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Anesthetic Management of a Parturient with Type IV Osteogenesis Imperfecta Undergoing Cesarean Delivery
Abstract Number: F-66
Abstract Type: Case Report/Case Series
INTRODUCTION: Osteogenesis imperfecta (OI) is a rare group of genetic conditions of autosomal dominant inheritance characterized by bones that fracture easily due to collagen defects. Management of parturients with OI may present significant anesthetic challenges and literature is scarce on this topic. We describe successful cesarean delivery anesthesia in a patient with type IV OI.
CASE REPORT: A 28-year-old term G3P2 with type IV OI presented for elective cesarean delivery (CD). Her past surgical history was significant for posterior spinal correction and fusion with Harrington instrumentation due to severe scoliosis. Physical examination revealed a height of 4’4”, weight of 55 kg, and a Mallampati 1 airway. Notably, she had a barrel chest, persistent severe scoliosis despite spinal fusion, and an impressive keloid midline vertical scar extending from T1 to S2 that was 3 cm in width in the lumbar region. The decision was made to proceed with general endotracheal anesthesia via RSI with cricoid pressure. She was intubated using a video laryngoscope with a 6.5 ETT and an arterial line was placed immediately after induction. CD proceeded uneventfully. A healthy infant was born (Apgars 3/8) and the patient was successfully extubated.
DISCUSSION: There are four well-established types of OI, with symptoms ranging from bone fragility to short stature, scoliosis, tooth breakage, and hearing loss. Both neuraxial and general anesthesia techniques have been described in the setting of OI. Neuraxial placement in patients with scoliosis and/or previous back surgery may be complicated by technical difficulty, positioning challenges, decreased efficacy, and unpredictable spread. There are many other anesthesia-related concerns associated with OI. The inflation of automated blood pressure cuffs may result in fractures; thus, the decision was made to utilize invasive blood pressure monitoring. Impaired platelet function has been documented in OI patients, requiring a patient’s coagulation status to be evaluated prior to regional anesthesia. Succinylcholine should be avoided due to the potential for fractures from fasciculations. Finally, hyperthermia may be noted in these patients intraoperatively, but it is not a precursor for malignant hyperthermia.
"Pregnancy Outcomes in Women with Osteogenesis Imperfecta." The Journal of Maternal-Fetal and Neonatal Medicine (2016).
Stoetling’s Anesthesia and Co-Existing Disease, 6th edition.