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CESAREAN DELIVERY FOR A PATIENT WITH FREEMAN-SHELDON SYNDROME
Abstract Number: RF7B10-252
Abstract Type: Case Report Case Series
INTRODUCTION: Freeman-Sheldon is a rare genetic condition. This condition is so rare that in the early 1990’s only 65 cases were reported.. It is characterized by abnormalities of the face, hands, and feet. Features include microstomia, microglossia, micrognathia, and high-artched palates (1) These can lead to problems in speech development and swallowing. Joint deformities include camptodactyly, scoliosis, and talipes equinovarus (2). Those affected have an increased risk of malignant hyperthermia (3). Inheritance patterns vary, although an autosomal dominant pattern is common.
CASE: A 34 year-old G1P0 at 27 weeks and 4 days with a history of Freeman-Sheldon syndrome, acute pulmonary embolism, and preeclampsia with severe features underwent a cesarean section. She had a history of scoliosis with Harrington rods that extended from her thoracic to lumbar spine. Due to preeclampsia with severe features and deteriorating respiratory status requiring BIPAP with settings of 25/5, she underwent a classical cesarean section with general anesthesia.
Our patient had a difficult airway, which included a mallampati III, limited neck extension, a thyromental distance of 2, and micrognathia. We wanted to ensure a successful intubation and minimize any hypoxia, hypercarbia, and hypotension that could worsen her already compromised respiratory status. Our patient agreed to an awake intubation. We anesthetized her airway with viscous 4% lidocaine, administered 100 mg of I.V. lidocaine, and 0.2 mg of glycopyrrolate to improve intubating conditions. She did not react when we tested her airway with a tongue blade.
We successfully performed an awake video laryngoscope intubation, and then induced with propofol and ketamine. We employed a propofol infusion and utilized EEG monitoring to keep our patient deeply anesthetized and reduce awareness under anesthesia. We avoided any agent that could potentially trigger MH in this susceptible patient. She was successfully extubated in the I.C.U. after further management of her pulmonary embolus.
Sheldon-Freeman syndrome is incredibly rare. Care must be taken to minimize the risk of malignant hyperthermia by avoiding any triggering agents. One must be prepared with alternative modalities for securing the airway, which included in our case a fiberoptic intubation and a setup for a possible surgical airway. The patient above and her premature infant did well postoperatively. This patient was an emergency case that required careful and prudent anesthetic management to optimize intubating conditions, prevent malignant hyperthermia, and safely and expeditiously deliver using a general anesthetic for cesarean delivery.
1. Stevenson, DA et al. (2006) Pediatrics. 117: 754-762.
2. Cruickshanks, G, et al. (1999) Canadian Journal of Anesthesia. 46: 783-787.
3. Fisher, K. (2016) International journal of obstetric anesthesia. 27: 81-84