[Anesthetic management of an infant with Freeman-Sheldon syndrome].
We report the anesthetic management of Freeman-Sheldon (whistling face) syndrome in a two-month-old boy scheduled for lateral canthoplasty. He had features of the syndrome including blepharophimosis, hypertelorism, a flat nose, microstomia with a limited opening, micrognathia, a very short webbed neck, scoliosis and multiple arthrogryposis. He was fed with a naso-gastric tube and suffered from several episodes of aspiration and oxygen desaturation. Difficult airway and intubation were anticipated. Anesthesia was induced via a mask with sevoflurane, although mask ventilation was difficult. Direct laryngoscopy and the insertion of a laryngeal mask airway were impossible due to microstomia with the limited opening as anticipated. A naso-tracheal intubation was achieved using a fiberoptic bronchoscope via a fiberoptic mask while ventilating the lungs. The operation and anesthesia afterwards were uneventful. In the ward, he was given supplemental oxygen but with occasional desaturation episodes. Thirteen days after the operation he was found cyanotic and resuscitation was attempted but failed. Autopsy demonstrated the hypoplasia of the lungs and thorax, atelectasis and bronchitis.
Department of Anesthesiology, Osaka Medical Center, Research Institute for Maternal and Child Health, Izumi 594-1101.
Megumi
M
Okawa
Keiko
K
Kinouchi
Seiji
S
Kitamura
Akihiro
A
Taniguchi
Noriyuki
N
Sasaoka
Kazuo
K
Fukumitsu
D000015:Abnormalities, Multiple; D000768:Anesthesia, General; D001176:Arthrogryposis; D016569:Blepharophimosis / Q000601:surgery; D019882:Blepharoplasty; D019465:Craniofacial Abnormalities; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D008865:Microstomia; D019990:Perioperative Care; D012600:Scoliosis; D013577:Syndrome