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Elective Caesarean Delivery in a Patient with Spondylometaphyseal Dysplasia
Abstract Number: T-25
Abstract Type: Case Report/Case Series
Case Report: 29 year-old G1PO at 28 2/7 with spondylometaphyseal dwarfism admitted for increasing shortness of breath from progressive pulmonary compromise related to body habitus and a phenotypically normal fetus. The patient noted progressive orthopnea and dyspnea on exertion. Her medical/surgical history was significant for asthma, GERD, gastroparesis, depression, and 2 lower extremity procedures with straightening pins. She was admitted for 40 days prior to the procedure for monitoring of pulmonary function. Unless there were earlier fetal or maternal indications, the plan was to proceed with elective Cesarean delivery at 34 weeks gestation via classic vertical incision and hysterotomy. On the morning of surgery, her airway exam showed intact dentition, Mallampati III, short thyromental distance, adequate mouth opening, short neck, and limited neck extension. Prior to induction, an L3-4 epidural was placed in the sitting position for postoperative analgesia in anticipation of pain from the classic incision and existing pulmonary compromise. Rapid sequence induction was performed with lidocaine, propofol, and rocuronium. A video laryngoscope was utilized with a 5.0 millimeter endotracheal tube. The vocal cords were seen with some difficulty, and the tube passed after 2 attempts. She delivered a healthy fetus (APGAR: 2 at 5 min, 8 at 5 min) through a low-transverse incision and had an uneventful intraoperative course. After delivery, the epidural was loaded with 2 mL bupivacaine 0.5% and morphine 2 mg. She was extubated after the procedure, and, per patient request, the epidural was removed that evening. She was dismissed home on postpartum day 4.
Discussion: These patients may present unique challenges to the anesthesia provider including: difficult intubation, difficult ventilation secondary to restrictive lung disease, cervical cord compression and instability, difficult regional placement, and potential need for prolonged respiratory support. Our patient had progressive pulmonary compromise from her short stature and phenotypically normal fetus; therefore, adequate postoperative analgesia was necessary to prevent pulmonary complications. The use of regional anesthesia has been described in patients with spondylometaphyseal dysplasia, but the risk of extensive or unreliable spread of neuraxial anesthetic is increased. Given the unpredictable spread of neuraxial anesthesia and baseline orthopnea, we elected to proceed with general anesthesia for the case and neuraxial analgesia for pain control. Fortunately, obstetricians were able to perform a low transverse incision and the patient was highly motivated to return to baseline activity. Pain control was adequate with single injection epidural morphine, oral opiates, and multimodal analgesia.
References: 1) Walters BA, et al. Am J Med Gen 2004;129A(3):265-76. 2) Benson KT, et al. Anesthesiology 1985;63:548-50. 3) Kumar MM, et al. Int J Obst Anesth 2002; 11(3): 225-7.