An anaesthetic in ophthalmology in patients with hereditary vascular edema

The patient, female, 55 years old, 56kg, 160 cm, due to the car accident injury "right eye fracture injury, right eye socket fracture" admitted to the hospital, intended in the whole hemp down the "right eye contents out of the accompaniment of the eye implants , eye socket forming surgery , eyelid cleavage stitching." Thediagnosed 10 years ago at Concord Hospital in Beijing"hereditaryvascularedema (hereditary angioedema, HAE)check the body: conscious, expression pain, face skin mucosa light moderate edema, accompanied by abdominal painBlood routine, chest tablets, electrocardiograms are basically normalEye CT show: right eye fracture, right upper jaw sinus wall, right eye socket inside and outside side wall, double-sided nasal bone, right bow multiple fracturesSerum supplements C30.63g/L (normal values 0.9 to 1.8g/L) and C40.068g/L (normal values 0.1 to 0.4g/L)diagnosis: multi-site injury, eye fracture (right), eye fracture (right), traumatic glass volume blood (right), hereditaryvascularedemaafter multidisciplinary consultation sororities such as anaesthetic, otolaryngology, ophthalmology, rheumatismimmunology, the male hormone Danarin 600mg prevention treatment, infusion of fresh frozen plasma (FFP) 400ml and dynamic monitoring serum supplements, the art is prepared for tracheostology After the above treatment, after 3d patients face facial mucosal edema reduction, no abdominal pain, review C30.83g/L and C40.092g/L Open peripheral intravenous infusion channel after admission, in-room BP120/70mmHg, SpO298% preoperative medication: Atropine 0.3mg After preparing 400ml FFP and tracheotomy bags before surgery, start full hemp induction: propofol 60mg and fentanyl 0.1mg Induced smooth, mask continuous oxygen, FiO2 80%, oxygen flow 5L/min Conventional disinfection of the towel, ophthalmologist to 2% Lidoca in line right ball after nerve block Anesthetic maintenance: propofol 1.5 to 2 mg kg-1 h-1 and rifenite 0.1?g.kg-1-min-1 Maintain SpO2 , RR12 to 20 times / minute, BP90 to 120 / 60 to 70mmHg, BIS40 to 60 The operation went smoothly, lasted 1h, infusion 400 mlFFP, and complications such as throat edema and eye reflexes occurred during the operation After waking up without breathing difficulties, the patient was sent back to the ICU and re-infusion FFP200ml after the operation due to abdominal pain After 1d transfer to the general ward, after 4d successfully discharged A total of FFP1000ml was infusion during the perination period, urticaria appeared during postoperative infusion of FFP, and intramuscular injection of isopropyl 50 mg was treated discussion
HAE is a rare genetic disease caused by genetic defects, there is no uniform diagnostic standards and cure methods, only to control the symptoms The main clinical is characterized by repeated swelling of the skin, respiratory tract and internal organs, when edema occurs in the airways, can cause throat edema, if the rescue is not timely, 1/3 patients can be suffocated to death When edema occurs in the stomach intestinal , abdominal pain and other symptoms of acute abdominal disease, often misdiagnosed as appendicitis, leading to emergency caesarean section The cause of the disease is related to the reduction or functional defect of C1 inhibitor inhibitor (C1 inhibitor, C1INH) in the patient's serum, so that C1 is over-activated, the cleavage of C4 and C2 is out of control, and the resulting slowly-stimulated peptides increase, so as to increase the permeability of microvascular vessels and cause edema Unlike allergic or drug-induced vascular edema, glucocorticoids or antihistamines are ineffective against HAE-induced edema The prevalence of HAE is about 1/10000 to 1/50000 The most commonly used drug in the country is dacanazole, the maintenance dose depends on the disease, ranging from 50 to 600 mg per day As C1INH, the source of plasma, has not yet entered China, there is still a lack of therapeutic drugs for acute haE attacks clinical to apply FFP to treat HAE-induced throat edema, breathing difficulties, vomiting and abdominal pain, infusion volume in 200 to 1000 ml, individual patients in the early infusion abdominal pain aggravated, after infusion 30 to 90min symptoms improved and after 2 to 12h completely subside A small number of patients can develop urticaria, and intramuscular injection of isopropyl 50 mg reduces allergic reactions Infusion FFP shortens the acute exacerbation process in HAE patients and reduces the symptoms of systemic edema However, the method has the risk of worsening symptoms, and the infusion process needs to be closely observed this case of patients due to trauma haE acute attack, clinical manifestations to face skin mucosa light moderate edema and abdominal pain mainly, there is no breathing difficulties and throat edema Because hemp can not inhibit the stress stimulation of the proposed eye surgery, and the surgical stimulation has the possibility of inducing throat edema The combined infusion propofol and rifenite can produce satisfactory anaesthetic effects under BIS monitoring, creating a good operating environment for eye surgery Therefore, this time selected general anesthesia, but did not line tracheal intubation, nor placed the throat cover and pharynx ventilation channel and other sound door ventilation device, in order to avoid stimulating the patient's respiratory tract and induced throat edema, but through the mask oxygen this non-invasive ventilation method to control the patient's respiratory tract on the basis of fully ensuring the effect of nerve blockafter after the ball, reduce the applied dose of narcotic drugs, ensure adequate oxygen supply and avoid CO2 retention, and maintain appropriate anaesthetic depth However, only the mask to oxygen of the airway management method is worth exploring, the risk of anesthesia is greater The key points of the patient's anaesthetic management: (1) to learn more about the patient's medical history, morbidity, medication and the recent presence of airway and throat edema; In summary, haE incidence is low, misdiagnosis rate and mortality is high, clinicians do not fully understand it, the lack of acute onset treatment drugs in China, clinically to Danaazole and FFP prevention the main incidence of laryngosis, the risk of anesthesia is greater Once a severe throat edema is induced, the trachea should be cut open