Citanest with octapression R ), following preparations for emergency C1–INH administration and tracheotomy. Intratracheal intubation was not performed to avoid the stimulation of the trachea. The duration of the operation was 65 min, and there was less bleeding. Steroids were administered intraoperatively for treating the swelling associated with tooth extraction procedures. Urgent tracheotomy was prepared during the surgery but was not performed since there was no edema. The patient was treated with antimicrobial agents for 4 days after the surgery to prevent infection and was discharged 3 days after the surgery without any postoperative edema ( Fig. 1 B). Afterwards, she had a good course ( Fig. 1 C).