Abstract
UNLABELLED: 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.
CASE PRESENTATION: 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.
DISCUSSION: 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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 6-8 |
| Number of pages | 3 |
| Journal | Journal of the Mississippi State Medical Association |
| Volume | 57 |
| Issue number | 1 |
| State | Published - Jan 1 2016 |
| Externally published | Yes |