Abstract

NM-209

Appropriateness of Outpatient Anesthesia for a Child with Freeman Sheldon Syndrome

Fox J, Montgomery C
University of Kentucky, Lexington, KY, USA

Introduction: Freeman Sheldon syndrome (FSS) is a rare multi-system congenital myopathy that presents a unique challenge due to the risk of difficult intubation and IV access. The disorder is characterized by significant craniofacial abnormalities, contractures of the distal limbs, and thickened subcutaneous tissue. We review the appropriateness for outpatient anesthesia in a two-year old, ten kilogram female with FSS for the application of a Mehta cast to treat congenital scoliosis.
Case Description: The patient initially presented to an ambulatory surgery center for casting, however the case was postponed based on the patient’s small mouth opening and anticipated airway difficulty, and rescheduled at an inpatient hospital. The patient was brought to the operating room and standard ASA monitors placed. Inhalational induction commenced with sevoflurane in nitrous oxide and oxygen. IV access was obtained with a 24 gauge catheter. Endotracheal anesthesia was required secondary to increased abdominal and thoracic pressures during casting. No muscle relaxant was given. Three attempts were made at securing the patient’s airway. The first two with direct laryngoscopy utilizing a miller 1 and macintosh 2 blades, revealed a Cormack grade 4 view. The GlideScope® 2 blade was used on the third attempt revealing a grade 3 view. A styleted 4.0 oral endotracheal tube was then successfully passed and anesthesia maintained using sevoflurane. The patient tolerated the procedure and anesthetic without complications. She was extubated in a deep plane and taken to PACU for observation and then discharged home.
Discussion: FSS was first described in 1938 and although no epidemiologic data exists, there have been less than 100 case reports and even fewer in the anesthetic literature. Patients have a characteristic “whistling face” secondary to microstomia, micrognathia, and pursed lips. These features lead to significant difficulty with intubation. Distal contractures of the limbs along with fibrosis of the subcutaneous tissue lead to challenges with IV access. There have been several case reports indicating the potential for increased susceptibility to malignant hyperthermia in FSS, however this has not been well established. Inhalation induction was chosen in our case due to anticipated difficulty obtaining IV access. Dantrolene was available if necessary. Based upon these characteristics the initial surgery was appropriately delayed at the ambulatory center and later occurred at the inpatient hospital where adequate airway and IV tools were available. The patient was subsequently scheduled at the outpatient surgery center for her repeat castings based upon the initial successful anesthetic management and has had positive outcomes.
Conclusion: Despite the potential for difficult airway and IV access, FSS is a complex disorder that can be managed on an outpatient basis if adequate measures are taken in preparation for the anesthetic.
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