Labor Analgesia and Anesthesia Considerations in a Primigravida with Osteogenesis Imperfecta Type IV and Prior Spinal Instrumentation and Fusion for Scoliosis
Abstract Number: 179
Abstract Type: Case Report/Case Series
Introduction: Osteogenesis imperfecta (OI) is a rare group of collagen synthesis disorders leading to excessive fragility of bones and an increased propensity to fracture. A primagravida with OI type IV with prior spinal fusion and instrumentation for idiopathic scoliosis presented for evaluation for labor analgesia and anesthetic options for possible cesarean section (C/S).
Case Report: A 22-year-old primigravida with OI type IV at 29 weeks gestation presented for evaluation of labor analgesia and anesthetic options for C/S, if required. The patient had previously undergone spinal fusion from the T2 to L1 level secondary to scoliosis approximately twelve years earlier. The patient had numerous fractures of her ribs and all extremities with multiple orthopedic surgeries throughout her lifetime. Despite the multiple healed fractures, the patient was ambulatory upon presentation. Previous surgeries were conducted under general anesthesia (GA) utilizing both volatile anesthetics as well as total intravenous anesthesia (TIVA). There were no documented malignant hyperthermia (MH) -like reactions. Laboratory data showed a hemoglobin level of 11.2 gm/dL, a hematocrit of 32.6%, and a platelet count of 217,000. Airway examination revealed a Mallampati class 3, a thyromental distance of 4.5 cm, and full range of motion of the cervical spine. The patient was advised that epidural analgesia would likely be ineffective due to possible adhesion of the dural sac to the posterior spinal canal from previous spinal fusion. Possible labor analgesic options for vaginal delivery would include continuous spinal catheter, single shot spinal, or remifentinil patient controlled analgesia combined with a pudendal nerve block. If the patient would require urgent or emergent C/S, anesthetic options include GA utilizing TIVA, a single shot spinal, or a continuation of a previously placed spinal catheter. The patient was also informed that arterial cannulation for blood pressure monitoring may be advisable as there have been reported cases of extremity fractures associated with noninvasive blood pressure cuffs and two large bore intravenous catheters would be placed upon her admission in labor because of the possibility of pelvic fracture and internal bleeding.
Discussion: Pregnancy in patients with OI combined with corrective spinal fusions for scoliosis present many unique challenges for anesthesiologists. Patients with OI have a substantially increased risk of MH or MH-like reactions. Patients with types III or IV OI have particularly brittle bones. Spontaneous vaginal deliveries have been associated with pelvic fractures, leg fractures from placement into stirrups, and upper extremity fractures from NIBP cuffs. Epidural placement could lead to spinal fractures of posterior lamina in patients with severe disease. Proper preparation for these patients should lead to improved maternal outcomes with a significant reduction in peripartum morbidity.