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Anesthetic Management of a Parturient with Limb Girdle Muscular Dystrophy Type 2D For Cesarean Delivery
Abstract Number: FCA-414
Abstract Type: Case Report Case Series
Muscular dystrophies are genetic neuromuscular disorders associated with abnormalities of the muscle membrane and are characterized by progressive loss of skeletal muscle function(1). Limb girdle muscular dystrophy (LGMD) is characterized by severe weakness that primarily involves the shoulder and hip girdles(2). LGMD can be autosomal recessive or dominant(3). The literature is limited in regards to pregnancy and delivery in patients with the disease(4). The following case report describes the successful anesthetic management of a parturient with LGMD at our institution.
A 33 year-old G1 presented with Class III obesity (BMI of 42) and LGMD Type 2D. She was diagnosed at age 22 via muscle biopsy and the only one in her family with LGMD. Although she could perform some activities of daily living, her mobility was limited and required the assistance of a wheelchair. Her cardiac history was significant for cardiomyopathy. A third trimester echocardiogram showed normal LV and RV function with an ejection fraction of 60%. Her pulmonary history was found to be unremarkable. She was scheduled for induction at 39 weeks and 6 days, but presented to labor and delivery at 40 weeks and 4 days after experiencing spontaneous rupture of membranes. She requested an epidural for analgesia. Given her medical history, body habitus and desire to avoid general anesthesia, the plan was to place an epidural. Her epidural was successfully placed while sitting with one attempt at the L3-L4 interspace. Labor analgesia was initiated with a continuous infusion of bupivacaine and fentanyl. After laboring for more than 24 hours, a decision was made by the obstetricians to perform a cesarean section for failure to progress. She received a total of 20mL of 2% lidocaine with epinephrine through her epidural catheter which provided bilateral T4 surgical block. She delivered a healthy boy 20 minutes after incision. Intraoperatively, she suffered hemorrhage secondary to uterine atony which resolved with a dose of carboprost 250mcg IM. She remained hemodynamically stable. After meeting postpartum goals, she was discharged home on POD# 5 with daily enoxaparin 40mg SQ to prevent deep vein thrombosis.
This case illustrates the successful use of epidural anesthesia in providing a safe and uneventful cesarean delivery in a parturient with LGMD. Women with LGMD have been reported to have an increase in progression and exacerbation of their disease during pregnancy(3). Given the risks due to the increased physiological demands of pregnancy, the possibility of worsening respiratory function and potentially developing rhabdomyolysis and malignant hyperthermia, neuraxial anesthesia is preferred over general anesthesia(4).
1. Lancet. 2002;359:687-695
2. Neuromuscul Disord 2003; 13:532-44
3. International Journal of Obstetric Anesthesia. 2007; 16:370-374
4. Obstetric Medicine. 2010; 3:81-82