Text/Yangcheng Evening News all-media reporter Zhang Hua
Correspondents Zhang Yang, Lin Xiaoyun, Liao Fang
Photo/Courtesy of the hospital
On the morning of March 25, Mr. Liang's family from Guangzhou waited anxiously outside the operating room of Sun Yat-sen Memorial Hospital at Sun Yat-sen University.
In the operating room, a special operation prepared for Mr. Liang's 13-year-old son is being carried out in an intense and orderly manner. Malignant hyperthermia, a disease that makes all anesthesia practitioners smell discolored, is the source of anxiety and worry for Mr. Leung's family. It all started with an operation two months ago...
▲The medical team urgently rescued the child
Malignant hyperthermia occurred during the operation, and the body temperature rose to 42 °C at one point
According to Mr. Liang's recollection, his son was found to have a right pinna deformity since birth, and was diagnosed as "congenital right-sided chinese and foreign ear deformity with hearing impairment" in the Department of Otorhinolaryngology of Sun Yat-sen Memorial Hospital of Sun Yat-sen University.
About 1 hour after the operation, the surgeon Associate Professor Liang Maojin and the attending physician Chen Yuebo found that the child's muscle fibrillation in the operating area was obvious.
At the same time, the attending physician of the Department of Anesthesiology, Hu Chuwen, also observed an abnormal change in a series of vital signs in the child: in less than 10 minutes, his body temperature rose from 38.1 °C to 39.2 °C, his heart rate rose from 98 beats/min to 122 beats/min, etco2 rose from 45 mmHg to 103 mmHg, and the above indicators showed a continuous upward trend.
▲Vital signs when the child has "malignant hyperthermia"
The anesthesiologist and surgeon immediately realized the seriousness of the situation, and The attending physician Hu Chuwen initially suspected that the child had "malignant hyperthermia", immediately stopped inhaling the anesthetic and eluted the inhaled anesthetic in the respiratory circuit with atmospheric flow, and quickly asked Professor Lin Daowei, deputy director of the Department of Anesthesiology, to direct the rescue; Associate Professor Liang Maojin immediately asked Professor Huang Xiaoming, director of the Department of Otolaryngology, and Professor Zhang Zhigang, deputy director of the Department of Otolaryngology, to urgently close the surgical cavity and actively cooperate with the rescue.
After Professor Lin Daowei arrived at the scene, he accurately and quickly assessed the child's condition, affirmed the possibility of malignant hyperthermia, and pointed out that the child was currently in critical condition and needed to immediately organize further rescue work, including immediate physical cooling, correction of acidosis and electrolyte disorders, correction of arrhythmias, monitoring of blood sugar, maintenance of urine output, etc.
For a time, the doctors and nurses in the operating room staged a rescue battle. Professor Lin Daowei immediately contacted Wu Junyan, director of the Pharmacy Department of the hospital, and urgently invoked the special drug dantrologin for malignant high fever rescue.
As the minutes and seconds passed, the child's body temperature reached a maximum of 42 ° C, and the heart rate reached a maximum of 168 beats per minute.
After about an hour and a half of thrilling rescue, Xiao Liang's condition finally turned critical, and the vital signs gradually stabilized, the body temperature dropped below 38 ° C, and the heart rate dropped to less than 100 beats per minute.
Subsequently, the trabecular was transferred to the ICU for close monitoring and survived the 48-hour danger period after the malignant high fever. For safety reasons, the surgeon and the anesthesiologist jointly decided to recommend that they undergo elective surgery after recovering from discharge.
Re-admitted to the hospital and successfully completed the operation within 3 hours
Two months later, Xiao Liang returned to the hospital for surgery.
By summarizing the experience of the first surgical anesthesia, combined with the latest progress of malignant hyperthermia anesthesia program at home and abroad, under the guidance of Professor Lin Daowei, Attending Physician Hu Chuwen designed a personalized anesthesia program for Xiaoliang, using all-rely intravenous anesthesia, avoiding the use of inhalation anesthetics and drugs such as succinylcholine that may induce malignant hyperthermia, and doing a good job in risk plans and emergency treatment measures for various anesthesia complications, including malignant hyperthermia, and closely monitoring changes in vital signs during surgery.
It took about 3 hours for Associate Professor Liang Maojin to successfully complete the operation for Xiao Liang.
Only 5 minutes after the completion of the operation, Xiao Liang woke up smoothly, resumed spontaneous breathing, did not appear malignant fever again, and the hanging hearts of all the medical staff involved in the operation were finally put down.
▲Associate Professor Liang Maojin performed surgery on Xiao Liang
The onset of malignant hyperthermia is rare but dangerous, with a mortality rate of up to 73.5%
Professor Lin Daowei introduced that malignant hyperthermia is a family hereditary disease mainly induced by volatile inhalation anesthetics and depolarizing muscle relaxants (such as succinylcholine), and its incidence is about 1/10000-1/250000. "I've been practicing medicine for decades, and I've known about the disease in books and never really seen it. This case is also the first case of malignant hyperthermia recorded in our hospital, which is indeed rare. ”
How dangerous is malignant hyperthermia?
Professor Lin Daowei said that malignant hyperthermia can be said to be one of the most serious anesthesia-related complications in the perioperative period, and once the disease occurs, the disease progresses rapidly and eventually dies. Patients usually have no abnormal performance, no different from normal people, usually after general anesthesia suddenly appear end-expiratory CO2 partial pressure continues to rise, and the body temperature rises sharply, up to 40 ° C or more, if the treatment is not timely, multi-organ failure can occur rapidly.
According to experts, the current clinical special drug for malignant hyperthermia is dantrolline, according to domestic literature reports, in the past due to the lack of dantroroline in China, the mortality rate of malignant hyperthermia was as high as 73.5%. At present, with the localization of danquo lorraine, the rescue success rate of the disease is expected to be greatly improved.
▲ Hu Chuwen attending physician removed the tracheal catheter for The trabecle
Expert Reminder:
There is a family history of malignant hyperthermia, and genetic testing should be improved
Associate Professor Liang Maojin mentioned that after Mr. Liang's son stabilized his condition, he improved genetic testing, and the results showed that a missense variant of the RYR1 gene carrying a malignant hyperthermia susceptibility gene was detected, which was considered a pathogenic variant.
After learning the genetic test results, Mr. Liang has been worried, on the one hand, he is eager to continue to solve the problem of the shape of the ear that has plagued the child for more than ten years through surgery, on the other hand, he is afraid of the malignant high fever that appears in anesthesia.
Associate Professor Liang Maojin patiently explained that congenital pinna deformities require two to three surgical treatments in stages, as long as the operation is fully prepared, a suitable anesthesia plan is formulated, and drugs that can cause malignant hyperthermia are avoided, and the smooth completion of the operation can be ensured. The remarks dispelled the concerns and worries of Mr. Liang's family, and said that they would continue follow the doctor's advice and continue follow-up treatment.
Professor Lin Daowei reminded that for relatives of patients with malignant hyperthermia, the following three points need to be paid attention to:
1. Malignant hyperthermia is a genetic disease, so people with a family history of malignant hyperthermia should improve genetic testing, exclude susceptibility, and achieve early prevention;
2. Although the incidence of malignant hyperthermia is low, the mortality rate is high, and some commonly used anesthetic drugs are the precipitating factors for malignant hyperthermia, so it is very important to prevent it from the source;
3. Before surgery, we must inform the doctor that "I have relatives who have had malignant hyperthermia", so that the surgical and anesthesiologists can take appropriate preventive measures in advance to minimize the possibility of malignant hyperthermia. (For more news, please pay attention to Yangcheng Pie pai.ycwb.com)
Source | Yangcheng Evening News Yangcheng Pie
Editor-in-charge | Liu Xinyu
Proofreading | He Qiyun