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Anesthetic Considerations for End Stage Amyotrophic Lateral Sclerosis in Pregnancy
Abstract Number: RF6BI-495
Abstract Type: Case Report Case Series
Amyotrophic Lateral Sclerosis is a progressive neuromuscular disease that usually presents in the fifth decade of life or later, with only 3.2% of cases diagnosed before the age of 40. This case report describes a 42 year old G9P6117 with history of 7 vaginal deliveries and advanced ALS (diagnosed at age 39), quadriplegia, dysarthria, dysphagia, severely restricted pulmonary mechanics (FVC 26%, FEV1 21%) and chronic venous stasis who presented for induction of labor at 33 weeks, 5 days, due to worsening pulmonary function and lower extremity edema.
Upon arrival, the patient received betamethasone and the fetus was found to be in transverse lie. Prior to external cephalic version (ECV), two peripheral IVs and an arterial line were placed. In the OR, an epidural was placed in the lateral position at L3-4 and bolused with 2% lidocaine with epinephrine. The ECV was performed successfully with a T8 sensory block, and the patient returned to labor and delivery where an epidural solution of 0.1% ropivacaine + 2mcg/mL fentanyl was infused at a rate of 6mL/hr. After 6 hours of labor and AROM, the fetus returned to the transverse position, requiring Cesarean section under epidural analgesia. Delivery was complicated by moderate post partum hemorrhage requiring transfusion, and post-operative pain was managed with a multimodal approach, including an epidural infusion of 0.2% ropivacaine.
ALS is rarely seen in pregnancy, though scattered case reports exist describing parturients in various stages of the disease. Afflicted women may deliver vaginally, as the disease does not affect the smooth muscle of the uterus, and the pelvic floor is relaxed. Our patient’s disease course was typical, beginning with weakness of the intrinsic muscle of the hands and progressing over four years to bulbar weakness and quadriplegia.
Patients with ALS have restrictive pulmonary physiology, but maintain FRC, RV and gas exchange. The respiratory demands of pregnancy may precipitate acute respiratory failure in these patients. Our patient required BiPAP to maintain oxygenation > SpO2 92%. Parturients with ALS are also at high risk for aspiration due to oropharyngeal dysphagia, and benefit from early scheduled IV GI prophylaxis.
Epidural anesthesia has been used successfully in parturients with ALS, but local anesthetics and narcotics must be dosed cautiously due to the risk of exacerbating weakness. Though sometimes necessary, general anesthesia poses several challenges. First, patients with significant respiratory involvement may decompensate with opioid administration. Rapid sequence induction and endotracheal intubation should be performed to avoid aspiration, though there is a risk of prolonged intubation depending on the severity of respiratory involvement. Non-depolarizing paralytics should be avoided if possible (ALS patients may not require paralytics for intubation) while succinylcholine is contraindicated due to the risk of hyperkalemia and cardiac arrest.