///2019 Abstract Details
2019 Abstract Details2019-07-13T07:45:15-05:00

Management of a Parturient with Spinal Muscle Atrophy Type III

Abstract Number: RF5AH-103
Abstract Type: Case Report Case Series

Chantal L . Mendes M.D. 1 ; Jared Kafer D.O.2; Kalpana Tyagaraj M.D.3

Introduction: Spinal Muscle Atrophy (SMA) is an autosomal recessive, neuromuscular disorder associated with degeneration of spinal anterior motor neurons with an incidence of 1/10,000 births. We present a parturient with history of SMA Type III, who underwent a Cesarean section. Case presentation: We describe the perioperative management of a wheel chair bound 28-year-old female, G1P0, with SMA Type III. History was significant for intrahepatic cholestasis of pregnancy, anemia, controlled asthma, PCOS, and an MRI of the brain revealing multiple white matter plaques, suspicious of multiple sclerosis. Patient had severe thoracic and lumbar dextro-scoliosis and underwent surgical spinal fusion without any instrumentation. Initially, the obstetric care team planned to perform labor induction at 37 weeks gestation for intrahepatic cholestasis of pregnancy. A multidisciplinary discussion was held involving obstetric care team, anesthesiologists and neonatologists, and the decision was made to perform a C-Section in anticipation of difficult vaginal delivery due to her wheel chair bound status and pelvic insufficiency. The patient received spinal anesthesia with hyperbaric bupivacaine and fentanyl. Patchy analgesia existed on the left lower abdominal quadrant which was successfully supplemented with intravenous ketamine. Following C-Section and ligation of left uterine artery, the patient was admitted to surgical intensive care unit for postoperative care. Patient received hydromorphone PCA for pain management. On postpartum day two, she was transferred to the floor and transitioned to oral pain medication. Patient was discharged on postpartum day three. Discussion: SMA is a genetic defect due to a homozygous deletion of exon 7 in survival motor neuron 1 gene resulting in a lack of SMN protein in spinal anterior motor neurons. Its classification, types 0-IV, is based on age of onset and severity of symptoms. Classic findings include progressive muscle weakness, joint contractures, restrictive pulmonary disease, scoliosis, and dysphagia.Anesthetic management goals include a multidisciplinary discussion for successful outcomes. A comprehensive history and physical exam are essential with complete assessment of pulmonary function, severity of muscle weakness and spinal deformities. If neuraxial anesthesia is planned, previous spinal surgery and severe scoliosis can result in incomplete block. Local anesthetic dose needs to be lowered to prevent a high block, which can worsen compromised respiratory function. If general anesthesia is chosen, difficult intubation due to limited cervical spine mobility, avoiding succinylcholine due to upregulation of acetylcholine receptors and cautious use of nondepolarizing muscle relaxants are anticipated. References: Islander G. Anesthesia and spinal muscle atrophy. Pediatr Anesth. 2013 Feb; 23: 804-816. Bollag et al. Anesthetic management of spinal muscle atrophy type II in a parturient. Local Reg Anesth. 2011; 4:15-20.



SOAP 2019