Schwartz Jampel syndrome is a rare genetic disorder that involves developmental delay, short body structure, phenotypic facial defects, and musculoskeletal system abnormalities. The child was discharged on the seventh postoperative day. Paracetamol 10 mg/kg was administered for postoperative analgesia. The surgery lasted 115 min, was completed without any problems, and LMA was removed. During the surgery, the cardiac pulse was 110–140 beats/min, body temperature was 36.6–36.90C, and EtCO2 level was 32–37 mm Hg. Maintenance of anesthesia was provided with TIVA using 6–10 mg/kg/h propofol and remifentanil 0.05–0.25 μg/kg/min. After then, laryngeal mask airway (LMA) size 1.5 was successfully inserted in the supine position. Anesthesia was induced with propofol 1 mg/kg and fentanyl 0.5 μg/kg. Following the failed caudal block, TIVA was decided to be applied. The child was placed in a lateral decubitus position for the caudal block. 0.05 mg/kg midazolam and 1 mg/kg ketamine were administered for sedation, and ventilation was provided via the mask. The heart rate was 126 beats/min, SpO2 was 99%, and body temperature was 36.8☌. Monitoring, including electrocardiogram, pulse oximetry, and body temperature measurement, was applied.

Due to the risk of developing malignant hyperthermia, necessary preparation (removal of inhalation agents from the anesthesia device, supply of cold serum and drapes, and dantrolene) was provided. There was no pathology in the cardiopulmonary system examination and laboratory tests.

There was a possibility of difficult intubation due to abnormal facial defects, the contraction of the jaw muscles, and the limitation of head and neck movements. In addition, the child had a seizure again 1 month ago. The child had been hospitalized in the intensive care unit due to epilepsy and had been used 200 mg/day carbamazepine. I present total intravenous anesthesia (TIVA) using propofol and remifentanil without neuromuscular blockers (NMBs) was successfully performed in a child with Schwartz Jampel syndrome (SJS).īilateral undescended testis surgery was planned for an 18-month-old child weighing 7 kg with SJS.

Schwartz jampel syndrome treatment