Abstract
Femoral Hypoplasia-Unusual Facies Syndrome (FH-UFS) is a rare congenital condition. We treated a 17-year-old woman with FH-UFS and hypoplasia in both legs and arms, micrognathia, and extreme trismus. The excision of the coronoid process under general anesthesia was planned to improve the extreme trismus. She had undergone general anesthesia on several occasions for the treatment of cleft palate, plastic surgery for her hands and legs, and scoliosis surgery.
For the induction of general anesthesia, tracheal intubation was smoothly performed using a microscope inserted through the nasal cavity under sedation with the continuous injection of propofol smoothly. General anesthesia was maintained with air-oxygen-sevoflurane and the continuous injection of remifentanil without any remarkable episodes. We tried in vain to obtain an arterial line during the operation. After completing the operation, the patient was transferred to the intensive care unit (ICU) and the tracheal tube was removed on the following morning. No remarkable episodes occurred while the patient was in the ICU. FH-UFS is reportedly associated with a high maternal blood glucose level during gestation. However, only 38.2% of patients with FH-UFS are born from pregnant woman with high blood glucose levels, and other pathological background factors remain unknown. Our case was a typical case of FH-UFS, and no particular difficulties occurred except for the extreme trismus and the difficulty in obtaining an arterial line.
