A Case of Freeman-Sheldon Syndrome: Anesthetic Challenges.
Patients with Freeman-Sheldon Syndrome (FSS) often need multiple surgical procedures. We present a case of FSS and discuss the anesthetic challenges associated with the case.


METHODS
A 10-week-old female with FSS presented for elective Nissen fundoplication and gastrostomy tube insertion. She had a history of difficult intubation at birth. General anesthesia with inhalational anesthetic and spontaneous respirations technique was used. Fiber optic bronchoscope (FOB)-assisted nasal intubation was successful after failed attempts with a Miller blade, GlideScope, and intubation through a laryngeal mask airway (LMA). She did not exhibit any signs of malignant hyperthermia (MH) during or immediately after the procedure.


CONCLUSIONS
Challenges to the anesthesiologist in a case with FSS include establishing IV access, intubating the trachea, risk of MH and MH-like symptoms, and postoperative pulmonary complications. Proper multidisciplinary preoperative planning is essential for optimum care of these patients, preferably in a tertiary care center.
Thomas
T
Hamilton
Madhankumar
M
Sathyamoorthy
D000768:Anesthesia, General; D001999:Bronchoscopy; D003394:Craniofacial Dysostosis / Q000150:complications / Q000601:surgery*; D005260:Female; D018662:Fundoplication; D005774:Gastrostomy; D006801:Humans; D007223:Infant; D007442:Intubation, Intratracheal