Anesthesia management for emergency laparotomy in a pediatric patient with suspected hereditary angioedema.

Hereditary angioedema (HAE) is caused by complement factor 1 inhibitor (C1-INH) deficiency, and its mode of inheritance is autosomal dominant. We present a case of an 8-year-old patient who required emergency laparotomy after a traffic accident. General anesthesia with tracheal intubation was necessary. The patient's mother and maternal grandmother had been diagnosed with HAE. HAE is associated with high mortality when airway edema is caused by tracheal intubation. It was impossible to rule out HAE preoperatively in the patient. Therefore, we presumed that he had HAE and treated him with pasteurized C1-INH concentrate. The patient underwent laparotomy uneventfully. Several days after the operation, the laboratory data revealed that the perioperative plasma complement 1 q subunit (C1q) protein level and C1-INH function were not lowered. The diagnosis of HAE was not confirmed, but it was not possible to rule out the diagnosis either. The prophylactic use of a C1-INH in this case may be justified, because the procedure was an emergency and because of the high mortality associated with tracheal intubation in patients with HAE.

[Case of myotonic dystrophy eventually diagnosed by preoperative Allen's test].

A 59-year-old woman was scheduled for resection of a parotid tumor under general anesthesia. Preoperative interview was done 7 days before the operation. Laboratory data were normal except for a slightly elevated CPK level. Though ECG showed abnormal P and T waves, exercise ECG was normal. Respiratory function test revealed decreased %VC. She did not complain of motor weakness and atrophy of distal muscles. When the anesthesiologist did Allen's test, slow relaxation of hand grip, i.e. grip myotonia, was observed. Further history taking disclosed that she had a family history of myotonic dystrophy (MD) in her son. She was diagnosed as MD by a neurologist on the day of preoperative interview, although she lacked obvious symptoms and consciousness of MD. The operation was performed as scheduled and she was anesthetized with oxygen-nitorous oxide, propofol, fentanyl and vecuronium. Vecuronium was not antagonized because of the adverse effect of neostigmine reported in MD patients. The operation and anesthesia were conducted uneventfully. Her recovery from anesthesia was so smooth that she was discharged uneventfully. In this case, careful examination led to diagnosis of MD and prevented perioperative complications associated with MD. It is important to examine patients meticulously not to miss trivial symptoms in our daily visits.