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.
A 30-year-old female with no significant past medical history presented to our labor and delivery ward for induction of labor. Due to failure to progress, she was proceeded to cesarean delivery. Intraoperatively, it was noted that her uterus was hypotonic; she required supplemental methylergometrine to control the bleeding from the uterine atony. However, within three minutes of intramuscular (IM) administration, she complained of chest pain. She then subsequently developed pulmonary edema in the postoperative care unit, which required supplemental oxygen. She was found to have elevated troponin and brain natriuretic peptide (BNP), along with radiologic features of fluid overload suggestive of congestive cardiac failure, which all lead to the diagnosis of non-ST myocardial infarction. The patient had a normal computed tomography (CT) pulmonary angiogram, echocardiogram, and serial electrocardiograms (ECGs). She was successfully discharged from the hospital on postoperative day 4 with resolution of her symptoms and improving cardiac enzymes. Cardiology outpatient follow-up was arranged.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.