Freeman-Sheldon syndrome (FSS) is an exceedingly rare congenital disorder with an unspecified prevalence. FSS is caused by a mutation in the embryonic skeletal muscle myosin heavy chain 3 gene. Patients may have facial abnormalities that put them at risk of difficult airway intubation. These facial abnormalities include micrognathia, macroglossia, high-arched palate, prominent forehead, and mid-face hypoplasia. Additionally, skeletal abnormalities such as joint contractures, scoliosis with resultant restrictive lung disease, and camptodactyly (bent fingers) can be noted. These features played an important role in the anesthetic management of our FSS patient. Perioperative planning and optimization were crucial in her anesthetic management as she underwent an urgent cesarean section due to preeclampsia with severe features.
Keywords: acute pulmonary embolism; bilevel positive airway pressure; c-section; cranio-carpal-tarsal dysplasia; difficult airway management; freeman-sheldon syndrome; pre-eclampsia; syndromic scoliosis; whistling face syndrome; windmill-vane-hand syndrome.
Copyright © 2021, Fayed et al.