Limb-girdle muscular dystrophy in pregnancy: a narrative review

Abstract

Limb-girdle muscular dystrophy (LGMD) poses unique challenges for women during pregnancy, necessitating comprehensive care and tailored management strategies. The present narrative review aims to examine the unique challenges and management strategies required for women with LGMD during pregnancy. With over 30 genetic subtypes identified and the potential for additional discoveries through advanced diagnostic techniques, preconception counseling plays a crucial role in informing prospective parents about reproductive risks and available options. Baseline assessments, including cardiac and pulmonary evaluations, are essential to guide antenatal care, alongside genetic testing for precise diagnosis and counseling. Optimizing maternal health through respiratory exercises, cardiac monitoring, and individualized exercise and nutrition plans is paramount to avoid potential complications. During pregnancy, close monitoring of maternal and fetal well-being is important, with collaborative care between obstetricians and specialists. An individualized approach to delivery mode considering factors such as muscle strength, pelvic size, and fetal presentation is crucial. While vaginal delivery has been proven to be possible, the need for an emergency cesarean delivery should always be kept in mind. Regional anesthesia is preferred, with proactive planning for potential respiratory support. Bupivacaine has been shown to be effective with epidural catheters that may be used for prolonged relief with opioids like morphine and fentanyl, while also evaluating the patients’ respiratory function. Postpartum considerations include pain management, mobility support, breastfeeding assistance, and emotional support. Early mobilization and tailored physiotherapy regimens may promote optimal recovery, while comprehensive breastfeeding guidance is needed to address challenges related to muscle weakness. Access to mental health resources and support networks is essential to helping individuals cope with the emotional demands of parenthood alongside managing LGMD. By addressing the unique needs of pregnant individuals with LGMD, healthcare providers can optimize maternal and fetal outcomes while supporting individuals in their journey to parenthood.