A case of caesarean section anaesthetic in patients with dynamic protein type 2 genotype central nucleopathy

The patient, female, 35 years old, 59kg, suspended for 37-2 weeks, was admitted to hospital for deliveryPast history: The patient was diagnosed with central nuclear muscle disease (centronuclear myopathy, CNM) in 1995 and identified its disease-causing basis as the dynamic protein 2 gene (DNM2), a chronic history of hepatitis B, at the Huada Genetic Testing Center in 2017Check body: T36.5 degrees C, HR99 times / minute, RR20 times / minute, BP122/70mmHg;heart color super: poor coordination of left ventricular wall movement, mild reflux of two and three tip valves, EF57%; Preoperative examination: pregnancy 37 plus 2 weeks, ASAIII level, NYHA heart function level III, high bow, small jaw, open mouth 3 fingers, mild restrictive ventilation disorderThe far end of the limb or the lower limb muscle force 3-4 level, tendon reflection weakenedIt is scheduled to be performed on December 22, 2017, with a general anaesthetic of the trachea intubationsurgery: The patient performed a caesarean section on December 22, 2017, entered the room to inject 40mg of sodium nylon sodium acetate, and used 1% Lidocarin to perform local immersion anesthesia at the surgical incision site before the start of the operationAnesthetic induction: Midazolam 2mg, Shufentani 20 ?g, propofol 120mgTrachea intubation: Visible trachea duct No7 under the laryngoscopeanaesthetic maintenance: propofol 4.0mg kg-1 h-1, Riffentani 10?g.kg-1-h-1 continuous pumpingDuring surgery, the airway pressure was maintained at around 18 cmH2ODuring the operation successfully cut out a baby boy, the operation used 0.35% ropica in 20 ml at the incision layer by layer immersion analgesicAfter being admitted to PACU30min, the patient is fully awake, muscle strength recovers, and breathing is normal after the tube is removeddiscussion
CNM is a congenital myopathy characterized by cell nuclei abnormality in a large number of myofibers that are not characterized by apparent myofibrosis or compensating growth The pathological characteristic is that the proportion of muscle fiberwith with central nucleus is obviously increased, and the clinical characteristic is non-progressive or slow progressive muscle weakness According to its genetic method can be divided into 4 types: X chain recessive genetic, autosomal dominant genetic, autosomal recessive genetics and exudation type The disease-causing basis because of DNM2, the course of progress is slow, neonatal period to adulthood can start the disease, clinical symptoms vary, often to the limb far end or near end muscle weakness as the first symptom only a small number of patients with congenital myopathy in pregnancy literature reported that the cesarean anaesthetic treatment is of little significance Therefore, the greatest risk of anesthesia comes from the unknown, preoperative anesthesia assessment mainly considers the following aspects: (1) the effect of anesthesia on respiratory muscle, swallowing, cough reflection; in the choice of anesthesia mode, because the patient's nerve conduction slows down, muscle force decreased, especially to the far end of the limb obvious, and the drug to reduce nerve excitability and conductivity, whether there is an impact on the patient's nerve function recovery, whether the development of the patient's condition will be aggravated, it is not known Therefore, in order to try not to affect the movement and recovery of muscle force, the use of general anesthesia to assist local incision immersion, abandoned intra-vertebral anesthesia second, taking into account the patient's high bow, small jaw, in the larynx housing placement process may be difficult, and pregnant women's stomach draining time is longer, caesarean section in the abdominal compression is prone to reflux misabsorption, so a relatively higher safety of tracheal intubation full hemp In the selection of whole hemp drugs, the incidence of anesthesia and malignant hypertherance is reported to be 1/100000 to 1/5000, while the incidence rate of patients with genetic abnormalities can be as high as 1/3000 Therefore, inhalation of drugs such as narcotic drugs, which are prone to induced malignant high fever, should be avoided in such patients Secondly, this patient is an autosomal dominant genetic DNM2 CNM patient, the lesions are located in myosin, myamindrugs may affect the activity of myoglobin, adverse to the patient's motor function and prognosis Therefore, in the process of anesthesia, should avoid the use of muscle loose medicine in a word, CNM has a large impact on multi-organ function throughout the body, the risk of anesthesia surgery is very high, should do a good job of detailed preoperative evaluation, the development of personalized anaesthetic program, mainly consider not affecting the patient's actin and neuromuscular function, avoid the occurrence of serious complications in surgery, in order to meet the needs of surgery at the same time, to ensure the safety of patients and reduce the impact on motor function