Anesthetic management of spinal muscle atrophy type II in a parturient

Abstract

We report the peripartum management of a 30-year-old wheelchair-bound nullipara woman with spinal muscular atrophy (SMA) type II, including severe restrictive lung disease and Harrington rods. At 38 weeks gestation, she was admitted for an induction of labor with neuraxial analgesia, but she subsequently had to be delivered via cesarean section under general anesthesia. We describe the anesthetic implications of SMA on labor and delivery management and review the available literature.

Combined spinal-epidural anesthesia in a patient with spinal muscular atrophy type II undergoing cesarean section: A case report

“…There is limited literature describing anesthetic management of a pregnant women with SMA and both regional blocks and general anesthesia have been used 7 . However, Bollag et al 6 present a literature review of 18 case reports describing anesthetic management of patients with SMA. There were 12/18 patients delivered via scheduled cesarean section: 7/12 patients received general anesthesia (GA), 4/7 were due to failed neuraxial anesthesia; 4/12 patients received neuraxial anesthesia, 1/4 required a secondary neuraxial technique; 1/12 patients received local anesthetic infiltration and i.v.…”

“…There were 12/18 patients delivered via scheduled cesarean section: 7/12 patients received general anesthesia (GA), 4/7 were due to failed neuraxial anesthesia; 4/12 patients received neuraxial anesthesia, 1/4 required a secondary neuraxial technique; 1/12 patients received local anesthetic infiltration and i.v. sedation; 2/18 patients delivered via non-scheduled cesarean section and 4/18 patients had vaginal deliveries 6 . In case series of 12 patients with SMA, who delivered a total of 17 infants, obstetric complications were reported in 13/17 (76%) deliveries, and included: premature labor (6/17 deliveries) and preterm delivery (2/17 deliveries); prolonged labor (4/17 deliveries); and prolonged recovery postpartum (6/12 women), cesarean section (3/12).…”

“…Epidural anesthesia may fail due to unpredictable inadequate spread of local anesthetics, particularly if there had been severe scoliosis and corrective back surgery using Harrington rods, due to scar tissue, and if you use an epidural catheter or continuous spinal catheter allows careful titration to achieve the desired dermatome level 8 . Positioning a wheelchair-bound patient and defining landmarks can be difficult, therefore it may be beneficial to use ultrasound-guidance prior to attempting placement of neuraxial anesthesia 6 . Reported doses administered for spinal anesthesia range from 7.5-14 mg hyperbaric bupivacaine, fentanyl 15-25 μg, and morphine 0.1 mg 3,8,11 .…”

“…Other options reported for cesarean section include local anesthetic infiltration (0.5% lidocaine 100 mL) supplemented with i.v. sedation (midazolam, morphine and propofol) and occasionally with the addition of oxygen/nitrous oxide, but this technique will not provide a block as dense as with a neuraxial technique 6,13 . Ilioinguinal, iliohypogastric and transversus abdominis plane nerve blocks have been used as options for intra-and post-operative pain control 5,14 .…”

“…Severe RLD is often present in patients with SMA, and with the additional stress from physiological changes of pregnancy, pulmonary function can be worsen during pregnancy but may improve post-delivery 9 . However, postoperative ventilatory support in the intensive care unit may be necessary due to respiratory weakness 6,18 . Intra-and postoperative analgesia can be achieved using multimodal analgesia regimens, including opioids 3,7,20 .…”