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Anesthetic Considerations in a Parturient with Freeman Sheldon Syndrome
Abstract Number: T-64
Abstract Type: Case Report/Case Series
Introduction: Freeman Sheldon Syndrome (FSS) is a rare genetic disorder characterized by malformations of the face, oral cavity, and musculoskeletal system. Complications during anesthesia have been reported in individuals with this disorder. The most common complication is difficult intubation. Malignant hyperthermia, muscle rigidity and pyrexia have been reported in patients receiving MH triggering anesthesia1-4. We present the anesthetic management of a pregnant patient with FSS undergoing an elective cesarean delivery.
Case Report: A 36-year-old pregnant woman at 36 weeks gestation with past medical history of FSS and cannabis use, was booked for elective cesarean delivery due to concerns about hip dislocation if a vaginal birth was attempted. She had numerous orthopedic and maxillofacial procedures as a child that were uneventful. Her past medical history also included pacemaker insertion for arrhythmias from chronic methamphetamine use, spontaneous abortion that did not require any intervention and gestational diabetes during this pregnancy that is controlled on diet. Physical findings were camptodactyly, contractures in distal extremities, small nose, lengthened philtrum, and masked facies. She had severely limited mouth opening of approximately 2cm. (figure). She had a Mallampati III score, poor dentition and shortened thyromental distance of 3cm. We concluded that intubation would be difficult, but that bag mask ventilation was possible. She also had a mild lumbar kyphoscoliosis with curvature to her left side. Intravenous cannula insertion was successful after 3rd attempt using Ultrasound. Combined spinal epidural was attempted. Epidural space was detected but spinal needle placed through the epidural needle failed to enter the subarachnoid space, so spinal component was abandoned. Adequate block was achieved via epidural top up. The rest of the procedure was uneventful. Post-operative pain was well controlled with oral medications, and the patient was discharged two days post operatively.
Discussion: Challenges and complications faced by anesthesiologists in managing a patient with FSS can be numerous. As these patients progress into adulthood and become pregnant, anesthetic care will be required.
1. Jones R et al. Anesthesiology 1992;77:599-600.
2. Altunhan H et al.Case report. Genetic couns 2010;21:347-51.
3. Gurjar V et al. Maxillofac. Oral Surg 2013;12:395-99.
4. Munro HM et al. Paediatric Anesthesia 1997;7:345-8.