Sugammadex was present in the OR before anesthesia induction. For anesthesia induction, we injected 30 μg of fentanyl, 20 mg of propofol, and 8 mg of rocuronium. As the possibility of malignant hyperthermia inhalation induction with sevoflurane with spontaneous breathing was unsafe, we decided to do rapid sequence induction of anesthesia.įirst, we gave an intravenous line with 20 g chatter from the patient’s right hand. Because the patient did not cooperate, an awake intubation plan was impossible. The anesthetic machine was cleaned of volatile agents by disconnecting and removing the vaporizer and renewing CO 2 absorbent using a new disposable breathing circuit and O 2 flushing for 50 minutes.ĭantrolene was sufficiently available. Difficult intubation equipment and considerations were arranged (LMA and pediatric fiberoptic bronchoscope, video laryngoscope, and special blades for difficult intubation were available at the bedside in the OR).Ī four-year-old female with Schwartz-Jampel syndrome and epilepsy Due to limited mouth opening and micrognathia, difficult intubation was predictable despite a prior history of anesthesia. The patient’s weight was 10 kg.Įvaluation of the airway and the Mallampati scoring were unsuccessful due to the patient’s inability in mouth opening and facial muscle rigidity ( Figure 1). In the preoperative examination, she had a small ASD in echocardiography with a normal EF of 60%. She also had a history of knee fracture surgery one year ago with no anesthesia problems. She had a drug history of Tegretol syrup and baclofen tablets.
Her symptoms started with muscle weakness at 10 months old, leading to a myotonia diagnosis. She had a family history of SJS, with an uncle also suffering from SJS. Due to the small number of cases of these patients and the lack of experience in administering general anesthesia to these patients, we decided to present this case.Ī four-year-old female with American Society of Anesthesiologists (ASA) status III, SJS, and epilepsy was scheduled for Blepharoplasty in Rasuol Akram Hospital. Facial muscle contraction leads to a “mask-like” appearance with pursed lips and blepharophimosis ( 1, 2). The treatment of these patients is challenging for anesthesiologists. Patients display microstomia, micrognathia, thermoregulatory disturbance, jaw muscle rigidity, myopathy, anatomical deformities, and malignant hyperthermia. Weakness and stiffness, abnormal bone development, short stature, joint contractures, and facial dysmorphisms. Schwartz-Jampel Syndrome (SJS) is a rare autosomal recessive disease characterized by muscle General Anesthesia Intubation Myotonia Schwartz-Jampel Syndrome 1. We managed this patient by applying Rocuronium, propofol, and C-MAC video laryngoscopy. These patients may have difficult intubation and be prone to malignant hyperthermia.
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