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Anesthetic management for vaginal delivery in a parturient with Limb-Girdle Muscular Dystrophy and obesity
Abstract Number: T1D-10
Abstract Type: Case Report/Case Series
Intro: Limb-Girdle Muscular Dystrophy (LGMD) is a rare disorder with symptoms ranging from mild muscle weakness to severe respiratory compromise. Anesthetic concerns in affected parturients include airway abnormalities, elevated aspiration risk, respiratory insufficiency, myocardial abnormalities, anesthetic sensitivity, and increased obstetric complications. We discuss a case of vaginal delivery (VD) following neuraxial analgesia (NA) in a parturient with LGMD.
Case: A 23yo woman, G1P0, with LGMD diagnosed at age 12, presented at 34+1 weeks with premature rupture of membranes. She had mild aortic regurgitation and Lambl’s excrescence on echocardiography. She reported occasional palpitations, rapid limb fatigue, and dyspnea on exertion. Motor exam was grossly normal. Comorbidities included controlled asthma and obesity (BMI 37.8). CPK (343) and CKMB fraction (7.8) were elevated.
A 17G epidural Tuohy needle was inserted at the L3-L4 interspace with loss of resistance to saline at 8 cm. An epidural catheter was secured at 13 cm. A test dose (3 cc 1.5% lido with 1:200,000 epi) was followed by 6 cc 0.125% bupi with fentanyl 2 mg/cc. A PCEA of 6 cc/hr continuous and 6 cc demand bolus q15 min was maintained for labor. Bilateral T8 sensory level was achieved in 30 minutes. The patient delivered an infant via VD and remained hemodynamically stable with adequate pain relief. The catheter was removed uneventfully.
Discussion: Pregnancy exacerbates LGMD symptoms including diaphragmatic weakness, aspiration, cardiomyopathy, and conduction abnormalities. Decreased lung function in LGMD with hypercarbia, maternal, and intrauterine fetal death has been reported1. Reduced expiratory reserve increases incidence of atelectasis and pneumonia. Obese LGMD patients may also develop restrictive disease, pulmonary hypertension, and cor pulmonale2. General anesthesia (GA) risks are compounded by increased sensitivity to opioids, volatiles, and paralytics3.
Compared to spinal, epidural management may facilitate control of hemodynamics and respiratory mechanics. Despite this patient’s respiratory risks and opioid sensitivity, we administered dilute epidural fentanyl without adverse effects. Given GI tract hypomotility and aspiration risk, adequate NPO status and prophylaxis including antacids, prokinetics, and H2-blockers are recommended4. In case of bulbar involvement, anesthesiologists should consider RSI for GA with a non-depolarizing agent with a predictable recovery profile to avoid electrolyte swings and exaggerated myotonic fasciculations.
Our case highlights the benefits of successful epidural management in a parturient with LGMD. Because of such varied clinical presentation, these patients benefit from individualized multidisciplinary management.
1 Awater C et al. Eur J Obstet Gyn Reprod Biol 2012;162:153.
2 Javan J et al. Int J Med and Pharm Case Reports 2017;10:1.
3 Álvarez A. 2015; www.orphanesthesia.eu
4 Nair A et al. Indian Anaesth Forum 2012;14:1.