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Osteogenesis Imperfecta and Regional Anesthesia in a Parturient
Abstract Number: S-44
Abstract Type: Case Report/Case Series
Osteogenesis Imperfecta (OI) or “brittle bone disease” involves osteopenia with primary defects in the protein matrix of connective tissue and bone (1,2) and varies in severity. Clinical presentations include excessive bone fragility, fractures, short stature, scoliosis, triangular face (small jaw, large vault), bruising, and increased laxity of skin, ligaments and heart valves (1,2). There are eight subgroups; type 1 is the mildest and type 2 the most severe. The optimal mode of delivery is controversial as labor and vaginal delivery could lead to pelvic (mom) and fetal fractures, although cesarean section (C/S) does not result in fracture reduction (1). Anesthetic management has included general, epidural and spinal anesthesia (3). Particular concerns include patient fragility, vertebral column abnormalities (short stature, kyphoscoliosis leading to respiratory compromise), bleeding (platelet adhesion abnormalities), and airway abnormalities, (1,3) all of which make GA and particularly RA challenging (1,3).
An 18 y/o primigravida with a history of type I OI presented at 37+ weeks in labor for an elective C/S despite counseling that vaginal delivery would not increase her fracture risk. She had declined fetal testing. Our patient had a history of surgeries for multiple fractures and repair of a globe rupture. She weighed 126 lbs and was 4’8” tall. Physical exam revealed a mallampati class III airway with no limited neck motion. Examination of her spine demonstrated small interspaces and mild kyphoscoliosis. Due to short stature and history of no prior vertebral fractures, we chose a CSE (plt 140,000/μL, no easy bruising) using 11.5 mg of hyperbaric bupivacaine, 10 mcg of fentanyl and 100 mcg of hydromorophone as the anesthetic. Our patient delivered a viable healthy baby (no signs of OI) weighing 5 lbs, 7 oz. with Apgars of 9 & 9.
The advantages of spinal anesthesia are its rapid onset, reliability and minimal transplacental drug passage (1,3). However, in patients with OI, maternal short stature and kyphoscoliosis can make spinal anesthesia difficult. The amount of drug to use without causing a total spinal in a patient with a possibly difficult airway is a concern. The distribution of local anesthetic may be unpredictable depending upon the degree of scoliosis. Placement of an epidural can be problematic as well. If vertebral abnormalities do not prevent its placement, a CSE can incorporate the benefits of spinal (dense solid block) and epidural anesthesia (extended duration), while minimizing the risk of hypotension and high/total spinal by using a decreased spinal dose. With proper preoperative assessment and preparation of a parturient with OI, CSE may be a viable anesthetic option for elective C/S.
1. Obstetric Anesthesia and Uncommon Disorders, 2nd ed. Cambridge Inc,2008
2. Journal of the American Academy of Orthopaedic Surgeons 1998;6:225-236
3. Anesthesiology 1984;61:91-93