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Anesthetic Considerations for a Parturient with Freeman-Sheldon Syndrome
Abstract Number: RF7A10-80
Abstract Type: Case Report Case Series
Intro: Freeman-Sheldon syndrome (FSS) is rare genetic disorder associated with malformations of the face, oral cavity, and musculoskeletal system. This case presents the anesthetic considerations of a parturient with FSS.
Case: A 23 y/o F G1P0 at 38 weeks gestation with a past medical history of hypertension and FSS presents to labor and delivery unit for trial of labor. Surgical history was pertinent for multiple upper and lower extremity surgeries, as well as T2-T12 scoliosis correction with Harrington rods. Physical exam was notable for short stature 4’7” and BMI 32. A midline longitudinal scar extended throughout her thoracic spine. Airway exam revealed a flat nose, small mouth opening, short inter-incisor distance, webbed neck, and a limited range of motion of the cervical spine. Outside hospital anesthetic records documented a previous difficult airway but detailed airway management was unobtainable. The patient was concerned about the risks of neuraxial anesthesia, but after informed consent, the patient elected epidural analgesia for labor. An epidural catheter was successfully placed at L3-L4 using a loss of resistance technique to air. An intentional dural puncture with a 25 gauge spinal needle was performed to confirm placement. The patient delivered via an uncomplicated spontaneous vaginal delivery and the epidural catheter was removed postpartum.
Discussion: Anesthetic considerations for patients with FSS include neuaxial anesthesia, airway management, and IV access. These patients have scoliosis and musculoskeletal contractures that require surgical correction making neuraxial placement more difficult. Surgical interventions may result in a compromised/completely obliterated epidural space resulting in patchy spread of local anesthetics or an inadvertent dural puncture. Bone grafting and fusions create limited areas of needle placement. Dural puncture epidural (DPE) may be used to confirm successful midline entry into the epidural space. A study by Chau et al. found DPE result in a statistically significant reduction in unilateral epidural analgesia, improved caudal spread of analgesia, and reduction in provider top-offs compared to standard epidurals. In addition, abnormal anatomy (small mouth opening, micrognathia, webbed neck with limited range of motion) puts the patient at risk for difficult ventilation and oral intubation. Limited nasopharyngeal space in this population may limit the feasibility of a nasal approach to endotracheal intubation. Awake Fiberoptic intubation is indicated. Finally, Restrictive movement of extremities secondary to joint deformities/contractures and scar tissue from previous surgery can make IV placement difficult
Chau et al. Anesthesia & Analgesia 2017;124(2),560-569
Fisher et al. International Journal of Obstetric Anesthesia 2016;27, 81-84
Hebi el al. International Anesthesa Research Society 2010;111(6), 1511-1519
Viehmeyer et al. Case Reports in Anesthesiology,2018, 1-4